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Histiocytosis

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https://www.readbyqxmd.com/read/28099400/cns-directed-prophylactic-approach-to-langerhans-cell-histiocytosis
#1
Shinsaku Imashuku, Yoko Shioda, Akira Morimoto
No abstract text is available yet for this article.
January 17, 2017: Journal of Pediatric Hematology/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
#2
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...
January 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28097846/-solitary-and-congenital-juvenile-xanthogranuloma-case-report
#3
Leticia Lazarte, Paola C Stefano, Marcela Bocian, Verónica Solernou, Adrián Martín Pierini, Andrea Bettina Cervini
Juvenile xanthogranuloma is a bening pathology and it represents the most common form of non-Langerhans cell histiocytosis. It is characterized by the presence of papules or firm nodules of a pinkish or yellow-brownish nature, which mainly compromise the skin and, exceptionally, other organs. It is a self-limited entity having a spontaneous regression during the first five years of life. We report the case of a one-month-old patient who presented a congenital tumor in the abdomen, whose histopathology showed the presence of multinucleated giant Touton cells, which are typical of this pathology...
1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28095017/spectrum-of-xanthogranulomatous-processes-in-the-abdomen-and-pelvis-a-pictorial-review-of-infectious-inflammatory-and-proliferative-responses
#4
Kelsey S Bourm, Christine O Menias, Kamran Ali, Kinan Alhalabi, Khaled M Elsayes
OBJECTIVE: Xanthogranulomatous (XG) processes are rare inflammatory conditions with the characteristic pathologic feature of lipid-laden macrophages or histiocyte cells. Imaging findings are nonspecific and can simulate aggressive neoplastic processes. XG processes can be caused by infection, inflammation, histolytic process, or an inherited lysosomal disorder. XG infectious processes are mainly seen in cholecystitis and pyelonephritis, but several other organs can also be involved. Histiocytic processes can be divided into Langerhans and non-Langerhans cell histiocytosis...
January 17, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#5
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 10, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28087041/dermoscopy-of-langerhans-cell-histiocytosis
#6
Biswanath Behera, Munisamy Malathi, Nagendran Prabhakaran, Kamat Divya, Devinder Mohan Thappa, Bheemanathi Hanuman Srinivas
No abstract text is available yet for this article.
February 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28078666/pediatric-erdheim-chester-disease-with-aggressive-skin-manifestations
#7
H-H Su, W Wu, Y Guo, H-D Chen, S-J Shan
Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, is uncommon and characterized by the accumulation of CD68+/CD1a- foamy histiocytes. It is extremely rare in children. The skin lesions of pediatric ECD was not systemically described before. We report a case of ECD in a 3.5 year old Chinese boy. The patient present with generalized skin and bone involvement for 3 years duration. Marked generalized annular maculopapular lesions with central atrophy which differed from previously reported adult xanthoma-like papules or periorbital xanthelasma-like lesions...
January 12, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28077484/-bizarre-rash-adult-onset-cutaneous-langerhans-cell-histiocytosis
#8
Andre Boo Shern Khoo, Jennifer Garioch
No abstract text is available yet for this article.
January 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28076684/radiographic-differentiation-of-advanced-fibrocystic-lung-diseases
#9
Masanori Akira
The concept of end-stage lung disease suggests a final common pathway for most diffuse parenchymal lung diseases. In accordance with this concept, end-stage disease is characterized radiographically and pathologically by the presence of extensive honeycombing. However, sequential computed tomographic (CT) scans obtained from patients with chronic diffuse lung disease evolve over time to show various advanced lung disease patterns other than honeycombing. Also, several radiographically distinct honeycomb patterns, including microcystic, macrocystic, mixed, and combined emphysema and honeycombing differentiate one advanced lung disease from another...
January 11, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28072848/physiologic-determinants-of-exercise-capacity-in-pulmonary-langerhans-cell-histiocytosis-a-multidimensional-analysis
#10
Camille Rolland-Debord, Stephanie Fry, Jonathan Giovannelli, Carole Langlois, Nicolas Bricout, Bernard Aguilaniu, Agnes Bellocq, Olivier Le Rouzic, Stephane Dominique, Alain Delobbe, Geraldine François, Abdellatif Tazi, Benoit Wallaert, Cecile Chenivesse
BACKGROUND: Reduced exercise capacity severely impacts quality of life in pulmonary Langerhans cell histiocytosis. Ascertaining mechanisms that impair exercise capacity is necessary to identify targets for symptomatic treatments. METHODS: Dyspnea, pulmonary function tests and cardiopulmonary exercise test were analysed in 62 study participants. Data were compared between subjects with impaired and normal aerobic capacity (V'O2 peak less than 84% versus 84% predicted or more)...
2017: PloS One
https://www.readbyqxmd.com/read/28066975/targeting-brak-mek-mtor-and-now-akt-in-histiocytosis-the-ongoing-revolution-of-therapies-in-orphan-diseases
#11
Julien Haroche
No abstract text is available yet for this article.
January 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28061539/langerhans-cell-histiocytosis-of-skull-a-retrospective-study-of-18-cases
#12
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/28058158/a-rare-cause-of-proptosis-in-childhood-langerhans-cell-histiocytosis
#13
Mustafa Vatansever, Esra Vatansever, Erdem Dinç, Ayça Sarı, Tuba Kara
A three-year-old male patient was admitted to the clinic with proptosis in his right eye. He had a history of fever with an unknown etiology. In examination, right proptosis was observed and an immobile mass was palpated at the lateral wall of the right orbita. Eye movement was unrestricted in all directions and anterior and posterior segment examination was normal in both eyes. On computed tomography, diffuse bone destruction and expansion was observed in the right orbital lateral wall and other cranial bones...
August 2016: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/28051224/erdheim-chester-disease-and-radiobiphosphonates-bone-scan-from-a-simple-peripheral-form-to-a-rare-axial-location-a-case-report
#14
Salah Oueriagli Nabih
The Erdheim-Chester disease is a rare form of non Langherans histiocytosis. It can be expressed by a multi systemic disease interesting: bone, lung, heart, brain, retro peritoneum, skin and retro-orbital tissue. The diagnosis is established under the radiological aspect, bone scan uptake and immunohistological profile. The bone lesions are almost constant and rather characteristic of bone scintigraphy. We related the case of a young female patient of 26 years old with Erdheim-Chester bone disease. Diagnosis was revealed by diabetes insipidus (nerve damage) and diffuses bones pain...
June 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/28045847/langerhans-cell-histiocytosis-presenting-as-chronic-otitis-externa
#15
William P McCullough, Avrum N Pollock
No abstract text is available yet for this article.
January 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28035124/your-epoch-is-not-for-trying-it-s-for-living-and-for-dying
#16
Leonid P Churilov
The article is devoted to biographies of three Russian physicians of the Silver Age (a period in the History of Russian culture between 1890 and 1917). They made early, significant and internationally recognized contribution into medical science and became eponymous, although social disasters of the twentieth century caused deep impact on their subsequent lives and careers, so their role was shadowed from global medical community. The article analyzes biographies and academic achievements of A-F.K. Siewert (aka: Zivert, Ziwert, von Siewert) (1872-1922), known for first description of the hereditary dyskinesia of cilia (as a triad of: situs inversus of the viscera, abnormal frontal sinuses producing sinusitis and bronchiectasis); S...
December 2016: Psychiatria Danubina
https://www.readbyqxmd.com/read/28027079/differentiating-intralymphatic-histiocytosis-intravascular-histiocytosis-and-subtypes-of-reactive-angioendotheliomatosis-review-of-clinical-and-histologic-features-of-all-cases-reported-to-date
#17
Sean E Mazloom, Alicia Stallings, Angela Kyei
Reactive angioendotheliomatosis (REA) is a rare benign angioproliferative condition of the skin, which has been noted to occur in patients with a variety of underlying systemic diseases. Histopathologically, this condition is characterized by vascular proliferation, and endothelial cell hyperplasia within the lumina and around dermal vessels, without significant cellular atypia. Since the first case of RAE was reported in 1958, multiple histologic patterns of benign cutaneous vascular proliferations with similar clinical presentations to RAE have been described in the literature and have been proposed as subtypes of the originally described condition...
January 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28004130/multifocal-bone-and-bone-marrow-lesions-in-children-mri-findings
#18
Maria Raissaki, Stelios Demetriou, Konstantinos Spanakis, Christos Skiadas, Nikolaos Katzilakis, Emmanouil G Velivassakis, Eftichia Stiakaki, Apostolos H Karantanas
Polyostotic bone and bone marrow lesions in children may be due to various disorders. Radiographically, lytic lesions may become apparent after loss of more than 50% of the bone mineral content. Scintigraphy requires osteoblastic activity and is not specific. MRI may significantly contribute to the correct diagnosis and management. Accurate interpretation of MRI examinations requires understanding of the normal conversion pattern of bone marrow in childhood and of the appearances of red marrow rests and hyperplasia...
December 21, 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/28000234/eruptive-xanthogranuloma-in-a-healthy-adult-male
#19
Ashley Ederle, Kevin H Kim, Jerad M Gardner
Xanthogranuloma is a benign, non-Langerhans cell histiocytosis primarily diagnosed in infants and children, although a subset occurs in adults. Multifocal eruptive presentation of xanthogranuloma is very rare with only four previous cases reported in the literature to our knowledge. We describe a case of eruptive xanthogranuloma in a 49-year-old man who presented with sudden onset of numerous asymptomatic, red-yellow to orange papules on the face, scalp, axilla, flank, and scrotum. Histologic features were consistent with xanthogranuloma with diffuse mixed infiltrate of foamy histiocytes, Touton giant cells, and lymphocytes...
December 21, 2016: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/27990389/laryngeal-involvement-with-fatal-outcome-in-progressive-nodular-histiocytosis-a-rare-case-report
#20
Aarti Salunke, Vasudha Belgaumkar, Ravindranath Chavan, Rinkesh Dobariya
Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution. We report a 60-year-old patient with novel clinical features in the form of extensive noduloulcerative lesions, ichthyotic patches, and laryngeal involvement culminating in fatal outcome prior to therapeutic intervention. Although the presenting features were baffling, histopathology and immunohistochemistry clinched the diagnosis of PNH...
November 2016: Indian Dermatology Online Journal
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