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World Journal of Gastroenterology 2008年第18期14卷 2939-2941页

作者： anselm Chi-Wai lee Kin-Hung Poon Wing-Hong Lo Lap-Gate Wong Department of Pediatrics & Adolescent Medicine Tuen Mun Hospital New Territories Hong Kong 999077 China Department of Clinical Pathology Tuen Mun Hospital New Territories Hong Kong 999077 China

A 10-year-old Chinese boy who had a history of congenital thrombocytopathy presented with severe iron deficiency anemia secondary to chronic gastric inflammation and duodenal ulcerations. Subtle oculocutaneous albinism led to the finding of diminished dense bodies in the platelets under electron microscopy, hence the diagnosis of Hermansky-Pudlak syndrome (HPS). Biopsies from the stomach and duodenum revealed a lymphocytic infiltration in the submucosa, but H pylori infection was absent. The gastroduodenitis responded to the treatment with omeprazole while iron deficiency anemia was corrected by oral iron therapy. HPS is a rare cause of congenital bleeding disorder with multisystemic manifestations. Upper gastrointestinal involvement is rare and should be distinguished from a mere manifestation of the bleeding diathesis.

关键词： Albinism Duodenal ulceration Hermansky-Pudlak syndrome Inflammatory bowel disease Thrombocytopathy

Management of thoracoabdominal neuroblastoma:a 13-year experience

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World Journal of Pediatric Surgery 2019年第2期2卷 47-52页

作者： Chan Hon Chui anselm lee Surgery Centre for Children SingaporeSingapore Children's Haematology and Cancer Centre SingaporeSingapore

background Thoracoabdominal neuroblastoma is a unique surgical *** reviewed our experience focusing on disease patterns and corresponding surgical *** Among 310 patients in our neuroblastoma database,30(9.7%)had thoracoabdominal ***’clinical charts were reviewed and *** disease patterns were identified:solitary thoracoabdominal tumor(group A,n=15)and multifocal tumors in thorax and abdomen(group B,n=15).Operative approaches were categorized based on routes of surgical *** Thirty patients with average age of 4.1(range 0.8-12.8)years were *** received preoperative *** 15 group A patients,four were stage 3 intermediate risk(IR)and 11 were stage 4 high risk(HR).Surgical approaches included single-incision thoracoabdominal approach(n=10),laparotomy-cum-transdiaphragmatic approach(n=3),and laparotomy-cum-thoracotomy approach(n=2).One patient had 10%residual disease and the rest achieved gross total *** complications included chylous ascites(n=3),intestinal obstruction(n=3),pneumonia(n=1),spinal cord infarction(n=1)scoliosis(n=2)and thoracoabdominal nerves palsy(n=3).Among 15 group B patients,all were stage 4 with five IR and 10 *** components were found in the posterior mediastinum(n=7),superior mediastinum(n=4),middle mediastinum(n=1),parietal pleura(n=3)and lungs(n=3).Surgical approaches included multi-incision laparotomy-cum-thoracotomy(n=14)and laparotomy-cum-transdiaphragmatic approach(n=1).Gross total resection was achieved in all *** complications included chylous ascites(n=3).Overall,all nine IR patients survived without evidence of disease and 9(42.8%)HR patients died of *** was no perioperative *** Surgical resection of thoracoabdominal neuroblastoma is feasible and *** its complexity,thoracoabdominal neuroblastoma has comparable treatment outcomes when compared with single-compartmental disease

关键词： neuroblastoma abdominal chemotherapy

High resolution HLA-matched unrelated donor hematopoietic stem cell transplantation may be considered a first line therapy for children with severe aplastic anemia

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High resolution HLA-matched unrelated donor hematopoietic st...

中华医学会第十七次全国儿科学术大会

作者： Yasuhiro Okamoto Godfrey Chi-fung CHAN Suradej Hongeng TAN Poh Lin TAN Ah Moy Kleebsabai Sanpakit anselm lee Shanghai Jiao Tong University School of Medicine Shanghai Children Prince of Wales Hospital Hong Kong Kagoshima University Medical and Dental Hospital Japan Queen Mary Hospital the University of Hong Kong Faculty of Medicine Ramathibodi Hospital Mahidol University Thailand National University Health System Singapore KK Women’s and Children’s Hospital Singapore Faculty of Medicine Siriraj hospital Mahidol UniversityThailand Nanfang Hospital Southern Medical University China Mount Elizabeth Hospital Singapore

From August 2000 to November 2011,127 children under the age of 18 years with acquired severe aplastic anemia(SAA) received hematopoietic stem cell transplantation(HSCT) in one of the 10 Asia Pacific institutions of the VABMT Consortium,including 53 with matched sibling donor(MSD) and 74 with alternative donor(AD).Among these 74 AD,22 were matched unrelated donor(MUD), 32 were mismatched unrelated donor(MMUD) and 20 were mismatched related donor(MMRD).

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血管性血友病Ⅲ型误诊为血友病A一例

中华儿科杂志 2007年第12期45卷 950-950页

作者：李志伟李泽荷黄立己香港新界青松观道屯门医院儿童及青少年科香港新界青松观道屯门医院病理部

患儿男，15岁，因右足踝肿痛及背部肌肉疼痛于2006年6月入院。患儿出生后3个月因自发性出血及创伤后流血不止，多次诊断为血友病A（因子Ⅷ水平1％）。患儿反复出现关节腔出血；并在2岁时并发颅内出血，接受因子Ⅷ浓缩制剂替代治疗。13... 详细信息

患儿男，15岁，因右足踝肿痛及背部肌肉疼痛于2006年6月入院。患儿出生后3个月因自发性出血及创伤后流血不止，多次诊断为血友病A（因子Ⅷ水平1％）。患儿反复出现关节腔出血；并在2岁时并发颅内出血，接受因子Ⅷ浓缩制剂替代治疗。13岁时曾因不明严重血便导致急性重度贫血，血红蛋白降至40g／L。患儿胞妹10岁因自发性出血进行实验室检查，活化部分凝血活酶时间（AFTT）为69．3s，

关键词：血友病A 血管性血友病活化部分凝血活酶时间自发性出血误诊 Ⅲ型急性重度贫血实验室检查

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anselm Kiefer

Anselm Kiefer

Orthopedic surgery and its complication in systemic lupus erythematosus

作者： Baque Dominique. (1945-)Kiefer anselm

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World Journal of Orthopedics 2014年第1期5卷 38-44页

作者： anselm Mak Division of Rheumatology Department of Medicine University Medicine Cluster Department of Medicine Yong Loo Lin School of Medicine National University of Singapore

Systemic lupus erythematosus(SLE) is a multi-systemic immune-complex mediated autoimmune condition which chiefly affects women during their prime year. While the management of the condition falls into the specialty of internal medicine, patients with SLE often present with signs and symptoms pertaining to the territory of orthopedic surgery such as tendon rupture, carpal tunnel syndrome, osteonecrosis, osteoporotic fracture and infection including septic arthritis, osteomyelitis and spondylodiscitis. While these orthopedicrelated conditions are often debilitating in patients with SLE which necessitate management by orthopedic specialists, a high index of suspicion is necessary in diagnosing these conditions early because lupus patients with potentially severe orthopedic conditions such as osteomyelitis frequently present with mild symptoms and subtle signs such as low grade fever, mild hip pain and back tenderness. Additionally, even if these orthopedic conditions can be recognized, complications as a result of surgical procedures are indeed not uncommon. SLE per se and its various associated pharmacological treatments may pose lupus patients to certain surgical risks if they are not properly attended to andmanaged prior to, during and after surgery. Concerted effort of management and effective communication among orthopedic specialists and rheumatologists play an integral part in enhancing favorable outcome and reduction in postoperative complications for patients with SLE through thorough pre-operative evaluation, careful peri-operative monitoring and treatment, as well as judicious postoperative care.

关键词： orthopedics Complications Surgery Systemic lupus erythematosus Operation

CD137L,a driver of harmful inflammation in the nervous system

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Neural Regeneration Research 2023年第11期18卷 2387-2388页

作者： Hiu Yi Wong Amanda C.Y.Chan anselm Mak Herbert Schwarz Division of Life Science State Key Laboratory of Molecular NeuroscienceMolecular Neuroscience CenterThe Hong Kong University of Science and TechnologyHong Kong Special Administrative RegionChina Hong Kong Center for Neurodegenerative Diseases Hong Kong Special Administrative RegionChina Division of Neurology University Medicine ClusterNational University HospitalSingapore Department of Medicine Yong Loo Lin School of MedicineNational University of SingaporeSingapore Division of Rheumatology University Medicine ClusterNational University Health SystemDepartment of MedicineYong Loo Lin School of MedicineNational Univesity of SingaporeSingapore Department of Physiology and Immunology ProgrammeYong Loo Lin School of MedicineNational University of SingaporeSingapore Integrative Sciences and Engineering Programme NUS Graduate SchoolNational University of SingaporeSingapore

CD137 (TNFRSF9,4-1BB) is a member of the tumor necrosis factor (TNF) receptor family and a potent costimulatory *** levels of CD137 are expressed on T cells upon ***137 signaling in T cells,either by cognate interaction with antigen-presenting cells (APC)or by agonistic anti-CD137 antibodies,strongly enhances proliferation,interferon-y secretion,and cytolytic activity of T ***,CD137 signaling is a main driver of cellular,type 1 helper T cells (Th1)and type 1 cytolytic T cells (Tc1) polarised immune responses.

关键词： CD137 CD137L inflammation

Green-light fundus autofluorescence in diabetic macular edema

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International Journal of Ophthalmology(English edition) 2013年第1期6卷 75-80页

作者： Lukas Reznicek Simeon Dabov Christos Haritoglou anselm Kampik Marcus Kernt Aljoscha S Neubauer Department of Ophthalmology Ludwig Maximilians University

AIM:To evaluate the role of central green-light fundus autofluorescence (FAF) in diabetic macular edema(DME). METHODS:A consecutive series of 92 study eyes with diabetic retinopathy were included. Out of those, 51 diabetic eyes had DME and were compared to 41 diabetic eyes without DME. In all subjects, green-light FAF images were obtained, quantified and classified into various FAF patterns. Cross-sectional optical coherence tomography (OCT) scans were obtained for evaluation of Inner/Outer segment (IS/OS) layer integrity, measurements of central RPE-IS/OS layer thickness as well as classification of DME into various subtypes. ·RESULTS:Meancentralgreen-lightFAFintensityofeyes with DME (1.289 ±0.140)log did not significantly differ from diabetic patients without DME (1.317 ±0.137)log. Most classifiable FAF patterns were seen in patients with cystoid DME. Mean central retinal thickness (CRT) of all study eyes with DME was (501.9±112.4)μm compared to (328.2±27.0)μm in diabetic patients without DME. Patients with DME had significantly more disrupted photoreceptor IS/OS layers than diabetic patients without DME (28/51 vs 5/41, P <0.001). Mean RPE-IS/OS thickness of patients with DME (60.7±14.1)μm was significantly (P<0.001) lower than in diabetic eyes without DME (73.5 ±9.4)μm. Correlation analysis revealed non-significant correlations of green-light FAF intensity and OCT parameters in all subtypes of DME. ·CONCLUSION:Our results indicate a poor correlation of central green-light FAF intensity with CRT, IS/OS layer integrity or RPE-IS/OS layer thickness in diabetic patients with or without DME and its various subtypes. Thus, central green-light FAF is not suitable for detection of retinal thickening in DME.

关键词： diabetic macular edema fundus autofluorescence optical coherence tomography

My mother is looking blue

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World Journal of Emergency Medicine 2019年第4期10卷 251-252页

作者： Howard Tat Chun Chan anselm Wang Hei Hui Colin Graham Joseph Walline Department of Accident and Emergency Medicine Hong Kong Hospital AuthorityPrince of Wales HospitalShatinHong KongChina Accident and Emergency Medicine Academic Unit The Chinese University of Hong KongPrince of Wales HospitalShatinHong KongChina

Dear editor,A 76-year-old Chinese female presented by ambulance to the Emergency Department complaining of dizziness,headache and *** son claimed that the patient“turned blue”three hours prior to onset of the patient’s *** noted the patient’s SpO2 was 83%on room air with no improvement with a nonrebreather *** medical history was signifi cant for diabetes and ***,social and medication history were non-contributory.

关键词： mother looking blue Emergency Department

Correlation of leaking microaneurysms with retinal thickening in diabetic retinopathy

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International Journal of Ophthalmology(English edition) 2011年第3期4卷 269-271页

作者： Lukas Reznicek Marcus Kernt Christos Haritoglou Michael Ulbig anselm Kampik Aljoscha S Neubauer Department of Ophthalmology Ludwig-Maximilians University

AIM: To investigate the contribution of fluorescein angiographic leaking microaneurysms (leak-MA) versus non-leaking microaneurysms (non-leak-MA) to retinal thickening in diabetic ***: A consecutive series of 38 eyes from 24 patients with diabetic retinopathy was ***-MA and non-leak-MA in each eye were selected in pairs at corresponding topographic *** was defined by late phase fluorescein angiograms compared to early *** thickness was measured with Heidelberg Spectralis OCT topographically aligned on early phase angiograms at the MA site and within a 1 mm ***: In all eyes,significant retinal thickening at the site of leaking compared to non-leaking microaneurysms was observed (356±69μm vs 318±56μm,P <0.001),showing a mean increase in thickness in the areas of leak-MA vs non-leak-MA of 38±39μm (95% confidence interval 2551μm,P<0.001).All 1mm area measurements also showed significant (P<0.001) thickening of the leak-MA with average thickness of leak-MA vs non-leak-MA as 351±67μmvs 319±59μm;minimum thickness 311±62μm vs 284±60μm;maximum thickness 389±78μm vs 352±66μm;and retina volume 26.4±6.0mmvs 23.6±3.7mm3,***: Leaking of microaneurysms on fluorescein angiography appears to cause focal thickening of retina,which can be measured with high-resolution ***,targeting leaking microaneursyms in diabetic retinopathy has the potential to reduce retinal thickening.

关键词： diabetic retinopathy microaneurysm retinal thickness OCT