Fibrodysplasia Ossificans Progressiva FOP | ACVR1(c.617G>A; R206H)
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FOP Treatment Guidelines – Medicinska riktlinjer för FOP.

På sidan 48-53 finns en mall med mediciner hur dom bör tas och varför. Sidan 54-55 en sammanfattning av de viktigaste punkterna. Rådgivning för läkare/patienter och anhöriga: Vid minsta tveksamhet och vid akutsituation kontakta våra specialister och rådgivare i Philadelphia USA eller konsultera IPC representant i Sverige. För dig som har FOP eller är förälder rekommenderar vi att börja med att skriva ut två exemplar av sidorna 50-55 i treatment guidelines och sedan går vidare och läser ur en handbok för familjer.

INNEHÅLLSFÖRTECKNING FOP MEDICAL TREATMENT GUIDELINES.

Ladda ner FOP Treatment Guidelines, uppdaterad version 18/10-13.

INNEHÅLL:

  • Page 5. ABSTRACT
  • Page 6. I. THE CLINICAL AND BASIC SCIENCE BACKGROUND OF FOP.
    Page6.
    A. Introduction
    B. Classic Clinical Features of FOP
    C. Other Skeletal Anomalies in FOP
    Page 7.
    D. Radiographic Features of FOP
    E. Histopathology of FOP Lesions
    Page 8.
    F. Laboratory Findings in FOP
    G. The Immune System & FOP
    H. Misdiagnosis of FOP
    Page 9.
    I. Epidemiologic, Genetic & Environmental Factors in FOP
    J. FOP & the BMP Signaling Pathway
    K. The FOP Gene
    Page 10.
    L. Structural and Functional Consequences of the FOP Mutation
    M. Genetic Testing & FOP
    N. Animal Models of FOP
    Page 11.
    O. Challenges of Therapeutic Assessment in FOP.
  • Page 12. II. THE PATHOLOGIC & PATHOLOGIC-BASED TREATMENT OF FOP.
    Page 12.
    A. Introduction
    B. Corticosteroids
    Page 13.
    C. Mast Cell Inhibitors
    Page 15.
    D. Cyclo-oxygenase-2 Inhibitors & NSAIDs
    Page 17.
    E. Aminobisphosphonates
    Page 23.
    F. Acute & Chronic Pain Management in FOP
    Page 24.
    G. Muscle Relaxants
    H. Chemotherapy Agents & Radiation Therapy
    I. Bone Marrow Transplantation
    Page 25.
    J. Rosiglitazone
    Page 27.
    K. Retinoic Acid Receptors Agonists
    Page 29.
    L. Miscellaneous Agents
    M. Targeting ACVR1/ALK2; Definitive Targets for Therapy. 
  • Page 31. III. SPECIAL MEDICAL CONSIDERATIONS.
    Page 31.
    A. Introduction
    B. Injury Prevention in FOP
    Page 32.
    C. Spinal Deformity in FOP
    Page 33.
    D. Cardiopulmonary Function in FOP
    Page 34.
    E. Immunizations & FOP
    Page 38.
    F. Limb Swelling & FOP
    G. Pressure Sores in FOP
    Page 39.
    H. Fractures in FOP
    I. Preventive Oral Healthcare in FOP
    Page 40.
    J. Dental Anesthesia in FOP
    K. General Anesthesia in FOP
    Page 42.
    L. Orthodontics in FOP
    M. Submandibular Flare-ups of FOP
    Page 44.
    N. Hearing Impairment in FOP
    O. Kidney Stones & FOP
    P. Rehabilitation Issues in FOP
    Page 46.
    Q. Pregnancy Issues in FOP.

SIDOR ATT SKRIVA UT. Kortfattad medicinsk information om hur man förhåller sig till en patient med FOP. Mediciner som används idag och möjliga typer av mediciner för framtiden:

  • Page 48. IV. CURRENT TREATMENT CONSIDERATIONS
  • Page 50. V. CLASSES OF MEDICATIONS (TABLE 1)
  • Page 50. i. Class I Medications
  • Page 52. ii Class II Medications
  • Page 53. iii. Class III Medications
  • Page 54. VI. SUMMARY OF KEY PRACTICE POINTS
  • Page 56. VII. CONCLUSIONS
  • Page 57. VIII. ACKNOWLEDGEMENTS
  • Page 58. FIGURE 1: SYMPTOMATIC TREATMENT SCHEMA IN FIBRODYSPLASIA OSSIFICANS PROGRESSIVA (FOP)
  • Page 59. IX. REFERENCES

Page 59.
I. The Clinical and Basic Science Background of FOP
A. Introduction
B. Classic Clinical Features of FOP
Page 60.
C. Other Skeletal Anomalies in FOP
D. Radiographic Features of FOP
E. Histopathology of FOP Lesions
Page 61.
F. Laboratory Findings in FOP
Page 62.
G. The Immune System
H. Misdiagnosis in FOP
I. Epidemiologic, Genetic & Environmental Factors in FOP
Page 63.
J. FOP & The BMP Signaling Pathway
Page 64.
K. The FOP Gene
Page 65.
L. Structural and Functional Consequences of the FOP Mutation
Page 66.
M. Genetic Testing in FOP
N. Animal Models in FOP
Page 67.
O. Challenges of Therapeutic Assessment in FOP

  • Page 69. II. THE PATHOLOGIC AND PATHOPHYSIOLOGIC–BASED TREATMENT OF FOP

Page 69.
A. Introduction
B. Corticosteroids
C. Mast Cell Inhibitors
Page 70.
D. Cyclo-oxygenase 2 inhibitors & NSAIDS
Page 71.
E. Aminobisphosphonates
Page 73.
F. Acute & Chronic Pain Management in FOP
Page 73.
G. Muscle Relaxants
Page 74.
H. Chemotherapy Agents & Radiation Therapy
I. Bone Marrow Transplantation
J. Rosiglitazone
Page 75.
K. Retinoic Acid Receptor Agonists
L. Miscellaneous Agents
Page 76.
M. Targeting ACVR1/ALK2: Definitive Targets for Therapy

  • Page 77. III. SPECIAL MEDICAL CONSIDERATIONS.
    Page 77.

    A. Introduction
    B. Injury Prevention in FOP
    C. Spinal Deformity in FOP
    D. Cardiopulmonary Function in FOP
    E. Immunizations & FOP
    Page 78.
    F. Limb Swelling & FOP
    Page 79.
    G. Pressures Sores in FOP
    H. Fractures in FOP
    I. Preventive Oral Healthcare in FOP
    Page 80.
    J. Dental Anesthesia in FOP
    K. General Anesthesia in FOP
    L. Orthodontics & FOP
    M. Submandibular Flare-ups in FOP
    N. Hearing Impairment in FOP
    O. Kidney Stones & FOP
    Page 81.
    P. Rehabilitation Issues in FOP
    Q. Pregnancy Issues in FOP
  • Page 82. IV. SUMMARY OF KEY PRACTICE POINTS
  • Page 83. V. CONCLUSIONS
  • Page 84. X. THE INTERNATIONAL CLINICAL CONSORTIUM ON FIBRODYSPLASIA OSSIFICANS PROGRESSIVA.

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