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Supplemental material, sj-pdf-1-tag-10.1177_1756284820965790 for Rapid test detection of anti-infliximab antibodies: performance comparison with three different immunoassays by Cátia Rocha, Paula Lago, Samuel Fernandes, Luís Correia, Francisco Portela, Ana Isabel Vieira, Marta Patita, Bruno Arroja, Paula Ministro, Catarina Alves, Cláudia Camila Dias and Fernando Magro in Therapeutic Advances in Gastroenterology

Supplemental material, sj-pdf-2-tag-10.1177_1756284820965790 for Rapid test detection of anti-infliximab antibodies: performance comparison with three different immunoassays by Cátia Rocha, Paula Lago, Samuel Fernandes, Luís Correia, Francisco Portela, Ana Isabel Vieira, Marta Patita, Bruno Arroja, Paula Ministro, Catarina Alves, Cláudia Camila Dias and Fernando Magro in Therapeutic Advances in Gastroenterology

Background: Hepatic glycogenosis (HG) is a complication of poorly controlled type 1 diabetes mellitus (T1DM), characterized by glycogen accumulation in hepatocytes. Mauriac syndrome (MS) is a glycogenic hepatopathy, initially described in 1930, characterized by growth failure, delayed puberty, cushingoid appearance, hepatomegaly with abnormal liver enzymes, and hypercholesterolemia. HG is a condition with good prognosis and fast resolution after adequate glycemic control (although it has potential for relapse), with no case of evolution to end-stage liver disease described. Case: We describe a 26-year-old female, with T1DM complicated by severe retinopathy. The patient maintained poor glycemic control since childhood, presenting glycated hemoglobin persistently higher than 10% and recurrent episodes of ketoacidosis. In adolescence, she developed hepatomegaly and fluctuating elevation of aminotransferases and triglycerides. A small, nonrepresentative hepatic biopsy suggested macrovacuolar steatosis and mild fibrosis. After 15 years of diabetes, the patient was referred for gastroenterology clinic due to chronic diarrhea and exuberant hepatomegaly. Laboratory showed persistent elevation of aminotransferases and triglycerides. Bilirubin, iron metabolism, and coagulation were normal; viral serologies and autoimmune study were negative. Upper endoscopy, ileocolonoscopy, and enteroscopy presented no lesions. Abdominal magnetic resonance imaging displayed massive hepatomegaly. Liver biopsy was repeated showing marked nuclear glycogenization, mild steatosis, and no fibrosis; electron microscopy revealed very large deposits of glycogen and pleomorphic mitochondria with an unusually dense matrix, described for the first time in this entity. The diagnosis of MS variant and diarrhea due to autonomic neuropathy were assumed. Conclusion: Currently, HG is a well-recognized disease that occurs at any age and can be present without the full spectrum of features initially described for MS. In the era of insulin therapy, this entity represents a rare complication, caused by low therapeutic compliance. Introdução: A glicogenose hepática (GH) é uma complicação da diabetes mellitus tipo 1 (DM1) mal controlada, caracterizada pela acumulação de glicogénio nos hepatócitos. A síndrome de Mauriac (SM) é uma hepatopatia glicogénica, descrita inicialmente em 1930, caracterizada por atraso de crescimento, puberdade tardia, fácies cushingoide, hepatomegalia, elevação das enzimas hepáticas e hipercolesterolemia. A GH é uma condição com bom prognóstico e com rápida resolução após controlo adequado da glicémia (apesar de ter potencial para recidiva), não havendo descrito nenhum caso de evolução para end-stage liver disease. Caso: Descrevemos o caso de uma mulher de 26 anos, com DM1 complicada por retinopatia grave. A doente manteve inadequado controlo glicémico desde a infância, apresentando hemoglobina glicada persistentemente superior a 10% e episódios recorrentes de cetoacidose. Na adolescência, desenvolveu hepatomegalia, elevação flutuante das aminotransferases e triglicéridos. A biópsia hepática sugeriu esteatose macrovacuolar e fibrose ligeira. Após 15 anos de evolução da diabetes, a doente foi encaminhada para consulta de gastrenterologia por diarreia crónica e hepatomegalia exuberante. Laboratorialmente verificou-se elevação persistente das aminotransferases e dos triglicéridos. A bilirrubina, o metabolismo do ferro e a coagulação eram normais; as serologias virais e o estudo auto-imune foram negativos. A endoscopia digestiva alta, ileocolonoscopia e enteroscopia não apresentavam alterações. A ressonância magnética abdominal mostrou hepatomegalia maciça. A biópsia hepática foi repetida, mostrando glicogenização nuclear acentuada, esteatose leve e ausência de fibrose; a microscopia eletrônica revelou depósitos volumosos de glicogênio e mitocôndrias pleomórficas com uma matriz extraordinariamente densa, descrita pela primeira vez nesta entidade. Foi assumido o diagnóstico de glicogenose hepática no contexto de SM e diarreia devido a neuropatia autonómica. Conclusão: Atualmente, a GH é uma entidade bem reconhecida que pode ocorrer em qualquer idade e pode estar presente sem o espectro completo das características descritas inicialmente para a SM. Na era da insulinoterapia, esta patologia representa uma complicação rara, causada pela baixa adesão à terapêutica.

Background: Percutaneous endoscopic gastrostomy (PEG) is a safe procedure and major morbidity is unusual. However, the number of PEG fed patients is increasing all over the world and complications may become more and more frequent. Case report: We describe a 73 years old woman with persistent vomit after replacement of the standard PEG tube with a Foley catheter. An upper GI endoscopy showed the catheter pulled into the duodenum causing gastric outlet obstruction. It was removed and replaced by a suitable standard PEG tube, allowing PEG feeding to be resumed. Discussion: Previous reports pointed the risk of this complication, almost always associated with insertion of a Foley-type catheter. Replacement of PEG tubes should be performed by experienced teams using standard PEG tubes and the use of Foley-type catheters for this purpose should be banned from routine practice. Resumen Introducción: la gastrostomía endoscópica percutánea (PEG) es un procedimiento seguro con una morbilidad poco común. Sin embargo, el número de pacientes alimentados por PEG está aumentando en todo el mundo y las complicaciones pueden llegar a ser cada vez más frecuentes. Caso clínico: se describe el caso de una mujer de 73 años que se presentó con vómitos persistentes después de la sustitución de la sonda PEG estándar por una sonda de tipo Foley. La endoscopia digestiva mostró el catéter situado en el duodeno causando obstrucción de la salida gástrica. La sonda Foley fue retirada y sustituida por una sonda PEG estándar adecuada, lo que permitió el reinicio de la alimentación por PEG. Discusión: descripciones anteriores en la literatura señalaron el riesgo de esta complicación, casi siempre asociada a la inserción de un catéter de tipo Foley. La sustitución de sondas PEG debe ser realizada por equipos experimentados; el uso de catéteres de tipo Foley para este fin debe ser evitado en la práctica clínica habitual.

The authors report the case of a 75 year-old woman admitted to the emergency room with abdominal pain and coffee ground vomiting. Marked epigastric distension with tenderness and signs of severe dehydration were present. Upper GI endoscopy showed a black esophagus covered by a large amount of dark fluid, diffuse hyperaemia and superficial erosions. Marked distortion of gastric anatomy caused by stomach rotation and a large paraesophageal hernia was observed and the pylorus was not identified. Chest X-ray and CT scan confirmed the presence of an organoaxial gastric volvulus with antero superior rotation and incarceration of the gastric antrum, which was located above the diaphragm. Immediate surgery repaired the diaphragmatic hernia, obtained volvulus reduction and a Nissen fundoplication was performed to prevent recurrence. The patient was discharged without further complications. Acute gastric volvulus is a rare entity that may manifest with vomiting due to gastric outlet obstruction and gastrointestinal bleeding associated with mucosal ischemia and sloughing. Emergency surgery wass required to resolve symptoms and prevent complications.