[Controversial issues in the Giornale Italiano di Nefrologia: how to treat patients with focal segmental glomerular sclerosis]. / Le contoversie del Giornale Italiano di Nefrologia: come trattare i pazienti con glomerulosclerosi focale segmentaria?

Primary focal segmental glomerular sclerosis (FSGS) commonly presents with nephrotic syndrome. Spontaneous remission is rare and persistent nephrotic syndrome is a marker of poor prognosis. For this reason, obtaining remission using drugs with minimal side effects is desirable. The treatment of FSGS, however, represents a challenge. Not only is there a lack of prospective controlled trials, but FSGS is a syndrome of unknown pathophysiology, generally treated with drugs having a mechanism of action that is poorly understood in this setting, the use of which has often drawn criticism because it is based on empirical assumptions rather than pathogenetic evidence. At present, corticosteroids are the standard first-line approach in patients with idiopathic FSGS. Cytotoxic agents and cyclosporin A constitute a good therapeutic option for steroid-dependent patients or frequent relapsers. Mycophenolate mofetil, rituximab and plasmapheresis should be used as rescue treatment because further studies are required to determine their safety and efficacy. Clearly, real progress in FSGS treatment can only be obtained by research focused on the pathophysiology of this disease, so that a therapeutic approach can be defined that is based on reason rather than chance.

[Nephrotic proteinuria with type 2 diabetes mellitus and autoimmune thyroiditis]. / Proteinuria nefrosica in paziente con diabete mellito di tipo II e tiroidite autoimmune.

This study reports on a 67-year-old man, suffering from type 2 diabetes mellitus for 11 years along with arterial hypertension and autoimmune thyroiditis, in whom nephrotic proteinuria was detected together with a mild reduction in GFR. No autoantibodies or monoclonal proteins were detected in blood and urine. Renal biopsy material examined by light microscopy, immunofluorescence and electron microscopy showed AL amyloidosis. This case underlines the role of renal biopsy in patients with type 2 diabetes mellitus, in whom renal diseases other than diabetic nephropathy may occur frequently.

[Thin glomerular basement membrane disease]. / La nefropatia a membrane basali sottili.

Thin glomerular basement membrane disease (TBMD) is a hereditary nephropathy characterized by thinning of the glomerular basement membrane evinced by electron microscopy and, clinically, by isolated hematuria without extrarenal manifestations. Familial aggregation is found in 50-60% of cases, with autosomal dominant transmission. TBMD is considered to belong to the type IV collagen spectrum of diseases, since heterozygous mutations of the COL4A3 or COL4A4 gene have been detected in more than 30% of patients. The disease is found in 1-2% of biopsies, but the prevalence in the general population may be higher. The differential diagnosis with Alport's syndrome may be difficult and requires accurate family investigations, immunohistochemical evaluation of type IV collagen alpha chains in renal tissue and, if appropriate, genetic studies. Progression towards chronic renal failure, although rare, has been reported in some patients, and may be related to the phenotypical variability of COL4A3/COL4A4 mutations, to a missed Alport syndrome, or to superimposed glomerular disease. Patients suffering from TBMD and affected relatives should be periodically examined for signs of disease progression and informed about the possibility of transmitting the autosomal recessive form of Alport's syndrome.

Low-dosage ibopamine treatment in progressive renal failure: a long-term multicentre trial.

A multicentre trial (11 nephrology centres) was carried out to test the effects of ibopamine, an orally active dopamine-like drug, on the progression of chronic renal failure. For a 2-year period 189 chronic renal failure patients (serum creatinine level 1.5-4.0 mg/dl) were observed. They were homogeneous for basic nephropathy, degree of residual renal function, blood pressure, and proteinuria. The patients were randomly divided into two groups: 96 took ibopamine at a dosage of 100 mg/day (group A) and 93 served as controls (group B). All were on a low-protein diet (mean 0.8 g/kg body weight). By the end of the observation period, the rate of decrease of the renal function indexes in time proved significantly slower (1.8 times) in group A than in group B. The survival curves for renal function (pre-established end points were creatinine level increases equal to or > 20% and equal to or > 40% of the basal values) proved significantly better (p < 0.02 and p < 0.002 respectively) in group A than in group B. The mean plasma creatinine values rose by 17% in group A and by 36% in group B. The creatinine clearance decreased by 5% in treated patients and by 14% in the controls. Statistical analysis ruled out any possible centre effect. The trial suggests that low-dosage ibopamine administration may be used as a valid and safe pharmacological adjunct for retarding the progression of renal failure in patients with mild or moderate chronic renal impairment.

[Doppler echography and color Doppler echography in the assessment of the vascular functional aspects of medical nephropathies]. / Contributo dell'eco Doppler e dell'eco color Doppler nella valutazione degli aspetti funzionali vascolari delle nefropatie mediche.

This study was aimed at finding a Doppler parameter to distinguish, among medical nephropathies, the ones with glomerular from those with vascular or tubulointerstitial involvement. Therefore, 32 patients (20 men and 10 women, average age: 43 years, range: 10-77 years) with clinical and laboratory signs of medical renal disease were examined with color-Doppler US. The resistive index (RI, n.v. < 0.70), as calculated from the Doppler waveform signal was especially considered to assess eventual significant changes differentiating renal diseases according to the different kinds of involvement. RI values were compared with renal biopsy findings, creatininemia levels and clinical and laboratory variables as hematuria and proteinuria. Histology diagnosed 18 glomerulonephritis, 4 glomerulonephritis with interstitial involvement and 10 vascular and tubulointerstitial nephroses, with 1 tubular necrosis. Doppler US demonstrated a normal RI value in 17/18 patients with glomerulonephrosis (mean value: 0.59 +/- 0.05). In one case only, even though biopsy indicated the involvement of one glomerulus only (membranous GN II stage), RI was high--i.e., 0.79. In 4 patients with simultaneous glomerular and interstitial involvement, the mean RI value was 0.17 +/- 0.01. In the 10 cases of tubulointerstitial or vascular nephrosis, the RI was 0.83 +/- 0.07. As far as the correlation between creatininemia levels and RI is concerned, in 8 patients with high values (1.5-8 mg/dl), the mean RI was 0.72 +/- 0.08 and only a weak correlation was found between the RI and the renal failure degree as expressed by creatininemia levels. Therefore, the RI seems to be related more to the site of the disease in the renal field than to renal failure degree. Doppler US seems to be capable of characterizing medical nephrosis, distinguishing glomerular from vascular or tubulointerstitial involvement. In this application area, the combined use of Doppler and color-Doppler US allowed each examination to be performed in a relatively short time--i.e., 30 minutes on the average.

Behavior of beta 2-microglobulin (B2-m) serum levels in uremic patients.

This study was performed in 97 hemodialysis patients (85 on HD and 12 on CAPD) to investigate the possible correlation between B2-m and hemodialysis-related amyloidosis syndromes (HRA-S); differences in B2-m and HRA behavior between patients hemodialysed with cellulose and synthetic membranes were also included in the present study. Data indicate that B2-m levels are not correlated with dialysis length or with the type of membrane used for the dialysis. On the contrary, in 16 patients with the Carpal Tunnel Syndrome, a significant correlation was found between the CTS, the dialysis length and the type of membrane (greater incidence with cellulosic membranes).

Epidemiology of viral hepatitis in dialysis centers: a national survey.

Because of the great problem of viral hepatitis in hemodialysis patients, the Italian Society of Nephrology decided to perform a national epidemiologic survey. We contacted 467 nephrological centers by a questionnaire which let us have information on 25,746 uremic patients: 18,338 on HD, 2,250 on PD and 5,176 with kidney transplant, respectively 78.5% of the total Italian dialysed patients and 91.4% of the total transplanted patients. Statistical analyses were performed. HBV infections occur in 7.8% of the patients (2,008 cases) but considering that 485 cases became spontaneously negative, the true overall incidence of chronic carriers falls to 4.9%. The main causes of the infection are reported as transfusions (64.3%) and dialysis environment (12%). The vaccination program performed by 93.2% of the centers, obtained an efficacious seroconversion in 4,626 of 7,790 cases vaccinated: the vaccine currently most utilized is the recombinant type administered by means of 3 versus 4 boosters. In the 2nd part of the survey, we report information concerning the presence of nephropaties associated with HBV infections in nonuremic patients (208 cases). We present and discuss the clinical picture of the nephropaties, the hystologic bioptic pattern and the prognosis of the kidney pathology.

Risk factors and clinical expression of HCV infection in hemodialysis patients.

In our Dialytic Unit, the prevalence of antibodies to HCV (anti-HCV) was 16.4% in 1990 (73 hemodialyzed) and 21% in 1991 (105 hemodialyzed). The incidence of seroconversion was 13.1%. The transaminase behavior was similar in a group of anti-HCV-positive and a group of anti-HCV-negative patients and with previous HBV infection. Blood transfusions and dialytic age did not affect anti-HCV positivity.

[Cerebral cysticercosis: report of a case and review of the literature]. / Cisticercosi cerebrale: descrizione di un caso e revisione della letteratura.

A case of cysticercosis with scattered calcifications has been reported; this, owing to the rarity of cases brought to our notice with certainty, is considered a medical curiosity. The case is subjected to analysis by us and we review the literature pertaining to such cases.

[Diabetes mellitus and acute myocardial infarct]. / Diabete mellito e infarto miocardico acuto.

A brief overview of the relevant literature is followed by the presentation of data obtained in a series of 389 males and 127 females admitted for acute myocardial infarct between 1-1-1974 and 31-12-1978. Diabetes mellitus was more common in these patients (13.9% in males and 12.5% in females) than in the general population (5%).