The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis

Robert S. Dobrin; John R. Hoyer; Thomas E Nevins; Harvey Sharp; William C. Gentry; Robert L. Vernier

doi:10.1016/S0022-3476(77)80556-8

The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis

Robert S. Dobrin, John R. Hoyer, Thomas E Nevins, Harvey Sharp, William C. Gentry, Robert L. Vernier

Pediatrics - Nephrology

Research output: Contribution to journal › Article › peer-review

2 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	901-909
Number of pages	9
Journal	The Journal of pediatrics
Volume	90
Issue number	6
DOIs	https://doi.org/10.1016/S0022-3476(77)80556-8
State	Published - Jun 1977

Bibliographical note

Funding 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.

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title = "The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis",

abstract = "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.",

author = "Dobrin, {Robert S.} and Hoyer, {John R.} and Nevins, {Thomas E} and Harvey Sharp and Gentry, {William C.} and Vernier, {Robert L.}",

note = "Funding 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.",

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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.

N1 - Funding 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.

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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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The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis

Abstract

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