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

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

OBJECTIVETo investigate the application of bundles of intervention in the treatment of esophageal carcinoma anastomotic leak.

METHODSFrom January 2014 to May 2015, 44 cases of esophageal carcinoma anastomotic fistula were treated by bundles of intervention (through the collection of a series of evidence-based treatment and care measures for the treatment of diseases) in Department of Thoracic Surgery, Huai'an First Hospital, Nanjing Medical University (bundles of intervention group), and 68 patients with esophageal carcinoma postoperative anastomotic leak from December 2013 to January 2012 receiving traditional therapy were selected as the control group. The clinical and nutritional indexes of both groups were compared.

RESULTSThere were no significant differences in general data and proportion of anastomotic leak between the two groups. Eleven patients died during hospital stay, including 3 cases in bundles of intervention group(6.8%) and 8 cases in control group (11.8%) without significant difference(P = 0.390). In bundles of intervention group, 1 case died of type III( intrathoracic anastomotic leak, 2 died of type IIII( intrathoracic anastomotic leak. In control group, 2 cases died of type III( cervical anastomotic leak, 2 died of type III( intrathoracic anastomotic leak and 4 of type IIII( intrathoracic anastomotic leak. The mortality of bundles of intervention group was lower than that of control group. The duration of moderate fever [(4.1±2.4) days vs. (8.3±4.4) days, t=6.171, P=0.001], the time of antibiotic use [(8.2±3.8) days vs.(12.8±5.2) days, t=5.134, P = 0.001], the healing time [(21.5±12.7) days vs.(32.2±15.8) days, t=3.610, P=0.001] were shorter, and the average hospitalization expenses[(63±12) thousand yuan vs. (74±19) thansand yuan, t=3.564, P=0.001] was lower in bundles of intervention group than those in control group. Forty-eight hours after occurrence of anastomotic leak, the levels of hemoglobin, albumin and prealbumin were similar in both groups. However, at the time of fistula healing, the levels of hemoglobin [(110.6±10.5) g/L vs.(103.8±11.1) g/L, t=3.090, P=0.002], albumin [(39.2±5.2) g/L vs.(36.3±5.9) g/L, t=2.543, P=0.013] and prealbumin [(129.3±61.9) g/L vs.(94.1±66.4) g/L, t=2.688, P=0.008] were significantly higher in bundles of intervention group.

CONCLUSIONIn the treatment of postoperative esophageal carcinoma anastomotic leak, application of bundles of intervention concept can significantly improve the nutritional status and improve the clinical outcomes.

Anastomotic Leak ; mortality ; therapy ; Anti-Infective Agents ; therapeutic use ; Carcinoma ; complications ; surgery ; Esophageal Fistula ; complications ; mortality ; therapy ; Esophageal Neoplasms ; complications ; surgery ; Esophagectomy ; adverse effects ; mortality ; Female ; Fever ; epidemiology ; etiology ; Hemoglobins ; metabolism ; Hospital Costs ; statistics & numerical data ; Humans ; Male ; Middle Aged ; Nutritional Status ; Patient Care Bundles ; mortality ; statistics & numerical data ; Prealbumin ; metabolism ; Serum Albumin ; metabolism ; Treatment Outcome

OBJECTIVETo investigate the nutritional status in acute stage ischemic stroke and its relation to disease severity and prognosis of patients.

METHODSFifty patients with ischemic stroke were admitted in hospital within 48 h after onset. National Institute of Health stroke scale (NIHSS) was used to assess the severity of stroke. Physical index and laboratory index were measured on d1, d7 and d14 after admission. Physical index included body weight, body mass index, triceps skin folds, upper arm circumference and arm muscle circumference. Laboratory index included prealbumin, high sensitivity C-reactive protein (hs-CRP), complement C3 and cortisol. The severity of metabolic disturbance was expressed as the difference of biochemical indexes between the d7 and d1. All cases were followed up for 6 months. The prognosis of stroke was evaluated with modified Rankin (mRankin) scores.

RESULTSNo significant changes of physical indexes were found between d7 and d1. The levels of prealbumin and complement C3 on d7 after admission were significantly decreased compared to d1 (198.8 mg/L±20.3 mg/L vs 286.7 mg/L±23.8 mg/L and 0.6 g/L±0.1 g/L vs 1.0 g/L±0.1 g/L, respectively, both P<0.05). The levels of hs-CRP and cortisol at d7 were significantly increased compared to d1 (495.2 nmol/L±39.5 nmol/L vs 24.1 mg/L±5.2 mg/L and 396.4 nmol/L±41.3 nmol/L vs 5.1 mg/L±1.2 mg/L, respectively, both P<0.05). On d14 after admission hs-CRP (13.2 mg/L±4.5 mg/L) and cortisol levels (463.4 nmol/L±32.1 nmol/L) were still significantly higher than d1 (both P<0.05). However, there were no difference in prealbumin (259.2 mg/L±22.8 mg/L) and complement C3 (0.8 g/L±0.2 g/L) levels between d1 and d14 after admission. Correlation analysis revealed that the NIHSS scores and mRankin scores were correlated with nutrition metabolism disturbances (P<0.05).

CONCLUSIONNutrition metabolism disturbances in patients with acute ischemic stroke are related to the disease duration, the severity and prognosis of stroke.

C-Reactive Protein ; metabolism ; Complement C3 ; metabolism ; Humans ; Hydrocortisone ; blood ; Nutritional Status ; Prealbumin ; metabolism ; Prognosis ; Severity of Illness Index ; Stroke ; diagnosis ; physiopathology

Phenylketonuria is an autosomal recessive disorder caused by a deficiency of phenylalanine hydroxylase. Transthyretin has been implicated as an indicator of nutritional status in phenylketonuria patients. In this study, we report that phenylalanine and its metabolite, phenylpyruvic acid, affect MAPK, changing transthyretin expression in a cell- and tissue-specific manner. Treatment of HepG2 cells with phenylalanine or phenylpyruvic acid decreased transcription of the TTR gene and decreased the transcriptional activity of the TTR promoter site, which was partly mediated through HNF4alpha. Decreased levels of p38 MAPK were detected in the liver of phenylketonuria-affected mice compared with wild-type mice. In contrast, treatment with phenylalanine increased transthyretin expression and induced ERK1/2 activation in PC-12 cells; ERK1/2 activation was also elevated in the brainstem of phenylketonuria-affected mice. These findings may explain between-tissue differences in gene expression, including Ttr gene expression, in the phenylketonuria mouse model.
Animals ; Brain Stem/metabolism/pathology ; Disease Models, Animal ; Gene Expression Regulation ; Hep G2 Cells ; Hepatocyte Nuclear Factor 4/metabolism ; Humans ; Liver/*metabolism/pathology ; Mice ; Mice, Mutant Strains ; Mitogen-Activated Protein Kinase 3/genetics/*metabolism ; Organ Specificity ; Phenylalanine/metabolism ; Phenylalanine Hydroxylase/deficiency ; Phenylketonurias/*genetics/metabolism/pathology/physiopathology ; Phenylpyruvic Acids/metabolism ; Prealbumin/*biosynthesis/genetics ; p38 Mitogen-Activated Protein Kinases/genetics/*metabolism

OBJECTIVETo analyze the proteomic characteristics of Gan (肝)-stagnancy syndrome (GSS) by seeking the differential protein in blood and tissues of GSS model rats.

METHODSGSS model rats were established by chronic restraint stress, keeping rats in restrain chamber for 6 h every day for 21 successive days. Their blood and liver samples were collected at the end of experiment for differential protein detection with methods of isoelectrofocusing and polyacrylamide SDS-PAGE, silver staining, and scanning. The gel images were analyzed with Imagemaster 2D Elite software, and the excavated differential protein spots were identified with matrix assistant laser resolving TOF mass spectrometry, Western blot, ELISA, and RT-PCR, respectively.

RESULTSA method for isolating the protein in blood serum and tissues by two-dimensional gel electrophoresis was established and optimized. Six serum proteins and three liver proteins that differentially expressed were identified. The down-regulated differential proteins in serum of GSS model rats were serum albumin precursor, beta 1 globin, antibody against muscle acetylcholine receptor, Ig lambda-2 C region, and transthyretin (TTR), and those in liver tissue were aryl sulfotransferase, enoyl-CoA hydratase, and TTR. TTR down-regulation was found in both serum and liver. Preliminary biological information analysis showed that these differential proteins involved in immune, neuroendocrine, nutrition, and substance metabolism.

CONCLUSIONProteomic analysis of differential proteins showed that TTR, aryl sulfotransferase, and enoyl-CoA hydratase expressions are downregulated in the GSS model rats, suggesting that the susceptibility of cancer could be enhanced by chronic stress.

Amino Acid Sequence ; Animals ; Chronic Disease ; Disease Models, Animal ; Electrophoresis, Gel, Two-Dimensional ; Liver ; metabolism ; Male ; Molecular Sequence Data ; Prealbumin ; genetics ; Proteomics ; methods ; Rats ; Rats, Wistar ; Reproducibility of Results ; Restraint, Physical ; Silver Staining ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization ; Stress, Psychological ; complications ; metabolism ; Syndrome ; Transcription, Genetic

OBJECTIVETo assess the risk factors of stress-related ulcer and gastrointestinal hemorrhage after pancreaticoduodenectomy.

METHODSFrom May 1999 to July 2007, 285 periampullary cancer patients underwent pancreaticoduodenectomy in our hospital. The clinical data, pathological results, type of operation, and postoperative treatment were retrospectively analyzed. Patients with stress-related ulcer and gastrointestinal hemorrhage were selected for risk factor analysis, and other patients were taken as control group.

RESULTS35 patients (12.3%) developed stress-related ulcer and gastrointestinal hemorrhage following pancreaticoduodenectomy. Pathological examination showed pancreatic cancer in 5 cases, duodenal cancer in 8, common bile duct cancer in 10, ampullary carcinoma in 11, and solid-pseudopapillary tumors in 1. Single variate analysis demonstrated that alcohol, preoperative bilirubin level, operation time, lymph node metastasis, prealbumin decrease after operation and other complication were significantly associated with the stress-related ulcer and gastrointestinal hemorrhage. Logistic regression in multivariate analysis revealed that preoperative bilirubin level, operation time, other complication, prealbumin decrease after surgery were independent risk factors.

CONCLUSIONStress-related ulcer and gastrointestinal hemorrhage are one of the most common complications after pancreaticoduodenectomy. Preoperative bilirubin level, operation time, other complications, and prealbumin decrease after operation are four independently risk factors.

Adolescent ; Adult ; Aged ; Alcoholism ; complications ; Ampulla of Vater ; Bilirubin ; blood ; Common Bile Duct Neoplasms ; complications ; pathology ; surgery ; Duodenal Neoplasms ; complications ; pathology ; surgery ; Female ; Gastrointestinal Hemorrhage ; etiology ; Humans ; Lymphatic Metastasis ; Male ; Middle Aged ; Multivariate Analysis ; Pancreatic Neoplasms ; complications ; pathology ; surgery ; Pancreaticoduodenectomy ; adverse effects ; Peptic Ulcer ; etiology ; Prealbumin ; metabolism ; Retrospective Studies ; Risk Factors ; Stress, Psychological ; complications ; Young Adult

GLuc (Gaussia luciferase) is a secreted luciferase with high sensitivity. In this study, we primarily compared expression character of PTTR with that of PCMV, relied on easy secretion, high sensitivity and simple and fast detection of GLuc. We firstly constructed two plasmids pAAV2-neo-TTR-GLuc and pAAV2-neo-CMV-GLuc. Then, 4 cell lines were transfected with the two plasmids in aid of Lipofectamine 2000, including Huh7 and HepG2, which are derived from liver cells, as well as HEK293 and HeLaS3 cells, which are non-liver cell lines. We monitored the expression of GLuc in the supernatant of these cell cultures at different time points post-transfection. Furthermore, we injected the two plasmids with different doses into BALB/c mice by the means of hydrodynamic delivery and monitored the GLuc expression in vivo with 2.5 microl tail tip blood since 2 h post-injection. The cell assay results suggested that the expression of GLuc driven by CMV promoter was significantly higher than that of GLuc driven by TTR promoter. And, the luciferase activity of GLuc driven by CMV promoter was 50-300 times higher than that of GLuc driven by TTR promoter in HEK293 and HeLaS3 cell lines, but less than 10 times higher than that of GLuc driven by TTR promoter in the HepG2 and Huh7 cell lines, indicating the relative liver-specificity of TTR promoter. In the animal assay, the higher luciferase activity was determined in CMV promoter group than in TTR promoter group at different doses of the two plasmids. But the expression patterns for the two promoters differed obviously. The expression of GLuc driven by CMV promoter reached the maximum 10 hours post-injection and declined rapidly; while the expression of GLuc driven by TTR promoter reached the maximum 48 hours after delivery, and declined very slowly. These results implied that PTTR could keep expression of driven gene in a long time although its expression intensity is lower than PCMV's. Thus, it is more suitable for maintaining longer expression of target genes in liver.
Animals ; Cell Line ; Cytomegalovirus ; genetics ; metabolism ; Gene Expression ; Gene Transfer Techniques ; Genes, Reporter ; Humans ; Luciferases ; genetics ; metabolism ; Male ; Mice ; Mice, Inbred BALB C ; Prealbumin ; genetics ; metabolism ; Promoter Regions, Genetic

OBJECTIVETo evaluate the effects of selective hepatic vascular exclusion (SHVE) on prevention of serious hemorrhage and air embolism during hepatectomy and on the liver function after operation.

METHODSFrom January 2004 to March 2007, 29 huge hepatic tumors were resected in our department. Both SHVE and Pringle maneuver were used to control the blood loss during hepatectomy. They were divided into two groups: SHVE group (15 cases) and Pringle group (14 cases). Data regarding the intraoperative and postoperative courses of the patients were analyzed.

RESULTSThere was no significant difference between the two groups regarding the age, sex, tumor size, cirrhosis, HbsAg positive rate and operating time (P > 0.05). Intraoperative blood loss was reduced significantly in the SHVE group (P < 0.05). The serum prealbumin levels on the postoperative day 1, 3 and 7 in SHVE group were significantly higher than those in the Pringle group (P < 0.05). The serum ALT value in SHVE group was significantly lower than that in the Pringle group on postoperative day 1, 3 and 7. The mean drainage volume in SHVE group was significantly less than that in the Pringle group on postoperative day 1 and 2. Liver failure occurred in two cases of the Pringle group, while no one in the SHVE group. Rupture of hepatic vein with massive blood loss occurred in 3 cases and air embolism in one case of the Pringle group, but did not occur in any case of the SHVE group.

CONCLUSIONWhen the selective exclusion of hepatic outflow and inflow is applied in hepatectomy, the resection rate of huge hepatic tumors and operative tolerance of hepatectomy are improved. It is a safe and rational operation type, and provides an optimal choice for hepatectomy.

Adolescent ; Adult ; Aged ; Alanine Transaminase ; blood ; Bile Duct Neoplasms ; blood ; blood supply ; surgery ; Bile Ducts, Intrahepatic ; Blood Loss, Surgical ; Carcinoma, Hepatocellular ; blood ; blood supply ; surgery ; Cholangiocarcinoma ; blood ; blood supply ; surgery ; Female ; Hepatectomy ; methods ; Hepatic Veins ; surgery ; Humans ; Intraoperative Care ; Liver ; blood supply ; surgery ; Liver Neoplasms ; blood ; blood supply ; surgery ; Male ; Middle Aged ; Prealbumin ; metabolism ; Young Adult

OBJECTIVETo investigate the effects of glutamine enriched enteral feeding on immunoregulation in burn patients.

METHODSTwenty burn patients were randomly divided into enteral nutrition (EN) group and enteral immune nutrition (EIN) group, with 12 patients in each group. Patients in EN group received a standard enteral formula, while those in EIN group received an enteral formula enriched with glutamine after hospital admission. Nutritional support was continued for 10 days. Blood samples were obtained to determine plasma level of total protein (TP), albumin (ALB), prealbumin (PAB) and transferrin (TF) at 1, 4, 7, 10 post-burn days (PBD). At the same time the concentration of immunoglobulin (IgA, IgG and IgM) were determined, the percentage of CD3+, CD4+, CD8+ subpopulations of T lymphocytes, and the ratio of CD4+/CD8+ were determined by FCM.

RESULTS(1) There were no obvious difference of the plasma level of TP, ALB, TF, CD3+, IgM between the two groups at each time-point (P > 0.05). (2) The plasma PAB contents in EIN group were significant higher than that in EN group on 4 PBD [(90 +/- 14 vs 60 +/- 15) mg/L, P < 0.05], 7 PBD [(92 +/- 16 vs 64 +/- 13) mg/L, P < 0.05] and 10 PBD [(106 +/- 21 vs 72 +/-16) mg/L, P < 0.05]. (3) The percentage of CD4+ subpopulation and ratio of CD4+/CD8+ in EIN group were obviously higher than those in EN group on 7 PBD [CD4+ (55 +/- 5 vs 45 +/- 5)%, CD4+/CD8+ (1.92 +/- 0.31 vs 1.53 +/- 0.27)%, P < 0.05] and 10 PBD [CD4+ (56 +/- 5 vs 49 +/- 5)%, CD4+/CD8+ (2.36 +/- 0.36 vs 1.72 +/- 0.42), P < 0.05]. (4) The concentration of IgA and IgG in EIN group were markedly higher than that in EN group on 7 PBD [IgA (2.8 +/- 0.6 vs 2.2 +/- 0.5) g/L, IgG (12.1 +/- 1.3 vs 9.8 +/- 1.2) g/L, P < 0.05] and 10 PBD [IgA (3.1 +/- 0.6 vs 2.5 +/- 0.5) g/L, IgG (14.2 +/- 1.3 vs 10.4 +/- 1.3) g/L, P < 0.05].

CONCLUSIONThese findings suggest that glutamine enriched enteral feeding can improve nutritional status by promoting the synthesis of IgA, IgG, and increasing the PAB concentration, and corrected immunologic dysfunction in burn patients.

Adolescent ; Adult ; Burns ; blood ; immunology ; therapy ; Enteral Nutrition ; Female ; Glutamine ; therapeutic use ; Humans ; Immunoglobulin A ; biosynthesis ; Immunoglobulin G ; biosynthesis ; Male ; Middle Aged ; Prealbumin ; metabolism ; T-Lymphocyte Subsets ; immunology ; Young Adult