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https://www.readbyqxmd.com/read/28819917/polyglandular-autoimmune-syndromes
#1
REVIEW
G J Kahaly, L Frommer
BACKGROUND: In recent years, scientific knowledge pertaining to the rare ORPHAN polyglandular autoimmune syndrome (registered code ORPHA 282196) has accumulated. OBJECTIVE: To offer current demographic, clinical, serological and immunogenic data on PAS. METHODS: Review of the pertinent and current literature. RESULTS: Polyglandular autoimmune syndromes (PAS) are multifactorial diseases with at least two coexisting autoimmune-mediated endocrinopathies...
August 17, 2017: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/28747936/psychometric-properties-of-the-self-perception-profile-for-children-in-children-with-chronic-illness
#2
Mark A Ferro, Jennie Tang
OBJECTIVE: The Self-Perception Profile for Children (SPPC) is a commonly used measure of self-concept in children, but little research has examined its psychometric properties in children newly-diagnosed with chronic illness. METHOD: Confirmatory factor analysis and examination of reliability and convergent and discriminant validity of the SPPC was conducted in 31 children newly-diagnosed with asthma, diabetes, epilepsy, food allergy, or juvenile arthritis. RESULTS: The unidimensionality of each domain of the SPPC was confirmed, internal reliability was robust (α=...
July 2017: Journal of the Canadian Academy of Child and Adolescent Psychiatry
https://www.readbyqxmd.com/read/28686329/juvenile-onset-generalized-lipodystrophy-due-to-a-novel-heterozygous-missense-lmna-mutation-affecting-lamin-c
#3
Nivedita Patni, Chao Xing, Anil K Agarwal, Abhimanyu Garg
The LMNA gene contains 12 exons and encodes lamins A and C by alternative splicing within exon 10. While mutations in lamin A specific residues cause several diseases including lipodystrophy, progeria, muscular dystrophy, neuropathy, and cardiomyopathy, only three families with mutations in lamin C-specific residues are reported with cardiomyopathy, neuropathy, and muscular dystrophy so far. We now report two brothers with juvenile-onset generalized lipodystrophy due to a lamin C-specific mutation. The proband, a 23-year-old Caucasian male was reported to have generalized lipodystrophy at 3 weeks of age, developed diabetes, hypertriglyceridemia, hypertension and liver problems and died with complications of cirrhosis, and kidney failure...
July 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28680339/exceptional-manifestation-of-polyautoimmunity-in-a-very-young-girl-a-case-report
#4
Agnieszka Mroczkowska-Juchkiewicz, Jacek Postępski, Edyta Olesińska, Paulina Krawiec, Elżbieta Pac-Kożuchowska
Polyautoimmunity is defined as the presence of more than one autoimmune disease in a single patient. The exact pathogenic mechanisms responsible for the coexistence of distinct autoimmune diseases within an individual have not been clearly explained. We report a case of a very young girl with the extremely rare co-existence of four distinct autoimmune diseases i.e. juvenile idiopathic arthritis, type 1 diabetes mellitus, coeliac disease and autoimmune hepatitis, recognized based on validated international classification criteria...
2017: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/28625550/maternal-exposure-to-western-diet-affects-adult-body-composition-and-voluntary-wheel-running-in-a-genotype-specific-manner-in-mice
#5
Layla Hiramatsu, Jarren C Kay, Zoe Thompson, Jennifer M Singleton, Gerald C Claghorn, Ralph L Albuquerque, Brittany Ho, Brett Ho, Gabriela Sanchez, Theodore Garland
Some human diseases, including obesity, Type II diabetes, and numerous cancers, are thought to be influenced by environments experienced in early life, including in utero. Maternal diet during the perinatal period may be especially important for adult offspring energy balance, potentially affecting both body composition and physical activity. This effect may be mediated by the genetic background of individuals, including, for example, potential "protective" mechanisms for individuals with inherently high levels of physical activity or high basal metabolic rates...
June 15, 2017: Physiology & Behavior
https://www.readbyqxmd.com/read/28615030/the-autoimmune-burden-in-juvenile-idiopathic-arthritis
#6
Elena Tronconi, Angela Miniaci, Andrea Pession
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory arthritis of unknown origin which can be considered an autoimmune disease (AD). The aim of this study is to analyse the presence of two or more autoimmune diseases (polyautoimmunity) in patients suffering from JIA and to evaluate the occurrence of ADs in their families. METHODS: Seventy-nine patients diagnosed with JIA aged 0-21 years, admitted to the Paediatric Rheumatology Unit, Sant'Orsola-Malpighi Hospital, Bologna were screened for ADs...
June 14, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28488272/pathophysiological-characteristics-of-preproinsulin-specific-cd8-t-cells-in-subjects-with-juvenile-onset-and-adult-onset-type-1-diabetes-a-1-year-follow-up-study
#7
Mahinder Paul, Darshan Badal, Neenu Jacob, Devi Dayal, Rakesh Kumar, Anil Bhansali, Sanjay Kumar Bhadada, Naresh Sachdeva
AIMS/HYPOTHESIS: Among the beta-cell associated antigens, preproinsulin (PPI) has been shown to play a key role in the pathogenesis of type 1 diabetes (T1D). PPI-specific autoreactive CD8+ T cells emerge early during beta-cell destruction and persist in peripheral circulation during diabetes progression. However, the influence of insulin therapy on phenotype of autoreactive CD8+ T cells in T1D including, juvenile-onset T1D (JOT1D), and adult-onset T1D (AOT1D) is not yet known. METHODS: We followed the time course of PPI-specific CD8+ T cells in JOT1D and AOT1D subjects that achieved glycemic control after 1 year of insulin therapy, using major histocompatibility complex-I (MHC-I) dextramers by flow cytometry...
May 10, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28472973/arterial-stiffness-by-oscillometric-device-and-telomere-lenght-in-juvenile-idiopathic-artrhitis-with-no-cardiovascular-risk-factors-a-cross-sectional-study
#8
Maria Mercedes Picarelli, Luiz Cláudio Danzmann, Lucas Kich Grun, Nevton Teixeira Rosa Júnior, Patrícia Lavandovsky, Fátima Theresinha Costa Rodrigues Guma, Renato T Stein, Florência Barbé-Tuana, Marcus Herbert Jones
BACKGROUND: Advances in juvenile idiopathic arthritis (JIA) treatment is promoting free disease survival. Cardiovascular disease (CVD) may emerge as an important cause of morbidity and mortality. Pulse wave velocity (PWV), a surrogate marker of arterial stiffness, and telomere length (TL) are considered as potential predictors of CVD and its outcomes. The study aim was to assess PWV, TL in a JIA population and to test its correlation. In a cross sectional study, 24 JIA patients, 21 controls for TL and 20 controls for PWV were included...
May 4, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28458655/diabetes-and-insulin-injection-modalities-effects-on-hepatic-and-hippocampal-expression-of-11%C3%AE-hydroxysteroid-dehydrogenase-type-1-in-juvenile-diabetic-male-rats
#9
Véronica Rougeon, Marie-Pierre Moisan, Nicole Barthe, Marie-Christine Beauvieux, Jean-Christophe Helbling, Véronique Pallet, Nathalie Marissal-Arvy, Pascal Barat
BACKGROUND: Dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis is often encountered in diabetes, leading to several clinical complications. Our recent results showing an elevated tetrahydrocortisol/tetrahydrocorticosterone ratio in morning urine of diabetic children compared to that of controls suggest an increased nocturnal activity of 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) in the former. QUESTION: We hypothesized that these observations could be explained by a reduced inhibition of hepatic 11β-HSD1 activity by exogenous insulin owing to its subcutaneous (SC) administration and absence of first hepatic passage...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28435047/histological-and-cognitive-alterations-in-adult-diabetic-rats-following-an-episode-of-juvenile-diabetic-ketoacidosis-evidence-of-permanent-cerebral-injury
#10
Nicole Glaser, Jennifer Sasaki-Russell, Michael Cohen, Christopher Little, Martha O'Donnell, Jeffrey Sall
Evidence suggests that diabetic ketoacidosis (DKA) may cause subtle cognitive alterations in children but the mechanisms are poorly understood. Acute DKA is associated with reactive astrogliosis and microglial activation in a rat model. Whether these inflammatory changes permanently alter brain histology is unknown. We aimed to determine whether DKA results in permanent alterations in brain histology and whether these changes are associated with cognitive deficits in a rat model. We induced diabetes in juvenile rats with streptozotocin at 4 weeks of age...
May 22, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28428167/developing-a-patient-centered-mhealth-app-a-tool-for-adolescents-with-type-1-diabetes-and-their-parents
#11
Bree E Holtz, Katharine M Murray, Denise D Hershey, Julie K Dunneback, Shelia R Cotten, Amanda J Holmstrom, Arpita Vyas, Molly K Kaiser, Michael A Wood
BACKGROUND: Type 1 diabetes (T1D) afflicts approximately 154,000 people under 20 years of age. Three-quarters of adolescents are not achieving glycosylated hemoglobin (HbA1c) targets, which leads to negative health outcomes. Mobile health (mHealth), the use of technology in health, has been used successfully to improve health in many chronic conditions, including diabetes. OBJECTIVE: The purpose of this study was to use patient-centered research methods to inform and improve the design and functionality of our T1D app, MyT1DHero, and to provide insight for others who are designing a health app for adolescents and parents...
April 19, 2017: JMIR MHealth and UHealth
https://www.readbyqxmd.com/read/28378047/-confocal-microscope-examination-of-the-corneal-nerve-plexus-as-biomarker-for-systemic-diseases-view-from-the-corneal-nerve-plexus-on-diabetes-mellitus-disease
#12
S Baltrusch
It is estimated that approximately 50% of patients with diabetes mellitus suffer from polyneuropathy, which is frequently diagnosed too late. Consequently, the question arises whether imaging procedures of the eye, namely optical coherence tomography of the retina and confocal microscopy of the cornea are suitable for the diagnostics and follow-up control of neurodegenerative changes in patients with diabetes mellitus. De Clerck and co-workers could demonstrate this by a systematic review of studies. Of these studies 11 were further evaluated with respect to corneal confocal microscopy...
July 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/28343613/effects-of-the-use-of-growth-hormone-in-children-and-adolescents-with-juvenile-idiopathic-arthritis-a-systematic-review
#13
Renan Bazuco Frittoli, Barbara Sugui Longui, Amanda Meireles Silva, Antônio de Azevedo Barros Filho, Maria Ângela Reis de Góes Monteiro, Simone Appenzeller
INTRODUCTION: Children with juvenile idiopathic arthritis (JIA) often have impaired growth and short stature. There is evidence that the therapeutic use of growth hormone (GH) is useful and safe in these patients. OBJECTIVE: To analyze the effects of GH use in patients with JIA. METHOD: A systematic review of the literature over the last 18 years in Medline and Embase databases. The criteria were analyzed independently by the researchers. We used the following keywords: "growth hormone", "arthritis, juvenile", "arthritis, rheumatoid", "child" and "adolescent"...
March 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28333965/worm-infestations-and-development-of-autoimmunity-in-children-the-abis-study
#14
Johnny Ludvigsson, Michael P Jones, Åshild Faresjö
Worm infestations influence the immune system and may therefore decrease the risk for autoimmune diseases. The aim of the study was to determine whether children who have developed autoimmune disease were less likely to have had worm infestations in childhood. The ABIS-study is a prospective population-based cohort study of children born in southeast Sweden 1997/99. 17.055 children participated. As of June 2014 116 individuals had developed Type 1 diabetes, 181 celiac disease, and 53 Juvenile Rheumatoid Arthritis...
2017: PloS One
https://www.readbyqxmd.com/read/28298568/comparison-of-disease-characteristics-organ-damage-and-survival-in-patients-with-juvenile-onset-and-adult-onset-systemic-lupus-erythematosus-in-a-combined-cohort-from-2-tertiary-centers-in-turkey
#15
Bahar Artim-Esen, Sezgin Şahin, Erhan Çene, Yasemin Şahinkaya, Kenan Barut, Amra Adrovic, Yasemin Özlük, Işın Kılıçaslan, Ahmet Omma, Ahmet Gül, Lale Öcal, Özgür Kasapçopur, Murat İnanç
OBJECTIVE: Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE). METHODS: For the study, 719 patients (76.9%) with aSLE and 216 (23.1%) with jSLE were examined. Comparisons between the groups were made for demographic characteristics, clinical features, auto-antibody profiles, damage, and survival rates...
May 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28281839/glycemic-control-protects-against-trabecular-bone-microarchitectural-damage-in-a-juvenile-male-rat-model-of-streptozotocin-induced-diabetes
#16
Guilherme José Pimentel Lopes de Oliveira, Túlio Luiz Durigan Basso, Lucas Amaral Fontanari, Ana Paula de Souza Faloni, Élcio Marcantonio, Silvana Regina Perez Orrico
PURPOSE: To determine which features of the bone microarchitecture are affected by established diabetes mellitus (DM) and the effectiveness of glycemic control in the protection of bone tissue. MATERIAL AND METHODS: Sixty juvenile Wistar male rats were divided into three groups of 20 animals: a control group (C) that included healthy animals, a diabetic group (D) that included animals with induced diabetes, and a controlled diabetic group (CD) that included animals with induced diabetes that were treated with insulin...
March 10, 2017: Endocrine Research
https://www.readbyqxmd.com/read/28277284/effects-of-the-use-of-growth-hormone-in-children-and-adolescents-with-juvenile-idiopathic-arthritis-a-systematic-review
#17
Renan Bazuco Frittoli, Barbara Sugui Longui, Amanda Meireles Silva, Antônio de Azevedo Barros Filho, Maria Ângela Reis de Góes Monteiro, Simone Appenzeller
INTRODUCTION: Children with Juvenile Idiopathic Arthritis (JIA) often have impaired growth and short stature. There is evidence that the therapeutic use of growth hormone (GH) is useful and safe in these patients. OBJECTIVE: To analyze the effects of GH use in patients with JIA. METHOD: A systematic review of the literature over the last 18 years in Medline and Embase databases. The criteria were analyzed independently by the researchers. We used the following keywords: "growth hormone", "arthritis, juvenile", "arthritis, rheumatoid", "child" and "adolescent"...
July 9, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28132032/effect-of-salacia-reticulata-w-and-clitoria-ternatea-l-on-the-cognitive-and-behavioral-changes-in-the-streptozotocin-induced-young-diabetic-rats
#18
R Rajashree, Rajani Patil, Sanjiva D Khlokute, Shivaprasad S Goudar
BACKGROUND: Diabetes mellitus (DM) of juvenile onset affects both the peripheral and central nervous systems (CNS). However, central effects are less documented and studied than peripheral deficits. Currently, the only feasible treatment available for type 1 DM (T1DM) is insulin which has its own limitations. Hence, an alternative therapy, especially a newer herbal formulation is very much the need of the time. The present study aimed to determine the effects of the alcoholic extracts of roots of the Salacia reticulata W...
March 1, 2017: Journal of Basic and Clinical Physiology and Pharmacology
https://www.readbyqxmd.com/read/28070780/laparoscopic-sleeve-gastrectomy-in-adolescents-with-or-without-syndromic-obesity-two-years-follow-up
#19
Angelo Iossa, Francesco De Peppo, Romina Caccamo, Brad Michael Watkins, Francesca Abbatini, Emanuela Ceriati, Gianfranco Silecchia
INTRODUCTION: Childhood obesity is an emerging health problem. Surgical treatment of obese adolescents, particularly those affected by congenital syndrome, represents a controversial issue. The aim of this multicenter study was to retrospectively assess the results of laparoscopic sleeve gastrectomy (LSG) in a cohort of adolescents affected by morbid obesity, with or without congenital syndromes. MATERIALS AND METHODS: Forty-one obese (BMI 49 ± 6 kg/m(2)) adolescents with mean age of 16 ± 3 years (58...
January 9, 2017: Eating and Weight Disorders: EWD
https://www.readbyqxmd.com/read/28065675/altered-expression-of-irs2-and-grb2-in-demyelination-of-peripheral-neurons-implications-in-diabetic-neuropathy
#20
Mallahalli S Manu, Kuruvanthe S Rachana, Gopal M Advirao
Demyelination of the peripheral nerves and dysfunction of Schwann cells (SCs) are the chronic complications involved in the development of peripheral neuropathy among diabetic patients. Insulin signaling plays an important role in restoring the myelin proteins in diabetic peripheral neuropathy (DPN). Since insulin levels are altered in diabetes, it becomes of great interest to appreciate the role and regulation of docking and adaptor protein, how these proteins respond to variations in the levels of insulin as experienced in juvenile diabetes...
April 2017: Neuropeptides
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