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Hallermann-Streiff syndrome is a rare disease; approximately 150 cases have been reported in the world literature. The syndrome consists of proportionate nanism; hypotrichosis; atrophy and extreme thinness of skin, particulary over the facial area; an usual "bird-like" face with mandibular hypoplasia; a prominent thin, pointed nose; congenital cataracts; and severe dental abnormalities. We report a case of Hallermann-Streiff syndrome in... |
Purpose : Pneumoccocus is one of the most important causes of invasive infection through the childhood period and the prevelance of antibiotics resistance of pneumococcus is increasing worldwide. A 7-valent conjugate vaccine has been developed. It is important to know the prevalence of each serotype of pneumococci in the countries where the vaccine is used to estimate the coverage rate... |
Purpose : It is known that single umbilical artery is frequently associated with gastrointestinal or urogenital anomaly, however, routine renal sonography has been debated in healthy neonate with isolated single umbilical artery. This study is designed to determine the usefulness of routine renal sonography in apparently healthy infants with an isolated single umbilical artery. Methods : Thirty healthy neonates with... |
Purpose : There was a outbreak of aseptic meningitis in Busan, 2002. We report the clinical features and causative viruses. Methods : Two hundred seventy six children with aseptic meningitis who were admitted to the Department of Pediatrics, Maryknoll Hospital between January and December, 2002 were included. CSF, stool and throat swab viral cultures were done in 244 of these... |
Purpose : We measured the umbilical cord arterial concentrations of isoprostane(8-iso-PGF2α) and intended to decide whether the umbilical cord arterial concentrations of isoprostane could be used as a useful parameter for lipid peroxidation in newborn infants. Methods : The isoprostane and malondialdehyde(MDA) concentrations of the umbilical cord were measured by enzyme immunoassay and TBARS(thiobarbituric acid reactive substance) assay in 33... |
Purpose : This study was performed to follow the natural course and size change of isolated atrial septal defect(ASD) secundum. Methods : Among the newly diagnosed with ASD secundum at the division of pediatric cardiology in Yonsei cardiovascular hospital from January 1996 to December 2000, 89 patients with pure ASD secundum were checked by the serial echocardiographic evaluation to measure... |
Purpose : To find out the myocardial protective effect of cardioxane for the myocardial damage by doxorubicin. Methods : Using Eighteen rabbits(2.0-3.2 kg), doxorubicin(30 mg/m2) was injected intravenously once a week in group I(12 rabbits) and cardioxane(600 mg/m2) was injected at 20-30 minutes before doxorubicin administration in group II(6 rabbits). After this, we operated on the rabbits when the total cumulative... |
Purpose : The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. Methods : Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance... |
Purpose : This study aimed to evaluate risk factors of the first year relapse in children with nephrotic syndrome(NS) without the need for biopsy. Methods : We reviewed, retrospectively, 78 children diagnosed with steroid responsive nephrotic syndrome between July 1997 and June 2002. Median years to follow up were 4.4 years(range : 1-5 years). We divided the patients into two groups(group... |
Purpose : Benign childhood epilepsy with centrotemporal spikes(BECT) is characterized by an excellent prognosis. Therefore, the necessity for the use of antiepileptic drugs is controversial. The object of this study is to know about the long-term follow-up of BECT, comparing daily treatment with antiepileptic drug(AED) versus no medication. Methods : We retrospectively studied 56 cases of BECTs, examined at Pediatric Neurology... |
Purpose : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated... |
Purpose : Kawasaki disease is notorious for coronary arterial complication which is usually developed as a febrile disease in early childhood. Increased polymorphonucleus(PMN) cell levels in acute phases may be associated with the pathophysiology of Kawasaki disease. We studied the relationship between coronary arterial dilatation and elastase activity which was excreted from PMN cell and roles as an important factor... |
Klippel-Trenaunay syndrome is a rare mesodermal phakomatosis characterized by cutaneous haemangiomata(usually unilateral and involving an extremity), venous varicosities and osseous and soft tissue hypertrophy, of the affected limb. Sturge-Weber syndrome, also a mesodermal pharkomatosis, is characterized by a port-wine nevus, which is present from birth and covers the face and cranium in the territory of the first division of the... |
Cardiac rhabdomyomas are relatively uncommon and associated with tuberous sclerosis in 40-50% cases. We report a 10-month-old infant with tuberous sclerosis who presented with ventricular arrythmias and status epilepticus. There were hypopigmented macules on the body, periventricular calcifications, renal cyst and cardiac rabdomyomas just below the aortic valve. The patient required resection of left ventricular subaortic masses due to sustained... |
Herpes simplex virus infection of the hand in children occurs after auto-inoculation from herpetic gingivostomatitis or herpes labialis. Herpetic whitlow should be suspected based on clinical signs. Diagnosis can be made by PCR or virus culture. Many misdiagnosed cases suggests that this disease is not sufficiently known. Surgical interventions may be harmful and should be avoided. We report a case... |
Intestinal lymphangiectasia, one of the protein-losing gastroenteropathies, is an uncommon disease characterized by dilated intestinal lymphatics, enteric protein loss, edema, hypoalbuminemia, and lympocytopenia. Small bowel biopsy and CT have been used to confirm the diagnosis of intestinal lymphangiectasia. Small bowel biopsy shows collections of abnormal dilated lacteals in submucosa with distortion of villi and CT findings have been described as... |
Hydrocarbon pneumonitis is an inflammatory change in the lungs caused by ingestion or inhalation of household products that contain hydrocarbons. We experienced a rare case of hydrocarbon pneumonitis with a pulmonary hemorrhage in a 3-year-old girl after ingestion of paint thinner which has a high mortality. She was admitted due to dyspnea, vomiting, hemoptysis, and needed to support mechanical ventilation.... |
Canavan disease, also known as van Bogaert-Bertrand disease, is a rare autosomal recessive disorder characterized by early an onset and a progressive spongyform degeneration of the brain, associated with an edema of the central nerve system, intramyelinic swelling and neurologic symptoms. This disorder is most prevalent in people of Ashkenazi Jewish descent but has been observed in other ethnic groups.... |
Neonatal herpes simplex virus(HSV) infections result in significant morbidity and mortality. Although acyclovir treatment has improved survival, severe neurological sequelae can occur in the majority of survivors. HSV infections limited to the skin, eyes and mouth(SEM) can cause neurologic impairment. A direct correlation exists between the development of neurologic deficits and the frequency of cutaneous HSV recurrences. National Institutes of... |