نتایج جستجو برای: pndm

تعداد نتایج: 79  

Journal: :American journal of medical genetics. Part A 2016
Asma Deeb Abdelhadi Habeb Walid Kaplan Salima Attia Suha Hadi Amani Osman Jamal Al-Jubeh Sarah Flanagan Elisa DeFranco Sian Ellard

Neonatal diabetes mellitus (NDM) can be transient (TNDM) or permanent (PNDM). Data on NDM from the Gulf region are limited to few studies on PNDM.The objective of this study was to describe the genetic and clinical spectrum of NDM and estimate its incidence in AbuDhabi, capital of the United Arab Emirate (UAE). Patients were identified from the pediatric diabetes clinics and sequencing of known...

2018
Hoi-Kuan Kong Xuan Liu Wai U. Lo Qing Pan Carmen O. K. Law Ting F. Chan Pak L. Ho Terrence C. K. Lau

Small RNAs (sRNAs) play significant roles in regulating gene expression post-transcriptionally in response to environmental changes in bacteria. In this work, we identified and characterized six novel sRNAs from an emerging multidrug-resistance (MDR) plasmid pNDM-HK, a New Delhi metallo-β-lactamase 1 gene (blaNDM−1)-carrying IncL/M plasmid that has caused worldwide threat in recent years. These...

Journal: :The Journal of clinical investigation 2008
Carlo Colombo Ottavia Porzio Ming Liu Ornella Massa Mario Vasta Silvana Salardi Luciano Beccaria Carla Monciotti Sonia Toni Oluf Pedersen Torben Hansen Luca Federici Roberta Pesavento Francesco Cadario Giorgio Federici Paolo Ghirri Peter Arvan Dario Iafusco Fabrizio Barbetti

Permanent neonatal diabetes mellitus (PNDM) is a rare disorder usually presenting within 6 months of birth. Although several genes have been linked to this disorder, in almost half the cases documented in Italy, the genetic cause remains unknown. Because the Akita mouse bearing a mutation in the Ins2 gene exhibits PNDM associated with pancreatic beta cell apoptosis, we sequenced the human insul...

Journal: :Orphanet Journal of Rare Diseases 2007
Michel Polak Hélène Cavé

Transient (TNDM) and Permanent (PNDM) Neonatal Diabetes Mellitus are rare conditions occurring in 1:300,000-400,000 live births. TNDM infants develop diabetes in the first few weeks of life but go into remission in a few months, with possible relapse to a permanent diabetes state usually around adolescence or as adults. The pancreatic dysfunction in this condition may be maintained throughout l...

Journal: :Antimicrobial agents and chemotherapy 2013
Rémy A Bonnin Patrice Nordmann Alessandra Carattoli Laurent Poirel

IncL/M-type plasmids R446b and R471a were originally isolated from Morganella morganii (formerly Proteus morganii) as the first members of the IncL/M group of multidrug resistance (MDR) plasmids (1, 2). IncL/M plasmids are now commonly identified among environmental and clinical isolates (3–5). This group of plasmids can be considered an emerging threat since it has been increasingly identified...

Journal: :Arquivos brasileiros de endocrinologia e metabologia 2008
Thais Della Manna Claudilene Battistim Vanessa Radonsky Roberta D Savoldelli Durval Damiani Fernando Kok Ewan R Pearson Sian Ellard Andrew T Hattersley André F Reis

Heterozygous activating mutations of KCNJ11 (Kir6.2) are the most common cause of permanent neonatal diabetes mellitus (PNDM) and several cases have been successfully treated with oral sulfonylureas. We report on the attempted transfer of insulin therapy to glibenclamide in a 4-year old child with PNDM and DEND syndrome, bearing a C166Y mutation in KCNJ11. An inpatient transition from subcutane...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2004
Peter Proks Jennifer F Antcliff Jon Lippiat Anna L Gloyn Andrew T Hattersley Frances M Ashcroft

Inwardly rectifying potassium channels (Kir channels) control cell membrane K(+) fluxes and electrical signaling in diverse cell types. Heterozygous mutations in the human Kir6.2 gene (KCNJ11), the pore-forming subunit of the ATP-sensitive (K(ATP)) channel, cause permanent neonatal diabetes mellitus (PNDM). For some mutations, PNDM is accompanied by marked developmental delay, muscle weakness, ...

Journal: :Diabetes care 2014
Nele Myngheer Karel Allegaert Andrew Hattersley Tim McDonald Holger Kramer Frances M Ashcroft Johan Verhaeghe Chantal Mathieu Kristina Casteels

OBJECTIVE Sulfonylureas (SUs) are effective at controlling glycemia in permanent neonatal diabetes mellitus (PNDM) caused by KCNJ11 (Kir6.2) mutations. RESEARCH DESIGN AND METHODS We report the case of a woman with PNDM who continued high doses of glibenclamide (85 mg/day) during her pregnancy. The baby was born preterm, and presented with macrosomia and severe hyperinsulinemic hypoglycemia r...

Masoud Dehghan Tezerjani, Mohammad Yahya Vahidi Mehrjardi Mohammadreza Dehghani, Seyed Mehdi Kalantar

Neonatal diabetes mellitus (NDM) is a rare kind of diabetes characterized by hyperglycemia and low levels of insulin. Clinically, it is categorized into two main types: transient NDM (TNDM) and permanent NDM (PNDM). These types are diagnosed based on duration of insulin dependence early in the disease. In TNDM, diabetes begins in the first few weeks of life with remission in a few months. Howev...

2015
Huseyin Demirbilek Ved Bhushan Arya Mehmet Nuri Ozbek Jayne A L Houghton Riza Taner Baran Melek Akar Selahattin Tekes Heybet Tuzun Deborah J Mackay Sarah E Flanagan Andrew T Hattersley Sian Ellard Khalid Hussain

BACKGROUND Neonatal diabetes mellitus (NDM) is a rare form of monogenic diabetes and usually presents in the first 6 months of life. We aimed to describe the clinical characteristics and molecular genetics of a large Turkish cohort of NDM patients from a single centre and estimate an annual incidence rate of NDM in South-Eastern Anatolian region of Turkey. DESIGN AND METHODS NDM patients pres...

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