PMID- 31096181 OWN - NLM STAT- Publisher LR - 20240227 IS - 2052-0573 (Print) IS - 2052-0573 (Electronic) IS - 2052-0573 (Linking) VI - 2019 DP - 2019 May 16 TI - Management of primary hyperparathyroidism in pregnancy: a case series. LID - EDM190039 [pii] LID - 10.1530/EDM-19-0039 [doi] LID - 19-0039 AB - Primary hyperparathyroidism (PHPT) is characterised by the overproduction of parathyroid hormone (PTH) due to parathyroid hyperplasia, adenoma or carcinoma and results in hypercalcaemia and a raised or inappropriately normal PTH. Symptoms of hypercalcaemia occur in 20% of patients and include fatigue, nausea, constipation, depression, renal impairment and cardiac arrythmias. In the most severe cases, uraemia, coma or cardiac arrest can result. Primary hyperparathyroidism in pregnancy is rare, with a reported incidence of 1%. Maternal and fetal/neonatal complications are estimated to occur in 67 and 80% of untreated cases respectively. Maternal complications include nephrolithiasis, pancreatitis, hyperemesis gravidarum, pre-eclampsia and hypercalcemic crises. Fetal complications include intrauterine growth restriction; preterm delivery and a three to five-fold increased risk of miscarriage. There is a direct relationship between the degree of severity of hypercalcaemia and miscarriage risk, with miscarriage being more common in those patients with a serum calcium greater than 2.85 mmol/L. Neonatal complications include hypocalcemia. Herein, we present a case series of three women who were diagnosed with primary hyperparathyroidism in pregnancy. Case 1 was diagnosed with multiple endocrine neoplasia type 1 (MEN1) in pregnancy and required a bilateral neck exploration and subtotal parathyroidectomy in the second trimester of her pregnancy due to symptomatic severe hypercalcaemia. Both case 2 and case 3 were diagnosed with primary hyperparathyroidism due to a parathyroid adenoma and required a unilateral parathyroidectomy in the second trimester. This case series highlights the work-up and the tailored management approach to patients with primary hyperparathyroidism in pregnancy. Learning points: Primary hyperparathyroidism in pregnancy is associated with a high incidence of associated maternal fetal and neonatal complications directly proportionate to degree of maternal serum calcium levels. Parathyroidectomy is the definitive treatment for primary hyperparathyroidism in pregnancy and was used in the management of all three cases in this series. It is recommended when serum calcium is persistently greater than 2.75 mmol/L and or for the management of maternal or fetal complications of hypercalcaemia. Surgical management, when necessary is ideally performed in the second trimester. Primary hyperparathyroidism is genetically determined in ~10% of cases, where the likelihood is increased in those under 40 years, where there is relevant family history and those with other related endocrinopathies. Genetic testing is a useful diagnostic adjunct and can guide treatment and management options for patients diagnosed with primary hyperparathyroidism in pregnancy, as described in case 1 in this series, who was diagnosed with MEN1 syndrome. Women of reproductive age with primary hyperparathyroidism need to be informed of the risks and complications associated with primary hyperparathyroidism in pregnancy and pregnancy should be deferred and or avoided until curative surgery has been performed and calcium levels have normalised. FAU - McCarthy, Aisling AU - McCarthy A AD - University Hospital Galway, Galway, Ireland. FAU - Howarth, Sophie AU - Howarth S AD - Department of Diabetes and Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Khoo, Serena AU - Khoo S AD - Department of Diabetes and Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Hale, Julia AU - Hale J AD - Department of Diabetes and Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Oddy, Sue AU - Oddy S AD - Department of Clinical Biochemistry, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Halsall, David AU - Halsall D AD - Department of Clinical Biochemistry, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Fish, Brian AU - Fish B AD - Department of Head and Neck Surgery, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Mariathasan, Sashi AU - Mariathasan S AD - Department of Diabetes and Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Andrews, Katrina AU - Andrews K AD - East Anglian Medical Genetics Service, Cambridge University Hospitals NHS Foundation Trust, Cambridge Biomedical Campus, Cambridge, UK. FAU - Oyibo, Samson O AU - Oyibo SO AD - Department of Diabetes and Endocrinology, Peterborough City Hospital, North West Anglia NHS Foundation Trust, Peterborough, UK. FAU - Samyraju, Manjula AU - Samyraju M AD - Department of Obstetrics and Gynecology, Peterborough City Hospital, North West Anglia NHS Foundation Trust, Peterborough, UK. FAU - Gajewska-Knapik, Katarzyna AU - Gajewska-Knapik K AD - Department of Obstetrics and Gynecology, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Park, Soo-Mi AU - Park SM AD - East Anglian Medical Genetics Service, Cambridge University Hospitals NHS Foundation Trust, Cambridge Biomedical Campus, Cambridge, UK. FAU - Wood, Diana AU - Wood D AD - Department of Diabetes and Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Moran, Carla AU - Moran C AD - Department of Diabetes and Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK. FAU - Casey, Ruth T AU - Casey RT AD - Department of Diabetes and Endocrinology, Cambridge University NHS Foundation Trust, Cambridge, UK. AD - Department of Medical Genetics, Cambridge University, Cambridge, UK. LA - eng PT - Journal Article DEP - 20190516 PL - England TA - Endocrinol Diabetes Metab Case Rep JT - Endocrinology, diabetes & metabolism case reports JID - 101618943 PMC - PMC6528402 OTO - NOTNLM OT - 2019 OT - Abdominal discomfort OT - Bone OT - CT scan OT - Calcium (serum) OT - Calcium to creatinine clearance ratio OT - Diarrhoea OT - Diet OT - Fatigue OT - Female OT - Fluid repletion OT - Gestational diabetes mellitus OT - Headache OT - Histopathology OT - Hypercalcaemia OT - Hyperparathyroidism (primary) OT - MEN1 OT - May OT - Molecular genetic analysis OT - Nausea OT - Novel diagnostic procedure OT - PTH OT - Parathyroid adenoma OT - Parathyroid hyperplasia OT - Parathyroidectomy OT - Phosphate (serum) OT - Polydipsia OT - Polyuria OT - Pregnant adult OT - SPECT scan OT - Sestamibi scan OT - Ultrasound scan OT - United Kingdom OT - Vitamin D OT - Vomiting OT - White EDAT- 2019/05/17 06:00 MHDA- 2019/05/17 06:00 PMCR- 2019/05/16 CRDT- 2019/05/17 06:00 PHST- 2019/04/12 00:00 [received] PHST- 2019/04/26 00:00 [accepted] PHST- 2019/05/17 06:00 [entrez] PHST- 2019/05/17 06:00 [pubmed] PHST- 2019/05/17 06:00 [medline] PHST- 2019/05/16 00:00 [pmc-release] AID - EDM190039 [pii] AID - 10.1530/EDM-19-0039 [doi] PST - aheadofprint SO - Endocrinol Diabetes Metab Case Rep. 2019 May 16;2019:19-0039. doi: 10.1530/EDM-19-0039.