Flashcards in Kidney Diseases with Proteinuria Deck (11):
1. Describe (derangement of) normal glomerular physiology.
nephrotic syndrome can present with loss of normal interdigitation of podocytes or altered capillary permeability
disturbing the interaction between podocytes and BM can alter slit diaphragm (permeability) as well as causing foot detachment
2. Describe the pathophysiology of diseases presenting with the nephrotic syndrome. Define nephrotic disease including signs.
nephrotic syndrome is caused by a derangement in glomerular capillary walls, resulting in increased permeability to plasma proteins with the following signs
nephrotic range proteinuria (>3.5 g/d)
hyperlipidemia (increased production by liver)
3,4 . Describe the morphology and corresponding clinical presentation of Minimal Change Disease
young patients presenting with
massive proteinuria (>3.5g/d), hypoalbuminemia, generalized edema, hyperlipidemia and lipiduria
only microscopic abnormality will be loss effaced podocyte foot processes on EM
unknown etiology, usually cured with prednisone
3,4 . Describe the morphology and corresponding clinical presentation of Focal Segmental Glomerular Sclerosis.
nephrotic syndrome (massive proteinuria (>3.5g/d), hypoalbuminemia, generalized edema, hyperlipidemia and lipiduria)
LM shows focal segmental glomerular
sclerosis (capillary loops appear collapsed, urinary space larger), EM shows foot process effacement
primary RSGS etiology is unknown, circulating factor may be a factor; poorly responsive to steroids (can progress to ESRF)
5. Describe clinical disease entities presenting with nephrotic syndrome.
minimal change nephrotic syndrome (children)
focal segmental glomerulosclerosis (idiopathic or secondary)
membranous nephropathy (idiopathic o secondary)
5. Describe clinical disease entities presenting with nephrotic syndrome- secondary causes of FSGS.
since podocyte injury is central the following can contribute to damage:
sickle cell disease
obesity (BMI to kidney perfusion low)
reduction in renal mass (unilateral renal agenesis)
Describe the proteins linking of podocytes in the slit diaphragm
nephrine molecules have cysteine loops that help to link the processes from two cell together
3,4 . Describe the morphology and corresponding clinical presentation of Congentital nephrotic syndrome (Finnish type).
nephrin mutations cause the development of renal failure in infancy due to podocytes inability to form and link
3,4 . Describe the morphology and corresponding clinical presentation of Membranous nephropathy.
patients of all ages see damage due to immune complexes, antigen is in/of the podocyte, complexes can build up sub epithelial or with/in BM
silver stain on EM shows vertical spikes and granular pattern on Immunoflorescence due to deposition of C3 and IgG along peripheral capillary walls
etiology: autoantibodies to M-type phospholiase A2 receptor; outcomes vary and treatment includes ACEI, prednisone and immunosuppressive agents
5. Describe clinical disease entities presenting with nephrotic syndrome- secondary causes of membranous glomerular nephropathy.
infectious organisms: hep B, malaria, syphilis
Drugs: catopril, NSAIDs, penicillamine, probenecid
Tumors (lymphomas and carcinomas