Hematopoietic stem cell transplantation for people with β-thalassaemia.

Background: Thalassaemia is an autosomal recessive blood disorder, caused by mutations in globin genes or their regulatory regions, resulting in a reduced rate of synthesis of one of the globin chains that make up haemoglobin. In β-thalassaemia there is an underproduction of β-globin chains combined with excess of free α-globin chains. The excess free α-globin chains precipitate in red blood cells, leading to their increased destruction (haemolysis) and ineffective erythropoiesis.

Vitamin K supplementation for cystic fibrosis.

Background: Malabsorption and deficiency of fat-soluble vitamins K may occur in cystic fibrosis, a genetic disorder affecting multiple organs. Vitamin K is known to play an important role in both blood coagulation and bone formation, hence the role of supplementation of vitamin K in this category needs to be reviewed. This is an updated version of the review.

Percutaneous transluminal angioplasty for treatment of chronic cerebrospinal venous insufficiency (CCSVI) in people with multiple sclerosis.

Background: Multiple sclerosis (MS) is a leading cause of neurological disability in young adults. The most widely accepted hypothesis regarding its pathogenesis is that it is an immune-mediated disease. It has been hypothesised that intraluminal defects, compression, or hypoplasia in the internal jugular or azygos veins may be important factors in the pathogenesis of MS.

Vitamin D for the management of multiple sclerosis.

Background: This review is an update of a previously published review, "Vitamin D for the management of multiple sclerosis" (published in the Cochrane Library; 2010, Issue 12). Multiple sclerosis (MS) is characterised by inflammation, demyelination, axonal or neuronal loss, and astrocytic gliosis in the central nervous system (CNS), which can result in varying levels of disability. Some studies have provided evidence showing an association of MS with low levels of vitamin D and benefit derived from its supplementation.

Vitamin K supplementation for cystic fibrosis.

Background: Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation.

Hematopoietic stem cell transplantation for people with ß-thalassaemia major.

Background: Thalassemia is an inherited autosomal recessive blood disorder, caused by mutations in globin genes or their regulatory regions. This results in a reduced rate of synthesis of one of the globin chains that make up haemoglobin. In ß-thalassaemia major there is an underproduction of ß-globin chains combined with excess of free α-globin chains.

Neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis.

Background: Cystic fibrosis is the most common, life-threatening, recessively inherited disease of Caucasian populations. It is a multisystem disorder caused by a mutation in the gene encoding the cystic fibrosis transmembrane conductance regulator protein which is important in producing sweat, digestive juices and mucus.The impaired or absent function of this protein results in the production of viscous mucus within the lungs and an environment that is susceptible to chronic airway obstruction and pulmonary colonization by a range of pathogenic bacteria.

Vitamin K supplementation for cystic fibrosis.

Background: Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation.

Hematopoietic stem cell transplantation for people with ß-thalassaemia major.

Background: Thalassemia is an inherited blood disorder, caused by mutations in regulatory genes and transmitted as an autosomal recessive disorder, which results in a reduced rate of synthesis of one of the globin chains that make up haemoglobin. In ß-thalassaemia major there is an underproduction of ß-globin chains combined with excess of free α-globin chains. The excess free α-globin chains damage the red blood cell membranes, leading to their destruction and a phenomenon termed ineffective erythropoiesis.

Neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis.

Background: Cystic fibrosis is the most common, life-threatening, recessively inherited disease of Caucasian populations. It is a multisystem disorder caused by a mutation in the gene encoding the cystic fibrosis transmembrane conductance regulator protein which is important in producing sweat, digestive juices and mucus.The impaired or absent function of this protein results in the production of viscous mucus within the lungs and an environment that is susceptible to chronic airway obstruction and pulmonary colonization by a range of pathogenic bacteria.

Vitamin K supplementation for cystic fibrosis.

Background: Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation.

Percutaneous transluminal angioplasty for treatment of chronic cerebrospinal venous insufficiency (CCSVI) in multiple sclerosis patients.

Background: Multiple sclerosis (MS) is a leading cause of neurological disability in young adults. The most widely accepted hypothesis regarding its pathogenesis is that it is an immune-mediated disease. It has been hypothesised more recently that chronic venous congestion may be an important factor in the pathogenesis of MS.

Routine neonatal circumcision for the prevention of urinary tract infections in infancy.

Background: Neonatal circumcision is a fairly common surgical procedure that may be carried out for medical reasons, one of them being prevention of urinary tract infections (UTI) in male infants. Circumcision could help in reducing the incidence of UTI by reducing periurethral bacterial colonization, which is accepted as a potential risk factor in UTI. Evidence is needed to inform the benefits or harm for the routine use of this intervention.

Beta2-adrenoceptor agonists for dysmenorrhoea.

Background: Dysmenorrhoea is a common gynaecological complaint that can affect as many as 50% of premenopausal women, 10% of whom suffer severely enough to be rendered incapacitated for one to three days during each menstrual cycle. Primary dysmenorrhoea is where women suffer from menstrual pain but lack any pathology in their pelvic anatomy. Beta2-adrenoceptor agonists have been used in the treatment of women with primary dysmenorrhoea but their effects are unclear.

An AMSTAR assessment of the methodological quality of systematic reviews of oral healthcare interventions published in the Journal of Applied Oral Science (JAOS).

Background: Systematic reviews are not an assembly of anecdotes but a distillation of current best available evidence on a particular topic and as such have an important role to play in evidence-based healthcare. A substantial proportion of these systematic reviews focus on interventions, and are able to provide clinicians with the opportunity to understand and translate the best available evidence on the effects of these healthcare interventions into clinical practice. The importance of systematic reviews in summarising and identifying the gaps in evidence which might inform new research initiatives is also widely acknowledged.

Hematopoietic stem cell transplantation for people with ß-thalassaemia major.

Background: Thalassemia is an inherited blood disorder, caused by mutations in regulatory genes and transmitted as an autosomal recessive disorder, which results in a reduced rate of synthesis of one of the globin chains that make up haemoglobin. In ß-thalassaemia major there is an underproduction of ß-globin chains combined with excess of free α-globin chains. The excess free α-globin chains damage the red blood cell membranes, leading to their destruction and a phenomenon termed ineffective erythropoiesis.

Antiemetics for reducing vomiting related to acute gastroenteritis in children and adolescents.

Background: Vomiting is a common manifestation of acute gastroenteritis in children and adolescents. When untreated it can be a hindrance to oral rehydration therapy, which is the cornerstone in the management of acute gastroenteritis. Evidence is needed concerning the safety and efficacy of antiemetic use for vomiting in acute gastroenteritis in children.

Vitamin K supplementation for cystic fibrosis.

Background: Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation.

Vitamin D for the management of multiple sclerosis.

Background: Multiple sclerosis is a disease of the central nervous system characterized by demyelination of the nerve sheaths which can result in varying levels of disability. Disease occurrence and progression are considered by some to be associated with low serum levels of vitamin D. Studies investigating vitamin D supplementation in MS patients have illustrated a noticeable improvement in the course of the disease.

Neuraminidase inhibitors for the treatment of influenza infection in people with cystic fibrosis.

Background: Cystic fibrosis is the most common, life-threatening, recessively inherited disease of Caucasian populations. It is a multisystem disorder caused by a mutation in the gene encoding the cystic fibrosis transmembrane conductance regulator protein which is important in producing sweat, digestive juices and mucus.The impaired or absent function of this protein results in the production of viscous mucus within the lungs and an environment that is susceptible to chronic airway obstruction and pulmonary colonization by a range of pathogenic bacteria.