Résumé
Carnitine (beta-hydroxy-gamma-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic jaundice and poor weight gain, and who responded dramatically to oral carnitine supplementation.
Sujets)
Humains , Nouveau-né , Carnitine , Esters , Acides gras , Ictère rétentionnel , Naissance vivante , Prise de poidsRésumé
Carnitine (beta-hydroxy-gamma-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic jaundice and poor weight gain, and who responded dramatically to oral carnitine supplementation.
Sujets)
Humains , Nouveau-né , Carnitine , Esters , Acides gras , Ictère rétentionnel , Naissance vivante , Prise de poidsRésumé
BACKGROUND AND OBJECTIVES: A history of poor weight gain can often be elicited in young children with chronic upper airway obstruction resulting from adenotonsillar hypertrophy. The purpose of this study was to determine the incidence of growth disturbance and what effect, if any tonsillectomy and/or adenoidectomy has on subsequent growth in a group of children. MATERIALS AND METHODS: The subjects consisted of 78 patients with adenotonsillar hypertrophy who had been treated between March 1997 and September 1997 at the department of otolaryngology, Saint Benedict Hospital. Available preoperative data were collected including sex, age at surgery, tonsillar hypertrophy grade, presence of snoring and sleep apnea, preoperative weight, preoperative eating disorder, and URI frequency. RESULT: Many had improvements in growth after adenotonsil-lectomy. The improvement in growth appears to be obvious in chidren with eating disorder, snoring, and sleep apnea resulting from adenotonsillar hypertrophy. CONCLUSION: We can conclude that there exists a relationship beween adenotonsillar hypertrophy children and preoperative eating disorder, growth delay and snoring.