Congenital nephrotic syndrome and agammaglobulinemia: A therapeutic dilemma

Abstract

The primary immunodeficiency practice parameters outline use of gamma globulin as treatment for diseases of hypo- and agammaglobulinemia. There is no consensus, however, on use of immunoglobulin replacement in secondary immunodeficiencies such as protein-losing disease states. Congenital nephrotic syndrome is an exceedingly rare disorder lacking data on comorbid conditions such as agammaglobulinemia, although infection risk is known to be high as serious bacterial infection was demonstrated in 85% of a 41-patient cohort. [1] Mahan J.D. Mauer S.M. Sibley R.K. Vernier R.L. Congenital nephrotic syndrome: evolution of medical management and results of renal transplantation. J Pediatr. 1984; 105: 549-557 Abstract Full Text PDF PubMed Scopus (113) Google Scholar Harris examined the use of intravenous immunoglobulin (IVIG) in a congenital nephrotic patient and showed that, within 30 hours, 55% could be found in the urine. [2] Harris H.W. Umetsu D. Geha R. Harmon W.E. Altered immunoglobulin status in congenital nephrotic syndrome. Clin Nephrol. 1986; 25: 308-313 PubMed Google Scholar A review evaluating the use of IVIG in pediatric nephrotic syndrome illustrated a significant benefit on preventing infection; however, the quality and sample sizes of the reviewed studies were called into question. [3] Wu H.M. Tang J.L. Cao L. Sha Z.H. Li Y. Interventions for preventing infection in nephrotic syndrome. Cochrane Database Syst Rev. 2012; 18: 4 Google Scholar IVIG treatments have even been shown to resolve proteinuria and improve kidney function in 1 small cohort of patients with congenital nephrotic syndrome secondary to diffuse mesangial sclerosis. [4] Gentner J.E. Wood E.G. Vogler C.A. Beck A.M. Knutsen A.P. Remission of congenital nephrotic syndrome from diffuse mesangial sclerosis with IVIG therapy. J Allergy Clin Immunol. 2008; 121: S83 Abstract Full Text Full Text PDF Google Scholar In adult-onset nephrotic syndrome, IVIG has been shown to mitigate infections. [5] Ogi M. Yokoyama H. Tomosugi N. et al. Risk factors for infection and immunoglobulin replacement therapy in adult nephrotic syndrome. Am J Kidney Dis. 1994; 24: 427-436 PubMed Scopus (81) Google Scholar