Amiloidoza Tip Lant Usor de Imunoglobulina Probleme de Diagnostic

|PARTEA GENERALA                                                          |1  |
|                                                                         |   |
|Introducere                                                              |2  |
|                                                                         |   |
| CAPITOLUL I. Amiloidoza :o cale finala comuna pentru depozitarea        |   |
|patologica a proteinelor in ţesut.                                       |3  |
|I.1. Proprietaţile fizico-chimice ale fibrilelor de amiloid              |3  |
|I.2. Clasificarea amiloidozelor                                          |5  |
|I.2.A.1. Amiloidozele primare                                            |6  |
|I.2.A.2. Amiloidozele secundare                                          |7  |
|I.2.A.3. Amiloidozele ereditare                                          |7  |
|I.2.A.4. Amiloidozele asociate cu beta 2 microglobulina                  |15 |
|I.2.B.1. Amiloidozele ereditare localizate                               |15 |
|I.2.B.2. Amiloidozele non-ereditare localizate                           |18 |
|                                                                         |   |
|CAPITOLUL II. Amiloidoza tip lant usor: bazele structurale ale patologiei|   |
|asociate lanţurilor uşoare                                               |19 |
|II.1. Clasificarea bolilor prin tulburări de conformaţie a               |   |
|  proteinelor                                                            |19 |
|II.2. Caracterizarea moleculara a lanţurilor uşoare                      |20 |
|II.2.1. Genetica lanţurilor uşoare                                       |20 |
|II.2.2. Structura lanţurilor uşoare                                      |23 |
|II.3. Fibrilogeneza in amiloidoza tip lant usor                          |26 |
|                                                                         |   |
|CAPITOLUL III. Amiloidoza tip lant usor: diagnostic si strategie         |28 |
|terapeutica                                                              |   |
|III.1.Manifestari clinice si caracteristici fiziopatologice              |28 |
|III.2.Diagnostic                                                         |31 |
|III.3.Strategii terapeutice                                              |34 |
|III.3.1. Reducerea sau anihilarea productiei precursorilor de amiloid    |35 |
|III.3.2.Terapia de inhibare a amiloidogenezei si stimularea resorbtiei   |   |
|depozitelor de                                                           |   |
|amiloid                                                                  |37 |
|III.3.3.Terapia suportiva a functiei organelor lezate prin depunere de   |38 |
|amiloid                                                                  |   |
|                                                                         |   |
|PARTE SPECIALA                                                           |   |
|                                                                         |   |
|OBIECTIVELE TEZEI                                                        |41 |
|                                                                         |   |
|CAPITOLUL IV: MATERIALE SI METODE                                        |42 |
|IV.1. Pacienţi : lot si protocol de investigaţii clinice                 |43 |
|IV.2. Examen histopatologic si imunohistochimic                          |44 |
|IV.3.Identificarea componentului monoclonal                              |45 |
|IV.4.Analiza si sinteza datelor                                          |45 |
|                                                                         |   |
|CAPITOLUL V.Analiza datelor clinice: rezultate si discutii               |48 |
|V.Rezultate si discutii                                                  |49 |
|CAPITOLUL VI  .Concluzii                                                 |72 |
|BIBLIOGRAFIE                                                             |73 |
                                        CUPRINS
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