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Knowing your patients’ insulin-like growth factor-1 (IGF-1) level is an important step in the diagnosis of severe primary IGF-1 deficiency (SPIGFD).3 A diagnosis of SPIGFD is made when a child’s IGF-1 concentration is a standard deviation score (SDS) of ≤ -3.0 and their growth hormone (GH) levels are within the normal range or are elevated, and their height is an SDS of ≤ -3.0.1
After the general assessment of a patient with short stature (which may include a review of the patient’s growth history and medical history, physical examination, or general laboratory tests) to investigate other causes, an investigation may then be conducted to assess the growth hormone (GH)-IGF-1 axis prior to the initiation of therapy. 3,8
GH, growth hormone; IGF-1; insulin-like growth factor-1; PIGFD, primary IGF-1 deficiency; SPIGFD, severe primary IGF-1 deficiency; SDS, standard deviation score. *Defined by a height of SDS ≤ -3.0, basal IGF-1 SDS ≤ -3.0, and normal or elevated GH
A patient with primary IGFD may exhibit a number of unique clinical features in addition to having short stature, including: 6,9
SPIGFD may be the cause of a severe impairment in growth. 1
The diagnosis of SPIGFD is made when a patient meets the following criteria:
Data and formula from Centers for Disease Control and Prevention. SDS, standard deviation score. *SPIGFD is defined by a height of SDS ≤ -3.0 (shaded area), basal IGF-1 SDS ≤ -3.0, and normal or elevated GH.1
Testing for severe primary IGF1 deficiency in children with short stature and suspected defect in the GH-IGF-1 axis is essential because these children have a limited time to reach their full growth potential with treatment before epiphyseal fusion. Timely diagnosis allows for the condition to be treated for a longer period prior to epiphyseal fusion.1,3
Tests that may be used to help distinguish SPIGFD from GHD include:10
In children previously diagnosed with a growth disorder and treated with GH, treatment response may vary by diagnosis, as well as by age and gender. If a child’s growth velocity is tracking below the average on an appropriate growth chart, this may be an indication that additional evaluation or re-evaluation is necessary. This evaluation may include an assessment of compliance, and whether other diagnoses, including SPIGFD, should be considered.
Common adverse reactions include hypoglycemia, local and systemic hypersensitivity, and tonsillar hypertrophy.
INCRELEX® (mecasermin) is indicated for the treatment of growth failure in pediatric patients aged 2 years and older with severe primary IGF-1 deficiency* (IGFD), or with hormone (GH) gene deletion who have developed neutralizing antibodies to GH.1
Limitations of use: INCRELEX® is not a substitute to GH for approved GH indications. INCRELEX® is not indicated for use in patients with secondary forms of IGFD, such as GH deficiency, malnutrition, hypothyroidism, or chronic treatment with pharmacologic doses of anti-inflammatory steroids.1
*Severe primary IGF-1 deficiency (IGFD) is defined by height standard deviation score ≤ -3.0 and basal IGF-1 standard deviation score ≤ -3.0 and normal or elevated GH.