Family Cases of Autoimmune Liver Disease

Абдулганиева, Д. И.; Одинцова, А. Х.; MUKHAMETOVA, D. D.; Черемина, Н. А.

doi:10.33978/2307-3586-2019-15-28-62-65

Cited by 3 publications

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“…Оба заболевания могут прогрессировать до цирроза печени [51]. Семейные случаи перекреста аутоиммунного гепатита и первичного билиарного холангита встречаются редко [52]. Авторы представили такие наблюдения у сибсов.…”

Section: клиникаunclassified

Primary Biliary Cholangitis

Pandit¹,

Samant

2017

Diagnostic Pathology: Hepatobiliary and Pancreas

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медицинский исследовательский центр трансплантологии и искусственных органов имени академика В.И. Шумакова» Минздрава России, Москва, Российская Федерация 2 ФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. Сеченова Минздрава России (Сеченовский университет), Москва, Российская Федерация Первичный билиарный холангит (ПБХ), ранее известный как первичный билиарный цирроз, является органоспецифическим аутоиммунным заболеванием, которым страдают преимущественно женщины среднего возраста и которое не встречается у детей. Распространенность ПБХ различается в зависимости от географического расположения страны. За последние 30 лет отмечен рост заболеваемости и одновременно достигнут значительный прогресс в понимании патогенеза ПБХ благодаря развитию инновационных технологий в молекулярной биологии, иммунологии и генетике. Наличие антимитохондриальных антител и холестаз при биохимическом анализе достаточны для постановки диагноза, без необходимости проведения биопсии печени. Мишенями при ПБХ являются мелкие и средние желчные протоки. При первой стадии заболевания наблюдается гранулематозная деструкция желчных протоков; при второй -потеря желчных протоков, их пролиферация, увеличение размеров портальных трактов с хроническим воспалением; при третьей -фиброз с образованием септ, потеря желчных протоков и холестаз; при четвертой стадии -цирроз печени. Ранее выживаемость пациентов с ПБХ составляла от 7,5 до 16 лет, но она значительно повысилась при лечении урсодезоксихолевой кислотой и обетихолевой кислотой. При отсутствии эффекта от лечения и развитии терминальной стадии печеночной недостаточности проводят трансплантацию печени.

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Section: клиникаunclassified

Primary Biliary Cholangitis

Pandit¹,

Samant

2017

Diagnostic Pathology: Hepatobiliary and Pancreas

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Evaluation of Small Intestinal Permeability in Patients with Overlap Syndrome (Autoimmune Hepatitis/Primary Biliary Cholangitis)

Акберова

Odintcova

Абдулганиева

2023

Rossijskij žurnal gastroènterologii, gepatologii, koloproktolog

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Аim: to evaluate the state of small intestine permeability by the “double sugar test” in patients with overlap syndrome (autoimmune hepatitis / primary biliary cholangitis (AIH / PBC)).Materials and methods. Prospectively, 56 people were included in the study. Of these, 26 were diagnosed with AIH/PBC, 30 were in the control group. The diagnosis was made in accordance with the current recommendations. The average age of patients was 49.7 ± 13.8 years, healthy volunteers — 48.6 ± 9.2 years. The determination of the permeability of the small intestine was carried out by a “double sugar test” (the ratio of lactulose/mannitol in urine), using the method of high-performance liquid chromatography — mass spectrometry.Results. In patients with AIH/PBC, an increase in intestinal permeability was found — 0.20 [0.09; 0.30] (p < 0.001) compared with the control group 0.01 [0.01; 0.02]. We divided patients at the stage of liver damage. An increased small intestinal permeability was revealed: hepatitis stage — 0.19 [0.13; 0.30] (p < 0.001), liver cirrhosis stage — 0.18 [0.09; 0.30] (p < 0.05) compared with the control group. In the early stages of disease (1 month from the onset of the disease) had an increased lactulose/mannitol ratio — 0.13 [0.05; 0.26] (p < 0.001) compared to the control group. In the presence of portal hypertension (PH), small intestinal permeability was increased — 0.18 [0.09; 0.30] (p < 0.001) compared with the control group.Conclusions. An increase in small bowel permeability was found in patients with overlapping syndrome. All patients had increased intestinal permeability (regardless of the presence of extrahepatic manifestations).

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Thirteen-Year Follow-Up of a Patient with Liver Cirrhosis Resulting from the Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis: Severe COVID-19 and Liver Transplantation

Odintsova,

Akberova,

Sadykova

et al. 2024

Rossijskij žurnal gastroènterologii, gepatologii, koloproktolog

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Aim: to present the difficulties of long-term management of a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) who suffered from severe COVID-19 infection.Key points. The diagnosis of liver cirrhosis as an outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) was established at the patient’s age of 33 years. At the age of 40, the patient became pregnant for the first time, the pregnancy proceeded well, and a cesarean section was performed at 36 weeks. At the age of 45, the patient suffered a severe new coronavirus infection, followed by decompensation of liver cirrhosis, which required liver transplantation 4 months after COVID-19, followed by a favorable postoperative course.Conclusion. This clinical case demonstrates the successful onset and outcome of pregnancy in a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis). The pronounced activity of the disease after severe new coronavirus infection required liver transplantation with successful outcome.

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Family Cases of Autoimmune Liver Disease

Cited by 3 publications

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Primary Biliary Cholangitis

Primary Biliary Cholangitis

Evaluation of Small Intestinal Permeability in Patients with Overlap Syndrome (Autoimmune Hepatitis/Primary Biliary Cholangitis)

Thirteen-Year Follow-Up of a Patient with Liver Cirrhosis Resulting from the Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis: Severe COVID-19 and Liver Transplantation

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