[CIS-PAGID] immune deficiency with kidney disease on peritoneal dialysis

[Nancy Kingston]

Dear Colleagues,
I have been following a 3 1/2 yo African American female for the last couple months.  She has recently moved to my area and transferred care, and due to some difficult social issues has not always been able to be followed completely, it seems.  Therefore, past clinical history may not be complete...
She was born in 2/2008. and was apparently diagnosed with TTP/atypical HUS in 12/2008.   Related complement studies (Factor B, Factor H, Factor I,  CD46, C3, C4), all were reportedly normal.
She had some issues with DVT's, and a low ADAMS 13.  
In the summer of 2009 she was diagnosed with significant oropharyngeal candidiasis and draining otitis.  A tracheal aspirate was positive for staph at that time as well.  Later that summer, she also had a labial fungal infection.  
A bone marrow biopsy that Fall 2009 was normocellular, but had changes consistent with microangiopathic hemolytic anemia.
 About one month later, an echocardiogram showed decreased LV diastolic function.  
A renal biopsy showed enlarged glomeruli, tubular atrophy with moderate to severe interstitial fibrosis.  
She has had immuniztions up to six months of age and this did include rotavirus.

Labs done  (patient age 2) were reported as protective pneumococcal antibodies to 10/14 serotypes and protective to HIB
ALC 1200
Total T cells 819
CD4 396
CD8 198

CD19 848
CD 1046
Unfortunately, no IgG, A, M, E available when I first saw her and I have not been able to find these records.
HIV negative

She does not have dysmorphic features. 

She had been maintained on either pentamidine or Bactrim due to the low T cells as well as q 3 wk IVIG.

When she presented to me, her IgG level was 506mg/dl.  This was 4-5 wks post infusion
Since having q 3 wk infusions, she has maintained an IgG level between 850-1400 without significant infection.
The CD4 level is 278 (560-2700), CD8 level is 229 (330-1400), CD3 level is 737 (1000-3900), CD19 level is 295 (200-1300), CD16 level is 557 (90-1200)
She does have normal mitogen studies.
She does have bronchiectais.
She has tubulointerstitial disease with IgM and C3 deposits on her kidney biopsy.

She is now on peritoneal dialysis and will need a kidney transplant.  At this time she has a PICC line, but IV access is a problem, so we have considered a change to subcutaneous infusions of IgG for this reason.  The first issue is that we would like to continue IgG replacement as it has been helpful for her, but this is an issue with the dialysis.  If any one has thoughts regarding this I would welcome them.  In addition, we would like to pursue further immunologic work up.  It has been suggested to see how she does with kidney transplant first and then consider immune diagnosis and transplant.  Would you agree with this?
Thanks for your help.
Nandy Wasserbauer, DO
Akron Children's Allergy and Immunology,
Akron, OH
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