Chimeric antigen receptor T-cell therapy for marrow and extramedullary relapse of infant acute lymphoblastic leukemia.

Chimeric antigen receptor (CAR) T-cells, engineered autologous T-cells that target antigens found in leukemia, have shown durable remissions in relapsed acute lymphoblastic leukemia (ALL). Infant ALL with KMT2A rearrangements (KMT2Ar) is a rare, aggressive form of leukemia associated with extramedullary disease both at diagnosis and at relapse, and overall outcomes for these patients are dismal. Here we report the successful use of tisagenlecleucel, a CAR T-cell product approved for relapsed/refractory ALL, in a patient with KMT2Ar infant ALL who was treated for combined marrow and extramedullary (renal) relapse.

Clinical utilization of whole-body PET/MRI in childhood sarcoma.

Hybrid positron emission tomography (PET)/magnetic resonance imaging (MRI) has emerged as a useful tool that combines the superior tissue contrast of MRI with the targeted functional imaging of PET. In the assessment of sarcomas in children, PET/MRI has the potential to serve as a single point of service, allowing superior anatomical imaging and evaluation of metabolic uptake during one imaging session. In this pictorial essay, we review our preliminary experience with PET/MRI in the evaluation of pediatric sarcoma. The limitations and contraindications of PET/MRI are also discussed.

Liver failure and x-linked immunodeficiency type 47.

Patients with defects in the ATP6AP1 gene have rarely been described. ATP6AP1-related disorders are a subtype of CDG, which result in enzyme deficiencies affecting multiple organ systems ranging from mild to life-threatening. Of the 13 patients described, all had hepatopathy, but this is the first case to be successfully transplanted. We describe two brothers who developed hyperbilirubinemia shortly after birth and progressed to liver failure, case 1 by 12 months of age, with successful transplant 2 years later, and case 2 by 4 months of age, who passed away while awaiting liver transplant. Both boys were found to have a new variant in the ATP6AP1 gene: c.932/p.Leu311Gln. Although the identified ATP6AP1 gene variant was classified as unknown significance at the time, both children's phenotypes fit with what has been described for ATP6AP1-related disorders. Therefore, this result appears to have been diagnostic for both boys. This rare type of CDG, X-linked immunodeficiency type 47 (OMIM #300972), particularly in patients who progress to liver failure requiring transplant, should be included on the differential of liver failure in infants and toddlers, and its gene should be added to the diagnostic workup for such cases.

E-cigarette, or vaping, product use-associated lung injury in adolescents: a review of imaging features.

BACKGROUND: There has been a recent increase in recognition of lung disease related to the use of electronic cigarettes (called "vaping"). These patients present with acute respiratory illness following exposure to vaporized cannabis or nicotine products and sometimes require hospitalization and intensive care. We describe the imaging findings of this disease entity in the pediatric population. OBJECTIVE: To describe the radiologic findings of lung injury associated with electronic cigarette use (vaping) in the adolescent pediatric population. MATERIALS AND METHODS: We identified all adolescents with acute respiratory illness and a history of electronic cigarette use who presented at our institution within a 3-month period (June 2019 through August 2019). We excluded adolescents with potential intercurrent pulmonary disease. We reviewed the charts for symptomatology and laboratory and pathology data. In addition, we reviewed the chest radiographs and chest CTs of these adolescents. RESULTS: The review group consisted of 12 teenage pediatric patients (10 boys and 2 girls; mean age 16.9 years, range 16.0-17.7 years) with acute respiratory illness found to have a temporal association with electronic cigarette use for cannabis products, nicotine, or both. Other etiologies for illness in these adolescents had been excluded by clinical and laboratory evaluation. All of the adolescents were admitted to the hospital for treatment. The clinical presentations included dyspnea, abdominal pain and constitutional symptoms. Pulmonary function testing that was performed in all patients during admission or follow-up demonstrated reduced diffusion capacity in 4/12 (33%), an obstructive ventilatory pattern in 4/12 (33%), a restrictive pattern in 1/12 (8%), and a mixed obstructive and restrictive pattern in 2/12 (17%) adolescents. Bronchoalveolar lavage studies, performed in 9 of the 12 adolescents, revealed inflammatory cells and lipid-laden macrophages. All of the patients underwent CT of the chest; the findings were notable for centrilobular ground-glass nodules (11/12; 92%) and confluent ground-glass opacities (12/12; 100%), with frequent subpleural sparing (9/12; 75%). Additionally, 6/12 (50%) adolescents demonstrated small pleural effusions; 6/12 (50%) had mild bronchial wall thickening; 9/12 (75%) had enlarged hilar or mediastinal lymph nodes; and 2/12 (17%) had a small pericardial effusion. CONCLUSION: As seen in our teenage population, e-cigarette, or vaping, product use-associated lung injury (EVALI) is characterized by centrilobular ground-glass nodules and ground-glass opacities with subpleural sparing. The imaging findings are most consistent with acute lung injury resulting from toxic inhalation. Because adolescent pediatric patients might not be forthcoming with their history of electronic cigarette use, it is important for the pediatric radiologist to be aware of the imaging patterns of this disease.

Complications of percutaneous gastrostomy and gastrojejunostomy tubes in children.

Percutaneous feeding tubes are generally considered a safe option for enteral feeding and are widely used in children who require long-term nutritional support. However, complications are not infrequent and can range from bothersome to life-threatening. Radiologists should be familiar with the imaging appearances of potential complications for optimal patient care. In this review, we discuss radiologic appearances of common complications and less frequent but serious complications related to percutaneous feeding tubes. Additionally, as fluoroscopic feeding tube evaluation is often requested as the initial imaging study, we also discuss the fluoroscopic appearances of some uncommon complications.

Use of Low-Dose Computed Tomography in Children With Nephrolithiasis: An Analysis From 2 Tertiary Pediatric Centers.

INTRODUCTION: Although ultrasound (US) is the preferred first-line imaging for pediatric nephrolithiasis, CT may be necessary in cases of a nondiagnostic US or when US in not available. Utilization of dose reduction strategies in children undergoing CT for nephrolithiasis is not well described. We compared use of low-dose CT (LDCT) in children presenting to 2 pediatric centers. METHODS: We performed a retrospective chart review of children ≤ 17 years of age presenting with suspected nephrolithiasis to 2 tertiary children's hospitals, inclusive of those referred to these centers from nonpediatric facilities between 2013 and 2019. Children were included with an index CT scan from either the pediatric or referring center while those who had prior documented CT for nephrolithiasis within the study period or missing radiation dose assessment were excluded. The primary outcome was LDCT as defined as radiation dose < 3 mGy. The primary comparator was pediatric vs outside referral center. Exploratory analysis evaluated other factors associated with LDCT, including radiation dosage as a continuous variable. RESULTS: A total of 155 individuals met inclusion criteria, with 126 (81.3%) receiving standard dose and 29 (18.7%) receiving LDCT. Pediatric facilities were more likely to utilize LDCT as compared to referral centers (P < .05). Older age and higher BMI were also found to be associated with increased radiation dose exposure. CONCLUSIONS: Pediatric facilities utilized LDCT more frequently, although age and BMI may also influence imaging choices. An understanding of the factors associated with dose reduction in CT will impact future efforts to explore optimum imaging stewardship in pediatric nephrolithiasis.

Trichobezoar: A Rare Cause of Protein-losing Enteropathy.

Protein-losing enteropathy (PLE) is caused by protein loss through the gastrointestinal tract which results in hypoalbuminemia. The most common causes of PLE in children include cow milk protein allergy, celiac disease, inflammatory bowel disease, hypertrophic gastritis, intestinal lymphangiectasia, and right-sided heart dysfunction. We present a case of a 12-year-old male with bilateral lower extremity edema, hypoalbuminemia, elevated stool alpha-1-antitrypsin, and microcytic anemia. He was found to have a trichobezoar in the stomach extending to the jejunum, an unusual cause of PLE. The patient underwent an open laparotomy and gastrostomy to remove the bezoar. Follow-up confirmed resolution of hypoalbuminemia.

Imaging Practices and Implications in Young Infants With Urinary Tract Infection.

OBJECTIVES: Management of infants aged ≤60 days with urinary tract infections (UTI) is challenging. We examined renal imaging in infants aged ≤60 days with UTI at a tertiary care children's hospital to identify the impact of standardizing renal ultrasound (RUS) interpretation. METHODS: We retrospectively studied infants aged ≤60 days hospitalized for UTI or fever with urine culture and renal imaging obtained and final diagnosis of UTI. RUS initially had noncriterion-based (NCB) interpretation by experienced pediatric radiologists. For this study, a single pediatric radiologist used a criterion-based (CB) hydronephrosis grading system to reinterpret films initially classified as "abnormal" on the NCB reading. We compared final renal imaging results between NCB and CB groups. RESULTS: Of 193 infants, 180 (93%) had inpatient RUS with 114 (63%) abnormal NCB interpretation. Of those with initially abnormal NCB interpretation, 85 OF 114 (75%) had minor and 29 OF 114 (25%) had significant abnormality by CB reinterpretation. In follow-up, the CB "minor abnormality" group showed 25% abnormal renal imaging, whereas the "significant abnormality" group showed 77% abnormal renal imaging with 54% having high-grade reflux on a voiding cystourethrogram (VCUG). Patients with CB inpatient RUS minor abnormality showed 3% abnormal RUS at follow-up, but 13% showed high-grade reflux on VCUG. CONCLUSIONS: Standardized RUS interpretation in young infants with UTI improved the accuracy of identification of abnormalities on follow-up renal imaging. In patients with CB minor abnormality on inpatient RUS, our results suggest limited utility of follow-up RUS; however, follow-up VCUG remained useful to identify high-grade reflux.

E-cigarette, or Vaping, Product Use-Associated Lung Injury-Lessons Learned: A Case Series.

E-cigarette, or vaping, product use has been declared an epidemic, and a new disease has emerged from their use. We describe 4 patients with significant acute lung injury related to e-cigarette use who underwent bronchoscopy and bronchoalveolar lavage. All cases posed anesthetic challenges, including increased airway reactivity, hypoxia, increasing oxygen requirements, and, in 2 severe cases, the need for continued postprocedural mechanical ventilatory support. It is imperative that all members of the treating team are aware of the disproportionate risk of respiratory complications to anticipate the possible need for increased postprocedural respiratory support.

Impaired lung function following e-cigarette or vaping product use associated lung injury in the first cohort of hospitalized adolescents.

BACKGROUND: Beginning June 2019, Children's Wisconsin was the first hospital to identify a cohort of adolescent patients hospitalized with symptoms likely associated with e-cigarette use. Our report adds to the growing literature describing the radiographic, gross and cytopathologic bronchoscopic findings, and short-term lung function outcomes in this cohort of adolescents with e-cigarette or vaping product use associated lung injury (EVALI). METHODS: We present 15 adolescents hospitalized from June to September, 2019 with confirmed EVALI. We abstracted data from inpatient hospitalization and first outpatient pulmonary clinic visit. RESULTS: There were 15 patients (11 male, 12 White) with a mean age of 17.1 years. All patients presented with subacute pulmonary, gastrointestinal and constitutional complaints. Diagnostic workup was guided by the Centers for Disease Control criteria for confirmed EVALI case surveillance. Flexible bronchoscopy was performed in 13/15 patients with 10/13 demonstrating gross pathologic abnormalities. Seven of 15 patients required intensive care and 2 met criteria for pediatric Acute Respiratory Distress Syndrome. Patients had dramatic improvement with systemic glucocorticoid therapy and 14/15 were discharged on room air. Eleven patients were seen as outpatients. Despite 11/11 patients reporting resolved or improved symptoms, 7/11 had abnormalities on pulmonary function testing. We initiated inhaled corticosteroids for 5/11 patients and 4/11 patients remained on their corticosteroid wean. CONCLUSIONS AND RELEVANCE: We report short-term outcomes of the first cohort of adolescent patients hospitalized with EVALI. An association is observed between clinical improvement and treatment with systemic corticosteroids. However, residual airway reactivity or diffusion abnormalities persisted when patients were re-evaluated in the short-term period (mean 4.5 weeks).

Avoiding misdiagnosis in postnatal presentation of congenital diaphragmatic hernia: A report of two cases and review of radiologic features.

Diagnosis of a congenital diaphragmatic hernia after the first day of life can be challenging. The clinical symptoms are often nonspecific, and initial radiographic findings in these patients may mimic many other acute chest conditions, including pneumonia, pleural effusion, and pneumothorax. In turn, diagnostic uncertainty may expose the patient to unnecessary and even potentially harmful interventions such as thoracostomy tube placement. As such, it is imperative that radiologists remain aware of this uncommon entity and recognize imaging findings which may provide clues to its diagnosis.

Intravascular ultrasound for transvenous interventions.

Since the development of intravascular ultrasound in the late 1980s, the modality has been used both to image the vascular system and to direct interventions in target vessels. Intravascular ultrasound (IVUS) was initially used to image atherosclerosis and aid in its treatment, but it has more recently been employed within the venous system, allowing for both intravenous and transvenous image-guided interventions. IVUS is now used for both direct and transjugular intrahepatic portosystemic shunt placement, for transcaval liver biopsy and transcaval puncture of type II endoleaks, and for cardiac mass biopsy, among other interventions. The use of IVUS not only yields potential for reduced fluoroscopy dose in and increased safety of established procedures, but it also allows for the development of altogether new procedures.