Gout Flashcards
Inheritance of LNS/Juvenile gout
X-linked recessive
Mutation in LNS/juvenile gout
Deficiency of HGPRT enzyme
3 major hallmarks of LNS/juvenile gout
Hyperuricemia
Cognitive and behavioral disturbances (self-mutilation)
Neurological dysfunction
General causes of increased urate production causing hyperuricemia
Diet
Increased endogenous production
Accelerated purine nucleotide degradation
Hemolytic, pernicious, or EPO anemias
Obesity
Causes of increased endogenous production of urate
Increased activity of PRPP synthetase
Decreased activity of HGPRT
General causes of decreased urate excretion leading to hyperuricemia
Renal insufficiency
Acidosis
Toxemia of pregnancy
Diuretics and low dose salicylates
Crystal induced inflammatory arthritis marked by transient attacks
Gout
Initiated by monosodium urate crystals deposited within and around joints leading to cytokine production and recruitment of neutrophils
Gout
Podagra
Acute gout causing swelling, erythema, pain, and tenderness of the metatarsophalangeal joint of the first toe
Joint aspirations with needle-shaped negatively birefringent crystals in synovial fluid
Gout
Repetitive precipitation or urate crystals during acute attacks where urates coat the surface of and deposit within the synovium
Chronic tophaceous arthritis
Pathognomonic hallmark of gout
Accumulation of tophi in extra-articular sites
Tophi
Aggregates of urates rimmed with macrophages, lymphocytes, and foreign body giant cells seen in gout
Another name for pseudogout
Calcium pyrophosphate deposition disease (CPPD)
Deposition of calcium pyrophosphate in tissues, often involving the knee
Pseudogout
