Purpose : This study was designed to evaluate the effect of growth hormones on children with growth hormone deficiency(GHD) or idiopathic short stature(ISS). Methods : Between January 1988 to July 2003, 45 patients(M26, F19) with GHD and 24 patients (M13, F11) with ISS were enrolled in this study. Height standard deviation score(Ht SDS) for chronological age(CA) and Ht SDS for bone... |
Purpose : Williams syndrome(WS) is an autosomal dominant disorder resulting from a submicroscopic deletion of contiguous genes on the long arm of chromosome 7. We evaluated the physical, neurodevelopmental, and behavioral characteristics of children and adults with WS confirmed by fluorescence in situ hybridization. Methods : The clinically suspicious twelve cases with cardiac anomaly were confirmed as WS with fluorescence in... |
Purpose : Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. Methods : This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations... |
Purpose : Myocardial scintigraphy has been reported to be useful in adults, but its usefulness in children is limited. This study was done to determine the feasibility and accuracy of 99mTc-MIBI SPECT(sestamibi, methoxy-isobutyl-isonitrarite myocardial single emission computed tomography, Dp-SPECT) after dipyridamole infusion to detect coronary obstructive lesions in Kawasaki disease (KD). Methods : Dp-SPECT was performed in 21 control(group 1), 8... |
Purpose : Williams syndrome is characterized by supravalvular aortic stenosis, mental retardation and peculiar facial appearance. Its genetic etiology is considered to be a hemizygotic deletion in Chromosome 7q11.23, which includes the elastin gene. We examined the hemizygotic deletion of Chromosome 7q11.23 in 12 Korean Williams syndrome patients and 8 patients with isolated supravalvular aortic stenosis and performed deletion mapping... |
Purpose : Syncope is not rare and is caused by various conditions ranging from common physiologic derangements to life-threatening conditions. However, there are limited reports regarding syncope in children. We retrospectively analyzed our experiences of the syncope in children. Methods : Retrospective analysis of the medical records of patients with syncopal episodes between October 1985 and June 1996. Results : Sixty-eight patients(male; 36, female; 32,... |
Purpose : There are controversies in the operative methods of aorta with ventricular septal defect in infancy. Recent results of single stage operation for coarctation of aorta with intracardiac defects were regarded as comparable to staged operation. We evaluated our interim results after single stage operation for coarctation of aorta with large ventricular septal defect in infancy. Methods : Twenty six infants who had received... |
Two families of dilated cardiomyopathy are reported. In the first family, two sons were affected. The elder child presented with congestive symptoms at the age of 5 months and was diagnosed to have dilated cardiomyopathy. The etiology of cardiomyopathy was thought to be idiopathic until his younger brother presented with near collapse at the age of 5 days and was diagnosed to have the... |
Congenital coronary artery fistulas are rare congenital heart anomalies. Surgical closure of these fistulas was the therapy of choice till recently. The recent development of a new accurate coil-delivery system has enabled us to embolize the vessels. Percutaneous transcatheter coil embolization is a safe and effective approach to treating coronary artery fistulas and should be considered as the best treatment... |
Purpose : Hypertrophic cardiomyopathy(HCMP) is a primary cardiac muscular disease with high risk of sudden cardiac death. This study is performed to understand the clinical features of children with HCMP. Methods : Retrospective analysis of the medical records of the patients diagnosed at Seoul National University Children's Hospital between October 1985 and June 1996 was done. Excluded the patients who had... |
Purpose : Branch pulmonary artery stenosis remains a clinically challenging lesion. Results of surgical angioplasty are rarely rewarding, and balloon angioplasty is not uniformly successful. So, endovascular stent has been applied recently to these lesions. But this new modality has not been full yevaluated. The aim of this study is to evaluate the short-term result of stent implantation in postoperative branch pulmonary artery stenosis. Methods :... |
Pulmonary artery sling is a rare vascular anomaly wherein the left pulmonary artery arises from the right pulmonary artery and then traverses between the esophagus and the trachea toward the hilum of the left lung. Associated anomalies are common, particularly those of the tracheobronchial tree, which have been found in more than 50% of patients. Cardiovascular anomalies such as persistent... |
The recognition that deletion of 22ql1 is a common cause of varied malformations and clinical disorders took more than a decade to achieve. Deletions of chromosome 22ql1 have been seen in association with DiGeorge syndrome (DGS) and velo¡ⓒ cardio-facial syndrome (VCFS). The large clinical overlap between DiGeorge syndrome and velo-cardio-facial syndrome suggest an aetiological connection. CATCH 22 synd¡ⓒrome is characterized... |
Purpose : We performed this study in order to evaluate the outcome of the mechanical valve replacement in the right side of the heart in children. Methods : Retrospective analysis of the clinical records of 26 patients who had undergone a right sided valve replacement between October 1986 and September 1992 at Seoul National University Children뭩 Hospital. Results : Twenty six children... |
Purpose : In concern of diagnosis and treatment of transient idiopathic hypocalcemia, a major cause of infantile, we studied the onset of age, clinical characteristics, laboratory findings, and duration of treatment and recovery of transient idiopathic hypocalcemia. Methods : The author observed 19 infants with transient idiopathic hypocalcemia who had been admitted to Yonsei university hospital due to convulsion for 4... |
Purpose : Sex hormone binding globulin(SHBG) is produced in liver and binds to testostero-ne and estradiol. Plasma SHBG levels are increased by estrogen and thyrotoxicosis and decreased by testostrone, insulin, growth hormone, prolactin, obesity and noninsulin dependent diabetes mellitus. SHBG levels in patients with NIDDM is known to be higher than normal, but SHBG levels in children with IDDM is controversial.... |
Purpose : The DCRV is a cardiac anomaly in which the right ventricle is divided into two dif-ferent pressure chambers by aberrant hypertrophied muscle bundles arising from the lower infundibular septal region. DCRV may be associated with other congenital cardiac anomalies and surgical treatment is required because we know that this obstruction is progressive. In this report, we reviewed clinically... |
Purpose : The relative incidence of fetal cardiac anomalies and associated extracardiac anomalies in Korean fetuses were studied. Methods : Findings in fetal echocardiography and autopsy were reviewed using 129 fetuses with cardiac anomalis detected at a gestational period under 38 weeks from 1991 Jan. to 1994 June. Results : The most common fetal cardiac anomaly was isolated atrial septal defect(28 cases... |
Purpose : To evaluate the appropriateness of the transcatheter occlusion with Rashkind um-brella occlusion device Methods : Among the 54 cases who had been tried transcatheter closure of patent ductus ar teriosus from January, 1992 till December, 1993 at Seoul National Univerity Children's Hospital, the first follow-up evaluation with Doppler echocardioram were done in 43 cases at the mean i-nterval of... |
We report four cases of unilateral pulmonary vein atresia without associated congenital intracardiac anomalies to illustrate a part of the clinical and radiological characteristics and its diagnosis. Pulmonary vein atresia was in right side in all cases. Narrowing of contralateral pulmonary vein and pure red cell anemia were combined in one cases. In four cases, initial presentations were hemoptysis and... |
Hyperglycemia is a well-recognized side effect of L-asparaginase in remission induction therapy of acute lymphocytic leukemia. Since hyperglycemia has preceded fatal diabetic ketoacidosis or hyperosmotic nonketotic coma in some patients, early detection and treatment of this complication are important. We determined retrospectively the risk of hyperglycemia in 117 patients with leukemia who had received L-asparaginase (& prednisolone). The results were as... |
We experienced 3 cases of Candida esophagitis in infancy which were diagnosed by esophageal endoscopy, First case, 10 month-old boy with combined immune deficiency had suffered from oral thrush and poor feeding for more than 4 months. Esophageal endoscopy revealed multiple whitish creamy patches on the friable erythematous and necrotic mucosa of the esophagus, He was firstly treated with amphotericin-B... |
We reviewed 26 cases of Cardiac rhabdomyoma in children which were diagnosed between 1986 and 1991 in Seoul National University Children's Hospital. Twenty-two cases were associated with tuberous sclerosis. Total tumor count was 58 and mean tumor count per patient was 2.23. Their Cardiac rhabdomyoma were diagnosed from fetal period through 12 years after birth. Median age was 9.5 months.... |
A case of recurrent rhinocerebral mucormycosis that has occurred during an induction chemotherapy for acute megakaryocytic leukemia in a 10 year-old boy is reported. He had suffered from high fever, proptosis, right eye ball pain and necrotic inflammation of hard palate during the chemotherapy of leukemia. CT scan of the paranasal sinus showed inflammatory change of right ethmoid and maxillary... |