OBJECTIVETo explore the clinical features, diagnosis and treatment of pediatric myelodysplastic syndrome (MDS).

METHODSTwenty-eight children with MDS between January 2006 and March 2012 were enrolled in the study. Clinical symptoms, signs, laboratory examinations, treatment and outcomes were retrospectively studied.

RESULTSAnemia (96%), bleeding (68%), fever (68%) and hepatosplenomegaly (61%) were main clinical manifestaions in the 28 patients. Three cases (11%) converted into acute monocytic leukemia (M5), erythroleukemia (M6) or acute megakaryocytic leukemia (M7) one to two months later. Bone marrow proliferation mainly demonstrated as active or obviously active. One or two lineages of hematopoietic dysplasia were mostly observed in all 28 cases and obvious iron metabolism disorders were found in these patients. Cytogenetic abnormalities were detected in 45% of the 28 cases, most of which were numeral chromosome abnormalities. T cell, B cell and NK cell numbers decreased, Th cell numbers decreased, Ts cell numbers increased and Th /Ts inversed. Eight cases gave up treatment when confirmed. Of the 8 cases receiving symptomatic and supportive treatment alone, one was lost, one showed disease stability, and the remaining 6 cases showed disease progression. One patient who underwent induced differentiation and one who received hematopoietic therapy showed disease progression. Ten patients underwent chemotherapy. Two cases had no bone marrow remission after single agent chemotherapy. Of the 8 cases who underwent multi-drug combination chemotherapy, 4 cases achieved partial or complete remission of bone marrow.

CONCLUSIONSPediatric MDS is characterized by a lack of typical clinical manifestations, and a high rate of conversion to leukemia. Bone marrow proliferation is mainly active in children with MDS. One or two lineages of hematopoietic dysplasia is common. Among the cytogenetic abnormalities, numeral chromosome abnormalities are common. Obvious iron metabolism disorders and abnormal cellular immunity are found in children with MDS. Multi-drug combination chemotherapy appears to slow the course of the disease.

Adolescent ; Bone Marrow Examination ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Myelodysplastic Syndromes ; blood ; classification ; diagnosis ; therapy

OBJECTIVETo investigate the best choice of operation opportunity and operation plan for limb fractures combined with severe craniocerebral trauma in children.

METHODSFrom January 2005 to July 2012,36 patients with limb fractures and severe craniocerebral trauma were received,including 24 males and 12 females aged from 1 to 13 years old (mean, 6.1 +/- 3.0). The time from injury to hospital was (18.0 +/- 15.0) h. Glasgow coma score were less than 8 with an average of 6.4 +/- 1.3. AIS-ISS score were 25.9 +/- 8.1. Thirteen patients were open fracture, 23 were closed fracture. Patients were divided into immediate operation group (21 patients) received fracture fixation with 24 h, the average time was (15.0 +/- 7.4) h, and delayed operation group (15 patients) received fracture fixation after 24 h, the average time was (165.4 +/- 114.6) h. All patients were treated by open reduction, and 33 cases by internal fixation, 3 cases were external fixation. Operative time, blood loss, fracture healing time and brain trauma,physical trauma, postoperative rehabilitation situation were observed and evaluated.

RESULTSAll patients were healed at stage I ,and no dead, aggravating of coma, disorders of breathing and circulation occurred during operation. Operative time,blood loss,healing time in immediate operation group was (44.5 +/- 25.3) min, (47.1 +/- 36.5) ml, (2.7 +/- 0.5) months, respectively; while in delayed operation group was (87.0 +/- 40.0) min, (112.7 +/- 67.5) ml, (3.8 +/- 1.2) months,respectively; and there were obvious differences between two groups. There was no siginificant meaning in Glasgow coma score and Fugl-Meyer motor function between immediate operation group (4.7 +/- 0.6, 97.9 +/- 2.7) and delayed operation group (4.7 +/- 0.5, 97.7 +/- 3.9) (t = 0.23, P > 0.05; t = 0.11, P > 0.05).

CONCLUSIONThe condition of limb fractures combined with severe craniocerebral trauma in children is seriously, comfortable surgical opportunity should according to particular case, and immediate operation can performed on the condition of stabled vital signs.

Adolescent ; Child ; Child, Preschool ; Craniocerebral Trauma ; surgery ; Extremities ; injuries ; surgery ; Female ; Follow-Up Studies ; Fracture Fixation ; Fracture Fixation, Internal ; Fractures, Open ; surgery ; Humans ; Infant ; Male

OBJECTIVETo investigate the relationship between the biological features and the treatment efficacy and prognosis in acute myeloid leukemia subtype M2 (AML-M2) patients with chromosome 8 and 21 translocation.

METHODSBy using Cox regression model and Kaplan-Meier analyses, prognostic factors in 54 cases of de novo adult AML with t(8;21) in our institute from 1990 to 2003 were retrospectively analyzed.

RESULTThe complete remission (CR) rates were 81.9% for all M2 patients, 82.4% for patients with normal karyotype, 88.5% for patients with t(8;21) [P > 0.05 for normal karyotype vs t(8;21)], 100.0% for 28 patients with t(8;21) alone and 75.0% for 24 patients with additional chromosome abnormalities (P < 0.01). The actuarial 3 year overall survival(OS) was 26% for M2 patients with normal karyotype, 25% for patients with t(8;21) [P > 0.05 for normal karyotype vs t(8;21)], in whole t(8;21) group, 46.4% for patients with t(8;21) alone and 0% for patients with additional chromosome abnormalities (P < 0.01). Multivariate analysis of prognostic factors showed that chromosome abnormalities besides t(8;21) was the only factor affecting CR, disease-free survival (DFS) and OS. DFS of allogeneic hematopoietic stem cell transplantation (HSCT) and intermediate-dose cytarabine/high dose cytarabine (IDAC) groups were better than the group received routine dose cytarabine as postremission therapy (P < 0.01).

CONCLUSIONAML with t(8;21) is not a single defined AML subset, and patients with additional chromosome abnormalities have a worse prognosis. HSCT and IDAC could improve the outcome. HSCT is the best choice for patients with high risks, especially with additional chromosome abnormalities.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Chromosomes, Human, Pair 21 ; genetics ; Chromosomes, Human, Pair 8 ; genetics ; Female ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Myeloid, Acute ; drug therapy ; genetics ; surgery ; therapy ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Translocation, Genetic

OBJECTIVETo evaluate the indications and surgical procedure of bronchial and pulmonary artery sleeve resection for patients with centrally located non-small cell lung cancer, and how to prevent complications.

METHODSFrom July 1989 to Aug 2000, 32 cases of central NSCLC were treated with bronchial and pulmonary arterial sleeve resection and reconstruction. The results were retrospectively analyzed.

RESULTSThe complication rate was 25.0% (8/32), the mortality rate in 30-day postoperation was 6.3% (2/32), the overall 1-, 3- and 5-year survival rate was 82.8% (24/29), 50.0% (11/22) and 33.3% (4/12), respectively.

CONCLUSIONBronchial and pulmonary arterial sleeve resection and reconstruction in the treatment of patients with central NSCLC can not only maximize preservation of functional pulmonary parenchyma and improve patients, quality of life, but also provide an opportunity for those patients with poor pulmonary function to receive surgical resection of the tumor.

Adult ; Aged ; Bronchi ; surgery ; Carcinoma, Non-Small-Cell Lung ; surgery ; Female ; Humans ; Lung Neoplasms ; surgery ; Male ; Middle Aged ; Postoperative Complications ; prevention & control ; Pulmonary Artery ; surgery ; Reconstructive Surgical Procedures

OBJECTIVETo detect the factors relevant to stenosis of tracheal graft and to find feasible methods to solve this problem.

METHODSSixteen mongrel dogs were divided into groups A and B randomly and equally. Five-ring-length tracheal segments were allotransplanted. All grafts and anastomotic sites were covered with omental pedicles. In group A, no immunosuppressant was given and in group B, the recipients were treated with cyclosporine. The animals were sacrificed 4 weeks after operation, and their postmortem specimens were examined grossly and histologically. All allografts were assessed by percent patency. Epithelial regeneration and morphology of the cartilage were semiquantitatively evaluated.

RESULTSStructural integrity of the allografts were maintained better in group B than in group A. Tracheal stenosis was found to be more serious in group A. The scores of epithelial regeneration and cartilage morphology were higher in group B than in group A, and in each group positive correlation was found between the percent patency and the score of epithelial regeneration or cartilage morphology.

CONCLUSIONSImmunosuppressive drugs are necessary to maintain the structure of allografts. Tracheal stenosis is correlated closely with epithelial regeneration and morphological maintenance of the cartilage.

Animals ; Dogs ; Immunosuppressive Agents ; pharmacology ; Male ; Trachea ; pathology ; transplantation ; Transplantation, Homologous

OBJECTIVETo investigate the optimal time and method of the early repair of the full layer eyelid defect caused by chemical burn.

METHODSFree nasal septum mucosal cartilage flap with muscle flap, skin grafting, or skin flap were performed in 18 cases (19 eyelids) with chemical burn within 4 postburn weeks. Eyelid reconstruction and corneal transplantation were performed at the same time in 4 patients.

RESULTSAll the reconstructed eyelids and transplanted cornea survived. The incidence of severe complications, such as exposure keratitis, corneal ulcer and eyeball perforation decreased.

CONCLUSIONFull layer eyelid defect caused by chemical burn should receive early reconstruction and repair, including timely reconstruction of eyelid for the sake of protecting the eyesight and of alleviating the inflammatory reactions, and the corneal transplantation should be done at the same time to avoid corneal perforation. Nasal septum mucosal cartilage flap could be ideal for the eyelid reconstruction.

Adult ; Burns, Chemical ; surgery ; Corneal Transplantation ; Eyelids ; injuries ; surgery ; Female ; Humans ; Male ; Middle Aged

Adolescent ; Child ; Child, Preschool ; Chorda Tympani Nerve ; diagnostic imaging ; surgery ; Cochlear Implantation ; methods ; Female ; Humans ; Male ; Radiography ; Young Adult

To investigate the molecular mechanism of angiotensin II (Ang II) receptor activation in adult rat cardiac fibroblasts, the expressions of cell signal transduction-associated genes were studied by using cDNA microarray. Cardiac fibroblasts of adult Sprague-Dawley rats (230~250 g) were isolated and cultured. The cells were divided into 4 groups: Ang II, Ang II + losartan, Ang II + PD123319, Ang II + losartan + PD123319. The expressions of Ang II receptors were studied by immunohistochemical staining. Total RNA was extracted and purified. After cDNA synthesis and biotin-16-dUTP labeling, the probes were denatured and hybridized with GEArray Q Series mouse G Protein-coupled Receptors Signaling Pathway Finder Gene Array (MM-025) containing 96 genes associated with 11 pathways. The arrays were scanned with a Uniscand1000 scanner and further analyzed with GEArray Analyzer software. RT-PCR was used to further confirm the results of gene microarray. The results of immunohistochemical staining showed that the expression of Ang II type 2 (AT2) receptor was evidently induced by Ang II stimulation when Ang II type 1 (AT1) receptor was blocked. The results of gene array indicated that blocking AT1 receptor changed 34 genes (more than 2 folds), 30 were down-regulated and 4 were up-regulated. The maximum change was not beyond 20 folds. The following 9 pathways were activated: cAMP/PKA, Ca2+, PKC, PLC, MAPK, PI-3 kinase, NO-cGMP, Rho, NF-kappaB pathways. Blockade of AT2 receptor caused 64 genes changing more than 2 folds (48 were down-regulated and 16 were up-regulated). Eleven pathways were basically activated. The change of the following 7 genes was over 30 folds: Cyp19a1 (37 folds), Il1r2 (42 folds), Cflar (53 folds), Bcl21 (31 folds), Pik3cg (278 folds), Cdkn1a (90 folds), Agt (162 folds). According to the activated extent, the signal transduction pathways in turn were PI-3 kinase, NF-kappaB and JAK-STAT pathways. Blocking both AT1 and AT2 receptors changed 46 genes more than 2 folds (36 were down-regulated and 10 were up-regulated). Eleven pathways were basically activated. The results of RT-PCR of IL-1beta and TNF-alpha confirmed the observations in gene microarray. Our results show that Ang II can induce a high expression of AT2 receptor in adult rat cardiac fibroblasts when AT1 receptor is blocked, and the signal mechanism of AT2 receptor is clearly different from that of AT1 receptor.
Angiotensin II ; pharmacology ; Angiotensin Receptor Antagonists ; pharmacology ; Animals ; Fibroblasts ; metabolism ; Gene Expression ; Imidazoles ; pharmacology ; Losartan ; pharmacology ; Myocardium ; cytology ; Pyridines ; pharmacology ; Rats ; Rats, Sprague-Dawley ; Receptor, Angiotensin, Type 1 ; metabolism ; Receptor, Angiotensin, Type 2 ; metabolism ; Signal Transduction

OBJECTIVEThe study was designed to investigate the clinical characteristics and the effects of therapeutic proposal on Kawasaki disease (KD).

METHODSClinical features, diagnosis and treatment for totally 942 patients with KD hospitalized during Jan, 2000 to Dec, 2004 were reviewed. Clinical features of typical and incomplete KD were compared. Also, influential factors for KD resistant to intravenous immune globulin (IVIG) therapy were analyzed. Five hundred and ten cases were followed up for analyzing the prognosis of coronary artery lesion (CAL).

RESULTS(1) 774 cases were diagnosed as typical KD, and 168 cases as incomplete KD. The incidence of infants with incomplete KD was higher than that of infants with typical KD (18.5% vs. 10.1%, P < 0.01). As compared with typical KD, the cases of incomplete KD had a long duration of fever before final diagnosis [(7.7 +/- 2.9) d vs. (7.0 +/- 2.4) d, P < 0.01], high hemoglobin level [Hb, (106.6 +/- 13.4) g/L vs. (103.5 +/- 12.3) g/L, P < 0.01], high hematocrit [Hct, (32.0 +/- 4.3)% vs. (31.0 +/- 4.0)%, P < 0.01], and high prevalence of CAL (23.8% vs. 16.8%, P < 0.05), respectively. The occurrence rate and emerging time of clinical manifestations in incomplete KD and in typical KD were presented, respectively: non-exudative conjunctivitis [occurrence rate, 64.9% vs. 93.5%; emerging time, (4.4 +/- 1.4) d vs. (4.0 +/- 1.6) d, respectively (P < 0.05 or P < 0.01)], erythema and cracking of lips [occurrence rate, 50.6% vs. 94.8%; emerging time, (4.9 +/- 1.4) d vs. (4.5 +/- 1.6) d, respectively (P < 0.05 or P < 0.01)], rash [occurrence rate, 35.1% vs. 87.7%; emerging time, (3.9 +/- 1.9) d vs. (3.4 +/- 1.7) d, respectively (P < 0.05 or P < 0.01)], erythema and edema of extremity [occurrence rate, 26.8% vs. 71.4%; emerging time, (6.7 +/- 1.5) d vs. (5.3 +/- 1.7) d, respectively (P < 0.01)], cervical lymphadenopathy [occurrence rate, 34.5% vs. 68.0%; emerging time, (4.3 +/- 2.5) d vs. (3.6 +/- 2.2) d, respectively (P < 0.05 or P < 0.01)], strawberry tongue [occurrence rate, 31.0% vs. 59.8%; emerging time, (5.6 +/- 2.2) d vs. (4.9 +/- 1.8) d, respectively (P < 0.05 or P < 0.01)], membranous desquamation of fingertips [occurrence rate, 34.5% vs. 56.3%; emerging time, (11.7 +/- 3.3) d vs. (10.3 +/- 2.7) d, respectively (P < 0.01)], and desquamation peri-anus [occurrence rate, 42.9% vs. 50.0%; emerging time, (6.7 +/- 2.7) d vs. (6.9 +/- 2.5) d, respectively (P > 0.05)]. Except for peri-anus desquamation, other clinical manifestations in incomplete KD were sporadical as compared to typical KD. (2) Six per cent (51/857) of cases were resistant to the IVIG therapy. As compared to the group responding to IVIG therapy, high prevalence of CAL (31.4% vs. 17.1%, P < 0.05), long fever duration [(10.6 +/- 3.9) d vs. (7.5 +/- 2.3) d, P < 0.01], low Hb level [(99.9 +/- 14.1) g/L vs. (104.3 +/- 12.4) g/L, P < 0.01], low Hct [(30.1 +/- 4.5)% vs. (31.2 +/- 4.0)%, P < 0.05], low platelet [PLT, (256.9 +/- 142.4) x 10(9)/L vs. (309.7 +/- 131.5) x 10(9)/L, P < 0.05], and low albumin level [ALB, (27.8 +/- 8.4) g/L vs. (33.5 +/- 6.7) g/L, P < 0.01] were found in the group resistant to IVIG therapy, respectively. (3) In patients who received IVIG 1 g/kg and 2 g/kg, the recovery rates from CAL were 83.1% and 89.7% (P > 0.05), respectively. The prevalence of CAL in those without CAL in acute and subacute stages was 0.9% and 3.5% (P > 0.05), respectively, during 2 year-follow-up period.

CONCLUSION(1) Infants appeared to have more chances to suffer from incomplete KD. Incomplete KD had high prevalence of CAL. The peri-anus desquamation might be an important clue for early diagnosis of incomplete KD. (2) In acute stage, the influential factors for KD resistance to IVIG therapy included prolonged fever, non-elevated PLT, and persistent decrease in Hb, Hct and ALB levels. (3) Children receiving IVIG 1 g/kg and 2 g/kg had the similar effects on recovery and prevention from CAL within the first two years after KD onset.

Adolescent ; Blood Platelets ; drug effects ; Child ; Child, Preschool ; China ; Coronary Aneurysm ; drug therapy ; epidemiology ; etiology ; prevention & control ; Coronary Artery Disease ; complications ; diagnosis ; drug therapy ; physiopathology ; Dose-Response Relationship, Drug ; Female ; Fever ; drug therapy ; physiopathology ; Follow-Up Studies ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Immunologic Factors ; Infant ; Infant, Newborn ; Male ; Prognosis ; Retrospective Studies ; Risk Factors ; Treatment Outcome

OBJECTIVETo study the clinical characteristics and therapeutic outcome of Ph+ acute lymphoblastic leukemia (ALL).

METHODSThirty previously untreated cases of Ph+ B-ALL were diagnosed in our institute. The patients were treated with combination chemotherapy of CODP +/- L regimen, Imatinib (400 approximately 600 mg/d) was continuously given to those who couldn't reach CR. Fourteen patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT) after CR, while 16 received consolidation of intensive chemotherapy.

RESULTSThirty (32.6%) of 92 ALL patients were diagnosed as Ph+ ALL, with a median age of 25.5 (14 - 60). Among them Ph+ as the sole anomaly was seen in 16 patients, and Ph+ with additional chromosome abnormalities in 14. Besides the B cell markers, 23 (76.7%) patients had CD34+ and 13 (43.3%) CD13+ and/or CD33+. Nineteen of the Ph+ ALL patients underwent molecular analysis; 13 (68.4%) expressed P190 and 6 (31.6%) P210. Increased WBC (> 30 x 10(9)/L) was found in 22/30 cases while WBC > 100 x 10(9)/L in 9/30 cases. The chemotherapy complete remission rate was 68.8% in patients with only Ph+ versus 28.6% in those with additional chromosome abnormalities. All seven refractory/relapsed patients reached CR with Imatinib therapy. The total complete remission rate was 73.3% in all Ph+ ALL patients. The median remission duration was shorter in patients with additional chromosome than in those with only Ph+ (1 vs 7 months, P < 0.05), and so was the survival period (7 vs 9 months, P > 0.05). The remission duration was significantly longer in patients received allo-HSCT than in those received chemotherapy only (8 vs 0.5 month, P < 0.05), and so was the survival period (12.5 vs 6 months, P < 0.05).

CONCLUSIONAdditional chromosome abnormalities negatively affect the prognosis and therapeutic effect of Ph+ ALL patients. Imatinib is effective for the induction therapy of Ph+ ALL. The survival period of patients who received allo-HSCT was obviously longer than those who received chemotherapy only.

Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation ; methods ; Humans ; Male ; Middle Aged ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; pathology ; surgery ; Prognosis ; Retrospective Studies ; Survival Analysis ; Treatment Outcome ; Young Adult