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Hadidi R.,Pediatric Nephrology
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia | Year: 2014

To determine the patterns of renal disease among Jordanian children based on kidney biopsy results as well as correlating clinical presentation with histopathological diagnosis, we studied the medical records of 55 children who had kidney-biopsy kidney disease at the Prince Hamza Hospital from August 2006 to April 2012. The most common indication for biopsies was steroid-resistant nephrotic syndrome (25%), followed by steroid-dependent nephrotic syndrome (20%). The most common diagnosis of primary glomerular disease was minimal change disease (27%), followed by focal segmental glomerulosclerosis (21%), mesangioproliferative glomerulonephritis (12.7%) and IgA nephropathy (7.3%). The most common secondary renal disease was Henoch-Schonlein purpura (HSP) in 10.9% of the patients, followed by systemic lupus erythematosus in 9.1%. The kidney biopsy was normal in 3.6%. Cyclosporine protocol biopsy was performed in four (7.3%) patients. Gross hematuria after kidney biopsy occurred in 5.5% of cases; however, none of these patients required blood transfusion. Kidney biopsy is a safe procedure that needs to be performed once indicated and our results are comparable with those carried out elsewhere in Jordan. The study also shows the importance of establishing a national registry for pediatric renal disease.

Honda M.,Tokyo Metropolitan Childrens Hospital | Warady B.A.,Pediatric Nephrology
Pediatric Nephrology | Year: 2010

Encapsulating peritoneal sclerosis (EPS) is the most serious complication of long-term peritoneal dialysis (PD), with a mortality rate that exceeds 30%. There have been many reports of the incidence of EPS being strongly correlated to the duration of PD. Patients on PD for longer than 5 years, and especially those receiving this treatment for more than 8 years, should undergo careful and repeated surveillance for risk factors associated with the development of EPS. The development of ultrafiltration failure, a high dialysate/plasma creatinine ratio, as determined by the peritoneal equilibration test, peritoneal calcification, a persistently elevated C-reactive protein level, and severe peritonitis in patients on PD for longer than 8 years are signals that should prompt the clinician to consider terminating PD as a possible means of preventing the development of EPS. The impact of the newer, biocompatible PD solutions on the incidence of EPS has not yet been determined. © 2008 IPNA.

Webb T.N.,Pediatric Nephrology | Ramratnam M.,University of Pittsburgh | Evans R.W.,University of Pittsburgh | Orchard T.,University of Pittsburgh | And 3 more authors.
Pediatric Nephrology | Year: 2014

Background: Atherosclerosis causing renal artery stenosis (RAS) is one of the most common secondary causes of hypertension in adults, but is rare in children. Case-diagnosis/treatment: RAS associated with coronary artery stenosis was diagnosed in a teenage patient who presented with intermittent chest pain and elevated blood pressures for 6 years. The diagnosis of RAS was suspected after physical examination revealed an abdominal bruit. Renal ultrasound with Doppler revealed normal appearing kidneys with high velocity in the aorta and renal arteries. Computed tomography angiography (CTA) of the chest and abdomen demonstrated generalized calcified atherosclerotic narrowing of the arteries including the renal, celiac, superior mesenteric and coronary arteries in the setting of hyperlipidemia. The lipid panel revealed hypercholesterolemia with elevated serum plant sterol concentrations, suggesting the diagnosis of sitosterolemia. Cardiac catheterization demonstrated left anterior descending artery and left circumflex artery stenosis, which required bypass of the left anterior descending artery and stenting of the left circumflex artery. Aggressive lipid control was recommended and he was treated medically with a beta-blocker, low-dose angiotensin-converting enzyme inhibitor, aspirin, statin, and clopidogrel. Conclusion: Although very rare, generalized atherosclerosis caused by genetic disorders should be considered an underlying cause for severe hypertension in children with hyperlipidemia. © 2014 IPNA.

Ayoob R.,Pediatric Nephrology | Schwaderer A.,Pediatric Nephrology
Pediatric Nephrology | Year: 2011

In the last 10 years, the incidence of kidney stones has increased in the pediatric population, and this rise has been paralleled by a significant increase in pediatric obesity rates in the USA. The purpose of this study was to evaluate percentage body fat (%BF) measured by dual energy X-ray absorptiometry (DXA) in hypercalciuric children with and without kidney stones. A retrospective chart review was performed on children with idiopathic hypercalciuria based on a 24-h urine calcium excretion of >4 mg/kg/day or >200 mg/day who had undergone DXA scanning. Patients were then classified by sex and by %BF (3 categories; normal: <27% girls, <21% boys; at risk for obesity: 27-36% girls, 21-30% boys; obese: >36% girls, >30% boys). The 2003-2004 NHANES data were used as a control. Fifty patients (24 males) were analyzed, of whom 26% were assessed as having a normal %BF, 44% as being at risk for obesity, and 30% as being obese. Children with an increased %BF had a significantly higher occurrence of kidney stones (p=0.03) than those with a normal %BF. No significant differences were noted in 24-h urine chemistries between the groups. In conclusion, an increased %BF was associated with an increased occurrence of kidney stones in children with idiopathic hypercalciuria. © 2011 IPNA.

Avni F.E.,University Clinics of Brussels Erasme Hospital | Hall M.,Pediatric Nephrology
Pediatric Radiology | Year: 2010

This review highlights the changes that have occurred in the general approach to cystic renal diseases in children. For instance, genetic mutations at the level of the primary cilia are considered as the origin of many renal cystic diseases. Furthermore, these diseases are now included in the spectrum of the hepato-renal fibrocystic diseases. Imaging plays an important role as it helps to detect and characterize many of the cystic diseases based on a detailed sonographic analysis. The diagnosis can be achieved during fetal life or after birth. Hyperechoic kidneys and/or renal cysts are the main sonographic signs leading to such diagnosis. US is able to differentiate between recessive and dominant polycystic kidney diseases, hepatocyte nuclear factor 1 Beta mutation, glomerulocystic kidneys and nephronophtisis. MR imaging can, in selected cases, provide additional information including the progressive associated hepatic changes. © Springer-Verlag 2010.

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