1/11. porphyria cutanea tarda associated with an acute gastrointestinal bleed: the roles of supplemental iron and blood transfusion.We describe a case of porphyria cutanea tarda (PCT) induced by blood transfusion and oral iron supplementation in an 80-year-old white woman. The patient experienced acute blood loss from 2 duodenal ulcers 2 months prior to presentation. During her hospitalization for the gastrointestinal bleed, her anemia was treated with blood transfusion, iron supplementation, and erythropoietin. Multiple blistering lesions developed on her skin 2 months after hospital discharge. Clinical and laboratory findings were consistent with a diagnosis of porphyria cutanea tarda. Treatment included discontinuation of iron therapy, local skin care, and phlebotomy, which prevented the development of more lesions. The roles of iron overload and chronic renal disease in the pathogenesis of the porphyria are discussed.- - - - - - - - - - ranking = 1keywords = iron overload, overload, iron (Clic here for more details about this article) |
2/11. porphyria cutanea tarda with menopausal exacerbation: the possible role of menstruation as natural phlebotomy.We describe a 48-year-old woman with a 12-year history of porphyria cutanea tarda who showed a remarkable exacerbation of her eruptions accompanied by high serum levels of iron and ferritin at menopause. As iron storage is known to be a triggering factor of porphyria cutanea tarda, the possible role of menstruation as natural phlebotomy to prevent porphyria cutanea tarda exacerbation is discussed.- - - - - - - - - - ranking = 0.0154304015494keywords = iron (Clic here for more details about this article) |
3/11. Beta-thalassaemia minor and porphyria cutanea tarda.A case of porphyria cutanea tarda occurring in association with beta-thalassaemia minor is reported in a 33-year-old Northern-Irish woman. Aetiological factors in this case included oral and parenteral iron therapy for refractory anaemia which was subsequently diagnosed as beta-thalassaemia minor, and the use of an oestrogen-containing oral contraceptive pill.- - - - - - - - - - ranking = 0.0077152007746998keywords = iron (Clic here for more details about this article) |
4/11. porphyria cutanea tarda, hepatitis c, alcoholism, and hemochromatosis: a case report and review of the literature.porphyria cutanea tarda (PCT) is associated with estrogen, certain medications, alcohol abuse, hepatitis viruses, and iron overload. Numerous studies have demonstrated an increased incidence of hepatitis c in patients with PCT; therefore, hepatitis screening should be routinely performed on these patients. On the other hand, although studies have long suspected hereditary hemochromatosis (HH) to be an underlying condition of PCT, many physicians have a low index of suspicion. Also, diagnosis of HH has been difficult until recently, when the gene mutation was identified. We present a case of a patient with PCT, hepatitis c, and alcoholism who was homozygous for the HH gene mutation.- - - - - - - - - - ranking = 0.9459935945771keywords = iron overload, overload, iron (Clic here for more details about this article) |
5/11. association of porphyria cutanea tarda with hereditary hemochromatosis.BACKGROUND: An increased frequency of hereditary hemochromatosis gene mutations occurs in patients with porphyria cutanea tarda. polymerase chain reaction analysis of peripheral blood for hemochromatosis gene (HFE) mutations is available for clinical use. Early detection and treatment of hereditary hemochromatosis limit disease progression and improve life expectancy. OBJECTIVE: We present 8 patients with porphyria cutanea tarda subsequently found to have hereditary hemochromatosis or mutations in the HFE gene. methods: Retrospective review of patients in whom both porphyria cutanea tarda and hereditary hemochromatosis or HFE gene mutations were diagnosed between 1976 and 2000. RESULTS: Eight patients with porphyria cutanea tarda (6 males, 2 females; age range, 4-60 years; mean age at diagnosis of porphyria cutanea tarda, 42 years) were subsequently found to have hepatic iron overload or HFE gene mutations. Two patients had liver biopsy findings compatible with homozygous hereditary hemochromatosis. In the other 6 patients, HFE gene analysis revealed 3 homozygous C282Y, 1 compound heterozygous C282Y/H63D, and 2 heterozygous C282Y mutations. Seven patients (88%) had no specific signs or symptoms of hereditary hemochromatosis at diagnosis. In 5 patients (63%), the diagnosis of hereditary hemochromatosis or HFE gene mutation was initially suspected by the dermatologist. CONCLUSION: porphyria cutanea tarda can be an important cutaneous marker for patients with mutations of the HFE gene. HFE gene analysis should be done in patients who present with porphyria cutanea tarda. The dermatologist may play a key role in the early diagnosis of subclinical hereditary hemochromatosis in patients who present with porphyria cutanea tarda.- - - - - - - - - - ranking = 0.9459935945771keywords = iron overload, overload, iron (Clic here for more details about this article) |
6/11. Occurrence of porphyria cutanea tarda during peginterferon/ribavirin therapy for chronic viral hepatitis c.porphyria cutanea tarda (PCT) is a metabolic disorder characterized by a reduced hepatic activity of uroporphynogen decarboxylase (URO-D), an enzyme of the heme synthesis. The clinical features of PCT may be brought into light by hepatic injury induced by hepatitis c virus (HCV). A significant association between HCV and PCT is well recognized, although the role of HCV in the appearance of PCT is still debated because confounding factors often coexist, such as alcohol, other viruses, drugs or iron overload (). HCV therapy may improve PCT although PCT was rarely reported as a de novo occurrence during an interferon/ribavirin therapy (Jessner et al. Hepatology 2002;36:1301-1302); here, we describe two such other cases.- - - - - - - - - - ranking = 0.9459935945771keywords = iron overload, overload, iron (Clic here for more details about this article) |
7/11. Sporadic porphyria cutanea tarda due to haemochromatosis.Haemochromatosis is a hereditary iron-overload syndrome caused by increased intestinal iron absorption and characterised by accumulation of potentially toxic iron in the tissues. Sometimes this disease presents as a cutanea porphyria. We describe a patient with joint complaints and blistering skin lesions on sun-exposed skin. After identifying the porphyria cutanea tarda by urine analysis we found that the serum activity of uroporphyrinogen decarboxylase (UROD) was normal, meaning a partial inactivation of UROD in liver tissue due to external factors. Further investigation showed the homozygous Cys282Tyr missense mutation and high levels of serum ferritin. It is important to recognise the symptoms of iron overloading at an early stage because hereditary haemochromatosis needs to be treated immediately. We therefore advocate routine sampling of ferritin levels in patients with unexplained joint complaints.- - - - - - - - - - ranking = 1.0520352326856keywords = iron overload, overload, iron (Clic here for more details about this article) |
8/11. Hepatoerythropoietic porphyria precipitated by viral hepatitis.porphyria cutanea tarda (PCT), the condition resulting from a deficiency of hepatic uroporphyrinogen decarboxylase activity, is the commonest form of porphyria. Both acquired and familial form exist and are commonly associated in adults with liver disease and hepatic iron overload. The condition is extremely rare in children; most cases of childhood PCT are familial and some particularly severe cases have been shown to have a hepatoerythropoietic porphyria or homozygous uroporphyrinogen decarboxylase deficiency. A case is described of hepatoerythropoietic porphyria in which the disease was first precipitated at the age of two by a coincidental hepatitis a infection and improved as the hepatitis cleared. This paper reviews the evidence that viral hepatitis may precipitate overt PCT in children in a manner analogous to the precipitation of PCT in adults by alcohol associated liver disease.- - - - - - - - - - ranking = 0.9459935945771keywords = iron overload, overload, iron (Clic here for more details about this article) |
9/11. Angiosarcoma, porphyria cutanea tarda, and probable chloracne in a worker exposed to waste oil contaminated with 2,3,7,8-tetrachlorodibenzo-p-dioxin.A worker developed angiosarcoma, porphyria cutanea tarda, and skin lesions characteristic of mild chloracne. About 10 years earlier he had been employed at a truck terminal in Saint Louis, missouri, at a time when it was sprayed with waste oil contaminated with 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD). The occurrence of these three rare conditions in a single exposed worker supports the aetiological relation between environmental exposure to TCDD and the subsequent development of soft tissue sarcoma and porphyria cutanea tarda.- - - - - - - - - - ranking = 0.0077152007746998keywords = iron (Clic here for more details about this article) |
10/11. porphyria cutanea tarda in a patient on chronic ambulatory peritoneal dialysis.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.- - - - - - - - - - ranking = 0.9459935945771keywords = iron overload, overload, iron (Clic here for more details about this article) |
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