Significant benefits of AIP testing and clinical screening in familial isolated and young-onset pituitary tumors

Context Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are responsible for a subset of familial isolated pituitary adenoma (FIPA) cases and sporadic pituitary neuroendocrine tumors (PitNETs). Objective To compare prospectively diagnosed AIP mutation-positive (AIPmut) PitNET patients with clinically presenting patients and to compare the clinical characteristics of AIPmut and AIPneg PitNET patients. Design 12-year prospective, observational study. Participants & Setting We studied probands and family members of FIPA kindreds and sporadic patients with disease onset ≤18 years or macroadenomas with onset ≤30 years (n = 1477). This was a collaborative study conducted at referral centers for pituitary diseases. Interventions & Outcome AIP testing and clinical screening for pituitary disease. Comparison of characteristics of prospectively diagnosed (n = 22) vs clinically presenting AIPmut PitNET patients (n = 145), and AIPmut (n = 167) vs AIPneg PitNET patients (n = 1310). Results Prospectively diagnosed AIPmut PitNET patients had smaller lesions with less suprasellar extension or cavernous sinus invasion and required fewer treatments with fewer operations and no radiotherapy compared with clinically presenting cases; there were fewer cases with active disease and hypopituitarism at last follow-up. When comparing AIPmut and AIPneg cases, AIPmut patients were more often males, younger, more often had GH excess, pituitary apoplexy, suprasellar extension, and more patients required multimodal therapy, including radiotherapy. AIPmut patients (n = 136) with GH excess were taller than AIPneg counterparts (n = 650). Conclusions Prospectively diagnosed AIPmut patients show better outcomes than clinically presenting cases, demonstrating the benefits of genetic and clinical screening. AIP-related pituitary disease has a wide spectrum ranging from aggressively growing lesions to stable or indolent disease course

Acides naphténiques hydrates de gaz (influence de l'interface eau/huile sur les propriétés dispersantes d'un brut acide.)

Actuellement, les compagnies pétrolières sont amenées à exploiter des gisements de bruts situés à des profondeurs sous-marines de plus en plus importantes. Dans ces conditions de production (hautes pressions et basses températures), se pose le problème du bouchage des conduites pétrolières par les hydrates de gaz, composés cristallins constitués d'eau et de gaz. Il a été montré que certains bruts asphalténiques forment des émulsions eau dans huile (E/H) stables sur plusieurs mois et évitent la formation des bouchons d'hydrate. Dans le cadre de cette thèse, les propriétés "anti-hydrates" d'un brut acide AH provenant d'Afrique de l'Ouest sont étudiées. Ce brut comporte des acides naphténiques, composés hydrocarbures de formule brute RCOOH réactifs au pH et à la salinité de la phase aqueuse. Dans un premier temps, les propriétés émulsifiantes du brut AH ont été explorés. Il a été montré que la stabilité des émulsions E/H avec ce brut provient principalement des résines lourdes et des asphaltènes. Les naphténates RCOO-, forme basique des acides naphténiques, diminuent la stabilité des émulsions E/H. L'étude du brut AH vis à vis des hydrates de gaz a montré que ce brut présente des propriétés anti-agglomérantes modérées grâce aux résines lourdes et aux asphaltènes. Par contre, les naphténates RCOO- favorisent la formation des bouchons d'hydrate. D'autre part, il a été relevé que l'agglomération des particules d'hydrate accélère la cinétique de formation des hydrates et favorise le cassage des émulsions E/H. Un mécanisme d'agglomération par contact particule/gouttelette est proposé pour interpréter ces comportements. Enfin, nous proposons un modèle des équilibres physico-chimiques des acides naphténiques dans le mélange binaire eau/brut AH, afin de transposer les résultats obtenus en laboratoire aux conditions réelles.Nowadays, the development of offshore oil production under increasing water depths (high pressures and low temperatures) has led oil companies to focus on gas hydrates risks. Hydrates are crystals containing gas and water molecules which can plug offshore pipelines. It has been shown that some asphaltenic crude oils stabilise water-in-oil emulsions (W/O) during several months and exhibit very good anti-agglomerant properties avoiding hydrate plugs formation. In this work, we have studied the anti-hydraté properties of a West African acidic crude oil called crude AH. This oil contains naphthenic acids, RCOOH hydrocarbons which are sensitive to both the pH and the salinity of the water phase. The emulsifying properties of the crude AH have firstly been explored. It has been shown that heavy resins and asphaltenes are the main compounds of the crude AH responsible for the long term stability of the W/O emulsions whereas the napthenates RCOO- lead to less stable W/O emulsions. Dealing with hydrates, the crude AH exhibits moderate anti-agglomerant properties due to the presence of heavy resins and asphaltenes. However, the naphthenates RCOO- drastically increase the formation of hydrate plugs. Moreover, it has been pointed out that hydrate particles agglomeration accelerates the kinetics of hydrate formation and enhances the water/oil separation. In order to explain these behaviours, a mechanism of agglomeration by "sticking" between a hydrate particle and a water droplet has been proposed. Finally, we have developed a model which describes the physico-chemical equilibria of the naphthenic acids in the binary system water/crude AH, in order to transpose the results obtained in the laboratory to the real oilfield conditions.PAU-BU Sciences (644452103) / SudocSudocFranceF