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MRCP I > NEPHROLOGY > Flashcards

NEPHROLOGY Flashcards

1
Q

Adrenal cortex (mnemonic GFR - ACD)

A
  • Zona Glomerulosa (on outside): mineralocorticoids, mainly Aldosterone
  • Zona Fasciculata (middle): glucocorticoids, mainly Cortisol
  • Zona Reticularis (on inside): androgens, mainly Dehydroepiandrosterone (DHEA)
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2
Q

Renin

A
  • Released by JGA cells in kidney in response to ↓ renal perfusion, low sodium
  • Hydrolyses angiotensinogen to form angiotensin I
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3
Q

Factors stimulating renin secretion

A
  • ↓ BP → ↓ renal prefusion
  • Hyponatremia
  • Sympathetic nerve stimulation
  • Catecholamines
  • Erect posture
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4
Q

Factors reducing renin secretion

A

β-blockers
NSAIDS

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5
Q

Angiotensin

A
  • ACE in lung converts angiotensin I → angiotensin II
  • Vasoconstriction leads to raised BP
  • Stimulates thirst
  • Stimulates aldosterone and ADH release
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6
Q

Aldosterone

A

Released by the zona glomerulosa in response to raised angiotensin II, potassium, and ACTH levels

Causes retention of Na+ in exchange for K+/H+ in distal tubule

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7
Q

Anemia in CRF:

A

Correction o iron with IV if needed
* Ferritin should be > 200 ng/mL before starting EPO
* EPO is used to target Hb 10-12 (>11 or hematocrit >33%) reach the target within 4 months
* Corrected Hb of > 13.5 is associate with HTN crisis
* Hb < 10.5 ↑ risk of seizures.

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8
Q

Erythropoietin:

A

(EPO) is a hematopoietic growth factor that stimulates the production of erythrocytes. The main uses of erythropoietin are to treat the anemia associated with chronic renal failure and that associated with cytotoxic therapy

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9
Q

Side-effects of erythropoietin

A

HTN and HTN crisis, potentially → encephalopathy and seizures (BP ↑ in 25% of patients)
* EPO induced seizures occurs after 90 days fro starting the treatment
* Bone aches
* Flu-like symptoms
* Skin rashes, urticaria
* Pure red cell aplasia* (due to antibodies against erythropoietin)
* Raised packed cell volume (PCV) =HCT → ↑ risk of thrombosis (e.g. Fistula)
* Iron deficiency 2nd to ↑ erythropoiesis

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10
Q

There are a number of reasons why patients may fail to respond to erythropoietin therapy

A

Iron deficiency
* Inadequate dose
* Concurrent infection/inflammation
* Hyperparathyroid bone disease
* Aluminum toxicity

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11
Q

Indication for Urgent Dialysis:

A
  • Severe acidosis
  • Pulmonary edema due to volume overload
  • Hyperkalemia
  • Uremic pericarditis
  • Severe uremic symptoms
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12
Q

Hyperacute graft rejection is due

A

pre-existent antibodies to HLA antigens and is therefore IgG
mediated

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13
Q

Graft survival
time frames

A

1 year = 90%, 10 years = 60% for cadaveric transplants
* 1 year = 95%, 10 years = 70% for living-donor transplants

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14
Q

Renal Transplant: Post-op problems (can cause graft dysfunction) – up to 4 months post-op:

A

Acute rejection: risk is great in 1st 2 weeks occurs in 30-50% of cases
* Ciclosporin toxicity
* A TN of graft
* V ascular thrombosis
* Urine leakage
* UTI

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15
Q

Hyperacute graft rejection

A
  • Due to antibodies against donor HLA type 1 antigens
  • Rarely seen due to HLA matching
    Management of acute graft failure (< 6 months)
  • Acute rejection: give steroids, if resistant use monoclonal antibodies
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16
Q

Causes of chronic graft failure (> 6 months)

A

Chronic allograft nephropathy
* Ureteric obstruction
* Recurrence of original renal disease (MCGN > IgA > FSGS)

Hyperacute graft rejection is due to pre-existent antibodies to HLA antigens and is therefore IgG
mediated

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17
Q

Autosomal Dominant Polycystic Kidney Disease:

A

(ADPKD) is the most common inherited cause of kidney disease, affecting 1 in 1,000 Caucasians. Two disease loci have been identified, PKD1 and PKD2, which code for polycystin-1 and polycystin-2 respectively

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18
Q

ADPKD - Ultrasound diagnostic criteria

A

In < 20 yrs age, CT scan is not needed
* In < 20 yrs age, ultrasound gives false –ve
* 2 cysts, unilateral or bilateral, if aged < 30 years
* 2 cysts in both kidneys if aged 30-59 years
* 4 cysts in both kidneys if aged > 60 years

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19
Q

ADPKD Associtaed conditions:

A

Colonic diverticula (with any related symptoms, screen by barium enema)
* Mitral Valve Prolapse (needs echo screening)

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20
Q

ADPKD Management:

A
  • Painkillers
  • Urinary tract infections: → ABX
  • ↑BP control
  • End-stage renal disease → Transplantation
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21
Q

Autosomal Recessive Polycystic Kidney Disease (ARPKD)

A

much less common than autosomal dominant disease (ADPKD). It is due to a defect in a gene located on chromosome 6
Diagnosis may be made on prenatal ultrasound or in early infancy with abdominal masses and renal failure. End-stage renal failure develops in childhood. Patients also typically have liver involvement, for example portal and interlobular fibrosis

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22
Q

Nephrotic Syndrome:
Triad of

A
  1. Proteinuria (> 3g/24hr) causing
  2. Hypoalbuminemia (< 30g/L) and
  3. Edema
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23
Q

Nephrotic Syndrome path

A

Loss of antithrombin-III (↑↑↑), proteins C and S and associated rise in fibrinogen levels
predispose to thrombosis. Loss of TBG lowers total, but not free thyroxin levels

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24
Q

Nephrotic Syndrome Causes

A
  1. Glomerulonephritis (GN, c. 80%)
    * Minimal change GN (causes 75% in children, 25% in adults)
    * Membranous GN
    * Focal Segmental GlomeruloSclerosis
  2. Systemic disease (c. 20%) * Amyloidosis
    * SLE
  3. Drugs
    * Gold (sodium aurothiomalate), penicillamine
  4. Others
    * Congenital
    * Neoplasia: carcinoma, lymphoma, leukemia, myeloma
    * Infection: bacterial endocarditis, hepatitis B, malaria
    * Renal vein thrombosis
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25
Q

Complications Nephrotic Syndrome

A

↑ risk of infection due to urinary immunoglobulin loss
* ↑ risk of thromboembolism related to loss of antithrombin III and plasminogen in the urine
* Hyperlipidemia
* Hypocalcemia (vitamin D and binding protein lost in urine)
* Acute renal failure could be due to thrombotic renal veins it comes with lion pain and
hematuria.

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26
Q

Membranous glomerulonephritis
Presentation
Cause

A

Presentation: proteinuria / nephrotic syndrome / CRF
* Cause: infections, rheumatoid drugs, malignancy
* 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRF

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27
Q

IgA nephropathy - AKA Berger’s disease, mesangioproliferative GN

A
  • Typically young adult with hematuria following an URTI
  • Associated with Henoch-Schonlein purpura
  • Mesangial hypercellularity (mesangioproliferative)
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28
Q

. Diffuse proliferative glomerulonephritis

A

Classical post-streptococcal glomerulonephritis in child
* Presents as nephritic syndrome / ARF
* Most common form of renal disease in SLE (IV)

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29
Q

Minimal change disease

A

Typically a child with nephrotic syndrome (accounts for 80%)
* Causes: Hodgkin’s, NSAIDs
* Good response to steroids

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30
Q

Focal segmental glomerulosclerosis

A

May be idiopathic or secondary to HIV, heroin
* Presentation: proteinuria / nephrotic syndrome / CRF

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31
Q

Rapidly progressive glomerulonephritis (RPGN) - AKA Crescentic Glomerulonephritis

A

Rapid onset, often presenting as ARF
* Causes include Goodpasture’s, ANCA positive vasculitis (e.g. Wegener’s granulomatosis)

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32
Q

. Mesangiocapillary glomerulonephritis (membranoproliferative)

A

Type 1: cryoglobulinemia, hepatitis C → associated with low C4
* Type 2: partial lipodystrophy → associated with low C3

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33
Q

Disorders associated with glomerulonephritis and low serum C3 levels:

A

Post-streptococcal glomerulonephritis
* Subacute bacterial endocarditis
* Systemic lupus erythematosus
* Mesangiocapillary glomerulonephritis

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34
Q

Membranous glomerulonephritis renal biopsy

A

Renal biopsy demonstrates:
* Sub-epithelial immune complex (mainly IgG and C3) deposition in the glomerulus
* Electron microscopy: the basement membrane is thickened with sub-epithelial electron dense
deposits (IgG, C3)

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35
Q

Membranous glomerulonephritis Causes

A

Idiopathic
* Infections: hepatitis B, malaria
* Malignancy: lung cancer, lymphoma, leukemia
* Drugs: gold, penicillamine, NSAIDs
* SLE (class V disease)

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36
Q

Membranous glomerulonephritis Management

A

Immunosuppressant: steroids, cyclophosphamide, chlorambucil e.g. Ponticelli regime
* BP control
* Consider anticoagulation

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37
Q

IgA Nephropathy: Basics

A

Also called Berger’s disease or mesangioproliferative glomerulonephritis
* Commonest cause of glomerulonephritis worldwide
* Pathogenesis unknown, ?Mesangial deposition of IgA immune complexes
* Histology: mesangial hypercellularity, positive immunofluorescence for IgA & C3

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38
Q

Differentiating between IgA nephropathy and post-streptococcal (diffuse proliferative) glomerulonephritis:

A
  • Post-streptococcal glomerulonephritis is associated with low complement levels
  • Main symptom in post-streptococcal glomerulonephritis is proteinuria (although hematuria can
    occur)
  • There is typically an interval between URTI and the onset of renal problems in post-
    streptococcal glomerulonephritis
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39
Q

IgA Nephropathy:Presentations

A

Young ♂, recurrent episodes of Hematuria, usually painless (sometimes with no renal impairment)
* Typically associated with mucosal infections e.g., URTI
* Nephrotic range proteinuria is rare
* Renal failure

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40
Q

IgA Nephropathy Associated conditions

A

Alcoholic cirrhosis
* Celiac disease/dermatitis herpetiformis

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41
Q

IgA Nephropathy Prognosis

A

25% of patients develop ESRF
* Markers of good prognosis: frank hematuria
* Markers of poor prognosis: ♂ gender, proteinuria (especially > 2 g/day), hypertension,
smoking, hyperlipidemia, ACE genotype DD

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42
Q

Minimal Change Glomerulonephritis presentation

A

Nearly always presents as nephrotic syndrome, accounting for 75% of cases in children and 25% in adults

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43
Q

Minimal Change Glomerulonephritis treatments

A

prednisolone

ACE inhibitors may be used to ↓ proteinuria in patients with heavy proteinuria or who have a slow response to prednisolone

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44
Q

Minimal Change causes

A

Causes: majority of cases are idiopathic, but in around 10-20% a cause is found:
* Drugs: NSAIDs, rifampicin
* Hodgkin’s lymphoma, NHL and thymoma
* Infectious mononucleosis

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45
Q

Minimal Change Features

A
  • Nephrotic syndrome
  • Normotension - hypertension is rare
  • Hematuria is very rare
  • Highly selective proteinuria*
  • Renal biopsy: electron microscopy shows fusion of podocytes
46
Q

Minimal Change Management

A
  • Majority of cases (80%) are steroid responsive
  • Cyclophosphamide is the next step for steroid resistant cases
    Prognosis is overall good, although relapse is common. Roughly:
  • 1/3 have just one episode
  • 2/3 have relapses:
    o 1/3 have infrequent relapses
    o 1/3 have frequent relapses which stop before adulthood
47
Q

Mesangiocapillary Glomerulonephritis: Overview

A

AKA membranoproliferative glomerulonephritis
* May present as nephrotic syndrome, hematuria or proteinuria
* Poor prognosis

48
Q

Mesangiocapillary Glomerulonephritis: Type 1

A

Subendothelial immune deposits
* Cause: Cryoglobulinemia (with low C4), hepatitis C

49
Q

Type 2 RTA (proximal)

A
  • ↓ HCO3- reabsorption in proximal tubule
  • Hypokalemia
  • Complications include osteomalacia
  • Causes include idiopathic, as part of Fanconi
    syndrome, Wilson’s disease, cystinosis,
    outdated tetracyclines
  • Treat: Bicarb replacement + Thiazide diuretics
50
Q

Focal Segmental Glomerulosclerosis Causes

A
  • Idiopathic
  • Secondary to other renal pathology e.g. IgA nephropathy, reflux nephropathy
  • HIV
  • Heroin
  • Alport’s syndrome
  • Sickle-cell
51
Q

Alport’s Syndrome

A

is usually inherited in an X-linked dominant pattern*. It is due to a defect in the gene which codes for type IV collagen resulting in an abnormal glomerular-basement membrane (GBM). The disease is more severe in ♂s with ♀s rarely developing renal failure

A favorite question in the MRCP is an Alport’s patient with a failing renal transplant. This may be caused by the presence of anti-GBM antibodies leading to a Goodpasture’s syndrome like picture

52
Q

Alport’s syndrome usually presents in childhood. The following features may be seen:

A
  • Progressive renal failure
  • Bilateral sensorineural deafness
  • Lenticonus: protrusion of the lens surface into the anterior chamber
  • Retinitis pigmentosa
53
Q

Renal Stones: RF

A

Risk factors
* Dehydration
* Hypercalciuria, hyperparathyroidism, hypercalcemia
* Cystinuria NOT CYSTINOSIS
* High dietary oxalate
* Renal tubular acidosis
* Medullary sponge kidney, polycystic kidney disease
* Beryllium or cadmium exposure

54
Q

Drug causes renal stones

A

Drugs that promote calcium stones: loop diuretics, steroids, acetazolamide, theophylline
* Thiazides can prevent calcium stones (↑ distal tubular calcium resorption)

55
Q

Proteus infections renal stone

A

stag-horn calculi i

56
Q

Acute management of renal colic

A

Diclofenac 75 mg by intramuscular injection is the analgesia of choice for renal colic. A second dose can be given after 30 minutes if necessary. (PR diclofenac is an alternative)

57
Q

Prevention of renal stones

A

Calcium stones
* High fluid intake
* Low animal protein, low salt diet (a low calcium diet has not been shown to be superior to a
normocalcemic diet)
* Thiazide diuretics (↑ distal tubular calcium resorption)
* Stones < 5 mm will usually pass spontaneously
* Lithotripsy, nephrolithotomy may be required

58
Q

Oxalate stones

A

Cholestyramine ↓ urinary oxalate secretion
* Pyridoxine ↓ urinary oxalate secretion

59
Q

Diabetic Nephropathy five stages

A

Stage 1
* Hyperfiltration: ↑ in GFR (60-140 ml/min/1.73m2)
* May be reversible
Stage 2 (silent or latent phase)
* Most patients do not develop microalbuminuria for 10 years
* GFR remains elevated
Stage 3 (incipient nephropathy)
* Microalbuminuria (albumin excretion of 30 - 300 mg/day, dipstick negative)
Stage 4 (overt nephropathy)
* Persistent proteinuria (albumin excretion > 300 mg/day, dipstick positive)
* Hypertension is present in most patients
* Histology shows diffuse glomerulosclerosis and focal glomerulosclerosis (kimmelstiel-wilson
nodules)
Stage 5
* End-stage renal disease, GFR typically < 10ml/min
* Renal replacement therapy needed

60
Q

Screening Diabetic Nephropathy

A

Screening
* All patients should be screened annually
* Albumin:Creatinine ratio (ACR) in early morning specimen
* ACR > 2.5 = microalbuminuria

61
Q

Management Diabetic Nephropathy

A

Dietary protein restriction
* Tight glycemic control
* BP control: aim for < 130/80 mmHg
* Benefits independent of blood pressure control have been demonstrated for ACE inhibitors and
angiotensin II receptor blockers - these may be used alone or in combination
* Control dyslipidemia e.g. Statins

62
Q

Proteinuria

A

is an important marker of chronic kidney disease, especially for diabetic nephropathy. NICE recommend using the albumin:creatinine ratio (ACR) in preference to the protein:creatinine ratio (PCR) when identifying patients with proteinuria as it has greater sensitivity. For quantification and monitoring of proteinuria, PCR can be used as an alternative, although ACR is recommended in diabetics. Urine reagent strips are not recommended unless they express the result as an ACR

63
Q

Causes of transient or spurious non-visible hematuria

A

Urinary tract infection
* Menstruation
* Vigorous exercise
* Sexual intercourse

64
Q
A
65
Q

Fanconi syndrome

A

describes a generalized disorder of renal tubular transport resulting in:
* Type 2 (proximal) renal tubular acidosis
* Aminoaciduria
* Glycosuria
* Phosphaturia
* Osteomalacia

66
Q

all types of renal tubular acidosis (RTA) are associated with

A

hyperchloremic metabolic acidosis (normal anion gap)

67
Q

Type 1 RTA (distal)

A

nability to generate acid urine (secrete H+) in distal tubule
* Hypokalemia
* Complications include nephrocalcinosis and
renal stones
* Causes include idiopathic, RA, SLE, Sjogren’s

68
Q
A
69
Q

Type 4 RTA (hyperkalemic RTA)

A

is not actually a tubular disorder at all nor does it have a clinical picture similar to the other RTAs. It was included in the classification of RTA as it is associated with a mild (normal anion gap) metabolic acidosis due to a physiological ↓ in proximal tubular ammonium excretion.

70
Q

Type 3 RTA (Juvenile RTA) is combined proximal & distal RTA.

A

Features:
* Results from inherited carbonic anhydrase II deficiency.
* Mutations in the gene encoding this enzyme give rise to:
o Autosomalrecessivesyndromeofosteopetrosis o Renal tubular acidosis
o Cerebral calcification
o Mental retardation.
* 70% of the reported cases are from the Magreb region of North Africa Type 3 is rarely discussed

71
Q

Prerenal Uremia vs Acute Tubular Necrosis

A

Urine sodium
< 20 mmol/L vs > 30 mmol/L

72
Q

Papillary Necrosis: Causes

A

Causes
* Chronic analgesia use
* Sickle cell disease
* TB
* Acute pyelonephritis
* Diabetes Mellitus

73
Q

Papillary Necrosis: Features

A

Fever, loin pain, hematuria
* IVU (intravenous urogram or IVP for pyelogram) - papillary necrosis with renal scarring - ‘cup
& spill’

74
Q

Sterile Pyuria:

A

Causes
* Partially treated UTI
* Urethritis e.g. Chlamydia, TB and ureaplasma urealyticum
* Renal tuberculosis
* Renal stones
* Appendicitis
* Bladder/renal cell cancer
* Adult polycystic kidney disease
* Analgesic nephropathy

75
Q

Renal Cell Cancer associations
t arises from proximal renal tubular epithelium

A
  • More common in middle-aged men
  • Smoking
  • Von hippel-lindau syndrome
  • Tuberous sclerosi
76
Q

Renal Cell Cancer Features

A
  • Classical triad: hematuria, loin pain, abdominal mass
  • Pyrexia of unknown origin
  • Left varicocele (due to occlusion of left testicular vein)
  • Endocrine effects: may secrete erythropoietin (polycythemia), parathyroid hormone
    (hypercalcemia), renin, ACTH
  • 25% have metastases at presentation
77
Q

Renal Cell Cancer Management

A

Radical nephrectomy for confined disease
* α-interferon and interleukin-2 have been used to ↓ tumor size and also treat patients with
metastases
* Receptor tyrosine kinase inhibitors (e.g. Sorafenib, sunitinib) have been shown to have superior
efficacy compared to interferon-α

78
Q

Wilm’s Tumor (Nephroblastoma)

A

is one of the most common childhood malignancies. It typically presents in children less than 5 years of age, with a median age of 3 years.

79
Q

Wilm’s Tumor (Nephroblastoma) features

A

eatures
* Abdominal mass (most common presenting feature)
* Painless hematuria
* Flank pain
* Other features: anorexia, fever
* Unilateral in 95% of cases
* Metastases are found in 20% of patients

80
Q

Wilm’s Tumor (Nephroblastoma) Associations

A

Beckwith-Wiedemann syndrome is a inherited condition associated with organomegaly, macroglossia, abdominal wall defects, Wilm’s tumor and neonatal hypoglycemia
* As part of WAGR syndrome: Wilms Aniridia, Genitourinary malformations, mental Retardation. It results from a deletion on chromosome 11 resulting in the loss of several genes.
* Hemihypertrophy
* Around one-third of cases are associated with a mutation in the WT1 gene on chromosome 11

81
Q

Renal Vascular Disease

A

hypertension, chronic renal failure or ‘flash’ pulmonary edema.FMD is more common in young women and characteristically has a ‘string of beads’ appearance on angiography.

82
Q

Renal Vascular Disease Investigation

A

MR angiography is now the investigation of choice
* CT angiography
* Conventional renal angiography is less commonly performed used nowadays, but may still have
a role when planning surgery

83
Q

HIV-associated nephropathy (HIVAN)

A

Massive proteinuria
* Normal or large kidneys
* Focal segmental glomerulosclerosis with focal or global capillary collapse on renal biopsy
* Elevated urea and creatinine
* Normotension

84
Q

Goodpasture’s syndrome

A

IgG deposits on renal biopsy
* Anti-GBM antibodies

85
Q

goodpastures mediated by?

A

a type II hypersensitivity reaction
autoimmune disease triggered when the patient’s immune system
anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen.

86
Q

goodpastures features F

A

Features
* Pulmonary hemorrhage
* Followed by rapidly progressive glomerulonephritis

87
Q

Investigations good pastures

A

Renal biopsy: linear IgG deposits along basement membrane
* ↑ transfer factor secondary to pulmonary hemorrhages
* Lung biopsy: accumulation of hemosidren laden macrophages with alveoli

88
Q

Hemolytic Uremic Syndrome

A

generally seen in young children and produces a triad of:
* Acute renal failure
* Microangiopathic hemolytic anemia
* Thrombocytopenia

89
Q

Causes Hemolytic Uremic Syndrome

A

Post-dysentery - classically E coli 0157:H7 (‘verotoxigenic’, ‘enterohemorrhagic’)
* Tumors
* Pregnancy
* Cyclosporine, the Pill
* SLE
* HIV

90
Q

HUS Investigations

A
  • Full blood count: anemia, thrombocytopenia, fragmented blood film
  • U&E: acute renal failure
  • Stool culture
91
Q

HUS

A

Management
* Treatment is supportive e.g. Fluids, blood transfusion and dialysis if required
* There is no role for antibiotics, despite the preceding diarrheal illness in many patients
* The indications for plasma exchange in HUS are complicated. As a general rule plasma
exchange is reserved for severe cases of HUS NOT associated with diarrhea

92
Q

Phenylketonuria (PKU)

A

is an autosomal recessive condition caused by a disorder of phenylalanine metabolism.

defect in phenylalanine hydroxylase, an enzyme which converts phenylalanine to tyrosine

gene for phenylalanine hydroxylase is located on chromosome 12

93
Q

Phenylketonuria (PKU) Features

A

Usually presents by 6 months e.g. With developmental delay
* Child classically has fair hair and blue eyes
* Learning difficulties
* Seizures, typically infantile spasms
* Eczema
* ‘Musty’ odor to urine and sweat*

94
Q

Cystinuria what is this

A

an autosomal recessive disorder characterized by the formation of recurrent renal stones. It is due to a defect in the membrane transport of cystine, ornithine, lysine, arginine (mnemonic = COLA)
Genetics

Recurrent renal stones
* Are classically yellow and crystalline, appearing semi-opaque on x-ray

95
Q

Cystinuria genetics

A

Chromosome 2: SLC3A1 gene, chromosome 19: SLC7A9

96
Q

Cystinuria D+M

A

Diagnosis
* Cyanide-nitroprusside test
Management
* Hydration
* D-penicillamine
* Urinary alkalinization

97
Q

Homocystinuria

A

are autosomal recessive disease caused by deficiency of cystathione β- synthetase. This results in an accumulation of homocysteine which is then oxidized to homocysteine.

98
Q

Homocystinuria features

A
  • Often patients have fine, fair hair
  • Musculoskeletal: may be similar to Marfan’s - arachnodactyly etc
  • Neurological patients may have learning difficulties, seizures
  • Ocular: downwards dislocation of lens (Marfan has upward dislocation of lens)
  • ↑ Risk of arterial and venous thromboembolism except coronaries.
  • Also malar flush, livedo reticularis
99
Q

Alkaptonuria: (black urine disease or alcaptonuria)

A
  • Rare inherited genetic disorder of phenylalanine and tyrosine metabolism
  • Autosomal recessive
  • Common in Slovakia and the Dominican Republic than in other countries.
  • Due to a defect in the enzyme homogentisate 1,2-dioxygenase, which participates in the
    degradation of tyrosine. As a result, a toxic tyrosine byproduct called homogentisic acid (or alkapton) accumulates in the blood and is excreted in urine in large amounts. Excessive homogentisic acid causes damage to cartilage (ochronosis, leading to osteoarthritis) and heart valves as well as precipitating as kidney stones.
100
Q

Alkaptonuria features

A

The main symptoms of alkaptonuria are due to the accumulation of homogentisic acid in tissues. In the joints this leads to cartilage damage, specifically in the spine, leading to low back pain at a young age in most cases. Cartilage damage may also occur in the hip and shoulder. Joint replacement surgery (hip and shoulder) is often necessary at a relatively young age.

Valvular heart disease, - progressive cases valve replacement may be necessary. Coronary artery disease may be accelerated in alkaptonuri

ar wax exposed to air turns red or black

101
Q

Benign prostatic hyperplasia (BPH) RF

A

Risk factors
* Age: around 50% of 50-year-old men will have evidence of BPH and 30% will have symptoms.
Around 80% of 80-year-old men have evidence of BPH
* Ethnicity: Black > White > Asian

102
Q

BPH typically presents with lower urinary tract symptoms (LUTS), which may be categorized into:

A

Voiding symptoms (obstructive): weak or intermittent urinary flow, straining, hesitancy,
terminal dribbling and incomplete emptying
* Storage symptoms (irritative) urgency, frequency, urgency incontinence and nocturia
* Post-micturition: dribbling
* Complications: urinary tract infection, retention, obstructive uropathy

103
Q

BPH Management options

A

W atchful waiting
* Medication: α-1 antagonists, 5 α-reductase inhibitors. The use of combination therapy was
supported by the medical therapy of prostatic symptoms (MTOPS) trial
* Surgery: transurethral resection of prostate (TURP)

104
Q

α-1 antagonists e.g. tamsulosin, alfuzosin

A

↓ smooth muscle tone (prostate and bladder)
* Considered first-line, improve symptoms in around 70% of men
* Adverse effects: dizziness, postural hypotension, dry mouth, depression

105
Q

5 α-reductase inhibitors e.g. finasteride

A
  • Block the conversion of testosterone to dihydrotestosterone (DHT), which induces BPH
  • Unlike α-1 antagonists causes a reduction in prostate volume and hence may slow disease
    progression. This however takes time and symptoms may not improve for 6 months. They may
    also ↓ PSA concentrations by up to 50%
  • Adverse effects: erectile dysfunction, ↓ libido, ejaculation problems, gynecomastia
106
Q

Prostate cancer: Risk Factors:

A
  • Age
  • ↑ fat diet
  • Family Hx
  • BPH is not a risk factor
107
Q

Prostate cancer: Management

A

Localized disease = T1/2
T1 - clinically unapparent disease:
* If life expectancy < 10 years then watchful waiting
* If life expectancy > 10 years then offer:
o Radical prostatectomy o Radical radiotherapy

T2 - palpable disease confined to prostate* Radical prostatectomy
* Radical radiotherapy (often if older patient)

Locally advanced disease (T3/4)
* T3 = beyond prostatic capsule }
* T4 = involves bladder neck or rectum
* Most men will have occult mets

Disseminated disease - hormonal therapy
Radiotherapy

108
Q

Synthetic GnRH agonist

A

E.g. Goserelin (zoladex) is a synthetic GnRH agonist which provides negative feedback
to the anterior pituitary.
o Cover initially with anti-androgen to prevent rise in testosterone

109
Q

Bladder cancer:

A

common urological cancer with most cases being transitional cell carcinomas. It has a ♂:♀=3:1 with women generally having a worse prognosis than men. The most classical presentation is with total, gross, painless hematuria.

110
Q

Bladder cancer: Associated Factors:

A
  • Smoking
  • Occupational: aniline dyes used in printing and textile industry, rubber manufacture
  • Aromatic amines
  • Prior radiation treatment to the pelvis
  • Exposure to a urinary metabolite of cyclophosphamide (acrolein).
  • Schistosomiasis (S. hematobium infection)
  • Mutations on 17p13.1, the gene coding for p53, mutations of which are associated with high-
    grade bladder cancer
  • Mutation on 9p15 and 9p16, another tumor suppressor gene associated with low grade and
    superficial tumors.
  • Drugs: cyclophosphamide

MRCP I (21 decks)

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