Dysautonomia: Getting a Handle on POTS
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Orthostatic Intolerance V1 4.2.11, F10 Overview V2 6036-7,7038,7040,7042 Orthostatic Intolerance
NASA-STD-3001 Technical Brief Orthostatic Intolerance V1 4.2.11, F10 Overview V2 6036-7,7038,7040,7042 Orthostatic Intolerance Executive Summary Orthostatic intolerance (OI) is an abnormal response to standing upright caused by an inability to maintain arterial blood pressure and perfuse cerebral tissue. It can result in presyncope and, ultimately, syncope (i.e. loss of consciousness). Specifically in the spaceflight community, OI is a major concern when crewmembers are re-introduced to gravity after landing due to decreased plasma volume and sympathetic nervous system dysfunction. OI must also be considered for standing crew experiencing acceleration loads. A variety of countermeasures can be implemented to reduce the symptoms of and/or completely mitigate OI. Standards Overview NASA-STD-3001 Vol. 1 4.2.11 Fitness-for-Duty Orthostatic Hypotension Standard (with Appendix F.10) All approved countermeasures (fluid loading and compression garments) shall be utilized to mitigate symptoms of orthostatic hypotension (presyncopal/syncopal symptoms) to prevent impacts to performance of critical mission tasks. NASA-STD-3001 Vol. 2 6.3.2.8 Fluid Loading Water Quantity for Earth Entry [V2 6036] 6.3.2.9 Crew recovery Water Quantity [V2 6037] 7.4.1 Physiological Countermeasures Capability [V2 7038] The system shall provide countermeasures to meet crew bone, muscle, sensory-motor, and cardiovascular requirements defined in NASA-STD-3001, Vol. 1. 7.4.3 Physiological Countermeasure Operations [V2 7040] The physiological countermeasure system design shall allow the crew to unstow supplies, perform operations, and stow items within the allotted countermeasure schedule. 7.4.5 Orthostatic Intolerance Countermeasures [V2 7042] The system shall provide countermeasures to mitigate the effects of orthostatic intolerance when transitioning from microgravity to gravity environments. -
Dermatologic Manifestations and Complications of COVID-19
American Journal of Emergency Medicine 38 (2020) 1715–1721 Contents lists available at ScienceDirect American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem Dermatologic manifestations and complications of COVID-19 Michael Gottlieb, MD a,⁎,BritLong,MDb a Department of Emergency Medicine, Rush University Medical Center, United States of America b Department of Emergency Medicine, Brooke Army Medical Center, United States of America article info abstract Article history: The novel coronavirus disease of 2019 (COVID-19) is associated with significant morbidity and mortality. While Received 9 May 2020 much of the focus has been on the cardiac and pulmonary complications, there are several important dermato- Accepted 3 June 2020 logic components that clinicians must be aware of. Available online xxxx Objective: This brief report summarizes the dermatologic manifestations and complications associated with COVID-19 with an emphasis on Emergency Medicine clinicians. Keywords: COVID-19 Discussion: Dermatologic manifestations of COVID-19 are increasingly recognized within the literature. The pri- fi SARS-CoV-2 mary etiologies include vasculitis versus direct viral involvement. There are several types of skin ndings de- Coronavirus scribed in association with COVID-19. These include maculopapular rashes, urticaria, vesicles, petechiae, Dermatology purpura, chilblains, livedo racemosa, and distal limb ischemia. While most of these dermatologic findings are Skin self-resolving, they can help increase one's suspicion for COVID-19. Emergency medicine Conclusion: It is important to be aware of the dermatologic manifestations and complications of COVID-19. Knowledge of the components is important to help identify potential COVID-19 patients and properly treat complications. © 2020 Elsevier Inc. -
Dermatological Findings in Common Rheumatologic Diseases in Children
Available online at www.medicinescience.org Medicine Science ORIGINAL RESEARCH International Medical Journal Medicine Science 2019; ( ): Dermatological findings in common rheumatologic diseases in children 1Melike Kibar Ozturk ORCID:0000-0002-5757-8247 1Ilkin Zindanci ORCID:0000-0003-4354-9899 2Betul Sozeri ORCID:0000-0003-0358-6409 1Umraniye Training and Research Hospital, Department of Dermatology, Istanbul, Turkey. 2Umraniye Training and Research Hospital, Department of Child Rheumatology, Istanbul, Turkey Received 01 November 2018; Accepted 19 November 2018 Available online 21.01.2019 with doi:10.5455/medscience.2018.07.8966 Copyright © 2019 by authors and Medicine Science Publishing Inc. Abstract The aim of this study is to outline the common dermatological findings in pediatric rheumatologic diseases. A total of 45 patients, nineteen with juvenile idiopathic arthritis (JIA), eight with Familial Mediterranean Fever (FMF), six with scleroderma (SSc), seven with systemic lupus erythematosus (SLE), and five with dermatomyositis (DM) were included. Control group for JIA consisted of randomly chosen 19 healthy subjects of the same age and gender. The age, sex, duration of disease, site and type of lesions on skin, nails and scalp and systemic drug use were recorded. χ2 test was used. The most common skin findings in patients with psoriatic JIA were flexural psoriatic lesions, the most common nail findings were periungual desquamation and distal onycholysis, while the most common scalp findings were erythema and scaling. The most common skin finding in patients with oligoarthritis was photosensitivity, while the most common nail finding was periungual erythema, and the most common scalp findings were erythema and scaling. We saw urticarial rash, dermatographism, nail pitting and telogen effluvium in one patient with systemic arthritis; and photosensitivity, livedo reticularis and periungual erythema in another patient with RF-negative polyarthritis. -
Review Cutaneous Patterns Are Often the Only Clue to a a R T I C L E Complex Underlying Vascular Pathology
pp11 - 46 ABstract Review Cutaneous patterns are often the only clue to a A R T I C L E complex underlying vascular pathology. Reticulate pattern is probably one of the most important DERMATOLOGICAL dermatological signs of venous or arterial pathology involving the cutaneous microvasculature and its MANIFESTATIONS OF VENOUS presence may be the only sign of an important underlying pathology. Vascular malformations such DISEASE. PART II: Reticulate as cutis marmorata congenita telangiectasia, benign forms of livedo reticularis, and sinister conditions eruptions such as Sneddon’s syndrome can all present with a reticulate eruption. The literature dealing with this KUROSH PARSI MBBS, MSc (Med), FACP, FACD subject is confusing and full of inaccuracies. Terms Departments of Dermatology, St. Vincent’s Hospital & such as livedo reticularis, livedo racemosa, cutis Sydney Children’s Hospital, Sydney, Australia marmorata and retiform purpura have all been used to describe the same or entirely different conditions. To our knowledge, there are no published systematic reviews of reticulate eruptions in the medical Introduction literature. he reticulate pattern is probably one of the most This article is the second in a series of papers important dermatological signs that signifies the describing the dermatological manifestations of involvement of the underlying vascular networks venous disease. Given the wide scope of phlebology T and its overlap with many other specialties, this review and the cutaneous vasculature. It is seen in benign forms was divided into multiple instalments. We dedicated of livedo reticularis and in more sinister conditions such this instalment to demystifying the reticulate as Sneddon’s syndrome. There is considerable confusion pattern. -
Livedo Reticularis-An Unusual Skin Manifestation of Disseminated
Vol.35 No.3 Case report 139 Livedo reticularis-an unusual skin manifestation of disseminated strongyloidiasis: a case report with literature review Pariya Ruxrungtham MD, Ratchathorn Panchaprateep MD PhD, Pravit Asawanonda MD DSc. ABSTRACT: RUXRUNGTHAM P, PANCHAPRATEEP R, ASAWANONDA P. LIVEDO RETICULARIS-AN UNUSUAL SKIN MANIFESTATION OF DISSEMINATED STRONGYLOIDIASIS: A CASE REPORT WITH LITERATURE REVIEW. THAI J DERMATOL 2019; 35: 139-143. DIVISION OF DERMATOLOGY, DEPARTMENT OF MEDICINE, FACULTY OF MEDICINE, CHULALONGKORN UNIVERSITY; AND KING CHULALONGKORN MEMORIAL HOSPITAL, BANGKOK, THAILAND. A 71-year-old woman, with active autoimmune hepatitis, was treated with immunosuppressive drugs and presented with a1-month history of fever and diarrhea, dyspnea, and sudden eruptions of purpuric macules on the abdomen typical for disseminated strongyloidiasis, together with presence of Strongyloid larvae in rectum and sigmoid colon biopsies, and sputum fresh smear. Eight days into ivermectin treatment net-like purpuric patches on both thighs were observed and faded completely within 24 hours. The patient recovered fully after treatment completion. Key words: Disseminated strongyloidiasis, thumbprint purpura, livedo reticularis From :Division of Dermatology, Department of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand . Corresponding author: Pravit Asawanonda MD DSc., email: [email protected] Received: 22 October 2018 Revised: 24 January 2019 Accepted: 25 September 2019 140 Ruxrungtham P et al Thai J Dermatol, July-September, 2019 being treated with corticosteroids and azathioprine. The diagnosis was E. coli septicemia and she was treated with vancomycin, meropenem and metronidazole. Figure 1 Strongyloid larvae on sputum smear Figure 3 Livedo reticularis-like lesions on both thighs on day 8 of ivermectin treatment During admission, petechiae and purpuric macules or “thumbprint purpura” appeared on her abdomen and both upper thighs suggestive of disseminated strongyloidiasis (Figure 2). -
Neurocardiogenic Syncope and Associated Conditions: Insight Into Autonomic Nervous System Dysfunction
Türk Kardiyol Dern Arş - Arch Turk Soc Cardiol 2013;41(1):75-83 doi: 10.5543/tkda.2013.44420 75 Neurocardiogenic syncope and associated conditions: insight into autonomic nervous system dysfunction Nörokardiyojenik senkop ve ilişkili durumlar: Otonomik sinir sistemi işlev bozukluğuna bir bakış Antoine Kossaify, M.D., Kamal Kallab, M.D. Department of Syncope and Electrophysiology, ND Secours/USEK University Hospital, Byblos, Lebanon Summary– Neurocardiogenic syncope is known to be asso- Özet– Nörokardiyojenik senkopun mekanizması hala tam ola- ciated with autonomic nervous system dysfunction, although rak aydınlatılamamasına ragmen otonom sinir sistemi işlev the mechanism has not been entirely elucidated. In this study, bozukluğu ile ilişkili olduğu bilinmektedir. Bu yazıda nörokardi- we sought to highlight the pathogenic role of the autonomic yojenik senkop patogenezinde otonom sinir sisteminin rolünü nervous system in neurocardiogenic syncope and to review destekleyen en göze çarpıcı konuları araştırırken, temelinde the associated co-morbidities known to have a dysautonomic otonom sinir sistemi bozukluğunun olduğu bilinen komorbid basis. Herein we discuss migraine, orthostatic hypotension, durumları da gözden geçidik. Bu amaçla otonom sinir siste- postural orthostatic tachycardia syndrome, endothelial dys- minin patogenezdeki rolü ve klinik çıkarımları üzerine odak- function, chronic fatigue syndrome, and carotid sinus hyper- lanarak migren, ortostatik hipotansiyon, postüral ortostatik sensitivity with a focus on the pathogenic role of the autonom- taşikardi sendromu, endotel işlev bozukluğu, kronik yorgunluk ic nervous system and any consecutive clinical implications. sendromu ve karotis sinüs hipersensitivitesi tartışıldı. Tilt testi Other conditions, such as pre-syncopal heart rate acceleration sırasında ortaya çıkabilen senkop öncesi kalp atımlarında hız- and/or instability and pre-syncopal breathing instability, which lanma ve/veya instabilite ve senkop öncesi instabil solunum occur during a tilt test, are discussed in the same perspective. -
Ehlers-Danlos Syndrome and the Overlap with Orthostatic Intolerance
10/9/2014 Ehlers‐Danlos Syndrome Ehlers‐Danlos Syndrome and the Overlap with Orthostatic Intolerance • Heterogeneous disorder of connective tissue • Characterized by varying degrees of: October 9, 2014 Skin hyperextensibility Joint hypermobility Cutaneous fragility Peter C. Rowe, MD •Most forms of EDS result from mutations in genes Sunshine Natural Wellbeing Foundation Professor of encoding fibrillar collagens or the collagen‐ Chronic Fatigue and Related Disorders modifying enzymes Johns Hopkins University School of Medicine 1. Royce PM, Steinmann B, Superti‐Furga A. The Ehlers‐Danlos syndrome. In: Connective Tissue and its Heritable Baltimore, USA Disorders. New York: Wiley‐Liss, 1993: 351‐407. 2. de Paepe A, Malfait F. The Ehlers‐Danlos syndrome, a disorder with many faces. Clin Genetics 2012;82:1‐11. Presenter Disclosure Information Ehlers‐Danlos Syndrome Peter C. Rowe, MD • Prevalence unknown, estimated at 1:5000 •Because fibrillar collagen provides strength and structure to essentially all tissues and organs, EDS •No relationships to disclose has widespread clinical manifestations •Early varicose veins, easy bruising •Easy fatigability and widespread pain common Royce PM, Steinmann B, Superti‐Furga A. The Ehlers‐Danlos syndrome. In: Connective Tissue and its Heritable Disorders. New York: Wiley‐Liss, 1993: 351‐407. EDS, JH, and Orthostatic Intolerance Classification of EDS Beighton P, et al. Am J Med Genetics 1998;77:31‐7. Overview of Ehlers‐Danlos Syndrome Classical Illustrative case (formerly EDS I and II) Orthostatic intolerance in EDS and JH Hypermobility (formerly EDS III) Challenges Vascular (formerly EDS IV) Kyphoscoliosis Arthrochalasia 3 generations with Classical EDS. Note hemosiderin deposition in knees and Dermatosparaxis shins, varicose vein stripping on R 1 10/9/2014 de Paepe A, Malfait F. -
GP GUIDE to Pots (Postural Tachycardia Syndrome)
GP GUIDE TO PoTS (Postural Tachycardia Syndrome) 1 WHAT IS PoTS? PoTS was characterised in 1993, but previously existed under various other names including irritable heart, soldier’s heart and idiopathic orthostatic intolerance. It is a heterogeneous group of disorders sharing similar characteristics. On assuming upright posture, there is an excessive increase in heart rate associated with symptoms of orthostatic intolerance and sympathetic over-activity. There is brain hypoperfusion, usually in the absence of hypotension. When humans adopt upright posture, approximately 500ml of blood drops into the abdominal cavity and limbs. A normal autonomic nervous system responds with immediate peripheral vasoconstriction and an increase in heart rate of up to 20bpm. In POTS, it is considered that vasoconstriction is inadequate, resulting in pooling of blood, relative hypovolaemia and reduced venous return to the heart. Heart rate, inotropic status and, in some patients, catecholamine levels increase further to compensate. Dizziness and syncope can occur in the presence of normal BP; in fact some patients with PoTS have a hypertensive response to standing. HOW COMMON IS PoTS? The incidence in the UK is unknown. However, it is probably under-diagnosed due to lack of awareness and non-specific symptomatology. It is five times more common in women and tends to affect people age 15 to 50. 2 SYMPTOMS OF PoTS Dizziness GI upset Syncope / pre-syncope Sweating Orthostatic headache Nausea Fatigue Insomnia Poor memory Weakness Poor concentration Visual greying or blurring Sense of anxiety Acrocyanosis (purplish hands/feet) Exercise intolerance Palpitations (tachycardia / ectopics) Tremulousness Neck/shoulder pain (muscle ischaemia) Patients may have some or all of the above symptoms. -
What Is the Autonomic Nervous System?
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.74.suppl_3.iii31 on 21 August 2003. Downloaded from AUTONOMIC DISEASES: CLINICAL FEATURES AND LABORATORY EVALUATION *iii31 Christopher J Mathias J Neurol Neurosurg Psychiatry 2003;74(Suppl III):iii31–iii41 he autonomic nervous system has a craniosacral parasympathetic and a thoracolumbar sym- pathetic pathway (fig 1) and supplies every organ in the body. It influences localised organ Tfunction and also integrated processes that control vital functions such as arterial blood pres- sure and body temperature. There are specific neurotransmitters in each system that influence ganglionic and post-ganglionic function (fig 2). The symptoms and signs of autonomic disease cover a wide spectrum (table 1) that vary depending upon the aetiology (tables 2 and 3). In some they are localised (table 4). Autonomic dis- ease can result in underactivity or overactivity. Sympathetic adrenergic failure causes orthostatic (postural) hypotension and in the male ejaculatory failure, while sympathetic cholinergic failure results in anhidrosis; parasympathetic failure causes dilated pupils, a fixed heart rate, a sluggish urinary bladder, an atonic large bowel and, in the male, erectile failure. With autonomic hyperac- tivity, the reverse occurs. In some disorders, particularly in neurally mediated syncope, there may be a combination of effects, with bradycardia caused by parasympathetic activity and hypotension resulting from withdrawal of sympathetic activity. The history is of particular importance in the consideration and recognition of autonomic disease, and in separating dysfunction that may result from non-autonomic disorders. CLINICAL FEATURES c copyright. General aspects Autonomic disease may present at any age group; at birth in familial dysautonomia (Riley-Day syndrome), in teenage years in vasovagal syncope, and between the ages of 30–50 years in familial amyloid polyneuropathy (FAP). -
Cutaneous Findings in Patients on Anticoagulants Caleb Creswell, MD Dermatology Specialists Disclosure Information
Cutaneous findings in patients on Anticoagulants Caleb Creswell, MD Dermatology Specialists Disclosure Information • I have no financial relationships to disclose Objectives 1) Identify underlying causes of actinic or senile purpura 2) Recognize coumadin skin necrosis and understand proper treatment 3) Recognize heparin skin necrosis and understand that underlying HIT is often present Actinic (Senile) Purpura • Common on forearms of elderly individuals • Most important factor is chronic sun exposure – Thins dermal collagen and blood vessel walls • Anticoagulants may exacerbate but are rarely the main culprit Actinic Purpura Actinic Purpura Leukocytoclastic Vasculitis • Don’t mistake LCV for actinic purpura Ecchymoses • No topical agents have been shown to speed resorption of RBCs and hemosiderin • Pulsed-dye Laser can help Coumadin Induced Skin Necrosis • Coumadin: Occurs between 3-5 days after initiating therapy – Due to transient protein C deficiency – Increased risk with intrinsic protein C deficiency – Occurs in areas with significant adipose tissue – Treatment: Heparinize and continue coumadin Coumadin Induced Skin Necrosis Other Coumadin Skin Reactions • Extremely rare cause of morbilliform drug rash • Can cause leukocytoclastic vasculitis – Can occur weeks to months after starting medication Photo of Morbilliform CADR Leukocytoclastic Vasculitis Heparin Induced Skin Necrosis • Heparin: Occurs 1-14 days after starting – Often starts at injection site and spreads – Due to HIT Type II (Thrombocytopenia will be present) Heparin Induced -
Generalized Livedo Reticularis Like Eruption Induced by Trimethoprim/Sulfamethoxazole: a Case Report with Concomitant Myelosuppression
Our Dermatology Online Case Report GGeneralizedeneralized llivedoivedo rreticulariseticularis llikeike eeruptionruption iinducednduced bbyy ttrimethoprim/sulfamethoxazole:rimethoprim/sulfamethoxazole: A ccasease rreporteport wwithith cconcomitantoncomitant mmyelosuppressionyelosuppression Pinar Incel Uysal1, Basak Yalcin1, Onder Bozdogan2 1Dermatology Department, Ankara Numune Training and Research Hospital, Ankara, Turkey, 2Pathology Department, Ankara Numune Training and Research Hospital, Ankara, Turkey Corresponding author: Dr. Pinar Incel Uysal, E-mail: [email protected] ABSTRACT Livedo reticularis is a reticular discoloration of the skin because of the vascular anatomy of the skin. The condition most commonly affects the legs. Drug induced livedo reticularis which is an acknowledged side effect of amantadine, tends to be widespread, asymptomatic, benign rash. There are also reports of livedoid eruption induced with drugs including dapsone, imatinibe, gefitinibe. We describe a case of livedo reticularis like eruption and haemotological toxicity with trimetophrim-sulfamethoxazole. The purpose of this report is to remind clinicians of this rare, benign side effect of the common prescribed medication. Key words: Livedoid eruption; Skin rash; Trimetophrim-Sulfamethoxazole INTRODUCTION after introduction of trimetophrim-sulfamethoxazole (TMP-SMX). The rash and bone morrow suppression Livedo reticularis is a network like livedoid eruption disappeared after withdrawal of the drug. which commonly occurs after physical exposure such as cold. Drug induced livedo reticularis is a very rare CASE REPORT manifestation. In the literature there are reports that were associated with drugs including amantadine, dapsone, A 31-year-old woman with acute otitis media presented imatinibe, rasagiline [1-5]. These reactions usually show with widespread eruption for 4 days. She was prescribed benign course and resolve without complication after TMP-SMX for acute otitis media a week before the discontinuation of the causative drug. -
The Skin in Genetically-Controlled Metabolic Disorders P
Review Article J Med Genet: first published as 10.1136/jmg.10.2.101 on 1 June 1973. Downloaded from Journal of Medical Genetics (1973). 10, 101. The Skin in Genetically-controlled Metabolic Disorders P. C. H. NEWBOLD Department of Medicine, Cambridge University Medical School, Hills Road, Cambridge CB2 2QL Diseased nature oftentimes breaks forth however, it may lead to difficulty in assessing intelli- In strange eruptions.-Henry IV, part 1, III, i. gence, which is within normal range in 50% of The skin is now commonly accepted as a mirror patients. There is suggestive evidence of a re- of internal disease, but as with other looking-glasses, lationship between subnormal folate levels and low the evidence offered may be selected or ignored. intelligence (Carey et al, 1968), and studies have The epidermis is an interesting structure, especially shown increased turnover of folate coenzymes and rich in tyrosine, phenylalanine, tryptophan, and resulting folate depletion in these patients (Carey et histidine, when compared with the corium (Roth- al, 1968; Butterworth, Krumdieck, and Baugh, man, 1965). Tyrosinaemia and histidinaemia do 1971). There is also a high incidence of an organic not include cutaneous manifestations, but culture of brain syndrome following intracranial vascular skin fibroblasts is a helpful diagnostic tool for study- thromboses (Dunn, Perry, and Dolman, 1966), ing metabolic defects such as citrullinaemia, cysti- copyright. nosis, and maple-syrup urine disease (Scriver, 1969). Most of the conditions now to be described are rare, but if these metabolic diseases were com- mon, there could be no human race as we know it. Homocystinuria http://jmg.bmj.com/ This most informative anomaly was discovered during a study of mentally retarded patients in Ire- land (Carson and Neill, 1962).