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Standards of Care in Diabetes2024. Diabetes Care , 47(Suppl 1)

For patients with obesity, the increased risk is even higher

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Its glucagon receptor affinity is tuned specifically to engage hepatic GCGR without producing a counter-regulatory hyperglycemic signal that would negate the GLP-1 and GIP contributions

Major characteristics include infantile lethargy and hypotonia causing poor feeding and failure to thrive, developmental and intellectual disability, hypogonadism (small external genitalia and pubertal insufficiency), hyperphagia leading to morbid obesity if uncontrolled, short stature, characteristic facial appearance and body habitus, and a typical behavioral phenotype that includes temper tantrums and compulsive traits