Growth
hormone
deficiency
| Subcutaneous injection of biosynthetic (i.e., recombinant) GH (rhGH) started as soon as GH deficiency is identified.
The initial dose of rhGH is based on body weight in childhood. The dose ↑s w/↑ body weight to a maximum during puberty. When final height is achieved, rhGH dose is ↓ to 0.2-0.4 mg/d for persons age <60 yrs & 0.1-0.2 mg/d for person age >60 yrs.
|
The ideal rhGH dose will raise & maintain IGF1 levels to between mean & upper limit of normal. 1 There is ↑ support for rhGH treatment in young adults because of possible effects on fat metabolism, lean body mass, & bone mineral density. 2
|
TSH
deficiency
| Thyroid hormone replacement (L-thyroxine) 1-3 µg/kg/day | Thyroid hormone replacement should not be initiated until adrenal function has been assessed & adrenal insufficiency treated, if present. |
Micropenis in
male infants
| 50 mg testosterone enanthate intramuscularly every 4 wks for a total of 3-4 doses | |
LH & FSH
deficiency (in
those w/treated
GH deficiency
& normal
growth before
adolescence) | Sex steroid replacement to induce secondary sex characteristics
In males: starting at 12-13 yrs, monthly injections of 100 mg testosterone enanthate, gradually ↑ by 50 mg every 6 mos to a dose of 200-300 mg/mo In females: starting at 11-12 yrs, 17 beta-estradiol or estradiol valerate, cycling w/progesterone (medroxyprogesterone acetate, 5-10 mg/d) & micronized progesterone (200 mg/d from 1st to 12th day of each month)
| Treatment w/sex steroids is often continued to maintain secondary sex characteristics. |
LH & FSH
deficiency (in
those
w/untreated
GH deficiency) | Sex hormone replacement is given in lower doses & started at a later age to ensure maximal growth before epiphyseal closure. | |
|
Infertility
| Gonadotropin replacement | Note: Infertility in persons w/PROP1-related CPHD is secondary to hypogonadotropic hypogonadism; thus, appropriate treatment is gonadotropin replacement rather than use of clomiphene citrate, which requires an intact pituitary gland. |
ACTH
deficiency
|
Long-term mgmt: 8-10 mg/m2 oral hydrocortisone per 24 hrs divided into 2 or 3 doses. For minor stress (e.g., fever, minor illness), hydrocortisone dose is doubled or tripled until illness has resolved. For major stress (e.g., surgery, significant illness), hydrocortisone is ↑ to 50-100 mg & administered parenterally w/fluid replacement.
| For persons w/GH deficiency, the lowest safe dose of hydrocortisone is used to avoid interfering w/growth response to GH therapy. |
