Childhood membranous nephropathy, circulating antibodies to the 58-kD TIN antigen, and anti-tubular basement membrane nephritis: an 11-year follow-up
| Autor: | AS Charonis, IB Petri, I Haszon, Sándor Túri, P Szenohradszky, RJ Butkowski, Béla Iványi, Emöke Endreffy, Tibor Kalmár |
|---|---|
| Rok vydání: | 1998 |
| Předmět: |
Male
Pathology medicine.medical_specialty Nephrotic Syndrome Genotype Biopsy Telomere-Binding Proteins Kidney Glomerulonephritis Membranous Antibodies End stage renal disease Membranous nephropathy HLA Antigens medicine Humans Microhematuria Child Membrane Glycoproteins medicine.diagnostic_test business.industry Histocompatibility Testing Glomerulonephritis medicine.disease medicine.icd_9_cm_classification Combined Modality Therapy Kidney Transplantation Tubulointerstitial nephritis antigen Nephrology Immunology Antigens Surface Mesangial proliferative glomerulonephritis Nephritis Interstitial Drug Therapy Combination Renal biopsy business Nephritis Cell Adhesion Molecules Follow-Up Studies |
| Zdroj: | Scopus-Elsevier |
| ISSN: | 1523-6838 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|