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δ-ALA Synthase
δ-Aminolaevulinic acid
Uroporphyrinogen-III
5. Heriditary Coproporphyrinogen-III
coproporphyria (HC) oxidase
Protoporphyrinogen-IX
6. Variegate
porphyria (VP) Protoporphyrinogen – IX oxidase
Protoporphyrin-IX
Ferrochelatase
7. Erythropoetic or
porphyia (EP) Heme synthase
←Fe++
Heme
Aims and objectives
puberty
Pink _ 91.6 _ +++ Since CEP
childhood
Hoesh Test
Negative Positive
T. porphyrins Watson-
Schwartz test
Negative Positive
≤ 35umol/mmol > 35umol/mmol of
creatinine Not
of creatinine Absorption spectra : Peak
porphyria
at 553nm & at 540nm
Cutaneous
manifestation Severe
Neurovascular photosensivity
neurovisce
manifestation ral
Since After
manifestati
childhood Puberty on
Cutaneous Neurovisceral
manifestation manifestations
CEP PCT
EPP VP,HCP AIP
Conclusion:
Any Laboratory having
spectrophotometer with wavelength
scanning mode can diagnose
porphyria.
With this work VP and HCP can not
be differentiated.
Our future aim
Work is in progress for solvent extraction of
different porphyrins from urine, followed by its
quantitation. Then all the different porphyrias
can be diagnosed completely based on
laboratory
. findings.
Congenital erythropoetic porphyria
Porphyria cutanea tarda
Our research group:
Our Institution
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