Description/EtiologyThe hemoglobino

Description/EtiologyThe hemoglobinopathies are a heterogeneous group of autosomal recessive disorders,characterized by abnormal synthesis or structure of the oxygen-carrying hemoglobin (Hgb)molecule in red blood cells (RBCs). Sickle cell disease (SCD) and the thalassemias are themost common hemoglobinopathies.In SCD, an alteration in the Hgb protein structure distorts the shape of RBCs. The resulting“sickle” shaped RBCs are fragile and can occlude small blood vessels. The hallmarksof SCD are chronic hemolytic anemia, painful vaso-occlusive crisis, and multiple organdamage. Early mortality is usually due to organ failure. SCD results from inheriting thecausative mutation from both parents; carriers of just one mutated gene are considered tohave sickle cell trait, which does not cause signs and symptoms. (For more information, seeQuick Lesson About … Sickle Cell Anemia ).The thalassemias are a wide spectrum of disorders characterized by reduced synthesis ofone or more subunits of the globin protein, a component of Hgb. The resulting imbalancein production of globin chains reduces Hgb stability and causes hypochromia (i.e., paleRBCs due to decreased Hgb content), microcytosis (i.e., small RBCs), ineffective RBCformation, and hemolytic anemia. Clinical manifestations are diverse, ranging fromasymptomatic hypochromia and microcytosis to profound anemia leading to death in uteroor in early childhood. Most thalassemia-related deaths are due to cardiac involvement. Inthe most severe form of α-thalassemia, α-thalassemia major, affected individuals usuallydie before or shortly after birth. The most severe form of β-thalassemia is β-thalassemiamajor, also known as Cooley’s anemia. (For more information, see Quick Lesson About …Thalassemia, Beta ).Hemoglobinopathies are suspected based on family history and clinical manifestations;blood tests demonstrating abnormal Hgb confirm the diagnosis. Although bone marrowtransplantation can be curative in patients with Hgb disorders, this therapy is not availableto most patients due to several factors, including lack of suitable donors and severetreatment-related morbidity. Treatment goals include symptom control and managementof disease complications. Patients with SCD are treated with analgesics, daily antibioticprophylaxis to age 5 to reduce risk of developing serious blood infections, pneumococcalvaccination, and folic acid supplementation to increase RBC production. Intensivesupportive care is needed for management of vaso-occlusivecrisis in SCD. Surgeryis often indicated for management of complications, including chronic leg ulcers andavascular necrosis. Standard therapy for more severe forms of thalassemia is regular bloodtransfusions with well-monitored iron chelation therapy.

Results (English) 1:

Description The / Etiology
the The Hemoglobinopathies Are A Group Of Heterogeneous an autosomal the recessive therapy disorders,
Characterized By Abnormal synthesis The Structure Of The oxygen's or Add-Carrying hemoglobin's (Hgb)
Molecule In Red Blood Cells (RBCs are). Cell Disease Sickle (SCD,) And The Thalassemias Are The
Most Common Hemoglobinopathies.
In SCD, an alteration in the Hgb protein structure distorts the shape of RBCs. Resulting the The
"Sickle" Are Shaped RBCs are Fragile Small Blood Vessels And Can Occlude. Hallmarks the The
Of SCD, of anemia, hemolytic Are Chronic, Painful Vaso-occlusive, Crisis, And Multiple diffuse organ
Damage. Early mortality is usually due to organ failure . SCD results from inheriting the
causative mutation from both parents; Just One Of Gene by the mutated Are Considered Carriers To
Have Sickle Cell Trait, Which Does Not cause's Signs And Symptoms. (: For More Information, by see
the Quick * About the Lesson ... the Cell Anemia Sickle).
A dedicated wide Spectrum Of therapy disorders Thalassemias Are the The Characterized By Reduced synthesis The Of
One Of The the globin subunits, or Add More Protein, A Component Of Hgb. Resulting an imbalance the The
In Production Of Stability And the globin, chains Reduces Hgb Causes Hypochromia (IE, Pale
RBCs are content Advanced Due To Decreased Hgb), Microcytosis (IE, Small RBCs are), the RBC Ineffective
Formation, And of anemia, hemolytic. Used diverse Manifestations Are the Clinical, ranging's From
asymptomatic, Hypochromia And Microcytosis To Death In Utero To Leading produces profound of anemia.
or in early childhood. Most thalassemia-related deaths are due to cardiac involvement. The In
The Most Severe Form Of-thalassemia, Α, Α-thalassemia, Major, AFFECTED Individuals Usually
Die Before or Add Shortly After Birth. Most Severe Form Of Β-thalassemia, the The Is Β-thalassemia,
Major, Also Known As Cooley, of anemia's. (: For More Information, by see the Quick * About the Lesson ...
thalassemia, Beta version,).
Hemoglobinopathies are suspected based on family history and clinical manifestations;
blood tests demonstrating abnormal Hgb confirm the diagnosis . Bone Marrow although
Transplantation Can Be Curative In Patients With Hgb therapy disorders, This Therapy Is Not Available
To Most Patients Due To Several Factors, Including Severe Lack Of Suitable And Donors.
treatment-related morbidity. Symptom Control And Management Goals include Special treatment This
Of Disease Complications. With SCD, Are Treated With analgesics,, Patients, daily's Antibiotic
Prophylaxis To Age 5 To reduce model Risk Of Developing Blood Infections Serious, Pneumococcal
Vaccination, And Supplementation of folic Acid Production To Increase the RBC. The Intensive
Supportive care water essen Is Needed For Management Of Vaso-In SCD, Occlusivecrisis. Surgery
Is often Do Indicated For Management Of Complications, Including Chronic Leg Ulcers And
Avascular necrosis is. For More Severe thalassemia, Forms Of Therapy: Standard Is Regular Blood
Transfusions With Well-standard iron Monitored the chelation Therapy.

Results (English) 2:

Description/Etiology The hemoglobinopathies are a heterogeneous group of autosomal recessive disorders, characterized by abnormal synthesis or structure of The oxygen-carrying hemoglobin (HGB) molecule in red blood cells (RBCs). Sickle cell disease (SCD) and the Thalassemias are The most common hemoglobinopathies. In SCD, an alteration in The HGB protein structure distorts The shape of RBCs. The resulting "Sickle" shaped the RBCs are fragile and can occlude small blood vessels. The hallmarks of SCD are chronic hemolytic anemia, painful vaso-occlusive crisis, multiple organ damage. Early mortality is usually due to organ failure. SCD results from inheriting The causative mutation from both parents; carriers of just one mutated gene are considered to have Sickle cell trait, which does not cause signs and symptoms. (For more information, see Quick Lesson About... Sickle Cell Anemia). The Thalassemias are a wide spectrum of disorders characterized by reduced synthesis of one or more subunits of The globin protein, a component of HGB. The resulting imbalance in production of globin chains reduces HGB stability and causes hypochromia (i.e., pale RBCs due to decreased Hgb the content), microcytosis (i.e., small RBCs), ineffective RBC formation, and hemolytic anemia. Clinical manifestations are diverse, ranging from asymptomatic hypochromia and microcytosis to profound anemia leading to death in utero or in Early childhood. Most thalassemia-related deaths are due to cardiac involvement. In The most severe form of Α-thalassemia, Α-thalassemia major, affected individuals usually die before or shortly after birth. The most severe form of β-thalassemia is Β-thalassemia major, and known as Cooley's anemia. (For more information, to see the "Quick Lesson About...", the Hemoglobinopathies are suspected based on family history and clinical manifestations; blood tests demonstrating abnormal HGB confirm the diagnosis. Although bone marrow transplantation can be curative in patients with HGB disorders, this therapy is not available to most patients due to several factors, including lack of suitable donors and severe treatment-related morbidity. Treatment goals include symptom control and management of disease complications. Patients with SCD are treated with analgesics, daily antibiotic prophylaxis to age 5 to reduce risk of developing serious blood infections, pneumococcal vaccination, and folic acid supplementation to increase RBC production. Intensive supportive care is needed For management of vaso-occlusivecrisis in SCD. Surgery is often indicated For management of complications, including chronic leg ulcers and avascular necrosis. Standard therapy For More severe forms of thalassemia is regular blood transfusions with well-monitored iron chelation therapy.

Results (English) 3:

accounting
Blood cell disease is a kind of self recessive disease.
Synthesis of structural abnormalities of hemoglobin carried by oxygen, hgb41
The molecules in holmium RBCs and sick cell diseases holmium SCD 41 and ASTA.
The most common blood cell disease.
In SCD, the change of HGB protein structure makes RBC shape deformed.
The "sik" shaped RBC is fragile and can seal small blood containers.
Chronic hemolytic anemia, vascular occlusion, multiple organ pain
Severe lethality is usually due to organ damage.
Caused by a mutation in a parent and carrying a mutated gene
There is a trace of a sick cell, which will not cause signs and symptoms. For more information, see
Quick lesson about cell euthanasia
Ophthalmopathy is a widespread disease characterized by reduced synthesis.
One or more units of whole protein, a component of HGB, result in imbalance.
The reasons for the reduction of HGB stability and low chromium in global chain production: i.e.pale
RBC duel reduces HGB content and small cells, i.e. small RBCs 41 and invalid RBC
The clinical manifestations are various
Low chromium and microcellular infiltrative anemia lead to intrauterine death.
In early childhood, the most serious deaths were related to heart disease.
The most severe alpha metabolic syndrome, most alpha metabolic syndrome, usually affects individuals
Death before or shortly after birth. The most serious is β - assemia.
Mainly, I also know that this is a good memory. For more information, see Quebec lesson about
Tarasemia and beta
Schistosomiasis is based on family history and clinical manifestations.
Blood test confirmed the diagnosis of abnormal HB
Transplantation can treat patients with HGB disorder, which is not available treatment.
Most patients have several factors, including suitable donors and several
Treatment objectives include symptom control and management.
Combination of antifungal drugs in the treatment of Parkinson's disease
Five ways to prevent pneumonia to reduce the risk of serious blood infection
Vaccination and folic acid supplementation improved RBC production.
It is necessary to support the surgical treatment of vascular occlusive diseases.
It is often used to deal with complex things, including chronic and chronic diseases.
Standardized treatment of several typical hemorrhagic hemorrhagic diseases with vascular necrosis
Fusion of iron chelation therapy and well monitoring