A 58-year-old male was referred to our center by his primary care physician due to nephrotic syndrome. He had massive proteinuria (7.38 g/day) and hypoalbuminemia (1.9 g/dl). He had been treated for diabetes since the age of 50 years and had been started on insulin therapy at the age of 55 years. He had been well-managed (HbA1c, 5.3%), although a pre-proliferative retinopathy and mild renal dysfunction (sCr, 1.38 mg/dl) became evident. His renal biopsy revealed 5 of 22 glomeruli with global sclerosis. The residual glomeruli showed massive foam cell infiltration in the glomerular capillaries and severely widened mesangium (fig. ). These foam cells stained positive for CD68 (not shown). Transmission electron microscope images also revealed foam cell infiltration mainly located in the capillaries containing scattered lipid droplets and lamellar bodies in their cytoplasm (fig. ). The glomerular tufts were enlarged with hyalinosis, mesangiolysis and microaneurysmal formation, which were consistent with diabetic glomerulosclerosis. The interstitium and tubules showed moderate fibrosis and atrophy (50% of the cortical region). The foamy change was seen in some tubular epithelial cells, but no interstitial foamy cells were found. The interlobular arteries showed a moderate degree of fibrous intimal thickening, and the arterioles also showed a moderate degree of subendothelial hyalinosis. Immunofluorescence studies revealed nonspecific linear IgG deposits along the GBM; however, no significant deposit was found by electron microscopy. The patient's lipid profile showed type III HLP (table ). He had an apoE2 homozygote phenotype and the Arg158Cys genotype with no other mutations. Three years after the diagnosis, he progressed to renal failure and was started on hemodialysis.
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.