[CIS PIDD] [cis-pidd] CVID and profuse diarrhea

[CIS-PIDD]

Dear all,

I have a difficult case of CVID patient whom I recently started to see and
would like to ask your opinions

- 18 year old female diagnose as CVID at the age of 3 years in 2002
- currently, bronchiectasia with probable invasive aspergilosis, liver
cirrhosis, renal tubulopathy, and *significant diarrhea with PCR only
positive salmonella and norovirus*

<2002>
- was diagnosed as CVID with a hx of frequent pneumonia
- managed to grow along growth curve until age 10-11
- failure to grow since age 12
- performed ok school life on IVIG without hospitalization until 2010-11

<20010-11>
- hospitalization for work-up on poor weight gain and GI pathology
- 2010: pathology reported, esophageal varix grade I,  duodenal bx showed
atrophy of villi, apoptotic bodies with increased intraepithelial
lymphocytes, abdomen CT showed multiple LN enlargement and enterocolitis,
hepatosplenomegaly
- 2011: bx of terminal ileum showed also above findings and intestinal
lymphangiectaisa

<2015>
- In August, she developed really really serious profuse diarrhea.
- significant weight loss and now the body weight is  25-30 kg, seriously
cachexia condition since then
- extensive parasite, bacterial, viral, fungal infection as for enteric
pathogen revealed positive PCR for salmonella spp. and norovirus in the
stool and no other cultured organisms
- colon bx showed lots of damaged mucosa and massive neutrophil
infiltration, crypt abscesses, increased lymphocyte infiltration than
before.
- I reviewed the slides with the pathologist and he says her pathology
looks somewhat different from inflammatory bowel diseases (UC or Crohn's
disease)

<2016>
- Early this month, she presented to ER with hemopysis -> w/u showed
possible invasive  pulmonary aspergillois, for this she is on *ambisome*
- She still has serious diarrhea 15 times per day.  Her output is almost
over 3000 cc and she is 26 kg today.
- Salmonella and norovirus PCR is still positive as of Jan 2016 >)
oral *rifaximin
and ribavirin* were started 5 days ago.
- It seems like whatever she eats or we infuse, she cannot hold nutrients,
electrolytes in her body.

<labs>
*- Initial labs in July 2002 was *
lympho subset: CD3 2870, CD4 1790, CD8 1080, B cells 160 (5%), NK cell 5
Ig G 33, IgA 6, IgM 4 mg/dL
- As I reviewed her past labs, her IgG level has been in 200-400 mg/dL on
IVIG tx and IgA and IgM levels has been also less than 5 mg/dL..

*- Labs in December 2015 was*
CD3 2072 cells /uL, CD4 833, CD8 1175, *B cells 0*, NK 43 cells/uL
hypoalbuminemia, electrolyte imbalance, etc..

At this point, I would like to ask your opinion on management option. Is
there anything that I can do further for her?
- I plan to f/u PCR for salmonella and norovirus after two wks of tx and
also perform terminal ileum and colon bx to see the pathology change. But I
am not sure whether pathogens would be easily cleared and the pathology
would be improved..
- I am thinking she might need some immunomoluatory agents or steroid..but
I am concerned with her lung (aspergillosis) and gut pathogens (salmonella
and noro) at this point..Maybe can I try at least oral budesonide?

I would appreciate your thoughts and suggestion.

Regards,

YaeJean


Yae-Jean Kim, MD, PhD

Associate Professor
Division of Infectious Diseases and Immunodeficiency
Department of Pediatrics,
Samsung Medical Center,
Sungkyunkwan University School of Medicine,
81 Irwon-ro, Gangnam-gu, Seoul, South Korea

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