[Vascular purpura and Guillain--Barre syndrome in patients with severe Wilson--Konovalov disease].
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Biomedical subjects
Publications and source records attributed to T N Lopatkina.
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AIM: To examine clinical features of Sjogren's syndrome (SS) and morphological picture of the lesser salivary glands (LSG) in chronic hepatitis C (CHC). MATERIAL AND METHODS: The examination of 42 patients with SS and chronic HCV infection (mean age 44.3 +/- 13.7 years) has detected signs of chronic hepatitis and hepatic cirrhosis, respectively, in 31 (71.4%) and 11 (26.2%) patients. "Dry syndrome" was diagnosed by criteria of European SS Study Group. LSG biopsy of the lower lip was conducted in 23 (54.7%) of 42 patients. RESULTS: The "dry" syndrome in CHC ran subclinically in 73.8% patients. Apparent symptoms of SS were seen primarily in middle-aged and aged women with CHC history over 10 years. The first signs of SS occurred in 25 (59.5%) patients 2.9 +/- 3.1 years prior to diagnosis of hepatic disease. All the patients had xerostomy. Xerophthalmia was recorded 1.5 times less frequently. In 16 (47.1%) patients with CHC "dry eye" and in 6 (17.6%) patients dry keratoconjunctivitis were detected. Pathohistological changes of LSG were diagnosed in 21 (91.3%) of 23 patients with CHC. In the majority of cases (86.9%) the glands exhibited insignificant inflammatory infiltration and advanced fibrosis. LSG in CHC is characterized by fibrosis prevalence over cell infiltration. 83.3% CHC patients had SS and other extrahepatic lesions. SS was most evident in 28.6% CHC patients with cryoglobulinemia. CONCLUSION: Registration of SS symptoms in CHC patients depends on targeted examination of patients with chronic HCV infection. The severity of the symptoms correlates directly with the infection duration and age of the patient. LSG lesions in CHC patients with SS are characterized by fibrosis pre-domination over cell infiltration.
The necessity of the search for new drugs to treat chronic hepatitis B (CHB) is explained by the necessity to prevent hepatic cirrhosis (HC) and hepatocellular carcinoma. Treatment of HBeAg-negative CHB rests on the same principles as of HBeAg-positive one. Efficacy of nucleoside analogue lamivudin is well studied in HBeAg-positive CHB. The aim of this study was to evaluate lamivudine efficacy in therapy of HBeAg-negative CHB. Lamivudine (epivir--150 mg/day or zeffix--100 mg/day) was given for 1 year to 10 patients (5 males, 5 females, mean age 49.5 +/- 13.5). Their blood serum contained no HBeAg but contained HBeAb and HBVDNA. Chronic hepatitis was verified morphologically in 9 patients of whom 2 had HC and 2 developing HC. Moderate activity of the disease was in 4 patients, low--in 5. All the patients had a high ALT level (150 +/- 140 U/l, 60-528 U/l, high normal value 40 U/l). ALT and HBV DNA in the serum were examined by polymerase chain reaction in the course of treatment and for 6 months after its end. To the end of the treatment a complete response (absence of HBVDNA and normalization of ALT) was achieved in 8 (80%) patients. 5 (63%) of them 2-4 months after the end of the treatment had the exacerbation with appearance of HBVDNA in the serum and elevation of ALT level. A persistent response (6 months after lamivudin treatment) was in 3 (30%) patients, in 2 of them HBsAg was not detected. Lamivudin therapy is effective in HBeAg-negative CHB. In this study a high baseline level of ALT was the factor predisposing to a lasting response to treatment.
The trial enrolled 38 patients with chronic HCV-infection and Sjogren's syndrome (mean age 44.3 +/- 13.7 years). Biopsy of the minor salivary glands (MSG) was made in 20 patients. Polymerase chain reaction was used to study 20 MSG biopsies, 38 samples of native saliva for HCV-RNA. Saliva samples were also studied for Herpes virus DNA (EBV, CMV, HHV-VI type). All the patients with VHC appeared to have signs of xerostomia, 24 (63.2%) patients had xerophthalmia. MSG pathohistological changes were found in 19 (95%) patients. In the majority of cases (86.9%) they were characterized by mild infiltration and advanced fibrosis. HCV-RNA was found in the saliva of 23 (57.5%) patients, in MSG tissue--in 9 (39.1%) patients. HCV-RNA detection in the saliva did not depend on the degree of viremia, viral RNA in MSG correlated with viral load. EBV and HHV-VI, HHV-VI only and EBV were detected only in 7 (18.4%), 10 (26.3%) and 6 (15.8%) patients, respectively. Xerostomia occurred with the same rate (26.1 and 31.3%) in patients with and without herpes viruses in the saliva. Detection rate for HCV-RNA in the saliva was not related with viremia degree. Sjogren's disease symptoms in CHC patients did not depend on the presence or absence of DNA of herpes viruses in the saliva.
AIM: To determine factors affecting overall survival and risk to develop hepatocellular carcinoma (HCC) in patients with hepatic cirrhosis (HC) of mixed (viral, alcoholic) etiology. MATERIAL AND METHODS: Mono- and multi-variance analysis of prognostic effects of such factors as age of the patients, mixed infection VHB/VHC, markers of HBV replication, antibodies to nuclear antigen of HBV (HBcAb) without HBs-system in the serum ("isolated" HBcAb), duration of viral infection, alcohol intake and abuse, dilatation of the esophageal veins, some laboratory parameters were studied in 55 HC patients having at least one marker of hepatitis B virus (HBV) and/or hepatitis C virus (HCV), long history of alcohol abuse. RESULTS: It was found that risk of HCC was associated with duration of alcohol abuse and infection, mixed HBV/HCV infection, age 60 and older. Of independent significance was only duration of alcohol abuse. Lethal outcomes in HC patients in the mixed infection were due to development of HCC (36%) and HC complications (64%). Survival of the patients was less in severe dilatation of the esophageal veins, high clinicolaboratory index, low level of serum albumin, presence of "isolated" BcAb and mixed viral infection. CONCLUSION: Of the greatest prognostic efficacy in respect of survival was the model combining "isolated" HBcAb, the degree of esophageal veins dilatation and hepatitis activity.
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Upon analysis of 550 cases of different chronic diffuse pulmonary diseases included in a group of interstitial diseases of the lungs (IDL) the authors came to the conclusion that IDL incorporate such variants as alveolitis, pulmonary vasculitis and pulmonary hemorrhages; granulomatosis covers exogenic allergic alveolitis, alveolitis in chronic active hepatitis; vasculitis group includes such rare diseases as necrotizing sarcoid granulomatosis vasculitis and lymphoid granulomatosis; fibrosing alveolitis--secondary alveolitis in sclerodermia systematica, rheumatoid arthritis, Sjogren's disease, chronic active hepatitis. Knowledge of IDL etiology (environmental, occupational, induced by radionuclides, drugs, viruses, fungi) with focus on drug affection of the lungs is thought of value. Biopsy and bronchial lavage findings are compared clinically and morphologically. Mechanisms of pulmonary fibrosis and approaches to inhibition of pulmonary fibrosis progression are outlined.
Various profiles of serum HBV markers (HbsAg) were identified in 16 patients, markers of active replication in 4 patients (HbeAg, 2 cases; HBcAb IgM, 1 case; HBcAb IgM and HbsAg IgM, 1 case) out of 22 patients with Sjögren disease (SD) having serum HBV markers and systemic manifestations. Of these 3 had a history of acute viral hepatitis, 12 were previously at risk to be infected with hepatitis viruses, 7 had neither the disease nor its risk factors, none of them had chronic active hepatitis or hepatic cirrhosis, 7 exhibited clinical signs of pseudolymphoma, 1 had serum monoclonal IgM kappa in the presence of HBV active replication markers (HBsAg IgM, HbcAb IgM). The present and previous data (on association of SS with chronic active hepatitis and hepatic cirrhosis due to HBV) suggest the involvement of the virus in SD etiology.
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Myocarditis attended chronic active hepatitis in 22 patients (in 13 of them the outcome of hepatitis was liver cirrhosis). Twelve patients recovered, postmyocarditis cardiosclerosis developed in 6 and 4 patients died. Chronic myocarditis in the stage of aggravation was revealed in 3 cases at autopsy and severe postmyocarditis cardiosclerosis with manifested dilatation of the heart cavities in 1 case.
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Different patterns of serum HBV markers (HBsAg, HBsAb, HBeAg, HBeAb, HBcAb) were identified in 82 patients (12 with chronic active hepatitis, 67 with hepato-cirrhosis developing in the outcome of chronic active hepatitis, and in 3 with primary biliary cirrhosis) of 105 (78%) patients with chronic liver diseases. Besides, IgM HBcAb were identified in 9 of 47 patients (19.1%), HBsAg IgM in 4 of 9 patients (44.4%), antibodies to the delta-agent in 8 of 23 (34.8%) suffering from chronic liver diseases with the presence of serum HBV markers. Chronic liver diseases with the presence of HBsAg and/or other serum HBV markers were characterized by a variety of clinico-laboratory manifestations determined by the involvement of some organs and systems in a process. A degree of activity of chronic liver diseases was not correlated with a replication phase, an integration phase, and an immunological resolution phase in 49 patients with identified phases of development of HBV-infection. More frequent identification of serum HBV markers including those of a replication phase was not noticed among patients on prolonged immunosuppressant therapy.
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