5-Hydroxyindoleacetic acid (5-HIAA) and homovanillic acid (HVA) were measured by high-performance liquid chromatography (HPLC) in lumbar cerebrospinal fluid (CSF) obtained from febrile children subdivided according to the presence or absence of convulsions. Lumbar puncture was made either early (mean time 2 h) or late (3-6 days) after the febrile convulsion. The level of 5-HIAA was significantly decreased in children early and late after the febrile convulsion as compared with the convulsion-free group, but the HVA level was reduced only early after the febrile convulsion. These results support the hypothesis that a decrease in CSF 5-HIAA may be a biologic marker of susceptibility to convulsions and indicate that the transient decrease in HVA is a secondary phenomenon related to occurrence of convulsions.

Background The spectrum of Interferon-beta-associated nephropathy (IFN-β) remains poorly described and the potential features of this uncommon association remain to be determined. Methods In this study, we retrospectively analyzed the clinical, laboratory, histological and therapeutic data of patients with biopsy-proven renal disease in a context of IFN-β treatment administered since at least 6 months. Results Eighteen patients (13 women, median age 48 years) with biopsy-proven renal disease occurring during IFN-β therapy were included. The median exposure to IFN-β (14 patients were treated by IFN-β1a and four patients by IFN-β1b) was 67 months (ranged from 23 to 165 months). The clinical presentation consists in hypertension (HT) (83%), malignant HT (44%), proteinuria >1g/g (94%), reduced renal function (78%), biological hallmark suggesting thrombotic microangiopathy (TMA) (61%), edematous syndrome (17%), or nephritic syndrome (11%). The pathological findings included typical features of isolated TMAs in 11 cases, isolated focal segmental glomerulosclerosis (FSGS) lesions in two cases, and five cases with concomitant TMA and FSGS lesions. An exploration of the alternative complement pathway performed in 10 cases (63%) did not identify mutations in genes that regulate complement system. The statistical analysis highlighted that the occurrence of IFN-β-associated TMA was significantly associated with Rebif®, with a weekly dose >50 µg and with multiple weekly injections. In all cases IFN-β therapy was consequently discontinued. Patients with TMA lesions received other therapies included corticosteroids (44%), eculizumab (13%) or plasmatic exchanges (25%). At the end of a 36-month median follow-up, persistent hypertension and persistent proteinuria were observed in 61% and 22% of patients. Estimated glomerular filtration rate <60 ml/min/1.73m2 was present in 61% of patients. Conclusion IFN-β-associated nephropathy must be sought in the case of hypertension and/or proteinuria onset during treatment. When TMA and/or FSGS are observed on renal biopsy, early discontinuation of IFN-β is essential.

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