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[Securing the airway in children with the Morquio-Brailsford syndrome].

Mucopolysaccharidosis IVA (Morquio-Brailsford syndrome) results from an inborn deficiency of n-acetyl-galactosamine-6-sulphate sulphatase. Clinical features include skeletal deformities with hypoplasia or absence of the odontoid process of the axis. The resulting atlanto-axial subluxation compresses the spinal cord, resulting in cervical myelopathy. Without treatment, quadriplegia ensues sooner or later; consequently, surgical decompression and dorsal fusion of the cervical vertebrae is recommended, either prophylactically or therapeutically. Anaesthesiological management must focus on protect…

A second reported malignancy in a patient with Morquio syndrome.

Morquio syndrome is a rare lysosomal storage disease that affects multiple organ systems. However, it is rarely associated with malignancy. We present the case of a 30-year old man with Morquio syndrome associated with gastric adenocarcinoma. This case also demonstrates two other findings that have not been previously described in patients with Morquio syndrome - malrotation of brainstem and cerebellum, without clinical neurologic deficit, and persistence of fetal lobulation in the kidneys.

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… Type IVA is caused by the deficiency of enzyme N-acetyl-galactosamine-6-sulfatase causing intracellular accumulation of keratan sulfate and chondroitin 6-sulfate whereas type IVB is caused by β-galactosidase deficiency causing accumulation of keratan sulfate only. 1 Morquio patients can present with … Type IVA is caused by deficiency of enzyme N-acetyl-galactosamine-6-sulfatase and type IVB is caused by β-galactosidase deficiency. 1 Clinical presentation and other features of Morquio syndrome are summarized in Table 1 . …

The Health-Promoting Properties and Clinical Applications of Rice Bran Arabinoxylan Modified with Shiitake Mushroom Enzyme-A Narrative Review.

Rice bran arabinoxylan compound (RBAC) is derived from defatted rice bran hydrolyzed with Lentinus edodes mycelial enzyme. It has been marketed as a functional food and a nutraceutical with health-promoting properties. Some research has demonstrated this rice bran derivative to be a potent immunomodulator, which also possesses anti-inflammatory, antioxidant, and anti-angiogenic properties. To date, research on RBAC has predominantly focused on its immunomodulatory action and application as a complementary therapy for cancer. Nonetheless, the clinical applications of RBAC can extend beyond canc

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… In an in vivo study by Zheng et al. [ 79 ], male Wistar rats were injected with D-galactosamine to induce liver injury similar to human hepatitis B. Rats were pre-treated with RBAC at 20 mg/kg body weight or 60 mg/kg body weight, intraperitoneally. … Further experiments also show that the suppressive effects of RBAC on hepatitis induced by D-galactosamine are related to the inhibition of nuclear factor- κ B, mitogen-activated protein kinase, and CD14 expression [ 80 ]. …

Immunohistochemical detection of sepsis-induced lung injury in human autopsy material.

This review addresses our present-day knowledge on the role of different cellular adhesion molecules, cytokines and glycoproteins for the de … ent expression for ConA, MAA and SNA), the main finding is that no binding sites for alpha-N-acetyl-galactosamine (as investigated by MPA immunoreactivity) can be detected on alveolar epithelial cells and mucous parts of subepithelial seromucous glands in sepsis cases in contrast to the presence of such binding sites in controls. Since most intracellular pathog …

Экспериментальные и клинические исследования фармакологических действий рода ахиллия: всеобъемлющий и обновленный обзор.

ЦЕЛЬ: Виды рода Ахиллеа (от семейства Compositae или Asteraceae) широко используются для их многочисленных фармакологических свойств. В настоящем документе рассматриваются фармакологические действия и их возможные основополагающие молекулярные механизмы, о которых сообщается в отношении различных видов ахиллы. МАТЕРИАЛЫ И МЕТОДЫ: использовались различные базы данных, включая PubMed, Science Direct и Scopus. Результаты: Для этих растений были продемонстрированы эффекты иммуноспрепрессии, против воспламенения и против окислителей. Кроме того, было продемонстрировано, что эти растения оказывают н

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… Hepatoprotective effects Pre-treatment of mice with crude extract of A. millefolium prevented D-galactosamine and lipopolysaccharide (LPS)-induced rise in plasma alanine aminotransferase (ALT) and aspartate aminotransferase (AST). … The extract improved architecture, parenchymal congestion, cellular swelling and apoptosis, indicating that the hepatoprotective effect of A. millefolium against D-galactosamine and LPS-induced hepatitis, through possible calcium channel blocking activity (Yaeesh et al., 2006). …

Recent Advances in Doxorubicin Formulation to Enhance Pharmacokinetics and Tumor Targeting.

Doxorubicin (DOX), a widely used drug in cancer chemotherapy, induces cell death via multiple intracellular interactions, generating reactive oxygen species and DNA-adducted configurations that induce apoptosis, topoisomerase II inhibition, and histone eviction. Despite its wide therapeutic efficacy in solid tumors, DOX often induces drug resistance and cardiotoxicity. It shows limited intestinal absorption because of low paracellular permeability and P-glycoprotein (P-gp)-mediated efflux. We reviewed various parenteral DOX formulations, such as liposomes, polymeric micelles, polymeric nanopar

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… FCE28069/PK2 is an HPMA polymer–DOX conjugate linked to a galactosamine structure, which binds to the hepatic asialoglycoprotein receptor. It was designed for treating primary liver cancer. … , IV | FCE28069/PK2 Phase II | Galactosamine-mediated uptake Liver-specific delivery using galactosamine-modified polymers, and a partial response was achieved in patients with liver cancer Grade 4 neutropenia and grade 3 mucositis | [ 57 ] 3. …

Case report: Successful treatment of a patient presenting with a very rare association of acute lymphoblastic leukemia and mucopolysaccharidosis type IVA.

Treating Acute Lymphoblastic Leukemia (ALL) in patients with genetic disorders poses significant challenges for onco-hematologists. Mucopolysaccharidosis type IVA (MPS-IVA) is a lysosomal storage disorder that clinically manifests with progressive and multi-systemic comorbidities, primarily affecting the bone, cartilage, spine, heart and lungs. We report a unique case of B-lineage ALL in a patient with MPS-IVA, who was successfully cured with a personalized chemo-radiotherapy approach. The treatment strategy aimed to balance a curative-intent chemotherapy attempt with the minimization of life-

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… MPS-IVA is a lysosomal storage disorder caused by mutations in the GALNS gene ( NM_000512.5 ), which leads to a deficiency of the enzyme N-acetyl-galactosamine-6-sulfate sulfatase (GALNS) and the accumulation of glycosaminoglycans (GAG) keratan sulfate and chondroitin 6-sulfate. …

Osteoarthritis of the knee - biochemical aspect of applied therapies: a review.

The most prevalent form of arthritis is osteoarthritis (OA) of the knee, which is characterized by a degeneration of articular cartilage resulting in the development of osteophytes, or bone spurs. Main goals of OA treatment are to reduce pain, slow the disease progression, and improve joint function and the quality of life. The purpose of this study was to verify all the therapies recommended by the European Society for Clinical and Economic Aspects of Osteoporosis and Osteoarthritis (ESCEO) from the biochemical point of view. Non-steroidal anti-inflammatory drugs (NSAIDs) inhibit the synthesi

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… Chondroitin sulfate, the main component of extracellular matrix (ECM) is an unbranched complex polysaccharide consisting of glucuronic acid and N-acetyl-D-galactosamine. Its crucial role is to create osmotic pressure [ 51 ]. …

Oroxylin A: A Promising Flavonoid for Prevention and Treatment of Chronic Diseases.

There have been magnificent advancements in the understanding of molecular mechanisms of chronic diseases over the past several years, but these diseases continue to be a considerable cause of death worldwide. Most of the approved medications available for the prevention and treatment of these diseases target only a single gene/protein/pathway and are known to cause severe side effects and are less effective than they are anticipated. Consequently, the development of finer therapeutics that outshine the existing ones is far-reaching. Natural compounds have enormous applications in curbing seve

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… Similarly, another study was conducted to identify the effects of OA against LPS and/or D-galactosamine-induced acute liver injury in mice. …

Trials and Tribulations of MicroRNA Therapeutics.

The discovery of the link between microRNAs (miRNAs) and a myriad of human diseases, particularly various cancer types, has generated significant interest in exploring their potential as a novel class of drugs. This has led to substantial investments in interdisciplinary research fields such as biology, chemistry, and medical science for the development of miRNA-based therapies. Furthermore, the recent global success of SARS-CoV-2 mRNA vaccines against the COVID-19 pandemic has further revitalized interest in RNA-based immunotherapies, including miRNA-based approaches to cancer treatment. Cons

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… RG-101: RG-101, an antagomir of miR-122 developed by Regulus Therapeutics, is an N-acetyl-d-galactosamine(GalNAc)-conjugated synthetic RNA oligonucleotide that targets and inhibits miR-122, which is involved in HCV replication for patients with HCV. …

Recognition and management of persistent chylomicronemia: A Joint Expert Clinical Consensus by the National Lipid Association and the American Society for Preventive Cardiology.

Extreme hypertriglyceridemia, defined as triglyceride (TG) levels ≥1000 mg/dL, is almost always indicative of chylomicronemia. The current diagnostic approach categorizes individuals with chylomicronemia into familial chylomicronemia syndrome (FCS; prevalence 1-10 per million), caused by the biallelic combination of pathogenic variants that impair the lipolytic action of lipoprotein lipase (LPL), or multifactorial chylomicronemia syndrome (MCS, 1 in 500). A pragmatic framework should emphasize the severity of the phenotype and the risk of complications. Therefore, we endorse the term "persiste

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… Gaudet D , Karwatowska-Prokopczuk E , Baum SJ , Vupanorsen, an N-acetyl galactosamine-conjugated antisense drug to ANGPTL3 mRNA, lowers triglycerides and atherogenic lipoproteins in patients with diabetes, hepatic steatosis, and hypertriglyceridaemia . …

Targeting the Endocannabinoidome in Pancreatic Cancer.

Pancreatic Ductal adenocarcinoma (PDAC), the most common malignancy of the pancreas, is an aggressive and lethal form of cancer with a very high mortality rate. High heterogeneity, asymptomatic initial stages and a lack of specific diagnostic markers result in an end-stage diagnosis when the tumour has locally advanced or metastasised. PDAC is resistant to most of the available chemotherapy and radiation therapy treatments, making surgery the most potent curative treatment. The desmoplastic tumour microenvironment contributes to determining PDAC pathophysiology, immune response and therapeutic

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… Protective role of cannabinoid receptor 2 activation in galactosamine/lipopolysaccharide-induced acute liver failure through regulation of macrophage polarization and microRNAs. J. Pharmacol. Exp. Ther. 2015;353:369–379. doi: 10.1124/jpet.114.220368. 69. Glogauer J., Blay J. …

Recognition and management of persistent chylomicronemia: A joint expert clinical consensus by the National Lipid Association and the American Society for Preventive Cardiology.

Extreme hypertriglyceridemia, defined as triglyceride (TG) levels ≥1000 mg/dL, is almost always indicative of chylomicronemia. The current diagnostic approach categorizes individuals with chylomicronemia into familial chylomicronemia syndrome (FCS; prevalence 1-10 per million), caused by the biallelic combination of pathogenic variants that impair the lipolytic action of lipoprotein lipase (LPL), or multifactorial chylomicronemia syndrome (MCS, 1 in 500). A pragmatic framework should emphasize the severity of the phenotype and the risk of complications. Therefore, we endorse the term "persiste

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… Vupanorsen, an N-acetyl galactosamine-conjugated antisense drug to ANGPTL3 mRNA, lowers triglycerides and atherogenic lipoproteins in patients with diabetes, hepatic steatosis, and hypertriglyceridaemia Eur Heart J 41 40 2020 3936 3945 10.1093/eurheartj/ehaa689 32860031 PMC7750927 90 Bhatt D.L. …

TGF-β in hematologic malignancies: molecular functions and clinical applications.

Transforming growth factor-β (TGF-β) represents a family of multifunctional cytokines, primarily secreted by megakaryocytes, monocytes, T lymphocytes, bone marrow stromal cells, and other cell types. TGF-β plays an essential role in various physiological processes, including the regulation of cell proliferation, differentiation, apoptosis, and immune homeostasis. As a key immunoregulatory cytokine, TGF-β contributes to an immunosuppressive network within the microenvironment of hematologic malignancies by modulating the functions of both adaptive and innate immune cells. Current studies have s

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… TAMs acquire their characteristic phenotype by expressing high levels of TGF-β, IL-10, macrophage galactose N−acetyl−galactosamine−specific lectin 1 (MGL1), Dectin−1, CXCL10, CXCL9, and other interferon−responsive genes. …

The Therapeutic Potential of Secreted Factors from Dental Pulp Stem Cells for Various Diseases.

An alternative source of mesenchymal stem cells has recently been discovered: dental pulp stem cells (DPSCs), including deciduous teeth, which can thus comprise potential tools for regenerative medicine. DPSCs derive from the neural crest and are normally implicated in dentin homeostasis. The clinical application of mesenchymal stem cells (MSCs) involving DPSCs contains various limitations, such as high cost, low safety, and cell handling issues, as well as invasive sample collection procedures. Although MSCs implantation offers favorable outcomes on specific diseases, implanted MSCs cannot su

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… Favorable factors included within the DPSC-CM and endogenous tissue-repairing factors induced by DPSC-CM therapeutic function in combination attenuate D-galactosamine-induced ALF or carbon tetrachloride-induced LF. 7. …

Mesenchymal Stromal Cell Therapy in Solid Organ Transplantation.

Transplantation is the gold-standard treatment for the failure of several solid organs, including the kidneys, liver, heart, lung and small bowel. The use of tailored immunosuppressive agents has improved graft and patient survival remarkably in early post-transplant stages, but long-term outcomes are frequently unsatisfactory due to the development of chronic graft rejection, which ultimately leads to transplant failure. Moreover, prolonged immunosuppression entails severe side effects that severely impact patient survival and quality of life. The achievement of tolerance, i.e., stable graft

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… AMSC-derived exosomes alleviate lipopolysaccharide/d-galactosamine-induced acute liver failure by miR-17-mediated reduction of TXNIP/NLRP3 inflammasome activation in macrophages. EBioMedicine (2018) 36:140–50. 10.1016/j.ebiom.2018.08.054 82. …

What is new in the pathogenesis and treatment of IgA glomerulonephritis.

Recently, new findings have been clarified concerning both pathogenesis and treatment of IgA nephritis. The four hits theory has been confirmed but several genetic wide association studies have allowed finding several genes connected with the pathogenesis of the disease. All these new genes apply to each of the four hits. Additionally, new discoveries concerning the microbiota and its connection with immune system and IgA generation have allowed finding out the role of the mucosa in IgA nephropathy pathogenesis. The IgA treatment is also changed included the future possibilities. The treatment

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… Each O-glycan chain is based on a core N-acetyl galactosamine unit that is O-linked to serine or threonine. Moreover, abnormalities in O-glycosylation represent hit 2 of the development of IgA nephritis. The interactions of different pathogenic elements are required to produce IgAN. …

Causes, clinical findings and therapeutic options in chylomicronemia syndrome, a special form of hypertriglyceridemia.

The prevalence of hypertriglyceridemia has been increasing worldwide. Attention is drawn to the fact that the frequency of a special hypertriglyceridemia entity, named chylomicronemia syndrome, is variable among its different forms. The monogenic form, termed familial chylomicronemia syndrome, is rare, occuring in 1 in every 1 million persons. On the other hand, the prevalence of the polygenic form of chylomicronemia syndrome is around 1:600. On the basis of the genetical alterations, other factors, such as obesity, alcohol consumption, uncontrolled diabetes mellitus and certain drugs may sign

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… Gaudet D Karwatowska-Prokopczuk E Baum SJ Hurh E Kingsbury J Bartlett VJ Figueroa AL Piscitelli P Singleton W Witztum JL Geary RS Tsimikas S O'Dea LSL the Vupanorsen Study Investigators Vupanorsen, an N-acetyl galactosamine-conjugated antisense drug to ANGPTL3 mRNA, lowers triglycerides and atherogenic …

Dietary Polyphenols and Their Biological Significance

Dietary polyphenols represent a wide variety of compounds that occur in fruits, vegetables, wine, tea, extra virgin olive oil, chocolate and other cocoa products. They are mostly derivatives and/or isomers of flavones, isoflavones, flavonols, catechins and phenolic acids, and possess diverse biological properties such as antioxidant, antiapoptosis, anti-aging, anticarcinogen, anti-inflammation, anti-atherosclerosis, cardiovascular protection, improvement of the endothelial function, as well as inhibition of angiogenesis and cell proliferation activity. Most of these biological actions have bee

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… The cytoprotective effect of alpha-tocopherol and daidzein against d-galactosamine-induced oxidative damage in the rat liver Metabolism 2007 56 865 875 17570244 10.1016/j.metabol.2007.01.005 180 Kuzu N. Metin K. Dagli A.F. Akdemir F. Orhan C. Yalniz M. Ozercan I.H. Sahin K. Bahcecioglu I.H. …

CoREST in pieces: Dismantling the CoREST complex for cancer therapy and beyond.

Several landmark studies over the past decade have uncovered a critical role of the CRL3KBTBD4 ubiquitin ligase complex in regulating stability of corepressor of repressor element 1 silencing transcription factor (CoREST) complex proteins and normal hematopoietic stem cell self-renewal. There is now mounting evidence that the CoREST complex plays oncogenic roles, although the contributions of its catalytic versus noncatalytic functions remain unclear. Here, we summarize and discuss mechanisms whereby the CoREST complex coopts tissue-specific transcription factors to elicit pathogenic activity

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… The LSD1 inhibitor GSK-LSD1 was also been shown to counteract oxidative stress and acute liver injury caused by lipopolysaccharide/ d -galactosamine exposure in mice ( 189 ). …