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Biomedical subjects

M S Ignatova

Publications and source records attributed to M S Ignatova.

At least 19 recordsLinked to original sources

[Current ideas of hereditary nephropathies].

The paper describes a number of hereditary nephropathies, including hereditary nephritis (Alport's syndrome), the most common genetically determined renal disease, in the context of recent genetic studies. The specific features of tuberous sclerosis, a systemic disease inherited in a monogenic manner are outlined. Dysmetabolic nephropathy with oxalate-calcium crystalluria is presented as an example of multifactorial pathology.

Chromosome Aberrations↗

[Ecopathology of the kidneys and individual sensitivity to heavy metal salts].

Investigations performed in the region contaminated with heavy metal salts revealed high prevalence of renal diseases in children. The test for blood polymorphic proteins indicated signs of genetic predisposition to renal damage. Greater occurrence in the population with econephropathy of a rare allele of transferrin C3 may be the cause of enhanced oxidative-radical processes in renal cells. Individual sensitivity of children to heavy metal salts assessed by leukocytolysis and high incidence of somatic mutations to determine T-lymphocyte microclones deficient by HGPRT may help in specification of the affections detected in the regions contaminated with heavy metal salts.

Adolescent↗

[Nephropathies in a region contaminated by heavy metal salts and the possibilities for therapeutic and prophylactic measures].

The study of the population in the region contaminated with heavy metal salts has revealed high incidence of nephropathies even in preschool children manifesting initially in the majority of cases with hematuria. All the patients had the signs of urinary dysembryogenesis and marked membranopathological process. Long-term exposure to even small doses of heavy metals is supposed to cause nephropathy. Urinary disease arose more frequently in those genetically predisposed to renal and urinary tract affections. Because urolithiasis is a frequent result of dismetabolic nephropathy in endemic regions, it is advisable to perform active monitoring of children with environmental nephropathy using membrane-stabilizing measures. Optimal for these purpose could be xidifon. Further studies are needed to elucidate the problem of rapid elimination of heavy metals with chelating agents.

Adolescent↗

[The first experience in Russia of using DNA diagnosis in Alport's syndrome in a family with a unique morphological picture of the kidney lesion].

Clinicomorphological findings are reported for two children from families with hereditary predisposition to hematuria characterized by early occurrence of chronic renal insufficiency, neurosensory hypoacusis, congenital ocular abnormalities inherited by sex-linked dominant type. Light microscopy of nephrobiopsies revealed diffuse mesangial proliferation in both children. Final diagnosis of Alport's syndrome was feasible only on molecular-genetic level after polymerase chain reactions had identified mutation in collagen type 4 alpha-5-chain gene on a long arm of X-chromosome in genotypes of both patients and their mothers. Genetical, clinical, morphological, evolutional and diagnostic aspects of Alport's syndrome are reviewed.

Adolescent↗

[Plasmapheresis in the combined therapy of progressive forms of glomerulonephritis].

29 patients aged 6-16 with glomerulonephritis lasting 4-5 years received multimodality treatment with plasmapheresis as a component. The majority of the patients suffered from primary glomerulonephritis in mesangio- or membrano-proliferative morphological variants. Previous long-term conventional therapy (prednisolone, cytostatics, anticoagulants and antiaggregation drugs) failed. The test course comprised 1-3 plasmapheresis sessions (centrifuge method on [symbol: see text] apparatus), cyclophosphamide or maintenance methyl-prednisolone pulse therapy, heparin and curantil. One-third of the patients achieved remission lasting from 5 months to 3 years, in the other one-third the improvement was as short as 2-4 weeks, and the last one-third appeared non-responders. Improvement of clinical indices occurred in parallel with trends to reduction in the levels of CIC, IgG, B-lymphocytes, T-helpers, inhibition of lymphocyte succinate dehydrogenase activity, better phagocytosis. No complications which may prohibit plasmapheresis use in glomerulonephritis were observed. Adjuvant plasmapheresis use in glomerulonephritis treatment needs further studies.

Adolescent↗

[Current concepts of oxalate nephropathies (clinical and population studies)].

The paper presents the results of clinical and laboratory examination made in 3 groups of children: populational, hospital and control (a total of 176 patients). The children were diagnosed to have variants of dysmetabolic nephropathy (DN) which had become a problem not only for urolithiasis-endemic regions, but also for the Middle Russia. The study involving characterization of cytomembranes, renal tissue biopsy allowed conclusion on nonspecific DN symptoms. Obligatory symptoms were those of OCC, microhematuria and/or mild proteinuria, changes in cytomembranes, weak tubular function, tubulo-interstitial changes. DN genesis is thought multifactorial, involving genetic predisposition, biochemical defects, ecological hazards.

Adolescent↗

[The modern concepts of hereditary nephritis].

The authors describe the results of modern studies into the problems of genetics, clinical picture, prognosis and prospects of the treatment of inherited nephritis. It is assumed that at the basis of inherited nephritis there lies generalized impairment of the basal membranes, which is determined by mutation of X chromosome that codes the structure of the chains of the fourth fraction of collagen. The phenotypic heterogeneity of the disease is accounted for by mutation of different alleles in a solitary locus. The clinical characteristics of inherited nephritis without hypoacusis and Alport's syndrome in inbred and outbred families is provided as are specific features of the disease evolution. The results and efficacy of kidney transplantation in patients with inherited nephritis in the phase of chronic renal failure are discussed.

Biopsy↗

[Study of serum levels of alpha 1-antitrypsin and alpha macroglobulins in children with glomerulonephritis].

To define the clinico-pathogenetic importance of alpha 1-inhibitor of proteinases and alpha 2-macroglobulin of the blood in children with glomerulonephritis, a study was made of the phenotype of alpha 1-inhibitor of proteinases and its concentration in the blood serum of 156 patients with different clinical forms of glomerulonephritis. Overall 1290 practically healthy children were examined as control. The patients suffering from glomerulonephritis did not demonstrate phenotypes responsible for acute deficiency of alpha 1-inhibitor of proteinases (PISS, PISZ). A relationship was established between the amount of alpha 1-inhibitor of proteinases in the blood serum in children with different clinical forms of glomerulonephritis: the patients with the nephrotic form manifested a significant decrease of the inhibitor concentration in the blood serum, whereas in the hematuric form, a significant rise of it was recorded. All the patients suffering from glomerulonephritis showed a significant increase of the content of alpha 2-macroglobulin, particularly in the nephrotic form, which is likely to be determined by the enhanced output of the given protein and its negligible loss with urine in connection with a high molecular weight.

Adolescent↗

[Hormonal interactions and glucocorticoid receptors in patients with the nephrotic syndrome].

As many as 27 children aged 6 to 15 years with morphologically verified nephropathies were examined. Four variants of changes in the thyroid status, characteristic of children with different variants of nephrotic syndrome were distinguished: 1) biochemical signs of primary hypothyroidism, 2) biochemical signs of secondary hypothyroidism, 3) low content of T3, 4) dysfunction of the hypophyseal and thyroid system. It is shown that the low level of steroid receptors, thyroid hormones that the low level of steroid receptors, thyroid hormones (T3 and T4) and cortisol is typical of children with the signs of renal dysplasia. It is assumed that superaddition under such conditions of immune glomerulopathy (glomerulonephritis and nephrotic syndrome) gives rise to the resistance to the treatment with glucocorticoids.

Adolescent↗

[The first experience of using membranotropic agents in the treatment of the nephrotic syndrome].

To treat children suffering from the nephrotic syndrome, use was made of the membrano-stabilizing agents: zaditen that also has an antiallergic action; dimephosphon, a membrano-stabilizer and immunomodulator. The basis for differentiated use of the drugs was formed by the examination data which enabled one to identify the signs of atopy in children with the hormone-dependent nephrotic syndrome, marked signs of the instability of cellular membranes and different immunologic deviations in children with the hormone-resistant variety of the nephrotic syndrome. During zaditen treatment, the majority of the children with the hormone-resistant nephrotic syndrome manifested an appreciable decrease of the process activity; in some cases, including those with hormone dependence, the treatment with prednisolone could be reduced. In part of the children with the hormone-resistant nephrotic syndrome, the treatment with dimephosphone resulted in a decrease of proteinuria, reduced the instability of cellular membranes, and improved the immunologic parameters.

Adjuvants, Immunologic↗