Hospitalisation of adults with Down syndrome: lesson from a 10-year experience from a community hospital.

BACKGROUND: Life expectancy of individuals with Down syndrome (DS) has improved significantly over the past decades. However, there are sparse data documenting the co-morbidities and hospitalisation of adult patients with DS in the literature. The aim of this study was to characterise the co-morbidities and pattern of hospitalisation in adult patients with DS during a 10-year period at the community hospital as well as to compare hospitalisation parameters with the general adult population during the same years. METHOD: We reviewed the medical records of 81 hospitalisations from 37 patients with DS aged 21 to 68 years at Metrowest Medical Center during a 10-year period and compared with those of the general adult population during the same time. Co-morbidities were also described. RESULTS: Adults with DS had a mean age at admission of 48.6 ± 8.8 years with the median length of stay of 3 days (interquartile range 4 days). Male patients were hospitalised longer than female patients (mean 5.0 vs. 2.8 days; P < 0.05), and patients who lived at home were admitted at earlier ages than those who came from residential healthcare facility (mean 41.5 vs. 52.2 years; P < 0.001). The most common cause of hospitalisation was pneumonia/aspiration syndrome (29.6%), and the most common co-morbidity was gastroesophageal reflux disease (GERD)/dysphagia (70.3%). Presence of GERD/dysphagia or seizure disorder was significantly associated with multiple admission and readmission within 1 month (P < 0.05). The mortality rate was 4.9%, and the rate of intensive care unit admission was 8.6%. CONCLUSIONS: Our cohort did not show statistically significant different hospitalisation parameters such as inpatient mortality and average length of stay when compared with general adult population hospitalised at the same years. The care of adult patients with DS presents challenges in internal medicine due to its unique co-morbid profile and signifies the importance of multidisciplinary approach. In order to improve the care of this patient population, their co-morbidities, particularly GERD/dysphagia and seizure disorder, should be optimally managed and comprehensively addressed when patients are hospitalised.

Hypocalcemia and bone mineral density changes following denosumab treatment in end-stage renal disease patients: a meta-analysis of observational studies.

The incidence of hypocalcemia and bone mineral density (BMD) changes in end-stage renal disease (ESRD) patients on denosumab remains unclear. We performed this meta-analysis to assess the incidence of denosumab-associated hypocalcemia and effects of denosumab on BMD in ESRD patients. A literature search was conducted using MEDLINE, EMBASE, and Cochrane Database from inception through November 2017 to identify studies evaluating incidence of denosumab-associated hypocalcemia and changes in serum calcium, phosphate, alkaline phosphatase (ALP), parathyroid hormone (PTH), and BMD from baseline to post-treatment course of denosumab in ESRD patients. Study results were pooled and analyzed using a random-effect model. The protocol for this meta-analysis is registered with PROSPERO (International Prospective Register of Systematic Reviews; no. CRD42017081074). Six observational studies with a total of 84 ESRD patients were enrolled. The pooled estimated incidence of hypocalcemia during denosumab treatment was 42% (95% CI 29-55%, I2 = 0%). Hypocalcemia occurred approximately 7 to 20 days after the first dose and reached nadir of low calcium levels in the first 2 weeks up to 2 months. However, there were no significant changes in serum calcium or phosphate from baseline to post-treatment course (≥ 3 months after treatment) with mean differences [MDs] of 0.20 mg/dL (95% CI, - 0.30 to 0.69 mg/dL) and - 0.10 mg/dL (95% CI, - 0.70 to 0.49 mg/dL). There were significant reductions in ALP and PTH levels with standardized mean differences (SMDs) of - 0.65 (95% CI - 1.13 to - 0.16) and - 1.89 (95% CI - 3.44 to - 0.34), respectively. There were significant increases in T-scores with MDs of 0.39 (95% CI 0.10 to 0.69) and 0.79 (95% CI 0.60 to 0.98) for lumbar spine and femoral neck, respectively. Our study demonstrates the estimated incidence of denosumab-associated hypocalcemia in dialysis patients of 42%. From baseline to post-treatment course, although there are no differences in serum calcium and phosphate, our findings suggest significant reductions in ALP and PTH and a significant increase in BMD. Currently, denosumab should not be considered as the treatment of choice in ESRD patients until more safety and efficacy data are available.

Osteoporosis and bone mineral density in patients with Wilson's disease: a systematic review and meta-analysis.

This systematic review aims to assess the occurrence and risks of osteopenia and osteoporosis in patientswith Wilson's disease (WD). A literature search was conducted utilizing EMBASE and MEDLINE frominception through April 2017. Studies assessing the occurrence or risk of osteopenia and/or osteoporosis inWD patients were included. Effect estimates from the individual study were extracted and combined usingrandom-effect, generic inverse variance method of DerSimonian and Laird. Of 754 studies, four studies with283 WD patients met the eligibility criteria and were included in the data analysis. The pooled prevalencerates of osteopenia and osteoporosis in WD patients were 36.5% (95% confidence interval [CI]: 14.8%-65.7%) and 27.7% (95%CI: 8.6%-60.9%), respectively. When meta-analysis was limited only to adults, the estimated prevalence rates of osteopenia, osteoporosis, and vertebral fracture were 50.0% (95%CI: 42.0%-58.0%), 17.6% (95%CI: 6.7%-38.6%) and 8.01% (95%CI: 4.05%-15.2%), respectively. Meta-regressionshowed significant impacts of age (negative correlation; P=0.002) and male status (positive correlation;P < 0.001) on the prevalence of osteoporosis. The data on risks of osteopenia and osteoporosis in WDpatients were limited. We suggests that there are potential associations of WD with osteopenia and/orosteoporosis. Also, young age and male status are correlated with the higher prevalence of osteoporosis inWD patients.