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29 Cards in this Set
- Front
- Back
How is copper bound and eliminated
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Ceruloplasmin (>95% of plasma copper)
5-10% of serum copper is bound to albumin Only elimination is biliary excretion |
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Copper and redox
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Copper inhibits sulfydryl groups on enzymes
-GSH reductase Generates oxidative stress -Cu+ catalyzes formation of OH radical |
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DMPS and Copper
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CMPS worsens Hemolysis
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Cu and Erythrocytes
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Cu++ is reduced to Cu+
Generates Hydroxyl radical -Fe++ to Fe+++ methemoglobin -RBC membrane: Hemolysis |
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Cu and Liver
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Free intracellular Cu ions generate OH
-lipid peroxication -mitochondrial damage -DNA adducts -Cetnrilobular necrosis |
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Kidney and Cu
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Bioaccumulates Cu
-Oxidant stress -ATN -Hemoglobinuria (from hemolysis) |
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Cu and CNS
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Carrier mediated albumin bound Cu
Wilson Disease |
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Cu Salt (copper sulfate) GI
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Blue vomitus
Ulceration Hemorrhage |
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Copper Sulfate Hematologic
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Hemolysis (biphasic)
Methemoglobin |
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Renal and copper sulfate
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ATN
Hemoglobinuria Methemoglobinuria |
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Copper Sulfate and Hepatotoxicity
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Jaundice
Centrolobular necrosis |
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Copper sulfate and Skin
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Green skin with irritation
Green hue hair shafts |
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Copper and Labs
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Methemoglobin
(copper salts radiolucent) Serum Copper -for confirmation or investigation |
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serum [Cu]
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Whole blood matrix better correlation with clinical findings
There is relationship between whole blood Cu and severity of poisoning |
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Cu Chelation
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BAL
Oral D-Peniciallamine Concurrently or after BAL (prevents hemolysis in Wilson disease) |
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Metal Fume Fever and Cu
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Severe flu like illness
Self-limited, resolves within 24 hours No long term sequellae |
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Metal Fume Fever Labs
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Leukocytosis
Normal CXR |
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Chronic Ocular Cu exposure
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Chalcosis Lentis
Brown discoloration of lens or cornea |
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Vineyard Sprayers Lung
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Portugese vineyard workers applying Bordeaux solution
1-2% copper sulfate associated with hydrated lyme Interstitial pulm fibrosis, histiocytic granulomas with Cu Lung Adenocarcinomas, hepatic angiocarcinoma, micronodular cirrhosis |
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Wilson Disease
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Cu Accumulation
Autosomal Recessive Liver capacity for Cu storage exhausted |
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Wilson CNS
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Cu not taken up by neurons
Increased extracellular Cu causes free radical production Lenticular nucleus in Basal ganglia |
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Wilson Disease clinical
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Liver disease most common presentation
8-18 yrs Transaminasemia, hepatitis, FHF, cirrhosis Chronic Liver disease may precede neurologic symptoms by years Kayser Fleischer Rings -Golden brown deposit at level of Descemet's membrane |
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Wilson Disease Clinical Neuro Findings
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Dysarthria
Dyspraxia Ataxia Typical: Parkinson like extrapyramidal signs Begins with mild tremor, speech, writing problems |
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Wilson Disease Psychiatric
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Most common-depression
Labile mood psychosis |
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Wilson Lab
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Low serum ceruloplasmin
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Wilson MRI
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Lesions in 60% neuro symptomatic and 19% asymptomatic
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Wilson Disease Treatment
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Symptomatic- Penicillamine
Presymptomatic- Penicillamine or trientine Zn (competes with intestinal Cu absorption) |
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Idiophathic Copper toxicosis
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Excessive dietary copper intake
-Cu contaminated water -Brass vessels to store milk Serum Cu and ceruloplasmin markedly increased |
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Menkes "Kinky Hair" syndrome
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Genetic Defect in Cu absorption
Mental retardation Thermoregulatory dysfunction Hypopigmentation connective tissue abnormalities Pili torti (kinky hair) |