Early Effects of Elexacaftor-Tezacaftor-Ivacaftor Therapy on Magnetic Resonance Imaging in Patients with Cystic Fibrosis and Advanced Lung Disease.

This is a prospective, observational study involving three Cystic Fibrosis (CF) adult patients, evaluating the changes in chest magnetic resonance imaging (MRI) three months after the start of elexacaftor/tezacaftor and ivacaftor therapy. MRI showed a drastic reduction in mucus plugging and bronchial wall thickening, with an improvement in the diffusion-weighted MRI score. Similarly, a marked improvement in spirometric parameters, nutritional status, and sweat chloride was observed. Our preliminary data confirm that chest MRI could be a useful tool to assess disease progression in CF patients on modulatory drug therapy.

Validity of epicardial fat volume as biomarker of coronary artery disease in symptomatic individuals: Results from the ALTER-BIO registry.

BACKGROUND: To determine if an increased epicardial fat volume (EFV) is associated with coronary artery disease (CAD) in individuals with symptoms of cardiovascular (CV) disease. METHODS: Coronary Computed Tomographic Angiography (CCTA), demographic and clinical variables of 1344 individuals were retrieved: semi-automated measurements for EFV and coronary artery calcifications (CAC) were obtained. Individuals were grouped into three categories according to the presence of CAD, resulting in absent (CAD0), non-obstructive (CAD1) or obstructive (CAD2) disease-groups. Relation of EFV with CAD was assessed with two approaches: 1) presence of any CAD; 2) each individual CAD category. RESULTS: Median EFV was 90.52 ml (range 11.27-442.21 ml); median CAC was 56.5 (range 0-10,144); 848 individuals (63.1%) were categorized as CAD0, 326 (24.3%) as CAD1, 170 (12.6%) as CAD2. EFV was lower in subjects without CAC (EFVmedian = 66.5 ml), as compared to those with CAC 0.1-100 (EFVmedian = 91.47), CAC 100.1-400 (EFVmedian = 97.46) and CAC >400 (EFVmedian = 109.48) (p < 0.001). EFV was lower in CAD0 (EFVmedian = 87.21 ml), as compared to CAD1 (EFVmedian = 93.89 ml) and CAD2 (EFVmedian = 102.98 ml) individuals (p < 0.001). A logistic regression model built by including demographic and clinical variables showed inconsistent predictive value of EFV for either CAD1 or CAD2 (p > 0.05). CONCLUSIONS: In the setting of symptomatic individuals, an increased amount of epicardial fat was associated with larger amount of coronary artery calcifications and was observed in individuals with obstructive CAD, however without predictive value to confidently determine CAD presence and severity.

Quantification of epicardial fat with cardiac CT angiography and association with cardiovascular risk factors in symptomatic patients: from the ALTER-BIO (Alternative Cardiovascular Bio-Imaging markers) registry.

PURPOSE: We aimed to assess the association between features of epicardial adipose tissue and demographic, morphometric and clinical data, in a large population of symptomatic patients with clinical indication to cardiac computed tomography (CT) angiography. METHODS: Epicardial fat volume (EFV) and adipose CT density of 1379 patients undergoing cardiac CT angiography (918 men, 66.6%; age range, 18-93 years; median age, 64 years) were semi-automatically quantified. Clinical variables were compared between diabetic and nondiabetic patients to assess potential differences in EFV and adipose CT density. Multiple regression models were calculated to find the clinical variables with a significant association with EFV and adipose CT density. RESULTS: The median EFV in diabetic patients (112.87 mL) was higher compared with nondiabetic patients (82.62 mL; P < 0.001). The explanatory model of the multivariable analysis showed the strongest associations between EFV and BMI (ß=0.442) and age (ß=0.365). Significant yet minor association was found with sex (ß=0.203), arterial hypertension (ß=0.072), active smoking (ß=0.068), diabetes (ß=0.068), hypercholesterolemia (ß=0.046) and cardiac height (ß=0.118). The mean density of epicardial adipose tissue was associated with BMI (ß=0.384), age (ß=0.105), smoking (ß=0.088), and diabetes (ß=0.085). CONCLUSION: In a large population of symptomatic patients, EFV is higher in diabetic patients compared with nondiabetic patients. Clinical variables are associated with quantitative features of epicardial fat.

Chest Imaging of a rare case of cat-scratch disease in a 2-years-old baby.

Cat-scratch disease (CSD) is usually a self-limiting infection that in the majority of cases occurs as lymphadenitis in children who have been scratched or bitten by a cat. Rarely, Bartonella henselae is cause of fever of unknown origin (FUO), with dissemination to various organs, mimicking an inflammatory rather than a lymphoproliferative disease. This manuscript will present a case of thoracic manifestations of CSD in an immunocompetent 2-years baby without history of cat contact, with fever of unknown origin, investigated by chest CT and MRI.

Half-dose versus full-dose macrocyclic gadolinium at 3-T magnetic resonance imaging in paediatric bone and soft-tissue disease.

BACKGROUND: Given the recent concerns about gadolinium-based contrast agent safety, dose reduction strategies are being investigated. OBJECTIVE: To compare half-dose and standard full-dose gadoterate meglumine at 3-tesla (T) MRI in paediatric bone and soft-tissue diseases. MATERIALS AND METHODS: We prospectively enrolled 45 children (age range 2.7 months to 17.5 years, median age 8.7 years, 49 total anatomical segments) with bone and soft-tissue diseases (neoplastic, inflammatory/infectious, ischaemic and vascular) imaged at 3-T MRI. Two consecutive half-doses of gadoterate meglumine (0.05 mmol/kg body weight) were administered. Two sets of post-contrast T1-weighted images were obtained, one after the first half dose and the other after the second half dose. For qualitative analysis, three radiologists, masked to the gadolinium dose, compared the diagnostic quality of the images. For quantitative analysis, we compared signal-to-noise ratio and contrast-to-noise ratio at half and full doses. RESULTS: Signal-to-noise ratio and contrast-to-noise ratio did not vary significantly between the two groups. Qualitative analysis yielded excellent image quality in both post-contrast image datasets (Cohen κ=0.8). CONCLUSION: In paediatric bone and soft-tissue 3-T MRI, it is feasible to halve the standard dose of gadoterate meglumine without losing image quality.

[A rare case of intussusception in a seventeen year old boy: diagnosis, management and review of the literature.] / Un raro caso di invaginazione intestinale in un adolescente: diagnosi, management e revisione della letteratura.

Intussusception is the most common cause of intestinal obstruction and acute abdomen in the first year of life. Approximately in the 80% of cases intussusception occurs when the last ileal loop is pulled into the cecum, passing through the ileocecal valve, and finally resulting in the displacement of cecum in the upper abdominal. It could be related with mesenteric adenitis because enlarged lymph nodes, together with peristalsis, can serve as "lead point". Other forms of intussusception are ileo-ileal and colo-colic. The aetiology in infants and adults can be very variable and most often linked with secondary causes, such as benign or malignant lesions (polyps, tumors, lymphomas, intestinal duplication cyst, Meckel's diverticulum). In this paper we describe a rare case of idiopathic ileo-ileal intussusception in a 17 year old boy. We also discuss, with a careful analysis of the literature, the diagnostic and therapeutic protocol in case of intussusception ileo-colic approved at the Meyer Children's Hospital.

Imaging of congenital pulmonary malformations.

Congenital pulmonary malformations represent a broad spectrum of anomalies that may result in varied clinical and pathologic pictures, ranging from recurrent pulmonary infections and acute respiratory distress syndrome, which require timely drug therapy, up to large space-occupying lesions needing surgical treatment. This classification includes three distinct anatomical and pathological entities, represented by Congenital Cystic Adenomatoid Malformation, Bronchopulmonary Sequestration and Congenital Lobar Emphysema. The final result in terms of embryological and fetal development of these alterations is a Congenital Lung Hypoplasia. Since even Bronchial Atresia, Pulmonary Bronchogenic Cysts and Congenital Diaphragmatic Hernias are due to Pulmonary Hypoplasia, these diseases will be discussed in this review (1, 2).

Curved Needles in CT-Guided Fine Needle Biopsies of Abdominal and Retroperitoneal Small Lesions.

PURPOSE: To demonstrate the advantages of using curved needles in fine needle aspiration (FNA) with CT-guided, for analyzing abdominal and/or retroperitoneal small lesions which are impossible to reach with conventional non-surgical biopsy techniques, particularly in cases in which the cytology sample was not possible to obtain by means of US or CT guide with axial images. MATERIALS AND METHODS: An authorization for CT-guided FNA in patients with neoplasms is not required by the institutional review board of our Institute. From April 2012 to November 2014, the study included retrospectively 25 patients (16 M, 9 F) who underwent CT-guided FNA of abdominal and/or retroperitoneal small lesions (<30 mm), in which the ultrasound and axial CT scans were not reliable guides for the biopsy procedure because of the interposition of anatomical obstacles. Patients with suspected lymphomas or sarcomas, pediatric patients and patients with bleeding diathesis were excluded. Cytology reports were used for evaluating suitability. RESULTS: The biological material was considered to be suitable for cytological study, with a diagnostic value in all 25 cases, finding in particular: out of 23 neoplastic lesions (85%), 21 were malignant (90.2%) and 2 were benign (8%). 2 out 25 were non-neoplastic benign lesions (8%). No procedural complications arose in any of the cases (0%). CONCLUSION: Using curved needles, there is an effective improvement in CT-guided FNA of abdominal and retroperitoneal small lesions which are difficult to achieve with conventional CT or ultrasound guide.

Eosinophilic cystitis and nephrogenic adenoma of the bladder: a rare association of 2 unusual findings in childhood.

Neither eosinophilic cystitis nor nephrogenic adenoma is often diagnosed in children, with few pediatric cases being reported in the literature. Although these maladies share the same predisposing conditions, namely, chronic irritation or injury to the urothelium and lower urinary tract and symptoms such as dysuria, hematuria, and urinary frequency, their concomitance is exceptional. Herein, we report the case of an 8-year-old boy with a previous history of bladder surgery presenting histologically diagnosed eosinophilic cystitis and nephrogenic adenoma.

A case of portal vein thrombosis after laparoscopy-assisted splenectomy and cholecystectomy in a child.

We report the case of an 8-year-old boy with a red cell membrane disorder who developed, soon after undergoing laparoscopic cholecystectomy and splenectomy, complete thrombosis of the right branch and a partial occlusion of the left branch of the portal vein. The child was affected by a right hemiparesis because of a hypoxic-ischemic disorder that occurred in the first hours of life and was heterozygous for the methylenetetrahydrofolate reductase gene mutation 677C-T. Intravenous heparin and aspirin were initiated on postoperative day 7. Heparin treatment was switched to the subcutaneous route after the first 24 hours. The symptoms subsided 3 days after the beginning of treatment, whereas complete resolution of portal vein thrombosis was observed 2 months later. A review of the literature is reported, and the possible pathogenetic mechanisms underlying portal vein thrombosis are discussed.

Congenital ureteral valves: two new cases and a review of the literature.

Ureteral valves are a rare clinical finding. Frequently they are associated with other urological anomalies such as complete or incomplete renal duplication, ectopic ureter, vesicoureteral reflux and horseshoe kidney. Hydronephrosis is the most common clinical sign. We report two cases of ureteral valves recently encountered at the Surgical Department of Sant Joan de Dèu Hospital in Barcelona, and discuss the diagnosis and surgical treatment with a review of the literature from 1926.