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https://www.readbyqxmd.com/read/28299525/poems-syndrome-an-enigma
#1
REVIEW
Rahma Warsame, Uday Yanamandra, Prashant Kapoor
POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis...
March 15, 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28291506/immunotactoid-glomerulopathy-leading-to-the-discovery-of-poems-syndrome%C3%A2
#2
Carole Philipponnet, Jean-Louis Kemeny, Cyril Garrouste, Martin Soubrier, Anne-Elisabeth Heng
Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits...
March 14, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28247959/therapeutic-plasma-exchange-in-chronic-dysimmune-peripheral-neuropathies-a-10-year-retrospective-study
#3
Philippe Codron, Maud Cousin, Jean-François Subra, Vivien Pautot, Franck Letournel, Christophe Verny, Julien Cassereau
INTRODUCTION: Therapeutic plasma exchange (TPE) can be proposed in the treatment of chronic dysimmune peripheral neuropathies (CDPN). Actual guidelines are however based on few studies, and indications and protocols still remain to be clarified. We conducted a 10-year retrospective study in order to assess the effectiveness and tolerance of TPE in CDPN. METHODS: All patients treated for CDPN with TPE from October 2006 to March 2016 in the university hospital of Angers were included...
March 1, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28247771/poems-syndrome-and-idiopathic-portal-hypertension-a-possible-association
#4
Sara Campos, Cláudia Agostinho, Maria Augusta Cipriano
A 48-year old female patient was admitted to the emergency department with upper gastrointestinal bleeding. Endoscopy showed large esophageal varices that were treated with band ligation. She had been treated with cyclophosphamide, melphalan, lenalidomide and corticosteroids for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy or edema, M protein, skin changes). She had no other risk factors for chronic liver disease. Laboratory and radiological examinations could not confirm the etiology of portal hypertension...
March 1, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28243170/elbow-ulnar-neuropathy-treatment-by-anterior-transposition-of-the-ulnar-nerve
#5
Antonio Tufi Neder, Regina de Azevedo Alves, Arlindo Gomes Pardini, Marcelo Riberto, Milton Mazer
OBJECTIVES: Retrospective clinical evaluation of 31 patients who underwent ulnar nerve decompression at the elbow and subcutaneous anterior transposition. METHODS: From January 2000 to December 2013, 71 patients underwent subcutaneous anterior transposition of the ulnar nerve. Thirty-one patients returned for evaluation. The mean follow-up period was 60 months. Patients were evaluated for the degree of satisfaction after surgery, paresthesia, pain, Tinel sign, Froment test and sensitivity test by esthesiometer, muscle strength of the intrinsic muscles and deep flexor of the fifth digit, visual analogic pain scale (VAS) and were subjected to the QuickDash questionnaire...
July 2016: Acta Ortopedica Brasileira
https://www.readbyqxmd.com/read/28223738/peroral-endoscopic-myotomy-for-treatment-of-guillain-barre-syndrome-associated-achalasia-a-rare-case
#6
Seung Kak Shin, Kyoung Oh Kim, Eui Joo Kim, Su Young Kim, Jung Ho Kim, Yoon Jae Kim, Jun-Won Chung, Kwang An Kwon, Dong Kyun Park
Guillain-Barre syndrome (GBS)-associated achalasia is a very rare disease of uncertain cause. We report the case of a patient diagnosed with GBS-associated type I achalasia who was successfully treated with peroral endoscopic myotomy (POEM). A 30-year-old man who was diagnosed with GBS 3 mo before was referred to our department with dysphagia and meal-related regurgitation. The results of esophagography, endoscopy, and high-resolution manometry (HRM) revealed type I achalasia. POEM that utilized a submucosal tunneling technique was performed to treat the GBS-associated type I achalasia...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28196699/-sarcoidosis-flare-after-autologous-stem-cell-transplantation-an-immune-paradox
#7
A Marchal, F Charlotte, P Maksud, J Haroche, F Lifferman, M Miyara, S Choquet, Z Amoura, F Cohen Aubart
INTRODUCTION: Sarcoidosis is a systemic granulomatous disorder of unknown cause. Apparition or flare of previously diagnosed sarcoidosis following hematopoietic stem cell transplantation (HSCT) has rarely been reported. OBSERVATION: We report a 62-year-old woman who presented a radiological flare of sarcoidosis post-autologous stem cell transplantation for a POEMS syndrome. Imaging findings and lymph node histology, which revealed non-caseating granuloma, were consistent with the sarcoidosis diagnosis...
February 10, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28133484/polyneuropathy-as-novel-initial-manifestation-in-a-case-of-nonsecretory-poems-syndrome-with-sj%C3%A3-gren-s-syndrome
#8
Minrui Liang, Zhixing Jiang, Zhiguang Lin, Bobin Chen, Hejian Zou, Weiguo Wan, Jun Liu
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a paraneoplastic syndrome driven by plasma cell dyscrasias. We report a patient with novel initial manifestation of polyneuropathy, which was considered due to Sjögren's syndrome but with poor response to methylprednisolone (120 mg/d) and intravenous immunoglobulin (IVIg). Further investigation by imaging tests and following biopsy eventually confirmed the diagnosis of POEMS syndrome secondary to solitary plasmocytoma...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28100909/impact-of-pretransplant-induction-therapy-on-autologous-stem-cell-transplantation-for-patients-with-newly-diagnosed-poems-syndrome
#9
J Li, M-H Duan, C Wang, X-F Huang, W Zhang, X-X Cao, T-N Zhu, J-L Zhuang, B Han, H Cai, H-C Cai, D-B Zhou
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a rare plasma cell dyscrasia sometimes treated with a haematopoietic cell autotransplant. We analyzed data from 138 subjects with newly diagnosed POEMS syndrome receiving a autotransplant at our center. Thirty-two subjects with severe end-organ dysfunction ineligible for immediate autotransplant received pretransplant therapy, which made a subsequent autotransplant feasible. Pretransplant therapy resulted in vascular endothelial growth factor (VEGF) remissions in 15 (47%)...
February 14, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28031090/treatment-of-breast-cancer-accompanied-by-poems-syndrome-a-case-report
#10
Jian-Yu Dong, Yan Yan, Min-Feng Liu, Zhao-Ze Guo, Jing-Yun Guo, Chang-Sheng Ye
No abstract text is available yet for this article.
March 20, 2016: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/28003622/cranial-pachymeningeal-involvement-in-poems-syndrome-evaluation-by-pre-and-post-contrast-flair-and-t1-weighted-imaging
#11
Fumiaki Ueda, Miho Okuda, Hiroyuki Aburano, Yuichi Yoshie, Osamu Matsui, Toshifumi Gabata
PURPOSE: To evaluate the cranial pachymeningeal involvement of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome using pre- and post-contrast fluid-attenuated inversion recovery (FLAIR) and T1-weighted imaging (T1WI). METHODS: The appearance of pachymeningeal involvement in nine cases of POEMS syndrome was evaluated using pre- and post-contrast FLAIR and T1WI. The degree of pachymeningeal thickening was graded as normal or abnormal using pre-contrast FLAIR...
December 22, 2016: Magnetic Resonance in Medical Sciences: MRMS
https://www.readbyqxmd.com/read/27998645/thrombotic-microangiopathy-associated-with-monoclonal-gammopathy
#12
Aishwarya Ravindran, Ronald S Go, Fernando C Fervenza, Sanjeev Sethi
Thrombotic microangiopathy (TMA) is a rare disease comprising of a diverse set of disorders linked by a common histologic finding of endothelial injury. Monoclonal immunoglobulins may act as a potential trigger in the pathogenesis of TMA. To determine the prevalence of monoclonal gammopathy and clinicopathological features of TMA associated with monoclonal immunoglobulin, we performed a retrospective study in adults (18 and older) with a clinical diagnosis of TMA. Of 146 patients with TMA, we detected monoclonal immunoglobulin in 20 patients (13...
December 17, 2016: Kidney International
https://www.readbyqxmd.com/read/27930460/ocular-manifestations-and-treatment-outcomes-in-chinese-patients-with-poems-syndrome
#13
Xiao Zhang, Qian-Qian Cai, Xu-Fei Huang, Xin-Xin Cao, Hao Cai, Dao-Bin Zhou, Rong-Ping Dai, Jian Li
PURPOSE: To evaluate the relationship of serum vascular endothelial growth factor (VEGF) levels and ocular manifestations in Chinese patients with POEMS syndrome. METHODS: This is a prospective study. Forty-one treatment-naive patients were enrolled from April 2014 to November 2014. Among the 41 patients, 40 had complete ocular examination, spectral domain optical coherence tomography scan, and serum VEGF measurement before treatment and every 3-month interval after lenalidomide and dexamethasone treatment...
December 7, 2016: Retina
https://www.readbyqxmd.com/read/27866728/what-is-new-in-2015-in-dysimmune-neuropathies
#14
REVIEW
T Kuntzer
This review discusses and summarizes the concept of nodopathies, the diagnostic features, investigations, pathophysiology, and treatment options of chronic inflammatory demyelinating polyradiculoneuropathy, and gives updates on other inflammatory and dysimmune neuropathies such as Guillain-Barré syndrome, sensory neuronopathies, small-fiber-predominant ganglionitis, POEMS syndrome, neuropathies associated with IgM monoclonal gammopathy and multifocal motor neuropathy. This field of research has contributed to the antigenic characterization of the peripheral motor and sensory functional systems, as well as helping to define immune neuropathic syndromes with widely different clinical presentation, prognosis and response to therapy...
December 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27834942/measurement-of-%C3%AE-isomerized-c-terminal-telopeptide-of-type-i-collagen-in-patients-with-poems-syndrome-diagnostic-prognostic-and-follow-up-utilities
#15
X Huang, C Zhang, C Wang, Q Cai, X Cao, H Cai, L Zhang, J Feng, D Zhou, J Li
No abstract text is available yet for this article.
November 11, 2016: Blood Cancer Journal
https://www.readbyqxmd.com/read/27822103/the-danish-national-multiple-myeloma-registry
#16
REVIEW
Peter Gimsing, Morten O Holmström, Tobias Wirenfelt Klausen, Niels Frost Andersen, Henrik Gregersen, Robert Schou Pedersen, Torben Plesner, Per Trøllund Pedersen, Mikael Frederiksen, Ulf Frølund, Carsten Helleberg, Annette Vangsted, Peter de Nully Brown, Niels Abildgaard
AIM: The Danish National Multiple Myeloma Registry (DMMR) is a population-based clinical quality database established in January 2005. The primary aim of the database is to ensure that diagnosis and treatment of plasma cell dyscrasia are of uniform quality throughout the country. Another aim is to support research. Patients are registered with their unique Danish personal identification number, and the combined use of DMMR, other Danish National registries, and the Danish National Cancer Biobank offers a unique platform for population-based translational research...
2016: Clinical Epidemiology
https://www.readbyqxmd.com/read/27807187/clinical-spectrum-of-castleman-disease-associated-neuropathy
#17
Elie Naddaf, Angela Dispenzieri, Jay Mandrekar, Michelle L Mauermann
OBJECTIVE: To define the peripheral neuropathy phenotypes associated with Castleman disease. METHODS: We conducted a retrospective chart review for patients with biopsy-proven Castleman disease evaluated between January 2003 and December 2014. Patients with associated peripheral neuropathy were identified and divided into 2 groups: those with Castleman disease without POEMS syndrome (CD-PN) and those with Castleman disease with POEMS syndrome (CD-POEMS). We used a cohort of patients with POEMS as controls...
December 6, 2016: Neurology
https://www.readbyqxmd.com/read/27799073/nerve-pathologic-features-differentiate-poems-syndrome-from-cidp
#18
Ezequiel A Piccione, Janean Engelstad, Peter J Dyck, Michelle L Mauermann, Angela Dispenzieri, P James B Dyck
The objective of this study is to determine if the nerve pathology in patients with POEMS syndrome is different from CIDP. We hypothesized that nerve biopsies from patients with POEMS syndrome would have more small vessels and axonal degeneration but less inflammation than CIDP.We performed a retrospective analysis of nerve biopsies performed on "classic" CIDP and POEMS cases. Nerve biopsies were blinded and reviewed by two of the authors (EAP, PJBD). Teased fibers, paraffin-embedded sections, semithin sections and immunostains were analyzed...
October 31, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27790622/an-occult-malignancy-behind-a-demyelinating-disease-poems-syndrome
#19
Saberio Lo Presti, Prashanth Kanagarajah, Daniela Pirela, Diana Morlote, Mike Cusnir
We report a case of a 38-year-old man presenting with bilateral lower extremity weakness and paresthesias that progressed during a 4-month period to severe polyneuropathy forcing the patient to be bed bound. Throughout his multiple hospitalizations, he was treated erroneously for chronic inflammatory demyelinating polyneuropathy, without significant improvement in his symptoms. In addition, he developed hepatosplenomegaly (organomegaly); endocrinopathies such as diabetes mellitus, central hypogonadism, and hypothyroidism; monoclonal spike evidenced in the serum electrophoresis; and hyperpigmentation of skin, altogether consistent with POEMS syndrome...
October 2016: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/27738309/young-patient-with-arterial-thrombosis-and-skin-changes-as-the-onset-manifestations-poems-syndrome
#20
Ting-Ting Han, Shuang Zheng, Zeng-Ai Chen, Wei Liu, Yao-Min Hu
POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Arterial thrombosis is a distinctively unusual feature in patients with POEMS syndrome. We report a 33-year-old man with intermittent amaurosis of left eye and skin changes as the onset manifestations, who was finally confirmed as having POEMS syndrome. Most notably, this was a young man without high risk factors of arterial thrombosis and no monoclonal protein was detected until the repeated measurement later...
November 8, 2016: Oncotarget
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