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Clinical and laboratory features in 27 children and adolescents (24 females, 3 males) with lupus nephritis were analysed and correlated with pathohistologic findings obtained by renal biopsy. The mean age at the onset of the disease was 12.1 +/- 1.9 (range 0.1-7.0) years. In the 30% of patients nephritis was one of the presenting features of the disease. The most frequent signs of the lupus nephritis at the time of biopsy were proteinuria (9 pts), proteinuria and haematuria (9 pts), and nephrotic syndrome (8 pts), while 7 patients showed decreased glomerular filtration rate (GFR), and hypertension was present in five. Distribution of patients according to the WHO morphologic classification of Lupus nephritis was as follows: 6 pts showed class Ib, 6 class II, 2 class III and 13 class IV. More severe renal dysfunction, manifested by nephrotic syndrome, proteinuria with haematuria, decreased GFR and hypertension was significantly associated with proliferative lupus nephritis (combined WHO classes III and IV). Nephrotic syndrome, haematuria and decreased serum C3 with significant probability predicted proliferative lupus nephritis, while decreased GFR was of borderline predictive value.

The association of a spondyloepiphyseal dysplasia and disproportionate short stature with focal glomerular sclerosis is reported in two girls. Renal disease manifested by proteinuria at the age of 2.5 and 11 years, leading to treatment-resistant nephrotic syndrome over 15 and 45 months, respectively. One patient went into end-stage renal failure shortly after nephrotic syndrome developed, the other died from sepsis. The association of spondyloepiphyseal dysplasia and focal glomerular sclerosis with nephrotic syndrome may represent a distinct disease entity.

Stimulation of T-lymphocytes derived from some patients with common variable immunodeficiency (CVID) syndrome results in defective proliferation. The underlying mechanism is related to the inability of stimulated cells to secrete IL-2 while the expression of IL-2 receptor (IL-2R) is normal. We have identified a patient whose peripheral T-cells failed to proliferate and secrete IL-2 upon stimulation. The addition of recombinant IL-2 restored proliferation. The defect did not seem to be caused by accessory cell failure since the patient's adherent cells produced IL-1 and IL-6, and addition of allogeneic irradiated cells did not induce proliferation. Stimulation of CVID T-cells with phorbol esters and Ca2+ ionophore induced both IL-2 secretion and proliferation, indicating the absence of a defect in the transcription and/or translation of the IL-2 gene. The patient's T-cells expressed high levels of CD3. The majority of T-cells expressed the CD38 molecule which is normally found on thymocytes or activated T-cells but not peripheral blood T-cells and HLA-DR, another activation marker. However, CD25 (the IL-2R) and CD1, a marker of more immature thymocytes, were not expressed. Finally, the patient's cells were sensitive to an in vitro corticosteroid treatment. The possibilities that this patient's T-cells represent anergic T-cells or not fully matured thymocytes are discussed.

Penicillin allergy in children is overdiagnosed and paediatricians, fearing the possibility of fatal anaphylaxis, seldom question a report that a child is allergic to penicillin. Laboratory tests to predict whether a patient will react to penicillin are unhelpful in routine clinical practice and skin tests themselves carry a significant risk of anaphylaxis and even death. A few simple inquiries can often exclude the diagnosis. However, a history of penicillin therapy associated with an immediate reaction suggests penicillin allergy.