Bone health is compromised in adult patients with childhood-onset autoimmune-polyendocrinopathy-candiadis-ectodermal dystrophy (APECED)

ObjectiveInitial benchmarking of childhood-onset systemic lupus erythematosus (cSLE) quality indicators revealed suboptimal performance across multiple centers. Our aim was to improve cardiovascular and bone health screenings at a tertiary treatment center for cSLE. This included annual measurements of vitamin D, lipid profiles, and bone mineral density through dual-energy x-ray absorptiometry (DXA).MethodsQuality improvement methodology was applied to design and implement a standardized previsit planning process to electronically entered and saved orders for needed screenings prior to a scheduled clinic visit. Process outcomes were measured using statistical process control charts. Univariate analyses were completed to assess patient-level factors.ResultsDuring the study, 123 patients with cSLE participated across 619 clinic visits. The percentage of patients with completed screenings improved from 54% to 92% for annual vitamin D, 55% to 84% for annual lipid profiles, and 57% to 78% for DXA, which was sustained for more than 1 year. Providers responded to a majority of abnormal results, and improvement in the average vitamin D level was observed over time. Higher levels of disease activity, damage, number of clinic visits, and screenings completed at baseline were observed in patients with all screenings completed at the end of the intervention.ConclusionImplementation of elements of the chronic illness care model for cSLE management improved performance of cardiovascular and bone health screenings, a step toward preventing longterm morbidity in cSLE. Our study also suggests that more patient interaction with the healthcare system may promote successful completion of health maintenance screenings.

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Fractures, Bone Mineral Density, and Final Height in Craniopharyngioma Patients with a Follow-up of 16 Years

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/clinem/dgz279 ◽

2020 ◽

Vol 105 (4) ◽

pp. e1397-e1407 ◽

Cited By ~ 1

Author(s):

Selveta S van Santen ◽

Daniel S Olsson ◽

Marry M van den Heuvel-Eibrink ◽

Mark Wijnen ◽

Casper Hammarstrand ◽

...

Keyword(s):

Bone Mineral Density ◽

Bone Mineral ◽

Bone Health ◽

Final Height ◽

Z Score ◽

Mineral Density ◽

Childhood Onset ◽

Cross Sectional ◽

T Score

Abstract Context Pituitary hormonal deficiencies in patients with craniopharyngioma may impair their bone health. Objective To investigate bone health in patients with craniopharyngioma. Design Retrospective cross-sectional study. Setting Dutch and Swedish referral centers. Patients Patients with craniopharyngioma (n = 177) with available data on bone health after a median follow-up of 16 years (range, 1-62) were included (106 [60%] Dutch, 93 [53%] male, 84 [48%] childhood-onset disease). Main outcome measures Fractures, dual X-ray absorptiometry-derived bone mineral density (BMD), and final height were evaluated. Low BMD was defined as T- or Z-score ≤-1 and very low BMD as ≤-2.5 or ≤-2.0, respectively. Results Fractures occurred in 31 patients (18%) and were more frequent in men than in women (26% vs. 8%, P = .002). Mean BMD was normal (Z-score total body 0.1 [range, -4.1 to 3.5]) but T- or Z-score ≤-1 occurred in 47 (50%) patients and T-score ≤-2.5 or Z-score ≤-2.0 in 22 (24%) patients. Men received less often treatment for low BMD than women (7% vs. 18%, P = .02). Female sex (OR 0.3, P = .004) and surgery (odds ratio [OR], 0.2; P = .01) were both independent protective factors for fractures, whereas antiepileptic medication was a risk factor (OR, 3.6; P = .03), whereas T-score ≤-2.5 or Z-score ≤-2.0 was not (OR, 2.1; P = .21). Mean final height was normal and did not differ between men and women, or adulthood and childhood-onset patients. Conclusions Men with craniopharyngioma are at higher risk than women for fractures. In patients with craniopharyngioma, a very low BMD (T-score ≤-2.5 or Z-score ≤-2.0) seems not to be a good predictor for fracture risk.

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