Sickle Cell Anemia

ETHNIC QUESTION & ANTENATAL SCREENING for SICKLE CELL/THALASSAEMIA [EQUANS]

by Scott Yates

Public Health Implications of Sickle Cell Trait: A Report of the CDC Meeting

by Melissa Creary

What Do Physicians, Insurers, and Consumers Need To Know About Hydroxyurea for Appropriate Utilization? The Consumer's Perspective

by Melissa Creary

A Qualitative Analysis of Best Self-management Practices: Sickle Cell Disease

by Melissa Creary

Oxygen isotopes as a biomarker for sickle-cell disease? Results from transegnic mice expressing human hemoglobin S genes

by Laurie Reitsema

Co-authored with Douglas E. Crews. Published in the American Journal of Physical Anthropology (2011).

The origins of sickle-cell disease (SCD) are well understood, as are its evolutionary pressures on humans and... more The origins of sickle-cell disease (SCD) are well understood, as are its evolutionary pressures on humans and pathological presentation. However, because it has not been possible to identify SCD in archaeological contexts, its biocultural effects on past populations are unknown. Previous research investigating oxygen isotope fractionation during respiration among anemics suggests that oxygen isotopes in bone apatite may provide a biological marker for SCD in skeletal remains. This pilot study reports d18O ratios in bone apatite of transgenic laboratory mice expressing human SCD globins and compares them to healthy control mice. The d18O ratios of sick mice are significantly lower than those of healthy mice (25.6% vs. 24.5%; P 5 0.002), and the sickest mice exhibit the lowest ratios of all (mean d18O 5 25.8%). These preliminary results suggest that this method may be usefully applied to skeletal materials of past human populations whose diets and water sources do not differ substantially.

Human sickle cell blood modulates endothelial heme oxygenase activity: effects on vascular adhesion and reactivity

by Roberto Motterlini

ARTERIOSCLEROSIS, THROMBOSIS AND VASCULAR BIOLOGY
Sandip K. BAINS, Roberta FORESTI, Jo HOWARD, Sangeeta ATWAL, Colin J. GREEN and Roberto MOTTERLINI

OBJECTIVES. Sickle cell disease (SCD) is characterized by extensive hemolysis, increased cellular adhesion, and... more OBJECTIVES. Sickle cell disease (SCD) is characterized by extensive hemolysis, increased cellular adhesion, and vaso-occlusion. Tissues from sickle patients express heme oxygenase-1 (HO-1), the enzyme that degrades free heme/hemoglobin to the signaling molecule carbon monoxide, and the antioxidants biliverdin/bilirubin. Here, we examined the HO response in endothelial cells exposed to human sickle blood and determined whether this response is beneficial for SCD.
METHODS AND RESULTS: We measured HO activity in human and bovine aortic endothelial cells incubated with human sickle or normal blood. Sickle blood increased HO activity, which was enhanced by hypoxia and was caused mainly by the red cell components of sickle blood. Oxidized hemoglobin was higher in sickle blood and increased markedly over time. Interestingly, HO activity correlated inversely with patients' hemoglobin levels and positively with bilirubin and lactate dehydrogenase. HO-1 induction, exogenous biliverdin, or carbon monoxide markedly decreased adhesion of sickle blood to the endothelium, and sickle red cells partially inhibited relaxation mediated by carbon monoxide in isolated aortas.
CONCLUSIONS: Our results highlight important associations between SCD and HO byproducts, which may counteract vascular complications of SCD.

Blood transfusions for treating acute chest syndrome in people with sickle cell disease.

by Zbys Fedorowicz

Inhaled nitric oxide for acute chest syndrome in people with sickle cell disease

by Zbys Fedorowicz

Piracetam for reducing the incidence of painful sickle cell disease crises.

by Zbys Fedorowicz

Antibiotics for treating osteomyelitis in people with sickle cell disease

by Nikki Jahnke

Authors: Arturo J Martí-Carvajal, Luis H Agreda-Pérez
Cochrane Review Group: Cystic Fibrosis & Genetic Disorders

This review was updated in January 2010 and will be updated again in January 2012.

Background
Osteomyelitis (both acute and chronic) is one of the most common infectious complications in people... more Background
Osteomyelitis (both acute and chronic) is one of the most common infectious complications in people with sickle cell disease. There is no standardized approach to antibiotic therapy and treatment is likely to vary from country to country. Thus, there is a need to identify the efficacy and safety of different antibiotic treatment approaches for people with sickle cell disease suffering from osteomyelitis.


Objectives
To determine whether an empirical antibiotic treatment approach (monotherapy or combination therapy) is effective and safe as compared to pathogen-directed antibiotic treatment and whether this effectiveness and safety is dependent on different treatment regimens, age or setting.


Search strategy
We searched The Group's Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings. We also searched the LILACS database (1982 to 11 November 2010), African Index Medicus (11 November 2010), ISI Web of Knowledge (11 November 2010) and World Health Organization International Clinical Trials Registry Platform (11 November 2010).

Date of most recent search of the Haemoglobinopathies Trials Register: 08 December 2010.


Selection criteria
We searched for published or unpublished randomised and quasi-randomised controlled trials.


Data collection and analysis
Each author intended to independently extract data and assess trial quality by standard Cochrane Collaboration methodologies, but no eligible randomised controlled trials were identified.


Main results
This update was unable to find any randomised or quasi-randomised controlled trials on antibiotic treatment approaches for osteomyelitis in people with sickle cell disease.


Authors' conclusions
We were unable to identify any relevant trials on the efficacy and safety of the antibiotic treatment approaches for people with sickle cell disease suffering from osteomyelitis. Randomised controlled trials are needed to establish the optimum antibiotic treatment for this condition.

Serum Melatonin Level and Oxidative Stress In Sickle Cell Anemia

by Edis Belini Junior

This study evaluated serum melatonin levels in patients with sickle cell anemia (SCA) and compared the results to... more This study evaluated serum melatonin levels in patients with sickle cell anemia (SCA) and compared the results to lipid peroxidation by determining thiobarbituric acid-reactive substances (TBARS) and Trolox equivalent antioxidant capacity (TEAC). The group studied was composed of 15 SCA patients and 24 subjects without hemoglobinopathies. The average melatonin level was significantly reduced in the SCA patients (p<0.001) when compared to the control group. The SCA patients showed significantly higher values for TBARS and TEAC when compared to values obtained for the control group (p<0.001 and p<0.01). Results from the correlation analysis in the SCA group were not statistically significant for any parameters except for TBARS and TEAC levels, which had a positive correlation (r=0.51; p=0.04), suggesting the participation of melatonin in antioxidant defense. The use of melatonin could be a possible therapeutic target for improving antioxidant defense and to reduce oxidative damage, alleviating symptoms associated with SCA

The Xmnl polymorphic site 5 ' to the gene G gamma in a Brazilian patient with sickle cell anaemia - fetal haemoglobin concentration, haematology and clinical features

by Edis Belini Junior

We report a 20-year-old female with sickle cell anaemia and with an HbF concentration of 15.8%. The patient was not... more We report a 20-year-old female with sickle cell anaemia and with an HbF concentration of 15.8%. The patient was not using hydroxyurea and was not receiving regular blood transfusions. The patient never had chronic manifestations of sickle cell anaemia, only pain crises of a mild intensity. After laboratory tests, we found that she was homozygous for HbS with the Bantu/atypical haplotype, and was heterozygous for the Xmnl site. The influence of the Xmnl site on the expression of HbF can explain the amelioration in clinical features in this haplotype association in a case of sickle cell anaemia

Thesis Abstract-Oxidative stress in sickle cell patients: influence of haplotypes and specific medication

by Edis Belini Junior

Al Arrayed A, Al Hajeri A. Client’s Satisfaction of the Premarital Counseling Service in Bahrain. Bahrain Medical Bulletin. 2009. 31(3):15-22.

by Amani Al Hajeri

دراسة الرضا عن خدمة الفحص قبل الزواج لدى المراجعين في البحرين

Al Arrayed S, Al Hajeri A. Premarital genetic counseling: A new law in the Kingdom of Bahrain, Journal of Health, Social and Environmental Issues. 2005;6 (2): 31-34.

by Amani Al Hajeri

الفحص قبل الزواج وقانون جديد في البحرين

Al Hajeri AA, Fedorowicz Z, Omran A, Tadmouri GO. Piracetam for reducing the incidence of painful sickle cell disease crises. Cochrane Database of Systematic Reviews 2007, Issue 2. Art. No.: CD006111. DOI: 10.1002/14651858.CD006111.pub2.

by Amani Al Hajeri

A Al Hajeri, GR Serjeant, Z Fedorowicz. Inhaled nitric oxide for acute chest syndrome in people with sickle cell disease. Cochrane Database of Systematic Reviews 2008, Issue 1. Art. No.: CD006957. DOI: 10.1002/14651858.CD006957.

by Amani Al Hajeri

العلاج باكسيد النيتروجين لمتلازمة الصدر الحادة في المصابين بفقر الدم المنجلي -السكلر

Public awareness of sickle cell disease in Bahrain

by Amani Al Hajeri

دراسة عن مدى وعي العامة بمرض فقر الدم المنجلي (السكلر) في البحرين

for citation: Al Arrayed A, Al Hajeri A. Public awareness of sickle cell disease in Bahrain. Ann Saudi Med. 2010; 30(4):284-88.

Background and Objectives : Previous studies that have assessed patient awareness of the management of sickle cell... more Background and Objectives : Previous studies that have assessed patient awareness of the management of sickle cell disease (SCD) indicated a lack of awareness of the disease and possibly a need for more public education. Therefore, we measured public awareness in Bahrain of SCD.
Methods : The study was conducted from December 2006 to February 2007. A questionnaire was distributed among 2000 persons selected from among the general public. The participants had face-to-face interviews with either a health professional or a trained interviewer.
Results : Most (93%) had heard of SCD and 89% knew that it can be diagnosed by a blood test, but 51% did not know the prevalence of SCD in Bahrain. Eighty-four percent recognized it as a hereditary disorder and 72% said that it can skip generations. Females showed better knowledge than males and married persons seems to know more about SCD than unmarried ones.
Conclusion : There is a good level of knowledge about SCD among the public, though some of the respondents were confused about the difference between the carrier state of a disease and the disease itself. There is wide acceptance and appreciation of the SCD prevention campaigns being conducted in Bahrain, such as the premarital service and the student screening program.