A Case of Raine Syndrome

Raine syndrome was described as an unknown syndrome in 1989. It is characterized by severe craniofacial anomalies with microcephaly, hypoplastic nose, depressed nasal bridge, exophthamos/protosis, gum hypertrophy, cleft palate, low-set ears, small mandible, narrow chest, wide cranial sutures and choanal atresia or stenosis, by generalized osteosclerosis with subperiosteal thickening of ribs, clavicles and diaphysis of long bones, and by intracranial calcifications in the particularly periventricular area. It undergoes an autosomal recessive inheritance. Twelve cases of Raine syndrome have been reported in the literature. However, a case of Raine syndrome in Korea has not been reported yet. Therefore, we describe a female newborn with Raine syndrome with a brief review of the literatures.

A Case of Scoliosis Due to a Foreign Body (Pencil) in the Colon

Scoliosis describes a lateral curvature of the spine and is often associated with cosmetic and functional impairments due to severe deformity of the spine. The incidence of adolescent scoliosis is 2-4% of children between 10 and 16 years of age. Eighty five percent of them are idiopathic, in which the most common type of scoliosis is right side-bending. In addition, it is classified into congenital, and secondary scoliosis such as neuromuscular disease and neurofibromatosis. Congenital scoliosis is associated with abnormalities of urinary system(20%), congenital heart diseases(15 %), and other abnormalities(10%) such as syringomyelia. We experienced a case of scoliosis by a foreign body(a pencil) in the colon which has never been reported up to date. A brief review of the literature was made.

Clinical Features of Food Protein-Induced Enterocolitis Syndrome

PURPOSE: Food protein-induced enterocolitis syndrome(FPIES) is a symptom complex of severe vomiting and diarrhea which is known as a food-related gastrointestinal hypersensitivity disorder without evidence of IgE-mediated sensitivity. We described the clinical characteristics of FPIES in young infants. METHODS: Twenty-eight patients, aged 7 to 120 days, were included who were diagnosed as FPIES by clinical criteria and food challenges. The clinical and laboratory features on admission and the findings from endoscopic biopsies were investigated. Food challenges with milk and soy were performed. RESULTS: Nineteen patients(67%) were admitted with diarrhea, four(15%) with vomiting and five (18%) with both vomiting and diarrhea. Fourteen patients(50%) were ill enough to require evaluation for sepsis but with negative results. Leukocytosis was noticed in twenty(71%), acute phase reactants increased in eighteen(64%), metabolic acidosis was observed in seventeen(61%) and hypoalbuminemia in twelve(43%). Duodenal biopsy specimens showed edema and acute inflammation in all patients. Villus atrophy was found in sixteen patients(57%) with no significant correlation between the degree of villus atrophy and the symptom severity. Diarrhea was elicited in 64% of total food challenges, vomiting and diarrhea in 25%, vomiting only in 7%. Shock state was developed in 25% of challenges. CONCLUSION: Our results suggest that food-related gastrointestinal hypersensitivity can cause a syndrome similar clinically to severe infection and FPIES should be suspected in young infants who have protracted diarrhea with or without vomiting.

Abruptio Placenta: Perinatal Outcome in Pregnancy-induced Hypertensive and Normotensive Pregnant Women / 대한산부인과학회잡지

OBJECTIVE: The objective of this study was to compare perinatal outcomes of pregnancy-induced hypertensive and normotensive women experiencing abruptio placentae, Our hypothesis is that pregnancy-induced hypertensive women have a less favorable perinatal outcome than do normotensive women. METHODS: Women with the diagnosis of abruptio placentae delivered between August 1, 1989 and December 1, 1996, composed the study group (n=92) in this case-control study. The women with abruptio placentae were divided according to their hypertensive (n=37) or normotensive (n=55) status. Maternal and neonatal medical records were reviewed and abstracted for demographic variables, antepartum complications, delivery route, abruptio placentae grade, neonatal gender, birth weight, Apgar score and perinatal mortality. We compared these perinatal outcome variables between the pregnancy-induced hypertensive and normotensive pregnant women. RESULTS: The incidence of abruptio placentae was 0.35%. The two groups of woman wne similar with regard to age and parity. Abruptio placentae grades 2 occurred more often in hypertensive women (P=0.0053). Pregnancy-inducedhypertensive women were similar to normotensive women with regard to antenatal complications. The mean gestational age of delivery, delivery route, neonatal weight and sex were similar between two groups of women. Neonates from pregnancy-induced hypertensive women were no more likely to have low 1 and 5-minute Apgar score or to die than those from normotensive women. Statistical analysis was performed with two-tailed independent t-test and Kruskal-Wallis analysis. CONCLUSION: Although pregnancy-induced hypertensive women experiencing abruptio placentae are more likely to have grade 2 abruptio placentae with fetal distress, the overall perinatal outcome was not significantly different from that of normotensive women experiencing abruptio placentae.

A Clinical Study about the Risk Factors of the Neonatal Clavicular Fracture / 대한산부인과학회잡지

No abstract available.