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Branchial dysplasia, mental deficiency, club feet, and inguinal herniae: a report of two further cases associated with paucity of interlobular bile ducts.
  1. J C Lambert,
  2. M C Saint-Paul,
  3. F Bastiani,
  4. V Paquis,
  5. A M Saunieres
  1. Unité de Génétique Médicale, Hôpital de Cimiez, Nice, France.


    The observation of two new cases in a previously reported family has brought about a change in the delineation of the syndrome initially defined. To the abnormalities already described (branchial dysplasia, mental deficiency, club feet, inguinal herniae) must be added a paucity of interlobular bile duts; the relationship between this new syndrome and the Alagille syndrome requires reconsideration.

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