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alpha-Thalassemia

A disorder characterized by reduced synthesis of the alpha chains of hemoglobin. The severity of this condition can vary from mild anemia to death, depending on the number of genes deleted.
Also Known As:
A-Thalassemia; Alpha Thalassemia; Disease, Hemoglobin H; Thalassemia alpha; Thalassemia, Alpha; alpha-Thalassemias; Hemoglobin H Disease; Thalassemia-alpha
Networked: 1146 relevant articles (12 outcomes, 125 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Anemia
2. Sickle Cell Anemia (Hemoglobin S Disease)
3. Hydrops Fetalis (Fetal Edema)
4. Glucosephosphate Dehydrogenase Deficiency
5. Pain (Aches)

Experts

1. Fucharoen, Supan: 22 articles (01/2018 - 01/2002)
2. Fucharoen, Goonnapa: 20 articles (01/2018 - 01/2002)
3. Sanchaisuriya, Kanokwan: 17 articles (01/2015 - 01/2002)
4. Giordano, Piero C: 17 articles (08/2010 - 08/2002)
5. Harteveld, Cornelis L: 17 articles (08/2010 - 08/2002)
6. Fucharoen, Suthat: 15 articles (01/2014 - 05/2002)
7. Fucharoen, S: 10 articles (10/2003 - 01/2000)
8. Winichagoon, Pranee: 9 articles (05/2010 - 05/2002)
9. van Delft, Peter: 9 articles (01/2010 - 08/2002)
10. Jetsrisuparb, Arunee: 8 articles (12/2021 - 01/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to alpha-Thalassemia:
1. DNA (Deoxyribonucleic Acid)IBA
2. IronIBA
3. Hemoglobins (Hemoglobin)IBA
4. GlobinsIBA
5. hemoglobin Bart'sIBA
6. Fetal Hemoglobin (Hemoglobin F)IBA
7. Hemoglobin EIBA
8. zeta-GlobinsIBA
9. Hydroxyurea (Hydrea)FDA LinkGeneric
10. PolymersIBA
01/01/1989 - "Our analyses of equilibrium polymer formation at physiological oxygen saturation values suggest that small decreases in polymer formation at intermediate levels of hemoglobin F may give rise to a small decrease in anemia (as associated with homozygous alpha-thalassemia coexistent with sickle cell anemia), but that greater reductions in polymer formation may be necessary to effect a significant improvement in disease severity. "
01/01/1985 - "In our studies, the polymer fraction in sickle red cells was determined from reported mean values for hemoglobin composition and mean corpuscular hemoglobin concentration (MCHC) in 12 groups of patients with sickle hemoglobinopathies (homozygotes for HbS, with and without coexistent alpha-thalassemia or various forms of the hereditary persistence of fetal hemoglobin [HPFH], beta+-, beta 0-, and delta beta-thalassemia, and heterozygotes for HbS with HbA). "
02/13/2009 - "To achieve the best CE separation, several parameters were evaluated for optimizing the separation conditions, including the capillary coating, the concentrations of polymer sieving matrix, the sizes and concentrations of GNPs, the buffer concentrations, and the pH. The final CE method for separating a 200-base pair (bp) DNA ladder and alpha-thalassemia deletions used a DB-17 capillary, 0.6% poly(ethylene oxide) (PEO) prepared in a mixture of GNP(32nm) solution and glycine buffer (25mM, pH 9.0) (80:20, v/v) as the sieving matrix with 1microM YO-PRO-1 for fluorescence detection; the applied voltage was -10kV (detector at anode side) and the separation temperature was 25 degrees C. "

Therapies and Procedures

1. Prenatal Care (Care, Prenatal)
2. Nebulizers and Vaporizers (Inhaler)
3. Ligation
4. Splenectomy
5. Blood Transfusion (Blood Transfusions)
09/12/2017 - "Here, we present a case of a 5-year-old male child of Middle Eastern origin who had been diagnosed as having alpha thalassemia and was undergoing chronic blood transfusions. "
09/01/1995 - "The compound heterozygosity expresses as a thalassemia intermedia with moderate anemia, a variable need for blood transfusions, Heinz body formation, and a red cell morphology which is typical for such a condition. "
01/01/2023 - "Of 13 citations, 12 studies (n=82) reported diagnostic criteria for mirror syndrome: maternal edema (11/12), fetal hydrops (9/12), placental edema (6/12), placentomegaly (5/12), and preeclampsia (2/12); 12 studies (n=82) described the clinical presentation of mirror syndrome as maternal edema (62.2%), hypoalbuminemia (54.9%), anemia (39.0%), and new-onset hypertension (39.0%); 4 studies (n=36) reported that hemodilution was present in all patients; 8 studies (n=36) reported the etiology of fetal hydrops, with the most common being structural cardiac malformations (19.4%), alpha thalassemia (19.4%), Rh isoimmunization (13.9%), and nonimmune hydrops fetalis (13.9%); and 6 studies (n=47) reported maternal complications, 89.4% of which were major: postpartum hemorrhage (44.7%), hemorrhage requiring blood transfusion (19.1%), intensive care unit admission (12.8%), heart failure (10.6%), pulmonary edema (8.5%), and renal dysfunction (8.5%). "
07/01/2020 - "Furthermore, 3.7 kb alpha-thalassemia deletion homozygotes had a significantly higher age of the first manifestation, lower number of blood transfusions by year, higher hemoglobin, lower mean corpuscular volume, mean corpuscular hemoglobin, and lower hemolytic rate observed by a lower number of reticulocytes count. "
05/01/2020 - "Patients with hemoglobin H disease, which is a type of α-thalassemia, are usually transfusion independent, except in occasions where an external stressful factor leads to a drop in hemoglobin and necessitates blood transfusion. "