[CIS PIDD] [cis-pidd] Newborn with chronic diarrhea and near absent plasma cells in the gut mucosa

[Haddad Elie]

Kate, you just stole it to me...

Elie
________________________________
De : Sullivan, Kathleen [sullivak at mail.med.upenn.edu]
Envoyé : lundi 14 octobre 2013 10:37
À : CIS-PIDD
Objet : Re: [cis-pidd] Newborn with chronic diarrhea and near absent plasma cells in the gut mucosa

I would also consider tricho-hepato-enteric syndrome.  Tip is bamboo hair.

On Oct 14, 2013, at 10:28 AM, Yeşim Yılmaz Demirdağ wrote:


There is no history of IUGR, no cytopenia, no nail, or skin symptoms.
Thanks!
Yesim


Yesim Yilmaz Demirdag, MD

Assistant Professor of Pediatrics

Division of Allergy and Immunology

Columbia University Medical Center

3959 Broadway, Room 107N

New York, NY 10032

phone: (212) 305 2300<tel:%28212%29%20305%202300>





On Mon, Oct 14, 2013 at 10:21 AM, Jyonouchi, Soma C <JYONOUCHI at email.chop.edu<mailto:JYONOUCHI at email.chop.edu>> wrote:

Is there any history of IUGR?  Dyskeratosis congenita patients can develop a severe enteropathy with apoptotic cells on biopsy.  Patients can also have a T+B-NK- phenotype.  It sounds like it would be worthwhile to send telomere length testing for this patient.



Best,


Soma Jyonouchi, MD
Children's Hospital of Philadelphia
Division of Allergy and Immunology
Phone (215) 590-2549<tel:%28215%29%20590-2549>
Fax (215) 590-4529<tel:%28215%29%20590-4529>
________________________________
From: Yeşim Yılmaz Demirdağ [dryesimyilmaz at gmail.com<mailto:dryesimyilmaz at gmail.com>]
Sent: Monday, October 14, 2013 10:16 AM
To: CIS-PIDD
Subject: [cis-pidd] Newborn with chronic diarrhea and near absent plasma cells in the gut mucosa

Dear all,

I would like to ask your advice in this case:

The baby is a one-month-old boy, he is ex-premie at 35 wks EGA.

Pregnancy was uncomplicated with the exception of unknown GBS status. Baby had some apnea episodes and stayed in NICU for 2 days on Amp and Gent. He was sent home on day 3, but readmitted on day 6 with hypovolemic shock and kidney failure due to severe diarrhea.

Cultures were all negative. The stool pH was 3.8.  Stool reducing substances, eosinophils, wright stain, and Charcot-Leyden crystals were negative.  Stool guaiac was +.  Stool cultures, ova and parasite exams, and rotavirus antigen studies were negative.  Stool amylase was <3, lipase was 20, and fecal fat was borderline elevated.  Neutral fat was <60 droplets/HPOF, with total fat <100 droplets/HPF.   Giardia and Cryptococcus were negative. No viral study was sent at that time.

Intestinal biopsy report: Small intestinal mucosa with villous blunting and rare intraepithelial lymphocytes, with paucity to nearly absent mucosal plasma cells. Gastric oxyntic mucosa with a rare apoptotic body. Negative for Helicobacter pylori (H&E stain). Rectum, biopsy: Colonic mucosa with crypt distortion and crypt apoptosis and absence of lamina propria plasma cells.


Immunoglobulins: IgG: 474 mg/dl, IgA < 6 mg/dl, IgM: 18.8 mg/dl (day 25 of life)


Lymphocyte subsets: Normal T cells, slightly decreased NK cells (144 cells/mcl (170- 1100), and low B cells (5%, absolute: 118 cells/mcl).


He is clinically well now on TPN, slowly tolerating elemental formula. He has not been on any antibiotics except for the first few days of life.


Thank you and have a great week!


Yesim



Yesim Yilmaz Demirdag, MD

Assistant Professor of Pediatrics

Division of Allergy and Immunology

Columbia University Medical Center

3959 Broadway, Room 107N

New York, NY 10032

phone: (212) 305 2300<tel:%28212%29%20305%202300>






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Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
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(f) 267-426-0363



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