Electronic reporting of rare endocrine conditions within a clinical network: results from the EuRRECa project.

Objective: The European Registries for Rare Endocrine Conditions (EuRRECa, eurreb.eu) includes an e-reporting registry (e-REC) used to perform surveillance of conditions within the European Reference Network (ERN) for rare endocrine conditions (Endo-ERN). The aim of this study was to report the experience of e-REC over the 3.5 years since its launch in 2018. Methods: Electronic reporting capturing new encounters of Endo-ERN conditions was performed monthly through a bespoke platform by clinicians registered to participate in e-REC from July 2018 to December 2021. Results: The number of centres reporting on e-REC increased to a total of 61 centres from 22 countries. A median of 29 (range 11, 45) paediatric and 32 (14, 51) adult centres had reported cases monthly. A total of 9715 and 4243 new cases were reported in adults (age ≥18 years) and children, respectively. In children, sex development conditions comprised 40% of all reported conditions and transgender cases were most frequently reported, comprising 58% of sex development conditions. The median number of sex development cases reported per centre per month was 0.6 (0, 38). Amongst adults, pituitary conditions comprised 44% of reported conditions and pituitary adenomas (69% of cases) were most commonly reported. The median number of pituitary cases reported per centre per month was 4 (0.4, 33). Conclusions: e-REC has gained increasing acceptability over the last 3.5 years for capturing brief information on new encounters of rare conditions and shows wide variations in the rate of presentation of these conditions to centres within a reference network. Significance statement Endocrinology includes a very wide range of rare conditions and their occurrence is often difficult to measure. By using an electronic platform that allowed monthly reporting of new clinical encounters of several rare endocrine conditions within a defined network that consisted of several reference centres in Europe, the EuRRECa project shows that a programme of e-surveillance is feasible and acceptable. The data that have been collected by the e-reporting of rare endocrine conditions (e-REC) can allow the continuous monitoring of rare conditions and may be used for clinical benchmarking, designing new studies or recruiting to clinical trials.

Predicting poor outcome in patients with suspected COVID-19 presenting to the Emergency Department (COVERED) - Development, internal and external validation of a prediction model.

BACKGROUND: A recent systematic review recommends against the use of any of the current COVID-19 prediction models in clinical practice. To enable clinicians to appropriately profile and treat suspected COVID-19 patients at the emergency department (ED), externally validated models that predict poor outcome are desperately needed. OBJECTIVE: Our aims were to identify predictors of poor outcome, defined as mortality or ICU admission within 30 days, in patients presenting to the ED with a clinical suspicion of COVID-19, and to develop and externally validate a prediction model for poor outcome. METHODS: In this prospective, multi-center study, we enrolled suspected COVID-19 patients presenting at the EDs of two hospitals in the Netherlands. We used backward logistic regression to develop a prediction model. We used the area under the curve (AUC), Brier score and pseudo-R2 to assess model performance. The model was externally validated in an Italian cohort. RESULTS: We included 1193 patients between March 12 and May 27 2020, of whom 196 (16.4%) had a poor outcome. We identified 10 predictors of poor outcome: current malignancy (OR 2.774; 95%CI 1.682-4.576), systolic blood pressure (OR 0.981; 95%CI 0.964-0.998), heart rate (OR 1.001; 95%CI 0.97-1.028), respiratory rate (OR 1.078; 95%CI 1.046-1.111), oxygen saturation (OR 0.899; 95%CI 0.850-0.952), body temperature (OR 0.505; 95%CI 0.359-0.710), serum urea (OR 1.404; 95%CI 1.198-1.645), C-reactive protein (OR 1.013; 95%CI 1.001-1.024), lactate dehydrogenase (OR 1.007; 95%CI 1.002-1.013) and SARS-CoV-2 PCR result (OR 2.456; 95%CI 1.526-3.953). The AUC was 0.86 (95%CI 0.83-0.89), with a Brier score of 0.32 and, and R2 of 0.41. The AUC in the external validation in 500 patients was 0.70 (95%CI 0.65-0.75). CONCLUSION: The COVERED risk score showed excellent discriminatory ability, also in an external validation. It may aid clinical decision making, and improve triage at the ED in health care environments with high patient throughputs.

Costs and quality of life in a randomized trial comparing minimally invasive and open distal pancreatectomy (LEOPARD trial).

BACKGROUND: Minimally invasive distal pancreatectomy decreases time to functional recovery compared with open distal pancreatectomy, but the cost-effectiveness and impact on disease-specific quality of life have yet to be established. METHODS: The LEOPARD trial randomized patients to minimally invasive (robot-assisted or laparoscopic) or open distal pancreatectomy in 14 Dutch centres between April 2015 and March 2017. Use of hospital healthcare resources, complications and disease-specific quality of life were recorded up to 1 year after surgery. Unit costs of hospital healthcare resources were determined, and cost-effectiveness and cost-utility analyses were performed. Primary outcomes were the costs per day earlier functional recovery and per quality-adjusted life-year. RESULTS: All 104 patients who had a distal pancreatectomy (48 minimally invasive and 56 open) in the trial were included in this study. Patients who underwent a robot-assisted procedure were excluded from the cost analysis. Total medical costs were comparable after laparoscopic and open distal pancreatectomy (mean difference €-427 (95 per cent bias-corrected and accelerated confidence interval €-4700 to 3613; P = 0·839). Laparoscopic distal pancreatectomy was shown to have a probability of at least 0·566 of being more cost-effective than the open approach at a willingness-to-pay threshold of €0 per day of earlier recovery, and a probability of 0·676 per additional quality-adjusted life-year at a willingness-to-pay threshold of €80 000. There were no significant differences in cosmetic satisfaction scores (median 9 (i.q.r. 5·75-10) versus 7 (4-8·75); P = 0·056) and disease-specific quality of life after minimally invasive (laparoscopic and robot-assisted procedures) versus open distal pancreatectomy. CONCLUSION: Laparoscopic distal pancreatectomy was at least as cost-effective as open distal pancreatectomy in terms of time to functional recovery and quality-adjusted life-years. Cosmesis and quality of life were similar in the two groups 1 year after surgery.

Systematic review of outcomes after distal pancreatectomy with coeliac axis resection for locally advanced pancreatic cancer.

BACKGROUND: Pancreatic cancer involving the coeliac axis is considered unresectable by most guidelines, with a median survival of 6-11 months. A subgroup of these patients can undergo distal pancreatectomy with coeliac axis resection, but consensus on the value of this procedure is lacking. The evidence for this procedure, including the impact of preoperative hepatic artery embolization and (neo)adjuvant therapy, was evaluated. METHODS: A systematic review was performed according to the PRISMA guidelines until 27 May 2015. The primary endpoint was overall survival; secondary endpoints included morbidity and radical resection rates. RESULTS: A total of 19 retrospective studies, involving 240 patients, were included. The methodological quality of the studies ranged from poor to moderate. A radical resection was reported in 74·5 per cent (152 of 204), major morbidity in 27 per cent (26 of 96), ischaemic morbidity in 9·0 per cent (21 of 223) and 90-day mortality in 3·5 per cent (4 of 113). Overall, 35·5 per cent of patients (55 of 155) underwent preoperative hepatic artery embolization without an apparent beneficial impact on ischaemic morbidity. Overall, 15·7 per cent (29 of 185) had neoadjuvant and 51·0 per cent (75 of 147) had adjuvant therapy. There was a difference in survival between patient series where less than half of patients had (neo)adjuvant chemotherapy and series where more than half were receiving this treatment: case-weighted median overall survival was 16 (range 9-48) versus 18 (10-26) months respectively (P = 0·002). Overall median survival for the whole study population was 14·4 (range 9-48) months. CONCLUSION: Distal pancreatectomy with coeliac axis resection seems a valuable option for selected patients with pancreatic cancer involving the coeliac axis with acceptable morbidity and mortality, and a median survival of 18 months when combined with (neo)adjuvant therapy.

Laparoscopic radical 'no-touch' left pancreatosplenectomy for pancreatic ductal adenocarcinoma: technique and results.

BACKGROUND: Laparoscopic left pancreatectomy has been well described for benign pancreatic lesions, but its role in pancreatic adenocarcinoma remains open to debate. We report our results adopting a laparoscopic technique that obeys established oncologic principles of open distal pancreatosplenectomy. METHODS: This is a post hoc analysis of a prospectively kept database of 135 consecutive patients undergoing laparoscopic left pancreatectomy, performed across two sites in the UK and the Netherlands (07/2007-07/2015 Southampton and 10/2013-07/2015 Amsterdam). Primary outcomes were resection margin and lymph node retrieval. Secondary endpoints were other perioperative outcomes, including post-operative pancreatic fistula. Definition of radical resection was distance tumour to resection margin >1 mm. All patients underwent 'laparoscopic radical left pancreatosplenectomy' (LRLP) which involves 'hanging' the pancreas including Gerota's fascia, followed by clockwise dissection, including formal lymphadenectomy. RESULTS: LRLP for pancreatic adenocarcinoma was performed in 25 patients. Seven of the 25 patients (28 %) had extended resections, including the adrenal gland (n = 3), duodenojejunal flexure (n = 2) or transverse mesocolon (n = 3). Mean age was 68 years (54-81). Conversion rate was 0 %, mean operative time 240 min and mean blood loss 340 ml. Median intensive/high care and hospital stay were 1 and 5 days, respectively. Clavien-Dindo score 3+ complication rate was 12 % and ISGPF grade B/C pancreatic fistula rate 28 %; 90-day (or in-hospital) mortality was 0 %. The pancreatic resection margin was clear in all patients, and the posterior margin was involved (<1 mm) in 6 patients, meaning an overall R0 resection rate of 76 %. No resection margin was microscopically involved. Median nodal sample was 15 nodes (3-26). With an average follow-up of 17.2 months, 1-year survival was 88 %. CONCLUSIONS: A standardised laparoscopic approach to pancreatic adenocarcinoma in the left pancreas can be adopted safely. Our study shows that these results can be reproduced across multiple sites using the same technique.

Technical Aspects of Laparoscopic Distal Pancreatectomy for Benign and Malignant Disease: Review of the Literature.

Distal pancreatectomy is the standard curative treatment for symptomatic benign, premalignant, and malignant disease of the pancreatic body and tail. The most obvious benefits of a laparoscopic approach to distal pancreatectomy include earlier recovery and shorter hospital stay. Spleen-preserving distal pancreatectomy should be attempted in case of benign disease. Spleen preservation can be achieved preferably by preserving the splenic vessels (Kimura technique), but also by resecting the splenic vessels and maintaining vascularity through the short gastric vessels and left gastroepiploic artery (Warshaw technique). Several studies have suggested a higher rate of spleen preservation with laparoscopy. The radical antegrade modular pancreatosplenectomy has become mainstay for treating pancreatic cancer and can be performed laparoscopically as well. Evidence on the feasibility and safety of laparoscopic distal pancreatectomy for cancer is scarce. Despite the obvious advantages of laparoscopic surgery, postoperative morbidity remains relatively high, mainly because of the high incidence of pancreatic fistula. For decades, surgeons have tried to prevent these fistulas but to date no strategy has been confirmed to be effective in 2 consecutive randomized studies. Pragmatic multicenter studies focusing on technical aspects of laparoscopic distal pancreatectomy are lacking and should be encouraged.

[Diagnostic policy in patients with a suspected ruptured abdominal aortic aneurysm]. / Diagnostisch beleid bij klinische aanwijzingen voor een geruptureerd aneurysma van de aorta abdominalis.

The clinical diagnosis of a ruptured aortic aneurysm is unreliable, and in haemodynamically stable patients both ultrasound and CT are used to improve diagnostic accuracy. In any patient with a suspected ruptured aneurysm, ultrasound is done upon arrival of the patient in the hospital to confirm or exclude the presence of an aortic aneurysm. If facilities for endovascular reconstruction are available at the hospital, an immediate CT scan with intravenous contrast follows, to find out if and how endovascular treatment should be performed. If endovascular reconstruction is not available, a CT scan without using contrast is carried out. If the CT scan shows a ruptured aneurysm, the patient is transported to the Operating Department without further delay. If the CT scan does not show any signs of rupture, there is time for further investigation. If no alternative condition to explain the patient's symptoms is found, further diagnostic tests such as repeat ultrasound, CT with contrast material and laboratory tests can be performed. If no other serious condition emerges, the aortic aneurysm should be considered symptomatic. In this case, the patient should undergo a rapid, but thorough pre-operative work-up, and go for operation as soon as possible by a rested and experienced operating team as part of the regular day programme.

[Enterovesical fistula in diverticulitis]. / Enterovesicale fistel bij diverticulitis.

Three men aged 52, 60, and 38 years, complained of dysuria and recurrent cystitis. One patient mentioned pneumaturia, a feature which later appeared to be present in all three. Ultrasound examination showed air in the bladder, and demonstrated the fistulous tract in two cases. Computed tomography identified the third fistula. In all 3 the enterovesical fistula was a complication of sigmoid diverticulitis. After sigmoid resection they recovered well. The key to the diagnosis of enterovesical fistula is to think of it. Pneumaturia and faecaluria are pathognomonic symptoms. Both ultrasound and computed tomography may be helpful in the diagnosis.

Application of AMBER in single- and dual-energy digital imaging: improvement in noise level and display dynamic range.

The combination of computed radiography (CR) and advanced multiple beam equalization radiography (AMBER) was evaluated for both single- and dual-energy chest radiography. The improved signal-to-noise ratio found with CR and AMBER resulted in a better visualization of structures in the mediastinum and basal lung than that found with CR alone. For the central lung, no improvement was seen. Because of the compressed dynamic range with CR and AMBER, contrast on hard copies and video monitors could be high without a sacrifice in image latitude. Dual-energy images showed a considerably lower noise level. The combined use of AMBER and CR promises to overcome the dynamic range limitations of digital displays while improving signal-to-noise ratio.