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https://www.readbyqxmd.com/read/29212418/clinical-characteristics-and-outcome-of-childhood-acute-promyelocitic-leukemia-apl-in-saudi-arabia-a-multicenter-saphos-leukemia-group-study
#1
Wasil Jastaniah, Abdulrahman Alsultan, Saad Al Daama, Walid Ballourah, Mohamed Bayoumy, Faisal Al-Anzi, Omar Al Shareef, Mohammed Burhan Abrar, Reem Al Sudairy, Ibrahim Al Ghemlas
BACKGROUND: Acute promyelocytic leukemia (APL) is a rare form of acute myelogenous leukemia (AML). Survival rates exceed 80% in developed countries. Successful treatments rely on all-trans retinoic acid with anthracycline-based chemotherapy. Availability of modern care and public knowledge play important roles in pediatric APL survival. METHOD: A cytogenetic diagnosis of APL was confirmed in 30 (14.5%) out of 207 children consecutively diagnosed with de novo AML between January 2005 and December 2012 at nine cancer care centers in Saudi Arabia...
December 7, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29185291/clinical-images-marked-inflammation-in-a-patient-with-cervical-vertebral-sapho-complicated-by-vertebral-body-collapse-and-severe-kyphosis
#2
Sara Borok, Gideon Flusser, Ori Elkayam
No abstract text is available yet for this article.
November 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29034454/successful-treatment-of-sapho-syndrome-with-apremilast
#3
S Adamo, J Nilsson, A Krebs, U Steiner, A Cozzio, L E French, A G A Kolios
Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare disease with inflammatory osteoarticular and skin involvement. The pathogenesis of SAPHO syndrome remains unclear, but evidence suggests it may be an autoinflammatory disease triggered upon exposure to infectious agents in genetically predisposed individuals. Induction of the IL-23/Th17 axis as well as neutrophil activation seem to play a key role, and therapies targeting these immunological pathways, including TNF-inhibitors, ustekinumab, secukinumab and the IL-1 inhibitor anakinra are potential treatment options that need further investigation...
October 16, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/29033438/small-cell-lung-cancer-associated-with-sapho-syndrome
#4
Kosuke Imamura, Shinya Sakata, Yasumiko Sakamoto, Sho Saeki
No abstract text is available yet for this article.
October 16, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28977552/synovitis-acne-pustulosis-hyperostosis-osteitis-sapho-paradoxical-reactions-and-different-responses-to-tumour-necrosis-factor-inhibitors
#5
Jean Liew, Teena Huan Xu, Cong-Qiu Chu
No abstract text is available yet for this article.
July 28, 2017: Rheumatology
https://www.readbyqxmd.com/read/28968231/pustulotic-arthro-osteitis-sonozaki-syndrome-a-rare-case-report
#6
Hatice Resorlu, Besir Sahin Inceer, Sevilay Kılıc, Selda Isık
Pustulotic arthro-osteitis is a rare disease involving the skin and musculoskeletal system that was first described by Sonozaki. Onset is frequently seen at age 30-40. The prevalences between the sexes are similar. Palmoplantar pustulosis and sternoclavicular joint involvement are the most typical findings. It may be difficult to distinguish seronegative spondyloarthropathies and SAPHO syndrome due to sacroiliac joint, vertebral column and peripheral joint involvement. Arthritis being non-erosive and short-lived in character and the absence of deformity or contracture in the joints are significant clinical characteristics...
September 22, 2017: Journal of Back and Musculoskeletal Rehabilitation
https://www.readbyqxmd.com/read/28924111/synovitis-acne-pustulosis-hyperostosis-osteitis-sapho-syndrome-with-significant-bilateral-pleural-effusions
#7
Shoichi Hasegawa, Hiroki Yabe, Naoya Kaneko, Eri Watanabe, Takahisa Gono, Chihiro Terai
We herein report a rare case of a 66-year-old woman who had synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome with marked sternal osteitis and bilateral pleural effusions. SAPHO syndrome was diagnosed based on the characteristic features of a hyperostotic sternum and thoracic spine. The inflammatory changes of sternal osteitis and involvement of the adjacent soft tissue were assumed to be the cause of the pleural effusions. The effusions decreased during the natural course of the disease and resolved after methotrexate therapy...
October 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28884797/sapho-syndrome-associated-with-hidradenitis-suppurativa-and-pyoderma-gangrenosum-successfully-treated-with-adalimumab-and-methotrexate-a-case-report-and-review-of-the-literature
#8
REVIEW
Dunja A Vekic, Jane Woods, Peter Lin, Geoffrey D Cains
SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a rare inflammatory condition describing the combination of skin, bone, and joint manifestations that has a heterogeneous presentation. We report a case of severe SAPHO syndrome in association with hidradenitis suppurativa and pyoderma gangrenosum in a 27-year-old male. The patient had an initial migratory arthritis affecting the knees, ankles, metacarpophalangeal joints, proximal interphalangeal joints, wrists, shoulder, and lower back, which progressed to a persistent arthritis and swelling at the sternum, shoulders, wrists, hands, feet, and lower back...
September 7, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28880693/clinical-features-and-radiological-findings-of-67-patients-with-sapho-syndrome
#9
Hiroshi Okuno, Munenori Watanuki, Yoshiyuki Kuwahara, Akira Sekiguchi, Yu Mori, Shin Hitachi, Keiki Miura, Ken Ogura, Mika Watanabe, Masami Hosaka, Masahito Hatori, Eiji Itoi, Katsumi Sato
OBJECTIVES: The purpose of this study was to facilitate the understanding of the SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis) syndrome by analyzing the clinical and radiological features of 67 Japanese patients with SAPHO syndrome. METHODS: Sixty-seven Japanese patients (female/male: 44/23, mean age at onset: 48.5 years) were diagnosed with SAPHO syndrome from 2002 to 2013 at our hospital. Medical records and radiological imaging of these patients were retrospectively reviewed...
September 21, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28774447/sapho-and-recurrent-multifocal-osteomyelitis
#10
REVIEW
Simon Greenwood, Antonio Leone, Victor N Cassar-Pullicino
SAPHO and recurrent multifocal osteomyelitis are complex inflammatory conditions that clinical radiologists play an essential part in diagnosing. They present with a wide range of musculoskeletal and skin manifestations, and exhibit several key diagnostic features that, when present, make the diagnoses unequivocal. The overall population group is young. Diagnostic delay is common with a relapsing and remitting clinical course and often subtle early radiologic findings. This article provides an up-to-date insight into both conditions, including their multifaceted pathogenesis, effective therapeutic options, and advanced imaging features, to arm radiologists with the knowledge required to make the diagnoses confidently in a timely manner...
September 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28765183/a-case-of-bronchiolitis-obliterans-organising-pneumonia-associated-with-sapho-synovitis-acne-pustulosis-hyperostosis-osteitis-syndrome
#11
Fawad Hameed, Henry Steer
A 57-year-old woman with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome presented with recurrent episodes of pneumonia. She was treated with multiple courses of antibiotics with no success. The transbronchial biopsy undertaken via bronchoscopy revealed organising pneumonia (OP). She was treated with steroids and responded well with full clinical recovery and normalisation of her chest X-ray.To our knowledge, this is the first reported case of OP in association with SAPHO syndrome. This case report highlights the importance of considering OP in patients with SAPHO syndrome who present with chest infection...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28725947/clinical-heterogeneity-of-sapho-syndrome-challenging-diagnose-and-treatment
#12
REVIEW
Francesco Cianci, Angelo Zoli, Elisa Gremese, Gianfranco Ferraccioli
Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare disease which is often misdiagnosed and under-recognized, because of its peculiar and heterogeneous clinical presentation. Its main features consist of cutaneous and osteoarticular manifestations, the latter affecting more often the anterior chest wall and having typical radiologic findings. There are no validated diagnostic criteria for SAPHO and no guidelines for treatment, due mainly to its rarity; as a consequence, therapy is empirical and aimed to control pain and modifying inflammatory process...
September 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28696038/recognizing-syndromic-hidradenitis-suppurativa-a-review-of-the-literature
#13
REVIEW
J Gasparic, P Theut Riis, G B Jemec
Hidradenitis suppurativa (HS) is an inflammatory skin disease causing painful inflammation and suppuration. It may occur in rare syndromes: follicular occlusion, Bazex-Dupré-Christol, Down's, KID, PAPASH, PASS, PASH, and SAPHO syndromes, as well as Dowling-Degos disease. An overview of syndromic HS may inform the search for aetiological factors in HS. PubMed, Ovid and Web of Science were systematically searched using '(hidradenitis OR acne invers*) AND (syndrome OR KID OR PASS OR PAPA OR PASH OR SAPHO OR bazex-dupre OR 'dowling degos' OR triad OR tetrad)' and Cochrane Library using 'hidradenitis OR acne invers*'...
July 11, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28634314/-a-case-of-moyamoya-disease-associated-with-sapho-syndrome
#14
Nobutaka Horie, Mayuko Baba, Ken Kawada, Yuuki Matsunaga, Eisaku Sadakata, Yoichi Morofuji, Tsuyoshi Izumo, Minoru Morikawa, Takeo Anda, Takayuki Matsuo
Moyamoya disease is a unique occlusive disease of the internal carotid artery(ICA)with moyamoya vessels that can lead to transient ischemic attacks and hemorrhagic stroke. When other inherited or acquired disorders and conditions occur in conjunction with moyamoya disease, the syndrome is known as quasi-moyamoya disease. We report the case of a 34-year-old woman with a past history of SAPHO(Synovitis-Acne-Pustulosis-Hyperostosis-Osteomyelitis)syndrome, who presented with arm weakness and headache. Magnetic resonance angiography revealed severe terminal stenosis of the left ICA with moyamoya vessels, and she was diagnosed as moyamoya disease associated with SAPHO syndrome...
June 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/28579754/paradoxical-sapho-syndrome-observed-during-anti-tnf%C3%AE-therapy-for-crohn-s-disease
#15
Hitoshi Amano, Reikei Matsuda, Tomohiko Shibata, Daisuke Takahashi, Shinichiro Suzuki
Currently, anti-TNFα antibodies are used to treat Crohn's disease. We report on a 45-year-old Japanese female with Crohn's disease developing SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome following exposure to the anti-TNFα antibody adalimumab. Initially, adalimumab induced remission, but the patient showed SAPHO syndrome 11 weeks following the start of adalimumab therapy for the first time. Cutaneous and articular involvement were exacerbating the condition, so adalimumab was discontinued and the patient was put on low-dose methotrexate to control her symptoms...
2017: Biologics: Targets & Therapy
https://www.readbyqxmd.com/read/28545486/depression-in-patients-with-sapho-syndrome-and-its-relationship-with-brain-activity-and-connectivity
#16
Jie Lu, Yanping Duan, Zhentao Zuo, Wenrui Xu, Xuewei Zhang, Chen Li, Rong Xue, Hanzhang Lu, Weihong Zhang
BACKGROUND: Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a rare disease and there is no related literature concerning psychiatric symptoms in SAPHO patients. Thus, we believe that this will be the first paper to explore the episode and the neurobiological basis of depression symptoms in SAPHO patients using resting state functional magnetic resonance imaging (rs-fMRI). Twenty-eight SAPHO patients and fifteen age- and gender- matched normal controls (NC) were consecutively submitted to psychiatric evaluation and rs-fMRI scanning...
May 25, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28532819/il-23-th17-targeted-therapies-in-sapho-syndrome-a-case-series
#17
Daniel Wendling, François Aubin, Frank Verhoeven, Clément Prati
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)...
December 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28516971/salivary-biomarkers-and-proteomics-future-diagnostic-and-clinical-utilities
#18
M Castagnola, E Scarano, G C Passali, I Messana, T Cabras, F Iavarone, G Di Cintio, A Fiorita, E De Corso, G Paludetti
Saliva testing is a non-invasive and inexpensive test that can serve as a source of information useful for diagnosis of disease. As we enter the era of genomic technologies and -omic research, collection of saliva has increased. Recent proteomic platforms have analysed the human salivary proteome and characterised about 3000 differentially expressed proteins and peptides: in saliva, more than 90% of proteins in weight are derived from the secretion of three couples of "major" glands; all the other components are derived from minor glands, gingival crevicular fluid, mucosal exudates and oral microflora...
April 2017: Acta Otorhinolaryngologica Italica
https://www.readbyqxmd.com/read/28500364/radiologists-need-to-be-aware-of-secondary-central-venous-stenosis-in-patients-with-sapho-syndrome
#19
Mizuho Suzuki, Hidenori Kanazawa, Takeshi Shinozaki, Hideharu Sugimoto
OBJECTIVES: We aimed to define central venous stenosis (CVS) caused by sternocostoclavicular hyperostosis as a feature of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome on routine contrast-enhanced computed tomography (CT) images. The relationship between SAPHO syndrome and CVS without venous thrombosis caused by anterior chest wall compression has not been investigated. Therefore, the present study evaluated CVS in patients with SAPHO syndrome at our hospital. METHODS: We retrospectively reviewed contrast-enhanced CT images of ten patients with suspected or diagnosed SAPHO syndrome between January 2007 and November 2015...
May 12, 2017: European Radiology
https://www.readbyqxmd.com/read/28499891/sapho-autophagy-il-1-foxo1-and-propionibacterium-cutibacterium-acnes
#20
Jean-Marie Berthelot, Stéphane Corvec, Gilles Hayem
Overt infection by Propionibacterium acnes is lacking in many SAPHO syndromes, and antibiotics have only a transient and incomplete effect, either in SAPHO syndrome or acne. As several auto-inflammatory bone disorders sharing overproduction of IL-1β can mimic SAPHO, this syndrome could partly depend on genetically encoded overproduction of IL-1β. However, cyclic intracellular infections, mostly by P. acnes, can contribute to the enhanced IL-1β release by some skin cells, and probably by bone cells. P. acnes is indeed a powerful trigger of NLRP3-inflammasome activation and IL-1β, leading to osteitis and enhanced mesenchymal cells differentiation in osteoblasts...
May 9, 2017: Joint, Bone, Spine: Revue du Rhumatisme
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