Monatsschr Kinderh
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Serial pulmonary function tests were performed in 13 preterm infants with severe RDS and 16 premature neonates with healthy lungs (8 intubated because of hypovention after birth, 8 were breathing spontaneously). Airflow was measured by a pneumotachograph, pressure changes were determined by airway pressure in ventilated infants or esophageal pressure in spontaneously breathing neonates. Pulmonary mechanics were calculated by a computerized system (PEDS/Medical Associated Services, Hatfield, Pennsylvania). ⋯ In the course of the disease, improvement in gas exchange preceded increase of compliance. Intraindividual comparisons in the acute and recovery phase of RDS and in infants with normal lungs showed higher values for compliance and lower values for airway pressure and resistance during spontaneous breaths. The differences between dynamic compliance of the respiratory system measured during mechanical ventilation, and dynamic lung compliance recorded during spontaneous breaths are due to influences of the respirator on the infant's lung.
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Pulmonary hypertension without any cardiovascular malformation was diagnosed by heart catheterization in a 4 year old girl with trisomy 21. A suspected obstructive sleep apnea syndrome was confirmed by polysomnography which revealed numerous obstructive apneas and hypopneas (apnea-index 23/h) with marked oxygen desaturation and a disturbed sleep pattern. Three months after adenotonsillectomy the mother reported her daughter having a quiet sleep without snoring. ⋯ Facial dysmorphias and muscle hypotonia predispose patients with trisomy 21 to obstructive sleep apnea, especially if hypertrophy of tonsills and adenoids coexist. Frequent arousals and hypoxia during sleep can result in failure to thrive and pulmonary hypertension. These consequences can be prevented by early diagnosis and treatment.
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Wolman's disease is a rare inherited disorder of lipid metabolism in which large amounts of triglycerides and cholesteryl esters accumulate in the visceral organs. The main clinical features of the infantile form of the disease are failure to thrive, vomiting and diarrhoea, hepatosplenomegaly and radiological evidence of calcification of the adrenals. We were able to follow the course of this disease in a female turkish infant. ⋯ At the age of 4.5 months she was readmitted with severe hepatosplenomegaly, hypochromic anemia and fever of unknown origin. Calcifications of the adrenals and lymphocytic vacuoles led to the diagnosis of Wolman's disease. Deficiency of acid lipase activity in leucocytes could establish this diagnosis.