نتایج جستجو برای: congenital metabolic disorders

تعداد نتایج: 966749  

Journal: :iranian journal of child neurology 0
abolfazl movafagh phd,associate professor, department of medical genetics, shahid beheshti university of medical sciences (sbmu), tehran, iran fatemeh saffari md,associate professor, department of medical genetics, shahid beheshti university of medical sciences (sbmu), tehran, iran ghesmat mohamadzadeh md,assistant professor of pediatric endocrinology, metabolic disease research center, qazvin university of medical science, qazvin, iran marjan shakiba assistant professor of pediatric endocrinology, shahid beheshti university of medical sciences, tehran, iran

objective deficient enzyme activity may cause congenital metabolic defects. these defectsare inherited in an autosomal recessive, autosomal dominant, and x-linkedpatterns. this study was aimed at investigating the occurrence of metabolicdiseases in qazvin province. materials & methods this cross-sectional study was performed on 79,100 children aged 12 years orless between 2000 and 2010. clinica...

پایان نامه :دانشگاه آزاد اسلامی - دانشگاه آزاد اسلامی واحد علوم دارویی - پژوهشکده علوم 1392

purpose a metabolic abnormality such as obesity is a major obstacle in the maintenance of the human health system and causes various chronic diseases including type 2 diabetes, hypertension, cardiovascular diseases, as well as various cancers. this study was designed to summarize the recent scientific knowledge regarding the anti-obesity role of curcumin (diferuloylmethane), which is isolated f...

2012
Bob Z. Wang Pradeep Siriwardana Deepa Taranath

Congenital disorders of glycosylation are a rare group of metabolic disorders that can result in multiorgan disease. This article describes a novel finding of macular hypoplasia in congenital disorders of glycosylation type Ia.

Journal: :Gaceta sanitaria 2010
Isabel Río Adela Castelló Mireia Jané Ramón Prats Carmen Barona Rosa Más Marisa Rebagliato Oscar Zurriaga Francisco Bolúmar

OBJECTIVE To assess the quality of data on births in the Natural Population Movement (NPM) and congenital metabolic disorders registers with regard to calculation of reproductive and perinatal health indicators. METHODS The following comparisons between registers were made: (1) the total number of births to mothers living in Catalonia and Valencia from 2005 to 2006, (2) the percentage of miss...

Journal: :Brain : a journal of neurology 2010
Eva Morava Ron A Wevers Vincent Cantagrel Lies H Hoefsloot Lihadh Al-Gazali Jeroen Schoots Arno van Rooij Karin Huijben Connie M A van Ravenswaaij-Arts Marjolein C J Jongmans Jolanta Sykut-Cegielska Georg F Hoffmann Peter Bluemel Maciej Adamowicz Jeroen van Reeuwijk Bobby G Ng Jorieke E H Bergman Hans van Bokhoven Christian Körner Dusica Babovic-Vuksanovic Michel A Willemsen Joseph G Gleeson Ludwig Lehle Arjan P M de Brouwer Dirk J Lefeber

Cerebellar hypoplasia and slowly progressive ophthalmological symptoms are common features in patients with congenital disorders of glycosylation type I. In a group of patients with congenital disorders of glycosylation type I with unknown aetiology, we have previously described a distinct phenotype with severe, early visual impairment and variable eye malformations, including optic nerve hypop...

Journal: :avicenna journal of medical biochemistry 0
maryam esfahani department of clinical biochemistry, faculty of pharmacy, isfahan university of medical sciences, isfahan, ir iran mostafa baranchi department of physical education and sports sciences, tarbiat modares university, tehran, ir iran mohammad taghi goodarzi research center for molecular medicine, hamadan university of medical sciences, hamadan, ir iran; hamadan university of medical sciences, hamadan, ir iran. tel: +98-8138380462, fax: +98-8138380208

2006
Kim M. Cecil

Introduction Congenital metabolic disorders refer to inherited defects producing metabolic abnormalities in the human body. Disorders that disrupt the central nervous system (CNS) are of great importance. These disorders produce a paradoxical situation for neuroradiologists and imaging scientists investigating them. One would hypothesize that an error in metabolism would produce either an exces...

Journal: :medical journal of islamic republic of iran 0
firouzeh nili from the department of pediatrics, imam khomeini hospital, tehran university of medical sciences,

a four day old female infant was admitted because of poor feeding, vomiting and jaundice. laboratory examination showed hyperkalemia, mild hyponatremia and renal tubular acidosis type 4. serum aldosterone and plasma renin activity were elevated but serum cortisol, 17 -hydroxyprogesterone, acth, 24 hour urinary 17- ketoste roid, pregnanetriol, renal function and sonogram were normal and hence ps...

Journal: :iranian journal of child neurology 0
nosratollah seyed shahabi

abstract hypotonia is a serious neurologic problem in neonatal period. although hypotonia is a nonspecific clinical finding but it is the most common motor disorder in the newborn. the objective of this study was to determine the frequency of neonatal hypotonia then to ascertain of the most common causes methods this cross –sectional prospective study was carried out on the 3281 term infants ho...

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