Wilson – Konovalov Disease: Clinical Cases with Different Manifestations and Outcomes
Аim: to describe clinical cases of Wilson – Konovalov disease in pediatric patients.Key points. The first clinical case demonstrates the manifestation of Wilson – Konovalov disease with unexplained mild elevation of aminotransferases at the age of 6 ...
O. V. Samodova +2 more
doaj +1 more source
ABSTRACT Background Therapeutic apheresis (TA) is an established treatment modality for hematologic, neurologic, and immunologic disorders, yet access remains severely limited in sub‐Saharan Africa. Donor apheresis, including platelet apheresis collection from healthy donors, represents an important complementary modality supporting blood product ...
Nosa Bazuaye +33 more
wiley +1 more source
Xenogeneic, extracorporeal liver perfusion in primates improves the ratio of branched-chain amino acids to aromatic amino acids (Fischer's ratio) [PDF]
In fulminant hepatic failure (FHF), the development of hepatic encephalopathy is associated with grossly abnormal concentrations of plasma amino acids (PAA).
Puhl, G. +7 more
core +1 more source
This review focuses on the role of autophagy and mitophagy in maintaining pancreatic β‐cell function and homeostasis. We discuss how genetic defects affecting these pathways contribute to the development of type 1, type 2, monogenic, and gestational diabetes. We further explore their potential as therapeutic targets. Created in BioRender.
Yunkyeong Lee +2 more
wiley +1 more source
Chronobiology of Cancer: How Aging Fuels Oncogenesis at the Molecular Level
This graphical abstract illustrates the key biological pathways linking aging with cancer development and progression. In the upper left, cumulative exposure to ultraviolet radiation, toxins, and reactive oxygen species (ROS) causes DNA damage and genomic instability, whereas age‐related decline in repair mechanisms, such as ATM/ATR, BER, and NER ...
Anu Singh, Aroonima Misra, Sufian Zaheer
wiley +1 more source
Pediatric acute liver failure of undetermined cause: A research workshop [PDF]
Pediatric acute liver failure (PALF) is a potentially devastating condition that occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of
Estella M, Alonso +3 more
openaire +2 more sources
Higher Waitlist Mortality in Pediatric Acute-on-chronic Liver Failure in the UNOS Database
Objectives: Acute-on-chronic liver failure (ACLF), whereas increasingly well-defined in adults, has been poorly characterized in pediatric patients other than having a poor prognosis.
John Goss +11 more
core +1 more source
Onasemnogene Abeparvovec in Patients With SMA: Interim Results of the RESTORE Registry in Japan
ABSTRACT Objective There are limited real‐world data regarding the safety and effectiveness of onasemnogene abeparvovec (OA; Zolgensma) infusion, a one‐time gene replacement therapy, for Japanese patients with spinal muscular atrophy (SMA). We aimed to improve understanding of the real‐world outcomes for OA in Japan.
Kayoko Saito +8 more
wiley +1 more source
Analysis of Viral Testing in Nonacetaminophen Pediatric Acute Liver Failure [PDF]
ABSTRACTObjective:Viral infections are often suspected to cause pediatric acute liver failure (PALF), but large‐scale studies have not been performed. We analyzed the results of viral testing among nonacetaminophen PALF study participants.Methods:Participants were enrolled in the PALF registry.
Kathleen B, Schwarz +8 more
openaire +2 more sources
Acute liver failure in paracetamol overdose: management, transplantation and best practice [PDF]
In the United Kingdom the most common drug taken in overdose is paracetamol, which is recognised as a major cause of acute liver failure. However death rates from acute liver failure have fallen due to the rapid availability and accessibility of the ...
Toplis, Emma, Mortimore, Gerri
core +1 more source

