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
Medicine, General & Internal
Erica B. Esrick, Leslie E. Lehmann, Alessandra Biffi, Maureen Achebe, Christian Brendel, Marioara F. Ciuculescu, Heather Daley, Brenda MacKinnon, Emily Morris, Amy Federico, Daniela Abriss, Kari Boardman, Radia Khelladi, Kit Shaw, Helene Negre, Olivier Negre, Sarah Nikiforow, Jerome Ritz, Sung-Yun Pai, Wendy B. London, Colleen Dansereau, Matthew M. Heeney, Myriam Armant, John P. Manis, David A. Williams
Summary: This study confirms BCL11A inhibition as an effective approach for inducing HbF in sickle cell disease. Preliminary evidence suggests that shmik-based gene knockdown offers a favorable risk-benefit profile in treating sickle cell disease.
NEW ENGLAND JOURNAL OF MEDICINE
(2021)
Article
Biochemistry & Molecular Biology
Anoosha Habibi, Giovanna Cannas, Pablo Bartolucci, Ersi Voskaridou, Laure Joseph, Emmanuelle Bernit, Justine Gellen-Dautremer, Corine Charneau, Stephanie Ngo, Frederic Galacteros
Summary: Sickle cell disease is a group of inherited hemoglobin disorders characterized by altered deformability of red blood cells and various complications. Hydroxyurea is a major therapeutic agent for sickle cell patients, but its safety during pregnancy is not well-established. This study aims to describe pregnancy outcomes in the European Sickle Cell Disease COhoRT-HydroxyUrea study and share the results with the scientific community.
Article
Hematology
Shehu U. Abdullahi, Surayya Sunusi, Mohammed Sani Abba, Saifuddeen Sani, Hauwau Aminu Inuwa, Safiya Gambo, Awwal Gambo, Bilya Musa, Brittany V. Covert Greene, Adetola A. Kassim, Mark Rodeghier, Nafiu Hussaini, Mariana Ciobanu, Muktar H. Aliyu, Lori C. Jordan, Michael R. DeBaun
Summary: This study investigated the effectiveness of fixed oral moderate-dose hydroxyurea compared with fixed oral low-dose hydroxyurea for secondary stroke prevention in children with sickle cell anemia. The results showed that there was no evidence to support the superiority of moderate-dose hydroxyurea in reducing the risk of stroke or death compared to low-dose hydroxyurea.
Review
Immunology
Claire C. Sharpe, Abid Suddle, Sara Stuart-Smith
Summary: Sickle cell disease is a common genetic disorder with a global prevalence of over 300,000 people. While standard care can help patients live into adulthood, complications can still arise, leading to end-stage organ disease. Solid organ transplantation is an established treatment option for sickle cell patients, but careful patient selection and management are crucial for success. Hematopoietic stem cell transplantation and gene therapy may offer future treatments for adult patients with chronic organ damage, but they are not yet widely available. Currently, early intervention and comprehensive care can minimize the need for solid organ transplantation later in life.
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
Pharmacology & Pharmacy
Akancha Pandey, Jeremie H. Estepp, Rubesh Raja, Guolian Kang, Doraiswami Ramkrishna
Summary: This study developed pharmacokinetics and pharmacodynamics models to investigate the dosing and efficacy of hydroxyurea treatment in children with sickle cell disease (SCD). The models provided excellent fits for individuals with known or correctly inferred adherence, and can be used to determine optimal dosing regimens and study the impact of non-adherence on treatment outcomes.
Review
Biochemistry & Molecular Biology
Pagona Flevari, Ersi Voskaridou, Frederic Galacteros, Giovanna Cannas, Gylna Loko, Laure Joseph, Pablo Bartolucci, Justine Gellen-Dautremer, Emmanuelle Bernit, Corine Charneau, Anoosha Habibi
Summary: This article reports a case of myelodysplastic syndrome and highlights the lack of significant conclusions due to the scarcity of similar cases.
Article
Chemistry, Multidisciplinary
Abhishek Gour, Pankul Kotwal, Ashish Dogra, Dilpreet Kour, Sumit Dhiman, Amit Kumar, Sanjeev Kumar Digra, Ajay Kumar, Gurdarshan Singh, Utpal Nandi
Summary: Andrographolide shows significant antisickling activity and may be a potential adjuvant therapy for sickle cell anemia. However, caution should be taken when used concomitantly with hydroxyurea.
Article
Hematology
Andrew M. Heitzer, Jennifer Longoria, Victoria Okhomina, Winfred C. Wang, Darcy Raches, Brian Potter, Lisa M. Jacola, Jerlym Porter, Jane E. Schreiber, Allison A. King, Guolian Kang, Jane S. Hankins
Summary: Neurocognitive impairment is common in sickle cell disease, and this study found that older age was associated with a decrease in overall intelligence quotient for patients with HbSS/HbS beta(0)-thalassaemia. Early initiation of hydroxyurea treatment was associated with higher neurocognitive scores across most domains, suggesting its potential in limiting the detrimental effects of SCD on cognitive functions.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Surgery
Alicia D. Menchaca, Candace C. Style, Anthony D. Villella, Maria Burdjalov, Tariku J. Beyene, Peter C. Minneci, Oluyinka O. Olutoye
Summary: This study investigated the association between hydroxyurea therapy and the need for surgical splenectomy in patients with sickle cell disease (SCD). The results showed that patients receiving hydroxyurea had a higher rate of surgical splenectomy and underwent the procedure at a younger age. These findings provide useful information for clinicians and patients.
JOURNAL OF SURGICAL RESEARCH
(2023)
Article
Hematology
Ruth Namazzi, Robert Opoka, Andrea L. Conroy, Dibyadyuti Datta, Abner Tagoola, Caitlin Bond, Michael J. Goings, Moon-Suhn Ryu, Sarah E. Cusick, Nancy F. Krebs, Jeong Hoon Jang, Wanzhu Tu, Russell E. Ware, Chandy C. John
Summary: Data from small clinical trials show that zinc supplementation reduces infection in adolescents and adults with sickle cell anemia (SCA). However, there is a lack of studies on the effects of zinc supplementation for infection prevention in children with SCA in Africa. This randomized trial in Ugandan children aged 1.00-4.99 years with SCA found that daily zinc supplementation did not prevent severe or invasive infections. Zinc deficiency was also prevalent among the supplemented children. Further investigation is needed to determine the optimal zinc dosage and the role of zinc in preventing stroke or death in SCA.
Article
Hematology
Sindy N. Escobar Alvarez, Elizabeth R. Myers
Summary: The Doris Duke Charitable Foundation provided funding for sickle cell disease (SCD) research through the Innovations in Clinical Research Awards program, resulting in substantial advancements in diagnosis and treatment of the disease. The research investment showed substantial financial return and led to significant innovations in the field.
Article
Pharmacology & Pharmacy
Claire Pressiat, Marie-Georgine Rakotoson, Anoosha Habibi, Caroline Barau, Raphaele Arrouasse, Frederic Galacteros, Thomas Stehle, Vincent Audard, Anne Hulin, Pablo Bartolucci
Summary: This study developed a population pharmacokinetics model of hydroxyurea in sickle cell disease patients, finding that estimated glomerular filtration rate (eGFR) and body weight impact HU pharmacokinetics. Urinary HU fractions and renal clearance differed between different chronic kidney disease groups.
BRITISH JOURNAL OF CLINICAL PHARMACOLOGY
(2021)
Article
Hematology
Wally R. Smith, Donna K. McClish, Richard Lottenberg, India Y. Sisler, Daniel Sop, Shirley Johnson, Anthony Villella, Darla Liles, Elizabeth Yang, Ian Chen
Summary: Patients eligible for hydroxyurea treatment who were exposed to patient navigators showed improvements in starting hydroxyurea treatment and adherence, but there were no significant differences in hematological parameters between the experimental and control groups at 12 months.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Editorial Material
Obstetrics & Gynecology
Mara Curaba, Jonathan Poels, Anne van Langendonckt, Jacques Donnez, Christine Wyns
FERTILITY AND STERILITY
(2011)
Article
Obstetrics & Gynecology
Mara Curaba, Magali Verleysen, Christiani Andrade Amorim, Marie-Madeleine Dolmans, Anne van Langendonckt, Outi Hovatta, Christine Wyns, Jacques Donnez
FERTILITY AND STERILITY
(2011)
Article
Endocrinology & Metabolism
Christine Wyns
GYNECOLOGICAL ENDOCRINOLOGY
(2013)
Article
Obstetrics & Gynecology
C. Wyns, G. Abu-Ghannam, J. Poels
GYNECOLOGIE OBSTETRIQUE & FERTILITE
(2013)
Article
Obstetrics & Gynecology
C. Wyns, M. Curaba, S. Petit, B. Vanabelle, P. Laurent, J-F X. Wese, J. Donnez
HUMAN REPRODUCTION
(2011)
Article
Obstetrics & Gynecology
E. A. F. Dancet, T. M. D'Hooghe, W. Sermeus, I. van Empel, H. Strohmer, C. Wyns, D. Santa-Cruz, L. G. Nardo, D. Kovatchki, L. Vanlangenakker, J. Garcia-Velasco, B. Mulugeta, W. L. D. M. Nelen, J. A. M. Kremer
HUMAN REPRODUCTION
(2012)
Article
Obstetrics & Gynecology
D. De Neubourg, K. Bogaerts, C. Wyns, A. Albert, M. Camus, M. Candeur, M. Degueldre, A. Delbaere, A. Delvigne, P. De Sutter, M. Dhont, M. Dubois, Y. Englert, N. Gillain, S. Gordts, W. Hautecoeur, E. Lesaffre, B. Lejeune, F. Leroy, W. Ombelet, S. Perrier D'Hauterive, F. Vandekerckhove, J. Van der Elst, T. D'Hooghe
HUMAN REPRODUCTION
(2013)
Article
Obstetrics & Gynecology
Jonathan Poels, Anne Van Langendonckt, Marie-Christine Many, Francois-Xavier Wese, Christine Wyns
HUMAN REPRODUCTION
(2013)
Letter
Obstetrics & Gynecology
D. De Neubourg, K. Bogaerts, C. Wyns, M. Camus, A. Delbaere, A. Delvigne, P. De Sutter, M. Dubois, S. Gordts, B. Lejeune, F. Leroy, F. Vandekerckhove, T. D'Hooghe
HUMAN REPRODUCTION
(2014)
Article
Obstetrics & Gynecology
Christine Wyns, Mara Curaba, Bernard Vanabelle, Anne Van Langendonckt, Jacques Donnez
HUMAN REPRODUCTION UPDATE
(2010)
Article
Reproductive Biology
J. Poels, A. Van Langendonckt, J. P. Dehoux, J. Donnez, C. Wyns
Article
Hematology
Aime Lukusa Kazadi, Rene Makuala Ngiyulu, Jean Lambert Gini-Ehungu, Jean Marie Mbuyi-Muamba, Michel Ntetani Aloni
Article
Oncology
Michel Ntetani Aloni, Renault Sitwaminya Kambere, Antoine Molua, Joseph Nzinga Dilu, Pierre Manianga Tshibassu, Aime Kazadi-Lukusa, Rene Makuala Ngiyulu, Raphael Mbona Kalengayi, Jean Lambert Gini Ehungu
Article
Pediatrics
Fefe Khuabi Matondo, Kikuni Takaisi, Adolphine Bedi Nkuadiolandu, Aime Kazadi Lukusa, Michel Ntetani Aloni
INTERNATIONAL JOURNAL OF PEDIATRICS
(2016)