Background: Spinal muscular atrophy (SMA) is a group of rare, inherited neuromuscular disorders. Bone health is often a neglected issue in children with SMA. This study aimed to evaluate the bone health status of children with SMA in Hong Kong. Methods: This retrospective study included children with SMA who were managed in the Neuromuscular Disorder Clinics of 2 quaternary centers in Hong Kong. Bone health status was assessed by fracture history, bone mineral density (BMD) measured by dual energy X-ray absorptiometry, and serum 25-hydroxy-vitamin D (25[OH]D) level. Results: Thirty-two children were included (males, 12). The median age was 10.8 years. BMD assessments were performed in 17 patients (SMA type 1=2, type 2=8, type 3=7). Low BMD was observed in 16 out of 17 patients. Four had a history of long bone fractures and were started on bisphosphonates. SMA types, age at last visit, sex, ambulation, and 25(OH)D level were not associated with fracture history or BMD Z-scores. Only one fulfilled the 2019 International Society for Clinical Densitometry (ISCD) pediatric definition of osteoporosis, with both low BMD and a history of clinically significant fracture. Conclusions Children with SMA on disease-modifying treatments commonly had Low BMD and a history of fractures, but osteoporosis was uncommon according to the 2019 ISCD pediatric definition. A special definition of osteoporosis may be needed for this high-risk group.

INTRODUCTION: This study aimed to assess safety, local tumour progression (LTP) and risk factors for LTP after radiofrequency ablation (RFA) of liver tumours in a single centre. METHODS: All consecutive patients treated with RFA for liver tumours between January 2009 and October 2012 were included. Previously treated lesions that progressed were excluded. Using electronic medical records, the following data was captured: patient demographics, pre-procedural laboratory results, Child-Pugh status, tumour characteristics, development of tumoral seeding, RFA complications and LTP. Possible risk factors for LTP were identified using Cox regression. RESULTS: In total, 555 liver tumours were treated in 337 patients. 483 (87.0%) hepatocellular carcinomas, 52 (9.4%) colorectal metastases and 20 (3.6%) other tumour types were treated. Mean tumour size was 2.1 ± 1.1 (range 0.4-6.8) cm. Mean follow-up duration was 387 days. 416 (75.0%) lesions had no LTP at the last imaging. 70 (12.6%) patients had minor complications requiring observation, while 7 (1.3%) patients had significant complications requiring prolonged hospitalisation or further interventions. Only one case of tumour seeding was detected. Using multivariate Cox regression, the following factors were statistically significant in predicting LTP: hilar location (relative ratio [RR] 3.988), colorectal metastases (RR 2.075), size (RR 1.290) and younger age (RR 0.982). CONCLUSION RFA of liver tumours is safe and effective, with a low significant complication rate of 1.3%. Hilar lesions are most prone to LTP, followed by lesions that were larger in size and colorectal metastases. 75.0% of patients showed no LTP at the last follow-up.