Nephrotic syndrome in infancy can spontaneously resolve

Area of Science:

• Pediatric Nephrology
• Infectious Diseases
• Genetics

Background:

• Nephrotic syndrome in the first year of life (NSFL) is a serious condition with a poor prognosis, often unresponsive to immunosuppression and leading to end-stage renal failure.
• Current management is primarily supportive, with limited treatment options for most affected infants.

Observation:

• Four infants presented with severe nephrosis postnatally, preceded by symptoms suggestive of viral infections.
• One infant had confirmed pertussis, requiring respiratory support, and presented with varying degrees of mesangial expansion and glomerular changes on renal biopsy.
• Despite the severity, all patients achieved spontaneous remission with supportive care, including albumin infusions, diuretics, antibiotics, and ACE inhibitors.

Findings:

• The study describes a distinct subgroup of NSFL characterized by infectious prodromes and a favorable prognosis, with complete resolution of nephrosis and normal renal function.
• This is the first reported case of pertussis (whooping cough) as a potential cause of nephrotic syndrome in infancy.
• Genetic and autoimmune investigations were negative, ruling out common genetic mutations (NPHS1, NPHS2, WT1, LAM-β) and autoimmune disorders.

Implications:

• The findings suggest that NSFL may have a more heterogeneous etiology and prognosis than previously understood, with infectious triggers playing a role in some cases.
• Identifying this subgroup with a good prognosis can help tailor management strategies and improve patient counseling.
• Further research into the link between viral infections, including pertussis, and NSFL is warranted to understand the underlying mechanisms.