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Orphanet Journal of Rare Diseases Sep 2022Hereditary transthyretin amyloidosis (hATTR) is a progressive and fatal disease with heterogenous clinical presentations, limited diagnosis and poor prognosis. This...
BACKGROUND
Hereditary transthyretin amyloidosis (hATTR) is a progressive and fatal disease with heterogenous clinical presentations, limited diagnosis and poor prognosis. This retrospective analysis study aimed to report the genotypes and phenotypes of herediary transthyretin amyloidosis (hATTR) in Chinese through a systematic review of published literature.
METHODS
The systematic review included structured searches of peer-reviewed literature published from 2007 to 2020 of following online reference databases: PubMed, Web of Science and the literature database in China. Extracted data included sample size, personal information (sex, age, natural course, family history), mutation type, clinical milestones and reason of death.
RESULTS
We described 126 Chinese patients with hereditary transthyretin amyloidosis identified through a systematic review of 30 studies. The most common genotype in the Chinese population was Gly83Arg (25, 19.8%), which most likely presented visual and neurological abnormalities without reported death. The second and third most common genotypes were Val30Met (20, 15.9%) and Val30Ala (10, 7.9%). Peripheral neurological manifestations (91, 72%) were dominant in 126 patients. The followed manifestation was autonomic neurological abnormalities (73, 58%). Half of the cases were reported to have visual disorders, and nearly one-third of the cases presented cardiac abnormalities. Among all 126 reported patients, 46.03% were classified as neurological type, 30.16% as mixed type and only 2.38% as cardiac type. In addition. Chinese patients were mostly early onset, with age of onset at 41.8 (SD: 8.9) years, and the median time from onset to death was 7.5 [IQR: 5.3] years. Patients with cardiac involvement had a shorter survival duration (log Rank (Mantel-Cox), χ = 26.885, P < 0.001).
CONCLUSIONS
This study focused on 126 Chinese hATTR patients obtained from a literature review. A total of 26 kinds of TTR mutations were found and the most common one was Gly83Arg. As for phenotype, 46.03% were classified as neurological type, 30.16% as mixed type and only 2.38% as cardiac type. Chinese hATTR patients were mostly early onset (AO 41.8 years), and the median time from onset to death was 7.5 years.
Topics: Amyloid Neuropathies, Familial; Humans; Phenotype; Prealbumin; Retrospective Studies
PubMed: 36056432
DOI: 10.1186/s13023-022-02481-9 -
Cancer Cell International Jan 2022In recent years, the Fibrinogen to pre-albumin ratio (FPR) has been reported in many studies to be significantly associated with the prognosis of various cancers. This... (Review)
Review
BACKGROUND
In recent years, the Fibrinogen to pre-albumin ratio (FPR) has been reported in many studies to be significantly associated with the prognosis of various cancers. This systematic review and meta-analysis aimed to investigate the prognostic value of FPR in malignant tumors of the digestive system based on available evidence.
METHODS
The relevant articles published before July 1, 2021, were systematically retrieved from electronic databases to evaluate the effect of Fibrinogen to pre-albumin ratio (FPR) on the prognosis of patients with malignant digestive system tumors and calculate the hazard ratio (HR) and the corresponding 95% confidence interval (CI).
RESULT
Thirteen articles, all from China, including 15 cohort studies and a total of 5116 cases, were included in this study. A high FPR was associated with poor overall survival (HR = 1.88, 95%CI 1.53-2.32, P < 0.001), recurrence-free survival (HR = 2.29, 95%CI 1.91-2.76, P < 0.001), progression-free survival (HR = 1.96, 95%CI: 1.33-2.90, P = 0.001), complications (HR = 1.78, 95%CI: 1.06-3.00, P = 0.029), disease-free survival (HR = 1.46, 95%CI: 1.08-1.97, P = 0.013) was significantly associated with cancer-specific survival (HR = 1.44, 95%CI: 1.15-1.79, P = 0.001). Even though intergroup differences were present, FPR was strongly associated with overall and relapse-free survival, and sensitivity analysis suggested that our results were stable.
CONCLUSION
FPR can be used as a valuable indicator to predict the prognosis of patients with malignant digestive system tumors.
PubMed: 35033080
DOI: 10.1186/s12935-022-02445-w -
Hellenic Journal of Cardiology : HJC =... 2019Light-chain amyloidosis and transthyretin-related amyloidosis (wild-type and mutated) are three main types of systemic amyloidosis associated with a clinically relevant... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Light-chain amyloidosis and transthyretin-related amyloidosis (wild-type and mutated) are three main types of systemic amyloidosis associated with a clinically relevant cardiac involvement. In this study, we compared prognosis in patients with different categories of cardiac amyloidosis using meta-analysis and present a systematic review.
METHODS
A systematic literature search was performed through Jan 1, 2018, and two reviewers independently extracted data and assessed risk of bias. We extracted MACE and death endpoint events and hazard ratios from regression models and performed a meta-analysis of the multiple prognosis association studies.
RESULTS
We observed that there were significant MACE differences between patients diagnosed with transthyretin amyloidosis and light-chain amyloidosis (OR: 2.09; 95% CI: 1.06-4.12; P = 0.03), and the same is true in the sub-comparison between AL and mATTR or wtATTR (AL vs. mATTR: OR: 1.72; 95% CI: 1.06-2.82; P = 0.03; AL vs. wtATTR: OR: 1.48; 95% CI: 0.85-2.58; P = 0.17). However, no significant difference was observed between two transthyretin types (P = 0.17). Overall death rate evaluated showed that compared with transthyretin-related amyloidosis, light-chain type showed a significant difference (P < 0.05). The prognostic analysis showed that types of amyloidosis, LVEF, NYHA, restrictive filling pattern, E-wave deceleration time, E/E' ratio, and low QRS voltage were predictors of cardiac-related mortality.
CONCLUSION
Patients diagnosed with light-chain amyloidosis has a poor prognosis compared with transthyretin-related amyloidosis, while no difference was proved in prognostic analysis between wild-type and mutated TTR amyloidosis. Some clinical factors related to the death prognosis, such as the LVEF, restrictive filling pattern, E-wave deceleration time, and E/E' ratio are important prognostic factors.
Topics: Adult; Aged; Aged, 80 and over; Amyloid Neuropathies, Familial; Cardiomyopathies; Case-Control Studies; Female; Humans; Immunoglobulin Light-chain Amyloidosis; Male; Middle Aged; Prealbumin; Prognosis; Ventricular Function, Left
PubMed: 30742933
DOI: 10.1016/j.hjc.2019.01.015 -
Frontiers in Oncology 2021Prealbumin is a sensitive indicator of liver function and nutritional status.
BACKGROUND
Prealbumin is a sensitive indicator of liver function and nutritional status.
OBJECTIVES
This meta-analysis aimed to examine the association of the serum prealbumin level with the prognosis of patients with hepatocellular carcinoma (HCC) undergoing hepatectomy.
METHODS
We comprehensively searched the PubMed, Embase, Wanfang, China Academic Journals (CNKI), and SinoMed databases up to September 1, 2021. Eligible studies should report the association of the serum prealbumin level with prognosis and provide the multivariable-adjusted risk estimates of the outcomes of interest in HCC patients undergoing hepatectomy.
RESULTS
A total of 11 studies with 7,442 HCC patients were identified and analyzed. Meta-analysis of a fixed effects model showed that a low serum prealbumin level was associated with poor overall survival [hazard ratio (HR) = 1.54, 95% confidence interval (CI) = 1.42-1.68], recurrence-free survival (HR = 1.34, 95% CI = 1.17-1.52), and a higher risk of postoperative hepatic insufficiency (HR = 2.21; 95% CI = 1.36-3.60) in HCC patients. Sensitivity and subgroup analyses confirmed the robustness of low serum prealbumin in predicting poor overall survival.
CONCLUSIONS
This meta-analysis indicated that a low preoperative serum prealbumin level was significantly associated with adverse prognosis in HCC patients undergoing hepatectomy.
PubMed: 34746015
DOI: 10.3389/fonc.2021.775425 -
Medicine Feb 2024Nutritional problems in the early stages of severe burns are prominent and seriously affect the clinical outcomes of patients. Our aim is to analyze the effects of early... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Nutritional problems in the early stages of severe burns are prominent and seriously affect the clinical outcomes of patients. Our aim is to analyze the effects of early enteral nutrition (EEN) in patients with severe burns.
METHODS
In this study, relevant articles were searched in 8 English and Chinese data, with a time limit from the creation of the database to June 2023. Two researchers independently completed the search, screening and quality assessment of the articles. We conducted a systematic review and meta-analysis of randomized controlled trials that examined EEN therapy in people with severe burns. We compared the effects of EEN and non-EEN therapy in severely burned patients. The outcomes were mortality, gastrointestinal complications, nutritional indicators, gastrointestinal hormones, sepsis, length of hospital stay and wound healing time. Categorical variables were expressed as OR and 95% CI was calculated, and continuous variables were expressed as MD and 95% CI was calculated. The protocol for this systematic review was registered in PROSPERO on May 12, 2023 (identifier CRD42023422895).
RESULTS
Nineteen studies with a total of 1066 participants met the inclusion criteria. When comparing EEN therapy with non-EEN therapy, the experiment group had significantly lower mortality [OR = 0.39, 95% CI (0.20, 0.74), P = .004], lower wound healing time [MD = -10.77, 95% CI (-13.66,-7.88), P < .00001], fewer gastrointestinal complications [OR = 0.18, 95% CI (0.09, 0.36), P < .00001], lower rates of gastrointestinal hemorrhage [OR = 0.12, 95% CI (0.04, 0.36), P = .0001], lower rates of sepsis [OR = 0.40, 95% CI (0.24, 0.66), P = .0005], shorter length of hospital stay [MD = -12.08, 95% CI (-13.61, 9.19-10.56), P < .00001], and higher prealbumin levels [MD = 29.04, 95% CI (21.98, 36.10), P < .00001], higher total albumin levels [MD = 6.74, 95% CI (4.29, 9.19), P < .00001], and gastrin levels [MD = 15.93, 95% CI (10.12, 21.73), P < .00001]. However, there was no significant difference in albumin between the 2 groups [MD = 2.62, 95% CI (-0.30, 5.55), P = .08] or motilin levels [MD = 12.48, 95% CI (-43.59, 68.56), P = .66].
CONCLUSIONS
EEN plays an important role in the rehabilitation of patients with severe burns. EEN is beneficial to reduce complications and the length of hospital stay, maintain organ function, optimize the nutritional status of patients, promote wound healing, and improve the survival rate of patients.
Topics: Humans; Enteral Nutrition; Time Factors; Burns; Gastrointestinal Diseases; Sepsis; Length of Stay
PubMed: 38363893
DOI: 10.1097/MD.0000000000037023 -
World Journal of Gastroenterology Nov 2019It has been suggested that chronic pancreatitis (CP) may be an independent risk factor for development of cardiovascular disease (CVD). At the same time, it seems that...
BACKGROUND
It has been suggested that chronic pancreatitis (CP) may be an independent risk factor for development of cardiovascular disease (CVD). At the same time, it seems that congestive heart failure (CHF) and CP share the responsibility for the development of important clinical conditions such as sarcopenia, cachexia and malnutrition due to development of cardiac cachexia and pancreatic exocrine insufficiency (PEI), respectively.
AIM
To explore the evidence regarding the association of CP and heart disease, more specifically CVD and CHF.
METHODS
A systematic search of MEDLINE, Web of Science and Google Scholar was performed by two independent investigators to identify eligible studies where the connection between CP and CVD was investigated. The search was limited to articles in the English language. The last search was run on the 1st of May 2019. The primary outcomes were: (1) Incidence of cardiovascular event [acute coronary syndrome (ACS), chronic coronary disease, peripheral arterial lesions] in patients with established CP; and (2) Incidence of PEI in patients with CHF.
RESULTS
Out of 1166 studies, only 8 were eligible for this review. Studies regarding PEI and CHF showed an important incidence of PEI as well as associated malabsorption of nutritional markers (vitamin D, selenium, phosphorus, zinc, folic acid, and prealbumin) in patients with CHF. However, after substitution of pancreatic enzymes, it seems that, at least, loss of appetite was attenuated. On the other side, studies investigating cardiovascular events in patients with CP showed that, in CP cohort, there was a 2.5-fold higher incidence of ACS. In another study, patients with alcohol-induced CP with concomitant type 3c diabetes had statistically significant higher incidence of carotid atherosclerotic plaques in comparison to patients with diabetes mellitus of other etiologies. Earlier studies demonstrated a marked correlation between the clinical symptoms in CP and chronic coronary insufficiency. Also, statistically significant higher incidence of arterial lesions was found in patients with CP compared to the control group with the same risk factors for atherosclerosis (hypertension, smoking, dyslipidemia). Moreover, one recent study showed that PEI is significantly associated with the risk of cardiovascular events in patients with CP.
CONCLUSION
Current evidence implicates a possible association between PEI and malnutrition in patients with CHF. Chronic pancreatic tissue hypoxic injury driven by prolonged splanchnic hypoperfusion is likely to contribute to malnutrition and cachexia in patients with CHF. On the other hand, CP and PEI seem to be an independent risk factor associated with an increased risk of cardiovascular events.
Topics: Cachexia; Exocrine Pancreatic Insufficiency; Heart; Heart Failure; Humans; Malnutrition; Pancreas; Pancreatitis, Chronic; Risk Factors
PubMed: 31802835
DOI: 10.3748/wjg.v25.i44.6561 -
ESC Heart Failure Apr 2024The prevalence of transthyretin-associated amyloidosis cardiomyopathy (ATTR-CM) has grown because of newer non-invasive diagnosis tools. Detecting the presence of... (Review)
Review
The prevalence of transthyretin-associated amyloidosis cardiomyopathy (ATTR-CM) has grown because of newer non-invasive diagnosis tools. Detecting the presence of extra-cardiac ATTR manifestations such as musculoskeletal pathologies considered 'red flags', when there is minimal or non-cardiac clinical involvement is primordial to carry out an early diagnosis. The aim of this systematic review is to examine the prevalence of musculoskeletal, ATTR-deposition-related co-morbidities in patients already diagnosed with ATTR-CM, specifically carpal tunnel syndrome, ruptured biceps tendon, spinal stenosis, and trigger finger. We performed a systematic review using PRISMA guidelines. Inclusion criteria were all studies in English and Spanish language and participants had to be patients diagnosed with ATTR-CM, by any diagnostic method, with the musculoskeletal co-morbidities subject of this review. The quality of the studies was based on the Risk of Bias Tool. This systematic review included 22 studies for final analysis. Carpal tunnel syndrome is reported in 21 studies, brachial biceps tendon rupture is reported in three, and spinal stenosis in eight studies. No articles that accomplished all the inclusion criteria for trigger finger were found. Regarding to the quality of the studies, all of them were categorized as being of high and moderate quality. The frequent association between ATTR-CM and carpal tunnel syndrome, ruptured biceps tendon, and lumbar spinal is confirmed, and the onset of these co-morbidities usually precedes the diagnosis of by years. This association defines them as red flags that should be search proactively due to the current treatment possibilities and the severity of the presentation of cardiac amyloidosis.
Topics: Humans; Prealbumin; Spinal Stenosis; Carpal Tunnel Syndrome; Trigger Finger Disorder; Amyloid Neuropathies, Familial; Cardiomyopathies; Morbidity
PubMed: 38130034
DOI: 10.1002/ehf2.14622 -
Medicine Sep 2020Not only has the placement rate of enteral feeding tubes during operations for esophageal cancer increased, but also has number of patients who choose to continue... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Not only has the placement rate of enteral feeding tubes during operations for esophageal cancer increased, but also has number of patients who choose to continue enteral feeding at home instead of removing the feeding tube at discharge. The impacts of home enteral nutrition (HEN) after esophagectomy in esophageal cancer patients are analyzed.
METHODS
A systematic review was conducted in accordance with PRISMA and Cochrane guidelines. English and Chinese databases, including PubMed, Embase, Web of Science, The Cochrane Library, Scopus, CBM, CNKI, and Wan Fang were searched from inception to December 7, 2019. Randomized controlled trials evaluating the short-term outcomes of HEN following esophagectomy in cancer patients were included. The risk of bias of the included studies was appraised according to the Cochrane risk of bias tool. The summary of relative risk/weighted mean difference (WMD) estimates and corresponding 95% confidence interval (95% CI) were calculated using fixed- and random-effects models.
RESULTS
Nine randomized controlled trials involving 757 patients were included in the meta-analysis. Compared with oral diet, HEN was associated with significantly increased body weight (WMD 3 kg, 95% CI 2.36-3.63, P < .001), body mass index (WMD 0.97 kg/m, 95% CI 0.74-1.21, P < .001), albumin (WMD 3.43 g/L, 95% CI 2.35-4.52, P < .001), hemoglobin (WMD 7.23 g/L, 95% CI 5.87-8.59, P < .001), and total protein (WMD 5.13 g/L, 95% CI 3.7-6.56, P < .001). No significant differences were observed in prealbumin and gastrointestinal adverse reactions. Physical (WMD 8.82, 95% CI 6.69-10.95, P < .001) and role function (WMD 12.23, 95% CI 2.72-21.74, P = .01) were also significantly better in the HEN group. The nausea/vomiting (WMD -5.43, 95% CI -8.29 to -2.57, P = .002) and fatigue symptoms (WMD -11.76, 95% CI -16.21 to -7.32, P < .001) were significantly reduced. Appetite loss (WMD -8.48, 95% CI -14.27 to -4.88, P = .001), diarrhea (WMD -3.9, 95% CI -7.37 to -0.43, P = .03), and sleep disturbance (WMD -7.64, 95% CI -12.79 to -2.5, P = .004) in the HEN group were also significantly less than the control group.
CONCLUSIONS
HEN improved nutrition status, physical and role function, and reduced nausea/vomiting, fatigue, appetite loss, diarrhea, and sleep disturbance compared with an oral diet in esophageal cancer patients postsurgery. HEN did not increase adverse reactions.
Topics: Enteral Nutrition; Esophageal Neoplasms; Esophagectomy; Home Care Services; Humans
PubMed: 32899043
DOI: 10.1097/MD.0000000000021988 -
Medicine Jan 2020Whether combined parenteral nutrition (PN) and enteral nutrition (EN) is superior to EN alone remains controversial. (Meta-Analysis)
Meta-Analysis
BACKGROUND
Whether combined parenteral nutrition (PN) and enteral nutrition (EN) is superior to EN alone remains controversial.
OBJECTIVES
This study aimed to evaluate the efficacy and safety of combined PN and EN versus EN alone for critically ill patients based on published randomized controlled trials (RCTs).
DATA SOURCES
Studies designed as RCTs evaluating the treatment effectiveness of combined PN and EN versus EN alone for critically ill patients were identified from PubMed, Embase, and the Cochrane Library from inception to April 2019.
METHODS
The pooled relative risks and weighted mean differences with corresponding 95% confidence intervals were calculated using the random-effects model. Twelve RCTs recruiting a total of 5609 adults and 1440 children were selected for the final meta-analysis.
RESULTS
The summary relative risks indicated that combined PN and EN was not associated with the risk of all-cause mortality, respiratory infection, urinary tract infection, and nutrition-related complications. Moreover, combined PN and EN was associated with longer hospital stay and higher albumin and prealbumin levels compared with EN alone. No significant differences were, however, found between combined PN and EN and EN alone in terms of ventilatory support, intensive care unit stay, and transferrin and C-reactive protein levels.
CONCLUSIONS
This study showed that combined PN and EN significantly increased hospital stay duration and albumin and prealbumin levels compared with EN alone for critically ill patients. Large-scale RCTs should be conducted to compare the treatment effectiveness of combined PN and EN versus EN alone for critically ill patients due to a specific cause.
Topics: Critical Illness; Enteral Nutrition; Humans; Parenteral Nutrition; Randomized Controlled Trials as Topic
PubMed: 32011471
DOI: 10.1097/MD.0000000000018778 -
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