Exam 3 -- Renal Disease Flashcards

(181 cards)

1
Q

How long must there be a structural or functional kidney abnormality in order to consider the condtion as chronic?

A

3 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

True or false: less than 10% of US adults have some sort of chronic kidney disease (CKD)

A

False; greater than 10%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

When would a condition be considered end stage renal disease (ESRD)?

A

When something extra is needed to keep the patient alive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

True or false: African Americans are more likely to have ESRD than Caucasians

A

True; this may have some connection with uneven distribution of healthcare

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What sorts of ocular symptoms may occur with renal disease?

A

Calcium deposits, lid edema, corneal changes, aniridia, cataracts, uveitis, optic nerve head edema, drusen around macula, retinal changes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the three excretory functions of the kidney?

A

Removal of waste products, excretion of foreign substances (drugs, etc), regulate blood composition (amount of water, ion balance, acid-base balance)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

True or false: the kidney has little, if any, endocrine function

A

False; it is involved with calcium regulation and uptake (calcitriol), RBC production (erythropoietin), autoregulation of blood flow and water reabsorption (prostaglandins, NO, and endothelins have one or both of these functions)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Of the blood received by the kidney, how much is actually filtered?

A

20%; the other 80% is for the kidney tissue itself and to transport the substances that are saved.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the flow rate of blood through the kidneys?

A

About 1.2 L/min

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the name of the structure that connects the kidney to the bladder?

A

Ureter; it connects at the hilus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Which has a higher sodium concentration, the renal medulla or cortex?

A

Medulla

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Where are juxtoglomerular cells located, and what is their function?

A

They are next to the glomerulus in the afferent arteriole; they are specialized smooth muscle cells that interact with baroreceptors to release renin in response to decrased blood pressure.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What characteristics of the glomerulus contribute to its filtration ability?

A

Fenestrated endothelium, incomplete basement membrane, podocytes with foot processes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Know that the structure of the glomerulus is similar to the structure of choriocapilaris and the RPE cells, so things that affect the glomerulus could affect the eye too.

A

Free card

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Where are the mesangial cells and what is their function?

A

They are between the glomerular capillaries and function to hold the capillaries together. They also play a phagocytic role and have contractile capabilities to control blood flow.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What happens to renal blood flow with sympathetic nervous system activation?

A

Afferent arteriole constricts and renin is released. Renin end result (through angiotensin II) is more vasoconstriction, especially of the efferent arteriole.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Which chemical mediators affect renal blood vessels?

A

Renin (indirectly through angiotensin II), prostaglandins, NO, endothelin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

The glomerulus can be thought of as a negatively charged colander. What are the two main factors that characterize glomerulus selective permeability?

A

Size and charge (big things can’t fit through and negatively charged things are repelled)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Filtrate in the kidney is mainly composed of what?

A

Water, ions, glucose, amino acids, bicarbonate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

True or false: the glomerulus plays a large role in reabsorption

A

False; it plays no role in reabsorption. It’s like when you clean out your dresser drawer; you just dump everything out onto the bed. Glomerulus just dumps stuff into the rest of the nephron, which then reabsorbs most of it.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

The transport of many filtrate substances is related to reabsorption of which ion?

A

Sodium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What sorts of molecular transport are involved in reabsorption from the filtrate?

A

Exchange, shared transport, solvent drag

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Which two characteristics are major modifiers of reabsorption?

A

Concentration of substances (there more there are, the more that can be reabsorbed), rate of flow (faster flow decreases time for transporter to get its job done)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is the addition of substances into the filtrate called?

A

Secretion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Which substances are secreted in the proximal tubule?
Uric acid, organic acids
26
Which substances are reabsorbed in the proximal tubule?
Water, NaCl, bicarbonate, potassium, glucose, amino acids
27
Which substances are secreted in the descending loop of Henle?
None
28
Which substances are reabsorbed in the descending loop of Henle?
Water, due to hyperosmolarity
29
Which substances are secreted in the ascending loop of Henle?
None
30
Which substances are reabsorbed in the ascending loop of Henle?
NaCl and potassium, through active transport
31
Which substances are secreted in the distal tubule?
Potassium, hydrogen
32
Which substances are reabsorbed in the distal tubule?
Water, NaCl (increased by aldosterone), calcium (increased by PTH)
33
Which substances are secreted in the collecting duct?
Potassium (in exchange for NaCl reabsorption; increased by aldosterone)
34
Which substances are reabsorbed in the collecting duct?
Urea, water (increased by vasopressin), NaCl (in exchange for potassium secretion; increased by aldosterone)
35
In normal function, all of the ions and molecules of the filtrate experience some amount of reabsorption, except one. Which is it?
Creatinine
36
Where are the macula densa cells located, and what is their function?
They are located at the junction of the ascending loop of Henle with the distal tubule. Their function is to taste the filtrate to sense the concentration of NaCl. If [NaCl] is low, they tell the juxtaglomerular cells to release more renin so as to reabsorb more water.
37
The renin-angiotensin-aldosterone system acts to increase BP. As briefly as possible, outline this system.
Renin from kidney combines with angiotensinogen from liver to form angiotensin I, which is converted by ACE from lungs to angiotensin II. Angiotensin II increases thirst and causes vasoconstriction; it acts on the proximal tubule to increase sodium and water retention; it also acts on the pituitary to release vasopressin. Vasopressin acts on the kidney to increase water reabsorption. Pituitary also stimulates release of aldosterone from adrenal gland, which acts to increase sodium and water retention
38
What are the major stimulators for vasopressin release?
Decreased blood volume (sensed by baroreceptors in many places), increased blood osmolarity (primarily sodium and mannitol, but also glucose)
39
What stimulates erythropoietin release from the kidneys, and what effect does it have?
Hypoxia is the most common stimulant of erthyropoietin release; erythropoietin stimulates bone marrow production of RBCs
40
Calcitriol helps absorb calcium from the gut and is important for building bone. What molecule does it act on? What bone issue might a person with CKD have?
Calcitriol converts vitamin D to its metabolically active form (D3); CKD can cause osteoporosis
41
True or false: the kidneys can convert lactic acid to glucose
True.
42
What are the autocrine hormones or molecules that act in the kidneys?
Endothelins, NO, prostaglandins
43
The endothelins are peptides that are kidney autocrine hormones. What do endothelins do in the kidneys?
They increase BP through vasoconstrition and salt/water retention.
44
NO has autocrine functions in the kidneys. What does it do?
It causes more water to excreted; it is also involved in the macula densa feedback loop.
45
What is the overall effect of renal prostaglandins?
They increase renal bloodflow by preventing vasoconstriction; they also impair water reabsorption by blocking vasopressin in the collecting duct, prevent water and sodium reabsorption in the tubules, and prevent potassium excretion
46
Acute kidney failure can be brought about by which classes of common medications?
NSAIDs and steroids; these interfere with initial filtration, resulting in edema
47
The kidneys influence blood pH by excreting __________ when the blood is too acidic and ___________ when the blood is too basic.
Hydrogen ions; bicarbonate
48
How much (%) of the glomerular filtrate ends up becoming urine?
1%
49
What is the basis of the glomerular filtration rate test?
You have the patient ingest a known amount of a substance that they can't get anywhere else, one that will be put into the filtrate by the glomerulus but not reabsorbed by the tubules, and take timed urine and blood samples to see how quickly this substance is eliminated from the body.
50
What is the basis of the creatinine clearance rate? What important use does this test have for optometrists (or any doctor)?
Have the patient collect their urine for 24 hours, then you draw their blood. Measure the creatinine in the urine and the creatinine in the blood to see how well the kidney is excreting the creatinine (none should be reasbsorbed). You would use this test to adjust a patient's dosage of medications.
51
Serum creatinine is a useful screening test for kidney disease. If a person's serum creatinine level is twice the normal value, what does this indicate about their kidney function?
2x the creatinine level means 50% kidney function (3x would mean 25%, and 4x would mean 12.5%, etc.)
52
The blood, urea, nitrogen (BUN) test is one that can be used to indicate kidney function. It measures levels of urea in the blood. Generally, how much urea should be reabsorbed, and how much should be excreted? A/An ______________ (increased/decreased) value of a BUN test compared to the normal would indicate reduced kidney function. What causes this value to change?
50% should be reabsorbed, 50% should be excreted. Increased BUN value would indicate reduced kidney function. This is because decreased glomerular filtration rate allows more reabsorption of urea.
53
What is azotemia?
An increase in BUN without symptoms.
54
What substances would you not expect to find in the urine of a healthy individual? What else could you use a urinalysis to look for?
You expect no protein, no blood, and no glucose. You could use urinalysis to look a patient's pH (amount of dehydration) or look for presence of WBCs (possible infection)
55
The presence of which protein in the urine may be the first sign of kidney involvement in DM?
Albumin
56
What values tend to increase with kidney failure? Which tend to decrease?
Increase: serum potassium and phosphate; Decrease: serum calcium, pH, and bicarbonate
57
What is the NGAL test good for?
It is useful for detecting acute kidney damage so we can catch the issue earlier and have a better chance at fixing it. The other tests don't catch damage until it is worse.
58
Which imaging technique involves a camera being worked up into the bladder?
Cytoscopy
59
Which imaging technique is the only one that can give an idea of kidney function, rather than only structure? What concern may there be with the contrast agent used?
Intravenous pyelogram (IVP); its contrast agent is related to iodine, so it can't be used with patients allergic to iodine
60
What are the four categories of descriptors that can be used to describe a case of kidney disease?
Time (acute vs. chronic); structure affected (glomerular, tubular, etc); where issue is located (pre-renal, interstitial, post-renal); cause of issue (diabetic)
61
End stage renal disease (ESRD) is defined by kidney function reduced to ____________% of normal.
15
62
What is the most common cause of kidney failure? Second most common? What immune conditions may trigger kidney disease?
Diabetes is #1, HTN is #2; SLE, Sjogren's, scleroderma can also cause kidney failure
63
True or false: symptoms of kidney disease tend to occur early on in the disease and always are acute
False; they tend to occur later on and can occur either acutely or slowly progressive
64
What are some of the common first symptoms of kidney disease?
High BP, swelling of legs, pulmonary edema, fatigue, HA, weight loss, N&V, itching (due to calcium and phosphate build up in tissues leading to drying), increased tendency to bleed (due to lower erythropoietin and therefore lower RBCs), cognitive impairment
65
Suppose a patient has azotemia. Once the patient has signs or symptoms (or we are able to identify the signs or symptoms), then call it what?
Uremia
66
What are some of the symptoms that CKD can cause in the blood?
Anemia (pallor, lethargy, breathlessness on exercise) and platelet abnormalities (epistaxis and bruising) due to low levels of erythropoietin
67
What are some of the symptoms that CKD can cause in the skin?
Pigmentation (due to MSH not being excreted as well) and pruritus (due to increased levels of calcium and phosphate drying out tissue)
68
What are some of the symptoms that CKD can cause in the GI tract?
Anorexia, N&V, diarrhea (due to acid-base imbalance and ion imbalance)
69
What are some of the symptoms that CKD can cause in the endocrine system?
Amenorrhea, erectile dysfunction, infertility (due to hormonal imbalance)
70
True or false: the tingling due to polyneuropathy in CKD extends only as far as the wrists and ankles
False; it extends up most of the arm/leg (stocking-glove pattern)
71
What are some of the symptoms that CKD can cause in the CNS?
Confusion, coma, seizures (due to ion imbalance in the blood)
72
What are some of the symptoms that CKD can cause in the cardiovascular system?
Pericarditis (due to ion imbalance), HTN, peripheral vascular disease, heart failure (due to fluid buildup)
73
What are some of the symptoms that CKD can cause in the renal system?
Nocturia (due to lying down at night and fluid in legs being filtered by kidneys), salt and water retention, edema
74
What are some of the symptoms that CKD can cause in the bones?
Osteomalacia, bone pain, hyperparathyroidism
75
Acute renal failure is an abrupt decrease in renal function. What are a few general causes of ARF? Which cause is the most common cause? Which is most deadly?
Some medications (interference with function, hypersensitivity reactions, toxicity), ischemia, autoimmune disease, infections. Ischemia due to heart surgery is the most common cause, and ischemia in general is the most deadly cause.
76
Signs and symptoms of ARF reflect loss of regulatory, excretory, and endocrine function, but these can take a while to present. What is the most common first sign of ARF?
Decrease in urine output
77
True or false: if a patient survives the initial episode of ARF, they will likely recover full kidney function
True.
78
What major class of medications can cause ARF?
NSAIDs, by interfering with prostaglandin synthesis and thus preventing vasodilation. This basically causes the kidney to become ischemic.
79
Hypersensitivity reactions to certain drugs can be a cause of ARF. Which drugs or classes of drugs can do this? What type of hypersensitivity reaction is possible?
Penicillin, ciprofloxacin, sulfonamides, NSAIDs; type I (anaphylactic) and type IV (delayed) are both possible
80
Toxicity (i.e. drug overdose) is one possible cause of ARF that typically occurs within one week of treatment. Which classes of drugs are common causes of renal toxicity?
Aminoglycosides (theorized that 10% of patients on this class have nephrotoxicity); radiographic contrast agents
81
A patient being sent out for an MRI of the orbit would be at greater risk of developing ARF if they have failed to do what?
Drink enough water.
82
Acute tubular necrosis occurs in response to acute ischemia or nephrotoxic insult. The glomerular filtration rate declines abruptly and tubular epithelial cells are damaged or destroyed, decreasing ion transport.
Free card
83
In acute tubular necrosis, intratubular pressure can rise. What is the mechanism of this spike in pressure? What happens as the pressure continues to rise?
Dead tubular epithelial cells slough off and start blocking the tubule, backing up the fluid that is still coming. As the pressure gets great enough, fluid can be forced into the tissue of the medulla, raising the pressure there enough to collapse other tubules; this raised pressure can also damage the renal vasculature.
84
Acute tubular necrosis can result in damage to renal vasculature. What is the result of damaged renal vasculature?
Endothelin release is increased, NO and prostaglandin release are decreased, all of which result in vasoconstriction. This vasoconstriction results in reduced urine volume, increased body fluid, and increased blood waste products.
85
What is a form of ARF that may be seen first by an optometrist (due to the presenting symptoms)?
Malignant hypertension
86
What are the symptoms of malignant hypertension?
Occipital HA, N&V, visual scotoma and spots
87
Malignant hypertension is characterized by…?
Diastolic BP > 120mmHg (systolic is even more elevated above normal), optic nerve head edema, encephalopathy (confusion, non-responsiveness), high BP leading to cardiovascular abnormalities. This is caused by renal failure!
88
True or false: malignant hypertension is not a very big deal
False; it is a medical emergency, requiring immediate and aggressive anti-hypertensive therapy
89
Which three kidney conditions discussed in class are diseases that affect the glomerulus?
Nephrotic syndrome, acute glomerulonephritis (aka nephritic syndrome), membranoproliferative glomerulonephritis
90
Which three kidney conditions discussed in class are diseases that affect the tubules and inserstitium?
Tubular interstitial nephropathy, pyelonephritis, acute tubular necrosis
91
Nephrotic syndrome is characterized by a reduction of podocytes around the glomerulus, making the capillary walls leaky. What are some urine or blood characteristics you might observe in a patient with this syndrome?
Proteinuria (protein in the urine), hypoalbuminemia (because protein is leaving the body through the urine); low blood levels of albumin trigger production of all plasma proteins, leading to hyperlipiuria and hyperlipidemia. Low levels of albumin also make the patient clot more, increasing risk of thromboembolism.
92
What causes the edema seen in nephrotic syndrome? Which structures are often affected first?
Decreased albumin in the blood shifts the osmotic balance so fluid stays in tissues (this can cause pitting edema). Eyelids are most often affected first.
93
True or false: you would expect a patient with nephrotic syndrome to have hypertension
False; nephrotic patients are not necessarily hypertensive (because the extra fluid is in the interstitial fluid, not the vasculature).
94
Nephrotic syndrome seems to be caused by injury of some sort followed by an immune response. What are a few common causes of nephrotic syndrome?
Diabetes, HTN, lupus, NSAIDs (toxic effect), membranoproliferative glomerulonephritis.
95
ACE inhibitors and angiotensin receptor blockers (ARBs) are part of the treatment plan for individuals with nephrotic syndrome. What effects do they have?
They cause a decrease in renin, which means that the efferent arteriole vasodilates more. This puts less pressure on the glomerulus, thus decreasing the GFR. They also improve the charge and size selectivity of the glomerular basement membrane by keeping the slit material between podocytes more intact.
96
Besides ACE inhibitors and ARBs, what can be done to treat a patient with nephrotic syndrome?
Remove fluid with diuretics (loop diuretics especially), restrict fluid and sodium intake, have patient on a prophylactic blood thinner during the first 6 months (time of highest risk of clotting).
97
Should other medical treatments not work, what type of medication might be used as a "last stand" in treating nephrotic syndrome? What types of patients are most commonly placed on this type of medication?
Immune suppressive drugs (normally corticosteroids). Children most commonly. (This treatment works pretty well.)
98
Corticosteroids are sometimes used as a last stand treatment for nephrotic syndrome. Their use is, however, controversial. Why?
The disease tends to come back when you try to take the patient off the steroids.
99
True or false: nephrotic syndrome, with time, tends to remit as the body heals itself
True, unless there has been permanent injury done to the kidneys such as can happen with diabetes or HTN.
100
True or false: glomerulonephritis (aka nephritic syndrome) is characterized by inflammation of the glomerulus and nephron that follows injury.
False; it is thought that nephritic syndrome is inflammation right from the start.
101
In a person with nephritic syndrome, what substance might you find in the urine that would not be there in a normal person?
Blood (hematuria)
102
One of the first signs of nephritic syndrome may be ___________
Oliguria (reduction in urine output)
103
True or false: you would expect HTN in nephrotic and nephritic syndromes
False; HTN is not common in nephrotic syndrome (fluid is retained in tissue) but is in nephritic syndrome (fluid is retained in vasculature)
104
True or false: nephritic syndrome, unlike nephrotic syndrome, does not have edema as one of its signs
False; nephritic syndrome can manifest with mild edema (of eyelids and face first), but it doesn't progress to pitting edema like nephrotic syndrome does.
105
One of the common causes of nephritic syndrome is post streptococcal infection. What occurs to cause the syndrome?
10-14 days after the strep infection, the body is still fighting bits and pieces of strep that is left over in the body. Some of the pieces end up in the kidneys, and inflammation there causes damage. (Ag-Ab complex is thought to be what is causing the inflammatory response.)
106
True or false: nephritic syndrome, if caused by a prior streptococcal infection, is most common in children and occurs most often in the winter
False; it is indeed more common in children but it occurs most commonly in summer and autumn.
107
Nephritic syndrome most commonly follows strep throat infection in the ______________ (northern/southern) US, and most commonly follows impetigo in the _______________ (northern/southern) US.
Northern; southern
108
Besides post-strep infection, what else can cause nephritic syndrome?
Staphylococci/gram-negative bacterial infections (sub-acute bacterial endocarditis, dental abscess, shutn infections); lupus
109
What is the treatment plan for nephritic syndrome? How many patients with this syndrome experience complete recovery?
Salt restriction, diuretics and other hypertensives. Most patients (90-95%) experience complete recovery.
110
True or false: nephritic syndrome commonly progresses to chronic renal failure
False.
111
What sort of structures are changed in membranoproliferative glomerularnephritis?
The basement membrane (membrano) has thick deposits on it (proliferative) in the glomerulus and the nephron (glomeruloneph); caused by immune damage (itis).
112
What is another name for membranoproliferative glomerulonephritis?
Dense deposit disease
113
What structures are damaged by the immune system in membranoproliferative glomerulonephritis?
Capillaries and supporting mesangium
114
There are three subtypes of membranoproliferative glomerulonephritis. Type II is associated with what kind of retinal finding?
Bilateral central clustered drusen
115
Type II membranoproliferative glomerulonephritis is often found in teens. Finding bilateral central clustered drusen in a teenager should make you think about this condition when trying to make a diagnosis.
Free card
116
Initially, the VA and VF of a patient with type II membranoproliferative glomerulonephritis are unchanged. What long term ocular complications may arise?
Poor night vision, subretinal neovascular membranes, macular detachment, central serous retinopathy, and retinal atrophy.
117
Tubulointerstitial nephropathy (TIN) is a group of inflammatory kidney diseases involving the interstitium and the tubules but sparing the glomeruli and renal vessels. If caused by a bacterial infection of the renal pelvis, what would that be called? What if the origin is noninfectious?
If caused by renal pelvis infection, = pyelonephritis. If noninfectious, interstitial nephritis.
118
TINU is an acute form of tubulointerstitial nephritis with uveitis. What age range and which gender have a higher prevalence of TINU?
10-33 years, female
119
The majority of patients with TINU present with what sign? What other symtpoms might these patients have?
Bilateral uveitis (conjunctival injection, fine keratitic precipitates, iridocyclitis); half of patients present with fever, weight loss, and fatigue
120
True or false: ocular signs of TINU often precede renal signs
True (in 35% of cases); this may be an important diagnostic tool in catching TINU
121
True or false: TINU rarely recurs.
False; it recurs in 50%
122
What would you use to treat TINU?
Oral corticosteroids (not topical, as you would normally use for uveitis)
123
Acute pyelonephritis is an inflammation of the kidney and renal pelvis. What is most commonly the cause for this condition? In which gender does it most often occur?
Caused by bacteria entering kidney via bladder and ureters; most common in women due to length an dlocation of urethra.
124
Acute pyelonephritis is more common in people with what condition?
Pregnancy (fetus pushes bladder into a more horizontal position so it's easier for bacteria to get in)
125
What signs/symptoms might a patient with acute pyelonephritis experience?
Fever, dysuria, flank/back pain, pyruia (pus in urine)
126
True or false: patients with acute pyelonephritis usually respond well to antibiotics
True.
127
Chronic pyelonephritis is slowly progressive. What underlying disorder commonly causes this condition?
UTI (urinary tract infection) from obstruction, vesicoureteric reflux (urine traveling retrograde) or diabetic nephropathy
128
True or false: the signs/symptoms of chronic pyelonephritis are quite specific, so it is usually diagnosed early on
False; the signs/symptoms are often vague ("don't feel well"), so diagnosis usually occurs late
129
Patients with chronic pyelonephritis usually end up on renal dialysis.
Free card
130
Which condition is the single most important cause of ESRD in the US?
Diabetic nephropathy
131
Good blood sugar control is critical in delaying diabetic nephropathy. Often, the first sign of diabetic nephropathy is microalbuminuria. What medications are used in diabetics to try and reduce the amount of protein in the urine? What stages of the disease occur once microalbuminuria has happened?
ACE inhibitors and ARBs are used to decrease protein in the urine. Once albumin gets into the urine, patient develops nephrotic syndrome and azotemia 3-5 years later, and ESRD 1-5 years after that.
132
What changes cause some of the pathology of diabetic nephropathy?
Glomerulus capillary BM thickening, renal atherosclerosis (affecting afferent and efferent arterioles), and pyelonephritis.
133
Fabry disease is an error of glycosphingolipid metabolism caused by a defect in which enzyme?
Alpha-galactosidase A
134
What type of inheritance pattern causes Fabry disease?
X-linked recessive, so more common in males
135
True or false: carriers of Fabry disease never show manifestations of the disease
False; carriers can show some manifestations
136
The signs/symptoms of Fabry disease are variable, making it difficult to diagnose. What are some of these signs/symptoms? Which sign is often the first sign?
Burning sensation in hands, raised rash on butt/groin/thighs, decreased ability to sweat. Most common first sign is corneal whorl (verticillata), which is present in over 90% of males with this condition.
137
Besides verticillata, what other ocular signs can be seen in Fabry disease?
Conjunctival vessel tortuosity in inferior conjunctiva; retinal venous dilation with possible hemorrhages; cataracts (often spoke-like)
138
What systemic issues could a patient with Fabry disease have?
Renal failure due to glycolipid deposition in renal vessels (evidenced by proteinuria, azotemia, uremia); cardiovascular or cerebrovascular disease, GI dysfunction
139
By what age do half of patients with Fabry disease suffer from renal failure? By what age do virtually all Fabry disease patients suffer from renal failure?
Age 35; age 55
140
What testing can be done in the diagnosis of Fabry disease?
Enzyme assay to measure amount of alpha-GAL activity; genetic testing for mutation
141
What is the treatment plan for patients with Fabry disease?
IV agalsidase beta given for four hours every two weeks (for the rest of patient's life); this protects kidney function from the lipid deposits. This can change life expectancy of these patients.
142
Nephrolithiasis/urolithiasis is formation of a stone in the urine collecting system, usually in the ureter. This condition usually occurs when a patient becomes deheydrated. What conditions or medications might cause formation of stones?
Gout and acetazolamide were the things mentioned in class as making stone formation more likely.
143
What type of substance makes up most kidney stones? Second most common?
Calcium; magnesium
144
True or false: nephrolithiasis occurs in the 20-45 age range but only occurs with symptoms in 1% of people
False; it does occur in this age range (20-45) but occurs with symptoms in 5% of people (1 in 20).
145
Symptomatic nephrolithiasis is more common in which gender?
Male, due to length of the urethra
146
Nephrolithiasis can cause what substance to appear in the urine? This is similar to which other condition mentioned in class? What fact about this feature in nephrolithiasis that might help differentiate it from this other condition?
Blood (hematuria), which is also found in nephritic syndrome. Hematuria of nephrolithiasis is accompanied by pain; in nephritic syndrome, it is usually painless.
147
How would you diagnosis nephrolithiasis?
With a CAT scan.
148
90% of kidney stones pass spontaneously. What can be done to help ease the passage?
Pain relief meds, alpha adrenergic blockers and calcium channel blockers to relax smooth muscle of ureter, antibiotics.
149
If a stone is too large to pass, what other treatments may be tried?
Shockwave lithotripsy (shockwaves to break up the stone into smaller stones, which all then have to pass); failing that, surgery.
150
Renal cell carcinoma makes up what percentage of all malignant kidney tumors? What percentage of all adult cancers?
80-90% of renal tumors; 2% of all adult cancers
151
True or false: renal cell carcinoma is less common among cigarette smokers than non cigarette smokers?
False; it is more common
152
Which gender has a higher prevalence of renal cell carcinoma?
Male
153
Renal cell carcinoma is associated with what disease?
Von Hippel-Lindau disease
154
What is the most common presenting manifestation of renal cell carcinoma? What other condition discussed in class mentions this manifestation?
Painless hematuria; nephritic syndrome also features painless hematuria
155
There is a "classic triad" of signs/symptoms of renal cell carcinoma, though it is not common for a patient to have all three. What are these three signs/symptoms? What other symptoms may occur?
Hematuria (painless), dull flank pain (as opposed to the sharp flank pain of nephrolithiasis), palpable flank mass. Other symptoms inlcude a long-standing fever, elevated ESR (due to increased erythropoietin from tumor), weight loss and fatigue (classic cancer signs)
156
Which portion of the nephron does renal cell carcinoma develop from? Is this condition vascular?
It develops from the proxiimal tubules and is highly vascular.
157
What are the treatment options for renal cell carcinoma?
Surgical removal of kidney, percutaneous cryoablation, anti-VEGF, anti-PDGFR
158
True or false: Wilms' tumor is the second most common kidney malignancy and the third most common organ cancer in children
True.
159
Wilms' tumor has a high incidence in children with what ocular condition?
Aniridia
160
What are the treatment options for Wilms' tumor?
Radiotherapy, nephrectomy, chemotherapy
161
True or false: if a patient with Wilms' tumor survives for at least two years, they usually have a normal lifespan
True.
162
What are a few categories of antihypertensive drugs?
Diuretics (increase water excretion, relax smooth muscle), beta-blockers (decrease HR and renin release), calcium channel blockers (relax smooth muscle), ACE inhibitors, ARBs, and renin inhibitors for inhibiting the renin system
163
Long-term use of diuretics is more useful in treatment HTN in a patient with chronic renal disease than one with normal kidney function. Why is this?
In healthy kidneys, the renal system eventually balances out and returns blood volume to normal, countering the effect of the diuretic. In renal failure, this feedback mechanism is missing, allowing for long-term use of diuretics in HTN treatment.
164
Which classes of diuretics are commonly used in treatment of kidney, heart, and high BP?
Thiazides, loop diuretics, potassium-sparing diuretics
165
Which class of diuretic is commonly used in treatment of glaucoma and disc edema?
Carbonic anhydrase inhibitor (acetazolamide
166
Which classes of diuretics are commonly used in treatment of glaucoma?
Osmotic diuretics (mannitol, urea, isosorbide)
167
Renal replacement therapy is a general term that includes dialysis and kidney transplant. What GFR might indicate that renal replacement therapy should be initiated?
15 ml/min for diabetics, 10 ml/min for nondiabetics
168
It has been suggested that dialysis may increase a patient's risk of death from heart attack due to ion imbalance. What are a few criteria (other than GFR) that may possibly be used to judge when dialysis should be used?
Pericarditis*, neuropathy*, encephalopathy, heart failure, acidosis, loss of weight, persistant hyperkalemia, edema not responsive to diuretics
169
What are the three most common replacement therapies in the US?
Hemodialysis, peritoneal dialysis, renal transplantation
170
Briefly describe how hemodialysis works.
Blood is circulated into artificial kidney machine, in an opposite direction to another fluid (dialysate). These two fluids are separated by a semipermeable membrane which allows diffusion of ions and low molecular weight substances into the dialysate. Blodo then circulates back into the body.
171
What are some drawbacks of hemodialysis? Some risks?
3.5 hours, 3x a week; creatinine is not well cleared; diet need to be modified due to protein effect of dialysis. Risks include anemia, pulmonary edema, ionic dysfunction, infection, heart dysfunction, high mortality rate.
172
True or false: patients on dialysis experience less visual impairment than is expected for their age.
False; they experience more visual impairment than is expected for their age.
173
Briefly describe how peritoneal dialysis works
Catheter placed in peritoneal cavity allows installation of dialysate into cavity. Peritoneum acts as semipermeable membrane to allow ions/waste to be pulled into dialysate. The dialysate is removed after a period of time and more is instilled.
174
What are some advantages of peritoneal dialysis over hemodialysis?
Takes less time, requires less dietary restrictions, filtration is more consistent, residual kidney function is retained longer, is more cost efficient.
175
What are some disadvantages of peritoneal dialysis over hemodialysis?
More complex in terms of patient's responsibilities; possibility of peritonitis (easier route of infection)
176
Corticosteroids (prednisone) used to be used as immunosuppressants in kidney transplants. Now, what classes of drugs are used? What do these classes do?
Calcineurin inhibitors (decrease IL-2 production by T-cells) and anti-metabolites (decrease lmphocyte proliferation and activation)
177
What specific drugs (not corticosteroids) are used as immunosuppressants for renal transplantation? Which pair of these drugs is the most common?
Cyclosporine or tacrolimus (calcineurin inhibitors) and azathioprine and mycophenolate (anti-metabolites); tacrolimus and mycophenolate is the most common combination
178
What are some ocular effects of corticosteroid use?
Cataracts, risk of glaucoma, papilledema
179
What are a few complications that may arise from renal transplantation?
Immunosuppressive disorders, secondary HTN (prednisone and/or native renin production), infection (UTI, hepatitis, pneumonia), graft rejection, malignancy
180
Malignancy is one of the possible complications of renal transplantation. Which specific types of cancer were mentioned in class?
Skin cancer, Kaposi's sarcoma (treat with Rapamune), ocular squamous cell carcinoma
181
Which medications should be avoided if possible in a patient with renal compromise?
Anything toxic to kidney; aminoglycosides and tetracyclines (except doxycycline) specifically mentioned