AIIMS May 2011 – MCQ 72
A child presents with failure to thrive with frequent vomiting, diarrhoea, hepatic splenomegaly and abdominal distension. CT shows adrenal calcification. Which of the following is the diagnosis?
A. Adrenal haemorrage
B. Wolmans disease
C. Pheochromocytoma
D. Addison’s disease
Correct answer: B. Wolmans disease
4 Comments
wolmans disease
wolmans disease
signs and symptoms of Wolman disease usually appear shortly after birth, typically in the first few weeks of life. Affected infants may have the following:
* Feeding difficulties with frequent vomiting
* Diarrhea (loose frequent stools)
* Swelling of the abdomen (abdominal distention)
* Enlargement of the liver (hepatosplenomegaly) and spleen (splenomegaly)
* Failure to gain weight or sometimes weight loss
A distinct finding associated with Wolman disease is the accumulation of chalky material (calcification) in the adrenal gland.
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* Calcification of the adrenal glands is not detectable by physical examination, but can be seen with an X-ray, CT scan or other imaging investigations
wolmans [repeat nov 2009]
yes it is wolman disease w occure in early infancy but when occur later it is called cesd