Zhonghua yi xue za zhi = Chinese medical journal; Free China ed最新文献

{"title":"Hereditary hemorrhagic telangiectasia.","authors":"Ya-Fen Peng, Liang‐Kung Chen, Y. Chou, F. Chang, Shinn-Jang Hwang","doi":"10.32388/wtlzrr","DOIUrl":"https://doi.org/10.32388/wtlzrr","url":null,"abstract":"Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a rare disorder characterized by arteriovenous communications in visceral organs. The diagnosis of HHT consists of recurrent epistaxis, mucocutaneous telangiectasis, visceral vascular lesion and familial occurrence. HHT can be definitely diagnosed with the presence of all these three criteria. The prevalence of liver involvement of HHT was reported to range from 8 to 31%. Herein, we present a 75-year-old male who was diagnosed as having HHT with liver involvement, based on the findings of recurrent epistaxis, mucosal telangiectasis on the lower lip and hepatic arteriovenous malformation. The clinical presentations of this patient are discussed, and the literature is reviewed.","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79176826","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Juvenile myoclonic epilepsy.","authors":"W. Shian, C. Chi","doi":"10.32388/sbbbal","DOIUrl":"https://doi.org/10.32388/sbbbal","url":null,"abstract":"BACKGROUND\u0000Recognition of the significance of juvenile myoclonic epilepsy (JME) in the English-language neurological literature is relatively new. There are many factors responsible for delay in diagnosis of JME, including lack of familiarity with the syndrome, failure to elicit a history of myoclonic jerking and absence or generalized tonic-clonic seizures predating myoclonic jerks in some patients.\u0000\u0000\u0000METHODS\u0000The medical and electroencephalographic (EEG) records of seven Chinese children with JME, four males and three females, were reviewed.\u0000\u0000\u0000RESULTS\u0000The age of onset ranged from 10 to 14 years with mean 11.8 +/- 1.6 years. The precipitating factors were sleep deprivation (5/7), photostimulation (3/7), hyperventilation (2/7) and menstruation (1/3). Childhood and juvenile absence epilepsies predated JME in three and one patients, respectively. All patients had concomitant grand mal on awakening. Generalized 3-4 Hz polyspikes-wave complexes occurred in all patients, and two patients had additional 3-4 Hz spike-wave complexes. These activities were provoked with photic stimulation (3/7) and hyperventilation (2/7). All patients were treated with valproate for more than four years, and relapse occurred six months to one year after discontinuation of valproate.\u0000\u0000\u0000CONCLUSIONS\u0000JME is a benign generalized epilepsy with a strong genetic basis. Valproate is the drug of choice up to now, but long-term (life-long) medication may be needed.","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-02-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84505600","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital muscular dystrophy.","authors":"F. Huang, S. Mak, C. Chi","doi":"10.5580/e3d","DOIUrl":"https://doi.org/10.5580/e3d","url":null,"abstract":"Defect: Type of CMD: Extracellular matrix protein defects: • Laminin-alpha2-deficient CMD (MDC1A) • Ullrich CMD (UCMD 1, 2, 3) Integrin-alpha7 deficiency (ITGA7) Glycosyltransferases (abnormal Oglycosation of alpha-dystroglycan) • Walker-Warburg syndrome • MEB disease • Fukuyama CMD (FCMD) • CMD + secondary laminin deficiency 1 (MDC1B) • CMD + secondary laminin deficiency 2 (fukitin related protein deficiency, MDC1C) • CMD with mental retardation and pachygyria (mutation in LARGE, MDC1D) Proteins of the endoplasmic reticulum – rigid-spine syndrome (RSMD1)","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2016-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76018987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute pancreatitis complicated with periappendicitis.","authors":"Hsiao-Chung Hsia,&nbsp;Lie-Kai Shoung,&nbsp;Mei-Ling Chen,&nbsp;David W Wong","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Colonic complications of severe acute pancreatitis are quite uncommon and always occur in the transverse colon and splenic flexure. Here we report the case of a 47-year-old male with mild acute pancreatitis (Ranson's score 1) who suffered from acute right lower quadrant pain during hospitalization. After conservative treatment failed, he underwent open appendectomy under the impression of acute appendicitis. However, the pathology revealed only periappendicitis. Small bowel ileus was noted on plain film of the abdomen and a high ascitic lipase level was found during operation suggesting that the periappendicitis resulted from the spreading of the pancreatic inflammatory exudate via the small bowel mesentery route. This report suggests that although rare, periappendiceal involvement mimicking acute appendicitis remains possible in even mild acute pancreatitis.</p>","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22289850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Limited feasibility of routinely analyzing fetal cells from maternal blood by using magnetic activated cell sorting and polymerase chain reaction for prenatal diagnosis.","authors":"Sheng-Kai Lin,&nbsp;Esther Shih-Chu Ho,&nbsp;Yune-Tin Hsieh,&nbsp;Fong-Chiu Lo,&nbsp;Huei-Yue Lai,&nbsp;Ming-Huei Chen","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>To assess the feasibility of analyzing fetal cells from maternal circulation by using magnetic activated cell sorting (MACS) and polymerase chain reaction (PCR) for prenatal diagnosis.</p><p><strong>Methods: </strong>Thirty-one high-risk (either advanced maternal age or abnormal serum Down screening) pregnant women (14-22 weeks) were enrolled. Twenty ml of venous blood from each woman after amniocentesis were pretreated with density gradient centrifugation and sorted by MACS with monoclonal antibodies: anti-CD71 (n = 26) or anti-GPA (n = 5). Nested PCR with Y-specific probes--Y1.5-Y1.8 (n = 10) and Amelogenin (n = 21) were then applied to the sorted nucleated red blood cells (NRBCs) for fetal sex determination. These results were compared with cytogenetic data. To assess the sensitivity of PCR, different proportions of known male and female cultured amniocytes were mixed and amplified for gender identification.</p><p><strong>Results: </strong>Karyotypes were normal in all fetuses (18 females and 13 males). The proportions of NRBCs (in total cells) sorted by MACS--anti-GPA or anti-CD71 were 50% (2000 +/- 1500) and 85% (350 +/- 280), respectively. Accuracies of sex determination by PCR-Amelogenin or Y1.5-Y1.8 were 76.2% (16/21) and 50% (5/10), respectively. Three cases resulted in PCR failure. Assay of nested PCR inferred that after cell sorting, existence of at least 20% of male fetal cells mixed in maternal blood circulation was required for prenatal diagnosis under current methodology.</p><p><strong>Conclusions: </strong>We confirmed the existence of fetal NRBCs in maternal blood during pregnancy. The low accuracy of sex determination (76.2%) may be attributed to contamination of either maternal NRBCs or non-NRBCs. No conclusive data, however, so far demonstrates the ideal marker to identify the origin of NRBCs. Without specific fetal cell marker and more sophisticated fetal cell analysis methodologies, in our experience, the feasibility of routinely analyzing fetal cells from maternal blood for prenatal diagnosis is limited.</p>","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22289846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genistein and tyrphostin AG 556 block the action potential shortening in septic shock.","authors":"Chern-En Chiang,&nbsp;Hsiang-Ning Luk,&nbsp;Tsui-Min Wang,&nbsp;Joen-Rong Sheu,&nbsp;Philip Yu-An Ding","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>We have previously shown that an increase in NO activity activated ATP-sensitive potassium channel (K(ATP)) and shortened action potential duration (APD) in an endotoxic shock model. Because the increase in NO production and the decrease of APD appear to be downstream late events in endotoxic shock, we hypothesized that a common signaling pathway might mediate these effects.</p><p><strong>Methods: </strong>Using a guinea pig model of endotoxic shock, we investigated the effect of genistein and tyrphostin AG 556 on the cardiac action potential. Adult Hartley guinea pigs (300 to 450 gm) were randomized into 2 treatment parts. In the chronic treatment part, guinea pigs were randomized to receive daily subcutaneous injection of one of the five agents: saline, genistein, tyrphostin AG 556, daidzein, and vehicle for 10 days. In the acute treatment part, these agents were administered by intraperitoneal injection 1 hour before endotoxic shock. The animals were then anesthetized and mechanically ventilated, and underwent 6-hour endotoxic shock or sham experiment.</p><p><strong>Results: </strong>In the chronic treatment part, the plasma nitrate concentration, myocardial guanosine 3',5'-cyclic monophosphate (cGMP) content, and APD at 90% repolarization (APD90) of papillary muscle showed no difference in the five groups before endotoxic shock. After 6-hour endotoxic shock, the elevation of plasma nitrate concentration and myocardial cGMP content was found significant in the control, the daidzein, and the vehicle groups, but was blunted in the genistein and the tyrphostin groups. The shortening of APD90 of papillary muscle was also significant in the control, the daidzein, and the vehicle groups, but blunted in the genistein and tyrphostin groups. There were similar findings in the acute treatment part, except the weaker effect of genistein and tyrphostin.</p><p><strong>Conclusions: </strong>Genistein and tyrphostin AG 556, either administered chronically or acutely, significantly attenuate the cardiac APD shortening in endotoxic shock, presumably through the decrease in the plasma nitrate and the cardiac cGMP production. It is suggested that tyrosine kinase signaling plays an important role in the modulation of APD in endotoxic shock.</p>","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22289400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aggressive natural killer cell lymphoma/leukemia.","authors":"Po-Shing Lee,&nbsp;Wei-Shou Hwang","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>We report a case of aggressive natural killer (NK) cell lymphoma/leukemia in a 70-year-old woman presenting with fever, hepatosplenomegaly, retroperitoneal lymphadenopathy, and elevated serum CA19-9. The patient died 4 days after diagnosis. Neoplastic NK cells were identified in the blood and retroperitoneal lymph node biopsy. Their phenotypes were confirmed by extensive flow cytometric and immunohistochemical studies. In situ hybridization for Epstein-Barr virus (EBV)-associated RNA (EBER) was positive. Various forms of NK cell neoplasm were reviewed and discussed.</p>","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22289851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Non-steroidal anti-inflammatory drug and antacid co-prescription in Taiwan: analysis of national insurance claims.","authors":"Tzeng-Ji Chen,&nbsp;Jui-Yao Liu,&nbsp;Shinn-Jang Hwang","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Antacids were usually co-prescribed with non-steroidal anti-inflammatory drugs (NSAIDs), although no broad evidences were available as to the effects of antacids in preventing NSAID-associated gastropathy. We performed a survey of national insurance claims for outpatient services in Taiwan to determine the extent of this co-therapy nationwide.</p><p><strong>Methods: </strong>The National Health Insurance Research Database supplied the sampling datasets for analysis. They represented 0.2% of the entire claims for outpatient medical services in 1999. Co-prescribing was assessed as NSAIDs and antacids on the same prescription. The selection and grouping of NSAIDs followed the guidelines of the Anatomical Therapeutic Chemical (ATC) Classification System recommended by the World Health Organization. Only the oral drugs prescribed on regular visits were taken into account.</p><p><strong>Results: </strong>In totally 425,442 prescriptions with 1,825,604 items of drugs, non-aspirin NSAIDs were present in 108,818 (25.6%) prescriptions and antacids in 235,252 (55.3%) prescriptions respectively. Furthermore, antacids were present in 71.3% of prescriptions that contained NSAIDs and in 49.8% of prescriptions that did not contain NSAIDs (p < 0.001). Significant association of NSAIDs and antacids existed in different specialties of prescribing physicians, but the co-prescription rate (antacids in NSAIDs prescriptions) varied from the highest of 92.8% in the neurosurgery to the lowest of 49.8% in the pediatrics. Significant association of NSAIDs and antacids also existed at different levels of health care facilities, where the co-prescription rates were 80.9% at medical centers, 83.5% at regional hospitals, 87.4% at local hospitals, and 66.6% at primary care units. The subgroup of oxicams was more frequently co-prescribed with antacids than other subgroups (odds ratio = 1.51, p = 0.001).</p><p><strong>Conclusions: </strong>Concomitant prescription of oral non-aspirin NSAIDs and antacids was indeed a popular practice in Taiwan. Beside their effects in alleviating the NSAID-associated dyspepsia, the role of antacids in preventing NSAID-associated peptic ulcers or in masking the warning symptoms of these ulcers demands further evaluation.</p>","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22289845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"In vitro growth inhibition by indomethacin on human oral squamous cell carcinoma lines synergistically suppressed by all-trans retinoic acid correlating to apoptosis.","authors":"Cheng-Chieh Yang,&nbsp;Shi-Fen Tu,&nbsp;Cheng-Hsien Wu,&nbsp;Richard Che-Shoa Chang,&nbsp;Shou-Yen Kao","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Indomethacin, an NSAID capable of inhibiting the effect of both cyclooxygenase and lipooxygenase, has been reported to repress the growth of breast cancer, skin cancer and head & neck cancer, etc. Inhibition in the some cell lines of oral squamous cell carcinoma (OSCC) has also been reported. The purpose of this study was primarily to explore the cellular response of human OSCC lines after indomethacin or retinoic acid (RA) treatment and its correlation to apoptosis phenomenon.</p><p><strong>Methods: </strong>Five human OSCC cell lines--KB, SCC15, SCC25, OEC-M1 and OC2--were used for this in vitro study. By direct cell number counting, the cellular response was observed under incremental indomethacin concentrations of 50 microM, 100 microM, 200 microM and 400 microM, in order to select the most appropriate concentration for further study. Then 200 microM indomethacin and all-trans RA at 1 microM were used in the 2nd experiment to explore the intensity of their inhibitory effects individually and potential synergistic inhibition when exerted together. While in the 3rd part, TdT-mediated-dUTP nick-end labeling (TUNEL) method was used for in situ apoptosis assay to see if the apoptosis rate varied with these two agents.</p><p><strong>Results: </strong>All 5 cell lines constantly showed growth suppression with positive dosage effect of indomethacin. Synergistic inhibition by combined treatment of indomethacin and RA was seen in RA responsive lines of SCC15 and SCC25, whereas other RA-resistant clones showed no synergism of this combined treatment. The in situ detection of apoptosis by TUNEL assay revealed a significantly higher ratio of apoptotic cells in the indomethacin/RA treated SCC15 and SCC25 than in controls.</p><p><strong>Conclusions: </strong>The study provides the value of further exploration on the mechanism of how indomethacin inhibiting cancer cell growth and how RA-sensitive OSCC cell lines are synergistically suppressed by conjoint treatment of RA and indomethacin. This study also highlights the value to see how the apoptotic pathway responds differently to the indomethacin/RA treatment.</p>","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22289847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Segmental pancreatectomy for benign tumor of the pancreas.","authors":"Cheng-Hsi Su,&nbsp;Yi-Ming Shyr,&nbsp;Wing-Yiu Lui,&nbsp;Fang-Ku P'eng","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Enucleation of pancreatic tumor has the potential risk for damage of the main pancreatic duct. Benign tumors located in the neck or body of the pancreas are usually removed by left (spleno-) pancreatectomy or pancreatoduodenectomy. Standard pancreatic resection may result in serious loss of normal pancreatic parenchyme and impairment of pancreatic function. The aim of this study is to evaluate the results of segmental pancreatectomy, a limited resection of the midportion of the pancreas, in patients with benign tumor of the pancreas.</p><p><strong>Methods: </strong>Four patients with benign tumor over pancreatic neck or body were treated with segmental pancreatectomy after pathological confirmation by frozen section. The proximal stump was closed and distal stump was anastomosed with a Roux-en-Y pancreaticojejunostomy. Clinical evaluation, routine blood sugar, stool fat examination and abdominal ultrasonography were performed for their follow up.</p><p><strong>Results: </strong>Segmental pancreatectomy was satisfactory in these four patients. The pathologic examinations revealed serous cystadenoma. No mortality was noted. Minor pancreatic fistula was found in three of them and was treated conservatively. No obvious exocrine insufficiency was noticed. One patient had diabetes mellitus before operation, which was persisted postoperatively.</p><p><strong>Conclusions: </strong>Segmental pancreatectomy is a safe and effective alternative to major pancreatic resection in selected patients with benign tumor of the pancreas. This procedure has a surgical risk similar to that of the standard operation, but preserves more pancreatic tissues, which may prevent pancreatic function impairment.</p>","PeriodicalId":24073,"journal":{"name":"Zhonghua yi xue za zhi = Chinese medical journal; Free China ed","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"22289848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}