Collagenous sprue: don´t forget connective tissue in chronic diarrhea evaluation.

Collagenous sprue is a rare disease of the small bowel characterized by mucosal atrophy and excessive subepithelial collagen deposition. The etiology remains unclear and the diagnosis is based upon patient´s clinical picture and anatomopathological findings. Clinically, collagenous sprue is characterized by persistent diarrhoea, severe malabsorption, multiple nutrient deficiencies and progressive weight loss. Differential diagnosis includes celiac disease, which is mandatory to rule out because of their frequent association. Glutenfree diet is the first therapeutic step, but it usually is not effective. However, recent studies show high success rates with immunomodulators, mainly corticosteroids. We report the case of a patient presenting with chronic diarrhea and severe malabsorption who was diagnosed with collagenous sprue, with no response to gluten free diet, but with excellent response to budesonida.

[Enteropathy-associated T-cell lymphoma: a diagnostic challenge]. / Linfoma T asociado a enteropatía: un reto diagnóstico.

Diagnosis of refractory celiac disease (CD) is based on exclusion of other disorders, persistence of malabsorptive symptoms and villous atrophy, despite a strict gluten-free diet for at least 6-12 months. Detection of alterations in the intraepithelial lymphocyte population is crucial for diagnosis. A subgroup of patients with refractory CD may develop severe complications such as enteropathy-associated T cell lymphoma (EATL). We present the case of a patient with longstanding silent CD who developed EALT, highlighting the challenge posed by the diagnosis and treatment of this entity.

[Anticonvulsant hypersensitivity syndrome: an entity to be remembered]. / Síndrome de hipersensibilidad a anticomiciales: una entidad para recordar.

Anticonvulsant hypersensitivity syndrome is an unpredictable, potentially fatal drug reaction to aromatic anticonvulsants such as carbamazepine, phenytoin and phenobarbital. The hallmark features include fever, eosinophilia, rash and involvement of one or more internal organs. Clearly established diagnostic criteria and treatment guidelines are lacking. A high index of suspicion is required to identify this syndrome, allowing early withdrawal of the drug and avoiding re-exposure. We report an illustrative case of anticonvulsant hypersensitivity syndrome and review the published literature.

[Adult celiac disease and intraepithelial lymphocytes. New options for diagnosis?]. / Enfermedad celíaca del adulto y linfocitos intraepiteliales. Nuevas opciones para el diagnóstico?

BACKGROUND: Permanent changes have been found in intraepithelial lymphocyte (IEL) subsets in patients with celiac disease. OBJECTIVE: The main aim of this study was to demonstrate the utility of determining CD3()/CD7(+) and T cell receptor (TCR) gamma-delta IEL subsets by flow cytometry as a diagnostic marker of adult celiac disease. PATIENTS AND METHODS: We performed a prospective study in a sample of 128 adult patients (70 with celiac disease and 58 controls). In all patients, distal duodenal biopsy was performed and IEL subsets were determined by flow cytometry. RESULTS: Patients with celiac disease showed an increase in gamma-delta IEL subsets and a decrease in CD3(-)/CD7(+) IEL subsets in comparison with the control group, independently of diet. CONCLUSIONS: The results indicate that IEL subset determination by flow cytometry could be useful to confirm diagnosis of celiac disease. IEL subsets should be investigated in diseases other than celiac disease, as well as in patients with potential or latent celiac disease.

[A rare cause of gastric outlet obstruction: Bouveret's syndrome]. / Una causa infrecuente de obstrucción gástrica: síndrome de Bouveret.

Bouveret's syndrome is a rare type of gallstone ileus in which a gallstone enters the intestinal tract via a cholecystoenteric fistula and is lodged in the duodenum or the stomach. Since the first description by León Bouveret in 1896, fewer than 200 cases have been described in the worldwide literature. Mortality is high, at 25%, but may be related to the advanced age of the typical patient and comorbidities, as well as diagnostic delay. Diagnosis may be made with radiological (abdominal X-ray, ultrasound, computed tomography or magnetic resonance imaging) and endoscopic techniques. Endoscopy is preferred as the first therapeutic option but is frequently unsuccessful and surgery is often required. We present the case of a patient admitted to hospital with a history of vomiting after eating and epigastric pain. The management of this rare cause of gastric outlet obstruction is discussed.

Non-dietary therapeutic clinical trials in coeliac disease.

Coeliac disease is a permanent immunological intolerance to gluten proteins in genetically predisposed individuals. The only management is life-long strict adherence to a gluten-free diet. Unfortunately, compliance with gluten-free diet is very difficult in practice due to the widespread presence of gluten in Western diets. For this reason, about 50% of coeliacs following a gluten-free diet continue to suffer from symptoms and present with autoantibodies and/or villous atrophy while on a gluten-free diet. It is therefore important to explore new therapies to improve the management of coeliac disease. To date, five experimental therapies have been tested in randomized and controlled clinical trials. Larazotide acetate reduces the para-cellular passage of gluten to the lamina propria by preventing the opening of intercellular tight junctions. The endopeptidases ALV003 and AN-PEP break down gluten to produce less or non-toxic peptide fragments. A therapeutic vaccine is being tested with the aim of developing gluten tolerance. Finally, infection with the nematode Necator americanus and treatment with the CCR9 antagonist Traficet-EN have also been reported. While substantial progress has been made in the last few years, it is important to remember that all these investigational therapies are in research stage and are generally being considered as "adjunctive" therapies to the gluten-free diet and not as substitutes of the gluten-free diet at this point in time.

Ataxia y síndrome frontal en una paciente joven resuelto con dieta sin gluten / Ataxia and frontal syndrome in a young woman resolved with a gluten-free diet

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Esprúe colágeno: no olvidemos el tejido conectivo en la evaluación de la diarrea crónica / Collagenous sprue: don’t forget connective tissue in chronic diarrhea evaluation

El esprúe colágeno es una patología infrecuente del intestino delgado caracterizada por atrofia de la mucosa y depósito excesivo de colágeno a nivel subepitelial. Su etiología es desconocida y su diagnóstico se realiza en base a la presencia tanto de un cuadro clínico compatible como de hallazgos anatomopatógicos sugestivos. Los pacientes suelen presentar diarrea crónica, malabsorción, deficiencias nutricionales graves y una marcada pérdida ponderal. Dentro del diagnóstico diferencial es mandatorio descartar enfermedad celiaca por su frecuente asociación a la misma. La dieta sin gluten es el primer escalón terapéutico, pero generalmente no es efectiva. Sin embargo, estudios recientes señalan altas tasas de éxito mediante el uso de inmunomoduladores. Presentamos el caso de un paciente con diarrea crónica y malabsorción grave diagnosticado de esprúe colágeno, sin respuesta a la dieta sin gluten, en el que el tratamiento con budesonida ha conseguido una excelente respuesta(AU)

Collagenous sprue is a rare disease of the small bowel characterized by mucosal atrophy and excessive subepithelial collagen deposition. The etiology remains unclear and the diagnosis is based upon patient’s clinical picture and anatomopathological findings. Clinically, collagenous sprue is characterized by persistent diarrhoea, severe malabsorption, multiple nutrient deficiencies and progressive weight loss. Differential diagnosis includes celiac disease, which is mandatory to rule out because of their frequent association. Gluten-free diet is the first therapeutic step, but it usually is not effective. However, recent studies show high success rates with immunomodulators, mainly corticosteroids. We report the case of a patient presenting with chronic diarrhea and severe malabsorption who was diagnosed with collagenous sprue, with no response to gluten free diet, but with excellent response to budesonida(AU)

Atrofia vellositaria sin enfermedad celíaca: ¿un nuevo síndrome o más confusión? / Non celiac villous atrophy: More confusion or a new syndrome?

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Enteropatía por olmesartán / Enteropathy by olmesartan

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Sensibilidad al gluten no celíaca: ¿una verdadera entidad o solo una quimera? / Non-celiac gluten sensitivity: A true entity or just a chimera?

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Actualización en esprúe colágeno: el tejido conectivo como causa de diarrea crónica / Update on collagenous sprue: Connective tissue as a cause of chronic diarrhea

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Linfoma T asociado a enteropatía: un reto diagnóstico / Enteropathy-associated T-cell lymphoma: a diagnostic challenge

El diagnóstico de la enfermedad celíaca resistente (ECR) se establece, tras la exclusión de otras entidades, ante la persistencia de datos clínicos de malabsorción y atrofia vellositaria durante 6-12meses a pesar de una estricta dieta sin gluten (DSG). La detección de alteraciones en la población linfocitaria intraepitelial es importante para su diagnóstico. Un subgrupo de pacientes con ECR pueden desarrollar complicaciones severas, como linfoma T asociado a enteropatía (LTAE). Presentamos el caso de un paciente con EC silente de larga evolución que finalmente derivó en LTAE y que evidencia el reto que supone para el clínico tanto el diagnóstico como el tratamiento de esta entidad (AU)

Diagnosis of refractory celiac disease (CD) is based on exclusion of other disorders, persistence of malabsorptive symptoms and villous atrophy, despite a strict gluten-free diet for at least 6-12months. Detection of alterations in the intraepithelial lymphocyte population is crucial for diagnosis. A subgroup of patients with refractory CD may develop severe complications such as enteropathy-associated T cell lymphoma (EATL). We present the case of a patient with longstanding silent CD who developed EALT, highlighting the challenge posed by the diagnosis and treatment of this entity (AU)