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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 -
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 -
Journal of Neurochemistry Mar 2021The liver-derived, circulating transport protein transthyretin (TTR) is the cause of systemic hereditary (ATTRv) and wild-type (ATTRwt) amyloidosis. TTR stabilization...
The liver-derived, circulating transport protein transthyretin (TTR) is the cause of systemic hereditary (ATTRv) and wild-type (ATTRwt) amyloidosis. TTR stabilization and knockdown are approved therapies to mitigate the otherwise lethal disease course. To date, the variety in phenotypic penetrance is not fully understood. This systematic review summarizes the current literature on TTR pathophysiology with its therapeutic implications. Tetramer dissociation is the rate-limiting step of amyloidogenesis. Besides destabilizing TTR mutations, other genetic (RBP4, APCS, AR, ATX2, C1q, C3) and external (extracellular matrix, Schwann cell interaction) factors influence the type of onset and organ tropism. The approved small molecule tafamidis stabilizes the tetramer and significantly decelerates the clinical course. By sequence-specific mRNA knockdown, the approved small interfering RNA (siRNA) patisiran and antisense oligonucleotide (ASO) inotersen both significantly reduce plasma TTR levels and improve neuropathy and quality of life compared to placebo. With enhanced hepatic targeting capabilities, GalNac-conjugated siRNA and ASOs have recently entered phase III clinical trials. Bivalent TTR stabilizers occupy both binding groves in vitro, but have not been tested in trials so far. Tolcapone is another stabilizer with the potential to cross the blood-brain barrier, but its half-life is short and liver failure a potential side effect. Amyloid-directed antibodies and substances like doxycycline aim at reducing the amyloid load, however, none of the yet developed antibodies has successfully passed clinical trials. ATTR-amyloidosis has become a model disease for pathophysiology-based treatment. Further understanding of disease mechanisms will help to overcome the remaining limitations, including application burden, side effects, and blood-brain barrier permeability.
Topics: Amyloid; Amyloidosis, Familial; Animals; Gene Knockdown Techniques; Humans; Prealbumin
PubMed: 33155274
DOI: 10.1111/jnc.15233 -
International Journal of Cardiology May 2024Transcatheter aortic valve replacement (TAVR) is a successful treatment for aortic stenosis (AS) patients, and previous studies indicate favorable outcomes for those... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Transcatheter aortic valve replacement (TAVR) is a successful treatment for aortic stenosis (AS) patients, and previous studies indicate favorable outcomes for those with concomitant aortic stenosis and transthyretin-associated cardiac amyloidosis (TTRCA-AS). However, the impact of TAVR on more adverse outcomes in TTRCA-AS patients compared to those with AS alone is still uncertain, with conflicting findings reported in the literature.
METHODS
PubMed and Scopus were extensively searched from inception till August 2021. Studies were included if they reported data for prevalence and outcomes including mortality and cardiovascular-related hospitalization events in TTRCA-AS patients referred for TAVR. The data for these outcomes were pooled using a random effects model and forest plots were created.
RESULTS
After initially screening 146 articles, 6 were shortlisted for inclusion in our analysis. Pooled analysis demonstrated a 13.3% [95% CI: 10.9-16.5; p = 0.307] prevalence of TTRCA in patients with AS undergoing TAVR. The incidence of mortality and cardiovascular (CV) hospitalization in patients with TTRCA-AS undergoing TAVR were 28.3% [95% CI: 18.7-39.0, p = 0.478] and 21.1% [95% CI: 10.2-34.5, p = 0.211], respectively.
CONCLUSION
The overall pooled TTRCA-AS prevalence was reported to be 13.3% in AS patients who underwent TAVR. Furthermore, transthyretin-associated CA was found to be associated with an increased risk of mortality and hospitalization. Large patient population studies are required to assess the safety and efficacy of TAVR in TTRCA-AS patients, as current research report data from small patient cohorts.
Topics: Humans; Transcatheter Aortic Valve Replacement; Prealbumin; Treatment Outcome; Aortic Valve Stenosis; Amyloidosis; Aortic Valve; Risk Factors
PubMed: 38367883
DOI: 10.1016/j.ijcard.2024.131854 -
Bioscience Reports Jun 2020The improvement of malnutrition with levocarnitine in maintenance hemodialysis (MHD) patients is controversial. We performed a meta-analysis to evaluate the efficacy of... (Meta-Analysis)
Meta-Analysis
The improvement of malnutrition with levocarnitine in maintenance hemodialysis (MHD) patients is controversial. We performed a meta-analysis to evaluate the efficacy of levocarnitine in improving malnutrition in MHD patients. We performed a literature search for relevant articles related to the treatment of malnutrition by L-carnitine in MHD patients in PubMed, Embase, Web of Science, China National Knowledge Infrastructure, and Wanfang databases. We set the publication dates from 1950 to July 2019. The levels of albumin, prealbumin, total protein, and transferrin before and after treatment were used for assessing malnutrition. Twenty-seven studies were included in the present analysis. The results of the random effects model indicated that L-carnitine treatment improved the albumin level in patients on MHD patients. The pooled standardized mean difference of albumin level was 2.51 (95% confidence interval (CI): 2.13-2.90, P<0.001). The pooled total protein level was 3.83 (95% CI: 2.41-5.24, P = 0.000) and the pooled transferrin level was 0.35 (95% CI: 0.18-0.52, P = 0.000). Significant differences were observed with the total protein and transferrin levels. The results indicated that levocarnitine significantly improved the prealbumin level in patients on MHD. The pooled prealbumin level was 70.86 (95% CI: 42.99-98.73, P = 0.000). No publication bias was detected (P>0.05). The present meta-analysis indicated that L-carnitine can have a favorable effect on malnutrition biomarkers in patients on MHD, including the increase in albumin, total protein, transferrin, and prealbumin levels. The L-carnitine could be an option for treatment of MHD patients.
Topics: Adult; Aged; Aged, 80 and over; Biomarkers; Carnitine; Dietary Supplements; Female; Humans; Kidney Diseases; Male; Malnutrition; Middle Aged; Nutritional Status; Prealbumin; Renal Dialysis; Serum Albumin, Human; Transferrin; Treatment Outcome
PubMed: 32490516
DOI: 10.1042/BSR20201639 -
Neurosurgery Oct 2021Preoperative malnutrition has been implicated in adverse events after elective surgery, potentially impacting patient outcomes.
BACKGROUND
Preoperative malnutrition has been implicated in adverse events after elective surgery, potentially impacting patient outcomes.
OBJECTIVE
As a potentially modifiable risk factor, we sought to determine which assessments of nutritional status were associated with specific adverse events after spine surgery. In addition, we explored if a preoperative nutritional improvement intervention may be beneficial in lowering the rates of these adverse events.
METHODS
The literature search yielded 115 abstracts relevant to the PICO (patient/population, intervention, comparison, and outcomes) questions included in this chapter. The task force selected 105 articles for full text review, and 13 met criteria for inclusion in this systematic review.
RESULTS
Malnutrition, assessed preoperatively by a serum albumin <3.5 g/dL or a serum prealbumin <20 mg/dL, is associated with a higher rate of surgical site infections (SSIs), other wound complications, nonunions, hospital readmissions, and other medical complications after spine surgery. A multimodal nutrition management protocol decreases albumin and electrolyte deficiencies in patients with normal preoperative nutritional status. It also improves overall complication rates but does not specifically impact SSIs.
CONCLUSION
It is recommended to assess nutritional status using either serum albumin or prealbumin preoperatively in patients undergoing spine surgery.The full guidelines can be accessed at https://www.cns.org/guidelines/browse-guidelines-detail/4-preoperative-nutritional-assessment.
Topics: Elective Surgical Procedures; Humans; Neurosurgeons; Nutrition Assessment; Retrospective Studies; Spine
PubMed: 34490884
DOI: 10.1093/neuros/nyab318 -
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 -
World Journal of Surgical Oncology Jul 2019Postsurgical patients' oral feeding begins with clear fluids 1-3 days after surgery. This might not be sufficiently nutritious to boost the host immune system and... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Postsurgical patients' oral feeding begins with clear fluids 1-3 days after surgery. This might not be sufficiently nutritious to boost the host immune system and provide sufficient energy in gastric neoplastic patients to achieve the goal of enhanced recovery after surgery (ERAS). Our objective was to analyze the significance of early postoperative feeding tubes in boosting patients' immunity and decreasing incidence of overall complications and hospital stay in gastric cancer patients' post-gastrectomy.
METHODS
From January 2005 to May 24, 2019, PubMed and Cochrane databases were searched for studies involving enteral nutrition (EN) feeding tubes in comparison to parenteral nutrition (PN) in gastric cancer patients undergoing gastrectomy for gastric malignancies. Relative risk (RR), mean difference (MD), or standard mean difference (SMD) with 95% confidence interval (CI) were used to estimate the effect sizes, and heterogeneity was assessed by using Q and χ statistic with their corresponding P values. All the analyses were performed with Review Manager 5.3 and SPSS version 22.
RESULTS
Nine randomized trials (n = 1437) and 5 retrospective studies (n = 421) comparing EN feeding tubes and PN were deemed eligible for the pooled analyses, with a categorized time frame of PODs ≥ 7 and PODs < 7. Ratio of CD4+/CD8+ in EN feeding tubes was the only outcome of PODs < 7, which showed significance (MD 0.22, 95% CI 0.18-0.25, P < 0.00001). Regarding other immune indicators, significant outcomes in favor of EN feeding tubes were measured on POD ≥ 7: CD3+ (SMD 1.71; 95% CI 0.70, 2.72; P = 0.0009), CD4+ (MD 5.84; 95% CI 4.19, 7.50; P < 0.00001), CD4+/CD8+ (MD 0.28; 95% CI 0.20; 0.36, P < 0.00001), NK cells (SMD 0.94; 95% CI 0.54, 1.30; P < 0.00001), nutrition values, albumin (SMD 0.63; 95% CI 0.34, 0.91; P < 0.001), prealbumin (SMD 1.00; 95% CI 0.52, 1.48; P < 0.00001), and overall complications (risk ratio 0.73 M-H; fixed; 95% CI 0.58, 0.92; P = 0.006).
CONCLUSION
EN feeding tube support is an essential intervention to elevate patients' immunity, depress levels of inflammation, and reduce the risk of complications after gastrectomy for gastric cancer. Enteral nutrition improves the innate immune system and nutrition levels but has no marked significance on certain clinical outcomes. Also, EN reduces the duration of hospital stay and cost, significantly.
Topics: Enteral Nutrition; Gastrectomy; Humans; Immunity, Innate; Length of Stay; Nutritional Status; Nutritive Value; Parenteral Nutrition; Postoperative Care; Postoperative Complications; Prognosis; Randomized Controlled Trials as Topic; Stomach Neoplasms; Time Factors; Treatment Outcome
PubMed: 31269969
DOI: 10.1186/s12957-019-1658-9 -
World Journal of Gastrointestinal... May 2023Gastrointestinal surgery is a complicated process used to treat many gastrointestinal diseases, and it is associated with a large trauma: Most patients often have...
BACKGROUND
Gastrointestinal surgery is a complicated process used to treat many gastrointestinal diseases, and it is associated with a large trauma: Most patients often have different degrees of malnutrition and immune dysfunction before surgery and are prone to various infectious complications during postoperative recovery, thus affecting the efficacy of surgical treatment. Therefore, early postoperative nutritional support can provide essential nutritional supply, restore the intestinal barrier and reduce complication occurrence. However, different studies have shown different conclusions.
AIM
To assess whether early postoperative nutritional support can improve the nutritional status of patients based on literature search and meta-analysis.
METHODS
Articles comparing the effect of early nutritional support and delayed nutritional support were retrieved from PubMed, EMBASE, Springer Link, Ovid, China National Knowledge Infrastructure, China Biology Medicine databases. Notably, only randomized controlled trial articles were retrieved from the databases (from establishment date to October 2022). The risk of bias of the included articles was determined using Cochrane Risk of Bias V2.0. The outcome indicators, such as albumin, prealbumin, and total protein, after statistical intervention were combined.
RESULTS
Fourteen literatures with 2145 adult patients undergoing gastrointestinal surgery (1138 patients (53.1%) receiving early postoperative nutritional support and 1007 patients (46.9%) receiving traditional nutritional support or delayed nutritional support) were included in this study. Seven of the 14 studies assessed early enteral nutrition while the other seven studies assessed early oral feeding. Furthermore, six literatures had "some risk of bias," and eight literatures had "low risk". The overall quality of the included studies was good. Meta-analysis showed that patients receiving early nutritional support had slightly higher serum albumin levels, than patients receiving delayed nutritional support [MD (mean difference) = 3.51, 95%CI: -0.05 to 7.07, = 1.93, = 0.05]. Also, patients receiving early nutritional support had shorter hospital stay (MD = -2.29, 95%CI: -2.89 to -1.69), = -7.46, < 0.0001) shorter first defecation time (MD = -1.00, 95%CI: -1.37 to -0.64), = -5.42, < 0.0001), and fewer complications (Odd ratio = 0.61, 95%CI: 0.50 to 0.76, = -4.52, < 0.0001) than patients receiving delayed nutritional support.
CONCLUSION
Early enteral nutritional support can slightly shorten the defecation time and overall hospital stay, reduce complication incidence, and accelerate the rehabilitation process of patients undergoing gastrointestinal surgery.
PubMed: 37342843
DOI: 10.4240/wjgs.v15.i5.953 -
Infectious Disorders Drug Targets 2023Recent studies have suggested the role of primary laboratory tests in addition to clinical symptoms for patients suspected to have coronavirus disease 2019 (COVID-19),... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Recent studies have suggested the role of primary laboratory tests in addition to clinical symptoms for patients suspected to have coronavirus disease 2019 (COVID-19), which play a significant role in the diagnosis of COVID-19. However, the results of these studies are contradictory. The present study was conducted to evaluate biochemical, serological, and immunological tests for the diagnosis of COVID-19 patients.
METHODS
This study was presented in accordance with the PRISMA protocol. This protocol is registered with the code CRD42019145410 in PROSPERO. We conducted a comprehensive literature search in databases, including Web of Science, PubMed/Medline, CINAHL Scopus, Cochrane Library, EMBASE, Science Direct, and EBSCO to find citations from the beginning of January 2019 until the beginning of April 2020 without any restrictions.
RESULTS
Finally, 51 studies, including 5,490 COVID-19 patients, were included in the present metaanalysis. The prevalence of different factors observed in laboratory findings was as follows: the prevalence of lymphopenia in patients with COVID-19 accounted for 51.6% (95% CI: 44.0-59.1), elevated C-reactive protein (CRP) was 63.6% (95% CI: 57.0-69.8), elevated erythrocyte sedimentation rate (ESR) was 62.5% (95% CI: 50.1-73.5), elevated tumor necrosis factor alpha (TNFα) was 28.7% (95% CI: 9.0-62.1), elevated serum amyloid-A level was 74.7% (95% CI: 50.0-89.7), elevated procalcitonin level was 72.6% (95% CI: 58.1-83.5), elevated interleukin-6 level was 59.9% (95% CI: 48.2-70.5), reduced CD3 level was 68.3% (95% CI: 50.1-82.2), reduced CD4 level was 62.0% (95% CI: 51.1- 71.6), elevated lactate dehydrogenase (LDH) level accounted for 53.1% (95% CI: 43.6-62.4), elevated brain natriuretic peptide (BNP) accounted for 48.9% (95% CI: 30.4-67.7), reduced albumin and reduced pre-albumin levels in patients with COVID-19 were estimated to be 54.7% (95% CI: 38.1-70.2) and 49.0% (95% CI: 26.6-71.8), and D-dimer level was 44.9% (95% CI: 31.0-59.6).
CONCLUSION
The results show lymphopenia, elevated ESR level, elevated CRP level, elevated serum amyloid-A, elevated TNFα, elevated procalcitonin level, elevated interleukin-6 level, reduced CD3, reduced CD4, elevated BNP, elevated LDH, reduced albumin, reduced pre-albumin, and elevated Ddimer levels as the most common findings at the time of admission.
Topics: Humans; Interleukin-6; Procalcitonin; Tumor Necrosis Factor-alpha; COVID-19; Immunologic Tests; Lymphopenia; Albumins; COVID-19 Testing
PubMed: 37646320
DOI: 10.2174/1871526523666230228144910