[Current concepts of oxalate nephropathies (clinical and population studies)]. / Sovremennye predstavleniia ob oksalatnykh nefropatiiakh (klinicheskie i populiatsionnye issledovaniia).

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.

[The modern concepts of hereditary nephritis]. / Sovremennye predstavleniia o nasledstvennom nefrite.

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.

[Health status of children born to women with renal pathology]. / Sostoianie zdorov'ia detei, rozhdennykh zhenshchinami s patologiei pochek.

The children born to mothers suffering from glomerulonephritis, pyelonephritis and hereditary nephritis were followed up for 4-5 and more years. This allowed a conclusion about a considerable rate of the birth of children with pathology of the urinary system organs (USO) and with diseases of other organs which were diagnosed for the first time at the age of 3-10 years as a result of goal-oriented investigations. The groups of the children born to women with renal diseases were marked by a high perinatal lethality studied by means of retrospective questionnaire. As for the structure of the USO diseases, the children manifested the predominance of the disease patterns associated with dysembryogenesis at the organ (anatomic abnormalities), tissue (dysplasia of the renal tissue) and at the cellular levels (metabolic nephropathies). In children born to women with hereditary nephritis, USO pathology was of the same kind and occurred only in the form of hereditary nephritis, which corresponds to the concepts of monogenously inherited pathology. The demonstration during pregnancy of a considerable rate of the environmental effects capable of exerting a damaging action on the embryonal development of children born to women with glomerulo- and pyelonephritis suggests a concomitant genesis of USO diseases in children born to mothers suffering from renal diseases. The authors discuss measures aimed at the prevention or reduction of the incidence of USO pathology in children as well as at the recognition of pathologies in children born to women with renal diseases at the predisease stage.

[Clinico-genetic characteristics of hereditary nephritis in different populations]. / Kliniko-geneticheskaia kharakteristika nasledstvennogo nefrita v raznykh populiatsiiakh.

Some data are presented on the clinical features of a course of hereditary nephritis in persons of 13 nationalities residing in the central zone of the RSFSR, in Central Asia and East Slovakia (the Czechoslovak Socialist Republic). At least 2 types of hereditary nephritis transmission (the dominant x-chromosome-linked one was more common than the autosomal-dominant one) not differing in their clinical course were revealed. Basing on a study of the clinical features of a course of disease in 123 autobred and 52 inbred families a more severe course of nephritis was observed in children from the inbred families.

[A method of separate determination of enzymuria associated with impairment of glomerular filtration in kidney tissue]. / Sposob razdel'nogo opredeleniia fermenturii, sviazannoi s povrezhdeniem glomeruliarnogo fil'tra i pochechnoi tkani.

Possibility of separate estimation of urinary enzymes after impairment of glomerular system and kidney tissue was considered theoretically. Activities of lactate dehydrogenase, alkaline phosphatase, aminopeptidase and gamma-glutamyl transferase were studied in blood serum and urine of II patients with nephrotic syndrome (4 patients with nephrotic form of glomerulonephritis and 7 patients with mixed form of glomerulonephritis). Urinary enzymes derived from blood serum constituted 20-30% of total enzymatic activity in urine of the patients with mixed form of glomerulonephritis and it was related only to the blood serum enzymes with relatively low molecular mass and high activity (alkaline phosphatase and LDH). The results obtained are consistent with the published data showing that kidney tissue is mainly responsible for appearance of the enzymes in urine.

[Clinical significance of enzymuria in glomerulonephritis in children]. / Klinicheskoe znachenie fermenturii pri glomerulonefrite u detei.

The authors presented the results of a study of enzymuria (cholinesterase, gamma-glutamine transferase, alkaline phosphatase, beta-galactosidase and lactate dehydrogenase with separate determination of N- and M-subunits) in 20 patients with a mixed form of glomerulonephritis (GN), 36 with the nephrotic form of GN and 13 patients with the hematuric form of GN. The clinical importance of the determination of enzymatic activity in the urine in GN of children lies in the recognition of the degree of damage of the glomerular filter as well as the nephrothelium. Basing on enzymuria pathophysiological syndromes found in various combinations in the above forms of GN were identified. Three degrees of damage of the permeability of the glomerular filter were defined for high molecular proteins. Differences in individual values of the activity of some enzymes gave rise to differential-diagnostic coefficients as well as differential-diagnostic tables which could be used for differential diagnosis between the GN mixed and nephrotic forms.