ABSTRACT
BACKGROUND: Carnitine is essential for transporting long-chain fatty acids into mitochondria and promotes energy metabolism via ß-oxidation of long-chain fatty acids. Although carnitine is also present in the peripheral blood, 98% of total carnitine is stored in muscle tissue. Neuromuscular diseases accompanied by muscle atrophy are likely to lead to secondary carnitine deficiency, owing to the reduced amount of total carnitine stored in the body. CASE PRESENTATION: An 8-y-old Japanese boy with Fukuyama-type congenital muscular dystrophy accompanied by severe psychomotor retardation had been constantly bedridden, suffered from dysphagia, and had been fed through a gastrostomy tube since the age of 1 y. Regular oral carnitine supplementation (5 mg/kg/d of levocarnitine) was initiated at the age of 7 y, which increased serum carnitine value to within the normal range (serum total carnitine concentration, 58.5-60.9 µmol/L; acylcarnitine concentration, 45.8-55.0 µmol/L; free carnitine concentration, 5.9-12.7 µmol/L). He developed a fever, vomiting, and gastrointestinal bleeding at the age of 8 y. He fell into a coma and visited an emergency room 12 h later. Hypoglycemia and hypocarnitinemia (serum total carnitine concentration, 3.7 µmol/L; acylcarnitine concentration, 2.9 µmol/L; free carnitine concentration, 0.8 µmol/L; acyl-to-free carnitine ratio, 3.6) were observed, and he was found to be negative for urinary ketone bodies. CONCLUSIONS: Neuromuscular diseases accompanied by muscle atrophy may lead to acute carnitine deficiency, even if the serum carnitine concentration is within the normal range before onset. During sick days, it may be necessary to modify a patient's treatment, such as increasing both oral supplementation and intravenous administration of carnitine.
Subject(s)
Carnitine , Muscular Dystrophies , Male , Humans , Amino Acids , Fatty Acids , Muscular Atrophy , Gastrointestinal Hemorrhage , VomitingABSTRACT
This paper is an examination of irritable behaviour in very low-birth-weight infants in relation to caffeine or aminophylline. We assessed tremulous movement (a sub-score of General Movements Optimality Score) in 18 caffeine-treated subjects and 18 aminophylline-treated subjects. Caffeine, unlike aminophylline, was not associated with irritable behaviour at standard dose.
Subject(s)
Caffeine/adverse effects , Central Nervous System Stimulants/adverse effects , Infant Behavior , Infant, Very Low Birth Weight/physiology , Irritable Mood , Aminophylline/administration & dosage , Aminophylline/adverse effects , Aminophylline/therapeutic use , Apnea/drug therapy , Bronchodilator Agents/administration & dosage , Bronchodilator Agents/adverse effects , Bronchodilator Agents/therapeutic use , Caffeine/administration & dosage , Caffeine/therapeutic use , Central Nervous System Stimulants/administration & dosage , Central Nervous System Stimulants/therapeutic use , Female , Humans , Infant, Newborn , Infant, Premature, Diseases/drug therapy , Male , MovementABSTRACT
BACKGROUND: Following the increase in the number of children with food allergies, support systems are now required for school lunches, but a large-scale factual investigation has not been carried out. OBJECTIVE: We evaluated the features of elimination diet due to food allergy and the support system in kindergartens and schools. METHODS: A prefecture-based questionnaire survey regarding measures for food allergies in school lunches of all kindergartens, public elementary schools, and public junior high schools (631 facilities) was conducted in Oita Prefecture, Japan. RESULTS: The recovery rate of the questionnaire was 99.5%, which included 106,008 students in total. A total of 1,562 children (1.5%) required elimination diets. The rate of children on elimination diets in kindergartens and elementary/junior high schools that required medical certification by a physician was 1.2% (324 among 27,761 children), which was significantly lower than the 1.8% of children (1,227 among 68,576 students) on elimination diets at the request of guardians without the need for medical certification (p < 0.0001). A total of 43.9% of the kindergartens and schools said that they would contact guardians if symptoms were observed after accidental ingestion, while a low 8.1% stated that they provided support to children themselves, including the administration of adrenaline auto-injectors. CONCLUSION: Medical certification reduces the number of children requiring elimination diets, but it has not been adequately implemented. Furthermore, waiting to contact guardians after symptoms are observed may lead to the delayed treatment of anaphylaxis. Cooperation between physicians and teachers is desired to avoid the overdiagnosis and undertreatment of children with food allergies.
