The cutaneous porphyrias: a review

Murphy,

doi:10.1046/j.1365-2133.1999.02754.x

Cited by 71 publications

(33 citation statements)

References 69 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Scarring, hypertrichosis and hyperpigmentation can arise as a consequence of repeated skin eruptions. 11 The pathology of the cutaneous manifestations is mediated through direct photodynamic damage from absorbed ultraviolet light and oxygen free radical species production. 1 The enzymatic defects of HCP and VP are below the conversion of PBG to HMB, however it has been demonstrated that the addition of coproporphyrinogen III (HCP) and protoporphyrinogen IX (VP) lowered HMBS activity in human lymphoblasts and erythrocytes in vitro.…”

Section: Classification and Geneticsmentioning

confidence: 99%

The neurologic manifestations of the acute porphyrias

Simon

Herkes

2011

Journal of Clinical Neuroscience

View full text Add to dashboard Cite

Section: Classification and Geneticsmentioning

confidence: 99%

The neurologic manifestations of the acute porphyrias

Simon

Herkes

2011

Journal of Clinical Neuroscience

View full text Add to dashboard Cite

“…Bu da oksidatif hasara, DNA ve hücre membranının lipid peroksidasyonuna yol açarak, deri frajilitesi ve bül oluşumu ile sonuçlanır (11). Hastalık otozomal dominant geçişli olabildiği gibi olguların %80'i edinseldir ve multifaktöriyel etiyolojilidir (7). Hastalığın fenotipinin ortaya çıkması için bir veya birden fazla faktör gereklidir.…”

Section: Discussionunclassified

A Case of Porphyria Cutanea Tarda in a Patient Undergoing Dialysis

Guney¹,

Köktürk²,

Polat³

2010

Turk J Dermatol

View full text Add to dashboard Cite

ÖzetPorfirinojenler, hemoglobin ve sitokrom enzimleri gibi tüm hem proteinlerinin yapı taşlarıdır. Porfirya adı verilen bazı kalıtsal ve edinsel hastalık durumlarında, hemoglobin sentezinin bu ara ürünlerinin miktarı artar. Porfirya kutanea tarda en sık görülen porfirya tipidir. Bu tip özellikle güneş gören bölgelerde fotosensitivite sonucu ortaya çıkan büllerle karakterizedir. Porfirya kutanea tardanın; karaciğer hastalıkları, böbrek hastalıkları, HCV pozitifliği ile birlikteliği raporlanmıştır. Bu makalede hemodiyaliz uygulanan hastada gelişen bir porfirya kutanea tarda olgusu sunulmaktadır. AbstractPorphyrinogens are the building blocks of all the hemoproteins, such as hemoglobin and the cytochrome enzymes. In certain inherited and acquired disease states, called the porphyrias, these intermediate metabolites of hemoglobin synthesis are increased. Porphyria cutanea tarda is the most common type of porphyria. It is characterized by photosensitivity resulting in bullae, especially on sun-exposed parts. Liver diseases, kidney diseases, hepatitis C virus infection concomitant with porphyria cutanea tarda have been reported. We reported porphyria in a patient undergoing dialysis. (Turk J Dermatol 2010; 4: 75-7)

show abstract

“…It is well documented that a clinically overt form of either subgroup is precipitated by risk factors (the most important of them being alcohol, iron, hepatitis and estrogen), which can contribute to inactivation of UD in the liver [12][13][14][15][16][17].…”

Section: Introductionmentioning

confidence: 99%

Erythrocyte uroporphyrinogen decarboxylase activity and therapeutic phlebotomy in porphyria cutanea tarda

Kószó

Morvay

Boda

2000

J. Porphyrins Phthalocyanines

View full text Add to dashboard Cite

On the basis of the uroporphyrinogen decarboxylase ( UD ) activity in the erythrocytes, and the family history of the disease, different types of porphyria cutanea tarda ( PCT ) can be distinguished. In some cases, however, the distinction may involve some uncertainty (overlapping of subgroups). The question arises of whether the current erythrocyte UD activities in the different types of PCT are determined merely genetically. The erythrocyte UD activities in 72 unrelated patients with different forms of PCT (62 with type I PCT and 10 with type II PCT ), in different stages of the disease, were measured in order to test whether the activity exhibits any change during the long period of recovery. In both types the activities were faintly but significantly increased, from 94.9% (in PCT I) or 54.3% (in PCT II) up to 98.4% or 56.1% respectively. In both types the lower activity in the untreated condition can be attributed to a combination of several factors, including oxidative damage to UD , which results in a minor additional inhibition of the genetically determined enzyme activities.

show abstract

The cutaneous porphyrias: a review

Cited by 71 publications

References 69 publications

The neurologic manifestations of the acute porphyrias

The neurologic manifestations of the acute porphyrias

A Case of Porphyria Cutanea Tarda in a Patient Undergoing Dialysis

Erythrocyte uroporphyrinogen decarboxylase activity and therapeutic phlebotomy in porphyria cutanea tarda

Contact Info

Product

Resources

About