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Purpose : Medium chain acyl-CoA dehydrogenase(MCAD) deficiency is an autosomal recessive disoder of β oxidation of fatty acids and characterized by episodic hypoglycemia, vomiting, convulsion, encephalopathy, apnea, and sudden death related to fasting or infection resembling Reye syndrome or sudden infant death syndrome. In acute stage, mortality rate is very high and survivors have significant risk of developmental disability and chronic somatic illness. However,... |
Purpose : The early and efficient diagnosis of neonatal sepsis still remains a difficult task. Reliable laboratory test is not available yet and treatment is mainly based on the physical appearance of infants. And high number of negative blood cultures in cases of clinically diagnosed sepsis further emphasize the need for a more reliable index for early diagnosis. Intercellular adhesion molecule 1(ICAM-1) has been... |
Purpose : The immature neonatal immune system is thought to result in increased risk of infection. Receptors for the Fcmoiety of IgG(FcγR) are important in antibodymediated clearance of microbes by monocytes/macrophages and immune regulation in lymphocytes. As an approach to understanding their role in neonatal life, we have compared the constitutive expression of the three Fc receptors-FcγRI(CD64), FcγRII(CD32) and FcγRIII(CD16)-by neonatal and adult blood monocytes... |
Purpose : Accurate diagnosis and prompt treatment of infection with Streptococcus pyogenes (group A streptococci) is required as it may cause many complications, and chronic serious sequelae, acute rheumatic fever and acute glomerulonephritis. Recently, the assay methods of ASO concentration tend to be changed into quantitatively from semi-quantitative or qualitative methods in this country. We would like to know relationships and degree of concurrences between... |
Purpose : To investigate the postoperative patterns of weight-gain in early infants with ventricular septal defects(VSDs), we analyze 48 patients who received primary surgical closure of the symptomatic VSDs during 6 months of age in the period of 1993 through 1995. Methods : Forty-sixt infants met criteria for inclusion in the study. The mean follow up period was 14 months(3 to 36 months). Weight... |
Purpose : Endoscopic retrograde cholangiopancreatography(ERCP) may provide more definite diagnosis, preoperative guidance, and endoscopic therapy in many children with known or suspected disorders of the pancreas and biliary tract. But the reports about the ERCP in children are rare, so this study is conducted to evaluate the indication, efficacy and safety of the ERCP in children. Methods : Twenty nine ERCPs in 19 children... |
Purpose : Vesicoureteral reflux(VUR) is the major cause of urinary tract infection (UTI) in children. Prolonged untreated UTI associated with VUR may result in serious complications, such as renal scarring, renal atropy, and decreased renal function. Therefore, follow up must be continued until UTI is cured completely, to prevent of serious complication. The authors conducted this study to evaluate results of the follow-up of... |
Purpose : It has been reported that patients with nephrotic syndrome have high serum IgE value and IL-4 involve in IgE synthesis, IL-6 is an autocrine growth factor for the proliferation of mesangial cells. We studied association between serum cytokines and of nephrotic syndrome. Methods : We measured serum IL-6, IL-4, IFN-Υ, CD23 in 14 children with nephrotic syndrome and 3 healthy children by... |
Purpose : Germ cell tumors have variable clinical characteristics according to the age, sex, primary site, and pathologic diagnosis. To provide the therapeutic principle, the clinical characteristics, response to the treatment, and prognostic factors were analyzed. Methods : Medical records of 57 children who were diagnosed as malignant germ cell tumor in Seoul National University Children' s Hospital from January 1986 till June 1996 were... |
Background : Gene transfer with vectors derived from murine retroviruses is restricted to cells which are proliferating and synthesizing DNA at the time of infection. Accordingly, selective introduction of genes encoding for susceptibility to otherwise nontoxic drugs(suicide genes) into proliferating tumor may be used to treat cancer. We investigated the efficacy of in vitro transduction of neuroblastoma cell with the herpes simplex-thymidine kinase(HSV-tk) gene followed... |
Purpose : Many studies showed that the asthmatic children mostly have psychosocial problem such as depression or anxiety. The object of this study is to help the asthmatic children and their family by knowing what is their mothers‘ biggest concern about their asthmatic children. Methods : We made up a questionnaire with asked the mothers by telephone. If they though asthma had a certain effect... |
Purpose : Acute septic arthritis in infancy and childhood is uncommon. Delay of diagnosis and inappropriate treatment of septic arthritis results in permanent physical sequelae. We studied the initial treatment, clinical manifestations, involved sites, and etiologic organisms of septic arthritis. Methods : We reviewed 74 patients who were diagnosed septic arthritis in Departments of Pediatrics and Orthopedic Surgery, Yonsei University College of Medicine from July 1986... |
Lipoid pneumonia is a chronic, interstitial, proliferative inflammation resulting from aspiration of lipoid material and occurs principally in debilated infants. We experienced a case of lipoid pneumonia with hypercalcemia in a 15 month-old boy who showed irritability, poor weight gain, cough, dyspnea, fatigue, diarrhea and pale face after ingestion of SqualeneⓇ(shark liver oil : 20-30 cap/day) by force for 2 months. Calcium preparation was also... |
Protein losing entropathy was a secondary disorder of the gastrointestinal tract, which is associated with lymphatic obstruction, inflammatory and noninflammatroy disease. It' s clinical manifestations were abdominal pain, edema, diarrhea and growth failure. We described a 12-year-old boy, who had chronic diarrhea for 3 months and edema of lower extremities for 7 days. He also had severe hypoalbuminemia, iron definency anemia, liver diseases and malnurition,... |
von Gierke disease(type Ia glycogen storage disease) is an inherited disease associated with accumulation of glycogen in the liver, kidney, intestine and erythrocytes due to the defect of glucose-6-phosphatase activity. Hepatomegaly, doll face, anemia, bleeding tendency and increased susceptability to infection are common features observed during infancy. Hypoglycemia especially fasting hypoglycemia is typical metabolic derangement in this disease, followed by metabolic acidosis, lactic acidemia, hyperlipidemia, hyperuricemia,... |