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https://www.readbyqxmd.com/read/28731669/-langerhans-cell-histiocytosis-with-vertebral-involvement-and-soft-tissue-extension-clinical-case
#1
Tai C Luong, Adriana Scrigni, Marcela Paglia, Mariano Garavaglia, Nuria Aisenberg, Hernán Rowensztein, Claudia Sampor
Langerhans cell histiocytosis is a heterogeneous disease of unknown etiology characterized by proliferation of Langerhans cells. It is a rare disease. Bone involvement is common but vertebral disease is rare. We present a 4 year old patient with abdominal pain and neurologic symptoms. Magnetic resonance showed vertebra plana in D9 with involvement of paravertebral soft tissues. The child underwent surgery for decompression and biopsy. Biopsy confirmed Langerhans cell histiocytosis. She was treated with vinblastine and prednisone during 6 months following LCH-III with complete recovery of neurologic symptoms...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28724596/severe-destructive-nasopharyngeal-granulomatosis-with-polyangiitis-with-superimposed-skull-base-pseudomonas-aeruginosa-osteomyelitis
#2
Mitchell S von Itzstein, Jithma P Abeykoon, Daniel D Summerfield, Jennifer A Whitaker
Skull base osteomyelitis in the setting of granulomatosis with polyangiitis (GPA) is rare and entails significant diagnostic challenges. We present a case of a 65-year-old Caucasian man with a history of rheumatoid arthritis, off immunosuppression for 18 months, who presented with 2 years of chronic headaches, severe fatigue, saddle nose deformity and 20-kilogram unintentional weight loss. Maxillofacial CT revealed an extensive destructive sinonasal and erosive skull base process. Laboratory evaluation showed equivocal elevation of antiproteinase 3 antibodies with negative antineutrophil cytoplasmic antibody panel...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28717763/burkitt-s-lymphoma-and-b-cell-lymphoma-unclassifiable-with-features-intermediate-between-diffuse-large-b-cell-lymphoma-and-burkitt-s-lymphoma-in-patients-with-hiv-outcomes-in-a-south-african-public-hospital
#3
Gerhard Sissolak, Matthew Seftel, Thomas S Uldrick, Tonya M Esterhuizen, Nooroudien Mohamed, Danie Kotze
PURPOSE: Burkitt's lymphoma (BL) is a common HIV-associated lymphoma in South Africa. B-cell lymphoma unclassifiable with features intermediate between diffuse large B-cell lymphoma and Burkitt's lymphoma (BL/DLBCL) also occurs in HIV infection. Outcomes of HIV-infected patients with BL or BL/DLBCL in a resource-constrained setting are not defined. METHODS: We performed a retrospective study of HIV-positive patients with BL or BL/DLBCL treated from 2004 to 2012 with curative intent at a publically funded academic medical center in South Africa...
June 2017: Journal of Global Oncology
https://www.readbyqxmd.com/read/28691528/ribbing-disease-a-systematic-review
#4
Bart G Pijls, Koen Steentjes, Jan W Schoones, Sander Pd Dijkstra
Background Ribbing disease, or multiple diaphyseal sclerosis, is a rare benign bone dysplasia. Purpose To systematically review the literature to determine the clinical and radiological presentation of patients with Ribbing disease as well as the effects of attempted treatments. Material and Methods We considered individual patient data of patients diagnosed with Ribbing disease derived from patient reports and patient series. All stages of the review were performed by two reviewers independently. Standard descriptive statistics were used for quantitative analyses and mixed model analyses were used when appropriate Results The literature search yielded 420 unique hits of which 23 studies were included, covering a total of 40 patients of whom 29 had bilateral involvement...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28690390/integrated-care-of-muscular-dystrophies-in-italy-part-1-pharmacological-treatment-and-rehabilitative-interventions
#5
Luisa Politano, Marianna Scutifero, Melania Patalano, Alessandra Sagliocchi, Antonella Zaccaro, Federica Civati, Erika Brighina, Gianluca Vita, Sonia Messina, Maria Sframeli, Maria Elena Lombardo, Roberta Scalise, Giulia Colia, Maria Catteruccia, Angela Berardinelli, Maria Chiara Motta, Alessandra Gaiani, Claudio Semplicini, Luca Bello, Guja Astrea, Giulia Ricci, Maria Grazia D'Angelo, Giuseppe Vita, Marika Pane, Adele D'Amico, Umberto Balottin, Corrado Angelini, Roberta Battini, Lorenza Magliano
This paper describes the pharmacological therapies and rehabilitative interventions received by 502 patients with Muscular Dystrophies, evaluated in relation to patient's socio-demographic and clinical variables, and geographical areas. Data were collected by the MD-Socio-Demographic and Clinical Schedule (MD-SC-CS) and by the Family Problems Questionnaire (FPQ). The most part of the enrolled patients were in drug treatment. The number of the medications increased in relation to patient's age, disability degree and duration of illness and was higher among patients with Duchenne Muscular Dystrophy (DMD) compared with Becker (BMD) or Limb-Girdle Muscular Dystrophies (LGMD)...
March 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/28638581/images-in-clinical-medicine-tophi
#6
Amr Salama, Richard Alweis
Tophi (plural of tophus, Latin for "stone") are stone-like deposits of monosodium urate in the soft tissues, synovial tissues, or in bones near the joints. They are pathognomonic for gout, the most common inflammatory arthritis in the United States, with an estimated lifetime prevalence of 4%. It is usually the end result of loss of the balance between uric acid production and excretion. It can be found anywhere in the body especially in areas of friction or trauma. It is usually painless and rarely to present as the initial manifestation of gout...
March 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28579851/transformation-of-follicular-lymphoma-to-a-high-grade-b-cell-lymphoma-with-myc-and-bcl2-translocations-and-overlapping-features-of-burkitt-lymphoma-and-acute-lymphoblastic-leukemia-a-case-report-and-literature-review
#7
Alina M Bischin, Russell Dorer, David M Aboulafia
Most commonly, histologic transformation (HT) from follicular lymphoma (FL) manifests as a diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS). Less frequently, HT may result in a high-grade B-cell lymphoma (HGBL) with MYC and B-cell lymphoma protein 2 (BCL2) and/or BCL6 gene rearrangements, also known as "double-hit" or "triple-hit" lymphomas. In the 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms, the category B-cell lymphoma, unclassifiable was eliminated due to its vague criteria and limiting diagnostic benefit...
2017: Clinical Medicine Insights. Blood Disorders
https://www.readbyqxmd.com/read/28579833/multiple-myeloma-in-niger-delta-nigeria-complications-and-the-outcome-of-palliative-interventions
#8
Ogbonna Collins Nwabuko, Elizabeth Eneikido Igbigbi, Innocent Ijezie Chukwuonye, Martin Anazodo Nnoli
BACKGROUND: Multiple myeloma (MM) is one of the hematological malignancies that require palliative care. This is because of the life-threatening nature and the suffering associated with the illness. The aim of this study is to bring to the fore the complications experienced by people living with MM in the Niger-Delta region of Nigeria and the outcome of various palliative interventions. METHODS: This was a 10-year multi-center retrospective study of 26 patients diagnosed and managed in three major centers in the Niger-Delta region of Nigeria from January 2003 to December 2012...
2017: Cancer Management and Research
https://www.readbyqxmd.com/read/28579151/validation-of-the-association-of-recist-changes-with-survival-in-men-with-metastatic-castration-resistant-prostate-cancer-treated-on-swog-study-s0421
#9
Guru Sonpavde, Gregory R Pond, Melissa Plets, Catherine M Tangen, Maha H A Hussain, Primo N Lara, Amir Goldkorn, Mark G Garzotto, Philip C Mack, Celestia S Higano, Nicholas J Vogelzang, Ian M Thompson, Przemyslaw W Twardowski, Peter J Van Veldhuizen, Neeraj Agarwal, Michael A Carducci, J Paul Monk, David I Quinn
BACKGROUND: Phase 2 trials evaluating new agents for metastatic castration-resistant prostate cancer (mCRPC) have relied on bone scan and prostate-specific antigen changes to assess activity. Given the increasing detection of measurable disease, Response Evaluation Criteria in Solid Tumors (RECIST) changes warrant consideration to evaluate activity. We validated the association of RECIST 1.0 changes with survival in men with mCRPC receiving docetaxel. PATIENTS AND METHODS: Data for men with measurable disease from the Southwest Oncology Group (SWOG) S0421, a phase 3 trial in men with mCRPC receiving docetaxel and prednisone plus placebo or atrasentan, were used...
May 10, 2017: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/28578002/mir-124-contributes-to-glucocorticoid-resistance-in-acute-lymphoblastic-leukemia-by-promoting-proliferation-inhibiting-apoptosis-and-targeting-the-glucocorticoid-receptor
#10
Yan-Ni Liang, Yan-Lai Tang, Zhi-Yong Ke, Yue-Qin Chen, Xue-Qun Luo, Hua Zhang, Li-Bin Huang
Acute lymphoblastic leukemia (ALL) is characterized by the accumulation of abnormal lymphoblasts in the bone marrow and blood. Though great progress has been made for improvement in clinical treatment during the past decades, some children with ALL still relapsed. Glucocorticoid (GC) resistance is an important clinical problem for ALL treatment failure. Therefore, further understanding of the mechanism of GC resistance and exploring novel therapeutic strategies are crucial for improving treatment outcome. The reported involvement of microRNAs (miRNAs) in drug resistance implied that deregulated miRNA expression might contribute to GC treatment response of ALL...
May 31, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28566677/high-grade-transformation-in-a-splenic-marginal-zone-lymphoma-with-a-cerebral-manifestation
#11
Xiaoning Gao, Jie Li, Ji Lin, Daihong Liu, Li Yu, Quanshun Wang
BACKGROUND Splenic marginal zone lymphomas (SMZLs) are generally uncommon, indolent lymphomas that typically affect older adults, but the development of the transformation to high-grade lymphoma may occur in a small proportion of patients and represents a rare event with blastic cell infiltration in the lymph nodes and bone marrow. CASE REPORT Here, we present a young adult patient who was diagnosed with a SMZL and developed a high-grade transformation to diffuse large B cell lymphoma (DLBCL) with central nervous system involvement...
June 1, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28559645/comorbidity-between-htlv-1-associated-adult-t-cell-lymphoma-leukemia-and-verrucous-carcinoma-a-case-report
#12
Miller Valencia, Luis Moreno
BACKGROUND: Adult T-cell Leukemia/Lymphoma (ATLL) is classified as a peripheral CD4+ T-cell neoplasm caused by the human T-cell lymphotropic virus type 1 (HTLV-1). Typical symptoms are associated with leukemic infiltration; however, atypical and exaggerated manifestations of verrucous carcinoma have also been described. CASE REPORT: We present here the case of a patient with multiple skin lesions, ischemic necrosis in the hallux and lymphadenopathies. Biopsies were taken, which showed verrucous epidermal carcinoma and cutaneous lymphoma...
March 30, 2017: Colombia Médica: CM
https://www.readbyqxmd.com/read/28556506/dissociated-agonist-of-glucocorticoid-receptor-or-prednisone-for-active-rheumatoid-arthritis-effects-on-p1np-and-osteocalcin-pharmacodynamics
#13
S Shoji, A Suzuki, D J Conrado, M C Peterson, J Hey-Hadavi, D McCabe, R Rojo, B K Tammara
Fosdagrocorat (PF-04171327), a dissociated agonist of the glucocorticoid receptor, has potent anti-inflammatory activity in patients with rheumatoid arthritis with reduced adverse effects on bone health. To identify fosdagrocorat doses with bone formation marker changes similar to prednisone 5 mg, we characterized treatment-related changes in amino-terminal propeptide of type I collagen (P1NP) and osteocalcin (OC) with fosdagrocorat (1, 5, 10, or 15 mg) and prednisone (5 or 10 mg) in a phase II randomized trial (N = 323)...
May 27, 2017: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/28549731/clinical-and-sonographic-biomarkers-of-structural-damage-progression-in-ra-patients-in-clinical-remission-a-prospective-study-with-12-months-follow-up
#14
Julio Ramírez, José Antonio Narváez, Virginia Ruiz-Esquide, Javier Hernández-Gañán, Andrea Cuervo, José Inciarte-Mundo, Maria Victoria Hernández, Miguel Sampayo-Cordero, José Luis Pablos, Raimon Sanmartí, Juan D Cañete
OBJECTIVE: To determine clinical and sonographic biomarkers predicting structural damage progression at 12 months of follow-up as measured by magnetic resonance imaging (MRI) in rheumatoid arthritis (RA) patients in clinical remission. PATIENTS AND METHODS: We included patients with RA in clinical remission, defined as 28-joint disease activity score (DAS28)-erythrocyte sedimentation rate (ESR) <2.6 for >6 months. Ultrasound scans of both hands and knees and MRI of the dominant hand were performed at baseline and at 12 months...
May 1, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28535619/prevalence-and-incidence-of-osteoporotic-fractures-in-patients-on-long-term-glucocorticoid-treatment-for-rheumatic-diseases-the-glucocorticoid-induced-osteoporosis-tool-giotto-study
#15
M Rossini, O Viapiana, M Vitiello, N Malavolta, G La Montagna, S Maddali Bongi, O Di Munno, R Nuti, C U Manzini, C Ferri, L Bogliolo, A Mathieu, F Cantatore, A Del Puente, M Muratore, W Grassi, B Frediani, G Saviola, P Delvino, L Mirone, G Ferraccioli, G Tripi, I Piazza, D Gatti
Osteoporosis and fractures are common and invalidating consequences of chronic glucorticoid (GC) treatment. Reliable information regarding the epidemiology of GC induced osteoporosis (GIOP) comes exclusively from the placebo group of randomized clinical trials while observational studies are generally lacking data on the real prevalence of vertebral fractures, GC dosage and primary diagnosis. The objective of this study was to evaluate the prevalence and incidence of osteoporotic fractures and to identify their major determinants (primary disease, GC dosage, bone mineral density, risk factors, specific treatment for GIOP) in a large cohort of consecutive patients aged >21 years, on chronic treatment with GC (≥5 mg prednisone - PN - equivalent) and attending rheumatology centers located all over Italy...
May 22, 2017: Reumatismo
https://www.readbyqxmd.com/read/28427101/tanshinol-alleviates-osteoporosis-and-myopathy-in-glucocorticoid-treated-rats
#16
Guanghua Chen, Xinle Zhang, Han Lin, Guizhi Huang, Yahui Chen, Liao Cui
Tanshinol is a major water-soluble active component of Salvia miltiorrhiza. In this study, we aimed to investigate whether tanshinol has potential therapeutic effects against glucocorticoid-induced osteoporosis and glucocorticoid-induced myopathy. Ninety-six female Sprague-Dawley rats were randomly assigned to five groups: a control group, a model group, and three model groups treated with 25 or 50 mg/kg of tanshinol, or calcitriol. All model groups received prednisone acetate for 90 days to induce glucocorticoid-induced osteoporosis...
April 20, 2017: Planta Medica
https://www.readbyqxmd.com/read/28421240/a-randomised-phase-ii-study-of-repeated-rhenium-188-hedp-combined-with-docetaxel-and-prednisone-versus-docetaxel-and-prednisone-alone-in-castration-resistant-prostate-cancer-crpc-metastatic-to-bone-the-taxium-ii-trial
#17
Joyce M van Dodewaard-de Jong, John M H de Klerk, Haiko J Bloemendal, Daniela E Oprea-Lager, Otto S Hoekstra, H Pieter van den Berg, Maartje Los, Aart Beeker, Marianne A Jonker, Joe M O'Sullivan, Henk M W Verheul, Alfons J M van den Eertwegh
BACKGROUND: Rhenium-188-HEDP is a beta-emitting radiopharmaceutical used for palliation of metastatic bone pain. We investigated whether the addition of rhenium-188-HEDP to docetaxel/prednisone improved efficacy of chemotherapy in patients with CRPC. METHODS: Patients with progressive CRPC and osteoblastic bone metastases were randomised for first-line docetaxel 75 mg/m(2) 3-weekly plus prednisone with or without 2 injections of rhenium-188-HEDP after the third (40 MBq/kg) and after the sixth (20 MBq/kg) cycle of docetaxel...
April 18, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28414669/a-case-of-hepatosplentic-t-cell-lymphoma-a-rare-aggressive-tumor-of-the-young
#18
S Cingam, S Patel, N Koshy
INTRODUCTION: Hepatosplenic T-cell lymphoma (HSTCL); is an unusual entity first described in 1990 that predominantly affects middle-aged men and is classified by WHO under peripheral T-cell lymphomas. We present a 26-year-old man with HSCTL treated with a non-CHOP regimen. CASE: A 26 year old immigrant from Cameroon without significant past medical history presented with abdominal discomfort that was first noted 1 month prior at which time he was elbowed in abdomen during a basketball game...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28342637/first-line-rituximab-combined-with-short-term-prednisone-versus-prednisone-alone-for-the-treatment-of-pemphigus-ritux-3-a-prospective-multicentre-parallel-group-open-label-randomised-trial
#19
Pascal Joly, Maud Maho-Vaillant, Catherine Prost-Squarcioni, Vivien Hebert, Estelle Houivet, Sébastien Calbo, Frédérique Caillot, Marie Laure Golinski, Bruno Labeille, Catherine Picard-Dahan, Carle Paul, Marie-Aleth Richard, Jean David Bouaziz, Sophie Duvert-Lehembre, Philippe Bernard, Frederic Caux, Marina Alexandre, Saskia Ingen-Housz-Oro, Pierre Vabres, Emmanuel Delaporte, Gaelle Quereux, Alain Dupuy, Sebastien Debarbieux, Martine Avenel-Audran, Michel D'Incan, Christophe Bedane, Nathalie Bénéton, Denis Jullien, Nicolas Dupin, Laurent Misery, Laurent Machet, Marie Beylot-Barry, Olivier Dereure, Bruno Sassolas, Thomas Vermeulin, Jacques Benichou, Philippe Musette
BACKGROUND: High doses of corticosteroids are considered the standard treatment for pemphigus. Because long-term corticosteroid treatment can cause severe and even life-threatening side-effects in patients with this disease, we assessed whether first-line use of rituximab as adjuvant therapy could improve the proportion of patients achieving complete remission off-therapy, compared with corticosteroid treatment alone, while decreasing treatment side-effects of corticosteroids. METHODS: We did a prospective, multicentre, parallel-group, open-label, randomised trial in 25 dermatology hospital departments in France (Ritux 3)...
March 22, 2017: Lancet
https://www.readbyqxmd.com/read/28272212/ophthalmic-manifestations-in-igg4-related-disease-clinical-presentation-and-response-to-treatment-in-a-french-case-series
#20
MULTICENTER STUDY
Mikael Ebbo, Matthieu Patient, Aurelie Grados, Matthieu Groh, Julien Desblaches, Eric Hachulla, David Saadoun, Sylvain Audia, Aude Rigolet, Benjamin Terrier, Antoinette Perlat, Constance Guillaud, Frederic Renou, Emmanuelle Bernit, Nathalie Costedoat-Chalumeau, Jean-Robert Harlé, Nicolas Schleinitz
IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling "modified" comprehensive diagnostic criteria with pathological documentation were retained for the study...
March 2017: Medicine (Baltimore)
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