Exocrine pancreatic insufficiency is seen in:: (A) Shwachman-Diamond syndrome, (B) Diamond-Blackfan syndrome, (C) Rubinstein-Taybi syndrome, (D) Seckel syndrome
Answer is A. Disorders associated with pancreatic insufficiency include: Cystic fibrosis Shwachman-Diamond syndrome Pearson syndrome Johanson-Blizzard syndrome Chronic pancreatitis & protein-calorie malnutrition . Shwachman-Diamond syndrome- autosomal recessive syndrome with pancreatic insufficiency, neutropenia, neutrophil chemotaxis defects, metaphyseal dysostosis, failure to thrive & sho stature.