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https://www.readbyqxmd.com/read/28315918/fluorrine-18-fluorodeoxyglucose-positron-emission-tomography-computed-tomography-of-adult-liver-langerhans-cell-histiocytosis
#1
Hengshan Ji, Jiang Wu, Guifen Yang, Chuanjin Sun, Hong Zhu, Longjiang Zhang
OBJECTIVE: Adult liver Langerhans cell histiocytosis (LCH) is an extremely rare desease. This paper reports a 40 years old male patient who was diagnosed as liver LCH though ultrasound-guided liver biopsy. The initial Fluorrine-18- fluorodeoxyglucose positron emission tomography/ computed tomography ((18)F-FDG PET/CT) showed multiple nodular low-density lesions in liver without obvious elevated (18)F-FDG uptake. Four years later, the follow-up (18)F-FDG PET/CT showed the liver multiple lesions with slightly elevated (18)F-FDG uptake...
March 20, 2017: Hellenic Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/28285639/denosumab-for-the-treatment-of-adult-multisystem-langerhans-cell-histiocytosis
#2
Polyzois Makras, Marina Tsoli, Athanasios D Anastasilakis, Maria Thanou, Gregory Kaltsas
PURPOSE: An etiological treatment is currently lacking for Langerhans Cell Histiocytosis (LCH). Receptor activator of nuclear factor κB ligand (RANKL) appears to play a central role in the lesional immunological process inducing compensatory osteoprotegerin (OPG) activation. In a preliminary study we aimed to evaluate for the first time the use of denosumab, a RANKL inhibitor, as a targeted treatment strategy in LCH in order to support and enhance endogenous OPG action in order to control or alter the lesional immunological process...
April 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28275281/influence-of-light-conditions-and-light-sources-on-clinical-measurement-of-natural-teeth-color-using-vita-easyshade-advance-4-0-%C3%A2-spectrophotometer-pilot-study
#3
Ivona Posavec, Vladimir Prpić, Dubravka Knezović Zlatarić
OBJECTIVES: The purpose of this study was to evaluate and compare lightness (L), chroma (C) and hue (h), green-red (a) and blue-yellow (b) character of the color of maxillary right central incisors in different light conditions and light sources. MATERIALS AND METHODS: Two examiners who were well trained in digital color evaluation participated in the research. Intraclass correlation coefficients (ICCs) were used to analyze intra- and interobserver reliability. The LCh and L*a*b* values were determined at 08...
December 2016: Acta Stomatologica Croatica
https://www.readbyqxmd.com/read/28262495/-18-f-fdg-pet-ct-in-follow-up-evaluation-in-pediatric-patients-with-langerhans-histiocytosis
#4
J R Garcia, E Riera, P Bassa, S Mourelo, M Soler
PURPOSE: We evaluated the impact of (18)F-FDG PET/CT in identifying sites of active disease and to assess therapeutic follow up in a group of pediatric patients with Langerhans cell histiocytosis (LCH). METHOD: During 2007-2013, 13 (18)F-FDG PET/CT studies were performed for follow-up in 7 patients with a diagnosis of LCH (4 female, 3 male; 1-12 years-old). PET findings were analyzed and correlated with the CT and MRI. Findings were also follow-up by these techniques...
March 2, 2017: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/28255140/idiopathic-thrombocytopenic-purpura-in-childhood-langerhans-cell-histiocytosis-in-adulthood-more-than-a-chance-association
#5
Y C Kuan, M N Nurain
Described herein, a case of Langerhans cell histiocytosis (LCH) in an adult with Idiopathic Thrombocytopenic Purpura (ITP) diagnosed at age ten. She presented with cranial diabetes insipidus, later developed hypogonadotrophic hypogonadism and multiple cervical lympadenopathy from which histopathology of excisional biopsy confirmed LCH. Magnetic resonance imaging showed thickened pituitary stalk. Association of ITP and LCH is unknown but the question of LCH presenting as isolated thrombocytopenia in childhood only to be discovered in adulthood when there was pituitary and bone involvement remains...
February 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28248858/severe-sclerosing-cholangitis-after-langerhans-cell-histiocytosis-treated-by-liver-transplantation-an-adult-case-report
#6
Yunhua Tang, Zhiheng Zhang, Maogen Chen, Weiqiang Ju, Dongping Wang, Fei Ji, Qingqi Ren, Zhiyong Guo, Xiaoshun He
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis. LCH diseases may occur in a single organ or multisystem organ. The patients with multisystem involvement usually have a poor prognosis. Liver involvement in multisystem LCH results in severe complications, such as obvious sclerosing cholangitis (SC) with jaundice. METHODS: We reported a 31-year-old man developed severe SC due to multisystem LCH and was successfully treated by liver transplantation (LT)...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28238607/leydig-cell-hyperplasia-in-children-case-series-and-review
#7
REVIEW
Nicole Mennie, Sebastian K King, Mohan Marulaiah, Peter Ferguson, Yves Heloury, Chris Kimber
INTRODUCTION: Leydig cell hyperplasia (LCH) and Leydig cell tumours (LCTs) in children are rare, typically presenting with precocious puberty. Previously, orchidectomy was the routine management; however, more recently, testis-sparing surgery has been performed with good results. We present a series of unusual presentations of LCH, raising new management questions, and a review of the literature regarding LCH and LCT in children. STUDY DESIGN: We performed a literature search using Ovid Medline, PubMed, and Google Scholar, producing 456 articles...
February 10, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28238339/lobular-capillary-hemangiomas-case-report-and-review-of-literature-of-vascular-lesions-of-the-nasal-cavity
#8
Akina Tamaki, Eric Babajanian, Brian D'Anza, Kenneth Rodriguez
Vascular tumors of the nasal cavity can represent a variety of pathologies. In this case report, we discuss two patients presenting with a large vascular lesion occupying the nasal cavity. Significant bleeding was encountered during the initial attempts for endoscopic surgical resection. One lesion was successfully excised following preoperative embolization while a second following sphenopalatine artery vascular ligation. In both cases, final pathology showed lobular capillary hemangioma (LCH). We present a literature review and discussion of LCH and other vascular tumors that present in the nasal cavity...
February 13, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/28219109/the-use-of-braf-v600e-mutation-specific-immunohistochemistry-in-pediatric-langerhans-cell-histiocytosis
#9
Leomar Y Ballester, Miguel D Cantu, Karen P H Lim, Stephen F Sarabia, Lizmery Suarez Ferguson, C Renee Webb, Carl E Allen, Kenneth L McClain, Carrie A Mohila, Jyotinder N Punia, Angshumoy Roy, Dolores H López-Terrada, M John Hicks, Kevin E Fisher
BRAF p.V600E mutations are detected in greater than 50% of pediatric Langerhans cell histiocytosis (LCH) lesions. However, the use of mutation-specific BRAF V600E immunohistochemistry (IHC) as a surrogate for molecular testing in pediatric LCH is unknown. We tested the mutation-specific BRAF V600E monoclonal antibody (clone VE1) in formalin-fixed, paraffin-embedded LCH samples from 26 pediatric patients (14 males and 12 females, ages 7 mo-17 y) using allele-specific real-time polymerase chain reaction (PCR) with a limit of detection of 0...
February 20, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28194436/real-time-genomic-profiling-of-histiocytoses-identifies-early-kinase-domain-braf-alterations-while-improving-treatment-outcomes
#10
Lynn H Lee, Anjelika Gasilina, Jayeeta Roychoudhury, Jason Clark, Francis X McCormack, Joseph Pressey, Michael S Grimley, Robert Lorsbach, Siraj Ali, Mark Bailey, Philip Stephens, Jeffrey S Ross, Vincent A Miller, Nicolas N Nassar, Ashish R Kumar
Many patients with histiocytic disorders such as Langerhans cell histiocytosis (LCH) or Erdheim-Chester disease (ECD) have treatment-refractory disease or suffer recurrences. Recent findings of gene mutations in histiocytoses have generated options for targeted therapies. We sought to determine the utility of prospective sequencing of select genes to further characterize mutations and identify targeted therapies for patients with histiocytoses. Biopsies of 72 patients with a variety of histiocytoses underwent comprehensive genomic profiling with targeted DNA and RNA sequencing...
February 9, 2017: JCI Insight
https://www.readbyqxmd.com/read/28182116/effective-braf-inhibitor-vemurafenib-therapy-in-a-2-year-old-patient-with-sequentially-diagnosed-langerhans-cell-histiocytosis-and-erdheim-chester-disease
#11
Zsófia Váradi, Rita Bánusz, Judit Csomor, Krisztián Kállay, Edit Varga, Gabriella Kertész, Monika Csóka
Erdheim-Chester disease (ECD) is a rare histiocytic disorder, characterized by the xanthomatous infiltration of tissues by CD68-positive and CD1a-/CD100-negative foamy histiocytes. In childhood, ECD is exceptionally rare, and only a dozen cases have been published so far. The cooccurence of Langerhans cell histiocytosis (LCH) and ECD is even rarer. Here, we report a 2-year-old boy, the youngest patient in the literature so far, who was diagnosed with concomitant BRAF mutation-positive LCH and ECD. In his case, conventional LCH treatment proved to be ineffective, but he is the youngest patient who was successfully treated with the BRAF inhibitor vemurafenib...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28123731/bone-langerhans-cell-histiocytosis-with-pulmonary-involvement-in-an-adult-non-smoker-a-case-report-and-brief-review-of-the-literature
#12
Jie Shen, Shicheng Feng
Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause, which encompasses a set of disorders of multiple organs with various clinical presentations that share the common characteristic of sizeable Langerhans cell infiltration. The clinical spectrum of LCH ranges from solitary bone lesions to involvement of other organs. lung involvement in LCH may be part of a multisystem disease, which almost exclusively occurs in adult smokers, while it is overlooked or misdiagnosed in the majority of non-smokers...
January 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28099396/successful-treatment-of-refractory-langerhans-cell-histiocytosis-of-the-choroid-plexus-in-a-child-with-pulse-dexamethasone-and-lenalidomide
#13
Ramya Uppuluri, Sreejith Ramachandrakurup, Ravikanth Balaji, Divya Subburaj, Atish Bakane, Revathi Raj
Refractory/relapsed Langerhans cell histiocytosis (LCH) has a difficult course with a guarded prognosis. Salvage treatments available are both expensive and highly toxic. On the basis of the pathophysiology of LCH, we used a novel protocol including pulse dexamethasone and lenalidomide in a child with refractory LCH involving the choroid plexus, which resulted in durable remission with minimal toxicity. The protocol was extrapolated from the FIRST trial for patients with multiple myeloma. We present the clinical course, treatment protocol, and outcome in this child, who is at present disease free and in remission 18 months posttreatment...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28090481/salvage-treatment-of-relapsed-refractory-lch
#14
Jong Jin Seo
No abstract text is available yet for this article.
December 2016: Blood Research
https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#15
REVIEW
Jean Donadieu, Sébastien Héritier
DEFINITION: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels...
January 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28061539/langerhans-cell-histiocytosis-of-skull-a-retrospective-study-of-18-cases
#16
Xiang-Heng Zhang, Ji Zhang, Zheng-He Chen, Ke Sai, Yin-Sheng Chen, Jian Wang, Chao Ke, Chen-Chen Guo, Zhong-Ping Chen, Yong-Gao Mou
BACKGROUND: The present study presents 18 cases of Chinese patients harboring a Langerhans cell histiocytosis (LCH) of the skull. METHODS: Eighteen consecutive patients were diagnosed as LCH of the skull and confirmed pathologically between March 2002 and February 2014. In the present study, the patients of LCH without skull involvement were excluded. According to disease extent at diagnosis, the 18 LCH patients with skull involvement were divided into three groups: (I) unifocal-monosystem group, including ten cases with solitary skull lesion; (II) multifocal-monosystem group, including two cases with multiple bone lesions and no extra-skeletal involvement; (III) multisystem group, including six cases with LCH lesions involving both skeletal and extra-skeletal system...
November 28, 2016: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/28057035/control-and-eradication-of-porcine-reproductive-and-respiratory-syndrome-virus-type-2-using-a-modified-live-type-2-vaccine-in-combination-with-a-load-close-homogenise-model-an-area-elimination-study
#17
Poul H Rathkjen, Johannes Dall
BACKGROUND: Porcine reproductive and respiratory syndrome virus (PRRSV) causes significant animal and economic losses worldwide. The infection is difficult to control and PRRSV elimination at local level requires coordinated intervention among multiple farms. This case study describes a successful elimination of PRRSV from all 12 herds on the Horne Peninsula, Denmark, using a combination of load, close, homogenise (LCH) using PRRSV type 2 modified-live vaccine, optimised pig flow, and'10 Golden Rules' (10GR) for biosecurity management...
January 5, 2017: Acta Veterinaria Scandinavica
https://www.readbyqxmd.com/read/28032306/the-efficacy-of-an-american-indian-culturally-based-risk-prevention-program-for-upper-elementary-school-youth-residing-on-the-northern-plains-reservations
#18
John J Usera
Culturally-based risk behavior prevention programs for American Indian elementary school children are sparse. Thus a group of American Indian educators collaborated in the creation of a program that helps children make healthy decisions based on their cultural and traditional value system. In this paper the effectiveness of Lakota Circles of Hope (LCH), an elementary school culturally-based prevention program was studied and evaluated. Three cohorts of fourth and fifth graders participated in a mixed methods quasi-experimental evaluative research design that included focus groups and surveys prior to and following the intervention...
December 29, 2016: Journal of Primary Prevention
https://www.readbyqxmd.com/read/27951529/predictor-variables-of-developing-anterior-pituitary-deficiencies-in-a-group-of-paediatric-patients-with-central-diabetes-insipidus-and-langerhans-cell-histiocytosis
#19
Elisa Vaiani, Carmen Malossetti, Lina Margarita Vega, Pedro Zubizarreta, Jorge Braier, Alicia Belgorosky
BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unknown etiopathogenesis. Central diabetes insipidus (CDI) is the most frequent endocrine manifestation and is a known risk factor for the development of further anterior pituitary hormone deficiencies (APD). However, not all CDI patients develop APD, as observed during prolonged periods of follow-up. AIM: To find predictors of developing APD in LCH children with CDI followed in our institution...
2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/27935269/from-adolescent-to-elder-rats-motivation-for-palatable-food-and-cannabinoids-receptors
#20
Amancio-Belmont Octavio, Romano-López Antonio, Ruiz-Contreras Alejandra Evelin, Méndez-Díaz Mónica, Prospéro-García Oscar
To analyze motivation, food self-administration and decision-making were evaluated in adolescent, adult, and aged rats. Subjects were trained to press a lever (fixed ratio, FR1 and FR5) in an operant chamber, to obtain chocolate flavor pellets. They assessed the progressive ratio (PR), extinction, and reinstatement of the behavior. To estimate decision-making for food, rats were trained in the conditioned place preference (CPP) paradigm: (a) associating one compartment with lab chow (LCh) one day and the other compartment with rice krisspies (RK), the next day...
December 9, 2016: Developmental Neurobiology
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