[CIS PIDD] [cis-pidd] Baby with intestingal lymphangiectasia

[Burcin Uygungil]

Hi Joe,

Is she on a high MCL diet? Sometimes this can help decrease the overall losses so you can then gauge what her baseline IgG would be with dietary changes alone. This is not an easy diet though. 

In my experience you would be chasing your tail but weekly or twice weekly subq IgG might be able to keep her levels up. Oftentimes replacement is not necessary. Depending on how far they live or how often you see her, you could just monitor levels closely. 

Burcin

> On Jul 22, 2015, at 2:07 PM, Church, Joseph <JChurch at chla.usc.edu> wrote:
> 
> Colleagues:
>  
> I have consulted on an 18 month-old girl with intestinal lymphangiectasia and unilateral lymphedema.  She has experienced no infectious complications.
>  
> However, labs demonstrate
>             Serum albumin 2.3 g/dL
>             IgG      83 mg/dL
>             IgA      <7 mg/dL
>             IgM      27 mg/dL       
>             Tetanus antibody 0.49 IU/mL (considered protective)
>             Haemophilus influenzae antibody 0.48 mcg/mL (considered unprotective)
>             Pneumococcal antibody response to Prevnar 13 positive (>1.3 mcg/ml) for 10 of 12 serotypes tested.
>             CD3+ 46% (1399/mcL)
>             CD4+ 14% (328/mcL)
>             CD8+ 46% (1113/mcL)
>             CD19+ 24% (590/mcL)
>             NK 16% (391/mcL)
>             PHA and PWM responses decreased (~ 25% of concurrent control)
>  
> The child is clearly losing protein, immunoglobulins and T-cells (CD4+ > CD8+), but has fair circulating antibody responses to tetanus toxoid and conjugated pneumococcal vaccine.
>  
> I would appreciate your opinions regarding immunoglobulin replacement therapy.
>  
> Joe Church, MD
> Children’s Hospital Los Angeles
>  
>  
> 
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