Endocrine Abstracts

Lipodystrophy syndromes are a rare and heterogeneous disease characterized by loss of subcutaneous adipose tissue, deposition of fat in ectopic areas, and consequently metabolic impairment, and its recognition is based on physical examination and clinical history. Because of the complexity of the disease, pregnancy represents a dainty and challenging event, still understudied. Here we report a case of a 28 year-old Caucasian female followed for type 2 diabetes and hypertriglyceridemia since she was 21 in the context of acquired partial lipodystrophy. Past medical history included childhood lymphocytic leukemia treated by chemo- and radiotherapy and allogeneic bone marrow transplant and subsequently she developed heart disease with left ventricular dysfunction (ejection fraction EF 45% in 2007) that was treated with beta-blocker and ACEi, and it normalized during follow-up. Diabetes mellitus wasn’t adequately controlled (time in range TIR 5%, time above range TAR 95%, time below range TBR 0%, glucose management indicator GMI 11.3%) with insulin, metformin, and semaglutide; hypertriglyceridemia was discreetly controlled with PUFA and fenofibrate. In December 2021 she became unexpectedly pregnant and semaglutide, metformin, fenofibrate, and ACEi were stopped, PUFA was reduced and, after gynecological consultation, acetylsalicylic acid was added. During pregnancy Ambulatory Glucose Profile (AGP) reports improved with a TIR in the third trimester of 77%. Leptin values were periodically evaluated during pregnancy and in the early post-partum period: a progressive increase of levels was shown, with a peak in the third trimester (41.53 ng/ml), then after the delivery, there was a decline. Despite achieving good glycemic control, at 33 weeks of gestation, the patient developed acute pulmonary edema with the need for urgent cesarean delivery. The echocardiographic evaluation showed severe left ventricular diastolic dysfunction (FE 30%) that was treated with invasive ventilation (NIV), diuretic, nitroglycerin, inotropic drug, and ivabradine. No adipose tissue depots were observed at cardiac MRI. Furthermore, on suspicion of Sheehan syndrome, the patient started cortone acetate and then discontinued due to evidence of normal HPA axis function. The newborn was in good general condition except for the development of transient hypoglycemia and the finding of triple X syndrome. The management of lipodystrophies in pregnancy appears to be challenging and still poorly studied. Increased leptin values may suggest placental or fetal involvement in the metabolic improvement of the patient.