Haemophilia type A Clinical signs

Haemophilia type A
Clinical signs
In terms of clinical diagnosis is rare in newborn. The increased incidence of disease is between 1-5 years of age. A percentage between 30-40% of cases are diagnosed in adults. Diagnosis may be issued and fortuitously, as a result of bleeding injuries. Thus, 6% of cases are diagnosed in the newborn, infant 8%, 32% in a range of life-2 years and 40% over 2 years old. They do not appear to cut the umbilical cord tissue is increased because trombelastina, occurring in circumcision. The newborn is diagnosed more easily when there is a family history of haemophilia. Features installed hemorrhage: a hemorrhage caused, occurring after minor trauma, shows an increased duration of bleeding externalized; have registered deeply in the cavity of capsules.
The disease has a chronic evolution, with periods of calm. The absence of petechial lesions differential diagnosis helps immune thrombocytopenic purpura, because it affected tissue hemostasis. Intramuscular hematoma show similar clinical Tabolul superficial level buttocks, deltoid, pectoralis, deep in the psoas muscle, retroperitoneal a rate of 10-15%.
Functional prognosis is negative when there is compression of the radial nerve, the sciatic vascular package, sense organs: eye, ear internal functional rebound.
This event in haemophilia haemarthroses that determine the occurrence of ankylosis in chronic evolution, especially in May vicious position. A percentage of 89% of the cases shows haemarthrosis were affected: knee, malleolus, elbows, punches, hip. Of these 50% shows a low severity, 30% are 20% of medium gravity and high gravity.
After the place of occurrence, the bleeding is calsifica: visceral bleeding, bleeding in open-wounds 50%, mouth bleeding, bruises, cut, epistaxis (mucosal), the latter are abundant and have a prolonged time can lead to collapse and anemia. Visceral Bleeding hematuria include severe repetitive, at a rate of 20%, gastrointestinal bleeding, bleeding of the sense organs: eye, inner ear, brain hemorrhage, bleeding after surgery and medical interventions.
Clinical forms of hemophilia are: family form that is found in 60% and sporadic form in 40%.
Depending on the severity of factor VIII, there are several forms of disease: - Severe forms in less than 1% of cases; - Forms a percentage average of 1-4%; - Mild in the rate of 4-25%; - Latent form in a higher percentage of 25%.