The patient is a 28-year-old Hispanic female with a past medical history of\nhypertension, high serum cholesterol, and gastritis who presented to the emergency\nroom for the evaluation of abdominal pain of 2 days duration. Six months prior, she\nhad similar pain. An upper gastrointestinal (GI) endoscopy at that time showed\npeptic ulcer disease and no other mucosal lesions. The patient had a strong family\nhistory of renal disease and 2 of her siblings required a renal transplant, but she\ndid not know what kidney pathology they had. Her medications included Lisinopril\noral 10 mg tablet daily and Pantoprazole oral 40 mg daily. On presentation at the\nemergency room (ER), her height and weight were 157 cm and 61 kg, respectively, and\nher blood pressure was 206/121 mm Hg. On physical examination, the patient had\nepigastric tenderness. The rest of her physical examination was unremarkable.\nRadiology was ordered to exclude any anatomical abnormality. A computed tomography\nscan showed neither atrophy nor enlargement of the kidneys. She then followed up as\nan outpatient with a nephrologist shortly after her ER visit. Her laboratory work up\nduring that visit revealed the following: nephrotic range proteinuria (9.2 g protein\non 24-hour urine collection; reference range, 0-2.5 g/d), hypoalbuminemia (300\nµmol/L; reference range, 421-662 µmol/L), hypoproteinemia (50 g/L; reference range,\n60-80 g/L), elevated serum creatinine (148.5 µmol/L; reference range, 44-80 μmol/L),\nhigh chloride (110 mmol/L; reference range, 98-106 mmol/L), and low hemoglobin (100\ng/L; reference range, 120-160 g/L). The lipid profile showed an increased total\ncholesterol level (8.3 mmol/L; reference range, <5.2 mmol/L), elevated\ntriglycerides (3.7 mmol/L; reference range, <1.70 mmol/L), elevated low-density\nlipoprotein fraction (5.9 mmol/L; reference range, <2.6 mmol/L), and decreased\nhigh-density lipoprotein fraction (1.1 mmol/L; reference range, ≥1.55 mmol/L). After\ntreating her blood pressure, the patient underwent a US-guided renal biopsy.
A 51-year-old Japanese woman was admitted for evaluation of proteinuria in 2011. She had a history of appendicitis at 12 years of age, cholelithiasis at 30 years of age, myoma at 46 years of age, and at least a 5-year history of dyslipidemia. Proteinuria (1+) had been first identified during a medical checkup 3 years prior and was ignored until increasing to 3+ in 2011. Her parents and brothers suffered from dyslipidemia but had no history of renal disease.\nOn admission, her height and weight were 161 cm and 49 kg, respectively, and her blood pressure was 137/94 mm Hg. On physical examination, slight pitting edema was observed; however, there were no xanthomas. Urinary protein excretion was 4.12 g/g Cre, and the serum triglyceride level was high. She did not exhibit nephrotic syndrome. The other laboratory findings are shown in Table . A computed tomography scan showed neither atrophy nor enlargement of the kidneys.\nA renal biopsy specimen was processed in August 2011 using standard methods, and in the sections for light microscopy, the renal cortex was found to contain 11 glomeruli, 2 of which showed sclerosis. The remaining glomeruli were enlarged by distinct dilatation of the capillary lumina, which were filled with thrombus-like substances that stained slightly with periodic acid-Schiff. Some glomeruli exhibited moderate mesangial cell proliferation and increased mesangial matrix, and double contours of the capillary wall were partially observed. No crescents or adhesion in glomeruli were found (Fig. –c). A direct immunofluorescence showed no deposition of IgG, IgA, IgM, C1q, C3, or fibrinogen in glomeruli. Staining with Oil red O showed lipid droplets in the capillary lumina, which were stained positive for apoE (Fig. , e). Electron microscopic findings showed capillary dilation and occlusion with numerous lamellated lipid granules. Thin basement membranes with swollen endothelial cells were also observed. The histological findings and the results of apoE analysis (described below) satisfied the diagnostic criteria for LPG [].\nTreatment with fenofibrate and irbesartan was initiated. Two weeks later, serum triglycerides and urinary protein excretion were reduced to 200 mg/dL and 1 g/g Cre, respectively. Two months later, the former was within the normal range, and the latter was reduced to less than 0.5 g/g Cre. Eight months later, urinary protein excretion ceased and did not recur thereafter (Fig. ).\nIn a second biopsy in August 2013, the specimen contained 22 glomeruli, 4 of which were obsolescent. Two of the glomeruli were filled with thrombi, but thrombi were absent in the other glomeruli (Fig. ).