Glomerulonephritis in a Child with Liver Cirrhosis

Kazunari Kaneko, MD, Department of Pediatrics, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113 (Japan) Dear Sir, Recently it has been focused that glomerulonephritis with end-stage liver disease (ESLD), which has been termed hepatic glomerulonephritis (HGN), occurs not only in adults, but also in children [1-3]. In terms of renal histology, although Milner et al. [1] have reported that membranoproliferative glomerulonephritis (mesangiocapillary glomerulonephritis; MCGN) is a common finding, Noble-Jamieson et al. [2] and Ohtomo et al. [3] have reported that mesangial proliferative glomerulonephritis with the mesangial deposition of IgA is a predominant histological finding in children with ESLD. Little is known about HGN in terms of prognosis and responsiveness to drug therapy such as prednisone. We wish to report on a young girl with MCGN caused by ESLD who seemed to respond to a short course of prednisone. A 9-year-old girl suffering from ESLD by biliary atresia was recently referred to us by the pediatric surgeon because of the appearance of massive proteinuria and hematuria during followup for her impaired liver function. At the time of referral, her liver function was disturbed by cholestasis. The liver biopsy specimen revealed marked fibrosis. Urinal-ysis demonstrated nephrotic-range proteinuria (4.8-24.0 g/day) and hematuria (+++ by dipstick test) with a few red blood cell casts. Although her renal function was not disturbed (creatinine clearance 111 ml/min/ 1.73 m2), hypoproteinemia (total protein 46 g/l, normal 64-81; serum albumin 23 g/l, normal 4053) and hypocomplementemia (C3 670 mg/l, normal 700-1,300; C4 180 mg/l, normal 200-500) were noted. The serum IgA level was slightly elevated (2,850 mg/l, normal 330-2,360), while the levels of serum IgG and IgM were not increased (9.5 g/l and Fig. 1. a Light microscopic findings showing mesangial cell proliferation associated with capillary wall thickening. Periodic acid-methe-namine. ×200. b Immunofluores-cence microscopic finding, C3 demonstrating marked deposition along the capillary wall in a granular pattern. ×400. 1,170 mg/l, respectively), and cryoglobulin was not detected. Circulating immune complex containing IgA was not detected. A renal biopsy specimen showed mesangial proliferation and capillary wall thickening which are compatible with typical MCGN in all of