Postural orthostatic tachycardia syndrome(POTS) is a relatively rare syndrome recognised since 1940. It is a heterogenous condition with orthostatic intolerance due to dysautonomia and is characterised by rise in hear...Postural orthostatic tachycardia syndrome(POTS) is a relatively rare syndrome recognised since 1940. It is a heterogenous condition with orthostatic intolerance due to dysautonomia and is characterised by rise in heart rate above 30 bpm from base line or to more than 120 bpm within 5-10 min of standing with or without change in blood pressure which returns to base line on resuming supine position. This condition present with various disabling symptoms such as light headedness, near syncope, fatigue, nausea, vomiting, tremor, palpitations and mental clouding, etc. However there are no identifiable signs on clinical examination and patients are often diagnosed to have anxiety disorder. The condition predominantly affects young female between the ages of 15-50 but is rarely described in older people. We describe an older patient who developed POTS which recovered over 12 mo. Recognising this condition is important as there are treatment options available to alleviate the disabling symptoms.展开更多
Background Postural orthostatic tachycardia syndrome （POTS） is a common clinical problem in children and adolescents. The previous diagnostic approach to POTS of children and adolescents is based on a series of test...Background Postural orthostatic tachycardia syndrome （POTS） is a common clinical problem in children and adolescents. The previous diagnostic approach to POTS of children and adolescents is based on a series of tests to exclude all other causes, which is time and medical resource consuming. Recently, a new diagnostic approach has been developed. The present study was designed to statistically analyze the results of clinical investigation items and the cost for the diagnosis of POTS in children patients, and evaluate cost changes in the diagnosis of POTS. Methods A total of 315 children patients were divided into two groups according to diagnosis period, including group I diagnosed in 2002-2006 （100 cases） and group II in 2007-2010 （215 cases） and the diagnostic item-based distribution of the cost was analyzed. The diagnostic costs were compared between two groups using SPSS17.0. Results The per-capita cost of diagnosis in group I was （621.95±2.1.10） Yuan, costs of diagnostic tests （head-up tilt test standing test, etc） accounted for 8.68% and the exclusive tests for 91.32%. The per-capita cost of diagnosis in group II was （542.69±2.3.14） Yuan, diagnostic tests accounted for 10.50% and exclusive tests for 89.50%. Comparison of the total cost of diagnostic tests between the two groups showed significant differences （P〈0.05）. Conclusion The cost of POTS diagnosis has been declined in recent years, but the cost of exclusive diagnosis is still its major part.展开更多
Background The abnormal blood volume regulation is one of the most important pathogenesis in postural tachycardia syndrome in children.This study was designed to investigate the plasma atrial natriuretic peptide and a...Background The abnormal blood volume regulation is one of the most important pathogenesis in postural tachycardia syndrome in children.This study was designed to investigate the plasma atrial natriuretic peptide and antidiuretic hormone levels in postural tachycardia syndrome children,and their associations with the changes in heart rate and blood pressure in head-up test.Methods Twenty-one postural tachycardia syndrome patients （（12±2） years） and 26 healthy children （（12±1） years） were included.According to blood pressure changes in head-up test,the postural tachycardia syndrome patients were divided into two subgroups：postural tachycardia syndrome with orthostatic hypertension and postural tachycardia syndrome without orthostatic hypertension.The plasma atrial natriuretic peptide and antidiuretic hormone levels were measured using enzyme-linked immunosorbent assay.Results The plasma atrial natriuretic peptide level in postural tachycardia syndrome patients was higher than the control （P=0.004）,whereas the difference in plasma antidiuretic hormone level between postural tachycardia syndrome and controls was not significant （P=0.222）.The plasma antidiuretic hormone level of patients suffering from postural tachycardia syndrome with orthostatic hypertension was much higher than that of children having postural tachycardia syndrome without orthostatic hypertension （P ＜0.05）.In postural tachycardia syndrome patients,the updght max heart rate was positively correlated with the plasma atrial natriuretic peptide level （r=0.490,P＜0.05） and the upright systolic blood pressure was positively correlated with the plasma antidiuretic hormone levels （r=0.472,P ＜0.05）.Conclusions There was a disturbance of plasma atrial natriuretic peptide and antidiuretic hormone in postural tachycardia syndrome children.展开更多
Background: There is an increasing recognition of patients with Ehlers Danlos Syndromes. The laxity of the ligaments and the weakness of the connective tissue has resulted in increasing number of patients requiring su...Background: There is an increasing recognition of patients with Ehlers Danlos Syndromes. The laxity of the ligaments and the weakness of the connective tissue has resulted in increasing number of patients requiring surgical intervention. Ehlers Danlos Syndromes are not about hypermobile joints only, they are associated with multiple co-existing conditions such as Chiari malformation, Tethered Cord Syndrome, spinal instability, abdominal pain, Dysautonomia and Mast Cell Activation Syndrome. The combined incidence of Ehlers Danlos Syndromes is 1 in 5000 people. Most experts believe that the actual incidence is much higher. Many of these cases are under-diagnosed. Nevertheless, patients with Ehlers Danlos Syndromes, diagnosed or undiagnosed often require surgical intervention. This review article has been written to shed light on the need for special consideration during anesthesia. Objectives: Our objective was to conduct a review of anesthetic considerations in patients with Ehlers Danlos Syndromes. Study Design: We used a narrative review design. Methods: This review was done using searches of PubMed, MEDLINE/OVID, SCOPUS, and manual searches of the bibliographies of known primary and review articles from inception to 2019. Other data sources included hand searches of publications driven by manuscript authors. Search terms included concepts of “Ehlers Danlos Syndrome”, “EDS”, “pain”, “anesthesia”, “surgery” and combination of terms. Search method was not restricted to any one language. Results: Articles were screened by title, abstract, and full article review. They were then analyzed by specific clinical indications and appropriate data was presented based on critical analysis of those articles. Limitations: More studies about the effect of anesthetic techniques and Ehlers Danlos Syndromes are required. Conclusions: Patients with Ehlers Danlos Syndromes may present with an array of coexisting medical conditions such as Dysautonomia, Mast Cell Activation Syndrome, Chiari Malformation, Tethered Cord Syndrome, Craniocervical instability, Gastroparesis, altered sensitivity to local anesthetics. Anesthetic techniques need to be modified according to the presentation. Preoperatively, a note should be made of the joints that sublux most often, craniocervical instability. They are prone to temporomandibular joint subluxation, obstructive sleep apnea and tracheomalacia, spontaneous pneumothorax, POTS, MCAS, insensitivity to local anesthetics and coagulation disorders. Intraoperative considerations should include fiberoptic intubation, IV fluid loading for POTS, avoid drugs that release histamine, unpredictable response to opioids, precautions for gastroparesis. Patient should be positioned to avoid joint subluxation and over stretching nerves. Postoperative considerations include maintaining hemodynamic stability, avoid exacerbation of symptoms of MCAS, high suspicion of occult bleeding, high risk of post dural puncture headache in patients undergoing spinal procedure or neuroaxial block.展开更多
Background The incidence of postural tachycardia syndrome （POTS） has been increasing in children and adolescents,while clinical characteristics of POTS in the pediatric population are not fully understood.Methods An...Background The incidence of postural tachycardia syndrome （POTS） has been increasing in children and adolescents,while clinical characteristics of POTS in the pediatric population are not fully understood.Methods An observational study was performed in 150 pediatric patients aged between 5 and 18 years who underwent head-up tilt test （HUTT） with the diagnosis of POTS at Peking University First Hospital from March 2008 to August 2013.Demographic data,clinical presentation,autonomic parameters,laboratory findings,and treatments were recorded.Results POTS in children commonly occurred in the age of 7-14 years.Dizziness （84.00％） was the most common symptom,followed by weakness （72.00％） and orthostatic syncope （62.67％）.Positive family history of orthostatic intolerance （Ol） was found in 24.64％ of children with POTS.And 33.09％ of them had preceding infection history as precipitating events.Ten percent of them suffered from orthostatic hypertension.Hyperadrenergic status was documented in 51.28％ of 39 patients who were tested for the standing norepinephrine levels.More than half of POTS patients,with 24-hour urinary sodium level ＜124 mmol/24 hours,were suitable for treatment of salt supplementation.At least 25.74％ of POTS patients were of positive acetylcholine receptor （AChR） antibody.Low iron storage in children with POTS was relatively rare.Most patients responded well to treatments,43.51％ of patients recovered,while 7.63％ of them had relapse after symptoms disappeared.Conclusions POTS is a relatively common condition with complex pathophysiology and heterogeneous clinical manifestation.A comprehensive therapeutic regimen is recommended for the treatment.展开更多
Background Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance, and its incidence in children is approximately 6.8% (1)The pathogenesis of POTS is complex with multiple, overlapping, ...Background Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance, and its incidence in children is approximately 6.8% (1)The pathogenesis of POTS is complex with multiple, overlapping, interacting pathophysi-ological mechanisms. Although the specific pathogenic mechanism has remained perplexing, with the discovery of various gasotransmitters and biological peptides, the vascular dysfunction has aroused overwhelming attention. Data sources On the basis of searching in a wide range of recent original literatures, we reviewed the pathogenesis of vas-cular dysfunction in children with POTS. Results The flow-mediated vasodilation of POTS patients was greater than that of healthy controls, and the vasodilator factors were increased in patients with POTS under basal condition or under a standing position, while the vasoconstriction factors were reduced. Conclusions Vascular dysfunction, as one of pathogenesis in pediatric POTS patients, affects the occurrence and develop-ment of diseases through a variety of factors.展开更多
文摘Postural orthostatic tachycardia syndrome(POTS) is a relatively rare syndrome recognised since 1940. It is a heterogenous condition with orthostatic intolerance due to dysautonomia and is characterised by rise in heart rate above 30 bpm from base line or to more than 120 bpm within 5-10 min of standing with or without change in blood pressure which returns to base line on resuming supine position. This condition present with various disabling symptoms such as light headedness, near syncope, fatigue, nausea, vomiting, tremor, palpitations and mental clouding, etc. However there are no identifiable signs on clinical examination and patients are often diagnosed to have anxiety disorder. The condition predominantly affects young female between the ages of 15-50 but is rarely described in older people. We describe an older patient who developed POTS which recovered over 12 mo. Recognising this condition is important as there are treatment options available to alleviate the disabling symptoms.
文摘Background Postural orthostatic tachycardia syndrome （POTS） is a common clinical problem in children and adolescents. The previous diagnostic approach to POTS of children and adolescents is based on a series of tests to exclude all other causes, which is time and medical resource consuming. Recently, a new diagnostic approach has been developed. The present study was designed to statistically analyze the results of clinical investigation items and the cost for the diagnosis of POTS in children patients, and evaluate cost changes in the diagnosis of POTS. Methods A total of 315 children patients were divided into two groups according to diagnosis period, including group I diagnosed in 2002-2006 （100 cases） and group II in 2007-2010 （215 cases） and the diagnostic item-based distribution of the cost was analyzed. The diagnostic costs were compared between two groups using SPSS17.0. Results The per-capita cost of diagnosis in group I was （621.95±2.1.10） Yuan, costs of diagnostic tests （head-up tilt test standing test, etc） accounted for 8.68% and the exclusive tests for 91.32%. The per-capita cost of diagnosis in group II was （542.69±2.3.14） Yuan, diagnostic tests accounted for 10.50% and exclusive tests for 89.50%. Comparison of the total cost of diagnostic tests between the two groups showed significant differences （P〈0.05）. Conclusion The cost of POTS diagnosis has been declined in recent years, but the cost of exclusive diagnosis is still its major part.
基金This study was funded by the grants from the National Twelfth Five- Year Plan for Science ＆ Technology Support （No. 2012BAI03B03）, the Major Basic Research Project of China （No. 2012CB517806 and No. 201 ICB503904） and the National Natural Science Foundation of China （No. 81121061）.
文摘Background The abnormal blood volume regulation is one of the most important pathogenesis in postural tachycardia syndrome in children.This study was designed to investigate the plasma atrial natriuretic peptide and antidiuretic hormone levels in postural tachycardia syndrome children,and their associations with the changes in heart rate and blood pressure in head-up test.Methods Twenty-one postural tachycardia syndrome patients （（12±2） years） and 26 healthy children （（12±1） years） were included.According to blood pressure changes in head-up test,the postural tachycardia syndrome patients were divided into two subgroups：postural tachycardia syndrome with orthostatic hypertension and postural tachycardia syndrome without orthostatic hypertension.The plasma atrial natriuretic peptide and antidiuretic hormone levels were measured using enzyme-linked immunosorbent assay.Results The plasma atrial natriuretic peptide level in postural tachycardia syndrome patients was higher than the control （P=0.004）,whereas the difference in plasma antidiuretic hormone level between postural tachycardia syndrome and controls was not significant （P=0.222）.The plasma antidiuretic hormone level of patients suffering from postural tachycardia syndrome with orthostatic hypertension was much higher than that of children having postural tachycardia syndrome without orthostatic hypertension （P ＜0.05）.In postural tachycardia syndrome patients,the updght max heart rate was positively correlated with the plasma atrial natriuretic peptide level （r=0.490,P＜0.05） and the upright systolic blood pressure was positively correlated with the plasma antidiuretic hormone levels （r=0.472,P ＜0.05）.Conclusions There was a disturbance of plasma atrial natriuretic peptide and antidiuretic hormone in postural tachycardia syndrome children.
文摘Background: There is an increasing recognition of patients with Ehlers Danlos Syndromes. The laxity of the ligaments and the weakness of the connective tissue has resulted in increasing number of patients requiring surgical intervention. Ehlers Danlos Syndromes are not about hypermobile joints only, they are associated with multiple co-existing conditions such as Chiari malformation, Tethered Cord Syndrome, spinal instability, abdominal pain, Dysautonomia and Mast Cell Activation Syndrome. The combined incidence of Ehlers Danlos Syndromes is 1 in 5000 people. Most experts believe that the actual incidence is much higher. Many of these cases are under-diagnosed. Nevertheless, patients with Ehlers Danlos Syndromes, diagnosed or undiagnosed often require surgical intervention. This review article has been written to shed light on the need for special consideration during anesthesia. Objectives: Our objective was to conduct a review of anesthetic considerations in patients with Ehlers Danlos Syndromes. Study Design: We used a narrative review design. Methods: This review was done using searches of PubMed, MEDLINE/OVID, SCOPUS, and manual searches of the bibliographies of known primary and review articles from inception to 2019. Other data sources included hand searches of publications driven by manuscript authors. Search terms included concepts of “Ehlers Danlos Syndrome”, “EDS”, “pain”, “anesthesia”, “surgery” and combination of terms. Search method was not restricted to any one language. Results: Articles were screened by title, abstract, and full article review. They were then analyzed by specific clinical indications and appropriate data was presented based on critical analysis of those articles. Limitations: More studies about the effect of anesthetic techniques and Ehlers Danlos Syndromes are required. Conclusions: Patients with Ehlers Danlos Syndromes may present with an array of coexisting medical conditions such as Dysautonomia, Mast Cell Activation Syndrome, Chiari Malformation, Tethered Cord Syndrome, Craniocervical instability, Gastroparesis, altered sensitivity to local anesthetics. Anesthetic techniques need to be modified according to the presentation. Preoperatively, a note should be made of the joints that sublux most often, craniocervical instability. They are prone to temporomandibular joint subluxation, obstructive sleep apnea and tracheomalacia, spontaneous pneumothorax, POTS, MCAS, insensitivity to local anesthetics and coagulation disorders. Intraoperative considerations should include fiberoptic intubation, IV fluid loading for POTS, avoid drugs that release histamine, unpredictable response to opioids, precautions for gastroparesis. Patient should be positioned to avoid joint subluxation and over stretching nerves. Postoperative considerations include maintaining hemodynamic stability, avoid exacerbation of symptoms of MCAS, high suspicion of occult bleeding, high risk of post dural puncture headache in patients undergoing spinal procedure or neuroaxial block.
文摘Background The incidence of postural tachycardia syndrome （POTS） has been increasing in children and adolescents,while clinical characteristics of POTS in the pediatric population are not fully understood.Methods An observational study was performed in 150 pediatric patients aged between 5 and 18 years who underwent head-up tilt test （HUTT） with the diagnosis of POTS at Peking University First Hospital from March 2008 to August 2013.Demographic data,clinical presentation,autonomic parameters,laboratory findings,and treatments were recorded.Results POTS in children commonly occurred in the age of 7-14 years.Dizziness （84.00％） was the most common symptom,followed by weakness （72.00％） and orthostatic syncope （62.67％）.Positive family history of orthostatic intolerance （Ol） was found in 24.64％ of children with POTS.And 33.09％ of them had preceding infection history as precipitating events.Ten percent of them suffered from orthostatic hypertension.Hyperadrenergic status was documented in 51.28％ of 39 patients who were tested for the standing norepinephrine levels.More than half of POTS patients,with 24-hour urinary sodium level ＜124 mmol/24 hours,were suitable for treatment of salt supplementation.At least 25.74％ of POTS patients were of positive acetylcholine receptor （AChR） antibody.Low iron storage in children with POTS was relatively rare.Most patients responded well to treatments,43.51％ of patients recovered,while 7.63％ of them had relapse after symptoms disappeared.Conclusions POTS is a relatively common condition with complex pathophysiology and heterogeneous clinical manifestation.A comprehensive therapeutic regimen is recommended for the treatment.
文摘Background Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance, and its incidence in children is approximately 6.8% (1)The pathogenesis of POTS is complex with multiple, overlapping, interacting pathophysi-ological mechanisms. Although the specific pathogenic mechanism has remained perplexing, with the discovery of various gasotransmitters and biological peptides, the vascular dysfunction has aroused overwhelming attention. Data sources On the basis of searching in a wide range of recent original literatures, we reviewed the pathogenesis of vas-cular dysfunction in children with POTS. Results The flow-mediated vasodilation of POTS patients was greater than that of healthy controls, and the vasodilator factors were increased in patients with POTS under basal condition or under a standing position, while the vasoconstriction factors were reduced. Conclusions Vascular dysfunction, as one of pathogenesis in pediatric POTS patients, affects the occurrence and develop-ment of diseases through a variety of factors.
