Sy Ha

Arterial stiffness and endothelial function in patients with β-thalassemia major

BACKGROUND Increased iron store has been linked to risk of cardiovascular disease. Structural alterations of arteries in beta-thalassemia major patients and in vitro functional disturbance of vascular endothelial cells by thalassemic serum have been described. We sought to determine whether arterial stiffness and endothelial function are altered in vivo. METHODS AND RESULTS Thirty thalassemia patients (16 male) aged 22.2+/-7.4 years were recruited. Left ventricular (LV) mass and function were assessed echocardiographically. Carotid and brachioradial artery stiffness was assessed by stiffness index and pulse-wave velocity (PWV), respectively. Brachial artery endothelial function was assessed by vascular response to reactive hyperemia (flow-mediated dilation [FMD]) and sublingual glyceryl trinitrate. These indexes were compared with those of 30 age- and sex-matched controls. None of the patients had LV systolic or diastolic dysfunction. When compared with controls, patients had greater absolute (113.8+/-38.0 versus 109.0+/- 32.6 g, P=0.04) and indexed (82.4+/-17.5 versus 66.7+/-12.7 g/m(2), P<0.001) LV mass, carotid artery stiffness index (8.1+/-3.5 versus 5.5+/-1.6, P<0.001), and brachioradial PWV (8.9+/-2.4 versus 7.9+/-1.7 m/s, P= 0.03). Their FMD was impaired (3.5+/-3.3% versus 8.8+/-3.9%, P<0.001), whereas glyceryl trinitrate- mediated dilation was preserved (17.9+/-7.6% versus 16.3+/-6.1%, P=0.40). Both stiffness index and PWV correlated inversely with magnitude of FMD (r=-0.40, P=0.03; r=-0.41, P=0.03) and positively with indexed LV mass (r=0.50, P=0.005; r=0.40, P=0.027). Nonetheless, no significant correlation existed between ferritin level and carotid stiffness, PWV, or FMD. CONCLUSIONS Increased arterial stiffness, endothelial dysfunction, and LV hypertrophy occur in patients with beta-thalassemia major, which may result in reduction of mechanical efficiency of the heart.

Analysis of MLL-Derived Transcripts in Infant Acute Monocytic Leukemia with a Complex Translocation (1;11;4)(q21;q23;p16)

It has been proposed, on the basis of cytogenetic studies and molecular analysis of MLL-derived transcripts in acute leukemia with 11q23 rearrangement, that only one fusion gene transcript present on the der(11) chromosome is critical for leukemogenesis. This view is challenged by a recent observation in a case of leukemia with a complex translocation that results in MLL being fused in-frame to two different partner genes. We investigated a case of infant monocytic leukemia with a complex translocation, (1;11;4)(q21;q23;p16). Molecular studies revealed MLL rearrangement by both fluorescence in situ hybridization and Southern blot analysis, and MLL/AF1q, but not the reciprocal message (i.e., AF1q/MLL), was amplified by polymerase chain reaction. Sequence analysis of MLL/AF1q revealed an in-frame fusion between MLL exon 6 and the breakpoint located six bases upstream of the ATG start site for AF1q. Our data suggest that only one form of MLL fusion gene is implicated in leukemogenesis in our case to t(1;11;4).

MR Quantitative Susceptibility Imaging for the Evaluation of Iron Loading in the Brains of Patients with β-Thalassemia Major

BACKGROUND AND PURPOSE: Patients with β-thalassemia require blood transfusion to prolong their survival, which could cause iron overload in multiple organs, including the heart, liver, and brain. In this study, we aimed to quantify iron loading in the brains of patients with β-thalassemia major through the use of MR quantitative susceptibility imaging. MATERIALS AND METHODS: Thirty-one patients with thalassemia with a mean (± standard deviation) age of 25.3 (±5.9) years and 33 age-matched healthy volunteers were recruited and underwent MR imaging at 3T. Quantitative susceptibility images were reconstructed from a 3D gradient-echo sequence. Susceptibility values were measured in the caudate nucleus, putamen, globus pallidus, red nucleus, substantia nigra, dentate nucleus, and choroid plexus. General linear model analyses were performed to compare susceptibility values of different ROIs between the patients with thalassemia and healthy volunteers. RESULTS: Of the 31 patients, 27 (87.1%) had abnormal iron deposition in one of the ROIs examined. Significant positive age effect on susceptibility value was found in the putamen, dentate nucleus, substantia nigra, and red nucleus (P = .002, P = .017, P = .044, and P = .014, respectively) in the control subjects. Compared with healthy control subjects, patients with thalassemia showed significantly lower susceptibility value in the globus pallidus (P < .001) and substantia nigra (P = .003) and significantly higher susceptibility value in the red nucleus (P = .021) and choroid plexus (P < .001). CONCLUSIONS: A wide range of abnormal susceptibility values, indicating iron overloading or low iron content, was found in patients with thalassemia. MR susceptibility imaging is a sensitive method for quantifying iron concentration in the brain and can be used as a potentially valuable tool for brain iron assessment.

Epidemiology and outcome of post-transplant iymphproliferative disorder in pediatric liver transplant recipients

This free journal suppl. entitled: Special Issue: Fifth International Symposium on Childhood, Adolescent and Young Adult Non-Hodgkin Lymphoma Abstracts, 22 October 2015, Varese, ItalyThis free journal suppl. entitled: Special Issue: Fifth International Symposium on Childhood, Adolescent and Young Adult Non-Hodgkin Lymphoma Abstracts ... 2015

Epidemiology and outcome of post-transplant lymphoproliferative disorders in pediatric liver transplant receipient

This free journal suppl. entitled: Special Issue: Fifth International Symposium on Childhood, Adolescent and Young Adult Non-Hodgkin Lymphoma Abstracts, 22 October 2015, Varese, ItalyThis free journal suppl. entitled: Special Issue: Fifth International Symposium on Childhood, Adolescent and Young Adult Non-Hodgkin Lymphoma Abstracts ... 2015

Treatment of children with drug-resistant Graft-Versus-Host-Disease by extracorporeal photopheresis

PHYSICAL ACTIVITY THROUGH HOME-BASED EXERCISE-GAMING AFTER CHILDHOOD BRAIN TUMOUR TREATMENT- A FEASABILITY STUDYCONTENTS: Oral presentations (O + number) Poster presentations (P + number) O-0001 O-0004 Neuroblastoma I P-0001 P-0071 Acute Lymphoblastic Leukaemia O-0005 O-0008 Epidemiology P-0072 P-0102 Myeloid leukemias O-0009 O-0012 Rare Tumours I P-0103 P-0132 Lymphomas O-0013 O-0018 Leukemia I P-0133 P-0162 Bone tumours O-0019 O-0024 Psychosocial I P-0163 P-0182 Soft tissue sarcomas O-0025 O-0030 Rare Tumours II P-0183 P-0201 Renal tumours O-0031 O-0036 Leukemia II P-0202 P-0208 Germ cell tumours O-0037 O-0042 Late Effects P-0209 P-0225 Rare tumours O-0043 O-0048 Brain Tumours I P-0226 P-0238 Liver tumours O-0049 O-0054 SIOP Award Session P-0239 P-0287 Neuroblastoma O-0055 O-0058 Germ Cell Tumours P-0288 P-0303 Surgery (IPSO) O-0059 O-0062 Liver Tumours P-0304 P-0305 Radiation Oncology (PROS) O-0063 O-0066 Rare Tumours II P-0306 P-0322 Retinoblastoma O-0066 O-0072 Brain Tumours II P-0323 P-0377 Brain tumours O-0073 O-0078 Psychosocial II P-0378 P-0418 Others O-0079 O-0084 Renal and Sarcomas I P-0419 P-0426 Hystiocytosis O-0085 O-0090 Neuroblastoma II P-0427 P-0448 New drugs/experimental therapeutics O-0091 O-0096 Bone tumours P-0449 P-0458 Epidemiology O-0097 O-0102 Supportive Care P-0459 P-0486 Late effects O-0103 O-0107 Leukemia III P-0487 P-0529 Supportive care/Palliative care O-0108 O-0113 Neuroblastoma III P-0530 P-0548 Psychosocial O-0114 O-0119 Renal and Sarcomas II P-0549 P-0574 Nursing O-0120 O-0125 Lymphoma O-0126 O-0131 Brain tumours III O-0132 O-0137 Renal and Sarcomas III O-0138 O-0163 Nurses Publications (Pub + number) O-0164 O-0196 IPSO O-0197 O-0212 ICCCPOParents/Survivors Pub-0001 Pub-0269 Publication Only 2013 Wiley Periodicals, Inc. DOI 10.1002/pbc.24719 Published online in Wiley Online Library (wileyonlinelibrary.com.). 45 CONGRESS OF THE INTERNATIONAL SOCIETY OF PAEDIATRIC ONCOLOGY (SIOP) 2013 HONG KONG, CHINA, 25–28 SEPTEMBER, 2013 SIOP ABSTRACTS FREE PAPER SESSION: FREE PAPER SESSION 01 NEUROBLASTOMA I

Outcome of hematopoietic stem cell transplantation In refractory/relapsed infant leukemia

PHYSICAL ACTIVITY THROUGH HOME-BASED EXERCISE-GAMING AFTER CHILDHOOD BRAIN TUMOUR TREATMENT- A FEASABILITY STUDYCONTENTS: Oral presentations (O + number) Poster presentations (P + number) O-0001 O-0004 Neuroblastoma I P-0001 P-0071 Acute Lymphoblastic Leukaemia O-0005 O-0008 Epidemiology P-0072 P-0102 Myeloid leukemias O-0009 O-0012 Rare Tumours I P-0103 P-0132 Lymphomas O-0013 O-0018 Leukemia I P-0133 P-0162 Bone tumours O-0019 O-0024 Psychosocial I P-0163 P-0182 Soft tissue sarcomas O-0025 O-0030 Rare Tumours II P-0183 P-0201 Renal tumours O-0031 O-0036 Leukemia II P-0202 P-0208 Germ cell tumours O-0037 O-0042 Late Effects P-0209 P-0225 Rare tumours O-0043 O-0048 Brain Tumours I P-0226 P-0238 Liver tumours O-0049 O-0054 SIOP Award Session P-0239 P-0287 Neuroblastoma O-0055 O-0058 Germ Cell Tumours P-0288 P-0303 Surgery (IPSO) O-0059 O-0062 Liver Tumours P-0304 P-0305 Radiation Oncology (PROS) O-0063 O-0066 Rare Tumours II P-0306 P-0322 Retinoblastoma O-0066 O-0072 Brain Tumours II P-0323 P-0377 Brain tumours O-0073 O-0078 Psychosocial II P-0378 P-0418 Others O-0079 O-0084 Renal and Sarcomas I P-0419 P-0426 Hystiocytosis O-0085 O-0090 Neuroblastoma II P-0427 P-0448 New drugs/experimental therapeutics O-0091 O-0096 Bone tumours P-0449 P-0458 Epidemiology O-0097 O-0102 Supportive Care P-0459 P-0486 Late effects O-0103 O-0107 Leukemia III P-0487 P-0529 Supportive care/Palliative care O-0108 O-0113 Neuroblastoma III P-0530 P-0548 Psychosocial O-0114 O-0119 Renal and Sarcomas II P-0549 P-0574 Nursing O-0120 O-0125 Lymphoma O-0126 O-0131 Brain tumours III O-0132 O-0137 Renal and Sarcomas III O-0138 O-0163 Nurses Publications (Pub + number) O-0164 O-0196 IPSO O-0197 O-0212 ICCCPOParents/Survivors Pub-0001 Pub-0269 Publication Only 2013 Wiley Periodicals, Inc. DOI 10.1002/pbc.24719 Published online in Wiley Online Library (wileyonlinelibrary.com.). 45 CONGRESS OF THE INTERNATIONAL SOCIETY OF PAEDIATRIC ONCOLOGY (SIOP) 2013 HONG KONG, CHINA, 25–28 SEPTEMBER, 2013 SIOP ABSTRACTS FREE PAPER SESSION: FREE PAPER SESSION 01 NEUROBLASTOMA I

Treatment outcome of post-transplant lymphoproliferative disorder in paediatric liver transplant recipients using rituximab-based regimen

PHYSICAL ACTIVITY THROUGH HOME-BASED EXERCISE-GAMING AFTER CHILDHOOD BRAIN TUMOUR TREATMENT- A FEASABILITY STUDYCONTENTS: Oral presentations (O + number) Poster presentations (P + number) O-0001 O-0004 Neuroblastoma I P-0001 P-0071 Acute Lymphoblastic Leukaemia O-0005 O-0008 Epidemiology P-0072 P-0102 Myeloid leukemias O-0009 O-0012 Rare Tumours I P-0103 P-0132 Lymphomas O-0013 O-0018 Leukemia I P-0133 P-0162 Bone tumours O-0019 O-0024 Psychosocial I P-0163 P-0182 Soft tissue sarcomas O-0025 O-0030 Rare Tumours II P-0183 P-0201 Renal tumours O-0031 O-0036 Leukemia II P-0202 P-0208 Germ cell tumours O-0037 O-0042 Late Effects P-0209 P-0225 Rare tumours O-0043 O-0048 Brain Tumours I P-0226 P-0238 Liver tumours O-0049 O-0054 SIOP Award Session P-0239 P-0287 Neuroblastoma O-0055 O-0058 Germ Cell Tumours P-0288 P-0303 Surgery (IPSO) O-0059 O-0062 Liver Tumours P-0304 P-0305 Radiation Oncology (PROS) O-0063 O-0066 Rare Tumours II P-0306 P-0322 Retinoblastoma O-0066 O-0072 Brain Tumours II P-0323 P-0377 Brain tumours O-0073 O-0078 Psychosocial II P-0378 P-0418 Others O-0079 O-0084 Renal and Sarcomas I P-0419 P-0426 Hystiocytosis O-0085 O-0090 Neuroblastoma II P-0427 P-0448 New drugs/experimental therapeutics O-0091 O-0096 Bone tumours P-0449 P-0458 Epidemiology O-0097 O-0102 Supportive Care P-0459 P-0486 Late effects O-0103 O-0107 Leukemia III P-0487 P-0529 Supportive care/Palliative care O-0108 O-0113 Neuroblastoma III P-0530 P-0548 Psychosocial O-0114 O-0119 Renal and Sarcomas II P-0549 P-0574 Nursing O-0120 O-0125 Lymphoma O-0126 O-0131 Brain tumours III O-0132 O-0137 Renal and Sarcomas III O-0138 O-0163 Nurses Publications (Pub + number) O-0164 O-0196 IPSO O-0197 O-0212 ICCCPOParents/Survivors Pub-0001 Pub-0269 Publication Only 2013 Wiley Periodicals, Inc. DOI 10.1002/pbc.24719 Published online in Wiley Online Library (wileyonlinelibrary.com.). 45 CONGRESS OF THE INTERNATIONAL SOCIETY OF PAEDIATRIC ONCOLOGY (SIOP) 2013 HONG KONG, CHINA, 25–28 SEPTEMBER, 2013 SIOP ABSTRACTS FREE PAPER SESSION: FREE PAPER SESSION 01 NEUROBLASTOMA I

Acute lymphoblastic leukaemia (ALL) in Hong Kong children: treatment outcome from 1993 to 2012

PHYSICAL ACTIVITY THROUGH HOME-BASED EXERCISE-GAMING AFTER CHILDHOOD BRAIN TUMOUR TREATMENT- A FEASABILITY STUDYCONTENTS: Oral presentations (O + number) Poster presentations (P + number) O-0001 O-0004 Neuroblastoma I P-0001 P-0071 Acute Lymphoblastic Leukaemia O-0005 O-0008 Epidemiology P-0072 P-0102 Myeloid leukemias O-0009 O-0012 Rare Tumours I P-0103 P-0132 Lymphomas O-0013 O-0018 Leukemia I P-0133 P-0162 Bone tumours O-0019 O-0024 Psychosocial I P-0163 P-0182 Soft tissue sarcomas O-0025 O-0030 Rare Tumours II P-0183 P-0201 Renal tumours O-0031 O-0036 Leukemia II P-0202 P-0208 Germ cell tumours O-0037 O-0042 Late Effects P-0209 P-0225 Rare tumours O-0043 O-0048 Brain Tumours I P-0226 P-0238 Liver tumours O-0049 O-0054 SIOP Award Session P-0239 P-0287 Neuroblastoma O-0055 O-0058 Germ Cell Tumours P-0288 P-0303 Surgery (IPSO) O-0059 O-0062 Liver Tumours P-0304 P-0305 Radiation Oncology (PROS) O-0063 O-0066 Rare Tumours II P-0306 P-0322 Retinoblastoma O-0066 O-0072 Brain Tumours II P-0323 P-0377 Brain tumours O-0073 O-0078 Psychosocial II P-0378 P-0418 Others O-0079 O-0084 Renal and Sarcomas I P-0419 P-0426 Hystiocytosis O-0085 O-0090 Neuroblastoma II P-0427 P-0448 New drugs/experimental therapeutics O-0091 O-0096 Bone tumours P-0449 P-0458 Epidemiology O-0097 O-0102 Supportive Care P-0459 P-0486 Late effects O-0103 O-0107 Leukemia III P-0487 P-0529 Supportive care/Palliative care O-0108 O-0113 Neuroblastoma III P-0530 P-0548 Psychosocial O-0114 O-0119 Renal and Sarcomas II P-0549 P-0574 Nursing O-0120 O-0125 Lymphoma O-0126 O-0131 Brain tumours III O-0132 O-0137 Renal and Sarcomas III O-0138 O-0163 Nurses Publications (Pub + number) O-0164 O-0196 IPSO O-0197 O-0212 ICCCPOParents/Survivors Pub-0001 Pub-0269 Publication Only 2013 Wiley Periodicals, Inc. DOI 10.1002/pbc.24719 Published online in Wiley Online Library (wileyonlinelibrary.com.). 45 CONGRESS OF THE INTERNATIONAL SOCIETY OF PAEDIATRIC ONCOLOGY (SIOP) 2013 HONG KONG, CHINA, 25–28 SEPTEMBER, 2013 SIOP ABSTRACTS FREE PAPER SESSION: FREE PAPER SESSION 01 NEUROBLASTOMA I

Comparison of two consecutive cohorts of childhood acute myeloid leukemia in Hong Kong

PHYSICAL ACTIVITY THROUGH HOME-BASED EXERCISE-GAMING AFTER CHILDHOOD BRAIN TUMOUR TREATMENT- A FEASABILITY STUDYCONTENTS: Oral presentations (O + number) Poster presentations (P + number) O-0001 O-0004 Neuroblastoma I P-0001 P-0071 Acute Lymphoblastic Leukaemia O-0005 O-0008 Epidemiology P-0072 P-0102 Myeloid leukemias O-0009 O-0012 Rare Tumours I P-0103 P-0132 Lymphomas O-0013 O-0018 Leukemia I P-0133 P-0162 Bone tumours O-0019 O-0024 Psychosocial I P-0163 P-0182 Soft tissue sarcomas O-0025 O-0030 Rare Tumours II P-0183 P-0201 Renal tumours O-0031 O-0036 Leukemia II P-0202 P-0208 Germ cell tumours O-0037 O-0042 Late Effects P-0209 P-0225 Rare tumours O-0043 O-0048 Brain Tumours I P-0226 P-0238 Liver tumours O-0049 O-0054 SIOP Award Session P-0239 P-0287 Neuroblastoma O-0055 O-0058 Germ Cell Tumours P-0288 P-0303 Surgery (IPSO) O-0059 O-0062 Liver Tumours P-0304 P-0305 Radiation Oncology (PROS) O-0063 O-0066 Rare Tumours II P-0306 P-0322 Retinoblastoma O-0066 O-0072 Brain Tumours II P-0323 P-0377 Brain tumours O-0073 O-0078 Psychosocial II P-0378 P-0418 Others O-0079 O-0084 Renal and Sarcomas I P-0419 P-0426 Hystiocytosis O-0085 O-0090 Neuroblastoma II P-0427 P-0448 New drugs/experimental therapeutics O-0091 O-0096 Bone tumours P-0449 P-0458 Epidemiology O-0097 O-0102 Supportive Care P-0459 P-0486 Late effects O-0103 O-0107 Leukemia III P-0487 P-0529 Supportive care/Palliative care O-0108 O-0113 Neuroblastoma III P-0530 P-0548 Psychosocial O-0114 O-0119 Renal and Sarcomas II P-0549 P-0574 Nursing O-0120 O-0125 Lymphoma O-0126 O-0131 Brain tumours III O-0132 O-0137 Renal and Sarcomas III O-0138 O-0163 Nurses Publications (Pub + number) O-0164 O-0196 IPSO O-0197 O-0212 ICCCPOParents/Survivors Pub-0001 Pub-0269 Publication Only 2013 Wiley Periodicals, Inc. DOI 10.1002/pbc.24719 Published online in Wiley Online Library (wileyonlinelibrary.com.). 45 CONGRESS OF THE INTERNATIONAL SOCIETY OF PAEDIATRIC ONCOLOGY (SIOP) 2013 HONG KONG, CHINA, 25–28 SEPTEMBER, 2013 SIOP ABSTRACTS FREE PAPER SESSION: FREE PAPER SESSION 01 NEUROBLASTOMA I