Hyposomatotropism Treatment & Management

Updated: Oct 30, 2015
  • Author: Sunil Kumar Sinha, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
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Treatment

Medical Care

Replacement dosages of recombinant human growth hormone (rhGH)

For patients with hyposomatotropism, effective replacement dosages of rhGH are 0.175-0.75 mg/kg/wk, and therapy should be individualized. Dividing the weekly dose into 6 or 7 daily subcutaneous injections is more effective than dividing the total dose into 3 doses administered on alternate days.

Height outcome

In the authors' personal experience in treating patients with GH deficiency (GHD) starting at younger than 4 years, the patient's final height may exceed the target height.

The patient's final height is best correlated with his or her pretreatment chronologic age. It is also related to the height standard deviation score and to the child's predicted adult height, duration of therapy, and birth length. In many studies, the final height was closely correlated with the height standard deviation score, the patient's age at onset of puberty, weight, and serum concentrations of GH binding protein (indicators of GH receptor mass).

Early recognition of GHD is essential for an optimal outcome in terms of height.

Patterns of growth during childhood

Growth during childhood follows predictable patterns.

  • Before birth - Prenatal growth averages 1.2-1.5 cm each week.

  • Infancy and childhood - Growth velocity increases to 15 cm (approximately 6 inches) per year over the first 2 years and then slows to 6 cm (approximately 2 inches) per year until puberty.

  • Puberty - A second peak in growth velocity occurs during puberty. This peak occurs earlier but is lower in magnitude and is shorter in girls than in boys.

The table below summarizes the pubertal peak of growth velocity in children.

Table. Characteristics of the Pubertal Peak of Growth Velocity in Girls and Boys (Open Table in a new window)

Characteristic

Girls

Boys

Mean age at peak height velocity, y

11.5

13

Magnitude, cm/y

8.5

9.5

Duration, y

5

6

In children who are receiving rhGH therapy, growth velocity usually exceeds reference values in the first few years. Therefore, suspect partial GH resistance or noncompliance if suboptimal growth velocity is observed at the beginning of rhGH therapy.

Treatment principles

Monitor patients with visits every 3 months.

Conduct physical examination.

  • Perform funduscopy to exclude pseudotumor cerebri.

  • Pubertal staging should be performed during each visit because gonadal steroids have a notable effect on skeletal maturation.

  • Monitor patients by measuring their height in centimeters and weight in kilograms.

Obtain interim histories.

Monitor medical therapy.

  • Adjust drug dosages by weight, and monitor patients for adverse effects of therapy (see Medication).

  • For patients whose condition does not respond well to weight-based therapy, some clinicians advocate titrating dosages according to insulinlike growth factor (IGF)-1 levels. The goal is to maintain an IGF-1 value in the upper quartile for the child's age and sex.

Bone age can be used to determine the remaining growth potential for patients with GHD who are approaching their final height. Bone ages have no proven role in monitoring GH therapy.

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Surgical Care

Brain tumors and/or secondary hydrocephalus indicate a need for consultation with a neurosurgeon.

A pediatric surgeon may be consulted to address cleft lip repair, cleft palate repair, and/or cosmetic reconstruction of the facies in a patient with long-standing GHD.

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Consultations

Consultations with the specialists listed below may aid in the care of patients with hyposomatotropism.

  • Neurosurgeon

  • Plastic surgeon

  • Radiation oncologist

  • Neurooncologist

  • Psychologist

  • Nutritionist

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