The incidence of periungual desquamation and thrombocytosis in Kawasaki disease and the importance of systematic observation in the subacute phase.

Background: Periungual desquamation and thrombocytosis are characteristic of the subacute phase of Kawasaki disease (KD). However, accurate observations of periungual desquamation and thrombocytosis are lacking. Methods: This retrospective study included patients with acute-phase KD who received treatment at seven affiliated university hospitals in Korea between 2015 and 2017. Data were extracted from an anonymized registry established by the Korean Society of Kawasaki Disease. We investigated whether the findings of patients observed according to a set protocol until the subacute stage (group I) were different from those of patients observed without the use of a protocol (group II). Results: A total of 879 patients with KD were included in the analysis. Periungual desquamation was observed in 85% and 12.7% of patients in groups I and II, respectively. Thrombocytosis was observed in 76.7% and 44.7% of patients in groups I and II, respectively. Furthermore, compared to the initial test, the platelet counts of patients increased 100% and 67.9% in group I and II, respectively. When incomplete KD was defined only by the main symptoms during the acute stage and the diagnostic criterion of periungual desquamation during the subacute stage was excluded, the significant difference in the incidence of incomplete KD between groups I and II was no longer apparent. Conclusion: Performing regular and detailed observations has resulted in a higher incidence of periungual desquamation and thrombocytosis during the subacute phase of KD than those reported in recent studies. This indicates that until now, we have been neglecting the observation of symptoms and signs during the subacute phase. Regular monitoring during this period can also aid in differentiating suspected cases of KD and facilitate appropriate follow-up of complications.

Incomplete Kawasaki Disease Presenting as Retropharyngeal Abscess: A Case Report and Review of the Literature.

Kawasaki disease (KD) is a cryptic and self-limiting vasculitis predominantly seen in children, often posing a diagnostic challenge due to its varied clinical presentations. Among these, the emergence of deep neck infections, particularly retropharyngeal abscesses, stands out as an extremely rare manifestation. Herein, we present a case of a 10-year-old girl who was hospitalized for fever, neck pain, swollen cervical lymph nodes, and increased inflammation markers. Although anti-infective treatment was initiated, her condition remained unchanged. A cervical computed tomography scan revealed an abscess in the retropharyngeal space. Remarkably, on the third day post-admission, the patient developed symptoms synonymous with KD, such as conjunctival redness, reddened lips, and a strawberry tongue. Subsequent treatment with high-dose intravenous immunoglobulins (IVIG) and oral aspirin led to swift symptom relief, including complete abscess resolution verified by a follow-up neck magnetic resonance imaging. This unique co-presentation of KD and a retropharyngeal abscess, possibly linked to infections like Streptococcus or Staphylococcus aureus, underscores the importance of quick diagnosis and KD management, especially when conventional treatments prove ineffective.

Dilemma in diagnosing incomplete Kawasaki disease in a resource limited setting.

Introduction and importance: Kawasaki disease (KD) is an acute febrile systemic vasculitis that predominantly affects small to medium sized vessels and mostly occurs in children below 5 years of age. The morbidity and mortality mostly occur due to cardiac involvement. Case presentation: The authors present a case of a 5-year-old male child from hilly region of Nepal who presented with fever for 7 days along with strawberry tongue and non-exudative conjunctivitis without rashes, extremity changes or lymphadenopathy. A suspicion of incomplete KD (IKD) was made. The notable investigation findings were increased erythrocyte sedimentation rate, C-reactive protein, leucocyte count and platelets. Echocardiography showed normal findings. Based on the clinical features and supplemental laboratory findings, a diagnosis of IKD was made. The patient improved after intravenous immunoglobulin and Aspirin. Clinical discussion: The main learning objective that the authors get from this case is the challenges in the diagnosis of IKD in the resource limited setting like Nepal. Whether or not to start intravenous immunoglobulin is a dilemma for the physician in most of the cases of suspected IKD, due to the high cost and poor availability of intravenous immunoglobulin in this setting. Hence, the use of inflammatory markers, supplemental laboratory findings together with the few diagnostic criteria met by the patient helps in making a diagnosis and institute timely treatment with intravenous immunoglobulin and aspirin. Conclusion: Diagnosis of KD in difficult in resource limited setting.

ST-Segment Elevation Myocardial Infarction in a 32-Year-Old Man With a History of Incomplete Kawasaki Disease.

The role of the incomplete form of Kawasaki disease in future cardiovascular risk is unknown. The present case demonstrates that even a healthy young man with only a history of incomplete Kawasaki disease can develop endothelial dysfunction and suffer myocardial infarction. We did not obtain ethical/institutional review board approval for our submission because this is not a clinical study, but the patient gave written informed consent to publish the case. (Level of Difficulty: Advanced.).

[Diagnosis and treatment of incomplete Kawasaki disease in children]. / å„¿ç«¥ä¸å®Œå ¨æ€§å·å´Žç— çš„è¯Šæ²».

Kawasaki disease (KD) is a febrile disease mainly observed in children aged <5 years, with medium- and small-vessel vasculitis as the main lesion. Although KD has been reported for more than 50 years and great progress has been made in the etiology and pathology of KD in recent years, there is still a lack of specific indicators for the early diagnosis of KD, especially with more difficulties in the diagnosis of incomplete Kawasaki disease (IKD). At present, there are no clear diagnostic criteria for IKD, which leads to the failure of the timely identification and standardized treatment of IKD in clinical practice and even induce the development of coronary artery lesion. This article reviews the concept, epidemiological features, diagnosis, treatment, and follow-up management of IKD, in order to deepen the understanding of IKD among clinical workers and help to improve the clinical diagnosis and treatment of KD in China.

Diagnosis and treatment of incomplete Kawasaki disease in children / 中国当代儿科杂志

Kawasaki disease (KD) is a febrile disease mainly observed in children aged <5 years, with medium- and small-vessel vasculitis as the main lesion. Although KD has been reported for more than 50 years and great progress has been made in the etiology and pathology of KD in recent years, there is still a lack of specific indicators for the early diagnosis of KD, especially with more difficulties in the diagnosis of incomplete Kawasaki disease (IKD). At present, there are no clear diagnostic criteria for IKD, which leads to the failure of the timely identification and standardized treatment of IKD in clinical practice and even induce the development of coronary artery lesion. This article reviews the concept, epidemiological features, diagnosis, treatment, and follow-up management of IKD, in order to deepen the understanding of IKD among clinical workers and help to improve the clinical diagnosis and treatment of KD in China.

[Clinical experience in the treatment of incomplete Kawasaki disease with no response to intravenous immunoglobulin: a case report]. / é™è„‰æ³¨å°„å ç–«çƒè›‹ç™½æ— åº”ç­”ä¸å®Œå ¨æ€§å·å´Žç— 1例临床经验交流.

This article reports a case of incomplete Kawasaki disease with no response to intravenous immunoglobulin (IVIG). A girl, aged 1 year, had the symptoms of fever, rash, finger desquamation, and coronary artery ectasia. She still had fever at 36 hours after the first dose of IVIG treatment, and her temperature returned to normal after the second dose of IVIG treatment. The follow-up after 1 month showed that the coronary artery diameter returned to normal. This article summarizes the experience in the treatment of incomplete Kawasaki disease with no response to IVIG in order to reduce the incidence of coronary artery damage.

Explore the mechanism of incomplete Kawasaki disease and identify a novel biomarker by weighted gene co-expression network analysis.

Incomplete Kawasaki Disease is a complex disease that often occurs in infants and has substantial coronary artery damage. Its pathogenesis is unclear and lacks specific diagnostic markers. The purpose of our study is to research the mechanism of incomplete Kawasaki Disease use of bioinformatic methods and identify potential biomarkers. We performed weighted gene co-expression network analysis to analyze the data set GSE68004 and identified modules and genes which were correlated with the disease. Through functional annotation and enrichment analysis, we determined the biological function and signal pathway of these genes. We further used lasso regression and ROC curve to screen genes and determined that the final candidate gene was HSPB11and hsa-miR-155-5p that regulates its expression. Finally, we validated the screened gene using an independent dataset and construct a TF-miRNA network. Through the relationships of TFs and hsa-miR-155-5p, we found is hsa-miR-155-5p closely related to hypoxia-related transcription factors, which may be a new direction in the research of Kawasaki disease.

A Sri Lankan infant with immunoglobulin resistant incomplete Kawasaki disease with a vesicular psoriasiform rash, hypertension and late onset small joint arthritis: a case report.

BACKGROUND: Kawasaki disease (KD) is a medium and small vessel vasculitis which usually has a good response to immunoglobulin therapy (IVIG). We present a case of incomplete KD with IVIG resistance associated with an unusual combination of vesicular guttate-psoriasiform rash, hypertension and late onset small joint arthritis. CASE PRESENTATION: A four-month-old male infant from Sri Lanka presented with high fever, conjunctival redness, pedal oedema and skin rash. He was found to have hypertension since admission with a high white cell count and high inflammatory markers. There was poor response to intravenous antibiotics and subsequent 2D echocardiogram revealed coronary artery aneurysms suggestive of KD. In the third week of illness he developed a vesiculo-papular rash involving face, trunk and limbs - which on biopsy revealed features of guttate psoriasis. Fever spikes continued and the coronary arteries showed progressive dilatation despite timely intravenous immunoglobulin administered on day 6 and methylprednisolone administered on day 10-13. Therapeutic response by means of reduction of fever was seen only after initiation of intravenous infliximab on day 28 of illness for which the fever responded within 24 hours. He developed a small joint arthritis of hands and feet on day 40 of illness which responded only after initiating methotrexate therapy. The hypertension persisted for 4 months after the onset of the illness before complete resolution. CONCLUSION: This case report depicts an unusual presentation of KD with a vesicular guttate-psoriasiform eruption, hypertension and late onset small joint arthritis. It highlights that clinicians should be aware of the fact that KD could present with such atypical manifestations and could develop unusual complications.

Incomplete Kawasaki Disease in an Infant: A Case Report and Literature Review.

The term "incomplete Kawasaki Disease (IKD)" was first used to describe patients with coronary complications who did not fulfill the classical diagnostic criteria for Kawasaki Disease (KD). The risk of coronary artery involvement is similar if not greater in cases of IKD. However, the recognition of IKD is challenging and often delayed, especially in infants. Multiple algorithms have been formulated to identify cases of IKD utilizing supplemental clinical, echocardiographic, and laboratory features. Although fever is not required for a diagnosis of KD in the Japanese guideline, most of the current guidelines, including those of the American Heart Association (AHA), consider the presence of fever for at least seven days a requirement for the diagnosis of both KD and IKD in infants. We present a case of IKD in a four-month-old female who presented with fever for less than three days and did not follow the current AHA algorithm for IKD. An echocardiogram obtained 10 days later revealed a coronary artery aneurysm, and a retrospective diagnosis of IKD was made. A review of the literature identified similar cases with a growing consensus on the need to redefine the role of fever. Pediatricians should search for coronary artery lesions in cases of high clinical suspicion, even if the fever period is short, particularly in those less than six months. Additionally, further innovative research is directly needed to identify immunological and cellular markers that could be tested early in the course of the disease and guide the management.

Case Report: Structural Changes in the Coronary Vessel Wall in a Patient With Incomplete Kawasaki Disease.

Background: Kawasaki disease (KD) is an acute systemic vasculitis of infants and young children that affects medium-sized vessels. Conventional cardiac imaging techniques, such as cardiac catheterization, are useful for characterizing the coronary arterial lesion (CAL) size and luminal diameter of the diseased coronary artery segment in patients with KD, but there are limitations to the visualization of the detailed vascular anatomy. Optical coherence tomography (OCT) is a high-resolution intracoronary arterial imaging modality that can distinguish the three layers of the coronary arterial wall. Several studies have reported coronary artery wall abnormalities in KD patients with coronary arterial aneurysm or regressed aneurysm. However, there have been no reports on changes in the coronary artery wall in cases of incomplete KD without CAL. Case Presentation: We herein report an 11-year-old girl with a history of incomplete KD without coronary arterial aneurysms. She had been diagnosed with perimembranous ventricular septal defect (VSD) after birth and had experienced incomplete KD at 1 year old. During her hospitalization for KD, she did not receive intravenous immunoglobulin (IVIG), because she did not meet the Harada score or criteria for treatment in patients with incomplete KD established by the American Heart Association. No dilatation or coronary artery aneurysm were observed on transthoracic echocardiography in the acute or follow-up period. At 11 years old, she received cardiac catheterization and coronary angiography (CAG) for the evaluation of a VSD and follow-up of KD. CAG demonstrated no aneurysm, dilatation, or significant stenosis of the coronary arteries. We performed an OCT study, which revealed the presence of intimal thickening, disruption of the media, and neovascularization in the left anterior descending artery. Conclusion: OCT demonstrates the structural changes of CA even in the patient with incomplete KD who have not been treated with IVIG.

The impact of the American Heart Association guidelines on patients treated for incomplete Kawasaki disease.

OBJECTIVES: To compare patients treated for incomplete Kawasaki disease whose practitioners followed versus did not follow American Heart Association criteria and to evaluate the association of cardiology consultation with adherence to these guidelines. STUDY DESIGN: Single centre retrospective cohort study of patients <18 years old who received ≥1 dose of intravenous immunoglobulin for Kawasaki disease between 01/2006 and 01/2018. We collected demographics, clinical and laboratory data, coronary artery abnormalities, and cardiology consultation status. Patients treated for incomplete Kawasaki disease were divided into two groups based on adherence versus nonadherence to American Heart Association guidelines and compared by Wilcoxon rank sum test and chi-squared or Fisher's exact test. RESULTS: Of the 357 patients treated for Kawasaki disease, 109 (31%) were classified as incomplete Kawasaki disease. The American Heart Association algorithm for identifying patients with incomplete Kawasaki disease was followed in 81/109 (74%). Coronary artery abnormalities were present in 46/109 (42%) of the patients who were treated for incomplete Kawasaki disease. Cardiology consultation was more frequent in those fulfilling American Heart Association criteria for the diagnosis of incomplete Kawasaki disease versus those who did not fulfill criteria (76% versus 48%, p = 0.005). CONCLUSIONS: Over 25% of patients treated for incomplete Kawasaki disease did not meet American Heart Association guidelines. Guidelines were more frequently followed when the paediatric cardiology team was consulted. Consulting physicians with experience and expertise in the evaluation and management of incomplete KD should be strongly considered in the care of these patients.

Kawasaki disease in Malaysia: Biochemical profile, characterization, diagnosis and treatment.

Introduction: Kawasaki disease (KD) is an acute idiopathic systemic vasculitis with a self- limiting course that predominantly affects children under 5 years old, particularly in the East Asian countries. Nevertheless, to date, the data on KD in Malaysia are limited. This study aimed to evaluate the epidemiology, clinical features, treatment, and outcomes of KD among the pediatric patients admitted to Hospital Canselor Tunku Muhriz (HCTM), Kuala Lumpur, Malaysia. Method: A retrospective cohort study of 66,500 pediatric patients presented at HCTM from the year 2004 to 2021 was conducted. Results: 62 KD cases out of 66,500 pediatric admissions were reported, with a male-to-female ratio of 1.58 to 1. Majority of KD patients (95.0%) were younger than 5 years old. Prior infection was reported in 5 KD patients (8.1%). Apart from the classical features, manifestations of various organ systems including cardiovascular (16.1%), gastrointestinal (43.5%), neurological (1.61%), musculoskeletal (1.61%), and genitourinary (17.7%) systems were observed. There was a significant association between sterile pyuria and coronary artery aneurysm (CAA) (p < 0.05). Interestingly, abnormal liver parameters (p < 0.05) and incomplete KD (p < 0.05) were significantly related to IVIG resistance. Discussion: The presence of family history, immunological disorder, and previous infection in our KD patients suggested that there is a possibility of genetic, immunological, and infectious roles in the pathophysiology of KD. IVIG resistance is more likely to occur in KD patients with hepatic dysfunction or incomplete KD presentation. These findings highlighted the significant contribution of laboratory parameters to the prognosis of KD, prompting more in-depth research on the KD scoring systems and their relevance in this country.

Non-resolving Pneumonia As Presentation of Incomplete Kawasaki Disease in Arabic Girl.

Kawasaki disease (KD), particularly incomplete form, might present with wide spectrum clinical features. The treatment regimen includes a combination of intravenous immunoglobulins (IVIG) and aspirin. The use of steroids has been studied as an adjunctive therapy and its role in preventing coronary artery (CA) complications is still debatable. Here, we are presenting a rare presentation of incomplete KD. A previously healthy 5-year-old Arab girl, presented with clinical features consistent with pneumonia, rash, and enlarged cervical lymph nodes. On admission, antibiotics were administered intravenously in addition to steroids considering her reactive airway disease history which resulted in interim improvement. Yet, upon clinical worsening, her clinical status was revised, laboratory and physical examination revealed raised inflammatory markers, new opacity of pulmonary consolidation on chest X-ray, and peeling of skin. Because of high clinical suspicion of incomplete KD combined with her echocardiography that showed prominent coronary arteries, diagnosis of incomplete KD was made. After treating her with IVIG and aspirin, the patient made a full recovery. We are reporting pneumonia-like presentation of incomplete KD. High index of suspicion is required to diagnosis and treat promptly to prevent complications.

Kawasaki Disease Shock Syndrome in the Eastern Region of Saudi Arabia: Case Series.

Kawasaki disease (KD) is a treatable medium-sized vasculitis in the pediatric population consisting of a myriad of specific signs and symptoms. A new entity of the disease, Kawasaki disease shock syndrome (KDSS), is defined as a KD patient presenting with signs of hypoperfusion. Our aim is to describe the signs and symptoms of KDSS and how it is treated and its consequences. Out of 37 patients diagnosed with KD in the period between January 2018 and December 2019 in hospitalized patients younger than 14 years of age at Maternity and Children's Hospital in Al-Hassa, Eastern Province, Saudi Arabia, 3 (8.10%) patients fulfilled the diagnostic criteria for KDSS: 2 (66%) were male and 1 (33%) was female. The cardinal feature in all of them was peripheral cardiovascular collapse. Two patients (66%) were found to have aseptic meningitis. All patients were treated with immunomodulatory agents (intravenous immunoglobulin) and all responded well to anti-inflammatory doses of aspirin. KDSS is the shock state of KD presenting with hypoperfusion symptoms, mainly irritability and changes in the level of consciousness and peripheral cardiovascular collapse. Awareness of such presentation and management by immunomodulatory medications helps in recovery and prevention of tragic consequences of such disease.

Atypical Kawasaki Disease Complicated With Coronary Artery Aneurysm: A Case Report and Review of Literature.

BACKGROUND: Kawasaki disease (KD) is an acute, febrile systemic vasculitis of early childhood. A small group of KD patients does not meet the classical presentation of KD, termed incomplete KD. Incomplete or atypical KD patients are usually infants and older children. Because of atypical manifestations of KD, timely diagnosis of KD is difficult, which leads to coronary artery complication Case presentation: We report the case of a nine-year-old boy who developed fever and right side parotitis with painful cervical lymphadenopathy leading to torticollis as the first symptom of Kawasaki disease (KD). A series of investigations revealed elevated inflammatory markers and aneurysmal dilation of coronary artery on echocardiogram, and thus he was diagnosed with atypical KD. Intravenous immunoglobulin was given and the child responded well. Coronary artery aneurysm resolved by six months. CONCLUSION: A high index of suspicion should be maintained in children presenting with fever and unusual manifestations like lymphadenopathy and parotitis, especially where empiric antibiotics were ineffective. Evaluation of cardiac function and coronary artery status with echocardiography is helpful in defining the diagnosis of KD in such cases. As it is a noninvasive test, it should be undertaken at the first possible clinical suspicion.

Correct identification of incomplete Kawasaki disease.

Incomplete Kawasaki disease (IKD) is characterized by a longer fever time, younger age of onset, and higher incidence of coronary artery disease compared with complete Kawasaki disease. Kawasaki disease is often difficult to diagnose early because of its incomplete clinical symptoms. This issue could delay treatment and harm the health of the child. This article reviews the clinical characteristics and pathogenesis of IKD to help clinicians understand the symptoms of IKD, make the correct diagnosis, and provide timely treatment.

Difference in serum miRNA expression between immunoglobulin-sensitive and -insensitive incomplete Kawasaki disease patients.

The present study aimed to investigate the expression of microRNAs (miRNAs/miRs) and inflammatory factors in patients with immunoglobulin-sensitive and IVIG-insensitive incomplete Kawasaki disease (KD). One hundred and eighty-five patients with incomplete KD were included as the study group (KD group), and 182 patients with respiratory infection as the control group. Neutrophil to lymphocyte ratio (NLR), C-reactive protein (CRP) levels, alanine aminotransferase (ALT), aspartate aminotransferase (AST), white blood cell count (WBC), hemoglobin level (Hb), platelet count (PLT) and T cell subsets (CD3+, CD3+ CD4+) were compared. Patients in the KD group received aspirin (30 mg/kg orally daily) and gamma globulin (IVIG, 1 g/kg intravenously daily). According to the sensitivity to IVIG, patients were divided into IVIG-sensitive group and IVIG-insensitive KD group. The relative expression levels of miRNA-21, miRNA-145, miRNA-155 and miRNA-199b-5p in the serum were detected by RT-qPCR. Serum TNF-α, IL-6 and IL-1ß levels were assessed using ELISA. Before treatment, the neutrophil to lymphocyte ratio (NLR), levels C-reactive protein, and leukocytes in the KD group were significantly higher compared with the control group (P<0.05). After medical intervention, the relative expression of miRNA-21, miRNA-145 and miRNA-155 in the serum of patients in IVIG-sensitive and IVIG-insensitive KD groups were increased when compared with these levels in the control group (P<0.05). Meanwhile, the relative expression of miRNA-199b-5p was decreased (P<0.05). Compared with the IVIG-sensitive KD group, the relative expression levels of miRNA-145 and miRNA-155 were increased in the serum of patients in the IVIG-insensitive KD group (P<0.05). Compared with the control group, the levels of TNF-α, IL-6 and IL-1ß were increased in the serum of patients in the IVIG-sensitive and IVIG-insensitive KD groups (P<0.05). Compared with the IVIG-sensitive KD group, the serum levels of TNF-α and IL-6 were increased in patients of the IVIG-insensitive KD group (P<0.05). Except for NLR and CRP, there were differences in the expression of peripheral blood miRNA-145, miRNA-155 and serum TNF-α and IL-6 in patients with immunoglobulin-sensitive and -insensitive incomplete KD.

Clinical profile of Kawasaki disease in children admitted at a tertiary care hospital of North India and their short-term follow-up.

Aim: The aim of this study was to evaluate presenting symptoms, clinical features, and laboratory tests for the diagnosis of Kawasaki disease (KD) in children and their short-term follow-up at a tertiary care hospital of North India from April 2017 to March 2020. Materials and Methods: A total of 31 children (23 boys and 8 girls) up to 10 years of age were included in this study. The diagnosis of KD was made as per the American Heart Association 2017 guidelines. Clinical features, laboratory parameters, and coronary involvement were compared between the complete and incomplete KD groups. Results: The incidence of complete versus incomplete KD was 19 (61.2%) versus 12 (38.7%) children, respectively. Change in extremities and oral mucosal changes were more encountered in the complete KD group as compared to the incomplete KD group (100% vs. 58.3%, P = 0.004, and 78.9% vs. 33.3%, P = 0.002, respectively). Coronary artery aneurysm was seen in 54% of the patients on echocardiography which was greater in the incomplete KD group (83.3%) as compared to the complete KD group (36.8%). The median time from the onset of symptoms to intravenous immunoglobulin infusion was <10 days in 84.2% of the patients with complete KD versus 41.7% with incomplete KD which was statistically significant. Fifty percent of the children with coronary ectasia and small aneurysm had normal coronaries at follow-up of 6 months. Conclusion: KD is probably underdiagnosed in most developing countries, like that of ours, and requires a high index of suspicion.

Sudden death due to incomplete Kawasaki disease: A case report.

Due to the lack of typical clinical manifestations, the incomplete Kawasaki disease (KD) is easily misdiagnosed and missed. The sudden death risk in incomplete KD cases is similar to typical KD. In this study, we report a case of a 1-year-old boy who died suddenly without any warning after incomplete KD. The boy was admitted due to fever and a cough, with preliminary diagnosis of acute severe bronchial pneumonia, but no typical KD characteristics. After antibiotics and supportive treatment, the condition worsened. Finally, the boy died after the ineffective rescue. The pathology revealed that the boy suffered from incomplete KD. From this case, we experience that, when infants or children have a long-lasting fever, the possibility of KD should not be ignored. In addition, when managing such patients, the frequency of heart ultrasound should be increased as appropriate.