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Kawasaki disease (KD) is an acute, self-limiting, and medium-sized vasculitis, which has been the commonest cause of acquired heart disease in children in developed countries.Without timely diagnosis and treatment, up-to 25% of the affected children may develop coronary artery abnormalities (CAA). Due to the lack of the specific diagnostic method, KD is mainly diagnosed according to the clinical criteria.As a result, typical KD is recognized easily, but it is a big challenge to diagnose KD patients with incomplete or atypical symptoms.The pandemic of novel coronavirus disease 2019 (COVID-19) around the world makes the diagnosis of KD even more complex.In this review, hot issues in diagnosing KD were discussed according to the 2017 guidelines for diagnosis and treatment of KD recently published by the American Heart Association (AHA), expecting to provide help for diagnosis of KD children.
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Objective To explore the effect of different concentrations of endothelin-1 (ET-1)on the en-dogenous nitric oxide (NO)and hydrogen sulfide (H2S)pathways of vascular smooth muscle cells (A7r5 cell lines)in rats.Methods A7r5 cell lines were divided into the control group and the experimental group.ET-1 at a concentra-tion of 10 -8-10 -6 mol/L was added into the experimental group,and as for the control group,the same volume of sterile phosphate buffered saline (PBS)buffer solution was added.The content of NO and H2S in A7r5 cell lines was detected by fluorescent NO probe and H2S probe after ET-1 stimulation for 48 h,respectively.The content of NO in the supernatant was measured by NO assay kit at 48 h of the incubation.The content of H2S in the supernatant was measured by polarographic H2S sensor at 48 h of the incubation. The expressions of inducible nitric oxide synthase (NOS2),endothelial nitric oxide synthase (NOS3),cystathionine -γ -lyase (CSE),cystathionine -β -synthase (CBS)and proliferating cell nuclear antigen (PCNA)were detected by the Western blot method.Results The rela-tive fluorescence intensity of the content of NO in the A7r5 cell lines of ET-1 10 -8,10 -7 and 10 -6mol/L groups (0. 078 ± 0. 080,0.075 ± 0.002,0.056 ± 0.009)was markedly lower than that in the control group(0.094 ± 0. 061), and the differences were statistically significant(F＝15.248,P＜0.05);Compared with the control group[(2. 131 ± 0. 484)μmol/L],the content of NO in the supernatant of the experimental groups [(1.391 ± 0.134 )μmol/L, (1.219 ± 0. 280)μmol/L,(1.116 ± 0.181)μmol/L]was significantly decreased,and the differences were statistically significant(F＝20.833,P＜0.01);NOS2 protein expression(0.457 ± 0.097,0.462 ± 0.116,0.438 ± 0.180)was decreased markedly compared with that of the control group(0.721 ± 0.222),and the differences were statistically sig-nificant(F＝6.196,P＜0.01),but the expression of NOS3 showed no significant differences(F＝2.669,P＞0.05). The relative fluorescence intensity of the content of H2S in the A7r5 cell lines of ET-1 10 -8,10 -7 and 10 -6mol/L groups (0.063 ± 0.002,0.056 ± 0.008,0.042 ± 0.009)was markedly lower than that in the control group (0.082 ± 0. 006),and the differences were statistically significant(F ＝16.297,P＜0.01);Compared with the control group [(29.439 ±4.236)μmol/L],the content of H2S in the supernatant of the experimental groups [(17.516 ±5.144) μmol/L,(14.481 ± 4.885)μmol/L]was significantly decreased,and the differences were statistically significant (F＝12.518,P ＜0.01).CBS protein expression(0.359 ± 0.096,0.270 ± 0.038,0.174 ± 0.051)was decreased markedly compared with that of the control group(0.707 ± 0.107),and the differences were statistically significant (F＝20.833,P＜0.01),and the expression of CSE showed no significant differences(F＝0.708,P＞0.05).The data showed that PCNA protein expression in the 10 -7mol/L ET-1 group(0.686 ± 0.180)significantly increased com-pared with that of the control group(0.437 ± 0.191),and the difference was statistically significant (t＝ -2.840,P＜0.01).Conclusion ET-1 stimulation can lead to the proliferation of vascular smooth muscle cells and down-regu-late its endogenous NO and H2S pathways.
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Arrhythmia-induced cardiomyopathy (AIC) is a myocardial disease condition in which left ventricular dysfunction and cardiomegaly are induced or mediated by atrial or ventricular arrhythmias.The pathophysiological mechanisms remain unclear.Early recognition of AIC and provision of prompt treatment with pharmacological or ablative techniques could result in symptom resolution and recovery of ventricular function.But,the long-term prognosis of these patients is not clear and needs further observation and research.
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Objective To investigate the clinical effect and the prospect of Infliximab in treatment of intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) patients.Methods Clinical features,inflammatory markers and coronary changes were observed in 2 cases of IVIG-resistant KD patients hospitalized in Peking University First Hospital,who were treated effectively by Infliximab.Relevant researches on the mechanism and progress of the Infliximab treatment for IVIG-resistant KD in the last 10 years were reviewed at the same time.Results Two KD patients hospitalized in Peking University First Hospital had been treated with 2 g/kg IVIG for 2 times and followed by methylprednisolone treatment.However,fever and other clinical manifestations occurred again after 2 days and 6 days when temperature returned normal.They both defervesced and all the symptoms were improved after 1 dose of Infliximab (5 mg/kg) by laboratory examinations.Four published literatures of the basic research and 9 retrospective or prospective clinical researches of Infliximab treatment of KD showed that Infliximab alleviated the inflammatory level in the KD patients significantly.Complete remission was up to 72.73％-92.11％.Those KD patients defervesced within 12 h,with dramatic improvement of symptoms and signs.Arthralgia also disappeared in the patients with arthritis.Only 1 case was complicated with hepatitis in the acute phase and cholecystitis in recovery time.A phase 3 randomised,double-blinded,placebo-controlled trial had been done to assess the addition of Infliximab to the standard therapy.Conclusions Infliximab is a feasible choice for IVIG-resistant KD patients.Efficacy and safety of Infliximab for KD treatment have been proved in the literature.However,Infliximab for KD treatment has not been indicated in the drug instruction,so the informed consent from the guardians and Ethics Committee is needed.
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<p><b>OBJECTIVE</b>To explore possible mechanisms of postural tachycardia syndrome (POTS) by comparing plasma intermedin (IMD) during head-up tilt test (HUTT) in children with POTS.</p><p><b>METHOD</b>The study subjects were divided into two groups: POTS group and control group. The POTS group consisted of twenty-nine children (male 14, female 15) with POTS, the mean age (12.4 ±3.1) years old, admitted into Peking University First Hospital from November 2013 to June 2014. The control group consisted of 32 healthy children (male 17, female 15). Their mean age was (11.6±2.2) years old, who were confirmed as healthy by physical examination and HUTT. Finapres Medical System was used to continuously monitor heart rate and blood pressure during HUTT, and electrocadiogram was performed. Supine systolic and diastolic blood pressure, mean arterial pressure (MAP), ΔMAP (standing mean arterial pressure-supine MAP), supine heart rate and ΔHR (standing HR-supine HR) were compared between POTS group and control group. Sandwich immunoluminescence assay was used to test plasma IMD. The plasma IMD level was compared in supine between POTS and control group. The plasma IMD level in supine was compared with HUTT in POTS group.</p><p><b>RESULT</b>No significant differences were found in age, height, weight, supine systolic and diastolic blood pressure, MAP, ΔMAP and supine heart rate between POTS group and control group (P>0.05). ΔHR in POTS group was significantly higher than that of control group ((48±10) vs. (22±7) beats /min, t=9.797, P<0.05). The plasma IMD level in POTS group was lower than that of control group in supine position ((497±61)×10(-6) vs. (529±58)×10(-6) mg/L, t=2.117, P<0.05). But, it was higher during HUTT than supine IMD in POTS group ((537±57) ×10(-6) vs. (497±61)×10(-6) mg/L, t=-2.464, P<0.05). The plasma delta IMD level (HUTT vs. supine) was positively correlated with delta HR in POTS group (r=0.435, P<0.05).</p><p><b>CONCLUSION</b>The excessively high heart rate during HUTT have a positive correlation with plasma IMD, which may play a role in the pathogenesis of POTS in children.</p>
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<p><b>OBJECTIVE</b>To analyze the clinical value of 24-hour urinary sodium determination in children with postural tachycardia syndrome (POTS).</p><p><b>METHOD</b>Fifty-eight POTS children and 10 healthy children (control group) from Peking University First Hospital during June 2012 to May 2014 were enrolled. Their 24-hour urinary sodium and plasma sodium levels were compared. Correlation analysis was done between 24-hour urinary sodium and symptom scores in children with POTS. All patients were treated with oral rehydration salts. The POTS patients were divided into hyponatriuria group (urinary sodium < 124 mmol/24 h) and hypernatriuria group (urinary sodium ≥ 124 mmol/24 h). Kaplan-Meier curve was used to analyze the effects of different 24-hour urinary sodium levels in children with POTS receiving rehydration salts therapy.</p><p><b>RESULT</b>The 24-hour urinary sodium levels of children with POTS were significantly lower than that of control group ((110. 0 ± 45. 8) vs. (221. 3 ± 103. 6) mmol/24 h, t =3. 339, P = 0. 008), while no statistical significance was found in plasma sodium between the two groups ((139. 7 ± 2. 1) vs. (139. 7 ± 2. 3) mmol/L, t = 0. 082, P = 0. 935). Pearson correlation analysis showed that 24-hour urinary sodium and severity of symptoms in children patients were negatively correlated (r = - 0. 654, P < 0. 001) . Urinary sodium < 124 mmol/24 h was used as the cut-off value, there were 43 cases in hyponatriuria group and 15 cases in hypernatriuria group. The symptom scores were significantly higher in hyponatriuria group (10. 2 ± 3. 7 vs. 5. 0 ± 1. 8, P < 0. 001), there was no significant difference in other basic information and hemodynamic data between groups (P > 0. 05). Logistic regression analysis revealed that urine sodium < 124 mmol/24 h was independent risk factor for effectiveness of rehydration salts in POTS patients (OR = 0. 043, 95% CI:0. 004 - 0. 499, P = 0. 012). Kaplan-Meier survival analysis showed the long-term effect of patients receiving oral rehydration salts in hyponatriuria group was significantly better than that in hypernatriuria group (86. 0 % vs. 60. 0%, χ2 = 8. 471, P = 0. 004).</p><p><b>CONCLUSION</b>Twenty-four hours urinary sodium is a good indicaor for guiding children with POTS receiving rehydration salts therapy.</p>
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Child , Humans , Case-Control Studies , Fluid Therapy , Hemodynamics , Postural Orthostatic Tachycardia Syndrome , Urine , Rehydration Solutions , Salts , Sodium , UrineABSTRACT
<p><b>OBJECTIVE</b>To summarize clinical features and diagnosis of Chinese infantile patients with glycogen storage disease type II (GSD II).</p><p><b>METHOD</b>Six infant patients with GSD II diagnosed from January 2012 to June 2014 in the Department of Pediatrics, Peking University First Hospital were enrolled into this study. Clinical information of the 6 patients, including clinical manifestation, blood biochemistry, chest X-ray, echocardiogram, electrocardiogram, acid alpha-glucosidase (GAA) activity and GAA gene mutation analysis by direct sequencing of polymerase chain reaction (PCR) product were reviewed.</p><p><b>RESULT</b>Of the 6 patients, five were female and one was male, five of whom were classic infantile type while the other one was atypical. The age of onset ranged from birth to 3-month-old. All patients had varying degrees of generalized muscle weakness, hypotonia and development retardation or retrogression. Other common findings were feeding difficulties in two patients, tongue weakness in two patients, respiratory distress in four patients, macroglossia in one patient, and hepatomegaly in two patients. Left ventricular hypertrophy and cardiomegaly were obvious in all the six patients. All six patients were found to have a enlarged heart in physical examination, and three patients who underwent a chest X-ray examination had an enlarged heart shadow. Four patients who had an echocardiography were found to have myocardial hypertrophy. The electrocardiogram in three patients showed short PR intervals and high voltage. The creatine kinase (CK) levels were three to seven times elevated. The mildest elevated CK was 441 IU/L, and the highest CK level was 1 238 U/L. Assay of GAA enzyme activity in whole blood showed significantly reduced activity (1.3 nmol/ (spot·d) to 2 nmol/(spot·d)) in the patients tested. Gene sequencing in 4 patients showed 8 pathogenic mutations, including 6 missense mutations, one nonsense mutation and one frameshift mutation. The missense mutations were c.998C > A (p.Thr333Lys), c.1280T > C (p.Met427Thr), c.1760T > C (p.Leu587Pro), c.1924G > T (p.Val642Phe), c.2012T > A (p.Met671Lys) and c.2105G > A (p.Arg702His). The nonsense mutation was c2662G > T (p.Glu888X), and the frameshift mutation was c2812_2813delTG (p.Cys938fs). The 5 classic infantile patients died at the age of 7 to 22 months. The atypical infantile patient was 2 years and five months old according to our latest follow up.</p><p><b>CONCLUSION</b>Infantile GSD II had similar motor manifestations and cardiac involvements, blood biochemical test, imaging findings, enzyme assays, though there were slight differences. The probability of GSD II should be taken into consideration if an infant has both muscular disease and cardiac involvement.</p>
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Female , Humans , Infant , Infant, Newborn , Male , Asian People , Glycogen Storage Disease Type II , Diagnosis , Pathology , Macroglossia , Muscle Weakness , Mutation , Mutation, Missense , Polymerase Chain Reaction , alpha-Glucosidases , Genetics , MetabolismABSTRACT
<p><b>BACKGROUND</b>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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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 (OI) 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.</p><p><b>CONCLUSIONS</b>POTS is a relatively common condition with complex pathophysiology and heterogeneous clinical manifestation. A comprehensive therapeutic regimen is recommended for the treatment.</p>
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Adolescent , Child , Child, Preschool , Female , Humans , Male , Blood Pressure , Physiology , Exercise Therapy , Methods , Heart Rate , Physiology , Postural Orthostatic Tachycardia Syndrome , Diagnosis , Drug Therapy , Therapeutics , Urine , Retrospective Studies , Salts , Therapeutic Uses , Sodium , UrineABSTRACT
Myocarditis is one of the most common acquired heart diseases in children,and one of the most common causes of a pediatric dilated cardiomyopathy phenotype.The myocarditis is a difficult issue in the diagnosis and the optimal means of therapy.A recent Pediatric Cardiomyopathy Registry (PCMR) analysis in the largest group of pediatric myocarditis patients ever studied confirmed that the most common outcome in pediatric myocarditis was cardiac recovery,but approximately 30％ of pediatric myocarditis patients would die or undergo heart transplantation.Animal studies and adult experience suggested that autoimmunity might contribute to cardiac dysfunction in myocarditis.Immunosuppressive and immunomodulating therapy for pediatric myocarditis remains controversial.Small case series have shown benefit of these therapies in pediatric myocarditis.A limited number of biomarkers associated both good (recovery) and poor (death or transplantation) outcomes could be identified.We should do our best to find these biomarkers in the future.
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Pulmonary hypertension (PH) is a clinical hemodynamic syndrome characterized by increased pulmonary artery pressure and pulmonary vascular resistance. It can cause right ventricular failure, and even death. Pediatric PH is not very common, but is a greatly hazardous disease that leads to a high mortality rate. Therefore, many registry organizations have been established in the world to strengthen the study of diagnosis and treatment of the disease, and improve the understanding of pediatric PH. This article reviewed recently published researches, as well as presented a comprehensive understanding of PH, including definition, classification, epidemiology, prognosis and treatment.
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Child , Humans , Disease Management , Hemodynamics , Hydrogen-Ion Concentration , Hypertension, Pulmonary , Incidence , Prognosis , Pulmonary Artery , Vascular ResistanceABSTRACT
Syncope is a common clinical problem in children and adolescents. It is a major challenge for practicing physicians, and medical resource utilization and expenses associated with syncope management are enormous. A diagnostic protocol to syncope must be developed for children and adolescents for convenient and effective final diagnosis, and an analysis of cost-effectiveness is meaningful. Thus, according to the studies of syncope in children in China,the Chinese Pediatric Cardiology Society proposed the guidelines for diagnosis of syncope in children in China, and developed a simplified diagnostic protocol for children and adolescents with syncope. According to a multi-center prospective study,the diagnostic protocol in children and adolescents with syncope results in an improvement of diagnostic yield.