Thursday, June 23, 2011

Haemophilia type A - Clinical signs

In terms of clinical diagnosis is rare in newborn. The increased incidence of disease is 1-5 years age range. A percentage between 30-40% of cases are diagnosed in adults.Diagnosis may be issued and fortuitous, due to bleeding injuries.Thus, 6% of cases are diagnosed in newborns, 8% sugar, 32% in the range of 1-2-year life and 40% over 2 years old. They appear to cut the umbilical cord tissue is increased because trombelastina, appear to be circumcised. The newborn is diagnosed easier when there a family history of haemophilia.Features installed bleeding: is bleeding caused, occurring after minor trauma, has a duration of bleeding increased outward, have headquarters deep in the cavities of capsules.
The disease has a chronic evolution, with periods of calm.The absence of lesions petechiae differential diagnosis helps immune thrombocytopenic purpura, because it affected tissue hemostasis.Highlights clinical Tabolul intramuscular hematoma present superficially in the buttocks, deltoid, pectoral and deep in the psoas muscle, retroperitoneal a rate of 10-15%.
Functional prognosis is negative when there is compression of the radial nerve, sciatica, vascular packages, sense organs: eye, ear known of internal functional.
This haemarthroses as a manifestation in haemophilia in the evolution of chronic occurrence of ankylosis, especially May Positions vicious. A percentage of 89% of the cases have been affected haemarthrosis: knee, malleolus, elbows, punches, hip. Of these 50% have a low severity, 30% are of moderate severity and 20% higher.
After the place of appearance, bleeding in the calsifica: visceral bleeding, bleeding in open-wounds 50%, mouth bleeding, bruising, cutting, epistaxis (mucosal), the latter are abundant and have a prolonged time can cause collapse and anemia.Visceral bleeding include severe hematuria, repetitive, at a rate of 20%, GI bleeding, bleeding of the sense organs: eyes, inner ear, brain hemorrhage, postoperative bleeding and medical interventions.
Clinical forms of hemophilia are: familial form, which is found in 60% and sporadic form in 40%.
Depending on the severity of the factor VIII there are many forms of disease:- Severe forms in less than 1% of cases;- Forms a mean rate of 1-4%;- Mild in the rate of 4-25%;- Latent form in a higher percentage of 25%.

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