-
Yan-zhi Zhang,
Hui Xiong,
Xiao-zhu Wang,
Shuo Wang,
Jing Luo,
Jing-min Wang,
Yu-wu Jiang,
Xing-zhi Chang,
Hong Pan,
Jian-guang Qi, Wan-zhen Li,
Yun Yuan,
Xi-ru Wu
[show abstract]
[hide abstract]
ABSTRACT: To improve the diagnosis and management of Duchenne/Becker muscular dystrophy(DMD/BMD).
Clinical features of 90 cases of DMD/BMD were collected. Genomic DNA was extracted using standard procedures from the peripheral blood leukocytes, and multiplex ligation-dependent probe amplification (MLPA) was applied to detect DMD gene to identify genetic mutation. For those patients whose deletion/duplication mutation was not identified, FKRP gene mutation analysis was performed using PCR-DNA direct sequence. All the cases were followed up.
Among the 90 cases of clinically diagnosed DMD/BMD, exons deletion of DMD was detected in 58 cases (64.44%), and exons duplication in 9 (10.00%). Among the 34 mothers with an affected boy but without previous genetic conformation, 17 were confirmed to be carriers with gene deletion/duplication. None of the 23 cases, without detected DMD gene deletion/duplication, carried FKRP gene mutation. Fourteen children were given short-term intermittent prednisone therapy (0.75 mg/kg daily during the first 10 days of each month). The course was not long enough and the sample size was too small to conclude any benefits or side effects. Prenatal diagnosis was provided for one mother in her next pregnancy detecting a female carrier fetus.
DMD gene deletions mainly occurs between exons 45 and 54, while duplications mostly at 5'-terminus. Identification of the characteristics and types of gene mutation may facilitate the recognition and prognosis prediction of DMD/BMD. MLPA is a non-complex and quick diagnostic tool for DMD/BMD and its carriers, and also helpful in genetic counseling.
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences 12/2010; 42(6):661-6.
-
[show abstract]
[hide abstract]
ABSTRACT: The study was designed to explore the value of the New York University Pediatric Heart Failure Index (NYU PHFI) for diagnosing and grading chronic heart failure in children.
Totally 105 children with chronic heart failure or structural heart disease but without signs and symptoms of heart failure were enrolled. They were diagnosed using modified Ross score, NYU PHFI and NT-proBNP, respectively. According to modified Ross score as the referent criteria, the diagnostic value of NYU PHFI in quantifying chronic heart failure severity in children was studied. Furthermore, according to the grading of heart failure using modified Ross score, the area under the ROC curves of NYU PHFI was examined, respectively, in order to find out the optimal cut-off point.
NYU PHFI score was positively correlated with the modified Ross score (r = 0.909, P = 0.000). According to modified Ross score, NYU PHFI scores in different severity of heart failure in children differed significantly (F = 80.034, P = 0.000). A significantly positive correlation was found between plasma NT-proBNP and modified Ross score, and between NT-proBNP and NYU PHFI score. Correlation coefficients between plasma NT-proBNP and modified Ross score, and between plasma NT-proBNP and NYU PHFI score were 0.752 and 0.918, respectively. The correlation between NYU PHFI and plasma NT-proBNP was superior to that between modified Ross score and plasma NT-proBNP. According to modified Ross scores of 0 - 2 as being without heart failure, 3 - 6 as mild degree of heart failure, 7 - 9 as moderate degree of heart failure and 10 - 12 as severe degree of heart failure, the areas under the ROC curve of the NYU PHFI diagnosing if heart failure was present, differentiating moderate from mild and severe from moderate heart failure were 0.982, 0.942 and 0.918, respectively, and the sum of sensitivity and specificity was favorite when 6, 10 and 13 scores were set as cut-off value diagnosing the presence of heart failure, differentiating moderate from mild, and severe from moderate heart failure, respectively. According to above classification of heart failure based on NYU PHFI score, plasma NT-proBNP concentration was significantly different in different degree of heart failure (F = 53.31, P < 0.001). Plasma NT-proBNP concentration in those without heart failure was significantly lower than that of mild heart failure, and it was also significantly lower in mild heart failure than that of severe heart failure.
NYU PHFI was highly valuable for diagnosing chronic heart failure in children and 0 - 6 scores as being without heart failure, 7 - 10 scores as mild degree, 11 - 13 scores as moderate degree and 14 - 30 scores as severe degree of heart failure could be used as the reference criteria of different severities of heart failure.
Zhonghua er ke za zhi. Chinese journal of pediatrics 09/2010; 48(9):703-7.
-
Zhonghua er ke za zhi. Chinese journal of pediatrics 01/2010; 48(1):36-8.
-
Li Chen,
Cheng Wang,
Hong-Fang Jin,
Hong Tian,
Li Wang,
Xiu-Fen Hu,
Hong-Wei Wang, Wan-Zhen Li,
Zhong-Dong Du,
Yu-Li Wang,
Qing-You Zhang,
Jun-Bao Du
[show abstract]
[hide abstract]
ABSTRACT: To examine the diagnostic value of head-up tilt test in children with unexplained syncope (UPS).
A total of 379 children (171 males, 208 females) aged 3-18 years, mean age (12 +/- 3) years with unexplained syncope from Beijing, Hunan, Hubei and Shanghai and undergoing baseline head-up tilt tests (BHUTT) or head-up tilt tests potentiated with nitroglycerine (SNHUTT) under a quiet circumstance were selected as the syncope group. Ten healthy children (5 males, 5 females) aged 9-15 years with a mean age of (11.4 +/- 2.1) years, were recruited as the control group. SPSS 10.0 software was used for data analysis.
In 379 children with unexplained syncope, 67 (17.7%) were of postural orthostatic tachycardia syndrome (POTS), 157 (41.4%) of vasovagal syncope vasoinhibitory pattern, 14 (3.7%) of vasovagal syncope cardioinhibitory pattern, 47 (12.4%) of vasovagal syncope mixed pattern, 1 (0.3%) of orthostatic hypotension (OH) and 93 children (24.5%) of UPS. In syncope group and control group, the positive rate of BHUTT was 55.9% and 0 respectively and it was 75.5% and 20.0% respectively for SNHUTT. During BHUTT, the mean time of positive response occurrence was (16 +/- 12) minutes, and the posture when positive response appeared was at a tilt angle of 60 degrees. For SNHUTT, the mean time of positive response occurrence was (6 +/- 4) minutes and the posture was at a tilt angle of 60 degrees when potentiated with nitroglycerine.
HUTT is an objective diagnostic tool of UPS. With a high diagnostic positive rate, SNHUTT can improve the diagnostic positive rate of BHUTT. Meanwhile the time of positive response occurrence during SNHUTT is markedly shorter than BHUTT.
Zhonghua yi xue za zhi 07/2009; 89(28):1947-50.
-
[show abstract]
[hide abstract]
ABSTRACT: To explore the clinical characteristics of cardiac syncope (CS) in children, and understand their significance in predicting the cardiac syncope.
Twenty-three patients were referred to our department for evaluation of syncope. The diagnosis of the above cases was cardiac syncope. Each patient was interviewed using a standard questionnaire. The clinical histories and standard baseline electrocardiogram were analyzed to identify the variables contributing to the diagnosis of CS in children.
A cardiac cause was identified in 23 syncopal patients presenting to the Department of Pediatrics, Peking University First Hospital: sick sinus syndrome in 7, congenital long QT syndrome in 4, third degree atrioventricular block in 2, supraventricular tachycardia in 2, ventricular tachycardia in 1, atrial fibrillation in 1, pacemaker dysfunction in 1, idiopathic pulmonary hypertension in 3, hypertrophic cardiomyopathy in 1, and dilated cardiomyopathy in 1. The average age of CS patients was 9 years. In totally 23 patients, exertion related syncope spells were found in 14 cases (60.9%), syncope spells at various position 7/23 (30.4%), absence of prodromes in 12/23 (52.2%), syncope spells with incontinence in 4/23 (17.4%), history of heart disease in 4/23 (17.4%). Abnormal standard baseline electrocardiogram was found in 21 cases (91.7%).
The children with cardiac syncope have overt clinical features, especially abnormal findings in electrocardiogram and exertion related syncope spells are the most common clinical features.
Zhonghua er ke za zhi. Chinese journal of pediatrics 02/2009; 47(1):44-7.
-
[show abstract]
[hide abstract]
ABSTRACT: The study was designed to examine the effect of selective alpha1 receptor agonist midodrine hydrochloride in the treatment of children with postural orthostatic tachycardia syndrome.
Fifty-five children (23 male, 32 female, age 5 - 19 yrs, mean age 12.3 +/- 3.1 yrs) who came from Peking University First Hospital were included in the study and clinical investigations as well as standing test, basic head-up tilt test and sublingual nitroglycerin-provocated head-up tilt test under quiet circumstance were conducted. They were randomly divided into treatment group (with midodrine hydrochloride and oral rehydration salt treatment) and control group (with oral rehydration salt treatment only). At last, the disease-free rate, improvement rate and effective rate of symptoms, and the rate of HUT from positive to negative response were compared between control group and treatment group. SPSS 10.0 software was used for the statistical analysis of these data.
The symptom improvement rate in treatment group was significantly higher than that of control group after three and six weeks of treatment (100.0% vs. 42.4%, P < 0.001; 100.0% vs. 42.4%, chi2 = 19.352, P < 0.001). The disease-free rate at follow-up end-point in treatment group was significantly higher than that of control group (77.3% vs. 27.3%, chi2 = 13.239, P < 0.001). The effective rate at follow-up end-point in treatment group was also significantly higher than that of control group (100.0% vs. 36.4%, chi2 = 22.647, P < 0.001). The rate of HUT changing from positive to negative response between two groups after three weeks of treatment was not significantly different (31.8% vs. 12.1%, P > 0.05), but it was significantly different (81.0% vs. 48.5%, P < 0.05) after six weeks of treatment.
Selective alpha1 receptor agonist midodrine hydrochloride is effective in the treatment of children with postural orthostatic tachycardia syndrome.
Zhonghua er ke za zhi. Chinese journal of pediatrics 09/2008; 46(9):688-91.
-
Li Chen,
Jun-bao DU,
Qing-you Zhang,
Cheng Wang,
Zhong-dong DU,
Hong-wei Wang,
Hong Tian,
Jian-jun Chen,
Yu-li Wang,
Xiu-fen Hu, Wan-zhen Li,
Ling Han
[show abstract]
[hide abstract]
ABSTRACT: Syncope is a common pediatric emergency. Based on an epidemiologic survey in the USA, around 15% of children experienced syncopal attack, which strongly influenced the life, study and hurt the children mentally and physiologically. Therefore, exploring the therapeutic regimen has become a hot topic in the field of pediatric cardiology. The aim of this study was to examine the effect of beta receptor blocker in the treatment of children with autonomous nerve mediated syncope.
Totally 103 children (43 males, 60 females, age 5 - 19 yrs, median 12.0 +/- 2.6 yrs) with autonomous nerve mediated syncope from Beijing, Hunan, Hubei and Shanghai, were included in this study. Forty-nine of them suffered from vasovagal syncope (VVS) and 54 suffered from postural tachycardia syndrome (POTS). They were randomly divided into treatment group accepting oral metoprolol treatment and control group accepting oral rehydration salt treatment. The frequency of syncopal episodes and the outcome of head-up tilt (HUT) test were observed. SPSS 10.0 software was used for the statistical analysis of these data.
The cure rate of children who suffered from VVS and POTS and took oral metoprolol was 60.61% and 68.75%, respectively, but in the control group, the cure rate was only 18.75% and 0.00%, respectively. The rate of improvement of children who suffered from VVS and POTS and were treated with oral metoprolol was 15.15% and 15.63%, respectively, and in the control group, it was 6.25% and 40.91%, respectively. The effective rates for cases of VVS and POTS treated with oral metoprolol were higher than those of cases received oral rehydration salt treatment (P < 0.01). The percentage of the change from positive HUT to negative for children with VVS and POTS who took oral metoprolol therapy was 60.61% and 68.75%, respectively, but in control group, it was only 18.75% and 9.09%, respectively (P < 0.01). There was a significant difference in the percentage of the change from positive HUT to negative between children with VVS treated with oral metoprolol and with oral rehydration salt (P < 0.01). Also, a significant difference was found in the percentage of the change from positive HUT to negative between children with POTS treated with oral metoprolol and with oral rehydration salt (P < 0.01).
beta receptor blocker is effective in the treatment of children with VVS or POTS.
Zhonghua er ke za zhi. Chinese journal of pediatrics 12/2007; 45(12):885-8.
-
[show abstract]
[hide abstract]
ABSTRACT: To investigate the value of hemodynamic changes during head-up tilt (HUT) test and predictive value thereof in evaluating the efficacy of metoprolol therapy in children with vasovagal syncope (VSS).
Twenty-six consecutive children with history of VSS diagnosed by head-up tilt (HUT) or sublingual nitroglycerin potentiated head-up tilt (SNHUT), who were treated with metoprolol for 6 approximately 12 months and followed up for (18 +/- 9) months (12 approximately 36 months), were divided into two groups according to effect of metoprolol: effective treatment group (n = 16, aged 12 +/- 2) without VSS recurrence during treatment and fellow-up, and futile treatment group (n = 10, aged 12 +/- 3). The heart rate changes during HUT or SNHUT were evaluated between the two groups.
There were no significant differences between these 2 groups with regard to the demographic and clinical characteristics including age, gender, history, syncope spells, follow-up time and heart rate, mean blood pressure in supine position and during positive response. For example, the baseline heart rate of the effective treatment group was 81 +/- 12 beats/min, not significantly different from that of the futile treatment group (78 +/- 8 beats/min, P = 0.804). However, during tilt test 16 of the 26 patients in the effective treatment group showed tachycardia before positive response, with the mean heart rate of (123 +/- 15) beats/min, whereas all 10 patients in the futile group did not have tachycardia before positive response, with the mean heart rate of (96 +/- 17) beats/min. If an increase of 30 beats/min was taken as a borderline in heart rate during positive response in HUT compared with that of baseline value, in respect of predicting the metoprolol efficacy in the treatment of VVS, the sensitivity was 81% (13/16), the specificity was 80% (8/10), and the diagnostic value was 81% (21/26).
A marked increase in heart rate in HUT or SNHUT is a better predictor of metoprolol efficacy in the treatment of children with VVS.
Zhonghua yi xue za zhi 06/2007; 87(18):1260-2.
-
[show abstract]
[hide abstract]
ABSTRACT: Syncope is a common problem in children and adolescents. Such an event may have multiple possible causes, ranging from benign conditions to life-threatening diseases. Syncope is a major challenge for the practicing physicians. It is very important to know the etiologic and clinical characteristics of syncope in children. This study aimed to improve diagnostic efficacy of syncope in children by analyzing the etiology and clinical characteristics of syncope.
The investigators retrospectively analyzed the causes of syncope and diagnostic workup of 154 consecutive children seen in Department of Pediatrics, Peking University First Hospital because of a syncopal event.
Autonomic-mediated reflex syncope (AMS) was the most common cause of syncope (65.6%), whereas cardiac disorders were found in 10 cases (6.5%) comprising the second cause of syncope in children. Other causes included psychologic problems and neurological and metabolic disorders. Although many causes were studied, 25 cases (16.2%) were found to have uncertain etiologies yet. The children with AMS were commonly seen in pubertal girls, and they had clear inducement of syncope and prodromes. The children with cardiac syncope often had history of cardiac diseases, and they were often younger than those with AMS. Lack of prodromes of syncope, exercise-related syncope, syncope spells seen in any body position, frequent syncope spells and sudden death in family were clues of cardiac syncope. Neurological disorders should be considered if there are any of the followings: syncope with seizure activity, syncope spells seen in any position, and a postictal phase of disorientation or neurologic abnormal signs. A metabolic cause was entertained when the child had a history of metabolic diseases, prolonged anger, or violent vomiting and diarrhea. Children with psychiatric disorders were adolescent girls with prolonged syncope spells, and had more frequent syncopal episodes. Most children with syncope were evaluated by many of diagnostic tests, but most of those tests were not goal-directed approach. Since persons with cardiac syncope were at increased risk for death from any cause, electrocardiography was recommended in almost all children with syncope. Neurologic testing including electroencephalography, computed tomography, etc. were rarely helpful unless neurologic signs and symptoms are present. Holter electrocardiography and echocardiography were most useful in children with suspected cardiac syncope. There was little benefit of screening cardiac enzyme in children with syncope. Routine blood tests (blood electrolytes and blood glucose, etc) rarely yield diagnostically useful information unless the children had the history of metabolic diseases. Head-up tilt testing was most useful in children with recurrent syncope in whom heart disease was not suspected. The children with frequent syncope, long lasting syncopal episode and clear psychiatric inducement of syncope should be evaluated by psychiatric testing.
Syncope in children may result from a wide variety of causes, and clinicians often use a wide range of investigation to try to achieve a diagnosis. But most of investigations have low diagnostic yield. Thorough history taking, physical examination and electrocardiography are the core of the syncope workup.
Zhonghua er ke za zhi. Chinese journal of pediatrics 02/2007; 45(1):59-63.
-
[show abstract]
[hide abstract]
ABSTRACT: Brain natriuretic peptide (BNP) is a cardiac hormone and its plasma level increases in congestive heart failure and myocarditis. An increased level of serum BNP has been reported in children in the acute stage of Kawasaki disease (KD). But the mechanism of increased level of BNP in children with KD has not been elucidated and the change in BNP in children with KD in China has not been reported. The aim of this study was to investigate the change in plasma N-terminal pro-brain natriuretic peptide (NT-pro BNP) in children with KD, examine the value of NT-pro BNP in the diagnosis of KD and explore the mechanism of the change in plasma NT-pro BNP in children with KD.
Thirteen patients, aged from 4 months to 56 months, with KD were enrolled and nine patients with acute upper respiratory infection were used as controls. Blood sample was obtained to measure plasma NT-pro BNP concentrations in the acute (n = 13) and convalescent (n = 8) phases of KD and in the acute phase of the control patients. Plasma NT-pro BNP was measured using enzyme immunoassay. Other laboratory data including complete blood cell count, C-reactive protein, etc, were also measured in acute phase in both groups. The serum cardiac troponin I was also detected in acute phase of children with KD. All patients with KD had complete echocardiographic study, including measurement of left ventricular end diastolic diameter (LVDd), left ventricular ejection fraction (LVEF) and left ventricular inflow velocity through the mitral annulus (including E-velocity and A-velocity). Two dimensional echocardiography was performed to check for coronary lesions of patients with KD. The correlation between plasma NT-pro BNP and the above parameters was analyzed.
The mean plasma NT-pro BNP concentration in patients with KD in the acute phase was (691 +/- 86) ng/L, and it was (47 +/- 10) ng/L in patients of control group. The plasma NT-pro BNP in patients with KD in the acute phase was significantly higher than that of the control group (P < 0.001). In 8 cases of KD, the plasma NT-pro BNP concentrations were measured both in the acute and convalescent phase. The mean plasma NT-pro BNP concentration in the acute phase of KD was (636 +/- 89) ng/L and it was (164 +/- 35) ng/L in the convalescent phase. The level of plasma NT-pro BNP decreased significantly in the convalescent phase (P < 0.01). Through linear regression analysis, there was no significant correlation between the plasma concentrations of NT-pro BNP in acute phase of KD and LVEF, LVDd and E/A ratio, respectively. But the NT-pro BNP level correlated positively with C-reactive protein and white blood cells counts (r = 0.615, P < 0.05 and r = 0.547, P < 0.05), respectively. NT-pro BNP level correlated positively with serum cTnI, a sensitive biologic marker of cardiac injury (r = 0.611, P < 0.05).
The plasma NT-pro BNP concentration increased in the acute phase and decreased significantly in the convalescent phase of KD. The plasma NT-pro BNP might be one of the useful biological markers of KD, and the mechanism of change in plasma NT-pro BNP in KD might be associated with cardiac injury and inflammatory factors.
Zhonghua er ke za zhi. Chinese journal of pediatrics 01/2007; 44(12):886-90.
-
[show abstract]
[hide abstract]
ABSTRACT: Detecting the atrioventricular annular velocity along the long axis of ventricle by tissue Doppler imaging (TDI) is a useful modality to quantitatively assess global myocardial function. The present study was designed to quantitatively assess ventricular function in healthy children by TDI and to evaluate the clinical effect of growth and echocardiographic parameters on TDI velocities during childhood.
The study enrolled 242 healthy children aged 3 days to 17 years and they were divided into 8 groups: < 1 month of age group (37 cases), 1 month-of age group (28 cases), 7 months-of age group (21 cases), 1 year-of age group (36 cases), 4 years-of age group (40 cases), 7 years-of age group (26 cases), 10 years-of age group (28 cases) and > or = 13 years of age group (26 cases). Pulsed wave TDI velocities were obtained at the lateral mitral annulus (MA-L), basal septum (MA-S) and lateral tricuspid annulus (TA) during ventricular systole (Sa), early diastole (Ea) and late diastole (Aa), and Ea/Aa and E/Ea were obtained. Conventional echocardiography performed done and the parameters of left ventricular end-diastolic dimension (LVEDD), left ventricular ejection fraction (LVEF), the transmitral and transtricuspid flow E wave and A wave velocities and E/A ratio were obtained. TDI parameters were compared with demographic and echocardiographic variables.
Sa, Ea and Ea/Aa were the lowest in children < 1 month of age [MA-L: Sa (4.8 +/- 0.7) cm/s, Ea (6.6 +/- 1.1) cm/s; MA-S: Sa (4.1 +/- 0.6) cm/s, Ea (5.0 +/- 0.8) cm/s; TA: Sa (6.2 +/- 1.2) cm/s, Ea (6.4 +/- 1.0) cm/s], and increased with age. The increase was significant from 1 month- to 1 year-of age group 1 year-of age group: MA-L: Sa (8.5 +/- 2.0) cm/s, Ea (16.3 +/- 2.6) cm/s; MA-S: Sa (7.2 +/- 0.8) cm/s, Ea (12.2 +/- 1.6) cm/s; TA: Sa (12.6 +/- 2.3) cm/s, Ea (14.7 +/- 2.6) cm/s. Ea and Sa of TA reached the older children's value earlier than those of the mitral annulus did. Aa increased in the 1 month-of age group compared to < 1 month of age group and remained stable beyond 1 year-of age group. Mitral annulus E/Ea ratio was high among neonates to 7-months-old children (MA-L: 9.2 +/- 2.1, MA-S: 12.1 +/- 2.9), and decreased with age, and there was a significant decrease in 1 year-of age group (MA-L: 5.9 +/- 1.2, MA-S: 7.8 +/- 1.3). In these healthy children, all the above TDI parameters except Aa were influenced by age, body surface area (BSA), LVEDD and heart rate. The influence of age and BSA showed a logarithm model. LVEDD was the main factor that influenced Sa and Ea of MA-L and MA-S, and it was the only single factor that influenced E/Ea ratio at mitral annulus.
This study demonstrated that the left and right ventricular function developed with age in childhood, and it developed most rapidly during infancy and toddler period. The right ventricular function matured earlier than that of the left ventricle. Cardiac growth, age, and heart rate had important clinical effects on TDI velocities during childhood, and LVEDD had the most important influence on left ventricular systolic and diastolic function.
Zhonghua er ke za zhi. Chinese journal of pediatrics 11/2006; 44(10):738-42.
-
[show abstract]
[hide abstract]
ABSTRACT: To explore the different hemodynamic patterns during the course of head-up tilt tests in children with unexplained syncope and the association of clinical features with different hemodynamic patterns in head-up tilt test in children with unexplained syncope.
100 pediatric patients with unexplained syncope, aged 11 +/- 2 (6-16), 36 male and 64 female, with the mean course of 10 +/- 16 months (2 d-5 y), underwent head-up tilt tests or head-up tilt tests potentiated with nitroglycerine under quiet circumstance. Blood pressure and heart rate were monitored simultaneously. According to their different hemodynamic patterns, they were divided into vasovagal response pattern, postural orthostatic tachycardia syndrome (POTS) response pattern, orthostatic hypotension (OH) response pattern and normal response pattern. The vasovagal response was divided into vasodepressor, cardioinhibitory and mixed patterns. The distribution and different clinical features of different response patterns in the unexplained syncope were also studied.
Fifty (50%) of the 100 children with unexplained syncope displayed the hemodynamic pattern of vasovagal response, among which 31 (31%) displayed the pattern of vasodepressor response, 7 (7%) cardioinhibitory response, and 12 (12%) mixed response. Thirty-three patients (33%) displayed POTS response, 2 (2%) OH response, and 15 (15%) the normal hemodynamic response. Patterns of dysautonomic response and chronotropic incompetence were not observed in these children with unexplained syncope. The age of the children with normal response during HUT was 10 +/- 3 years, significantly younger than that of the children with vasovagal response and POTS response (12 +/- 2 and 12 +/- 2, both P < 0.01). There were no differences in sex ratio and duration of syncope among the vasovagal response, POTS and normal response. But the syncopal spells in the children with POTS response was less frequent and the baseline heart rate of the children with POTS response was 81 +/- 7, significantly faster than that of the children with vasovagal response and normal response (71 +/- 9 and 74 +/- 7, both P < 0.01). There was no significant difference in the baseline blood pressure among the children with vasovagal response, POTS and normal response. There were also no significant differences in the age, sex ratio, and duration of syncope, number of syncopal spells, baseline heart rate, and baseline blood pressure among the children with vasodepressor response, cardioinhibitory response and mixed response.
There are different hemodynamic response patterns in head-up tilt testing in children with unexplained syncope, and there was some association between hemodynamic response patterns and their clinical features.
Zhonghua yi xue za zhi 07/2005; 85(28):1962-5.
-
Zhonghua er ke za zhi. Chinese journal of pediatrics 06/2005; 43(5):392.
-
[show abstract]
[hide abstract]
ABSTRACT: Lightheadedness, dizziness, chest discomfort, headache, pallor, palpitation, blurred vision, fatigue, and syncope are main symptoms of postural orthostatic tachycardia syndrome (POTS), but they are also common problems in clinical pediatrics. Besides, most of POTS cases have normal findings in routine examinations on cardiac and central nervous system. Therefore, they are often unable to be correctly diagnosed and treated in time. Since head-up tilt test (HUT) was introduced to diagnose unexplained syncope in children, it was discovered that the most common pattern of unexplained syncope in children was vasovagal syncope. But in the course of the investigation, we and other groups identified a large subgroup of patients who had a less severe form of orthostatic intolerance characterized by postural tachycardia, lightheadedness, dizziness, and chest discomfort, etc. This disorder has become generally known as the POTS, which in children is not rare, but its report was not seen in China so far. The aim of this article is to recognise the clinical pictures of POTS in children and to explore its diagnostic criteria and therapeutic protocol.
All 28 pediatric patients of POTS who met the diagnostic criteria were selected to be the observation group. Age and sex distribution were observed, and the duration of symptoms, baseline heart rate and blood pressure were also recorded. The frequency of symptoms, the rate of misdiagnosis and the efficiency of therapeutic protocol were also analysed.
Twenty-eight patients were diagnosed as POTS, occupying 31.8% of all unexplained syncope or lightheadedness in children. Eleven were males and 17 females. The ratio of male to female was 1:1.5. All the 28 cases were from 6 to 16 years old, 5 of which were < 10 years and 23 were from 10 to 16 years old, including 10. The duration of symptoms of POTS in children was from 1 month to 6 years. The average was about 13 months, and more than one half were in 6 months. The most common orthostatic symptoms were lightheadedness or dizziness, syncope, chest discomfort, pallor and blurred vision associated with nausea and/or vomiting. During HUT or standing, an increase in heart rate > 35 beats per minute within 10 minutes was the most common finding, and some were associated with > 120 beats per minute within 10 minutes after HUT or standing up. The average time of the occurrence of abnormal responses was about 5 minutes. Especially, we found only 36% of patients were diagnosed with standing up test. Most of them were diagnosed with HUT. POTS in children was misdiagnosed as epilepsy or myocarditis in 43% patients. Multiple treatment protocol including health education and supportive, physical and medical therapy were most helpful in children with POTS.
POTS is commonly seen in school-aged girls. The common symptoms include dizziness, syncope and chest distress. HUT is an important tool in its diagnosis. A comprehensive therapeutic regimen is recommended in the treatment.
Zhonghua er ke za zhi. Chinese journal of pediatrics 03/2005; 43(3):165-9.
-
[show abstract]
[hide abstract]
ABSTRACT: Syncope is one of the common problems in clinical pediatrics. In recent years, vasovagal reflex was identified as the most common reason for unexplained syncope. Head-up tilt test (HUT) was regarded as one of the important diagnostic criteria. But the sensitivity of baseline head-up tilt test (BHUT) is low. To improve the sensitivity of HUT, we evaluated the diagnostic value of head-up tilt testing potentiated with sublingual nitroglycerin (SNHUT) in children with unexplained syncope.
Twenty-five patients (11 male and 14 female, mean age 11.8 +/- 2.1 years, ranged from 8 to 15) with unexplained syncope and 10 healthy children in control group (5 male and 5 female, mean age 11.4 +/- 2.1 years, ranged from 9 to 15) were studied. The patients and healthy children were tilted upright to 60 degrees for 45 minutes at BHUT. If syncope did not occur, sublingual nitroglycerin (4 - 6 microg/kg, maximum dose 300 microg) was administered, and continued at same degree for 20 minutes.
Compared with controls, there was no significant difference in sex, age, supine blood pressure and supine heart rate of syncopal group (P > 0.05). During BHUT, 12 patients (48%) and none of the controls had positive response, whereas another 8 patients and 2 controls had positive response during SNHUT. In syncopal group, total positive rate was 80%. The sensitivity, specificity, and diagnostic value of SNHUT were all 80%. During SNHUT, the mean time to positive response was 5.9 +/- 2.9 minutes (from 4 to 11 minutes), and only 1 patient had mild headache.
Head - up tilt testing potentiated with sublingual nitroglycerin (4 - 6 microg/kg, maximum dose 300 microg) as pharmacological provocation test was a useful and an objective diagnostic tool for evaluating vasovagal syncope in children.
Zhonghua er ke za zhi. Chinese journal of pediatrics 06/2004; 42(5):371-4.
-
Zhonghua er ke za zhi. Chinese journal of pediatrics 07/2003; 41(6):479.
