No abstract

The patterns associated with Sjögren's syndrome and normal patients matched the ones in the literature. The tear ferning test classification is reproductible when the Rolando classification was used for Sjögren's syndrome patients.

The purpose of this study was to assess the results of therapy with mycophenolate mofetil (MMF) in children with idiopathic nephrotic syndrome (INS) who were both steroid- and cyclophosphamide-resistant. Treatment lasted a minimum of 6 months, and follow-up data were collected over a 2-year period. The children were divided into two groups: Group 1 (n=34) comprised patients who had received cyclosporine A (CsA) before the initiation of MMF therapy; Group 2 (n=18) comprised patients who received only MMF. Among the 34 patients of Group 1, complete and partial remission were achieved in seven (20.6%) and 13 patients (38.6%), respectively; there was no response in 14 patients (41.2%). Among the 18 patients in Group 2, complete and partial remission occurred in five (27.8%) and six (33.3%) patients, respectively; there was no response in seven patients (38.9%). Eight patients developed chronic kidney disease. The main side-effects were gastrointestinal complaints (n=11, 21%), recurring severe infections (n=1, 1.9%), and mild thrombocytopenia/leucopenia (n=1, 1.9%). MMF proved to be therapeutically effective in 59.5% of the cases. These beneficial effects need to be confirmed in studies with a long-term follow-up after discontinuation of the treatment. Our statistical analysis of the results of therapy with MMF did not reveal any significant difference between its use alone or following CsA administration.

Apoptosis occurs in kidney and pancreas allograft biopsies, markedly in acute rejection in pancreas biopsies. Although apoptosis may reflect a mechanism of down-regulation of the allograft immune response by eliminating infiltrating cells, the elimination of graft cells may result in graft damage, particularly in pancreas transplantation.

Hemolytic uremic syndrome is characterized by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and renal failure. Clinical/ pathologic data, along with the treatment and outcome of 8 adult patients with HUS, are described. There were 7 females and 1 male, age 30.7 +/- 12 years; 7 were White and 1 Black. Three patients were kidney graft recipients, 2 of whom were receiving cyclosporine; 2 patients were postpartum; 1 case followed an abortion; 1 occurred with prodromic infection; and 1 case was without a causal factor. All patients presented with hematuria and 6 with oligoanuria. Laboratory data showed hemolytic anemia with schistocytes, LDH values were 2584 +/- 2191 U/L, platelets were 79,000 +/- 40,000/mL, creatinine concentrations were 5.9 +/- 2.5 mg/dL. Renal biopsy showed thrombotic microangiopathy. Two had predominant glomerular involvement. 2 showed renal cortical necrosis, 4 were marked by predominant arteriolar involvement. In 5 patients dialytic therapy was performed. All were treated with fresh-frozen plasma infusion and 6 with plasmapheresis. Three patients died, 2 without recovery of renal function. In conclusion, the trigger events were related to renal transplant in 3.2 of them taking cyclosporine; 3 with pregnancy; 1 to precedent infection; and 1 with no causal factor. There was no correlation between histological form and outcome in this group of patients. The benefit of plasmapheresis was evident in the recovery of the extrarenal manifestations, although it did not change the renal outcome. The prognosis is poor, with a high mortality (37.5%) and/or end-stage renal failure (37.5%). Complete recovery of renal function was obtained in 25%.