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Insulin and oral agents for managing cystic fibrosis-related diabetes.

BACKGROUND: Insulin therapy is recommended by the Cystic Fibrosis Foundation when cystic fibrosis-related diabetes has been diagnosed. Diagnosis is based on an elevated fasting blood glucose level greater than 6.94 mmol/liter (125 mg/deciliter) or symptomatic diabetes for random glucose levels greater than 11.11 mmol/liter (200 mg/deciliter). OBJECTIVES: To examine the evidence that, when treated with agents for managing diabetes, people with cystic fibrosis improve their sugar metabolic control resulting in beneficial impact on lung function and the ability to maintain optimal weight. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also handsearched abstracts from pulmonary and North American Cystic Fibrosis Conference symposia. Date of the most recent search of the Group's Trials Register: December 2004. SELECTION CRITERIA: Randomized controlled trials comparing all methods of diabetes therapy for one month or longer in people with cystic fibrosis. DATA COLLECTION AND ANALYSIS: No studies were found which were eligible for inclusion in this review. MAIN RESULTS: Six references to four studies were identified by the searches, but none were eligible for inclusion in the review as they were not randomized controlled trials. AUTHORS' CONCLUSIONS: While some cystic fibrosis centers use oral medications to help control diabetes, a condition which complicates the course of cystic fibrosis, insulin therapy is the recommended and most widely used treatment method. Lung function has been reported to improve with the use of insulin, but this has not been correlated to the degree in which sugar metabolism has been affected. While the Cystic Fibrosis Foundation recommends insulin therapy be used in managing diabetes, this systematic review identifies the need for a multicentre randomized controlled trial assessing both the efficacy of insulin or other insulin-releasing or insulin-sensitizing medications and their possible adverse effects in managing cystic fibrosis-related diabetes.

Administration, Oral↗

Establishing a transplant coordinator-led living kidney donor follow-up clinic.

BACKGROUND: The long-term risks of renal failure and hypertension are statistically low for living kidney donors as a group, but can have serious consequences for the individual. OBJECTIVES: To describe the experience with a transplant coordinator-led living donor follow-up clinic. METHOD: Living kidney donors are reviewed on an annual basis by a designated coordinator (registered nurse). A 24-hour urine collection estimates renal function. Blood pressure and blood chemistry are measured and urinalysis performed. Current health status and wound discomfort are assessed. Any medical problems identified are referred to a specialist hospital department or to the donor's family practitioner. RESULTS: Fifty-nine appointments were booked and 12 (20%) donors did not attend. Renal function was within acceptable limits for all attending donors. Three donors had raised blood glucose levels and 8 donors were hypertensive; all were referred to family practitioners. Forty-seven donors (35 new, 12 return) completed a questionnaire on the follow-up provided. Thirty-eight (81%) were satisfied with the follow-up, and 47 (100%) agreed this clinic provided adequate follow-up. Thirty-three (70%) donors stated they preferred that the transplant coordinator performed the follow-up, 3 (6%) preferred the family practitioner, and 11 (23%) had no preference. CONCLUSIONS: There are many possible solutions to the provision of lifelong care of living kidney donors. The model of a transplant coordinator-led clinic appears to have a high degree of patient acceptance, perhaps because of the continuity of care provided by a known member of the transplant team. Further work is required to identify reasons for nonattendance.

Aftercare↗

[Ambrosia, Franseria, Xanthium from the Bonaparte Herbarium].

Recently, French Association for Ragweed Study (AFEDA) founders published, in French, a book "Ambrosia, ragweed, biological pollutants". This association was founded in 1982. The authors referred to Bonaparte Herbarium set up in the Université Claude-Bernard Lyon 1. This herbarium is ranking second in France and seventh in the world. There were three aims for this work: to set up, for this herbarium, a complete list of different species of Ambrosia, Franseria and their pollen grains and to study with them Xanthium whose pollen grains look like Ambrosia, to establish a chronological order for Ambrosia that were collected in Europe and in some countries of the New World, to compare some pollen grains of Ambrosia and Xanthium (scanning electronic microscope). Good state plants in spite of about a century of conservation could be selected. They were photographied and some pollens so. The consultation of this herbarium brought a better morphological knowledge of different species of Ambrosia, Franseria and Xanthium, (plants and pollen grains). The authors were able to broadcast this knowledge to scientists and interested public through their book.

Ambrosia↗

Prophylactic antibiotics for cystic fibrosis.

BACKGROUND: Patients with cystic fibrosis are sometimes prescribed antibiotics to take continuously on a prophylactic (preventative) basis. This approach is most commonly used in infants where the objective is to reduce pulmonary infection with Staphylococcus aureus and prevent lung damage. This approach may also be used in older patients. This review evaluates the evidence for the effectiveness of this approach and considers potential adverse effects. OBJECTIVES: To compare continuous oral antibiotic prophylaxis with no prophylaxis (short courses of oral antibiotics given as clinically indicated) in patients with cystic fibrosis. This review considers both the effectiveness of prophylaxis (bacteria isolated from the respiratory tract, requirement for additional antibiotic treatment, lung function, survival) and the adverse effects. SEARCH STRATEGY: The Cochrane Cystic Fibrosis and Genetic Disorders Group clinical trials register was used. This comprises references identified from a comprehensive search of electronic databases, as well as hand searching relevant journals and conference abstracts. Companies manufacturing anti-staphylococcal antibiotics were also approached for unpublished data. Date of the most recent search of the Group's specialised register: November 1999. SELECTION CRITERIA: All randomised or pseudo-randomised trials where continuous oral prophylactic antibiotics, given for a period of at least one year, were compared to intermittent antibiotic therapy given "as required." Cystic fibrosis patients of any disease severity were considered. DATA COLLECTION AND ANALYSIS: Trials were assessed for eligibility, methodological quality and data extraction by two reviewers (AS & SW). The following outcomes were assessed: lung function; nutrition (weight standard deviation score); survival; requirement for additional antibiotic treatment; isolates of pathogens from the respiratory tract; occurrence of adverse reactions to prophylactic antibiotics. MAIN RESULTS: Only two studies, totalling 66 patients (over half of whom were infants), were suitable for inclusion in the review. A reduced prevalence of Staphylococcus aureus in the respiratory secretions was seen in children receiving anti-staphylococcal antibiotic prophylaxis, although no effect was seen on other common pathogens. This is associated with a reduced requirement for additional courses of oral antibiotics and fewer hospital admissions in the first two years of life in patients receiving prophylaxis. No effect on infant lung function has been shown after one year of prophylactic treatment. Data are not available on adverse effects of the interventions. As the duration of the studies reviewed has been of two years or less, conclusions cannot be drawn about the long term effects of prophylaxis on acquisition of Pseudomonas aeruginosa and survival. REVIEWER'S CONCLUSIONS: Anti-staphylococcal antibiotic prophylaxis may be of benefit when commenced early in infancy and continued up to two years of age. There is insufficient evidence from this review to say whether use in older children, or adults, or for periods of over two year is beneficial.

Adult↗

Macrolide antibiotics for cystic fibrosis.

BACKGROUND: Chronic severe infection with Pseudomonas aeruginosa, affects many people with cystic fibrosis (CF). There is evidence from the laboratory and from other disease processes that macrolide antibiotics, whilst not directly active against Pseudomonas aeruginosa, may have indirect actions against this organism. OBJECTIVES: We aimed to test the hypotheses that, in people with CF, macrolide antibiotics:(1) improve clinical status compared to placebo or another antibiotic;(2) do not have unacceptable adverse effects. If benefit was demonstrated, we aimed to assess the optimal type, dose and duration of macrolide therapy. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. We contacted principal investigators known to work in the field, previous authors and pharmaceutical companies who manufacture macrolide antibiotics for unpublished or follow-up data (December 2003). Most recent search of the Group's register: January 2004 SELECTION CRITERIA: Published or unpublished randomised controlled trials of macrolide antibiotics compared to placebo, another class of antibiotic or another macrolide antibiotic. Studies comparing regimens of the same macrolide antibiotic at different doses will also be included. DATA COLLECTION AND ANALYSIS: Two reviewers independently extracted data and assessed study quality. Three groups were contacted for missing data and we hope to include these in future reviews. MAIN RESULTS: Searches identified 14 studies, four were included in this review (296 participants). Two studies enrolled adults, one children (a significant number of whom were not colonised with Pseudomonas aeruginosa) and one both adults and children. All the clinical studies reported small but significant improvements in respiratory function with azithromycin versus placebo. Meta-analysis at the one-month and six-month time points demonstrates a significant benefit with respect to relative change in FEV1 (at six months, for n = 104, azithromycin and n = 114, placebo; WMD 5.82% (95% CI 2.45 to 9.20)). The largest study reported a significant increase in mild adverse events (nausea, diarrhoea and wheezing). REVIEWERS' CONCLUSIONS: There is clear evidence from these studies of a small but significant improvement in respiratory function following treatment with azithromycin. The largest study employed a three times a week dose and, in this study, treatment with azithromycin was associated with a significant increase in mild adverse events. Further studies are needed to clarify the precise role of azithromycin in the treatment of CF lung disease.

Anti-Bacterial Agents↗

Psychological therapies for thalassaemia.

BACKGROUND: Thalassaemia is a group of genetic blood disorders characterised by the absence or reduction in the production of haemoglobin. Severity is variable from less severe anaemia, through thalassaemia intermedia, to profound severe anaemia (thalassaemia major). In thalassaemia major other complications include growth retardation, bone deformation, and enlarged spleen. Blood transfusion is required to treat severe forms of thalassaemia, but this results in excessive accumulation of iron in the body (iron overload), removed mostly by a drug called desferrioxamine through 'chelation therapy'. Non-routine treatments are bone marrow transplantation (which is age restricted), and possibly hydroxyurea, designed to raise foetal haemoglobin level, thus reducing anaemia. In addition, psychological therapies seem appropriate to improving outcome and adherence to medical treatment. OBJECTIVES: To examine the evidence that in patients with thalassaemia, psychological treatments improve the ability to cope with the condition, and improve both medical and psychosocial outcome. SEARCH STRATEGY: The Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Also, searches on the Internet were performed. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in patients with thalassaemia. DATA COLLECTION AND ANALYSIS: No trials of psychological therapies were found in the literature for inclusion at the present time. MAIN RESULTS: There are no results to be reported at present. REVIEWER'S CONCLUSIONS: As a chronic disease with a considerable role for self-management, psychological support seems appropriate for managing thalassaemia. However, no conclusions can be made about the use of specific psychological therapies in thalassaemia from the information currently available. This systematic review has clearly identified the need for well designed, adequately-powered, multicentre, randomised controlled trials assessing the effectiveness of specific psychological interventions for thalassaemia.

Adaptation, Psychological↗

Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease.

BACKGROUND: Bronchodilators are used to treat bronchial hyper-responsiveness in asthma. Bronchial hyper-responsiveness may be a component of the acute chest syndrome in people with sickle cell disease. Therefore, bronchodilators may be useful in the treatment of acute chest syndrome. OBJECTIVES: To assess the benefits and risks associated with the use of bronchodilators in people with acute chest syndrome. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. Additional searches were carried out on MEDLINE (1966 to 2002) and EMBASE (1981 to 2002).Date of the most recent search of the Group's haemoglobinopathies register: May 2002. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials. Trials using quasi-randomisation methods will be included in future updates of this review if there is sufficient evidence that the treatment and control groups are similar at baseline. DATA COLLECTION AND ANALYSIS: We found no trials investigating the use of bronchodilators for acute chest syndrome in people with sickle cell disease. MAIN RESULTS: We found no trials investigating the use of bronchodilators for acute chest syndrome in people with sickle cell disease. REVIEWER'S CONCLUSIONS: If bronchial hyper-responsiveness is an important component of some episodes of acute chest syndrome in people with sickle cell disease, the use of inhaled bronchodilators may be indicated. There is need for a well-designed, adequately powered randomised controlled trial to assess the benefits and risks of the addition of inhaled bronchodilators to established therapies for acute chest syndrome in people with sickle cell disease.

Acute Disease↗

Totally implantable vascular access devices for cystic fibrosis.

BACKGROUND: Totally implantable vascular access devices are widely used in people with cystic fibrosis to provide intermittent venous access for therapeutic infusions. Their use is associated with some complications such as thrombosis, embolism and infection. OBJECTIVES: To assess if totally implantable venous access devices are a safe and effective route for providing venous access for intermittent administration of intravenous antibiotics in people with cystic fibrosis, also to assess strategies to reduce possible complications of totally implantable venous access devices (e.g. anticoagulants to reduce the risk of thrombosis). SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. Date of the most recent search: May 2003. SELECTION CRITERIA: Randomised and quasi-randomised controlled trials which compared the use of totally implantable venous access devices in people with cystic fibrosis to other means of vascular access, trials which compared the different types of these devices against each other and trials which assessed strategies to reduce complications of these devices. DATA COLLECTION AND ANALYSIS: No relevant trials were identified. MAIN RESULTS: No trials were included in this review. REVIEWER'S CONCLUSIONS: Totally implantable vascular access devices are widely used in people with cystic fibrosis to provide intermittent venous access for therapeutic infusions. Reports of their use in people with cystic fibrosis suggest that they are safe and effective. These reports also suggest that certain interventions might reduce the risk of complications; however, it is disappointing that these reports have not been assessed by randomised controlled trials. This systematic review identifies the need for a multicentre randomised controlled trial assessing both efficacy and possible adverse effects of totally implantable venous access devices in cystic fibrosis.

Catheters, Indwelling↗

Sodium channel blockers for cystic fibrosis.

BACKGROUND: People with cystic fibrosis (CF) have increased transport of the salt, sodium across their airway lining. Over-absorption of sodium results in the dehydration of the liquid that lines the airway surface and is a primary defect in people with CF. OBJECTIVES: To determine whether the topical administration of drugs that block sodium transport improves the respiratory condition of people with CF. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, handsearching relevant journals and abstract books of conference proceedings. We contacted principal investigators known to work in the field, previous authors and pharmaceutical companies who manufacture ion transport agents for unpublished or follow-up data. Most recent search of the Group's register: March 2006 SELECTION CRITERIA: Published or unpublished randomised controlled trials (RCTs) or quasi-randomised controlled trials of sodium channel blockers compared to placebo or another sodium channel blocker or the same sodium channel blocker at a different dosing regimen. DATA COLLECTION AND ANALYSIS: Two authors independently extracted data. Meta-analysis was limited due to differing study designs. MAIN RESULTS: Four RCTs, with a total of 205 participants, examining the topical administration of the short-acting sodium channel blocker, amiloride, compared to placebo were identified as eligible for inclusion in the review. For three studies, interventions for six months were completed and it was possible to calculate relative change in respiratory function (FVC). There was a significant difference found in relative change in FVC in favour of placebo (GIV analysis of weighted mean difference for FVC; 1.51% (95% confidence interval -2.77 to -0.25). There were no significant differences identified in other clinically relevant outcomes. AUTHORS' CONCLUSIONS: We found no evidence that the topical administration of a short-acting sodium channel blocker improves respiratory condition in people with cystic fibrosis and some limited evidence of deterioration in lung function.

Cystic Fibrosis↗

Autologous bone marrow transplantation in acute lymphoblastic leukaemia.

The purpose of this review is to assess the current status of autografting in acute lymphoblastic leukaemia. In the first part of the review, the prognostic factors are discussed. Only the studies that contained adequate response and survival data were selected for analysis, including peer-reviewed articles, book chapter and proceedings of international meetings. The most current and complete references were used for series reported more than once. In the second part, some areas of discussion and controversy, such as the timing of autografting and the role of marrow purging are argued. In the last part of this review we have discussed our recent experience with blood progenitor cells collected during early haemopoietic function restoration after intensive conventional treatment for patients with cytogenetic abnormalities. The first preliminary results seem highly interesting and confirm our results achieved with the same procedure in Ph-positive chronic myelogenous leukaemia.

Adolescent↗

Liver disease in the Talmud.

Diseases of the liver have been of interest to physicians throughout the ages. There are references to hepatic diseases in ancient writings on medicine as far back as the Egyptian papyrii (1). In this article, I deal with the multiple references to the liver and liver disease that are found in the books of the Talmud. The areas discussed include anatomy, physiology, trauma, liver disease, jaundice, cholelithiasis, and hematemesis. The modes of therapy and their theory give an interesting perspective to Middle Eastern medicine 1,500 years ago.

Animals↗

The malevolent personality disorder?

The identification of any new psychopathology or syndrome has been based on numerous clinical enquiries and theoretical discussions and has assumed an important place in the literature of psychology and psychiatry. Using two case descriptions and the subject described in a recent book, this report presents a theoretical discussion of an unusual personality organization, referred to as the malevolent personality disorder. Differential diagnosis, theorizing about pathogenesis, clarification of salient psychodynamics, and treatment considerations are explored.

Adult↗

Clinical experience with management of "near-miss" cases in obstetrics.

Near-miss cases in life-threatening obstetric patients occurring over a one year period are analysed retrospectively with regards to morbidity measured in terms of hospital stay, utilisation of high dependency ward and intensive care beds and adequacy of clinical management. One-hundred and twenty two cases occurred among 9932 deliveries. Massive obstetric haemorrhage (54.2%) and hypertensive disorders of pregnancy (36.9%) were the two main diagnostic groups. Seventy one (58.2%) cases were referred from peripheral centres for obstetric management and 77 (63.1%) were not booked at this hospital for antenatal care. A majority were not ill-looking (92 cases) at the time of admission but turned for the worse in the course of labour. Interventional measures taken in clinical management were considered appropriate in all cases. Delay in instituting treatment was present in 6 cases. Remediable measures were recognised in 15 (12.3%). This study, apart from supplementing mortality audits, demonstrates that high risk obstetric patients can be triaged at the time of admission to labour wards by trained midwives and junior doctors in busy obstetric units without compromising standards of care.

Adult↗

Autologous bone marrow transplantation in acute lymphoblastic leukemia: biological and clinical aspects.

The purpose of this review is to assess the current status of autologous bone marrow transplantation in acute lymphoblastic leukemia. In the first part of the review the biological aspects of minimal residual disease are discussed and in the second part the most recent clinical results analyzed. Only the studies that contained adequate response and survival data were selected for analysis, including peer-reviewed articles, book chapters and proceedings of international meetings. The most current or complete references were used for series reported more than once.

Antineoplastic Combined Chemotherapy Protocols↗

Problems and complications in the management of upper limb anomalies.

Nonsurgical problems and complications of orthotist-prosthetist techniques are not addressed in this article. There are a number of complications, such as decubiti, joint contracture or damage, tendon injury, wound healing, etc., which are not discussed because they are similar in cause, identification, and treatment to the same adult problems. Though it is possible to list the more common complications seen for each congenital anomaly, the tedious repetitiveness of such an approach dissuaded the author. General principles can always be specifically applied when the occasion requires. It is also possible to detail painstakingly the techniques of coping with each complication, but such would require book rather than chapter exegesis, and those who need these details are referred to the bibliography. The theme of this article is that there is no longer much distinction in the mind of the consumer clientele between the problem of the congenital anomaly, the results of treatment, and the complications of those treatments. The provider must therefore be wary, extensively informed, and exceedingly communicative, to the point of suggesting secondary consultation as warranted or desired. Despite this almost adversarial approach, the rewards are still great, for more can be accomplished than ever before, the child will seldom show motivational problems in using what is provided, and gratitude is not lost coinage.

Amniotic Band Syndrome↗

Comparison of American medical dictionaries.

Although American medical dictionaries are a valuable part of any medical library collection, the attributes of each of the four major dictionaries are often unknown and the reference material contained in each unused. The medical librarian should be aware of the differences and values of each dictionary and try to have at least one edition of each available to library users in order to maintain an adequate reference collection.

Book Selection↗

Traditional Chinese medicine and treatment of neonatal jaundice.

OBJECTIVE: Treatment with herbs may increase the risk of neonatal jaundice (NNJ). It is logical to look into the current practice in some hospitals in China where herbs are being used in the treatment of NNJ. It is also the purpose of this study to find out the chemical constituents and actions of the herbs, and the rationale of the treatment. METHODS: Twenty reports, from 1973 to 1989, from different parts of China, come in a published book and the paediatric journals written in the Chinese language. The Zhong Yao Da Zi Dian, an encyclopedia of Chinese materia medica, and other books on the pharmacology and applications of Chinese materia medica were also referred to in the study. FINDINGS: Yin-chen (oriental wormwood or Artemisia) was the most commonly used herbs for NNJ (95%). Others were Da-huang (rhubarb or Rheum officinale), Huang-qin (skullcap root or Scutellaria), Gan-cao (licorice or glycyrrhiza) and Huang-lian (goldthread rhizome or Copts chinesis). Huang-lian, which contains the alkaloid berberine, was used in 4 centers (20%). Berberine can cause severe acute hemolysis in babies with G6PD deficiency. Currently, Yin-chen comes as a decoction Artemisia composita and an intravenous preparation. These preparations have potential central nervous system and cardiovascular toxicities. CONCLUSIONS: Chinese herbs have many pharmacological substances and therefore multiple actions. In recent years, Chinese herbs are used in conjunction with "Western" drugs, rendering the study of the effects of herbs on NNJ extremely difficult. The efficacy and safety of phototherapy for NNJ have been firmly established, thus diminishing the need for drug treatment. What is the present day role, therefore, of herbal medicine for NNJ? Is there a place for further research of these herbal medicines?

Drugs, Chinese Herbal↗