Iványi B, Haszon I, Endreffy E, Szenohradszky P, Petri I B, Kalmár T, Butkowski R J, Charonis A S, Túri S
Department of Pathology, The Clinics of Pediatrics, Albert Szent-Györgyi Medical University, Szeged, Hungary.
Am J Kidney Dis. 1998 Dec;32(6):1068-74. doi: 10.1016/s0272-6386(98)70085-x.
Childhood membranous nephropathy (MNP) with anti-tubular basement membrane (anti-TBM) nephritis is a rare disorder that may have extrarenal manifestations. This article describes a new case to be added to the 10 previously reported. A renal biopsy specimen from a 1-year-old white boy with nephrotic syndrome, microhematuria, and hypertension showed MNP (granular global IgG, IgA and C3, and segmental IgM and C1q) associated with hypercellularity and granular deposits of IgM and C1q in the mesangium, arteriolar IgA, and linear TBM IgG, IgA, and C3. A biopsy at age 4 years showed MNP (IgG and C3) and linear IgG and C3 along the TBM. Six months later, temporary glucosuria suggested a mild tubular dysfunction. Biopsy at age 8 years showed sclerosing MNP (IgG and C3), linear TBM IgG and C3, and chronic active tubulointerstitial nephritis (TIN). Indirect immunofluorescence showed circulating anti-TBM antibodies, and the enzyme-linked immunosorbent assay (ELISA) approach verified strong reactivity with the 58-kd TIN antigen. Despite trials with steroids, chlorambucil, azathioprine, and cyclosporine, end-stage renal disease developed by the age of 9 years. At age 10 years, the patient received a cadaveric kidney transplant. With the patient now aged 12 years, the graft is still functioning well, without any clinical evidence of disease recurrence. Neurological, ocular, and abdominal symptoms, including nonbacterial diarrhea, were observed during the follow-up period. The pathophysiology of these extrarenal symptoms remains unclear. Serotyping and genotyping of HLA antigens (A2, A10, B12, B41, DR5 [1101, 1103-4, 1106 or 1108-1113], DR6 [1303, 1312, or 1413], DRB3 [*0101 and 0201-2 or 0301], DQA1 [*0501 homozygous], and DQB1 [*0301 homozygous]) did not indicate any HLA association similar to those described previously in childhood MNP with anti-TBM nephritis (HLA-B7 in four patients, HLA-DR8 in two patients). The presented case is the fifth in the literature that displays reactivity with the 58-kd TIN antigen, and for which data on HLA antigens are reported.
儿童膜性肾病（MNP）合并抗肾小管基底膜（anti-TBM）肾炎是一种罕见疾病，可能有肾外表现。本文描述了一例新病例，将其添加到之前报道的10例病例中。一名1岁白人男孩，患有肾病综合征、镜下血尿和高血压，其肾活检标本显示为MNP（免疫球蛋白G、免疫球蛋白A和补体C3呈颗粒状全肾沉积，免疫球蛋白M和补体C1q呈节段性沉积），伴有系膜区细胞增多以及免疫球蛋白M和补体C1q的颗粒状沉积、小动脉免疫球蛋白A沉积，以及肾小管基底膜免疫球蛋白G、免疫球蛋白A和补体C3呈线性沉积。4岁时的活检显示为MNP（免疫球蛋白G和补体C3），以及沿肾小管基底膜的免疫球蛋白G和补体C3呈线性沉积。6个月后，出现一过性糖尿，提示轻度肾小管功能障碍。8岁时的活检显示为硬化性MNP（免疫球蛋白G和补体C3）、肾小管基底膜免疫球蛋白G和补体C3呈线性沉积，以及慢性活动性肾小管间质性肾炎（TIN）。间接免疫荧光显示循环抗肾小管基底膜抗体，酶联免疫吸附测定（ELISA）方法证实与58-kD肾小管间质性肾炎抗原具有强反应性。尽管使用了类固醇、苯丁酸氮芥、硫唑嘌呤和环孢素进行治疗，但该患儿在9岁时发展为终末期肾病。10岁时，该患者接受了尸体肾移植。患者现在12岁，移植肾仍功能良好，没有任何疾病复发的临床证据。在随访期间观察到神经、眼部和腹部症状，包括非细菌性腹泻。这些肾外症状的病理生理学仍不清楚。对人类白细胞抗原（HLA）抗原进行血清分型和基因分型（A2、A10、B12、B41、DR5 [1101、1103 - 4、1106或1108 - 1113]、DR6 [1303、1312或1413]、DRB3 [*0101和0201 - 2或0301]、DQA _ 1 [*0501纯合子]和DQB _ 1 [*0301纯合子]），未发现与之前报道儿童MNP合并抗TBM肾炎（4例患者为HLA - B7，2例患者为HLA - DR8）相似 的HLA相关性。本文报道的病例是文献中第五例与58-kD肾小管间质性肾炎抗原呈反应性且报告了HLA抗原数据的病例。
