Lipid apheresis effectively reduced triglyceride levels from 8652 mg/dL to 346 mg/dL at 3 months but did not prevent severe hypotension leading to panhypopituitarism.
Severe hypertriglyceridemia-induced pancreatitis in pregnancy can lead to catastrophic outcomes including fetal death and panhypopituitarism despite effective triglyceride lowering with lipopheresis.
Absolute Event Rate: 0% vs 0%
Abstract Introduction Hypertriglyceridemia-related pancreatitis (HIP) in pregnancy is associated with higher risks of organ dysfunction, intensive care requirement, and complications including fetal death, preterm birth, and placental abruption. We present a case with high mortality risk and unexpected long-term sequelae. Clinical Case A 34-year-old woman, 25 weeks pregnant, had four prior HIP episodes causing two miscarriages and two intrauterine fetal deaths between weeks 20–25. She presented with severe nausea, vomiting, and epigastric pain radiating to the back for 10 hours. History included type 2 diabetes; she was on insulin detemir 10 U/day and fenofibrate 267 mg/day, though recent compliance was poor. On physical examination: Blood pressure was 135/80 mmHg, pulse was 109/min regular, temperature was 36.7°C, respiratory rate was 22/min. Laboratory results showed Lipase:179 U/L (0–67) Amylase:49 U/L (28–100) Triglycerides:8652 mg/dL (0–150) WBC:10.9×10³/µL, NEU:8.9×10³/µL CRP:9 mg/dL. Renal and liver function tests, LDH could not be measured due to lipemic serum. (Table-1) HIP was diagnosed; oral intake stopped. IV insulin 8 U/h, 0.9% NaCl 200 mL/h, and 5% dextrose 50 mL/h were started. Fetal heart activity was normal initially. After six hours, the patient developed fever 38.5°C, hypotension 80/50 mmHg, and confusion. She was transferred to ICU, intubated, and underwent 2 days of lipoprotein apheresis (Table-2). Meropenem and vasopressors were initiated. By Day 1’s end, obstetric reassessment revealed absent fetal heart activity. On Day 5, labor was induced, and she delivered a stillborn. Abdominal CT showed necrotizing pancreatitis. After 3 weeks of treatment, she was discharged. Outpatient triglycerides at 3 and 6 months were 346 and 228 mg/dL, respectively. At 6 months post-ICU, she presented with fatigue, amenorrhea, muscle weakness, 30 kg weight loss, slow speech, and alopecia. Examination revealed myxedematous facies, dry skin, alopecia, bone tenderness; pulse 44/min. TSH: 0.9 mU/L (0.5–4) Free T4: 0.25 ng/dL (0.58–1.38) Free T3: 0.88 ng/dL (2.1–4.5) Morning cortisol: 0.4 µg/dL (5–22.6) ACTH: 11 pg/mL (46) FSH: 6.6 IU/L, LH: 2.3 IU/L, estradiol: 15 ng/L IGF-1: 37 µg/L (92–222) Panhypopituitarism secondary to septic shock and hypotension was diagnosed. Pituitary MRI showed partial empty sella.(Figure-1) Hydrocortisone 100 mg IV loading, followed by 50 mg q6h, was initiated; on Day 3, levothyroxine 100 mcg/day was added. Muscle strength and skin color improved rapidly. A week later, Free T4 0.50 ng/dL and Free T3 1.27 ng/dL normalized partially. She was discharged on prednisolone 5 mg AM+2.5 mg PM and combined oral contraceptives. Conclusion Lipopheresis is an effective treatment for HIP but does not always prevent catastrophic outcomes. Severe hypotension can cause irreversible damage, particularly panhypopituitarism, in pregnant women with an enlarged pituitary gland.Figure 1:Partial Empty Sella Table 1:Laboratory ResultsWorsening acidosis on arterial blood gas analysis and a marked elevation in pancreatitis markers are observed. Table 2:Effect of Therapeutic Lipid Apheresis on Triglyceride Levels
Salva et al. (Thu,) reported a other. Lipid apheresis effectively reduced triglyceride levels from 8652 mg/dL to 346 mg/dL at 3 months but did not prevent severe hypotension leading to panhypopituitarism.