PMID- 30326482
OWN - NLM
STAT- MEDLINE
DCOM- 20200116
LR  - 20200116
IS  - 1663-2826 (Electronic)
IS  - 1663-2818 (Linking)
VI  - 91
IP  - 4
DP  - 2019
TI  - Perioperative Hypotensive Crisis in an Adolescent with a Pancreatic VIPoma and 
      MEN1-Gene Variant.
PG  - 285-289
LID - 10.1159/000493396 [doi]
AB  - BACKGROUND: Vasoactive intestinal peptide-secreting tumours (VIPomas) lead to 
      high-volume secretory diarrhoea with hypokalaemia, as well as hyperglycaemia and 
      hypercalcaemia. Diagnosis is often delayed. CASE DESCRIPTION: We present a 
      13-year-old girl with a distal pancreatic VIPoma diagnosed on her second hospital 
      presentation who became severely hypotensive on anaesthetic induction prior to 
      tumour removal, likely due to the vasodilatory effect of supraphysiological VIP 
      levels. Prior to the second surgical attempt, an octreotide infusion was started 
      preoperatively to suppress systemic VIP levels and counter the potential for 
      VIP-induced hypotension upon tumour manipulation, and the tumour was successfully 
      resected. Hyperparathyroidism and history of GI tumour resection were 
      subsequently identified in the father, and the two members were found to have a 
      heterozygous variant of uncertain significance in the multiple endocrine 
      neoplasia type 1 (MEN1) gene. However, as this family meets the diagnostic 
      criteria for MEN1 clinically, ongoing surveillance for MEN1 tumours and genetic 
      counseling for at-risk family members are required despite the non-pathogenic 
      genetic result. CONCLUSION: This case highlights the importance of screening for 
      a VIPoma in patients with high-volume secretory diarrhoea and preventing 
      cardiovascular complications with perioperative VIP suppression. Furthermore, 
      careful interpretation of genetic results within the clinical context is 
      required.
CI  - (c) 2018 S. Karger AG, Basel.
FAU - Acosta-Gualandri, Alejandra
AU  - Acosta-Gualandri A
AD  - Division of Endocrinology, Department of Pediatrics, British Columbia Children's 
      Hospital and The University of British Columbia, Vancouver, British Columbia, 
      Canada.
FAU - Kao, Kung-Ting
AU  - Kao KT
AD  - Division of Endocrinology, Department of Pediatrics, British Columbia Children's 
      Hospital and The University of British Columbia, Vancouver, British Columbia, 
      Canada.
FAU - Wong, Tiffany
AU  - Wong T
AD  - Division of Allergy and Immunology, Department of Pediatrics, British Columbia 
      Children's Hospital and The University of British Columbia, Vancouver, British 
      Columbia, Canada.
FAU - Webber, Eric
AU  - Webber E
AD  - Department of Pediatric Surgery, British Columbia Children's Hospital, Vancouver, 
      British Columbia, Canada.
FAU - Armstrong, Linlea
AU  - Armstrong L
AD  - Department of Medical Genetics, British Columbia Children's Hospital, Vancouver, 
      British Columbia, Canada.
FAU - Panagiotopoulos, Constadina
AU  - Panagiotopoulos C
AD  - Division of Endocrinology, Department of Pediatrics, British Columbia Children's 
      Hospital and The University of British Columbia, Vancouver, British Columbia, 
      Canada, dpanagiotopoulos@cw.bc.ca.
LA  - eng
PT  - Case Reports
DEP - 20181016
PL  - Switzerland
TA  - Horm Res Paediatr
JT  - Hormone research in paediatrics
JID - 101525157
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adolescent
MH  - Female
MH  - *Genetic Variation
MH  - Humans
MH  - *Hypotension/genetics/physiopathology
MH  - *Pancreatic Neoplasms/genetics/physiopathology/surgery
MH  - *Perioperative Period
MH  - Proto-Oncogene Proteins/*genetics
MH  - *Vipoma/genetics/physiopathology/surgery
OTO - NOTNLM
OT  - MEN1
OT  - Multiple endocrine neoplasia
OT  - Neuroendocrine tumour
OT  - VIPoma
OT  - Vasoactive intestinal polypeptide
OT  - Watery diarrhoea syndrome
EDAT- 2018/10/17 06:00
MHDA- 2020/01/17 06:00
CRDT- 2018/10/17 06:00
PHST- 2018/05/24 00:00 [received]
PHST- 2018/08/30 00:00 [accepted]
PHST- 2018/10/17 06:00 [pubmed]
PHST- 2020/01/17 06:00 [medline]
PHST- 2018/10/17 06:00 [entrez]
AID - 000493396 [pii]
AID - 10.1159/000493396 [doi]
PST - ppublish
SO  - Horm Res Paediatr. 2019;91(4):285-289. doi: 10.1159/000493396. Epub 2018 Oct 16.