Sindrome di Ehlers-Danlos: un raro caso di ischemia intestinale e renale

Caso clinico:Un uomo di 52 anni giunge alla nostra osservazioneper dolore epigastrico irradiato ai fianchi ed alvo chiuso a feci egas da alcuni giorni; ipertrofia prostatica all\u2019anamnesi patologicaremota. Obiettivamente addome trattabile, non dolorabile,peristalsi vivace; PA 180/100 mmhg. Gli esami ematochimicimostravano leucocitosi neutrofila (WBC 17,95), incremento ditroponina I (0,108 ng/ml), D-dimero 1207 (ng/ml), LDH (513U/l), CPK (1102 U/l), AST (96 U/l) e ALT (120 U/l) ed elevatiindici biologici di flogosi (PCR 18,9 mg/dl). La TC addomeevidenziava ectasie, dissezione e microaneurismi di tronco celiaco,aa mesenteriche, arcata pancreatico-duodenale ed ileocolica consegni di ischemia intestinale diffusa e lesioni infartuali renalibilaterali per dissezione di vari tratti delle arterie renali. Presenteinoltre dissezione dell\u2019arteria carotide interna destra extracranicaalla TC vasi epicranici. Negativa risultava la sierologia immunitaria;la ricerca genetica per malattia del collagene confermava ladiagnosi di sindrome di Ehlers-Danlos di tipo IV. Il paziente \ue8 stato trattato con eparina ev, antiipertensivi ev con buona rispostaclinica e laboratoristica. Dimesso e seguito in regimeambulatoriale con discrete condizioni cliniche, decedevaimprovvisamente a sette mesi dalla diagnosiConclusioni:La S. di Ehlers-Danlos \ue8 una malattia ereditariadovuta ad alterazioni del collagene e del tessuto connettivo conquadri clinici eterogenei. La forma vascolare (tipo IV) \ue8 la pi\uf9 gravepotendo determinare rottura di grossi vasi e perforazioni visceralifino all\u2019exitus

CHA2DS2-VASc and HAS-BLED scores do not predict NVAF-related events in a population of critically ill patients

Background: Non-valvular atrial fibrillation(NVAF) is commonamong critically-ill subjects and is associated to worseoutcomes.NVAF-related morbility is associated to thromboembolicand haemorragic complications.Guidelines suggest stratifying thethrombotic risk with CHA2DS2-VASc and the haemorrhagic risk withHAS-BLED. These scores are not validated for the critically-ill, andguidelines are not able to suggest any evidence-based approach.Patients and Methods: Single-cohort, perspective study enrollingall the consecutive patients admitted to our department for a crit-ical illness and affected by NVAF. We excluded patients admittedfor trauma or surgical pathologies. Embolic outcome(TE) was de-fined as the occurrence of embolic manifestations at the admis-sion or during the hospitalization. Haemorragic outcome(MH) wasdefined as the occurrence of major haemorrhage according toISTH criteria during the hospitalization or at the 12-months follow-up. For each patient, we evaluated age, sex, admission diagnosis,comorbidities, CHA2DS2-VASc, HAS-BLED, TE and MH. Results: 519 subjects[age:75.6(\ub111.9);males:50.3%;comorbidi-ties:2(0-6)].38 MH(7,3%),80 TE(15,4%).HAS-BLED: median of2(0-5),CHA2-DS2-VASc:median of 3(0-6);among MH, HAS-BLED:median of 3(1-4), CHA2-DS2-VASc:median of 2(1-3);amongTE,HAS-BLED: median of 2(1-3), CHA2-DS2-VASc:median of 3(1-4);CHA2DS2-VASc had an AUC of 0.56[95%CI:0.50-0:63(p=0.06)] for TE;HAS-BLED had an AUC of 0.53 [95%CI:0.44-0:62(p=0.53)] for MH. Discussion: In this population, CHA2DS2-VASc and HAS-BLEDhad limited predictive value for TE and MH