A Case of Laurence-Moon-Biedl Syndrome / 대한소아소화기영양학회지

Laurence-Moon-Biedl syndrome is an autosomal recessive disorder characterized by obesity, hypogenitalism, polydactyly, mental retardation and retinitis pigmentosa. Occasionally, this syndrome is accompanied by renal anomaly, nystagmus, cataract, syndactyly, microcephaly, oxycephaly and congenital heart disease. Recently, we have experienced a 8-year-old female patient who has retinitis pigmentosa, obesity, and polydactyly. We report a case of Laurence-Moon-Biedl syndrome with a review of literature.

Survey on the Pattern of Food Intake during Early Childhood and Allergic Disease / 소아알레르기및호흡기학회지

PURPOSE: Change of foods intake during infancy and early childhood is being indicated as a causal factor for recent dramatic increase in the number of allergic diseases in children. The food allergens may have an important role for the development of allergic diseases including atopic dermatitis in children. Early prevention and treatment of atopic dermatitis are important to prevent of allergic disease later in life. METHODS: The questionaire survey about the diet patterns of their children and the parents' awareness and knowledge on the relationship between food and allergy was performed for the parents of 316 patients aged under 48 month old at 7 university hospitals (4 allergy clinics and 3 general clinics) or 4 private clinics in September, 2002. RESULTS: Formular fed is more common in all age group and it comprised over 70% in the group under age 6 month old in compare with breast milk fed. The mean age of the start of weaning was 5.2+/-1.8 months in a child with a history of allergy, and 4.6+/-2.0 months who had no allergy history. The most common reason to start weaning was nutritional supplement and the next was to train for adult foods. Fruit juice was the first introduced weaning food in most cases. Among 316 children, 108 children had experience of having grain diet, live diet or natural diet for better nutrition. More than half of the parents had relatively correct knowledge about allergy such as the relationship between food and allergy especially in the group with a allergic disease. Many parents started grain diet, live diet, a soup of bone, and the white of an egg to their children under 12 months of age. CONCLUSION: In general, most of the parents enrolled had relatively correct information about the time and the purpose of weaning. However, their knowledge about the kinds of food and time of first exposure is at issue to be pointed out. Especially as a point of view for early intervention of allergic disease, continued more active education about the food allergens and their relation to allergic diseases are strongly recommended.