[Severe malabsorption syndrome and exudative enteropathy in hypogammaglobulinemia: complete involution with intravenous immunoglobulin substitution].

A 35 year old woman with primary hypogammaglobulinaemia developed intestinal villous atrophy, a severe malabsorption syndrome, osteomalacia and protein-losing enteropathy. The syndrome did not respond to treatment with antibiotics, vitamins, or gluten free diet. Regular intravenous administration of a native immunoglobulin preparation induced continuous elevation of IgG serum levels above 240 mg/dl. This led to rapid, complete and persistent normalisation of all clinical symptoms and pathologic findings. Additional therapy was not required. Side effects of the treatment that has now been maintained for 48 months were not observed.