Rheumatology Book

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Amyloidosis

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  1. Pathophysiology
    1. Disturbed endogenous protein metabolism
      1. Primary
      2. Secondary to chronic tissue breakdown
    2. Organ dysfunction arises from deposition
  2. Symptoms
    1. Asthenia
    2. Weight loss
    3. Paresthesias
    4. Organ specific symptoms
  3. Signs
    1. Organ specific signs
    2. Macroglossia
    3. Hypertension
    4. Lymphadenopathy
    5. Hepatomegaly
    6. Splenomegaly
    7. Purpura
    8. Nephrotic Syndrome
    9. Edema
    10. Joint pain, muscle pain
    11. Serous cavity fluid
  4. Labs
    1. Biopsy under Congo red or thioflavine-T staining
      1. Exam of Gingiva, rectum, involved tissues
      2. Green birefringence in polarized light
    2. Serum Immunoglobulins altered

Amyloidosis (C0002726)

Definition (MSH)A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.
Definition (CSP)any disease manifested by the pathogenic accumulation of amyloid in organs and tissues.
Definition (NCI)A group of diseases in which protein is deposited in specific organs (localized amyloidosis) or throughout the body (systemic amyloidosis). Amyloidosis may be either primary (plasma cell neoplasm) or secondary (caused by a long standing infection or another disease or some types of cancer). Generally, primary amyloidosis affects the nerves, skin, tongue, joints, heart, and liver; secondary amyloidosis often affects the spleen, kidneys, liver, and adrenal glands.
ConceptsDisease or Syndrome (T047)
ICD9277.3, 277.30, 277.30
MSHD000686
Englishamyloid disease, Amyloidoses, Amyloidosis
Spanishamiloidosis
Parent ConceptsFormer or Present Systemic Disease (C0150840), Concomitant or past diseases (C0150941), Rheumatic illness with extraarticular and/or constitutional features (fever, rash, pleurisy,etc) OR Systemic illness with rheumatic manifestation (C0221589), Metabolic Diseases (C0025517), Amyloidosis (C0002726), Pathologic Processes (C0030660), Protein Disorders NEC (C0549551), Other disorders of metabolism (C0268329), cancer-related problem/condition (C0280950), Lymphoproliferative Disorders (C0024314), Inborn Errors of Metabolism (C0025521), Paraproteinemias (C0030489), Degenerative disorder (C1285162), Duplicate concept (C1274013)
SourcesAIR, AOD, COSTAR, CSP, CST, ICD9CM, LCH, MSH, MTH, MTHICD9, NCI, NDFRT, PDQ, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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