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Pichi F, Aljneibi S, Neri P, Rishi P, Cicinelli MV, Introini U, Sharma S, Munk MR, Pasadhika S, Gonzalez-Lopez JJ, Lembo A, Nucci P, Choudhry N, Carreño E. Multimodal Imaging Study of Patients With Vitamin A Deficiency Retinopathy. Ophthalmic Surg Lasers Imaging Retina 2023; 54:330-336. [PMID: 37352397 DOI: 10.3928/23258160-20230513-01] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/25/2023]
Abstract
OBJECTIVES To describe multimodal imaging findings of vitamin A deficiency retinopathy. METHODS A retrospective study of patients with serum retinol < 0.3 mg/L. Fundus color photos, spectral domain-optical coherence tomography (SD-OCT), and fundus autofluorescence (FAF) were reviewed and, when available, electrophysiological tests were analyzed. RESULTS Forty-five eyes (63.9 ± 15.7 years) were included. Ultra-widefield fundus photography showed drusen-like deposits (53.3%) and macular retinal pigment epithelium (RPE) mottling (40%). The deposits were hypoautofluorescent, and a perifoveal hyperautofluorescent ring was present in 8.9%. By SD-OCT, the ellipsoid zone had an irregular appearance (100%) and conical deposits anterior to the RPE (33.3%). Electroretinogram (ERG) (66.7%) showed a decrease in b-wave in the scotopic registers, and microperimetry (4.4%) showed decreased foveal sensitivity. After vitamin A supplementation, SD-OCT and FAF showed resolution of all findings. Forty percent of eyes had restoration of the scotopic registers in ERG and improved macular sensitivity by microperimetry (4.4%). CONCLUSIONS Vitamin A deficiency causes a mild cone dysfunction in addition to the more severe absent rod response. [Ophthalmic Surg Lasers Imaging Retina 2023;54:330-336.].
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Maluf SW, Wilhelm Filho D, Parisotto EB, Medeiros GDSD, Pereira CHJ, Maraslis FT, Dornelles Schoeller CC, Rosa JSD, Fröde TS. DNA damage, oxidative stress, and inflammation in children with celiac disease. Genet Mol Biol 2020; 43:e20180390. [PMID: 32555942 PMCID: PMC7288666 DOI: 10.1590/1678-4685-gmb-2018-0390] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2019] [Accepted: 07/12/2019] [Indexed: 02/07/2023] Open
Abstract
The objective of this study was to evaluate the level of genomic instability in patients with celiac disease and to establish a relationship between inflammation, oxidative stress, and DNA damage in these patients. Myeloperoxidase (MPO) activity, adenosine deaminase, nitric oxide (NOx), thiobarbituric acid, catalase (CAT), superoxide dismutase (SOD), glutathione peroxidase (GPx), and DNA damage were evaluated in peripheral blood samples from 47 celiac disease patients and 31 controls. Patients with celiac disease presented higher levels of DNA damage in comparison to controls (p=0.023). This difference was also observed for markers of oxidative stress, such as CAT (p=0.011) and SOD (p=0.013), and inflammatory markers such as MPO (p < 0.001) and NOx (p=0.009). Positive correlations were found between DNA damage levels and the values of CAT (r=0.405; p=0.009) and SOD (r=0.516; p < 0.001). Positive correlations were also found between GPx and NOx (r=0.349; p=0.030) and MPO and NOx (r=0.239; p=0.039). CAT and NOx showed a negative correlation (r= −0.315; p=0.042). In conclusion, intestinal inflammation can have systemic effects, causing an imbalance between oxidant and antioxidant markers, which may promote increased levels of DNA damage.
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Affiliation(s)
- Sharbel Weidner Maluf
- Universidade Federal de Santa Catarina, Hospital Universitário, Laboratório de Genética, Florianópolis, SC, Brazil
| | - Danilo Wilhelm Filho
- Universidade Federal de Santa Catarina, Departamento de Ecologia e Zoologia, Florianópolis, SC, Brazil
| | - Eduardo Benedetti Parisotto
- Universidade Federal de Mato Grosso do Sul, Faculdade de Ciências Farmacêuticas, Alimentos e Nutrição, Campo Grande, MS, Brazil
| | | | | | - Flora Troina Maraslis
- Universidade Federal de Santa Catarina, Hospital Universitário, Laboratório de Genética, Florianópolis, SC, Brazil
| | | | - Julia Savan da Rosa
- Universidade Federal de Santa Catarina, Centro de Ciências de Saúde, Departamento de Análises Clínicas, Florianópolis, SC, Brazil
| | - Tânia Silvia Fröde
- Universidade Federal de Santa Catarina, Centro de Ciências de Saúde, Departamento de Análises Clínicas, Florianópolis, SC, Brazil
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Taggart MW, Foo WC, Lee SM. Tumors of the Gastrointestinal System Including the Pancreas. ONCOLOGICAL SURGICAL PATHOLOGY 2020:691-870. [DOI: 10.1007/978-3-319-96681-6_12] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/06/2025]
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Ciccocioppo R, Kruzliak P, Cangemi GC, Pohanka M, Betti E, Lauret E, Rodrigo L. The Spectrum of Differences between Childhood and Adulthood Celiac Disease. Nutrients 2015; 7:8733-51. [PMID: 26506381 PMCID: PMC4632446 DOI: 10.3390/nu7105426] [Citation(s) in RCA: 38] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2015] [Revised: 10/06/2015] [Accepted: 10/12/2015] [Indexed: 02/06/2023] Open
Abstract
An old saying states that ''children are not little adults" and this certainly holds true for celiac disease, as there are many peculiar aspects regarding its epidemiology, diagnosis, clinical presentations, associated diseases, and response to treatment in pediatric compared to adult populations, to such an extent that it merits a description of its own. In fact, contrary to the past when it was thought that celiac disease was a disorder predominantly affecting childhood and characterized by a malabsorption syndrome, nowadays it is well recognized that it affects also adult and elderly people with an impressive variability of clinical presentation. In general, the clinical guidelines for diagnosis recommend starting with specific serologic testing in all suspected subjects, including those suffering from extraintestinal related conditions, and performing upper endoscopy with appropriate biopsy sampling of duodenal mucosa in case of positivity. The latter may be omitted in young patients showing high titers of anti-transglutaminase antibodies. The subsequent management of a celiac patient differs substantially depending on the age at diagnosis and should be based on the important consideration that this is a lifelong condition.
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Affiliation(s)
- Rachele Ciccocioppo
- Rachele Ciccocioppo, Center for the Study and Cure of Celiac Disease, Clinica Medica I, Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, 19-27100 Pavia, Italy.
| | - Peter Kruzliak
- International Clinical Research Center, St. Anne's University Hospital and Masaryk University, 65691 Brno, Czech Republic.
| | - Giuseppina C Cangemi
- Rachele Ciccocioppo, Center for the Study and Cure of Celiac Disease, Clinica Medica I, Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, 19-27100 Pavia, Italy.
| | - Miroslav Pohanka
- Faculty of Military Health Sciences, University of Defence, Trebešská 1575-500 01 Hradec Kralove, Czech Republic.
- Department of Geology and Pedology, Faculty of Forestry and Wood Technology, Mendel University in Brno, 61300 Brno, Czech Republic.
| | - Elena Betti
- Rachele Ciccocioppo, Center for the Study and Cure of Celiac Disease, Clinica Medica I, Department of Internal Medicine, IRCCS Policlinico San Matteo Foundation, University of Pavia, 19-27100 Pavia, Italy.
| | - Eugenia Lauret
- Gastroenterology Unit, Hospital Universitario Central de Asturias, 33000 Oviedo, Spain.
| | - Luis Rodrigo
- Gastroenterology Unit, Hospital Universitario Central de Asturias, 33000 Oviedo, Spain.
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Biagi F, Marchese A, Ferretti F, Ciccocioppo R, Schiepatti A, Volta U, Caio G, Ciacci C, Zingone F, D'Odorico A, Carroccio A, Ambrosiano G, Mansueto P, Gasbarrini A, Piscaglia AC, Andrealli A, Astegiano M, Segato S, Neri M, Meggio A, de Pretis G, De Vitis I, Gobbi P, Corazza GR. A multicentre case control study on complicated coeliac disease: two different patterns of natural history, two different prognoses. BMC Gastroenterol 2014; 14:139. [PMID: 25103857 PMCID: PMC4127435 DOI: 10.1186/1471-230x-14-139] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/03/2014] [Accepted: 07/28/2014] [Indexed: 12/14/2022] Open
Abstract
Background Coeliac disease is a common enteropathy characterized by an increased mortality mainly due to its complications. The natural history of complicated coeliac disease is characterised by two different types of course: patients with a new diagnosis of coeliac disease that do not improve despite a strict gluten-free diet (type A cases) and previously diagnosed coeliac patients that initially improved on a gluten-free diet but then relapsed despite a strict diet (type B cases). Our aim was to study the prognosis and survival of A and B cases. Methods Clinical and laboratory data from coeliac patients who later developed complications (A and B cases) and sex- and age-matched coeliac patients who normally responded to a gluten-free diet (controls) were collected among 11 Italian centres. Results 87 cases and 136 controls were enrolled. Complications tended to occur rapidly after the diagnosis of coeliac disease and cumulative survival dropped in the first months after diagnosis of complicated coeliac disease. Thirty-seven cases died (30/59 in group A, 7/28 in group B). Type B cases presented an increased survival rate compared to A cases. Conclusions Complicated coeliac disease is an extremely serious condition with a high mortality and a short survival. Survival depends on the type of natural history.
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Affiliation(s)
- Federico Biagi
- Coeliac Centre/First Department of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, University of Pavia, P,le Golgi, 19, I-27100, Pavia, Italy.
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Brown AC. Gluten sensitivity: problems of an emerging condition separate from celiac disease. Expert Rev Gastroenterol Hepatol 2012; 6:43-55. [PMID: 22149581 DOI: 10.1586/egh.11.79] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Gluten sensitivity appears to be emerging as a separate condition from celiac disease, yet no clear definition or diagnosis exists. As a result, patients with gluten sensitivity experience delayed diagnosis and continuing symptoms if they consume gluten. This emerging medical problem may involve human genetics, plant genetic modifications, gluten as a food additive, environmental toxins, hormonal influences, intestinal infections and autoimmune diseases. The treatment is similar to that for celiac disease - a gluten-free diet. The use of a gluten-free diet or an elimination diet is encouraged in assisting people to determine whether or not they are gluten sensitive. It is time to not only recognize, but to treat and further research gluten sensitivity, as unconfirmed environmental factors continue to spread this problem further into the general population.
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Affiliation(s)
- Amy C Brown
- Department of Complementary and Alternative Medicine, John A Burns School of Medicine, University of Hawaii, 651 Ilalo Street, MEB 223, Honolulu, HI 96813, USA.
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Ferreri AJ, Zinzani PL, Govi S, Pileri SA. Enteropathy-associated T-cell lymphoma. Crit Rev Oncol Hematol 2011; 79:84-90. [DOI: 10.1016/j.critrevonc.2010.06.006] [Citation(s) in RCA: 44] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/23/2010] [Revised: 06/01/2010] [Accepted: 06/25/2010] [Indexed: 01/24/2023] Open
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Donnelly SC, Ellis HJ, Ciclitira PJ. Pharmacotherapy and management strategies for coeliac disease. Expert Opin Pharmacother 2011; 12:1731-44. [PMID: 21718231 DOI: 10.1517/14656566.2011.592140] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
INTRODUCTION Coeliac disease is a common disease that affects approximately 1% of Northern European and American populations. Evidence suggests it is caused by an inappropriate immune response in genetically susceptible patients to dietary gluten found in wheat, rye, barley and, in a small minority of patients, oats. Treatment involves a lifelong gluten-free diet. This diet limits nutritional variety and is costly and difficult to maintain. AREAS COVERED This review covers the current treatment options available and discusses novel emerging therapies for coeliac disease. EXPERT OPINION Novel therapies are still in early stages of development and therefore, at present, a gluten-free diet remains the treatment of choice in coeliac disease due to its low side-effect profile. A replacement for a gluten-free diet would be superior to an adjunct; in this case dietary modification of gluten may well have the least side effects, be tolerated by a wider group of coeliac patients and therefore be accepted. Search terms used: Pubmed, Medline and clinicaltrials.gov were searched with 'celiac disease' and 'therapy' as MESH terms. Patent database was searched using the term 'celiac disease'. Conference attendance at DDW Chicago 2011 and Columbia 2010 was also used to gain further information from conference abstracts.
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Affiliation(s)
- Suzanne C Donnelly
- King's College London, Division of Nutrition and Diabetes, The Rayne Institute, St Thomas' Hospital, Gastroenterology Laboratory, 4th Floor Lambeth Wing, London, SE1 7EH, UK
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Prasad Shanbhogue AK, Prasad SR, Jagirdar J, Takahashi N, Sandrasegaran K, Fazzio RT, Fidler JL. Comprehensive Update on Select Immune-Mediated Gastroenterocolitis Syndromes: Implications for Diagnosis and Management. Radiographics 2010; 30:1465-87. [DOI: 10.1148/rg.306105520] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
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Singhal M, Makharia G, Bakhshi S. Childhood chronic myeloid leukemia with celiac disease. Pediatr Blood Cancer 2010; 54:177. [PMID: 19731332 DOI: 10.1002/pbc.22270] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
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Cardona DM, Zhang X, Liu C. Loss of carbamoyl phosphate synthetase I in small-intestinal adenocarcinoma. Am J Clin Pathol 2009; 132:877-82. [PMID: 19926579 DOI: 10.1309/ajcp74xgrfwtflju] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022] Open
Abstract
Carbamoyl phosphate synthetase I (CPS1), normally found in hepatocytes and small-intestine (SI) enterocytes, is the antigen of Hep Par 1 antibody. Expression of CPS1 in invasive SI adenocarcinoma seems to be lost. We retrospectively collected 36 total specimens, which included 31 SI adenomas and 21 adenocarcinomas. We used 34 cases of duodenitis as a control group. Immunohistochemical and Western blot analyses were performed to determine CPS1 expression. The normal SI mucosa, all 34 cases of duodenitis, and all 29 adenomas with low-grade dysplasia demonstrated diffuse Hep Par 1 expression. Of the 21 invasive adenocarcinomas, 15 lost antigen expression (71%). These data are statistically significant (P < .05). Western blot analysis confirmed the immunohistochemical findings, with strong CPS1 expression within the normal mucosa and adenoma and complete loss in the invasive tumor. The differential expression of Hep Par 1 in dysplastic vs malignant tumors of the SI may be diagnostically useful in difficult cases.
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Garrido A, Luque A, Vázquez A, Hernández JM, Alcántara F, Márquez JL. [Primary small bowel neoplasms as a complication of celiac disease]. GASTROENTEROLOGIA Y HEPATOLOGIA 2009; 32:618-21. [PMID: 19625106 DOI: 10.1016/j.gastrohep.2009.05.003] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/12/2009] [Revised: 05/03/2009] [Accepted: 05/29/2009] [Indexed: 02/04/2023]
Abstract
Celiac disease is produced by gluten intake in genetically susceptible children and adults and is the most common severe food intolerance in western countries. Eliminating gluten from the diet is essential in these patients, since most of the complications that can occur are more frequent if there is lack of treatment adherence. The most serious complication in these patients is the development of neoplasms, the most frequent being enteropathy-associated T-cell lymphoma; however, an increase in the incidence of small bowel adenocarcinoma has also been described. We present two cases of small bowel carcinoma in patients with celiac disease, which were diagnosed at the onset of the disease.
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Affiliation(s)
- Antonio Garrido
- Servicio de Aparato Digestivo, Hospital General Virgen del Rocío, Sevilla, España.
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Stojiljković V, Todorović A, Pejić S, Kasapović J, Saicić ZS, Radlović N, Pajović SB. Antioxidant status and lipid peroxidation in small intestinal mucosa of children with celiac disease. Clin Biochem 2009; 42:1431-7. [PMID: 19560448 DOI: 10.1016/j.clinbiochem.2009.06.009] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2009] [Revised: 06/12/2009] [Accepted: 06/18/2009] [Indexed: 02/07/2023]
Abstract
OBJECTIVE To explain the role of oxidative stress in the pathology of celiac disease. DESIGN AND METHODS The activities of antioxidant enzymes and the levels of glutathione and lipid hydroperoxides were measured in the samples of small intestinal biopsies from 39 children with different forms of the disease and in 19 control subjects. RESULTS The activities of analyzed enzymes varied significantly between the examined groups. An increase in the activities of superoxide dismutase was observed in patients with active and silent celiac disease, while the activities of glutathione peroxidase and glutathione reductase and the glutathione content were significantly reduced. The level of lipid hydroperoxides was significantly elevated in these groups. CONCLUSIONS Oxidative stress is an important factor in the pathogenesis of celiac disease. The antioxidant capacity of celiac patients is significantly reduced, mostly by a depletion of glutathione. Natural antioxidants and appropriate dietary supplements could be important complements to the classic therapy of celiac disease.
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Affiliation(s)
- Vesna Stojiljković
- Laboratory of Molecular Biology and Endocrinology, Vinca Institute of Nuclear Sciences, University of Belgrade, Belgrade, Serbia.
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¿Se debe hacer cribado de enfermedad celíaca en la hepatitis crónica por el virus C? Med Clin (Barc) 2009; 132:603-4. [DOI: 10.1016/j.medcli.2008.05.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2008] [Accepted: 05/06/2008] [Indexed: 11/17/2022]
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Garrido A, Verdejo C, Luis Márquez J, Giráldez Á, Trigo C, Belda O. Linfoma intestinal y paniculitis mesentérica: complicaciones de una enfermedad celíaca no diagnosticada. GASTROENTEROLOGIA Y HEPATOLOGIA 2008; 31:221-4. [DOI: 10.1157/13117914] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
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Akram S, Murray JA, Pardi DS, Alexander GL, Schaffner JA, Russo PA, Abraham SC. Adult autoimmune enteropathy: Mayo Clinic Rochester experience. Clin Gastroenterol Hepatol 2007; 5:1282-90; quiz 1245. [PMID: 17683994 PMCID: PMC2128725 DOI: 10.1016/j.cgh.2007.05.013] [Citation(s) in RCA: 156] [Impact Index Per Article: 8.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND & AIMS Autoimmune enteropathy is a rare cause of intractable diarrhea associated with circulating gut autoantibodies and a predisposition to autoimmunity. It is rarely observed in adults, with only 11 cases reported to date. METHODS Fifteen adults with autoimmune enteropathy were identified at the Mayo Clinic, Rochester, from May 2001-June 2006. The demographic, clinical, and treatment data were abstracted from their records. RESULTS The study population was 87% white, 47% female, with median age of 55 years (interquartile range, 42-67 years). All patients had protracted diarrhea, weight loss, and malnutrition. Celiac disease was excluded by lack of response to gluten-free diet or absence of the celiac disease susceptibility HLA genotypes. Fourteen patients were tested for gut epithelial cell antibodies, and 93% were positive for anti-enterocyte and/or anti-goblet cell antibodies. Predisposition to autoimmune diseases was noted in 80%, as indicated by a variety of circulating autoantibodies. Small intestinal histopathologic findings included subtotal villous atrophy and lymphoplasmacytic infiltration in the lamina propria with relatively few surface intraepithelial lymphocytes. T-cell receptor gene rearrangement studies were negative in all cases. Immunosuppressive therapy was required in 93% of cases. Clinical improvement was noted in 60% after 1-8 weeks of steroid therapy. CONCLUSIONS Autoimmune enteropathy is a heterogeneous disease and should be considered in the differential diagnosis of malabsorption and small bowel villous atrophy. The presence of gut epithelial cell antibodies can help confirm the diagnosis. No single agent is unequivocally effective in inducing remission, and immunosuppressive therapy is required in most cases.
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Affiliation(s)
- Salma Akram
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN
| | - Joseph A. Murray
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN
| | - Darrell S. Pardi
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN
| | - Glenn L. Alexander
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN
| | - John A. Schaffner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, Mayo Clinic, Rochester, MN
| | - Pierre A. Russo
- Department of Pathology and Laboratory Medicine, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Susan C. Abraham
- Division of Anatomic Pathology, Department of Pathology, Mayo Clinic College of Medicine, Rochester, Minnesota
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Mallant M, Hadithi M, Al-Toma AB, Kater M, Jacobs M, Manoliu R, Mulder C, van Waesberghe JH. Abdominal computed tomography in refractory coeliac disease and enteropathy associated T-cell lymphoma. World J Gastroenterol 2007; 13:1696-700. [PMID: 17461472 PMCID: PMC4146948 DOI: 10.3748/wjg.v13.i11.1696] [Citation(s) in RCA: 47] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate computed tomography (CT) findings, useful to suggest the presence of refractory celiac disease (RCD) and enteropathy associated T cell lymphoma (EATL).
METHODS: Coeliac disease (CD) patients were divided into two groups. GroupI: uncomplicated CD (n = 14) and RCD typeI(n = 10). Group II: RCD type II (n = 15) and EATL (n = 7).
RESULTS: Both groups showed classic signs of CD on CT. Intussusception was seen in 1 patient in groupIvs 5 in group II (P = 0.06). Lymphadenopathy was seen in 5 patients in group II vs no patients in groupI(P = 0.01). Increased number of small mesenteric vessels was noted in 20 patients in groupIvs 11 in group II (P = 0.02). Eleven patients (50%) in group II had a splenic volume < 122 cm3vs 4 in groupI(14%), 10 patients in groupI had a splenic volume > 196 cm3 (66.7%) vs 5 in group II (33.3%) P = 0.028.
CONCLUSION: CT scan is a useful tool in discriminating between CD and (Pre) EATL. RCD II and EATL showed more bowel wall thickening, lymphadenopathy and intussusception, less increase in number of small mesenteric vessels and a smaller splenic volume compared with CD and RCDI.
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Affiliation(s)
- Maarten Mallant
- Department of Radiology, VU University Medical Center, Amsterdam, The Netherlands
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Abenavoli L, Proietti I, Leggio L, Ferrulli A, Vonghia L, Capizzi R, Rotoli M, Amerio PL, Gasbarrini G, Addolorato G. Cutaneous manifestations in celiac disease. World J Gastroenterol 2006; 12:843-52. [PMID: 16521210 PMCID: PMC4066147 DOI: 10.3748/wjg.v12.i6.843] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Celiac disease (CD) is an autoimmune gluten-dependent enteropathy characterized by atrophy of intestinal villi that improves after gluten-free diet (GFD). CD is often associated with extra-intestinal manifestations; among them, several skin diseases are described in CD patients. The present review reports all CD-associated skin manifestations described in the literature and tries to analyze the possible mechanisms involved in this association. The opportunity to evaluate the possible presence of CD in patients affected by skin disorders is discussed.
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Affiliation(s)
- L Abenavoli
- Institute of Internal Medicine, Catholic University, L.go Gemelli 8, 00168 Rome, Italy
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Abstract
Celiac disease is a complex autoimmune enteropathy that affects the small bowel in genetically predisposed individuals. It is thought that celiac disease is the result of an inappropriate T cell-mediated immune response against ingested gluten protein. The characteristic lesion of the small intestinal mucosa includes loss of absorptive villi and infiltration of the lamina propria with inflammatory cells. The clinical presentation of celiac disease varies greatly depending on patient's age, duration and extent of the disease, and the presence of extraintestinal manifestations. Unfortunately, most patients with celiac disease have either silent or atypical presentations, thus escaping diagnosis for several years. Medical nutrition therapy with lifelong adherence to a strict gluten-free diet is the only accepted treatment of celiac disease. Individuals at risk for this entity should undergo appropriate serologic testing, but there is no evidence to support mass screening.
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Affiliation(s)
- Nisha Chand
- Gastroenterology, Hepatology and Nutrition, Virginia Commonwealth University Medical Center, and the Division of Gastroenterology, Hepatology and Nutrition, Hunter Holmes McGuire Veterans Affairs Medical Center, Richmond, VA 23249, USA
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Abstract
Ulcerative jejunoileitis and enteropathy-associated T-cell lymphoma are rare conditions described in patients with refractory coeliac disease. Ulcerations affect the small bowel and are unrelated to drugs, ischaemia, infections or other known causes. We describe a female patient with an unclassified enteropathy who experienced several episodes of jejunoileal ulcerations. Several resections of the small bowel segments were necessary. The repetitive ulcerations were either from cytotoxic T cells, the patient developed a T-cell lymphoma, and malignant cells could be detected at the bottom of the ulcers, or from acid-producing cells in areas of gastric metaplasia. Two mechanisms might thus be responsible for the occurrence of repetitive ulceration, and require different treatment strategies. The patient is currently being treated with proton pump inhibitors, oral steroids and parenteral nutrition.
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Affiliation(s)
- Christoph Elsing
- Department of Gastroenterology and Surgery, St Elisabeth Hospital, Dorsten, Germany.
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Mino-Kenudson M, Brown I, Lauwers G. Histopathological diagnosis of gluten-sensitive enteropathy. ACTA ACUST UNITED AC 2005. [DOI: 10.1016/j.cdip.2005.05.002] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
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23
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Sonet A, Théate I, Delos M, Montfort L, Mineur P, Driesschaert P, Michaux L, Ferrant A, Bosly A. Clinical and pathological features of 14 non-Hodgkin's lymphomas associated with coeliac disease. Acta Clin Belg 2004; 59:143-51. [PMID: 15462511 DOI: 10.1179/acb.2004.021] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
BACKGROUND It is well established that enteropathy associated T-cell lymphoma is associated with malabsorption which is due to gluten sensitivity (coeliac disease). Our study was performed to define the clinical features, histological subtypes, response to treatment, and outcome of the association of coeliac disease and T-cell lymphoma. PATIENTS AND METHODS A retrospective study was performed in the UCL Group of Hematology to collect data on patients with a diagnosis of non-Hodgkin's lymphoma and coeliac disease. Fifteen cases were observed between 1985 and 1999. Case records for all but one patient were available and the pathological specimens of 14 patients were reviewed by two pathologists. RESULTS Six previously diagnosed coeliac patients developed lymphoma; interval between coeliac symptoms and onset of the lymphoma ranged from 2 to 48 years (median 16 years). Five patients had coeliac disease and non-Hodgkin's lymphoma diagnosed concomitantly or less than 6 months before the symptoms leading to the diagnosis of lymphoma. Three patients had the diagnosis of coeliac disease after lymphoma diagnosis (1, 8 and 10 years later respectively). Ten non-Hodgkin's lymphomas were of T-cell origin and 4 were B-cell lymphomas. Eight out of 14 presented on a surgical emergency. Thirteen were treated using chemotherapy. The median survival from the diagnosis of enteropathy associated T-cell lymphoma was 12 months (range 1-126). CONCLUSIONS Lymphomas associated with coeliac disease are heterogeneous and their diagnosis is difficult. The enteropathy-associated T-cell lymphoma is the most frequent, aggressive and fatal complication of coeliac disease but it is not rare to observe association with B-cell lymphoma. Chemotherapy is highly toxic in those patients. Despite a poor prognosis, long-term survival can be expected in a fraction of these patients.
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Affiliation(s)
- A Sonet
- Groupe d'Hématologie de l'UCL, Département d'Hematologie, Université Catholique de Louvain, Belgium.
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Nonami A, Yokoyama T, Takeshita M, Ohshima K, Kubota A, Okamura S. Human herpes virus 8-negative primary effusion lymphoma (PEL) in a patient after repeated chylous ascites and chylothorax. Intern Med 2004; 43:236-42. [PMID: 15098608 DOI: 10.2169/internalmedicine.43.236] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
We describe a case of malignant lymphoma which presented in the body cavities without identifiable tumor masses. Malignant lymphoma cells showed strong atypia with prominent nuclei and basophilic cytoplasm containing vacuoles. The chromosomes showed diploidy and complex abnormalities including translocations and deletions. We diagnosed this patient with primary effusion lymphoma (PEL), even though she tested negative for human herpes virus-8 (HHV-8) which has been suggested to be causally related to PEL. Interestingly, the patient also showed complicated protein-losing enteropathy, and PEL occurred after repeated chylous ascites and chylothorax. The possible pathogenesis of this rare disease is discussed here.
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Affiliation(s)
- Atsushi Nonami
- Department of Hematology, National Kyushu Medical Center, Fukuoka
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Abstract
Celiac disease (CD) has a wide range of clinical presentations and is being diagnosed with increasing frequency in patients in the 4th and 5th decades of life. The diagnosis of CD is confirmed by a combination of clinical, serological, and morphological findings associated with a response to a gluten-free diet. In small-bowel mucosal biopsy specimens, abnormalities range from minimal (increased villous intraepithelial lymphocytes only) to severe (complete villous blunting with crypt hyperplasia). Recognition of CD is important because appropriate therapy of this condition will obviate the risk of its severe complications.
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Affiliation(s)
- Donald A Antonioli
- Department of Pathology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02215, USA.
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26
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Múgica F, Sesplugues R, Torrado J, Aranzadi MJ, Pérez Cámara B, Recasens M, Muñagorri A. [Tuberculosis, another cause of generalized lymphadenopathies in celiac disease]. GASTROENTEROLOGIA Y HEPATOLOGIA 2002; 25:432-3. [PMID: 12069707 DOI: 10.1016/s0210-5705(02)70278-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Abstract
Aim of this review is to describe the extremely variable clinical presentation of coeliac disease. Moreover due to these varying manifestations, "coeliac literature" is characterised by a very rich terminology, which has not been unified, so far To help readers, not familiar with that terminology, we have given an explanation for the most common coeliac terms.
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Affiliation(s)
- F Biagi
- Gastroenterology Unit, IPCCS Policlinico San Matteo, Pavia, Italy.
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28
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Martini S, Mengozzi G, Aimo G, Pagni R, Sategna-Guidetti C. Diagnostic Accuracies for Celiac Disease of Four Tissue Transglutaminase Autoantibody Tests Using Human Antigen. Clin Chem 2001. [DOI: 10.1093/clinchem/47.9.1722] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
Affiliation(s)
- Silvia Martini
- Dipartimento di Medicina Interna, Università di Torino, 10126 Torino, Italy
| | - Giulio Mengozzi
- Unità Operativa Autonoma Laboratorio Analisi Chimico-Cliniche, Azienda Ospedaliera San Giovanni Battista, Corso Bramante, 88, 10126 Torino, Italy
| | - Giuseppe Aimo
- Unità Operativa Autonoma Laboratorio Analisi Chimico-Cliniche, Azienda Ospedaliera San Giovanni Battista, Corso Bramante, 88, 10126 Torino, Italy
| | - Roberto Pagni
- Unità Operativa Autonoma Laboratorio Analisi Chimico-Cliniche, Azienda Ospedaliera San Giovanni Battista, Corso Bramante, 88, 10126 Torino, Italy
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29
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Corrao G, Corazza GR, Bagnardi V, Brusco G, Ciacci C, Cottone M, Sategna Guidetti C, Usai P, Cesari P, Pelli MA, Loperfido S, Volta U, Calabró A, Certo M. Mortality in patients with coeliac disease and their relatives: a cohort study. Lancet 2001; 358:356-61. [PMID: 11502314 DOI: 10.1016/s0140-6736(01)05554-4] [Citation(s) in RCA: 383] [Impact Index Per Article: 16.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Although previous studies have shown increased mortality in patients with coeliac disease and their relatives, no data are available in relation to different patterns of clinical presentation. We assessed mortality in patients with coeliac disease and their first-degree relatives. METHODS We enrolled, in a prospective cohort study, 1072 adult patients with coeliac disease consecutively diagnosed in 11 gastroenterology units between 1962 and 1994, and their 3384 first-degree relatives. We compared the number of deaths up to 1998 with expected deaths and expressed the comparison as standardised mortality ratio (SMR) and relative survival ratio. FINDINGS 53 coeliac patients died compared with 25.9 expected deaths (SMR 2.0 [95% CI 1.5-2.7]). A significant excess of mortality was evident during the first 3 years after diagnosis of coeliac disease and in patients who presented with malabsorption symptoms (2.5 [1.8-3.4]), but not in those diagnosed because of minor symptoms (1.1 [0.5-2.2]) or because of antibody screening (1.2 [0.1-7.0]). SMR increased with increasing delay in diagnosis and for patients with poor compliance with gluten-free diet. Non-Hodgkin lymphoma was the main cause of death. No excess of deaths was recorded in relatives with coeliac disease. INTERPRETATION Prompt and strict dietary treatment decreases mortality in coeliac patients. Prospective studies are needed to clarify the progression of mild or symptomless coeliac disease and its relation to intestinal lymphoma.
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Affiliation(s)
- G Corrao
- Cattedra di Statistica Medica, Università di Milano-Bicocca, 20126, Milano, Italy.
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Ozge A, Karakelle A, Kaleağasi H. Celiac disease associated with recurrent stroke: a coincidence or cerebral vasculitis? Eur J Neurol 2001; 8:373-4. [PMID: 11422441 DOI: 10.1046/j.1468-1331.2001.00233.x] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
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Abstract
OBJECTIVE The diagnosis of coeliac disease (CD) is based on the responsiveness of the enteropathy to a gluten-free diet (GFD). This implies that terms such as 'non-responsive CD' and 'refractory CD' are almost paradoxical. In spite of this, these terms are commonly used in the literature, often with different and confusing meanings. METHODS On the basis of both a review of the literature and our clinical experience, we propose the following classification. A condition characterized by a refractory enteropathy, not due to lymphoma, ulcerative jejunoileitis or collagenous sprue, but in which gluten sensitivity has been shown previously or could be shown while the patients were on an immunosuppressive therapy should be indicated as refractory CD. Those patients in whom gluten sensitivity can be excluded should be considered to be affected by non-coeliac refractory sprue. Finally, patients in whom the presence of CD cannot be either confirmed or excluded should be considered to be affected by undefined sprue. RESULTS Twenty-four certain refractory patients are described in the literature. The data suggest a diagnosis of refractory CD in 13 patients, non-coeliac refractory sprue in three patients, and undefined sprue in eight patients. CONCLUSIONS We define refractory CD as a form of CD that no longer responds to a GFD. Non-coeliac refractory sprue is a condition unrelated to CD. It could be either an independent condition or a common end point of different enteropathies.
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Affiliation(s)
- F Biagi
- Gastroenterology Unit, University of Pavia, IRCCS Policlinico San Matteo, Italy.
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Ruskoné-Fourmestraux A, Rambaud JC. Gastrointestinal lymphoma: prevention and treatment of early lesions. Best Pract Res Clin Gastroenterol 2001; 15:337-54. [PMID: 11355919 DOI: 10.1053/bega.2000.0177] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Gastrointestinal lymphomas comprise a group of distinct clinicopathological entities. Differences in lifestyle and environmental factors between countries could account for the variety in the distribution of the main subtypes: low-grade B-cell lymphomas of the mucosa-associated lymphoid tissue type, alpha-chain disease and enteropathy (coeliac disease)-associated T-cell lymphoma (EATL). The possibility of preventing these lymphomas implies a knowledge of their natural history together with an identification of potential predisposing factors. The development of the lymphoid hyperplasia and subsequently low-grade lymphoma with the possibility of high-grade transformation is a multifactorial process involving both antigenic and host-related factors. The pathogenic role of Helicobacter pylori and gluten has been demonstrated in gastric lymphoma and enteropathy-associated T-cell lymphoma respectively, while environmental factors, especially non-specific bacterial ones, may play a major role in the pathogenesis of alpha-chain disease. The most difficult task in preventing these lymphomas is the recognition of early lesions likely to regress after the removal of the exogenous stimulus.
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MESH Headings
- Antineoplastic Combined Chemotherapy Protocols/administration & dosage
- Combined Modality Therapy
- Female
- Gastrointestinal Neoplasms/complications
- Gastrointestinal Neoplasms/diagnosis
- Gastrointestinal Neoplasms/mortality
- Gastrointestinal Neoplasms/prevention & control
- Humans
- Lymphoma, B-Cell, Marginal Zone/complications
- Lymphoma, B-Cell, Marginal Zone/diagnosis
- Lymphoma, B-Cell, Marginal Zone/mortality
- Lymphoma, B-Cell, Marginal Zone/prevention & control
- Lymphoma, Non-Hodgkin/complications
- Lymphoma, Non-Hodgkin/diagnosis
- Lymphoma, Non-Hodgkin/mortality
- Lymphoma, Non-Hodgkin/prevention & control
- Male
- Mass Screening/methods
- Precancerous Conditions/diagnosis
- Prognosis
- Severity of Illness Index
- Survival Rate
- Treatment Outcome
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Affiliation(s)
- A Ruskoné-Fourmestraux
- Service de Gastroentérologie, Hôtel Dieu, 1, Place Parvis Notre Dame, Paris, cedex 04, 75181, France
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Araya M, Mondragón A, Pérez-Bravo F, Roessler JL, Alarcón T, Rios G, Bergenfreid C. Celiac disease in a Chilean population carrying Amerindian traits. J Pediatr Gastroenterol Nutr 2000; 31:381-6. [PMID: 11045834 DOI: 10.1097/00005176-200010000-00010] [Citation(s) in RCA: 33] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
BACKGROUND Although clinical manifestations of celiac disease may change throughout life, clinical, histologic, immunologic, and genetic studies show that there are incomplete forms of this condition, making it difficult to define the disease at a given moment. Because there is no information published in the Latin American-Amerindian population, this study was conducted to assess relations between these parameters in Chileans with celiac disease and their first-degree relatives. METHODS Sixty-two persons with confirmed celiac disease (mean age, 17.9 +/- 5.1 years; 78.3% females) and 126 relatives (mean age, 27.9 +/- 17.2 years; 65.1% females) were evaluated. Clinical manifestations, antiendomysial antibodies (EMAs), and human leukocyte antigen (HLA) haplotypes were studied in patients. Additionally, jejunal biopsy specimens were assessed (light microscopy) in EMA-positive (EMA+) relatives. RESULTS Of the patients, 24.1% adhered to a strict gluten-free diet; 26% were oligosymptomatic, and none were malnourished; 45% were EMA+; 13.8% who ingested gluten were EMA-negative (EMA-); one patient consuming a strict gluten-free diet was EMA+. The DQA1*0501 allele was present in the highest frequency (48%, P < 0.0005), whereas combinations of DQ8 were predominant. Of the relatives, 4.8% were EMA+; they had a significantly higher frequency of diarrhea, weight loss, and anorexia (P < 0.03); and all had abnormal histology in biopsy specimens. CONCLUSIONS After childhood, celiac disease is oligosymptomatic and is often unrecognized by patients. Disease in 13.8% of patients and in 4.8% relatives appeared as incomplete forms of celiac disease. Predominance of DQ8 HLA haplotypes reflects the genetic Spanish-Mapuche heritage of this population.
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Affiliation(s)
- M Araya
- Human Nutrition/Clinical Nutrition Department, Institute of Nutrition and Food Technology, University of Chile, Santiago
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34
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Biagi F, Lorenzini P, Corazza GR. Literature review on the clinical relationship between ulcerative jejunoileitis, coeliac disease, and enteropathy-associated T-cell. Scand J Gastroenterol 2000; 35:785-90. [PMID: 10994614 DOI: 10.1080/003655200750023129] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Affiliation(s)
- F Biagi
- Gastroenterology Unit, IRCCS Policlinico San Matteo, University of Pavia, Italy
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35
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Patey-Mariaud De Serre N, Cellier C, Jabri B, Delabesse E, Verkarre V, Roche B, Lavergne A, Brière J, Mauvieux L, Leborgne M, Barbier JP, Modigliani R, Matuchansky C, MacIntyre E, Cerf-Bensussan N, Brousse N. Distinction between coeliac disease and refractory sprue: a simple immunohistochemical method. Histopathology 2000; 37:70-7. [PMID: 10931221 DOI: 10.1046/j.1365-2559.2000.00926.x] [Citation(s) in RCA: 121] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
AIMS We recently showed that refractory sprue is distinct from coeliac disease, the former being characterized by abnormal intraepithelial T-lymphocytes expressing a cytoplasmic CD3 chain (CD3c), lacking CD3 and CD8 surface expression, and showing TCRgamma gene rearrangements. To take advantage of the abnormal phenotype of CD3c + CD8 - intraepithelial lymphocytes (IEL) in refractory sprue we developed a simple method to distinguish coeliac disease from refractory sprue. METHODS AND RESULTS Comparative immunohistochemical studies using anti-CD3 and anti-CD8 antibodies were applied on paraffin-embedded and frozen biopsy specimens in refractory sprue (n = 6), coeliac disease (n = 10), healthy controls (n = 5) and suspected refractory sprue (n = 6). Comparable results were obtained on fixed and frozen biopsy specimens. In four of the six patients with suspected refractory sprue, abnormal CD3c + CD8 - IEL and TCRgamma gene rearrangements were found, as in refractory sprue; the remaining two patients had normal (CD3 + CD8 +) IEL and no TCRgamma gene rearrangements. Both patients had coeliac disease, as one failed to comply with a gluten-free diet, while the other was a slow responder. CONCLUSION This simplified immunostaining method using anti-CD3 and anti-CD8 antibodies on paraffin sections can distinguish active coeliac disease from refractory sprue and should prove useful in clinical practice.
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Affiliation(s)
- N Patey-Mariaud De Serre
- Department of Pathology and Université René Descartes-Paris V (EA 219), INSERM E 9925, Paris, France
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Dolor abdominal y diarrea en un varón de 33 años. Med Clin (Barc) 2000. [DOI: 10.1016/s0025-7753(00)71530-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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Rostami K, Kerckhaert J, Tiemessen R, von Blomberg BM, Meijer JW, Mulder CJ. Sensitivity of antiendomysium and antigliadin antibodies in untreated celiac disease: disappointing in clinical practice. Am J Gastroenterol 1999; 94:888-94. [PMID: 10201452 DOI: 10.1111/j.1572-0241.1999.983_f.x] [Citation(s) in RCA: 321] [Impact Index Per Article: 12.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
OBJECTIVE We have undertaken a study to assess the efficiency of serological tests in the diagnosis of celiac disease (CD) during the period January 1, 1994 to January 7, 1997. Our aim was to evaluate the sensitivity of IgA antiendomysium (EMA) and IgA antigliadin (AGA) with regard to the degree of histological abnormality in biopsy specimens of small intestine in untreated celiac disease patients and first-degree relatives. METHODS The study population comprised 101 cases: 85 untreated celiac patients and 16 first-degree relatives with a mean age of 42 yr (range, 2-76 yrs). Sixteen of 85 were excluded from study because they did not satisfy the study or diagnostic criteria of CD. EMA and AGA have been compared with the degree of villous atrophy (VA) in 69 celiac patients and 16 relatives according to the Marsh criteria of 1992. We divided the Marsh III histology into three subgroups as follows: Marsh IIIa (partial VA), Marsh IIIb (subtotal VA), and Marsh IIIc (total villous atrophy). RESULTS The specificity and positive predictive value of EMA for CD was excellent, because all EMA-positive patients (n = 42) were diagnosed with CD. The sensitivity of EMA, however, differed between CD subgroups; in patients with total VA, the sensitivity of EMA was 100% (17/17). However, in patients with partial VA (Marsh IIIa), the sensitivity of EMA was disappointing, only 9/29 (31%). Three of 72 celiacs with Marsh IIIb and Marsh IIIc had IgA deficiency and were excluded from the study. Elevated AGA has been detected in the sera of 39 of 69 (62%) patients. A combination of EMA and AGA tests showed a sensitivity of 76% (53/69). None of 16 first-degree relatives with Marsh I-II had positive EMA. CONCLUSIONS Interpretation of negative serology needs great awareness. Although EMA sensitivity in total villous atrophy is excellent, in partial villous atrophy the sensitivity of EMA appears to be disappointing. Our experience shows that EMA and AGA have only limited value in screening programs for CD.
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Affiliation(s)
- K Rostami
- Department of Hepatogastroenterology, Rinjstate Hospital, Arnhem, The Netherlands
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Gogos CA, Nikolopoulou V, Zolota V, Siampi V, Vagenakis A. Autoimmune cholangitis in a patient with celiac disease: a case report and review of the literature. J Hepatol 1999; 30:321-4. [PMID: 10068113 DOI: 10.1016/s0168-8278(99)80079-8] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Autoimmune cholangitis is a rare chronic cholestatic liver disease. We describe the case of a 65-year-old woman with celiac disease who presented to us with fever, jaundice and weight loss. Serum biochemical study showed marked increase in alkaline phosphatase and gammaGT levels. Antinuclear antibodies were positive, while antimitochondrial and anti-smooth-muscle antibodies were negative. Liver biopsy was compatible with primary autoimmune cholangitis. The patient was successfully treated with azathioprine and methylprednisolone. We describe here the uncommon association of autoimmune cholangitis with celiac disease and review the prevalence of liver diseases in patients with celiac disease.
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Affiliation(s)
- C A Gogos
- Department of Medicine, Patras University Medical School, Greece.
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39
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Papke J, Raude E. [Recurrent tetany as the first symptom of late manifesting celiac disease]. MEDIZINISCHE KLINIK (MUNICH, GERMANY : 1983) 1998; 93:619-23. [PMID: 9849053 DOI: 10.1007/bf03042677] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
BACKGROUND The diagnostic classification of a malabsorption is often made more difficult, particularly in oligosymptomatic forms. CASE REPORT A female patient is presented, where the diagnosis of malabsorption syndrome was concluded because of recurring tetanias. This could be traced back to an oligosymptomatic celiac disease. Diagnostic course of action, differential diagnosis as well as further observation during therapy are discussed. CONCLUSION Oligosymptomatic developments of celiac disease are common amongst adults and present a diagnostic challenge. The existence of a malabsorption should be considered, even if the momentary individual symptoms are unclarified.
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Affiliation(s)
- J Papke
- Praxis für Innere Medizin, Neustadt in Sachsen, Ludwigsburg
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40
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Affiliation(s)
- F Biagi
- Department of Medicine, University of Bologna, Italy
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41
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Cellier C, Patey N, Mauvieux L, Jabri B, Delabesse E, Cervoni JP, Burtin ML, Guy-Grand D, Bouhnik Y, Modigliani R, Barbier JP, Macintyre E, Brousse N, Cerf-Bensussan N. Abnormal intestinal intraepithelial lymphocytes in refractory sprue. Gastroenterology 1998; 114:471-81. [PMID: 9496937 DOI: 10.1016/s0016-5085(98)70530-x] [Citation(s) in RCA: 238] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
BACKGROUND & AIMS The etiology of refractory sprue is unclear. To gain insight into its pathogenesis, the phenotype and T-cell receptor (TCR) gene rearrangement status of intestinal lymphocytes were analyzed in a group of patients with clinical or biological features of celiac disease but either initially or subsequently refractory to a gluten-free diet. METHODS Intestinal biopsy specimens were obtained from 26 adults: 6 patients with refractory sprue, 7 patients with active celiac disease, and 13 normal controls. The phenotype of intestinal lymphocytes was studied by immunohistochemistry and, in 3 patients with refractory sprue, by cytometry of lymphocytes purified from intestinal biopsy specimens. TCR rearrangements were assessed by studying TCRgammaV-J junctional regions from DNA extracted from intestinal biopsy specimens and purified intestinal lymphocytes. RESULTS In the 6 patients with refractory sprue, but not in normal controls or patients with active celiac disease, the intestinal epithelium was massively infiltrated by small lymphocytes that lacked CD8, CD4, and TCR, contained intracytoplasmic but not surface CD3epsilon chains, and exhibited restricted TCRgamma gene rearrangements. CONCLUSIONS Refractory sprue is associated with an abnormal subset of intraepithelial lymphocytes containing CD3epsilon and restricted rearrangements of the TCRgamma chain but lacking surface expression of T-cell receptors.
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Affiliation(s)
- C Cellier
- Department of Gastroenterology, Hôpital Laënnec, Paris, France
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Koberstein B, Wedell J, Balzer K. [Acute abdomen in endemic sprue--a rare complication]. MEDIZINISCHE KLINIK (MUNICH, GERMANY : 1983) 1998; 93:43-6. [PMID: 9505079 DOI: 10.1007/bf03045040] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
BACKGROUND The celiac sprue, a small intestinal disease usually becoming apparent by a malabsorption syndrome, is caused by gluten respectively gliadin intolerance which leads to intestinal mucosal damage finally resulting in complete villous atrophy. CASE REPORT A propos of the case of a 60-year old woman with an uneventful course of celiac sprue of many years we report about a rare complication of this disease. Investigating recurrent attacks of abdominal pain a barium contrast examination revealed an intestinal stenosis which clinically resulted in an acute abdomen finally requiring surgery. Histologically a chronic ulcerative jejunoileitis was found. The main differential diagnosis is a malignant intestinal lymphoma, this however couldn't be found. Regarding this differential diagnosis surgical removal of the diseased parts of the bowel is the therapy of choice. Because of the possible transition from ulcerative jejunoileitis to intestinal lymphoma a postoperative close follow-up is recommended.
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43
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Biagi F, Ellis HJ, Morris MA, Ciclitira PJ. The illness of Gerard Manley Hopkins. Lancet 1997; 349:1775-6. [PMID: 9193410 DOI: 10.1016/s0140-6736(05)63000-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
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