Background: Hemophagocytic lymphohistiocytosis (HLH) is considered a medical emergency that should be recognized in patients with fever, splenomegaly, and progressive deterioration of the general condition. Laboratory findings include cytopenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. For HLH diagnosis, it is essential, although not mandatory, to perform a bone marrow biopsy. Given its nature, secondary causes of HLH should be sought, mainly infections, hemato-oncological disorders, autoimmune diseases, and auto-inflammatory conditions. Case report: We present the case of a female adolescent who presented with fever and lower gastrointestinal bleeding. Upon admission, acute liver failure and pancytopenia were documented. A bone marrow aspirate was performed, which revealed hemophagocytosis; other tests confirmed HLH diagnosis. During the diagnostic approach, inflammatory bowel disease was diagnosed. The patient received first-line treatment with an adequate response. Conclusions: Inflammatory bowel disease can be considered a cause of secondary HLH, particularly in patients with suggestive symptoms, such as digestive bleeding in the absence of other secondary causes of HLH.
La pancreatitis crónica hereditaria es una enfermedad poco frecuente que se puede presentar como pancreatitis aguda, aguda recurrente y pancreatitis crónica, tiene un curso similar a otras causas de pancreatitis aguda o crónica pero habitualmente hay una larga evolución antes del diagnóstico; en este tipo de patología los síntomas se presentan en una edad temprana, antes de la segunda década de vida, con un riesgo mayor de desarrollar adenocarcinoma de páncreas, además previo a esto pueden desarrollar complicaciones como: estenosis del conducto pancreático e insuficiencia endocrina y exocrina. El manejo implica el tratamiento médico para el dolor, insuficiencia exocrina, endocrina y en caso de complicaciones tratamiento endoscópico o quirúrgico.
introducción: el síndrome de mauriac se caracteriza por glucogenosis hepática, hepatomegalia, hipertransaminasemia, hiperlipidemia mixta, falla del crecimiento y retraso puberal. presentación de caso: adolescente femenino de 15 años de edad con diagnóstico de diabetes mellitus tipo 1 a los 6 años, falta de apego en el tratamiento con insulinas de acción rápida y prolongada. presenta hipertransaminasemia, hiperglucemia en ayunas, hepatomegalia y talla por debajo del carril familiar. discusión: el síndrome de mauriac es la principal complicación hepática de la diabetes mellitus tipo 1. el manejo temprano del síndrome evita la falla en el crecimiento y el retraso puberal.
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