Cochrane Db Syst Rev
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Cochrane Db Syst Rev · Sep 2021
ReviewEnzyme replacement therapy with galsulfase for mucopolysaccharidosis type VI.
Mucopolysaccharidosis type VI (MPS VI) or Maroteaux-Lamy syndrome is a rare genetic disorder caused by the deficiency of arylsulphatase B. The resultant accumulation of dermatan sulphate causes lysosomal damage. The clinical symptoms are related to skeletal dysplasia (i.e. short stature and degenerative joint disease). Other manifestations include cardiac disease, impaired pulmonary function, ophthalmological complications, hepatosplenomegaly, sinusitis, otitis, hearing loss and sleep apnea. Intellectual impairment is generally absent. Clinical manifestation is typically by two or three years of age; however, slowly progressive cases may not present until adulthood. Enzyme replacement therapy (ERT) with galsulfase is considered a new approach for treating MPS VI. ⋯ One study was included involving 39 participants who received either ERT with galsulfase (recombinant human arylsulphatase B) or placebo. This small study was considered overall to have an unclear risk of bias in relation to the design and implementation of the study, since the authors did not report how both the allocation generation and concealment were performed. Given the very low certainty of the evidence, we are uncertain whether at 24 weeks there was a difference between groups in relation to the 12-minute walk test, mean difference (MD) of 92.00 meters (95% confidence interval (CI) 11.00 to 172.00), or the three-minute stair climb, MD 5.70 (95% CI -0.10 to 11.50). In relation to respiratory tests, we are uncertain whether galsulfase makes any difference as compared to placebo in forced vital capacity in litres (FVC (L) (absolute change in baseline), given the very low certainty of the evidence. Cardiac function was not reported in the included study. We found that galsulfase, as compared to placebo, may decrease urinary glycosaminoglycan levels at 24 weeks, MD -227.00 (95% CI -264.00 to -190.00) (low-certainty evidence). We are uncertain whether there are differences between the galsulfase and placebo groups in relation to adverse events (very low-certainty evidence). In general, the dose of galsulfase was well tolerated and there were no differences between groups. These events include drug-related adverse events, serious and severe adverse events, those during infusion, drug-related adverse events during infusion, and deaths. More infusion-related reactions were observed in the galsulfase group and were managed with interruption or slowing of infusion rate or administration of antihistamines or corticosteroids drugs. No deaths occurred during the study. AUTHORS' CONCLUSIONS: The results of this review are based only on one small study (a 24-week randomised phase of the study and prior to the open-label extension). We are uncertain whether galsulfase is more effective than placebo, for treating people with MPS VI, in relation to the 12-minute walk test or the three-minute stair climb, as the certainty of the evidence has been assessed as very low. We found that galsulfase may reduce urinary glycosaminoglycans levels. We are also uncertain whether there are any differences between treatment groups in relation to cardiac or pulmonary functions, liver or spleen volume, overnight apnea-hypopnea, height and weight, quality of life and adverse effects. Further studies are needed to obtain more information on the long-term effectiveness and safety of ERT with galsulfase.
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Cochrane Db Syst Rev · Sep 2021
ReviewTreatments for seizures in catamenial (menstrual-related) epilepsy.
This is an updated version of a Cochrane Review previously published in 2019. Catamenial epilepsy describes worsening seizures in relation to the menstrual cycle and may affect around 40% of women with epilepsy. Vulnerable days of the menstrual cycle for seizures are perimenstrually (C1 pattern), at ovulation (C2 pattern), and during the luteal phase (C3 pattern). A reduction in progesterone levels premenstrually and reduced secretion during the luteal phase is implicated in catamenial C1 and C3 patterns. A reduction in progesterone has been demonstrated to reduce sensitivity to the inhibitory neurotransmitter in preclinical studies, hence increasing risk of seizures. A pre-ovulatory surge in oestrogen has been implicated in the C2 pattern of seizure exacerbation, although the exact mechanism by which this surge increases risk is uncertain. Current treatment practices include the use of pulsed hormonal (e.g. progesterone) and non-hormonal treatments (e.g. clobazam or acetazolamide) in women with regular menses, and complete cessation of menstruation using synthetic hormones (e.g. medroxyprogesterone (Depo-Provera) or gonadotropin-releasing hormone (GnRH) analogues (triptorelin and goserelin)) in women with irregular menses. Catamenial epilepsy and seizure exacerbation is common in women with epilepsy. Women may not receive appropriate treatment for their seizures because of uncertainty regarding which treatment works best and when in the menstrual cycle treatment should be taken, as well as the possible impact on fertility, the menstrual cycle, bone health, and cardiovascular health. This review aims to address these issues to inform clinical practice and future research. ⋯ This review provides very low-certainty evidence of no treatment difference between norethisterone and placebo, and moderate- to low-certainty evidence of no treatment difference between progesterone and placebo for catamenial epilepsy. However, as all the included studies were underpowered, important clinical effects cannot be ruled out. Our review highlights an overall deficiency in the literature base on the effectiveness of a wide range of other hormonal and non-hormonal interventions currently being used in practice, particularly for those women who do not have regular menses. Further clinical trials are needed in this area.
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Cochrane Db Syst Rev · Sep 2021
ReviewEffects of consumers and health providers working in partnership on health services planning, delivery and evaluation.
Health services have traditionally been developed to focus on specific diseases or medical specialties. Involving consumers as partners in planning, delivering and evaluating health services may lead to services that are person-centred and so better able to meet the needs of and provide care for individuals. Globally, governments recommend consumer involvement in healthcare decision-making at the systems level, as a strategy for promoting person-centred health services. However, the effects of this 'working in partnership' approach to healthcare decision-making are unclear. Working in partnership is defined here as collaborative relationships between at least one consumer and health provider, meeting jointly and regularly in formal group formats, to equally contribute to and collaborate on health service-related decision-making in real time. In this review, the terms 'consumer' and 'health provider' refer to partnership participants, and 'health service user' and 'health service provider' refer to trial participants. This review of effects of partnership interventions was undertaken concurrently with a Cochrane Qualitative Evidence Synthesis (QES) entitled Consumers and health providers working in partnership for the promotion of person-centred health services: a co-produced qualitative evidence synthesis. ⋯ The effects of consumers and providers working in partnership as an intervention, or as part of a multi-component intervention, are uncertain, due to a lack of high-quality evidence and/or due to a lack of studies. Further well-designed RCTs with a clear focus on assessing outcomes directly related to partnerships for patient-centred health services are needed in this area, which may also benefit from mixed-methods and qualitative research to build the evidence base.
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Cochrane Db Syst Rev · Sep 2021
ReviewRoux-en-Y versus Billroth-I reconstruction after distal gastrectomy for gastric cancer.
Gastric cancer is the fifth most common cancer diagnosed worldwide. Due to improved early detection rates of gastric cancer and technological advances in treatments, a significant improvement in survival rates has been achieved in people with cancer undergoing gastrectomy. Subsequently, there has been increasing emphasis on postgastrectomy syndrome (e.g. fullness, delayed emptying, and cold sweat, amongst others) and quality of life postsurgery. However, it is uncertain which types of reconstruction result in better outcomes postsurgery. ⋯ The evidence suggests that there is little to no difference between Roux-en-Y and Billroth-I reconstruction for the outcome health-related quality of life. The evidence for the effect of Roux-en-Y versus Billroth-I reconstruction on the incidence of anastomotic leakage is very uncertain as the incidence of this outcome was low. Although the certainty of evidence was low, we found some possibly clinically meaningful differences between Roux-en-Y and Billroth-I reconstruction for short-term outcomes. Roux-en-Y reconstruction probably reduces the incidence of bile reflux into the remnant stomach compared to Billroth-I reconstruction. Billroth-I reconstruction may shorten postoperative hospital stay compared to Roux-en-Y reconstruction, but the evidence is very uncertain. Billroth-I reconstruction may reduce postoperative overall morbidity compared to Roux-en-Y reconstruction. Future trials should include long-term follow-up of health-related quality of life and body weight loss.
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Cochrane Db Syst Rev · Sep 2021
ReviewLamotrigine in the maintenance treatment of bipolar disorder.
Bipolar disorder is a chronic mental disorder with repetitive mania/hypomania as well as depressive episodes, which eventually results in marked impairment in overall functioning and health-related quality of life. A worldwide prevalence rate of 2.4% has been reported. The risk of suicide is higher in people with bipolar disorder than those with other mental disorders. Therefore, effective management of bipolar disorder in the maintenance period is warranted to minimize the risk of relapse or recurrence. Although lithium has been the standard treatment of bipolar disorder for many years, it is associated with adverse effects and teratogenicity. Lamotrigine is approved to be expected for prevention of recurrence for the maintenance treatment of bipolar disorder. In addition, lamotrigine is as effective as lithium. Therefore, we performed a systematic review to confirm the efficacy and safety of lamotrigine in the maintenance treatment of bipolar disorder. ⋯ Low- to moderate-certainty evidence collectively suggests that lamotrigine may be superior to placebo as a treatment modality for bipolar disorder. In comparison to lithium, people with bipolar disorder seem to tolerate lamotrigine better in the long run; however, the demonstrated efficacy in the maintenance of bipolar disorder was similar between the two groups.