Purpose: e use of morning basal serum cortisol levels as an alternative to dynamic testing for assessment of hypothalamic-pituitary-adrenal (HPA) axis has previously been reported. e purpose of this study was to determine the lower and upper cuto values that would obviate subsequent HPA axis testing.Methods: A single-centre, retrospective study from a tertiary care endocrinology clinic was conducted, analyzing data from 106 adult individuals referred for HPA axis testing who had undergone a 0800-0900 morning basal serum cortisol test followed by a standard dose (250 μg) adrenocorticotropin (ACTH) stimulation test.e ability of morning basal serum cortisol values to predict post-ACTH 30 or 60 minute peak cortisol value of >500 or >550 nmol/L was investigated.Results: A morning basal cuto of <128 nmol/L is su cient for predicting a post-ACTH value < 550 nmol/L, and morning basal cuto levels of >243 nmol/L and >266 nmol/L predict peak post-ACTH values of >500 and >550 nmol/L respectively, obviating the need for dynamic testing. Regression analysis further demonstrated the log-linear relationship between morning basal and peak levels, while also nding a signi cant decrease in peak post-ACTH levels for patients diagnosed with secondary hypothyroidism (76 nmol/ L lower, p=0.003) or secondary hypogonadism (61 nmol/L lower, p=0.02). ese data suggest that the risk of cortisol de ciency is signi cantly higher in individuals with additional pituitary insu ciencies. e odds ratios for cortisol de ciency in patients with history of isolated secondary hypothyroidism was 3.41 (p=0.015), with isolated secondary hypogonadism was 4.77 (p=0.002) and with both was 7.45 (p=0.0002). Conclusion:Morning basal serum cortisol levels show promise as an e ective screening test for HPA insu ciency for most patients. Clinicians should consider the high probability of HPA insu ciency in patients with one or more pituitary insu ciencies.
AS is responsible for significant morbidity in children, including susceptibility to adrenal crisis. The minimal estimated incidence reported is for the entire paediatric population and would be much higher in the at-risk group (ie, children treated with GCs). Close monitoring of growth and possible symptoms of AS, which may be non-specific, are important in children on all forms of GC therapy including ICS. To reduce the risk of AS, physicians must be aware of the risk of AS, revisit GC doses frequently and use the lowest effective dose.
Alstrom syndrome is characterized by childhood obesity, progressive retinal degeneration, and sensorineural hearing loss with diabetes mellitus (DM) developing later in childhood and adulthood. The course of diabetes in children with this condition has not been described. We aim to describe the diagnosis, management, and course of diabetes in a series of children followed in our center. A retrospective chart review of all seven children with Alstrom syndrome was performed. Patients, aged 4.5-22 yr, had typical features of Alstrom syndrome. Five were diagnosed with DM at a median age of 11.5 yr. At diagnosis of DM, mean fasting blood glucose (FBG) was normal at 82.8 ± 12.6 mg/dL (4.6 ± 0.7 mmol/L), but random or oral glucose tolerance test (OGTT) values were > 200 mg/dL (11.1 mmol/L). Two patients had periods of poor control despite high-dose insulin and show better A1C, off insulin and with other therapy. In our series, DM in Alstrom syndrome begins by age 14 yr. At diagnosis of DM, FBG was normal. Hence the diagnosis may be missed if screening is performed with FBG alone. We conclude that OGTT should be considered annually from age 6 to 7 yr and in established DM, if glycemic control is poor on insulin, escalating doses may not be effective.
BACKGROUND AND OBJECTIVES Referrals of transgender and gender-diverse (trans) youth to medical clinics for gender-affirming care have increased. We described characteristics of trans youth in Canada at first referral visit. METHODS Baseline clinical and survey data (2017–2019) were collected for Trans Youth CAN!, a 10-clinic prospective cohort of n = 174 pubertal and postpubertal youth <16 years with gender dysphoria, referred for hormonal suppression or hormone therapy, and 160 linked parent-participants. Measures assessed health, demographics, and visit outcome. RESULTS Of youth, 137 were transmasculine (assigned female) and 37 transfeminine (assigned male); 69.0% were aged 14 to 15, 18.8% Indigenous, 6.6% visible minorities, 25.7% from immigrant families, and 27.1% low income. Most (66.0%) were gender-aware before age 12. Only 58.1% of transfeminine youth lived in their gender full-time versus 90.1% of transmasculine (P < .001). Although transmasculine youth were more likely than transfeminine youth to report depressive symptoms (21.2% vs 10.8%; P = .03) and anxiety (66.1% vs 33.3%; P < .001), suicidality was similarly high overall (past-year ideation: 34.5%, attempts: 16.8%). All were in school; 62.0% reported strong parental gender support, with parents the most common support persons (91.9%). Two-thirds of families reported external gender-related stressors. Youth had met with a range of providers (68.5% with a family physician). At clinic visit, 62.4% were prescribed hormonal suppression or hormone therapy, most commonly depot leuprolide acetate. CONCLUSIONS Trans youth in Canada attending clinics for hormonal suppression or gender-affirming hormones were generally healthy but with depression, anxiety, and support needs.
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