MCID: PRM227
MIFTS: 23

Primary Orthostatic Hypotension

Categories: Eye diseases, Fetal diseases, Genetic diseases, Metabolic diseases, Nephrological diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Primary Orthostatic Hypotension

MalaCards integrated aliases for Primary Orthostatic Hypotension:

Name: Primary Orthostatic Hypotension 20 58
Neurogenic Orthostatic Hypotension 20

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Orphanet 58 ORPHA182058

Summaries for Primary Orthostatic Hypotension

GARD : 20 P rimary orthostatic hypotension is a rare type of orthostatic hypotension. It is not a disease per se, but a condition caused by several disorders that affect a specific part of the autonomic nervous system, such as multiple system atrophy, young-onset Parkinson's disease, pure autonomic failure, dopamine beta-hydroxylase deficiency, familial dysautonomia, and pure autonomic failure among others. The autonomic nervous system is the part of the nervous system that regulates certain involuntary body functions such as heart rate, blood pressure, sweating, and bowel and bladder control. Orthostatic hypotension is a form of low blood pressure that happens when standing-up from sitting or lying down. Common symptoms may include dizziness, lightheadedness, generalized weakness, leg buckling, nausea, blurry vision, fatigue, and headaches. Additional symptoms can include chest pain (angina), head and neck pain (often affecting neck and shoulders with a coat hanger distribution), decline in cognitive functioning such as difficulty concentrating, temporary loss of consciousness or "blackout". Some people with primary orthostatic hypotension may also have high blood pressure when lying down. The treatment depends upon several factors including the specific underlying cause including The treatment depends upon several factors including the specific underlying cause and may include physical counter-maneuvers like lying down, sitting down, squatting clenching buttocks, leg crossing, and support garment and medication.

MalaCards based summary : Primary Orthostatic Hypotension, also known as neurogenic orthostatic hypotension, is related to genetic primary orthostatic hypotension and multiple system atrophy 1. An important gene associated with Primary Orthostatic Hypotension is ACHE (Acetylcholinesterase (Cartwright Blood Group)). The drugs Droxidopa and Dopamine have been mentioned in the context of this disorder. Affiliated tissues include spinal cord.

Related Diseases for Primary Orthostatic Hypotension

Diseases in the Primary Orthostatic Hypotension family:

Orthostatic Hypotension 1 Orthostatic Hypotension 2

Diseases related to Primary Orthostatic Hypotension via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 43)
# Related Disease Score Top Affiliating Genes
1 genetic primary orthostatic hypotension 11.3
2 multiple system atrophy 1 10.8
3 parkinson disease, late-onset 10.8
4 autonomic neuropathy 10.6
5 pure autonomic failure 10.6
6 orthostatic intolerance 10.3
7 diabetic autonomic neuropathy 10.3
8 dysautonomia 10.3
9 dementia, lewy body 10.2
10 parkinsonism 10.2
11 amyloidosis 10.2
12 diabetic neuropathy 10.2
13 type 2 diabetes mellitus 10.1
14 hypertension, essential 10.1
15 varicose veins 10.1
16 supranuclear palsy, progressive, 1 10.1
17 coronary heart disease 1 10.1
18 spastic paraplegia, intellectual disability, nystagmus, and obesity 10.1
19 atrioventricular block 10.1
20 first-degree atrioventricular block 10.1
21 atrial fibrillation 10.1
22 urinary tract infection 10.1
23 postural orthostatic tachycardia syndrome 10.1
24 autonomic nervous system disease 10.1
25 polyneuropathy 10.1
26 congestive heart failure 10.1
27 neuropathy 10.1
28 rem sleep behavior disorder 10.1
29 achalasia 10.1
30 diabetes mellitus 10.1
31 hypoglycemia 10.1
32 amyloid neuropathy 10.1
33 baroreflex failure 10.1
34 fainting 10.1
35 pik3ca-related overgrowth syndrome 10.1
36 anhidrosis 10.0
37 neurogenic bladder 10.0
38 diarrhea 10.0
39 impotence 10.0
40 central nervous system disease 10.0
41 nervous system disease 10.0
42 autonomic dysfunction 10.0
43 syncope 10.0

Graphical network of the top 20 diseases related to Primary Orthostatic Hypotension:



Diseases related to Primary Orthostatic Hypotension

Symptoms & Phenotypes for Primary Orthostatic Hypotension

Drugs & Therapeutics for Primary Orthostatic Hypotension

Drugs for Primary Orthostatic Hypotension (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 30)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Droxidopa Approved, Investigational Phase 4 23651-95-8 443940
2
Dopamine Approved Phase 4 62-31-7, 51-61-6 681
3
Fludrocortisone Approved, Investigational Phase 4 127-31-1 31378
4
Midodrine Approved Phase 4 133163-28-7, 42794-76-3 4195
5 Pyridostigmine Bromide Phase 4 101-26-8
6 Anticonvulsants Phase 4
7 Cholinergic Agents Phase 4
8 Cholinesterase Inhibitors Phase 4
9 Bromides Phase 4
10 Antiparkinson Agents Phase 4
11 Anti-Inflammatory Agents Phase 4
12 Adrenergic alpha-Agonists Phase 4
13 Vasoconstrictor Agents Phase 4
14 Adrenergic Agents Phase 4
15 Neurotransmitter Agents Phase 4
16 Sympathomimetics Phase 4
17 Adrenergic Agonists Phase 4
18
Norepinephrine Approved Phase 3 51-41-2 439260
19
Mannitol Approved, Investigational Phase 3 69-65-8 6251 453
20 diuretics Phase 3
21 Atomoxetine Hydrochloride Phase 2
22
Yohimbine Approved, Investigational, Vet_approved Early Phase 1 146-48-5 8969
23
Melatonin Approved, Nutraceutical, Vet_approved Early Phase 1 73-31-4 896
24 Adrenergic alpha-Antagonists Early Phase 1
25 Adrenergic Antagonists Early Phase 1
26 Mydriatics Early Phase 1
27 Antioxidants Early Phase 1
28 Protective Agents Early Phase 1
29 Antihypertensive Agents Early Phase 1
30 Yohimbe Early Phase 1

Interventional clinical trials:

(show all 30)
# Name Status NCT ID Phase Drugs
1 Efficacy and Safety of Midodrine and Atomoxetine Treatment in Patients With Neurogenic Orthostatic Hypotension : A Prospective Randomized Study Completed NCT03350659 Phase 4 Midodrine;Atomoxetine
2 A Multi-Center, Double-Blind, Randomized, Placebo-Controlled, Crossover Study to Assess the Clinical Benefit of Midodrine Hydrochloride in Patients With Neurogenic Orthostatic Hypotension Completed NCT00046475 Phase 4 Midodrine Hydrochloride
3 Treatment and Prognosis of Neurogenic Orthostatic Hypotension : A Prospective Randomized Study Completed NCT02308124 Phase 4 Midodrine;Pyridostigmine Bromide;Midodrine + pyridostigmine
4 A Multi-Center, Double-Blind, Randomized, Placebo-controlled, Crossover Study to Assess the Clinical Benefit of Midodrine Hydrochloride in Subjects With Moderate to Severe Neurogenic Orthostatic Hypotension Completed NCT00555880 Phase 4 Midodrine hydrochloride;Placebo
5 RESTORE: A Clinical Study of Patients With Symptomatic Neurogenic Orthostatic Hypotension to Assess Sustained Effects of Droxidopa Therapy Recruiting NCT02586623 Phase 4 Droxidopa capsules;Placebo capsules
6 Evaluation of Efficacy and Tolerance of Fludrocortisone (Flucortac) in the Treatment of Orthostatic Neurogenic Hypotension Recruiting NCT04128137 Phase 4 Fludrocortisone
7 Evaluation of Brain Perfusion and Oxygenation in PD Patients With Neurogenic Orthostatic Hypotension: 4 Week Comparison of Droxidopa Versus Placebo Recruiting NCT03229174 Phase 4 Droxidopa;Placebo
8 A Phase IV, Multi-Center, Double-Blind, Parallel Group, Randomized, Placebo-Controlled Study to Assess the Clinical Benefit of Three Doses of Midodrine Hydrochloride (ProAmatine®) in Subjects With Neurogenic Orthostatic Hypotension Terminated NCT00046163 Phase 4 midodrine hydrochloride (ProAmatine®)
9 Phase III, Multi-Center, Study to Assess the Clinical Effect of Droxidopa in Subjects With Primary Autonomic Failure, Dopamine Beta Hydroxylase Deficiency or Non-Diabetic Neuropathy and Symptomatic NOH Completed NCT00782340 Phase 3 Placebo;Droxidopa
10 An Open-label Study, to Assess the Long-term Safety and Clinical Benefit of Droxidopa in Subjects With PAF, Dopamine Beta Hydroxylase Deficiency or Non-diabetic Neuropathy and Symptomatic Neurogenic Orthostatic Hypotension Completed NCT00738062 Phase 3 Droxidopa;Placebo
11 Phase III, Multi-Center, Study to Assess the Clinical Effect of Droxidopa in Subjects With Primary Autonomic Failure, Dopamine Beta Hydroxylase Deficiency or Non-Diabetic Neuropathy and Symptomatic NOH Completed NCT00633880 Phase 3 Placebo;Droxidopa
12 A Multi-center, Open-label Study to Assess the Long-term Safety of Droxidopa in Subjects With Primary Autonomic Failure, Dopamine Beta Hydroxylase Deficiency or Non-Diabetic Neuropathy and Symptomatic Neurogenic Orthostatic Hypotension Completed NCT01132326 Phase 3 Droxidopa
13 A Multi-center, Double-blind, Randomized, Parallel-Group, Placebo-Controlled Study to Assess the Clinical Effect of Droxidopa in the Treatment of Symptomatic Neurogenic Orthostatic Hypotension in Patients With Parkinson's Disease Completed NCT01176240 Phase 3 Droxidopa
14 A Phase 3, 4-week, Multicenter, Randomized, Double-blind, Placebo-controlled, Parallel-group Study of TD-9855 in Treating Symptomatic Neurogenic Orthostatic Hypotension in Subjects With Primary Autonomic Failure Recruiting NCT03750552 Phase 3 ampreloxetine;Placebo
15 A Phase 3, 22-week, Multi-center, Randomized Withdrawal Study of TD-9855 in Treating Symptomatic Neurogenic Orthostatic Hypotension in Subjects With Primary Autonomic Failure Recruiting NCT03829657 Phase 3 ampreloxetine;Placebo
16 A Phase 3, 182-week, Open-Label, Extension Study to Investigate the Safety and Tolerability of TD-9855 in Treating Symptomatic Neurogenic Orthostatic Hypotension (Symptomatic nOH) in Subjects With Primary Autonomic Failure Recruiting NCT04095793 Phase 3 ampreloxetine
17 A Clinical Study of Patients With Symptomatic Neurogenic Orthostatic Hypotension to Assess Sustained Effects of Droxidopa Therapy Terminated NCT01927055 Phase 3 Droxidopa;Placebo
18 A Phase II, Randomised, Placebo-Controlled, Double-Blind, Replicated Crossover, Pilot Study on the Effect of Fipamezole on Neurogenic Orthostatic Hypotension in Patients With Multiple System Atrophy or Parkinson's Disease Unknown status NCT00758849 Phase 2 Placebo;Fipamezole
19 A Phase 2 Study to Assess the Effect of TD-9855 in Subjects With Neurogenic Orthostatic Hypotension Completed NCT02705755 Phase 2 TD-9855;Placebo
20 Phase II Study of Midodrine for Neurogenic Orthostatic Hypotension Completed NCT00004268 Phase 2 midodrine
21 Effect of L-Dihydroxyphenylserine (L-DOPS) on Falls in Patients With Neurogenic Orthostatic Hypotension (NOH) Recruiting NCT03567447 Phase 2 Droxidopa
22 Phase 2 Norepinephrine Transporter Blockade, Autonomic Failure IND117394 12/28/12 Active, not recruiting NCT02784535 Phase 2 Atomoxetine;Placebo
23 An Open-Label Study To Assess The Clinical Benefit Of Droxidopa In Subjects With Chronic Fatigue Syndrome Terminated NCT00977171 Phase 2 Droxidopa
24 A Randomized, Open-Label, Three-Period, Three-Sequence, Single-Dose Crossover and Separate Three-Daily-Dose Treatment Period Study Comparing the Pharmacokinetic Profiles Following Oral Dosing of 300 mg of Droxidopa in the Fed Versus Fasted State, the Bioequivalence of Three 100 mg Capsules of Droxidopa Versus a Single 300 mg Capsule of Droxidopa, and 300 mg of Droxidopa Given Three Times at Four Hour Intervals in Healthy, Elderly Subjects Completed NCT01149629 Phase 1 Droxidopa;Droxidopa;Droxidopa
25 The Efficacy of Adjustable Lower Abdominal Compression in Neurogenic Orthostatic Hypotension Completed NCT01223391
26 Norepinephrine Transporter Blockade as a Pathophysiological Biomarker in Neurogenic Orthostatic Hypotension Completed NCT01316666
27 Effect of Midodrine vs Abdominal Compression on Cardiovascular Risk Markers in Autonomic Failure Patients Recruiting NCT04620382 Early Phase 1 Midodrine;Placebo pill
28 Use of Accelerometer for Quantification of Neurogenic Orthostatic Hypotension Symptoms Recruiting NCT04782830 placebo pill;Midodrine or atomoxetine pill
29 Assessment of Inspiratory Breathing Devices to Improve Orthostatic Tolerance in Neurogenic Orthostatic Hypotension Active, not recruiting NCT00962884
30 Identifying the Pathophysiology of Neurogenic Orthostatic Hypotension and the Effects of Melatonin on Reducing Supine Hypertension in Peripherally Intact Versus Denervated Post-ganglionic Sympathetic Nerves Terminated NCT02963181 Early Phase 1 Yohimbine;Melatonin

Search NIH Clinical Center for Primary Orthostatic Hypotension

Genetic Tests for Primary Orthostatic Hypotension

Anatomical Context for Primary Orthostatic Hypotension

MalaCards organs/tissues related to Primary Orthostatic Hypotension:

40
Spinal Cord

Publications for Primary Orthostatic Hypotension

Articles related to Primary Orthostatic Hypotension:

(show top 50) (show all 290)
# Title Authors PMID Year
1
Neurogenic Orthostatic Hypotension. Lessons From Synucleinopathies. 61
33705537 2021
2
Do indices of baroreflex failure and peripheral noradrenergic deficiency predict the magnitude of orthostatic hypotension in Lewy body diseases? 61
33710459 2021
3
Characterization of the changes in supine blood pressure with long-term use of droxidopa in patients with neurogenic orthostatic hypotension. 61
33521332 2021
4
Pharmacokinetics and pharmacodynamics of ampreloxetine, a novel, selective norepinephrine reuptake inhibitor, in symptomatic neurogenic orthostatic hypotension. 61
33782836 2021
5
Management Strategies for Comorbid Supine Hypertension in Patients with Neurogenic Orthostatic Hypotension. 61
33687577 2021
6
Quantitative analysis of dysautonomia in patients with autonomic dysreflexia. 61
33634338 2021
7
Factors contributing to racial differences in neurogenic orthostatic hypotension. 61
33550496 2021
8
Clinical and neurohormonal characteristics in African Americans with neurogenic orthostatic hypotension. 61
33502643 2021
9
Pharmacokinetics of Ampreloxetine, a Norepinephrine Reuptake Inhibitor, in Healthy Subjects and Adults with Attention-Deficit/Hyperactive Disorder or Fibromyalgia Pain. 61
32856281 2021
10
A Call to Action: The Role of Healthcare Providers in Reducing the Burden Associated with Neurogenic Orthostatic Hypotension. 61
32720118 2020
11
Pharmacologic treatment of orthostatic hypotension. 61
32979782 2020
12
Validation of the new index of baroreflex function to identify neurogenic orthostatic hypotension. 61
33189018 2020
13
Trick or Treat? Licorice-Induced Hypokalemia: A Case Report. 61
33391895 2020
14
Neurogenic Orthostatic Hypotension: An Underrecognized Complication of Parkinson Disease. 61
32649380 2020
15
Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia. 61
32906171 2020
16
Autonomic Dysfunction in the Synucleinopathies. 61
32906169 2020
17
Cardiac sympathetic innervation and vesicular storage in pure autonomic failure. 61
32945121 2020
18
Clinical Trials for Neurogenic Orthostatic Hypotension: A Comprehensive Review of Endpoints, Pitfalls, and Challenges. 61
32906173 2020
19
Management of Orthostatic Hypotension, Postprandial Hypotension, and Supine Hypertension. 61
33058087 2020
20
Validation of the Neurogenic Orthostatic Hypotension Ratio with Active Standing. 61
32596818 2020
21
Multiple System Atrophy - Cerebellar Type: Clinical Picture and Treatment of an Often-Overlooked Disorder. 61
33173654 2020
22
Management of neurogenic orthostatic hypotension in the heart failure patient. 61
32559655 2020
23
[Non-drug therapy for neurogenic orthostatic hypotension]. 61
32594694 2020
24
Mechanisms underlying unawareness of neurogenic orthostatic hypotension. 61
32170599 2020
25
Droxidopa as an effective treatment for refractory neurogenic orthostatic hypotension and reflex bradycardia in amyloid light-chain amyloidosis: a case report. 61
32560740 2020
26
Cardiovascular autonomic function testing in multiple system atrophy and Parkinson's disease: an expert-based blinded evaluation. 61
32415621 2020
27
The impact of supine hypertension on target organ damage and survival in patients with synucleinopathies and neurogenic orthostatic hypotension. 61
32516630 2020
28
Parkinson Disease and Orthostatic Hypotension in the Elderly: Recognition and Management of Risk Factors for Falls. 61
32489712 2020
29
Cardiovascular Safety Considerations in the Treatment of Neurogenic Orthostatic Hypotension. 61
32204870 2020
30
Neurogenic orthostatic hypotension induced by tizanidine. 61
31535247 2020
31
Efficacy of atomoxetine versus midodrine for neurogenic orthostatic hypotension. 61
32202069 2020
32
Cardiovascular Disorders Mediated by Autonomic Nervous System Dysfunction. 61
31764015 2020
33
Autonomic History, Examination, and Laboratory Evaluation. 61
31996620 2020
34
Persistent unexplained chest pain and dyspnea in a patient with coronary artery disease: a case report. 61
32019498 2020
35
Evidence of Impaired Cerebellar Connectivity at Rest and During Autonomic Maneuvers in Patients with Autonomic Failure. 61
31529276 2020
36
Real-world droxidopa or midodrine treatment persistence in patients with neurogenic orthostatic hypotension or orthostatic hypotension. 61
32200263 2020
37
Management of Orthostatic Hypotension. 61
31996627 2020
38
Neurogenic Orthostatic Hypotension: State of the Art and Therapeutic Strategies. 61
32943966 2020
39
Efficacy of atomoxetine versus midodrine for neurogenic orthostatic hypotension. 61
31856425 2020
40
Association of innervation-adjusted alpha-synuclein in arrector pili muscles with cardiac noradrenergic deficiency in autonomic synucleinopathies. 61
31673840 2019
41
Neurogenic Orthostatic Hypotension in Parkinson Disease: A Primer. 61
31456212 2019
42
Neurogenic orthostatic hypotension impairs information processing speed and attention. 61
31526820 2019
43
Management of coexistent neurogenic orthostatic hypotension and supine hypertension. 61
31599484 2019
44
Progressive supranuclear palsy is not associated with neurogenic orthostatic hypotension. 61
31484717 2019
45
Pure autonomic failure presenting as Harlequin syndrome. 61
31331696 2019
46
Cognitive and Behavioral Changes in Patients Treated With Droxidopa for Neurogenic Orthostatic Hypotension: A Retrospective Review. 61
31517701 2019
47
Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy. 61
31506870 2019
48
Orthostatic hypotension in hereditary transthyretin amyloidosis: epidemiology, diagnosis and management. 61
31452021 2019
49
(r)Alpha Lipoic Acid Is a Safe, Effective Pharmacologic Therapy of Chronic Orthostatic Hypotension Associated with Low Sympathetic Tone. 61
31548784 2019
50
Initiation of droxidopa during hospital admission for management of refractory neurogenic orthostatic hypotension in severely ill patients. 61
31368635 2019

Variations for Primary Orthostatic Hypotension

Expression for Primary Orthostatic Hypotension

Search GEO for disease gene expression data for Primary Orthostatic Hypotension.

Pathways for Primary Orthostatic Hypotension

GO Terms for Primary Orthostatic Hypotension

Sources for Primary Orthostatic Hypotension

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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