Evaluation of cardiovascular and lipid profile abnormalities in obese childern and adolescents

The prevalence of childhood obesity has risen dramatically in past several decades. Hormonal and genetic factor are rarely the cause of childhood obesity. Because obese children may suffer life long physical and emotional consequences, this imperative to discuss prevention with parents during well- child examinations. The obesity-related cardiovascular and lipid profiles are determined in obese children and adolescents living in Mashahd, Northeast of Iran. Fifty two obese individuals, with mean age 10.46 +/- 1.25 yrs-old and associated body mass index [BMI] over 95 percentile for their age and sex and twenty healthy none obese individuals as control group with mean age 10.68 +/- 1.33 yrs-old with normal BMI for that age and sex participated in the study. Left ventricular [LV] mass, using Two-D and M-mode echocardiography, blood pressure and lipid profiles [total cholesterol, HDL-LDL and triglycerides] of all participants were obtained. In obese group the mean value of LV-mass was 53.78 +/- 9.68g, body mass index was 28 +/- 3.7 g/m2, total cholesterol was 203.38 +/- 30.20 mg/dl, HDL was 49.35 +/- 8.9mg/dl, LDL was 127.9 +/- 24.32mg/dl and triglycerides was 138 +/- 66 mg/dl. In control subjects the mean LV-mass was 25.5 +/- 4.8 g and Lipid profiles were in their normal limits. Both the mean of LV-mass and the frequency of dyslipidemia are increased in obese children and adolescents. Therefore, in obese children and adolescents, their evaluation should be considered as a routine approach for the prediction of middle-age-cardiovascular diseases

Ellis-Van-Creveld Syndrome: a report of five cases

Background and purpose: Syndrom of Ellis-Van-Creveld is a tetrad of chondrodysplasia, ectodermal dysplasia, polyductyly, and congenital heart disease, of which chondrodysthrophy of the tubular bones is the most common feature, while central nervous system [CNS] and urinary tract anomalies are some of its rarer associations. The aim of this study was to determine the clinical presentation of this rare autosomal recessive syndrome

Case report: Five cases reffered to Imam Reza hospital in Mashad for two years from 2002 to 2003. They were Iranian originally. First two cases, and 4 and 5, were sisters who presented all four classic features of EVC. Case 3 presented two features of this syndrome. The yougest was newborn and the oldest one was 11.5 years old

Conclusion: We observed five cases of this syndrom during two years. This syndrome has no treatment. Respecting relative marriage culture between iranians, it is important for prevention of this syndrome by avioding kingsman marriage

Turner syndrome: Cardiovascular abnormalities and correlation with clinical phenotype and karyotype

Turner Syndrome [TS] is the most common sex chromosomal abnormality. Patients [Pts] with TS are known to have higher incidence of congenital heart disease [CHD] than the general population. No studies have reported echocardiographic data according to the phenotype and chromosomal pattern in our country. The aim of our study was to assess an institutional experience. During 1995 to 2002, 37 unselected Pts with TS underwent cardiologic evaluation at the Imam-Reza Hospital. Karyotype distribution was: 45,X [65%], X-structural abnormalities [30%] and X-mosaism [5%].The mean age at the time of diagnosis was 12.81 +/- 4.48 years. The prevalence of CHD was 21%. Coarctation of Aorta [CoA] was the most prevalent CHD. [Absolute and relative risk was 16% and 75% respectively] The Pts with 45, X karyotype had the greatest prevalence of CHD, and the PTS with X-mosaism showed no signs of CHD. The PTS with severe dysmorphic signs had 45, X karyotype and showed a significant higer relative risk of cardiac malformations. There is an absolute higher prevalence of CHD in Pts with TS. An accurate, periodic cardiologic evaluation is necessary in all Pts with TS.

[Primary hypertrophic cardiomyopathy in noonan syndrome]

The term "Noonan syndrome" has been applied to males and females who have certain phenotypic features that occur also in turner syndrome, but these boys and girls have normal karyotypes. The disorder is usually sporadic, but affected siblings of the same and different genders have been reported. The most common abnormalities are short stature, webbing of the neck, pectus carinatum or pectus excavatum, cubitus valgus, and congenital heart disease. We describe a case of Noonan Syndrome that admited in hospital with initial diagnosis of Turner syndrome and referred for routine evaluation of cardiovascular abnormalities. Physical examination, electrocardiogram and chest x-ray and echocardiographic finding confirmed severe nonobstructive hypertrophic cardiomyopathy. After chromosomal analysis that revealed normal 46xx karyotype diagnosis of Noonan syndrome have been done. We concluded that Noonan syndrome should be considered in differntial diagnosis short statue in girls with Turner features