Respiratory Failure Due to Idiopathic Pneumonia Syndrome in a Pediatric Patient After Recipient-derived Allogeneic Chimeric Antigen Receptor T-Cell Therapy.

Idiopathic pneumonia syndrome (IPS) is a life-threatening complication of hematopoietic cell transplantation, but it is not clearly described following chimeric antigen receptor (CAR) T-cell therapy. We describe a child who developed IPS after receiving tisagenlecleucel for post-hematopoietic cell transplantation relapsed acute lymphoblastic leukemia and had a remarkable improvement after treatment with corticosteroids and etanercept. We discuss the implications of cytokine signaling in IPS and immunologic considerations of allogeneic CAR T cells. We anticipate that the incidence of IPS and other allogeneic phenomena will be observed more often as allogeneic CAR T cells are employed in more varied settings with more mismatched donors.

Imaging evaluation of the pediatric mediastinum: new International Thymic Malignancy Interest Group classification system for children.

Mediastinal masses are commonly identified in the pediatric population with cross-sectional imaging central to the diagnosis and management of these lesions. With greater anatomical definition afforded by cross-sectional imaging, classification of mediastinal masses into the traditional anterior, middle and posterior mediastinal compartments - as based on the lateral chest radiograph - has diminishing application. In recent years, the International Thymic Malignancy Interest Group (ITMIG) classification system of mediastinal masses, which is cross-sectionally based, has garnered acceptance by multiple thoracic societies and been applied in adults. Therefore, there is a need for pediatric radiologists to clearly understand the ITMIG classification system and how it applies to the pediatric population. The main purpose of this article is to provide an updated review of common pediatric mediastinal masses and mediastinal manifestations of systemic disease processes in the pediatric population based on the new ITMIG classification system.

Relationship of Vitamin D Deficiency and Fatty Liver in Children as Defined by Multiple Imaging and Histologic Endpoints.

OBJECTIVES: The relationship between vitamin D deficiency (VDD) and pediatric nonalcoholic fatty liver disease (NAFLD) remains uncertain due to conflicting results and few studies with histologic endpoints. We therefore used multiple imaging and histologic NAFLD endpoints to more comprehensively assess the association between VDD and NAFLD in a large pediatric population. METHODS: Data were obtained from an ongoing pediatric NAFLD study in Bronx, NY. Briefly, overweight and obese children aged 2-18 years with alanine aminotransferase (ALT) levels ≥ 35 U/L were serially enrolled. Liver biopsy was obtained in accordance with clinical guidelines. All participants had liver imaging, namely, controlled attenuation parameter (CAP; Echosens, France) to assess steatosis and, to assess fibrosis, vibration controlled transient elastography (VCTE; FibroScan™, Echosens, France) and acoustic radiation force impulse (ARFI; Philips, Netherlands) imaging. Levels of 25-hydroxyvitamin D were measured serologically. RESULTS: N=276 (88%) of 315 participants had 25-OH vitamin D results, of whom 241 (87%) were Hispanic, 199 (72%) were male, and 92 (33%) underwent liver biopsy. VDD was univariately associated with high waist circumference (p=0.004), high-density lipoprotein level (p=0.01), season (p=0.009), and CAP score (p=0.01). In multivariate analysis, only waist circumference (p=0.0002) and biopsy inflammation grade (p=0.03) were associated with VDD, though the latter had not approximated statistical significance in univariate analysis (p=0.56). There was no association between VDD and hepatic steatosis, ballooning, NAFLD Activity Score, ARFI or VCTE elasticity scores. CONCLUSIONS: VDD was not associated with NAFLD defined by imaging and histologic endpoints, except for a possible relation with histologic inflammation grade.

Bladder prolapse through a patent urachus presenting as an umbilical mass in the newborn: characteristic prenatal sonographic findings and the diagnostic benefit of postnatal cystography.

Bladder prolapse through a patent urachus is rare. We present a newborn with an unusual exophytic, erythematous umbilical mass. Voiding cystography readily demonstrated continuity of the bladder dome with the umbilical mass, confirming bladder prolapse through a patent urachus. The diagnosis of bladder prolapse was rapidly made in a second newborn with similar imaging and clinical findings and confirmed by cystography. We discuss the embryology of this condition including the association with a vesico-allantoic cyst in utero. Pre- and postnatal images are presented. The use of cystography in diagnosis is emphasized.

Feasibility study of a novel portable digital radiography system modified for fluoroscopy in the neonatal intensive care unit.

BACKGROUND: A portable radiographic system capable of fluoroscopic imaging in the neonatal intensive care unit (NICU) potentially benefits critically ill neonates by eliminating the need to transport them to a fluoroscopy suite. OBJECTIVE: To evaluate whether a portable bedside fluoroscopy system in the NICU can deliver comparable image quality at a similar dose rate to a standard system in a fluoroscopy suite. MATERIALS AND METHODS: In phase A, 20 patients <3 years of age and scheduled to undergo upper gastrointestinal series (upper GI) or voiding cystourethrograms (VCUG) in the radiology fluoroscopy suite were recruited to evaluate a portable fluoroscopic unit. A modified portable radiographic system with a cassette-sized detector and an in-room fluoroscopy system were sequentially used in the same examination. Four radiologists compared the image quality of 20 images from each system using the Radlex score (1-4) for five image quality attributes. The radiation dose rates for the portable and in-suite systems were collected. In phase B, fluoroscopy studies were performed in 5 neonates in the NICU and compared to the 20 previous neonatal studies performed in the department. Clinical workflow, examination time, fluoroscopy time, scattered radiation dose and patient radiation dose were evaluated. RESULTS: In phase A, average dose rates for in-room and portable systems were equivalent, (0.322 mGy/min and 0.320 mGy/min, respectively). Reader-averaged Radlex scores for in-room and portable systems were statistically significantly greater (P<0.05) for all attributes on the portable system except for image contrast. In phase B, scattered radiation from the average fluoroscopy time (26 s) was equivalent to the scattered radiation of 2.6 portable neonatal chest radiographs. Procedure time and diagnostic quality were deemed equivalent. The average dose rate in the NICU with the portable system was 0.21 mGy/min compared to 0.29 mGy/min for the in-room system. CONCLUSION: The portable fluoroscopy unit is capable of providing comparable image quality at equivalent dose levels to an in-room system for neonates with minimal risks to the staff and other patients in the NICU.

Acute chest syndrome in the setting of SARS-COV-2 infections-A case series at an urban medical center in the Bronx.

New York City has emerged as one of the epicenters of the SARS-COV-2 pandemic, with the Bronx being disproportionately affected. This novel coronavirus has caused significant respiratory manifestations raising the concern for development of acute chest syndrome (ACS) in patients with sickle cell disease (SCD). We report a series of pediatric SCD SARS-COV-2-positive patients admitted with ACS. SARS-COV-2-positive SCD patients, who did not develop ACS, were the comparison group. Hydroxyurea use (P-value = .02) and lower absolute monocyte counts (P-value = .04) were noted in patients who did not develop ACS. These preliminary findings need to be further evaluated in larger cohorts.

Methods to improve the noninvasive diagnosis and assessment of disease severity in children with suspected nonalcoholic fatty liver disease (NAFLD): Study design.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is strongly associated with obesity and is the most common liver disease in the developed world. In children with suspected NAFLD, present guidelines suggest consideration of alternative diagnoses via extensive blood testing, though the yield of this work up is unknown. Furthermore, the gold standard diagnostic test for NAFLD remains liver biopsy, making the development of non-invasive tests critically important. OBJECTIVES: Our objectives are: 1) to determine the accuracy of elastography and multiple serum biomarkers - each assessed individually and as algorithms (including those previously tested in adults) - for the diagnosis of nonalcoholic steatohepatitis (NASH) and early fibrosis in children and (2) to examine the utility of extensive testing for rare alternative diagnoses in overweight or obese children with elevated alanine aminotransferase (ALT) suspected to have NAFLD. DESIGN: This is an ongoing, cross-sectional study in children 2-18 years of age with up to 2 years of prospective follow up. Eligible patients are asymptomatic, overweight or obese, and have an ALT ≥35 U/L upon enrollment. Two forms of elastography are obtained serially along with anthropometric data and routine laboratory tests. Elastography and serum biomarkers are also performed immediately prior to any clinically-indicated biopsy. METHODS: Between April 2015 and April 2018, 193 children have been enrolled in this ongoing study and 71 have undergone liver biopsy. Here we carefully report the rationale, methodology, and preliminary data for this study.

From the bottom of the heart: Measuring liver iron concentration on cardiac MRI.

Patients with hemochromatosis require regular surveillance of liver and cardiac iron concentration with liver and cardiac MRI. However, cardiac MRI includes a part of the liver in the field of view. The purpose of this retrospective and prospective study is to determine if liver T2* measured on cardiac MRI may be used as a surrogate for T2* obtained on standard liver MRI. Liver iron concentrations were measured on cardiac and liver MRI in 21 patients. Although statistically significant, the difference may be clinically insignificant as the same patients merited chelation therapy when relying on either the cardiac or liver MRI.

Vascular anomalies: A pictorial review of nomenclature, diagnosis and treatment.

Vascular anomalies, including vascular malformations and tumors, are frequently straightforward to detect; however, accurate diagnosis and appropriate treatment are often challenging. Misdiagnosis of these lesions can lead clinicians in the wrong direction when treating these patients, which can have unfavorable results. This review presents an overview of the classification systems that have been developed for the diagnosis of vascular lesions with a focus on the imaging characteristics. Pictorial examples of each lesion on physical examination, as well as non-invasive and minimally invasive imaging are presented. An overview of the endovascular treatment of these lesions is also given. In some cases, vascular anomalies may be associated with an underlying syndrome and several of the most commonly encountered syndromes are discussed. Understanding of the classification systems, familiarity with the treatment options and knowledge of the associated syndromes are essential for all physicians working with this patient population. The approach to the described entities necessitates an organized multi-disciplinary team effort, with diagnostic imaging playing an increasingly important role in the proper diagnosis and a combined interventional radiologic and surgical treatment method showing promising results.

Multidetector-row computed tomography enterographic assessment of the ileal-anal pouch: descriptive radiologic analysis with endoscopic and pathologic correlation.

OBJECTIVE: To describe the multidetector-row computed tomography enterographic (MD-CTE) features of the ileal-anal pouch after ileal pouch anal anastomosis (IPAA) surgery and correlate them with pouch endoscopy and histopathologic findings. METHODS: All MD-CTE examinations performed on patients who underwent IPAA from July 1, 2005 to December 1, 2010 (n = 35; 16 [45.7%] men; mean age, 37.7 years; age range, 22-72 years) were retrospectively evaluated in consensus by 2 radiologists. All studies were evaluated for the presence of multiple imaging features. Two radiographic scores were then calculated: a total radiographic score and a radiographic active inflammation score. In patients who underwent MD-CTE, pouch endoscopy, and biopsy within 30 days (n = 13), both scores were correlated with findings on pouch endoscopy and histopathology. RESULTS: Of the 35 patients, 33 (94%) had at least one MD-CTE finding of active or chronic pouch inflammation and 27 patients (77%) had at least one MD-CTE finding of active pouch inflammation. Of the 13 patients who underwent endoscopy and biopsy, the total radiographic score demonstrated a strong positive correlation with endoscopic score (r = 0.81; P = 0.001) and a moderate positive correlation with histopathologic score (r = 0.56; P = 0.047). The radiographic active inflammation score demonstrated a strong positive correlation with endoscopic score (r = 0.83; P = 0.0004), but only a weak nonsignificant positive correlation with histopathologic score (r = 0.492, P = 0.087). CONCLUSIONS: In patients who had IPAA surgery, findings on MD-CTE correlate positively with findings on pouch endoscopy and histopathology and are sensitive measures for pouch inflammation with high positive predictive value. Thus, MD-CTE can be a useful noninvasive test in the early evaluation of symptomatic patients.