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Jud Philipp, Hafner Franz
No abstract text is available yet for this article.
February 20, 2018: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
Guglielmo Pranteda, Andrea D 'arino, Flavia Pigliacelli, Giulia Pranteda, Gian L Rampioni-Vinciguerra, Ugo Bottoni
No abstract text is available yet for this article.
April 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Tatiana V Beketova, Mikhail Y Volkov, Evgeniy A Naryshkin, Tatiana M Novoselova, Evgeniy L Nasonov
Recent advances in pharmacology have greatly expanded the drug repertoire for treatment of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic disorder, a type of the ANCA-associated vasculitis. Important features of this disease are eosinophilia and anti-myeloperoxidase ANCA presence in around 30-70% of patients. Primary therapy of EGPA includes steroids and cytotoxic drugs, e.g., cyclophosphamide, azathioprine, or methotrexate...
March 21, 2018: Clinical Rheumatology
Manphool Singhal, Pankaj Gupta, Surjit Singh, Niranjan Khandelwal
Kawasaki disease (KD) is an acute idiopathic vasculitis affecting infants and children. Coronary artery abnormalities and myocarditis are the major cardiovascular complications of KD. Coronary artery abnormalities develop in 15-25% of untreated KD. Two-dimensional transthoracic echocardiography has hitherto been considered the modality of choice for evaluation of children with KD. There are, however, several limitations inherent to echocardiography - including limited evaluation of distal vessels, left circumflex artery and poor acoustic window in growing children...
October 31, 2017: Global Cardiology Science & Practice
Tarek Hamed Attia, Saed M Morsy, Bashier A Hassan, Al Shymaa A Ali
Kawasaki disease is an acute vasculitis of early childhood. Its incidence varies among different ethnic groups with higher rates among Asians. In this case series, we presented four cases of Kawasaki disease with incomplete or atypical presentations in Egyptian children. Two cases presented with meningitis, which is not a criteria for the diagnosis of Kawasaki disease. The other two cases presented with pharyngitis and fever, which did not respond to antibiotics. The clinical criteria for diagnosis of Kawasaki disease were either incomplete or appeared sequentially...
October 31, 2017: Global Cardiology Science & Practice
Seung Woo Jeong, Do Hee Kim, Mi Young Han, Sung-Ho Cha, Kyung Lim Yoon
Kawasaki disease (KD) is a childhood vascular disorder of unknown etiology. Concerns have recently been raised regarding vaccinations as a potential risk factor for KD. In addition, various forms of vasculitis have been reported as adverse events following administration after various vaccines. Patients exhibiting post vaccination KD have previously been described; however, thus far, to the best of our knowledge, only one patient exhibiting post influenza vaccination KD has been reported in Japan. The present study describes a case of KD 24 h after immunization with influenza in an infant (age, 18 months) following 6 days of high fever, a body rash that had persisted for 2 days and nonsuppurative bilateral conjunctivitis...
March 2018: Biomedical Reports
Fumika Honda, Hiroto Tsuboi, Hirofumi Toko, Toshihiko Terasaki, Mayu Terasaki, Masaru Shimizu, Ayako Ohyama, Mizuki Yagishita, Hiroyuki Takahashi, Masahiro Yokosawa, Hiromitsu Asashima, Shinya Hagiwara, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
No abstract text is available yet for this article.
March 21, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
D D K Abeyaratne, C Liyanapathirana, C L Fonseka, P W M C S B Wijekoon
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement. It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement. However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation. We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption...
2018: Case Reports in Medicine
Jolanta Parada-Turska, Monika Turska
Polyarteritis nodosa is a systemic necrotizing vasculitis which predominantly affects medium-sized arteries. It is a rare disease nowadays. Both the nomenclature and the classification of polyarteritis nodosa was amended several times in the past. Currently, there is a distinction between the primary form described as classical polyarteritis nodosa and other forms that are associated with their probable cause e.g. with viral hepatitis B, C or HIV infection. Moreover, polyarteritis-like necrotizing vasculitis can appear in the course of genetic diseases caused by mutations in single genes...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Dorota Suszek, Maria Majdan
Cryoglobulinemia is defined as the presence of cryoglobulins in the blood. Cryoglobulinemia is often observed in the course of many diseases (infection, hematological disorders, autoimmune disorders) or has an idiopathic character. The classification of cryoglobulinemia is based on the immunological analysis of cryoglobulins and the activity of the rheumatoid factor (RF). The presence of cryoglobulins may induce cryoglobulinemic vasculitis (CV) which manifests with skin changes, arthritis and the dysfunction of internal organs...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Burçin Şanlidag, Ceyhun Balkan, Nerin Bahçeciler
Kawasaki Disease (KD) is a systemic autoimmune vasculitis that affects small and medium sized vessels. Main complication of Kawasaki Disease is coronary artery aneurism, which has higher risk in case of delayed diagnosis and treatment. Although, complete and incomplete KD cases in different types of immune deficiency diseases have been presented up to date, clinical course of KD in patients with hypogammaglobulinemia (HG) has not been reported. Herein, a case diagnosed as incomplete KD in a child with transient HG of infancy has been reported...
April 1, 2018: Archivos Argentinos de Pediatría
Juyoung Yoo, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
We investigated the development rate and time, risk factors, predictors, and aetiologies of hospitalised infection in Korean patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We retrospectively reviewed the medical records of 154 patients with AAV. Hospitalised infection was considered only when patients were admitted for serious infection related to AAV or AAV treatment. The gap-time was defined as the period from diagnosis to the first hospitalised infection or to the last visit for uninfected patients...
March 20, 2018: Clinical Rheumatology
Ho Jae Kim, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
Mean platelet volume (MPV) was known to be associated with the inflammatory burdens in various diseases, but not microscopic polyangiitis (MPA) yet. In this study, we investigated the association between MPV and Birmingham vasculitis activity score (BVAS) in 88 patients with MPA. We retrospectively reviewed the medical records of 88 patients with MPA. Clinical and laboratory data at diagnosis and partially at follow-up were collected. Linear regression analyses were used to clarify the association between MPV and BVAS...
March 19, 2018: Rheumatology International
Risa Inagawa, Hideshi Okada, Genzou Takemura, Kodai Suzuki, Chihiro Takada, Hirohisa Yano, Yoshiaki Ando, Takahiro Usui, Yasuaki Hotta, Nagisa Miyazaki, Akiko Tsujimoto, Ryogen Zaikokuji, Atsumu Matsumoto, Tomonori Kawaguchi, Tomoaki Doi, Takahiro Yoshida, Shozo Yoshida, Keisuke Kumada, Hiroaki Ushikoshi, Izumi Toyoda, Shinji Ogura
BACKGROUND: The most recent diagnostic criteria for sepsis includes organ failure. Microvascular endothelial injury is thought to lead to the multiple organ failure seen in sepsis, although the precise mechanism is still controversial. Acute respiratory distress syndrome (ARDS) is the primary complication during the sequential development of multiple organ dysfunction in sepsis, and endothelial injury is deeply involved. Sugar-protein glycocalyx coats all healthy vascular endothelium, and its disruption is one factor thought to contribute to microvascular endothelial dysfunction during sepsis...
March 16, 2018: Chest
Francisco Galeano-Valle, Pablo Demelo-Rodriguez, Luís Álvarez-Sala-Walther, Blanca Pinilla-Llorente, Miguel Jesús Echenagusia-Boyra, Hugo Rodriguez-Abella, Jorge Del-Toro-Cervera
Behçet's Disease (BD) is a rare multi-systemic inflammatory disorder classified as a systemic vasculitis of unknown aetiology. Vascular involvement occurs in approximately 5-51.6% cases, affecting venous and arterial vessels. Cardiac involvement is rare in BD (6%). There have been published approximately 93 cases of BD associated with intracardiac thrombosis, with different treatments and courses. We present a case of a 35-year-old spanish male that, after a percutaneous pharmacomechanical thrombectomy with venous stent placement, developed high fever and extensive venous thrombosis despite anticoagulation including intracardiac thrombosis (ICT) in the right ventricle and pulmonary embolism that leaded to the diagnosis of BD...
February 2018: Intractable & Rare Diseases Research
Karla Arévalo Ruales, Rosa Negueroles Albuixech, José Loaiza Gongora, Elena Grau García, José Ivorra Cortés, José A Román Ivorra
OBJECTIVE: Polymyalgia rheumatica (PR) can be associated with large vessel vasculitis (LVV). We evaluate the diagnostic role of18 F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) and its impact on the treatment of LVV associated with PR. MATERIALS AND METHODS: Retrospective study of patients diagnosed with PR. Data was collected from health records. Blood analysis included acute-phase reactants (APR), C-reactive protein (CRP) and erythrocyte sedimentation rate...
March 14, 2018: Reumatología Clinica
Chi-Hone Lien, Ming-Dar Lee
No abstract text is available yet for this article.
March 15, 2018: Journal of Pediatrics
Luca Navarini, Domenico Paolo Emanuele Margiotta, Marta Vadacca, Antonella Afeltra
In the last two decades, white adipose tissue (WAT) has been recognized as a key actor of many physiological and pathological conditions. WAT is able to produce mediators, named "adipokines", which may affect systemic homeostasis. In particular, leptin is not only involved in appetite and energy metabolism, but also in immune system. Increasing evidence established that leptin can regulate both innate and adaptive immunity mainly with pro-inflammatory effects but also, to a lesser extent, with anti-inflammatory features...
March 13, 2018: Cancer Letters
Kenji Yorita, Kazuya Tsuji, Yoko Takano, Naoto Kuroda, Kei Sakamoto, Kaoru Arii, Yukio Yoshimoto, Kimiko Nakatani, Satoshi Ito
BACKGROUND: Acantholytic squamous cell carcinoma (ASQCC), histologically characterized by intercellular bridge loosening, is recognized as a rare variant of squamous cell carcinoma (SQCC). ASQCC may demonstrate a worse prognosis than conventional SQCC. Pulmonary ASQCC is particularly rare; its biological behavior and prognostic data have not been reported. CASE PRESENTATION: We report the clinical and autopsy findings of a 71-year-old Japanese man with pulmonary ASQCC...
March 16, 2018: BMC Cancer
Mitsuhiro Akiyama, Tsutomu Takeuchi
IgG4-related disease is a heterogeneous immune-mediated fibroinflammatory condition that can affect every single organ. This disease is more prevalent in the elderly (the mean age of patients is above 60 years) and the prevalence rate is estimated to be over 4.6 per 100,000 population. Before making a diagnosis, the exclusion of malignancies, lymphoma, anti-neutrophil cytoplasmic antibody-associated vasculitis, multicentric Castleman disease, and other mimickers is crucial for appropriate treatment. Broad management guidelines have been published emphasizing the need for prompt treatment and the use of glucocorticoids as first-line drug therapy for induction of remission...
March 15, 2018: Drugs & Aging
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