Hemophilia Blood Disorder

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Questions and Answers

What is the inheritance pattern of hemophilia A and B?

Autosomal recessive

Y-linked dominant

Autosomal dominant

X-linked recessive (correct)

What is the most common symptom of hemophilia?

Sudden bloody noses

Bleeding in the urine or stool

Prolonged bleeding (correct)

Easy bruising

What determines the severity of hemophilia symptoms?

Amount of clotting factors in the blood (correct)

Age of the individual

Family medical history

Sex of the individual

What is the clotting factor level in severe hemophilia?

Less than 1% of the normal clotting factors Signup and view all the answers

What is the first step in diagnosing hemophilia?

Complete history and physical examination Signup and view all the answers

What is the type of mutation that can cause hemophilia?

Intrachromosomal recombination Signup and view all the answers

What happens when people have hemophilia?

Their blood doesn't clot and bleeding doesn't slow down or stop Signup and view all the answers

What is the most common type of hemophilia?

Hemophilia A Signup and view all the answers

What is a common symptom of hemophilia in the mouth?

Bleeding of the mouth and gums Signup and view all the answers

Who is more likely to inherit hemophilia?

Both males and females equally Signup and view all the answers

What is necessary for a female to show symptoms of Hemophilia A or B?

Two copies of the mutated gene Signup and view all the answers

Where are the genes for Hemophilia A and B located?

X chromosome Signup and view all the answers

What is Hemophilia C?

A type of hemophilia caused by a mutation in the F11 gene Signup and view all the answers

How is Hemophilia C inherited?

Autosomal recessive Signup and view all the answers

What is necessary for a person to have symptoms of Hemophilia C?

Two copies of the mutated F11 gene Signup and view all the answers

What determines the severity of hemophilia?

The amount of clotting factor in the blood Signup and view all the answers

At what age are children with hemophilia often diagnosed?

12 to 18 months Signup and view all the answers

What is a common symptom of hemophilia in children?

Easy bleeding with minor injury Signup and view all the answers

What does a high PT/INR result indicate?

The body takes longer than normal to form blood clots Signup and view all the answers

What does a complete blood count (CBC) measure?

The amounts and sizes of RBCs, hemoglobin, WBCs, and platelets Signup and view all the answers

What is the purpose of a specific clotting factor test?

To show levels of specific clotting factor levels Signup and view all the answers

What is the scope of hemophilia's impact?

Worldwide Signup and view all the answers

What is the primary goal of dental management for hemophiliacs?

To prevent gum disease and maintain good oral health Signup and view all the answers

Why should hemophiliacs avoid certain pain medications?

Because they can cause bleeding Signup and view all the answers

What is an alternative to aspirin and ibuprofen for hemophiliacs?

Tylenol Signup and view all the answers

Why should intensive dental treatments be carried out in hospitals or community dental services?

To manage significant bleeding risks Signup and view all the answers

What is recommended for hemophiliacs to prevent gum disease and buildup of dental tartare?

Regular visits to the dentist every 6 months Signup and view all the answers

What is an important oral hygiene practice for hemophiliacs?

Using a toothbrush with medium texture bristles Signup and view all the answers

What percentage of the population in certain regions is affected by hemophilia?

15-30% Signup and view all the answers

What is the current status of a cure for hemophilia?

There is no cure available Signup and view all the answers

What is the main treatment for hemophilia?

Protein Replacement Therapy Signup and view all the answers

What is the purpose of Protein Replacement Therapy for hemophilia?

To replace missing clotting factors Signup and view all the answers

Why may people with hemophilia C require treatment?

Because they are going to have surgery or certain dental treatment Signup and view all the answers

What is a common oral manifestation of hemophilia?

Excessive gingival bleeding Signup and view all the answers

Why may people with hemophilia experience joint pain and limited mouth opening?

Due to TMJ bleeds Signup and view all the answers

What is a key aspect of life enhancement for people with hemophilia?

All of the above Signup and view all the answers

Study Notes

Hemophilia

A rare, genetic blood disorder where blood doesn't clot and bleeding doesn't slow down or stop due to low clotting factors.
Three main types: Hemophilia A, Hemophilia B, and Hemophilia C, each caused by a deficiency of a specific clotting factor (factor VIII, factor IX, and factor XI, respectively).

Inheritance Patterns

Hemophilia A and B are X-linked recessive diseases, where males are more affected because they only need one mutated gene to show symptoms.
Hemophilia C is an autosomal recessive disease, where both males and females are equally affected and need two mutated genes to show symptoms.

Symptoms

Prolonged bleeding, sudden bloody noses, bleeding of the mouth and gums, oozing after tooth extractions, bleeding in the urine or stool, easy bruising, joint bleeding (hemarthrosis), and bleeding into the brain.
Severity of symptoms depends on the severity of the mutation.

Severity and Clotting Factor Levels

Hemophilia can be categorized as mild, moderate, or severe based on the amount of clotting factors in the blood.
Clotting factor levels:
- Severe hemophilia: less than 1% of normal clotting factors.
- Moderate hemophilia: 1% to 5% of normal clotting factors.
- Mild hemophilia: 5% to 30% of normal clotting factors.

Diagnosis

Healthcare provider will start with a complete history and physical examination.
Tests may include:
- Complete blood count (CBC).
- Prothrombin time test (PT).
- Specific clotting factor test.
- Genetic or DNA testing.

Epidemiology

Hemophilia affects patients worldwide, with varying prevalence rates in different regions.

Treatment

Currently, there is no cure for hemophilia.
Main treatment is Protein Replacement Therapy, which involves infusing clotting factor concentrates (factor VIII for Hemophilia A or factor IX for Hemophilia B) into a vein to replace missing or low clotting factors.
Additional treatments may include fresh frozen plasma and medications to prevent clotting factor breakdown.

Oral Manifestations and Dental Management

Symptoms in the oral cavity include excessive gingival bleeding, high rate of infections, joint bleeds, and orofacial pain.
Dental management involves:
- Obtaining medical history and communicating with hematologists.
- Avoiding certain pain medications that can exacerbate bleeding.
- Intensive treatments, such as deep cleaning and tartar removal, should be carried out in hospitals or community dental services.
- Maintaining good oral health through regular dental visits, brushing, and interdental cleaning aids.

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Description

Hemophilia is a genetic blood disorder where the blood doesn't clot properly, leading to slow or uncontrolled bleeding. This occurs due to low levels of clotting factors in the blood.