Protein-losing enteropathy following the Fontan procedure in a child with intestinal lactase deficiency treated with lactose-free diet.

UNLABELLED: A 12-year-old girl presented with a protein-losing enteropathy. Symptoms started 4 weeks after undergoing the Fontan procedure at the age of 1.5 years for mitral atresia, ventricular septal defect, and double-outlet right ventricle. Upon referral for 3 weeks of rehabilitation after multiple interventional measures and drug treatments, she appeared in a dystrophic state, with decreased plasma protein and electrolyte levels along with occasional tetanic convulsions. Blood glucose levels after a lactose tolerance test were markedly reduced. The introduction of a lactose-free diet was quickly effective, with plasma protein and electrolyte levels raised to normal levels, and the girl's body weight increased without ascites or oedema. Molecular genetic examination revealed a homozygous C/C13910 polymorphism in the LCT gene. CONCLUSION: Protein-losing enteropathy in the Fontan circulation may be provoked by lactase deficiency and should therefore be ruled out to exclude this rather common condition.

Assessment of the cardiovascular system in pediatric chronic kidney disease: a pilot study.

Long-term survival of children and adolescents with chronic kidney disease (CKD) is mainly limited by cardiovascular disease. Pediatric CKD patients (n = 26) on conservative treatment, dialysis and after renal transplantation were compared with healthy controls (n = 24) with respect to cardiovascular status. Mean baseline diameter of the brachial artery was significantly higher, and mean flow-mediated vasodilation (FMD) was significantly reduced, in CKD patients. CKD patients showed significantly increased left ventricular mass index, blood pressure (BP) values and age-related values of mean carotid intima-media thickness [intima-media thickness-standard deviation score (IMT-SDS)] compared with those of controls. Approximately 60% of patients presented with impaired FMD (< or = 5.79%), which was significantly associated with intima-media thickening, although only three patients (12%) presented with both, impaired FMD and increased age-related IMT. The latter was significantly associated with higher values for day-time BP. In contrast, duration and degree of CKD, mode of renal replacement therapy, homocysteine levels and concomitant medication showed no association with cardiovascular status. The majority of our pediatric CKD patients showed reduced endothelial function, which may have preceded the development of carotid arteriopathy. Therefore, routine assessment of FMD may be a useful tool to identify CKD patients at risk of progressive cardiovascular morbidity.

Improvement of early vascular changes and cardiovascular risk factors in obese children after a six-month exercise program.

OBJECTIVES: The present study aimed to assess the effect of a 6-month exercise program in obese children on flow-mediated vasodilation (FMD) carotid intima-media thickness (IMT) and cardiovascular risk factors (RF). BACKGROUND: Childhood obesity contributes to adult obesity and subsequent cardiovascular disease. Physical inactivity is a major RF for obesity, endothelial dysfunction, and elevated carotid IMT, culminating in early atherosclerotic disease. METHODS: Sixty-seven obese subjects (age 14.7 +/- 2.2 years) were randomly assigned to 6 months' exercise or non-exercise protocol. We examined the influence of exercises (1 h, 3 times/week) on FMD, IMT, and cardiovascular risk profile. RESULTS: Compared with lean control subjects, obese children demonstrated at baseline significantly impaired FMD (4.09 +/- 1.76% vs. 10.65 +/- 1.95%, p < 0.001), increased IMT (0.48 +/- 0.08 mm vs. 0.37 +/- 0.05 mm, p < 0.001), and a number of obesity-related cardiovascular RF. Significant improvements were observed in the exercise group for IMT (0.44 +/- 0.08 mm, p = 0.012, -6.3%) and FMD (7.71 +/- 2.53%, p < 0.001, +127%). This improvement correlated with reduced RF, such as body mass index standard deviation scores, body fat mass, waist/hip ratio, ambulatory systolic blood pressure, fasting insulin, triglycerides, low-density lipoprotein/high-density lipoprotein ratio, and low-degree inflammation (C-reactive protein, fibrinogen). CONCLUSIONS: The present study documented increased IMT, impaired endothelial function, and various elevated cardiovascular RF in young obese subjects. Regular exercise over 6 months restores endothelial function and improves carotid IMT associated with an improved cardiovascular risk profile in obese children.

Impaired flow-mediated vasodilation, carotid artery intima-media thickening, and elevated endothelial plasma markers in obese children: the impact of cardiovascular risk factors.

OBJECTIVES: Childhood obesity contributes to the development of adult obesity and subsequent cardiovascular disease. The present study aimed to assess vascular status (flow-mediated vasodilation [FMD], intima-media thickness [IMT]) and to analyze plasma surrogate endothelial markers (von Willebrand factor [vWf], E-selectin, and thrombomodulin) in obese children as compared with controls. Associations between early morphologic and functional vascular changes, surrogate soluble markers of early atherosclerosis, and the cardiovascular risk profile were determined. METHODS: We examined 32 obese children versus 20 control subjects. All of the children underwent identical screening, comprehensive risk factor assessment, and measurements of E-selectin, vWf, thrombomodulin, FMD, and IMT. RESULTS: Compared with controls, obese children demonstrated significantly impaired FMD and increased IMT. Concentrations of soluble E-selectin and thrombomodulin were significantly elevated in obese children, whereas vWf showed no significant differences between obese children and controls. FMD, IMT, E-selectin, and thrombomodulin were significantly associated with various risk factors, including the extent of obesity, arterial hypertension, fibrinogen, C-reactive protein, and low physical fitness. CONCLUSIONS: The present study documented increased IMT, impaired endothelial function, and elevated plasma markers of endothelial activation and injury in obese children. Morbid obesity, arterial hypertension, subclinical inflammation, and low physical fitness formed a risk profile associated with the risk of early atherosclerosis in these children. Sonographic assessment of vascular status and the estimation of soluble endothelial plasma markers, combined with comprehensive risk factor screening, may form a rationale to identify high-risk children susceptible to early atherosclerotic disease and to monitor vascular changes during follow-up studies and therapeutic measures.

Thoratec left ventricular assist device for bridging to recovery in fulminant acute myocarditis.

Fulminant acute myocarditis can be the cause of rapid cardiac decompensation that is resistant to maximal medical therapy. Successful weaning from left ventricular mechanical support is very rare in fulminant myocarditis. We report the case of a young patient with viral myocarditis who was successfully weaned from a Thoratec left ventricular assist device with full recovery of myocardial function.