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
Hematology
Amina Nardo-Marino, Jesper Petersen, John N. Brewin, Henrik Birgens, Thomas N. Williams, Jorgen A. L. Kurtzhals, David C. Rees, Andreas Glenthoj
Summary: This study found a significant correlation between Oxygenscan-derived biomarkers and modifiers of disease severity in SCA, but failed to find any independent predictive value of Oxygenscan in clinical outcomes.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Respiratory System
Fatma Kort, Anoosha Habibi, Francois Lionnet, Marie-France Carette, Antoine Parrot, Laurent Savale, Hilario Nunes, Bernard Maitre, Frederic Schlemmer, Jean-Marc Naccache
Summary: Chronic interstitial lung abnormalities have been reported in patients with sickle cell disease, possibly due to a bronchiolar mechanism rather than more acute chest syndrome.
Article
Medicine, General & Internal
Julie Gargot, Marie-Claire Parriault, Antoine Adenis, Jerome Clouzeau, Kim-Anh Dinh Van, Balthazar Ntab, Antoine Defo, Mathieu Nacher, Narcisse Elenga
Summary: French Guiana has the highest incidence of sickle cell disease, and the incidence of stroke in children with sickle cell disease needs further investigation. SS/S beta 0 children have a higher risk of acute chest syndromes, elevated Lactate Dehydrogenase levels, and a higher incidence rate of stroke.
FRONTIERS IN MEDICINE
(2022)
Article
Hematology
Sara R. Rashkin, Evadnie Rampersaud, Guolian Kang, Kenneth I. Ataga, Jane S. Hankins, Winfred Wang, Jeremie H. Estepp, Mitchell J. Weiss, Jeffrey Lebensburger, Rima S. Zahr
Summary: Albuminuria can predict kidney disease progression in individuals with SCA, but introducing reno-protective therapies before the onset of albuminuria may attenuate disease progression. A genetic risk score (GRS) for SCA-related nephropathy may provide an improved one-time test for early identification of high-risk patients. Utilizing a GRS from a recent large trans-ethnic meta-analysis, three single nucleotide polymorphisms were identified to be associated with time to first albuminuria episode in children with SCA.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Hematology
Stephan L. Kienzle, Mark Rodeghier, Robert Liem
Summary: This study found that children with SCA had lower HRV during exercise testing and recovery. Further investigation on HRV and exercise safety in SCA children is needed.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Multidisciplinary Sciences
Huan Cao, Aristotelis Antonopoulos, Sadie Henderson, Heather Wassall, John Brewin, Alanna Masson, Jenna Shepherd, Gabriela Konieczny, Bhinal Patel, Maria-Louise Williams, Adam Davie, Megan A. Forrester, Lindsay Hall, Beverley Minter, Dimitris Tampakis, Michael Moss, Charlotte Lennon, Wendy Pickford, Lars Erwig, Beverley Robertson, Anne Dell, Gordon D. Brown, Heather M. Wilson, David C. Rees, Stuart M. Haslam, J. Alexandra Rowe, Robert N. Barker, Mark A. Vickers
Summary: The surface-exposed high mannose N-glycans on diseased or oxidized red blood cells bind to mannose receptor CD206 on phagocytes, mediating phagocytosis in sickle cell disease and malaria. This recognition of surface N-linked high mannose glycans as a response to cellular stress is a common molecular mechanism in the pathogenesis of sickle cell disease and resistance to severe malaria in individuals with sickle cell trait.
NATURE COMMUNICATIONS
(2021)
Article
Hematology
Nancy S. Green, Caterina Rosano, Paul Bangirana, Robert Opoka, Deogratias Munube, Philip Kasirye, Michael Kawooya, Samson K. Lubowa, Ezekiel Mupere, Andrea Conroy, Frank J. Minja, Amelia K. Boehme, Min Suk Kang, Lawrence S. Honig, Richard Idro
Summary: Cerebrovascular injury is common in children with sickle cell anaemia (SCA) in sub-Saharan Africa, but limited access to MRI-MRA hinders detection. Blood-based biomarkers, including neurofilament light chain (NfL), may help identify cerebral infarcts. In a study with Ugandan children with SCA, NfL levels were significantly higher in those with MRI-detected infarcts and elevated TCD velocity. NfL levels could potentially be used as a biomarker for SCA cerebrovascular injury.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Public, Environmental & Occupational Health
Grace Irimu, Jalemba Aluvaala, Lucas Malla, Sylvia Omoke, Morris Ogero, George Mbevi, Mary Waiyego, Caroline Mwangi, Fred Were, David Gathara, Ambrose Agweyu, Samuel Akech, Mike English
Summary: This study analyzed data of neonatal and pediatric admissions in Kenyan public hospitals, highlighting high mortality rates among neonates and significant variations in mortality across hospitals, indicating preventable causes of death.
Article
Hematology
Jeremie H. Estepp, Ze Cong, Irene Agodoa, Guolian Kang, Juan Ding, M. Beth McCarville, Jane S. Hankins, Winfred C. Wang
Summary: Children with sickle cell anaemia and conditional TCD flow velocities are at increased risk of stroke. The SCCRIP study demonstrated that increases in haemoglobin and fetal haemoglobin can lead to decreased TCD velocities in these children. Pharmacological intervention to increase haemoglobin and fetal haemoglobin was shown to normalize TCD velocities in this real-world dataset.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Hematology
Oyindamola Christiana Adebayo, DieuMerci Kabasele Betukumesu, Agathe Bikupe Nkoy, Oluyomi Modupe Adesoji, Pepe Mfutu Ekulu, Lambertus P. Van den Heuvel, Elena N. Levtchenko, Veerle Labarque
Summary: The study found a high burden of kidney damage among Congolese children and provided evidence of the possible role of APOL1 and HMOX1 in making children more susceptible to kidney complications.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
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
Clinical Neurology
Christine K. Fox, Liza Leykina, Nancy K. Hills, Janet L. Kwiatkowski, Julie Kanter, John J. Strouse, Jenifer H. Voeks, Heather J. Fullerton, Robert J. Adams
Summary: The study found that the incidence rate of hemorrhagic stroke in patients with sickle cell anemia increases with age, especially in adult patients over 18 years old. Structural vascular abnormalities such as moyamoya arteriopathy and aneurysms are common etiologies for hemorrhage, and screening may be warranted.
Review
Hematology
Andrea Piccin, Niamh O'Connor-Byrne, Massimo Daves, Kelvin Lynch, Amin Daei Farshbaf, Ignacio Martin-Loeches
Summary: Sickle cell disease is a genetic disorder with clinical manifestations that overlap with autoimmune diseases, making it difficult to distinguish clinically. Timely diagnosis is crucial for appropriate treatment.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Hematology
Christina M. Barriteau, Abraham Chiu, Mark Rodeghier, Robert I. Liem
Summary: The study showed that patients with sickle cell anaemia had lower cerebral StO(2) during exercise and also demonstrated differences in quadriceps StO(2) compared to controls. Further research on tissue oxygenation during exercise in sickle cell anaemia patients is warranted to better understand the impact of the disease on oxygenation levels.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Multidisciplinary Sciences
Bijun Wen, James M. Njunge, Celine Bourdon, Gerard Bryan Gonzales, Bonface M. Gichuki, Dorothy Lee, David S. Wishart, Moses Ngari, Emmanuel Chimwezi, Johnstone Thitiri, Laura Mwalekwa, Wieger Voskuijl, James A. Berkley, Robert H. J. Bandsma
Summary: Blood metabolomic and proteomic profiles can differentiate between children with severe malnutrition who survive and those who die. The profiles of children who died show increased metabolic substrates, microbial metabolites, acute phase proteins, and inflammatory markers, indicating disruptions in bioenergetic pathways and sepsis-like responses.
Review
Immunology
Husheem Michael, Joshua O. Amimo, Gireesh Rajashekara, Linda J. Saif, Anastasia N. Vlasova
Summary: Malnutrition increases the risk of infections and leads to impaired immune function and micronutrient deficiencies. Kwashiorkor has significant impacts on children's health, immunity, and gut functions. Pigs are a valuable model for studying the pathophysiology and immunology of kwashiorkor.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Alice Kamau, Moses Musau, Stella Mwakio, David Amadi, Amek Nyaguara, Philip Bejon, Anna C. Seale, James A. Berkley, Robert W. Snow
Summary: In an area with high levels of sulfadoxine-pyrimethamine (SP) resistance on the Kenyan coast, intermittent preventive treatment for pregnant women with SP continues to be associated with a reduced risk of low birthweight deliveries and stillbirths.
CLINICAL INFECTIOUS DISEASES
(2023)
Article
Hematology
Evarist Mulyahela Wilson, Irene Kida Minja, Ferdinand Mabula Machibya, Agnes Jonathan, Julie Makani, Paschal Ruggajo, Emmanuel Balandya
Summary: The purpose of the study was to determine the oxygen saturation in the dental pulp of primary teeth in children with sickle cell disease (SCD) and sickle cell trait (SCT). The study found that children with SCD had lower oxygen saturation in their teeth compared to those with SCT, while the oxygen saturation in their fingers remained unaffected.
JOURNAL OF BLOOD MEDICINE
(2022)
Editorial Material
Hematology
Julie Makani, Marina Cavazzana, Kalpna Gupta, Obiageli Nnodu, Isaac Odame, Leon Tshilolo, Russell Ware, Lucio Luzzatto
AMERICAN JOURNAL OF HEMATOLOGY
(2022)
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
Medicine, General & Internal
Alison Talbert, Moses Ngari, Christina Obiero, Amek Nyaguara, Martha Mwangome, Neema Mturi, Nelson Ouma, Mark Otiende, James Berkley
Summary: The number of young infants admitted to a county hospital in Kenya is increasing. The inpatient case fatality rate is 16% and the 1-year post-discharge mortality rate is 5.7%. Inpatient deaths are associated with disease severity, while post-discharge deaths are associated with hospitalization length, leaving against advice, and referral. This highlights the need for improved post-discharge care and services.
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)
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
Health Care Sciences & Services
Paraskevi Massara, Arooj Asrar, Celine Bourdon, Moses Ngari, Charles D. G. Keown-Stoneman, Jonathon L. Maguire, Catherine S. Birken, James A. Berkley, Robert H. J. Bandsma, Elena M. Comelli
Summary: This study assessed the performance of six methods for detecting different types of outliers, proposed two novel methods for outlier trajectory detection, and evaluated the impact of outliers on growth pattern detection. The results showed that model-based outlier detection methods performed best for measurements, especially for low and moderate error intensities. The clustering-based outlier trajectory method performed exceptionally well across all types and intensities of errors. Comparing growth groups with and without outliers demonstrated that outliers can alter group membership.
BMC MEDICAL RESEARCH METHODOLOGY
(2023)
Letter
Nutrition & Dietetics
Marko Kerac, Marie McGrath, James A. Berkley, Carlos S. Grijalva-Eternod, Natasha Lelijveld, Martha Mwangome, Eleanor Rogers
JOURNAL OF NUTRITIONAL SCIENCE
(2023)
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)
Article
Nutrition & Dietetics
Susan Thurstans, Stephanie Wrottesley, Bridget Fenn, Tanya Khara, Paluku Bahwere, James A. Berkley, Robert E. Black, Erin Boyd, Michel Garenne, Sheila Isanaka, Natasha Lelijveld, Christine M. McDonald, Andrew Mertens, Martha Mwangome, Kieran S. O'Brien, Heather Stobaugh, Sunita Taneja, Keith P. West, Saul Guerrero, Marko Kerac, Andre Briend, Mark Myatt
Summary: The risk of death from undernutrition is higher in younger children than in older children. Boys have a higher prevalence of undernutrition than girls. The risk of death is higher in younger children for underweight and stunting, but not for wasting. There are no sex differences in mortality risk for all deficits.
MATERNAL AND CHILD NUTRITION
(2023)