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,... |
Purpose : Postinfectious acute glomerulonephritis usually needs no renal biopsy. But atypical clinical course and laboratory results indicate a need for renal biopsy. Therefore, to investigate clinicopathological characteristics of postinfectious acute glomerulonephritis, we compared clinical manifestations of biopsy group with those of non-biopsy group. Methods : We reviewed the records of clinical and pathological data of 20 cases diagnosed by... |
Purpose : A retrospective study was undertaken to test the hypothesis that febrile neonates, who have bacterial infections, can be accurately predicted early by diagnostic criteria. Methods : We conducted a retrospective study of 152 infants, 28 days or less of age, whose rectal temperature was at least 38℃. Past history, family history, finding on physical examinations and results of CBC,... |
The most clearly delineated condition with prominent cutaneous leukocytoclastic vasculitis which presents to a pediatrician is Henoch-Schönlein purpura. There is general agreement that in Henoch-Schönlein syndrome the skin manifestations have a very benign long-term course and that corticosteroids are of little value. Dapsone appears to be of special value in diseases characterized by an accumulation of polymorphonuclear neutrophils notably leukocytoclastic... |
Primary intestinal lymphangiectasia is a rare congenital disorder characterized by diffuse or localized ectasia of the enteric lymphatics, often in association with lymphatic abnormalities elsewhere in the body. The pathogenesis of these abnormal lymphatic structures is uncertain. Ectatic lymphatics may be located in the mucosa, submucosa, or subserosa, leading to a loss of protein and lymphocytes into the gut or... |
Purpose : The authors performed this study to find out the associated GI disease and H. pylori infection in children with iron-deficiency anemia(IDA). Methods : Twenty-six children older than 2 years with IDA and no history of insufficient iron intake were investigated from 1994 to 1998. Gastrofiberoscopic examination and biopsy and rapid urease test were performed with serum H. pylori IgG.... |
Purpose : Intestinal malrotation is a developmental anomaly and may be complicated by volvulus and intestinal necrosis. We performed anatomic classification and compared clinical profiles to understand their relationship. Methods : Twenty-eight children were diagnosed as intestinal malrotation at Seoul National University Children's Hospital between Jan. 1980 to Dec. 1995. The patients were classified into 4 groups by age at... |
Purpose : Due to controveries surrounding in the incidence of coarctation of aorta which reports previously stated rare among Orientals, we evaluated clinical characteristics, natural courses of coarctation of aorta and effects of different surgical modalities. Methods : Medical records of 51 patients with coarctation of aorta, diagnosed by 2-dimensional echocardiography in Kyungpook University Hospital Pediatric Cardiac Laboratory from June 1985... |
Purpose : The survival rate of those with acute lymphoblastic leukemia(ALL) has been affected by several prognostic factors and previous reports show that a 4-year event-free survival(EFS) for high-risk(HR) group(Age≥10 years and/or WBC≥50,000/mm3 at diagnosis) was approximately 64%. We analyzed treatment outcomes and risk factors in HR-ALL. Methods : Medical records of 27 patients with HR-ALL diagnosed between May, 1985 and... |
Purpose : It has been shown that a considerable number of acute myeloid leukemia(AML) patients had both myeloid and lymphoid antigens. CD7 is a lymphoid antigen, often expressed in AML cells. We investigated 40 AML patients to clarify the relationship between the cellular characteristics and clinical features. Methods : We reviewed 8 CD7 positive(CD7+) AML patients and 32 CD7 negative(CD7-)... |
Allergic bronchopulmonary aspergillosis(ABPA) is an inflammatory disease which causes a hypersensitivity to Aspergillus spores growing in the bronchi. The clinical syndrome is characterized by asthma, recurrent pulmonary infiltrations or mucoid impaction, eosinophilia, and central bronchiectasis. A 12-year-old boy was admitted to our hospital because of right lower chest pain and cystic mass-like shadows on a chest X-ray film. He had... |
Individuals with Down syndrome have a high incidence of hematologic diseases such as transient myeloproliferative disorder(TMD) & acute leukemia. Because it is difficult to distinguish TMD from acute myeloblastic leukemia, the diagnosis in neonate, who have Down syndrome, should be made with extreme caution. TMD usually undergoes spontaneous remission within a few months, but acute leukemia can develope after remission... |
Purpose : A study was undertaken to assess the social background of abandoning children at adoption institutions. The results were compared to those of three previous reports from the institution. Methods : A total of 536 children who had been admitted to the institution during the period of 1993 to 1996 were subjected to the study. The medical records were... |
Purpose : In recent years, the authors experienced a distinctive clinical entity of multiple intestinal ulcerations and perforations in infants. The purpose of this study was to describe the clinical characteristics of this entity, examine th possible pathogenesis and the effective treatment. Methods : Seven infants underwent abdominal exploration under suspicion of surgical abdomen and were noted to have multiple intestinal... |
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