A case of overlapping primary sclerosing cholangitis and autoimmune hepatitis presented as acute on chronic liver failure

7-14% of patients suffering from primary sclerosing cholangitis (PSC) may present with clinical and serological features, indicating an overlap syndrome with autoimmune hepatitis (AIH). In an otherwise indolent course of a chronic liver disease, an exacerbation can result in acute liver failure (ALF). A 22-year-old man with a history of thrombotic events and verified thrombophilia (PAI-1 mutation) presented with jaundice (bilirubin=211,3 μmol/L) and abdominal discomfort. His synthetic liver function rapidly deteriorated (AST=3084 U/L, ALT=2928 U/L, ALP=154 U/L, GGT=63 U/L). Initial workup excluded viral causes and he was referred to the University Hospital Merkur with the diagnosis of ALF of unknown etiology. Whilst pursuing the underlying cause of the ALF and concomitant anaemia, the diagnosis of Crohn’s disease was established via lower endoscopy. In the following days, the patient’s state severely worsened, including the development of stage 3 hepatic encephalopathy. 7 days after admission, the patient was successfully transplanted. Histological assessment confirmed the diagnosis of overlapping AIH and PSC. The patient was uneventful for 2.5 years, when after the rise of liver enzymes (AST=296 U/L, ALT=682 U/L, GGT=93 U/L, ALP=165 U/L), a liver biopsy confirmed the relapse of AIH. Remission was attained with the administration of corticosteroids. In the 3-year follow-up, the patient was relapse-free without thromboembolic events. This complex case emphasizes the importance of prompt management of ALF and the difficulty of distinguishing acute on chronic liver failure from ALF. Overlapping liver diseases can be accompanied by various conditions, most commonly inflammatory bowel diseases, which makes the treatment particularly challenging

PRECIPITATING FACTORS AND CLINICAL FEATURES OF DIABETIC KETOACIDOSIS

vod: Dijabetička ketoacidoza (DKA) jedna je od najozbiljnijih akutnih komplikacija šećerne bolesti (ŠB). Pojedina istraživanja su pokazala da su infekcije precipitirajući čimbenik u polovice ispitanika. Nekoliko novijih istraživanja naglašava da je loše pridržavanje liječenja također česti uzrok DKA. Cilj: Identifi cirati najčešće precipitirajuće čimbenike za DKA u Republici Hrvatskoj. Ispitanici i postupci: Ovo retrospektivno multicentrično istraživanje uključivalo je bolesnike sa ŠB-om tipa 1 ili tipa 2 s dijagnozom DKA između 1. siječnja 2014. i 31. prosinca 2018. i liječenih u 5 različitih središta za liječenje ŠB-a: Dubrovnik, Našice, Split, Zagreb i Osijek. U analizu je uključena samo prve epizoda DKA. Pacijenti koji boluju od steroidnog ŠB-a i ŠB-a zbog endokrinih poremećaja kao što su akromegalija i Cushingov sindrom bili su isključeni. Rezultati: Istraživanjem je obuhvaćeno 160 bolesnika (55 % muškaraca), od kojih je 68% imalo ŠB tip 1. Srednja dob ispitanika bila je 42 godine (od 18 do 89). Najčešći uzrok DKA bila je infekcija (57 %), zatim slabo kontrolirani ŠB (37 %) i prva prezentacija ŠB-a (9 %), dok je u 7% bolesnika DKA bila uzrokovana ostalim uzrocima kao što su kvar inzulinske pumpe, moždani ili srčani udar. U skupini bolesnika s infekcijama najčešće su bile infekcije mokraćnog sustava (30 %), probavne infekcije (30 %) i infekcije respiratornog trakta (19 %), dok je 21 % bolesnika imalo druge izvore infekcije. U 36 ovih bolesnika uz infekciju je bio prisutan i prethodno loše kontroliran ŠB, a u 12 % DKA uzrokovana infekcijom bila je prvo očitovanje bolesti. U bolesnika sa ŠB-om tipa 2 infekcije su češće bile uzrok DKA nego u bolesnika sa ŠB-om tipa 1 (P < 0,05). U bolesnika sa ŠB-om tipa 1, slabo regulirana glikemija je češće uzrok DKA (31%) nego u bolesnika sa ŠB-om tipa 2 (18 %). Zaključak: Najčešći precipitirajući čimbenici za razvoj DKA su infekcije i loša regulacija ŠB-a. Potrebna je bolja edukacija bolesnika o važnosti redovite primjene inzulina i korekcije terapije tijekom akutne bolesti.Introduction: Diabetic ketoacidosis (DKA) is one of the most serious acute complications of diabetes mellitus (DM). In some studies, infections have been found to be a precipitating factor in more than half of the subjects. On the other hand, several recent studies emphasize that poor treatment adherence is also a common cause of DKA. Objective: To identify the most common precipitating factors for DKA in Croatia. Patients and Methods: This retrospective, multicenter study included DM type 1 or DM type 2 patients diagnosed with DKA between January 1, 2014 and December 31, 2018, and treated in 5 different DM treatment centers, i.e., Dubrovnik, Našice, Split, Zagreb and Osijek. Only the fi rst episode of DKA was included in the analysis. Patients receiving steroids and DM due to endocrine disorders such as acromegaly and Cushing\u27s syndrome were excluded. Results: The study included 160 patients (55% of men), of whom 68% had DM type 1. The mean age of the respondents was 42 (18-89) years. The most common cause of DKA was infection (57%), followed by poorly controlled DM (37%) and fi rst presentation of DM (9%), while in 7% of patients DKA was due to other causes such as insulin pump failure, stroke or myocardial infarction. In the group of patients with infections, urinary tract infections (30%), gastrointestinal infections (30%) and respiratory tract infections (19%) were most common, whereas 21% of patients had other sources of infection. In 36% of these patients, the infection was associated with previously poorly controlled diabetes, and in 12% of them, DKA caused by the infection was the fi rst manifestation of the disease. In patients with type 2DM, infections were more often the cause of DKA than in patients with type 1DM (p<0.05).Poorly controlled glycemia appeared to be a more frequent cause of DKA in patients with type 1 DM (31%) than in patients with type 2 DM (18%). Conclusion: The most common precipitating factors for the development of DKA were infections and poor diabetes management. Better education of patients about the importance of regular insulin administration and correction of therapy in acute illness could reduce the risk of DKA

Analysis of clinical features of adult patients admitted for ketoacidosis at the Emergency Department of the University Hospital Center Zagreb

Dijabetička ketoacidoza (DKA) jedna je od životno ugrožavajućih komplikacija koja se potencijalno javlja u pacijenata oboljelih od dijabetesa melitusa (DM). Cilj ovog istraživanja bio je istražiti i prikazati najčešće simptome i vrijednosti laboratorijskih parametara među šezdeset i jednim pacijentom s DKA primljenih u hitni bolnički prijemu, KBC Zagreb, od kojih je najveći broj oboljelih od dijabetesa tip 1 (n=37; 61%). Tahikardija je bila dominantan klinički znak zabilježen u pacijenata u trenutku prijema (n=38; 62%). Opći simptomi (n=27; 45%), povraćanje (n=27; 45%), pojačano žeđanje (n=26; 43%) i poremećaji svijesti (n=17; 28%) najčešći su simptomi kojima su se ljudi prezentirali u hitnoj službi. U manjeg broja pacijenata (n=9; 15%) s teškom acidozom zamijećeni su znatni elektrolitski poremećaji, osobito razine serumskog kalija, u vidu hiper ili hipokalemije. U 97% ispitanika loše regulirana glikemija identificirana je kao glavni faktor koji predisponira nastanak DKA. Daljnja edukacija i senzibilizacija bolesnika o mogućim akutnim i kroničnim komplikacijama šećerne bolesti te o važnosti adekvatnog i savjesnog uzimanja propisane terapije ključni su elementi u poboljšanju ishoda liječenja i smanjenju broja teških komplikacija.Diabetic ketoacidosis (DKA) is acute and life-threatening complication of diabetes. The aim of this study was to investigate and display most common symptoms and laboratory measurement values among 61 patient with DKA admitted to the Emergency Department of KBC Zagreb, most of whom had type 1 diabetes (n=37; 61%). Tachycardia was the dominant clinical sign noted in the patients following admission (n=38; 62%). General symptoms (n=27; 45%), vomiting (n=27; 45%), excessive thirst (n=26; 43%) and altered mental status (n=17; 28%) were the most common presenting symptoms. In a smaller number of patients (n=9; 15%) with deep acidosis, grave electrolyte disturbances were detected in a form of both hyper and hypokalemia. In 97% of our patients, we've identified bad glycemic regulation as the main factor predisposing the occurence of DKA. Further patient education and consciousness-raising of the potential acute and chronic complications of diabetes as well as the importance of regular and adequate engagement in therapeutic processes are the key elements in the improvement of treatment outcomes and minimizing the number of severe complications

Analysis of clinical features of adult patients admitted for ketoacidosis at the Emergency Department of the University Hospital Center Zagreb

Dijabetička ketoacidoza (DKA) jedna je od životno ugrožavajućih komplikacija koja se potencijalno javlja u pacijenata oboljelih od dijabetesa melitusa (DM). Cilj ovog istraživanja bio je istražiti i prikazati najčešće simptome i vrijednosti laboratorijskih parametara među šezdeset i jednim pacijentom s DKA primljenih u hitni bolnički prijemu, KBC Zagreb, od kojih je najveći broj oboljelih od dijabetesa tip 1 (n=37; 61%). Tahikardija je bila dominantan klinički znak zabilježen u pacijenata u trenutku prijema (n=38; 62%). Opći simptomi (n=27; 45%), povraćanje (n=27; 45%), pojačano žeđanje (n=26; 43%) i poremećaji svijesti (n=17; 28%) najčešći su simptomi kojima su se ljudi prezentirali u hitnoj službi. U manjeg broja pacijenata (n=9; 15%) s teškom acidozom zamijećeni su znatni elektrolitski poremećaji, osobito razine serumskog kalija, u vidu hiper ili hipokalemije. U 97% ispitanika loše regulirana glikemija identificirana je kao glavni faktor koji predisponira nastanak DKA. Daljnja edukacija i senzibilizacija bolesnika o mogućim akutnim i kroničnim komplikacijama šećerne bolesti te o važnosti adekvatnog i savjesnog uzimanja propisane terapije ključni su elementi u poboljšanju ishoda liječenja i smanjenju broja teških komplikacija.Diabetic ketoacidosis (DKA) is acute and life-threatening complication of diabetes. The aim of this study was to investigate and display most common symptoms and laboratory measurement values among 61 patient with DKA admitted to the Emergency Department of KBC Zagreb, most of whom had type 1 diabetes (n=37; 61%). Tachycardia was the dominant clinical sign noted in the patients following admission (n=38; 62%). General symptoms (n=27; 45%), vomiting (n=27; 45%), excessive thirst (n=26; 43%) and altered mental status (n=17; 28%) were the most common presenting symptoms. In a smaller number of patients (n=9; 15%) with deep acidosis, grave electrolyte disturbances were detected in a form of both hyper and hypokalemia. In 97% of our patients, we've identified bad glycemic regulation as the main factor predisposing the occurence of DKA. Further patient education and consciousness-raising of the potential acute and chronic complications of diabetes as well as the importance of regular and adequate engagement in therapeutic processes are the key elements in the improvement of treatment outcomes and minimizing the number of severe complications

Analysis of clinical features of adult patients admitted for ketoacidosis at the Emergency Department of the University Hospital Center Zagreb

Dijabetička ketoacidoza (DKA) jedna je od životno ugrožavajućih komplikacija koja se potencijalno javlja u pacijenata oboljelih od dijabetesa melitusa (DM). Cilj ovog istraživanja bio je istražiti i prikazati najčešće simptome i vrijednosti laboratorijskih parametara među šezdeset i jednim pacijentom s DKA primljenih u hitni bolnički prijemu, KBC Zagreb, od kojih je najveći broj oboljelih od dijabetesa tip 1 (n=37; 61%). Tahikardija je bila dominantan klinički znak zabilježen u pacijenata u trenutku prijema (n=38; 62%). Opći simptomi (n=27; 45%), povraćanje (n=27; 45%), pojačano žeđanje (n=26; 43%) i poremećaji svijesti (n=17; 28%) najčešći su simptomi kojima su se ljudi prezentirali u hitnoj službi. U manjeg broja pacijenata (n=9; 15%) s teškom acidozom zamijećeni su znatni elektrolitski poremećaji, osobito razine serumskog kalija, u vidu hiper ili hipokalemije. U 97% ispitanika loše regulirana glikemija identificirana je kao glavni faktor koji predisponira nastanak DKA. Daljnja edukacija i senzibilizacija bolesnika o mogućim akutnim i kroničnim komplikacijama šećerne bolesti te o važnosti adekvatnog i savjesnog uzimanja propisane terapije ključni su elementi u poboljšanju ishoda liječenja i smanjenju broja teških komplikacija.Diabetic ketoacidosis (DKA) is acute and life-threatening complication of diabetes. The aim of this study was to investigate and display most common symptoms and laboratory measurement values among 61 patient with DKA admitted to the Emergency Department of KBC Zagreb, most of whom had type 1 diabetes (n=37; 61%). Tachycardia was the dominant clinical sign noted in the patients following admission (n=38; 62%). General symptoms (n=27; 45%), vomiting (n=27; 45%), excessive thirst (n=26; 43%) and altered mental status (n=17; 28%) were the most common presenting symptoms. In a smaller number of patients (n=9; 15%) with deep acidosis, grave electrolyte disturbances were detected in a form of both hyper and hypokalemia. In 97% of our patients, we've identified bad glycemic regulation as the main factor predisposing the occurence of DKA. Further patient education and consciousness-raising of the potential acute and chronic complications of diabetes as well as the importance of regular and adequate engagement in therapeutic processes are the key elements in the improvement of treatment outcomes and minimizing the number of severe complications