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Metabolic signatures in an adolescent with Silver-Russell syndrome and outcomes after bariatric surgery

  • Héléna Mosbah
    Affiliations
    Institute of Cardiometabolism and Nutrition, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Paris 13, Paris, France

    Sorbonne Universités, University Paris 06, Paris, France
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  • Irène Netchine
    Affiliations
    Sorbonne Universités, University Paris 06, Paris, France

    Assistance Publique-Hôpitaux de Paris-Armand Trousseau Hospital, Department of Pediatric Endocrinology, Paris, France
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  • Christine Poitou
    Correspondence
    Correspondence: Christine Poitou, M.D., Ph.D., Assistance Publique-Hôpitaux de Paris, Pitié Salpêtrière Hospital, IE3 M, 47-83 Boulevard de l’hôpital, 75671 Paris Cedex 13.
    Affiliations
    Institute of Cardiometabolism and Nutrition, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Paris 13, Paris, France

    Sorbonne Universités, University Paris 06, Paris, France
    Search for articles by this author
Published:February 02, 2017DOI:https://doi.org/10.1016/j.soard.2017.01.044
      Silver-Russell syndrome (SRS) is a rare epigenetic disorder associated with hypomethylation of the 11 p15 paternal imprinting center region 1 (ICR1) regulating the insulin-like growth factor-2 (IGF2)/H19 locus in 30%–60% of cases. The phenotype of SRS patients is well described in children, including prenatal and postnatal growth retardation, relative macrocephaly at birth, protruding forehead, body asymmetry, and feeding difficulties in early life [
      • Wakeling E.L.
      • Brioude F.
      • Lokulo-Sodipe O.
      • et al.
      Diagnosis and management of Silver-Russell syndrome: first international consensus statement.
      ]. It has been reported that adults could be at risk of obesity, type 2 diabetes, and cardiovascular complications [
      • Takenouchi T.
      • Awazu M.
      • Eggermann T.
      • Kosaki K.
      Adult phenotype of Russell-Silver syndrome: a molecular support for Barker-Brenner’s theory: adults with Russell-Silver syndrome.
      ]. In severe obesity refractory to medical treatment, bariatric surgery has proven its efficiency regarding weight loss and improvement of cardiometabolic co-morbidities. However, no data are available regarding the effectiveness of bariatric surgery in patients with SRS with severe obesity. We report the case of an obese adolescent with SRS in which detailed explorations of her metabolic condition, including body composition and adipose tissue alterations, have been performed, and we present the outcomes of laparoscopic sleeve gastrectomy (SG).
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