Lung Function Evaluated By Structured Light Plethysmography in Children After Lung Surgery: A Preliminary Analysis.

Background: Structured light plethysmography (SLP) is a novel light-based method that captures chest wall movements to evaluate tidal breathing. Methods: Thirty-two children who underwent lung surgery were enrolled. Their clinical history was collected along with spirometry and SLP. Results: Median age of surgery was 9 months (interquartile range 4-30). Most frequent diagnosis was congenital pulmonary airway malformation (14/32), then pulmonary sequestration (9/32), tumor (5/32), and bronchogenic cyst (4/32). The most frequent surgical approach was lobectomy (59%), segmentectomy (38%), and complete resection (3%). More than 80% had surgery when younger than 3 years of age. Eight patients had short-term complications (pleural effusion was the most frequent), while long-term effects were reported in 15 patients (19% recurrent cough, 13% thoracic deformities, 13% airway infections, 9% wheezing, 6% reduced exercise tolerance, and 3% columnar deformities). Spirometry was normal in 9/22 patients. Nine patients had a restrictive pattern, while 4 showed a mild bronco-reactivity. Ten patients did not perform spirometry because of young age. SLP revealed the presence of obstructive pattern in 10% of patients (IE50 > 1.88) and showed a significant difference between the two hemithorax in 29% of patients. Discussion: SLP may be a new method to evaluate lung function, without collaboration and radiation exposure, in children who underwent lung resection, also in preschool age.

Propranolol for the treatment of infantile hemangiomas: a nine-year monocentric experience from a tertiary hospital.

BACKGROUND: Propranolol is currently considered the first-line therapy for problematic infantile hemangiomas (IH), the most common benign vascular neoplasm of infancy. OBJECTIVES: We present a retrospective observational study aimed at assessing the efficacy of propranolol in 44 IH patients. MATERIALS & METHODS: A nine-year retrospective review considering clinicodemographical and therapy-related variables was performed on medical records of infants treated for IH with oral propranolol. Each lesion was assessed through a numeric severity score based on size and colour both at baseline and after treatment conclusion (p <0.05 was considered statistically significant). RESULTS: Complete remission was achieved in 90.7% cases of IH with a general mean improvement in severity of 94.94%. No severe adverse effects were reported. Preterm patients showed a superior response compared to term infants, even though the difference was not significant (p=0.185). CONCLUSION: Propranolol showed high efficacy in terms of safety profile and cosmetic results. Prematurity and precocious therapy could be linked to a superior response.

Immune checkpoint inhibitors and Carbon iON radiotherapy In solid Cancers with stable disease (ICONIC).

ICONIC is a multicenter, open-label, nonrandomized phase II clinical trial aiming to assess the feasibility and clinical activity of the addition of carbon ion radiotherapy to immune checkpoint inhibitors in cancer patients who have obtained disease stability with pembrolizumab administered as per standard-of-care. The primary end point is objective response rate, and the secondary end points are safety, survival and disease control rate. Translational research is an exploratory aim. The planned sample size is 27 patients. The study combination will be considered worth investigating if at least four objective responses are observed. If the null hypothesis is rejected, ICONIC will be the first proof of concept of the feasibility and clinical activity of the addition of carbon ion radiotherapy to immune checkpoint inhibitors in oncology.

ICONIC is a multicenter, open-label, nonrandomized, phase II clinical trial aiming to evaluate the feasibility and clinical activity of the addition of carbon ion radiotherapy to immune checkpoint inhibitors in cancer patients who have obtained disease stability with pembrolizumab administered as per standard-of-care. Considering that no clinical trials have been conducted thus far to assess the safety of the association between immune checkpoint inhibitors and carbon ion radiotherapy, the current clinical study will provide controlled data about the safety of this unprecedented therapeutic combination. Clinical Trial Registration: NCT05229614 (ClinicalTrials.gov).

Endoscopic ultrasound diagnostic gain over computed tomography and magnetic resonance cholangiopancreatography in defining etiology of idiopathic acute pancreatitis.

BACKGROUND: About 10%-30% of acute pancreatitis remain idiopathic (IAP) even after clinical and imaging tests, including abdominal ultrasound (US), contrast-enhanced computed tomography (CECT) and magnetic resonance cholangiopancreatography (MRCP). This is a relevant issue, as up to 20% of patients with IAP have recurrent episodes and 26% of them develop chronic pancreatitis. Few data are available on the role of EUS in clarifying the etiology of IAP after failure of one or more cross-sectional techniques. AIM: To evaluate the diagnostic gain after failure of one or more previous cross-sectional exams. METHODS: We retrospectively collected data about consecutive patients with AP and at least one negative test between US, CECT and MRCP, who underwent linear EUS between January 2017 and December 2020. We investigated the EUS diagnostic yield and the EUS diagnostic gain over different combinations of these cross-sectional imaging techniques for the etiologic diagnosis of AP. Types and frequency of EUS diagnosis were also analyzed, and EUS diagnosis was compared with the clinical parameters. After EUS, patients were followed-up for a median of 31.5 mo to detect cases of pancreatitis recurrence. RESULTS: We enrolled 81 patients (63% males, mean age 61 ± 18, 23% with previous cholecystectomy, 17% with recurrent pancreatitis). Overall EUS diagnostic yield for AP etiological diagnosis was 79% (20% lithiasis, 31% acute on chronic pancreatitis, 14% pancreatic solid or cystic lesions, 5% pancreas divisum, 5% autoimmune pancreatitis, 5% ductal abnormalities), while 21% remained idiopathic. US, CECT and MRCP, taken alone or in combination, led to AP etiological diagnosis in 16 (20%) patients; among the remaining 65 patients, 49 (75%) obtained a diagnosis at EUS, with an overall EUS diagnostic gain of 61%. Sixty-eight patients had negative US; among them, EUS allowed etiological diagnosis in 59 (87%). Sixty-three patients had a negative CECT; among them, 47 (74%) obtained diagnosis with EUS. Twenty-four had a negative MRCP; among them, 20 (83%) had EUS diagnosis. Twenty-one had negative CT + MRCP, of which 17 (81%) had EUS diagnosis, with a EUS diagnostic gain of 63%. Patients with biliary etiology and without previous cholecystectomy had higher median values of alanine aminotransferase (154 vs 25, P = 0.010), aspartate aminotransferase (95 vs 29, P = 0.018), direct bilirubin (1.2 vs 0.6, P = 0.015), gamma-glutamyl transpeptidase (180 vs 48, P = 0.006) and alkaline phosphatase (150 vs 72, P = 0.015) Chronic pancreatitis diagnosis was more frequent in patients with recurrent pancreatitis at baseline (82% vs 21%, P < 0.001). During the follow-up, AP recurred in 3 patients, one of which remained idiopathic. CONCLUSION: EUS is a good test to define AP etiology. It showed a 63% diagnostic gain over CECT + MRCP. In suitable patients, EUS should always be performed in cases of IAP. Further prospective studies are needed.

Cardiac dysfunction in Multisystem Inflammatory Syndrome in Children: An Italian single-center study.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a novel condition temporally associated with SARS-CoV2 infection. Cardiovascular involvement is mainly evident as acute myocardial dysfunction in MIS-C. The aim of this study was to describe the cardiac dysfunction in patients with MIS-C, defining the role of severity in the clinical presentations and outcomes in a single cohort of pediatric patients. METHODS: A single-center retrospective study on patients diagnosed with MIS-C, according to the Center for Disease Control and Prevention (CDC) definition, and referred to Vittore Buzzi Children's Hospital in Milan from November 2020 to February 2021. Patients were managed according to a local approved protocol. According to the admission cardiac left ventricular ejection fraction (LVEF), the patients were divided into group A (LVEF < 45%) and group B (LVEF ≥45%). Pre-existing, clinical, and laboratory factors were assessed for evaluating outcomes at discharge. RESULTS: Thirty-two patients were considered. Cardiac manifestations of MIS-C were reported in 26 patients (81%). Group A included 10 patients (9 M/1F, aged 13 years [IQR 5-15]), and group B included 22 patients (15 M/7 M, aged 9 years [IQR 7-13]). Significant differences were noted among clinical presentations (shock, diarrhea, intensive care unit admission), laboratory markers (leucocytes, neutrophils, and protein C-reactive), and cardiac markers (troponin T and N-terminal pro B-type Natriuretic Peptide) between the groups, with higher compromission in Group A. We found electrocardiogram anomalies in 14 patients (44%) and rhythm alterations in 3 patients (9%), without differences between groups. Mitral regurgitation and coronary involvement were more prevalent in group A. Total length of hospital stay and cardiac recovery time were not statistically different between groups. A recovery of cardiac functioning was reached in all patients. CONCLUSION: Despite significant differences in clinical presentations and need for intensive care, all of the MIS-C patients with significant cardiac involvement in this study completely recovered. This suggests that the heart is an involved organ and did not influence prognosis if properly treated and supported in the acute phase.

Baseline Amino Acid Substitutions in the NS5A ISDR and PKR Binding Domain of Hepatitis C and Different Fibrosis Levels and Levels of Development of Hepatocellular Carcinoma in Patients Treated with DAAs.

Variations in the interferon sensitivity-determining region (ISDR) within the NS5A region were related to the development of hepatocellular carcinoma (HCC) in patients infected with hepatitis C virus (HCV). The aim of the study was to investigate a relationship between ISDR/PKR substitutions and their association with liver fibrosis or HCC development. A total of 316 patients infected with HCV and treated with DAAs were evaluated. HCV RNA was quantified and sequenced before treatment. The liver fibrosis stage was assessed by transient elastography and equalized to METAVIR scores. Multivariate analysis showed that ≥3 substitutions in ISDR and ≥6 in PKR-bd were significantly associated with advanced fibrosis. Advanced fibrosis was observed in patients with higher substitutions in ISDR and PKR-bd. A higher correlation between advanced fibrosis and a high frequency of ≥3 substitutions in ISDR and ≥6 in PKR-bd was observed in patients infected with genotype 2c. In addition, in a higher proportion of HCC patients, advanced fibrosis (40.4% vs. 88.2%; p < 0.001) and ≥6 substitutions in PKR-bd (15.4% vs. 41.2%; p = 0.01) was observed. In conclusion, a higher number of substitutions in ISDR and PKR-bd were associated with advanced liver fibrosis, suggesting a use of like predictors for progression in the liver damage. A significantly higher number of PKR-bd substitutions was observed in HCC patients; in particular, in patients infected with HCV genotype 2c.