scholarly journals Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis

Nutrients ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 2070 ◽  
Author(s):  
Fatma Ilgaz ◽  
Alex Pinto ◽  
Hülya Gökmen-Özel ◽  
Julio César Rocha ◽  
Esther van Dam ◽  
...  

There is an ongoing debate regarding the impact of phenylketonuria (PKU) and its treatment on growth. To date, evidence from studies is inconsistent, and data on the whole developmental period is limited. The primary aim of this systematic review was to investigate the effects of a phenylalanine (Phe)-restricted diet on long-term growth in patients with PKU. Four electronic databases were searched for articles published until September 2018. A total of 887 results were found, but only 13 articles met eligibility criteria. Only three studies had an adequate methodology for meta-analysis. Although the results indicate normal growth at birth and during infancy, children with PKU were significantly shorter and had lower weight for age than reference populations during the first four years of life. Impaired linear growth was observed until the end of adolescence in PKU. In contrast, growth impairment was not reported in patients with mild hyperphenylalaninemia, not requiring dietary restriction. Current evidence indicates that even with advances in dietary treatments, “optimal” growth outcomes are not attained in PKU. The majority of studies include children born before 1990s, so further research is needed to show the effects of recent dietary practices on growth in PKU.

2021 ◽  
Vol 10 (5) ◽  
pp. 1141
Author(s):  
Gianpaolo Marte ◽  
Andrea Tufo ◽  
Francesca Steccanella ◽  
Ester Marra ◽  
Piera Federico ◽  
...  

Background: In the last 10 years, the management of patients with gastric cancer liver metastases (GCLM) has changed from chemotherapy alone, towards a multidisciplinary treatment with liver surgery playing a leading role. The aim of this systematic review and meta-analysis is to assess the efficacy of hepatectomy for GCLM and to analyze the impact of related prognostic factors on long-term outcomes. Methods: The databases PubMed (Medline), EMBASE, and Google Scholar were searched for relevant articles from January 2010 to September 2020. We included prospective and retrospective studies that reported the outcomes after hepatectomy for GCLM. A systematic review of the literature and meta-analysis of prognostic factors was performed. Results: We included 40 studies, including 1573 participants who underwent hepatic resection for GCLM. Post-operative morbidity and 30-day mortality rates were 24.7% and 1.6%, respectively. One-year, 3-years, and 5-years overall survival (OS) were 72%, 37%, and 26%, respectively. The 1-year, 3-years, and 5-years disease-free survival (DFS) were 44%, 24%, and 22%, respectively. Well-moderately differentiated tumors, pT1–2 and pN0–1 adenocarcinoma, R0 resection, the presence of solitary metastasis, unilobar metastases, metachronous metastasis, and chemotherapy were all strongly positively associated to better OS and DFS. Conclusion: In the present study, we demonstrated that hepatectomy for GCLM is feasible and provides benefits in terms of long-term survival. Identification of patient subgroups that could benefit from surgical treatment is mandatory in a multidisciplinary setting.


2019 ◽  
Vol 26 (3) ◽  
pp. 910-920 ◽  
Author(s):  
Sani Abubakar Saddiq ◽  
Abu Sufian Abu Bakar

Purpose The purpose of the study is to investigate the impact of economic and financial crimes on the economies of emerging and developing countries. Design/methodology/approach Preferred Reporting Items for Systematic review and Meta-Analysis (PRISMA) guidelines and meta-analysis of economics research reporting guidelines were used to conduct a quantitative synthesis of empirical evidence on the impact of economic and financial crimes in developing and emerging countries. Findings A total of 103 studies were searched, out of which 6 met the selection/eligibility criteria of this systematic review. The six selected studies indicated that economic and financial crimes have a negative impact in emerging and developing countries. Originality/value To the best knowledge of the authors, no published systematic review of the impact of economic and financial crimes in developing countries has been conducted to date.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e15540-e15540
Author(s):  
Andrew MacCormick ◽  
Mark Puckett ◽  
Adam Streeter ◽  
Somaiah Aroori

e15540 Background: Recent research has demonstrated the impact that body composition parameters can have on the outcomes following cancer surgery. Adipose tissue deposition in muscle, known as myosteatosis, can be detected on pre-operative imaging. This systematic review aims to analyse the impact of pre-operative myosteatosis on long-term outcomes following surgery for gastro-intestinal malignancy. Methods: Using MeSH terms, a systematic search of the databases PubMed MEDLINE, EMBASE, Cochrane, CINAHL and AMED was performed. Studies were included if they reported hazard ratios (HR) analysing the impact of pre-operatively defined myosteatosis, or similar term, on the long-term outcomes following surgery for gastro-intestinal malignancy. A total of 39 full texts articles were reviewed for inclusion, with 19 being included after the inclusion criteria were applied. A sub-group analysis was performed for those studies reporting outcomes for colorectal cancer patients only. Results: The total number of included patients across all studies was 14,481. Patients with myosteatosis had a significantly poorer overall survival, according to univariate (HR 1.82, 95% CI 1.67 – 1.99) and multivariable (HR 1.66, 95% CI 1.49 – 1.86) analysis. This was also demonstrated with regards to cancer-specific survival (univariate HR 1.62, 95% CI 1.18 – 2.22, multivariable HR 1.73, 95% CI 1.48 – 2.03) and recurrence-free survival (univariate HR 1.28, 95% CI 1.10 – 1.48, multivariable HR 1.38, 95% CI 1.07 – 1.77). Conclusions: This review demonstrates that patients with pre-operative myosteatosis have poorer long-term outcomes following surgery for gastro-intestinal malignancy. Therefore, myosteatosis should be used for pre-operative optimisation and as a prognostic tool before surgery. More standardised definitions of myosteatosis and further cohort studies of patients with non-colorectal malignancies are required.


2021 ◽  
Vol 10 (19) ◽  
pp. 4462
Author(s):  
Konstantinos G. Kyriakoulis ◽  
Anastasios Kollias ◽  
Garyphallia Poulakou ◽  
Ioannis G. Kyriakoulis ◽  
Ioannis P. Trontzas ◽  
...  

The role of immunomodulatory agents in the treatment of hospitalized patients with COVID-19 has been of increasing interest. Anakinra, an interleukin-1 inhibitor, has been shown to offer significant clinical benefits in patients with COVID-19 and hyperinflammation. An updated systematic review and meta-analysis regarding the impact of anakinra on the outcomes of hospitalized patients with COVID-19 was conducted. Studies, randomized or non-randomized with adjustment for confounders, reporting on the adjusted risk of death in patients treated with anakinra versus those not treated with anakinra were deemed eligible. A search was performed in PubMed/EMBASE databases, as well as in relevant websites, until 1 August 2021. The meta-analysis of six studies that fulfilled the inclusion criteria (n = 1553 patients with moderate to severe pneumonia, weighted age 64 years, men 66%, treated with anakinra 50%, intubated 3%) showed a pooled hazard ratio for death in patients treated with anakinra at 0.47 (95% confidence intervals 0.34, 0.65). A meta-regression analysis did not reveal any significant associations between the mean age, percentage of males, mean baseline C-reactive protein levels, mean time of administration since symptoms onset among the included studies and the hazard ratios for death. All studies were considered as low risk of bias. The current evidence, although derived mainly from observational studies, supports a beneficial role of anakinra in the treatment of selected patients with COVID-19.


2019 ◽  
Vol 53 (17) ◽  
pp. 1070-1077 ◽  
Author(s):  
Myles Calder Murphy ◽  
Mervyn J Travers ◽  
Paola Chivers ◽  
James Robert Debenham ◽  
Sean Iain Docking ◽  
...  

ObjectiveTo assess the effectiveness of heavy eccentric calf training (HECT) in comparison with natural history, traditional physiotherapy, sham interventions or other exercise interventions for improvements in pain and function in mid-portion Achilles tendinopathy.DesignA systematic review and meta-analysis were conducted as per the PRISMA guidelines.Data sourcesPUBMED, CINAHL (Ovid) and CINAHL (EBSCO) were searched from inception until 24 September 2018.Eligibility criteriaRandomised controlled trials comparing HECT to natural history, sham exercise, traditional physiotherapy and other exercise interventions were included. Primary outcome assessing pain and function was the Victorian Institute of Sports Assessment-Achilles.ResultsSeven studies met the inclusion criteria. This review suggests HECT may be superior to both natural history, mean difference (MD) (95% CI) of 20.6 (11.7 to 29.5, one study) and traditional physiotherapy, MD (95% CI) of 17.70 (3.75 to 31.66, two studies). Following removal of one study, at high risk of bias, due to pre-planned sensitivity analysis, this review suggests HECT may be inferior to other exercise interventions, MD (95% CI) of −5.65 (-10.51 to −0.79, three studies). However, this difference is unlikely to be clinically significant.ConclusionCurrent evidence suggests that HECT may be superior to natural history and traditional physiotherapy while HECT may be inferior to other exercise interventions. However, due to methodological limitations, small sample size and a lack of data we are unable to be confident in the results of the estimate of the effect, as the true effect is likely to be substantially different.Systematic review registryPROSPERO registration number: CRD4201804493Protocol referenceThis protocol has been published open access: Murphy M, Travers MJ, Gibson, W. Is heavy eccentric calf training superior to natural history, sham rehabilitation, traditional physiotherapy and other exercise interventions for pain and function in mid-portion Achilles tendinopathy? Systematic Reviews 2018; 7: 58


2020 ◽  
Vol 64 (6) ◽  
pp. 547-555
Author(s):  
Vasilios Pergialiotis ◽  
Christina Nikolaou ◽  
Dimitrios Haidopoulos ◽  
Maximos Frountzas ◽  
Nikolaos Thomakos ◽  
...  

<b><i>Introduction:</i></b> Several studies have implicated the PIK3/AKT pathway in the pathophysiology of cancer progression as its activation seems to be aberrant in several forms of cancer. The purpose of the present systematic review is to evaluate the impact of PIK3CA mutations on survival outcomes of patients with cervical cancer. <b><i>Methods:</i></b> We used the Medline (1966–2020), Scopus (2004–2020), ClinicalTrials.gov (2008–2020), EMBASE (1980–2020), Cochrane Central Register of Controlled Trials (CENTRAL) (1999–2020), and Google Scholar (2004–2020) databases in our primary search along with the reference lists of electronically retrieved full-text papers. Statistical meta-analysis was performed with the RevMan 5.3 software. <b><i>Results:</i></b> Overall, 12 articles were included in the present study that comprised 2,196 women with cervical cancer. Of those, 3 studies did not report significant differences in survival outcomes among patients with mutated versus wild-type PIK3CA tumors, 5 studies reported decreased survival outcomes, and 3 studies revealed increased survival rates. The meta-analysis revealed that patients with the mutated PIK3CA genotypes had worse overall survival compared to patients with wild-type PIK3CA (HR 2.31; 95% CI: 1.51, 3.55; 95% PI: 0.54, 9.96; data from 3 studies) and the same was observed in the case of DFS rates (HR 1.82; 95% CI: 1.47, 2.25; 95% PI: 1.29, 2.56; data from 4 studies). <b><i>Conclusion:</i></b> Current evidence concerning the impact of PIK3CA mutations on survival outcomes of patients with cervical cancer is inconclusive, although the majority of included studies support a potential negative effect, primarily among those with squamous cell carcinoma tumors.


2020 ◽  
Author(s):  
Ben Grodzinski ◽  
Rory Durham ◽  
Oliver Mowforth ◽  
Daniel Stubbs ◽  
Mark R N Kotter ◽  
...  

Abstract Objective Degenerative cervical myelopathy (DCM) is a disabling neurological condition. The underlying degenerative changes are known to be more common with age, but the impact of age on clinical aspects of DCM has never been synthesised. The objective of this study is to determine whether age is a significant predictor in three domains—clinical presentation, surgical management and post-operative outcomes of DCM. Methods a systematic review of the Medline and Embase databases (inception to 12 December 2019), registered with PROSPERO (CRD42019162077) and reported in accordance with preferred reporting items of systematic reviews and meta-analysis (PRISMA) guidelines, was conducted. The inclusion criteria were full text articles in English, evaluating the impact of age on clinical aspects of DCM. Results the initial search yielded 2,420 citations, of which 206 articles were eventually included. Age was found to be a significant predictor in a variety of measures. Within the presentation domain, older patients have a worse pre-operative functional status. Within the management domain, older patients are more likely to undergo posterior surgery, with more levels decompressed. Within the outcomes domain, older patients have a worse post-operative functional status, but a similar amount of improvement in functional status. Because of heterogenous data reporting, meta-analysis was not possible. Conclusion the current evidence demonstrates that age significantly influences the presentation, management and outcomes of DCM. Although older patients have worse health at all individual timepoints, they experience the same absolute benefit from surgery as younger patients. This finding is of particular relevance when considering the eligibility of older patients for surgery.


2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Chengxian Yang ◽  
Ge Li ◽  
Shenzhong Jiang ◽  
Xinjie Bao ◽  
Renzhi Wang

Abstract Biochemical remission after transsphenoidal surgery is still unsatisfied in acromegaly patients with macroadenomas, especially with invasive macroadenomas. Concerning the impact of preoperative somatostatin analogues (SSAs) on surgical outcomes, previous studies with limited cases reported conflicting results. To assess current evidence of preoperative medical treatment, we performed a systematic review and meta-analysis of comparative studies. A literature search was conducted in Pubmed, Embase, and the Cochrane Library. Five randomized controlled trials (RCT) and seven non-RCT comparative studies were included. These studies mainly focused on pituitary macroadenomas though a small number of microadenoma cases were included. For safety, preoperative SSAs were not associated with elevated risks of postoperative complications. With respect to efficacy, the short-term cure rate was improved by preoperative SSAs, but the long-term cure rate showed no significant improvement. For invasive macroadenomas, the short-term cure rate was also improved, but the long-term results were not evaluable in clinical practice because adjuvant therapy was generally required. In conclusion, preoperative SSAs are safe in patients with acromegaly, and the favorable impact on surgical results is restricted to the short-term cure rate in macroadenomas and invasive macroadenomas. Further well-designed RCTs to examine long-term results are awaited to update the finding of this meta-analysis.


Vascular ◽  
2009 ◽  
Vol 17 (5) ◽  
pp. 243-252 ◽  
Author(s):  
Benjamin O. Patterson ◽  
Peter J. Holt ◽  
Robert J. Hinchliffe ◽  
Matt M. Thompson ◽  
Ian M. Loftus

Current evidence suggests that carotid endarterectomy (CEA) performed within 2 weeks of symptoms produces better long-term results than if it is delayed. Urgent endarterectomy following unstable presentations such as crescendo transient ischemic attack (cTIA) or progressive stroke has been associated with variable results. The evidence for this treatment strategy required reviewing. A systematic review of articles related to urgent CEA between 1980 and 2008 was performed. For cTIA, there was an odds ratio of 5.6 (95% confidence interval 3.3–9.7, p ≤ .0001) for combined stroke or death compared with surgery for “standard” indications. For unstable stroke, the odds ratio was 5.5 (95% confidence interval 3.1–9.3, p ≤ .0001). Patients with unstable neurologic presentations are at higher risk of complications if operated on urgently. Clearer definitions would help more precise patient selection to avoid inadvertently operating on patients with an unacceptably high risk of poor outcome.


BMJ Open ◽  
2019 ◽  
Vol 9 (3) ◽  
pp. e019368 ◽  
Author(s):  
Bruno Rodrigues Rosa ◽  
Antonio José Ledo Alves da Cunha ◽  
Roberto de Andrade Medronho

BackgroundRandomised controlled trials have evaluated the recombinant tetravalent dengue vaccine (CYD-TDV). However, individual results may have little power to identify differences among the populations studied.ObjectiveTo evaluate efficacy, immunogenicity and safety of CYD-TDV in the prevention of dengue in children aged 2–17 years.DesignSystematic review and meta-analysis.Data sourcesMEDLINE (from 1950 to 5 December 2018), EMBASE (from 1947 to 5 December 2018) and Cochrane (from 1993 to 5 December 2018).Eligibility criteria of studiesRandomised trials comparing efficacy, immunogenicity and safety of CYD-TDV with placebo or other vaccines for preventing dengue cases in children aged 2–17 years.Outcome measuresEfficacy, immunogenicity and safety of CYD-TDV.Study appraisal and methodsCalculations were made of relative risk (RR) and mean difference (MD) for dichotomous and continuous outcomes, respectively. All estimates were calculated considering a 95% CI estimate. A p<0.05 was considered statistically significant.ResultsNine studies involving 34 248 participants were included. The overall efficacy of CYD-TDV was 60% (RR 0.40 (0.30 to 0.54)). Serotype-specific efficacy of the vaccine was 51% for dengue virus type-1 (DENV-1) (RR 0.49 (0.39 to 0.63)); 34% for DENV-2 (RR 0.66 (0.50 to 0.86)); 75% for DENV-3 (RR 0.25 (0.18 to 0.35)) and 77% for DENV-4 (RR 0.23 (0.15 to 0.34)). Overall immunogenicity (MD) of CYD-TDV was 225.13 (190.34 to 259.93). Serotype-specific immunogenicity was: DENV-1: 176.59 (123.36 to 229.83); DENV-2: 294.21 (181.98 to 406.45); DENV-3: 258.78 (146.72 to 370.84) and DENV-4: 189.35 (141.11 to 237.59). The most common adverse events were headache and pain at the injection site.LimitationsThe main limitation of this study was unclear or incomplete data.Conclusions and implications of key findingsCYD-TDV is considered safe and able to partially protect children and adolescents against four serotypes of DENV for a 1-year period. Despite this, research should prioritise improvements in vaccine efficacy, thus proving higher long-term protection against all virus serotypes.PROSPERO registration numberCRD42016043628.


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