Real-World Outcomes after 36-Month Treatment with Ranibizumab 0.5 mg in Patients with Visual Impairment due to Diabetic Macular Edema (BOREAL-DME).

Purpose: To assess the efficacy, safety, and follow-up of 36-month treatment with ranibizumab in patients with diabetic macular edema (DME) in real-life setting.

Methods: This is a prospective phase 4 observational study. Between December 2013 and April 2015, 84 ophthalmologists enrolled a total of 290 adult patients initiating ranibizumab for visual impairment due to DME and treated them according to their routine practice.

Liver adenomatosis in patients with hepatocyte nuclear factor-1 alpha maturity onset diabetes of the young (HNF1A-MODY): Clinical, radiological and pathological characteristics in a French series.

Background: Liver adenomatosis (LA) is a rare disease resulting from biallelic inactivation of the hepatocyte nuclear factor-1 alpha (HNF1A) gene, which induces the proliferation of adenoma cells in liver parenchyma. Liver adenomatosis has only been documented in case reports from patients carrying a HNF1A germline mutation. We have evaluated the frequency of LA among a large cohort of patients with HNF1A-maturity onset diabetes of the young (MODY), previously termed "MODY3," and herein describe its clinical, radiological, and pathological characteristics.

Impact of pain on cognitive functions in primary Sjögren syndrome with small fiber neuropathy: 10 cases and a literature review.

Primary Sjögren syndrome (pSS) is a chronic systemic autoimmune disease characterized by xerophthalmia, xerostomia, and potential peripheral or central neurological involvement. In pSS, the prevalence of cognitive disorders is generally sparse across literature and the impact of pain on cognitive profile is unclear. The aim of this study was to determine the relation between pain, cognitive complaint, and impairment in a very homogenous population of 10 pSS patients with painful small fiber neuropathy (PSFN) and spontaneous cognitive complaint.

Activation of the receptor for advanced glycation end products and consequences on health.

Advanced glycation end products (AGE) resulted from a reaction between free amino group of proteins and carbohydrates. This reaction is followed by oxidation and molecular rearrangement. Alternatively AGEs can be produced by glycolysis and oxidation.

Influence of pulse pressure and spontaneous variations of macular thickness in patients with diabetic macular oedema.

Purpose: To study spontaneous variations of central macular thickness (CMT) and its relation to blood pressure (BP) in patients with diabetic macular oedema (DME).

Methods: 23 diabetic patients presenting with DME with a CMT ≥ 260 μm on optical coherence tomography (OCT-3, Carl Zeiss Meditec, CA) were followed every 2 weeks for 3 months. At baseline, ambulatory 24H-BP monitoring (ABPM) was performed, as well as five CMT measurements (9 am, 12 am, 3 pm, 6 pm and 9 am the day after).

Sjögren Syndrome-Associated Small Fiber Neuropathy: Characterization From a Prospective Series of 40 Cases.

We conducted the current study to analyze the clinical, immunologic, and neurophysiologic features of primary Sjögren syndrome (pSS)-associated sensory small fiber neuropathies (SFNs). Forty consecutive pSS patients with SFN were included. SFN was defined by the presence of suggestive sensory painful symptoms with normal nerve conduction studies and abnormal neurophysiologic tests for small nerve fibers or a low intraepidermal nerve fiber density at skin biopsy.

The clinical spectrum of RET proto-oncogene mutations in codon 790.

Objective: Due to a strong genotype-phenotype correlation, the timing of prophylactic thyroidectomy in rearranged during transfection (RET) gene mutation carriers is usually dictated by genetic analysis.

Subjects And Methods: We report a nationwide retrospective study of the clinical data of 77 French patients from 19 families with a mutation in codon 790 of the RET proto-oncogene.

Results: The average age at diagnosis was 35.

A novel ALMS1 splice mutation in a non-obese juvenile-onset insulin-dependent syndromic diabetic patient.

Insulin-dependent juvenile-onset diabetes may occur in the context of rare syndromic presentations suggesting monogenic inheritance rather than common multifactorial autoimmune type 1 diabetes. Here, we report the case of a Lebanese patient diagnosed with juvenile-onset insulin-dependent diabetes presenting ketoacidosis, early-onset retinopathy with optic atrophy, hearing loss, diabetes insipidus, epilepsy, and normal weight and stature, who later developed insulin resistance. Despite similarities with Wolfram syndrome, we excluded the WFS1 gene as responsible for this disease.

Electrocardiographic abnormalities in centenarians and octogenarians: a case-matched study.

Background: Centenarians have been proposed as a model of successful aging but recent studies suggest a high prevalence of cardiovascular diseases. Some findings on their electrocardiograms (ECGs) are simply age-related and others mirror underlying diseases. We aimed to identify ECG features truly associated with extreme age.

Ephrin-B2-activated peripheral blood mononuclear cells from diabetic patients restore diabetes-induced impairment of postischemic neovascularization.

We hypothesized that in vitro treatment of peripheral blood mononuclear cells (PB-MNCs) from diabetic patients with ephrin-B2/Fc (EFNB2) improves their proangiogenic therapeutic potential in diabetic ischemic experimental models. Diabetes was induced in nude athymic mice by streptozotocin injections. At 9 weeks after hyperglycemia, 10(5) PB-MNCs from diabetic patients, pretreated by EFNB2, were intravenously injected in diabetic mice with hindlimb ischemia.

Immunocytochemistry with cytokeratin 19 and anti-human mesothelial cell antibody (HBME1) increases the diagnostic accuracy of thyroid fine-needle aspirations: preliminary report of 150 liquid-based fine-needle aspirations with histological control.

Background: Thyroid nodules are relatively common (7% of the population) but are malignant in only 5%-10% of cases. Fine-needle aspiration (FNA) to detect cancer can have > 90% sensitivity but only 50%-65% specificity because of false-positive results, which necessitates surgical controls. We aimed to assess the diagnostic accuracy of immunocytochemistry (ICC) of thyroid FNA to improve its sensitivity and specificity.

Role of prophylactic thyroidectomy in RET 790 familial medullary thyroid carcinoma.

Background: We describe a family harboring RET 790 mutation and review the role of prophylactic thyroidectomy for medullary thyroid carcinoma.

Methods: We evaluated in detail both clinical and biological follow-up and reviewed literature reports.

Results: Among 86 family members, 15 of 22 members screened harbored the 790 mutation.

Evaluation of retinal function and flicker light-induced retinal vascular response in normotensive patients with diabetes without retinopathy.

Purpose: To correlate retinal function with vascular response to flicker light in normotensive patients with diabetes without diabetic retinopathy (DR).

Methods: Twenty-eight normotensive patients with diabetes (11 with type 1, 17 with type 2) without DR and 28 sex- and age-matched healthy control subjects underwent color vision and contrast sensitivity testing, pattern, full-field, and multifocal electroretinography, and evaluation of the vascular response to flicker light with the dynamic vessel analyzer.

Results: In the patients with diabetes, electroretinogram (ERG) pattern responses, b-wave in the scotopic bright flash ERG, a-wave and b-wave in the photopic single-flash ERG, and oscillatory potential responses were significantly impaired compared with those in control subjects.

[Biological procedures, civil and military health facilities, and recommendations of good practice and hospital reform: the example of diabetes management].

In the context of implementing hospital reforms, the objective of this work was to compare practice in relation to evidence-based guidelines and recommendations for good practice in diabetes screening and management. Laboratory test consumption was determined for patients hospitalized for diabetes in 2005 in three public hospitals (one civilian, two military) taking care of diabetic patients and performing related biological tests. For the 395 admissions in these three hospitals during 2005 [Diagnosis-related group (DRG) 10M02V "Diabetes, age 36 to 69 years without co-morbidity"], the average length of stay and the number of biological acts ["B"] performed were lower than those given by the French national health cost study scale and by the Montpellier University Hospital database.

Insulin gene mutations resulting in early-onset diabetes: marked differences in clinical presentation, metabolic status, and pathogenic effect through endoplasmic reticulum retention.

Objective: Heterozygous mutations in the human preproinsulin (INS) gene are a cause of nonsyndromic neonatal or early-infancy diabetes. Here, we sought to identify INS mutations associated with maturity-onset diabetes of the young (MODY) or nonautoimmune diabetes in mid-adult life, and to explore the molecular mechanisms involved.

Research Design And Methods: The INS gene was sequenced in 16 French probands with unexplained MODY, 95 patients with nonautoimmune early-onset diabetes (diagnosed at <35 years) and 292 normoglycemic control subjects of French origin.

Blood pressure normalization by fixed perindopril/indapamide combination in hypertensive patients with or without associate metabolic syndrome: results of the OPTIMAX 2 study.

The aim of the observational pharmaco-epidemiological study Optimax II was to seek whether the pre-existence of a metabolic syndrome (MS) defined by the NCEP-ATP III criteria impacts blood pressure (BP) control in hypertensive patients receiving a fixed perindopril/indapamide combination therapy. The primary objective of the study was to compare in patients with and without MS the rate of BP control defined as a systolic BP < or = 140 mmHg and a diastolic BP < or = 90 mmHg. Patients were prospectively included and the follow-up lasted 6 months.

A possible hypophosphatemia-induced, life-threatening encephalopathy in diabetic ketoacidosis: a case report.

Hypophosphatemia, a common metabolic disorder, is usually silent and diagnosed by blood tests. However, misdiagnosis may result in delayed phosphate repletion, responsible for significant morbidity and potential mortality. We report an exceptional case of hypophosphatemia-related, life-threatening encephalopathy.

[Drug treatment in type 2 diabetes (part 2)].

Insulin secretagogues and insulin sensitizers can be combined with one another as well as with other treatments (described below). Alpha-glucosidase inhibitors delay intestinal absorption of carbohydrates and reduce postprandial glycemia. Orlistat and sibutramine improve insulin sensitivity by helping patients lose weight.

[Drug treatment of type 2 diabetes].

Drug treatment of 2 diabetes is intended to normalize glycosylated hemoglobin levels (HbA(1c)<6.5%) and thereby prevent the development of micro- and macrovascular complications. Oral antidiabetic agents target the metabolic abnormalities that cause diabetes.