[A contribution to the differential diagnostics of sclerosing cholangitides]. / Príspevek k diferenciální diagnostice sklerozujících cholangitid.

Sclerosing cholangitides represent a group of chronic biliary obstructive diseases which include primary sclerosing cholangitis (PSC), IgG4 associated sclerosing cholangitis (IgG4-SC) and secondary sclerosing cholangitis (SSC). The manifestations of the diseases are similar, but their asymptomatic course is also frequent. IgG4-SC belongs to the group of IgG4 associated diseases and it is the most frequently related to type 1 autoimmune pancreatitis. Diagnosing of IgG4-SC is based on typical histopathological images, shape changes revealed by diagnostic imaging, serological tests, concurrent impairment of other organs and response to therapy, where IgG4-SC responds well to treatment with corticoids, whereas the only possibility for the remaining units is endoscopic intervention or liver transplantation. Secondary sclerosing cholangitis may develop as a result of many different insults affecting the biliary tree. Among them, the most frequently described include long-lasting biliary obstruction, surgical injury of the biliary tree, and ischemic cholangitis in liver allotransplants or recurrent pancreatitis. We use serological and imaging examination in PSC diagnostics, sometimes we have to resort to liver biopsy. PSC is to a significant degree accompanied by the presence of idiopathic bowel disease, typically ulcerative colitis. As a result, PSC may lead to cirrhosis of the liver and it is a precancerous condition of several malignancies. With regard to variable locations of the biliary tree injuries concerning the aforementioned units, also certain malignancies in subhepatic landscape need to be considered in the differential diagnosis: pancreatic cancer and cholangiogenous carcinoma.Key words: genetic factors - IBD - IgG4 cholangitis - liver transplantation - bile duct cancer - ursodeoxycholic acid - primary sclerosing cholangitis - secondary cholangitis - sclerosing cholangitis.

[The rational diagnostic of cholangiocarcinoma]. / Racionální diagnostika cholangiokarcinomu.

Cholangiocarcinoma (CC) is a rare malignant tumour arising from cholangiocytes, and its prognosis is usually unfavourable, mostly as a result of late diagnosis of the tumour. The current incidence of cholangiocarcinoma in the Czech Republic is 1.4/100,000 inhabitants per year; in less than 30 % of patients with CC, one of the known risk factors can be identified, most frequently, primary sclerosing cholangitis. Only patients with early diagnosed and surgically amenable cholangiocarcinoma are likely to have a longer survival time; in their case, survival for more than five years has been achieved in 20 % to 40 %. From the perspective of the need for early diagnosis of CC, a significant part is played by imaging and histopathologic evaluation; the early diagnostic significance of oncomarkers is limited. The rational early diagnosis of CC consists in effective use of differentiated advantages of different imaging modalities - MRI with DSA appears to be the optimal method, endosonography is a sensitive method for the identification of malignancy in the hepatic hilum or distal common bile duct, MRCP (magnetic resonance cholangiopancreatography) is used to display pathological changes in the biliary tree, ERCP (endoscopic retrograde cholangiopancreatography) allows material removal for histopathological examination. Other new approaches are also beneficial, such as IDUS - intraductal ultrasonography of biliary tract or SPY-GLASS, enabling examination of the bile ducts by direct view with the possibility of taking targeted biopsies. Sensitivity and specificity of histology and cytology can be increased by using the molecular cytogenetic FISH method, i.e. fluorescence in situ by hybridization, with a specificity of 97 %.

[Autoimmune pancreatitis - diagnostic consensus]. / Autoimunitní pankreatitida - diagnostický konsenzus.

The autoimmune type of pancreatitis represents the specific disease of pancreas, with significant contribution of autoimmune processes in its etiopathogenesis. Currently, there are two proved subtypes of this particular pancreatopathy, which are defined clinically, histomorphologically and serologically. They have many histomorphological signs in common, but differ in the presence of so-called granulocytic epithelial lesions (GEL), which are absent in subtype 1. The subtype 1 is characterized by the presence of gammaglobulines, esp. immunoglobuline G4 and IgG4 positive extrapancreatic lesions. The subtype 2 is typically associated with the inflammatory bowel diseases, esp. ulcerative colitis. But the common characteristic of both subtypes is the fact response to applied steroid treatment. Due to diverse diagnostic criteria in the past, in 2011 the consensus for the diagnosis of autoimmune pancreatitis was announced. It is based on clinical symptoms, biochemical results, the results got by using of imaging methods, histomorphology and positive response to steroid treatment. The matter to be solved is the question of early differential diagnosis between focal autoimmune pancreatitis and adenocarcinoma of pancreatic head. From imaging methods are MRI/CT, MRCP (in Asia ERCP), EUS with targeted biopsy of the gland (under EUS control), are recommended as the methods of choice.

Forensic and clinical issues in the use of frangible projectile.

Frangible projectiles for firearms, which break apart on impact, are mainly used by law enforcement agencies for training purposes, but can also be used for police interventions. Apart from the usual absence of lead in the projectiles, the main advantage of using frangible projectiles is the reduced risk of ricochet after impact with a hard target. This article describes the design and function of frangible projectiles, and describes gunshot wounds caused by ultra-frangible projectiles which fragment after penetration of soft tissues. Shooting experiments performed by the authors confirmed that differences in the geometry and technology of frangible projectiles can significantly modify their wounding effects. Some frangible projectiles have minimal wounding effects because they remain compact after penetration of soft tissues, comparable to standard fully jacketed projectiles. However, a number of ultra-frangible projectiles disintegrate into very small fragments after impact with a soft tissue substitute. In shooting experiments, we found that the terminal behavior of selected ultra-frangible projectiles was similar in a block of ballistic gel and the soft tissues of the hind leg of a pig, except that the degree of disintegration was less in the gel.

[Low response rate to a vaccination against hepatitis B in patients with end-stage liver disease]. / Malá úcinnost vakcinace proti virové hepatitide B u pacientu s pokrocilým jaterním onemocnením.

Hepatitis B vaccination was started in 41 patients with end-stage liver disease who were liver transplant candidates. Patients received three 20 microg or, starting from 1999, 40 microg doses of recombinant vaccine at 0, 2 and 4 weeks. Blood samples were obtained 4 weeks after vaccination or each revaccination; patients without protective hepatitis B surface antibodies (anti-HBs) were once or repeatedly revaccinated. Thirty-eight patients received at least 3 doses of vaccine. Protective anti-HBs level (≥ 10 IU/l) was detected in 17 of 34 patients (50 %) after the third up to eight dose. No case of chronic HBsAg carrier status was detected. Immunisation against hepatitis B in persons with liver cirrhosis is associated with a poor response and new vaccines should be considered for these patients.