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El-Guindi MA et al. Hepatic immunohistochemistry of bile transporters in progressive familial intrahepatic cholestasis El-Guindi MA et al. In this issue of Annals of Hepatology, investigators from the National Liver Institute at Menofiya University aimed to evaluate hepatic expression of proteins involved in bile acid export and homeostasis as a potential means to distinguish progressive familial intrahepatic cholestasis (PFIC) from other causes of neonatal cholestasis. The study included 50 pediatric patients (median age 86.5 days), of whom 25 were diagnosed phenotypically as PFIC (2 with PFIC1, 17 with PFIC2 and 6 with PFIC3) and 25 with a variety of non-PFIC cholestatic disorders. Expression of bile salt export pump (BSEP) and multidrug resistance 3 (MDR3) proteins was assayed in these 50 patients by immunohistochemistry on deparaffinized liver tissue sections from Tru-Cut needle biopsy specimens and compared between groups. As expected, expression of BSEP and MDR3 proteins was less frequent in PFIC patients compared to non-PFIC (p = 0.077 and p = 0.048, respectively), thus the absence of these proteins was suggestive of PFIC, although not exclusively specific. Interestingly, none of the patients in the PFIC group were positive for both BSEP and MDR3; therefore, positive staining for both proteins appears to rule-out PFIC with a negative predictive value of 100%. El-Guindi et al. concluded that MDR3 and BSEP immunostaining is a PFIC and in differentiating it from other causes of neonatal cholestiasis.

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2016-06-23   |   208 visitas   |   Evalua este artículo 0 valoraciones

Vol. 15 Núm.2. Marzo-Abril 2016 Pags. 152-153 Ann Hepatol 2016; 15(2)