Редкие (орфанные) заболеванияи синдромы бронхо-легочной системы

This program of lectures and seminars deals with the topic of "rare (orphan) diseases and syndromes of bronchopulmonary system"

1    lecture - a common part.

Subject:       "Epidemiology and register of rare (orphan) bronchi-pulmonary diseases in Russia and in the world."

Summary: provides an overview and definition of almost all diseases on the subject to the assessment of risk factors and methods of epidemiological studies. The analysis of the register of this group of diseases and syndromes to date and evaluation of promising possibilities population genetic studies. We study the questions of clinical, radiological, pathological diagnosis. Data on drug and non-drug therapies, including an analysis of all currently available orphan medikametov. Understand questions pulmonary lung transplantation in rare bronchopulmonary diseases and syndromes.

2    Lecture / Seminar - Diseases

Subject:       "pulmonary or thoracic manifestation with rheumatic diseases"

Summary: The questions of participation and influence of pulmonary disorders in rheumatic diseases and vasculitis, an evaluation of epidemiological data, deals with the diagnosis and screening of patients with connective tissue diseases.

                Introducing the disease induced by drugs - their aetiology, diagnosis and analysis of pictures of the diseases caused by individual drugs and pharmacology.

                As an example nosological understands:

·         Idiopathic pulmonary fibrosis - IPF.

                Understand the issues of definitions, details are given on the aetiology, epidemiology, clinical picture, diagnosis, treatment and prognosis.

3    lecture / seminar - Diseases

As nosological examples are:

·        Idiopathic interstitial pneumonia (IIPs)

Summary:    The questions of the actual classification, definition, presents data on the diagnosis and differential diagnosis of these conditions. We consider the acute and sub-acute state, as well as conditions associated with smoking. Is the question on the differential diagnosis of these states with cryptogenic operating call pneumonia.

                      Separate issues of rare idiopathic lymphocytic interstitial pneumonia

·        LIP and idiopathic pleuro-parenchymal fibro-elastosis

·        PPEF, and also the most rare condition and interstitial disorders that defy the classification.

4    lecture / seminar - Diseases

      As an example nosological highlights:

      Interstitial fibrosis, centred in the airways

·         Airway-cantered interstitial fibrosis

·         ACIF

Summary:    detailed analysis and study of the epidemiology, aetiology, pathogenesis, clinical forms, particularly diagnostic approaches and differential diagnosis. The data on the forecast and prospects of treatment.

            As examples of these clinical entities are also considered:

·        Sarcoidosis

·        Chronic berylliosis

·        Bronchocentric granulomatosis

·        Lymphomatoid granulomatosis

                      The analysis and study of the causes of the orphan states data, especially diagnostic approaches, the search for genetic markers. The problems of therapy, including examples and orphan medicines and their mechanisms of action. The problems of extra-pulmonary manifestations of sarcoidosis, in particular lymph node lesion, skin, eyes, bones, joints, muscles and nervous system. Separate deals with sarcoidosis of the heart, liver and spleen. We study the questions of therapeutic principles, flow control and forecasting.

5    lecture / seminar - Diseases

As an example nosological highlights:

·         Exogenous allergic alveolitis

·         extrinsic allergic alveolitis

·         EAA (amerik - Hypersensitivity pneumonitis)


Summary:    The questions of definitions, epidemiology, pathogenesis, clinical manifestations, acute and chronic forms of alveolitis. Conduct a detailed review of the diagnostic criteria: laboratory, functional, radiological, pathological differential diagnostic. Particular attention is paid to the research bronchoalveolar lavage and inhaled provocative test. A review of therapeutic interventions, prevention and prognosis.

A separate analysis of the forms that occur in people who work with poultry and light farmer "wet" light and the role of mycobacteria in the EAA development.

6    lecture / seminar - Diseases

Summary:    represents a further overview of the rare bronchopulmonary diseases with lighting issues epidemiological situation in the world and in Russia, as well as features diagnostical criteria. The problems of therapy and patient management data standards, including by orphan drugs.

         As nosological examples are:

·        Pulmonary histiocytosis of Langerhans cells,

·        Burt's syndrome-Hogg-Dube (Birt-Hogg-Dube-Syndrome)

·        Eosinophilic pneumonia and hypereosinophilic syndrome

A review of the epidemiology, aetiology and pathogenesis of conditions data, pathophysiology, classification, discusses various clinical pictures with the diagnosis and differential diagnosis. The data on the therapy, prognosis and prospects for the use of orphan preparations.

7    lecture / seminar - Diseases

As nosological examples are:

·        Pulmonary alveolar proteinosis

·        Amyloidosis

·        Pulmonary alveolar microlithiasis

·        Idiopathic pulmonary hemosiderosis

Summary:    The report focuses on questions of interstitial lung diseases of childhood, including epidemiology, particular symptoms, diagnostics, classification, methods of treatment and different patterns of diseases.

8    lecture / seminar - Diseases

As nosological examples are:

·        Primary ciliary dyskinesia

·        Syndrome Svayera-James-MacLeod (Swyer-James-Macleod-Syndrome)

·        Rare forms of asthma

·        Rare diseases of the trachea

Summary:    The questions of the actual classification, definition, presents data on the diagnosis and differential diagnosis of these conditions. Consider acute and sub-acute state, place them in the classification of this group of diseases.

In the study of issues tracheal diseases, particular attention is paid to the role of cartilage hyperplasia, polychondritis, osteochondroplasty. A review of both benign and malignant forms of rare diseases of the trachea and the methods of their treatment.

Additional Information:

In each lecture as a special application demonstrates the latest and several previously known orphan medicines in relation to specific rare bronchi-pulmonary diseases and syndromes.

We give medication status analyses, availability, and data of the leading pharmaceutical companies in the world that manufacture these drugs. To provide information about available today orphan medicines intended not only for treatment but also for the diagnosis and prevention of rare diseases and syndromes.