| The auther analysed 29 patients who were treated with prednisolone, including nephrotic syndrome(13 cases). Acute leukemia (10 cases), bronchial asthma(2 cases), juvenile rheumatoid arthritis(2 cases) and ulcerative colitis(1 cases) patients in childhood, after remission induction, prolonged alternate or interrupted treatment was used. The auther observed patterns of height velocity of these patients from 1.6 to 10.3 years. The results were as... |
| We analyzed the clinical and laboratory features of ten children with acute megakaryoblastic leukemia (M7) and compared the findings with those reported in the literature. The diagnosis was supported by ultrastructural examination of platelet peroxidase or immunophenotyping for glycoprotein Iib/IIIa. Of the ten children, five were girls and five were boys. The median age at diagnosis was 13 months. Two... |
| Early infantile epileptic encephalopathy is characterized by its early onset (less than 3 months of age), frequent tonic spasms, suppression-burst on EEG, extremely poor response to treatment and grave outcome. The authors experienced a case of early infantile epileptic encephalopathy. The patient was a 1-month-old girl with a complaint of generalized tonic convulsion since 2 days of age, and her EEG... |
| Congenital Pelger-Huet anomaly is an autosomal dominant disorder characterized by incomplete nuclear segmentation of neutrophils without functional disturbance. The morphology of these mature but hyposegmented neutrophils may be confused with that of immature neutrophils (bands), thereby suggesting bacterial infection. We experienced a case of congenital Pelger-Huët anomaly on routine hematologic examination in a 7-year old boy who was admitted because of... |
| Wilson disease is an autosomal recessive abnormality in the hepatic excretion of copper that results in toxic accumulation of the metal in liver, brain, and other organs. Hemolytic anemia frequently complicates the courses of Wilson disease and may be the initial clinical feature of Wilson disease. Since hemolysis may be preceding the onset of hepatic manifestation and Kayser-Fleischer ring is... |
| This study reviewed 66 episodes of septicemia occurring in 56 children with neoplastic disease at Seoul National University Children뭩 hospital over 31 month period. The most frequent organisms causing 45 episodes in neutropenic patient were Escherichia coli (28%), Klebsiella species (18%), Pseudomonas species (9%), polymicrobic (9%,), and Staphylococcus aureus (7%). The overal mortality was 13% (5/39). The most frequent organisms... |
| Gastrointestinal symptoms of Henoch-Schönlein commonly present abdominal pain, nausea, nomitting, melena and hematemesis and resolve mostly without any complications. Some times life threatening gastrointestinal manifestations such as massive hemorrhage, intussusception, intestinal necrosis and perforation may develop We report a case of the Henoch-Schönlein purpura with intestinal lesion in 11 year female patient who presented with colicky abdominal pain and bloody stool... |
| Cystic hygroma is a true, benign, congenital multi-cystic tumor. It is thought to be arised from failure of the lymphatic system to communicate with the venous system. Frequently it occurs at birth, and it is found most commonly in the neck as painless and fluctuant mass. For many years, surgical excision has been considered as the treatment of choice. However, excision... |
| The authors evaluated 19 childrenwith chronic renal failure patients diagnosed and followed up at the Department of Pediatrics, Seoul National University Hospital for the detection of acquired renal cystic disease (ARCD) with two radiologists by the means of ultrasonography. The results were as follows: 1) Of the 19 patients, male to femals ratio was 14:5 and of them, hemodialysis and peritoneal dialysis... |
| Acute disseminated encephalomyelitis(ADEM) is an acute inflammatory demyelinating disease of the central nervous system. We experienced a case of ADEM in an 8 year-old boy who showed headache, vomiting, weakness of lower extremities and visual disturbance. Brain magnetic resonance imaging demonstrated multiple patch high signal intensity in both optic nerves, both thalamus, splenium of corpus callosum, and subcortical white matter... |
| Anomalous origin of the pulmonary artery from the ascending aorta is a rare congenital cardiovascular anomaly which usually involves the right pulmonary artery. Because congestive heart failure and the pulmonary vascular obstructive changes develop early in life, early diagnosis and surgical correction are essential. We experienced two cases of anomalous origin of the right pulmonry artery from the ascending aorta; aorticopulmonary septal... |
| Pseudohypoaldosteronism(PHA) is rare salt losing disease which is characterized by mineralcorticoid unresponsiveness of the end organ. Severe hyponatremia and hyperkalemia are present despite high plasma aldosterone. We experienced a case of PHA in a 40-days old male infant who was presented with anorexia, vomiting and lethargy for several days. Labortory data showed hyponatremia, hyperkalemia and metabolic acidosis. Renal function was normal... |
| Exercise and allergen challenges are the main tools to investigate the bronchial responsiveness in experimentally provoked asthma. It is recognized that LAR, which often follows early asthmatic response(EAR) by 3 to 10 hours after challenge, is more closely related to the basic pathogenetic mechanisms operating in bronchial asthma. Although exercise induces an EAR within the first hour similar to allergen... |
| We reviewed the medical records of 14 children (8 girls, 6 boys), diagnosed as hemolytic uremic syndrome at Seoul National University Children뭩 Hospital from 1981 to 1990. The age at presentation ranged from 1 month to 10 years, with a mean age of 2.7 years. Only eight (57.1%) of the children had diarrheal prodrome and five (35.7%) had grossly bloody... |
| The syndrome of congenital hypopitutarism is a rare but well recognized entity. The typical clinical picture consists of presentation in infanncy with hypoglycemia, microgenitalia, and evidence of multiple anterior pituitary hormone deficiencies, but not diabetes inspidus. Recently, magnetic resonance imaging has disclosed a consistent abnormality in many patients with congenital hypopituitarism as well as idiopathic hypopituitarism characterized by an adenohypophysis... |
| A statistical study was performed on 221 ICU cared pediatric patients who were admitted to the department of pediatrics, Yonsei university college of medicine, Seoul, Korea from Apr. 1983 to Mar. 1991. The results were as follows : 1) Total number of subject were 221, it was 7.5% of total ICU patients and 1.3% fo total admitted pediatric patients. The male to... |
| Antimicrobial susceptibility for 25 stranis of methicillin senitive Staphylococcus (MSSA) and 25 strains of methicillin resistant Staphylococcus aureus (MRSA), isolated from in-patients at Kosin Medical Center from January, 1989 to July, 1990, were tested by the tube dilution method. 1) For MSSA, sensitivity tests showed 21% was sensitive to cefadroxil. Seventy one percent was intermediate and 8% was resistant. 2) 96% of... |
| The congenital hepatic fibrosis is a rare condition defined pathologically by the presence within the line of bands of fibrous tissue which after contain linear or circular spaces lined by bile duct cells, It is commonly associated with intrahepatic portal hypertension but hepatocellular function is almost always preserved. The prinicipal clinical features of this disease are abdominal distension, firm hepatomegaly,... |
| 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... |
| Congenital dyserythropoietic anemia(CDA) is a rare disease characterized by ineffective erythropoiesis, specific cytopathology of the nucleated red cells in the bone marrow and secondary hemochromatosis. CDA is classified into 4 types on the basis of results of electron microscopic and serologic studies. We experienced a case of congenital dyserythropoietic anemia in a 5/12 year-old boy. Diagnosis was established by macrocytosis, anisopoikilocytosis in PB... |
| We had treated a hepatic rickets in 6 infants with neonatal hepatitis from June 1989 to August 1990 at Asan Medical Center. The results were as follows 1) Age of 6 patients were below 4 months and 4 patients were male, 2 patients were female. 2) The clinical findings were hepatosplenomegaly(5 cases), jaundice(4 cases), craniotabes (4 cases), failure to thrive(2 cases), hypocalcemiac seizure(2... |
| The parmacokinetics and biological effects of an recombinant methionyl growth hormone(LBD-003) produced by the expression of genomic hGH in a yeast cell line, were studied in growth hormone deficient children. Twelve patients were randomly assigned to two age-matched subcutaneous and intramuscular groups. Each group consists of 6 patients. Each subject received an dosage of 0.1 U hGH(LBD-003)/kg, BW, Blood was drawn before... |
| Despite the scientific advances made in medical science, the potential for serious hemorrhagic complications remain an important problem in patients undergoing open heart surgery with extracorporeal circultation (OHSEC). Hematologic abnormalities that have been reported in association with OHSEC and that may lead to a hemorrhagic tendency include thrombocytopenia, alteration of coagulation factors, advanced fibrinolysis and inadequate neutralization of heparin. We investigated... |
| The clinical picture of beingn recurrent intrahepatic cholestasis (BRIC) consists of multiple epi- sodes of cholestatic jaundice without extrahepatic bile duct obstruction. Since first description by Summerskill and Walshe in 1959, almost 100 patients with BRIC have been described. The mechanism involved in the initiation and perpetution of an episode of cholestasis are unknown. Altered bile acid metabolism has been proposed to play a... |
| We experienced a case of acute megakaryoblastic leukemia (M7) with Down syndrome. The patient was admitted due to premature SGA, which revealed characteristic facial figure of Down syndrome. M7 was diagnosed with PB smear which showed abundant megakaryoblast and confirmed by using the monoclonal antiplatelet glycoprotein IIb/IIIa antibody (J 15). On 25th hospital day, the patient gained weight but discharged without further treatment of M7. Authors... |
| Septo-optic dysplasia, which was first described by de Morsier in 1956, is a rare developmental anomaly of anterior midline structures of brain, combining agenesis of the septum pellucidum and hypoplasia of the optic nerves, chiasm, and infundibulum. It is considered a form of holoprosence- phaly. It is a common cause of hypopituitarism in children, and the pituitary hormone most commonly affected is growth hormone, but... |
| Watson-Alagille syndrome is a kind of familial intrahepatic biliary atresia associated with charac- teristic face and cardiovascular anomaly. Growth and developmental delay, anomalies of the eyes such as posterior embryotoxon, deformities of the skeletal system are often the manifestations of this syndrome. We experienced three cases of Watson-Alagille syndrome recently. These patients showed charac- teristic faces and had peripheral pulmonary artery stenosis diagnosed by pulmonary... |
| This study was conducted to investigate the effects of CNS prophylaxis with 1800 cGy cranial irradiation and intrathecal methotrexate (MTX) chemotherapy on intellectual function of the chil- dren with acute lymphocytic leukemia (ALL). Thirteen longterm survivors with ALL who were treated with 1800 cGy cranial irradiation and intrathecal MTX for CNS prophylaxis at pediatric department of Yeungnam University Hospital from May, 1983 to November, 1988... |
| Congenital cystic diseases of the lung (CCDL) is a clinical group of disorders characterized by a cystic appearance of pulmonary tissue. It can be classified into four distinct categories: 1) Congenital lobar emphysema (CLE), 2) Congenital cystic adenomatoid malformation (CCAM), 3) Pulmonary sequeestration (PS), and ^Bronchogenic cyst (BC). 24 patients with CCDL admitted to the Seoul National University Children’s Hospital from January 1984 to June... |
| In patients with certain types of congenital heart malformations, an adequate interatrial communi- cation is essential for survival and balloon atrial septostomy (BAS) is a life saving procedure in these infants. However, BAS alone is not successful in older infants and children with thickened atrial septum, and blade septostomy is indicated in these situations. This procedure so far has not been reported in Korean literature... |
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