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Anti-sclerostin - is there an indication?

Several decades ago, a clinical condition that included severe bone overgrowth was described in a few patients in South Africa. The autosomal-recessive disease that later was named sclerosteosis was found to be caused by a mutation in the SOTS gene causing a lack of the protein sclerostin. This protein is produced by osteocytes and exerts its effect as an inhibitor of bone formation by blocking the Wnt signaling pathway. By the use of a monoclonal antibody that can block sclerostin a novel therapeutic pathway for rebuilding bone has been described. Preclinical studies have shown increased bone

[Medium-term results of anatomic straight-shaft IMAGE implantation in primary hip joint endoprosthetics--a prospective study].

AIM: The IMAGE prosthesis was developed with the objective of proximal load induction to avoid stress shielding and proximal bone resorption. In this study patients were followed clinically and radiologically with a special emphasis on osteointegration of the shaft in the proximal and distal regions. In addition, the reconstruction of the bony geometry of the proximal humerus was investigated. METHOD: 95 consecutive patients (mean age: 61.1 years) with an IMAGE shaft were followed up prospectively over five years after primary hip arthroplasty. Radiologically, the osteointegration was apprais

Bisphosphonate-induced exposed bone (osteonecrosis/osteopetrosis) of the jaws: risk factors, recognition, prevention, and treatment.

PURPOSE: Bisphosphonates inhibit bone resorption and thus bone renewal by suppressing the recruitment and activity of osteoclasts thus shortening their life span. Recently three bisphosphonates, Pamidronate (Aredia; Novartis Pharmaceuticals, East Haven, NJ), Zoledronate (Zometa; Novartis Pharmaceuticals), and Alendronate (Fosamax; Merck Co, West Point, VA) have been linked to painful refractory bone exposures in the jaws. MATERIALS AND METHODS: One hundred-nineteen total cases of bisphosphonate-related bone exposure were reviewed. RESULTS: Thirty-two of 119 patients (26%) received Aredia, 48

Geriatric chronic recurrent multifocal osteomyelitis (CRMO) mimicking multifocal multiple myeloma: a first in an octogenarian.

Chronic recurrent multifocal osteomyelitis (CRMO), an autoinflammatory bone disorder characterized by non-bacterial osteomyelitis causing recurrent multifocal bone lesions, is a well-known, yet uncommon pediatric condition that rarely affects adults; to date, it has never been diagnosed over the age of 75. The following report will discuss the first octogenarian diagnosed with CRMO and therefore represents an exceptionally rare presentation of a rare disease. An 83-year-old woman presented with progressive right shoulder, forearm, and hip pain, with associated weight loss and global weakness,

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… CRMO and SAPHO syndrome have been shown to share many of the same characteristics, including osteitis, a multifocal presentation, and hyperostosis and thus are sometimes diagnosed interchangeably. … Despite their relatively older age, our patient was diagnosed with CRMO, as they presented with multifocal lesions of their extremities without any evidence of cutaneous lesions or hyperostosis. …

Сфеноидный грибковый носорогсинусит по всему спектру заболеваний: серия неинвазивных грибковых шариков и смертельный острый инвазивный грибковый носорогсинусит.

ВВЕДЕНИЕ: Сфеноидный грибковый носорогсинит является необычным, но клинически важным субъектом, начиная от неинвазивного грибкового шара и заканчивая быстро фатальным острым инвазивным грибковым носорогом. ПРЕДОСТАВЛЕНИЕ КИСЛОТА: Мы сообщаем о двух противоположных случаях грибкового заболевания сфеноида. Во-первых, 28-летняя иммунокомпетентная женщина с хронической односторонней головной болью, вызванной изолированным феноидным грибком, который успешно лечился эндоскопической феноидотомией и расчисткой. Вторым был 49-летний мужчина с сахарным диабетом, который подавался с орбитальным участием,

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… Sagittal images showed expansion of the left sphenoid sinus with opacification extending to the sphenoethmoidal recess and associated wall hyperostosis. There was no radiologic evidence of erosion into the sella, clivus, cavernous sinus region, or carotid canal. … CT findings suggestive of a sphenoid fungal ball include isolated sphenoid opacification, intralesional hyperdensity or calcification, wall thickening, and hyperostosis, without aggressive extrasinus erosion. …

Physicians' perspectives on the diagnosis and treatment of adult SAPHO syndrome: a cross-sectional study from China.

OBJECTIVE: To investigate the practices of doctors in the diagnosis and treatment of adult SAPHO syndrome. METHODS: A cross-sectional study was conducted by distributing online questionnaires to physicians across five hospitals, specifically targeting departments involved in the diagnosis and treatment of adult SAPHO syndrome, including rheumatology, dermatology, and orthopedics. RESULTS: A total of 103 clinical physicians participated in the survey.The three most commonly observed clinical features of adult SAPHO syndrome were elevated ESR and CRP (82/84), focal swelling and fever (73/84),

IL-23/Th17 targeted therapies in SAPHO syndrome. A case series.

SAPHO syndrome is a rare entity with skin and rheumatologic inflammatory presentation. The treatment is not standardized, and in case of inadequate response to anti-inflammatory drugs, the use of anti-TNF or anti-IL-1 biologic treatments has been reported. The IL-23/Th17 axis may be involved in SAPHO syndrome. We report the results of six courses of IL-23 and IL-17 targeted therapies (3 ustekinumab and 3 secukinumab) in patients with SAPHO syndrome unresponsive to previous treatments (csDMARDs and bDMARDs). With a mean treatment duration of 5.5 months, improvement of skin symptoms was noticed

Inherited Arterial Calcification Syndromes: Etiologies and Treatment Concepts.

PURPOSE OF REVIEW: We give an update on the etiology and potential treatment options of rare inherited monogenic disorders associated with arterial calcification and calcific cardiac valve disease. RECENT FINDINGS: Genetic studies of rare inherited syndromes have identified key regulators of ectopic calcification. Based on the pathogenic principles causing the diseases, these can be classified into three groups: (1) disorders of an increased extracellular inorganic phosphate/inorganic pyrophosphate ratio (generalized arterial calcification of infancy, pseudoxanthoma elasticum, arterial calcif

Life-threatening disseminated tuberculosis as a complication of TNF-α blockade in an adolescent.

Life-threatening disseminated tuberculosis developed in a 17-year-old girl who was treated with the TNF-α blocker adalimumab for refractory SAPHO syndrome. The patient presented to the emergency department with dyspnea and somnolence and within 2 h developed the clinical picture of a septic shock. In addition to this unusual presentation, she showed a complicated course with increasing cerebral granuloma formation in spite of adequate antimycobacterial treatment. Immune reconstitution after discontinuation of TNF blockade may contribute to this "paradoxical reaction." Possible implications for

First missense mutation in the SOST gene causing sclerosteosis by loss of sclerostin function.

Sclerosteosis is a rare bone dysplasia characterized by greatly increased bone mass, especially of the long bones and the skull. Patients are tall, show facial asymmetry and often have syndactyly. Clinical complications are due to entrapment of cranial nerves. The disease is thought to be due to loss-of-function mutations in the SOST gene. The SOST gene product, sclerostin, is secreted by osteocytes and transported to the bone surface where it inhibits osteoblastic bone formation by antagonizing Wnt signaling. In a small Turkish family with sclerosteosis, we identified a missense mutation (c.4

Associations between treatments and response to treatments from a large multicentre study of equine sinonasal disease.

BACKGROUND: No large study has examined the treatment and response to treatment of horses with sinonasal disease confirmed by computed tomog … ve cure (OR 0.38, 95% CI 0.24-0.61, p = 0.001) than those treated by other methods. Cases with bone hyperostosis graded as 'moderate or severe' were less likely to be cured than those with 'none or mild' (OR 0.54, 95% CI 0.35-0.82, p = 0.004). MAIN LIMITATIONS: Retrospective study with incomplete data for some cases. CONCLUSIONS: Oral extraction and sinoscop …

New explanation for autosomal dominant high bone mass: Mutation of low-density lipoprotein receptor-related protein 6.

LRP5 encodes low-density lipoprotein receptor-related protein 5 (LRP5). When LRP5 with a Frizzled receptor join on the surface of an osteobl … tion. In both families the LRP6 mutation co-segregated with striking generalized osteosclerosis and hyperostosis. Clinical features shared by the seven LRP6 HBM family members and ten LRP5 HBM patients included a broad jaw, torus palatinus, teeth encased in bone and, reportedly, resistance to fracturing and inability to float in water. For both HBM disorders, …

Surgically managed human cystic echinococcosis in north-eastern Iran: a single center's experience from 2001 to 2008.

Tumor necrosis factor-alpha (TNFalpha) inhibition is effective in the treatment of moderate-to-severe psoriasis. We report on 120 patients f … g spondylitis (n = 21), psoriasis (n = 10), Crohn disease (n = 8), SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome (n = 3), psoriatic arthritis (n = 2), and other diagnoses (n = 15). Psoriasis (except palmoplantar pustular type) was the most common adverse effect during anti-TNFalpha treatment (n = 73), followed by palmoplantar pustular psoria …

Correction of coronal suture synostosis using suture and dura mater allografts in rabbits with familial craniosynostosis.

OBJECTIVE: Resynostosis following surgical correction of craniosynostosis is a common clinical correlate. Recent studies suggest that the du … d patent coronal sutures, suture-only allografts had fused coronal sutures with extensive endosteal hyperostosis, dura mater-only allografts had some new bone in the suturectomy site that resembled rudimentary osteogenic fronts, and suturectomy controls had extensive endosteal bone formation and resynostosis of the suturectomy site. Significantly (p < .05) mor …

Computer-aided diagnosis system for thoracic computed tomography of rib fractures in older emergency patients: A preliminary study.

The peculiarities of older individuals related to osteoporosis and hyperostosis may lead to a higher rate of misdiagnosis of rib fractures on computed tomography (CT) images in older than in middle-aged/young people when using radiologist-only reading. However, none of these studies on rib fracture computer-aided diagnostic (CAD) systems grouped patients by age or evaluated the value of using CAD in older patients. To address these gaps, we divided 1,012 blunt chest trauma emergency patients who underwent chest CT into middle-aged/young and older groups with a cutoff age of 60 years. CT images…

Magnetic Resonance Imaging in 50 Captive Non-domestic Felids - Technique and Imaging Diagnoses.

Magnetic resonance imaging (MRI) is the recognized gold standard for diagnostic imaging of the central nervous system in human and veterinary patients. Information on the use of this modality and possible imaging abnormalities in captive non-domestic felids is currently limited to individual case reports or small case series. This retrospective study provides information on technique and imaging findings in a cohort of cases undergoing MRI at an academic Veterinary Medical Center. The University of Tennessee College of Veterinary Medicine MRI database was searched for non-domestic felids under

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… Another lion was diagnosed with calvarial hyperostosis but also tested positive for toxoplasmosis. The clinical significance of calvarial hyperostosis was therefore undetermined. … One additional lion had a diagnosis of “probable occipital hyperostosis” but had only mild clinical signs at presentation and was reported to be neurologically normal 8 years after the MRI study. …

Sclerostin neutralization unleashes the osteoanabolic effects of Dkk1 inhibition.

The WNT pathway has become an attractive target for skeletal therapies. High-bone-mass phenotypes in patients with loss-of-function mutations in the LRP5/6 inhibitor Sost (sclerosteosis), or in its downstream enhancer region (van Buchem disease), highlight the utility of targeting Sost/sclerostin to improve bone properties. Sclerostin-neutralizing antibody is highly osteoanabolic in animal models and in human clinical trials, but antibody-based inhibition of another potent LRP5/6 antagonist, Dkk1, is largely inefficacious for building bone in the unperturbed adult skeleton. Here, we show that

Nuclear Medicine Imaging in the Dentomaxillofacial Region.

Nuclear medicine studies evaluate physiology on a molecular level providing earlier detection of lesions before morphologic change is evident. 99mTc-MDP and 18F-fluoride bone scans detect osteomyelitis earlier than radiographs and computed tomography (CT); aid in diagnosis of temporomandibular joint disorder; and evaluate activity of condylar hyperplasia, extent of Paget disease, and viability of bone grafts. 18F-FDG PET/CT distinguish between soft tissue and bone infections and diagnose osteomyelitis complicated by fracture or surgery. FDG PET is more accurate than CT alone and has a major ro

Acne fulminans.

Acne fulminans (AF) is a rare and severe form of inflammatory acne presenting clinically with an abrupt outburst of painful, hemorrhagic pustules and ulceration, that may or may not be associated with systemic symptoms, such as fever, polyarthritis, and laboratory abnormalities. It typically affects male teenagers with a pre-existing acne. Although the pathogenetic mechanism has not been established yet, a role of genetic, abnormal immunologic response, drugs intake, hormonal imbalance and viral infection, as causal factors, has been identified. AF may occur as a single disease or may be assoc