We report a one-day-old Korean boy with 2-Methylbutyryl-CoA dehydrogenase(2-MBCDase) deficiency detected by urine organic acid and acylglycine analysis, plasma acylcarnitine analysis and confirmed by enzyme assay and Western blot. The patient was born at 35 weeks and three days with oligohydroamnios and premature rupture of membrane for 31 hours, as a second child of healthy non-consanguineous parents. There was no... |
Purpose : Several methods of IV γ-globulin(IVG) infusion are effective in the treatment of autoimmune disease, including idiopathic thrombocytopenic purpura(ITP). But it is not known which method is more effective in the treatment of ITP. The effectiveness of these two methods of IVG infusion was studied in terms of platelet recovery rate, side effects and recurrence rate. Methods : Forty seven... |
A 23-month-old girl visited with chronic cough and her chest radiograph showed miliary tuberculosis. There was no neurological abnormality. But CSF findings showed WBC 22/mm3(lymphocyte 20%, neutrophil 80%) and positive result of polymease chain reaction(PCR) for M. tuberculosis. MR imaging showed multiple ring enhanced nodules and ovoid nonenhancing bright signal lesion on the cerebrum, cerebellar parenchyme, and left basal ganglia.... |
Purpose : It is important to diagnose and treat newborn patients with congenital hypothyroidism as soon as possible because of neurodevelopmental outcome. If we can detect more severe forms of congenital hypothyroidism with neonatal screening test, the results of treatment will improve. Methods : Sixty-four term infants whose TSH levels in neonatal screening test had been higher than 20μIU were recalled.... |
Purpose : To determine the effects of massage therapy on growth, development, hormones, immune function, hepatic function, hematopoietic function and sleep pattern of preterm infants. Methods : Thirty-one preterm infants of less than 35 weeks gestational age, who were admitted to Eulji Medical College Hospital Neonatal Intensive Care Unit between August 1998 and May 1999, and were in the state without mechanical ventilation or... |
Megacystis Microcolon Intestinal Hypoperistalsis(MMIH) Syndrome is a rare cause of functional neonatal bowel obstruction, characterized by hypoperistalsis, narrow distal ileum and colon, and bladder distension. We report a case of MMIH syndrome and review the literature. The patient was born after 34 weeks of gestation with marked abdominal distension. Her birth weight was 2,830g with 276ml of urine drained by... |
Purpose : Rotavirus infection is a leading cause of severe gastroenteritis among infants and young children worldwide. In temperate regions, institutional outbreaks of the disease occur mainly in cold dry weather, whereas in tropical settings its seasonal course is less well defined. We studied the annual changes in the seasonal prevalence of rotavirus infection for 5 years. Methods : The study was conducted on... |
Purpose : To investigate the cut-off value of creatine kinase(CK) BB according to blood sampling time in predicting neonatal prognosis, we studied the time-related releasing pattern of CK isoenzymes after birth asphyxia. Methods : CK was measured within 1 hour and at 6-10 hours after birth from 45 newborn infants who were suspected to be perinatal asphyxia or fetal distress. The... |
We experienced a case of DiGeorge syndrome in a 25-day-old male infant presented with micrognathia, short neck, fish-shaped mouth and intractable seizures with a loading dose of phenobarbital & dilantin. The serum calcium level was 3.7mg/dl, ionized calcium level was 0.62mmol/L, and parathyroid hormone carboxy-terminal level was 0.01ng/ml. We treated with it calcium gluconate infusion, low phosphorous formula milk feeding, and... |
A five-year-old boy received a blunt trauma on chest by a kindergarten bus on Feb. 29, 1996. Pulmonary hemorrhage and pericardiac effusion were developed, followed by multi-organ failure threatening his life. All symptoms were improving when pansystolic harsh murmur(Ⅲ/Ⅵ) originating from a tiny ventricular rupture with a blood leak to the pericardial space was auscultated on the 12th day after the... |
Since Feingold and his collegues first describes the trisomy 9 syndrome in 1973, approximately 30 patients with trisomy of the chromsome 9 have been described. Trisomy 9 has been reported as either partial or complete. Complete trisomy is rare and incompatible with a a long life. Before this report, this syndrome has not been reported in Korea. A neonate was diagniosed... |
Purpose : Since the effect of intravenous immunoglobulin (IVIG) therapy in Kawasaki disease was reported in 1984, the combined therapy of IVIG & oral aspirin has been popular. In early period, the protocol of 400mg/kg/day for 3-5 days of IVIG had been used, but rescently, the protocol of 2g/kg/day single dose has been preferred. So authers performed a clinical study to compare th efficacies... |
We have experienced a case of congenital pyloric atresia associated with epidermolysis bullosa in a premature newborn who was born at the gestation period of 33+3 week. She showed a few blisters on left ankle at birth and the easy formation of blisters involving the area of trauma or friction with depigmentation after healing. The histologic finding of the lesion showed junctional epidermolysis bullosa. Abdominal... |
Purpose : To investigate the usefulness of the serum Creatine kinase(CK), especially brain typical creatine kinase(CK-BB) as an indicator of brain damage extent, mortality and long term nurologic sequelae, the study was done. Methods : CK and CK isoenzyme activites were measured at 6-10 hours after birth in 160 asphyxiated newborn infants who admitted in Eulji General Hospital from August 1990 to July 1994. Infants... |
Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly involving optic nerve hypolasia, midline anomalies of the brain and variable hypothalamicpituitary dysfunction. We experienced a case of septo-optic dysplasia in 56-day old male infant who presented with prolonged jaundice. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the ophthalmoscopic examination... |
Purpose : Iron deficiency anemia(IDA) is the most common nutritional deficiency. in young infants. This study was designed to determine a correlation between the duration of breast feeding and the severity of IDA, and to determine the recommending start point of iron fortified diet. Methods : The correlation between duration of breast feeding, and severity of iron deficiency. and IDA was... |
Creatine kinase (CK) isoenzyme are considered as a specific tissue injury marker. Brain tyupe isoenzyme of CK (CK-BB) was measured in 44 healthy and 33 asphyxiated preterm and fullterm infants. Samples were drawn from peripheral blood at six to ten hours after birth. There were no difference of CK-BB and CK-BB% between preterm and fullterm infants. CK-BB and CK-BB% were well... |
The measurement of blood pressure in neonates is an important diagnostic procedure. But the measurement of blood pressure has not been performed routinely because of difficulty in measuring blood pressure and varable normal range according to measuring apparatus. Recently some accurate and convenient apparatus of measuring blood pressure have been introduced in neonatal care, so the reference values... |
We experienced a case of rhinocerebral form of mucormycosis in a 9-year-old male suffered from acute lymphocytic leukemia(FAB L2). On 15th day of induction chemotherapy(Hospital day 23) pain, tenderness and swelling on left maxillary sinus. He expired on Hospital day 47. |
This study was undertaken to evaluate the Natueral-Killer (NK) cell and Lymphokine-Activated-Killer (LAK) cell activity of cancer patients for the prupose of understanding of spontaneous cell-mediated cytotoxicity (SCMC) in cancer patients and understanding of interrelationship between NK cell and LAK cell. Total 9 cases of malignant tumor (7 cases: Acute leukemia, 2 cases: malignant lymphoma) below 15 years of age, who were on remission... |