Table 4.

Recommended Surveillance for Individuals with X-Linked Protoporphyria

System/ConcernEvaluationFrequency
Erythrocyte
protoporphyrin levels &
plasma total porphyrins
Both free & zinc-chelatedAnnually
Anemia Complete blood count w/indices
Iron store depletion Serum ferritin levels
Hepatic involvement Hepatic function (liver transaminases)
US exam (if cholelithiasis is suspected)As indicated
Fibroscan® to evaluate for hepatic fibrosis
Vitamin D deficiency Vitamin D 25-OH levels whether or not receiving supplementsAnnually

US = ultrasound

From: X-Linked Protoporphyria

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