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摘要
Background Adolescent acromegaly is a rare disorder and these patients present with tall stature/gigantism, tumor mass effects and menstrual irregularities
Patients and Methods 34 consecutive (26 males) patients having onset of disease prior to 21 years of age were included in this retrospective analysis Their clinical features and treatment outcome were studied
Results Mean age and lag time at presentation were 21 6 +/- 3 9 years and 5 1 +/- 3 5 years respectively Common presenting manifestations included acral enlargement, tumor mass effects and menstrual irregularities Mean height at presentation was 174 6 +/- 13 7 cms (range 150 - 210 cm) and one third had gigantism (height >= 97(th) percentile, WHO growth charts) Hypertension and glucose intolerance were seen in 15% and 23 5% respectively Mean nadir GH after glucose load was 58 2 +/- 13 7 ng/ml and IGF -1 was 534 8 +/- 132 8 ng/ml Half of the patients had concomitant hyperprolactinemia Almost all (97%) had macroadenoma and anterior pituitary hormone deficiencies were frequent (75%) Patients with gigantism were younger (19 6 +/- 4 9 vs 22 6 +/- 2 9 years, p=0 001), had higher GH values (66 68 +/- 27 22 vs 53 98 +/- 15 99 ng/ml, p=0 04) and hypogonadism was more common (90 9% vs 56 5%, p=0 03) than those with normal stature 32 patients (94 1%) were treated primarily with surgery, 7(21 9%) received post operative radiotherapy Mean duration of follow up was 33 1 +/- 10 1 months Only 30% had nadir GH values of < 1ng/ml
Conclusion One third of adolescent patients had acrogigantism These patients were younger, had higher GH levels and concurrent hypogonadism was more common Cure could be achieved only in about one third of the patients
- 出版日期2010-10
- 单位河北医科大学
