Biomedical subjects

Chun Chen

Publications and source records attributed to Chun Chen.

At least 19 recordsLinked to original sources

PubMed2026

Genetic Analysis of Genomic and Methylomic Variation and Identification of Multi-Trait Mutants in Rice Carried on Chang'e-5.

Global food security is facing challenges from population growth to diminishing arable land. Space mutation breeding holds promise for overcoming the variation limitations in conventional breeding; however, the mutagenic effects of the deep-space environment on rice and the transgenerational inheritance patterns of induced variations remain unclear. In this study, rice seeds carried by the Chang'e-5 spacecraft were used as materials. Whole-genome sequencing and whole-genome bisulfite sequencing were performed on the first (SP1) and second generations (SP2) of space-mutagenized plants after their return to Earth. The results showed that the number of genomic variants in the SP2 generation increased significantly compared with SP1, and SNPs, homozygous sites, and variants in coding regions were more heritable. The genome-wide methylation level was elevated in the SP2 generation, and among differentially methylated cytosines, those in the CG context exhibited the highest heritability. Furthermore, large-scale screening for nitrogen efficiency, tolerance to PEG-induced stress, and germination-stage cold resistant mutants was conducted in the SP2 generation, and phenotypic validation was performed in the third generation (SP3). By integrating multi-omics analyses of representative mutants to mine candidate genes, a number of heritable elite mutants were obtained, and seven candidate genes for key traits were identified. This study systematically elucidates the transgenerational inheritance patterns of deep-space-induced variation in rice. The multi-trait mutants obtained provide valuable germplasm resources for gene cloning and breeding applications in rice.

DNA methylation↗

PubMed2026

KIT and FLT3-ITD mutations do not predict outcomes in pediatric core-binding factor acute myeloid leukemia: findings from the C-HUANAN-AML-15 multicenter cohort study.

Although core-binding factor acute myeloid leukemia (CBF-AML) is generally considered a favorable-risk subtype in children, disease relapse remains a significant concern. The prognostic relevance of co-occurring mutations, particularly KIT and FLT3-ITD, remains debatable, and treatment intensity may modulate their impact. This multicenter analysis included 289 children (< 14 years) with newly diagnosed CBF-AML enrolled in the C-HUANAN-AML-15 study (2015-2023). KIT and FLT3-ITD mutations were identified via cytogenetic analysis and targeted sequencing. Measurable residual disease (MRD) was evaluated by multiparameter flow cytometry (MFC) and quantitative polymerase chain reaction (PCR) following induction chemotherapy. Survival analyses were performed using Kaplan-Meier and Cox regression methods. This multicenter analysis included 289 children (< 14 years) with newly diagnosed CBF-AML enrolled in the C-HUANAN-AML-15 study (2015-2023). KIT and FLT3-ITD mutations were identified via cytogenetic analysis and targeted sequencing. Measurable residual disease (MRD) was evaluated by multiparameter flow cytometry (MFC) and quantitative polymerase chain reaction (PCR) following induction chemotherapy. Survival analyses were performed using Kaplan-Meier and Cox regression methods. KIT mutations were detected in 103 patients (35.6%), predominantly involving exon 17 (69.9%), and were associated with extramedullary disease, sex chromosome loss, and trisomy 22. No significant differences in 5-year event-free survival (EFS), overall survival (OS), or cumulative incidence of relapse (CIR) were observed between patients with and without KIT mutations. FLT3-ITD mutations (5.5% of patients) did not adversely affect outcomes. Neither mutation independently predicted survival. MRD positivity (MFC-MRD ≥ 0.1%) after the second induction cycle strongly predicted inferior EFS and OS and higher CIR, with corresponding results observed for molecular MRD and parallel findings for PCR-based MRD. In this large multicenter cohort, KIT and FLT3-ITD mutations did not adversely affect the prognosis of pediatric CBF-AML treated according to the C-HUANAN-AML-15 protocol. MRD after induction was the most powerful predictor of relapse and survival, underscoring its importance for risk stratification in future pediatric AML trials.

Humans↗

PubMed2025

Effective detection of 148 cases chromosomal mosaicism by karyotyping, chromosomal microarray analysis and QF-PCR in 32,967 prenatal diagnoses.

BACKGROUND: Detection of mosaicism has always been difficult in prenatal diagnosis, which is to assess the value of karyotyping combined with three different molecular genetic tests for prenatal diagnosis. Retrospective review of chromosomal mosaicism (CM) was conducted in 32,967 pregnant women from January 2015 to December 2022. METHODS: A total of 148 fetuses diagnosed with chromosomal mosaicism by karyotyping with copy number variant sequencing (CNV-seq)/ chromosomal microarray analysis (CMA) and quantitative fluorescent polymerase chain reaction (QF-PCR) were selected, and the results from three the methods were compared and further analyzed. The χ2 test for multiple group rates was for the 5 clinical prenatal diagnostic indication groups was used to do multiple comparison tests for statistical analysis. Inconsistent results between methods were identified and further analyzed. RESULTS: A total of 148 CM cases was detected (0.45%, 148/32967), of which karyotyping was detected in combination with CMA in 73 cases (73/85), with CNV-seq in 5 cases (5/11), and with QF-PCR in 35 cases (35/52) and the mosaic conformity rates of the three methods compared with karyotyping were 85.9% (CMA), 67.3% (QF-PCR), and 45.5% (CNV-seq), respectively. There were 49 cases of autosomal mosaicism (49/148, 33.1%) and 99 cases of sex CM (99/148, 66.9%). There were 9 cases of small supernumerary marker chromosome (sSMC)with CMA detection clarified the origin of chromosome fragments. The non-invasive prenatal testing (NIPT) group and the ultrasound abnormality group had the highest detection rates, accounting for 35.1% and 22.3%. CONCLUSIONS: In chromosomal mosaicism, there are inconsistent results between different detection methods. Therefore, karyotyping combined with CMA/CNV-seq and FISH methods significantly improves the detection rate of chromosomal mosaicism and also confirms experimental data in the literature, which is of great value for prenatal diagnosis.

Humans↗

PubMed2008

Phenotypic and genetic diversity of rhizobia isolated from nodules of the legume genera Astragalus, Lespedeza and Hedysarum in northwestern China.

Twenty-nine rhizobial isolates from root nodules of the wild Legumes Astragalus, Lespedeza and Hedysarum growing in the northwestern region of China, were characterized by numerical taxonomy, RFLP and sequencing of PCR-amplified 16S rDNA genes, and cross-nodulation with selected Legume species. Based on the results from numerical taxonomy, the isolates could be divided into two main groups (Clusters 1 and 2) and some single isolates at 82% similarity. CLuster 1 contained six isolates from Astragalus, Lespedeza and Hedysarum spp. Cluster 2 consisted of nine isolates from Astragalus and Hedysarum species. The phytogenetic analysis based on 16S rRNA gene sequences showed that SH199, representing cluster 1, belonged to the Rhizobium-Agrobacterium group, and SH290B, representing cluster 2, was closely related to R. galegae and R. huautlense.

Astragalus Plant↗

PubMed2007

Hypoxia reoxygenation induces premature senescence in neonatal SD rat cardiomyocytes.

AIM: To investigate whether hypoxia reoxygenation induces premature senescence in neonatal Sprague-Dawley (SD) rat cardiomyocytes. METHODS: Cardiomyocytes were isolated from neonatal SD rat heart and identified by immunohistochemistry. The control cultures were incubated at 37 degree centigrade in a humidified atmosphere of 5% CO(2) and 95% air. The hypoxic cultures were incubated in a modular incubator chamber filled with 1% O(2), 5% CO(2), and balance N2 for 6 h. The reoxygenated cultures were subjected to 1% O(2) and 5% CO(2) for 6 h, then 21% oxygen for 4, 8, 12, 24, and 48 h, respectively. Cell proliferation was determined using bromodeoxyuridine labeling. The ultrastructure of cardiomyocytes was observed by using an electron microscope. beta-Galactosidase activity was determined by using a senescence beta-galactosidase Staining Kit. p16( INK4a ) and telomerase reverse transcriptase (TERT) mRNA levels were measured by real time quantitative PCR. TERT protein expression was determined by immunohistochemistry. Telomerase activities were assayed by using the Telo TAGGG Telomerase PCR ELISAplus kit. RESULTS: The initial cultures consisted of pure cardiomyocytes identified by immunohistochemistry. The proportion of BrdU positive cells was reduced significantly in the hypoxia reoxygenation-treated group (P< 0.01). Under the condition of hypoxia reoxygenation, mitochondrial dehydration appeared; p16( INK4a ) and TERT mRNA levels, beta-galactosidase activity, TERT protein expression and telomerase activities were all significantly increased (P< 0.01 or P< 0.05). CONCLUSION: These data indicate that premature senescence could be induced in neonatal SD rat cardiomyocytes exposed to hypoxia reoxygenation. Although TERT significantly increased, it could not block senescence.

Animals↗

PubMed2006

The Integrin alpha9beta1 contributes to granulopoiesis by enhancing granulocyte colony-stimulating factor receptor signaling.

The integrin alpha9beta1 is widely expressed on neutrophils, smooth muscle, hepatocytes, endothelia, and some epithelia. We now show that mice lacking this integrin have a dramatic defect in neutrophil development, with decreased numbers of granulocyte precursors in bone marrow and impaired differentiation of bone marrow cells into granulocytes. In response to granulocyte colony-stimulating factor (G-CSF), alpha9-deficient bone marrow cells or human bone marrow cells incubated with alpha9beta1-blocking antibody demonstrated decreased phosphorylation of signal transducer and activator of transcription 3 and extracellular signal-regulated protein kinase. These effects depended on the alpha9 subunit cytoplasmic domain, which was required for formation of a physical complex between alpha9beta1 and ligated G-CSF receptor. Integrin alpha9beta1 was required for granulopoiesis and played a permissive role in the G-CSF-signaling pathway, suggesting that this integrin could play an important role in disorders of granulocyte development and other conditions characterized by defective G-CSF signaling.

Animals↗

PubMed2006

Down-regulation of the expression of beta1,4-galactosyltransferase V promotes integrin beta1 maturation.

In previous study, we have shown that beta1,4-galactosyltransferase V (GalT V) functions as a positive growth regulator in glioma. Here, we reported that down-regulation of the expression of GalT V in SHG44 cells by transfection with antisense cDNA specifically up-regulated the expression of cell surface integrin beta1 without the change of its mRNA, and with integrin beta1 125 kDa mature form increased and 105 kDa precursor form decreased. It is well known that the N-glycans of integrins modulate the location and functions of integrins. The SHG44 cells transfected with antisense cDNA of GalT V demonstrated decreased Golgi localization of integrin beta1, strengthened the interaction between integrin alpha5 and beta1 subunit, and enhanced the adhesion ability to fibronectin and the level of focal adhesion kinase phosphorylation. Our results suggested that the down-regulation of the expression of GalT V could promote the expression of cell surface integrin beta1 and subsequently inhibit glioma malignant phenotype.

Astrocytoma↗

PubMed2006

Identification of human liver mitochondrial aldehyde dehydrogenase as a potential target for microcystin-LR.

Microcystins (MCs) are hepatotoxins produced by a variety of freshwater cyanobacteria. The toxicity of these hepatotoxins is a severe health issue for both humans and livestock; MCs have been implicated in the development of liver cancer, necrosis, and even deadly intrahepatic bleeding. Microcystin-LR (MC-LR) is the MC variant most commonly encountered in a contaminated aquatic system. Thus far, MC-LR has only been shown to target the serine/threonine protein phosphatases 1 and 2A (PP1 and PP2A) and it is still unknown whether MC-LR can bind and inhibit any other protein targets inside the cell. To find potential MC-LR targets, we screened a phage display library for peptide ligands that specifically recognize MC-LR. Using these peptide sequences as guides, we performed a series of bioinformatics analyses revealing that MC-LR binds human liver aldehyde dehydrogenase 2 (ALDH2) at residues 447-451. We confirmed MC-LR binding of ALDH2 via automated docking computation, which yielded results matching our experimental and bioinformatics analyses. ALDH2 dysfunction may lead to aldehyde-induced reactive oxygen species (ROS) generation and, in turn, apoptosis. Therefore, ALDH2 could potentially be a target of MC-LR associated with the process of ROS-induced apoptosis. Our current study presents a new approach to the study of interactions of biological molecules by combining phage display technology with computational methods.

Aldehyde Dehydrogenase↗

PubMed2006

Experimental simulation of transmission of an obligate aphid pathogen with aphid flight dispersal.

The wide dispersal of Entomophthorales-caused mycoses that usually regulate aphid populations is most likely to be associated with the flight of infected alates. This hypothesis was examined via simulated flight and postflight colonization of Myzus periscae alates exposed to spore showers of Pandora neoaphidis, a common obligate aphid pathogen prevalent world wide. A total number of 407 alates were showered in different batches, then individually flown in a computer-monitoring flight mill system and reared on cabbage leaves for 14-day free reproduction and contagious infection within progeny colonies at 20-23 degrees C. On average, 80.6% of them flew 2.6 km in 1-5 h, survived 3.2 days, produced 5.3 nymphs, and transmitted their infection to progeny successfully. However, 9.8% of the flown alates left no progeny although they survived at least 1 day prior to mycosis while the rest were not mycosed, producing significantly more nymphs during the first week. The flight distances of the infected (0.01-10.2 km) or uninfected alates (0.1-8.3 km) were exponentially correlated to the flight time (r( 2) >or= 0.98). When grouped by the flight ranges of <1.0, 1.0-3.0, 3.0-5.0 and >5.0 km, the number of live aphids and the proportion of mycosed individuals per progeny colony over colonization days fit well to a complex logistic model (r( 2) = 0.984) and modified Gompertz model (r( 2) = 0.978) respectively. Both models included flight distance, postflight survival time, premycosis fecundity and primary infection rate as independent variables to affect the developmental rates. The results highlight the significant role of infected alates in the wide dispersal of P. neoaphidis-caused mycoses among aphids.

Animals↗

PubMed2006

ADAM33 is not essential for growth and development and does not modulate allergic asthma in mice.

A disintegrin and metalloprotease 33 (ADAM33) is a transmembrane protease and integrin ligand that has been identified as an asthma susceptibility gene product. To determine whether ADAM33 plays important roles in mammalian development and the modulation of allergic airway dysfunction, we generated ADAM33-null mice by gene targeting. ADAM33-null mice were born at expected Mendelian ratios, and both male and females developed normally and were fertile. No anatomical or histological abnormalities were detected in any tissues. In an animal model of allergic asthma, ADAM33-null mice showed normal allergen-induced airway hyperreactivity, immunoglobulin E production, mucus metaplasia, and airway inflammation. Our results demonstrate that ADAM33 is not essential for growth or reproduction in the mouse and does not modulate baseline or allergen-induced airway responsiveness.

ADAM Proteins↗

PubMed2006

[Risk factors and treatment of hemorrhagic cystitis in children after hematopoietic stem cell transplantation].

OBJECTIVE: Hemorrhagic cystitis (HC) is one of the common complications of hematopoietic stem cell transplantation (HSCT), which causes significant pain, prolongs hospitalization, and occasionally results in renal failure and death. This study aimed at investigating the incidence, risk factors, and outcome of HC in children post umbilical cord blood transplantation (UCBT) and peripheral blood stem cell transplantation (PBSCT). METHODS: From October 1998 to June the clinical records of 53 pediatric patients (aged 2-18 years with median age of 7.5 years) in our HCST center who underwent UCBT (n = 37) and PBSCT (n = 16) were retrospectively analyzed. Thirty out of 53 patients were diagnosed as hereditary hemolytic anemia (56.6%), and the others as haematological malignancies (43.4%): of whom 8 had acute lymphoblastic leukemia, 12 acute myeloid leukemia, 2 chronic myeloid leukemia and 1 non-hodgkin lymphoma. Conditioning regimen varied according to disease and clinical status, however based on cyclophosphamide (CTX, 120-200 mg/kg) and busulphan (BU, 12-16 mg/kg) in the cohort. Total body irradiation (TBI) or total lymphoid irradiation was added in 7 patients respectively. The patients were divided into regular treatment group (RTG) with 15 cases who received hyperhydration, alkalinizing, diuresis and Mesna during CTX infusion and prostaglandin E1 (PGE1) group (PEG) with 38 cases who received hyperhydration, alkalinizing, diuresis and Mesna plus prostaglandin E1 (0.03 microg/kg.h). The risk factors of HC were examined by univariate and multivariate analysis. RESULTS: In all, 11 of the 53 transplanted patients developed HC (21%) with a median onset time of day +15 (rage day +2 - +25). HC was classified as early in 4 (36%) and late in 7 (64%), and scored as grade Iin 2 cases (18%), grade II in 4 (36%) and grade III in 5 (46%). There was no significant difference between RTG and PEG in the incidence of HC, however, the incidence was much higher in the group of patients who were > or = 6 years old, positive group of graft-versus-host disease (GVHD) and group of cytomegalovirus (CMV) infection than that in the group of patients who were < 6 years of age (32% vs. 8%, P < 0.05), negative group of GVHD (35% vs. 7%, P < 0.05) and CMV non-infected group (62% vs. 13%, P < 0.05), respectively. Furthermore, by multivariate analysis, > or = 6 years old (OR = 3.53, P < 0.05) and CMV infection (OR = 4.31, P < 0.05) were significant risk factors for HC. Three of 11 patients were treated with bladder irrigation. All patients recovered from HC in a median 12.8 days (range 2-53 days). CONCLUSION: Older age (> or = 6 years) as well as CMV infection were the risk factors of HC. Both hyperhydration and Mesna were effective in preventing HC, while addition of PGE1 could not reduce the incidence of HC. The prognosis of HC in children post HSCT was satisfactory.

Adolescent↗

PubMed2006

[Time distribution of ventricular arrhythmias in patients with Brugada syndrome].

OBJECTIVE: To study the characterization of time distribution of ventricular arrhythmias in patients with Brugada syndrome (BrS) using Holter monitoring and ICD follow-up. METHODS: Patients with BrS [all male, mean age (41.07 +/- 11.49) years], were divided into ventricular fibrillation (VF) group (n = 7) and no ventricular fibrillation (N-VF) group (n = 7). Premature ventricular capture (PVC) and VF episodes were detected by Holter monitoring and ICD recording. RESULTS: The 24 hours total number of PVCs ranged from 0 to 74 (mean 9.61 +/- 17.23) in most of the patients and were similar between VF group and N-VF group. The percentage of PVC episodes in VF group was significantly higher than that in N-VF group from nocturnal time to early morning (22:00 to 7:00, 98.67% vs. 44.14%, P < 0.01). There were total 75 VF episodes during (23.18 +/- 17.96) months' follow-up in 5 patients with BrS, 93.3% of which occurred from nocturnal time to early morning (22:00 to 7:00). CONCLUSIONS: The episodes of PVC were enriched from nocturnal time to early morning in BrS patients, this time distribution could be a new noninvasive risk stratification factor for BrS. The episodes of VF in BrS patients were also enriched from nocturnal time to early morning and this time characteristic of episodes of VF could be used to guide drug therapy.

Adult↗

PubMed2005

Narrow QRS tachycardia with ventriculoatrial dissociation mediated by a left fasciculoventricular fiber.

A 30-year-old man presented with narrow QRS tachycardia. The intracardiac electrocardiogram showed an atrial-HIS (AH) interval of 75 msec and a HIS-ventricular (HV) interval of 44 msec during baseline. Atrial incremental pacing revealed HV shortening, with apparent incomplete right bundle branch block (RBBB) morphology without QRS complex axis deviation. The induced tachycardia exhibited several QRS morphologies: a narrow QRS, complete RBBB and complete left bundle branch block (LBBB) morphology. Spontaneous conversion of the QRS pattern from wide to narrow was observed. The cycle length of the tachycardia was significantly shortened (from 316 to 272 ms) from LBBB morphology to narrow QRS complex. The atrial activation was dissociated from the ventricular activation during all tachycardias. Each QRS complex during tachycardia was preceded by a HIS deflection and HV interval was 35 ms, which was shorter than that of sinus rhythm. HIS deflection was earlier than right bundle potential during all kinds of tachycardia. This tachycardia is most likely mediated by a left fasciculoventricular fiber which connects the HIS bundle below the atrioventricular node to the myocardial tissue of the left ventricle. The HIS-Purkinje system is used as an antegrade conduction limb and the fasciculoventricular fiber as a retrograde limb in the tachycardia circuit.

Adult↗

PubMed2005

Non-contact mapping and linear ablation of the left posterior fascicle during sinus rhythm in the treatment of idiopathic left ventricular tachycardia.

AIM: The reentry circuit of idiopathic left ventricular tachycardia (ILVT) has been demonstrated to be confined to the left posterior Purkinje network. We hypothesized that mapping and linear ablation of the left posterior fascicle (LPF) during sinus rhythm guided by non-contact mapping can effectively modify the arrhythmogenic substrate in patients with ILVT and abolish the tachycardia. METHODS: Six patients with ILVT, consisting of one case in which conventional mapping failed three times, one recurrent case, one non-inducible case and three common cases, were included in the study. After a three-dimensional endocardial geometry of the left ventricle (LV) was created, the conduction system in the LV was mapped during sinus rhythm using a filter setting of 8 Hz. The His bundle area, left bundle branch, fascicles and sinus breakout point (SBO) were mapped in detail and tagged as special landmarks in the geometry. A linear lesion was placed perpendicular to the wave front propagation direction of the LPF, 1cm above the SBO. There was a small Purkinje potential preceding the ventricular activation at its starting and ending point. RESULTS: The mean tachycardia cycle length of ILVT in this study was 340.3+/-51.4ms. After a mean of 5.5+/-1.6 radiofrequency deliveries, the clinical tachycardias could not be induced and the 12-lead surface ECG showed right QRS axis deviation (mean 39.7+/-26.0 degrees) in all patients. The total procedure time was 160.0+/-32.2 min with fluoroscopic time of 26.0+/-6.8 min. No ILVT was inducible during control stimulation, and none recurred during a mean follow-up of 13.0+/-4.8 months. CONCLUSION: Mapping and linear ablation of the Purkinje network in LPF area guided by non-contact mapping is an effective and safe treatment of ILVT with radiofrequency energy, especially for those ILVTs which were unsuccessfully treated by conventional means or were non-inducible or non-sustained during the procedure.

Adolescent↗

PubMed2005

Epizootiological modeling of Pandora neoaphidis mycosis transmission in Myzus persicae colonies initiated by primarily infected alates.

Pandora neoaphidis transmission was monitored within progeny colonies initiated by infected Myzus periscae alates individually flown for 1 to 5 h. Mycosis progress in the colonies was well fitted (r2= 0.97) to a modified logistic or Gompertz model that included their flight distance, postflight survival time, premycosis fecundity, and primary infection rate as influential variables.

Animals↗

PubMed2005

[Study on lysosomes degradation of ricin A chain].

OBJECTIVE: To study lysosomes involvement in the degradation of ricin A chain. METHODS: A lysosome-targeted singal KFERQ was added to the C terminus of rRTA by DNA recombinant technology. A pKK223.3 expression system in E. coli was used to produce recombinant ricine A chain (rRTA) and rRTA-KFERQ. Recombinant proteins were purified by affinity chromatography using Blue-Sepharose 6B. The cytotoxicity of recombinant proteins was measured by the MTT method. RESULTS: Recombinant RTA-KFERQ was 49.87%, 54.18% and 88.68% less cytotoxic than RTA itself on the three cell lines HEPG2, Hela and A549, respectively. CONCLUSION: Lysosomes can degrade, but not completely inactivate RTA in different cells, suggesting cells may have other degradation pathways for RTA.

Chromatography, Affinity↗

PubMed2005

[Prophylaxis and treatment of chronic graft versus host disease].

OBJECTIVE: Chronic graft versus host disease (cGVHD) is the most common late complication after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and it represents the major cause of mortality in long-term survivors. Over the past decade, although conventional therapy has achieved complete responses in approximately 50% of patients, the prophylaxis and treatment of cGVHD are still not satisfactory. In the late years, utilization of new immunosuppressant such as tacrolimus (FK506), mycophenolate mofetil (MMF) on cGVHD improved the curative effects. This study tried to analyze the results of combination of methylprednisolone (MP), MMF and FK506 or cyclosporine A (CSA) as immunosuppressive therapies for cGVHD and to explore the effective regimen for children. METHODS: Forty-five patients received allo-HSCT. Among them 32 received UCBT and 13 received PBSCT. The conditional regimen mainly consisted of busalphan, cyclophosphamide, antihuman thymocyte globulin, fludarabin, melphalan, thiotepa and total lymph node irradiation. Prophylaxis of GVHD consisted of CSA, MP and MMF. Patients with cGVHD received a regimen with combination of MP, MMF and FK506 or CSA. RESULTS: Seventeen out of 32 patients who received UCBT were engrafted. while 9 out of 13 patients who received PBSCT were engrafted. Nine cases of the 30 engrafted patients developed cGVHD (morbidity 30%). Among the 17 patients who received UCBT, 3 developed cGVHD (18%). Among the 13 patients who received PBSCT, 6 developed cGVHD (46%). Six cGVHD continued from aGVHD (6/9). One patient was given CSA plus MMF, and 8 were given three-drug regimen with MP, MMF and FK506. The overall response rate was 100%. Two patients died of CMV-IP or septicemia (mortality 20%). Seven (78%) patients survived (event free survival, EFS) longer than 3 years. The side effects included hepatotoxicity, nephrotoxicity, hypertension, articular capsulitis and arrhythmia. The main complication and the major causes of death were infection. CONCLUSION: The incidence of cGVHD is low in children. The incidence of cGVHD after PBSCT is higher than that after UCBT. aGVHD is a highly dangerous factor. Combined therapy of MP plus MMF and FK506 or CSA is safe and effective for the treatment of cGVHD in children.

Child↗

PubMed2005

[The electrophysiological study and implantable cardioverter defibrillator therapy for the patients with Brugada syndrome].

OBJECTIVE: Clinical observation of electrophysiological study and implantable cardioverter defibrillator (ICD) therapy in patients with Brugada syndrome. METHODS: Ten patients (all male) with Brugada wave (spontaneous or propafenone test positive in ECG) underwent electrophysiological study (EPS). The mean age was (41 +/- 10) years. They had no structural heart disease with echocardiogram and the angiogram work-up. The ICD implanted in the patients with EPS-induced ventricular fibrillation in those who were available. RESULTS: Three patients had the history of familial sudden cardiac death (SCD). Four patients had repeated syncope episodes, two of them had documented ventricular fibrillation during syncope episodes. The AH and HV intervals were 50 - 124 (86 +/- 21) ms and 41 - 84 (58 +/- 15) ms. The ventricular fibrillation was induced in four patients with syncope and atrioventricular reentry tachycardia in one patient with palpitation. Three patients had spontaneous or inducible atrial fibrillation. The ICD implanted in three patients with inducible ventricular fibrillation. Due to economic issue, one patient without ICD implantation had got SCD during follow-up. The patient with atrioventricular reentry tachycardia underwent a successful left atrioventricular accessory pathway ablation. CONCLUSION: The Brugada patients with syncope and high rate of inducible ventricular fibrillation in EPS are the high risk population for SCD, in whom ICD should implant promptly to prevent SCD.

Adult↗