Results 81 to 90 of about 21,085 (234)

Ultrasound findings associated with neonatal acute liver failure secondary to gestational alloimmune liver disease: A case series

JPGN Reports, EarlyView.
Abstract Objectives Gestational alloimmune liver disease is the leading cause of neonatal acute liver failure, hypothesized to emerge from maternal exposure to an antigen that is expressed on fetal hepatocytes, resulting in maternal‐fetal alloimmune attack and activation of the complement cascade.
Naseem Ravanbakhsh, Ashley M. Upton, Chun‐Sing Huang, Nhu Thao N. Galván, John Goss, Sanjiv Harpavat, Jose A. Hernandez   +6 more
wiley   +1 more source

Diagnosis and Management of Prenatal Hereditary Pyropoikilocytosis

Prenatal Diagnosis, EarlyView.
ABSTRACT Hereditary pyropoikilocytosis (HPP) is a severe hemolytic anemia caused by variants in SPTA1, SPTB, and EPB41. These weaken horizontal interactions in the erythrocyte cytoskeleton, causing membrane fragmentation and splenic sequestration. It will readily cause fetal anemia and often hydrops fetalis. Prenatal diagnosis requires first ruling out
Connor Hartzell, Samantha Stover, Nora Gibson, Timothy Olson, James Connelly, Matthew Grace, Jennifer Andrews   +6 more
wiley   +1 more source

Long‐term outcomes of haploidentical hematopoietic stem cell transplantation with antithymocyte globulin–based myeloablative conditioning in pediatric refractory or relapsed non‐Hodgkin lymphoma

Pediatric Investigation, EarlyView.
Single‐center retrospective data showed that ATG‐based myeloablative haploidentical hematopoietic stem cell transplantation provides durable remission and favorable long‐term survival in children with refractory/relapsed non‐Hodgkin lymphoma, achieving high engraftment rates, low relapse incidence, and acceptable toxicity.
Chenguang Jia, Jie Zheng, Meng Yuan, Hongbo He, Yibo Fan, Bin Wang, Guanghua Zhu, Jun Yang, Yanhui Luo, Lixiao Cai, Pan Zhang, Yanlong Duan, Maoquan Qin   +12 more
wiley   +1 more source

Hemophagocytic Lymphohistiocytosis Syndrome Secondary to Disseminated Histoplasmosis in a Presumably Immunocompetent Adolescent: A Novel Case

, 2022
Hemophagocytic Lymphohistiocytosis Syndrome Secondary to Disseminated Histoplasmosis in a Presumably Immunocompetent Adolescent: A Novel ...
Mark T. Cunningham (12468553), Jyoti Panicker (12468556), Thomas Loew (7376363), Daniel S. Cirotski (12468550)   +3 more
core   +1 more source

Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family) [PDF]

, 2019
Family hemophagocytic lymphohistiocytosis (hemophagocytic syndrome) is a rare hereditary disease, which is based on a disturbance of the regulation of the immune response, leading to proliferation and activation of histiocytes, phagocytosis of peripheral
V. A. Greshnyakova, S. P. Kaplina, N. A. Efremova, T. A. Bykova, A. A. Osipova, L. G. Goryacheva   +5 more
core   +1 more source

An Atypical Skin Presentation of Adult‐Onset Still's Disease With Histological Description: A Case Report

Journal of Cutaneous Pathology, EarlyView.
ABSTRACT Adult‐onset Still's disease (AOSD) is an auto inflammatory disorder with a variable clinical presentation, and without a pathognomonic diagnostic test, characterized by high spiking fever, arthralgia/arthritis, a suggestive skin rash, elevated white blood cell count 10 G/L (> 80% neutrophils), elevated ferritin, with glycosylated ferritin < 20%
M. Nordmann, T. Mazza, S. Menzinger, G. Kaya, S. Bogiatzi, S. Vandenberghe‐Dürr, M. Blanchard, J. Stirnemann   +7 more
wiley   +1 more source

Primary Hemophagocytic Lymphohistiocytosis in an Infant

, 2014
The paper considers the results of a clinical observation of an infant with hemophagocytic lymphohistiocytosis complicated with spontaneous splenic rupture.
T.A. Khalturyna, V.P. Kandyba, O.A. Zorya, A.I. Kozhemiaka, M.I. Perkhun, V.A. Klymenko, O.V. Piontkovska, A.V. Postnikov   +7 more
core   +1 more source

Adult-onset central nervous system hemophagocytic lymphohistiocytosis: a case report [PDF]

, 2015
© 2015 Pastula et al.Background: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome with both genetic and acquired causes characterized by elevated cytokine levels, hyperinflammation, and overactivation of lymphocytes and macrophages.
Cha, Soonmee, Bollen, A, Ralph, J, Soonmee Cha, Douglas, VC, Jeffrey Ralph, Vanja C. Douglas, Gerald F. Reis, Andrew Bollen, Mark Burish, Douglas, Vanja, Daniel M. Pastula, Pastula, DM, Reis, GF, Burish, M   +14 more
core   +2 more sources

Primary Cutaneous Gamma‐Delta T‐Cell Lymphoma Complicating Long‐Standing Immunosuppressed Dermatomyositis

Journal of Cutaneous Pathology, EarlyView.
ABSTRACT Primary cutaneous gamma‐delta T‐cell lymphoma (PCGD‐TCL) is a rare cytotoxic lymphoma with key oncogenic drivers in the JAK/STAT pathway. Also primarily involving the subcutaneous adipose tissue, subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) is more frequently encountered in scenarios of autoimmune disorders.
Bennett Christie‐Nguyen, Youn H. Kim, David F. Fiorentino, Danielle Tartar, Ryanne Brown, Robert A. Novoa, Kerri E. Rieger   +6 more
wiley   +1 more source

Inborn errors of immunity in children with neuroinflammation

Developmental Medicine &Child Neurology, EarlyView.
Abstract Inborn errors of immunity (IEIs), an expanding group of monogenic disorders with diverse clinical manifestations, are increasingly recognized to include neuroinflammatory disease. Examples of diseases included under this umbrella are Aicardi–Goutières syndrome, deficiency of adenosine deaminase 2, familial haemophagocytic lymphohistiocytosis ...
Eppie M Yiu, Dilan Dissanayake, Jessie Cunningham, Helen Branson, Ori Scott, E Ann Yeh   +5 more
wiley   +1 more source