Ayşe Öner

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.

Genetic risk factors of amyloidogenesis in familial mediterranean fever

Background/Aims: Evaluation of the risk factors, and phenotype-genotype correlation of familial Mediterranean fever (FMF) gene (MEFV) and serum amyloid A1 (SAA1) gene polymorphisms in renal amyloidosis. Methods: We investigated MEFV and SAA1 genotypes (α, β, and γ isoforms) in 50 FMF patients and 50 healthy children. Tel-Hashomer criteria were used for the diagnosis and severity scoring of FMF. Results: The most common MEFV mutation and SAA1 genotype were M694V/M694V (n = 26/50) and SAA1 α/α (n = 26/50), respectively. Positive family history for amyloidosis was significantly higher (p < 0.001) with more severe clinical course (p = 0.006) in the amyloidosis group than the non-amyloid group. In M694V/M694V mutation, erysipelas-like skin erythema (p = 0.029), arthritis (p = 0.004), arthralgia (p < 0.001) were significantly more frequent with higher severity scores (p = 0.008) than the patients with other mutations. Comparison of the SAA1 α/α genotype with other genotypes revealed more frequent arthritis (p = 0.003) in the SAA1 α/α genotype. In amyloidosis group patients having both M694V/M694V and SAA1 α/α genotypes were the largest subgroup (n = 14, p < 0.001). Logistic regression analysis for amyloidosis corrected risk revealed a 1.2 times increase in M694V/M694V, a 2.4 times increase in SAA1 α/α genotypes and a 2.5 times increase when both are together. Conclusion: Positive family history for amyloidosis and presence of SAA1 α/α genotype in M694V/M694V mutation may predispose to amyloidosis by increasing the clinical severity. Therefore, in such children early colchicine treatment might be recommended even if they are asymptomatic.

Cranial nerve involvement in childhood polyarteritis nodosa

Amongst a variety of neurological manifestations of childhood polyarteritis nodosa, cranial nerve involvement is unusual. We report 4 cases with cranial nerve palsies in a series of 36 biopsy-proven patients. Two cases presented with IIIrd nerve palsy alone, one with right IIIrd and left IVth nerve palsy, and one with peripheral VIIth nerve paresis. All 4 patients showed good response to prednisolone and cyclophosphamide treatment. Cranial nerve involvement in childhood polyarteritis nodosa seems not so rare when patients are followed on long term basis.

Long-Term Efficacy and Safety of Quadruple Therapy in Childhood Diffuse Proliferative Lupus Nephritis

In this study, we evaluated the frequency, clinical presentation, treatment protocols, prognostic factors, and outcome in children with diffuse proliferative lupus nephritis (DPLN). Between June 1990 and December 2004, 46 patients were diagnosed to have systemic lupus erythematosus (SLE), and 26 of them (56.5%) were found to have DPLN. Renal manifestations were present in 25 patients, and the majority of them presented with severe renal findings, such as nephrotic syndrome and renal failure. All patients were given a quadruple therapy protocol including 6–12 monthly courses of methyl prednisolone pulse therapy combined with oral prednisolone, oral cyclophosphamide, azathioprine, and dipyridamole. Nineteen of these patients were regularly followed up with a mean follow-up period of 5.9 years. Complete remission was achieved in 15 of 19 patients, and chronic renal failure developed in four patients. Renal survival rate was calculated to be 78.9% at the end of 5, 10, and 14 years. Although nephrotic range proteinuria, hypoalbuminemia, renal failure, and activity index above 12/24 at presentation seemed to be associated with poor prognosis, no significant difference could be found. Hypertension and chronicity index greater than 6/12 were found to be bad prognostic predictors. We concluded that satisfactory results were achieved with our quadruple therapy protocol; thus, more aggressive and expensive therapies can be avoided and preserved for more serious and persistent diseases.

Henoch Schönlein purpura and amebiasis

Abstract The pathogenesis of Henoch Schonlein purpura (HSP) is unknown but is believed to result from an immune complex reaction to various antigenic stimuli, such as infectious agents. However, its association with Entamoeba histolytica has not been reported before. We present an 11 ‐year‐old boy with HSP, confirmed by the demonstration of leukocytoclastic vasculitis from skin and diffuse endocapillary proliferative glomerulonephritis, together with immunoglobulin A and complement component C3 deposition from renal biopsies. Cysts and trophozoites of Entamoeba histolytica were detected from the stool of the patient at the same time and disappeared after the treatment with metranidasole. The temporal association of these two disorders is either coincidental or due to a causal relationship between them.

Acute poststreptococcal glomerulonephritis followed by acute rheumatic carditis: an unusual case

1. Jakobsson B, Nolstedt L, Svensson L, Soderlundh S, Berg U (1992) 99mTechnetium-dimercaptosuccinic acid scan in the diagnosis of acute pyelonephritis in children: relation to clinical and radiological findings. Pediatr Nephrol 6: 328334 2. Handmaker H (1982) Nuclear renal imaging in acute pyelonephritis. Semin Nucl Med 12:246-253 3. Wikstad I, Hannerz L, Karlsson A, Eklof AC, Olling S, Aperia A (1990) 99mTc DMSA scintigraphy in the diagnosis of acute pyelonephritis in rats. Pediatr NephroI 4:331-334 4. Haycock GB (1986) Investigation of urinary txact infection. Arch Dis Child 6 t: 1155 1158 5, Magil HL (1987) Diagnostic imaging in children with urinary tract infection. S Med J 12: 15571565 6. Lebowitz KL, Mandell J (1987) Urinary tract infection in children: putting radiology in its place. Radiology 165:1 9 7. Thomos V. Shelokov A, Forland M (1974) Antibody coated bacteria in the urine and the site of urinary-tract-infection. N Engl J Med 290: 588-590

Mycophenolate mofetil therapy in a child with Churg–Strauss syndrome

Churg–Strauss syndrome (CSS) is a rare vasculitis of childhood associated with asthma and allergies. It commonly occurs in middle-aged men and very few cases in childhood have been reported. It usually occurs in conjunction with a long history of asthma and other allergic manifestations such as chronic allergic rhinitis, followed by eosinophilia, pulmonary infiltrates, and vasculitis. Not all components may be present at one time and the disease may first occur as a localized disorder. The disease course is often very prolonged and therapy with immunosuppressive agents is usually required for years. We present an 11-year-old boy with CSS who was first admitted to our hospital at 2 years of age. The patient developed severe cutaneous lesions during the follow-up period and was treated successfully with mycophenolate mofetil (MMF).

Necrotizing fasciitis in a child: a rare complication of idiopathic nephrotic syndrome

In nephrotic syndrome there is an increased tendency for bacterial infections due to immunological changes secondary to proteinuria, treatment (including steroids), and other as yet unknown causes. However, necrotizing fasciitis (NF) is an uncommon complication of the disease and has rarely been reported in nephrotic children. We report a 14-month-old boy with nephrotic syndrome who developed sepsis and NF as a complication. He was treated successfully with intensive medical and surgical treatment.

Carbamazepine induced systemic lupus erythematosus: Another warning

Carbamazepine induced systemic lupus erythematosus is a very rare phenomenon. Seven cases have been reported so far. We report another case documented with both clinical and serologic data and discuss some possible variations in related serology.

Crescentic glomerulonephritis in children: a single centre experience

BackgroundCrescentic glomerulonephritis (CsGN) is characterized by crescents in 50% or more of glomeruli and clinically by a sudden and progressive decline in renal function.MethodsWe evaluated the etiology, clinical features, prognostic factors and long-term outcome of CsGN. Between January 2000 and December 2010, 45 children (26 girls, 19 boys) with biopsy-proven CsGN (>50% crescents) were investigated retrospectively.ResultsThe mean age of the patients was 130.86±33.77 months. The mean duration of symptoms prior to diagnosis was 26±12 days (4-40 days). Most of the children had hypertension (62.2%), macroscopic hematuria (73.3%), oligoanuria (44.4%), edema (51.1%) and purpuric rash (40%) at presentation. The final clinical status of the patients was complete remission (n=21), partial remission (n=5) or chronic kidney disease (n=19). Adverse outcomes were significantly associated with a long duration between the onset of symptoms and treatment (P=0.038), the presence of oligoanuria (P=0.006), a severe decreased glomerular filtration rate (GFR <30 mL/min/1.73m²) and the need for dialysis (P=0.003) on admission, the ratio of crescents (>75%) (P=0.03), and the ratio of fibrous crescents (P=0.015).ConclusionThe outcome of CsGN in children continues to be poor, and it should be treated as a renal emergency.