Pseudoporphyria Cutanea Tarda and Non-A Non-S Hepatitis in a CAPD Patient

1987 ◽  
Vol 7 (4) ◽  
pp. 231-233 ◽  
Author(s):  
Martin Zeier ◽  
Manfred Doss ◽  
Traugott Ziegler ◽  
Eberhard Ritz ◽  
Michael Rambausek
Keyword(s):  
Peritoneal Dialysis ◽  
Porphyria Cutanea Tarda ◽  
Skin Lesions ◽  
Chronic Glomerulonephritis ◽  
Red Cell ◽  
Uroporphyrinogen Decarboxylase ◽  
Bullous Dermatosis ◽  
One Year ◽  
Serum Aminotransferases ◽  
Capd Patient

A 47-year-old man was treated by continuous ambulatory peritoneal dialysis (CAPD) because of chronic glomerulonephritis. One year later he developed skin friability and bullous dermatosis resembling porphyria cutanea tarda. The skin lesions were associated with an elevation of serum aminotransferases, most probably due to a blood transfusion-induced, non-A non-B hepatitis. Urinary, fecal and blood porphyrins including the activity of red cell uroporphyrinogen decarboxylase were normal. Thus we accepted the diagnosis of pseudoporphyria cutanea tarda in view of the relatively frequent association of cutaneous disease with maintenance dialysis.

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
Keyword(s):  
Liver Disease ◽  
Hepatitis C ◽  
Porphyria Cutanea Tarda ◽  
Elevated Serum ◽  
Sun Exposure ◽  
Skin Lesions ◽  
Virologic Response ◽  
Uroporphyrinogen Decarboxylase ◽  
Cutaneous Manifestations ◽  
Progressive Liver Disease

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.

scholarly journals Porphyria cutanea tarda in a patient on chronic ambulatory peritoneal dialysis.

1996 ◽  
Vol 7 (3) ◽  
pp. 397-402
Author(s):  
J C Ruggian ◽  
S Fishbane ◽  
F J Demento ◽  
J K Maesaka ◽  
G L Frei
Keyword(s):  
Peritoneal Dialysis ◽  
Porphyria Cutanea Tarda ◽  
Esrd Patient ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Hemodialysis Patients ◽  
Uroporphyrinogen Decarboxylase ◽  
Hepatic Iron Overload ◽  
Proper Diagnosis

Porphyria cutanea tarda is a disorder of heme biosynthesis resulting from a defect or deficiency in the enzyme uroporphyrinogen decarboxylase. Heme precursors accumulate in the blood, urine, stool, and skin, where exposure to sunlight results in the clinical manifestations. Porphyria cutanea tarda has been described in adult hemodialysis patients. The pathogenesis of porphyria cutanea tarda in this population is thought to be related to the inability of hemodialysis to adequately clear porphyrin precursors, resulting in increased precursor serum levels, precursor skin deposition, and subsequent clinical manifestations. A proper diagnosis of porphyria cutanea tarda in hemodialysis patients requires fractionation of serum porphyrins. Normalization of the porphyrin profile and reversal of the dermal manifestations require the withdrawal of hepatotoxic agents and the reversal of hepatic iron overload. A case of porphyria cutanea tarda in an adult ESRD patient treated with continuous ambulatory peritoneal dialysis is described. In this patient, the disease was related to elevated serum levels of phenytoin, which had been administered for seizure disorder.

scholarly journals Comparative Proteomic Analysis of Peritoneal Dialysate from Chronic Glomerulonephritis Patients

2013 ◽  
Vol 2013 ◽  
pp. 1-10 ◽  
Author(s):  
Hsin-Yi Wu ◽  
Alex Chien Hwa Liao ◽  
Chien-Cheng Huang ◽  
Pao-Chi Liao ◽  
Chih-Chiang Chien ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
Drug Targets ◽  
Protein Identification ◽  
Chronic Glomerulonephritis ◽  
Two Dimensional Gel Electrophoresis ◽  
Western Blots ◽  
Peritoneal Dialysate ◽  
Liquid Chromatography Tandem Mass ◽  
One Year ◽  
First Time

Peritoneal dialysis (PD) frequently contributes to peritoneal damage which cannot be easily identified without invasive techniques, implying the urgent need for biomarkers and revealing mechanisms. Chronic glomerulonephritis (CGN) is one of the leading causes of receiving dialysis treatment. Here, we attempted to analyze the peritoneal dialysate collected from CGN patients when they receive continuous ambulatory peritoneal dialysis (CAPD) treatment for the first time and after a year to reveal the protein changes that resulted from PD. Proteins were displayed by two-dimensional gel electrophoresis (2DE). Altered gel spots were digested followed by liquid chromatography-tandem mass spectrometry (LC-MS/MS) analysis for protein identification. Eight proteins were found to have differential expression levels between two groups. Their differential expressions were validated by Western blots in other sets of peritoneal dialysates. Proteins identified with higher levels in the first-time dialysate suggested their dominant appearance in CGN patients, while those that showed higher levels in peritoneal dialysate collected after one year may result from initial peritoneal inflammation or changes in the permeability of the peritoneum to middle-sized proteins. All the identified proteins may provide a perceptiveness of peritoneal changes caused by PD and may function as potential biomarkers or drug targets.

Scanning Electron Microscopy of Staphylococcus Epidermidis Adherence to Continuous Ambulatory Peritoneal Dialysis Catheters

1985 ◽  
Vol 43 ◽  
pp. 520-521
Author(s):  
William J. Lamoreaux ◽  
David L. Smalley ◽  
Larry M. Baddour ◽  
Alfred P. Kraus
Keyword(s):  
Electron Microscopy ◽  
Scanning Electron Microscopy ◽  
Peritoneal Dialysis ◽  
Continuous Ambulatory Peritoneal Dialysis ◽  
Staphylococcus Epidermidis ◽  
Sterile Saline ◽  
Intravascular Devices ◽  
Capd Patient ◽  
Scanning Electron

Infections associated with the use of intravascular devices have been documented and have been reported to be related to duration of catheter usage. Recently, Eaton et al. reported that Staphylococcus epidermidis may attach to silastic catheters used in continuous ambulatory peritoneal dialysis (CAPD) treatment. The following study presents findings using scanning electron microscopy (SEM) of S. epidermidis adherence to silastic catheters in an in vitro model. In addition, sections of polyvinyl chloride (PVC) dialysis bags were also evaluated by SEM.The S. epidermidis strain RP62A which had been obtained in a previous outbreak of coagulase-negative staphylococcal sepsis at local hospitals was used in these experiments. The strain produced surface slime on exposure to glucose, whereas a nonadherent variant RP62A-NA, which was also used in these studies, failed to produce slime. Strains were grown overnight on blood agar plates at 37°C, harvested from the surface and resuspended in sterile saline (0.85%), centrifuged (3,000 rpm for 10 minutes) and then washed twice in 0.1 M phosphate-buffered saline at pH 7.0. Organisms were resuspended at a concentration of ca. 106 CFU/ml in: a) sterile unused dianeal at 4.25% dextrose, b) sterile unused dianeal at 1.5% dextrose, c) sterile used dialysate previously containing 4.25% dextrose taken from a CAPD patient, and d) sterile used dialysate previously containing 1.5% dextrose taken from a CAPD patient.

Psychological Reactions to Continuous Ambulatory Peritoneal Dialysis

1984 ◽  
Vol 13 (4) ◽  
pp. 299-307 ◽  
Author(s):  
Michael T. Geiser ◽  
Craig Van Dyke ◽  
Robyn East ◽  
Michael Weiner
Keyword(s):  
Health Care ◽  
Peritoneal Dialysis ◽  
Active Control ◽  
Continuous Ambulatory Peritoneal Dialysis ◽  
Well Being ◽  
Psychological Reactions ◽  
Grief Reaction ◽  
One Year

The first twenty patients who entered our continuous ambulatory peritoneal dialysis (CAPD) program from March, 1979 to February, 1981 were interviewed to assess their psychological reactions to CAPD. Six patients were successfully maintained on CAPD for more than one year. CAPD provided patients with a greater sense of well-being, strength, and independence. This independence required adherence to a strict schedule of exchanges. Reactions to the loss of CAPD followed the pattern of a grief reaction. Those patients who were self-disciplined and comfortable assuming active control of their health care proved to be the best candidates for CAPD.

scholarly journals Identification of ATP2C1 mutations in the patients of Hailey-Hailey disease

2020 ◽  
Author(s):  
xiaoli li ◽  
dingwei zhang ◽  
jiahui ding ◽  
li li ◽  
zhenghui wang
Keyword(s):  
Nonsense Mutation ◽  
Polypeptide Chain ◽  
Stop Codon ◽  
Skin Lesions ◽  
Signaling Proteins ◽  
Missense Mutations ◽  
Functional Region ◽  
Bullous Dermatosis ◽  
Normal Human ◽  
Regulatory Effects

Abstract Background: Familial benign chronic pemphigus, also known as Hailey-Hailey disease (HHD), is a clinically rare bullous Dermatosis. However the mechanism has not been clarified. The study aim to detect novel mutations in exons of ATP2C1 gene in HHD patients; to explore the possible mechnism of HHD pathogenesis by examining the expression profile of hSPCA1, miR-203, p63, Notch1 and HKⅡ proteins in the skin lesions of HHD patients. Methods: Genomic DNA was extracted from peripheral blood of HHD patients. All exons of ATP2C1 gene in HHD patients were amplified by PCR and the products were purified and sequenced. All related signaling proteins of interest were stained by using skin lesion tissues from HHD patients and miR-203 levels were also determined. Results: One synonymous mutation c.G2598A (in exon 26), one nonsense mutation c.C635A and two missense mutations c.C1286A (p.A429D) and c. A1931G (p. D644G) were identified. The nonsense mutation changed codon UCG to stop codon UAG, causing a premature polypeptide chain of the functional region A. The two missense mutations were located in the region P (phosphorylation region) and the Mn binding site of hSPCA1. The level of hSPCA1 was significantly decreased in HHD patients compared to the normal human controls, accompanied by an increase of miR-203 level and a decrease of p63 and HKⅡ levels. Conclusion: In our study, we found four mutations in HHD. Meanwhile we found increase of miR-203 level and a decrease of p63 and HKⅡ levels. In addition, Notch1, which was negatively regulated p63, is downregulated. These factors may be involved in the signaling pathways of HHD pathogenesis. Our data showed that both p63 and miR-203 may have significant regulatory effects on Notch1 in the skin.

scholarly journals Identification of ATP2C1 mutations in the patients of Hailey-Hailey disease

2020 ◽  
Author(s):  
xiaoli li ◽  
dingwei zhang ◽  
jiahui ding ◽  
li li ◽  
zhenghui wang
Keyword(s):  
Nonsense Mutation ◽  
Polypeptide Chain ◽  
Stop Codon ◽  
Skin Lesions ◽  
Signaling Proteins ◽  
Missense Mutations ◽  
Functional Region ◽  
Bullous Dermatosis ◽  
Normal Human ◽  
Regulatory Effects

Abstract Background: Familial benign chronic pemphigus, also known as Hailey-Hailey disease (HHD), is a clinically rare bullous Dermatosis. However the mechanism has not been clarified. The study aim to detect novel mutations in exons of ATP2C1 gene in HHD patients; to explore the possible mechnism of HHD pathogenesis by examining the expression profile of hSPCA1, miR-203, p63, Notch1 and HKⅡ proteins in the skin lesions of HHD patients. Methods: Genomic DNA was extracted from peripheral blood of HHD patients. All exons of ATP2C1 gene in HHD patients were amplified by PCR and the products were purified and sequenced. All related signaling proteins of interest were stained by using skin lesion tissues from HHD patients and miR-203 levels were also determined. Results: One synonymous mutation c.G2598A (in exon 26), one nonsense mutation c.C635A and two missense mutations c.C1286A (p.A429D) and c. A1931G (p. D644G) were identified. The nonsense mutation changed codon UCG to stop codon UAG, causing a premature polypeptide chain of the functional region A. The two missense mutations were located in the region P (phosphorylation region) and the Mn binding site of hSPCA1. The level of hSPCA1 was significantly decreased in HHD patients compared to the normal human controls, accompanied by an increase of miR-203 level and a decrease of p63 and HKⅡ levels. Conclusion: In our study, we found four mutations in HHD. Meanwhile we found increase of miR-203 level and a decrease of p63 and HKⅡ levels. In addition, Notch1, which was negatively regulated p63, is downregulated. These factors may be involved in the signaling pathways of HHD pathogenesis. Our data showed that both p63 and miR-203 may have significant regulatory effects on Notch1 in the skin.

scholarly journals Identification of ATP2C1 mutations in the patients of Hailey-Hailey disease

2020 ◽  
Author(s):  
xiaoli li ◽  
dingwei zhang ◽  
jiahui ding ◽  
li li ◽  
zhenghui wang
Keyword(s):  
Nonsense Mutation ◽  
Polypeptide Chain ◽  
Stop Codon ◽  
Skin Lesions ◽  
Signaling Proteins ◽  
Missense Mutations ◽  
Functional Region ◽  
Bullous Dermatosis ◽  
Normal Human ◽  
Regulatory Effects

Abstract Background: Familial benign chronic pemphigus, also known as Hailey-Hailey disease (HHD), is a clinically rare bullous Dermatosis. However the mechanism has not been clarified. The study aim to detect novel mutations in exons of ATP2C1 gene in HHD patients; to explore the possible mechnism of HHD pathogenesis by examining the expression profile of hSPCA1, miR-203, p63, Notch1 and HKⅡ proteins in the skin lesions of HHD patients. Methods: Genomic DNA was extracted from peripheral blood of HHD patients. All exons of ATP2C1 gene in HHD patients were amplified by PCR and the products were purified and sequenced. All related signaling proteins of interest were stained by using skin lesion tissues from HHD patients and miR-203 levels were also determined. Results: One synonymous mutation c.G2598A (in exon 26), one nonsense mutation c.C635A and two missense mutations c.C1286A (p.A429D) and c. A1931G (p. D644G) were identified. The nonsense mutation changed codon UCG to stop codon UAG, causing a premature polypeptide chain of the functional region A. The two missense mutations were located in the region P (phosphorylation region) and the Mn binding site of hSPCA1. The level of hSPCA1 was significantly decreased in HHD patients compared to the normal human controls, accompanied by an increase of miR-203 level and a decrease of p63 and HKⅡ levels. Conclusion: In our study, we found four mutations in HHD. Meanwhile we found increase of miR-203 level and a decrease of p63 and HKⅡ levels. In addition, Notch1, which was negatively regulated p63, is downregulated. These factors may be involved in the signaling pathways of HHD pathogenesis. Our data showed that both p63 and miR-203 may have significant regulatory effects on Notch1 in the skin.

scholarly journals Efficiency and safety of using a peritoneal dialysis catheter weighted with a stainless steel ballast : the Limousin experience

2019 ◽  
Vol 2 (4) ◽  
pp. 193-200
Author(s):  
Bénédicte Larivière-Durgueil ◽  
Rémi Boudet ◽  
Marie Essig ◽  
Stéphane Bouvier ◽  
Ali Abdeh ◽  
...  
Keyword(s):  
Stainless Steel ◽  
Peritoneal Dialysis ◽  
Infectious Complications ◽  
Control Group ◽  
Peritoneal Dialysis Catheter ◽  
Causal Factors ◽  
Catheter Migration ◽  
Mechanical Complications ◽  
Increased Risk ◽  
One Year

Objective: To assess the recurrence of PD catheter migration after the introduction of a walnut ballast. Materials and Methods: Retrospective study from 1999 to 2014 of PD patients followed in Limousin. Were compared two groups: ballast group (patients who benefited from the establishment of stainless steel ballast at the intraperitoneal catheter extremity) with 26 patients and control group with 204 patients. The primary endpoint was the occurrence of an episode catheter’s migration after ballast’s establishment. Secondary objectives were (i) to determine the causal factors leading to the catheter weighting, (ii) to ensure the safety of the procedure on the following criteria: infectious complications, mechanicals complications, epurations criteria, and catheter’s survival. Results: More than one year after the implementation of the ballast, no recurrent migration was observed in 86.6% of cases. It wasn’t found an increased risk of infections (OR = 0.5, 95% CI [0.22, 1.13]) or mechanical complications (OR = 1.77- 95% CI [0.77, 4.05]) between the two groups. The adequation criteria were similar: KT / V total : 2.37 in the control group and 2.28 in the ballast group (p = 0.63). The survival of the ballast catheter was comparable among the two groups (p = 0.983). Three causal factors that led to the ballast were identified: automated peritoneal dialysis (APD) (OR = 0.38, 95% CI [0.16, 0.9]), the failure from the first use of the catheter (OR = 19.48, CI 95 % [7.67, 49.48]) and the incarceration of the omentum (OR = 15.84, 95% CI [5.81, 43.21]). Conclusion: The ballast used in these study appears to prevent recurrence of migration, without any impact in terms of infectious or mechanical complications, or on the dialysis criteria or on catheter’s survival. However this catheter does currently not have an EC authorization

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