Objective: To investigate the histological features and clinical manifestations in different types of cardiac amyloidosis to improve diagnostic accuracy. Methods: The histopathological features and clinical manifestations of 48 patients diagnosed with cardiac amyloidosis by Congo red stain and electron microscopy through endomyocardial biopsy were collected in West China Hospital of Sichuan University from January 2018 to December 2021. Immunohistochemical stains for immunoglobulin light chains (κ and λ) and transthyretin protein were carried out, and a review of literature was made. Results: The patients age ranged from 42 to 79 years (mean 56 years) and the male to female ratio was 1.1 to 1.0. The positive rate of endomyocardial biopsy was 97.9% (47/48), which was significantly higher than that of the abdominal wall fat (7/17). Congo red staining and electron microscopy were positive in 97.9% (47/48) and 93.5% (43/46), respectively. Immunohistochemical stains showed 32 cases (68.1%) were light chain type (AL-CA), including 31 cases of AL-λ type and 1 case of AL-κ type; 9 cases (19.1%) were transthyretin protein type (ATTR-CA); and 6 cases (12.8%) were not classified. There was no significant difference in the deposition pattern of amyloid between different types (P>0.05). Clinical data showed that ATTR-CA patients had less involvement of 2 or more organs and lower N-terminal pro-B-type natriuretic peptide (NT-proBNP) than the other type patients (P<0.05). The left ventricular stroke volume and right ventricular ejection fraction of ATTR-CA patients were better than the other patients (P<0.05). Follow-up data of 45 patients was obtained, and the overall mean survival time was 15.6±2.0 months. Univariate survival analysis showed that ATTR-CA patients had a better prognosis, while cardiac amyloidosis patients with higher cardiac function grade, NT-proBNP >6 000 ng/L, and troponin T >70 ng/L had a worse prognosis (P<0.05). Multivariate survival analysis showed that NT-proBNP and cardiac function grade were independent prognostic factors for cardiac amyloidosis patients. Conclusions: AL-λ is the most common type of cardiac amyloidosis in this group. Congo red staining combined with electron microscopy can significantly improve the diagnosis of cardiac amyloidosis. The clinical manifestations and prognosis of each type are different and can be classified based on immunostaining profile. However, there are still a few cases that cannot be typed; hence mass spectrometry is recommended if feasible.
Humans ; Male ; Female ; Adult ; Middle Aged ; Aged ; Prealbumin/metabolism* ; Stroke Volume ; Cardiomyopathies/pathology* ; Congo Red ; Ventricular Function, Right ; Amyloidosis/pathology* ; Prognosis

Humans ; Cardiomyopathies/genetics* ; Prealbumin/genetics*

Humans ; Hospital Mortality ; Prealbumin ; Propensity Score ; Heart Failure ; Natriuretic Peptide, Brain ; Peptide Fragments ; Biomarkers

Transthyretin (TTR) is a small liver-secreted plasma protein that shows close correlations with changes in lean body mass (LBM) during the entire human lifespan and agglomerates the bulk of nitrogen (N)-containing substrates, hence constituting the cornerstone of body building. Amino acids (AAs) dietary restriction causes inhibition of TTR production and impairs the accretion of LBM reserves. Inflammatory disorders result in cytokine-induced abrogation of TTR synthesis and urinary leakage of nitrogenous catabolites. Taken together, the data indicate that malnutrition and inflammation may similarly suppress the production of TTR through distinct and unrelated pathophysiological mechanisms while operating in concert to downsize LBM stores. The hepatic synthesis of TTR integrates both machineries, acting as a marker of reduced LBM resources still available for defense and repair processes. TTR operates as a universal surrogate analyte that allows for the grading of residual LBM capacity to reflect disease burden. Measurement of TTR is a simple, rapid, and inexpensive micro-method that may be reproduced on a daily basis, hence ideally suited for the follow-up of the most intricated clinical situations and as a reliable predictor of any morbidity outcome.
Biomarkers ; Humans ; Inflammation/metabolism* ; Liver/metabolism* ; Prealbumin

Objective: To explore the effects of enteral immunonutrition support therapy on nutritional metabolism, immune function, and inflammatory response in adult burn patients at nutritional risk as assessed by the modified 2^nd nutrition risk screening (NRS) 2002. Methods: A prospective randomized controlled study was conducted. From December 2019 to January 2022, 500 adult patients who were admitted to the Affiliated Huaihai Hospital of Xuzhou Medical University and had nutritional risk assessed by the modified 2^nd NRS 2002 were recruited into the study. According to burn severity, the patients were divided into common burn patients (n=450) and severe burn patients (n=50). According to the random number table, the patients with common burn were divided into common burn diet nutrition group and common burn diet enteral immunonutrition group, with 225 patients in each group, and the patients with severe burn were divided into severe burn diet enteral non-immunonutrition group and severe burn diet enteral immunonutrition group, with 25 patients in each group. The patients in each group were given the corresponding nutritional support therapies on the basis of routine burn treatment. On post injury day (PID) 1, 3, 7, 14, and 21, the total energy intake and total protein intake of the patients in 4 groups were recorded, the plasma prealbumin, albumin, transferrin, serum immunoglobulin A (IgA), IgG, IgM, peripheral blood CD3 positive T cell percentage, CD4 positive T cell count, CD8 positive T cell count, the ratio of CD4 positive T cells to CD8 positive T cells, natural killer cell percentage, plasma interleukin-6 (IL-6), free mitochondrial DNA (mtDNA) copy number, and soluble triggering receptor expressed on myeloid cells-1 (sTREM-1) of the patients in 4 groups were detected, and the nitrogen balance of the patients in 4 groups on the day was calculated. On PID 7, 14, and 21, the modified 2^nd NRS 2002 scores of the patients in 4 groups were reassessed. The sepsis incidence during treatment and the length of hospital stay of the patients in 4 groups and the length of intensive care unit (ICU) stay of the patients in the 2 severe burn groups were recorded. Data were statistically analyzed with chi-square test, Fisher's exact probability test, Mann-Whitney U test, independent sample t test, analysis of variance for repeated measurement, and Bonferroni correction. Results: A total of 476 patients completed the trial, with 213 patients in common burn diet nutrition group (112 males and 101 females, aged (37±19) years), 218 patients in common burn diet enteral immunonutrition group (115 males and 103 females, aged (42±16) years), 22 patients in severe burn diet enteral non-immunonutrition group (11 males and 11 females, aged (35±8) years), and 23 patients in severe burn diet enteral immunonutrition group (12 males and 11 females, aged (35±8) years). Compared with those in common burn diet nutrition group, the patients in common burn diet enteral immunonutrition group had significantly higher total energy intake on PID 1 (t=6.06, P<0.01), significantly lower total energy intake on PID 7 and significantly lower total protein intake on PID 1 (with t values of 6.17 and 4.59, respectively,P<0.01). On PID 21, the total energy intake of patients in severe burn diet enteral immunonutrition group was significantly lower than that in severe burn diet enteral non-immunonutrition group (t=2.70, P<0.01). The total protein intake of patients in severe burn diet enteral immunonutrition group and severe burn diet enteral non-immunonutrition group were similar at each time point post injury (P>0.05). Compared with those in common burn diet nutrition group, the patients in common burn diet enteral immunonutrition group had significantly higher level of prealbumin on PID 3, 7, 14, and 21 (with t values of 2.05, 2.33, 2.45, and 2.11, respectively, P<0.05), significantly higher level of albumin on PID 7, 14, and 21 (with t values of 2.30, 2.56, and 2.15, respectively, P<0.05), significantly higher level of transferrin on PID 7 and 14 (with t values of 1.99 and 2.27, respectively, P<0.05), significantly higher nitrogen balance on PID 14 and 21 (with t values of 2.51 and 2.07, respectively, P<0.05), and significantly lower modified 2^nd NRS 2002 score on PID 21 (t=1.99, P<0.05). Compared with those in severe burn diet enteral non-immunonutrition group, the patients in severe burn diet enteral immunonutrition group had significantly higher level of prealbumin on PID 3, 7, 14, and 21 (with t values of 2.50, 2.64, 2.18, and 2.39, respectively, P<0.05), significantly higher level of albumin​on PID 7, 14, and 21 (with t values of 2.27, 2.39, and 2.69, respectively, P<0.05), significantly higher level of transferrin and nitrogen balance but significantly lower modified 2^nd NRS 2002 score on PID 14 and 21 (with t values of 2.30, 2.35, 2.41, 2.16, 2.31, and 2.73, respectively, P<0.05). Compared with those in common burn diet nutrition group, patients in common burn diet enteral immunonutrition group had significantly higher level of IgA and IgG on PID 7, 14, and 21 (with t values of 2.19, 2.36, 2.17, 2.49, 1.97, and 2.24, respectively, P<0.05), significantly higher level of IgM on PID 21 (t=2.06, P<0.05), significantly higher percentage of CD3 positive T cells and ratio of CD4 positive T cells to CD8 positive T cells on PID 3, 7, 14, and 21 (with t values of 2.49, 2.25, 2.33, 2.41, 2.39, 2.24, 2.46, and 2.18, respectively, P<0.05), significantly higher CD4 positive T cell count (with t values of 2.15 and 2.27, respectively, P<0.05) but significantly lower CD8 positive T cell count on PID 14 and 21 (with t values of 2.58 and 2.35, P<0.05), and significantly higher percentage of natural killer cells on PID 7, 14, and 21 (with t values of 2.53, 2.21, and 2.36, respectively, P<0.05). Compared with those in severe burn diet enteral non-immunonutrition group, patients in severe burn diet immunonutrition group had significantly higher level of IgA on PID 7 and 14 (with t values of 2.15 and 2.03, respectively, P<0.05), significantly higher level of IgG on PID 7, 14, and 21 (with t values of 2.09, 2.56, and 2.15, respectively, P<0.05), significantly higher level of IgM on PID 21 (t=2.08, P<0.05), significantly higher percentage of CD3 positive T cells, CD4 positive T cell count, and percentage of natural killer cells on PID 14 and 21 (with t values of 2.52, 2.14, 2.14, 2.39, 2.56, and 2.19, respectively, P<0.05), significantly lower CD8 positive T cell count but significantly higher ratio of CD4 positive T cells to CD8 positive T cells on PID 7, 14, and 21 (with t values of 2.27, 2.81, 2.01, 2.11, 2.69, and 2.05, respectively, P<0.05). Compared with those in common burn diet nutrition group, patients in common burn diet enteral immunonutrition group had significantly lower level of IL-6 (with t values of 2.34 and 2.32, respectively, P<0.05) and significantly lower free mtDNA copy number on PID 14 and 21 (with Z values of -2.28 and -2.34,respectively, P<0.05), significantly lower level of sTREM-1 on PID 7, 14, and 21 (with t values of 2.02, 2.94, and 3.72, respectively, P<0.05). Compared with those in severe burn diet enteral non-immunonutrition group, patients in severe burn diet enteral immunonutrition group had significantly lower level of IL-6 and sTREM-1 on PID 7, 14, and 21 (with t values of 2.15, 2.29, 2.47, 2.43, 2.07, and 2.32, respectively, P<0.05), and significantly lower free mtDNA copy number on PID 14 and 21 (with Z values of -2.49 and -2.21, respectively, P<0.05). During treatment, the sepsis incidences of patients in 2 common burn groups were similar (P>0.05), the sepsis incidences of patients in 2 severe burn groups were similar (P>0.05). The length of ICU stay of patients in severe burn diet enteral immunonutrition group was (11±3) d, which was significantly shorter than (14±3) d in severe burn diet enteral non-immunonutrition group (t=3.12, P<0.01). The length of hospital stay of patients in common burn diet enteral immunonutrition group was significantly shorter than that in common burn diet nutrition group (t=3.11, P<0.01). The length of hospital stay of patients in severe burn diet enteral non-immunonutrition group was similar to that in severe burn diet enteral immunonutrition group (P>0.05). Conclusions: Enteral immunonutrition support therapy for adult burn patients at nutritional risk assessed by the modified 2^nd NRS 2002 can better improve the nutritional status and the immune function of patients, reduce inflammatory response of the body, and shorten the length of hospital stay in common burn patients and the length of ICU stay in severe burn patients.
Adult ; Burns/therapy* ; DNA, Mitochondrial ; Enteral Nutrition ; Female ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Interleukin-6 ; Male ; Middle Aged ; Nitrogen ; Prealbumin ; Prospective Studies ; Sepsis ; Transferrins

Objective: To summarize and analyze the clinical effect of fecal microbiota transplantation (FMT) combined with nutritional support and psychotherapy in patients with "Tetralogy of Tongji" (comprising chronic gastrointestinal dysfunction, mental and psychological disorders, malnutrition, and endocrine disorders). Methods: A longitudinal study was conducted. The inclusion criteria were as follows: (1) patients were under 70 years of age; (2) patients exhibited chronic gastrointestinal dysfunction (in accordance with the Rome IV diagnostic criteria for irritable bowel syndrome ie. chronic functional constipation, diarrhea, abdominal pain and abdominal distention) with onset occurring more than one year previously; (3) patients exhibited malnutrition (body mass index ≤ 18.5 kg/m²); (4) patients exhibited depression, anxiety, or state as diagnosed by a psychologist using the Hamilton anxiety rating scale (HAMA) and the Hamilton depression scale (HAMD); (5) patients were women of childbearing age with amenorrhea or menstrual disorder with a duration ≥6 months. Patients were excluded if they exhibited gastrointestinal bleeding, short bowel syndrome, radiation-induced intestinal injury, intestinal obstruction or inflammatory bowel disease, recurrent/metastatic tumors, systemic infectious diseases, life-threatening systemic comorbidities, intorlerate to nasojejunal, percutaneous gastrostomy / jejunostomy or FMT. The clinical data of 43 patients at Shanghai Tenth People's Hospital exhibiting the "Tetralogy of Tongji" and who received microflora transplantation combined with nutritional support and psychotherapy from June 2017 to June 2021 was prospectively collected. There were 12 males and 31 females with a mean age of 35.2±16.7 years. All 43 patients had chronic gastrointestinal dysfunction. Of these, 24 patients had depression and 19 had anxiety. There were 26 women of reproductive age, including 13 cases of menstrual disorder and 9 cases of amenorrhea. The treatment intervention was a combination of FMT (microflora solution or microflora capsule), nutritional support (enteral nutrition) and psychological intervention. The following were assessed before treatment and 1, 3, 6 months after treatment: (1) gastrointestinal function was assessed using the gastrointestinal symptoms rating scale (GSRS), where a higher score is indicative of more serious gastrointestinal symptoms, and the gastrointestinal quality of life index (GIQLI), where a higher score is indicative of higher quality of life; (2) psychological status was assessed using HAMA and HAMD scores, where a lower score is indicative of reduced severity of anxiety or depression symptoms, respectively; (3) nutritional status was assessed by measurements of total blood protein, albumin, fibrinogen and prealbumin, as well as measurements of body mass and body mass index (BMI); (4) neuroendocrine function was assessed by measurement of blood levels of cortisol, dopamine and noradrenaline, as well as menstruation in women of reproductive age. Results: The follow-up rates at 1, 3 and 6 months after treatment were 90.7% (39/43), 72.1% (31/43) and 55.8% (24/43), respectively. The total effective rate for chronic gastrointestinal dysfunction was 81.4% (35/43), of which the average GSRS score decreased from 29.35±3.56 before treatment to 18.25±2.56 in the sixth month (P<0.001). The average GIQLI score increased from 56.23±10.34 before treatment to 91.04±20.39 in the sixth month (P<0.001). All patients had malnutrition before treatment. After 6 months, their body weight had increased from 40.61±8.88 kg to 50.45±6.23 kg (P<0.001), and BMI had increased from 15.17±1.87 kg/m² to 19.58±1.42 kg/m² (P<0.001). The average total protein level was 60.99± 5.99 g/L before treatment. After 6 months, this had increased to 64.21±4.23 g/L (F=2.715, P=0.022). The average prealbumin level increased from 150.14±56.04 mg/L before treatment to 258.17±86.94 mg/L after 6 months (F=15.124, P<0.001). In this study, 24 patients with depression/depressed state were included. After treatment, the average HAMD score in these patients decreased from 22.79±6.63 before treatment to 9.92±7.24 after 6 months (P<0.001). There were 19 patients with anxiety disorder/anxiety state. After treatment, the average HAMA score in these patients decreased from 17.15±4.34 before treatment to 7.73±4.10 after 6 months (P<0.001). Observing the endocrine efficacy of 26 women of childbearing age, it was found that the effective rate of this treatment on endocrine regulation was 69.2% (18/26). Although there was no significant change in blood cortisol levels after 6 months, average blood dopamine levels decreased from 32.91±10.65 nmol/L before treatment to 13.02±5.58 nmol/L after 6 months (P<0.001). Average blood norepinephrine levels decreased from 49.75±15.23 ng/L before treatment to 19.21±9.58 ng/L after 6 months (P<0.001). Conclusion: The strategy of FMT combined with nutritional support and psychological intervention is effective in improving the symptoms of the "Tetralogy of Tongji".
Adolescent ; Adult ; Amenorrhea ; China ; Constipation ; Dopamine ; Fecal Microbiota Transplantation ; Female ; Fibrinogen ; Gastrointestinal Diseases ; Humans ; Hydrocortisone ; Infant ; Longitudinal Studies ; Male ; Malnutrition ; Middle Aged ; Norepinephrine ; Nutritional Support ; Prealbumin ; Psychosocial Intervention ; Quality of Life ; Treatment Outcome ; Young Adult

Objective: To investigate the association between prealbumin and the long-term prognosis of patients with hilar cholangiocarcinoma(HCCA) following radical surgery. Methods: The clinical data of 262 HCCA patients who underwent radical surgery admitted from January 2010 to January 2017 at the First Affiliated Hospital of Army Medical University were collected,retrospectively. There were 158 males and 104 females; aged (57.6±9.9)years old(range:32 to 78 years). According to the preoperative serum prealbumin level(170 mg/L),the patients were divided into low prealbumin group(n=143) and normal prealbumin group(n=119). Follow-up until September 2020,the main research indicator was overall survival(OS), and the secondary research indicator was recurrence-free survival(RFS). The measurement data conforming to the normal distribution adopted the t test,the measurement data not conforming to the normal distribution adopted the Mann-Whitney U test,and the count data adopted the χ² test. The Kaplan-Meier method was used to calculate the cumulative survival rate. The Log-rank test was used for univariate analysis of the cumulative survival rate. Variables with P<0.10 in univariate analysis were included in the Cox proportional hazards model for multivariate analysis. Results: The 1-, 3-, and 5-year OS rate of the 262 patients was 73.4%, 32.1%, and 24.0%, respectively, and the 1-, 3-, and 5-year RFS rate was 54.6%, 25.2%, and 16.2%, respectively. Median OS and RFS were 21 months and 12 months for patients with low prealbumin and 25 months and 19 months for patients with normal prealbumin. The OS rate and RFS rate of patients in the low prealbumin group were lower than those in the normal prealbumin group, and the difference was statistically significant (both P<0.05). The results of univariate analysis indicated that low prealbumin, CA19-9>150 U/L, tumor infiltration length>3 cm, preoperative jaundice, macrovascular invasion, microvascular invasion, lymph node metastasis, and poor differentiation maybe the risk factors of OS,and low prealbumin,tumor invasion length>3 cm,macrovascular invasion, microvascular invasion,lymph node metastasis,and poor differentiation maybe the risk factors of RFS for postoperative for radical resection in patients with HCCA (all P<0.10). Multivariate results suggested that low prealbumin,tumor invasion length>3 cm,microvascular invasion,lymph node metastasis,and poor differentiation were independent risk factors affecting OS and RFS in patients with HCCA after radical operation (all P<0.05). Conclusion: Preoperative prealbumin level can predict the long-term prognosis of patients with hilar cholangiocarcinoma following radical surgery.
Adult ; Aged ; Bile Duct Neoplasms/surgery* ; Cholangiocarcinoma/surgery* ; Female ; Humans ; Klatskin Tumor/surgery* ; Lymphatic Metastasis ; Male ; Middle Aged ; Prealbumin ; Prognosis ; Retrospective Studies

Amyloid Neuropathies, Familial/drug therapy* ; Benzoxazoles ; Humans ; Prealbumin

Amyloid Neuropathies, Familial/genetics* ; Humans ; Prealbumin/genetics*

China ; Consensus ; Heart ; Humans ; Medicine, Chinese Traditional ; Prealbumin