We experienced a case of Rotor syndrome in an 8 year 6 month old boy who presented with icteric sclera and icteric skin on whole body. His clinical and laboratory characteristics were as follows; 1) Jaundice appeared from several months ago before admission without any clinical disturbance. 2) Direct bilirubin was more increased than indirect bilirubin. 3) Plasma indocyanine green (ICG) kinetics test revealed hepatic excretory in this patient that after intravenous injection of a standard dose of ICG (0.5mg/kg), its initial plasma disappearance is decreased, resultin in markedly increased retention of the dye 45 to 50 minutes after administration. 4) Oral cholangiography, ultrasonogram, and computed tomogram of abdomen were normal. 5) Liver biopsy specimen showed no dark brown pigmentations in the hepatic cells and any other pathologic abnormalities. 6) Nearly similar clinical and laboratory findings were observed 26 months later.
Abdomen ; Bilirubin ; Biopsy ; Cholangiography ; Hepatocytes ; Humans ; Hyperbilirubinemia, Hereditary* ; Indocyanine Green ; Infant ; Injections, Intravenous ; Jaundice ; Kinetics ; Liver ; Male ; Pigmentation ; Plasma ; Sclera ; Skin ; Ultrasonography

To elucidate the clinical characteristics-especially its clinical pattern and outcome-of children with encephalopathy, we carried out a retrospective review on medical records of 45 patients who were initially diagnosed as encephalopathy except mumps meningitis, Reye's syndome and neonatal hypoxic ischemic encephalopathy at the Department of pediatrics, Hanyang University children's hospital from Jaunary 1986 to February 1994. The results were summarized as follows: 1) Twenty five were male and twenty were female with male to female ratio 1.3:l. 2) There were no association with death rate and conscious level, abnormal liver function test, and sugar level in CSF on admission. 3) Death rate (33.3%) of group with seizure was higher than that (13.3%) of group without seizure on admission, but there was no statistical significance (p=0.28). 4) Death rate (7.5%) of group (l) which initial therapy started before 12hours was lower than that (43.4%) of group (ll) which initial therapy started after 12hours, but there was no statistical significance (p=0.09). However complete recovery rate (66.7%) of the former group was higher than that (26.7%) of the latter group with statistical significance (p<0.05). Therefore, early diagnosis and treatment before 12hours was essential to recovery and good prognosis in childern with acute encephalopathy.
Child* ; Early Diagnosis ; Female ; Humans ; Hypoxia-Ischemia, Brain ; Liver Function Tests ; Male ; Medical Records ; Meningitis ; Mortality ; Mumps ; Pediatrics ; Prognosis ; Retrospective Studies ; Seizures

No abstract available.
Child* ; Humans ; Prognosis*

In order to obtain some informations on the nature and relative activity of the phosphatases present in various helminths, biochemical studies have been made in thirteen kinds of worm parasites including the adults and larvae (Fasciola hepatica, Eurytrema pancreaticum, Paramphistomum sp., Taenia solium, Taenia pisiformis, Dipylidium caninum, Diphyllobothrium mansoni, Cysticercus cellulosae, Cysticercus fasciolaris and Sparganum). A comparison based on the analysis of pH-activity curves was made among these helminths. The worm materials were mostly obtained alive from an abattoir and removed from the organs or tissues of the animal hosts naturally infected. Sparganum and Cysticercus cellulosae, however, are collected from the subcutaneous tissue of the patients by surgical removal. The worms thoroughly washed were weighed and transferred with 0.1 M Tris buffer to a chilled glass grinder (Capacity; 15 ml) and homogenized in the cold. The homogenate was centrifuged at 5000 RPM for 30 minutes. The supernatant was pipetted off for determination of the phosphatase activity. Incubation mixtures consisted of 1 ml substrate, 1 ml buffer and 0.5ml extract. The buffers used were Tris (Hydroxymethyl) aminomethane and citric acid monohydrate and the substrate was paranitrophenyl phosphate (1 gm/25 ml). These mixtures were incubated at the temperature of 37 C for 30 minutes in water bath. The absorbance or transferance of mixture was determined colorimetrically by "Spectronic 20 "spectrophotometer at 410 nm against a distilled water blank. The amount of phenol liberated was then calculated from a standard curve using phenol solutions. Controls consisted of unincubated mixtures. The results were deducted from this experiment. The phosphatase activity occurred over all parasitic helminths used in this experiment. In trematodes, pH-activity curves have demonstrated two peaks of phosphatase activity in Fasciola hepatica and Paramphistomum species. However the acid phosphatase activity was predominantly found and the alkaline phosphatase activity was found distinctly to be low in all three species. In Eurytrema pancreaticum, the pH-activity curves displayed two peaks in acid phosphatase activity, one at pH 5.0 and the other pH 9.0. In cestodes, both alkaline and acid phosphatase activity displayed the pH optima 5.0 and 9.0 to 10.0 in the adult tapeworms. However, major activity in the adults is due to the alkaline phosphtases. In contrast to the adults, Cysticercus and sparganum showed the higher activity in acid phosphatases which predominates in the larvae. In all cases of nematodes, the pH optimum for acid phosphatase was 4.0 to 6.0. A preponderance of acid phosphatase activity was shown in the extract of intestine of Ascaris lumbricoides. The aspect that phosphatases are correlated with phosphorylated passage of substances through the cuticle of helminths and may also be involved in carbohydrate metabolism is discussed.
parasitology-helminth-trematoda-cestoda ; Fasciola hepatica ; Eurytrema pancreaticum ; Paramphistomum sp. ; Taenia solium ; Taenia pisiformis ; Dipylidium caninum ; Diphyllobothrium mansoni ; Cysticercus cellulosae ; Cysticercus fasciolaris ; sparganum ; alkaline phosphatase ; acid phosphatase ; biochemistry

No abstract available.
Child* ; Ductus Arteriosus, Patent* ; Humans

Epstein-Barr virus (EBV) has been linked to a spectrum of neoplastic conditions, including Burkitt's lymphoma, nasopharyngeal carcinoma, Hodgkin's disease, lymphoepithelioma-like carcinomas and malignant lymphomas in immunocompromised state. To determine the prevalence and the subtype of EBV in gatrointestinal malignancies, fifty cases of adenocarcinomas and seventeen cases of malignant lymphomas were analyzed by EBERs in situ hybridization and polymerase chain reaction using primers for EBNA-1, EBNA-2A and EBNA-2B, on the paraffin sections. In addition, immunohistochemical stain for p53 protein was performed to investigate the potential role of EBV infection on tumor suppressor gene, p53, during tumorigenesis. EBER was detected in 6 of 26 gastric adenocarcinomas, 2 of 24 colon adenocarcinomas, and 8 of 17 malignant lymphomas. EBER was more prevalent in malignant lymphoma arising in the intestine (6/6) than in the stomach (2/11), and was detected in both B and T cell phenotypes. EBNA-1 was positive in 11 of 16 EBER positive cases and the subtyping was possible in 8; both type 1 and 2 were detected in gastric cancers, whereas only type 2 was found in intestinal neoplasms. In adenocarcinomas the high rate of p53 protein overexpression was found in both EBER positive (8/8) and negative cases (32/42), whereas the positive rate was higher in EBER positive cases (7/8) than in EBER negative cases (4/9) of malignant lymphomas. From the results, it can be concluded that EBV infection and the p53 tumor suppressor gene are independently associated in a significant portion of the gastrointestinal malignancies, but the mechanism of action remains to be elucidated.
Adenocarcinoma* ; Burkitt Lymphoma ; Carcinogenesis ; Colon ; Epstein-Barr Virus Infections ; Gastrointestinal Tract ; Genes, Tumor Suppressor ; Herpesvirus 4, Human* ; Hodgkin Disease ; In Situ Hybridization ; Intestinal Neoplasms ; Intestines ; Lymphoma* ; Paraffin ; Phenotype ; Polymerase Chain Reaction ; Prevalence ; Stomach ; Stomach Neoplasms

Estrogen and progesterone receptors exist in the epithelial and stromal cells of the endometrium. Proliferative disorders of the endometrium may be associated with autocrine and paracrine actions of estrogen and progesterone in epithelial and stromal cells. This study was performed to evaluate the differences estrogen and progesterone receptor(ER/PR) expression in the epithelial and stromal cells of endometrial hyperplasias and adenocarcinomas using immunohistochemical methods. Immunohistochemical analysis of proliferating cell nuclear antigen(PCNA) was done to evaluate a possible correlation between PCNA and hormone receptor expression. Evaluation was based on samples from 31 simple hyperplasias, 30 complex hyperplasias, and 32 adenocarcinomas. The immunohistochemical expression of ER, PR and PCNA in epithelial and stromal cells were examined according to a scoring system based on the percentage of positive cells and the staining intensity. The results were as follows; 1) The expression of ER and PR in epithelial cells showed a graded, significant decreases in simple hyperplasia, complex hyperplasia and endometrial carcinoma, in that order(ER: P=0.008, PR: P= 0.026). 2) PR expression in the stromal cells showed a significant decrease between hyperplasia and adenocarcinoma(P=0.003). The difference in ER expression was not significant. 3) In stromal cells, the decrease in PR expression was more prominent than the decrease in ER expression when complex hyperplasia was compared to simple hyperplasia. 4) The PCNA expression in simple and complex hyperplasia and adenocarcinoma was not higher than the expression of PCNA in nomal proliferative endometrium. There was no significant difference in PCNA expression between simple and complex hyperplasia and adenocarcinoma(P=0.073). 5) A negative correlation between PCNA and ER/PR expression was not demonstrated in simple and complex hyperplasia, or in adenocarcinoma. Endometrial hyperplasia and adenocarcinoma are probably related to a paracrine action of estrogen and progesterone in epithelial and stromal cells. A progressive loss of PR expression in stromal cells may induce abnormal proliferation of endometrium due to a disrupted hormonal balance.
Adenocarcinoma

No abstract available.
Peptic Ulcer Perforation* ; Peptic Ulcer*

Direct contact method of autoradiography was applied in order to know in Eurytrema pancreaticum which was incubated at various intervals such as 60, 120, 240, and 360 minutes in the vitro media added with radioactive succinic acid, C(14)-succinate. The distribution of the radioactive succinate was clarified by this technique and possible explanation was presented. The autoradiographic procedures were essentially the same as those reported previously by Yoon et al. (1964). The most distinct autoradiograms were obtained after 240 minutes incubation. The radioactivity of this labelled succinic acid chiefly concentrated in reproductive organs, such as testes, ovary, egg containing uterine tubules and vitelline follicles.
paraisology-helminth-trematoda-Eurytrema pancreaticum ; autoradiography ; metabolism-succinic acid ; succinic acid

OBJECTIVE: Although ovarian cancer is the leading cause of death among all cancers of the female reproductive tract, the genetic alterations involved in ovarian cancer remains largely unknown. Recently, mutations of the p53 gene have been documented in many types of human cancer including ovarian cancer. METHODS: In tbe present study, p53 gene mutation was examined in DNA samples extracted from paraffin embedded surgical specimens of ovarian cancer. Furthermore, clinicopathological parameters were examined in relation to p53 gene mutation in order to understand the role of p53 mutation in the development of ovarian cancer. Using the polymerase chain reaction(PCR) and single strand conformational polymarphism(PCR-SSCP), p53 gene mutation was examined and the mutations were confirmed by DNA scquencing in 17 cases of ovarian cancer. RESULTS: Abnormal bands indicating mutation were detected in 2/17(11.8%). DNA sequencing confirmed in 2 mutations and revealed C to T and A to T nucleotide chmges. In clinicopathological parameters, FIGO stage, grade, and recunence were not correlated with the p53 gene mutation. However, the recurrence rate was higher in patients with mutant p53 compared with those with wild type p53(50.0% vs 13.3%), altbough this is not statisticaUy significant. CONCLUSION: In conclusion, p53 gene mutation shows no correlation with stage, grade and recurrence, and p53 gene mutation does not appear to be a marker that predicts the biological behavior or the outmme of the disease. This study suggested useful data to elucidate the mechanism of chemotherapy-resistant ovarian cancer and further p53 expression assay would be mandatory for p53 nonfunctioning ovarian cancas.
Cause of Death ; DNA ; Female ; Genes, p53* ; Humans ; Ovarian Neoplasms* ; Paraffin ; Recurrence ; Sequence Analysis, DNA