title: [Morphological alternations and hemostatic disturbances as manifestation of mesenchymal dysplasia in nephroptosis patients].
abstract: The examination of 36 nephroptosis patients (33 females and 3 males, mean age 23 years) included assessment of hemostasis before and after nephropexia (15 patients), histological investigation of renal parenchyma biopsies (15 patients) and those of the skin from the lateral abdominal surface and musculus lumborum fascia (5 patients). Changes characteristic for an early stage of microcystosis were found in all the renal tissue biopsies. In the skin and muscular connective tissue there were dystrophic changes. Collagen fibers were undergoing elastoid degeneration. As to hemostatic disorders, there were diverse platelet dysfunctions and/or thrombocytopenias, 9 patients had low plasma level of Willebrand's factor, 28 showed impaired terminal clotting. Combination of the above defects was registered in 12 of 36 examinees. After nephropexia, platelet count and fibrinogen level noticeably increased, hemostatic disorders aggravated. Thus, nephroptosis is manifestation of connective tissue pathology associated with marked hemostatic changes.
authors.forename: A I
authors.initials: AI
authors.lastname: Neĭmark
authors.forename: I E
authors.initials: IE
authors.lastname: Sibul'
authors.forename: T S
authors.initials: TS
authors.lastname: Taranina
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000150:complications; D002455:Cell Division; D005260:Female; D005340:Fibrinogen / Q000378:metabolism; D005500:Follow-Up Studies; D006487:Hemostasis; D006801:Humans; D007674:Kidney Diseases / Q000150:complications / Q000473:pathology* / Q000601:surgery; D008297:Male; D010974:Platelet Aggregation; D010976:Platelet Count; D012867:Skin / Q000473:pathology; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D015415:Biomarkers; D014841:von Willebrand Factor; D005340:Fibrinogen
vernacular_title: Morfologicheskie izmeneniia i narysheniia gemostaza kak proiavlenie mezenkhimal'noĭ displazii u bol'nykh nefroptozom.