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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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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. 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

13. 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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14. 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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15. 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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16. 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

17. 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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18. 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

19. 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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20. 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

21. 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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22. 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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23. 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

24. 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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25. 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

26. 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

27. 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

28. 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

29. 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

30. 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

31. 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

32. Successful management of invasive aspergillosis with voriconazole and amphoteracin B therapy in a patient with acute mycloid leukemia (AML-M2)

Author

Nauman Fazal, Manzoor, Sidra, Azim, and Zehra, Fadoo

Subjects
Male, Antifungal Agents, Neutropenia, Fever, Antineoplastic Agents, Triazoles, Leukemia, Myeloid, Acute, Pyrimidines, Treatment Outcome, Amphotericin B, Aspergillosis, Humans, Voriconazole, Child
Abstract

An eleven-year-old boy presented with one month's history of fever and weight loss. He was diagnosed with Acute Mycloid Leukemia (AML-M2). During treatment he developed recurrent infections with neutropenia requiring prolonged antibiotics and subsequently developed invasive aspergillosis. He was treated with amphotericin B and Voriconazole. This case shows the efficacy and safety of combined antifungal therapy, including voriconazole, for invasive aspergillosis complicating AML.

Published
2011

33. Healthcare-associated infections among pediatric oncology patients in Pakistan: risk factors and outcome

Author

Anwar Ul Haque, Zehra Fadoo, Naveed ur Rehman Siddiqui, and Rabia Wali

Subjects
Healthcare associated infections, Male, Pediatrics, medicine.medical_specialty, Adolescent, Cross-sectional study, Urinary system, Microbiology, Risk Factors, Virology, Neoplasms, Pediatric oncology, Prevalence, Medicine, Humans, Pakistan, Child, Cross Infection, Bacteria, business.industry, Mortality rate, Fungi, Infant, General Medicine, Bacterial Infections, University hospital, Infectious Diseases, Parenteral nutrition, Increased risk, Cross-Sectional Studies, Treatment Outcome, Mycoses, Child, Preschool, Parasitology, Female, business
Abstract

Introduction: Pediatric oncology patients are at increased risk of contracting healthcare-associated infections (HAIs), which are responsible for increased morbidity and mortality rates as well as treatment costs. This study aimed to identify the frequency of HAIs among pediatric oncology patients and their outcome. Methodology: Pediatric oncology patients admitted between January 2009 and June 2010 in a pediatric ward at Aga Khan University Hospital, Karachi, Pakistan, who developed HAIs, were analyzed. Results: A total of 90 HAIs were identified in 32 patients in 70 admissions. The HAI rate among pediatric oncology patients was 3.1/100 admission episodes. Bloodstream infections (63 episodes, 90.0%) were the most common, followed by urinary tract infection (two episodes, 2.9%). Gram-positive infections were seen in 54 (60%) patients, followed by Gram-negative infection in 34 (37.8%), and fungi in 2 (2.8%) cases. Coagulase negative staphylococci was the most common Gram-positive and Escherichia coli and Pseudomonas aeruginosa were most common Gram-negative infections. Mortality rate among pediatric oncology patients who developed HAIs was 12.5% (4/32). Total parental nutrition use and length of stay longer than 30 days were the identified risk factors associated with increased mortality among pediatric oncology patients who developed HAIs. Conclusion: We report an HAI rate among pediatric oncology patients of 3.1/100 admission episodes with a mortality rate of 12.5% in Pakistan. Further studies should be done, especially in the developing world, to identify the risk factors associated with increased mortality among pediatric oncology patients so that adequate measures can be taken to reduce the mortality among these patients.

Published
2010

34. Aplastic anemia: clinicohaematological features, treatment and outcome analysis

Author

Rabia, Wali, Zehra, Fadoo, Salman, Adil, and Muhammad Ahmed, Naqvi

Subjects
Male, Adolescent, Child, Preschool, Hematopoietic Stem Cell Transplantation, Anemia, Aplastic, Humans, Infant, Female, Child, Retrospective Studies
Abstract

To determine the clinicohaematological features, treatment and outcome of children diagnosed with aplastic anemia at a single institution.Observational study.The Aga Khan University Hospital, Karachi, from January 1999 till December 2008.Medical records of children aged less than 15 years of age diagnosed with aplastic anemia were reviewed. Clinicohaematological features, treatment and its response to therapy and outcome were recorded. Results were described in percentages.Ninety patients were diagnosed to have aplastic anemia (AA); 65 were male during the study period. Age ranged from 1 to 15 years. Fever in 65 patients (72.2%), pallor in 53 (58.8%), skin bleeding in 49 (54.4%) and epistaxis in 31(34.4%) were the most common and frequent presenting features. Congenital (Fanconi's) anemia was found in 15 (16.6%) and acquired idiopathic in 75 (83.4%) of patients. Very severe aplastic anemia (VSAA) was seen in 29 (32.2%), 26 (28.9%) had severe AA and 17 (18.9%) had moderate AA. Eight patients (8.9%) underwent haematopoietic stem cell transplantation (HSCT), 12 (13.3%) received immunosuppressive therapy (IST) and 70 patients (77.7%) received other and supportive therapy. Five (62.5%) patients showed complete response to HSCT and 3 (37.5%) failed to engraft. IST showed complete response in 3 (25%), partial response in 5 (41.6%) and no response in 4 (33.3%). Twenty two patients (24.4%) expired either due to infection in 16 (72.7%, fungal in 6, bacterial in 10) and intracranial haemorrhage in 6 (27.3%) cases.Majority of cases with AA were acquired and idiopathic in etiology. VSAA and SAA were frequent. Response to HSCT and IST was sub-optimal.

Published
2010

35. Imiglucerase treatment in Gaucher's disease

Author

Uzma, Shah, Naila, Nadeem, Yousef, Husen, and Zehra, Fadoo

Subjects
Lysosomal Storage Diseases, Male, Gaucher Disease, Treatment Outcome, Disease Progression, Glucosylceramidase, Humans, Infant, Female, Prognosis, Recombinant Proteins
Abstract

Gaucher's disease is an inherited lysosomal storage disorder with a deficiency of the enzyme glucocerbrosidase that manifests with clinical features of anemia, hepato-splenomegaly, skeletal destruction and organ dysfunction due to the accumulation of glucocerbrosides. There are several types of Gaucher's disease with varying prognosis and clinical progression of disease. We describe two cases followed at the Aga Khan University, Karachi, Pakistan, with different forms of the disorder. The enzyme Imiglucerase (Cerezyme, Genzyme) has been used to treat Type 1 Gaucher disease while the neuronopathic type has been resistant to therapy. We used Imiglucerase 60 microg/kg every 2 weeks in one patient with Type 1 Gaucher disease and followed hepatic, splenic volumes and blood counts. Treatment with Imiglucerase resulted in a decrease in splenic size, reduced requirements for transfusions and an improvement in cardiopulmonary symptoms.

Published
2008

36. Clinical characteristics of childhood cancer in emergency room in a tertiary hospital in Pakistan

Author

Zehra Fadoo, Amna Jawaid, Nick Brown, and Khubaib Arif

Subjects
Pediatrics, medicine.medical_specialty, business.industry, Incidence (epidemiology), Cancer, 030208 emergency & critical care medicine, Emergency department, 030204 cardiovascular system & hematology, Airway obstruction, medicine.disease, Tumor lysis syndrome, 03 medical and health sciences, 0302 clinical medicine, Spinal cord compression, Emergency Medicine, medicine, Etiology, Coagulopathy, business, Letter to the Editor
Abstract

Worldwide, cancer is an important cause of mortality in children aged over 1 year.[1] Numerically, the major cancers include acute lymphoblastic leukemia, CNS tumors and lymphomas.[2–6] Cancer incidence is increasing in children globally as well as in Pakistan but the etiology is poorly understood.[7] There are an estimated 160 000 new cases and 90 000 deaths per year worldwide in children aged under 15 years.[8] The exact incidence in Pakistan is not known as there is no national tumor registry. Early diagnosis in childhood is vital in terms of outcome.[9–14] However, presentations are non-specific which may lead to a delay in both diagnosis and treatment and a poorer outcome.[15–17] Knowledge of common presentation and initial management of oncological emergencies is, therefore, essential for the emergency department (ED) care provider. Common oncological emergencies with which the emergency physician might encounter include tumor lysis syndrome, coagulopathy, hyperleukocytosis, mediastinal mass with airway obstruction and spinal cord compression all of which have substantial morbidity and mortality if left untreated. There is no previous data available on frequency and clinical presentations of childhood cancer in the emergency room from Pakistan, so our goal was to identify the presentation of childhood malignancy in ED.

Published
2016
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37. Factor XIII deficiency in children--clinical presentation and outcome

Author

Zehra, Fadoo and Ali Faisal, Saleem

Subjects
Male, Treatment Outcome, Factor XII Deficiency, Risk Factors, Child, Preschool, Humans, Infant, Female, Blood Coagulation Tests, Child, Retrospective Studies
Abstract

To determine the demographic features and clinical outcome of children with Factor XIII deficiency.Observational case series.The Aga Khan University Hospital, Karachi, from January 1996 to December 2006.Records of all hospitalized pediatric patients with discharge diagnosis of FXIII D, on the basis of factor XIII assay 5 mol/L urea test were retrospectively reviewed and abstracted on a pre-specified proforma. Demographic features, coagulation profile, family history and outcomes were noted.A total of 10 charts were reviewed. There were 5 boys and 5 girls. Almost all the children (9/10) were less than 5 years of age, out of whom 5 (50%) were infants, and 3 were neonates. Bruises and prolonged bleeding after trauma was the major presenting complaints in 80%, followed by prolonged bleeding from the umbilical stump in 2 patients. Nine patients had past history of prolonged umbilical bleeding. Two patients had history of FXIII D in siblings, while 2 had history of prolonged bleeding in other family members (cause unknown). Consanguinity was present in 80% of the families. Initial coagulation screen were normal in all patients. Two patients had intracranial hemorrhage, proved on neuro-imaging, were managed with plasma infusions and required craniotomy. The rest were managed conservatively with plasma transfusions. All were discharged alive in good clinical condition. Almost all were followed regularly in clinic with monthly cryoprecipitate transfusions.Although factor XIII deficiency is a rare genetic disorder in children with history of bruising, prolonged umbilical bleeding, family history of bleeding and consanguinity with normal initial coagulation screen (PT, APTT and platelets), FXIII D should be ruled out.

Published
2007

39. Effect of L-type calcium channel blocker (amlodipine) on myocardial iron deposition in patients with thalassaemia with moderate-to-severe myocardial iron deposition: protocol for a randomised, controlled trial

Author

Alina Sadaf, Zehra Fadoo, Farheen Quadri, Amarah Shakoor, Mohammad Khurshid, Maaman Zahoor, Babar Hasan, Zaffar Sajjad, Steven D. Colan, Arjumand Rizvi, Najveen Alvi, and Fateh Ali Tipoo

Subjects
Male, Time Factors, Thalassemia, Cardiomyopathy, Calcium channel blocker, Severity of Illness Index, law.invention, Electrocardiography, Randomized controlled trial, law, Protocol, Prospective Studies, Child, Prospective cohort study, Chelating Agents, Heart, General Medicine, Calcium Channel Blockers, Treatment Outcome, Drug Therapy, Combination, Female, Cardiomyopathies, medicine.drug, medicine.medical_specialty, Iron Overload, Adolescent, Calcium Channels, L-Type, medicine.drug_class, Iron, Magnetic Resonance Imaging, Cine, Young Adult, Pharmacotherapy, Internal medicine, Severity of illness, medicine, Humans, Amlodipine, Dose-Response Relationship, Drug, business.industry, Myocardium, Paediatrics, Stroke Volume, medicine.disease, Surgery, business, Follow-Up Studies
Abstract

Introduction Sideroblastic cardiomyopathy secondary to repeated blood transfusions is a feared complication in thalassaemia. Control of myocardial iron is thus becoming the cornerstone of thalassaemia management. Recent evidence suggests a role for L-type Ca2+ channels in mediating iron uptake by the heart. Blocking the cellular iron uptake through these channels may add to the benefit of therapy to standard chelation in reducing myocardial iron. We aim to determine the efficacy of amlodipine (a calcium channel blocker) as an adjunct to standard aggressive chelation in retarding myocardial iron deposition in thalassaemics with or without cardiomyopathy. Outcomes The primary outcome is to compare the efficacy of amlodipine+chelation (intervention) versus standard chelation (control) in retarding myocardial iron deposition. Secondary outcomes include the effect of amlodipine therapy on systolic and diastolic function, strain and strain rate and liver iron content. Methods and analysis This is a single-centre, parallel-group, prospective randomised control trial. Twenty patients will be randomised in a 1:1 allocation ratio into the intervention and control arms. In addition to conventional echocardiography, MRI T2* values for assessment of cardiac and liver iron load will be obtained at baseline and at 6 and 12 months. Cardiac T2* will be reported as the geometric mean and per cent coefficient of variation, and an increase in cardiac T2* values from baseline will be used as an end point to compare the efficacy of therapy. A p Value of

Published
2014
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