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Adult onset stills

Anthony J Ocon, Alysia V Kwiatkowski, Ruben Peredo-Wende, Richard Blinkhorn
A 61-year-old Caucasian man presented with a fever of unknown origin, a transient erythematous rash on his right upper extremity and chest pressure after being treated for erythema migrans (Lyme disease). Echocardiogram demonstrated a large pericardial effusion with tamponade. He underwent pericardiostomy with tube placement. Workup for infectious and malignant etiologies was negative. Histology of the pericardium showed acute on chronic fibrinous haemorrhagic pericarditis. The patient met criteria for adult-onset Still's disease...
August 16, 2018: BMJ Case Reports
Syunsuke Yamanaka, Erik D Skarsgard, Ran D Goldman
Question A 10-year-old girl who was seen in my office last week with acute-onset abdominal pain and fever was referred to an emergency department, was diagnosed with appendicitis, and was treated conservatively with antibiotics, without surgery. Has the paradigm for treating appendicitis changed, and which is the preferred treatment of appendicitis in children: antibiotics or appendectomy? Answer For more than 100 years, surgical management was the principal treatment of acute appendicitis. Potential adverse events associated with appendectomy include bleeding, surgical site infection, and ileus, as well as stress for children and their parents...
August 2018: Canadian Family Physician Médecin de Famille Canadien
Rômulo Ribeiro do Vale, Nathalia da Silva Conci, Alexandre Pinheiro Santana, Mauricio Baptista Pereira, Natália Yume Hissayasu Menezes, Vilma Takayasu, Lorena Silva Laborda, Aloísio Souza Felipe da Silva
Celiac disease (CD)-also known as gluten-sensitive enteropathy-is a chronic, genetically predisposing and autoimmune entity with a wide range of clinical manifestations triggered by gluten ingestion, which affects 1% of the general population. Currently, up to 60% of the diagnosis of CD is in adults due to the atypical course of the disease. The severe acute onset of CD-also called celiac crisis-is very uncommon and is still not well documented in adults. We report the case of a 58-year-old man who presented a 45-day history of subtle-onset diarrhea followed by malabsorption syndrome with progressive weight loss, anasarca, and electrolyte disturbances...
July 2018: Autopsy & Case Reports
Ayse Deniz Elmali, Ruken Simsekoglu, Erdi Sahin, Canan Duman Ilki, Ozge Uygun, Oguzhan Coban, Candan Gurses
Subacute sclerosing panencephalitis (SSPE) is a well-known childhood disease; however, the adult onset of SSPE cases are also widely recognized where the oldest case reported is 52 years old. We report a 61-year-old woman patient presenting with atypical clinical and EEG features, diagnosed with SSPE. Measles and SSPE have decreased dramatically owing to worldwide immunization programs; however, there are still reasons to consider SSPE in differential diagnosis even in patients presenting with atypical clinical findings and older ages...
August 13, 2018: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
Roberto Giacomelli, Piero Ruscitti, Yehuda Shoenfeld
Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology usually affecting young adults; spiking fever, arthritis and evanescent rash are commonly observed during the disease. Other frequently observed clinical features include sore throat, hepatomegaly, splenomegaly, lymphadenopathy and serositis. Furthermore, AOSD patients may experience different life-threating complications. Macrophage activation syndrome (MAS) has been reported up to 15% of AOSD patients and it is considered to be the most severe complication of the disease being characterised by high mortality rate...
August 1, 2018: Journal of Autoimmunity
Manuela de Oliveira Fragomeni, Denis Bernardi Bichuetti, Enedina Maria Lobato Oliveira
BACKGROUND: Multiple Sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are acquired demyelinating syndromes of the central nervous system more frequently in young adults and their beginning before 18 years of age is rare. They are autoimmune diseases with distinct pathophysiology, clinical presentation, treatment and prognoses. During childhood these conditions often present similar clinical features and differential diagnosis among pNMOSD, pMS and acute disseminated encephalomyelitis (ADEM) is still difficult at disease onset...
July 20, 2018: Multiple Sclerosis and related Disorders
Mirna Chehade, Stacie M Jones, Robbie D Pesek, A Wesley Burks, Brian P Vickery, Robert A Wood, Donald Y M Leung, Glenn T Furuta, David M Fleischer, Alice K Henning, Peter Dawson, Robert W Lindblad, Scott H Sicherer, J Pablo Abonia, Joseph D Sherrill, Hugh A Sampson, Marc E Rothenberg
BACKGROUND: Eosinophilic esophagitis (EoE) is increasingly common, but data on phenotypic aspects are still incomplete. OBJECTIVES: To describe the clinical, endoscopic and histopathological features of a large number of children and adults with EoE across the US. METHODS: This was a multi-site single visit registry enrolling subjects aged 6 months-65 years with EoE. Participants provided responses regarding their medical history, with verification of the diagnosis and history by the study teams...
July 31, 2018: Journal of Allergy and Clinical Immunology in Practice
Nynke Oosterhof, Laura E Kuil, Herma C van der Linde, Saskia M Burm, Woutje Berdowski, Wilfred F J van Ijcken, John C van Swieten, Elly M Hol, Mark H G Verheijen, Tjakko J van Ham
Microglia are brain-resident macrophages with trophic and phagocytic functions. Dominant loss-of-function mutations in a key microglia regulator, colony-stimulating factor 1 receptor (CSF1R), cause adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP), a progressive white matter disorder. Because it remains unclear precisely how CSF1R mutations affect microglia, we generated an allelic series of csf1r mutants in zebrafish to identify csf1r-dependent microglia changes. We found that csf1r mutations led to aberrant microglia density and distribution and regional loss of microglia...
July 31, 2018: Cell Reports
J Scibilia, C M Rossi, L M Losappio, C Mirone, L Farioli, V Pravettoni, E A Pastorello
BACKGROUND AND OBJECTIVE: Wheat ingestion can lead to different disorders such as IgE-mediated food allergy and wheat-dependent exercise-induced anaphylaxis (WDEIA), associated with impaired quality of life and significant morbidity. Allergy to wheat is relatively benign in children, however its natural history in adults is still unknown. Objective: We evaluated the natural history of wheat hypersensitivity in atopic patients with adult-onset wheat- allergy assessed by placebo-controlled-challenge...
July 30, 2018: Journal of Investigational Allergology & Clinical Immunology
Ju-Yang Jung, Bunsoon Choi, Hasan Md Sayeed, Chang-Hee Suh, Ye Won Kim, Hyoun-Ah Kim, Seonghyang Sohn
We examined the expression of human leukocyte antigen (HLA) and composition of differentiated T cells in the peripheral blood to understand the characteristics of the immune changes in patients with adult-onset Still's disease (AOSD). This study enrolled patients with AOSD (n = 14), patients with rheumatoid arthritis (RA, n = 20), and healthy controls (HC, n = 20). The percentage of surface-stained cells with HLA-DP, DQ, and DR alleles and the composition of differentiated T cells in peripheral blood leukocytes (PBLs) were evaluated by flow cytometry...
January 2018: International Journal of Immunopathology and Pharmacology
Yoshifumi Tada, Satomi Inokuchi, Akihito Maruyama, Rie Suematsu, Mariko Sakai, Yuri Sadanaga, Nobuyuki Ono, Yojiro Arinobu, Syuichi Koarada
The objectives of this study are to determine whether the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classification criteria for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (SJIA) can be used to identify MAS in patients with adult-onset Still's disease (AOSD). Using laboratory data from 76 AOSD patients with and without MAS, we analyzed the ability of the collective and individual constitutive elements of the 2016 MAS in SJIA criteria and additional laboratory measures to discriminate between AOSD patients with (n = 16) and without (n = 60) MAS...
July 26, 2018: Rheumatology International
Mahdieh Fazel, Joseph F Merola, Drew J B Kurtzman
Systemic inflammatory disorders frequently involve the skin, and when cutaneous disease develops, such dermatologic manifestations may represent the initial sign of disease and may also provide valuable prognostic information about the underlying disorder. Familiarity with the various skin manifestations of systemic disease is therefore paramount and increases the likelihood of accurate diagnosis, which may facilitate the implementation of an appropriate treatment strategy. An improvement in quality of life and a reduction in the degree of morbidity may also be a realized benefit of accurate recognition of these skin signs...
July 2018: Clinics in Dermatology
Dylan Lawless, Shelly Pathak, Thomas Edward Scambler, Lylia Ouboussad, Rashida Anwar, Sinisa Savic
TNFAIP3 encodes the NF-κB regulatory protein A20. High-penetrance heterozygous mutations in TNFAIP3 cause a haploinsufficiency of A20 (HA20), inadequate inhibition of NF-κB pathway, and an early onset autoinflammatory disorder. However, the clinical phenotype of patients with HA20 varies greatly and clinical diagnoses prior to establishing the genetic cause, included both autoimmune and autoinflammatory conditions. Here, we present the first patient with HA20, who was previously diagnosed with AOSD but was later found to have a novel heterozygous variant in TNFAIP3 and who was successfully treated with anti-IL6 receptor biologic tocilizumab (RoActemra)...
2018: Frontiers in Immunology
Enikő Palkó, Szilárd Póliska, István Sziklai, András Penyige
Cholesteatoma is an epidermal cyst with still unknown pathomechanism. The aim of the current study was to investigate molecular differences in the background of the hyperproliferative property and aggressive behavior typical of the cholesteatoma epithelium. The expression of three cytokeratin genes (KRT1, KRT10 and KRT19), the matrix metalloproteinase 9 gene (MMP9) and the tumor suppressor TP53 gene was measured by qRT-PCR in surgical samples of pediatric and adult cholesteatoma cases and their expression level was compared to that of normal skin samples from the retroauricular region of control individuals...
2018: PloS One
Hussein Nassereddine, Charlotte Fite, Diane Kottler, Vincent Descamps, Anne Couvelard, Liliane Marot, Lydia Deschamps
No abstract text is available yet for this article.
July 11, 2018: Journal of Cutaneous Pathology
Karolina E Hilse, Anne Rupprecht, Monika Egerbacher, Sarah Bardakji, Lars Zimmermann, Andrea E M Seiler Wulczyn, Elena E Pohl
The involvement of mitochondrial uncoupling proteins 2 and 3 in the pathogenesis of cardiovascular diseases is widely acknowledged. However, contradictory reports show that the functions of UCP2/UCP3 are still disputed. We have previously described that UCP2 is highly abundant in cells that rely on glycolysis, such as stem, cancer and activated immune cells. In contrast, high amounts of UCP3 are present in brown adipose tissue, followed by heart and skeletal muscles - all known to metabolize fatty acids (FA) to a high extent...
2018: Frontiers in Physiology
Daisuke Usuda, Yoshiki Furumura, Kento Takeshima, Ryusho Sangen, Yasuhiro Kawai, Yuji Kasamaki, Yoshitsugu Iinuma, Tsugiyasu Kanda
BACKGROUND: Adult onset Still's disease is a systemic auto-inflammatory condition of unknown etiology characterized by intermittent spiking high fever, an evanescent salmon-pink or erythematous maculopapular skin rash, arthralgia or arthritis, and leukocytosis. Recently, a high level of interleukin-18 has been reported as a new characteristic marker. On the other hand no reports have been published on high interleukin-18 as a marker in older patients. We report a case of adult onset Still's disease in an older patient successfully treated with steroids in which interleukin-18 was a useful marker of disease activity...
July 10, 2018: Journal of Medical Case Reports
Bella Mehta, Shanthini Kasturi, Julie Teruya-Feldstein, Steven Horwitz, Anne R Bass, Doruk Erkan
No abstract text is available yet for this article.
July 2018: HSS Journal: the Musculoskeletal Journal of Hospital for Special Surgery
Karen O Moss, Carolyn H Still, Lenette M Jones, Gabrielle Blackshire, Kathy D Wright
African Americans are disproportionately impacted by hypertension, suffering earlier onset and greater severity of the disease, and premature cardiovascular mortality, than the general population. Managing hypertension is an unending concern for African American older adults. A better understanding of the self-management preferences of this vulnerable population will lead to the enhanced design of culturally acceptable interventions. This descriptive study examined focus group participants' perspectives on engaging in hypertension self-management activities with the goal of developing a co-created intervention...
June 1, 2018: Western Journal of Nursing Research
Giovanni Pomponio, Diletta Olivari, Massimo Mattioli, Alessia Angeletti, Giulia Rossetti, Gaia Goteri, Armando Gabrielli
RATIONALE: IgG4-related disease (IgG4-RD) is an emerging immune-mediated disease characterized by multi-organ involvement and variable clinical behavior. PATIENT CONCERNS: We describe the case of a 50-year-old woman affected by a rare variant of IgG4-RD, characterized by eyelid xanthelasmas, adult-onset asthma and salivary and lacrimal glands enlargement. Multiple lymphadenopathies and a pulmonary mass were present at initial evaluation. INTEVENTIONS: After a single course of rituximab (2g in 2 refracted doses), an almost complete clinical remission was achieved without chronic steroid administration...
June 2018: Medicine (Baltimore)
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