[Role of ultrasound dopplerography with acute captopril test in assessment of renal hemodynamics in chronic glomerulonephritis]. / Pol' ul'trazvukovoi dopplerografii s ispol'zovaniem ostroi farmakologicheskoi proby s kaptoprilom v otsenke pochechnoi gemodinamiki pri khronicheskom glomerulonefrite.

AIM: To examine blood flow in renal and intrarenal arteries and its changes in the acute pharmacological test with captopril in patients with chronic glomerulonephritis (CGN). MATERIAL AND METHODS: Renal circulation was studied in 50 patients with CGN using ultrasound dopplerography (USDG) of renal vessels on the unit GE Logiq 400 CL PRO Series. The velocity and indices of peripheral blood resistance in the major renal artery (RA) and in intrarenal arteries were estimated. In 26 patients the blood flow was studied again after intake of 50 mg captopril. RESULTS: Poor renal blood flow was registered in cortical parenchyma in 36% CGN patients (with chronic renal failure in 75%). Multifactorial regression analysis has demonstrated that only blood creatinine was independently related with slowing down of the blood flow at the level of RA and intrarenal arteries. Morphological index of activity correlated with resistance indices while a high sclerosis index correlated with blood flow slowing. Older patients had higher resistance indices. Captopril significantly accelerated blood flow and insignificantly changed indices of peripheral resistance including those in CRF patients. CONCLUSION: Poor blood flow in the cortical layer of renal parenchyma in CGN, according to USDG, occurs rather frequently and was associated with CRF and older age of the patients. Blocking of renin-angiotensin system at the level of angiotensin II formation improves renal blood flow in most of the patients.

[Use of a three-component scheme of immunosuppression on morphologic and immunohistochemical changes of transplants in the early postoperative period]. / Primenenie trekhkomponentnykh skhem immunosupressii na morfologicheskie i immunogistokhimicheskie izmeneniia transplantata v blizhaishem posleoperatsionnom periode.

Three-component (cyclosporin A, corticosteroids and azathioprine) immunosuppression has been widely introduced in the treatment of recipients of renal transplants because it allows a significant reduction of the components' doses in greater effectiveness. The analysis of the results of 83 puncture biopsies obtained in the immediate postoperative period after kidney transplantation has shown that administration of an imidazole derivative allows raising therapeutic concentration of cyclosporin up to 200-300 ng/ml, thus preventing rejection crises. However, increased blood concentration of cyclosporin does not increase its toxicity as a result of a significant fall in the overall level of the metabolites.

[The course and prognosis of mesangioproliferative glomerulonephritis]. / Techenie i prognoz mezangioproliferativnogo nefrita.

AIM: A retrospective analysis of a clinical course of mesangioproliferative glomerulonephritis (MPGN) in patients with glomerular deposition of IgA (IgA nephropathy--IgA-N), with glomerular deposition of other Ig to determine prognostic factors of MpGN progression including IgA-N and to examine the patients' sensitivity to immunodepressive therapy. MATERIAL AND METHODS: 2000 patients with primary MPGN followed up from 1980 to 1999 from the disease onset to development of chronic renal failure (creatinine > 2.5 mg%). Factors affecting kidney survival were studied using the Cox regression model, factors predicting sensitivity to immunodepressive therapy--using multiple logistic regression. RESULTS: IgA-N differed by the course and prognosis from other forms of MPGN. In IgA-N urinary syndrome and macrohematuria were encountered more frequently, in other forms of MPGN more frequent was nephrotic syndrome. Prognosis of patients with IgA-N was worse than in MPGN patients without IgA deposition: 10-year "renal survival" (creatinine < 2.5 mg%) was 64 and 97% (p < 0.05), respectively. Prognosis-deteriorating factors for MPGN patients were the following: male sex, nephritis onset in 40-year-olds and older subjects, acute nephritic syndrome (creatinine > 1.5 mg%), high proteinuria, hematuria (> 50 in sight), the presence of synechia and TIC in renal biopsy, location of immune deposits both in the mesangium and basal glomerular membranes. The responders to the immunodepressive therapy had 10-year renal survival 100%. Positive results of immunodepressive therapy were observed significantly more frequently in patients with normal level of creatinine, moderate hematuria, absence of synechias and TIC in renal biopsy, given large total course dose of corticosteroids and cytostatics. Efficiency of oral cyclophosphamide and its intravenous pulse-therapy did not differ significantly. In pulse therapy an average cumulative dose was lower 6 times, side effects occurred 3 times less frequently. CONCLUSION: The importance of morphological information for prognosis and predicting sensitivity of MPGN patients to immunosuppressive therapy necessitates renal biopsy before therapy. Intravenous pulse therapy with cyclophosphamide is preferable as an active treatment in patients with sclerosis in renal biopsy.

[Systemic AL-amyloidosis with extensive pulmonary and vascular lesions]. / Sistemnyi AL-amiloidoz s preimushchestvennym porazheniem legkikh i sosudov.

Here is an observation data on systemic AL-amiloidosis with primary lesion of lungs and vessels (a false hemorrhagic syndrome, ischemic insult in the region of middle cerebral artery). At the same time lesions of kidneys and heart became of minor significance. Amiloid lesion of lungs was characterized by unusual combination of symptoms: a galloping hydrothorax, a vast focus in the consolidation of pulmonary tissue with focuses of calcification, relapsing pneumothorax.

[Subdivision of certain morphological variants of chronic glomerulonephritis]. / O droblenii nekotorykh morfologicheskikh form khronicheskogo glomerulonefrita.

Modern methods allow to detail morphological classification of chronic glomerulonephritis, to adapt it to the clinical classification and to recommend it for practical use. This specification concerns minimal changes and a group of mesangial chronic glomerulonephritis. The term "minimal changes" is a light-microscopic definition and covers rather a heterogeneous group of diseases or their initial manifestations. Differential diagnosis of these diseases is feasible only at the electron microscopic level. A group of chronic glomerulonephritis (mesangioproliferative and mesangiocapillary) includes variants distinguished on the basis of immunohistochemical, light microscopic and electron microscopic methods. Of them, the immunohistochemical method is most valuable for differentiation of mesangioproliferative glomerulonephritis.

[Urinary fibronectin as an indicator of kidney fibrosis in nephritis]. / Fibronektin mochi kak pokazatel' protsessov fibrozirovaniia v pochke pri nefrite.

AIM: To investigate clinicomorphological relationships between elevated urinary excretion of fibronectin (FN) and development of fibrosis in the kidney in patients with lupus nephritis (LN) and chronic glomerulonephritis (CGN). MATERIALS AND METHODS: Urinary FN excretion was measured at radial immunodiffusion in 54 LN patients. Of them, 15 patients had inactive LN, 39 patients had active LN varying in clinical forms. Urinary FN was also measured by passive hemagglutination in 36 CGN patients (11 inactive CGN and 25 active CGN cases). Biopsy specimens were obtained from 49 patients with active nephritis (43 with CGN and 6 with LN). FN deposits were studied immunohistochemically and morphometrically with determination of relative fibrosis area. RESULTS: Urinary FN excretion in patients with nephritis was higher than in healthy controls. In active CGN and LN the levels of FN were significantly higher than in inactive CGN and LN. The highest FN urinary concentrations were registered in patients with severe CGN and LN, especially in the presence of renal failure and arterial hypertension. Among them, the highest individual values were observed in patients with rapidly progressive nephritis. No positive correlations were found between the degree of the urinary FN excretion increment and degree of proteinuria. This suggests local-renal origin of most urinary FN. Morphologically, FN deposits were revealed in 73% of the biopsies. In most of the patients with severe nephritis both in CGN and LN there was a diffuse distribution of FN in the glomerules and interstitium. A correlation with a morphological nephritis type was absent, but existed between FN presence in the renal biopsies and relative area of interstitium (fibrosis). CONCLUSION: FN excreted in high amounts with urine in nephritis originates from the kidneys and reflects severity of fibrogenesis in the kidney.

[Clinical and diagnostic significance of microalbuminuria and activity of tubular enzyme N-acetyl-beta-D-glucosaminidase (NAG) determination in patients with hyperuricosuria]. / Kliniko-diagnosticheskoe znachenie opredeleniia mikroal'buminurii i aktivnosti kanal'tsevogo fermenta N-atsetil-beta-D-gliukozaminidazy u lits s giperurikozuriei.

AIM: Development of the approaches to detection of hyperuricosuric stage of purin metabolism derangement and specification of methods for early diagnosis of urate damage to the kidney. MATERIALS AND METHODS: The study included 115 young subjects whose parents suffer from gout with renal involvement or isolated urate nephropathy. Each patient was examined clinically with evaluation of family history for gout risk factors. Three times for 9 months measurements were made of uricemia and uricosuria, microalbuminuria and activity of tubular enzyme NAG. RESULTS: 45 (39.1%) patients had neither disturbances of purin metabolism nor renal affection. 70 (60.9%) patients had hyperuricosuria. In 23 (32.9%) of them microalbuminuria increased to > 20 mg/day, NAG activity to 5 u/l. Chronic tubulointerstitial nephritis was diagnosed in 17 (73.9%) patients. Six patients (26.1%) developed asymptomatic affection of the kidneys. The rest 47 patients had normal levels of microalbuminuria and NAG. There were 3 cases of hyperuricemia with microalbuminuria rising to 160-200 mg/day and further development of urinary syndrome. Hyperuricemia in them was registered at microalbuminuria higher that 160 mg/day. CONCLUSION: Microalbuminuria higher than 20 mg/day and NAG activity higher than 5 u/day are important diagnostic indicators of renal affection in hyperuricosuria including asymptomatic one. Microalbuminuria above 160 mg/day gives grounds to discuss the role of morphological changes prior to proteinuria.

[Difficulties in the diagnosis of hereditary familial amyloidosis]. / Trudnosti diagnostiki semeinogo nasledstvennogo amiloidoza.

A 24-year-old woman had fever with skin rash, marked hepatolienal syndrome from the age of 1.5 years followed in later life by involvement of central nervous system and kidneys. Morphologically, there was accumulation of amyloid FAP in hepatic and rectal tissues. Family history of amyloidosis and the above evidence confirmed hereditary nature of the disease.

[Isolated amyloidosis of the islands of Langerhans and diabetic mellitus]. / Izolirovannyi amiloidoz ostrovkov podzheludochnoi zhelezy i sakharnyi diabet.

One observation of isolated amyloidosis of the insular apparatus of the pancreas followed by the development of diabetes mellitus and diabetic microangiopathy in various organs is described. Total amyloid involvement of all insular apparatus is pointed out.

[Morphologic characteristics of secondary tubulointerstitial nephritis in humans and in rabbit experiments]. / Morfologicheskaia kharakteristika vtorichnogo tubulointerstitsial'nogo nefrita u cheloveka i v éksperimente u krolikov.

Tubulo-stromal relationships were studied in rabbit and human kidneys in secondary tubulo-interstitial nephritis (TIN) of immune genesis to establish the species differences in the course and manifestations of this disease. TIN of rabbits was studied on the model of serum disease (13 animals) and in humans on renal biopsies from patients with mesangioproliferative GN (22 cases). The correlation of relative volumes of tubule structural elements characteristic for each species undergoes changes when pathologic process develops; new correlations are similar in both humans and rabbits, although the time of involvement of tubular part of nephron into the pathologic process is different. Immunohistochemically and electron-microscopically a diffuse character of the damage is shown.

[Characteristics of tubulo-stromal relationships in the kidneys of normal rabbits and humans]. / Osobennosti tubulostromal'nykh vzaimootnoshenii v pochkakh normal'nykh krolikov i cheloveka.

Extraglomerular cortex and medulla structural elements of kidneys, obtained from 10 male normal rabbits and 10 human beings (8 male and 2 female), who died from accidents at the age of 16-40 years, were studied in comparison. Morphometric studies with statistical evaluation are presented in diagrams, showing the percentage correlation of structural elements of rabbit and human kidneys. It is shown that the difference in life conditions of the studied species may result in the difference of these correlations The obtained results must be taken in to account in the comparative study of pathologic processes in the human and rabbit kidneys.

[Lesions of the digestive organs in Sjogren's disease]. / Porazhenie organov pishchevareniia pri bolezni Shegrena.

The clinicofunctional and morphological status of the stomach, biliferous system, pancreas, large and small intestine was studied in 83 patients with Sjogren's syndrome and disease (SS and SD). Chronic gastritis with secretory insufficiency was shown to develop in SD. Morphological changes of the gastric mucosa were represented by chronic gastritis with glandular involvement and chronic atrophic gastritis of immune genesis. Pathology of the extrahepatic system of bilification was detected in 87% of patients. The most common pathologies diagnosed in these patients were chronic cholecystitis (51%) and biliary dyskinesia (25%). Changes of the chemical composition of the bile (arise of its lythogenic properties) were observed. Various disorders of pancreatic function were detected in 85% of SS and SD patients. X-ray and endoscopic investigations revealed duodenal, intestinal and colon hypokinesia, less frequently signs of enteritis and colitis in one-third of the patients. The irritable colon syndrome was found in 40%. On the whole, involvement of different parts of the intestine was observed in 92%. Morphological changes in the duodenal and sigmoid colon mucosa were typical of chronic diffuse duodenitis and sigmoiditis in a subacute SD course, and chronic atrophic duodenitis and sigmoiditis in a chronic SD and SS course.

[Tubulointerstitial nephritis]. / Tubulo-interstitielle Nephritis.

A concept of the pathogenesis of tubular interstitial nephritis (TIN) is presented, based on histologic, immunohistologic and electromicroscopic investigations on 61 patients with glomerulonephritis, lupus erythematodes and rheumatoid arthritis. The pathogenic model is a hypersensitivity reaction which leads to alterations in the tubular basement membrane (TBM) and results in changes in the TBM and secondary damage to stroma, vessels and tubular cells. The findings change depending on the stage, reflecting the transition from an acute to a chronic process. In acute TIN which is generally a drug allergy, the dominant characteristic of the inflammatory process allows the reaction to be categorized in edematous (72%), cellular (23%) and tubular necrotic (5%) types. The cellular types show 4 subdivisions: plasma cellular, eosinophil-granulocytic, lymphohistiocytic and granulomatous. Chronic TIN is in particular characterized through the destruction of the TBM, dystrophy and atrophy of the tubules as well as their degeneration, lymphohistiocytic infiltrates and sclerotic stroma reactions. A distinction is made between cellular destructive and atrophic-sclerotic types. At view of the etiology and pathogenesis a distinction should be made between an infectious and non-infectious TIN and further by the character of the immunoreaction, into a primary and secondary form. If there glomerular changes are present and there is evidence of an immune phenomenon in the glomeruli and TBM we recommend the use of the term glomerulo-tubulo-interstitial nephritis.

[Acute tubulo-interstitial nephritis combined with uveitis (clinical case and review of the literature)]. / Ostryi tubulointerstitsial'nyi nefrit, sochetaiushchiisia s uveitom (klinicheskoe nabliudenie i obzor literatury).

The authors have presented a review of literature and their own data on acute tubulointerstitial nephritis combined with uveitis in a patient aged 47. In foreign literature this combination is defined as a separate syndrome (TIN-uveitis or TINU). The development of nonspecific symptoms (fatigue, weakness, nausea, loss of body mass, pains in the joints, skin rash) was characterized by change in some laboratory indices (raised ESP, anemia, eosinophilia, hyperproteinemia, hypergammaglobulinemia) and nephropathy. The signs of nephropathy were polyuria, a decrease in relative urine density, proteinuria of tubular genesis, renal glucosuria and aminoaciduria. A decrease in glomerular filtration and an increase in the level of blood serum creatinine were noted in most of the cases. In spite of the development of renal failure TINU prognosis is favorable: renal function gets back to normal by itself or after corticosteroid therapy. Uveitis shows a tendency to recurrence. The immune genesis of the TINU syndrome is assumed.

[Clinico-morphological characteristics of thymomegaly]. / Kliniko-morfologicheskaia kharakteristika timomegalii.

48 autopsy cases of thymomegaly in children up to 14 years and in adults from 18 to 55 years are studied. The patients died either from the acute or chronic infectious diseases resistant to the therapy or suddenly during or several hours after the diagnostic manipulations, small surgical interventions. Analysis of clinical data and morphological immunohistochemical and ultrastructural studies of the thymus and organs of the immune and endocrine system showed the thymomegaly in the majority of cases to be a local manifestation of the endocrine-immune deficiency syndrome. Primary (congenital) and secondary (acquired) thymomegaly are to be distinguished. A primary thymomegaly occurs in children and adults having congenital pluriglandular endocrinopathy with hypocortisism as well as an adrenal hypo- or aplasia with an antigenic stimulation. A secondary thymomegaly occurs in children of the first year of life and reflects the first phase of the thymic response in the activation of the immune T-system predominantly prolonged due to the immature state of the hypothalamico-hypophyseal-adrenal system. In the adults secondary thymomegaly occurs in cases of the acquired endocrinopathy with hypocortisism.