[CIS PIDD] [cis-pidd] Renal Transplant in CGD

[CIS-PIDD]

Hi Richard,

There are reports of primary interstitial kidney disease (FSGS, MPGN, papillary necrosis, interstitial nephritis, etc.) in patients  with CGD scattered in the literature and kidney transplant has been successful at least in patients with p47phox deficiency. (Bolanowski A, Mannon RB, Holland SM, Malech HL, Aschan J, Palmblad J, et al. Successful renal transplantation in patients withchronic granulomatous disease. Am J Transplant. 2006;6(3):636–9.)

Steve Holland and I reviewed kidney disease in a small cohort of patients from the NIH (Leiding JW 2013 JoCI) and at the time of that publication, there were a handful of reports of intrinsic kidney disease in CGD. Citations included:

12. Carson MJ, Chadwick DL, Brubaker CA, Cleland RS, Landing
BH. Thirteen boys with progressive septic granulomatosis. Pediatrics.
1965;35:405–12. Epub 1965/03/01.
13. Dilworth JA, Mandell GL. Adults with chronic granulomatous
disease of “childhood”. Am J Med. 1977;63(2):233–43. Epub
1977/08/01.
14. Kimpen J, Van Damme-Lombaerts R, Van den Berghe G, Proesmans
W. Autosomal recessive chronic granulomatous disease associated
with 18q-syndrome and end-stage renal failure due to Henoch-
Schonlein nephritis. Eur J Pediatr. 1991;150(5):325–6. Epub 1991/
03/01.
15. van Rhenen DJ, Koolen MI, Feltkamp-Vroom TM, Weening RS.
Immune complex glomerulonephritis in chronic granulomatous
disease. Case report of an eighteen-year-old girl. Acta Med Scand.
1979;206(3):233–7. Epub 1979/01/01.
16. Frifelt JJ, Schonheyder H, Valerius NH, Strate M, Starklint H.
Chronic granulomatous disease associated with chronic glomerulonephritis.
Acta Paediatr Scand. 1985;74(1):152–7. Epub 1985/01/01.
17. Forbes GS, Hartman GW, Burke EC, Segura JW. Genitourinary
involvement in chronic granulomatous disease of childhood. AJR
Am J Roentgenol. 1976;127(4):683–6. Epub 1976/10/01.
18. De Seigneux R, Kanfer A, Terrioux P, Sraer JD, Whitworth JA.
Letter: Renal amyloidosis in chronic granulomatous disease. Br
Med J. 1974;4(5938):230. Epub 1974/10/26.
19. Dechelette E, Rossignol AM, Gout JP, Poirot P, Frappat P, Bost M.
[Renal amylosis and chronic septic granulomatosis. Apropos of a
case]. Pediatrie. 1977;32(6):UNKNOWN. Epub 1977/09/01. Amylose
renale et granulomatose septique chronique. A propos d’une
observation.
20. Kaltenis P, Mudeniene V, Maknavicius S, Seinin D. Renal amyloidosis
in a child with chronic granulomatous disease and invasive
aspergillosis. Pediatr Nephrol. 2008;23(5):831–4. Epub 2008/
01/12.
21. Peces R, Ablanedo P, Seco M. [Amyloidosis associated with
chronic granulomatous disease in a patient with a renal transplant
and recurrent urinary tract infections]. Nefrologia. 2002;22
(5):486–91. Epub 2002/12/25. Amiloidosis asociada a enfermedad
granulomatosa cronica en una paciente con un trasplante renal e
infecciones urinarias de repeticion.
22. Yamazaki H, Nishi S, Chou T, Nakagawa Y, Shimada H, Nunoi H,
et al. Two brothers with p47-phox-deficient chronic granulomatous
disease associated with end-stage renal failure. Nephrol Dial
Transplant. 1995;10(12):2334–6. Epub 1995/12/01.

Hope this helps and good luck,
Jen Leiding
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, January 14, 2016 12:22 PM
To: CIS-PIDD
Subject: [cis-pidd] Renal Transplant in CGD

The 16 yo daughter of a patient with x-linked CGD (genotype on the father not done but he is now 47 so I suspect a mild phenotype) now presents in renal failure due to long standing MPGN. Oxidative burst assay was about 30% of the lower limit of normal and flow analysis showed two cell populations. Grandmother has similar Lyonization and some clinical problems. Only the minority population, about 30% of cells, responded to stimulus. The nephrologist asks if she is a transplant candidate.

There were respiratory tract infections and a mildly low IgG with a normal IgA and IgM at age five that was felt to be due to protein loss. This patient has had very inconsistent follow up. She has been seen only five times since 2009, the last about 18 mo ago so I have limited information about her recent infections.

Given the diminished phagocytic cell function, will the immunosuppression necessary for a renal allograph be a high risk for fatal fungal infection?

She was posted to the PAGID List Serve in 2009. See below. At the time, there were no useful suggestions to explain the reaction to Cellcept. Any thoughts now?

"Was treated with prednisone for 3 years for MPGN and stopped a year ago. Cellcept was started. Within 24 hours of starting Cellcept she developed cervical and submandibular and axillary swollen nodes that were tender with some fever. Cellcept was stopped and the nodes slowly improved taking one month to normalize. This occurred three times with the same result. Was seen by pcp and treated with antibiotics without benefit. Seemed to be associated with periorbital edema (related to MPGN). Last dose of Cellcept was one year ago but has had persistent intermittent painful adenopathy."

--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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