OBJECTIVE: To study the distribution characteristics of thalassemia genotype in Han Population in Sanya of Hainan Province. METHODS: Gap PCR and reverse dot hybridization were used to detect and analyze the thalassemia gene in 572 suspected thalassemia carriers of Han Population in Sanya. RESULTS: Among the 572 Han Population in Sanya, 271 cases of thalassemia gene abnormality were detected, among which 161 cases were founded to be carriers of α-thalassemia gene. A total of 9 genotypes were detected, in the following order of the detection rate was －－^SEA/αα，－α^3.7/αα，－α^4.2/αα，－－^SEA/－α^3.7，－－^SEA/－α^4.2，－α^4.2/－α^4.2，－α^3.7/－α^4.2，－α^3.7/－α^3.7，－－^SEA/－－^SEA. Among them, the deletion type (－－^SEA/αα) in southeast Asia was the most common, accounting for 66 cases. 99 cases of β-thalassemia were detected, there were 7 genotypes, all of which were heterozygous. The order of the detection rate was CD41-42/βN, IVS-II-654/βN, CD17/βN, CD71-72/βN, -28/βN, －29/βN, CD27-28/βN. Among them, CD41-42/βN was the most common, accounting for 51 cases. In addition, 11 cases of combined α and β thalassemia were detected. Five kinds of genotypes were checked out, the order of detection rate was －α^3.7/αα composite CD41-42/βN, －－^SEA/αα composite IVS-II-654/βN, －α^4.2/－α^4.2 composite CD41-42/βN, －α^4.2/αα composite －29/βN , －－^SEA/ －α^4.2 composite CD41-42/βN. CONCLUSION Han Population in Sanya of Hainan Province is a high-risk population of thalassemia, the genotype characteristics are different from other areas with high incidence of thalassemia in China. The main type of α-thalassemia is the deficiency mutation of southeast Asia, while CD41-42 heterozygous mutation is the main type of β-thalassemia.
China/epidemiology* ; Genotype ; Heterozygote ; Humans ; Mutation ; alpha-Thalassemia/genetics* ; beta-Thalassemia

OBJECTIVE: To analyze the characteristics, prognosis and risk factors of bloodstream infection in patients with hematological malignancies in the tropics, so as to provide evidence for the prevention and treatment of bloodstream infection. METHODS: The clinical features, blood culture results and prognosis of patients with bloodstream infection in patients with hematological malignancies admitted to Hainan Hospital of PLA General Hospital were retrospectively studied. RESULTS: The most common primary infection site of the 81 patients with hematological malignancies was lung (46.91%), followed by PICC (11.11%). The detection rate of Gram-positive bacteria and Gram-negative bacteria in the blood culture was 60.98% and 30.02%, respectively. Coagulase-negative staphylococci was the most common Gram-positive bacteria resulting in bloodstream infection in our study. Of the Gram-negatives, Klebsiella pneumoniae (34.38%) was predominant, followed by Escherichia coli (18.75%) and Pseudomonas aeruginosa (18.75%). Gram-positive bacteria was highly sensitive (100%) to vancomycin, linezolid and tigecycline. Study showed that Gram-negative bacteria had low sensitive to quinolones, in particular, the resistance rate of Escherichia coli to quinolones was as high as 83.33%. In terms of overall survival (OS), the 30-days OS of patients with Gram-negative and Gram-positive septicemia was 77.42% and 92.00%, respectively. There was no statistically significant difference between the two groups. Multivariate analysis revealed that septic shock (P=0.001, RR=269.27) was an independent risk factor for 30-day mortality, and remission status (P=0.027, RR=0.114) was an independent predictor of a favourable outcome of bloodstream infection in patients with hematological malignancies. CONCLUSION Gram-positive bacteria are the main pathogens causing bloodstream infections in patients with hematological malignancies in the tropics. Improving the care of PICC is an important measure to reduce the incidence of bloodstream infection in patients with hematological malignancies in the tropics. A correct treatment relieving disease and effective prevention and treatment of septic shock can reduce mortality of patients with bloodstream infection in patients with hematological malignancies in the tropics.
Anti-Bacterial Agents/therapeutic use* ; Bacteremia/drug therapy* ; Drug Resistance, Bacterial ; Gram-Negative Bacteria ; Hematologic Neoplasms/drug therapy* ; Humans ; Microbial Sensitivity Tests ; Prognosis ; Retrospective Studies ; Sepsis

OBJECTIVE: To analyze the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for treatment of acute leukemia in the tropical area. METHODS: Twelve acute leukemia patients who were underwent allo-HSCT from April 2013 to November 2018 in Hainan Hospital of Chinese PLA General Hospital were selected, including 5 cases of acute lymphoblastic leukemia (ALL) and 7 case of acute myeloid leukemia (AML). Three cases received HLA matched sibling hematopoietic stem cell transplantation, 8 cases received haploidentical hematopoietic stem cell transplantation, 1 cases received partially mismatched unrelated hematopoietic stem cell transplantation. Pretreatment regimen: 9 cases received modified BU/CY+ATG pretreatment regimen, 3 cases received BU/CY pretreatment regimen. Graft-versus-host disease (GVHD) prevention regimen: all patients received cyclosporine A, mycophenolate mofetil combined with short-term methotrexate regimen. The clinical efficacy of allo-HSCT in treatment of acute leukemia in the tropical area was analyzed by detecting hematopoietic reconstitution, GVHD, infection, relapse and survival after transplantation. RESULTS: All the 12 patients achieved granulocyte reconstruction and megakaryocyte reconstruction. The median time of granulocyte reconstruction was 11.5 (6-14) days, and the median time of megakaryocytic reconstruction was 12.5 (10-22) days. Within 100 days after transplantation, the acute GVHD occurved in 8 cases, including 6 cases of Ⅱ-Ⅳ degree acute GVHD and 2 cases of Ⅲ-Ⅳ degree acute GVHD, 11 cases survived more than 100 days after transplantation, and the chronic GVHD occurred in 1 case, which was mildly limited. Pulmonary infection occurred in 7 cases, cytomegaloviremia occurred in 6 cases, EB viremia occurred in 6 cases, and hemorrhagic cystitis occurred in 5 cases. 2 cases relapsed and eventually died, and the remaining 10 patients survived without disease until the date of follow-up. The median follow-up time was 4 (1-68) months, 83.3% (10/12) survived without disease, and 16.7% (2/12) relapsed. CONCLUSION Allo-HSCT is an effective method for the treatment of acute leukemia in adults. Leukemia patients should be transplanted as soon as possible after remission. The incidence of pulmonary fungal infection in transplanted patients in tropics is high, therefore the prevention and treatment of fungal infection should be strengthened.
Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Myeloid, Acute ; Transplantation Conditioning ; Transplantation, Homologous

OBJECTIVE: To investigate the incidence, clinical features of U2AF1 gene mutation in patients with acute myeloid leukemia(AML) and its effect of prognosis. METHODS: A total of 161 patients with AML were enrolled. The second-generation sequencing method was used to detect U2AF1 gene mutation, and the relationship between U2AF1 mutation and clinical features, prognosis was analyzed. RESULTS: The mutation rate of U2AF1 gene in 161 AML patients was 3.73%. The counts of peripheral blood leukocytes and platelets in the U2AF1 gene mutation group were lower than those in the wild type group. The complete response rate of U2AF1 gene mutation group was 66.67%, while that in wild type group was 55.48%, which shows no significant difference between the two groups (P=0.70). The median EFS of wild type group and the mutant group was not reached and reached to 133 days, respectively (P=0.03), while the medium OS in two groups was not reached and reached to 210 days (P=0.01). CONCLUSION The AML patients with U2AF1 mutation positive have a poor prognosis as compared with the wild type group, which may be a poor prognostic factor for acute myeloid leukemia.

BACKGROUNDAnti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a treatable autoimmune neurologic syndrome that occurs with or without tumor association. However, some severe cases are refractory to systemic immunotherapy. This pilot study aimed to evaluate the utility and safety of intrathecal methotrexate injection for severe patients with anti-NMDAR encephalitis who did not respond to first-line immunotherapy.

METHODSIntrathecal injections with methotrexate and dexamethasone were performed weekly in four legible patients within consecutive 4 weeks. Cerebrospinal fluid (CSF) was collected at baseline and each time of intrathecal injection for identification of anti-NMDAR antibody titers.

RESULTSSignificant clinical improvement was observed in three patients associated with a stepwise decrease of CSF anti-NMDAR antibody titers (maximum: 1/320 to minimum: 1/10). After 2 months of follow-up, they were able to follow simple commands and had appropriate interactions with people (modified Rankin scale [mRS] of 0-2). At 12 months of follow-up, they all had returned to most activities of daily life (mRS of 0), and no relapses were reported. One patient showed no clinical improvement and died of neurologic complications.

CONCLUSIONSIntrathecal treatment may be a potentially useful supplementary therapy in severely affected patients with anti-NMDAR encephalitis. Further large cohort study and animal experiment may help us elaborate the utility of intrathecal injection of methotrexate and its mechanism of action.

Objective To propose time-effect standards and standard treatment measures for battlefield extremity injuries.Methods By conducting retrospective analysis of battlefield extremity injuries in the militariy in different countries using literature retrieval and comparative analysis,time-effect standards and standard technical measures of battlefield extremity injuries were outlined.Results In wars of conventional weapons,extremity injuries are the most prevalent.Such treatment should give top priority to the timing of rescue and be implemented in conjunction with the injury classification.Conclusion Time-effect standards and standard treatment measures for battlefield extremity injuries are proposed to strengthen the time-effect treatment capacity of battlefield extremity injuries.

BACKGROUNDAutoimmune encephalitis associated with antibodies against γ-aminobutyric acid B receptor (GABA B R) in patients with limbic encephalitis (LE) was first described in 2010. We present a series of Han Chinese patients for further clinical refinement.

METHODSSerum and cerebrospinal fluid (CSF) samples from patients referred to the program of encephalitis and paraneoplastic syndrome of Peking Union Medical College Hospital were tested with indirect immunofluorescence. Clinical information of patients with anti-GABA B R antibody positivity was retrospectively reviewed, and descriptive statistical analysis was performed.

RESULTSAll eighteen anti-GABA B R antibody-positive cases had limbic syndromes, and electroencephalogram (EEG) or neuroimaging evidence fulfilled the diagnostic criteria of LE. Four patients had additional antibodies against Hu in serum and one had anti-N-methyl-d-aspartate receptor antibody in both sera and CSF. Seventeen (17/18) patients presented with new-onset refractory seizure or status epileptics. Twelve (12/18) patients had memory deficits, 11 (11/18) patients had personality change, 7 (7/18) patients had disturbance of consciousness, and 3 (3/18) patients showed cerebellar dysfunction. One patient with LE had progressive motor and sensory polyneuropathy. Lung cancer was detected in 6 (6/18) patients. Ten (10/18) patients showed abnormality in bilateral or unilateral mediotemporal region on magnetic resonance imaging. Ten (10/18) patients had temporal lobe epileptic activity with or without general slowing on EEG. Seventeen patients received immunotherapy and 15 of them showed neurological improvement. Four patients with lung cancer died within 1-12 months due to neoplastic complications.

CONCLUSIONSOur study demonstrates that most Han Chinese patients with anti-GABA B R antibody-associated LE have prominent refractory epilepsy and show neurological improvement on immunotherapy. Patients with underlying lung tumor have a relatively poor prognosis. Testing for anti-GABA B R antibodies is necessary for patients with possible LE or new-onset epilepsy with unknown etiology.

Adult ; Autoantibodies ; immunology ; China ; Electroencephalography ; Epilepsy ; immunology ; pathology ; Female ; Humans ; Limbic Encephalitis ; immunology ; pathology ; Male ; Middle Aged ; Receptors, N-Methyl-D-Aspartate ; immunology ; Retrospective Studies ; gamma-Aminobutyric Acid ; metabolism

OBJECTIVETo compare the pharmacokinetic parameters of pyridostigmine bromide dispersible tablets and common tablets in rabbits.

METHODSTwelve rabbits were given an oral dose (60 mg) of pyridostigmine bromide dispersible tablets or common tablets in a randomized crossover study. The plasma concentration of pyridostigmine bromide was determined by reversed-phase ion pair chromatography. The pharmacokinetic parameters were calculated using DAS2.1.1 software.

RESULTSThe pharmacokinetic parameters showed no significant differences in rabbit plasma between pyridostigmine bromide dispersible tablets and common tablets. The two tablets had a C(max) of 1.83∓0.08 mg·L(-1) and 1.68∓0.03 mg·L(-1), tmax of 2.33∓0.41 h and 2.58∓0.20 h, AUC(0-24) of 15.50∓0.62 mg·h·L(-1) and 15.14∓0.30 mg·h·L(-1), AUC(0-∞) of 15.82∓0.70 mg·h·L(-1) and 15.57∓0.32 mg·h·L(-1), respectively. The relative bioavailability F(0-24) was 102.38% and F(0-∞) was 101.61% for the dispersible tablets.

CONCLUSIONThe two tablets are bioequivalent in rabbits.

Administration, Oral ; Animals ; Biological Availability ; Female ; Male ; Pyridostigmine Bromide ; administration & dosage ; blood ; pharmacokinetics ; Rabbits ; Tablets ; Therapeutic Equivalency

The aim of this study was to explore the mechanism underlying the blast crisis of chronic myeloid leukemia. Analysis of gene expression profiles of chronic myeloid leukemia patients in chronic phase and blast crisis were analyzed by using cDNA microarray representing 4096 genes for finding the differential expression genes. The results indicated that 74 differential expression genes were identified in at least 3 gene chips in blast crisis compared with chronic phase, among them 52 genes were down-regulated and 22 genes were up-regulated in blast crisis. The differential expression genes were involved in these genes including genes related to cell structure/mobility, signal transduction, transcription factor, related immunity, metabolism, cell cycle, oncogene/anti-oncogene, cell receptor, protein translation/synthesis and some unknown functions. It is concluded that the blast crisis of CML is resulted from abnormality and interaction of multigene, among them functional abnormal genes related to signal transduction, cell cycle, cell differentiation and immunity may be the critical genes for chronic myeloid leukemia blast crisis.
Adult ; Aged ; Female ; Gene Expression Profiling ; Gene Expression Regulation, Leukemic ; Genes, Neoplasm ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; genetics ; metabolism ; Male ; Middle Aged ; Oligonucleotide Array Sequence Analysis ; Signal Transduction

OBJECTIVETo investigate the hemodynamic changes of primary hepatocellular carcinoma (HCC) evolved from hepatic cirrhosis using CT perfusion imaging.

METHODSThirty-two patients with primary hepatocellular carcinoma evolved from virus-induced fibrosis or cirrhosis underwent dynamic CT scanning of the target slices for 60 min. The perfusion parameters of the hepatic parenchyma and HCC including the blood flow (BF), blood volume (BV), mean transit time (MTT), permeability-surface area product (PS), hepatic arterial fraction (HAF), IRF time of arrival (IRF TO) were obtained. Paired-sample t test was used to determine the differences in the perfusion parameters between the hepatic parenchyma and the primary HCC mass.

RESULTSCompared with hepatic BF (117.13-/+31.05 ml/100 mg/min), BV (14.73-/+3.91 ml/100 mg), PS (31.93-/+5.91 ml/100 mg/min), HAF (25.02-/+8.19%), MTT (12.79-/+3.31 s), IRF TO (3.14-/+1.09 s), the primary HCC mass showed significant increments in the BF (239.69-/+96.07 ml/100 mg/min), BV (20.26-/+6.73 ml/100 mg), PS (37.50-/+9.50 ml/100 mg/min), HAF (68.97-/+15.22%) with decreased MTT (7.17-/+1.38 s) and IRF TO (2.42-/+0.94 s). Significant differences were found in all the perfusion parameters between the hepatic parenchyma and HCC (P<0.05).

CONCLUSIONLiver perfusion parameters can represent the hemodynamic changes in the HCC derived from hepatic cirrhosis.

Adult ; Aged ; Carcinoma, Hepatocellular ; diagnostic imaging ; etiology ; physiopathology ; Female ; Hemodynamics ; Hepatitis ; complications ; Humans ; Image Interpretation, Computer-Assisted ; methods ; Liver Cirrhosis ; complications ; Liver Neoplasms ; diagnostic imaging ; etiology ; physiopathology ; Male ; Middle Aged ; Perfusion ; methods ; Radiographic Image Enhancement ; instrumentation ; methods ; Tomography, X-Ray Computed