Hansen's disease and hemophilia
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Hemophilia is a rare genetic bleeding disorder caused by a deficiency in clotting factors VIII or IX. Those with severe hemophilia can experience repeated spontaneous internal bleeding episodes, leading to joint damage, muscle atrophy, and loss of mobility. Proper treatment with clotting factor concentrates can prevent or treat bleeding episodes. However, around 30% of severe hemophilia patients develop inhibitors against treatment. Effective management requires early prophylactic treatment, education, coordinated multidisciplinary care, and continued research.
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This document summarizes coagulation disorders and disseminated intravascular coagulation (DIC). It describes four categories of bleeding disorders: those due to vascular abnormalities, platelet abnormalities, coagulation factor deficiencies, and a combination of these. It provides details on hemophilia A and B, which are deficiencies in coagulation factors VIII and IX, respectively. It covers the incidence, clinical symptoms, laboratory testing, and treatment of coagulation factor deficiencies. It then defines DIC as a thrombohemorrhagic disorder caused by excessive coagulation activation, and lists conditions associated with DIC and describes its pathogenesis, pathophysiology, clinical manifestations, laboratory diagnosis, and treatment.
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• It is a complex natural physiological response.
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Rheumatoid arthritis is an autoimmune disease that causes inflammation of the joints. It commonly affects the small joints in the hands and feet first. Key features include persistent joint swelling and stiffness that improves with movement. It can lead to long-term joint damage, deformity and disability if left untreated. Treatment involves medications like NSAIDs for pain relief and disease-modifying antirheumatic drugs (DMARDs) like methotrexate to reduce symptoms and prevent further joint damage. DMARDs work best when started early in the disease course. The goals of treatment are to relieve pain, prevent disability and educate patients about managing their condition.
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- 2. * * Hemophilia is a genetically transmitted disorder of deficiency of circulating blood clotting factor. Hemophilia A and B are rare disorders which result from a deficiency of clotting factor VIII&IX. * repeated spontaneous and post-traumatic internal bleeding episodes * muscle atrophy, synovitis, chronic hemophilic arthropathy, joint and muscle contractures, Limb length inequality and loss of proprioception. * significant impact on the quality of life
- 3. *The disabling joint bleeds can be prevented or treated with the coagulation factor concentrate that the haemophilia patient lacks, the so-called factor concentrate. *About 30 per cent of those who have a severe form of haemophilia develop antibodies (inhibitors), which reduce the effect of the treatment
- 4. * • Prophylactic treatment from an early age. • Treatment at home • Centralised haemophilia care • A chain of care that functions: the haemophilia centre – the patient’s local hospital unit – the patient • Education and information campaigns • National and international co-operation around research and development • A continuous evaluation and quality control of the care process
- 6. * Overview : The bleeding score summarizes the number and type of hemarthroses experienced by a hemophiliac. This can be monitored over time to follow disease severity and the effectiveness of interventions. Steps: (1) determine the number of hemarthroses per year (2) determine how many of these are minor and how many are major episodes. Criteria for minor hemarthrosis: all of the following: (1) minimal to mild pain (2) no to minimal swelling (3) minimal restriction of motion (4) resolution within 24 hours of treatment Criteria for major hemarthrosis: one or more of the following: (1) significant pain (2) effusion (3) limitation in motion (4) failure to respond within 24 hours of treatment Major Hemarthroses Minor Hemarthroses Bleeding Score 0 AND 0 0 0 AND 1 - 3 1 1 − 2 OR 4 - 6 2 >= 3 OR >= 7 3 Overview : A clinical score for hemophilic arthropathy can be based on the physical examination. This was developed by the Orthopedic Advisory Committee of the World Federation of Hemophilia. Parameters: (1) swelling (2) muscle atrophy (3) axial deformity (4) crepitus on motion (5) range of motion (6) flexion contracture (7) instability Parameter Finding Points swelling none 0 present 2 present with chronic synovitis 2S muscle atrophy none 0 minimal (< 1 cm decrease in circumference) 0 present 1 axial deformity (knee or ankle only) normal 0 mild varus or valgus deformity (see below) 1 moderate to marked varus or valgus deformity (see below) 2 crepitus on motion none 0 present 1 range of motion 0-9% loss in full range of motion 0 10-33.33% loss in full range 1
- 7. * *FISH *HAL *Gilbert joint score *Euro HQoL scale * CHO‐KLAT and the Haemo‐QoL for children
- 8. * *Leprosy, a chronic infectious disease affecting mainly the skin and the nerves, was among the first infection to be associated with a specific causative organism – Mycobacterium leprae. Pathological types *Tuberculoid type (non-infectious) *Lepromatous type (infectious) *Indeterminate type *Borderline type – Tuberculoid ,Lepromatous *Incubation Period: 2-10 years
- 9. * *Skin lesion consistent with leprosy and with definite sensory loss *Positive skin smears *Unknown patophysiology
- 10. *Patients with fewer bacilli, the paucibacillary (PB) group were recommended to be treated with 2 drugs given for only 6 months, while for the remaining , called the multibacillary (MB) group, 3 drug combination for 2 years or till smear negativity, which ever was later, was advocated. *Rifampicin, Ofloxacin + Monocycline, Dapsone and Clofazimine
- 11. * * Blindness or glaucoma. * Disfiguration of the face (including permanent swelling, bumps, and lumps). * Erectile dysfunction and infertility in men. * Kidney failure. * Muscle weakness that leads to claw-like hands or an inability to flex the feet. * Permanent damage to the inside of the nose, which can lead to nosebleeds and a chronic, stuffy nose. * Permanent damage to the nerves outside the brain and spinal cord, including those in the arms, legs, and feet.
- 12. * * Trophic ulcers * Foot drop * Partial and Total claw hand * Joint stiffness * Post – tendon transfer impairments * Post – amputation impairments * Psychological impairments (Social stigma) * Economic/Social impairments * Balance impairments * Posture & movement impairments * Mobility impairments * Endurance impairments