Key Pediatric Clinical Practice Guidelines in 2017

John Anello; Brian Feinberg; John Heinegg; Yonah Korngold; Richard Lindsey; Cristina Wojdylo; Olivia Wong, DO

Disclosures

January 10, 2018

In This Article

Growth Failure in Children

Drug and Therapeutics Committee and Ethics Committee of the Pediatric Endocrine Society

Use growth hormone (GH) to normalize adult height (AH) and avoid extreme shortness in children and adolescents with growth hormone deficiency (GHD).

Suggest against routine cardiac testing, dual x-ray absorptiometry (DXA) scanning, and measurement of lipid profiles in children and adolescents treated with GH.

Establish a diagnosis of GHD without GH provocative testing in patients possessing all of the following 3 conditions: auxological criteria, hypothalamic-pituitary defect (such as major congenital malformation [ectopic posterior pituitary and pituitary hypoplasia with abnormal stalk], tumor, or irradiation), and deficiency of at least one additional pituitary hormone.

GHD due to congenital hypopituitarism should be diagnosed without formal GH provocative testing in a newborn with hypoglycemia who does not attain a serum GH concentration above 5 µg/L and has deficiency of at least one additional pituitary hormone and/or the classical imaging triad (ectopic posterior pituitary and pituitary hypoplasia with abnormal stalk).

Recommend against reliance on GH provocative test results as the sole diagnostic criterion of GHD.

Suggest sex steroid priming prior to provocative GH testing in prepubertal boys older than 11 yr and in prepubertal girls older than 10 yr with AH prognosis within −2 SD of the reference population mean in order to prevent unnecessary GH treatment of children with constitutional delay of growth and puberty.

Recommend against the use of spontaneous GH secretion in the diagnosis of GHD in a clinical setting.

Recommend an initial GH dose of 0.16-0.24 mg/kg/wk (22-35 µg/kg/day) with individualization of subsequent dosing.

Suggest measurement of serum insulin-like growth factor-I (IGF-I) levels as a tool to monitor adherence and IGF-I production in response to GH dose changes. Suggest that the GH dose be lowered if serum IGF-I levels rise above the laboratory-defined normal range for the age or pubertal stage of the patient.

During puberty, recommend against the routine increase in GH dose to 0.7 mg/kg/wk in every child with GHD.

Recommend that GH treatment at pediatric doses not continue beyond attainment of a growth velocity below 2─2.5 cm/yr. The decision to discontinue pediatric dosing prior to attainment of this growth velocity should be individualized.

Recommend that prospective recipients of GH treatment receive anticipatory guidance regarding the potential adverse effects of intracranial hypertension, slipped capital femoral epiphysis (SCFE), and scoliosis progression.

Recommend monitoring of GH recipients for potential development of intracranial hypertension, SCFE, and scoliosis progression by soliciting pertinent history and performing a physical examination at every follow-up clinic visit; further testing should be pursued if indicated.

Recommend re-assessment of both the adrenal and thyroid axes after initiation of GH therapy in patients whose cause of GHD is associated with possible multiple pituitary hormone deficiencies (MPHD).

For GH initiation after completion of tumor therapy with no evidence of ongoing tumor, a standard waiting period of 12 mo to establish "successful therapy" of the primary lesion is reasonable, but can also be altered depending on individual patient circumstances.

Recommend that patients with multiple (≥3) pituitary hormone deficiencies regardless of etiology, or GHD with a documented causal genetic mutation or specific pituitary/hypothalamic structural defect except ectopic posterior pituitary, be diagnosed with persistent GHD.

Recommend re-evaluation of the somatotropic axis for persistent GHD in persons with GHD and deficiency of only one additional pituitary hormone, idiopathic isolated GHD (IGHD), IGHD with or without a small pituitary/ectopic posterior pituitary, and after irradiation.

Suggest that measurement of the serum IGF-I concentration be the initial test of the somatotropic axis if re-evaluation of the somatotropic axis is clinically indicated.

Recommend GH provocative testing to evaluate the function of the somatotropic axis in the transition period if indicated by a low IGF-I level.

Suggest that GH treatment be offered to individuals with persistent GHD in the transition period. There is evidence of benefit; however, the specifics of the patient population that benefits, the optimal time to re-initiate treatment, and the optimal dose are not clear.

Because there is overlap in response between dosing groups, suggest initiating GH at a dose of 0.24 mg/kg/wk, with some patients requiring up to 0.47 mg/kg/wk.

Recommend the use of IGF-I therapy to increase height in patients with severe primary IGF-I deficiency (PIGFD).

Recommend a trial of GH therapy before initiating IGF-I for patients with unexplained IGF-I deficiency. Patients with hormone signaling defects known to be unresponsive to GH treatment can start directly on IGF-I replacement; these include patients with very low or undetectable levels of GH-binding protein (GHBP) and/or proven GH receptor (GHR) gene mutations known to be associated with Laron syndrome/GH insensitivity syndrome (GHIS), GH-neutralizing antibodies, STAT5b gene mutations, and IGF1 gene deletion or mutation.

Suggest an IGF-I dose of 80-120 µg/kg BID. Similar short-term outcomes were seen with 80 and 120 μg, but published studies had limitations, and there is no strong evidence supporting superiority of one dose over the other.

Reference

  • Guidelines for growth hormone and insulin-like growth factor-I treatment in children and adolescents: growth hormone deficiency, idiopathic short stature, and primary insulin-like growth factor-I deficiency. Drug and Therapeutics Committee and Ethics Committee of the Pediatric Endocrine Society. Horm Res Paediatr. 2016;86:361-97. https://www.karger.com/Article/FullText/452150

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