Adolescent ; Bone Marrow Transplantation ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Thalassemia ; surgery ; Transplantation Chimera

Autoimmune Lymphoproliferative Syndrome ; diagnosis ; genetics ; immunology ; therapy ; Biomarkers ; blood ; Child, Preschool ; Humans ; Male ; Mutation ; Prednisolone ; administration & dosage ; therapeutic use ; Ultrasonography, Doppler, Color ; fas Receptor ; genetics

Female ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Periodontitis ; complications ; physiopathology ; Pregnancy ; Pregnancy Complications, Infectious ; physiopathology ; Pregnancy Outcome

Objective To explore the clinical characteristic of rapidly progressive glomerulonephritis(RPGN) induced by the antithyroid agents[propylthiouracil(PTU) and methimazole(MMI)].Methods The analysis was made according to the drug histories,clinical manifestation,serology investigations,renal biopsy in 2 children.Results Two cases presented with gross hematuria,proteinuria and renal dysfunction.One case with antineutrophil cytoplasmic antibody(ANCA)-positive and vasculitis lesion with cresent in the renal biopsy;another presented with mesengial proliferation and cresent.They both had been diagnosed RPGN induced by antithyroid agents for 5 years because of Graves disease.The treatment included that PTU and MMI were withdrawn and then combined prednisone with mycophenolate mofetil(MMF)were applied.One year after treatment,the urine analysis and renal function returned to normal.Conclusions The antithyroid agents may induce RPGN.A early diagnosis and immunosuppressive therapy can improve its prognosis of the disease.

Objective To explore the incidence,clinical status,risk factors and outcomes of invasive fungal infections(IFIs)after allogeneic hematopoietic stem cell transplantation (Allo-HSCT) in pediatric patients.Methods Forty-one Patients who were underwent Allo-HSCT were selected from 2005 to 2006. Of 41 patients, 24 were boys and 17 were girls,aged 2-13 years old. Twenty-six cases with ?-thalassemia, 1 case with adrenoleukodystrophy,and the left 14 cases with other hematologic disorders.Twenty patients underwent bone marrow transplantation,19 patients underwent peripheral blood stem cell transplantation,2 patients underwent bone marrow transplantation and cord blood transplantation.Fourteen patients received Allo-HSCT from HLA-matched sibling donors or HLA mis-matched parents, 27 patients received Allo-HSCT from unrelated donors. Based on different types of transplant, patients were conditioned with busulfan, cyclophosphamide and Anti-thymocyte immune globulin. Fludalabine, total body irration, thiotepa or melphalan was used additionly in some cases. Cyclosporine A and mycophnolate mofetil were used as prophylaxis of graft versus host disease (GVHD).Results IFIs was observed in 5 cases(5/41 cases,12.2%),this comprised cases of proven,probable and possible IFIs at rates of 2.4%,4.9%,4.9%.The time of IFIs was 9-120 d after transplantation,the majority of IFIs in 3/5 cases(60%)children occurred within the first month.The difference of IFIs between patients who recived high-dose corticosteroid and those with no or conventional-dose corticosteroid was significant(?2=8.201 P=0.004);Regarding conditioning regimens,the IFIs of patients who with Thiotepa (TT) was significanthy higher than that of compared with those without TT(?2=9.549 P=0.002).The total effective rate was 40%.The effective rates of the patients with confimed diagnosis,cli-nical diagnosis,and with recommended diagnosis respectively were 100%,0 and 50% respectively.Conclusions IFIs is an important complication after Allo-HSCT,and the high-dose corticosteroid therapy and conditioning regimens with TT are the risky factors for IFIs.Aspergillus is the main pathogen bacteria.

Objective To investigate the clinical features,treatment response and prognosis in children with Takayasu′s arteritis(TA) in order to improve the understanding of TA.Methods A retrospective study of 10 children with TA was performed.All of them were admitted and diagnosed in Peking University First Hospital from Jan.1998 to Oct.2008.The clinical features,laboratory tests,imaging modalities,treatment response and prognosis were all collected and evaluated.Results There were 3 boys and 7 girls in the 10 patients with TA,and the ratio of male to female was 12.3.The onset was from 4 months to 9 years old,with average age at 5.5 years old.The average duration of diagnosis was 7.6 months.The incidences of hypertension,vascular bruits,albuminuria,convulsion were present in 100%,100%,70% and 40%,respectively.The clinical types included typeⅡ(60%),type Ⅲ(10%) and type Ⅳ(30%).The acute phase inflammatory indices of activity such as erythrocyte sedimentation rate(ESR),C-reactive protein(CRP) and white blood cell(WBC) were not evidently increased.Tuberculosis infection was found in 6 out of 10 patients and anti-tuberculosis treatment was performed.Six patients were treated with steroids and 3 cases of them were also given immunosuppressives cyclophosphamide or methotrexate.Three of the 10 patients received anti-hypertensive and vasodilator.Two patients received percutaneous translurminal angioplasty and 1 patient received nephrectomy.One patient died of renal failure,heart failure and shock.Conclusions The patients with TA had high prevalence of tuberculosis infection,diagnosis as often late because of lack of specific clinical features at the acute inflammatory period.When organic ischaemia occurred,treatment response was usually unsatisfactory.Patients with multi-systemic and multi-viscera lesions should have comprehensive examination,especially for those with hypertension,pulseless and vascular bruits,in order to rule out TA.Early ultrasonography,computed tomography and magnetic resonnance image methods are valued in eariler diagnosis and they are the key factors to improve prognosis.

Child, Preschool ; Epilepsy, Rolandic ; diagnosis ; drug therapy ; physiopathology ; Humans ; Male ; Prognosis ; Steroids ; therapeutic use ; Treatment Outcome

Objective To investigate the changes and prognostic significance of bone marrow(BM) oil drop and megakaryocyte counts after chemotherapy in acute myeloid leukemia (AML) patients (non-M3).Methods Ninty-nine adult patients with denovo AML (non-M3) were retrospectively analyzed to evaluate the change of BM oil drop and megakaryocyte counts and their influences on overall survival(OS) and disease free survival (DFS) during all stages of standardized therapy.Results The median DFS and OS were 21 (2-88);months and 70 (4-89) months,respectively; and 3-year predicted DFS and OS were 47.3 ％ and 55.8 ％,respectively.After AML patients (non-M3) achieving complete remission (CR) by induction therapy,BM oil drop tended to increase along with postremission chemotherapy cycle accumulation, while megakaryocyte counts tended to decrease.The univariate analysis indicated that megakaryocyte counts decreased after the second course of postremission therapy. BM oil drop increased after the first to the third course of postremission therapy.Grade of myelofibrosis in BM biopsy,serum lactate dehydrogenase (LDH) level at diagnosis,flow cytometric immunophenotyping, the percentage of BM blast cells at diagnosis and the percentage of residual leukemic cells (RLC) during aplasia (7-10 days after the end of induction therapy) had prognostic significance.Multivariable COX analysis indicated the percentage of BM blast cells at diagnosis and change of BM oil drop after the third postremission therapy were independent prognostic factors for DFS (P =0.010,0.018 respectively),and RLCs during aplasia and change rate of the megakaryocyte counts after the second postremission therapy were independent prognostic factors for OS (P =0.009, 0.038respectively).Conclusion After AML patients (non-M3) achieving CR by induction therapy,BM oil drop tends to increase along with postremission chemotherapy cycles accumulation,while the megakaryocyte counts tend to decrease.Dynamic observations of bone marrow oil drop and megakaryocyte counts are helpful for assessing the prognosis of acute myeloid leukemia (non-M3).

Objective To improve the acknowledge of diagnosis and therapy of aggressive systemic mastocytosis (ASM).Methods One ASM patient was reported and the literatures were reviewed.Results As a rare subtype of SM,ASM is characterized by multiple organs involvement,and often accompanied by bone marrow dysfunction,osteolytic lesions and palpable hepatomegaly or splenomegaly which usually indicate the high mast cell burden.Conclusion ASM meets criteria for SM and has one or more C findings.Variable factors affect the prognosis of ASM patients and the formulation of the clinical treatment strategy which leads to the highly individualized therapies.

The total RNA was extracted from ginseng leaves of Panax ginseng. The Cu/Zn-SOD gene was amplified via RT-PCR and the pET-28(a)-Cu/Zn-SOD expression vector was constructed. The pET-28 (a)-Cu/Zn-SOD recombinant plasmid was transformed into Escherichia coli BL21 (DE3) competent cells and was induced by IPTG in order to select optimal induction of expression conditions. The target protein was purified by the nickel ions (Ni ) affinity chromatography and the target protein enzyme activity was determinated by the xanthine oxidase method. The similarity of the Cu/Zn-SOD gene sequences and the Cu/Zn-SOD gene sequences of Korean ginseng in NCBI was 99. 00%. The target protein expression level was about 44.42%, and the molecular weight was 16.30 kDa after the pET-28(a)-Cu/Zn-SOD recombinants were induced by IPTG. The purified Cu/Zn-SOD protease activity reached 10,596.69 U x mg(-1). The P. ginseng pET-28(a)-Cu/Zn-SOD prokaryotic expression vector was built by the method of molecular biology, which provided the foundation for studying the Cu/Zn-SOD biology function.
Cloning, Molecular ; Escherichia coli ; genetics ; Gene Expression ; Genetic Engineering ; methods ; Genetic Vectors ; genetics ; Panax ; enzymology ; genetics ; Sequence Analysis ; Superoxide Dismutase ; genetics ; isolation & purification ; metabolism