Congenital syphilis is one of the most well-known congenital infections, yet it remains a worldwide public health problem. Congenital syphilis can involve any organ system and present with various symptoms. However, early diagnosis of congenital syphilis is difficult because more than half of the affected infants are asymptomatic, and the signs in symptomatic infants may be subtle and nonspecific. Here,... |
Purpose : Kawasaki disease (KD) is a systemic vasculitis, a leading cause of pediatric acquired heart disease. Histopathological findings of coronary artery lesion (CAL) in KD indicate destruction of the coronary artery wall with diffuse vasculitis. Matrix metalloproteinases (MMPs) and their endogenous tissue inhibitors (TIMPs) might play central roles in this process. Special attention to MMP-9 has recently been emerging.... |
Purpose : The objective of this study is to determine the change of C-reactive protein (CRP) levels in the peripheral blood of preterm infants at birth according to the stage of intrauterine inflammation. Methods : A total of 187 infants (<32 weeks of gestation) were divided into a “no histologic chorioamnionitis” [HCAM (-), n=85] group and a “histologic chorioamnionitis” [HCAM... |
Herpes zoster in infancy is very rare but can be developed following intrauterine or postnatal exposure to varicella zoster virus. We report a case of herpes zoster in a 4-month-old male infant. He had no prior history of primary varicella or varicella vaccination. His mother had no history of varicella infection and no contact history with varicella during pregnancy. He... |
Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in... |
Primitive neuroectodermal tumors(PNETs) are a group of small round cell tumors that may arise in the central or peripheral nervous system. In the extracranial location, these neoplasms may occur anywhere and at any age group, but most likely in the bone and soft tissue of children and young adults. The incidence of adrenal gland involvement is very rare. This tumor... |
Purpose : Apoptosis is a process of active cell death which has been suggested to be part of hippocampal cell loss caused by kainic acid(KA). Immature rats showed higher susceptibility and mortality to KA but did not develop recurrent seizure, long term behavioral or neuropathologic changes. We investigated whether this was due to age-dependent resistance, and elucidated the molecular mechanics which mediate P53-induced apoptosis,... |
Purpose : c-fos is rapidly and transiently induced in the intact CNS by a wide variety of exogenous stimuli that include seizures, glutamate receptor activation, sensory stimulation and stress. In adult animals, systemic KA administration produces limbic seizures that results in c-fos protein expression, irreversible morphological changes and localized neuronal death. So we studied the pattern of c-fos protein expression... |
Purpose : The nephrotic syndrome is characterized by proteinuria, hypoproteinemia, edema and hyperlipidemia. These can change body homeostasis and cause hypertension. This study was designed to determine the relationship between the forearm vasodilating capacity and serum cholesterol level of nephrotic syndrome patients. Methods : 15 Nephrotic syndrome patients and 17 normal control children who visited Chung-ang University Youngsan Hospital from Sep.... |
The idiopathic long QT syndrome is a very rare disorder in which affected individuals have an unusual electrocardiographic repolarization abnormality (QT prolongation) and a propensity to syncope and fatal ventricular arrhythmia. Since Jervell and Lange-Nielsen first described this syndrome in 1957, progression has been made in the pathophysiology and in the treatment. Sinus bradycardia may be associated with this syndrome sometimes,... |
Recently, NAG activity has gained increasing importance as an aid in the diagnosis of renoparenchymal diseases. Elevation of urine NAG activity has been found to be an indicator of renoparenchymal diseases. To evaluate the diagnostic value of the NAG activity test in the renal disease, we carried out clinical study on 31 cases of renal disease patients who had been... |
Malignant atrophic papulosis (Degos’ disease) is characterized by porcelain-white skin lesions. It has unknown etiology and bad prognosis. Death usually occurs from multiple intestinal perforations weeks to years after onset. We experienced a case of Degos’ disease in a 12-year-old male patient who had porcelain white papules on face, trunk and extremities. Histopathologic studies showed broad epidermal necrobiosis with thrombotic arteriolar occlusion. A brief review of... |
Echocardigraphy was done on the first and the second day of life in 23 normal full term newborns to evaluate the sequential change of cardiac function of the normal neonates. The results were as follows. 1) On the first day of life, all of the measurements of diastolic filling except peak A velocity were significantly different when the right and left ventricle were compared. For... |
Congenital hypofibrinogenemia is a rare congenital bleeding disorder, inherited by autosomal dominant or recessive trait. We experienced one case of congenital hypofibrinogenemia in 4 year-old female patient with chief complaints of headache and ecchymosis. History of continuous umbilical cord bleeding and frequent bleeding tendency against minor trauma were revealed on her past medical history. On laboratory data, bleeding time, platelet aggregation test were normal and... |
Asymptomatic human rotavirus (HRV) infection rate was studied by comparing the rate of HRV excretion in diarrhea cases with that in nondiarhea group of children under preschool age. HRV antigen in stool was detected by enzyme-linked immunosorbent assay (ELISA) from children under 6 years of age who were admitted to Pediatric Department of Chung-Ang University Medical Center from November 1988 to mid-April 1989. 1) HRV antigen... |
Congenital pulmonary lymphangiectasis is a rare abnormality usually becoming manifest at birth and almost invariably fatal in neonatal period or early infancy. Chylothorax is an infrequent manifestation of this disease. We report a patient with pulmonary lymphangiectasis, 3 year old male, who had been suffered from persistent respiratory distress since 7 days after birth, in whom the diagnosis was suggested by the appearance of... |