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AIM: To investigate the differences in varying stages of non-proliferative diabetic retinopathy(NPDR)using optical coherence tomography angiography(OCTA).METHODS: Cross-sectional study. A total of 77 cases(77 eyes)of diabetic patients were included, and they were divided into non-diabetic retinopathy(NDR; 23 eyes)and non-proliferative diabetic retinopathy(NPDR; 54 eyes)groups, further subdivided into mild NPDR(20 eyes), moderate NPDR(20 eyes), and severe NPDR(14 eyes). Foveal avascular zone(FAZ)area, superficial and deep capillary plexus densities(SSP and DSP), and visual acuity(LogMAR)were compared between NDR and NPDR groups. Furthermore, the visual acuity, FAZ area and levels of SSP and DSP were compared in different degrees of NPDR. Correlation analysis were conducted to elucidate relationships between FAZ area, visual acuity, SSP, DSP, and severity of the disease.RESULTS: Compared with the NDR group, the visual acuity(LogMAR)and macular FAZ area increased, while SSP and DSP were decreased in the NPDR group(P<0.05); there were significant differences in visual acuity, FAZ area and SSP and DSP levels in different degrees of NPDR(P<0.05). Visual acuity(LogMAR)and FAZ area displayed a positive correlation with the severity of disease, while SSP and DSP showed a negative correlation.CONCLUSION: With the progression of NPDR, the visual acuity(LogMAR)and FAZ area increased, and the SSP and DSP decreased.
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Objective:To describe the clinical features and prognosis of congenital anomalous origin of coronary artery(AOCA) in children to increase our understanding of the disease.Methods:This retrospective study included children diagnosed with AOCA using computed tomography coronary angiography(CTCA) admitted to the Department of Cardiology, Beijing Children′s Hospital, Capital Medical University, from January 1, 2014 to December 31, 2019.The clinical presentations, laboratory results, imaging analyses, treatments, and prognoses of these patients were analyzed.Results:A total of 208 children, including 105 boys and 103 girls, we evaluated the ages(9.03±4.18)years old with AOCA.Of these, 157 cases(75.5%) presented with cardiac symptoms, such as chest tightness, palpitations, dizziness, syncope, fatigue, and decreased endurance.Three cases(1.4%) had atypical symptoms of paroxysmal crying, dyspnoea and cyanosis, and 48 cases(23.1%) were asymptomatic.Levels of serological markers of myocardial injury were elevated in 59 cases(28.4%), and 140 cases(67.3%) had predominant ST-T abnormalities on electrocardiograms.Transthoracic echocardiography identified 27 cases (13%) with cardiac enlargement and ten cases(4.8%) with left ventricular systolic dysfunction.There were 126 cases(60.6%) with the anomalous origin of the left coronary artery revealed by CTCA, 50 cases(24.0%) with the anomalous origin of the right coronary artery and 32 cases(15.4%) with bilateral coronary arteries of anomalous origin.Five children underwent surgical treatment, and the remaining 203 children were treated conservatively with drugs.The whole group was successfully treated, and no death case was recorded during the follow-up period.Conclusion:AOCA may cause different degrees of myocardial ischemia.Diverse clinical presentations and diagnostic limitations of transthoracic echocardiography often lead to missed diagnosis or misdiagnosis.In contrast, CTCA has high diagnostic accuracy and can be used to identify the location and course of the coronary ostia.Hence, the management of AOCA should be tailored on a case-to-case basis, taking into consideration of the specific type of coronary origin, with surgical intervention being warranted if necessary.
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Objective:To analyze the clinical characteristics of Kawasaki disease(KD)with complications of acute abdominal disease and to improve the diagnosis, treatment and prognosis of patients.Methods:A total of 2 931 cases with KD hospitalized from January 2016 to December 2019 in our department were retrospectively analyzed, including 14 children with acute abdominal disease(acute abdomen group). And 62 patients with KD but without acute abdominal disease were randomly selected as the control group.The clinical characteristics, laboratory examination and treatment between two groups were compared and analyzed.Results:There were 8 males and 6 females.The average age of patients was(4.46±0.74) years.Compared with control group, there was no significant difference in age, sex and proportion of coronary artery injury in acute abdomen group( P>0.05). Acute abdominal disease group were more likely to have gastrointestinal symptoms, intravenous immunoglobulin(IVIG) resistance and longer fever duration( P<0.05). C-reactive protein(CRP), alanine aminotransferase(ALT), aspartate aminotransferase(AST), γ-glutamyltranspeptidase(GGT) and total bilirubin(TBIL) in acute abdominal disease group were significantly higher( P<0.05). There was no significant difference in white blood cell, erythroayte sedimentation rate, hemoglobin, platelet, Na + , albumin between two groups( P>0.05). All of the 14 patients used IVIG, 3 of them were treated with hormone(methylprednisolone)and 1 patient accepted infliximab.All patients were discharged from hospital.After follow-up for 6 months to 3 years, all patients had no sequelae of digestive system. Conclusion:KD can be complicated with acute abdominal disease, some of which as the first symptom.Gastrointestinal manifestations such as abdominal pain are common.For children with obvious gastrointestinal symptoms, IVIG resistance, and long duration of fever, attention should be paid to the possibility of acute abdominal disease.For children with significantly elevated CRP, ALT, AST, GGT, and TBIL should to be alert to the possibility of acute abdominal disease.KD complicated with acute abdominal disease generally has good prognosis.It is important to treat KD in the first place.
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Objective:To provide a theoretical basis for diagnosing and treating recurrent Kawasaki disease based on data analysis of clinical symptoms.Methods:Data analysis of children with recurrent Kawasaki disease admitted to Beijing Children′s Hospital of Capital Medical University from January 2016 to December 2019 was conducted, including comparisons of the initial onset and the recurrence on patients′ clinical features, auxiliary examination and treatment.Results:During the four-year scale, 3 041 children with Kawasaki disease were admitted to the department of Cardiology.The recurrence data involved 65 children[male∶female 3.1∶1, average aged(2.42 ± 2.04) years]. First, children′s fever duration was(5.66 ± 2.37) days in recurrence data, significantly shorter than that of their initial onset.The recurrence data also reported a lower incidence of rash and limb changes, together with respiratory and gastrointestinal symptoms.Then, 19 cases out of the 65 children got coronary artery lesion(CAL) at their initial onset.A relief of CAL was reported from 15 children when the disease recurred, along with 11 new-reported CAL cases.Intravenous immunoglobulin(IVIG) refractory Kawasaki disease cases accounted for nine at the initial onset and 12 at the recurrent onset, respectively.Five IVIG refractory recurrent cases reported significant relief after 2 g/kg IVIG treatment.Conclusion:The recurrent Kawasaki disease in children usually occurs among children under three years old, within the first year after the initial onset.Most of the recurrent cases report a shorter fever duration and less typical clinical symptoms than their first onset.In some cases, IVIG treatment showed effects initially but failed to work when the disease recurred.Therefore, glucocorticoid or infliximab should be considered for further treatment.
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Objective:To summarize the clinical and genetic characteristics of catecholaminergic polymorphic ventricular tachycardia (CPVT) in children caused by CASQ2 gene variants. Methods:The clinical data of 8 children (4 males and females, respectively) with CPVT caused by CASQ2 gene variants admitted to Beijing Children′s Hospital, Capital Medical University from January 2017 to November 2018 were retrospectively analyzed.The targeted next generation sequencing was employed to identify CASQ2 variants and Sanger sequencing was conducted to conform the candidate variants and determine the parental origin. Results:As for 8 children in this study, the average age of onset was 6.4 years, the mean age at diagnosis was 9.4 years, and the average interval from onset to diagnosis was 3 years.Only 2 cases had clearly diagnosis at onset, other 6 cases had a delay to diagnosis and 3 cases of them were diagnosed at other hospitals as having epilepsy and did not respond to anti-epileptic therapy.During physical activity and/or emotional stress, 8 cases presented with recurrent syncope and were able to regain consciousness after a few minutes.They had no a history of sudden cardiac death or family history.There was no abnormality on resting electrocardiogram during the paroxysmal interval in 6 cases and mild sinus bradycardia in 2 cases.Typical bidirectional ventricular tachycardia (VT) and/or polymorphic VT were detected in 8/8 cases and 5/5 cases, respectively, based on Holter electrocardiography and cardiac stress test.The CASQ2 gene variant was found in all children, with 6 cases carrying compound heterozygous variants and 2 cases carrying homozygous variants.A total of 9 different CASQ2 variants were detected in 8 cases, of which 5 had not been previously reported.According to the family-line verification, all of them had a familial variant, with no novel variants.All 8 cases were treated orally with β-blockers, with asymptomatically recurrent episodes, with a mean follow-up of 1.5 years, during which implantable cardioverter defibrillation was performed in 1 case owing to severe sinus bradycardia.There was no death case among them. Conclusions:CPVT with CASQ2 variants is characterized by early onset before preschool age, recurrent syncope after exercise or emotional stress and bidirectional/polymorphic VT.Early diagnosis of CPVT remains challenging due to delayed diagnosis or misdiagnosis.Treatment with β-blockers can achieve favorable effectiveness and safety.Five novel variants in this study would further expand the database of CASQ2 genes.
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Objective:To summarize characteristics and rules of blood pressure variability (BPV) in children with H-type hypertension, in an attempt to explore some basis for further selection of the time points of treatment.Methods:A total of 117 children diagnosed as essential hypertension in Children′s Hospital Affiliated to Capital Institute of Pediatrics from March 2018 to September 2019 were selected as the research objective.According to the serum level of homocysteine (Hcy), those children were divided into the simple essential hypertension group (74 cases, Hcy≤13.9 μmol/L) and the H-type hypertension group (43 cases, Hcy>13.9 μmol/L). All children received 24 h ambulatory blood pressure monitoring (24 h ABPM), and BPV indexes were recorded and subject to group comparison.Meanwhile, the serum Hcy level was monitored.Besides, the demographic data and such blood biochemical indexes as blood glucose, blood fat and renal function were recorded or measured for an analysis of the correlation between BPV and each index.Results:There were no significant differences in gender, age, body mass index (BMI) and biochemical indexes between the simple essential hypertension group and the H-type hypertension group (all P>0.05). Compared with the simple essential hypertension group, the H-type hypertension group had a significantly higher 24 h systolic blood pressure standard deviation [24 h SSD, (11.21±3.23) mmHg vs.(9.64±2.73) mmHg, 1 mmHg=0.133 kPa, t=-2.806, P<0.05], nighttime systolic blood pressure standard deviation [nSSD, (10.79±3.89) mmHg vs.(9.26±3.23) mmHg, t=-2.292, P<0.05], and nighttime diastolic blood pressure standard deviation [nDSD, (10.23±3.53) mmHg vs.(8.73±2.93) mmHg, t=-2.617, P<0.05]. Pearson correlation analysis showed that the serum Hcy level was significantly positively correlated with 24 h, SSD, nSSD and nDSD ( r=0.194, 0.183, 0.182, all P<0.05). Conclusions:24 h SSD in children with H-type hypertension is significantly higher, with an obvious increase in both nSSD and nDSD, and the serum Hcy level was significantly positively correlated with BPV, which suggested that it was required to effectively control the serum Hcy level, reduce the range of blood pressure fluctuation and select the optimal time points of treatment, thus delaying the progress of hypertension.
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Objective@#To investigate the clinical features and improve the diagnosis and treatment of anomalous origin of the left coronary artery from the right coronary sinus with an interarterial course (ALCA-R-IAC) between the ascending aorta and main pulmonary artery in children.@*Methods@#A retrospective analysis of the clinical manifestation, laboratory test, radiological feature, treatment and prognosis were conducted in four female children presented with ALCA-R-IAC in Beijing Children′s Hospital from November 2015 to June 2018.@*Results@#The four girls with onset age of 7.5-14.7 years were diagnosed with ALCA-R-IAC by CT coronary angiography (CTCA). Four children presented with exercise-induced syncope and clinical manifestations of acute myocardial infarction including 3 patients with acute left heart failure, 1 cardiogenic shock and 1 cardiac arrest. Nervous system involvement was found in one patient. Troponin I increased significantly to 20.65-50.00 μg/L in the four patients. Electrocardiogram (ECG) developed signs of left main coronary artery involvement. Echocardiography revealed reduced left ventricular ejection fraction (LVEF) of 25%-45% in three children and suspected anomalous origin of the left coronary artery in one child. CTCA showed an anomalous left coronary artery originating from the right coronary sinus, which had an interarterial course between the aorta and pulmonary artery leading to a slim left main coronary trunk. Two children underwent unroofing procedure and the other two children in whom physical activities were restricted received conservative managements. During a regular follow-up period of 12-43 months, all the children survived without recurrent symptoms and had good prognosis.@*Conclusions@#ALCA-R-IAC can present as exercise-related syncope and acute myocardial infarction, even sudden death in children and adolescents. CTCA is helpful to clarify the early diagnosis of ALCA-R-IAC. Surgical intervention is the main treatment for ALCA-R-IAC and strenuous physical activities should be avoided.
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Objective To evaluate the safety of anesthesia endoscopic retrograde cholangio pancreatography (ERCP) under general anesthesia.Methods From January 1st,2008 to June 30th,2016,patients underwent ERCP under general anesthesia were enrolled as anesthesia group and from January 1st,2005 to December 31st,2007,patients accepted ERCP without anesthesia were enrolled as control group.Chi-square test was performed to analyze disease composition,conditions during operation,success rate of operation and complications in these two groups.Results A total of 14 724 patients with ERCP under general anesthesia and 2 102 patients received ERCP without anesthesia were enrolled.In 14 724 patients with ERCP under general anesthesia,1 799 cases had malignant biliary and pancreatic diseases and 12 925 cases with biliary and pancreatic diseases.During the operation,transient hypoxemia occurred in 441 cases (3.00％) and relieved by increasing oxygen flow,lower anesthetic dose or lifting lower jaw.The success rate of ERCP in the anesthesia group (98.41 ％,14 490/14 724) was higher than that in the control group (97.34％,2 046/2 102),and the difference was statistically significant (x2 =11.500,P=0.001).The incidence rate of post-ERCP pancreatitis in the anesthesia group was 2.35％ (346/14 724),which was lower than that in the control group (3.85％,81/2 102),and the difference was statistically significant (x2 =16.813,P＜0.01).Conclusion ERCP under general anesthesia is safe,which could increase the success rate of operation and reduce the incidence rate of post-ERCP pancreatitis.
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Objective To compare the difference of the effects and safety of endoscopic submucosal dissection (ESD) and surgery for early gastric cancer. Methods We searched the Pubmed, CBM, Embase, Cochrane Library, CNKI, CQVIP and WanFang data from January 1990 to June 2016 studies comparing endoscopic resection with gastrectomy for treatment of early gastric cancer. We selected the eligible studies according the including and excluding criteria. The quality of the included studies was assess using the Newcastle-Ottawa Scale (NOS), then using Revman 5.3 to make the Meta analysis. Result The meta-analysis enrolled 12 studies with 4331 patients, all of the studies were retrospectively analyzed. The result of the meta-analysis showed that there were no significant difference regarding the recurrence rate [(22/2586, 0.85%) vs (6/1134, 0.53%), P = 0.370] and five-year survival rate [(852/909, 93.72%) vs (707/746, 94.77%), P = 0.340] between endoscopic resection and gastrectomy. Gastrectomy was associated with higher en bloc resection rate, which were 100.00% and 92.23% respectively. However, gastrectomy was also related to longer operative time (SMD = -3.04, 95%CI: -3.64 ~ -2.45, P = 0.000) and hospital stay (SMD = -2.53, 95%CI: -3.73 ~ -1.32, P = 0.000). The postoperative complication was also higher than endoscopic, which were (45/816, 5.50%) vs (101/686, 14.72%) respectively. Conclusion There were no significant difference regarding recurrence rate and five-year survival rate between endoscopic and gastrectomy. While the en bloc resection rate was lower than gastrectomy, endoscopic offers a shorter hospital stay, shorter operative time with minimal invasive and fewer operating and postoperative complications than gastrectomy. Endoscopic should be recommended as a standard treatment for early gastric cancer with indications.
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Objective To investigate the clinical features and prognostic factors in children with idi-opathic pulmonary arterial hypertension(IPAH). Methods The data of children with IPAH diagnosed in Beijing Children′s Hospital from January 2006 to December 2015 were collected. The cases were divided into survival group and death group according to the prognosis. The risk factors influencing prognosis in children with IPAH were identified by Logistic regression analysis. Results Total 26 patients were enrolled in this study,and 17 of them survived and 9 of them were dead. IPAH was found to be more common in school-age children. The average age of the patients was(6. 6 ± 3. 6)years and the male to female ratio was 1. 17∶1. All of the children had the symptoms with decreased activity tolerance and fatigue. The incidence of syncope and dyspnea and edema of lower extremity were 34. 6%,23. 1%. Accentuated pulmonic second sound(P2) was detected in 92. 3% of patients during physical examination, which was also the most common sign. About 50. 0% patients were functional class Ⅲ-Ⅳ. Echocardiography showed that all childrens′ mean pulmonary artery systolic pressure(PASP) was(79. 0 ± 20. 3) mmHg(1 mmHg=0. 133 kPa) and 16 of them with right ventricular dilatation. Electrocardiogram with ST-T changes and right ventricular high voltage were common. The average plasma level of brain natriuretic peptide(BNP) in all cases was(870. 4 ± 720. 9) pg/ml. The single factor analysis illustrated that syncope(OR=26. 25,95%CI 3. 04 -226. 60,P=0. 003),heart func-tional class Ⅲ-Ⅳ(OR=19. 199,95%CI 1. 88 -196. 53, P=0. 0128), PASP≥70 mmHg(OR =9. 936, 95%CI 1. 81-∞,P=0. 005),BNP≥850 pg/ml(OR=59. 991,95%CI 4. 69-767. 62,P=0. 002) indica-ted the worse outcome. Conclusion IPAH in children is rare and the prognosis is poor. Syncope, cardiac function grade,PASP and the levels of BNP are the main important factors affecting the prognosis of children with IPAH. BNP has important clinical significance for the evaluation of IPAH and the prognosis of the disease.
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Objective To analyze the clinical manifestations,diagnosis and treatment of idiopathic left ventricular tachycardia(ILVT)in infants and young children.Methods The clinical data,including manifestations,lab results and treatments of 37 cases of ILVT in infants and young children were analyzed retrospectively.Results ILVT were seen more in young children older than 1 year old and male infants.A total of 37 cases with ILVT showed diverse clinical manifestations,5 children had cardiac insufficiency with oliguria and edema,2 children had syncope.There were 8 children without any symptoms,and 22 children had some symptoms,which mainly included agitation and crying,weakness and sweaty,vomitting and pale com-plexion.Holter showed incessant ILVT in 20 cases(54.1%),and paroxysmal in 17 cases(45.9%).Of the 37 cases,9 cases presented tachycardia-induced cardiomyopathy(TIC),8 of them were secondary to the in-cessant ILVT.Children with TIC compared with those without TIC,the ventricular rate were faster[(206.2 ± 38.7)beats/min vs.(171.8 ±38.7)beats/min,P<0.05],the duration were longer[(22.6 ± 15.3)days vs. (9.8 ±6.0)days,P<0.05].A total of 34 cases were given by antiarrhythmic drugs,verapamil was higher effective in reversion to sinus rhythm.Radiofrequency ablations were conducted in 8 cases,resulting in ven-tricular tachycardia elimination.All of children were fine during following up periods.After successful control of tachycardia,left ventricular end-diastolic diameter and left ventricular ejection fraction in patients with TIC gradually recovered[(35.4 ± 7.2 )mm vs.(28.9 ± 5.6 )mm,P <0.05;(46.7 ± 4.0 )% vs.(70.1 ± 1 .5 )%,P<0.0 1 ] .Conclusion ILVT in infants and young children are rare,and present different manifes-tions.An incessant ILVT with faster ventricular rate and longer duration are more frequently complicated by TIC.Radiofrequency ablation is the effective way of radical cure of the disease.The diagnosis and treatment for the tachycardia are particularly important in early time,and the prognosis are good.
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Objective To report one case of the children's acute rhabdomyolysis(RM) with myocardial hypertrophy for the first time and the related data in order to improve the clinical diagnose and the treatment efficacy.Methods Clinical data of the patient with myocardial hypertrophy and pericardial effusion treated in the Heart Center of Beijing Children's Hospital Affiliated to Capital Medical University were retrospectively analyzed.According to the symptoms,signs,laboratory examination,the diagnosis was made and related literature was reviewed.Results A 7-years-old girl was characterized by myalgia,weakness,and dark urine.Physical examination showed extremity swelling,clammy skin,lower extremity muscle tenderness positive.Creatine kinase (CK) and myoglobin levels increased.So she was clearly diagnosed of acute RM undoubtedly.Without physical,metabolic and autoimmune factors,the patient was only had positive mycoplasma antibodies and might be coursed by mycoplasma infection.The muscular involvement was so severe that the patient had the heart failure,myocardial hypertrophy and pericardial effusion was conformed to ultrasound cardiogram(UCG).After treatment with hydration,alkalization,anti-infection,support and symptomatic therapy for a few days,the clinical symptoms improved obviously,CK returned to normal,myocardial hypertrophy and pericardial effusion disappeared.One-year-follow-up study indicated that the child's animal force and body movements were norrmal,and CK and UCG were negative.Conclusions Acute RM is a rare disorder and less recognized in children,and myocardial hypertrophy and pericardial effusion may be caused by acute RM.If the diagnosis and treatment are done earlier,the prognosis will be good,without permanent complications.