The effect of increasing the dose of hydroxychloroquine (HCQ) in patients with refractory cutaneous lupus erythematosus (CLE): An open-label prospective pilot study.

Background: Up to 30% of patients with cutaneous lupus erythematosus (CLE) fail to respond to hydroxychloroquine (HCQ).

Objectives: We sought to evaluate the efficacy of increased daily doses of HCQ on cutaneous response in refractory CLE.

Methods: We conducted an open-label prospective study between 2010 and 2014.

Systematic review with meta-analysis: infliximab and immunosuppressant therapy vs. infliximab alone for active ulcerative colitis.

Background: The benefit of the combination of infliximab (IFX) and immunosuppressant (IS) therapy is debated in ulcerative colitis (UC).

Aims: To determine whether the combination of IFX and IS therapy is more effective than infliximab alone for active UC regardless of prior IS use.

Methods: We identified all controlled trials including patients with moderate-to-severe active UC, treated by either IFX or combined IFX-IS therapy.

[Injectable preparation of labeled leucine with the carbon 13 for a clinical research program on the Alzheimer disease: pharmaceutical control of raw materials and the finished product and stability study].

Introduction: The L-leucine labeled (L-[U-(13)C] Leu) is a stable isotopic tracer administered by parenteral route within the framework of a new clinical research program concerning the diagnosis of the Alzheimer's disease. To meet regulatory requirements and have ready to use solution with an expiration date, a pharmaceutical control of raw materials and the finished product followed by a stability study were realised.

Materials And Method: After the pharmaceutical control of raw materials, the solution of L-[U-(13)C] Leu was prepared according to the good practices preparation.

The motor function measure to study limitation of activity in children and adults with Charcot-Marie-Tooth disease.

Objective: To study the applicability and responsiveness of the motor function measure (total score and sub-scores D1, D2 and D3) in patients with Charcot-Marie-Tooth disease.

Patients And Methods: Two hundred and thirty-three patients aged 4-86 years were included in the descriptive study. Scores and sub-scores were analyzed by age and by disease subtypes.

Rasch analysis of the motor function measure in patients with congenital muscle dystrophy and congenital myopathy.

Objectives: To monitor treatment effects in patients with congenital myopathies and congenital muscular dystrophies, valid outcome measures are necessary. The Motor Function Measure (MFM) was examined for robustness, and changes are proposed for better adequacy.

Design: Observational study based on data previously collected from several cohorts.

English cross-cultural translation and validation of the neuromuscular score: a system for motor function classification in patients with neuromuscular diseases.

Objective: To develop and validate an English version of the Neuromuscular (NM)-Score, a classification for patients with NM diseases in each of the 3 motor function domains: D1, standing and transfers; D2, axial and proximal motor function; and D3, distal motor function.

Design: Validation survey.

Setting: Patients seen at a medical research center between June and September 2013.

Systemic delivery of siRNA down regulates brain prion protein and ameliorates neuropathology in prion disorder.

One of the main challenges for neurodegenerative disorders that are principally incurable is the development of new therapeutic strategies, which raises important medical, scientific and societal issues. Creutzfeldt-Jakob diseases are rare neurodegenerative fatal disorders which today remain incurable. The objective of this study was to evaluate the efficacy of the down-regulation of the prion protein (PrP) expression using siRNA delivered by, a water-in-oil microemulsion, as a therapeutic candidate in a preclinical study.

Preoperative evaluation in aortic endocarditis: findings on cardiac CT.

Objective: The purpose of this study was to study the feasibility and diagnostic capability of preoperative cardiac CT for depicting aortic valvular pseudoaneurysms and vegetations in patients referred for aortic endocarditis requiring surgical intervention.

Materials And Methods: Consecutive patients presenting with active aortic endocarditis requiring surgical intervention were included. CT scan examinations were performed for assessing coronary artery status.

Painful camptocormia: the relevance of shaking your patient's hand.

Camptocormia is an abnormal posture with marked flexion of thoracolumbar spine that abates in the recumbent position. Camptocormia has been described in various neurological (Parkinsonism), muscular (myopathy), psychogenic or orthopedic disorders. There are several hypotheses that can explain this impaired posture but they are usually related to the concomitant pathologies.

[A case of intrahepatic gastrinoma].

The Zollinger-Ellison syndrome is due to an endocrine gastrin-secreting tumor, the gastrinoma. This tumor is often malignant and patients develop metastases in 25% of cases. The usual localizations of gastrinomas are at the head of the pancreas, the duodenal wall and the peripancreatic lymph nodes.

[Endocrine tumor arising in heterotopic gastric pancreas].

We report the case of a 49-year-old caucasian woman, in whom an endocrine tumor arising in gastric heterotopic pancreas was diagnosed. The patient was treated surgically with a gastric wedge resection. Heterotopic pancreas is a benign anatomic condition, probably widely underdiagnosed because usually asymptomatic.

Specificity of basement membrane thickening in severe asthma.

Background: Reticular basement membrane (RBM) thickness is considered a hallmark for airway remodeling in airway diseases such as asthma. It is still unclear whether this measurement could be associated with disease severity or apply to chronic obstructive pulmonary disease (COPD). A wide range of results, at baseline or after therapeutic intervention, have been reported using different measurement methods.

Long-term follow-up of biventricular pacing using a totally endocardial approach in patients with end-stage cardiac failure.

Background: Besides standard left ventricular (LV) stimulation via the coronary sinus, a transseptal approach allows left ventricular endocardial stimulation. We report our long-term observations with biventricular stimulation, using a strictly endocardial system for patients presenting with severe congestive heart failure.

Methods: Six patients with nonischemic cardiomyopathy (mean age = 60 +/- 9.

[Neurosurgical treatment in childhood dystonias and dyskinesias].

Aim: To present the post-surgery clinical results of deep brain stimulation of the internal globus pallidus (GPi) in a group of paediatric patients with primary and secondary dystonic-dyskinetic syndrome.

Patients And Methods: Between November 1996 and May 2006, 121 patients were submitted to interventions with bilateral deep brain stimulation of the GPi, 58 of whom were children (age range: 5-16 years); 35 were cases of primary dystonia, of which 17 were carriers of the DYT1 mutation and 10 were not. A further 8 cases were myoclonic dystonias, 3 of whom presented the DYT11 mutation.

Frequency of retinal cavernomas in 60 patients with familial cerebral cavernomas: a clinical and genetic study.

Objectives: To define the frequency of retinal lesions in a large panel of patients with familial cerebral cavernomas and to screen the cerebral cavernous malformation genes in patients with cerebral and retinal lesions.

Methods: Fundus examination was proposed to each of the index patients of 70 families with cerebral cavernous malformation who have been included in a prospective clinical and neuroradiological follow-up. All of the coding exons of the KRIT1, MGC4607, and PDCD10 genes were screened as previously described.

Evidence for an incomplete mitral isthmus block after failed ablation of a left postero-inferior concealed accessory pathway.

We report the case of a young woman in whom previous ablation of a concealed left-sided accessory pathway (AP) created an iatrogenic mitral block. The mitral block was responsible for a split retrograde atrial activation pattern during orthodromic atrioventricular re-entrant tachycardia (AVRT). The differential diagnoses are discussed.

Monomorphic ventricular tachycardia induced by cardiac resynchronization therapy in patient with severe nonischemic dilated cardiomyopathy.

We report the case of a patient with severe nonischemic dilated cardiomyopathy in whom cardiac resynchronization therapy (CRT) was the source of incessant, drug-resistant, monomorphic ventricular tachycardia (VT). VT recurrences were only resolutive with inactivation of CRT and reactivation of CRT reproduced VT occurrence. The possible pathophysiology of the VT and the potential ventricular proarrhythmic risk related to CRT are discussed.

Postoperative analgesia after total-hip arthroplasty: Comparison of intravenous patient-controlled analgesia with morphine and single injection of femoral nerve or psoas compartment block. a prospective, randomized, double-blind study.

Background: The authors compared the analgesic effects and quality of rehabilitation of three analgesic techniques after total-hip arthroplasty in a double-blind, randomized trial.

Methods: Forty-five patients were assigned to 1 of 3 groups, patient-controlled analgesia with morphine (PCA), femoral nerve block (FNB), or psoas compartment block (PCB). At the end of the procedure performed under general anesthesia, nerve blocks using 2 mg/kg of 0.

Effect of acetazolamide on ocular hemodynamics in pseudotumor cerebri associated with inflammatory bowel disease.

Purpose: To describe the hemodynamic effect of oral acetazolamide administration on ocular perfusion in a patient with pseudotumor cerebri associated with Crohn disease.

Design: Interventional case report.

Methods: A 20-year-old woman with a 5-year history of Crohn disease presented with a 2-week history of headache and blurred vision in both eyes.

The mandibular canal of the "old man" of Cro-Magnon: anatomical-radiological study.

Introduction: The radiological study of the 'old man' of Cro-Magnon mandible complements the one published about the mandibular canal of the "Neanderthal Man" of La Chapelle-aux-Saints with which it is compared.

Objective: The purpose of this study was to explore the internal structure of this famous fossil.

Material And Methods: The mandible of the "old man" of Cro-Magnon (22,000 BC) was studied with panoramic radiography and CT scanning.