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Porphyria cutanea tardae

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https://www.readbyqxmd.com/read/28416230/porphyria-cutanea-tarda%C3%A2-in-a-patient-with-hepatitis-c
#1
Elisabeth Gómez Moyano, Leandro Martínez Pilar, Maria Dolores Fernandez Ballesteros, Daniel Jesús Godoy Diaz
No abstract text is available yet for this article.
April 14, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28400347/squiz-your-knowledge-expanding-scarring-alopecia-in-a-patient-with-porphyria-cutanea-tarda
#2
Sara Campos, André Lencastre
No abstract text is available yet for this article.
April 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28369802/direct-acting-antivirals-for-hepatitis-c-virus-induce-a-rapid-clinical-and-biochemical-remission-of-porphyria-cutanea-tarda
#3
A Combalia, J To-Figueras, M Laguno, M Martinez-Rebollar, P Aguilera
Sporadic porphyria cutanea tarda (PCT) is strongly associated with HCV infection in our population (1,2,3,4.) . Therapeutic options for PCT include phlebotomies and low-dose 4-aminoquinolines, which show high rates of disease remission. However, some PCT patients may present frequent relapses attributable to resistance/intolerability/poor adherence to conventional treatments and/or persistence of risk factors. In 2014 we reported the first case of a patient with active PCT and HCV-HIV co-infection cured with direct antiviral agents (DAAs) (5) ...
March 29, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28321838/porphyria-cutanea-tarda-an-intriguing-genetic-disease-and-marker
#4
REVIEW
Nancy S Handler, Marc Z Handler, Matthew P Stephany, Glenn A Handler, Robert A Schwartz
Porphyrias are a group of intriguing genetic diseases of the heme pathway, of which porphyria cutanea tarda (PCT) is the most common. Resulting from a defect in enzymes in the porphyria pathway, PCT has been linked to several conditions. Recent studies have demonstrated a change in thinking regarding the human immunodeficiency virus (HIV) and development of PCT. The exacerbation of PCT with contraction of HIV is now believed to result from coinfection from the hepatitis C virus (HCV). Blistering of sun-exposed skin, a classic presenting sign of PCT, is not exclusive to the condition...
March 21, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28260267/performing-therapeutic-venesection-in-a-doctor-s-surgery
#5
Lim Hy, Ho Wk Ho
BACKGROUND: Although venesection was widely applied in the past for the treatment of various ailments and diseases, in modern medical practice, it is indicated in very few conditions, namely, hereditary haemochromatosis, polycythaemia and porphyria cutanea tarda. OBJECTIVE: This article briefly reviews the pathophysiology of these conditions, and the rationale and goals of therapeutic venesection as a treatment modality. It also summarises the venesection procedure itself and the considerations for setting up a venesection service in a doctor's surgery...
March 2017: Australian Family Physician
https://www.readbyqxmd.com/read/28252812/hepatitis-c-virus-and-its-cutaneous-manifestations-treatment-in-the-direct-acting-antiviral-era
#6
REVIEW
L E Wiznia, M E Laird, A G Franks
New all-oral direct-acting antivirals (DAA) have changed the hepatitis C virus (HCV) treatment landscape. Given that dermatologists frequently encounter HCV-infected patients, knowledge of the current treatment options and their utility in treating HCV-associated dermatologic disorders is important. In addition to highlighting the new treatment options, we review four classically HCV-associated dermatologic disorders - mixed cryoglobulinaemia (MC), lichen planus (LP), porphyria cutanea tarda (PCT) and necrolytic acral erythema (NAE) - and examine the role for all-oral direct-acting antiviral (DAA) regimens in their treatment...
March 2, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28210512/porphyria-cutanea-tarda-in-a-patient-with-end-stage-renal-disease-a-case-of-successful-treatment-with-deferoxamine-and-ferric-carboxymaltose
#7
Natacha Rodrigues, Fernando Caeiro, Alice Santana, Teresa Mendes, Leonor Lopes
Porphyria cutanea tarda (PCT) is a rare disease, with a strong association with hepatitis C virus. PCT is particularly problematic in end-stage renal disease patients as they have no renal excretion of porphyrins and these are poorly dialyzed. Also, conventional treatment of PCT is compromised in these patients as hydroxychloroquine is contraindicated, phlebotomies with the stipulated frequency are poorly tolerated in already anaemia-prone patients, and iron-chelating agents are less efficient in removing iron and contribute to worsening anaemia...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28133909/anti-hcv-for-porphyria-cutanea-tarda
#8
Ayman Abdelmaksoud
No abstract text is available yet for this article.
January 30, 2017: Dermatologic Therapy
https://www.readbyqxmd.com/read/28130583/a-case-of-porphyria-cutanea-tarda-of-the-liver-exhibiting-multifocal-macrovesicular-steatosis-in-the-background-of-microvesicular-steatosis-probably-caused-by-uneven-iron-accumulation
#9
Marie Nishiyama, Keiko Sakamoto, Yoshinobu Shinagawa, Ayako Morita, Hiroshi Urakawa, Ritsuko Fujimitsu, Hiroshi Shakado, Yoshihiro Hamada, Kengo Yoshimitsu
A 61-year-old man with chronic hepatitis B and a history of alcohol overconsumption was admitted to our hospital for the scrutiny of multiple echogenic liver nodules. CT and hepatobiliary phase of gadoxetate-enhanced MR imaging revealed no nodular lesions. Quantitative fat fraction images and R2* map of MR imaging suggested homogeneous steatosis and uneven iron deposition in the liver, namely moderately and severely elevated R2* values at the nodules and surrounding background liver, respectively. Biopsy specimens showed macrovesicular fatty liver and less iron deposition at the echogenic nodules, and microvesicular fatty change and more prominent iron deposition at the surrounding liver tissue...
January 27, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28108048/bullous-pseudobullous-pustular-dermatoses
#10
Mark R Wick
Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Examples of such disorders include impetigo, Herpes virus infections, pemphigus, bullous pemphigoid and pemphigoid gestationis, epidermolysis bullosa acquisita, IgA-related dermatoses, inherited epidermolysis bullosa variants, Hailey-Hailey disease, and porphyria cutanea tarda...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/27796941/hepatic-porphyria-a-narrative-review
#11
REVIEW
Sumant Arora, Steven Young, Sudha Kodali, Ashwani K Singal
Porphyrias are a group of metabolic disorders, which result from a specific abnormality in one of the eight enzymes of the heme biosynthetic pathway. These have been subdivided based on the predominant site of enzyme defect into hepatic and erythropoietic types and based on clinical presentation into acute neurovisceral and cutaneous blistering porphyrias. This review focuses on hepatic porphyrias, which include acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), aminolevulinic acid dehydratase deficiency porphyria (ADP), and porphyria cutanea tarda (PCT)...
November 2016: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/27767238/squamous-cell-carcinoma-of-the-penis-successfully-treated-with-imiquimod-5-cream-in-a-porphyria-cutanea-tarda-patient
#12
Pablo Fernández-Crehuet, Ricardo Ruiz-Villaverde, Juan J Rios-Martin, Amalia Pérez-Gil, Francisco Camacho-Martinez
No abstract text is available yet for this article.
October 21, 2016: Dermatologic Therapy
https://www.readbyqxmd.com/read/27732687/resolution-of-porphyria-cutanea-tarda-in-patients-with-hepatitis-c-following-ledipasvir-sofosbuvir-combination-therapy
#13
Yun Tong, Ye Kyung Song, Stephen Tyring
No abstract text is available yet for this article.
December 1, 2016: JAMA Dermatology
https://www.readbyqxmd.com/read/27669078/porphyria-cutanea-tarda
#14
Eric M Neverman, Rochelle Parker
No abstract text is available yet for this article.
October 1, 2016: Journal of the American Osteopathic Association
https://www.readbyqxmd.com/read/27661980/the-d519g-polymorphism-of-glyceronephosphate-o-acyltransferase-is-a-risk-factor-for-familial-porphyria-cutanea-tarda
#15
Colin P Farrell, Jessica R Overbey, Hetanshi Naik, Danielle Nance, Gordon D McLaren, Christine E McLaren, Luming Zhou, Robert J Desnick, Charles J Parker, John D Phillips
Both familial and sporadic porphyria cutanea tarda (PCT) are iron dependent diseases. Symptoms of PCT resolve when iron stores are depleted by phlebotomy, and a sequence variant of HFE (C282Y, c.843G>A, rs1800562) that enhances iron aborption by reducing hepcidin expression is a risk factor for PCT. Recently, a polymorphic variant (D519G, c.1556A>G, rs11558492) of glyceronephosphate O-acyltransferase (GNPAT) was shown to be enriched in male patients with type I hereditary hemochromatosis (HFE C282Y homozygotes) who presented with a high iron phenotype, suggesting that GNPAT D519G, like HFE C282Y, is a modifier of iron homeostasis that favors iron absorption...
2016: PloS One
https://www.readbyqxmd.com/read/27627571/clinical-biochemical-and-molecular-characteristics-of-the-main-types-of-porphyria
#16
REVIEW
Urszula Szlendak, Ksenia Bykowska, Agnieszka Lipniacka
Porphyrias are diverse disorders that arise from various inherited enzyme defects in the heme biosynthesis pathway, except for porphyria cutanea tarda (PCT), in which the enzyme deficiency in most cases is acquired. The biosynthetic blocks resulting from the defective enzymes are largely expressed either in the liver or bone marrow, the sites where the majority of heme is produced. Although the pathophysiologic mechanisms of the clinical manifestations of the porphyrias are not fully understood, two cardinal features prevail: skin photosensitivity and neurologic symptoms of intermittent autonomic neuropathy, acute neurovisceral attacks, and disorders of the nervous system...
March 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/27579753/porphyria-cutanea-tarda-in-a-hiv-positive-patient
#17
Valéria Aparecida Zanela Franzon, Emanuella Stella Mikilita, Fernanda Henriques Camelo, Rosana Camargo
This is a case report about Porphyria cutanea tarda (PCT) and its relationship with the infection caused by the human immunodeficiency virus (HIV). Cutaneous porphyria is an illness caused by enzymatic modification that results in partial deficiency of uroporphyrinogen decarboxylase (Urod), which may be hereditary or acquired. Several studies suggest that HIV infection associated with cofactors might trigger the development of porphyria cutanea tarda. In this case report, we present a patient infected with HIV, who after the introduction of antiretroviral therapy (ART) enjoyed clinical improvement of porphyria cutanea tarda symptoms...
July 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27550066/can-curative-antivirals-benefit-porphyria-cutanea-tarda-in-hepatitis-c-patients
#18
LETTER
M M Hatch, Z Nawas, R Kollipara, S K Tyring
No abstract text is available yet for this article.
April 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27486346/clinical-applications-of-therapeutic-phlebotomy
#19
Kyung Hee Kim, Ki Young Oh
Phlebotomy is the removal of blood from the body, and therapeutic phlebotomy is the preferred treatment for blood disorders in which the removal of red blood cells or serum iron is the most efficient method for managing the symptoms and complications. Therapeutic phlebotomy is currently indicated for the treatment of hemochromatosis, polycythemia vera, porphyria cutanea tarda, sickle cell disease, and nonalcoholic fatty liver disease with hyperferritinemia. This review discusses therapeutic phlebotomy and the related disorders and also offers guidelines for establishing a therapeutic phlebotomy program...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27410690/novel-treatment-using-cimetidine-for-erythropoietic-protoporphyria-in-children
#20
Joanna H Tu, Sarah L Sheu, Joyce M Teng
Importance: Erythropoietic protoporphyria (EPP) is a rare hereditary disease of heme biosynthesis that manifests as severe photosensitivity and hepatotoxicity. There have been no effective treatments to date. Cimetidine has been shown to inhibit heme biosynthesis and results in symptomatic improvement in patients with acute intermittent porphyria (AIP) and porphyria cutanea tarda (PCT). There is only 1 report in the literature describing the use of cimetidine in the effective treatment of an adult patient with EPP...
November 1, 2016: JAMA Dermatology
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