Results 81 to 90 of about 24,959 (186)

Case Report: Early-onset portal hypertension revealing pediatric ADPKD with a truncating PKD1 variant

Frontiers in Pediatrics
BackgroundAutosomal dominant polycystic kidney disease (ADPKD) is a multisystemic ciliopathy whose clinical spectrum extends into childhood. Although hepatic cysts are the most common extrarenal manifestation, early portal hypertension associated with ...
Ana Katerin Minota Idarraga, Ana Katerin Minota Idarraga, Ivan Mauricio Llanos Arango   +2 more
doaj   +1 more source

Oral Lichenoid Lesions in Patients With Dyskeratosis Congenita: A Retrospective Case Series

Oral Diseases, EarlyView.
ABSTRACT Background Dyskeratosis congenita (DKC) is a rare inherited multisystem disorder primarily affecting the mucocutaneous and hematopoietic systems. Classically, it presents with the ectodermal triad of reticulate skin pigmentation, nail dystrophy, and oral leukoplakia.
Yehuda Zadik, Ori Finfter, Tevel Amiel, Amina Massarwa, Refael Cohen, Jawad Abu Tair, Anna Novikov, Polina Stepensky, Irina Zaidman, Ehud Even‐Or   +9 more
wiley   +1 more source

Combination of Pirfenidone and Andrographolide Ameliorates Hepatic Stellate Cell Activation and Liver Fibrosis by Mediating TGF-β/Smad Signaling Pathway

Analytical Cellular Pathology
Background. Biliary atresia (BA) is a devastating congenital disease characterized by inflammation and progressive liver fibrosis. Activation of hepatic stellate cells (HSCs) plays a central role in the pathogenesis of hepatic fibrosis.
Guang Xu, Tidong Ma, Chonggao Zhou, Fan Zhao, Kun Peng, Bixiang Li   +5 more
doaj   +1 more source

[Clinical and pathological features of 20 cases of congenital hepatic fibrosis]. [PDF]

Zhonghua Gan Zang Bing Za Zhi, 2023
Wang A, Lu ZJ, Gu XF, Liu JP, Lu CL.
europepmc   +1 more source

Malignant Transformation in Oral Potentially Malignant Disorders

Oral Diseases, EarlyView.
ABSTRACT Background In 2020, the World Health Organization (WHO) Collaborating Centre for Oral Cancer convened an international expert workshop to establish an updated consensus on the nomenclature and classification of oral potentially malignant disorders (OPMDs).
Miguel Ángel González‐Moles, Pablo Ramos‐García, Saman Warnakulasuriya   +2 more
wiley   +1 more source

Congenital Hepatic Fibrosis in an Aborted Holstein-Friesian Fetus

Acta Veterinaria
Congenital hepatic fibrosis (CHF) is a rare condition characterized by abnormal accumulation of fibrous tissue in the liver, leading to liver dysfunction.
Savić Božidar, Milovanović Bojan, Stanojević Slobodan, Kureljušić Branislav, Jezdimirović Nemanja, Levakov Aleksandra Fejsa   +5 more
doaj   +1 more source

Congenital Hepatic Fibrosis Presenting With Pancytopenia. [PDF]

JPGN Rep, 2021
Mankala VM, Davis JL, Patel CV, Lin HC.
europepmc   +1 more source

Born early, age fast: Consequences of premature birth on chronic disease and accelerated ageing

The Journal of Physiology, EarlyView.
Abstract figure legend ELGANs are exposed to several postnatal pro‐oxidant stressors, including ambient and supplemental oxygen, mechanical ventilation, infections, hyperalimentation, excessive glucocorticoids and intermittent hypoxia. Since endogenous antioxidant defences are underdeveloped, this imbalance promotes oxidative stress and inflammation ...
Estelle B. Gauda, Julijana Ivanovska, Dwayne Mascarenhas, Jeffrey Antwi, Atefeh Mohammadi, Bonny Jasani   +5 more
wiley   +1 more source

Cannabinoid exposure during pregnancy: Cardiorespiratory effects and offspring outcomes

The Journal of Physiology, EarlyView.
Abstract figure legend Prenatal exposure to cannabinoids has been investigated across human and animal studies to understand its impact on physiological development. Evidence suggests that early‐life cannabinoid exposure influence multiple developmental processes, extending beyond neurodevelopmental outcomes to potentially affect placental function ...
Luis Gustavo A. Patrone, Luciane H. Gargaglioni   +1 more
wiley   +1 more source

Efficacy and Safety of SGLT2 Inhibitors in Patients With Genetic Lipodystrophy: A Real‐Life Experience From a National Reference Network

Diabetes, Obesity and Metabolism, Volume 28, Issue 11, Page 10318-10329, November 2026.
ABSTRACT Aim Lipodystrophy syndromes are rare diseases with metabolic and cardiovascular consequences. This study explores the metabolic and renal effects of sodium‐glucose cotransporter 2 inhibitors (SGLT2i) in patients with genetic lipodystrophy. Patients and Methods Patients with familial partial lipodystrophy (n = 57) or congenital generalised ...
Léna Robert, Ines Belalem, Corinne Vigouroux, Hippolyte Dupuis, Marie Christine Vantyghem, Mathilde Simonson, Estelle Nobecourt, Héléna Mosbah, Sophie Beliard, Stéphanie Jellimann, Sonja Janmaat, Camille Vatier, Sophie Lamothe   +12 more
wiley   +1 more source