Restoration of height after 11 years of letrozole treatment in 11?-hydroxylase deficiency

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dc.authorid0000-0002-1044-6888
dc.contributor.authorAtay, Zeynep
dc.contributor.authorTuran, Serap
dc.contributor.authorBu?daycı, Onur
dc.contributor.authorGüran, Tülay
dc.contributor.authorBereket, Abdullah
dc.date.accessioned2019-12-27T06:32:05Z
dc.date.available2019-12-27T06:32:05Z
dc.date.issued2020
dc.departmentİstanbul Medipol Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı
dc.description.abstract11?-hydroxylase deficiency (11?-OHD) is the second most common form of congenital adrenal hyperplasia (CAH). Males with 11?-OHD CAH are often diagnosed late with a significantly advanced bone age leading to a poor height prognosis due to early closure of epiphysis. Delaying epiphyseal fusion by treatment of aromatase inhibitors (AIs) might be a useful strategy in patients with very advanced bone ages. However, there are limited data regarding the effect on final height and long-term safety of this approach. We report our experience with 11 years of letrozole treatment and 17 years of follow-up in a boy with 11?-OHD. He presented at 2 years and 11 months of age with a bone age of 13 years (predicted adult height, PAH, 129.5 cm). Letrozole was added after 1 year of glucocorticoid treatment due to no improvement in height prognosis (130 cm), and continued until the age of 14 years and 11 months. He also received GnRH analog treatment at 10 years and 3 months of age for 2.5 years due to central activation of puberty. He reached a final height of 165.2 cm (35.2 cm above his PAH). This long-term treatment with letrozole was associated with changes in vertebral morphology such as vertebral body end-plate changes, Schmorl nodes, and mild protrusions in the intervertebral discs. Testicular volumes, gonadotropins, testosterone, and anti-Müllerian hormone were normal at age 20 years. A spermiogram showed a normal count but impaired sperm motility and morphology. This unique case represents the longest duration of AI treatment reported in CAH and the first case in which letrozole was started before puberty with the final height reported. We conclude that AIs may restore height in selected patients with CAH with very advanced bone age and severely compromised height prognosis.
dc.identifier.citationAtay, Z., Turan, S., Bu?daycı, O., Güran, T. ve Bereket, A. (2020). Restoration of height after 11 years of letrozole treatment in 11?-hydroxylase deficiency. Hormone Research in Paediatrics, 92(3), 203-208. http://doi.org/10.1159/000501456
dc.identifier.doi10.1159/000501456
dc.identifier.endpage208
dc.identifier.issn1663-2818
dc.identifier.issn1663-2826
dc.identifier.issue3
dc.identifier.scopusqualityQ1
dc.identifier.startpage203
dc.identifier.urihttp://doi.org/10.1159/000501456
dc.identifier.urihttps://hdl.handle.net/20.500.12511/4753
dc.identifier.volume92
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherKarger
dc.relation.ispartofHormone Research in Paediatricsen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subject11?-Hydroxylase Deficiency
dc.subjectAdvanced Bone Age
dc.subjectAromatase Inhibitors
dc.subjectCongenital Adrenal Hyperplasia
dc.subjectLetrozole
dc.subjectShort Stature
dc.titleRestoration of height after 11 years of letrozole treatment in 11?-hydroxylase deficiency
dc.typeArticle

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