Manju Chandran

Summary: Erdheim–Chester disease (ECD) is a potentially fatal condition characterized by infiltration of multiple organs by non-Langerhans histiocytes. Although endocrine dysfunction has been reported in association with ECD, to date, there have been no previous reports of empty sella syndrome (ESS) associated with it. We report the case of a patient with ECD who had symptomatic ESS. A 55-year-old man of Chinese ethnicity initially presented with symptoms of heart failure, fatigue and knee joint pain. Physical examination revealed xanthelasma, gynaecomastia, lung crepitations, hepatomegaly and diminished testicular volumes. He had laboratory evidence of hypogonadotrophic hypogonadism, secondary hypoadrenalism and GH deficiency. Imaging studies showed diffuse osteosclerosis of the long bones on X-ray, a mass in the right atrium and thickening of the pleura and of the thoracic aorta on fusion positron emission tomography–computed tomography. Magnetic resonance imaging (MRI) of the brain showed an empty sella. The diagnosis of ECD was confirmed by bone biopsy.Learning points: ECD is a multisystemic disease that can affect the pituitary and other organs. The diagnosis of ECD is based on clinical and radiological features and histology, showing lipid-laden CD68⁺CD1a⁻S100⁻histiocytes surrounded by fibrosis. The finding of xanthelasmas especially in the presence of normal lipid levels in the presence of a multisystem infiltrative disorder should raise the suspicion of ECD. Systemic perturbation of autoimmunity may play a role in the pathogenesis of ECD and is an area that merits further research.

It is well known that one fragility fracture begets another. Fracture Liaison Services have been shown to narrow the care gap that exists in the care of patients with fragility fractures. A secondary fracture prevention programme "OPTIMAL" (Osteoporosis Patient Targeted and Integrated Management for Active Living) has been in existence in the public restructured hospitals and polyclinics of Singapore since 2008 and this is beginning to show significant beneficial results in terms of identification and management of fragility fractures. However, significant obstacles in the path of appropriate management of the patient with a fragility fracture still exist. A concerted, multipronged and interdisciplinary approach is needed to overcome these barriers.

A fragility fracture signals the potential for the occurrence of a subsequent fracture, and it often represents the first opportunity for care of osteoporosis. It is now well accepted knowledge that fracture liaison services can close or, at the very least, narrow the care gap that exists in the management of these sentinel events in patients with osteoporosis. The challenge, however, lies in translating this knowledge into practice and to implement these programs in health care systems with varied infrastructure and delivery practices operating in varied geographical environments. A review of a functioning model of care in an open health system in an Asian country and a short account of the problems encountered and the lessons learned during the course of its implementation are provided.

Summary A significant care gap exists in the management of osteoporotic fractures. Osteoporosis Patient Targeted and Integrated Management for Active Living (OPTIMAL) is a secondary fracture prevention program instituted in the public hospitals of Singapore. We aim to describe the operational characteristics of OPTIMAL and initial audit data of patients who were recruited into the program at Singapore General Hospital. Introduction Fractures often represent the first opportunity for care of osteoporosis. However, a significant care gap still exists in the management of these sentinel events and underdiagnosis and undertreatment of osteoporotic fractures are prevalent worldwide. Fracture liaison services run by care coordinators have been shown to reduce the fracture care gap. OPTIMAL is a clinician champion-driven, case manager-run secondary fracture prevention program set up in the public hospitals of Singapore in 2008. Methods We present the operational characteristics and initial audit data of OPTIMAL from the largest tertiary teaching hospital in Singapore. Results One thousand and fourteen patients have been recruited into OPTIMAL at our hospital since 2008, and 476 patients are currently in active follow-up. Two hundred and eighty-seven patients had completed a 2-year follow-up at the hospital as of August 2012 and were evaluated; 97.5 % of these patients had DXA evaluation upon enrollment into the program, and 62 % of the patients reported compliance with an exercise program over the 2-year follow-up. Compliance to osteoporosis medications as estimated by the medication possession ratio (MPR) was 72.8 ± 34.5 % at 2 years with patients maintaining good compliance (MPR ≥ 80 %) for an average of 20.2 months (95 % CI 19.3–21.1). Conclusion Our report provides the first compelling evidence of the potential success of a secondary fracture prevention program from an Asian country. The ultimate success of the program will be determined by fracture outcomes and cost effectiveness, but in the interim, clear evidence of enhanced assessment and treatment rates has been demonstrated.

Objective. Limited information exists on the physical characteristics, bone mineral density (BMD), 25-hydroxyvitamin D [25(OH)D] levels and other biochemical parameters in patients with hip fractures in Asia. We aimed to explore whether these parameters differ between trochanteric and femoral neck (cervical) hip fractures in Singaporean patients. Methodology. 275 patients with osteoporotic hip fractures were evaluated. Multivariate regression models were used to investigate the relationship between physical characteristics, biochemical parameters, renal function, BMD and type of hip fracture. Results. The mean 25(OH)D level noted was 20.4 (8.1) ng/mL. Fifty percent of patients had levels less than 20 ng/mL. Patients with trochanteric fractures were heavier than those with cervical fractures ( p =0.021). Logistic regression analysis showed that males were more likely to have trochanteric fractures (OR=1.79, 95% CI 1.03-3.11), and females with trochanteric fractures were older than those with cervical fractures (OR=0.97, 95% CI 0.94-1.00). Conclusion. Vitamin D deficiency was prevalent in half of our population of hip fracture patients. Trochanteric fractures were more common than cervical fractures in men and in older women. In contrast to findings noted in Caucasian populations, neither BMD nor other bone metabolic parameters examined were associated with the preferential occurrence of one type of osteoporotic hip fracture over the other.

Osteoporosis is a major public health problem worldwide and it is of particular significance in a rapidly aging society such as Singapore. In order to prevent the devastating sequelae of osteoporosis, it is important, to diagnose the condition early, to identify its associated risk factors and to initiate pharmacotherapy promptly when required. In addition, it is important to increase awareness of this condition amongst both health care professionals and the public. The Singapore Ministry of Health (MOH) Clinical Practice Guidelines published in 2008 was an update of a previously published version in 2002. It aimed at providing the best evidence-based recommendations for diagnosis, classification, evaluation and multidisciplinary management of osteoporosis and osteoporotic fractures in Singapore. This article summarizes the guidelines, discusses their applicability and utilization in Singapore and briefly outlines updates in management since the publication of the CPG.