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OBJECTIVES: To assess the role of CT venography (CTV) in the diagnosis of venous thromboembolism (VTE) during the postpartum period. MATERIALS AND METHODS: This multicenter prospective cohort study was conducted between April 2016 and April 2020 in 14 university hospitals. All women referred for CT pulmonary angiography (CTPA) for suspected pulmonary embolism (PE) within the first 6 weeks postpartum were eligible. All CTPAs were performed on multidetector CT machines with the usual parameters and followed by CTV of the abdomen, pelvis, and proximal lower limbs. On-site reports were compared to expert consensus reading, and the added value of CTV was assessed for both. RESULTS: The final study population consisted of 123 women. On-site CTPA reports mentioned PE in seven women (7/123, 5.7%), all confirmed following expert consensus reading, three involving proximal pulmonary arteries and four limited to distal arteries. Positive CTV was reported on-site in nine women, five of whom had negative and two indeterminate CTPAs, bringing the VTE detection rate to 11.4% (14/123) (95%CI: 6.4-18.4, p = 0.03). Expert consensus reading confirmed all positive on-site CTV results, but detected a periuterine vein thrombosis in an additional woman who had a negative CTPA, increasing the VTE detection rate to 12.2% (15/123) (95%CI: 7.0-19.3, p = 0.008). Follow-up at 3 months revealed no adverse events in this woman, who was left untreated. Median Dose-Length-Product was 117 mGy.cm for CTPA and 675 mGy.cm for CTPA + CTV. CONCLUSION: Performing CTV in women suspected of postpartum PE doubles the detection of venous thromboembolism, at the cost of increased radiation exposure. CLINICAL RELEVANCE STATEMENT: CTV can help in the decision-making process concerning curative anticoagulation in women with suspected postpartum PE, particularly those whose CTPA results are indeterminate or whose PE is limited to the subsegmental level. KEY POINTS: Postpartum women are at risk of pulmonary embolism, and CT pulmonary angiography can give equivocal results. CT venography (CTV) positivity increased the venous thromboembolism detection rate from 5.7 to 11.4%. CTV may help clinical decision-making, especially in women with indeterminate CTPA results or subsegmental emboli.
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BACKGROUND: Juvenile Xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis, occurring mainly in infancy. With an extracutaneous lesion, its diagnosis is difficult, because of a wide clinical spectrum. Here we demonstrate and characterize imaging features of 11 patients with JXG of the head and neck in various locations. MATERIAL AND METHODS: We recorded clinical data and reviewed all imaging studies of 11 patients with JXG of the head and neck. Ultrasonography (US) alone was performed in 1 patient; MRI alone in 6 patients; US and MRI in 1 patient; and US, CT, and MRI in 3 patients. We evaluated the following characteristics in all studies: location and number of lesions, echogenicity and vascularization on US, density on CT, signal intensity on T 1 - and T 2 -weighted images, ADC and enhancement on MRI, and tumor boundaries and bone involvement. RESULTS: Lesions were well-defined in 9 cases, and bone erosion was present in 2. On US, lesions were hypoechoic or hyperechoic and with or without vascularization. On CT, lesions were hyper-dense, with no calcification. On MRI, lesions were mildly hyper-intense or iso-intense on T 1 -weighted images in 8 of 9 patients, hypo-intense on T2-weighted images in 7 of 10, low ADC in 7 of 9, and enhancement in 7 of 7. CONCLUSIONS: The diagnosis of extra cutaneous JXG may be proposed, with the following suggestive criteria: age < 1 year, well-defined lesion, mild hyper-intensity on T 1 -weighted images, hypo-intensity on T 2 -weighted images, low ADC, enhancement, and possible adjacent bone involvement.
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Cabeza , Imagen por Resonancia Magnética , Ultrasonografía , Xantogranuloma Juvenil , Humanos , Xantogranuloma Juvenil/diagnóstico por imagen , Xantogranuloma Juvenil/patología , Masculino , Femenino , Preescolar , Lactante , Niño , Imagen por Resonancia Magnética/métodos , Ultrasonografía/métodos , Cabeza/diagnóstico por imagen , Cabeza/patología , Cuello/diagnóstico por imagen , Cuello/patología , Tomografía Computarizada por Rayos X , AdolescenteRESUMEN
The number of healthcare workers occupationally exposed to ionizing radiation (IR) is increasing every year. As health effects from exposure to low doses IR have been reported, radiation protection (RP) in the context of occupational activities is a major concern. This study aims to assess the compliance of healthcare workers with RP policies, according to their registered cumulative dose, profession, and perception of radiation self-exposure and associated risk. Every healthcare worker from one of the participating hospitals in France with at least one dosimetric record for each year 2009, 2014, and 2019 in the SISERI registry was included and invited to complete an online questionnaire including information on the worker's occupational exposure, perception of IR-exposure risk and RP general knowledge. Hp(10) doses were provided by the SISERI system. Multivariate logistic regressions were used. Dosimeter wearing and RP practices compliance were strongly associated with 'feeling of being IR-exposed' (OR = 3.69, CI95% 2.04-6.66; OR = 4.60, CI95% 2.28-9.30, respectively). However, none of these factors was associated with RP training courses attendance. The main reason given for non-compliance is unsuitability or insufficient numbers of RP devices. This study provided useful information for RP policies. Making exposed workers aware of their own IR-exposure seems to be a key element to address in RP training courses. This type of questionnaire should be introduced into larger epidemiological studies. Dosimeter wearing and RP practices compliance are associated to feeling being IR-exposed. RP training courses should reinforce workers' awareness of their exposure to IR.
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Exposición Profesional , Protección Radiológica , Humanos , Conocimientos, Actitudes y Práctica en Salud , Personal de Salud , Radiometría , Radiación Ionizante , Hospitales , Exposición Profesional/prevención & control , Exposición Profesional/análisisRESUMEN
BACKGROUND: Advances in surgical and neonatal care have led to improved survival of patients with Åsophageal atresia (OA) over time. Morbidity remains significant, with one-third of patients being affected by a postoperative complication. Several aspects of management are not consensual, such as the use of Åsophagogram before starting oral feeding. METHODS: We conducted a multicenter retrospective study, including all children with OA that underwent a primary anastomosis in the first days of life, between 2012 and 2018 in five French centers, to determine the usefulness of postoperative Åsophagogram during the 10 days after early primary repair of OA to diagnose the anastomotic leak and congenital Åsophageal stenosis. RESULTS: Among 225 included children, 90 (40%) had a routine Åsophagogram and 25 (11%) had an anastomotic leak, clinically diagnosed before the scheduled Åsophagogram in 24/25 (96%) children at median postoperative day 4. Ten patients had associated congenital Åsophageal stenosis diagnosed on the Åsophagogram in only 30% of cases. CONCLUSION: Early Åsophagogram is rarely useful in the diagnosis of an anastomotic leak, which is clinically diagnosed before performing an Åsophagogram in the majority of cases. The need for a postoperative Åsophagogram should be evaluated on a case-by-case basis. IMPACT: Early Åsophagogram is not helpful in the diagnosis of an anastomotic leak in the majority of cases. An anastomotic leak is most often diagnosed clinically before performing an Åsophagogram. Early postoperative Åsophagogram could be helpful for the diagnosis of congenital Åsophageal stenosis. However, dysphagia occurs later and early diagnosis of congenital Åsophageal stenosis has no impact on the management and outcome of asymptomatic children. Indication of postoperative Åsophagogram has to be evaluated on a case-by-case basis.
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Atresia Esofágica , Estenosis Esofágica , Recién Nacido , Niño , Humanos , Atresia Esofágica/diagnóstico por imagen , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Estenosis Esofágica/diagnóstico por imagen , Estenosis Esofágica/cirugía , Estenosis Esofágica/complicaciones , Fuga Anastomótica/diagnóstico por imagen , Fuga Anastomótica/etiología , Estudios Retrospectivos , Complicaciones PosoperatoriasRESUMEN
OBJECTIVES: Health workers exposed to ionizing radiation account for + 50% of workers exposed to man-made radiation in France. Over the last decade, the use of radiation in medicine has increased due to the introduction of new practices. The EXposition des Professionnels de santE aux RayonnemenTs ioniSants study aims to evaluate and characterize the trends in radiation exposure of health workers in France between 2009 and 2019. METHODS: This retrospective study includes all health workers with at least one dosimetric record in the system for occupational dosimetry registration (Système d'information de la surveillance de l'exposition aux rayonnements ionisants) database for each of the years 2009, 2014, and 2019, in the hospitals included in the study. Individual external doses and socio-professional data were collected. Statistical analyses include descriptions, graphs, and logistic regressions. RESULTS: A total of 1457 workers were included (mean age: 39.8 years, 59% women). The average exposure significantly decreased between 2009 and 2019 (-0.008 mSv/year, p < 0.05). There were large discrepancies in trends according to professions, departments, hospitals, and gender. Over the 10-year study period, radiologic technologists and physicians were the most exposed (0.15 mSv (95%CI 0.14-0.16) and 0.13 mSv (0.06-0.21), respectively), but their exposure tended to decrease. Workers in nuclear medicine departments had the highest radiation exposure (0.36 mSv (0.33-0.39)), which remained stable over time. Thirty-eight percent of recorded doses were nonzero in 2009, decreasing to 20% in 2019. CONCLUSIONS: This study allowed to identify physicians and radiologic technologists in nuclear medicine departments as the most exposed medical workers in France, and to show an overall decrease trend in radiation exposure. This should be instructive for radiation monitoring and safety of exposed medical workers. KEY POINTS: ⢠Radiation exposure of healthcare workers in most medical departments has steadily decreased between 2009 and 2019 in several French hospitals. ⢠The number of zero doses consistently increased during the study period. ⢠Workers in nuclear medicine departments are the most exposed, especially radiologic technologists and physicians.
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Exposición Profesional , Exposición a la Radiación , Monitoreo de Radiación , Humanos , Femenino , Adulto , Masculino , Dosis de Radiación , Estudios Retrospectivos , Radiación Ionizante , Cuerpo MédicoRESUMEN
BACKGROUND: Prenatal diagnoses of cystic malformations of the posterior fossa mainly encompass arachnoid cysts, Blake's pouch cysts and Dandy-Walker syndrome. To date, vermian cysts have not been reported prenatally. OBJECTIVES: To report a series of fetuses with a vermian cyst. MATERIALS AND METHODS: This was a single-center retrospective study conducted from 2012 to 2021. We included all fetuses presenting with a vermian cyst and excluded all other types of posterior fossa cyst. The cyst was visible at prenatal ultrasound (US) and/or magnetic resonance imaging (MRI). Postnatal imaging and/or clinical outcome data were available. RESULTS: Sixteen fetuses fulfilled the inclusion criteria with a strong female predominance (n=13). US and MRI were performed at a mean gestational age of 29+5 and 33+1 weeks, respectively. In all patients, the cyst was in the vermian horizontal fissure. The mean longest dimension was about 10 mm. The vermis and other posterior fossa structures were otherwise normal. At postnatal imaging, 13 children underwent brain imaging including 11 MRIs with complete regression (n=9), stability (n=1) and increase in size (n=3) of the cyst. Psychomotor development was normal in 14 children. One child (with an inner ear malformation) showed a slight delay in walking and language acquisition. Slight walking ataxia was present in another child. CONCLUSION: We report 16 fetuses with posterior fossa cysts located within the vermis at the level of the horizontal fissure, diagnosed at US and/or MRI and carrying an overall excellent neurological prognosis.
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Quistes , Síndrome de Dandy-Walker , Malformaciones del Sistema Nervioso , Embarazo , Niño , Humanos , Femenino , Lactante , Masculino , Estudios Retrospectivos , Diagnóstico Prenatal/métodos , Síndrome de Dandy-Walker/diagnóstico , Síndrome de Dandy-Walker/patología , Fosa Craneal Posterior/anomalías , Fosa Craneal Posterior/diagnóstico por imagen , Fosa Craneal Posterior/patología , Imagen por Resonancia Magnética/métodos , Ultrasonografía Prenatal/métodosRESUMEN
BACKGROUND: The SIOP-Renal Tumor Study Group (RTSG) does not advocate invasive procedures to determine histology before the start of therapy. This may induce misdiagnosis-based treatment initiation, but only for a relatively small percentage of approximately 10% of non-Wilms tumors (non-WTs). MRI could be useful for reducing misdiagnosis, but there is no global consensus on differentiating characteristics. PURPOSE: To identify MRI characteristics that may be used for discrimination of newly diagnosed pediatric renal tumors. STUDY TYPE: Consensus process using a Delphi method. POPULATION: Not applicable. FIELD STRENGTH/SEQUENCE: Abdominal MRI including T1- and T2-weighted imaging, contrast-enhanced MRI, and diffusion-weighted imaging at 1.5 or 3 T. ASSESSMENT: Twenty-three radiologists from the SIOP-RTSG radiology panel with ≥5 years of experience in MRI of pediatric renal tumors and/or who had assessed ≥50 MRI scans of pediatric renal tumors in the past 5 years identified potentially discriminatory characteristics in the first questionnaire. These characteristics were scored in the subsequent second round, consisting of 5-point Likert scales, ranking- and multiple choice questions. STATISTICAL TESTS: The cut-off value for consensus and agreement among the majority was ≥75% and ≥60%, respectively, with a median of ≥4 on the Likert scale. RESULTS: Consensus on specific characteristics mainly concerned the discrimination between WTs and non-WTs, and WTs and nephrogenic rest(s) (NR)/nephroblastomatosis. The presence of bilateral lesions (75.0%) and NR/nephroblastomatosis (65.0%) were MRI characteristics indicated as specific for the diagnosis of a WT, and 91.3% of the participants agreed that MRI is useful to distinguish NR/nephroblastomatosis from WT. Furthermore, all participants agreed that age influenced their prediction in the discrimination of pediatric renal tumors. DATA CONCLUSION: Although the discrimination of pediatric renal tumors based on MRI remains challenging, this study identified some specific characteristics for tumor subtypes, based on the shared opinion of experts. These results may guide future validation studies and innovative efforts. LEVEL OF EVIDENCE: 3 Technical Efficacy Stage: 3.
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Neoplasias Renales , Radiología , Tumor de Wilms , Técnica Delphi , Imagen de Difusión por Resonancia Magnética , Humanos , Neoplasias Renales/diagnóstico por imagenRESUMEN
OBJECTIVES: Increased risks of central nervous system (CNS) tumors and leukemia associated with computed tomography (CT) exposure during childhood have been reported in recent epidemiological studies. However, no evidence of increased risks was suggested in a previous analysis of the French CT cohort. This study benefits from an updated cohort with a longer follow-up and a larger sample size of patients. METHODS: The patients were followed from the date of their first CT (between 2000 and 2011) until their date of cohort exit defined as the earliest among the following: 31 December 2016, date of death, date of first cancer diagnosis or date of their 18th birthday. Cancer incidence, vital status, cancer predisposing factors (PFs), and additional CT scans were collected via external national databases. Hazard ratios (HRs) associated to cumulative organ doses and sex were estimated from Cox models. RESULTS: At the end of follow-up, mean cumulative doses were 27.7 and 10.3 mGy for the brain and the red bone marrow (RBM), respectively. In patients without PFs, an HR per 10 mGy of 1.05 (95% CI: 1.01-1.09) for CNS tumors, 1.17 (95% CI: 1.09-1.26) for leukemia, and 0.96 (95% CI: 0.63-1.45) for lymphoma was estimated. These estimates were not modified by the inclusion of CT scans performed outside the participating hospitals or after the inclusion period. CONCLUSIONS: This study shows statistically significant dose-response relationships for CNS tumors and leukemia for patients without PFs. KEY POINTS: ⢠Computed tomography is the most important contributor to the collective dose for diagnostic imaging to the French population. ⢠Concerns have been raised about possible cancer risks, particularly after exposure to CT in childhood, due to the greater radiation sensitivity of children and to their longer life expectancy. ⢠Analysis of the updated French CT cohort shows statistically significant dose-response relationships for CNS tumors and leukemia.
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Neoplasias del Sistema Nervioso Central , Leucemia , Neoplasias Inducidas por Radiación , Niño , Estudios de Cohortes , Humanos , Incidencia , Neoplasias Inducidas por Radiación/epidemiología , Neoplasias Inducidas por Radiación/etiología , Dosis de Radiación , Tomografía Computarizada por Rayos X/efectos adversos , Tomografía Computarizada por Rayos X/métodosRESUMEN
Shwachman-Diamond syndrome (SDS) is a recessive disorder typified by bone marrow failure and predisposition to hematological malignancies. SDS is predominantly caused by deficiency of the allosteric regulator Shwachman-Bodian-Diamond syndrome that cooperates with elongation factor-like GTPase 1 (EFL1) to catalyze release of the ribosome antiassociation factor eIF6 and activate translation. Here, we report biallelic mutations in EFL1 in 3 unrelated individuals with clinical features of SDS. Cellular defects in these individuals include impaired ribosomal subunit joining and attenuated global protein translation as a consequence of defective eIF6 eviction. In mice, Efl1 deficiency recapitulates key aspects of the SDS phenotype. By identifying biallelic EFL1 mutations in SDS, we define this leukemia predisposition disorder as a ribosomopathy that is caused by corruption of a fundamental, conserved mechanism, which licenses entry of the large ribosomal subunit into translation.
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Mutación , Factores de Elongación de Péptidos/genética , Factores de Iniciación de Péptidos/biosíntesis , Ribonucleoproteína Nuclear Pequeña U5/genética , Síndrome de Shwachman-Diamond/genética , Síndrome de Shwachman-Diamond/metabolismo , Adolescente , Animales , Células Cultivadas , Análisis Mutacional de ADN , Modelos Animales de Enfermedad , Susceptibilidad a Enfermedades , Femenino , Estudio de Asociación del Genoma Completo , Humanos , Lactante , Masculino , Ratones , Ratones Transgénicos , Modelos Moleculares , Linaje , Factores de Elongación de Péptidos/química , Factores de Elongación de Péptidos/metabolismo , Fenotipo , Conformación Proteica , Ribonucleoproteína Nuclear Pequeña U5/química , Ribonucleoproteína Nuclear Pequeña U5/metabolismo , Síndrome de Shwachman-Diamond/diagnóstico , Relación Estructura-Actividad , Secuenciación Completa del GenomaRESUMEN
BACKGROUND: In severe obesity, left ventricular (LV) and right ventricular (RV) remodeling and contractile dysfunction have been documented, but less is known regarding left atrial (LA) dysfunction and its association with LV/RV remodeling, especially in children. PURPOSE: To assess the effects of severe childhood obesity on cardiac function by using multichamber strain analysis with MRI. STUDY TYPE: Prospective. SUBJECTS: Forty-five children aged 7-18 years (including 20 with severe obesity, defined as a body mass index values above the 99th percentile). FIELD STRENGTH: 5 T. SEQUENCE: Steady-state-free-precession (SSFP) images in short-axis views and longitudinal two- and four-chamber views. ASSESSMENT: Cardiac strain measurements were derived from standard SSFP cine images by using a dedicated MR imaging feature tracking software. Inter- and intra-rater reliability were evaluated. STATISTICAL TESTS: Independent sample t test, Spearman's correlation coefficient, principal component analysis, Bland-Altman analysis, and intra-class correlation coefficients (ICC). A P value <0.05 was considered statistically significant. RESULTS: As compared to children without obesity, those with obesity showed significantly reduced LA reservoir function (22.2% ± 6.4% vs. 33.8% ± 9.0%) and contractile function (5.4% ± 3.2% vs. 13.3% ± 8.0%) as well as significantly decreased absolute values for LA longitudinal strain in reservoir and contraction phases and LA radial motion fraction in reservoir and contraction phases. Children with severe obesity showed significantly reduced absolute RV radial motion fraction (-10.6% ± 2.9% vs. -18.2% ± 2.9%) and circumferential strain (-10.6% ± 2.9% vs. -16% ± 2.5%) as well as higher LV mass index (28.7% ± 5.1% vs. 21.7 ± 4.6 g/m2 ) along with significantly reduced LV ejection fraction (56.4% ± 3.9% vs. 60% ± 4.1%), LV radial strain (56% ± 6% vs. 61.8% ± 11.3%), and longitudinal strain (-17.8% ± 1.8% vs. -20.3% ± 3.2%). Reliability was good to excellent, with ICC ranging from 79.1% to 97.7%. DATA CONCLUSION: MR feature-tracking strain analysis revealed multichamber dysfunction in severely obese children with impaired LA reservoir and atrial contraction phases, which suggest an early loss in the compensatory ability of atrial contraction with severe obesity. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY STAGE: 3.
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Obesidad Mórbida , Obesidad Infantil , Adolescente , Niño , Humanos , Imagen por Resonancia Magnética , Imagen por Resonancia Cinemagnética , Obesidad Infantil/complicaciones , Obesidad Infantil/diagnóstico por imagen , Estudios Prospectivos , Reproducibilidad de los Resultados , Función Ventricular IzquierdaRESUMEN
RATIONALE: Three-dimensional reconstruction (3DR) of preoperative images may improve the presurgical assessment of tumours prior to removal. We aimed to analyse the advantages and discrepancies of preoperative 3DR in paediatric tumours. METHODS: We conducted a prospective observational study from 2016 to 2019, including patients with thoraco-abdominal tumours having predictable surgical risks on preoperative images (encasement of vessels posing vascular risks, ie, neuroblastic and soft tissue tumours or parenchyma preservation of the invaded organ, ie, liver and kidney). A comparison of 2D/3DR and surgical findings was performed. RESULTS: Twenty-four patients, with a median age at surgery of 68.2 months (13 days-203 months), were operated on for neuroblastoma (n = 7), renal tumour (n = 7), hepatic tumour (n = 4) and others (n = 6; bone sarcoma of the iliac branch, abdominal lymph nodes of a recurrent testicular germ cell tumour, pseudoinflammatory tumour of the omentum, thoracic lipoblastoma, desmoplastic tumour, solid and pseudopapillar tumour of the pancreas). Reconstruction was of poor quality in two patients with renal tumours because computed tomography (CT) had no excretory phase. Discrepancies between 3DR and surgical findings occurred in two patients, one because of poor assessment of caliceal infiltration by renal nodules and the other because of inadequate reconstruction of renal vein thrombosis. For all the other tumours, 3DR improved the visualisation and precise location of vessels during surgery. CONCLUSION: High-quality preoperative images are mandatory to provide the best 3DR. In the majority of cases, 3DR is of significant help during surgery to better identify vascular structures within tumours and preserve parenchyma.
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Procesamiento de Imagen Asistido por Computador/métodos , Imagenología Tridimensional/métodos , Neoplasias/patología , Cuidados Preoperatorios , Tomografía Computarizada por Rayos X/métodos , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Neoplasias/diagnóstico por imagen , Neoplasias/cirugía , Pronóstico , Estudios ProspectivosRESUMEN
BACKGROUND: Hordeum murinum is a specie of grass rarely reported among the aspirated foreign body. It has high tissue penetration power and may cause lung damages. CASE PRESENTATION: We report the case of a 15-month-old girl who choke while playing in the grass without any evident cause. This episode was immediately followed by vomiting and coughing with traces of blood. While she was fine during the following week, she relapsed at day (D) 7 with fever. At D10, she was finally hospitalized for signs of respiratory distress. The chest CT-scan revealed a voluminous right sub pleural empyema with an aerial component, responsible for the collapse of the right lower lobe, and complicated by a pneumopleurocutaneous fistula to the right paravertebral muscles. Intravenous antibiotics were prescribed, but no invasive procedure was performed. At D18, the spikelet of a false barley spontaneously externalized through the fistula. Evolution was favorable thereafter with disappearance of the fever and progressive decrease of the biological inflammatory syndrome. The follow-up at 4 months was reassuring, with normal clinical evaluation, and complete regression of the empyema on the chest X-rays. CONCLUSIONS: Hordeum murinum is a rare type of foreign body, and the aspiration often goes unnoticed. In these peculiar cases, CT-scans can be as informative as bronchoscopies, and the evolution is usually favorable after fistulization.
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Fístula , Cuerpos Extraños , Hordeum , Broncoscopía , Humanos , Lactante , PulmónRESUMEN
A wide range of craniofacial malformations can be diagnosed in utero using ultrasonography. However, even with highly experienced sonographers and diagnostic physicians and optimal conditions of examination, some anatomical structures cannot be properly analyzed by this technique. The aim of this pictorial essay is to show the additional value of fetal magnetic resonance imaging and computed tomography in this setting and to illustrate the role of these modalities in facial clefts; craniosynostosis; ear, eye and nose abnormalities; otomandibular dysplasias; and facial cephaloceles.
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Anomalías Craneofaciales , Anomalías Craneofaciales/diagnóstico por imagen , Cara/diagnóstico por imagen , Feto , Humanos , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVES: The aim of the study was to report a subtype of dysraphism designated as myelic limited dorsal malformation (MyeLDM) and to describe its characteristics at prenatal ultrasound (US). METHODS: It was a retrospective study from 2014 to 2020 based on second-line US evaluation of patients referred to our institution for myelomeningocele (MMC). Magnetic resonance imaging and acetylcholine esterase evaluation in the amniotic fluid were also offered. Major and minor criteria for open and closed dysraphism were defined and recorded for each patient. Patients were included as MyeLDM when both criteria of closed and open dysraphism were observed in the same fetus. Correlations were obtained with the postpartum data. RESULTS: Twenty patients fulfilled the inclusion criteria, some of them being very close to MMC, others very close to limited dorsal myeloschisis (LDM), and others lying in between. There were 13 live-born neonates and 7 terminations of pregnancy. Correlations between prenatal and postpartum data were overall very good. CONCLUSION: Our series describe the ultrasonographic characteristics of an intermediate type of dysraphism and suggest that there is a continuum between MMC and LDM with numerous possibilities of hybrid forms (MyeLDM) sharing characteristics of both open and closed dysraphisms.
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Meningomielocele , Disrafia Espinal , Líquido Amniótico , Femenino , Humanos , Recién Nacido , Imagen por Resonancia Magnética , Meningomielocele/diagnóstico por imagen , Embarazo , Estudios RetrospectivosRESUMEN
COPA (coatomer subunit α) syndrome is a newly recognised cause of interstitial lung disease in children and adults, frequently associated with arthritis and renal dysfunction. We report a 11-year-old girl with disease limited to major pulmonary haemosiderosis manifesting at the age of 2 years, due to a heterozygous p.(Arg233His) mutation in COPA Her interferon (IFN) signature was elevated (10.312 and 12.429, healthy <2.466), as was the level of serum IFNα (211 fg/mL, healthy <10 fg/mL). STAT1 phosphorylation in T lymphocytes and monocytes was increased as compared with healthy controls. Based on these results she was treated with the JAK1/2 inhibitor ruxolitinib, which resulted in reduction in IFN signalling and appeared to be associated with partial though incomplete decrease in the severity of her pulmonary disease. Patients with alveolar haemorrhage of unknown origin should be considered for COPA screening. Functional tests can help to personalise patient therapy.
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Hemorragia/tratamiento farmacológico , Hemosiderosis/tratamiento farmacológico , Enfermedades Pulmonares/tratamiento farmacológico , Pirazoles/uso terapéutico , Niño , Femenino , Hemosiderosis/genética , Humanos , Enfermedades Pulmonares/genética , Nitrilos , Pirimidinas , Hemosiderosis PulmonarRESUMEN
This case series examines cardiac MRI findings in four children and adolescents admitted to intensive care in April 2020 for multisystem inflammatory syndrome and Kawasaki disease-like features related to coronavirus disease 2019 (COVID-19). Acute myocarditis occurred less than 1 week after onset of fever and gastrointestinal symptoms. Physical examination showed rash and cheilitis or conjunctivitis. All patients recovered after intravenous immunoglobulin therapy. Severe acute respiratory syndrome coronavirus 2 reverse transcription polymerase chain reaction was negative in nasopharyngeal, stool, and respiratory samples and was positive on serology. Cardiac MRI showed diffuse myocardial edema on T2 short tau inversion-recovery sequences and native T1 mapping, with no evidence of late gadolinium enhancement suggestive of replacement fibrosis or focal necrosis. These findings favor postinfectious myocarditis in children and adolescents with COVID-19.
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Infecciones por Coronavirus/complicaciones , Imagen por Resonancia Magnética/métodos , Neumonía Viral/complicaciones , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico por imagen , Síndrome de Respuesta Inflamatoria Sistémica/etiología , Betacoronavirus , COVID-19 , Niño , Femenino , Corazón/diagnóstico por imagen , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Masculino , Miocarditis/diagnóstico por imagen , Miocarditis/etiología , Miocarditis/terapia , Pandemias , SARS-CoV-2 , Síndrome de Respuesta Inflamatoria Sistémica/terapia , Resultado del TratamientoRESUMEN
OBJECTIVES: To report on a dose survey conducted by the French societies of radiology and paediatric imaging (SFR and SFIPP) and the French public service expert in nuclear and radiological risks (IRSN), and to suggest new diagnostic reference levels (DRLs) for paediatric CT. METHODS: Dose reports concerning children aged 0-15 years and five CT procedures (brain, petrous bone, mediastinum, lung and abdomen-pelvis) performed in 2015-2016 were collected from 15 imaging departments on a voluntary basis. Volume CT dose index (CTDIvol) and dose-length product (DLP) were recorded for at least 10 patients per procedure, per centre and per age group. New DRLs were calculated as the 3rd quartiles of the distributions of the median values. RESULTS: Results from 3253 CT examinations were obtained. The exposure levels observed were much lower than for the previous surveys (2007-2008 and 2010-2013) and among the lowest values currently published. A good homogeneity between facilities was also observed. New DRLs are proposed for each procedure and age group. CONCLUSIONS: This new survey contributes to the continuing optimisation process in French paediatric CT practice. Its results have been recently used to update the national paediatric CT DRLs. KEY POINTS: ⢠New national diagnostic reference levels (DRLs) are proposed for several paediatric CT procedures. ⢠The DRLs proposed for brain, chest and abdomen-pelvis procedures are among the lowest published worldwide.
Asunto(s)
Tomografía Computarizada de Haz Cónico/métodos , Dosis de Radiación , Abdomen/diagnóstico por imagen , Adolescente , Encéfalo/diagnóstico por imagen , Niño , Preescolar , Femenino , Francia , Encuestas de Atención de la Salud , Humanos , Lactante , Recién Nacido , Masculino , Mediastino/diagnóstico por imagen , Pelvis/diagnóstico por imagen , Hueso Petroso/diagnóstico por imagen , Valores de Referencia , Tórax/diagnóstico por imagenRESUMEN
OBJECTIVES: To report on a dose survey conducted by the French societies of radiology and paediatric imaging (SFR and SFIPP) and the French public service expert in nuclear and radiological risks (IRSN), and to suggest new diagnostic reference levels (DRLs) for paediatric plain radiography and diagnostic fluoroscopy. METHODS: Dose reports concerning four radiography procedures and three diagnostic fluoroscopy procedures performed in 2015-2016 on children aged 0-15 years were collected from 16 imaging departments on a voluntary basis. Air kerma-area product (PKA) was recorded for at least 10 patients per procedure, per centre and per age group. New DRLs were calculated as the 3rd quartiles of the distributions of the median values. RESULTS: Results from 2249 radiography examinations and 1235 diagnostic fluoroscopy procedures were obtained. A noticeable variability in patient exposure between facilities was observed in diagnostic fluoroscopy and to a lesser extent in plain radiography. DRLs are proposed for each procedure and each age group. CONCLUSIONS: This study showed that the applicable national DRLs in plain radiography were obsolete and its results have been recently used by the French authorities to update them. In diagnostic fluoroscopy, large differences of doses were observed, even among departments accustomed to paediatric procedures. The new national DRLs will be useful for optimising diagnostic practice. KEY POINTS: ⢠New national diagnostic reference levels (DRLs) are proposed for several paediatric procedures in plain radiography and diagnostic fluoroscopy. ⢠Even between departments accustomed to paediatric procedures, substantial differences of practice were observed, especially in diagnostic fluoroscopy.
Asunto(s)
Fluoroscopía/métodos , Dosis de Radiación , Radiografía/métodos , Adolescente , Niño , Preescolar , Francia , Encuestas de Atención de la Salud , Humanos , Lactante , Recién Nacido , Valores de ReferenciaRESUMEN
The radiological distinction of Wilms tumor (WT) nodules from nephrogenic rests (NR) in patients with multifocal unilateral WT or bilateral disease is challenging. The study aims to compare the radiology assessment of kidney nodules with their final histology in 48 patients. The final histology of 118 nodules corresponded to the initial radiological diagnosis while 40 (25%) nodules were misdiagnosed, 20 being initially diagnosed WT on imaging were proved to be NR at histology. The size of nodules at diagnosis might help to distinguish WT from NR before surgery. Homogeneity did not seem to be a key feature.
Asunto(s)
Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patología , Riñón/diagnóstico por imagen , Riñón/patología , Tumor de Wilms/diagnóstico por imagen , Tumor de Wilms/patología , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
OBJECTIVE: This study was undertaken to describe the spectrum of lung computed-tomography (CT) findings in children with pulmonary Langerhans cell histiocytosis (PLCH) and to evaluate for this population the CT-scan nodule and cyst scores proposed by adult pulmonologists at diagnosis and during follow-up. METHODS: Among 175 children with PLCH identified in the French national population-based Langerhans cell histiocytosis cohort, 60 were retrospectively selected by the availability of CT for a central review by three pediatric radiologists. These 60 patients are representative of childhood PLCH for almost all clinical aspects, except a lower percentage of risk organ involvement (38% vs 54%; P = 0.05). RESULTS: The 60 children's chest CT scans (n = 218) were reviewed. At diagnosis, 63% of them had nodules, 53% had cysts, and 29% had both. The percentages of patients with nodules or cysts increased from diagnosis to peak disease activity, respectively, from 63% to 73% and from 53% to 66%. The costophrenic angle was involved in 71%. Patients with pneumothorax (25%) had a higher median cyst score. Alveolar consolidation was observed in 34%. Patients with low CT-scan nodule and cyst scores had no long-term pulmonary sequelae. CONCLUSIONS: Well-known characteristics of adult PLCH (nodules and cysts) were observed in children. The chest CT scores proposed by adult pulmonologists could easily be applied to childhood PLCH. Lesions in children, unlike those in adults, are frequently located near the costophrenic angles. Alveolar consolidation might be considered an atypical feature of childhood PLCH.