Cochrane

Review question We reviewed the evidence for the effectiveness and safety of statins in children with inherited high blood cholesterol. Background Familial hypercholesterolemia is an inherited disease in which the blood cholesterol level is high. Vascular disease, i.e. furring up of the blood vessels, often occurs at an earlier age than usual, especially ...

Review question We reviewed the evidence available to see if rituximab is effective and safe when treating clotting factor inhibitors in people with severe hemophilia. This is an update of a previously published Cochrane Review. Background Hemophilia A and B are inherited conditions in which there is either reduced levels (or none at all) of factor ...

Review question We reviewed the evidence about the effect of testing antibiotics in combination for acute airway infections in people with long-term (chronic) infection with Pseudomonas aeruginosa. Background The main cause of death in people with cystic fibrosis is chronic lung infection. People with cystic fibrosis now live longer due to the aggressive ...

We reviewed the evidence for the use of oral supplements to increase calorie intake in people with cystic fibrosis. Background Cystic fibrosis affects many organs, including the digestive system, and can lead to food not being absorbed as it should be, which in turn leads to growth problems. Children with cystic fibrosis need more energy than other ...

Review question We reviewed the evidence about the effect of vibrating devices (e.g. Flutter, acapella, cornet, Quake®, intrapulmonary percussive ventilation, high frequency chest wall oscillators (e.g. Vest®), VibraLung® and MetaNeb®) to help people with cystic fibrosis clear their airways of mucus. This is an update of a previously published review. Background People ...

Review question What is the best way of using antibiotics to eliminate lung infections caused by a germ called Pseudomonas aeruginosa in people with cystic fibrosis? Background Cystic fibrosis is an inherited condition where the airways often become blocked with mucus. It is associated with chest infections, which can lead to progressive breathing ...

Review question What is the effect of hydroxyurea on clinical outcomes (changes in pain crises, life-threatening illnesses, survival, haemoglobin levels, quality of life and side effects) in people with sickle cell disease (SCD) of any genotype? Background SCD is an inherited genetic disorder that creates problems with haemoglobin (the substance ...

Review question We reviewed the evidence about the benefits and adverse effects of giving regular antibiotics to people with cystic fibrosis to prevent infection with a germ called Staphylococcus aureus. Background Cystic fibrosis blocks the airways with mucus and causes frequent airway infections. These can lead to death from breathing failure. ...

Review question We reviewed the evidence of the effect of intravenous (given into a vein) magnesium and oral (taken by mouth) magnesium on the frequency of painful crises (sickle cell crises with severe pain due to blockages of the blood supply to bones, joints, lungs, liver, spleen, kidney, eye or central nervous system), length of hospital stay ...

Protocol: This review aims to evaluate the effectiveness and safety of laxative agents of differing types for preventing DIOS (complete and incomplete) in children and adults with CF. If possible, we aim to assess the optimal laxative regimen by comparing the evidence for osmotic laxatives, stimulant laxatives and mucolytic agents.