Primary acrodermatitis enteropathica is an autosomal recessive genetic disorder and manifests when two defective genes are inherited (one from each parent).
Carriershave one normal gene and one defective gene and will not usually have any symptoms of the disease.
The SLC39A4 gene located on chromosome 8q24.3 codes for the zinc transporter protein, ZIP4. A mutation in SLC39A4 disturbs the uptake, transport and overall optimum control and level of is affected causing partial absorption of zinc in ones intestine.
Also a large number of enzymes require zinc as a cofactor hence a defect in enzymes or not the right amount of enzymes are absorbed for further body function.
Excessive urinary loss of zinc (nephrotic syndrome)
Low levels of albumin and high catabolic states (trauma, thermal burn, extensive surgery, or cirrhosis
Necrolytic migratory erythema (glucagonoma) – distinctive rash around that affects normally the genital and anal region, the buttocks, groin and lower legs
Complications /Information to beware of/General tips:
Do not wait, phone for an ambulance if have or develop:
Hepatosplenomegaly (enlargement of liver and spleen)
Please talk to your healthcare professional (i.e. Medical Doctor/Pharmacist) for further advice
Detailed Information
Please copy and paste any key words from the title: Zinc Deficiency in the following respective 'Medtick References and/or Sources' to find out more about the disease (this also may include diagnosis tests and generic medical treatments).
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