Gentaur Pub

#39367693   2024/10/05 To Up

The endocrine manifestations of adults with spinal muscular atrophy.

Changes in body composition in patients with spinal muscular atrophy (SMA) can cause endocrine abnormalities that are insufficiently studied in adults. We aimed to assess the endocrine profile in a cohort of adults with SMA. Second, we compared body composition and endocrine profiles between nonambulatory and ambulatory patients and between different types of SMA.
Matej Rakusa, Blaž Koritnik, Lea Leonardis, Katja Goricar, Tjasa Rudolf, Dejan Firbas, Žiga Snoj, Mojca Jensterle

1893 related Products with: The endocrine manifestations of adults with spinal muscular atrophy.

500IU5ugmin 2 cartons100.00 ul30 reactions1200 units11 ml

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#39367607   2024/10/03 To Up

Bone-protective effects of neutralizing angiopoietin-like protein 4 monoclonal antibody in rheumatoid arthritis.

Despite recent advances, rheumatoid arthritis (RA) patients remain refractory to therapy. Dysregulated overproduction of angiopoietin-like 4 protein (ANGPTL4) is thought to be contributed to the disease development. ANGPTL4 was initially identified as a regulator of lipid metabolism, which is hydrolyzed to N-terminal (nANGPTL4) and C-terminal (cANGPTL4) fragments in vivo. cANGPTL4 is involved in several non-lipid-related processes, including angiogenesis and inflammation. The present study revealed that the level of ANGPTL4 was markedly elevated in the sera and synovial tissues from patients with RA versus controls. The administration of a neutralizing antibody against cANGPTL4 (anti-cANGPTL4 Ab) resulted in the inhibition of inflammatory processes and bone loss in animal models of collagen-induced arthritis (CIA) and adjuvant-induced arthritis (AIA). Transcriptomic and proteomic profiling of synovial tissues from AIA model indicated that the anti-cANGPTL4 Ab inhibited fibroblast-like synoviocytes (FLS) immigration and inflammatory-induced osteoclastogenesis. Mechanistically, the anti-cANGPTL4 Ab has been shown to inhibit TNF-α-induced inflammatory cascades in RA-FLS through the sirtuin 1/nuclear factor-κB signaling pathway. Moreover, the anti-cANGPTL4 Ab was found to block FLS invasion- and immigration-induced osteoclast activation. Collectively, these findings identify ANGPTL4 as a prospective biomarker for the diagnosis of RA, and targeting cANGPTL4 may represent a potential therapeutic strategy.
Liqing Ke, Qifei He, Jing Qu, Xiyue Wang, Kaibo Li, Xun Gong, Lan Li, Jiake Xu, Qiuliyang Yu, Hao Yu, Xuefei Lin, Jian Li, Nguan Soon Tan, Wei Sun, Liang Li, Peng Zhang, Wenxiang Cheng

2082 related Products with: Bone-protective effects of neutralizing angiopoietin-like protein 4 monoclonal antibody in rheumatoid arthritis.

100ug100100ug100100ug100100ug1 Set1 Set1 Set1 Set100ug Lyophilized

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#39367497   2024/10/04 To Up

Observation on the effect of reconstruction of the forearm rotation function with an external fixator in the treatment of congenital radioulnar synostosis.

To observe the effect of the forearm rotation function reconstruction with an external fixator in treating congenital radioulnar synostosis.
Yanzhao Dong, Junfang Xu, Feipeng Wang, Yunheng Jia, GuoMing Feng, Yu Liu, Han Xue

2857 related Products with: Observation on the effect of reconstruction of the forearm rotation function with an external fixator in the treatment of congenital radioulnar synostosis.

0.1 mg1 ml

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#39367398   2024/10/04 To Up

Corneal stromal lenticule transplantation for the treatment of congenital optic disc pit maculopathy : a case report and review.

Congenital optic disc pit (ODP) is a relatively uncommon congenital anomaly of the optic disc, which seriously affects the patient's vision when combined with optic disc pit maculopathy(ODP-M). Currently, the treatment of ODP-M remains a clinical challenge and a focus of research.
Xiaojuan Zhang, Gang Qiao, Yu Quan, Yukun He, Hui Jiang

2264 related Products with: Corneal stromal lenticule transplantation for the treatment of congenital optic disc pit maculopathy : a case report and review.

10 mg1.0 mg10 mg

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#39367295   2024/10/04 To Up

Performance of health and wellness centre in providing primary care services in Chhattisgarh, India.

Primary health care has regained its importance in global policy making. In 2018, the Government of India initiated the Ayushman Bharat - Comprehensive Primary Health Care (AB-CPHC) programme. It was based on upgrading the existing primary health facilities into Health and Wellness Centers (HWCs). The current study aimed to assess the readiness and performance of HWCs in providing comprehensive primary health care services in India's Chhattisgarh state.
Narayan Tripathi, Priyanka Parhad, Samir Garg, Silka Shubhadarshini Biswal, Senthilkumar Ramasamy, Animesh Panda, Abhishek Kumar Shastri, Aniruddha Bhargav, Chandrashekhar Bopche, Vahab Ansari, Anjulata Sahu, Rohit Rajput, Anju Gupta, Manisha Gupta, Sanjana Agrawal, Rajesh Sharma, Mohammed Ahmed, Sudipta Ghosh, Jyotiraditya Samrat, Dipti Yadav, Pramita Sharma, Vikash R Keshri

1510 related Products with: Performance of health and wellness centre in providing primary care services in Chhattisgarh, India.

100ug5 mg96 wells (1 kit)100 assays100ug Lyophilized100 μg96T

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#39367272   2024/10/05 To Up

Biallelic and monoallelic variants in EFEMP1 can cause a severe and distinct subtype of heritable connective tissue disorder.

Variants in EFEMP1, encoding Fibulin-3, were previously reported as a rare cause of heritable connective tissue disorder (HCTD) with recurrent hernias and joint hypermobility. We report three new cases with biallelic or monoallelic EFEMP1 variants and severe hernia phenotypes. Two male siblings of 10 and 13 years old presented with marfanoid habitus, recurrent inguinal and umbilical hernias, generalized joint hypermobility, and scoliosis. Parents and halfsiblings reported joint hypermobility and umbilical hernias. The eldest boy died at age 16 from incarcerated gastrointestinal herniation complicated by gastric and bowel necrosis with perforation. Autopsy revealed widespread intestinal diverticula. Immunohistochemistry of skin and fascia tissue did not reveal any abnormalities, including normal staining of elastic fibers. Both siblings harbored compound heterozygous likely pathogenic EFEMP1 variants (c.1320 + 2T > A, p.? and c.698G > A, p.Gly233Asp). An unrelated 58-year-old male had marfanoid features, high myopia, recurrent diaphragmatic and inguinal hernias, and chronic gastrointestinal dilatation with severe malabsorption. Both his dizygotic twin-brother and mother had recurrent hernias and high myopia. This man died at 59 years of age, and autopsy showed extensive diaphragmatic herniation, bowel diverticula, and pulmonary emphysema. A heterozygous EFEMP1 splice-variant (c.81 + 1G > A, p.?) was identified, causing exon skipping leading to a start-loss. Targeted genome reanalysis nor RNA-sequencing revealed a second variant at the other allele. The reported individuals expand the clinical and pathological phenotypes of EFEMP1-related disease, a distinct entity within the spectrum of HCTD. The severe and recurrent hernias, gastrointestinal dilatation, and diverticulosis result in an increased risk for life-threatening complications, demanding early recognition and close monitoring.
M O Mol, T J van Ham, N Bannink, H T Bruggenwirth, J C Escher, J M Kros, J J M Renkens, L van Unen, R M Verdijk, J Vlot, V J M Verhoeven, S Demirdas

1760 related Products with: Biallelic and monoallelic variants in EFEMP1 can cause a severe and distinct subtype of heritable connective tissue disorder.

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#39367221   2024/10/04 To Up

Risk factors associated with late-onset hearing loss in children with congenital cytomegalovirus: a systematic review.

To search for existing evidence of prognostic factors related to the development of late-onset hearing loss (LOHL) in infants with congenital cytomegalovirus (cCMV).
María Fernández-Rueda, Christian Calvo-Henriquez, Rafael Fernández-Liesa, Alfredo García-Fernández, Roberto Pedrero-Tomé, Daniel Blázquez-Gamero, Joaquín De Vergas Guiterrez

1343 related Products with: Risk factors associated with late-onset hearing loss in children with congenital cytomegalovirus: a systematic review.

100 μg96 samples100 1 Set100ug

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#39367123   2024/10/04 To Up

Profibrotic monocyte-derived alveolar macrophages are expanded in patients with persistent respiratory symptoms and radiographic abnormalities after COVID-19.

Monocyte-derived alveolar macrophages drive lung injury and fibrosis in murine models and are associated with pulmonary fibrosis in humans. Monocyte-derived alveolar macrophages have been suggested to develop a phenotype that promotes lung repair as injury resolves. We compared single-cell and cytokine profiling of the alveolar space in a cohort of 35 patients with post-acute sequelae of COVID-19 who had persistent respiratory symptoms and abnormalities on a computed tomography scan of the chest that subsequently improved or progressed. The abundance of monocyte-derived alveolar macrophages, their gene expression programs, and the level of the monocyte chemokine CCL2 in bronchoalveolar lavage fluid positively associated with the severity of radiographic fibrosis. Monocyte-derived alveolar macrophages from patients with resolving or progressive fibrosis expressed the same set of profibrotic genes. Our findings argue against a distinct reparative phenotype in monocyte-derived alveolar macrophages, highlighting their utility as a biomarker of failed lung repair and a potential target for therapy.
Joseph I Bailey, Connor H Puritz, Karolina J Senkow, Nikolay S Markov, Estefani Diaz, Emmy Jonasson, Zhan Yu, Suchitra Swaminathan, Ziyan Lu, Samuel Fenske, Rogan A Grant, Hiam Abdala-Valencia, Ruben J Mylvaganam, Amy Ludwig, Janet Miller, R Ian Cumming, Robert M Tighe, Kymberly M Gowdy, Ravi Kalhan, Manu Jain, Ankit Bharat, Chitaru Kurihara, Ruben San Jose Estepar, Raul San Jose Estepar, George R Washko, Ali Shilatifard, Jacob I Sznajder, Karen M Ridge, G R Scott Budinger, Rosemary Braun, Alexander V Misharin, Marc A Sala

2010 related Products with: Profibrotic monocyte-derived alveolar macrophages are expanded in patients with persistent respiratory symptoms and radiographic abnormalities after COVID-19.

96 tests100 μg1 Setcasecase1 L.100 10mg

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#39367115   2024/10/04 To Up

Pharmacokinetic and pharmacodynamic evaluation of various vasopressin doses and routes of administration in a neonatal piglet model.

E
Marwa Ramsie, Po-Yin Cheung, Megan O'Reilly, Tze-Fun Lee, Georg M Schmölzer

2818 related Products with: Pharmacokinetic and pharmacodynamic evaluation of various vasopressin doses and routes of administration in a neonatal piglet model.

10 mg100ug500 MG25 mg100ug96T1 ml50 mg25 mg100ul100ul10 mg

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