Laura C Lane1, Josephine Flowers2, Helen Johnstone1, Tim Cheetham1,3. 1. Department of Paediatric Endocrinology, Great North Children's Hospital, Newcastle-Upon-Tyne, UK. 2. Department of Paediatrics, Sunderland Royal Hospital, Sunderland, UK. 3. Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
Abstract
BACKGROUND: There is little adult height data in patients with familial male-limited precocious puberty (FMPP) and no management consensus. We assessed the treatment and adult height in local patients with FMPP and those reported in the literature. METHODS: Growth data were obtained on four local patients with FMPP and a search performed to obtain management details and adult height data on cases in the literature. UK (90) population standards were used to calculate standard deviation scores (SDS). RESULTS: Adult height data were available on 25 men with FMPP of whom 21 were treated. Median adult height SDS of patients was -1.5 SD with a mid-parental target of -0.6 SD (p=0.1). Eight patients (32%) had an adult height above the mid-parental target and seven patients (28%) had a height >2 SD below the mean. The median height SD was -0.03 in untreated patients and +0.5 SD in those receiving an aromatase inhibitor. There was no relationship between height and age at presentation. CONCLUSIONS: Aromatase inhibitor therapy is associated with a positive height outcome in FMPP but the outcome with and without intervention is unpredictable. Clinicians need to be cautious when counselling families about the potential height outcome in FMPP.
BACKGROUND: There is little adult height data in patients with familial male-limited precocious puberty (FMPP) and no management consensus. We assessed the treatment and adult height in local patients with FMPP and those reported in the literature. METHODS: Growth data were obtained on four local patients with FMPP and a search performed to obtain management details and adult height data on cases in the literature. UK (90) population standards were used to calculate standard deviation scores (SDS). RESULTS: Adult height data were available on 25 men with FMPP of whom 21 were treated. Median adult height SDS of patients was -1.5 SD with a mid-parental target of -0.6 SD (p=0.1). Eight patients (32%) had an adult height above the mid-parental target and seven patients (28%) had a height >2 SD below the mean. The median height SD was -0.03 in untreated patients and +0.5 SD in those receiving an aromatase inhibitor. There was no relationship between height and age at presentation. CONCLUSIONS: Aromatase inhibitor therapy is associated with a positive height outcome in FMPP but the outcome with and without intervention is unpredictable. Clinicians need to be cautious when counselling families about the potential height outcome in FMPP.
Authors: Chelsi Flippo; Vipula Kolli; Melissa Andrew; Seth Berger; Tricia Bhatti; Alison M Boyce; Daniel Casella; Michael T Collins; Emmanuèle Délot; Joseph Devaney; Stephen M Hewitt; Thomas Kolon; Ashwini Mallappa; Perrin C White; Deborah P Merke; Andrew Dauber Journal: J Endocr Soc Date: 2022-08-12