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EJHaem Nov 2023Lymphoid cancers are among the most frequent cancers diagnosed in adolescents and young adults (AYA), ranging from approximately 30%-35% of cancer diagnoses in... (Review)
Review
Lymphoid cancers are among the most frequent cancers diagnosed in adolescents and young adults (AYA), ranging from approximately 30%-35% of cancer diagnoses in adolescent patients (age 10-19) to approximately 10% in patients aged 30-39 years. Moreover, the specific distribution of lymphoid cancer types varies by age with substantial shifts in the subtype distributions between pediatric, AYA, adult, and older adult patients. Currently, biology studies specific to AYA lymphomas are rare and therefore insight into age-related pathogenesis is incomplete. This review focuses on the paradigmatic epidemiology and pathogenesis of select lymphomas, occurring in the AYA patient population. With the example of posttransplant lymphoproliferative disorders, nodular lymphocyte-predominant Hodgkin lymphoma, follicular lymphoma (incl. pediatric-type follicular lymphoma), and mediastinal lymphomas (incl. classic Hodgkin lymphoma, primary mediastinal large B cell lymphoma and mediastinal gray zone lymphoma), we here illustrate the current state-of-the-art in lymphoma classification, recent molecular insights including genomics, and translational opportunities. To improve outcome and quality of life, international collaboration in consortia dedicated to AYA lymphoma is needed to overcome challenges related to siloed biospecimens and data collections as well as to develop studies designed specifically for this unique population.
PubMed: 38024596
DOI: 10.1002/jha2.785 -
Journal of Pediatric Gastroenterology... Jan 2023
Topics: Humans; Pancreas; Pancreatectomy
PubMed: 36245077
DOI: 10.1097/MPG.0000000000003640 -
European Journal of Pediatrics Jan 2022In newborns with congenital diaphragmatic hernia (CDH), the mediastinal shift caused by the herniated organs negatively affects lung development. Assessment of the fetal...
In newborns with congenital diaphragmatic hernia (CDH), the mediastinal shift caused by the herniated organs negatively affects lung development. Assessment of the fetal magnetic resonance imaging (MRI) mediastinal shift angle (MSA) was shown to have an inverse correlation with the total fetal lung volume (TFLV), being associated with neonatal survival. However, a possible association with postnatal morbidity has never been investigated. We hypothesize that the degree of the mediastinal shift could be associated with higher respiratory and cardiocirculatory impairment, requiring intensive treatments and extended hospitalization in survivors. We retrospectively consider a cohort of isolated, left-sided CDH, for whom we calculated the MSA and the observed/expected (O/E) TFLV at fetal MRI. We performed a data collection regarding inotropic or vasoactive support, treatment with pulmonary vasodilators, mechanical ventilation, and length of stay. General linear models were performed. The MSA and O/E TFLV were inversely correlated (Pearson's coefficient - 0.65, p < 0.001), and deceased patients showed higher MSA values then survivors (p = 0.011). Among survivors, an increase in MSA was associated with longer pharmacological treatments (dobutamine: p = 0.016; dopamine: p = 0.049; hydrocortisone: p = 0.003; nitric oxide: p = 0.002; sildenafil: p = 0.039; milrinone: p = 0.039; oxygen: p = 0.066), and mechanical ventilation (p = 0.005), with an increasing trend in the length of hospitalization (p = 0.089).Conclusion: The MSA indirectly reflects lung hypoplasia and is associated with a higher neonatal intensity of cares. However, further studies are needed to consolidate the results.Trial registration: The study is an exploratory post hoc analysis of the registered NeoAPACHE protocol at ClinicalTrials.gov with the identifier NCT04396028. What is Known: • In congenital diaphragmatic hernia, the lung size, liver position, and defect side are the most common prenatal prognostic parameters used in clinical practice for morbidity and mortality prediction. • Lung hypoplasia, strictly associated with lung size, is estimated by observed/expected lung to head ratio and observed/expected total fetal lung volume with prenatal ultrasound and fetal magnetic resonance imaging, respectively. What is New: • A new, faster, more straightforward, and less operator-dependent tool to assess CDH severity could be the mediastinal shift angle calculation with fetal magnetic resonance imaging. • Postnatal clinical severity, considered as a postnatal cardiovascular and respiratory impairment that indirectly reflects lung hypoplasia, is associated with an increased mediastinal shift angle calculation.
Topics: Female; Gestational Age; Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Lung; Lung Volume Measurements; Magnetic Resonance Imaging; Pregnancy; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 34302257
DOI: 10.1007/s00431-021-04207-8 -
The Journal of Maternal-fetal &... Jan 2022To compare ultrasound (US) and magnetic resonance imaging (MRI) in the assessment of mediastinal shift angles (MSAs) in fetuses affected by isolated left congenital... (Observational Study)
Observational Study
OBJECTIVES
To compare ultrasound (US) and magnetic resonance imaging (MRI) in the assessment of mediastinal shift angles (MSAs) in fetuses affected by isolated left congenital diaphragmatic hernia (CDH). The use of MRI-MSA and US-MSA as prognostic factor for postnatal survival in fetal left CDH was also explored.
METHODS
This was an observational study of 29 fetuses with prenatally diagnosed isolated left CDH, assessed with both US and MRI examinations between January 2015 and December 2018. The US-MSA measurements performed within 2 weeks from the MRI assessment were considered for the analysis. The primary outcome was the postnatal survival rate.
RESULTS
No significant difference between US and MRI MSAs was detected ( = .419). Among the 29 cases, there were 21 alive infants, for an overall postnatal survival rate of 72.41%. After stratifying for postnatal survival, the best cutoffs with the highest discriminatory power in terms of sensibility and specificity were 42.1° for the US-MSA and 39.1° for the MRI-MSA. The performance of MRI-MSA in predicting postnatal survival was close to that of US-MSA in terms of sensitivity (62.5 versus 50.0%), specificity (80.9 versus 90.5%), positive predictive value (55.6 versus 66.7%), negative predictive value (85.0 versus 82.6%) and accuracy (75.9 versus 79.3%). There was no statistically significant difference between the two modalities ( > .05 for all).
CONCLUSIONS
MRI and US can be interchangeably used for the assessment of MSA in prenatally diagnosed isolated left CDH. Moreover, MSA measured by both US and MRI was confirmed to be correlated with perinatal outcome in terms of survival.
Topics: Female; Fetus; Gestational Age; Hernias, Diaphragmatic, Congenital; Humans; Lung; Magnetic Resonance Imaging; Pregnancy; Retrospective Studies; Ultrasonography; Ultrasonography, Prenatal
PubMed: 31973612
DOI: 10.1080/14767058.2020.1716714 -
Frontiers in Pediatrics 2022Fetal MRI has played an essential role in the evaluation and management of congenital diaphragmatic hernia (CDH). We aimed to investigate whether the mediastinal shift...
OBJECTIVE
Fetal MRI has played an essential role in the evaluation and management of congenital diaphragmatic hernia (CDH). We aimed to investigate whether the mediastinal shift angle (MSA) value was associated with the prognosis and the severity of left CDH and explore the relationship between the MSA value and fetal and neonatal cardiac structures and functions.
METHODS
From January 2012 to December 2020, the fetal MSA values of left CDH in our institution were retrospectively measured. Other prenatal parameters and clinical outcomes of them are collected. We also measured the fetal and postnatal echocardiography parameters to analyze linear correlation with MSA values.
RESULTS
A total of 94 patients with left CDH were included. MSA was significantly higher in the deceased group than in the survived group [((38.3 ± 4.7)° vs. 32.3 ± 5.3)°, < 0.001]. The MSA value of the high-risk defect group [CDH Study Group (CDHSG) C/D type] was significantly higher than that of the low-risk defect group [CDHSG A/B type; (36.0 ± 4.9)° vs. (30.1 ± 4.8)°, < 0.001]. The AUC for severity was 0.766 (95% CI, 0.661-0.851, < 0.0001) and the best cut-off value for MSA was 30.7°. Higher MSA correlates with decreased fetal -score of left ventricle (LV) width, the diameter of the mitral valve (MV), peak velocity of MV and tricuspid valve (TV), and neonatal LV end-diastolic diameter (LVEDD) and velocity of tricuspid regurgitation (TR; < 0.05).
CONCLUSION
A high MSA value can effectively predict high-risk defects and high mortality of left CDH. The higher the MSA value, the worse the neonatal conditions, the respiratory and cardiovascular prognosis. The MSA values could reflect the level of left heart underdevelopment, including decreased dimensions and diastolic dysfunction of the left ventricle.
PubMed: 35799694
DOI: 10.3389/fped.2022.907724 -
European Radiology Mar 2023To investigate the predictive value of mediastinal shift angle (MSA) in congenital diaphragmatic hernia (CDH).
OBJECTIVES
To investigate the predictive value of mediastinal shift angle (MSA) in congenital diaphragmatic hernia (CDH).
METHODS
A retrospective analysis was performed on 87 fetuses with prenatally diagnosed left-sided CDH (LCDH) and 88 controls. MSA was measured on magnetic resonance imaging (MRI). Lung area to head circumference ratio (LHR), ratio of the observed/expected LHR (O/E LHR), total fetal lung volume (TFLV), and observed/expected total fetal lung volume (O/E TFLV) were also measured. Correlation of MSA with pulmonary hypertension (PH), extracorporeal membrane oxygenation (ECMO) use, duration of hospitalization and survival in neonates with CDH was analyzed. Performance of MSA in prediction of postnatal outcomes was compared with LHR, O/E LHR, TFLV, and O/E TFLV.
RESULTS
There were significant differences in MSA values not only between the CDH group and the control group but also in CDH patients with different survival outcomes. MSA was inversely correlated with O/E LHR, O/E TFLV, and TFLV. MSA, LHR, O/E LHR, TFLV, and O/E TFLV could all be used to predict survival of CDH patients. In addition, the receiver operating characteristic (ROC) curve showed that the test performance of MSA was similar to that of TFLV, O/E TFLV, and O/E LHR, but superior to that of LHR. MSA was also correlated with PH, need for ECMO support, and duration of hospitalization.
CONCLUSION
MRI measurement of MSA can provide various prognostic information for prenatally diagnosed LCDH, in addition to postnatal survival. The test performance of MSA is similar to TFLV, O/E TFLV, and O/E LHR.
KEY POINTS
• Mediastinal shift angle (MSA) can be measured quickly and reproducibly on MRI images. • MSA could provide more prognostic information other than postnatal survival for LCDH with good test performance. • MSA should be incorporated into prenatal risk stratification for LCDH to improve planning of postnatal management.
Topics: Pregnancy; Female; Infant, Newborn; Humans; Hernias, Diaphragmatic, Congenital; Lung; Retrospective Studies; Lung Volume Measurements; Fetus; Hypertension, Pulmonary; Ultrasonography, Prenatal; Magnetic Resonance Imaging; Risk Assessment; Gestational Age
PubMed: 36180644
DOI: 10.1007/s00330-022-09142-2 -
Interactive Cardiovascular and Thoracic... Aug 2022Even after transplantation of favourable donor lungs, some recipients require prolonged weaning from mechanical ventilation, indicating a poor prognosis. We investigated...
OBJECTIVES
Even after transplantation of favourable donor lungs, some recipients require prolonged weaning from mechanical ventilation, indicating a poor prognosis. We investigated the effects of prolonged mechanical ventilation (PMV) for >14 days on the recovery and survival of patients who underwent cadaveric lung transplantation in relation to their physical traits.
METHODS
We retrospectively reviewed patients who underwent cadaveric lung transplantation (age ≥15 years) at a single centre between April 2015 and December 2020 and classified them into PMV and non-PMV groups (>14 and ≤14 days of mechanical ventilation postoperatively, respectively). The factors predicting PMV comprised clinical factors (e.g. marginal donor) and physical features, namely flat chest, narrow fourth intercostal space (length, <5 mm), mediastinal shift, thoracic mediastinal-occupying ratio (TMOR) >40% and sarcopenia, according to the logistic regression analysis. The log-rank test was used to examine the association between TMOR >40% and 3-year prognosis.
RESULTS
The PMV group comprised 17 (33%) of 51 recipients. Multivariable logistic analysis showed that the TMOR >40% (odds ratio, 7.3; 95% confidence interval, 1.3-40.1; P = 0.023) was an independent preoperative predictive factor for PMV postoperatively. Stepwise analysis revealed intraoperative extracorporeal membrane oxygenation and reoperation as postoperative predictive factors in addition to TMOR >40%. Recipients with TMOR >40% had significantly worse 3-year survival than other recipients (71.2% vs 100.0%, respectively; P = 0.008).
CONCLUSIONS
Recipients with a TMOR >40% may be long-term ventilator dependent and have a poor prognosis.
Topics: Adolescent; Cadaver; Humans; Lung Transplantation; Prognosis; Respiration, Artificial; Retrospective Studies; Time Factors
PubMed: 35445700
DOI: 10.1093/icvts/ivac106 -
Magnetic Resonance in Medical Sciences... Oct 2023The anterior mediastinum is the most common location of mediastinal tumors, and thymic epithelial tumors are the most common mediastinal tumors. It is important to...
The anterior mediastinum is the most common location of mediastinal tumors, and thymic epithelial tumors are the most common mediastinal tumors. It is important to differentiate thymic epithelial tumors from malignant lymphomas and malignant germ cell tumors because of the different treatment strategies. Dynamic contrast-enhanced MRI and diffusion-weighted imaging can provide additional information on the differential diagnosis. Chemical shift imaging can detect tiny fat tissues in the lesion and is useful in differentiating thymic hyperplasia from other solid tumors such as thymomas. MRI findings reflect histopathological features of mediastinal tumors, and a comprehensive evaluation of MRI sequences is important for estimation of the histopathological features of the tumor. In this manuscript, we describe the MRI findings of anterior mediastinal solid tumors and the role of MRI in the differential diagnosis.
Topics: Humans; Mediastinum; Mediastinal Neoplasms; Diagnosis, Differential; Thymus Neoplasms; Magnetic Resonance Imaging; Neoplasms, Glandular and Epithelial
PubMed: 35296589
DOI: 10.2463/mrms.rev.2021-0098 -
Cureus Nov 2022Emphysema is a progressive and degenerative lung disease that most commonly occurs due to many years of smoking or exposure to smoke and irritants. It is also seen in...
Emphysema is a progressive and degenerative lung disease that most commonly occurs due to many years of smoking or exposure to smoke and irritants. It is also seen in the congenital absence of the alpha-1-antitrypsin enzyme. Bullous emphysema is an advanced stage of the disease where strictures of the bronchi permit the inspired air to enter the bronchi but close on expiration, causing air retention and alveolar dilation, destruction, and atrophy. Multiple small bullae coalesce to form a giant bulla (defined as occupying more than one-third of the hemithorax), which causes respiratory symptoms and mediastinal shifting and leads to a poor general condition of the patient. Here, we present the cases of two patients diagnosed with bullous emphysema who presented within three months of each other. This article details the similarities and differences in the approach to both cases and the learning experience from these presentations, especially in acute symptomatology. Bullous emphysema is usually confused with a pneumothorax on a simple chest X-ray; hence, it is imperative to look for the lung margins and confirm the diagnosis using computed tomography of the thorax.
PubMed: 36505170
DOI: 10.7759/cureus.31182 -
Clinical Radiology Dec 2023Anterior mediastinal masses can be difficult to characterise on computed tomography (CT) due to the wide spectrum of normal appearances of thymic tissue as well as the... (Review)
Review
Anterior mediastinal masses can be difficult to characterise on computed tomography (CT) due to the wide spectrum of normal appearances of thymic tissue as well as the challenge of differentiating between benign and malignant pathologies. Additionally, attenuation of cystic mediastinal lesions can be misinterpreted on CT due to varying attenuation values. Anecdotally, non-vascular magnetic resonance imaging (MRI) of the thorax is underutilised across radiology departments in the UK, but has been shown to improve diagnostic certainty and reduce unnecessary surgical intervention. T2-weighted MRI is useful in confirming the cystic nature of lesions, whereas chemical shift techniques can be utilised to document the presence of macroscopic and intra-cellular fat and thus help distinguish between benign and malignant pathologies. In this review article, we present a practical approach to using MRI for the characterisation of anterior mediastinal lesions based on our clinical experience in a UK district general hospital.
Topics: Humans; Mediastinal Neoplasms; Magnetic Resonance Imaging; Thymus Gland; Tomography, X-Ray Computed; Mediastinum
PubMed: 37709611
DOI: 10.1016/j.crad.2023.08.015