A 3-year-old child who underwent Kasai portoenterostomy for biliary atresia now has progressive fatigue and icterus. Examination reveals loss of ankle jerks and truncal ataxia. Hemoglobin is 7 g/dL with reticulocytes 8%, smear shows fragmented cells and acanthocytes, and direct Coombs test is negative. Which deficiency best explains all these findings?

• A Vitamin K
• B Vitamin E ✓
• C Essential fatty acid
• D Copper

Explanation

Vitamin E is fat soluble, so chronic cholestasis after biliary atresia leads to deficiency. Vitamin E protects red cell membranes, so deficiency produces a Coombs-negative hemolytic anemia with acanthocytes, while its antioxidant role in the nervous system produces posterior column and spinocerebellar dysfunction with areflexia and ataxia. Vitamin K deficiency prolongs PT but does not cause hemolysis or neuropathy.

Reference: Nelson Textbook of Pediatrics, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.