the second week on the kidney: beginning with tubular disorders. the normal function of the renal tubules is reabsorption of various electrolytes, thus many tubular disorders result in excess amounts of these materials in the urine. fanconi's syndrome is a congenital or acquired syndrome that is often fatal by the 20's that leads to dysfunction of the proximal tubule, resulting in cystinosis, glucosuria, phosphaturia, aminoaciduria, bicarbonate wasting. cystinuria and aminoaciduria are failed reabsorption of cysteine or amino acids in general, leading to excess excretion in the urine. in both, cystine crystals might form and create symptoms similar to that of a patient with renal colic. wilson's disease is a rare congenital disease that involves copper deposition in the tubules, along with the liver, brain, and RBC's. the pathognomonic sign for wilson's disease is the "kayser fleischer" rings seen in the eye.
renal glucosuria is a tubular dysfunction that results in spilling of glucose into the urine despite normal or low blood glucose levels. it is often asymptomatic and might be found by glucose in the urine after a 24 hour fast. renal acidosis is another condition that involves acidosis which might be due to impaired secretion of H+ in the distal tubule or impaired reabsorption of bicarbonate in the proximal tubule. the latter might be associated with potassium wasting and muscle dysfunction as well. potassium absorption can also be affected by tubular dysfunction, as seen in bartter's and liddle's syndrome. nephrogenic diabetes insipidus is a congenital and generally fatal disease that might show up in infants in which the kidney's ADH receptors are dysfunctional, resulting in polydipsia, polyuria and hypotonic urine.
diabetic nephropathy is a complication that occurs in about 1/3 of cases of diabetes mellitus which involves glycosylation of the nephron and the dysfunction that results. it might occur 15-20 years after a diagnosis of DM and might be heavily influenced (or prevented) by diet and lifestyle factors early on. the first signs are not overt and may include increased GFR and mild proteinuria. patients with stage II DN will see a drop in GFR, more noticeable proteinuria (resulting in edema symptoms as well), and increasing hypertension. stage III is the last stage, the last few years of a ~20 year process which involves greatly reduced GFR, massive proteinuria, hypertension, and retinopathy. patients with severe DN are at increased risk for infections as well due to the higher glucose levels in the urine.
polycystic kidney disease is a hereditary disorder that results in multiple bilateral cysts that increase size and decrease function of the kidneys. it can manifest as proteinuria, mild hematuria, low back pain, infection, and colic due to formation of stones. late stage, it might also be accompanied by hepatic involvement such as portal HTN and esophageal varices. it will show proteinuria and hematuria on lab diagnosis, as well as pyuria which might appear even without infection. 50% of patients with PKD will end up with renal failure in 10 years.
interstitial tubular disorder is another possible tubular dysfunction that occurs due to drug toxicity or hypersensitivity- most commonly from seizure medication. these drugs can affect both the tubules and the interstitial space, causing a variety of symptoms including that of tubular dysfunction, renal failure, UTI, as well as a host of dermatological manifestations depending on the causative agent. labs might resemble an infection without the presence of bacteria: eosinophilia, proteinuria, hematuria. a patient with ITD would present clinically with enlarged kidneys.
cystitis is a UTI of the bladder, commonly due to vaginal uptake in women and chronic bacterial prostatitis in men. it manifests mostly through urinary symptoms: frequency, urgency, dysuria, occasional hematuria, and possibly suprapubic or lower back pain. there are generally no remarkable PE findings other than general achiness. a UA might show turbid urine and possibly hematuria.
if acute infection reaches the kidneys, acute pyelonephritis results. this condition is more common in girls / pregnant women or patients who have urethral instrumentation. it is most commonly due to e.coli, klebsiella, enterobacter and proteus, which may be present in a urine culture in excess of 100,000 / ml. symptoms include fever/chills/nausea/vomiting, as well as kidney tenderness and enlargement, and possible urinary symptoms as well. lab tests might show neutrophilia, bacteruria, and presence of WBC casts is a pathognomonic sign.
untreated or poorly managed acute pyelonephritis might lead to chronic pyelonephritis, which in turn may lead to chronic renal failure if fibrosis is widespread enough. these patients have a vague and inconsistent clinical presentation, sometimes having fever, abdominal/flank pain, and obstructive symptoms. a PE for a patient with CP should include checking for HTN and anemia symptoms, as well as edema, CVA tenderness, and palpation of the kidney and bladder. definitive diagnosis is made by IVU.
interstitial cystitis is a disorder of the bladder lining, related to dysfunction of the GAG layer. rather than an infectious origin, IC is commonly triggered by such foods as cranberry juice, potassium rich foods, coffee, alcohol, tomatoes, etc. it presents as a more severe cystitis, with urgency, frequency, and severe abdominal pain which can be severe enough to limit sexual activity. diagnosis is made by excluding other bladder conditions and can be made definitively by bladder wall biopsy, which would show hunnel's patches: tiny ulcerations.
a urinary calculi is a stone in the ureter, commonly made of calcium oxalate, struvite, uric acid, or cystine. they appear more frequently in men and are often idiopathic, although several risk factors have been identified- such as low water intake, excess vitamin C intake, and a sedentary lifestyle. people with UC will present with episodes of severe, colicky flank pain that may radiate down to the thigh, as well as hematuria and urinary symptoms. a UA might show hematuria, pyuria, and crystals-- a 6 sided crystal points to a stone made of cystine.
some notes on cancers of the kidney and bladder: most kidney tumors are adenocarcinomas, which are commonly caused by drugs and environmental toxins and might present asymptomatically due to the high functional reserve of the kidneys. wilm's tumor mostly affects children and may present as an asymptomatic mass. neuroblastoma is a systemic, often fatal condition which affects the kidney as well and can be identified by blackness around the eyes. bladder tumors are most often caused by cigarette smoking, and can cause hematuria, secondary infection, flank pain with obstructive symptoms. the conventional treatment strategy is sloughing off of cancer cells via instillation of BCG within the bladder.
questions
various tubular disorders...
1. what is fanconi's syndrome?
2. what is the prognosis for patients with fanconi's syndrome?
3. what is cystinuria?
4. patients with cystinuria can present similarly to which condition?
5. what is aminoaciduria?
6. cystine renal stones in a patient with aminoaciduria might indicate...
7. how is aminoaciduria diagnosed?
8. what is wilson's disease?
9. wilson's disease might present similarly to...
10. what is the pathognomonic sign for wilson's disease?
11. what is the prognosis for patients with wilson's disease?
12. if diagnosed early, what might be of benefit to patients with wilson's disease?
more tubular disorders...
13. what is renal glucosuria?
14. how is renal glucosuria diagnosed?
15. what is renal acidosis?
16. renal acidosis from proximal tubule dysfunction often accompanies which disease?
17. distal tubule dysfunction is often accompanied by...
18. what is bartter's syndrome?
19. what is liddle's syndrome?
20. what is nephrogenic diabetes insipidus?
21. what are the signs and symptoms of nephrogenic diabetes insipidus?
diabetic nephropathy...
22. what is diabetic nephropathy?
23. describe the hallmarks of the first stage of diabetic nephropathy. how long after DM diagnosis is the onset?
24. what are the hallmarks of the second stage of diabetic nephropathy?
25. what are the hallmarks of the third stage of diabetic nephropathy?
26. patients with diabetic nephropathy are more prone to...
27. what is the treatment strategy for patients in stage I DN?
28. what is the survival rate for stage II or III DN patients who need dialysis?
polycystic kidney disease...
29. what is polycystic kidney disease?
30. what are the signs and symptoms of PKD?
31. what are some symptoms that might appear late in the course of PKD?
32. what are the lab results for PKD?
33. what is the prognosis for PKD?
interstitial tubular disease...
34. what is ITD?
35. what are medications that commonly trigger ITD?
36. what are the signs and symptoms of ITD?
37. what are some typical lab results for a patient with ITD?
38. what is a PE finding one might find on a patient with ITD?
renal / urinary tract infections...
39. what are some general symptoms of a lower UTI?
40. what are some general symptoms of an upper UTI?
41. blood borne infections are more likely seen in which demographic?
42. what are some physiological mechanisms that fight against infections in the urinary tract?
43. what are some factors that predispose an individual to getting a UTI?
cystitis...
44. what is a common etiology of cystitis for females?
45. what is the most common etiology of cystitis for males?
46. what are the typical cystitis PE findings?
47. what are the signs and symptoms of cystitis?
48. what are some lab findings for cystitis?
acute pyelonephritis...
49. which demographic is AP most common in?
50. which microorganisms are most commonly implicated in AP?
51. what are the signs and symptoms of AP?
52. what are the lab markers one might see with an AP patient?
53. a urine culture might show...
chronic pyelonephritis...
54. CP accounts for what percentage of renal failure cases?
55. what is CP caused by?
56. how does CP lead to renal failure?
57. what is the symptom picture for CP?
58. CP symptoms might appear similarly to what other condition?
59. what should be checked for on a PE of a patient with CP?
60. definititve diagnosis of CP is made by...
61. what are some strategies for improving kidney health?
interstitial cystitis...
62. what gender is more affected by IC?
63. what is the pathophysiology of IC?
64. what are some common foods that irritate IC?
65. how does IC present clinically?
66. 60% of patients with IC experience...
67. how long does IC generally take to diagnose?
68. what are the diagnostic criteria for IC?
69. what is the pathognomonic sign for IC?
70. what is unique about the UA for patients with IC?
urinary calculi...
71. which gender is more prone to UC?
72. 75% of urinary calculi are due to what etiology?
73. 20% of urinary calculi are due to...
74. what are the most common types of stones found in UC?
75. what are the signs and symptoms of UC?
76. what might be found on a urinalysis of UC?
77. 6 sided crystals in a UA of UC is indicative of...
78. what are some risk factors for UC?
cancer...
79. what percentage of kidney tumors is represented by adenocarcinomas?
80. what are some etiological agents in adenocarcinoma of the kidneys?
81. what are the signs / symptoms of adenocarcinoma?
82. wilm's tumor is most common in which demographic?
83. wilm's tumor most commonly presents as...
84. what is a characteristic sign for neuroblastoma?
85. what is a common etiology for bladder cancer?
86. what are the signs and symptoms of bladder cancer?
answers
1. congenital or acquired dysfunction of proximal tubule that results in cystinosis, glucosuria, phosphaturia, aminoaciduria, and bicarbonate wasting.
2. most die in 20's.
3. impaired reabsorption of cysteine in the tubules which causes increased urinary excretion and formation of cystine calculi.
4. renal colic.
5. impaired absorption of all amino acids.
6. chronic renal failure.
7. excess amino acids and cystine crystals on UA.
8. a rare congenital disorder which involves copper deposition in various places in the body such as the renal tubules, liver, brain, RBC's.
9. hepatitis.
10. kayser fleischer rings.
11. generally poor due to late diagnosis.
12. treatment with zinc.
13. inherited condition that involves low blood glucose combined with excess excretion of glucose.
14. normal or low serum glucose but glucose in urine after overnight fast.
15. chronic acidosis which results either from impaired resorption of bicarbonate in proximal tubule or impaired secretion of H+ in distal tubule.
16. fanconi's syndrome, wilson's disease, multiple myeloma, vitamin D deficiency, heavy metal toxicity.
17. potassium wasting and the resulting muscle dysfunction.
18. hypokalemia due to dysfunction in thick ascending loop of henle.
19. rare disorder that resembles primary aldosteronism with hypertension and hyperkalemic alkalosis.
20. dysfunction of ADH receptors on the kidney.
21. polydipsia, polyuria, hypotonic urine.
22. a complication that occurs in about a third of cases of diabetes mellitus resulting from glycosylation of the nephron.
23. may appear 15-20 years after DM diagnosis; undetectable increase in GFR, mild proteinuria.
24. decreased GFR
proteinuria
edema due to low protein
hypertension.
25. azotemia (increases seen in BUN and creatinine)
GFR less than 1/3
massive proteinuria
hypertension
retinopathy
26. infections due to higher glucose levels in urine, neurogenic bladder, hypertension.
27. watch diet: low sugars, high vegetables
monitor blood pressure
watch for retinal fundus changes
28. 2-3 years.
29. an autosomal dominant or recessive disease that results in multiple bilateral cysts that increase the size and reduce the function of the kidneys.
30. asymptomatic, or
lumbar pain
hematuria
infection
colic due to stones [back blood bugs stones] [bloody stones on the back of bugs]
31. hepatic symptoms: portal HTN, esophageal varices.
32. proteinuria, occasional hematuria, pyruria (even without infection)
33. 50% chance for kidney failure in 10 years.
34. acute renal failure due to drug reaction / hypersensitivity that affects tubules and interstitial tissue.
35. seizure medications.
36. variable:
UTI sxs
tubular dysfunction sxs
oliguria
urticaria, photosensitivity, erythema nodosum, epidermal necrolysis
37. eosinophilia, proteinuria, hematuria, but no bacteria.
38. enlarged kidneys.
39. more urinary symptoms: dysuria, frequency, urgency, hematuria, etc. suprapubic pain.
40. kidney tenderness, fever/chills, N/V, sometimes diarrhea.
41. IV drug users.
42. immune response via WBC's and antibodies, urine acidity, and voiding.
43. congenital malformations (hypospadias)
poor or ineffective hygiene
frequent intercourse, anal sex
high urine pH
catheterization
low water intake
high carb diet / diabetes
44. uptake of bacteria from vagina.
45. chronic bacterial prostatitis.
46. patient in general distress and malaise, but not as severe as pyelonephritis.
47. dysuria, urgency, frequency, nocturia
suprapubic and lower back pain
48. turbid urine and occasional hematuria.
49. girls / pregnant women, or patients with catheterization.
50. e.coli, klebsiella, proteus, enterobacter.
51. fever/chills, N/V
flank pain, kidney tenderness and enlargement
urinary frequency / urgency in 1/3.
52. elevated neutrophils, bacteria, WBC casts in the urine.
53. more than 100,000 organisms / ml.
54. 2-3%.
55. recurrent acute pyelonephritis.
56. CP leads to fibrosis which decreases renal function, ultimately leading to chronic renal failure.
57. vague, inconsistent. may have
fever
flank / abdominal pain
obstructive symptoms
58. chronic interstitial nephritis.
59. signs of HTN, anemia, edema, CVA tenderness, palpation of kidneys and bladder.
60. IVU.
61. drink more water (up to half your weight??)
regulate protein intake
reduce unnecessary medications
62. females 10:1.
63. disruption of interior lining of the bladder, related to dysregulation of GAG layer. may also be abnormal accumulation of mast cells.
64. cranberry
coffee / tea
alcohol / tobacco
tomatoes
potassium
65. as a more severe cystitis:
frequency, urgency (enough to keep housebound) and severe abdominal pain
66. pain with intercourse.
67. 4.5 years on avg.
68. exclusion of other bladder conditions
irritative voiding
specific cystography changes or
aggravation from potassium
69. hunner's patches: tiny ulcerations seen in bladder wall biopsy.
70. consistently free of bacteria, unlike many other bladder conditions; not infective.
71. 3-4 times more common in males.
72. idiopathic.
73. uric acid abnormalities.
74. calcium oxalate, struvite, uric acid, cystine.
75. hematuria
episodes of severe, spastic, colicky pain that may radiate from flank to thigh
urinary symptoms: urgency, frequency, dysuria.
76. may have hematuria, pyuria, crystals.
77. cystine crystals.
78. low fluid intake
sedentary lifestyle
excess vitamin C
low vitamin B6, mg
79. 86%.
80. drugs
heavy metals
radiation
viruses
lifestyle factors: smoking, coffee, etc.
81. often asx because of high functional reserve of kidneys, but may present with colic, hematuria.
82. children.
83. asymptomatic mass.
84. black under the eyes.
85. smoking.
86. hematuria
infection
obstructive symptoms, flank pain
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