Cochrane Db Syst Rev
-
Cochrane Db Syst Rev · Jun 2019
Enzyme replacement therapy with laronidase (Aldurazyme®) for treating mucopolysaccharidosis type I.
Mucopolysaccharidosis type I can be classified as three clinical sub-types; Hurler syndrome, Hurler-Scheie syndrome and Scheie syndrome, with the scale of severity being such that Hurler syndrome is the most severe and Scheie syndrome the least severe. It is a rare, autosomal recessive disorder caused by a deficiency of alpha-L-iduronidase. Deficiency of this enzyme results in the accumulation of glycosaminoglycans within the tissues. The clinical manifestations are facial dysmorphism, hepatosplenomegaly, upper airway obstruction, skeletal deformity and cardiomyopathy. If Hurler syndrome is left untreated, death ensues by adolescence. There are more attenuated variants termed Hurler-Scheie or Scheie syndrome, with those affected potentially not presenting until adulthood. Enzyme replacement therapy has been used for a number of years in the treatment of Hurler syndrome, although the current gold standard would be a haemopoietic stem cell transplant in those diagnosed by 2.5 years of age. This is an updated version of the original Cochrane Review published in 2013 and previously updated in 2015. ⋯ The current evidence demonstrates that laronidase is effective when compared to placebo in the treatment of mucopolysaccharidosis type I. The included study was comprehensive, with few participants and of low quality. The study included all of the key outcome measures we wished to look at. It demonstrated that laronidase is efficacious in relation to reducing biochemical parameters (reduced urine glycosaminoglycan excretion) and improved functional capacity as assessed by forced vital capacity and the six-minute-walk test. In addition glycosaminoglycan storage was reduced as ascertained by a reduction in liver volume. Laronidase appeared to be safe and, while antibodies were generated, these titres were reducing by the end of the study. More studies are required to determine long-term effectiveness and safety and to assess the impact upon quality of life. Enzyme replacement therapy with laronidase can be used pre- and peri-haemopoietic stem cell transplant, which is now the gold standard treatment in those individuals diagnosed under 2.5 years of age. We do not anticipate any further trials to be undertaken and therefore do not plan to update this review.
-
Cochrane Db Syst Rev · Jun 2019
Meta AnalysisProbiotics for preventing acute otitis media in children.
Acute otitis media (AOM), or acute middle ear infection, is one of the most frequently occurring childhood diseases, and the most common reason given for prescribing antibiotics in this age group. Guidelines often recommend antibiotics as first-line treatment for severe AOM. However, antibiotics also lead to antibiotic resistance, so preventing episodes of AOM is an urgent priority. ⋯ Probiotics may prevent AOM in children not prone to AOM, but the inconsistency of the subgroup analyses suggests caution in interpreting these results. Probiotics decreased the proportion of children taking antibiotics for any infection. The proportion of children experiencing adverse events did not differ between the probiotic and comparator groups. The optimal strain, duration, frequency, and timing of probiotic administration still needs to be established.
-
Cochrane Db Syst Rev · Jun 2019
Risk of ovarian cancer in women treated with ovarian stimulating drugs for infertility.
This is an updated version of the original Cochrane Review published in the Cochrane Library in 2013 (Issue 8) on the risk of ovarian cancer in women using infertility drugs when compared to the general population or to infertile women not treated. The link between fertility drugs and ovarian cancer remains controversial. ⋯ Since the last version of this review, only a few new relevant studies have provided additional findings with supporting evidence to suggest that infertility drugs may increase the risk of ovarian cancer slightly in subfertile women treated with infertility drugs when compared to the general population or to subfertile women not treated. The risk is slightly higher in nulliparous than in multiparous women treated with infertility drugs, and for borderline ovarian tumours. However, few studies have been conducted, the number of cancers is very small, and information on the dose or type of fertility drugs used is insufficient.
-
Cochrane Db Syst Rev · Jun 2019
Meta AnalysisNutritional interventions for preventing stunting in children (birth to 59 months) living in urban slums in low- and middle-income countries (LMIC).
Nutritional interventions to prevent stunting of infants and young children are most often applied in rural areas in low- and middle-income countries (LMIC). Few interventions are focused on urban slums. The literature needs a systematic assessment, as infants and children living in slums are at high risk of stunting. Urban slums are complex environments in terms of biological, social, and political variables and the outcomes of nutritional interventions need to be assessed in relation to these variables. For the purposes of this review, we followed the UN-Habitat 2004 definitions for low-income informal settlements or slums as lacking one or more indicators of basic services or infrastructure. ⋯ All the nutritional interventions reviewed had the potential to decrease stunting, based on evidence from outside of slum contexts; however, there was no evidence of an effect of the interventions included in this review (very low- to moderate-certainty evidence). Challenges linked to urban slum programming (high mobility, lack of social services, and high loss of follow-up) should be taken into account when nutrition-specific interventions are proposed to address LBW and stunting in such environments. More evidence is needed of the effects of multi-sectorial interventions, combining nutrition-specific and sensitive methods and programmes, as well as the effects of 'up-stream' practices and policies of governmental, non-governmental organisations, and the business sector on nutrition-related outcomes such as stunting.
-
In most pregnancies that miscarry, arrest of embryonic or fetal development occurs some time (often weeks) before the miscarriage occurs. Ultrasound examination can reveal abnormal findings during this phase by demonstrating anembryonic pregnancies or embryonic or fetal death. Treatment has traditionally been surgical but medical treatments may be effective, safe, and acceptable, as may be waiting for spontaneous miscarriage. This is an update of a review first published in 2006. ⋯ Available evidence from randomised trials suggests that medical treatment with vaginal misoprostol may be an acceptable alternative to surgical evacuation or expectant management. In general, side effects of medical treatment were minor, consisting mainly of nausea and diarrhoea. There were no major differences in effectiveness between different routes of administration. Treatment satisfaction was addressed in only a few studies, in which the majority of women were satisfied with the received intervention. Since the quality of evidence is low or very low for several comparisons, mainly because they included only one or two (small) trials; further research is necessary to assess the effectiveness, safety and side effects, optimal route of administration and dose of different medical treatments for early fetal death.