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.
Body Size ; Body Surface Area ; Cardiomyopathy, Hypertrophic ; Child* ; Echocardiography ; Follow-Up Studies ; Growth Hormone* ; Heart ; Heart Ventricles ; Humans

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.
Child ; Male ; Female ; Humans ; Adenocarcinoma

No abstract available.

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.
Aneurysm* ; Cyanosis ; Heart Septal Defects, Ventricular* ; Humans ; Infant, Newborn ; Pulmonary Artery ; Pulmonary Valve Stenosis* ; Systolic Murmurs ; Tachypnea ; Thorax

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.
Adult ; Biopsy, Fine-Needle ; Carotid Body ; Chromatin ; Cytoplasm ; Female ; Glomerulosclerosis, Focal Segmental* ; Humans ; Neck ; Nephrotic Syndrome* ; Nuclear Envelope ; Paraganglioma ; Thyroid Gland

BACKGROUND: The purposes of this study are to determine the prevalence of malnutrtion and its trend of change and to establish its relationship with dysphagia and functional outcome in rehabilitation patients. METHOD: We included 39 patients who were admitted into rehabilitation ward, and we evaluated nutri- tional parameters(serum albumin, serum lymphocyte, and weight loss) and dysphagia at the time of admi- ssion and four weeks after admission. We analyzed total rehabilitation inpatients and stroke patients who were major inpatients of rehabilitation ward. We measured Functional Independence Measure(FIM) and length of stay as clinical outcome scales. RESULTS: Malnutrition was observed in 76.9% of total patients and 69.6% in stroke patients at the time of admission. After four weeks, malnutrition was observed in 65.4% and 66.7%, respectively. Prevalence of dysphagia was 25.0% in non-malnourished patients and 22.0% in malnourished patients at the time of admission. It was not changed significantly after four weeks of rehabilitation. The length of stay was 31.3+/-16.0 days in non-malnourished patients and 39.8+/-19.4 days in malnourished patients. The FIM effi- ciency was 1.0+/-0.6 in non-malnourished patients and 2.3+/-6.5 in malnourished patients. CONCLUSION: Our findings suggest that malnutrition was highly prevalent in rehabilitation inpatients and it did not improved significantly through four weeks of rehabilitation. Therefore, it is important to be concerned about malnutrition and it needs proper and active management.
Deglutition Disorders ; Humans ; Inpatients* ; Length of Stay ; Lymphocytes ; Malnutrition* ; Prevalence ; Rehabilitation* ; Serum Albumin ; Stroke ; Weights and Measures

Oculocutaneous albinism resulting from genetic defect of melanin synthesizing system is characterized by pale skin, straw-colored hair, hypopigmentation of the iris, hypoplasia of fovea, photophobia, low visual acuity and strabismus. In general, oculocutaneous albinism can be distinguished by its clinical feature and hair follicle incubation test but should be diagnosed by electron microscopic findings of the skin which is exposed to sunlight. We experienced a case of 6-year-old female oculocutaneous albinism that showed clinical typical features and was diagnosed through electron microscopic finding of many immature melanosomes of the skin in the back of the hand. We report this unusual case with literature review.
Albinism, Oculocutaneous* ; Child ; Female ; Hair ; Hair Follicle ; Hand ; Humans ; Hypopigmentation ; Iris ; Melanins ; Melanosomes ; Photophobia ; Skin ; Strabismus ; Sunlight ; Visual Acuity

Malignant germ cell tumors of the ovary are uncommon neoplasms. Although 20-25% of all ovarian tumors are derived from germ cells, only about 3% of germ cell tumors are malignant. Mixed germ cell tumors contain at least two malignant germ cell elements. These lesions should be managed with combination chemotherapy, preferably BEP. Recently we experienced a case of mixed germ cell tumor with 6 components of germ cell and sarcomatous change in a 11 year old girl. Preoperative CA-125, B-hCG, aFP, LDH, a-1-antitrypsin were elevated and the final pathologic report was mixed germ cell tumor composed of endodermal sinus tumor, embryonal carcinoma, mature and immature teratoma, choriocarcinoma, dysgerminoma and sarcomatous change, Postoperative chemotherapy with 6 courses of BEP regimen was performed and all tumor markers became normal after 4 courses of chemotherapy. What we interested in this case was several components of germ cells and sarcomatous change and the sarcomatous change might be derived from the mature cystic teratoma component, so we present this case with a brief review of the literatures here.
Carcinoma, Embryonal ; Child ; Choriocarcinoma ; Drug Therapy ; Drug Therapy, Combination ; Dysgerminoma ; Endodermal Sinus Tumor ; Female ; Germ Cells* ; Humans ; Neoplasms, Germ Cell and Embryonal* ; Ovary ; Pregnancy ; Teratoma ; Biomarkers, Tumor

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.
Adolescent ; Busan ; Ceruloplasmin ; Child ; Diagnosis ; Early Diagnosis ; Hepatitis ; Hepatolenticular Degeneration* ; Humans ; Liver ; Liver Cirrhosis ; Liver Function Tests ; Mass Screening ; Retrospective Studies

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.
Aorta ; Aorta, Thoracic ; Axis, Cervical Vertebra ; Congenital Abnormalities ; Down Syndrome ; Echocardiography ; Electrocardiography ; Endocardial Cushion Defects ; Female ; Humans ; Male ; Pediatrics ; Rare Diseases ; Seoul ; Tetralogy of Fallot* ; Vena Cava, Superior