Nephrotic and nephritic syndromes

Question 1

Minimal change disease

Answer 1

Main cause of Nephrotic syndrome (i.e oedema, proteinuria and hypoalbuminemia) T-cell and cytokine mediated damage to the glomerular basement membrane. Treatment is Steroid therapy, cyclophosphamide if severe or refractory

Question 2

Focal segmental glomerulosclerosis

Answer 2

Causes nephrotic syndrome. Causes are: Idiopathic
secondary to other renal pathology, reflux nephropathy, HIV, heroin, Alport’s syndrome, sickle-cell. There is a notably high recurrence rate in kidney transplants.

Question 3

IgA nephropathy

Answer 3

Also called Bergers disease causes Nephritic syndrome. Deposition of IgA complexes. Typically young men arrive at ED 1-2 days after an URTI or other infective illness with frank hematuria. Important to distinguish from post-streptococcal glomerulonephritis which presents weeks after triggering illness and has more stark proteinuria. Biopsy will show proliferative changes w/ IgA deposits

Question 4

Post-streptococcal glomerulonephritis

Answer 4

Can cause nephrotic or nephritic syndrome. Presents 7-14 days after infection w/ streptococcal organism. Biopsy shows diffuse proliferative glomerulonephritis
endothelial proliferation with neutrophils. Immunofluorescence shows granular or ‘starry sky’ appearance. Good prognosis

Question 5

Mesangiocapillary glomerulonephritis/Membranoproliferative glomerulonephritis

Answer 5

Can present as nephrotic or nephritic syndrome. Poor prognosis, treated with steroids. Three types, type two is also referred to as dense deposit disease.

Question 6

Rapidly progressive glomerulonephritis

Answer 6

Causes Nephritic syndrome. Term used to describe a rapid loss of renal function associated with the formation of epithelial crescents in the majority of glomeruli. Causes include Goodpasture’s, Wegeners (granulomatosis w/ polyangiitis) and SLE.

Question 7

Alport syndrome

Answer 7

Causes nephritic syndrome. Usually inherited in an X-linked dominant pattern. Due to a defect in the gene which codes for type IV collagen resulting in an abnormal glomerular-basement membrane (GBM). Also causes bilateral sensorineural deafness
lenticonus (protrusion of the lens surface into the anterior chamber) retinitis pigmentosa Biopsy shows splitting of lamina densa seen on electron microscopy

Question 8

Amyloidosis

Answer 8

Causes nephrotic syndrome. Can be specific to kidneys or effect multiple other organ systems. Biopsy will show apple green birefringence on congo red staining.

Question 9

Diabetic nephropathy

Answer 9

Causes nephrotic syndrome