Neurological involvement in children with hemolytic uremic syndrome.

Our objective was to establish the rate of neurological involvement in Shiga toxin-producing Escherichia coli-hemolytic uremic syndrome (STEC-HUS) and describe the clinical presentation, management and outcome. A retrospective chart review of children aged ≤ 16 years with STEC-HUS in Children's Health Ireland from 2005 to 2018 was conducted. Laboratory confirmation of STEC infection was required for inclusion. Neurological involvement was defined as encephalopathy, focal neurological deficit, and/or seizure activity. Data on clinical presentation, management, and outcome were collected. We identified 240 children with HUS; 202 had confirmed STEC infection. Neurological involvement occurred in 22 (11%). The most common presentation was seizures (73%). In the neurological group, 19 (86%) were treated with plasma exchange and/or eculizumab. Of the 21 surviving children with neurological involvement, 19 (91%) achieved a complete neurological recovery. A higher proportion of children in the neurological group had renal sequelae (27% vs. 12%, P = .031). One patient died from multi-organ failure.Conclusion: We have identified the rate of neurological involvement in a large cohort of children with STEC-HUS as 11%. Neurological involvement in STEC-HUS is associated with good long-term outcome (complete neurological recovery in 91%) and a low case-fatality rate (4.5%) in our cohort. What is Known: • HUS is associated with neurological involvement in up to 30% of cases. • Neurological involvement has been reported as predictor of poor outcome, with associated increased morbidity and mortality. What is New: • The incidence of neurological involvement in STEC-HUS is 11%. • Neurological involvement is associated with predominantly good long-term outcome (90%) and a reduced case-fatality rate (4.5%) compared to older reports.

Pneumomediastinum in a child with severe COVID-19.

The current global pandemic of the novel coronavirus SARS-CoV2 is a threat to the health and lives of millions of people worldwide. The latest statistics from the World Health Organisation show that there have been 6,515,796 confirmed cases worldwide with 387,298 confirmed deaths (last update 5 June 2020, 10:41 CEST). The majority of critically unwell patients with SARS-CoV2 are adults and the radiological findings associated with them are consistent throughout the literature. However, the reported paediatric cases are few, and as such, there is a limited body of evidence available. More international data is needed, not only on the clinical presentation, but also the radiological findings, so that health-care providers are better able to understand and diagnose this pandemic disease. We describe a case of a previously healthy 9-year-old female who presented to the Emergency Department with symptoms suggestive of raised intracranial pressure. Her CT revealed a medulloblastoma and post-operatively she tested positive for SARS-CoV2. She had a rapid deterioration in her clinical condition and required admission to the intensive care unit (ICU). We provide the supporting radiology along her clinical course in order to demonstrate important insights into this disease in children, including the unusual pnemomediastinum complications which occurred as part of her clinical course. This case is the first reported of its kind.

Rapid diffusion-weighted MRI for the investigation of recurrent temporal bone cholesteatoma.

BACKGROUND: Suspected cholesteatoma recurrence is commonly investigated with magnetic resonance imaging (MRI) of the temporal bone. Non-echo planar diffusion-weighted imaging (non-EP DWI) has become the sequence of choice. PURPOSE: To assess the agreement between an MRI protocol incorporating both non-EP DWI and contrast-enhanced sequences, and a shortened protocol without contrast-enhanced sequences in the assessment of suspected cholesteatoma recurrence. MATERIALS AND METHODS: One hundred consecutive MRIs, consisting of T2-weighted, non-EP DWI and pre- and post-contrast T1-weighted sequences, were reviewed by two radiologists at a tertiary referral centre. Agreement between the two protocols was assessment by means of a weighted Cohen kappa coefficient. RESULTS: We found a near perfect agreement between the two protocols (kappa coefficient with linear weighting 0.98; 95% confidence interval 0.95-1.00). There were two cases in which the two protocols were discordant. In both cases, the lesion measured <3 mm and images were degraded by artefact at the bone-air interface. The shortened protocol without post-contrast sequences yielded a 32% reduction in acquisition time. CONCLUSION: When non-EP DWI is available, contrast-enhanced sequences can be omitted in the vast majority of cases without compromising diagnostic accuracy. Contrast-enhanced sequences may provide additional value in equivocal cases with small (<3 mm) lesions or in cases where images are degraded by artefact.

Comparative Effectiveness of Imaging Modalities for the Diagnosis and Treatment of Intussusception: A Critically Appraised Topic.

RATIONALE AND OBJECTIVES: The purpose of this study was to critically appraise and compare the diagnostic performance of imaging modalities that are used for the diagnosis of intussusception and methods used in the treatment of ileocolic intussusception. METHODS: A focused clinical question was constructed and the literature was searched using the patient, intervention, comparison, outcome (PICO) method comparing radiography, ultrasound, and computed tomography in the detection of intussusception. The same methods were used to compare pneumatic (gas) reduction and hydrostatic (liquid) reduction using saline, water-soluble contrast, and barium. Retrieved articles were appraised and assigned a level of evidence based on the Oxford University Centre for Evidence-Based Medicine hierarchy of validity for diagnostic studies. RESULTS: The retrieved sensitivity for the diagnosis of intussusception using plain radiography is 48% (95% confidence interval [CI], 44%-52%), with a specificity of 21% (95% CI, 18%-24%). The retrieved sensitivity for the diagnosis of intussusception using ultrasound is 97.9% (95% CI, 95%-100%), with a specificity of 97.8% (95% CI, 97%-99%). Based on a good quality meta-analysis, the combined success rate of gas enema reduction was shown to be 82.7% (95% CI, 79.9%-85.6%) compared to a combined success rate of 69.6% (95% CI, 65.0%-74.1%) for liquid enema reduction. CONCLUSIONS: The best available evidence recommends ultrasound as the diagnostic modality of choice for the diagnosis of ileocolic intussusception in children. In stable children without signs of peritonism, nonoperative reduction is the treatment of choice. Pneumatic (gas) reduction enema has been shown to be superior to hydrostatic (liquid) enema reduction.

Evidence-based Practice of Radiology.

Current health care reform in the United States is producing a shift in radiology practice from the traditional volume-based role of performing and interpreting a large number of examinations to providing a more affordable and higher-quality service centered on patient outcomes, which is described as a value-based approach to the provision of health care services. In the 1990 s, evidence-based medicine was defined as the integration of current best evidence with clinical expertise and patient values. When these methods are applied outside internal medicine, the process is called evidence-based practice (EBP). EBP facilitates understanding, interpretation, and application of the best current evidence into radiology practice, which optimizes patient care. It has been incorporated into "Practice-based Learning and Improvement" and "Systems-based Practice," which are two of the six core resident competencies of the Accreditation Council for Graduate Medical Education and two of the 12 American Board of Radiology milestones for diagnostic radiology. Noninterpretive skills, such as systems-based practice, are also formally assessed in the "Quality and Safety" section of the American Board of Radiology Core and Certifying examinations. This article describes (a) the EBP framework, with particular focus on its relevance to the American Board of Radiology certification and maintenance of certification curricula; (b) how EBP can be integrated into a residency program; and (c) the current value and likely place of EBP in the radiology information technology infrastructure. Online supplemental material is available for this article.

CD141⁺ myeloid dendritic cells are enriched in healthy human liver.

BACKGROUND & AIMS: Extensive populations of liver immune cells detect and respond to homeostatic perturbation caused by damage, infection or malignancy. Dendritic cells (DCs) are central to these activities, governing the balance between tolerance and immunity. Most of our knowledge about human liver DCs is derived from studies on peritumoral tissue. Little is known about the phenotype and function of DCs, in particular the recently described CD141(+) subset, in healthy human liver and how this profile is altered in liver disease. METHODS: During liver transplantation, healthy donor and diseased explant livers were perfused and hepatic mononuclear cells isolated. Dendritic cell subset frequency and phenotype were characterised in liver perfusates by flow cytometry and the function of CD141(+) DCs was evaluated by mixed lymphocyte reactions (MLRs) and measuring cytokine secretion. RESULTS: Almost one third of liver CD11c(+) myeloid DCs (mDCs) expressed CD141 compared to <5% of circulating mDCs. Hepatic CD141(+) DCs demonstrated pro-inflammatory function in allogeneic MLRs, inducing T cell production of interferon gamma (IFN-γ) and interleukin (IL)-17. While CD123(+) plasmacytoid DCs (pDCs) and CD1c(+) mDCs were expanded in diseased liver perfusates, CD141(+) DCs were significantly depleted. Despite their depletion, CD141(+) DCs from explant livers produced markedly increased poly(I:C)-induced IFN lambda (IFN-λ) compared with donor DCs. CONCLUSIONS: Accumulation of CD141(+) DCs in healthy liver, which are significantly depleted in liver disease, suggests differential involvement of mDC subsets in liver immunity.