No abstract available.
Adolescent ; Child* ; Humans ; Puberty*

PURPOSE:Insuline-like growth factor I(IGF-I) is polypeptide mitogen and mediate growth effect of growth hormone(GH). It's serum level is regulated by GH. The aim of this study is to evaluate whether -2 standard deviation of IGF-I level in normal short stature after insuline and L-dopa stimulation test has any diagnostic significance in GH deficiency. METHOD:We included 64 children with GH deficiency(complete GH deiciency 18 cases, partial GH deficiency 46 cases). Their height was below 10 percentile of korean children's standard growth chart. Control group was 175 children whose test results were normal after insuline and L-dopa stimulation test. Serum growth hormone level was measured by IRMA(immunoradiometric assay) with "Daiichi" kit(Japan) and serum IGF-I level was measured with 125I RIA kit (U.S.A). RESULTS: 1)Serum IGF-I level in normal stimulation test result group was increased with the age and the level was higher in female than that of male. 2)Using the cut-off value of -2SD of IGF-I level in control group, sensitivity was 17.2%, specificity was 98.86%, positive predictive value was 84.62%, negative predictive value was 76.55%, and test accuracy was 76.99%. Sensitivity and test accuracy was 44.44% and 93.26% in th complete GH deficiency, respectively. 3)Serum IGF-I level was significantly correlated with peak GH level with insuline stimulation test in control and GH deficiency group(Y=0.018889X+11.32 r= 0.23930 P=0.0014, Y=0.008592X+4.189 r=0.28141 P=0.0267). But serum IGF-I level was was not correlated with peak GH level with L-dopa stimulation test(Y= 0.005609X+13.88 r=0.06625 P=0.3823, Y=0.008293X+2.98 r=0.20895 P=0.1031). CONCLUSION: Serum IGF-I level in GH deficiency group was lower than that of control group and had wide variation of normal range. Based upon above results IGF-I level has limited clinical value in the diagnosis of GH deficiency.
Child ; Diagnosis ; Female ; Growth Charts ; Growth Hormone* ; Humans ; Insulin ; Insulin-Like Growth Factor I* ; Levodopa ; Male ; Reference Values ; Sensitivity and Specificity

No abstract available.
Child* ; Humans ; Neurons*

BACKGROUND/AIMS: In order to determine the relationship between the HBV precore mutant and the severity of liver disease in Korea, we performed liver biopsies in patients with HBV related chronic liver disease and compared the types of mutations and histologic findings in the same liver tissue simultaneously. METHODS: HBV DNA in liver tissues was amplified by polymerase chain reaction (PCR). The precore mutants were detected by PCR-SSCP(single strand conformation polymorphism), cloning the amplified PCR products and direct sequencing for them. RESULTS: 1. HBV DNA was detected in liver tissues of 28 cases among 30 patients with PCR. And with SSCP, the most cases were mixed type infections. 2. The HBV precore mutants were found in 12 cases among the total number of 28 cases(42.9%) and all mutations were G to A change at nucleotide 1896, creating a stop codon at codon 28. However, 10 cases among 12 mutants were associated with simultaneous another mutation at different positions or regions;9 cases at core gene region, 2 cases at nucleotide 1856(C to T change at codon 15), one case at core promoter, and one case with double mutations at nucleotide 1837 and 1846 respectively. Also, all HBV precore mutants were combined with wild type HBV sequence. 3. The relationship between HBV precore mutants and HBeAg status revealed that 4 cases from 13 HBeAg positive(30.8%) and 8 from 15 HBeAg negative or Anti-Hbe positive(53.3 %) were mutants. 4. In analysis of the types of mutants and histopathological findings of liver diseases, 6 among 15 chronic active hepatitis(40.0%), all 3 cases with hepatocellular carcinoma(100,0 %), 2 among 4 asymptomatic carriers with minimal histopathologic changes(50.0%) and a case with chronic lobular heaptitis(100.0%) showed precore region mutation. CONCLUSION: The patterns of HBV precore mutants in Korea could be summarized as followings. Firstly, most of the mutations are composed of G to A change at nucleotide 1896. Secondly, the most of the mutants at nuclmtide 1896 have been associated with simultaneous mutations at core promoter, core gene, and rarely at other positions, and manifested usua'ly mixed type viremic conditions. Thirdly, although precore mutation could be occurred in asymptomatic carrier, this type of mutation might be closely related with chronic or severe liver disease. However, it needs further investigations hereafter.
Biopsy ; Clone Cells ; Cloning, Organism ; Codon ; Codon, Terminator ; DNA ; Hepatitis B e Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Korea ; Liver Diseases ; Liver* ; Polymerase Chain Reaction ; Polymorphism, Single-Stranded Conformational

PURPOSE: Hepatitis B virus(HBV) with various mutations has been reported. The aims of this study were to investigate the frequency and manifestation of HBV pre-S/S mutations in children with chronic hepatitis B infection. METHODS: Sera from 17 children with chronic hepatitis B infection were analyzed by direct sequencing of polymerase chain reaction amplification of HBV DNA. Results: Seventeen cases of adr type were analyzed. The deletions in HBV pre-S region were observed in 3(17.6%) of 17 cases. Of 3 deleted cases, 2 had an in-phase deletion in the pre-S1 region spanning 18 bp. Another case had a 18 bp and 3 bp deletions in the pre-S1 region. Many point mutations in HBV pre-S region were detected in all cases and these mutations were observed more frequently in the pre-S2 region than the pre-S1 region. Six point mutations in the pre-S1 region were observed. Eight point mutations in pre-S2 region were observed. Point mutations in the S region were detected in 14(82.4%) of 17 cases. Among these, mutations of the "a" determinant were detected in 4(23.5%) of 17 cases. Mutations at codon 130 and at codon 146 were noted in 2 cases. Combined mutations at codon 124, 126, 146 and at 130, 131, 136, 146 were noted in the other 2 cases. Mutations except "a" determinant region included at codon 3, 29, 73, 120, 184, 214, 226, 227. CONCLUSION: These observations suggest that deletion and point mutations in HBV pre-S1, pre- S2 regions and point mutations in HBV S region are frequent in the children with chronic hepatitis B infection.
Child* ; Codon ; DNA ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis B, Chronic* ; Hepatitis* ; Hepatitis, Chronic* ; Humans ; Point Mutation ; Polymerase Chain Reaction

No abstract available.
Esophageal Atresia*

No abstract available.
Neurofibromatoses* ; Pseudarthrosis* ; Ulna*

PURPOSE: The aims of this study were to investigate the frequencies and role of hepatitis B virus(HBV) precore and core promotor mutations in children with chronic hepatitis B infection. METHODS: Sera from 46 children with chronic hepatitis B infection were analyzed by direct sequencing of polymerase chain reaction product of HBV DNA. In this study, the patients were divided into vertical and non-vertical groups according to the mode of HBV transmission. Statistical analysis was performed by using Fisher's exact test. RESULTS: Forty-six adr type of HBV DNA were analyzed. The mutations in HBV precore region were observed in 12(26.1Yo) of 46 cases. The GA mutation of nucletide(nt) 1896 was observed in 5 cases(10.9Yo). The frequency of mutations in HBV precore region of the non-vertical group (6/16; 37.5Fo) was higher than that of the vertical group(6/30; 20M), but there was no statistical significance. The mutation in HBV core promotor region was observed in 40(87.0%) of 46 cases. The A-->T mutation of nt 1762 or G-->A mutation of nt 1764 were observed in 24(52.2%) of 46 cases, and 23 cases revealed combined mutation at both positions 1762 and 1764. The frequency of mutations in HBV core promotor region of the vertical group(28/30; 93.3Yo) was higher than that of the non-vertical group(12/16; 75.0M), but there was no statistical significance. The frequencies of mutations in HBV precore and core promotor regions of the HBeAg negative patients was higher than that of HBeAg positive patients, but there was no statistical significance. Also there were no significant correlations between the frequencies of mutations in HBV precore and core promotor regions and AST, ALT level or the level of HBV DNA. CONCLUSION: These observations suggest that mutations in HBV precore and core promotor regions were frequently detected in children with chronic hepatitis B infection. There were no statistical significant differences in the frequencies of mutations in HBV precore and core promotor regions between vertical and non-vertical transmission groups. (J Korean Pediatr Soc 2000; 43:779-791)
Child* ; DNA ; Hepatitis B e Antigens ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis B, Chronic* ; Hepatitis* ; Hepatitis, Chronic* ; Humans ; Polymerase Chain Reaction ; Promoter Regions, Genetic

Hepatocellular carcinoma (HCC) is malignant tumor frequently occurring in Koreans. There have been few reports regarding the cytologic findings of fine needle aspiration (FNA) of HCC. Most have suggested a diagnostic problem in the cytology distinguishing HCC from some benign hepatic lesions-for example, a regeneration nodule in cirrhosis and liver cell adenoma. In spite of its high frequency in Korea, no cytologic study has been reported, concerning the FNA of HCC. In an attempt to achieve cytologic criteria for the diagnosis of HCC, the authors studied retrospectively cytopathologic findings of 247 cases of HCC. These cases were confirmed either by histologic examination including lobectomy, biopsy, or cell block materiai, or, when tissue diagnosis was unavailable, by a high serum alpha-fetoprotein level (over 400 l. U.). All aspiration smears were stained by the Papanicolaou method. In each case, the smears were analyzed for cell patterns and various cytomorphology of the tumor cells. The smear background was assessed for the presence of tumor cell necrosis and inflammatory components and compared to that of metastatic carcinomas. The cell patterns were classified as trabecular, acinar, dispersed, and irregular. The cytologic parameters analyzed included the degree of nuclear atypia and the presence of mitoses, intranuclear cytoplasmic inclusions, nucleolar prominency, endotheiial lining, multinucleated giant cells, eosinophiic globules, bile, and Mallory body.Most of the FNA of HCC showed markedly cellular smears. The tumor cells were most frequently arranged in a trabecular pattern (80.3%). The irregular (12.6%), the acinar (5.5%), and the dispersed patterns (1.7%) followed in decreasing frequency. Individual hepatoma cells were larger than normal liver cells. However, they had morphologic features characteristic of the hepatic cells: the cells were round or polygonal, their cytoplasm was abundant and granular with eosinophilic or amphophilic stainability, and their nuclei were round to oval, located centrally, and tended to have prominent nucleoli. Anaplasia and pleomorphism of tumor cells were generally mild to moderate. These findings existed even in very well differentiated cases. Mitotic figures were present in about 85% of the cases. Prominent nucleoli were observed only in about half the cases. The frequency of other cytologic features was as follows: intranuclear cytoplasmic inclusion in 86.8%; endothelial lining in 56.1%: bile in 19.8%; and giant cells in 60.1%. Clear cells were often present in11.7%, Most aspiration smears of HCC displayed clean background without necrosis or inflammatory material in contrast to the dirty, necrotic background of metastatic cancers and cholangiocarcinomas. Based on the above mentioned features, it is suqqested that the cytologic critieria most important for the diagnosis of HCC include a markedly cellular smear, trabecular pattern, hepatocytoid appearance of tumor cells, endothelial lining, the presence of bile, giant cells, intranuclear cytoplasmic inclusions, and prominent nucleoli, Among these, trabecular pattern, endothelial lining, giant cells and clean smear background are points to be considered in differentiating HCC from metastatic and cholangiocellular carcinoma.
Adenoma, Liver Cell ; alpha-Fetoproteins ; Anaplasia ; Bile ; Biopsy ; Biopsy, Fine-Needle ; Carcinoma, Hepatocellular ; Child* ; Cholangiocarcinoma ; Cytoplasm ; Diagnosis ; Endothelial Cells ; Eosinophils ; Fibrosis ; Giant Cells ; Glomerulonephritis* ; Hepatocytes ; Humans ; Inclusion Bodies ; Korea ; Liver ; Mitosis ; Necrosis ; Regeneration ; Retrospective Studies

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