Journal of pediatric endocrinology & metabolism : JPEM · 2018
Adult height in patients with familial male-limited precocious puberty and the role of an aromatase inhibitor in patient management.
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Lane Laura C, Flowers Josephine, Johnstone Helen, Cheetham Tim
Аннотация
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.
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.