Porphyrine
Porphyrine
Metabolit | Molgewicht | Einheiten | Faktor |
---|---|---|---|
Uroporphyrin | 830,76 | μg, nmol | 1,204 |
Heptacarboxaporphyrin | 786,75 | μg, nmol | 1,271 |
Hexacarboxyporphyrin | 742,74 | μg, nmol | 1,346 |
Pentacarboxyporphyrin | 698,73 | μg, nmol | 1,431 |
Koproporphyrin | 654,72 | μg, nmol | 1,527 |
Protoporphyrin | 562,66 | μg, nmol | 1,777 |
μg/24 h | μg/g Krea | μmol/mol Krea | Relativer Anteil (%) | |
---|---|---|---|---|
Uroporphyrin | <27 | <33 | <4,5 | 1,4–32,9 |
Heptacarboxyporphyrin | <8 | <10 | <1,5 | 0,7–9,8 |
Hexacarboxyporphyrin | <6 | <7 | <1,0 | 0,3–3,5 |
Pentacarboxyporphyrin | <4 | <5 | <0,8 | 0,6–7,3 |
Koproporphyrin | <100 | <120 | <20,7 | 51,0–93,0 |
Gesamtporphyrine | <145 | <174 | <30,0 |
μg/g | nmol/g | |
---|---|---|
Uroporphyrin | <6 | <7 |
Heptacarboxyporphyrin | <2 | <3 |
Hexacarboxyporphyrin | <1 | <1 |
Pentacarboxyporphyrin | <3 | <4 |
Koproporphyrin | <24 | <37 |
Protoporphyrin | <80 | <142 |
Andere Porphyrine | <5 | <8 |
Gesamtporphyrine | <85 | <151 |
Uro | Hepta | Hexa | Penta | Kopro | Uro/Kopro | Kopro I/III | Vereinbar mit |
---|---|---|---|---|---|---|---|
↑↑↑ | ↑↑–↑↑↑ | ↑↑ | ↑ | n–↑ | >>1 | 0,2 – 0,5 | Chronische hepatische Porphyrie (PCT) |
↑↑↑ | n–↑↑ | n–↑ | n–↑↑ | ↑↑–↑↑↑ | 1–>>1 | var | Akute hepatische Porphyrie (AHP) |
↑↑↑ | ↑↑ | ↑ | ↑↑ | ↑↑↑ | 0,4–2,0 | >12 | CEP (Morbus Günther) |
n–↑ | n | n | ↑–↑↑ | ↑↑↑ | <0,1 | <0,05 | Akutes toxisches Porphyriesyndrom |
n | n | n | n | n–↑ | <0,2 | >2,5 | Cholestase, RS, DJS |
n | n | n | n–↑ | ↑ (-↑↑) | <0,2 | var | Sekundäre Koproporphyrinurie* |
Hepta | Hexa | Penta | Kopro | Proto | Sonstige | Kopro I/III | Vereinbar mit |
---|---|---|---|---|---|---|---|
↑↑ | ↑–↑↑ | n–↑ | n–↑ | n | Isokopro | 1,5–3,0 | Chronische hepatische Porphyrie (PCT) |
n | n | n–↑ | ↑↑–↑↑↑ | ↑–↑↑ | – | 0,15–0,40 | |
n | n | n | ↑–↑↑ | ↑↑–↑↑↑ | Hardero | <0,15 | Porphyria variegata (VP) |
n–↑ | n–↑ | n–↑ | ↑–↑↑↑ | n | – | >18 | CEP (Morbus Günther) |
n | n | n | n–↑ | n–↑↑ | Meso | 1,5–3,0 | Erythr. Protoporphyrien (EPP, XLPP) |