History of dislike for sweet food items is typically present in:
Ans. is 'c' i.e. Hereditary fructose intoleranceRef.: Harrison 17th/e p 2458 & 16th/e p 2321 (15th/e p2289) In hereditary fructose intolerance patients are healthy and asymptomatic until fructose or sucrose (table sugar) is ingested. With the ingestion of sugar severe clinical symptoms appear. Which are -JaundiceHepatomegalyVomitingLethargyIrritabilityConvulsionsProteinuriaHereditary fructose intolerance is caused by the deficiency of Aldolase B.Aldolase B catalyzes the following reactionFructose - 1 - phosphate Aldolase B Glyceraldehyde* Dihydroxyacetone phosphate Deficiency of this enzyme causes rapid accumulation of fructose -1- phosphate and initiates severe toxic symptoms when exposed to fructose.Diagnosis -It is diagnosed by the presence of reducing substance (fructose) in the urine.The appearance of hypoglycemia and hypophosphatemia after fructose (200 mg/kg) loading is diagnosticTreatment:Treatment consists of eliminating fructose from the dietAs affected individuals grow up they may recognize the association of nausea and vomiting with fructose containing foods and selectively avoids them
Diabetes mellitus
Glycogen storage disease
Hereditary fructose intolerance
Galactosemia
Disorder of Metabolism & Connective Tissue