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Rong-Long Chen,
Kuo-Sin Lin,
Wan-Hui Chang,
Yuh-Lin Hsieh,
Bow-Wen Chen,
Tang-Her Jaing,
Chao-Ping Yang,
Iou-Jih Hung,
Ching-Tien Peng,
San-Ging Shu, [......],
Jiann-Shiuh Chen,
His-Che Liu,
Shu-Huey Chen,
Der-Cherng Liang,
Shyh-Shin Chiou,
Tai-Tsung Chang,
Jiunn-Ming Sheen, Chih-Cheng Hsiao,
Shin-Nan Cheng,
Jung-Chung Lin
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Rong-Long Chen,
Kuo-Sin Lin,
Wan-Hui Chang,
Yuh-Lin Hsieh,
Bow-Wen Chen,
Tang-Her Jaing,
Chao-Ping Yang,
Iou-Jih Hung,
Ching-Tien Peng,
San-Ging Shu, [......],
Jiann-Shiuh Chen,
His-Che Liu,
Shu-Huey Chen,
Der-Cherng Liang,
Shyh-Shin Chiou,
Tai-Tsung Chang,
Jiunn-Ming Sheen, Chih-Cheng Hsiao,
Shin-Nan Cheng,
Jung-Chung Lin
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ABSTRACT: BACKGROUND: Expression of toll-like receptor-4 (TLR4) on tumor cells is known to mediate innate immune responses that influence tumor cell growth and migration. This study aimed to characterize TLR4 expression and elucidate its functional significance in human hepatoblastoma (HB) cells. PROCEDURE: Immunohistochemistry (IHC) was used to determine TLR4 expression level and its distribution pattern in HB liver tissues. Transcripts of tumor necrosis factor (TNF)-α, interleukin (IL)-8, matrix metalloproteinase (MMP)-2, MMP-13, tissue inhibitor of metalloproteinases (TIMP)-1, and TIMP-2 in HB HepG2 cells with lipopolysacharide (LPS) treatment were measured by quantitative PCR. Soluble cytokines and peptides in conditioned media were measured by ELISA. MMP-2 activity was determined by using gelatin zymography. Cell motility and invasiveness was determined using wound healing migration and Matrigel invasion assays, respectively. RESULTS: TLR4 IHC staining demonstrated that TLR4 overexpression in HB liver tissues dramatically vanished after chemotherapy. In vitro study using an HB cell line, HepG2, showed that TLR4 agonist, LPS, significantly decreased transcripts of IL-8 and TNF-α, but did not affect MMP-13 mRNA level. By contrast, LPS only down-regulated IL-8 production and MMP-2 gelatinolytic activity. The latter might be in part due to the increased levels of MMP-2/TIMP-2 complex in conditioned media, thus leading to the decreased motility and invasiveness of HepG2 cells. CONCLUSIONS: HB cells overexpress TLR4, whereas TLR4 agonistic treatment inhibits migration and invasion of HB HepG2 cells. These findings suggest that TLR4 signaling pathway is a potential therapeutic target for control of HB tumor progression. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc.
Pediatric Blood & Cancer 05/2012; · 1.89 Impact Factor
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ABSTRACT: Wu L.-M., Sheen J.-M., Shu H.-L., Chang S.-C. & Hsiao C.-C. (2012) Predictors of anxiety and resilience in adolescents undergoing cancer treatment. Journal of Advanced Nursing00(0), 000-000. doi: 10.1111/j.1365-2648.2012.06003.x ABSTRACT: Aims. To report a study examining the relationships among coping, anxiety and resilience and to identify predictors of anxiety and resilience in adolescents undergoing cancer treatment. Background. Anxiety is the main psychological disturbance in adolescents with cancer, but predictors in the context of anxiety related cancer treatments have not been investigated. Design. Cross-sectional study. Methods. Adolescents (n = 131) recruited from three medical centres between 2010-2011. The eligible participants were diagnosed with cancer, without mental disease and receiving chemotherapy. Participants were assessed with the paediatric cancer coping scale, revised children's manifest anxiety scale, second edition, and the Haase adolescent resilience in illness scale. Results. Over 20% of participants scored high on worry. The most commonly used coping strategy was cognitive coping, followed by problem-oriented coping and finally by defensive coping. There was a statistically significant correlation between defensive coping and level of worry. Resilience was positively correlated with cognitive coping and problem-oriented coping. The cognitive coping and defensive coping were found to predict anxiety and resilience significantly by a step-wise multiple regression analysis and accounted for 40·9% and 46·5% of total variance, respectively. Conclusions. Cognitive coping and defensive coping are predictors for the level of anxiety and resilience in adolescents undergoing cancer treatment. Health providers should evaluate coping behaviour in patients and work towards a cognitive and problem-oriented coping style that will benefit the patient's mental health during treatment.
Journal of Advanced Nursing 04/2012; · 1.48 Impact Factor
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Yung-Li Yang, Chih-Cheng Hsiao,
Hsuan-Yu Chen,
Kai-Hsin Lin,
Shiann-Tarng Jou,
Jiann-Shiuh Chen,
Te-Kau Chang,
Jiunn-Ming Sheen,
Sung-Liang Yu,
Meng-Yao Lu,
Chao-Neng Cheng,
Kang-Hsi Wu,
Shih-Chung Wang,
Jiaan-Der Wang,
Hsiu-Hao Chang,
Shu-Rung Lin,
Shu-Wha Lin,
Dong-Tsamn Lin
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ABSTRACT: The absence of biallelic TCRγ deletion (ABD) is a characteristic of early thymocyte precursors before V(D)J recombination. The ABD was reported to predict early treatment failure in T-cell acute lymphoblastic leukemia (ALL). This study aimed to investigate its prognostic value in Taiwanese patients with T-cell ALL.
Forty-five children with T-cell ALL were enrolled from six medical centers in Taiwan. Quantitative DNA polymerase chain reaction (Q-PCR) was performed to check the status of TCRγ deletion. The threshold for homozygous deletions by Q-PCR was defined as a fold-change <0.35.
ABD was found in 20 patients [20:45] who had higher incidences of induction failure than those without ABD (P = 0.03; hazard ratio [HR] = 8.13; 95% confidence interval [95% CI] = 1.23-53.77) after multivariate regression analysis. Patents with ABD also had inferior EFS and OS (P = 0.071 and 0.0196, respectively). Multivariate Cox analysis indicated that the association between ABD and overall survival was independent of age and leukocyte count on presentation (P = 0.036; HR = 4.25; 95% CI = 1.10-16.42).
The absence of TCRγ deletion is a predictor of a poor response to induction chemotherapy for pediatric patients with T-cell ALL in Taiwan. Providing patients with T-cell ALL and ABD with alternative regimens may be worthwhile to test in future clinical trials.
Pediatric Blood & Cancer 12/2011; 58(6):846-51. · 1.89 Impact Factor
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Yung-Li Yang,
Chia-Cheng Hung,
Jiann-Shiuh Chen,
Kai-Hsin Lin,
Shiann-Tarng Jou, Chih-Cheng Hsiao,
Jiunn-Ming Sheen,
Chao-Neng Cheng,
Kang-Hsi Wu,
Shu-Rung Lin,
Sung-Liang Yu,
Hsuan-Yu Chen,
Meng-Yao Lu,
Shih-Chung Wang,
Hsiu-Hao Chang,
Shu-Wha Lin,
Yi-Ning Su,
Dong-Tsamn Lin
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ABSTRACT: Despite current risk-directed therapy, approximately 15-20% of pediatric patients with acute lymphoblastic leukemia (ALL) have relapses. Recent genome-wide analyses have identified that an alteration of IKZF1 is associated with very poor outcomes in B-cell progenitor ALL. In this study, we determined the prognostic significance of IKZF1 deletions in patients with childhood ALL. This study analyzed 242 pediatric B-cell progenitor ALL patients in Taiwan. We developed a simple yet sensitive multiplex quantitative PCR coupled with capillary electrophoresis to accurately determine the allele dose of IKZF1, and high resolution melting was used for mutation screening for all coding exons of IKZF1. Twenty-six (10.7%) pediatric B-cell progenitor ALL patients were found to harbor these deletions. Most of the deletions were broader deletions that encompassed exon 3 to exon 6, consistent with previous reports. Genomic sequencing of IKZF1 was carried out in all cases and no point mutations were identified. Patients with IKZF1 deletions had inferior event-free survival (P < 0.001), and overall survival (P = 0.0016). The association between IKZF1 deletions and event-free survival was independent of age, leukocyte count at presentation, and cytogenetic subtype by multivariate Cox analysis (P = 0.003, hazard ratio = 2.45). This study indicates that detection of IKZF1 deletions upon diagnosis of B-cell progenitor ALL may help to identify patients at risk of treatment failure. IKZF1 deletions could be incorporated as a new high-risk prognostic factor in future treatment protocols. To the best of our knowledge, this is the first study to examine the poor prognosis of IKZF1 deletions in an Asian population.
Cancer Science 07/2011; 102(10):1874-81. · 3.33 Impact Factor
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ABSTRACT: Children with acute lymphoblastic leukemia (ALL) frequently present with hepatomegaly and mild liver functional impairment. Severe jaundice is rarely seen as a presenting feature. Such patients often present diagnostic dilemmas and therapeutic difficulties. Here we report a 15-year-old boy presenting with severe jaundice and hyperferritinemia, whose bone marrow smear showed B-lineage precursor ALL. We treated him with intravenous immunoglobulin, steroid, and etoposide; then his condition improved. ALL should be considered as a possible diagnosis in severely jaundiced children. Steroid and etoposide can be used as first aid when many chemotherapeutic drugs are contraindicated.
Journal of Pediatric Hematology/Oncology 03/2011; 33(3):e117-9. · 1.16 Impact Factor
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Yung-Li Yang,
Shu-Rung Lin,
Jiann-Shiuh Chen, Chih-Cheng Hsiao,
Kai-Hsin Lin,
Jiunn-Ming Sheen,
Chao-Neng Cheng,
Kang-Hsi Wu,
Shu-Wha Lin,
Sung-Liang Yu,
Hsuan-Yu Chen,
Meng-Yao Lu,
Hsiu-Hao Chang,
Ching-Tzu Yen,
Jing-Fang Lin,
Ying-Hui Su,
Ya-Ping Li,
Chien-Yu Lin,
Shiann-Tarng Jou,
Dong-Tsamn Lin
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ABSTRACT: The classification of B-lineage acute lymphoblastic leukemia (ALL) by specific chromosomal translocations has prognostic implications for risk-directed therapy. Reverse transcription-polymerase chain reaction (RT-PCR) assay is a useful tool for detecting fusion transcripts from common chromosomal translocations of ALL cells.
Multiplex RT-PCR and nested-PCR assays were used to detect ALL-type BCR-ABL1 transcripts of the t(9;22), TCF-PBX1 transcripts of t(1;19), the MLL-AF4 transcripts of t(4;11), and 2 variants of ETV6-RUNX1 of the cryptic t(12;21) in 148 leukemic samples upon diagnosis. The patients received risk-directed protocols of the Taiwan Pediatric Oncology Group-ALL-2002 that consisted of multiple chemotherapeutic agents of different intensities. Event-free survival (EFS) and overall survival (OS) rates were analyzed for genetic abnormalities detected by multiplex PCR and conventional cytogenetic analysis by the Kaplan-Meier method, and compared with the Mantel-Haenszel test. The Cox proportional hazards model was implemented to identify independent prognostic factors for EFS and OS.
In this cohort of Taiwanese children, the relative frequencies of the 4 translocations of B-lineage ALL were 8% with ALL-type t(9;22)/BCR-ABL1, 4% with (1;19)/TCF-PBX1, 2% with t(4;11)/MLL-AF4, and 17.6% with t(12;21)/ETV6-RUNX1. Patients with t(12;21)/ETV6-RUNX1 fusion, hyperdiploidy, and t(1;19)/TCF-PBX1 fusion had the most favorable outcomes, whereas those with the t(9;22)/BCR-ABL1 fusion or t(4;11) and other MLL gene rearrangement had poor prognosis (P<0.001 for EFS and OS). BCR-ABL1, MLL gene rearrangement, and very high-risk group were independent prognostic factors after Cox regression analysis.
The biological factors of leukemia cells are associated with treatment outcomes in childhood ALL. Multiplex RT-PCR assay is an efficient and sensitive diagnostic tool that may improve the ability to accurately and rapidly risk-stratify children with ALL.
Journal of Pediatric Hematology/Oncology 10/2010; 32(8):e323-30. · 1.16 Impact Factor
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ABSTRACT: Intussusception is a common cause of abdominal pain in children. Although most cases are idiopathic, about 10% of cases have a pathologic lead point. Burkitt's lymphoma is not a common etiology. Burkitt's lymphoma might present primarily as intussusception in children but has rarely been associated with appendicitis. We report a case in which a 10-year-old obese boy who initially presented with acute appendicitis due to ileocolic intussusception with appendiceal invagination. He underwent one-trocar laparoscopy and antibiotic treatment. The symptoms recurred 10 days after discharge. Colonoscopy disclosed ileocecal Burkitt's lymphoma as the pathological lead point. This case emphasizes the importance of the age of the patient and the anatomic location of the intussusception related to possible etiology, and hence the most appropriate surgical procedure.
Journal of the Formosan Medical Association 06/2010; 109(6):476-9. · 1.13 Impact Factor
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ABSTRACT: Deferiprone (L1) has been recommended as an effective oral chelation therapy for patients with β-thalassemia major (TM). From 1999 to 2004, 114 patients with TM from five treatment centers were enrolled in this program: iron (Fe) was chelated with L1 in 57 patients, deferoxamine (DFO) in 26, and combined L1/DFO therapy in 31. We found that serum ferritin (SF) was significantly lower in nine patients receiving L1 for more than 5 years (p = 0.04), 22 patients receiving L1 for 1–2 years (p < 0.01) and 31 receiving the combined therapy (p = 0.01), yet significantly higher in those receiving DFO only (p < 0.01). One patient showed transient neutropenia; arthropathy in one patient and gastrointestinal upset in two were noted, with no significant change in alanine aminotransferase (ALT) level. Of 17 patients who were submitted to a liver biopsy, 15 showed no significant change in hepatic fibrosis scores after therapy with L1. None of the 88 patients, including 31 who received the combined therapy, have abandoned oral L1 treatment due to adverse effects. Results of this study proved that L1 or combined therapy with L1 and DFO is effective in reducing SF; incidence of adverse events was low in patients with TM.
07/2009; 30(1):125-130.
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ABSTRACT: Tuberculous myositis is extremely rare, even in immunocompromised hosts. We present a case of disseminated tuberculous myositis in a girl with secondary acute myelogenous Leukemia following successful chemotherapy for undifferentiated sarcoma of the maxillary sinus. The diagnosis was established by direct visualization of acid-fast bacilli in the biopsied nodule and by typical pathologic findings. Three weeks after initiation of antituberculosis treatment, the patient experienced both clinical and radiologic improvement.
Pediatrics & Neonatology 05/2009; 50(2):74-7. · 0.75 Impact Factor
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ABSTRACT: To retrospectively evaluate clinical features, treatment, and outcome of patients with hepatoblastoma (HB) and hepatocellular carcinoma (HCC).
From January 1994 to December 2007, 16 patients of HB and 13 cases of HCC were reviewed.
The mean age of HB patients was much younger than HCC patients (1.2 vs. 11.5 y). There was an 84.6% positive rate for hepatitis B surface antigen in HCC, but none for HB. Mean serum alpha-fetoprotein level was higher with HCC (654,158 ng/mL) than the HB patients (352,843 ng/mL), especially higher in HCC with lung metastasis. Among the HB patients, 12 (75%) had thrombocytosis and 6 (37.5%) had microcytic anemia with high or normal ferritin, whereas only 3 of 13 with HCC (23.1%) had thrombocytosis and none had microcytic anemia. All HBs were resectable either before or after chemotherapy, but only 4 (30.8%) HCCs were resectable. Five-year disease-free survival rate was significantly higher in HB (87%) than in HCC (30%, P<0.001).
Hepatitis B infection was still the most important factor associated with HCC in children even after the national vaccination program against hepatitis B. Extreme thrombocytosis, anemia, alpha-fetoprotein levels are important factors associated with difference in long-term outcomes in children with HB and HCC.
Journal of Pediatric Hematology/Oncology 02/2009; 31(2):91-6. · 1.16 Impact Factor
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ABSTRACT: Over the past few decades, Taiwan has seen striking improvements in the life expectancy of its 400 registered beta-thalassemia major (beta-TM) patients due mainly to adequate transfusion regimens and effective iron chelation therapy. Since 1995, Taiwanese citizens have enjoyed universal health care through National Health Insurance (NIH), receiving comprehensive treatment at minimal cost. In 1984, a national program for thalassemia prevention, control, and hematopoietic stem cell transplantation (HSCT) was initiated. Recent data show 1- and 2-year event-free survival rates of 85 and 78%, respectively. Chelation agents like deferoxamine (DFO), deferiprone (L1) and deferasirox (DFRA) are available in Taiwan, and therapy is tailored to individuals based on drug availability and tissue distribution of iron load. Intensive chelation regimens combining L1 and DFO are recommended in patients with cardiac complications, while DFRA has been found to be effective in reducing serum ferritin, with acceptable side effects. Here, we report advances in thalassemia treatment in Taiwan and suggest treatment guidelines.
Hemoglobin 01/2009; 33(5):304-11. · 1.30 Impact Factor
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ABSTRACT: Hepatic veno-occlusive disease (VOD), also called sinusoidal obstruction syndrome, is rapidly progressing and involves life-threatening complications that can occur in patients receiving chemotherapy and/or bone marrow transplantation. No completely satisfactory treatmentstrategies have yet been established. We present a case of rhabdomyosarcoma ina 21-month-old boy who developed pancytopenia, dyspnea, jaundice, massive ascites and body weight gain of more than 10% after receiving conventional chemotherapy. Hepatic VOD was diagnosed. He recovered after supportive care and treatment with high-dose methylprednisolone.
Pediatrics & Neonatology 09/2008; 49(4):141-4. · 0.75 Impact Factor
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ABSTRACT: Minimal residual disease (MRD) in 56 children with acute lymphoblastic leukemia (ALL) was quantified simultaneously by flow cytometry and by RQ-PCR of WT1 transcripts. Six patients failed remission induction, all had detectable MRD by flow cytometry, and two had undetectable MRD by WT1 assay. Among 41 patients, who achieved remission and had overexpression of WT1 transcripts at diagnosis, the two techniques gave concordant MRD results in 26 and discordant MRD results in 15. Nine patients did not show overexpression of WT1 at diagnosis. Our results indicate that RQ-PCR measurements of WT1 may be of limited value for monitoring MRD in childhood ALL.
Leukemia Research 11/2007; 31(10):1351-7. · 2.92 Impact Factor
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ABSTRACT: Deferiprone (L1) has been recommended as an effective oral chelation therapy for patients with beta-thalassemia major (TM). From 1999 to 2004, 114 patients with TM from five treatment centers were enrolled in this program: iron (Fe) was chelated with L1 in 57 patients, deferoxamine (DFO) in 26, and combined L1/DFO therapy in 31. We found that serum ferritin (SF) was significantly lower in nine patients receiving L1 for more than 5 years (p = 0.04), 22 patients receiving L1 for 1-2 years (p < 0.01) and 31 receiving the combined therapy (p = 0.01), yet significantly higher in those receiving DFO only (p < 0.01). One patient showed transient neutropenia; arthropathy in one patient and gastrointestinal upset in two were noted, with no significant change in alanine aminotransferase (ALT) level. Of 17 patients who were submitted to a liver biopsy, 15 showed no significant change in hepatic fibrosis scores after therapy with L1. None of the 88 patients, including 31 who received the combined therapy, have abandoned oral L1 treatment due to adverse effects. Results of this study proved that L1 or combined therapy with L1 and DFO is effective in reducing SP; incidence of adverse events was low in patients with TM.
Hemoglobin 01/2006; 30(1):125-30. · 1.30 Impact Factor
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Pathology 01/2006; 37(6):552-4. · 2.38 Impact Factor
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ABSTRACT: Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. It is often complicated with Kasabach-Merritt syndrome. The authors describe a 2-month-old girl who developed a rapidly enlarging deep subcutaneous solid tumor in the left axilla. The tumor size was about 10x7 cm and there was no skin change over the mass. The results of laboratory tests were significant for a moderate anemia (7.0 g/dL) and a profound thrombocytopenia (3x10/L). The preoperative radiologic findings were suspicious for a soft tissue sarcoma with scapular erosion. Anemia and thrombocytopenia were corrected by preoperative corticosteroid therapy and blood component transfusion. The tumor was excised totally and the pathologic findings were consistent with Kaposiform hemangioendothelioma. Serial blood examinations showed that the hemoglobin and platelet count returned to the normal range after surgery.
Journal of Pediatric Hematology/Oncology 12/2005; 27(11):596-8. · 1.16 Impact Factor
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ABSTRACT: Rhabdomyosarcoma arising within a congenital cystic adenomatoid malformation (CCAM) is an unusual entity. The patient underwent a lobectomy of his right lower lobe of lung due to a CCAM at the age of two. One year later, he developed a solid embryonal rhabdomyosarcoma at the same location. He received 1-year period chemotherapy and when the tumor reduced to a resectable size, surgical excision was done. The tumor cells appeared more differentiated after chemotherapy. The patient remains disease free to date, 16 months after surgery.
Pediatric Blood & Cancer 12/2005; 45(6):841-5. · 1.89 Impact Factor
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ABSTRACT: Neuroblastoma is an extremely malignant solid tumor in children, characterized by spontaneous differentiation and regression. An epidermal growth factor-like homeotic protein, delta-like (dlk), has been involved in differentiation of neuroblastoma cell lines, but is unknown in in vivo expression of neuroblastoma. By using in situ hybridization and immunohistochemistry, dlk mRNA and protein expression were studied in formalin-fixed archival tissues from 10 patients with neuroblastoma, five with ganglioneuroblastoma, and five with ganglioneuroma. Three adrenal tissues from children died of diseases other than adrenal tumors and one from an adult with pheochromocytoma were severed as normal and disease controls. The results showed strong immunoreactive dlk staining in endothelial cells in neuroblastoma, ganglioneuroblastoma and ganglioneuroma. Dlk was detectable in mature neuromatous stroma and gangliocytes of ganglioneuroma, but not in neuroblasts of neuroblastoma and ganglioneuroblastoma, neither in gangliocytes of ganglioneuroblastoma. In contrast, dlk mRNA expression was mainly observed in the gangliocytes, but was less intense in the neuroblasts and neuromatous stroma cells. Endothelial cells were essentially devoid of dlk mRNA expression. The findings indicated that there is differential expression of dlk gene and protein among neuroblastoma, ganglioneuroblastoma and ganglioneuroma. The stronger expression of dlk in gangliocytes in ganglioneuroma, in contrast to weaker or no expression in gangliocytes in ganglioneuroblastoma and neuroblasts in neuroblastoma, suggests upregulation of dlk during differentiation of neuroblastoma into more benign form. Furthermore, higher dlk protein expression in the tumor endothelium than in the endothelium of normal adrenal gland implies that dlk may regulate the endothelial function in neuroblastic tumors.
Modern Pathology 06/2005; 18(5):656-62. · 4.79 Impact Factor
