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Drugs for preventing red blood cell dehydration in people with sickle cell disease.
Cochrane Database Syst Rev 2007; (4):CD003426CD

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's Haemoglobinopathies Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of the most recent search of the Group's Haemoglobinopathies Trials Register: November 2006.

SELECTION CRITERIA

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 authors independently selected studies for inclusion, assessed study quality and extracted data from the included studies.

MAIN RESULTS

Of the 39 studies identified, one met the inclusion criteria. This study tested the effectiveness of zinc sulphate to prevent sickle cell-related crises in a total of 145 participants and 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 to -2.15). However, 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 the study.

AUTHORS' CONCLUSIONS

While the results of zinc for reducing sickle-related crises are encouraging, larger and longer-term multicentre studies over a number of years are needed to evaluate the effectiveness of this therapy for people with sickle cell disease.

Authors

No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review
Systematic Review

Language

eng

PubMed ID

17943791

Citation

Singh, P C., and S K. Ballas. "Drugs for Preventing Red Blood Cell Dehydration in People With Sickle Cell Disease." The Cochrane Database of Systematic Reviews, 2007, p. CD003426.
Singh PC, Ballas SK. Drugs for preventing red blood cell dehydration in people with sickle cell disease. Cochrane Database Syst Rev. 2007.
Singh, P. C., & Ballas, S. K. (2007). Drugs for preventing red blood cell dehydration in people with sickle cell disease. The Cochrane Database of Systematic Reviews, (4), p. CD003426.
Singh PC, Ballas SK. Drugs for Preventing Red Blood Cell Dehydration in People With Sickle Cell Disease. Cochrane Database Syst Rev. 2007 Oct 17;(4)CD003426. PubMed PMID: 17943791.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Drugs for preventing red blood cell dehydration in people with sickle cell disease. AU - Singh,P C, AU - Ballas,S K, Y1 - 2007/10/17/ PY - 2007/10/19/pubmed PY - 2008/1/18/medline PY - 2007/10/19/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's Haemoglobinopathies Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Date of the most recent search of the Group's Haemoglobinopathies Trials Register: November 2006. SELECTION CRITERIA: 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 authors independently selected studies for inclusion, assessed study quality and extracted data from the included studies. MAIN RESULTS: Of the 39 studies identified, one met the inclusion criteria. This study tested the effectiveness of zinc sulphate to prevent sickle cell-related crises in a total of 145 participants and 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 to -2.15). However, 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 the study. AUTHORS' CONCLUSIONS: While the results of zinc for reducing sickle-related crises are encouraging, larger and longer-term multicentre studies over a number of years are needed to evaluate the effectiveness of this therapy for people with sickle cell disease. SN - 1469-493X UR - https://www.unboundmedicine.com/medline/citation/17943791/Drugs_for_preventing_red_blood_cell_dehydration_in_people_with_sickle_cell_disease_ L2 - https://doi.org/10.1002/14651858.CD003426.pub2 DB - PRIME DP - Unbound Medicine ER -