Obilježja glomerularne bazalne membrane u Alportovom sindromu i nefropatiji tankih bazalnih membrana ; Characteristics of the glomerular basement membrane in Alport's syndrome and thin basement membrane nephropathy
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Medientyp:
E-Book
Titel:
Obilježja glomerularne bazalne membrane u Alportovom sindromu i nefropatiji tankih bazalnih membrana ; Characteristics of the glomerular basement membrane in Alport's syndrome and thin basement membrane nephropathy
Erschienen:
[Erscheinungsort nicht ermittelbar]: Sveučilište u Zagrebu. Medicinski fakultet.; University of Zagreb. School of Medicine., 2021
Sprache:
Kroatisch
Entstehung:
Hochschulschrift:
Dissertation, Sveučilište u Zagrebu. Medicinski fakultet.; University of Zagreb. School of Medicine., 2021
Anmerkungen:
Beschreibung:
Ciljevi istraživanja: Rasvijetliti kompleksni pojam ''tankih GBM-a'' i utvrditi ulogu imunohistokemijske analize na lance α3, α4 i α5 kolagena tipa IV pri razlikovanju nefropatije tankih glomerularnih bazalnih membrana (TBMN) od Alportovog sindroma (AS) i subtipiziranju AS-a. --- Ispitanici i metode: Provedeno je retrospektivno istraživanje na tkivu biopsija nativnih bubrega (130 ispitanika) i bubrežnih presadaka (90 ispitanika) uz provođenje standardiziranog mjerenja GBM-a i imunohistokemijske analize na lance kolagena tipa IV. --- Rezultati: Određen je referentni raspon uredne debljine GBM-a (muškarci 268 – 412 nm, žene 213 – 389 nm) te patohistološke i kliničke karakteristike ispitanika s AS-om, TBMN-om, TBMN-om udruženim s FSGS-om i hereditarnim nefritisom (pojam korišten za ispitanike koji su imali morfološke značajke između AS-a i TBMN-a). Postoji značajna razlika u kliničkim parametrima, svjetlosno-mikroskopskim i ultrastrukturnim značajkama između opisanih skupina. Udio tankih GBM-a u nultim biopsijama bubrežnih presadaka iznosi 31 % analiziranih uzoraka. Nema statistički značajne razlike između ispitanika s tankim GBM-a i bez tankih GBM-a u nultim biopsijama obzirom na kliničke varijable, stupanj kroničnih promjena prema Banff klasifikaciji i kliničkim pokazateljima 12 mjeseci nakon transplantacije. --- Zaključak: Imunohistokemijsko bojenje u 20 % ispitanika pridonosi subtipiziranju AS-a, a standardna devijacija mjerenja debljine GBM-a od 61 nm može pridonijeti razlikovanju AS-a i TBMN-a sa osjetljivošću od 80,7 % i specifičnošću 74,2 %. Za potpuno subtipiziranje bolesnika iz Alportovog spektra potrebna je uz patohistološku i kliničku također i genska analiza. ; Aim: To elucidate concept of thin GBM" and evaluate immunohistochemical staining for α3, α4 and α5 chains of collagen type IV in differentiation of thin glomerular basement membrane nephropathy (TBMN) from Alport syndrome (AS) and subclassification of AS. --- Patients and methods: A retrospective research of native (130 subjects) and transplanted kidney biopsy tissue (90 subjects) was performed with standardized GBM measurements and immunohistochemical analysis of the type IV collagen chains. --- Results: The reference span of normal GBM thicknesses (men 268 - 412 nm, women 213 - 389 nm) and the histopathological and clinical characteristics of patients with AS, TBMN, TBMN associated with FSGS and hereditary nephritis (a term used for patients who had morphological features between AS and TBMN) were determined. There is a difference in clinical parameters, light-microscopy and ultrastructural features among the groups. The prevalence of thin GBM in zero time kidney transplant biopsies is 31%. There are no differences between patients with or without thin GBM in clinical variables, Banff chronic changes scores and clinical parameters 12 months after transplantation. --- Conclusion: Immunohistochemical staining in 20% of patients contributed to AS subtyping. Standard deviation of average GBM thicknesses of 61 nm can contribute to differentiation of AS and TBMN with sensitivity of 80.7% and specificity of 74.2%. Pathohistological, clinical and also genetic processing is required to completely subtype Alport spectrum patients."