Associations of IGF-I, IGFBP-3 on early Catch-up Growth in Small for Gestational Age Infants / 대한주산의학회잡지

OBJECTIVE: This study was aimed to analyze the effect of Insulin-like growth factor (IGF)-I and Insulin-like growth factor binding protein (IGFBP)-3 on early catch-up growth in small for gestational age (SGA) infants. METHODS: We prospectively studied in 18 SGA infants who were admitted at Chungnam National University Hospital from January 2004 to January 2006. We divided them into two groups of intrauterine growth retardation; symmetric and asymmetric type. Maternal and neonatal characteristics and the value of IGF-1 and IGFBP-3 at birth were compared between the two groups. After 12 months later, we checked their body weight and height to evaluate catch-up growth. RESULTS: Among 18 SGA infants, 5 were male and 13 were female. Average gestational age at birth was 38.4+/-1.1 weeks. Average birth weight was 2,359+/-154 g. Nine babies showed symmetric intrauterine growth retardation. Thirteen babies achieved catch-up growth at 12 month of age. There was no correlation between the characteristics of SGA and catch-up growth. However, the IGF-I and IGFBP-3 values were significantly higher in babies who showed catch-up growth. CONCLUSION: We were able to predict catch-up growth at early infancy by evaluating serum IGF-I and IGFBP-3 values at birth in SGA infants.

A Case of Fanconi Anemia Diagnosed by a Chromosome Breakage Test with Skin Fibroblasts / 대한혈액학회지

Fanconi anemia is an autosomal recessive disease that's characterized by congenital anomalies, defective hematopoiesis and a high risk of developing acute myeloid leukemia and certain solid tumors. The clinical phenotype is extremely variable; therefore, the diagnosis is frequently delayed until the pancytopenia appears. Chromosomal instability, especially on exposure to an alkylating agent, may be seen in affected patients and it is the basis for a diagnostic test. This cellular phenotype can be demonstrated in cultured T cells, B cells, fibroblasts and fetal cells cultured from both amniotic fluid and chorionic villi. But somatic mosaicism may make the diagnosis of Fanconi anemia difficult because of inconclusive chromosome breakage studies. If the test is negative in lymphocytes and yet the clinical setting is highly suspicious, then the skin fibroblasts must be assessed. Because skin fibroblasts are somatic cells, a definitive test can be performed on primary skin fibroblasts. In this report we describe a case of Fanconi anemia that was diagnosed with the use of cultured skin fibroblasts, and this was despite the normal breakage studies in the peripheral blood.

Prenatal diagnosis and clinical course of restrictive foramen ovale in otherwise normal heart / 소아과

PURPOSE: Premature narrowing of the foramen ovale is rare but serious clinical entity. Prenatal narrowing or obstruction of the foramen ovale shows symptoms such as right heart failure, fetal hydrops, triscupid regurgitation, left heart obstructive disease, and supraventricular tachycardia. This study aimed to assess the prenatal diagnosis and postnatal clinical course of restrictive foramen ovale in utero in otherwise normal heart. METHODS: The subjects were five patients diagnosed with restrictive foramen ovale in utero from January 2001 to June 2005 at Chungnam National University Hospital. The diagnostic criteria was defined when the maximum diameter in a 4-chamber view is less than 2.5 mm and there is a continuous doppler velocity at the foramen ovale of more than 0.6m/s. RESULTS: At the time of diagnosis of restrictive foramen ovale, gestation age was 34~37 wks, and chief complaints were fetal arrhythmia(2 cases), pericardial effusion, Ebstein anomaly and subaortic stenosis. Two cases which were diagnosed fetal hydrops and supraventricular tachycardia delivered by emergent cesarian section. Five cases were found to have right heart dilatation on echocardiogram after birth, but right heart dilatation became normalized at day 7 after birth and the clinical courses were not eventful. CONCLUSION: Identifying an obstructed foramen ovale in the fetus warrants the further search for additional cardiac and extracardiac anomalies, which may alter the prognosis. Delivery should be induced if possible in cases of foramen ovale obstruction with signs of cardiac decompensation.

A Case of Catheter-related Thrombosis Treated with Local Low-dose Urokinase / 대한혈액학회지

Although indwelling central venous catheters guarantee a reliable vascular access, and are essential for the management of children undergoing anticancer chemotherapy or stem cell transplantation, these catheters may cause serious mechanical, infectious and thrombotic complications. Central venous catheter-related thrombosis is one of the most important complications that may interfere with the course of treatment. A number of regimens utilizing urokinase have been used but the optimum management of this common problem remains undetermined. We report an 8 year-old boy, who had catheter-related atrial thrombus treated successfully with urokinase. A short course treatment with the use of low-dose urokinase was feasible for the thrombolysis of catheter-related right atrial thrombus in this boy diagnosed with neuroblastoma and undergoing high-dose chemotherapy with autologous peripheral blood stem cell rescue. This treatment was not associated with bleeding.

A Case of Chronic Active Epstein-Barr Virus Infection with Autoimmnune Hepatitis and a Coronary Aneurysm / 대한혈액학회지

Infectious mononucleosis caused by primary infection of Epstein-Barr virus (EBV) is a self-limiting lymphoproliferative disease, and shows concomitant clinical features such as pyrexia, anorexia, sore throat, cervical lymphadenopathy, liver dysfunction and hepatosplenomegaly. In rare cases, EBV establishes a latent infection in B lymphocytes and runs a chronic course and shows infectious mononucleosis-like symptoms repeatedly. This syndrome, named chronic active EBV infection, may trigger an autoimmune disease that mainly affectes the liver and red blood cells, and carries a very poor prognosis. The cardiovascular complications of chronic active EBV infection are very rare and may be associated with coronary arterial disease. This case describes a 5-year-old boy, who developed chronic active EBV infection and was diagnosed as having autoimmune hepatitis with a coronary aneurysm.