This study intended to calculate the cephalometric norms of Korean adult and to compare those with norms of the North American Caucasian by Moyers. Cephalometric headplates of 41 males and 31 females ranged in age from eighteen to twenty-six with normal occlusion and pleasing face were employed for this investigation. The tracings of the standard lateral cephalograms were analyzed by Moyers' method. As a result of this study, the following conclusion can be made. 1. Norms of Korean adult male and female were calculated. 2. There was no significant sexual difference in the basic craniofacial morphology. 3. The size of craniofacial skeleton was larger in male than in female. 4. The Korean male showed lower value of mandibular plane angle to craial base than that of the Caucasian male. 5. There was no significant difference in the anterior total facial height, however, in the posterior facial height the Korean male was larger than the Caucasian male, which manifested that the Korean male held more square-shaped profile. 6. The anteroposterior length of anterior and posterior cranial base, maxillary and mandibular skeletal and dentoalveolar effective length of the Korean male were shorter than those of the Caucasian male, and this suggested that the craniofacial profile depth of the Korean male was shorter than that of the Caucasian male.
Adult* ; Female ; Humans ; Male ; Skeleton ; Skull Base

Isolated deficiency of 3-methylcrotonyl CoA carboxylase is a rare disorder of the catabolic pathway for leucine and many patients have mild symptoms or no symptom. However, the introduction of tandem mass spectrometry in newborn screening has revealed an unexpectedly high incidence of this disorder. We report an asymptomatic premature infant with isolated 3-methylcrotonyl CoA carboxylase deficiency detected by newborn screening program using tandem mass spectrometry. She was born at preterm, 36 weeks of gestation and her birth weight was 1,912 gm. She was delivered by Cesarian section due to maternal preeclampsia and oligohydramnios. An elevation of 3-hydroxyisovalerylcarnitine in a blood sample obtained at Seven days was detected by tandem mass screening. Massively elevated excretion of 3-hydroxyisovalerate and 3-methylcrotonylglycine was detected in the urine collected at 15 days. L-carnitine(100 mg/kg/day) was administrated orally to correct sencondary carnitine deficiency. Carnitine is conjugated with metabolites, to decrease the potential toxic effects. She is asymptomatic to date, and her growth and development are within normal limits.
Birth Weight ; Carnitine ; Female ; Growth and Development ; Humans ; Incidence ; Infant, Newborn* ; Infant, Premature ; Leucine ; Mass Screening* ; Oligohydramnios ; Pre-Eclampsia ; Pregnancy ; Tandem Mass Spectrometry*

PURPOSE: To compare the effect of breast milk containing fortifier with preterm formula on bone mineral metabolism in premature infants, we evaluated the state of bone mineralization, biochemical alteration and the frequency of rickets during the first five months of life. METHODS: Fourteen fortified breast milk-fed infants and eleven preterm formula-fed infants who were born at Il-Sin Christian Hospital from August, 1996 through July, 1997, were studied. The breast milk-fed group received human milk fortifier. The intake of Ca, P, Mg, protein, and fat was calculated at one month of age. Birth weight and weight at one, two and five months of age were measured. Serum Ca, P, alkaline phosphatase and 25-hydroxyvitamine D were measured at one month of age, and the wrist received X-ray examinations monthly. Total body bone mineral content was measured by Dual energy X-ray absorptiometry (Lunar WI) at two and five months of age. RESULTS: The two groups were similar in birth weight, gestational age, and weight at one, two and five months of age. Enteral Ca, P, protein, Mg, and fat intake, and urinary excretion of Ca, P were similar among the two groups. Serum Ca, P, ALP and 25-hydroxyvitamin D were not different. Occurrence of rickets and bone mineral content were similar among the two groups. CONCLUSION: Fortified breast milk-fed infants and preterm formula-fed infants showed no difference in total body bone mineral content and occurrence of rickets. Ultimately, we could enhance the advantage of breast milk fed to premature infants by adding fortifier.
Absorptiometry, Photon ; Alkaline Phosphatase ; Birth Weight ; Bone Density ; Breast ; Calcification, Physiologic ; Gestational Age ; Humans* ; Infant ; Infant, Newborn ; Infant, Premature ; Metabolism* ; Milk, Human* ; Rickets* ; Wrist

Citrullinemia is an inborn error of urea cycle metabolism caused by deficiency of arginosuccinate synthetase. It is characterized by hyperammonemia and high citrulline level in serum, CSF and urine. The clinical symptoms include vomiting, lethargy, seizure, coma and ultimately death if hyperammonemia is not controlled. We report a case of 9- day old male with citrullinemia who was initially treated with sodium benzoate during acute stage followed by gradual weaning to discontinuation. Hyperammonemia was well controlled by low protein milk diet and arginine.
Arginine* ; Citrulline ; Citrullinemia* ; Coma ; Diet* ; Humans ; Hyperammonemia ; Lethargy ; Ligases ; Male ; Metabolism ; Milk ; Seizures ; Sodium Benzoate ; Urea ; Vomiting ; Weaning

No abstract available.
Danazol* ; Panniculitis*

PURPOSE: The purpose of this study was to determine those factors which could contribute to the development of necrotizing enterocolitis(NEC) in fullterm. METHODS: We retrospectively reviewed the medical record of 20 full-terms with NEC(> or = modified Bell's staging criteria IIa) who were admitted to the Neonatal Intensive Care Unit of Il Sin Christian hospital from January 1998 through July 2005, and for each case, the next 2 healthy newborns were matched as controls. RESULTS: Mean gestational age and birth weight in the fullterm with NEC group was 38.42 weeks and 2,915 g; in the healthy fullterm without NEC group, it was 38.61 weeks and 3,148 g. When compared with the control group, NEC infants had a significantly higher frequency of chorioamnionitis, protracted diarrhea. As for Apgar score at 1 min <7, respiratory problem, congenital heart disease. there were no differences in frequency of preeclampsia, maternal diabetes, maternal drug abuse, meconium-stained amniotic fluid, polycythemia or exchange transfusion. CONCLUSION: Most of these full term infants have a predisposing factor before developing NEC. Our study suggested that NEC in fullterm infants was significantly associated with protracted diarrhea, and congenital heart disease.
Amniotic Fluid ; Apgar Score ; Birth Weight ; Causality ; Chorioamnionitis ; Diarrhea ; Enterocolitis, Necrotizing* ; Female ; Gestational Age ; Heart Defects, Congenital ; Humans ; Infant ; Infant, Newborn* ; Intensive Care, Neonatal ; Medical Records ; Polycythemia ; Pre-Eclampsia ; Pregnancy ; Retrospective Studies ; Risk Factors* ; Substance-Related Disorders

No abstract available.
Mucinoses*

No abstract available.
Chlorpromazine* ; Hyperpigmentation ; Pigmentation*

No abstract available.
Granuloma Annulare ; Rheumatoid Nodule

No abstract available.
Granuloma Annulare ; Rheumatoid Nodule