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33 Cards in this Set
- Front
- Back
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Porphyrinogen are?
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precursors to the porphyrin compounds and are colorless compounds
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Major sites of synthesis for heme is in the?
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Liver and bone marrow
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Mature RBC's lack _____ therefore can not make_____
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Mitochondria, Heme
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Heme is formed by the insertion of Fe into protoporphyrin IX by ______.
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Ferrochelatase
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Porphyrias are....
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Genetic disorders of porphyrin synthesis
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Most porphyrian are AR but ______________ is AD
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Congenital erythropoetic porphyria
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PORPHYRINS are...
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colored compounds
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TYPE III ARE ?
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normal
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Type I are?
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abnormal
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Acute Intermittent Porphyria (AIP) there is a deficiency in?
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HMB synthetase
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What accumulates in the blood with somebody with Acute intermittent porphyria?
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Porphobilinogen and ALA
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What are the main symptoms of intermittent porphyria?
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Abdominal pain is main symptom
& mental disturbances like anxiety, depression |
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What is seen in acute attacks of intermittent porphyria?
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Increase in ALA and PBG in urine/blood in acute attack
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What is the treatment of Intermittent porphyria?
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Treat: Avoid drugs, give IV heme
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Congenital Erythropoietic Porphyria (CEP)
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There is deficient URO synthase activity
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Major Clinical Symptoms of Congenital Erythropoetic Porphyria?
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Cutaneous photosensitivity; skin damage
Reddish urine (diaper) and teeth; anemia Accumulated porphyrinogens oxidized to porphyrins |
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Treatment of Congenital Eruthropoetic Porphyria?
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avoid sun, blood transfusions may help
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What enzymes are inhibited by Lead?
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ALA-Dehydratase and Ferrochelatase
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Where does the degradation of heme take place?
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Liver and Spleen
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What sugar motiety is attached to bilirubin in Zone III of the liver?
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Gluconate
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What is the final breakdown product of purine degradation?
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Uric acid
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What is the lesch-Nyhan syndrome?
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Lacks Nucleotide Salvage (purine)
Purine slavage problem owing to absence of HGPRTase. which results in excess uric acid production bc there is increase in PRPP which stimulates PRPP Synthetase |
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What are the clinical findings of someone with Lesch-Nyhans Syndrome:
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Retardation, Gout, Afrssion and choreathetosis
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Adenosine Deaminase deficiency causes?
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SCIDS (Severe Combined Immunodeficiency disease
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What joint is effected most commonly is somebody with Gout?
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1st metatarsophalngeal Joint--> Erosive Arthritis
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What is the most common cause of Gout?
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Under excretion of Uric acid
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What are the crystals called that are found in gout?
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Monosodium Urate crystals
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Uric acid crystals are?
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Negative apple colored Birefringence
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Birefringence means what?
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or double refraction, is the decomposition of a ray of light into two rays (the ordinary ray and the extraordinary ray) when it passes through certain types of material,
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What food should one avoid if they have gout?
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Seafoods, Organ meats(liver), meats, fish, poultry, tofu, alcohol, avoid diuretics
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What population of people are at the highest risk for getting Gout?
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African American men 40-50y/o that are obese
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What are the monosodium uric acid crystals in the joints called?
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Tophi
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What is the treatment for Gout?
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Change diet and Allopurinol
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