1.Treatment of thalassemia major with unrelated donor bone marrow transplantation.
Wei-guo ZHU ; Zhi-chun FENG ; He-kui LAN ; Chuan-cang ZHANG ; Qiu-ping LI ; Zheng-dao LI
Chinese Journal of Pediatrics 2005;43(8):599-602
OBJECTIVEAllogeneic marrow transplantation is a curative therapy for thalassemia, but no more than 30% of patients have HLA-indentical sibling marrow donor. The selection of alternative donors of unrelative marrow and the study on the probability of treating thalassemia major with unrelated donor bone marrow transplantation are of importance.
METHODSNine children with thalassemia were included in the study, and their gene mutational type were homozygote of thalassemia and double heterozygote, respectively. All of them were finally diagnosed of thalassemia major, and treated with unrelated donor bone marrow transplantation. To high-resolution HLA typing, two patients were matched, five had one unmatched isoform and two had two unmatched isoforms. The erythrocyte blood type was not matched in six patients. The preparative regimen included busulfan (oral use, 16 mg/kg, divided for 4 days), cyclophosphamide (intravenous use, 200 mg/kg, divided for 4 days), antithymocyte immunoglobulin (intravenous use, 30 mg/kg, divided for 3 days), and fludarabine (intravenous use, 125 mg/m(2), divided for 3 days). Ciclosporin A and methotrexate were used for graft-versus-host disease (GVHD) prophylaxis.
RESULTSAll patients had allergen reactions. One had hypotension. Five patients experienced I degrees approximately III degrees acute GVHD in the skin, while one had II degrees acute GVHD in liver. One patient had III degrees GVHD of intestines and gradually developed chronic GVHD in the skin, lungs and brain. One patient died of pulmonary hemorrhage. The duration when peripheral blood neutrophil count exceeded 0.5 x 10(9)/L was 12 - 26 days. The recovery time of WBC was as long as 23 - 110 days. Thrombocytes exceeded 50 x 10(9) within 61 approximately 142 days. The time when hemoglobin reached 100 g/L varied from 23 to 116 days. The last blood transfusion was on 13 - 62 days. Eight patients were fully grafted, while one was not grafted. During the 6 - 24 months of follow-up, seven patients' genotype of thalassemia major became normal. The erythrocyte blood type of five patients also changed into the same as that of donor. The hemoglobin was kept over 110 g/L without blood transfusion.
CONCLUSIONThe transplantation of unrelated donor bone marrow for thalassemia major was successful. Unrelated donor bone marrow transplantation could cure thalassemia major, which expanded the marrow donor source for the transplantation of thalassemia major.
ABO Blood-Group System ; Bone Marrow Transplantation ; adverse effects ; Child ; Child, Preschool ; Disease-Free Survival ; Female ; Follow-Up Studies ; Graft Rejection ; Graft Survival ; Histocompatibility Testing ; Humans ; Infant ; Male ; Transplantation Tolerance ; Transplantation, Homologous ; adverse effects ; Treatment Outcome ; beta-Thalassemia ; diagnosis ; therapy
2.Comparison of functional parameters of small airways between patients with typical asthma and cough-variant asthma.
Shu-Yu CHEN ; Ze-Kui FANG ; Si FANG ; Qi-Xiao SHEN ; Xi HE ; Cui-Lan WANG ; Hua-Peng YU
Journal of Southern Medical University 2017;37(3):330-336
OBJECTIVETo compare the functional parameters of the small airways and clinical characteristics between patients with typical asthma (TA) and cough-variant asthma (CVA).
METHODSForty-three newly diagnosed asthmatic patients were enrolled, including 15 with TA and positive bronchial provocation test [TA BPT(+)], 12 with TA and positive bronchial dilation test [TA BDT(+)] and 16 with CVA, and 27 healthy subjects served as the control group. All the subjects were required to complete data acquisition, asthma control test, asthma control test scale, fractional exhaled nitric oxide, airway resistance and pulmonary function tests, BPT or BDT.
RESULTSThe interval from onset to a definite diagnosis of TA BDT(+) was longer than that of TA BPT(+), while that of CVA was the shortest (P=0.022). The pulmonary functional parameters of TA BDT (+) was significantly lower than those of the other 3 groups (P<0.05). MMEF, MEF, MEF, and MEFin patients with TA BDT(+), TA BPT(+) and CVA were significantly lower than those in the control group (P<0.01). The resonant frequency, respiratory impedance, resistance at 5 Hz, resistance at 20 Hz, and reactance at 5 Hz were significant higher in patients with TA BDT (+) than in the control subjects, while these parameters showed no significant differences among TA BPT (+), CVA and control groups. The airway resistance in TA BPT(+), CVA, and control groups increased after BPT, and the patients with TA BPT(+) showed greater changes in airway resistance than those in CVA and control groups. In CVA patients, FeNO showed a strong positive correlation with respiratory impedance (r=0.523, P=0.038), resistance at 5 Hz (r=0.542, P=0.030), and resistance at 20 Hz (r=0.524, P=0.037), and the airway responsiveness showed a strong positive correlation with resistance at 20 Hz (Rho=-0.512, P=0.043).
CONCLUSIONCVA is the early stage of TA, and CVA, TA BPT(+), and TA BDT(+) may represent different stages of asthma. Uncontrolled, prolonged CVA may evolve into TA BPT (+), whose further progression can cause damages of the pulmonary function and small airway function and leads eventually to TA BDT (+).
3.Changes of peripheral blood chromosomal centromere aberration in patients with cytomegalovirus infection after anti-viral treatment.
Lan GAO ; Yan-hui LIU ; Li-fen LI ; Ya-min WU ; Ming-bo WANG ; Jing SHI ; Bing YUAN ; Juan SONG ; Yong-ming HE ; Dai-kui WEI
Journal of Southern Medical University 2009;29(9):1846-1847
OBJECTIVETo study the changes of peripheral blood chromosomal centromere aberration in patents with cytomegalovirus (CMV) infection after anti-viral treatment.
METHODSSixty-two patients with early spontaneous abortion and CMV infection analyzed for their peripheral blood chromosomal centromere using simultaneous silver staining before and after anti-viral treatment.
RESULTSThe patients with CMV infection had high rate of centromere aberration, which was decreased significantly after anti-viral treatment (P<0.0001).
CONCLUSIONCMV infection is a risk factor for peripheral blood chromosomal centrimere aberration. Anti-viral treatment can decrease the rate of centrimere aberration aberration. Detection of peripheral blood chromosomal centrimere aberration allows the assessment of the severity of infection and the condition after treatment.
Abortion, Spontaneous ; blood ; genetics ; Adult ; Antiviral Agents ; therapeutic use ; Centromere ; genetics ; Chromosome Aberrations ; Cytomegalovirus Infections ; blood ; drug therapy ; genetics ; Female ; Humans ; Pregnancy ; Pregnancy Complications, Infectious ; blood ; drug therapy ; genetics ; Young Adult
4.Study on a fatal pregnant woman died from by avian influenza (H5N1).
Qun LI ; Yu LAN ; Cui-ling XU ; Yan LIU ; Tong-sheng WU ; Le-ying WEN ; Ni-juan XIANG ; Ye ZHANG ; Jia-bing WU ; Jie DONG ; Chuan-long XIONG ; Xiao-ling XU ; Wan-fu HU ; Zhong-jie LI ; Dai-lin HU ; Lei ZHOU ; Ming-ying MA ; Zhi-tao LIU ; Xu-xiang LIU ; Li-ping LIU ; Jun WANG ; Shou-kui HU ; Jun HE ; Yong WANG ; Xian-xiang LI ; Fu-qing WU ; Yue-long SHU ; Mao-wu WANG ; Zi-jun WANG ; Wei-zhong YANG ; Yu WANG ; Hong-jie YU
Chinese Journal of Epidemiology 2006;27(4):288-292
OBJECTIVETo ascertain the causation of a pregnant woman with undefined pneumonia reported from the People's Hospital of Tongling city in Anhui province on November 2005.
METHODSEpidemiological and clinical information of the case was collected from the keypersons close to the case and referring to the medical record. A medical observation was carried out on the close contacts of the case and sick or dead poultry. Tracheal aspirates being collected were tested by both RT-PCR and real-time PCR to detect viral nucleic acids of A/H5N1, and were inoculated into special pathogen free (SPF) embryonated hens' eggs.
RESULTSThe pregnant woman was found to have been contacted with the sick/dead poultry directly on the 4th day before onset of illness. All the 122 close contacts were healthy after a 10-day medical observation. The major clinical features of the case were viral pneumonia with rapidly developed leukopenia and lymphopenia. The progress to acute respiratory distress syndrome and multiple organ dysfunction syndromes was found at clinical presentation. HA and NA gene of A/H5N1 virus were positive. The 8 gene fragments of A/Anhui/1/2005 (H5N1) isolated from the tracheal aspirates had not carried genes from a human virus through reassortment, and the receptor-binding site of the hemagglutinin was polybasic cleavage site.
CONCLUSIONThis was the first documented case of H5N1 infection in pregnant woman. The immunotolerant state of pregnancy might have predisposed to the fatal outcome of the patient.
Adult ; China ; Fatal Outcome ; Female ; Humans ; Influenza A Virus, H5N1 Subtype ; genetics ; isolation & purification ; Influenza, Human ; complications ; pathology ; Multiple Organ Failure ; Pneumonia ; virology ; Polymerase Chain Reaction ; Pregnancy ; Pregnancy Complications, Infectious ; virology ; Respiratory Distress Syndrome, Adult ; Trachea ; virology