A prediction of internship performance.

A prediction of internship performance. Previous studies of predictors for occupational therapy internship performance were based on the Report of Student Performance, the tool used before 1974 to evaluate students' clinical performance. The instrument was never tested for validity or reliability. No studies for prediction of clinical performance used the present Field Work Performance Report (FWPR), which was tested for validity and reliability before its adoption late in 1973. Thus, new studies using the FWPR were indicated. Grades from 16 selected college courses and the pre-occupational therapy grade-point average were studied to determine significant relationships with the physical disability intership grades of 72 students from Colorado State University. Neurology was significant at the .0004 level. Further study is necessary to confirm the present findings and to determine additional predictors for clinical performance of Colorado State University students.


Role of the asymmetrical tonic neck reflex in hand visualization in normal infants. Fourteen infants were each videotaped 7 times within the first 12 weeks of life to determine whether or not the asymmetrical tonic neck reflex had a role in placing their hands within their fields of vision. Infants' arms were more frequently out of the reflex position. Hands were within the peripheral visual field more often than in the focal visual field or not within the visual field. The proportion of observations that hands were in a particular visual field varied in accordance with the presence or absence of the reflex. A higher proportion of reflex observations occurred in the focal visual field, whereas a higher proportion of observations made when the infants were not in the reflex position occurred in the peripheral visual field. Further, the proportion of focal field observations in the reflex position was greater than the proportion of observations out of the reflex position for the first six of the seven ages. This study confirms that when infants are in the asymmetrical tonic neck reflex position, their hands are more likely to be in a position in which they can be visualized.


Advising the mastectomy patient about reconstruction. Breast reconstruction deserves critical consideration and discussion in each patient's case. The psychologic benefits from the procedure are enormous. Reconstructive goals are: skin coverage, contour restoration and formation of the nipple-areola. The prospect of reconstruction must have no effect on the procedure for extirpation of the cancer. Breast reconstruction is now possible even after the removal of large amounts of skin and muscle.


In-toeing gait in children.

In-toeing gait in children. Torsional deformities in the lower extremities, resulting from constrained intrauterine position, tend to spontaneously correct toward the adult configuration during growth. Internal tibial torsion, metatarsus adductus and femoral anteversion are clinically evident conditions that occur because of arrested normal postnatal development. An understanding of the dynamics of spontaneous correction permits assessment of the child with in-toeing gait as well as the selection of proper treatment.


Infection of glutaraldehyde-preserved porcine valve heterografts. Gross, histologic and ultrastructural changes associated with bacterial infection are described in four porcine valve heterografts that had been in place in patients for 6 days to 28 months. In one patient, culture of the aortic tissue tag included in the heterograft container grew Mycobacterium chelonei; however, examination of the heterograft, recovered at necropsy 6 days after implantation, revealed small colonies of bacteria that differed morphologically from mycobacteria. A second heterograft was the site of staphylococcal infection associated with extensive destruction of collagen in the leaflets. Similar destruction was observed in a third heterograft, which was found to have organisms on ultrastructural study even though bacterial cultures of the valve were negative. The fourth heterograft, from a patient who died of coronary embolism secondary to dislodgment of vegetative material, contained structures resembling lysed bacteria. Observations in these 4 patients and review of published reports of infection involving 43 other patients with porcine valve heterografts indicates that infection in these valves: (1) develops in the fibrin layer that covers the cusps, (2) can involve the collagen in the leaflets, and (3) is uncommonly (three patients) associated with valve ring abscesses.


Effect of diltiazem, a calcium antagonist, on myocardial ischemia. In line with studies on the metabolism of the ischemic myocardium, the effectiveness of diltiazem hydrochloride, a potent calcium antagonist, in reducing the effects of ischemia was evaluated. Nonischemic and ischemic tissue samples were examined in two groups of dogs--Group I, dogs receiving no drug and killed after 60 minutes of regional ischemia, and Group II, dogs given diltiazem after 10 minutes of ischemia and killed 50 minutes later. Administration of diltiazem proved beneficial in several ways: The decrease in adenosine-5'-triphosphate in the ischemic region was halved, inhibition of anaerobic glycolysis was reduced, tissue levels of lactic acid and free fatty acids were lowered and the contractility of glycerinated heart muscle fibers was improved. However, administration of the drug did not influence mitochondrial function. Mitochondrial oxygen consumption and respiratory control were reduced by equal amounts in both groups, as was mitochondrial calcium ion binding. These observations demonstrate that diltiazem is capable of minimizing the consequences of acute ischemic, although the beneficial effects do not extend to all aspects of myocardial metabolism.


Canine electrophysiology of encainide, a new antiarrhythmic drug. Encainide, a new benzanilide derivative with high potency and a good therapeutic/toxic ratio, was evaluated with the use of standard His bundle recording techniques to determine its effects on the cardiac conduction system in closed chest animals. Twenty mongrel dogs weighing 18 to 29 kg were anesthetized with 4 percent chloralose and classified into groups: group 1, a control group and groups 2, 3, and 4, which were given 0.3, 0.9 and 2.7 mg/kg body weight, respectively, of encainide in an intravenous infusion over a 15 minute period. Plasma concentration, blood pressure, surface electrocardiogram and atrial and His bundle electrograms were recorded before, during and after drug infusion for a total of 120 minutes. Heart rate, A-H and H-V intervals, the QRS complex and Q-Tc interval were measured every 5 minutes during sinus rhythm and with constant atrial pacing. In addition, sinus nodal recovery time and atrial, atrioventricular (A-V) nodal and left ventricular refractory periods were measured before and immediately after infusion and every 30 minutes for 2 hours. Peak plasma concentration averaged 450 ng/ml in group 2, 1,300 ng/ml in group 3 and 4,000 ng/ml in group 4. Blood pressure was not altered at any dose level throughout the study. The QRS complex and H-V interval were significantly prolonged (P less than 0.005) at doses of 0.9 mg/kg and greater. These effects correlated well with plasma concentration. There was no significant change in heart rate, corrected sinus nodal recovery time, A-H interval, Q-Tc interval atrial, A-V nodal or left ventricular refractory period. It is concluded that, unlike other antiarrhythmic agents, encainide prolongs His-Purkinje system conduction without significantly affecting conduction or refractoriness of other parts of the cardiac conduction system in animals.


Effects of thioridazine (Mellaril) on ventricular electrophysiologic properties. The effects of therapeutic and toxic doses of thioridazine (Mellaril) (10 and 50 mg/kg body weight, respectively) on ventricular electrophysiologic properties were studied in 12 anesthetized dogs. Threshold pacing currents (diastolic threshold), effective refractory period and conduction time were significantly increased, and idioventricular automaticity was suppressed after administration of 10 mg/kg of thioridazine; the effects were much more pronounced after administration of 50 mg/kg. Rapidly repetitive responses or tachycardia could be induced in the ventricle by two early premature beats in 9 of the 12 dogs after the 50 mg/kg dose, but they did not occur before drug administration or after the 10 mg/kg dose. These results indicate that the antiarrhythmic and arrhythmogenic effects of thioridazine are dose-dependent and that careful monitoring with frequent electrocardiograms is needed for patients receiving large doses of this drug.


Multiple gated cardiac blood pool imaging for left ventricular ejection fraction: validation of the technique and assessment of variability. The intrinsic variability and accuracy of left ventricular ejection fraction determined by multiple gated cardiac blood pool imaging was evaluated in 83 patients. Ejection fraction by gated studies correlated well with data from first pass radionuclide angiocardiography (r = 0.94) and from contrast angiography (r = 0.84). Intra- and interobserver variabilities of absolute ejection fraction were minimal (mean +/- standard deviation 1.4 +/- 1.2 and 1.6 +/- 1.5 percent, respectively) and were not different for normal (ejection fraction 55 percent or greater) and abnormal patients. Ejection fraction was determined twice in 70 patients: on the same day at intervals separated by 1 to 2 hours (41 patients) and on 2 different days (29 patients). Ejection fraction ranged from 18 to 91 percent and was normal in 37 patients. There was no difference in mean serial variabilities of absolute ejection fraction for all repeat studies performed on the same and separate days (3.3 +/- 3.1 versus 4.3 +/- 3.1 percent (not significantly different). The mean variability of absolute ejection fraction for repeat studies in normal patients was significantly greater than in abnormal patients (5.4 +/- 4.4 versus 2.1 +/- 2.0 percent, P less than 0.01). The incidence rate of absolute interstudy changes of 5 percent or more was significantly higher in normal than in abnormal patients (P less than 0.01). This differential variability should be considered in interpreting sequential changes in left ventricular ejection fraction. To be attributed to nonrandom physiologic alterations, the absolute change in ejection fraction should be 10 percent or more in normal patients and 5 percent or more in abnormal patients.


Management of surgical complete atrioventricular block in children. Because there is disagreement concerning the efficacy of and indication for permanent pacemaker implantation in children with postoperative complete (third degree) atrioventricular (A-V) block, experience in the management of this problem at one institution was reviewed. Thirty-four patients with postoperative complete atrioventricular block were identified. They ranged in age from 4 months to 22 years and in weight from 4 to 60 kg and were evaluated from 1 month to 20 years postoperatively. Complete A-V block developed within 24 hours of operation in 28 of the 34 patients. A permanent pacemaker was implanted in 13 of the 28. Death occurred in 4 of these 13 patients and in 5 of the remaining 15 patients who did not have an artificial permanent pacemaker. Complete A-V block developed later than 1 day (2 days to 4 months) postoperatively in 6 of the 34 patients; all 6 of these patients survived, and only 3 required permanent pacemaker implantation. Intracardiac electrophysiologic studies were performed by 14 of the 34 patients. The site of complete block was above the His bundle in 5, within the His bundle in 2, and below the His bundle in 4; it was undetermined in 3. The results of intracardiac electrophysiologic studies are important in delineating the natural history of surgically induced complete A-V block and in the clinical management of this lesion. Permanent pacemaker implantation is indicated if complete A-V block persists longer than 2 weeks postoperatively and if the site of the block is within or below the bundle of His.


The fast-slow form of atrioventricular nodal reentrant tachycardia in children. An unusual form of atrioventricular (A-V) nodal reentry is described as the underlying mechanism for incessant tachycardia in two children. During tachycardia a fast pathway was utilized for anterograde conduction and a slow pathway for retrograde conduction. This is the reverse of the usual form of A-V nodal reentrant tachycardia, in which the slow pathway is utilized for anterograde conduction and the fast pathway for retrograde conduction. One patient had a smooth ventriculoatrial (V-A) conduction curve demonstrating exclusive utilization of the slow pathway for retrograde conduction. The other had a discontinuous V-A conduction curve demonstrating failure of retrograde fast pathway conduction with resultant slow pathway conduction. In both cases the retrograde effective refractory period of the fast pathway was longer than that of the slow pathway, resulting in the establishment of this unusual reentry circuit. Both patients had a superior P axis with a P-R interval shorter than the R-P interval during tachycardia, features described in a significant number of children with incessant tachycardia. This unusual form of reentrant tachycardia can be suggested by its electrocardiographic pattern and is another mechanism for reentrant tachycardia not previously documented in children.


Noninvasive visualization of coronary arterial aneurysm in infants and young children with mucocutaneous lymph node syndrome with two dimensional echocardiography. Real time two dimensional echocardiographic studies of the main coronary arteries were performed in 30 normal subjects and 58 patients with the mucocutaneous lymph node syndrome. Four echocardiographic coronary arterial patterns were observed: linear, dilated, fusiform or spherical. In normal subjects the left main coronary artery generally had a linear pattern but had a dilated pattern in two patients. The right coronary artery could not be visualized clearly. In 7 of the 58 patients with the mucocutaneous lymph node syndrome, a portion of the left main coronary artery had a fusiform or spherical pattern, or both, and the stem of the right coronary artery also had a spherical pattern in 2 patients. In all five patients who had coronary angiography, the procedure confirmed an aneurysm in the left main coronary artery or its branching point and the stem of the left anterior descending coronary artery or the stem of the right coronary artery, or both. In three of the five patients, coronary arterial aneurysms were predicted before coronary angiography was performed. Thus, two dimensional echocardiography is a reliable noninvasive method for evaluating coronary arterial aneurysms in infants and young children.


Sucking action of the left ventricle: demonstration of a physiologic principle by a gunshot wound penetrating only the right side of the heart. This report describes a man who died after a gunshot wound that entered the right atrium and exited from the right ventricle without entering the cardiac septa or the left side of the heart. At necropsy, the left atrial appendage was found to be inverted and invaginated into the mitral orifice. The invagination of the left atrial appendage is viewed as anatomic evidence that a negative left ventricular pressure was created as the left ventricular volume rapidly decreased as a result of right-sided cardiac exsanguination. Previously reported experiments in animals demonstrating the sucking (negative pressure) action of the left ventricle during ventricular diastole are summarized. The prerequisite for creation of a negative pressure in the ventricles during diastole is an extreme diminution in left ventricular volume, in this case as a result of right-sided cardiac bleeding. Only a vacuum effect of the left ventricle during diastole can explain the inversion and invagination of the left atrial appendage in this patient.


Fatal acute bacterial myocarditis after dentoalveolar abscess. A 19 year old woman presented with chest pain after a dental extraction for a dentoalveolar abscess. Electrocardiographic and serum isoenzyme changes were consistent with acute anterior myocardial infarction. At autopsy bacteria were demonstrated, within the myocardium in the absence of a myocardial abscess or endocarditis. This case illustrates the occurrence of isolated acute bacterial myocarditis after a dental extraction.


Comparative apparent digestibility of casein in holoxenic, axenic, and Clostridium bifermentans monoassociated rats. The aim of the present study was to determine the role of the intestinal microflora in the dietary utilization of casein. The apparent digestibility of dry matter, organic matter, energy, and nitrogen was measured in holoxenic, axenic, and Clostridium befermentans monoassociated rats deprived or not deprived of their pancreatic and bile secretions. The apparent digestibility of energy decreased in all the animals whatever the bacterial environment, after ligation of the common duct. Concerning the apparent digestibility of nitrogen, the intestinal microflora allowed the rats to compensate for the absence of the pancreatic proteolytic enzymes. It is suggested that a small part of casein, which was not hydrolyzed when the animals were deprived of their pancreatic and bile secretions, underwent a hydrolysis from the bacterial proteases.


Obesity, lipids, and glucose intolerance. The Framingham Study. Some lipid and lipoprotein accompaniments of obesity and its association with glucose intolerance are examined in the Framingham cohort of 5209 men and women ages 30 to 59 examined biennially over 18 years. While beta and pre-beta lipoproteins were positively correlated with relative weight, high density lipoprotein cholesterol was inversely correlated. The association was strongest for high density lipoprotein cholesterol, varying little by age and sex. Triglyceride was a close second, but unlike high density lipoproteins, it and other lipids were more closely associated with obesity in men than women and in younger than older persons. Obese persons tended to have a greater likelihood of glycosuria and an increasing prevalence of diabetes. Relative weight in the Framingham cohort rose in both sexes to age 54, remained essentially unchanged until age 62 and then began to decline. Despite such changes body weights even 18 years apart had a correlation of 0.8. Men from each succeeding birth cohort were heavier, women were lighter, but even women from the most recent birth cohort were much more frequently above "desirable" weight than below it.


II. In vitro restoration by levamisole of mitogen responsiveness in zinc-deprived rats. The effect of levamisole on the in vitro responses of splenic and peripheral blood lymphocytes to phytohemagglutinin were investigated in zinc-deprived and pair-fed control rats. The in vitro response of lymphocytes from zinc-deprived rats was markedly impaired in comparison with that of controls. The addition of levamisole to cultures of zinc-deprived splenic lymphocytes significantly improved their response to phytohemagglutinin by 54%. The addition of levamisole to cultures of zinc-deprived peripheral blood lymphocytes significantly improved their response by 116.2%. The addition of levamisole to control lymphocyte culture had no effect. This study may be the first to evaluate the beneficial effect of an immunomodulating agent on immune function in a nutritionally deprived host.


Changing patterns of dietary iron overload in black South Africans. Over the last 17 years there has been a significant reduction in the prevalence and severity of dietary iron overload in urban blacks of Johannesburg. This is attributed to a decrease in the consumption of traditionally brewed beer of high iron content over this period. A 40% reduction was found in hepatic iron concentrations measured in necropsy specimens from 248 male patients who died in 1976 as compared with 220 who died in 1959 and 1960. While hepatic iron concentrations rose with age in both groups there was no evidence of iron accumulation during the period between the two studies. Hepatic iron concentrations measured in 345 female subjects were many fold less than those of males and the 1976 group did not differ significantly from the 1959 to 1960 group. A paradoxical increase in the prevalence of portal fibrosis and cirrhosis was seen and may be due to the effects of increased ingestion of spirits and fortified wine in recent years. Iron overload was significantly greater in males with carcinoma of the esophagus and in those with idiopathic heart failure when compared to subjects who died of other causes. This suggests excessive exposure of these subjects to traditionally brewed beverages and the adulterants present in them.


Lactation and fertility.

Lactation and fertility. During the last decade there have been many reports indicating a decline in breast feeding practices in the urban areas of developing countries. This decline might have adverse effects on maternal and child health. A study of 1079 urban hospital attending women was undertaken to evaluate their breast feeding practices. Prolonged (mean duration of lactation 19.8 months) and successful lactation (failure of lactation occurred in only 3.5%) was common among this group. But a trend toward shorter duration of breast feeding was found among educationally and economically better off segment. There was very good correlation between mean duration of lactation, lactational amenorrhea, and interpregnancy interval. Therefore, it is possible that decrease in duration of lactation might result in shorter interpregnancy interval. The duration of lactation appeared to be "fixed" for each individual irrespective of age and parity. This in turn resulted in "fixed" duration of lactational amenorrhea and interpregnancy interval under conditions of unhindered lactation and uncontrolled fertility. Conception during lactational amenorrhea was low (7.7%) but once periods were reestablished lactation offered very little protection against conception.


Intakes and sources of dietary fiber in the British population. Intakes of dietary fiber and its different components have been measured in a random sample of the population in Cambridgeshire, England and compared with data from the British National Food Survey. Sixty-three men and women ages 20 to 80 were included in the sample. Total dietary fiber intake was 19.9 +/- 5.3 g/day compared with the calculated value of 19.7 g/day from the 1976 National Food Survey. There was a 4-fold range in fiber intake from 8 to 32 g/day; no significant trends with age or between men and women were detected. Vegetables supplied the majority of the fiber (41.3%); cereals 30.5%, and fruit and mixed sources 28.2%. Of the components of dietary fiber noncellulosic polysaccharide, cellulose, and lignin intakes were 13.8, 4.7, and 1.4 g/day, respectively. In the noncellulosic fraction, hexoses contributed 7.4 g, pentoses and uronic acids 3.3 and 3.0 g. Vegetables and unrefined cereals were the main sources of pentose. These intakes are low in comparison with limited international data from developing countries and of a similar order to those known in dietary experiments to produce low stool weights, slow transit time, and concentrated feces. They could readily be increased by simple dietary changes.


Nutrient intakes in an urbanized Micronesian population with a high diabetes prevalence. Dietary data was obtained from 77 adult Micronesian (Nauruan) subjects during a diabetes epidemiological survey. The life-style of this isolated Pacific population is almost completely Westernized and they have been shown to have a very high diabetes prevalence rate. Nearly all food consumed by the islanders is imported from Australia. The main caloric intake of both males and females was at least twice those recommended for Western or developing Pacific populations. The diet meets or exceeds the Australian National Health and Medical Research Council recommended allowance for calories, protein, fat, and ascorbic acid, but thiamin intake was inadequate. The percentage of fat in the diet was lower than that consumer by Western populations. Obesity is a pronounced feature of this population and the high caloric intake coupled with reduced physical activity would appear to be a major factor in relation to this. This Micronesian group has a genetic susceptibility to diabetes which may have been unmasked by the change from traditional to Western life-style. This problem may not be unique to Nauru and, with progressive Westernization, many other Polynesian and Micronesian populations may be at risk.


Opposite changes in aggregability of platelets stored in plasma and in blood. The effect of storage on the aggregability of platelets in plasma and in whole blood was studied with blood samples obtained from 11 normal subjects. Compared to aggregation of platelets in fresh samples, those stored in plasma showed an increase in aggregation, and in whole blood a decrease in aggregation. The decreased aggregation in the latter samples was prevented by including exogenous glucose in stored blood samples. Similar studies were performed on 25 patient platelet samples that had been judged as hyperaggregable by standard procedure, including the presence of "spontaneous" aggretation in 13 specimens. Only seven samples prepared from stored blood still showed hyperaggregability; spontaneous aggregation remained in only five samples.


Comparison of microchromatography and electrophoresis with elution for hemoglobin A2 (Hb A2) quantitation. Microcolumns prepared in the authors' laboratory, two commercial microchromatography kits, and electrophoresis with elution were compared for Hb A2 quantitation. Day-to-day imprecision of microchromatographic methods was similar (CV 4.7--6.6%) and somewhat less than electrophoresis with elution (CV 8.0--9.1%). Both commercial kits showed variable imprecision in different lots; one lot of Kit B gave erratic results due to resin leakage. From 49 patient specimens, Kit A microcolumns and those of the authors identified the same 14 patients with an elevated percentage of Hb A2 and showed good correlation (P = 0.90), although Kit A showed constant bias toward higher values. Electrophoresis with elution resulted in a false-positive and a false-negative value, did not correlate well with microcolumns (P = 0.78 and 0.76), and showed proportional bias toward lower values for an elevated percentage of Hb A2. Commercial kits were convenient, relatively quick, and cost-effective. Frozen, stabilized hemolysates performed well for quality control.


Heparin monitoring and thrombosis.

Heparin monitoring and thrombosis. The activated parial thromboplastin time is susceptible to changing concentrations of factor VII even in the presence of heparin. Heparin delays, but does not abolisn, thrombin generation, and this delay is obviated by factor VIII. It is not known if activated parial thromboplastin time shortened by increased factor VIII demands an increase in heparin administration.


Normal serum iron and elevated total iron-binding capacity in iron-deficiency states. Five nonanemic patients with normal erythrocytic indexes were found to have normal serum iron, elevated total iron-binding capacity, normal percentage of saturation of transferrin, and depleted bone marrow iron stores. These findings suggest that during the development of iron deficiency, an elevation of total iron-binding capacity occurs before the decrease of the serum iron. This may represent a compensatory mechanism to mobilize all traces of tissue iron to maintain normal erythropoiesis.


Ultrastructural study of human IgG and IgG-IgM crystalcryoglobulins. Thirty human cryoglobulin precipitates obtained from 21 patients were fixed and examined by electron microscopy; following biochemical isolation and identification. This study showed that the fine structure of cryoprecipitates depends on the involved immunoglobulins and on their respective quantities. Monoclonal IgG kappa 1 or 3 cryoglobulins with antibody activity form crystalling precipitates of 22-nm diameter rods and annuli. When polyclonal, they form 6-nm-wide filaments. Mixed IgG and IgM cryoprecipitates appear as cylindric and annular bodies with an internal diameter of 12 nm and a total diameter of 62 nm. When IgM predominates over IgG, globular condensations with a diameter of 30 nm are seen. Mixtures in which the IgG is more abundant than the IgM include fingerprint-like periodic condensations.


A computer system for cytology in a private laboratory using a PDP 8/E minicomputer. A computer software package written in Dibol for a PDP 8/E computer is described that is designed for the needs of the cytology department of a moderate-sized private medical laboratory. Computerized accessioning of cases, report preparation and printing, historical filing, activity summary reports, and direct patient and physician billing are provided by the relatively inexpensive system. The software has been in use for over two years in our cytology department and has resulted in increased efficiency, cost-effectiveness, and quality of reports.


Quantitation of serum tobramycin concentration using high-pressure liquid chromatography. A high-pressure liquid chromatography method for the quantitative determination of tobramycin in serum is described. The antibiotic was separated from serum by chromatography on a silica gel column. The adsorbed antibiotic was derivatized with o-phthalaldehyde, and then eluted with isopropanol. The derivatized tobramycin was separated by reverse-phase chromatography and quantitated by fluorometry. Serum concentrations as low as 0.5 microgram/ml could be accurately measured. A linear response for serum samples containing tobramycin ranging from 0 to 20 microgram/ml was obtained. Other antibiotics, including various aminoglycosides, did not interfere with the tobramycin assay. Comparison with a standard microbiologic assay gave a correlation coefficient of 0.99. This chemical assay is sensitive, precise, specific, and can be performed in 30 minutes.


Simple modification of the cellulose acetate method for identifying adult hemoglobin (hemoglobin A) in dried specimens of newborn blood. Definition of hemoglobin A in cellulose acetate electrophoretograms of eluate from air-dried specimens of newborns' blood was improved by adding glycerine to the Tris--ethylenediametetraacetic acid--borate buffer system. Examination of 1,672 randomly received samples showed no reduction in ability to detect hemoglobin S with the modified buffer. The method may be adopted as a useful adjunct to current technics for rapid electrophoretic screening of neonatal dried blood samples.


Serum glycine-conjugated bile acids in pediatric hepatobiliary disorders. Measurements of serum bile acids (glycine conjugates of cholic, chenodeoxycholic, deoxycholic, and lithocholic acids) by radioimmunoassay in a variety of pediatric hepatobiliary disorders showed elevations in neonatal hepatitis syndromes, cholestasis, and hepatitis of extrahepatic or intrahepatic origin. Measurements of individual serum bile acids failed to differentiate between the various neonatal hepatitis syndromes. In one patient with cholestasis, the increased levels of bile acids observed returned to normal following therapy with cholestyramine and phenobarbital. In chronic active hepatitis the serum bile acid values correlated well with the bilirubin and SGOT in response to therapy with corticosteroids. These data confirm suggestions that serum cholylglycine and chenodeoxycholylglycine levels are a sensitive indicator of disturbed hepatic function and can be used in monitoring the course, activity, and therapeutic response in various hepatitis syndromes. In Reye's syndrome and protracted diarrhea of infancy, elevations in serum bile acids were detected without associated hyperbilirubinemia and provided additional evidence of disturbed hepatic function.


Usefulness of macroscopic urinalysis as a screening procedure. A preliminary report. The macroscopic examination (physicochemical testing) is a sufficient laboratory screening procedure for routine urinalyses when accurately assessed. Only in those patients for whom routine urine specimens are macroscopically positive or in symptomatic patients with or without known renal or urinary-tract disease should a microscopic examination be necessary. Results of this study of more than 900 consecutive routinely screened urine specimens indicate that there is less than a 3% diagnostic yield when the urine sediment is examined after a negative macroscopic examination.


A program for residency training in clinical pathology. The goals, organization, components, instruction approaches, and the continuing assessment of a clinical pathology residency program are described. This program emphasizes training by rotation in the services of the clinical laboratory, application and interpretation of laboratory data for patient care, laboratory management, teaching conferences, and research and development.


An intrinsic coagulation pathway inhibitor in a 3-year-old child. An intrinsic coagulation pathway inhibitor with acitivty against factor XI was detected in the plasma of a 3-year-old child, following a respiratory infection. The inhibitor was present transiently, but it was clinically significant and was manifiested by extensive bruises. Abnormalities of clotting and the bruising tendency disappeared simultaneously without treatment before the inhibitor could be definitively characterized. It was possibly the consequence of an adenovirus infection, as an elevated adenovirus titer was demonstrated in the patient's blood. It is possible that inhibitors of this type are more common sequelae of viral infections than realized, and perhaps a systematic effort should be made to detect them and to define their etiology and mechanism of action.


In-vitro synthesis of an anti-BI cold agglutinin complicating a case of lymphoma. A woman of 47 with lymphocytic lymphoma was found to have a high-titer cold autoagglutinin of anit-BI specificity. Her group B erythrocytes autoagglutinated of anti-BI specificity. Her group B erythrocytes autoagglutinated in vitro and the direct antiglobulin reaction was positive, but she had no symptom of cold intolerance, no evidence of hemolysis, and she could receive transfusions of compatible group O erythrocytes. In addition, evidence for synthesis of the autoantibody by the lymphoma cells was obtained by short-term bone-marrow culture.


Wound infection associated with Vibrio alginolyticus. A 42-year-old man had an infected ulcerated lesion of the anterior aspect of the right lower extremity. Vibrio alginolyticus was twice isolated from the lesion. Emphasis is placed on the clinical and laboratory characteristics of this organism and its differentiation from other halophilic vibrios.


Primary ovarian failure, juvenile rheumatoid arthritis, and vitiligo. A female adolescent with primary ovarian failure was found to have circulating autoantibodies to her ovaries. Primary ovarian failure in pediatrics is usually attributed to gonadal dysgenesis. Autoimmune oophoritis is a rare cause and is most often associated with Addison's disease and other autoimmune endocrinopathies. Our patient also had juvenile rheumatoid arthritis and vitiligo. To our knowledge, this case represents the first reported instance of this particular constellation of autoimmune disorders.


Pneumococcal sepsis and pneumonia in the neonate. Two neonates had early onset Diplococcus pneumoniae septicemia and pneumonia. Both recovered after intensive respiratory and antibiotic therapy, although a review of the literature indicates that early pneumococcal septicemia has been associated with an extremely high mortality. Pneumococcal septicemia in a one-year period accounted for 12% of all documented neonatal bacteremias. This may indicate an increasing incidence of this organism in newborns.


Acute Haemophilus pneumonia in childhood. In our hospital Haemophilus influenzae type B seems to be a common cause of acute childhood pneumonia. In the past five years, 34 children with acute Haemophilus pneumonia were identified. Although these children generally had an uncomplicated segmental pneumonia associated with a bacteremia, 13 of the children had pneumonia with a pleural effusion. These children with Haemophilus pneumonia represented 18% of the children hospitalized with systemic Haemophilus disease and almost a third of those hospitalized with acute bacterial pneumonia from whom the causal agent was isolated.


Prosthetic valve endocarditis due to Haemophilus parainfluenzae biotype II. Haemophilus parainfluenzae endocarditis is characterized by great variation in the acuteness of presentation, difficulty in isolation of the pathogen, a 50% to 60% incidence of major arterial emboli, and variability of response to therapy. Prosthetic valve endocarditis (PVE) due to H parainfluenzae biotype II occurred in a 14-year-old girl with congenital heart disease and a Starr-Edwards mitral valve prosthesis. Management was complicated by a prolonged culture-negative period (eight days), intermittent bacteremia (only five of 15 positive blood cultures), an embolus to the right femoral artery, progressive congestive heart failure, and urgent prosthestic valve replacement. Cure was achieved with 44 days of ampicillin sodium-gentamicin sulfate therapy monitored by serum bactericidal titers.


Phototherapy and plasma immunoreactive prostaglandin A values. Its effect in premature infants. Plasma immunoreactive prostaglandin A (iPGA) values were determined for 14 premature infants before and after 48 hours of phototherapy and in a control group of six age-matched premature infants. At 4 to 8 days of life, the infants who had received phototherapy for 48 hours had significantly lower iPGA values compared with controls. At 2 to 3 days of life, four infants with a clinically apparent patent ductus arteriosus were found to have significantly elevated iPGA values compared with controls. After 48 hours of phototherapy, these infants likewise had a significant decrease in iPGA values compared with controls; in all four infants, the ductus closed spontaneously. Phototherapy is an effective method for decreasing plasma iPGA values in premature infants.


Demineralization in cystic fibrosis detected by direct photon absorptiometry. Bone mineral content, bone width, and their ratio were measured in patients with cystic fibrosis (CF) using monoenergetic photon absorptiometry. Serial measurements of the radius and ulna were made in 27 patients with CF and were compared with 968 age-matched controls. Demineralization was found in 37% of the boys and 63% of the girls. Patients under age 10 years had normal bone mineral content and nine of 15 patients aged 13 or older were demineralized (P less than .01). Demineralization correlated with the extent of weight reduction in patients (P less than .001). Patients most likely to be demineralized were adolescent girls. To our knowledge, this is the first report of bone mineral status of children with CF, and the results indicate that a sizable proportion of these patients may be demineralized without overt rickets.


Gallium-67-citrate scanning in tuberculous peritonitis. A 32-year old black, alcoholic male was noted to have an abnormal Gallium-67-citrate scan during a work-up for persistent fever. Surgical biopsies revealed findings of the dry form of tuberculosis peritonitis. The literature concerning Gallium-67-citrate scanning was reviewed and its usefulness in inflammatory disease states (including two reports of positive scans in tuberculous peritonitis) was demonstrated. In addition, we have considered the possible mechanisms of Gallium-67-citrate uptake by inflammatory cells and its probable relationship to the granulomas associated with tuberculous peritonitis. We conclude that Gallium-67-citrate scanning is an important diagnostic procedure in the evaluation of a fever of unknown origin. Moreover, it may significantly shorten the diagnostic work-up of those cases of tuberculous peritonitis.


Endoscopic, histological and ultrastructural correlations in chronic gastritis. Thirty-three patients presenting with dyspepsia were examined with the Olympus Fiber-gastroscope. An endoscopic diagnosis was made in each case and multiple gastric biopsies were obtained for histological and ultrastructural assessment. The endoscopic, histological and ultrastructural findings were compared with each other. Of 33 patients endoscoped, 29 were found to have endoscopic evidence of various types of gastritis and all of these latter had histological evidence of some form of gastritis. Histological confirmation of specific types of endoscopically diagnosed gastritis, however, was only found in 3/9 cases of chronic atrophic gastritis (CAG), 10/14 cases of chronic (superficial) gastritis (CG) and in none of six cases of acute gastritis (AG), indicating that endoscopic diagnosis of specific types of gastritis is relatively inaccurate. Endoscopic diagnosis should thus be restricted to presence or absence of gastritis, leaving the specific typing to histological assessment of the gastric biopsies. Multiple gastric biopsies should be obtained even though the gastric mucosa appears normal endoscopically, since histological evidence of gastritis was found in three out of four cases with endoscopically normal gastric mucosa. Comparison of histological diagnosis with electron microscopy showed that generally there is good correlation between the severity of the histological changes and the ultrastructural grade of damage as defined in this study. It would appear that E.M. examination of the gastric biopsies will not significantly increase the diagnostic accuracy of light microscopy, although it has elucidated the various cellular changes which characterize chronic gastritis. The rough surface contours, the large gastric pit and the increasing number of surface microvilli, seen by scanning E.M., aid the grading of the disease process. Some of these changes are reflected in observations made by transmission E.M. where in addition the basal intercellular edema of the mucosal layer and the appearance of electron-dense mucosal lining cells are observed. The latter, which are the counterpart of "intestinal metaplasia", possess mucus granules which resemble those of gastric mucosal lining cells or intestinal goblet cells, or both.


Gallstone-linked tubular stenosis of the common bile duct. The transpancreatic segment of the distal common bile duct can be extrinsically stenosed by a pure gallstone-linked pancreatic disorder. It has a specific roentgen configuration--long, tubular and smooth. In two instances it was associated with severe suppurative complications. Precholedochotomy cholangiography is required to identify such long segment benign tubular stenosis of the distal common bile duct as an indication for management alternatives and as a guide to judicious common bile duct exploration.


Hyperhaptoglobinemia and carcinoma of the gallbladder. Cancer of the gallbladder is an uncommon malignancy which is seldom diagnosed early and often denotes a poor prognosis. Two reported cases describe the association of hyperhaptoglobinemia (greater than 700 mg./dl.) and advanced carcinoma of the gallbladder. When a patient presents with liver involvement from metastatic adenocarcinoma of suspected gastrointestinal origin and a markedly elevated serum haptoglobin (greater than 700 mg./dl.), it is suggested that a high index of suspicion be held for carcinoma of the gallbladder.


Meat bolus impaction of the lower esophagus associated with a paraesophageal hernia. Successful noninvasive treatment with intravenous glucagon. Ferrucci and Long recently reported the relief of esophageal food impaction with the use of intravenous glucagon in three patients. Our first experience in using this new procedure was very successful and gratifying. The essence and purpose of this brief communication is to record and further substantiate the efficacy of a new treatment modality.


Stricture of the esophagogastric junction with adjacent extraluminal cavity following radiation. We report a radiation stricture of the esophagogastric junction with an adjacent extraluminal cavity formed secondary to radiation in a patient receiving 4,500 rads for a histiocytic lymphoma of the stomach. We suggest that the presence of a radiosensitive lymphoma may have undermined the normal reparative response to radiation of the esophagogastric junction and predisposed it to stricture and cavity formation at this dose of radiation.


Intramural hematoma of the gastric cardia. A case of intramural hematoma of the gastric cardia occurring in a young woman with S.L.E. and on warfarin is presented and discussed. This unusual case illustrates the importance of careful evaluation of radiological mass lesions of the cardia.


Surgical treatment for pneumatosis cystoides intestinalis complicating jejunoileal by-pass. A case of chronic pneumatosis cystoides intestinalis, complicating jejunoileal by-pass procedure for morbid obesity, is described. Grave clinical symptoms associated with irreversible segmental mucosal atrophy and mural fibrosis resulting from untreated long-standing pneumatosis intestinalis, required resection of the whole by-passed small intestine. It is suggested that this simple and safe surgical procedure is justified in selected cases. Conservative treatment of asymptomatic patients with pneumatosis intestinalis, however, may prevent further surgical intervention in these patients.


Emphysematous cholecystitis.

Emphysematous cholecystitis. The radiographic spectrum of emphysematous cholecystitis can be divided into three stages. Diagnosis on plain abdominal film is difficult in the first stage. Two cases are presented in which a circular gas filled structure in the right upper quadrant led to a correct diagnosis in the earliest stage. This was confirmed by obtaining contrast studies to rule out other etiologies.


Relationship of relevant factors of atherosclerosis to menopause in Japanese women. The relationship of menopause to associated factors of atherosclerosis was investigated during October 1973 to December 1976. The subjects were 1674 premenopausal and 428 postmenopausal women aged 35 to 54, who were living in Toda city, an urban area of Japan. Serum cholesterol and triglyceride were higher in postmenopausal women than in premenopausal women. This trend was also observed in serum uric acid and blood hemoglobin. On the other hand, systolic and diastolic blood pressure and relative body weight had no consistent relationship to menopause.


Exogenous hormones and other drug exposures of children with congenital heart disease. A history of oral contraceptive use, hormonal pregnancy tests, prescribed hormones and other drugs was obtained from 390 mothers of infants with congenital heart disease and 1254 mothers of normal infants in Massachusetts. The data show a small positive association between estrogen/progesterone exposure and cardiac malformation, the prevalence ratio estimate of exposed to non-exposed being 1.5 (90 per cent confidence limits are 1.0, 2.1). No association was evident, however, between hormones and trunco-conal or any other class of defect among the cases, an observation which casts doubt on a causal relationship betweem hormones and cardiovascular malformations. Several other drugs were reported more frequently by cases' mothers. These include: ampicillin; aspirin; a combined anti-nausea agent (doxylamine succinate, dicyclomine hydrochloride and pyridoxine hydrochloride); chlordiazopoxide, codeine, diazepam, diphenylhydantoin; insulin; phenobarbital; phenothiazine; phenylephrine; and tetracycline.


Childhood cultural experience and the incidence of coronary heart disease in Hawaii Japanese men. Possible effects of cultural variables on the risk of coronary heart disease (CHD) were examined in 7705 men of Japanese ancestry living in Hawaii. The six-year incidence of CHD was related to birthplace (Japan or elsewhere), number of years spent in Japan, ability to read and write Japanese, ability to speak Japanese, and to an index of preference for a traditional Japanese diet. Statistically significant inverse relationships were found between CHD incidence and all of these variables which reflect the degree of exposure to Japanese culture during childhood. In multivariate analyses where the major CHD risk factors were taken into account, years spent in Japan and ability to read and write Japanese remained significant. Hence, exposure to Japanese culture during childhood appears to protect against CHD in adulthood. This may explain, in part, the gradient of CHD frequency among Japanese in Japan, Japanese-Americans, and Caucasians that could not be entirely attributed to the established risk factors.


Premature mortality attributable to smoking and hazardous drinking in Canada. All causes of death related to the two risk factors, smoking and hazardous drinking, have been reviewed followed by a selection of those causes of death for which the causal role of the risk factor appears to be quasi-certain. For each cause, existing epidemiologic data were reviewed and used to determine the fraction of premature mortality which could be attributed to each factor (called the attributable fraction). This fraction was then multiplied by the corresponding Canadian premature mortality measured in terms of deaths between ages one and 70 and potential years of life lost (PYLL) between ages one and 70, which gives a higher weight to younger deaths. Of the 73,440 deaths between ages one and 70 in Canada in 1974, 12% (or 8718 deaths) were found to be attributable to current smoking and 6% (4716) to hazardous drinking. In terms of PYLL between ages one and 70, hazardous drinking ranks ahead of current smoking with 10% (or 132,044 PYLL) of the total PYLL, whereas current smoking represents 8% (105,085 PYLL) of the total . Regardless of whether premature mortality is expressed in terms of deaths or PYLL, about 18% of Canadian premature mortality is attributable to current smoking and/or drinking (with the range of possible values being 14-22%).


Influenza A and B virus infection in infants and young children during the years 1957-1976. Influenza A virus activity was demonstrated in infants and young children from metropolitan Washington, DC during each of 19 successive August-July respiratory disease years, and during 17 of these years at least 2% of hospitalized respiratory disease patients yielded an influenza A or B virus and/or showed an influenza A or B serum complement-fixing (CF) antibody response. Between October 1957 and July 1976, 14.3% of 860 croup patients and 5.3% of a total of 5655 hospitalized respiratory patients, including croup patients, showed evidence of influenza A or B infection. The mean period of hospitalization with either virus was about 8 days, though serious infection with influenza A virus was 4.5 times more common than with influenza B virus. Both influenza viruses were detected more frequently in respiratory disease outpatients than in respiratory disease inpatients. Patients with serious influenza A virus infections were especially likely to have croup (particularly during the H3N2 era), to be seen during December through February, and to be black male infants. During the peak month of a composite of 13 consecutive influenza A virus outbreaks, influenza A virus infection was demonstrated in 67.6% of croup patients and in 35.6% of all hospitalized respiratory patients including croup patients. During the peak month of a composite of 6 consecutive influenza B virus outbreaks, influenza B virus infection was demonstrated in 36.0% of croup patients and in 10.8% of all hospitalized respiratory disease patients including croup patients.


Histoplasmosis in South Carolina: support for the microfocus concept. An outbreak of anthropurgic histoplasmosis occurred in rural South Carolina when a canebrake which served as a blackbird roost was cleared with a bulldozer. Seven days later the index patient became ill with histoplasmosis. Nine other patients contracted the disease, but only the index case was fatal. Serologic studies on the 10 patients demonstrated high titers in the histoplasmin latex agglutination test within two to three weeks after exposure. Serologic titers to the yeast-form Histoplasma capsulatum antigen in the complement fixation test appeared most frequently, but these antibodies were not diagnostic because complement-fixing antibodies to Blastomyces dermatitidis appeared at the same time. Soil studies demonstrated that viable particles of H. capsulatum persisted at declining levels over a nine-year period. A skin test survey showed that 27.3% of eighth grade students who resided within a 20 km radius of the contaminated site and who were lifelong residents of the same dwelling had a positive histoplasmin skin test.


Differences in the clinical manifestations of sicca syndrome in the presence and absence of rheumatoid arthritis. In this restrospective study we review the clinical features of patients with the sicca syndrome in the presence and absence of rheumatoid arthritis. All patients were followed at the National Institutes of Health for at least five years after the diagnosis of sicca syndrome was established. Twenty-two patients had sicca syndrome alone, and 21 patients had definite rheumatoid arthritis and the sicca syndrome. Rheumatoid arthritis tended to precede the developement of sicca syndrome. The mean age at diagnosis of sicca syndrome is the same in both groups. No significant differences in serum innumoglobulins, the third component of complement (C3), rheumatoid factor titer and salivary histopathology were found. However, the clinical features were quite distinct. Patients with sicca syndrome alone had a significantly greater frequency of recurrent parotitis, Raynaud's phenomenon, purpura, lymphadenopathy, myositis and renal involvement. The clinical characteristics of these two groups coupled with the known serologic and genetic differences suggest that sicca syndrome alone is a distinct pathologic entity.


Hermansky-Pudlak syndrome. Pulmonary manifestations of a ceroid storage disorder. The Hermansky-Pudlak syndrome is a form of oculocutaneous albinism, characterized by a qualitative platelet defect and deposition of ceroid-like material throughout the reticuloendothelial system. During a 16 month period five patients with Hermansky-Pudlak syndrome presented with symptoms, chest films and pulmonary function studies consistent with restrictive pulmonary disease. In two patients, lung biopsies revealed diffuse interstitial fibrosis. However, light and electron microscopy demonstrated ceroid-like material within alveolar macrophages. In addition, two patients presented with inflammatory bowel disease with deposition of ceroid-like material in the colon. This disorder appears to be more common than is currently recognized and should be considered in the differential diagnosis of diffuse interstitial pulmonary disease and inflammatory bowel disease. A relationship between the deposition of ceroid-like material and pulmonary fibrosis is discussed in light of recent research concerning inflammatory processes. In view of the serious pulmonary, gastrointestinal and hematologic consequences of this syndrome, there is a need for genetic counseling of these patients.


In vitro leukocyte thymidine uptake and prognosis in chronic lymphocytic leukemia. Among 60 patients with chronic lymphocytic leukemia, higher in vitro uptake of tritiated (3H) thymidine by leukocytes of a standard volume of peripheral blood was associated with a higher lymphocyte count, a more advanced stage, greater frequency of functional impairment and shorter survival. Appropriate analyses demonstrated that leukocyte thymidine uptake correlated with survival independently of these other disease features. Relative thymidine uptake (radioactivity per 10(3) lymphocytes) did not prove to be a useful prognostic parameter. Among 33 patients not receiving antileukemic therapy at the time of study, 15 of 17 (88 per cent) of those with higher thymidine uptake values, but only 3 of 16 (19 per cent) of those with lower values, were treated during a median follow-up period of four and a half years (p less than 0.001). Seven of the former group, but none of the latter group, died during the first three years of follow-up (p less than 0.01). We conclude that thymidine uptake by circulating leukocytes constitutes a relatively accurate index of the proliferating leukemic cell mass in this disease and provides useful prognostic information.


Spatial reconstruction of human femoral atheromas showing regression. Reports of atherosclerotic changes in human subjects previously described have been based on evaluation of arteriographic edge contours. They imply unchanged roentgenographic and physiologic conditions, including identical patient positioning and vascular tone which cannot always be obtained in sequential studies. We have previously described the development of quantitative angiographic densitometry which permits measurement of vascular cross-sectional chord length distributions and areas, independent of rotational changes in vascular position. In this paper we report on application of the method to sequential femoral angiograms in two patients in whom there were significant increases in vascular cross-sectional area after a program of exercise and risk reduction. The method excludes interpretive errors due to circumferential changes in vascular tone.


Plasma levels and hepatic extraction of renin and aldosterone in alcoholic liver disease. Arterial plasma levels and hepatic extraction of renin and aldosterone (ALDO) were measured in 24 patients with alcoholic liver disease and in 14 normal subjects being evaluated as prospective kidney donors. Patients with liver disease had higher plasma concentrations and lower fractional hepatic extractions of both renin and ALDO than the normal subjects. The quantity of renin extracted by the liver was highly correlated with plasma renin in both normal subjects and patients. Plasma ALDO concentration was positively correlated with plasma renin (p less than 0.001) but not with serum sodium, potassium or albumin concentration, inferior vena cava pressure, corrected hepatic venous wedge pressure, plasma volume or sulfobromophthalein storage or transport. Sixteen patients were restudied after one month. Six had received 40 mg/day of prednisolone, and the remaining 10 had received a placebo. Neither group had a change in plasma volume, corrected hepatic venous wedge pressure, plasma concentration or hepatic extraction of renin or ALDO. Serum albumin concentration increased and inferior vena cava pressure decreased with prednisolone therapy. These studies document high plasma levels and impaired hepatic extraction of renin and ALDO in patients with liver disease that are not corrected by short-term prednisolone therapy.


Multiple floppy valves: an echocardiographic syndrome. Echocardiograms of 400 patients with mitral valve prolapse examined at the Peter Bent Brigham Hospital between 1974 and 1977 were reviewed. Eleven patients (3 per cent) were found to have prolapse (10 patients) or large excursion of the tricuspid valve (one patient) and large excursion of the aotric valve (four patients) or dilatation of the aotric root (seven patients) in addition to mitral valve prolapse. Two of these 11 patients underwent mitral valve replacement, and myxomatous degeneration of the valves was noted on pathologic examination. Almost half of the patients with multiple floppy valves (five of 11) had symptoms of congestive heart failure. In contrast to reported series of isolated mitral valve prolapse, in which female preponderance has been documented, 10 of the 11 patients were male. The syndrome of multiple floppy valves may represent either a unique entity or a more advanced form of the same process which underlies mitral valve prolapse.


Clinical use of a calcium antagonistic agent (nifedipine) in acute pulmonary edema. Nifedipine induces vascular smooth muscle relaxation through a calcium antagonistic action. The possibility of clinical use of the drug as a ventricular unloading agent has been explored in this study. In patients with hypertensive (seven cases), primary (seven cases) or rheumatic (aortic insufficiency five cases, mitral regurgitation five cases) cardiac disease, nifedipine, administered in a single sublingual dose (10 mg), relieved acute pulmonary edema. Circulatory variations from control were the following: decrease of systemic and pulmonary arterial pressures, and of vascular resistances, of pulmonary wedge pressure, of left ventricular diastolic and systolic dimensions (echocardiography); increase of cardiac and stroke index, of left ventricular mean rate of circumferential fiber shortening, of left and right mean pre-ejection delta P/delta t and mean rate of ejection; improvement of forward output in primary and rheumatic disease. Nifedipine benefits acute congestive heart failure by sustained fall of both preload and afterload and, possibly, by an enhanced contractility. It seems to have an appropriate indication in cases in which left ventricular afterload reduction is desirable.


Acquired agranulocytosis with granulocyte specific cytotoxic autoantibody. Multiple infections and severe neutropenia were found in a previously healthy 29 year old man with no history of similar syndromes in the family, drug ingestion or exposure to environmental toxins. There was no evidence at the time of presentation of diseases previously associated with agranulocytosis (e.g., neoplasia, thyrotoxicosis, chronic infection, collagen-vascular disease or leukoagglutinating antibody). His serum contained a nonagglutinating, complement-dependent, cytotoxic antibody, however, reactive with peripheral blood granulocytes from 35 per cent of normal donors. The neutropenia was not affected by steroids but resolved promptly after splenectomy. Microscopic examination of the spleen revealed ingestion of polymorphonuclear leukocytes by splenic macrophages. Family studies indicated that the target antigen was non-HLA and that the antibody was not absorbed by lymphocytes or platelets. We conclude that the agranulocytosis was autoimmune in origin and suggest that similar myeloid-specific immune responses could influence granulocyte tranfusion and bone marrow transplantation by alloimmune "rejection" that would not be avoided by matching only for HLA specificities.


Pulmonary infarction in sickle cell trait. A young black man presented with unexplained pleuritic chest pain. A hematologic evaluation revealed sickle cell trait, Chest roentgenograms, ventilation/perfusion lung scanning and a pleural-parenchymal lung biopsy documented pulmonary infarction. Sickle cell trait with resultant pulmonary infarction should be considered in black subjects with unexplained pulmonary diseases.


Fibrosing alveolitis and hepatitis B surface antigen-associated chronic active hepatitis in a patient with immunoglobulin A deficiency. Fibrosing alveolitis is described in a 22 year old woman with immunoglobulin A (IgA) deficiency and hepatitis B surface antigen (HBsAg)-associated chronic active hepatitis. At lung biopsy HBsAg was detected by indirect immunofluorescence in the alveolar space but not in the septal fibrosis. We discuss the possible relationships between IgA deficiency on the one hand, and HBsAg-associated lung and liver diseases on the other hand.


Multiple adenocarcinomas and premalignant changes in "backwash" ileitis. In a patient with long-staning ulcerative colitis and "backwash" ileitis, multiple carcinomas developed in the colon and ileum. In both locations premalignant mucosal changes of the basal cell proliferation type were seen adjacent to and remote from sites of carcinoma. Although the frequency of such premalignant and malignant changes in "backwash" ileitis is unknown, their concurrence in this case suggests that ulcerative colitis involving the terminal ileum increases the risk of small bowel carcinoma.


Diffuse pulmonary hemorrhage and rapidly progressive renal failure. An uncommon presentation of Wegener's granulomatosis. A 57 year old woman presented with rapidly progressive renal failure and diffuse pulmonary hemorrhage and life-threatening respiratory failure promptly developed; these conditions resolved after bilateral nephrectomy. Renal pathology revealed rapidly progressive glomerulonephritis and vasculitis with granular deposition of immunoglobulin on immunofluorescent staining. One year later, multiple nodular cavitating pulmonary infilrates developed, and lung biopsy was diagnostic of Wegener's granulomatosis. Therapy with cyclophosphamide resulted in resolution of the pulmonary lesions. Diffuse pulmonary hemorrhage and rapidly developing renal failure mimicking Goodpasture's syndrome was the initial manifestation of Wegener's granulomatosis in this patient.


Establishing criteria for competency-based education. Requisite to the development of competency-based curricula in the medical laboratory sciences is the translation of competency statements into terminal performance objectives so that the achievement of career-entry competence can be measured. To truly represent competence in practice, the conditions and standards of acceptable performance for the objectives must be those expected of career-entry practitioners on their jobs. A method is presented for the establishment of conditions under which the behaviors specified in competency statements are carried out in the "real world" of medical laboratory practice and the verification of standards of accuracy and speed in performance that represent competence at career entry. The career-entry practitioner is defined as the graduate of a medical laboratory education program who has been working in the field for six months to one year. The specification of that target group for verification of career-entry competence is explained. The necessity for use of the clinical laboratory for evaluation of career-entry competence of students prior to graduation through acheivement of terminal performance objectives is stressed.


Leak detection and low level monitoring of halogenated anesthetics. Halogenated compounds used in anesthetics are generally detected and monitored by gas chromatography or infrared analysis. For a quick, reliable, and economical method of detecting these compounds, the HLD-440 leak detector proved to be effective. The instrument is simple to calibrate and operate. It is safe and is capable of detecting halogenated anesthetics in concentrations at least as low as 2 parts per million.


Cognitive modifiability in retarded adolescents: effects of instrumental enrichment. The Instrumental Enrichment program, designed to modify the cognitive structures of retarded, disadvantaged adolescents, was contrasted with a General Enrichment program both in a residential setting and a day center. This study is based on 57 matched pairs drawn from the total research sample of 218 adolescents. One group in a residential center (n = 24) and one group in a day center (n = 33) received Instrumental Enrichment; one group in a residential center (n = 24) and one group in a day center (n = 33) received General Enrichment. The Instrumental Enrichment program extended over 2 years, and the subjects were pre- and posttested on a battery of criterion measures: general and specific cognitive tests, scholastic achievement, classroom interaction, and self-concept. The results indicated significantly better performance by the groups receiving Instrumental Enrichment than by the groups receiving General Enrichment on the tests of specific cognitive functions and scholastic achievement and on some of the classroom interaction scales. Results for the residential groups compared with the day-center groups revealed significant differences on the tests of scholastic achievement, in favor of the residential groups and on the classroom interaction scales in favor of the day-center groups.


Hierarchical assessment of social competence. Three properties associated with the Test of the Hierarchy of INductive Knowledge (THINK) were examined, namely, that the processes assessed by the measure are (a) cumulative and hierarchical, (b) developmental, and (c) related to behavioral criteria. Analyses of data collected from 120 EMR children between the ages of 9 and 14 years generally support the developmental and criterion-related features of the measure, but only partially confirm the hierarchical component. Discussion of the results was focused on the emphasis necessary in subsequent empirical work and the measure's potential for instructional applications in relation to current assessment issues in special education.


Measuring the use of a principle by retarded adolescents and nonretarded children on a Redundancy Series Test. A series test composed of geometric figures placed in redundant patterns was designed to measure the ability of retarded adolescents to use of principle. It was administered to institutionalized and noninstitutionalized retarded adolescents and to nonretarded children from nursery school through fourth grade. Results revealed that four of the items accounted for most of the errors and that these four critical items were particularly sensitive to an inadequate scanning strategy. The retarded groups showed a mental age lag at least 1 year, 6 months and did not perform above chance on the critical items.


Gaze patterns of mentally retarded adults in two contrasting environments. Two matched samples of mentally retarded adults were video recorded on two occasions, before and after one group was transferred to a different type of hospital, and their gaze patterns analyzed. Overall, only slight changes after transfer were found, but analysis according to amount of looking at the interviewer on the first occasion showed that those transferred subjects who initially looked little of the time significantly increased their amount of looking after transfer. Some correlations with the Adaptive Behavior Scale were also reported.


Speed of retrieving information from the lexicon of mentally retarded adolescents. Thirty-six institutionalized retarded adolescents named 48 colored pictures of objects. Winters, Winter, and Burger (1978) had previously established that for nonretarded adults the estimated age-of-acquisition of the names of these 48 objects ranged from 2 years, 6 months to 7 years of age. We found that pictures whose names had been estimated by nonretarded adults to have been acquired earlier were named more accurately and faster by retarded adolescents than were pictures whose names were estimated to have been acquired later. The implications of these results were discussed.


Affiliation and friendship of mentally retarded residents in group homes. The social behavior of 208 mentally retarded residents in 18 group homes was observed and analyzed in terms of their (a) informal grouping behavior, (b) affiliation, and (c) intense social relationships ("friendships"). Moderately to mildly retarded residents engaged in "social" types of behavior in groups (primarily dyads) and "neutral" types of behavior when alone. In contrast, profoundly retarded residents did not behave differently when others were present, although they did display social types of behavior. Residents' affiliative behavior was not as related to personal characteristics (sex and intelligence) as it was to (a) size of the group home, (b) average intelligence of the residents in the home, (c) sex ratio in the home, and (d) homogeneity of the residents' backgrounds. Although residents in large homes affiliated more extensively with others, intense relationships were as likely in large as in small homes. Sixteen intense friendships were identified and described. In total, the results support the view that the group home environment can influence residents' affiliation and friendship.


Telephone-skills training for retarded adults: group and individual demonstrations with and without verbal instruction. Effects of telephone-skills training delivered to institutionalized retarded adults in group and individual demonstrations, both with and without verbal instructions, were examined. The trials effect was significant for four telephone tasks and for the sum of the four tasks. Persons in all conditions, including the practice control condition, significantly improved skill performance. These findings indicate that, for skill development tasks with retarded adults, demonstration and verbal summaries may add little to the practice experience and that group delivery of training procedures appears to be cost-effective.


An analysis of overcorrection movements. Overcorrection procedures were designed for three profoundly retarded adults such that the overcorrection activities were topographically dissimilar to (i.e., did not physically prevent) the stereotyped behavior targeted for treatment. Using reversal designs, we recorded data on both the target behavior and the behavior used as the overcorrection responses. Results showed that overcorrection was effective in reducing the stereotyped behavior for each subject; however, the rates of the overcorrection responses either remained variable or decreased during the treatment phases. These findings suggest that although overcorrection is a valuable technique in reducing behavior, the overcorrection responses (e.g., restitutional acts, positive practice, functional movement training) may not increase in the absence of specific reinforcement contingencies. The importance of utilizing differential reinforcement procedures, especially with mentally retarded persons, in conjunction with ocercorrection was discussed.


Accumulation of and release from proactive inhibition in short-term memory by retarded and nonretarded persons. One group of EMR adolescents and adults and three groups of nonretarded children and adolescents were compared during accumulation of and release from proactive inhibition. All groups demonstrated the effects of and release from proactive inhibition. Analyses of intralist intrusions indicated that these errors did not differentially affect performance by any group during the accumulation of proactive inhibition and did not occur during its release. These results were discussed in terms of the groups' discrimination abilities within and between taxonomies, short-term memory limitations, and the utilization of categorical information on this task by the mentally retarded subjects.


Movement coding and memory in retarded children. Three experiments on the coding and retention of movement-generated information were performed on two groups of mildly retarded children varying in MA. The cue to be reproduced in each case was the terminal position of the limb that studies with adults have shown to require central processing activity for maintained performance. In Experiment 1, although the older MA group was superior, both groups showed similar decrements in performance over a 15-second retention interval. In Experiment 2 the procedures were adopted in an attempt to overcome performance deficits. Subjects were allowed to choose (preselect) their own movements voluntarily in addition to performing constrained, experimenter-defined movements. Preselected reproduction was superior to constrained at all three retention intervals (0, 7, and 15 seconds) but was not statistically different among age groups. Also, performance was maintained for both groups over 7 seconds but deteriorated over 15 seconds. These results were replicated in Experiment 3, which also showed that an interpolated motor task designed to block rehearsal processes interfered with reproduction at the 7- and 15-second retention-interval conditions. The findings indicated that mildly retarded children could maintain motor information over brief time periods and also illustrated the important contribution of the planning component in facilitating the coding of motoric information.


Digit span estimation and the effects of explicit strategy training on recall of EMR individuals. Accuracy of digit span estimation by EMR subjects at two different MA levels, 8 and 11, was assessed. The effects of explicit strategy training, generalized instruction, and no training on recall performance and apportionment of study time were compared. The older group was more realistic in their digit span estimation than was the younger group. Explicit strategy training facilitated digit recall and evoked longer study times than did the other conditions.


Correlates of social competence in retarded children. The relationship of role-taking and referential-communication skills with social competence was assessed in 32 retarded children (mean IQ = 56.4; mean CA = 13 years, 7 months). Significant associations were found between two types of teacher measures of children's social competence, between measures made by teachers and children themselves, and among scores of social competence and the skills of role-taking and referential communication. The findings suggest ways of assessing social adaptation more directly, which may be extended into specific interventions.


Cardiac change of retarded and nonretarded infants to an auditory signal. Five mentally retarded infants and 5 nonretarded infants were presented a tone for 15 trials. Heart-rate change from prestimulus level showed a deceleration on four 1-second intervals for the nonretarded babies and no change for the retarded babies. Comparison of the two groups of infants revealed a difference on five of the 1-second intervals following stimulus onset. When the total heart-rate change scores were examined for each baby, no overlap of the scores was evident for the infants in the nonretarded group with the individual scores of the retarded infants.


Exploration and play in retarded and nonretarded preschool children: effects of object complexity and age. Three-dimensional random polygon objects ranging in complexity between 4 and 40 turns were presented to 12, 30-month-old and 12, 42-month-old moderately retarded and nonretarded children. Time spent in exploration and in play was measured over three successive exposure-time blocks. Exploration time for the combined sample of retarded and nonretarded younger children did not vary with object complexity. The older retarded children explored objects of low complexity more than did either the combined sample of younger children or the older nonretarded children. Nonretarded older children explored complex objects more than did the combined sample of younger children. Exploration time decreased linearly with exposure-time blocks. Play time increased linearly with successive exposure-time blocks and decreased linearly with increasing complexity.


Early childhood education for psychosocially disadvantaged children: effects on psychological processes. The effects of early educational treatment upon the components of psychological development of children at high risk for sociocultural retardation were examined in 50 children. Twenty-six children attended, from infancy, a day-care center where educational treatment in the form of systematic curriculum was provided; 24 control children were educationally untreated. The McCarthy Scales of Children's Abilities were administered to both groups at 42 months of age. The educationally treated group's scores were superior to the educationally untreated group on the Verbal, Perceptual-Performance, Quantitative, and Memory Scales but not on the Motor scale. Early education appeared to improve the children's ability to attend to, comprehend, and carry out abstract and complex tasks.


Mislabeling and replication: methodological caveats. Subsequent to the publication in 1973 of the Manual on Terminology and Classification in Mental Retardation and the 1977 revisions, investigators have failed to adhere to the revisions. Additionally, some have failed to provide descriptive characteristics of subjects that would permit replication. Implications of these issues were discussed.


Adenylate cyclase from human decidua parietalis. Adenylate cyclase (EC 4.6.1.1) activity was demonstrated in membrane fractions of human decidua parietalis. Adenylate cyclase activity was associated with membrane fractions of decidua but not chorion or amnion. Prostaglandins PGE1 and PGE2 but neither PGD2 nor PGF2 alpha stimulated this activity at gestational ages 18 to 40 weeks. Fluoride and guanosine triphosphate stimulated decidual adenylate cyclase; however, epinephrine-stimulated adenylate cyclase activity was demonstrable only in the presence of guanosine triphosphate. The possible roles of adenylate cyclase activity in the decidua are discussed.


Ultrasonic assessment of uterine emptying in first-trimester abortions induced by intravaginal 15-methyl prostaglandin F2 alpha methyl ester. Early first-trimester abortions were induced by a single intravaginal pessary containing 3 mg of 15 methyl prostaglandin F2 alpha methyl ester in 17 patients. The procedure was successful in 16 patients (94%). Intrauterine events were observed by means of serial ultrasonic B scans. Pain, bleeding, and dislodgement of the gestational sac had commenced within 6 hours. Significant gastrointestinal side effects occurred in the majority of patients despite medication but they were usually of short duration. Vaginal bleeding, which was not heavy, continued for about 10 days and during most of this time the uterus contained products of conception. It is concluded that the method is effective during the first 7 weeks of gestation.


Pain of first-trimester abortion: its quantification and relations with other variables. Among 2,299 patients on whom first-trimester abortions were performed after administration of local anesthesia, 97 per cent reported experiencing some degree of pain. Independent ratings of the pain severity were obtained from the patients and also from the doctors and counselors who observed them. Although the rating procedures used by patients from those used by doctors and counselors, the three sources agreed significantly in evaluating pain levels of individual patients. Data from all three sources indicated that pain produced during the abortion procedure tended to be minor in severity. The ranking of relative painfulness of the eight stages of the aborton procedure based on average ratings obtained from doctors was nearly identical to that based on average ratings obtained from counselors. (Patients did not rate the separate stages.) On average, the patients rated the pain as being less than earache or toothache, but more than headache or bachache. The youngest patients experienced the most pain, and the oldest experienced the least. Both gestational age and cervical dilatation were related to pain in a curvilinear fashion, i.e., for both variables, patients in extreme categories experienced more pain than those in intermediate categories. Preprocedure fearfulness was positively related to intraoperative pain. No support was found for the expectation that oral administration of 5 mg. of diazepam reduces pain during this procedure.


Pyridoxine treatment of chemical diabetes in pregnancy. Thirteen women with chemical diabetes diagnosed in late pregnancy were found to excrete excessive amounts of urinary xanthurenic acid after a tryptophan load, indicative of a relative pyridoxine (vitamin B6) deficiency. Treatment with 100 mg pyridoxine daily for 14 to 23 days restored the urinary xanthurenic acid excretion to normal in all patients. Improvement of glucose tolerance was observed in only two of the patients studied, deterioration in six, and no significant change in the remaining five. The insulin response to glucose was unaltered during pyridoxine therapy.


Obstetric factors influencing outcome in infants weighing from 1,001 to 1,500 grams. The neonatal mortality rate has dramatically improved in recent years. Occurrence of these losses is concentrated in low-birth-weight infants. At Los Angeles County-University of Southern California Medical Center, the infants weighing less than 1,500 grams contribute two thirds of neonatal deaths, although they may represent only 1.5 per cent of total births. Such observations have led to the evaluation of obstetric factors as they relate to the outcome in the low-birth-weight infants weighing from 1,001 to 1,500 grams. Retrospective evaluation of 201 low-birth-weight-infants' charts demonstrated a tendency by the obstetrician to clinically underestimate fetal weight. In cases where fetal estimated weight and measured birth weight correlated, the mortality rate was 20 per cent; in cases of underestimation, the mortality rate rose to 50 per cent. Other obstetric factors evaluated were the use of FHR monitoring, the method of delivery, and the significance of the Apgar score.


Pre-ejection period of cardiac cycles in fetal lamb. The pre-ejection period (PEP) of the fetal cardiac cycle was studied on 22 chronically instrumented pregnant ewes. The PEP was measured from the onset of the Q wave on fetal electrocardiogram to the onset of the upstroke on the fetal arterial blood pressure curve. Lengthening of the PEP was observed in association with an increase in gestational age, fetal body weight, fetal brow-rump length, and fetal heart weight. Consequently, the PEP values were calculated to those at fetal heart weight of 10 grams. The PEPc (calculated) was prolonged by acidosis but no significant relationship was found between the PEPc and arterial blood pO2. An increase in coronary blood flow was associated with a shortening of the PEPc in the fetuses under normal physiologic conditions. Fetal hypoxemia and respiratory acidosis created by administration of 10 per cent O2 and 20 per cent CO2 with 20 per cent O2 to the mother increased fetal coronary flow and was in general associated with a prolonged pre-ejection period.


Antepartum determination of pre-ejection period of fetal cardiac cycle: its relation to newborn body weight. Simultaneous recordings of the fetal electrocardiogram and ultrasound Doppler-cardiogram were used for the determination of the pre-ejection period (PEP) of the fetal cardiac cycles. All 50 fetuses selected for the present study experienced uneventful perinatal lives despite maternal antepartum complications which required hospitalization. A significant positive correlation between the PEP and gestational age of the fetuses was again demonstrated. The PEP of the fetus measured within one week prior to delivery was also found to be significantly correlated with the newborn body weights at birth. Statistical analysis demonstrates that fetal body weight was more closely related to the physiologic lengthening of the PEP.


Experience with sonography as an adjunct to amniocentesis for prenatal diagnosis of fetal genetic disorders. This report summarized our experience with a consecutive series of 800 women referred for midtrimester amniocentesis for prenatal diagnosis of a fetal genetic abnormality. The results are compared before and after introduction of sonography immediately prior to the tap as a routine adjunct of the procedure. Following use of sonography there was a statistically significant reduction in the frequency of bloody taps, and an increase in the proportion of successful and informative initial taps. Moreover, in many instances sonography provided information useful for identification of fetal abnormalities or for management of pregnancy. These findings support the recommendation that examination by sonography should be utilized routinely as an adjunct to amniocentesis, despite the uncertainities relating to possible long-term adverse effects of intrauterine exposure to ultrasound.


Effects of long-term estrogen replacement therapy. I. Metabolic effects. Two groups of hypoestrogenic women are analyzed by retrospective comparisons. Patients were observed by a single group of physicians for at least five years; 301 patients were treated with replacement estrogen and 309 patients were untreated. Incidence figures for various metabolic diseases present at entry and both during and after estrogen therapy were compared by the usual statistical analysis and by statistical adjustments for certain group differences (Mantel-Haenszel statistic). The long-term administration of estrogen to these relatively young women with hypoestrogenism was associated with significantly lower rates of development of cardiovascular disease, hypertension, osteoporosis, and fractures. Detrimental effects were a higher rate of abnormal uterine bleeding and an increase in the likelihood of developing adenocarcinoma of the endometrium. Effects of estrogen preparation, dosage, method of therapy, duration of therapy, and the addition of synthetic progestins are presented.


Elevated plasma prolactin concentration in cholestasis of pregnancy. The plasma concentrations of prolactin and estradiol-17 beta were measured by specific radioimmunoassays in 150 women with normal pregnancies and 76 women with cholestasis of pregnancy. At 33 to 34 weeks of gestation plasma prolactin concentrations were 187 +/- 23 ng/ml (mean +/- S.E.M.) for normal pregnancy and 341 +/- 38 ng/ml for cholestasis (p less than 0.001). At 35 to 36 weeks they were 254 +/- 24 and 355 +/- 26 ng/ml (p less than 0.01), and at 37 to 38 weeks 175 +/- 14 and 365 +/- 34 ng/ml (p less than 0.001), respectively. Higher prolactin levels in the cholestasis group were not related to differences in plasma estradiol-17 beta concentrations. No correlation was found between plasma prolactin and serum aminotransferase levels, or between prolactin levels and placental weight. The mechanisms by which plasma prolactin levels become elevated in cholestasis of pregnancy remain to be elucidated.


Maternal and fetal renin activity and renin and big renin concentrations in second-trimester pregnancy. Plasma renin activity (PRA) and the concentrations of renin (PRC) and big renin (PBRC) have been determined in maternal and fetal blood, and renin and big renin have been measured in amniotic fluid, at 16 to 20 weeks of gestation. Gradients between peripheral arterial and venous and uterine venous maternal circulation were not apparent for PRA, PRC, or PBRC. PRC and PBRC but not PRA were consistently higher in fetal cord blood than in the maternal compartment. The concentrations of big renin and of renin were tenfold higher in amniotic fluid than in maternal plasma and were significantly correlated in amniotic fluid but not maternal or fetal plasma.


The value of diagnostic ultrasound as a screening test for intrauterine growth retardation: comparison of nine parameters. In an attempt to devise a screening method for the detection of unsuspected IUGR, a battery of ultrasonic measurements of the fetus were performed in a series of 255 patients at approximately 32, 34, and 36 weeks. The results obtained from the 16 growth-retarded and the 239 normally grown babies were evaluated. While no single measurement was found to discriminate the two groups, a simple combination of crown-rump length times area of trunk allowed us to identify an at-risk group comprising 11 per cent of the population. The false positive and false negative results were acceptable at levels of 10 and 6 per cent, approximately. It is considered that these results would justify a larger and prospectively randomized controlled trial to assess the benefits and drawbacks of applying these measurements in a screening schedule.


Fetal heart rate acceleration in labor: excellent prognostic indicator. This study explores the significance of fetal heart rate acceleration in labor. Fetal heart rate accelerations were studied in 1,677 monitored laboring patients. Fetal outcome was analyzed according to perinatal mortality rate. Apgar scores, and incidence of late deceleration. The presence of accelerations of the fetal heart rate during labor proved to be an excellent indicator of subsequent good fetal outcome.


Screening for alcohol-related problems in an outpatient obstetric-gynecologic clinic. Three methods of screening for alcohol-related problems were tested in 499 to 565 consecutive patients in an obstetric-gynecologic outpatient clinic. The methods included a 31 item self-administered questionnaire, a six-item physician-administered questionnaire, and a blood alcohol test. Medical records were reviewed for mention of conditions sometimes associated with alcohol use. Sixteen percent of the women were heavy drinkers, and 18% were found on screening to be probable or potential problem drinkers. In women under age 40 years heavy drinking was associated with a higher incidence of physical problems sometimes associated with excessive alcohol use, and significantly lower birth weights were observed in infants born to obstetric patients who were positive on screening for alcohol-related problems.


Galactorrhea and pituitary tumors in postpill and non-postpill secondary amenorrhea. One hundred sixty-seven women with secondary amenorrhea were observed from six months to four years. In 66 patients, the amenorrhea followed the discontinuation of oral contraceptives (postpill) while in the remaining 101 the amenorrhea was not temporally pill related (non-postpill). Galactorrhea was present in 43 (65%) of those with postpill amenorrhea and in 32 (32%) of those with non-postpill amenorrhea (p less than 0.001). Tomography of the sella turcica was performed in the 75 women with galactorrhea and in the 35 without galactorrhea who did not have withdrawal uterine bleeding following progesterone administration and who had low or normal serum follicle-stimulating hormone levels (hypothalamic-pituitary failure). Forty of the 75 patients with amenorrhea and galactorrhea had radiographic evidence of a pituitary tumor whereas only eight of 35 patients with hypothalamic-pituitary failure without galactorrhea had an abnormal sella turcica (p less than 0.01). The incidence of radiographic abnormalities in those with galactorrhea was similar in both the postpill and non-postpill groups.


Factors for improved genetic counseling for retinoblastoma based on a survey of 55 families. Of 55 families in which one or more patients with retinoblastoma were treated, five of these families involved more than one patient. The remaining 50 were sporadic cases. Two of the five familial cases involved collateral inheritance and three involved direct inheritance. Factors important for genetic counseling included the time of onset of first symptoms, the age of the father, the occurrence of a second primary tumor, unilateral vs bilateral involvement, and the cytogenetic analysis of the patient's chromosomes. Additionally, mutational mosaicism was considered as a cause for sporadic cases of retinoblastoma. Use of the risk figures derived from this study should provide more precise genetic counseling for parents, siblings, and patients with retinoblastoma.


Electroretinographic testing as an aid in detection of carriers of X-chromosome-linked retinitis pigmentosa. Twenty-two of 23 obligate female carriers in nine families with known X-chromosome-linked retinitis pigmentosa were detected on the basis of abnormal full-field electroretinograms (ERGs). Only 14 of these carriers had fundus findings characteristic of the carrier state. Electroretinograms of carriers were either reduced in amplitude to white light under dark-adapted conditions or delayed in cone b-wave implicit time, or both. Daughters of obligate carriers had either normal ERGs or abnormal ERGs similar to those recorded from obligate carriers. Abnormal ERGs of carriers of X-chromosome-linked retinitis pigmentosa contrasted with the normal ERGs recorded from female carriers of autosomal recessive disease. These data support the idea that ERG testing of female relatives of males with retinitis pigmentosa can help to establish for a given family whether the mode of inheritance is X-chromosome-linked or autosomal recessive.


Enlargement of a histologically documented choroidal nevus. An elevated pigmented mass of the choroid remained stable for 6 1/2 years and then was documented photographically to have increased in size. The tumor stained on fluorescein angiography, produced a visual field defect, and differentially took up radioactive phosphorus. The eye was enucleated and on histologic examination contained a nevus of the choroid. This case shows that enlargement of a choroidal nevus may occur during adulthood, and suggests that despite newer and more sophisticated diagnostic techniques, large nevi and small melanomas of the choroid may be clinically indistinguishable.


The effect of indomethacin pretreatment on aphakic cystoid macular edema. In patients treated with oral indomethacin for four weeks after intracapsular cataract extraction, five of 57 eyes (9%) had cystoid macular edema four to six weeks postoperatively as shown by fluorescein angiography. In a control group not treated with indomethacin, 15 of 34 eyes (44%) had cystoid mascular edema. Oral indomethacin, therefore, caused a statistically significant decrease in the incidence of aphakic cystoid macular edema.


Ocular sarcoidosis occurring as a unilateral optic disk vascular lesion. A 36-year-old black man who was taking low doses of systemic corticosteroids for systemic sarcoidosis had a vascular lesion of the left optic disk as the only ocular sign. The lesion regressed over a four-month period with high dosage corticosteroid therapy, but he developed optic atrophy and visual acuity of no light perception.


Sterilization of scleral homografts with ionizing irradiation. We devised a method of sterilization and preservation of sclera that we have found reliable and convenient. Gamma irradiation is a highly effective method of sterilization. Airtight, watertight, plastic packaging of frozen irradiated sclera allows for safe long-term storage and for convenient use in the operating room.


Cycloscopy of pseudoexfoliation.

Cycloscopy of pseudoexfoliation. We examined patient's eyes with pseudoexfoliation gonioscopically and cycloscopically. In almost all eyes with pseudoexfoliation, accumulation was found in the zonules and ciliary processes in addition to the lens surface and iris border. In the fellow eyes of unilaterally affected eyes, pseudoexfoliative deposits were found in the zonules and ciliary processes in more than one half of the cases, leading us to conclude that pseudoexfoliative deposits in the zonule and ciliary process may be an early and consistent characteristic of pseudoexfoliation and may be an integral part of the clinical picture of pseudoexfoliation.