Artery Aneurysms In Children Research Articles

Determination of Serum Procalcitonin Level in Kawasaki Disease

Background: The most frequent cause of coronary artery aneurysm in children is Kawasaki disease (KD). Recently, limited studies on procalcitonin (PCT) were performed to find a biomarker for the diagnosis or prognosis of children with KD. Objectives: This study aimed to compare serum PCT levels between complete and incomplete KD and testify to the predictive validity of PCT for intravenous immunoglobulin (IVIG) resistance and predicting coronary artery lesions (CALs). Methods: This cross-sectional study was conducted at Namazi hospital in Shiraz, Iran, in 2019. All KD patients admitted to the hospital were included, with parental consent obtained. Kawasaki disease patients were categorized as complete KD (cKD) or incomplete KD (iKD). Two-dimensional echocardiography was performed, and peripheral venous blood was examined for PCT levels and other markers. All patients received IVIG and aspirin as standard treatment. The presence of coronary artery abnormalities (CAAs) was determined based on coronary artery size and morphology. We used Stata software version 14.0 for data analysis. Logistic regression models identified predictive factors for coronary complications. This study determined the optimal PCT cut-off point for predicted coronary complications using receiver operating characteristic (ROC) curve analysis. Approval was obtained from the Research Ethics Committees of Shiraz University of Medical Sciences. Results: Procalcitonin values in 38 children hospitalized with acute KD ranged from 0.2 ng/mL to 10 ng/mL, with a mean of 2.65 ng/m. There was no correlation of PCT with patients’ age or gender, incomplete KD, or IVIG resistance. The serum PCT concentration was higher in patients with CAL (P = 0.009). The best PCT cut-off value for CAL prediction was 2.5 ng/mL (corresponding sensitivity = 81.8% and specificity = 68.7%), considering CAL prevalence as the studied group. Conclusions: Supplementary research is needed to determine the sensitivity and specificity of PCT for the diagnosis and prognosis of KD. Procalcitonin might be of value in predicting which children are at increased risk for CALs to intensify therapy.

EE61 Cost Effectiveness of Treating Ivig-Resistant Kawasaki Disease Children with Second Round IVIG Versus Infliximab

Intravenous immunoglobulin (IVIG) is a known effective treatment to reduce the incidence of coronary artery aneurysms in children with Kawasaki Disease (KD). However, there are IVIG-resistant patients and a lack of clinical trial data to determine the best second round treatment. Using recent effectiveness findings from the KIDCARE study, this research aimed to determine if second round IVIG or infliximab is cost effective in KD patients with persistent or recrudescent fever.

The Negative Predictive Value of Harada Scoring for Coronary Artery Dilatation or Aneurysm in Children with Kawasaki Disease: A Cross-sectional Study.

Background: The most common cause of coronary artery aneurysms in children is Kawasaki disease (KD). One of the risk ratings used to predict coronary artery aneurysms is the Harada score. This study aims to assess the negative predictive value (NPV) of Harada scoring in children with KD.Methods: This cross-sectional study was conducted in Mashhad (Iran) from January 2014 to December 2017. The Harada score was generated for each patient using demographic, laboratory, and echocardiography data retrieved from their medical records. The patients were divided into typical and atypical cases, and the sensitivity, specificity, positive predictive value (PPV), and NPV were calculated. The Chi square test, independent t test, Mann–Whitney U test, and Fisher exact test were used to analyze the data in SPSS software (version 23.0). P≤0.05 was considered statistically significant.Results: The study involved 168 individuals with a mean age of 29.91±19.52 months, including 103 typical and 65 atypical cases. Regarding cardiac complications, the atypical group had a significantly greater rate of coronary artery tapering (P=0.030). Out of 101 patients with cardiac complications, the Harada score was positive in 57 cases, while it was falsely negative in the remaining 44 patients. The calculated sensitivity, specificity, PPV, and NPV were 56.44%, 53.73%, 64.77%, and 45%, respectively.Conclusion: The findings revealed that the Harada scoring is ineffective in detecting coronary artery aneurysms or dilation in KD patients.

Risk Factors for Coronary Artery Aneurysm in Children with Kawasaki Disease: A Case-control Study

Coronary artery aneurysms (CAA) are common in children with Kawasaki disease (KD) who have delayed diagnosis. This can increase the high cardiovascular burden and cause acquired heart defects in adulthood. This study aims to investigate the risk factors for CAA in children with KD. An observational retrospective case-control study was carried out in Surabaya Tertiary Hospital. We collected the medical records in the child care installation, Pediatrics ward, from 2016 to 2019. The research participants were pediatric patients who were treated with KD. The exclusion criteria were incomplete medical record data regarding the risk factors studied. The risk factors studied included age, sex, duration of fever, haemoglobin, leukocytes levels, platelets levels, C-reactive protein (CRP), and albumin levels. Twenty-eight medical records of children with KD were collected in this study; only 17 eligible patients were observed. The confirmed CAA was found in 8 patients, while 9 patients were non-CAA aged ranging from 3-124 months. Almost 60% of patients had fever duration > 7 days, and 47.1% of patients had hemoglobin <10 g/dL. Platelets were the only risk factor that had a significant correlation for developing CAA in children with KD, with a p-value of 0.015 at a 95% confidence interval (CI), odds ratio (OR) of 24 (1.785 – 336.227). Almost half of KD patients in this study were at risk for developing CAA; platelet value was a risk factor that we could consider in the administration of aspirin and IVIG therapy, providing the follow-up results and evaluating aneurysm progression or regression after IVIG administration is needed to prevent poor long-term outcomes.

Prediction of Coronary Artery Aneurysms in Children With Kawasaki Disease Before Starting Initial Treatment.

Background: Precisely predicting coronary artery aneurysms (CAAs) remains a clinical challenge. We aimed to investigate whether coronary dimensions adjusted for body surface area (Z scores) on baseline echocardiography and clinical variables before primary treatment could predict the presence of late CAAs.Methods: We conducted a retrospective study including children hospitalized for Kawasaki disease and received intravenous immunoglobulin within 10 days of illness. We defined late CAAs as a maximum Z score (Zmax) ≥2.5 of the left main, right, or left anterior descending coronary artery at 11–60 days of illness. Associations between late CAAs and clinical parameters and baseline maximum Z scores were analyzed.Results: Among the 314 included children, 31 (9.9%) had late CAAs. Male, higher C-reactive protein, and higher baseline Zmax were risk factors of late CAAs. Late CAAs were significantly associated with baseline Zmax ≥2.0 vs. <2.0 (25 [32.5%] vs. 6 [2.5%], P < 0.001). The odds ratio for late CAAs among the patients with baseline Zmax ≥2.0 vs. <2.0 was 18.5 (95% confidence interval, 7.23 to 47.41, P < 0.001). The sensitivity, specificity, positive predictive value, and negative predictive value of baseline Zmax ≥2.0 for the presence of later CAAs were 80.6, 81.6, 32.5, and 97.5%, respectively.Conclusions: Findings from this study suggest that Zmax ≥2.0 of coronary arteries on baseline echocardiography may be used to predict children at a high risk of late CAAs and allow for targeted early intensification therapy.

Coronary artery aneurysms in children is not always Kawasaki disease: a case report on Takayasu arteritis

BackgroundCoronary artery (CA) aneurysms in children are a rare but potentially life-threatening finding and are highly associated with Kawasaki disease (KD).Case presentationWe describe a four-year-old female with a vasculitis and CA aneurysms. She had a prolonged course with recurrent fever and systemic inflammation several times upon discontinuation of steroid treatment. Due in part to the CA aneurysms, she initially was diagnosed with KD but due to the unusual clinical course, further evaluation was performed. Abdominal and chest MRI/A revealed diffuse aortitis suggestive of a large vessel vasculitis, specifically Takayasu arteritis. With treatment targeted for Takayasu arteritis, there was resolution of fever and inflammation and the CA aneurysms improved.ConclusionsThis case demonstrates the utility in broadening the differential diagnosis in cases of presumed KD with CA involvement in which the clinical course is atypical for KD.

The relationship between TNF-like protein 1A and coronary artery aneurysms in children with Kawasaki disease.

Kawasaki disease (KD) is an acute, systemic vasculitis of unknown etiology that occurs predominantly in infants and children, and the most crucial complication of KD is coronary artery aneurysm (CAA). Tumor necrosis factor (TNF)-like protein 1A (TL1A) is a member of the TNF superfamily, which possesses the ability of maintaining vascular homeostasis and regulating immune responses. This study aimed to examine serum TL1A levels in KD patients, and to investigate the relationship between TL1A and CAAs in children with KD. Blood samples were recruited from 119 KD patients, 35 febrile controls (FCs), and 37 healthy controls (HCs). The KD group was further divided into KD with CAAs (KD-CAAs) and KD non-CAAs (KD-NCAAs) groups. Serum TL1A levels were measured using enzyme-linked immunosorbent assays, and clinical parameters were collected from KD patients. Serum TL1A levels of KD patients in the acute phase of KD were significantly higher than in the FC and HC groups. In particular, serum TL1A levels were substantially increased in the KD-CAA group compared with the KD-NCAA group. Furthermore, TL1A levels in the KD group were positively correlated with the duration of fever and the time point of IVIG and WBC levels, but negatively correlated with levels of RBC, Hb and albumin. TL1A might be involved in KD-associated vasculitis and in the development of CAAs.

Association between Mycoplasma pneumoniae Infection and Coronary Artery Aneurysm in Children with Kawasaki Disease

Background: The epidemiology of Kawasaki disease (KD) suggests that an infectious agent may be a potential disease trigger in susceptible children. Several studies have shown that the cause of KD may be associated with Mycoplasma pneumoniae (M. pneumoniae) infection. Objectives: We aimed to investigate the relationship between M. pneumoniae infection and coronary artery aneurysm (CAA) in children with KD in China. Methods: From January 2015 to December 2018, a total of 330 children with KD met the inclusion criteria. Relevant data were extracted and analysed. Results: The children were stratified into two groups according to M. pneumoniae infection status. Significant differences were identified in the proportion of patients with fever &gt; 10 days, the occurrence of small CAA, and the average serum sodium, pre-albumin (PA), and albumin levels but not in the occurrence of medium and giant CAA between the two groups. According to binary logistic regression, M. pneumoniae infection (OR: 0.515; 95% CI: 0.309 - 0.860; P = 0.011), serum sodium levels (OR: 0.910; 95% CI: 0.851 - 0.972; P =0.005), and PA (OR: 0.900; 95% CI: 0.854 - 0.949; P ≤ 0.001) levels were independently associated with occurrence of small CAA. Conclusions: We demonstrated that M. pneumoniae infection, serum sodium and PA levels are inversely related to the occurrence of small CAA. These results suggest that M. pneumoniae infection may be associated with a decreased incidence of small CAA. Further large-sample studies are needed.

Risk for Systemic Artery Aneurysm in Children with Kawasaki Disease

In Kawasaki disease (KD), coronary artery aneurysm (CAA) is the most commonly recognized complication. The incidence of systemic artery aneurysm (SAA)

MiR-222-3p in Platelets Serves as a Distinguishing Marker for Early Recognition of Kawasaki Disease.

Kawasaki disease (KD) is an acute vasculitis, which leads to 20% of sufferers developing coronary artery aneurysm in children if not appropriately treated. Therefore, the early diagnosis of KD is essential for alleviating the risk of developing heart disease. MicroRNAs (miRNAs) are a large class of small non-coding RNAs which post-transcriptionally regulate gene expression and have been shown to play critical roles in numerous biological processes and diseases. In this study, we used high-throughput miRNA sequencing and found dozens of miRNAs are highly expressed in platelets. By comparing the miRNA expression profile of platelets of acute KD patients and other febrile patients, miR-222-3p is validated to be significantly upregulated in platelets of acute KD patients. Furthermore, KEGG pathway analysis shows that targets of miR-222-3p are enriched in immune-related signaling pathways. Our study uncovers the potential of miR-222-3p in platelets as biomarker for early diagnosis of Kawasaki disease.

Treatment and follow-up of congenital coronary artery fistula with giant coronary artery aneurysm in children

Objective To analyze the treatment and follow-up of congenital coronary artery fistula (CAF) with giant coronary artery aneurysm (GCAA) in children. Methods The clinical data were analyzed retrospectively in 13 patients who were diagnosed as congenital CAF with GCAA between July 2009 and December 2016 in Guangzhou Women and Children′s Medical Center.There were 8 boys and 5 girls.The median age was 18 months, ranging from 40 days to 12 years old.The body weight ranged from 3.8 kg to 29.0 kg with a median of 8.8 kg.Fistulas originated from right coronary artery accounted in 8 patients, with 5 from left coronary artery.Fistulas drained into right atrium in 3 patients, right ventricular in 8 patients and left ventricular in 2 patients.Single fistula occurred in 12 patients and multiple fistulas in 1 patient.The diameter of coronary artery aneurysm ranged from 8 mm to 16 mm with a median of 9.2 mm. Results One patient had tachypnea and growth retardation without heart murmur.The other 12 patients were asymptomatic with heart murmurs occasionally found in routine physical examination.One patient underwent fistula ligation without cardiopulmonary bypass (CPB). The remaining 12 cases received fistula correction with beating heart CPB.Direct suture was used in 10 patients and autologous pericardial patch in other 2 patients.Two patients were associated with atrial septal defect (ASD) and underwent repair of ASD concurrently.The coronary artery aneurysm remained ori-ginal shape without any intervention during the operation.The mean hospital delay was (11.0±2.5) days.Two patients had decreased ejection fraction as low as 38% within 3 days after the operation, but went up to over 50% in follow-up 1 month later.Transient T wave change occurred in 7 patients, and another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.All 13 patients had antiplatelet therapy with 12 taking Aspirin and one taking Dipyridamole.The dosage was 3-5 mg/(kg·d) with duration ranging from 3 days to 13 months with a median of 1 month.During the perioperative period and the follow-up period (3 months to 8 years), all patients were asymptomatically alive.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status, the diameter of dilated coronary arteries was not changed after the operation.There was no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia. Conclusions GCAA can be combined with congenital CAF in children, so it needs early operation.The evidence-based intervention of coronary artery aneurysm and usage of anticoagulant and antiplatelet therapy in pediatric patients was still lacking, which needs long-term follow-up. Key words: Coronary artery fistula; Child; Giant coronary artery aneurysm; Antiplatelet; Follow-up

Transcatheter Closure of Congenital Coronary Artery Fistulas with a Giant Coronary Artery Aneurysm in Children: Experiences from a Single Center.

Background:Transcatheter closure of congenital coronary artery fistulas (CCAFs) is an alternative therapy to surgery; however, data regarding transcatheter closure for CCAF with a giant coronary artery aneurysm (CAA) in pediatric patients are still limited due to the rarity of the disease. We aimed to evaluate the efficacy and safety of transcatheter closure for CCAF with a giant CAA in a pediatric population at a single center.Methods:Medical records of pediatric patients (<18 years old) who underwent transcatheter closure of CCAF with a giant CAA between April 2007 and September 2016 at Guangdong Cardiovascular Institute (Guangdong, China) were reviewed.Results:Twelve patients (median age, 6.1 years; range, 1.9–11.0 years) underwent successful transcatheter closure procedures. One patient underwent closure at both the entry and exit points of the CAA, three patients underwent closure at the exit point of the CAA, and eight patients underwent closure at the entry point of the CAA. After a mean follow-up of 7.2 years (range, 0.5–9.8 years), one patient (with closure at the exit point of the CAA) underwent transcatheter re-intervention because of a significant residual shunt. She eventually underwent a surgical procedure due to aneurysm dilation after the second intervention. One patient experienced thrombus formation within the CAA after the procedure. Among those with closure at the entry point of the CAA, a mild-to-moderate residual shunt was detected in three patients.Conclusions:Transcatheter closure appears to be a safe and effective alternative therapy for CCAF with a giant CAA in the pediatric population. Closure at the entry point of the CAA, and closure at both the entry and exit points when feasible, may reduce the risk of postinterventional complications.

Risk factors for coronary artery aneurysms in children with Kawasaki disease

Objective To explore risk factors for coronary artery aneurysms (CAA) in children with Kawasaki disease (KD), and reduce the rate of serious sequelae of cardiovascular diseases. Methods All the patients were diagnosed as KD in Children's Hospital Affiliated to Medical College of Zhejiang University from January 2009 to January 2014. A total of 679 cases was included, and 42 with concurrent CAA, 181 with concurrent coronary artery expansion (CAD), and 456 without coronary artery damage cases (non-coronary artery lesion, NCAL). Coronary artery damage was related to factors such as gender, age, fever, white blood cell count (WBC), hemoglobin (Hb), platelet count (PLT), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), alanine aminotransferase, serum potassium, plasma amino terminal brain natriuretic peptide precursor (NT-proBNP), and acrylic ball resistance. SPSS 18.0 software package was used for risk factor analysis. Qualitative data using chi-square test, was used to analyze the high risk factors of CAA group, and logistic multivariate regression analysis was also used. Results Chi-square test showed that male, with febrile days >14 d, NT-proBNP ≥ 1 000 ng/L, immunoglobulin resistance was more likely to have KD concurrent CAA (P 14 d [OR=12.436, 95% CI (4.599, 33.631), P=0.000], NT-proBNP≥1 000 ng/L [OR=3.305, 95% CI (1.401, 7.794), P=0.005], and immunoglobulin resistance [OR=3.842, 95% CI (1.562, 9.453), P=0.000] were independent risk factors for KD concurrent CAA. Conclusions Male children, febrile days >14 d, NT-proBNP≥1 000 ng/L, and immunoglobulin resistance were independent risk factors of CAA. Key words: Mucocutaneous lymph node syndrome/CO; Coronary aneurysm/CO; Risk factors

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Giant Coronary Artery Aneurysms Associated with Kawasaki Disease Detected on Whole-heart Magnetic Resonance Coronary Angiographic Screening

Kawasaki disease (KD) is one of the most important causes of coronary artery aneurysms in children and young adults. However, the natural course of the disease and the patient prognosis remain obscure. A 72-year-old asymptomatic man with undiagnosed KD underwent whole-heart magnetic resonance coronary angiography during a health checkup. The imaging disclosed giant aneurysms in the proximal portion of the right coronary artery and the left anterior descending artery. The patient was successfully treated with coronary artery bypass grafting. The present case suggests that there may be a substantial number of patients who have attained middle to old age with undiagnosed KD.

Relationship between adipokines and coronary artery aneurysm in children with Kawasaki disease

Body fat is an important source of adipokines not only in association with energy balance, but also with inflammatory and immune responses. This study investigated the relationship between serum levels of adipokines and coronary artery aneurysm in patients with Kawasaki disease (KD). Levels of leptin, adiponectin, and resistin were measured in 165 cases, including 4 groups: the control group (n = 85), KD with normal coronary arteries (n = 41), KD with dilatation and/or ectasia (n = 31), and KD with coronary aneurysm (n = 8). White blood cells counts (WBC), red blood cells counts (RBC), hemoglobin (HB), Hematocrit (Hct), platelet count, C reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were tested in children. Levels of adiponectin and resistin levels were significantly elevated; hemoglobin significantly decreased in the group of KD with coronary aneurysm compared with the controls or other KD subgroups. There were markedly positive relationships between levels of resistin and CRP, and negative relationships between levels of resistin and RBC in patients with KD. Levels of adiponectin, resistin, and hemoglobin were associated with the development of coronary aneurysm in children with KD. The up-regulation of resistin secreted from adipose tissue may be closely linked toup-regulation of systemic proinflammatory markers in acute KD.

Congenital hepatic artery aneurysm: A case report in early infancy

Hepatic artery aneurysm in children is a very unusual pathologic entity. As most patients with this anomaly are asymptomatic, the diagnosis is usually made as an incidental finding on imaging studies performed for other reasons. Here, we report a 35-day-old infant with hepatic artery aneurysm, which was diagnosed accidentally as a liver mass during echocardiography. Considering the age of diagnosis, she most likely had a congenital type of hepatic artery aneurysm. To the best of our knowledge, this is the youngest case to be reported in the literature.

Middle cerebral artery aneurysms in children: case series and review

Pediatric intracranial aneurysms are rare lesions that differ from their adult counterparts. Aneurysms involving the middle cerebral artery (MCA) are particularly challenging to treat in children, as they are often fusiform and cannot undergo direct clipping alone. The authors recently treated a patient with a heavily calcified, dysplastic, left-sided MCA aneurysm. The present study was performed to evaluate the authors' previous operative and follow-up experience with these difficult lesions. The authors performed a review of a prospectively maintained database of all aneurysms treated at Methodist Hospital in Indianapolis, Indiana, from January 1990 through November 2010. Relevant operative notes, clinical charts, and radiological reports were reviewed for all patients 18 years of age or younger. A total of 2949 patients with aneurysms were treated over the study period, including 28 children (0.95%). Seven children harbored MCA aneurysms. Five of these 7 aneurysms (71.4%) were fusiform. Two patients were treated with direct clipping, 2 underwent parent vessel occlusion without bypass, and 3 underwent aneurysm trapping with extracranial-intracranial vessel bypass. Long-term follow-up data were available in 6 cases. All 6 patients had a 1-year follow-up Glasgow Outcome Scale score of 5. Long-term radiological follow-up was available in 4 patients. One patient required a reoperation for a recurrent aneurysm 4 years after the initial surgery. Middle cerebral artery aneurysms in children are often fusiform, giant, and incorporate the origins of proximal artery branches. Direct clipping may not be possible; trapping of the lesion may be required. Children seem to tolerate surgical trapping with or without bypass extremely well. Aggressive therapy of these rare lesions in children is warranted, as even patients presenting with a poor clinical grade may have excellent outcomes. Long-term surveillance imaging is necessary because of the risk of aneurysm recurrence.

Congenital-idiopathic superficial femoral artery aneurysm in a 7-year-old child

Superficial femoral artery aneurysm in children is distinctly uncommon, and usually results from infection, vasculitis, connective tissue disorder, or trauma. We report a 7-year-old girl who had multiple fusiform aneurysms of the right superficial femoral artery, with no evidence of related disorders. The patient successfully underwent aneurysm resection and femoral artery reconstruction with autogenous saphenous vein. Histologic examination revealed intimal thickening with fibroplasia without severe inflammatory infiltrates or cystic medial necrosis, suggesting a congenital-idiopathic arterial aneurysm. Three years after the procedure, the saphenous vein graft is fully patent and the patient is in good condition.

Single nucleotide polymorphism rs2229634 in the ITPR3 gene is associated with the risk of developing coronary artery aneurysm in children with Kawasaki disease

Kawasaki disease (KD) is the most common form of pediatric vasculitis. Though its etiology is unknown, researchers have suggested that it is related to genetics. The inositol 1,4,5-triphosphate receptor type 3 (ITPR3) gene has a strong association with the development of type 1 diabetes and, plays a critical role in the development of autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, and Graves' disease. The aim of study is to examine the association of ITPR3 polymorphisms with KD risk in Taiwanese children. This study evaluates the single nucleotide polymorphisms (SNP) rs2229634 in the ITPR3 gene with KD in a case-control study involving 93 KD patients and 680 healthy, gender- and age-matched controls. The frequency of the rs2229634 T/T genotype was significantly higher in KD patients with coronary artery aneurysm (CAA) than in patients without CAA [odds ratio (OR) = 2.56, 95% confidence interval (95% CI) = 1.35-4.88, P = 0.004]. In addition, KD patients with the T/T genotype elevated mean serum levels of C-reactive protein compared with patients with the C/C or C/T genotype (12.2 mg dL(-1) vs. 8.5 mg dL(-1) , P = 0.036). In conclusion, the results of this study suggest that the rs2229634 SNP in the ITPR3 gene is associated with the risk of CAA formation in Taiwanese KD patients.