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4.
Pol J Pathol ; 70(3): 155-161, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31820858

RESUMO

Organic basis of gastrointestinal symptoms is in the scope of many specialists. In this article lymphocytic gastritis, relatively newly described and not widely-known entity is presented. The lesion is characterized by presence of numerous mature lymphocytes in the surface and foveolar epithelium, as well as lymphocytic infiltration of the lamina propria. According to the definition at least 25 lymphocytes per 100 gastric epithelial cells is now required for the diagnosis. Literature found in wide range of databases was searched for morphological features of lymphocytic gastritis and its relationship with others coexisting or predisposing conditions or lesions. A strong positive correlation between celiac disease and Helicobacter pylori infection, and occurrence and severity of lymphocytic gastritis was revealed. A relationship was also found between lymphocytic gastritis and gastric lymphomas and other conditions.


Assuntos
Doença Celíaca/complicações , Gastrite/patologia , Infecções por Helicobacter/complicações , Linfócitos , Mucosa Gástrica/patologia , Humanos
5.
Maturitas ; 130: 41-49, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31706435

RESUMO

Turner syndrome is one of the most common sex chromosomal anomalies, characterized by the complete or partial loss of one X chromosome. Females with Turner syndrome are characterized by skeletal abnormalities, short stature and primary ovarian insufficiency. The aim of this narrative review was to identify the underlying mechanisms of osteoporosis in Turner syndrome, summarize its clinical manifestations and provide suggestions regarding the management of osteoporosis. Girls and women with Turner syndrome have lower bone mineral density and a higher fracture rate than healthy individuals. The most important risk factors for osteoporosis are inadequately treated primary ovarian insufficiency, followed by intrinsic bone abnormalities. Comorbidities that further increase the risk of osteoporosis include vitamin D deficiency, celiac disease and inflammatory bowel disease. In addition, hearing problems can predispose to falls. Early initiation of hormone replacement therapy (HRT) at the age of 11-13 years, prompt titration to the adult dose after 2 years and long-term follow-up to ensure compliance with HRT are the cornerstones of osteoporosis prevention in women with Turner syndrome.


Assuntos
Terapia de Reposição Hormonal , Osteoporose/etiologia , Osteoporose/prevenção & controle , Síndrome de Turner/complicações , Densidade Óssea , Osso e Ossos/anormalidades , Doença Celíaca/complicações , Feminino , Fraturas Ósseas/etiologia , Fraturas Ósseas/prevenção & controle , Humanos , Doenças Inflamatórias Intestinais/complicações , Menopausa Precoce , Insuficiência Ovariana Primária/complicações , Síndrome de Turner/diagnóstico , Síndrome de Turner/tratamento farmacológico , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/tratamento farmacológico
6.
Transplant Proc ; 51(9): 3155-3158, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31611125

RESUMO

We present a case report of a boy diagnosed with both chronic granulomatous disease (CGD) and familial celiac disease (CD) who underwent cord blood transplantation from a partially matched sibling donor. The presentation of CD resembled Crohn-like enteropathy, which is a canonical manifestation of CGD. Nearly 1 year post-hematopoietic stem cell transplantation (HSCT), a gluten-containing diet was reintroduced, and no reappearance of clinical, serologic, or histologic markers of CD was observed. The relatively high incidence of rare genetic diseases in pediatric patients suggests the need for additional caution in the interpretation of symptoms mimicking already known hallmarks of more common conditions. In addition, the presented data confirm the previous rare observations that allogeneic HSCT leads to durable induction of gluten tolerance in patients with CD, which can warrant its use in patients with refractory subtypes of CD.


Assuntos
Doença Celíaca/complicações , Transplante de Células-Tronco de Sangue do Cordão Umbilical/métodos , Doença Granulomatosa Crônica/complicações , Doença Granulomatosa Crônica/cirurgia , Doença Celíaca/genética , Criança , Pré-Escolar , Doença Granulomatosa Crônica/genética , Humanos , Lactente , Masculino , Irmãos , Transplante Homólogo
7.
Hautarzt ; 70(12): 960-963, 2019 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-31584112

RESUMO

Coeliac disease and vitiligo are immune-mediated disorders that are often associated with other immune-mediated disorders. In a prospective study we included 174 patients with vitiligo between the ages of 3 and 79 years (mean 38.2 years) to investigate whether there is an increased risk for coeliac disease in patients with vitiligo. We determined immunoglobulin A and IgA- and IgG-antibodies against tissue transglutaminase, while also optionally measuring blood count, ferritin, and endomysial-IgA-antibodies. In 3 of 174 (1.7%) vitiligo patients, coeliac disease was diagnosed serologically and by duodenal biopsy. Assuming a coeliac disease prevalence of less than 0.0033%, the incidence is statistically significant. In two other patients with vitiligo, coeliac disease was already known and confirmed with biopsy. If these two patients are included in the calculation, 2.8% (5 von 176) of vitiligo patients have coeliac disease. This value is statistically significant even with a higher coeliac disease prevalence of 0.01. Thus, it is recommended that celiac-disease-specific antibodies also be determined during routine blood workup in vitiligo patients. In case of positive results, a gastroduodenoscopy with biopsy of the small intestine is recommended for diagnosis confirmation. If celiac disease is unlikely, a trial of gluten-free diet for a specific time should nevertheless be discussed with individuals affected by vitiligo because repigmentation appears possible.


Assuntos
Doença Celíaca , Vitiligo , Adolescente , Adulto , Idoso , Autoanticorpos , Doença Celíaca/complicações , Doença Celíaca/epidemiologia , Doença Celíaca/imunologia , Criança , Pré-Escolar , Humanos , Imunoglobulina A , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Vitiligo/complicações , Vitiligo/epidemiologia , Vitiligo/imunologia , Adulto Jovem
10.
Medicina (Kaunas) ; 55(9)2019 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-31505858

RESUMO

Celiac disease (CD) is an autoimmune enteropathy that primarily affects the small intestine and is characterized by atrophy of intestinal villi. The manifestations of the disease improve following a gluten-free diet (GFD). CD is associated with various extra-intestinal diseases. Several skin manifestations are described in CD patients. The present paper reviews all CD-associated skin diseases reported in the literature and tries to analyze the pathogenic mechanisms possibly involved in these associations. Different hypotheses have been proposed to explain the possible mechanisms involved in every association between CD and cutaneous manifestations. An abnormal small intestinal permeability seems to be implicated in various dermatological manifestations. However, most of the associations between CD and cutaneous diseases is based on case reports and case series and a few controlled studies. To better assess the real involvement of the cutaneous district in CD patients, large multicentric controlled clinical trials are required.


Assuntos
Doença Celíaca/patologia , Pele/patologia , Doença Celíaca/complicações , Humanos , Dermatopatias/etiologia , Dermatopatias/patologia
11.
Int Arch Allergy Immunol ; 180(4): 250-254, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31522183

RESUMO

INTRODUCTION: Chronic spontaneous urticaria (CSU) in childhood affects the quality of life of the patient and may be associated with other autoimmune diseases. The aim of this study was to investigate the association of autoimmune diseases with CSU in children. METHODS: In a 3-year period, from 2015 to 2018, forty-nine children were diagnosed with CSU and monitored in the Outpatient Pediatric Allergy Clinic of the University Hospital of Ioannina in Northwestern Greece. The comorbidity with other autoimmune diseases was investigated in this population by autoantibody evaluation. RESULTS: Of the 49 children with CSU, 1 had autoantibodies for celiac disease (CD), which was confirmed by duodenal biopsy via gastroscopy. Four children had high serum levels of anti-thyroid peroxidase antibodies but normal thyroid function. No other specific autoantibodies were detected. CONCLUSION: The prevalence of autoimmune diseases among our children with CSU was low. Nevertheless, we think it is important to test children with CSU for other autoimmune diseases. CD can be diagnosed in children with CSU even in the absence of other indicative signs.


Assuntos
Autoanticorpos/sangue , Doenças Autoimunes/imunologia , Doença Celíaca/sangue , Imunoglobulina E/sangue , Adolescente , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doença Celíaca/complicações , Doença Celíaca/diagnóstico , Criança , Pré-Escolar , /patologia , Comorbidade , Feminino , Humanos , Lactente , Masculino , Mastócitos/imunologia
13.
G Chir ; 40(3): 225-229, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31484013

RESUMO

INTRODUCTION: Jejunal adenocarcinoma is a very rare disease but the frequency of this rare carcinoma is higher in celiac patients. We report the first case report of a second jejunal loop adenocarcinoma associated with celiac disease. PRESENTATION OF CASE: A 47-year-old woman, with a history of celiac disease. Computerized tomographic scans of the abdomen and pelvis demonstrated a severe retroperitoneal lymphoadenopathy, para-aortic, inter-aorto-caval, porto-caval, posterior pancreaticoduodenal space, celiac trunk, lesser gastric curvature, lymph node grouping. The patient underwent digiunal resection and regional lymphadenectomy. Diagnosis was poorly differentiated jejunal adenocarcinoma, infiltrating subserosal adipose tissue, metastasing in five out of eight regional lymph nodes. U.I.C.C. 2017 grading = pT3 pN2 G3 R0; Stage IIIB. DISCUSSION: The jejunum accounts for 11-25% of small bowel adenocarcinoma, that accounts for less than 5% of gastrointestinal cancer, notwithstanding that 90% of the mucosa surface area of the digestive tract is made by small intestine. To the best of our knowledge, this is the first report on a second loop jejunal adenocarcinoma complicating celiac disease. In our study, the diagnosis of cancer was made by computed tomography (CT) of abdomen and the patient was operated. For the diagnosis of small bowel tumour, CT enteroclysis has a sensitivity of 85-95% and a specificity of 90-96%. Complete resection (RO) of the jejunal adenocarcinoma, with regional lymph nodes resection and jejuno-jejunal anastomosis should be performed. CONCLUSION: After curative surgical resections of small bowel adenocarcinoma, adjuvant chemo-therapy has not shown a clear benefit in retrospective studies. Preoperative Chemo-Radio-therapy and careful Imaging Staging are the first steps to planning surgery.


Assuntos
Adenocarcinoma/complicações , Doença Celíaca/complicações , Neoplasias do Jejuno/complicações , Doenças Raras/complicações , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/cirurgia , Feminino , Humanos , Neoplasias do Jejuno/diagnóstico por imagem , Neoplasias do Jejuno/cirurgia , Jejuno/diagnóstico por imagem , Jejuno/cirurgia , Excisão de Linfonodo , Pessoa de Meia-Idade , Doenças Raras/diagnóstico por imagem , Doenças Raras/cirurgia , Tomografia Computadorizada por Raios X
14.
Gastroenterol. hepatol. (Ed. impr.) ; 42(7): 449-457, ago.-sept. 2019. ilus, graf
Artigo em Inglês | IBECS | ID: ibc-183840

RESUMO

Wheat is a common cereal in the Western diet and an important source of protein as well as fiber. However, some individuals develop adverse reactions to a wheat-containing diet. The best characterized is celiac disease which develops after intake of gluten in individuals with genetic predisposition. Other wheat-related conditions are less well defined in terms of diagnosis, specific trigger and underlying pathways. Despite this, the overall prevalence of wheat-related disorders has increased in the last decades and the role of microbial factors has been suggested. Several studies have described an altered intestinal microbiota in celiac patients compared to healthy subjects, but less information is available regarding other wheat-related disorders. Here, we discuss the importance of the intestinal microbiota in the metabolism of wheat proteins and the development of inflammatory or functional conditions. Understanding these interactions will open new directions for therapeutic development using bacteria with optimal wheat protein degrading capacity


El trigo es un cereal frecuente en la dieta occidental y una importante fuente de proteínas y fibra. Sin embargo, algunas personas presentan reacciones adversas a una dieta con trigo. La más conocida es la enfermedad celíaca, que se manifiesta después del consumo de gluten por parte de individuos con predisposición genética. Otras enfermedades relacionadas con el trigo no están tan bien definidas por lo que respecta al diagnóstico, el desencadenante específico y las vías subyacentes. A pesar de ello, la incidencia general de trastornos relacionados con el trigo ha aumentado en las últimas décadas, y se ha sugerido el papel de factores microbianos. Varios estudios han descrito cambios de la microbiota intestinal en pacientes celíacos frente a individuos sanos, pero hay menos información sobre otros trastornos relacionados con el trigo. En este artículo tratamos la importancia de la microbiota intestinal en el metabolismo de las proteínas del trigo y el desarrollo de trastornos inflamatorios o funcionales. El conocimiento de estas interacciones abrirá nuevas vías para el desarrollo terapéutico con bacterias con una capacidad óptima de degradación de las proteínas del trigo


Assuntos
Humanos , Triticum/metabolismo , Hipersensibilidade a Trigo/complicações , Doença Celíaca/complicações , Doença Celíaca/enzimologia , Doença Celíaca/metabolismo , Microbioma Gastrointestinal
15.
BMJ Case Rep ; 12(8)2019 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-31466978

RESUMO

A 45-year-old man presented with a 10-year history of relapsing oedema of the lips. Moreover, he exhibited recurrent facial nerve palsy since the age of 10 years, coeliac disease since the age of 12 years, atopic eczema, allergic rhinitis and asthma. Physical examination revealed lip swelling and lingua plicata. Thus, he presented the classic triad of Melkersson-Rosenthal syndrome which includes recurrent orofacial oedema, facial nerve palsy and fissured tongue. A lip biopsy confirmed our clinical diagnosis.This case is particularly rare, as the classic triad is seen only in a minority of the cases. Moreover, allergic and coeliac diseases were observed concomitantly. This paper illustrates a potential pathophysiological interconnection between these pathologies in which interferon gamma could play a key role. To our knowledge, this is the first case report in which Melkersson-Rosenthal syndrome has been observed concurrently with coeliac disease.


Assuntos
Doença Celíaca/complicações , Hipersensibilidade/complicações , Lábio/imunologia , Síndrome de Melkersson-Rosenthal/complicações , Antivirais/administração & dosagem , Antivirais/uso terapêutico , Biópsia , Doença Celíaca/patologia , Paralisia Facial/diagnóstico , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Hipersensibilidade/imunologia , Hipersensibilidade/patologia , Interferon gama/imunologia , Lábio/patologia , Masculino , Síndrome de Melkersson-Rosenthal/tratamento farmacológico , Síndrome de Melkersson-Rosenthal/imunologia , Síndrome de Melkersson-Rosenthal/reabilitação , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Recidiva , Língua Fissurada/diagnóstico , Resultado do Tratamento , Valaciclovir/administração & dosagem , Valaciclovir/uso terapêutico
16.
J Clin Pediatr Dent ; 43(4): 274-280, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31283894

RESUMO

Objectives: Celiac disease (CD) is an autoimmune disease with typical, atypical and asymptomatic forms, in which many oral manifestations have been recognized. This study aims to evaluate the prevalence of oral manifestations as well as explore if oral examination could be used as a first diagnostic screening tool for atypical or asymptomatic forms. Study Design: 45 CD patients, between 2 and 18 years (mean age 10.3) and 45 healthy subjects, age and gender-matched, were examined for hard and soft tissue lesions such as dental enamel defects (DED), dental caries, aphthous-like ulcers (ALU), atrophic glossitis, geographic tongue, median rhomboid glossitis. Results: Statistically significant differences between the two groups were observed for the prevalence of DED (in 64,4% CD and 24,46% control patients, p=0.001), their location in the teeth (incisal: p=0.0001, middle: p=0.002, cervical: p=0.007), as well as for the prevalence of ALU (in 40% CD as opposed to 4,44% control patients, p=0.001). Conclusion: The presence of DED and ALU could be used as a sign of alert for possible atypical and asymptomatic forms of CD.


Assuntos
Doença Celíaca , Cárie Dentária , Hipoplasia do Esmalte Dentário , Esmalte Dentário , Odontopatias , Adolescente , Doença Celíaca/complicações , Criança , Cárie Dentária/complicações , Esmalte Dentário/patologia , Hipoplasia do Esmalte Dentário/classificação , Humanos , Prevalência , Odontopatias/complicações , Úlcera
17.
Postgrad Med ; 131(7): 496-500, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31359810

RESUMO

Objectives: The commonest types of peripheral neuropathy in the context of Coeliac Disease (CD) and gluten sensitivity (GS) are length-dependent symmetrical sensorimotor neuropathies and sensory ganglionopathies. In patients with such neuropathy, (gluten neuropathy), peripheral neuropathic pain is prevalent suggesting involvement of small fibers. The purpose of this report was to describe the clinical characteristics of patients with CD or GS and pure small fiber neuropathy (SFN). Methods: We reviewed the records of all patients that had been referred to the Gluten-Related Neurological Disorders clinic who had clinical and neurophysiological evidence of SFN. All patients had serological evidence of gluten sensitivity (GS) prior to commencing GFD. All patients were offered a duodenum biopsy. Patients with comorbidities that could cause SFN were excluded. Results: We identified 13 patients (9 males) with SFN and gluten sensitivity. Of 11 patients who underwent duodenal biopsy 10 had evidence of enteropathy (CD). Mean age at onset of pain was 53.5 ± 11.4 years (range 34-72) and mean age of CD/GS diagnosis was 50.8 ± 10.4 years (range 34-68). In 8 patients (61.5%) pain was the presenting feature. Neurophysiological assessment suggested a length-dependent small fiber neuropathy in 11 patients, whereas in 2, a non-length dependent pattern was identifying suggesting that the predominant pathology lies in the dorsal root ganglia. Conclusion: SFN can be a presenting feature of CD and GS and, therefore, screening for CD and GS should be included in the diagnostic workup of patients with idiopathic SFN.


Assuntos
Doença Celíaca/fisiopatologia , Condução Nervosa , Neuralgia/fisiopatologia , Neuropatia de Pequenas Fibras/fisiopatologia , Adulto , Idoso , Anticorpos/imunologia , Autoanticorpos/imunologia , Doença Celíaca/complicações , Doença Celíaca/dietoterapia , Doença Celíaca/imunologia , Dieta Livre de Glúten , Feminino , Proteínas de Ligação ao GTP/imunologia , Gliadina/imunologia , Humanos , Imunoglobulina A/imunologia , Imunoglobulina G/imunologia , Masculino , Pessoa de Meia-Idade , Neuralgia/etiologia , Estudos Retrospectivos , Neuropatia de Pequenas Fibras/etiologia , Transglutaminases/imunologia
18.
Surg Pathol Clin ; 12(3): 733-743, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31352985

RESUMO

The gastrointestinal tract is a common extranodal site of involvement by lymphomas. These may be diagnostically challenging because they can mimic a variety of benign conditions and may be difficult to subclassify when malignant. The classification of gastrointestinal lymphomas is an evolving area with some recent changes. Although some of these entities are rare, they are important to recognize because of the variable clinical presentations, comorbidities, and treatment implications. This article explores new and revised entities in gastrointestinal lymphoproliferative disorders.


Assuntos
Neoplasias Gastrointestinais/diagnóstico , Transtornos Linfoproliferativos/diagnóstico , Doença Celíaca/complicações , Doença Crônica , Diagnóstico Diferencial , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/terapia , Humanos , Transtornos Linfoproliferativos/genética , Transtornos Linfoproliferativos/terapia , Prognóstico
19.
Medicina (Kaunas) ; 55(7)2019 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-31311098

RESUMO

Celiac disease (CD) is a systemic autoimmune disease driven by gluten-ingestion in genetically predisposed individuals. Although it primarily affects the small bowel, CD can also involve other organs and manifest as an extraintestinal disease. Among the extraintestinal features of CD, hematologic ones are rather frequent and consist of anemia, thrombocytosis (thrombocytopenia also, but rare), thrombotic or hemorrhagic events, IgA deficiency, hyposplenism, and lymphoma. These hematologic alterations can be the sole manifestation of the disease and should prompt for CD testing in a suggestive clinical scenario. Recognition of these atypical, extraintestinal presentations, including hematologic ones, could represent a great opportunity to increase the diagnostic rate of CD, which is currently one of the most underdiagnosed chronic digestive disorders worldwide. In this review, we summarize recent evidence regarding the hematological manifestations of CD, with focus on practical recommendations for clinicians.


Assuntos
Doença Celíaca/complicações , Doenças Hematológicas/etiologia , Anemia/etiologia , Anemia/fisiopatologia , Doença Celíaca/fisiopatologia , Doenças Hematológicas/fisiopatologia , Humanos , Deficiência de IgA/etiologia , Deficiência de IgA/fisiopatologia , Linfoma/etiologia , Linfoma/fisiopatologia
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