Angiotensin-Converting Enzyme Gene Polymorphism in Children with Idiopathic Nephrotic Syndrome, Effect on Biopsy Findings.

OBJECTIVE: Angiotensin converting enzyme (ACE) converts angiotensin I into angiotensin II. The ACE gene shows an I/D polymorphism, which correlates with ACE concentrations. The aim of this study is to evaluate the distribution of the ACE I/D genotype in children with idiopathic nephrotic syndrome (INS) and healthy controls and study the effect of this polymorphism on clinical and pathologic findings. METHODS: ACE gene I/D polymorphism of 104 patients with INS and 119 controls were determined. RESULTS: The DD, ID, and II genotypes were found in 58.7%, 22.1%, and 19.2% of the patients, and in 79.8%, 2.5%, and 17.6% of controls, respectively (p > 0.05). The ID genotype was seen more frequently in patients resistant to treatment. CONCLUSION: The observed differences with previous reports suggest the influence of the genetic background on disease course. The ACE I/D gene polymorphism's role seems to be more important in renal disease progression than susceptibility.

Association of bone mineral density with biochemical markers of bone turnover in hemodialysis children.

INTRODUCTION: Although some descriptive and cross-sectional studies have been reported about bone mass in chronic kidney disease (CKD) children, only a few studies investigated markers of bone turnover and the bone mass measurements. OBJECTIVES: The aim of this study was to evaluate the association between bone mineral density (BMD) and biochemical markers of bone turnover in hemodialysis (HD) children. PATIENTS AND METHODS: The children who had received dialysis for at least the preceding 6-month were included. BMD was measured for total body, the lumbar spine and the femoral neck and the blood samples were tested to assess biochemical bone turnover markers. RESULTS: The study group was comprised of 27 patients with CKD, 9 males (33%) and 18 females (67%) with the mean±SD age of the subjects 14.9±4.5 years. Positive significant correlations of parathyroid hormone (PTH) with total body bone densitometry Z-score, lumbar spine and femoral neck Z-score(r=0.43, P=0.06; r=0.41, P=0.08 and r=0.45, P=0.05, respectively) was noted. In addition, positive significant correlations calcium and total body, lumbar spine and femoral neck Z-score (r=0.52, P=0.02; r=0.28, P=0.23 and r=0.36, P=0.12, respectively) was seen. Interestingly, a positive significant correlation between alkaline phosphatase (ALP) and lumbar spine Z-score was found (r=0.46, P=0.04), while the correlation of this parameter with total body and femoral neck Z-score was not significant (P>0.05). CONCLUSION: In our study, majority of patients with CKD had low level of BMD. In addition, lower levels of calcium (Ca), phosphorus (P), PTH and 25 (OH) vitamins D in patients with abnormal BMD Z-scores were detected.

Clinical Course and Effective Factors of Primary Vesicoureteral Reflux.

Vesicoureteral reflux (VUR) is one of the most important causes of urinary tract infection and renal failure in children. It is a potentially self-limited disease. The aim of this study was to evaluate the clinical course and significant factors in children with primary VUR. The medical charts of 125 infants and children (27.2 % males, 72.8% females) with all grades of primary VUR were retrospectively reviewed. Mean age at diagnosis was 22.3 ± 22.9 months. 52% of patients had bilateral VUR. Mild reflux (Grade I, II) was the most common initial grade. 53.6% of patients achieved spontaneous resolution. 30.1% of patients had decreased renal function on initial DMSA renal scan, significantly in males and severe VUR. Reflux nephropathy occurred in 17.6% of patients, especially in renal damage and male sex. No significant association was observed between recurrent urinary tract infection with the severity of VUR, and the presence of renal damage at admission. Age at diagnosis, gender, grade, laterality, the absence of recurrent urinary tract infection and renal damage had a significant correlation between spontaneous VUR resolution. Spontaneous resolution of primary VUR occurred significantly in female patients, age ≤ 30 months at diagnosis, mild-to-moderate VUR, unilateral reflux, the absence of recurrent urinary tract infection, and renal damage.

Resistant hypertension: current status, future challenges.

Resistant hypertension in adolescents is increasing in frequency and is increasingly recognized as having significant short- and long-term health consequences. It may be seen in up to 30% of all hypertensive patients cared for. Adolescents with resistant hypertension are at higher cardiovascular (CV) risk due to a long history of severe hypertension complicated by other CV risk factors such as obesity. Common causes of resistant hypertension include primary aldosteronism, sleep apnea, diabetes and chronic kidney disease. Careful blood pressure (BP) measurement and thorough evaluation of patients with sustained BP elevation should make a possible early diagnosis of resistant hypertension. Successful treatment requires identification and reversal of life-style factors contributing to treatment resistant and diagnosis and appropriate treatment of causes of hypertension. Improved pharmacologic therapies may offer the potential for preventing or at least ameliorating early CV disease. This review highlights these and other important issues in the evaluation and management of adolescents with resistant hypertension and provides practical guidance to the practitioners involved in caring for such patients.

The Effect of Long-term Steroid Therapy on Linear Growth of Nephrotic Children.

OBJECTIVE: Steroids are still the mainstay of management of nephrotic syndrome (NS). It was shown that steroids could impair growth and development of children. However, other clinical studies have shown conflicting results. METHODS: Hospital records of 147 children with diagnosis of NS who were followed during 1988-2008 are reviewed relating to height measurements. All patients were treated with prednisolone and had been followed for at least five years. Height measures were transformed into standard deviation score (SDS). Information on dose and duration of prednisolone therapy, histological findings of biopsy as well as concomitant use of steroid-sparing agents (SSA) were also analyzed. FINDINGS: Mean age at onset of NS was 5.94 years and at last follow-up visit 15.08 years. All patients had normal renal function during entire duration of the study. Analysis of the whole population did not show any significant alterations in the height SDS (Ht(SDS)) between the first and the last follow-up visit (P=0.5; -0.76±2.0 vs. -0.89±2.05 respectively). The patients were divided into two subgroups. Subgroup A, which achieved growth improvement, was composed of 62 children (initial Ht(SDS) -1.63; final Ht(SDS) -0.08; P<0.001) and subgroup B, that showed growth retardation, included 85 children (initial Ht(SDS) -0.13; final Ht(SDS) -1.59; P<0.001). CONCLUSION: No statistically significant retardation of linear growth was observed in the study population as a group following treatment with prednisolone according to the guidelines of ISKDC. Although about 62 subjects had growth retardation, children treated with prednisolone were not different from those who had increased growth.

An estimation of steroid responsiveness of idiopathic nephrotic syndrome in Iranian children.

OBJECTIVE: Idiopathic Nephrotic syndrome (INS) is the most common form of nephrotic syndrome (NS) in children with the potential of progression to end stage renal disease (ESRD). INS is steroid-responsive in most children, but not all patients respond to it. The aim of this study was to determine the rate of steroid responsiveness in children with INS that referred to Children's Medical Center since 1995 to 2007. METHODS: In as a cross sectional study, the medical records of all children with INS aged 1 to 15 years who were referred to our referral hospital was reviewed. All patients with onset of disease less than 1 year of age, spontaneous remission, secondary forms of NS associated with systemic diseases, and follow up duration of less than 12 months were excluded from the study. Patients were categorized into 6 groups: Group 1 needed biopsy prior to any treatment, group 2 non-relapsing NS, group 3 infrequently relapsing NS, Group 4 frequently relapsing NS, group 5 steroid dependent NS and group 6 steroid resistant NS. FINDINGS: A total of 238 patients were enrolled in the study. Kidney biopsy was performed in 79 cases. Minimal change lesion (MCL) was the most common (36.7%) pathological diagnosis. Steroid responsiveness was found in 81.5% of all cases including: 96% of MCL (consisting of biopsy proven cases and presumed ones), 32% of focal and segmental glomerulosclerosis, 73% of diffuse mesangial proliferation and 58% of membranoproliferative glomerulonephritis patients. During minimal follow up period of 12 months, there were 194 patients in remission, 32 patients with active NS, and 12 patients in ESRD. CONCLUSION: Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy.