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https://www.readbyqxmd.com/read/29684651/the-geography-of-smallpox-in-england-before-vaccination-a-conundrum-resolved
#1
Romola Jane Davenport, Max Satchell, Leigh Matthew William Shaw-Taylor
Smallpox is regarded as an ancient and lethal disease of humans, however very little is known about the prevalence and impact of smallpox before the advent of vaccination (c.1800). Here we use evidence from English burial records covering the period 1650-1799 to confirm a striking geography to smallpox patterns. Smallpox apparently circulated as a childhood disease in northern England and Sweden, even where population densities were low and settlement patterns dispersed. However, smallpox was a relatively rare epidemic disease in southern England outside the largest cities, despite its commercialised economy and the growing spatial interconnectedness of its settlements...
April 16, 2018: Social Science & Medicine
https://www.readbyqxmd.com/read/29684563/long-term-results-of-prophylactic-donor-lymphocyte-infusions-for-patients-with-multiple-myeloma-after-allogeneic-stem-cell-transplantation
#2
Maximilian Gröger, Nico Gagelmann, Christine Wolschke, Ute-Marie von Pein, Evgeny Klyuchnikov, Max Christopeit, Axel Zander, Francis Ayuk, Nicolaus Kröger
OBJECTIVE: The major reason for treatment failure after allografting in multiple myeloma (MM) is relapse. Donor lymphocyte infusions are considered a valuable post-transplant strategy mainly for relapsed patients but using them to prevent relapse in MM has been reported rarely. In the present study we examined the efficacy of prophylactic donor lymphocyte infusions after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in myeloma patients with a long-term follow-up of more than 5 years...
April 20, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29684428/validation-of-a-cd1b-tetramer-assay-for-studies-of-human-mycobacterial-infection-or-vaccination
#3
Erik D Layton, Krystle K Q Yu, Malisa T Smith, Thomas J Scriba, Stephen C De Rosa, Chetan Seshadri
CD1 tetramers loaded with lipid antigens facilitate the identification of rare lipid-antigen specific T cells present in human blood and tissue. Because CD1 proteins are structurally non-polymorphic, these tetramers can be applied to genetically diverse human populations, unlike MHC-I and MHC-II tetramers. However, there are no standardized assays to quantify and characterize lipid antigen-specific T cells present within clinical samples. We incorporated CD1b tetramers loaded with the mycobacterial lipid glucose monomycolate (GMM) into a multi-parameter flow cytometry assay...
April 20, 2018: Journal of Immunological Methods
https://www.readbyqxmd.com/read/29684363/immunolabeling-of-cleared-human-pancreata-provides-insights-into-three-dimensional-pancreatic-anatomy-and-pathology
#4
Michaël Noë, Neda Rezaee, Kaushal Asrani, Michael Skaro, Vincent P Groot, Pei-Hsun Wu, Matthew T Olson, Seung-Mo Hong, Sung Joo Kim, Matthew J Weiss, Christopher L Wolfgang, Martin A Makary, Jin He, John L Cameron, Denis Wirtz, Nicholas J Roberts, G Johan A Offerhaus, Lodewijk A A Brosens, Laura D Wood, Ralph H Hruban
Visualizing pathologies in three dimensions (3D) can provide unique insights into the biology of human diseases. A rapid and easy-to-implement dibenzyl ether-based technique was used to clear thick sections of surgically resected human pancreatic parenchyma. Protocols were applicable to both fresh and formalin-fixed paraffin-embedded tissue. The penetration of antibodies into dense pancreatic parenchyma was optimized using both gradually increasing antibody concentrations and centrifugal flow. Immunolabeling with antibodies against CK19 was visualized using both light sheet and confocal laser scanning microscopy...
April 20, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29684361/high-frequency-of-ovarian-cyst-development-in-vhl-2b-snf5-mice
#5
Yasumichi Kuwahara, Leslie M Kennedy, Anthony N Karnezis, E Lorena Mora-Blanco, Arlin B Rogers, Christopher D Fletcher, David G Huntsman, Charles W M Roberts, W Kimryn Rathmell, Bernard E Weissman
The new paradigm of mutations in chromatin modifying genes as driver events in the development of cancers has proved challenging to resolve the complex influences over disease phenotypes. In particular, impaired activities of members of the SWI/SNF chromatin remodeling complex have appeared in an increasing variety of tumors. Mutations in SNF5, a member of this ubiquitously expressed complex, arise in almost all cases of malignant rhabdoid tumor in the absence of additional genetic alterations. Therefore, we studied how activation of additional oncogenic pathways might shift the phenotype of disease driven by SNF5 loss...
April 20, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29684339/sorafenib-induced-tumor-lysis-syndrome-in-a-patient-with-metastatic-hepatocellular-carcinoma
#6
Sardar Zakariya Imam, Mohammad Faizan Zahid, Muhammad Asad Maqbool
Tumor lysis syndrome is a potentially lethal complication of chemotherapy, usually associated with aggressive hematologic malignancies. We describe the case of a young patient with metastatic hepatocellular cancer who developed rapid and fatal tumor lysis syndrome following initiation of sorafenib therapy. Although rare with sorafenib therapy for hepatocellular carcinoma, tumor lysis syndrome is serious complication. Patients with a high burden of disease at therapy initiation should have their metabolic parameters measured prior to starting therapy and closely followed for the first 1-2 weeks while being treated...
April 17, 2018: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/29684307/osteochondroma-of-the-zygomatic-arch-a-case-report-and-review-of-the-literature
#7
R Patel, G Obeid
An osteochondroma, when reported in the maxillofacial region, is a benign neoplasm that involves the skull base, maxillary sinus, zygomatic arch, or mandible. Most commonly, the osteochondroma occurs in the coronoid process and the condyle. One rare subtype of osteochondroma reported in the literature, termed Jacob disease, arises from the coronoid process and interferes with the zygomatic arch. This report describes a unique case of an isolated osteochondroma arising from the zygomatic arch and interfering with the coronoid process, which was treated through surgical excision by an intraoral approach...
March 31, 2018: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29684021/laboratory-and-molecular-surveillance-of-paediatric-typhoidal-salmonella-in-nepal-antimicrobial-resistance-and-implications-for-vaccine-policy
#8
Carl D Britto, Zoe A Dyson, Sebastian Duchene, Michael J Carter, Meeru Gurung, Dominic F Kelly, David R Murdoch, Imran Ansari, Stephen Thorson, Shrijana Shrestha, Neelam Adhikari, Gordon Dougan, Kathryn E Holt, Andrew J Pollard
BACKGROUND: Children are substantially affected by enteric fever in most settings with a high burden of the disease, including Nepal. However pathogen population structure and transmission dynamics are poorly delineated in young children, the proposed target group for immunization programs. Here we present whole genome sequencing and antimicrobial susceptibility data on 198 S. Typhi and 66 S. Paratyphi A isolated from children aged 2 months to 15 years of age during blood culture surveillance at Patan Hospital, Nepal, 2008-2016...
April 23, 2018: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/29683955/successful-management-of-a-child-with-drug-induced-necrotizing-pancreatitis-during-acute-lymphoblastic-leukemia-therapy-a-case-report
#9
Anna Płotka, Agnieszka Wziątek, Jacek Wachowiak, Katarzyna Derwich
Acute pancreatitis in children acute lymphoblastic leukemia is commonly caused by drugs, for example, L-Asparaginase, pegapargase, steroids. The incidence of this complication is estimated at 6.7% to 18%. Although the majority of drug-induced acute pancreatitis cases are mild, severe cases can rarely occur. This work presents a case of successful management of a child with drug-induced necrotizing pancreatitis during acute lymphoblastic leukemia therapy. This case illustrates that comprehensive care and immediate intensive treatment can rescue patient despite poor prognosis...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683949/a-rare-case-of-hemophagocytic-lymphohistiocytosis-associated-with-myelodysplastic-syndrome-and-trisomy-8-in-a-pediatric-patient
#10
Seh Hyun Kim, Dae Yong Yi, Na Mi Lee, Sin Weon Yun, Soo Ahn Chae, In Seok Lim
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting in clinical and biochemical manifestations of extreme inflammation. Myelodysplastic syndrome (MDS) represents a heterogenous group of clonal hematopoietic disorders. The development of MDS is common in children with trisomy of chromosome 8. Here, we report a fatal case of 8-year-old girl who was admitted to the emergency department with status epilepticus, and later diagnosed with HLH associated with MDS and trisomy of chromosome 8. We believe this is the first reported case of HLH associated with MDS and trisomy 8 in a pediatric patient...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29683821/urothelial-carcinoma-in-situ-of-the-bladder-with-glandular-differentiation-report-of-92-cases
#11
Zhiming Yang, Jonathan I Epstein
Urothelial carcinoma in situ (CIS) of the bladder with glandular differentiation (CIS-GL) is rare with some showing an association with small cell carcinoma. There is a paucity of data on whether CIS-GL diagnosed in the absence of invasive carcinoma is associated with an increased risk of developing small cell carcinoma of the bladder. Twenty-seven cases of CIS-GL were identified from the consult files of one of the authors from 2008 to 2015 without prior or coexisting invasive carcinoma at the time of diagnosis...
April 20, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29683365/rare-diseases-in-romania-a-response-to-transposition-and-implementation-of-eu-rare-diseases-policy-in-eastern-europe
#12
Emilia Severin
No abstract text is available yet for this article.
April 23, 2018: Expert Review of Pharmacoeconomics & Outcomes Research
https://www.readbyqxmd.com/read/29683292/dermatitis-artefacta-in-childhood-and-adolesence-a-spectrum-of-disease
#13
Padma Mohandas, Jane Ravenscroft, Anthony Bewley
Dermatitis Artefacta (DA) or Artefactual skin disease (ASD) is a factitious skin disorder rarely reported in the paediatric population. Skin lesions are produced deliberately either consciously or in a dissociative state to satisfy an underlying psychological need. Children may present with acutely formed skin changes or with chronic lesions, quite often having seen other specialists during their journey. The mechanism of formation of skin lesions can vary from the application of pigment onto the skin to simulate disease or more destructive techniques like the injection of irritant substances into the skin...
April 19, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/29683226/low-incidence-of-heparin-induced-skin-lesions-in-orthopedic-surgery-patients-with-low-molecular-weight-heparins
#14
M Schindewolf, M Paulik, H Kroll, R Kaufmann, M Wolter, W-H Boehncke, E Lindhoff-Last, A Recke, R J Ludwig
BACKGROUND: Heparins are widely prescribed for prevention and therapy of arterial and venous thromboembolic diseases. Heparin-induced skin lesions are the most frequent adverse effects of subcutaneous heparin treatment in non-surgical patients (7.5-39.8%); no data exist on surgical patients. Commonly, they are due to a delayed-type hypersensitivity reaction (DTH), but may also be a manifestation of life-threatening heparin-induced thrombocytopenia (HIT). Lesions of both entities resemble initially...
April 23, 2018: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29683194/clinical-experience-with-multigene-carrier-panels-in-the-reproductive-setting
#15
Catherine Terhaar, Nicole Teed, Rachel Allen, Lindsay Dohany, Christina Settler, Carol Holland, Ryan E Longman
OBJECTIVES: Expanded carrier testing is acknowledged as an acceptable strategy for carrier testing by the American College of Obstetrics and Gynecology. Limited studies have investigated positivity rates of expanded carrier panels. We describe our experience with three commercial laboratory panels varying in size from 3 to 218 disorders. METHODS: We reviewed outcomes for three multigene carrier screening panels: Trio (3 diseases), Standard (23 diseases), and Global (218 diseases)...
April 23, 2018: Prenatal Diagnosis
https://www.readbyqxmd.com/read/29682736/medical-comorbidities-in-patients-with-lichen-planopilaris-a-retrospective-case-control-study
#16
Raymond M Fertig, Shasa Hu, Austin J Maddy, Alexandra Balaban, Nouf Aleid, Adam Aldahan, Antonella Tosti
BACKGROUND: Lichen planopilaris (LPP) is a rare inflammatory lymphocyte-mediated disease of the scalp considered to have an autoimmune pathogenesis. OBJECTIVES: To identify the prevalence of medical comorbidities in patients with classic LPP (CLPP) and frontal fibrosing alopecia (FFA). METHODS: The medical records of 206 LPP patients and 323 control patients were retrospectively reviewed for existing comorbidities. The control group consisted of 257 patients with androgenetic alopecia (ICD 9 = 704...
April 16, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29682612/salvage-pelvic-lymph-node-dissection-after-fluciclovine-positron-emission-tomography-computed-tomography-detected-prostate-cancer-recurrence
#17
Madeline Cancian, Jorge Pereira, Joseph F Renzulli
Background: Multiple new systemic agents have been targeted to metastatic prostate cancer, with decreased progression of disease but no cure. Surgical management of metastatic disease has been gaining interest, primarily in the setting of high-risk prostatectomies. However, metastasis-directed surgical intervention has been employed in rare scenarios, especially in oligometastatic disease. We report here on a salvage robot-assisted pelvic lymph node dissection for a solitary metastatic site. Case Presentation: A 63-year-old Hispanic man who was initially treated with prostatectomy for intermediate risk cancer developed rapid biochemical recurrence...
2018: Journal of Endourology Case Reports
https://www.readbyqxmd.com/read/29682541/madelung-s-disease-lipectomy-or-liposuction
#18
REVIEW
Chun-Ye Chen, Qing-Qing Fang, Xiao-Feng Wang, Min-Xia Zhang, Wan-Yi Zhao, Bang-Hui Shi, Li-Hong Wu, Li-Yun Zhang, Wei-Qiang Tan
Background: Madelung's disease is a rare lipid metabolic disorder characterized by diffuse, uncapsulated lipomas in the neck, shoulder, and other areas. It mainly affects middle-aged men and is related to alcohol abuse, and the cause is not clear. Surgical treatments include lipectomy and liposuction. Methods: This systematic review analyzed the treatment of Madelung's disease described in 52 articles including complete patient details, published between 2000 and 2015, and retrieved from the Web of Science, PubMed, Medline, and Embase...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29682464/gastric-inflammatory-fibroid-polyp-tumor-with-acute-intestinal-obstruction-vanek-s-tumor-can-mimick-a-giant-gastrointestinal-stromal-tumor-or-a-gastric-lymphoma
#19
Francesco Fleres, Carmelo Mazzeo, Antonio Ieni, Maurizio Rossitto, Eugenio Cucinotta
An inflammatory fibroid polyp (IFP) is a solitary rare benign neoplasm of the gastrointestinal tract, frequently located in the gastric antrum. IFPs account for about 0.1% of all gastric polyps. We report a case of a giant gastric inflammatory polyp of 2.5 cm × 7 cm that determines a gastric outlet obstruction called "ball valve syndrome" mimicking a gastrointestinal stromal tumor (GIST) and a gastric lymphoma, with an intestinal obstruction of high origin. Therefore, due to acute presentation we have decided to submit the patient to a subtotal gastrectomy...
2018: Journal of Visualized Surgery
https://www.readbyqxmd.com/read/29682452/extending-the-phenotypic-spectrum-of-sengers-syndrome-congenital-lactic-acidosis-with-synthetic-liver-dysfunction
#20
David B Beck, Kristina Cusmano-Ozog, Nickie Andescavage, Eyby Leon
Sengers syndrome is a rare autosomal recessive mitochondrial disease characterized by lactic acidosis, hypertrophic cardiomyopathy and bilateral cataracts. We present here a case of neonatal demise, within the first day of life, who initially presented with severe lactic acidosis, with evidence of both chorioamnionitis and cardiogenic shock. Initial metabolic labs demonstrated a severe lactic acidosis prompting genetic testing which revealed a homozygous pathogenic variant for Sengers syndrome in AGK, c.979A >  T; p...
April 13, 2018: Translational Science of Rare Diseases
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