Nephrology Copyright (c) 2013 University of Massachusetts Medical School All rights reserved. http://escholarship.umassmed.edu/peds_nephrology Recent documents in Nephrology en-us Wed, 13 Feb 2013 18:02:18 PST 3600 Hematuria http://escholarship.umassmed.edu/peds_nephrology/7 http://escholarship.umassmed.edu/peds_nephrology/7 Thu, 31 May 2012 13:10:29 PDT Summary: Provides rapid-access information on the diagnosis, treatment, and follow-up of Hematuria.

]]> Ann E. Salerno Hematuria Synthetic DDAVP for nocturnal enuresis and the risk of symptomatic hyponatremia: which treatment now? Which form? http://escholarship.umassmed.edu/peds_nephrology/6 http://escholarship.umassmed.edu/peds_nephrology/6 Thu, 31 May 2012 08:58:41 PDT Recently, several cases of seizure secondary to hyponatremia have been reported in children treated with intranasal DDAVP1), 2). This has rarely been reported with the oral form. Should the intranasal form be banned for the treatment of nocturnal enuresis (NE)? Or should any form of DDAVP be banned? What are the precautions to take to avoid such an event? We hereunder summarize the literature on the subject, and present the recommendations of the Swiss Group for Pediatric Nephrology and the current position of Swiss medic.

]]> Francois Cachat et al. Deamino Arginine Vasopressin Nocturnal Enuresis Hyponatremia Vascular involvement in tuberous sclerosis http://escholarship.umassmed.edu/peds_nephrology/5 http://escholarship.umassmed.edu/peds_nephrology/5 Thu, 31 May 2012 07:58:50 PDT Vascular involvement in tuberous sclerosis (TS) is rare. Central and peripheral aneurysms and large and medium size arterial stenotic-occlusive disease have been reported in patients with TS. We present here three pediatric patients with TS and severe vascular abnormalities, followed by a review of the literature. The three cases include a 14-month-old girl with polycystic kidneys and cerebral tubers who had a large asymptomatic abdominal aortic aneurysm, a 2-year-old boy with multiple features of TS who had hypertension and was found to have mid-aortic syndrome with bilateral renal artery stenosis, and an 18-year-old girl with abdominal pain and TS features who had greater than 70% celiac artery stenosis. In all cases, noninvasive vascular imaging modalities were utilized for either initial diagnosis, surveillance, or both. These cases highlight the collaborative roles of the pediatric nephrologist and cardiovascular imager in the diagnosis and management of the vascular complications in TS patients. Appropriate care can only be made through a high index of suspicion.

]]> Ann E. Salerno et al. Aneurysm Aorta Aortic Aneurysm, Abdominal Arterial Occlusive Diseases Blood Vessels Child, Preschool Female Follow-Up Studies Humans Hypertension Infant Infant, Newborn Male Renal Artery Obstruction Time Factors Tuberous Sclerosis Cardiorespiratory fitness in pediatric renal transplant recipients http://escholarship.umassmed.edu/peds_nephrology/4 http://escholarship.umassmed.edu/peds_nephrology/4 Thu, 31 May 2012 07:58:48 PDT BACKGROUND: The impact of body size, fat-free mass (FFM), and fat mass (FM) on cardiorespiratory fitness in pediatric renal transplant recipients (TX) has not been established. Study objectives were to assess maximal oxygen consumption (VO2max) in TX and controls, adjusted for body composition, and to identify risk factors for reduced fitness in TX.

METHODS: Cycle ergometry and dual-energy X-ray absorptiometry were obtained in 50 TX and 70 controls, ages 8 to 21 years. Control recruitment was targeted to include obese subjects with body mass index Z-scores comparable with TX. Allometric regression models were used.

RESULTS: TX had significantly lower height Z-scores (P<0.001) and comparable body mass index Z-scores. VO2max per body weight (mL/kg/min) and per FFM (mL/kgFFM/min) did not differ between groups. However, VO2max was 13% lower (95% CI 18, 8; P<0.001) in TX, compared with controls, adjusted for FM, FFM, sex, and race. Greater FFM, lower FM, non-black race, and male sex were independently associated with greater VO2max. Within TX, hemoglobin levels were positively associated with VO2max (P=0.04) and sirolimus use was associated with lower VO2max (P<0.01).

CONCLUSIONS: TX had significant VO2max deficits that were not captured by conventional measures (mL/kg/min). Greater FM was an independent risk factor for low VO2max. Lower fitness in TX may be related to sirolimus effects on skeletal muscle.

]]> Christine B. Sethna et al. Absorptiometry, Photon Adiposity Adolescent Body Height Body Weight Case-Control Studies Child Continental Population Groups Cross-Sectional Studies Exercise Test Female Humans Kidney Transplantation Male *Oxygen Consumption *Physical Fitness Regression Analysis Risk Assessment Risk Factors Sex Factors Treatment Outcome Young Adult Fluid resuscitation in the intensive care unit http://escholarship.umassmed.edu/peds_nephrology/3 http://escholarship.umassmed.edu/peds_nephrology/3 Thu, 31 May 2012 07:58:46 PDT Letter to the Editor regarding the Saline versus Albumin Fluid Evaluation (SAFE) Study published in the May 27, 2004 issue of NEJM.

]]> William A. Primack et al. Albumins Critical Illness Fluid Therapy Humans Hypovolemia Sodium Chloride Concordant prune belly syndrome in monozygotic twins http://escholarship.umassmed.edu/peds_nephrology/2 http://escholarship.umassmed.edu/peds_nephrology/2 Thu, 31 May 2012 07:58:45 PDT We report 2 cases of concordant prune belly syndrome occurring in monozygotic twins. In addition to suggesting a genetic basis for this disease, our 12-year follow-up of these cases illustrates that these patients with an otherwise poor prognosis can have normal growth, development, and renal function with appropriate treatment.

]]> Kethandapatti C. Balaji et al. *Diseases in Twins Humans Infant, Newborn Male Polymorphism, Restriction Fragment Length *Prune Belly Syndrome *Twins, Monozygotic Recurrent bacteremia with enteric pathogens in recessive polycystic kidney disease http://escholarship.umassmed.edu/peds_nephrology/1 http://escholarship.umassmed.edu/peds_nephrology/1 Thu, 31 May 2012 07:58:44 PDT Eight children with autosomal recessive polycystic kidney disease (ARPKD) and recurrent bacteremia with enteric pathogens are described. Typical clinical features of bacterial cholangitis were absent, although in five patients histological and/or microbiological data indicated that the bacteremic episodes originated in the biliary tree. Bacteremia with enteric pathogens or recurrent culture-negative febrile illness in a child with ARPKD should raise suspicion of cholangitis, even in the absence of typical clinical findings.

]]> Clifford E. Kashtan et al. Bacteremia Child Child, Preschool Cholangitis Female Humans Infant Intestines Liver Cirrhosis Polycystic Kidney, Autosomal Recessive Recurrence