Prime

Type your tag names separated by a space and hit enter

Drugs for preventing red blood cell dehydration in people with sickle cell disease.

Abstract

BACKGROUND

Sickle cell disease is an inherited disorder of haemoglobin, which results in abnormal red blood cells. These can deform and cause blockages in blood vessels, leading to acute crises such as pain, stroke and splenic sequestration, and chronic organ and tissue damage. Recently research has begun to focus on therapies which prevent the red blood cells deforming by reducing the loss of water and ions from the cells. However, little is known about the effectiveness and safety of such drugs.

OBJECTIVES

To assess the relative risks and benefits of drugs which aim to prevent sickle cell related crises by reducing red blood cell dehydration.

SEARCH STRATEGY

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialist register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Date of the most recent search of the Group's specialised register: December 2001.

SELECTION CRITERIA

All those randomised or quasi-randomised controlled trials of drugs which aim to prevent sickle cell crises by reducing red cell dehydration, compared to placebo or an alternative treatment.

DATA COLLECTION AND ANALYSIS

Both reviewers independently selected trials for inclusion, assessed trial quality and extracted data from the included studies.

MAIN RESULTS

Of the 27 trials identified, two met the inclusion criteria. The two trials tested the effectiveness of zinc sulphate and piracetam to prevent sickle cell related crises in a total of 246 patients. A reduction in pain crises was shown in the piracetam study over one year (weighted mean difference (WMD) -1.9 (95% CI -3.01, -0.79)), although blood counts were not significantly changed. The zinc trial showed a significant reduction in the total number of pain, haemolytic, aplastic and sequestration crises over one and a half years (WMD -2.83 (95% CI -3.51, -2.15)), but our analysis was limited by non-reporting of standard deviations for some data. Changes to red cell parameters and blood counts were inconsistent. No serious adverse events were noted in either trial.

REVIEWER'S CONCLUSIONS

While the results of both zinc and piracetam for reducing sickle related crises are encouraging, larger, and/or longer term multicentre trials over a number of years are needed to evaluate the effectiveness of these therapies for patients with sickle cell disease.

Links

  • Publisher Full Text
  • Authors+Show Affiliations

    ,

    Institute of Child Health, University of Liverpool, Alder Hey Children's Hospital, Eaton Road, Liverpool, UK, L12 2AP. cerir@liverpool.ac.uk

    Source

    MeSH

    Anemia, Sickle Cell
    Antisickling Agents
    Dehydration
    Erythrocytes
    Humans
    Piracetam
    Randomized Controlled Trials as Topic
    Zinc Sulfate

    Pub Type(s)

    Journal Article
    Meta-Analysis
    Review

    Language

    eng

    PubMed ID

    12519597

    Citation

    TY - JOUR T1 - Drugs for preventing red blood cell dehydration in people with sickle cell disease. AU - Riddington,C, AU - De Franceschi,L, PY - 2003/1/10/pubmed PY - 2003/2/22/medline PY - 2003/1/10/entrez SP - CD003426 EP - CD003426 JF - The Cochrane database of systematic reviews JO - Cochrane Database Syst Rev IS - 4 N2 - BACKGROUND: Sickle cell disease is an inherited disorder of haemoglobin, which results in abnormal red blood cells. These can deform and cause blockages in blood vessels, leading to acute crises such as pain, stroke and splenic sequestration, and chronic organ and tissue damage. Recently research has begun to focus on therapies which prevent the red blood cells deforming by reducing the loss of water and ions from the cells. However, little is known about the effectiveness and safety of such drugs. OBJECTIVES: To assess the relative risks and benefits of drugs which aim to prevent sickle cell related crises by reducing red blood cell dehydration. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialist register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Date of the most recent search of the Group's specialised register: December 2001. SELECTION CRITERIA: All those randomised or quasi-randomised controlled trials of drugs which aim to prevent sickle cell crises by reducing red cell dehydration, compared to placebo or an alternative treatment. DATA COLLECTION AND ANALYSIS: Both reviewers independently selected trials for inclusion, assessed trial quality and extracted data from the included studies. MAIN RESULTS: Of the 27 trials identified, two met the inclusion criteria. The two trials tested the effectiveness of zinc sulphate and piracetam to prevent sickle cell related crises in a total of 246 patients. A reduction in pain crises was shown in the piracetam study over one year (weighted mean difference (WMD) -1.9 (95% CI -3.01, -0.79)), although blood counts were not significantly changed. The zinc trial showed a significant reduction in the total number of pain, haemolytic, aplastic and sequestration crises over one and a half years (WMD -2.83 (95% CI -3.51, -2.15)), but our analysis was limited by non-reporting of standard deviations for some data. Changes to red cell parameters and blood counts were inconsistent. No serious adverse events were noted in either trial. REVIEWER'S CONCLUSIONS: While the results of both zinc and piracetam for reducing sickle related crises are encouraging, larger, and/or longer term multicentre trials over a number of years are needed to evaluate the effectiveness of these therapies for patients with sickle cell disease. SN - 1469-493X UR - https://www.unboundmedicine.com/medline/citation/12519597/full_citation L2 - http://dx.doi.org/10.1002/14651858.CD003426 ER -