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

[Jyonouchi, Soma C]

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
Fax (215) 590-4529
________________________________
From: Yeşim Yılmaz Demirdağ [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





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