Prediction of kidney survival in children with primary focal segmental glomerulosclerosis (a two-center study)

Background: Focal segmental glomerulosclerosis (FSGS) is one of the most common glomerulopathies in children leading to end stage renal disease (ESRD). Different values of median renal survival have been reported among different ethnicities and races. Many factors are assumed to be responsible for ESRD in these patients. In this study, we tried to determine median renal survival (MRS) and also some clinical and histopathological features predisposing FSGS patients to ESRD in two referral hospitals in Tehran. Methods: The study involved 103 FSGS patients (61 males and 42 females) with a mean age of 7.08 ± 0.68 years. The diagnosis was made based on kidney biopsies. All kidney biopsies were studied by light and immunofluorescent microscopes. Ocular grids (counting squares) were used as the standard method to calculate the percentage of cortical interstitial fibrosis (CIF). The percentage of glomerular sclerosis was presented as renal injury score. Glomerular filtration rate (GFR)�50 ml/min was considered as renal death or the end point. Patients were followed for 1 to 15 years, until occurrence of renal death. Results: The MRS was 9.04 ± 1.8 yrs. The renal survival reached 72, 47 and 17 after 5, 10 and 15 years, respectively. Univariate analysis showed significant reverse correlation (P50, peritubular fibrosis, periglomerular fibrosis, tubular atrophy and CIF�20. However, multivariate analysis revealed only a reverse correlation between renal survival with CIF�20 and also hypertension (P<0.0001 and P<0.05, respectively). Conclusions: In our patients, FSGS showed a rapid course towards ESRD compared with patients of western countries. Perhaps some ethnic and genetic factors such as angiotensin converting enzyme genotypes (ACE-DD) can be considered as a cause of this rapid progression. Also, we carried this study only on children and adolescents which might change the MRS results

Laparoscopic v open donor nephrectomy for pediatric kidney recipients: Preliminary report of a randomized controlled trial

Background and Purpose: Laparoscopic surgery is widely accepted for nephrectomy in adult renal transplantation. The success of this technique has not been compared with open donor nephrectomy (ODN) in children. Patients and Methods: In this randomized clinical trial, 40 adult kidney donors were randomly divided into two groups: 20 cases of laparoscopic donor nephrectomy (LDN) and 20 of ODN. Recipients had an age of <15 years. Our exclusion criteria were previous renal transplantation, hemolytic uremic syndrome, focal segmental glomerulosclerosis, oxalosis in the recipients, and multiple renal arteries bilaterally in donors. Results: All donor nephrectomies were completed as scheduled, and no patients undergoing LDN required conversion to open nephrectomy. No patients in either the ODN or the LDN group required reoperation. Acute rejection was diagnosed in six patients receiving kidneys procured by ODN (30) and 4 patients (20) receiving kidneys obtained by LDN (P = 0.3). No recipients or donors died. At 1 year, the graft survival times in the ODN and LDN groups were 310.8 ± 28.8 and 302.7 ± 28.2 days, respectively (P = 0.8). Conclusion: At our medical center, pediatric LDN recipients had graft outcomes similar to those of ODN recipients. We recommend LDN for harvest of kidneys for pediatric recipients at experienced centers. © 2007 Mary Ann Liebert, Inc

Angiotensin-converting enzyme gene polymorphism and the progression rate of focal segmental glomerulosclerosis in Iranian children

Aim: Focal segmental glomerulosclerosis (FSGS) is one of the most common forms of glomerulonephritis leading to end-stage renal disease (ESRD). A few clinical and paraclinical factors are considered as contributing factors in progression rate. However, there are controversial reports on the relationship between ACE gene polymorphism and rapidity of progression of FSGS to ESRD in different populations. To elucidate this issue, we investigated the relationship between the insertion (I) and deletion (D) ACE gene polymorphism and rapidity of progression of FSGS to ESRD in Iranian children. Methods: Forty-one children aged 1-18 years admitted to St AlZahra Hospital, Isfahan, and St Ali Asghar Hospital, Tehran, Iran, with idiopathic FSGS were enrolled. Renal death was defined as a glomerular filtration rate (GFR) of less than 50 mL/min per 1.73 m2 or a decreased GFR to less than 50 compare to baseline. Reaching renal death in less or more than 2 years were labelled as rapid progressors (RP) or slow progressors (SP), respectively. Intron 16 of the ACE gene was amplified by the polymerase chain reaction technique. Results: Twenty-eight patients were male and 13 were female. In 15 RP patients, the genotype distribution was 26.6 DD, 6.7 II and 66.7 ID. In 26 SP patients, the genotype was similar (38.6 DD, 7.6 II and 53.8 ID, P &gt; 0.05). There were no statistically significant differences for ACE I/D gene polymorphism between the two groups of patients (P &gt; 0.05). Conclusion: Our study revealed no correlation between ACE I/D gene polymorphism and rapidity of progression of FSGS to ESRD in Iranian children. © 2008 The Authors