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Journal of Cellular Biochemistry Mar 2006Prostate cancer (CaP) is unique among all cancers in that when it metastasizes to bone, it typically forms osteoblastic lesions (characterized by increased bone... (Review)
Review
Prostate cancer (CaP) is unique among all cancers in that when it metastasizes to bone, it typically forms osteoblastic lesions (characterized by increased bone production). CaP cells produce many factors, including Wnts that are implicated in tumor-induced osteoblastic activity. In this prospectus, we describe our research on Wnt and the CaP bone phenotype. Wnts are cysteine-rich glycoproteins that mediate bone development in the embryo and promote bone production in the adult. Wnts have been shown to have autocrine tumor effects, such as enhancing proliferation and protecting against apoptosis. In addition, we have recently identified that CaP-produced Wnts act in a paracrine fashion to induce osteoblastic activity in CaP bone metastases. In addition to Wnts, CaP cells express the soluble Wnt inhibitor dickkopf-1 (DKK-1). It appears that DKK-1 production occurs early in the development of skeletal metastases, which results in masking of osteogenic Wnts, thus favoring osteolysis at the metastatic site. As metastases progress, DKK-1 expression decreases allowing for unmasking of Wnt's osteoblastic activity and ultimately resulting in osteosclerosis at the metastatic site. We believe that DKK-1 is one of the switches that transitions the CaP bone metastasis activity from osteolytic to osteoblastic. Wnt/DKK-1 activity fits a model of CaP-induced bone remodeling occurring in a continuum composed of an osteolytic phase, mediated by receptor activator of NFkB ligand (RANKL), parathyroid hormone-related protein (PTHRP) and DKK-1; a transitional phase, where environmental alterations promote expression of osteoblastic factors (Wnts) and decreases osteolytic factors (i.e., DKK-1); and an osteoblastic phase, in which tumor growth-associated hypoxia results in production of vascular endothelial growth factor and endothelin-1, which have osteoblastic activity. This model suggests that targeting both osteolytic activity and osteoblastic activity will provide efficacy for therapy of CaP bone metastases.
Topics: Adult; Bone Neoplasms; Carcinoma; Humans; Intercellular Signaling Peptides and Proteins; Male; Models, Biological; Osteogenesis; Osteolysis; Osteosclerosis; Prostatic Neoplasms; Signal Transduction; Wnt Proteins
PubMed: 16447163
DOI: 10.1002/jcb.20735 -
Annals of the Rheumatic Diseases Feb 1975Osteosclerosis of the terminal finger phalanges was assessed by means of a grading score in two age-matched groups of 96 females, each suffering from rheumatoid...
Osteosclerosis of the terminal finger phalanges was assessed by means of a grading score in two age-matched groups of 96 females, each suffering from rheumatoid arthritis (RA) and osteoarthrosis (OA), respectively, and in a sample of 894 males and females, above the age of 44 years, from Dutch small town populations. Cortical thickness of the therminal phalanges decreases markedly with age. Whereas in the age group 45-54 years, terminal phalangeal osteosclerosis is more pronounced in females, at higher ages the difference between the sexes disappears. The diameter of the terminal cortex increases gradually from the second to the fifth finger. No differences were found between the RA and the OA groups, nor were these groups different from the population survey except for a lower incidence of terminal phalangeal osteosclerosis in female osteoarthrotics between 45 and 55 years of age, compared to females of the same age group from the population sample.
Topics: Age Factors; Aged; Arthritis, Rheumatoid; Female; Fingers; Hand; Humans; Male; Middle Aged; Netherlands; Osteosclerosis; Radiography; Sex Factors
PubMed: 1124958
DOI: 10.1136/ard.34.1.82 -
Neurotherapeutics : the Journal of the... Oct 2008This review focuses on recent data regarding inflammatory demyelinating neuropathies and neuropathies associated with monoclonal gammopathies. We describe both acute and... (Review)
Review
This review focuses on recent data regarding inflammatory demyelinating neuropathies and neuropathies associated with monoclonal gammopathies. We describe both acute and chronic inflammatory neuropathies, and we discuss conditions ranging from mostly cell-mediated to antibody-mediated disorders. These diseases are characterized by proximal and distal sensory motor involvement. Treatments are based on immune-modulation and/or immune-suppression. Work-up sequence and therapeutical modes are discussed in the light of recently published data, with a special interest on new treatment modalities.
Topics: Animals; Cryoglobulinemia; Demyelinating Diseases; Glycoproteins; Guillain-Barre Syndrome; Hereditary Sensory and Motor Neuropathy; Humans; Multiple Myeloma; Myelin Sheath; Osteosclerosis; POEMS Syndrome; Paraproteinemias; Polyneuropathies
PubMed: 19019303
DOI: 10.1016/j.nurt.2008.08.012 -
Proceedings of the Royal Society of... Apr 1950
Topics: Humans; Melorheostosis
PubMed: 15417575
DOI: No ID Found -
Folia Morphologica 2018The aim of this study was to analyse the prevalence and morpho-metric parameters of idiopathic osteosclerosis (IO) in a Chilean population. IO is an intraosseous growth...
BACKGROUND
The aim of this study was to analyse the prevalence and morpho-metric parameters of idiopathic osteosclerosis (IO) in a Chilean population. IO is an intraosseous growth of compact, benign, unilocular, non-expandable bone that is referred to as an anatomical variation.
MATERIALS AND METHODS
A cross-sectional study was performed using 1000 digital panoramic radiographs of adults in which data on the location (maxillary/ mandibular, right/left hemiarcades), shape, position to the dental apex, and the prevalence of IOs were observed in relation to gender and age. The morphometric parameters evaluated were area, height, width, and the linear distances of the IO up to the midline and at the base of the mandible.
RESULTS
The overall prevalence was 2.8% (27 individuals), with the majority of cases in women (66.7%) in the second, third, and fifth decades of life, but without significant differences. All cases were present in the mandible (100%), usually in the left hemiarcade (59.3%), molar (48.2%) and premolar (44.4%) regions; at the height of the dental apices (65.5%), with an irregular shape (40.7%) and round (37%). The area of the IOs was 33.9 ± 20.1 mm2, with a height of 7.7 ± ± 3.1 mm, width of 6.6 ± 3.1 mm, and the distance from the IO to the mandible median line was 26.6 ± 10.7 mm and 9.7 ± 3.7 mm to the mandibular base.
CONCLUSIONS
All the data observed corroborate with previous studies; the IO does not present a large difference in the Chilean population evaluated compared to pre-vious studies carried out in other populations. (Folia Morphol 2018; 77, 2: 272-278).
Topics: Adolescent; Adult; Chile; Cross-Sectional Studies; Female; Humans; Male; Mandible; Middle Aged; Molar; Osteosclerosis; Prevalence
PubMed: 28933800
DOI: 10.5603/FM.a2017.0085 -
Acta Reumatologica Portuguesa 2020
Topics: Humans; Male; Melorheostosis; Middle Aged; Trigger Finger Disorder
PubMed: 32578577
DOI: No ID Found -
Medical Principles and Practice :... 2022The purpose of the study was to create an artificial intelligence (AI) system for detecting idiopathic osteosclerosis (IO) on panoramic radiographs for automatic,...
OBJECTIVE
The purpose of the study was to create an artificial intelligence (AI) system for detecting idiopathic osteosclerosis (IO) on panoramic radiographs for automatic, routine, and simple evaluations.
SUBJECT AND METHODS
In this study, a deep learning method was carried out with panoramic radiographs obtained from healthy patients. A total of 493 anonymized panoramic radiographs were used to develop the AI system (CranioCatch, Eskisehir, Turkey) for the detection of IOs. The panoramic radiographs were acquired from the radiology archives of the Department of Oral and Maxillofacial Radiology, Faculty of Dentistry, Eskisehir Osmangazi University. GoogLeNet Inception v2 model implemented with TensorFlow library was used for the detection of IOs. Confusion matrix was used to predict model achievements.
RESULTS
Fifty IOs were detected accurately by the AI model from the 52 test images which had 57 IOs. The sensitivity, precision, and F-measure values were 0.88, 0.83, and 0.86, respectively.
CONCLUSION
Deep learning-based AI algorithm has the potential to detect IOs accurately on panoramic radiographs. AI systems may reduce the workload of dentists in terms of diagnostic efforts.
Topics: Humans; Artificial Intelligence; Deep Learning; Radiography, Panoramic; Algorithms; Osteosclerosis
PubMed: 36167054
DOI: 10.1159/000527145 -
Orphanet Journal of Rare Diseases Apr 2020Raine syndrome (RS) is a rare autosomal recessive disorder caused by biallelic loss-of-function mutations of FAM20C. The most common clinical features are microcephaly,...
BACKGROUND
Raine syndrome (RS) is a rare autosomal recessive disorder caused by biallelic loss-of-function mutations of FAM20C. The most common clinical features are microcephaly, exophthalmos, hypoplastic nose and severe midface hypoplasia, leading to choanal atresia. The radiological findings include generalized osteosclerosis and brain calcifications. RS is usually lethal during the neonatal period due to severe respiratory distress. However, there exists a non-lethal RS form, the phenotype of which is extremely heterogeneous. There is paucity of data about clinical course and life expectancy of these patients.
RESULTS
This is the first description of follow-up features of non-lethal RS patients. Moreover, we present three unpublished cases. There are five Asian and two Arab patients. All were born to consanguineous parents. The most common neonatal comorbidity was respiratory distress secondary to choanal atresia. A variable degree of neurodevelopmental delay was seen in the majority of our cases and seizures and hearing or vision involvement were also frequent. Neurological and orthopedic issues were the most frequent complications seen at follow-up in our group. Persistent hypophosphatemic rickets was the most striking endocrinological manifestation, which was scarcely responsive to therapy with phosphate salts and alfacalcidol. Life expectancy of our patients goes beyond childhood, with the oldest of those described being 18 years old at present.
CONCLUSIONS
Manifestations of RS in those surviving the neonatal period are being increasingly recognized. Our study supports previous findings and provides clinical and biochemical observations and data from longer follow up. Finally, we propose multidisciplinary follow up for patients with non-lethal RS.
Topics: Abnormalities, Multiple; Adolescent; Casein Kinase I; Cleft Palate; Exophthalmos; Extracellular Matrix Proteins; Humans; Infant, Newborn; Microcephaly; Osteosclerosis
PubMed: 32299476
DOI: 10.1186/s13023-020-01373-0 -
Leukemia Research Jul 2010Evaluation of bone marrow fibrosis and osteosclerosis in myeloproliferative neoplasms (MPN) is subject to interobserver inconsistency. Performance data for currently... (Comparative Study)
Comparative Study
Evaluation of bone marrow fibrosis and osteosclerosis in myeloproliferative neoplasms (MPN) is subject to interobserver inconsistency. Performance data for currently utilized fibrosis grading systems are lacking, and classification scales for osteosclerosis do not exist. Digital imaging can serve as a quantification method for fibrosis and osteosclerosis. We used digital imaging techniques for trabecular area assessment and reticulin fiber quantification. Patients with all Philadelphia negative MPN subtypes had higher trabecular volume than controls (p
osteosclerosis helps differentiate primary myelofibrosis from other MPN. Numerical quantification of fibrosis highly correlated with subjective scores, and interobserver correlation was satisfactory. Digital imaging provides accurate quantification for osteosclerosis and fibrosis. Topics: Adult; Aged; Biopsy; Bone Marrow Examination; DNA Mutational Analysis; Female; Fibrosis; Humans; Image Interpretation, Computer-Assisted; Janus Kinase 2; Male; Microscopy; Middle Aged; Myeloproliferative Disorders; Observer Variation; Osteosclerosis; Receptors, Thrombopoietin; Reproducibility of Results; Reticulin; Retrospective Studies; Severity of Illness Index
PubMed: 20122729
DOI: 10.1016/j.leukres.2010.01.005 -
Postgraduate Medical Journal Aug 1977The osteopetroses are a group of conditions which are characterized by varying combinations of bony sclerosis and modelling defects. Classical osteopetrosis may be... (Review)
Review
The osteopetroses are a group of conditions which are characterized by varying combinations of bony sclerosis and modelling defects. Classical osteopetrosis may be inherited as an autosomal dominant or autosomal recessive: the former variety is benign, heterogeneous and comparatively common, while the latter is precocious, potentially lethal and rare. Many other craniotubular dysplasias and hyperostoses are loosely grouped with the osteopetroses. The commonest of these is the autosomal dominant form of craniometaphyseal dysplasia, while the others which are well known include Pyle disease, and van Buchem disease. Sclerosteosis is a progressive condition in which massive cranial thickening is associated with syndactyly and gigantism. Each of these disorders has specific clinical and radiographic features, which permit recognition. Diagnostic accuracy is crucial for treatment, prognostication and effective genetic management.
Topics: Chromosome Aberrations; Chromosome Disorders; Humans; Hyperostosis, Cortical, Congenital; Osteopetrosis; Radiography; Terminology as Topic
PubMed: 335376
DOI: 10.1136/pgmj.53.622.507