Haemophilic arthritis


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Haemophilic arthritis

  1. 1. About The AuthorDr Manoj R. kandoi is the founder president of “Institute of Arthritis Care & Prevention”an NGO involved in the field of patient education regarding arthritis. Besides providingliterature to patient & conducting symposiums, the institute is also engaged in creatingpatients “Self Help Group” at every district level. The institute also conducts a certificatecourse for healthcare professionals & provide fellowship to experts in the field ofarthritis.The author has many publications to his credit in various journals. He has also written a book “ The Basics Of Arthritis” for healthcare professionals.The author can be contacted at:Dr manoj R. kandoiC-202/203 Navare ArcadeShiv Mandir Road, Opposite Dena BankShiv mandir Road, Opposite Dena bankShivaji Chawk, Ambarnath(E) Dist: Thane Pin:421501State: Maharashtra Ph: (0251)2602404 Country: IndiaMembership Application forms of the IACR for patients & healthcare professionalscan be obtained from.Institute of Arthritis Care & PreventionC/o Ashirwad HospitalAlmas mension, SVP Road, New Colony,Ambarnath(W) Pin:421501 Dist: ThaneState: Maharashtra Country: IndiaPh: (0251) 2681457 Fax: (0251)2680020Mobile ;9822031683Email: drkandoi@yahoo.co.inPreface:Studies have shown that people who are well informed & participate actively intheir own care experience less pain & make fewer visits to the doctor than do other people with arthritis. Unfortunately in India & many third world countries we do nothave patient education & arthritis self management programs as well as support groups.This is an attempt to give a brief account of various arthritis, their prevention & selfmanagement methods which can serve as useful guide to the patients of arthritis.It would be gratifying if the sufferers of the disease knew most of what is given in thebook.AcknowledgementI am thankful to Dr (Mrs) Sangita Kandoi for her immense help in proofreading & for herinvaluable suggestions. The help rendered by Nisha Jaiswal is probably unrivalled.Thanks also to vidya, praveen, rizwana and parvati for their continous supportthroughout the making of the book. The author is grateful to his family for the constantinspiration they offered. The author alone is responsible for the shortcoming in this pieceof work. He welcomes suggestions for improvement from the readers.
  2. 2. Haemophilic ArthritisIntroduction:Haemephilia include a group of disease affecting males but transmitted by female andcharacterized by prolonged coagulation and a life long tendency to excessive bleeding.Types: 1. Haomophilia A (factor VIII deficiency) 2. Haemophilia B (Christmas disease factor IX deficiency) 3. Von willebrallds disease: There is abnormality of platelet function.Process of blood caugulation: Intrinsic pathway Extrinsic pathway Factor XII Factor IX Tissue factor VII Factor XI Factor VIII (tissue thromboplastin) activated on activated on foregin platelet Surface surface Factor X Factor V PF3 Common pathway Ca Prothrombin Thrombin Fibrinogen FibrinGrades of factor VIII deficiency:Grade I: < 1 % severe bleedingGrade II: < 5% gross bleeding with minor traumaGrade III: < 5 -25% severe bleeding after trauma or surgeryGrade IV: < 25 -50% bleeding after major trauma or surgeryPathophysiology of haemophilic arthropathy:
  3. 3. Haemorrhage in the joint (Spontaneous or following trauma) Blood remain fluid in the joint due to lack of caugulation Plasma absorbed RBC phagocytosis Iron deposition in synovial Tissue and articular cartilage Chronic inflammation with Synovial proliferation and Pannus formationRheumatological manifestations: 1. Haemaphilic arthropathy 2. Intramuscular or soft tissue haemorrhege (may cause pseudotumor or compartment syndrome) 3. Septic arthritis. a. Haemophilic arthropathy:  Acute hemarthrosis: It usually starts when child begins to walk characterized by pain, increased temperature swelling and restricted motion. Severity and recurrences is dependent on factor levels (if there are 75% normal, there is a lesser tendency towards haemarrhage).  Sub acute haemophilic arthropathy: It usually follows repeated episodes of joint bleeding characterized by features suggestive of chronic synovitis, It is accompanied with muscle laxity and ligament laxity. Polyarticular involvement is uncommon.  Chronic haemophilic arthropathy: The main feature are of joint deformity, fibrous ankylosis and osteophyte over growth.Extra skeletal manifestations: 1. Sub cutaneous bleeding with tendency to bruise easily. 2. Mucous membrane and internal bleeding  Haematuria  Epistaxis  Bleeding into brain or spinal cord  Mouth, gums, lips and tongue bleeding.Diagnosis: 1. Family history: Especially suggesting a sex linked disorders. 2. Preliminary cougulation testing: Tests include APTT, PT, thrombin clotting time (TCT) and bleeding time. In haemophilia A & B, APTT is prolonged, if bleeding time is prolonged one should consider diagnosis of Von willebrands disease. 3. Specific factor essay: To differentiate between haemophilia A & B.
  4. 4. Radiological staging of haemophilic arthritis:Stage I: Soft tissue swellingStage II:  Soft tissue swelling  Osteopenia of epiphyses  Joint space maintained  Subchondral cysts are present  Squaring of the patella  Intercondylar notch femur and trochlear notch of ulna widenedStage III:  Disorganization of joint  ? Ankylosis  Large subchondral cystsManagement of haemophilia - A: 1. Local haemostatic agents: Such as thrombin or gelfoam if bleeding site is accessible. 2. Factor VIII replacement: a. Cryoprecipitate: it is stored frozen b. Freeze dried factor VIII concentrates -the advantages being ability to store in domestic refrigerator and higher purity. 3. Non blood products such as synthetic vasopressin may be used in mild to moderatecases. 4. General care: It includes a. Prophylactic immunization b. Correction of anaemia if present c. To avoid bleeding including drugs such as aspirin. 5. Treatment of acute haemarthritis: a. Cold application b. Analgesics c. Joint immobilization d. Replacement of coagulation factor e. Gradual physiotherapy 6. Treatment of chronic arthropathy: a. Prophylactic factor replacement to prevent recurrences of bleeding. b. Rest c. Gradual physiotherapy d. Intraarticular gluco corticoids to reduce symptoms and recurrent hemathrosis. e. Synoviectomy for chronic synovitis unresponsive to conservative therapy. f. For recent contractures: Plaster splinting, dynamic traction, exercises. g. Postsubluxation of tibia: dynamic traction h. Painful unstable joints: Orthotic splintage i. Supracondylar osteotomy for severe FFD knee j. TA lengthening for TA contractures k. Total joint replacement in cases with advanced joint destruction. l. Arthrodesis in severe destructive arthritis
  5. 5. m. ORIF for fracture nonunion.Rheumatologic manifestations of sickle cell anaemia: 1. Bone infarction 2. Septic arthritis 3. Osteomyelitis 4. Hemarthrosis 5. Hyperuricemia and gout 6. Vascular necrosis of bone 7. Hand foot syndrome 8. Muscle necrosis Arthritis With Leukemia: Acute monoarticular mimicking acute rheumatic fever may occur, particularly in acute monocytic leukemia.