A case of membranoproliferative glomerulonephritis accompanied with hyper IgM immunodeficiency Kenichiro SHIGEMOTO, Koji WADA, Naoki HAMAGUCHI, Akira HIRABAYASHI, Shunji TOI, Koji USUI, Michiko ARITA, Noriaki YORIOKA and Michio YAMAKIDO The Second Department of Internal Medicine, Hiroshima University School of Medicine Jotaro HATA. The First Department of Pathology, Hiroshima University Scool of Medicine, Hiroshima
1) Urlne analysls 7)Serologlcal exam. 1 1)ECG W.N.L. sugar 0% CRP (-) urobillnogen N(+) RA (+) 12)Renal function albumin 3(+) ASLO (-) PSP test 15' 40% occult (-) ASK ~2560 total 73% sediment n.p. DNA (-) GFR 78ml/min B-J protein (-) Wa-R (-) RPF 650ml/min urine volume HBs Ag (-) RBF 1118ml/min 1200 `2000ml/day HBs Ab (-) Ccr 78.7ml/min daily urinary protein Thyrold test (-) Fishberg 1.027 3.5 `5.0g/day Mlcrosome test (-) BUN 14mg/dl 2) Stool Paul-Bunnell ~896 Creatlnin 0.8mg/dl occult (-) Cold agglutination ~1024 Urlc acid 3.9mg/dl 3) CBC Toxoplasma ~256 Na 142 meq/l RBC 454 ~10 4/mm3 Typhus O ~40 K 3.9mEq/L WBC 9600/mm3 Paratyphus AO ~80 Cl 104mEq/L (St 10, Seg 48. Ly 28, Mo 14) 8)Chest X-P n.p. Ca 4.3 meq/l Hb 14.1g/dl 9)Whole body bone X-P n.p. P 3.1mg/dl Ht 41.9% 10)Whole body Ga sclntigram Pit 27.3 ~10 4/mm3 n.p. I.V.P. n.p. 4) BSR 62/86mm 5) Blood coagulation exam. 13) Immunological exam. bleeding time 1'30" Serum protein a1-at 257mg/dl PT 233% Alb 57.4% a1-ag 75mg/dl PTT 47.8sec a1-gl 5.0% a2-hs 92mg/dl Fibrinogen 310mg/dl a2-gl 19.7% a2-macro 432mg/dl FDP 5ƒÊg/ml ƒà-gl 11.4% a2-pag(sp3) 7.3mg/dl Urine FDP 0.25ƒÊg/ml ƒá-g1 6.5% (N:4.2 «) 6)Blood chemical exam. Immunoglobulin C3 106mg/dl TP 5.5g/dl IgG 30mg/dl C4 30mg/dl Alb 2.9g/dl IgA 10mg/dl CH50 31U/ml A/G 1.13 IgM 2052mg/dl IgG-IC 1ƒÊg/ml T.Bil 0.6 mg/dl IgE(RIST) 330IU/ml ZnTT 2U/L IgD 7.2mg/dl I gm-ic 11 ƒêg/ml GOT 15U/L B cell function Control GPT 7U/L IgG production 160mg/ml 2711mg/ml LDH 368U/L IgA production 3.6mg/ml 8.6mg/ml Ch.E 526U/L Lymphocyte subpopulation Al-p 56 U/L a -Leu 1 (Total T cell) 68.5% (50.1 `72.5) CPK FBS 36U/L 89mg/dl a-leu 2a (Suppressor T cell) 23.5%(14.6 `32.2) 285mg/dl a-leu 3a (Helper T cell) 40.8%(31.2 `60.8)TC 352mg/dl a-hla-dr (B cell+monocyte) 7.0%(7.3 `23.9)TG (Natural Killer 21.0%(8.0 `22.0) a -Leu 7 cell) PPD 0.17 ~ƒêg/ml: 0 ~0/5 ~7mm PHA 37.8% 10ƒÊg/ml: 0 ~0/30 ~30mm
10) Goldman AS, et al: Dysgammaglobulinemic antibody deficiency syndrome: Increased ćm - globulins and decreased ćg-and ća-globulins. J Pediatr 70: 16, 1967. 11) Levitt D, et al: Hyper IgM immunodefici ency: A primary dysfunction of B-lymphocyte isotype switching. J Clin Invest 72: 1650, 1983. 12) Geha RS, et al: Hyper immunoglobulin M immunodeficiency (Dysgammaglobulinemia): Presence of immunoglobulin M-secreting plas macytoid cells in peripheral blood and failure of immunoglobulin M-immunoglobulin G switch in B-cell differentiation. J Clin Invest 64: 385, 1979. 13) Mitsuya H, et al: Evidence for the failure of IgA specific T helper activity in a patient with immunodeficiency with hyper IgM. J Clin Lab Immunol 2: 337, 1979. 14) Krantman HJ, et al: Abnormal B cell differentiation and variable increased T cell suppression in immunodeficiency with hyper 1981. -IgM. Clin Exp Immunol 40: 147, 1980. 15) Salcedo DP, et al: Cellular basis of hyper IgM immunodeficiency. J Clin Lab Immunol 10: 29,