Lane Laura C, Flowers Josephine, Johnstone Helen, Cheetham Tim
Department of Paediatric Endocrinology, Great North Children's Hospital, Newcastle-Upon-Tyne, UK.
Department of Paediatrics, Sunderland Royal Hospital, Sunderland, UK.
J Pediatr Endocrinol Metab. 2018 Apr 25;31(5):551-560. doi: 10.1515/jpem-2017-0363.
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.
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).
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.
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.
