PURPOSE: Hepatocellular carcinoma (HCC) is a rarely occurring disease in the pediatric population. We report our center's experience of management of HCC in children and adolescents. METHODS: From 1996 to 2012, 16 patients aged 18 or younger were diagnosed with HCC at our center. The medical records of these 16 patients were retrospectively reviewed. RESULTS: There were 9 boys and 7 girls. Median age at diagnosis of HCC was 14.5 years. All patient had pathologically confirmed diagnosis of HCC. Three patients had distant metastasis at the time of HCC diagnosis. Eight patients were surgically managed, including 4 liver resections, 3 liver transplantations, and 1 intraoperative radiofrequency ablation. The remaining 8 patients received systemic chemotherapy. Overall, 6 patients are alive at median 63.6 months after diagnosis of HCC. All survivors were surgically managed patients. CONCLUSION: HCC is a rare disease occurring in childhood. Patients with systemic disease have poor outcome. Liver transplantation may be a good option for treatment of pediatric HCC.
Adolescent* ; Carcinoma, Hepatocellular* ; Catheter Ablation ; Child* ; Diagnosis ; Drug Therapy ; Female ; Humans ; Liver ; Liver Transplantation ; Medical Records ; Neoplasm Metastasis ; Pediatrics ; Rare Diseases ; Retrospective Studies ; Survivors

Transformation and progression of breast cancer are thought to be caused by an accumulation of complex genetic alterations, but little is known about specific changes. In this study, the author has undertaken a genome-wide screening to detect genetic changes in 20 cases of breast cancer among Koreans, including 16 infiltrating ductal carcinomas, 2 medullary carcinomas, 1 invasive lobular carcinoma, and 1 borderline phyllodes tumor. Comparative genomic hybridization (CGH) was used to screen for DNA sequence gains and losses across all human chromosomes. Simultaneous immunohistochemical staining for c-erbB-2 (Her-2/neu), c-myc, cyclin D1, and p53 protein was done to make comparisons with nuclear grade and that with CGH results. Biotin-labeled tumor DNA and digoxigenin-labeled normal DNA were hybridized to normal metaphase cells. The fluorescence signals were captured by fluorescence microscope after detection by avidin-FITC and anti-digoxigenin rhodamine. Then, the ratio of fluorescence was calculated by an image analyzer. The immunohistochemical staining was done in paraffin-embedded tissue with an LSAB kit and avidin-biotin complex (ABC) method. The CGH results showed gains on chromosomes 8q (40%), 1q (30%), 17q (15%), 20q (15%), 18q (15%), 5p (15%), and 13q (15%). Deletions were on chromosomes 17p (45%) and 22q (20%). High-level amplifications (green/red ratio >1.5) were noted on chromosomes 1p31, 1q, 3q25-qter, 5p, 7q31-qter, 8q, 9p22-qter, 10p, 11p, 11q22-qter, 12p, 12q24, 14q21-qter, 15q23-qter, 17q, 18p, 18q12-qter, 20p, and 20q. By comparison with infiltrating ductal carcinoma, the two medullary carcinomas showed high-level amplification on chromosomes 1p31, 1q, 8q, 10p, 11p and 12p. c-erbB-2, c-myc, cyclin D1, and p53 protein expression was immunohistochemically detected in 9 of 20 (45%), 8 of 20 (40%), 10 of 20 (50%), and 13 of 20 (65%), respectively. The results indicate that the amplification on chromosome 8q, 1q and the deletions on chromosomes 17p and 22q are the most frequent genetic alterations in breast cancers among Koreans. The results reveal a different pattern of genetic alteration from previous studies. The CGH results were not correlated with the immunohistochemical profiles. The amplification pattern of medullary carcinomas was quite different from the pattern of infiltrating ductal carcinomas. The CGH was thought to be very useful in the screening of genetic alterations of solid tumors.
Base Sequence ; Breast Neoplasms ; Breast* ; Carcinoma, Ductal ; Carcinoma, Lobular ; Carcinoma, Medullary ; Chromosomes, Human ; Comparative Genomic Hybridization* ; Cyclin D1 ; DNA ; Fluorescence ; Humans ; Mass Screening ; Metaphase ; Phyllodes Tumor ; Rhodamines

This study was undertaken in order to estimate the incidence of leukemia among Koreans. Medical records were studied of patients with diagnoses of either ICD-9 038 (septicemia), or 204-208 (leukemias), or 284 (aplastic anemia), or 289 (other diseases of the blood and blood-forming organs) in the claims sent in by medical care institutions throughout the country to the Korea Medical Insurance Corporation (KMIC) during the period from January 1, 1986 to December 31, 1987. These records were abstracted in order to identify and confirm new cases of leukemia among the beneficiaries of KMIC, which covers about 10% of the whole Korean population. Using these data from the KMIC, the incidence rates of leukemia among Koreans were estimated as of July 1st, 1986 to June 30, 1987. The crude incidence rate of all types of leukemia among Koreans is estimated to be 3.45 (95% CI; 0.77-9.55) and 2.29 (95% CI; 0.28-7.81) per 100,000 in males and females, respectively. The cumulative rate for the age span 0-64 is 0.25% in males and 0.18% in females, and for the age span 0-74, 0.35% in males and 0.23% in females. The adjusted rates for the standard world population are 3.90 and 2.48 per 100,000 in males and females, respectively. The relative frequencies by type are 51.5% for AML, 21.6% for ALL, 20.2% for CML, and only 1.5% for CLL. The incidence patterns of various types of leukemia, of which this is the first report in Korea, are analyzed and presented.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent ; Adult ; Age Factors ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Incidence ; Infant ; Korea/epidemiology ; Leukemia/*epidemiology ; Leukemia, Lymphocytic, Chronic, B-Cell/epidemiology ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/epidemiology ; Leukemia, Myeloid, Acute/epidemiology ; Male ; Middle Aged ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/epidemiology ; Sex Factors

No abstract available.
DNA*

No abstract available.
Blood Cell Count ; Bone Marrow Cells/metabolism/pathology ; Humans ; Leukemia, Promyelocytic, Acute/complications/*diagnosis/pathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Multiple Myeloma/complications/*diagnosis/pathology ; Paraproteinemias/diagnosis ; Syndecan-1/metabolism

No abstract available.
Humans

BACKGROUND/AIMS: Infection of pancreatic necrosis is one of the leading cause of death in patients with severe necrotizing pancreatits. Because of high mortality rate up to 50%, immediate surgical debridement including pancreatectomy is recommended. However, early surgical treatment still showed high mortality rate and better treatment strategy is required. This study was conducted to evaluate the outcomes of early intensive non-surgical treatments in patients with infected necrotizing pancreatitis. METHODS: This study was based on retrospective analysis of 71 patients with acute severe necrotizing pancreatitis (APACHE II score>or=8, or Ranson's score>or=3, and pancreatic necrosis on CT scan), who were admitted to medical center during past 16 years. Infection of pancreatic necrosis was confirmed by fine needle aspiration, and early intensive medical treatments comprised of prophylactic antibiotics coverage, fluid resuscitation, organ preserving supportive measures, and percutaneous catheter drainage were carried out. RESULTS: Among the enrolled patients, infections were suspected in 46 patients, but fine needle aspirations were done only in 32 patients. In 21 patients, infections of necrotic tissue were confirmed by bacteriology, while other 11 patients showed no evidence of bacterial growth. Of 21 patients with infected necrosis, initial surgical interventions were performed in 2 patients, while initial medical treatments were performed in 19 patients. The success rate of medical treatment group in infected necrotizing pancreatitis was 79% (15/19). The mortality rate of medical treatment group and surgical treatment group was 5% (1/19) and 50% (1/2). CONCLUSIONS: Early intensive medical treatment seems to be a good therapeutic strategy, even if the infection has developed in pancreatic necrosis. Further prospective randomized studies are required to confirm this finding.
Bacterial Infections/diagnosis/*prevention & control ; Humans ; Pancreatitis, Acute Necrotizing/complications/diagnosis/*therapy ; Retrospective Studies ; Treatment Outcome

BACKGROUND: Many Studies regarding hemodynamic changes by various vasodilators, such as nitroprusside, nifedipine, and hydralazine have been reported, however little data are available upon acute hemodynamic change due to captopril, an angiotensin converting enzyme inhibitor especially in chronic regurgitant valvular heart disease. Therefore the aim of this study is to evaluate the acute hemodynamic effects of sublingual captopril in patients with regurgitant valvular heart diseases. METHODS: Among the 9 patients enrolled in this study, 5 patients mitral regurgitation, 2 had aortic regurgitation, and 2 had both. Five had patients were male and 4 were female. Before, 15 minutes and 30 minutes after administration of 25mg of captopril via sublingual route, forward cardiac output was measured three times using Swan-Ganz catheter. Right and left cardiac catheterization were also done at each phase and measurement of pulmonary capillary wedge pressures, pulmonary artery pressures, right atrial pressures, aortic pressures, left ventricular pressures were done. RESULTS: 1) Heart rate, pulmonary capillary wedge pressures, cardiac output and cardiac indices left ventricular end-diastolic pressure, diastolic and mean aortic pressures, and diastolic pulmonary artery pressure showed no significant change after administration of sublingual captopril. 2) Systolic aortic pressure decreased significantly from basal value(130+/-35) to 15 minute value(126+/-39). 3) Systemic vascular resistance at 15 minute showed significant reduction as compared with basal value(from 1743+/-551 to 1642+/-491). Pulmonary vascular resistance at 30 minutes(254+/-193) was significantly lower than basal value(282+/-229). CONCLUSIONS: Reductions of systemic and pulmonary vascular resistance occurred relatively rapidly, however, acute effects on cardiac output and pulmonary capillary wedge pressures were not evident. Clinical implication of sublingual captopril in patients with regurgitant valvular heart diseases is worth evaluationg by more extensive hemodynamic studies.
Aortic Valve Insufficiency ; Arterial Pressure ; Arteries ; Atrial Pressure ; Blood Pressure ; Capillaries ; Captopril* ; Cardiac Catheterization ; Cardiac Catheters ; Cardiac Output ; Catheters ; Female ; Heart Rate ; Heart Valve Diseases* ; Hemodynamics* ; Humans ; Hydralazine ; Male ; Mitral Valve Insufficiency ; Nifedipine ; Nitroprusside ; Peptidyl-Dipeptidase A ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Vascular Resistance ; Vasodilator Agents ; Ventricular Pressure

The medical records of inpatients with diagnoses of either ICD-9 193(malignant neoplasm of the thyroid gland) or 226(benign neoplasm of the thyroid gland) in the claims sent in by medical care institutions throughout the country, to the Korea Medical Insurance Corporation (KMIC) during the period from January 1, 1986 to December 31, 1987 were abstracted. These records were abstracted in order to identify and confirm new cases of thyroid cancer among the beneficiaries of the KMIC. Using these data, the incidence rate of thyroid cancer among Koreans was estimated as of July 1, 1986 through June 30, 1987. The crude rates were estimated to be 0.76(95% Cl: 0.63-0.87) and 3.87(95% Cl: 3.60-4.14) per 100,000 in males and females, respectively, and the cumulative rates for the age spans 0-64 and 0-74 in males were 0.06% and 1.10%, respectively. In females, those were equally 0.35%. The age-adjusted rate for the world population was 0.93 per 100,000 in males, which is one of the lowest levels in the world. However, the adjusted rate in females was 3.96 per 100,000, which is an average level and very similar to that of the Chinese in Singapore and Shanghai. A similar tendency was shown in the case of the truncated rates for the age group of 35-64, which was 1.91 per 100,000 in males and 8.82 per 100,000 in females.
Adolescent ; Adult ; Age Factors ; Aged ; Epidemiologic Factors ; Female ; Humans ; Korea/epidemiology ; Male ; Middle Aged ; Sex Factors ; Thyroid Neoplasms/*epidemiology/pathology

PURPOSE: Diabetes insipidus(DI) has been known to be a relatively common complication after craniotomy. We have investigated the incidence and clinical course of DI in children related to craniotomy for a brain tumor and determined the risk factors of postoperative DI. METHODS: Sixty-two pediatric patients, who have undergone craniotomy for a brain tumor(including stereotactic biopsy) from February 1995 through January 1998, were included in this study. We reviewed hospital records retrospectively and analyzed daily fluid intake and output, electrolytes and osmolarity of serum and urine, and urine specific gravity. RESULTS: Of 62 patients, DI developed preoperatively in 7 patients and postoperatively in 7 patients. Preoperative DI composed of 4 germinoma, 2 craniopharyngioma and a histiocytosis, followed by permanent DI after operation. All of the postoperative DI were composed of suprasellar tumors, including 4 craniopharyngioma, which progressed to permanent DI in 5 cases and transient DI in 2 cases. The 5 patients had a triphasic response. The initial phase of DI began within 12 hours postoperatively followed by antidiuretic phase at the 2nd-6th postoperative day, lasted 1-6 days and finally all patients entered permanent DI. Hypopituitarism developed in 10 patients and all of them were accompanied by permanent DI. CONCLUSION: DI is a common complication after neurosurgery for the hypothalamic or pituitary area. The high-risk factors of permanant DI are preoperative DI, combined hypopituitarism or triphasic response. Therefore, it is important to closely monitor this high-risk group, and we should consider endocrinological evaluation in patients who had undergone craniotomy for a brain tumor.
Brain Neoplasms* ; Brain* ; Child* ; Craniopharyngioma ; Craniotomy* ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic* ; Electrolytes ; Germinoma ; Histiocytosis ; Hospital Records ; Humans ; Hypopituitarism ; Incidence ; Neurosurgery ; Osmolar Concentration ; Retrospective Studies ; Risk Factors ; Specific Gravity