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Biomedical subjects

Sally C Davies

Publications and source records attributed to Sally C Davies.

10 recordsLinked to original sources
PubMed2005

Surgery in sickle cell disease.

Persons with sickle cell disease (SCD) are more likely to undergo surgery than are the general population during their lifetime. For example, cholecystectomy as a consequence of gallstones is more frequent in persons with SCD, as is hip arthroplasty in younger people as a result of avascular necrosis of the femoral head. Because surgery exposes patients to many of the factors that are known to precipitate red blood cell sickling, persons with SCD undergoing surgery require meticulous clinical care to prevent perioperative sickle cell-related complications. Even with meticulous care, approximately 25% to 30% of patients will have a postoperative complication. This article provides readers with information about the role of surgery in SCD and the measures that should be taken to ensure patients are well cared for in the perioperative period.

Anemia, Sickle Cell↗
PubMed2005

Cannabis use in sickle cell disease: a questionnaire study.

Cannabinoids are increasingly being considered for the management of various painful conditions, and could be considered as an option for treating acute pain in sickle cell disease (SCD). The objective of this study was to determine the extent of use of cannabis in the community for pain and other symptom relief, and its side effects during self-administration in patients with SCD. Patients attending Central Middlesex Hospital in London were invited to complete a structured self-administered anonymous questionnaire. Eighty-six young adults with HbSS, HbSC and HbSbetathalassaemia disease (median age 30 years) participated in the study. Results showed that 31 (36%) had used cannabis in the previous 12 months to relieve symptoms associated with SCD. The main route in all but two patients was by smoking. The main reasons for use were to reduce pain in 52%, and to induce relaxation or relieve anxiety and depression in 39%. Symptoms related to sedation and mood effects were reported in 77% of patients. The majority of patients (58%) expressed their willingness to participate in studies of cannabis as a medicine. We conclude that research in the use of cannabinoids for pain relief in SCD would be both important and acceptable to adult patients.

Acute Disease↗
PubMed2003

The role of hydroxyurea in the management of sickle cell disease.

Sickle cell disease (SCD) is one of the most common genetic diseases with some 250,000 new births each year. Most patients suffer intermittent pain crises and life-threatening events while life expectancy is considerably reduced. Until the last decade management was purely preventative or supportive aimed at symptom control. Apart from stem cell transplant, there is no cure but the oral chemotherapeutic drug hydroxyurea (HU) has now established a role in ameliorating the disease and improving life expectancy for most patients. There are side effects and risks of HU treatment in SCD but for moderate and severely affected patients, the benefits can be significant.

Anemia, Sickle Cell↗
PubMed2003

What haemophilia could learn from the sickle cell work in the International Cochrane Collaboration.

It is well recognized that individuals required to make informed decisions about healthcare interventions often do not have ready access to reliable reviews of the available evidence, with healthcare professionals, researchers, policy makers and consumers often overwhelmed by unmanageable amounts of information. Expert reviews go some way to presenting new information in a concise and readable format. However, these are generally not subject to the same scientific rigour as the actual primary research they summarize. The International Cochrane Collaboration was established to address this need through the development and publication of rigorous, peer-reviewed, well-performed systematic reviews. This article will briefly summarize the basic principles of the Cochrane Collaboration, primarily focusing on its work in the field of inherited haemoglobinopathies, in particular sickle cell disease, and highlight potential areas of interest for haemophilia.

Anemia, Sickle Cell↗
PubMed2002

A mathematical model predicts that calreticulin interacts with the endoplasmic reticulum Ca(2+)-ATPase.

A robust mathematical model developed from single cell calcium (Ca(2+)) dynamics has enabled us to predict the consequences of over-expression of endoplasmic reticulum-located chaperones. Model predictions concluded that calreticulin interacts with the lumenal domain of the sarcoplasmic and endoplasmic reticulum Ca(2+)-activated ATPase (SERCA) pump, altering pump affinity for Ca(2+) (K(1/2) switches from 247 to 431 nM) and hence generating Ca(2+) oscillations. Expression of calreticulin in the ER generated an average of six transient-decline oscillations during the Ca(2+) recovery phase, upon exposure to maximal levels of the agonist ATP. In contrast, normal cells produced a single Ca(2+) transient with few or no oscillations. By conditioning the model to experimental data, parameters for generation and decay of IP(3) and SERCA pump kinetics were determined. To elucidate the possible source of the oscillatory behavior three possible oscillators, 1) IP(3), 2) IP(3)R, and 3) SERCA pump, were investigated and parameters constrained by experimental data to produce the best candidate. Each of the three oscillators generated very good fits with experimental data. However, converting a normal exponential recovery to a transient-decline oscillator predicted that the SERCA pump is the most likely candidate for calreticulin-mediated Ca(2+) release, highlighting the role of this chaperone as a signal protein within the endoplasmic reticulum.

Calcium↗
PubMed2002

Patterns of mortality in sickle cell disease in adults in France and England.

INTRODUCTION: An understanding of the causes of death among patients with sickle cell disease may be informative for both epidemiology and pathogenesis. This information should aid anticipation of dangerous clinical conditions, counselling patients and design of preventive therapies. PATIENTS AND METHODS: All deaths known to four European sickle cell disease centres over a 10-year period were retrospectively analysed. The circumstances of death were classified as follows: (1) acute sickle related vaso-occlusion; (2) chronic organ failure related to sickle cell disease; (3) infection; and (4) miscellaneous causes. RESULTS: Sixty-one adult patients (mean age: 32+/-11 years) died during the study period. Twelve patients suddenly died at home; most of them exhibited symptoms of vaso-occlusion but in eight patients, the cause of death was unknown. The primary cause of death in the 53 evaluable patients was sickle related vaso-occlusion (27 out of 53; 51%) which manifested mainly by acute multiorgan failure (n=13) and acute chest syndrome (n=9). Ten of the 27 patients (37%) who died in these circumstances had an apparent mild disease before their deaths. Ten patients (19%) died of documented infection. Ten of the evaluable patients (19%) died of a chronic terminal visceral involvement related to sickle cell disease which was mainly liver cirrhosis. Four patients died by suicide or because of refusal of care and two patients died of iatrogenic complication. CONCLUSION: The primary cause of death in adults appears to be vaso-occlusive, even in patients with no overt organ-system failure. Our results emphasise that the circumstances of death in sickle cell disease are different between adults and children. The deaths among adults appear not to be easily assigned to a few preventable causes as they are in children.

Adolescent↗