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  • 1
    Language: English
    In: International Journal of Molecular Sciences, 01 November 2017, Vol.18(11), p.2472
    Description: Transfusion-dependent thalassemia (TDT) is an inherited disorder characterized by absent or defective production of α- or β-hemoglobin chains. If untreated, the disease invariably culminates in death in early infancy due to cardiac failure or overwhelming infection. Although there is clear...
    Keywords: Transfusion-Dependent Thalassemia ; Hematopoietic Stem Cell Transplantation ; Cord Blood Transplantation ; Biology
    E-ISSN: 1422-0067
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  • 2
    Language: English
    In: Quality of Life Research, 2013, Vol.22(3), pp.625-634
    Description: Byline: Ming-Horng Tsai (1,5,6), Jen-Fu Hsu (5), Wen-Jiun Chou (2), Chao-Ping Yang (1,5), Tang-Her Jaing (1,5), Iou-Jih Hung (1,5), Hwey-Fang Liang (6), Hsuan-Rong Huang (5), Yu-Shu Huang (3,4,5) Keywords: Psychosocial adjustment; Quality of life; Children; Cancer; Stress Abstract: Purpose To evaluate caregiver-reported psychosocial adjustment and health-related quality of life (HrQoL) of Taiwanese children with newly diagnosed cancer and their caregivers during the first 6 months of treatment. Methods Caregivers of 89 newly diagnosed children completed the child behavior checklist, the pediatric quality of life inventory (PedsQL.sup.[TM] 4.0), the Parenting Stress Index, and the SF-36 questionnaire at diagnosis, and again 3 and 6 months into treatment. They were compared with a group of age- and sex-matched controls from general community. Results Significantly worse HrQoL in both children and their caregivers and greater parenting stress were noted in the cancer group than the controls during the first 6 months. Children with cancer were found to have significantly more internalizing behavioral problems and somatic complaints, especially those younger than 12 years old. After starting chemotherapy, significant decrease in parenting stress and improvements of both caregivers and children's HrQoL were noted within the first 6 months, although not to the level comparable with normal controls. Conclusions Although children and their caregivers can adjust themselves gradually during the first 6 months after diagnosis of cancer, intervention and efforts aimed at reducing their distress and promoting adjustments are still required during this period. Author Affiliation: (1) Division of Pediatric Hematology/Oncology, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan (2) Division of Child Psychiatry, Chang Gung Memorial Hospital, Kaohsiung, Taiwan (3) Division of Pediatric Psychiatry, Chang Gung Memorial Hospital, Taoyuan, Taiwan (4) Department of Child Psychiatry and Sleep Center, Chang Gung Memorial Hospital, 5, Fu-Shing St., Kwei-Shan, Taoyuan, 333, Taiwan (5) College of Medicine, Chang Gung University, Taoyuan, Taiwan (6) Chang Gung Institute of Technology, Chiayi, Taiwan Article History: Registration Date: 02/04/2012 Accepted Date: 02/04/2012 Online Date: 22/04/2012
    Keywords: Psychosocial adjustment ; Quality of life ; Children ; Cancer ; Stress
    ISSN: 0962-9343
    E-ISSN: 1573-2649
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  • 3
    Language: English
    In: International Journal of Hematology, 2013, Vol.97(1), pp.144-146
    Description: In the present study, we describe unrelated umbilical cord blood transplantation (CBT) in a 7-year-old Taiwanese boy with atypical chronic myeloid leukemia (BCR-ABL 1 negative). Physical examination was notable for splenomegaly. Cytogenetic analyses from the bone marrow revealed a t(3;5)(p21;q31) translocation. The patient then underwent CBT from an HLA-mismatched (two loci by serotype, three loci by genotype) unrelated donor of Caucasian origin. Times to neutrophil and platelet engraftment were 21 and 62 days post-transplant, respectively. Acute graft-versus-host disease following transplantation was minimal. The patient remains in continuing hematological remission with full donor chimerism 28 months after transplantation.
    Keywords: Unrelated cord blood transplantation ; Atypical chronic myeloid leukemia ; Cytogenetic analyses
    ISSN: 0925-5710
    E-ISSN: 1865-3774
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  • 4
    Language: English
    In: Pediatrics & Neonatology, October 2017, Vol.58(5), pp.462-464
    Description: To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.pedneo.2016.09.005 Byline: Tsung-Yen Chang, Tang-Her Jaing, Shih-Hsiang Chen Author Affiliation: Division of Hematology/Oncology, Department of Pediatrics, Linkou Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan Article History: Received 21 June 2016; Revised 22 August 2016; Accepted 12 September 2016
    Keywords: Medicine
    ISSN: 1875-9572
    E-ISSN: 22121692
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  • 5
    Language: English
    In: PLoS ONE, 01 January 2013, Vol.8(10), p.e76711
    Description: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, virus-triggered immune disease. Hypersensitivity to mosquito bite (HMB), a presentation of Chronic Active Epstein-Barr Virus infection (CAEBV), may progress to HLH. This study aimed to investigate the immunologic difference between...
    Keywords: Sciences (General)
    E-ISSN: 1932-6203
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  • 6
    Language: English
    In: International Journal of Clinical Oncology, April, 2012, Vol.17(2), p.124(7)
    Description: Byline: Shih-Hsiang Chen (1), Chao-Ping Yang (1), Tang-Her Jaing (1), Jin-Yao Lai (2), Iou-Jih Hung (1) Keywords: Central venous catheter; Catheter-related bloodstream infection; Pediatric oncology; Removal Abstract: Background Long-term central venous catheter (CVC) implantation has become more affordable in Taiwan since 1995. Surgical removal of the catheter may be the essential treatment for catheter-related bloodstream infections (CRBSI). The aim of this study was to evaluate the clinical features and microbial isolates in pediatric cancer patients with removal of CVC for CRBSI. Procedure The records of positive blood culture from hospitalized pediatric oncology patients between 1995 and 2004 were reviewed. One hundred and forty-three patients implanted with a long-term CVC were further identified. Results Seventeen catheters in 16 patients developed catheter-related bacteremia that needed catheter removal. The rate of catheter removal was 11.9%. The median device life was 7.7 months. Six catheters were removed within 3 months of insertion. Nine of the 17 catheters were removed from patient younger than 2 years. Eight infections occurred during severe neutropenia, and 6 patients had refractory or relapsed underlying disease. The cultural isolates were Gram-negative bacilli in 7, Gram-positive in 5, fungi in 5, and atypical mycobacterium in 1. The frequency of catheter removal for infection control was significantly higher in the first 5 years (1994--1999) compared to the last 5 years (2000--2004) (30.9 vs. 4.0%, p = 2.3 x 10.sup.-4). Conclusions Factors such as microbiological isolates, age of infection, the status of malignancy, and neutropenia are related to catheter outcome. The reduction in patients with positive cultures needing removal of the catheters can be related to improved nursing care and more aggressive antibiotic therapy. Author Affiliation: (1) Division of Pediatric Hematology/Oncology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, 5 Fu-Shin Street, Kuei Shan 333, Taoyuan, Taiwan (2) Division of Pediatric Surgery, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan Article History: Registration Date: 02/06/2011 Received Date: 02/03/2011 Accepted Date: 30/05/2011 Online Date: 18/06/2011
    Keywords: Pediatrics -- Health Aspects ; Blood Tests -- Health Aspects ; Nursing -- Health Aspects ; Cancer Research -- Health Aspects ; Catheterization -- Health Aspects ; Medical Schools -- Health Aspects
    ISSN: 1341-9625
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  • 7
    Language: English
    In: International Journal of Hematology, 2015, Vol.102(1), pp.48-52
    Description: Overweight/obese patients with acute myeloid leukemia (AML) are reported as experiencing inferior outcomes and greater numbers of treatment-related complications. We retrospectively studied 58 children with newly diagnosed AML who received chemotherapy at Chang Gung Memorial Hospital between January 2003 and December 2011. Patients enrolled were considered overweight if body mass index (BMI) was ≥85th percentile. Fifteen of 58 (25.9 %) patients were judged overweight by this criterion. Patients diagnosed in the last third of this period (2009–2011) had a higher average BMI ( P  = 0.06). The rates of documented infection in overweight patients and non-overweight patients were not significantly different (53.3 vs. 62.8 %). The 5-year event-free survival (EFS) of overweight patients was superior to that of non-overweight patients (78.8 vs. 55.4 %). Patients ( n  = 11) who received hematopoietic stem cell transplantation (HSCT) in first remission (CR1) had a significantly higher 5-year EFS (87.5 vs. 55.2 %, P  = 0.04). Among 47 children who did not receive HSCT in CR1, 10 (21.3 %) were overweight. The 5-year EFS of overweight patients was consistently superior to that for non-overweight patients (70.0 vs. 51.2 %). In conclusion, overweight/obese children with AML did not experience poor outcomes in the present study.
    Keywords: Acute myeloid leukemia ; Children ; BMI ; Obese ; Overweight
    ISSN: 0925-5710
    E-ISSN: 1865-3774
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  • 8
    Language: English
    In: Journal of Pain and Symptom Management, February 2014, Vol.47(2), pp.271-282
    Description: Since the development of palliative care in the 1980s, “do not resuscitate” (DNR) has been promoted worldwide to avoid unnecessary resuscitation in terminally ill cancer patients. This study aimed to evaluate the effect of a palliative care consultation service (PCCS) on DNR designation and to identify a subgroup of patients who would potentially benefit from care by the PCCS with respect to DNR designation. In total, 2995 terminally ill cancer patients (with a predicted life expectancy of less than six months by clinician estimate) who received care by the PCCS between January 2006 and December 2010 at a single medical center in Taiwan were selected. Among these, the characteristics of 2020 (67.4%) patients who were not designated as DNR at the beginning of care by the PCCS were retrospectively analyzed to identify variables pertinent to DNR designation. A total of 1301 (64%) of 2020 patients were designated as DNR at the end of care by the PCCS. Male gender and primary liver cancer were characteristics more predominantly found among DNR-designated patients who also had worse performance status, higher prevalence of physical distress, and shorter intervals from palliative care referral to death than did patients without DNR designation. On univariate analysis, a higher probability of DNR designation was associated with male gender, duration of care by the PCCS of more than 14 days, patients' prognostic awareness, family's diagnostic and prognostic awareness, and high Palliative Prognostic Index (PPI) scores. On multivariate analysis, duration of care by the PCCS, patients' prognostic awareness, family's diagnostic and prognostic awareness, and a high PPI score constituted independent variables predicting DNR-designated patients at the end of care by the PCCS. DNR designation was late in terminally ill cancer patients. DNR-designated cancer patient indicators were high PPI scores, patients' prognostic awareness, family's diagnostic and prognostic awareness, and longer durations of care by the PCCS.
    Keywords: Terminal Cancer ; Palliative Care ; Do-Not-Resuscitate ; Palliative Prognostic Index ; Medicine
    ISSN: 0885-3924
    E-ISSN: 1873-6513
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  • 9
    Language: English
    In: Journal of Clinical Immunology, 2010, Vol.30(4), pp.593-601
    Description: Byline: Wen-I Lee (1,2,3), Jing-Long Huang (2,3), Tang-Her Jaing (5), Kang-Hsi Wu (4), Yin-Hsiu Chien (6), Kuei-Wen Chang (7) Keywords: Wiskott--Aldrich Syndrome (WAS); X-linked thrombocytopenia (XLT); primary immunodeficiency diseases (PIDs); Taiwan; Chinese; genetic analysis Abstract: Background Wiskott--Aldrich syndrome (WAS) is an X-linked immunodeficiency characterized by microthrombocytopenia, eczema, and recurrent infections. However, the more than 500 patient mutations described are mainly based on Caucasian and Japanese populations. This study investigated Taiwanese patients with WASP mutations since 1985 as part of a long-term comprehensive study in primary immunodeficiency diseases (PIDs) covering 23 million inhabitants. Methods Clinical manifestations, immunologic functions, and WASP gene sequencing and expressions were analyzed in WAS patients. And, those patients with idiopathic thrombocytopenic purpura and "small" thrombocytopenia were enrolled. Results Of 16 patients studied in 1993--2009, 12 presented as classic WAS phenotype and four had X-linked thrombocytopenia (XLT). Almost all correlated to the WASP expression level and severity of infections. Causes of mortality in the 12 classic WAS patients were mass bleeding, Staphylococcus aureus sepsis, and cytomegalovirus (CMV) pneumonitis in three non-transplant cases, and EBV-associated lymphoproliferative disorder and CMV pneumonitis in two non-engrafted transplant patients. Splicing mutations of Int 8 (+5) G〉A in cousins and insertion of 1023 C in unrelated families presented as both XLT and classic WAS phenotype in the same mutations. Four XLT patients, including two novel mutations of 1023 Ins C (in 2) and "double" missense mutations of 1378 C〉T and 1421 T〉C had relatively higher CD4+ memory cells and/or activated lymphocytes (CD3+CD69+) compared with those of classic WAS patients. Conclusions The lower ratio of XLT to classic WAS patients underestimates the burden of Taiwanese patients with WASP mutations, especially the XLT phenotype. A clustering pattern on exon 1 and five unique mutations (deletion of 45-48 ACCA, IVS 1 (-1) G〉C, large deletion of promoter and exon 1 and 2, insertion 1023 C, and 1378 C〉T and 1421 T〉C) explain the genetic variations in different ethnic groups, despite the possibility of selection and ascertainment bias. Author Affiliation: (1) Primary Immunodeficiency Care and Research (PICAR) Institute, Chang Gung Memorial & Children's Hospital and University College of Medicine, 5 Fu-Shing St. (Pediatric Office 12 L), Kwei-Shan, Taoyuan, Taiwan (2) Primary Immunodeficiency Care and Research (PICAR) Institute, Chang Gung Memorial & Children's Hospital and University College of Medicine, Taoyuan, Taiwan (3) Department of Pediatric Allergy, Immunology and Rheumatology, Chang Gung Memorial & Children's Hospital and University College of Medicine, Taoyuan, Taiwan (4) Department of Pediatric Hematology and Oncology, China Medical University, Taichung, Taiwan (5) Department of Pediatric Hematology and Oncology, Chang Gung Memorial & Children's Hospital and University College of Medicine, Taoyuan, Taiwan (6) Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan (7) Department of Pediatric Gastroenterology Chang Gung Memorial & Children's Hospital and University College of Medicine, Taoyuan, Taiwan Article History: Registration Date: 11/02/2010 Received Date: 14/01/2010 Accepted Date: 10/02/2010 Online Date: 16/03/2010 Article note: Disclosure This study was supported by grants from the Chang-Gung Medical Research Progress (CMRPG 32069 and 450021) and the National Science Council (96-2314-B-182A-053-MY2).
    Keywords: Wiskott–Aldrich Syndrome (WAS) ; X-linked thrombocytopenia (XLT) ; primary immunodeficiency diseases (PIDs) ; Taiwan ; Chinese ; genetic analysis
    ISSN: 0271-9142
    E-ISSN: 1573-2592
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  • 10
    Language: English
    In: International Journal of Hematology, 2015, Vol.102(1), pp.41-47
    Description: Osteonecrosis (ON) is a potentially disabling complication encountered in children who receive chemotherapy for acute lymphoblastic leukemia (ALL). Considering the possible effect of ethnic difference on the clinical features of symptomatic ON in pediatric ALL, we retrospectively evaluated 245 children with ALL who were treated at Chang Gung Memorial Hospital, Linkou, between 2002 and 2011. Six (2.4 %) patients developed symptomatic ON in a total of 17 sites during the follow-up period. Diagnosis of ON was confirmed by X-ray in seven, magnetic resonance imaging in two, and bone scan in three patients. The estimated cumulative incidence of symptomatic ON in newly diagnosed ALL was 3.4 % at 8 years. Four patients received ON-directed surgical interventions, including total hip replacement in three and arthroplasty in one. The incidence of ON was significantly higher among girls ( P  = 0.03), patients 〉10 years old ( P  = 2.2 × 10 −4 ), and patients who had received more intensive chemotherapy regimen ( P  = 0.02). These results indicate that the incidence and risk factors in our institute were similar to those observed in Western countries. Future studies surveying the impact on the quality of life of childhood ALL survivors in Taiwan are warranted.
    Keywords: Acute lymphoblastic leukemia ; Chemotherapy ; Children ; Complication ; Osteonecrosis
    ISSN: 0925-5710
    E-ISSN: 1865-3774
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