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Volume 125, Issue 5, Pages (November 2003)

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1 Volume 125, Issue 5, Pages 1470-1475 (November 2003)
Familia liver adenomatosis associated with hepatocyte nuclear factor 1α inactivation1   Yannick Bacq, Emmanuel Jacquemin, Charles Balabaud, Emmanuelle Jeannot, Beatrice Scotto, Sophie Branchereau, Christophe Laurent, Pascal Bourlier, Daniele Pariente, Anne De Muret, Monique Fabre, Paulette Bioulac-Sage, Jessica Zucman-Rossi  Gastroenterology  Volume 125, Issue 5, Pages (November 2003) DOI: /j.gastro

2 Figure 1 Wedge liver biopsy specimen (case A1). The general liver architecture is preserved; numerous foci of steatotic and clear hepatocytes are dispersed in the parenchyma (resembling nontumoral liver of case A2; see Figure 2). Figure 2 Part of left hepatectomy (case A2). Near a typical steatotic adenoma (on the right), the liver parenchyma exhibited several steatotic microadenomas and foci (on the left). Gastroenterology  , DOI: ( /j.gastro )

3 Figure 1 Wedge liver biopsy specimen (case A1). The general liver architecture is preserved; numerous foci of steatotic and clear hepatocytes are dispersed in the parenchyma (resembling nontumoral liver of case A2; see Figure 2). Figure 2 Part of left hepatectomy (case A2). Near a typical steatotic adenoma (on the right), the liver parenchyma exhibited several steatotic microadenomas and foci (on the left). Gastroenterology  , DOI: ( /j.gastro )

4 Figure 2 Part of left hepatectomy (case A2). Near a typical steatotic adenoma (on the right), the liver parenchyma exhibited several steatotic microadenomas and foci (on the left). Gastroenterology  , DOI: ( /j.gastro )

5 Figure 3 Families with germline mutation in HNF-1α. Diagonal hatched boxes and circles indicate patients with liver adenomatosis, and vertical hatched boxes and circles indicate patients with diabetes. The black circle shows a patient with both gestational diabetes and liver adenomatosis. The result of the HNF-1α mutation screening is indicated by NM (patient with heterozygous mutation) or NN (patients without mutation). Age at diagnosis or testing is indicated in years (y). Gastroenterology  , DOI: ( /j.gastro )

6 Figure 4 MRI (case B3). Liver adenomatosis diagnosed by systematic familial screening in a 23-year-old woman without hepatic symptoms. (A) On coronal balanced fast field echo MRI, the largest lesion (9 cm) distorted the liver contours and repressed venous contours. (B) On the transverse T1-weighted MRI obtained during the hepatic arterial phase, the lesions were large and hyperintense with borders well defined. Gastroenterology  , DOI: ( /j.gastro )


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