Editorial Material
Multidisciplinary Sciences
Anna Nowogrodzki
Summary: The United States has made progress in reducing child mortality, but adults have been neglected in this regard.
Article
Hematology
Peter Olupot-Olupot, George Tomlinson, Thomas N. Williams, Leon Tshilolo, Brigida Santos, Luke R. Smart, Kathryn McElhinney, Thad A. Howard, Banu Aygun, Susan E. Stuber, Adam Lane, Teresa S. Latham, Russell E. Ware
Summary: The REACH study provides hydroxyurea at maximum tolerated dose for children with sickle cell anemia in sub-Saharan Africa, and it not only reduces SCA-related clinical events but also decreases malaria incidence by around 50%. The study analyzed the associations between hydroxyurea and lower malaria rates, through recording infections in clinical sites across Angola, Democratic Republic of Congo, Kenya, and Uganda. The results showed that ANC and splenomegaly were significant factors associated with malaria risk, and hydroxyurea treatment at MTD led to lower malaria incidence through incompletely defined mechanisms.
Article
Pediatrics
Adetokunbo Olayinka Joacquim, Adeseye Micheal Akinsete, Christopher Imokhuede Esezobor
Summary: This study aimed to investigate the prevalence, severity, hospital outcome, and factors associated with acute kidney injury (AKI) in hospitalized children with sickle cell anemia (SCA). The results showed that AKI occurred in 27 out of 155 children with SCA (17.4%), with 33.3% reaching stage 3. Hepatomegaly, splenomegaly, dipstick proteinuria, and hematuria were more common in AKI patients. AKI patients had lower hematocrit and serum bicarbonate compared to those without AKI. AKI was also associated with a longer hospital stay.
Article
Hematology
Peter Olupot-Olupot, Roisin Connon, Sarah Kiguli, Robert O. Opoka, Florence Alaroker, Sophie Uyoga, Margret Nakuya, William Okiror, Julius Nteziyaremye, Tonny Ssenyondo, Eva Nabawanuka, Juliana Kayaga, Cynthia Williams Mukisa, Denis Amorut, Rita Muhindo, Gary Frost, Kevin Walsh, Alexander W. Macharia, Diana M. Gibb, A. Sarah Walker, Elizabeth C. George, Kathryn Maitland, Thomas N. Williams
Summary: Sickle cell anemia is common in sub-Saharan Africa, and a trial in Uganda and Malawi found that 12% of children had known sickle cell anemia and 17% had unknown sickle cell anemia. Children with sickle cell anemia showed better outcomes in various indicators.
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
Article
Pediatrics
Asmaa M. Zahran, Khaled Saad, Khalid Elsayh, Shaimaa M. Khalaf, Khaled Hashim Mahmoud, Amira Elhoufey, Helal F. Hetta
Summary: Among children with SCD, HU treatment exhibited significant qualitative and quantitative effects on Tregs. HU treatment in SCD decreases the frequency of Tregs, as well as the levels of the most suppressive Tregs: HLA-DR+, CD39+, and CD69+. At the same time, HU increases the proportion of naive CD45RA+ Tregs. Our study showed the impact of HU therapy on Tregs in children with SCD.
PEDIATRIC RESEARCH
(2022)
Article
Clinical Neurology
Djamila L. Ghafuri, Brittany Covert Greene, Bilya Musa, Awwal Gambo, Abdulrasheed Sani, Shehu Abdullahi, Binta J. Wudil, Halima Bello-Manga, Safiya Gambo, Matin Ghafuri, Holly Cassell, Kathleen Neville, Fenella Kirkham, Adetola A. Kassim, Muktar H. Aliyu, Michael R. DeBaun, Lori C. Jordan
Summary: In Nigeria, capacity-building strategies were implemented in two primary stroke prevention trials for children with SCA. Training programs, equipment donation, and collaboration with local government officials were vital in establishing sustainable pediatric SCA primary stroke prevention programs in Africa.
PEDIATRIC NEUROLOGY
(2021)
Article
Genetics & Heredity
Vivian Paintsil, Evans Xorse Amuzu, Isaac Nyanor, Emmanuel Asafo-Adjei, Abdul Razak Mohammed, Suraj Abubakar Yawnumah, Yaa Gyamfua Oppong-Mensah, Samuel Blay Nguah, Paul Obeng, Elliot Eli Dogbe, Mario Jonas, Victoria Nembaware, Gaston Mazandu, Kwaku Ohene-Frempong, Ambroise Wonkam, Julie Makani, Daniel Ansong, Alex Osei-Akoto
Summary: This article describes the experiences of establishing a registry for sickle cell disease (SCD) patients in Kumasi, Ghana. Starting from December 2017, over 3,000 SCD patients were enrolled into the registry, which involved comprehensive review of medical records and obtaining ethical clearance. The registry will provide valuable longitudinal data for SCD research in Ghana and Africa.
FRONTIERS IN GENETICS
(2022)
Article
Pediatrics
Marianne E. Yee, Kristina W. Lai, Nitya Bakshi, Joanna K. Grossman, Preeti Jaggi, Alexander Mallis, Yun F. Wang, Robert C. Jerris, Peter A. Lane, Inci Yildirim
Summary: This retrospective cohort study examined children with SCD who had blood cultures collected from 2010 to 2019, and found that BSI remains a risk for these children with an incidence rate of 0.89 per 100 person-years. Sickle cell anemia genotypes and chronic transfusions were associated with higher odds of BSI, while hydroxyurea was associated with lower odds of BSI.
Article
Genetics & Heredity
Beatrice E. Gee, Andrea Pearson, Iris Buchanan-Perry, Roger P. Simon, David R. Archer, Robert Meller
Summary: Whole transcriptome RNA-sequencing revealed differential expression of 223 genes and differential transcript expression of 441 genes in whole blood samples from sickle cell anemia (SCA) patients compared to controls. The differentially expressed RNA were enriched for genes associated with hemoglobin and ubiquitin-proteasome pathway. Further analysis showed higher expression of gamma globin genes in SCA patients, and identified non-coding RNAs associated with HBG1 and HBG2 mRNA levels. These findings suggest novel regulatory mechanisms for fetal hemoglobin regulation in SCA.
FRONTIERS IN GENETICS
(2022)
Article
Hematology
Abdoul K. Dembele, Claudine Lapoumeroulie, Mor Diaw, Oumarou Tessougue, Lucile Offredo, Dapa A. Diallo, Saliou Diop, Jacques Elion, Yves Colin-Aronovicz, Pierre-Louis Tharaux, Xavier Jouven, Marc Romana, Brigitte Ranque, Caroline Le Van Kim
Summary: This study analyzed blood cell-derived microparticles in stable SCD patients living in sub-Saharan Africa, revealing associations between microparticles and complications such as retinopathy, priapism, and leg ulcers. Furthermore, the study suggests a weak relevance of the hyper-hemolysis versus hyper-viscous paradigm in Africa.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Immunology
Hannah K. Peng, Kevin J. Dombkowski, Gary L. Freed, Susan E. Creary, Dominic Smith, Sarah L. Reeves
Summary: Children with sickle cell disease (SCD) had higher annual flu immunization rates than those without SCD, but over 50% remained unimmunized. Among children with SCD, there were no significant differences in immunization rates by age; however, adolescents aged 13-17 without SCD were less likely to receive annual flu immunization compared to children aged 6-35 months.
Article
Hematology
Monica L. Hulbert, Melanie E. Fields, Kristin P. Guilliams, Priyesha Bijlani, Shalini Shenoy, Slim Fellah, Alison S. Towerman, Michael M. Binkley, Robert C. McKinstry, Joshua S. Shimony, Yasheng Chen, Cihat Eldeniz, Dustin K. Ragan, Katie Vo, Hongyu An, Jin-Moo Lee, Andria L. Ford
Summary: This study investigated the effect of hematopoietic stem cell transplant (HSCT) on cerebral hemodynamics in children with sickle cell disease (SCD). It found that HSCT normalized cerebral blood flow and oxygen extraction fraction in SCD children, reducing the risk of strokes.
Article
Public, Environmental & Occupational Health
Hannah K. Peng, Kevin J. Dombkowski, Gary L. Freed, Susan E. Creary, Dominic Smith, Sarah L. Reeves
Summary: Children with sickle cell disease had higher completion rates of primary immunization series compared to those without the disease at both 19 months and 35 months. However, more than 40% of children with sickle cell disease did not receive all recommended immunizations by age 19 months. Utilizing immunization information systems is recommended to improve routine immunization coverage for these children.
AMERICAN JOURNAL OF PREVENTIVE MEDICINE
(2021)
Article
Hematology
Chibuzo J. J. Aguwa, Alicia D. D. Cannon, James F. F. Casella, Bruce K. K. Shapiro, Eboni I. I. Lance
Summary: Despite the availability of developmental screening guidelines, children with sickle cell disease (SCD) are not receiving regular and appropriate screening for neurodevelopmental disorders (NDDs). Many children are screened outside the recommended ages, and there is a lack of autism-specific screening.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Review
Pharmacology & Pharmacy
Daniel Dexter, Patrick T. McGann
Summary: Sickle cell disease is a common and life-threatening inherited blood disorder that mainly affects sub-Saharan Africa. Although there have been significant advances in the care of individuals with SCD, these interventions are only available for a small percentage of the global SCD population. In Africa, SCD still results in early mortality for a large number of infants. Efforts are being made to prioritize SCD in many African countries, but the widespread use of hydroxyurea, a primary treatment for SCD, still faces barriers.
Article
Immunology
Julie A. Bettinger, Michael A. Irvine, Hennady P. Shulha, Louis Valiquette, Matthew P. Muller, Otto G. Vanderkooi, James D. Kellner, Karina A. Top, Manish Sadarangani, Allison McGeer, Jennifer E. Isenor, Kimberly Marty, Phyumar Soe, Gaston De Serres
Summary: This study examined the short-term safety of COVID-19 vaccines in adults with a previous history of SARS-CoV-2 infection. It found that adults with moderate or severe previous infection were more likely to experience health events after each vaccine dose, while the risk associated with previous infection was attenuated after subsequent doses.
CLINICAL INFECTIOUS DISEASES
(2023)
Article
Infectious Diseases
Joan Robinson, Tammie Dewan, Shaun K. Morris, Ari Bitnun, Peter Gill, Tala El Tal, Ronald M. Laxer, E. Ann Yeh, Carmen Yea, Rolando Ulloa-Gutierrez, Helena Brenes-Chacon, Adriana Yock-Corrales, Gabriela Ivankovich-Escoto, Alejandra Soriano-Fallas, Marcela Hernandez-de Mezerville, Jesse Papenburg, Marie-Astrid Lefebvre, Alireza Nateghian, Behzad Haghighi Aski, Ali Manafi, Rachel Dwilow, Jared Bullard, Suzette Cooke, Lea Restivo, Alison Lopez, Manish Sadarangani, Ashley Roberts, Nicole Le Saux, Jennifer Bowes, Rupeena Purewal, Janell Lautermilch, Jacqueline K. Wong, Dominique Piche, Karina A. Top, Cheryl Foo, Luc Panetta, Joanna Merckx, Michelle Barton
Summary: This study describes the clinical course and outcomes in children with technology dependence (TD) hospitalized with SARS-CoV-2 infection. The results show that children with TD have an increased risk of COVID-19 hospitalization, but all patients were discharged home in the absence of end-stage chronic conditions.
Article
Public, Environmental & Occupational Health
Hilda J. Tutuba, Agnes Jonathan, William Lloyd, Upendo Masamu, Emanuela Marco, Julie Makani, Paschal Ruggajo, Benson R. Kidenya, Irene K. Minja, Emmanuel Balandya
Summary: The study assesses the efficacy of maternal health education and maternal screening for Sickle Cell Disease (SCD) on knowledge and uptake of infant screening for SCD among pregnant women attending antenatal clinics in Dar-es-salaam, Tanzania. The results demonstrate that the interventions of maternal health education and maternal screening for SCD are effective in raising knowledge and improving the uptake of infant diagnosis for SCD.
Article
Hematology
Mboka Jacob, Jamie M. Kawadler, Russell Murdoch, Magda Ahmed, Hilda Tutuba, Upendo Masamu, Karin Shmueli, Dawn E. Saunders, Chris A. Clark, Jinna Kim, Shifa Hamdule, Julie Makani, Hanne Stotesbury, Fenella J. Kirkham
Summary: Brain injury is a common complication of sickle cell anaemia (SCA), leading to reduced volume of white matter (WM) and grey matter (GM) structures in patients. This study examines the impact of silent cerebral infarction (SCI), vasculopathy, and anaemia on WM and regional GM volumes in African children. Results show that SCA patients have smaller volumes of WM, cortical and subcortical GM, as well as specific brain regions, compared to controls. SCI and vasculopathy further decrease subcortical GM volume, while SCA patients without SCI or vasculopathy exhibit larger putamen and hippocampus volumes. Haemoglobin levels only significantly affect GM volumes in controls. Overall, SCA patients generally have reduced GM volumes, although some subcortical regions may be spared. SCI and vasculopathy can impact changes in subcortical GM and WM volume, while anaemia may affect brain volume in non-SCA children.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Multidisciplinary Sciences
Emily R. Begnel, Bhavna H. Chohan, Ednah Ojee, Judith Adhiambo, Prestone Owiti, Vincent Ogweno, LaRinda A. Holland, Carolyn S. Fish, Barbra A. Richardson, Adam K. Khan, Rabia Maqsood, Efrem S. Lim, Manish Sadarangani, Dara A. Lehman, Jennifer Slyker, John Kinuthia, Dalton Wamalwa, Soren Gantt
Summary: Postpartum women and their infants in Nairobi, Kenya are at high risk for SARS-CoV-2 infection, with 66% of mothers and 44% of infants testing positive for the virus. Maternal infection is associated with an increased risk of infant infection, while HIV-exposed infants do not have a significantly higher risk. Most cases were asymptomatic, and women living with HIV did not have a substantially increased risk of infection or severe COVID-19.
Article
Genetics & Heredity
Vivian Paintsil, Mwashungi Ally, Hezekiah Isa, Kofi A. Anie, Josephine Mgaya, Malula Nkanyemka, Victoria Nembaware, Yaa Gyamfua Oppong-Mensah, Flora Ndobho, Lulu Chirande, Abel Makubi, Obiageli Nnodu, Ambroise Wonkam, Julie Makani, Kwaku Ohene-Frempong
Summary: This study aimed to establish minimum standards for the management of Sickle Cell Disease (SCD) in order to improve patient health outcomes. A review of 15 management guidelines resulted in the development of a comprehensive document with recommendations for all six different referral levels.
FRONTIERS IN GENETICS
(2023)
Review
Microbiology
Mahin Delara, Nirma Khatri Vadlamudi, Manish Sadarangani
Summary: Group B Streptococcus is a bacterium that can cause infections in pregnant women and newborns, resulting in adverse outcomes. Current preventive measures include intrapartum antibiotic prophylaxis, but it is not effective for all cases. Probiotics and immunization in pregnancy show promising results in preventing Group B Streptococcus infections. Challenges in developing GBS vaccines include understanding the optimal timing of administration and potential serotype switching.
Article
Pediatrics
Andrew S. J. Marshall, Alexandra Scrivens, Jennifer L. Bell, Louise Linsell, Pollyanna Hardy, Jean Yong, Rachel Williams, Eleri Adams, Manish Sadarangani, Edmund Juszczak, Charles C. Roehr, NeoCLEAR Collaborative Group
Summary: This study found that using a sitting position instead of a lying position during lumbar punctures in newborns can improve success rates. Sitting lumbar punctures are safe, cost-neutral, and well-tolerated. These results strongly support the widespread adoption of sitting technique for neonatal lumbar punctures.
LANCET CHILD & ADOLESCENT HEALTH
(2023)
Letter
Hematology
Florence Urio, Siana Nkya, Josephine Mgaya, Helen Rooks, Peter Ponsian, Sara El Hoss, Teddy Mselle, Julie Makani, Stephan Menzel
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Infectious Diseases
Tilmann Schober, Shaun K. Morris, Julie A. Bettinger, Catherine Burton, Scott A. Halperin, Taj Jadavji, Kescha Kazmi, Jacqueline Modler, Manish Sadarangani, Jesse Papenburg
Summary: The aim of this study was to determine the characteristics associated with inappropriate antibiotic use among children hospitalized for influenza. The results showed that over two-thirds of hospitalized children received antibiotics, including over half of those without an appropriate indication for antibiotic treatment. Differences among study centers suggest the importance of contextual determinants of antibiotic use.
Article
Public, Environmental & Occupational Health
Marilou Kiely, Fazia Tadount, Ernest Lo, Manish Sadarangani, Shu Qin Wei, Ellen Rafferty, Caroline Quach, Shannon E. Macdonald
Summary: This study found that females have a higher risk of adverse reactions following influenza vaccines compared to males, regardless of age and vaccine type. Transparent communication of this risk could increase trust in vaccines and reduce vaccine hesitancy.
JOURNAL OF EPIDEMIOLOGY AND COMMUNITY HEALTH
(2023)
Article
Pediatrics
Tiffany Fitzpatrick, Khaled Alsager, Manish Sadarangani, Anne Pham-Huy, Luis Murguia-Favela, Shaun K. Morris, Cynthia H. Seow, Pierre-Philippe Piche-Renaud, Tajdin Jadavji, Otto G. Vanderkooi, Karina A. Top, Cora Constantinescu
Summary: The study found that in utero exposure to biologic agents did not significantly affect lymphocyte subsets and the safety of live rotavirus vaccination. Following SIC assessment, the majority of infants were able to safely receive rotavirus vaccination.
LANCET CHILD & ADOLESCENT HEALTH
(2023)
Article
Medicine, General & Internal
Malou Bourdeau, Nirma Khatri Vadlamudi, Nathalie Bastien, Joanne Embree, Scott A. Halperin, Taj Jadavji, Kescha Kazmi, Joanne M. Langley, Marc H. Lebel, Nicole Le Saux, Dorothy Moore, Shaun K. Morris, Jeffrey M. Pernica, Joan Robinson, Manish Sadarangani, Julie A. Bettinger, Jesse Papenburg
Summary: This study provides an overview of the epidemiology and burden of RSV-associated hospitalizations among children and adolescents in Canadian tertiary pediatric hospitals from 2017 to 2022. The findings show that the number of RSV hospitalizations increased during this period, particularly during the COVID-19 pandemic. However, the severity of illness did not change compared to the prepandemic period.
Article
Hematology
Emmanuela E. Ambrose, Benson R. Kidenya, Mwesige Charles, Joyce Ndunguru, Agnes Jonathan, Julie Makani, Irene K. Minja, Paschal Ruggajo, Emmanuel Balandya
Summary: The purpose of this study was to assess the clinical and haematological outcomes of Hydroxyurea in children with Sickle cell anaemia (SCA) in North-western Tanzania, and to identify the barriers to its utilization. The study compared the outcomes of children with SCA at baseline and after at least one year of Hydroxyurea treatment accessed via cash, insurance, and projects. The results showed that children who accessed Hydroxyurea through insurance and projects experienced significant improvement in clinical and haematological outcomes. However, there were several barriers to access, including high cost, insurance challenges, and drug unavailability. These findings emphasize the need for efforts to improve sustainable access to Hydroxyurea for all SCA patients.
JOURNAL OF BLOOD MEDICINE
(2023)
Article
Oncology
Carol-Ann Benn, Cassandra P. T. Mbanje, Dominic Van Loggerenberg, Julie Makani
Summary: This study aimed to assess the benefit of culturally similar breast clinic navigators in facilitating treatment adherence and improving overall care in patients. Through counselling sessions and regular telephone follow-up, breast clinic navigators were able to address navigation concerns, provide support for the patient, and inform the multidisciplinary team on the patient's thought process and potential barriers for care. Thus, treatment plans were personalised, resulting in improved, holistic care.
EUROPEAN JOURNAL OF BREAST HEALTH
(2023)
