نتایج جستجو برای: creatine deficiency syndrome

تعداد نتایج: 748934  

2017
Jessie M. Cameron Valeriy Levandovskiy Wendy Roberts Evdokia Anagnostou Stephen Scherer Alvin Loh Andreas Schulze

Creatine deficiency syndrome (CDS) comprises three separate enzyme deficiencies with overlapping clinical presentations: arginine:glycine amidinotransferase (GATM gene, glycine amidinotransferase), guanidinoacetate methyltransferase (GAMT gene), and creatine transporter deficiency (SLC6A8 gene, solute carrier family 6 member 8). CDS presents with developmental delays/regression, intellectual di...

2017
Patrick Cherin Christophe de Jaeger Jean-Charles Crave Jean-Christophe Delain Abir Tadmouri Zahir Amoura

BACKGROUND Antisynthetase syndrome is a rare and debilitating multiorgan disease characterized by inflammatory myopathy, interstitial lung disease, cutaneous involvement, and frequent chronic inflammation of the joints. Standard treatments include corticosteroids and immunosuppressants. In some cases, treatment resistance may develop. Administration of immunoglobulins intravenously is recommend...

Journal: :iranian journal of allergy, asthma and immunology 0
mohammad salehi sadaghiani research center for immunodeficiencies, pediatrics center of excellence, children's medical center, tehran university of medical sciences, tehran, iran asghar aghamohammadi research center for immunodeficiencies, pediatrics center of excellence, children's medical center, tehran university of medical sciences, tehran, iran mahmoud-reza ashrafi department of pediatrics, pediatrics center of excellence, children's medical center, tehran university of medical sciences, tehran, iran firozeh hosseini research center for immunodeficiencies, pediatrics center of excellence, children's medical center, tehran university of medical sciences, tehran, iran hassan abolhassani research center for immunodeficiencies, pediatrics center of excellence, children's medical center, tehran university of medical sciences, tehran, iran nima rezaei research center for immunodeficiencies, pediatrics center of excellence, children's medical center, tehran university of medical sciences, tehran, iran and molecular immunology research center, department of immunology, school of medicine, tehran university of medical sciences, tehran, iran

autism is a neurodevelopmental disorder, characterized by poor social interaction and communication impairment and repetitive behavior. autism is considered as a genetic and multifactorial disorder, with diverse risk factors involved. herein, we report a 13-year-old male with common variable immunodeficiency (cvid), who was diagnosed with autism at the age of 3 years old. as  there  are  some  ...

Journal: :iranian journal of pathology 2013
sharique ahmad sufia ahmad khan shivam shingla shirish bhatnagar kabeer ahmad khan

the hallmarks of leukocyte adhesion deficiency (lad) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. these molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. over las...

Journal: :Genetika 2023

Cerebral Creatine Deficiency Syndromes (CCDS) are congenital metabolic disorders in the creatine metabolism pathway. In this study, we evaluated clinical, phenotypic, radiological and genetic features of patients with CCDS. We tried to identify early diagnosis clues patients. Especially, reviewed causes delay late diagnosis. line these findings, is confirmed by enzyme tests next generation sequ...

Journal: :Pediatrics 2016
Andreas Schulze Margaret Bauman Anne Chun-Hui Tsai Ann Reynolds Wendy Roberts Evdokia Anagnostou Jessie Cameron Alixandra A Nozzolillo Shiyi Chen Lianna Kyriakopoulou Stephen W Scherer Alvin Loh

BACKGROUND AND OBJECTIVE Creatine deficiency may play a role in the neurobiology of autism and may represent a treatable cause of autism. The goal of the study was to ascertain the prevalence of creatine deficiency syndromes (CDSs) in children with autism spectrum disorder (ASD). METHODS In a prospective multicenter study, 443 children were investigated after a confirmed diagnosis of ASD. Ran...

Journal: :Neurology 2008
F-G Debray Y Boulanger A Khiat J-C Decarie J Orquin M-S Roy A Lortie F Ramos N M Verhoeven E Struys H J Blom C Jakobs E Levy G A Mitchell M Lambert

OBJECTIVE To investigate whether secondary impairment of the transmethylation pathway is a mechanism underlying the neurologic involvement in homocystinuria due to remethylation defects. METHODS Twelve patients with neurologic disease due to remethylation defects were examined by brain magnetic resonance spectroscopic imaging ((1)H MRSI). Brain N-acetylaspartate, choline-containing compounds ...

Journal: :Food Science and Nutrition 2023

Abstract Dietary creatine has been recently put forward as a possible intervention strategy to reduce post‐COVID‐19 fatigue syndrome yet no clinical study so far evaluated its efficacy and safety for this perplexing condition. In parallel‐group, randomized placebo‐controlled double‐blind trial, we analyzed the effects of 6‐month supplementation (4 g monohydrate per day) on various patient‐ clin...

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