Nephrotic syndrome, a constellation of symptoms that separates the paediatric nephrologist from their adult colleagues, a clinical triad of oedema, hypoalbuminaemia and albuminuria. The vast majority of patients respond to steroids, their oedema melting away after a few weeks of prednisolone. So in paediatric practice all children between the ages of two and twelve years, who present with nephrotic syndrome, are treated with an initial course of 60mg/m2 of prednisolone daily. Once they respond, the challenge has been to determine how to wean down the steroid and how to treat those who relapse. Even in the last decade weaning regimes have varied from two to six months in length.
Throughout the decades, nephrologists have attempted to define nephrotic syndrome by age at presentation, congenital, infantile, childhood; by histology, minimal change disease, focal segmental glomerulosclerosis, diffuse mesangial sclerosis; by genetics, as a plethora of genes responsible for slit diaphragm formation and podocyte kinetics were described. No categorisation has proven as useful however, as treatment response. Whether a child’s disease is filed under steroid sensitive, steroid resistant or steroid dependant is of more consistent clinical use than any other means of definition. Nephrotic syndrome remains a true paediatrician’s disease, encouraging us to observe the child not their histology or genotype.
The discovery that treatment with cortisone or ACTH could lead to remission and a reduction in mortality was practice changing in the early 1950s when few children with nephrotic syndrome survived. In the 40s nephrotic syndrome was a much more frightening condition, children ballooning to twice their weight before dying of thromboses and overwhelming sepsis. Senior colleagues remember how kids with nephrotic syndrome were occasionally put into bed beside children with measles because the T-cell response would trigger remission in a lucky few, though I imagine many succumbed to the measles.
PREDNOS is a practice challenging paper, not least because it attempts to put some rigor onto what has been a very opinion based field with differences between centres in terms of weaning regimes. It has been a long time coming and many discussions about standardising weaning regimes have been put off “until PREDNOS”. So now that it’s here what will change?
Not only does this paper show us that there is little difference between 8 weeks and 16 weeks of prednisolone in terms of disease outcomes but it also neatly defines what we can expect for patients with steroid sensitive disease.
They are not unlucky if they have a relapse, they are lucky if they don’t have one. Expect relapses. A relapse is not the fault of too rapid a weaning regime.