36 results on '"Elfeky R"'
Search Results
2. Author Correction: Long-term safety and efficacy of lentiviral hematopoietic stem/progenitor cell gene therapy for Wiskott–Aldrich syndrome
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Magnani, A., Semeraro, M., Adam, F., Booth, C., Dupré, L., Morris, E. C., Gabrion, A., Roudaut, C., Borgel, D., Toubert, A., Clave, E., Abdo, C., Gorochov, G., Petermann, R., Guiot, M., Miyara, M., Moshous, D., Magrin, E., Denis, A., Suarez, F., Lagresle, C., Roche, A. M., Everett, J., Trinquand, A., Guisset, M., Bayford, J. Xu, Hacein-Bey-Abina, S., Kauskot, A., Elfeky, R., Rivat, C., Abbas, S., Gaspar, H. B., Macintyre, E., Picard, C., Bushman, F. D., Galy, A., Fischer, A., Six, E., Thrasher, A. J., and Cavazzana, M.
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- 2022
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3. NON-PTLD MALIGNANCY POST-HSCT FOR PRIMARY IMMUNODEFICIENCY-4 CASES: PH-AB122
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Elfeky, R., Grainger, J., Loughran, S., Hobin, D., Watt, D., Abinun, M., Slatter, M., and Gennery, A.
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- 2014
4. HAEMATOPOIETIC STEM CELL TRANSPLANTATION FOR RAG1/2 SEVERE COMBINED IMMUNODEFICIENCY OR OMENN SYNDROME: A SINGLE CENTRE EXPERIENCE: PH-P559
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Elfeky, R., i, Nadem Z., Brigham, K., Barge, D., Hambleton, S., Abinun, M., Flood, T., Cant, A., Slatter, M., and Gennery, A.
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- 2014
5. Non-alcoholic Fatty Liver Disease and ECG changes in Egyptian Patients
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Alsawaby, A S, primary, Elfeky, R A, primary, Mohamed, A E, primary, Abdelaziz, H, primary, Saleh, S A, primary, Mohammed, H G, primary, Montasser, I F, primary, and Abdelbary, M H, primary
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- 2020
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6. Assessment of Toll Like receptor 2 Gene Polymorphism in Children with B Cell Defects and Respiratory Tract Infections
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Mahana, N A A, primary, Reda, S M, primary, Kamel, T B, primary, Elfeky, R A, primary, Erfan, D M, primary, and Kamal, T M, primary
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- 2020
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7. 132 Improved survival and outcome of HLA-mismatched donor hematopoietic stem cell transplantation in children with primary immunodeficiencies using new graft manipulation strategies in the UK
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Elfeky, R, primary, Shah, RM, additional, Unni, MNM, additional, Rao, K, additional, Chiesa, R, additional, Amrolia, P, additional, Worth, A, additional, Flood, T, additional, Abinun, M, additional, Nademi, Z, additional, Hambleton, S, additional, Cant, AJ, additional, Gilmour, K, additional, Adams, S, additional, Ahsan, G, additional, Barge, D, additional, Gennery, AR, additional, Qasim, W, additional, Slatter, M, additional, and Veys, P, additional
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- 2018
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8. PATIENT-CENTRED SCREENING FOR PRIMARY IMMUNODEFICIENCY, A MULTI-STAGE DIAGNOSTIC PROTOCOL DESIGNED FOR NONIMMUNOLOGISTS: 2011 UPDATE
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De Vries, E., primary, Cardona, A. A., additional, Abdul Latiff, A. H., additional, Badolato, R., additional, Brodszki, N., additional, Cant, A. J., additional, Carbone, J., additional, Casper, J. T., additional, Ciznar, A., additional, Cochino, A. V., additional, Derfalvi, B., additional, Driessen, G. J., additional, Elfeky, R., additional, El-Ghoneimy, D., additional, Espanol, T., additional, Etzioni, A., additional, Gambineri, E., additional, Gilmour, K., additional, Gonzalez-Granado, L. I., additional, Guseva, M. N., additional, Haverkamp, M. H., additional, Helminen, M., additional, Honig, M., additional, Kanariou, M. G., additional, Kirschfink, M., additional, Klein, C., additional, Kuijpers, T. W., additional, Kutukculer, N., additional, Martire, B., additional, Meyts, I., additional, Niehues, T., additional, Pignata, C., additional, Reda, S. M., additional, Renner, E. D., additional, Rezaei, N., additional, Rizzi, M., additional, Sampalo Lainz, M. A., additional, Sargur, R. B., additional, Sediva, A., additional, Seidel, M. G., additional, Seneviratne, S. L., additional, Soler-Palacin, P., additional, Tommasini, A., additional, and Warnatz, K., additional
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- 2014
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9. Patient-centred screening for primary immunodeficiency, a multi-stage diagnostic protocol designed for non-immunologists: 2011 update
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Array Эльфеки, Array Гилмор, Array Хельминен, Array В. Кочино, Array Чижняр, Array Эспаньол, Array Х. Абдул-Латиф, Array Седива, Array Б. Саргур, Array Нихюэс, Array Пиньята, Array Варнатц, Array Рицци, Array Гамбинери, Array Альварес Кардона, Array Н. Гусева, Array Киршфинк, Array Д. Реннер, Array де Вриз, М Х Array, Array B. Кёйперс, Array И. Гонсалес-Гранадо, Array Г. Канариу, Array Хёниг, Array Карбоне, Array Й. Дриссен, Array Резаеи, Array Г. Зайдель, Array Мейтс, Array Томмазини, Array Ациони, Array Т. Каспер, Array Кляйн, Array Дерфальви, Array Дж. Кант, Array Солер-Паласин, Array Мартире, Array А. Сампало Лайнс, Array М. Рида, Array Бадолато, Array Кутукджюлер, Array Л. Сеневиратне, Array Бродшки, Array Эль-Гонейми, AII - Amsterdam institute for Infection and Immunity, Paediatric Infectious Diseases / Rheumatology / Immunology, de Vries, E., Alvarez Cardona, A., Abdul Latiff, A. H., Badolato, R., Brodszki, N., Cant, A. J., Carbone, J., Casper, J. T., Čižnár, P., Cochino, A. V., Derfalvi, B., Driessen, G. J., Elfeky, R., El Ghoneimy, D., Espanol, T, Etzioni, A, Gambineri, E, Gilmour, K, Gonzalez Granado, Li, Haverkamp, Mh, Helminen, M, Hönig, H, Kanariou, Mg, Kirschfink, M, Klein, C., Kuijpers, T. W., Kutukculer, N., Martire, B., Meyts, I., Niehues, T., Pignata, Claudio, Reda, S. M., Renner, E. D., Rezaei, N., Rizzi, M., Sampalo Lainz, M. A., Sargur, R. B., Sediva, A., Seidel, M. G., Seneviratne, S. L., Soler Palacín, P., Tommasini, A., Warnatz, K., de Vries, E, and Tommasini, A
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Adult ,immunological evaluation ,medicine.medical_specialty ,Translational Studies ,update ,General Practice ,Immunology ,Infections ,primary immunodeficiency ,Pediatrics ,Diagnostic protocol ,Immunological evaluation ,Primary immunodeficiency ,Update ,Immunophenotyping ,Clinical Protocols ,Recurrence ,Patient-Centered Care ,medicine ,Humans ,Mass Screening ,Immunology and Allergy ,Medical physics ,Age of Onset ,Child ,Medical History Taking ,Physical Examination ,Immunodeficiency ,Protocol (science) ,diagnostic protocol ,business.industry ,Incidence ,Immunologic Deficiency Syndromes ,RC581-607 ,Cytotoxicity Tests, Immunologic ,medicine.disease ,Lymphocyte Subsets ,Multi stage ,Early Diagnosis ,Phenotype ,PID, diagnostic protocols ,Medical emergency ,Immunologic diseases. Allergy ,business ,Patient centred ,Granulocytes - Abstract
Summary Members of the European Society for Immunodeficiencies (ESID) and other colleagues have updated the multi-stage expert-opinion-based diagnostic protocol for non-immunologists incorporating newly defined primary immunodeficiency diseases (PIDs). The protocol presented here aims to increase the awareness of PIDs among doctors working in different fields. Prompt identification of PID is important for prognosis, but this may not be an easy task. The protocol therefore starts from the clinical presentation of the patient. Because PIDs may present at all ages, this protocol is aimed at both adult and paediatric physicians. The multi-stage design allows cost-effective screening for PID of the large number of potential cases in the early phases, with more expensive tests reserved for definitive classification in collaboration with a specialist in the field of immunodeficiency at a later stage.
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- 2012
10. Allogeneic Hematopoietic Stem Cell Transplantation in Immunodeficiency-Centromeric Instability-Facial Dysmorphism (ICF) Syndrome: an EBMT/ESID Inborn Errors Working Party Study.
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Berghuis D, Mehyar LS, Abu-Arja R, Albert MH, Barnum JL, von Bernuth H, Elfeky R, Lewalle P, Laberko A, Ghosh S, Slatter MA, Weemaes CMR, Yesilipek A, Sirait T, Neven B, Gennery AR, and Lankester AC
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- Humans, Child, Preschool, Child, Male, Female, Infant, Adolescent, Young Adult, Immunologic Deficiency Syndromes therapy, Immunologic Deficiency Syndromes diagnosis, Transplantation Conditioning methods, Treatment Outcome, Primary Immunodeficiency Diseases therapy, Primary Immunodeficiency Diseases diagnosis, Hematopoietic Stem Cell Transplantation methods, Transplantation, Homologous, Graft vs Host Disease etiology, Graft vs Host Disease diagnosis
- Abstract
Immunodeficiency-Centromeric instability-Facial dysmorphism (ICF) syndrome is an inborn error of immunity characterized by progressive immune dysfunction and multi-organ disease usually treated with antimicrobial prophylaxis and immunoglobulin substitution. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment, but data on outcome are scarce. We provide a detailed description of disease characteristics and HSCT outcome in an international cohort of ICF syndrome patients. Eighteen patients (including all four genotypes) were enrolled. Main HSCT indications were infections (83%), enteropathy/failure to thrive (56%), immune dysregulation (22%) and myelodysplasia/haematological malignancy (17%). Two patients underwent pre-emptive HSCT after early diagnosis. Patients were transplanted between 2003-2021, at median age 4.3 years (range 0.5-19), after myeloablative or reduced-intensity conditioning, from matched sibling or matched family donors, matched unrelated or mismatched donors in 39%, 50% and 12% of cases respectively. Overall survival was 83% (all deaths occurred within the first 5 months post-HSCT; mean follow-up 54 months (range 1-185)). Acute GvHD occurred in 35% of patients, severe (grade III) in two (12%), while none developed chronic GvHD. At latest follow-up (median 2.2 years (range 0.1-14)), complete donor chimerism was achieved in 15/17 surviving patients. All survivors demonstrated normalized T and B cell numbers. Immunoglobulin substitution independence was achieved in all but two patients. All survivors recovered from pre-transplant infections, enteropathy/failure to thrive and immune dysregulation. All three patients transplanted at young age (≤ 3 years), after early diagnosis, survived. The favourable clinical and immunological HSCT outcome in this cohort of patients supports the timely use of this curative treatment in ICF syndrome., (© 2024. The Author(s).)
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- 2024
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11. Pediatric Adapted Risk index (PARI) to predict 2-year transplant related mortality post-HSCT in children.
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Elfeky R, Builes N, Pearce RM, Kania SP, Nademi Z, Lucchini G, Chiesa R, Amrolia PJ, Sorror ML, Veys P, and Rao K
- Abstract
Several attempts have been made to optimize pre-transplant risk assessment to improve hematopoietic stem cell transplantation (HSCT) decision-making and to predict outcome post- HSCT. However, its relevance to the pediatric population remains unclear. We report the results of revalidation of the HCT-CI in 874 children who received 944 HSCTs for malignant or non-malignant diseases at a single centre. After finding the HCT-CI invalid in our patient population; we proposed a modified pediatric adapted scoring system that captures risk factors (RF) and comorbidities (CoM) relevant to pediatrics. Each RF/CoM was assigned an integer weight based on its hazard ratio (HR) for TRM; 0 (HR <1.2), 1 (1.2 ≥HR <1.75), 2 (1.75 ≥HR <2.5), 3 (HR ≥2.5) .Using these weights, the pediatric adapted HSCT-RI (PARI) was devised, and patients were divided into 4 risk groups; group 1 without RF/CoM, group 2: scores 1-2, group 3: scores 3-4, group 4: scores ≥5. There was a linear increase in 2-year TRM from group 1 to 4 (TRM= 6.2% in group 1, 50.9% in group 4). PARI was successfully validated on an internal and external cohort of pediatric patients. Comparing models using c-statistics, PARI was found to be a better model than HCT-CI in predicting 2-year TRM in children with Akaike's and Schwarz's Bayesian information criteria (AIC and BIC) of 1069.245 and 1073.269; respectively using PARI vs 1223.158 and 1227.051; respectively using HCT-CI. We believe that PARI will be a valuable tool enabling better counselling and decision making for pediatric HSCT patients., (Copyright © 2024 American Society of Hematology.)
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- 2024
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12. Outcomes of HLA-mismatched HSCT with TCRαβ/CD19 depletion or post-HSCT cyclophosphamide for inborn errors of immunity.
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Lum SH, Albert MH, Gilbert P, Sirait T, Algeri M, Muratori R, Fournier B, Laberko A, Karakukcu M, Unal E, Ayas M, Yadav SP, Fisgin T, Elfeky R, Fernandes J, Faraci M, Cole T, Schulz A, Meisel R, Zecca M, Ifversen M, Biffi A, Diana JS, Vallée T, Giardino S, Ersoy GZ, Moshous D, Gennery AR, Balashov D, Bonfim C, Locatelli F, Lankester A, Neven B, and Slatter M
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- Humans, Child, Child, Preschool, Female, Male, Infant, Adolescent, Retrospective Studies, Young Adult, Lymphocyte Depletion, Transplantation Conditioning methods, HLA Antigens immunology, Adult, Treatment Outcome, Infant, Newborn, Hematopoietic Stem Cell Transplantation adverse effects, Hematopoietic Stem Cell Transplantation methods, Cyclophosphamide therapeutic use, Cyclophosphamide administration & dosage, Receptors, Antigen, T-Cell, alpha-beta, Antigens, CD19, Graft vs Host Disease etiology, Graft vs Host Disease prevention & control
- Abstract
Abstract: HLA-mismatched transplants with either in vitro depletion of CD3+ T-cell receptor (TCR)αβ/CD19 (TCRαβ) cells or in vivo T-cell depletion using posttransplant cyclophosphamide (PTCY) have been increasingly used for patients with inborn errors of immunity (IEIs). We performed a retrospective multicenter study via the EBMT registry on 306 children with IEIs undergoing their first transplant between 2010 and 2019 from an HLA-mismatched donor using TCRαβ (n = 167) or PTCY (n = 139). The median age for hematopoietic stem cell transplantation (HSCT) was 1.2 years (range, 0.03-19.6 years). The 3-year overall survival (OS) was 78% (95% confidence interval (CI), 71-84) after TCRαβ and 66% (57-74) after PTCY (P = .013). Pre-HSCT morbidity score (hazard ratio [HR], 2.27; 1.07-4.80, P = .032) and non-busulfan/treosulfan conditioning (HR, 3.12; 1.98-4.92, P < .001) were the only independent predictors of unfavorable OS. The 3-year event-free survival (EFS) was 58% (50%-66%) after TCRαβ and 57% (48%-66%) after PTCY (P = .804). The cumulative incidence of severe acute graft-versus-host disease (GvHD) was higher after PTCY (15%, 9%-21%) than TCRαβ (6%, 2%-9%, P = .007), with no difference in chronic GvHD (PTCY, 11%, 6%-17%; TCRαβ, 7%, 3%-11%, P = .173). The 3-year GvHD-free EFS was 53% (44%-61%) after TCRαβ and 41% (32%-50%) after PTCY (P = .080). PTCY had significantly higher rates of veno-occlusive disease (14.4% vs TCRαβ 4.9%, P = .009), acute kidney injury (12.7% vs 4.6%, P = .032), and pulmonary complications (38.2% vs 24.1%, P = .017). Adenoviremia (18.3% vs PTCY 8.0%, P = .015), primary graft failure (10% vs 5%, P = .048), and second HSCT (17.4% vs 7.9%, P = .023) were significantly higher in TCRαβ. In conclusion, this study demonstrates that both approaches are suitable options in patients with IEIs, although they are characterized by different advantages and outcomes., (© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.)
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- 2024
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13. A well child with prolonged oral thrush: an unexpected diagnostic journey.
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Gounari E, Elfeky R, Ghataore L, Muhi-Iddin N, Buchanan CR, and Arya VB
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- Humans, Male, Child, Preschool, Candidiasis, Oral diagnosis, Candidiasis, Oral therapy
- Abstract
Oral thrush is a familiar presentation in both general practice and paediatrics, and is usually responsive to treatment in the community. Here, we present the diagnostic journey of a previously well boy aged 3 years who presented with treatment-resistant thrush and describe how 'unexpected' results led to eventual diagnosis and management. This intriguing case was managed jointly by district hospital general paediatric team and tertiary hospital specialist teams., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2024
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14. The Human Phenotype Ontology in 2024: phenotypes around the world.
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Gargano MA, Matentzoglu N, Coleman B, Addo-Lartey EB, Anagnostopoulos AV, Anderton J, Avillach P, Bagley AM, Bakštein E, Balhoff JP, Baynam G, Bello SM, Berk M, Bertram H, Bishop S, Blau H, Bodenstein DF, Botas P, Boztug K, Čady J, Callahan TJ, Cameron R, Carbon SJ, Castellanos F, Caufield JH, Chan LE, Chute CG, Cruz-Rojo J, Dahan-Oliel N, Davids JR, de Dieuleveult M, de Souza V, de Vries BBA, de Vries E, DePaulo JR, Derfalvi B, Dhombres F, Diaz-Byrd C, Dingemans AJM, Donadille B, Duyzend M, Elfeky R, Essaid S, Fabrizzi C, Fico G, Firth HV, Freudenberg-Hua Y, Fullerton JM, Gabriel DL, Gilmour K, Giordano J, Goes FS, Moses RG, Green I, Griese M, Groza T, Gu W, Guthrie J, Gyori B, Hamosh A, Hanauer M, Hanušová K, He YO, Hegde H, Helbig I, Holasová K, Hoyt CT, Huang S, Hurwitz E, Jacobsen JOB, Jiang X, Joseph L, Keramatian K, King B, Knoflach K, Koolen DA, Kraus ML, Kroll C, Kusters M, Ladewig MS, Lagorce D, Lai MC, Lapunzina P, Laraway B, Lewis-Smith D, Li X, Lucano C, Majd M, Marazita ML, Martinez-Glez V, McHenry TH, McInnis MG, McMurry JA, Mihulová M, Millett CE, Mitchell PB, Moslerová V, Narutomi K, Nematollahi S, Nevado J, Nierenberg AA, Čajbiková NN, Nurnberger JI Jr, Ogishima S, Olson D, Ortiz A, Pachajoa H, Perez de Nanclares G, Peters A, Putman T, Rapp CK, Rath A, Reese J, Rekerle L, Roberts AM, Roy S, Sanders SJ, Schuetz C, Schulte EC, Schulze TG, Schwarz M, Scott K, Seelow D, Seitz B, Shen Y, Similuk MN, Simon ES, Singh B, Smedley D, Smith CL, Smolinsky JT, Sperry S, Stafford E, Stefancsik R, Steinhaus R, Strawbridge R, Sundaramurthi JC, Talapova P, Tenorio Castano JA, Tesner P, Thomas RH, Thurm A, Turnovec M, van Gijn ME, Vasilevsky NA, Vlčková M, Walden A, Wang K, Wapner R, Ware JS, Wiafe AA, Wiafe SA, Wiggins LD, Williams AE, Wu C, Wyrwoll MJ, Xiong H, Yalin N, Yamamoto Y, Yatham LN, Yocum AK, Young AH, Yüksel Z, Zandi PP, Zankl A, Zarante I, Zvolský M, Toro S, Carmody LC, Harris NL, Munoz-Torres MC, Danis D, Mungall CJ, Köhler S, Haendel MA, and Robinson PN
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- Humans, Phenotype, Genomics, Algorithms, Rare Diseases, Biological Ontologies
- Abstract
The Human Phenotype Ontology (HPO) is a widely used resource that comprehensively organizes and defines the phenotypic features of human disease, enabling computational inference and supporting genomic and phenotypic analyses through semantic similarity and machine learning algorithms. The HPO has widespread applications in clinical diagnostics and translational research, including genomic diagnostics, gene-disease discovery, and cohort analytics. In recent years, groups around the world have developed translations of the HPO from English to other languages, and the HPO browser has been internationalized, allowing users to view HPO term labels and in many cases synonyms and definitions in ten languages in addition to English. Since our last report, a total of 2239 new HPO terms and 49235 new HPO annotations were developed, many in collaboration with external groups in the fields of psychiatry, arthrogryposis, immunology and cardiology. The Medical Action Ontology (MAxO) is a new effort to model treatments and other measures taken for clinical management. Finally, the HPO consortium is contributing to efforts to integrate the HPO and the GA4GH Phenopacket Schema into electronic health records (EHRs) with the goal of more standardized and computable integration of rare disease data in EHRs., (© The Author(s) 2023. Published by Oxford University Press on behalf of Nucleic Acids Research.)
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- 2024
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15. Prevalence and Characteristics of Non-tuberculous Mycobacteria (NTM) Infection in Recipients of Allogeneic Hematopoietic Stem Cell Transplantation: a Systematic Review and Meta-analysis.
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Cinicola BL, Ottaviano G, Hashim IF, Zainudeen ZT, Hamid IJA, and Elfeky R
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- Adult, Child, Humans, Prevalence, Risk Factors, Transplant Recipients, Retrospective Studies, Nontuberculous Mycobacteria, Hematopoietic Stem Cell Transplantation adverse effects
- Abstract
Purpose: Non-tuberculous mycobacteria (NTM) infections in hematopoietic stem cell transplantation (HSCT) recipients represent a diagnostic and therapeutic challenge. Here, we aimed to review and analyze current literature on incidence, clinical presentation, and outcome of NTM infection after allogeneic HSCT., Methods: We performed a systematic review and meta-analysis of available literature regarding NTM infection in children and adults receiving allogeneic HSCT., Results: We identified 56 articles eligible for the analysis. Among 15 studies, describing 15,798 allogeneic HSCT, we estimated a prevalence of 1.26% (95% CI 0.72, 1.93) of NTM after transplant. Analysis of 175 patients with NTM infection showed a median time of diagnosis of 318 days after HSCT, an increased prevalence in adults (82.9%), and a most frequent pulmonary involvement (44%). Comparison between children and adults revealed an earlier post-transplant disease onset (median 130 days vs 287 days) and most frequent non-pulmonary presentation in children. A vast heterogeneity of therapeutic approach reflected the lack of universal recommendations regarding drug combination and duration of therapy. Overall, NTM-related mortality accounted for 33% in this systematic review., Conclusion: Although rare, NTM infections can complicate post-transplant course with a high mortality rate in children and adults. The lack of prospective studies and guidelines prevents identification of risk factors and therapeutic recommendations., (© 2023. Crown.)
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- 2023
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16. Immunoglobulin Replacement Therapy During COVID-19 Pandemic: Practical and Psychological Impact in Patients with Antibody Deficiency.
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Maimaris J, O'Sullivan A, Underhill I, Green G, Symes A, Lowe D, Burns S, Campbell M, and Elfeky R
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- Humans, Pandemics, COVID-19 Vaccines, Quality of Life, Immunoglobulins therapeutic use, Fatigue, COVID-19 epidemiology, Primary Immunodeficiency Diseases
- Abstract
Purpose: The COVID-19 pandemic has impacted on how health services deliver care and the mental health of the population. Due to their clinical vulnerability, to reduce in-hospital attendances during the COVID-19 pandemic, modifications in immunoglobulin treatment regimens were made for patients with antibody deficiency. These patients were also likely to experience social isolation due to shielding measure that were advised. We aimed to investigate the impact of modifying immunoglobulin treatment regimen on infection and mental health burden during shielding restrictions., Method: Patients on immunoglobulin replacement therapy (IGRT) responded to a standardised questionnaire examining self-reported infection frequency, anxiety (GAD-7), depression (PHQ-8), fatigue (FACIT), and quality of life during the pandemic. Infection frequency and immunoglobulin trough levels were compared to pre-pandemic levels., Results: Patients who did not change treatment modality or those who received immunoglobulin replacement at home during the pandemic reported fewer infections. In patients who received less frequent hospital infusions, there was no significant increase in infections whilst immunoglobulin trough levels remained stable. There was no significant difference in anxiety, or depression scores between the treatment modality groups. Patients reported higher fatigue scores compared to the pre-COVID general population and in those discharged following hospitalisation for COVID., Conclusion: Changing immunoglobulin treatment regimen did not negatively impact infection rates or psychological wellbeing. However, psychological welfare should be prioritised for this group particularly given uncertainties around COVID-19 vaccination responsiveness and continued social isolation for many., (© 2023. The Author(s).)
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- 2023
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17. Non-osteopenic Bone Pathology After Allo-hematopoietic Stem Cell Transplantation in Patients with Inborn Errors of Immunity.
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Golwala ZM, Bhat NG, Xu-Bayford J, Stankova T, Adams S, Morris EC, Qasim W, Booth C, Worth A, Kusters MA, and Elfeky R
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- Child, Humans, Risk Factors, Incidence, Retrospective Studies, Transplantation Conditioning, Wiskott-Aldrich Syndrome, Hematopoietic Stem Cell Transplantation adverse effects, Lymphohistiocytosis, Hemophagocytic etiology
- Abstract
Purpose: There is a lack of data on post-HSCT non-osteopenic bone pathology specifically for children with inborn errors of immunity (IEI). We collected data on non-osteopenic bone pathology in children with IEI post-HSCT over two decades in a large tertiary pediatric immunology center., Methods: Descriptive study with data analysis of bone pathology in allo-HSCT for IEI was performed between 1/1/2000 to 31/12/2018 including patients alive at follow-up to July 2022. Records were analyzed for bone pathology and risk factors. Exclusion criteria included isolated reduced bone density, fractures, and skeletal anomalies due to underlying IEI and short stature without other bone pathology. Bone pathologies were divided into 5 categories: bone tumors; skeletal dysplasia; avascular necrosis; evolving bone deformities; slipped upper femoral epiphysis., Results: A total of 429 children received HSCT between 2000 and 2018; 340 are alive at last assessment. Non-osteopenic bone pathology was observed post-HSCT in 9.4% of patients (32/340, mean 7.8 years post-HSCT). Eleven patients (34%) had > 1 category of bone pathology. Seventeen patients (17/32; 53%) presented with bilateral bone pathology. The majority of patients received treosulfan-based conditioning (26/32; 81.2%). Totally, 65.6% (21/32) of patients had a history of prolonged steroid use (> 6 months). Pain was the presenting symptom in 66% of patients, and surgical intervention was required in 43.7%. The highest incidence of bone pathologies was seen in Wiskott-Aldrich syndrome (WAS) (n = 8/34; 23.5%) followed by hemophagocytic lymphohistiocytosis patients (n = 3/16; 18.8%)., Conclusion: Non-osteopenic bone pathology in long-term survivors of allo-HSCT for IEI is not rare. Most patients did not present with complaints until at least 5 years post-HSCT highlighting the need for ongoing bone health assessment for patients with IEI. Children presenting with stunted growth and bone pathology post-HSCT should undergo skeletal survey to rule out development of post-HSCT skeletal dysplasia. Increased rates and complexity of bone pathology were seen amongst patients with Wiskott-Aldrich syndrome., (© 2023. Crown.)
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- 2023
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18. Combined novel homozygous variants in both SGPL1 and STAT 1 presenting with severe combined immune deficiency: case report and literature review.
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Roa-Bautista A, Sohail M, Wakeling E, Gilmour KC, Davis M, Gait A, Lucchini G, Cox D, Elfeky R, and Kusters M
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- Humans, Aldehyde-Lyases genetics, Aldehyde-Lyases metabolism, Janus Kinases metabolism, Signal Transduction, STAT Transcription Factors metabolism, STAT1 Transcription Factor genetics, STAT1 Transcription Factor metabolism, Infant, Newborn, Immunologic Deficiency Syndromes, Nephrotic Syndrome genetics, Severe Combined Immunodeficiency
- Abstract
Background: Sphingosine phosphate lyase insufficiency syndrome (SPLIS) is associated with biallelic variants in SGPL1 , comprising a multisystemic disease characterized by steroid resistant nephrotic syndrome, primary adrenal insufficiency, neurological problems, skin abnormalities and immunodeficiency in described cases. Signal transducer and activator of transcription 1 (STAT1) plays an important role in orchestrating an appropriate immune response through JAK-STAT pathway. Biallelic STAT1 loss of function (LOF) variants lead to STAT1 deficiency with a severe phenotype of immunodeficiency with increased frequency of infections and poor outcome if untreated., Case Presentation: We report novel homozygous SGPL 1 and STAT1 variants in a newborn of Gambian ethnicity with clinical features of SPLIS and severe combined immunodeficiency. The patient presented early in life with nephrotic syndrome, severe respiratory infection requiring ventilation, ichthyosis, and hearing loss, with T-cell lymphopenia. The combination of these two conditions led to severe combined immunodeficiency with inability to clear respiratory tract infections of viral, fungal, and bacterial nature, as well as severe nephrotic syndrome. The child sadly died at 6 weeks of age despite targeted treatments., Conclusion: We report the finding of two novel, homozygous variants in SGPL1 and STAT1 in a patient with a severe clinical phenotype and fatal outcome early in life. This case highlights the importance of completing the primary immunodeficiency genetic panel in full to avoid missing a second diagnosis in other patients presenting with similar severe clinical phenotype early in life. For SPLIS no curative treatment is available and more research is needed to investigate different treatment modalities. Hematopoietic stem cell transplantation (HSCT) shows promising results in patients with autosomal recessive STAT1 deficiency. For this patient's family, identification of the dual diagnosis has important implications for future family planning. In addition, future siblings with the familial STAT1 variant can be offered curative treatment with HSCT., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Roa-Bautista, Sohail, Wakeling, Gilmour, Davis, Gait, Lucchini, Cox, Elfeky and Kusters.)
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- 2023
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19. Use of eculizumab in children with allogeneic haematopoietic stem cell transplantation associated thrombotic microangiopathy - a multicentre retrospective PDWP and IEWP EBMT study.
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Svec P, Elfeky R, Galimard JE, Higham CS, Dalissier A, Quigg TC, Bueno Sanchez D, Han Lum S, Faraci M, Cole T, Pichler H, Benítez-Carabante MI, Horakova J, Gonzalez-Vicent M, Yanir A, Fagioli F, Wölfl M, von der Weid N, Protheroe R, Krivan G, Speckmann C, James B, Avcin SL, Bertrand Y, Verna M, Riha P, Patrick K, Cesaro S, Kalwak K, Bierings M, Büchner J, Mellgren K, Prohászka Z, Neven B, Lankester A, and Corbacioglu S
- Subjects
- Child, Humans, Retrospective Studies, Antibodies, Monoclonal, Humanized, Thrombotic Microangiopathies drug therapy, Thrombotic Microangiopathies etiology, Thrombotic Microangiopathies diagnosis, Hematopoietic Stem Cell Transplantation adverse effects
- Abstract
Terminal complement blockade by humanised monoclonal antibody eculizumab has been used to treat transplantation-associated thrombotic microangiopathy (TA-TMA) in recent years. This retrospective international study conducted by the Paediatric Diseases (PDWP) and Inborn Error Working Party (IEWP) of the European Society for Blood and Marrow Transplantation (EBMT) describes outcome and response of 82 paediatric patients from 29 centres who developed TA-TMA and were treated with eculizumab between January 2014 and May 2019. The median time from hematopoietic stem cell transplantation (HSCT) to TA-TMA manifestation was 92 days (range: 7-606) and from TA-TMA diagnosis to the start of eculizumab treatment 6 days (range: 0-135). Most patients received eculizumab weekly (72%, n = 55) with a standard weight (kg)-based dose (78%, n = 64). Six months from beginning of eculizumab therapy, the cumulative incidence of TA-TMA resolution was 36.6% (95% CI: 26.2-47) and the overall survival (OS) was 47.1% (95% CI: 35.9-57.5). All 43 patients with unresolved TA-TMA died. The cause of death was HSCT-related in 41 patients. This study also documents poor outcome of patients without aGvHD and their frequent concomitant viral infections. Considering recent publications, intensified eculizumab dosing and complement monitoring could potentially improve upon outcomes observed in this study., (© 2022. The Author(s), under exclusive licence to Springer Nature Limited.)
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- 2023
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20. Disease Progression of WHIM Syndrome in an International Cohort of 66 Pediatric and Adult Patients.
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Geier CB, Ellison M, Cruz R, Pawar S, Leiss-Piller A, Zmajkovicova K, McNulty SM, Yilmaz M, Evans MO 2nd, Gordon S, Ujhazi B, Wiest I, Abolhassani H, Aghamohammadi A, Barmettler S, Bhar S, Bondarenko A, Bolyard AA, Buchbinder D, Cada M, Cavieres M, Connelly JA, Dale DC, Deordieva E, Dorsey MJ, Drysdale SB, Ehl S, Elfeky R, Fioredda F, Firkin F, Förster-Waldl E, Geng B, Goda V, Gonzalez-Granado L, Grunebaum E, Grzesk E, Henrickson SE, Hilfanova A, Hiwatari M, Imai C, Ip W, Jyonouchi S, Kanegane H, Kawahara Y, Khojah AM, Kim VH, Kojić M, Kołtan S, Krivan G, Langguth D, Lau YL, Leung D, Miano M, Mersyanova I, Mousallem T, Muskat M, Naoum FA, Noronha SA, Ouederni M, Ozono S, Richmond GW, Sakovich I, Salzer U, Schuetz C, Seeborg FO, Sharapova SO, Sockel K, Volokha A, von Bonin M, Warnatz K, Wegehaupt O, Weinberg GA, Wong KJ, Worth A, Yu H, Zharankova Y, Zhao X, Devlin L, Badarau A, Csomos K, Keszei M, Pereira J, Taveras AG, Beaussant-Cohen SL, Ong MS, Shcherbina A, and Walter JE
- Subjects
- Humans, Receptors, CXCR4 genetics, Disease Progression, Immunologic Deficiency Syndromes diagnosis, Immunologic Deficiency Syndromes epidemiology, Immunologic Deficiency Syndromes genetics, Warts diagnosis, Warts epidemiology, Warts genetics, Agammaglobulinemia genetics, Neutropenia genetics, Lymphopenia complications
- Abstract
Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome (WS) is a combined immunodeficiency caused by gain-of-function mutations in the C-X-C chemokine receptor type 4 (CXCR4) gene. We characterize a unique international cohort of 66 patients, including 57 (86%) cases previously unreported, with variable clinical phenotypes. Of 17 distinct CXCR4 genetic variants within our cohort, 11 were novel pathogenic variants affecting 15 individuals (23%). All variants affect the same CXCR4 region and impair CXCR4 internalization resulting in hyperactive signaling. The median age of diagnosis in our cohort (5.5 years) indicates WHIM syndrome can commonly present in childhood, although some patients are not diagnosed until adulthood. The prevalence and mean age of recognition and/or onset of clinical manifestations within our cohort were infections 88%/1.6 years, neutropenia 98%/3.8 years, lymphopenia 88%/5.0 years, and warts 40%/12.1 years. However, we report greater prevalence and variety of autoimmune complications of WHIM syndrome (21.2%) than reported previously. Patients with versus without family history of WHIM syndrome were diagnosed earlier (22%, average age 1.3 years versus 78%, average age 5 years, respectively). Patients with a family history of WHIM syndrome also received earlier treatment, experienced less hospitalization, and had less end-organ damage. This observation reinforces previous reports that early treatment for WHIM syndrome improves outcomes. Only one patient died; death was attributed to complications of hematopoietic stem cell transplantation. The variable expressivity of WHIM syndrome in pediatric patients delays their diagnosis and therapy. Early-onset bacterial infections with severe neutropenia and/or lymphopenia should prompt genetic testing for WHIM syndrome, even in the absence of warts., (© 2022. The Author(s).)
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- 2022
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21. Retrospective, Landmark Analysis of Long-term Adult Morbidity Following Allogeneic HSCT for Inborn Errors of Immunity in Infancy and Childhood.
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Day JW, Elfeky R, Nicholson B, Goodman R, Pearce R, Fox TA, Worth A, Booth C, Veys P, Carpenter B, Hough R, Gaspar HB, Titman P, Ridout D, Workman S, Hernandes F, Sandford K, Laurence A, Campbell M, Burns SO, and Morris EC
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- Adult, Chimerism, Humans, Morbidity, Retrospective Studies, Transplantation Conditioning, Graft vs Host Disease etiology, Hematopoietic Stem Cell Transplantation adverse effects
- Abstract
Purpose: Allogeneic hematopoietic stem cell transplant (HSCT) remains the treatment of choice for patients with inborn errors of immunity (IEI). There is little published medical outcome data assessing late medical complications following transition to adult care. We sought to document event-free survival (EFS) in transplanted IEI patients reaching adulthood and describe common late-onset medical complications and factors influencing EFS., Methods: In this landmark analysis, 83 adults surviving 5 years or more following prior HSCT in childhood for IEI were recruited. The primary endpoint was event-free survival, defined as time post-first HSCT to graft failure, graft rejection, chronic infection, life-threatening or recurrent infections, malignancy, significant autoimmune disease, moderate to severe GVHD or major organ dysfunction. All events occurring less than 5 years post-HSCT were excluded., Results: EFS was 51% for the whole cohort at a median of 20 years post HSCT. Multivariable analysis identified age at transplant and whole blood chimerism as independent predictors of long-term EFS. Year of HSCT, donor, conditioning intensity and underlying diagnosis had no significant impact on EFS. 59 events occurring beyond 5 years post-HSCT were documented in 37 patients (45% cohort). A total of 25 patients (30% cohort) experienced ongoing significant complications requiring active medical intervention at last follow-up., Conclusion: Although most patients achieved excellent, durable immune reconstitution with infrequent transplant-related complications, very late complications are common and associated with mixed chimerism post-HSCT. Early intervention to correct mixed chimerism may improve long-term outcomes and adult health following HSCT for IEI in childhood., (© 2022. The Author(s).)
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- 2022
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22. Curation and expansion of Human Phenotype Ontology for defined groups of inborn errors of immunity.
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Haimel M, Pazmandi J, Heredia RJ, Dmytrus J, Bal SK, Zoghi S, van Daele P, Briggs TA, Wouters C, Bader-Meunier B, Aeschlimann FA, Caorsi R, Eleftheriou D, Hoppenreijs E, Salzer E, Bakhtiar S, Derfalvi B, Saettini F, Kusters MAA, Elfeky R, Trück J, Rivière JG, van der Burg M, Gattorno M, Seidel MG, Burns S, Warnatz K, Hauck F, Brogan P, Gilmour KC, Schuetz C, Simon A, Bock C, Hambleton S, de Vries E, Robinson PN, van Gijn M, and Boztug K
- Subjects
- Biological Ontologies, Humans, Phenotype, Genetic Diseases, Inborn classification, Immune System Diseases classification, Rare Diseases classification
- Abstract
Background: Accurate, detailed, and standardized phenotypic descriptions are essential to support diagnostic interpretation of genetic variants and to discover new diseases. The Human Phenotype Ontology (HPO), extensively used in rare disease research, provides a rich collection of vocabulary with standardized phenotypic descriptions in a hierarchical structure. However, to date, the use of HPO has not yet been widely implemented in the field of inborn errors of immunity (IEIs), mainly due to a lack of comprehensive IEI-related terms., Objectives: We sought to systematically review available terms in HPO for the depiction of IEIs, to expand HPO, yielding more comprehensive sets of terms, and to reannotate IEIs with HPO terms to provide accurate, standardized phenotypic descriptions., Methods: We initiated a collaboration involving expert clinicians, geneticists, researchers working on IEIs, and bioinformaticians. Multiple branches of the HPO tree were restructured and extended on the basis of expert review. Our ontology-guided machine learning coupled with a 2-tier expert review was applied to reannotate defined subgroups of IEIs., Results: We revised and expanded 4 main branches of the HPO tree. Here, we reannotated 73 diseases from 4 International Union of Immunological Societies-defined IEI disease subgroups with HPO terms. We achieved a 4.7-fold increase in the number of phenotypic terms per disease. Given the new HPO annotations, we demonstrated improved ability to computationally match selected IEI cases to their known diagnosis, and improved phenotype-driven disease classification., Conclusions: Our targeted expansion and reannotation presents enhanced precision of disease annotation, will enable superior HPO-based IEI characterization, and hence benefit both IEI diagnostic and research activities., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)
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- 2022
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23. The Protective Role of Maternal Immunization in Early Life.
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Cinicola B, Conti MG, Terrin G, Sgrulletti M, Elfeky R, Carsetti R, Fernandez Salinas A, Piano Mortari E, Brindisi G, De Curtis M, Zicari AM, Moschese V, and Duse M
- Abstract
With birth, the newborn is transferred from a quasi-sterile environment to the outside world. At this time, the neonatal immune system is inexperienced and continuously subject to a process of development as it encounters different antigenic stimuli after birth. It is initially characterized by a bias toward T helper 2 phenotype, reduced T helper 1, and cytotoxic responses to microbial stimuli, low levels of memory, and effector T and B cells and a high production of suppressive T regulatory cells. The aim of this setting, during fetal life, is to maintain an anti-inflammatory state and immune-tolerance. Maternal antibodies are transferred during pregnancy through the placenta and, in the first weeks of life of the newborn, they represent a powerful tool for protection. Thus, optimization of vaccination in pregnancy represents an important strategy to reduce the burden of neonatal infections and sepsis. Beneficial effects of maternal immunization are universally recognized, although the optimal timing of vaccination in pregnancy remains to be defined. Interestingly, the dynamic exchange that takes place at the fetal-maternal interface allows the transfer not only of antibodies, but also of maternal antigen presenting cells, probably in order to stimulate the developing fetal immune system in a harmless way. There are still controversial effects related to maternal immunization including the so called "immunology blunting," i.e., a dampened antibody production following infant's vaccination in those infants who received placentally transferred maternal immunity. However, clinical relevance of this phenomenon is still not clear. This review will provide an overview of the evolution of the immune system in early life and discuss the benefits of maternal vaccination. Current maternal vaccination policies and their rationale will be summarized on the road to promising approaches to enhance immunity in the neonate., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Cinicola, Conti, Terrin, Sgrulletti, Elfeky, Carsetti, Fernandez Salinas, Piano Mortari, Brindisi, De Curtis, Zicari, Moschese and Duse.)
- Published
- 2021
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24. Case Report: A Novel IL2RG Frame-Restoring Rescue Mutation Mimics Early T Cell Engraftment Following Haploidentical Hematopoietic Stem Cell Transplantation in a Patient With X-SCID.
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Steininger J, Leiss-Piller A, Geier CB, Rossmanith R, Elfeky R, Bra D, Pichler H, Lawitschka A, Zubarovskaya N, Artacker G, Matthes-Leodolter S, Eibl MM, and Wolf HM
- Subjects
- Allografts, Humans, Infant, Interleukin Receptor Common gamma Subunit genetics, Interleukin Receptor Common gamma Subunit immunology, Male, CD4-Positive T-Lymphocytes immunology, CD8-Positive T-Lymphocytes immunology, Hematopoietic Stem Cell Transplantation, Mutation, X-Linked Combined Immunodeficiency Diseases genetics, X-Linked Combined Immunodeficiency Diseases immunology, X-Linked Combined Immunodeficiency Diseases therapy
- Abstract
Mutations of the interleukin 2 receptor γ chain (IL2RG) result in the most common form of severe combined immunodeficiency (SCID), which is characterized by severe and persistent infections starting in early life with an absence of T cells and natural killer cells, normal or elevated B cell counts and hypogammaglobulinemia. SCID is commonly fatal within the first year of life, unless the immune system is reconstituted by hematopoietic stem cell transplantation (HSCT) or gene therapy. We herein describe a male infant with X-linked severe combined immunodeficiency (X-SCID) diagnosed at 5 months of age. Genetic testing revealed a novel C to G missense mutation in exon 1 resulting in a 3' splice site disruption with premature stop codon and aberrant IL2 receptor signaling. Following the diagnosis of X-SCID, the patient subsequently underwent a TCRαβ/CD19-depleted haploidentical HSCT. Post transplantation the patient presented with early CD8
+ T cell recovery with the majority of T cells (>99%) being non-donor T cells. Genetic analysis of CD4+ and CD8+ T cells revealed a spontaneous 14 nucleotide insertion at the mutation site resulting in a novel splice site and restoring the reading frame although defective IL2RG function was still demonstrated. In conclusion, our findings describe a spontaneous second-site mutation in IL2RG as a novel cause of somatic mosaicism and early T cell recovery following haploidentical HSCT., Competing Interests: Author ME was employed by company Biomedizinische Forschungs GmbH. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Steininger, Leiss-Piller, Geier, Rossmanith, Elfeky, Bra, Pichler, Lawitschka, Zubarovskaya, Artacker, Matthes-Leodolter, Eibl and Wolf.)- Published
- 2021
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25. Outcome of Non-hematological Autoimmunity After Hematopoietic Cell Transplantation in Children with Primary Immunodeficiency.
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Lum SH, Elfeky R, Achini FR, Margarit-Soler A, Cinicola B, Perez-Heras I, Nademi Z, Flood T, Cheetham T, Worth A, Qasim W, Amin R, Rao K, Chiesa R, Bredius RGM, Amrolia P, Abinun M, Hambleton S, Veys P, Gennery AR, Lankester A, and Slatter M
- Subjects
- Adolescent, Autoimmune Diseases diagnosis, Child, Child, Preschool, Disease Management, Disease Susceptibility, Female, Graft vs Host Disease diagnosis, Graft vs Host Disease epidemiology, Graft vs Host Disease etiology, Hematopoietic Stem Cell Transplantation adverse effects, Hematopoietic Stem Cell Transplantation methods, Humans, Immune Reconstitution, Incidence, Infant, Lymphocyte Count, Male, Primary Immunodeficiency Diseases therapy, Prognosis, Retrospective Studies, Risk Factors, Transplantation Chimera, Treatment Outcome, Autoimmune Diseases epidemiology, Autoimmune Diseases etiology, Autoimmunity, Primary Immunodeficiency Diseases complications, Primary Immunodeficiency Diseases epidemiology
- Abstract
Purpose: Knowledge of post-hematopoietic cell transplantation (HCT) non-hematological autoimmune disease (AD) is far from satisfactory., Method: This multicenter retrospective study focuses on incidence, risk factors, and outcomes of post-HCT AD in 596 children with primary immunodeficiency (PID) who were transplanted from 2009 to 2018., Results: The indications of HCT were severe combined immunodeficiency (SCID, n = 158, 27%) and non-SCID PID (n = 438, 73%). The median age at HCT was 2.3 years (range, 0.04 to 18.3 years). The 5-year overall survival for the entire cohort was 79% (95% cumulative incidence (CIN), 74-83%). The median follow-up of surviving patients was 4.3 years (0.08 to 14.7 years). The CIN of post-HCT AD was 3% (2-5%) at 1 year post-HCT, 7% (5-11%) at 5 years post-HCT, and 11% (7-17%) at 8 years post-HCT. The median onset of post-HCT AD was 2.2 years (0.12 to 9.6 years). Autoimmune thyroid disorder (n = 19, 62%) was the most common post-HCT AD, followed by neuromuscular disorders (n = 7, 22%) and rheumatological manifestations (n = 5, 16%). All patients but one required treatment for post-HCT AD. After multivariate analysis, age at transplant (p = 0.01) and T cell-depleted graft (p < 0.001) were significant predictors of post-HCT AD. None of the T cell-depleted graft recipients developed post-HCT AD. Patients with a lower CD3+ count at 6 months post-HCT had a significant higher incidence of post-HCT AD compared to disease controls. Graft-versus-host disease, viral infection, and donor chimerism had no association with post-HCT AD., Conclusion: Post-HCT AD occurred in 11% at 8 years post-HCT and its occurrence was associated with older age at HCT and unmanipulated graft.
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- 2021
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26. New insights into risk factors for transplant-associated thrombotic microangiopathy in pediatric HSCT.
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Elfeky R, Lucchini G, Lum SH, Ottaviano G, Builes N, Nademi Z, Battersby A, Flood T, Owens S, Cant AJ, Young H, Greener S, Walsh P, Kavanagh D, Annavarapu S, Rao K, Amrolia P, Chiesa R, Worth A, Booth C, Skinner R, Doncheva B, Standing J, Gennery AR, Qasim W, Slatter M, and Veys P
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- Child, Humans, Risk Factors, United Kingdom, Graft vs Host Disease epidemiology, Graft vs Host Disease etiology, Hematopoietic Stem Cell Transplantation adverse effects, Thrombotic Microangiopathies epidemiology, Thrombotic Microangiopathies etiology
- Abstract
This study aimed to identify a risk profile for development of transplant-associated thrombotic microangiopathy (TA-TMA) in children undergoing hematopoietic stem cell transplantation (HSCT). Between 2013 and 2016, 439 children underwent 474 HSCTs at 2 supraregional United Kingdom centers. At a median of 153 days post-HSCT, TA-TMA occurred among 25 of 441 evaluable cases (5.6%) with no evidence of center variation. Sex, underlying disease, intensity of the conditioning, total body irradiation-based conditioning, the use of calcineurin inhibitors, venoocclusive disease, and viral reactivation did not influence the development of TA-TMA. Donor type: matched sibling donor/matched family donor vs matched unrelated donor vs mismatched unrelated donor/haplo-HSCT, showed a trend toward the development of TA-TMA in 1.8% vs 6.1% vs 8.3%, respectively. Presence of active comorbidity was associated with an increased risk for TA-TMA; 13% vs 3.7% in the absence of comorbidity. The risk of TA-TMA was threefold higher among patients who received >1 transplant. TA-TMA rates were significantly higher among patients with acute graft-versus-host disease (aGVHD) grades III to IV vs aGVHD grade 0 to II. On multivariate analysis, the presence of active comorbidity, >1 transplant, aGVHD grade III to IV were risk factors for TA-TMA (odds ratio [OR]: 5.1, 5.2, and 26.9; respectively), whereas the use of cyclosporine A/tacrolimus-based GVHD prophylaxis was not a risk factor for TA-TMA (OR: 0.3). Active comorbidity, subsequent transplant, and aGVHD grades III to IV were significant risk factors for TA-TMA. TA-TMA might represent a form of a vascular GVHD, and therefore, continuing control of aGVHD is important to prevent worsening of TA-TMA associated with GVHD., (© 2020 by The American Society of Hematology.)
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- 2020
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27. Delaying haematopoietic stem cell transplantation in children with viral respiratory infections reduces transplant-related mortality.
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Ottaviano G, Lucchini G, Breuer J, Furtado-Silva JM, Lazareva A, Ciocarlie O, Elfeky R, Rao K, Amrolia PJ, Veys P, and Chiesa R
- Subjects
- Adolescent, Child, Child, Preschool, Disease-Free Survival, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Retrospective Studies, Survival Rate, Time Factors, Hematopoietic Stem Cell Transplantation, Respiratory Tract Infections mortality, Transplantation Conditioning, Virus Diseases mortality
- Abstract
Viral respiratory infections (VRIs) contribute to the morbidity and transplant-related mortality (TRM) after allogeneic haematopoietic stem cell transplantation (HSCT) and strategies to prevent and treat VRIs are warranted. We monitored VRIs before and after transplant in children undergoing allogeneic HSCT with nasopharyngeal aspirates (NPA) and assessed the impact on clinical outcome. Between 2007 and 2017, 585 children underwent 620 allogeneic HSCT procedures. Out of 75 patients with a positive NPA screen (12%), transplant was delayed in 25 cases (33%), while 53 children started conditioning with a VRI. Patients undergoing HSCT with a positive NPA screen had a significantly lower overall survival (54% vs. 79%) and increased TRM (26% vs. 7%) compared to patients with a negative NPA. Patients with a positive NPA who delayed transplant and cleared the virus before conditioning had improved overall survival (90%) and lower TRM (5%). Pre-HSCT positive NPA was the only significant risk factor for progression to a lower respiratory tract infection and was a major risk factor for TRM. Transplant delay, whenever feasible, in case of a positive NPA screen for VRIs can positively impact on survival of children undergoing HSCT., (© 2019 British Society for Haematology and John Wiley & Sons Ltd.)
- Published
- 2020
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28. Immune reconstitution following hematopoietic stem cell transplantation using different stem cell sources.
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Elfeky R, Lazareva A, Qasim W, and Veys P
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- Allografts, Animals, Humans, Antigens, CD19 immunology, CD4-Positive T-Lymphocytes immunology, Hematopoietic Stem Cell Transplantation, Hematopoietic Stem Cells immunology, Receptors, Antigen, T-Cell, alpha-beta immunology, Recovery of Function
- Abstract
Introduction : Adequate immune reconstitution post-HSCT is crucial for the success of transplantation, and can be affected by both patient- and transplant-related factors. Areas covered : A systematic literature search in PubMed, Scopus, and abstracts of international congresses is performed to investigate immune recovery posttransplant. In this review, we discuss the pattern of immune recovery in the post-transplant period focusing on the impact of stem cell source (bone marrow, peripheral blood stem cells, and cord blood) on immune recovery and HSCT outcome. We examine the impact of serotherapy on immune reconstitution and the need to tailor dosing of serotherapy agents when using different stem cell sources. We discuss new techniques being used particularly with cord blood and haploidentical grafts to improve immune recovery in each scenario. Expert opinion : Cord blood T cells provide a unique CD4+ biased immune reconstitution. Initial studies using targeted serotherapy with cord grafts showed improved immune recovery with limited alloreactivity. Two competing haploidentical approaches have developed in recent years including TCRαβ/CD19 depleted grafts and post-cyclophosphamide haplo-HSCT. Both approaches have comparable survival rates with limited alloreactivity. However, delayed immune reconstitution is still an ongoing problem and could be improved by modified donor lymphocyte infusions from the same haploidentical donor.
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- 2019
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29. New graft manipulation strategies improve the outcome of mismatched stem cell transplantation in children with primary immunodeficiencies.
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Elfeky R, Shah RM, Unni MNM, Ottaviano G, Rao K, Chiesa R, Amrolia P, Worth A, Flood T, Abinun M, Hambleton S, Cant AJ, Gilmour K, Adams S, Ahsan G, Barge D, Gennery AR, Qasim W, Slatter M, and Veys P
- Subjects
- Adolescent, Antigens, CD19 immunology, Child, Child, Preschool, Graft vs Host Disease etiology, Graft vs Host Disease immunology, Humans, Infant, Primary Immunodeficiency Diseases immunology, Receptors, Antigen, T-Cell, alpha-beta immunology, Stem Cell Transplantation adverse effects, Virus Diseases etiology, Virus Diseases immunology, Primary Immunodeficiency Diseases therapy, Stem Cell Transplantation methods
- Abstract
Background: Mismatched stem cell transplantation is associated with a high risk of graft loss, graft-versus-host disease (GvHD), and transplant-related mortality. Alternative graft manipulation strategies have been used over the last 11 years to reduce these risks., Objective: We investigated the outcome of using different graft manipulation strategies among children with primary immunodeficiencies., Methods: Between 2006 and 2017, 147 patients with primary immunodeficiencies received 155 mismatched grafts: 30 T-cell receptor (TCR) αβ/CD19-depleted grafts, 43 cord blood (CB) grafts (72% with no serotherapy), 17 CD34
+ selection with T-cell add-back grafts, and 65 unmanipulated grafts., Results: The estimated 8-year survival of the entire cohort was 79%, transplant-related mortality was 21.7%, and the graft failure rate was 6.7%. Posttransplantation viral reactivation, grade II to IV acute graft-versus-host disease (aGvHD), and chronic graft-versus-host disease (cGvHD) complicated 49.6%, 35%, and 15% of transplantations, respectively. Use of TCRαβ/CD19 depletion was associated with a significantly lower incidence of grade II to IV aGvHD (11.5%) and cGvHD (0%), although with a greater incidence of viral reactivation (70%) in comparison with other grafts. T-cell immune reconstitution was robust among CB transplants, although with a high incidence (56.7%) of grade II to IV aGvHD. Stable full donor engraftment was significantly greater at 80% among TCRαβ+ /CD19+ -depleted and CB transplants versus 40% to 60% among the other groups., Conclusions: Rapidly accessible CB and haploidentical grafts are suitable alternatives for patients with no HLA-matched donor. Cord transplantation without serotherapy and TCRαβ+ /CD19+ -depleted grafts produced comparable survival rates of around 80%, although with a high rate of aGvHD with the former and a high risk of viral reactivation with the latter that need to be addressed., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)- Published
- 2019
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30. Chronic Cholangiopathy Associated with Primary Immune Deficiencies Can Be Resolved by Effective Hematopoietic Stem Cell Transplantation.
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Hadžić N, Nademi Z, Deheragoda M, Zen Y, Elfeky R, Worth A, Veys P, Mieli-Vergani G, and Davies EG
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- Age Factors, Biopsy, Needle, Child, Preschool, Cholangiopancreatography, Endoscopic Retrograde methods, Cholangitis, Sclerosing diagnostic imaging, Cholangitis, Sclerosing epidemiology, Cholangitis, Sclerosing pathology, Chronic Disease, Cohort Studies, Databases, Factual, Disease Progression, Disease-Free Survival, Female, Hematopoietic Stem Cell Transplantation mortality, Hospitals, Pediatric, Humans, Immunohistochemistry, Infant, Male, Primary Immunodeficiency Diseases diagnosis, Prognosis, Retrospective Studies, Risk Assessment, Severity of Illness Index, Sex Factors, Survival Analysis, Treatment Outcome, United Kingdom, Cause of Death, Cholangitis, Sclerosing therapy, Hematopoietic Stem Cell Transplantation methods, Primary Immunodeficiency Diseases epidemiology, Primary Immunodeficiency Diseases therapy
- Abstract
Objectives: To investigate effects and outcome of hematopoietic stem cell transplantation (HSCT) on sclerosing cholangitis, in pediatric patients with different primary immunodeficiencies (PIDs)., Study Design: From databases in 2 tertiary centers for immunodeficiencies and liver disease, we have identified children with PIDs and sclerosing cholangitis, who have paired clinical, radiologic, and histologic information before and after HSCT and studied their clinical progress and outcome., Results: Seven of 13 children (53.8%) died at a median interval of 4 months (range, 3 months-5 years) after HSCT. However, 6 surviving children (46.2%) with different PIDs and less severe cholangiopathies showed an improvement in markers of liver injury within months of successful unrelated reduced intensity conditioning HSCT. The repeated native liver biopsy, performed in 4 patients at a median of 96 (range, 4-144) months post-HSCT, showed a considerable improvement. Biochemical markers of liver function in the survivors completely normalized after a median of 13 months (range, 2-48). All patients continue to have a mildly dilated extrahepatic biliary system on ultrasonography with no intrahepatic ductal changes on magnetic resonance cholangiography after a follow-up of median 18 years (range, 2-20)., Conclusions: Effective HSCT has the potential to improve biochemical and histologic features of cholangiopathy in children with PIDs, presumably by clearance of chronic infection following establishment of immune competence. However, careful patient selection is critical as advanced liver injury is often associated with serious complications and mortality., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)
- Published
- 2019
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31. The influence of stem cell source on transplant outcomes for pediatric patients with acute myeloid leukemia.
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Keating AK, Langenhorst J, Wagner JE, Page KM, Veys P, Wynn RF, Stefanski H, Elfeky R, Giller R, Mitchell R, Milano F, O'Brien TA, Dahlberg A, Delaney C, Kurtzberg J, Verneris MR, and Boelens JJ
- Subjects
- Adolescent, Child, Child, Preschool, Cord Blood Stem Cell Transplantation, Female, Graft vs Host Disease etiology, Hematopoietic Stem Cell Transplantation methods, Histocompatibility, Humans, Infant, Male, Quality of Life, Retrospective Studies, Siblings, Treatment Outcome, Unrelated Donors, Young Adult, Hematopoietic Stem Cell Transplantation standards, Leukemia, Myeloid, Acute therapy, Pediatrics methods, Tissue Donors
- Abstract
When hematopoietic stem cell transplant (HSCT) is necessary for children with acute myeloid leukemia (AML), there remains debate about the best stem cell source. Post-HSCT relapse is a common cause of mortality, and complications such as chronic graft versus host disease (cGVHD) are debilitating and life-threatening. To compare post-HSCT outcomes of different donor sources, we retrospectively analyzed consecutive transplants performed in several international centers from 2005 to 2015. A total of 317 patients were studied: 19% matched sibling donor (MSD), 23% matched unrelated donor (MUD), 39% umbilical cord blood (UCB), and 19% double UCB (dUCB) recipients. The median age at transplant was 10 years (range, 0.42-21 years), and median follow-up was 4.74 years (range, 4.02-5.39 years). Comparisons were made while controlling for patient, transplant, and disease characteristics. There were no differences in relapse, leukemia-free survival, or nonrelapse mortality. dUCB recipients had inferior survival compared with matched sibling recipients, but all other comparisons showed similar overall survival. Despite the majority of UCB transplants being HLA mismatched, the rates of cGVHD were low, especially compared with the well-matched MUD recipients (hazard ratio, 0.3; 95% confidence interval, 0.14-0.67; P = .02). The composite measure of cGVHD and leukemia-free survival (cGVHD-LFS), which represents both the quality of life and risk for mortality, was significantly better in the UCB compared with the MUD recipients (HR, 0.56; 95% confidence interval, 0.34-1; P = .03). In summary, the use of UCB is an excellent donor choice for pediatric patients with AML when a matched sibling cannot be identified., (© 2019 by The American Society of Hematology.)
- Published
- 2019
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32. Umbilical cord blood transplantation without in vivo T-cell depletion for children with MHC class II deficiency.
- Author
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Elfeky R, Furtado-Silva JM, Chiesa R, Rao K, Lucchini G, Amrolia P, Worth A, Gaspar B, Qasim W, and Veys P
- Subjects
- Child, Preschool, Female, Humans, Infant, Lymphocyte Depletion methods, Male, Cord Blood Stem Cell Transplantation methods, Histocompatibility Antigens Class II, Immunologic Deficiency Syndromes therapy
- Published
- 2018
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33. Non-posttransplant lymphoproliferative disorder malignancy after hematopoietic stem cell transplantation in patients with primary immunodeficiency: UK experience.
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Unni MNM, Elfeky R, Rao K, Nademi Z, Chiesa R, Amrolia P, Skinner R, Slater O, Worth A, Flood T, Abinun M, Hambleton S, Qasim W, Gaspar HB, Cant AJ, Gennery AR, Veys P, and Slatter MA
- Subjects
- Adolescent, Child, Child, Preschool, Female, Humans, Incidence, Male, Neoplasms etiology, Retrospective Studies, United Kingdom, Hematopoietic Stem Cell Transplantation, Immunologic Deficiency Syndromes therapy, Neoplasms epidemiology
- Published
- 2018
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34. T-cell receptor αβ + and CD19 + cell-depleted haploidentical and mismatched hematopoietic stem cell transplantation in primary immune deficiency.
- Author
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Shah RM, Elfeky R, Nademi Z, Qasim W, Amrolia P, Chiesa R, Rao K, Lucchini G, Silva JMF, Worth A, Barge D, Ryan D, Conn J, Cant AJ, Skinner R, Abd Hamid IJ, Flood T, Abinun M, Hambleton S, Gennery AR, Veys P, and Slatter M
- Subjects
- Alemtuzumab immunology, Antilymphocyte Serum immunology, Busulfan analogs & derivatives, Busulfan immunology, CD3 Complex immunology, Child, Child, Preschool, Female, Graft vs Host Disease immunology, Graft vs Host Disease prevention & control, Hematopoietic Stem Cell Transplantation, Humans, Infant, Male, Retrospective Studies, Thiotepa immunology, Transplantation Conditioning methods, Vidarabine analogs & derivatives, Vidarabine immunology, Antigens, CD19 immunology, Immunologic Deficiency Syndromes immunology, Immunologic Deficiency Syndromes therapy, Receptors, Antigen, T-Cell, alpha-beta immunology
- Abstract
Background: Allogeneic hematopoietic stem cell transplantation (HSCT) is used as a therapeutic approach for primary immunodeficiencies (PIDs). The best outcomes have been achieved with HLA-matched donors, but when a matched donor is not available, a haploidentical or mismatched unrelated donor (mMUD) can be useful. Various strategies are used to mitigate the risk of graft-versus-host disease (GvHD) and rejection associated with such transplants., Objective: We sought to evaluate the outcomes of haploidentical or mMUD HSCT after depleting GvHD-causing T-cell receptor (TCR) αβ CD3
+ cells from the graft., Methods: CD3+ TCRαβ+ /CD19+ depleted grafts were given in conditioned (except 3) children with PIDs. Treosulfan (busulfan in 1 patient), fludarabine, thiotepa, and anti-thymocyte globulin or alemtuzumab conditioning were used in 77% of cases, and all but 4 received GvHD prophylaxis., Results: Twenty-five patients with 12 types of PIDs received 26 HSCTs. Three underwent transplantation for refractory GvHD that developed after the first cord transplantation. At a median follow-up of 20.8 months (range, 5 month-3.3 years), 21 of 25 patients survived and were cured of underlying immunodeficiency. Overall and event-free survival at 3 years were 83.9% and 80.4%, respectively. Cumulative incidence of grade II to IV acute GvHD was 22% ± 8.7%. No case of visceral or chronic GvHD was seen. Cumulative incidences of graft failure, cytomegalovirus, and/or adenoviral infections and transplant-related mortality at 1 year were 4.2% ± 4.1%, 58.8% ± 9.8%, and 16.1% ± 7.4%, respectively. Patients undergoing transplantation with systemic viral infections had poor survival in comparison with those with absent or resolved infections (33.3% vs 100%)., Conclusion: CD3+ TCRαβ+ and CD19+ cell-depleted haploidentical or mMUD HSCT is a practical and viable alternative for children with a range of PIDs., (Copyright © 2017 American Academy of Allergy, Asthma & Immunology. All rights reserved.)- Published
- 2018
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35. Treosulfan and Fludarabine Conditioning for Hematopoietic Stem Cell Transplantation in Children with Primary Immunodeficiency: UK Experience.
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Slatter MA, Rao K, Abd Hamid IJ, Nademi Z, Chiesa R, Elfeky R, Pearce MS, Amrolia P, Worth A, Flood T, Abinun M, Hambleton S, Qasim W, Gaspar HB, Cant AJ, Gennery AR, and Veys P
- Subjects
- Adolescent, Adult, Alemtuzumab administration & dosage, Allografts, Busulfan administration & dosage, Child, Child, Preschool, Disease-Free Survival, Female, Follow-Up Studies, Graft vs Host Disease etiology, Graft vs Host Disease mortality, Graft vs Host Disease prevention & control, Humans, Infant, Male, Risk Factors, Survival Rate, United Kingdom, Vidarabine administration & dosage, Busulfan analogs & derivatives, Hematopoietic Stem Cell Transplantation, Immunologic Deficiency Syndromes mortality, Immunologic Deficiency Syndromes therapy, Transplantation Conditioning, Vidarabine analogs & derivatives
- Abstract
We previously published results for 70 children who received conditioning with treosulfan and cyclophosphamide (n = 30) or fludarabine (n = 40) before undergoing hematopoietic stem cell transplantation (HSCT) for primary immunodeficiency (PID). Toxicity was lower and T cell chimerism was better in the patients receiving fludarabine, but cohort numbers were relatively small and follow-up was short. Here we report outcomes of 160 children who received homogeneous conditioning with treosulfan, fludarabine, and, in most cases, alemtuzumab (n = 124). The median age at transplantation was 1.36 years (range, .09 to 18.25 years). Donors included 73 matched unrelated, 54 1 to 3 antigen-mismatched unrelated, 12 matched sibling, 17 other matched family, and 4 haploidentical donors. Stem cell source was peripheral blood stem cells (PBSCs) in 70, bone marrow in 49, and cord blood in 41. Median duration of follow-up was 4.3 years (range, .8 to 9.4 years). Overall survival was 83%. No patients had veno-occlusive disease. Seventy-four patients (46%) had acute GVHD, but only 14 (9%) greater than grade II. Four patients underwent successful retransplantation for graft loss or poor immune reconstitution. Another patient experienced graft rejection and died. There was no association between T cell chimerism >95% and stem cell source, but a significant association was seen between myeloid chimerism >95% and use of PBSCs without an increased risk of significant GVHD compared with other sources. All 11 patients with severe combined immunodeficiency diagnosed at birth were alive at up to 8.7 years of follow-up. Long-term studies are needed to determine late gonadotoxic effects, and pharmacokinetic studies are needed to identify whether specific targeting is advantageous. The combination of treosulfan, fludarabine, and alemtuzumab is associated with excellent results in HSCT for PID., (Copyright © 2017 The American Society for Blood and Marrow Transplantation. All rights reserved.)
- Published
- 2018
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36. Surveillance of catheter-associated urinary tract infection in 4 intensive care units at Alexandria university hospitals in Egypt.
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Talaat M, Hafez S, Saied T, Elfeky R, El-Shoubary W, and Pimentel G
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Bacteria classification, Bacteria drug effects, Bacteria isolation & purification, Catheter-Related Infections microbiology, Child, Child, Preschool, Egypt epidemiology, Female, Fungi classification, Fungi drug effects, Fungi isolation & purification, Hospitals, University, Humans, Incidence, Infant, Intensive Care Units, Male, Microbial Sensitivity Tests, Middle Aged, Prospective Studies, Risk Factors, Urinary Tract Infections microbiology, Young Adult, Catheter-Related Infections epidemiology, Urinary Tract Infections epidemiology
- Abstract
Background: We sought to measure the incidence rate of catheter-associated urinary tract infections (CAUTIs), identify risk factors associated with acquiring the infections; and identify the etiologic and antibiotic resistant patterns associated with CAUTIs in the intensive care units (ICUs) of a large University Hospital in Alexandria, Egypt., Methods: Prospective active surveillance of CAUTIs was conducted in 4 ICUs during a 13-month period from January 1, 2007 through January 31, 2008 in Alexandria University Hospital using the standard Centers for Disease Control National Nosocomial Infection Surveillance (NNIS) case definitions. Rates were expressed as the number of infections per 1000 catheter days., Results: During the study period, 757 patients were monitored after ICU admission, with either existing indwelling urinary catheters (239), or got catheters inserted after ICU admission (518), for a total duration of 16301 patient days, and 10260 patient catheter days. A total of 161 episodes of infection were diagnosed, for an overall rate of 15.7 CAUTIs per 1000 catheter days. Important risk factors associated with acquiring CAUTI were female gender (Relative risk (RR), 1.7; 95% confidence interval (CI); 1.7-4.3), and previous catheterization within the same hospital admission (RR, 1.6; 95% CI; 1.3-1.96). Patients admitted to the chest unit, patients =40 years, patients with prolonged duration of catheterization, prolonged hospital and ICU stay had a significantly higher risk of acquiring CAUTIs. Out of 195 patients who had their urine cultured, 188 pathogens were identified for 161 infected patients; 96 (51%) were Candida, 63 (33.5%) gram negatives, 29 (15.4%) gram positives. The prevalence of ESBL producers among K. pneumoniae and E. coli isolates was 56% (14/25) and 78.6% (11/14), respectively., Conclusion: Despite infection control policies and procedures, CAUTI rates remain a significant problem in Alexandria University hospital. Using the identified risk factors, tailored intervention strategies are now being implemented to reduce the rates of CAUTIs in these 4 ICUs., (2010 Association for Professionals in Infection Control and Epidemiology, Inc. All rights reserved.)
- Published
- 2010
- Full Text
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