67 results match your criteria: "Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling[Affiliation]"

Article Synopsis
  • Hutchison-Gilford progeria syndrome (HGPS) is a genetic disorder caused by a mutation in the LMNA gene, leading to rapid aging and serious health issues, including bone density loss and a shorter life span.
  • In studies using Lmna progeria mice, researchers analyzed bone mineralization and found similarities in mineral content across various ages but noted a higher number of empty osteocyte lacunae, indicating bone deterioration.
  • The findings highlighted significant reductions in bone volume and abnormal growth plates in Lmna mice, suggesting that bone dysplasia occurs due to problems in bone formation despite normal turnover rates.
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We performed a retrospective nationwide register-based cohort study which included all in-hospital patients aged ≥ 50 with pelvic fracture (PF) between 2010 and 2018 in Austria. We identified patients who were hospitalized with sepsis within 180 days following a PF event. Aetiology of sepsis was divided by unspecified, gram positive, gram negative and other.

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The lacunocanalicular network (LCN) is an intricate arrangement of cavities (lacunae) and channels (canaliculi), which permeates the mineralized bone matrix. In its porosity, the LCN accommodates the cell network of osteocytes. These two nested networks are attributed a variety of essential functions including transport, signaling, and mechanosensitivity due to load-induced fluid flow through the LCN.

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Bone Quality and Mineralization and Effects of Treatment in Osteogenesis Imperfecta.

Calcif Tissue Int

December 2024

Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Med. Dept. Hanusch Hospital, Vienna, Austria.

Osteogenesis imperfecta (OI) is a rare congenital bone dysplasia characterized by high fracture rates and broad variations in clinical manifestations ranging from mild to increasingly severe and perinatal lethal forms. The underlying mutations affect either the synthesis or processing of the type I procollagen molecule itself or proteins that are involved in the formation and mineralization of the collagen matrix. Consequently, the collagen forming cells, the osteoblasts, become broadly dysfunctional in OI.

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Longitudinal course of circulating miRNAs in a patient with hypophosphatasia and asfotase alfa treatment: a case report.

JBMR Plus

October 2024

1st Medical Department, Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, Hanusch Hospital, 1140, Vienna, Austria.

Hypophosphatasia (HPP) is characterized by low activity of tissue nonspecific alkaline phosphatase (TNSALP). The enzyme replacement therapy asfotase alfa has been approved for childhood-onset forms of HPP. MicroRNAs (miRNAs) have emerged as a novel disease biomarker, with potential application in therapy monitoring.

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3D osteocyte lacunar morphometry of human bone biopsies with high resolution microCT: From monoclonal gammopathy to newly diagnosed multiple myeloma.

Bone

December 2024

Max Planck Institute of Colloids and Interfaces, Department of Biomaterials, 14476 Potsdam, Germany; Group of Bioengineering in Regeneration and Cancer, Biogipuzkoa Health Research Institute, 20014 San Sebastian, Spain; IKERBASQUE, Basque Foundation for Science, 48009 Bilbao, Spain. Electronic address:

Osteocytes are mechanosensitive, bone-embedded cells which are connected via dendrites in a lacuno-canalicular network and regulate bone resorption and formation balance. Alterations in osteocyte lacunar volume, shape and density have been identified in conditions of aging, osteoporosis and osteolytic bone metastasis, indicating patterns of impaired bone remodeling, osteolysis and disease progression. Osteolytic bone disease is a hallmark of the hematologic malignancy multiple myeloma (MM), in which monoclonal plasma cells in the bone marrow disrupt the bone homeostasis and induce excessive resorption at local and distant sites.

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Bone's resistance to fracture depends on its amount and quality, the latter including its structural and material/compositional properties. Bone material properties are dependent on bone turnover rates, which are significantly elevated immediately following menopause. Previously published data reported that following menopause, the amount of organic matrix synthesized at actively forming surfaces is significantly decreased, while glycosaminoglycan content was also modulated at resorbing surfaces, in the cancellous compartment.

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Nutritional Behavior of Patients with Bone Diseases: A Cross-Sectional Study from Austria.

Nutrients

June 2024

Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Medical Department Hanusch Hospital, 1140 Vienna, Austria.

Background: A balanced diet rich in calcium and protein is recommended for bone-healthy people and osteoporosis patients, but it may also be important for rare bone disease (RBD). Little data is available on RBD and diet. Therefore, the aim of this study was to evaluate the nutritional behavior of patients with RBD.

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Dislocation force of scleral flange-fixated intraocular lens haptics.

BMC Ophthalmol

March 2024

Vienna Institute for Research in Ocular Surgery (VIROS), A Karl Landsteiner Institute, Hanusch Hospital, Heinrich-Collin Str. 30, AT-1140, Vienna, Austria.

Purpose: To measure the dislocation forces in relation to haptic material, flange size and needle used.

Setting: Hanusch Hospital, Vienna, Austria.

Design: Laboratory Investigation.

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Combination of osteogenesis imperfecta and hypophosphatasia in three children with multiple fractures, low bone mass and severe osteomalacia, a challenge for therapeutic management.

Eur J Med Genet

November 2023

Department of Pediatric Endocrinology and Diabetology, Competence Center for Rare Diseases of the Calcium and Phosphate Metabolism, Angers University Hospital, Angers, France.

Osteogenesis imperfecta (OI) and hypophosphatasia (HPP) are rare skeletal disorders caused by mutations in the genes encoding collagen type I (COL1A, COL1A2) and tissue-non-specific isoenzyme of alkaline phosphatase (ALPL), respectively. Both conditions result in skeletal deformities and bone fragility although bone tissue abnormalities differ considerably. Children with OI have low bone mass and hypermineralized matrix, whereas HPP children develop rickets and osteomalacia.

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It is not well established to what extent previous immunizations offer protection against infections with the SARS-CoV-2 Omicron variant in dialysis patients. We aimed to define the relevant humoral response in dialysis patients using a SARS-CoV-2 IgG chemiluminescence microparticle immunoassay (CMIA) compared to the activity of neutralizing antibodies assessed by a virus neutralization test. Next, we aimed to determine differences in humoral and cellular response levels over time among patients infected or not infected by the Omicron variant of SARS-CoV-2.

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Vitamin C Deficiency Deteriorates Bone Microarchitecture and Mineralization in a Sex-Specific Manner in Adult Mice.

J Bone Miner Res

October 2023

Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Med. Dept. Hanusch Hospital, Vienna, Austria.

Article Synopsis
  • Vitamin C is crucial for bone health, with low levels linked to a higher risk of fractures.
  • Using advanced imaging techniques, the study compared bone structure and mineral density in mice with and without Vitamin C supplementation.
  • Results showed that Vitamin C deficiency significantly reduced bone thickness and volume, especially in female mice, indicating its important role in bone formation and mineralization.
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Structural and functional heterogeneity of mineralized fibrocartilage at the Achilles tendon-bone insertion.

Acta Biomater

August 2023

Mechanics of Biological and Bioinspired Materials Laboratory, Department of Aerospace and Mechanical Engineering, University of Liège, Liège, Belgium. Electronic address:

A demanding task of the musculoskeletal system is the attachment of tendon to bone at entheses. This region often presents a thin layer of fibrocartilage (FC), mineralized close to the bone and unmineralized close to the tendon. Mineralized FC deserves increased attention, owing to its crucial anchoring task and involvement in enthesis pathologies.

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Background: X-linked hypophosphatemia (OMIM 307800) is a rare bone disease caused by a phosphate-wasting condition with lifelong clinical consequences. Those affected suffer from bone pain, complex skeletal deformities, impaired mobility and a reduced quality of life. Early osteoarthritis and reduced range of motion of the lower limbs are known pathologies in XLH patients.

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Autoimmune polyendocrine syndrome type-1 (APS1) is characterized by autoimmune manifestations affecting different organs from early childhood on. Immunological abnormalities, the resulting endocrinopathies, and their treatments may compromise bone health. For the first time in APS1, we analyzed transiliac bone biopsy samples by bone histomorphometry and quantitative backscattered electron imaging in three adult patients (female P1, 38 years; male P2, 47 years; male P3, 25 years).

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Osteogenesis imperfecta (OI) is a genetically and clinically heterogeneous disorder characterized by bone fragility and reduced bone mass generally caused by defects in type I collagen structure or defects in proteins interacting with collagen processing. We identified a homozygous missense mutation in SEC16B in a child with vertebral fractures, leg bowing, short stature, muscular hypotonia, and bone densitometric and histomorphometric features in keeping with OI with distinct ultrastructural features. In line with the putative function of SEC16B as a regulator of trafficking between the ER and the Golgi complex, we showed that patient fibroblasts accumulated type I procollagen in the ER and exhibited a general trafficking defect at the level of the ER.

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Breaking the Gingival Barrier in Periodontitis.

Int J Mol Sci

February 2023

Clinic of Operative Dentistry, Periodontology and Preventive Dentistry, Saarland University, 66421 Homburg, Germany.

The break of the epithelial barrier of gingiva has been a subject of minor interest, albeit playing a key role in periodontal pathology, transitory bacteraemia, and subsequent systemic low-grade inflammation (LGI). The significance of mechanically induced bacterial translocation in gingiva (e.g.

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Significance Statement: Renal osteodystrophy (ROD) contributes substantially to morbidity in CKD, including increased fracture risk. Metabolic acidosis (MA) contributes to the development of ROD, but an up-to-date skeletal phenotype in CKD-associated acidosis has not been described. We comprehensively studied associations between acidosis and bone in patients with CKD using advanced methods to image the skeleton and analyze bone-tissue, along with biochemical testing.

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Osteocyte lacunae in transiliac bone biopsy samples across life span.

Acta Biomater

February 2023

Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Medical Department Hanusch Hospital, Vienna, Austria & Vienna Bone and Growth Center, Vienna, Austria.

Article Synopsis
  • Using quantitative backscattered electron imaging (qBEI), researchers analyzed osteocyte lacunae in bone samples from people under and over 30 years old, finding significant age-related differences in their density and porosity.
  • The study identified that older individuals have a 20% reduction in osteocyte characteristics and established reference data for diagnosing bone conditions, highlighting how mineral density declines with age.
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Identifying adult hypophosphatasia in the rheumatology unit.

Orphanet J Rare Dis

December 2022

Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1St Medical Department, Hanusch Hospital, Heinrich-Collin-Strasse 30, 1140, Vienna, Austria.

Background: The most frequent manifestation in adult hypophosphatasia (HPP) is musculoskeletal pain. The unspecific nature of its clinical presentation may prevent correct diagnosis. The aim of the study was to assess the prevalence of ALPL mutations in adult patients treated in rheumatological outpatient facilities with evident musculoskeletal symptoms typical for HPP.

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The spatial distribution of mineralization density is an important signature of bone growth and remodeling processes, and its alterations are often related to disease. The extracellular matrix of some vertebrate mineralized tissues is known to be perfused by a lacunocanalicular network (LCN), a fluid-filled unmineralized structure that harbors osteocytes and their fine processes and transports extracellular fluid and its constituents. The current report provides evidence for structural and compositional heterogeneity at an even smaller, subcanalicular scale.

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Hyperthyroidism causes secondary osteoporosis through favoring bone resorption over bone formation, leading to bone loss with elevated bone fragility. Osteocytes that reside within lacunae inside the mineralized bone matrix orchestrate the process of bone remodeling and can themselves actively resorb bone upon certain stimuli. Nevertheless, the interaction between thyroid hormones and osteocytes and the impact of hyperthyroidism on osteocyte cell function are still unknown.

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Accelerated mineralization kinetics in children with osteogenesis imperfecta type 1.

Bone

January 2023

Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Med. Dept. Hanusch Hospital, Vienna, Austria; Vienna Bone and Growth Center, Vienna, Austria.

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Article Synopsis
  • Vitamin C is crucial for the formation and maintenance of bone by influencing connective tissue integrity through its role in collagen maturation and epigenetic regulation.
  • It modulates chromatin accessibility, enhancing the expression of key genes for osteoblast development while affecting histone marks and DNA methylation.
  • Lack of Vitamin C disrupts bone development, and its epigenetic roles could be targeted for therapies against bone degeneration conditions.
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Cortical bone material / compositional properties in growing children and young adults aged 1.5-23 years, as a function of gender, age, metabolic activity, and growth spurt.

Bone

December 2022

Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Heinrich Collin Str. 30, A-1140 Vienna, Austria. Electronic address:

Bone material / compositional properties are significant determinants of bone quality, thus strength. Raman spectroscopic analysis provides information on the quantity and quality of all three bone tissue components (mineral, organic matrix, and tissue water). The overwhelming majority of the published reports on the subject concern adults.

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