Abstract
HHG in AIP has not been reported. A 20 yr. old male with AIP and inappropriate secretion of ADH was hyposmic, tall (178 cm), eunuchoid (U/L, 0.84), had a high-pitched voice, absent facial hair and Tanner 2-3 pubic hair. His testes were small (R, 8 ml; L, 10 ml). Karyotype was XY. Basal serum FSH, 5.5 mIU/ml; LH, 5.6 mIU/ml and T, 54 ng/dl were all low and PRL was normal. Mean nocturnal LH (6.5 ± 0.2 (SE) mIU/ml) was low and showed no periodic pulses. Mean nocturnal T (156 ± 13 ng/dl) was also low, with minimal fluctuations between 2200 h and 0300 h; however, between 0300 h and 0800 h two peaks of T occurred simultaneously with those of cortisol. The responses of LH to LHRH and TSH and PRL to TRH were normal. Nocturnal GH secretion was characterized by 3 peaks. Basal GH was normal and increased to 16 ng/ml during AITT and remained above 10 ng/ml between 60 and 150 minutes. Basal T, 252 ng/dl, increased to 2049 ng/dl after 4 days of im hCG (5000 units/d). Basal 24 h urine Δ amino levulinic acid (ALA), 18 mg and porphobilinogen (PBG), 68 mg, respectively, were high and did not change after 4 days of hCG (ALA, 25 mg/d; PBG, 51 mg/d). Three attacks of AIP occurred during 8 months prior to T therapy, but none occurred during 7 months of hCG or T therapy. Conclusions: 1) Hypothalamic dysfunction in AIP may cause HHG 2) hCG and T do not alter ALA and PBG excretion and may protect these patients from repeated attacks.
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Radfar, N., Foley, T., Katoh, A. et al. 327 HYPOGONADOTROPIC HYPOGONADISM (HHG) IN ACUTE INTERMIT TENT PORPHYRIA (AIP). Pediatr Res 12 (Suppl 4), 418 (1978). https://doi.org/10.1203/00006450-197804001-00332
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DOI: https://doi.org/10.1203/00006450-197804001-00332