The Long QT Syndrome with Impaired Atrioventricular Conduction: A Malignant Variant in Infants

Distal Conduction Disease in the Long QT Syndrome. Introduction : The long QT syndrome (LQTS) is occasionally complicated by impaired AV conduction, mostly 2:1 AV block. This form of LQTS can manifest before birth or during neonatal life, and it is more sporadic than familial. It is usually an isolated disorder, although it can be accompanied by a variety of cardiovascular and other anomalies. In spite of different treatment modes, mortality is high.
Methods and Results : The reported case presented not only with 2:1 AV conduction, but also with Wenckebach episodes with impaired right and left bundle branch conduction, and decremental conduction in the His-Purkinje axis. We also observed sinus pauses and accelerated AV junctional escape beats.
Conclusion : Our findings, and similar observations by others., suggest involvement of the sinus node and the distal conduction system in this form of the LQTS. Several histologic studies have documented abnormalities within the conduction system, including apoptosis. Because of the rare occurrence and poor prognosis of the LQTS with impaired AV conduction, international guidelines for diagnosis and treatment are needed. Development of an internal cardiac defibrillator for this young age group is necessary.     

Clinical and echocardiographic survey of the Ehlers-Danlos syndrome

Cardiac abnormalities such as mitral valve prolapse (MVP) are reported to be common features of the Ehlers Danlos syndrome (EDS), and it has been suggested that the majority of patients with type IV EDS will have cardiac involvement and vascular aneurysms. However, the evidence for valve lesions is inconsistent and often based on early clinical studies using mainly M-mode echo. We studied 33 patients (six male, 27 female; median age 35 yr) with EDS (30 type I, II or III, three type IV) and 30 age- and sex-matched controls. The study assessed skin stretch and joint hypermobility using Beighton and Contompasis scores. Echocardiographic examination included standard two-dimensional views from the parasternal and apical windows, and measurement of the aorta at four sites (annulus, sinotubular junction, arch and abdominal aorta). Echocardiographic abnormalities were found in four patients (12.1%) (one atrial septal aneurysm, one tricuspid prolapse, two MVP) and two controls (6.7%). MVP was found in 6.1% of EDS patients and 7% of controls. Seven patients had previously been diagnosed as having MVP; only two were shown to have true MVP using current criteria. None of those with type IV EDS had any echocardiographic abnormality. No patients with EDS had mean aortic dimensions outside the normal range at any of the points tested. Cardiac symptoms were more frequent amongst the patients than controls (atypical chest pain 48%, P = 0.0001; palpitation 39%, P = 0.001; exertional dyspnoea 30%). A wide range of rheumatological complaints were reported (current arthralgia 75%; recent back pain 72%, P = 0.005; recurrent dislocation 72%). Contrary to earlier published observations, we have not found an increased incidence of cardiac abnormalities in EDS. This syndrome may be relatively more benign, from the cardiac point of view, than was previously thought.      

口服右旋索他洛尔生存研究(SWORD)

标题　ａ）口服左旋－Ｓｏｔａｔｏｌ治疗心肌梗死后左室功能不全患者生存研究（ＳＷＯＲＤ）：试验依据、设计和方法　　　ｂ）右旋－Ｓｏｔａｔｏｌ对亚急性和陈旧性心肌梗死后左室功能不全患者病死率的影响研究作者　ＷａｌｄｏＡＬ,ＣａｍｍＡＪ,ｄｅｒｕｙｔｅｒＨ,等ａ）ＡｍＪＣａｒｄｉｏｌ,１９９５,７５：１０２３～１０２７ｂ）Ｌａｎｃｅｔ,１９９６,３４８：７～１２　　研究疾病：充血性心力衰竭。目的：右旋－Ｓｏｔａｔｏｌ为一种具有专一性钾通道阻滞作用的抗心律失常药物。本研究将对其降低心肌梗死后左室功能不全…     

Transfected leukocyte integrin CD11b/CD18 (Mac-1) mediates phorbol ester-activated, homotypic cell:cell adherence in the K562 cell line

The CD11b/CD18 leukocyte integrin molecule mediates diverse neutrophil adherence-related functions, including cell:cell and cell:extracellular matrix attachments. To study the individual role of this leukocyte integrin in cell adherence in hematopoietic cells, we expressed the CD11b/CD18 complex on the surface of K562 cells, a cell line derived from an individual with chronic myelogenous leukemia in blast crisis. We used an amphotrophic retroviral vector designated LCD18SN, harboring the complete coding sequence for the CD18 subunit, to transfer the CD18 cDNA into K562 cells and select stable cell lines. The CD11b subunit in the expression plasmid pREP4 was transfected into these K562/CD18 cells by electroporation and stable cell clones were selected. These K562 cells possessed RNA and intracellular protein for each subunit, and they expressed the CD11b/CD18 heterodimer on the cell surface. When CD11b/CD18 expressing K562 cells were stimulated with phorbol myristate acetate (50 ng/mL) for 24 to 48 hours, these K562 cells formed dense cell:cell aggregates. This homotypic aggregation required both activation of the CD11b/CD18 complex and the induction of the counter- receptor for CD11b/CD18 on the conjugate cell. This cell line will (1) enable the structure-function relationships between cell activation and homotypic adherence to be assessed, (2) provide the opportunity to identify accessory molecules required for activation of the CD11b/CD18 complex, and (3) facilitate the identification of novel ligands for the CD11b/CD18 complex.     

Arg-Gly-Asp-dependent occupancy of GPIIb/IIIa by applaggin: evidence for internalization and cycling of a platelet integrin

Using indirect immunofluorescence microscopy we examined the distribution and cycling of GPIIb/IIIa after binding to applaggin, a high-affinity Arg-Gly-Asp (RGD)--containing ligand. Resting, unfixed platelets were incubated with applaggin for 30 minutes at 37 degrees C, and bound applaggin was detected by an affinity-purified rabbit anti- applaggin antibody. Examination of intact cells showed a rim pattern for applaggin, consistent with its binding to the platelet surface. Staining of Triton X-100--permeabilized cells showed an intracellular pool of applaggin. Competition of applaggin binding by either AP-2, an anti-GPIIb/IIIa monoclonal antibody (MoAb) that blocks fibrinogen binding, or the synthetic peptide RGDW eliminated both surface and intracellular staining, indicating that applaggin is binding to GPIIb/IIIa in an RGD-dependent manner. Inhibition of platelet activation by PGE1 and theophylline had no effect on the observed staining patterns, indicating that cellular activation is not required for surface binding and subsequent internalization. To evaluate whether occupancy of functional binding sites on GPIIb/IIIa is required for internalization, we used mAb15, an anti-GPIIIa antibody that neither blocks fibrinogen binding nor induces the expression of ligand-induced binding sites on GPIIb/IIIa. In these studies mAb15 was internalized in a manner analogous to both AP-2 and applaggin, showing that occupancy of the RGD binding site is not required to initiate receptor internalization. To estimate the size of the newly internalized pool of applaggin, 125I-applaggin--binding studies were performed. Displacement of bound 125I-applaggin by excess unlabeled applaggin or EDTA showed that at least 17% of bound applaggin was nondisplaceable when binding was performed under conditions permitting membrane flow and internalization. These data indicate that GPIIb/IIIa is internalized in unstimulated platelets independent of cellular activation or occupancy of the functional binding site(s) of GPIIb/IIIa by RGD-containing ligands. Thus, internalization of GPIIb/IIIa may represent a mechanism by which the surface expression of this adhesion receptor is regulated.     

Multifocal pyomyositis: report of two cases

Pyomyositis is a primary infection of the striated muscles. We describe the clinical and imaging features of pyomyositis in two patients, one diabetic and the other immunocompetent. Treatment with incision, drainage and antibiotics was successful and resulted in full recovery. Increased awareness, especially in immuno‐competent patients, should lead to earlier diagnosis, correct treatment and a better outcome.     

Obtaining accurate patient‐specific perioperative anaesthetic costs in orthopaedic anaesthesia

□ The aim of the study was to obtain perioperative patient‐specific costs associated with orthopaedic anaesthesia □ A prospective observational study design was used to determine fixed, semi‐fixed, and variable costs □ The preliminary results of the first 60 patients reports a mean total perioperative anaesthetic cost of £291 (SD: £103.9, median: £278.09, IQR: £219.3–£369.5) □ Fixed and semi‐fixed costs are a major cost component of anaesthesia; variable costs account for only 12 per cent of total cost □ The results of this study will be used in an economic evaluation examining the use of different anaesthetic techniques in orthopaedics     

Chronic daily headache with migrainous features due to papilledema-negative idiopathic intracranial hypertension

Patients with the syndrome of chronic daily headache often report migrainous symptoms and consequently are diagnosed as having a primary headache syndrome. We report two cases of idiopathic intracranial hypertension causing chronic daily headache with migrainous features in the absence of associated papilledema.