Congenital Nephrotic Syndrome of the Finnish Type
Congenital Nephrotic Syndrome of the Finnish Type Helen Liapis, MD Joseph Gaut, MD, PhD Key Facts Terminology Finnish nephropathy (FN) Occurs in neonates up to 3 months of age and…
Parathyroid Gland: Diagnosis and Margins
Parathyroid Gland: Diagnosis and Margins Parathyroid hyperplasia usually involves all 4 glands but can be asymmetric with marked variation in the extent of glandular involvement (pseudoadenomatous variant). Adenomas are usually…
Classification of Tubulointerstitial Diseases
Classification of Tubulointerstitial Diseases Robert B. Colvin, MD Acute interstitial nephritis was originally described in 1898 by William Councilman as a systemic response to certain extrarenal infections (here an untreated…
Gaucher Glomerulopathy
Gaucher Glomerulopathy A. Brad Farris, III, MD Key Facts Terminology Gaucher cells accumulate in renal parenchyma Etiology/Pathogenesis Glucosylceramide accumulation 2° to ↓ glucosylceramidase activity Clinical Issues Proteinuria, hematuria, nephrotic syndrome…
IgA Acute Glomerulonephritis Associated with S. Aureus
IgA Acute Glomerulonephritis Associated with S. Aureus Robert B. Colvin, MD Key Facts Etiology/Pathogenesis Staphylococcus aureus, coagulase positive, often MRSA Increased risk with diabetes mellitus, neoplasia, old age Clinical Issues…
Lipoprotein Glomerulopathy
Lipoprotein Glomerulopathy Helen Liapis, MD Joseph Gaut, MD, PhD Key Facts Etiology/Pathogenesis APOE gene mutations Clinical Issues Majority of cases reported in patients of Asian ancestry Microscopic Pathology Glomerular intracapillary…
Fibronectin Glomerulopathy
Fibronectin Glomerulopathy Neeraja Kambham, MD Key Facts Terminology Nonimmune lobular glomerulopathy with massive mesangial and subendothelial fibronectin deposits Etiology/Pathogenesis Autosomal dominant inheritance Mutations in fibronectin FN1 gene documented recently in…
Nail-Patella Synorome
Nail-Patella Synorome Helen Liapis, MD Joseph Gaut, MD, PhD Key Facts Etiology/Pathogenesis LMX1B mutations Clinical Issues Iliac horns are pathognomonic (70% of cases) Absent, hypoplastic, or dystrophic nails Small, irregularly…
Focal Segmental Glomerulosclerosis Classification
Focal Segmental Glomerulosclerosis Classification A. Brad Farris, III, MD Light microscopy of a case of collapsing FSGS shows a glomerulus with areas of sclerosis (silver stain). Immunofluorescence for C3 shows…
Podocin Deficiency
Podocin Deficiency Helen Liapis, MD Joseph Gaut, MD, PhD Key Facts Clinical Issues Proteinuria, may be nephrotic range 90% of familial cases progress to ESRD within ˜ 6 years 35%…
