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Abnormal sweat electrolytes in a case of celiac disease and a case of psychosocial failure to thrive. Review of other reported causes.

Sweat electrolytes were initially elevated in a child who was diagnosed as having celiac disease and also in one with psychosocial failure to thrive. Subsequent sweat tests were normal after nutritional status of the patients had improved with therapy. The reports of elevated sweat electrolytes in conditions other than cystic fibrosis are discussed. It is emphasized that sweat test methods other than the quantitative pilocarpine iontophoresis method are not reliable. The occurrence of false-positive sweat tests demonstrates the need for care in the interpretation of elevated sweat electrolytes and the necessity of performing repeat quantitative pilocarpine iontophoresis for the establishment of the diagnosis of CF. Celiac disease and malnutrition from other causes may cause sweat electrolytes to be elevated.

Celiac Disease↗

Celiac disease in autoimmune cholestatic liver disorders.

OBJECTIVES: In this study, serological screening for celiac disease (CD) was performed in patients with autoimmune cholestasis to define the prevalence of such an association and to evaluate the impact of gluten withdrawal on liver disease associated with gluten sensitive enteropathy. METHODS: Immunoglobulin A endomysial, human and guinea pig tissue transglutaminase antibodies, and immunoglobulin A and G gliadin antibodies were sought in 255 patients with primary biliary cirrhosis, autoimmune cholangitis, and primary sclerosing cholangitis. RESULTS: Immunoglobulin A endomysial and human tissue transglutaminase antibodies were positive in nine patients (seven primary biliary cirrhosis, one autoimmune cholangitis, and one primary sclerosing cholangitis), whose duodenal biopsy results showed villous atrophy consistent with CD. Two of these patients had a malabsorption syndrome, and one had iron-deficiency anemia. Clinical and biochemical signs of cholestasis did not improve after gluten withdrawal in the three patients with severe liver disease. A longer follow-up of the six celiac patients with mild liver damage is needed to clarify whether gluten restriction can contribute to slow down the progression of liver disease. CONCLUSIONS: The high prevalence of CD (3.5%) in autoimmune cholestasis suggests that serological screening for CD should be routinely performed in such patients by immunoglobulin A endomysial or human tissue transglutaminase antibodies.

Adult↗

Celiac disease.

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Celiac Disease↗

Hypocalciuria in overt and subclinical celiac disease.

OBJECTIVE: To investigate indices of calcium (Ca) homeostasis in celiac disease (CD). METHODS: Urinary Ca excretion rate, intestinal absorption of strontium (Sr) (used as index of intestinal Ca absorption), and other variables related to these end points were measured in newly diagnosed, untreated adult patients (n = 32) with overt and subclinical CD and compared with those of healthy controls (n = 27). Subclinical CD was defined by the absence of diarrhea (> or = 3 bowel movements/24 h), steatorrhea (fecal fat excretion > 6 g/24 h), and low body mass index (weight/height, kg/m2 < 21). RESULTS: Compared with controls, untreated celiac patients had 2 x lower Ca excretion (p < 0.0001) in 24-h and overnight urine (fed condition) but normal Ca excretion in urine samples collected under fasting (2-h) condition; the increase in urinary Ca excretion from fast to fed condition was 4 x lower in untreated celiac patients (p < 0.0001). Patients with overt and subclinical CD did not have significantly different urinary Ca excretion rates. Sr absorption rate was 45% lower in untreated patients than controls (p < 0.0001). Patients with overt and subclinical CD did not have significantly different Sr absorption rates. Sr absorption rate (r = 0.576, p < 0.0001) related to the increase in urinary Ca excretion from fast to fed condition. In celiac patients, 24-h urinary Ca excretion increased by 52% (p < 0.0001) over baseline after 6 months of gluten-free diet, and urinary Ca excretion under fasting condition did not significantly change. CONCLUSIONS: Overt and subclinical CD is associated with low urinary Ca excretion under fed condition, which relates to low intestinal absorption.

Adult↗

Assessing the causal association between celiac disease and Alzheimer disease and frontotemporal dementia: A bidirectional Mendelian randomization approach.

This study aimed to investigate the bidirectional causal relationship between celiac disease (CD) and the risk of Alzheimer disease (AD) or frontotemporal dementia (FTD) using Mendelian randomization (MR), in order to clarify prior inconsistent findings. We analyzed summary-level genome-wide association study (GWAS) data for CD, AD, and FTD. Single-nucleotide polymorphisms (SNPs) strongly associated with each condition were selected as genetic instruments. MR analysis was conducted in 2 directions: from CD to AD/FTD and from AD/FTD to CD. Mendelian Randomization Pleiotropy RESidual Sum and Outlier (MR-PRESSO) was used to detect and correct for pleiotropy, and Cochran Q assessed heterogeneity. Leave-one-out and Mendelian Randomization-Egger (MR-Egger) regression sensitivity analyses were performed to evaluate robustness. No evidence of a causal effect was found between CD and either AD or FTD in either direction (P&#x2005;>&#x2005;.05). Similarly, genetic liability to AD or FTD did not increase the risk of CD. Sensitivity analyses supported the robustness of the results, showing no pleiotropy or heterogeneity. Our findings suggest that CD is not causally linked to the development of AD or FTD. While shared genetic factors or comorbidities may exist, the association is likely noncausal, and other mechanisms of cognitive decline in CD patients warrant further study.

Humans↗

The gluten connection: the association between schizophrenia and celiac disease.

OBJECTIVE: Schizophrenia affects roughly 1% of the population and is considered one of the top 10 causes of disability worldwide. Given the immense cost to society, successful treatment options are imperative. Based on initial findings, gluten withdrawal may serve as a safe and economical alternative for the reduction of symptoms in a subset of patients. METHOD: A review of the literature relevant to the association between schizophrenia and celiac disease (gluten intolerance) was conducted. RESULTS: A drastic reduction, if not full remission, of schizophrenic symptoms after initiation of gluten withdrawal has been noted in a variety of studies. However, this occurs only in a subset of schizophrenic patients. CONCLUSION: Large-scale epidemiological studies and clinical trials are needed to confirm the association between gluten and schizophrenia, and address the underlying mechanisms by which this association occurs.

Autoimmune Diseases↗

Serum antigliadin antibody levels as a screening criterion before jejunal biopsy indication for celiac disease in a developing country.

The objective of the present study was to determine the efficacy of detection of antigliadin immunoglobulins G and A (IgG and IgA) for the diagnosis of celiac disease in a developing country, since other enteropathies might alter the levels of these antibodies. Three groups were studied: 22 patients with celiac disease (mean age: 30.6 months), 61 patients with other enteropathies (mean age: 43.3 months), and 46 patients without enteropathies (mean age: 96.9 months). Antigliadin IgG and IgA ELISA showed sensitivity of 90.9 and 95.5%, respectively. With the hypothetical values of prevalence ranging from 1:500 to 1:2000 liveborns, the positive predictive value varied from 8.5 to 2.3% for IgG and from 4.8 to 1.1% for IgA. Considering the patients without enteropathies, specificity was 97.8 and 95.7% for IgG and IgA, respectively. In patients with other enteropathies, specificity was 82.0 and 84.1%, respectively. When patients with and without other enteropathies were considered as a whole, specificity was 88.8 and 91.6%, respectively. The specificity of positive IgG or IgA was 93.5% in children without enteropathies and 78.7% in the presence of other enteropathies. The negative predictive value for hypothetical prevalences varying from 1:500 to 1:2000 liveborns was 99.9%. Thus, even in developing countries where the prevalence of non-celiac enteropathies is high, the determination of serum antigliadin antibody levels is a useful screening test prior to the jejunal biopsy in the investigation of intestinal malabsorption.

Analysis of Variance↗

Malignancy in a 19-year experience of adult celiac disease.

Malignancy has developed in 10 of 93 patients with celiac disease who attended this hospital from 1959 to 1978. Four patients developed lymphoma, 5 a squamous carcinoma of the esophagus, and 1 an adenocarcinoma of the ileum. Because the majority of patients were ingesting varying amounts of gluten at the time of their follow-up no conclusion can be drawn about the possible role of a gluten-free diet in the prevention of malignancy.

Adenocarcinoma↗

The coexistence of cystic fibrosis and celiac disease.

Two patients with cystic fibrosis (CF) who subsequently developed celiac disease (CD) are described. Since organ culture of intestinal mucosa has been used to establish an in vitro model for the study of CD, we utilized this opportunity to determine whether duodenal mucosa obtained from each of these two patients and their immediate families differed in its organ culture behavior from mucosa obtained from patients with CD alone. Additionally, as specific HL-A types are associated with CD, we used HL-A typing to determine whether the two patients with CF-CD differed genetically from patients with CD alone. One of our patients was HL-A8, the most common type associated with CD; the other was HL-A12, as are many of the non-HL-A8 celiac patients. The response in organ culture of the mucosa of these two patients was the same as the response in organ culture of the mucosa from patients with CD alone. These and other data suggest that CD occurring in patients with CF is no different than CD occurring alone.

Adolescent↗

Systemic lupus erythematosus with celiac disease: a report of five cases.

Systemic lupus erythematosus and celiac disease (CD) are rarely reported in combination. We report five cases seen over a 4-year period. The two conditions occurred concomitantly in one patient, whereas the CD antedated the lupus in one patient and postdated the lupus in the remaining three patients. Villous atrophy on duodenal biopsy specimens with a favorable response to a gluten-free diet was noted in all five patients. Only four patients had positive serological tests for CD and only three had abdominal symptoms.

Adolescent↗

Erythema elevatum diutinum associated with celiac disease: response to a gluten-free diet.

An 11-year-old girl with a history of insulin-dependent diabetes mellitus had erythema elevatum diutinum (EED) associated with a celiac disease related to a possible kidney disease. Dapsone did not improve the skin manifestations. However, the lesions disappeared after a gluten-free diet was begun. To our knowledge, this report describes the first case of EED in a patient with celiac disease.

Celiac Disease↗

Celiac disease presenting as entero-enteral intussusception.

Cramping abdominal pain with intermittent intestinal obstruction finally prompted investigation in a 4 1/2-year-old boy with severe failure to thrive (FTT). An entero-enteric intussusception was corrected, and celiac disease was identified as the cause of his inanition. Concomitant FTT and cramping abdominal pain should prompt investigation for celiac disease and small-bowel intussusception.

Abdominal Pain↗

[Up-date on the etiopathogenesis of celiac disease].

Wheat, oat, rye and barley flours are toxic for celiac patients. Prevalence and incidence of Celiac Disease (CD), quite variable from country to country, are very high in Austria (1 out of 476 born alive) and low in France (1 out of 41.667 born alive). This difference is probably due to its multifactorial genesis. In a multicentric Italian study, histocompatibility antigens of HLA complex II were typed in 460 CD children. DR3 was present in 63% of the cases (Relative Risk = RR: 6.8), DR7 in 67% (RR: 3.8) and DR3/DR7 in 22.5% (RR: 10.5), while in 7.7 of patients both antigens were absent. Therefore in a certain percentage of CD patients these risk antigens are absent, while in the normal population they can be present. The probability of CD increases when HLA DR3 and DR7 are present (but their absence cannot exclude the disease. The main etiological factor is gluten and its fractions (B, B1, B2, fraction 9 etc.). It seems that breast feeding can prevent or delay the onset of CD, while the age at gluten introduction does not modify the risk. Pathogenetic mechanisms are still under discussion: 3 theories are under investigation. 1) Enzymatic theory: a proteolytic enzyme for gluten digestion could be lacking. This theory is not yet proven, while Bruce et al. found in the jejunal mucosa of CD patients an elevation of a transglutaminase, which binds the gluten to enterocytes. Its level does not seem to vary with the diet. 2) Lectinic theory: the gluten bind the enterocyte membrane by a lectinic mechanism and damage it.(ABSTRACT TRUNCATED AT 250 WORDS)

Celiac Disease↗

Antibodies to E1b protein-derived peptides of enteric adenovirus type 40 are associated with celiac disease and dermatitis herpetiformis.

Sera from 41 children with untreated celiac disease (CD), and 16 children with dermatitis herpetiformis (DH), and 57 matched controls were studied for serum antibodies to synthetic peptides derived from an early E1b protein of adenovirus type 12 and type 40 (Ad12, Ad40) and A-gliadin. In addition to peptides which share homology with A-gliadin, "nonhomologous" peptides derived from Ad12 and Ad40 E1b proteins were used as antigens in ELISA. Patients with CD and DH had significantly higher IgG antibody levels than those of controls to the nonhomologous Ad40 E1b peptide. Concomitant presence of antipeptide IgA antibodies to A-gliadin and Ad12 E1b or Ad40 E1b peptides (which share no homology with A-gliadin) increased the risk of CD/DH suggesting that both adenovirus infections and gluten exposure contribute to the development of CD/DH.

Adenovirus E1B Proteins↗

[Clinical data on celiac disease with an early or late onset].

Typical symptoms in celiac disease (CD) are usually associated with early onset of the disease, whereas an atypical symptomatology has more often a later onset. The aim of this study was to evaluate the prevalence of some clinical signs and symptoms in children whose CD started before one year of age ("early onset" 135 children, M/F 50/85, mean age at onset 6.9 +/- 1.9 months) and in children whose disease started later ("late onset", M/F 14/26, mean age at onset 26.3 +/- 26.7 months). We analyzed: a) time lapse between gluten introduction and onset of symptoms, b) prevalence of patients with gastrointestinal symptoms alone and that of patients with gastrointestinal plus extraintestinal symptoms, c) frequency of each symptom. We then evaluated the influence of breast feeding and age of gluten introduction on time lapse. Our results showed that typical gastrointestinal symptoms, like diarrhea anorexia and abdominal distension prevailed both in children with early and late onset; whereas failure to thrive was significantly more frequent in children with an early onset CD (p < 0.01). Breast feeding delayed onset of symptoms: time lapse was significantly longer in children breast fed for a longer time (p < 0.001). On the contrary, age at first gluten ingestion seemed to have no influence on age at onset, since it was similar in both groups.

Adolescent↗

Serum granzymes and CD30 are increased in children's milk protein sensitive enteropathy and celiac disease.

BACKGROUND: Cow's milk protein sensitive enteropathy (CMSE) and celiac disease (CD) associate with upregulation of local intestinal immune responses, including activation of cytotoxic lymphocytes. OBJECTIVE: We investigated whether this upregulation can be detected at the systemic level by analyzing serum concentrations of granzymes A (GrA) and B (GrB), soluble Fas, and CD30. METHODS: Fifty-seven children with a final diagnosis of CMSE in 23 subjects (18 untreated and 5 on a diet), 20 untreated CD patients, and 14 control subjects with no gastrointestinal disease were examined by endoscopy for gastrointestinal complaints. Duodenal biopsies were studied for basic histology, CD3, alphabeta and gammadelta T-cell receptors (TCRs), and serum samples by commercial ELISA assays. RESULTS: Concentrations of GrA and GrB were significantly higher in untreated CMSE and in CD as compared with the control subjects. Measurable GrB was present in only 20% of the control subjects but in 100% of patients with CMSE. CD30 was similarly increased in both untreated CMSE and CD cases, whereas in treated CMSE cases the concentrations were equal to the control subjects. All groups showed similar soluble Fas. The numbers of duodenal CD3 + , alphabeta- and gammadelta-TCRs correlated with the serum granzyme and CD30 levels. CONCLUSION: The intestinal immune response in CMSE and CD is manifested by the increase in serum GrA, GrB, and CD30; the measurement of these markers provides a new practical and objective complementary means for diagnosis and assessment of treatment response. However, this has to be confirmed by more studies before routine use.

Adolescent↗

Infertility and celiac disease: do we need more than one serological marker?

OBJECTIVES: Celiac disease (CD) prevalence is higher in women with infertility. Our study aims were to evaluate the prevalence of undiagnosed CD in Arab infertile women and to explore the usefulness of using more than one serological marker in the diagnostic screening for CD in this population. METHODS: Women with unexplained infertility (n = 192) and age-matched healthy controls (n = 210) were prospectively enrolled. Serum was tested for human tissue transglutaminase antibodies (TTG), antiendomysial antibodies (EMA), and immunoglobulin A. Intestinal biopsy was offered to women with positive serology or immunoglobulin A (IgA) deficiency. RESULTS: CD was diagnosed in five infertile women (2.65%) and in one control (0.5%) (p = 0.11). Gastrointestinal complaints were present in 60% (three of five) of women with CD and 11.8% (22 of 187) of women without CD (p = 0.017). Anemia was reported in 80% of infertile women with CD and 4.8% of infertile women without CD (p = 0.0001). CONCLUSIONS: Undiagnosed CD is prevalent in Arab infertile women as well as in Arab women in general. CD in Arab infertile women is frequently associated with gastrointestinal complaints and anemia. EMA testing is sufficient in suspected cases.

Adolescent↗