We have read with interest the Letter to the Editor by Simsek Bulos N, Shahin HM. Familial Mediterranean fever in children: and colleagues [1] reporting the efficacy of colchicine in a the expanded clinical profile. QJM e d1999; 92: 309-318 nephrotic paediatric patient with amyloidosis due to familial 6. Sarkissian A, Papazian M, Sanamiam A, Babloyan A. Does Mediterranean fever (FMF ) with resolution of proteinuria. colchicine alter the course of amyloidosis in children with familial Mediterranean fever?

Amyloidosis is not the only renal involvement in FMF pediatric patients.The purpose of this study is to evaluate the renal biopsy results and look for the trends over the time in FMF children. MethodsFrom 1993 to 2014 renal biopsies were done in 83 FMF patients with renal involvement (mean age 12±4 year; range 2.2-18; 45 males) at "Arabkir" MC -ICAH. The diagnosis of FMF based on international clinical criteria and genetic analysis.

Introduction and Aims:The most severe complication of FMF is potentially preventable amyloid nephropathy (AN), leading to end stage renal disease (ESRD). The short-and long-term results of kidney transplantation (Tx) in these patients ( pts) have not sufficiently been studied. The aim of this study is to evaluate results of Tx in pts with FMF and AN. Methods: 15 pts with FMF and ESRD presumably due to AN were evaluated, corresponding to 12.6% of all transplanted from living-related donors at our centre between 1995 and 2014. Diagnosis of FMF and AN was based on typical clinical presentation, genetic analysis (in 9) and native kidney biopsy (in 3, all revealing amyloidosis by Congo Red stain). Mean age was 36.5 ± 11.3 years (20.3-52.3), 10 men. The median duration of haemodialysis (HD) was 7.1 months. Six patients had blood components transfusion (4) or pregnancies (2) as a risk for sensitization. The donors' mean age was 42.7 ± 8.2 years (28.6-58.2), 9 women. Immunosuppression (IS) consisted of prednisolone, cyclosporine A (CsA) or tacrolimus and antimetabolites (azathioprine or mycophenolate mofetil (MMF). Induction therapy was used selectively, mainly in case of sensitization (n=3). Colchicine was added after 1 week in all, starting with 0.5 mg/day. Results: Twelve pts are followed up at our center; one emigrated, one returned to HD (loss of Tx function due to reduced IS because of pulmonary tuberculosis) and one died 1 month after Tx from systemic amyloidosis. One-and 5-year graft and pts' survival was 92.3% and 92.9%, respectively. Mean serum creatinine in patients with a functioning graft was 113 ± 26.3 μmol/l (75-161), eGFR by CKD-EPI formula is 67.8 ± 21.8 ml/min (33.6-97.1). Acute rejection, observed in 8 patients (5 with one and 3 with repeated episodes) resolved in 6 with methylprednisolone (MP).Two received ATG, one lost his graft. Complications included acute tubulonecrosis (2), lymphocele (2) and urethral stricture, transient hyperglycemia, CMV disease, pulmonary tuberculosis, basal cell carcinoma, distal ureteral necrosis (1 each). FMF attacks occurred in 11 (73.3%) pts. Side effects of colchicine combined with IS were diarrhea (8) and severe myalgia (3), requiring dose adjustment. Two pts developed frequent FMF attacks after conversion from CsA to tacrolimus, requiring increased colchicine dosage. Recurrent renal amyloidosis was diagnosed in one after 2.4 years. Earlier biopsies in 5 other patients did not show AN. No patient had proteinuria exceeding 0.5 g/l. Comparison of Tx results in pts with FMF versus those without FMF revealed no significant differences in the incidence of acute rejection ( p>0.05), Tx and patients survival (log rank >0.05) and the spectrum of complications. Conclusions: Tx in carefully selected patients with FMF is as effective as in recipients with other diseases. The main problem is the combination of the basic IS medications with colchicine, which requires careful titration of individual doses.

The spectrum of pathology in native kidney biopsies varies considerably between different countries. Based on similar biopsy policy and joint workup, biopsy data of native kidneys of children in Yerevan (Armenia) and Zurich (Switzerland) were compared over a period of two decades (1993-2002 and 2003-2012). A total of 487 renal biopsies in Yerevan (EVN), n = 253; median age 11.2 years (range 0.8-18; 56 % males) and in Zurich (ZRH), n = 234; median age 8.7 years (range 0.1-18; 61 % males) were analyzed. Biopsies from EVN were locally analyzed by light microscopy (LM) and sent to ZRH for electron microscopy (EM) and immunohistochemistry. Biopsies from ZRH were evaluated by LM, EM, and immunofluorescence. The significant difference concerns the high frequency of amyloidosis in EVN (25.4 % in the first and 19.4 % in the second decade vs. 0 % in ZRH) and of IgA nephropathy in ZRH (30.2 % in the first and 26.1 % in the second decade vs. 8.1 in EVN). Certain forms of glomerulonephritis (membranoproliferative type I and membranous) and primary focal segmental glomerulosclerosis tended to be more frequent in EVN than in ZRH. Amyloid nephropathy due to familial Mediterranean fever is still highly frequent in Armenia with a slight decrease in the second decade. In Switzerland, the most common finding was IgA nephropathy.