OBJECTIVETo investigate the clinicopathological manifestations of early renal amyloidosis (AL) and its diagnostic criteria.

METHODSFifteen cases with early renal amyloidosis admitted from 1994 to 2001 were collected from the hospital, and their clinical and pathological features were reviewed. Of them, the initial diagnoses were not made by depending findings from the light microscopy (LM) and immunofluorescense (IF), but confirmed by electron microscopy (EM) afterwards. Immuno-electron microscopy (IEM) were applied for amyloidosis typing.

RESULTSMost patients of early renal AL were in the middle to old age. Nephrotic syndrome was the most prominent symptoms and signs accompanying with rare microscopic hematuria and hypertension. Most of them had a normal renal function. Pathological examinations of renal biopsies using LM and IF showed mild mesangial proliferation and mild thickening of glomerular basement membrane (GBM). Immunoglobulins and complements were negative or only scanty in certain cases, but in all cases there was a light chain protein deposition homogeously. There were 4 cases of minimal change glomerulopathy, 5 cases of mild mesangial proliferative glomerulonephritis, 5 cases of stage I membranous nephropathy, and 1 case of cast nephropathy diagnosed with LM. The amyloid fibrils (diameter 8 - 10 nm) were randomly distributed in the mesangium, along GBM and at the arteriolar wall under EM. Additionally, Congo red staining was positive. IEM demonstrated that amyloid fibrils labeled with colloid gold was combined with a kind of light chain protein which was confirmed as the light chain type of AL.

CONCLUSIONSThe diagnosis of early renal AL was occasionally neglected by depending only findings of LM and LF. However, special amyloid fibrils can be detected using EM. EM observation is an indispensable technique for the diagnosis of early renal AL and the typing of AL may further be determined by using IEM.

Adult ; Aged ; Amyloidosis ; metabolism ; pathology ; Basement Membrane ; metabolism ; Female ; Humans ; Immunoglobulin Light Chains ; metabolism ; Kidney Diseases ; metabolism ; pathology ; Kidney Glomerulus ; metabolism ; pathology ; Male ; Microscopy, Immunoelectron ; Middle Aged

OBJECTIVETo investigate the pathologic features and diagnostic algorithm of light chain nephropathy (LCN).

METHODSSeven cases of LCN were studied by light microscopy, electron microscopy and immunolabeling of light chains (kappa, lambda) by immunofluorescence and immunoelectron microscopy.

RESULTSThe histopathology of 7 cases by light microscopy was variable, with 3 cases showing nodular glomerulosclerosis, 1 case showing mild to moderate mesangial proliferation, and 3 cases showing cast nephropathy with minimal glomerular change. Immunofluorescence study revealed positive staining of a single type of light chain in mesangium (nodular pattern) or along glomerular basement membrane (linear), along tubular basement membrane and around arteriolar walls in all the 7 cases. Ultrastructurally, electron-dense granular deposits were identified in mesangium, subendothelial aspect of glomerular basement membrane, outer aspect of tubular basement membrane and arteriolar walls. Immunogold labeling of light chains showed distinct labeling of a single type light chain in the granular electron-dense materials (5 cases being kappa-positive and 2 being lambda-positive).

CONCLUSIONSLCN typically shows nodular glomerulosclerosis. The ultrastructural change is characteristic and important for diagnosis. Immunolabeling of light chains by immunofluorescence and immunoelectron microscopy carries further diagnostic value, especially in cases with minimal light microscopic change.

Adult ; Aged ; Female ; Glomerulosclerosis, Focal Segmental ; immunology ; pathology ; Humans ; Immunoglobulin Light Chains ; immunology ; Immunoglobulin kappa-Chains ; immunology ; Immunoglobulin lambda-Chains ; immunology ; Kidney Diseases ; immunology ; pathology ; Kidney Glomerulus ; immunology ; pathology ; ultrastructure ; Male ; Microscopy, Fluorescence ; Microscopy, Immunoelectron ; Middle Aged

OBJECTIVE: To investigate the effects of ulinastatin (UTI) on cerebral inflammatory response during cardiopulmonary bypass (CPB). METHODS: Twenty-four NYHA II-III patients (13 males and 11 females) aged 23-45 years, undergoing elective cardiac valve replacement under hypothermic CPB were randomly divided into 2 groups: ulinastatin group (Group U, n=12) and control group (Group C, n=12). In group U, UTI (1.2 x 10(4) U/kg) was given intravenously after the induction of anesthesia, 0.6 x 10(4) U/kg UTI was added to the priming solution, and 0.6 x 10(4) U/kg UTI was given about 5 min before the aortic decamping. In Group C, normal saline was given instead of UTI. Internal jugular vein was cannulated and the catheter was advanced retrogradely till jugular bulb. Blood samples were taken simultaneously from artery and jugular bulb after induction of anesthesia (T1), 60 min (T2) and 6 h (T3) after discontinuation of CPB for determination of TNFalpha, IL-6, IL-8 and IL-10. The juguloarterial gradients of these cytokines (deltaTNFalpha, deltaIL-6, deltaIL-8, and deltaIL-10) were calculated. RESULTS: In Group C, arterial levels of TNFalpha, IL-6, IL-8, IL-10 at T2 and T3, deltaTNFalpha, deltaIL-8 and deltaIL-10 at T2, deltaTNFalpha, deltaIL-6 and deltaIL-10 at T3 significantly increased (P < 0.01). deltaIL-8 increased at T3 (P < 0.05). In Group U, arterial levels of IL-6, IL-8, IL-10 at T2, arterial levels of IL-6, IL-8,IL-L-10 and deltaTNFalpha, deltaIL-8 at T3 significantly increased (P < 0.01). Arterial levels of TNFalpha at T2 and T3, deltaTNFalpha, deltaIL-10 at T2, deltaIL-6 at T3 increased (P < 0.05). Arterial levels of TNFalpha, IL-6 and deltaTNFalpha, deltaIL-8 at T2, arterial levels of TNFalpha and deltaIL-6 at T3 in Group U were lower than those in Group C (P < 0.05). Arterial levels of IL-6 at T3, IL-8 at T2 and T3 in Group U were significantly lower than those in Group C (P < 0.01). Arterial levels of IL-10 and deltaIL-10 at T3 in Group U were higher than those in Group C (P < 0.05). CONCLUSION Systemic and cerebral activation of inflammatory response during CPB can be alleviated by ulinastatin.
Adult ; Cardiopulmonary Bypass ; adverse effects ; Encephalitis ; etiology ; metabolism ; prevention & control ; Female ; Glycoproteins ; therapeutic use ; Heart Valve Prosthesis Implantation ; Humans ; Interleukin-10 ; blood ; Interleukin-6 ; blood ; Interleukin-8 ; blood ; Male ; Middle Aged ; Trypsin Inhibitors ; therapeutic use ; Tumor Necrosis Factor-alpha ; metabolism

OBJECTIVETo examine the relationship between apolipoprotein E (Apo E) gene polymorphism and risk of coronary artery disease (CAD), analyzing association of polymorphism with classical risk factors.

METHODSA total of 124 patients (including 84 Han population and 40 Uygur population) with angiographically verified CAD or myocardial infarction were prospectively evaluated. Data referring to hypertension, diabetes, and tobacco consumption were recorded. The levels of total cholesterol (TC), high density lipoprotein (HDL) cholesterol, Apo A1 and B, and triglycerides (TG) were determined. DNA was obtained from 124 patients and 70 controls. In order to determine Apo E genotypes, DNA was PCR amplified and digested with HhaI. The genetic polymorphism of Apo E is due to three common alleles, epsilon (epsilon) 2, epsilon3, epsilon4, at a single autosomal gene locus. These alleles determine the six phenotypes E2/2, E3/3, E4/4, E4/2, E4/3, and E3/2.

RESULTSIn Uygur population, the frequency of the epsilon2, epsilon3, and epsilon4 was 0.155, 0.648, and 0.197 respectively. In Han population, the frequency of the epsilon2, epsilon3, and epsilon4 was 0.081, 0.772, and 0.146 respectively. In the patient group, the frequency of the epsilon2, epsilon3, and epsilon4 was 0.060, 0.758, and 0.182 respectively. In the control group, the frequency of the epsilon2, epsilon3, and epsilon4 was 0.193, 0.671, and 0.136 respectively. epsilon2 frequency of Uygur' patients and controls was 0.050 and 0.290 respectively. Serum low density lipoprotein (LDL) cholesterol, TC, and TG values tended to decrease from the Apo E-4 phenotypes to Apo E-2 phenotypes. When deletion polymorphism of epsilon2 was compared with the common risk factors for CAD, its risk ratio (RR) is 4.38.

CONCLUSIONSThese studies confirm and find that Apo E phenotype distribution in Uygur population differs significantly from that in Han population in Xinjiang. CAD patients have significantly lower epsilon2 allele and slightly higher epsilon3 or epsilon4 allele frequency than controls, especially in Uygur population. It shows protective effects of epsilon2 on CAD.

Adult ; Aged ; Alleles ; Angina Pectoris ; genetics ; Angina, Unstable ; genetics ; Apolipoproteins E ; genetics ; Asian Continental Ancestry Group ; China ; DNA ; genetics ; Ethnic Groups ; Female ; Gene Frequency ; Genetic Predisposition to Disease ; Humans ; Male ; Middle Aged ; Myocardial Infarction ; genetics ; Phenotype ; Polymorphism, Genetic

OBJECTIVETo study the characteristics of infrasound and its effects on the workers at power plants.

METHODSThe audible noise and infrasound in three thermoelectricity plants were measured and by using Symptom Checklist 90 (SCL-90), the subjective sensation, and the physiological indices were compared between exposed workers and controls.

RESULTSThe infrasound levels were different at different measure sites of the three thermoelectricity works ranging from 40 to 98 dB. There was still 40 approximately 80 dB infrasound even when the soot blower stopped running. Different apparatus produced different infrasound levels with the highest ranging from 62 to 115 dB. A single frequency (16 Hz) infrasound was produced in certain department during working hour with sound pressure levels of 110 to 120 dB, but the audible noise sound pressure level was less than 70 dB. There was no significant difference in the indices representing vision fatigue and neurobehaviour function between exposed workers and controls. Workers at certain department experienced evident subjective sensation of neurobehavioral dysfunction, and the scores of somatization, depression, hostility, phobic anxiety, and psychotism in the SCL-90 were significantly higher in the exposed group than in the control and the norm in China (P < 0.05).

CONCLUSIONInfrasound is ubiquitous in the working environment, but usually, the noise levels are less than 120 dB. In some special production department, there is persistent infrasound above 110 to 120 dB. No obvious health effects are found among those who are exposed to infrasound below 100 dB. However, the workers who are chronically exposed to infrasound above 110 to 120 dB present notable subjective sensation of autonomic neurobehavioral dysfunction, and their psychological health status is not as good as those in the control and those in the domestic normal pattern.

Adult ; China ; Humans ; Occupational Exposure ; adverse effects ; Power Plants ; Sound ; adverse effects ; Workplace

OBJECTIVETo detect the expression of thyroid transcription factor 1 (TTF-1) and study its application in the diagnosis of lung carcinomas.

METHODSOf 134 specimens from lung lobectomy, 105 were primary lung carcinomas including 76 non-small cell carcinomas (NSCLCs), 28 small cell lung cancers (SCLCs) and 1 complex carcinoma (SCLC and SCC), and 29 were metastatic carcinomas. Expression of TTF-1 was detected by immunohistochemistry. The expression level of TTF-1 was graded as, +:6% to 25% of tumor cells positive, ++:26% to 50%, +++:51% to 75%, and ++++:> 76%.

RESULTSThe positive nuclear immunoreactivity of TTF-1 was identified in 23 of 28 SCLCs (82.1%), but none in squamous cell cancer (SCC) (P < 0.001). The positive expression rate of TTF-1 in lung adenocarcinomas (ACs) was 73.8% (31/42). There was no correlation between TTF-1 expression and ACs differentiation or ACs subtypes (P > 0.05). All but one (thyroid follicular carcinoma) metastatic ACs were TTF-1-positive. Mesenchymal component and lymphoid or inflammatory cells were consistently TTF-1-negative.

CONCLUSIONA significant difference of TTF-1 expression may assist in distinguishing SCLC from SCC, lymphoma or inflammatory lesions. Owing to its restrictive expression in lung tissue, TTF-1 might be used to differentiate primary from metastatic adenocarcinoma of the lung.

Adenocarcinoma ; diagnosis ; metabolism ; Breast Neoplasms ; pathology ; Carcinoma, Non-Small-Cell Lung ; diagnosis ; metabolism ; Colorectal Neoplasms ; pathology ; Diagnosis, Differential ; Humans ; Lung Neoplasms ; diagnosis ; metabolism ; secondary ; Nuclear Proteins ; biosynthesis ; Thyroid Nuclear Factor 1 ; Transcription Factors ; biosynthesis

OBJECTIVETo discuss the clinicopathological features and prognostic factors of gastric lymphoma.

METHODS83 gastric lymphoma cases were analyzed retrospectively in accordance to the criteria of the new World Health Organization classification for neoplastic diseases of the hematopoietic and lymphoid tissues. The correlations between clinicopathological features, therapeutic measures and survival were discussed.

RESULTSThe age of patients ranged from 25 to 77, with a median of 52. The number of males were similar to that of females. There were no specific symptoms. The most common symptoms were stomach ache (60 cases, 72%) or discomfort. The duration of symptoms was often long and with a history of chronic gastric diseases (21 cases, 25%). 13 cases had multiple lesions in the gastrointestinal mucosa. 51 cases (61%) were accompanied by lymph node involvement. According to the new World Health Organization classification for neoplastic diseases of the hematopoietic and lymphoid tissues, 57 cases were extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT)-type (MALT lymphoma), 23 were diffuse large B cell lymphoma accompanying MALT lymphoma, 2 were diffuse large B cell lymphoma and 1 was follicular lymphoma. Of all the cases, 31 were stage I E, 38 stage II E, 8 stage III E and 6 stage IV by the Ann Arbor staging system (1972). The total 5-year and 10-year survival rates were 77.8% and 70.1% respectively, with the mean survival time of 146 months. The 5-year and 10-year survival rates of MALT lymphoma were 77.4% and 72.3%, the 5-year and 10-year survival rates of diffuse large B cell lymphoma accompanying MALT lymphoma were 81.8% and 68.2%, the 5-year survival rate of diffuse large B cell lymphoma was 50.0%.

CONCLUSIONSThere are no specific symptoms in gastric lymphoma patients. Extranodal marginal zone lymphoma of MALT-type is the main histopathological type of gastric lymphoma, often accompanied by multiple mucosa involvement and also often accompanied by a history of chronic gastric disease. The lesion is usually localized for a long time, with a very good prognosis. Survival rate has a significant correlation with lymph node involvement and clinical stage. No correlations were found between the survival rates with age, gender, B symptoms, invasive depth of the wall of stomach, the size and range of the tumors or different therapeutic measures.

Adult ; Aged ; Chemotherapy, Adjuvant ; Female ; Follow-Up Studies ; Gastrectomy ; methods ; Humans ; Lymphatic Metastasis ; Lymphoma ; pathology ; surgery ; therapy ; Lymphoma, B-Cell ; pathology ; surgery ; therapy ; Lymphoma, B-Cell, Marginal Zone ; pathology ; surgery ; therapy ; Lymphoma, Large B-Cell, Diffuse ; pathology ; surgery ; therapy ; Male ; Middle Aged ; Neoplasm Staging ; Radiotherapy, Adjuvant ; Retrospective Studies ; Stomach Neoplasms ; pathology ; surgery ; therapy ; Survival Rate

OBJECTIVETo study the pattern of lymph node spread in papillary thyroid carcinoma (PTC) with clinically negative node (cN0).

METHODSA total of 106 patients with cN0 PTC who underwent total or subtotal thyroidectomy plus unilateral or bilateral lateral neck dissection (LND, level II-V or level I-V) at West China Hospital of Sichuan University between April 2004 and August 2010 were analyzed retrospectively.

RESULTSThe lateral neck lymph node metastasis in cN0 PTC was significantly associated with sex (male, P = 0.007), tumor stage (T3/T4, P = 0.006), tumor size (> 1 cm, P = 0.014) and the number of positive central lymph nodes (≥ 2, P < 0.001), but not with age and multifocal tumor. Level III (47/116, 40.5%) was the most prevalent metastatic site, followed by level IV (41/116, 35.3%), level II (18/116, 15.5%) and level V (2/29, 6.9%). Of the cases with lymph node metastases in level III and IV, 89.8% (79/88) of primary thyroid tumors existed in the lower and middle sites of the thyroid lobes, while in the cases with lymph node metastases in level II, 77.8% (14/18) of primary thyroid tumors in the upper sites of the thyroid lobes, and 83.3% of cases with level II metastases were accompanied with level III metastases. Two cases with level V metastases were accompanied with metastases in levels II, III and IV.

CONCLUSIONSLND should be considered for cN0 PTC in male, with T3/T4 lesions and positive central lymph nodes ≥ 2, and the range of dissection should include level III and IV. Dissection of level II should be considered in cN0 PTC with primary tumor localized in the upper site of the thyroid lobe or with level III metastasis. Dissection of level V should be considered at present of metastases in level II, III, and IV. For cN0 PTC with tumor size < 1 cm, confined to the thyroid and without lymph node metastasis in the central compartment, LND is not recommended.

Adolescent ; Adult ; Aged ; Carcinoma ; pathology ; Carcinoma, Papillary ; Child ; Female ; Humans ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; pathology ; Male ; Middle Aged ; Neck Dissection ; Neoplasm Staging ; Retrospective Studies ; Thyroid Neoplasms ; pathology ; Young Adult

OBJECTIVETo investigate the influence of thymidylate synthase (TS) gene polymorphisms on high-dose methotrexate (HD-MTX)-related toxicities in childhood acute lymphoblastic leukemia (ALL).

METHODSA total of 73 children who were diagnosed with ALL between March 2011 and March 2013 were included into this study. Genomic DNAs were extracted from their peripheral blood. And then the genotypes of TS 5'-UTR were determined by direct DNA sequencing after PCR. The toxicity response of 73 patients receiving HD-MTX chemotherapy were observed and recorded, and plasma MTX concentrations at 42-48 hours after chemotherapy were measured.

RESULTSThe main HD-MTX-related toxicities of 73 patients receiving HD-MTX chemotherapy were neutropenia, decreased hemoglobin level, thrombocytopenia, liver toxicity, mucosal damage, and gastrointestinal reactions. There were no significant differences in the incidence rate of HD-MTX-related toxicities between children with different TS 5'-UTR genotypes after chemotherapy (P>0.05). TS 5'-UTR genotype was not significantly correlated with plasma MTX concentrations at 42-48 hours after chemotherapy (P>0.05).

CONCLUSIONSTS gene polymorphisms have no influence on the incidence of HD-MTX-related toxicities in childhood ALL.

Antimetabolites, Antineoplastic ; adverse effects ; Child ; Child, Preschool ; Female ; Genotype ; Humans ; Infant ; Male ; Methotrexate ; adverse effects ; Polymorphism, Genetic ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; genetics ; Thymidylate Synthase ; genetics

Objective: To study the efficacy, safety and long-term outcomes of integrated strategy of bortezomib-based induction regimens followed by autologous hematopoietic stem cell (ASCT) and maintenance therapy in Chinese multiple myeloma (MM) patients. Methods: 200 MM patients receiving integrated strategy of bortezomib--based induction regimens followed by ASCT and maintenance therapy were retrospectively and prospectively analyzed from December 1. 2006 to April 30. 2018. Results: The complete remission rates (CR) and better than very good partial remission rates (VGPR) after induction therapy, transplantation and maintenance therapy were respectively 31% and 75.5%, 51.8% and 87.7%,73.6% and 93.4%. There was no difference between 4 cycles and more than 5 cycles induction chemotherapy. The negative rate of MRD detection by flow cytometry was 17.6% and 38.2% respectively after induction and 3 months after transplantation. The negative rate of MRD gradually increased during the maintenance therapy. The success rate of high dose CTX combined with G-CSF mobilization was 95.5% and transplantation related mortality (TRM) was zero. The median time to progress (TTP) was 75.3 months and the median overall survival (OS) was 99.5 months. TTP of patients obtaining CR and negative MRD after induction were longer that those of no CR and positive MRD. TTP and OS of patients receiving triple-drug induction and ASCT in early stage were longer than those of double-drug induction and ASCT in late stage. LDH≥240 U/L, high risk cytogenetics, ISS II+III stage and HBsAg positive were prognostic factors at diagnosis. However, only MRD and high risk cytogenetics were independent prognostic factors after transplantation and maintenance therapy. The clinical characteristics of patients of TTP ≥6 years were listed below: light-chain type M protein, ISS I stage, normal level of hemoglobin and platelet, normal LDH, HBsAg negative, chromosome 17p-negative, good response and sustained good response. Conclusions: Integrated strategy of bortezomib-based induction regimens followed by ASCT and maintenance therapy can significantly improve the short-term and long-term efficacy. The prognostic factors of TTP in different disease stages were different. Response to treatment, especially MRD, played a more important role in prognostic factors.
Antineoplastic Combined Chemotherapy Protocols ; Bortezomib/therapeutic use* ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation ; Humans ; Induction Chemotherapy ; Multiple Myeloma/therapy* ; Retrospective Studies ; Stem Cell Transplantation ; Transplantation, Autologous ; Treatment Outcome