Endocrine Abstracts

Background: A 23-year-old woman diagnosed with Dunnigan-type familial partial lipodystrophy (FPLD) attended the joint Antenatal/Endocrine Clinic at 13 weeks of gestation. She was diagnosed at age 7 and subsequently developed type 2 diabetes at age 11 years. She was managed initially with metformin followed by addition of insulin. It was an unplanned pregnancy and her booking HbA1c was 8.4% (IFCC 68 mmol/mol).

Prior to pregnancy, she was on a basal bolus regime of Levemir and Novorapid and her total daily insulin dose was ~180 units. At booking, we added Metformin 500 mg twice daily, which was later increased to three times daily.

Her insulin requirements escalated rapidly to a total daily dose of 250 units by 25 weeks gestation. At this stage, the use of Humulin R (U500) was considered and discussed with the patient. However, she presented with vaginal spotting and went into spontaneous labour at 26 weeks. She delivered a live female infant who was admitted briefly to the special care baby unit. During labour she was managed with intravenous sliding scale insulin.

Conclusion: Familial Lipodystrophy is a group of rare disorders associated with numerous metabolic complications. Diabetes, familial lipodystrophy and pregnancy in combination all compound and confer a severe insulin resistant state which if poorly controlled, can have an adverse effect on pregnancy outcome. Data on the use of U500 insulin during pregnancy is limited however there are a handful of case reports of its use in pregnancy with successful management of glycaemic and obstetric outcome.

This case is a reminder of the challenges in the glycaemic management of patients with lipodystrophy particularly in pregnancy. It also adds to the limited literature available on pregnancy outcome of patients with lipodystrophy.