Reduced spontaneous growth hormone secretion in patients with Turner's syndrome.
We evaluated growth hormone (GH) secretion in 81 patients with Turner's syndrome (TS) (mean age 10.7+/-3.6 y) with respect to karyotype, auxological characteristics and growth response to GH treatment (1 IU/kg/wk). None of the patients had spontaneous puberty or had started replacement therapy with estrogens. Thirty-nine patients (48%) had monosomia 45X, 29 (36%) structural abnormalities of the X chromosome and 13 (16%) X mosaicism. Before the start of GH therapy, each patient underwent an evaluation of mean nocturnal GH concentration (MGHC) and 75 patients also underwent 2 pharmacological tests. MGHC of the TS patients did not differ from that of 29 prepubertal GH-deficient girls (GH peaks < 8 microg/l after pharmacological tests) and both groups were lower (p < 0.0001 and p < 0.0005, respectively) than MGHCs of 27 short normal girls (GH peak > 8 microg/l). MGHC of the patients with TS was negatively correlated (p < 0.001) with bodyweight excess (BWE) at multiple regression analysis. MGHC of the TS patients with BWE < 20% was significantly higher (p < 0.02) than that of the TS patients with BWE > 20%, but again did not differ from that of the GH-deficient patients and was lower (p < 0.001) than that of the short normal girls. MGHC did not significantly differ between the 3 groups subdivided according to karyotype. Forty-four percent of the TS patients showed GH responses to pharmacological tests < 8 microg/l. Height velocity SDS at first and second year of therapy was not influenced by MGHC levels, chronological or bone age, target height or BWE. In conclusion, spontaneous secretion in our patients with TS was lower than that of the short normal prepubertal girls and did not differ from that of GH-deficient subjects, even if we excluded overweight patients. The level of GH secretion was unable to predict GH response to treatment.
First Paediatric Clinic, University of Bologna, Italy.
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Pirazzoli
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Mazzanti
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Bergamaschi
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Perri
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Scarano
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Nanni
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Zucchini
S
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Gualandi
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Cicognani
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Cacciari
D000293:Adolescent; D000365:Age Determination by Skeleton; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D015259:Dopamine Agents; D004314:Down Syndrome / Q000150:complications; D001330:Electronic Data Processing; D005260:Female; D006130:Growth Disorders / Q000150:complications / Q000175:diagnosis; D019382:Human Growth Hormone / Q000097:blood / Q000172:deficiency* / Q000378:metabolism* / Q000627:therapeutic use; D006801:Humans; D007621:Karyotyping; D007980:Levodopa; D008297:Male; D011863:Radioimmunoassay; D014424:Turner Syndrome / Q000150:complications / Q000188:drug therapy / Q000378:metabolism*; D014960:X Chromosome / Q000235:genetics
D015259:Dopamine Agents; D019382:Human Growth Hormone; D007980:Levodopa