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The role of lysosomal acid lipase deficiency in dyslipidemia and liver disorders.
Rev Esp Enferm Dig
January 2025
Digestive Diseases, Hospital Universitario Virgen de las Nieves, España.
Cholesterol ester storage disease (CESD) is a rare autosomal recessive lysosomal storage disorder caused by mutations in the LIPA gene, leading to reduced lysosomal acid lipase activity, cholesterol ester accumulation, and systemic manifestations including liver dysfunction and dyslipidemia. We report the case of a 25-year-old male presenting with subacute jaundice, hyperbilirubinemia (total bilirubin 51 mg/dL, predominantly direct), and dyslipidemia characterized by elevated total cholesterol and low HDL cholesterol levels. Initial diagnostic workup for acute hepatitis and liver dysfunction, including serological and imaging studies, was unremarkable.
View Article and Find Full Text PDFMultiscale interstitial fluid computation modeling of cortical bone to characterize the hydromechanical stimulation of lacunar-canalicular network.
Bone
December 2024
College of Biomedical Engineering, Taiyuan University of Technology, Taiyuan, Shanxi, China.
Bone tissue is a biological composite material with a complex hierarchical structure that could continuously adjust its internal structure to adapt to the alterations in the external load environment. The fluid flow within bone is the main route of osteocyte metabolism, and the pore pressure as well as the fluid shear stress generated by it are important mechanical stimuli perceived by osteocytes. Owing to the irregular multiscale structure of bone tissue, the fluid stimulation that lacunar-canalicular network (LCN) in different regions of the tissue underwent remained unclear.
View Article and Find Full Text PDFHistochemical assessment of the femora of Spontaneously Diabetic Torii-Lepr (SDT-fa/fa) rats that mimic type II diabetes.
J Oral Biosci
December 2024
Oral Functional Prosthodontics.
Objective: To elucidate the mechanisms underlying diabetic osteoporosis, we conducted a comprehensive histological examination of the femora of Spontaneously Diabetic Torii-Lepr (SDT-fa/fa) rats, an established model of obesity-related type 2 diabetes.
Materials And Methods: Femora from 12 30-week-old male SDT-fa/fa rats and age-matched Sprague-Dawley (SD) rats (controls) were used for detailed histochemical analyses, including tartrate-resistant acid phosphatase (TRAP), cathepsin K, alkaline phosphatase (ALP), phosphoethanolamine/ phosphocholine phosphatase 1 (PHOSPHO1), dentin matrix protein (DMP)-1, matrix extracellular phosphoglycoprotein (MEPE), sclerostin, osteocalcin staining, silver impregnation, von Kossa staining, and micro-computed tomography (CT).
Results: Micro-CT and hematoxylin-eosin staining demonstrated significantly reduced trabecular bone volume in the femoral metaphyses of SDT-fa/fa rats.
Unveiling surfactant protein-A dynamics in human fetal lung development: histological and immunohistochemical insights from Myanmar.
Folia Morphol (Warsz)
December 2024
School of Basic Medical Sciences, Xi'an Jiaotong University Health Science Center, Xi'an, China.
Background: Surfactant protein-A (SP-A) is the most prevalent protein in the pulmonary surfactant system and it is expressed in Type II alveolar epithelial cells.
Materials And Methods: We evaluated SP-A expression in 92 fetal human lungs at various gestational ages in Myanmar (Burma) using hematoxylin and eosin staining and immunohistochemical assays.
Results: We detected tubular structures in the fetal lungs during the canalicular stage of development at gestational weeks 22-25.
Surgical outcomes and management of complications of StopLoss Jones tube: a single-center study.
Int Ophthalmol
December 2024
Department of Ophthalmology, Division of Oculoplastic and Lacrimal Surgery Unit, Erciyes University Medical Faculty, Kayseri, Türkiye.
Purpose: We evaluated the surgical outcomes and management of complications associated with the minimally invasive conjunctivodacryocystorhinostomy using the StopLoss Jones (SLJT) tube of proximal lacrimal canal obstruction.
Methods: The study retrospectively included 22 eyes of 15 patients who underwent SLJT for proximal canalicular obstruction. Age, gender, follow-up duration, etiology of canalicular obstruction, previous surgery, tube size, complications, and the need for additional surgical intervention were assessed.
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