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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 945-946
Radiological quiz - abdomen

The Institute of Child Health, 11, Dr. Biresh Guha Street, Kolkata: 700017, India

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Date of Submission28-Aug-2006
Date of Acceptance10-Nov-2006

How to cite this article:
Thapa R. Radiological quiz - abdomen. Indian J Radiol Imaging 2006;16:945-6

How to cite this URL:
Thapa R. Radiological quiz - abdomen. Indian J Radiol Imaging [serial online] 2006 [cited 2020 Dec 5];16:945-6. Available from:
A four-month-old male child born to non-consanguineous parents presented with gradually increasing abdominal distention since one month of age along with occasional episodes of diarrhea and vomiting since age of 2 months. Two other siblings were normal. On examination, hepatomegaly to about 8 cms below the right costal margin was noted while the spleen was palpable 5 cms below the left costal margin. Both the liver and the spleen were firm, sharp and non-tender. Neurological examination revealed grossly delayed developmental milestones and hypotonia. Examination of the other systems was normal. Investigations revealed hemoglobin of 10.2 gm %, SGOT-145 U/L, SGPT--238 U/L, serum triglycerides-184 mg % and cholestrol-172 mg %. Ultrasonography confirmed hepatosplenomegaly. Plain radiograph of the abdomen [Figure - 1] showed symmetrically enlarged and calcified adrenal glands on both sides. Liver biopsy revealed extensive fatty change with PAS positive material, which was digested by diastase without evidence of cholestasis or cirrhosis. The estimation of acid lipase enzyme showed zero level in the patient.

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1.Abramov A, Schorr S, William S, Wolman M. Generalized xanthomatosis with calcified adrenals. Am J Dis Child 1956; 91: 282-286.  Back to cited text no. 1    
2.Assmann G, Seedorf U. Acid Lipase deficiency: Wolman disease and cholesterol ester storage disease. In Scriver CR, Beaudet AL, Sly WS. Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. McGraw Hill & Co, New York; 1998; 2563-2587.   Back to cited text no. 2    
3.Wolman M, Sterk VV, Gatt S, Frenkel M. Primary familial xanthomatosis with calcified adrenals -Report of two more siblings of a described case. Pediatrics 1961; 28: 742-746.   Back to cited text no. 3  [PUBMED]  
4.Marshall WC, Ockenden BG, Fosbrooke AS, Cumings JN. Wolman disease. A rare lipidosis with adrenal calcification. Arch Dis Child 1969; 44: 331-341.   Back to cited text no. 4    
5.Krivit W, Freese D, Chan KW, Kulkarni R. Wolman's disease: a review of treatment with bone marrow transplantation and considerations for the future. Bone marrow transplant 1992; 10(suppl 1): 97-101.   Back to cited text no. 5  [PUBMED]  

Correspondence Address:
R Thapa
The Institute of Child Health, 11, Dr. Biresh Guha Street, Kolkata: 700017
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-3026.32394

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