Clinuvel Pharmaceuticals Limited ( ASX: CUV; XETRA-DAX: UR9; ADR: CLVLY ) today announced that analyses of its Phase III US study (CUV039) evaluating the administration of SCENESSE (afamelanotide 16mg) to patients diagnosed with erythropoietic
protoporphyria (EPP) had shown a clinically meaningful treatment effect.
We have studied patients with congenital erythropoietic
porphyria and hepato-erythropoietic
porphyria, arising from double defects in the enzymes uroporphyrinogen cosynthase and uroporphyrinogen decarboxylase respectively, as well as homozygous VP.
The functional signaling routes by which (a) iron status, (b) erythropoietic
activity, (c) hypoxia, and (d) inflammation affect hepcidin expression are increasingly being investigated.
Anaesthetic management of a patient with erythropoietic
porphyria for ventricular septal defect closure.
However, the severity of hypoxia in that study did not correlate with the erythropoietic
It took two years for medics to diagnose her with erythropoietic
protoporphyria, which affects fewer than 400 people in the UK.
Tia's incurable condition was noticed at one -though doctors disagreed on a diagnosis before she was found to have rare erythropoietic
Results released today from a Phase III trial of the drug SCENESSE (R), conducted by Melbourne-based Clinuvel Pharmaceuticals Limited (ASX:CUV) (PINK:CLVLY), have shown that the drug has the ability to reduce and prevent painful phototoxic reactions experienced by patients with erythropoietic
Future studies are needed to determine the effect of incremental erythropoietic
activity on iron homeostasis in anemic, malnourished birds.
Dose and cost comparison of erythropoietic
agents in the inpatient hospital setting.
Where anaemia is due to ACD erythropoietic
agents are indicated and should be combined with intravenous iron supplements.
Staff time and costs for anaemia management with erythropoietic
stimulating agents in patients on haemodialysis.
Classification and main clinical features of the porphyrias [Taken from: Kauppinen, 2005] Type of Main Clinical Porphyria Symptoms Acute Porphyrias Acute Intermittent Porphyria Acute Attacks Hereditary Coproporphyria Acute attacks and skin fragility Variegate Porphyria Acute attacks and skin fragility Aminolevulinate Acute attacks and/or Dehydratase peripheral neuropathy Deficiency Porphyria Non Acute Porphyrias Congenital Erythropoietic
Severe photosensitivity Porphyria and haemolysis Erythropoietic
Photosensitivity Protoporphyria and liver damage Porphyria Cutanea Tarda Skin fragility and chronic liver disease Table 2.
activity causes TfR synthesis to be upregulated and thereby increase the soluble transferrin receptor (sTfR) level.
The addition of the sugar chains endows this recombinant protein with a longer serum half-life and sustained erythropoietic
activity as compared with conventional recombinant human EPO (rHuEPO) preparations.