A Case of an Imported Dengue Hemorrhagic Fever with Spontaneous Bleeding: Case Report and Review of the Literature / 소아감염

Dengue is an acute febrile viral disease which is found in tropical and sub-tropical regions around the world. Dengue fever has steadily increased in both incidence and distribution over the past 50 years. Even though Korea is not an endemic country for dengue fever, with the increasing numbers of overseas travelers in Korea, the numbers of imported dengue cases are steadily increasing. Here, we report a case of imported dengue hemorrhagic fever in a Korean child presenting with fever and epistaxis. Dengue fever should be considered if a patient who has a recent travel history to endemic areas showed classical symptoms.

A Case of Myocarditis by Measles Virus

Myocarditis is inflammation, necrosis and myocytolysis of myocardium by many infectious, connective tissue, granulomatous, toxic, or idiopathic process. Viruses are probably the most important cause of myocarditis. But the exact cause remains unknown in the majority of patients with presumed myocarditis. We describe a case of myocarditis by measles virus in a 11-year-old girl with a brief review of related literatures.

A Case of Gas Forming Enterocolitis Associated with Kawasaki Disease / 대한소아소화기영양학회지

Kawasaki Disease, an acute systemic vasculitis of unknown etiology, is the leading cause of acquired heart disease in children in many parts of the world. It predominantly affects children under 5 years of age and has many clinical symptoms. We experienced a case of gas forming enterocolitis associated with Kawasaki Disease. Aeromonas hydrophilia was isolated from her stool culture. So,we report the case with a brief review of its literature.

The Study of the Initial Presentations of Wilson Disease at Diagonosis / 대한소아소화기영양학회지

PURPOSE: To review the clinical and laboratory features of patients with Wilson disease at diagnosis. METHODS: In this retrospective study, records of all 20 patients, who were diagnosed as having Wilson disease at the Paik hospital in Busan from 1990 to 2000, were reviewed. RESULTS: Out of 20 patients, 12 pateints (60%) have hepatic presentation alone, 2 patients (10%) have neurologic presentation, 4 patients (20%) have hepatic and neuropsychiatric presentation, and one patient (5%) has hematologic presentation at diagnosis. One patient (5%) has neither symptom nor laboratory finding of Wilson disease except very low serum ceruloplasmin level and positive family history. Family screening test revealed 3 cases of Wilson disease. 12 patients were revealed to be combined with liver cirrhosis at diagnosis. CONCLUSION: Early diagnosis and treatment is very important in patients with Wilson disease. Children or adolescents who manifest symptoms of hepatitis, who has prolonged elevation of liver enzymes, and has family history of hapatitis of unknown origin, with mild hematologic or urinary abnormalities must be suspected to have Wilson disease. Also, in adolescents with extrapyramidal symptoms or other neuropsychiatric symptoms, liver function test should be done.

A Case of Infantile Nephrotic Syndrome with Focal Segmental Glomerulosclerosis

Paraganglioma is a benign tumor arising in the paraganglion system scattered throughout the body, but its cytopathologic findings arenot well known. We experienced a case of paraganglioma of carotid body diagnosed by fine needle aspiration. The patient was a 30 year-old female who suffered from the left neck mass for 3 years. The mass was 3x3crn in size without pulsation or bruit. Cytologically, the smear revealed aggregated and singly scattered tumor cells having abundant pale cytoplasm and indistinct cell borders. Their nuclei were round to oval, but enlarged nuclei were occasionally observed. The nuclear membrane was smooth with fine clumping of chromatin. Differentiation from metastatic follicular carcinoma of the thyroid gland was difficult.

Cardiac Structure and Function and It's Alterations after Growth Hormone Treatment in Children with Growth Hormone Deficiency / 대한소아내분비학회지

PURPOSE:This study was performed to investigate the status of cardiac structure and function and to assess their alterations after growth hormone(GH) treatment in children with growth hormone deficiency(GHD). METHODS:Interventricular septal thickness and left ventriclular posterior wall thickness, ejection fraction(EF), fractional shortening(FS), systolic time interval(STI) of left ventricle were measured by two-dimensional and M-mode echocardiography in sixteen children with GHD and age, sex matched sixteen children with GH normal short stature as control. The measure were done before GH treatment and at 6 and 12 months of GH treatment, respectively. RESULTS: 1)Left ventricular posterior wall thickness in GHD group was significantly thinner than that of control group(P<0.05). 2)Interventricular septal thickness and left ventricular posterior wall thickness were increased with GH treatment from 10.4+/-1.7mm, 8.1+/-1.8mm before GH treatment to 11.0+/-0.9mm, 8.7+/-0.7mm and 11.2+/-1.7mm, 9.7+/-1.8mm at 6 and 12 months of GH treatment, respectively. The increment of left ventricular posterior wall thickness after 12 months GH treatment revealed statistic significance(P<0.05). 3)There was no significant alterations of EF, FS, STI of left ventricle after GH treatment at 6 months and 12 months, respectively. CONCLUSION: Left ventricular posterior wall thickness in GHD group was significantly thin compared to that of control group(P<0.05). GH treatment in GHD children for 12 months, resulted statistically significant increase(P<0.05) in posterior wall thickness. There is no evidence of hypertrophic cardiomyopathy after GH treatment. But we could not exclude the possibility of these alterations were induced by an increased overall body size and body surface area after GH treatment. To clarify the exact alterations of cardiac structures and function in children with GHD after GH treatment, long term follow-up studies should be necessary.

A Case of Superior Mesenteric Artery Aneurysm associated with Marfan Syndrome

Superior mesenteric artery aneurysm is very rare complication of Marfan syndrome, especially in children. A 11 years old male patient was admitted to the hospital because of fluctuating high fever and diagnosed as infective endocarditis and Marfan syndrome. During antibiotics therapy, fever was slowly decreased but abdominal pain was developed and pulsatile abdominal mass was palpable in the midline of the abdomen. And the diagnosis was made as superior mesenteric artery aneurysm by ultrasonogaphy and abdominal aortogram. Resection of aneurysm was performed successfully. A brief review of the related literatures was made.

A Case of Superior Mesenteric Artery Aneurysm associated with Marfan Syndrome

Superior mesenteric artery aneurysm is very rare complication of Marfan syndrome, especially in children. A 11 years old male patient was admitted to the hospital because of fluctuating high fever and diagnosed as infective endocarditis and Marfan syndrome. During antibiotics therapy, fever was slowly decreased but abdominal pain was developed and pulsatile abdominal mass was palpable in the midline of the abdomen. And the diagnosis was made as superior mesenteric artery aneurysm by ultrasonogaphy and abdominal aortogram. Resection of aneurysm was performed successfully. A brief review of the related literatures was made.

Expression of the Ki-67 antigen Using Monoclonal Antibody MIB-1 in Children with Glomerulonephritis

The aim of the present study is to demonstrate the usefulness of intraoperative fine needle aspiration (FNA) of pancreatic lesions in 30 patients. A conclusive diagnosis was done in 27 patients and the diagnoses of three patients were deferred. No complications followed the procedure. Based on histologic findings of the resected specimens in 20 cases and of cell blocks in 10 cases, the final diagnoses were adenocarcinoma in 19 cases, chronic pancreatitis in nine cases and tuberculosis in two cases. The sensitivity, specificity and diagnostic accuracy were 95%, 100% and 96% resepectively and there were no false positives. The smear of aspirate was stained with toluidine blue and examined by light microscope. The presence of there-dimensional clusters of disoriented cells and the increased nuclear/cytoplasmic ratio with large prominent nucleoli were the most helpful criteria for a diagnosis of malignancy in the pancreas. The intraoperative FNA of pancreatic lesions was considered as a simple, safe, and highly specific and sensitive tool in differentiating benign from malignant lesions. The intraoperative FNA can be recommended as the first tool of choice of intraoperative diagnostic procedure in lesions of the pancreas.

A Case of Transverse Myelitis associated with Systemic Lupus Erythematosus

Transverse myelitis is a rare complication of systemic lupus erythematosus (SLE) and its prognosis is very poor including death or severe neurologic sequelae. We report a 14-year-old girl with transverse myelitis who was not exactly diagnosed as SLE before the onset of neurologic symptoms. Transverse myelitis was diagnosed based on the clinical presentations, cerebrospinal fluid analysis and MRI findings. We employed aggressive treatment with pulse methylprednisolone for acute episodes followed by monthly cyclophosphamide pulse therapy. For the first several months, clinical improvement of the neurologic impairment was noted, but unfortunately her neurological course was on exacerbation.

A Case of Clear Cell Sarcoma of the Kidney / 대한신장학회잡지

Clear cell sarcoma of the kidney(CCSK) is one of the rare malignant renal tumors in childhood and is different from Wilms tumor by its pathologic features, clinical manifestations and propensity to metastasize to the skeletal system and brain. We report a case of CCSK on the right kidney in a 14-month-old girl presented with abdominal distention and palpable huge abdominal mass on physical examination, followed by metastasis to the rib on the left upper thorax. After right nephrectomy, chemotherapy with National Wilms Tumor Study DD regimen(vincristine+dactinomycin+adriamycin) was continued without any evidence of relapse until the last follow-up, 1 year after beginning chemotherapy. A brief review of related literatures was made.

Congenital Giant Aneurysm of Pulmonary Artery-Associated with Ventricular Septal Defect and Pulmonary Stenosis : A Case Report

Aneurysm of the pulmonary artery is a rate entity. A neonate was seen with cyanosis and tachypnea. There was a grade 4/6 systolic murmur along the left sternal border. The chest X-ray showed a round mass shadow in the left parahilar region. Echocardiogram showed large Ventricular Septal Defect and mild Pulmonary Stenosis. The cardiac angiogram showed giant aneurysm of pulmonary artery. Surgical intervention was advised. However, the patient was discharged against operative plan. And the patient died two weeks later.

Clinical Studies of Tetralogy of Fallot Associated with Complete Atrioventricular Septal Defect

PURPOSE: The association of the tetralogy of Fallot(TOF) with the complete form of atrioventricular septal defect(AVSD) is rare cardiac malformation.The predominant clinical findings in both groups of cases were those associated with tetralogy of Fallot, but certain features need to be emphasized, however. METHODS: Forteen(2%) out of 659 patients with tetralogy of Fallot seen at the department of pediatrics, Seoul National University Hospital from January 1986 to June 1994, were associated with the complete form of AVSD. We reviewed clinical records, echocardiographic findings, angiographic findings and operative findings of patients. Our diagnostic criteria for TOF with complete AVSD are as follows. 1) Anterosuperior displacement of infundibular septum and RVOT obstruction. 2) Large (nonrestrictive) VSD and an overriding aorta. 3) Primum ASD with common AV valve.Goose-neck deformity of LVOT. RESULTS: 1) Only 14 cases(2%) of the 659 TOF patients were associated with complete AVSD. 2) The male to female ratio was 1:2.5. 3) The predominant features were those associated with TOF, but 9 patients(64.4%) had Down's syndrome. 4) The electrocardiogram showed left axis deviation except 2 patients. 5) Most common type among 14 patients with complete AVSD defect was Rastelli type C(78.5%). 6) Associated cardiovascular anomaly included PDA, ASD of secundum type, right aortic arch, left superior vena cava. 7) Among 12 patients who underwent operations, 9 patients are still alive and followed up. CONCLUSIONS: TOF associated with complete AV septal defect was a rare disease. There was a clinical difference between TOF and AVSD combined groups of case. The presence of Down's syndrome or left-axis deviation with TOF increases the likelihood of associated complete AVSD.Careful preoperative evaluation of these patient is essential to their management and successful operative treatment.

A Case of Congenital Anterior Urethral Valve

No abstract available.