Abstract 228: HYQVIA in Postural Orthostatic Tachycardia Syndrome(POTS)

2016 ◽  
Vol 119 (suppl_1) ◽  
Author(s):  
Muhammad M Ahsan ◽  
Tara Thompson ◽  
Chandralekha Ashangari ◽  
Amer Suleman
Keyword(s):  
Immune Deficiency ◽  
Blood Pressure ◽  
Volume Expansion ◽  
Orthostatic Intolerance ◽  
Autonomic Failure ◽  
Exercise Intolerance ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Primary Immune Deficiency ◽  
Severe Combined Immunodeficiencies ◽  
Common Variable

Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia associated with variety of symptoms like Headache, Abdominal discomfort, Dizziness/presyncope, Nausea, Fatigue, Lightheadedness, Sweating Sleep disorder, Tremor, Anxiety, Palpitations, Exercise intolerance which is believed to be caused by an underlying infectious and/or autoimmune trigger. HYQVIA is an immune globulin with a recombinant human hyaluronidase indicated for the treatment of Primary Immunodeficiency (PI) in adults. This includes, but is not limited to, common variable immunodeficiency (CVID), X-linked agammaglobulinemia, congenital agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies. HYQVIA is a gammaglobulin and is a slow metabolizer given subcutaneously once a month.There is data that Intravenous immunoglobulin(IVIg) helps POTS patients. We present a case of 57 year old female diagnosed with autonomic failure,orthostatic intolerance and primary immune deficiency. She was given IVIg for primary immune deficiency. She was previously reported for severe autonomic failure. From then, she was doing extremely well, had more energy and she thinks more clearly. She also had much better attitude. She was getting albumin every 2 weeks or so for volume expansion. She felt IVIg had helped her. Her immunoglobulin was switched from Gammagard to HYQVIA 35 grams. After she did, her blood pressure had gone up. She took it with albumin and she felt palpitations and chest pain.She was stopped on albumin, as albumin and HYQVIA combined could cause more volume expansion and push her into fluid overload or even congestive heart failure. After she stopped albumin without HYQVIA, she did well. Similarly, she cut back on midodrine which is an α1 adrenergic agonist, and works by stimulating receptors that noradrenaline normally works on. After swallowing, midodrine is rapidly converted into another, more active drug that binds to noradrenaline receptors causing blood vessels to narrow, thereby increasing blood pressure andreduction in heart rate in POTS patients. She was only HYQVIA and had been feeling extremely well.

Abstract 222: Nerve Conduction Study in Postural Orthostatic Tachycardia Syndrome

2016 ◽  
Vol 9 (suppl_2) ◽  
Author(s):  
Chandralekha Ashangari ◽  
Samreen F Asghar ◽  
Sadaf Syed ◽  
Amna A Butt ◽  
Amer Suleman
Keyword(s):  
Heart Rate ◽  
Nerve Conduction Study ◽  
Nerve Conduction ◽  
Clinical Symptoms ◽  
Orthostatic Intolerance ◽  
Tibial Nerve ◽  
Sensory Nerves ◽  
Exercise Intolerance ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Medical Diagnostic

Background: Postural orthostatic tachycardia syndrome (POTS) is an autonomic disturbance characterized by the clinical symptoms of orthostatic intolerance, mainly light headedness, fatigue, sweating, tremor, anxiety, palpitation, exercise intolerance and near syncope on upright posture. These are relieved on lying down. Patients also have a heart rate >120 beats/min (bpm) on standing or increase their heart rate by 30 bpm from a resting heart rate after standing for 10 min. A nerve conduction study (NCS) is a medical diagnostic test commonly used to evaluate the function, especially the ability of electrical conduction, of the motor and sensory nerves of the human body. The aim of this study is to demonstrate median, ulnar, peroneal, tibial nerve conduction results POTS patients. Methods: 177 patients were selected randomly from our clinic with POTS. Nerve conduction results of median, ulnar, peroneal, tibial nerves were reviewed from electronic medical records. Results: Out of 177 patients, 151 patients are females (85%, n=151, age 32.07±11.10), 26 patients are males (15%, n=26, age 29.08±17.40).Median nerve conduction results are 57.83 m/sec ±7.58 m/sec, Ulnar nerve conduction results are 56.62 m/sec ±6.85 m/sec, Peroneal nerve conduction results are 49.96 m/sec ±6.85 m/sec, Tibial nerve conduction results are 50.70 m/sec ±6.86 m/sec. Conclusion: The nerve conduction velocities tend to be within normal range in Postural Orthostatic Tachycardia Syndrome (POTS) patients.

Posturalinės ortostatinės tachikardijos sindromas

2013 ◽  
Vol 23 (1) ◽  
pp. 127-132
Author(s):  
Alina Vilkė ◽  
Justina Mikšaitė ◽  
Andrius Macas
Keyword(s):  
Heart Rate ◽  
Atrial Flutter ◽  
Autonomic Dysfunction ◽  
Mayo Clinic ◽  
Orthostatic Intolerance ◽  
Normal Sinus Rhythm ◽  
Exercise Intolerance ◽  
Vertical Position ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Patients Âgés

Orthostatic intolerance defines a group of symptoms characterized by cerebral hypo-perfusion and/or sympathetic activation that appear on standing upright and remit in the supine position. Patients may complain of headache, nausea, abdominal pain, light headedness, diminished concentration, syncope, anxiety, weakness, fatigue, exercise intolerance, palpitations, dyspepsia, and chest pain. POTS criteria: increased heart rate 30 beats/min or more contractions within the first 10 min of a change in the vertical position, there is no position-induced hypo-tension, orthostatic intolerance symptoms. POTS is the most common form of orthostatic intolerance. This is the most common syndrome among young people, who have autonomic dysfunction clinic. POTS patients ages - young, between 14 and 45 years. POTS ethology is heterogeneous. It was found that POTS can cause a variety of reasons, but which is primary and which are secondary - remains unclear. We assessed the case: 28 years old patient was hospitalized to Lithuanian University of Health Sciences Kaunas hospital for Abnormal nor epinephrine surgical treatment of oesophageal achalasia. The start of surgery clearance and adrenergic receptor sensitivity in idiopathic (laparoscopic cardiomiotomy) and gas insuffliation was madeorthostatic intolerance.without any complications. But when the patient‘s position was changed (reverse Tredelenburg) was monitorised atrial flutter (he-art rate 130 beats per minute, blood pressure 146/106 mmHg). For atrial flutter correction were used KCl, Mg SO4, and intravenous esmolol. After that, heart rate gradually decreased from 130 beats/ min to 92-80 beats/min. During all surgery, the patient‘s condition was stable, but a normal sinus rhythm observed at the end of operation, when the patient was returned to her primary position.There was a research in Mayo Clinic (Minnesota, USA) which objective - to investigate perioperative patients with postural orthostatic tachycardia syndrome (POTS) preparation, and to identify unexpected complications during operation. The research was conducted on the 152 patients to identify all surgical procedures performed during general anaesthesia between January 1, 1993 andDecember 31, 2006 at Mayo Clinic. There were selected 13 patients (12 women, 1 man) of 152. From research there was found that autonomic dysfunction associated with POTS may present unusual physiologic challenges in the perioperative period.

Postural Orthostatic Tachycardia Syndrome: Mechanisms and New Therapies

2020 ◽  
Vol 71 (1) ◽  
pp. 235-248 ◽  
Author(s):  
Philip L. Mar ◽  
Satish R. Raj
Keyword(s):  
Volume Expansion ◽  
Venous Return ◽  
Orthostatic Intolerance ◽  
Beta Blockers ◽  
Treatment Strategies ◽  
Upright Position ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Treatment Regimens ◽  
Pathophysiologic Mechanism ◽  
Pathophysiologic Mechanisms

Postural orthostatic tachycardia syndrome (POTS) is a clinically heterogeneous disorder with multiple contributing pathophysiologic mechanisms manifesting as symptoms of orthostatic intolerance in the setting of orthostatic tachycardia (increase in heart rate by at least 30 beats per minute upon assuming an upright position) without orthostatic hypotension. The three major pathophysiologic mechanisms include partial autonomic neuropathy, hypovolemia, and hyperadrenergic state. Patients often will exhibit overlapping characteristics from more than one of these mechanisms. The approach to the treatment of POTS centers on treating the underlying pathophysiologic mechanism. Stockings, abdominal binders, and vasoconstrictors are used to enhance venous return in partial neuropathic POTS. Exercise and volume expansion are the main treatment strategies for hypo-volemic POTS. For hyperadrenergic POTS, beta-blockers and avoidance of norepinephrine reuptake inhibitors is important. Attempts should be made to discern which pathophysiologic mechanism(s) may be afflicting patients so that treatment regimens can be individualized.

Downregulation of growth hormone in postural orthostatic tachycardia syndrome: insights from the SYSTEMA cohort

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Johansson ◽  
J Schulte ◽  
F Ricci ◽  
M Persson ◽  
R Sutton ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Heart Rate ◽  
Growth Hormone ◽  
Plasma Levels ◽  
Orthostatic Intolerance ◽  
Statistical Tests ◽  
Growth Hormone Level ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Funding Source ◽  
Significant Difference

Abstract Background Postural orthostatic tachycardia syndrome (POTS) is a variant of cardiovascular autonomic disorder occurring predominantly in young women. POTS is characterized by an excessive heart rate increase when assuming upright posture accompanied by symptoms of orthostatic intolerance. The pathophysiology of POTS has not been fully established and is believed to be multifactorial. Purpose We aimed to investigate the alterations in circulating growth hormone level in POTS. Methods We conducted an age-matched case-control study enrolling 42 patients with POTS (age 31±9 years; 36 women) verified by positive head-up tilt testing and cardiovascular autonomic tests, and 46 controls (32±9 years; 35 women) with negative active standing test and no history of syncope, orthostatic intolerance and endocrine disease. We measured plasma levels of growth hormone using a high-sensitivity chemiluminescence immunoassay in relation to presence of POTS diagnosis. All study participants completed the validated Orthostatic Hypotension Questionnaire (OHQ), consisting of two components: the symptoms assessment scale (OHSA) and daily activity scale (OHDAS) to evaluate the burden of symptoms. We applied standard statistical tests for group differences. Growth hormone values were log-transformed and standardized before the group comparison. Results POTS patients had significantly lower plasma levels of growth hormone (ng/mL) (median=0.53, IQR, 0.10–2.83 vs. median=2.33, IQR, 0.26–7.2, p=0.04) than controls. Levels of growth hormone were reversely related to OHDAS (p=0.049) among POTS patients. Supine heart rate was significantly higher in POTS patients (69.0±11.1 beats/min vs. 63.3±10.8 beats/min, p=0.02), as well as diastolic blood pressure (72.9±9.1 mmHg vs. 69.0±8.5 mmHg, p=0.04). We observed no significant difference in supine systolic blood pressure (116.6±13.3 mmHg vs. 115.2±10.0 mmHg, p=0.60). POTS patients had a significantly higher composite OHQ score than controls (60.0±18.6 vs. 4.2±7.5, p<0.001), as well as OHSA (36.2±10.0 vs. 3.6±6.4, p<0.001) and OHDAS (23.8±9.7 vs. 0.6±1.3, p<0.001). Conclusion(s) Our study shows that patients with POTS have significantly reduced plasma levels of circulating growth hormone. Lower growth hormone levels among POTS patients are associated with increased impairment of daily life activities. Further studies are necessary to confirm our findings in the independent populations and explain the mechanisms behind this alteration. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Crafoord Foundation, Swedish Heart and Lung Foundation

scholarly journals Distinct neurohumoral biomarker profiles in children with hemodynamically defined orthostatic intolerance may predict treatment options

2016 ◽  
Vol 310 (3) ◽  
pp. H416-H425 ◽  
Author(s):  
Ashley L. Wagoner ◽  
Hossam A. Shaltout ◽  
John E. Fortunato ◽  
Debra I. Diz
Keyword(s):  
Blood Pressure ◽  
Orthostatic Intolerance ◽  
Treatment Options ◽  
Pressure Control ◽  
Hemodynamic Response ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Compensatory Mechanisms ◽  
Oh Groups ◽  
Arterial Blood ◽  
Head Up Tilt

Studies of adults with orthostatic intolerance (OI) have revealed altered neurohumoral responses to orthostasis, which provide mechanistic insights into the dysregulation of blood pressure control. Similar studies in children with OI providing a thorough neurohumoral profile are lacking. The objective of the present study was to determine the cardiovascular and neurohumoral profile in adolescent subjects presenting with OI. Subjects at 10–18 yr of age were prospectively recruited if they exhibited two or more traditional OI symptoms and were referred for head-up tilt (HUT) testing. Circulating catecholamines, vasopressin, aldosterone, renin, and angiotensins were measured in the supine position and after 15 min of 70° tilt. Heart rate and blood pressure were continuously measured. Of the 48 patients, 30 patients had an abnormal tilt. Subjects with an abnormal tilt had lower systolic, diastolic, and mean arterial blood pressures during tilt, significantly higher levels of vasopressin during HUT, and relatively higher catecholamines and ANG II during HUT than subjects with a normal tilt. Distinct neurohumoral profiles were observed when OI subjects were placed into the following groups defined by the hemodynamic response: postural orthostatic tachycardia syndrome (POTS), orthostatic hypotension (OH), syncope, and POTS/syncope. Key characteristics included higher HUT-induced norepinephrine in POTS subjects, higher vasopressin in OH and syncope subjects, and higher supine and HUT aldosterone in OH subjects. In conclusion, children with OI and an abnormal response to tilt exhibit distinct neurohumoral profiles associated with the type of the hemodynamic response during orthostatic challenge. Elevated arginine vasopressin levels in syncope and OH groups are likely an exaggerated response to decreased blood flow not compensated by higher norepinephrine levels, as observed in POTS subjects. These different compensatory mechanisms support the role of measuring neurohumoral profiles toward the goal of selecting more focused and mechanistic-based treatment options for pediatric patients with OI.

Abstract 116: Antinuclear Antibody levels Study in Postural Orthostatic Tachycardia Syndrome

2016 ◽  
Vol 9 (suppl_2) ◽  
Author(s):  
Chandralekha Ashangari ◽  
Samreen F Asghar ◽  
Sadaf Syed ◽  
Amer Suleman
Keyword(s):  
Heart Rate ◽  
Medical Records ◽  
Antinuclear Antibody ◽  
Clinical Symptoms ◽  
Orthostatic Intolerance ◽  
Exercise Intolerance ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Autoimmune Reaction ◽  
Autonomic Disturbance ◽  
Antibody Levels

Background: Postural orthostatic tachycardia syndrome (POTS) is an autonomic disturbance characterized by the clinical symptoms of orthostatic intolerance, mainly light headedness, fatigue, sweating, tremor, anxiety, palpitation, exercise intolerance and near syncope on upright posture. These are relieved on lying down. Patients also have a heart rate >120 beats/min (bpm) on standing or increase their heart rate by 30 bpm from a resting heart rate after standing for 10 min. An antinuclear antibody (ANA) test measures the amount and pattern of antibodies in blood that work against own body (autoimmune reaction). If there are more antibodies in the blood than normal, the test is positive. The aim of this study is to demonstrate ANA levels in POTS. Methods: 151 patients were selected randomly from our clinic with POTS. Patients ANA levels were reviewed from electronic medical records and performed data analysis. Results: Out of 151 patients, 129 patients are females (85%, n=129, age 32±10.98), 22 patients are males (15%, n=22, age 27.90±12.11). 110/151(73%) patients had negative ANA, 41(27%) patients had positive ANA. Conclusion: Our research results demonstrated that ANA levels are positive in one fourth of Postural Orthostatic Tachycardia Syndrome (POTS) patients.

scholarly journals Novel mutation in the CD27 gene in a patient presenting with hypogammaglobulinemia, bronchiectasis and EBV-driven lymphoproliferative disease

2020 ◽  
Vol 13 (2) ◽  
pp. e233482 ◽  
Author(s):  
Rashmi Kishore ◽  
Aditya Gupta ◽  
Aditya Kumar Gupta ◽  
Sushil Kumar Kabra
Keyword(s):  
Immune Deficiency ◽  
Novel Mutation ◽  
Late Presentation ◽  
Lymphoproliferative Disease ◽  
Primary Immune Deficiency ◽  
Delay In Diagnosis ◽  
Barr Virus ◽  
Life Threatening ◽  
Epstein Barr ◽  
Common Variable

CD27 deficiency is a rare primary immune deficiency which affects T cells, B cells and NK cells and is associated with hypogammaglobulinemia. Clinical presentation varies from asymptomatic disease to life-threatening Epstein Barr Virus (EBV)-driven complications including malignancy. Delay in diagnosis and late presentation adversely affects the clinical outcome and survival. We report a 10-year-old girl who had been symptomatic since 3 years of age with recurrent infections, developed bronchiectasis and was found to have hypogammaglobulinemia. Initially diagnosed as common variable immune deficiency, she had persistent lymphadenopathy, hepatosplenomegaly and pancytopenia, raising a clinical suspicion of a lymphoproliferative condition. On investigation, she was found to have a novel mutation involving the CD27 gene with very high EBV load. She was given rituximab injections to which she showed partial response and later developed non-Hodgkin’s lymphoma .

Different haemodynamic patterns in head-up tilt test on 400 paediatric cases with unexplained syncope

2014 ◽  
Vol 25 (5) ◽  
pp. 911-917 ◽  
Author(s):  
Yilmaz Yozgat ◽  
Cem Karadeniz ◽  
Rahmi Ozdemir ◽  
Onder Doksoz ◽  
Mehmet Kucuk ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Orthostatic Intolerance ◽  
Vasovagal Syncope ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Response Patterns ◽  
Baseline Blood Pressure ◽  
Test Protocol ◽  
Tilt Testing ◽  
Head Up Tilt ◽  
Unexplained Syncope

AbstractObjective: To assess haemodynamic patterns in head-up tilt testing on 400 paediatric cases with unexplained syncope. Methods: Medical records of 520 children who underwent head-up tilt testing in the preceding year were retrospectively evaluated, and 400 children, 264 (66%) girls and 136 (34%) boys, aged 12.6±2.6 years (median 13; range 5–18), with unexplained syncope were enrolled in the study. Age, sex, baseline heart rate, baseline blood pressure, frequency of symptoms, and/or fainting attacks were recorded. The test protocol consisted of 25 minutes of supine resting followed by 20 minutes of 70° upright positioning. Subjects were divided into nine groups according to their differing haemodynamic patterns. Results: There were no statistically significant differences between the groups with regard to age, gender, baseline blood pressure, and frequency of syncope (p>0.05). The response was compatible with orthostatic intolerance in 28 cases (7.0%), postural orthostatic tachycardia syndrome in 24 cases (6.0%), asymptomatic postural orthostatic tachycardia syndrome in 26 cases (6.5%), orthostatic hypotension in seven cases (1.7%), vasovagal syncope in 38 cases (9.5%), and negative in 274 cases (69.2%). Vasovagal syncope response patterns were of type 3 in nine cases (2.2%), type 2A in 10 cases (2.5%), type 2B in two cases (0.5%), and type 1 (mixed) in 17 cases (4.25%). Conclusions: In the 400 paediatric cases with unexplained syncope, nine different haemodynamic response patterns to head-up tilt testing were discerned. Children with orthostatic intolerance syndromes are increasingly referred to hospitals because of difficulty in daily activities. Therefore, there is need for further clinical trials in these patient groups.

Implanted ports in adults with primary immunodeficiency

2018 ◽  
Vol 19 (4) ◽  
pp. 375-377
Author(s):  
James C Barton ◽  
Jackson Clayborn Barton ◽  
Luigi F Bertoli
Keyword(s):  
Immune Deficiency ◽  
Immunoglobulin G ◽  
Venous Access ◽  
Primary Immune Deficiency ◽  
Subcutaneous Immunoglobulin ◽  
Duration Of Treatment ◽  
Immunoglobulin G Subclasses ◽  
Catheter Fracture ◽  
Immunoglobulin G Subclass ◽  
Common Variable

Background: We sought to learn more about the utility and safety of implanted ports for monthly immunoglobulin G infusions in adults with primary immune deficiency. Methods: We reviewed charts of adults who were referred to a single practice during the interval 2006–2016 for evaluation and management of frequent or severe upper and lower respiratory tract and other infections, subnormal total immunoglobulin G or immunoglobulin G subclasses, and suboptimal responses to polyvalent pneumococcal polysaccharide vaccinations; were diagnosed to have primary immune deficiency; and were advised to undergo immunoglobulin G therapy. Results: Of 606 patients, 20 (19 women, 1 man; 16 immunoglobulin G subclass deficiency, 4 common variable immunodeficiency; 3.3%) needed implanted ports because they had inadequate upper extremity superficial venous access. Median age at diagnosis was 48 years (range: 32–65 years). In total, 17 of the 20 patients preferred monthly in-office intravenous immunoglobulin G treatment to weekly at-home subcutaneous immunoglobulin G. The other three patients could not be treated with subcutaneous immunoglobulin G (unfavorable self-treatment experiences and insurance limitations). Median duration of treatment via implanted ports was 73 months (range: 10–153 months). In the man, the first implanted port was replaced after 26 months due to catheter fracture of unknown cause. His second port has been used for 112 months. We observed no other port-related failure, infections, thrombosis, or other adverse events. Conclusion: The utility and safety of implanted ports in adults with primary immune deficiency for whom subcutaneous immunoglobulin G therapy is not desired or feasible are probably similar to those of ports in patients without primary immune deficiency.

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