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 [
[1]
]. It has been reported that adults could be at risk of obesity, type 2 diabetes, and
cardiovascular complications [
[2]
]. 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).To read this article in full you will need to make a payment
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References
- Diagnosis and management of Silver-Russell syndrome: first international consensus statement.Nat Rev Endocrinol. 2017; 13: 105-117
- Adult phenotype of Russell-Silver syndrome: a molecular support for Barker-Brenner’s theory: adults with Russell-Silver syndrome.Congenit Anom. 2015; 55: 167-169
- Correct homeostasis model assessment (HOMA) evaluation uses the computer program.Diabetes Care. 1998; 21: 2191-2192
- Adipose tissue remodeling in children: the link between collagen deposition and age-related adipocyte growth.J Clin Endocrinol Metab. 2012; 97: 1320-1327
- Increased infiltration of macrophages in omental adipose tissue is associated with marked hepatic lesions in morbid human obesity.Diabetes. 2006; 55: 1554-1561
- Metabolic and adipose tissue signatures in adults with Prader-Willi syndrome: a model of extreme adiposity.J Clin Endocrinol Metab. 2015; 100: 850-859
- Paternally inherited igf2 mutation and growth restriction.N Engl J Med. 2015; 373: 349-356
- Insulin-like growth factors (IGFs), IGF receptors, and IGF-binding proteins: roles in skeletal muscle growth and differentiation.Gen Comp Endocrinol. 2010; 167: 344-351
- Autocrine action of IGF2 regulates adult β-cell mass and function.Diabetes. 2015; 64: 4148-4157
- Effect of in utero and early-life conditions on adult health and disease.N Engl J Med. 2008; 359: 61-73
- The insulin-like growth factor system in obesity, insulin resistance and type 2 diabetes mellitus.J Clin Med. 2014; 3: 1561-1574
Article info
Publication history
Published online: February 02, 2017
Accepted:
January 31,
2017
Received in revised form:
January 29,
2017
Received:
December 18,
2016
Identification
Copyright
© 2017 American Society for Metabolic and Bariatric Surgery. Published by Elsevier Inc. All rights reserved.
