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ABSTRACT: To investigate clinical characteristics and predictive factors of refractory Mycoplasma pneumoniae pneumonia (RMPP) in children so as to recognize and treat the disease earlier.
The data including febrile time, inflammatory markers (WBC, neutrophil, CRP) and radiological features of 213 children hospitalized with Mycoplasma pneumoniae pneumonia (MPP) (72 with refractory MPP and 141 with mild MPP were retrospectively analyzed). The primary diagnostic criteria of refractory MPP: the patient's condition still deteriorates after treatment with macrolides for more than 5 days. The independent variables which had significant difference in univariate analysis was analyzed by multivariate logistic regression analysis. The predictive criteria of RMPP were further applied in 100 other patients prospectively. Kappa test was used to evaluate the accuracy rate.
Refractory MPP patients: febrile time was more than 10 days, white blood cell (WBC) count was (3.8 - 18.5)×10(9)/L in peripheral blood routine test, CRP was 38 mg/L - > 160 mg/L, large lobar consolidation with high density (> 2/3 pulmonary lobe, CT value 40 - 50 HU, without air bronchogram). Mild MPP patients: febrile time was less than 10 days, CRP was often less than 40 mg/L. Independent risk factors for RMPP were febrile time, CRP, large consolidation area with high density in lungs with or without pleural effusion (OR = 1.586, P = 0.017; OR = 4.344, P = 0.001; OR = 2.660, P = 0.012), CT value 40 - 50 HU which were demonstrated by logistic regression analysis. The specificity, sensitivity and Youden index for this diagnostic test were respectively 0.96, 0.94 and 0.90 at a CRP cut off of 40 mg/L. The sensitivity, specificity, and Kappa value for the above criteria to diagnose RMPP were respectively 0.96, 0.94 and 0.9.
The predictive factors for RMPP are febrile time (> 10 days), CRP (> 40 mg/L), large lobar consolidation with high density (> 2/3 pulmonary lobe, CT value > 40 HU with or without pleural effusion) for the purpose of treating earlier.
Zhonghua er ke za zhi. Chinese journal of pediatrics 12/2012; 50(12):915-8.
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ABSTRACT: Streptococcus pneumoniae necrotizing pneumonia (SPNP) was reported elsewhere but not in China yet. Inappropriate treatment due to poor recognition of this disease could influence its prognosis. This paper presents the clinical characteristics, diagnosis and treatment of SPNP hoping to elevate pediatrician's recognition level for this disease.
Clinical manifestations, radiological findings, treatment and prognosis of 20 patients (9 boys, 11 girls) who had been hospitalized with SPNP in Beijing Children's Hospital from 2004-2011 were retrospectively analyzed.
The patients included in this study aged from 9 months to 6 years [(27.9 ± 15.8) m] and were healthy before admission. They were febrile for 8 to 50 days [(27.7 ± 13.5) d] and hospital day was 24 - 55 days [(36.5 ± 8.3) d]. The general condition of all subjects was relatively poor and they all had fever and cough. One child had moderate fever and nineteen children had high fever. Dyspnea was found in sixteen children. Fine rales were found on auscultation in 18 children, among whom diffuse wheeze appeared in 4 children, and wheezy phlegm was found in two children. Signs of pleural effusion were discovered in all cases by physical examination and chest X-ray. White blood cell (WBC) count was 16.2 - 60.95×10(9)/L and neutrophil was 70.5% - 80.2% in peripheral blood routine test. Erythrocyte sedimentation rate (ESR) was 44 - 109 mm/h [(69.6 ± 16) mm/h]and C-reactive protein (CRP) was 80 - > 160 mg/L. The pleural effusion biochemistry and routine test revealed a WBC count of 6400×10(6)/L-too much to count, polykaryocyte of 51% - 90%, glucose of 0.02 - 1.8 mmol/L, protein of 32 - 51 g/L and LDH of 5475 IU/L-or higher. Pleural effusion culture in all cases and blood culture in 2 cases was positive for Streptococcus pneumoniae. Chest X-ray or CT revealed high density and well-distributed lobar consolidation in one lung or two lungs initially. Single or multiple low density lesions in the area of lobar consolidation were found a week later, accompanied by multiple cystic shadow or cavity at the same time or afterwards. Bulla of lung appeared later. Pleural effusions were found in all patients. Seven cases complicated with hydropneumothorax, two with otitis media, one with heart failure, one with cardiac insufficiency. Seventeen patients were treated with vancomycin or teicoplanin or linezolid two with amoxicillin and clavulanate potassium. Other two patients had been treated with meropenem and cephalosporin antibiotics respectively before admission, and they had been at recovery stage when they were hospitalized. Thoracic close drainage and thoracoscopy were performed respectively in 18 cases and 3 cases, respectively. After a follow up of more than 6 months, chest CT showed that almost all lesions in lungs recovered during 4-6 months. No one received pneumonectomy.
SPNP has special manifestations. The incidence in infants is higher. Patients' general condition is poor and febrile course is relatively long. All patients manifested fever and cough, with a presence of dyspnea in most of them. WBC, neutrophil and CRP elevated apparently. The characteristic of pleural effusion indicates empyema. In early stage, the chest X-ray and CT showed high-density lobar lesions, followed by low-density lesions and cyst gradually. Bulla of lung and/or hydropneumothorax may appear at the late stage. But if diagnosed and treated promptly, the prognosis of SPNP was relatively good.
Zhonghua er ke za zhi. Chinese journal of pediatrics 06/2012; 50(6):431-4.
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ABSTRACT: Burkitt lymphoma (BL) is an aggressive B-cell non-Hodgkin lymphoma (NHL) and it appears to be one of the most common childhood cancers in equatorial areas. Unprecedented gains have been made in the cure rates for BL during the past two decades and these reflect steady improvements in treatment protocols and a multidisciplinary approach to patient care. However, the life-threatening side effects associated with conventional treatment urge us to explore new strategies. Arsenic trioxide (ATO), a natural product that has improved the prognosis of acute promyelocytic leukemia (APL) from highly fatal to highly curable, has also been proven to be effective in treating BL cell lines through multiple pathways in our study. Our data indicates that ATO can inhibit the proliferation of BL cell lines through 1) arresting the cell cycle; 2) decreasing the respiratory function and transmembrane potential of mitochondrial; and 3) downregulating the expressions of Survivin, Bcl-2, MCL-1, and VEGF. We therefore suggest that dissecting the pharmaceutical mechanism of ATO at the molecular and cellular levels may be a good strategy to explore the value of traditional natural products in treating high malignant Burkitt lymphoma.
Oncology Research Featuring Preclinical and Clinical Cancer Therapeutics 01/2011; 19(3-4):149-63. · 1.30 Impact Factor
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ABSTRACT: Prompt diagnosis of Mycobacterium tuberculosis (MTB) infection is an essential step in tuberculosis control and elimination. However, it is often difficult to accurately diagnose pediatric tuberculosis (TB). The tuberculin test (TST) may have a low specificity because of cross-reactivity with antigens present in Mycobacterium bovis bacillus Calmette-Guerin (BCG) and other mycobacteria, especially in China with a predominantly BCG-vaccinated population. Early-secreted antigenic target 6-kDa protein (ESAT-6) and culture filtrate protein 10 (CFP-10), stand out as suitable antigens that induce an interferon-gamma (IFN-γ) secreting, T-cell-mediated immune response to infection. While, considered the higher costs and complexity of the IFN-γ release assay (TSPOT), we aimed to evaluate the TSPOT and TST test in the clinical diagnosis of pediatric tuberculosis and to establish a diagnostic process suitable for China.
The sensitivity and specificity of the assay were evaluated in total seventy four children with active tuberculosis and fifty one nontuberculous children with other disease, and then the results were compared with TST. Logistic regression models were used to identify variables that were associated with positive results for each assay. The independent variables included sex, age, birth place, vaccination history, close contract with an active TB patient.
The sensitivity of TSPOT was higher than TST in active TB children with or without BCG vaccination, as well as in children with culture-confirmed TB. But the difference was not significant statistically. Combining results of the TSPOT and TST improved the sensitivity to 94.6%. Agreement of the TST and TSPOT was low (77.0%, κ = 0.203) in active TB patients. The difference in specificity between TSPOT and TST test was statistically significant (94.1% vs. 70.6%, P = 0.006). Specificity of the two tests in patients without prior BCG vaccination history was similar (80.0% vs. 60.0%). The concordance between the two tests results in BCG vaccinated subjects was low (71.7%, κ = 0.063). For TSPOT, none of the included risk factors was significantly associated with positive results. For TST, BCG vaccination (OR: 1.78; 95%CI: 1.30 - 2.00) was significantly associated with positive results.
Although IFN-γ release assay had relatively high sensitivity and specificity, we also should consider the higher costs and complexity of this test. Therefore, TSPOT could be used as the complementary tool of TST in circumstances when a suspected patient with negative TST results, or to exclude a positive TST result caused by BCG vaccination.
Chinese medical journal 10/2010; 123(20):2786-91. · 0.86 Impact Factor
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Lei Wang,
Liang Cui,
Jia-ping Wei,
Guang-ping Li,
Guo-xian Qi,
Yu-ming Hao,
Wen-zhi Wang, Hui-min Li,
Jun Liu,
Dong-ju Jiang,
Yu-dong Zhang
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ABSTRACT: To compare the efficacy and safety of intravenous levosimendan and dobutamine in patients with decompensated heart failure refractory to conventional medications.
Patients were recruited into this multicentre, randomised, positive-controlled and parallel-group study to receive either levosimendan or dobutamine therapy. In the levosimendan group, an initial loading dose of levosimendan of 12 microg x kg was infused over 10 min, followed by a continuous infusion of 0.1 microg x kg(-1) x min(-1) for 1 h and then 0.2 microg x kg(-1) x min(-1) for 23 h. In the control group, dobutamine was infused for 1 h at an initial dose of 2 microg x kg(-1) x min(-1) without a loading dose, followed by a continuous infusion of 4 microg x kg(-1) x min(-1) for 23 h. Hemodynamic responses at 24 h were evaluated by echocardiography (in both groups) and Swan-Gans catheter (in the levosimendan group). Clinical assessment was performed to evaluate efficacy and safety of the medications.
A total of 225 patients from 12 medical centers were evaluated; 119 assigned to levosimendan and 106 assigned to dobutamine group. The effectiveness rate was 31.9% (38 patients) in the levosimendan group and 17.9% (19 patients) in the dobutamine group (P < 0.01). At 24 h, left ventricular ejection fraction (LVEF) was improved by 6. 4% in the levosimendan group, compared with 4.6% in the dobutamine group (P > 0.05). Stroke volume (SV) was increased by 11.1 ml in the levosimendan group and 2.8 ml in the dobutamine group respectively (P < 0.05). Dyspnea and clinical manifestations improvements were more significant in levosimendan therapy group compared to dobutamine group. There were less adverse effects including hypokalemia, hypotension and ventricular premature beats in the levosimendan group than in the dobutamine group (P < 0.05).
Levosimendan was well tolerated and superior to dobutamine for patients with decompensated heart failure refractory to conventional medications.
Zhonghua xin xue guan bing za zhi [Chinese journal of cardiovascular diseases] 06/2010; 38(6):527-30.
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ABSTRACT: To explore the clinical manifestation, immune abnormality and outcome of disseminated Bacille Calmette-Guérin (BCG) infection in children.
The clinical data of 18 children with disseminated BCG infection seen from January 2000 to December 2007 were analyzed retrospectively.
Thirteen of the children were male among 18 patients. Disseminated infection first appeared in armpit lymph nodes ipsilateral to the vaccination site, then spread to lungs in 15, lymphnodes of mediastinum or abdominal cavity in 18, skin and soft tissues in 5, skeletons in 4, liver in 4, spleen in 8, kidney, adrenal gland or meninges in 3. Twelve children were diagnosed to have primary immunodeficiency; 3 had severe combined immunodeficiency (SCID); 7 had chronic granulomatous disease (CGD), 2 had IL-12/IFN-gamma passageway deficiency. Eleven of the 18 patients died, and the remaining 7 patients were followed up from 1 to 9 years and are alive at present, but presented recurrent skin and bone tuberculosis in 4 and recurrent other infection in 3.
Most Children with disseminated BCG infection had primary immunodeficiency. CGD and IL-12/IFN-gamma passageway deficiency accounted for considerable proportion, so special immune function should be detected in these patients. The prognosis was poor. The type of the immunodeficiency diseases should be identified in early stage and the specific immune treatment should be given to the patients.
Zhonghua er ke za zhi. Chinese journal of pediatrics 01/2010; 48(1):65-8.
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ABSTRACT: This study was purposed to set up real-time quantitative RT-PCR technique and to measure leukemia fusion gene transcripts in patients with chronic myeloid leukemia (CML), acute lymphoblastic leukemia (ALL) and acute promyelocytic leukemia (APL). All plasmids containing the target gene sequences were constructed to establish the standard curves. A TaqMan based real-time quantitative RT-PCR was performed to measure aberrant fusion gene transcripts in 130 samples of peripheral blood (PB) or bone marrow (BM) from 49 patients with leukemia. The results showd that the BCR-ABL(P210) transcripts were detected in 28 (82.4%) out of 34 CML patients (the ratios of BCR-ABL(P210)/ABL varied from 0.01 to 3.19) and also in 2 (33.3%) out of 6 ALL patients. The BCR-ABL(P190) transcripts were detected in 2 (33.3%) out of 6 ALL patients. The BCR-ABL(P210) and BCR-ABL(P190) transcripts were both detected in 1 (2.9%) CML patients. The PML/RARalpha transcripts were detected in 7 (77.8%) out of 9 APL patients (the ratio of PML-RARa/ABL varied from 0.0014 to 3.199). The relative frequency of both bcr1 and bcr3 was 42.9%, while that of bcr2 was 14.3%. The transcript level of aberrant fusion gene varied from the clinical situation of patient. It is concluded that real-time quantitative PCR is a reliable, innovative and promising technology with high sensitivity and specialty. It has potential clinical value for defining diagnosis, typing tumor, selecting treatment, measuring the tumor load, monitoring fusion gene expression level and evaluating therapeutic strategies, which is worthy to be popularized.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 09/2009; 17(4):969-73.
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ABSTRACT: To introduce the early clinical features and the main points of diagnosis and treatment of children with pancreatic pleural fistula presenting massive bloody pleural effusion.
The clinical data of symptoms, signs, laboratory examination, diagnosis and treatment methods of pancreatic pleural fistula in a child are presented.
A 4-year-old boy presented with one month history of intermittent fever, cough, chest tightness and chest pain. His pleural effusion collected from three thoracic closed drainage was hemorrhagic. He had no positive abdominal signs. His abdominal B-mode ultrasonography showed rough pancreatic echo. The levels of amylase in blood and pleural fluid were 495 U/L and 35 938 U/L, respectively. There was a fistulous tract from the thoracic cavity leading to the pancreas at thoracic surgical exploration. The child was cured by total parenteral nutrition, intravenous infusion of somatostatin and a stent placed in the pancreatic duct by endoscopic retrograde cholangiopancreatography (ERCP).
The child with pancreatic pleural fistula in early stage mainly present respiratory symptoms and lack abdominal symptoms, so the diagnosis and treatment is often delayed. Significant increase of pancreatic amylase level in pleural effusion can be an important clue and basis for the diagnosis. If conservative therapy fails, the examination and treatment with ERCP should be considered.
Zhonghua er ke za zhi. Chinese journal of pediatrics 08/2009; 47(8):621-3.
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ABSTRACT: The aim of this study was to investigate the effect of different concentrations of arsenic trioxide (As(2)O(3)) on apoptosis of lymphoblastoid Raji cell line and its possible mechanisms, as well as its relation to the expression of mcl-1 gene. The Raji cells were treated with different concentrations of As(2)O(3), the classical DNA ladder of cell apoptosis was detected by agar gel electrophoresis, the changes of mitochondrial membrane potential in Raji cells were assayed by confocal laser scanning microscopy, the changes of mcl-1 gene expression after exposure of cells to As(2)O(3) was detected by real-time quantitative fluorescence PCR. The results indicated that 1 micro mol/L As(2)O(3) did not lead to significant apoptosis of Raji cells, 2 - 8 micromol/L As(2)O(3) induced Raji cell apoptosis. Along with increase of drug concentration, the mitochondrial respiratory function and membrane potential of Raji cells obviously decreased. At same time, the expression level of mcl-1 gene were significantly down-regulated. It is concluded that As(2)O(3) can markedly decrease mitochondrial respiratory function and membrane potential of Raji cells, and down-regulate expression of mcl-1 gene, which may be the mechanisms resulting in cell apoptosis.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 07/2009; 17(3):597-601.
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ABSTRACT: This study was aimed to investigate the JAK2V617F mutation in myeloproliferative disorders (MPD) and to evaluate the significance of JAK2V617F in diagnosis and therapy of MPD. The bcr/abl fusion gene in 70 MPD patients was detected by reverse transcription polymerase chain reaction (PT-PCR). The JAK2V617F mutation was detected by allele-specific polymerase chain reaction (AS-PCR) and the results were confirmed by sequence analysis. The results indicated that the bcr/abl fusion gene could be detected in 38 patients with chronic myeloid leukemia (CML), but not in the 32 none-CML patients. The JAK2V617F mutation was detected in 12 out of 16 (75%) patients with polycythemia vera (PV), 3 out of 10 (30%) patients with essential thrombocythemia (ET), 3 out of 6 (50%) patients with idiopathic myelofibrosis (IMF), but not in any of the CML patients. The JAK2V617F mutation frequencies between CML and bcr/abl negative MPD patients were statistically significant (p < 0.05). It is concluded that the JAK2V617F may be a characteristic molecular event in PV, ET and IMF patients which may serve as an important molecular marker for the diagnosis and classification of the three diseases.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 06/2009; 17(3):541-4.
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ABSTRACT: Mcl-1 (myeloid cell leukemia-1) is a member of Bcl-2 (B cell leukemia-2) family, which may play an important role in cell apoptosis regulation, occurrence and development of tumors. This paper reviews advance of studies on the function of the mcl-1 gene and MCL-1 protein, the signal transduction pathways (JAK/STAT, MAPK, PI-3K) regulating the expression of mcl-1 gene, and the relationship between mcl-1 gene and hematologic malignancies.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 05/2009; 17(2):509-12.
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ABSTRACT: The aim of this study was to investigate the bcl-2/IgH expression levels in patients with follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) and its clinical significance. The bcl-2/IgH expression levels in bone marrow (BM) and/or peripheral blood (PB) of 20 patients were detected by using SYBR Green I real-time polymerase chain reaction, and the dynamic monitoring for bcl-2/IgH expression level in 4 of these patients was performed. The results showed that in patients with bcl-2/IgH-positive there was no statistically significant difference in the relative copy-numbers of bcl-2/IgH fusion gene in BM and PB (4.23 and 2.73 respectively, p = 0.107), but the difference was significant before and after treatment (3.61 and 2.69 respectively, p = 0.000), the expression level of bcl-2/IgH fusion gene in newly diagnosed and relapsed group was remarkably higher than that in remission group (p = 0.008). The bcl-2/IgH expression level in PB increased evidently 3 months prior to the clinical relapse in one case out of dynamically monitored 4 cases. It is concluded that the bcl-2/IgH expression level is associated with the disease status, the expression level is high in newly diagnosed and relapsed patients and low in those who achieved remission, the bcl-2/IgH fusion gene expression level decreased evidently after therapy, this change may be related to the clinical disease progression, the results suggest that peripheral blood can be regarded as the resource for detection of bcl-2/IgH fusion gene.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 05/2009; 17(2):368-72.
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ABSTRACT: Tuberculosis is still a public health problem. Host genetic factors, such as polymorphisms in NRAMP1 gene, may play a role in the development of tuberculosis. To clarify the effect of NRAMP1 gene polymorphisms on the development of childhood tuberculosis, the association of NRAMP1 gene polymorphisms with susceptibility to tuberculosis in the ethnic Han Chinese children was investigated.
From January 2005 to March 2008, 130 ethnic Han children with tuberculosis (TB group) were enrolled. Three hundred and ninety hospitalized ethnic Han children for physical examination in the surgery department were used as the control group. The controls were matched with tuberculosis children by age, sex and area. PCR-RFLP analysis was performed on DNA samples to identify allele genotypes of INT4 and D543N in NRAMP1 gene. Genotype frequency differences between tuberculosis patients and controls were analyzed using x2 test.
No statistical difference was found in the genotype frequency of variants G/C and C/C at the INT4 locus between the TB and the control groups. At the D543N locus, the frequency of genotype variants (G/A and A/A) was significantly higher in the TB group (34/130) than that in the control group (66/390) (x2=5.349, P<0.05; OR=1.74, 95%CI=1.08-2.79). When stratified by sex, differences in the genotype distribution were observed only in females at the D543N locus, which the variant genotypes were higher in the TB group (16/52) than in the control group (21/155) (x2=7.866, P<0.05; OR=2.84, 95%CI=1.34-5.99). For males, there was no difference between the TB and the control groups. At the INT4 locus, no difference was observed between the two groups in boys and girls.
Genotypic variation at the D543N locus in NRAMP1 gene may be associated with susceptibility to tuberculosis in ethnic Han Chinese children. Variant genotypes in NRAMP1 gene (G/A and A/A) may be susceptible genotypes to tuberculosis in ethnic Han Chinese children. Girls with variant genotypes were more susceptible to tuberculosis.
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics 04/2009; 11(4):283-7.
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ABSTRACT: The key molecular events in the pathogenesis of myeloproliferative disorders (MPD) have been poorly defined to date, except the case of chronic myeloid leukaemia with the associated rearranged gene bcr/abl. In recent years, a number of different studies described the detection of JAK2 V617F mutation in haematopoietic cells from polycythemia vera patients and other MPDs, which indicates that it plays an important role in the pathogenesis of MPDs. In this review, the JAK2 V617F point mutation and its detection methods, its clinical correlations with MPDs and other malignant hepatopathies were summarized.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 03/2009; 17(1):238-42.
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ABSTRACT: This study was aimed to investigate the apoptosis induced by arsenic trioxide (As2O3) in lymphoma Raji cells and its possible mechanisms. The inhibitory effect of different concentration of As2O3 on cell proliferation was tested by MTT assay. Apoptosis was observed with electron microscope and DNA electrophoresis. The distribution of cell cycles and cell apoptosis were detected by flow cytometry. The results showed that the 1-8 micromol/L As2O3 inhibited Raji cell growth effectively in a dose-and time-dependent manner. As2O3 at 2-8 micromol/L could induce the cell apoptosis and cell cycle arrest. However, As2O3 at 1 micromol/L inhibited Raji cells proliferation only by cell cycle arrest, without any signs of cell apoptosis. In conclusion, substantial proliferation inhibition, cell cycle arrest and apoptosis in Raji cells could be induced by As2O3. Cell cycle arrest happens with apoptosis, when treated with As2O3.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 01/2009; 16(6):1320-4.
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ABSTRACT: Although primary ciliary dyskinesia (PCD) is a group of inherited diseases, accurate diagnosis and appropriate clinical care to prevent and treat the complications could maintain patients' quality of life and normal life span. The diagnosis of PCD may often be delayed because it is frequently misdiagnosed as bronchitis, sinusitis and otitis. This study aimed to analyze and summarize the clinical features of PCD and explore diagnostic and differential diagnostic procedures in children.
Patients were all chosen from the inpatient department of Beijing Children's Hospital, Capital Medical University between 1990 - 2006. The tunica mucosa bronchiorum and/or nasal mucous membrane were gained through bronchoscope in children suspected to have PCD. The ciliary ultrastructures were analyzed through the electron microscope. The clinical features and procedures of the diagnosis and differential diagnosis in children with PCD were analyzed.
There were totally 26 children diagnosed as PCD with 10 (38.5%) Kartagener syndrome. All Kartagener syndrome children had mirror image dextrocardia with normal cardiac structure and situs inversus viscerum. The bronchoscopy performed in eight of 10 Kartagener syndrome children showed bronchus transposition. Twenty-six children came from twenty-five families. Although the siblings of four probands also had the symptoms of chronic cough with sputum, running nose and recurrent respiratory infections, only a boy and his sister were diagnosed as Kartagener syndrome simultaneously. Their parents and the other family members were healthy. Of the 26 patients, 11 were boys and 15 were girls. The median age at diagnosis was 8.7 years. The age of onset was between the second day after delivery and fifteen years old, median age was 3 years. The course of disease before diagnosis was eleven days to twelve years (median 3.5 years). All the children had the symptom of cough, 24 of which had productive cough. Seven cases were found to have clubbing fingers. Dynein arm defect was found in 10 children, 6 of them had total absence of dynein arms and 4 had decreased dynein arm numbers. Microtube derangements were found in 8 children. One Kartagener syndrome child had a normal cilia structure. Bronchiectasis, consolidation and increased lung markings were found in 8, 6 and 7 patients separately on the radiographic study. Twenty patients had sinusitis. Nine of sixteen children had decreased PEF, FEV1 and/or FEF 25 - 75 on the pulmonary function test. Fifteen culture samples obtained from 6 children's sputum and/or bronchoalveolar lavage fluid were positive for 8 strains of Pseudomonas aeruginosa, 5 strains of Streptococcus pneumoniae and 2 strains of Candida albicans. In 1 subject more than one organism were found in the same sample. Hearing lost and gastroesophageal reflux were detected in 3 of 4 and 3 of 5 examined children respectively.
The onset of PCD can occur from neonate to adolescence and usually has a chronic course. The common symptom of pediatric PCD was productive cough and significant growth retardation. The most common ultrastructural abnormalities associated with PCD were the total absence of dynein arms, decreased dynein arm numbers and microtube derangement. Some patients have normal ciliary structures. Bronchiectasis, consolidation and sinusitis were usually seen on the radiography. Pseudomonas aeruginosa and Streptococcus pneumoniae were the two common bacterial organisms obtained from sputum and/or bronchoalveolar lavage fluid of PCD children. Some patients have mixed infections. PCD children have high percentages of hearing lost and gastroesophageal reflux.
Zhonghua er ke za zhi. Chinese journal of pediatrics 09/2008; 46(8):618-22.
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ABSTRACT: Arsenic trioxide has been used to treat lymphoma experimentally since it was used to treat acute promyelocytic leukemia successfully. Massive works in this field have been done throughout the world. It was found that arsenic trioxide exerted an anti-lymphomatic effect via many pathways, and many substances could increase or reduce this effect. Arsenic trioxide can be used to treat relapsed and refractory lymphomas resistant to other chemotherapies with some therapeutic effects and limited side effects, which indicates that arsenic trioxide is a new potential drug for lymphoma. This article is an overview about these foundational and clinical studies.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 01/2008; 15(6):1335-9.
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ABSTRACT: This study was purposed to establish a real-time quantitative reverse transcription polymerase chain reaction (RT-PCR) for detection of PML/RARa fusion gene in patients with acute promyelocytic leukemia (APL) and to explore the relationship between the expression level of PML/RARa fusion gene transcript and the clinical status or efficacy of the therapy in APL. The conventional RT-PCR was used to amplify PML/RARa gene from cultured NB4 cells. Standard curves were constructed by modified real-time PCR on standard template after 10-fold serial dilutions of cDNA of 1 microg NB4 cells. The sensitivity, stability and repeatability of this method was determined. The PML/RARa gene transcripts of bone marrows in 4 APL patients before and after treatment and in 1 APL patient relapsed after complete remission were dynamically detected by modified real-time quantitative RT-PCR. The results indicated that the sensitivity of real-time quantitative RT-PCR for detecting PML/RARa fusion gene was about 10(-5) microg cDNA from NB4 cells, the repeatability and reproducibility of this method were satisfactory, intra-and inter-assay coefficients of variation were 2.1% and 3.8%. The copy numbers of PML/RARa transcripte reflecting PML/RARa fusion gene expression level in 4 newly diagnosed patients with APL were 1884, 5533, 1803, 4677 and the median was 3 475. After ATRA + chemotherapy copy numbers of PML/RARa transcript decreased to 40, 135, 79, 29, and mean was 121. Another patient's PML/RARa gene copy number was 8600 at diagnosis, the gene copy number was 730 after therapy for 4 months, although he was in complete remission, but copy number increased to 11 000 when APL relapsed 3 months later. The copy number efficiently reduced to 1200 after ATRA + chemotherapy. It is concluded that the established real-time quantitative RT-PCR method is sensitive, reliable, accurate and repeatable. The efficiency of method was finally tested for patient samples, showing a PML/RARa transcript copy number in APL patients significantly decrease after therapy, and increase at the time of relapse which indicate that changes of fusion gene expression levels coincide with clinical progress of disease. This method can be used to detect the minimal residual disease, assess response to treatment and evaluate prognosis of disease.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 09/2007; 15(4):714-9.
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ABSTRACT: This study was aimed to investigate the effects of As2O3 on proliferation of B lymphoma Raji cell and to study the expression changes of VEGF mRNA. The modified MTT was adopted to evaluate the effect of As2O3 on proliferation of Raji cells. Semi-quantitative RT-PCR was used to detect the expression of VEGF121 and VEGF165 mRNA in Raji cells exposed to As2O3. The results showed that the As2O3 significantly inhibited the proliferation of Raji cells, the relationship between the inhibition rate and the concentrations of As2O3 was dose- and time-dependent. The VEGF121 and VEGF165 mRNA expressions were found in Raji cells, and both were well-matched in expression levels. After treatment of As2O3 at 1 micromol/L for 48 hours, the expression levels of VEGF121 and VEGF165 mRNA were significantly down-regulated and demonstrated negative correlation with the time of exposure to As2O3. Otherwise some difference were observed in effects of As2O3 on VEGF121 and VEGF165, the expression of VEGF165 mRNA was down-regulated earlier and longer than that of VEGF121, while no similar correlation was found in selected concentration groups. It is concluded that As2O3 can significantly inhibit the growth of Raji cells and may exerted its anti-angiogenesis effects by down-regulating the VEGF mRNA expression even in a low concentration for a long term.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 09/2006; 14(4):704-7.
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ABSTRACT: To investigate the optimal reaction conditions of clonal rearrangements of immunoglobulin heavy chain (IgH) gene using polymerase chain reaction (PCR) with consensus primers and the feasibility of detecting this gene using SYBR green I real-time quantitative PCR with consensus primers, the systemic experiments were performed, which included annealing temperature, concentration of primer, the amount of Taq enzyme, concentration of dNTP and Mg(2+), number of PCR cycles. Detection of this gene on SYBR green I real-time quantitative PCR with consensus primers was carried out under the optimal reaction parameters obtained from previous study, and the sensitivity of IgH rearrangements gene was examined by using SYBR green I real-time quantitative PCR. The results indicated that the optimal annealing temperature was 60 degrees C, the optimal concentration of primers was 0.8 micromol/L, the satisfactory Taq enzyme amount was 0.5 U, the optimal concentration of dNTP was 100 micromol/L, the optimal concentration of Mg(2+) was 3.0 mmol/L, the suitable number of cycle was 40 cycles. Amplification of IgH rearrangement gene and detection of desired gene fluorescence signal on SYBR green I real-time PCR were performed. The sensitivity of IgH gene using this quantitative PCR was 10(4)/ml. It is concluded that the optimal reaction parameters for amplification of clonal IgH rearrangements gene by using PCR technique was determined, and stable and specific amplification of desired gene with consensus primers was performed. Basically, IgH rearrangement gene was successfully detected by SYBR green I real-time PCR.
Zhongguo shi yan xue ye xue za zhi / Zhongguo bing li sheng li xue hui = Journal of experimental hematology / Chinese Association of Pathophysiology 09/2005; 13(4):645-50.
