Objective To explore the adverse effect of arsenic trioxide (As2O3) on liver,kidney and heart function during treating children patients with acute promyelocytic leukemia (APL) at therapeutic dose.Methods Sixty-five APL cases received As2O3 by intravenous drip and organic toxicity were selected as our subjects.The indices of liver,heart and kidney were measured.Results Of all subjects,19 cases(29.2％) occurred liver damage,including 15 cases(23.1％) mild and 4 cases(6.2％) moderate toxicity.The levels of alanine aminotransferase of patients before treatment was (19.9 ±9.5) U/L,and (24.3 ± 11.8) U/L,(25.0 ± 14.4) U/L at 1 st and 2nd weeks after treatment,higher than those before the treatment (P ＜ 0.05).However,level of alanine aminotransferase was back to normal at 3th weeks after treatment.Meanwhile the levels of aspartate aminotransferase at 1st,2nd and 3th weeks after treatment were (38.3 ± 16.5),(39.1 ± 15.5),(35.3 ± 20.6) U/L respectively,higher than that before treatment((28.5 ± 8.8) U/L,P ＜ 0.05 or 0.01),and it was back to normal at 4th weeks.(2) The levels of urinary cystatin C were (2.51 ± 1.45) mg/L,(3.05 ± 1.13) mg/L,(2.46 ± 1.21) mg/L at 2nd,3th,4th weeks after treatment,significantly higher than that before treatment ((1.98 ±0.68) mg/L,P ＜0.05 or 0.01).And the levels of urinary β2 microglobulin at 2nd,3th,4th weeks after treatment were significantly higher than that before treatment (P ＜0.05 or 0.01) and back to normal at 5 weeks after treatment.(3) Nine cases at remission stage showed the symptoms of palpitation,precordial discomfort and increased heart rate,and all those symptoms were mild.And the symptoms disappear at the 3th week after the treatment.Creatine kinase at the 2nd weeks after treatment was (90.2 ± 32.5) U/L,higher than that before treatment ((78.5 ± 22.3) U/L).The levels of creatine kinase isoenzyme at 2nd,3th weeks after treatment were (8.3 ± 4.8) U/L,(8.5 ± 5.6) U/L,higher than that before treatment ((6.3 ± 3.5) U/L).The serum creatine kinase mass at 4th weeks((3.9 ±2.0) g/L) was significantly higher than that before treatment ((2.8 ± 1.9) g/L),and then gradually be back to normal.Conclusion The routine dose As2O3 in treatment of APL children show less toxicity in liver,kidney,and heart Those adverse effects are transient,reversible and they occurred at 1-3 week after As2O3 treatment.Serum alanine aminotransferase,aspartate aminotransferase and urinary cystine protease inhibitors,β2 micro ring protein and serum creatine kinase MB mass might be served as sensitive indicators of organ damage.

Objective To investigate the role of Castleman′s disease in the pathogenesis of paraneoplastic pemphigus (PNP). Methods In six PNP patients associated with Castleman′s disease, routine immunohistochemistry was performed on tumor tissue. Reverse transcription - PCR, DNA sequencing of cloned PCR product and in situ hybridization (ISH) were used to estimate the clonality of the B-cells in the tumors. The expression of the specific tumor B-cell clones was evaluated by Northern blot. Six patients with Castleman′s disease without mucocutaneous lesion and 3 patients with reactive lymphadenopathy were used as the controls. Results Immunohistochemistry showed that CD20-positive B-cells in high density located in lymphoid follicles. The PCR produced one discrete band of about 128 bp in every paraneoplastic pemphigus patients. After sequencing the cloned PCR product, only two kinds of highly homologous sequences were found in all of the PNP patients. The 128 bp sequences were the major clones seen in all patients, and the 122 bp sequences were the relatively minor one seen in 4 patients. Anti-sense RNA probe transcribed from a clone of 128 bp was used in ISH. Signals of ISH located in cytoplasm of the cells in follicles of the tumors. Furthermore, this probe was also used for Northern blot and showed a strong signal in PNP patients. Conclusions Castleman′s disease associated with PNP share a major B-cell clone. The B-cell clone is expressed and maybe produces functional antibody initiating the mucocutaneous immune injury.

Objective To identify features of antibodies in the supernatants of cultured Castleman's disease cells.Methods Lymphocytes of Castleman's disease were isolated and cultured.Immunofluorescence and immunoblot assays were performed with IgG extracted from culture supernatants.The immunoglobulin heavy chaingene of cultured tumor B cells was analyzed by RT-PCR,cloning and sequencing.ResultsIg Gextracted from culture supernatant scouldattachtotheepithelialcellsurfacesofmousebladdertissues.Theantibodycouldalsoidentifytwoantigencomponents,210000and190000,ofnormalhumanepidermaltis-sues.ThesequencesimilaritywasfoundinimmunoglobulinheavychaingeneofculturedtumorBcellscom-paredwiththatof6patientswithCastleman'sdiseasepreviouslyreported.Conclusions Castleman's tumor associated with paraneoplastic pemphigus can secret autoantibody with similar features to that found in patients'sera.

Objective To analyze the pathogenesis,therapy and outcome of pediatric cases with coagulation disorders (CD).Methods All these 137 patients were diagnosed as CD with the methods of hemoglutination five items and/or disseminated intravascular coagulation indexes.Then activity of specific coagulation factors,morphology of bone marrow,hepatorenal function and some other relative tests were performed to find out the cause of CD or the primary disease.Results Forty-three cases were diagnosed as genetic CD with 29 as hemophilia A,4 as hemophilia B and 10 as Von Willebrand disease;while the other 94 patients as acquired CD with 15 as vitamin K-dependence coagulation factor deficiency,22 as hepatic dysfunction,30 as disseminated intravascular coagulation and 1 as thrombotic thrombocytopenic purpura.Genetic CD was treated with replacement therapy to reduce the complication.There was 1 case in this group died of intracranial hemorrhage.Acquired CD was treated with short-term,specific and necessary replacement therapy on the basis of reasonable treatment of primary diseases.Eleven cases died finally in this cohort with 7 cases as liver failure and the other 4 cases as terminal leukemia or lymphoma.Conclusion Pediatric patients with CD were caused by genetic or acquired diseases.In clinic the reason of CD was mainly acquired.The treatment of genetic CD is the replacement of specific coagulation factor for life-long term.The outcome dependes on the lack of degree.While the therapy for acquired CD aims at the primary disease.The principle of blood transfusion is short-term and the outcome dependes on the therapic effects of primary diseases.

Objective To study the renal toxicity of arsenic trioxide (As2O3) with therapeutic dose in acute promyelocytic leukemia (APL) in childhood. Methods Renal toxicity of 37 APL was monitored. The examinations of urinary routine, urinary microproteins[αt1-microglobulin (α1-MG), microalbumin (mAlb),β2-microglobulin (β2-MG), transferrin(TRF)] and renal function were performed. Results Five cases with leukocyturia, three cases with hematuria, six cases with proteinuria were observed before therapy. Ketonuria occurred in six cases associated with fever and less diet; overall abnormality disappeared in the first week. No significant changes of blood uric nitrigen(BUN), serum creatinine(Cr) and uric acid (UA) were founded in induction remission. Compared with tests before As2O3 infusion, obvious increase of uric α1-MG occurred in second week with arsenic trioxide, obvious increase of uric β2-MG in third week (P ＜0.01), slow recovery of uric α1-MG and β2-MG in fifth week. No significant changes of uric mAlb and TRF were seen in induction remission. Conclusion The renal toxicity of As2O3 was gentle in general therapeutic dose, renal tubercular damage could be seen. The important monitoring period were the second to fifth week in induction remission.Influence of As2O3 cumulant on renal function was not serious in the near future in childhood. The combined detection of urinary microproteins with dynamic variety could detect early renal damage with As2O3.

ObjectiveTo evaluate the clinical application of bidirectional Glenn shunt without extracorporeal circulation(ECC) on treatment of children with complicated congenital heart disease (CHD).MethodsForty-six patients with complicated CHD(without ECC group) underwent bidirectional Glenn shunt without ECC,and 40 patients with complicated CHD (with ECC group) underwent bidirectional Glenn shunt with ECC.The therapeutic effect was compared between two groups.ResultsThere was no operative mortality in two groups.The pulmonary artery pressure in without ECC group was significantly lower than that in with ECC group [( 16.7 ± 1.2) mm Hg ( 1 mm Hg =0.133 kPa) vs.( 18.9 ± 1.0) mm Hg,t =4.686,P=0.026 ].Duration of respirator assistance after operation in without ECC group was significantly lower than that in with ECC group [ ( 12.2 ± 2.7) h vs.( 19.2 ± 2.8) h,t =2.972,P =0.041 ].There was no significant difference in saturation of blood oxygen and the rate of chylothorax after operation between two groups (P＞0.05).The symptoms and signs including cyanosis and breath were markedly alleviative in all patients.ConclusionBidirectional Glenn shunt without ECC is an effective and safe method for complicated CHD.

Acute lymphoblastic leukemia（ALL）is the most common pediatric cancer and has good outcome.However，the prognosis of relapsed/refractory ALL in children is poor.Intensive reinduction chemotherapy and hematopoietic stem cell transplantation have become alternative treatments. Bortezomib，as the primary generation proteasome inhibitor，has been approved for the treatment of multiple myeloma and mantle cell lymphoma. In recent years，several clinical studies have confirmed promising responses and well tolerated of bortezomib combined with various chemotherapeutic drugs in treating children with relapsed/refractory ALL，to improve the complete remission rate and survival rate. This article reviews the mechanisms of bortezomib in ALL and the clinical study progress and safety of bortezomib combined with chemotherapy in children with relapsed/refractory ALL.

Pituitary abscess is a rare but potentially life-threatening disease. Headache, visual disturbance, and hypopituitarism are the most common presenting symptoms of pituitary abscess. On magnetic resonance imaging(MRI), pituitary abscess may present as a round sellar cystic lesion, hypo- or isointense on T1 imaging and hyper- or isointense on T2 imaging with peripheral gadolinium enhancement. Diagnosis usually is made during surgical exploration when pus is found in a cystic lesion. The mainstay of treatment is transsphenoidal surgical resection in combination with antibiotic therapy, although a craniotomy approach is warranted in select conditions. Here we report 3 cases of pituitary abscess in our hospital and review the literatures.