The Chédiak-Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disorder. Some cases with CHS develop the accelerated phase characterized by pancytopenia, high fever and lymphohistiocytic infiltration of liver, spleen and lymph nodes. The treatment of the accelerated phase of CHS is difficult. We describe a case with CHS in the accelerated phase who had multiple polyposis and pulmonary infiltration that was probably due to involvement of CHS. She was successfully treated with high-dose methylprednisolone at her first admission. At her second admission, splenectomy was performed to remove hypersplenism, and her clinical, radiological and hematological findings improved significantly.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1159/000203725 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Nanoparticle Adjuvants Incorporating Haptens Drive Potent Immune Tolerance to Accelerate Hair Regrowth.
ACS Appl Mater Interfaces
January 2025
Wuya College of Innovation, Shenyang Pharmaceutical University, Shenyang, Liaoning 110016, China.
Alopecia areata (AA) is a prevalent autoimmune condition that causes sudden hair loss and poses significant psychological challenges to affected individuals. Current treatments, including corticosteroids and Janus kinase inhibitors, fail to provide long-term efficacy due to adverse effects and relapse after cessation. This study introduces a nanoparticle (NP) system that codeliver diphenylcyclopropenone (DPCP) and rapamycin (RAPA) prodrugs to induce immune tolerance and promote hair regeneration.
View Article and Find Full Text PDFMultiple Kinase Small Molecule Inhibitor Tinengotinib (TT-00420) Alone or With Chemotherapy Inhibit the Growth of SCLC.
Cancer Sci
January 2025
Department of Medical Oncology, Jilin Cancer Hospital, Changchun, China.
There is an urgent need to develop new targeted treatment agents for small cell lung cancer (SCLC). Tinengotinib (TT-00420) is a novel, multi-targeted, and spectrally selective small-molecule kinase inhibitor that has shown significant inhibitory effects on certain solid tumors in preclinical studies. However, its role and mechanism of action in SCLC remain unclear.
View Article and Find Full Text PDFA parallel Hilbert transform arctangent phase demodulation (PHT-ATAN) method based on overlapping computation is proposed for phase demodulation of laser heterodyne Doppler vibrometers. The method suppresses the end point effects by utilizing overlapping computation and data concatenation and accelerates phase demodulation through parallel processing. Simulation and experimental results demonstrate that when the algorithm's parallelism is ≥4, the computation speed of this method increases by over 100% compared to traditional methods, while maintaining the signal-to-noise ratio and accuracy of the phase demodulation results.
View Article and Find Full Text PDFRefreshable memristor via dynamic allocation of ferro-ionic phase for neural reuse.
Nat Commun
January 2025
School of Optoelectronic Science and Engineering, University of Electronic Science and Technology of China, Chengdu, China.
Neural reuse can drive organisms to generalize knowledge across various tasks during learning. However, existing devices mostly focus on architectures rather than network functions, lacking the mimic capabilities of neural reuse. Here, we demonstrate a rational device designed based on ferroionic CuInPS, to accomplish the neural reuse function, enabled by dynamic allocation of the ferro-ionic phase.
View Article and Find Full Text PDFCombined anti-leukemic effect of gilteritinib and GSK-J4 in FLT3-ITD acute myeloid leukemia.
Transl Oncol
January 2025
The First Clinical Medical College of Lanzhou University, Lanzhou 730000, China; Department of Hematology, The First Hospital of Lanzhou University, Lanzhou 730000, China. Electronic address:
Gilteritinib treats acute myeloid leukemia (AML) with the FMS-like receptor tyrosine kinase-3 (FLT3) internal tandem duplication (ITD) mutation. Dysregulation of histone modification affects the genesis and progression of AML. Strategies targeting key histone regulators have not been applied to the treatment of AML.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!