SUMMARY Refractory celiac disease is an uncommon condition which might be associated to poor prognosis. It is often treated with immunosuppressive medications, with poor results. It is divided in type 1 and type 2, the latter carrying a high risk for lymphoma and mortality. A case of a 41 year old female patient with refractory celiac disease type 2 is reported. She was treated with oral budesonide for six months, achieving histological remission.

ABSTRACT Background: Paracoccidioidomycosis is a systemic mycosis considered endemic and limited to Latin America with the majority of registered cases originating from Brazil. The purpose of this paper was to report a case of a female patient with paracoccidioidomycosis mimicking inflammatory bowel disease and to systematically review available cases of the intestinal presentation of this infectious disease. Case report: Female patient, 32-years old, previously asymptomatic, presenting with acute pain in the lower right abdomen, associated with signs of peritoneal irritation and abdominal distension. Urgent surgery was performed, which identified a severe suppurative perforated ileitis. The anatomopathological study revealed fungal structures shaped as a ship’s pilot wheel in Grocott-Gomori’s staining, suggestive of Paracoccidioides spp. Methods: Studies were retrieved based on Medical Subject Headings and Health Sciences Descriptors, which were combined using Boolean operators. Searches were run on the electronic databases Scopus, Web of Science, MEDLINE (PubMed), BIREME (Biblioteca Regional de Medicina), LILACS (Latin American and Caribbean Health Sciences Literature), SciELO (Scientific Electronic Library Online), Embase, and Opengray.eu. Languages were restricted to English, Spanish and Portuguese. There was no date of publication restrictions. The reference lists of the studies retrieved were searched manually. Simple descriptive analysis was used to summarize the results. Results: Our search strategy retrieved 581 references. In the final analysis, 34 references were included, with a total of 46 case reports. The most common clinical finding was abdominal pain and weight loss present in 31 (67.3%) patients. Most patients were treated with itraconazole (41.3%) and amphotericin B (36.9%). All-cause mortality was 12.8%. Conclusions: Paracoccidioidomycosis should be suspected in endemics areas, specially as a differential diagnosis for inflammatory bowel disease. Endoscopic tests and biopsy are useful for diagnosis and treatment with antifungal drugs seem to be the first treatment option to achieve a significant success rate.

RACIONAL: O esôfago de Barrett é uma complicação da doença do refluxo gastroesofágico com importante potencial de malignização. Relata-se que a expressão do marcador tumoral p53 se acentua com a progressão displasia-adenocarcinoma. OBJETIVO: Avaliar a expressão da p53 no epitélio de Barrett com presença ou não de displasia conforme dois critérios de positividade. MATERIAL E MÉTODOS: O material foi constituído por biopsias endoscópicas de 42 doentes com esôfago de Barrett. Cortes histológicos foram corados pela hematoxilina-eosina, pelo PAS-alcian blue e avaliados quanto à expressão imunoistoquímica da p53. O diagnóstico de displasia foi firmado pela concordância entre três patologistas. Foram utilizados dois critérios de positividade para a p53: 1. a coloração de, pelo menos, metade dos núcleos e 2. o encontro de qualquer núcleo corado. RESULTADOS: O número total de fragmentos foi de 229, com média de 5,4 por paciente. A displasia foi detectada em seis (14,3%) casos. Para diferentes critérios de positividade, a p53 foi detectada, respectivamente, em 5 (13,9%) e 14 (38,9%) com epitélio metaplásico não-displásico. Especificamente nos seis casos displásicos, a p53 foi detectada, conforme o critério de positividade, em um (16,7%) e quatro (66,7%) casos, respectivamente. CONCLUSÕES: Nesta pequena série, a expressão imunoistoquímica da p53, independente do critério de positividade, não foi de auxílio para a confirmação de alterações displásicas no esôfago de Barrett.

BACKGROUND: Barrett's esophagus is the most serious complication of the gastroesophageal reflux disease and presents a malignant potential. The expression of the tumoral marker p53 increases with the dysplasia-adenocarcinoma sequence. AIMS: To evaluate the p53 expression in Barrett's esophagus with or without dysplasia according to the two positive immunostaining criteria. MATERIALS AND METHODS: The material was constituted by endoscopic biopsy specimens from 42 patients with Barrett's esophagus. Section ectionss of formalinof formalin-fixed and paraffin-embedded biopsies were stained with hematoxylin-eosin, PAS-alcian blue and evaluated the p53 immunohistochemical expression. Two p53 immunostaining criteria were utilized: 1. the staining of, at least, half of the nuclei, and 2. the staining of any nucleus. The diagnosis of dysplasia was confirmed by the agreement between three pathologists. RESULTS: The total number of tissue specimens was 229, with an average of 5.4 specimens per patient. Dysplasia, with agreement for all pathologists examining the same set of slides, was detected in six (14.3%) cases. According to the two different p53 immunostaining criteria, the protein was detected in non-dysplastic Barrett's metaplasia, respectively, in 5 (13.9%) and 14 (38.9%) patients. Specificaly in the six dysplastic cases, p53 was detected, according to the immunostaining criteria, in one (16.7%) and four (66.7%) cases, respectively. CONCLUSIONS: In this group, p53 immunohistochemical expression, regardless of positive criteria take into account, was not useful for detecting dysplasia in Barrett's esophagus.

RACIONAL: O potencial maligno do esôfago de Barrett é bem reconhecido. A vigilância endoscópica e a abordagem terapêutica se baseiam na presença e graduação da displasia. Contudo, a validade do diagnóstico histopatológico pode ser questionada devido à precária reprodutibilidade tanto intra como interobservador. OBJETIVO: Avaliar a concordância intra e interobservador no diagnóstico de displasia no esôfago de Barrett. MATERIAL E MÉTODOS: O material foi constituído por 42 blocos de parafina contendo fragmentos de esôfago provenientes de biopsias endoscópicas de portadores de esôfago de Barrett. Cortes de 3 micrômetros foram corados pela hematoxilina-eosina e pelo PAS-alcian blue. A leitura das lâminas foi realizada de maneira cega, em microscópio óptico. A reprodutibilidade intra e interobservador utilizou o teste kappa. RESULTADOS: O número total de fragmentos foi de 229, com média de 5,45 (1 a 18) fragmentos por paciente. O diagnóstico de displasia de baixo grau firmado pelos diferentes patologistas variou de 21,4% a 52,4%. A concordância intra-observador para o diagnóstico de displasia de baixo grau foi fraca (kappa = 0,30). A concordância interobservador para o diagnóstico de displasia de baixo grau foi pobre, com escore kappa oscilando entre 0,05 e 0,16. O diagnóstico de displasia, firmado pela concordância entre todos os patologistas, foi de 14,3%. CONCLUSÕES: A concordância no diagnóstico histopatológico de displasia de baixo grau no esôfago de Barrett, tanto intra quanto interobservador, é ruim. Idealmente, à semelhança da displasia de alto grau, o diagnóstico de displasia de baixo grau no esôfago de Barrett também deveria ser confirmado por mais de um patologista.

BACKGROUND: Barrett's esophagus is a well-known pre-malignant condition. Pathologic interpretation of biopsy specimens guides endoscopic surveillance as well as the therapeutic approach that will be carried out. However, the predictive value of histopathologic diagnosis can be questioned due to its poor intra- and interobserver reproducibility. AIMS: To assess intra- and interobserver variability in the diagnosis of Barrett's dysplasia. MATERIAL AND METHODS: Three-micrometer thick sections from biopsy specimens from 42 patients with Barrett's esophagus were stained with hematoxylin-eosin and PAS-alcian blue. The reading of the slides was carried out blindly in a light microscope. Intra and interobserver variability in the interpretation of the slides was determined by kappa statistics. RESULTS: The number of tissue specimens was 229, with average of 5.45 (1 to 18) fragments for patient. Low grade dysplasia was diagnosed by pathologists in 21.4% to 52.4% of the cases. The intra-observer agreement for the diagnosis of low grade dysplasia was slight (kappa = 0.30). The interobserver agreement for the diagnosis of low grade dysplasia was poor, with kappa scores between 0.05 and 0.16. The diagnosis of dysplasia, with agreement for all pathologists examining the same set of slides, was 14.3%. CONCLUSIONS: Pathologic interpretation of Barrett's dysplasia may be subject to marked intra- and interobserver variabiliaty. Interpretation of low grade dysplasia, as high grade dysplasia, should also be considered for review by two or more pathologists.