Pubertal induction in girls with ovarian insufficiency aims to mimic normal puberty, a highly complex process. Here we amalgamate the sparse global evidence and propose three options for pubertal induction regimens including oral ethinyloestradiol, and oral and transdermal 17β-oestradiol. The introduction of progestogens is discussed and the transition to hormone supplementation for adult women. The merits and disadvantages of the different options are detailed. The available evidence indicates that transdermal 17β-oestradiol has the most favourable efficacy, safety and cost profile but randomised controlled trials are urgently required to determine which regimen provides the best clinical outcomes.

Research into experiences of social isolation and loneliness has predominantly focused on older adults, i.e. post-retirement, as a significant at-risk group. Similarly, research exploring disability and loneliness has been associated with old age and conceptualised as an inevitable outcome of ‘failing’ health. This study seeks to conceptualise experiences of disability from a wider age group to understand if occurrences of social isolation and loneliness are commonplace. Fifteen qualitative biographical narrative interviews were completed by a Community Research Team, including seven males and eight females aged 32–89. A Disability Studies approach was applied to identify significant pathways from isolation into emotional loneliness, experienced by participants; fourteen of whom were affected by disability issues. Key risk factors were identified relating to disabled participants’ experiences of ‘loss’. Loss was associated with ‘loss of ability’, ‘loss due to bereavement’, ‘loss of social connectivity’ and ‘loss of self-confidence’. Participants connected life events concerning loss with spending time alone, leading to feelings of emotional loneliness. The findings illustrate key risk factors in being alone during evenings and weekends, periods where disabled participants were most likely to experience subjective feelings of emotional loneliness. (NOTE: The views expressed in this article are those of the authors not necessarily those of their employing agencies.)

In humans, growth hormone (GH) and cortisol are secreted in a pulsatile fashion and a mutual bidirectional interaction between the GH/insulin-like growth factor (IGF)-I axis and hypothalamic-pituitary-adrenal (HPA) axis has been established. Classic congenital adrenal hyperplasia (CAH) is characterized by a defect in the synthesis of glucocorticoids and often mineralocorticoids, and adrenal hyperandrogenism. In view of the sexually dimorphic pattern in GH secretion, we investigated the GH-cortisol bihormonal secretory dynamics in male and female children with classic CAH. Thirty-eight children with classic 21-hydroxylase deficiency (M: 13, F: 25; age range: 6.1-18.8 yr) were studied prospectively. Serum GH and cortisol concentrations were determined at 20 min intervals for 24 hours. The irregularity of GH and cortisol pattern was assessed using approximate entropy (ApEn), a scale- and model-independent statistic. The synchrony of joint GH-cortisol dynamics was quantified using the cross-ApEn statistic. Cross-correlation analysis of GH and cortisol concentrations was computed at various time lags covering the 24-h period. There was no gender difference in mean 24-hour serum GH (males vs females: 5.25 +/- 4.72 vs 4.44 +/- 2.64 mIU/l) or cortisol (156.2 +/- 44.6 vs 172.0 +/- 58.5 nmol/l) concentrations. For GH, ApEn values were significantly higher in females (0.66 +/- 0.14) than in males (0.53 +/- 0.16) (p = 0.009). No difference in cortisol ApEn values was noted between sexes (0.53 +/- 0.21 vs 0.54 +/- 0.12). Cross-ApEn values of paired GH-cortisol, with cortisol leading GH, were significantly higher in females (0.94 +/- 0.14) than in males (0.83 +/- 0.20) (p = 0.03). These findings suggest that females with classic 21-hydroxylase deficiency have a more irregular pattern of GH secretion and a more asynchronous joint GH and cortisol dynamics than their male counterparts.

Salt losing conditions can be challenging to manage well during infancy and childhood. Prolonged sodium depletion is known to be associated with growth failure and other problems in young animals 1. We describe a unique case of a child with both Salt-Wasting 21-Hydroxylase Deficiency (SW21OHD) and Cystic Fibrosis (CF).

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