Results 61 to 70 of about 138,072 (341)

NRASQ61R Expression in Lymphatic Endothelial Cells Causes Enlarged Vessels, Hemorrhagic Chylous Effusions, and High Mortality in a Mouse Model of Kaposiform Lymphangiomatosis

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Background Kaposiform lymphangiomatosis (KLA) is an aggressive complex lymphatic anomaly. Patients exhibit malformed lymphatic vessels and often develop hemorrhagic effusions and elevated angiopoietin‐2 (Ang‐2) levels. A somatic NRAS p.Q61R (NRASQ61R) mutation has been associated with KLA.
C. Griffin McDaniel, Patricia Pastura, Joshua P. Scallan, Timothy D. Le Cras   +3 more
wiley   +1 more source

B-cell Prolymphocytic Leukemia in a Young Male [PDF]

, 2011
B-cell prolymphocytic leukemia [B-PLL] is a neoplasm of B prolymphocytes affecting the peripheral blood, bone marrow and spleen. The principal disease characteristics are massive splenomegaly with absent or minimal peripheral lymphadenopathy and a ...
Fernandes, H, Jayaprakash, C, Pailoor, K, Swethadri, GK   +3 more
core  

Sertraline Treatment Can Mimic Niemann‐Pick Type C Biomarker Profile: A Diagnostic Pitfall

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Background Oxysterols (cholestane‐3β,5α,6β‐triol and 7‐ketocholesterol) and N‐palmitoyl‐O‐phosphocholineserine (PPCS) are sensitive biomarkers for Niemann‐Pick disease type C (NPC) screening. However, false‐positive results occur, with a biomarker profile suggestive of NPC despite the absence of pathogenic variants in genes involved in NPC or ...
Maria Makrygianni, Cecile Pagan, Antony Citterio‐Quentin, Isabelle Rouvet, Giulia Dingeo, Cyril Hanin, Claudine Laurent‐Levinson, Samia Pichard, Bénédicte Héron, Clément Gourguechon, Daniele Mandia, Jerome Guitton, Carole Lacout, Nicolas Schleinitz, Florient Potier, Olivier Flabeau, Elsa Besse‐Pinot, Foudil Lamari, Magali Pettazzoni, Yann Nadjar   +19 more
wiley   +1 more source

Foci of Schistosomiasis mekongi, Northern Cambodia: II. Distribution of infection and morbidity. [PDF]

, 1999
In the province of Kracheh, in Northern Cambodia, a baseline epidemiological survey on Schistosoma mekongi was conducted along the Mekong River between December 1994 and April 1995. The results of household surveys of highly affected villages of the East
Biays, S, Legros, P, Lormand, J D, Ly, C S, Men, C, Odermatt, P, Philips, M, Saem, C, Sokha, K, Stich, A H, Tanner, M   +10 more
core   +1 more source

Natural history of patients with non cirrhotic portal hypertension: Comparison with patients with compensated cirrhosis [PDF]

, 2018
Background. The knowledge of natural history of patients with portal hypertension (PH) not due to cirrhosis is less well known than that of cirrhotic patients. Aim.
Aprile, Francesca, Gioia, Stefania, Merli, Manuela, Nardelli, Silvia, Nicoletti, Valeria, Pasquale, Chiara, Pentassuglio, Ilaria, Riggio, Oliviero   +7 more
core   +1 more source

Splenomegaly – Diagnostic validity, work-up, and underlying causes

PLoS ONE, 2017
Purpose Our aim was to assess the validity of the ICD-10 code for splenomegaly in the Danish National Registry of Patients (DNRP), as well as to investigate which underlying diseases explained the observed splenomegaly.
Emelie Curovic Rotbain, Dennis Lund Hansen, O. B. Schaffalitzky de Muckadell, F. Wibrand, A. Meldgaard Lund, H. Frederiksen   +5 more
semanticscholar   +1 more source

Biomimetic Copper‐Doped Nano‐Aluminum Adjuvant Potentiates Therapy in Chemoresistant Acute Myeloid Leukemia

Advanced Healthcare Materials, EarlyView.
We developed a novel copper‐doped aluminum nano‐adjuvant (CuNA) to overcome cytarabine resistance in acute myeloid leukemia (AML). CuNA effectively sensitizes drug‐resistant AML cells to cytarabine by inducing mitochondrial dysfunction and inhibiting HMGCR/GPX4 to amplify ferroptosis.
Chao He, Lingxiao Zhang, Yue Xiong, Yu Wang, Yuqing Zeng, Dandan Su, Ran Wang, Ying Li, Hongbo Chen, Fang Cheng, Zhi Ping Xu   +10 more
wiley   +1 more source

Sarcoidosis presenting as isolated massive splenomegaly: A case report

Clinical Case Reports
Key Clinical Message Sarcoidosis is a systemic granulomatous disease with an unknown cause, marked by the presence of noncaseating granulomas in the affected organs.
Melissa Kyriakos Saad, Imad El Hajj, Elias Saikaly   +2 more
doaj   +1 more source

A mutation in caspase-9 decreases the expression of BAFFR and ICOS in patients with immunodeficiency and lymphoproliferation [PDF]

, 2015
Lymphocyte apoptosis is mainly induced by either death receptor-dependent activation of caspase-8 or mitochondria-dependent activation of caspase-9. Mutations in caspase-8 lead to autoimmunity/lymphoproliferation and immunodeficiency. This work describes
Boggio, E, Cappellano, G, Chiocchetti, A., Clemente, N, Dianzani, C, Dianzani, I, Dianzani, U, Gigliotti, Cl, Notarangelo, Ld, Orilieri, E, Pignata, C, Quinti, Isabella, Ramenghi, U, Santoro, C, Toth, E, Valletti, Pa   +15 more
core   +2 more sources

DCAF13 Safeguards Hematopoietic Stem Cells via RRS1‐Regulated Ribosome Biogenesis

Advanced Science, EarlyView.
This study establishes DCAF13 as an essential regulator for hematopoietic stem cell (HSC) function. Its deletion in mice causes lethal pancytopenia and HSC depletion. Mechanistically, DCAF13 interacts with RRS1 and mediates its non‐degradative K27‐linked ubiquitination, thereby stabilizing RRS1 to maintain ribosome biogenesis and protein translation ...
Mengke Li, Yuxin Wu, Shuai Zhou, Peipei Tang, Jiaming Wei, Ling Liu, Zhenyi Wang, Deyang Shi, Shengnan Yuan, Qingyu Zhang, Shihui Wang, Qing Zhang, Huan Zhao, Rui Zhang, Yingying Wang, Shiwei Yang, Xiangli Chen, Xiaojing Shi, Guangzhi Liu, Yuwei Zhang, Jinming Li, Xu Dong Zhang, Rick F Thorne, Dongping Wei, Zumin Zhu, Song Chen   +25 more
wiley   +1 more source