PMID- 33597314
OWN - NLM
STAT- Publisher
LR  - 20240222
IS  - 2052-0573 (Print)
IS  - 2052-0573 (Electronic)
IS  - 2052-0573 (Linking)
VI  - 2021
DP  - 2021 Feb 17
TI  - Primary hyperparathyroidism and Zollinger Ellison syndrome during pregnancy: a 
      case report.
LID - EDM200130 [pii]
LID - 10.1530/EDM-20-0130 [doi]
LID - 20-0130
AB  - SUMMARY: Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited endocrine 
      disorder with a high rate of penetrance. The incidence of MEN1 is 1/30,000 in the 
      general population; however, it is quite rare for a patient to present for 
      medical attention with MEN1 for the first time in pregnancy. Primary 
      hyperparathyroidism (PHPT) is one of the most common features of MEN1. The 
      incidence of PHPT occurring in pregnancy is 1%. Despite advances in the medical, 
      surgical and obstetric care over the years, management of this condition during 
      pregnancy may be challenging. It can be difficult to identify pregnant women with 
      PHPT requiring intervention and to monitor safely. Hypercalcemia can result in 
      significant maternal and fetal adverse outcomes including: miscarriage, 
      intrauterine growth restriction, preterm delivery, neonatal hypocalcaemia, 
      pre-eclampsia and maternal nephrolithiasis. Herein, we present a case study of a 
      lady with a strong family history of MEN1, who was biochemically proven to have 
      PHPT and evidence of Zollinger Ellison Syndrome (ZE) on endoscopy. This patient 
      delayed her assisted pregnancy plans for in vitro fertilization (IVF) until 
      completion of the MEN1 workup; nevertheless, she spontaneously achieved an 
      unplanned pregnancy. As a result, she required intervention with 
      parathyroidectomy in the second trimester of her pregnancy as her calcium level 
      continued to rise. This case study highlights the workup, follow up and 
      management of MEN1 presenting with PHPT and ZE in pregnancy. LEARNING POINTS: 
      Women of childbearing age who are suspected to have a diagnosis of primary 
      hyperparathyroidism ideally should have genetic testing and avoid pregnancy until 
      definitive plans are in place. Zollinger Ellison syndrome in pregnancy means 
      off-label use of high dose of proton pump inhibitors (PPI). Use of PPI in 
      pregnancy is considered to be safe based on retrospective studies. Omeprazole, 
      however, is FDA class C drug because of lack of large prospective studies or 
      large case series during pregnancy. Calcium supplements in the form of calcium 
      carbonate must be converted to calcium chloride by gastric acid in order to be 
      absorbed, however, patients rendered achlorhydric as a result of PPI use will 
      have impaired absorption of calcium. Therefore, use of calcium citrate might be 
      considered a better option in this case.
FAU - Ali, Dalal
AU  - Ali D
AUID- ORCID: 0000-0002-5378-5548
AD  - St. Vincent's University Hospital, Dublin, Ireland.
FAU - Divilly, Patrick
AU  - Divilly P
AD  - St. Vincent's University Hospital, Dublin, Ireland.
FAU - Prichard, Ruth
AU  - Prichard R
AD  - St. Vincent's University Hospital, Dublin, Ireland.
FAU - O'Toole, Dermot
AU  - O'Toole D
AD  - St. Vincent's University Hospital, Dublin, Ireland.
AD  - St. James's University Hospital, Dublin, Ireland.
AD  - Trinity College, Dublin, Ireland.
FAU - O'Shea, Donal
AU  - O'Shea D
AD  - St. Vincent's University Hospital, Dublin, Ireland.
AD  - University College Dublin, Dublin, Ireland.
FAU - Crowley, Rachel K
AU  - Crowley RK
AD  - St. Vincent's University Hospital, Dublin, Ireland.
AD  - University College Dublin, Dublin, Ireland.
LA  - eng
PT  - Journal Article
DEP - 20210217
PL  - England
TA  - Endocrinol Diabetes Metab Case Rep
JT  - Endocrinology, diabetes & metabolism case reports
JID - 101618943
PMC - PMC7923119
EDAT- 2021/02/19 06:00
MHDA- 2021/02/19 06:00
PMCR- 2021/02/17
CRDT- 2021/02/18 05:55
PHST- 2020/08/02 00:00 [received]
PHST- 2021/01/25 00:00 [accepted]
PHST- 2021/02/19 06:00 [pubmed]
PHST- 2021/02/19 06:00 [medline]
PHST- 2021/02/18 05:55 [entrez]
PHST- 2021/02/17 00:00 [pmc-release]
AID - EDM200130 [pii]
AID - 10.1530/EDM-20-0130 [doi]
PST - aheadofprint
SO  - Endocrinol Diabetes Metab Case Rep. 2021 Feb 17;2021:20-0130. doi: 
      10.1530/EDM-20-0130.