Congenital central hypoventilation syndrome,also known as “Ondine′s curse”,is charac-terized by hypoventilation during sleep and impaired ventilatory responses to hypercapnia and hypoxemia. This disorder is associated with a malfunction of the nerves that control involuntary body functions and abnor-mal development of early embryonic cells that form the spinal cord. This review summarized the latest pro-gression in the pathogenesis,clinical symptoms,diagnosis,treatment and prognosis.

Neonatal fungal infections include superficial skin infections and deep fungal infections.Fungal infection is more common during the neonatal period,which is related with newborns(especially premature children),low birth weight and immune dysfunction.In addition,long-term application of broad-spectrum antibiotics and adrenal corticosteroids also induce fungal infections.Severe or disseminated fungal infection can lead to serious illness and even death.Neonatal fungal infections usually show no special clinical manifestations,and can thus result in the delayed diagnosis and treatment.This article discussed the pathogenic species of fungal infection and the risk factors.

were measured at the end of inspiratory.ResultsThe differences of following parameters were statistically significant between the healthy control group, mild PH group, moderate PH group and severe PH group: velocity time integral of ventricular systolic wave (SVTI), velocity time integral of ventricular diastolic wave (DVTI), AR, AR/S, ARVTI/SVTI, AR/(S+D), ARVTI/(SVTI+DVTI,F=2.940, 2.838, 3.373, 7.681, 3.478, 12.052, 4.806;P<0.05,<0.05,<0.05,<0.01,<0.05,<0.01,<0.01). Correlation analysis showed that ratio of AR/(S+D) was positively correlated with pulmonary artery mean pressure (r= 0.575,P<0.01). ConclusionsThe ratio of AR/(S+D) of MHV Doppler flow spectra was correlated with pulmonary artery mean pressure. The ratio increased along with the increasing of pulmonary pressure in CHD patient. The Doppler echocardiographic detection of MHV flow had a certain reference value for the diagnosis of PH. The ratio of AR/(S+D) could be a new non-invasive parameter for evaluating PH.

Objective To investigate the effects of urotensin Ⅱ/urotensin Ⅱreceptor ( UⅡ/UT) system on the expression of inflammatory signal molecules p 38 mitogen-activated protein kinase ( p38 MAPK) and nuclear factor-κB ( NF-κB ) in lipopolysaccharide ( LPS )-stimulated Kupffer cells ( KCs ) . Methods Rat KCs were isolated and purified by means of in situ perfusion and density gradient centrifuga-tion.The isolated cells were randomly divided into six treatment groups including group 1:UⅡ(-) urantide (-)LPS(-), group 2:UⅡ(+)urantide(-)LPS(-), group 3: UⅡ(-)urantide(+)LPS(-), group 4:UⅡ(-)urantide(-)LPS(+), group 5:UⅡ(+) urantide(-) LPS(+) and group 6:UⅡ(-)urantide(+) LPS(+) .Western blot assay was performed to detect p 38 MAPK/p-p38 MAPK protein and NF-κB p65 sub-unit.The DNA-binding activity of NF-κB was tested by electrophoretic mobility shift assay (EMSA).Re-sults There was no significant difference with the expression of p 38 MAPK protein in KCs among the six groups (P>0.05).The expression of p65 protein and p-p38 MAPK and the DNA-binding activity of NF-κB were significantly enhanced in LPS-stimulated KCs from groups 4, 5 and 6 in comparison with those in group 1 (P<0.01).No significant differences with the levels of p65 protein and phosphor-p38 MAPK and the DNA-binding activity of NF-κB were observed between UⅡ/urantide-treated cells ( group 2 or group 3) and untreated cells (group 1) (all P>0.05), but that were decreased in group 6 than those in group 4 (all P<0.01).Conclusion UⅡ/UT system participated in the activation of p38 MAPK and NF-κB signaling pathways in LPS-stimulated primary Kupffer cells .

Due to the improvement of infant survival rates,Candidas have been proved to be the third most common pathogen of late-onset sepsis in NICU,and invasive fungal infection of high-risk infants is in-creasing attention. As the diagnosis is difficult,treatment is often delayed,high mortality and severe disability are also caused,it's becoming a research hot spot to assess whether antifungal prophylaxis is beneficial. Now a number of studies have been performed to discuss the prophylactic role of fluconazole and nystatin,but has not yet reached a consensus. This review described the influence of chemoprophylaxis on fungal infection, colonization and drug resistance.

Objective To evaluate the clinical outcome of autologous hemopoietic stem cell transplantation (AHSCT) for hematological malignancies. Methods Data of 61 patients with hematological malignancies who underwent AHSCT in Xiangya Hospital from April 1994 to August 2008 were retrospectively analyzed. There were 30 acute non-lymphoblastic leukemias (ANLL), 25 non-Hodgkin lymphoma (NHL), 3 Hodgkin lymphoma (HL), and 3 plasmacytoma. Mel 160 mg/m2 + Ara-C 2.0/2.5 g × 2 +Cy 1.8 g/m2 × 2, or TBI 8-10 Gy + Cy 1.8 g/m2 × 2 were mainly included in pretreatment regimens. Results All patients had rapid hemopoietic reconstitution. There was one patient who died of heart failure during the transplantation process. The rate of AHSCT related death was 1.6 %. The median follow up duration was 52(2-211) months. Forty-seven of 61 patients were still alive during the analysis. The probabilities of disease free survival (DFS) at 5 years were significantly different between these two groups: (77.5±5.5) % for AHSCT groups and (31.6±7.3) % for synchronous intensive chemotherapy groups(P ＜0.01). Conclusion AHSCT can be safely performed as an important treatment constituent for hematological malignancies.

OBJECTIVE: To explore the clinical and pathologic features of angioimmunoblastic T-cell lymphoma(AITL) and provide evidence for diagnosis. METHODS: Eighteen AITL patients (9 males and 9 females aged from 14 to 70 years) were retrospectively analyzed in Xiangya Hospital of Central South University from July 2002 to September 2007. RESULTS: Characteristic features at the presentation of AITL included generalized lymphadenopathy, fever, splenomegaly, and skin rashes with polyclonal hyper-gammaglobulinemia and other hematological abnormalities (such as Coombs-positive hemolytic anemia), which often involved the bone marrow and had well-described histologic features. The positive rate for CXCL13 was 93.3%. CONCLUSION Repeated lymphadenbiopsy is helpful for AITL diagnosis. Routine histological and immunohistochemical examinations (especially including CXCL13) play significant role in the diagnosis and differential diagnosis of AITL.
Adolescent ; Adult ; Aged ; Chemokine CXCL13 ; metabolism ; Female ; Humans ; Immunoblastic Lymphadenopathy ; diagnosis ; metabolism ; pathology ; Lymphoma, T-Cell, Peripheral ; diagnosis ; metabolism ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Young Adult

OBJECTIVE: To explore the effect of trichostatin A (TSA) alone and combination with imatinib on the proliferation and apoptosis of K562R cells. METHODS: 3-(4,5-dimethylthiazol-2-yl) -5(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium(MTS) assay was used to observe the proliferation of K562R cells and apoptosis was analyzed by annexin-V/propidium iodide(PI) staining. RESULTS: After exposure to TSA, the proliferation of K562R cells was inhibited, and the effect was in both time- and dose-dependent manner. The apoptosis was induced. Combined with imatinib, the effect of proliferation inhibition was better. CONCLUSION TSA combined with imatinib can inhibit the proliferation of K562R cells and induce its apopotosis. TSA may become a new drug for imatinib-resistant patients.
Antineoplastic Agents ; pharmacology ; Apoptosis ; drug effects ; Benzamides ; Cell Proliferation ; drug effects ; Drug Synergism ; Humans ; Hydroxamic Acids ; pharmacology ; Imatinib Mesylate ; K562 Cells ; Piperazines ; pharmacology ; Protein-Tyrosine Kinases ; antagonists & inhibitors ; Pyrimidines ; pharmacology

Objective To explore the relationship between the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase p22phox subunit C242T gene polymorphism and large artery atherosclerotic cerebral infaretion(LAACI) among Han population in Songjiang district of Shanghai city.Methods The polymerase chain reaction-restrictive fragment length polymorphism analysis and gene sequencing techniques were adopted to conduct the gene type and allele frequency detection of C242T gene polymorphism loci in 310 Han patients with LAACI and 330 healthy individuals as control group in Shanghai Songjiang area.Then the correlation between C242T gene polymorphism and LAACI was analyzed.Results The level of MDA and the activity of NADPH oxidase in the LAACI group were significantly higher than those in the control group (P＜0.05).The smoking rate,alcohol drinking rate,hypertension rate,systolic pressure,diastolic pressure,and levels of blood glucose,TC,TG,LDL,ox-HDL and ox-LDL in the LAACI group were higher than those in the control group (P＜0.05),while the HDL level in LAACI group was lower than that in the control group (P＜0.05).The frequencies of CT+TT genetype and T allelic gene in the LAACI group were much higher than those in the control group (P＜0.05);the Logistic regression analysis found that smoking,hypertension,systolic pressure,diastolic pressure,blood glucose level,Lp (a),ox-HDL level,MDA level,NADPH oxiolase activity and NADPH C242T genotype were independent risk factors for LAACI.Conclusion NADPH oxidase P22phox C242T gene polymorphism has a correlation with LAACI and is another independent risk factor for LAACI except for hypertension,smoking and lipid oxidation.

OBJECTIVE: To study the efficacy of allogeneic hemotopoietic stem cell transplantation (allo-HSCT) for hematological malignancy. METHODS: A total of 104 patients with hematological malignancy, who underwent allo-HSCT in Xiangya Hospital from December 1999 to January 2010, were retrospectively analyzed. Of the patients, the transplantation related mortality (TRM), relapse rate (RR), 5-year overall survival (OS) and disease free survival (DFS) were estimated by Kaplan-Meier analysis. The unfavorable prognostic factors were also statistically examined. RESULTS: Hematopoietic reconstitution was achieved in 101 patients. At the last data of follow-up, the incidences of severe acute graft versus host disease (aGVHD) and extensive chronic GVHD were 15.38% and 25.53%, and the TRM and RR were 15.66% and 21.76%, respectively. The estimated 5-year OS and DFS for all patients were (73.49±4.59)% and (63.10±5.32)%, respectively. Those for acute myeloid leukemia (AML) patients were (63.00±9.51)% and (49.30±9.96)%, and those for chronic myeloid leukemia (CML) patients were (83.87±5.06)% and (74.55±6.79)%, respectively. The survival analysis suggested the poor prognostic factors for allo-HSCT recipients including female sex, severe aGVHD and refractory hematological malignancy. Further multivariate analyses revealed that severe aGVHD and refractory hematological malignancy were the independent risk factors of poor prognosis for the recipients (P<0.05). The 5-year DFS of severe aGVHD and refractory hematological malignancy patients was (48.22±12.69)% and (42.09±12.31)%, respectively. The TRM of severe aGVHD, HLA-mismatched graft and unrelated donor transplant was significantly higher than that of the corresponding control groups (57.14% vs. 4.81%, 33.33% vs. 10.41%, 26.09% vs. 9.28%; P<0.05). The RR of refractory hematological malignancy was significantly higher than that of the control group (41.09% vs. 15.63%, P<0.05). CONCLUSION The treatment of allo-HSCT can improve the disease free survival of patients with hematological malignany and is an important therapeutic method for hematological malignancy. Severe aGVHD and refractory hematological malignancy are the independent risk factors of poor prognosis for the allo-HSCT recipients with hematological malignancy.
Adolescent ; Adult ; Child ; China ; epidemiology ; Female ; Graft vs Host Disease ; epidemiology ; Hematologic Neoplasms ; therapy ; Hematopoietic Stem Cell Transplantation ; methods ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; therapy ; Leukemia, Myeloid, Acute ; therapy ; Male ; Middle Aged ; Retrospective Studies ; Transplantation, Homologous ; Young Adult