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.
Molecular characterization of erythropoietic protoporphyria
in South Africa.
The UK Erythropoietic Protoporphyria
register: a progress report.
b-carotene as an oral photoprotective agent in erythropoietic protoporphyria
The changes in liver pathology with griseofulvin are similar to those found with human erythropoietic protoporphyria
(EP)-associated liver failure that occurs in a subset of patients and probably depends on genetic and physiologic susceptibility factors to determine its onset (Bloomer et al.
The six-month, randomised, multicentre, double-blind, placebo-controlled US study (CUV030) was primarily designed to confirm the efficacy and safety of subcutaneous bioresorbable afamelanotide implants (SCENESSE[R]) in reducing the severity of phototoxic skin reactions in patients with the rare light intolerance disorder erythropoietic protoporphyria
(EPP), allowing them to lead 'more normal' lives.
Proposed indications under study include dermatologic indications such as vitiligo and erythropoietic protoporphyria
, inflammatory bowel disease, ocular indications such as uveitis and dry eye, and nephritis and other kidney conditions.
Patients with VP have a sharply defined fluorescence emission maximum at an excitation wavelength of 626 [+ or -] 1 nm (15); the presence of this characteristic peak efficiently separates patients with VP from those with AIP, erythropoietic protoporphyria
, congenital erythropoietic porphyria, PCT, and hereditary coproporphyria (15, 16).
In addition, Clinuvel has recently announced initiation of a Phase II trial in the United States for the treatment of erythropoietic protoporphyria
(EPP), an orphan disease which affects around 3,000 Americans.
Diseases associated with photosensitivity include lupus erythematosus, porphyria cutanea tarda, erythropoietic protoporphyria
, variegate porphyria, xeroderma pigmentosum, and albinism.
This conclusion may be supported by the finding that the spectrum of the plasma of the LP patients resembled both in its shape and instability the spectrum of the plasma of an erythropoietic protoporphyria
patient, in which the abnormality in the spectrum is apparently because of the presence of a high concentration of free PP (20).
(EPP) is an inherited disorder caused by a partial deficiency of the enzyme ferrochelatase.