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The aetiology of coeliac disease (CD) has many similarities to that of dermatitis herpetiformis (DH), except that DH lesions are mainly manifested in the skin. Mucosal enzyme deficiency plays an important part in CD pathology. Clinical studies indicated that the gluten exposure in CD could be partly corrected by the use of enzyme supplementation. Objective: Enzyme therapy, using enterically coated tablets containing caricain, was investigated as a means of protecting patients with DH against wheat gluten. Methods: A randomized, placebo-controlled clinical trial was carried out on 20 DH patients in clinical remission. The patients were divided into two groups of 10, one group given a placebo daily and the other the enzyme – containing tablets. Both groups were challenged with 6g of gluten daily in a double-blind trial. Symptoms and signs of skin involvement were recorded and graded at the start of the trial, after 7 days and after 14 days. Blood was also taken at the start and after 14 days and assayed for IgA EMA and anti-gliadin antibodies. Results: After 7 days the major features associated with DH were more severe and more common with the placebo compared with enzyme therapy. Before 14 days, seven patients in total, six on placebo, had to withdraw from the trial because of the effects of the gluten challenge whilst 2 patients on therapy developed blisters, erythema and itching. Serological tests indicated that IgA EmA antibodies and anti-gliadin antibodies after 14 days were not affected significantly, but indicated that abnormally high antibodies titers of both types were present in 8 patients at the start of the trial, suggesting the need for enzyme therapy in addition to the normal gluten-free diet for patient well-being. Conclusions: This study supports the use of enzyme supplementation as a safeguard for patients with DH on a nominal gluten-free diet.

Klíčová slova
caricain, endomysiální protilátky IgA, protilátky antigliadinu,
MeSH
celiakie MeSH
dermatitis herpetiformis * diagnóza dietoterapie imunologie krev patofyziologie MeSH
dospělí MeSH
dvojitá slepá metoda MeSH
enzymoterapie * MeSH
enzymy aplikace a dávkování farmakologie imunologie MeSH
gastrointestinální trakt patofyziologie MeSH
gluteny * aplikace a dávkování diagnostické užití farmakologie imunologie škodlivé účinky účinky léků MeSH
histologické techniky metody využití MeSH
imunologické testy metody využití MeSH
interpretace statistických dat MeSH
kožní manifestace * MeSH
lidé středního věku MeSH
lidé MeSH
ochranné faktory MeSH
potravní doplňky MeSH
randomizované kontrolované studie jako téma * MeSH
senioři MeSH
sérologie MeSH
tablety MeSH
věkové faktory MeSH
výsledek terapie MeSH
Check Tag
dospělí MeSH
lidé středního věku MeSH
lidé MeSH
mužské pohlaví MeSH
senioři MeSH
ženské pohlaví MeSH
Publikační typ
práce podpořená grantem MeSH
Geografické názvy
Austrálie MeSH
Polsko MeSH

Článek

Non-classical celiac disease: often missed

International journal of celiac disease. 2014 ; 2 (3) : 76-85.

ISSN 2334-3427
Medvik
Zdroj

Celiac disease (CeD) is an immune-mediated enteropathy triggered by ingestion of gluten in genetically susceptible individuals. CeD is a global disease and estimated to affect approximately one percent of the global population. With advent of simple serological tests for the diagnosis, the number of individuals diagnosed with CeD is increasing exponentially. It was initially thought that gluten hypersensitivity in CeD is limited to small intestine only and all other features are secondary to malabsorption, but it is now recognized that the hypersensitivity to gluten is not limited to small intestine alone and may affect other organs such as skin, brain, and bones independent of intestinal involvement. CeD is now considered a multi-system disorder and their clinical presentation may be with gastrointestinal symptoms, called “classical CeD” or more often with non-gastrointestinal symptoms called “non-classical CeD”. These patients may present with short stature, anemia, liver dysfunction (asymptomatic increase in transaminases, chronic liver disease, autoimmune hepatitis), cutaneous manifestations (dermatitis herpetiformis, oral ulcers), reproductive diseases (infertility, recurrent abortions), neurological manifestations (ataxia, peripheral neuropathy), and metabolic disorders (osteopenia/osteoporosis). What determines these variable phenotypes remain unclear but likely is a result of genetic as well as environmental factors. Many of these patients with non-classical CeD are likely to report to specialists other than gastroenterologists such as hematologists, endocrinologists, rheumatologists or gynecologists. Unfortunately, the awareness about non-classical presentations of CeD amongst health care professionals remains low. There is an urgent need to increase awareness among health care professionals about varied manifestations of CeD in order to decrease the burden of undiagnosed CeD.

Článek

Autoimunitní puchýřnaté dermatózy

Salavec, Miloslav, 1958-
Autor Autorita

Postgraduální medicína. 2007 ; 9 (č. 5) : s. 521-528.

ISSN 1212-4184
Medvik
Zdroj

Článek shrnuje klasifikaci, etiopatogenetické poznatky, klinické obrazy, možnosti diagnostiky a terapie závažných kožních onemocnění. I přes nejasnosti o etiologii autoimunitních kožních nemocnění je dokumentován vývoj poznatků o patogenetických mechanismech onemocnění, stejně jako o rozšiřujících se možnostech terapie.