Postural orthostatic tachycardia syndrome (POTS) is a debilitating disease that predominantly affects young women. It is a multifactorial disorder that is characterized by severe tachycardia and orthostatic intolerance. Patients with POTS experience a variety of cardiac, neurological, and immunological symptoms that significantly reduce quality of life. In this review, a comprehensive framework is provided to aid in helping identify and treat patients with POTS. Given its heterogenous nature, it is crucial to understand each component of POTS in relation to one another instead of distinct parts. The framework highlights the overlap among the five main subtypes of POTS based on its pathophysiology (neuropathic, hypovolemic, primary hyperadrenergic, joint-hypermobility-related, and immune-related). Emphasis is placed on incorporating a multidisciplinary approach when treating patients with POTS, especially with a new focus towards immunotherapy. Although research has advanced our knowledge of POTS, there is still a critically unmet need to further our understanding and provide patients with the relief they need.

Postural orthostatic tachycardia syndrome (POTS) is increasingly recognized as a complication affecting recovery from concussion. Individuals with POTS demonstrate refractory dizziness, lightheadedness, cognitive dysfunction, fatigue, headache, chronic pain, nausea and gastrointestinal dysmotility, activity and exercise intolerance, syncope, and tachycardia. Subtypes of POTS may include hypovolemia, hyperadrenergic states, autonomic neuropathy, and underlying autoimmunity, which may variably impact response to rehabilitation in varying ways. The subtle presentation of POTS postconcussion is often mistaken for underlying anxiety, conversion disorder, or lack of motivation for recovery. This article will present clinical features of POTS that may arise after concussion, and propose a role for physical therapists in the diagnosis and management of POTS during concussion recovery. Data recorded and entered into a database during clinic visits from a large pediatric institution indicate that 11.4% of individuals diagnosed with POTS report onset of symptoms within 3 months of sustaining a concussion. Activation of the sympathetic nervous system can result in lightheadedness, shortness of breath, chest pain, tachycardia, palpitations on standing or with exertion, and activity and exercise intolerance. Identified comorbidities in people with POTS such as joint hypermobility and autoimmune disorders can further influence recovery. Physical therapists may identify signs and symptoms of POTS in a subset of individuals who remain refractory to typical interventions and who exhibit symptom exacerbation with orthostatic activity. Incorporation of an individualized POTS exercise program into current established concussion interventions may be useful, with emphasis on initial recumbent exercises and ongoing physical therapy assessment of exercise tolerance for dosing of activity intensity and duration.Video Abstract available for more insights from the authors (see Supplemental Digital Content 1, available at: http://links.lww.com/JNPT/A211).

The anaphylactoid reaction described follows cessation of ranitidine in a 19-year-old female with the disease cluster: mast cell activation syndrome, hypermobile Ehlers-Danlos syndrome and postural tachycardia syndrome. Anaphylaxis can give wide-ranging symptoms from rhinorrhoea and urticaria to tachycardia and system-wide, life-threatening, anaphylactic shock. Individuals with a disorder of mast cell activation can experience many such symptoms. H receptor antagonists, such as ranitidine, are commonly prescribed in this population. A mechanism for the reaction is proposed in the context of ranitidine, as an inverse agonist, causing upregulation of H histamine receptors and raised histamine levels due to enzyme induction. This effect, following extended and/or high antihistamine dosing, may have implications for other individuals with a disorder of mast cell activation, such as mastocytosis or mast cell activation syndrome. There are potential policy and patient guidance implications for primary and secondary care with respect to cessation of H antagonists.

Cognitive and emotional processes are influenced by interoception (homeostatic somatic feedback), particularly when physiological arousal is unexpected and discrepancies between predicted and experienced interoceptive signals may engender anxiety. Due to the vulnerability for comorbid psychological symptoms in forms of orthostatic intolerance (OI), this study investigated psychophysiological contributions to emotional symptomatology in 20 healthy control participants (13 females, mean age 36 ± 8 years), 20 postural tachycardia syndrome (PoTS) patients (18 females, mean age 38 ± 13 years) and 20 vasovagal syncope (VVS) patients (15 females, mean age 39 ± 12 years). We investigated indices of emotional orienting responses (OR) to randomly presented neutral, pleasant and unpleasant images in the supine position and during the induced interoceptive threat of symptom provocation of head-up tilt (HUT). PoTS and VVS patients produced greater indices of emotional responsivity to unpleasant images and, to a lesser degree, pleasant images, during interoceptive threat. Our findings are consistent with biased deployment of response-focused emotion regulation (ER) while patients are symptomatic, providing a mechanistic underpinning of how pathological autonomic overexcitation predisposes to anxiogenic traits in PoTS and VVS patients. This hypothesis may improve our understanding of why orthostasis exacerbates cognitive symptoms despite apparently normal cerebral autoregulation, and offer novel therapeutic targets for behavioural interventions aimed at reducing comorbid cognitive-affective symptoms in PoTS and VVS.

Postural tachycardia syndrome (POTS) is a form of dysautonomia which presents with complex symptoms including orthostatic intolerance. Several medications are prescribed for POTS; however, the efficacy of sustained medical treatment has not been well-investigated. Here, we conducted a 2 × 2 factorial design, randomized, clinical trial of a 3-month medical treatment regimen in POTS patients. Patients were randomly allocated to 4 treatment groups (Group 1: propranolol; Group 2: bisoprolol; Group 3: propranolol + pyridostigmine; Group 4: bisoprolol + pyridostigmine). The orthostatic intolerance questionnaire (OIQ), Beck depression inventory-II (BDI-II), and short-form health survey (SF-36) were conducted at baseline, 1 and 3 months after treatment. Seventy-seven patients who completed the 3-month follow-up were analyzed. In total, every clinical score improved significantly after medical treatment. The OIQ score was significantly lower than that at baseline (18.5 ± 6.7) after 1 month (12.5 ± 4.5, P < 0.01), which decreased further after 3 months (7.8 ± 5.7, P < 0.01). The OIQ score improvements were consistent across every treatment group. In the subgroup analysis of 59 patients who did not receive antidepressants, the BDI-II score significantly decreased after treatment, regardless of the regimen. Physical components of the SF-36 improved after 3 months in every group, while mental components improved only in Group 3. The amount of changes in each score was similar among groups throughout the comparisons. Sustained medical treatment is beneficial to POTS patients, not only for orthostatic intolerance symptoms but also for depression and diminished quality of life, even without prescriptions for antidepressants. The efficacy of each regimen in POTS patients was comparable. NCT02171988.

To investigate whether there is a difference in the neuro-autonomic and hemodynamic parameters in patients with postural tachycardia syndrome (POTS) according to onset time of orthostatic tachycardia during the Head-up tilt (HUT) test. We retrospectively reviewed neuro-autonomic and hemodynamic data from patients with orthostatic intolerance. A standardized battery of neuro-autonomic tests, including the HUT, Valsalva maneuver, heart rate deep breathing and quantitative sudomotor axon reflex test, was performed. The beat-to-beat derived hemodynamic parameters, including systolic blood pressure (BP) (SBP), mean BP (MBP), diastolic BP, heart rate (HR), total peripheral resistance, stroke volume, and cardiac output, were also extracted from HUT test. According to onset time of orthostatic tachycardia (OT), we divided patients into two groups; HR increase "within 10 minutes" (i.e., early OT) and HR increase "after 10 minutes" (i.e., late OT). We identified 210 patients with OT during HUT test. Forty-three percent of patients were included in early OT group and 57% were late OT group. Patients in late OT group were older than early OT group. Late OT group tended to have baseline hypertension more frequently compared to early OT group. After adjusting for age, late OT group showed higher baseline SBP and MBP, and lower increase of HR during HUT test than early OT group. However, other neuro-autonomic and hemodynamic parameters did not show significant differences between two groups. Orthostatic tachycardia might be developed in the late period during the HUT test. There were no remarkable differences in neuro-autonomic and hemodynamic parameters between early and late OT groups. It seems reasonable to extend the duration of HUT test to at least 20 min, to increase the diagnostic yield in patients with a history of orthostatic intolerance suspected as having POTS.

Complete congenital stationary night blindness (CSNB) is most often x-linked or recessive, and associated with a transmission defect from photoreceptors to bipolar cells. This produces a characteristic "negative" Schubert-Bornschein type of scotopic rod-cone electroretinogram (ERG) with a large a-wave and minimal b-wave. CSNB from abnormalities in phototransduction can be recessive or dominant and is much less common. This produces a Riggs type of ERG with loss of the rod a-wave as well as the b-wave. We report the clinical and ERG findings from a family with autosomal dominant CSNB that was shown previously to have a new GNAT1 mutation with a novel mechanism of action. They provide a classic demonstration of the Riggs-type ERG and have an unusual systemic association. Clinical case report of a father and daughter. A Chinese father and daughter presented with good visual acuity, moderate myopia, and lifelong night blindness. Both show normal fundi except for mild myopia, and fundus autofluorescence and OCT images are normal. Their ERGs illustrate the typical Riggs-type ERG with no rod a-wave (they have only a small cone-dominated combined response). They also have postural orthostatic tachycardia syndrome (POST), which is an autonomic dysfunction disorder thought usually to be sporadic. The retinal gene analyses revealed no abnormalities that might account for POST. Our family's ERG showed essentially no rod response, consistent with a Danish GNAT1 pedigree but different from the Nougaret GNAT1 pedigree that shows partial preservation of rod signal. A genetic connection between CSNB and POST would be intriguing, but we found no evidence for this.

Mast cell activation disorders (MCADs) consist of episodic systemic symptoms due to mast cell mediator release. Diagnosis is based on clinical presentation and determination of high levels of tryptase or histamine. Ehlers-Danlos syndrome (EDS) and postural tachycardia syndrome (POTS) frequently coexist. It has been described that individuals with these syndromes can even present symptoms compatible to MCADs, which could represent a new specific phenotype. Preliminary genetic data suggest a role for tryptase in the pathogenesis of MCADs, EDS, and POTS association. Studies with larger samples evaluating clinics, genetics, and histopathology are required to define the real correlation between these 3 clinical entities.

To identify the evidence base and evaluate the efficacy of each treatment for postural tachycardia syndrome (POTS) in light of a recent consensus statement highlighting the lack of treatment options with clear benefit to risk ratios for this debilitating condition. The CENTRAL (Cochrane Central Register of Controlled Trials), PubMed, and Embase databases from inception to May 2017 were searched using the terms postural AND tachycardia AND syndrome. A total of 135 full-text publications were screened after excluding duplicates (n=681), conference abstracts (n=467), and records that did not relate to POTS therapy (n=876). We included 28 studies with at least 4 patients with POTS in which symptomatic response was reported after more than 4 weeks of therapy. This review was performed according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement. Two investigators independently performed the data extraction and evaluated the quality of evidence. This study comprised 25 case series and 3 small randomized controlled trials that evaluated 755 and 103 patients with POTS, respectively. Interventions directed at increasing intravascular volume, increasing peripheral or splanchnic vascular tone, controlling heart rate, and increasing exercise tolerance demonstrate moderate efficacy (range, 51%-72%). Few data exist on their comparative effectiveness. Significant heterogeneities were seen in terms of patient age, symptom severity, and the measures used to evaluate treatment efficacy. The current evidence base to guide optimal management of patients with POTS is extremely limited. More high-quality collaborative research with standardized reporting of symptom response and treatment tolerability is urgently needed.

Gastrointestinal symptoms are among the most common complaints in patients with postural tachycardia syndrome (POTS). In some cases, they dominate the clinical presentation and cause substantial disabilities, including significant weight loss and malnutrition, that require the use of invasive treatment to support caloric intake. Multiple cross-sectional studies have reported a high prevalence of gastrointestinal symptoms in POTS patients with connective tissue diseases, such as Ehlers-Danlos, hypermobile type, and in patients with evidence of autonomic neuropathy. Previous studies that evaluated gastric motility in these patients reported a wide range of abnormalities, particularly delayed gastric emptying. The pathophysiology of gastrointestinal symptoms in POTS is likely multifactorial and probably depends on the co-morbid conditions. In patients with POTS and Ehlers-Danlos syndromes, structural and functional abnormalities in the gastrointestinal connective tissue may play a significant role, whereas in neuropathic POTS, the gastrointestinal tract motility and gut hormonal secretion may be directly impaired due to localized autonomic denervation. In patients with normal gastrointestinal motility but persistent gastrointestinal symptoms, gastrointestinal functional disorders should be considered. We performed a systematic review of the literature related to POTS and gastrointestinal symptoms have proposed possible mechanisms and discussed diagnosis and treatment approaches for delayed gastric emptying, the most common gastrointestinal abnormality reported in patients with POTS.

Vaccines stimulate a person's immune system to produce an adequate reaction against a specific infectious agent; i.e. the person is protected from that disease without having to get it first. As vaccines are administrated to healthy subjects, they are held to the highest standards of safety. Regarding human papillomavirus (HPV) vaccines, at present three prophylactic vaccines are licensed (bivalent HPV 16/18, quadrivalent HPV 6/11/16/18 and the nonovalent HPV 6/11/16/18/31/33/45/52/58 vaccine). Pre- and post-licensure studies (i.e. not yet for nonovalent HPV vaccine) confirm that HPV vaccines are generally safe and well-tolerated, site injections symptoms are the most common adverse events (AEs) reported, and pain is the most frequently referred local symptom. Serious AEs are rare and not associated with severe sequelae, at least no vaccine-related deaths have occurred. Despite these scientific evidences, it is still difficult to explain to the population the importance of a good vaccination programme. There are many determinants for HPV vaccines hesitancy which represent a barrier that must be overcome in order to increase vaccine coverage, including psychological reactions, religious or cultural aspects, and fear of possible AEs (demyelinating diseases, Complex Regional Pain Syndrome - CRPS, or Postural Orthostatic Tachycardia Syndrome - POTS). A weak communication strategy which frequently suffers due to spread of unverified news by media and websites may lead to the failure of a vaccination programme. Such a situation happened in Japan (2013), due to which a great number of women remain vulnerable to HPV-related cancers. In order to resolve the issues around HPV vaccines acceptance, it is necessary to use good communication strategies. Multicomponent and dialogue-based interventions seem to be the most effective, especially if an adequate language is used, customized according to the vaccination programme target.

We previously reported on a subgroup of postural orthostatic tachycardia syndrome (POTS) patients who may also have features of neurocardiogenic syncope as well. In this subgroup of patients, we found syncope and presyncope were predominant clinical features. To understand the mechanism of syncope in this subgroup, we identified 39 patients who underwent loop recorder insertion. We reviewed charts of 450 patients who had POTS and syncope seen at the University of Toledo Medical Center from 2003 to 2017. Thirty-nine patients had at least four episodes of syncope in the last 6 months and were included for this study. All of these patients had a prior evaluation with a Holter and an event monitor which were inconclusive. Thirty-nine patients, 33 (85%) women, aged 20-46 years, were included in this study. All patients demonstrated prolonged asystole (>6 seconds) or severe bradycardia (heart rate < 30 beats/min) during their syncope on implantable loop recorder (IRL). Fifteen patients demonstrated an asystole of >10 seconds and also had prolonged and convulsive syncope. All patients had abrupt syncope without any warning sign. All patients underwent dual-chamber pacemaker implantation using a closed loop stimulation algorithm. Syncope were completely eliminated in all patients following pacemaker implantation; however, they continued to have orthostatic tachycardia. POTS patients with unusually frequent syncope should be considered for ILR implantation if other monitoring modalities like 48-hour Holter monitor or event recorder are inconclusive. ILR may identify a subgroup of POTS patients who may benefit from pacemaker implantations.

The incidence of Postural Orthostatic Tachycardia Syndrome (POTS) has grown in recent years. Whether this is an emerging epidemic or increasing recognition is unclear. We retrospectively examined 8790 tilt-table tests (HUT) performed between 1997 and 2014 in patients with orthostatic intolerance and/or suspected syncope. Tests were reclassified according to the current diagnostic criteria for POTS. The number of POTS diagnoses in proportion to the number of tilt tests performed per year was calculated. The number of papers published with the term "Postural Orthostatic Tachycardia Syndrome" 1997-2014 was retrieved from PubMed and related to yearly POTS incidence. Eight-hundred-and-seventy-five tests with suspected POTS were thoroughly evaluated. The reclassification of test results yielded 243 POTS diagnoses (age, 27.0 ± 11.8 years). An increase in total number of POTS diagnoses was observed but the proportion of POTS-positive tests per year was relatively constant (≈2-3%) except for the period 2013-2014 (≈7%). The increase in POTS diagnoses was preceded by an increase in number of POTS-related papers in PubMed. The proportion of POTS diagnoses among patients investigated for suspected syncope and/or orthostatic intolerance was relatively constant 1997-2012. The growing number of POTS-related publications in PubMed preceded the steep increase in diagnostic rate of POTS observed after 2012.

Ehlers-Danlos syndromes are a clinically and genetically heterogeneous group of rare inherited connective tissue disorders. Hypermobile Ehlers-Danlos syndrome is one of the common types and not infrequently encountered in pregnancy. While, in the majority of women with hypermobile Ehlers-Danlos syndrome, the pregnancy is uncomplicated, it is important to be aware of the condition in view of potential complications such as recurrent joint dislocations and history of surgical joint stabilization procedures, secondary autonomic pain and postural orthostatic tachycardia syndrome. Increased awareness of the condition and a multi-disciplinary approach to the management of these women in pregnancy result in good outcome for the mother and the baby. We report the clinical characteristics and outcome of pregnancies in eight women with hypermobile Ehlers-Danlos syndrome and present a review of the literature with particular reference to management in a pregnant woman with joint hypermobility syndrome.

Postural orthostatic tachycardia syndrome (POTS) is a disabling condition characterized by orthostatic intolerance with tachycardia in the absence of drop-in blood pressure. A custom-built near-infrared spectroscopy device (NIRS) is applied to monitor the muscle oxygenation, noninvasively in patients undergoing incremental head-up tilt table (HUT). Subjects (6 POTS patients and 6 healthy controls) underwent 30 mins of 70°on a HUT. The results showed a significant difference in deoxyhemoglobin (Hb), change-in-oxygenation (ΔOxy) and blood volume (ΔBV) between patients and healthy controls. However, oxyhemoglobin (HbO) showed a significantly faster rate of change in the healthy controls during the first 10 mins of the tilt and during the recovery. This NIRS muscle oximetry tool provides quantitative measurements of blood oxygenation monitoring in diseases such as POTS.

Postural tachycardia syndrome (POTS) is one of the most common causes of orthostatic intolerance and is being increasingly recognized in clinical practice. Gastrointestinal (GI) symptoms are reported commonly in patients with POTS and pose a considerable management challenge, making it imperative that gastroenterologists be aware of this condition and its GI comorbidities. Although the evidence presented herein does not prove causation, it does support an association between GI symptoms, GI dysmotility, and POTS. At present, the evaluation and treatment of GI symptoms in patients with POTS remains largely empirical. General measures to treat POTS may lead to improvement in both GI and non-GI symptoms. GI symptoms refractory to these measures should prompt further diagnostic evaluation of gastrointestinal dysmotility and appropriate dietary and pharmacologic management. This review focuses its attention on the involvement of the GI tract in POTS including a discussion of GI symptoms and conditions associated with POTS, followed by an analysis of abnormalities in gut physiology described in POTS, and concluding with an overview of management and suggestions for research directions.

Cyclic vomiting syndrome (CVS) in children is characterized by frequent hospitalizations, multiple comorbidities, and poor quality of life. In the absence of robust data, the treatment of CVS remains largely empiric starting with the 2008 NASPGHAN Consensus Statement recommendations of cyproheptadine for children < 5 years of age and amitriptyline for those ≥ 5 years with propranolol serving as the second-line agent. Comprehensive management begins with lifestyle alterations, and extends to medications, supplements, and stress reduction therapies. Standard drug therapy is organized by the four phases of the illness: (1) interictal (preventative medications and mitochondrial supplements), (2) prodromal (abortive agents), (3) vomiting (fluids/energy substrates, antiemetics, analgesics, and sedatives) and (4) recovery (supportive care and nutrition). Because the response to treatment is heterogeneous, clinicians often trial several different preventative strategies including NK1 antagonists, cautious titration of amitriptyline to higher doses, anticonvulsants, Ca-channel blockers, and other TCA antidepressants. When the child remains refractory to treatment, reconsideration of possible missed diagnoses and further mono- or combination therapy and psychotherapy can be guided by accompanying comorbidities (especially anxiety), specific subphenotype, and when available, genotype. For hospital intervention, IV fluids with 10% dextrose, antiemetics, and analgesics can lessen symptoms while effective sedation in some instances can truncate severe episodes. Although management of CVS remains challenging to the clinician, approaches based upon recent literature and accumulated experience with subgroups of patients has led to improved treatment of the refractory and hospitalized patient. What is Known: • Cyclic vomiting syndrome is a complex disorder that remains challenging to manage. • Previous therapy has been guided by the NASPGHAN Consensus Statement of 2008. What is New: • New prophylactic approaches include NK1 antagonists and higher dosages of amitriptyline. • Strategies based upon comorbidities and subphenotype are helpful in refractory patients.

To describe two consecutive cases of Postural Orthostatic Tachycardia Syndrome (POTS)-related chronic abdominal pain control in children after bilateral splanchnic block. Two pediatric patients with chronic abdominal pain related to POTS received celiac and T11 splanchnic plexus block for pain control. While celiac plexus block did not provide any substantial relief of their pain, splanchnic block provided a long-term improvement in pain scores, nausea and vomiting cessation. Bilateral T11 splanchnic block should be considered in patients with POTS-related chronic abdominal pain, nausea and vomiting.

Upright hyperventilation occurs in ~25% of our patients with postural tachycardia syndrome (POTS). Poikilocapnic hyperventilation alone causes tachycardia. Here, we examined changes in respiration and hemodynamics comprising cardiac output (CO), systemic vascular resistance (SVR), and blood pressure (BP) measured during head-up tilt (HUT) in three groups: patients with POTS and hyperventilation (POTS-HV), patients with panic disorder who hyperventilate (Panic), and healthy controls performing voluntary upright hyperpnea (Voluntary-HV). Though all were comparably tachycardic during hyperventilation, POTS-HV manifested hyperpnea, decreased CO, increased SVR, and increased BP during HUT; Panic patients showed both hyperpnea and tachypnea, increased CO, and increased SVR as BP increased during HUT; and Voluntary-HV were hyperpneic by design and had increased CO, decreased SVR, and decreased BP during upright hyperventilation. Mechanisms of hyperventilation and hemodynamic changes differed among POTS-HV, Panic, and Voluntary-HV subjects. We hypothesize that the hyperventilation in POTS is caused by a mechanism involving peripheral chemoreflex sensitization by intermittent ischemic hypoxia. NEW & NOTEWORTHY Hyperventilation is common in postural tachycardia syndrome (POTS) and has distinctive cardiovascular characteristics when compared with hyperventilation in panic disorder or with voluntary hyperventilation. Hyperventilation in POTS is hyperpnea only, distinct from panic in which tachypnea also occurs. Cardiac output is decreased in POTS, whereas peripheral resistance and blood pressure (BP) are increased. This is distinct from voluntary hyperventilation where cardiac output is increased and resistance and BP are decreased and from panic where they are all increased.

We present a precision medical perspective to assist in the definition, diagnosis, and management of Post Treatment Lyme Disease Syndrome (PTLDS)/chronic Lyme disease. PTLDS represents a small subset of patients treated for an erythema migrans (EM) rash with persistent or recurrent symptoms and functional decline. The larger population with chronic Lyme disease is less understood and well defined. Multiple Systemic Infectious Disease Syndrome (MSIDS) is a multifactorial model for treating chronic disease(s), which identifies up to 16 overlapping sources of inflammation and their downstream effects. A patient symptom survey and a retrospective chart review of 200 patients was therefore performed on those patients with chronic Lyme disease/PTLDS to identify those variables on the MSIDS model with the greatest potential effect on regaining health. Results indicate that dapsone combination therapy decreased the severity of eight major Lyme symptoms, and multiple sources of inflammation (other infections, immune dysfunction, autoimmunity, food allergies/sensitivities, leaky gut, mineral deficiencies, environmental toxins with detoxification problems, and sleep disorders) along with downstream effects of inflammation may all affect chronic symptomatology. In part two of our observational study and review paper, we postulate that the use of this model can represent an important and needed paradigm shift in the diagnosis and treatment of chronic disease.

A 24-year-old Japanese man with type 2 diabetes mellitus and diabetic neuropathy was admitted to our ward to evaluate the cause of orthostatic intolerance. During a head-up tilt test, his heart rate increased from 105 to 155 beats/minute within 3 minutes, and chest discomfort began. He was diagnosed with postural orthostatic tachycardia syndrome (POTS), and orthostatic intolerance disappeared after β-blocker treatment. Scintigraphy using I-metaiodobenzylguanidine showed decreased cardiac uptake. Power spectral analysis of heart rate variability for 24 hours in Holter electrocardiography demonstrated decreases in both sympathetic and parasympathetic nervous system activities, with a greater decrease in parasympathetic activity than sympathetic activity. The relative sympathetic hyperactivity in the present patient with diabetic neuropathy seemed to be related to POTS.

Our understanding about Postural Tachycardia Syndrome (POTS) has advanced significantly over the last 25 years. Despite the significant advances that have been made in defining the syndrome and finding some treatments for our patients, there is much work to be done to significantly improve our understanding of the disorder and improve therapeutics. In this article, 5 NEEDS are identified that will be required over the next several years if we want future care to move beyond where we are in the present. These include: (1) a NEED for better administrative data to track POTS diagnoses and the impact of the illness; (2) a NEED to improve physician awareness about POTS, which is a prerequisite for improved access to care; (3) a NEED to better understand the multiple pathophysiologies underlying POTS and the roles of the different medical comorbidities; (4) a NEED for data on effective treatments for POTS; and (5) a NEED for more research funding to study POTS.

Orthostatic intolerance, including postural tachycardia syndrome, is often associated with gastrointestinal symptoms. In the vast majority of the cases, the gastrointestinal symptoms are not secondary to the orthostatic disorder, but rather just a comorbid condition. This concept is critical, since treatment aimed at the orthostatic condition will not improve the gastrointestinal symptoms. Only when the gastrointestinal symptoms develop in the upright position and improve or resolve in the supine position, they may be related to the orthostatic stress. The most common symptoms associated with orthostatic intolerance include nausea, dyspepsia, bloating and constipation. The majority of subjects do not have gastroparesis. The chapter discusses available treatments of these conditions.

IntroductionSevere fatigue and cognitive dysfunction are frequent symptoms in patients with postural orthostatic tachycardia syndrome. They can be debilitating, and often do not resolve despite improvement in haemodynamic symptoms. Our analysis was intended to assess clinical outcomes of medication treatment for these symptoms in a large, single-centre paediatric programme.Materials and MethodsWe performed a retrospective review of patients treated for fatigue and cognitive dysfunction. Patients aged 18 years or younger at the time of initial diagnosis were included. Patients who had a specific medication ordered five or more times for these symptoms were confirmed by chart review for clinical improvement. Percentage of patients with clinical improvement for each medication and overall for all medications, as well as the number of medications per patient required to achieve improvement, were assessed. Data were analysed based on gender as well. t-Test and χ2 analyses were used to assess for differences between means in variables, or specific variables. RESULTS: A total of 708 patients met study criteria, of whom 517 were treated for fatigue or brain fog. Overall efficacy was 68.8%, with individual medication effectiveness ranging from 53.1 (methylphenidate) to 16.5% (atomoxetine). There was no significant difference in efficacy with respect to gender. The median number of medications used per patient was 2, without gender difference. Therapy was limited by side effects or lack of efficacy.DiscussionMedications are effective in the improvement of fatigue and cognitive dysfunction in these patients. However, trials of multiple medications may be needed before achieving clinical improvement.

Postural orthostatic tachycardia syndrome encompasses multiple disabling symptoms that interfere with daily activities. Non-pharmacologic approaches can be insufficient and can require adjunctive medications to manage symptoms. Minimal data exist in the literature on medication outcomes in these patients. We reviewed our database for medication management outcomes.Materials and MethodsPatients aged 18 years and younger at initial diagnosis met the inclusion criteria. All prescribed patient medications were extracted from the electronic health record, excluding medications for unrelated symptoms or comorbid diseases. Medications were grouped by symptom class consistent with our programme utilisation protocol. Within symptom classification, therapy was deemed successful when a specific dose was prescribed at least five consecutive times without changes; this was confirmed by chart review. Individual medications and overall percentage of successful therapies within symptom classifications were assessed, with further analysis by gender. t-Test, χ2, and Mann-Whitney U-test were used to assess for differences in specific variables, as appropriate. A total of 708 patients met the study criteria. The percentage of patients with effective therapy by symptom includes light-headedness (52.2%), headache (48.2%), nausea (39.1%), dysmotility (43.4%), pain (53.4%), and insomnia (42.8%). Insomnia therapy was better for females; all other therapies showed no gender difference. The median number of therapies prescribed per patient per symptom was 2 for light-headedness, headache, and insomnia, and 1 for nausea, dysmotility, and pain.DiscussionSymptoms associated with this disorder can be effectively managed with various medications. Further randomised studies are needed to better ascertain true efficacy compared with placebo.