中晚期重型乙型肝炎血浆置换与内科治疗生存率的比较

观察血浆置换、内科治疗对中晚期重型乙型病毒性肝炎生存率的影响,探索中晚期重型肝炎的治疗方法。方法观察在内科治疗基础上给予血浆置换患者的肝功能、并发症及疾病的转归,与同期仅予内科综合治疗的患者相比较,对相应的临床资料进行统计学分析,从而了解两种治疗方法的疗效。结果血浆置换组36例,18例好转、18例死亡,内科治疗组32例,13例好转、19例死亡,两组之间患者生存情况无统计学差异;肝功能指标(ALT、AST、SB、ALB、TC、ChE和凝血酶原时间(PT)也无统计学差异。结论与内科治疗相比较,在其基础上给予血浆置换并不能提高中晚期重型病毒性肝炎生存率;中晚期重型肝炎患者的预后决定于其肝功能衰竭的程度。     

δ-aminolevulinic acid dehydrase (porphobilinogen synthase) in two families with inherited enzyme deficiency

The inheritance of a deficient delta-aminolevulinic acid dehydrase (ALA-D; synonym: porphobilinogen synthase; EC 4.2.1.24) was studied in blood samples of two families over three generations. The propositus in each family was a young male acute hepatic porphyria patient with an almost complete ALA-D deficiency in the homozygous state (ALA-D activity less than 2% of controls). Heterozygotes are clinically non-affected (mean ALA-D 36% of controls). The mode of transmission could be traced by enzyme activity and electrophoretic polymorphism studies. Heterozygotes are detected by the demonstration of enzyme activity in the gel. The notation D was used for the gene expressing the defective enzyme. The "phenotype" D-1 was observed in six, the "phenotype" D-2 in three of all heterozygotes studied. These results are compatible with a single normal allele in heterozygotes responsible for enzyme activity. Quantitative assays and the segregation pattern in both families suggest a 3-allele-system for the inheritance of ALA-D deficiency.     

Mucopolysaccharidosis type IIIC (Sanfilippo): early clinical presentation in a large Turkish pedigree

Two sisters from a large consanguineous Turkish family with Sanfilippo C disease presented within the first 2 years of life. They had coarse facial features, organomegaly and discrete radiological signs of dysostosis multiplex. Urinary glycosaminoglycan excretion was elevated, heparan sulfate being the major component. The uptake of radioactive sulfate into fibroblasts from both patients was abnormal. A deficiency of acetylCoA: alpha-glucosamine transferase was demonstrated in fibroblasts from one patient. To our knowledge, this is the first report of the disease presenting in early infancy.     

In vivo molecular targeted radiotherapy

Unsealed radionuclides have been in clinical therapeutic use for well over half a century. Following the early inappropriate clinical administrations of radium salts in the early 20th century, the first real clinical benefits became evident with the use of (131)I-sodium iodide for the treatment of hypothyroidism and differentiated thyroid carcinoma and (32)P-sodium phosphate for the treatment of polycythaemia vera. In recent years the use of bone seeking agents (89)Sr, (153)Sm and (186)Re for the palliation of bone pain have become widespread and considerable progress has been evident with the use of (131)I-MIBG and (90)Y-somatostatin receptor binding agents. Although the use of monoclonal antibody based therapeutic products has been slow to evolve, the start of the 21st century has witnessed the first licensed therapeutic antibody conjugates based on (90)Y and (131)I for the treatment of non-Hodgkin's lymphoma. The future clinical utility of this form of therapy will depend upon the development of radiopharmaceutical conjugates capable of selective binding to molecular targets. The availability of some therapeutic radionuclides such as (188)Re produced from the tungsten generator system which can produce activity as required over many months, may make this type of therapy more widely available in some remote and developing countries.Future products will involve cytotoxic radionuclides with appropriate potency, but with physical characteristics that will enable the administration of therapeutic doses with the minimal need for patient isolation. Further developments are likely to involve molecular constructs such as aptamers arising from new developments in biotechnology.Patient trials are still underway and are now examining new methods of administration, dose fractionation and the clinical introduction of alpha emitting radiopharmaceutical conjugates. This review outlines the history, development and future potential of these forms of therapy.     

Three novel PROC gene lesions causing protein C deficiency

Hallam PJ, Mannucci P, Tripodi A, Bevan D, Laursen B, Tengborn L, Wacey A, Cooper DN. Three novel PROC gene lesions causing protein C deficiency. Clin Genet 1998: 54: 231–233. 0 Munksgaard, 1998
Missense mutations. three of them novel (Am210→Val, Asn248→ Ile, Ah355→Val), were found in the protein c ( PROC ) genes of 7 patients with inherited protein C deficiency associated with venous thrombosis. Comparison with the phenotypic effects of mutations in the analogous residues of factor IX causing hdernophilia B and the use of molecular modelling has provided explanations as to how these lesions might alter either the structure, function or secretion of the protein C molecules encoded.     

Familial insertional translocation of a portion of 3q into 11q resulting in duplication and deletion of region 3q22.1→q24 in different offspring

The use of elongated prophase and prometaphase chromosome preparations has allowed detection of an insertion of a small segment of 3q into 11q in a kindred with 4 balanced carriers and 8 unbalanced offspring. Those with partial 3q deletion have a true multiple congenital anomalies/mental retardation (MCA/MR) syndrome with an appearance suggestive of the Schwartz-Jampel syndrome.     

Epidemiology of adverse drug reactions in intensive care units. A multicentre prospective survey

Summary Clinically relevant events possibly attributable to drug exposure have been monitored prospectively over a period of six months in 27 general intensive care units. Fifty-four events attributed to drugs were reported in 51 patients during their stay in hospital, corresponding to an overall incidence of 1.35%. The behaviour of the physicians following attribution of the events to a prescribed drug is analyzed and discussed in detail with respect to its relationship to the quality and severity of the reaction, and the classes of drugs. Twenty-four of the 4537 monitored admissions during the six months were due to life-threatening emergencies linked to the administration of drugs (14) and radio-contrast media (10) (overall incidence 0.5%). While the clinical burden attributable to adverse drug reactions in Intensive Care Units appears to be relatively small, the analysis shows that there is ample room for a greater reduction in their incidence. Coordinators: Drs M. L. Farina and G. Tognoni, Istituto di Ricerche Farmacologiche Mario Negri, Milan; Dr F. Procaccio, Neurosurgical ICU, Ospedale Ca' Granda, Niguarda, Milan.Investigators: Drs G. Barusco, Rovigo; F. Bassi, Milan; L. Bianchetti, Torino; E. Carchietti, Udine; G. Chilloni, Reggio Emilia; G. Costantini, Savigliano (CN); P. Ferrero, Aosta; E. Geat, Trento; F. Gorgerino, Torino; A. Lusini, Empoli (FI); G. Mantovani, Ferrara; S. Marchi, Bologna; P. Marcovigi, Forli; G. Marraro, Merate (CO); F. Merlo, Vicenza; E. Pagni, Bagno a Ripoli (FI); R. Pellegrino, Cuneo; C. Peruselli, Milan; A. Piovesano, Pordenone; R. Rinaldo, Cremona; R. Ruggerini, Piacenza; S. Sammartino, Torino; A. Sartore, Cittadella (PD); A. Scaglioli, Carpi (MO); G. Scopa, Terni; G. Zeffiro, Treviso; P. Zuccoli, Parma     

"银屑灵"治疗银屑病351例临床观察

本文报道用银屑灵治疗银屑病351例,疗程60天,对有效病例延长疗程可提高治愈率.临床痊愈率32.2%,总有效率73.8%,点滴状银屑病疗效最好.随访2年,复发率19.5%.仅少数病例有轻微胃肠道反应,但有6例治疗前发现肝功能异常,作者认为可能属银屑病的肝脏损害,经治疗后转正常.并对治疗机理作了初步探讨.     

中医药对糖尿病足溃疡相关细胞因子调控作用的研究进展

糖尿病足溃疡是糖尿病严重的破坏性并发症,致残率及致死率逐年增高,严重威胁人类身心健康。中医药治疗糖尿病足溃疡,注重辨证论治与整体观念相结合,不仅能改善中医证候,更能在加速创面愈合的同时减少创面的复发,一定程度上延缓了糖尿病足溃疡的进一步恶化,降低其致残率及致死率。现代研究发现,糖尿病足溃疡难以愈合与各种细胞因子如炎症因子、生长因子、趋化因子等分布异常密切相关;随着现代医学对中医药研究的不断深入,中药单体及复方调控细胞因子治疗糖尿病足溃疡已成为研究热点,该文通过对目前国内外研究现状进行归纳,得出以下总结：(1)芝麻酚、栀子苷、当归补血汤、紫朱软膏等调节肿瘤坏死因子-α(TNF-α)、白细胞介素（IL）-1、IL-6、IL-10等炎症因子,抑制创面炎症。(2)白芷、丹酚酸B、四效散、壮药拔毒生肌膏等调节血管内皮生长因子（VEGF）、血小板衍生生长因子（PDGF）、转化生长因子（TGF）、表皮生长因子（EGF）等生长因子,促进创面胶原沉积及血管新生。(3)芍药苷、隐丹参酮、蜂毒、回阳生肌汤等调节CXC趋化因子配体（CXCL）1、CXCL2、CC趋化因子配体（CCL）2、CCL3、基质细胞衍生...