Sotos syndrome or Cerebral Gigantism
Diagnosis : From around 2-4 years of age. Tall stature and dysmorphic features.
Sotos syndrome, or gigantism, usually presents in early childhood. The physical characteristics are tall stature, large hands and feet and poor circulation. There may also be special educational needs. Bone age is often advanced and puberty usually occurs early so excessive tall stature may not be a feature of adulthood.
A doctor’s recommendation to begin a child on growth hormone (GH) is based on consideration of many factors, including a complete evaluation of the child’s growth pattern, general health, medical and family history, and results of laboratory tests. Parents may have many questions when they are considering a doctor’s recommendation to start their child on GH. Perhaps most importantly, parents well want to understand the potential benefits and risks of GH treatment. The pediatric endocrinologist who is evaluating the child is the best person to answer these questions. This fact sheet about the possible effects of GH is intended to serve as a brief overview for parents, not as a substitute for a thorough discussion with the child’s health care team. Much of the information contained here about the physical effects of GH is based on the official statement of the European Society of Pediatric Endocrinology cocerning the safety and toxicity of GH, which has also been endorsed by the Lawson Wilkins Pediatric Endocrine Society in the United States.
GH deficient children will grown faster, often 2 to 3 times their pre-treatment growth rate during the first year of therapy. This increased (“catch-up”) rate wanes over time, but they should continue to grow at a normal rate while receiving therapy. GH deficient children who respond well to GH will be taller as adults than they would have been if not treated.
FDA approved indications
• GH deficiency(1985)
• Turner syndrome (1995)
• Chronic renal insufficiency – pretransplantation and post transplant (1995)
• Children born SGA who have not shown evidence of catch-up growth by 2 years of age (2001)
• Prader Willi syndrome with short stature( 2000)
• Idiopathic short stature >2. SD below the mean in height and who are unlikely to catch up in height (2003)
• Adult Growth Hormone Deficiency
• SHOX Deficiency 2006
• Noonan syndrome 2007
• HIV Wasting
• Cystic fibrosis
• X-linked hypophosphatemic rickets
• Precocious puberty
• Chronic glucorticoid use
• Thallasemia with short stature
• Trisomy syndrome
• Severe Burns
• IVF Spermatogensis
• Anti ageing
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