ABSTRACT
Case history A 41-year-old man was admitted because of acute, severe central abdominal pain and vomiting. He was a heavy drinker and had smoked about 40 cigarettes/day for the past 20 years. His temperature was 37.2°C, blood pressure 134/82 mm Hg, pulse rate 110 beats/min, and respiration rate 30 breaths/min. He was overweight (BMI of 27 kg/m2). On examination, generalized eruptive xanthomata were observed all over the trunk, back, buttocks, arms, legs (Figure 1a), and knees, while fundoscopy revealed lipemia retinalis (Figure 1b). Abdominal distension, tenderness, and absent sounds on auscultation indicated the presence of ileus. An electrocardiogram showed sinus tachycardia, and a chest X-ray was normal. Abdominal films showed a proximal jejunum ‘sentinel loop’ and a CT scan of the abdomen verified pancreatic inflammation and mild splenomegaly without evidence of gallstones.The appearance of the patient’s serum was milky (Figure 1c). Laboratory testing showed leukocytosis (white blood cell count, 11.8 × 103/mm3 with 80% neutrophils), serum glucose 480 mg/dl (26.7 mmol/l), creatinine 1.6 mg/dl (141 µmol/l, reference range 53-106 µmol/l), serum sodium 129 mmol/l (reference range 135-145 mmol/l), total cholesterol 830 mg/dl (21.5 mmol/l), triglycerides 8900 mg/dl (100 mmol/l), and HDL-cholesterol 32 mg/dl (0.8 mmol/l). After diluting serum to ‘remove’ the effect of lipid particles, hyperamylasemia was evident (395 IU/l (upper normal limit, 90 IU/l), while the urine amylase was 5410 IU/l (upper normal limit, 600 IU/l). Arterial pH was 7.2, pCO2 14 mm Hg, and bicarbonate 6 mmol/l. A urine specimen revealed glycosuria and ketonuria. Agarose gel lipoprotein electrophoresis revealed increased chylomicrons and very-low-density lipoproteins (VLDLs).
