Hepatomegalija i povišene aminotransferaze u bolesnice s loše reguliranom šećernom bolešću

Elevated liver function tests, right upper quadrant abdominal pain, and hepatomegaly occurring in a diabetic patient treated with high doses of insulin may point to the presence of pathologic glycogen accumulation in the liver parenchymal cells. The condition was first described in children, however, studies performed in adults have shown a condition that is similar in many aspects. A case is presented of a 41-year-old female diabetic patient with abnormal liver tests and hepatomegaly. Abdominal ultrasound confirmed liver enlargement without any signs of fatty liver. Liver biopsy revealed a picture compatible with glycogenosis. As excessive hepatic glycogen deposition occurred at an adult age and without a related family history, while the patient presented with normal mental and motor development, the diagnoses of Mauriac syndrome and hereditary were ruled out. The condition was attributed to insulin hyperdosage. The patient was recommended improved glycemic control, more appropriate diet and physical exercise. On follow-up visit 3 months of discharge from the hospital, significant hepatomegaly regression was demonstrated by palpation and ultrasonography, and was accompanied by normalization of serum aminotransferases, blood glucose and glycosylated hemoglobin. Elevated serum aminotransferases and alkaline phosphatase with hepatomegaly as a consequence of hepatocellular glycogen accumulation can occur in diabetic patients of any age who are treated with high doses of insulin, and should therefore be included in the differential diagnosis.Pojava povišenih jetrenih enzima, bolova u desnom gornjem abdominalnom kvadrantu i hepatomegalije u bolesnika sa šećernom bolešću liječenih visokim dozama inzulina može ukazivati na patološko nakupljanje glikogena u stanicama jetrenog parenhima. Ovo je stanje prvotno opisano u djece, a kasnije studije provedene na odraslima pokazale su jednaku kliničku sliku. Prikazan je slučaj 41-godišnje žene oboljele od šećerne bolesti s poremećenim jetrenim nalazima i hepatomegalijom, kod koje je ultrazvuk potvrdio povećanje jetre bez znakova steatoze, a biopsijom je postavljena dijagnoza glikogenoze. S obzirom na to da je izrazito nakupljanje glikogena u ovom slučaju nastupilo u odrasloj dobi, bez pozitivne obiteljske anamneze, a bolesnica je imala uredan psihički i tjelesni razvoj, iz diferencijalne dijagnoze se je mogao isključiti Mauriacov sindrom i nasljedna glikogenoza. Uzrok ovoga stanja u ove bolesnice bilo je liječenje osnovne bolesti visokim dozama inzulina. Stoga joj je preporučena poboljšana kontrola glikemije praćena ispravnom dijetom i tjelesnom aktivnošću. Na kontrolnom pregledu nakon tri mjeseca došlo je do regresije hepatomegalije potvrđene palpacijom i ultrazvukom, te do normaliziranja serumskih aminotransferaza, glukoze u krvi i glikoziliranog hemoglobina. Povišene aminotransferaze i alkalna fosfataza uz hepatomegaliju mogu se naći kod bolesnika dijabetičara liječenih visokim dozama inzulina, kao posljedica nakupljanja glikogena u jetrenim stanicama, pa ovo stanje treba uključiti u diferencijalnu dijagnozu

The Role of Endoscopic Ultrasound in Evaluation of Gastric Subepithelial Lesions

A subepithelial mass is a common finding during endoscopic procedures. Endoscopic ultrasound (EUS) is an important diagnostic modality in the evaluation of subepithelial lesions of the gastrointestinal tract. EUS is the diagnostic test of choice to assess the size, margins, the layer of origin, echotexture, and to differentiate between an intramural and extramural lesion. However, the EUS imaging lacks the specificity. EUS-guided fine needle aspiration (EUS-FNA) or core biopsy can help establish a tissue diagnosis and potentially characterize malignant risk. The aim of this article is to review the diagnosis and management of the most common subepithelial gastric lesions with an emphasis on the role of endoscopic ultrasound

Hepatitis C Virus (HCV) Treatment in Croatia: Recent Advances and Ongoing Obstacles

The prevalence of hepatitis C virus (HCV) antibodies in Croatia is low in the general population (reported <1%), similar to the prevalence rates of many European countries, but is higher in the populations at risk, especially among intravenous drug users. With the development of new classes of direct-acting antiviral agents and interferon-free regimens, the landscape of HCV treatment has completely changed. Management of HCV infection in Croatia is in accordance with the European Association for the Study of the Liver (EASL) recommendations published in 2015, recently updated Croatian Guidelines (published in April 2016) and the recommendations of Croatian Health Insurance Fund (HZZO) which covers the costs of treatment. HZZO approved simeprevir at the beginning of 2015. By the end of the 2015 sofosbuvir, combination of sofosbuvir + ledipasvir and the combination of ombitasvir, paritaprevir and ritonavir ± dasabuvir became available. Although the drawback of these new highly effective treatments is their price, prioritization of patients on a national level offers equal opportunities to patients in need for treatment. Due to improvements in therapy and prevention, clinical care for patients with HCV in Croatia advanced significantly during the last two years

Complications of Liver Cirrhosis

Ciroza jetre, zadnji stadij kronične jetrene bolesti, ima za posljedicu niz patofi zioloških poremećaja i kliničkih manifestacija, koje se kod pojedinog bolesnika javljaju samostalno ili u različitim kombinacijama. U tekstu su detaljno opisane patofi ziologija, dijagnostika i posljednje smjernice za liječenje portalne hipertenzije i krvarenja iz varikoziteta jednjaka te ascitesa, spontanoga bakterijskog peritonitisa, portalne encefalopatije, hepatorenalnog sindroma, hepatopulmonalnog sindroma i hepatocelularnog karcinoma.Cirrhosis, the end stage of chronic liver disease, results in a broad range of pathophysiologic abnormalities and clinical manifestations, presenting either separately or in various combinations in an individual patient. Pathophysiology, diagnosis and latest guidelines for management of portal hypertension with variceal hemorrhage, ascites, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and hepatocellular carcinoma are each discussed in detail

Complications of Liver Cirrhosis

Ciroza jetre, zadnji stadij kronične jetrene bolesti, ima za posljedicu niz patofi zioloških poremećaja i kliničkih manifestacija, koje se kod pojedinog bolesnika javljaju samostalno ili u različitim kombinacijama. U tekstu su detaljno opisane patofi ziologija, dijagnostika i posljednje smjernice za liječenje portalne hipertenzije i krvarenja iz varikoziteta jednjaka te ascitesa, spontanoga bakterijskog peritonitisa, portalne encefalopatije, hepatorenalnog sindroma, hepatopulmonalnog sindroma i hepatocelularnog karcinoma.Cirrhosis, the end stage of chronic liver disease, results in a broad range of pathophysiologic abnormalities and clinical manifestations, presenting either separately or in various combinations in an individual patient. Pathophysiology, diagnosis and latest guidelines for management of portal hypertension with variceal hemorrhage, ascites, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and hepatocellular carcinoma are each discussed in detail

TREATMENT GUIDELINES FOR PATIENTS WITH GENOTYPE 1 CHRONIC HEPATIS C INFECTION

Standard liječenja kroničnog hepatitisa C genotipa 1 do 2011. godine bila je kombinacija pegiliranog interferona-alfa 2a ili 2b (PEG IFN-α2) i ribavirina. Slijedom odobrenja novih, učinkovitijih lijekova tzv. prve generacije inhibitora virusnih proteaza boceprevira i telaprevira za kliničku primjenu u Europi i SAD-u 2011. g., postoji potreba osuvremenjivanja nacionalnih smjernica za liječenje kroničnog hepatitisa C genotipa 1 u Hrvatskoj. Pri donošenju novih preporuka za liječenje kroničnog hepatitisa C genotipa 1 u Hrvatskoj uzeli smo u obzir rezultate registracijskih studija za boceprevir i telaprevir, postojeće nacionalne smjernice u EU (Velika Britanija, Švedska, Njemačka, Francuska i Italija), preporuke EASL i AASLD te vlastita iskustva u dosadašnjem liječenju bolesnika s kroničnim hepatitisom C genotipa 1 dvojnom terapijom. U tekstu su navedene preporuke za prethodno neliječene kao i liječene bolesnike prema vrsti prethodnog virološkog odgovora. Kod neliječenih bolesnika s niskim stadijem fibroze, te povoljnim prediktorima virološkog odgovora preporuča se nadalje liječenje dvojnom terapijom. Liječenje trojnom terapijom preporuča se za neliječene bolesnike s visokim stadijem fibroze (F3 i F4), kao i one s umjerenim stadijem fibroze (F2) i kombinacijom nepovoljnih prediktora za izliječenje dvojnom terapijom (stariji >40 godina, non-CC IL28B genotip, non-RVR). U skupini prethodno liječenih bolesnika trojna terapija se preporuča za relapsere bez obzira na stadij fibroze, parcijalne respondere s visokim stadijem fibroze (F3 i F4), te individualni pristup u primjeni trojne terapije za nul-respondere.Dual therapy based on the combination of pegylated interferon-alpha 2a or 2b (PEG IFN-α2) and ribavirin has been considered standard-of-care treatment for chronic hepatitis C genotype 1 up to 2011. The first generation of protease inhibitors, boceprevir and telaprevir, have been approved for clinical use in Europe and USA since 2011. Therefore, national guidelines for the treatment of chronic hepatitis C genotype 1 have been updated to include new and more efficient therapeutic options. Croatian guidelines are based on the results of registration clinical trials for boceprevir and telaprevir, national guidelines of several EU countries (United Kingdom, Sweden, Germany, France and Italy), EASL and AASLD recommendations, as well as on the results of chronic hepatitis C genotype 1 treatment with dual therapy at the national level. The Croatian guidelines include recommendations for treatment-naïve and treatment-experienced patients (based on the type of virologic response to the first-line treatment). In treatment-naïve patients with mild fibrosis and favorable predictors of treatment outcome, dual therapy is the recommended treatment option. In treatment-naïve patients with advanced fibrosis (F3 and F4) as well as in patients with moderate fibrosis (F2) and unfavorable predictors of treatment outcome (age >40 years, non-CC IL-28B genotype, non-RVR), triple therapy is recommended. Triple therapy is also recommended for relapsers (irrespective of fibrosis stage) and partial responders with advanced fibrosis (F3 and F4). Lead-in treatment strategy during triple therapy is recommended for null-responders

Hepatomegalija i povišene aminotransferaze u bolesnice s loše reguliranom šećernom bolešću

Elevated liver function tests, right upper quadrant abdominal pain, and hepatomegaly occurring in a diabetic patient treated with high doses of insulin may point to the presence of pathologic glycogen accumulation in the liver parenchymal cells. The condition was first described in children, however, studies performed in adults have shown a condition that is similar in many aspects. A case is presented of a 41-year-old female diabetic patient with abnormal liver tests and hepatomegaly. Abdominal ultrasound confirmed liver enlargement without any signs of fatty liver. Liver biopsy revealed a picture compatible with glycogenosis. As excessive hepatic glycogen deposition occurred at an adult age and without a related family history, while the patient presented with normal mental and motor development, the diagnoses of Mauriac syndrome and hereditary were ruled out. The condition was attributed to insulin hyperdosage. The patient was recommended improved glycemic control, more appropriate diet and physical exercise. On follow-up visit 3 months of discharge from the hospital, significant hepatomegaly regression was demonstrated by palpation and ultrasonography, and was accompanied by normalization of serum aminotransferases, blood glucose and glycosylated hemoglobin. Elevated serum aminotransferases and alkaline phosphatase with hepatomegaly as a consequence of hepatocellular glycogen accumulation can occur in diabetic patients of any age who are treated with high doses of insulin, and should therefore be included in the differential diagnosis.Pojava povišenih jetrenih enzima, bolova u desnom gornjem abdominalnom kvadrantu i hepatomegalije u bolesnika sa šećernom bolešću liječenih visokim dozama inzulina može ukazivati na patološko nakupljanje glikogena u stanicama jetrenog parenhima. Ovo je stanje prvotno opisano u djece, a kasnije studije provedene na odraslima pokazale su jednaku kliničku sliku. Prikazan je slučaj 41-godišnje žene oboljele od šećerne bolesti s poremećenim jetrenim nalazima i hepatomegalijom, kod koje je ultrazvuk potvrdio povećanje jetre bez znakova steatoze, a biopsijom je postavljena dijagnoza glikogenoze. S obzirom na to da je izrazito nakupljanje glikogena u ovom slučaju nastupilo u odrasloj dobi, bez pozitivne obiteljske anamneze, a bolesnica je imala uredan psihički i tjelesni razvoj, iz diferencijalne dijagnoze se je mogao isključiti Mauriacov sindrom i nasljedna glikogenoza. Uzrok ovoga stanja u ove bolesnice bilo je liječenje osnovne bolesti visokim dozama inzulina. Stoga joj je preporučena poboljšana kontrola glikemije praćena ispravnom dijetom i tjelesnom aktivnošću. Na kontrolnom pregledu nakon tri mjeseca došlo je do regresije hepatomegalije potvrđene palpacijom i ultrazvukom, te do normaliziranja serumskih aminotransferaza, glukoze u krvi i glikoziliranog hemoglobina. Povišene aminotransferaze i alkalna fosfataza uz hepatomegaliju mogu se naći kod bolesnika dijabetičara liječenih visokim dozama inzulina, kao posljedica nakupljanja glikogena u jetrenim stanicama, pa ovo stanje treba uključiti u diferencijalnu dijagnozu

TREATMENT GUIDELINES FOR PATIENTS WITH GENOTYPE 1 CHRONIC HEPATIS C INFECTION

Standard liječenja kroničnog hepatitisa C genotipa 1 do 2011. godine bila je kombinacija pegiliranog interferona-alfa 2a ili 2b (PEG IFN-α2) i ribavirina. Slijedom odobrenja novih, učinkovitijih lijekova tzv. prve generacije inhibitora virusnih proteaza boceprevira i telaprevira za kliničku primjenu u Europi i SAD-u 2011. g., postoji potreba osuvremenjivanja nacionalnih smjernica za liječenje kroničnog hepatitisa C genotipa 1 u Hrvatskoj. Pri donošenju novih preporuka za liječenje kroničnog hepatitisa C genotipa 1 u Hrvatskoj uzeli smo u obzir rezultate registracijskih studija za boceprevir i telaprevir, postojeće nacionalne smjernice u EU (Velika Britanija, Švedska, Njemačka, Francuska i Italija), preporuke EASL i AASLD te vlastita iskustva u dosadašnjem liječenju bolesnika s kroničnim hepatitisom C genotipa 1 dvojnom terapijom. U tekstu su navedene preporuke za prethodno neliječene kao i liječene bolesnike prema vrsti prethodnog virološkog odgovora. Kod neliječenih bolesnika s niskim stadijem fibroze, te povoljnim prediktorima virološkog odgovora preporuča se nadalje liječenje dvojnom terapijom. Liječenje trojnom terapijom preporuča se za neliječene bolesnike s visokim stadijem fibroze (F3 i F4), kao i one s umjerenim stadijem fibroze (F2) i kombinacijom nepovoljnih prediktora za izliječenje dvojnom terapijom (stariji >40 godina, non-CC IL28B genotip, non-RVR). U skupini prethodno liječenih bolesnika trojna terapija se preporuča za relapsere bez obzira na stadij fibroze, parcijalne respondere s visokim stadijem fibroze (F3 i F4), te individualni pristup u primjeni trojne terapije za nul-respondere.Dual therapy based on the combination of pegylated interferon-alpha 2a or 2b (PEG IFN-α2) and ribavirin has been considered standard-of-care treatment for chronic hepatitis C genotype 1 up to 2011. The first generation of protease inhibitors, boceprevir and telaprevir, have been approved for clinical use in Europe and USA since 2011. Therefore, national guidelines for the treatment of chronic hepatitis C genotype 1 have been updated to include new and more efficient therapeutic options. Croatian guidelines are based on the results of registration clinical trials for boceprevir and telaprevir, national guidelines of several EU countries (United Kingdom, Sweden, Germany, France and Italy), EASL and AASLD recommendations, as well as on the results of chronic hepatitis C genotype 1 treatment with dual therapy at the national level. The Croatian guidelines include recommendations for treatment-naïve and treatment-experienced patients (based on the type of virologic response to the first-line treatment). In treatment-naïve patients with mild fibrosis and favorable predictors of treatment outcome, dual therapy is the recommended treatment option. In treatment-naïve patients with advanced fibrosis (F3 and F4) as well as in patients with moderate fibrosis (F2) and unfavorable predictors of treatment outcome (age >40 years, non-CC IL-28B genotype, non-RVR), triple therapy is recommended. Triple therapy is also recommended for relapsers (irrespective of fibrosis stage) and partial responders with advanced fibrosis (F3 and F4). Lead-in treatment strategy during triple therapy is recommended for null-responders

Adjusted Blood Requirement Index as Indicator of Failure to Control Acute Variceal Bleeding

Aim: To estimate the clinical value of adjusted blood requirement index (ABRI) in relation to other criteria for failure of variceal bleeding control proposed at Baveno consensus workshops and to evaluate ABRI as an early predictor of occurrence of other Baveno criteria and identification of possible predictors of unfavorable ABRI. Methods: We retrospectively analyzed the data on 60 patients admitted to the hospital due to acute variceal bleeding. Number of treatment failures according to Baveno II-III and Baveno IV definitions and criteria was compared. We tested the ABRI’s predictability of other Baveno IV and Baveno II-III criteria. Logistic regression analysis was performed to ascertain independent variables that predict ABRI≥0.75. Results: Failure to control variceal bleeding occurred in 40 of 60 patients according to Baveno II-III criteria, and in 35 of 60 patients according to Baveno IV criteria. Excluding the criterion of “transfusion of 2 units of blood or more (over and above the previous transfusions)”and ABRI criterion, failure to control variceal bleeding was observed in 17 and 14 of 60 patients, respectively. Congruence of ABRI with other criteria was present in about two-thirds of the cases. ABRI≥0.75 was associated with increased risk of positive other Baveno criteria,particularly modified Baveno II-III (odds ratio [OR] 4.10; 95% confidence interval [CI], 1.11-15.05) and Baveno IV without ABRI (OR 4.37; 95% CI, 1.04-18.28). Independent predictors of ABRI≥0.75 identified in logistic regression analysis were male sex (P<0.001) and higher hematocrit values (P=0.004). Conclusion: We found low congruence between ABRI and other Baveno criteria. It seems that criteria related to the quantity of blood transfusions are not reliable indicators of treatment failure