[Home artificial respiration in a girl with Ondine-Hirschsprung syndrome (Haddad's syndrome)].

A case of 5.5-year-old girl with Haddad syndrome (congenital central hypoventilation syndrome-CCHS or Ondine's curse and Hirschsprung's disease) is presented. At the age of 1.

Radioangioscintigraphy and Doppler echocardiography in the quantification of left-to-right shunt.

The magnitude of left-to-right shunt in 55 children with isolated congenital heart disease [atrial septal defect (ASD) or ventricular septal defect (VSD) (muscular and perimembranous)] was estimated by two methods: radionuclide quantification and Doppler echocardiography [flow (L/min) = mean velocity x area x ejection time x heart rate]. We found little difference between the magnitude of left-to-right shunt obtained with Doppler echocardiography and that with radioangioscintigraphy for a whole group of patients (N = 55, -11.42% to 12.

Complex disorder of neuronal migration in an infant with possible congenital cytomegalovirus infection.

A ten-months-old girl was evaluated for developmental delay, increased muscle tone and seizures. CT and MRI revealed un uncommon combination of two different manifestations of neuronal migration disturbance: agyria/pachygyria and subcortical laminar heterotopia ("double cortex" syndrome). The occurrence of these two manifestations of neuronal migration dosorders in the same individual is quite unusual.

[Incipient pericardial tamponade as the first symptom of systemic lupus erythematosus in 2 children].

Two patients (a boy and a girl), with systemic lupus erythematosus (SLE), in which pericarditis with threatening tamponade was an initial symptom of disease, are presented. Pericardial tamponade is very rare initial manifestation, described in only 1-3% of all the cases of SLE. The typical clinical features of tamponade are described (tachycardia, hypotension, venous congestion) and the importance of echocardiography in early diagnosis of pericarditis and pericardial tamponade is pointed out.