Results 111 to 120 of about 22,345 (254)

Advances in understanding the pathogenesis of HLH

, 2013
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disorder resulting from immune dysfunction reflecting either primary immune deficiency or acquired failure of normal immune homeostasis.
Usmani, G. Naheed, Woda, Bruce A., Newburger, Peter E.   +2 more
core   +1 more source

Secondary HLH Case Report Highlighting Clinical Challenges [PDF]

, 2018
A 19-year-old patient with relapsed acute myeloid leukemia (AML) developed severe and prolonged cytopenia and unexplained jaundice and fever after salvage chemotherapy. His workup revealed hemophagocytosis on the bone marrow biopsy.
Ghada Elgohary, Riad El Fakih, Said Y. Mohamed, Randa Alnounou   +3 more
core   +1 more source

Prognostic Value of Skin Lesion Characteristics in Subcutaneous Panniculitis‐Like T‐Cell Lymphoma: A Retrospective Cohort Study Assessing the Applicability of T Category of the TNM Classification for Primary Cutaneous Lymphomas Other Than Mycosis Fungoides and Sézary Syndrome

International Journal of Dermatology, EarlyView.
Subcutaneous panniculitis‐like T‐cell lymphoma. Survival outcomes according to the presence of hemophagocytic lymphohistiocytosis (HLH) and T category. Relapse‐free survival was inferior in patients presenting with generalized (T3) skin lesions. Overall survival was poor in patients with HLH. Relapse‐free survival was also reduced in patients with HLH.
Myoung Eun Choi, Joon Min Jung, Soo Hyun Jeon, Chong Hyun Won, Sung Eun Chang, Mi Woo Lee, Woo Jin Lee   +6 more
wiley   +1 more source

Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease

Internal Medicine Journal, EarlyView.
Abstract Idiopathic multicentric Castleman disease (iMCD) is a rare condition. The pathogenesis is incompletely understood; however, interleukin‐6 (IL‐6) is a major mediator. The clinical presentation is heterogeneous, from mild constitutional symptoms to severe multi‐organ failure.
Dipti Talaulikar, Jun Yen Ng, Adam Bryant, Annmarie Bosco, Hugh C. Caterson, Pratyush Giri, Pravin Hissaria, Simon J. Harrison, Stephen Lade, Connull Leslie, Jacinta Perram, H. Miles Prince, Dejan Radeski, Maureen Rischmueller, Craig Wallington‐Gates, Nicholas Weber, Alberta Hoi   +16 more
wiley   +1 more source

Effect of FLX on intracellular cAMP response of MLTC-1 cells to hLH or FSK/sub-hLH.

, 2019
(A), (C) Real-time recording of luminescence under stimulation of MLTC-1 cells by hLH and FSK/sub-hLH in the presence of 0 to 100μM FLX. (B), (D) Dose-dependent effects of FLX on hLH and FSK/sub-hLH responses respectively, determined by the Area Under ...
Danièle Klett (252303), Thi Mong Diep Nguyen (776734), Laura Filliatreau (6796871), Yves Combarnous (252322)   +3 more
core   +1 more source

When to consider an inborn error of immunity: clues for physicians

Internal Medicine Journal, EarlyView.
Abstract The term inborn errors of immunity (IEIs) refers to the rapidly expanding group of genetic disorders causing dysregulation of the immune system. With improved genetic testing in recent years, the number of defined IEIs and their range of phenotypic presentations has grown vastly, with more than 550 IEIs now described.
Meera Thangarajah, Lucinda J. Berglund
wiley   +1 more source

Polygenic mutations in the cytotoxicity pathway increase susceptibility to develop HLH immunopathology in mice

, 2016
International audienceHemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disease. Inherited forms of HLH are caused by biallelic mutations in several effectors of granule-dependent lymphocyte-mediated cytotoxicity.
Sepulveda, Fernando, Maschalidi, Sophia, Fischer, Alain, de Saint Basile, Geneviève, Ménasché, Gaël, Garfa-Traore, Meriem, Garrigue, Alexandrine   +6 more
core   +1 more source

Reconfiguring biofortification strategies to transform food systems and address micronutrient deficiency of the 21st century

Journal of Integrative Plant Biology, EarlyView.
This review explores how to make staple foods and horticultural crops more nutritious, including how artificial intelligence‐based screening of gene banks helps deploy nutritionally rich germplasm into breeding. Genome editing can help develop crops richer in minerals, vitamins, and health‐promoting compounds, supporting healthier diets and more ...
Rhowell Jr. N. Tiozon, Alisdair R. Fernie, Nese Sreenivasulu   +2 more
wiley   +1 more source

Effect of A-769662 on intracellular cAMP response of MLTC-1 cells to hLH or FSK/sub-hLH.

, 2019
(A), (C) Real-time recording of luminescence under stimulation of MLTC-1 cells by hLH and FSK/sub-hLH respectively in presence of 0 to 1000μM A-769662. (B), (D) Dose-dependent effects of A-769662 on hLH and FSK/sub-hLH responses respectively, determined ...
Danièle Klett (252303), Thi Mong Diep Nguyen (776734), Laura Filliatreau (6796871), Yves Combarnous (252322)   +3 more
core   +1 more source

The Evolving Landscape of CHD Genetics: A Contemporary Guide to Genetic Testing and Management

Journal of Paediatrics and Child Health, EarlyView.
ABSTRACT Congenital heart disease (CHD) is the most common birth defect, affecting an estimated 9.4/1000 infants globally. The genetics of CHD is complex, with most cases thought to have multifactorial aetiology, implicating both genetic and environmental factors.
Bridget R. O'Malley, Gary F. Sholler, Janine Smith, Gillian M. Blue   +3 more
wiley   +1 more source