| ID | 159 |
|---|---|
| Name | HEMOPHILIA (Factor VIII Deficiency) |
| Cause | |
| Signs Symptoms | |
| Diagnosis | |
| Investigations | Investigations: 1. Blood- R/E is normal, rarely there may be anemia when the bleeding is profuse. 2 B.T is normal but coagulation time is markedly prolonged. 3. Factor VIII level of blood is diminished. Normal level 0.50-1.50 i.u/ml. Below 0.10i.u/ml is pathological. |
| Management | |
| Introduction | Hemophilia is a heriditary disorder characterised by decreased anti-hemophilic factor (factor VIII) thus leading to prolong bleeding tendency. It is inherited as a sex linked recessive character-females carry the gene of the disease while the males suffer |
| History | |
| Etiology | |
| Clinical Features | Clinical features: The most common manifestation is hemorrhage into joints. They usually develop spontaneously and are associated with pains, which may be severe, with swelling, warmth and muscle spasm. The knee joints are most frequently affected, but ankles, hips, shoulders, elbows and wrists are also common sites. Hemorrhage into musculature and soft tissues also occur. Intra-abdominal bleeding and hemorrhage from the gastrointestinal tract is relatively common. Superficial trauma gives rise to uncontrolled bleeding. Intracranial hemorrhage may occur. Tooth extraction will also promote prolonged bleeding. Severity of hemophilia: Severe: Spontaneous hemarthosis & muscle hematoma Moderate: Mild trauma or surgery cause hematoma. Mild: Major surgery or injury result in excess bleeding |
| Preventions | |
| Treatment | Treatment: 1. General- Patient should be advised to avoid sports, swimming, cycling etc. Regular dental examination and careful dental hygiene is essential. Planned preoperative and post-operative care in surgical procedure. 2. Treatment of bleeding: a. Bed rest is essential. b. Fresh blood transfusion should be given. c. Local hemostasis is most valuable e.g. pressure, immobilisation, local application of adrenaline, topical thrombin etc. d. Anti hemophilic globulin can be given i.v, low or high potency factor VIII concentrates may also be given. 3. Physiotherapy and orthopedic correction may be required |
| Complications | |
| Prognosis | |
| Types | |
| Classification | |
| Observation | |
| Pathology |
© Pakistan Drug Directory. All Rights Reserved.
Designed By: Pakistan Drug Directory Team