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A quiz is presented related to 10 year old patient with an unusual cause of diarrhea in a child with nephrotic syndrome.

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS).Methods: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically.Results: Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ≥ 2, compared to patients with FFS < 2. Patients with FFS ≥ 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007).Conclusions: Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis. [ABSTRACT FROM AUTHOR]

The article describes the case of steroid-resistant nephrotic syndrome in a child. Highlights include exclusion of Familial Mediterranean fever (FMF) through FMF mutation analysis, the level of urine mevalonic acid during an attack, and discontinuation of steroid and colchicine treatments after confirmation of the diagnosis.

The prognosis of Henoch-Schönlein purpura (HSP) nephritis is more severe than originally thought, with a significant portion progressing to deterioration of renal function in adulthood. Proteinuria adversely affects the outcome. The aim of this study was to evaluate the initial single-center results of a treatment protocol for severe HSP nephritis based on the Heaton classification. Age, gender, clinical features and duration of disease follow-up were assessed. Glomerular filtration rate (GFR), urinalysis and 24-hour urinary protein excretion were analyzed. All patients with severe renal involvement were biopsied and a treatment plan was assigned: Class II received oral steroids, Class III (with crescentic nephritis) received additional oral cyclophosphamide 2 mg/kg/d for 12 weeks, and Classes IV and V received azathioprine for 9 months subsequent to the treatment for Class III. All patients received angiotensin converting enzyme (ACE) inhibitors regardless of their blood pressure values. Eighteen patients presenting with severe HSP nephritis, defined as heavy proteinuria and/or decreased renal function, were evaluated. Based on the renal histology, 5, 10, 1 and 2 of the patients were classified as Classes II, III, IV and V, respectively. At presentation, 7 of the patients had impaired renal function with GFR below 75 ml/min/1.73 m2. With the presented treatment schema, all GFR returned to normal at the end of four years of follow-up. There was no proteinuria in any of the patients; only 8 had microscopic hematuria. This preliminary study suggests a stepwise treatment according to the renal histology. The excellent results with complete disappearance of proteinuria and nonnal renal function justify the use of the aforementioned immunosuppressive protocol with ACE inhibition. Long-term, multicenter controlled studies are needed to verify our results. [ABSTRACT FROM AUTHOR]

The article describes the case of an eight-year-old girl diagnosed with nephrotic syndrome. Highlights include the response of the patient to steroid treatment, remarkable ascites and increased parenchymal thickness according to abdominal ultrasonography, and acellular eosiophilic material deposition in glomerulus.