Transthyretin amyloid polyneuropathy in France: A cross-sectional study with 413 patients and real-world tafamidis meglumine use (2009-2019).

OBJECTIVE: We aimed to describe characteristics of patients with ATTR variant polyneuropathy (ATTRv-PN) and ATTRv-mixed and assess the real-world use and safety profile of tafamidis meglumine 20mg. METHODS: Thirty-eight French hospitals were invited. Patient files were reviewed to identify clinical manifestations, diagnostic methods, and treatment compliance. RESULTS: Four hundred and thirteen patients (296 ATTRv-PN, 117 ATTRv-mixed) were analyzed. Patients were predominantly male (68.0%) with a mean age of 57.2±17.2 years. Interval between first symptom(s) and diagnosis was 3.4±4.3 years. First symptoms included sensory complaints (85.9%), dysautonomia (38.5%), motor deficits (26.4%), carpal tunnel syndrome (31.5%), shortness of breath (13.3%), and unexplained weight loss (16.0%). Mini-invasive accessory salivary gland or punch skin and nerve biopsies were most common, with a performance of 78.8-100%. TTR genetic sequencing, performed in all patients, revealed 31 TTR variants. Tafamidis meglumine was initiated in 156/214 (72.9%) ATTRv-PN patients at an early disease stage. Median treatment duration was 6.00 years in ATTRv-PN and 3.42 years in ATTRv-mixed patients. Tafamidis was well tolerated, with 20 adverse events likely related to study drug among the 336 patients. CONCLUSION: In France, ATTRv patients are usually identified early thanks to the national network and the help of diagnosis combining genetic testing and mini-invasive biopsies.

Antimicrobial prophylaxis protocol based on rectal swab culture before prostate biopsy to prevent infectious complications: a prospective randomized comparative study.

PURPOSE: To evaluate the benefit of targeted antibiotic prophylaxis (TAP) based on rectal swab culture in comparison with standard empiric antimicrobial prophylaxis in patients undergoing transrectal ultrasound-guided needle biopsy of the prostate (TRUS-BP), as well as to assess rate of fecal carriage of Fluoroquinolone-resistant Enterobacterales FQRE. PATIENTS AND METHODS: We prospectively analyzed data that randomized 157 patients within two groups: (G1) TAP according to rectal swab performed 10 days before PB; (G2): empirical antibiotic prophylaxis with ciprofloxacin. Prevalence of FQRE digestive carriage and risk factors were investigated. Incidence of infectious complications after (TRUS-BP) in each group was compared. RESULTS: G2 included 80 patients versus 77 in G1. There was no difference between the two groups regarding age, diabetes, prostate volume, PSA, number of biopsy cores, and risk factors for FQRE. In G2, the prevalence of FQRE digestive carriage was 56.3% all related to E. coli species. In the case of digestive carriage of FQRE, TAP according to the rectal swab culture with third-generation cephalosporins was performed in 73.3%. Patients with FQRE had history of FQ use within the last 6 months in 17.8% (p = 0.03). Rate of febrile urinary tract infection after PB was 13% in G1 and 3.8% in G2 (p = 0.02). CONCLUSIONS: Incidence of FQ resistance in the intestinal flora of our local population was prevalent. Risk factor for resistance was the use of FQ within the last 6 months. TAP adapted to rectal swab, mainly with third-generation cephalosporins, significantly reduced the rate of infectious complications after (TRUS-BP).

Non-thermal plasma disinfecting procedure is harmless to delicate items of everyday use.

Non-thermal plasma (NTP) is a well-known decontamination tool applicable for a wide range of microorganisms and viruses. Since the recent COVID-19 pandemic highlighted the need to decontaminate all daily used items, it is highly desirable to address the applicability of NTP, including its possible harmful effects. To the best of our knowledge, a comprehensive characterization of NTP effects on sensitive materials is still lacking. We investigated the potential damage to common materials of daily use inflicted by air atmospheric NTP generated in Plasmatico v1.0. The materials tested were paper, various metals, and passive and active electronic components modelling sensitive parts of commonly used small electronic devices. The NTP-exposed paper remained fully usable with only slight changes in its properties, such as whitening, pH change, and degree of polymerization. NTP caused mild oxidation of copper, tinned copper, brass, and a very mild oxidation of stainless steel. However, these changes do not affect the normal functionality of these materials. No significant changes were observed for passive electronic components; active components displayed a very slight shift of the measured values observed for the humidity sensor. In conclusion, NTP can be considered a gentle tool suitable for decontamination of various sensitive materials.

Squamous and glandular metaplasia related to a renal pelvis stone leading to secondary pyeloureteral junction syndrome.

Keratinizing squamous metaplasia of the renal pelvis is a rare lesion of the upper urinary tract that can occur in the context of chronic aggression to the urothelium, potentially leading to a secondary pyeloureteral junction syndrome. We report the case of a 43-year-old patient discovered intraoperatively in relation to a renal pelvis stone causing a pyeloureteral junction syndrome. The extemporaneous histological examination ruled out a malignant process, and we performed a pyeloplasty according to KUSS-ANDERSON technique. This pathology should be recognized by the urologist for appropriate management. Treatment is conservative, with extended follow-up to detect recurrences or carcinomatous degenerations.

Prostate adenocarcinoma metastasis to the testis: A new case report.

Prostate cancer (PCa) in the second most common cancer in men worldwide. It commonly metastasizes to the bone, lymph nodes, liver and lungs. Synchronous or metachronous testicular metastasis is a rare finding, generally diagnosed incidentally after bilateral orchidectomy for hormonal management in patients with advanced PCa, or at autopsy. We report a case of a 55-year-old male, presenting a PCa and who developed a single testicular metastasis treated by radical orchidectomy, while he was under hormonotherapy.

Paratesticular leiomyoma: A case report of a rare entity.

Paratesticular leiomyoma is an extremely rare benign tumour. It is often asymptomatic. It is sometimes difficult to distinguish leiomyomas from malignant testicular tumours, which leads to radical orchidectomy, despite its benign nature. Magnetic resonance imaging can be helpful to make conservative management of this lesion.

3D deep convolutional neural network segmentation model for precipitate and porosity identification in synchrotron X-ray tomograms.

New developments at synchrotron beamlines and the ongoing upgrades of synchrotron facilities allow the possibility to study complex structures with a much better spatial and temporal resolution than ever before. However, the downside is that the data collected are also significantly larger (more than several terabytes) than ever before, and post-processing and analyzing these data is very challenging to perform manually. This issue can be solved by employing automated methods such as machine learning, which show significantly improved performance in data processing and image segmentation than manual methods. In this work, a 3D U-net deep convolutional neural network (DCNN) model with four layers and base-8 characteristic features has been developed to segment precipitates and porosities in synchrotron transmission X-ray micrograms. Transmission X-ray microscopy experiments were conducted on micropillars prepared from additively manufactured 316L steel to evaluate precipitate information. After training the 3D U-net DCNN model, it was used on unseen data and the prediction was compared with manual segmentation. A good agreement was found between both segmentations. An ablation study was performed and revealed that the proposed model showed better statistics than other models with lower numbers of layers and/or characteristic features. The proposed model is able to segment several hundreds of gigabytes of data in a few minutes and could be applied to other materials and tomography techniques. The code and the fitted weights are made available with this paper for any interested researcher to use for their needs (https://github.com/manasvupadhyay/erc-gamma-3D-DCNN).

Vesico-vaginal fistula in Tunisia: Epidemiology and risk factors of treatment failure.

INTRODUCTION: Vesico-vaginal fistula (VVF) is a global healthcare problem that has a high prevalence in developing countries. The aim of this work is to study the epidemiological, clinical and therapeutic characteristics of VVF post-obstetric and gynecologic procedures in order to identify the predictive factors of surgical treatment failure. METHODS: Data were collected from 132 VVF patients in our institution between 1985 and 2017. VVF was classified according to Zmerli's classification. Patients underwent surgical treatment and were evaluated after a follow-up period of 6 months. Successful treatment was defined as the absence of urine leakage whereas recurrence was defined as the presence of urine leakage immediately after the surgery or after a period of dryness with a confirmed VVF. Risk factors of surgical treatment failure were identified. RESULTS: The average age of patients was 44 years. The patients were multiparous in 62% of cases. VVF was consecutive to hysterectomy in 62.1% of cases, and after childbirth in 34%. VVF was retro-trigonal in 99 cases (75%) and trigonal in 33 cases (25%). The average size of the fistula was 1cm (0.3-2cm). VVF repair was performed by vaginal approach in 68% of cases and abdominal approach in 32% of cases. Treatment failure was noted in 36 patients (27%). Predictive factors of treatment failure were: vaginal fibrosis (P<0.001); trigonal location of the fistula (P<0.001); large diameter of the VVF>1cm (P<0.001); and complex and complicated fistulas (P=0.02). CONCLUSION: Although Tunisia is a developing country, the main cause of VVF was not obstetrical. Treatment failure, noted in almost one third of cases, was, in our series, correlated with the quality of the vaginal tissue, the size and the location of the fistula, and its complexity. LEVEL OF PROOF: 4.

A cadaveric anatomical study of the adrenals: Relationship with the posterior abdominal wall muscles revisited.

AIM OF THE STUDY: The main difficulties during retroperitoneal laparoscopic adrenalectomies are due to its location. Our objective was to define the relationship of the adrenals with the diaphragm and the psoas muscle. METHODS: Our work is an anatomical dissection of 80 fresh cadavers' adrenals. To study the right adrenal, we performed a right nephrectomy and adrenal remained attached to the Inferior vena cava by its main vein. On the left, the edges of the adrenal have been identified by needles and the adrenal was reclined to study its projection on the posterior muscular wall. RESULTS: The right adrenal is located higher, 13mm [4-20mm] above the medial arcuate ligament (MAL) in 16 cases (40%). Its lower border was at the same level as the MAL in 18 cases (45%) and 11mm [10-17mm] below the MAL in 6 cases (15%). The posterior support of the right adrenal was the right crus of the diaphragm (Right-CD) in 34 cases (85%) and straddling the Right-CD and the psoas in 6 cases (15%). The study of the relationships of the left adrenal with the MAL showed that the lower edge of the gland was at its same level in 16 cases (40%) and below in 24 cases (60%) by 14mm [8-24mm]. The posterior support of the left adrenal was the left crus of the diaphragm (Left-CD) in 16 cases (40%) and straddling the Left-CD and the psoas in 24 cases (60%). CONCLUSIONS: Our results showed that the right adrenal is higher. The MAL is an important posterior element to the adrenal gland that could serve as an anatomical landmark to identify the adrenal during laparoscopic adrenalectomy.

Systematic review and literature appraisal on methodology of conducting and reporting critical-care echocardiography studies: a report from the European Society of Intensive Care Medicine PRICES expert panel.

BACKGROUND: The echocardiography working group of the European Society of Intensive Care Medicine recognized the need to provide structured guidance for future CCE research methodology and reporting based on a systematic appraisal of the current literature. Here is reported this systematic appraisal. METHODS: We conducted a systematic review, registered on the Prospero database. A total of 43 items of common interest to all echocardiography studies were initially listed by the experts, and other "topic-specific" items were separated into five main categories of interest (left ventricular systolic function, LVSF n = 15, right ventricular function, RVF n = 18, left ventricular diastolic function, LVDF n = 15, fluid management, FM n = 7, and advanced echocardiography techniques, AET n = 17). We evaluated the percentage of items reported per study and the fraction of studies reporting a single item. RESULTS: From January 2000 till December 2017 a total of 209 articles were included after systematic search and screening, 97 for LVSF, 48 for RVF, 51 for LVDF, 36 for FM and 24 for AET. Shock and ARDS were relatively common among LVSF articles (both around 15%) while ARDS comprised 25% of RVF articles. Transthoracic echocardiography was the main echocardiography mode, in 87% of the articles for AET topic, followed by 81% for FM, 78% for LVDF, 70% for LVSF and 63% for RVF. The percentage of items per study as well as the fraction of study reporting an item was low or very low, except for FM. As an illustration, the left ventricular size was only reported by 56% of studies in the LVSF topic, and half studies assessing RVF reported data on pulmonary artery systolic pressure. CONCLUSION: This analysis confirmed sub-optimal reporting of several items listed by an expert panel. The analysis will help the experts in the development of guidelines for CCE study design and reporting.

Ultrasound-guided vascular access in critical illness.

Over the past two decades, ultrasound (US) has become widely accepted to guide safe and accurate insertion of vascular devices in critically ill patients. We emphasize central venous catheter insertion, given its broad application in critically ill patients, but also review the use of US for accessing peripheral veins, arteries, the medullary canal, and vessels for institution of extracorporeal life support. To ensure procedural safety and high cannulation success rates we recommend using a systematic protocolized approach for US-guided vascular access in elective clinical situations. A standardized approach minimizes variability in clinical practice, provides a framework for education and training, facilitates implementation, and enables quality analysis. This review will address the state of US-guided vascular access, including current practice and future directions.

Upper limb onset of hereditary transthyretin amyloidosis is common in non-endemic areas.

BACKGROUND AND PURPOSE: The aim is to describe an uncommon phenotype of hereditary ATTR neuropathy with upper limb onset. METHODS: The French TTR Familial Amyloid Polyneuropathy database was used for a retrospective evaluation of 32 consecutive patients with upper limb onset of the neuropathy (study group) and they were compared to 31 Portuguese early-onset patients and 99 late-onset patients without upper limb onset. RESULTS: Initial upper limb symptoms were mostly sensory. Lower limb symptoms began 2.3 ± 3 years after upper limb symptoms. Twenty-four (75%) patients were initially misdiagnosed, with 15 different diagnoses. More patients in the study group had a Neuropathy Impairment Score upper limb/lower limb ratio > 1 compared to the late-onset patient group. The study group had significantly more pronounced axonal loss in the median and ulnar motor nerves and the ulnar sensory and sural nerves. On radial nerve biopsies (n = 11), epineurial vessels were abnormal in six cases, including amyloid deposits in vessel walls (3/11), with vessel occlusion in two cases. CONCLUSION: Upper limb onset of hereditary ATTR neuropathy is not rare in non-endemic areas. It is important to propose early TTR sequencing of patients with idiopathic upper limb neuropathies, as specific management and treatment are required.

[ST-elevation myocardial infarction (STEMI) in the elderly]. / Syndrome coronaire aigu avec sus-décalage du segment ST (SCA ST+) chez le sujet âgé.

Admission in cardiology departments of patients over 80 years old, even nonagenarians, for ST-segment elevation myocardial infarction (STEMI) is not uncommon in 2018. The management of these high risk and polypathological patients, with atypical clinical presentation, is not based on international guidelines or randomized studies, but rather on retrospective studies, expert consensus, and common sense. Each decision has to be individualized to the patient's situation. This review, after a clinical case, aims to guide the clinician in the specific management of these patients, from the symptoms, to reperfusion strategy, and, as fast as possible, hospital discharge.

Retro-peritoneal paraganglioma, diagnosis and management.

INTRODUCTION: Paragangliomas, defined as extra-adrenal chromaffin-cells tumors, are rarely located in the retro-peritoneum. Clinical presentation is similar to pheochromocytoma, and mainly depends on the producing character of the tumor. Positive diagnosis requires plasmatic and urinary hormonal assays. Radiological and isotopic explorations are essential before surgery. The only curative therapeutic strategy is surgical, associated to peri-operative prevention and monitoring of the frequently reported hemodynamic and cardiovascular disorders. Outcome depends of the metastatic character of the tumor, the presence of tumor remnant after surgical resection. Genetic study is recommended; the risk of recurrence and association to other neoplasm is more described in genetic forms. MATERIAL AND METHODS: Authors report 5cases of retro-peritoneal paraganglioma, operated in the department of urology of Hospital, between 2013 and 2017. Observations are about 2men and 3women. Clinical presentation is not always specific and paraganglioma may be discovered fortuitously. Two patients have been operated by coelioscopic approach, midline incision was performed in two other cases, and dorsal lumbotomy associated to a Rutherford-Morrison incision in a patient. RESULTS: Two patients presented resistant hypertension and palpitation associated to suspect retro-peritoneal masses in imagery and elevated urinary methoxylated derivates before surgery. One patient was asymptomatic and the tumor was discovered in imagery. Per-operative hypertensive crisis and sinus tachycardia occurred in a case. The average follow-up period is 22.8months. Hypertension and palpitation disappeared after surgery. There was no recurrence for all the operated patients. CONCLUSION: Retro-peritoneal paraganglioma is a rare condition. Symptoms are not specific and clinical presentation may be similar to pheochromocytoma. Abdominal CT-scan and MRI, in association with MIBG scintigraphy are strongly evocative. Histological examination ensures diagnosis. Per-operative cardio-vascular disorders are to consider and must prevented and managed by anesthesiologists. Complete surgical resection is the only curative treatment and avoids recurrences.

Splenogonadal fusion - a rare cause of scrotal swelling: a case report.

BACKGROUND: Splenogonadal fusion is a rare and benign condition. Diagnosis is challenging for clinicians. Despite its indolence, diagnosis is often confirmed after orchidectomy. Surgery is mandatory, particularly to rule out the extremely rare association with malignancy. CASE PRESENTATION: We report a case of splenogonadal fusion in a 38-year-old North African man presenting a palpable scrotal mass. We describe clinical aspects, pathogenic hypothesis, radiological features, as well as surgical management principles. CONCLUSIONS: Splenogonadal fusion is rarely suspected and diagnosed preoperatively. A diagnosis is made once an ectopic testicular mass is associated with cryptorchidism and suggestive radiological signs. A better knowledge of the clinical and radiological features of splenogonadal fusion provides an opportunity for conservative surgery.

The ICM research agenda on critical care ultrasonography.

PURPOSE: Critical care ultrasonography has utility for the diagnosis and management of critical illness and is in widespread use by frontline intensivists. As there is a need for research to validate and extend its utility, the Editor of Intensive Care Medicine included critical care ultrasonography as a topic in the ICM Research Agenda issue. METHODS: Eleven international experts in the field of critical care ultrasonography contributed to the writing project. With the intention of developing a research agenda for the field, they reviewed best standards of care, new advances in the field, common beliefs that have been contradicted by recent trials, and unanswered questions related to critical care ultrasonography. RESULTS: The writing group focused on the provision of training in critical care ultrasonography, technological advances, and some specific clinical applications. CONCLUSIONS: The writing group identified several fields of interest for research and proposed ten research studies that would address important aspects of critical care ultrasonography.

Transitional Bladder Cell Carcinoma Spreading to the Skin.

Cutaneous metastases from bladder malignancies are rare. We report the case of a 74 year old man who underwent cysto-prostatectomy and adjuvant chemotherapy for a pT3b N+ bladder transitional cell carcinoma. Four months later, he presented with skin disseminated pigmented lesions. Skin biopsy confirmed cutaneous metastasis from urothelial carcinoma.

Multimodality Imaging of Cardiac Transthyretin Amyloidosis 16 Years After a Domino Liver Transplantation.

We report the case of a 62-year-old man hospitalized in May 2015 for symptomatic heart failure. His medical history included two liver transplantations. The first liver transplantation was performed in 1999 for a mixed alcoholic and hepatitis C-related cirrhosis and the patient received the liver of another patient with Val30Met transthyretin amyloidosis using the domino technique. In 2008, he complained of neuropathic pains and an iatrogenic-acquired transthyretin amyloidosis was diagnosed. On cardiac evaluation, amyloidosis was suspected. In March 2010, a second liver transplantation was performed with a deceased donor without complication. In May 2015, a first episode of symptomatic heart failure occurred and cardiac amyloidosis was investigated by a multimodality evaluation. Electrocardiogram, cardiac biomarkers, echocardiography, and cardiac MRI were in favor of the diagnosis of amyloidosis, whereas 99m Tc-dicarboxypropane diphosphonate scintigraphy was not. Endomyocardial biopsy finally confirmed the positive diagnosis of iatrogenic-acquired cardiac amyloidosis. This case is, to the best of our knowledge, the first to report biopsy-proven cardiac amyloidosis induced by domino liver transplantation and progressing heart failure in spite of retransplantation. The diagnostic modalities are discussed. This case should alert physicians to the cardiac risk in domino liver transplanted patients.