Obesity-related cardiovascular behavior in children.

OBJECTIVE: Obesity is a chronic metabolic disorder and may associate with cardiovascular geometrical, structural and functional changes. The aim of this study is to assess the relationship between body mass index (BMI), body surface area (BSA) and arterial-ventricular elasticity (Ea and Ees respectively) and cardiovascular coupling and myocardial wall stress and fiber stress in obese children. PATIENTS AND METHODS: Sixty non-obese healthy children with BMI <85th percentile aged 6-17 years and 65 age and sex-matched children with BMI of ≥95th percentile, were included in the study. Beside cardiac systolic and diastolic functions, left verntricular (LV) systolic and diastolic dimensions and volumes (LVDs, LVDd, LVVs, LVVd respectively), LV mass (LVM), LV end-systolic pressure (LVESP), meridional end-systolic wall stress (ESWm), myocardial fiber stress (MFS), Midwall Shortening Fraction (SFmid), heart rate corrected circumferential fiber shortening (VCFc), predicted mid wall fiber shortening for a measured fiber stress (mid wall VCFc), right ventricular (RV) and LV work index (RVWI, LVWI), LV relative wall thickness (LVRWT), arterial elastance (Ea), LV end-systolic elastance (Ees) and end-systolic pressure volume relationship (ESPVR) were calculated. RESULTS: LVDs and LVDd, LV mass (LVM), ESWSm, MFS, SF mid, Midwall VCFc and LVWI found to be significantly (p<0.001) higher, while Ea, Ees, ejection fraction (EF), fractional shortening (FS), VCFc-ESWS, RVWI, ESWSm/LVVs, LV end-systolic pressure (Pes)/LVVs and LVM/LVVd values were significantly (p<0.001) lower among obese group. By increasing age and BMI the Ea and Ees, ESWSm/LVVS and RVWI decrease; while LVDd, LVVd and stroke volume (SV) values increase. There was a reverse-relation between BMI percentiles and EF and FS.

Arterial and left ventricular end-systolic elastance in normal children.

OBJECTIVE: Assessing the left ventricular (LV) functions in Pediatric Intensive Care Unit, and those pediatric patients with compromised ventricular performance or enhanced systolic or diastolic load e.g. congestive heart failure, hypertension, dilated/hypertrophic cardiomyopathies is a real challenge. Currently used noninvasive methods fail giving quantitative measures to asses cardiac performance and do not allow evaluation of ventriculo-arterial interaction. Non-invasive method of cardiovascular performance determination by measuring left ventricle end-systolic elastance (Ees), arterial elastance (Ea) and the ventriculoarterial coupling (VAC), though interaction between LV and arterial network, is possible. PATIENTS AND METHODS: Hundred and forty two otherwise normal children (1 week to 17 years old) were randomly selected. Routine transthoracic echocardiographic and Doppler studies were carried out by an experienced pediatric cardiologist. The results have been evaluated statistically. RESULTS: We found that the Ea and Ees(sb) show powerful negative correlation with BSA (r = -0.65, -0.72 respectively) of the children. CONCLUSIONS: We suggest that this simple measurement method may be applied at bedside to evaluate ventricular performance of the children.

Long term follow-up of four patients with Keutel syndrome.

Keutel syndrome (KS) [OMIM 245150] is an autosomal recessive hereditary syndrome characterized by multiple peripheral pulmonary stenoses (PPS), brachytelephalangia, inner ear deafness, and abnormal cartilage ossification or calcification. Mutations in the matrix Gla protein (MGP) gene have been reported in different unrelated families with KS previously. MGP is an extracellular matrix protein and calcification inhibitor; mutations in its encoding gene result in cartilage ossification or calcification, the main presenting feature of KS. This report describes the findings of four sisters with KS born to consanguineous parents were followed for 26 years in an irregular fashion. During follow-up of the patients over the years the complications appear to be mostly involving the respiratory system. Permanent skin rashes, papillary microcarcinoma of the thyroid, asthma, massive bullous pulmonary emphysema, severe systemic arterial hypertension, and short term memory loss were observed during long term follow-up. The fertility status of the patients were also observed and infertility was observed in one of three married patients.

HLA and rheumatic fever in Turkish Children.

We report the results of research on human leukocyte allo-antigen (HLA) and rheumatic fever (RF), the first published study to be carried out among Turkish children with RF. Ninety-three Turkish children, aged between 6 and 16 years (mean: 8 +/- 2.6), with RF participated in the study. Of the total, 26 patients had their first attack and 39 had acute rheumatic activity at the time of registration. The results demonstrate (1) negative but not significant association between HLA-A2 and RF; (2) a positive association between HLA-DR4 and RF (p less than 0.001); (3) a significant association between HLA-DR4 and carditis, but not with isolated arthritis. These results corroborate the concept of race-specific genetically determined familial susceptibility to the development of rheumatic heart disease.

Keutel syndrome: a report of four cases.

We report four new cases of Keutel syndrome. Clinical manifestation included abnormal cartilage ossification, multiple peripheral pulmonary stenosis (PPS), brachytelephalangism, subnormal IQ, repeated respiratory infections, otitis media and hearing loss. All four children have a typical facial appearance and are of consanguineous parents. Father and the fifth offspring exhibit a normal phenotype; the mother has pulmonary stenosis. This observation confirms Keutel syndrome as a distinct autosomal recessive syndrome.

Treatment of nocturnal enuresis: a placebo-controlled trial with piracetam, diphenylhydantoin and psychotherapy.

The study population consisted of 100 children with nocturnal enuresis (NE) aged between six and 14 years, who had been randomly selected amongst the enuretic outpatients at the Ankara S.S.K. Children's Hospital. A placebo-controlled evaluation of piracetam, diphenylhydantoin and psychotherapy was carried out. At the end of the eighth week of the study, it was discovered that the therapeutic effects observed in the patients administered piracetam did not differ from those given the placebo. Therefore, we recommend that psychotherapy in association with a placebo be the first step in the treatment of NE children.