Enfermedad de Kawasaki. Presentación atípica en un paciente pediátrico

La enfermedad de Kawasaki es un síndrome mucocutáneo caracterizado por vasculitis, que afecta medianos vasos; su principal manifestación es un síndrome febril agudo de, al menos, cinco días de duración y en muchas ocasiones de etiología desconocida. Se aprecia, fundamentalmente, en niños menores de cinco años de edad. Se considera que es frecuente, pero existen subregistros. Se caracteriza por tener dos formas de presentación: típica o atípica. Se presenta el caso clínico de un niño que fue hospitalizado debido a síndrome febril agudo asociado a malestar general e irritabilidad. Al examen físico se observaron edemas discretos en manos y pies así como erupción cutánea, no hepatomegalia ni alteraciones oculares. Teniendo en cuenta la epidemiología, lo atípico del cuadro clínico y los resultados de estudios hemoquímicos, se concluyó el diagnóstico de enfermedad de Kawasaki atípica. Se impuso tratamiento específico y se logró una evolución satisfactoria y la no aparición de complicaciones inmediatas. Esta entidad nosológica requiere de una adecuada valoración clínica-epidemiológica de los pacientes, así como de un minucioso examen físico y un diagnóstico precoz para lograr la evolución favorable de los pacientes y la no presencia de secuelas cardiacas, que pondrían en peligro la vida del paciente y/o su calidad de vida futura.

Kawasaki disease (KD) is a mucocutaneous syndrome characterized by vasculitis that affects medium vessels; its main manifestation is an acute febrile syndrome lasting at least five days and often of unknown etiology. It appears mainly in children under five years of age, it is considered to be frequent, but there are underreportings. It is characterized by having two presentation forms: typical or atypical. The clinical case of a child who was hospitalized due to acute febrile syndrome associated with malaise and irritability is presented. The physical examination revealed discrete edema in the hands and feet as well as a rash, no hepatomegaly or ocular alterations. Taking into account the epidemiology, the atypical clinical picture and the results of hemochemical studies, the diagnosis of atypical Kawasaki disease was concluded. Specific treatment was imposed and a satisfactory evolution was achieved with no immediate complications. This nosological entity requires an adequate clinical-epidemiological evaluation of the patients, as well as a meticulous physical examination and an early diagnosis to achieve a favorable patients' evolution and the absence of cardiac sequelae, which would endanger the patients' life and/or their future quality of life.

Analysis of coronary angiographic findings in 117 children with Kawasaki disease and coronary artery lesion / 中华实用儿科临床杂志

Objective:To analyze the coronary angiographic (CAG) characteristics of coronary artery lesion (CAL) in children with Kawasaki disease (KD), and to clarify the necessity of CAG in the diagnosis and treatment of KD combined with CAL in children.Methods:It was a retrospective study to analyze the clinical data, electrocardiogram, echocardiography, time and findings of CAG in children with KD and CAL who underwent CAG in Shanghai Children′s Medical Center of Shanghai Jiao Tong University School of Medicine from January 2013 to August 2022.The distribution, type, severity, and prognosis of CAL were analyzed.Results:A total of 117 children with KD and CAL were included in the analysis.The onset age of KD was from 2 months to 12.8 years old, and the age of performing CAG was from 8 months to 18.1 years old.A total of 234 coronary artery lesions were detected in 117 cases.Among them, CAL in the right coronary artery (RCA), left anterior descending branch (LAD), left main coronary artery and left circumflex artery were detected in 96 branches(41.1%), 78 branches(33.3%), 44 branches(18.8%), and 16 branches(6.8%), respectively.Unilateral coronary artery involvement was detected in 43 cases (36.8%), of which LAD was the dominant; while bilateral involvement was detected in 74 cases (63.2%), among which, LAD and RCA were the most involved arteries.Stratified by the degree of coronary involvement, large coronary aneurysms and severe coronary stenosis were most frequently occurred in the RCA and LAD.In contrast, 10 cases (13.6%), 20 cases (24.3%), 55 cases (45.8%) and 37 cases (67.3%) of intraluminal lesions were found in small, medium and large coronary aneurysms, and stenosis or occlusion, respectively.The incidence of intraluminal lesions tended to be higher in the site of severe lesions.CAG showed stenosis or occlusion in a total of 55 cases, and collateral circulation at varying degrees was found in cases of severe stenosis or occlusion.Conclusions:CAL in children with KD are complex and varied.Although clinical symptoms, routine electrocardiogram and cardiac ultrasound may indicate severe CAL.Their applications are limited by the diagnosis of the type (especially stenosis), degree, and extent of CAL, as well as the detection of extracoronary lesions.CAG is of great significance to identify vascular lesions and guide clinical management of KD combined with CAL in children.

Study on the evolution of TCM syndromes of 171 cases of Kawasaki disease under the intervention of gamma globulin therapy based on factor analysis / 国际中医中药杂志

Objective:To study the evolution of Traditional Chinese Medicine (TCM) syndromes of 171 cases of Kawasaki disease (KD) under the intervention of gamma globulin therapy based on factor analysis.Methods:A cross-sectional study. 171 cases of KD children hospitalized in the Department of Cardiology of Guangzhou Women's and Children's Medical Center from July 2019 to December 2020 were collected. All patients were treated with intravenous gamma globulin (2 g/kg) for 1 week. According to the results of the treatment with C-ball, 171 children with C-ball sensitive KD were selected to collect the four diagnostic data, and the representative syndromes of defensive level, qi level, yin level, and nutritive level were observed. Factor analysis was used to analyze the evolution of syndrome in 171 children with KD c-ball sensitivity.Results:The result of factor analysis showed that the KMO statistics of 171 children with c-ball sensitivity before treatment was 0.792, and Bartley test was significant ( P<0.01). 16 common factors were extracted, and 23 syndromes were screened, mainly including defensive level disorder, qi level disorder,nutritive level disorder,yin level disorder, heat stagnation and blood stasis syndrome. One week after treatment, the statistic of KMO test was 0.787, and Bartley test was significant ( P<0.01). 9 common factors were extracted, and 10 syndromes were screened, mainly including qi deficiency syndrome, yin deficiency syndrome and blood stasis syndrome. Conclusion:Before treatment, the TCM syndromes in KD C-cell sensitive children are mainly nutritive level disorder, defensive level disorder, qi level disorder yin level disorder, and heat stagnation and blood stasis syndrome; after treatment, the main TCM syndromes are mainly qi deficiency syndrome, yin deficiency syndrome and blood stasis syndrome.

Brief communication: Vitamin D serum levels in American tegumentary leishmaniasis from an endemic area in Northeast Brazil

Abstract Introduction The pathogenesis of cutaneous and mucosal leishmaniasis is associated with different immune responses. Vitamin D may modulate the immune system. Here we evaluate the association of vitamin D levels with the severity of the clinical forms of cutaneous and mucosal leishmaniasis. Methods We conducted an observational study evaluating the association between vitamin D levels, disease severity and therapeutic response in patients with cutaneous and mucosal leishmaniasis. Additionally, we conducted a cross-sectional study to compare vitamin D levels in patients with leishmaniasis and healthy subjects. Hypovitaminosis D was defined as a serum level of 25 (OH) D < 30 ng/mL. Results In patients with leishmaniasis, vitamin D serum levels were 38.5 ± 11.54 ng/mL, and 37.5 ± 10.43 ng/mL in healthy subjects The prevalence of hypovitaminosis D was 23.3% and 20.0%, respectively (p = 0.72). There was no correlation between vitamin D serum levels, disease severity, and healing time in the mucosal leishmaniasis group. Conclusion Vitamin D levels are not associated with neither susceptibility nor severity of tegumentary leishmaniasis.

Surgical Correction of Ascending Aortic Aneurysm Without Coronary Dilatation After Kawasaki Disease in a 3-Year-Old Child

Abstract Kawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.

American Cutaneous Leishmaniasis in Eastern Minas Gerais: veiled reality of a neglected disease / Leishmaniose Tegumentar Americana no Leste de Minas Gerais: realidade velada de uma doença negligenciada

Objective: to analyze the notified and confirmed cases of ACL in a municipality in east Minas Gerais, from 2007 to 2020. Methods: a combined study was carried out as a cross-sectional and an ecological approach of time series type, using notified and confirmed ACL cases, from 2007 to 2020. Primary and secondary data were used. Data were analyzed using descriptive and inferential statistics (simple linear regression, T-test, Mann-Whitney, chi-square (χ2) at a 5% significance level). Results: a total of 219 cases were reported with a decreasing temporal trend, with a higher frequency observed for the cutaneous form (82.6%), age group 40 to 59 years (32.1%), black race (56.4%), and completed elementary school (47.7%). Individuals with the mucosal clinical form had lesions for a longer time, a greater chance of not progressing to cure, and used more vials of meglumine antimoniate when compared to patients with the cutaneous form. Conclusions: different correlations were observed between the variables studied and the profile of involvement described in the scientific literature, with the clinical form predominantly cutaneous and with a good prognosis.

Objetivo: analisar os casos notificados e confirmados de LTA em um município do leste de Minas Gerais, no período de 2007 a 2020. Métodos: foi realizado um estudo combinado com abordagem transversal e ecológica do tipo série temporal, utilizando casos notificados e confirmados de LTA, de 2007 a 2020. Foram utilizados dados primários e secundários. Os dados foram analisados por meio de estatística descritiva e inferencial (regressão linear simples, teste T, Mann-Whitney, qui-quadrado (χ2) com nível de significância de 5%). Resultados: foram notificados 219 casos com tendência temporal decrescente, com maior frequência observada para a forma cutânea (82,6%), faixa etária de 40 a 59 anos (32,1%), raça negra (56,4%) e ensino fundamental completo (47,7%). Indivíduos com a forma clínica mucosa apresentaram maior tempo de lesão, maior possibilidade de não evoluir para cura e utilizaram mais ampolas de antimoniato de meglumina quando comparados aos pacientes com a forma cutânea. Conclusões: foram observadas diferentes correlações entre as variáveis estudadas e o perfil de acometimento descrito na literatura científica, com a forma clínica predominantemente cutânea e com bom prognóstico.

Successful Surgical Treatment of Coronary Aneurysm, Ascending Aortic Aneurysm, and Bicuspid Aorta for a Kawasaki Disease Patient

Abstract Kawasaki disease was first reported in 1967, and it was classified as an autoimmune vasculitis of the small and medium arteries. It is a self-limiting condition that occurs mostly in childhood, but it may involve complications — such as coronary artery aneurysms, myocardial ischemia, and arrhythmias — with significant morbidity and mortality that occur later in life. In this article, we present the association of an ascending aortic aneurysm with bicuspid aortic disease in addition to coronary aneurysm in a 55-year-old patient diagnosed with Kawasaki disease.

Gender-Wise histological differences in the human upper lip

Introduction: Knowledge of morphometry of lip lining help in deciding the best site for choosing graft for its better uptake during several dermal grafting procedures following craniofacial cancers or cosmetic procedures. It also proves useful in dermatopharmacokinetics, in which we monitor the effect of drugs acting on connective tissue by translabial route and lip augmentation surgeries (esthetic surgery) where care is to be given for dermal fillers not to be injected into the muscle core of lip. Materials and Methods: Ten human male cadavers and 10 human female cadavers were procured. The rectangle‑shaped skin specimen (1 cm × 1 cm) through the upper lip was stained with hematoxylin and eosin stain. A total of 40 slides were prepared. Readings were obtained with the help of CATCAM E series HD cameras which was installed in light microscope. Results: The mean value of thickness of skin (epidermis + dermis) of the lip was 664.72 μm among males while 769.20 μm among females. Conclusion: The epithelium of females is marginally thicker than males. Edp: sc (epidermis/stratum corneum) ratio can suggest that giving drugs through translabial route will be easy in females as compared to males in the upper lip as the stratum corneum is the main barrier in drug transfusion and its absorption secondary to epidermis as a whole. The number of rete pegs per field at the dermoepidermal junction was higher in males which ensures more stability of skin of male lips compared to females

Enfermedad de Kawasaki en pediatría: caso clínico

la enfermedad de Kawasaki (EK) es una vasculitis multisistémica que se presenta en la infancia. Por lo general es una afección aguda, febril, autolimitada, de etiología desconocida y puede desarrollar alteraciones cardiovasculares; su expresión clínica es variable: presenta inflamación de los vasos de mediano calibre y de múltiples tejidos: pulmonar, meníngeo, cardiaco, urinario, gastrointestinal, musculoesquelético, neurológico o linfático. El diagnóstico y el tratamiento buscan disminuir la duración de los síntomas y prevenir la presencia de aneurismas coronarios.

Kawasaki disease (KD) is a multisystemic vasculitis that presents in childhood. It is usually an acute, febrile, self-limited disease of unknown etiology and may develop cardiovascular alterations; its clinical expression is variable as it presents inflammation of medium caliber vessels and multiple tissues: pulmonary, meningeal, cardiac, urinary, gastrointestinal, musculoskeletal, neurological or lymphatic. Diagnosis is clinical, and treatment aims to reduce the duration of clinical symptoms and prevent the presence of coronary aneurysms. Objective: to describe the clinical case of an infant patient with KD, in which clinical criteria characteristic of this pathology were identified. Case report: a 22-month-old male patient was admitted to the emergency room with a 14-day evolutionary clinical picture consisting of fever, emetic episodes, abdominal pain, unresponsive to acetaminophen, and diarrheal episodes. Two days later, he manifested generalized exanthema in the inguinal region that spread to the right testicle, back, and thorax. Subseguently, he presented bilateral nonpurulent conjunctivitis for three days. He received treatment with topical steroids and oral antihistamines; partial improvement with subseguent evolution in right testicularedema and bilateral desquamation in hands and feet. KD was suspected, a transthoracic echocardiogram was sent without coronary aneurysmal dilatations, and treatment with ASA was started without indication, given the evolution of immunoglobulin. Conclusions: KD is infrequent in childhood and should be suspected in patients with prolonged febrile symptoms unresponsive to conventional treatments and in whom the presence of other pathologies is ruled out.

a doença de Kawasaki (DK) é uma vasculite multissistêmica que ocorre na infância. Geralmente é uma doença aguda, febril, autolimitada, de etiologia desconhecida, podendo desenvolver alterações cardiovasculares; sua expressão clínica é variável, apresentando inflamação de vasos de médio porte e de múltiplos tecidos: pulmonar, meníngeo, cardíaco, urinário, gastrointestinal, musculoesquelético, neurológico ou linfático. O diagnóstico é clínico e o tratamento visa reduzir a duração dos sintomas clínicos e prevenir a presença de aneurismas coronábanos. Objetivo: descrever o caso clínico de um paciente infantil com DK, no qual foram identificados critérios clínicos característicos dessa patologia. Caso clínico: paciente do sexo masculino, 22 meses de idade meses de idade, admitido no departamento de emergência com um curso clínico de 14 dias, consistindo em febre, episódios eméticos e dor abdominal, sem resposta ao acetaminofeno, além de episódios diarreicos. Dois dias depois, ele desenvolveu uma erupção cutánea generalizada na região inguinal que se espalhou para o testículo direito, costas e tórax. Posteriormente, ele apresentou conjuntivite bilateral não purulenta por três dias. Ele foi tratado com esferoides tópicos e anti-histamínicos orais; houve melhora parcial com posterior evolução do edema testicular direito, descamação bilateral nas mãos e nos pés. Suspeitou-se de DK, portanto foi realizado ecocardiograma transtorácico sem dilatações aneurismáticas coronarianas e iniciado tratamento com AAS, sem indicação devido ao tempo de evolução da imunoglobulina. Conclusões: a DK não é frequente na infância e deve ser suspeitada em pacientes com sintomas febris prolongados que não respondem aos tratamentos convencionais e nos quais a presença de outras patologias é descartada.

Uma visão odontológica sobre a leishmaniose tegumentar americana: revisão de literatura / A dental view on american tegumentary leishmaniosis: literature review

A Leishmaniose é um grupo de doenças causadas por protozoários do gênero Leishmania. Dentre as Leishmanioses do novo mundo está a Leishmaniose Tegumentar Americana (LTA), que podem se manifestar como lesões primárias na mucosa ou pele da região de cabeça e pescoço. Essas lesões podem afetar a cavidade oral e/ou o paciente apresentar sinais e sintomas detectáveis pelo cirurgião-dentista. O objetivo deste trabalho é realizar um levantamento bibliográfico sobre LTA nas principais bases de dados, buscando relacioná-lo a esta área de atuação. Para isso, foram realizadas buscas nas bases de dados SciELO, Pubmed/MEDLINE e LILACS com os descritores "Leishmaniose Mucocutânea", "Medicinal oral" e "Diagnóstico", nas línguas inglesa e portuguesa, no período entre 2011 a 2021. Como resultado foram encontrados 379 artigos e, após considerar os critérios de inclusão e exclusão, foram selecionados 20 artigos e 6 informes epidemiológicos. Logo, a LTA deve fazer parte do diagnóstico diferencial de lesões compreendidas nas áreas de atuação do cirurgião-dentista, possibilitando um diagnóstico precoce e encaminhamento para adequado tratamento médico. Além disso, esse conhecimento é necessário para que políticas públicas de prevenção e educação sejam mais eficazes, visando a diminuição da prevalência dessa patologia e aumento da qualidade de vida do paciente.

Leishmaniasis is a group of diseases caused by protozoa of the genus Leishmania. Among the Leishmaniasis of the new world is American Tegumentary Leishmaniasis (ATL), which can manifest as primary lesions in the mucosa or skin of the head and neck region. These lesions can affect the oral cavity and / or the patient has signs and symptoms detectable by the dentist. The objective of this work is to carry out a bibliographic survey on ATL in the main databases, seeking to relate it to this area of ​​activity. For this purpose, searches were carried out in the SciELO, Pubmed / MEDLINE and LILACS databases with the descriptors "Leishmaniasis Mucocutânea", "Oral Medicinal" and "Diagnostic", in the English and Portuguese languages, in the period between 2011 and 2021. As a result, 379 articles were found and, after considering the inclusion and exclusion criteria, 26 articles were selected. Therefore, ATL should be part of the differential diagnosis of lesions in the areas in which the dentist operates, enabling an early diagnosis and referral to appropriate medical treatment. In addition, this knowledge is necessary for public policies on prevention and education to be more effective, aiming at decreasing the prevalence of this pathology and increasing the patient's quality of life.

Manifestaciones mucocutáneas en pacientes hospitalizados por COVID 19 en un hospital de alta complejidad / Mucocutaneous manifestations in patients hospitalized with covid-19 in a high complexity hospital

Resumen Introducción: el SARS-CoV-2 es un virus ARN de cadena simple que compromete diferentes órganos, incluyendo la piel. Los pacientes que cursan con este virus pueden presentar diferentes manifestaciones mucocutáneas. Objetivo: determinar la prevalencia de las lesiones mucocutáneas en pacientes hospitalizados por infección SARS-CoV-2/COVID 19 en el Hospital San Vicente Fundación Rionegro entre abril y junio del 2021. Materiales y métodos: estudio descriptivo transversal en pacientes hospitalizados con diagnóstico de infección por COVID-19 confirmado por pruebas serológicas y con lesiones mucocutáneas. Resultados: de 600 pacientes hospitalizados por COVID-19, 16 presentaron lesiones mucocutáneas para una prevalencia del 2,6%, y entre estos, 13 requirieron manejo en unidad de cuidados intensivos por síndrome de dificultad respiratoria aguda severa o enfermedad severa (81,25%) asociado a marcadores de mal pronóstico elevados. Los más elevados fueron la PCR, LDH o la presencia de linfopenia. Las lesiones vasculares fueron las más frecuentes (42,9%), que se manifestaron como púrpuras, vasculitis, livedo racemosa, perniosis y pseudoperniosis. Las erupciones o exantemas fueron de tipo maculopapulares (28,6%), eritematosas (19%) y urticariales (9,5%); además de úlceras en cavidad oral (8,8%) y vesículas (2,9%). El 75% de estos pacientes presentaron lesiones cutáneas en la fase activa de la enfermedad y el 25% en la fase resolutiva. Las morbilidades más frecuentes fueron hipertensión arterial (30%) y diabetes (20%). Seis pacientes fallecieron (37,5%). Conclusiones: las manifestaciones mucocutáneas asociadas al COVID-19, especialmente las de tipo vascular como las púrpuras, vasculitis y livedo racemosa, se asocian a formas graves de la enfermedad, especialmente en pacientes con ventilación mecánica asistida con un alto índice de mortalidad.

Abstract Introduction: SARS-CoV-2 is a single-stranded RNA virus that affects different organs, including the skin. Patients with this virus can present different mucocutaneous manifestations. Objective: to determine the prevalence of skin lesions in patients hospitalized for SARS-CoV-2 / COVID 19 infection at the Hospital San Vicente Fundación Rionegro between April and June 2021. Materials and methods: descriptive cross-sectional study in hospitalized patients with a diagnosis of infection by COVID-19 confirmed by serological tests and with mucocutaneous lesions. Results: of 600 patients hospitalized for COVID-19, 16 presented mucocutaneous lesions for a prevalence of 2,6 %, and among these, 13 required management in the intensive care unit due to severe acute respiratory distress syndrome or severe illness (81,25%) associated with elevated markers of poor prognosis. The most increased were PCR, LDH and/or lymphopenia. Vascular lesions were the most frequent (42,9%), manifested as purples, vasculitis, livedo racemosa, perniosis and pseudoperniosis. The eruptions or rashes were maculopapular (28.6%), erythematous (19%) and urticarial (9,5%), and ulcers in the oral cavity (8,8%) and vesicles (2,9%). 75% of these patients had skin lesions in the active phase of the disease and 25% in the resolution phase. The most frequent morbidity was arterial hypertension (30%) and diabetes (20%). six patients (37,5%) died. Conclusions: the mucocutaneous manifestations associated with COVID-19, especially those of a vascular type such as purples, vasculitis and livedo racemosa, are associated with severe forms of the disease, especially in patients with assisted mechanical ventilation with a high mortality rate.

Effects of Notch1 signaling on regulatory T cells and coronary artery lesions in childhood Kawasaki disease / 中华风湿病学杂志

Objective:To explore the effect of Notch1 signaling on regulatory T cells and its roles in vascular damage in patients with Kawasaki disease (KD).Methods:A total of 42 children with KD were enrolled in the present study from March 2019 to June 2020, as 32 age-matched healthy children were recruited as control. The proportions of CD4 +CD25 hiFoxp 3+ regulatory T cells (Treg) and expressions of transcription factor forkhead box protein 3 (Foxp3), cytotoxic T lymphocyte associated antigen-4 (CTLA4), glucocorticoid-induced tumor necrosis factor receptor (GITR), and Notch1 protein were evaluated by flow cytometry. Chromatin immunoprecipitation was conducted to detect acetylation level of histone H4 (H4Ac) associated with the promoter of Foxp3 gene and its binding abilities of Notch1 intracellular domain 1 (NICD1), recombination signal binding protein for immunoglobulin kappa J region (RBP-J) and p300 in CD4 + T cells. Transcription levels of Foxp3, presenilin 1 (PSEN1), mastermind like transcriptional coactivator 1 (MAML1), and RBP-J in CD4 + T cells were determined by real-time polymerase chain reaction (PCR). Concentrations of interleukin (IL)-10 and transforming growth factor-β (TGF-β) in plasma and culture supernatant stimulated with Jagged1 were measured by enzyme linked immunosorbent assay. Independent-sample t-test, Pearson correlation analysis was used as the statistical method in this study. Results:① The frequencies of Treg in acute KD patients decreased significantly [(4.3±1.5)% vs (7.9±2.9)%; t=6.41, P<0.001], as protein levels of Foxp3, CTLA4 and GITR and concentrations of IL-10 and TGF-β in plasma reduced remarkably in acute KD patients ( t=6.87, P<0.001; t=4.26, P<0.001; t=7.88, P<0.001; t=8.42, P<0.001; t=13.01, P<0.001). All parameters afore-mentioned in patients combined with coronary artery lesions (CAL) were lower than those of patients without coronary artery lesions (NCAL) ( t=5.83, P<0.001; t=3.83, P<0.001; t=3.28, P=0.002; t=5.05, P<0.001; t=5.96, P<0.001; t=5.17, P<0.001), and increased after therapy ( t=7.13, P<0.001; t=6.10, P<0.001; t=4.31, P<0.001; t=6.55, P<0.001; t=7.40, P<0.001; t=7.84, P<0.001). ② H4Ac associated with promoter of Foxp3 gene and the binding abilities of NICD1 and p300 in acute KD patients were lower than those of the controls ( t=10.25, P<0.001; t=6.93, P<0.001; t=6.75, P<0.001), and increased remarkably after therapy ( t=7.72, P<0.001; t=4.16, P<0.001; t=5.76, P<0.001). Meanwhile, the three items in CAL group were found to be less than those of NCAL group ( t=6.08, P<0.001; t=2.66, P=0.011; t=6.02, P<0.001). Pearson correlation analysis showed a positive correlation between H4Ac associated with Foxp3 promoter and its mRNA level in acute KD patients ( r=0.47, P<0.001). No statistical significant difference about the binding ability of RBP-J with Foxp3 promoter were found among the groups ( t=0.57, P>0.05; t=0.61, P>0.05; t=1.20, P>0.05). ③ Protein level of Notch1 and the expressions of PSEN1, MAML1 and RBP-J mRNA in CD4 + T cells from acute KD patients were down-regulated remarkably ( t=5.28, P<0.001; t=6.31, P<0.001; t=11.78, P<0.001; t=8.06, P<0.001), and restored after therapy ( t=4.77, P<0.001; t=6.43, P<0.001; t=11.95, P<0.001; t=7.79, P<0.001). In parallel, the four indexes aforementioned of CAL group were lower than those of NCAL group ( t=3.16, P=0.003; t=4.13, P<0.001; t=5.42, P<0.001; t=4.05, P<0.001). Upon rhJagged1 stimulation for 48 hours, H4Ac level of Foxp3 promoter and its binding abilities with NICD1 and p300 in CD4 + T cells in KD patients and control group was significantly higher than those of untreated group [(KD: t=15.36, P<0.001; t=7.25, P<0.001; t=14.29, P<0.001), (Ctrl: t=7.87, P<0.001; t=5.71, P<0.001; t=8.74, P<0.001)], as the binding ability of RBP-J with Foxp3 promoter increased slightly without statistically significant difference (KD: t=1.11, P>0.05; Ctrl: t=1.37, P>0.05). Simultaneously, H4Ac level of Foxp3 promoter and its binding abilities with NICD1 and p300 in KD group were still lower than those of the control group after stimulation ( t=3.86, P<0.001; t=3.42, P=0.001; t=2.85, P=0.006). ④ After incubation of PBMC from heathy children with KD serum, the proportion of Treg cells, protein level of Foxp3 and expressions of Notch1 and RBP-J in CD4 + T cells in the group treated with IVIG increased significantly compared with the untreated group ( t=7.10, P<0.001; t=10.16, P<0.001; t=8.06, P<0.001; t=9.77, P<0.001), as well as H4Ac level of Foxp3 promoter and its binding abilities with NICD1 in the group treat with IVIG were also higher than the latter ( t=7.24, P<0.001; t=8.24, P<0.001). Conclusion:Insufficiency and impaired function of Treg caused by aberrant Notch1 signaling may be the important factor contributing to immune dysfunction and vascular damage in KD.

Clinical analysis of 105 cases of Kawasaki disease complicated by hypoproteinemia / 中国基层医药

Objective:To analyze the clinical characteristics of children with Kawasaki disease complicated by hypoproteinemia.Methods:The clinical data of 105 children patients with Kawasaki disease who received treatment in Ji'an Central People's Hospital, China between January 2015 and December 2019 were retrospectively analyzed. These patients were divided into normal-level albumin (ALB) group (≥ 35 g/L, n = 54) and low-level ALB group (< 35 g/L, n = 51). White blood cell count, hemoglobin, C-reactive protein, erythrocyte sedimentation rate, alanine aminotransferase, aspartate aminotransferase, total bile acid, and coronary artery B-ultrasound examination results were compared between the two groups. Results:The incidence of hypoproteinemia was similar among all age groups. There was significant difference in coronary artery dilatation rate between the two groups (χ 2 = 5.49, P < 0.05). There were no significant differences in the percentage of patients with hand and foot scleredema and urinary protein-positive rate between the two groups (χ 2 = 0.40, 0.39, both P > 0.05). White blood cell count, erythrocyte sedimentation rate, C-reactive protein level in the low-level ALB group were 15.2 × 10 9/L, (82.1 ± 26.1) mm/h, 94.7 mg/L, respectively, which were significantly higher than those in the normal-level ALB group [11.5 × 10 9/L, (54.5 ± 26.2) mm/h, 43.65 mg/L, Z = -2.94, t = 5.40, Z = -6.01, all P < 0.01]. There were no significant differences in alanine aminotransferase, aspartate aminotransferase, and total bile acid levels between the two groups (all P > 0.05). Conclusion:The incidence of Kawasaki disease complicated by hypoproteinemia is unrelated to age and it is related to inflammatory reaction. Hypoproteinemia is a risk factor of coronary artery dilatation. This study is of great science.

Comparative analysis of clinical laboratory indicators between Kawasaki disease and systemic juvenile idiopathic arthritis / 中国基层医药

Objective:To explore the differences of clinical laboratory indicators between Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (SJIA), providing objective evidence for diagnosis and differential diagnosis of these diseases.Methods:A total of 41 children patients with KD (KD group) and 33 children patients with SJIA (SJIA group) who received treatment in Huainan Maternal and Child Health Hospital between September 2017 and January 2022 were retrospectively analyzed. An additional 50 healthy children who concurrently received physical examination in the same hospital were included in the control group. Platelet count (PLT), white blood cell count (WBC), and erythrocyte sedimentation rate (ESR) as well as C-reactive protein (CRP), serum procalcitonin (PCT), interleukin-6 (IL-6), interleukin-10 (IL-10), and serum ferritin (SF) levels were compared among groups before treatment.Results:One-way analysis of variance and pairwise q test were performed to compare laboratory indicators among KD, SJIA and control groups. CRP, ESR, SF and IL-6 levels in the KD group were significantly lower than those in the SJIA group [CRP: (57.80 ± 25.23) mg/L vs. (77.72 ± 45.64) mg/L; ESR: (67.02 ± 28.80) mm/h vs. (83.84 ± 47.64) mm/h; SF: (320.21 ± 182.53) μg/L vs. (945.58 ± 604.65) μg/L; IL-6: (50.35 ± 20.54) ng/L vs. (89.35 ± 45.54) ng/L, q = 4.34, 3.42, 11.51, 8.85, all P < 0.05]. IL-10 level in the KD group was significantly higher than that in the SJIA group [(18.52 ± 16.71) ng/L vs. (10.01 ± 3.24) ng/L, q = -5.25, P < 0.05]. WBC, CRP, ESR, PCT, PLT, IL-6, IL-10 and SF in the KD and SJIA groups were significantly higher than those in the control group (all P < 0.05). Conclusion:Detection of CRP, ESR, SF, IL-6, IL-10 in blood can provide objective evidence for the early diagnosis and differential diagnosis of KD and SJIA, thereby reducing the misjudgment of clinical diagnosis.

Changes of humoral and cellular immune indexes in peripheral blood of children with Kawasaki disease at different stages / 中国医师进修杂志

Objective:To investigate the dynamic change characteristics of peripheral blood interferon-γ (INF-γ), interleukin (IL)-4 levels and T helper cell (Th)1/Th2 balance in acute, subacute and restoration stages of children with Kawasaki disease (KD).Methods:Forty-one children with KD received treatment in Women′s and Children′s Hospital Affiliated of School of Medicine University of Electronic Science and Technology of China, Chengdu Women′s and Children′s Central Hospital from May 2017 to January 2020 were enrolled as the observation group, and 41 healthy children examinee from the same period were enrolled as the control group. Children with KD of the observation group were performed with tuberculin pure protein derivative (PPD) test when acute and restoration stage of KD respectively. Peripheral venous blood were drawn from KD children of the observation group in acute, subacute and restoration stage and the control group respectively, serum immune globulin IgG, IgA, IgM and IgE, serum IFN-γ and IL-4 levels were detected by enzyme linked immunosorbent assay (ELISA).Results:Positive rates of PPD test in the restoration stage was higher than that in the acute stage: 65.85%(27/41) vs.17.07%(7/41), there was statistical difference ( χ2 = 20.10, P<0.05). The levels of serum IgG, IgA, IgM and IgE in the acute stage , subacute stage and restoration stagewere gradually decreased ( P<0.05). The levels of serum IgG, IgA, IgM and IgE in the restoration stage and the control group had no significant differences ( P>0.05). The levels of serum IFN-γ and IFN-γ/IL-4 in the acute stage , subacute stage and restoration stage were gradually increased ( P<0.05), the level of IL-4 was gradually decreased ( P<0.05), but the levels of serum IFN-γ, IL-4 and IFN-γ/IL-4 in the restoration stage and the control group had no significant differences ( P>0.05). Conclusions:Among the children with KD in acute stage, serum level of IFN-γ is decreased while serum IL-4 level is increased, and Th1/Th2 balance shifts to Th2. Along with the stabilization of disease, the levels of serum IFN-γ and IL-4 are normalized, and Th1/Th2 balance presents a recovering trend and they almost recover to normal after entering the restoration stage.

Oblivion: autopsy findings of a 31-year-old man with sudden cardiac arrest, a case report of a sequalae of Kawasaki disease

ABSTRACT A 31-year-old man presented to the hospital after suffering a sudden cardiac arrest. Despite optimal therapy, the patient passed away. His medical history included febrile rash at age 2. At autopsy, there was aneurysmal dilation and severe coronary artery stenosis by atherosclerotic plaques and myocardial fibrosis. These findings were presumed to be due to complications of Kawasaki disease, given the remote history of severe febrile rash as a toddler and the presence of chronic coronary artery injury, recanalization, and thrombosis with ischemic heart disease leading to sudden cardiac collapse and death.

Are the clinical features of leprosy and American tegumentary leishmaniasis worse in patients with both diseases?

ABSTRACT This cross-sectional population-based study compared clinical features of leprosy and American tegumentary leishmaniasis (ATL) in patients diagnosed with both diseases (n=414) and in those diagnosed with only leprosy (n=27,790) or only ATL (n=24,357) in Mato Grosso State, which is a hyperendemic area for both diseases in Midwest Brazil. All new cases of leprosy and ATL reported in the area from 2008 to 2017 were included. Patients diagnosed with both diseases were identified by a probabilistic linkage procedure applied to leprosy and ATL databases of the national reporting system. The distribution of the frequency of clinical features between groups was compared by the chi-square test, followed by a multivariate logistic regression. Patients diagnosed with both leprosy and ATL presented higher odds of having nerve damage (OR: 1.34; 95% CI: 1.09-1.66) and leprosy reactions (OR: 1.35; 95% CI: 1.04-1.76) compared to patients diagnosed only with leprosy. Mucocutaneous leishmaniasis (OR: 2.29; 95% CI: 1.74-3.00) was more frequent among patients with both diagnoses when compared to patients who only had ATL. In conclusion, patients diagnosed with both leprosy and ATL present more severe clinical features of such diseases. Our data can be useful for designing health policies aimed at timely and integrated management of leprosy and ATL in co-endemic areas.

Síndrome inflamatorio multisistémico postinfección por SARS-CoV-2: a propósito de un caso clínico / Post SARS-CoV-2 multisystem inflammatory syndrome: a clinical case / Síndrome inflamatória pós-infecção por SARS-CoV-2: relato de caso

En Uruguay, la pandemia por SARS-CoV-2 ha generado menos afectación en pacientes de la edad pediátrica, aumentando el número de casos positivos en este grupo etario de forma proporcional al aumento de la circulación del virus. La forma de presentación es generalmente asintomática o con síntomas respiratorios leves a moderados. El síndrome inflamatorio multisistémico postinfección por SARS-CoV-2 (SIM-C) ha sido descrito como una de las principales complicaciones postinfección. Se describe el primer caso de un paciente con SIM-C en la ciudad de Paysandú, Uruguay. Se trata de un escolar de 6 años que cursó una infección por SARS-CoV-2 un mes previo. Se presenta con un cuadro febril de 4 días de evolución asociado a lesiones de piel e inyección conjuntival y odinofagia, con parámetros inflamatorios elevados y afectación cardiológica. Se traslada a CTI local con buena evolución posterior. El alto índice de sospecha de SIM-C mejora el diagnóstico y en consecuencia la morbimortalidad de la enfermedad.

Summary: In Uruguay, the SARS-CoV-2 pandemic has affected the pediatric population less and the number of positive cases in this age group has increased proportionally to the rise of the virus circulation. The presentation is generally asymptomatic or with mild to moderate respiratory symptoms. Post-Infection Multisystem Inflammatory Syndrome by SARS-CoV-2 (MIS-C) has been described as one of the main post-infection complications. We describe the first case of a patient with MIS-C in the city of Paysandú, Uruguay. It is a 6-year-old schoolboy who had had a SARS-CoV-2 infection a month earlier. He showed a 4-day history of fever associated with skin lesions and conjunctival injection and odynophagia, with high inflammatory parameters and cardiac involvement. He was transferred to a local ICU and had a good subsequent evolution. The high index of suspicion of MIS-C improves the diagnosis and consequently the morbidity and mortality rates of the disease.

No Uruguai, a pandemia de SARS-CoV-2 gerou menos afetação em pacientes pediátricos, e o número de casos positivos nessa faixa etária aumentou proporcionalmente ao aumento da circulação do vírus. A forma de apresentação é geralmente assintomática ou com sintomas respiratórios leves a moderados. A Síndrome Inflamatória Multissistêmica Pós-Infecção por SARS-CoV-2 (MIS-C) tem sido descrita como uma das principais complicações pós-infecção. Descreve-se o primeiro caso de paciente com MIS-C na cidade de Paysandú, Uruguai. Ele é um estudante de 6 anos de idade que tinha tido uma infecção por SARS-CoV-2 um mês antes. Apresentou história de febre de 4 dias associada a lesões cutâneas e hiperemia conjuntival e odinofagia, com parâmetros inflamatórios elevados e envolvimento cardiológico. Foi transferido para uma UTI local com boa evolução posterior. O alto índice de suspeita de MIS-C melhora o diagnóstico e, consequentemente, a morbimortalidade da doença.

Dermatoses in Human Immunodeficiency Virus Infected Patients with A Focus on Infections: A 12-month Cross-sectional Study in Hospital Sungai Buloh

Background@#Cutaneous disorders are common clinical manifestations of the Human Immunodeficiency Virus (HIV). In the era of antiretroviral therapy (ART), the spectrum of cutaneous disorders in HIV-infected patients has changed. We assessed the types of dermatoses, including cutaneous infections in HIVpositive patients and the association between the peripheral CD4 cell count and the severity of skin infection.@*Methods@#All HIV-positive patients referred to the Dermatology Department of Hospital Sungai Buloh from January 2021 – December 2021 were enrolled in a prospective cross-sectional study. Patients were subjected to a complete medical and physical examination and appropriate investigation to confirm the diagnosis. @*Results@#A total of 112 (92.6%) male and 9 (7.4%) female patients with a mean age of 38.76 ± SD years participated. The majority of patients were Malay (56.2%), with MSM (54.5%) being the commonest mode of transmission. 65.2% of patients had CD4≥350 cells/ mm3 and 86.7% of patients were on ART. Infections (56.1%) were the most common group of mucocutaneous manifestations, with 45.6% of these due to viral infections. There was no statistically significant correlation between the CD4 count and the severity of skin involvement in bacterial (p=0.302), viral (p=0.145) and fungal (p=0.533) infections.@*Conclusion@#Viral infection were the commonest cutaneous manifestations in HIV- positive patients. The frequency and severity of the cutaneous infections were much more common in patients with more advanced immunosuppression.

Enfermedad de Kawasaki. Presentación de dos casos

RESUMEN La enfermedad de Kawasaki es una condición médica poco frecuente. Los niños pequeños son los más afectados y las complicaciones cardiovasculares de la enfermedad son las más temidas. Se describieron en este artículo dos casos diagnosticados en el servicio de Pediatría del Hospital General Docente de Placetas, Villa Clara. Ambos pacientes, preescolares, presentaron síntomas comunes, como: fiebre de más de 5 días, adenopatías, conjuntivitis no supurativa, edemas del dorso de las manos y pies, lengua aframbuesada y rash. Los elementos clínicos fueron, una vez más, la piedra angular para el diagnóstico. Ambos pacientes evolucionaron favorablemente al tratamiento empleado y no desarrollaron formas graves, ni secuelas de la enfermedad.

ABSTRACT Kawasaki disease is a rare medical condition. Young children are the most affected and the cardiovascular complications of the disease are feared the most. Two cases diagnosed in the pediatric service of the Hospital General Docente de Placetas, Villa Clara, were described in this article. Both patients, preschoolers, presented common symptoms, such as: fever for more than 5 days, lymphadenopathy, non-suppurative conjunctivitis, edema of the back of the hands and feet, a strawberry tongue and rash. The clinical elements were, once again, the cornerstone for the diagnosis. Both patients evolved favorably to the treatment used and did not develop serious forms or sequelae of the disease.

RESUMO A doença de Kawasaki é uma condição médica rara. As crianças pequenas são as mais afetadas e as complicações cardiovasculares da doença são as mais temidas. Neste artigo foram descritos dois casos diagnosticados no Serviço de Pediatria do Hospital General Docente Universitario de Placetas, Villa Clara. Ambos os pacientes, pré-escolares, apresentavam sintomas comuns, como: febre há mais de 5 dias, linfadenopatia, conjuntivite não supurativa, edema de dorso de mãos e pés, língua ramificada e erupção cutânea. Os elementos clínicos foram, mais uma vez, a pedra angular para o diagnóstico. Ambos os pacientes evoluíram favoravelmente ao tratamento utilizado e não desenvolveram formas graves ou sequelas da doença.