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[Morphologic contribution to a progeria syndrome (Hutchinson-Gilford) (author's transl)].

Report of necropsy findings of a 17 years old girl with a progeria syndrome. There was a high degree of generalized atherosclerosis, involving the visceral arteries, chiefly those to the kidneys. The patient died of nephrosclerosis with uraemia. The aetiology of progeria syndrome remains obscure, single findings (lipoprotein- and amino-acid metabolism) may be understood as an inborn error of metabolism.

Adolescent↗

Interdialysis blood pressure control by long haemodialysis sessions.

High blood pressure (BP) is a major factor contributing to the high incidence of cardiovascular morbidity and mortality in haemodialysis (HD) patients. According to predialysis casual BP measurements, long HD has been shown to provide good BP control. To confirm this result during the period between dialysis sessions, we performed ambulatory monitoring of BP in 91 non-selected HD patients (mean age, 58.7 (14.1) years; 14% incidence of nephrosclerosis and diabetes mellitus; treatment duration, 93.0 (77.2) months; 3 x 8 h/week, cuprophane, acetate buffer in 95% of the patients). Only one patient (1.1%) was receiving an antihypertensive medication. Ambulatory BP results were systolic (S) BP, 119.4 (19.9) mmHg; diastolic (D) BP, 70.6 (12.9) mmHg; mean (M) BP, 87.6 (13.9) mmHg. These values were significantly lower than the casual predialysis BP data and close to the reference values reported by Staessen et al. in a meta-analysis including 3476 normotensive subjects. The MBP was inversely correlated with the treatment duration, but not with interdialysis weight gain. The MBP increased significantly in the last part of the interdialysis period, and this rise was not correlated with the interdialysis weight gain. The nocturnal/diurnal ratios for SBP and DBP for the HD patients (0.97 and 0.92) were higher than the reference values reported by Staessen, (0.87 and 0.83), and argued against a nocturnal decrease in BP. We found that 52.1% of the patients had an abnormal nocturnal BP fall (MBP fall < 5%). This feature worsened during the second night of the interdialysis period.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Pressure Monitoring, Ambulatory↗

[Histopathology of the biopsied kidney and its related glomerular deterioration].

This paper presents the incidence of glomerulonephritis (-pathy: GN) from a large number of more than 10,000 serial renal biopsies examined in one laboratory using the same criteria over the past 25 yrs. in Japan. Each incidence is as follows: IgA nephropathy (IgAN), 33%; thin glomerular basement membrane disease (TBMD), 17.8; athletic pausal urinary abnormality (APUA), 8.2; primary membranous glomerulonephritis (memb GN), 6.5; while all others were less than 5% each. Out of 3,300 IgAN cases, 51% consisted of a minimal change IgAN (MCIgAN), while the IgAN cases with moderate to severe glomerular damage comprised 20% of all cases. In addition, the survival curves of the IgAN cases coincided with those of FGS and benign nephrosclerosis (BNS) with a similar extent of glomerular damages. On the other hand, glomerular damage mostly occurred due to intra- and intercapillary cell infiltration; poststreptococcal GN (AGN), lupus N, Cat. III a IV a and IV b, and MCIgAN all had a favorable outcome (follow-up mean: 12.4 +/- 6.7 yrs). More than 50% of the dialized patients came from both IgAN, focal type with hypertension, and IgAN with more than moderate glomerular damage regardless hypertension. The incidences of AGN, MPGN, HBvN have all decreased at the present time, while renal amyloidosis and crescentic glomerulonephritis (Cres. GN) have increased in number and this is reflected by the increased number of renal biopsies in elderly men. Glomerular deterioration is thus considered to be caused more by non-immunologic and hemodynamic injuries than by immune-derived, repeated inflammation, in human chronic glomerulonephritis(-pathy).

Biopsy↗

[Histopathological assessment of renal biopsy specimens of subjects with urine abnormality].

Between April 1985 and April 1992, 25,672 men (age 47 +/- 9 years, mean +/- SD) and 9,791 women (48 +/- 9 years) underwent mass urinalysis in the Center for Adult Complete Physical Examination in our hospital. The results revealed proteinuria in 6.3% of the men and 4.4% of the women and hematuria in 17.4% of the men and 37.8% of the women. Thirty-five subjects with asymptomatic persistent proteinuria and/or hematuria identified as a result of follow-up testing by the nephrologists at our hospital underwent renal biopsy. All of the biopsy specimens obtained were examined by light microscopy, fluorescence microscopy and electron microscopy. Histopathological findings in the biopsy specimens from these 35 subjects were as follows: One case (3%) of chronic pyelonephritis, 11 cases (31%) of IgA nephropathy, 4 cases (11%) of IgA nephropathy (severe type), 5 cases (14%) of membranous nephropathy, 4 cases (11%) of thin basement membrane disease (TMD), 7 cases (28%) of benign nephrosclerosis and 3 cases (9%) of minor glomerular abnormality. Light microscopy, fluorescence microscopy and electron microscopy for histopathological assessment of renal specimens, especially for the diagnosis of TDM, which was not uncommon, were indispensable tools in our study. Moreover, it is essential for proteinuria and hematuria to be tested simultaneously using the same standard method in all the urine specimens collected.

Adult↗

[Adverse effects of smoking from the nephrologic viewpoint].

Nicotine abuse may cause severe nephrourologic diseases such as nephrosclerosis, hypertension and tumour of the bladder. The major factors for urinary tract diseases are hypoxaemia and cancerogenic substances. Aetiology and prophylaxis of malignant bladder tumour is discussed.

Humans↗

Light-chain-induced renal tubular acidosis: effect of sodium bicarbonate on sodium-proton exchange.

We measured sodium-proton (Na+/H+) exchange in lymphocytes and platelets of a 46-year-old woman with the adult Fanconi syndrome before, during, and after treatment with NaHCO3. Kappa light chains in her urine and unique but rarely observed crystalline structures confirmed the presence of light-chain nephropathy. Her glomerular filtration rate was only moderately impaired at 72 ml/min. NaHCO3 at 1, 3, and 5 mmol/kg/day for 5 days increased her serum HCO3 and pH from 17 to 21 mmol/l and 7.28 to 7.39 respectively. Plasma renin and aldosterone values were decreased by NaHCO3. Na+/H+ exchange (delta Hi/min) was measured with the fluorescent marker BCECF after acidification of lymphocytes and platelets with sodium propionate at five (10-50 mM) doses. Na+/H+ exchange was accelerated in this patient compared to normal controls. NaHCO3 treatment significantly decreased Na+/H+ exchange in lymphocytes, but not in platelets. These findings suggest that Na+/H+ exchange can be influenced by NaHCO3 ingestion at doses that only modestly affect systemic pH. Since Na+/H+ exchange is involved in stimulus response coupling, cell growth regulation, cell differentiation, and perhaps the progression of nephrosclerosis, these observations may have clinical relevance.

Acidosis, Renal Tubular↗

Renal insufficiency due to angiotensin-converting enzyme inhibitors.

Angiotensin-converting enzyme (ACE) inhibitors represent a major therapeutic breakthrough for treatment of hypertension, congestive heart failure and various chronic renal diseases. They are effective generally well tolerated and safe for most patients. However, acute renal insufficiency or overt renal failure occurs in some patients with underlying critical renal artery stenosis (RAS), hypertensive nephrosclerosis, autosomal dominant polycystic kidney disease, diabetes mellitus, and chronic congestive heart failure. Diuretic-induced sodium depletion and underlying chronic renal insufficiency are the major predisposing factors for renal insufficiency in all of these patient populations. Renal insufficiency is usually asymptomatic, nonoliguric, associated with hyperkalemia, and in nearly every case completely reversible after discontinuation of the offending agent. Moreover, it can usually be managed in the outpatient setting by discontinuation of the ACE inhibitor, concomitant diuretic or both. An asymptomatic increase in serum creatinine in patients administered ACE inhibitors should raise the possibility of RAS; however, more common renal diseases should be considered. The decision to pursue testing for RAS should be done on an individual basis; moreover, it is imperative that patient willingness to undergo invasive procedures including angioplasty and/or surgery should be determined prospectively.

Acute Kidney Injury↗

Should elderly donors be accepted in a live related renal transplant program?

It is a matter of concern that the elderly donor may have increased risks in the peri-operative period due to age-related changes in various organ. Nephrosclerosis, atherosclerosis and low GFR of an elderly kidney may portend a poor graft outcome. A retrospective analysis of our live related renal transplant program (from June 1989 to December 1993) revealed that 27 of the donors were above 60 years of age. 21 of the recipients have been followed up for more than 1 year. These patients were compared with a cohort of 25 patients (donor age < 45 years) with similar HLA match, immunosuppressive protocol, and follow-up period more than 1 year. Graft survival at 1 year was 86% and 88% in the recipients from elderly and younger donors respectively; 1 patient in the control group died of fulminant sepsis. Mean follow-up was 21.6 months in the study group and 22.8 months in the control group. Allograft function was evaluated by serum creatinine and differential GFR by Tc DTPA scan. Serum creatinine (mg%) was 1.3 +/- 0.2 and 1.4 +/- 0.2 in the study group and 1.3 +/- 0.3, 1.2 +/- 0.3 in the control group at 3 and 12 months respectively. Glomerular filtration rate (ml/min) was 36.5 +/- 11.6 and 43.7 +/- 12.4 in the recipients from elderly donors whereas those from the younger donors had GFR (ml/min) of 40.6 +/- 9.6 and 49.6 +/- 14.2 at 3 and 12 months respectively, GFR continued to improve in both groups with follow-up. There was no difference in incidence or severity of ATN In the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Proteinuria and hypertension.

Albuminuria is more prevalent in patients with primary hypertension than in normotensive subjects of the general population. The presence of albuminuria predicts the presence of more severe target organ damage and is related to the risk of cardiovascular events. Preliminary results show albuminuria even in some normotensive individuals with a genetic risk of hypertension and in association with insulin resistance. While albuminuria is generally more frequent in the elderly, it is also found in young patients with mild to moderate primary hypertension. It is uncertain whether in these circumstances albuminuria indicates some "renal component" in the etiology of primary hypertension. Massive albuminuria may occur in subjects with "benign" nephrosclerosis. Whether albuminuria is a predictor of hypertensive renal damage requires further study. Albuminuria is reduced by antihypertensive treatment, but diverse effects on albuminuria are seen with different antihypertensive agents.

Adult↗

The long-term effect of primary disease on cadaver-donor renal transplant recipients.

1. Graft survival was similar at one year for the various diseases, but at 3 years, a 16% divergence was noted among diseases. IGAN patients had the highest graft survival rate. 2. Graft survival rates of IGAN, ALP, and PC in Black and White patients were similar, but in all other diseases, a high loss rate was seen after one year among Black patients. 3. Patient survival was almost identical for the various diseases among Whites and Blacks. 4. SLE patients with DR2 or DR3 had higher graft survival rates than SLE patients without these groups (p < 0.05 in Whites). 5. IDDM patients with DR3 or DR4 had higher graft survival rates than IDDM patients without these groups (p < 0.05 in Whites, p = ns in Blacks). 6. Nephrosclerosis patients with DR2 or DR4 had higher graft survival rates than those who did not (p = ns in Whites, p < 0.05 in Blacks). 7. CGN patients with DR1 had higher graft survival rates than CGN patients without DR1 (p < 0.00005 in Whites). 8. IDDM patients with SPK transplants had higher graft survival rates than IDDM patients grafted with a KAT (p < 0.000001). In recent years, almost 30% of IDDM patients had SPK transplants. 9. Patients with SPK grafts compared to KAT were younger, White, were more often DR3/4, and worked full-time. 10. The SPK effect was seen only at the excellent centers. At all other centers, SPK and KAT patients had the same graft survival rates.

Adolescent↗

[The correction of primary megaureter in children. The late results].

The surgical intervention on 261 ureterovesicular segments was performed in 202 patients aged 3 months-14 years. All the patients presented with megaureter varying in forms: refluxing megaureter (147 cases), nonrefluxing megaureter (94 cases), megaureter with ureterocele (20 cases). Histological investigations demonstrated congenital structural changes of the same type to be a morphological basis of diverse megaureter forms. These structural changes involved muscular and connective tissue elements of the ureteral wall. Being qualitatively continual, the components varied in quantity thus producing different maldevelopments. Urodynamic dysfunctions depended on ureteral dysplasia severity and form. Clear-cut outlines in the x-ray image of the refluxing, nonrefluxing and megaureter with ureterocele resulted from the condition and structure of the ostium ureteris and its submucosal segment. The same pathogenetic origin of megaureter forms gave grounds for conducting operations of the same type, resection of the defective ureter along with the ostium and its replacement for a morphofunctionally competent segment with formation of another ostium. The response to the surgery was assessed in 196 patients (249 ureters). The follow-up lasted from 1.5 to 22 years. Good responses were achieved in 90%, satisfactory in 3.2%, poor in 6.8% of the surgical patients. High efficacy of the operation says in favour of its pathogenetic validity. The megaureter correction created favourable conditions for renal growth and development. However, in 7 cases the improvement has changed for progressive nephrosclerosis with decline in the organ function or arterial hypertension. The ureter in such cases was in stable good condition. The above observations suggest the necessity of further investigations of this problem and continuation of the patients' follow-up.

Adolescent↗

[The nephrotoxic action of heavy crude with a high vanadium content and of its refinery products].

The samples of vanadium-rich oil obtained from the field in Buzachi peninsula and of this oil products were applied subcutaneously to hybrid mice. In response to the application 27.5-56.6% of the animals developed renal impairment manifest morphologically in the range from acute proliferative glomerulonephritis to nephrosclerosis. Nephrotoxicity of the products increased with elevation of their sublimation temperature. Vanadium concentrations grew respectively. Intraperitoneal introduction of the products to rats provoked acute intracapillary glomerulonephritis with partial tubular and glomerular necrosis associated with acute renal failure.

Animals↗

[The results of the organ-preserving surgical treatment of patients with kidney cancer].

42 patients underwent conservative surgery for renal cancer. Conservative surgery was applied in 6 patients with only kidney, in 17--with unilateral cancer and contralateral kidney damaged by urological disease, in 17--with simultaneous tumorous process and in 8 patients with unilateral renal cancer and sound contralateral organ. Terms of postoperative follow-up ranged from 9 to 121 months, 14 patients have died for this period, exacerbation of cancer or metastasis being the cause of death in 9 of them. 5-year survival rate made up 64.4% although with regard to deaths related to cancer it equaled 81%. In 5 patients operated kidneys demonstrated complete functional failure due to nephrosclerosis. 5-year survival rate correlated inversely with advance of the process and was higher in unilateral lesions as compared to that of patients with bilateral tumors.

Adenocarcinoma↗

Pressor effect of recombinant human erythropoietin: results of home blood pressure measurements in hemodialysis patients.

We investigated whether the treatment of anemic hemodialysis patients with a low dose of recombinant human erythropoietin (rHEpo) for a short period would increase the blood pressure (BP). Home BP measurements were used to detect minute increases in BP. Fifty-one anemic patients on maintenance hemodialysis with a hematocrit of 25% or less received rHEpo at the dose of 4500 IU/week by the intravenous route for 8 weeks. Overall, rHEpo did not increase the BP whether measured at home or in the clinic (causal BP). Hemoglobin concentration increased significantly from 7.1 +/- 0.7 to 8.8 +/- 0.7 g/dl. Patients were classified into two groups according to the change in mean (M) home BP induced by rHEpo: a pressor group (delta MBP > or = 5 mmHg, n = 17) and a non-pressor group (delta MBP < 5 mmHg, n = 34). The hemoglobin concentration rose significantly in both groups, but there was no change in casual BP. Home blood pressure measurements showed a gradual and continuous rise in BP in the pressor group, but not in the non-pressor group. Patients administered antihypertensive medications before rHEpo treatment accounted for 88% of the former and 50% of the latter groups. Two patients with malignant nephrosclerosis were included in the pressor group. The findings indicate that rHEpo, even given at a low dose for a short period, elevates the BP, as determined by home BP measurement, but not by casual measurements obtained in the clinic.

Adult↗

[Clinical analysis of cases with acute exacerbation of chronic renal failure].

We analyzed the total number of 42 events of acute exacerbation of chronic renal failure (CRF) in 35 patients who were admitted to the Matsuyama Red Cross Hospital Kidney Center between 1985 and 1990. These patients consisted of 16 males and 19 females with a mean age of 59.8 +/- 12.7 years. The most frequent original renal diseases were diabetic nephropathy (DM) and arteriosclerotic nephrosclerosis (NS). The most frequent acute exacerbation factors were dehydration and infection. Four cases died and one case transferred to chronic hemodialysis, but the other 37 events (88%) in 31 cases recovered to normal renal functioning. Tentative dialysis therapy was performed for 12 events, in which the cases with DM as the original disease and infection as the exacerbation factor were responsive to this therapy. In the analysis of long-term prognosis, 7 out of 26 cases died of non-renal diseases, 15 transferred to chronic dialysis therapy and only 4 remained in a CRF state. The intervals between the date of discharge and that of the start of dialysis therapy was shorter in the cases with DM (mean of 5 months) or chronic glomerulonephritis (mean of 7.6 months) than in cases with chronic interstitial nephritis, polycystic kidney disease and NS (mean of 17.7 months). We concluded that even though many of the cases eventually transferred to chronic dialysis therapy, appropriate therapy during the acute exacerbation period could allow recovery to a natural progressive or nonprogressive course in each case.

Acute Kidney Injury↗

[Nephropathy in pregnancy--an endothelial lesion?].

Morphological examination of renal biopsies from 90 women with preeclampsia (PE), assessment of the clinical data and clinicomorphological correlations produced the following results: 1. By light-microscopy the renal lesions in PE imitate a picture of glomerulonephritis of mesangial type with different degrees of severity. 2. Morphometric investigations confirmed the impression gained by light-microscopy of swelling of endothelial cells and podocytes as well as endocapillary cell proliferation and enlargement of the glomeruli. 3. The immunohistological findings are non-specific and argue against immune complex deposition, but are suggestive of insudative processes. In addition immunohistological investigations of fibronectin and factor VIII-associated antigen reveal a pathogenetic relevant alteration of endothelial cell. 4. Electronmicroscopy is the most valid diagnostic method allowing subdivision of the quantitative different lesions in various degrees of severity. Furthermore the use of this method allows elucidation of the dynamics of the underlying disease process, which progresses through successive stages i.e. early, fully developed and late stage, supporting the reversibility of these glomerular lesions. 5. Close correlations are found between the clinical parameters and morphological findings in nephropathy in pregnancy-induced hypertension. The hypertension, proteinuria and nephrotic syndrome, which characterize the clinical picture, correlate with the severity of the glomerular lesions and the further course of the disease. Moreover, hypertension also correlates with mesangial and subendothelial deposits and with focal segmental hyalinosis and sclerosis, occurring in some cases. The focal segmental hyalinosis and sclerosis should be regarded as hyperperfusion-lesions indicating benign nephrosclerosis and developing only facultatively in PE. 6. The first morphological substrate of nephropathy in pregnancy-induced hypertension with the key to pathogenesis present itself as endothelial lesion, possibly caused by oxygen free radicals, lipid-peroxides or hyperfusion. In result of the endothelial lesion an imbalance of the different mediator systems i.e. thromboxane-prostacyclin, endothelin-EDRF with dominance of vasoconstrictive reactions would be effective. Thus the following induction of coagulative, vasoconstrictive and proliferative processes results in the characteristic glomerular lesions in PE.

Adult↗

Prednisone withdrawal in HLA identical and one haplotype-matched live-related donor and cadaver renal transplant recipients.

Prednisone withdrawal was attempted in 121 of 915 renal transplants recipients between 1967 to 1992. There were 57 males, 64 females. Age range was 21 to 62 (mean 39.2 years). Etiology of renal failure was chronic glomerulonephritis (54), diabetic nephropathy (36), interstitial disease (17), hypertensive nephrosclerosis (6), and other (8). Kidney source was from HLA-identical living-related donors (LRD) in 54 (Group I), one haplotype-matched LRD in 23 (Group II), and cadaver in 44 (Group III). Prior to the introduction of cyclosporin A (CsA) in 1984, prednisone withdrawal was attempted only in Group I. After 1984, prednisone withdrawal was also attempted in patients in Groups II and III, selected on the basis of having had no rejection episodes during the six months after transplantation. Forty-five patients in Group I were treated with azathioprine (Aza) and prednisone, and the remaining patients in Groups I to III were treated with Aza, prednisone and CsA. Mean follow-up was 93 months (6 to 207). Prednisone was gradually tapered and withdrawn in 94 of 121 patients after a mean period of 22.5 months (9 to 60). In 27 other patients, the prednisone dosage has been tapered to 5 mg/day or less with the aim of discontinuing the drug. There were seven episodes of acute rejection (3 during taper, and 1 each at 6, 7, 24 and 114 months after prednisone withdrawal); all seven were successfully reversed. Four other patients developed chronic vascular rejection (2 during taper and 2 after withdrawal).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

CR1 activity on erythrocytes and renal glomeruli in patients with renal disorders.

Complement receptors for C3b/C4b (CRI) on erythrocytes (ERC1) and renal glomeruli (GCR1) were examined in patients with different types of renal disorders. Normal ECR1 activity was found on erythrocytes from patients with IgA glomerulonephritis, benign nephrosclerosis and other types of renal diseases. In patients with systemic lupus erythematosus (SLE) and glomerulonephritis ECR1 activity was low or absent in 75% of the patients. The GCR1 activity, however, was normal except in areas with complement deposits where GCR1 activity was abolished. During treatment with corticosteroids and azathioprine of patients with SLE the clinical response was followed by increased functional ECR1 activity. In those patients who did not respond the ECR1 activity was persistently low. Three patients with renal transplant all showed increased ECR1 activity.

Erythrocytes↗