Nephritic and Nephrotic syndrome, and Glomerulonephritis Flashcards
What is the definition of nephrotic syndrome?
Quadrad of:
- Proteinuria > 3g/24hrs
- Hypoalbuminaemia - < 30g/L
- Oedema
- Hypercholesterolaemia
What are primary renal diseases which can cause nephrotic syndrome?
- Minimal change disease
- Membranous nephropathy
- Focal segmental glomerulosclerosis
What is the most common cause of nephrotic syndrome in children?
Minimal change disease
What are secondary causes of nephrotic syndrome?
- Diabetes mellitus
- Lupus nephritis
- Hep B + C
- HIV
- Myeloma
- Amyloidosis
- Pre-eclampsia
- Malignancy
What is the pathophysiology of nephrotic syndrome?
Proteinuria results from podocyte and glomerular basement membrane pathology - normally have negative charge which prevents passage of protein into the urine.
There is an increase in the size and number of pores, allowing passage of more and larger molecules. Electrical charge is also involved in glomerular permeability.
Why do those with nephrotic syndrome develop hyperlipidaemia?
Hyperlipidaemia is the consequence of increased synthesis of lipoproteins (such as apolipoprotein B, C-III lipoprotein (a)), as a direct consequence of a low plasma albumin. There is also a reduced clearance of the principal triglycerides bearing lipoprotein (chylomicrons and VLDL) in direct response to albuminuria.
What is minimal change disease?
An altered cell-mediated immunologic response with abnormal secretion of lymphokines by T cells is thought to reduce the production of anions in the glomerular basement membrane, thereby increasing the glomerular permeability to serum albumin through a reduction of electrostatic repulsion. The loss of anionic charges is also thought to favor foot process fusion
What are symptoms that someone with nephrotic syndrome tend to present with?
- Generalised pitting oedema
- Frothy urine
- Systemic symptoms if secondary cause - cough, weight loss, night sweats etc.
What are clinical signs seen in nephrotic syndrome?
- Mild/severe pitting oedema - whole body
- Muehrcke’s lines
- Xanthelasmata
- Possible hypertension
- Normal JVP
- AScites
What are the different ways in which glomerulonephritis present?
- Isolated haematuria and/or proteinuria
- Nephrotic syndrome
- Nephritic syndrome
- Acute renal failure
- Chronic renal failure
What might the following child have?
Nephrotic syndrome
What would your differential diagnosis be for someone with features of nephrotic syndrome?
- CCF
- Liver disease
What investigations would you consider doing in someone with features of nephrotic syndrome?
- Bedside - urine dipstick, 24 hour urine collection
- Bloods - FBC, U+E’s, LFT, BNP, Serum albumin, ANCA, anti-GBM
- Imaging - Renal imaging, CXR, Dopplers - VTE
- Other - Throat swab, US guided Renal biospy
What is minimal change disease?
In this condition the glomeruli appear normal on light microscopy. The only abnormality seen on electron microscopy is fusion of the foot processes of epithelial cells (podocytes).
Thought to be due to immature differentiating CD34 stem cells rather than mature T lymphocytes
What proportion of nephrotic syndrome in children is caused by minimal change disease?
80%
What might you see on microscopy in someone with minimal change disease?
Fused podocytes
How would you manage someone with minimal change disease?
- Reduce oedema - Fluid restriction (1L/day), salt restriction
- High dose Prednisolone - 4-6 weeks
How would you generally manage someone with nephrotic syndrome?
- Treat underlying cause
- Reduce oedema - Fluid restriction (1L/day), salt restruction
- Loop diuretics (+/- thiazide if resistant oedema)
- Consider ACEi/ARB - reduce proteinuria
What are the main non-proliferative glomerulonephritidies?
- Minimal change disease
- FSGS
- MGN
What are the main proliferative glomerulonephritidies?
- IgA nephropathy
- Membranoprolifertive glomerulonephritis
- Post infectious glomerulonphritis
- Rapidly progressive glomerulonephritis
What type of GN is minimal change disease; proliferative or non-proliferative?
Non-proliferative
What type of GN is FSGS; proliferative or non-proliferative?
Non-proliferative
What type of GN is membranous glomerulonephritis?
Non-proliferative
What type of GN is IgA nephropathy; proliferative or non-proliferative?
Proliferative
What type of GN is Membranoproliferative GN; proliferative or non-proliferative?
Proliferative
What type of GN is Rapidly progressive GN; proliferative or non-proliferative?
Proliferative
What type of GN is Post infectious GN; proliferative or non-proliferative?
Proliferative
What are examples of rapidly progressive (crescentic) GNs?
- Goodpastures syndrome
- Lupus
-
Vasculitic disorders
- Wegeners
- Microscopic polyangitis
What syndrome does minimal change disease present with; nephritic or nephrotic syndrome?
Nephrotic syndrome
What syndrome does Focal segmental glomerulosclerosis present with; nephritic or nephrotic syndrome?
Nephrotic syndrome
What syndrome does membranoproliferative GN tend present with; nephritic or nephrotic syndrome?
Nephritic syndrome
What syndrome does Membranous GN tend present with; nephritic or nephrotic syndrome?
Nephrotic syndrome
What is Focal Segmental Glomerulosclerosis?
Segmental glomerulosclerosis is seen, which later progresses to global sclerosis. The deep glomeruli at the corticomedullary junction are affected first.
How would you manage someone with FSGS?
- ACEi/ARBs and BP control
- Corticosteroids - often ineffective
- Consider calcineurin inhibitors
How would you manage someone with membranous nephropathy?
- ACEI/ARB and BP control in all
- Consider immunosuppression