Dear Mohamed,
This is likely to be adult onset nesidioblastosis, noninsulinoma pancreatogenous hypoglycaemia syndrome (NIPHS), and he may need further surgery in the form of subtotal pancreatectomy.
Best regards
Mohammad
Dr. M A Al-Jubouri, MB ChB, MSc, EurClinChem, FRCP Edin, FRCPath
Consultant Chemical Pathologist
--------------------------------------------
On Thu, 8/1/15, Mohamed Elsammak <[log in to unmask]> wrote:
Subject: Unusual hypoglycemia case
To: [log in to unmask]
Date: Thursday, 8 January, 2015, 9:43
Dear colleagues:
We have this interesting case during our weekly joint
endocrine/laboratory meeting. I will grateful for advice
whether anything was missed, thoughts suggestions other
possible diagnosis.
----------------------------------------------
21 years old gentleman , not known to have any
medical problem, presented with progressive symptoms of
hypoglycemia.
He was investigated and found to have endogenous
hyperinsulinemia based on the following labs:
RBS 1.7 mmol/l
Insulin 99.4 pmol/l
Proinsulin 8.1 pmol/l
c-peptide 373 pmol/l
sulfonylurea screen : all were –ve
Autoimmune panel were –ve.
Pancreas CT, MRI and EUS all were normal.
Calcium stimulation test showed the following ( all unit
in pmol/l)
GD
SA
SMA
0
1106
875
1286
30
1757
2274
1955
60
1700
1815
1609
90
1751
1486
1421
120
1769
1370
1335
Based on that he was sent to OR and intraop US was
normal, so the decision was to remove the tail and part of
the body of the pancreas based on the increment (more than
2.5 folds in insulin level in teh splenic
artery) . Post-operatively, histopathology resultr
showed (feature suggestive of
islet cell hyperplasia).
For one month patient improved and he reported no more
hypoglycemia , after that he started to have attacks of
hypoglycemia , so Diazoxide was initiated with some
response, but for the last 2 months patient has frequent
hypoglycemia which not respond to any of the medical
therapy.
He showed no response to octeriotides and now kept
dextrose
Repeated labs during this admission showed same
endogenous hyperinsulinemia with no evidence of sulfonylurea
abuse.
Any thoughts or experience with similar cases will be
highly appreciated
Kind regards
Mohamed
Dr Mohamed Elsammak
MD, MSc, PhD, FRCPath
Consultant Chemical Pathologist
King Fahd Specialist Hospital Dammam
Tel: 0096638442222 x 1421
00966502952230
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