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

[Yeşim Yılmaz Demirdağ]

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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