Congenital renal abnormalities in the Laurence-Moon-Biedl syndrome.

Two children with Laurence-Moon-Biedl syndrome had radiographic evidence of cysts in the renal medulla, and one had impaired renal function and infection. The frequency of cystic disease in this syndrome implies that intravenous urography should be carried out on all patients with this syndrome. Children with renal abnormalities should be followed to avoid further damage from urinary infection, or from dehydration in those with a concentrating defect. Regular renal function tests should be carried out on other children. and full investigation should follow if abnormalities are found in order that the natural history of the often fatal renal lesion can be clarified.

Prognosis of Henoch-Schönlein nephritis in children.

All the survivors of a series of 88 patients with Henoch-Schönlein nephritis were examined after a follow-up of six and a half to 21 years (mean 9-9). Sixty-one patients had no demonstrable abnormality; six had minor urinary abnormalities; five had hypertension without urinary abnormally or renal dysfunction; four had heavy proteinuria; eight were in chronic renal failure, three of whom were on regular dialysis; and four patients had died within 25 months of onset. Neither corticosteroids nor immunosuppressive drugs alone or in combination appeared to influence the outcome. A clinical presentation with a combination of acute nephritis and a nephrotic syndrome and a high proportion of crescents in renal biopsy specimens was associated with a poor outcome. Neither the clinical presentation nor the renal morphology were, however, precise determinants of outcome. Outcome was not related to age, associated streptococcal infection, or recurrences of the rash. The clinical state two years after presentation was compared with the state six and a half years or more after presentation in 76 patients. The clinical state had changed in 32 patients, in 17 of whom it had deteriorated. It was not possible to identify with any certainty the patients who would deteriorate (or improve). Patients who have had Henoch-Schönlein nephritis should be followed up for at least five years.