Renal and Urinary Tract Congenital and ARF PATHOMA/FA Flashcards

1
Q

In UNILATERAL AGENESIS of kidney, what happens to the existing kidney? Is there any consequence of it?

A

HYPERTROPHY - Existing kidney does work of both

Consequence: Increased risk of RENAL FAILURE later in life with HYPERFILTRATION injury

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2
Q

In BILATERAL AGENESIS of the kidney, what are the 3 possible consequences that make up the POTTER SEQUENCE? What is the underlying cause?

A

UNDERLYING CAUSE: Oligohydramnios (low Amniotic fluid - kidney filtrate that the baby floats around in)
POTTER SEQUENCE = Lung hypoplasia + Flat face/Low set ears + Extremity dvlm defect

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3
Q

What are the 3 congenital malformations that result in cyst formation?

A
  1. DYSPLASTIC KIDNEY
  2. POLYCYSTIC KIDNEY DISEASE
  3. MEDULLARY CYSTIC KIDNEY DISEASE
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4
Q

Is DYSPLASTIC KIDNEY an INHERITED congenital malformation? Where do the cysts form, what is a main component of the cysts?

A

NO, NOT INHERITED - Low risk of having dysplastic kidney in subsequent pregnancy
CYSTS in the RENAL PARENCHYMA - Made of abnormal tissue (particularly cartilage)

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5
Q

Is POLYCYSTIC KIDNEY an INHERITED congenital malformation? Do cysts form unilaterally or bilaterally? Where are the cysts formed?

A

YES, INHERITED

BILATERAL enlarged kidney with Cysts in the RENAL CORTEX + MEDULLA

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6
Q

What are the 2 subtypes of POLYCYSTIC KIDNEY? What age population is most commonly affected by each?

A
  1. Autosomal recessive - JUVENILE form affecting INFANTS

2. Autosomal dominant - AD form affecting ADults

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7
Q

What are the clinical Sx of AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE?

A
  1. Worsening renal failure

2. HTN

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8
Q

What is a particularly important and dangerous complication that can occur in severe AUTOSOMAL RECESSIVE PKD?

A

POTTER SEQUENCE - AR PKD being so severe that it can manifest as bilateral agenesis (no kidney at all) -> Oligohydramnios -> Potter Sequence

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9
Q

What are the 2 common associations of AUTOSOMAL RECESSIVE PKD?

A

‘Cysts in the kidney, cysts in the liver’

Kidney Cysts + Hepatic Cyts + Hepatic fibrosis (resulting in PORTAL HTN)

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10
Q

What are the clinical Sx of AUTOSOMAL DOMINANT PKD?

A
  1. Worsening Renal Failure
  2. HTN
  3. Hematuria
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11
Q

What is the likely pathogenesis of AUTOSOMAL DOMINANT PKD?

A

Mutation in APKD1 or APKD2 gene

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12
Q

What are the 3 common associations of AUTOSOMAL DOMINANT PKD?

A

‘Cysts in the kidney, cysts in the liver, cysts in the brain’
Kidney Cysts + Hepatic Cysts + Cystic Dilatation (berry aneurysm) + Mitral valve prolapse

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13
Q

24yo pt has family history of renal disease and multiple deaths in the family due to renal conditions or a brain hemorrhage. What is on the #1 differential’s condition?

A

AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE

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14
Q

What are the 2 main differences between MEDULLARY CYSTIC KIDNEY DISEASE (MCKD) and POLYCYSTIC KIDNEY DISEASE (PKD)?

A

MCKD - Cysts form in the MEDULLARY COLLECTING DUCTS + Kidneys are SHRUNKEN

PCKD - Cysts form in the renal cortex + medulla
Kidneys are ENLARGED

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15
Q

What is the inheritance pattern of MEDULLARY CYSTIC KIDNEY DISEASE

A

AD

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16
Q

What is the most common congenital RENAL anomaly?

A

HORSESHOE KIDNEY

17
Q

In a HORSESHOE KIDNEY congenital abnormality, where is the kidney abnormally located in? Why?

A

LOWER ABDOMEN - Bec its embryological ascent from pelvis to abdomen is blocked -> Gets stuck in root of INFERIOR MESENTERIC ARTERY

17
Q

What are the 3 types of ACUTE RENAL FAILURE? Which is the most common cause of ARF?

A
  1. PRERENAL AZOTEMIA - Decreased renal blood flow
  2. POSTRENAL AZOTEMIA - Obstruction of urinary tract downstream from kidney
  3. INTRARENAL AZOTEMIA : ATN - Necrosis of tubular epithelial cells = most common cause of ARF
18
Q

What are the following values of PRERENAL AZOTEMIA: BUN:Cr, FENa, Urine Osm?

A

BUN:Cr > 15

INTACT Tubules: FENa500 - Can reabsorb Na+ and can concentrate urine

19
Q

What is the difference between EARLY STAGE and LATE STAGE of POST-RENAL AZOTEMIA? List values.

A

Tubular Damage
EARLY STAGE: NO tubular damage - BUN:Cr>15, FENa500
LATE STAGE: YES tubular damage - BUN:Cr2%, Urineosm

20
Q

In ACUTE TUBULAR NECROSIS (Intrarenal azotemia), what are the following ranges of BUN:Cr, FENa, and Urine Osm? What else is seen in the urine?

A
  1. BUN:Cr2%

3. Uosm

21
Q

What are the two possible etiologies of ATN? Which portion of the nephron unit are particularly susceptible?

A
  1. ISCHEMIC - PT and early TAL (medullary)

2. NEPHROTOXIC - PT

22
Q

Which etiology subtype of ATN (INTRARENAL Azotemia) is often preceded by PRE-RENAL azotemia? Why?

A

ISCHEMIC ATN is often preceded by PRE-RENAL due to DECREASED BLOOD SUPPLY to the tubules resulting in necrotic cells characterizing ATN

23
Q

Which cause of NEPHROTOXIC ATN is associated with OXALATE crystals in the urine?

A

ETHYLENE GLYCOL (i.e. ANTIFREEZE) - Blue and sweet

24
Q

What are the causes of NEPHROTOXIC ATN? What is the most common cause?

A

‘HEAR MU’
H- Heavy metals (Lead), E- Ethylene Glycol (oxalate crystals in urine), A- Aminoglycosides, R -radioactive dye , M - Myoglobinuria (from crush injury) , U- Urate (e.g. tumor lysis syndrome)

OR ‘ACRUEL’ - aminoglycosides/amphotericin B (NOT ATN, Type I RTA), crush injury (myoglobinuria), radioactive dye, urate, ethylene glycol, lead poisoning

25
Q

Which cause of NEPHROTOXIC ATN is associated with CHEMO? What 2 prophylactic treatments are administered pre-chemo to decrease ATN risk caused by the chemo agent?

A

URATE - E.g. Acute leukemia pt receives chemo - Rapidly kills cells -> Rapid nuclear breakdown -> High uric acid -> Damages tubules via ATN
1. HYDRATION - To maintain good urinary flow + 2. ALLOPURINOL - To block uric acid buildup

26
Q

Is ATN Irreversible or Reversible? How?

A

REVERSIBLE - Tubular cells have the ability to regenerate albeit it is a STABLE tissue so it takes some time to regenerate

27
Q

What is the acid-base status, K+ state, BUN/Cr, urine production of ATN?

A

METABOLIC ACIDOSIS (Decreased excretion of organic acids NH4+ and HPO42-), HYPERKALEMIA (Decreased K+ secretion/excretion due to damaged tubular cells), Elevated BUN/Cr - AZOTEMIA, OLIGURIA (Decreased urine prdn)

28
Q

How long does OLIGURIA associated with ATN last? Why?

A

2-3WEEKS bec tubular cells are STABLE CELLS (outside of the cell cycle, so it takes time to re-enter and regenerate)

29
Q

What is the TREATMENT of ATN?

A

SUPPORTIVE DIALYSIS bec electrolyte imbalances can be FATAL

30
Q

What is pathognomonic of EOSINOPHILS in the urine?

A

ACUTE INTERSTITIAL NEPHRITIS (Drug-induced)

31
Q

What are the 5P causes of ACUTE INTERSTITIAL NEPHRITIS?

A

Pee (diuretics), Pain-free (NSAIDS), Penicillin, Proton Pump Inhibitors, rifamPin

32
Q

What type of inflammatory reaction is involved in ACUTE INTERSTITIAL NEPHRITIS?

A

Drug-induced HYPERSENSITIVITY reaction involving the INTERSTITIUM and can spread to the TUBULES

33
Q

What are the clinical Sx of ACUTE INTERSTITIAL NEPHRITIS?

A

OLIGURIA
FEVER
RASH

34
Q

What is the Tx of ACUTE INTERSTITIAL NEPHRITIS?

A

Resolves with DRUG CESSATION

35
Q

What are the causes of PAPILLARY NECROSIS?

A

‘SAAD PAPa’ with PAPillary necrosis

S- sickle cell trait/disease, A- analgesics, A- acute pyelonephritis, D- Diabetes mellitus

36
Q

What are the clinical Sx of PAPILLARY NECROSIS? What could have been a precursor?

A

HEMATURIA + FLANK PAIN

Drug induced ACUTE INTERSTITIAL NEPHRITIS could have progressed to papillary necrosis

37
Q

What is the most common cause of ACUTE KIDNEY INJURY in hospitalized pts?

A

ACUTE TUBULAR NECROSIS