Long-Term Outcomes of Kidney Transplant Recipients With Juvenile Nephronophthisis.

OBJECTIVES: Nephronophthisis is the most common genetic cause of kidney failure in childhood. Treatment for nephronophthisis is symptomatic, and kidney transplant is a good treatment option when kidney failure has developed. We reported the outcomes of kidney transplant recipients with primary diagnosis of juvenile nephronophthisis who were followed-up in our center. MATERIALS AND METHODS: We retrospectively examined medical records of 17 kidney transplant patients with a primary diagnosis of juvenile nephronophthisis. We compared this group of 17 patients with kidney transplant recipients who had other etiologies of kidney failure in terms of transplant age, donor type, immunosuppressive treatment, acute rejection, graft loss rates, and glomerular filtration rates at 1 and 5 years posttransplant (N = 180 total analyzed). RESULTS: Among 180 kidney transplant recipients, the 17 patients (9.4%) with nephronophthisis had a mean age of 12.6 ± 4.3 years and mean follow-up time posttransplant of 79.5 ± 41.9 months. Five of 17 patients received a kidney transplant from a deceased donor (29.4%), and the remaining 12 patients (70.6%) received transplants from living related donors. Preemptive kidney transplant was performed in 4 patients (23.5%). There was a statistically significant difference (P < .05) in terms of acute rejection between patients with nephronophthisis (17.6%) versus patients with other primary diagnoses (34%). However, the patients with nephronophthisis versus those with other primary diagnoses were similar (P > .05) in terms of transplant age (12.6 ± 4.3 vs 13.8 ± 6.7 years, respectively) and follow-up time (79.5 ± 41.9 vs 59.1 ± 38.8 months, respectively). Donor type, immunosuppressive treatment, and 1-year (96.7 ± 23.2 vs 97.6 ± 28.4 mL/min/1.73 m2) and 5-year (84.7 ± 31.1 vs 86.7 ± 21.7 mL/min/1.73 m2) glomerular filtration rates were also similar (P > .05) between groups. CONCLUSIONS: Posttransplant prognosis was good among kidney transplant recipients with juvenile nephronophthisis.

Influence of body mass index on pediatric urolithiasis.

OBJECTIVE: In recent years, there has been increased incidence of urolithiasis in children. Changing nutritional patterns and sedentary lifestyles predispose to urolithiasis, as well as to the global rise in obesity. It has been established that the prevalence of high body mass index (BMI) is increasing in the pediatric population. The aim of the present study was to incorporate 24-h urine metabolic analysis results with BMI values to evaluate the tendency towards stone formation in children. METHODS: Eighty-four children were recruited to the study, stratified into three BMI categories as low, normal, or upper. All patients were evaluated with 24-h urine analysis results. Patients with a secondary cause of stone formation such as hyperparathyroidism, cystinuria, primary hyperoxaluria, inflammatory bowel disease, cystic fibrosis, history of prematurity and/or use of drug, recurrent urinary tract infection, and urinary tract anomaly were excluded. Additionally, it was ensured that none of the patients were taking specific medication or diet that could alter their acid-base metabolism and calcium, oxalate, and uric acid levels. RESULTS: Mean BMI of patients was 21.6 ± 2.9 kg/m(2). LBMI was found in 52 (61.9%), N-BMI in 20 (23.8%), and U-BMI in 12 (14.3%) of the patients. No significant differences were present between the three groups for stone sizes and numbers. The patients' characteristics and 24-h urine parameters for BMI groups are presented in the Table. DISCUSSION: In the literature, several studies have focused on the relationship between obesity and pediatric urinary stone disease. However, only a few evaluated the urinary metabolic analysis in pediatric patients. We have encountered different results from mainly adult studies and some pediatric studies. Our study shows that U-BMI children are not under greater risk for urolithiasis than the other groups. An important portion of our study group was in the L-BMI group; nevertheless we cannot conclude that having a low BMI predisposes to urolithiasis based on the urinary metabolic evaluation as well as the stone sizes and numbers. The N-BMI group has increased risk factors for urolithiasis rather than the other groups, according to results of 24-h urine analysis. CONCLUSION: The results of our study indicate that BMI itself could not be considered as a separate and definite risk factor for urolithiasis development in children. Although the mechanisms and causative factors for urinary stone formation are better defined in adults, further studies investigating these parameters in children are warranted.

Long-term prognosis of idiopathic nephrotic syndrome in children.

BACKGROUND: To investigate the demographic, clinical and laboratory data of the children with idiopathic nephrotic syndrome (INS), and to determine prognostic factors that affect the clinical outcome of the patients. METHODS: Medical charts of 372 patients diagnosed to have INS and followed up at least 5 years between January 1990 and December 2008 were evaluated, respectively. After initial demographic, clinical and laboratory findings of the patients were documented, therapeutic protocols, prognosis and prognostic factors were investigated. RESULTS: 299 of the patients (80.4%) were steroid responsive and 73 (19.6%) were not. Focal segmental glomerulosclerosis (FSGS) was observed in 57%, minimal change disease (MCD) in 20.6% and diffuse mesengial proliferation in 21.9% renal biopsy materials. Steroid sensitivity was higher in patients with MCD and under the age of five years. Resistance to steroids was higher in children with FSGS. Complete remission was achieved in 96% of patients who were sensitive to steroids and in 46.6% who were resistant. 15% of patients who were steroid resistant developed chronic kidney disease (CKD). CONCLUSION: Intercurrent infections and response to steroid therapy are the most important factors affecting the prognosis of the disease.

The relation between treatment and prognosis of childhood membranoproliferative glomerulonephritis.

BACKGROUND: The prognostic factors, the outcome and the most favorable treatment regimen are not entirely known for children with membranoproliferative glomerulonephritis (MPGN). MPGN is a rarely observed disease more prevalent in adolescents, so we aimed to review the clinical and histological properties, treatments and the outcome of our patients who were diagnosed as MPGN. METHODS: Fifty-one children - diagnosed with MPGN - were selected from biopsy records in Dr. Sami Ulus Maternity and Children's Hospital Pediatric Nephrology Department from January 1999 to January 2011. A retrospective analysis was made of 33 regularly followed children. RESULTS: Thirty-three patients were identified, 13 female and 20 male. Their age groups at presentation ranged from 4 to 15 years. The following duration was 26-144 months (mean 74). Following the initial treatment, 20 (60%) patients achieved complete remission. Six patients with nephrotic syndrome and one with non-nephrotic proteinuria showed partial remission. The condition of one patient with nephrotic syndrome was unchanged with the persisting symptoms. The one patient with nephrotic syndrome and four others with non-nephrotic proteinuria did not respond to initial treatment as their renal functions decreased gradually. CONCLUSION: We concluded that only degree of tubulointerstitial damage on the initial biopsy is determinative for prognosis of childhood MPGN. If the patient receives high doses of steroid therapy in the early stages, their treatment is more likely to be successful. The effect of immunosuppressive treatment on MPGN is not clear.