دانلود پاورپوینت پزشکی ( اختلالات متابولیسم پورفیرین Disorders of Porphyrins Metabolism )

(۳۴ اسلاید انگلیسی)

Excess amounts of porphyrins’ matabolic intermediate compounds in urine, feces, or blood indicate a metabolic block in heme synthesis.

Only three porphyrin compounds are clinically significant in humans: protoporphyrin (PROTO), uroporphyrin (URO), and coproporphyrin (COPRO).
An excess of the early precursors in the pathway of heme synthesis (ALA, porphobilinogen, or both) causes neuropsychiatric symptoms, including abdominal pain, vomiting, constipation, tachycardia, hypertension, psychiatric symptoms, fever, leukocytosis, and paresthesia.
Porphyrias in this category:
ALA dehydratase (ALAD) deficiency porphyria (ADP) or plumboporphyria (PP)
Acute intermittent porphyria (AIP).
The differentiation of neurologic porphyrias from other disorders is more difficult based on history and physical examination and must be verified by laboratory findings.This is thought to be due to axonal nerve deterioration in affected areas of the nervous system and vagal nerve dysfunction.