, Volume 58, Issue 10, pp 2784-2788

Multiple Hepatic Adenomas in a Child with Microvillus Inclusion Disease

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Case Presentation and Evolution

The patient, who was born full term to non-consanguineous parents following a pregnancy complicated only by polyhydramnios, was admitted with severe dehydration, weight loss and diarrhea on the sixth day of life. His electrolytes were significant for sodium 149 mmol/L, potassium 5 mmol/L, chloride 130 mmol/L, and bicarbonate 4.5 mmol/L. For the next 2 months, he suffered recurrent episodes of sepsis and suspected necrotizing enterocolitis. His stool output was secretory and large volume, ranging from 100 to 200 ml/kg/day, and did not change with cessation of enteral feeds. Workup included stool cultures and serum titers negative for enteric bacteria, viral and parasitic infections, normal inflammatory markers, normal immunoglobulins and complement, normal gastrin and urine catecholamines. An endoscopic biopsy at 3 months of life showed total atrophy of the intestinal villi. Periodic acid-Schiff staining revealed targetoid structures in the cytoplasm of ab