FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2047984926> ?p ?o ?g. }

• W2047984926 endingPage "124" @default.
• W2047984926 startingPage "122" @default.
• W2047984926 abstract "Associations between aplastic anaemia (AA) and other autoimmune (AI) diseases, particularly Graves disease, fasciitis or thymoma (Kumar & Goldman, 2002; Hirano et al, 2003) or coeliac disease (CD) with autoimmune disease (AI) have rarely been reported (Green & Cellier, 2007). The first three cases of CD associated with AA were recently published (Grey-Davies et al, 2008). We report the clinical and biological characteristics of five patients diagnosed with AA and CD in our centre. CD was diagnosed in three patients at the same time as AA while the two other patients were diagnosed with CD before AA (Table I). CD had always been suspected because of chronic diarrhoea with malabsorption and was diagnosed based on histology with mild to severe duodenal villous atrophy associated with an increase of intra-epithelial lymphocyte rate. These patients had also antibodies against Gliadin and/or transglutaminase. Observation of <30% cellularity during histopathological examination of bone marrow biopsies associated with pancytopenia diagnosed AA. Severity of AA was graded according to Camitta criteria from non-severe to very severe AA (Camitta, 1988). All patients had normal bone marrow cytogenetics and two had paroxysmal nocturnal haemoglobinuria (PNH). Four of our five patients were HLA DQ 02 as are the majority of CD patients (Green & Cellier, 2007). The main patients’ characteristics are shown in Table I. Haematological evolution was marked by progressive cytopenias and all patients were treated with anti-thymoglobulin (ATG) plus ciclosporin (CSA). Patient 1 responded to this regimen but remained CSA-dependent. The four other patients failed to respond to immunosuppression, three of whom underwent haematopoietic stem cell transplantation (Table I). Concerning CD, diarrhoea returned in Patients 1–3 despite following a gluten-free diet (GFD). Histology at relapse showed the persistence of duodenal villous atrophy only in Patient 2, which led to the clinical diagnosis of autoimmune enteropathy (AIE) in 1991. This hypothesis was supported by the good response to CSA. Patients 1 and 3, who also relapsed, were diagnosed with inflammatory bowel disease and collagenous colitis respectively. Unlike Grey-Davies et al (2008), only one of our patients (Patient 4) had no familial or personal medical histories of autoimmune disease (Table I). Constitutional T-regulatory-cell (Treg) deficit has been associated with various autoimmune diseases, including cytopenia and AIE (Gambineri et al, 2003). Mutation in the forkhead box protein P3 (FOXP3) is associated with a severe immune disorder, the immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) but some milder forms have been described, including diagnoses in adults (Zuber et al, 2007). An acquired Treg deficit was reported once in AA (Solomou et al, 2007). We failed to demonstrate that any of our patients had a Treg deficit but lymphocytes had been immuno-phenotyped (CD4+ CD25+FOXP3) only in living patients and only in Patient 5 at diagnosis, which renders interpretation impossible. All digestive biopsies were reviewed to look for arguments supporting another diagnosis including AIE. Classical CD that responds well to GFD is not usually associated with AIE but CD patients relapsing on GFD can be suspected of having AIE, or another sprue-like disease (Freeman, 2008). Digestive disease was apparently recurrent in 1–3 patients. Only Patient 2, suspected of having an AIE in 1991, had villous atrophy at diarrhoea relapse on GFD. She also had a history of idiopathic thrombopenic purpura (ITP), autoimmune haemolytic anaemia (AIHA), associated with common variable immune deficiency (CVID). Sera from four patients were collected to test for the presence of circulating anti-enterocyte antibodies by indirect immunofluorescence (IF) and a quantitative radioligand assay against the 75-kDa autoantigen [enzyme-linked immunosorbent assay (ELISA)] (method described in Patey-Mariaud de Serre et al, 2008) and were negative in three cases (Patient 1, 3 and 5). Unfortunately, there was no frozen serum sample available for Patient 2, who may have had clinical and histological AIE. Only patient 3’s serum was positive by ELISA. In conclusion, an AA-CD association is more frequent than previously thought. Another digestive disease may appear during AA-CD evolution. The systematic search for anti-Gliadin or transglutaminase antibodies as part of the immunological work-up for AA remains debatable. The prognosis of AA-CD is poor, mainly because of haematological complications. More studies are needed on this particular association, which has been largely underestimated until now." @default.
• W2047984926 created "2016-06-24" @default.
• W2047984926 creator A5010183839 @default.
• W2047984926 creator A5012691135 @default.
• W2047984926 creator A5019759127 @default.
• W2047984926 creator A5027850194 @default.
• W2047984926 creator A5049735878 @default.
• W2047984926 creator A5051559389 @default.
• W2047984926 creator A5064103067 @default.
• W2047984926 creator A5080673678 @default.
• W2047984926 date "2009-06-10" @default.
• W2047984926 modified "2023-09-30" @default.
• W2047984926 title "Coeliac disease and aplastic anaemia: a specific entity?" @default.
• W2047984926 cites W1967521412 @default.
• W2047984926 cites W1975842944 @default.
• W2047984926 cites W2047641254 @default.
• W2047984926 cites W2057417956 @default.
• W2047984926 cites W2062862675 @default.
• W2047984926 cites W2066935327 @default.
• W2047984926 cites W2091288322 @default.
• W2047984926 cites W2149689775 @default.
• W2047984926 cites W4236836788 @default.
• W2047984926 cites W4242467593 @default.
• W2047984926 doi "https://doi.org/10.1111/j.1365-2141.2009.07719.x" @default.
• W2047984926 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/19438483" @default.
• W2047984926 hasPublicationYear "2009" @default.
• W2047984926 type Work @default.
• W2047984926 sameAs 2047984926 @default.
• W2047984926 citedByCount "15" @default.
• W2047984926 countsByYear W20479849262014 @default.
• W2047984926 countsByYear W20479849262018 @default.
• W2047984926 countsByYear W20479849262019 @default.
• W2047984926 countsByYear W20479849262020 @default.
• W2047984926 countsByYear W20479849262021 @default.
• W2047984926 countsByYear W20479849262023 @default.
• W2047984926 crossrefType "journal-article" @default.
• W2047984926 hasAuthorship W2047984926A5010183839 @default.
• W2047984926 hasAuthorship W2047984926A5012691135 @default.
• W2047984926 hasAuthorship W2047984926A5019759127 @default.
• W2047984926 hasAuthorship W2047984926A5027850194 @default.
• W2047984926 hasAuthorship W2047984926A5049735878 @default.
• W2047984926 hasAuthorship W2047984926A5051559389 @default.
• W2047984926 hasAuthorship W2047984926A5064103067 @default.
• W2047984926 hasAuthorship W2047984926A5080673678 @default.
• W2047984926 hasBestOaLocation W20479849261 @default.
• W2047984926 hasConcept C126322002 @default.
• W2047984926 hasConcept C142724271 @default.
• W2047984926 hasConcept C203014093 @default.
• W2047984926 hasConcept C2777346216 @default.
• W2047984926 hasConcept C2779134260 @default.
• W2047984926 hasConcept C2779382419 @default.
• W2047984926 hasConcept C2780007613 @default.
• W2047984926 hasConcept C2780252810 @default.
• W2047984926 hasConcept C2780349322 @default.
• W2047984926 hasConcept C2780439177 @default.
• W2047984926 hasConcept C2781440808 @default.
• W2047984926 hasConcept C2993432164 @default.
• W2047984926 hasConcept C3019118998 @default.
• W2047984926 hasConcept C71924100 @default.
• W2047984926 hasConcept C90924648 @default.
• W2047984926 hasConceptScore W2047984926C126322002 @default.
• W2047984926 hasConceptScore W2047984926C142724271 @default.
• W2047984926 hasConceptScore W2047984926C203014093 @default.
• W2047984926 hasConceptScore W2047984926C2777346216 @default.
• W2047984926 hasConceptScore W2047984926C2779134260 @default.
• W2047984926 hasConceptScore W2047984926C2779382419 @default.
• W2047984926 hasConceptScore W2047984926C2780007613 @default.
• W2047984926 hasConceptScore W2047984926C2780252810 @default.
• W2047984926 hasConceptScore W2047984926C2780349322 @default.
• W2047984926 hasConceptScore W2047984926C2780439177 @default.
• W2047984926 hasConceptScore W2047984926C2781440808 @default.
• W2047984926 hasConceptScore W2047984926C2993432164 @default.
• W2047984926 hasConceptScore W2047984926C3019118998 @default.
• W2047984926 hasConceptScore W2047984926C71924100 @default.
• W2047984926 hasConceptScore W2047984926C90924648 @default.
• W2047984926 hasIssue "1" @default.
• W2047984926 hasLocation W20479849261 @default.
• W2047984926 hasLocation W20479849262 @default.
• W2047984926 hasOpenAccess W2047984926 @default.
• W2047984926 hasPrimaryLocation W20479849261 @default.
• W2047984926 hasRelatedWork W19206636 @default.
• W2047984926 hasRelatedWork W2019380343 @default.
• W2047984926 hasRelatedWork W2037244637 @default.
• W2047984926 hasRelatedWork W2044276960 @default.
• W2047984926 hasRelatedWork W2058712077 @default.
• W2047984926 hasRelatedWork W2095079590 @default.
• W2047984926 hasRelatedWork W2107957285 @default.
• W2047984926 hasRelatedWork W2415257904 @default.
• W2047984926 hasRelatedWork W25463327 @default.
• W2047984926 hasRelatedWork W4387063699 @default.
• W2047984926 hasVolume "146" @default.
• W2047984926 isParatext "false" @default.
• W2047984926 isRetracted "false" @default.
• W2047984926 magId "2047984926" @default.
• W2047984926 workType "article" @default.