RESUMEN
Liver abnormalities in severe acute respiratory syndrome-coronavirus 2 infection, including hepatitis and cholestasis, have been observed in adults and are associated with worse outcomes. We describe 2 adolescents with cholestasis and hepatitis with mild presentation of severe acute respiratory syndrome-coronavirus 2 lacking typical symptoms. Our intention is to raise index of suspicion for testing and protective equipment use.
Asunto(s)
Prueba de COVID-19 , COVID-19/diagnóstico , Hepatitis Viral Humana/virología , Ictericia Obstructiva/virología , Adolescente , COVID-19/complicaciones , Femenino , Hepatitis Viral Humana/diagnóstico , Humanos , Ictericia Obstructiva/diagnóstico , Masculino , Índice de Severidad de la EnfermedadRESUMEN
NH is the most common identifiable cause of ALF in the neonate. LT is the definitive treatment for neonates with NH who have failed medical therapy. Our aim was to determine the outcomes of LT in infants with NH. Patients (less than one yr of age) with NH who were listed for LT and patients who underwent LT between 1994 and 2013 were identified from the UNOS database for analysis. Risk factors for death and graft loss were analyzed by multivariate logistic regression. Thirty-eight infants with NH with a total of 43 transplants were identified. One- and five-yr patient and graft survival were 84.2%, 81.6%, 71.1%, and 68.4%, respectively. The outcomes for NH were not significantly different when compared to the same age-matched recipients with other causes of ALF. There were no statistically significant risk factors identified for graft loss or death. Ninety infants with NH were listed for LT. Reasons for removal included transplanted (49%), death (27%), too sick to transplant (7%), and improved status (13%). LT for infants with NH has a high rate of graft loss and death; however, outcomes are comparable to the same age-matched recipients with other causes of ALF.
Asunto(s)
Bases de Datos Factuales , Hemocromatosis/cirugía , Trasplante de Hígado , Femenino , Rechazo de Injerto/cirugía , Supervivencia de Injerto , Hemocromatosis/fisiopatología , Humanos , Recién Nacido , Fallo Hepático Agudo/cirugía , Masculino , Factores de Riesgo , Resultado del Tratamiento , Estados Unidos , Listas de EsperaRESUMEN
IgG4 sclerosing cholangitis (IgG4-SC) is an immune-mediated process that results in inflammation and fibrosis of the pancreatobiliary tract. Although IgG4-SC is predominantly associated with autoimmune pancreatitis, IgG4-SC as its own entity can be difficult to diagnose. Patients with IgG4-SC are typically men over the age of 60, and present clinically with obstructive jaundice, abdominal pain, and weight loss. The diagnosis of IgG4-SC may be difficult to differentiate from primary sclerosing cholangitis (PSC) or cholangiocarcinoma. IgG4-SC is morphologically characterized by dense lymphoplasmacellular infiltration, particularly IgG4+ plasma cells and CD4+ T cells, extensive fibrosis in bile duct walls, and obliterative phlebitis. In contrast to PSC, those with IgG4-SC often have elevated serum IgG4 and can be successfully treated with immunosuppression. Here, we present the first reported case of IgG4-SC in a pediatric patient with asymptomatic elevation in liver enzymes, bile duct strictures on imaging, characteristic pathology findings, elevated serum IgG4, and excellent response to corticosteroids. Pediatric gastroenterologists and hepatologists, as well as pediatric hepatopathologists, need to be aware of IgG4-SC as a disease entity. Although certain clinical and imaging findings mimic PSC, diagnosis of IgG4-SC and its appropriate treatment with corticosteroids often lead to remission and reversal of disease.
Asunto(s)
Enfermedades Autoinmunes/diagnóstico , Conductos Biliares Intrahepáticos/patología , Colangitis Esclerosante/diagnóstico , Inmunoglobulina G/inmunología , Hígado/patología , Adolescente , Enfermedades Autoinmunes/inmunología , Pancreatocolangiografía por Resonancia Magnética , Colangitis Esclerosante/inmunología , Humanos , MasculinoRESUMEN
This is a case report of the first patient with Alagille syndrome (AGS) to undergo a partial internal biliary diversion (PIBD) for the treatment of symptoms refractory to medical therapy. Alagille syndrome is a hereditary disease resulting in chronic cholestasis and hypercholesterolemia that can lead to severe and intractable pruritus and disfiguring and debilitating xanthomas. PIBD has proven to be an effective treatment option for other causes of cholestatic liver disease. This report reviews the immediate and 2-year follow-up of a patient after this surgical procedure. The results suggest that PIBD has potential to provide relief of intractable symptoms and improve the quality of life in patients with AGS while avoiding an external stoma. It does not, however, appear to prevent the progression of liver disease. Long-term follow-up is still needed.