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Severe Intestinal Lymphangiectasia Complicated by Nephrotic Syndrome Treated by Small Bowel, Liver, and Kidney Transplantation

A girl aged 2 years was examined for a 4-week history of periorbital edema, ankle swelling, and lethargy. Plasma albumin concentration was 13 g/L, with hypogammaglobulinemia (immunoglobulin G [IgG] 0.4 g/L), lymphopenia, and neutropenia (total leukocyte count, 1.3 ×10/L). Lymphocyte subsets indicated severe T-cell depletion with a CD3 count of 5 × 10 (normal count, 500 × 10). Regular immunoglobulin infusions and prophylactic antibiotics were prescribed. Twenty-four–hour urinary protein excretion was normal, but fecal 1antitrypsin levels were elevated at 29.0 g/dL (2 g/dL upper limit of normal), indicating a protein-losing enteropathy (PLE). Lymphangiography demonstrated normal distal intestinal lymphatics. Results of antigliadin, autoantibody screen, and sweat test were negative, and a jejunal biopsy showed dilated intestinal lymphatics. There were no positive isolates from multiple stool microscopy and cultures.