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64 results on '"Zehra Fadoo"'

1. Response to comment on ‘causal factors influencing the quality of treatment and survival in wilms tumor: A retrospective investigation’

Author: Syed Ibrahim Bukhari, Zahra Saeed Ahmed, Javeria Saeed, Kiran Hilal, Zehra Fadoo, Naureen Mushtaq, Bilal Mazhar Qureshi, and Sadaf Altaf
Subjects: Wilms tumor, Survival, Outcomes, Quality improvement, LMIC, Pediatrics, RJ1-570
Published: 2024
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2. Trends in antimicrobial susceptibility pattern of infectious agents isolated during febrile neutropenia (FN) events of acute lymphoblastic leukemia (AL) patients from pakistan

Author: Adan Zubair, Seema Irfan, Afia Zafar, Mohammad Zeeshan, Usman Sheikh, Zehra Fadoo, Ammarah Baig, and Syed Waqas Tahir
Subjects: susceptibility, resistance, bacterial culture, sensitivity, Microbiology, QR1-502
Abstract: AIM: To evaluate trends in antimicrobial susceptibility patterns of infectious agents isolated in Febrile neutropenia patients of Acute Lymphoblastic Leukemia. BACKGROUND: FN is the most feared complication amongst hematopoietic cancer patients. The increasing emergence of highly resistant organisms highlights the urgent need for ongoing surveillance systems and antibiotic stewardship in managing patients with FN. METHODS: MR numbers with diagnosis of ''FN and ALL'' from January 1, 2020 to June 30, 2022 retrieved from HIMS. Each febrile neutropenic event of a patient was taken as a single case. Patients’ demographic, risk factors and culture sensitivity results with outcome were collected through the hospital patient care inquiry (PCI). Data was recorded on Excel sheet and descriptive analysis was done by calculating percentages. RESULTS: Total FN events 211Culture positive 73 (34.55%)Pediatric population 79.4%Males 58.9%Chemotherapy recipients 100%Relapse 42.46%Bone marrow transplant 5.47%Hospital stay > 10 days 46.57%Severe neutropenia for >1 week 84.93%Central lines 69.8%Previous hospitalization >4 in last 12 months 54.7%Most common source of infection Respiratory and GastrointestinalSusceptibility results presented in tables and charts in additional files CONCLUSION: Although medical advancements have reduced the infectious complications amongst ALL patients suffering from FN, the upcoming challenge is the ever-growing resistance patterns of pathogens. Therefore, it is important that antimicrobial susceptibility patterns of the pathogens are identified which can guide appropriate treatment, and eventually reduce morbidity and mortality.
Published: 2024
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3. Retinoblastoma with and without Extraocular Tumor Extension

Author: Swathi Kaliki, MD, Vijitha S. Vempuluru, MD, Ido Didi Fabian, MD, Elhassan Abdallah, MD, Shehu U. Abdullahi, MD, Rula A. Abdulqader, MD, Aminatu A. Abdulrahaman, MD, Sherif Abouelnaga, MD, Dupe S. Ademola-Popoola, FMCOph, FWACS, Adedayo Adio, FWACS, Mahmoud A. Afifi, MD, Armin R. Afshar, MD, Priyanka Aggarwal, MD, Ada E. Aghaji, FMCOph MSc, Alia Ahmad, MRCPCH UK, Marliyanti N.R. Akib, MD, Adeseye M. Akinsete, MBBS, Lamis Al Harby, MD, Saleh A. Al Mesfer, MD, Mouroge H. Al Ani, MD, Silvia Alarcón Portabella, MD, Safaa A.F. Al-Badri, MD, Ana Patricia A. Alcasabas, MD, Saad A. Al-Dahmash, MD, Amanda Alejos, MD, Ernesto Alemany-Rubio, MD, Amadou I. Alfa Bio, MD, Yvania Alfonso Carreras, MD, Christiane E. Al-Haddad, MD, Hamoud H.Y. Al-Hussaini, MD, MSc, Amany M. Ali, MD, Donjeta B. Alia, MD, Mazin F. Al-Jadiry, MD, Usama Al-Jumaly, MD, Hind M. Alkatan, MD, Charlotta All-Eriksson, MD, PhD, Ali A.R.M. Al-Mafrachi, FIBMS, Argentino A. Almeida, MD, Khalifa M. Alsawidi, MD, Athar A.S.M. Al-Shaheen, MD, Entissar H. Al-Shammary, MD, Doreen Amankwaa-Frempong, MBChB, Primawita O. Amiruddin, MD, Inggar Armytasari, MD, Nicholas J. Astbury, FRCS, FRCOphth, Hatice T. Atalay, MD, Eda Ataseven, MD, La-ongsri Atchaneeyasakul, MD, Rose Atsiaya, OCO, Rudolf Autrata, MD, PhD, Julia Balaguer, MD, PhD, Ruhengiz Balayeva, PhD, Honorio Barranco, MD, PhD, Paulina Bartoszek, MD, Katarina Bartuma, MD, PhD, Covadonga Bascaran, MD, MSc, Nikolaos E. Bechrakis, MD, Maja Beck Popovic, MD, Ainura S. Begimkulova, MD, Sarra Benmiloud, MD, Rokia C. Berete, MD, PhD, Jesse L. Berry, MD, Anirban Bhaduri, MD, Sunil Bhat, MBBS, MD, Arpita Bhattacharyya, MD, Eva M. Biewald, MD, Elaine Binkley, MD, Sharon Blum, MD, Nadia Bobrova, MD, H. Culver Boldt, MD, Maria Teresa B.C. Bonanomi, MD, PhD, Gabrielle C. Bouda, MD, Hédi Bouguila, MD, PhD, Rachel C. Brennan, MD, Bénédicte G. Brichard, MD, PhD, Jassada Buaboonnam, MD, Aléine Budiongo, MD, Matthew Burton, FRCOphth, Patricia Calderón-Sotelo, MD, Doris A. Calle Jara, MD, Jayne E. Camuglia, FRANZCO, Miriam R. Cano, MD, MSc, Michael Capra, FRCPI, Shani Caspi, MD, Nathalie Cassoux, MD, PhD, Guilherme Castela, MD, Luis Castillo, MD, Jaume Català-Mora, MD, PhD, Isabel Caviedes, MD, Arthika Chandramohan, MD, Guillermo L. Chantada, MD, PhD, Shabana Chaudhry, MD, Bhavna Chawla, MD, Wensi Chen, MD, Faraja S. Chiwanga, MSc, Tsengelmaa Chuluunbat, MD, PhD, Krzysztof Cieslik, MD, Antony Clark, FRANZCO, Ruellyn L. Cockcroft, MB ChB , M Med Paed, Codruta Comsa, MD, Maria G. Correa Llano, MD, Timothy W. Corson, PhD, Line Couitchere, MD, Kristin E. Cowan-Lyn, MD, MBBS, Monika Csóka, MD, PhD, Wantanee Dangboon, MD, Anirban Das, MD, Pranab Das, MD, Sima Das, MS, Jacquelyn M. Davanzo, BSN, BSPH, Alan Davidson, MBChB, MPhil, Sonia De Francesco, MD, Patrick De Potter, MD, PhD, Karina Q. Delgado, MD, PhD, Hakan Demirci, MD, Laurence Desjardins, MD, Rosdali Y. Diaz Coronado, MD, Helen Dimaras, PhD, Andrew J. Dodgshun, M Phil, Carla R. Donato Macedo, MD, Monica D. Dragomir, MD, PhD, Yi Du, MD, Magritha Du Bruyn, MD, Johannes P. Du Plessis, MMed (Paed), Gagan Dudeja, MBBS, MS, Katrin Eerme, MD, I Wayan Eka Sutyawan, MD, Asmaa El Kettani, MD, Amal M. Elbahi, MD, James E. Elder, MBBS, Alaa M. Elhaddad, MD, PhD, Moawia M.A. Elhassan, MD, Mahmoud M. Elzembely, MD, Connor Ericksen, MD, Vera A. Essuman, FWACS, Ted Grimbert A. Evina, MD, Ifeoma R. Ezegwui, FMCOph, FWACS, FAEH, Zehra Fadoo, MBBS, Adriana C. Fandiño, MD, Mohammad Faranoush, MD, Oluyemi Fasina, FWACS, Delia D.P.G. Fernández, MSc, Ana Fernández-Teijeiro, MD, PhD, Allen Foster, FRCOphth, Shahar Frenkel, MD, PhD, Ligia D. Fu, MD, Soad L. Fuentes-Alabi, MD, MPH, Juan L. Garcia, MSc, David García Aldana, MD, Henry N. Garcia Pacheco, MD, Jennifer A. Geel, MBChB, MMed, Fariba Ghassemi, MD, Ana V. Girón, MD, Marco A. Goenz, MD, Aaron S. Gold, OD, Hila Golberg, MD, Glen A. Gole, MD, FRANZCO, Nir Gomel, MD, Efren Gonzalez, MD, Graciela Gonzalez Perez, MD, Liudmira González-Rodríguez, MD, Malka Gorfine, PhD, Jaime Graells, MD, Pernille A. Gregersen, MD, Nathalia D.A.K. Grigorovski, MD, Koffi M. Guedenon, MD, D Sanjeeva Gunasekera, MD, Ahmet K. Gündüz, MD, Himika Gupta, MD, Sanjiv Gupta, MS, Vineeta Gupta, MD, Theodora Hadjistilianou, MD, Patrick Hamel, MD, Syed A. Hamid, FCPS, Norhafizah Hamzah, MSc, Eric D. Hansen, MD, J William Harbour, MD, M. Elizabeth Hartnett, MD, Murat Hasanreisoglu, MD, Sadiq Hassan, MD, FWACS, Shadab Hassan, FRCS, FCPS, Wojciech Hautz, MD, Huda A. Haydar, CHD, Stanislava Hederova, MD, Laila Hessissen, MD, Hoby Lalaina, MD, Suradej Hongeng, MD, Diriba F. Hordofa, MD, G. Baker Hubbard, MD, Marlies Hummlen, MD, Kristina Husakova, MD, Allawi N. Hussein Al-Janabi, MD, Affiong A. Ibanga, MB.BCh, FMCOph, Russo Ida, MD, Vesna R. Ilic, MD, Ziyavuddin Islamov, MD, Vivekaraj Jairaj, DNB, Teyyeb A. Janjua, MD, FCPS, FRCSEd, Irfan Jeeva, FRCOphth, Xunda Ji, MD, Dong Hyun Jo, MD, PhD, Michael M. Jones, MD, PhD, FRANZCO, Theophile B. Amani Kabesha, MD, PhD, Rolande L. Kabore, MD, Abubakar Kalinaki, MD, Pius Kamsang, MD, Mehmet Kantar, MD, Noa Kapelushnik, MD, Tamar Kardava, PhD, Rejin Kebudi, MD, Jonny Keomisy, MD, Tomas Kepak, MD, Petra Ketteler, MD, Zohora J. Khan, MD, Hussain A. Khaqan, MD, Vikas Khetan, FRCS, FACS, Alireza Khodabande, MD, Zaza Khotenashvili, MD, Jonathan W. Kim, MD, Jeong Hun Kim, MD, PhD, Hayyam Kiratli, MD, Tero T. Kivelä, MD, Artur Klett, MD, PhD, Irem Koç, MD, Jess Elio Kosh Komba Palet, MD, Dalia Krivaitiene, MD, PhD, Mariana Kruger, Mmed Paed, PhD, Kittisak Kulvichit, MD, Mayasari W. Kuntorini, MD, Alice Kyara, BA, Geoffrey C. Lam, FRANZCO, Scott A. Larson, MD, Slobodanka Latinović, MD, PhD, Kelly D. Laurenti, MD, Yotam Lavi, MD, PhD, Alenka Lavric Groznik, MD, Amy A. Leverant, MD, Cairui Li, MD, Kaijun Li, MD, Ben Limbu, MD, Chun-Hsiu Liu, MD, Quah Boon Long, FRCS (Ed), MMed ( Ophth), FAMS, Juan P. López, MD, Robert M. Lukamba, MD, Sandra Luna-Fineman, MD, Delfitri Lutfi, MD, Lesia Lysytsia, MD, Shiran Madgar, MD, George N. Magrath, MD, Amita Mahajan, MD, Puja Maitra, MD, Erika Maka, MD, Emil K. Makimbetov, MD, Azza M.Y. Maktabi, MD, Carlos Maldonado, MD, Ashwin Mallipatna, MD, Rebecca Manudhane, MD, Lyazat Manzhuova, MD, Nieves Martín Begue, MD, PhD, Sidra Masud, MBBS, Ibrahim O. Matende, MD, M. Med (Oph), Clarissa C.D.S. Mattosinho, MD, Marchelo Matua, BAPH, Ismail Mayet, MD, Freddy B. Mbumba, MD, MMed Paed, John D. McKenzie, MD, Azim Mehrvar, MD, Aemero A. Mengesha, MD, Vikas Menon, MD, Gary John V.D.D. Mercado, MD, Marilyn B. Mets, MD, Edoardo Midena, MD, PhD, Audra Miller, MD, Divyansh K.C. Mishra, DNB, Furahini G. Mndeme, MD, Ahmed A. Mohamedani, FRCPath, Mona T. Mohammad, MD, FRCS, Annette C. Moll, MD, PhD, Margarita M. Montero, MD, Claude Moreira, MD, PhD, Prithvi Mruthyunjaya, MD, MHS, Mchikirwa S. Msina, MMed Ophth, Gerald Msukwa, MMed Ophth, Sangeeta S. Mudaliar, DNB Pediatric, Hassan Muhammad, MD, Kangwa I. Muma, MMed Ophth, FCOphth, Francis L. Munier, MD, Timothy G. Murray, MD, MBA, Kareem O. Musa, FWACS, FMCOphth, FICO, Asma Mushtaq, MD, Anne A. Musika, MD, Hamzah Mustak, MD, Tajudeen Mustapha, MBBS, FWACS, Okwen M. Muyen, MD, Khumo H. Myezo, Msc, Gita Naidu, MMed Paed, PhD, Natasha Naidu, MBCHB, FCS Ophthalmol, Akshay Gopinathan Nair, MD, Sundaram Natarajan, FRCS, Larisa Naumenko, MD, PhD, Paule Aïda Ndoye Roth, MD PhD, Yetty M. Nency, MD, Vladimir Neroev, MD, PhD, Yvonne Ng, MBChB ( Auckland) , FRANZCO, Marina Nikitovic, MD, PhD, Elizabeth D. Nkanga, FMCOph, Henry E. Nkumbe, MD, Marcel N. Numbi, MD, Kalle Nummi, MD, Murtuza Nuruddin, FRCS, Mutale Nyaywa, MD, MMed Ophth, FCOphth, Chinsisi Nyirenda, MD, Ghislaine Obono-Obiang, MD, Scott C.N. Oliver, MD, Joaquin Ooporto, MD, Miriam Ortega-Hernández, MD, Alexander Oscar, MD, Diego Ossandon, MD, Halimah Pagarra, MD, PhD, Vivian Paintsil, FWACP, Luisa Paiva, MD, Mahesh Shanmugam Palanivelu, FRCSED, Ruzanna Papyan, MD, Raffaele Parrozzani, MD, PhD, Claudia R. Pascual Morales, MD, Katherine E. Paton, MD, FRCSC, Jacob Pe'er, MD, Jesús Peralta Calvo, MD, Sanja Perić, MD, PhD, Chau T.M. Pham, MD, Remezo Philbert, MD, David A. Plager, MD, Pavel Pochop, MD, PhD, Rodrigo A. Polania, MD, Vladimir Polyakov, MD, Jimena Ponce, MD, Ali O. Qadir, MD, Seema Qayyum, FCPS, Jiang Qian, MD, Ardizal Rahman, MD, Purnima Rajkarnikar, MD, Rajesh Ramanjulu, MD, Aparna Ramasubramanian, MD, Marco A. Ramirez-Ortiz, MD, MPH, Jasmeen K. Randhawa, BA, Léa Raobela, MD, Riffat Rashid, MS, M. Ashwin Reddy, FRCOphth, Lorna A. Renner, FRCPCH (UK), David Reynders, MD, Dahiru Ribadu, FMCOph, Petra Ritter-Sovinz, MD, Anna Rogowska, MD, Duangnate Rojanaporn, MD, Livia Romero, MD, Soma R. Roy, DCO, Raya H. Saab, MD, Svetlana Saakyan, MD, PhD, Ahmed H. Sabhan, MD, Mandeep S. Sagoo, FRCS (Ed), Azza M.A. Said, MD, Rohit Saiju, MD, Beatriz Salas, MD, Sonsoles San Román Pacheco, MD, Gissela L. Sánchez, MD, Alma Janeth Sanchez Orozco, MD, Phayvanh Sayalith, MD, Trish A. Scanlan, MRCPI, MSc, Christoph Schwab, MD, Ahad Sedaghat, MD, Rachna Seth, DNB MNAMS, Mariana Sgroi, MD, Ankoor S. Shah, MD, PhD, Shawkat A. Shakoor, MS, Manoj K. Sharma, MD, Sadik T. Sherief, MD, Carol L. Shields, MD, David Sia, MB ChB, FRANZCO, Sorath Noorani Siddiqui, MD, Sidi Sidi cheikh, MD, PhD, Sónia Silva, MD, Arun D. Singh, MD, Usha Singh, MS, Penny Singha, MD, Rita S. Sitorus, MD, PhD, Alison H. Skalet, MD, PhD, Hendrian D. Soebagjo, MD, PhD, Tetyana Sorochynska, MD, PhD, Grace Ssali, MD, Andrew W. Stacey, MD, Sandra E. Staffieri, PhD, Erin D. Stahl, MD, David M. Steinberg, PhD, David K. Stones, MBChB, FCPaed, Caron Strahlendorf, MD, Maria Estela Coleoni Suarez, MD, Sadia Sultana, FCPS, Xiantao Sun, MD, Rosanne Superstein, MD, Eddy Supriyadi, MD, PhD, Supawan Surukrattanaskul, MD, Shigenobu Suzuki, MD, PhD, Karel Svojgr, MD, PhD, Fatoumata Sylla, MD, Gevorg Tamamyan, MD, PhD, Deborah Tan, MBBS, Alketa Tandili, MD, PhD, Jing Tang, MD, Fanny F. Tarrillo Leiva, MD, Maryam Tashvighi, MD, Bekim Tateshi, MD, PhD, Kok Hoi Teh, MD, Edi S. Tehuteru, MD, Luiz F. Teixeira, MD, Manca Tekavcic Pompe, MD, PhD, Abdullah Dahan M. Thawaba, MD, Tuyisabe Theophile, MSc, Helen Toledano, MBChB, Doan L. Trang, MD, Fousseyni Traoré, MD, Devjyoti Tripathy, MD, Samuray Tuncer, MD, Harba Tyau-Tyau, MD, Ali B. Umar, MD, FMCPath, Emel Unal, MD, Ogul E. Uner, BA, Steen F. Urbak, MD, PhD, Tatiana L. Ushakova, MD, Rustam H. Usmanov, MD, Sandra Valeina, MD, Paola Valente, MD, Milo van Hoefen Wijsard, MD, Jacqueline Karina Vasquez Anchaya, MD, Leon O. Vaughan, FRCS (Ed), Nevyana V. Veleva-Krasteva, MD, PhD, Nishant Verma, MD, Andi A. Victor, MD, PhD, Maris Viksnins, MD, Edwin G. Villacís Chafla, MD, Victor M. Villegas, MD, Victoria Vishnevskia-Dai, MD, Keith Waddell, DM, FRCP, FRCS, FRCOphth, Amina H. Wali, MD, FMCOph Nigeria, Yi-Zhuo Wang, MD, Nutsuchar Wangtiraumnuay, MD, FICO, Julie A. Wetter, MMed Rad Onc, FCRad Onc, Widiarti P. Riono, MD, Matthew W. Wilson, MD, Amelia D.C. Wime, MD, Atchareeya Wiwatwongwana, MD, Damrong Wiwatwongwana, MD, Charlotte Wolley Dod, MD, Emily S. Wong, FCOphth HK, FHKAM, Phanthipha Wongwai, MD, PhD, Si-qi Wu, MSc, Daoman Xiang, MD, PhD, Yishuang Xiao, MSc, Bing Xu, MD, Kang Xue, MD, Antonio Yaghy, MD, Jason C. Yam, FRCSEd, Huasheng Yang, MD, Jenny M. Yanga, MD, Muhammad A. Yaqub, MD, FCPS, FRCSEd, Vera A. Yarovaya, MD, Andrey A. Yarovoy, MD, PhD, Huijing Ye, MD, Roberto I. Yee, MD, Yacoub A. Yousef, MD, Putu Yuliawati, MD, Arturo M. López, MD, Ekhtelbenina Zein, MD, Yi Zhang, MD, PhD, Katsiaryna Zhilyaeva, MD, Nida Zia, MBBS, MCPS, Othman A.O. Ziko, MD, PhD, Marcia Zondervan, MBA, Sabrina Schlüter, MD, and Richard Bowman, FRCOphth
Subjects: External beam radiotherapy, Extraocular extension, Multimodal treatment, Retinoblastoma, Tumor, Ophthalmology, RE1-994
Abstract: Purpose: To study the treatment and outcomes of children with retinoblastoma (RB) with extraocular tumor extension (RB-EOE) and compare them with RB without extraocular tumor extension (RB-w/o-EOE). Design: Multicenter intercontinental collaborative prospective study from 2017 to 2020. RB-EOE cases included those with overt orbital tumor extension in treatment-naive patients. Cases with microscopic orbital extension detected postenucleation were excluded from the study. Participants: A total of 319 children with RB-EOE and 3116 children with RB-w/o-EOE. Intervention: Chemotherapy, enucleation, exenteration, radiotherapy. Main Outcome Measures: Systemic metastasis and death. Results: Of the 3435 RB patients included in this study, 309 (9%) were from low-income countries (LIC), 1448 (42%) from lower-middle income, 1012 (29%) from upper-middle income, and 666 (19%) patients from high-income countries. There was an inverse relationship between the percentage of RB-EOE and national income level, with 96 (31%) patients from LIC, 197 (6%) lower-middle income, 20 (2%) upper-middle income, and 6 (1%) patients from high-income countries (P = 0.0001). The outcomes were statistically significant for RB-EOE compared with RB-w/o-EOE: systemic metastasis (32% vs. 4% respectively; P = 0.0001) and metastasis-related death (63% vs. 6% respectively; P = 0.0001). Multimodal treatment was the most common form of treatment (n = 177; 54%) for RB-EOE, with most cases undergoing a combination of intravenous chemotherapy and enucleation (n = 97; 30%). Adjuvant external beam radiotherapy (EBRT) after surgery (enucleation/orbital exenteration) was given in only 68 (21%) cases. Kaplan–Meier analysis for systemic metastasis and metastasis-related death in RB-EOE was 28% and 57% at 1 year, 29% and 60% at 2 years, and 29% and 61% at 3 years, respectively. Cox regression analysis revealed that the risk of death from RB-EOE was greater in patients aged >4 years than
Published: 2025
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4. Development and testing of a videogame intervention for symptom management among 8–18 years old children with cancer: a study protocol

Author: Rubina Barolia, Zehra Fadoo, Sehrish Sajjad, Raisa B Gul, Saleem Sayani, and Ahmed N Abbasi
Subjects: Pediatrics, RJ1-570
Abstract: Introduction Evidence shows that cancer treatment-related symptoms could be managed effectively in 8–18 years old children through Digital Health Interventions (DHIs), consequently improving their health-related quality of life (HRQOL). However, limited research is available about digitally mediated educative health interventions for children with cancer from lower-middle income countries like Pakistan. This study aims to develop a videogame intervention for children with cancer and test the clinical efficacy of the videogame concerning HRQOL and cancer treatment-related symptoms. Moreover, the following feasibility outcomes will be recorded: acceptability, appropriateness, cost, feasibility and intervention fidelity.Methods and analysis An exploratory sequential mixed methods design is used in this study. In the first phase of the study, we interviewed 28 participants (14 child–parent dyads) and assessed their symptom experiences affecting children’s HRQOL. Moreover, their preferences for the development of the videogame were also elicited. Based on the findings from relevant literature and the interviews, we developed the videogame in collaboration with clinical and digital experts in the study’s second phase. In the third phase of the study, a pilot randomised controlled trial will be conducted at a Tertiary Care Hospital in Karachi, Pakistan. There will be two groups: the intervention group and the control group. The intervention group children will receive the videogame application for 8 weeks, during which symptom management strategies will be taught to them. Children in the attention control group will receive weekly WhatsApp messages on healthy behaviours.The primary outcome will be the HRQOL of children, and the secondary outcome will be cancer symptoms frequency and distress. These outcomes will be assessed preintervention and 8 weeks post intervention. The feasibility outcomes will be assessed quantitatively and qualitatively through a questionnaire, videogame dashboard, interviews with a subset of intervention group child–parent dyads and a focus group discussion with nurses and doctors, post intervention, respectively.Ethics and dissemination The study has been approved by the Ethics Review Committee of the Aga Khan University (2022-6833-21251). Data are accessible only to the research team in a secure form. The findings will be disseminated through publications.Trial registration number ClinicalTrials.gov Identifier NCT05796895, registered in April 2023.
Published: 2024
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5. Causal factors influencing quality of treatment and survival in Wilms Tumor: A retrospective investigation

Author: Syed Ibrahim Bukhari, Zahra Saeed Ahmed, Javeria Saeed, Kiran Hilal, Zehra Fadoo, Naureen Mushtaq, Bilal Mazhar Qureshi, and Sadaf Altaf
Subjects: Treatment abandonment, Nephroblastoma, Survival, Outcomes, Quality improvement, LMIC, Pediatrics, RJ1-570
Abstract: Background: Wilms Tumor (WT) is a highly curable cancer if treatment is appropriate and timely. The outcomes and prognostic factors in a large low- and middle-income country (LMIC) tertiary center were assessed. Materials and methods: Retrospective review of data of all patients, 0–15 years diagnosed between 2010 and 2020 with WT. Kaplan Meier curves were used for survival analysis, and the chi-square test was used for multivariate analysis. Results: Of the 40 patients enrolled (median age: 38 months) in the cohort, 10 had metastatic disease. The most common site of metastasis was lungs (6/10). Nine (22.5%) abandoned treatment. Large tumor (>500 ml) volume was found in half the patients at diagnosis. The majority of patients were treated per the SIOP approach. Out of 34 who went for surgery, 31 received neoadjuvant chemotherapy with tumor shrinkage to less than 500 ml in 26/31 (80%). Maximum tumor shrinkage was observed in the SIOP low-risk group (p
Published: 2023
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6. Outcome of sepsis in pediatric oncology patients admitted in pediatric intensive care unit: A developing country perspective

Author: Amna Afzal Saeed, Sadia Usman, Zehra Fadoo, and Qalab Abbas
Subjects: Pediatrics, RJ1-570
Abstract: Objective: To determine the outcome of sepsis i.e. severe sepsis and septic shock in pediatric oncology patients admitted in pediatric intensive care unit (PICU). Methods: Retrospective review of medical records of all children (1 month–16 years) having primary oncological diagnosis admitted in PICU with sepsis from January 2008 to June 2017 was done after ethical review committee approval. Data was collected on a structured proforma and included demographic details, clinical and laboratory/microbiological data and stage of chemotherapy, outcome (survived/expired). Results: Total 63 patients were identified, 42 (66.7%) were males, and median age was 93 months. Primary oncological diagnosis included Leukemia (n = 45, 71.4%), lymphoma (n = 12, 19.0%), solid tumor (n = 3, 4.2%), central nervous system tumor (n = 2, 3.2%) Out of the 63 admissions, 34.9% (n = 22) went into septic shock and 52.4% (n = 33) survived after admission to PICU. The most commonly found microbial organisms were gram positive cocci, followed by gram negative rods. Organ dysfunction, use of mechanical ventilation and septic shock were associated with mortality (p
Published: 2019
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7. Hepatitis C at presentation in a newly diagnosed infant with B Acute Lymphoblastic Leukemia

Author: Qurratulain Shahood Ahmed, Zehra Fadoo, Kamran Sadiq, and Sadaf Altaf
Subjects: Pediatrics, RJ1-570
Abstract: We report the unique case of a nine-month old patient diagnosed with B- Cell Acute Lymphoblastic Leukemia also positive for Hepatitis C virus, genotype 3 and a high viral load. With no existent literature to guide treatment of Hepatitis C in context of Infant Leukemia, treatment with direct-acting antivirals was deferred. A conservative approach to Hepatitis C was sought while he was undergoing chemotherapy, with the expectation of a spontaneous viral clearance, as has been documented for a quarter of otherwise healthy infants. Keywords: Hepatitis C, Infant leukemia, Direct acting antivirals
Published: 2018
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8. EWING’S SARCOMA: APPROACHING CENTURY SINCE THIS BONE CANCER MADE NEWS

Author: Zehra Fadoo and Mir Ibrahim Sajid
Subjects: Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Published: 2019
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9. Comparative Analysis of Surgical Site Infections in Pediatric Brain Tumor Patients: Hygiene Practices, Risk Factors, and Implications for Healthcare Costs and Mortality

Author: Syed Ibrahim Bukhari, Muhammad Sohaib Shahid, Naureen Mushtaq, Hira Saleem, Altaf Ali Laghari, Zahra Saeed Ahmed, Shayan Anwar, Farrah Bashir, Zehra Fadoo, Fatima Mir, and Sadaf Altaf
Subjects: neuro-oncology, pediatric, pediatric brain tumors, surgical site infection, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
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10. The state of health in Pakistan and its provinces and territories, 1990–2019: a systematic analysis for the Global Burden of Disease Study 2019

Author: Assad Hafeez, William James Dangel, Samuel M Ostroff, Ayyaz Gul Kiani, Scott D Glenn, Jaffar Abbas, Muhammad Sohail Afzal, Saira Afzal, Sajjad Ahmad, Ali Ahmed, Haroon Ahmed, Liaqat Ali, Muhammad Ali, Zahid Ali, Muhammad Arshad, Tahira Ashraf, Zulfiqar A Bhutta, Sadia Bibi, Zahid A Butt, Jai K Das, Zehra Fadoo, Asif Hanif, Khezar Hayat, Ayesha Humayun, Khalid Iqbal, Usman Iqbal, Nauman Khalid, Ejaz Ahmad Khan, Muhammad Shahzeb Khan, Ahmad Azam Malik, Muhammad Naveed, Shumaila Naz, Robina Khan Niazi, Zahra Zahid Piracha, Umar Saeed, Muhammad Salman, Zainab Samad, Muhammad Arif Nadeem Saqib, Syed Mahboob Shah, Izza Shahid, Masood Ali Shaikh, Hina Shamshad, Kanwar Hamza Shuja, Muhammad Suleman, Anayat Ullah, Irfan Ullah, Saif Ullah, Sana Ullah, Yasir Waheed, Abdul Waris, Simon I Hay, Christopher J L Murray, and Ali H Mokdad
Subjects: General Medicine
Published: 2023
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11. Immuno-AML, a Novel Immunogenomic Classifier Predicting Chemotherapy Response in Pediatric Patients with AML

Author: Aesha Ibrahim Khalel Ali, Darawan Rinchai, Sara Deola, Mohammed Elanbari, Dhanya Kizhakayil, Shimaa Mohammed Sherif Khedr, Mohammed Toufiq, Tommaso Mina, Kulsoom Ghias, Zehra Fadoo, Sheanna M. Herrera, Che-Ann Lachica, Blessing Dason, Anila Ejaz, Elkhansa E. Elgaali, Ayman Saleh, Davide Bedognetti, and Chiara Cugno
Subjects: Immunology, Cell Biology, Hematology, Biochemistry
Published: 2022
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12. Treatment outcomes and prognostic factors of childhood acute lymphoblastic leukemia in a low–middle income population: A multi‐institutional report from Pakistan

Author: Imran Nisar, Shahira Shahid, Fatimah Yousuf, Laila Saleem Lakhani, Shamvil Ashraf, Uzma Imam, Junaid Zaheer, Asim Belgaumi, and Zehra Fadoo
Subjects: Oncology, Pediatrics, Perinatology and Child Health, Hematology
Published: 2022
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13. Outcome of sepsis in pediatric oncology patients admitted in pediatric intensive care unit: A developing country perspective

Author: Qalab Abbas, Amna Afzal Saeed, Zehra Fadoo, and Sadia Usman
Subjects: Pediatric intensive care unit, Mechanical ventilation, medicine.medical_specialty, business.industry, Septic shock, Medical record, medicine.medical_treatment, Organ dysfunction, lcsh:RJ1-570, lcsh:Pediatrics, Hematology, medicine.disease, Sepsis, 03 medical and health sciences, 0302 clinical medicine, Oncology, 030220 oncology & carcinogenesis, Internal medicine, Pediatrics, Perinatology and Child Health, Etiology, Medicine, Stage (cooking), medicine.symptom, business, 030215 immunology
Abstract: Objective: To determine the outcome of sepsis i.e. severe sepsis and septic shock in pediatric oncology patients admitted in pediatric intensive care unit (PICU). Methods: Retrospective review of medical records of all children (1 month–16 years) having primary oncological diagnosis admitted in PICU with sepsis from January 2008 to June 2017 was done after ethical review committee approval. Data was collected on a structured proforma and included demographic details, clinical and laboratory/microbiological data and stage of chemotherapy, outcome (survived/expired). Results: Total 63 patients were identified, 42 (66.7%) were males, and median age was 93 months. Primary oncological diagnosis included Leukemia (n = 45, 71.4%), lymphoma (n = 12, 19.0%), solid tumor (n = 3, 4.2%), central nervous system tumor (n = 2, 3.2%) Out of the 63 admissions, 34.9% (n = 22) went into septic shock and 52.4% (n = 33) survived after admission to PICU. The most commonly found microbial organisms were gram positive cocci, followed by gram negative rods. Organ dysfunction, use of mechanical ventilation and septic shock were associated with mortality (p
Published: 2019

14. Pediatric hematology oncology during SARS‐CoV‐2: A brief communication of 28 patients and changes in clinical practice from a single institute in Pakistan

Author: Asim F. Belgaumi, Mir Ibrahim Sajid, Sadaf Altaf, Zehra Fadoo, and Naureen Mushtaq
Subjects: medicine.medical_specialty, 2019-20 coronavirus outbreak, Hematology, Coronavirus disease 2019 (COVID-19), business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Pediatric Hematology/Oncology, MEDLINE, COVID-19, Clinical Practice, Oncology, Internal medicine, Pediatrics, Perinatology and Child Health, Pandemic, Emergency medicine, medicine, Humans, Pakistan, Pediatrics, Perinatology, and Child Health, Child, business, Pandemics
Published: 2020
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15. Pediatric Cancer Management during SARS-CoV-2: A Brief Communication of 28 Patients and Changes in Clinical Practice from a Single Leading Institute in Pakistan

Author: Sadaf Altaf, Mir Ibrahim Sajid, Asim F. Belgaumi, Zehra Fadoo, and Naureen Mushtaq
Subjects: medicine.medical_specialty, business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Thalassemia, Neutropenia, medicine.disease, Group A, Pediatric cancer, Group B, The primary diagnosis, Clinical Practice, Internal medicine, medicine, business
Abstract: All tested patients were classified into two groups, Group A (patients who were tested positive) and Group B (patients who were tested negative). A total of 28 patients were evaluated. The primary diagnosis of patients in Group A was B-Cell ALL and Thalassemia Major (50% each), whereas, in Group B, the primary diagnosis was B-Cell ALL (46.1%) and AML. The most prevalent symptoms warranting testing in Group A were fever (100%) and cough (50%); Group B the most common symptoms were fever (53.8%), cough (26.9%), and neutropenia (15.4%). No mortality or significant morbidity was reported in either of the cohorts.
Published: 2020
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16. Novel Target Genes and Epigenetic Pathways Involved in High Risk Acute Lymphoblastic Leukemia

Author: Giusy Gentilcore, Ramzi Temanni, Tommaso Mina, Chiara Cugno, Muhammad Elnaggar, Fazulur R Vempalli, Sara Deola, Sheanna M Herrera, Che-Ann Lachica, Irene Cavattoni, Dhanya Kizhakayil, Zehra Fadoo, Kulsoom Ghias, Irene Pusceddu, Patrizia Comoli, Ayman Saleh, Najeeb Syed, and Anila Ejaz
Subjects: Lymphoblastic Leukemia, Immunology, Cancer research, Cell Biology, Hematology, Epigenetics, Biology, Biochemistry, Gene
Abstract: Acute leukemias (AL) is a major cause of cancer death in young age. Extensive research is being conducted to identify novel and innovative approaches for leukemia treatment. Transcriptomic and epigenetic studies might help to discover potential targets, paving the way for molecular-targeted therapies. We analyzed a cohort of 34 AL at diagnosis: 10 pediatric Acute Lymphoblastic Leukemias (8 B-cell, 1 T-cell and 1 bi-phenotypic ALL), 4 young adult ALL (2 B-cell and 2 T-cell phenotype), and 20 pediatric Acute Myeloid Leukemias (AML: 3 M1, 4 M2, 1 M3, 5 M4, 7 M5) with an average blasts population higher than 80% (85+/-11% in ALL; 83+/-13% in AML). Six out of 14 ALL, and 12 out of 20 AML fell under "high risk" category according to clinical standard risk stratification algorithms. On all patients we performed mRNA sequencing (20-million-reads on Illumina Hiseq 4000). Analyses were performed adjusting The expression of a set of 800 microRNAs (miRNAs) was evaluated by means of Nanostring miRNA panel. Expression signatures and associations among the different risk groups were calculated with t-tests and linear regression analyses. Applying stringent FDR statistical measurement, we discovered 3 genes that significantly differentiate the transcriptomic profile of high vsintermediate/standard-risk ALL in mRNAseq. The expression of PGR3 (p53 Responsive Gene) and long-non-coding RNAs (lncRNAs) ENSG00000228737 and ENSG00000253174 were respectively 45.5, 4.2 and 3.9 time downregulated in high-risk ALL. To explore more deeply the apoptosis pathway in ALL, we measured Tp53 expression and found it significantly downregulated in the high-risk vsintermediate-standard-risk ALL (p= Tp53 dysregulation is a known hallmark for tumor progression; Tp53 mutations - ranging from 1-2% to 10% in pediatric and adult ALs - correlate with worse prognosis. However, in our cohorts, this gene signature was found significant only in high-risk ALL, homogeneously distinguishing them from intermediate/standard-risk ALL. Transcriptome clinical variant analyses excluded pathogenetic known variants that could explain such marked difference. Also, it is unlikely that somatic genetic mutations acquired by the tumor would explain such a homogeneous behavior of high-risk ALL. Thus, we analyzed the p53 regulatory pathway. Interestingly we found that miRNAs known to be involved in p53 control were significantly upregulated in high-risk vs intermediate-standard-risk ALL (p We found PRG3 and Tp53 significantly downregulated in high-risk ALL, with PRG3 expression 45 times lower than intermediate/low-risks ALL. Deeper analyses pointed out to an apoptosis control program not generated by a somatic mutation in the tumor, nor a germline clinical patient variant, but by an epigenetic mechanism. We are currently validating these data in a larger cohort, adding also methylome analyses. It will be interesting also to explore the function of the lncRNAs markedly downregulated in our cohort, whose functions are still unknown or partially known. Because of the small numerosity of the ALL high-risk cohort, we were not able to dissect high-risk young adults (4/6) from pediatric ALL (2/6). Although the homogeneity of data suggests a shared apoptosis control mechanism, it will be worthy to explore in a larger cohort whether the general worse prognosis of young adults/adults vs pediatric ALL is at least partially explained by this mechanism. Disclosures No relevant conflicts of interest to declare.
Published: 2020
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17. A Wrinkle in Time: How Has Management of Pediatric Cancers Changed During COVID-19 in Pakistan?

Author: Sadaf Altaf, Mir Ibrahim Sajid, Zehra Fadoo, Naureen Mushtaq, and Asim F. Belgaumi
Subjects: 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Pandemic, Medicine, Cancer, medicine.symptom, business, medicine.disease, Virology, Wrinkle, Virus
Abstract: As the COVID-19 pandemic spreads across Pakistan, hospitals have undertaken measures in preventing exposure-driven spread of the virus With cancer patients alr
Published: 2020
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18. Metachronous translocation renal cell carcinoma in a child with successfully treated medulloblastoma

Author: Zehra Fadoo, Muhammad Arshad, Ayesha Saleem, Nasir Ud Din, and Mir Ibrahim Sajid
Subjects: 0301 basic medicine, Male, medicine.medical_specialty, medicine.medical_treatment, Lesion, 03 medical and health sciences, 0302 clinical medicine, Renal cell carcinoma, Neuroblastoma, Pediatric surgery, medicine, Humans, Cerebellar Neoplasms, Child, Carcinoma, Renal Cell, Medulloblastoma, Kidney, business.industry, General Medicine, medicine.disease, Nephrectomy, Kidney Neoplasms, 030104 developmental biology, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Pediatrics, Perinatology and Child Health, Neurology (clinical), Radiology, Neurosurgery, medicine.symptom, Neoplasm Recurrence, Local, business
Abstract: The most common primary CNS tumor in children is the medulloblastoma, which generally occurs in the posterior fossa and can spread through the CNS and spinal cord. Although the recurrence of renal cell carcinoma as a secondary tumor to neuroblastoma has been reported with successful anti-neoplastic treatment, the rare occurrence of a child who initially had medulloblastoma and then developed translocation renal cell carcinoma has never been reported before. We report the case of a 12-year-old boy who initially presented with complaints of vomiting and headache. An MRI head confirmed the presence of 4 × 4 × 3 cm lesion which was resected completely and histopathology report confirmed the diagnosis of medulloblastoma Grade IV. Four years later, the child came for a follow-up visit and during routine screening, a CT scan showed heterogeneous lesion arising from the lower pole calyx of right kidney. The patient was referred to pediatric surgery for right radical nephrectomy involving the right adrenal gland. The histopathology report was consistent with the diagnosis of translocation renal cell carcinoma. Central nervous system (CNS) tumors remain the leading cause of death among pediatric neoplasms. We advise genetic testing of index cases and the establishment of an international tumor registry for a challenging disease.
Published: 2019

19. Outcome of tumor lysis syndrome in pediatric patients with hematologic malignancies – a single-center experience from Pakistan

Author: Zehra Fadoo, Mohammad Faizan Zahid, Anwar ul Haq, Armaghan-e-Rehman Mansoor, Mujtaba Mubashir, and Arshalooz Jamila Rahman
Subjects: Tumor lysis syndrome, Oncology, medicine.medical_specialty, business.industry, Internal medicine, medicine, Hematology, medicine.disease, business, Single Center, Outcome (game theory)
Published: 2016
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20. Intracranial tumors in children: a 10-year review from a single tertiary health-care center

Author: Zehra Fadoo, Naureen Mushtaq, Quratulain Riaz, Mahadev Lohano, and Ehsun Naeem
Subjects: Male, Pediatrics, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Brain tumor, Disease, 03 medical and health sciences, 0302 clinical medicine, Epidemiology, medicine, Humans, Pakistan, Lost to follow-up, Child, Retrospective Studies, Medulloblastoma, business.industry, Brain Neoplasms, Infant, Newborn, Infant, General Medicine, medicine.disease, Radiation therapy, 030220 oncology & carcinogenesis, Child, Preschool, Pediatrics, Perinatology and Child Health, Vomiting, Female, Neurology (clinical), Neurosurgery, medicine.symptom, business, 030217 neurology & neurosurgery
Abstract: Brain tumors are the second most common pediatric malignancy and the most common cause of cancer-related mortality and morbidities. Major advances in terms of surgery, radiation, and chemotherapy have led to better outcomes in developed countries. Delayed diagnosis, advanced disease at presentation, late referrals, nosocomial infections, delays to radiotherapy, and poor support services are the major reasons for poorer outcomes in developing countries. Little is known about the profile of brain tumors in Pakistan. This study aims to evaluate the epidemiology, management, and clinical outcomes of children with brain tumors in Pakistan in a single tertiary care center. All children (0–16 years) with primary CNS tumors from 2004 to 2014 at Aga Khan University Hospital were reviewed retrospectively for clinical data, demographics, radiological findings, management, and outcome. One hundred seventy-five children were included in the study. Male to female ratio was 1.4:1. Most of the patients were in 5–10 years age group (38.9%). Most common presenting complains were headache 115 (65.7%) and vomiting 100 (57.1%). Predominant site was infratentorial 93 (53%). Glial tumors were 105 (60%) followed by embryonal 40(22.9%), craniopharyngiomas 25 (14.3%), and germ cell 1 (0.6%). Astrocytomas (25.7%) were the most common glial tumors while medulloblastoma (15.4%) was the most common embryonal tumor. Majority of the patients underwent surgical resection (78.8%). Radiation was given to 47 (26.8%) patients. A half of the patients, 89 (50%), were lost to follow-up. Forty-two (24%) patients expired, 20 (11.4%) are alive with residual disease while 15 patients (8.5%) were cured with no evidence of recurrence and regular follow-ups. This is the only study from Pakistan showing demographics of the childhood brain tumors. Significant improvement needs to be made for timely diagnosis, early referrals, and collaborated team efforts with multidisciplinary tumor board to improve outcome.
Published: 2019

21. Genetic aberrations involved in relapse of pediatric acute myeloid leukemia: A literature review

Author: Zehra Fadoo, Naveera Zafar, and Kulsoom Ghias
Subjects: Oncology, medicine.medical_specialty, Myeloid, Disease, 03 medical and health sciences, 0302 clinical medicine, Recurrence, Internal medicine, medicine, Humans, 030212 general & internal medicine, Child, Gene, business.industry, Remission Induction, Induction chemotherapy, Myeloid leukemia, General Medicine, SATB1, medicine.disease, Prognosis, PTPN11, Leukemia, Leukemia, Myeloid, Acute, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Mutation, business
Abstract: Globally, 15-20% of all children diagnosed with leukemia suffer from acute myeloid leukemia (AML), a rapidly progressive, clinically and biologically heterogeneous disease leading to the impaired differentiation of myeloid blast cells. Although 80% of patients achieve complete remission after induction chemotherapy, many relapse, negatively affecting overall out comes. The mechanisms underlying relapse have not been fully elucidated. This review aims to provide an overview of genetic aberrations involved in relapse of disease. A literature review on molecular mechanisms implicated in pediatric AML relapse spanning from 2003 to 2017 was conducted. PubMed, Medline, and Google Scholar were interrogated using relevant search terms. Of note, we examined a total of final 10 research papers from four large study groups that have utilized whole genome sequencing and molecular targeting of trio or paired samples of initial diagnosis, remission, and relapse. Their findings reveal that the genomic landscape of pediatric AML varies from diagnosis to relapse in different populations. Pediatric AML relapse is a systemic evolutionary illness accompanied by synchronized mutational hits impairing differentiation function. The irregular proliferative function is a consequence of mutations in signal transduction genes such as FLT3, RAS, PTPN11, and c-KIT and genes that code for transcription factors such as CEBPα, WT1, SATB1, GFI1, KLF2, and TBP are associated with relapse of disease. Identification of molecular markers unique to different stages of the disease in distinct populations can provide valuable information about disease prognosis and management.
Published: 2018

22. Upshaw-Schulman Syndrome With c.2728CT Mutation in ADAMTS13 Gene

Author: Zehra Fadoo, Bushra Moiz, and Shahzadi Resham
Subjects: Hemolytic anemia, medicine.medical_specialty, Thrombotic thrombocytopenic purpura, Mutation, Missense, ADAMTS13 Protein, Blood Component Transfusion, Congenital Thrombotic Thrombocytopenic Purpura, Gastroenterology, 03 medical and health sciences, Plasma, 0302 clinical medicine, hemic and lymphatic diseases, Internal medicine, medicine, Missense mutation, Humans, Upshaw–Schulman syndrome, Child, Purpura, Thrombotic Thrombocytopenic, business.industry, Hematology, medicine.disease, ADAMTS13, Purpura, Oncology, 030220 oncology & carcinogenesis, Pediatrics, Perinatology and Child Health, Female, medicine.symptom, Differential diagnosis, business, 030215 immunology
Abstract: Congenital thrombotic thrombocytopenic purpura is a rare autosomal recessive disorder presenting with hemolytic anemia, thrombocytopenia, micro vascular thrombosis, and end organ damage. Here, we present a case of a 7-year-old girl having recurrent neonatal hemolysis, developmental delay, frequent seizures, and thrombocytopenia. Characteristic clinical picture and gene sequencing of a disintegrin and metalloproteinase with thrombospondin motifs 13 confirmed the diagnosis of Upshaw-Schulman syndrome. She was treated successfully with plasma infusion. The patient is alive at 6-month post follow-up, and on regular plasma therapy. Congenital thrombotic thrombocytopenic purpura should be considered in the differential diagnosis of thrombocytopenia with hemolytic anemia in infants.
Published: 2018

23. Spectrum of Atypical Clinical Presentations in Patients with Biallelic PRF1 Missense Mutations

Author: Magnus Nordenskjöld, Cecilia Langenskiöld, Marie Meeths, Sule Unal, Yenan T. Bryceson, Zehra Fadoo, Bianca Tesi, João Pinho Silva, Jan-Inge Henter, Samuel C. C. Chiang, Rabia Muhammad Wali, Ayad Ahmed Hussein, Ramón Lecumberri, and Dalia H. El-Ghoneimy
Subjects: Hemophagocytic lymphohistiocytosis, biology, business.industry, Perforin Deficiency, Nonsense mutation, Hematology, Disease, medicine.disease, Lymphoma, Oncology, Perforin, hemic and lymphatic diseases, Pediatrics, Perinatology and Child Health, Immunology, biology.protein, Medicine, Missense mutation, Sibling, business
Abstract: Background Perforin, encoded by PRF1, is a pore-forming protein crucial for lymphocyte cytotoxicity. Biallelic PRF1 nonsense mutations invariably result in early-onset hemophagocytic lymphohistiocytosis (HLH), termed familial HLH type 2 (FHL2). In contrast, biallelic PRF1 missense mutations may give rise to later-onset disease and more variable manifestations. Procedure We retrospectively searched our database for patients from families with siblings carrying biallelic PRF1 missense mutations where at least one sibling did not develop HLH, and for patients with biallelic PRF1 missense mutations and an atypical presentation of disease. We reviewed their clinical, genetic, and immunological characteristics. Results In all, we identified 10 such patients, including three sibling pairs with discordant manifestations. Interestingly, in two families, siblings of late-onset HLH patients developed Hodgkin lymphoma but no HLH. In a third family, one sibling presented with recurrent HLH episodes, whereas the other remains healthy. Of note, the affected sibling also suffered from systemic lupus erythematosus. Additional unrelated patients with biallelic PRF1 missense mutations were affected by neurological disease without classical signs of HLH, gastrointestinal inflammation as initial presentation of disease, as well as a hematological malignancy. Compared to early-onset FHL2 patients, the patients with an atypical presentation displayed a partial recovery of NK cell cytotoxicity upon IL-2 stimulation in vitro. Conclusions Our findings substantiate and expand the spectrum of clinical presentations of perforin deficiency, linking PRF1 missense mutations to lymphoma susceptibility and highlighting clinical variability within families. PRF1 mutations should, therefore, be considered as a cause of several diseases disparate to HLH. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.
Published: 2015
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24. Clinical features and induction outcome of childhood acute lymphoblastic leukemia in a lower/middle income population: A multi-institutional report from Pakistan

Author: Uzma Imam, Laila Saleem Lakhani, Shamvil Ashraf, Imran Nisar, Junaid Zaheer, Fatimah Sireen Yousuf, Zehra Fadoo, Ahmed Naqvi, and Asim F. Belgaumi
Subjects: education.field_of_study, Pediatrics, medicine.medical_specialty, business.industry, Population, Cancer, Induction chemotherapy, Hematology, medicine.disease, Oncology, Pediatrics, Perinatology and Child Health, Cohort, medicine, Hypodiploidy, Hyperdiploidy, business, education, Childhood Acute Lymphoblastic Leukemia, Cohort study
Abstract: Background Acute lymphoblastic leukemia (ALL) is the most common cancer of childhood. Some evidence suggests differences in clinical and cytogenetic characteristics of ALL based on geographic and ethnic variations. However, data on ALL characteristics and early outcome of therapy from low/middle-income countries such as Pakistan are scanty. Procedure A prospective, multi-institutional cohort study in Karachi enrolled 646 newly diagnosed children with ALL over 3 years. Standard forms were used to collect demographic, clinical, and laboratory data at presentation and at the end of induction. Results Of the total, 66.1% (n = 427) were males. Median age was 6 (mean ± SE 6.87 ± 0.16; range 0.16–18) years. The most common clinical presentation was fever (88.7%). BPC-ALL was diagnosed in 78.5%, while 17.5% had T-ALL; 28.8% had a WBC >50 × 109/L. With 316 patients karyotyped, hypodiploidy and hyperdiploidy were seen in 5.1% and 10.7%, respectively. Of those tested, ETV6-RUNX1 translocation was detected in 13.2%, while BCR-ABL1 translocation and MLL gene rearrangements were seen in 7.3% and 4.6%, respectively. The cumulative loss to follow up before and during induction was 12.8% (n = 83) and 11.5% (n = 74) died before or during this phase. Induction was successfully completed by only 75.6% (n = 489) of the entire cohort and 69.6% (n = 450) achieved remission. Conclusion These patients had ALL with higher risk features than that reported from developed countries. One quarter failed to complete induction chemotherapy. This suboptimal result requires further study and development of innovative interventions, particularly focusing on the causes and solutions for late referral, abandonment, and infections. Pediatr Blood Cancer 2015;62:1700–1708. © 2015 Wiley Periodicals, Inc.
Published: 2015
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25. BIOL-02. INTRACRANIAL TUMORS IN CHILDREN: A 10 YEAR REVIEW FROM A SINGLE TERTIARY HEALTH CARE CENTRE

Author: Maha Dev, Quratulain Riaz, Ehsan Naeem, Zehra Fadoo, and Naureen Mushtaq
Subjects: Cancer Research, medicine.medical_specialty, Abstracts, Oncology, Health care centre, business.industry, Family medicine, Medicine, Neurology (clinical), business, Intensive care medicine
Abstract: OBJECTIVE: Brain tumors are the second most common malignancy in children and the most common cause of cancer related deaths. Major advances in management in terms of surgery, radiation and chemotherapy have led to better outcomes. Delayed diagnosis, advanced disease at presentation, late referrals, nosocomial infections, delays to radiotherapy and poor support services are the major reasons for poorer outcomes in developing countries. Little is known about the profile of brain tumors in Pakistan. This study aims to describe the clinical profile and outcome of primary brain tumors in children at a single tertiary center in Pakistan. METHODS/MATERIALS: All children (0 – 16 years) with primary CNS tumors from 2004 till 2014 at Aga Khan University Hospital were reviewed retrospectively for clinical data, demographics, radiological findings, management and outcome. RESULTS: 175 children were included in the study. Male to female ratio was 1.4:1. Most of the patients were in 5 -10 years age group (38.9%). Most common presenting complains were headache 115 (65.7%) & vomiting 100 (57.1%). Predominant site was supratentorial 91 (52%). Three patients (1.7%) had family history of cancers. Glial tumors were 93 (53.1%) followed by embryonal 40(22.9%), Craniopharyngiomas 25 (14.3%) & Germ cell 1 (0.6%). Low grade astrocytoma (23.4%) were the most common glial tumors while Medulloblastoma (15.4%) was the most common embryonal tumor. Majority of the patients underwent surgery only (53.7%). Radiation was given to 48 (27.4%) patients. Half of the patients 79 (45.1%) were lost to follow up. Patients expired were 41 (23.4%), 24 (13.7%) are alive with residual disease while 22 patients (12.6%) were cured. CONCLUSION: This is the only study from Pakistan showing demographics of the children with brain tumors. Significant improvement needs to be made for timely diagnosis, early referrals and collaborated team efforts with multidisciplinary tumor board to improve outcome.
Published: 2017

26. Febrile neutropenia in pediatric cancer patients: Experience from a tertiary health care facility of Pakistan

Author: Muhammad Matloob Alam and Zehra Fadoo
Subjects: Chemotherapy, Pediatrics, medicine.medical_specialty, business.industry, medicine.medical_treatment, medicine.disease, Pediatric cancer, Lymphoma, Health care, medicine, Absolute neutrophil count, Retrospective analysis, business, Prospective cohort study, Febrile neutropenia
Abstract: Aims Pediatric cancer patients with febrile neutropenia (FN) have an increased risk of infectious complications and mortality. Methods This study was a retrospective analysis of pediatric cancer patients with FN. Results Out of 872 episodes of FN, 559 (64.1%) were males and 313 (35.9%) females. The mean age was 5.32 ± 4.07 years. ALL (67.7%) was the most common diagnosis followed by AML (12.2%), lymphoma (9.9%) and solid tumors (5.8%). Age p = 0.043), AML (OR = 1.8; p = 0.019), patients who received chemotherapy within 2 week of FN (OR = 1.9; p = 0.007), absolute neutrophil count p p p p p Conclusion Prospective studies in large cooperative trials may be beneficial in evaluating these risk factors further.
Published: 2014
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27. Peripherally Inserted Central Venous Catheters in Pediatric Hematology/Oncology Patients in Tertiary Care Setting

Author: Sajida Ali, Muhammad Imran Nisar, Zehra Fadoo, Naureen Mushtaq, Raza Sayani, and Raza Iftikhar
Subjects: Male, Catheterization, Central Venous, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Pediatric Hematology/Oncology, MEDLINE, Developing country, Medical Oncology, Tertiary care, Neoplasms, medicine, Central Venous Catheters, Humans, Pakistan, Child, Intensive care medicine, Developing Countries, Chemotherapy, Tertiary Healthcare, business.industry, Infant, Newborn, Infant, Hematology, Hematologic Diseases, Oncology, Catheter-Related Infections, Child, Preschool, Pediatrics, Perinatology and Child Health, Female, Pediatric hematology, business
Abstract: Peripherally inserted central venous catheters (PICC) have been successfully used to provide central access for chemotherapy and frequent transfusions. The purpose of this study was to assess the feasibility of PICCs and determine PICC-related complications in pediatric hematology/oncology patients in a resource-poor setting.All pediatric patients (age below 16 y) with hematologic and malignant disorders who underwent PICC line insertion at Aga Khan University Hospital from January 2008 to June 2010 were enrolled in the study. Demographic features, primary diagnosis, catheter days, complications, and reasons for removal of device were recorded.Total of 36 PICC lines were inserted in 32 pediatric patients. Complication rate of 5.29/1000 catheter days was recorded. Our study showed comparable complication profile such as infection rate, occlusion, breakage, and dislodgement. The median catheter life was found to be 69 days.We conclude that PICC lines are feasible in a resource-poor setting and recommend its use for chemotherapy administration and prolonged venous access.
Published: 2015
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28. Risk Factors Associated with Anthracycline Induced Cardiac Dysfunction in Pediatric Patients

Author: Zehra Fadoo, Mehnaz Atiq, Abdul Sattar Shaikh, Shazia Mohsin, Qalab Abbas, and Muhammad Matloob Alam
Subjects: medicine.medical_specialty, Chemotherapy, Combination therapy, Anthracycline, Daunorubicin, Cumulative dose, business.industry, medicine.medical_treatment, Diastole, medicine.disease, Gastroenterology, Surgery, Sepsis, Internal medicine, medicine, Doxorubicin, business, medicine.drug
Abstract: Anthracyclines (i.e., doxorubicin, daunorubicin) have significant impact on outcome in many pediatric chemotherapy protocols and therefore remain the mainstay of treatment. The objective of this study was to identify the risk factors for anthracycline induced cardiac dysfunction in pediatric patients. Multiple logistic regression model was applied to assess the risk factors for development of cardiac dysfunction. 110 pediatric oncology patients were available for final analysis. 75 (66%) children were males and mean age was 74 ± 44 months. ALL (n = 70, 64%) was the most common primary diagnosis followed by lymphoma (n = 19; 17%) and AML (n = 12, 11%). Daunorubicin alone or in combination with doxorubicin was used in (n = 94, 85%) patients and cumulative dose n = 95; 86%) children. 24 (22%) children received radiation therapy as per protocol and sepsis were observed in 47 (43%) cases. Post anthracycline, 15 (14%) children had cardiac dysfunction within a month; out of them 10/15 (67%) had isolated diastolic dysfunction, while 28 (25%) developed dysfunction within a year. 19 (17%) had pericardial effusion. 11 expired and out of them, 7 had significant cardiac dysfunction. Cumulative dose > 300 mg/m2 (p p = 0.009; AOR 3.5) and sepsis (p = 0.002; AOR 2.6) were found to be independent risk factors associated anthracycline induced cardiac dysfunction. At univariant level use of daunorubicin alone or in combination therapy (p p 0.048, OR 9.7) were also found statistically significant. In conclusion anthracycline induced cardiac dysfunction is mostly related to cumulative dose > 300 mg/m2, use of Daunorubicin alone or in combination with doxorubicin, mode of delivery, radiation therapy and sepsis. Regular long term follow-up with cardiologist is the key point for early diagnosis and therapy for a long term survival.
Published: 2014
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29. New developments in the management of congenital Factor XIII deficiency

Author: Quratulain Merchant, Karim Abdur Rehman, and Zehra Fadoo
Subjects: Pediatrics, medicine.medical_specialty, Pregnancy, Factor XIII, treatment, business.industry, diagnosis, Plasma transfusions, Hematology, Review, inherited coagulative disorders, medicine.disease, Cryoprecipitate, medicine, Missense mutation, Factor XIII deficiency, Fresh frozen plasma, business, Coagulation Disorder, medicine.drug
Abstract: Congenital Factor XIII (FXIII) deficiency is a rare, inherited, autosomal recessive coagulation disorder. Most mutations of this condition are found in the A-subunit with almost half these being missense mutations. Globally, approximately one in three million people suffer from this deficiency. Factor XIII deficiency is associated with severe life threatening bleeding, intracranial hemorrhage, impaired wound healing, and recurrent pregnancy losses. FXIII is known to have a potential role in mediating inflammatory processes, insulin resistance, bone metabolism, neoplasia, and angiogenesis. The algorithm provided for FXIII diagnosis and classification will enable prompt identification and early intervention for controlling potential life threatening complications. Prophylactic replacement therapy using blood products containing FXIII such as fresh frozen plasma, cryoprecipitate, or using FXIII concentrate remains the mainstay for the management of FXIII deficiency. In most parts of the world, cryoprecipitate and plasma transfusions are the only treatments available. Management developments have revealed the effectiveness and safety of recombinant FXIII concentrate for prophylaxis and treatment. The aim of this review is to provide an overview of advancements made in the management of FXIII deficiency from the time it was first detected, highlighting novel developments made in recent years. Greater research is warranted in identifying novel approaches to manage FXIII deficiency in light of its underlying pathophysiology.
Published: 2013

30. Burden of Cardiac Siderosis in a Thalassemia-Major Endemic Population: A Preliminary Report From Pakistan

Author: Muhammad N. Ashraf, Babar Hasan, Dudley J. Pennell, Fateh Ali Tipoo, Ahmed Imran, Sadaf Altaf, Mohammed Khursheed, Asim Qidwai, Najveen Alvi, Zehra Fadoo, Salman Naseem Adil, and Bushra Moiz
Subjects: Adult, Male, medicine.medical_specialty, Pediatrics, Iron Overload, Siderosis, Demographics, Adolescent, Endemic Diseases, Iron, Population, 030204 cardiovascular system & hematology, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Preliminary report, Medicine, Humans, Pakistan, education, Child, A-Thalassemia, education.field_of_study, business.industry, beta-Thalassemia, Hematology, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Surgery, Oncology, Echocardiography, Child, Preschool, Pediatrics, Perinatology and Child Health, Ferritins, Female, business, Cardiac magnetic resonance, Cardiomyopathies
Abstract: To describe the initial experience and demographics of T2* cardiac magnetic resonance-based myocardial-iron quantification of transfusion-dependent thalassemia-major (TM) patients from Pakistan and the correlation with serum ferritin.Eligible TM patients presenting between April 2014 and April 2015 to Aga Khan University Hospital, Pakistan, for T2*CMR were included. The severity of myocardial-iron deposition was defined as follows: normal T2*20 ms, mild-moderate T2*10 to 20 ms, and severe T2*10 ms. Cardiac symptoms were classified using the NYHA functional classification. Echocardiographic systolic and diastolic functions were performed. Continuous variables were presented as the median (minimum-maximum value). Correlation was measured using the Spearman rank correlation. Multivariate logistic regression was used to determine factors associated with the NYHA functional class.A total of 83 patients (49 male and 34 female) with TM, age 19 (5 to 45) years at presentation for T2*CMR, were reviewed. At presentation, 70% of the patients were classified as NYHA class II or worse. T2*20 ms was observed in 62.6% of the patients, with 47% showing severe iron deposition (T210 ms). No correlation of T2*20 ms (r=-0.157, P=0.302) and T2*10 ms (r=-0.128, P=0.464) was observed with serum ferritin. On multivariate analysis, lower T2* values correlated with a worsening NYHA functional class.There is a high prevalence of severe myocardial iron load in Pakistani TM patients. Serum ferritin did not correlate with T2* values. Lower T2* was the only clinical factor associated with the NYHA functional class.
Published: 2016

31. Outcome and Prognostic Factors Seen in Pediatric Oncology Patients Admitted in PICU of a Developing Country

Author: Nida Akhtar, Sukaina Panju, Zehra Fadoo, and Anwarul Haque
Subjects: Male, Pediatrics, medicine.medical_specialty, Adolescent, Critical Care, medicine.medical_treatment, MEDLINE, Developing country, Intensive Care Units, Pediatric, Neoplasms, medicine, Risk of mortality, Humans, Pakistan, Child, Intensive care medicine, Retrospective Studies, Mechanical ventilation, Pediatric intensive care unit, business.industry, Medical record, Infant, Cancer, Retrospective cohort study, Prognosis, medicine.disease, Treatment Outcome, Child, Preschool, Pediatrics, Perinatology and Child Health, Female, business
Abstract: To evaluate the outcome and prognostic factors for oncology patients in the PICU of a tertiary care centre in a developing country. A retrospective chart review was done to assess the outcome of children with cancer in the pediatric intensive care unit (PICU) of a developing country from January 2000 through December 2009. 74 medical records were reviewed for data regarding demographics, admitting diagnosis, Pediatric Risk of Mortality (PRISM) III score and the therapeutic modalities used. Of the 74 children admitted with mean age of 6.3 years (range 1–14); 53 were boys (71.6%) and 21 were girls (28.4%). Majority of the patients (37%) had hematological malignancy. The major indication for PICU admission was post-operative care (32%) followed by acute respiratory failure (24.3%), neurological complications (20.3%). The median PRISM III score was 7.0 (range 0–30). The overall mortality was 32.4% (24/74). The mean length of PICU stay was 6.3 days (ranging from 0–28 days). Seventy percent (52/74) of the children had multi organ failure (MOF). Mortality was significantly related to presence of multi-organ dysfunction syndrome and high PRISM III scores on admission and use of inotropic support with mechanical ventilation. The mortality in children with cancer in PICU in the present study is comparable to previous reports and is related to higher PRISM III score, presence of multiorgan dysfunction syndrome and use of ICU therapies.
Published: 2011
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32. Chromosomal Abnormalities in Pakistani Children with Acute Lymphoblastic Leukemia

Author: Syed Sarwer Ali, Mohammad Khurshid, Zehra Fadoo, and Muhammad Shariq Shaikh
Subjects: Male, Cancer Research, medicine.medical_specialty, Pathology, Pediatrics, Epidemiology, G banding, Karyotype, Aneuploidy, Chromosomal translocation, Biology, Translocation, Genetic, medicine, Humans, Pakistan, Child, Retrospective Studies, Chromosome Aberrations, Acute leukemia, Public Health, Environmental and Occupational Health, Cytogenetics, Retrospective cohort study, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Prognosis, medicine.disease, Triploidy, Tetraploidy, Oncology, Cytogenetic Analysis, Female, Hyperdiploidy
Abstract: Background: Cytogenetic abnormalities have important implications in diagnosis and prognosis of acute leukemia and are now considered an important part of the diagnostic workup at presentation. Karyotype, if known at the time of diagnosis, guides physicians to plan appropriate management strategies for their patients. Aim and Objectives: To determine the cytogenetic profile of acute lymphoblastic leukemia (ALL) in Pakistani children in order to have insights regarding behavior of the disease. Materials and Methods: A retrospective analysis of all the cases of ALL (
Published: 2014
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33. Metachronous renal Ewing sarcoma/primitive neuroectodermal tumour in a survivor of Burkitt lymphoma

Author: Zehra Fadoo, Khurram Minhas, Kiran Hilal, and Kumail Khandwala
Subjects: Pathology, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Neuroectodermal Tumors, Sarcoma, Ewing, Nephroureterectomy, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, 0302 clinical medicine, Rare Disease, medicine, Humans, In patient, Chemotherapy, Kidney, Primitive neuroectodermal tumour, business.industry, Neoplasms, Second Primary, General Medicine, medicine.disease, Burkitt Lymphoma, Kidney Neoplasms, Nephrectomy, Lymphoma, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Female, Sarcoma, business, Rare disease
Abstract: We present a case of a 14-year-old girl who was diagnosed with Burkitt lymphoma in 2014. She was managed with chemotherapy and remained in remission for 3 years. On her surveillance imaging in 2017, a left-sided renal neoplastic mass was incidentally discovered. She underwent nephrectomy and pathology of the resected specimen revealed small cell tumour of the kidney with features favouring renal Ewing sarcoma/primitive neuroectodermal tumour. Molecular genetic analysis by fluorescence in situ hybridisation was performed which showed translocation of 22q12, thereby confirming the diagnosis. This is a rare secondary malignancy and an unusual association. This case highlights the importance and diagnostic dilemmas of rare secondary tumours in patients with such haematological malignancies and discusses its possible pathogenetic aspects.
Published: 2018
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34. Pediatric Lymphoma: A 10-year Experience at a Tertiary Care Hospital in Pakistan

Author: Matloob Alam, Ahmed Naqvi, Asim F. Belgaumi, Iqbal Azam, and Zehra Fadoo
Subjects: Male, Pediatrics, medicine.medical_specialty, Lymphoma, Pediatric Lymphoma, education, MEDLINE, Disease, Hospitals, University, Cause of Death, Humans, Medicine, Pakistan, Child, Survival rate, Retrospective Studies, Cause of death, business.industry, Retrospective cohort study, Hematology, Tertiary care hospital, University hospital, Survival Rate, body regions, Oncology, Pediatrics, Perinatology and Child Health, Female, business
Abstract: Lymphoma is the third most common childhood malignancy. Less information is available on this disease and its outcome from our country. We present here a single institute experience. A retrospective study was carried out at Aga Khan University Hospital, Karachi on children (15 y) diagnosed with lymphoma from 1998 to 2007. Sixty-eight patients were identified. Fifty-one children were diagnosed as non-Hodgkin lymphoma (NHL). Mean age of presentation was 8.4 years with male-to-female ratio of 5.8 : 1. Most common histopathologic subtype of NHL was Burkitt lymphoma (55%). Abdominal mass was the main presenting feature of Burkitt and diffuse large B cell lymphoma. T-lymphoblastic lymphoma presented mainly as mediastinal mass. Ten children died, 4 secondary to tumor lysis syndrome, 5 because of disease progression, and 1 with chemotherapy-induced toxicity. One-third of the patients left without treatment. Seventeen children were diagnosed as Hodgkin lymphoma with mixed cellularity as the commonest subtype (65%). Overall survival of children with NHL and Hodgkin lymphoma was 62% and 94%, respectively. A greater proportion of NHL, advanced stage, and profound male preponderance were observed. Improvement in survival can only be achieved with increasing awareness, identifying and tackling causes of abandonment, early referral, and better supportive care.
Published: 2010
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35. Vaccination guidelines for children with cancer and hematopoietic stem cell transplantation living in resource-poor countries

Author: Ahmed Naqvi, Zehra Fadoo, and Saima Alvi
Subjects: medicine.medical_specialty, business.industry, medicine.medical_treatment, fungi, Cancer, Developing country, Hematology, Hematopoietic stem cell transplantation, medicine.disease, Transplantation, Vaccination, Oncology, Immunization, Pediatrics, Perinatology and Child Health, Medicine, Infection control, business, Intensive care medicine, Developed country
Abstract: There is no consensus on the immunization guidelines for immunocompromised children. Some recommendations are, however, available for children living in developed countries. The spectrum of infectious diseases is different in resource-poor countries. Vaccinations against some of these infections are not a part of the immunization schedule for children living in developed countries. We have tried to include vaccinations against diseases, which are still prevalent and a major cause of morbidity and mortality in resource-poor countries. In these guidelines, the focus has been on the vaccine-preventable diseases prevalent in Pakistan but the same can be applied to other resource-poor countries. Pediatr Blood Cancer 2010; 54:3–7. © 2009 Wiley-Liss, Inc.
Published: 2009
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36. Clinical Profile and Short-Term Outcome of Pediatric Hyperleukocytic Acute Leukemia from a Developing Country

Author: Syed Ali Shazif, Baqari, Anwarul, Haque, Muhammad Shamvil, Ashraf, Muhammad Matloob, Alam, and Zehra, Fadoo
Subjects: Male, Leukemia, Myeloid, Acute, Leukocyte Count, Leukocytosis, Child, Preschool, Acute Disease, Humans, Infant, Female, Pakistan, Nervous System Diseases, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Child
Abstract: This study was conducted to determine the frequency, clinical profile, and short-term outcome of children with hyperleukocytosis at two pediatric oncology centers in Karachi. Of a total 1,045 patients, 13.97% (n=146) patients had hyperleukocytosis. Majority (61.7%, n=90) were under 10 years of age and 76% (n=146) were male. The symptom duration before diagnosis was more than 30 days in 49.3% (n=72). The median WBC count was 181 x109/L(IQR=130.45298.3) and extreme hyperleukocytosis (200 x109/L) was observed in 44.5% (n=65) patients. Majority (94.5%, n=138) of patients were diagnosed with acute lymphoblastic leukemia. One or more complications developed in 78% (n=114) of cases. Clinical and laboratory tumor lysis syndrome (TLS) was observed in 17.1% (n=25) and 39% (n=57) patients, respectively. Pulmonary and neurological complications related to leukostasis were noted in 9.5% (n=14) and 27.3% (n=40) of cases, respectively. Infectious complications occurred in 23.2% (n=34) patients. The case-specific mortality was 20.5% (n=30). No mortality was related to early complications of hyperleukocytosis.
Published: 2016

37. Targeted high-throughput sequencing for genetic diagnostics of hemophagocytic lymphohistiocytosis

Author: Jan-Inge Henter, Yenan T. Bryceson, Merja Möttönen, H. Haluk Akar, Johanna Hästbacka, Magnus Nordenskjöld, Miguel R. Abboud, Sule Unal, Turkan Patiroglu, Hans Christian Erichsen, Omer Devecioglu, Kristina Lagerstedt-Robinson, Tor Henrik Anderson Tvedt, Magnus Sabel, Ayşegül Ünüvar, Susana Nunes, Marie Meeths, Zühre Kaya, Burcu Fatma Belen, Ekrem Unal, Bianca Tesi, Samuel C. C. Chiang, Zehra Fadoo, Ebru Tugrul Saribeyoglu, Allen Eng Juh Yeoh, Eya Ben Bdira, Clinicum, Children's Hospital, and HUS Children and Adolescents
Subjects: Male, GRISCELLI SYNDROME TYPE-2, Hemophagocytic, PROTEIN, T-CELL, CHEDIAK-HIGASHI-SYNDROME, Cohort Studies, hemic and lymphatic diseases, Sequencing, Genetics(clinical), Prospective Studies, Age of Onset, Child, Genetics (clinical), Genetics, education.field_of_study, medicine.diagnostic_test, Middle Aged, musculoskeletal system, 3. Good health, DEFICIENCY, PERFORIN, Child, Preschool, Molecular Medicine, Female, hormones, hormone substitutes, and hormone antagonists, Adult, endocrine system, Adolescent, Population, ADULT-ONSET, Lymphohistiocytosis, Hemophagocytic, MUNC13-4, Young Adult, Genetic, lymphohistiocytosis, Genetic variation, Genetic predisposition, medicine, Humans, Genetic Predisposition to Disease, Genetic Testing, 1000 Genomes Project, education, Molecular Biology, Aged, Genetic testing, Hemophagocytic lymphohistiocytosis, Base Sequence, Genetic heterogeneity, business.industry, PRIMARY IMMUNODEFICIENCIES, Research, fungi, Infant, Newborn, Infant, Sequence Analysis, DNA, medicine.disease, Human genetics, Mutation, RAB27A MUTATIONS, 3111 Biomedicine, business
Abstract: Background: Hemophagocytic lymphohistiocytosis (HLH) is a rapid-onset, potentially fatal hyperinflammatory syndrome. A prompt molecular diagnosis is crucial for appropriate clinical management. Here, we validated and prospectively evaluated a targeted high-throughput sequencing approach for HLH diagnostics., BACKGROUND:Hemophagocytic lymphohistiocytosis (HLH) is a rapid-onset, potentially fatal hyperinflammatory syndrome. A prompt molecular diagnosis is crucial for appropriate clinical management. Here, we validated and prospectively evaluated a targeted high-throughput sequencing approach for HLH diagnostics.METHODS:A high-throughput sequencing strategy of 12 genes linked to HLH was validated in 13 patients with previously identified HLH-associated mutations and prospectively evaluated in 58 HLH patients. Moreover, 2504 healthy individuals from the 1000 Genomes project were analyzed in silico for variants in the same genes.RESULTS:Analyses revealed a mutation detection sensitivity of 97.3%, an average coverage per gene of 98.0%, and adequate coverage over 98.6% of sites previously reported as mutated in these genes. In the prospective cohort, we achieved a diagnosis in 22 out of 58 patients (38%). Genetically undiagnosed HLH patients had a later age at onset and manifested higher frequencies of known secondary HLH triggers. Rare, putatively pathogenic monoallelic variants were identified in nine patients. However, such monoallelic variants were not enriched compared with healthy individuals.CONCLUSIONS:We have established a comprehensive high-throughput platform for genetic screening of patients with HLH. Almost all cases with reduced natural killer cell function received a diagnosis, but the majority of the prospective cases remain genetically unexplained, highlighting genetic heterogeneity and environmental impact within HLH. Moreover, in silico analyses of the genetic variation affecting HLH-related genes in the general population suggest caution with respect to interpreting causality between monoallelic mutations and HLH. A complete understanding of the genetic susceptibility to HLH thus requires further in-depth investigations, including genome sequencing and detailed immunological characterization.
Published: 2015
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38. Congenital (infantile) fibrosarcoma of the scalp: a case series and review of literature

Author: Nasir Ud Din, Muhammad Shahzad Shamim, Zehra Fadoo, Naureen Mushtaq, and Khurram Minhas
Subjects: Male, Pathology, medicine.medical_specialty, Tomography Scanners, X-Ray Computed, Fibrosarcoma, Neurosurgical Procedures, Metastasis, Diagnosis, Differential, Medicine, Humans, Head and neck, Retrospective Studies, Scalp, business.industry, Brain Neoplasms, Soft tissue sarcoma, Infant, Newborn, Infant, General Medicine, medicine.disease, medicine.anatomical_structure, Pediatrics, Perinatology and Child Health, Female, Neurology (clinical), Neurosurgery, Sarcoma, Neoplasm Recurrence, Local, business, Infantile Fibrosarcoma, Follow-Up Studies
Abstract: Congenital infantile fibrosarcoma (CIFS) is a soft tissue sarcoma of infants mainly involving lower extremities usually presenting during the first year of life. A subset of cases occur in the head and neck, but scalp involvement is exceptionally rare. We report clinicopathological features of three cases of CIFS involving the scalp diagnosed between 2011 and 2012. The ages of the three patients at the time of diagnosis ranged from 12 to 90 days (mean 48 days). All were males and presented with scalp swelling at birth which grew rapidly in size. The tumor was located in the left temporal region in two cases and the right temporoparietal region in one case. On imaging, underlying bone involvement was noted in two cases. The mean size of the resected tumors was 8 cm. All cases exhibited a cellular tumor arranged in sheets of uniform oval to spindle cells, increased mitosis, and hemangiopericytoma-like vessels. All patients are alive after a mean follow-up of 39.6 months. Recurrence was seen in one case due to incomplete excision. No metastasis was seen in any of the cases. CIFS of the scalp is rare and carries a good prognosis. Underlying bone erosion is rare but was noted seen in two of our cases. A male predominance was seen in our cases.
Published: 2015

39. Spectrum of Atypical Clinical Presentations in Patients with Biallelic PRF1 Missense Mutations

Author: Bianca, Tesi, Samuel C C, Chiang, Dalia, El-Ghoneimy, Ayad Ahmed, Hussein, Cecilia, Langenskiöld, Rabia, Wali, Zehra, Fadoo, João Pinho, Silva, Ramón, Lecumberri, Sule, Unal, Magnus, Nordenskjöld, Yenan T, Bryceson, Jan-Inge, Henter, and Marie, Meeths
Subjects: Adult, Male, Adolescent, Perforin, Mutation, Missense, Humans, Lupus Erythematosus, Systemic, Female, Nervous System Diseases, Hodgkin Disease, Lymphohistiocytosis, Hemophagocytic, Retrospective Studies
Abstract: Perforin, encoded by PRF1, is a pore-forming protein crucial for lymphocyte cytotoxicity. Biallelic PRF1 nonsense mutations invariably result in early-onset hemophagocytic lymphohistiocytosis (HLH), termed familial HLH type 2 (FHL2). In contrast, biallelic PRF1 missense mutations may give rise to later-onset disease and more variable manifestations.We retrospectively searched our database for patients from families with siblings carrying biallelic PRF1 missense mutations where at least one sibling did not develop HLH, and for patients with biallelic PRF1 missense mutations and an atypical presentation of disease. We reviewed their clinical, genetic, and immunological characteristics.In all, we identified 10 such patients, including three sibling pairs with discordant manifestations. Interestingly, in two families, siblings of late-onset HLH patients developed Hodgkin lymphoma but no HLH. In a third family, one sibling presented with recurrent HLH episodes, whereas the other remains healthy. Of note, the affected sibling also suffered from systemic lupus erythematosus. Additional unrelated patients with biallelic PRF1 missense mutations were affected by neurological disease without classical signs of HLH, gastrointestinal inflammation as initial presentation of disease, as well as a hematological malignancy. Compared to early-onset FHL2 patients, the patients with an atypical presentation displayed a partial recovery of NK cell cytotoxicity upon IL-2 stimulation in vitro.Our findings substantiate and expand the spectrum of clinical presentations of perforin deficiency, linking PRF1 missense mutations to lymphoma susceptibility and highlighting clinical variability within families. PRF1 mutations should, therefore, be considered as a cause of several diseases disparate to HLH.
Published: 2015

40. Clinical features and induction outcome of childhood acute lymphoblastic leukemia in a lower/middle income population: A multi-institutional report from Pakistan

Author: Zehra, Fadoo, Imran, Nisar, Fatimah, Yousuf, Laila Saleem, Lakhani, Shamvil, Ashraf, Uzma, Imam, Junaid, Zaheer, Ahmed, Naqvi, and Asim, Belgaumi
Subjects: Male, Adolescent, Remission Induction, Infant, Newborn, Infant, Induction Chemotherapy, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Cohort Studies, Treatment Outcome, Social Class, Child, Preschool, Humans, Female, Pakistan, Prospective Studies, Child
Abstract: Acute lymphoblastic leukemia (ALL) is the most common cancer of childhood. Some evidence suggests differences in clinical and cytogenetic characteristics of ALL based on geographic and ethnic variations. However, data on ALL characteristics and early outcome of therapy from low/middle-income countries such as Pakistan are scanty.A prospective, multi-institutional cohort study in Karachi enrolled 646 newly diagnosed children with ALL over 3 years. Standard forms were used to collect demographic, clinical, and laboratory data at presentation and at the end of induction.Of the total, 66.1% (n = 427) were males. Median age was 6 (mean ± SE 6.87 ± 0.16; range 0.16-18) years. The most common clinical presentation was fever (88.7%). BPC-ALL was diagnosed in 78.5%, while 17.5% had T-ALL; 28.8% had a WBC50 × 10(9) /L. With 316 patients karyotyped, hypodiploidy and hyperdiploidy were seen in 5.1% and 10.7%, respectively. Of those tested, ETV6-RUNX1 translocation was detected in 13.2%, while BCR-ABL1 translocation and MLL gene rearrangements were seen in 7.3% and 4.6%, respectively. The cumulative loss to follow up before and during induction was 12.8% (n = 83) and 11.5% (n = 74) died before or during this phase. Induction was successfully completed by only 75.6% (n = 489) of the entire cohort and 69.6% (n = 450) achieved remission.These patients had ALL with higher risk features than that reported from developed countries. One quarter failed to complete induction chemotherapy. This suboptimal result requires further study and development of innovative interventions, particularly focusing on the causes and solutions for late referral, abandonment, and infections.
Published: 2014

41. Malignant mediastinal mass in children: a single institutional experience from a developing country

Author: Naureen, Mushtaq, Muhammad Matloob, Alam, Scherherzade, Aslam, Zehra, Fadoo, and Anwar-ul-Haq
Subjects: Male, Patient Care Team, Radiography, Leukemia, Lymphoma, Mediastinal Diseases, Humans, Female, Pakistan, Child, Mediastinal Neoplasms, Retrospective Studies
Abstract: To determine the clinical spectrum and management outcomes of paediatric patients with modiastinal mass in a Karachi hospital.Medical records of all cases of mediastinal masses in children diagnosed and treated between January 2005 and December 2011 were retrospectively reviewed to evaluate the mode of presentation, histopathological diagnosis, radiologic findings and management outcomes at Aga Khan University Hospital, Karachi, Pakistan. SPSS 19 was used for data analysis.A total of 37 patients of mediastinal masses were identified, and malignancy was found in 32 (86%) cases. The median age at diagnosis was 9 years (interquartile range: 4.7 years). Lymphoma 23 (72%) and leukaemia 8 (25%) were the most common causes of mediastinal mass. Nonspecific symptoms such as fever 26 (81%), cough 15 (47%) and dyspnoea 12 (37%) constituted the most commonly presenting complaints. Overall, 22 (68.7%) patients underwent surgical procedures (complete/partial resection of mass); local lymph node biopsy was performed in 5 (15.6%) cases; and computed tomography or ultrasound-guided biopsy was done in 2 (5.4%) patients. Besides, 27 (84.4%) patients were admitted to paediatric intensive care unit for supportive care, and assisted ventilation was required in 20 (62.5%) patients. The mean length of hospital stay was 9.3 +/- 6 days. None of the patients died due to complications related to mediastinal mass or diagnostic procedure.Although mortality rate has reduced significantly with refinements in the management protocols, but a high index of suspicion and comprehensive multidisciplinary approach is crucial to improve the morbidity and mortality.
Published: 2014

42. Ovarian haemorrhage: a rare presentation and diagnostic dilemma in factor XIII deficiency

Author: Muhammad Matloob, Alam, Ahmed Raza, Iftikhar, Naureen, Mushtaq, and Zehra, Fadoo
Subjects: Diagnosis, Differential, Adolescent, Factor XIII, Humans, Female, Hemorrhage, Ovarian Diseases, Tomography, X-Ray Computed, Factor XIII Deficiency
Abstract: Factor XIII (FXIII) deficiency is a rare (autosomal recessive) genetic disorder which is associated with delayed bleeding symptoms that occur hour or days after trauma. Spontaneous rupture of visceral organs due to FXIII deficiency is a rare cause of abdominal pain with grave consequences and can be easily confused with other abdominal pathologies because of normal standard coagulation profile in these patients. We report a 15-year-old girl with spontaneous ovarian haemorrhage and splenic haematoma with FXIII deficiency. She had normal coagulation screen along with normal FXIII screen initially on the 5M urea testing which lead to delay in her diagnosis.
Published: 2014

43. Squamous Cell Carcinoma of Tongue in an 11-Year-old Girl

Author: Fozia Naz, Zehra Fadoo, Shahid Pervez, Yousuf Husen, and Nizam-ul Hasan
Subjects: medicine.medical_specialty, media_common.quotation_subject, Paan, Tongue, Carcinoma, Humans, Medicine, Basal cell, Girl, Child, Areca, media_common, biology, business.industry, digestive, oral, and skin physiology, Hematology, Prognosis, medicine.disease, Betel, biology.organism_classification, Magnetic Resonance Imaging, Dermatology, Tongue Neoplasms, stomatognathic diseases, medicine.anatomical_structure, Oncology, Pediatrics, Perinatology and Child Health, Carcinoma, Squamous Cell, Female, business, Rare disease
Abstract: We report the case of an 11-year-old girl with squamous cell carcinoma of the tongue. This is a very rare disease in children with only a few cases reported in the literature. There is a great need to create awareness of the harmful and potentially lethal effects of chewing paan and betel nut not only in adults but also in children. Pediatricians and family physicians should also be aware of the possibility of this disease entity occurring at an earlier age so as to decrease delay in diagnosis and initiation of treatment.
Published: 2010
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44. Childhood acute lymphoblastic leukaemia: experience from a single tertiary care facility of Pakistan

Author: Naureen, Mushtaq, Zehra, Fadoo, and Ahmed, Naqvi
Subjects: Male, Survival Rate, Tertiary Care Centers, Child, Preschool, Humans, Female, Pakistan, Precursor Cell Lymphoblastic Leukemia-Lymphoma, Child, Prognosis, Retrospective Studies
Abstract: To evaluate the demographic features, outcome and prognostic factors seen in children with acute lymphoplastic leukaemia at a tertiary care hospital.The retrospective descriptive study was conducted at Aga Khan University Hospital, Karachi, comprising data related to children below 15 years of age and treated between January 1997 and December 2006. Kaplan Meir survival curves were used to describe overall and event-free survival rates. Cox Proportional Hazards model was used to describe factors associated with death and relapse. SPSS 16 was the main statistical tool.Of the total 121 children diagnosed with the condition, 79 (65.3%) were males; 86 (71.1%) patients were between 1-9 years of age; Immunophenotyping was done in 99 (81.81%) patients: 86 (87%) cases had precursor B and 13 (13.13%) had precursor T. Of the total, 106 (87.6%) patients opted for treatment, while 15 (11.6%) were lost to follow-up. Besides, 26 (21.7%) patients had at least one relapse; the most common site being bone marrow in 13 (50%) followed by central nervous system in 9 (36.6%). There were 20 (16.5%) deaths in the sample. Infection was the most frequent cause of death. The event-free survival and overall survival was 63% (n = 76) and 65% (n = 79) respectively.Through the clinical characteristics of children with acute lymphoblastic leukamia were similar to those reported in literature, the outcomes were inferior. The high rate of infections and relapse warrant better supportive care and risk-based approach.
Published: 2014

45. Extraneural Soft Tissue Metastasis in a Child with Anaplastic Ependymoma

Author: Zubair Ahmad, Mohammad Faizan Zahid, Ahmed Nadeem Abbasi, Muhammad Ehsan Bari, and Zehra Fadoo
Subjects: Pathology, medicine.medical_specialty, business.industry, medicine.disease, Extraneural, Fourth ventricle, Hydrocephalus, Metastasis, medicine, Vomiting, Histopathology, Headaches, medicine.symptom, Papilledema, business
Abstract: Extraneural Soft Tissue Metastasis in a Child with Anaplastic Ependymoma We present the case of a 5 year old boy who presented to us with complaints of worsening headaches, vomiting, drowsiness and weight loss. He also had difficulty maintaining balance and fell down frequently. Examination revealed elevated blood pressure, sluggish pupils and bilateral grade 1 papilledema. Imaging of the brain showed a mass in the fourth ventricle causing non-communicating hydrocephalus. The mass was excised and histopathology showed features of anaplastic ependymoma.
Published: 2014
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46. Congenital Factor VII Deficiency in Children at Tertiary Health Care Facility in Pakistan

Author: Bushra Moiz, Priyanka Jethwani, Zehra Fadoo, Muhammad Matloob Alam, and Karim Abdur Rehman
Subjects: Male, Pediatrics, medicine.medical_specialty, Adolescent, Factor VII Deficiency, Consanguinity, chemistry.chemical_compound, Sex Factors, Interquartile range, Health care, Medicine, Humans, Risk factor, Factor VII deficiency, Child, Retrospective Studies, Factor VII, business.industry, Tertiary Healthcare, Infant, Newborn, Infant, Hematology, General Medicine, Odds ratio, chemistry, Child, Preschool, Female, business, Gastrointestinal Hemorrhage, Intracranial Hemorrhages, Intracranial bleeding
Abstract: This study presents the demographics, clinical spectrum, and outcome of patients with congenital factor VII (FVII) deficiency at a tertiary care center over a period of 12 years. Of the 49 patients, 27 (55%) patients were males. Consanguinity was found in 92% of the patients. The median age of symptom onset was 2.4 (interquartile range [IQR]: 1.1-6.5) years with a median age of 5.8 (IQR: 3.1-10) years at diagnosis. Life-threatening complications like intracranial bleeding (ICB) and intra-abdominal bleeding (IAB) were observed in 8 (16.4%) patients. We found that 11 (55%) of the 20 patients with FVII coagulant activity (FVIIc) 5% were affected by severe symptoms. Age
Published: 2013

47. New developments in the management of congenital Factor XIII deficiency [Corrigendum]

Author: Karim Abdur Rehman, Zehra Fadoo, and Merchant Q
Subjects: Journal of Blood Medicine, Pediatrics, medicine.medical_specialty, lcsh:RC633-647.5, business.industry, Medicine, Factor XIII deficiency, lcsh:Diseases of the blood and blood-forming organs, Hematology, Corrigendum, business, medicine.disease
Abstract: Fadoo Z, Merchant Q, Rehman KA. Journal of Blood Medicine. 2013;4:65–73.On page 67 the legend of Figure 1 incorrectly states "Reprinted from Song JW, Choi JR, Song KS, Rhee JH. Plasma factor XIII activity in patients with disseminated intravascular coagulation. Yonsei Med J. 2006;47(2):196–200." The correct statement is "Reprinted from Muszbek L, Bagoly Z, Cairo A, Peyvandi F. Novel aspects of factor XIII deficiency. Curr Opin Hematol. 2011;18(5):366–372."On page 69 the legend of Table 1 states "Reprinted from Song JW, Choi JR, Song KS, Rhee JH. Plasma factor XIII activity in patients with disseminated intravascular coagulation. Yonsei Med J. 2006;47(2):196–200." The correct statement is "Reprinted from Kohler HP, Ichinose A, Seitz R, Ariens RA, Muszbek L; Factor XIII and fibrinogen SSC subcommittee of the ISTH. Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost. 2011;9(7):1404–1406."Read the original article
Published: 2013

48. Acute myeloid leukaemia in children: experience at a tertiary care facility of Pakistan

Author: Zehra, Fadoo, Naureen, Mushtaq, Saima, Alvi, and Muhammad, Ali
Subjects: Hospitals, University, Male, Leukemia, Myeloid, Acute, Treatment Outcome, Adolescent, Child, Preschool, Age Factors, Humans, Infant, Female, Pakistan, Child, Retrospective Studies
Abstract: To document the demographics and outcome of children with Acute Myeloid Leukemia (AML) treated at a tertiary care facility of Pakistan.A retrospective study was conducted at Aga Khan University on children (less than 15 years) diagnosed to have AML between January 2000 to May 2007.Total 40 cases were diagnosed out of which 37 charts were available for review.The average age of presentation was 8.5 +/- 4.5 years and 75% were males. The most common presenting feature was fever in 83% followed by bleeding in 41% and pallor in 39%. Initial WBC of100,000 was seen in 19% of patients. The most common FAB subtype was M4 39%. Twenty three patients underwent treatment out of which 12 patients are alive and in remission. Majority were followed up around 2 years and 6 months. Out of the 11 patients who died three had resistant disease, four relapsed and rest died due to sepsis mostly during induction.The most common sub type in our study is AML M4 although AML M2 is reported as predominant subtype. About a third of the patients could not start or complete therapy due to financial constraints. The overall survival for our patients who completed therapy was 52%.
Published: 2012

49. Acute lymphoblastic leukemia in a child with Fanconi's anaemia

Author: Naureen, Mushtaq, Rabia, Wali, Zehra, Fadoo, and Ali Faisal, Saleem
Subjects: Fanconi Anemia, Fatal Outcome, Rare Diseases, Adolescent, Bone Marrow, Precursor B-Cell Lymphoblastic Leukemia-Lymphoma, Humans, Chromosome Breakage, Female, Flow Cytometry
Abstract: Fanconi anaemia (FA) is an autosomal recessive inherited disorder with progressive bone marrow failure, associated congenital malformation and solid and haematological malignancies. Acute myeloid leukemia is the commonest haematological malignancy followed by myelodysplastic syndrome in children with FA. FA transformed into acute lymphoblastic leukemia (ALL) is a rare phenomenon and one of the rarest haematological malignancies associated with this disorder. We are reporting a 13 years old girl with FA and positive chromosomal breakage. She required regular blood product transfusion. She was planned for haematopoietic stem cell transplantation (HSCT) but the sibling-matched donor was found to have chromosomal breaks as well. Later on, her peripheral smear showed blast cell. Bone marrow showed pre-B ALL. She was started on chemotherapy but died shortly due to complications of the treatment. For this rare condition conservative management is indeed essential, however, safe and appropriate chemotherapy regimen is needed.
Published: 2011

50. Successful engraftment and survival following allogeneic hematopoietic stem cell transplant in a child with familial hemophagocytic lymphohistiocytosis

Author: Salman Naseem Adil, Natasha Ali, Zehra Fadoo, and Nehal Masood
Subjects: Male, Cure rate, business.industry, Prolonged fever, Hepatosplenomegaly, Hematopoietic Stem Cell Transplantation, Disease, Familial Hemophagocytic Lymphohistiocytosis, Human leukocyte antigen, Lymphohistiocytosis, Hemophagocytic, Survival Rate, Treatment Outcome, Bone Marrow, Child, Preschool, Pediatrics, Perinatology and Child Health, Immunology, medicine, Humans, Transplantation, Homologous, Allogeneic hematopoietic stem cell transplant, medicine.symptom, Stem cell, business, Child
Abstract: Hemophagocytic syndrome is a rare disorder mainly affecting children. Symptoms include prolonged fever, hepatosplenomegaly and cytopenias. Allogeneic stem cell transplant appears to provide the best overall cure rate in this disease. The authors report a young boy, the second child of consanguineous parents, diagnosed with familial hemophagocytic lymphohistiocytosis (HLH) who underwent allogeneic stem cell transplant form HLA matched father.
Published: 2011

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