Gastropatía hipertrófica por IgG4: reporte del primer caso en Chile / IgG4 related hypertrophic gastropathy: report of one case

IgG4 related disease is a systemic autoimmune disease, which can affect different organs. The most common digestive manifestation is autoimmune pancreatitis (AIP), followed by involvement of bile ducts and the major papilla. The stomach is only rarely involved. We report a 71-year-old diabetic woman consulting for jaundice and weight loss. Abdominal CAT scan, cholangio resonance imaging, endosonography and a serum IgG4 of five times the normal value, lead to the diagnosis of an autoimmune pancreatitis. An upper gastrointestinal endoscopy showed a diffuse thickening of gastric folds. The pathological study confirmed the presence of IgG4 positive plasma cells. The patient was successfully treated with steroids.

Severe hypoproteinemia as a harbinger of Ménétrier's disease in autoimmune pancreatitis / Hipoproteinemia grave como indicador de doença de Ménétrier na pancreatite autoimune

Summary Ménétrier's disease is an extremely rare disease of unknown etiology causing gastric mucosal hypertrophy and protein-losing gastropathy. Rare cases of this condition have been reported in patients with autoimmune diseases. However, to the best of our knowledge, Ménétrier's disease associated with autoimmune pancreatitis (AIP) has never been reported. We described a case of severe hypoproteinemia as a harbinger of Ménétrier's disease associated with AIP. The patient was successfully treated with octreotide and high-protein diet, which led to symptomatic remission and significant improvement in serum levels of albumin and recovery of the nutritional status. Thus, in AIP patients presenting with severe and persistent hypoproteinemia without apparent cause, clinicians need to consider Ménétrier's disease in the differential diagnosis. In this setting, endoscopic evaluation with histological examination of gastric biopsy material, including a full-thickness mucosal biopsy of involved mucosa, may be helpful in promptly establishing the diagnosis and allowing appropriate and timely therapy.

Resumo A doença de Ménétrier é uma condição extremamente rara, de etiologia desconhecida, caracterizada por hipertrofia da mucosa gástrica e gastropatia perdedora de proteína. Casos raros dessa patologia têm sido relatados em pacientes com doenças autoimunes. Até o momento, desconhecemos qualquer relato dessa doença associada à pancreatite autoimune (PAI). Descrevemos um caso de hipoproteinemia grave como indicador de doença de Ménétrier associada à PAI. O paciente foi tratado de forma satisfatória com octreotide e dieta hiperproteica, alcançando remissão sintomática, melhora significativa das concentrações de albumina e recuperação do estado nutricional. Portanto, em pacientes com PAI e hipoproteinemia grave e persistente, deve-se considerar a doença de Ménétrier como um diagnóstico diferencial. Nesses casos, a avaliação endoscópica com biópsia gástrica, incluindo biópsia de toda a espessura da mucosa, pode ser útil no estabelecimento do diagnóstico e do pronto início da terapêutica.

Enfermedad de Ménétrier con compromiso gástrico difuso y duodenal: Caso clínico / Ménétrier disease: Report of one case

Background: Ménétrier disease is a rare disorder of the stomach, characterized by giant hypertrophic folds that usually involve the gastric body and fundus, associated to hypoalbuminemia due to serum protein loss across the gastric mucosa. We report a 55-years-old male presenting with abdominal pain, vomiting, weight loss and hypoalbuminemia. Diffuse hypertrophic gastric folds, elevated ulcerated sessile lesions and focal duodenal involvement were seen at endoscopy. Biopsies showed foveolar hyperplasia and glandular atrophy with cystic dilatation. A total gastrectomy was performed with a good outcome.

Los genotipos de helicobacter pylori en gastritis no atrófica difieren de los encontrados en úlcera péptica, lesiones premalignas y cáncer gástrico en Colombia / Helicobacter pylori genotypes in non atrophic gastritis, peptic ulcer disease, premalignant lesions and gastric cancer in Colombia

Background: Helicobacter pylori is recognized as an etiologic agent of several gastric diseases. Bacterial genotypes have been related to clinical outcome in several populations. Aim: To compare cagA, vacA and iceA genotypes of Colombian isolates from patients with several gastrointestinal diseases, including gastric cancer. Material and methods: We used polymerase chain reactions to amplify vacA, cagA and iceA genes of 137 H pylori isolates coming from 26 patients with gastric cancer (GC), 34 with peptic ulcer (PU), 19 with intestinal metaplasia (IM), 23 with atrophic gastritis (AG) and 35 with non atrophic gastritis (NAG). Results: vacA s1-m1, cagA+, iceA+ were the most frequently found genotypes. vacA s1 and m1 subtypes were found in 92 (67 percent) and 82 (60 percent) cases respectively. Sixty three percent were cagA+ and 85 percent were iceA+. There was a lower prevalence of s1 allele in cases of NAG (43 percent), compared with GC, PU and IM (81 percent, 77 percent and 81 percent prevalence, respectively, p <0.01). Isolates from NAG also showed a low frequency of vacA m1 subtype (40 percent) compared with GC or IM (81 percent and 84 percent respectively, p <0.01). The prevalence of cagA+ strains was significantly higher in GC patients (80 percent) than in NAG patients (51.4 percent, p <0.01). No differences in the frequency of vacA s1a, s1b and iceA subtypes, were observed. Conclusions: A lower frequency of cytotoxic H pylori genotypes such as cagA and vacA s1m1 and a higher frequency of non cytotoxic genotypes, was observed in patients with NAG, when compared to patients with GC or PU. These results suggest that even in Colombia, vacA and cagA could be used as markers of increased virulence

Gastritis plasmocelular crónica activa asociada a citomegalovirus / Chronic active plasmacytic gastritis associated with cytomegalovirus

La gastritis plasmocelular crónica activa (GPCA) se caracteriza por la presencia de infiltrados inflamatorios crónicos predominantemente plasmocelulares que comprometen las células del cuello de las glándulas gástricas. Tanto la GPCA como la gastropatía de Ménétrier han sido vinculadas con el citomegalovirus (CMV). Con el propósito de probas esta asociación, se evaluó la relación fovéolo-glandular (F/G) y se determinó la presencia del AND (ácido desoxirribonucleico) del CMV mediante PCR (reacción en cadena de polimerasa) en 12 casos y 13 controles. Los ejemplos de GPCA presentaron enfermedad de Ménétrier en 2 casos; hiperplasia foveolar, en 6 casos, y relación F/G conservada, en 3 casos. En ninguno visualizaron imágenes de gastritis linfocitaria, ni alteraciones citopáticas por CMV. Tres casos se asociaron con carcinoma gástrico. La relación F/G fue menor de 1 en todos los controles. Once de los 12 casos con GPCA mostraron amplificación para le AND del CMV y los 13 controles fueron negativos. Los hallazgos sugieren una fuerte asociación, posiblemente secuencial, entre la infección por el CMV, la GPCA, la hiperplasia foveolar (con o sin desarrollo de enfermedad de Ménétrier) y el carcinoma gástrico. La GPCA podría representar un marcador histológico de la presencia de CMV en la zona germinativa de los cuellos glandulares. La propuesta es paralela a los hallazgos, las lesiones y la secuencia observables en la infección hepática por VHB (irus de hepatitis B), con el desarrollo de hepatitis crónica, cirrosis y hepatocarcinoma.

Reordenamiento de inmunoglobulinas en el diagnóstico diferencial del linfoma gástrico primario / Immunoglobulin rearrangement in the differential diagnosis of primary gastric lymphoma

Background: The traditional methods to distinguish Chronic Follicular Gastritis and Primary Gastric Lymphoma do not allow an adequate definitive diagnosis in a significant number of cases. The Molecular Biology diagnostic methods are based on the rearrangement of immunoglobulin genes. The polymerase chain reaction (PCR) specifically amplifies this rearrangement and allows molecular analysis of minimal tissue samples obtained with endoscopical biopsies. Aim: To test the usefulness of this PCR method in the differential diagnosis between Chronic Follicular Gastritis and Primary Gastric Lymphoma. Material and methods: We analyzed the endoscopical biopsies of six Chronic Follicular Gastritis cases and eight surgically treated Primary Gastric Lymphoma cases, six with the correct diagnosis in the endoscopical biopsies and two with a diagnosis of Chronic Follicular Gastritis. Results: A policlonal immunoglobulin rearrangement was found in the six cases with Chronic Follicular Gastritis. A monoclonal arrangement was found in 5 of 6 biopsies with the diagnosis of Primary Gastric Lymphoma. The same monoclonal rearrangement was observed in the two biopsies incorrectly diagnosed as Chronic Follicular Gastritis. Conclusions: PCR analysis of immunoglobulin rearrangement is a useful method in the differential diagnosis between Chronic Follicular Gastritis and Primary Gastric Lymphoma

Gastropatia hipertrofica de Menetrier asociado a poliarteritis nodosa / Hypertrophic gastritis of Menetrier associated with nodose polyarteritis

Se presenta un caso de sexo masculino de 61 años de edad cuya sintomatología comienza aparentemente 3 meses antes: anorexia, pérdida de peso, síndrome febril prolongado, mialgias, polineuropatía periférica, dolor abdominal, anemia, síndrome de insuficiencia renal, esplenomegalia detectado ecográficamente, ascitis, fosfatasas aldalinas elevadas, hipoprotidemia, caídas súbitas del hematocrito, úlcera gástrica con biopsias negativas para malignidad y donde el estudio histopatológico de la pieza operatoria (gastrectomía parcial), confirmó los diagnósticos de enfermedad de Ménétrier o gastropatía hipertrófica, asociada a poliarteritis nodosa. La primera explica la hipoprotidemia, los edemas, la ascitis. La segunda, la insuficiencia renal, la polineuropatía periférica, el compromiso muscular esplénico, hepático y la fiebre; mientras que la úlcera gástrica, las pérdidas de sangre, la anorexia, la pérdida de peso y el dolor abdominal pueden ser explicados por ambas enfermedades. En la bibliografía consultada no se detectó ningún caso con esta misma asociación

Doença de Ménétrier na criança: relato de três casos e revisäo da literatura / Ménétrier's disease in child: report of 3 cases and review of the literature

Säo descritos três casos de doença de Ménétrier na criança e 38 casos previamente relatados na literatura säo revisados. A doença se manifesta na criança com uma gastropatia perdedora de proteínas e, através de radiografia de esôfago-estômago e duodeno ou de endoscopia, demonstra-se uma característica de hipertrofia das pregas gástricas. Em contraste com a cronicidade habitual da doença no adulto, o curso é comumente benigno na criança, em os sintomas resolvendo-se espontaneamente em semanas e meses. Säo discutidas as possíveis etiologias e o diagnóstico diferencial. A doença de Ménétrier na criança pode ser mais comum do que tem sido relatada

Enfermedad de menetrier en el Hospital Nacional Guillermo Almenara Irigoyen: a propósito de un caso. / Menetrier's Disease in the Hospital Nacional Guillermo Almenara Irigoyen: apropos of a case.

A proposito un caso. Una mujer de 46 años de edad, presentó diarreas, lllenura epigástrica y baja de peso. El estudio radiológico endoscópico demostró grandes rugosidades de los pliegues gástricos y el diagnóstico de enfermedad de Menetrier fue confirmado por biopsia quirúrgica. Con la gastrectomía total y esofagoyeyunoanastomosis de Roux en Y, mejoró sus niveles de proteínas plasmáticas, pero continúa con diarrea postgastrectomía. Como factor etiológico se le atribuye uno de tipo multifactorial exógeno/endógeno: hereditario, alérgico, psíquico y transtorno adenomatoso endocrino. A propósito de este caso, se hace una revisión de literatura médica.

Doença de Ménétrier: apresentaçäo de um caso clínico-cirúrgico / Ménétrier's disease: presentation of clinical and surgical case

Os autores apresentam um caso clínico de doença de Ménétrier, diagnosticado através de endoscopia digestiva alta usando pinça de biópsia convencional. Este caso necessitou gastrectomia total porque o paciente, com freqüência, apresentava gastrite hemorrágica

Gastritis hipertrófica gigante (enfermedad de Menetrier) y cáncer de colon: asociación casual? / Hypertrophic gastritis large (Menetrier's disease) and colon cancer: casual association?

La gastritis hipertrófica gigante o enfermedad de Menetrier es una patología caracterizada por la presencia de plieges largos y gruesos de aspectos cerebriforme que envuelven parte o la totalidad del estómago. Los pacientes frecuentemente tienen síntomas del tracto gastrointestinal, pérdida de peso y edema por hipoproteinemia. Los estudios radiológicos y de laboratorio son claramente diagnósticos. Su degeneración maligna, así como su asociación con otras neoplasias del tubo digestivo, debe ser motivo de alarma e investigación. Se presenta un caso y se hace revisión de la literatura