What does HbSS stand for in sickle cell?
HbSS, or sickle cell anemia, is the most common subtype of sickle cell disease. In this subtype, the affected person inherits one sickle-cell gene from each parent. This is called a homozygous sickle cell disease.[^10]
What is HbSS patient?
Sickle cell disease refers to the group of disorders that affects haemoglobin to form abnormal haemoglobin molecules (HbS). Sickle cell anaemia is the name of the specific form of sickle cell disease in which there is homozygosity for the mutation that causes HbS (ie HBSS).
How is HbSS diagnosed?
The diagnosis of HbSC disease is made with Hb electrophoresis. The peripheral blood smear may have some sickled cells and a high proportion of target cells. In addition, microcytic, dehydrated, dense RBCs are seen. These may contain crystal-like condensations.
What causes sickle cell disease?
What causes sickle cell disease? Sickle cell is an inherited disease caused by a defect in a gene. A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father. A person who inherits just one gene is healthy and said to be a “carrier” of the disease.
What is the difference between HbS and HbSS?
HbS has a substituted amino acid at the 6th codon of the beta chain with valine replacing glutamic acid. Homozygous SCD patients (HbSS) have two copies of this altered Hb. Heterozygous HbSC individuals have a second mutated Hb in addition, HbC in which glutamic acid at the same position is replaced with lysine.
What is the difference between sickle cell anemia and thalassemia?
Thalassemia is a quantitative problem of too few globins synthesized, whereas sickle-cell anemia (a hemoglobinopathy) is a qualitative problem of synthesis of an incorrectly functioning globin. Thalassemias usually result in underproduction of normal globin proteins, often through mutations in regulatory genes.
What are the symptoms of sickle cell crisis?
This can be life-threatening and should be treated in a hospital. It happens when a large number of sickle cells get trapped in the spleen and cause it to suddenly get large. Symptoms include sudden weakness, pale lips, fast breathing, extreme thirst, abdominal (belly) pain on the left side of body, and fast heartbeat.
What is the life expectancy of sickle cell disease?
Results. Among children and adults with sickle cell anemia (homozygous for sickle hemoglobin), the median age at death was 42 years for males and 48 years for females. Among those with sickle cell-hemoglobin C disease, the median age at death was 60 years for males and 68 years for females.
What blood type causes sickle cell?
Like most genes, individuals inherit one from each parent. Examples: If one parent has sickle cell anemia (SS) and the other parent has normal (AA) blood, all of the children will have sickle cell trait.
What is the difference between HbC and HbS?
HbC has been described as being more advantageous than HbS because, even in homozygous individuals, it is usually non-fatal. However, in contrast to HbS, it does not prevent malaria due to P. vivax, and is less effective in resistance to falciparum malaria in heterozygous conditions.
What are the 3 types of sickle cell?
There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle Hemoglobin-C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.
What is HBSS buffer used for?
Hanks′ Balanced Salt solution (HBSS) is a buffer that mimics biological medium and stabilizes cells. It is useful in leucocyte labeling studies. HBSS is an effective preservative for periodontal ligament cells. HBSS lacks D-glucose and essential inorganic ions and is useful in starvation studies.
What is the use of HBSS in cell culture?
HBSS can be used for washing cells or as a solution for transporting cells and tissue culture. We are currently unable to calculate your contract price for this item.
What does HBSS stand for?
Hanks′ Balanced Salt solution (HBSS) is a buffer that mimics biological medium and stabilizes cells. It is useful in leucocyte labeling studies.
What is the role of HBSS in periodontology?
HBSS is an effective preservative for periodontal ligament cells. HBSS lacks D-glucose and essential inorganic ions and is useful in starvation studies. Phenol red has been shown to interfere with the growth of some cells at cloning densities.