Long-Term Retrospective Analysis of Gene Therapy with Alipogene Tiparvovec and Its Effect on Lipoprotein Lipase Deficiency-Induced Pancreatitis.

Alipogene tiparvovec (Glybera) is a gene therapy product approved in Europe under the "exceptional circumstances" pathway as a treatment for lipoprotein lipase deficiency (LPLD), a rare genetic disease resulting in chylomicronemia and a concomitantly increased risk of acute and recurrent pancreatitis, with potentially lethal outcome. This retrospective study analyzed the frequency and severity of pancreatitis in 19 patients with LPLD up to 6 years after a single treatment with alipogene tiparvovec. An independent adjudication board of three pancreas experts, blinded to patient identification and to pre- or post-gene therapy period, performed a retrospective review of data extracted from the patients' medical records and categorized LPLD-related acute abdominal pain events requiring hospital visits and/or hospitalizations based on the adapted 2012 Atlanta diagnostic criteria for pancreatitis.

Evaluating Continuity During Transfer to Adult Care: A Systematic Review.

Context: Appropriate outcomes are required to evaluate transition programs' ability to maintain care continuity during the transfer to adult care of youths with a chronic condition.

Objective: To identify the outcomes used to measure care continuity during transfer, and to analyze current evidence regarding the efficacy of transition programs.

Data Sources: PubMed (1948-2014), Web of Science (1945-2014), Embase (1947-2014), and the reference lists of the studies identified.

Is the benefit of laparoscopy maintained in elderly patients undergoing rectal cancer resection? An analysis of 446 consecutive patients.

Background: Several studies showed that age is significantly associated with impaired outcomes after open colorectal surgery. However, very few data exist on laparoscopic rectal cancer surgery in elderly patients. The aim of this study was to assess operative results of laparoscopic rectal cancer surgery according to age.

Proliferation and ovarian hormone signaling are impaired in normal breast tissues from women with BRCA1 mutations: benefit of a progesterone receptor modulator treatment as a breast cancer preventive strategy in women with inherited BRCA1 mutations.

Women with inherited BRCA1 mutations have an elevated risk (40-80%) for developing breast and ovarian cancers. Reproductive history has been reported to alter this risk, suggesting a relationship between ovarian hormone signaling and BRCA1-related tumor development. BRCA1 interactions with estrogen receptor (ER) and progesterone receptor (PR) signaling were previously described in human breast cancer cell lines and mouse models.

Do Biologics Protect Patients With Psoriasis From Myocardial Infarction? A Retrospective Cohort.

Background: Psoriasis is a chronic immune-mediated inflammatory disorder that affects approximately 2% to 3% of the population, which translates to 17 million in North America and Europe and approximately 170 million people worldwide. Although psoriasis can occur at any age, most cases develop before age 40 years. Some larger studies have noted bimodal age at onset with the first peak occurring at approximately age 30 years and the second peak at around 55 to 60 years, but most patients have a younger age of onset (15-30 years).

Sleep disorders and oral health: a cross-sectional study.

Objectives: Sleep disorders (SDs), particularly sleep deprivation, may alter the immune system and induce systemic inflammation. Recent evidence supports an association between SDs and periodontal diseases. This cross-sectional epidemiological study aims to compare oral health variables, such as the amount of plaque/calculus, gingival inflammation, and masticatory function, in individuals with and without SDs.

Estimated glomerular filtration rate but not solute carrier polymorphisms influences anemia in HIV-hepatitis C virus coinfected patients treated with boceprevir or telaprevir-based therapy.

Objectives: Ribavirin (RBV) induced anemia may be influenced by host genetic factors affecting RBV transport solute carrier (SLC) or metabolism inosine triphosphatase (ITPA), as already reported. We investigated the influence of single nucleotide polymorphisms (SNPs) on SLC genes on anemia, RBV trough concentration (Ctrough) and response in HIV-hepatitis C virus coinfected patients receiving triple therapy with boceprevir or telaprevir.

Methods: Patients from the ANRS HC26/HC27 studies were genotyped for SLC28A3 SNPs (rs10868138 and rs56350726) and SL29A1 SNPs (rs760370).

Olmesartan-induced enteropathy associated with cutaneous lesions.

Olmesartan is an angiotensin II receptor antagonist which may cause severe sprue-like enteropathy with duodenal villous atrophy. Skin lesions may be associated as reported for the first time in our case. Clinicians should be informed of this side effect and its reversibility after suspension of the drug.

Diagnostic delay of pyogenic vertebral osteomyelitis and its associated factors.

Objectives: Pyogenic vertebral osteomyelitis (PVO) is a rare disease with possible severe complications (e.g. sepsis and spinal cord injury).

[Zika virus infection during pregnancy].

A Zika virus epidemic is currently ongoing in the Americas. This virus is linked to congenital infections with potential severe neurodevelopmental dysfunction. However, incidence of fetal infection and whether this virus is responsible of other fetal complications are still unknown.

Addition of boceprevir to PEG-interferon/ribavirin in HIV-HCV-Genotype-1-coinfected, treatment-experienced patients: efficacy, safety, and pharmacokinetics data from the ANRS HC27 study.

Background: Scarce data exist on the efficacy and safety of the PEGylated-interferon/ribavirin/boceprevir regimen in HIV/HCV-coinfected patients who failed to respond to PEGylated-interferon/ribavirin treatment.

Objectives: To evaluate the efficacy and safety of this drug regimen and the impact of the addition of boceprevir(BOC) on atazanavir (ATV) or raltegravir (RAL) pharmacokinetic parameters in a subgroup of patients.

Methods: In this single-arm phase 2 trial, HIV-1/HCV-genotype-1-coinfected patients received PEGylated-interferonα2b (1.

Endoscopic description of renal papillary abnormalities in stone disease by flexible ureteroscopy: a proposed classification of severity and type.

Introduction: The goal of this original work was to describe papillary abnormalities using flexible ureteroscopy into a new classification and to assess their relation with stone composition.

Patients And Methods: We performed a prospective monocentric single-operator study aiming to describe various aspects of renal papillae. Data have been prospectively collected during consecutive 164 sequential flexible ureterorenoscopies required for the treatment of renal stones from May 2011 to March 2015.

Determinants of macroscopic anal cancer and precancerous lesions in 1206 HIV-infected screened patients.

Aim: Anal screening is recommended in HIV-positive patients, especially men who have sex with men (MSM), due to an increased incidence of anal cancer. The optimal screening methods are not generally agreed.

Method: Screening for anal lesions by anorectal examination, including anoscopy, was offered to HIV-positive outpatients in a tertiary care university hospital regardless of gender or sexual orientation.

Prognostic Value of Preoperative Brain Natriuretic Peptide Serum Levels in Liver Transplantation.

Background: Brain natriuretic peptide (BNP) serum concentration has been shown to be a preoperative predictor of postoperative outcome in high risk surgery. Whether it is able to predict early post-liver transplantation (LT) mortality in cirrhotic patients is unanswered.

Methods: Prospective monocenter observational study including all consecutive patients who received LT for cirrhosis and for whom a preoperative BNP serum dosage was available between January 2011 and December 2014.

Women infected with human immunodeficiency virus type 1 have poorer assisted reproduction outcomes: a case-control study.

Objective: To compare the efficacy of assisted reproductive technology (ART) in women infected with human immunodeficiency virus type 1 (HIV-1) versus HIV-negative controls.

Design: Retrospective case-control study.

Setting: University hospital ART unit.

Organic molecules in the Sheepbed Mudstone, Gale Crater, Mars.

Unlabelled: The Sample Analysis at Mars (SAM) instrument on board the Mars Science Laboratory Curiosity rover is designed to conduct inorganic and organic chemical analyses of the atmosphere and the surface regolith and rocks to help evaluate the past and present habitability potential of Mars at Gale Crater. Central to this task is the development of an inventory of any organic molecules present to elucidate processes associated with their origin, diagenesis, concentration, and long-term preservation. This will guide the future search for biosignatures.

Frequency and Risk Factors for Associated Lymphomas in Patients With Lymphomatoid Papulosis.

Background: Lymphomatoid papulosis (LyP) is classified as an indolent cutaneous lymphoma, but outcome dramatically worsens if LyP is associated with lymphoma. The frequency of this association remains unclear in the literature. Here, we assess the frequency and risk factors of association between LyP and another lymphoma in an 11-year retrospective study conducted in 8 dermatology departments belonging to the French Study Group on Cutaneous Lymphoma (FSGCL).

Does ex vivo CD34+ positive selection influence outcome after autologous hematopoietic stem cell transplantation in systemic sclerosis patients?

This EBMT Autoimmune Disease Working Party study aimed to evaluate the influence of CD34+ positive graft selection (CD34+) on the outcome of systemic sclerosis (SSc) patients after autologous hematopoietic stem cell transplantation (AHSCT). Clinical and laboratory data from 138 SSc patients at diagnosis, before and after AHSCT were retrospectively analyzed. CD34+ selection was performed in 47.

Membrane-anchored Serine Protease Matriptase Is a Trigger of Pulmonary Fibrogenesis.

Rationale: Idiopathic pulmonary fibrosis (IPF) is a devastating disease that remains refractory to current therapies.

Objectives: To characterize the expression and activity of the membrane-anchored serine protease matriptase in IPF in humans and unravel its potential role in human and experimental pulmonary fibrogenesis.

Methods: Matriptase expression was assessed in tissue specimens from patients with IPF versus control subjects using quantitative reverse transcriptase-polymerase chain reaction, immunohistochemistry, and Western blotting, while matriptase activity was monitored by fluorogenic substrate cleavage.

Improvement of skin wound healing in diabetic mice by kinin B2 receptor blockade.

Impaired skin wound healing is a major medical problem in diabetic subjects. Kinins exert a number of vascular and other actions limiting organ damage in ischaemia or diabetes, but their role in skin injury is unknown. We investigated, through pharmacological manipulation of bradykinin B1 and B2 receptors (B1R and B2R respectively), the role of kinins in wound healing in non-diabetic and diabetic mice.

Nab-paclitaxel plus gemcitabine for metastatic pancreatic adenocarcinoma after Folfirinox failure: an AGEO prospective multicentre cohort.

Background: There is currently no standard second-line treatment for metastatic pancreatic adenocarcinoma (MPA), and progression-free survival is consistently <4 months in this setting. The aim of this study was to evaluate the efficacy and tolerability of Nab-paclitaxel plus gemcitabine (A+G) after Folfirinox failure in MPA.

Methods: From February 2013 to July 2014, all consecutive patients treated with A+G for histologically proven MPA after Folfirinox failure were prospectively enrolled in 12 French centres.

Oral health in patients treated by positive airway pressure for obstructive sleep apnea: a population-based case-control study.

Purpose: Recent epidemiological evidence suggests that patients with obstructive sleep apnea (OSA) have an increased risk of periodontal disease. Little is known about the oral health of OSA patients treated by continuous or bi-level positive airway pressure (CPAP/BiPAP). The aim of this population-based case-control study was to compare oral health variables (amount of plaque, calculus, gingival inflammation, and masticatory function) between CPAP/BiPAP users and control subjects.