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Porphyria cutanea tardae

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
M M Hatch, Z Nawas, R Kollipara, S K Tyring
Porphyria cutanea tarda (PCT) is the most common type of porphyria. It is caused by decreased activity of uroporphyrinogen decarboxylase, the fifth enzyme in the heme biosynthetic pathway, which converts uroporphyrinogen to coproporphyrinogen. A strong association with hepatitis C (HCV) has been reported and infection with the virus is a common precipitant. This article is protected by copyright. All rights reserved.
August 23, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27486346/clinical-applications-of-therapeutic-phlebotomy
#13
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
#14
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
https://www.readbyqxmd.com/read/27293254/human-immunodeficiency-virus-associated-sporadic-nonfamilial-porphyria-cutanea-tarda
#15
Sibashish Kamal Guha, Debabrata Bandyopadhyay, Abanti Saha, Niharika Ranjan Lal
Porphyria cutanea tarda (PCT), a relatively uncommon metabolic disease, is the most common cutaneous porphyria. Here, we present the case of a patient diagnosed with sporadic, nonfamilial PCT that presented with classical cutaneous findings and multiple risk factors, including alcohol abuse, human immunodeficiency virus/AIDS, that have been strongly associated with the sporadic form of PCT.
May 2016: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/27259839/reflectance-confocal-microscopy-in-the-diagnosis-of-vesicobullous-disorders-case-series-with-pathologic-and-cytologic-correlation-and-literature-review
#16
F Lacarrubba, A E Verzì, M Pippione, G Micali
BACKGROUND: Vesicobullous disorders are characterized by intraepidermal or subepidermal blistering resulting from different pathogenetic mechanisms. The diagnosis is generally based on clinical examination and semi-invasive/invasive procedures such as cytology and histopathology. In vivo reflectance confocal microscopy (RCM) is a non-invasive technique for real-time, en face imaging of the epidermis and upper dermis with high resolution close to conventional histopathology. PURPOSE: To evaluate RCM features of different vesicobullous diseases and correlate with cytologic and histopathologic examination...
November 2016: Skin Research and Technology
https://www.readbyqxmd.com/read/27139922/cutaneous-porphyrias-causes-symptoms-treatments-and-the-danish-incidence-1989-2013
#17
Anne L Christiansen, Lise Aagaard, Aleksander Krag, Lars M Rasmussen, Anette Bygum
Porphyrias are rare diseases caused by altered haem synthesis leading to the accumulation of different haem intermediates. Neurovisceral attacks may occur in acute porphyrias, while photosensitivity is the presenting symptom in cutaneous porphyrias. We present here an overview of symptoms and a flowchart for the diagnosis of cutaneous porphyrias, with recommendations for monitoring and an update of treatment options. From the Danish Porphyria Register, we present the incidences and approximate prevalences of cutaneous porphyrias within the last 25 years...
November 2, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/26924097/extrahepatic-manifestations-of-hepatitis-c-a-meta-analysis-of%C3%A2-prevalence-quality-of-life-and-economic-burden
#18
Zobair Younossi, Haesuk Park, Linda Henry, Ayoade Adeyemi, Maria Stepanova
BACKGROUND & AIMS: Hepatitis C virus (HCV) infection has hepatic and extrahepatic manifestations with various costs and impairments to health-related quality of life (HRQL). We performed a meta-analysis to determine the prevalence of extrahepatic manifestations in patients with HCV infection, how these impair HRQL, and their costs. METHODS: We performed systematic reviews of the literature using MEDLINE, CINAHL, and the Cochrane Systematic Review Database, from 1996 through December 2014, to identify studies of the following extrahepatic manifestations of HCV infection: mixed cryoglobulinemia, chronic kidney or end-stage renal disease, type 2 diabetes, B-cell lymphoma, lichen planus, Sjögren's syndrome, porphyria cutanea tarda, rheumatoid-like arthritis, or depression...
June 2016: Gastroenterology
https://www.readbyqxmd.com/read/26908385/porphyria-cutanea-tarda-associated-with-hfe-c282y-homozygosity-iron-overload-and-use-of-a-contraceptive-vaginal-ring
#19
James C Barton, Corwin Q Edwards
Porphyria cutanea tarda (PCT) is characterized by decreased uroporphyrinogen decarboxylase activity in hepatocytes, uroporphyrin I and heptacarboxyl porphyrin III accumulation, photosensitivity dermatitis, and increased storage iron. In women, estrogen therapy, including oral contraceptives, postmenopausal hormone replacement, and tamoxifen for breast cancer treatment, is a risk factor for PCT. We report the case of a woman who presented with PCT, HFE C282Y homozygosity, and hepatic iron overload and was using a contraceptive vaginal ring containing ethinyl estradiol, an estrogen...
2016: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/26872826/acute-intermittent-porphyria-precipitated-by-atazanavir-ritonavir
#20
Sheena Bharti, Prashant Malhotra, Bruce Hirsch
Porphyrias are a group of metabolic disorders that are relatively uncommon and underdiagnosed. Although the association between HIV infection and antiretrovirals with porphyria cutanea tarda is well established, there is less data linking HIV and the acute hepatic porphyrias. We report the first case of acute intermittent porphyria precipitated by the drugs atazanavir and ritonavir, presenting with unexplained abdominal pain.
February 11, 2016: International Journal of STD & AIDS
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