The growth curve of single cell organisms in batch cultivation could divide into 6 phases, lag phase, acceleration phase, log phase, deceleration phase, stationary phase, and death phase, based on specific growth rate during cultivation process. There were significantly differences between deceleration phase and the other phases in cell growth, substrate consumption, product formation, and genes express profile. The deceleration phase was highly important to fermentation process. However, cognizing and teaching to the deceleration phase had been considerably weakened since a long period. So it should be strengthened.

OBJECTIVETo investigate the safety and effectiveness of HLA-mismatched allogeneic hematopoietic stem cell transplantation (allo-HSCT) combined with related haploidentical bone marrow infusion for treatment of hematologic malignancies and to explore the mathod for reduction of aGVHD incidence and clinical significance.

METHODSA total of 30 patients with hematologic malignancies (8 cases of AML, 17 AML, 2 MDS and 3 Mix-AL) received related haploidentical and unrelated HLA-mismatched allo-HSCT combined with related haploidentical bone marrow infusion. Among them 20 cases received related haploidentical transplantation of the first donor, 10 cases received unrelated HLA-mismatched treaplantation. The new conditioning regimen for the patients underwent allo-HSCT consisted of fludarabine, busulfan, Me-CCNU and cyclophosphamide. The drugs for GVHD prophylaxis included cyclosporine A and methotrexate, while mycophenolate mofetil and rabbit anti-T-lymphocyte globulin (ATG) were used.

RESULTSAll the patients achieved full engraftment. The median time for neutrophils to reach over 0.5 × 10(9)/L was 14 days (8-26 days), while the median time for platelets to reach over 20 × 10(9)/L was 11.5days (10-24 days). The incidence of I-II grade of aGVHD at 100 d was 22.28% (95% CI 9.9%-34.7%), the incidences of II-IV and III-IV grade of aGVHD were 22.7% (95% CI, 10%-35.4%) and 12.7% (95% CI 6.9%-15.5%) respectively. The incidences of I-II and III-IV cGVHD were 13.3% (95% CI, 1.4%- 26.8%) and 3.3 % (95% CI, 0%-12.2%), one case (3.3%) was in extensive cGVHD. DFS and OS of 2 years were 81.1% (95% CI, 66.0%-96.2%) and 68.2% (95% CI 51.0%-85.4%).

CONCLUSIONThese data suggest that the incidence of grade II-IV grade of aGVHD in recipients of 2 partially HLA-matched units was lower, co-infusion of haplo-BM and partially matched units in allogeneic transplantation is safe and effective for reducing the incidence of aGVHD and improving the survival in DFS.

Antilymphocyte Serum ; therapeutic use ; Busulfan ; therapeutic use ; Cyclosporine ; therapeutic use ; Graft vs Host Disease ; prevention & control ; HLA Antigens ; genetics ; Hematologic Neoplasms ; therapy ; Hematopoietic Stem Cell Transplantation ; Humans ; Incidence ; Leukemia ; therapy ; Mycophenolic Acid ; analogs & derivatives ; therapeutic use ; Stem Cell Transplantation ; Tissue Donors ; Transplantation Conditioning ; Transplantation, Homologous ; Vidarabine ; analogs & derivatives ; therapeutic use

OBJECTIVETo study the clinical features, diagnosis and therapy of hydroa vacciniforme-like cutaneous T cell lymphoma.

METHODSThe clinical presentations and the findings of laboratory examinations and skin biopsy of affected tissue in a child with hydroa vacciniforme-like cutaneous T cell lymphoma were retrospectively reviewed.

RESULTSThe child manifested as rash, fever and lymph node intumesce. Rash was pantomorphia, including edematous erythema, vesicles, crusts, necrosis and depressed scar, and it was mild in winter and severe in summer, mainly involving in the face and extremities. Epstein-Barre virus (EBV)-IgM was positive. Histopathological findings revealed focal lymphocyte invasion in subcutaneous panniculus adiposus, mainly surrounding the blood vessels. Immunohistochemistry showed CD3 (+), CD43 (+), CD20 (-), pax-5 (-), TIA (+), CD5 (+), CD8 (+), Granmye (+) and CD4 (-). The clinical symptoms were improved after glucocorticoid treatment in this child.

CONCLUSIONSHydroa vacciniforme-like cutaneous T cell lymphoma has special clinical manifestations. This disorder may be definitely diagnosed by skin biopsy of affected tissue and immunohistochemistry assay. Glucocorticoid treatment is effective. EBV infection may be related to the development of this disorder.

Child, Preschool ; Female ; Humans ; Hydroa Vacciniforme ; pathology ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; immunology ; pathology ; Skin ; pathology ; Skin Neoplasms ; drug therapy ; immunology ; pathology

BACKGROUNDThe cochlear hydrops analysis masking procedure (CHAMP) is a new diagnostic technique for Meniere's disease (MD). But its value has not been well proven. This study aimed to evaluate the diagnostic value of CHAMP for MD.

METHODSCHAMP test was taken in three populations using the Auditory Evoked Potential system delivered by Bio-logic Systems Corporation: (1) otologically normal subjects; (2) patients clinically diagnosed with definite MD; (3) patients clinically diagnosed with probable and possible MD.

RESULTSAccording to the comparison between the normal and definite MD group, if the abnormal criterion of CHAMP was defined as latency delay less than 0.3 ms, then the corresponding sensitivity was only 52%. However, if the abnormal criterion was defined as latency delay between 0.6 and 3.8 ms, then a sensitivity of 93% and a specificity of 100% can be achieved. The complex amplitude ratio showed a significant overlap between normal and definite MD group. If the abnormal criterion was defined as a complex amplitude ratio less than 0.95, the corresponding specificity was only 50%. However, if the abnormal criterion was defined as less than 0.80, the corresponding sensitivity was 60%, and the specificity was 97%. If the abnormal criterion of CHAMP was defined as latency delay less than 0.6 ms or the complex amplitude ratio less than 0.80, CHAMP result can be obtained in all subjects with good sensitivity and specificity.

CONCLUSIONSCHAMP can differentiate patients with Meniere's disease from otologically normal subjects with high sensitivity and specificity. The recommended criterion of abnormal CHAMP was a latency delay less than 0.6 ms or a complex amplitude ratio less than 0.80.

Adolescent ; Adult ; Aged ; Audiometry, Evoked Response ; Endolymphatic Hydrops ; diagnosis ; physiopathology ; Female ; Humans ; Male ; Meniere Disease ; diagnosis ; physiopathology ; Middle Aged ; Young Adult

Female ; Humans ; Lymphoma, T-Cell ; diagnosis ; Middle Aged ; Multiple Myeloma ; diagnosis

OBJECTIVETo investigate the role of AT2 receptors in the secretion of tumor necrosis factor alpha (alpha-TNF) and interleukin 1 beta (IL1 beta) in adult rat cardiac fibroblasts.

METHODSAdult rat cardiac fibroblasts in in vitro culture were divided into control, Ang II, AngII + Losartan, and AngII + PD123319 groups with corresponding treatments. Radioimmunoassay was used to determine alpha-TNF and IL1 beta levels in the supernatant of the treated cardiac fibroblasts.

RESULTSAng II treatment resulted in significantly increased alpha-TNF and IL1 beta levels. Compared with AngII group, IL1 beta level was decreased by 69.1% and 78.7% and alpha-TNF by 58.7% and 65.9% after blocking AT1 and AT2 receptors, respectively.

CONCLUSIONAT2 receptors are involved in alpha-TNF and IL1 beta secretions in cardiac fibroblasts.

Angiotensin II ; pharmacology ; Animals ; Fibroblasts ; drug effects ; metabolism ; secretion ; Gene Expression Regulation ; drug effects ; Interleukin-1beta ; secretion ; Male ; Myocardium ; cytology ; Rats ; Rats, Sprague-Dawley ; Receptor, Angiotensin, Type 2 ; metabolism ; Tumor Necrosis Factor-alpha ; secretion

OBJECTIVEWernicke's encephalopathy (WE) is an acute neuropsychiatric syndrome resulting from thiamine deficiency, which is associated with significant morbidity and mortality. The disorder is still greatly underdiagnosed in children because of either a relatively non-specific clinical presentation in some cases or unrecognized clinical setting. The aim of this literature review was to provide knowledge of pediatric WE in an effort to assist in early diagnosis, thereby reducing the morbidity and mortality.

METHODSThe clinical manifestations, characteristic magnetic resonance imaging (MRI), diagnosis and treatment of one case and the other 35 cases reported in the last decade in children were summarized.

RESULTSThirty-six cases (22 boys and 14 girls, 2-month to 16-year-old) were analyzed. All the other 35 cases except for our case had underlying diseases: improper feeding in 25/35 cases, long-time vomiting in 5/35 cases, immunosuppressive therapy in 4/35 cases, long-time total parenteral nutrition without multivitamin preparations supplementation in 3/35 cases and anorexia nervosa in 1/35 case. The classic triad (mental-status changes, nystagmus and ophthalmoplegia, and ataxia) was seen in 6/36 cases. The other clinical manifestations included consciousness disturbance in 24/36 cases, infection in 22/36 cases, pathological reflex and muscular tension changes in 18/36 cases, convulsion in 17/36 cases, developmental delay in 4/36 cases and failure to thrive in 2/36 cases. Cerebrospinal fluid examination was performed in 31/36 cases, and a slightly raised protein concentration was seen in 7/31 cases. The cerebrospinal fluid lactate levels were detected in 4/36 cases (all increased), serum lactic acid levels in 7/36 cases (6/7 cases increased), serum pyruvate in 4/36 cases (all increased), thiamine pyrophosphate effect (TPPE) in 9/36 cases (all increased), and serum thiamine in 2/36 cases (increased in 1/2 cases). The brain computed tomography (CT) scan was conducted in 20/36 cases and 16/20 cases showed abnormal hypodensity in bilateral basal ganglia, one case revealed diffuse cortical atrophy. The brain MR scan was conducted in 13/36 cases and all the 13 cases revealed symmetrical abnormal signal in bilateral mamillary body and basal ganglia, and 7/13 cases showed abnormal signals in the tegmentum of midbrain, cerebral aqueduct and white matter around the third and fourth ventricles. The diagnosis of WE was confirmed by MR in 12 cases, triad combined with MR in 3 cases, autopsy in 1 case among the 13 cases who underwent MR scan. The diagnosis of WE was confirmed by the TPPE and/or lactate levels in 9/11 cases. The initial thiamine was given by intravenous or intramuscular infusion in 33/36 cases, unknown method in 1 case, orally in 1 case and no thiamine was used in 1 case. The dosage of thiamine was 100 mg daily in 29/35 cases, unknown in 3/35 cases, 50 mg daily in 2/35 cases, 600 mg daily in 1/35 case. 34/35 patients' clinical symptoms improved during 24 hours to 1 week after initial treatment, and 1 case died due to no response to thiamine. Nineteen patients were followed up for 2-2.5 months and 17 cases recovered completely.

CONCLUSIONWernicke's encephalopathy can be difficult to diagnose because of a relatively non-specific clinical presentation. The characteristic MRI findings and the dramatic response of neurological signs to parenteral thiamine will assist early clinical diagnosis. Early and timely thiamine supplementation could reverse the clinical features and improve the prognosis in most cases.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Sepsis ; complications ; Wernicke Encephalopathy ; complications ; diagnosis

Objective To report the coronary angiographic features and in-hospital outcome in coronary artery disease(CAD) patients with rheumatoid arthritis(RA) who underwent percutaneous coronary intervention(PCI)with stent implantation.Methods The study group comprised 69 consecutive patients with RA who underwent PCI in Beijing Friendship Hospital between January 2009 and June 2017. Medical records were analyzed retrospectively including clinical basic material, coronary angiogram data, and the incidence of major adverse events in hospital.Results Fifty five patients(79.7％) had one or more traditional CAD risk factors. Multivessel disease was present in 73.9％ of the patients, and the average SYNTAX score was(31.6±7.2). Two bare metal stents and 143 drug eluting stents were implanted. 50 cases(72.4％) required implantation of long stents(stent length>30 mm) .There were 25 patients developed major adverse events(36.2％) during hospitalization, including 1 case of cardiac death, 2 cases of stent thrombosis, 5 cases of PCI related MI, 5 cases of heart failure, 5 cases of gastrointestinal bleeding and 10 cases of contrast induced nephropathy(2 cases comorbided acute heart failure).Conclusions Patients with RA and CAD may have severe coronary lesions.PCI in these patients tends to result in an increased rate of major adverse events in-hospital. Hence there is a need of early identification and early prevention in these patients during perioperative period.

BACKGROUNDCoronary artery disease (CAD) is a leading cause of morbidity and mortality in patients with connective tissue diseases (CTDs). Risk factors and clinical characteristics in these patients are not equivalent to those in traditional CAD patients. The objective of this study was to report short- and long-term clinical outcomes in a consecutive series of patients with CTD who underwent percutaneous coronary intervention (PCI) with stent implantation.

METHODSThe study group comprised 106 consecutive patients with CTD who underwent PCI in Beijing Friendship Hospital between January 2009 and June 2012. Medical records were analyzed retrospectively including clinical basic material, coronary angiogram data, and the incidence of major adverse cardiac events (MACEs) during the short- and long-term (median 3 years) follow-up.

RESULTSNinety-two of the patients (86.8%) had one or more traditional CAD risk factors. Multivessel disease was present in more than 2/3 of patients (73.6%). The left anterior descending coronary artery was the most commonly affected vessel (65.1%). Five bare-metal stents and 202 drug-eluting stents were implanted. After a median follow-up period of 36 months, thirteen patients (12.3%) died from cardiac causes, the rate of stent thrombosis was 9.4%, and the rate of target vessel revascularization (TVR) was 14.2%. Multivariate analysis revealed that hypertension (hazard ratio [HR] = 3.07, 95% confidence interval [CI]: 1.30-7.24, P = 0.041), anterior myocardial infarction (HR = 2.77, 95% CI: 1.06-7.03, P = 0.04), longer duration of steroid treatment (HR = 3.60, 95% CI: 1.43-9.08, P = 0.032), and C-reactive protein level >10 mg/L (HR = 3.98, 95% CI: 1.19-12.56, P = 0.036) were independent predictors of MACEs.

CONCLUSIONSPatients with CTD and CAD may have severe coronary lesions. PCI in these patients tends to result in an increased rate of stent thrombosis and TVR during long-term follow-up, which may be influenced by traditional and nontraditional risk factors.

Aged ; C-Reactive Protein ; analysis ; Connective Tissue Diseases ; complications ; Coronary Angiography ; Drug-Eluting Stents ; Female ; Humans ; Male ; Middle Aged ; Percutaneous Coronary Intervention ; Retrospective Studies ; Treatment Outcome

BACKGROUNDVon Hippel-Lindau disease (VHL), a heritable autosomal dominant disease characterized by neoplasia in multiple organ systems, has rarely been reported in Asia. We genetically investigated a unique Chinese family with VHL disease and performed an analysis of the VHL protein stability.

METHODSGenomic deoxyribonucleic acid (DNA) extracted from peripheral blood was amplified by polymerase chain reaction (PCR) to three exons of the VHL gene in 9 members of the Chinese family with VHL disease. PCR products were directly sequenced. We estimated the effects of VHL gene mutation on the stability of pVHL, which is indicated by the free energy difference between the wild-type and the mutant protein (ΔΔG).

RESULTSThe Chinese family was classified as VHL type 1. Three family members, including two patients and a carrier, had a T to G heterozygotic missense mutation at nucleotide 515 of the VHL gene exon 1. This missense mutation resulted in the transition from leucine to arginine in amino acid 101 of the VHL protein. There was low stability of the VHL protein (the ΔΔG was 12.71 kcal/mol) caused by this missense mutation.

CONCLUSIONSWe first reported a family with this VHL gene mutation in Asia. This missense mutation is predicted to significantly reduce the stability of the VHL protein and contribute to the development of the renal cell carcinoma (RCC) phenotype displayed by this family. The genetic characterization and protein stability analysis of families with VHL disease are important for early diagnosis and prevention of the disease being passed on to their offspring.

Adult ; China ; Female ; Humans ; Male ; Middle Aged ; Mutation, Missense ; Protein Stability ; Von Hippel-Lindau Tumor Suppressor Protein ; chemistry ; genetics ; von Hippel-Lindau Disease ; genetics