[CIS PIDD] [cis-pidd] VACTERL and T-cell deficiency

[Church, Joseph]

Thanks, Prescott, and all who responded.  It seems that VACTERL associated with significant T-cell deficiency is quite uncommon, and if suspected consider questioning the VACTERL diagnosis.

Joe Church
Children's Hospital Los Angeles

From: Prescott Atkinson, M.D. [mailto:PAtkinson at peds.uab.edu]
Sent: Tuesday, May 19, 2015 3:01 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] VACTERL and T-cell deficiency

Hi Joe:

About 20 years ago we had a patient who fit this syndrome and had complete DiGeorge syndrome. Max Cooper invited Dick Hong down from Vermont and he provided the baby with a thymic transplant (placed in a sack of mesentery). Unfortunately she contracted CMV, possibly from breastmilk that she had received prior to her diagnosis, and she died of disseminated CMV several months later without ever producing significant numbers of T cells.

Prescott

T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel: 205-996-9582
Fax: 205-975-7080
Cell: 205-999-7688




From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Tuesday, May 19, 2015 8:39 AM
To: CIS-PIDD
Subject: [cis-pidd] VACTERL and T-cell deficiency

Colleagues:

I was asked if I had ever seen VACTERL/VATER syndrome associated with marked T-cell deficiency.  The only case I found was that of a VACTERL baby with chylothorax.

Any experience with this association?

Joe Church
Children's Hospital Los Angeles

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