Nephrotic and nephritic syndrome in the newborn.

Glomerular disorders in infancy can include nephrotic and nephritic syndromes. Congenital nephrotic syndrome (CNS) is most commonly caused by single gene mutations in kidney proteins, but may also be due to congenital infections or passive transfer of maternal antibodies that target kidney proteins. Prenatal findings of increased maternal serum α-fetoprotein and enlarged placenta suggest CNS. Neonatal nephritis is rare; its causes may overlap with those of CNS and include primary glomerulonephritis, systemic disease, infections, and transplacental transfer of maternal antibodies. These syndromes in the neonate can cause significant morbidity and mortality, making urgent diagnosis and treatment necessary.

A clinicopathologic study of forty-eight infants with nephrotic syndrome.

The clinical and histopathologic features of 48 children presenting with the nephrotic syndrome during the first year of life were analyzed. Proteinuria was discovered soon after birth to 3 months of age in 39 infants (congenital nephrotic syndrome), and nine infants had an infantile onset presenting between 4 and 12 months of age. Neither histologic parameters--microglomeruli, epithelial, or mesangial proliferation, focal segmental or global sclerosis, fibrinoid necrosis, or tubular microcysts--nor histologic classification--microcystic disease, mesangial proliferative glomerulonephritis, focal segmental glomerular sclerosis/hyalinosis-predicted the outcome. Rather, age at presentation was found to predict outcome: One of 39 infants with a congenital onset and seven of nine infants with an infantile onset underwent a complete remission (P less than 0.0001).

Congenital glomerulosclerosis and nephrotic syndrome in two infants. Speculations and pathogenesis.

The incidental finding of hyalinized glomeruli in otherwise normal infant kidneys is referred to as congenital glomerulosclerosis. Two infants had extensive glomerulosclerosis manifested by nephrotic syndrome, severe oliguria, and progressive renal failure. Both patients were believed to have had intrauterine infections. These two cases have unequivocally identified congenital glomerulosclerosis as one of the causes of nephrotic syndrome in infancy. In addition, they suggest that extensive glomerulosclerosis in some cases may be a result of congenital infections.

[Renal lesions in congenital syphilis. A case report]. / L'atteinte rénale de la syphilis congénitale. A propos d'une observation.

The authors describe a case of congenital syphilitic nephropathy in an infant. The review of the reports in the literature shows many similarities of this congenital disease with the syphilitic nephrotic syndrome in the adult. The prognosis is favorable provided the diagnosis is made sufficiently early. Histological studies utilizing light-, electron- and immunofluorescence-microscopy gave results consistent with extramembranous glomerulonephritis with possible evolution towards endo- and extracapillary proliferative glomerulonephritis. This is one of the rare renal diseases with a known antigen.

[Studies on the diaplacental transmission of Masugi-nephritis in the rabbit]. / Untersuchungen zur diaplazentaren Ubertragbarkeit der Masugi-Nephritis des Kaninchens

A diaplacentar transmission of the Masugi nephritis or the evocation of a clinical picture similar to the congenital glomerulonephritis could not be achieved in rabbits. Also, the juxtaglomerular index-values of the experimental group did not show a significant difference referred to the control group.