title: [Hereditary protein lithiasis and calcium lithiasis: two different forms of hereditary pancreatitis].
abstract: We previously reported that the most frequent cases of chronic pancreatitis were the consequence of pancreatic lithiasis and that there were different forms of pancreatic lithiasis with different etiologies and composition of calculi. The most frequent form is the calcic lithiasis, generally due to nutritional disorders. The second most frequent form is proteic lithiasis. In this paper, we report 1.) Ten hereditary cases on a total of 36 patients presenting with proteic lithiasis (age at clinical onset 15 +/- 12 years); 2.) one hereditary case on a total of 150 patients with proteic lithiasis. In these two different maladies, the transmission seems to be dominant, autosomal with incomplete penetrance. Hereditary pancreatitis is therefore a group of at least two different diseases, hereditary protein lithiasis, the most frequent one and hereditary calcic lithiasis exceptional.
authors.affiliation: Unité de Recherches de Physiologie et Physiopathologie Digestives, U 315 INSERM, Marseille.
authors.forename: H
authors.initials: H
authors.lastname: Sarles
authors.forename: J
authors.initials: J
authors.lastname: Camarena
authors.forename: J P
authors.initials: JP
authors.lastname: Bernard
authors.forename: J
authors.initials: J
authors.lastname: Sahel
authors.forename: R
authors.initials: R
authors.lastname: Laugier
mesh_terms: D000293:Adolescent; D002118:Calcium / Q000737:chemistry*; D002137:Calculi / Q000737:chemistry* / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D010195:Pancreatitis / Q000235:genetics*; D011506:Proteins / Q000737:chemistry*; D012189:Retrospective Studies
chemical_list: D011506:Proteins; D002118:Calcium
vernacular_title: Lithiase protéique et lithiase calcique héréditaires: deux formes différentes de pancréatite héréditaire.