Gastrointestinal presentation of kawasaki disease: A red flag for severe disease?

Background Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology and the main cause of acquired heart disease among children in the developed world. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease. Objective To evaluate whether presenting abdominal manifestations identify a group at major risk for Intravenous immunoglobulin (IVIG)-resistance and coronary lesions. Methods Retrospective study of KD patients diagnosed between 2000 and 2015 in 13 pediatric units in Italy. Patients were divided into 2 groups according to the presence or absence of abdominal manifestations at onset. We compared their demographic and clinical data, IVIG-responsiveness, coronary ectasia/aneurysms, laboratory findings from the acute and subacute phases. Results 302 patients (181 boys) were enrolled: 106 patients with, and 196 patients without presenting abdominal features. Seasonality was different between the groups (p = 0.034). Patients with abdominal manifestations were younger (p = 0.006) and more frequently underwent delayed treatment (p = 0.014). In the acute phase, patients with abdominal presentation had higher platelet counts (PLT) (p = 0.042) and lower albuminemia (p = 0.009), while, in the subacute phase, they had higher white blood cell counts (WBC) and PLT (p = 0.002 and p < 0.005, respectively) and lower red blood cell counts (RBC) and hemoglobin (Hb) (p = 0.031 and p 0.009). Moreover, the above mentioned group was more likely to be IVIG-resistant (p < 0.005) and have coronary aneurysms (p = 0.007). In the multivariate analysis, presenting abdominal manifestations, age younger than 6 months, IVIG- resistance, delayed treatment and albumin concentration in the acute phase were independent risk factors for coronary aneurysms (respectively p<0.005, <0.005, = 0.005 and 0.009). Conclusions This is the first multicenter report demonstrating that presenting gastrointestinal features in KD identify patients at higher risk for IVIG-resistance and for the development of coronary aneurysms in a predominantly Caucasian population

Extreme thrombocytosis in systemic juvenile idiopathic arthritis. A case report

A case of systemic onset juvenile idiopathic arthritis complicated by severe thrombocytosis treated with the interleukin 1 beta receptor antagonist with success is reported. Serial measurement of interleukin 1 beta plasma level confirmed the pathogenetic role of IL-1 beta in causing thromocytosi

Sustainable Restoration Guided by Scientific and Archival Investigations: The Bio-Cleaning of Lorenzo Duke of Urbino’s Sarcophagus, a Michelangelo’s Masterpiece in the Medici Chapels

The masterpiece this work deals with is the tomb of Lorenzo de’ Medici, Duke of Urbino, completed by Michelangelo around 1533 in the New Sacristy of Medici chapel in Florence. Sacristy underwent an extensive restoration (2013–2020) and Lorenzo’s sarcophagus was addressed in the last phase (2019–2020). The unique history and the mediocre state of conservation of the marble may be related to body decomposition processes that, according to the documentary research, required a scientific approach to tailor an appropriate intervention. The sarcophagus underwent a bio-cleaning procedure, using bacteria belonging to the ENEA-MIRRI (Microbial Resource Research Infrastructure Italian Node) collection. Spectroscopic analysis (FTIR in ATR and in TR—total reflection—mode and XRF) and microscopic analyses allowed a correct diagnosis of the composition of the coherent deposits, guiding the selection of appropriate bacterial strains. Deposits were composed of gypsum, calcium oxalate, calcium phosphates and proteins. Following a laboratory screening and a preliminary test on-site, the three strains, Serratia ficaria SH7, Pseudomonas stutzeri CONC11 and Rhodococcus sp. ZCONT, immobilised in an appropriate supportant gel, were selected by the quality of cleaning obtained. Two applications of the micro-packs, containing each individual strain, were able to remove the centuries-old deposits within 48 h. The bio-cleaning was selective, gradual and respectful of the marble, in accordance with the restorers’ opinion and reflecting the principles of sustainability

Inability of Asian risk scoring systems to predict intravenous immunoglobulin resistance and coronary lesions in Kawasaki disease in an Italian cohort

Since resistance to intravenous immunoglobulin (IVIG) is associated with coronary lesions (CALs) in Kawasaki disease (KD), it is crucial to identify patients at risk to protect them from coronary involvement. The available risk scores to predict IVIG resistance were developed in Asian populations in whom their effectiveness has been proven, but data on non-Asian children are limited. The aim of this study is to evaluate the ability of the Kobayashi, Egami, and Formosa risk scores to predict IVIG resistance and CALs in Italian patients with KD. A multicenter retrospective analysis involving children with KD diagnosed between 2000 and 2015 was carried out: 257 patients were enrolled (57.9% boys, 89.9% Caucasian); 43 patients were IVIG resistant (16.7%). The scores have low sensitivity and specificity in predicting IVIG resistance: respectively, KS 64% and 62.5%, ES 41.4% and 77.4%, and FS 70.8% and 44.9%. The predictive value of the 3 scores for predicting CALs was also poor. Conclusion: Kobayashi, Egami, and Formosa Scores are ineffective in predicting IVIG resistance and coronary involvement in a predominantly Caucasian cohort. A specific score system for mostly Caucasian children with KD is needed enable the early identification of those at risk for CALs who could benefit from intensified treatment.What is Known:\u2022 There are several risk scores developed in the Asian population to early identify patients with KD at risk for immunoglobulin-resistance and thus for coronary lesions.\u2022 Data are scarce on their effectiveness in non-Asian children.What is New:\u2022 We present a comprehensive analysis of the ability of 3 Asian risk scores in a cohort of mostly Caucasian children to predict immunoglobulin resistance and coronary involvement.\u2022 Low sensitivity and specificity of the Asian scores for immunoglobulin-resistance and coronary lesions suggest the need for criteria specific for different ethnicities