Unexplained diarrhoea and failure to thrive in 2 siblings with unusual facies and abnormal scalp hair shafts: a new syndrome

A family is described in which 2 siblings born to healthy parents presented with abnormal facies, persistent diarrhoea, and early death. Exhaustive pathological and biochemical investigations failed to find a cause. The scalp hair of both babies had an abnormal amino-acid composition, and presented an appearance that was unique on scanning electron microscopical examination; this fact and the clinical picture probably represents a new syndrome.     

Design and Objectives of the Evaluation of Oral Xemilofiban in Controlling Thrombotic Events (EXCITE) Study

Clinical trials have demonstrated the efficacy of glycoprotein (GP) IIb/IIIa antagonists in preventing the thrombotic end points of death, myocardial infarction, and urgent revascularization when they are administered at the time of percutaneous coronary revascularization (PTCR). It has been postulated that prolongation of receptor blockade beyond acute intervention would extend the clinical benefit of these agents. The Evaluation of Oral Xemilofiban in Controlling Thrombotic Events (EXCITE) study was a multicenter, international, randomized placebo-controlled trial of the oral GP IIb/IIIa antagonist Xemilofiban administered prior to and after PTCR. The study was designed to assess the efficacy and safety of continuing oral xemilofiban for 6 months to prevent these primary thrombotic end points. More than 7,200 patients were randomized in 29 countries to receive placebo or one of two doses of xemilofiban. Stenting was performed at the discretion of the operator. All patients received aspirin and periprocedural heparin; all stented patients received continuous xemilofiban, or ticlopidine for 2–4 weeks followed by xemilofiban-placebo. Most patients were also evaluated 1 month after conclusion of the study drug treatment. Clinical data from up to 6 months of drug treatment and 1 month posttreatment were used to evaluate the acute and long-term efficacy and safety of xemilofiban. Secondary end points included the need for any revascularization, repeat hospitalization for unstable angina, and nonhemorrhagic stroke. The cumulative incidence of bleeding events and effects of xemilofiban in stented and nonstented patients were evaluated. The efficacy of continuing xemilofiban and aspirin therapy as the sole antithrombotic medications following stent deployment was assessed against a ticlopidine and aspirin control group. The incremental clinical benefit of long-term receptor blockade over acute receptor antagonism was evaluated.     

MODERN LINES OF MANAGEMENT OF ECTOPIC PREGNANCY

The recent increase in the incidence of ectopic pregnancies was associated with rapid improvement in the diagnostic and therapeutic techniques. Quantitative serum B-HCG radioimmunoassay and high resolution vaginal ultrasonography have facilitated early diagnosis of ectopic pregnancy allowing a more conservative approach to patient management. Different conservative surgical and medical lines of management recently developed were associated with and increased chance of subsequent intrauterine pregnancy with no increase in the incidence of repeat ectopic pregnancy. Outpatient systemic medical treatment seems to be a preferred alternative to conservative surgery. In selected cases, it is associated with a lower complication rate and promising result for fertility. (Br J Clin Pract 1996; 50(7) : 376-380.)     

癌症化学预防的分子靶标

乙肝病毒和人乳头状瘤病毒分别是肝细胞癌和子宫颈癌的风险因素,针对这2种病毒感染的疫苗已在临床上成功用于癌症化学预防。分子靶向药物能够预防乳腺癌（雷洛昔芬与他莫昔芬）、大肠腺瘤（塞来昔布）和前列腺癌（非那雄胺）。然而,化学预防广泛应用于临床还不现实。分子靶标的深入研究将扩展化学预防的范围并使其个性化。     

Granulocyte colony-stimulating factor crosses the placenta and stimulates fetal rat granulopoiesis

We studied the effect of recombinant human granulocyte colony- stimulating factor (rhG-CSF) administration to pregnant rats upon fetal and neonatal myelopoiesis. Pregnant rats were treated with rhG-CSF twice daily for 2, 4, and 6 days before parturition. rhG-CSF crossed the placenta and reached peak fetal serum concentrations 4 hours after administration. Peak fetal serum levels were 1,000-fold lower than levels detected in the dam. Hematopoietic effects of rhG-CSF were assessed by cytologic analysis of the newborn blood, spleen, bone marrow, thymus, and liver. White blood cell counts were increased twofold to fourfold in newborns. This increase was due to circulating numbers of polymorphonuclear cells (PMN). rhG-CSF induced a myeloid hyperplasia in the newborn marrow consisting of immature and mature myeloid cells in the day-2 and day-4 treated pups. Bone marrow of pups treated for 6 days contained mostly hyper-segmented PMN with little or no increase in myeloid precursors. An increase in the number of postmitotic (PMN, bands, and metamyelocytes) and mitotic (promyeloblasts, myeloblasts, and metamyeloblasts) myeloid cells in the spleen of neonates was observed. No change was detected in splenic lymphocytes or monocytes. No effect of rhG-CSF was noted in the newborn liver or thymus. These results demonstrate that maternally administered rhG-CSF crosses the placenta and specifically induces bone marrow and spleen myelopoiesis in the fetus and neonate. The significant myelopoietic effects of rhG-CSF at low concentrations in the fetus suggest an exquisite degree of developmental sensitivity to this cytokine and may provide enhanced defense mechanisms to the neonate.     

神经外科中高渗盐注射液应用研究进展

控制脑水肿和颅内压(ICP)升高是神经外科围手术期治疗的重要组成部分.颅脑创伤、动脉梗塞、静脉高压/梗塞、大脑内出血、蛛网膜下腔出血、肿瘤和术后脑组织水肿的治疗过程中ICP的控制都是决定患者预后的关键因素.虽然利用渗透压脱水药物是控制ICP的最基础的工具,但却缺乏前瞻性研究以指导其运用,高渗盐被认为是甘露醇的替代物,早期的数据表明每种药的用药指征最终取决于ICP的病因.在这篇综述中,我们总结了有关高渗盐(HS)治疗颅内高压的相关数据,以及这些数据和我们有关HS的经验是如何指导目前的ICP治疗的.