Pediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2

Objectives To know the clinical presentation and outcome of children with pediatric infammatory multisystem syndrome temporally associated with SARS-CoV- 2 (PIMS-TS) at a pediatric tertiary care center in Chennai. Methods Clinical and biochemical parameters of 65 children with PIMS-TS treated between July and October 2020 were studied. All children had their COVID RT-PCR and IgG COVID antibodies tests done. Results Mean age of the study group was 5.65±3.68 y. Fever with red eyes, rash, vomiting, abdominal pain, and shock were common presenting features. Sixty percent of the study group had Kawasaki/incomplete Kawasaki features. Sixty-seven percent of the study group had coronary dilatation, 41% presented with shock, and 25% had left ventricular dysfunction. Coronary aneurysms were documented in 58% of the study group (z score more than 2.5). Respiratory presentation with pneumonia was seen in 10%. Four children presented with acute abdomen. Acute kidney injury, acute liver failure, hemolysis, pancytopenia, macrophage activation syndrome, encephalopathy, and multiorgan dysfunction syndrome (MODS) were other features. Forty-three percent required noninvasive oxygen support and 15.4% required mechanical ventilation. Intravenous immunoglobulin (73.8%) and methylprednisolone (49.8%) were used for therapy. Mortality in the study was 6%, which was due to MODS. Conclusions Acute febrile illness with mucocutaneous and gastrointestinal manifestations should have PIMS-TS as a possible diferential diagnosis and needs evaluation with infammatory markers and SARS-CoV-2 antibodies.

Steroid Pulse Therapy for Kawasaki Disease Complicated with Myocarditis.

Background: The clinical management of intravenous immunoglobulin-resistant Kawasaki disease shock syndrome (KDSS) is obscure. Case characteristics: Three children presented with intravenous immunoglobulin-resistant KDSS complicated with myocarditis. Outcome: All cases were successfully managed with steroid pulse therapy. Message: Steroid pulse therapy is effective in immunoglobulin-resistant KDSS.

Mercury Promotes Catecholamines Which Potentiate Mercurial Autoimmunity and Vasodilation: Implications for Inositol 1,4,5-Triphosphate 3-Kinase C Susceptibility in Kawasaki Syndrome

Previously, we reviewed biological evidence that mercury could induce autoimmunity and coronary arterial wall relaxation as observed in Kawasaki syndrome (KS) through its effects on calcium signaling, and that inositol 1,4,5-triphosphate 3-kinase C (ITPKC) susceptibility in KS would predispose patients to mercury by increasing Ca2+ release. Hg2+ sensitizes inositol 1,4,5-triphosphate (IP3) receptors at low doses, which release Ca2+ from intracellular stores in the sarcoplasmic reticulum, resulting in delayed, repetitive calcium influx. ITPKC prevents IP3 from triggering IP3 receptors to release calcium by converting IP3 to inositol 1,3,4,5-tetrakisphosphate. Defective IP3 phosphorylation resulting from reduced genetic expressions of ITPKC in KS would promote IP3, which increases Ca2+ release. Hg2+ increases catecholamine levels through the inhibition of S-adenosylmethionine and subsequently catechol-O-methyltransferase (COMT), while a single nucleotide polymorphism of the COMT gene (rs769224) was recently found to be significantly associated with the development of coronary artery lesions in KS. Accumulation of norepinephrine or epinephrine would potentiate Hg2+-induced calcium influx by increasing IP3 production and increasing the permeability of cardiac sarcolemma to Ca2+. Norepinephrine and epinephrine also promote the secretion of atrial natriuretic peptide, a potent vasodilator that suppresses the release of vasoconstrictors. Elevated catecholamine levels can induce hypertension and tachycardia, while increased arterial pressure and a rapid heart rate would promote arterial vasodilation and subsequent fatal thromboses, particularly in tandem. Genetic risk factors may explain why only a susceptible subset of children develops KS although mercury exposure from methylmercury in fish or thimerosal in pediatric vaccines is nearly ubiquitous. During the infantile acrodynia epidemic, only 1 in 500 children developed acrodynia whereas mercury exposure was very common due to the use of teething powders. This hypothesis mirrors the leading theory for KS in which a widespread infection only induces KS in susceptible children. Acrodynia can mimic the clinical picture of KS, leading to its inclusion in the differential diagnosis for KS. Catecholamine levels are often elevated in acrodynia and may also play a role in KS. We conclude that KS may be the acute febrile form of acrodynia.

Diagnosis of incomplete Kawasaki disease / 소아과

Several authors suggested that the clinical characteristics of incomplete presentation of Kawasaki disease are similar to those of complete presentation and that the 2 forms of presentation are not separate entities. Based on this suggestion, a diagnosis of incomplete Kawasaki disease in analogy to the findings of complete presentation is reasonable. Currently, the diagnosis of incomplete Kawasaki disease might be made in cases with fewer classical diagnostic criteria and with several compatible clinical, laboratory or echocardiographic findings on the exclusion of other febrile illness. Definition of incomplete presentation in which coronary artery abnormalities are included as a necessary condition, is restrictive and specific. The validity of the diagnostic criteria of incomplete presentation by the American Heart Association should be thoroughly tested in the immediate future.

Diagnosis of incomplete Kawasaki disease / 소아과

Several authors suggested that the clinical characteristics of incomplete presentation of Kawasaki disease are similar to those of complete presentation and that the 2 forms of presentation are not separate entities. Based on this suggestion, a diagnosis of incomplete Kawasaki disease in analogy to the findings of complete presentation is reasonable. Currently, the diagnosis of incomplete Kawasaki disease might be made in cases with fewer classical diagnostic criteria and with several compatible clinical, laboratory or echocardiographic findings on the exclusion of other febrile illness. Definition of incomplete presentation in which coronary artery abnormalities are included as a necessary condition, is restrictive and specific. The validity of the diagnostic criteria of incomplete presentation by the American Heart Association should be thoroughly tested in the immediate future.

Enfermedad de Kawasaki en niños hospitalizados en cinco centros de Barranquilla, Colombia, 2002-2008 / Kawasaki disease in children admitted to five centers in Barranquilla, Colombia, 2002-2008

Introducción: La enfermedad de Kawasaki se ha convertido en la principal causa de cardiopatíaadquirida en los niños. Sin embargo,aunque existen criterios diagnósticos bien definidos, éstos pueden presentarse dispersos a lo largo del curso de la enfermedad o simulardiferentes enfermedades, lo que ocasiona retraso en el diagnóstico y, con esto, mayor riesgo de afección cardiaca. El objetivo de este estudio fue reconocer las características clínicas y epidemiológicas de la enfermedad de Kawasaki en los niños hospitalizados en cinco centros de Barranquilla, entre 2002 y 2008. Pacientes y métodos: Se trata de un estudio retrospectivo, a partir de las historias clínicas de los pacientes menores de 14 añoshospitalizados en cinco centros asistenciales (Hospital Pediátrico, Hospital Niño Jesús, Clínica del Caribe, Clínica La Asunción y Clínica La Merced) de Barranquilla (Atlántico) desde el 1º de febrero de 2002 hasta el 31 de mayo de 2008, con diagnóstico de enfermedadde Kawasaki. Se estimaron parámetrosdescriptivos y prueba no paramétrica(test de los signos). Resultados: El 40% de los casos ocurrió en menores de un año. El 65% de los casos fueron niños procedentes del área urbana. La fiebre fue el principal motivo de consulta en 65% de los casos y el 95% se clasificó como enfermedad típica. Las alteraciones cardíacas se presentaron en el 30%. Los exámenes determinantes para medir la efectividad del tratamiento fueron los cambios en el recuento de plaquetas, la velocidad de sedimentación globular y la prueba de la proteína C reactiva (PCR). Conclusiones: La enfermedad de Kawasaki en nuestro medio presenta características clínicas y epidemiológicas similares a lasdescritas en otras latitudes. Se observaron variaciones en la velocidad de sedimentación globular, plaquetas y PCR en aquellos sujetos que recibieron gammaglobulina. La afección cardiaca es frecuente y se relaciona con la edad, el retraso en el diagnóstico y eltratamiento oportuno.

Introduction: Kawasaki disease has become the leading cause of acquired heart disease in children. However, although there are well-defined diagnostic criteria, these can occur scattered throughout the course of the disease or simulate different pathologiesleading to delayed diagnosis and increased risk of a heart condition. The purpose of this study is to recognize the clinical and epidemiologicalcharacteristics of Kawasaki diseasein children admitted to five medical centers in Barranquilla from 2002 to 2008. Patients and Methods: This is a retrospective study based on the medical records of patients less than 14 years old hospitalized in five medical centers in the city of Barranquilla, Atlántico, (Pediatric Hospital, Hospital Niño Jesús, Clinics: Caribbean, Assumption and La Merced) and diagnosed with Kawasaki disease from February 1, 2002 to May 31, 2008. Descriptive statistical parameters and non-parametric tests (sign test) have been estimated. Results: Forty percent of the cases occurred in infants less than one year old. Sixty percent (65%) of the patients were male children from the urban area. Fever was the main reason for consulting in 65% of the cases, and 95% of the patients were classified as having the typical disease. Ultrasonographic cardiac abnormalities were documented in 30% of the instances. The test measures for determining the effectiveness of treatment were based on changes in theplatelet count, the sedimentation rate, and the C-reactive protein (CRP).Conclusions: Kawasaki disease in ourenvironment shows clinical and epidemiological features similar to those described in other latitudes. There are variations in the sedimentation rate (ESR), platelet count, and CRP in those subjects who received intravenousimmunoglobulin. The heart conditionis common and is associated to age, delayed diagnosis, and timely treatment.

Doppler echocardiographic assessment of left ventricular function in Kawasaki syndrome

To investigate the effect of Kawasaki syndrome on left ventricular function, we studied 52 patients with Kawasaki syndrome at initial visit and after 3 months (36 patients). Using Pulsed Doppler echocardiogram,we obtained aortic velocity (peak and mean), acceleration time(AT),ejection time(ET), ratio of AT to ET(AT/ET), acceleration (peak and mean) and velocity time integral and mitral velocity of E and A waves(peak and mean) and velocity time integral. Mitral time for peak velocity time integral. Mitral time for peak velocity was significantly prolonged in Kawasaki syndrome,being a mean(+/-SD) of 66.2(+/-14) msec in the control group, 79.2(+/-13)msec at initial vist(p<0.05) and 79.4(+/-13) msec after 3 months (p<0.05). Aortic peak acceleration was significantly decreased in Kawasaki syndrome being a mean(+/-SD) of 2590(+/-785) cm/sec2 after 3 months (P<0.05).Aortic mean acceleration was also significantly decreased in Kawasaki syndrome being a mean(+/-SD) of 1575( +/-542)cm/sec2 in the control group, 1198(+/-351)cm/sec2at initial visit(p<0.05)and 1124 +/-275cm/sec2 after 3 months(p<0.01). Aortic acceleration time was significantly prolonged in Kawasaki svndrome being a mean(+/-SD) of 62(+/-13) msec in the control group, 72(+/-13) msec at initial visit(p<0.05) and 76(+/-16) msec in the control group, 72(+/-13) msec at initial visit(p<0.05) and 76(+/-16) msec after 3 months (p<0.01). We conclude that early abnormalities of left ventricular function, as assessed by echocardiograpy,gencrally persist after 3 months of onset.