Effects of VEGF on phenotypic severity in children with hereditary hemorrhagic telangiectasia.

The purpose of this study was to estimate vascular endothelial growth factor (VEGF) and transforming growth factor (TGF)-beta1 serum levels in children with hereditary hemorrhagic telangiectasia (HHT) type 1 and type 2 and to correlate them to the presence of arteriovenous malformations (AVMs). High VEGF levels were initially found in an infant who had been hospitalized with intestinal bleeding and suspected HHT. This case led to the evaluation of VEGF and TGF-beta1 by standard enzyme-linked immunosorbent assay in 13 children with HHT and familiarity. Patients were divided into 2 groups on the basis of the presence/absence of pulmonary AVMs. No significant difference was found for VEGF and TGF-beta1 levels in HHT patients versus controls. Among HHT patients, serum levels of VEGF in those without AVM were significantly lower than those with AVM and normal controls. No difference for TGF-beta1 levels was found in these patient subgroups. Low VEGF levels may represent a protection factor against the onset of pulmonary AVMs in HHT children. However, neither VEGF nor TGF-beta1 can be used as biochemical markers for an early diagnosis in HHT. The diagnosis of HHT still requires clinical criteria, which permitted to confirm the presence of the disease in the infant with intestinal bleeding.

Oxidative stress in obesity and metabolic syndrome in children and adolescents.

BACKGROUND/AIMS: The aim of this study was to investigate the alterations in the oxidant/antioxidant status in obese children with and without metabolic syndrome (MetS). METHODS: We recruited 25 Caucasian obese children with MetS, 30 Caucasian children with simple obesity and a control group of 30 Caucasian children. We performed diacron-reactive oxygen metabolites (d-ROMs) test and biological antioxidant potential (BAP) test in order to evaluate the oxidant-antioxidant status in recruited patients. RESULTS: d-ROM level was significantly higher in obese children with and without MetS (p = 0.005). The total antioxidant capacity (BAP level) was reduced in MetS and noMetS children compared to controls (p = 0.009). The subjects without MetS had higher d-ROMs test and lower BAP/d-ROMs ratio than subjects with MetS (although not significant). The ratio BAP/d-ROMs was higher in controls than noMetS and MetS children (p < 0.0001). d-ROM level was higher in prepubertal subjects with MetS than pubertal ones (p = 0.03). A direct correlation was found between d-ROM levels and BMI SDS (p = 0.0005), while an inverse correlation was found between BAP and BMI SDS (p = 0.004) and BAP/d-ROMs and BMI SDS (p = 0.0001). CONCLUSIONS: This result confirms that fat accumulation plays a key role in the pathogenesis of systemic oxidative stress already during pediatric age.

Urinary epidermal growth factor, monocyte chemotactic protein-1, and ß2-microglobulin in children with ureteropelvic junction obstruction.

BACKGROUND/PURPOSE: We demonstrated down-regulation of epidermal growth factor (EGF) and up-regulation of monocyte chemotactic protein-1 (MCP-1) in the renal parenchyma in children who underwent pyeloplasty for ureteropelvic junction obstruction (UPJO). These findings were paralleled by urinary levels of EGF and MCP-1 before and after surgery. The aim of this study is to evaluate the urinary excretion of these cytokines and ß2-microglobulin (ß2M) in children with urine flow impairment at the ureteropelvic junction or who underwent pyeloplasty. METHODS: Seventy-six patients with UPJO and 30 normal children (CTRL) were enrolled in the study. The UPJO patients were divided into obstructive (12), functional (36), and operated (28). Epidermal growth factor, MCP-1, and ß2M urinary levels were measured by enzyme-linked immunosorbent assay and normalized to urine creatinine. RESULTS: Urinary ß2M and MCP-1 increased significantly in the UPJO groups compared with the CTRL and significantly improved in the operated group. The obstructive group displayed reduced EGF excretion compared with the CTRL group. The urinary (u)EGF/uMCP-1, and uEGF/uß2M ratios significantly decreased in both untreated groups. In the operated group, these ratios improved significantly. CONCLUSIONS: The present study substantiates the role of urinary EGF, MCP-1, and ß2M as markers of tubulointerstitial damage in human obstructive nephropathy. Furthermore, it suggests that surgical intervention is effective in the management of children with UPJO.