PURPOSE: The objective was to determine the characteristics and prognostic factors of 86 Chinese patients with trisomy 8 aberrations and compare the prognostic value of International Prognostic System (IPSS) and Revised IPSS (IPSS-R) in this cohort. MATERIALS AND METHODS: A total of 86 cases diagnosed with primary myelodysplastic syndromes (MDS) with isolated tr8 or with tr8 and other additional cytogenetic aberrations diagnosed and treated at the Union Hospital, Tongji Medical College of Huazhong University of Science and Technology between July 2002 and March 2013 were reviewed. RESULTS: The median survival of the entire group was 23.0 months, and acute myeloid leukemia (AML) developed in 43% (37/86) patients within the follow up time. The univariate analysis revealed that overall survival (OS) was correlated with age, thrombocytopenia, absolute neutrophil count, marrow blasts, cytogenetic status and red blood cell transfusion at diagnosis, and the multivariate analysis revealed that age, marrow blasts, cytogenetic status and transfusion dependence were independent parameters for the OS. The cytogenetic complexity and marrow blasts had the strongest impact on the AML transformation by multivariate analysis. Comparing the two prognostic systems, both two systems could successfully discriminate risk groups for survival. IPSS-R was more refined than IPSS for predicting OS, but had no advantage in predicting the risk of AML development. CONCLUSION: This study confirmed the influence of clinical factors on the prognosis of 86 Chinese MDS patients with trisomy 8. In addition, IPSS-R can further refine prognostic discrimination in the IPSS risk categories.
Adolescent ; Adult ; Aged ; Asian Continental Ancestry Group/*genetics ; Chromosome Aberrations ; Chromosomes, Human, Pair 8 ; Disease Progression ; Female ; Humans ; Leukemia, Myeloid, Acute/*genetics ; Male ; Middle Aged ; Multivariate Analysis ; Myelodysplastic Syndromes/*ethnology/*genetics ; Prognosis ; Retrospective Studies ; *Trisomy

OBJECTIVETo compare clinical features and therapeutic response of patients with aplastic anemia with and without cytogenetic abnormalities.

METHODSClinical features of 133 patients with successful chromosomal analysis were retrospectively studied, and therapeutic response between patients with and without cytogenetic abnormalities was compared.

RESULTSCytogenetic abnormalities were found in 9 patients, which included trisomy 8 (4 cases), monosomy 7 (2 cases) and Xq- (1 case), 1q- (1 case) and 7q- (1 case). No significant difference was detected between patients with or without cytogenetic abnormalities in terms of age (50 vs. 58, P=0.337), sex ratio (male 55.56% vs. 62.10%, female 44.44% vs. 37.90%, P=0.762), or episode of acute aplastic anemia (44.44% vs. 37.10%, P=0.728). Patients with cytogenetic abnormalities had a tendency towards poorer rate of therapeutic response, which was however not significantly different from those without (55.56% vs. 79.03%, P=0.116). All of the 4 patients with +8 responded to treatment, whilst none of those with -7 or 7q- did.

CONCLUSIONNo significant difference was found between aplastic anemia patients with or without cytogenetic abnormalities in terms of clinical features and therapeutic response. Patients with trisomy 8 seem to have a favorable response towards treatment.

Adolescent ; Adult ; Aged ; Anemia, Aplastic ; genetics ; therapy ; Child ; Chromosome Aberrations ; Female ; Humans ; Karyotyping ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVETo study the effect of endothelin-1 (ET-1) and its antagonists on the expression of endothelin and its receptors mRNA in HSC-T6 cells.

METHODSCultured HSC-T6 cells were randomly divided into 7 groups: Sham control group, ET-1 group (10 nmol/L ET-1), BQ-123 group [1 micromol/L BQ-123, a selective endothelin receptor A (ETRA) antagonist], BQ-788 group [1 micromol/L BQ-788, a selective endothelin receptor B (ETRB) antagonist], ET-1 + BQ123 group (10 nmol/L ET-1 + 1 micromol/L BQ-123), ET-1 + BQ-788 group (10 nmol/L ET-1 + 1 micromol/L BQ-788) and ET-1 + BQ-788 group (10 nmol/L ET-1 + 1 micromol/L BQ-123 + 1 micromol/L BQ-788). The expression of endothelin receptor mRNA of HSC-T6 cells was determined by reverse-transcription polymerase chain reaction (RT-PCR).

RESULTSThe expression of ETRA mRNA in ET-1 + BQ123 + BQ788 and ET-1 + BQ788 group was significantly lower than ET-1 group (0.329 +/- 0.044 and 0.292 +/- 0.023 vs. 0.440 +/- 0.030 P < 0.05). Compared with ET-1 group, the expression of ETRB mRNA in ET-1 + BQ788 group was down regulated obviously (0.499 +/- 0.136 vs. 0.153 +/- 0.071, P < 0.05). There was no significant difference in ET-1 + BQ123 group and ET-1 + BQ123 + BQ788 group when compared with ET-1 group (0.499 +/- 0.136 vs. 0.496 +/- 0.103 and 0.299 +/- 0.129, P > 0.05).

CONCLUSIONSET-1 has no obvious effect on the expression of ETRA mRNA in HSC-T6. ET-1 may up-regulate the expression of ETRB mRNA. Act on ETRA receptor, ET-1 can inhibit the expression of ETRB mRNA.

Animals ; Cells, Cultured ; Endothelin-1 ; pharmacology ; Gene Expression Regulation ; drug effects ; Hepatocytes ; drug effects ; metabolism ; Oligopeptides ; pharmacology ; Peptides, Cyclic ; pharmacology ; Piperidines ; pharmacology ; RNA, Messenger ; genetics ; Rats ; Rats, Sprague-Dawley ; Receptor, Endothelin A ; biosynthesis ; drug effects ; genetics ; Receptor, Endothelin B ; biosynthesis ; drug effects ; genetics

Objective To study the L-carnosine induced apoptosis of human hepatocellular carcinoma HepG2 cells and its mechanism.Methods Human hepatocellular carcinoma HepG2 cells were cultured in vitro,then the cells in logarithmic phase were divided into 5 mM,20 mM,50 mM L-carnosine group and control group,treated with 5 mM,20 mM,50 mM L-carnosine and saline respectively.The proliferation of HepG2 cells was measured by CCK-8 assay after 24 hours and 48 hours treatment.The cell apoptosis and cell cycle were detected by flow cytometry after 48 hours treatment.The protein expression of Caspase-8,PI3K and Akt were detected by Western blot in each group after 48 hours treatment.Results CCK-8 and flow cytometry assay showed that both 20 mM and 50 mM L-carnosine treated group significantly inhibited the proliferation of HepG2 cells after 24 hours and 48 hours,and the inhibition were in a time and dose-dependent manner.Western blot revealed that the expressions of PI3K and Akt were down-regulated with the increase of L-carnosine concentration,while the expression of Caspase-8 was increased.Conclusion L-carnosine exhibits an inhibitory effect of the proliferation and promote apoptosis of human liver cancer cell line HepG2.The mechanism may be associated with the inhibition of the PI3K/Akt signaling pathway and activation of Caspase-8 signaling pathway.

OBJECTIVE: To study the clinical features of severe type 7 adenovirus pneumonia in children. METHODS: A retrospective analysis was performed for the clinical data of children who were diagnosed with severe type 7 adenovirus pneumonia from February to June, 2019. RESULTS: Among the 45 children, the male/female ratio was 3:2 and the median age was 14 months. All children had repeated fever, cough, and pulmonary moist rales, and the mean duration of fever was 14±4 days. The median time from fever to dyspnea was 8 days, and the time from fever to mechanical ventilation was 11.6±2.5 d. There was no significant increase in white blood cell count, with neutrophils as the main type. There were slight reductions in hemoglobin and albumin, while platelet and fibrinogen remained normal. There were increases in aspartate aminotransferase, lactate dehydrogenase, procalcitonin, and C-reaction protein. The detection rate of mixed pathogens was 84%. Effusion in both lungs was the major change on chest imaging (64%). Bronchoscopic manifestations were endobronchitis, tracheomalacia, and plastic bronchitis. The incidence rate of respiratory complications was 100%, and extrapulmonary complications mainly involved the circulatory system (47%), digestive system (36%), and nervous system (31%). Among the 45 children, 16 were administered with 400 mg/kg intravenous immunoglobulin (IVIG) daily for 5 days, with a mean duration of fever of 16±5 days, and 29 were administered with 1 g/kg IVIG daily for 2 days, with a mean duration of fever of 13±4 days; there was a significant difference in the mean duration of fever between the two groups (P=0.046). The overall mortality rate was 11%. CONCLUSIONS Severe type 7 adenovirus pneumonia in children has severe conditions, with a high incidence rate of complications and a high mortality rate, so it should be diagnosed and treated as early as possible.
Adenoviridae ; Bronchitis ; Female ; Fever ; Humans ; Infant ; Male ; Pneumonia, Viral ; Retrospective Studies

OBJECTIVETo explore the clinical spectrum, geographic location of human H7N9 avian influenza as well as the characteristics of population at high risk of infection, in order to develop strategies for the prevention and control of the infection. Clinical and epidemiological characteristics on the 6 confirmed human cases in Zhejiang were also analyzed.

METHODSReal-time fluorescent quantitative PCR was used to test the nucleic acid of human H7N9 avian influenza infection, from pharyngeal swabs of the patients and their close contacts. Face to face interview and descriptive method were used to collect related clinical and epidemiological data. Statistical analysis was performed by SPSS 17.0.

RESULTSSix confirmed cases were distributed in Hangzhou and Huzhou cities. The 6 confirmed human cases, including 5 males and 1 female were all confirmed with novel influenza A (H7N9) virus infection, with an average age as 60.83 years (with median as 64.50 years). Cough was the most common initial symptom to be noticed. The clinical manifestations would include fever, dizziness, pain of muscles, coughing, expectoration and short of breath. All the X-ray chest films showed severe pneumonia, and 5 of them having had other chronic diseases. None of the cases admitted to have had a history of exposure to ill/death avians. However, all of the cases had been frequently exposed to the agricultural-byproduct-trading-markets where the positive rate of novel influenza A (H7N9) virus in environmental specimens was up to 43.21%. 32 of the 375 close contacts (8.53%) to the 6 cases appeared abnormal symptoms, but no positive result related to novel influenza A (H7N9) virus nucleic acid was detected from their throat swabs.

CONCLUSIONAcute infection on the respiratory system seemed the main clinical manifestation. Elderly men, especially those with chronic diseases were under high risk of human infection with H7N9 avian influenza. The source of infection might be associated with the exposure to poultry. There was still lack of evidence to confirm the route of person to person transmission on H7N9 avian influenza.

Adult ; Aged ; China ; epidemiology ; Female ; Humans ; Influenza A Virus, H7N9 Subtype ; Influenza, Human ; epidemiology ; virology ; Male ; Middle Aged

Heat stroke(HS)is a serious life-threatening disease caused by heat injury and characterized by a core body temperature>40℃with central nervous system dysfunction and multi-organ failure.The main pathophysiological manifestations of HS are the thermal acute phase response and thermoregulatory imbalance.Proteins are particularly sensitive to heat,and the thermal environment can cause massive protein denaturation,resulting in the deposition of unfolded and misfolded proteins in the cytoplasm,causing cellular dysfunction and even death.The unfolded protein response(UPR),mainly divided into the endoplasmic reticulum UPR and the mitochondrial UPR,is an important physiological process that helps proteins to fold correctly or degrade irretrievably denatured proteins.This paper summarizes the regulatory mechanisms of UPR,the relationship between UPR and severe diseases,as well as the relationship between HS and UPR to provide new ideas for the treatment of HS.

OBJECTIVE: To investigate the role of tacrolimus-binding protein 38 (FKBP38) in follicle development and the mechanism by which Fkbp38 gene deletion causes premature ovarian insufficiency (POI). METHODS: The Cre-loxp system was used to construct oocyte-specific Fkbp38 knockout transgenic mice. The genotype of the transgenic mice was identified using PCR, and the expression of FKBP38 in the oocytes was verified. The numbers of primordial follicles, primary follicles, secondary follicles and antral follicles in Fkbp38 knockout mice and non-transgenic littermate control mice were counted with HE staining under a microscope for analyzing the effect of Fkbp38 deletion on follicular development. The fertility and serum sex hormone levels of the mice were determined by reproduction experiments and ELISA to assess ovarian function. Ovarian granulosa cell apoptosis of the mice was assessed using TUNEL assay. The activity of the downstream target protein of phosphorylated ribosomal S6 (PS6) of mTOR signaling pathway was detected, and the expressions of BCL-2 and BAX proteins were determined using immunofluorescence assay for assessing oocyte development in the mice. RESULTS: The oocyte-specific Fkbp38 knockout transgenic mouse model was successfully constructed, which showed decreased fertility, disordered sex hormone levels, and significantly reduced primordial follicles, primary follicles and secondary follicles in the ovary (P < 0.05), demonstrating POI-like changes. Compared with the control mice, oocyte-specific Fkbp38 knockout caused activation of the mTOR signaling pathway, significantly increased apoptosis of the granulosa cells, and obviously increased the BAX/BCL- 2 ratio by increasing BAX expression and reducing BCL-2 expression in the oocytes (P < 0.05). CONCLUSION FKBP38 plays an important role in follicle development, and Fkbp38 gene deletion in mice causes POI possibly by activating the mTOR signaling pathway and inducing granulosa cell apoptosis.
Female ; Humans ; Mice ; Animals ; Primary Ovarian Insufficiency/genetics* ; Apoptosis ; Signal Transduction ; Proto-Oncogene Proteins c-bcl-2 ; Granulosa Cells ; Mice, Transgenic ; Mice, Knockout ; TOR Serine-Threonine Kinases

Objective To evaluate the effect of Oncomelania hupensis snail control of cutting the beach group in the south of Shaobo Lake. Methods The general situation of the project of cutting the beach was surveyed, and the snail distribution was surveyed and the results were compared before and after cutting the beach in the beach group. Results The area of cutting beach was 928.33 hm², the cubic meter of earthwork was 1 717.00 m³, the area of dumping ground was 425.76 hm², the beach surface elevation was 3.2 m after cutting the beach, and the beach surface was fallen to 1.0 m under the ordinary water level. The area with snails was 44.69 hm² before cutting the beach in 2011 but the area with snails was 1.78 hm² after cutting the beach in 2018. The area with remaining snails was declined by 96.02% in 2018 as compared with that in 2011, and surviving snails were distributed on the uncut beach face. Conclusion In Shaobo Lake, the O. hupensis snail breeding environment is eliminated by raising or lowering the beach, so it is an effective measure of snail control in lake regions.

Madelung deformity is a rare deformity of forearm and wrist caused by growth disorders of distal radius ulnar and palmar epiphyseal. Current studies showed that its incidence mainly associated with trauma, epiphyseal developmental abnormalities, nutritional disorders and genetic deletion or mutation. The early clinical presentation is not typical, in middle and late time, wrist deformity and weak can appear. Plain film considered as the main means of diagnosis is often lack of early diagnosis significance. Although wrist joint magnetic resonance imaging showing early soft tissue and skeletal abnormalities were used for the early diagnosis of the disease, current domestic study in magnetic resonance imaging of this deformity is less. According to the size of the distal ulnar inclination angle and palm angle, this deformity can be divided into different types. The patients with severe deformity and symptoms usually need surgical intervention including ulna revision and osteotomy of the distal radius at present. Although the two operation can achieve good clinical results, the surgical trauma, infection and postoperative risk of joint activities are more.