A case of transient neonatal diabetes mellitus combined with congenital adrenal hyperplasia(CAH) is described. A female infant was born by cesarean delivery due to fetal distress, she had sunken eyeball and anterior fontanelle, large protruded tongue and thin subcutaneous tissues. She had large clitoris and progressive pigmentation on whole body was observed since 10th day of birth. Hyperglycemia and glycosuria was noted at 3rd day of birth. Level of insulin and C-peptide was 3.0 mU/L and 0.35 ng/mL respectively. Serum ACTH was 870.4 pg/mL and 17-hydroxyprogesterone was increased to 20,000 ng/dL. Serum Na was 124 mEq/L, K 5.6 mEq/L. Abdominal MRI showed no abnormality. Chromosomal study showed 46,XX. Genetic analysis with polymorphic DNA markers for chromosome 6 showed paternal uniparental isodisomy at D6S276, D6S1704 and DNA analysis of CYP 21 gene showed mutation at P435S. She required insulin therapy for 8 months after birth. Hydrocortisone and florinef was needed for the control of CAH.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Adrenocorticotropic Hormone ; C-Peptide ; Chromosomes, Human, Pair 6 ; Clitoris ; Cranial Fontanelles ; Diabetes Mellitus* ; DNA ; Female ; Fetal Distress ; Genetic Markers ; Glycosuria ; Humans ; Hydrocortisone ; Hyperglycemia ; Infant ; Insulin ; Magnetic Resonance Imaging ; Parturition ; Pigmentation ; Subcutaneous Tissue ; Tongue ; Uniparental Disomy

No abstract available.
Animals ; Hemorrhagic Fever with Renal Syndrome* ; Rats* ; Singapore*

Bed-side measurement of Vd/Vt has not been used clinically, because the measurement of Pico2 in using the Bohr equation is complicated and a time-consuming test. A new, simplified and time-saving, bed-side calculation of Vd/Vt by using the Radford nomogram has been developed. In order to verify the method of calculated Vd/Vt, we compared the two methods(measured versus calculated Vd/Vt) in 20 open heart patients. Two methods had direct correlationship(y=0.95X+0.04) with r=0.864, and so the authors considered that the calculating method in the measurement of Vd/Vt is advantageous in clinical use.
Heart ; Humans ; Nomograms

The clinical analysis was performed on 730 critically ill patients under 15 years old who had been admitted in respiratory intensive care unit(RICU) from January, 1983 to December 1984. 1) The mortality rates in 1983 and 1984 were 8.7% and 5.9% and the number of patients was increased but the mortality rate was decreased than before 1983. 2) the mortality was significantly high in neonates and infants but gradually decreased with age. 3) Of 730 patients, 30 of 437 male and 24 of 393 female patients were dead. 4) Of 730 patients, 667 were the patients of chest surgery and 56 were the patients of pediatric surgery but 7 were the patients in the departments other than in chest surgery or pediatric surgery. The Mortalities in chest surgery, pediatric surgery and other surgical departments were 5.4%(36 patients) 26.8%(15 patients) and 37.5%(3 patients). 5) Of 667 patients in department of chest surgery, 640 were the patients with the congenital heart disease and the mortality was 5.2%(33 patients) but 12 were the patients with acquired heart disease and the mortality was 16.7%(2 patients). Of 667 patients, 15 were the patients with noncardiac disease and mortality was 0.1%(1 patients). 6) The duration of the mechanical ventilation in RIDU patients, was 54.5 hours on the average and in the patients of mechanical ventilation more than 72 hours, the mortality was significantly increased compared with the patients of mechanical ventilation less than 72 hours. 7) The major causes of the death in RICU patients under 15 yearts old were LCOS(low cardiac output syndrome) and sepsis.
Adolescent ; Cardiac Output ; Critical Illness ; Female ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant ; Infant, Newborn ; Critical Care ; Male ; Mortality ; Respiration, Artificial ; Sepsis ; Thorax