Pediatric-Onset Postural Orthostatic Tachycardia Syndrome in a Single Tertiary Care Center

2020 ◽  
Vol 35 (8) ◽  
pp. 526-535 ◽  
Author(s):  
Anthony Staples ◽  
Nicolas R. Thompson ◽  
Manikum Moodley
Keyword(s):  
Heart Rate ◽  
Orthostatic Intolerance ◽  
Care Center ◽  
Tertiary Care ◽  
Categorical Variables ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Presenting Symptoms ◽  
Unexplained Pain ◽  
Patient Reported ◽  
Pediatric Onset

Aim: We characterize the pediatric postural orthostatic tachycardia syndrome (POTS) population seen at a single tertiary care referral center. Method: Retrospective chart review of all pediatric POTS patients seen in our center between 2015 and 2017. Pediatric POTS was defined as chronic, at least 3 months, symptoms of orthostatic intolerance associated with excessive orthostatic tachycardia as determined by tilt table testing with orthostatic heart rate increment of ≥40 bpm within 5 minutes of head-up tilt or absolute orthostatic heart rate ≥130 bpm for patients 13 years old and younger and ≥120 bpm for those 14 years and older. We looked at demographics, presenting symptoms, comorbidities, examination findings, investigation findings, treatment, and patient reported outcomes. Outcome measures were separated by patient report and group comparisons were made using 2-sample t tests or Mann-Whitney U tests for continuous variables and Fisher exact tests for categorical variables. Results: One hundred thirty-four patients with pediatric onset POTS were identified. The mean age was 15 years. Seventy-nine percent of patients were female and 90% were white. The most common presenting symptoms included dizziness/lightheadedness (88%), syncope (54%), and palpitations (40%). Many patients had significant comorbidities attributable to numerous bodily systems, most commonly headache syndromes (migraine 43%, nonspecific headache 22%, chronic daily headache 14%, and new daily persistent headache 5%) and chronic fatigue (60%). Low vitamin D and insufficient iron stores were commonly seen. The majority of patients improved or had resolution of symptoms following treatment (70%). When separated by outcome, statistically significant differences were found for glucose (patients whose symptoms resolved had higher median glucose), palpitations (patients whose symptoms resolved were less likely to have palpitations), constipation (patients whose symptoms were stable/worsened were more likely to have constipation), and unexplained pain (patients whose symptoms were stable/worsened were more likely to have unexplained pain). Conclusions: Pediatric POTS is a chronic condition with a fairly good prognosis following appropriate treatment. It is associated with numerous comorbidities that necessitate multidisciplinary expert care.

scholarly journals Circulating levels of growth hormone in postural orthostatic tachycardia syndrome

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Madeleine Johansson ◽  
Fabrizio Ricci ◽  
Janin Schulte ◽  
Margaretha Persson ◽  
Olle Melander ◽  
...  
Keyword(s):  
Heart Rate ◽  
Growth Hormone ◽  
Orthostatic Intolerance ◽  
High Sensitivity ◽  
Symptom Assessment ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Daily Life Activities ◽  
Median Plasma ◽  
Symptom Assessment Scale ◽  
Plasma Gh

AbstractPostural orthostatic tachycardia syndrome (POTS) is a cardiovascular autonomic disorder with poorly understood etiology and underlying pathophysiology. Since cardiovascular morbidity has been linked to growth hormone (GH), we studied GH levels in patients with POTS. We conducted an age-sex-matched case–control study in patients with POTS (age 31 ± 9 years; n = 42) and healthy controls (32 ± 9 years; n = 46). Plasma GH levels were measured using high-sensitivity chemiluminescence sandwich immunoassay. The burden of orthostatic intolerance symptoms was assessed by the Orthostatic Hypotension Questionnaire (OHQ), consisting of a symptom assessment scale (OHSA) and a daily activity scale (OHDAS). POTS patients had significantly higher composite OHQ score than controls, more symptoms and less activity. Supine heart rate and diastolic blood pressure (BP), but not systolic BP, were significantly higher in POTS. Median plasma GH levels were significantly lower in POTS (0.53 ng/mL) than controls (2.33 ng/mL, p = 0.04). GH levels were inversely related to OHDAS in POTS and supine systolic BP in POTS and controls, but not heart rate neither group. POTS is associated with lower GH levels. Impairment of daily life activities is inversely related with GH in POTS. A higher supine diastolic BP is inversely associated with GH levels in POTS and healthy individuals.

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.

scholarly journals Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center

2021 ◽  
Vol 0 ◽  
pp. 1-9
Author(s):  
Rattanavalai Nitiyarom ◽  
Sirirat Charuvanij ◽  
Surachai Likasitwattanakul ◽  
Chaiwat Thanoophunchai ◽  
Wanee Wisuthsarewong
Keyword(s):  
Juvenile Dermatomyositis ◽  
Care Center ◽  
Tertiary Care ◽  
Inflammatory Myopathy ◽  
Clinical Manifestations ◽  
Multicenter Studies ◽  
Extraskeletal Osteosarcoma ◽  
Calcinosis Cutis ◽  
Tertiary Referral Center ◽  
Presenting Symptoms

Background: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. Aim: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. Method: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. Results: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron’s rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. Limitations: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. Conclusion: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.

scholarly journals Postural orthostatic tachycardia syndrome in primary care: diagnosis, treatment and a case of African-American man presenting with POTS

2019 ◽  
Vol 12 (9) ◽  
pp. e229824
Author(s):  
Andrew T Del Pozzi ◽  
Michael Enechukwu ◽  
Svetlana Blitshteyn
Keyword(s):  
Heart Rate ◽  
Differential Diagnosis ◽  
African American ◽  
Clinical Features ◽  
Orthostatic Intolerance ◽  
Treatment Options ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Tilt Table Test ◽  
Tilt Table ◽  
Caucasian Women

Postural orthostatic tachycardia syndrome (POTS) is a heterogeneous autonomic disorder characterised by orthostatic intolerance and a rise in heart rate by at least 30 bpm or an absolute heart rate value of at least 120 bpm within 10 min of standing or during a tilt table test. Overwhelmingly, POTS affects young Caucasian women, which can lead physicians to miss the diagnosis in men or non-white patients. We describe a case of 29-year-old African-American man who developed lightheadedness, generalised weakness, tachycardia and palpitations and was subsequently diagnosed with POTS. We review its clinical features, differential diagnosis, pathophysiology and treatment options. We also emphasise that POTS should be considered as a differential diagnosis in any patient presenting with typical clinical features, who may not be in the usual demographics of the disorder.

scholarly journals Acute Kidney Injury and Atypical Features during Pediatric Poststreptococcal Glomerulonephritis

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Rose M. Ayoob ◽  
Andrew L. Schwaderer
Keyword(s):  
Acute Kidney Injury ◽  
Care Center ◽  
Tertiary Care ◽  
Elevated Serum ◽  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Small Subset ◽  
Acute Glomerulonephritis ◽  
Poststreptococcal Glomerulonephritis ◽  
Presenting Symptoms

The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN) usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males), mean age of 8 years, were analyzed. Ninety-four percent had elevated serum BUN levels and decreased GFR. Four of the hospitalized patients had complex presentations that included AKI along with positive ANA or ANCAs. Three patients required renal replacement therapy and two were thrombocytopenic. PSGN usually does not occur as a severe nephritis. Over the 12-month study period, 17 cases associated with low serum albumin in 53%, acute kidney injury in 94%, and thrombocytopenia in 18% were treated. The presentation of PSGN may be severe and in a small subset have associations similar to SLE nephritis findings including AKI, positive ANA, and hematological anomalies.

Validation of a Breastfeeding History Questionnaire for the Risk of In-Hospital Formula Supplementation Among Multiparous Women

2019 ◽  
Vol 35 (4) ◽  
pp. 665-671
Author(s):  
Whitney R. Bender ◽  
Nathanael C. Koelper ◽  
Mary D. Sammel ◽  
Celeste Durnwald
Keyword(s):  
Hospital Stay ◽  
Care Center ◽  
Tertiary Care ◽  
Categorical Variables ◽  
Chi Square ◽  
Multiparous Women ◽  
Chi Square Test ◽  
Student’S T ◽  
Relationship Of ◽  
The Relationship

Background: A woman’s prior breastfeeding history may influence future decisions regarding infant feeding. Few quantitative tools utilizing this information have been demonstrated to predict breastfeeding success. Research aim: To evaluate the efficacy of a prenatal breastfeeding history (BAP) questionnaire administered in prenatal care to predict in-hospital formula supplementation among multiparous women. Methods: This is a prospective observational study of multiparous women with singleton pregnancies who presented to a Baby-Friendly urban tertiary care center for 1st prenatal visit at < 20 weeks’ gestation. The BAP tool generates a numerical score, with higher score (≥ 2) indicating prior successful breastfeeding experiences. The primary outcome was occurrence of non-medically indicated formula supplementation during the postpartum hospital stay. Student’s t test and Pearson’s chi-square test were used to compare continuous and categorical variables. A multivariable logistic regression was performed to assess the relationship of BAP score to formula supplementation. Of 587 women screened, 433 (73.8%) mother–infant dyads were analyzed. Results: Rates of formula supplementation in women with BAP scores ≤ 1 were 67% (156/234) compared with 37% (73/199) in women with higher scores ( p < 0.0001). After controlling for race/ethnicity, insurance, and obesity, women with BAP scores of ≤ 1 were 2.6 times more likely to supplement formula than women with higher scores (a OR 2.62, 95% CI [1.70, 4.04], p < .0001). Conclusion: In this prospective validation study, women with negative prior breastfeeding experiences, as evidenced by a lower BAP score, were more likely to supplement formula during the postpartum hospital stay.

scholarly journals Cardiac Health Awareness Among Women Presenting for Routine Health Checkup at Tertiary Care Center: Time Trends Over 15 Years

2019 ◽  
Vol 04 (02) ◽  
pp. 079-084
Author(s):  
Shibba Takkar Chhabra ◽  
Gurleen Kaur ◽  
Samir Kapoor ◽  
Gagandeep Nagi ◽  
Devanshi Kajal ◽  
...  
Keyword(s):  
Care Center ◽  
Tertiary Care ◽  
Treatment Modalities ◽  
Age Group ◽  
Tertiary Care Center ◽  
Health Awareness ◽  
Health Checkup ◽  
Cardiac Health ◽  
Presenting Symptoms ◽  
The Past

Abstract Background Women have been thought to be protected against the perils of cardiovascular disease (CVD) till late in their lives. But the literature suggests quite the opposite with CVD being a major cause of death even in young women. In contrast, the lack of awareness among women is disheartening and needs to be addressed radically. Methods The study was designed and conducted as retrospective cohort at a tertiary care center. Data was collected from patients presenting for routine cardiac health checkup over the past 15 years. The parameters observed included age at presenting, symptoms and/or signs, plus area of residence or domicile. Results A total of 32,831 patients presented for routine cardiac health checkup, of which 9,211 (28.1%) patients were women and 23,620 (71.9%) were men. On 5 yearly cumulative assessment, the mean attendance of women was 28.1 ± 2.5% as compared with 71.9 ± 2.6% men. Trend observed over the past 15 years revealed little change in the number of women versus men presenting for cardiac health checkup annually. Statistical significance was seen at p < 0.01. Among the women presenting for the checkup, it was observed that most women were in the postmenopausal age group (42.8%), followed by perimenopausal age group (34.6%), and least in premenopausal age group (22.5%) during the timeline of the study. A similar trend was observed in the male attendance; the input of males being higher at all instances. Noteworthy were trends of urban women (69.6%) presenting for health checkup more often as compared with 30.4% visiting from rural residence. Conclusions Coronary artery disease (CAD) is not uncommon in female gender. It is accompanied by varying symptom presentation with high mortality. It is seen that cardiac health awareness is significantly lacking among women as compared with men. There are almost static trends observed over the past 15 years, especially in premenopausal age group and rural domicile. Corrective actions inclining toward campaigns and communication to distribute information on cardiac disease prevention and treatment modalities among women are needed to curb CAD. This may promote early detection of CAD leading to early interventions to promote a healthy heart among women. Recommendations and necessary actions steps for a woman oriented cardiac program are the need of the hour.

Laryngeal Web in the Pediatric Population: Evaluation and Management

2019 ◽  
Vol 162 (2) ◽  
pp. 234-240 ◽  
Author(s):  
Claire M. Lawlor ◽  
Natasha D. Dombrowski ◽  
Roger C. Nuss ◽  
Reza Rahbar ◽  
Sukgi S. Choi
Keyword(s):  
Pediatric Population ◽  
Care Center ◽  
Tertiary Care ◽  
Case Series ◽  
22Q11.2 Deletion Syndrome ◽  
Deletion Syndrome ◽  
Retrospective Case ◽  
Presenting Symptoms ◽  
Subglottic Extension ◽  
Type 3

Objective To discuss the presentation, evaluation, and management of pediatric laryngeal web. Study Design Retrospective case series. Setting Single tertiary care center. Subjects All patients with laryngeal web at Boston Children’s Hospital in the past 22 years. Methods No exclusion criteria. Charts mined for age at presentation, presenting symptoms, degree/location of web, associated syndromes, number/type of surgical procedures, and postoperative outcomes. Results Thirty-seven patients were included (13 male, 24 female). Average age at diagnosis was 3.7 years (0-19.5 years). Mean follow-up was 4.4 years (range, 0-16.4 years). There were 26 congenital webs (70.2%) and 11 acquired webs (29.8%). Presenting symptoms were vocal (29 patients, 78.4%) and respiratory (22 patients, 60%). Underlying syndromes or synchronous airway lesions included the following: premature (n = 5), congenital heart disease (n = 18), subglottic stenosis (n = 5), 22q11.2 deletion syndrome (n = 10), and recurrent respiratory papillomatosis (n = 4). There were 20 type 1 webs, 6 type 2 webs, 8 type 3 webs, and 3 type 4 webs; 10 had subglottic extension of the laryngeal web. Twelve patients were managed conservatively with observation. Eighty-four interventions were performed: 18 open and 66 endoscopic (sharp division, 32; dilation, 33; mitomycin C, 14; laser, 5; keel, 6; triamcinolone injection, 8; stent, 15; removal of granulation tissue, 5). Tracheotomy was required in 11 patients, and 5 patients were decannulated. Voice improved in 12 patients, with respiratory symptoms in 12 patients. Web recurred in 17 patients. One patient died due to airway complications. Conclusions Pediatric laryngeal web is an uncommon but challenging lesion. Patients need to be evaluated for comorbid syndromes and synchronous airway lesions. Management includes open and endoscopic procedures. Procedures should be tailored to the child’s presentation.

Presenting Symptoms and Diagnostic Work-up in Patients With Gastroparesis at a Tertiary-care Center

2015 ◽  
Vol 110 ◽  
pp. S1023-S1024
Author(s):  
Anjana Sathyamurthy ◽  
Mohamad H. Yousef ◽  
Harleen K. Chela ◽  
Bhupinder Romana ◽  
Zainab Arif ◽  
...  
Keyword(s):  
Care Center ◽  
Tertiary Care ◽  
Tertiary Care Center ◽  
Presenting Symptoms ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up
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