Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0268596 (
EMA
)
2,520
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
This study was undertaken to investigate the prevalence of coeliac disease in children and adolescents with Turner syndrome. Eighty-seven children and adolescents with Turner syndrome were screened for
IgA
-antiendomysium antibodies (
EMA
) and
IgA
-antigliadin antibodies (AGA), 5% (4/87) being found to be
EMA
-positive, and 15% (13/87) to have AGA levels above normal. Of the 10 patients who were either AGA- or
EMA
-positive and further investigated with intestinal biopsy, four manifested villous atrophy (i.e. all three of the
EMA
-positive patients, but only one of the seven AGA-positive patients). The results suggest
EMA
-positivity to be a good immunological marker for use in screening for coeliac disease, and such screening to be justified in patients with Turner syndrome.
...
PMID:Prevalence of coeliac disease in Turner syndrome. 1051 31
Coeliac disease has been reported to occur in 2-5 per cent of insulin-dependent diabetic patients (IDDM). Suitable non-invasive screening tests would allow identification of these patients. The aim of this study was to determine the value of the red cell distribution width (RDW) in detecting unrecognised coeliac disease in insulin-dependent diabetic patients (IDDM). All patients (n = 208) attending the Diabetic out-patient clinics at 2 adjacent centres who had a full blood picture and RDW carried out in the past 18 months were included. IDDM patients with an elevated RDW were identified and their charts were reviewed to determine if they had symptoms or laboratory abnormalities compatible with coeliac disease. They were invited to attend for serological screening. Ninety-five of 208 patients had an elevated RDW of whom 66 had non-insulin dependent diabetes mellitus and 29 had IDDM. Two of the 29 IDDM patients had died in the interim period. Six of the remaining 27 IDDM patients had previously been tested for serological markers associated with coeliac disease, of whom 1 had a positive antigliadin antibody titre (
IgA
-AGA 199 EU) and normal duodenal biopsy. Eighteen of the remaining 21 patients with IDDM consented to serological testing of whom only 1 had a positive titre of antiendomysial antibody (
IgA
-
EMA
) and villous atrophy. Although the RDW is known from previous studies to be a sensitive predictor for coeliac disease, this study has demonstrated its poor specificity in predicting IDDM patients who may have coeliac disease. The RDW is not recommended as a screening test for coeliac disease in patients with IDDM.
...
PMID:Application of red cell distribution width to screening for coeliac disease in insulin-dependent diabetes mellitus. 1054 Jul 81
The aim of this study was to investigate anti-gliadin (
IgA
-AGA and IgG-AGA), endomysial (
IgA
-EmA), and anti-reticulin (Ig-ARA) antibodies for monitoring celiac disease (CD) patients while on gluten-free and gluten-containing diets. Sera from 30 confirmed CD patients (13 boys, 17 girls), 1-24 years old, were examined for antibodies using ELISA (AGA) and Immunofluorescence (EmA, ARA) at 1, 3, 6, 9, and 12 months following institution of gluten-free diet and also at 3 and 6 months after challenge with gluten. One month following the exclusion of gluten from the diet, most antibodies are still positive. Twenty-three to 43% of antibodies remained positive by the end of the third month. At 6 and 9 months, 17% and 10% were positive, respectively. At 12 months no positive antibodies were detected. After gluten challenge, positive
IgA
-AGA and
IgA
-EmA titers were already demonstrated at 3 months (90% and 86%, respectively), while Ig-ARA titers showed a slow increase. Finally IgG-AGA responded with a slow decrease of titers to gluten-free diet levels and a fast increase upon provocation. The morphology of the intestine at diagnosis and during the periods of gluten-free diet and gluten challenge corresponds with the antibody titers. On the basis of these results, immunological markers may be applied to follow-up CD patients.
IgA
-AGA and
IgA
-
EMA
appear to be the most sensitive to dietary changes in gluten and correlate best with intestinal mucosal morphology.
...
PMID:Clinical application of immunological markers as monitoring tests in celiac disease. 1054 68
Celiac disease (CD) has been acknowledge as being responsible for numerous secondary pathologies, in particular autoimmune and neoplastic diseases. Whether CD is more prevalent in patients with non-Hodgkin's lymphoma (NHL) than in the normal population is not known. Accordingly, we carried out a study of 86 patients hospitalized in the Section of Oncology, Haematology and Internal Medicine of the Department of Medical, Oncological and Radiological Sciences of the University of Modena and Reggio Emilia and who, between 1988 and 1995 had been diagnosed as affected by NHL. On diagnosis, and before the beginning of antitumour therapy, all the patients were tested for antigliadin (AGA
IgA
and IgG) and antiendomysium (
EMA
) antibodies together with total class
IgA
antibody levels. Our findings showed that none of the 86 patients had an
IgA
deficit, while one tested positive for AGA
IgA
(43.9% v.n. < 7.5). The same patient also tested positive for
EMA
. The extremely high sensitivity and specificity of the AGA
IgA
and
EMA
led us to conclude that the patient was affected by CD, although his early death precluded confirmation by biopsy. The presence of one celiac patient among 86 NHL patients examined at the onset of the disease would suggest that CD is not infrequent in NHL. The numbers involved in our study are insufficient for statistical purposes, and we are therefore awaiting the results of a SIGEP multi-centre study into the connection between CD and lymphomas.
...
PMID:[Celiac disease and lymphoma]. 1068 58
To assess the usefulness of enzyme inhibition assay for the diagnosis of primary biliary cirrhosis (PBC), we determined the serial changes in enzymatic inhibitory antibody to pyruvate dehydrogenase complex (PDC) in patients with PBC, and compared the results to those of immunofluorescence and immunoblotting. Forty-nine sera from 19 patients with PBC who were followed-up for at least 16 months were tested for antimitochondrial antibodies (AMA) by indirect immunofluorescence, immunoblotting on bovine heart mitochondria, and enzyme inhibition assay using commercially available TRACE Enzymatic Mitochondrial Antibody (M2) Assay (
EMA
) kit. Of the 49 sera, 39 (80%), 35 (71%), 38 (78%), 31 (63%), and 36 (73%) were positive for AMA by immunofluorescence, for immunoglobulin G (IgG), IgM, and
IgA
class antibody against E2 subunit of PDC (PDC-E2) by immunoblotting, and for enzymatic inhibitory antibody to PDC by
EMA
, respectively. AMA titers determined by immunofluorescence did not change in 9 patients (47%), increased in 4 (21%), decreased in 3 (16%), and fluctuated in 3 (16%) during follow-up. The number of anti-M2 bands by immunoblotting did not change in 9 (47%), increased in 6 (32%), decreased in 2 (11%), and fluctuated in 2 (11%). Units of PDC activity by
EMA
did not change markedly in 16 (84%), increased in 2 (11%), and fluctuated in 1 (5%). Positive
EMA
results were common in cases with high levels of serum alkaline phosphatase and IgM, and the units of PDC activity by
EMA
correlated significantly and inversely with AMA titers by immunofluorescence, and serum reactivity to PDC-E2 by immunoblotting, respectively. There was no correlation between serial changes in biochemical data and units of PDC activity by
EMA
. In three patients who showed a decrease in AMA titers, AMA titers correlated more with
EMA
results than immunoblotting. Moreover, in a patient with fluctuating AMA titers, the units of PDC activity by
EMA
paralleled AMA titers. Our results suggest that
EMA
is useful for the diagnosis of AMA-positive PBC, and also could be used for monitoring the disease course in PBC.
...
PMID:Serial changes in enzyme inhibitory antibody to pyruvate dehydrogenase complex during the course of primary biliary cirrhosis. 1101 98
Antiendomysial antibodies (
EMA
) are today considered the most sensitive and specific serological marker of celiac disease (CD). The aim of the present study was to assess the occurrence of
EMA
of IgG isotype in
EMA
IgA
negative children with clinical suspicion of malabsorption and their relationship with CD. Serum
EMA
IgG1 determination was performed on 30
EMA
IgA
negative children with clinical suspicion of CD. Total serum
IgA
levels were further investigated. Sixty children with gastroenterological diseases other than CD were used as control disease patients and 63 healthy children were evaluated as the control group. Eighteen out of 30 children in the study showed
EMA
IgG1 positivity in sera and a villous height/crypt depth ratio <3:1 as index of intestinal atrophy. It is noticeable that a selective IgA deficiency was present in only 9 of 18
EMA
IgG1 positive children. In addition, clinical symptoms,
EMA
IgG1, and mucosal atrophy disappeared after 8-10 mo on a gluten-free diet. Neither
EMA
IgA
nor
EMA
IgG1 were detected in the children in the control groups. The other 12 children in study group showed no histologic abnormalities and were
EMA
IgG1 negative. In this study, we reveal a group of
EMA
IgG1 CD children without IgA deficiency. The diagnosis was based on the presence of gluten-dependent typical serological and histologic features of CD. Our data suggest that
EMA
IgG1 determination could be a new tool in the diagnostic workup of CD, useful in avoiding possible misdiagnosis.
...
PMID:Celiac disease diagnosis in misdiagnosed children. 1104 76
We investigated the presence of
IgA
anti-tissue transglutaminase (tTG) antibodies in untreated coeliac disease (CD) and other gastrointestinal diseases, and compared
IgA
tTG concentrations with anti-endomysium (
EMA
) immunofluorescent findings. The study included 116 untreated CD patients (74 female, 42 male, age range 15-78 years, median 47 years), 82 treated CD patients, 65 patients with normal duodenal histology, 260 disease control samples and 29 healthy volunteers.
IgA
anti-tTG,
EMA
, and anti-gliadin (AGA) antibodies were measured. Serum total
IgA
was measured in the CD patients. Two
IgA
-deficient untreated CD patients were excluded.
IgA
EMA
and
IgA
AGA were positive in 99 (87%) and 69 (61%), respectively, of the 114 untreated CD patients. Elevated
IgA
anti-tTG were found in 92/114 (81%) untreated coeliacs, 1/82 (1%) treated coeliacs, 2/65 (3%) non-coeliacs, 10/260 (4%) disease controls and 2/29 (7%) volunteers. Four of the untreated CD patients, with a normal serum total
IgA
concentration, were negative for all the serological tests.
IgA
anti-tTG concentrations were significantly higher in untreated coeliacs (median 10200 units/ml) than in other groups (Mann-Whitney, p<0.00001) and compared well with
IgA
EMA
titres (r(2)=0.54; p<0.0001).
...
PMID:Serum IgA tissue transglutaminase antibodies in coeliac disease and other gastrointestinal diseases. 1129 62
Recently, the endomysial antigen has been identified as the protein cross-linking enzyme known as tissue transglutaminase (tTG). Our objective was to compare a novel enzyme immunoassay (EIA) that detects
IgA
antibody against tTG to two standard IFA methods utilizing thin tissue sections of rat kidney/rat stomach (KS) and distal primate esophagus (PE) as substrates to detect
IgA
antibody against endomysium (
EMA
). Sera from 100 patients suspected of having gluten-sensitive enteropathy (GSE) and 23 sera possessing various antibodies used for EIA cross-reactivity studies were included. Additional tests, performed routinely in our laboratory, were utilized to further assess sera from patients suspected having GSE. These tests include anti-gliadin
IgA
antibody (AGA) and anti-reticulin
IgA
antibody (ARA) and are part of the European Society for Pediatric Gastroenterology and Nutrition (ESPGAN) revised criteria for diagnosing GSE. When compared to IFA using KS, the tTG EIA had a sensitivity of 87.5%, was 97.1% specific, and had an overall agreement of 94.0%. When compared to IFA using PE, the tTG EIA had a sensitivity of 92.6%, was 93.2% specific, and had an overall agreement of 93.0%. When the KS IFA was compared to the PE IFA for
EMA
, the KS IFA had a sensitivity of 96.3%, was 91.8% specific, and had an overall agreement of 93.0%. The majority of sera that were positive for tTG but were negative by IFA (KS, n = 2/PE, n = 5) possessed
IgA
antibodies against gliadin and/or reticulin. Five of six sera with negative results by PE IFA were positive by the KS IFA and possessed one or more antibodies to tTG and/or gliadin and/or reticulin. We conclude that the tTG EIA compares well to both KS and PE IFAs when detecting
IgA
antibody against endomysium. We do not recommend the use of PE to detect
EMA
primarily because of the inconsistencies (i.e., tissue selection, quality, and preparation) and limited availability of commercially prepared PE tissue.
...
PMID:IgA antibodies against endomysium and transglutaminase: a comparison of methods. 1134 22
Celiac disease is an autoimmune gastrointestinal disorder characterized by mucosal atrophy of the jejunum on exposure to gluten, a protein found in grains. The purpose of our study was to determine the prevalence of celiac disease in children with Downs syndrome in a U.S.-based Caucasian population. The 97 Downs syndrome children were screened for celiac disease using serum
IgA
-anti-endomysial antibody testing, which is highly specific and sensitive for the disorder. Children with titers greater than 1:5 (using the
IgA
endomysial antibody [
EMA
] test; EMA+) were considered affected. Ten children (10.3%) were EMA+. We examined their HLA DQA1 DQB1 genotype, karyotype, clinical characteristics, and the prevalence of celiac disease in their first-degree relatives. The nine available karyotypes were trisomy 21. Downs syndrome-specific mean height percentile was 64%+/-26% (range <5-99%) and weight percentile was 43%+/-28% (range 5-95%). Presence of diarrhea, constipation, vomiting, and abdominal pain was similar for children with and without celiac disease. Only bloating symptoms were significantly more frequent in those with celiac disease (EMA+). Seven of eight (88%) genotyped EMA+ children had the celiac disease-associated high-risk HLA DQA1*0501 DQB1*0201 genotype as compared with 13/ 80 (16%) of
EMA
- children. Five of 48 (10%) first-degree relatives of the celiac disease (EMA+) children were EMA+. In conclusion, celiac disease, as diagnosed by positive endomysial antibody tests, has an increased prevalence in children with Downs syndrome in the U.S. as compared with the general population (1/250). Clinical and growth characteristics do not distinguish between children with and without celiac disease. Based on these observations, it is recommended that children with Downs syndrome be screened for celiac disease.
...
PMID:Prevalence and clinical characteristics of celiac disease in Downs syndrome in a US study. 1142 58
We have evaluated a commercial assay for serum
IgA
class antibodies to tissue transglutaminase, the enzyme identified as the major endomysial autoantigen in coeliac disease (CD). Sera were available from 130 adults diagnosed with CD in Southern Derbyshire between 01 01 97 and 31 12 99. Sera from 100 patients without villous atrophy on small intestinal biopsy were controls. The ability of the assay to detect abnormally low total
IgA
levels was assessed using sera from 18 subjects with IgA deficiency. Sensitivity and specificity of this
IgA
-anti tissue transglutaminase (tTGA) assay (86.2%, 91.0%) were inferior to endomysial antibody (
EMA
; 93.8%, 100%). tTGA has significantly higher sensitivity than
IgA
-antigliadin (76.2%). tTGA was appropriately undetectable (<0.03 U/mL) in 17 of 18 subjects with selective IgA deficiency. The high likelihood ratio (35) for tTGA at levels >9.0 U/mL and methodological advantages over
EMA
suggest that tTGA could be used as a first line diagnostic test for CD. At tTGA levels of 4-9 U/mL, use of
EMA
as a second line test would improve specificity.
...
PMID:IgA-antitissue transglutaminase: validation of a commercial assay for diagnosing coeliac disease. 1207 74
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