Mass casualty incidents: are NHS staff prepared? An audit of one NHS foundation trust.

BACKGROUND: Lack of knowledge of an NHS trust's major incident policies by clinical staff may result in poorly coordinated responses during a mass casualty incident (MCI). AIM: To audit knowledge of the major incident policy by clinical staff working in a central London major acute NHS trust designated to receive casualties on a 24-h basis during a MCI. METHODS: A 12-question proforma was distributed to 307 nursing and medical staff in the hospital, designed to assess their knowledge of the major incident policy. Completed proformas were collected over a 2-month period between December 2006 and February 2007. RESULTS: A reply rate of 34% was obtained, with a reasonable representation from all disciplines ranging from nurses to consultants. Despite only 41% having read the policy in full, 70% knew the correct immediate action to take if informed of major incident activation. 76% knew the correct stand-down procedure. 56% knew the correct reporting point but less than 25% knew that an action card system was utilised. Nurses had significantly (p<0.01) more awareness of the policy than doctors. CONCLUSION: In view of the heightened terrorist threat in London, knowledge of major incident policy is essential. The high percentage of positive responses relating to immediate and stand-down actions reflects the rolling trust-wide MCI education programme and the organisational memory of the trust following several previous MCI in the capital. There is still scope for an improvement in awareness, however, particularly concerning knowledge of action cards, which are now displayed routinely throughout clinical areas and will be incorporated into induction packs.

Treatment of pulmonary hypertension in the general adult intensive care unit: a role for oral sildenafil?

Use of inhaled nitric oxide for treatment of pulmonary hypertension in adult critical illness is limited by its mode of delivery and high costs, prompting evaluation of alternative therapies. We report the use of oral sildenafil in a patient with severe secondary pulmonary hypertension and right ventricular dysfunction. Following reduction in mean pulmonary artery pressure and pulmonary vascular resistance with inhaled nitric oxide, crossover to sildenafil therapy maintained control of pulmonary hypertension, facilitating discontinuation of respiratory and cardiovascular organ support. The relative pulmonary vascular specificity of oral sildenafil, and its low cost, makes it an attractive therapeutic alternative to inhaled nitric oxide, and warrants further study.

Primary culture of human proximal renal tubular epithelial cells.

Sepsis and septic shock are major causes of acute renal failure (ARF). Although hemodynamic factors play a significant role in the pathogenesis of ARF during sepsis, it is now clear that nonhemodynamic factors are also extremely important. The predominant site of tissue injury in sepsis-induced ARF occurs within the proximal renal tubule. In vivo studies of the specific cellular mechanisms underlying renal injury are limited by the marked heterogeneity of the nephron. Establishing primary cultures of human proximal renal tubular epithelial cells (PTEC) provides a well-characterized in vitro model, phenotypically representative of PTEC in vivo. This in vitro system allows for investigation of the cellular mechanisms underlying proximal tubular injury during sepsis, in isolation without additional complicating cardiovascular and neuroendocrine factors.

Bullous dermatoses in end-stage renal failure: porphyria or pseudoporphyria?

Bullous dermatoses (BD) are well recognized in patients with end-stage renal disease (ESRD). It is important to distinguish pseudoporphyria (porphyrin accumulation due to decreased clearance) from true porphyrias, particularly those in which acute neurological attacks may occur. Investigation of the dialysis patient poses practical diagnostic difficulties because urinary porphyrin profiles are not available. We describe a patient on continuous ambulatory peritoneal dialysis (CAPD) with several recognized causative factors for porphyria cutanea tarda (PCT). The patient presented with a blistering photosensitive rash. We highlight the importance of investigating anuric patients with fractionation of both fecal and plasma porphyrins. Plasma porphyrins were grossly elevated (345 nmol/L; reference range, <13), whereas plasma porphyrins in a control group of CAPD patients without blistering rashes were only minimally elevated (mean, 23.9 nmol/L; SD, 11.0; n = 9). Fractionation of fecal porphyrins by high-performance liquid chromatography (HPLC) yielded a pattern typical of PCT. In addition to the contributory factors for PCT that were present, it is possible that porphyrin accumulation secondary to renal failure played a role in the expression of her disease. Patients with ESRD presenting with BD require careful evaluation, including fractionation of fecal porphyrins.

Quinine-induced immune thrombocytopenic purpura followed by hemolytic uremic syndrome.

Immune thrombocytopenic purpura (ITP) mediated by quinine-dependent platelet reactive antibodies is well recognized. More recently there have been a number of reports of quinine-induced hemolytic-uremic syndrome (HUS). We describe a patient with quinine-induced immune thrombocytopenia who subsequently developed HUS after re-exposure to a single dose of this drug. To our knowledge, this is the first such case reported. Multiple quinine-dependent antibodies have been characterized in the patient's serum. Initially, quinine-dependent antibodies were directed solely against the platelet glycoprotein complex GPIb/IX. After rechallenge with quinine, there was broadening of quinine-dependent antibody specificities, which were now also directed against the platelet glycoprotein complexes GPIb/IX and GPIIb/IIIa, endothelial cells, and leukocytes. We have shown quinine-dependent antibody-mediated endothelial cell activation, which supports an immunopathogenic role for quinine-dependent antibodies in the causation of this disease.

Idarubicin, high-dose cytarabine, and etoposide for induction of remission in acute leukemia.

Between 1990 and 1944, 52 newly diagnosed patients with primary (n = 47) or therapy-related (n = 5) acute myeloid leukemia (AML) under the age of 55 years received an induction chemotherapy protocol (designated BF12) consisting of idarubicin ([IDA] 5 mg/m2), high-dose cytarabine ([HD-Ara-C] 2 mg/m2 per 12 hours, 3-hour infusion), and etoposide ([VP-16] 100 mg/m2, 1-hour infusion) on each of 5 consecutive days. Thirty-seven of 51 assessable patients (72.5%), including all five patients with therapy-related AML, attained remission with one cycle. The overall remission rate was 78.4%. Total therapy of AML, with BF12 followed by two courses of consolidation therapy and allogeneic or unpurged autologous bone marrow transplantation (BMT) in first remission, has resulted in actuarial 3-year survival of 49.9% of consecutive unselected patients with newly diagnosed primary AML (minimum follow-up period, 1 year). Twenty-five patients have received BF12 for relapsed acute leukemia, including 13 relapsing after BMT. Five patients died of toxicity and were not assessable for response. Of the remaining 20 patients, five were refractory, two attained partial remissions, and 13 (65%) achieved complete remission (CR). Four of the 13 patients relapsing after BMT died of toxicity, four were refractory, and five of nine assessable patients (56%) attained CR. We conclude that the combination IDA/HD-Ara-C/VP-16 is highly effective in the treatment of newly diagnosed AML and relapsed acute leukemia.

T cell-depleted allogeneic bone marrow transplantation from a partially HLA-mismatched unrelated donor for progressive chronic lymphocytic leukemia and fludarabine-induced bone marrow failure.

A 49-year-old man with a 3-year history of chronic lymphocytic leukemia (CLL, stage B at diagnosis) responded well to four course of fludarabine, but developed marrow failure and prolonged pancytopenia lasting 9 months following the fifth course. Fludarabine therapy could not be continued due to pancytopenia, eventually resulting in disease progression. Bone marrow transplantation from an unrelated donor mismatched at one DRB1 locus and both DQB1 loci was performed as salvage therapy. The marrow was depleted of T cells with Campath-1G. Pre-transplant immunosuppression was enhanced with 600 cGy total lymphoid irradiation and Campath-1G infusions in addition to 120 mg/kg cyclophosphamide and 1200 cGy fractionated total body irradiation. Cyclosporine alone was used as post-transplant immunosuppression. Neutrophils reached 0.5x10(9)/1 on day 14 and platelets 50 x 10(9)/1 on day 40. No acute graft-versus-host disease was seen. Bulk disease detected on CT scanning prior to BMT was found to have disappeared 10 weeks after BMT. The marrow showed residual disease (5% CD5+/CD19+ cells) 9 weeks after transplantation, which had decreased markedly at 13 (0.5%) and 26 (0.4%) weeks. The patient is currently alive and well 10 months after BMT with no clinically detectable disease. We conclude that BMT from an unrelated donor is a feasible treatment option in advanced CLL.

Renal dysfunction following autologous bone marrow transplantation in adult patients with acute leukemia.

The serum creatinine level was used to determine the incidence of renal dysfunction in 70 adults with acute leukemia who were alive and well one year following autologous bone marrow transplantation (ABMT). Creatinine measurements at the time of ABMT, one year post-ABMT and at the last follow-up (12-128 months, median 35) were recorded, and a level of >120 micromol/l arbitrarily defined as clinically significant renal impairment. The incidence of renal impairment was 2.9% (n = 2) at 1 year, and 4.3% (n = 3) at the last follow-up in continuous remission. Significant renal impairment occurred after relapse in 8 of 12 patients, but was seen in only 3 of 58 patients who remained in remission (p < 0.001, Fisher's exact test), suggesting subclinical renal damage which became obvious with further nephrotoxic therapy. We conclude that clinically significant renal dysfunction is an uncommon long-term complication of ABMT, and should not be a concern in recommending this therapy to eligible patients.