Chapter 12 ( Kideny And Urinary Tract ) Flashcards

1
Q

The most common renal anomaly ?

A

Horseshoe kidney

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2
Q

Kidneys site in horseshoe kidney ? Why ?

A

In the lower abdomen
As the horseshoe kidney gets caught on the Inferior mesenteric artery root during its ascent from the pelvis to the abdomen

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3
Q

Unilateral renal agenesis results in ?

A

Hypertrophy of the existing kidney , hyperfiltration increases risk for renal failure later in life

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4
Q

Bilateral renal agenesis results in ?

A

Oligohydraminos
Potter Sequence
Incompatible with life

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5
Q

Potter sequence ?

A
Developmental events occurs in Oligohyraminos due to absence of amniotic fluid bubble and pressure against wall of the uterus , consists of :
Lung hypoplasia
Flat face 
Low set ears 
Developmental defects of the extremities
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6
Q

Dysplastic kidney defect ?

A

Noninherited congenital malformation of the renal parenchyma characterized by cysts and abnormal tissue

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7
Q

Mechanism of polycystic kidney ?

A

Inherited defect leading to bilateral enlarged kidneys with cysts in the RENAL CORTEX and MEDULA

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8
Q

Autosomal recessive form of PKD presents in who ? Presentation ? Associations ?

A

Infants

Presents as worsening renal failure and hypertension , newborns may present with Potter sequence

Associated with : congenital hepatic fibrosis that leads to portal hypertension , hepatic cysts

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9
Q

Autosomal dominant form of PKD presents in who ? Defect ? Presentation ? Associations ?

A

Young adults

Mutation in APKD1 or APKD2 gene

Presents as hypertension ( due to increased renin ) , hematuria and worsening renal failure

Associated with :
Berry aneurysm
Hepatic cysts
Mitral valve prolapse

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10
Q

Medullary cystic kidney disease defect ? Presentation ?

A

Inherited autosomal dominant defect leading to cysts in the medullary collecting ducts

Presents as shrunken kidney and worsening renal failure ( due to Parenchymal fibrosis )

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11
Q

Azotemia ?

A

Increased serum BUN and Creatinine

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12
Q

Prerenal azotemia lab findings ?

A

Serum BUN : Cr ratio > 15 ( because of increased reabsorption of fluid and BUN )
FENa < 1%
Urine osmolality > 500 mOsm/kg

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13
Q

Early stage of postrenal azotemia lab findings ?

A

Serum BUN:Cr ratio > 15

Normal tubular function

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14
Q

Long standing postrenal azotemia lab findings ?

A

Serum BUN:Cr ratio < 15
FENa > 2%
Urine osm < 500 mOsm/kg

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15
Q

Most common cause of acute renal failure ?

A

Acute tubular necrosis

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16
Q

Mechanism of acute tubular necrosis in causing acute renal failure ?

A

1- necrotic cells plug tubules —> obstruction —> ⬇️ GFR
2- dysfunctional tubular epithelium results in :
Decreased reabsorption of BUN ( serum BUN:Cr ratio < 15 )
Decreased reabsorption of Na ( FENa > 2% )
Inability to concentrate urine ( urine osm < 500 mOsm/kg )

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17
Q

Urine casts in acute tubular necrosis ?

A

Brown granular casts

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18
Q

Which parts of renal tubule are particularly susceptible to ischemic damage ?

A

Proximal tubule

Medullary segment of thick ascending limb

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19
Q

Which part of renal tubule is particularly susceptible to toxic agents ?

A

Proximal tubule

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20
Q

Toxic agents that cause nephrotoxic ATN ?

A
1- Aminoglycosides ( most common ) 
2- Heavy metals as lead 
3- Myoglobinuria 
4- Ethylene glycol ( associated with OXALATE CRYSTALS in urine )
5- Radiocontrast dye 
6- Urate ( as in tumor lysis syndrome )
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21
Q

How to decrease risk of urate induced ATN with chemotherapy ?

A

Hydration and Allopurinol before initiation of chemotherapy

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22
Q

Clinical features of ATN ?

A

Oliguria with brown granular casts
Elevated BUN and Cr
Hyperkalemia with metabolic acidosis

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23
Q

Why oliguria can persists foe 2-3 weeks before recovery from ATN ?

A

As tubular cells ( stable cells ) take time to reenter the cell cycle and regenerate

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24
Q

Acute interstitial nephritis mechanism ? Causes ? Presentation ?

A

Drug induced hypersensitivity involving the interstitium and tubules

Causes :
NSADs
Penicillin
Diuretics

Presents as : oliguria , fever and rash days to weeks after stating drug , eosinophilia may be seen in urine

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25
Renal papillary necrosis causes ? Presentation ?
``` Causes : 1- Chronic analgesic abuse 2- DM 3- Sickle cell trait or disease 4- Sever acute pyelonephritis ``` Presents as Gross hematuria and Flank pain
26
Nephrotic syndrome ?
Proteinuria > 3.5 gm/day
27
Most common cause of nephrotic syndrome in children ?
Minimal change disease
28
MCD is associated with ? On H and E stain , EM and IF ?
Hodgkin lymphoma On H and E : normal glomeruli , lipid may be seen in proximal tubule cells On EM : effacement of foot processes On IF : negative
29
Why there is excellent response to steroids in MCD ?
As the damage is mediated by cytokines from T cells
30
Most common couse of nephrotic syndrome in Hispanics and African Americans ?
Focal segmental glomerulosclerosis
31
FSG is associated with ? On H and E stain , EM and IF ?
HIV , Heroin use , sickle cell disease On H and E stain : focal and segmental sclerosis On EM : effacement of foot processes On IF : negative
32
Most common cause of nephrotic syndrome in Caucasian adults ?
Membranous nephropathy
33
Membranous nephropathy is associated with ? On H and E stain , EM and IF ?
Hepatitis B or C , solid tumors , SLE , Drugs ( as NSADs and penicillamine ) On H and E : thick glomerular basement membrane On EM : subepithelial deposits with Spike and Dome appearance On IF : Granular ( due to immune complex deposition )
34
Membranooproliferative glomerulonephritis On H and E stain , EM and IF ? Types ?
On H and E : thick glomerular basement membrane with tram track appearance On IF : Granular Types : 1- Type l subendothelial deposits on EM , associated with HBV , HCV 2- Type ll intramembranous deposits on EM , associated with C3 nephritic factor
35
C3 nephritic factor ?
Autoantibody that stabilizes C3 convertase leading to overactivation of complement , inflammation and low levels of circulating C3
36
Mechanism of nephrotic syndrome in DM ?
Nonenzymatic glycosylation of the vascular basement membrane resulting in hyaline arteriolosclerosis which affects glomerular efferent arteriole more than afferent arteriole —> high glomerular filtration pressure —> hyperfiltration injury —> microalbuminuria which eventually progress to nephrotic syndrome
37
Diabetic nephropathy characterized by what on H and E ?
Sclerosis of mesangium and formation of Kimmelstiel-Wilson nodules
38
Which drug slows progression of hyperfiltration induced damage in kidney ?
ACE inhibitors
39
Characteristics of nephritic syndrome ? Biopsy findings ?
Glomerular inflammation and bleeding : 1- limited proteinuria < 3.5 mg/day 2- oliguria and azotemia 3- salt retention with periorbital edema and hypertension 4- RBC casts and dysmorphic RBCs in urine Biopsy reveals hypercellular inflamed glomeruli ( due to immune complex deposition which activates complement , C5a attract neutrophils which mediate damage
40
PSGN On H and E stain , EM and IF ?
On H and E : hypercellular inflamed glomeruli On IF : Granular On EM : subepithelial humps
41
Consequence of PSNG in children and adults ?
In children : 1% progress to renal failure | In adults : 25% develops rapidly progressive glomerulonephritis
42
Rapidly progressive glomerulonephritis consequence ? On H and E ?
Progress to renal failure in weeks to months | On H and E : crescents in bowman capsule , crescents are comprised of fibrin and macrophages
43
Goodpasture syndrome RPGN IF ? Presentation ?
On IF : Linear ( due to antibody against collagen in glomerular and alveolar basement membrane ) Presentation : Hematuria and Hemoptysis in young adult males
44
Diffuse proliferative glomerulonephritis on IF ? Mechanism ? Most common in ?
Granular ( due to diffuse antigen antibody complex deposition usually subendothelial Most common type os renal disease in SLE
45
Wegener granulomatosis RPGN on IF ? Associated with ? Presentation ?
Negative IF Associated with c-ANCA Presents with nasopharyngeal symptoms as Sinusitis
46
Churge strausse syndrome on IF ? Associated with ?
Negative IF Associated with p-ANCA , granulomatous inflammation , eosinophilia and asthma are ( all of which distinguish it from microscopic polyangiitis )
47
Most common nephropathy worldwide ?
IgA nephropathy ( Berger disease )
48
IgA nephropathy on IF ? Presentation ?
On IF : IgA immune complex deposition is seen in the mesangium Presents during childhood as episodic gross or microscopic hematuria with RBC casts usually following mucosal infections as Gastroenteritis
49
Alport syndrome defect ? Mechanism ? Presentation ?
Most commonly X linked , inherited defect in type lV collagen Results in thinning and splitting of the glomerular basement membrane Presents as : Isolated hematuria Sensory hearing loss Ocular disturbances
50
Systemic signs in cystitis ?
Absent
51
Lab findings of cystitis ?
1- urinalysis : cloudy urine with > 10 WBCs/hpf 2- Dipstick - positive leukocyte esterase (due to pyuria) and nitrites ( bacteria converts nitrates to nitrites ) 3- culture : > 100,000 colony forming units
52
Etiology of cystitis ?
ESKPE 1- E coli 80% 2- Staphylococcus saprophyticus ( increased incidence in young sexually active females ) 3- Klebsiella pneumoniae 4- Proteus mirablilis : alkaline urine with ammonia scent 5- Enterococcus faecalis
53
Sterile pyuria ? Cause ?
Pyuria ( > 10 WBCs/hpf and leucocyte esterase ) with a negative urine culture Cause : Urethritis due to Chlamydia trachomatis or Neisseria gonorrhoeae
54
Pyelonephritis cause ? Presentation ?
Most common pathogens : E coli 90% Enterococcus faecalis Klebsiella species
55
Chronic pyelonephritis mechanism ? Microscopic findings ?
Interstitial fibrosis and atrophy of tubules due to multiple bouts of acute pyelonephritis due to vesicoureteral reflux or obstruction Leads to cortical scarring with blunted calyces Atrophic tubules containing eosinophilic proteinaceous material resemble thyroid follicles , waxy casts may be seen in urine
56
Scarring at upper and lower poles of kidney is characteristic of what ?
Chronic pyelonephritis due to vesicoureteral reflux
57
Calcium stones causes and ttt ?
Causes : Idiopathic hypercalciuria Hypercalcemia and its causes Crohn disease Ttt : Hydrochlorothiazide
58
Ammonium , magnesium , phosphate stone causes ? Ttt ?
Cause is infection with Urease positive organisms : Proteus vulgaris Klebsiella in which alkaline urine leads to formation of stone Ttt : classically it forms staghorn calculi in renal calyces which is surgically removed with eradication of pathogen
59
Uric acid stone causes ? Ttt ?
``` Causes ( risk factors ) : Hot arid climates Low urine volume Acidic pH of urine Its tge most common stone seen in patients with gout ( in leukemia or myeloproliferative disorders ) ``` Ttt : Hydration Alkalization of urine Allopurinol in patients with gout
60
The single type of renal stones that is radiolucent ?
Uric acid stone
61
Cysteine stone causes ? Ttt ?
Causes : cystinuria Ttt : hydration and alkalization of urine
62
Clinical features of chronic renal failure ?
``` 1- Uremia 2- Salt and water retention with hypertension 3- Hyperkalemia and metabolic acidosis 4- Anemia 5- Hypocalcemia 6- Renal osteodystrophy ```
63
Clinical features of uremia ?
``` Nausea and Anorexia Pericarditis Platelet dysfunction Encephalopathy with Asterixis Deposition of urea crystals in skin ```
64
Which cells produce erythropoietin in kidney ?
Renal peritubular interstitial cells
65
Complications of dialysis ?
Cysts often develop within shrunken end stage kidneys during dialysis that increase risk for renal cell carcinoma
66
Angiomyolipoma increased frequency in ?
Tuberous sclerosis
67
Renal cell carcinoma mechanism ? Presentation ? Gross ? Micro ?
Malignant epithelial tumor arising from kidney tubules Presentation : Classic triad of hematuria , palpable mass and flank pain ( rarely occur together , hematuria is the most common presenting symptom ) Fever , weight loss or paraneoplastic syndrome ( EPO , renin , PTHrP or ACTH ) Rarely may present with left sided varicocele Gross : a yellow mass Micro : the most common variant is Clear cytoplasm
68
Renal cell carcinoma pathogenesis ?
Loss of VHL (3p) tumor suppressor gene which leads to increased IGF-1 and increased HIF transcription factor ( increases VEGF and PDGF )
69
Sporadic renal cell carcinoma presentation ? Major risk factor ?
``` Adult male ( average 60 y ) as a single tumor in the upper pole of the kidney Major risk factor : cigarette smocking ```
70
Hereditary renal cell carcinoma presentation ?
In young adults and are often bilateral
71
Von Hippel-Lindau disease defect ? Increase risk for ?
Autosomal dominant associated with inactivation of VHL gene Leads to increased risk for : Hemangiblastoma of the cerebellum Renal cell carcinoma
72
Most common malignant renal tumor in children ?
Wilms tumor
73
Wilms tumor components ? Presentation ?
Blastema Primitive glomeruli and tubules Stromal cells Presents as : Large unilateral flank mass Hematuria Hypertension
74
WAGR syndrome component ? Defect ?
Defect : deletion of WT1 tumor suppressor gene ( located at 11p13 ) ``` Components : Wilms tumor Aniridia Genital abnormalities mental and motor Retardation ```
75
Denys-Drash syndrome defect ? Components ?
Associated with WT1 mutation Components : Wilms tumor Progressive renal disease Male pseudohermaphroditism
76
Beckwith-Wiedmann syndrome defect ? Components ?
Associated with mutations in WT2 gene cluster ( imprinted genes at 11p15.5 ) particularly IGF-1 ``` Components : Wilms tumor Neonatal hypoglycemia Muscular hemihypertrophy Organomegaly ```
77
Urothellial carcinoma risk factors ? Presentation ? Mechanism ?
Risk factors : Cigarette smocking ( polycyclic aromatic hydrocarbons ) Naphthylamine Azo dyes Long term cyclophosphamide or phenacetin use Presents in older adults as PAINLESS hematuria Mechanism : 1- Flat : develops as high grade flat tumor—> invades ( associated with p53 mutation ) 2- Papillary : develops as low grade papillary tumor —> high grade papillary tumor—> invades ( NOT associated with p53 mutation )
78
Characteristic of urothelial carcinoma ?
``` Multifocal Recure ( field defect ) ```
79
Risk factors for squamous cell carcinoma ?
Chronic cystitis Shistosoma hematobium infection Long standing nephrolithiasis
80
Adenocarcinoma of bladder arises from ?
1- Urachal remnant ( develops at the dome of the bladder ) 2- Cystitis glandularis 3- Exstrophy