Functional polymorphisms of BCL11A and HBS1L-MYB genes affect both fetal hemoglobin level and clinical outcomes in a cohort of children with sickle cell anemia

2020 ◽  
Vol 99 (7) ◽  
pp. 1453-1463
Author(s):  
Rahyssa Rodrigues Sales ◽  
André Rolim Belisário ◽  
Gabriela Faria ◽  
Fabiola Mendes ◽  
Marcelo Rizzatti Luizon ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Fetal Hemoglobin ◽  
Hemoglobin Level ◽  
Functional Polymorphisms ◽  
Myb Genes ◽  
Fetal Hemoglobin Level

Fetal hemoglobin level and its relationship with spleen size and malaria parasite density in Nigerian children with sickle cell anemia

2018 ◽  
Vol 11 (4) ◽  
pp. 133 ◽  
Author(s):  
MorenikeAgnes Akinlosotu ◽  
OlugbengaO Adeodu ◽  
SamuelAdemola Adegoke ◽  
SaheedBabajide Oseni ◽  
OlugbengaO Ayoola
Keyword(s):  
Sickle Cell ◽  
Parasite Density ◽  
Sickle Cell Anemia ◽  
Malaria Parasite ◽  
Fetal Hemoglobin ◽  
Hemoglobin Level ◽  
Spleen Size ◽  
Nigerian Children ◽  
Fetal Hemoglobin Level

Clinical epidemiology of individuals with Sickle cell anemia using Hydroxyurea at Muhimbili National Hospital, Dar Es Salaam, Tanzania

2020 ◽  
Vol 31 (1) ◽  
pp. 106-119
Author(s):  
Elisha Osati ◽  
Edward Kija ◽  
Florence Urio ◽  
Magdalena Lyimo ◽  
Siana Nkya ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Severe Pain ◽  
Clinical Epidemiology ◽  
Fetal Hemoglobin ◽  
Dar Es Salaam ◽  
Acute Chest Syndrome ◽  
Muhimbili National Hospital ◽  
National Hospital

Background: The pathophysiology of sickle cell disease (SCD) is complex and involves nitric oxide depletion, increased inflammation/adhesion molecules and vaso-occlusion in addition to the chronic hemolytic anemia. This pathophysiology results in systemic clinical complications including recurrent episodes of severe pain, stroke, acute chest syndrome (ACS) and an increased susceptibility to infection. SCD severity varies among individuals and fetal hemoglobin (HbF) is known as a major modulator of the disease. To date, hydroxyurea (HU) is a known intervention that acts by increasing HbF in individuals with SCD. The increase in HbF reduces the risk of ‘sickling’ events and improves clinical outcomes. This is the first study on the use of HU in individuals with SCA in Tanzania.Methods: A case-control study to determine the proportion, indications, clinical and laboratory outcomes of SCD patients with HU use was conducted at Muhimbili National Hospital in Dar Es Salaam, Tanzania.Results: Forty-two patients with Sickle cell anemia (SCA) on HU treatment and 32 patients with SCA not on HU treatment were enrolled. The proportion of HU use by individuals with SCA at Muhimbili National Hospital was 10 per 1000. The mean HbF % was 9.8 ± 2.4 vs 6.2 ±1.4 for controls (P <0.001). Thirty (71.4%) were enrolled for HU treatment due to central nervous system (CNS) events, frequent painful crises 11(26.2%) and recurrent anemia 1(2.4%). Thirty-two SCA patients (76.2%) reported improvements after being on HU for at least six months. Of these, 91% reported no history of severe pain that required hospitalizations since they started HU. Twenty patients (66.7%) out of those with CNS events reported not to have experienced convulsions after HU initiation.Conclusions: HbF was higher in patients who were on HU and had positive correlation with clinical outcomes. Further clinical trials are required to evaluate more effects of HU use among SCA individuals in Tanzania. Keywords: Sickle cell anemia, HU, Fetal hemoglobin, Tanzania.

The Effect of Sickle Cell Genetic Markers on Geographic Distribution and Relation to Pain Outcomes in the Multicenter Study of Hydroxyurea in Sickle Cell Anemia

2011 ◽  
Vol 07 (01) ◽  
pp. 64
Author(s):  
Samir K Ballas ◽  
Robert L Bauserman ◽  
William F McCarthy ◽  
Myron A Waclawiw ◽  
◽  
...  
Keyword(s):  
Genetic Markers ◽  
Sickle Cell ◽  
Multicenter Study ◽  
Fetal Hemoglobin ◽  
Hemoglobin Level ◽  
Future Research ◽  
Cell Disease ◽  
Pain Outcomes ◽  
Treatment Responder ◽  
Fetal Hemoglobin Level

Some genetic markers known to play a role in sickle cell disease were associated with classification as a treatment responder, pain-related outcomes, and equi-analgesic dosing in the Multicenter Study of Hydroxurea (MSH) cohort. However, when examined by sex, associations with equi-analgesic dosing were statistically significant for males only. Factors that increase the hemoglobin/hematocrit levels seem not to be beneficial. Future research should focus on factors that increase fetal hemoglobin level.

Association of fetal hemoglobin level with frequency of acute pain episodes in sickle cell disease (HbS-only phenotype) patients

2019 ◽  
Vol 75 ◽  
pp. 30-34 ◽  
Author(s):  
Bimal Prasad Jit ◽  
Pradeep Kumar Mohanty ◽  
Prasanta Purohit ◽  
Kishalaya Das ◽  
Siris Patel ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Pain ◽  
Fetal Hemoglobin ◽  
Hemoglobin Level ◽  
Cell Disease ◽  
Fetal Hemoglobin Level

scholarly journals High fetal hemoglobin level is associated with increased risk of cerebral vasculopathy in children with sickle cell disease in Mayotte

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Abdourahim Chamouine ◽  
Thoueiba Saandi ◽  
Mathias Muszlak ◽  
Juliette Larmaraud ◽  
Laurent Lambrecht ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Hemoglobin Level ◽  
Cell Disease ◽  
Increased Risk ◽  
Fetal Hemoglobin Level
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