Adult Height in 299 Patients with Turner Syndrome with or without Growth Hormone Therapy: Results and Literature Review

Author:

Dantas Naiara C.B.,Braz Adriana F.,Malaquias Alexsandra,Lemos-Marini SofiaORCID,Arnhold Ivo J.P.,Silveira Ester R.,Antonini Sonir R.,Guerra-Junior Gil,Mendonca BereniceORCID,Jorge AlexanderORCID,Scalco Renata C.ORCID

Abstract

<b><i>Context:</i></b> Treatment with growth hormone (GH) is considered effective in improving adult height (AH) in Turner syndrome (TS). However, there are few studies comparing AH between treated patients and a concurrent untreated group. <b><i>Objective:</i></b> To assess the efficacy of GH treatment in improving AH in TS and to review previous published studies with treated and untreated groups. <b><i>Participants and Methods:</i></b> We retrospectively analyzed clinical data and AH of a large cohort of GH-treated (<i>n</i> = 168) and untreated (<i>n</i> = 131) patients with TS. Data are shown as median and interquartile range (IQR). We assessed pretreatment variables related with AH and compared our results with 16 studies that also included an untreated group. <b><i>Results:</i></b> The GH-treated group was 6.2 cm taller than the untreated group (AH = 149 cm [IQR 144.5–152.5 cm] vs. 142.8 cm [IQR 139–148 cm], <i>p</i> &#x3c; 0.001) after 4.9 years of GH treatment with a dose of 0.35 mg/kg/week. AH SDS corrected for target height (TH) was 7.2 cm higher in GH-treated patients. AH SDS ≥−2 was more frequent in GH-treated patients (43%) than in untreated patients (16%, <i>p</i> &#x3c; 0.001). AH SDS was also more frequently within the TH range in the GH-treated group (52%) than in the untreated group (15%, <i>p</i> &#x3c; 0.001). Height SDS at start of GH therapy and TH SDS were positively correlated with AH (<i>p</i> &#x3c; 0.001; <i>R</i><sup>2</sup> = 0.375). Considering the current result together with previous similar publications, a mean AH gain of 5.7 cm was observed in GH-treated (<i>n</i> = 696) versus untreated (<i>n</i> = 633) patients. <b><i>Conclusions:</i></b> Our study strengthens the evidence for efficacy of GH therapy in patients with TS from different populations.

Publisher

S. Karger AG

Subject

Endocrinology,Endocrinology, Diabetes and Metabolism,Pediatrics, Perinatology, and Child Health

Reference28 articles.

1. Gravholt CH, Andersen NH, Conway GS, Dekkers OM, Geffner ME, Klein KO, et al. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting. Eur J Endocrinol. 2017 Sep;177(3):G1–G70.

2. Rao E, Weiss B, Fukami M, Rump A, Niesler B, Mertz A, et al. Pseudoautosomal deletions encompassing a novel homeobox gene cause growth failure in idiopathic short stature and Turner syndrome. Nat Genet. 1997 May;16(1):54–63.

3. Ranke MB, Pflüger H, Rosendahl W, Stubbe P, Enders H, Bierich JR, et al. Turner syndrome: spontaneous growth in 150 cases and review of the literature. Eur J Pediatr. 1983 Dec;141(2):81–8.

4. Lyon AJ, Preece MA, Grant DB. Growth curve for girls with Turner syndrome. Arch Dis Child. 1985 Oct;60(10):932–5.

5. Los E, Rosenfeld RG. Growth and growth hormone in Turner syndrome: looking back, looking ahead. Am J Med Genet C Semin Med Genet. 2019 Mar;181(1):86–90.

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