PURPOSE: We tried to find the relationships between the fetal beta-cell function and neonatal birth weight, anthropometric measures, and maternal characteristics, and to observe role of insulin as fetal growth factor in offsprings of mothers who have normal glucose metabolism. METHODS: We measured umbilical cord blood insulin and C-peptide concentrations in 236 neonates delivered at Cheil General Hospital from Jan. 1 to Jan. 31 1994. Their mothers had normal glucose metabolism during pregnancy. The birth weight and other anthropometric measures were performed within 24 hours after birth by same pediatrician. RESULTS: 1) The mean gestational age was 39.5+/--0.1 wk and mean birth weight was 3.3+/--0.2 Kg. According to birth weight percentile, the numbers of small for gestational age(SGA), adequete for gesational(AGA), and large for gestational age(LGA) infans were 3(1.3%), 178(75.4%), and 55(23.3%), respectively. There were no differences in gestational age, birth weight, length, head circumference, chest circumference, Ponderal index, and symmerty index between male and female infants. 2) The maternal weight gain during pregnancy was related to neonatal birth weight, length, and head circumference, but not to Ponderal index and symmetry index. 3) The mean cord blood insulin and C-peptide concentrations were 5.1+/--0.1uU/ml and 1.3+/--0.1 ng/ml. There was a significant positive correlation between insulin and C-peptide concentrations. 4) There were no significant differences in cord blood insulin and C-peptide concentrations among the SGA, AGA, and LGA group. 5) The cord blood insulin and C-peptide concentrations had weak positive correlations with neonatal birth weight, obesity, and gestational age, but nor with maternal weight gain during pregnancy. CONCLUSIONS: Although insulin has a significant role in fetal growth in diabetic pregnancy or intrauterine growth retardation(IUGR), our results suggest that it does not have a significant effect on fetal growth in offsprings of mothers who have normal glucose metabolism.
Birth Weight ; C-Peptide ; Female ; Fetal Blood* ; Fetal Development ; Gestational Age ; Glucose* ; Head ; Hospitals, General ; Humans ; Infant ; Infant, Newborn ; Insulin ; Male ; Metabolism* ; Mothers* ; Obesity ; Parturition ; Pregnancy ; Thorax ; Umbilical Cord* ; Weight Gain

We report 4 cases of malignant thymoma which were composed of 2 cases of invasive thymoma and 2 cases of thymic carcinoma. The cytologic findings of invasive thymoma were similar to those of benign thymoma. The distinctive cytologic features of thymic carcinoma were necrotic background, irregular clusters and individually scattered arrangement of anaplastic epithelial cells, and some scattered mature small lymphocytes. These findings may be found in the Hodgkin's lymphoma, seminoma, and metastatic squamous cell carcinoma, undifferentiated carcinoma, and large cell carcinoma of the lung. But, the feature of irregular clustering of anaplastic epithelial cell having scanty cytoplasm was different from Hodgkin's lymphoma and seminoma. Clinical and radiologic findings as well as cytologic finding were helpful in differential diagnosis of thymic carcinoma from metastatic carcinoma.
Breast ; Carcinoma ; Carcinoma, Large Cell ; Carcinoma, Squamous Cell ; Child* ; Cytoplasm ; Diagnosis, Differential ; Epithelial Cells ; Hodgkin Disease ; Humans ; Infant* ; Lung ; Lymphocytes ; Seminoma ; Thymoma ; Urinary Tract Infections* ; Urinary Tract*

PURPOSE: Persistent hyperinsulinemic hypoglycemia of infancy(PHHI), which is characterised by inappropriate insulin secretion in spite of hypoglycemia, needs urgent treatment to prevent cerebral hypoglycemic damage. Although pancreatectomy is the treatment of choice for PHHI, there are several complications which follow treatment. We suggest that aggressive medical therapy, when effective, is preferable to partial pancreatectomy. METHODS: We evaluated 8 patients with PHHI admitted to the Department of Pediatrics, Samsung Medical Center from November 1996 to January 1999. Children with hypoglycemia in the range of 3-50mg/dl were included. Octreotide was administered at dosage of 100-150 microgram/day. When the patients did not respond to octreotide, diazoxide and nifedipine were given in addition. RESULTS: In four of eight patients, octreotide was discontinued after 15 to 165 days. One patient was given diazoxide instead. The remaining 3 patients are still being treated with octreotide. CONCLUSION: We believe that maximum effort should be made to attain euglycemia with medication, and pancreatectomy should be reserved for patients in whom these measures fail to restore normoglycemia.
Child* ; Congenital Hyperinsulinism* ; Diazoxide ; Humans ; Hypoglycemia ; Insulin ; Nifedipine ; Octreotide ; Pancreatectomy ; Pediatrics

PURPOSE: This study was done to evaluate the parents' or guardians' compliance of obtaining individual informed consent before vaccination and the possible problems of using informed consent. METHODS: One hundred and twelve children were randomly selected among 2,820 children who visited Samsung Cheil Hospital during July 2002. A questionnaire about using informed consent of vaccination was given to all parents or guardians of these children. RESULTS: Most of the responders were mothers(80.4%). As for the places of vaccination, 87(77.7%) persons vaccinated their children at general hospitals. In response to a request for informed consent of vaccination, 73(65.2%) complied well with requests every time. In subscribing to informed consent, 62(55.4%) were pleased, 40(35.7%) stated it was necessary despite in inconveniance. Because of dislike of subscribing to informed consent(55), 33(60%) persons stated that it was difficult to subscribe to informed consent during the inconveniant time of nursing baby. Eighty(71.4%) found it useful to read informed consent for an understanding of potential negative reactions. Being informed of potential negative reactions, 105(93.8%) stated that they would vaccinate their children despite of the potential of a worrisome negative reaction. In dealing with negative reaction, 77(68.8%) said that they would contact a hospital, 32(28.6%) said that they would care for their children at home. Eleven(9.8 %) experienced negative reactions after vaccination. CONCLUSION: Compliance of parents to informed consent was relatively good. Parents or guardians should receive relevant information and the aim of informed consent also should be adequately understood.
Child* ; Compliance ; Hospitals, General ; Humans ; Informed Consent ; Nursing ; Parents* ; Surveys and Questionnaires* ; Vaccination*

PURPOSE: Congenital adrenal hyperplasia(CAH), which is classified into salt-wasting, simple virilization and non-classic type according to clinical features, is difficult to detect in early stages. Failure to diagnose it in the initial state may lead to life-threatening adrenal crisis, inappropriate male sex assignment in the genetic female, acceleration of skeletal maturation and subsequent short stature. Therefore, we studied the variables increasing the 17-hydroxyprogesterone(OHP) values for more specific and sensitive diagnosis of CAH. METHODS: We classified 3,532 newborns into variable factors; gestational age, birth weight, gender, delivery type, sampling date and stress. Then, we analysed the relationships between 17-OHP values and variable factors. RESULTS: The mean value of 17-OHP was 4.21+/-0.03ng/ml. There were significant differences among the variable factors except gender. The mean value of male was 4.26ng/ml, and that of female was 4.15ng/ml(P=0.10). The mean value of 17-OHP in vaginal delivered newborn was higher than C-section delivered ones(4.71ng/ml, 3.34ng/ml, P=0.0001). It was also higher in low birth weight(P=0.0001), in prematurity(P=0.001), those sampled within 4 days(P=0.0001), stressful condition and ventilator care-assisted(P=0.004). CONCLUSION: 17-OHP value in neonatal screening is influenced by several variables such as vaginal delivery, ventilator management, low birth weight, sampling date and prematurity. If the 17-OHP value is increased, we have to consider the variables influencing the increase in value and follow up with time interval or analysis of genetic mutations.
17-alpha-Hydroxyprogesterone* ; Acceleration ; Adrenal Hyperplasia, Congenital* ; Birth Weight ; Diagnosis ; Female ; Follow-Up Studies ; Gestational Age ; Humans ; Infant, Low Birth Weight ; Infant, Newborn* ; Male ; Mass Screening* ; Neonatal Screening ; Parturition ; Ventilators, Mechanical ; Virilism

PURPOSE: Congenital adrenal hyperplasia(CAH), which is classified into salt-wasting, simple virilization and non-classic type according to clinical features, is difficult to detect in early stages. Failure to diagnose it in the initial state may lead to life-threatening adrenal crisis, inappropriate male sex assignment in the genetic female, acceleration of skeletal maturation and subsequent short stature. Therefore, we studied the variables increasing the 17-hydroxyprogesterone(OHP) values for more specific and sensitive diagnosis of CAH. METHODS: We classified 3,532 newborns into variable factors; gestational age, birth weight, gender, delivery type, sampling date and stress. Then, we analysed the relationships between 17-OHP values and variable factors. RESULTS: The mean value of 17-OHP was 4.21+/-0.03ng/ml. There were significant differences among the variable factors except gender. The mean value of male was 4.26ng/ml, and that of female was 4.15ng/ml(P=0.10). The mean value of 17-OHP in vaginal delivered newborn was higher than C-section delivered ones(4.71ng/ml, 3.34ng/ml, P=0.0001). It was also higher in low birth weight(P=0.0001), in prematurity(P=0.001), those sampled within 4 days(P=0.0001), stressful condition and ventilator care-assisted(P=0.004). CONCLUSION: 17-OHP value in neonatal screening is influenced by several variables such as vaginal delivery, ventilator management, low birth weight, sampling date and prematurity. If the 17-OHP value is increased, we have to consider the variables influencing the increase in value and follow up with time interval or analysis of genetic mutations.
17-alpha-Hydroxyprogesterone* ; Acceleration ; Adrenal Hyperplasia, Congenital* ; Birth Weight ; Diagnosis ; Female ; Follow-Up Studies ; Gestational Age ; Humans ; Infant, Low Birth Weight ; Infant, Newborn* ; Male ; Mass Screening* ; Neonatal Screening ; Parturition ; Ventilators, Mechanical ; Virilism

BACKGROUND: Congenital adrenal hyperplasia (CAH) results from an inherited defect in enzymatic steps required to synthesize cortisol from cholesterol. 21-hydroxylase deficiency accounts for 95% cases of CAH. It appears that the frequency and the type of the responsible mutations differ according to the ethnic background and the type of mutation can predict the clinical outcomes such as salt losing type (SL), simple virilizing type (SV) and non-classic type (NC). METHODS: We have analyzed CYP21 genes in 55 Korean cases (110 chromosomes) of CAH by Southern blotting, PCR-dot hybridization and PCR amplification-created restriction site method. The patients include 43 cases of SL and 12 of SV. None of the NC was found. RESULTS: We found the mutations in 94% (103/110) of the examined chromosomes. A total of 10 types of mutations were discovered. The mutations include aberrant splicing of intron 2 (i2, 35%), CYP21 gene deletion (32%) and I172N (11%) in order. When the relationship between the clinical types and genotypes were correlated, most of the SL patients have either i2 (42%) or CYP21 gene deletion (41%), while SV patients have I172N (33%) or P30L (21%). The parents' mutation was investigated in 20 cases. In 4 families, one of the parents was not the obligatory heterozygote carrier i.e. did not have a mutation. The results suggest the high incidence of de novo mutation. CONCLUSION: We have identified the frequency of mutations of the CYP21 in Korean AGS patients. Our results shows that the clinical type of AGS can be predicted from the genotypes of CYP21. Also the high incidence of de novo mutation of CYP21 confirmed the genetic instability of major histocompatibility III region where the CYP21 is located.
Adrenal Hyperplasia, Congenital ; Adrenogenital Syndrome* ; Blotting, Southern ; Cholesterol ; Gene Deletion ; Genotype* ; Heterozygote ; Histocompatibility ; Humans ; Hydrocortisone ; Incidence ; Introns ; Parents ; Phenotype* ; Polymerase Chain Reaction ; Steroid 21-Hydroxylase*