Publications by authors named "Karina L Vivar"

12 Publications

  • Page 1 of 1

Use of telemedicine for ichthyosis: Patient advocacy group as conduit to expert physician advice.

Pediatr Dermatol 2021 Jan 23;38(1):137-142. Epub 2020 Nov 23.

Division of Dermatology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Background/objectives: Patients with rare diseases are challenged when it comes to finding physicians with expertise in their condition. The Foundation for Ichthyosis and Related Skin Types (FIRST) Tele-Ichthyosis program has provided telemedicine for patients and their families with keratinizing disorders since 2009. This study aims to characterize a decade of experience with the program.

Methods: This retrospective cohort study analyzed cases for demographics of patients and the clinicians who submitted their cases, nature of questions asked, number of expert responses, and characteristics of responses. Surveys were sent electronically to all users of the FIRST Tele-Ichthyosis service to assess experiences with the service and solicit constructive recommendations. Descriptive statistics were performed on the case review and responder surveys.

Results: Eighty-eight geographically diverse cases were reviewed showing increased use over time by various specialists for patients of all ages. Sixty-six percent of cases were definitively ichthyosis, and most submitters queried on diagnosis (47%) or treatment (72%). Most submitters described the service as easy to use (66.6%) and advice as timely (61.1%), clear (66.6%), and beneficial (61.1%). All submitters made suggestions for improvement (100%). Experts predominately worked with pediatric populations (70%) and reported self-motivation to volunteer and improve patients' lives (100%). Experts found technological barriers minor and provided feedback to enhance the service.

Conclusions: This report highlights how a rare-disease patient advocacy group successfully supports physician collaboration and patient outcomes through secure and efficient telemedicine. Lessons learned are highly relevant in the current healthcare environment.
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http://dx.doi.org/10.1111/pde.14460DOI Listing
January 2021

Cutaneous reactions in children treated with MEK inhibitors, BRAF inhibitors, or combination therapy: A multicenter study.

J Am Acad Dermatol 2020 Jul 17. Epub 2020 Jul 17.

Division of Pediatric Medicine Section of Dermatology, University of Toronto, Toronto, Canada.

Background: Treatment with BRAF inhibitors (BRAFI) and MEK inhibitors (MEKI) causes cutaneous reactions in children, limiting dosing or resulting in treatment cessation. The spectrum and severity of these reactions is not defined.

Objective: To determine the frequency and spectrum of cutaneous reactions in children receiving BRAFI and MEKI and their effects on continued therapy.

Methods: A multicenter, retrospective study was conducted at 11 clinical sites in the United States and Canada enrolling 99 children treated with BRAFI and/or MEKI for any indication from January 1, 2012, to January 1, 2018.

Results: All children in this study had a cutaneous reaction; most had multiple, with a mean per patient of 3.5 reactions on BRAFI, 3.7 on MEKI, and 3.4 on combination BRAFI/MEKI. Three patients discontinued treatment because of a cutaneous reaction. Treatment was altered in 27% of patients on BRAFI, 39.5% on MEKI, and 33% on combination therapy. The cutaneous reactions most likely to alter treatment were dermatitis, panniculitis, and keratosis pilaris-like reactions for BRAFI and dermatitis, acneiform eruptions, and paronychia for MEKI.

Conclusions: Cutaneous reactions are common in children receiving BRAFI and MEKI, and many result in alterations or interruptions in oncologic therapy. Implementing preventative strategies at the start of therapy may minimize cutaneous reactions.
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http://dx.doi.org/10.1016/j.jaad.2020.07.044DOI Listing
July 2020

Pine tar callus: A mimicker of a melanocytic lesion.

Pediatr Dermatol 2019 May 27;36(3):379-380. Epub 2019 Feb 27.

Northwestern University Feinberg School of Medicine, Chicago, Illinois.

We report a case of acral pigmented lesions due to pine tar, a common compound used on baseball bats to improve grip, deposition. The patient presented with an acute concern for a new melanocytic lesion, and dermoscopy revealed large brown globules, not typical of melanocytic neoplasms. We propose that the coupling of dermoscopy and a thorough clinical history of exogenous exposures in similar clinical presentations can provide reassurance in evaluating atypical appearing pigmented lesions.
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http://dx.doi.org/10.1111/pde.13773DOI Listing
May 2019

More than keratitis, ichthyosis, and deafness: Multisystem effects of lethal GJB2 mutations.

J Am Acad Dermatol 2019 Mar 2;80(3):617-625. Epub 2018 Oct 2.

Department of Dermatology, Yale School of Medicine, New Haven, Connecticut.

Background: Infant death in keratitis-ichthyosis-deafness (KID) syndrome is recognized; its association with specific genotypes and pathophysiology is inadequately understood.

Objective: We sought to discover characteristics that account for poor outcomes in lethal KID syndrome.

Methods: We collected 4 new cases and 9 previously reported, genotyped cases of lethal KID syndrome. We performed new molecular modeling of the lethal mutants GJB2 p.A88V and GJB2 p.G45E.

Results: Infant death occurred in all patients with GJB2 p.G45E and p.A88V; it is unusual with other GJB2 mutations. Early death with those 2 "lethal" mutations is likely multifactorial: during life all had ≥1 serious infection; most had poor weight gain and severe respiratory difficulties; many had additional anatomic abnormalities. Structural modeling of GJB2 p.G45E identified no impact on the salt bridge previously predicted to account for abnormal central carbon dioxide sensing of GJB2 p.A88V.

Limitations: This clinical review was retrospective.

Conclusion: GJB2 p.G45E and p.A88V are the only KID syndrome mutations associated with uniform early lethality. Those electrophysiologically severe mutations in GJB2 reveal abnormalities in many organs in lethal KID syndrome. All patients with KID syndrome may have subtle abnormalities beyond the eyes, ears, and skin. Early genotyping of KID syndrome births will inform prognostic discussion.
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http://dx.doi.org/10.1016/j.jaad.2018.09.042DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6372339PMC
March 2019

Stevens-Johnson syndrome/toxic epidermal necrolysis associated with zonisamide.

Clin Case Rep 2018 02 20;6(2):258-261. Epub 2017 Dec 20.

Department of Dermatology and Cutaneous Surgery University of South Florida Tampa Florida.

This report highlights zonisamide as a potential cause of serious cutaneous reactions as well as its cross-reactivity with other sulfonamides. Here, we present a case of SJS-TEN due to zonisamide, which was effectively treated with IVIg. Subsequently, the patient was transitioned to levetiracetam for seizure control.
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http://dx.doi.org/10.1002/ccr3.1288DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5799627PMC
February 2018

An Atopic Dermatitis Management Algorithm for Primary Care Providers and Assessment of Its Usefulness as a Clinical Tool.

Pediatr Dermatol 2017 Jul 25;34(4):402-407. Epub 2017 May 25.

Department of Pediatrics, University of California, San Francisco, San Francisco, California.

Background/objectives: There is a lack of primary care provider (PCP) understanding of atopic dermatitis (AD) treatments and topical steroid use. We designed an AD management algorithm for pediatric PCPs. We hypothesized that the algorithm would improve pediatric PCPs' knowledge of AD diagnosis and management.

Methods: Pediatric primary care resident and attending physicians at three residency programs were invited to participate in an electronic AD algorithm survey that contained demographic and 19 knowledge-based questions. Participants were randomized to intervention and control groups, with the intervention group receiving a short lecture and copy of our algorithm to use in an inpatient or outpatient setting for 2 months. Changes in scores between preintervention and postintervention surveys were compared.

Results: Of the 54 participants, those in the intervention group (n = 26) performed significantly better than those in the control group (n = 28) after controlling for pretest scores (β = 1.19 [95% confidence interval 0.07, 2.32], p = 0.04). The intervention group had a higher average score on the posttest knowledge questions (71% correct) than the control group (65% correct) (p = 0.06). The majority of physicians who received the algorithm agreed or strongly agreed that they liked using the algorithm.

Conclusion: The use of a management algorithm improved physician knowledge about the diagnosis and treatment of AD and was well accepted by physicians. Use of this management algorithm may lead to better recognition and management of AD, particularly earlier recognition of and therapy for superinfection, improving treatment outcomes and quality of life for patients and families.
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http://dx.doi.org/10.1111/pde.13157DOI Listing
July 2017

Epidermal programmed cell death-ligand 1 expression in TEN associated with nivolumab therapy.

J Cutan Pathol 2017 Apr 23;44(4):381-384. Epub 2017 Jan 23.

Department of Dermatology and Cutaneous Surgery, University of South Florida, Tampa, Florida.

Nivolumab is a programmed cell death receptor-1 (PD-1) antibody used in the treatment of metastatic or unresectable melanoma. Cutaneous reactions are the most common adverse events reported with these agents and are rarely severe or life-threatening. Here we present a case report describing the clinicopathological findings of a patient with a fatal toxic epidermal necrolysis (TEN) eruption associated with use of nivolumab for treatment of metastatic melanoma. The patient developed a pruritic, morbiliform eruption, which slowly progressed over 3 months to a tender, exfoliative dermatosis. Histology initially showed interface dermatitis and subsequently revealed full thickness epidermal necrosis. The diagnosis of TEN was made. From initial biopsy to TEN presentation, there was an increase in the number of CD8+ lymphocytes within the dermal-epidermal junction and an increase of programmed death ligand 1 (PD-L1) expression in both lymphocytes and keratinocytes. Despite treatment with infliximab, high-dose steroids and intravenous immunoglobulin, the patient expired. Herein we describe what we believe is the second case of TEN associated with anti-PD1 therapy reported in the literature. Increased expression of PD-L1 by immunohistochemistry was observed as the eruption progressed to TEN. Early diagnosis and treatment is necessary in these fatal TEN reactions secondary to the anti-PD-1 antibody therapies.
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http://dx.doi.org/10.1111/cup.12876DOI Listing
April 2017

Ecthyma associated with and .

JAAD Case Rep 2016 Nov 5;2(6):473-475. Epub 2016 Dec 5.

Department of Dermatology and Cutaneous Surgery, University of South Florida, Tampa, Florida.

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http://dx.doi.org/10.1016/j.jdcr.2016.09.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5148780PMC
November 2016

Influenza virus infection mimicking an acute abdomen in a female adolescent.

Influenza Other Respir Viruses 2014 Mar 26;8(2):140-1. Epub 2013 Dec 26.

Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA.

We report an adolescent with respiratory symptoms admitted for clinical signs of an acute abdomen. The only diagnostic finding was influenza A viral RNA detected in an upper respiratory tract specimen. Influenza should be considered in the differential diagnosis of children with respiratory illness and abdominal pain during influenza season.
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http://dx.doi.org/10.1111/irv.12222DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4186460PMC
March 2014

Health literacy assessment of labeling of pediatric nonprescription medications: examination of characteristics that may impair parent understanding.

Acad Pediatr 2012 Jul-Aug;12(4):288-96. Epub 2012 May 10.

Department of Pediatrics, New York University School of Medicine, NY 10016, USA.

Objective: Poor quality and variability of medication labeling have been cited as key contributors to medication misuse. We assessed the format and content of labels and materials packaged with common pediatric liquid nonprescription medications.

Methods: Descriptive study. A total of 200 top-selling pediatric oral liquid nonprescription medications (during the 52 weeks ending October 30, 2009) categorized as analgesic, cough/cold, allergy, and gastrointestinal products, with dosing information for children <12 years (representing 99% of U.S. market for these products) were reviewed. The principal display panel (PDP) and FDA Drug Facts panel (side panel) of each bottle, and associated box, if present, were independently examined by 2 abstractors. Outcome measures were content and format of active ingredient information and dosing instructions of the principal display panel and Drug Facts panel.

Results: Although almost all products listed active ingredients on the Drug Facts panel (side panel), nearly 1 in 5 (37 [18.5%]) did not list active ingredients on the PDP. When present, mean (SD) font size for PDP active ingredients was 10.7 (5.0), smaller than product brand name (32.1 [15.0]) and flavor (13.1 [4.8]); P < .001. Most products included directions in chart form (bottle: 167 [83.5%], box: 148 [96.1%], P < .001); mean (SD) font size: 5.5 (0.9; bottle), 6.5 (0.5; box), P < .001. Few products expressed dosing instructions in pictographic form: 4 (2.6%) boxes and 0 bottles. Nearly all products included the Food and Drug Administration-mandated sections.

Conclusions: The format and content of labels for nonprescription pediatric liquid medications could be improved to facilitate parent understanding of key medication information, including active ingredient information and dosing instructions.
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http://dx.doi.org/10.1016/j.acap.2012.02.010DOI Listing
December 2012

Introduction to the medical professions through an innovative medical student-run pipeline program.

J Natl Med Assoc 2011 Sep-Oct;103(9-10):832-8

Department of Medicine, Office of Diversity Affairs, NYU School of Medicine, New York, New York 10010, USA.

Underrepresented minorities (URMs) make up a disproportionately small percentage of medical school applicants, matriculants, and physicians relative to the general US population. Preprofessional pipeline programs may help introduce URMs to careers in the medical field. MiniMeds was developed as a paracurricular enrichment program that targeted URM students. The curriculum was designed and administered by medical students, and 2 trials of this program were conducted. Data were collected pre and post program through a survey that assessed knowledge of medical concepts and knowledge of and interest in careers in medicine. Attendance at program sessions correlated with baseline knowledge about medical professions. Knowledge about medical concepts increased significantly from baseline to follow-up for boys, a group significantly represented by URMs in our cohort. Median scores for knowledge of medical careers increased significantly from baseline to followup for URMs as well as for boys and girls. Preprofessional pipeline programs such as MiniMeds are able to engage and develop medical knowledge in URM students at a critical developmental age. Further evaluation and implementation of programs that incorporate medical students to actively develop and lead pipeline programs are warranted.
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http://dx.doi.org/10.1016/s0027-9684(15)30437-5DOI Listing
March 2012

Perceived barriers to care and attitudes towards shared decision-making among low socioeconomic status parents: role of health literacy.

Acad Pediatr 2012 Mar-Apr;12(2):117-24. Epub 2012 Feb 8.

Department of Pediatrics, New York University School of Medicine, NY 10016, USA.

Objective: Although low parent health literacy (HL) has been linked to poor child health outcomes, it is not known whether differences in perceptions related to access to care and provider-parent partnership in care are potential contributing factors. We sought to assess whether parent HL is associated with differences in perceived barriers to care and attitudes regarding participatory decision-making with the provider.

Methods: This was a cross-sectional analysis of data collected from parents presenting with their child to an urban public hospital pediatric clinic in New York City. Dependent variables were caregiver-reported barriers to care (ability to reach provider at night/on weekends, difficult travel to clinic) and attitudes towards participatory decision-making (feeling like a partner, relying on doctor's knowledge, leaving decisions up to the doctor, being given choices/asked opinion). The primary independent variable was caregiver HL (Short Test of Functional Health Literacy in Adults [S-TOHFLA]).

Results: A total of 823 parents were assessed; 1 in 4 (27.0%) categorized as having low HL. Parents with low HL were more likely to report barriers to care than those with adequate HL: trouble reaching provider nights/weekends, 64.9% vs. 49.6%, (p < 0.001, adjusted odds ratio [AOR] 1.7, 95% confidence interval [95% CI] 1.2-2.4); difficult travel, 15.3% vs. 8.0%, (p = 0.004, AOR 1.8, 95% CI 1.1-3.0). Low HL was also associated with not feeling like a partner (28.8% vs. 17.1%; AOR 2.0; 95% CI 1.4-3.0), preference for relying on the doctor's knowledge (68.9% vs. 52.2%; AOR 1.7; 95% CI 1.2-2.4), and preference for leaving decisions up to the doctor (57.7% vs. 33.3%; AOR 2.2; 95% CI 1.6-3.1).

Conclusions: Addressing issues of parent HL may be helpful in ameliorating barriers to care and promoting provider-parent partnership in care.
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http://dx.doi.org/10.1016/j.acap.2012.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3747780PMC
July 2012