The purpose of the review article is to demonstrate generalized ideas on the classification and diagnosis of cholestasis syndrome of various etiologies, to consider the possibility of using laboratory and instrumental research methods in real clinical practice in a comorbid patient. The main provisions. According to the mechanism of development, cholestasis is conditionally divided into intrahepatic and extrahepatic, as well as a mixed type. Extrahepatic cholestasis develops with mechanical obstruction of the main extrahepatic or main intrahepatic ducts. Intrahepatic cholestasis as a result of a number of diseases, such as acute viral hepatitis, primary biliary cholangitis, drug damage to the liver, amyloidosis of the liver. In real clinical practice, a combination of several etiological factors leading to the development of cholestasis is possible in a comorbid patient. A clinical observation is given when, in a patient with gallstone disease, melanoma and ulcerative colitis, after excluding a number of possible causes of cholestasis, autoimmune cross syndrome, autoimmune hepatitis and primary sclerosing cholangitis (PSC), was diagnosed, which allowed the initiation of immunosuppressive therapy with 48 mg corticosteroid (per day) and the preparation of ursodeoxycholic acid (UDCA) exhol at a dose of 1500 mg per day. Regardless of the cause of intrahepatic cholestasis, UDCA remains the drug for the treatment of first-line cholestatic lesions. Conclusion. Only a consistent methodological approach, taking into account all the possible causes of cholestasis, can lead to a correct diagnosis and timely adequate treatment in each case.
Due to the absence of the pathognomonic diagnostic criteria and to the diversity of clinical, serological and morphological manifestations, the diagnostic of the autoimmune hepatitis (AIH) remains to be a difficult task, which might lead to the delay of the timely beginning of the immunosuppressive therapy (IST), which in turn affects the disease outcomes. Aim.To studying the clinical, biochemical, immunological and morphological markers in patients with seronegative (SN) and seropositive (SP) AIH and the qualities of their response to the IST. Materials and methods.This retrospective cohort study included 82 AIH patients over the course of the years 20142019. All patients were selected in accordance with the criteria of the simplified assessment system of the IAIHG. Clinical, laboratorial and morphological characteristics of the AIH were analyzed. Therapy response was evaluated by the level of the ALT and IgG in 612 months after the start of the IST. The study material underwent statistical analysis using methods of parametrical and nonparametrical analysis. Statistical analysis was performed in the Statistica 13.3 (developed by StatSoft Inc., USA). Results.67/82 (81.70%) of the patients studied were women, median age of 54 years old [38; 70]. Patients with the diagnosis of the possible AIH according to the IAIHG made 85.4% (70 people). Almost everyone 96% (79/82) had morphological features of the interface-hepatitis with the lymphocytic/plasmocytic infiltration; emperipolesis was discovered in 63% of patients (49/82), hepatocellular rosette in 23% (19/82). Patients with SN AIH comprised 36.5% (30/82), with SP 63.4% (52/82). Comparative analysis demonstrated that the clinical profile in patients with SN and SP AIH is the same, while the incidence of immuno-associated diseases is significantly higher in the group of seronegative AIH. The morphological profile in the two AIH groups is identical in both typical and atypical manifestations. The number of responders to IST was 63% (19/30) SN AIH vs 67% SP AIH (35/52), did not differ significantly (p=0.529).However, that the number of patients with liver cirrhosis in the SN AIH group was twice as big as the ones with SP: 37% vs 17% (p=0.089). Conclusions.A comparative analysis of clinical, laboratory, morphological and clinical manifestations in the SN and SP AIH groups did not detected statistically significant significant differences, which may indicate that SN and SP AIH are the faces of one disease. It is possible that AB cannot be identified within the known spectrum of antibodies, or antibodies have slow expression, or are suppressed by the immune system. In any case, suspicions of AIH, in the absence of antibodies, it is recommended that liver biopsy be performed for the timely diagnosis of AIH and IST. Сirrhosis was more often diagnosed in the group SN AIH, which may be due to a later diagnosis, and therefore to untimely IST. The found frequent association of SN AIH with other immune-associated diseases requires a carefully study of this problem. The variety of clinical manifestations of AIH requires further study, the identification of clinical phenotypes with certain feature. This can help in the future to timely identify potentially problematic patients and predict a response to IST.
К л и н и ч е с к о е н а б л ю д е н и е 1 ГБУЗ «Московский клинический научно-практический центр имени А.С. Логинова» Департамента здравоохранения города Москвы (ГБУЗ МКНЦ имени А.С. Логинова ДЗМ); 111123, г. Москва, шоссе Энтузиастов, 86, Российская Федерация Винницкая Елена Владимировнад-р мед. наук, заведующая отделом гепатологии 1
A new approach to the diagnosis of PE has been the identification of latent and overt PE in recent years. The detailed assessment of the stages of PE presented in the paper actually reflects the severity of the disease. The diagnosis of latent PE is established based on the results of at least two psychometric tests repeated in dynamics, and the data of one computerized test. The diagnosis of apparent PE is often made by “excluding” other possible causes of brain dysfunction. It is shown that effective therapy of PE is based not only on the features of the pathogenesis, but is impossible without taking into account the various variants of the course of PE, the severity of the disease. Based on randomized controlled trials conducted in recent years, an effective strategy for the treatment of various forms of PE has been developed: episodic, recurrent. Recurrent, therapy-resistant, and obvious PE in the presence of hepatic insufficiency is an indication for liver transplantation.
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