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Background Acute kidney injury (AKI) is a common condition that is associated with poor short- and long-term outcomes. The aim of this nationwide cohort study was to profile the long-term outcome of patients admitted for AKI in France. Methods Based on the comprehensive French hospital discharge database, all hospitalizations for an AKI episode were categorized in four groups according to the presence of at least one dialysis session (RRT) and according to the coding of AKI as the principal or associated diagnosis (PRINC_DIAG or ASS_DIAG). The cumulative incidences of death and ESRD in each group were analyzed with a subdistribution hazard (Fine and Gray) model to take into account the competing risks between those two outcomes. A subgroup analysis was done for patients who were alive and not on RRT at hospital discharge. The effect of the initial clinical feature on death or ESRD was analyzed with an adjusted cause-specific Cox proportional hazard regression censored at other outcomes. All the models were adjusted for age, gender and comorbidities. Results In this nationwide cohort of 989,974 patients (median age 77 years) hospitalized with AKI during the 2009–2016 period, 422,739 (43%) patients died (235,572 during the first hospitalization) and 40,015 (4%) patients reached ESRD (5962 during first hospitalization) up to 31 December 2016. Former cardiovascular disease and CKD were diagnosed in 40% and 16% of patients, respectively. Patients without RRT and discharged from hospital had a cumulative incidence of ESRD which ranged from 5.3% [5.2–5.4] in the ASS_DIAG group to 28.7% [27.9–29.5] in the RRT-PRINC_DIAG group at 60 months. The cumulative incidence of death ranged from 31.0% [30.2–32.2] in the RRT-ASS_DIAG group to 45.5% [45.3–45.7] in the ASS_DIAG group. Initial clinical features were associated with outcome independent of comorbidities and age. Compared to RRT-PRINC_DIAG, PRINC_DIAG (HR: 0.4, 95% CI: 0.4–0.4), ASS_DIAG patients (HR: 0.1, 95% CI: 0.1–0.2) and RRT-ASS_DIAG (HR: 0.4, 95% CI: 0.4–0.5) patients were at lower risk of reaching ESRD. Results were similar in each age group and whether or not patients had a previous diagnosis related to urinary tract or kidney disease. RRT-ASS_DIAG patients had a higher risk of death (HR: 1.9, 95% CI: 1.9–2.0) as compared to RRT-PRINC_DIAG, while patients with AKI as principal diagnosis (PRINC_DIAG) or associated diagnosis (ASS_DIAG) not requiring dialysis had a lower risk (HR: 0.7, 95% CI: 0.6–0.7 and HR: 0.9, 95% CI: 0.9–0.9, respectively). Conclusions The major strength of our observational study was the national coverage obtained from the comprehensive French hospital discharge database and a long follow-up over seven years. Our study strengthens the current recommendations for long-term follow-up of patients with AKI. The novelty of this study is to propose a clinical classification of AKI episodes that is easy to detect in administrative medical databases and that is strongly associated with immediate and long-term outcomes. This novel classification is based on the perceived primacy of AKI as a driver of the illness that brought the patient to the hospital and the need of RRT. Further studies are now warranted to compare the performance of our classification to predict long-term outcome to other classifications, including the KDIGO, AKIN or RIFLE.

The patient, 56-year-old women had a 6 months history of light-chain (AL) amyloidosis with renal and cardiac involvement associated with IgG mutiple myeloma. AL amyloidosis was treated with Velcade–Cyclophosphamide–Dexamethasone and cardiac transplantation for a mayo clinic stage III amyloid cardiomyopathy 1 month ago. She …

ContextSeptic shock may be associated with relative adrenal insufficiency. Thus, a replacement therapy of low doses of corticosteroids has been proposed to treat septic shock.ObjectiveTo assess whether low doses of corticosteroids improve 28-day survival in patients with septic shock and relative adrenal insufficiency.Design and SettingPlacebo-controlled, randomized, double-blind, parallel-group trial performed in 19 intensive care units in France from October 9, 1995, to February 23, 1999.PatientsThree hundred adult patients who fulfilled usual criteria for septic shock were enrolled after undergoing a short corticotropin test.InterventionPatients were randomly assigned to receive either hydrocortisone (50-mg intravenous bolus every 6 hours) and fludrocortisone (50-µg tablet once daily) (n = 151) or matching placebos (n = 149) for 7 days.Main Outcome MeasureTwenty-eight-day survival distribution in patients with relative adrenal insufficiency (nonresponders to the corticotropin test).ResultsOne patient from the corticosteroid group was excluded from analyses because of consent withdrawal. There were 229 nonresponders to the corticotropin test (placebo, 115; corticosteroids, 114) and 70 responders to the corticotropin test (placebo, 34; corticosteroids, 36). In nonresponders, there were 73 deaths (63%) in the placebo group and 60 deaths (53%) in the corticosteroid group (hazard ratio, 0.67; 95% confidence interval, 0.47-0.95; P = .02). Vasopressor therapy was withdrawn within 28 days in 46 patients (40%) in the placebo group and in 65 patients (57%) in the corticosteroid group (hazard ratio, 1.91; 95% confidence interval, 1.29-2.84; P = .001). There was no significant difference between groups in responders. Adverse events rates were similar in the 2 groups.ConclusionIn our trial, a 7-day treatment with low doses of hydrocortisone and fludrocortisone significantly reduced the risk of death in patients with septic shock and relative adrenal insufficiency without increasing adverse events.

But Environ 5 % des cancers du pancreas (CaPa) sont familiaux (CaPaFa). Deux principaux genes de predisposition ont ete identifies : CDKN2A , implique egalement dans le melanome familial, et BRCA2 principalement dans la predisposition hereditaire au cancer du sein. La gravite de cette tumeur justifie un depistage precoce chez les sujets a haut risque. Buts de l’etude evaluer prospectivement a) la prevalence des anomalies de CDKN2A , CDK4 et BRCA2 dans une serie francaise de familles CaPaFa (≥ 2 sujets atteints de CaPa apparentes au 1 er ou 2 eme degre) ; b) l’interet du depistage par l’imagerie (TDM, IRM +/− echoendoscopie) chez les apparentes non atteints. Patients et Methodes 24 familles atteintes de CaPa (21 familles avec 2 CaPa, 3 familles avec 3 CaPa) ont eu une consultation d’oncogenetique dediee. Un prelevement genetique (apres recueil d’un consentement eclaire) etait realise dans les 14 familles pour lesquelles un cas index etait encore vivant. Les 4 exons du gene CDKN2A (1α,1β, 2 et 3) et l’exon 2 du gene CDK4 ont ete sequences dans 13 cas. De plus, une recherche de deletion de CDKN2A a ete realisee par MLPA dans 12 cas. Une recherche de mutation et de grand rearrangement du gene BRCA2 a ete realisee dans 10 cas. Pour les 10 familles dont les membres CaPa etaient decedes, seul un depistage par imagerie (scanographie, IRM +/− echoendoscopie) etait propose. Resultats Des anomalies genetiques ont ete trouvees chez deux proposants sur 14 : 1) un variant non-synonyme de CDKN2A c.430 C > T, p.R144C (famille avec 2 pts CaPa). Ce variant, situe dans le 4 eme domaine ankyrine de la proteine, etait absent de 200 ADN controles et predit affecter la fonction de la proteine (programmes Sift et Polyphen). L’analyse de segregation est en cours ; 2) une mutation non sens de BRCA2 c.373G > T (p.Glu49X) (famille avec 2 CaPa + 1 apparentee 1 er degre avec cancer du sein), qui avait deja ete decrite au prealable dans une famille predisposee au cancer du sein. Il n’y avait aucun cas de deletion CDKN2A ni de mutation CDK4 . Parmi les 9 apparentes de 6 familles ayant deja eu un depistage par imagerie, 4 avaient lesions kystiques des canaux 2 aires ; deux ont ete operes et avaient une TIPMP en dysplasie de bas grade. Conclusion 1) des anomalies des genes CDKN2A ou BRCA2 ont ete trouvees dans 20 % des familles, soulignant l’importance de la consultation d’oncogenetique dans les rares cas de CaPaFa ; 2) des lesions precancereuses de type TIPMP peuvent etre depistees chez les apparentes. Remerciements, financements, autres Remerciements au Club Francais du Pancreas.