OBJECTIVE: To explore the safety and efficacy of high-dose etoposide (VP-16) combined with recombinant human granulocyte colony-stimulating factor (rhG-CSF) as salvage mobilization for peripheral blood stem cells (PBSC) in newly diagnosed multiple myeloma (NDMM) patients. METHODS: From April 2021 to May 2023, eight NDMM patients who had failed to yield sufficient PBSC during initial mobilization with high-dose cyclophosphamide (CTX) combined with rhG-CSF underwent salvage mobilization with 1.2 g/m2 etoposide combined with rhG-CSF 10 μg/(kg·d). The effects and adverse reactions of initial mobilization and salvage mobilization were analyzed. RESULTS: For salvage mobilization and initial mobilization, the numbers of PBSC collections were 16 and 18, respectively. The mean value of total collected CD34⁺ cells were (11.90±5.75)×10⁶/kg and (1.67±0.75)×10⁶/kg (P =0.0010) in salvage mobilization group and initial mobilization group, respectively. The proportion of patients with a total collection of CD34⁺ cell count≥2×10⁶/kg were 100% and 37.5% (P =0.0625), and the proportion of patients with a total collection of CD34⁺ cell count≥5×10⁶/kg were 87.5% and 0% (P =0.0156) in salvage mobilization group and initial mobilization group, respectively. For five patients who underwent high-dose CTX initial mobilization but had a total CD34⁺ cell count < 2×10⁶/kg, successful collection was achieved through salvage mobilization with high-dose VP-16. Salvage mobilization with high-dose VP-16 was scheduled 2-3 weeks after failure of CTX mobilization. Adverse reactions of high-dose VP-16 mobilization did not increase compared to the initial mobilization with high-dose CTX. CONCLUSION As a salvage mobilization regimen, VP-16 1.2 g/m² combined with rhG-CSF is safe and highly effective in NDMM patients who failed to initial mobilization with high-dose CTX combined with rhG-CSF.
Humans ; Multiple Myeloma/therapy* ; Etoposide/therapeutic use* ; Hematopoietic Stem Cell Mobilization/methods* ; Cyclophosphamide/therapeutic use* ; Granulocyte Colony-Stimulating Factor ; Salvage Therapy ; Peripheral Blood Stem Cells ; Male ; Middle Aged ; Female ; Peripheral Blood Stem Cell Transplantation

Lipopolysaccharides (LPS) are major outer membrane components of Gram-negative bacteria. Unlike most Gram-negative bacteria, Acinetobacter baumannii can still survive and acquire polymyxin resistance after complete loss of LPS. Previous studies of LPS-deficient Acinetobacter baumannii mostly focused on LPS-deficient strains induced by polymyxin, whose background was complex and unstable. To investigate LPS loss-mediated polymyxin resistant-Acinetobacter baumannii, this study constructed a stable and clear background LPS-deficient strain by knocking out lpxC gene in Acinetobacter baumannii ATCC 19606 with CIRSPR/Cas9, and then studied the phenotypic changes of the lpxC-deficient strain including morphology, growth rate, antibiotic susceptibility, virulence, membrane permeability, and membrane potential. Animal experiments were approved by the Animal Care and Welfare Committee Institute of Medicinal Biotechnology, CAMS and PUMC (approval number: IMB-20240119D₉). The results indicated that the lpxC gene of Acinetobacter baumannii ATCC 19606 was successfully knocked out. After losing the lpxC, the strain underwent the morphological change from rod-shaped to spherical. Furthermore, it leads to reduced growth rate, enhanced membrane permeability, decreased membrane potential, lower virulence, and increased antibiotic susceptibility to β-lactams, quinolones, aminoglycosides, macrolides, glycopeptides. The lpxC deletion results in significant changes in membrane homeostasis and adaptability of Acinetobacter baumannii. Understanding the phenotypic changes of colistin-resistant Acinetobacter baumannii mediated by LPS loss is useful for exploring the resistance mechanism of Acinetobacter baumannii and developing new therapeutic strategies.

OBJECTIVE: To analyze the clinical efficacy of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) by using parental donors on thalassemia patients. METHODS: The 13 thalassemia patients treated by haplo-HSCT using parental donors in our hospital from July 1, 2016, to July 1, 2020 were retrospectively reviewed. Hematopoiesis reconstitution, the incidence of GVHD, infections and the long-term survival of the patients were analyzed. RESULTS: Twelve of the 13 patients were successfully implanted, the success rate of implantation was 92.3%. The median time of neutrophil and platelet engraftment was 12.5 days (range, 9-22 days) and 21 days (range,12-34 days), respectively. One patient achieved primary graft failure. Three (25%) patients developed to acute GVHD (aGVHD) and achieved complete remission after treatment. Chronic GVHD developed in three (25%) patients, one of them was extensive and under treatment, while one patient developed to severe bacterial infection (7.7%). CMV viremia was diagnosed in two patients (15.4%). There were no patients developed to CMV disease. Three (23.1%) patients achieved EB viremia after transplantation, one of them developed to EBV-related lymphocytic proliferative disease, while there were no patients showed invasive fungal infection. At the last follow-up, all patients survived, twelve of them were free from transfusion dependency. There were no transplant-related deaths. Projected overall and thalassemia-free survival at three years was 100% and 92.3%, respectively. CONCLUSION The transplant protocol of haplo-HSCT by using parental donors in patients with thalassemia has reliable source of donors, high incidence of successful implantation and low incidence of GVHD, which can be used as an effective way to increase the source of donors in children with thalassemia.
Child ; Cytomegalovirus Infections ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Parents ; Retrospective Studies ; Thalassemia/therapy* ; Transplantation Conditioning/methods* ; Treatment Outcome ; Viremia

OBJECTIVES: To investigate the distribution of body mass index (BMI) and risk factors for obesity in children with Diamond-Blackfan Anemia (DBA). METHODS: The children with DBA who attended National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, from January 2003 to December 2020 were enrolled as subjects. The related clinical data and treatment regimens were recorded. The height and weight data measured within 1 week before or after follow-up time points were collected to calculate BMI. The risk factors for obesity were determined by multivariate regression analysis in children with DBA. RESULTS: A total of 129 children with DBA were enrolled, among whom there were 80 boys (62.0%) and 49 girls (38.0%), with a median age of 49 months (range 3-189 months). The prevalence rate of obesity was 14.7% (19/129). The multivariate logistic regression analysis showed that the absence of ribosomal protein gene mutation was closely associated with obesity in children with DBA (adjusted OR=3.63, 95%CI: 1.16-11.38, adjusted P=0.027). In children with glucocorticoid-dependent DBA, obesity was not associated with age of initiation of glucocorticoid therapy, duration of glucocorticoid therapy, and maintenance dose of glucocorticoids (P>0.05). CONCLUSIONS There is a high prevalence rate of obesity in children with DBA, and the absence of ribosomal protein gene mutation is closely associated with obesity in children with DBA.
Child ; Male ; Female ; Humans ; Anemia, Diamond-Blackfan/genetics* ; Pediatric Obesity/complications* ; Glucocorticoids/therapeutic use* ; Prevalence ; Risk Factors ; Ribosomal Proteins/genetics* ; Mutation

OBJECTIVE: To retrospectively analyze the efficacy and safety of ruxolitinib therapy for children with thalassemia after unrelated or haploidentical stem cell transplantation. METHODS: From March 2020 to March 2021, 22 patients received successfully allogeneic hematopoietic stem cell transplantation in the Zhongshan Hospital of Xiamen University, from +30 to 100 days,those patients received ruxolitinib therapy (2.5 mg, twice daily) and all adverse reactions were observed, include aGVHD, cGVHD, CMV and EBV infection. RESULTS: 22 patients underwent allogeneic stem cell transplantation, 5 patients were diagnosed as aGVHD, 3 patients had grade I－II skin GVHD and 2 patients had grade II intestinal GVHD, those patients were cured. All patients were followed up for more than 21 weeks, 4 cases developed cGVHD, including 3 cases of localized liver GVHD and 1 case of pulmonary GVHD, those were relieved after active treatment. 8 patients had elevated EBV copies (>3×10³/ml), and 3 patients had increased CMV copies, the patients recovered after immunosuppressant and antiviral treatment. There was no CMV infection and EBV related post-transplantant lymphoproliferative disorders(PTLD), and no transplant related deaths. CONCLUSION Ruxolitinib can effectively reduce the incidence and severity of GVHD without affecting the hematopoietic recovery, and improve the survival status of thalassemia children after transplantation.
Antiviral Agents/therapeutic use* ; Child ; Graft vs Host Disease/prevention & control* ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Humans ; Immunosuppressive Agents/therapeutic use* ; Nitriles ; Pyrazoles ; Pyrimidines ; Retrospective Studies ; Thalassemia

Banxia Xiexin Tang is one of the classic prescriptions for treating digestive system diseases.In this paper，using the method of bibliometrics，information of Banxia Xiexintang in ancient Chinese medical literatures were collected and screened out 399 effective data from 238 kinds of ancient books．Based on the statistics and analysis of the history，drug composition，main disease and syndrome，principle of prescription，dosage，processing，preparation，decocting and taking methods of Banxia Xiexin Tang，it is found that Banxia Xiexin Tang originated from Treatise on Febrile and Miscellaneous Disease written by ZHANG Zhong-jing，a famous physician in the Eastern Han dynasty，it is composed of seven herbs，namely，Pinelliae Rhizoma，Scutellariae Radix，Zingiberis Rhizoma，Ginseng Radix et Rhizoma，Glycyrrhizae Radix et Rhizoma Praeparata cum Melle，Coptidis Rhizoma，and Jujubae Fructus. It was mainly used to treat pizheng of mixed cold and heat. Most of the Banxia Xiexin Tang recorded in later generations follow the prescription composition and indications in Treatise on Febrile and Miscellaneous Disease and its clinical application has been extended and expanded，among the 352 literatures with the main diseases and syndromes recorded，the most common （341） were pizheng，accounting for about 96.88%，in addition，it is also used sporadically for shuzheng，malaria，nausea，damp-wen，jaundice，etc. Among the 122 documents with drug dosage records，nearly half of them are identical with the original records in Treatise on Febrile and Miscellaneous Disease. Among the 112 literatures with pharmaceutical processing，licorice （86） was the most，most of them were roasted （80），followed by pinellia ternata （79），and most of them were "decoction washing and sliding" （67）. Among the 111 documents recorded in the decoction method，most of them inherited Treatise on Febrile and Miscellaneous Disease by "removing the dross and again cooking" （68），there are also "Water Decoction" （32） and "Ginseng Radix et Rhizoma and Jujubae Fructus Decoction" （11）. Among the 108 documents with the method of taking medicine，nearly half of them inherited Treatise on Febrile and Miscellaneous Disease，which is "a liter of warm taking ，three times daily". Based on the ancient Chinese medical literatures，Banxia Xiexintang was systematically analyzed in order to provide more accurate ancient literature reference for the clinical application and development of classic prescriptions.

Objective:To summarize the reason and treatment of ventricular electrical storm (VES) in adult patients after cardiac surgery.Methods:A total of 36 adult patients with VES after cardiac surgery in our hospital from 2011-08 to 2015-05 were retrospectively analyzed for their peri-operative and clinical conditions before and after VES occurrence.Results:All 36 patients suffered from VES after cardiac surgery during ICU stay including 23 patients with coronary artery bypass grafting (CABG),5 with CABG and ventricular aneurysm resection,5 with valve replacement,2 with Bentall operation and 1 with Marrow operation.There were 32/36 (88.89％) patients out of the breathing machine upon VES disappearing and discharged smoothly,4 (11.11％) patients died of multiple organ failure.There were 22 (61.11％) patients with myocardium ischemia induced VES.32 patients were followed-up for 1 year,28/36 (77.78％) patients recovered to sinus rhythm,2 (5.56％) received endocardial pacemaker placement and 2 (5.56％) suffered from atrial fibrillation;no cardiac death during follow-up period.Conclusion:VES was often occurred at the early stage in adult patients after cardiac surgery;it usually had clear reason or inducement,rapid and efficient treatment could achieve satisfactory results.

Objective To learn the status of human resources of vaccination clinics in Zhejiang province and to give suggestions for optimizing the allocation.Methods Investigation and the focus group interview were conducted in Zhejiang Province to learn the configuration standards in vaccination clinics.Results The mean number who attended the immunization work every vaccination day in those clinics was five.The number of staff in each clinic differed from county to county but all reached 0.9 persons serving for 10,000 residents.There should not be less than four persons in each vaccination clinic theoretically.It should be necessary to increase one vaccination staff in every 50 injection doses of vaccine workload.Conclusion The staff of vaccination clinics is inadequate and unbalanced in Zhejiang Province.We should increase staff numbers according to workload and served population.

Adult ; Arthropathy, Neurogenic ; etiology ; immunology ; pathology ; Female ; Granuloma, Plasma Cell ; complications ; immunology ; pathology ; Humans ; Lung Neoplasms ; complications ; immunology ; pathology

OBJECTIVETo summarize and analyze the experience in diagnosis and treatment of primary malignant tumors of the heart and pericardium.

METHODSThe clinical data of 24 patients with malignant tumors of the heart and pericardium treated in our hospital between Jun. 1980 and Mar. 2008 were retrospectively analyzed and the related literature was reviewed.

RESULTSAll 24 patients received surgical treatment. Radical resection in 10 cases and partial resection in 6 cases were performed, and the remaining 8 patients received only surgical exploration due to unresectable tumors. One patient died of cardiopulmonary failure in early postoperative stage and all the other patients died within 4 years after operation due to recurrence or metastases.

CONCLUSIONEchocardiography, CT, MRI, 3D-CT, CT of coronary artery and cardiac angiography are helpful for the diagnosis and selection of operation mode. Histological examination is necessary for the final diagnosis. Early diagnosis and surgical resection of the tumor as complete as possible, and combination with post-operative radiotherapy and/or chemotherapy may improve the survival of the patients.

Adolescent ; Adult ; Aged ; Female ; Follow-Up Studies ; Heart Neoplasms ; diagnosis ; surgery ; Humans ; Male ; Mesothelioma ; diagnosis ; surgery ; Middle Aged ; Myxoma ; diagnosis ; surgery ; Pericardium ; surgery ; Retrospective Studies ; Survival Rate ; Young Adult