Bone Abstracts

The development of optimally performing biomaterials mimicking the natural physiological processes underlying bone repair is essential for non-healing large bone lesions. We sought to develop scaffolds that fill the lesions and improve bone healing. We tested three scaffolds comprised of varying percentages of lactide (LA), caprolactone (CL), and methacrylate produced by two-photon photopolymerization; LCM 3 (8:2; 85%), 4 (9:1; 90%), and 6 (9:1; 40%). We hypothesized that the ...

Non-healing fractures caused by trauma, disease or tumour resection demand the use of bone grafts to support and stimulate the healing process. We sought to develop and evaluate the effect of a novel bioactive biodegradable biomaterial designed to fill large bone lesions and to improve bone healing. We used two-photon polymerised synthetic polymer scaffolds composed of lactide (LA), caprolactone (CL), and methacrylate (MA) with varying LA: CL ratio and percentage of methacryla...

Objective: The aim of this study is to compare the bone health index (BHI) for healthy Swiss children born in 1955 with healthy Swiss children born a generation later.Method: BHI is derived from the cortical thickness in the three middle metacarpals. It is determined with the BoneXpert medical device, which automatically analyses a standard bone age hand radiograph. The measurement result is independent of the sharpness of the image. The image data are f...

Background: Hypophosphatasia (HPP) is a heterogeneous rare, inherited disorder of bone and mineral metabolism caused by different mutations in the ALPL gene encoding the isoenzyme, tissue-nonspecific alkaline phosphatase (TNAP). Prognosis is very poor in severe perinatal forms with most patients dying from pulmonary complications of their skeletal disease. TNAP, a ubiquitous enzyme, is mostly known for its role in bone mineralization. TNAP deficiency, however, may als...

Objectives: Hypophosphatasia (HPP) is a rare metabolic disease caused by loss-of-function mutation(s) in the gene encoding tissue-nonspecific alkaline phosphatase (TNSALP). HPP in infants is characterized by poor skeletal mineralization, respiratory compromise, and a high risk of mortality. We previously reported improved mineralization and respiratory function in 15 patients enrolled in this second study of asfotase alfa, a bone-targeted recombinant human TNSALP, in infants a...

Silk fibroin (SF) sponges are a promising scaffold material for tissue engineering due to their biocompatibility, mechanical properties, and ability to support calcium-phosphate formation in vitro. However, previous studies have shown that SF can mineralize spontaneously in the presence of culture media, which has a detrimental effect on experimental integrity when analyzing how cells deposit bone-like tissue in tissue engineering studies. In this study we analyzed th...

Mutations of the ALPL-gene are closely related to hypophosphatasia (HPP), an inherited disorder of bone and mineral metabolism with clinically heterogeneous symptoms. To date 278 different mutations have been described, leading to reduction or completely loss of enzymatic activity of the tissue nonspecific alkaline phosphatase (TNAP).We present the case of a 6-year-old boy with clinical features and laboratory results consistent with infantile H...

Hypophosphatasia (HPP) is a rare disease characterized by low enzymatic activity of tissue-nonspecific alkaline phosphatase (TNSALP) resulting in an accumulation of its endogenous substrates like pyridoxal phosphate (PLP) and inorganic pyrophosphate (PPi). The ectoenzyme plays an important role during bone mineralization and might contribute to proper function of kidney and muscle. Neurological symptoms of HPP like seizures, anxiety disorders and depression provide ...

Objectives: Hypophosphatasia (HPP) is a rare, inherited metabolic disorder caused by loss-of-function mutations in the ALPL gene that encodes the tissue-nonspecific alkaline phosphatase TNAP (ORPHA 436). Its clinical presentation is highly heterogeneous with a remarkably wide-ranging severity. HPP affects patients of all age. Therefor diagnosis is often difficult and delayed. To improve the understanding of HPP in children and in order to shorten the diagnostic time span in th...

Objectives: Chronic non-bacterial osteomyelitis (CNO) is an inflammatory, non-bacterial disorder of the skeletal system of yet unknown etiology (ORPHA 324964). CNO predominantly affects the metaphyses of long bones, but lesions can occur at any sites of the skeleton. Patients present with local bone pain and inflammation and - to our experience - often suffer from functional impairment with significant disabilities of daily life. The objective of this study was to assess physi...