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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