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Hereditary Hemorrhagic Telangiectasia: A Rare Cause of Anemia.
No full textHereditary hemorrhagic telangiectasias (HHT), also known as Osler-Weber-Rendu syndrome, is an uncommon genetic disorder. It is inherited as an autosomal dominant disorder with varying penetrance and expression. The diagnosis of HHT requires the presence of at least three out of four clinical criteria. These so-called Curaçao criteria include epistaxis, telangiectasias, visceral involvement, and a family history of HHT in a first-degree relative. Visceral involvement can involve the gastrointestinal (GI) tract, resulting in the development of GI telangiectasias. One of the complications is anemia due to the chronic blood loss from these vascular malformations. Here, we present a case of a 26-year-old male who was diagnosed with HHT. He initially had episodes of epistaxis but now presented to us with features of anemia. According to the patient, he didn't have epistaxis for the past many months and on his esophagogastroduodenoscopy (EGD) and colonoscopy, there was evidence of multiple small telangiectasias seen in his stomach, duodenum, and colon. He was managed with blood transfusion and was discharged on oral iron supplementation. This is a rare cause of anemia and should be evaluated if other features of HHT are present
Even gold standard methods of body water measurement are not always in perfect agreement.
No full textLong-Term Prospective Outcomes Of Patients With Non-Muscle Invasive Bladder Cancer Following Holmium Laser Ablation.
No full textOBJECTIVES
To evaluate the long-term tumour recurrence, progression and cancer-specific mortality of patients following holmium laser ablation of non-muscle invasive bladder cancer (NMIBC) under topical anaesthesia. To identify variables that help to predict risk of tumour recurrence, progression and cancer-specific mortality.
MATERIALS & METHOD
A prospective study of 251 consecutive patients with 399 recurrent NMIBC who underwent outpatient Ho:YAG laser ablation under topical anaesthesia was carried out between September 2006 to September 2018. All patients underwent primary TURBT to obtain histology. All patients completed a patient satisfaction questionnaire and visual analogue scale (VAS) for procedure-related pain.
RESULTS
All 251 patients were analysed with an average age of 75.2 years and follow-up of 69.8 months. The majority of patients had multiple comorbidities with an ASA of 3 or 4. The proportion of tumour grade 1,2 & 3 disease and tumour stage Ta and T1 was 43.4%, 41.5%, 15.1%, 78.5% and 21.5% respectively. Overall 57.8% of patients had at least one tumour recurrence with an average time of 16.3 months. Risk factors were tumour grade, number of tumours at time of first laser ablation, and tumour stage. 19.1% of patients demonstrated tumour progression with an average time of 51 months. Only 5.6% of patients had progression to MIBC and the cancer specific mortality (CSM) rate was 5.2% with an average time to death of 71.2 months. Risk factors for CSM were tumour grade, stage, and patients age. The average VAS for pain was 0.6 out of 10 with all patients willing to have the procedure repeated if required.
CONCLUSION
Laser ablation of NMIBC is a safe, effective and well tolerated long-term option for patients, particular in those that have moderate to severe co-morbidities. The long-term cancer outcomes are best for younger patients with primary low grade and low stage disease
Practical Guidance for Measuring and Reporting Surgical Margins in Vulvar Cancer.
No full textSurgical resection with free surgical margins is the cornerstone of successful primary treatment of vulvar squamous cell carcinoma (VSCC). In general reexcision is recommended when the minimum peripheral surgical margin (MPSM) is <8 mm microscopically. Pathologists are, therefore, required to report the minimum distance from the tumor to the surgical margin. Currently, there are no guidelines on how to make this measurement, as this is often considered straightforward. However, during the 2018 Annual Meeting of the British Association of Gynaecological Pathologists (BAGP), a discussion on this topic revealed a variety of opinions with regard to reporting and method of measuring margin clearance in VSCC specimens. Given the need for uniformity and the lack of guidance in the literature, we initiated an online survey in order to deliver a consensus-based definition of peripheral surgical margins in VSCC resections. The survey included questions and representative diagrams of peripheral margin measurements. In total, 57 pathologists participated in this survey. On the basis of consensus results, we propose to define MPSM in VSCC as the minimum distance from the peripheral edge of the invasive tumor nests toward the inked peripheral surgical margin reported in millimeters. This MPSM measurement should run through tissue and preferably be measured in a straight line. Along with MPSM, other relevant measurements such as depth of invasion or tumor thickness and distance to deep margins should be reported. This manuscript provides guidance to the practicing pathologist in measuring MPSM in VSCC resection specimens, in order to promote uniformity in measuring and reporting
Survey of Less Invasive Surfactant Administration in England, slow adoption and variable practice.
No full textAIM
National survey to evaluate the uptake of LISA (Less Invasive Surfactant Administration) in neonatal units across England.
METHODS
A web-based survey was sent out by email to all 150 neonatal units in England. It consisted of questions regarding indications for LISA, the practicalities of the procedure, and reasons for not using this technique.
RESULTS
The response rate was 96% (144/150 units). Only 11% of units are using LISA, but majority (78%) would consider implementing LISA on their unit. 56% would also consider LISA on delivery suite. Challenges identified are having a guideline and staff training. 61% of units have set the target population ≥27 weeks. On sub-analysis, for tertiary units, the trend for LISA is ≥26 weeks. The median FiO2 threshold for LISA is 0.3 (IQR 0.3-0.4) in less than 28 weekers, and 0.4 in higher gestations. The most common suggestion for premedication is fentanyl (32%).
CONCLUSION
The uptake of LISA in England is low comparing to the rest of Europe. Even though many units are considering implementing LISA, there is lack of training and national guidelines. There is urgent need for standardisation of practice and clear indications for LISA. This article is protected by copyright. All rights reserved
Defence Anaesthesia transition from the Tri-Service Anaesthetic Apparatus to the Diamedica Portable Anaesthesia Machine 02.
No full textDefence Anaesthesia is changing its draw-over anaesthetic capability from the Tri-Service Anaesthetic Apparatus (TSAA) to the Diamedica Portable Anaesthesia Machine 02 (DPA02). The DPA02 will provide a portable, robust, lightweight and simple method for delivering draw-over volatile anaesthesia with the option of positive pressure ventilation through manual or mechanical operation for paediatric and adult patients. The UK Defence Medical Services uses a modified configuration of the DPA02; this paper seeks to explain the rationale for the differing configurations and illustrates alternative assemblies to support integration with other Defence Anaesthesia equipment. High-fidelity simulation training using the DPA02 will continue to be delivered on the Defence Anaesthesia Simulation Course (DASC). Conformité Européenne accreditation of DPA02 supports future UK live patient training in centres of excellence supervised by subject matter experts; this was not possible with the TSAA. This article is intended to be a key reference for all members of the Defence Anaesthesia team. Alongside other resources, it will be given as precourse learning prior to attending the DASC and the Military Operational Surgical Training. This article will also be issued with all Defence DPA02 units, supporting ease of access for review during future clinical exercises (including validation), prior to supervised live training and on operational deployments
Evaluation of a Novel Field-placement Algorithm for Locoregional Breast Cancer Radiotherapy Including the Internal Mammary Chain.
No full textAIMS
Irradiation of the internal mammary chain (IMC) is increasing following recently published data, but the need for formal delineation of lymph node volumes is slowing implementation in some healthcare settings. A field-placement algorithm for irradiating locoregional lymph nodes including the IMC could reduce the resource impact of introducing irradiation of the IMC. This study describes the development and evaluation of such an algorithm.
MATERIALS AND METHODS
An algorithm was developed in which six points representing lymph node clinical target volume borders (based on European Society for Radiotherapy and Oncology consensus nodal contouring guidelines) were placed on computed tomography-defined anatomical landmarks and used to place tangential and nodal fields. Single-centre testing in 20 cases assessed the success of the algorithm in covering planning target volumes (PTVs) and adequately sparing organs at risk. Plans derived using the points algorithm were also compared with plans generated following formal delineation of nodal PTVs, using the Wilcoxon signed rank test. Timing data for point placement were collected. Multicentre testing using the same methods was then carried out to establish whether the technique was transferable to other centres.
RESULTS
Single-centre testing showed that 95% of cases met the nodal PTV coverage dose constraints (binomial probability confidence interval 75.1-99.9%) with no statistically significant reduction in mean heart dose or ipsilateral lung V associated with formal nodal delineation. In multicentre testing, 69% of cases met nodal PTV dose constraints and there was a statistically significant difference in IMC PTV coverage using the points algorithm when compared with formally delineated nodal volumes (P < 0.01). However, there was no difference in axillary level 1-4 PTV coverage (P = 0.11) with all cases meeting target volume constraints.
CONCLUSIONS
The optimal strategy for breast and locoregional lymph node radiotherapy is target volume delineation. However, use of this novel points-based field-placement algorithm results in dosimetrically acceptable plans without the need for formal lymph node contouring in a single-centre setting and for the breast and level 1-4 axilla in a multicentre setting. Further quality assurance measures are needed to enable implementation of the algorithm for irradiation of the IMC in a multicentre setting
Patient-reported Quality of Life Outcomes in Patients Treated for Muscle-invasive Bladder Cancer with Radiotherapy ± Chemotherapy in the BC2001 Phase III Randomised Controlled Trial.
No full textBACKGROUND
BC2001, the largest randomised trial of bladder-sparing treatment for muscle-invasive bladder cancer, demonstrated improvement of local control and bladder cancer-specific survival from the addition of concomitant 5-fluorouracil and mitomycin C to radiotherapy.
OBJECTIVE
To determine the impact of treatment on the health-related quality of life (HRQoL) of BC2001 participants.
DESIGN, SETTING, AND PARTICIPANTS
458 UK patients with T2-T4a N0 M0 transitional cell carcinoma of the bladder.
INTERVENTION
Patients were randomised to the chemotherapy comparison (radiotherapy, 178, or chemoradiotherapy, 182); and/or to the radiotherapy comparison (standard, 108, or reduced high-dose volume radiotherapy, 111).
OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS
Patients completed Functional Assessment of Cancer Therapy-Bladder (FACT-BL) questionnaires at baseline, end of treatment (EoT), and 6, 12, 24, 36, 48, and 60 months after radiotherapy. The primary endpoint was change from baseline in the bladder cancer subscale (BLCS) at 12 months.
RESULTS AND LIMITATIONS
Data were available for 331 (92%) and 204 (93%) participants at baseline and for 192 (54%) and 114 (52%) at 12 months for the chemotherapy and radiotherapy comparisons, respectively. HRQoL declined at EoT (BLCS -5.06 [99% confidence interval: -6.12 to -4.00, p< 0.001]; overall FACT-B TOTAL score -8.22 [-10.76 to -5.68, p< 0.01]), recovering to baseline at 6 months and remaining similar to baseline subsequently. There was no significant difference between randomised groups at any time point.
CONCLUSIONS
Immediately following (chemo)radiotherapy, a significant proportion of patients report declines in HRQoL, which improve to baseline after 6 months. Two-thirds of patients report stable or improved HRQoL on long-term follow-up. There is no evidence of impairment in HRQoL resulting from the addition of chemotherapy.
PATIENT SUMMARY
Quality of life of bladder cancer patients treated with radiotherapy±chemotherapy deteriorates during treatment, but improves to at least pretreatment levels within 6 months. Addition of chemotherapy to radiotherapy does not affect patient-reported quality of life
Diagnostic accuracy and added value of qualitative radiological review of H-magnetic resonance spectroscopy in evaluation of childhood brain tumors.
No full textBackground
H-magnetic resonance spectroscopy (MRS) facilitates noninvasive diagnosis of pediatric brain tumors by providing metabolite profiles. Prospective studies of diagnostic accuracy and comparisons with conventional MRI are lacking. We aimed to evaluate diagnostic accuracy of MRS for childhood brain tumors and determine added clinical value compared with conventional MRI.
Methods
Children presenting to a tertiary pediatric center with brain lesions from December 2015 through 2017 were included. MRI and single-voxel MRS were acquired on 52 tumors and sequentially interpreted by 3 radiologists, blinded to histopathology. Proportions of correct diagnoses and interrater agreement at each stage were compared. Cases were reviewed to determine added value of qualitative radiological review of MRS through increased certainty of correct diagnosis, reduced number of differentials, or diagnosis following spectroscopist evaluation. Final diagnosis was agreed by the tumor board at study end.
Results
Radiologists' principal MRI diagnosis was correct in 69%, increasing to 77% with MRS. MRI + MRS resulted in significantly more additional correct diagnoses than MRI alone ( = .035). There was a significant increase in interrater agreement when correct with MRS ( = .046). Added value following radiologist interpretation of MRS occurred in 73% of cases, increasing to 83% with additional spectroscopist review. First histopathological diagnosis was available a median of 9.5 days following imaging, with 25% of all patients managed without conclusive histopathology.
Conclusions
MRS can improve the accuracy of noninvasive diagnosis of pediatric brain tumors and add value in the diagnostic pathway. Incorporation into practice has the potential to facilitate early diagnosis, guide treatment planning, and improve patient care