Outcome and prognosis of isolated carotid vasculitis.

OBJECTIVE: To assess the prognosis and outcome of patients with isolated carotid vasculitis. METHODS: We performed a retrospective multicenter study of 36 patients (median age at diagnosis was 37 [IQR 27-45] years and 11 [31 %] patients were men) with initial presentation as isolated carotid vasculitis. Study endpoints included vascular complications, relapses, and progression to large vessel vasculitis (i.e. Giant cell arteritis or Takayasu). RESULTS: The most frequent involvement was the left internal carotid artery (39 %), and 81 % had stenosis. After a median follow-up of 32 months [IQR 12-96], 21 (58 %) patients had a vascular event, including 31 % of new onset vascular lesions and 25 % of stroke/transient ischemic attack. Patients with stroke had less carotidynia at diagnosis (33 % vs 74 %, p = 0.046), higher significant carotid stenosis (i.e. > 50 %) (89 % vs. 30 %, p = 0.026) and higher severe carotid stenosis (i.e. >70 %) (67 % vs 19 %, p = 0.012), compared to those without stroke. Twenty (52 %) patients experienced relapses. High CRP at diagnosis was associated with relapses (p = 0.022). At the end of follow-up, 21 (58 %) patients were classified as having Takayasu arteritis, 13 (36 %) as isolated carotid vasculitis, and two (6 %) as giant cell arteritis. CONCLUSION: Carotid vasculitis may occur as a topographically limited lesion and is associated with significant rate of vascular complications.

Hepaticojejunostomy for bile duct injury: state of the art.

BACKGROUND: Hepaticojejunostomy (HJ) is the gold standard procedure for the reconstruction of the bile duct in many benign and malignant situations. One of the major situation is the bile duct injury (BDI) after cholecystectomy, either for early or late repair. This procedure presents some specificities associated to a debated management of BDI. PURPOSE: This article provides a state-of-the-art of the hepaticojejunostomy procedure focusing on bile duct injury including its indications and outcomes CONCLUSION: Performed at the right moment and respecting the technical rules, HJ provides a restoration of the biliary patency in the long term of 80 to 90%. It is the main surgical technique to repair BDI. Complications and failure of this procedure can be difficult to manage. That is why the primary repair requires an appropriate multidisciplinary approach associated with an expert high quality surgical technique.

Impact of cirrhosis in patients undergoing laparoscopic liver resection in a nationwide multicentre survey.

BACKGROUND: The aim was to analyse the impact of cirrhosis on short-term outcomes after laparoscopic liver resection (LLR) in a multicentre national cohort study. METHODS: This retrospective study included all patients undergoing LLR in 27 centres between 2000 and 2017. Cirrhosis was defined as F4 fibrosis on pathological examination. Short-term outcomes of patients with and without liver cirrhosis were compared after propensity score matching by centre volume, demographic and tumour characteristics, and extent of resection. RESULTS: Among 3150 patients included, LLR was performed in 774 patients with (24·6 per cent) and 2376 (75·4 per cent) without cirrhosis. Severe complication and mortality rates in patients with cirrhosis were 10·6 and 2·6 per cent respectively. Posthepatectomy liver failure (PHLF) developed in 3·6 per cent of patients with cirrhosis and was the major cause of death (11 of 20 patients). After matching, patients with cirrhosis tended to have higher rates of severe complications (odds ratio (OR) 1·74, 95 per cent c.i. 0·92 to 3·41; P = 0·096) and PHLF (OR 7·13, 0·91 to 323·10; P = 0·068) than those without cirrhosis. They also had a higher risk of death (OR 5·13, 1·08 to 48·61; P = 0·039). Rates of cardiorespiratory complications (P = 0·338), bile leakage (P = 0·286) and reoperation (P = 0·352) were similar in the two groups. Patients with cirrhosis had a longer hospital stay than those without (11 versus 8 days; P = 0·018). Centre expertise was an independent protective factor against PHLF in patients with cirrhosis (OR 0·33, 0·14 to 0·76; P = 0·010). CONCLUSION: Underlying cirrhosis remains an independent risk factor for impaired outcomes in patients undergoing LLR, even in expert centres.

ANTECEDENTES: El objetivo de este estudio fue analizar el impacto de la cirrosis en los resultados a corto plazo después de la resección hepática laparoscópica (laparoscopic liver resection, LLR) en un estudio de cohortes multicéntrico nacional. MÉTODOS: Este estudio retrospectivo incluyó todos los pacientes sometidos a LLR en 27 centros entre 2000 y 2017. La cirrosis se definió como fibrosis F4 en el examen histopatológico. Los resultados a corto plazo de los pacientes con hígado cirrótico (cirrhotic liver CL) (pacientes CL) y los pacientes con hígado no cirrótico (non-cirrhotic liver, NCL) (pacientes NCL) se compararon después de realizar un emparejamiento por puntaje de propension del volumen del centro, las características demográficas y del tumor, y la extensión de la resección. RESULTADOS: Del total de 3.150 pacientes incluidos, se realizó LLR en 774 (24,6%) pacientes CL y en 2.376 (75,4%) pacientes NCL. Las tasas de complicaciones graves y mortalidad en el grupo de pacientes CL fueron del 10,6% y 2,6%, respectivamente. La insuficiencia hepática posterior a la hepatectomía (post-hepatectomy liver failure, PHLF) fue la principal causa de mortalidad (55% de los casos) y se produjo en el 3,6% de los casos en pacientes CL. Después del emparejamiento, los pacientes CL tendieron a tener tasas más altas de complicaciones graves (razón de oportunidades, odds ratio, OR 1,74; i.c. del 95% 0,92-0,41; P = 0,096) y de PHLF (OR 7,13; i.c. del 95% 0,91-323,10; P = 0,068) en comparación con los pacientes NCL. Los pacientes CL estuvieron expuestos a un mayor riesgo de mortalidad (OR 5,13; i.c. del 95% 1,08-48,6; P = 0,039) en comparación con los pacientes NCL. Los pacientes CL presentaron tasas similares de complicaciones cardiorrespiratorias graves (P = 0,338), de fuga biliar (P = 0,286) y de reintervenciones (P = 0,352) que los pacientes NCL. Los pacientes CL tuvieron una estancia hospitalaria más larga (11 versus 8 días; P = 0,018) que los pacientes NCL. La experiencia del centro fue un factor protector independiente de PHLF (OR 0,33; i.c. del 95% 0,14-0,76; P = 0,010) pacientes CL. CONCLUSIÓN: La presencia de cirrosis subyacente sigue siendo un factor de riesgo independiente de peores resultados en pacientes sometidos a resección hepática laparoscópica, incluso en centros con experiencia.

Perioperative antibiotherapy should replace prophylactic antibiotics in patients undergoing pancreaticoduodenectomy preceded by preoperative biliary drainage.

BACKGROUND AND OBJECTIVES: Pancreaticoduodenectomy (PD) remains a morbid surgery. Preoperative biliary drainage (PBD) is often necessary before surgery but is associated with biliary contamination. We compared the postoperative complications of patients undergoing PBD who received the usual prophylactic antibiotics (PAs) or systematic antibiotherapy (ABT). METHODS: All patients who underwent surgery between 2008 and 2017 were included. Systematic perioperative ABT with piperacillin + tazobactam (ABT group) was implemented in 2014 as the standard of care for PBD. Patients treated in the period before such implementation, during which standard cefazolin was given, served as the controls (PAs group). The primary outcomes were postoperative complications. RESULTS: We included 122 patients with PBD who underwent surgery. There were no demographic differences between the two groups. Perioperative ABT was associated with a reduction in deep abdominal abscesses (36% vs 10%, P = .0008), respiratory tract infections (15% vs 3%; P = .02), bacteremia (41% vs 6%; P < .0001), and a shorter length of hospital stay (17 [13-27] vs 13 [10-14] days; P < .0001). ABT was a protective factor against the development of deep abdominal abscesses (odds ratio [OR] = 0.16; P = .001) whereas smoking (OR = 3.9) and pancreatic fistula (OR = 19.1) were risk factors. CONCLUSION: Systematic perioperative ABT in patients undergoing PD preceded by PBD may reduce deep surgical infections and the length of hospital stay.

Recurrent attacks of acute hepatic porphyria: major role of the chronic inflammatory response in the liver.

BACKGROUND: Acute intermittent porphyria (AIP) is an inherited disorder of haem metabolism characterized by life-threatening acute neurovisceral attacks due to the induction of hepatic δ-aminolevulinic acid synthase 1 (ALAS1) associated with hydroxymethylbilane synthase (HMBS) deficiency. So far, the treatment of choice is hemin which represses ALAS1. The main issue in the medical care of AIP patients is the occurrence of debilitating recurrent attacks. OBJECTIVE: The aim of this study was to determine whether chronic hemin administration contributes to the recurrence of acute attacks. METHODS: A follow-up study was conducted between 1974 and 2015 and included 602 French AIP patients, of whom 46 had recurrent AIP. Moreover, we studied the hepatic transcriptome, serum proteome, liver macrophage polarization and oxidative and inflammatory profiles of Hmbs-/- mice chronically treated by hemin and extended the investigations to five explanted livers from recurrent AIP patients. RESULTS: The introduction of hemin into the pharmacopeia has coincided with a 4.4-fold increase in the prevalence of chronic patients. Moreover, we showed that both in animal model and in human liver, frequent hemin infusions generate a chronic inflammatory hepatic disease which induces HO1 remotely to hemin treatment and maintains a high ALAS1 level responsible for recurrence. CONCLUSION: Altogether, this study has important impacts on AIP care underlying that hemin needs to be restricted to severe neurovisceral crisis and suggests that alternative treatment targeting the liver such as ALAS1 and HO1 inhibitors, and anti-inflammatory therapies should be considered in patients with recurrent AIP.

Peripheral nerve involvement in Fabry's disease: Which investigations? A case series and review of the literature.

BACKGROUND: Peripheral nerve system (PNS) involvement is common in Fabry's disease (FD), predominantly affecting the small nerve fibers that are difficult to investigate with conventional electrophysiological methods. PATIENTS AND METHODS: Eighteen patients followed for Fabry's disease underwent a prospective series of electroneurophysiological explorations, including a study of the cardiac parasympathetic autonomic nervous system (ANS) and electrochemical skin conductance (ESC) tests. Data were compared with those obtained in 18 matched healthy controls. RESULTS: All patients had at least one clinical sign suggestive of neuropathy: 16 reported an acrosyndrome and 12 had dyshidrosis. Cold hypoesthesia was found in 15 patients and heat hypoesthesia in 13. Electroneurophysiological investigations and study of the cardiac parasympathetic ANS were normal in all patients. The ESC was significantly lower in FD patients compared with controls. CONCLUSION: PNS involvement is common in FD and should be suspected in patients exhibiting an acrosyndrome, dyshidrosis and/or cold hypoesthesia. Conventional electrophysiological investigations are normal. New techniques, such as ESC, provide early diagnosis of small fiber involvement that currently requires more sophisticated tests difficult to apply in routine practice.

Characteristics and information searched for by French patients with systemic lupus erythematosus: A web-community data-driven online survey.

INTRODUCTION: To provide information about the needs of patients with systemic lupus erythematosus (SLE) using Carenity, the first European online platform for patients with chronic diseases. METHODS: At one year after its creation, all posts from the Carenity SLE community were collected and analysed. A focused cross-sectional online survey was performed. RESULTS: The SLE community included 521 people (93% females; mean age: 39.8 years). Among a total of 6702 posts, 2232 were classified according to disease-related topics. The 10 most common topics were 'lupus and …' either 'treatment', 'fatigue', 'entourage', 'sun exposure', 'diagnosis', 'autoimmune diseases', 'pregnancy', 'contraception', 'symptoms' or 'sexuality'. 112 SLE patients participated in the online survey. At the time of diagnosis, only 17 (15%) patients had heard of SLE and 84 (75%) expressed a need for more information on outcomes (27%), treatments (27%), daily life (14%), patients' associations (11%), symptoms (8%), the disease (8%) and psychosocial aspects (7%). When treatment was initiated, 48 patients (43%) would have liked more information about side effects (46%), long-term effects (21%), treatment duration/cessation (12.5%) and type (10%) and mechanism of action (8%) of treatments. All participants except one had used the internet to find information about SLE. Sources of information included healthcare providers (51%/61%/67%), journals/magazines (7%/12%/6%), lupus Websites (51%/77%/40%), web forums/blogs (34%/53%/19%), patients' associations (11%/23%/9%) accessed at 'just before diagnosis', 'just after diagnosis' and 'before treatment initiation'. CONCLUSIONS: Online patient communities provide original unbiased information that can help improve provision of information to SLE patients.

Restoration of intestinal continuity after colectomy for non-occlusive ischemic colitis.

BACKGROUND: Non-occlusive ischemic colitis (IC) is a rare and life-threatening abdominal disease associated with high rates of postoperative mortality. When surgery is performed, in patients with IC, either a Hartmann's procedure (HP) or a total colectomy and ileostomy is required. The possibility of restoration of intestinal continuity in surviving patients is an important issue. The aim of the present study was to report the outcome of surgically managed IC patients and to identify predictive factors for restoration of intestinal continuity and to assess the results of this procedure. METHODS: Between January 1997 and May 2011, 96 IC patients underwent total colectomy and 68 underwent left colectomy. IC was spontaneous in 62 patients and occurred after prior surgery in 102. Eighty patients died during the postoperative period and nine died during the follow-up from an unrelated disease. Fifteen patients were lost to follow-up. The remaining 60 surviving patients were our study population. RESULTS: There were 44 men and 16 women with a mean age of 67 years ± 12 SD. Restoration of bowel continuity was performed in 24 patients (40%). There were no predictive factors for restoration of intestinal continuity in terms of sex, age, IC etiology, and the extent of colon resection at primary surgery. The median interval between first surgery and restoration of bowel continuity was 7.9 months (range 0.2-35 months). There were no postoperative deaths and the overall morbidity rate was 45% (11/24). No patients developed anastomotic leak or underwent unplanned reoperation. CONCLUSIONS: Reversal of HP after IC is feasible in 40% of surviving patients with acceptable mortality and morbidity rates. This restoration of intestinal continuity should therefore be discussed for every surviving IC patient.

[Capecitabine-induced subacute cutaneous lupus: a case report]. / Lupus cutané subaigu induit par la capécitabine : un cas.

BACKGROUND: More than 100 drugs have been registered as inducing subacute cutaneous lupus erythematosus (SCLE). Recently, some types of chemotherapy have also been incriminated. If SCLE develops in a setting of neoplasia, two possibilities should be considered: it is either a paraneoplastic syndrome or it is caused by the chemotherapy, thus calling for important decisions on the benefit/risk of stopping potentially effective medication. We report a case of SCLE induced by Xeloda (capecitabine). PATIENTS AND METHODS: A 50-year-old female patient consulted with an annular erythematosquamous and pruriginous eruption, predominantly on areas of the body exposed to sunlight, occurring 4 months after the initiation of capecitabine for advanced colon cancer. She had presented systemic lupus erythematosus (SLE) for many years, which was not treated, was not progressive and had no cutaneous manifestations. The appearance of the cutaneous lesions, positivity for anti-SSA antibodies and the histological aspect led to diagnosis of SCLE. The lesions were resistant to treatment with hydroxychloroquine and systemic corticosteroids, but disappeared after discontinuation of capecitabine, suggesting chemotherapy-induced SCLE. DISCUSSION: Some types of chemotherapy such as capecitabine may reveal or induce SCLE lesions, whether or not there is a previous history of SLE. Cases of chemotherapy-induced cutaneous lupus reported to the French pharmacovigilance agency are rare, but this side effect must be recognised due to the constantly rising use of this type of anticancer agent.

Hepatic artery ligation for arterial rupture following liver transplantation: a reasonable option.

Hepatic artery (HA) rupture after liver transplantation is a rare complication with high mortality. This study aimed to review the different managements of HA rupture and their results. From 1997 to 2007, data from six transplant centers were reviewed. Of 2649 recipients, 17 (0.64%) presented with HA rupture 29 days (2-92) after transplantation. Initial management was HA ligation in 10 patients, reanastomosis in three, aorto-hepatic grafting in two and percutaneous arterial embolization in one. One patient died before any treatment could be initiated. Concomitant biliary leak was present in seven patients and could be subsequently treated by percutaneous and/or endoscopic approaches in four patients. Early mortality was not observed in patients with HA ligation and occurred in 83% of patients receiving any other treatment. After a median follow-up of 70 months, 10 patients died (4 after retransplantation), and 7 patients were alive without retransplantation (including 6 with HA ligation). HA ligation was associated with better 3-year survival (80% vs. 14%; p=0.002). Despite its potential consequences on the biliary tract, HA ligation should be considered as a reasonable option in the initial management for HA rupture after liver transplantation. Unexpectedly, retransplantation was not always necessary after HA ligation in this series.

[Successful treatment with rituximab in a refractory Stiff-person syndrome]. / Efficacité du rituximab dans le traitement d'un Syndrome de Stiff person réfractaire.

Stiff person syndrome is a rare autoimmune disorder characterized by axial and limb progressive stiffness with surimposed spasms and production of autoantibodies to glutamic acid decarboxylase (GAD). We report a case of a 50-year-old woman who developed a stiff person syndrome resistant to conventional immunosuppressive treatments. Eight months after treatment, indexes of stiffness and spasm frequency improved, while however, the blood and CSF rates of anti-GAD increased. This observation illustrates the complexity of stiff person syndrome immunopathogenesis as well as the relevance of rituximab in this indication.

[Ventilator-associated viral pneumonia]. / Pneumonies virales acquises sous ventilation mécanique.

Viral infections (especially respiratory infections) are not rare in critically ill non-immunocompromised patients. Efficient and rapid virologic diagnosis tests such as polymerase chain reaction (PCR) are now widely available. Herpesviridae (herpes simplex virus and cytomegalovirus) are the most frequent viruses detected among non-immunocompromised patients admitted to the intensive care unit (ICU). However, causal relationships between detected viruses and outcomes are still debated, with a variable level of demonstration among the different viruses. The aim of this review was to assess the role of viruses in causing mechanical ventilation-acquired pneumonias in non-immunocompromised ICU adult patients. We also discuss the possible physiopathology of these viral infections, as well as the opportunity for therapeutic interventions.

Bochdalek diaphragmatic hernia with bowel incarceration.

The purpose of this image is to illustrate a Bochdalek hernia diagnosis in a 39-year-old female patient treated on an emergency basis by means of CT-scan and following perioperative observation. Treatment consisted in initial laparoscopy that due to irreducible bowel incarceration was converted into hernia repair surgery through simple suture and thoracic drainage.

Metacarpophalangeal joint instability in trapeziometacarpal osteoarthritis: A systematic review.

Thumb metacarpophalangeal instability is commonly found in conjunction with trapeziometacarpal osteoarthritis. If not corrected, it can have detrimental effects on the outcome. The authors describe the two types of metacarpophalangeal deformities - hyperextension and valgus - their pathophysiology and the surgical repair techniques available to surgeons. An algorithm for treating this instability is presented.

[Diagnosis of vitamin B12 deficiency: a case illustrating diagnostic pitfalls]. / Diagnostic d'une carence en vitamine B12 ou le signe de l'ordonnance.

INTRODUCTION: Vitamin B12 deficiency is a longstanding public health problem which affects more than 20% of the elderly population. Among multiple causes of vitamin B12 deficiency, Biermer's disease is currently mentioned in about 25% of the cases. OBSERVATION: We report the case of a 71-year-old woman, taking folate substitution therapy who, over 2 years, progressively developed spinal combined sclerosis, subacute dementia and severe neuropathy leading to a bedridden state. The initial assessment revealed normocytic anemia, without vitamin B12 deficiency and without increased plasma level of biological markers. The plasma folate level was high. Vitamin B12 assay was repeated leading to the diagnosis of deficiency associated with the presence of intrinsic factor antibodies. DISCUSSION: This observation illustrates the broad spectrum of clinical presentations of vitamin B12 deficiency. In the present case, the lack of sensitivity of biological markers delayed diagnosis and had a dramatic impact on outcome. This case highlights the importance of promoting factors such as isolated folate substitution in B12 deficient patients.

[Artificial intelligence: Guidelines for internists]. / Des guidelines pour l'intelligence artificielle !

Following the emergence of open public databases and connected objects, big data and artificial intelligence are developing rapidly, especially in medicine, with many opportunities ranging from complex diagnostic assistance to real-time statistical analysis. In order to promote their development and guide their use in the field of internal medicine, guidelines and recommendations are needed. First of all, this article seeks to clarify the concepts of big data and artificial intelligence and the correlations between each other, and then to give an overview of the progress made at European level in this rapidly expanding field.

Fingertip nail bed injuries in children: Comparison of suture repair versus glue (2-octylcyanoacrylate) with 1-year follow-up.

The main objective of this study was to compare the medium-term results of nail bed repair in children using glue (2-octylcyanoacrylate) versus absorbable sutures. The secondary objective was to compare the results of treatment in the emergency room versus the operating room. This retrospective review of 74 fingertip nail bed lacerations (68 children) evaluated the appearance and pain at the last follow-up visit (minimum of 1 year), and the operating time. Mean age was 3.3 years at time of injury (range 10 months-13 years), with a mean follow-up of 2.6 (1-7) years. Thirty-six nail beds were repaired with glue; 38 were sutured. The clinical outcomes in the two groups were similar. The rate of nail dystrophy was 14% (5% major) regardless of the technique. Nail bed repair time was significantly shorter in the glue group (10.2 vs. 20.3min, p<0.001). Forty-five repairs were performed in the operating room and 29 in the emergency room. The complication rate (early infections) was significantly higher in patients treated in the emergency room. Tissue adhesive (2-octylcyanoacrylate glue) is a reliable option for repairing nail bed lacerations, both in terms of outcomes and speed of repair. Treatment in the operating room is preferable.

Structure of Debaryomyces castellii CBS 2923 phytase.

Phytate (myo-inositol hexakisphosphate) is the primary storage form of phosphate in seeds and legumes (Reddy et al., 1982). Phytases are phosphatases that hydrolyze phytate to less phosphorylated myo-inositol derivatives and inorganic phosphate. The crystal structure of phytase from Debaryomyces castellii has been determined at 2.3 A resolution. The crystals belonged to space group P6(5)22, with unit-cell parameters a = 121.65, c = 332.24 A. The structure was solved by molecular replacement and refined to a final R factor of 15.7% (R(free) = 20.9%). The final model consists of a dimer (with two monomers of 458 residues), five NAG molecules and 628 water molecules.

[Spontaneous rupture of hepatic biliary cyst]. / Rupture spontanée d'un kyste biliaire du foie.

Hepatic cysts (often called biliary cysts of the liver) are common and usually asymptomatic. Spontaneous cyst rupture into the peritoneum is a rare but potentially grave complication. This complication can be prevented by elective laparoscopic unroofing of the dome of the cyst. This is a simple and reliable technique which offers both accurate diagnosis and protection from spontaneous rupture.