نتایج جستجو برای: melas syndrome

تعداد نتایج: 622265  

Journal: :EBioMedicine 2018
Yi Shiau Ng Nichola Z Lax Paul Maddison Charlotte L Alston Emma L Blakely Philippa D Hepplewhite Gillian Riordan Surita Meldau Patrick F Chinnery Germaine Pierre Efstathia Chronopoulou Ailian Du Imelda Hughes Andrew A Morris Smaragda Kamakari Georgia Chrousos Richard J Rodenburg Christiaan G J Saris Catherine Feeney Steven A Hardy Takafumi Sakakibara Akira Sudo Yasushi Okazaki Kei Murayama Helen Mundy Michael G Hanna Akira Ohtake Andrew M Schaefer Mike P Champion Doug M Turnbull Robert W Taylor Robert D S Pitceathly Robert McFarland Gráinne S Gorman

Mutations in the m.13094T>C MT-ND5 gene have been previously described in three cases of Leigh Syndrome (LS). In this retrospective, international cohort study we identified 20 clinically affected individuals (13 families) and four asymptomatic carriers. Ten patients were deceased at the time of analysis (median age of death was 10years (range: 5·4months-37years, IQR=17·9years). Nine patients m...

Journal: :Clinical molecular pathology 1995
C W Lam K Jain K Y Chan D K Silva Y W Chan L J Wong

The clinical presentation and the biochemical and molecular genetic findings are described in a 13 year old Chinese boy with MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes). The diagnosis was initially suspected because of the characteristic clinical features and the strong family history of convulsions. Using polymerase chain reaction-restriction enzyme analy...

2016
J Derek Charlwood Guido Favia Michelle R. Sanford Anthony J. Cornel Catelyn C. Nieman Joao Dinis Allison M. Weakley Gregory C. Lanzaro Yoosook Lee

Presence of circumsporozoite protein (CSP) was Plasmodium falciparum detected by enzyme linked immunosorbent assay (ELISA) in a sample of s.s., and collected in Anopheles gambiae A. melas A. pharoensis Guinea-Bissau during October and November 2009. The percentage of P. infected samples (10.2% overall) was comparable to earlier studies falciparum from other sites in Guinea-Bissau (9.6-12.4%). T...

Journal: :Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 2014

Journal: :Journal of Korean Medical Science 2002
Dae Seong Kim Dae Soo Jung Kyu Hyun Park In Joo Kim Cheol Min Kim Won Ho Lee Soon Ki Rho

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episode (MELAS) and myoclonic epilepsy and ragged-red fibers (MERRF) are rare disorders caused by point mutation of the tRNA gene of the mitochondrial genome. To understand the pathogenetic mechanism of MELAS and MERRF, we studied four patients. Serially sectioned frozen muscle specimens with a battery of histochemical stai...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2005
Yohei Kirino Yu-Ichi Goto Yolanda Campos Joaquin Arenas Tsutomu Suzuki

Mutations in mtDNA are responsible for a variety of mitochondrial diseases, where the mitochondrial tRNA(Leu(UUR)) gene has especially hot spots for pathogenic mutations. Clinical features often depend on the tRNA species and/or positions of the mutations; however, molecular pathogenesis elucidating the relation between the location of the mutations and their leading phenotype are not fully und...

Journal: :Heart 1998
Y Okajima Y Tanabe M Takayanagi H Aotsuka

OBJECTIVE To investigate cardiac function in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) and clarify the clinical features of cardiomyopathy in MELAS. PATIENTS 11 consecutive patients with MELAS (mean age at initial examination 11.3 years, range 4 to 16 years) were enrolled in the study. Six were followed for more than five years. RESULTS...

2018
Iman S Al-Gadi Richard H Haas Marni J Falk Amy Goldstein Shana E McCormack

Context Endocrine disorders are common in individuals with mitochondrial disease. To develop evidence-based screening practices in this high-risk population, updated age-stratified estimates of the prevalence of endocrine conditions are needed. Objective To measure the point prevalence of selected endocrine disorders in individuals with mitochondrial disease. Design Setting and Patients The...

2011
Masayuki Ikeda

Since stroke in childhood, although rare, has many potential causes, identification of the underlying disease offers a diagnostic challenge. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a rare disorder often mistaken for other diseases. Here, we describe the case of a previously healthy 11-year-old girl who initially presented with gastroenteritis...

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