[Turner syndrome: overview of problems, present status, proposals for care and a protocol for monitoring in childhood, adolescence and adulthood].

Background: Girls and adolescents with Turner syndrome (TS) usually receive intensive medical care in a multidisciplinary team, coordinated by paediatric endocrinologist. Majority of them are discharged from specialist clinics following the induction of puberty and attainment of final height. Patients with Turner syndrome have a reduced life expectancy, they are known to have multi-system impairments in addition to the short stature and to the absence of sexual development.

[Growth in children with the exudative enteropathy syndrome due to a congenital heart defect--cor triatriatum dextrum].

Retarded growth in a child can be the sign of serious chronic disease. The authors present an account of a six-year-old boy where growth retardation persisted at least from the age of three. During this period his height dropped from the zone between the 25th and 50th percentile into the zone between the 3rd and 10th percentile.

Cardiomyopathy and changes of skeletal muscles in cystic fibrosis.

Cardiomyopathy (CMP) was found in 26 children with cystic fibrosis (CF), 24 of them died, the majority of them during the first 3 years of life. Only 4 of them were older than 10 years. 2 children are living.

Electrocardiographic changes can precede the development of myocardial hypertrophy in the setting of hypertrophic cardiomyopathy.

A comparison was made of electrocardiographic findings in 107 first-degree relatives of patients with hypertrophic cardiomyopathy without any clinical and echocardiographic signs of the disease and 188 healthy persons with a negative family history. A significantly larger number of electrocardiographic signs of left ventricular hypertrophy (P less than 0.05) and abnormal Q wave (P less than 0.

[The incidence of heart defects in children].

By examining all of 91,823 children born in 1980 in Bohemia, congenital heart disease was proved in 589 (6.415/1000 liveborns) and cardiomyopathy in 24 (0.261/1000 liveborns).

[Roentgenologic phenotype in syndromes associated with congenital heart defects].

In a group of children with congenital heart disease in 10.7% concurrently skeletal anomalies were observed (1085 children) and in 10.5% anomalies of the upper urinary pathways (1807 children).

Prevalence, treatment, and outcome of heart disease in live-born children: a prospective analysis of 91,823 live-born children.

All 91,823 children born in 1980 in Bohemia (population 6.314 million; area 52,478 square kilometers) were examined at least four times during infancy and at the age of three and four years. All children who died were autopsied and those with heart disease were selected.

Lorcainide treatment of Wolff-Parkinson-White syndrome in children and adolescents.

Lorcainide was used in 17 children and adolescents aged 14 days to 18 years (mean 6.8 years) with the preexcitation syndrome (W-P-W type). Lorcainide was able to control attacks of supraventricular tachycardia in eight of 11 patients with the W-P-W syndrome and tachyarrhythmias.

Results of radical surgical correction of ventricular septal defect.

This study evaluates results of radical correction of simple and complex ventricular septal defect in 85 children. 35 children were under 3 years of age. Two babies operated at the age of 6 and 8 months in a critical condition died after surgery.

Long-term results of anastomotic operations in 120 children with cyanotic congenital heart disease operated on the the first two years of life.

The author analyse results of anastomosis in 120 children with cyanotic heart disease operated on in the first two years of life. At the age up to 3 months of life 21 infants were operated on, 11 (52.4%) of whom died in connection with the procedure.