Factor Ⅶ deficiency has an estimated incidence of 1/500,000 in the general population and autosomal recessive pattern of inheritance. Factor Ⅶ deficiency is characterized by prolonged prothrombin time(PT), and normal activated partial thromboplastin time(aPTT) and bleeding time(BT). Definite diagnosis of this condition requires a specific Factor Ⅶ assay. The clinical features are variable and do not always correlate with the... |
Purpose : The renin-angiotensin system plays an important role in renal growth and development. Exposuring the neonate to angiotensin converting enzyme(ACE) inhibitor increases mortality and results in growth retardation and abnormal renal development. ACE inhibition in the developing kidney reduces the renal expression of TGF-β1 and EGF, which may account for renal growth impairment. This study was designed to... |
Purpose : The aim of this study was to evaluate the clinical efficiency of cardiac troponin T (cTnT) in detecting myocardial damage in neonatal asphyxia and to compare the diagnostic value of cTnT with creatine kinase MB(CK-MB). Methods : Sixty-three neonates were enrolled in this study, consisting of 27 asphyxiated infants (asphyxiated group; 1-min or 5-min Apgar score≤6) and 36 healthy... |
Purpose : This study was performed to observe the utilization of ultrasonography in locating the position of UAC and to compare the position with anatomical landmarks seen on radiography. Methods : Optimal position is when the catheter tip is located between T6-T10 or L3-L5 by anteroposterior radiography(AP-R) and above the diaphragm by cross-table lateral radiography(CTL-R). Ultrasonographic studies used a Hewlett Packard... |
Purpose : Long-term steroid therapy for various glomerular disorders in children has been known to decrease serum Vit D3 level and develop osteomalacia. The aim of this study was to observe the effect of long-term steroid therapy on growth and bone density in children with nephrotic syndrome. Methods : Bone density of 17 steroid-treated nephrotic syndrome was compared with that of... |
Purpose : Alteration in the serum level of thyroid hormone can occur following open heart surgery due to major stress and hemodilution after extracorporeal circulation but these changes have been ignored. The purpose of this study is to evaluate the changes of thyroid hormone level after open heart surgery and if these changes influence the prognosis. Methods : We evaluated 26... |
Purpose : To evaluate the association between neonatal mortality and the initial response to surfactant replacement therapy, and to examine the correlation between the response type and other prognostic factors. Methods : Sixty-seven neonates with respiratory distress syndrome(RDS) were divided into two groups; group I(n=51) who survived and group II(n=16) who died within the first 28 days of life after one... |
Purpose : Urinary red blood cell morphology has been used to localize the site of hematuria in the urinary tract. But the pathologic mechanism for dysmorphic urinary red blood cells remains undefIned. Our study was undertaken to investigate the mechanism of dysmorphic urinary RBCs in glomerular hematuria. We have attempted to reproduce in vitro the changes that occur in red cell... |
Purpose : Adrenocorticotropic Hormone(ACTH) therapy is useful in the treatment of patients with infantile spasm. However, serious side effects including Cushing syndrome, infections, hypertension, and electrolyte imbalance must be encountered. Moreover, hypertrophic cardiomyopathy in association with ACTH therapy has recently reported. We performed thiw study to evaluate the effect of ACTH on heart muscle. Methods : We administered ACTH subcutaneously to... |
Bone mineral densitometry is a useful procedure for the early detection of metabolic bone diseases and bone density changes caused by long term drug use. Dual energy X-ray absorpt- iometry(DEXA), a recently developed diagnostic radiologic method for evaluating bone mineral density is widely used because of its high sensitivity, easily obtaine results. and low dose radi- ation exposure. The study... |
Purpose : Atherosclerotic change begins in childhood, elevated blood lipid levels and a family history of premature coronary vascular diseases are important risk factors for coronary artery disease. Identification, follow-up and treatment of children at high risk of cardiovascular diseases are very effective and important for prevention of future coronary artery diseases. The purpose of this study is to determine... |
Purpose : Recent development in ultrasonic Doppler instrumentation have allowed the direct noninvasive assessment of human renal artery blood flow. The authors conducted this study to assess noninvasive characterization of normal renal artery blood flow variables hopping to establish standards in normal children. Methods : A total 97 normal Subjects(below the age 3 years) were examinated using by 2-D and... |
The hyperlipoproteinemias are the disturbance of lipid transport resulted from accelerated synthesis or retarded degradation of lipoproteins that transport cholesterol and trigycerides through plasma. These diseases are classified as type I, type IIa, type IIb, type III, type IV, type V, and hyper-α-triglyceride levels due to increases in both VLDL and chylomicrons and lipoprotein electrophoresis shows increases pre-β, β, and... |
Schölein-Henoch purpura is a generalized small vessel vasculitis characterized by non-thrombocytopenic purpura, arthritis, abdominal pain and nephritis. In 1913 Osler described an allergic purpura associated with hemiplegia. After then Lewis et al. Reported the cases of Schölein-Henoch purpura associated with convulsion, coma, confusion, intracranial hemorrhage, and chorea, CNS complication has been reported in 1-8% of children and subsided spontaneously in... |
Miescher syndrome comprises congenital acanthosis nigricans, hypertrichosis, failure to thrive and short stature, dysmorphism especially of the jaws and oral cavity. Insulin-resistant diabetes mellitus, and a characteristic general appearance. This report concerns a rare case of 12-year-old girl having insulin resistant diabetic mellitus with Miescher syndrome. The relevant literature was reviewed. |
The long term administration of anti-convulsants on children with convulsive disorders may cause osteomalacia, depending on the dosage, duration, type and combination of anti-convulsants administred. Fifteen children, comprising 10 male and 5 female, dignosed with convulsive disorders and administered with phenobarbital for a long term period of more than 9 months but less than 84 months and 15 healthy children... |
To determine the types of metabolic acidosis using anion gap in acute infantile diarrhea and to correlate it with clinical outcome, we examined 103 infants admitted with acute diarrhea. The serum electrolytes (sodium, potassium, chloride, phosphorus), creatinine, CO2 content and anion gap were measured on first admission day. They were classified group A with normal anion gap (8~16 mEq/L) and... |
The clinical symptom complex characterized by syncopal attacks and sudden death in patients with electrocardiographic anomalies, especially a prolonged Q-t interval is known as the Romano-Ward syndrome. When a similar syptom complex is accompanied by congenital deafness, it is called Jervell and Lange-Nieisen syndrome. This study was underataken to investigate the incidence of the latter syndrome in the... |
Although febrile convulsion(FC) is a common clinical entity with a high recurrence rate, no specific factors have been identified to be highly predictive of its recurrence. A total to 114 cases of FC identified during a 28 month period (January, 1989-May, 1991) was analysed. A silght predominance of boys over girls(1.3 to 1.0) was observed. The majority of cases(89.4%) were under 3... |
Eight neonates with transient tricuspid insufficiency are presented which was confirmed clinical and two dimensional echocardiographic assessment. We found that two dimensional Doppler echocardiography was very useful in the detection of transient tricuspid insufficiency during neonatal age as noninvasive method. Transient tricuspid insufficiency is a clinical disorder in the newborn period caused by myocardial dysfunction, secondary to asphyxia with or without... |
Obesity has been implicated as a significant risk factor for multiple medical condition including hypertention, glucose intolerance, hyperlipidemia, hyperisulinemia and fatty liver. Furthermore, obesity in childhood is believed to be a reliable predictor of a similar condition in adulthood. During the recent yars, there has been a rapid rise in the incidence of childhood obesity in Korea due to increased... |
Neonatal sepsis is a major contributing factor for increased mortality and morbidity in the newborn and its successful outcome depends on early detection and prompt institution of an effective antimicrobial therapy. Accurate diagnosis of the neonatal septicemia, however, is quite difficult because its early signs and symptoms are rather subtle. This study was conducted in an attempt to search for useful... |
A transient heart murmur is frequently heard in normal newborn who has no congenital heart disease. The cause of this murmur remains unclear but was speculated as hypoplasia of the pulmonary arterial branches or unusual alignment of the main pulmonary artery with its branches. Pulsed Doppler and two-dimensional echocardiographic studies were undertaken in 19 newborn infants with a transient murmur and... |
The platelet coiunt has not been part of routine hematologic profiles until recently, when the newer electronic blood cell counters began to include platelet count with all other blood cell counts. With the widespread use of newer generation electronic blood cell counters, elevated platelets coiunts are being encountered more ofter in pediatric practice. We reviewed all cases of marked thrombocytosis to... |
Generally, infants of diabetic mother(IDM) have various diseases and complications affecting multisystems resulting in high perinatal morbidity and mortality. It is well known that IDM have especially higher incidence of congenital anomalies including congenital heart disease(CH D). dTransposition of great arteries(d-TGA), ventricular septal defect and hypertrophic cardiomyopathy(HCM) form most of CHD occurring in IDM. Of these, HCM appears as a... |
Prostaglandin E1 (PGE1) was administered to 12 neonates with cyanotic congenital heart disease in whom pulmonary or systemic blood flow were entirely or significantly dependent on persistent patency of the ductus arteriosus. 10 neonates responded favorably to PGE1, by showing significant increase of PaO2 after PGE1 infusion but remaining 2 neonates with hypoplastic left heart syndrome failed to respond. The... |
Pulsed Doppler echocardiography was performed to evaluate the flow velocity pattern of the pulmonary vein in 28 patients with congenital heart diseases which comprised of 8 atrial septal defect (ASD), 14 ventricular septal defect (VSD) and 6 patent ductus arteriosus (PDA). 20 normal children served as controls. The objective of this study was to ascertain that pulmonary venous flow velocity is dependent on the... |
Supraventricular tachycardia is the most symptomatic dysrhythmia encountered in infants and children. Recognition of the dysrhythmias is of great clinical importance, since increasing number of infants will develop congestive heart failure and occasionally circulatory collapse. Retrospective study was done in 17 patients with supraventricular tachycardia, who were admitted to our hospital from 1983 to 1989. The results were as follows: 1) 47 percent of our patients with... |
The short-term effects of increasing hemoglobin concentration were evaluated on cardiac catheter- ization in nine patients with congenital heart diseases and relative anemia. Diagnosis were ventricular septal defect in six, tetralogy of Fallot in two, and endocardial cushion defect in one. Various hemodynamic data including pulmonary and systemic blood flow, pulmonary and systemic vascular resistance and the degree of shunt were compared before and... |