TY - JOUR
T1 - The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis
AU - Dobrin, Robert S.
AU - Hoyer, John R.
AU - Nevins, Thomas E
AU - Sharp, Harvey
AU - Gentry, William C.
AU - Vernier, Robert L.
PY - 1977/6
Y1 - 1977/6
N2 - 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.
AB - 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.
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U2 - 10.1016/S0022-3476(77)80556-8
DO - 10.1016/S0022-3476(77)80556-8
M3 - Article
C2 - 870658
AN - SCOPUS:0017503984
VL - 90
SP - 901
EP - 909
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
IS - 6
ER -