Luisa Berenise Gámez-González

Clinical manifestations associated with Kawasaki disease shock syndrome in Mexican children

Recently, there have been increasing reports of severe forms of Kawasaki disease (KD) associated with shock that have been managed in pediatric intensive care units. It has been suggested that KD is more severe in the Hispanic population. We conducted a study to determine the frequency of Kawasaki disease shock syndrome (KDSS) in our population and compared characteristics between patients with KD without shock and patients with KDSS. Data from 214 patients with KD treated in a tertiary pediatric hospital were collected during a 12-year period. We compared clinical and laboratory features of KD patients without shock and KDSS patients. Of 214 consecutive patients with KD, 11 (5xa0%) met the definition for KDSS. All of these patients received fluid resuscitation, seven (64xa0%) required inotropic treatment, and six (54xa0%), ventilatory support. On admission, seven of these patients (64xa0%) had an incomplete presentation of the disease, whereas in the group of patients without shock, the relative frequency of an incomplete presentation was 29xa0%. Twenty percent (3/11) of patients with KDSS presented giant coronary aneurysms versus none of 203 KD patients without shock (pu2009=u20090.001); myocardial infarction, 27xa0% (3/11), versus 1xa0% (2/203) (pu2009=u20090.001); and intravenous immunoglobulin (IVIG) resistance, 60xa0% (6/11), versus 12xa0% (24/203). Gastrointestinal manifestations in the acute phase occurred in 91xa0% of KDSS patients versus 30xa0% patients without shock (pu2009=u20090.001). Conclusion. Patients with KD presenting in shock seem to have an increase in gastrointestinal manifestations, incomplete presentation, IVIG resistance, and worse cardiac outcomes. Larger, prospective, multicentre studies should be carried out to corroborate these findings.

BCG and Kawasaki disease in Mexico and Japan

ABSTRACT Dr. Tomisaku Kawasaki was the first to describe BCG reactivation in Kawasaki Disease (KD), and this sign is present in about 30–50% of KD patients. It is a very specific early sign of the disease and although it has been recognized for decades, its pathophysiology continues to be an enigma. Recently, Yamada et al. reported a severe BCG reaction with tuberculid in 2 Japanese KD patients. We present 2 cases with KD and severe BCG reaction, one from Japan and the other from Mexico and review the policies of administration of BCG in both countries. The BCG vaccine has a worldwide coverage of 88%. Differences in BCG strains and methods of administration may influence BCG reactions in KD. The BCG reaction in the inoculation site may represent the most useful sign in KD.

Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease

Kawasaki disease shock syndrome (KDSS) is an uncommon presentation of Kawasaki disease (KD). KDSS has been associated with more severe markers of inflammation, coronary abnormalities and i.v. immunoglobulin (IVIG) resistance.

IgG levels in Kawasaki disease and its association with clinical outcomes

Previous studies have suggested an association of IgG levels (before and after IVIG infusion) with clinical outcomes in Kawasaki disease. A retrospective analysis was performed that included 418 patients with KD admitted to Tokyo Women’s Medical University Yachiyo Medical Center to evaluate pre- and post-IVIG IgG levels and its relation to outcomes. All patients received an initial IVIG infusion and aspirin; IgG levels were measured in 350 patients before IVIG (pre-IVIG IgG levels) and in 373 patients 48xa0h after starting IVIG infusion (post-IVIG IgG levels). Media and standard deviation of the pre- and post-IVIG IgG levels were reported and classified according to age. Also, IgG z-scores were calculated according to normal values of IgG by age. The number of cases and corresponding percentage of non-responders were reported by age and total patients. The association of pre-IVIG, post-IVIG IgG levels and post-IVIG IgG level/pre-IVIG IgG level ratio with no-response was evaluated by simple logistic regression model based on the IgG z-score, and regression coefficient, X2 value, p, and R2 of Nagelkerke were reported. Pre-IVIG and post-IVIG IgG levels presented an association with non-responders with statistical significance. This association was more evident between post-IVIG IgG levels and non-responders. Regarding coronary alterations, it was not possible to perform an adequate statistical analysis due the small number of patients. Pre- and post-IVIG infusion IgG levels could be an important biomarker in KD as well as in other inflammatory conditions. Higher IgG levels could be associated with a more effective immunomodulatory action and associated with better clinical outcomes.

Vital Signs as Predictor Factors of Intravenous Immunoglobulin Resistance in Patients With Kawasaki Disease

Kawasaki disease (KD) is the most common cause of acquired heart disease in children. Intravenous immunoglobulin (IVIG) may significantly lower the frequency of coronary artery complications. However, some patients do not respond to initial therapy and are at higher risk of developing coronary artery lesion. A retrospective analysis of data from 419 KD patients was performed. The patients were divided into IVIG responders (n = 318) and IVIG nonresponders (n = 101). Multivariate logistic regression analysis revealed neutrophil percentage, albumin, aspartate aminotransferase, heart rate, and body temperature were independent predictors of IVIG resistance. We generated a predictive scoring system by assigning 1 point for the presence of these parameters (neutrophil >80%, albumin <3.4 g/dL, aspartate aminotransferase >100 IU/L, heart rate >146 bpm, and body temperature >38.8°C). This scoring system had a sensitivity of 76.2% and specificity of 64.8%, and a positive predictive value of 40.1% and a negative predictive value of 89.4%. Vital signs may be helpful to detect KD patients with IVIG resistance.