Renal Flashcards
1
Q
What are the kidneys classified as in terms of their anatomical position?
A
Retroperitoneal organs
2
Q
How long are the kidneys in a normal person?
A
Approximately 10-12 cm
3
Q
Between which vertebrae do the kidneys sit?
A
T12 to L2
4
Q
Why is the right kidney positioned slightly lower than the left kidney?
A
Due to liver displacement
5
Q
What type of capsule surrounds each kidney?
A
Fatty capsule
6
Q
What is the blood supply source for each kidney?
A
Renal artery from the abdominal aorta
7
Q
How does venous drainage occur from the kidneys?
A
Through renal veins to the inferior vena cava
8
Q
What structure do ureters drain urine from?
A
Each renal pelvis
9
Q
What are the two main layers of the kidney?
A
Outer cortex and inner medulla
10
Q
What are the functional units of the kidney called?
A
Nephrons
11
Q
What components are included in each nephron?
A
- Renal corpuscle (glomerulus and Bowman’s capsule)
- Proximal convoluted tubule
- Loop of Henle (descending and ascending limbs)
- Distal convoluted tubule
- Collecting duct
12
Q
What drives solute and water into the Bowman’s space?
A
Vascular hydrostatic pressure across the capillaries
13
Q
What type of cells are found in the visceral inner layer of the Bowman’s capsule?
A
Podocytes and simple squamous epithelium
14
Q
What do macula densa cells detect?
A
Sodium concentrations within the distal convoluted tubule lumen
15
Q
What happens when there are lower sodium concentrations in the distal tubule?
A
It triggers vasodilation of the afferent renal arterioles and activation of the renin-angiotensin-aldosterone system (RAAS)
16
Q
What is the overall action of the RAAS?
A
To increase blood pressure to maintain end-organ perfusion
17
Q
What does renin cleave to form angiotensin I?
A
Angiotensinogen
18
Q
What is angiotensin I converted to and by which enzyme?
A
Angiotensin II by angiotensin converting enzyme (ACE)
19
Q
What are the three roles of angiotensin II?
A
- Causes vasoconstriction to increase blood pressure
- Stimulates aldosterone release from the adrenal cortex
- Promotes vasopressin release from the posterior pituitary gland
20
Q
What does aldosterone stimulate in the distal convoluted tubule?
A
Sodium uptake on the apical cell membrane
21
Q
What is the primary role of the loop of Henle?
A
Concentration of filtrate by removing sodium and chloride
22
Q
What is the final site of water reabsorption in the nephron?
A
Collecting duct
23
Q
What is the effect of ADH on the collecting duct?
A
Stimulates insertion of aquaporin channels for water reabsorption
24
Q
What is the normal urinary protein excretion level considered healthy?
A
< 150 mg per day
25
What does elevated urine protein levels indicate?
Albuminuria, hemoglobinuria, myoglobinuria, or excess immunoglobulins
26
What is the purpose of the Cockcroft-Gault equation?
To estimate creatinine clearance and assess renal impairment
27
What is the alternative marker of eGFR that is independent of age, sex, or muscle mass?
Cystatin C
28
What does the MDRD equation estimate?
Renal function taking into account age, gender, ethnicity, and serum creatinine concentration
29
What is the gold standard for precisely assessing renal function?
Radionucleotide testing
30
What is the normal pH range for serum?
7.35 - 7.45
31
What enzyme works to regulate hydrogen ion concentrations within serum?
Carbonic anhydrase
32
What does pseudohyponatraemia refer to?
A laboratory assay artifact secondary to hyperglycaemia, hyperglobulinaemia, or hyperlipidaemia
33
What is the primary mechanism for maintaining electrolyte balance in the kidneys?
Active transport mechanisms in various parts of the nephron
34
Fill in the blank: The collecting duct is impermeable to water unless _______ stimulates the insertion of aquaporin channels.
ADH
35
True or False: The proximal convoluted tubule is the site of maximal reabsorption of glucose, proteins, electrolytes, water, and bicarbonate.
True
36
What is the cause of hyponatraemia in euvolaemic states?
Syndrome of Inappropriate ADH (SIADH)
## Footnote
SIADH causes hyponatraemia due to excessive vasopressin secretion leading to fluid retention.
37
How can osmolality be determined?
Osmolality can be calculated using an equation or measured directly through laboratory testing
## Footnote
Direct measurement is more accurate.
38
What is the osmolal gap?
The difference between measured and calculated osmolality
## Footnote
It accounts for other solutes not included in the equation.
39
What is pseudohyponatraemia?
A laboratory assay artefact secondary to:
* Hyperglycaemia
* Hyperglobulinaemia
* Hyperlipidaemia
## Footnote
These conditions can falsely lower sodium levels in tests.
40
What are the hydration status categories for true hyponatraemia?
The hydration status categories are:
* Hypervolaemic
* Euvolaemic
* Hypovolaemic
## Footnote
Each category has different underlying causes.
41
What is the expected urine osmolality in euvolaemic hypotonic hyponatraemia?
Expected low urine osmolality is 100 mosm/L
## Footnote
Conditions such as Beer potomania and primary polydipsia can cause this.
42
What indicates inappropriately high urine osmolality in hyponatraemia?
Urine osmolality >100 mosm/L, followed by measuring urinary sodium
## Footnote
High urinary sodium typically reflects excess vasopressin secretion due to SIADH.
43
What are the causes of Syndrome of Inappropriate ADH (SIADH)?
Causes of SIADH include:
* CNS - infection, haemorrhage, malignancy
* Pulmonary - pneumonia
* Malignancy - lung cancer
* Drugs - SSRIs, antipsychotics, carbamazepine, desmopressin
* Infection - HIV, tuberculosis
* Endocrine - adrenal insufficiency
## Footnote
SIADH leads to fluid reabsorption independent of sodium reabsorption.
44
What is indicated for symptomatic hyponatraemia?
Intravenous hypertonic (3%) saline
## Footnote
Treatment aims to increase sodium concentration slowly to avoid complications.
45
What are the risks associated with rapid reversal of hyponatraemia?
Osmotic central pontine myelinolysis
## Footnote
This condition can lead to irreversible neurological disability.
46
What is the primary treatment for nephrogenic diabetes insipidus?
Removal of the cause
## Footnote
Desmopressin is ineffective in nephrogenic DI.
47
What are typical causes of hypokalaemia?
Typical causes include:
* Cellular redistribution
* GI losses
* Decreased intake
* Drugs such as diuretics, insulin, b-agonists
## Footnote
These can lead to serious cardiac and skeletal muscle dysfunction.
48
What is the presentation of hypokalaemic periodic paralysis?
Intermittent muscle weakness provoked by carbohydrate meal
## Footnote
This condition is usually autosomal dominant and common in Asian or Mexican populations.
49
What are common causes of hyperkalaemia?
Typical causes include:
* Acute or chronic renal failure
* Deficient aldosterone activity
* Medications such as RAAS blockers
* Dietary intake of high potassium foods
## Footnote
Hyperkalaemia can lead to severe cardiac arrhythmias.
50
What is the first step in managing severe hyperkalaemia?
Administration of intravenous calcium
## Footnote
This stabilizes the cardiac membrane.
51
What does metabolic acidosis result from?
Bicarbonate loss, reduced acid excretion, excessive acid production or ingestion
## Footnote
The anion gap helps predict the causes of metabolic acidosis.
52
What are the types of metabolic acidosis based on anion gap?
Increased anion gap and normal anion gap
## Footnote
Increased anion gap indicates excessive acid production; normal anion gap indicates bicarbonate loss.
53
What characterizes acute kidney injury (AKI)?
Acute increase in serum creatinine of 50% from baseline
## Footnote
Oliguria is also a defining feature according to KDIGO Guidelines.
54
What are the causes of AKI categorized into?
Pre-renal, intrinsic-renal, and post-renal causes
## Footnote
Each category has distinct mechanisms leading to kidney injury.
55
What is a common cause of intrinsic AKI in a hospital setting?
Acute tubular necrosis (ATN)
## Footnote
ATN is often secondary to ischaemia or direct drug toxicity.
56
What is the most common cause of normal anion gap metabolic acidosis?
Gastrointestinal losses of bicarbonate
## Footnote
Conditions such as vomiting and diarrhea are common.
57
What is the management for central diabetes insipidus?
Desmopressin
## Footnote
This vasopressin analogue helps manage the deficiency.
58
What does the Fluid Deprivation Test assess?
It assesses for diabetes insipidus and the site of pathology
## Footnote
Patients cannot drink water during the test.
59
What should be excluded first in cases of unclear aetiology of AKI?
Post-renal causes
## Footnote
This includes investigations such as urine microscopy, culture and sensitivity, urinary cast assessment, and imaging of the kidney, ureters, and bladder.
60
What does proteinuria suggest in urinary assessment?
A glomerular cause
## Footnote
Presence of protein in urine indicates potential damage to the glomeruli.
61
Normal red blood cells in urinary assessment suggest what condition?
Urothelial bleeding from post-renal tissues
## Footnote
Indicates that bleeding is likely occurring in the lower urinary tract.
62
Dysmorphic red blood cells in urine indicate which condition?
Glomerulonephritis
## Footnote
These cells are typically a sign of glomerular damage.
63
White blood cell casts in urine suggest what?
Interstitial nephritis
## Footnote
Indicates inflammation of the kidney interstitium.
64
Muddy brown casts in urine suggest which condition?
Acute tubular necrosis
## Footnote
This condition is characterized by damage to the renal tubules.
65
What further investigation is required for prolonged or severe unexplained AKI with proteinuria and haematuria?
Exclusion of vasculitis by performing a glomerulonephritis screen
## Footnote
This may include testing for ANCAs, Anti-GBM antibodies, and ANA.
66
What is often performed to establish the diagnosis in cases of AKI?
A renal biopsy
## Footnote
This procedure helps to determine the underlying cause of kidney injury.
67
What is critical to ongoing management of AKI?
Early investigation
## Footnote
Identifying the cause early can significantly affect treatment outcomes.
68
What is often the only treatment required for AKI?
Correction of the underlying cause
## Footnote
Addressing the root issue may resolve the kidney injury.
69
What is essential for pre-renal and some intrinsic causes of AKI?
Intravenous rehydration
## Footnote
This helps restore fluid balance and improve kidney function.
70
What types of drugs should be avoided in cases of AKI?
Drugs toxic to renal tissues
* NSAIDs
* ACE-Inhibitors
* Contrast media
* Gentamicin
## Footnote
These medications can exacerbate kidney injury.
71
What is key to managing electrolyte disturbances in AKI?
Maintaining euvolaemia and monitoring
## Footnote
This includes correcting issues such as hyperkalaemia.
72
Correction of metabolic acidosis with sodium bicarbonate in AKI may improve what?
Risk of requiring renal replacement therapy, multi-organ failure, and mortality
## Footnote
This is particularly relevant in ICU settings.
73
True or False: There is robust evidence to support the use of dopamine in AKI management.
False
## Footnote
Current guidelines do not recommend dopamine for this purpose.
74
In cases of severe hypotension, which medications can provide ionotropic support?
Noradrenaline and/or dobutamine
## Footnote
These agents help stabilize blood pressure.
75
What are indications for urgent haemodialysis?
* Hyperkalaemia refractory to medical management
* Uraemia with complications
* Severe fluid overload refractory to medical management
## Footnote
These conditions necessitate immediate intervention.
76
What are the two major forms of dialysis performed in the acute setting?
* Fluid removal (Ultrafiltration)
* Solute exchange (Haemodialysis)
## Footnote
Each method addresses different aspects of kidney failure.
77
What is a strong predictor of all-cause mortality in severe acute kidney injury?
Requirement of haemodialysis
## Footnote
This significantly increases the risk of mortality.
78
What is the risk of 30-day mortality for AKI in a hospital setting?
10-20%
## Footnote
This risk is independent of other factors and increases with renal replacement therapy.
79
What metabolic and electrolyte derangements can occur in severe AKI?
* Hyperkalaemia
* Hyperphosphataemia
* Metabolic acidosis
## Footnote
These disturbances may have serious health implications.
80
What can severe fluid overload in AKI lead to?
Life-threatening acute pulmonary oedema
## Footnote
This condition requires immediate medical attention.
81
What has been the trend in the incidence of end stage kidney disease in Australia since 2005?
A plateau around 2,500 new cases
## Footnote
This indicates a stabilization in new cases after an increase from 1960 to 2005.
82
What percentage of the general population reaches end stage renal failure requiring renal replacement therapy?
0.5%
## Footnote
This reflects the severity and rarity of progression to ESRF.
83
Which ethnic groups are at higher risk for end stage renal failure?
* Indigenous Australians
* African Americans
* Hispanics
* Asians
## Footnote
This is due to differences in risk factor profiles and genetics.
84
What is the formula for calculating creatinine clearance?
Cockcroft-Gault: [(140-Age) x weight (kg) x (1.04 if female, 1.23 if male)] / serum creatinine (micromol/L)
## Footnote
This formula helps estimate kidney function.
85
What is the first line screening blood test to assess for renal impairment?
Estimated Glomerular Filtration Rate
## Footnote
This test is less specific than creatinine clearance but widely used.
86
What is the risk factor for progression to end stage renal failure?
Decreased GFR
## Footnote
The risk is proportional to the stage of chronic kidney disease.
87
What is the most common cause of end stage renal failure in Australia?
Diabetes
## Footnote
This condition accounts for a significant proportion of new cases requiring renal replacement therapy.
88
What is the preferred staging for albuminuria?
A1 - A3
## Footnote
This replaces the previous micro- and macroalbuminuria classifications.
89
What should be initiated for all patients with albuminuria?
ACE-Inhibitor therapy or angiotensin receptor blockers (ARBs)
## Footnote
These medications help reduce the risk of progression to ESRF.
90
What is the recommended daily sodium intake for patients at risk of ESRF?
<2 grams per day
## Footnote
This restriction is advised to manage blood pressure and kidney health.
91
What is the current recommendation for protein consumption in patients with proteinuria?
<= 0.8 grams/kg/day
## Footnote
This guideline aims to manage kidney workload and slow progression to ESRF.
92
What is the blood pressure target for patients with risk factors for ESRF?
<130/80 mmHg
## Footnote
This target helps manage conditions that could lead to kidney failure.
93
What is the importance of intensive glucose control in diabetic patients with proteinuria?
It decreases rates of microvascular complications including nephropathy
## Footnote
However, it is not as effective as blood pressure management in preventing progression to ESKD.
94
What is the preferred modality of renal replacement therapy for most patients with ESKD?
Kidney transplantation
## Footnote
This option offers the best quality of life and is more cost-effective than dialysis.
95
What is the principle behind peritoneal dialysis?
Exchange of molecules across the peritoneal semi-permeable membrane
## Footnote
This method involves inserting dialysate into the peritoneum.
96
What are the two major forms of peritoneal dialysis?
* Continuous Ambulatory Peritoneal Dialysis
* Automated Peritoneal Dialysis
## Footnote
Each method has different operational characteristics and patient preferences.
97
What is a serious complication of peritoneal dialysis?
Peritonitis
## Footnote
This life-threatening condition requires prompt treatment and can lead to membrane failure.
98
What are common organisms cultured in cases of peritonitis?
* Staphylococcus epidermidis
* Escherichia coli
* Enterococcus
* Polymicrobial organisms
## Footnote
These infections can complicate treatment and require specific antibiotic coverage.
99
What are the indications for haemodialysis?
* Most effective method of dialysis
* Contraindication or failure of PD
* Need for renal replacement therapy
## Footnote
Haemodialysis is preferred in many clinical scenarios.
100
What are the adverse events associated with haemodialysis?
* AV fistula failure
* Infection
* Anticoagulation risks
* Increased cardiovascular disease risk
## Footnote
These complications can impact patient outcomes significantly.
101
What is calcium and phosphate homeostasis managed by prior to ESRF?
Parathyroid hormone (PTH)
## Footnote
PTH regulates calcium and phosphate levels in normal kidney function.
102
What is required for anticoagulation in endocarditis?
Anticoagulation with heparin
## Footnote
This places the patient at risk of bleeding and heparin-induced thrombocytopenia (HITS)
103
What risks are increased due to exposure of blood to machinery?
Blood-borne virus acquisition such as Hepatitis B, Hepatitis C, HIV, and Creutzfeldt-Jakob Disease
104
What is the leading cause of mortality in long-term hemodialysis (HD)?
Cardiovascular disease
105
What are the mechanisms by which parathyroid hormone (PTH) works at the bone?
Binds osteoblasts, increases RANK-Ligand secretion, inhibits osteoprotegrin release
106
What is the effect of PTH at the kidney?
Increases reabsorption of calcium but decreases phosphate reabsorption
107
What causes hyperphosphataemia in end-stage renal failure (ESRF)?
Reduced renal phosphate excretion and increased FGF-23 levels
108
What is the consequence of decreased calcitriol in ESRF?
Relative hypocalcaemia and increased parathyroid hormone (PTH) levels
109
What is secondary hyperparathyroidism?
Elevated PTH levels in ESRF due to impaired calcium and phosphate homeostasis
110
What is the first line treatment to reduce PTH and phosphate levels?
Calcitriol supplementation
111
What are phosphate binders used for?
To bind phosphate in the gastrointestinal tract and prevent absorption
112
What are calcimimetics and their function?
Drugs that bind the calcium sensing receptor at the parathyroid gland to reduce PTH secretion
113
What is the primary function of calcitriol at the bone?
Increases calcium and phosphate liberation
114
What is the consequence of chronic kidney disease mineral and bone disorder (CKD-MBD)?
Demineralisation of bone and increased risk of fractures
115
What is the relationship between hyperlipidaemia and cardiovascular risk in ESRF?
Lipid-lowering therapies lower cardiovascular risk in early CKD but less clear in established dialysis
116
What causes hypertension in ESRF?
Impaired salt and water balance, renal artery stenosis
117
What is the target blood pressure for patients with end-stage kidney disease (ESKD)?
130/80 mmHg
118
What is the most common cause of death in patients with ESKD?
Cardiovascular disease
119
What leads to anaemia in ESRF?
Decreased erythropoietin synthesis and EPO resistance due to chronic inflammation
120
What are the target hemoglobin levels for replacement in ESRF?
100-110 g/L
121
What is a common cause of iron deficiency in ESRF?
Chronic inflammation and reduced dietary iron absorption
122
What is the leading cause of death in ESRF?
Cardiovascular disease
123
What are the indications for kidney transplant in Australia?
* Type 2 Diabetes Mellitus
* Glomerulonephritis (IgA Nephropathy > FSGS)
* Hypertension
124
What are the eligibility criteria for living kidney donors?
* Adults >18 years of age
* Informed consent
* Compatible blood type and HLA-matching
* Normal kidney function
* No personal or family history of renal disorders
* Minimal comorbidities
* Low surgical risk
125
What are the contraindications for kidney donation?
* Active malignancy
* Uncontrolled infection
* Irreversible renal dysfunction
126
What is the significance of HLA-matching in kidney transplants?
Better matching is associated with improved long-term graft survival
127
What are the three major cellular signaling pathways involved in acute rejection?
* Antigen presentation
* Costimulation
* IL-2 positive self feedback
128
What histological features indicate acute cellular rejection?
Mononuclear infiltrates causing interstitial inflammation and tubular involvement
129
What is the treatment for acute cellular rejection?
Increased immunosuppression with glucocorticoids
130
What characterizes acute antibody-mediated rejection?
Antibody-mediated inflammation and cellular destruction in response to donor-specific antibodies
131
What is chronic allograft nephropathy?
A condition involving slow and progressive loss of kidney function following transplantation
132
What is antibody-mediated rejection?
A response that occurs in response to donor-specific antibodies following antigen presentation
## Footnote
Typically against donor HLA antigens, but can also be against non-HLA antigens such as angiotensin II type 1 receptor antigen.
133
What are the major causes of chronic allograft nephropathy?
Two major causes are:
* Antibody-mediated rejection
* Immunosuppressant drug effect
134
What are the histological findings in antibody-mediated rejection?
Histology shows:
* Glomerulopathy
* Vascular narrowing with intimal proliferation
* Chronic interstitial nephritis with fibrosis
* Lymphocytic infiltrate
135
What is the effect of calcineurin inhibitors on the kidneys?
They cause chronic allograft nephropathy due to their nephrotoxic effects
## Footnote
Examples include cyclosporine and tacrolimus.
136
What is the surgical approach for kidney transplantation?
The donor kidney is often engrafted inferior to the native kidneys in the iliac fossa
## Footnote
The renal artery is engrafted to the external iliac artery or directly to the aorta, and the renal vein is engrafted to the external vena cava.
137
What is the purpose of immunosuppression after a transplant?
To terminate Signals 1, 2, and 3 to prevent acute antibody-mediated rejection.
138
What is Basiliximab?
An anti-CD25 monoclonal antibody used for T-cell inactivation in induction therapy
## Footnote
It has minimal adverse effects and is used for >90% of induction therapy.
139
What are the adverse effects of calcineurin inhibitors?
They include:
* Nephrotoxicity
* Significant drug interactions via Cytochrome P450 pathway
* Concentration-dependent effects
140
What is the role of mycophenolate in immunosuppression?
Mycophenolate inhibits IMPDH enzyme to inhibit purine synthesis and cause G1 cellular arrest.
141
What are the diagnostic features of nephritic syndrome?
Diagnostic features include:
* Haematuria
* Proteinuria (less than 3.5g per day)
* Oedema
* Hypertension
* Decreased renal function
142
What characterizes nephrotic syndrome?
Characterized by:
* Proteinuria (greater than 3.5g per day)
* Hypoalbuminaemia
* Hyperlipidaemia
* Oedema
* Hypertension
* Hypercoagulability
143
What is Minimal Change Disease?
A non-proliferative cause of nephrotic syndrome, predominantly in children, with histology showing minimal changes from normal.
144
What are the first-line treatments for Focal Segmental Glomerulosclerosis?
First-line treatment is corticosteroids over a long period (6 months minimum)
## Footnote
Remission is achieved in half of the patients.
145
What is the most common form of GN causing nephrotic syndrome?
Membranous Nephropathy, typically affecting Caucasians between 30-55 years of age.
146
What are the common pathogens associated with infections post-transplant?
Common pathogens include:
* Bacterial infections in the respiratory tract, genitourinary tract, and skin
* Cytomegalovirus (CMV)
* BK Virus
* Herpes Simplex Virus (HSV)
* Varicella Zoster (VZV)
* Pneumocystic Jiroveci Pneumonia (PJP)
* Fungal/Yeast infections
147
What is the most common cause of death with a functioning transplant?
Cardiovascular disease.
148
What condition is characterized by new onset diabetes after transplant?
New Onset Diabetes After Transplant (NODAT) is characterized by predisposition from immunosuppressant drugs.
149
What are the causes of graft loss after transplantation?
Common causes include:
* Death with a functioning graft
* Chronic allograft nephropathy
* Acute rejection
* Recurrence of glomerulonephritis
150
What are the complications associated with nephrotic syndrome?
Increased risk of infections due to loss of immunoglobulins and fluid accumulation.
151
What is the most common form of glomerulonephritis causing nephrotic syndrome?
Membranous Nephropathy
## Footnote
Typically affects Caucasians between 30-55 years of age.
152
What percentage of cases does Primary Membranous Nephropathy account for?
>85% of cases
## Footnote
It is the primary form of Membranous Nephropathy.
153
What antibodies can be detected in 90-96% of Membranous Nephropathy cases?
Anti-phospholipase A2 receptor antibodies (Anti-PLA2R)
## Footnote
Anti-P2R antibodies can be tracked as a measure of disease activity.
154
What are the secondary causes of Membranous Nephropathy?
* Infections (syphilis, malaria, hepatitis viruses)
* Drugs (penicillamine, NSAIDs, gold)
## Footnote
These account for the remainder of cases.
155
What histological features are associated with Membranous Nephropathy?
* Immune complex deposition
* Complement activity (particularly C5-9)
* Spikes within the glomerular basement membrane
## Footnote
Immunofluorescence can identify immune complex deposits.
156
How is the natural history of Membranous Nephropathy divided?
* 1/3 enter remission
* 1/3 require ongoing stable treatment
* 1/3 progress to end-stage renal failure within ten years.
157
What initial treatment is recommended for all patients with Membranous Nephropathy?
* ACE inhibitors or ARB therapy
* Lipid-lowering therapy
* Lifestyle measures.
158
What is the effective treatment for patients with Membranous Nephropathy who have high proteinuria?
Cyclophosphamide
## Footnote
It provides a remission rate in excess of 80% at ten years.
159
What is the most common predisposing condition for Proliferative Glomerulonephritis?
Hepatitis C with cryoglobulinaemia.
160
What histological features are associated with Proliferative Glomerulonephritis?
Wire loops of membranes with mesangial deposition of immune complexes and complement.
161
What is the typical presentation for IgA Nephropathy?
Haematuria following sinusitis or pharyngitis.
162
What histological finding is characteristic of IgA Nephropathy?
Mesangial hypercellularity and IgA immune complex deposits.
163
What is the key treatment for IgA Nephropathy?
ACE inhibitors are indicated in all patients.
164
What condition is most commonly associated with Post-Streptococcal Glomerulonephritis?
Pharyngeal infection with streptococcus bacteria.
165
What is the pathophysiology of Post-Streptococcal Glomerulonephritis?
Immune complexes formed against streptococcal antigens deposited at the glomerulus.
166
What is a key feature of rapidly progressive glomerulonephritis?
Rapid deterioration in renal function to end-stage renal failure over weeks.
167
What are the common causes of rapidly progressive glomerulonephritis?
* Goodpasture's Syndrome (Anti-GBM Disease)
* Lupus
* Crescentic IgA nephropathy
* Vasculitis (ANCA positive).
168
What histological feature is classic for rapidly progressive glomerulonephritis?
Widespread crescent formation with a pauci-immune pattern.
169
What is the classification of Lupus Nephritis based on histological findings?
* Class I: Minimal disease
* Class II: Mesangial proliferation and deposits
* Class III: Focal proliferative GN
* Class IV: Diffuse proliferative GN
* Class V: Membranous GN.
170
What is the first-line treatment for Class III and IV lupus nephritis?
Induction with corticosteroids and mycophenolate or cyclophosphamide.
171
What is the most common genetic disorder contributing to end-stage renal failure?
Autosomal Dominant Polycystic Kidney Disease (ADPKD).
172
What genes are involved in Autosomal Dominant Polycystic Kidney Disease?
* PKD1 gene on chromosome 16
* PKD2 gene on chromosome 4.
173
What are the common clinical manifestations of ADPKD?
* Hypertension
* Decline in renal function
* Proteinuria
* Hepatic cysts
* Cerebral aneurysms.
174
What is the recommended management for patients with ADPKD?
* Strict cardiovascular risk factor control
* ACE inhibitors or ARBs
* High fluid intake (> 3 liters/day).
175
What is the genetic basis of Autosomal Recessive Polycystic Kidney Disease?
Genetic mutation in the PKHD1 gene on chromosome 6.
176
What is a common clinical presentation of Autosomal Recessive Polycystic Kidney Disease?
A young infant with systemic hypertension and abdominal pain.
177
What is the inheritance pattern of Tuberous Sclerosis?
Autosomal dominant inheritance.
178
What are the common renal manifestations of Tuberous Sclerosis?
* Angiomyolipomas
* Kidney cysts.
179
What is the most common pattern of inheritance in Alport Syndrome?
X-linked inheritance (85%).
180
What are the clinical manifestations of Alport Syndrome?
* Microscopic haematuria
* Proteinuria
* Progressive renal failure
* Hearing loss.
181
What histological finding is pathognomonic for Alport Syndrome?
Basement membrane thinning and thickening resulting in a 'basket weave' pattern.
182
What are common renal manifestations of inherited nephritis?
Microscopic haematuria and proteinuria
## Footnote
Progressive renal failure occurs over decades.
183
How does histology appear in inherited nephritis?
Similar to focal segmental glomerulosclerosis, with electron microscopy showing basement membrane thinning and thickening in a 'basket weave' pattern.
184
What is a common progressive complication associated with inherited nephritis?
Hearing loss
## Footnote
Hearing loss is progressive over time.
185
What visual disturbances are associated with inherited nephritis?
Cataracts, macular changes, and lenticonus often do not cause visual disturbance.
186
How can proteinuria be managed in inherited nephritis?
With ACE-Inhibitors.
187
What are the management options for end-stage renal failure (ESRF)?
Peritoneal dialysis, haemodialysis, kidney transplantation
## Footnote
The disease does not recur in the transplanted kidney.
188
What aids can assist with hearing loss in inherited nephritis?
Hearing aids.
189
What are rarely required for visual disturbances in inherited nephritis?
Contact lenses or corneal transplants.
190
What genetic mutations are associated with autosomal dominant polycystic kidney disease (ADPCKD)?
PKD1, PKD2.
191
Which genetic mutations are related to autosomal recessive polycystic kidney disease (ARPCKD)?
PXHD1, TSC1, TSC2.
192
What type of genetic inheritance is most commonly associated with Alport syndrome?
Mostly X-linked, rarely autosomal dominant or recessive.
193
What is the average age of onset for autosomal dominant polycystic kidney disease?
30-55 years.
194
At what age does autosomal recessive polycystic kidney disease typically manifest?
Infancy or childhood.
195
What are some extrarenal manifestations of genetic renal conditions?
Hepatic cysts, pancreatic cysts.
196
What cardiac condition is associated with certain genetic renal conditions?
Mitral valve prolapse.
197
What are common late-life complications in genetic renal conditions?
Hearing loss, vision loss.
198
What are the management options for patients requiring dialysis and transplant?
Dialysis, transplant, or both if required.
