| Purpose : Granulocyte-colony stimulating factor(G-CSF) and granulocyte macrophage-colony stimulating factor(GM-CSF) are principal cytokines in granulopoiesis and their physiologic effects are mediated through binding to specific cell surface receptors. Although it is known that the level of serum G-CSF and GM-CSF, and presentation of the receptors are increased in infectious diseases, there have been no studies to find the correlation between... |
| Purpose : Brain tumors are the second most common tumor in childhood, and medulloblastomas comprise 15-25% of brain tumors. The well known prognostic factors are age at diagnosis, stage of disease, and extent of surgical excision. In this study, we analysed the prognostic factors in patients who received chemotherapy after excision. Methods : We reviewed the medical records of 61... |
| Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal... |
| Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes... |
| Department of Pediatrics†, College of Medicine, Korea University, Seoul, Korea Interstitial pneumonia is a heterogenous group of inflammatory and fibrosing lesions that manifest themselves as infiltrative lung disease. Of these, nonspecific interstitial pneumonia is characterized as a variable degree of interstitial inflammation with or without fibrosis and is distinguished from usual interstitial pneumonia and desquamative interstitial pneumonia, histologically. The influx of... |
| Purpose : Bacillus Calmette-Gu rin(BCG) lymphadenitis is one of the most common complications of BCG vaccination. The involved lymph nodes usually subside spontaneously, but they may become enlarged and form an abscess. Treatment of these infants is controversial. The Pan American Health Organization recommends local isoniazid or rifampicin instillation for patients with suppurative BCG lymphadenitis. Methods : The study group comprised... |
| Purpose : The aim of this study is to determine and compare the effects of adjunctive therapy with different doses of recombinant human granulocyte-colony stimulating factor(rhG-CSF) on reversing sepsis-associated neonatal neutropenia, and their survival rate in a group I/II-type trial. Methods : RhG-CSF was injected subcutaneously to 10 septic-neutropenic neonates with doses of 10 μg/kg from Oct. 1995 to Sep. 1996,... |
| Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods :... |
| Syndrome of 4q deletion is characterized by an abnormal shape of the skull, craniofacial dysmorphism, cardiovascular malformations, genitourinary defects, limb and digital anomalies, and developmental delay. We experienced a case of 4q interstitial deletion in a 2 day-old female neonate who showed short extremities, partial agenesis of corpus callosum and congenital heart defects. We report the case with a brief... |
| Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe... |
| Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with... |
| Henoch-Schöenlein purpura, also known as anaphylactoid purpura, is characterized by palpable purpura, colicky abdominal pain, gastrointestinal hemorrhage, arthralgias, and renal involvement. Histopathologically, the condition represents a vasculitis, and in fact, it may be the most common vasculitis syndrome affecting children. The pathogenesis of Henoch-Schöenlein purpura remains poorly understood, but it is postulated that an unknown antigenic stimulus causes elevation of circulating IgA and that complement... |
| Purpose : Interstitial lung disease(ILD) is a rare and poorly characterized disorder in children with poor prognosis. To understand the ILD in children, we reviewed our experience with 21 patients who were diagnosed interstitial lung disease during 9-year period at Asan Medical Center retrospectively. Methods : Severity-of-illness score was measured by the Denver protocol. We evaluated underlying diseases, clinical manifestations, high resolution computed tomography findings,... |
| Purpose : The acute scrotum is a clinical syndrome that is defined as an acute, painful swelling of the scrotum or its contents. The symptoms and signs of acute scrotum are similar and its pathognomonic features are rarely detected. This study was performed to find a proper way to diagnose and treat acute scrotum promptly and correctly. Methods : From January 1990 through December... |
| Lymphangiomatosis, a benign tumor consisting of a cluster of dilated lymphatic channels, is very unusual. Most lymphangiomatoses are found in the neck and head area. Less than 5% are diagnosed intraabdominally and they are very infrequently encountered in the retroperitoneal area. Herein, we report a rare case of asymptomatic lymphangiomatosis of a 13 year-old boy during a routine chest... |
| Wernicke,s encephalopathy(WE), a neurological disorder caused by thiamine deficiency, is characterized by the triad of ocular symptoms, ataxia, and mental confusion. More than 90% of the cases are observed in chronic alcoholics. Other conditions less frequently associated with WE are anorexia nervosa, prolonged parenteral nutrition, hemodialysis, uremia, hyperemesis gravidarum, gastroplasty for morbid obesity, and acquired immunodeficiency syndrome(AIDS). One of the... |
| The interstitial lung diseases(ILD) comprises a large, heterogeneous group of disorders characterized by derangement of alveolar walls and alveolar capillary unit causing restrictive pulmonary dysfunction and disordered gas exchange. It is rare, especially in children, and the opinions on investigation and treatments are controversial. The classification of adult ILD is applied to that of children. But considering occurrence during the developing state of lung... |
| Acute scrotal disorders(ASD) in neonates-especially the torsion of testes- are rare, and the clinical presentations are quite different from those of adolescents or adults. The neonates with ASD are generally afebrile and do not show typical symptoms. Early diagnosis and proper treatment of ASD are very important but the differentiation of torsion and orchitis is not easy in neonates. The... |
| Purpose : In most instances, constipation is considered as idiopathic or functional. The total colonic transit time, traced by radio-opaque markers, makes possible the identification of the colon segment that has the motility alteration that causes constipation. We measured the total and segmental colonic transit time in children with chronic idiopathic constipation and compared the results with those without constipation... |
| The 49, XXXXY Klinefelter variant is distinctive; classic findings include mental retardation, hypogonadism and radioulnar synostosis. Its incidence is estimated to be 1/80,000 to 1/100,000 male births. We report a case of 49, XXXXY syndrome confirmed by chromosomal study in neonatal period, whose clinical features included a coarse face, short neck, micropenis, and other multiple minor anomalies including simian creases.... |
| Purpose : It is very important to study the individual risk factors which cause periventricular leukomalacia(PVL) and those which affect the process of cystic formation from liquefied periventricular white matter, in order to prevent and permit early diagnosis of cystic PVL that contributes to mortality and neurologic sequelaes in preterm infants. Therefore, the present study was designed to assess the... |
| Protein-losing enteropathy can result from primary or secondary intestinal lymphangiectasia. Secondary intestinal lymphangiectasia develops as a result of lymphatic obstruction or elevated lymphatic pressure. Cardiac lesions such as constrictive pericarditis, post-Fontan procedure state, and chronic congestive heart failure increase lymphatic pressure and may cause intestinal lymphangiectasia and protein-losing enteropathy. An 11-year-old girl who underwent corrective surgery for Tetralogy of Fallot... |
| Purpose : We investigated compounds from food sources given to children that may induce the differentiation of small intestinal epithelial cells in order to signal pathways that induce the proliferation and differentiation of small intestinal epithelial cells. Methods : We analyzed small intestinal epithelial cell differentiation using in vitro IEC-6 cells model. The growth curve of IEC-6 cells was obtained... |
| This is the first reported case of a unique interstitial deletion involving the long arm of chromosome 17 in a Korean male infant born to parents with normal karyotype. The patient presented with multiple congenital malformations suggestive of chromosomal anomaly including round face, upslanted palpebral fissure, hypertelorism, posteriorly rotated low set ear, micrognathia, microcephaly, finger- like thumb, bilateral hearing loss,... |
| Seconnday aneurysmal bone cyst is the disease that occurrs in the primary bone tumor and/or tumorus conditions Meticulous search is needed to make a correct diagnosis which dose not overlook the primary diseases. We have experinced an osteolytic lesion on the 7-year-old boy on his skull bone. Plain radiography of the skull revealed osteolytic lesion in the parieto-occipital bone. On... |
| Purpose : Neutrophils are central to the defences against bacterial infection, and in neonates the number of neutrophils are decreased due to inhibited production and phagocytic function. This induces high mortality rates in infants suffering from neonatal sepsis. Exogenous GM-CSF can increase the number of neutrophils and improve the phagocytic function. To establish the most cost effective dose of exogenous... |
| Purpose : Multicystic dysplastic kidney(MCDK) is characterized by the presence of multiple cysts of varying sizes. It is associated with ureteral or pelviureteral atresia, and a non-functioning kidney. The purpose of this study was to examine the clinical feature and the natural history of MCDK. Methods : A study was performed in 36 children with prenatal diagnosis of MCDK from 1989 to 1999. All... |
| Megacystis Microcolon Intestinal Hypoperistalsis(MMIH) Syndrome is a rare cause of functional neonatal bowel obstruction, characterized by hypoperistalsis, narrow distal ileum and colon, and bladder distension. We report a case of MMIH syndrome and review the literature. The patient was born after 34 weeks of gestation with marked abdominal distension. Her birth weight was 2,830g with 276ml of urine drained by... |
| Cystic lymphangiomas are comparatively rare, benign tumors of lymphatic system and their histogenesis are uncertain. About 75% of theses lesions are in the neck, 20% are in the axillary region, and 5% are in the mediastinum, retroperitoneal region or groin. But retroperitoneal cysticlymphangiomas are very rare. Retroperitoneal cystic lymphangiomas are usually found incidentally during diagnostic procedures performed for unrelated clinical... |
| Purpose : We studied smooth muscle strips from rabbit gastric antrum and low esophageal sphincter(LES) to explore the difference between newborn and adult rabbit on erythromycin(EM)- induced contractions. Another aim of this study was to determine the mechanism of LES contractile activities induced by erythromycin(EM). Methods : Muscle strips prepared from newborn and adult rabbits were stimulated with agonists such as KCl, motilin and EM,... |
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