[The effect of noninvasive positive pressure ventilation in children with severe motor and intellectual disabilities with respiratory insufficiency].

We investigated the clinical course of 20 children (persons) with severe motor and intellectual disabilities (SMID) who were treated with noninvasive positive pressure ventilation (NPPV) for respiratory insufficiency. NPPV was effective in 10 of 11 patients treated for acute respiratory failure, and in 7 of 9 patients treated for chronic respiratory failure. Twelve patients were treated with NPPV for more than one year. There were no complications associated with NPPV in any of the patients. NPPV improved ventilation impairment soon after ventilation was started, and avoided the need for the endtracheal intubation by adjusting airway management and the choice of mask in all but one of the patients with acute respiratory failure. NPPV in combination with wearing a chin strap was highly effective in patients with open state or upper airway obstruction. Five patients were successfully weaned off the ventilator soon after recovery from acute respiratory failure using NPPV, whereas 5 patients who continued NPPV during the chronic phase after recovery did not experience recurrent episodes of acute respiratory failure. We conclude that NPPV may be an effective treatment for SMID with respiratory insufficiency.

Longitudinal estimation of signal-averaged electrocardiograms in patients with Kawasaki disease.

BACKGROUND: Myocarditis associated with Kawasaki (KD) disease is prominent, but rarely detected by conventional methods. The hypothesis of this study is to see if signal-averaged electrocardiogram can detect myocarditis with KD. METHODS: We obtained signal-averaged electrocardiograms from 71 patients with KD (mean age 2.8 +/- 2.9 years) in the acute (1st-4th week), subacute (5-7th week), and chronic (8th week or later) phases (mean study period 3.5 +/- 1.7 years). Sixteen patients who had pericardial effusion, bundle branch block or myocardial ischemia were excluded from this study. The results were compared with those of Holter and 12-lead electrocardiograms, echocardiography and serum myocardial enzymes. They were also contrasted with the course of each patient. RESULTS: The incidence of abnormal findings on signal-averaged electrocardiogram was 18.2% in the acute phase versus 10.9% in the subacute and chronic phases. It differed significantly higher than the other conventional tests (P < 0.05). Four patients had abnormalities of signal-averaged electrocardiograms through all three phases. Among these four, two had reduced left ventricular contractility. However, these changes were transient and resolved in the subacute phase. All patients had good courses and no residue. CONCLUSION: This study shows the possibility that signal-averaged electrocardiogram is more useful to detect myocarditis associated with KD than the other conventional tests. However, we could not define the prognostic value of abnormal signal-averaged electrocardiograms during this study period.

Newborn with transverse facial cleft associated with polyhydramnios.

We describe here the case of a female newborn baby with a bilateral complete transverse facial cleft. Obstetrical ultrasound had revealed an increased amount of amniotic fluid from 28 weeks' gestation without fetal hydrops or congenital anomalies. A 1900-g baby girl born at 36 weeks' gestation presented with bilateral wide facial clefts with macrostomia, microphthalmia, nose, and auricular deformities. Her breathing was dependent on life support, which was discontinued 2 hours after birth. An autopsy revealed no congenital malformations in vital organs but the absence of the olfactory nerves. Polyhydramnios and respiratory arrest after birth were presumed to be due to central disintegration of swallowing and breathing, in this case with brain anomaly.