[CIS PIDD] Post-Fontan hypogam & PLE

[Boyce, Thomas G., M.D.]

Doubt PLE given NL or low stool A1AT. 



On Aug 22, 2012, at 4:57 PM, "Stan Ress" <Stan.Ress at uct.ac.za> wrote:


> 

> I wondered if PLE could account for isolated IgG deficiency in 67 year-old lady with preserved serum albumin. She has a complex history of surgery & irradiation for lung ca in 1997 & 2008. Recently presented with weight loss, diarrhoea, & food intolerance. Found to have IgG 6.04 g/L & 5.3 more recently, IgA 1.33 g/L, IgM 0.60g/L. No paraprotein serum peak or urine Bence Jones protein detectable. No evidence of malabsorbtion.  Serum Albumin 42 g/L (37-52), low serum gamma-globulin of 5.7 (8-13g/L). 

>  

> Could she still have PLE, despite normal serum albumin & low stool alpha-1-antitrypsin <0.18 mg/g (0.43-1.47), with normal serum alpha-1-antitrypsin level?

>  

> I would appreciate any comments/suggestions.

>  

> Thanks.

>  

> Stan Ress

>  

> --

> Stanley Ress

> Associate Professor of Medicine

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> >>> "Boyce, Thomas G., M.D." <Boyce.Thomas at mayo.edu> 8/22/2012 3:40 AM >>>

> Agree usually albumin is low in PLE but would check a stool alpha 1 antitrypsin to see if losing protein in stool. Also very hard to replete IgG in pts with PLE. 

> 

> 

> 

> On Aug 21, 2012, at 8:29 PM, "Church, Joseph" <JChurch at chla.usc.edu> wrote:

> 

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>> Colleagues:

>> 

>> I follow an 8yo boy with the following

>>             - Protein losing enteropathy (history of)

>>             - Fontan procedure for hypoplastic left heart

>>             - IgG 334 IgA 50 IgM58

>>             - CD3+ 26% (308), CD19 42% (478)

>>             - Serum albumin 4.7 mg/dL.

>> Has any one seen this pattern of immunodeficiency in the face of a very normal serum albumin level?

>> Thank you for your thoughts.

>> Joe Church

>> Children's Hospital Los Angeles

>> 

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