Coronary Aneurysms Research Articles

Coronary thrombosis and myocardial ischemia in Kawasaki disease: a case report

BackgroundCoronary artery thrombosis and myocardial ischemia caused by giant coronary aneurysms are the main causes of death in children with Kawasaki disease. The use of thrombolytic therapy in children with Kawasaki disease who have coronary thrombosis is a controversial topic, especially with respect to the timing of treatment.Case presentationIn this article, we report a case of a child aged two years and nine months with Kawasaki disease whose coronary arteries had no involvement in the acute phase. However, by only one week after discharge, the patient returned because we found giant coronary aneurysms complicated by thrombosis via echocardiography. Despite aggressive thrombolytic therapy, the child developed myocardial ischemia during thrombolytic therapy. Fortunately, because of timely treatment, the child’s thrombus has dissolved, and the myocardial ischemia has resolved.ConclusionsThis case suggests that for patients at high risk of coronary artery aneurysms, echocardiography may need to be reviewed earlier. Low-molecular-weight heparin should be added to antagonize the early procoagulant effects of warfarin when warfarin therapy is initiated. In the case of first-detected coronary thrombosis, aggressive thrombolytic therapy may be justified, particularly during the acute and subacute phases of the disease course.

Kawasaki Disease Diagnosis and Treatment in over 1000 Patients: A Continuum of Dysregulated Inflammatory Responses.

Kawasaki disease (KD) is a pediatric vasculitis, leading to coronary artery aneurysms (CAAs) in ~4-14%. Attention to the etiology and course of KD was generated by the close mimic of a SARS-CoV-2-induced phenotype, called multisystem inflammatory syndrome in children (MIS-C). A total of 1179 cases were collected from 2012 with ~50% of cases retrospectively included. Clinical characteristics were described and risk factors for CAA (persistence) were investigated. Phenotypic patterns of the prospectively included KD patients were evaluated. These patterns were also compared to the seronegative KD and seropositive MIS-C cases identified during the SARS-CoV-2 pandemic. KD mostly affected boys and children < 5 years. IVIG resistance, CAAs, and giant CAAs occurred in 24.5%, 21.4%, and 6.6%, respectively. Giant CAAs were significantly more likely to normalize to a normal Z score in patients that were younger than 2.5 years old at the time of initial giant CAA (χ2 test p = 0.02). In our prospective (SARS-CoV-2-seronegative) KD series, there was a diminishing male predominance over time, whereas the proportions of incomplete presentations (p < 0.001) and patients with circulatory shock (p = 0.04) increased since the COVID-19 pandemic. Pre- and post-pandemic KD cases presented with different levels of C-reactive protein, thrombocyte counts, and hemoglobin levels over the years. Compared to pandemic KD, SARS-CoV-2-seropositive MIS-C patients were older (p < 0.001), and more often required intensive care admission (p < 0.001), with a gradual decrease over time between 2020 and 2022 (p = 0.04). KD carried a substantial risk of CAA development in contrast to MIS-C. the phenotypic changes seen over the last twelve years of our prospective follow-up study suggest a spectrum of hyperinflammatory states with potentially different triggering events within this clinical entity.

Recurrence following percutaneous exclusion of giant coronary pseudoaneurysm: a case report

BackgroundEmergence of coronary giant pseudoaneurysm (PSA) after stent implantation is potentially catastrophic and may end up with life threatening complications if not managed promptly. There is scarcity of data in existing literature with respect to guidelines on the management of coronary PSA following stent implantation. We report the recurrence of coronary PSA following initial percutaneous management of a giant coronary PSA using multiple stent grafts.Case presentationA 38-year-old male who underwent primary angioplasty of the right coronary artery (RCA) about a month back, presented with dull aching precordial chest pain for the last 15 days. A repeat coronary angiography revealed giant coronary PSA in proximal to mid RCA. Considering the significantly large size of the coronary PSA with symptoms of impending rupture, the giant coronary PSA was successfully excluded by implanting three sequentially coronary stent grafts. However, after one and a half months, the patient again presented with a similar kind of dull aching chest pain. We found a recurrence of coronary PSA in a segment of the coronary artery distal to the portion excluded by stent grafts. This recurrent coronary PSA was once again successfully excluded by redeploying two more stent grafts with the help of a guide extension catheter.ConclusionsIn this case, vessel wall injury as a result of aggressive post dilatation using an oversized balloon during the index procedure was the contributor to the giant coronary PSA formation. It usually appears early after the index procedure (within 4 weeks). Though the usual strategy used to exclude coronary aneurysm is by using the minimal number of stent grafts (due to the inherent increased risk of restenosis/thrombosis in stent grafts) in post angioplasty traumatic aneurysm it is prudent to exclude the entire damaged artery by placing stent grafts to prevent recurrence in segments with even minimal dilatation on initial evaluation.

Coronary Artery Aneurysm or Ectasia as a Form of Coronary Artery Remodeling: Etiology, Pathogenesis, Diagnostics, Complications, and Treatment.

Coronary artery aneurysm or ectasia (CAAE) is a term that includes both coronary artery ectasia (CAE) and coronary artery aneurysm (CAA), despite distinct phenotypes and definitions. This anomaly can be found in 0.15-5.3% of coronary angiography. CAE is a diffuse dilatation of the coronary artery at least 1.5 times wider than the diameter of the normal coronary artery in a patient with a length of over 20 mm or greater than one-third of the vessel. CAE can be further subdivided into diffuse and focal dilations by the number and the length of the dilated vessels. Histologically, it presents with extensive destruction of musculoelastic elements, marked degradation of collagen and elastic fibers, and disruption of the elastic lamina. Conversely, CAA is a focal lesion manifesting as focal dilatation, which can be fusiform (if the longitudinal diameter is greater than the transverse) or saccular (if the longitudinal diameter is smaller than the transverse). Giant CAA is defined as a 4-fold enlargement of the vessel diameter and is observed in only 0.02% of patients after coronary. An aneurysmal lesion can be either single or multiple. It can be either a congenital or acquired phenomenon. The pathophysiological mechanisms responsible for the formation of CAAE are not well understood. Atherosclerosis is the most common etiology of CAAE in adults, while Kawasaki disease is the most common in children. Other etiological factors include systemic connective tissue diseases, infectious diseases, vasculitis, congenital anomalies, genetic factors, and idiopathic CAA. Invasive assessment of CAAE is based on coronary angiography. Coronary computed tomography (CT) is a noninvasive method that enables accurate evaluation of aneurysm size and location. The most common complications are coronary spasm, local thrombosis, distal embolization, coronary artery rupture, and compression of adjacent structures by giant coronary aneurysms. The approach to each patient with CAAE should depend on the severity of symptoms, anatomical structure, size, and location of the aneurysm. Treatment methods should be carefully considered to avoid possible complications of CAAE. Simultaneously, we should not unnecessarily expose the patient to the risk of intervention or surgical treatment. Patients can be offered conservative or invasive treatment. However, there are still numerous controversies and ambiguities regarding the etiology, prognosis, and treatment of patients with coronary artery aneurysms. This study summarizes the current knowledge about this disease's etiology, pathogenesis, and management.

A Giant Left Anterior Descending Artery Aneurysm and an Updated Review on Coronary Aneurysms

A Giant Left Anterior Descending Artery Aneurysm and an Updated Review on Coronary Aneurysms

Multiple giant coronary aneurysms: A rare form of coronary artery disease

Multiple giant coronary aneurysms: A rare form of coronary artery disease

Hospital Discharge Plan in Family Caregivers of Children with Coronary Artery Aneurysm in Kawasaki Disease: A Review

Objective: The study reviews status, main time nodes and hospital discharge services for family caregivers of children with Kawasaki disease complicated by coronary artery aneurysm to provide references for the development of hospital discharge preparation services for medical personnel and patients. Background: CAL of Kawasaki disease is the main cause of acquired heart disease in children, but there is not enough research on the readiness for hospital discharge. Design: Systematic review of observational and interventional studies.

Kawasaki Disease

Kawasaki disease (KD) is a type of acute inflammation of blood vessels, accompanied by a fever, for which the cause is yet unknown. It primarily affects children who are under the age of five. Kawasaki disease (KD) frequently results in the development of coronary artery lesions (CALs), which include dilated arteries, coronary artery aneurysms (CAAs), and potentially life-threatening myocardial infarction. under the age of five. Kawasaki disease (KD) frequently results in the development of coronary artery lesions (CALs). This case report aims to further explore the diagnosis and the management of KD. In this case report we discuss a 3-year-old male patient who was diagnosed with KD with the main complaints of fever, red eyes, rash, redness of lips with cracked and oral cavity. Patient’s lab work showed hypoalbuminemia, increased D-Dimer, and increased Troponin I. Electrocardiography and echocardiography was performed to observe cardiac complication. Soon aspirin and IVIG and showed an improvement. The child was treated for 6 days and then controlled at the outpatient clinic. Keywords: Kawasaki Disease, Pediatric, IVIG, Aspirin

A heavy ball: the rare giant right coronary artery aneurysm with thrombosis.

A heavy ball: the rare giant right coronary artery aneurysm with thrombosis.

Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome?: A case report and literature review.

Kawasaki disease (KD) is a vasculitis syndrome of small to medium-sized arteries that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. On rare occasions, it may accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). KDSS is a serious complication that can be presented to the emergency department as an initial feature when typical clinical symptoms of KD have not be detected. We report the case of a 12-year-old boy admitted with prolonged fever, bilateral non-purulent conjunctivitis, and signs of shock such as hypotension and tachycardia. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and sterile pyuria. He was initially treated with intravenous cefotaxime and vancomycin considering the possible diagnosis of toxic shock syndrome, while the treatment was not effective. Subsequent chest computerized tomography and ultrasound identified pulmonary consolidation and polyserous effusion. Echocardiography revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function. After a multidisciplinary consultation, a diagnosis of KDSS was made. To prevent coronary artery lesions and other severe complications, the patient immediately received immunoglobulin, corticoid, and acetylsalicylic acid. Soon afterwards, he showed significant improvement, with the temperature dropped to normal and hypotension corrected about 24 hours post-intravenous immunoglobulin therapy. Polyserous effusions also disappeared before discharge. Follow-up echocardiography revealed normal results. Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. For children with KD, any symptoms pointing to infection should be carefully considered. When there is no etiologic evidence, antibiotics should be used with caution. Our case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition, timely treatment, and close monitoring are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS.

Lymphocyte-C-reactive protein ratio combined with albumin upon admission predicts coronary artery dilation and aneurysm formation in pediatric patients with Kawasaki disease: a retrospective cohort study

ABSTRACT Background We aimed to explore simple and effective clinical parameters or combinations to predict coronary artery dilation and aneurysm formation in pediatric patients with Kawasaki disease (KD). Design and Methods This retrospective cohort study included pediatric patients with KD from January, 2013 to December, 2022. Multiple demographic and clinical data were collected, collated, and calculated from the medical records. Then they were divided into the coronary artery dilation and aneurysm formation group or the non-coronary artery dilation and aneurysm formation group. Lymphocyte-C-reactive protein ratio (LCR) was transformed into its natural logarithm and expressed as lnLCR. Results A total of 64 pediatric patients with KD were enrolled in this cohort study after 1:3 propensity score matching (PSM). For each unit increase in lnLCR, the possibility of coronary artery dilation and aneurysm formation decreased to 0.419 times the original value. The areas under the receiver operating characteristic (ROC) curves of lnLCR combined with albumin (ALB), ALB, and lnLCR to classify pediatric patients with KD into the coronary artery dilation and aneurysm formation group were 0.781, 0.692, and 0.743, respectively. Conclusion LCR combined with ALB upon admission is a promising predictor of coronary artery dilation and aneurysm formation in pediatric patients with KD.

Methylprednisolone pulse and prednisolone for intensification of primary treatment in Kawasaki disease patients at high risk of treatment resistance: a multicenter prospective cohort study.

The purpose of this study is to determine whether adding intravenous methylprednisolone pulse (IVMP) to primary adjunctive prednisolone with intravenous immunoglobulin (IVIG) improves treatment resistance and coronary artery aneurysms (CAA) in patients with Kawasaki disease (KD) with a high risk of treatment resistance. This multicenter, prospective, observational study was conducted at 28 hospitals in Japan from October 2016 to June 2020. For patients predicted to be resistant to treatment based on a Kobayashi score ≥ 5 and total bilirubin ≥ 1.0mg/dL, each hospital independently decided to add IVMP followed by prednisolone, prednisolone alone, or nothing to the primary IVIG therapy. In total, 2856 consecutive KD patients were enrolled; of these, 399 (14.0%) were predicted to be treatment resistant. Patients who were resistant to the primary treatment and required additional treatment comprised 59%, 20%, and 26% of the IVIG-alone group, IVIG-plus-prednisolone group, and IVIG-plus-IVMP group, respectively (P < .0001). The CAA incidence (Z score ≥ 2.5) at month 1 was similar among the treatment groups (6.7%, 4.8%, and 7.3%, respectively; P = .66). CAA occurred more frequently in patients who needed third- or later-line therapy.Conclusions: Primary adjunctive corticosteroid therapy improved the treatment response and suppressed inflammation. However, the study found no benefit of adding IVMP to prednisolone therapy. Patients receiving IVIG alone achieved coronary outcomes comparable to those of patients receiving primary adjunctive corticosteroid therapy although they were more likely to require additional rescue treatment. KD inflammation should be resolved no later than the third line of additional treatment to reduce the risk of CAA.Trial registration: University Hospital Medical Information Network Clinical Trials Registry in Japan ( https://www.umin.ac.jp/ctr/index.htm ) under code UMIN000024937.

Vasa vasorum enhancement on optical coherence tomography in Kawasaki disease.

Patients with Kawasaki disease (KD) prone to develop coronary artery aneurysm (CAA) with unknown etiology. We aimed to disclose the relationship between vasa vasorum (VV) and intimal thickening using optical coherence tomography (OCT) in KD. Forty-three coronary artery branches of 21 patients with KD were examined by OCT. The coronary arteries were classified into three groups: the CAA group (n = 9) in which CAAs remained since the acute phase, the regressed group (n = 16) in which CAAs were regressed, and the no CAA group (n = 18). The number and distribution of VV, and intimal thickening in coronary arteries were evaluated on OCT. Intimal thickening was significantly more severe in the CAA and regressed groups than in the no CAA group (median: 481, 474, and 218 μm, p = 0.001 and p < 0.001, respectively). The number of VV in the regressed group was significantly higher than that in the CAA and no CAA groups. The numbers of adventitial VV and internal VV were positively correlated with the intimal thickness (R = 0.64, p < 0.001; R = 0.62, p < 0.001, respectively). In the no CAA group, no internal VV were observed. VV enhances according to intimal thickening, suggesting that VV may have some link to the healing process, such as CAA regression and intimal thickening. Kawasaki disease (KD) is a vasculitis syndrome developing coronary artery aneurysm, however its etiology still remains unclear. Coronary artery imaging using optical coherence tomography (OCT) can reveal coronary arterial wall pathology, however OCT studies are limited in patients with KD. Using OCT, we disclosed the closed relationship between vasa vasorum enhancement and regressed coronary arterial lesions. Vasa vasorum enhancement is involved in the pathomechanism of the convalescent phase of KD.

Advancing Kawasaki Disease Research in the Arab World: Scoping Literature Review Analysis with Emphasis on Giant Coronary Aneurysms.

To evaluate giant aneurysms (GiAn) prevalence in Arab countries and examine contributing factors; and to review Kawasaki disease (KD) publication trends and collaborations among Arab nations. A scoping literature review was conducted to analyze the publications across the Arab world, spanning 16 countries from 1978 to 2023. The collected articles were a combination of database search with a call on Kawasaki Disease Arab Initiative (Kawarabi) members to share non-PubMed publications. Over 45years, 50 articles originated from the Arab Countries with a 30% average annual growth rate in KD research output. Publications were evenly split between case reports (42%) and institutional series (52%). Research productivity lagged in developing nations with UAE, KSA and Egypt, contributed to 64% of total publications. Among 26 institutional series, 256 coronary artery aneurysms (CAA) from a total of 1264 KD cases were reported. Of those, 25 CAA were GiAn (prevalence 1.43% [range 0-12.5%]). The initial KD misdiagnosis rate was 4%, and incomplete KD (iKD) averaged 10.6%. Series (38.5%) that did not report iKD correlated with a higher prevalence of CAA, but not of GiAn. Longer fever duration emerged as a pivotal factor for GiAn (OR 5.06, 95%CI 1.51-17). This review unveils the research landscape of KD in the Arab world over 45years. Initial misdiagnosis, untreated cases, delayed diagnosis and underreporting of iKD are contributing factors for an underestimated epidemiology, explaining the higher GiAn prevalence. This calls for strategic interventions to enhance KD research in these countries, aligning with Kawarabi's mission.

Meta-long Papyrus: Meta-analysis of mid to long-term outcomes of PK Papyrus covered stent.

Although covered stents (CS) represent a potentially life-saving intervention for coronary perforation (CAP), their application has expanded to other contexts, including coronary aneurysms (CAA). However, data regarding mid- and long-term outcomes of CS in these settings scenarios remains limited. This meta-analysis aims to evaluate major adverse cardiac events (MACE) from discharge through long-term follow-up in patients undergoing percutaneous coronary intervention with the new generation polyurethane-covered cobalt-chromium PK Papyrus CS. We conducted a meta-analysis of data from three observational trials that included long-term follow-up of patients who underwent PK Papyrus CS implantation: Papyrus-Spain, SOS PK Papyrus, and PAST-PERF registry. 332 patients underwent PK Papyrus CS implantation, 236 (71.1%) for CAP, 70 (21.1%) for CAA and 26 (7.8%) for other indications. After a mean follow-up of 16.2 months, the MACE was 14.3%, with Target Lesion Revascularization (TLR) being the most frequent (8.5%), followed by stent thrombosis (ST), 3.3% and cardiac death (CD), 2.6%. Comparing CAP and CAA subgroups, the MACE rate in CAA was significantly higher than CAP (21.4% vs 9.7%, p < 0.01), primary driven by ST (CAA: 8.6% vs CAP: 1.3%; p = 0.0015). The clinical outcomes following PK Papyrus CS implantation are deemed acceptable, considering the challenging scenarios and the existing alternative treatments. However, MACE rates in patients with CAA who received Papyrus PK CS were significantly higher than in those with CAP, underscoring the importance of meticulous patient selection and optimization of CS in these complex patients and coronary anatomies.

Cardiovascular manifestations in pediatric multisystem inflammatory syndrome associated with COVID-19 in a tertiary care pediatric center in Mexico City

The objective is to expose the cardiovascular alterations in patients diagnosed with pediatric inflammatory multisystem syndrome (PIMS) associated with COVID-19 during the SARS-CoV-2 pandemic, in order to understand the disease, its evolution, and optimal management upon diagnosis. Retrospective, observational, cross-sectional analytical study of patients diagnosed with PIMS according to the criteria of the World Health Organization at the National Institute of Pediatrics, from March 2020 to December 2021. During the study period, 77 patients with PIMS were diagnosed. The results showed correlation between the shock state and alteration of laboratory markers (platelets 144217.29 ± 139321.6 μL [p < 0.001], procalcitonin 27.37 ± 38.37 ng/ml [p = 0.05] and ferritin 1937.87 ± 2562.63 [p < 0.001]). The ventricular function in patients with shock was significantly lower compared to those without shock (49.6 ± 9.1% vs. 58.1 ± 8.4 %; t-Student p < 0.001), as well as injury to the left coronary artery (p = 0.02). There is a correlation between NT-proBNP and ventricular dysfunction (Kruskal-Wallis p = 0.007). Statistical significance was found in the association between death, elevation of inflammatory markers and ventricular dysfunction (p < 0.001). The cardiovascular alterations observed, in order of frequency, were pericardial effusion (25.7%), myocarditis (15%), mild ventricular dysfunction (13.5%) and small coronary aneurysm with predominance of the left coronary artery and the anterior descending one.

Etiology and prognosis of non-Kawasaki disease induced coronary aneurysms in children: a retrospective case series study

While Kawasaki disease (KD) induced coronary artery aneurysms (KD CAAs) in children are well studied, the features and prognosis of non-KD induced CAAs (non-KD CAAs) in the pediatric population are poorly documented. This case series study is to analyze the etiology and prognosis of non-KD CAAs in children and compare the characteristics of non-KD CAAs and KD CAAs. Non-KD CAA and KD CAA cases at our department from January 2022 to December 2023 were retrospectively collected. Etiologies and prognosis of non-KD CAAs were analyzed. Furthermore, demographic data, biochemical parameters and outcomes between children with Non-KD CAAs and children with KD CAAs were comparatively studied. Fifteen children with non-KD CAAs with a median age of 6 years and 117 children with KD CAAs with a median age of 2.0 years (p = 0.022) were included in this study. The causes of non-KD CAAs include: unknown etiologies (2 cases), coronary artery structural abnormalities (4), Takayasu arteritis (2), virus infection (2), cardiomyopathy (2), aplastic anemia with agranulocytosis (1), ANCA-associated vasculitis (1), and mucopolysaccharidosis (1). In the non-KD CAA group, there were a total of 19 CAAs with 3 being giant, 5 medium, and 11 small; 4 patients had complete CAA regression; an infant with a fistula between the right coronary artery and the coronary sinus complicated with cardiac enlargement died of heart failure. The KD group had significantly higher levels of CRP, white cells counts and ESR with zero mortality. Non-KD CAA cases had a significantly lower regression rate than KD-CAA cases (26.7% vs 66.7%, p = 0.004), and the probability of CAA regression in non-KD patients was 0.341 of that in KD patients (p = 0.006, OR = 0.341, 95% CI: 0.179–0.647).Conclusions: Various etiologies for Non-KD CAAs are identified. Patients with Non-KD CAAs were observed to have lower inflammatory indexes but poorer recovery than patients with KD CAAs. Therapeutic strategies different than those for KD may be needed for non-KD CAAs.What is Known:• Coronary artery aneurysm (CAA) in children is most commonly induced by Kawasaki disease (KD CAA), with a 50 ~ 70% regression rate in 1 to 2 years.• CAA induced by diseases other than KD (non-KD CAA) in children is rare and its prognosis remains largely unknown.What is New:• Most non-KD CAA cases are caused by coronary artery structural malformations.• Non-KD CAA in children has poorer prognosis and lower regression rate compared with KD CAA.• In addition to guideline directed anti-platelet and anti-coagulant therapies, treatments targeting the causal factor are necessary for non-KD CAA.

Phenotypic and mutational spectrum of 17 Chinese patients with Menkes Disease.

Menkes Disease (MD) is a fatal X-linked recessive disorder caused by mutations in the ATP7A gene. Severe cases typically die before the age of three. Mild MD and occipital horn syndrome are variants of MD characterized by a less severe phenotype and longer survival. This case series aims to validate previous findings, expand the clinical phenotype, identify novel ATP7A mutations of MD patients. Observational data with follow-up were collected from 17 genetically diagnosed Chinese MD patients. All 17 patients exhibited neurological symptoms, including delayed motor milestones (100%) and seizures (58.8%). Unspecific pregnancy or delivery complications occurred in 9 patients (52.9%). The most prevalent connective tissue problems were abnormal hair (76.5%), followed by skeletal and dental abnormalities (52.9%), skin problems (41.2%) and hernia (35.3%). Sensorineural hearing loss (17.6%) was previously unreported. Coronary artery aneurysm and patent foramen ovale (5.9%) were infrequent. One 16-year-old boy carries pathological exon 3-4 deletion, presents novel mild phenotype including short stature and cerebellar ataxia. Out of 13 patients with follow-up (median: 24 months), 7 patients (53.8%) died with median survival of 40 months (range: 21-48 months), 3 patients (23.1%) show severe motor development delay and 2 (15.4%) have refractory epilepsy, only the mild MD patient shows improved cerebellar ataxia. Sixteen ATP7A mutations were identified including 6 small indels (37.5%), 5 nonsense mutations (31.2%), 2 missense mutations (12.5%), 2 exon deletions (12.5%), and 1 splice site mutation (6.25%). Fourteen mutations were novel. Our study further broadens the phenotypic and genotypic spectrums of Menkes disease.

Clinico-Laboratory Profile and Outcome of Kawasaki Disease in Children in a Tertiary Care Hospital in Kathmandu

Introduction: Kawasaki disease (KD) is one of the most common vasculitis of childhood. It contributes significantly to acquired heart disease in children and results in coronary artery abnormalities including aneurysm in 25% of untreated children. This study aims to retrospectively assess the clinical course, laboratory results, and presenting symptoms of patients with complete and incomplete KD treated at tertiary care hospital in Kathmandu.Method: This retrospective study was conducted in the Department of Pediatrics. Data from January 2020 to December 2020 were retrieved from medical records department of the hospital and analyzed using Microsoft excel.Result: The total number of cases evaluated in our study was 9. The median age at the time of the diagnosis of KD was 24 months (median age for incomplete KD-21 months, complete KD-37 months). Male to female ratio was 1.2:1. The peak season of KD occurrence was Spring (March – May). Mean duration of stay in the hospital was 7 days. All children presented with fever, with an mean fever duration of 7 days at the time of presentation. Fever was present in every case. The most common principal symptom was a polymorphous skin rash, observed in 88% of the cases. Elevated CRP levels (≥3 mg/dl) were noted in all 9 cases. Cardiovascular complications were present in 88% of the patients, with coronary artery aneurysm (CAA) detected in 11%, coronary artery dilation in 22%, and mild pericardial effusion documented in 66% of the cases. Eight patients (88%) received IVIG, all 9 patients (100%) received aspirin, and 2 patients received steroids. There were no fatalities.Conclusion: KD is a common vasculitis in children which do not have significant sex predilection in oppose to most of the studies. Majority of cases occurred in spring season. The most common symptom was polymorphous rash. A high level of suspicion for diagnosing KD is essential, and early treatment is crucial to prevent complications and reduce the future economic burden on families and the nation.

The Effectiveness of No or Low-Dose versus High-Dose Aspirin in Treating Acute Kawasaki Disease: A Systematic Review and Meta-Analysis.

This systematic review and meta-analysis assesses the effectiveness of no or low-dose versus high-dose aspirin on the incidence of coronary artery aneurysms (CAAs), intravenous immunoglobulin (IVIG) resistance, hospital stay length, and fever duration during the acute phase of Kawasaki disease. Our review adheres to the Preferred Reporting Items for Systematic Reviews guidelines. The PubMed and Google Scholar databases were comprehensively searched to identify relevant studies in the literature, including observational studies and randomized controlled trials (RCTs). The primary outcome was the incidence of CAAs. The secondary outcomes were the hospital stay length, fever duration, and IVIG resistance. The risk of bias was assessed using the Newcastle-Ottawa scale for cohort studies and Cochrane's Risk of Bias Tool for RCTs. The data were analyzed using the Review Manager software. Twelve studies with a total of 68,495 participants met the inclusion criteria. The incidences of CAAs (odds ratio [OR] = 0.93; 95% confidence interval [CI] = 0.64-1.34) and IVIG resistance (OR = 1.46; 95% CI = 1.00-2.12) did not differ significantly between no or low-dose versus high-dose aspirin in treating acute KD. Moreover, the fever durations (mean difference [MD] = 3.55 h; 95% CI = -7.99-15.10) and hospital stay lengths (MD = -0.54 days; 95% CI = -2.50-1.41) were similar in the no and low-dose aspirin group compared to the high-dose aspirin group. Our review indicates that there are no significant differences in the incidences of CAA and IVIG resistance, fever durations, and hospital stay lengths between no or low-dose versus high-dose aspirin in treating the acute phase of KD.