[The differential diagnosis of Erdheim-Chester disease and IgG4-related diseases].

Abstract:

:The paper describes Russia's first diagnosed case of Erdheim--Chester disease (systemic histiocytosis) in a 65-year-old man who has been long treated for Ormond's disease (idiopathic retroperitoneal fibrosis). It also gives the data available in the literature on the pathogenetic components of these diseases and on the similarity of many clinical, laboratory, and morphological characteristics of these two immunoinflammatory diseases and covers the issues of their differential diagnosis. Invasive procedures with a careful morphological/immunomorphological examination of biopsy specimens obtained from affected tissues are shown to be necessary for accurate diagnosis. :Описан первый случай диагностики в России системного гистиоцитоза - болезни Эрдгейма-Честера у 65-летнего мужчины, длительно получавшего терапию по поводу болезни Ормонда (идиопатического ретроперитонеального фиброза). Приведены данные литературы по патогенетическим звеньям, сходству многих клинических, лабораторных и морфологических характеристик этих двух иммуновоспалительных заболеваний, освещены вопросы дифференциальной диагностики. Показано, что проведение инвазивных процедур с тщательным морфологическим/иммуноморфологическим исследованием биоптатов пораженных тканей является необходимым условием для правильной постановки диагноза.

journal_name

Ter Arkh

journal_title

Terapevticheskii arkhiv

authors

Vasilyev VI,Sokol EV,Kokosadze NV,Pavlovskaya AI,Komov DV,Palshina SG,Mukhortova OV,Safonova TN

doi

10.17116/terarkh201688570-76

subject

Has Abstract

pub_date

2016-01-01 00:00:00

pages

70-76

issue

5

eissn

0040-3660

issn

2309-5342

journal_volume

88

pub_type

杂志文章
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    abstract:AIM:To characterize the genotype and genotype-phenotype correlations in patients with Gaucher disease (GD) in the Russian Federation. MATERIALS AND METHODS:One hundred adult patients with GD type 1 were examined. Their clinical study encompassed the evaluation of the severity of osteoarticular lesions from instrumenta...

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  • [The manifestation of immune reactions and the chromium content of the blood in patients with occupational allergic dermatoses].

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    authors: Pavlovskaia NA,Somov BA,Iatsyna IV

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  • [Possibilities of eradicating the persistent HBsAg carrier state].

    abstract::Five HBsAg carriers were examined by clinical, biochemical, radionuclide, immunologic and histomorphologic methods. Different variants of structural and functional relations were established. Attempts were made to eradicate the persistent virus carrierstate with the aid of the antiviral drug virasol, the immunomodulat...

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    doi:

    authors: Farber NA,Ambrozaĭtis AK,Kelli EI,Vygodskiĭ VS,Savitskiĭ GI

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  • [The correlation of the indices of humoral immunity and of the activity of the renin-angiotensin-aldosterone system in hypertensive patients].

    abstract::The content of IgA, IgM, IgG and IgE was measured in 55 male patients with uncomplicated essential hypertension (EH) running a labile course (25 patients) and a stable course (30 patients). Analyzing the material, account was taken of aggravated heredity factor as regards arterial hypertension, established according t...

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    authors: Vil'chinskaia MIu,Zharova EA,Nasonov EL,Bosykh EG

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  • [Ursodeoxycholic acid-enhanced efficiency and safety of statin therapy in patients with liver, gallbladder, and/or biliary tract diseases: the RACURS study].

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  • [H. pylori in development of allergic diseases in patients with gastroduodenal diseases].

    abstract:AIM:To discover relations between allergic diseases and H. pylori infection in patients with gastroduodenal diseases. MATERIAL AND METHODS:The study was made of 113 patients (age 22-65, 34 males and 79 females) with chronic gastritis and duodenal ulcer. Of them, 60 patients (group 1) had concomitant allergy (urticaria...

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    doi:

    authors: Lazebnik LB,Chernutskaia SP,Gervazieva VB,Sukhareva GV

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  • [The effect of a single dose of nifedipine, intal, sodium thiosulfate and Essentiale on the blood level of calcium, hydroperoxides, thiol compounds and prostaglandins in bronchial asthma patients].

    abstract::A study was made of the impact of a single intake of nifedipine, intal, sodium thiosulfate and essentiale on respiratory function, the blood content of ionized calcium, total calcium, hydroperoxides, total and non-protein thiol groups, and prostaglandins in bronchial asthma patients. All the drugs exerted an antioxida...

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    doi:

    authors: Amatuni VG,Malaian KL,Zakaharian AK

    更新日期:1992-01-01 00:00:00

  • [Changes in fatty acids content in blood red cells of patients with iron-deficiency anemia treated with sorbifer and mildronate].

    abstract:AIM:To study fractional composition of red cell membrane (RCM) lipids in patients with severe iron-deficiency anemia (IDA) complicated by myocardiodystrophy prior to treatment and after 1-month combined treatment with sorbifer and mildronate. MATERIAL AND METHODS:Fatty acid composition of RCM lipids was studied in 12 ...

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    doi:

    authors: Goncharova EV,Govorin AV

    更新日期:2008-01-01 00:00:00

  • [Involvement of the hepatobiliary system and pancreas in obesity].

    abstract::The article provides an overview of current views on the involvement of the liver, gallbladder, and pancreas in patients with overweight and obesity. It considers the general issues of the pathogenesis of these conditions, their clinical features and diagnostic methods. :В статье приводится обзор современных взглядов...

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    authors: Akhmedov VA,Gaus OV

    更新日期:2017-01-01 00:00:00

  • [Comparative study of the dynamic activity of heart-specific blood enzymes in stenocardia, acute infarct and focal dystrophy of the myocardium and in their recurrences].

    abstract::The regular character and a tendency of the time course of the absolute activity of the heart-specific blood enzymes and their coefficients in focal myocardial dystrophy, angina of effort and rest, angina of effort and unstable angina after each attack of chest pains and their recurrences which were identical to those...

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    doi:

    authors: Makarovskiĭ VV

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  • [Comparative evaluation of various methods of immunosuppressive therapy in severe bronchial asthma].

    abstract::Altogether 216 patients with grave infection-dependent (49.5%) and autoimmune (50.5%) bronchial asthma received 311 courses of immunosuppressant therapy, with follow up periods up to 8 years. According to the clinico-immunologic data the patients were distributed into 4 groups. The basic drugs were (1) azathioprin, (2...

    journal_title:Terapevticheskii arkhiv

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    doi:

    authors: Rudyk BI

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  • [Role of certain neurohumoral factors in development of pulmonary hypertonia in patients with interstitial lung diseases].

    abstract:AIM:To ascertain the role of some neurohumoral factors--nitric oxide (NO), ACE, histamine--in development of pulmonary hypertension (PH) in patients with interstitial lung diseases (ILD). MATERIAL AND METHODS:A total of 32 ILD patients were examined. Of them 14 had idiopathic fibrosing alveolitis (IFA), 6 had exogenou...

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    doi:

    authors: Arkhipova DV,Kornev BM,Popova EN,Kogan EA,Osipenko VI,Privalova EV,Kobylenskiĭ AG,Meshcheriakova SA,Efremov EE

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  • [A patient with mitral prolapse: differential intervention programs in general medical practice].

    abstract:AIM:To develop and implement intervention programmes for patients with mitral prolapse (MP) under real conditions of general medical practices of Russia in the transition period. MATERIAL AND METHODS:The MP register covered 208 patients among the adult population (n = 3346) of 2 districts served by general practitione...

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    authors: Movshovich BL,Lisitsa DN

    更新日期:2002-01-01 00:00:00

  • [The problems in optimizing the delivery of general therapeutic care at the regional level during the transition to compulsory medical insurance].

    abstract::The authors hold that perfection of local therapeutic service in present-day situation when insurance principles are introduced in medical care may be achieved through design of medico-economic standards of guaranteed minimum of medical aid for each citizen of Russian Federation. This approach allows accurate estimati...

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    doi:

    authors: Riabov SI,Poliakov IV,Lind VA,Petukhova VV,Andreeva EN,Kaiukov IG,Ermakov IuA,Dobronravov VA,Rumiantsev ASh

    更新日期:1996-01-01 00:00:00

  • [Antibodies to cyclic citrullinized peptide in rheumatoid arthritis].

    abstract:AIM:To estimate a diagnostic value of antibodies to cyclic citrullinized peptide (CCP) in rheumatoid arthritis (RA). MATERIAL AND METHODS:The study was made of 85 RA patients. Of them, 48 patients had early RA, i.e. of 8 month and less duration. The control group consisted of 35 patients with non-differentiated arthri...

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    doi:

    authors: Aleksandrova EN,Chemeris NA,Karateev DE,Novikov AA,Brodetskaia KA,Nasonov EL

    更新日期:2004-01-01 00:00:00

  • [Immunological and rheological disorders in multiple myeloma patients].

    abstract::Overall 30 patients with multiple myeloma (MM) were examined for immunological and rheological parameters. MM was shown to be marked by imbalance of immunobiological reactivity, with the greatest changes being seen in the B cell immunity system. MM is also characterized by marked rheological shifts related to the immu...

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    authors: Abdulkadyrov KM,Bessmel'tsev SS,Liubimova NIu

    更新日期:1991-01-01 00:00:00

  • [Endothelial vasomotor dysfunction in some forms of systemic vasculitis].

    abstract:AIM:To study vascular endothelium in patients with systemic vasculitis (SV) with non-invasive methods. MATERIAL AND METHODS:A total of 65 SV patients entered the trial: 20 patients with Henoch-Schonlein purpura (HSP), 20 patients with thromboangitis obliterans (TO), 10 patients with polyarteritis nodosa (PN), 15 patie...

    journal_title:Terapevticheskii arkhiv

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    authors: Shilkina NP,Vinogradov AA

    更新日期:2007-01-01 00:00:00

  • [The role of free oxygen radicals in the pathogenesis of Fanconi's anemia].

    abstract::The authors describe the results of a study into antioxidants (superoxide dismutase and catalase) and production of the active forms of oxygen (superoxide anion radical, hydrogen peroxide, hydroxyl radical, and singlet oxygen) by blood and bone marrow leukocytes from patients with Fanconi's anemia. The generation of t...

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    doi:

    authors: Rumiantsev AG,Samochatova EV,Afanas'ev IB,Korkina LG,Suslova TB

    更新日期:1989-01-01 00:00:00

  • [Bosentan: a considerable increase in the survival of patients with pulmonary hypertension associated with systemic rheumatic diseases].

    abstract:AIM:To evaluate the short-term efficacy of the nonselective endothelin receptor antagonist bosentan in the treatment of pulmonary hypertension (PH) associated with diffuse connective tissue diseases (CTD), as well as its effect on survival in both monotherapy and in combination with other PH-specific agents. SUBJECTS ...

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    pub_type: 临床试验,杂志文章

    doi:

    authors: Volkov AV,Iudkina NN,Nikolaeva EV,Kurmukov IA,Glukhova SI,Nasonov EL

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  • [The potentials of intestinoscopy and guided biopsy in the diagnosis of diseases of the small intestine].

    abstract:AIM:To ascertain the role of intestinoscopy in diagnosis of small intestinal diseases. MATERIALS AND METHODS:Intestinoscopy with multiple topographic biopsies was made with SIF-10 device (Olympus) in 92 patients. RESULTS:Of 60 patients with absorption disorders, the diagnosis of gluten enteropathy (GEP), Whipple's di...

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    更新日期:1999-01-01 00:00:00

  • [Two-year results after implantation of a metallic stent EPHESOS in patients with coronary heart disease].

    abstract:AIM:To evaluate two-year results of using a metallic stent EPHESOS in patients with native atherosclerotic lesion of coronary arteries (without prior interventions). MATERIAL AND METHODS:The stent was implanted to 731 patients. 53% had manifestations of unstable angina. 42% of the lesions were complicated. The length ...

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    doi:

    authors: Belenkov IuN,Samko AN,Batyraliev TA,Pershukov IV,Niiazova-Karben ZA,Besnili F,Levitskiĭ IV,Zhamgyrchiev ShT,Vural A,Kalenich O

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  • [Interrelations of genetic markers (HLA antigens and haptoglobin variants) and the course of fibrous-cavernous pulmonary tuberculosis].

    abstract::Altogether 72 patients with different variants of a course of fibrous-cavernous pulmonary tuberculosis were investigated for the presence of HLA antigens and Hp phenotypes. It was shown that an increase in the frequency of antigen DR2 and Hp 2-2 could be used as a prognostic prerequisite in the assessment of a course ...

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    authors: Sadykov AS,Pospelov LE

    更新日期:1988-01-01 00:00:00

  • [The treatment of nephrotic-type chronic glomerulonephritis with Sandimmun (cyclosporin A)].

    abstract::Sandimmun (ciclosporin A) treatment of chronic glomerulonephritis with nephrotic syndrome in a dose 2.5-5 mg/kg for 6-10 months relieved nephrotic syndrome in 2 and resulted in clinical improvement in other 2 of 4 patients treated. In 3 cases the patients failed previous therapy with high-dose corticosteroids and cyto...

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    doi:

    authors: Mukhin NA,Tareeva IE,Krasnova TN,Shilov EM,Miroshnichenko NG

    更新日期:1995-01-01 00:00:00

  • [Morphological and Immunohystochemical Characteristics of Aspirated Thrombi in Patients with ST-Elevation Myocardial Infarction].

    abstract::The aim of study was to evaluate the morphological and immunohystochemical characteristics of aspirated thrombi in patients with ST-elevation myocardial infarction undergoing precutaneous coronary intervention to find the possible platelet activation and inflammatory cell involvement in coronary thrombi. MATERIALS AN...

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  • [The treatment of acute suppurative pyelonephritis in diabetes mellitus patients].

    abstract::The analysis of clinical, diagnostic and treatment efficacy data has been made for 160 diabetes mellitus patients (50 cases of insulin-dependent and 110 of noninsulin-dependent) with acute purulent pyelonephritis (APP). A diffuse form of the disease was detected in 100 patients (62.5%), purulent-destructive one in 60 ...

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    pub_type: 杂志文章

    doi:

    authors: Dovlatian AA

    更新日期:1993-01-01 00:00:00

  • [Hematological "masks" in systemic lupus erythematosus].

    abstract::The authors present literature data and original findings on characteristics and frequency of hematological disorders in SLE. Marked hemopathies are called SLE masks as in primary examination of the patient systemic blood diseases are not rejected this necessitating special investigations to exclude them. A rare case ...

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    pub_type: 杂志文章,评审

    doi:

    authors: Krasovskiĭ II,Shkodkin IV,Vladimirova NN

    更新日期:1997-01-01 00:00:00

  • [The possibilities of an individual approach to intra-articular drug administration in rheumatoid arthritis patients].

    abstract::A study was made of the possibilities of an individual approach to intraarticular administration of protease inhibitors, cytostatics and orgoteine in patients with rheumatoid arthritis (RA). The activity of hyaluronidase, superoxide dismutase, the level of rheumatoid factor and the phagocyte count in synovial fluid we...

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    pub_type: 杂志文章

    doi:

    authors: Matveĭkov GP,Soroka NF,Maksimovich AB

    更新日期:1989-01-01 00:00:00

  • [Characteristics of blood pressure in schoolchildren 7-17 years old (based on one-stage and prospective studies)].

    abstract::One-stage epidemiological survey permitted one to define the limits of normal blood pressure values in schoolchildren aged 7-17 years for each age-sexual group differentially. Isolated blood pressure elevation was found to be rare, amounting to 4.4% in boys and to 3.1% in girls. The rate of new cases of elevated blood...

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    pub_type: 杂志文章

    doi:

    authors: Tubol IB,Il'chenko IN,Akhmeteli MA,Zhukovskiĭ GS,Burlutskiĭ GI

    更新日期:1984-01-01 00:00:00

  • [Mechanism of action of endotoxins in chronic renal failure].

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    pub_type: 杂志文章

    doi:

    authors: Aklaev RN,Usmanov PB,Kim VF,Kalikulov D,Kazakov I

    更新日期:1989-01-01 00:00:00

  • [A comparative evaluation of the efficacy of the clinical use of SKN-4M-, SKT-6A- and polifepan-type sorbents in treating patients with chronic kidney failure (clinical and experimental studies)].

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    pub_type: 杂志文章

    doi:

    authors: Ryss ES,Riabov SI,Lutoshkin MB

    更新日期:1996-01-01 00:00:00