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A Guatemalan child with a history of meconium - iren - Printable Version

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A Guatemalan child with a history of meconium - iren - ArchivalUser - 09-08-2007

A Guatemalan child with a history of meconium ileus is brought in to a clinic because of a chronic cough. The mother notes a history of respiratory tract infections and bulky, foul-smelling stools. After assessment of the respiratory tract illness, the practitioner should also look for signs of


A. cystinuria

B. hypoglycemia

C. iron deficiency anemia

D. sphingomyelin accumulation

E. vitamin A deficiency




0 - ArchivalUser - 09-08-2007

E!!!


0 - ArchivalUser - 09-08-2007

yes dz is Cystic Fibrosis.. deficiency in fat soluble vitamens K, A, D, E


0 - ArchivalUser - 09-08-2007

The correct answer is E. The child likely has cystic fibrosis. In this disorder, an abnormality of chloride channels causes all exocrine secretions to be more viscous than normal. Pancreatic secretion of digestive enzymes is often severely impaired, with consequent steatorrhea and deficiency of fat-soluble vitamins, including vitamin A.



Cystinuria (choice A) is a relatively common disorder in which a defective transporter for dibasic amino acids (cystine, ornithine, lysine, arginine; COLA) leads to saturation of the urine with cystine, which is not very soluble in urine, and precipitates out to form stones.



Hypoglycemia (choice B) is not a prominent feature of children with cystic fibrosis who are on a normal diet. Hyperglycemia may occur late in the course of the disease.



Iron deficiency anemia (choice C) is not typically found in children with cystic fibrosis.



Sphingomyelin accumulation (choice D) is generally associated with deficiency of sphingomyelinase, as seen in Niemann-Pick disease.