SAPHO syndrome: A review Rukavina, Iva
Journal of children's orthopaedics,
02/2015, Letnik:
9, Številka:
1
Journal Article
Recenzirano
Odprti dostop
Introduction
Synovitis–acne–pustulosis–hyperostosis–osteitis (SAPHO) is an acronym for various osteoarticular and dermatological manifestations that can appear in the same patient. It is a rare ...syndrome, but since its awareness has increased, there have been more and more such reports in the literature.
Aims
The objectives of this review are to summarize the current state of knowledge on pediatric and adult-onset SAPHO syndrome, and to discuss treatment strategies that should be considered.
Results
The SAPHO syndrome can affect patients of any age, and its etiology is still not known. The syndrome has its cognizable radiological characteristics that are most important in making the diagnosis. There are several diagnostic criteria as well, but they need further validation. No standard treatment protocols are available and current treatment options are not evidenced-based due to the rarity of the syndrome. Therapy is empirical and aimed at easing pain and modifying the inflammatory process. It includes nonsteroidal anti-inflammatory drugs (NSAIDs) as the first-line agents. Antibiotics, corticosteroids, disease-modifying anti-rheumatic drugs, biologicals targeting tumor necrosis factor alpha or interleukin-1, and bisphosphonates have all been used with variable success. Surgery is reserved to treat complications. Even though it is a disease with good long-term prognosis, its treatment remains a challenge and the results are known to be disappointing, especially with the skin component of the disease.
Conclusion
It is expected that these patients present at the time of diagnosis and the treatment should be as early, effective, and safe as possible in order to prevent osteoarticular progression and to limit the adverse events associated with pharmacological drugs.
In this study, natural deep eutectic solvents (NADES) formed by choline chloride (ChCl), sucrose, fructose, glucose, and xylose, were used to extract antioxidants from the halophyte Polygonum ...maritimum L. (sea knotgrass) and compared with conventional solvents (ethanol and acetone). NADES and conventional extracts were made by an ultrasound-assisted procedure and evaluated for in vitro antioxidant properties by the radical scavenging activity (RSA) on the 2,2-diphenyl-1-picrylhydrazyl (DPPH) radical, oxygen radical absorbance capacity (ORAC), and copper chelating activity (CCA). Samples were profiled by liquid chromatography (LC)-electrospray ionization (ESI)-QTOF-MS analysis. ChCl:fructose was more efficient in the DPPH assay, than the acetone extract. ChCl:sucrose and ChCl:fructose extracts had the highest ORAC when compared with the acetone extract. NADES extracts had higher CCA, than the acetone extract. The phenolic composition of the NADES extracts was less complex than the conventional extracts, but the proportions of major antioxidants, such as flavonols and flavan-3-ols, were similar in all the solvents. Myricitrin was the major flavonoid in all of the samples, while gallic acid was the main phenolic acid in the conventional extracts and present in a greater amount in ChCl:fructose. Results suggest that NADES containing ChCl and sucrose/fructose can replace conventional solvents, especially acetone, in the extraction of antioxidants from sea knotgrass.
Background: Brain-gut interaction involves, among others, peptidergic
growth factors which are native in GI tract and have strong antiulcer potency and
thus could from periphery beneficially affect ...CNS-disorders. We focused on the
stable gastric pentadecapeptide BPC 157, an antiulcer peptidergic agent, safe in
inflammatory bowel disease trials and now in multiple sclerosis trial, native and
stable in human gastric juice.
Methods: Review of our research on BPC 157 in terms of brain-gut axis.
Results: BPC 157 may serve as a novel mediator of Robert's cytoprotection, involved
in maintaining of GI mucosa integrity, with no toxic effect. BPC 157 was successful
in the therapy of GI tract, periodontitis, liver and pancreas lesions, and in the healing of various tissues
and wounds. Stimulated Egr-1 gene, NAB2, FAK-paxillin and JAK-2 pathways are hitherto implicated.
Initially corresponding beneficial central influence was seen when BPC 157 was given peripherally and a
serotonin release in particular brain areas, mostly nigrostriatal, was changed. BPC 157 modulates
serotonergic and dopaminergic systems, beneficially affects various behavioral disturbances that
otherwise appeared due to specifically (over)stimulated/damaged neurotransmitters systems. Besides,
BPC 157 has neuroprotective effects: protects somatosensory neurons; peripheral nerve regeneration
appearent after transection; after traumatic brain injury counteracts the otherwise progressing course, in
rat spinal cord compression with tail paralysis, axonal and neuronal necrosis, demyelination, cyst
formation and rescues tail function in both short-terms and long-terms; after NSAIDs or insulin overdose
or cuprizone encephalopathies were attenuated along with GI, liver and vascular injuries.
Conclusion: BPC 157, a gastric peptide, may serve as remedy in various CNS-disorders.
We attempted throughout the NO-system to achieve the particular counteraction of the ketamine-induced resembling “negative-like” schizophrenia symptoms in rats using pentadecapeptide BPC 157, and ...NO-agents, NG-nitro-L-arginine methylester (L-NAME), and/or L-arginine, triple application. This might be the find out the NO-system organized therapy (i.e., simultaneously implied NO-system blockade (L-NAME) vs. NO-system over-stimulation (L-arginine) vs. NO-system immobilization (L-NAME+L-arginine)). The ketamine regimen (intraperitoneally/kg) included: 3 mg (cognitive dysfunction, novel object recognition test), 30 mg (anxiogenic effect (open field test) and anhedonia (sucrose test)), and 8 mg/3 days (social withdrawal). Medication (mg/kg intraperitoneally) was L-NAME (5), L-arginine (100), and BPC 157 (0.01), alone and/or together, given immediately before ketamine (L-NAME, L-arginine, and combination) or given immediately after (BPC 157 and combinations). BPC 157 counteracted ketamine-cognition dysfunction, social withdrawal, and anhedonia, and exerted additional anxiolytic effect. L-NAME (antagonization, social withdrawal) and L-arginine (antagonization, cognitive dysfunction, anhedonia) both included worsening cognitive dysfunction, anhedonia, and anxiogenic effect (L-NAME), social withdrawal, and anxiogenic effect (L-arginine). Thus, ketamine-induced resembling “negative-like” schizophrenia symptoms were “L-NAME non-responsive, L-arginine responsive” (cognition dysfunction), “L-NAME responsive, L-arginine non-responsive” (social withdrawal), “L-NAME responsive, L-arginine responsive, opposite effect” (anhedonia) and “L-NAME responsive, L-arginine responsive, parallel effect” (both anxiogening). In cognition dysfunction, BPC 157 overwhelmed NO-agents effects. The mRNA expression studies in brain tissue evidenced considerable overlapping of gene overexpression in healthy rats treated with ketamine or BPC 157. With the BPC 157 therapy applied immediately after ketamine, the effect on Nos1, Nos2, Plcg1, Prkcg, and Ptgs2 (increased or decreased expression), appeared as a timely specific BPC 157 effect on ketamine-specific targets.
Abstract Objective To define the clinical, radiologic and molecular characteristics of a patient with early progressive osteoarthritis and mild spondyloepiphyseal dysplasia. Methods We describe an ...18-year-old girl with early progressive osteoarthritis and mild spondyloepiphyseal dysplasia. The index case underwent a physical examination, anthropometric measurements and radiologic and laboratory studies. DNA of the patient and her only living parent (mother) was sequenced for the type II collagen gene (COL2A1). Results Mild scoliosis was noticed in the proband at the age of 13 years. At the same age, she began to have arthralgia in almost all the joints and osteoarthritis progressed fast, necessitating a hip, knee and ankle prosthesis at the age of 18 years. She was eumorphic with no ocular or hearing abnormalities. Molecular testing of the COL2A1 gene revealed a p.Gly204Val mutation. The mutation was absent in the healthy mother. Conclusion This patient provides further proof that an early osteoarthritic phenotype can be caused by a mutation in the COL2A1 gene.
Notre travail montre l’urbanisme de la ville de Zadar en Dalmatie, soit le développement de ses contenus urbains de ses débuts à l’Antiquité jusqu’à l’an 1409. Notre analyse urbanistique repose avant ...tout sur les monuments et autres vestiges matériels. Les sources historiques pertinentes furent bien évidemment prises en compte. Nous avons présenté la construction architecturale au cours de quatre périodes : Antiquité, Antiquité tardive, Haut Moyen Âge et Moyen Âge central et tardif. Pour chaque période, nous avons présenté les constructions de Zadar en quatre catégories distinctes d’après leur type d’architecture : fortifications, architecture profane publique, architecture profane privée et architecture sacrée. Les vestiges matériels liés à l’infrastructure de la ville furent également présentés (chaussées, canaux d’évacuation, etc.) Le développement de Zadar, à savoir la transformation de son urbanisme, fut analysé au travers de plusieurs époques en lien étroit avec la périodisation des événements historiques survenus sur la côte orientale de l’Adriatique. La période protohistorique fut également prise en compte. Notre analyse montre que la période antique définit le réseau orthogonal de voies de la cité. La construction intensive du Moyen Âge donna à la ville une physionomie urbaine qui conserva sur presque toute la surface de la cité les éléments principaux de l’urbanisme antique
This dissertation presents an analysis of the urbanism of the city of Zadar in Dalmatia, the development of the city and its urban contents from its beginnings in Antiquity until 1409. The urban analysis is based primarily upon the various surviving monuments and remnants. Relevant historical sources have also been taken into account. The different elements have been classified and presented according to four historical periods: Antiquity, Late Antiquity, Early Middle Ages, High and Late Middle Ages. Within each period, Zadar's buildings are categorised according to four typologies: Fortifications, Public Secular Structures, Private Secular Structures and Sacred Buildings. The remains of the city's infrastructure (sidewalks, drainage channels, etc.) have also been presented. The development of the city of Zadar, through its urban transformation, has been analysed through the outlined periods while keeping the usual periodization of general historic events on the eastern Adriatic coast. The protohistoric period has also been considered. Analysis has also revealed that the regular orthogonal grid of the city was defined in Antiquity, and that despite intensive construction during the medieval period when the city developed its urban physiognomy, nearly all the principle characteristics of the city established during Antiquity have been preserved.
Due to habitat loss and fragmentation, the hermit beetle, Osmoderma eremita species complex (Coleoptera; Scarabidae) and the European red click beetle, Elater ferrugineus Linnaeus, 1758 (Coleoptera: ...Elateridae) are considered threatened in many European countries. Their presence in a large part of South-Eastern Europe has been sporadically recorded and these findings are mainly historical. Here we present the most recent findings of both species, assembled mainly throughout this first systematic study aiming to collect data on the hermit beetle within mapping and monitoring activities of saproxylic species protected by the European Habitat Directive (Annexes II and IV of Council Directive 92/43/EEC) within the Mediterranean, Continental and Alpine biogeographical regions of Croatia, and current literature data. To facilitate mapping activities and species range monitoring to improve the proper management of suitable habitats, we created habitat suitability maps using recent findings for both species, and predicted distribution were overlapped and analysed with historical data and protected areas. Most of the individuals were captured using flight intercept cross-vein funnel traps baited with pheromone for the hermit beetle placed within natural forest dominated by oak, beech and montane beech-fir forests, on sites with and without any forest management. Among set of 11 environmental variables, autumn precipitation, spring mean temperature and slope contributed most to the beetle distribution models. Data analyses indicated that the European red click beetle has much wider environmental envelope in which it can occur and that the hermit beetle can be used as surrogate species for the European red click beetle.
Stress cardiomyopathy or Takotsubo cardiomyopathy is a new syndrome and still insufficiently recognized among emergency patients, hospitalized patients. Many different physical and emotional ...stressors are triggers, but individual susceptibility to Takotsubo cardiomyopathy can not be predicted. Takotsubo cardiomyopathy in surgical and critical care population is a huge diagnostic challenge. Apart that these patients are treated in stressful environment and conditions. Postoperatively due to change of consciousness and inability to speak we can not rely on verbal symptoms to make differential diagnosis. Although essential sometimes they can not be submitted to coronary angiography to exclude obstructive coronary disease due many risk factors. So, then we follow clinical course, electrocardiographic, radiologic and echocardiographic dynamic changes, laboratory findings and consiliar opinion to make diagnosis. We represent a case of Takotsubo cardiomyopathy in a 59-years old postmenopausal Caucasian woman after tracheal extubation. She was submitted to surgery of intraabdominal collection evacuation in short general endotracheal anesthesia.
Juvenile systemic lupus erythematosus (JSLE) is a systemic autoimmune chronic disease that can affect any part of the body. It is characterized by the formation of antibodies against nuclear ...antigens. Vasculitis may be found in SLE, but it scarcely complies with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) criteria. We report five cases of severe JSLE associated with AAV diagnosed between 1991 and 2013 in three university-based tertiary care centers. The patients (3 girls and 2 boys, aged 12 to 17) presented with a severe clinical picture and the following features: cytopenia (n=5), autoimmune hepatitis (n=3), lupus nephritis (n=1), pancreatitis (n=1), secondary antiphospholipid syndrome (n=2), impending respiratory failure (n=2), and gastrointestinal bleeding (n=1). All patients were proteinase 3 (PR3) ANCA positive, while two of them were myeloperoxidase (MPO) and PR3 ANCAs positive at the same time. They were treated with corticosteroids and immunosuppressive drugs. Remission of the disease was achieved in three patients. The course of the disease was worsening in two patients and we included rituximab (anti-CD20) in therapy. All of our patients presented as the most severe SLE patients, who must be diagnosed as soon as possible and treated very intensively. Since the comorbidity of JSLE and AAV occurs very rarely in children, presentation of such patients, their clinical pictures, treatment, and the course of the diseases are experiences that can be of great help.
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