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  • 1
    Electronic Resource
    Electronic Resource
    Skin morphology in porphyria cutanea tarda does not improve despite clinical remission (1991)
    Oxford, UK : Blackwell Publishing Ltd
    Clinical and experimental dermatology 16 (1991), S. 0 
    Details
    ISSN: 1365-2230
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Five patients with porphyria cutanea tarda (PCT) were treated with a prolonged, low-dose chloroquine regimen (250 mg twice weekly). Their skin symptoms disappeared, and the abnormal urinary porphyrin excretion normalized during treatment periods ranging from 6 to 17 (mean 11.8) months. The morphology of the skin was studied by light and electron microscopy methods before treatment, at the onset of remission, and 6–12 months later. In samples taken during remission, the PAS-positive thickening of the superficial dermal vessels was comparable to that before treatment. In electron microscopy, the vessels showed thickening because of reduplication of the basal lamina and perivascular deposition of amorphous material; no consistent changes were found during remission. The results show that the histopathological changes of the skin in PCT are of chronic nature, and are probably irreversible.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2230.1991.tb00400.x
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Altered coproporphyrin-isomer excretion in patients with the Dubin-Johnson syndrome
    Amsterdam : Elsevier
    International Journal of Biochemistry 12 (1980), S. 975-978 
    Details
    ISSN: 0020-711X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0020-711X(80)90195-0
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Effects of haem arginate on variegate porphyria (1990)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 123 (1990), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Four patients with variegate porphyria (VP) were treated with repeated haem arginate infusions daily for 4 days and then weekly for 4 weeks. After the initial four daily doses of haem arginate (haem 3 mg/kg), the excretion of faecal protoporphyrin (mean 579 nmol/g dry wt) fell to an almost normal level (mean 123 nmol/g dry wt), and that of coproporphyrin (mean 162 nmol/g dry wt) to the normal level (mean 21 nmol/g dry wt) in all patients. However, during the period of the four weekly infusions of haem the excretion of porphyrins increased almost to the pretreatment level. Phototesting showed no changes in the photoreactivity of the skin, and no improvement in skin lesions was seen during the treatment. Except for one case of thrombophlebitis no side-effects occurred.In a child with homozygous VP, four daily infusions of haem arginate (2 mg/kg) normalized the faecal protoporphyrin content, but had no effect on the increased erythrocyte protoporphyrin concentration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1990.tb06299.x
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  • 4
    Electronic Resource
    Electronic Resource
    Skin changes in variegate porphyria (1990)
    Springer
    Archives of dermatological research 282 (1990), S. 108-114 
    Details
    ISSN: 1432-069X
    Keywords: Porphyria ; Dermal vessels ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The skin of 20 patients with variegate porphyria (VP) was studied using light, fluorescent, and electron microscopy. Twelve patients had skin symptoms and markedly increased fecal protoporphyrin excretion. Their sun-exposed skin was characterized by homogeneous PAS-positive thickening and IgG deposition in the vessel walls. The basic ultrastructural change was thickening of the vascular walls caused by reduplication of the basal lamina and perivascular deposition of amorphous material. Qualitatively similar but less prominent histopathological changes occurred in sun-protected skin in some of the patients. Six patients had no skin symptoms but an increased porphyrin excretion. The light microscopical changes were comparable to those in the patients with skin symptoms, but the ultrastructural changes were less severe. No abnormal histopathological changes occurred in two symptomless patients who had low lymphocyte protoporphyrinogen oxidase activity but normal fecal porphyrin excretion. These results show that the primary site of skin damage in VP is the vessel wall, and that histopathological changes of the skin also occur in porphyric patients who have never had skin symptoms. Factors determining the occurrence of skin symptoms in VP are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00493468
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  • 5
    Electronic Resource
    Electronic Resource
    Remissions in newly diagnosed Type 1 (insulin-dependent) diabetes: influence of interferon as an adjunct to insulin therapy (1984)
    Springer
    Diabetologia 27 (1984), S. 193-197 
    Details
    ISSN: 1432-0428
    Keywords: Type 1 diabetes ; interferon ; insulin therapy ; remission
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We studied the effect of interferon as an adjunct to conventional insulin therapy on the early course of Type 1 diabetes in 43 newly diagnosed patients. Compared with conventional therapy, interferon administration slightly delayed the improvement of glucose homeostasis and the rise of high density lipoprotein cholesterol, while C-peptide secretion was unaffected. Independent of the type of therapy, 18 patients (42%) entered partial remission. The remission began 2.0±0.6 months (mean±SEM) from the start of therapy and lasted for 4.1±1.1 months. Seven patients (16%) were still in remission 1 year after diagnosis. The patients who entered remission had higher initial C-peptide secretion, lower glycosylated haemoglobin levels and better initial control than patients without remission. Thus, interferon provided no benefits as an adjunct to conventional insulin therapy in unselected patients with newly diagnosed Type 1 diabetes. An important factor for the development of remission was the presence of C-peptide secretion at the time of diagnosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00273805
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  • 6
    Electronic Resource
    Electronic Resource
    RFLP analysis of three different types of acute intermittent porphyria (1990)
    Springer
    Human genetics 〈Berlin〉 85 (1990), S. 160-164 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Restriction fragment length polymorphism (RFLP) analysis was performed in three Finnish families with different subtypes of acute intermittent porphyria (AIP): 1) cross-reacting immunological material (CRIM)-negative with low erythrocyte porphobilinogen (PBG)-deaminase activity, 2) CRIM-positive with low PBG-deaminase activity and 3) CRIM-negative with normal PBG-deaminase activity. The disease-associated RFLP haplotype (A2B1C2) of the PBG-deaminase gene was the same in each family. In all three families, RFLP linkage analysis resulted in highly positive lod scores. The maximal lod score (4.3) was obtained at the recombinant fraction of zero, thus confirming a tight linkage of AIP to the PBG-deaminase locus. Of the 62 family members tested, 30 had the disease-associated haplotype; in 5 of them, conventional tests for AIP were normal and in one, uncertain. RFLP analysis can thus reveal new gene carriers and help in the diagnosis of individuals with uncertain results in other laboratory tests.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00193189
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