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Hyper-responsiveness of patients with limb-girdle dystrophy to human growth hormone.

ABSTRACT The effect of human growth hormone (hGH) was measured upon the N, P, Na and K balance and body weight (bw) of 6 normal subjects (ages 27–55) and of 5 patients with limbgirdle muscular dystrophy of similar age. hGH was administered for seven days at a dosage of 0.0168, 0.0532 or 0.168 U/kg bw¾/day (doses A, B and C, respectively). Doses A and B were ineffective in all 6 normals, and dose C produced a weak anabolic effect in only 2 of these subjects. Contrastingly, dose C stimulated retention of N, P, Na and K and gain in wt in all 5 dystrophies; doses B and A caused an anabolic response in 4 and in 3 of these cases, respectively. Patients with limb-girdle dystrophy are at least 7 times more sensitive to hGH than normal subjects of the same age.