Herein we report a new familial form of hepatic disease. Each of the four patients had splenomegaly, hypersplenism, a small liver, biochemical evidence of hepatic excretory dysfunction and hepatocellular damage, kidneys without demonstrable cysts, and normal blood pressure. An evaluation of serum immunoproteins, autoantibodies, histocompatibility antigens, and mixed lymphocyte reactivity further defined the immunologic features of this syndrome. Extrahepatic manifestations included a papulosquamous dermatitis with deposition of immunoglobulins and complement in both normal and abnormal skin, a membranoproliferative glomerulonephritis with subendothelial deposits, arthritis, and pericardial, pleural, and synovial effusions.
Bibliographical noteFunding Information:
From the Departments of Pediatrics and Dermatology, University of Minnesota. Supported in part by the New York State Kidney Disease Institute 40013, National Cancer Institute Grant CA-08748, AI 10704-13, HL-06314, AM 05671-01, AM 01090-02, 5TO1-AM05560-09, and National Foundation 1-375. *Reprint address: Childrens Hospital Medical Center of Northern California, 51 and Grove Sts., Oakland, CA 94609.