Case Of the Week (COW) 24 August 2014
Answer:
Wolman Disease.
Findings:
Xray - bilateral adrenal calcification with retained triangular configuration, hepatosplenomegaly.
Discussion:
Wolman disease is due to abiochemical abnormality of acid lipase /acid esterase deficiency resulting in lipid ester accumulation in adrenal, liver, spleen, lymphnodes and small bowel.
Contributed By:
Prof Vijayalakshmi, Dr Natarajan Dr Jeya Rajaiah, Dr Kasi Visalatchi, Dr Narendran
Institute of Child Health, Chennai, India
Answer:
Wolman Disease.
Findings:
Xray - bilateral adrenal calcification with retained triangular configuration, hepatosplenomegaly.
Discussion:
Wolman disease is due to abiochemical abnormality of acid lipase /acid esterase deficiency resulting in lipid ester accumulation in adrenal, liver, spleen, lymphnodes and small bowel.
Contributed By:
Prof Vijayalakshmi, Dr Natarajan Dr Jeya Rajaiah, Dr Kasi Visalatchi, Dr Narendran
Institute of Child Health, Chennai, India