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Circulation. Genomic and Precision... Oct 2021The p.Val142Ile variant, predominantly found among people of African descent, is the most common cause of variant transthyretin amyloidosis and carriers predominantly...
BACKGROUND
The p.Val142Ile variant, predominantly found among people of African descent, is the most common cause of variant transthyretin amyloidosis and carriers predominantly develop a cardiomyopathy (variant transthyretin amyloidosis cardiomyopathy) phenotype. Yet, there are conflicting data on the prevalence and outcomes of p.Val142Ile variant carriers.
METHODS
We performed a systematic review of the prevalence and outcomes of p.Val142Ile variant transthyretin amyloidosis cardiomyopathy among subjects of African descent. We found 62 relevant articles after searching the MEDLINE databases from 1980 to 2020 that reported data for ≈150 000 subjects.
RESULTS
The reported worldwide prevalence of the p.Val142Ile variant is 0.3% to 1.6% in the general population. Among people of African descent, the reported prevalence from all studies ranges from 1.1% to 9.8%, but for studies with >1000 subjects, it is 3% to 3.5%. The prevalence of the p.Val142Ile variant in a region is dependent on the reported percentage of subjects who are of African descent in that region. p.Val142Ile variant transthyretin amyloidosis cardiomyopathy typically presents in the seventh to eighth decade of life and the majority of cases reported were male, with 25% to 38% diagnosed with atrial fibrillation. It was associated with a longitudinally worse quality of life and a lower adjusted survival compared with other types of transthyretin amyloidosis cardiomyopathy.
CONCLUSIONS
The p.Val142Ile variant is the most common variant of the transthyretin gene with most carriers being of African descent. The true penetrance is unknown but the p.Val142Ile variant is associated with increased rates of incident heart failure and portends a lower overall survival. Increased awareness could lead to earlier diagnosis and improved heart failure outcomes among those of African descent, which is of increasing importance given the advent of novel therapeutics for this disease.
Topics: Amino Acid Substitution; Amyloid Neuropathies, Familial; Cardiomyopathies; Female; Humans; Male; Mutation, Missense; Prealbumin; Prevalence; Risk Factors; Sex Factors
PubMed: 34461737
DOI: 10.1161/CIRCGEN.121.003356 -
Intermittent tube feeding for stroke patients with dysphagia: a meta-analysis and systematic review.Annals of Palliative Medicine Jul 2021Nutrition supports is essential to the prognosis of stroke patients with dysphagia. It's necessary to evaluate the effects and safety of intermittent tube feeding for... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Nutrition supports is essential to the prognosis of stroke patients with dysphagia. It's necessary to evaluate the effects and safety of intermittent tube feeding for stroke patients with dysphagia, to provide evidence for the management of dysphagia.
METHODS
Cochrane Library et al. databases were searched for randomized controlled trials (RCTs) on the intermittent tube feeding for stroke patients with dysphagia up to Feb 15, 2021. Bias risk assessment tool recommended by Cochrane was used for quality assessment, and Revman5.3 software was used for data analysis.
RESULTS
A total of 11 RCTs involving 762 stroke patients with dysphagia were included. Meta-analysis indicated that intermittent tube feeding could significantly increase the rate of dysphagia function improvement [odd ratio (OR ) = 5.22, 95% confidence interval (CI): 3.38-8.07], serum albumin level [mean difference (MD) = 3.07, 95% CI: 1.65-4.49], hemoglobin level (MD =1.55, 95% CI: 1.19-1.95), prealbumin level (MD =1.79, 95% CI: 1.46-2.12), and reduce the incidence of aspiration pneumonia (OR = 0.28, 95% CI: 0.15-0.53), incidence of aspiration (OR =0.27, 95% CI: 0.08-0.93) for stroke patients with dysphagia (all P<0.05), o significant difference in the triceps skinfold thickness (TSF) (MD =0.46, 95% CI: -0.24 to 1.19) and arm muscle circumference (MD =0.04, 95% CI: -0.28 to 0.36) between two groups were found (all P>0.05). Egger regression tests indicated that there was no publication bias between included RCTs (all P>0.05).
DISCUSSION
Intermittent tube feeding for stroke patients with dysphagia during the recovery period can not only ensure the nutritional supply, but also promote the recovery of swallowing function and reduce the occurrence of aspiration and aspiration associated pneumonia.
Topics: Deglutition Disorders; Enteral Nutrition; Humans; Nutritional Status; Stroke
PubMed: 34353034
DOI: 10.21037/apm-21-736 -
Heart Failure Reviews Mar 2022Transthyretin cardiac amyloidosis (ATTR-CM) is caused by the accumulation of misfolded transthyretin (TTR) protein in the myocardium. Diflunisal, an agent that... (Review)
Review
Transthyretin cardiac amyloidosis (ATTR-CM) is caused by the accumulation of misfolded transthyretin (TTR) protein in the myocardium. Diflunisal, an agent that stabilizes TTR, has been used as an off-label therapeutic for ATTR-CM. Given limited data surrounding the use of diflunisal, a systematic review of the literature is warranted. We searched the PubMed, MEDLINE, and Embase databases for studies that reported on the use of diflunisal therapy for patients with ATTR-CM. We included English language studies which assessed the effect of diflunisal in adult patients with ATTR-CM who received diflunisal as primary treatment and reported clinical outcomes with emphasis on studies that noted the safety and efficacy of diflunisal in cardiac manifestations of ATTR amyloidosis. We excluded studies which did not use diflunisal therapy or used diflunisal therapy for non-cardiac manifestations of TTR amyloidosis. We also excluded case reports, abstracts, oral presentations, and studies with fewer than 10 subjects. Our search yielded 316 records, and we included 6 studies reporting on 400 patients. Non-comparative single-arm small non-randomized trials for diflunisal comprised 4 of the included studies. The 2 studies that compared diflunisal versus no treatment found improvements in TTR concentration, left atrial volume index, cardiac troponin I, and global longitudinal strain. Overall, diflunisal use was associated with decreased mortality and number of orthotopic heart transplant in ATTR-CM patients. Although a smaller number of patients had to stop treatment due to gastrointestinal side effects and transient renal dysfunction, there were no severe reactions reported in the studies included in our review. This systematic review supports the use of diflunisal for ATTR-CM. Additional long-term analyses and randomized clinical trials are needed to confirm these results.
Topics: Adult; Amyloid Neuropathies, Familial; Diflunisal; Humans; Myocardium; Prealbumin
PubMed: 34272629
DOI: 10.1007/s10741-021-10143-4 -
European Review For Medical and... May 2021This systematic review and meta-analysis aimed to evaluate the association between the prealbumin and severity and mortality in COVID-19. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
This systematic review and meta-analysis aimed to evaluate the association between the prealbumin and severity and mortality in COVID-19.
MATERIALS AND METHODS
We performed a systematic literature search from PubMed, Embase, and Scopus databases up until 2 February 2021. The primary outcome was the poor outcome, a composite of mortality and severity. Severe COVID-19 was defined as COVID-19 that fulfill the criteria for severe pneumonia or patients with acute respiratory distress syndrome/disease progression/need for intensive care unit or mechanical ventilation. The effect estimates were a mean difference between patients with and without a poor outcome in mg/dL and odds ratio (OR) per 1 mg/dL decrease in prealbumin level. The effect estimates were reported with their 95% confidence interval (95% CI).
RESULTS
Nine studies comprising of 2104 patients were included in this systematic review and meta-analysis. Patients with poor outcome have lower prealbumin level (mean difference -71.48 mg/dL [95% CI -93.74, -49.22], p<0.001; I2: 85.9%). Every 1 mg/dL decrease in prealbumin level was associated with 1% increase in poor outcome (OR 0.992 [0.987, 0.997], p=0.004, I2: 81.7%). Meta-regression analysis showed that the association between the prealbumin level and poor outcome varies with gender (male) (coefficient: 3.50, R2: 100%, p<0.001), but not age, diabetes, hypertension, and chronic kidney disease.
CONCLUSIONS
Low serum prealbumin was associated with poor outcomes in patients with COVID-19.
Topics: COVID-19; Humans; Odds Ratio; Prealbumin; SARS-CoV-2; Severity of Illness Index; Sex Factors
PubMed: 34109596
DOI: 10.26355/eurrev_202105_25955 -
Amyloid : the International Journal of... Sep 2021Hereditary transthyretin (ATTRv) amyloidosis is a progressive multisystemic disease of adult-onset that arises from an inherited mutation in the transthyretin gene....
BACKGROUND
Hereditary transthyretin (ATTRv) amyloidosis is a progressive multisystemic disease of adult-onset that arises from an inherited mutation in the transthyretin gene. Currently available disease severity and progression evaluation tools only cover one single organ or system, impacting data collection uniformity and its use in clinical settings.
METHODS
The Jandhyala Method, including a systematic literature review and SMART interviews, was used to observe expert opinion from eight leaders in the treatment of ATTRv across Europe. The aim was to propose a multidisciplinary core dataset (CD) and disease severity scoring (DSS) tools.
RESULTS
The multidisciplinary team of experts identified 140 indicators that form part of the standard diagnostic and monitoring practice (SDMP) and should be collected as the ATTRv CD. Thirty-one (22%) of these indicators informed disease severity and comprised the ATTRv DSS, whilst 25 (18%) were deemed to monitor disease progression.
CONCLUSIONS
The resulting CD and DSS have different purposes. The ATTRv CD supports the collection of high-quality data for clinical research, whereas the ATTRv DSS can be rapidly conducted in a clinical setting and aid patient management.
Topics: Adult; Amyloid Neuropathies, Familial; Europe; Humans; Prealbumin; Severity of Illness Index
PubMed: 34042016
DOI: 10.1080/13506129.2021.1931099 -
Heart Failure Reviews May 2022V122I genotype variant (pV142I) is the most common hereditary transthyretin amyloidosis (hATTR) in the USA, with 3-3.5% of African-Americans being the carriers of this...
V122I genotype variant (pV142I) is the most common hereditary transthyretin amyloidosis (hATTR) in the USA, with 3-3.5% of African-Americans being the carriers of this mutation. We aimed to compare baseline clinical features, cardiac parameters, and mortality in V122I-ATTR with the wild-type ATTR and other hATTR subtypes. We systematically searched PubMed/Medline and Google Scholar databases to identify relevant studies from inception to 10th September, 2020 reporting phenotypic, echocardiographic, and/or laboratory parameters in patients with hereditary and wild types of cardiac amyloidoses. A total of 2843 patients from 7 individual studies with 67-100% males and an overall follow-up duration of 51.6 ± 30.4 months were identified. The mean age of diagnosis among wild-type ATTR patients was 77 years, followed by 71.2 and 65 years in V122I and T60A group patients, respectively. V122I patients were mostly black, had a poor quality of life, and highest mortality risk compared with other subtypes. Merely, the presence of V122I mutation was identified as an independent predictor of mortality. V30M subtype correlated with the least severe cardiac disease and a median survival duration comparable with T60A subtype. V122I ATTR is an aggressive disease, prevalent in African-Americans, and is associated with a greater morbidity and mortality, which is partly attributed to its misdiagnosis and/or late diagnosis. Current advances in non-invasive studies to diagnose hATTR coupled with concurrent drug therapies have improved quality of life and provide a survival benefit to these patients.
Topics: Aged; Amyloid Neuropathies, Familial; Cardiomyopathies; Female; Genotype; Humans; Male; Prealbumin; Quality of Life
PubMed: 33768376
DOI: 10.1007/s10741-021-10098-6 -
Frontiers in Medicine 2021Excessive inflammation and malnutrition are associated with coronavirus disease 2019 (COVID-19) severity and mortality. Combined biomarkers of malnutrition and...
Excessive inflammation and malnutrition are associated with coronavirus disease 2019 (COVID-19) severity and mortality. Combined biomarkers of malnutrition and inflammation, such as serum prealbumin, might be particularly attractive for early risk stratification. We conducted a systematic review and meta-analysis of studies reporting serum prealbumin in patients with COVID-19. We searched PubMed, Web of Science and Scopus, between January and November 2020, for studies reporting data on serum prealbumin, COVID-19 severity, defined as severe illness, prolonged viral load, receiving mechanical ventilation or admitted to intensive care unit (ICU), and mortality. Nineteen studies in 4,616 COVID-19 patients were included in the meta-analysis. Pooled results showed that serum prealbumin concentrations were significantly lower in patients with severe disease and non-survivors (standard mean difference, SMD, -0.92, 95% CI, -1.10 to -0.74, < 0.001). Extreme heterogeneity was observed ( = 77.9%; < 0.001). In sensitivity analysis, the effect size was not significantly affected when each study was in turn removed (range between -0.86 and -0.95). The Begg's ( = 0.06) and Egger's -tests ( = 0.26) did not show publication bias. Pooled SMD values were significantly and negatively associated with age ( = -2.18, = 0.045) and C-reactive protein ( = -3.85, = 0.002). In our meta-analysis, lower serum prealbumin concentrations were significantly associated with COVID-19 severity and mortality. This combined marker of malnutrition and inflammation might assist with early risk stratification and management in this group.
PubMed: 33575267
DOI: 10.3389/fmed.2021.638529 -
Clinical Neurology and Neurosurgery Mar 2021
Topics: Albumins; Alpha-Globulins; Ascites; Cerebrospinal Fluid Proteins; Cerebrospinal Fluid Shunts; Female; Heart Atria; Humans; Hydrocephalus; Meningeal Neoplasms; Meningioma; Middle Aged; Neoplasms, Multiple Primary; Paracentesis; Postoperative Complications; Prealbumin; Reoperation; Ventriculoperitoneal Shunt
PubMed: 33540175
DOI: 10.1016/j.clineuro.2021.106519 -
International Urology and Nephrology Aug 2021Almost all CKD patients have a poor nutritional status, while elderly CKD patients are specifically frail and malnourished. Our aim is to conduct a systematic review of...
PURPOSE
Almost all CKD patients have a poor nutritional status, while elderly CKD patients are specifically frail and malnourished. Our aim is to conduct a systematic review of the up to date primary studies that look at methods of improving nutritional status in CKD patients in the elderly demographic.
METHODS
A focussed and sensitive search strategy was applied to the PUBMED database to identify relevant English language articles. Once articles were identified a detailed quality and bias assessment was performed. Two independent researchers (MZ and SM) then subsequently carried out detailed data extraction and analysis and this was subsequently verified by a third researcher (IN).
RESULTS
A total of 19 studies were included in our systematic review which included 7 non-randomised control trials and 15 randomised controlled trials. The outcomes that we considered to be most relevant for our subject title were: mortality data, SGA, albumin, total protein, isoleucine, leucine, prealbumin, transferrin, leptin, valine, TAG, HDL, LDL and total amino acids. Detailed bias analysis of the different studies was also conducted.
CONCLUSION
This is the first systematic review of the literature, so far, on the subject, involving elderly CKD patients. The quality of trials is low, very heterogenic in patients, methods and outcomes. However, we found a positive effect of dietary interventions on the nutritional status of most patients studied, highlighted by improvement in serum albumin and SGA, the most measured outcomes.
Topics: Humans; Malnutrition; Nutritional Status; Renal Insufficiency, Chronic
PubMed: 33459956
DOI: 10.1007/s11255-020-02775-6 -
Clinical Nutrition (Edinburgh, Scotland) May 2021The safety and potential benefits of home enteral route nutrition (HERN), referring specifically to home enteral nutrition (HEN) and oral nutritional supplements (ONS)... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The safety and potential benefits of home enteral route nutrition (HERN), referring specifically to home enteral nutrition (HEN) and oral nutritional supplements (ONS) in this article, after upper gastrointestinal (GI) resection are inconsistent.
OBJECTIVE
To evaluate the impact of HERN on nutritional status, complications, and quality of life (QOL) after upper GI resection.
METHODS
This systematic review was conducted in accordance with the PRISMA statement. Nine databases were searched from inception to October 2019. Randomized controlled trials (RCTs) comparing the impact of HERN after upper gastrointestinal resection were included. Relative risk/weighted mean difference/standardized mean difference (RR/WMD/SMD) and corresponding 95% confidence intervals (95% CI) were calculated using fixed- or random-effects models.
RESULTS
Overall, 15 RCTs involving 1059 patients were included. Compared with normal oral diet, HERN significantly prevented weight loss (-3.95 vs -5.82 kg; SMD: 1.98 kg; 95% CI: 1.24-2.73); improved added-level of albumin (3.48 vs 2.41 g/L; SMD: 1.36 g/L; 95% CI: 0.81-1.91), hemoglobin (6.54 vs -1.29 g/L; WMD: 7.45 g/L; 95% CI: 5.05-9.86), pre-albumin (37.59 vs 7.35 mg/L; WMD: 21.6 mg/L; 95% CI: 5.96-37.24), and transferrin (63.08 vs 50.45 mg/L; WMD: 16.44 mg/L; 95% CI: 13.51-19.38); and reduced the incidence of malnutrition or latent malnutrition (RR = 0.54; P < 0.01). Subgroup analysis based on the approach of HERN showed that weight loss in the HEN subgroup was significantly lower than that of the control group (WMD = 2.69, P < 0.01), while there was no significant difference between the ONS subgroup and the control group (P = 0.1). The same results were found in albumin. Physical function (WMD: 5.29; 95% CI: 1.86-8.73) and fatigue (WMD: -8.59; 95% CI: -12.61, -4.58) dimensions in QOL were significantly better in the HERN group. No significant differences in gastrointestinal and tube-related complications.
CONCLUSION
HERN improved nutritional status and some dimensions of QOL in upper GI malignancy patients after surgery, without increasing complications. Subgroup analysis showed that HEN experienced more benefits than ONS.
Topics: Administration, Oral; Dietary Supplements; Enteral Nutrition; Gastrointestinal Neoplasms; Humans; Nutritional Status; Postoperative Complications; Quality of Life
PubMed: 33279310
DOI: 10.1016/j.clnu.2020.11.023