Keriman Tinaztepe

The effect of triple therapy on rapidly progressive type of Henoch-Schönlein nephritis

Twelve patients with Henoch-Schönlein purpura, aged 6–14 years (mean 10.3 years), presenting with rapidly progressive glomerulonephritis (RPGN) were investigated prospectively. Analysis of the initial clinical features revealed: oedema (8 patients), hypertension (7 patients), gross haematuria (11 patients), oliguria (5 patients) and a decreased glomerular filtration rate (GFR) (<40 ml/min per 1.73 m2, 8 patients). Renal biopsies were available in 9 patients and revealed focal necrotising and a fibroepithelial type of crescentic glomerulonephritis (with 60%–90% crescent formation). The remaining 3 patients fulfilled the clinical criteria of RPGN. Two patients who were in the acute stage required peritoneal dialysis for a period of 2 weeks. The treatment protocol in all patients consisted of intravenous pulse methylprednisolone (3 days), oral cyclophosphamide (2 months), oral dipyridamole (6 months) and oral prednisolone (3 months). At the end of triple therapy, GFR returned to normal in all but 1 patient. During a follow-up period of 9–39 months, 7 patients achieved complete remission, while 4 patients showed partial remission, 3 of whom had persistent proteinuria and haematuria and 1 microscopic haematuria only. One patient had persistent nephropathy with decreased GFR and macroscopic haematuria and nephrotic-range proteinuria. His renal biopsy, performed 30 months after the onset of the disease, showed chronic diffuse sclerosing glomerulonephritis and intratubular severe IgA deposition. Although our patient group was small, this type of intensive treatment appears to be effective; further studies are needed.

Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children

Abstract Renal involvement is common in childhood polyarteritis nodosa (PAN). We report a retrospective analysis of the presentation and clinical course of 26 patients with PAN and renal involvement. The mean age was 9.3 years (range 1–14 years) and there were 12 boys and 14 girls. Renal symptoms at presentation were as follows: 3 had isolated proteinuria, 9 had nephritic syndrome, 2 had nephritic and nephrotic components, and 10 had renal failure with one of the above features. Two patients with isolated hypertension were diagnosed by angiography and classified as classical PAN. Patients either received prednisone p.o. alone (n=9), or prednisone plus cyclophosphamide p.o. (n=11), or pulse steroids with prednisone p.o. and cyclophosphamide (n=2); 4 did not receive any treatment. Patients who were given cyclophosphamide had a significantly better outcome than those who did not. We suggest that oral cyclophosphamide therapy and corticosteroids are effective in the treatment of PAN. The overall 1-year and 5-year survival rates of the patients were 72.5% and 60%, respectively. In conclusion, renal disease is a serious manifestation of PAN necessitating prompt and aggressive treatment.

Urinary Tumor Necrosis Factor Levels in Primary Glomerulopathies

In the present study, urinary tumor necrosis factor-alpha (TNF) levels in nonproliferative glomerulopathies [minimal change disease (n = 4), focal glomerulosclerosis (n = 4), membranous glomerulonephritis (GN) (n = 1), and in patients with chronic glomerulopathies (n = 4)] were compared to proliferative ones [a rapidly progressive GN patient and 8 patients with mesangial proliferative GN and membranoproliferative GN (MPGN) who had clinically active disease]. The mean urine TNF levels of the proliferative group were significantly higher than both the nonproliferative GN and 4 controls, whereas the mean value of the nonproliferative group was not significantly different than the controls. The urine TNF levels in 4 MPGN patients with chronic disease and in 2 who entered remission were also very low. In the patients with active renal disease and cellular proliferation there were significant correlations between the urinary TNF levels and both proteinuria and the clinical activity scores. We suggest that in human proliferative glomerulopathies TNF may be implicated in the glomerular inflammation.

Diffuse Mesangial Sclerosis: A Unique Type of Congenital and Infantile Nephrotic Syndrome

Clinical and pathological findings in four Turkish infants with isolated diffuse mesangial sclerosis (DMS) are presented. All the patients were offsprings of consanguineous marriages and two had similarly affected sibs indicating an autosomal recessive inheritance. The onset of the nephrotic syndrome was at 7, 17, 11 and 3 months of age. They all died in a state of renal failure complicated by infections at the ages of 11, 33, 13 and 5 months. DMS was diagnosed at postmortem examination in all. Fluorescence-microscopical studies in all and an electron-microscopical study in one revealed nonspecific findings. The shorter survival in three of the cases was thought to be due to intervening infections. The variation of the clinical features along with the fluorescence and electron-microscopical findings are consistent with the previously mentioned heterogeneous aspect of DMS.

Successful renal transplantation in a child with ANCA-associated microscopic polyangiitis

Crescentic glomerulonephritis (CGN) is a clinicopathologic entity which is characterized by severe renal dysfunction of rapid onset with glomerular crescents. Type III CGN is associated with the absence of glomerular immune complex deposition (pauci-immune) and is associated with antineutrophil cytoplasmic antibody (ANCA). Microscopic polyangiitis and idiopathic pauci-immune necrotizing glomerulonephritis (NCGN) are strongly associated with ANCA directed against myeloperoxidase (anti-MPO). We describe here an unusual pediatric patient with MPO-ANCA-associated rapidly progressive glomerulonephritis (RPGN), emphasizing the management and outcome of the disease.

Familial membranoproliferative glomerulonephritis

Four and two male sibs of two separate families who had biopsy-proven membranoproliferative glomerulonephritis (MPGN) are presented. In the first family four sibs of the first-degree consanguineous marriage showed the clinical picture of nephrotic syndrome without hypocomplementaemia at initial laboratory findings. In the second family two affected sibs showed nephrotic and nephritic syndromes on admission. Family investigations showed normal serum complement, immunoglobulins, T-cell subsets, urine analysis, and serum biochemistry. HLA typing in the two families revealed a common antigen HLA A2 in all affected sibs. Some other reports give suggestive evidence of MPGN in siblings but this is the first report that showed the occurrence of MPGN in four sibs. Our data strengthened the concept that genetic factors are involved in the development of MPGN but additional immunogenetic studies will shed light on the genetic aspects of the disease.

Membranoproliferative glomerulonephritis in childhood: Factors affecting prognosis

Membranoproliferative glomerulonephritis (MPGN) is a distinctive form of chronic glomerulonephritis. We present the results of our 96 paediatric patients with MPGN, reporting the survival and factors affecting prognosis in these patients. There were 64 boys and 32 girls with an age range of 2–17 (mean 10.6±3.7) years. All patients initially received oral corticosteroid therapy; remission was achieved in 22.9%. The unresponsive 77.1% either received cyclophosphamide and/or pulse methylprednisolone; 25.4% and 50.0% of these patients entered complete remission, respectively. The overall 1-year renal survivals of the MPGN patients were 90.1%, 5-year and 10-year survival rates were 81.9% and 61%, respectively. At multivariate analysis the factors affecting renal prognosis were haematuria at presentation (p<0.05, risk factor 3.52), urinary protein/creatinine ratio (p<0.05, risk factor 1.06 per 1 unit) and low haemoglobin values (p<0.05, risk factor 1.43 for each 1 g/dl decrement). We suggest that more aggressive immunosuppressive therapy should be instituted in patients unresponsive to steroids and that the aforementioned risk factors are higher for the development of renal failure.