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FIG.\t33.3\nIllustration\tshowing\tmultisystem\tinvolvement\tin\tcystic\tfibrosis\t(CF)\tand\ttenacious\tsecretions.\nIn\tthe\tpast,\tmost\tpatients\twere\tdiagnosed\twith\tCF\tbecause\tof\tsymptoms\trelated\tto\tvarious\torgan\nsystems.\tAll\t50\tstates\tnow\tprovide\tnewborn\tscreening\tfor\tCF\tin\tthe\tfirst\t2\tto\t3\tdays\tof\tlife,\twith\tthe | [
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majority\tof\tcases\tdiagnosed\tby\t1\tmonth\tof\tage.\tScreening\tconsists\tof\tusing\ta\tneonatal\tblood\n\tspot\tto\ndetermine\tthe\tconcentration\tof\timmunoreactive\ttrypsinogen;\tif\tit\tis\televated,\tthen\tgenetic\tanalysis\nand\tsweat\tchloride\ttests\tare\tcompleted\tto\tconfirm\tthe\tdiagnosis.\tThe\tdiagnosis\tof\tCF\tin\tpersons | [
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other\tthan\tneonates\twho\thave\ta\tclinical\thistory\tconsistent\twith\tthe\tdisease\tor\thave\ta\tsibling\twith\tCF,\nis\tdetermined\tby\tan\tabnormal\tsweat\tchloride\ttest\t(chloride\tlevel\tin\tsweat\t>60\tmmol/L).\tIf\n\tthe\nchloride\tsweat\ttest\tis\tabnormal,\tgenetic\tanalysis\tis\tused\tto\tconfirm\tthe\tdiagnosis\tand\tthe\tCFTR\nmutation\t(\nKatkin,\t2014 | [
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mutation\t(\nKatkin,\t2014\n).\tAbnormal\tsweat\tchloride\ttests\tcan\toccur\twith\tmalnutrition\tand\tadrenal\ninsufficiency.\nPathophysiology\nPulmonary\tand\tsinus\tdisease\nDue\tto\tthe\tdifficulty\tof\texpelling\tthick\tand\tviscous\tmucus\tin\tthe\trespiratory\ttract,\tCF\tpatients\npresent\twith\tchronic\tpersistent\tcough,\n\tdyspnea,\tand\twheezing.\tThick\tand\tviscous\tmucus\tis | [
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dyspnea,\tand\twheezing.\tThick\tand\tviscous\tmucus\tis\nconsidered\ta\trich\tenvironment\tfor\tbacteria\tto\tgrow,\tleading\tto\ta\tform\tof\tobstructive\tairway\tdisease,\nbronchiectasis\n,\ta\tchronic\tcondition\tof\tdilatation\tof\tthe\tbronchi\tthat\tdevelops\tas\ta\tresult\tof\trecurrent\nlung\tinfections.\tExamination\tof\ta\tpatient\twith\tbronchiectasis\tmay\tshow\tpresence\tof\t\nclubbing | [
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clubbing\n.\tDigital\nclubbing\tis\tcharacterized\tby\tincreased\tdigital\n\ttip\tmass\tand\tincreased\tlongitudinal\tand\ttransverse\tnail\nplate\tcurvature\t(\nFig.\t33.4\n),\tand\tlung\tauscultation\treveals\tdiffuse\tcoarse\tcrackles\tin\tthe\tlungs\tand\ndecreased\tbreath\tsounds.\tChest\tradiograph\tmay\tshow\thyperinflation\tin\tadvanced\tdisease,\tand\ta | [
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computed\ttomography\t(CT)\tscan\tmay\tshow\tcystic\tand\tnodular\tdensities,\tthe\tclassical\tpicture\tof\ncystic\tbronchiectasis\t(\nFig.\t33.5\n).\tPulmonary\tfunction\n\ttesting\tshows\tairway\tobstruction.\tThe\nspirogram\tshows\treduced\tforced\tvital\tcapacity\t(FVC),\tknown\tas\t“restrictive\tpattern.”\tThe\treduced\nforced\texpiratory\tvolume\t(FEV\n1\n)\tand\tthe\treduced\tFEV\n1 | [
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FIG.\t33.4\nIllustration\tof\tclubbing\tin\ta\tpatient\twith\t“yellow\tnail\tsyndrome.”\nFIG.\t33.5\nComputed\ttomography\t(CT)\tscan\tof\ta\tpatient\tshowing\tcystic\tchanges\tof\tbronchiectasis\tin\tright\nupper\tlobe.\nSputum\tcultures\tare\tused\tto\tidentify\tbacteria\tgrowing\tin\tthe\trespiratory\ttract\tin\tCF\tpatients.\tMost\ncommon\tbacteria\tin\tCF\tpatients\tare\t\nStaphylococcus\taureus | [
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common\tbacteria\tin\tCF\tpatients\tare\t\nStaphylococcus\taureus\n\tand\t\nPseudomonas\taeruginosa\n,\twhich\ncolonize\tthe\tairways\tfrequently.\tEvidence\texists\tthat\tthese\tbacteria\tplay\tan\timportant\n\trole\tin\tdisease\nprogression.\tMany\tchildren\twith\tCF\tdevelop\tchronic\trhinosinusitis\twith\tnasal\tpolyposis.\tSome | [
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questions\tremain\tunanswered\tas\tto\tthe\torigin\tof\tpolyps\tin\tCF\tpatients.\nPancreatic\tdisease\tin\tcystic\tfibrosis\nSeveral\tdifferent\ttypes\tof\tpancreatic\tdisease\toccur\tin\tCF\tpatients.\nPancreatic\tinsufficiency.\nPancreatic\tinsufficiency\t(PI)\n,\tin\twhich\tthe\tpancreas\tfails\tto\tmake\tadequate\tenzymes\n\tto\tdigest\tfood | [
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to\tdigest\tfood\nin\tthe\tsmall\tintestine,\tis\tthe\tmost\tcommon\tgastrointestinal\tcomplication\tof\tCF,\taffecting\napproximately\t90%\tof\tpatients\tat\tsome\ttime\tin\ttheir\tlives\t(\nRogers,\t2013\n).\nUp\tto\t90%\tof\tpatients\texhibit\tfat\tmalabsorption\tby\t1\tyear\tof\tage.\tThe\tproduction\tof\tpancreatic | [
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enzymes\tis\tdecreased,\twhich\tleads\tto\tthe\tmalabsorption\tof\tfats\tand\tsteatorrhea.\t\nSteatorrhea\n\tis\ncharacterized\tby\tfoul\tsmelling,\n\tbulky,\toily\tstools\tand\tfailure\tto\tthrive\tor\tpoor\tweight\tgain.\tSuch\npatients\tmay\talso\tpresent\twith\tclinical\tfeatures\tof\tdeficiency\tof\tthe\tfat-soluble\tvitamins\tA,\tD,\tE,\tand | [
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K.\tThe\tCF\tpopulation\tfrequently\tpresents\twith\tsuboptimal\tvitamin\tD\tstatus.\tCF\tpatients\talso\tsuffer\nfrom\tvitamin\tK\tdeficiencies\tand\tneed\troutine\tsupplementation.\nPancreatitis.\nThe\tabnormal\tpancreatic\tsecretions\tcause\tprogressive\n\tpancreatic\tdamage,\tso\tthese\tpatients\tcan\npresent\twith\tacute\tor\trecurrent\tpancreatitis.\nCF-related\tdiabetes. | [
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Patients\twith\texocrine\tPI\talso\tmay\tdevelop\timpaired\tfunction\tof\tthe\tendocrine\tpancreas,\tleading\tto\nthe\tdevelopment\tof\t\ncystic\tfibrosis\trelated\tdiabetes\t(CFRD)\n.\tIt\tis\tthe\tmost\tcommon\tcomorbidity\tin\nthe\tCF\tpopulation,\toccurring\tin\t20%\tof\tthe\tadolescents\tand\t40%\tto\t50%\tof\tthe\tadults\twith\n\tCF.\tCFRD | [
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CF.\tCFRD\nis\tassociated\twith\tpoor\tgrowth,\tclinical\tand\tnutritional\tdeterioration,\tand\tearly\tdeath.\tThe\tAmerican\nDiabetes\tAssociation\tand\tthe\tCystic\tFibrosis\tFoundation\trecommend\tyearly\tCFRD\tscreenings,\nstarting\tat\tage\t10\t(see\t\nChapter\t29\n\tfor\tdiscussion\tof\tdiabetes\tmanagement),\tto\tbe\tperformed\tusing\ta | [
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for\tdiscussion\tof\tdiabetes\tmanagement),\tto\tbe\tperformed\tusing\ta\n2-hour\t75\tmg\toral\tglucose\ttolerance\ttest\tduring\ta\tperiod\tof\tstable\thealth.\tThe\tuse\tof\themoglobin\n\tA1C\nis\tnot\trecommended\tfor\tscreening\tbecause\tit\thas\tlow\tsensitivity\tin\tCFRD\t(\nMoran\tet\tal,\t2010\n). | [
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Moran\tet\tal,\t2010\n).\nHemoglobin\tA1C\tis\toften\tnormal\tin\tpatients\twith\tCFRD\tregardless\tof\tthe\tdegree\tof\thyperglycemia.\nHowever,\tif\tit\tis\talready\tmeasured\tand\tthe\tvalue\tis\t>6.5,\tthis\tis\tstill\tconsistent\twith\ta\tdiagnosis\tof\nCFRD.\nBone\tdisease\nDue\tto\tvitamin\tD\tdeficiency,\tbone\tdisease\tis\tcommon\tin\tCF\tpatients\tand\tis\tcharacterized\n\tby | [
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decreased\tmotility.\tThis\tdisorder\tinterferes\twith\tfat\tabsorption\tand\tappetite\tand\tcan\tlead\tto\tmultiple\nfood\tintolerances\tand\tdigestive\tdisturbances\t(\nBaker\tet\tal,\t2013\n)\t(see\t\nChapter\t27\n).\nMedical\tmanagement\nCF\tis\ta\tmultisystem\tdisease\tthat\trequires\ta\tmultidisciplinary\tapproach\tto\tmanage\tsymptoms,\tcorrect\ndeficiencies, | [
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deficiencies,\n\tand\tprevent\tcomplications\tand\tprogression\tof\tthe\tdisease.\tThe\tCF\tteam\tusually\tincludes\nmedical\tand\tnursing\tstaff;\trespiratory\ttherapists,\tphysical\ttherapists,\tregistered\tdietitian\nnutritionists;\tsocial\tworkers;\tand\tgenetic\tcounselors.\tImpairment\tof\tthe\trespiratory\tand | [
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gastrointestinal\tsystems\tis\tresponsible\tfor\tsignificant\tmortality\tand\tmorbidity.\tSinus\tinfection,\nglucose\tcontrol,\tnutritional\tstatus,\n\tand\tpsychosocial\tissues\tmust\tbe\tassessed\tand\tmanaged\tat\tregular\nintervals\t(\nFig.\t33.6\n).\nFIG.\t33.6\nAlgorithm\tfor\tdiagnosis\tand\tmanagement\tof\tcystic\tfibrosis. | [
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0.05200645700097084,
0.13733069598674774,
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] |
pancreatic\tenzymes\tsuch\tas\tlipase,\tamylase,\tand\tprotease\tfrom\tsecreting\tinto\tthe\tsmall\tintestine.\nThe\tbuild-up\tof\tthese\tenzymes\tin\tthe\tpancreas\tleads\tto\tauto\tdigestion\tof\n\tthe\tpancreas\tand\ndestruction\tof\tacinus.\tPancreatic\tacinus\tis\tthe\tsecretory\tunit\tof\texocrine\tpancreas,\twhere\tpancreatic\njuice\tis\tproduced.\tDestruction\tof\tpancreatic\tacinus,\tor | [
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juice\tis\tproduced.\tDestruction\tof\tpancreatic\tacinus,\tor\t\nacinar\n\tdestruction,\tresults\tin\timpaired\nsecretion\tof\tpancreatic\tjuice,\twhich\tresults\tin\tloose,\toily,\tfrequent\tstools\tand\tmalabsorption.\tThese\nindividuals\tneed\tPERT\tto\tcombat\tthe\tPI\tand\tmaintain\tadequate\tabsorption\tand\tdigestion\n\tof\tnutrients\n(see\t\nFocus\tOn:\n\tPERT\n).\n\t\nFOCUS\tON\nPERT | [
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emphasize.\tIf\tgastrointestinal\tsymptoms\tcannot\tbe\tcontrolled,\tenzyme\tdosage,\tpatient\tadherence,\nand\tenzyme\ttype\tshould\tbe\treevaluated.\tFecal\t\nelastase\n\t(protein-digesting\tenzyme\tsecreted\tby\tthe\npancreas\tand\tinvolved\tin\thydrolysis\tof\tpeptide\n\tbonds),\tfecal\tfat,\tor\tnitrogen\tbalance\tstudies\tmay\nhelp\tto\tevaluate\tthe\tadequacy\tof\tenzyme\tsupplementation. | [
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help\tto\tevaluate\tthe\tadequacy\tof\tenzyme\tsupplementation.\nThe\tacidic\tenvironment\tof\tthe\tstomach\tdoes\tnot\tallow\tthe\tenzymes\tof\tthe\tenteric\tcoated\tPERT\nto\tbe\tdegraded.\tThis\tnormally\toccurs\tonce\tthe\tpH\tis\tabove\t5.5,\tusually\twhen\tthe\tPERT\treaches\nthe\tduodenal\tjejunum.\tA\tcommon\tpractice\tis\tplacing\tcystic\tfibrosis\t(CF)\tpatients\ton\tproton\tpump\ninhibitors | [
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inhibitors\n\tin\torder\tto\tincrease\tthe\tduodenal\tpH\tby\tdecreasing\tgastric\tacid\tsecretion\t(\nRogers,\n2013\n).\nIntestinal\tabnormalities\tsuch\tas\tgastroesophageal\treflux\tdisease\t(GERD),\tmeconium\tileus\t(MI),\nand\t\ndistal\tintestinal\tobstruction\tsyndrome\t(DIOS)\n,\twhich\tis\tthe\tblockage\tof\tintestines | [
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located\tin\tthe\tbiliary\tepithelium.\tBile\tproduced\tin\tpatients\twith\tCF\tis\tthick\tand\ttenacious,\tcausing\nblockage\tof\tthe\tintrahepatic\tbile\tducts.\tBlockage\tof\tthese\tducts\teventually\tleads\tto\tcirrhosis.\tUse\nof\tursodeoxycholic\n\tacid\t(UDCA)\tmay\tdelay\tthe\tprogression\tof\tliver\tdisease\t(\nKappler\tet\tal,\t2012\n)\n(see\t\nChapter\t28 | [
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] |
Kappler\tet\tal,\t2012\n)\n(see\t\nChapter\t28\n);\thowever,\ta\trecent\tCochrane\tReview\tfound\tlittle\tevidence\tto\tsupport\troutine\tuse\nof\tUDCA,\tciting\tthe\tsmall\tnumber\tof\ttrials\tassessing\tits\teffectiveness\t(\nCheng\tet\tal,\t2017\n).\tAt\tthis\ntime,\tthere\tis\tlittle\tevidence-based\tresearch\tfor\tthe\tprevention\tand\tmanagement\tof\tliver\tdisease\tin\npersons\twith\tCF\t( | [
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persons\twith\tCF\t(\nPalanippan\tet\tal,\t2017\n).\nMedical\tnutrition\ttherapy\nMedical\tnutrition\ttherapy\t(MNT),\tcritical\tto\tmanagement\tof\tCF\tand\tall\tof\tits\tcomorbidities,\tis\tvital\tin\npromoting\tlongevity\tand\tpositive\toutcomes.\tMNT\tbegins\twith\tevaluation\tof\tthe\tnutritional\tstatus\tof\nthe\tpatient\t(\nBaker\tet\tal,\t2013\n)\t(see\t\nChapters\t4\n\tand\t\n5 | [
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the\tpatient\t(\nBaker\tet\tal,\t2013\n)\t(see\t\nChapters\t4\n\tand\t\n5\n).\tCF\tpatients\tfrequently\thave\tgrowth\tfailure,\nthe\tcause\tof\twhich\tis\tmultifactorial:\tmalabsorption,\tincreased\n\tenergy\tneeds,\tand\treduced\tappetite.\nNutritional\tstatus\tis\tclosely\tcorrelated\tto\tpulmonary\tfunction\tand\tsurvival\tin\tCF.\tAs\ta\tresult,\tclose | [
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] |
attention\tto\tgrowth\tstatus\tin\tchildren\tshould\tbe\tmonitored;\tclinical\tcare\tguidelines\tfor\tinfants\tand\npreschoolers\thave\tbeen\tpublished\t(\nBorowitz\tet\tal,\t2009\n;\t\nLahiri\tet\tal,\t2016\n).\tAll\tpatients\twith\tCF\nshould\thave\tregular\tnutritional\tassessments\tfor\tearly\tdetection\n\tof\tnutritional\tstatus\tdeterioration\n(see | [
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] |
of\tnutritional\tstatus\tdeterioration\n(see\t\nPathophysiology\tand\tCare\tManagement\tAlgorithm:\n\tCystic\tFibrosis\n).\tOlder\tchildren\tshould\tbe\nevaluated\tfor\tbone\tdensity,\tusing\tthe\tdual-energy\tx-ray\tabsorptiometry\t(DEXA)\t(see\t\nChapter\t23\n).\nCF\tis\ttypically\tassociated\twith\tmalnutrition;\thowever,\tthe\tnumber\tof\toverweight\tand\tobese\tpatients | [
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function.\tDietary\trecommendations\tshould\temphasize\ta\tbalanced\thealthy\tdiet\twith\tgood\texercise\nhabits.\nMajor\tgoals\tof\tnutritional\ttherapy\tare\tincreasing\tmuscle\tstrength,\tpromoting\toptimal\tgrowth\tand\nweight\tmaintenance,\tand\tenhancing\tthe\tquality\tof\tlife.\tTo\tachieve\tthese\tgoals,\tthe\tobjectives\tof | [
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treatment\tare\tto\tcorrect\tmaldigestion\tand\tmalabsorption\tand\tto\tprovide\n\tnutrients\tthat\tare\tcommonly\ndeficient.\nEnergy.\nEvidenced-based\tnutrition\tgoals\tfor\tindividuals\twith\tCF\tare\tbased\ton\tthe\tCystic\tFibrosis\tFoundation\nregistry\tdata\tanalysis\tfor\tage\tand\tsex:\n•\t\nNewborn\tto\t24\tmonths:\tweight/length\t≥50th\tpercentile,\tusing\tCenters\tfor\tDisease\tControl | [
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and\tPrevention\t(CDC)\tgrowth\tcharts\n•\t\n2\tto\t20\tyears\told:\tBMI\t50th\tto\t85th\tpercentile,\tusing\tCDC\tgrowth\tcharts\n•\t\nAdult\tfemales:\tBMI\t22\tto\t27\tkg/m\n2\n•\t\nAdult\tmales:\tBMI\t23\tto\t27\tmg/km\n2\nThese\tweight\tgoals\tare\tassociated\twith\ta\tdesirable\tpulmonary\tfunction.\nA\tbroad\trange\tof\tenergy\trequirements\tare\treported\tin\tpatients\twith\tCF,\tranging\tfrom\t120%\tto | [
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150%\tof\trecommendations\tfor\tthe\tgeneral\tpopulation,\tdepending\ton\tthe\tCF\tmutation,\tthe\tpatient’s\nage,\tdegree\tof\tmalabsorption,\tpresence\tof\tpulmonary\texacerbation,\n\tpulmonary\tfunction,\tsex,\npubertal\tstatus,\tpresence\tof\tadditional\tmedical\tcomplications\t(CFRD,\tCF\tliver\tdisease),\tand\tthe\ncurrent\tstate\tof\thealth\t(\nSchindler\tet\tal,\t2015\n). | [
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current\tstate\tof\thealth\t(\nSchindler\tet\tal,\t2015\n).\nA\tchallenging\tissue\tis\tattaining\tan\tadequate\tenergy\tintake\twith\tan\tenergy\tdense\tdiet.\tThe\ndetermination\tto\tinitiate\tenteral\tfeedings\tis\tindividualized\tfor\tthose\twho\tare\tunable\tto\tconsume\nadequate\tcalories\tand\tprotein\tto\tmeet\n\tgrowth/weight\tmaintenance\tgoals\tor\tthose\twith\tmoderate\tor | [
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growth/weight\tmaintenance\tgoals\tor\tthose\twith\tmoderate\tor\nsevere\tmalnutrition\t(\nSchwarzenberg,\t2016\n).\tTube\tfeeding\toptions\tinclude\tgastrostomy\ttube,\nnasogastric\ttube,\tand\tjejunostomy\ttube.\tThe\tmost\tcommon\tdelivery\toption\tis\tthe\tpercutaneous\nendoscopic\tgastrostomy\t(PEG)\ttube\t(see\t\nChapter\t12\n).\tAppropriate\tPERT\tmanagement\tshould\tbe | [
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] |
Chapter\t12\n).\tAppropriate\tPERT\tmanagement\tshould\tbe\nmaintained\twith\tgeneral\tguidelines\tbased\ton\tfat\tgrams\tprovided.\tIf\n\tthe\tpatient\tis\teating,\tPERT\tcan\nbe\tadministered\torally.\tFor\tthose\twho\tare\tnot\ttaking\tanything\tby\tmouth,\tPERT\tmay\tbe\tcrushed\n(depending\ton\tthe\ttype\tof\tPERT),\tdissolved\tin\ta\tsodium\tbicarbonate\tsolution,\tand\tthen\tadministered | [
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these\tpatients\tto\tdeficiencies\tof\tfat-soluble\tvitamins\tA,\tD,\tE,\tand\tK\tand\tsome\tminerals\teven\twith\nPERT\tuse.\tVitamin\tlevels\tshould\tbe\tassessed\tat\tdiagnosis\t(except\tthe\tnewborn)\tand\tsupplemented\tas\nindicated.\tCF-specific\tvitamins\tcontain\tfat\tand\twater-soluble\tvitamins\tas\twell\tas\tzinc\tto\tenhance | [
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absorption.\tDeficiencies\tof\tvitamins\tA,\tD,\tE,\tand\tK,\twhich\tsignificantly\taffect\tCF\tpatients,\treduce\ntheir\tresponse\tto\tpulmonary\tinfections.\nBecause\tbone\tdisease\tis\tcommon\tin\tpatients\twith\tCF\tand\tprogresses\twith\tage\t(\nRana\tet\tal,\t2014\n),\nthe\tCystic\tFibrosis\tfoundation\trecommends\tpatients\tbe\ttreated\twith\tcholecalciferol\t(D\n3\n).\tA\tdaily | [
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3\n).\tA\tdaily\nvitamin\tD\tdosage\tof\t1500\tto\t2000\tIU\tis\tsuggested\tby\tthe\tEndocrine\n\tSociety\twith\tdosages\tincreasing\nto\t10,000\tIU\tdaily\tfor\tCF\tpatients\t18\tor\tolder\t(\nRogers,\t2013\n).\nIt\tis\talso\trecommended\tthat\tthe\tCF\tpatient\tbe\tsupplemented\twith\tvitamin\tK.\tTaking\tat\tleast\t1000\nmcg/day\twas\tfound\tto\tachieve\tan\toptimal\tstatus\tof\tvitamin\tK\t(\nRogers,\t2013 | [
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] |
Rogers,\t2013\n).\nFat-soluble\tvitamin\ttesting\tis\timportant\tin\tthe\tidentification\tof\tdeficiencies\tin\tCF\tpatients\twith\tPI.\nFat-soluble\tvitamins\tare\n\tsupplemented\troutinely\tand\tannually\tmonitored.\tPatients\tmay\tbe\nnoncompliant\twith\tvitamin\tsupplementation\tor\trequire\thigher\tdosages\t(\nRana\tet\tal,\t2014\n).\nSalt. | [
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Rana\tet\tal,\t2014\n).\nSalt.\nExcessive\tsodium\tloss\tin\tperspiration\tin\tpatients\twith\tCF\tpredisposes\tthem\tto\thyponatremic\ndehydration\tunder\tconditions\tof\theat\tstress.\tMost\tpatients\tneed\tsupplementation\tof\tsodium\tchloride\nstarting\tin\tinfancy.\tThe\tamount\tof\tsalt\tsupplementation\n\tshould\tbe\tincreased\tunder\tcircumstances | [
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should\tbe\tincreased\tunder\tcircumstances\nsuch\tas\thigh\ttemperature\tand\thumidity;\ta\tdry,\tdesert\tclimate;\tstrenuous\texercise\tin\thot\tweather;\nexcessive\tsweating;\tfever;\tand\tpresence\tof\tdiarrhea\tor\tvomiting.\nZinc.\nAdditional\tzinc\tis\trecommended\tif\ta\tdeficiency\tis\tsuspected\tin\tpatients\twith\tan\tunexplained\tdecline | [
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in\tgrowth\tor\tappetite\tor\tthose\twith\tprolonged\tdiarrhea\t(\nSchindler\tet\tal,\t2015\n).\nAsthma\nAsthma\n\tis\ta\tchronic\tdisorder\tthat\taffects\tthe\tairways\tand\tis\tcharacterized\tby\tbronchial\thyper-\nreactivity,\treversible\tairflow\tobstruction,\tand\tairway\tremodeling.\tAsthmatic\tsymptoms\tinclude | [
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periodic\tepisodes\tof\tchest\ttightness,\tbreathlessness,\tand\twheezing.\tAsthma\thas\tbecome\tmore\nprevalent\tand\thas\tbeen\tincreasing\tat\tthe\trate\tof\t25%\tto\t75%\tevery\tdecade\tsince\t1960\tin\twesternized\ncountries\t(\nAllan\tand\tDevereux,\t2011\n).\tIt\taffects\tall\tage\tgroups.\nPathophysiology | [
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pollution,\trecurring\trespiratory\tinfections,\tgastrointestinal\tesophageal\treflux,\tsulfites\tin\tfoods,\tand\nmedication\tsensitivities\t(\nFanta,\t2017\n).\tA\tyounger\tgestational\tage\tand\ta\thigher\tinfant\tweight\tgain\tare\nassociated\twith\tthe\tdevelopment\tof\tasthma\t(\nSonnenschein-van\tder\tVoort\tet\tal,\t2014\n).\tLower | [
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2.\t\nAirflow\tobstruction\tthat\trecurs\tin\tresponse\tto\ta\ttrigger\n3.\t\nSymptoms\tconsistent\twith\tasthma\nSymptoms\tsuch\tas\twheezing,\tcoughing,\tshortness\tof\tbreath,\tand\tchest\ttightness\n\toccur\tin\tmost\npatients,\tand\tsymptoms\tthat\tworsen\tat\tnight\tis\ta\tcommon\tfeature.\tAlthough\tallergic\tasthma\tor | [
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“extrinsic\tasthma”\tis\tdue\tto\tchronic\tallergic\tinflammation\tof\tthe\tairways,\t“intrinsic\tasthma”\tis\ntriggered\tby\tnonallergic\tfactors\tsuch\tas\texercise,\tcertain\tchemicals,\tand\textreme\temotions\t(\nChih–\nHung\tGuo\tet\tal,\t2012\n).\nA\tlife-threatening\tsituation\twith\tmarkedly\tnarrow\tairways,\tknown\tas\tstatus\n\tasthmaticus,\tcan\tresult | [
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] |
asthmaticus,\tcan\tresult\nwhen\tasthma\thas\tnot\tbeen\ttreated\tproperly.\tCorticosteroid\ttherapy\tis\toften\tprescribed,\tbut\tchronic\nuse\tmay\tplace\tthe\tindividual\tat\trisk\tfor\t\nosteopenia\n\t(precursor\tto\tosteoporosis),\tbone\tfractures,\tor\nsteroid-induced\thyperglycemia\t(see\t\nAppendix\t13\n).\tSome\tevidence\tsupports\tthe\teffectiveness\tof | [
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] |
Appendix\t13\n).\tSome\tevidence\tsupports\tthe\teffectiveness\tof\nsublingual\timmunotherapy\tin\tthe\ttreatment\tof\tasthma\tand\trhinitis,\tbut\tmore\tstudies\n\tare\tneeded\ton\noptimal\tdosages\t(\nLin\tet\tal,\t2013\n).\nMedical\tmanagement\nThe\tessential\tcomponents\tof\tasthma\ttherapy\tare\troutine\tmonitoring\tof\tsymptoms\tand\tlung\tfunction, | [
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severity\tof\tthe\tasthma,\twhich\tcan\tbe\nclassified\tas\tan\tacute\tattack,\tintermittent,\tmild\tpersistent,\tmoderate\tpersistent,\tor\tsevere\tpersistent.\nQuick-relief\tand\tlong-term\tcontroller\tmedications\tare\tused\tas\ttherapy\tfor\tasthma.\tAlthough\tquick-\nrelief\tmedications\tinclude\tshort-acting\tbeta\tagonists\t(bronchodilators)\tand\tsteroid\tpills,\tlong-term | [
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controller\tmedications\tinclude\tinhaled\tcorticosteroid,\n\tlong-acting\tbeta\tagonists,\tand\tleukotriene\nmodifiers.\tLong-acting\tanticholinergics\tare\tnow\tconsidered\tas\tadditional\ttherapy\tin\tthose\tnot\ncontrolled\twith\tinhaled\tcorticosteroids\t(\nSobieraj\tet\tal,\t2018\n). | [
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controlled\twith\tinhaled\tcorticosteroids\t(\nSobieraj\tet\tal,\t2018\n).\nInhaled\tcorticosteroids\tare\tthe\tcornerstone\tof\tpharmacologic\tmanagement\twith\tpersistent\tasthma.\nSome\tpatients\twith\trefractory\tasthma\tneed\tmaintenance\tdoses\tof\tsystemic\tsteroids.\tBecause\tsteroids | [
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interactions.\nModulation\tof\tantioxidant\tintake\twith\tnutritional\tsupplementation\thas\ta\tbeneficial\teffect\ton\tthe\nseverity\tand\tprogression\tof\tasthma\t(\nFabian\tet\tal,\t2013\n).\tAlthough\ta\tslight\tinverse\tassociation\twas\nseen\tbetween\ta\tlow\tvitamin\tE\tintake\tand\twheezing\tsymptoms,\tno\tassociation\twas\tfound\tbetween\nvitamin\tE\tand\tasthma.\tFurther | [
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vitamin\tE\tand\tasthma.\tFurther\n\tstudies\tare\trequired\tto\tunderstand\tthe\tmechanism\tof\tvitamin\tE\ton\tthe\ninflammation\tof\tthe\timmune\tsystem\t(\nFabian\tet\tal,\t2013\n).\tLow\tblood\tcarotenoid\tlevels\talso\thave\tbeen\nlinked\twith\tasthma.\tA\tdiet\trich\tin\tantioxidants\tand\tmonounsaturated\tfats\tseems\tto\thave\ta\tprotective | [
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effect\ton\tchildhood\tasthma\tby\tcounteracting\toxidative\tstress\t(\nGarcia-Marcos\tet\tal,\t2013\n).\tThe\nrelationship\tbetween\tselenium\tstatus\n\tand\tthe\tincidence\tand\tseverity\tof\tasthma\thas\tbeen\tinconsistent\nin\thumans\tso\tselenium\tsupplementation\tis\tnot\trecommended\t(\nNorton\tet\tal,\t2012\n). | [
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been\treported\tto\timprove\tasthma\nsymptoms\tand\tlung\tfunction\t(\nAllan\tand\tDevereux,\t2011\n).\nConflicting\tresults\ton\tthe\tefficacy\tof\tvitamin\tD\tsupplementation\thave\tbeen\treported.\tIn\tone\tstudy\nan\tinsufficient\tserum\tlevel\tof\tless\tthan\t30\tng/dL\tof\tvitamin\tD\twas\tassociated\twith\tan\tincrease\tin | [
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asthma\texacerbation\tin\tthe\tform\tof\temergency\troom\t(ER)\tvisits\tand\thospitalizations\t(\nBrehm\tet\tal,\n2010\n).\tIn\tanother,\n\thigh\tdoses\tof\tvitamin\tD\tsupplementation\twere\tnot\tshown\tto\thave\tany\tprotective\neffect\t(\nLitonjua\tet\tal,\t2014\n).\tDue\tto\tthe\tconflicting\tresults\tof\tthese\tstudies,\tit\tcan\tbe\tconcluded\tthat | [
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vitamin\tD\tsupplementation\tshould\tnot\tbe\trecommended\tat\tthis\ttime\tas\ttherapy\tfor\tasthma\t(\nJiao\tand\nCastro,\t2015\n).\nOther\tnutritional\tinterventions\tcould\tpotentially\tinclude\tuse\tof\tprobiotics,\toral\tmagnesium,\tand\tthe\nMediterranean\tdiet.\tReduced\tdiversity\tor\taberrant\tcomposition\tof\tthe\tinfant\tgut\tmicrobiota\tmay\tbe | [
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] |
associated\twith\tthe\tdevelopment\tof\tasthma\tin\tlater\tlife,\tso\tbreastfeeding\tis\trecommended\tto\tensure\ngrowth\tof\thealthy\tmicrobiota\t(\nMilani\tet\tal,\t2017\n;\t\nHendaus\tet\tal,\t2016\n).\tA\tnutritionally\thealthy\tdiet\nsuch\tas\tthe\tMediterranean\tdiet\tmay\thelp\tprevent\tasthma\tduring\tchildhood\t(\nHendaus\tet\tal,\t2016\n).\tIn | [
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Hendaus\tet\tal,\t2016\n).\tIn\na\tsmall\tstudy\tof\t55\tmales\tand\tfemales\twith\tmild\tto\tmoderate\tasthma,\tsupplementing\twith\t340\tmg\nmagnesium/\tday\t(in\ttwo\tdivided\tdoses\tof\t170\tmg)\tfor\t6\tmonths\treduced\tbronchial\treactivity\tand\nimproved\tpulmonary\tfunction\ttests\t(\nKazaks\tet\tal,\t2010\n). | [
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] |
control\t(\nMa\tet\tal,\t2015\n).\nGastroesophageal\treflux\tdisease\t(GERD)\n\tand\tfood\tallergens\tare\tthe\ttwo\tmost\tcommon\tdietary\ntriggers\tfor\tasthma.\tGERD\tis\thighly\tprevalent\tin\tasthmatic\tpatients.\tA\tcritical\tcomponent\tof\tMNT\tfor\nasthmatic\tpatients\tis\ta\tdiet\tfree\tof\tknown\tirritants\tsuch\tas\tspicy\tfoods,\tcaffeine,\tchocolate,\tand\tacidic\nfoods\t(see\t\nChapter\t26 | [
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] |
foods\t(see\t\nChapter\t26\n).\tLimiting\tthe\tintake\tof\thigh\tfat\tfoods\tand\tportion\tcontrol\tcan\tprevent\tgastric\nsecretions,\twhich\texacerbate\tGERD.\nFood\tallergens\tand\tfood\tadditives\tare\tother\tpotential\tdietary\ttriggers\tfor\tasthma.\tAn\tIgE-mediated\nreaction\tto\ta\tfood\tprotein\tcan\tlead\tto\tbronchoconstriction.\tCompletely\tavoiding\tthe\tallergenic\tfood | [
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protein\tis\tthe\tonly\tdietary\ttreatment\tcurrently\tavailable\tfor\tfood\tallergies.\tSome\tsulfites,\tsuch\tas\npotassium\tmetabisulfite\tand\tsodium\tsulfide,\tused\tin\tthe\tprocessing\tof\tfoods\thave\tbeen\tfound\tto\tbe\ta\ntrigger\tfor\tasthmatics\t(\nGaur\tet\tal,\t2013\n)\nSome\tasthma\tpatients\tneed\tmaintenance\toral\tsteroids,\tand\tthese\tpatients\tare\tprone\tto\tdevelop | [
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and\tgenetic\tfactors\tare\talso\tfactors\tin\tthe\tdevelopment\n\tof\tCOPD\t(\nTable\t33.1\n).\tPatients\twith\tCOPD\nsuffer\tfrom\tdecreased\tfood\tintake\tand\tmalnutrition\tthat\tcauses\trespiratory\tmuscle\tweakness,\nincreased\tdisability,\tincreased\tsusceptibility\tto\tinfections,\tand\thormonal\talterations.\nTABLE\t33.1 | [
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-0.06789786368608475,
0.08731735497713089,
-0.023586992174386978,
-0.08172132819890976,
-0.025503767654299736,
0.013199953362345695,
0.0023599539417773485,
0.01810883730649948,
0.0075105358846485615,
0.016225391998887062,
-0.01934102550148964,
0.038365356624126434,
0.04992700740695,
0.05286077782511711,
0.04755033552646637,
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-0.01825816184282303,
-0.007689225021749735,
-0.0722121000289917,
0.01693582721054554,
0.0156569741666317,
-0.06146131455898285,
-0.03151465952396393,
0.11295731365680695,
-0.009579955600202084,
-0.020982304587960243,
-0.026276785880327225,
0.08392518758773804,
0.07335884124040604,
-0.07562553137540817,
-0.01119971927255392,
0.041648831218481064,
-0.0009420827263966203,
-0.07527878135442734,
-0.006436055526137352,
0.10151982307434082,
0.020199740305542946,
-0.007250905968248844,
-0.10804270207881927,
0.0717725157737732,
0.09839047491550446,
-0.008959023281931877,
0.01820899359881878,
-0.02080852910876274,
0.07036088407039642,
0.04145868867635727,
0.10893363505601883,
-0.016896173357963562
] |
TABLE\t33.1\nRisk\tFactors\tfor\tChronic\tObstructive\tPulmonary\tDisease\t(COPD)\nDefinite\nProbable\nTobacco\tsmoking\nOccupational\texposure\nExposure\tto\tbiomass\tfuel\tsmoke\nEnvironmental\ttobacco\tsmoke\nPulmonary\ttuberculosis\nRepeated\tlower\trespiratory\tinfection\tduring\tchildhood\nPoorly\ttreated\tasthma | [
0.00913655012845993,
-0.03301069140434265,
0.026111533865332603,
-0.010717072524130344,
-0.09521615505218506,
-0.010814385488629341,
0.03566312789916992,
0.016259415075182915,
-0.03623654693365097,
0.09480727463960648,
0.10777747631072998,
-0.11395647376775742,
0.03901093825697899,
0.060325987637043,
-0.061907295137643814,
0.0031915553845465183,
0.001845782739110291,
0.029659206047654152,
-0.05182164907455444,
0.009213465265929699,
0.021092088893055916,
0.1441999226808548,
0.03708785027265549,
-0.008467250503599644,
0.012746705673635006,
0.055939141660928726,
-0.07133424282073975,
-0.0377478301525116,
0.024076180532574654,
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0.056276775896549225,
0.009427645243704319,
0.004623750224709511,
0.11375002562999725,
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0.03321301192045212,
0.045194532722234726,
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0.06437208503484726,
0.04089514911174774,
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0.02664904110133648,
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0.037115320563316345,
0.026002438738942146,
0.05134199932217598,
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0.013418021611869335,
0.02614058554172516,
0.09234331548213959,
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0.023724786937236786,
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0.0969848558306694,
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0.029924677684903145,
0.05722213163971901,
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0.02841174229979515,
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0.04906797781586647,
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0.009079840034246445,
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0.012441151775419712,
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0.02531842514872551,
-0.04961731284856796,
-0.01013453584164381,
0.0192840825766325,
-0.072523333132267,
0.03564940392971039,
-0.05193949490785599,
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0.019339965656399727,
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0.06148210167884827,
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0.00011237256694585085,
0.011652424931526184,
0.021713417023420334,
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0.07360959053039551,
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0.013193135149776936,
0.028946707025170326,
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0.023018499836325645,
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0.14087292551994324,
-0.01516405027359724,
0.0013890413101762533,
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0.019405467435717583,
0.04852224886417389,
-0.027010351419448853,
-0.046652987599372864,
0.004136602859944105,
5.641240001266375e-33,
0.044442079961299896,
0.001771866693161428,
0.004557330626994371,
0.035740774124860764,
0.07956036180257797,
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0.011927744373679161,
0.07295970618724823,
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0.03161124885082245,
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0.06922629475593567,
0.04543669894337654,
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-0.07084205001592636,
0.05898695066571236,
-0.08588280528783798,
-0.004920781124383211,
-0.03284621983766556,
0.14683043956756592,
0.034271445125341415,
0.029298992827534676,
-0.017892612144351006,
-0.021464955061674118,
-0.021601418033242226,
-0.01107052806764841,
0.0033872767817229033,
0.0418064184486866,
0.012016375549137592,
-0.014051226899027824,
-0.053731463849544525,
0.0003829944471362978,
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0.024440482258796692,
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0.012714812532067299,
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0.00632478715851903,
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0.022831983864307404,
0.04965457320213318,
-0.04132039099931717,
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0.03747956454753876,
0.002071080030873418,
0.05087992548942566,
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0.005567209329456091,
0.002849010517820716,
0.03600548207759857,
0.0028853085823357105,
0.11750960350036621,
0.007441113702952862,
0.06673099845647812,
0.010727331042289734,
-0.032039280980825424,
0.01511759776622057,
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0.08050992339849472,
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0.04179951921105385,
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0.03135458007454872,
0.058007340878248215,
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0.0008167675114236772,
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0.008583426475524902,
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0.016100313514471054,
0.020132575184106827,
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0.006872584577649832,
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0.020381970331072807,
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0.029228217899799347,
0.06248951330780983,
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0.03154423460364342,
0.03232226148247719,
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0.005353677086532116,
0.026948101818561554,
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0.030542073771357536,
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0.040970440953969955,
0.005468876101076603,
0.026141775771975517,
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0.054929573088884354,
0.01663682609796524,
0.06888392567634583,
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0.06340738385915756,
0.03453384339809418,
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0.0051532918587327,
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0.06448639929294586,
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0.011614356189966202,
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0.03462456539273262,
0.03599730134010315,
0.09404775500297546,
0.07481187582015991,
0.001459909020923078,
-0.01380335632711649,
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-0.12019461393356323,
0.02305033430457115,
-0.005518009420484304,
0.0677899643778801,
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0.06287097930908203,
0.03196006640791893,
0.0004944527754560113,
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0.02601010724902153,
0.02690807543694973,
0.053402457386255264,
0.0744401067495346,
0.043864089995622635,
0.013507799245417118,
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0.06361040472984314,
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0.0919002965092659,
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0.04838775470852852,
0.0022745528258383274,
0.02717326581478119,
0.04213693365454674,
0.06072850525379181,
0.01622929982841015,
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0.01778118498623371,
0.03559109941124916,
0.07232546806335449,
-0.0236444603651762,
0.060697950422763824,
0.009900953620672226,
0.10454528778791428,
0.044412340968847275,
-4.90727494195653e-8,
-0.05211276933550835,
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0.02371537685394287,
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0.03542955219745636,
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0.0020119838882237673,
0.0948181003332138,
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0.050040196627378464,
0.07105200737714767,
-0.0757504478096962,
0.010448826476931572,
0.06664379686117172,
0.001548801432363689,
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0.030538173392415047,
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0.07502186298370361,
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0.03203142434358597,
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0.028720984235405922,
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0.0425574891269207,
0.07358257472515106,
0.06417004019021988,
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0.052028488367795944,
0.008678599260747433,
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0.0931558907032013,
0.010459202341735363,
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0.05991455540060997,
0.09350664168596268,
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0.056380536407232285,
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0.05573884770274162,
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0.004239946138113737,
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0.08315622806549072,
0.054688990116119385,
0.02059914544224739,
0.021660489961504936,
-0.01884164661169052,
0.09570174664258957,
0.05753675475716591,
0.10164732486009598,
0.02211468107998371
] |
Poorly\ttreated\tasthma\nAdapted\tfrom\tGupta\tD,\tet\tal:\tGuidelines\tfor\tdiagnosis\tand\tmanagement\tof\tchronic\tobstructive\tpulmonary\tdisease:\tJoint\tICS/NCCP\t(I)\nrecommendations,\t\nLung\tIndia\n\t30:228,\t2013.\nPathophysiology\nCOPD\n\tis\ta\tterm\tthat\tencompasses\t\nchronic\tbronchitis\n\t(a\tlong-term\tcondition\n\tof\tCOPD\tin\twhich | [
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0.09202343970537186,
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0.022318800911307335,
0.05933339148759842,
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chronic\tbronchitis\n\t(a\tlong-term\tcondition\n\tof\tCOPD\tin\twhich\ninflamed\tbronchi\tlead\tto\tmucus,\tcough,\tand\tdifficulty\tbreathing)\tand\t\nemphysema\n\t(a\tform\tof\tlong-\nterm\tlung\tdisease\tcharacterized\tby\tthe\tdestruction\tof\tlung\tparenchyma\twith\tlack\tof\telastic\trecoil).\nThese\tconditions\tmay\tcoexist\tin\tvarying\tdegrees\tand\tare\tgenerally\tnot\treversible. | [
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] |
Fig.\t33.7\n\tshows\nthe\toverlap\tbetween\tthese\tthree\tconditions:\tasthma,\tchronic\tbronchitis,\tand\temphysema.\n\tAsthma-\nCOPD\toverlap\tsyndrome\t(ACOS)\tis\ta\tnew\tentity\tthat\tentails\tthose\twho\thave\tfeatures\tof\tboth\tCOPD\nand\tasthma.\tACOS\twas\trecognized\tin\t2015\tby\tthe\tGlobal\tInitiative\tfor\tChronic\tObstructive\tLung | [
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