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TOTAL AGANGLIONOSIS OF THE COLON (HIRSCHSPRUNG'S DISEASE) and CONGENITAL FAILURE OF AUTOMATIC CONTROL OF VENTILATION (ONDINE'S CURSE)
Author(s) -
STERN M.,
HELLWEGE H. H.,
GRÄVINGHOFF L.,
LAMBRECHT W.
Publication year - 1981
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1981.tb07184.x
Subject(s) - medicine , congenital central hypoventilation syndrome , hirschsprung's disease , ventilation (architecture) , surgery , gastroenterology , pediatrics , hypoventilation , disease , respiratory system , mechanical engineering , engineering
. Stern, M., Hellwege, H. H., Grävinghoff, L. and Lambrecht, W. (Department of Paediatrics and Department of Surgery, University Hospital Eppendorf, Hamburg, Federal Republic of Germany). Total aganglionosis of the colon (Hirschsprung's disease) and congenital failure of automatic control of ventilation (Ondine's curse). Acta Paediatr Scand, 70:121, 1981.–Total aganglionosis of the colon presenting with small intestinal obstruction in the neonatal period was observed in combination with congenital alveolar hypoventilation requiring continuous mechanical ventilation in a boy. The patient died aged 15 months from acute dehydration due to enteritis, long after total resection of the aganglionic bowel had been performed. Pulmonary hypertension was found in the newborn period. There was progressive right ventricular myocardial hypertrophy. This is the fourth case reported with a combination of defects involving nerve cell function of the brain stem and gastrointestinal tract.