Contemporary update on imaging of cystic renal masses with histopathological correlation and emphasis on patient management.

This article presents an updated review of cystic renal mass imaging. Most cystic renal masses encountered incidentally are benign and can be diagnosed confidently on imaging and require no follow-up. Hyperattenuating masses discovered at unenhanced or single-phase enhanced computed tomography (CT) measuring between 20-70 HU are indeterminate and can be further investigated first by using ultrasound and, then with multi-phase CT or magnetic resonance imaging (MRI); as the majority represent haemorrhagic/proteinaceous cysts (HPCs). Dual-energy CT may improve differentiation between HPCs and masses by suppressing unwanted pseudo-enhancement observed with conventional CT. HPCs can be diagnosed confidently when measuring >70 HU at unenhanced CT or showing markedly increased signal on T1-weighted imaging. Although the Bosniak criteria remains the reference standard for diagnosis and classification of cystic renal masses, histopathological classification and current management has evolved: multilocular cystic renal cell carcinoma (RCC) has been reclassified as a cystic renal neoplasm of low malignant potential, few Bosniak 2F cystic masses progress radiologically during follow-up; RCC with predominantly cystic components are less aggressive than solid RCC; and Bosniak III cystic masses behave non-aggressively. These advances have led to an increase in non-radical management or surveillance of cystic renal masses including Bosniak 3 lesions. Tubulocystic RCC is a newly described entity with distinct imaging characteristics, resembling a pancreatic serous microcystadenoma. Other benign cystic masses including: mixed epithelial stromal tumours (MEST) are now considered in the spectrum of cystic nephroma and angiomyolipoma (AML) with epithelial cysts (AMLEC) resemble a fat-poor AML with cystic components.

Evidence for Extending Anomalous Miocene Volcanism at the Edge of the East Antarctic Craton.

Using field observations followed by petrological, geochemical, geochronological, and geophysical data we infer the presence of a previously unknown Miocene subglacial volcanic center ~230 km from the South Pole. Evidence of volcanism is from boulders of olivine-bearing amygdaloidal/vesicular basalt and hyaloclastite deposited in a moraine in the southern Transantarctic Mountains. 40Ar/39Ar ages from five specimens plus U-Pb ages of detrital zircon from glacial till indicate igneous activity 25-17 Ma. The likely source of the volcanism is a circular -735 nT magnetic anomaly 60 km upflow from the sampling site. Subaqueous textures of the volcanics indicate eruption beneath ice or into water at the margin of an ice mass during the early Miocene. These rocks record the southernmost Cenozoic volcanism in Antarctica and expand the known extent of the oldest lavas associated with West Antarctic rift system. They may be an expression of lithospheric foundering beneath the southern Transantarctic Mountains.

Oral L-menthol reduces thermal sensation, increases work-rate and extends time to exhaustion, in the heat at a fixed rating of perceived exertion.

PURPOSE: The study investigated the effect of a non-thermal cooling agent, L-menthol, on exercise at a fixed subjective rating of perceived exertion (RPE) in a hot environment. METHOD: Eight male participants completed two trials at an exercise intensity between 'hard' and 'very hard', equating to 16 on the RPE scale at ~35 °C. Participants were instructed to continually adjust their power output to maintain an RPE of 16 throughout the exercise trial, stopping once power output had fallen by 30%. In a randomized crossover design, either L-menthol or placebo mouthwash was administered prior to exercise and at 10 min intervals. Power output, [Formula: see text]O2, heart rate, core and skin temperature was monitored, alongside thermal sensation and thermal comfort. Isokinetic peak power sprints were conducted prior to and immediately after the fixed RPE trial. RESULTS: Exercise time was greater (23:23 ± 3:36 vs. 21:44 ± 2:32 min; P = 0.049) and average power output increased (173 ± 24 vs. 167 ± 24 W; P = 0.044) in the L-menthol condition. Peak isokinetic sprint power declined from pre-post trial in the L-menthol l (9.0%; P = 0.015) but not in the placebo condition (3.4%; P = 0.275). Thermal sensation was lower in the L-menthol condition (P = 0.036), despite no changes in skin or core temperature (P > 0.05). CONCLUSION: These results indicate that a non-thermal cooling mouth rinse lowered thermal sensation, resulting in an elevated work rate, which extended exercise time in the heat at a fixed RPE.

Multiparametric MRI of the anterior prostate gland: clinical-radiological-histopathological correlation.

Anterior prostate cancer (APC) is defined as a tumour in which more than half of malignant tissue is located anterior to the urethra. APCs are increasingly recognized as clinically important, particularly in patients undergoing active surveillance and for patients with negative non-targeted systematic transrectal ultrasound (TRUS)-guided biopsies but with persistent clinical suspicion of cancer. Multiparametric (mp) MRI has a crucial role for the diagnosis of anterior tumours, eventual histological sampling of suspicious lesions using image-guided targeted biopsy techniques, and potentially, to improve local staging of disease. mpMRI is accurate for the detection of APC and for differentiation of tumour from other anterior prostatic structures including benign prostatic hyperplasia (BPH) and the anterior fibromuscular stroma (AFMS). Characterization and reporting of APC should rely on the recently revised Prostate Imaging and Data Reporting System (PI-RADS) version 2.0 document. T2-weighted (T2W) imaging is emphasized as the determining sequence for assessment of the anterior prostate and specific features for APC on T2W imaging include: ill-defined/spiculated margin, lenticular shape, anterior/inferior location, and growth pattern (invasion of urethra or AFMS and crossing midline). Functional imaging, mainly with diffusion-weighted imaging, is also contributory and improves the sensitivity for detection of APC compared to T2W imaging alone. APCs commonly show positive surgical margins after radical prostatectomy and staging of disease extent using conventional clinical parameters is limited. mpMRI may have a future role to improve local staging of APC. This review illustrates the importance of mpMRI in APC using a clinical-radiological-histopathological approach.

Multiparametric MRI of solid renal masses: pearls and pitfalls.

Functional imaging [diffusion-weighted imaging (DWI) and dynamic contrast enhancement (DCE)] techniques combined with T2-weighted (T2W) and chemical-shift imaging (CSI), with or without urography, constitutes a comprehensive multiparametric (MP) MRI protocol of the kidneys. MP-MRI of the kidneys can be performed in a time-efficient manner. Breath-hold sequences and parallel imaging should be used to reduce examination time and improve image quality. Increased T2 signal intensity (SI) in a solid renal nodule is specific for renal cell carcinoma (RCC); whereas, low T2 SI can be seen in RCC, angiomyolipoma (AML), and haemorrhagic cysts. Low b-value DWI can replace conventional fat-suppressed T2W. DWI can be performed free-breathing (FB) with two b-values to reduce acquisition time without compromising imaging quality. RCC demonstrates restricted diffusion; however, restricted diffusion is commonly seen in AML and in chronic haemorrhage. CSI must be performed using the correct echo combination at 3 T or T2* effects can mimic intra-lesional fat. Two-dimensional (2D)-CSI has better image quality compared to three-dimensional (3D)-CSI, but volume averaging in small lesions can simulate intra-lesional fat using 2D techniques. SI decrease on CSI is present in both AML and clear cell RCC. Verification of internal enhancement with MRI can be challenging and is improved with image subtraction. Subtraction imaging is prone to errors related to spatial misregistration, which is ameliorated with expiratory phase imaging. SI ratios can be used to confirm subtle internal enhancement and enhancement curves are predictive of RCC subtype. MR urography using conventional extracellular gadolinium must account for T2* effects; however, gadoxetic acid enhanced urography is an alternative. The purpose of this review it to highlight important technical and interpretive pearls and pitfalls encountered with MP-MRI of solid renal masses.

Ten uncommon and unusual variants of renal angiomyolipoma (AML): radiologic-pathologic correlation.

Classic (triphasic) renal angiomyolipoma (AML) is currently classified as a neoplasm of perivascular epithelioid cells. For diagnosis of AML, the use of thin-section non-contrast enhanced CT (NECT) improves diagnostic accuracy; however, identifying gross fat within a very small AML is challenging and often better performed with chemical-shift MRI. Although the presence of gross intra-tumoural fat is essentially diagnostic of AML; co-existing intra-tumoural fat and calcification may represent renal cell carcinoma (RCC). Differentiating AML from retroperitoneal sarcoma can be difficult when AML is large; the feeding vessel and claw signs are suggestive imaging findings. AML can haemorrhage, with intra-tumoural aneurysm size >5 mm a more specific predictor of future haemorrhage than tumor size >4 cm. Diagnosis of AML in the setting of acute haemorrhage is complex; comparison studies or follow-up imaging may be required. Not all AML contain gross fat and imaging features of AML without visible fat overlap with RCC; however, homogeneity, hyperdensity at NECT, low T2-weighted signal intensity and, microscopic fat are suggestive features. Patients with tuberous sclerosis often demonstrate a combination of classic and minimal fat AML, but are also at a slightly increased risk for RCC and should be imaged cautiously. Several rare pathological variants of AML exist including AML with epithelial cysts and epithelioid AML, which have distinct imaging characteristics. Classic AML, although benign, can be locally invasive and the rare epithelioid AML can be frankly malignant. The purpose of this review is to highlight the imaging manifestations of 10 uncommon and unusual variants of AML using pathological correlation.

Pitfalls of adrenal imaging with chemical shift MRI.

Chemical shift (CS) MRI of the adrenal glands exploits the different precessional frequencies of fat and water protons to differentiate the intracytoplasmic lipid-containing adrenal adenoma from other adrenal lesions. The purpose of this review is to illustrate both technical and interpretive pitfalls of adrenal imaging with CS MRI and emphasize the importance of adherence to strict technical specifications and errors that may occur when other imaging features and clinical factors are not incorporated into the diagnosis. When performed properly, the specificity of CS MRI for the diagnosis of adrenal adenoma is over 90%. Sampling the in-phase and opposed-phase echoes in the correct order and during the same breath-hold are essential requirements, and using the first echo pair is preferred, if possible. CS MRI characterizes more adrenal adenomas then unenhanced CT but may be non-diagnostic in a proportion of lipid-poor adenomas; CT washout studies may be able to diagnose these lipid-poor adenomas. Other primary and secondary adrenal tumours and supra-renal disease entities may contain lipid or gross fat and mimic adenoma or myelolipoma. Heterogeneity within an adrenal lesion that contains intracytoplasmic lipid could be due to myelolipoma, lipomatous metaplasia of adenoma, or collision tumour. Correlation with previous imaging, other imaging features, clinical history, and laboratory investigations can minimize interpretive errors.

A single centre service evaluation of patients' experiences participating in radiotherapy clinical trials during and post COVID-19 in Northern Ireland, UK.

INTRODUCTION: Radiotherapy (RT) clinical trials allow patients to access cutting-edge innovative cancer treatments. Clinical Research Therapy Radiographers (CRRs) play an important role in the management and care of RT trial patients. The COVID-19 pandemic caused major disruption to RT trial delivery. Measures to mitigate COVID-19 risk continue to have an effect on patient contact and communication within cancer centres in the United Kingdom (UK). This study aimed to explore patient perspectives regarding their recent RT trial experience in Northern Ireland (NI), UK. METHODS: A single centre service evaluation was conducted in NI. Patients who were recruited into a RT clinical trial from January 2020 to January 2023 were invited to participate. Surveys were posted to 50 participants in April 2023. Quantitative and qualitative data was captured and analysed using descriptive statistics and Braun and Clarke's six-step thematic analysis framework respectively. Ethical approval was obtained through Ulster University and the NHS Trust. RESULTS: Forty-three of the 50 invited participants responded (86%). Forty-two respondents (79%) had a prostate cancer diagnosis. Forty-one (98%) participants indicated that CRRs were always approachable, polite and courteous and would recommend taking part in a RT trial to friends and family. Identified areas for improvement included aspects regarding consent and participant decision-making. CONCLUSION: This study suggests that despite the implemented measures to suspend research and mitigate COVID-19 risk, patients remained highly satisfied with the quality of care that they received through their participation in RT trials. IMPLICATIONS FOR PRACTICE: The results of this service evaluation will facilitate maintenance and improvement of patient focused delivery of cancer trials within the host centre. This study builds on evidence highlighting the importance of the CRR role and role development for radiographers.

Patients' perspectives of the skills and competencies of therapy radiographers/radiation therapists (TRs/RTTs) in the UK, Portugal and Malta; a qualitative study from the SAFE Europe project.

INTRODUCTION: The role of the Therapy Radiographer/Radiation Therapist (TR/RTT) is to provide radiotherapy to patients with a cancer diagnosis. This includes, not only administration of treatment, but also management of side-effects and provision of support/care. Despite this role being consistent throughout Europe, there is currently no standardisation of education for TRs/RTTs. The SAFE EUROPE project aims to standardize TR/RTT education to enable 'safe and free exchange' of TRs/RTTs across Europe. Consequently, this study aims to explore patients' perspectives regarding the current skills and competencies of TRs/RTTs. METHODS: From May 2021 to February 2022, semi-structured interviews were conducted with patients who had recently received radiotherapy in the UK, Malta and Portugal. Ethical approval for this study was granted by the NHS Research Ethics Committee with additional local approvals obtained. RESULTS: Forty-eight participants from the UK (n = 18), Portugal (n = 19), and Malta (n = 11) completed interviews. Participants described high satisfaction with TRs'/RTTs' competence and skills in all three countries. The main theme arising from the analysis was the importance of trust building with TRs/RTTs. Six factors were identified as influencing levels of trust: communication; side-effect management; team consistency; relational skills; patient dignity; and competence. A small number of patients reported feeling rushed and not having their physical and emotional needs met by TRs/RTTs. CONCLUSION: This multicentre study demonstrated that patients perceive TRs/RTTs in the UK, Malta and Portugal as highly competent and skilled. Practical recommendations are provided to address identified deficits in practice, which can be addressed through adaptation of TR/RTT education/training and clinical practice. IMPLICATIONS FOR PRACTICE: Recommendations arising from this study are important to ensure that TRs/RTTs have transferable skills that provide consistently high quality care to patients throughout Europe.

Changes in physical performance during British Army Junior Entry, British Army Standard Entry and Royal Air Force Basic Training.

INTRODUCTION: The purpose was to quantify physical performance in men and women during British Army Junior Entry (Army-JE), British Army Standard Entry (Army-SE) and Royal Air Force (RAF) basic training (BT). DESIGN: Prospective longitudinal study. METHODS: 381 participants ((339 men and 42 women) n=141 Army-JE, n=132 Army-SE, n=108 RAF) completed a 2 km run, medicine ball throw (MBT) and isometric mid-thigh pull (MTP), pre-BT and post-BT. To examine changes in pre-BT to post-BT physical test performance, for each course, paired Student t-test and Wilcoxon test were applied to normally and non-normally distributed data, respectively, with effect sizes reported as Cohen's D and with rank biserial correlations, respectively. A one-way between-subjects analysis of variance (ANOVA) (or Welch ANOVA for non-normally distributed data) compared performance between quartiles based on test performance pre-BT. Where the main tests statistic, p value and effect sizes identified likely effect of quartile, post hoc comparisons were made using Games-Howell tests with Tukey's p value. Data are presented as mean±SD, with statistical significance set at p<0.05. RESULTS: During BT, 2 km run time improved by 13±46 s (-2.1%±8.1%), 30±64 s (-4.8%±12.3%) and 24±27 s (-4.5%±5.1%) for Army-JE, Army-SE and RAF, respectively (all p<0.005). MBT distance increased by 0.27±0.28 m (6.8%±7.0%) for Army-JE (p<0.001) and 0.07±0.46 m (2.3%±10.9%) for Army-SE (p=0.040), but decreased by 0.08±0.27 m (-1.4%±6.0%) for RAF (p=0.002). MTP force increased by 80±281 n (10.8%±27.6%) for Army-JE (p<0.001) and did not change for Army-SE (-36±295 n, -0.7%±20.6%, p=0.144) or RAF (-9±208 n, 1.0±17.0, p=0.603). For all tests and cohorts, participants in the lowest quartile of pre-BT performance scores demonstrated greater improvements, compared with participants in the highest quartile (except Army-JE MBT, ∆% change similar between all quartiles). CONCLUSIONS: Changes in physical performance were observed for the three fitness tests following the different BT courses, and recruits with the lowest strength and aerobic fitness experienced greatest improvements.

One-year follow-up changes in weight are associated with changes in blood pressure in young Mexican adults.

OBJECTIVE: Increasing overweight and obesity rates in Mexico have been associated with increases in mortality from cardiovascular disease (CVD). This study assessed changes in body mass index (BMI) and body weight over 1 year, and explored whether these were associated with changes in CVD risk factors of blood pressure and fasting glucose in a cohort of young Mexican adults. STUDY DESIGN: Longitudinal data were obtained from a cohort of young Mexican adults applying to college. METHODS: Data were collected from college applicants for the 2008 academic year who re-applied in 2009. In total, 795 college applicants aged 18-20 years, of both sexes (48% males and 52% females), were included in the study. The screen included height, weight, and systolic (SBP) and diastolic (DBP) blood pressure measurements plus a blood draw following an overnight fast for fasting glucose. RESULTS: At baseline, 31.8% of the participants were overweight or obese. The mean 1-year change in body weight and BMI were 0.80 kg and 0.35 kg/m(2), respectively. One-year changes in body weight and BMI were associated with increased SBP and DBP for both men and women (P < 0.05), independent of baseline BMI. A weight gain of 5% or more was positively associated with increases in blood pressure among women (P < 0.05), but not among men. A weight loss of 5% or more was associated with reductions in SBP among women. CONCLUSIONS: One-year changes in weight were associated with changes in blood pressure.

The impact of COVID-19 on the mental health of radiography staff and managers in Northern Ireland, UK: The radiography managers' perspective.

INTRODUCTION: Increasing evidence suggests that the COVID-19 pandemic has influenced the mental health of health professionals, including radiographers. Less is known about the effect of the pandemic on the mental health of radiography managers. Radiography managers have led their teams through the pandemic, making unpopular decisions to safeguard staff and patients. This study explores radiography managers' perceptions regarding the impact of the COVID-19 pandemic on the mental health of themselves and their staff. METHODS: Ethical approval was obtained from the NHS Research Ethics Committee (ID 287032). Eleven interviews were conducted with therapeutic and diagnostic radiography managers between March-April 2021. Written information was also included from a paediatric diagnostic radiography manager. Data was analysed independently by 2 researchers using thematic analysis. RESULTS: Three central themes emerged: 1) Factors perceived to have negatively influenced mental health, which included changing PPE guidance, restructuring of work conditions, social isolation, challenges to patient care and lack of quality vacation leave. 2) Factors perceived to have positively influenced mental health, which included witnessing staff resilience and team camaraderie. 3) Support provided for mental health. CONCLUSION: Managers felt that they had implemented appropriate strategies to support their staff throughout the first year of the pandemic and expressed feeling responsible for the wellbeing of their staff. Strong empathy was evident towards staff and their experiences. Despite the availability of mental health support services, managers felt that resources were underutilised by radiography teams. IMPLICATIONS FOR PRACTICE: Managers should be proactive in communicating their appreciation for their staff in an era where remote working can add to disconnect between staff and management. Mental health support services should be promoted and continually reviewed, to ensure that appropriate support services are maintained.

Impact of COVID-19 on service delivery in radiology and radiotherapy.

INTRODUCTION: The COVID-19 pandemic has had a profound impact on radiography services globally. The reshaping of service delivery continues to impact patient management and the experience of the radiography workforce should be evaluated to determine how effective service delivery can be maintained in the ongoing and post-pandemic world. METHODS: A mixed methods approach was adopted. Questionnaires, designed using Qualtrics (Qualtrics, Provo, UT) online survey software, were used to survey radiographers throughout Northern Ireland (NI). Semi-structured interviews were conducted with radiography service managers in the NHS and private sector in NI. All interviews were digitally recorded, transcribed and coded independently by 2 researchers. RESULTS: A total of 106 Radiographers completed the online survey i.e. 82 Diagnostic and 24 Therapeutic. Variations were reported regarding staff concern for contracting COVID-19 and passing it on. Clinical workload was reported to fluctuate during the early period of the pandemic, however, both diagnostic and therapeutic radiographers reported workloads which were higher than normal at the time of the data collection. Nine service managers participated in the interviews plus two band 8 superintendent radiographers. Staff faced many challenges whilst delivering services due to COVID-19. The two most frequently cited challenges included issues related to (i) Implementation of PPE and (ii) Changes to work practices. CONCLUSION: A pre-prepared pandemic plan should be established and stress tested for the future. The plan should be devised in consultation with both the public and private sector to determine the very best use of resources. IMPLICATIONS FOR PRACTICE: The radiography workforce has worked continuously throughout the pandemic and needs to be supported to deal with the potential increase in demand for services in the post-pandemic world.

The lessons learned working in diagnostic and therapeutic radiography departments through the COVID-19 pandemic in Northern Ireland, UK. What can we do differently the next time?

INTRODUCTION: Following the emergence of the COVID-19 pandemic in January 2020, a radical restructure of NHS services occurred, prioritising the acute needs of infected patients. This included suspending routine procedures, leading to an inevitable resurgence in the future, placing increased demands on the NHS, including diagnostic and therapeutic radiographers. With radiography departments already experiencing staff shortages due to COVID-19 related illnesses and vulnerable staff shielding, there is a need to implement plans within radiography departments to ensure their sustainability in the future. METHODS: A mixed methods study was undertaken in Northern Ireland, involving distribution of a survey to diagnostic and therapeutic radiographers alongside conducting interviews with radiography department managers. RESULTS: 106 radiographers completed the survey, with 9 radiography managers and 2 band eight superintendents participating in interviews. Over 60% of participants felt that morale declined in their departments, with the majority feeling that the pandemic had a negative impact on their physical or mental health and wellbeing. Managers felt that to improve staff morale and motivation, incentives need to be offered including remuneration, flexible working and support for professional development. CONCLUSION: Whilst predicting when the next wave of a COVID-19 variant or the next pandemic will occur is impossible, preparation and planning will help manage the situation better. This requires identifying clinical areas for expansion/retraction and having access to additional staff to meet the demands on the service to ensure all patients receive care not just those acutely ill. IMPLICATIONS FOR PRACTICE: This study has identified key lessons learned from the pandemic within the radiography departments. This will enable preparation and strategic planning for future pandemics.

Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency.

Autoimmune lymphoproliferative syndrome (ALPS) is mainly caused by defects in the CD95 pathway. Raised CD3+TCRαß+CD4-CD8- double negative T cells and impaired T cell apoptosis are hallmarks of the disease. In contrast, the B cell compartment has been less well studied. We found an altered distribution of B cell subsets with raised transitional B cells and reduced marginal zone B cells, switched memory B cells and plasma blasts in most of 22 analyzed ALPS patients. Moreover, 5 out of 66 ALPS patients presented with low IgG and susceptibility to infection revealing a significant overlap between ALPS and common variable immunodeficiency (CVID). In patients presenting with lymphoproliferation, cytopenia, hypogammaglobulinemia and impaired B cell differentiation, serum biomarkers were helpful in addition to apoptosis tests for the identification of ALPS patients. Our observations may indicate a role for apoptosis defects in some diseases currently classified as CVID.

Complex surgery and perioperative systemic therapy for genitourinary cancer of the retroperitoneum.

Objective: The purpose of the present guideline is to recommend surgical or systemic treatment for metastatic testicular cancer; T3b or T4, or node-positive, and metastatic renal cell cancer (rcc); and T3, T4, or node-positive upper tract urothelial (utuc) cancer. Methods: Draft recommendations were formulated based on evidence obtained through a systematic review of randomized controlled trials, comparative retrospective studies, and guideline endorsement. The draft recommendations underwent an internal review by clinical and methodology experts, and an external review by clinical practitioners. Results: The primary literature search yielded eight guidelines, five systematic reviews, and twenty-seven primary studies that met the eligibility criteria. Conclusions: Cytoreductive nephrectomy should no longer be considered the standard of care in patients with T3b or T4, or node-positive, and metastatic rcc. Eligible patients should be treated with systemic therapy and have their primary tumour removed only after review at a multidisciplinary case conference (mcc). Adjuvant sunitinib after surgery is not recommended. Patients with venous tumour thrombus should be considered for surgical intervention. Patients with T3, T4, or node-positive utuc should have their tumour removed without delay. Decisions concerning lymph node dissection should be done at a mcc and be based on stage, expertise, and imaging. Adjuvant systemic treatment is recommended for resected high-risk utuc. Patients with metastasis-positive testicular cancer with residual tumour after systemic treatment should be treated at specialized centres. For all complex retroperitoneal surgeries, the evidence shows that higher-volume centres are associated with lower rates of procedure-related mortality, and patients should be referred to higher-volume centres for surgical resection.

Age-Dependent Signal Intensity Changes in the Structurally Normal Pediatric Brain on Unenhanced T1-Weighted MR Imaging.

BACKGROUND AND PURPOSE: Various pathologic and nonpathologic states result in brain parenchymal signal intensity changes on unenhanced T1-weighted MR imaging. However, the absence of quantitative data to characterize typical age-related signal intensity values limits evaluation. We sought to establish a range of age-dependent brain parenchymal signal intensity values on unenhanced T1WI in a sample of individuals (18 years of age or younger) with structurally normal brains. MATERIALS AND METHODS: A single-center retrospective study was performed. Gadolinium-naïve pediatric patients with structurally normal MR brain imaging examination findings were analyzed (n = 114; 50% female; age range, 68 days to 18 years). ROI signal intensity measurements were obtained from the globus pallidus, thalamus, dentate nucleus, pons, and frontal lobe cortex and subcortical white matter. Multivariable linear regression was used to analyze the relationship between signal intensity values and age. RESULTS: Results demonstrated a statistically significant association between signal intensity values and linear age in all neuroanatomic areas tested, except the frontal gray matter, (P < .01). There were no statistically significant differences attributable to patient sex. CONCLUSIONS: Age-dependent signal intensity values were determined on unenhanced T1WI in structurally normal pediatric brains. Increased age correlated with increased signal intensity in all brain locations, except the frontal gray matter, irrespective of sex. The biologic mechanisms underlying our results remain unclear and may be related to chronologic changes in myelin density, synaptic density, and water content. Establishing age-dependent signal intensity parameters in the structurally normal pediatric brain will help clarify developmental aberrations and enhance gadolinium-deposition research by providing an improved understanding of the confounding effect of age.

Special article: chronic granulomatous disease in the United Kingdom and Ireland: a comprehensive national patient-based registry.

There are no epidemiological studies from the British Isles of chronic granulomatous disease, characterized by recurrent, life-threatening bacterial and fungal infections and inflammatory sequelae. Patients were enrolled in a national registry and medical records were analysed. Of 94 subjects, 69 had X-linked disease, 16 had autosomal recessive disease and nine were unknown. Prevalence was 7.5/million for 1990-99 and 8.5/million for 1980-89. Suppurative adenitis, abscesses and pneumonia presented commonly. Twenty-three of 30 patients who underwent high resolution computerized tomography had chronic respiratory disease. Inflammatory sequelae included bowel stricture and urogenital tract granulomata. Growth failure was common; 75% of those measured were below the population mean. All patients received prophylactic antibiotics and 93% anti-fungal prophylaxis. Interferon gamma was used to treat infection, but rarely as prophylaxis. Despite prophylaxis, estimated survival was 88% at 10 years but 55% at age 30 years. Morbidity remains significant, severe infectious complications common. Curative treatments including stem cell transplantation should be considered for patients with frequent or serious complications.

Mutations in CHD7 in patients with CHARGE syndrome cause T-B + natural killer cell + severe combined immune deficiency and may cause Omenn-like syndrome.

More than 11 genetic causes of severe combined immunodeficiency (SCID) have been identified, affecting development and/or function of T lymphocytes, and sometimes B lymphocytes and natural killer (NK) cells. Deletion of 22q11.2 is associated with immunodeficiency, although less than 1% of cases are associated with T-B + NK + SCID phenotype. Severe immunodeficiency with CHARGE syndrome has been noted only rarely Omenn syndrome is a rare autosomal recessive form of SCID with erythroderma, hepatosplenomegaly, lymphadenopathy and alopecia. Hypomorphic recombination activating genes 1 and 2 mutations were first described in patients with Omenn syndrome. More recently, defects in Artemis, RMRP, IL7Ralpha and common gamma chain genes have been described. We describe four patients with mutations in CHD7, who had clinical features of CHARGE syndrome and who had T-B + NK + SCID (two patients) or clinical features consistent with Omenn syndrome (two patients). Immunodeficiency in patients with DiGeorge syndrome is well recognized--CHARGE syndrome should now be added to the causes of T-B + NK + SCID, and mutations in the CHD7 gene may be associated with Omenn-like syndrome.

Service user involvement in radiotherapy and oncology education; the patient perspective.

INTRODUCTION: The Health & Care Professions Council (HCPC) recently mandated the integration of the service-user voice into all aspects of allied healthcare education in the UK. However, the potential benefits and harms of this integration to service users are largely unknown. This study aimed to determine service user perspectives on relaying their personal experience of the cancer treatment pathway to students in an undergraduate Radiotherapy and Oncology programme. METHODS: A qualitative study was conducted where seven patients led educational sessions with students and were interviewed (1:1) 1 week later using an iterative semi-structured format. RESULTS: Unanimously, the primary motivation for participation was the opportunity for patients to tell their unique story to positively influence the future behaviour and understanding of student healthcare professionals. Patients experiencing significant cancer-related psychological trauma reported reacting more emotionally to the experience but also reported particularly positive benefits from their participation, including therapeutic healing. CONCLUSION: Findings highlight the array of benefits to service users associated with teaching in allied healthcare education. Post traumatic growth (PTG) may also potentially occur through this type of intervention in certain participants and this warrants further investigation in future studies.