Associated anomalies in children with congenital solitary functioning kidney

PubMedID: 15883823Congenital solitary functioning kidney (CSFK) is a relatively common renal malformation and in children is frequently complicated by anomalies of the ipsilateral genital organs and occasionally by anomalies of other organs. The aim of this study was to determine the incidence of associated urological, cardiac, gastrointestinal, hematological, neurological, skeletal, and other congenital malformations in children with CSFK. We retrospectively reviewed 87 consecutive cases of CSFK diagnosed at our hospital between 1995 and 2003. There were 45 boys and 42 girls, whose ages at diagnosis ranged from newborn to 16 years (mean 4.67±4.48 years). In all patients, CSFK was diagnosed by abdominal ultrasound and confirmed by radionuclide studies. In 46 patients (53%) the left kidney was absent, and in 41 patients (47%) the right kidney was absent. Overall associated anomalies were detected in 52 of the 87 children (60%) with CSFK. Urological anomalies were most common, with an incidence of 37% (32/87). Nonurological anomalies were detected in 38 children (44%) with CSFK; these included cardiac anomalies in 13, gastrointestinal anomalies in eight, hematological anomalies in five, neurological anomalies in three, and other organ anomalies in nine. In our study, more than half of the patients with CSFK had associated anomalies. For this reason we recommend abdominal ultrasound and voiding cystourethrogram for early recognition of urological anomalies and a careful physical examination for other organ anomalies in patients with CSFK. © Springer-Verlag 2005

May the best friend be an enemy if not recognized early: Hypernatremic dehydration due to breastfeeding

PubMedID: 16027579Hypernatremic dehydration is a serious condition in the newborn period. We present 5 infants with hypernatremic dehydration due to breastfeeding; one of them died because of brain edema during treatment. Hypernatremic dehydration in breast-fed newborns is secondary to insufficient lactation and all mothers should be encouraged about breastfeeding, taught the signs of successful breastfeeding, and the warning signs of dehydration. Here, we discuss hypernatremic dehydration in breastfeeding neonates, its causes and treatment. Copyright © 2005 by Lippincott Williams & Wilkins

Mitral annular calcification and brown tumor of the rib in a child with chronic renal failure

PubMedID: 15719256Mitral annular calcification and liquefaction necrosis of this lesion mimicking intracardiac tumor because of secondary hyperparathyriodism have been described in adult patients with chronic renal failure, but have not been reported in children. Chronic renal failure is one of the predisposing factors of this condition. We report the case of a 13-year-old patient with continuous ambulatory peritoneal dialysis with severe hyperparathyroidism who was found to have intracardiac and rib lesions considered to be brown tumors. © IPNA 2005

ACE gene polymorphism in Turkish children with acute post streptococcal glomerulonephritis [Akut poststreptokokkal glomerulonefritli Türk çocuklarinda ACE gen polimorfizmi]

Aim: The role of angiotensin converting enzyme (ACE) gene polymorphism in various renal and cardiac diseases have been discussed since 1990s. Acute poststreptococcal glomerulonephritis (APSGN) is a benign inflammatory renal disease of beta hemolytic streptococcus. The aim of this study is to investigate whether the ACE gene polymorphism is associated with susceptibility to APSGN and it's clinical and laboratory features. Material and Method The study comprised 52 pediatric patients with acute poststreptoccal glomerulonephritis; 29 males (56%) and 23 females (44%) with a mean age of 97±33 months. The control group consisted of 140 healthy individuals including 83 males (59%) and 57 females (41%) with a mean age of 42±13 years. Results: ACE genotypes were significantly different between the patients and the control group (p<0.05). Mean systolic blood pressure, mean diastolic blood pressure z scores were significantly lower in D/D genotype group compared to non- D/D genotype group (p<0.05). There were no statistically significant difference among groups in terms of age at onset of disease, sex, C3 level, GFR, BUN, creatinine, macroscopic hematuria, edema, and duration of the oliguric period. Conclusions: Our results show that D/D polymorphism of ACE gene is not a risk factor for APSGN and hypertension of APSGN

ACE gene polymorphism in Turkish children with nephrotic syndrome

PubMedID: 16825089Since 1990, the role of angiotensin converting enzyme (ACE) gene polymorphism in various renal and cardiac diseases is still debated. This study comprised 71 pediatric patients with nephrotic syndrome, 47 males (66%) and 24 females (34%) with a mean age of 57.4 ± 37.6 months, and a control group of 83 healthy males (59%) and 57 healthy females (41%) with a mean age of 505 ± 160.5 months. The distribution of the ACE genotype in the control group was II, 11%; ID, 53%; and DD, 36%, and the nephrotic syndrome was II, 4%; ID, 78%; and DD, 18%. Angiotensin-converting enzyme genotypes were significantly different between patients and control groups (p<0.05). The study groups consisted of 52 (73%) with steroid-sensitive nephrotic syndrome (SNSS) and 19 (27%) with steroid-resistant nephrotic syndrome (SRNS). The distribution of the ACE genotype was II, 6%; ID, 75%; and DD, 19% in the SSNS population and ID, 84% and DD, 16% in the SRNS population. No statistically significant difference was found between steroid sensitivity and ACE genotypes (p=0.5). The results show that ACE I/D polymorphism does not contribute to the steroid resistance, even though this study indicates that the presence of the I/D genotype has a much higher risk-approximately 2.8 times-of having nephrotic syndrome. Further studies with a larger number of patients are needed. Copyright © Informa Healthcare.This study was supported by the Cukurova University Research Foundation (Nr. 2002 LTP-48). The authors are grateful for the statistical assistance of Dr. Gülsah Seydaoglu. Also they would like to thank to Halil Gülsev for his help with the laboratory procedures and PD nurse Gülsen Kaygusuz and Dr. Banu Kilinç for their assistance

The effects of gemfibrozil on hyperlipidemia in children with persistent nephrotic syndrome

PubMedID: 11858378Persistent nephrotic syndrome is frequently accompanied by severe hyperlipidemia, and this may pose a substantial risk for cardiovascular disease. Lipid-lowering drugs are prescribed by many nephrologists for adult patients but rarely for nephrotic children. The present investigation was designed to evaluate the safety and efficacy of gemfibrozil in nephrotic children. Eight girls and four boys aged from 5 to 17 years were enrolled in this study. They were all steroid and immunosuppressive resistant patients with nephrotic range proteinuria. Placebo was administered to five patients and gemfibrozil was administered to seven patients for four months. Blood samples were taken for the determination of cholesterol, triglyceride, low-density lipoprotein (LDL), high-density lipoprotein (HDL), BUN, serum creatinine (Scr), ALT, AST, CPK, apolipoprotein A (apo A), apoliporotein B (apo B), and serum albumin levels during the initial and subsequent examinations. At the end of the fourth month, gemfibrozil reduced total cholesterol by 34%, LDL by 30%, apo B by 21% and triglycerides by 53% (p &lt; 0.05). HDL cholesterol and apo A levels were not significantly altered. Renal function and urine protein excretion were not affected by gemfibrozil. In this study gemfibrozil therapy had no side effects and had favorable effects on the lipoprotein profile of nephrotic patients

Aggressive angiomyxoma in a child with chronic renal failure

PubMedID: 15776247Aggressive angiomyxoma (AAM) is a rare and nonmetastasizing soft-tissue tumor predominantly found in the female pelvis and perineum. It has a high risk of local recurrence. We report the unusual case of a 15-year-old boy with an AAM presenting as a slowly enlarging scrotal mass. The patient had had chronic renal failure since 1997 and had needed hemodialysis for the previous 11 months. He presented with a 12-month history of a nontender soft mass in the right scrotum. Ultrasound examination revealed a solid mass in the scrotum. After surgical resection, pathological analysis disclosed spindle-shaped neoplastic cells widely separated by a myxoid stroma rich in collagen fibers and prominent irregularly shaped blood vessels; the histological examination confirmed an AAM. © Springer-Verlag 2005