Congenital hyperinsulinism: current trends in diagnosis and therapy

Congenital hyperinsulinism (HI) is an inappropriate insulin secretion by the pancreatic β-cells secondary to various genetic disorders. The incidence is estimated at 1/50, 000 live births, but it may be as high as 1/2, 500 in countries with substantial consanguinity. Recurrent episodes of hyperinsulinemic hypoglycemia may expose to high risk of brain damage. Hypoglycemias are diagnosed because of seizures, a faint, or any other neurological symptom, in the neonatal period or later, usually within the first two years of life. After the neonatal period, the patient can present the typical clinical features of a hypoglycemia: pallor, sweat and tachycardia. HI is a heterogeneous disorder with two main clinically indistinguishable histopathological lesions: diffuse and focal. Atypical lesions are under characterization. Recessive ABCC8 mutations (encoding SUR1, subunit of a potassium channel) and, more rarely, recessive KCNJ11 (encoding Kir6.2, subunit of the same potassium channel) mutations, are responsible for most severe diazoxide-unresponsive HI. Focal HI, also diazoxide-unresponsive, is due to the combination of a paternally-inherited ABCC8 or KCNJ11 mutation and a paternal isodisomy of the 11p15 region, which is specific to the islets cells within the focal lesion. Genetics and 18F-fluoro-L-DOPA positron emission tomography (PET) help to diagnose diffuse or focal forms of HI. Hypoglycemias must be rapidly and intensively treated to prevent severe and irreversible brain damage. This includes a glucose load and/or a glucagon injection, at the time of hypoglycemia, to correct it. Then a treatment to prevent the recurrence of hypoglycemia must be set, which may include frequent and glucose-enriched feeding, diazoxide and octreotide. When medical and dietary therapies are ineffective, or when a focal HI is suspected, surgical treatment is required. Focal HI may be definitively cured when the partial pancreatectomy removes the whole lesion. By contrast, the long-term outcome of diffuse HI after subtotal pancreatectomy is characterized by a high risk of diabetes, but the time of its onset is hardly predictable

The surgical management of atypical forms of congenital hyperinsulinism

Beyond the 2 classical forms of congenital hyperinsulinism, focal and diffuse, we report our experience on the surgical treatment of atypical forms. We define 2 subtypes among these atypical forms of hyperinsulinism: in case of a giant focal form the surgical strategy is the same as in focal forms. In case of hyperinsulinism caused by a mosaic, our experience suggests the benefit of a limited resection from the tail to the body of the pancreas. © 2011 Elsevier Inc

La recherche et la filière vigne et vin travaillent conjointement pour relever le défi du changement climatique

International audienc

Morphological mosaicism of the pancreatic islets: a novel anatomopathological form of persistent hyperinsulinemic hypoglycemia of infancy.

Morphological studies of the pancreas in persistent hyperinsulinemic hypoglycemia of infancy (PHHI) have focused on the diagnosis of focal vs. diffuse forms, a distinction that determines the optimal surgical management. ABCC8 or KCNJ11 genomic mutations are present in most of them

Using foresight exercise to design adaptation policy to climate change : the case of the French wine industry

Oral presentation. Colloque SFER "Politiques agricoles et alimentaires : trajectoires et réformes", 2018 Jun. 20-21, Montpellier, France

Les propositions de la profession au ministre pour se préparer au changement climatique : quels apports de la recherche à la stratégie présentée par la profession ?

National audienc

Travaux de prospective sur l'adaptation de la viticulture au changement climatique : quelles séries d'événements pourraient favoriser différentes stratégies d'adaptation ?

A multidisciplinary group of researchers and experts of public bodies working on the French wine sector met together to elaborate a foresight exercice with the construction of the plausible ways leading to four different adaptation strategies to climate change of the French wine sector to climate change. For the implementation of this work, the group, considered a single climatic scenario and difined four strategies of adaptation incorporating both geographical mobility and of integration of technological changes, used assumptions from three sources: assumptions from researchers of various disciplines in their own disciplinary field, pre-existing assumptions from former foresight works and assumptions defined according to the statements of actors of the sector on the impacts of climate change and on the possible methods of adaptation. All these assumptions were written in the form of simple sentences, giving one version and its contrary. The various assumptions were selected and then combined between them in order to create a blend within the disciplinary fields, without however inferring of orientation. The result of this work is the join-construction of four plausible ways each one leading to one of the four preselected strategies of adaptation: "conservative" strategy where the evolutions are limited, a "nomade" strategy where the relocalisation of the vineyards is the main vector of adaptation, an “innovating” strategy where the adaptation is the result of the systematic integration of innovations, and finally towards a "liberal" strategy largely opened to any kind of changes. These ways and strategies are not supposed to give a predictive image of the future. However while proposing possible or plausible futures, this work provides material which could be debated within the sector, without immediate challenges that could possibly bias the discussion From the expression of the challenges and consequences of the various ways and strategies in which they result, the actors of the French wine sector will be able to start a strategic reflection. The presentation will be focused on the description of the four ways leading to adaptation strategies and their elaboration. Methodology is specified in another article "Adaptation to the climate change: interest of a prospective approach", same authors