Cochrane Db Syst Rev
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Cochrane Db Syst Rev · Mar 2020
Review Meta AnalysisGlucocorticoid replacement regimens for treating congenital adrenal hyperplasia.
Congenital adrenal hyperplasia (CAH) is an autosomal recessive condition which leads to glucocorticoid deficiency and is the most common cause of adrenal insufficiency in children. In over 90% of cases, 21-hydroxylase enzyme deficiency is found which is caused by mutations in the 21-hydroxylase gene. Managing individuals with CAH due to 21-hydroxylase deficiency involves replacing glucocorticoids with oral glucocorticoids (including prednisolone and hydrocortisone), suppressing adrenocorticotrophic hormones and replacing mineralocorticoids to prevent salt wasting. During childhood, the main aims of treatment are to prevent adrenal crises and to achieve normal stature, optimal adult height and to undergo normal puberty. In adults, treatment aims to prevent adrenal crises, ensure normal fertility and to avoid the long-term consequences of glucocorticoid use. Current glucocorticoid treatment regimens can not optimally replicate the normal physiological cortisol level and over-treatment or under-treatment is often reported. ⋯ There are currently limited trials comparing the efficacy and safety of different glucocorticoid replacement regimens for treating 21-hydroxylase deficiency CAH in children and adults and we were unable to draw any firm conclusions based on the evidence that was presented in the included trials. No trials included long-term outcomes such as quality of life, prevention of adrenal crisis, presence of osteopenia, presence of testicular or ovarian adrenal rest tumours, subfertility and final adult height. There were no trials examining a modified-release formulation of HC or use of 24-hour circadian continuous subcutaneous infusion of hydrocortisone. As a consequence, uncertainty remains about the most effective form of glucocorticoid replacement therapy in CAH for children and adults. Future trials should include both children and adults with CAH. A longer duration of follow-up is required to monitor biochemical and clinical outcomes.
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Cochrane Db Syst Rev · Mar 2020
ReviewAntibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis.
Stenotrophomonas maltophilia is one of the most common emerging multi-drug resistant organisms found in the lungs of people with cystic fibrosis and its prevalence is increasing. Chronic infection with Stenotrophomonas maltophilia has recently been shown to be an independent predictor of pulmonary exacerbation requiring hospitalization and antibiotics. However, the role of antibiotic treatment of Stenotrophomonas maltophilia infection in people with cystic fibrosis is still unclear. This is an update of a previously published review. ⋯ This review did not identify any evidence regarding the effectiveness of antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis. Until such evidence becomes available, clinicians need to use their clinical judgement as to whether or not to treat Stenotrophomonas maltophilia infection in people with cystic fibrosis. Randomized clinical trials are needed to address these unanswered clinical questions.
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Cochrane Db Syst Rev · Mar 2020
Review Meta AnalysisSustained versus standard inflations during neonatal resuscitation to prevent mortality and improve respiratory outcomes.
At birth, infants' lungs are fluid-filled. For newborns to have a successful transition, this fluid must be replaced by air to enable gas exchange. Some infants are judged to have inadequate breathing at birth and are resuscitated with positive pressure ventilation (PPV). Giving prolonged (sustained) inflations at the start of PPV may help clear lung fluid and establish gas volume within the lungs. ⋯ Our meta-analysis of nine studies shows that sustained lung inflation without chest compression was not better than intermittent ventilation for reducing mortality in the delivery room (low-quality evidence ‒ GRADE) or during hospitalisation (moderate-quality evidence ‒ GRADE), which were the primary outcomes of this review. However, the single largest study, which was well conducted and had the greatest number of enrolled infants, was stopped early for higher mortality rate in the sustained inflation group. When considering secondary outcomes, such as rate of intubation, rate or duration of respiratory support, or bronchopulmonary dysplasia, we found no benefit of sustained inflation over intermittent ventilation (moderate-quality evidence ‒ GRADE). Duration of mechanical ventilation was shortened in the SLI group (low-quality evidence ‒ GRADE); this result should be interpreted cautiously, however, as it might have been influenced by study characteristics other than the intervention. There is no evidence to support the use of sustained inflation based on evidence from our review.
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Cochrane Db Syst Rev · Mar 2020
Review Meta AnalysisPsychological interventions for people with hemophilia.
Managing hemophilia is challenging both in terms of medical treatment and its broad impact on many aspects of the individual's life, including self-perception. Several psychosocial issues are potentially relevant in the clinical management of hemophilia, including it being a chronic and incurable condition; e.g. people with hemophilia must adapt to optimally interact with peers and to practice sports - even choosing a sport represents an issue for perceived limitations, expectations and cultural influences on the individual and their family. People with hemophilia can react by denying their condition and its manifestations and not adhering to treatment. Due to the complexity of relationships surrounding genetic diseases, parents and relatives may have their own issues that contribute to making life easier or more difficult for the person with hemophilia. Anxiety, sadness and depression resulting in mental health disorders are reported in this population and may influence quality of life (QoL) depending on cultural background, religious beliefs, family support and other variables. ⋯ Not all of the seven included trials analysed the effects of the interventions on our primary outcomes (mood and personal well-being, coping strategies and QoL). Three trials were conducted in the 1970s and 1980s using techniques of auto-hypnosis or relaxation and, in accordance with the needs and therapeutic possibilities of the time, they focused on secondary outcomes, e.g. frequency of bleeding (physical health) and adherence to the intervention. The four newer trials assessed psycho-educational interventions all mediated by the use of technologies (DVD or computer) and often created according to age needs of the target group. In these cases, attention was shifted to our pre-defined primary outcomes. This review has identified low- and very low-certainty evidence, prompting caution in its interpretation. The major problem we encountered was the heterogeneity of trial designs, of interventions and of outcome measures used across the trials. We strongly suggest that researchers consider developing a core outcome set to streamline future research; randomization was proven to be safe and acceptable, and blinding should be considered for those assessing patient-reported outcomes.
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Cochrane Db Syst Rev · Mar 2020
Review Meta AnalysisIntense pulsed light (IPL) therapy for the treatment of meibomian gland dysfunction.
Meibomian gland dysfunction (MGD) is the major cause of evaporative dry eye disease, which is the more prevalent form of dry eye disease. Intense pulsed light (IPL) therapy, involving treatment of the skin near the eyelids, has emerged as a potential treatment for MGD. ⋯ This systematic review finds a scarcity of RCT evidence relating to the effectiveness and safety of IPL as a treatment for MGD. Whether IPL is of value for modifying the symptoms or signs of evaporative dry eye disease is currently uncertain. Due to a lack of comprehensive reporting of adverse events, the safety profile of IPL in this patient population is also unclear. The current limitations in the evidence base should be considered by clinicians using this intervention to treat MGD, and outlined to individuals potentially undergoing this procedure with the intent of treating dry eye disease. The results of the 14 RCTs currently in progress will be of major importance for establishing a more definitive answer regarding the effectiveness and safety of IPL for treating MGD. We intend to update this review when results from these trials become available.