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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.

Recent advances in immunosuppressive therapy have dramatically reduced the incidence of acute rejection, thus improving graft survival. As a result, the importance of recurrence of the original nephropathy as a factor affecting the long-term outcome of the graft has grown considerably The incidence of recurrence increases with the increase in graft survival and can currently be estimated between 6 and 15% 10 years after surgery, with great variability among the different histological types of nephropathy. More than 50% of patients with recurrence experience progressive deterioration of graft function, and recurrence of the nephropathy accounts for long-term graft failure in more than 15% of cases. The original disease should be duly considered in all candidates for renal transplantation to identify patients at higher risk for recurrence and to define those treatment protocols devoted to risk reduction. Finally, the risk of disease recurrence should always be included among the parameters used in evaluating a possible transplantation from living donor.

In its early stages Wegener's Granulomatosis (WG) commonly presents symptoms in E.N.T. sites. However, these symptoms are not specific and vary greatly so that alone they rarely lead to a correct diagnosis. Anti-neutrophil cytoplasmic autoantibodies (ANCA) have recently been found in patients with WG. The presence of such autoantibodies is now considered a specific test for this disorder. Over the last 8 years, 11 patients with WG having E.N.T. involvement were observed. All were tested for the presence of ANCA and such antibodies were found in 10 out of 11 patients. Biopsy showed the histopathological characteristics of WG--i.e. granulomatous necrotizing vasculitis--in five cases (in one patient biopsy was carried out surgically in the lung). Subglottic localization was surprisingly common in these patients, being seen nearly as frequently as the sinonasal localization (generally the most common manifestation of WG in the E.N.T. district). Early identification of the WG subglottic stenosis made it possible to administer appropriate medical therapy, thus limiting the number of surgical procedures. Only one patient was temporarily tracheostomized. None underwent laryngo-tracheoplasty. A major clinical problem in WG is to keep the interval between onset and definitive diagnosis as short as possible. At the outset, WG nearly always displays symptoms and signs in E.N.T. sites. Unfortunately, however, the diagnosis is nearly always made when pulmonary, or even worse renal, localizations appear. This considerably worsens the quoad vitam and quoad valetudinem prognosis. The authors' experience leads them to conclude that Anti-neutrophil cytoplasmic autoantibodies, in their C-ANCA pattern, represent a useful tool for prompt diagnosis of Wegener Granulomatosis, particularly in limited or atypical forms.