Directed blood donor program decreases donor exposure for children with sickle cell disease requiring chronic transfusion.

In children with sickle cell disease (SCD), primary and secondary prevention of strokes require indefinite regular blood transfusion therapy. The risks associated with repeated transfusions include alloimmunization and increased donor exposure. The Charles Drew Program is a directed blood donor program designed to lower donor exposure, decreasing the associated complications of transfusion; however, no evidence exists demonstrating the magnitude of the benefit to the recipient. Further, the use of extended red blood cell (RBC) antigen matching for C, E, and K has been well documented in a clinical trial setting but not extensively evaluated in a standard care setting. The goal of this study is to assess the effectiveness in reducing alloimmunization when matching for C, E, and K and the magnitude of the decrease in donor exposure in a directed blood donor program. The rate of alloimmunization and reduction of donor exposure were determined during the course of 1 year in a cohort of children with SCD who received regular directed donor blood transfusions. A total of 24 recipients were in the program, 16 females and 8 males, 4 to 20 years of age. During 2008, alloimmunization was 0 percent and donor exposure was reduced by 20 percent, compared with usual care. Extended RBC antigen matching has the same benefit as in a clinical trial setting for patients with SCD receiving blood transfusion therapy. Despite significant effort, we only achieved a modest decrease in donor exposure and cannot determine the immediate benefit of a directed blood donor program.

Applying cognitive response testing in message development and pre-testing.

Pre-testing messages with audience members is a critical step in the creation of effective health information. Quantitative methods for message testing have limited effectiveness, as they cannot reveal complications with language and comprehension. Cognitive response testing (CRT), a form of qualitative research, allows the interviewer to probe for deeper understanding of comprehension and language by asking participants to paraphrase items, discuss thoughts or emotions that come to mind and offer suggestions for improvement. This study explores the usefulness of CRT in message development and testing, adding to the literature regarding qualitative methods in public health. CRT was employed to evaluate health messages on two topics-bioterrorism and influenza vaccination. This technique effectively identified message terminology and concepts that respondents found unfamiliar or confusing, providing the framework needed for message revision. Commonly misunderstood words were replaced and confusing concepts were explained in the revised messages, making pre-tested messages more likely to be appropriate for the intended audience. These findings are consistent with previous research that establishes the usefulness of CRT in the evaluation and development of health-related messages and surveys.

Hypertriglyceridaemia and malignancy.

Severe hypertriglyceridaemia is a rare presentation usually associated with acute pancreatitis. We present two case reports of severe hypertriglyceridaemia occurring in patients with previous autoimmune disease and haematological and solid malignancies but no evidence of prior or concurrent pancreatitis or hyperlipidaemia. These case reports illustrate that haematological and solid organ malignancies and autoimmunity may be relevant as exacerbating factors in the presentation of severe type IV or V hypertriglyceridaemia.

The use of multi-media messaging in the referral of musculoskeletal limb injuries to a tertiary trauma unit using: a 1-month evaluation.

OBJECTIVES: New developments in telecommunications will have a profound effect on the delivery of medical care throughout the world. In Northern Ireland three trauma centres provide fracture care for their own and surrounding Emergency Departments. All trauma referrals are currently taken by telephone. It is our experience that the verbal description of the radiographs of a musculoskeletal limb injury can be inaccurate, necessitating us to view the plain films of the patient. By utilising a recent advance in telecommunications technology, the launch of mobile handsets with multi-media messaging (MMS) service capability, it is now possible to digitally capture and instantly send an image of a plain film. PURPOSE: To evaluate the use of multi-media messaging as a supplement to the telephone referral of musculoskeletal limb injures. METHOD: : Following a referral using, the emergency physician and the trauma surgeon evaluated the multi-media consult through a survey questionnaire. RESULTS: Between the 1st December 2003 and the 1st January 2004, 46 multi-media consultations were performed. Picture quality was acceptable in all but one of the referrals. In 35 of the 46 referrals the multi-media image of the plain films was felt to improve the management of the patient. In 8 of the 46 referrals the multi-media image of the plain films was felt to change the management of the patient. CONCLUSION: A multi-media messaging store- and forward telemedicine system has potential to facilitate the rapid, cost-effective management of musculoskeletal limb injuries thereby enhancing clinical care.

Statin-fibrate combination: therapy for hyperlipidemia: a review.

Statins and fibrates are well-established treatments for hyperlipidaemias and the prevention of vascular events. However, fibrate + statin therapy has been restricted following early reports of rhabdomyolysis that mainly involved gemfibrozil, originally with bovastatin, and recently, with cerivastatin. Despite this limitation, several reports describing combination therapy have been published. This review considers these studies and the relevant indications and contraindications. Statin + fibrate therapy should be considered if monotherapy or adding other drugs (e.g. cholesterol absorption inhibitors, omega-3 fatty acids ornicotinic acid) did not achieve lipid targets or is impractical. Combination therapy should be hospital-based and reserved for high-risk patients with a mixed hyperlipidaemia characterised by low density lipoprotein cholesterol (LDL) >2.6 mmol/l(100 mg/dl, high density lipoprotein cholesterol (HDL) <1.0 mmol/l (40 mg/dl) and/or triglycerides> 5.6 mmol/l (500 mg/dl. These three 'goals' are individually mentioned in guidelines. Patients should have normal renal, liver and thyroid function tests and should not be receiving therapy with cyclosporine, protease inhibitors or drugs metabolised through cytochrome P450 (especially 3A4). Combination therapy is probably best conducted using drugs with short plasma half-lives; fibrates should be prescribed in the morning and statins at night to minimise peak dose interactions. Both drug classes should be progressively titated from low doses. Regular (3-monthly) monitoring of liver function and creatine kinase is required. In conclusion, fibrate + statin therapy remains an option in high-risk patents. However, long-term studies involving safety monitoring and vascular endpoints are required to demonstrate the efficacy of this regimen.

Measurement of human cytomegalovirus loads by quantitative real-time PCR for monitoring clinical intervention in transplant recipients.

Quantitative monitoring of human cytomegalovirus (HCMV) infection is helpful in determining appropriate antiviral management of transplant recipients. Quantitative PCR technologies have demonstrated accuracy in measuring systemic HCMV loads. A total of 298 consecutive whole-blood specimens submitted to the Clinical Virology Laboratory at Vanderbilt University Medical Center from 15 February to 31 October 1999 were included in the study. In addition to a qualitative colorimetric microtiter plate PCR assay (MTP-PCR) and a semiquantitative pp65 antigenemia assay, each specimen was measured for HCMV loads by a quantitative PCR assay performed on an ABI PRISM 7700 Sequence Detection System (TaqMan). Compared to results of the MTP-PCR, the sensitivity, specificity, positive predictive value, and negative predictive value were 70.5, 97.5, 87.8, and 92.8% for the antigenemia assay and were 96.7, 92.0, 75.6, and 99.1% for the TaqMan assay, respectively. There was a high correlation between antigenemia values and HCMV loads as determined by the TaqMan (r = 0.989; P < 0.001). Antigenemia values of 0, 1 to 10, 11 to 100, 101 to 1,000, and over 1,000 positive cells per 2 x 10(5) leukocytes corresponded to median HCMV loads measured by TaqMan of 125, 1,593, 5,713, 16,825, and 5,425,000 copies/ml, respectively. Corresponding to antigenemia values of 1 to 2, 10, and 50 positive cells per 2 x 10(5) leukocytes, HCMV viral loads of 1,000, 4,000, and 10,000 copies/ml are proposed as cutoff points for initiating antiviral therapy in patient groups with high, intermediate, and low risk of CMV diseases.

Drug treatment of combined hyperlipidemia.

Combined hyperlipidemia is increasing in frequency and is the most common lipid disorder associated with obesity, insulin resistance and diabetes mellitus. It is associated with other features of the metabolic syndrome including hypertension, hyperuricemia, hyperinsulinemia and highly atherogenic subfractions of lipoprotein remnant particles including small dense low density lipoprotein-cholesterol. This review examines the mechanisms by which combined hyperlipidemia arises and the various drugs including fibric acid derivatives, hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, and nicotinic acid which can be used either as monotherapy or in combination to manage it and to improve prognosis from atherosclerotic disease in diabetes mellitus, insulin resistant states and primary combined hyperlipidemia. The therapeutic approach to combined hyperlipidemia involves determination of whether the cause is hepatocyte damage or metabolic derangements. Combined hyperlipidemia due to hepatocyte damage should be treated by attention to the primary cause. In the case of metabolic dysfunction because of imbalance in glucose and fat metabolism, therapy of diabetes mellitus and obesity should be optimised prior to commencement of lipid lowering drugs. Both fibric acid derivatives and HMG-CoA reductase inhibitors can be used in the treatment of combined hyperlipidemia with fibric acid derivatives having greater effects on triglycerides and HMG-CoA reductase inhibitors on LDL-C though both have effects on the other cardiovascular risk factors. There is some evidence of benefit with both interventions in mild combined hyperlipidemias and large scale trials are underway. Fibric acid derivatives and HMG-CoA reductase inhibitor therapy can be combined with care, provided that gemfibrozil is avoided, fibric acid derivatives are given in the mornings and shorter half -life HMG-CoA reductase inhibitors are used at night. Combined hyperlipidemia emergencies occur with predominant hypertriglyceridemia in pregnancy or as a cause of pancreatitis. Therapy in the former should aim to reduce chylomicron production by a low fat diet and intervention to suppress VLDL-C secretion using omega-3 fatty acids. In the latter case, fluid therapy alone and medium chain plasma triglyceride infusions usually reduce levels satisfactorily though apheresis may be required. Blood glucose levels also need aggressive management in these conditions. Combined hyperlipidemia is likely to become an increasing problem with the increase in the prevalence of obesity and diabetes mellitus and needs aggressive management to reduce cardiovascular risk.

Very low-density lipoprotein apolipoprotein B-100 turnover in glycogen storage disease type Ia (von Gierke disease).

Mixed hyperlipidaemia is a common finding in glycogen storage disease type Ia (GSD Ia). Although cross-sectional studies have demonstrated increases in intermediate-density lipoproteins (IDLs) and reductions in lipoprotein lipase activity, no studies have investigated the dynamics of apolipoprotein B-100 (apo B) metabolism in GSD Ia. This study investigated apoB turnover in GSD Ia using an exogenous labelling method in one sib from a kinship with established GSD Ia. The study demonstrated normal hepatic secretion of very low-density lipoprotein (VLDL), but hypocatabolism of VLDL, probably due to lack of lipoprotein lipase activity. The production rate of IDL was slightly increased, but the turnover rate of low-density lipoprotein was normal. The findings suggest that, as well as a corn starch diet and dietary fat restriction, treatment of severe mixed hyperlipidaemia in GSD Ia and its attendant risk of pancreatitis should possibly involve fibrates that activate lipoprotein lipase and may enhance the clearance of IDL, rather than omega-3 fatty acids, which principally suppress hepatic secretion of VLDL.

A paradoxical severe decrease in serum HDL-cholesterol after treatment with a fibrate.

There have been a handful of reports in the literature of a paradoxical decrease in serum high density lipoprotein (HDL)-cholesterol in patients on fibrate drugs. The reason for this decline in cardioprotective HDL-cholesterol is not known and may have potential deleterious effects on the patient. This report describes a decrease in serum HDL-cholesterol in a patient on both simvastatin and bezafibrate. This patient also developed abnormal renal function, probably interstitial nephritis. In addition, the literature of fibrate induced serum HDL-cholesterol decline is reviewed and possible mechanisms for this phenomenon discussed.

A first British case of fish-eye disease presenting at age 75 years: a double heterozygote for defined and new mutations affecting LCAT structure and expression.

Fish-eye disease is a familial syndrome with corneal opacification, major high density lipoprotein (HDL) deficiency in plasma, significant cholesterol esterification in plasma on non-HDL lipoproteins, generally without premature coronary disease. This first British male case from unrelated British parents had infarcts when aged 49 and 73 years but was asymptomatic at age 81 years, with plasma cholesterol 4.3-7.1 mmol/litre, triglycerides 1.8-2.2 mmol/litre, HDL cholesterol < 0.1 mmol/litre, apolipoprotein A-I < 0.16 g/litre, lipoprotein(a) 0.61 g/litre. Cholesterol esterification was impaired using HDL-3 and A-I proteoliposomes but not using VLDL/IDL/LDL. The findings are those of LCAT deficiency with the classic fish-eye disease defect. Most of the 22 reported cases were homozygous or heterozygous for a Thr-Ile mutation at codon 123 of the lecithin:cholesterol acyltransferase (LCAT) gene. This patient was a double heterozygote for this mutation and a second new incompletely defined mutation affecting LCAT expression as defined by reduced mass and activity in plasma.

Community health collaboration models for the 21st century.

The development of partnerships with physicians and other health professionals is a key strategy to improving the community's health. This article reviews the role of nurse leaders as advocates for health care improvement and leading community improvement efforts.

Complex dislocation of the index metacarpophalangeal joint with entrapment of a sesamoid.

Entrapment of a sesamoid in a complex dislocation of the index metacarpophalangeal joint is a rare finding that may make even open reduction difficult. A case of unusual anatomic derangement of the volar plate is reported that highlights the underlying pathophysiology of complex dislocations.

Nosocomial acquisition of pertussis diagnosed by polymerase chain reaction.

Awareness is growing of the role of pertussis infection among adolescents and adults, and of the possibility of nosocomial transmission. However, pertussis remains difficult to diagnose. We report a case of nosocomially acquired pertussis in a healthcare worker, diagnosed by polymerase chain reaction. The high sensitivity and rapid turnaround time for this test is useful to confirm the diagnosis when advising a healthcare worker to refrain from working.

On the causes of collapse and sudden death by Avicenna.

Avicenna's views on the causes of collapse and sudden death are presented from a literal translation of the mediaeval Arabic text. Medical knowledge based on observation mixed with pure abstract reasoning forms the essence of Avicenna's medical writings.