Preoperative localization of an insulinoma: selective arterial calcium stimulation test performance.

PURPOSE: Preoperative localization of an insulinoma is recommended to improve the cure rate, but non-invasive procedures can fail to detect the tumour. The objective of the study was to assess the performance of a selective arterial calcium stimulation test in the preoperative localization of insulinomas that were not detected by conventional imaging procedures. METHODS: We conducted a monocenter retrospective case review of 13 patients who had endogenous hyperinsulinism and were treated between 1994 and 2013. Patients were selected on the basis of negative or doubtful non-invasive preoperative imaging. A selective arterial calcium stimulation test was performed by pancreatic and hepatic arteriography with selective intra-arterial calcium stimulation and hepatic venous sampling in order to obtain the plasma insulin measurement. We evaluated the efficacy of the test by comparing the results with an endoscopic ultrasound. RESULTS: Twelve of the 13 patients underwent surgery, and the presence of an insulinoma was proven in 11 patients by pathological analysis of the tumour. An endoscopic ultrasound was consistent with surgery in 71.4 % of cases, while selective arterial calcium stimulation was consistent with surgery in 90.9 % and allowed detection of an insulinoma in two additional patients with a negative endoscopic ultrasound. One false-negative and one false-positive arterial calcium test were observed. No adverse events were recorded except transient skin flush following calcium injection in one patient. CONCLUSION: The selective arterial calcium stimulation test is a sensitive diagnostic procedure for localizing insulinomas and may be considered when non-invasive radiological imaging does not allow the detection of an occult insulinoma.

Comparison of functional outcomes with purely laparoscopic sacrocolpopexy and robot-assisted sacrocolpopexy in obese women.

OBJECTIVE: To compare the functional outcomes and complication rates following laparoscopic sacrocolpopexy (LS) with those occurring in robot-assisted laparoscopic sacrocolpopexy (RALSCP) in obese women. PATIENTS AND METHODS: A comparative retrospective multicentre study was made, involving 39 obese women (BMI≥30 kg/m2) who underwent LS, and 17 obese women who underwent RASCLP. The operative parameters (length of operation, associated procedures, complication rate and length of hospitalization) and the objective and subjective results were evaluated at 12 months follow-up. RESULTS: The median (IQR) BMI was 30.5 kg/m2 (30-32) in the LS group vs 31.6 kg/m2 (30-34) in the RALSCP group (P=0.402). The anatomical results were comparable in both groups (LS vs RALSCP): post-operative stage of prolapse (POP-Q-ICS): stage 0-1: 34/39 (88%) vs 16/17 (94.1%), P=0.7; stage 2: 4/39 (10%) vs 0/17 (0%), P=0.7; stage 3-4: 1/39 (2%) vs 1/17 (5.9%), P=0.7. The complication rate was similar in both groups (LS vs RALSCP): bladder injury 2.5% (1/39) vs 0% (0/17), P=0.6, laparoconversion 5.1% (2/39) vs 5.9% (1/17), P=0.5. The overall reoperation rate was (LS vs RALSCP): 18% (7/39) vs 5.9% (1/17), P=0.4. CONCLUSION: Laparoscopic sacrocolpopexy and robot-assisted laparoscopic sacrocolpopexy have equal results in obese women. The complication rates and outcomes appear to be similar in both groups of obese women. LEVEL OF EVIDENCE: 3.

Global transcriptomic analysis in avocado nursery trees reveals differential gene expression during asymptomatic infection by avocado sunblotch viroid (ASBVd).

Avocado sunblotch viroid (ASBVd) is the type species of the family Avsunviroidae and the causal agent of avocado sunblotch disease. The disease is characterised by the presence of chlorotic lesions on avocado fruit, leaves and/or stems. Infected trees may remain without chlorosis for extended periods of time, though distorted growth and reduced yield has been observed in these cases. The molecular effects of ASBVd on avocado, and members of the Avsunviroidae on their respective hosts in general, remain poorly understood. Host global transcriptomic studies within the family Pospiviroidae have identified several host pathways that are affected during these plant-pathogen interactions. In this study, we used RNA sequencing to investigate host gene expression in asymptomatic avocado nursery trees infected with ASBVd. Transcriptome data showed that 631 genes were differentially expressed, 63 % of which were upregulated during infection. Plant defence responses, phytohormone networks, gene expression pathways, secondary metabolism, cellular transport as well as protein modification and degradation were all significantly affected by ASBVd infection. This work represents the first global gene expression study of ASBVd-infected avocado, and the transcriptional reprogramming observed during this asymptomatic infection improves our understanding of the molecular interactions underlying broader avsunviroid-host interactions.

[Infliximab-induced hepatitis during treatment of vulvar Crohn's disease]. / Hépatite induite par l'infliximab lors du traitement d'une maladie de Crohn vulvaire.

BACKGROUND: We report a case of acute cytolytic hepatitis induced by infliximab in a patient with severe vulvar Crohn's disease. PATIENTS AND METHODS: A 29-year-old Congolese woman presented with severe vulvar Crohn's disease active for 7 years. In view of resistance to standard medication (corticosteroids and metronidazole), treatment with infliximab 5mg/kg per injection was initiated. The patient developed acute cytolytic hepatitis 10 days after the first injection and the disease was asymptomatic. The various investigations confirmed the direct cytotoxicity of infliximab. A favourable outcome was gradually achieved after increasing the dosage of corticosteroids. At the same time, an improvement in the vulvar lesions was noted after this sole injection. DISCUSSION: Infliximab-induced is rare, with only 20 reported cases. The physiopathological mechanism is unknown and a number of aetiologies have been suggested. CONCLUSION: This new case raises the issue of the need for routine liver function testing during infliximab therapy given the asymptomatic nature of this effect.

[Influence of body mass index on the outcomes of robotic-assisted laparoscopic sacrocolpopexy: a comparative retrospective study]. / Impact de l'indice de masse corporelle sur les résultats de la promontofixation robot-assistée : étude comparative rétrospective.

OBJECTIVE: The aim of our study was to assess the impact of body mass index (BMI) on a robot-assisted laparoscopic sacrocolpopexy (RALS) to treat genital prolapse. METHODOLOGY: The study focused on a group of 56 women who went through a robot-assisted laparoscopic sacrocolpopexy (RALS) between 2009 and 2013. Patients were divided into 3 groups according to their BMI (kg/m(2)): BMI<25 (n=28), 25≤BMI<30 (n=16), BMI≥30 (n=12). The operating parameters, results and short-term complications were analysed according to the patients' BMI. RESULTS: The median BMI was 22.5kg/m(2) in group 1, 26.1kg/m(2) in group 2, and 31.6kg/m(2) in group 3 (P<0.001). The operation time was respectively 250 minutes (130-380), 230 minutes (150-410) and 255 minutes (170-370), for groups 1, 2 and 3 (P=0.689). The 3 groups spent 4 days in the hospital (P=0.562). Only one laparotomy in group 3 was reported (P=0.214). The rate of early complications was similar in groups 1, 2 and 3 with 3/28, 0/16 and 0/12 respectively. Anatomical short-term results were identical in the 3 groups with a satisfactory anatomical correction in 100% of all cases (ICS POP-Q<2). CONCLUSION: In this small group, we observed that the BMI had no impact neither on the operation time nor on the rate of complication.

The mortality rate of patients with open abdomen and contributing factors - a three-year audit in a major academic trauma unit.

BACKGROUND: Damage control surgery (DCS) is a widely used approach in trauma. An open abdomen carries complications, increased morbidity and mortality. This study aims to quantify the mortality rate, determine contributory factors and factors influencing the decision to perform DCS and assess morbidity in patients undergoing open abdomen. METHODS: A retrospective review was conducted on 205 patients in Charlotte Maxeke Johannesburg Academic Hospital Trauma Unit. The mortality rate was evaluated over a 24-hour, 7-day and 28-day period. Data were collected by a data collection sheet from 1 January 2016 to 31 December 2018. RESULTS: Of the 205 patients, 193 were male and the median age was 34.34 years. Penetrating trauma was the most predominant mechanism of injury in 162 (79%), with gunshot injuries seen in the majority (130/162). The mortality rate was 55/205 (26.8%) for open abdomen patients, 19/55 (34.5%) within the first 24 hours, 22/55 (40%) in the 24-hours to 7-days period, and 14/55 (25.4%) in the 8-day to 28-day period. Statistically significant factors contributing to mortality were haemodynamic instability, hypothermia, coagulopathy, massive transfusion, vasopressors, and significant associated injuries. Morbidities were entero-atmospheric fistula (EAF) in 7.3% (Clavien-Dindo grade IIIa), surgical site infection in 45.3% (Clavien-Dindo grade I) and ventral hernia in 10.24% (Clavien-Dindo grade IIIb). CONCLUSION: Most open abdomens were performed in males, with gunshot injuries being the most common mechanism. The majority of mortalities were within the 24-hours to 7-days period. The most common morbidity associated with an open abdomen was surgical site infection.

NOTCH2 related disorders: Description and review of the fetal presentation.

Signs of skeletal dysplasias are relatively common in fetuses with abnormal ultrasound (US) findings. The diversity of congenital skeletal disorders, the possibility of late-onset severe phenotypes and overlapping syndromes can be a challenge in the way of diagnosis, even if prenatal high-throuput sequencing allows for a better diagnosis, prognosis and genetic counseling. Hajdu-Cheney spectrum pathologies are rarely described in prenatal, and the signs associated remain poorly known, and do not include specific postnatal signs as acro-osteolysis and premature osteoporosis. We hereby report a couple for whom a medical termination of pregnancy was performed because a severe polymalformative syndrome associating severely short limbs with bowed long bones, severe cardiopathy, hyperechogenic kidneys and dysmorphism. After fetopathological and radiological examinations, Exome Sequencing (ES) was performed and revealed a de novo truncating mutation in the last exon of NOTCH2, responsible for Hajdu-Cheney or Serpentine Fibula Polycystic Kidney syndromes.

Behçet's disease uveitis.

Uveitis in Behçet's disease (BD) is frequent (40% of cases) and is a major cause of morbidity. The age of onset of uveitis is between 20 and 30 years. Ocular involvement includes anterior, posterior or panuveitis. It is non-granulomatous. Uveitis may be the first sign of the disease in 20% of cases or it may appear 2 or 3 years after the first symptoms. Panuveitis is the most common presentation and is more commonly found in men. Bilateralisation usually occurs on average 2 years after the first symptoms. The estimated risk of blindness at 5 years is 10-15%. BD uveitis has several ophthalmological features that distinguish it from other uveitis. The main goals in the management of patients are the rapid resolution of intraocular inflammation, prevention of recurrent attacks, achievement of complete remission, and preservation of vision. Biologic therapies have changed the management of intraocular inflammation. The aim of this review is to provide an update previous article by our team on pathogenesis, diagnostic approaches, identification of factors associated with relapse and the therapeutic strategy of BD uveitis.

[Ethmoidal metastasis revealing lung adenocarcinoma]. / Métastase ethmoïdale révélant un adénocarcinome pulmonaire.

OBJECTIVES: Ethmoidal cancers are rare and are mostly primitive adenocarcinomas. Sinuso-nasal metastases from adenocarcinoma occurring in other localisations are exceptional. We report a case of a patient presenting with an ethmoidal metastasis revealing a pulmonary adenocarcinoma. CASE REPORT: A 57 years-old patient consulted for a right fronto-orbital pain which had appeared 3 weeks prior, associated with a central scotoma of the right eye and a third and fifth cranial nerve palsy. MR-imaging revealed a right ethmoidal tumour invading the orbit. Pathology results were in favour of a metastasis from a secondary adenocarcinoma. The CT-scan disclosed a pulmonary tumour of the right apex with liver and spleen metastasis. CONCLUSION: Ethmoidal metastases are rare and often diagnosed in patients presenting with a previously known tumour. We report the first case of a pulmonary adenocarcinoma revealed by an ethmoidal metastasis. We emphasize the point that in ethmoidal tumours, pathological investigations are important for diagnosis.

[Parapharyngeal tumors: management and postoperative results]. / Tumeurs de l'espace parapharyngé: prise en charge et résultats postopératoires.

OBJECTIVES: Parapharyngeal space tumours are rare and mostly benign, and their access is difficult. The aim of the study was to define the optimal treatment of these tumours. MATERIAL AND METHODS: Clinical retrospective study, with histological, treatment, prognosis analysis of the parapharyngeal space tumours occurred between 1994 and 2012 and the surgical approach considerations. RESULTS: 35 patients were included with a male sex ratio predominance (51.43%, p = ns). The mean age of diagnosis was 49 years. The majority of tumours were benign (75.76%, p < 0.001). 54.5% originated from salivary gland with a majority of pleomorphic adenomas (42.42%). The second most frequent aetiology found was schwannoma (21.21%). 2 patients refused the treatment. Accidentally discovered were frequent (28.57%). In the symptomatic forms, dysphagia dominated (25.71%). Oropharyngeal (20%) and/or cervical (17.14%) mass were rare. A CT scan and a MR imaging were performed in 82.86% and 71.43% of the cases. A fine needle aspiration cytology was achieved in 10 cases with a positive predictive value of 60%. The cervical submandibular trans digastric approach was the most accomplished (40%). Other approaches were also used: Trans oral (25.71%), parotidectomy (14.71%), combined trans oral and cervical or parotidectomy (14.71%) and the cervical approached associated to a mandibulotomy (5.71%). A complementary treatment was proposed in 85.7% of the cases of malignant tumours. Post-operative complications were dominated by Horner's syndrome (n = 3), a temporary facial palsy (n = 5), definitive (n = 1), a velar insufficiency (n = 1), persistent pain (n = 6). CONCLUSIONS: The presence of 25% of malignant tumours and 40% of pleomorphic adenomas require removal of parapharyngeal tumours, even if non symptomatic.

Design of a prospective, longitudinal cohort of people living with type 1 diabetes exploring factors associated with the residual cardiovascular risk and other diabetes-related complications: The SFDT1 study.

Type 1 diabetes mellitus (T1DM) is associated with a high risk of cardiovascular (CV) complications, even after controlling for traditional CV risk factors. Therefore, determinants of the residual increased CV morbidity and mortality remain to be discovered. This prospective cohort of people living with T1DM in France (SFDT1) will include adults and children aged over six years living with T1DM, recruited throughout metropolitan France and overseas French departments and territories. The primary objective is to better understand the parameters associated with CV complications in T1DM. Clinical data and biobank samples will be collected during routine visits every three years. Data from connected tools, including continuous glucose monitoring, will be available during the 10-year active follow-up. Patient-reported outcomes, psychological and socioeconomic information will also be collected either at visits or through web questionnaires accessible via the internet. Additionally, access to the national health data system (Health Data Hub) will provide information on healthcare and a passive 20-year medico-administrative follow-up. Using Health Data Hub, SFDT1 participants will be compared to non-diabetic individuals matched on age, gender, and residency area. The cohort is sponsored by the French-speaking Foundation for Diabetes Research (FFRD) and aims to include 15,000 participants.

Early continuous glucose monitoring for predicting remission of type 2 diabetes 1 year after bariatric surgery.

BACKGROUND: Bariatric surgery in obese subjects can result in remission of type 2 diabetes (T2D) at a distant time post-surgery. The aim of our observational prospective single-centre study was to examine glycaemic patterns in adult T2D candidates for bariatric surgery using a continuous glucose monitoring (CGM) sensor for 14 days after surgery to search for indicators predictive of T2D remission 1 year later. METHODS: Patients underwent CGM preoperatively and for 14 days postoperatively. Thereafter, body weight and glycated haemoglobin (HbA1c) levels were monitored at 3, 6 and 12 months after surgery. RESULTS: A total of 31 patients (mean age 47±2 years) were analyzed. After surgery, mean interstitial glucose levels fell rapidly from 157±31mg/dL preoperatively to 109±35mg/dL postoperatively (P<0.001), reaching nadir levels from day 3 after surgery. Successful bariatric surgery (loss of excess weight ≥50%) was observed in 28 (90%) patients, and diabetes remission (HbA1c≤6% with no antidiabetic treatment) 1 year after surgery was noted in 21 (68%) patients. CGM for 14 days post-surgery allowed prediction of diabetes remission 1 year after surgery: time spent above range <14% and standard deviation (SD) of glucose levels <33mg/dL were both strong predictors of T2D remission. Indeed, the association of these two criteria predicted diabetes remission with a 100% positive predictive value, 81% sensitivity and 100% specificity and, when combined with the advanced Diabetes Remission (Ad-DiaRem) score, further increased predictive accuracy. CONCLUSION: The use of 14-day postoperative CGM recordings together with presurgical clinical scores can help to predict diabetes remission 1 year after bariatric surgery.

Practical implementation of automated closed-loop insulin delivery: A French position statement.

Automated closed-loop (CL) insulin therapy has come of age. This major technological advance is expected to significantly improve the quality of care for adults, adolescents and children with type 1 diabetes. To improve access to this innovation for both patients and healthcare professionals (HCPs), and to promote adherence to its requirements in terms of safety, regulations, ethics and practice, the French Diabetes Society (SFD) brought together a French Working Group of experts to discuss the current practical consensus. The result is the present statement describing the indications for CL therapy with emphasis on the idea that treatment expectations must be clearly defined in advance. Specifications for expert care centres in charge of initiating the treatment were also proposed. Great importance was also attached to the crucial place of high-quality training for patients and healthcare professionals. Long-term follow-up should collect not only metabolic and clinical results, but also indicators related to psychosocial and human factors. Overall, this national consensus statement aims to promote the introduction of marketed CL devices into standard clinical practice.

Genotype-phenotype correlations in fetuses and neonates with autosomal recessive polycystic kidney disease.

The prognosis of autosomal recessive polycystic kidney disease is known to correlate with genotype. The presence of two truncating mutations in the PKHD1 gene encoding the fibrocystin protein is associated with neonatal death while patients who survive have at least one missense mutation. To determine relationships between genotype and renal and hepatic abnormalities we correlated the severity of renal and hepatic histological lesions to the type of PKHD1 mutations in 54 fetuses (medical pregnancy termination) and 20 neonates who died shortly after birth. Within this cohort, 55.5% of the mutations truncated fibrocystin. The severity of cortical collecting duct dilatations, cortical tubule and glomerular lesions, and renal cortical and hepatic portal fibrosis increased with gestational age. Severe genotypes, defined by two truncating mutations, were more frequent in patients of less than 30 weeks gestation compared to older fetuses and neonates. When adjusted to gestational age, the extension of collecting duct dilatation into the cortex and cortical tubule lesions, but not portal fibrosis, was more prevalent in patients with severe than in those with a non-severe genotype. Our results show the presence of two truncating mutations of the PKHD1 gene is associated with the most severe renal forms of prenatally detected autosomal recessive polycystic kidney disease. Their absence, however, does not guarantee survival to the neonatal period.

[Update on the epidemiology of malaria in French Guiana]. / Situation epidémiologique du paludisme en Guyane.

The last study describing the epidemiology of malaria in French Guiana was published 20 years ago. Yet French Guiana in the Americas along with Mayotte in the Indian Ocean is the only French territory with persisting endemic malaria. The purpose of this study is to provide an update based on official malaria surveillance data as well as on information from hospital records and various field surveys that have been carried out in recent years. Due to recurrent problems in collecting thorough, continuous, and consistent data, exact determination of incidence by plasmodium species has always been difficult in French Guiana. These problems include not only the remote location of endemic areas and intense unpredictable migration patterns but also poor data collection methods that do not always ensure consistency and homogeneity. Another factor hindering thorough collection of conventional epidemiological data is the requirement for rapid effective treatment in remote regions. The overall incidence of malaria in French Guiana appears to have remained stable since the beginning of the decade with an average of 3,920 case reports per year for an incidence rate of 20 per thousand, noting that three fourths of the 206,000 inhabitants of French Guiana live outside of endemic areas. Overall involvement of P. falciparum and P. vivax appears to be equal with P. malariae accounting for only 2.6% of cases. Trends in recent years indicate an increase in the number of cases involving P. vivax especially in the eastern zones, i.e. in the Oyapock focus where annual incidences in children have reached up to 500 per thousand and in the whole region located between Saint Georges and Cayenne. Conversely a decrease in endemic levels has been observed in western areas, especially for P. falciparum in the upper and middle focuses of the Maroni. Most zones now causing problems are located near migration points, particularly in relation with clandestine gold panning activities. In the coastal strip where the three main cities with most of the population are located, most reported cases are imported but local cases may occur. In general local transmission in these areas has been promptly controlled but trends indicate that incidence of these events may be rising. Anopheles darlingi is still recognized as the main vector but its role in transmission is less obvious in eastern areas where increasing evidence suggests that other species may contribute to maintaining endemic levels. These findings indicate that the extensive resources deployed in this French territory (public financing, health care network, public awareness campaigns, and training of health care personnel in diagnosis and treatment of malaria) have helped reduce the number of severe cases in an unfavorable epidemiological setting.

Diabetes-related cardiomyopathy: The sweet story of glucose overload from epidemiology to cellular pathways.

Type 2 diabetes (T2D) is a major risk factor for heart failure (HF). Although the number of cases of myocardial infarction in the T2D population has been reduced by 25% over the last 10 years, the incidence of HF is continuously increasing, making it the most worrying diabetes complication. This strongly reinforces the urgent need for innovative therapeutic interventions to prevent cardiac dysfunction in T2D patients. To this end, epidemiological, imaging and animal studies have aimed to highlight the mechanisms involved in the development of diabetic cardiomyopathy. Epidemiological observations clearly show that hyperglycaemia correlates with severity of cardiac dysfunction and mortality in T2D patients. Both animal and cellular studies have demonstrated that, in the context of diabetes, the heart loses its ability to utilize glucose, therefore leading to glucose overload in cardiomyocytes that, in turn, promotes oxidative stress, accumulation of advanced glycation end-products (AGEs) and chronic activation of the hexosamine pathway. These have all been found to activate apoptosis and to alter heart contractility, calcium signalling and mitochondrial function. Although, in the past, tight glycaemic control has failed to improve cardiac function in T2D patients, recent clinical trials have reported cardiovascular benefit with hypoglycaemic antidiabetic drugs of the SGLT2-inhibitor family. This review, based on clinical evidence from mechanistic studies as well as several large clinical trials, covers 15 years of research, and strongly supports the idea that hyperglycaemia and glucose overload play a central role in the pathophysiology of diabetic cardiomyopathy.

[Is the bronchial atresia prenatal diagnosis possible?]. / Le diagnostic anténatal d'atrésie bronchique est-il possible ?

Bronchial atresia is a rare congenital malformation of the lung. The main-stem segmental or lobar bronchus fails to construct normally, which can lead to accumulation of mucus within the distal bronchi or lung hyperinflation of the obstructed lobe. The prenatal diagnosis is rare and difficult. We report two cases of fetuses who presented pathological examination of the lung on the ultrasonography, at 22 weeks of gestation, suspect of prenatal bronchial atresia diagnosis. We analysed this malformation through a literature review in order to discuss differential diagnosis to be evoked, as well as appropriate perinatal management.