Results 31 to 40 of about 2,135 (156)

Neonatal cholestasis is an early liver manifestation of children with acid sphingomyelinase deficiency

BMC Gastroenterology, 2022
Background Patients with acid sphingomyelinase deficiency (ASMD) may be referred to a hepatologist for liver manifestations. This study summarized the liver manifestations of patients with ASMD in the early disease course.
Neng-Li Wang, Jing Lin, Lian Chen, Yi Lu, Xin-Bao Xie, Kuerbanjiang Abuduxikuer, Jian-She Wang   +6 more
doaj   +1 more source

Determination of optimum irrigation scheduling for sorghum in Benna-Tsemay Woreda, Southern Ethiopia

International Journal of Agricultural Research, Innovation and Technology, 2021
Scarcity of water is the most severe constraint for sustainable development of agriculture in arid and semi-arid areas. Hence, novel irrigation water application systems need to be developed so that high crop yield and water productivity per unit of land
TM Lebiso, MM Mada
doaj   +1 more source

Quantification of lysosphingomyelin and lysosphingomyelin-509 for the screening of acid sphingomyelinase deficiency

Orphanet Journal of Rare Diseases, 2022
Background Acid sphingomyelinase deficiency (ASMD) is a lysosomal disorder caused by deficiency of acid sphingomyelinase (ASM) leading to the accumulation of sphingomyelin (SM) in a variety of cell types.
Francyne Kubaski, Alberto Burlina, Danilo Pereira, Camilo Silva, Zackary M. Herbst, Franciele B. Trapp, Kristiane Michelin-Tirelli, Franciele F. Lopes, Maira G. Burin, Ana Carolina Brusius-Facchin, Alice B. O. Netto, Edina Poletto, Tamires M. Bernardes, Gerson S. Carvalho, Ney B. Sorte, Fernanda N. Ferreira, Nilza Perin, Marta R. Clivati, Marnie T. S. de Santana, Sandra F. G. Lobos, Emilia K. E. A. Leão, Marcelo P. Coutinho, Paola V. Pinos, Maria L. S. F. Santos, Debora A. Penatti, Charles M. Lourenço, Giulia Polo, Roberto Giugliani   +27 more
doaj   +1 more source

Olipudase alfa enzyme replacement therapy for acid sphingomyelinase deficiency (ASMD): sustained improvements in clinical outcomes after 6.5 years of treatment in adults

Orphanet Journal of Rare Diseases, 2023
Background Enzyme replacement therapy with olipudase alfa, a recombinant human acid sphingomyelinase (rhASM), is indicated for non-central nervous system manifestations of acid sphingomyelinase deficiency (ASMD) in children and adults.
R. Lachmann, G. Diaz, M. Wasserstein, Nicole M. Armstrong, A. Yarramaneni, Yong Kim, Monica P. Kumar   +6 more
semanticscholar   +1 more source

Case report: The spectrum of SMPD1 pathogenic variants in Hungary

Frontiers in Genetics, 2023
Acid sphingomyelinase deficiency (ASMD) is an autosomal recessive disease caused by biallelic pathogenic variants in the sphingomyelin phosphodiesterase-1 (SMPD1) gene.
Maria Judit Molnar, Maria Judit Molnar, Tamas Szlepak, Tamas Szlepak, Ildikó Csürke, Szendile Loth, Rita Káposzta, Melinda Erdős, Antal Dezsőfi   +8 more
doaj   +1 more source

Adaptation and validation of a scale of self‐efficacy and social support for physical activity in Spanish patients with severe mental disorders

Brain and Behavior, 2020
Background People with severe mental disorders (SMDs) suffer problems of obesity, a sedentary life, and poor physical condition, mainly due to low levels of physical activity.
David Perez‐Cruzado, Elisa Vera‐Garcia, Fermin Mayoral‐Cleries, Juan Vicente Luciano, Antonio Cuesta‐Vargas   +4 more
doaj   +1 more source

24 Development of a suspicion index tool to aid diagnosis of ASMD disease

BMJ Paediatrics Open
Roberto Giugliani, Florian B Lagler, Georg Zimmermann   +2 more
exaly   +2 more sources

Expert opinion on patient journey, diagnosis and clinical monitoring in acid sphingomyelinase deficiency in Turkey: a pediatric metabolic disease specialist's perspective

Frontiers in Pediatrics, 2023
This review by a panel of pediatric metabolic disease specialists aimed to provide a practical and implementable guidance document to assist clinicians in best clinical practice in terms of recognition, diagnosis and management of patients with acid ...
Nur Arslan, Mahmut Coker, Gulden Fatma Gokcay, Ertugrul Kiykim, Halise Neslihan Onenli Mungan, Fatih Ezgu   +5 more
doaj   +1 more source

High-Performance Digital Devices Design by the ASMD-FSMD Technique for Implementation in FPGA

Applied Sciences
The paper presents an application of the ASMD-FSMD technique for designing high-performance digital circuits on the example of an implementation of sequential multipliers in reconfigurable FPGA devices. The method primarily enables multiple operations on
Valery Salauyou, Adam Klimowicz, T. Grześ   +2 more
semanticscholar   +1 more source

EFFECT OF IRRIGATION REGIMES AND BIOFERTILIZERS ON YIELD AND SOME WATER RELATIONS OF SOYBEAN PLANT [PDF]

Journal of Soil Sciences and Agricultural Engineering, 2013
Two field experiments were carried out at El-Karada station of irrigation requirements, Kafr El-sheikh Governorate, during the two summer seasons 2008 &2009, to study the effect of irrigation regimes (irrigating soybean plants at 25, 50 and 75% of ...
A. S. El-Henawy, Iman M. Soltan, M. R. Khalifa   +2 more
doaj   +1 more source