scholarly journals Porphyria Cutanea Tarda in a 54-Year-Old Patient with a History of Hepatitis C: A Case Report

2021 ◽  
Vol 2 (3) ◽  
pp. 96-98
Author(s):  
Carlan SJ

Background Porphyria CutaneaTarda (PCT) is the most common type of porphyria and is caused by a decrease in the activity of the hepatic enzyme uroporphyrinogen decarboxylase. It is expressed in both a sporadic form and genetic form and typically presents with cutaneous manifestations described as skin blisters in sun exposed areas. Case A 54-year-old male presented complaining of bullous itchy lesions on his hands and upper extremities that were at different stages of healing. Lab results were consistent with porphyria including elevated serum total porphyrins. He was scheduled for phlebotomy every other week for six weeks, hydroxychloroquine, minimize any sun exposure and to completely stop smoking. Conclusion Widespread skin lesions associated with underlying liver disease is a characteristic presentation for PCT. Hepatitis C is an antecedent risk factor for PCT, but can now be treated with antiviral therapy with the expectation of attainment of a sustained virologic response. Improvements in arresting progressive liver disease in Hepatitis C patients may improve PCT symptoms, as well. Keywords: Porphyria CutaneaTarda; Hepatitis C; Acquired liver disease.

2017 ◽  
Vol 26 (3) ◽  
pp. 309-317 ◽  
Author(s):  
Liana Gheorghe ◽  
Ioan Sporea ◽  
Speranța Iacob ◽  
Roxana Șirli ◽  
Anca Trifan ◽  
...  

Background & Aims: Hepatitis C virus (HCV) infection is a common condition with endemic prevalence in some areas of the world. In Romania, the mean prevalence is about 3%. New treatments have become available on the market in recent years and new drugs are in the pipeline. A re-evaluation of HCV therapy was considered mandatory. The Romanian Society of Gastroenterology and Hepatology undertook this task for the practitioners of this country.Methodology: A group of recognized experts was created who screened the available literature and the major available guidelines. A list of items requiring attention was created and these were discussed and rated. Decisions were taken by consensus.Recommendations: We present here the second part of the Society’s recommendations for chronic HCV infection treatment. An agreement between experts was reached regarding the therapy of the special categories of patients infected with HCV, complications and monitoring of the therapy, follow-up of the patients who reached sustained virologic response and re-treatment of the patients with therapy failure.Conclusions: This Position Paper represents a guide for the assessment and the therapy of HCV infection. The recommendations are in concordance with other guidelines but are applied to real-life conditions in Romania. Abbreviations: CKD: Chronic kidney disease; DAAs: Direct-acting antivirals; DDIs: Drug-drug interactions; ESDL: End-stage liver disease; FCH: Fibrosing cholestatic hepatitis; GT: Genotype; HCV: Hepatitis C virus; HCC: Hepatocellular carcinoma; LT: Liver transplantation; MELD score: Mayo-Clinic End-Stage Liver Disease score; PDC: Premature discontinuation; PWID: Persons who inject drugs; RASs: Resistance associated substitutions; RBV: Ribavirin; RCT: Randomized controlled trial; SAE: Serious adverse events; SRGH: Romanian Society of Gastroenterology and Hepatology; SVR: Sustained virologic response.


2006 ◽  
Vol 43 (3) ◽  
pp. 224-228 ◽  
Author(s):  
Idilio Zamin Jr ◽  
Angelo Alves de Mattos ◽  
Ângelo Zambam de Mattos ◽  
Eduardo Migon ◽  
Claudia Bica ◽  
...  

BACKGROUND: Nonalcoholic steatohepatitis is a chronic liver disease with a high prevalence in the general population and a potential to evolve into cirrhosis. It is speculated that iron overload could be associated with liver injury and unfavorable progress in affected patients. AIMS: To evaluate the prevalence of mutation of the hemochromatosis gene (HFE) in patients with nonalcoholic steatohepatitis and to correlate it with histological findings in liver specimens. PATIENTS AND METHODS: Twenty-nine patients with nonalcoholic steatohepatitis were evaluated. The presence of mutation in the hemochromatosis gene (C282Y and H63D) was tested in all patients and its result was evaluated in relation to hepatic inflammatory activity, presence of fibrosis, and iron overload in the liver. The control group was composed of 20 patients with normal liver function tests and 20 patients infected with the hepatitis C virus, with elevated serum levels of aminotransferases and with chronic hepatitis as shown by biopsy. RESULTS: Mutation of the hemochromatosis gene (C282Y and/or H63D) was diagnosed in 16 (55.2%) patients with nonalcoholic steatohepatitis, in 12 (60%) patients with hepatitis C and in 8 (40%) patients with no liver disease. No association was found between the presence of mutation and inflammatory activity, nor with the presence of fibrosis in patients with nonalcoholic steatohepatitis. An association was found between the presence of mutation and the occurrence of iron overload in liver, but there was no association between liver iron and the occurrence of fibrosis. CONCLUSIONS: The findings suggest that iron does not play a major role in the pathogenesis and progression of nonalcoholic steatohepatitis, and routine tests of the hemochromatosis gene mutation in these patients should not be recommended.


2020 ◽  
pp. 106002802096411
Author(s):  
Anthony J. Gentene ◽  
Allison M. Bell ◽  
Alicia Pence ◽  
Kelly Thomas ◽  
Collin Jakubecz ◽  
...  

Background: Direct-acting antivirals (DAAs) for treatment of hepatitis C virus (HCV) have resulted in great success through high attainment of sustained virologic response (SVR). Risk factors for DAA treatment failure are important to identify because of worsened outcomes with failure and high treatment cost. Objective: We sought to identify whether hospitalization during treatment affects SVR. The primary outcome was the difference in SVR at 12 weeks after treatment Methods: This multicenter, single health system retrospective cohort review compared achievement of SVR between patients hospitalized during DAA treatment for HCV with those not hospitalized during treatment. Results: Patients in the hospitalized cohort (n = 94) had more severe disease at baseline than nonhospitalized patients (n = 167) as indicated through higher Model for End-Stage Liver Disease (MELD) scores, Fibrosis-4 scores, and imaging-suggested or biopsy-confirmed cirrhosis. Patients hospitalized during treatment had lower SVR rates compared with those not hospitalized (87.2% vs 95.2%; P = 0.043) but failed to reach significance when inpatient mortality was excluded on secondary analysis (91.1% vs 95.2%; P = 0.195). Patients who were hospitalized and did not achieve SVR had higher MELD scores, were more likely to have intensive care unit stay, and had longer hospital stay compared with those who achieved SVR. Of 94 patients, 93 provided home supply of DAAs during hospitalization. Conclusion and Relevance: Patients hospitalized during DAA treatment for HCV had reduced rates of SVR. This reduced SVR rate may be driven by inpatient mortality and severity of liver disease. Patient education to bring home supply of medication for use during admission is an effective intervention.


2016 ◽  
Vol 37 (1) ◽  
pp. 61-70 ◽  
Author(s):  
Thomas Ward ◽  
Jason Gordon ◽  
Beverley Jones ◽  
Hayley Bennett ◽  
Samantha Webster ◽  
...  

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5290-5290
Author(s):  
Jovana Yudin ◽  
Farzana R. Bacchus ◽  
Mark A. Crowther

Abstract 5290 Porphyria Cutanea Tarda (PCT) is caused by a deficiency in uroporphyrinogen decarboxylase, an enzyme in the heme biosynthesic pathway. This leads to an excess of porphyrins, which cause blistering and skin fragility on sun exposure. The goals of treatment are iron depletion and reduction of plasma porphyrins to ameliorate skin symptoms. We reviewed the management of all PCT patients at St. Joseph's Hospital followed by the Hematology service, with the objective of describing their treatment course and its efficacy. The type of treatment received as well as the number and volume of phlebotomies were recorded. Outcomes of interest were skin symptom resolution (clinical remission) and biochemical remission, as measured by serum ferritin. Target ferritin was set at 30–50 μg/L. A total of 18 patients with PCT met our inclusion criteria; 14 males and 4 females. Three patients had the familial subtype of PCT while 15 had the sporadic form. Precipitants included alcohol, hepatitis C, hemochromatosis and estrogens. All patients received phlebotomy and three received hydroxychloroquine. Most patients were referred and initially diagnosed by dermatologists. The total volume of phlebotomies per patient ranged from 750–9500 mL for resolution of cutaneous symptoms which was achieved in 75% of patients. At the end of the study period, 15/18 (83%) patients were in clinical remission and 12/18 (67%) were in biochemical remission. Follow-up of PCT patients by a hospital hematology service allows for symptomatic control and biochemical remission in the vast majority of patients through the use of phlebotomy and occasionally hydroxychloroquine. Disclosures: No relevant conflicts of interest to declare.


Dermatology ◽  
2009 ◽  
Vol 218 (1) ◽  
pp. 15-21 ◽  
Author(s):  
Bernard Cribier ◽  
Christine Chiaverini ◽  
Nassim Dali-Youcef ◽  
Michèle Schmitt ◽  
Michèle Grima ◽  
...  

Hepatology ◽  
1993 ◽  
Vol 17 (4) ◽  
pp. 551-557 ◽  
Author(s):  
Mar Decastro ◽  
Javier Sánchez ◽  
Jesús F. Herrera ◽  
Asunción Cháves ◽  
Rafael Durán ◽  
...  

2020 ◽  
Vol 14 (1) ◽  
pp. 41-44
Author(s):  
Mahmood Ahmad ◽  
Muhammad Ayub ◽  
Fawad Iqbal Janjua ◽  
Abdul Moiz Bhatti ◽  
Nooman Gilani

ABSTRACT Background: In chronic hepatitis C infection, hepatic decompensation remained a contraindication to treatment for many years. The direct acting antiviral drugs have shown high treatment success even in decompensated liver disease. This study aims to assess the response and safety profile of Sofosbuvir and Daclatasvir in patients of decompensated cirrhosis with chronic hepatitis C. Patients and Methods: It was a prospective observational study conducted at Gastroenterology Department of Gujranwala Medical College/ DHQ teaching Hospital, Gujranwala from February 2016 to December 2017.Consecutive patients of hepatitis C with decompensated cirrhosis were enrolled in the study. Sofosbuvir 400mg and Daclatasvir 60mg was given to all patients without ribavirin for a period of 24 weeks. Sustained virologic was taken as primary end point. Results: A total of 140 patients were included in our study, 122 patients (87%) completed the study, 08 patients (5.7%) were lost to follow up, treatment discontinuation was seen in 06 patients (4.2%) & 04 patients (2.8%) died during the study. 110 patients (90.2%) achieved end treatment response (ETR), 12 patients (9.8%) remained treatment non-responder, 100 patients (82%) achieved sustained virological response (SVR12) and 10 patients (8%) had a relapse of HCV infection. Conclusion: Once daily oral Sofosbuvir plus Daclatasvir without Ribavirin achieved overall high rates of sustained virologic response in patients with chronic HCV having decompensated liver disease.


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