Brain and spine malformations and neurodevelopmental disorders in a cohort of children with CAKUT.

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) represent 20-30% of all birth defects and are often associated with extra-renal malformations. We investigated the frequency of brain/spine malformations and neurological features in children with CAKUT. METHODS: We reviewed the clinico-radiological and genetic data of 199 out of 1,165 children with CAKUT evaluated from 2006 to 2023 (99 males, mean age at MRI 6.4 years) who underwent brain and/or spine MRI. Patients were grouped according to the type of CAKUT (CAKUT-K involving the kidney and CAKUT-H involving the inferior urinary tract). Group comparisons were performed using χ2 and Fisher exact tests. RESULTS: Brain/spine malformations were observed in 101/199 subjects (50.7%), 8.6% (101/1165) of our CAKUT population, including midbrain-hindbrain anomalies (40/158, 25.3%), commissural malformations (36/158, 22.7%), malformation of cortical development (23/158, 14.5%), Chiari I anomaly (12/199, 6%), cranio-cervical junction malformations (12/199, 6%), vertebral defects (46/94, 48.9%), caudal regression syndrome (29/94, 30.8%), and other spinal dysraphisms (13/94, 13.8%). Brain/spine malformations were more frequent in the CAKUT-K group (62.4%, p < 0.001). Sixty-two subjects (62/199, 31.2%) had developmental delay/intellectual disability. Neurological examination was abnormal in 40/199 (20.1%). Seizures and/or electroencephalographic anomalies were reported in 28/199 (14%) and behavior problems in 19/199 subjects (9%). Developmental delay/intellectual disability was more frequent in kidney dysplasia (65.2%) and agenesis (40.7%) (p = 0.001). CONCLUSIONS: We report a relative high frequency of brain/spine malformations and neurodevelopmental disorders in children with CAKUT who underwent MRI examinations in a tertiary referral center, widening the spectrum of anomalies associated with this condition.

Microvascular obstruction extent predicts major adverse cardiovascular events in patients with acute myocardial infarction and preserved ejection fraction.

OBJECTIVES: To investigate the prognostic role of early post-infarction cardiac magnetic resonance (CMR) on long-term risk stratification of ST segment elevation myocardial infarction (STEMI) patients with preserved left ventricular ejection fraction (LVEF). METHODS: Seventy-seven STEMI patients treated by primary percutaneous coronary intervention (PCI) and LVEF > 50% at CMR were included. The median time between STEMI and CMR was 5 days (IQR 2-8). LV volumes and function, area at risk (on T2 weighted images), infarcted myocardium (on late enhanced images), intramyocardial hemorrhage, and early and late microvascular obstruction (MVO) were detected and measured. CMR tissue determinants were correlated with the incidence of major adverse cardiovascular events (MACEs) over a 5-year follow-up. RESULTS: During median follow-up of 4 years (range 3 to 5 years), eight (10%) patients experienced MACE, yielding an annualized event rate of 2.1%. All CMR tissue markers were not significantly different between MACE and no-MACE patients, except for the presence of late MVO (50% vs. 16%, respectively; p = 0.044) and its extent (2.30 ± 1.64 g vs. 0.18 ± 0.12 g, respectively; p = 0.000). From receiver-operating characteristic (ROC) curve (area under the curve 0.89; 95% confidence interval, 0.75-1.0; p = 0.000), late MVO extent > 0.385 g was a strong independent predictor of MACE at long-term follow-up (sensitivity = 87%, specificity = 90%; hazard ratio = 2.24; 95% confidence interval, 1.51-3.33; p = 0.000). CONCLUSIONS: Late MVO extent after primary PCI on CMR seems to be a strong predictor of MACE at 5-year follow-up in patients with LVEF > 50%. Noticeably, late MVO extent > 0.385 g provided relevant prognostic insights leading to improved long-term risk stratification. KEY POINTS: • Tissue markers provided by cardiac magnetic resonance aid in prognostic stratification after myocardial infarction • The occurrence of late microvascular obstruction after acute myocardial infarction increases risk of major adverse events at 5-year follow-up. • The greater microvascular obstruction extent on late gadolinium enhanced images is related to an increased risk of adverse events in patients with myocardial infarction and preserved left ventricular function.

Assessment of interstitial lung disease in systemic sclerosis using the quantitative CT algorithm CALIPER.

Interstitial lung disease (ILD) remains a major cause of morbidity and mortality in systemic sclerosis (SSc). Study aim is to characterize and quantify SSc-ILD by using Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER). Secondly, our objective is to evaluate which radiological pattern is predictive of lung function decline at 12 months follow-up. In the prospective study (IRB 5435), 66 SSc patients underwent high-resolution computerized tomography (HRCT) at baseline. HRCT was performed according to standard protocol using a CT 64GE light speed VCT power scanner. CALIPER classified lung parenchyma on volume units. Every volume unit was classified into radiological parenchymal patterns (honeycombing, reticular and ground glass). Pulmonary function tests (PFTs) were performed at baseline and after 12 months of follow-up. Cigarette smoking and other lung diseases unrelated to SSc are exclusion criteria. CALIPER analysis showed normal lung parenchyma 87.4 ± 9.8%, ground glass 2.8 ± 5.3%, reticular 4 ± 5.7%, and honeycombing 1 ± 1%. In multiple regression analysis, FEV1 (p < 0.0001), FVC (p = 0.001), and DLCO (p < 0.0001) measurements at baseline showed a negative correlation with the reticular pattern percentage. At follow-up, DLCO reduction showed a positive correlation (p < 0.001) with the percentage of ground glass pattern (r = 0.33, beta coefficient = 0.51). In the ROC curve analysis, ground glass score is a good predictor (0.75, p = 0.009; 95% CI 0.59-0.91) of DLCO worsening, defined as a decrease of more than 10% of DLCO. Using a cutoff ≥ 4.5 for ground glass score, the RR for DLCO worsening is 6.8 (p < 0.01; 95% CI 1.6-29.2). The results of this study show that CALIPER is useful not only for quantifying lung damage but also for assessing worsening PFTs, but larger studies are needed to confirm these preliminary data.Key Points• At baseline reticular pattern showed negative correlation with PFTs• At follow-up ground glass pattern predicts worsening of DLCO• CALIPER is a useful to quantify lung damage.

Tracheal Bronchus: High Resolution Computed Tomography diagnosis in a symptomatic patient.

A 75-year-old female underwent a High Resolution Computed Tomography (HRCT) scan for recurrent bronchitis and cough. HRCT images showed an anomalous supernumerary bronchus to the right upper lobe directly arising from the right side of distal trachea, corresponding to Tracheal Bronchus (TrB). TrB can cause recurrent right upper lobe pneumonia and special care is requiring during endotracheal intubation.