scholarly journals Combination of palmoplantar keratoderma and hair shaft anomalies, the warning signal of severe arrhythmogenic cardiomyopathy: a systematic review on genetic desmosomal diseases

2015 ◽  
Vol 53 (5) ◽  
pp. 289-295 ◽  
Author(s):  
Laura Polivka ◽  
Christine Bodemer ◽  
Smail Hadj-Rabia
Keyword(s):  
Systematic Review ◽  
Warning Signal ◽  
Hair Shaft ◽  
Palmoplantar Keratoderma ◽  
Arrhythmogenic Cardiomyopathy

scholarly journals Drugs Associated With the Development of Palmoplantar Keratoderma: A Systematic Review

2021 ◽  
pp. 120347542110045
Author(s):  
Sara Mirali ◽  
Abrahim Abduelmula ◽  
Asfandyar Mufti ◽  
Muskaan Sachdeva ◽  
Jensen Yeung
Keyword(s):  
Systematic Review ◽  
Tyrosine Kinase ◽  
Tyrosine Kinase Inhibitors ◽  
Complete Resolution ◽  
Kinase Inhibitors ◽  
Healthcare Providers ◽  
Latency Period ◽  
Braf Inhibitors ◽  
Palmoplantar Keratoderma ◽  
Drug Induced

Background Palmoplantar keratoderma (PPK) are a heterogenous group of hereditary and acquired disorders that are characterized by excessive epidermal thickening of the palms and/or soles. PPK has been described as a rare adverse event for some medications. The aim of this systematic review was to summarize outcomes in PPK associated with various medications. This data will assist dermatologists and other healthcare providers treating patients with drug-induced PPK. Methods EMBASE and MEDLINE databases were searched in accordance with PRISMA guidelines using the keyword “palmoplantar keratoderma.” 40 studies met the inclusion criteria. Results A total of 247 patients (mean age: 57.0 years) were included in the analysis. Among patients whose sex was reported, 60.3% ( n = 35/58) were male. PPK most frequently developed after treatment with BRAF inhibitors (73.7%, n = 182/247), BRAF inhibitors combined with MEK1/2 inhibitors (15.4%, n = 38/247), tyrosine kinase inhibitors (TKIs) (3.2%, n = 8/247), or chemotherapy (2.4%, n = 6/247). The mean latency period between initiation of the drug and onset of PPK was 7.6 months (range: 0.25-90 months). Improvement of PPK was reported in 24 cases, with 50% ( n = 12/24) achieving complete resolution and 50% ( n = 12/24) achieving partial resolution. All patients who achieved complete resolution stopped the suspected drug, with a mean resolution period of 2.4 months (range: 2 weeks-6 months). The most common treatments for PPK were keratolytic treatments ( n = 10) and topical corticosteroids ( n = 4). Conclusions PPK was most frequently associated with targeted kinase inhibitors, specifically BRAF, MEK1/2, and tyrosine kinase inhibitors.

scholarly journals Palmoplantar Keratoderma and Woolly Hair Revealing Asymptomatic Arrhythmogenic Cardiomyopathy

2019 ◽  
Vol 99 (9) ◽  
pp. 831-832
Author(s):  
L Guerra ◽  
M Magliozzi ◽  
A Baban ◽  
C Mambro ◽  
G Zenzo ◽  
...  
Keyword(s):  
Palmoplantar Keratoderma ◽  
Arrhythmogenic Cardiomyopathy ◽  
Woolly Hair

Missense variants in the spectrin repeat domain of DSP are associated with arrhythmogenic cardiomyopathy: A family report and systematic review

2020 ◽  
Vol 182 (10) ◽  
pp. 2359-2368
Author(s):  
Steffany Grondin ◽  
Avedis‐Christ Wazirian ◽  
Paloma Jorda ◽  
Donato G. Terrone ◽  
Johannie Gagnon ◽  
...  
Keyword(s):  
Systematic Review ◽  
Arrhythmogenic Cardiomyopathy ◽  
Spectrin Repeat ◽  
Missense Variants ◽  
Repeat Domain

scholarly journals The Role of Speckle Tracking Echocardiography in the Evaluation of Common Inherited Cardiomyopathies in Children and Adolescents: A Systematic Review

Diagnostics ◽  
2021 ◽  
Vol 11 (4) ◽  
pp. 635
Author(s):  
Dan M. Dorobantu ◽  
Curtis A. Wadey ◽  
Nurul H. Amir ◽  
A. Graham Stuart ◽  
Craig A. Williams ◽  
...  
Keyword(s):  
Systematic Review ◽  
Speckle Tracking ◽  
Circumferential Strain ◽  
Speckle Tracking Echocardiography ◽  
Radial Strain ◽  
Left Ventricular ◽  
Arrhythmogenic Cardiomyopathy ◽  
Apical Rotation ◽  
Inherited Cardiomyopathies

Speckle tracking echocardiography (STE) has gained importance in the evaluation of adult inherited cardiomyopathies, but its utility in children is not well characterized. We conducted a systematic review to evaluate the role of STE in pediatric inherited cardiomyopathies. PubMed, EMBASE, Web of Science, Scopus, CENTRAL and CINAHL databases were searched up to May 2020, for terms related to inherited cardiomyopathies and STE. Included were dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (ACM). A total of 14 cohorts were identified, of which six were in DCM, four in HCM, three in LVNC and one in ACM. The most commonly reported STE measurements were left ventricular longitudinal strain (Sl), circumferential strain (Sc), radial strain (Sr) and rotation/torsion/twist. Sl, Sc and were abnormal in all DCM and LVNC cohorts, but not in all HCM. Apical rotation and twist/torsion were increased in HCM, and decreased in LVNC. Abnormal STE parameters were reported even in cohorts with normal non-STE systolic/diastolic measurements. STE in childhood cardiomyopathies can detect early changes which may not be associated with changes in cardiac function detectable by non-STE methods. Longitudinal and circumferential strain should be introduced in the cardiomyopathy echocardiography protocol, reflecting current practice in adults.

Human-hair analysis

1992 ◽  
Vol 50 (1) ◽  
pp. 886-887
Author(s):  
Brenda E. Lambert ◽  
Ernest C. Hammond
Keyword(s):  
Human Hair ◽  
Hair Analysis ◽  
Hair Shaft ◽  
White Female ◽  
Chemical Processing ◽  
X Ray ◽  
Hair Samples ◽  
Wave Technique ◽  
Permanent Wave ◽  
Scanning Electron

The purpose of this study was to examine the external structure of four human hair shaft samples with the scanning Electron Microscope (SEM) and to obtain information regarding the chemical composition of hair by using the attached x ray microanalysis unit.The hair samples were obtained from two female subjects. Sample A was taken from a black female and had not undergone any type of chemical processing. Sample B, C, D were taken from a white female, and were natural, processed, and unpigmented, i.e. “gray”, respectively. Sample C had been bleached, tinted, and chemically altered using a permanent wave technique.

High-voltage EM description of the types and distribution of sensory endings in the inner conical body of the rat vibrissal follicle-sinus complex

1990 ◽  
Vol 48 (3) ◽  
pp. 410-411
Author(s):  
Tony M. Mosconi ◽  
Min J. Song ◽  
Frank L. Rice
Keyword(s):  
Mammalian Species ◽  
Dorsal Side ◽  
Hair Shaft ◽  
Sensory Innervation ◽  
Conical Body ◽  
Adult Rats ◽  
Perpendicular Plane ◽  
Ultrastructural Studies ◽  
Unmyelinated Axons ◽  
Sensory Endings

Whiskers or vibrissal follicle-sinus complexes (F-SCs) on the snouts of many mammalian species are structures that have complex, dense sensory innervation. The innervation of F-SCs is remarkably similar in all species with the exception of one site - the inner conical body (ICB). The ICB is an elongated cylindrical structure that encircles the hair shaft near the neck of the follicle. This site has received only cursory attention in ultrastructural studies of the F-SCAdult rats were perfused after the method of Renehan and Munger2. F-SCs were quartered longitudinally and embedded separately in Epon-Araldite. Serial 0.25 μm sections were cut in either the longitudinal or perpendicular plane through the ICB and examined with an AEI EM7 1.2 MV HVEM (Albany, NY) at 1000 KV. Sensory endings were reconstructed from serial micrographs through at least 20 μm in the longitudinal plane and through 10 μm in the perpendicular plane.From two to six small superficial vibrissal nerves converge upon the neck of the F-SC and descend into the ICB. The nerves branch into smaller bundles of myelinated and unmyelinated axons along the dorsal side of the hair shaft.

The effects of low-ratio n-6/n-3 PUFA on biomarkers of inflammation: a systematic review and meta-analysis

2021 ◽  
Author(s):  
Yali Wei ◽  
Yan Meng ◽  
Na Li ◽  
Qian Wang ◽  
Liyong Chen
Keyword(s):  
Systematic Review ◽  
Fatty Acid ◽  
Polyunsaturated Fatty Acid ◽  
Meta Analysis ◽  
Chain Polyunsaturated Fatty Acid ◽  
Inflammation Markers ◽  
Pufa Supplementation ◽  
Long Chain

The purpose of the systematic review and meta-analysis was to determine if low-ratio n-6/n-3 long-chain polyunsaturated fatty acid (PUFA) supplementation affects serum inflammation markers based on current studies.

Parent Education in Studies With Nonverbal and Minimally Verbal Participants With Autism Spectrum Disorder: A Systematic Review

2020 ◽  
Vol 29 (2) ◽  
pp. 890-902
Author(s):  
Lynn Kern Koegel ◽  
Katherine M. Bryan ◽  
Pumpki Lei Su ◽  
Mohini Vaidya ◽  
Stephen Camarata
Keyword(s):  
Systematic Review ◽  
Autism Spectrum Disorder ◽  
Parent Education ◽  
Autism Spectrum ◽  
Verbal Communication ◽  
Fidelity Of Implementation ◽  
Spectrum Disorder ◽  
Education Programs ◽  
Minimally Verbal ◽  
Parent Education Programs

Purpose The purpose of this systematic review was to identify parent education procedures implemented in intervention studies focused on expressive verbal communication for nonverbal (NV) or minimally verbal (MV) children with autism spectrum disorder (ASD). Parent education has been shown to be an essential component in the habilitation of individuals with ASD. Parents of individuals with ASD who are NV or MV may particularly benefit from parent education in order to provide opportunities for communication and to support their children across the life span. Method ProQuest databases were searched between the years of 1960 and 2018 to identify articles that targeted verbal communication in MV and NV individuals with ASD. A total of 1,231 were evaluated to assess whether parent education was implemented. We found 36 studies that included a parent education component. These were reviewed with regard to (a) the number of participants and participants' ages, (b) the parent education program provided, (c) the format of the parent education, (d) the duration of the parent education, (e) the measurement of parent education, and (f) the parent fidelity of implementation scores. Results The results of this analysis showed that very few studies have included a parent education component, descriptions of the parent education programs are unclear in most studies, and few studies have scored the parents' implementation of the intervention. Conclusions Currently, there is great variability in parent education programs in regard to participant age, hours provided, fidelity of implementation, format of parent education, and type of treatment used. Suggestions are made to provide both a more comprehensive description and consistent measurement of parent education programs.

Characteristics of the Frequency-Following Response to Speech in Neonates and Potential Applicability in Clinical Practice: A Systematic Review

2020 ◽  
Vol 63 (5) ◽  
pp. 1618-1635
Author(s):  
Céline Richard ◽  
Mary Lauren Neel ◽  
Arnaud Jeanvoine ◽  
Sharon Mc Connell ◽  
Alison Gehred ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cochrane Library ◽  
Neurodevelopmental Outcomes ◽  
Frequency Following Response ◽  
Ovid Medline ◽  
Published Evidence ◽  
Mesh Terms ◽  
Potential Applicability ◽  
Clinical Potential

Purpose We sought to critically analyze and evaluate published evidence regarding feasibility and clinical potential for predicting neurodevelopmental outcomes of the frequency-following responses (FFRs) to speech recordings in neonates (birth to 28 days). Method A systematic search of MeSH terms in the Cumulative Index to Nursing and Allied HealthLiterature, Embase, Google Scholar, Ovid Medline (R) and E-Pub Ahead of Print, In-Process & Other Non-Indexed Citations and Daily, Web of Science, SCOPUS, COCHRANE Library, and ClinicalTrials.gov was performed. Manual review of all items identified in the search was performed by two independent reviewers. Articles were evaluated based on the level of methodological quality and evidence according to the RTI item bank. Results Seven articles met inclusion criteria. None of the included studies reported neurodevelopmental outcomes past 3 months of age. Quality of the evidence ranged from moderate to high. Protocol variations were frequent. Conclusions Based on this systematic review, the FFR to speech can capture both temporal and spectral acoustic features in neonates. It can accurately be recorded in a fast and easy manner at the infant's bedside. However, at this time, further studies are needed to identify and validate which FFR features could be incorporated as an addition to standard evaluation of infant sound processing evaluation in subcortico-cortical networks. This review identifies the need for further research focused on identifying specific features of the neonatal FFRs, those with predictive value for early childhood outcomes to help guide targeted early speech and hearing interventions.

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