Vascular involvement in tuberous sclerosis.

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.

Hyponatremia among runners in the Boston Marathon.

BACKGROUND: Hyponatremia has emerged as an important cause of race-related death and life-threatening illness among marathon runners. We studied a cohort of marathon runners to estimate the incidence of hyponatremia and to identify the principal risk factors. METHODS: Participants in the 2002 Boston Marathon were recruited one or two days before the race. Subjects completed a survey describing demographic information and training history. After the race, runners provided a blood sample and completed a questionnaire detailing their fluid consumption and urine output during the race. Prerace and postrace weights were recorded. Multivariate regression analyses were performed to identify risk factors associated with hyponatremia. RESULTS: Of 766 runners enrolled, 488 runners (64 percent) provided a usable blood sample at the finish line. Thirteen percent had hyponatremia (a serum sodium concentration of 135 mmol per liter or less); 0.6 percent had critical hyponatremia (120 mmol per liter or less). On univariate analyses, hyponatremia was associated with substantial weight gain, consumption of more than 3 liters of fluids during the race, consumption of fluids every mile, a racing time of >4:00 hours, female sex, and low body-mass index. On multivariate analysis, hyponatremia was associated with weight gain (odds ratio, 4.2; 95 percent confidence interval, 2.2 to 8.2), a racing time of >4:00 hours (odds ratio for the comparison with a time of <3:30 hours, 7.4; 95 percent confidence interval, 2.9 to 23.1), and body-mass-index extremes. CONCLUSIONS: Hyponatremia occurs in a substantial fraction of nonelite marathon runners and can be severe. Considerable weight gain while running, a long racing time, and body-mass-index extremes were associated with hyponatremia, whereas female sex, composition of fluids ingested, and use of nonsteroidal antiinflammatory drugs were not.

Cardiorespiratory fitness in pediatric renal transplant recipients.

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.

Hemolytic uremic syndrome in a child with laboratory-acquired Escherichia coli O157:H7.

A 6-year-old girl touched an agar plate containing Escherichia coli O157:H7 while visiting a hospital laboratory, and subsequently, colitis and hemolytic uremic syndrome developed. Pulsed-field gel electrophoresis patterns of the isolate cultured from her stool and that from the laboratory were identical.

Creation of a novel hemodialysis bridge graft to extend the life of an adolescent.

All conventional dialysis access options were exhausted in a 19-year-old girl with end-stage renal disease (ESRD). Her course was medically and psychosocially complicated. To save her life, a subclavian artery to right atrium hemodialysis bridge graft was created so that she could be dialyzed successfully. Adolescents and young adults confronted with the exhaustion of dialysis access and transplant options face extremely difficult management decisions. Attention to the multi-disciplinary needs of these patients and a combined effort by medical, surgical, psychological, and social work teams are necessary to address properly these dilemmas.