Gastric interposition following transhiatal esophagectomy: CT evaluation

Transhiatal esophagectomy without thoracotomy (THE) but with gastric interposition results in less morbidity and mortality than standard transpleural esophagectomy with thoracotomy. Barium examination has been the primary radiographic study following THE for detecting postoperative complications. We reviewed computed tomography (CT) scans of 21 patients who had undergone THE and correlated CT appearance with clinical status and with findings of the barium studies. Local mediastinal recurrent neoplasm was detected by CT in seven patients; barium study within 2 weeks of the CT scan failed to detect tumor recurrence in three of these patients. A mediastinal abscess well delineated by CT was percutaneously drained under CT guidance. Water-soluble contrast medium/barium study is preferable for the evaluation of certain postoperative complications such as anastomotic leak. However, CT is the modality of choice for detecting locally recurrent neoplasm and distant metastases following THE and may also be helpful in patients with postoperative mediastinal abscess. Normal mediastinal CT anatomy after esophagectomy is reviewed in order to warn against pitfalls in scan interpretation.     

Mutation screening of the BTK gene in 56 families with X-linked agammaglobulinemia (XLA): 47 unique mutations without correlation to clinical course

OBJECTIVES: To determine the utility of single-stranded conformation polymorphism (SSCP) analysis for mutation screening in the BTK (Bruton's tyrosine kinase) gene, we investigated 56 X-linked agammaglobulinemia (XLA) families. To obtain genotype/ phenotype correlations, predicted protein aberrations were correlated with the clinical course of the disease. PATIENTS: This study included 56 patients with XLA, with or without a positive family history, who were diagnosed on the basis of their clinical features, low peripheral B-cell count, and low immunoglobulin levels. Ten patients with isolated hypogammaglobulinemia and 50 healthy males served as controls. METHODS: SSCP analysis was performed for the entire BTK gene, including the exon-intron boundaries and the promoter region. Structural implications of the missense mutations were investigated by molecular modeling, and the functional consequences of some mutations also were evaluated by in vitro kinase assays and Western blot analysis. RESULTS: We report the largest series of patients with XLA to date. All but 5 of the 56 index patients with XLA screened with SSCP analysis showed BTK gene abnormalities, and in 2 of the 5 SSCP-negative patients, no BTK protein was found by Western blot analysis. There were 51 mutations, including 37 novel ones, distributed across the entire gene. This report contains the first promoter mutation as well as 14 novel missense mutations with the first ones described for the Tec homology domain and the glycine-rich motif in the SH1 domain. Each index patient had a different mutation, except for four mutations, each in two unrelated individuals. This result supports the strong tendency for private mutations in this disease. No mutations were found in the controls. CONCLUSIONS: Our results demonstrate that molecular genetic testing by SSCP analysis provides an accurate tool for the definitive diagnosis of XLA and the discrimination of borderline cases, such as certain hypogammaglobulinemia or common variable immunodeficiency patients with overlapping clinical features. Genotype/ phenotype correlations are not currently possible, making prediction of the clinical course based on molecular genetic data infeasible.     

Dual effect of prostaglandin E2 on normal airways smooth muscle in vivo

Inhalation of prostaglandin E₂ by normal subjects caused initial bronchoconstriction followed by the predominant effect of bronchodilatation, which was maximal by 15 minutes. The degree of bronchoconstriction seen in the early phase appeared to be related to the initial tone of airways smooth muscle and was exaggerated after bronchodilatation with a large dose of ipratropium bromide. Bronchoconstriction induced by prostaglandin E₂ is a direct effect on muscle and not secondary to a vagal reflex initiated by airways irritant receptors.     

Central nervous system anoxic-ischemic insult in children due to near- drowning

An analysis of admission and follow-up CT examinations of the brains of 17 children who had nearly drowned (15 of whom were comatose and two of whom were conscious on admission) indicated that a normal initial CT scan is common in the majority of comatose patients, a severe neurologic outcome may develop in spite of a normal initial CT examination, and abnormal initial or follow-up CT findings indicate the strong but not inevitable probability of a severe neurologic outcome. We conclude that when there is no head trauma, an initial CT examination is not necessary. Prediction of the clinical outcome cannot be made on the basis of the initial CT findings.     

Involvement of Ser103 of the Rh polypeptides in G epitope formation

BACKGROUND: Almost all red cells that carry D and/or C antigens also express the G antigen (Rh12). A study was conducted on the molecular background of the G epitope. STUDY DESIGN AND METHODS: Two unrelated donors with the rare ccDEe, G- phenotype and one donor with the ccEe, G+ phenotype were studied. Genomic DNA and cDNA of these donors were studied with polymerase chain reaction, Southern blot, and sequence analysis, with special focus on exon 2, because it is only in this exon that there are supposed to be similarities between RHD and the RHC allele, but not between RHD and the RHc allele. RESULTS: In both ccDEe, G- donors, a nucleotide substitution was found in exon 2 of RHD; T307 was replaced by C307, which predicted a Ser->Pro substitution at amino acid position 103 of the D polypeptide. The ccEe, G+ donor carried the complete exon 2 of RHD. Moreover, despite the absence of all known D epitopes, this donor also carried RHD characteristics in exons 1 to 3 and exon 9 and further downstream. CONCLUSION: Ser103, encoded by exon 2 of the RH genes, is involved in G epitope formation.     

肾性高血压大鼠血压升高与主动脉肥厚及牛磺酸的干预

目的:观察肾血管性高血压大鼠的血压变化、主动脉肥厚情况及牛磺酸干预后血压及血管重构的变化。方法:实验于2005-06/12在咸宁学院医学院生理教研室完成。①选用60只Wistar大鼠,采用抽签法随机分为3组(n=20),即假手术组、高血压模型组和牛磺酸治疗组。后两组制备两肾一夹肾血管性高血压大鼠模型;假手术组大鼠除左肾动脉不放置银夹外,余下操作与模型组相同。②假手术组分笼喂养至第12周末;牛磺酸治疗组分笼喂养至第4周末,于术后第5周开始给予牛磺酸50mg/(kg·d)灌胃,1次/d,连续给药8周;高血压模型组以相同容积的蒸馏水替代牛磺酸灌胃给药,余同牛磺酸治疗组。③给药8周后,测量各组大鼠体质量和血压后处死,分离其胸主动脉以用于检测主动脉质量/体质量比值、胸主动脉的血管紧张素Ⅱ含量,应用石蜡切片的彩色图像分析技术测量胸主动脉中膜厚度及中膜截面积。结果:60只大鼠全部进入结果分析。①给药8周后,高血压模型组大鼠的血压、主动脉质量/体质量比值、胸主动脉的血管紧张素Ⅱ含量、胸主动脉中膜厚度及中膜截面积均显著高于假手术组(P<0.01)。②给药8周后,牛磺酸治疗组大鼠的血压、主动脉质量/体质量比值、胸主动脉的血管紧张素Ⅱ含量、胸主动脉中膜厚度及中膜截面积均显著低于高血压模型组(P<0.01)。结论:长疗程牛磺酸干预在抗高血压的同时具有抑制主动脉肥厚形成等血管重塑过程的作用。     

The VS and V blood group polymorphisms in Africans: a serologic and molecular analysis

BACKGROUND: VS and V are common red cell antigens in persons of African origin. The molecular background of these Rh system antigens is poorly understood. STUDY DESIGN AND METHODS: Red cells from 100 black South Africans and 43 black persons from Amsterdam, the Netherlands, were typed serologically for various Rh system antigens. Allele-specific polymerase chain reaction and sequencing of polymerase chain reaction products were used to analyze C733G (Leu245Val) and G1006T (Gly336Cys) polymorphisms in exons 5 and 7 of RHCE and the presence of a D-CE hybrid exon 3. RESULTS: The respective frequencies of all VS+ and of VS+ V-(r's) phenotypes were 43 percent and 9 percent in the South Africans and 49 percent and 12 percent in the Dutch donors. All VS+ donors had G733 (Val245), but six with G733 were VS- (4 V+w, 2 V-). The four VS- V+w donors with G733 appeared to have a CE-D hybrid exon 5. T1006 (Cys336) was present in 12 percent and 16 percent of donors from the two populations. With only a few exceptions, T1006, a D-CE hybrid exon 3, and a C410T (Ala137Val) substitution were associated with a VS+ V-phenotype ((C)ces or r's haplotype). Two VS+ V-individuals, with the probable genotype, (C)ces/(C)ces), were homozygous for G733 and for T1006. CONCLUSIONS: It is likely that anti-VS and anti-V recognize the conformational changes created by Val245, but that anti-V is sensitive to additional conformational changes created by Cys336.     

溃疡性结肠炎中西医治疗进展

１概况溃疡性结肠炎（以下简称溃结）是直肠与结肠部位一种原因未明与自身免疫有关的炎症性肠病,是以腹泻、粘液脓血便、腹痛及里急后重为主要症状,以结肠粘膜慢性炎症和溃疡形成为病理特点的消化道常见病和疑难病,严重者可有发热、关节炎、虹膜炎、皮肤结节性红斑等肠...     

槐胺碱抗心律失常作用

槐胺碱(sophoramine)是从豆科槐属植物苦豆子Sophora alpicuroides Lin.中提取的一种淡黄色生物碱。本文主要研究其抗心律失常的作用。