RESUMO
Hepatic glycogenosis (HG) is characterized by excessive glycogen accumulation in hepatocytes and represents a hepatic complication of diabetes that particularly occurs in patients with longstanding poorly controlled type 1 diabetes (T1D). HG has been reported to be a very rare disease, although it is believed to be extremely underdiagnosed because it is not possible to distinguish it from non-alcoholic fatty liver disease (NAFLD) unless a liver biopsy is performed. In contrast to HG, NAFLD is characterized by liver fat accumulation and is the more likely diagnosis for patients with type 2 diabetes and metabolic syndrome. The pathogenesis of HG involves the concomitant presence of insulin and excess glucose, which increases glycogen storage in the liver. HG is characterized by a transient elevation in liver transaminases and hepatomegaly. Differentiating between these two conditions is of the utmost importance because HG is a benign disease that is potentially reversible by improving glycemic control, whereas NAFLD can progress to cirrhosis. Therefore, HG should be suspected when liver dysfunction occurs in patients with poorly controlled T1D. The aim of this article is to review the epidemiology, clinical characteristics, pathogenesis and histology of HG.
RESUMO
La hipofisitis primaria corresponde a un proceso inflamatorio heterogéneo de la glándula hipofisaria y es una entidad a tener en cuenta en el diagnóstico diferencial de las lesiones ocupantes de espacio de lasilla turca. Su etiología no está bien definida, aunque hay varios aspectos que indican su origen autoinmunitario. Histológicamente hay 3 subtipos diferentes: linfocítica, granulomatosa y xantomatosa, y se ha postulado que podrían corresponder a diferentes expresiones de la misma entidad. El tipo y el grado de infiltración linfocitaria llevarán asociados una correspondencia clinicopatológica (diabetes insípida, hipopituitarismo, cefalea y alteraciones visuales como clínica más frecuente). En los casos de afección de hipófisis anterior únicamente, hablaremos de adenohipofisitis linfocitaria; si es del lóbulo posterior, infundíbulo neurohipofisitis linfocitaria, y si fuese una afección general, panhipofisitis linfocitaria. La importancia de reconocerla yace en la posibilidad de respuesta a un tratamiento antiinflamatorio (AU)
Primary hypophysitis is a heterogenous inflammatory process of the pituitary that should be included in the differential diagnosis of space-occupying lesions of the sellar region. The etiology of primary hypophysitis has still not been fully elucidated, although an autoimmune origin is suspected. Histologically, primary hypophysitis includes three main different subtypes: lymphocytic, granulomatous and xanthomatous; the question of whether these are truly distinct entities or simply different expressions of the same disease remains unclear. The type and degree of lymphocytic infiltration determine the clinical and pathological symptoms (most frequently diabetes insipidus, hypopituitarism, headache and visual defects). If only the anterior pituitary is affected by infiltration, the entity is known as lymphocytic adenohypophysitis, when the posterior lobe and the infundibulum are infiltrated, lymphocytic-infundibuloneurohypophysitis, and if the infiltration is global, lymphocytic panhypophysitis. The importance of recognizing this entity lies in the possibility of obtaining a response to medical antiinflammatory therapy (AU)
