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Boards Final Review > Kidney + Endocrine > Flashcards

Kidney + Endocrine Flashcards

1
Q

Non-inherited disorder leading to cysts in the kidney with abnormal tissue (ex. cartilage) surrounding it

A

Dysplastic kidney

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2
Q

Where do the cysts arise in PCKD? Medullary cystic kidney disease?

A

PCKD - Renal cortex and medulla (enlarged kidneys)

Medullar cystic kidney disease - cysts in medullar collecting ducts (shrunken kidneys)

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3
Q

What are the associated features in PCKD?

A

Autosomal recessive – hepatic cysts, hepatic fibrosis

Autosomal dominant – berry aneurysm (usually the cause of death), hepatic cysts, mitral valve prolapse

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4
Q

What is acute interstitial nephritis?

A

Hypersensitivity reaction involving the interstitium and tubules resulting in acute renal failure. Mainly caused by NSAIDs, PCNs and diuretics. It may progress to renal papillary necrosis which is coagulative necrosis of the medullary pyramids of the kidney due to vascular impairment.

Eosinophils + inflammatory infiltrate in the interstitium

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5
Q

What cancer is associated with minimal change disease?

A

Hodgkin’s lymphoma – massive overproduction of cytokines by B cells and the cytokines have a change to hit the kidney and knock out the barrier

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6
Q

What mediates minimal change disease?

A

Cytokine destruction of the podocytes – you ONLY lose albumin, no immunoglobulin is lost b/c there is such a small change

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7
Q

What is the pathogenesis of Membranous nephropathy?

A

Subepithelial Immune complex deposition – associated with Hep B, Hep C, SLE, drugs, or solid tumors - spike-and-dome appearance on EM due to BM proliferation around complexes

[note that Hep B, and C are also associated with type I membranoproliferative glomerulonephritis]

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8
Q

Which renal pathology is associated with C3 nephritic factor?

A

Type II membranoproliferative glomerulonephritis

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9
Q

What type of arteriolosclerosis and where in the kidney is seen with diabetes mellitus?

A

Hyaline arteriolosclerosis – preferentially seen in the efferent arterial [increasing back pressure to the glomerulus causing hyperfiltration and microalbuminuria]

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10
Q

Where in the kidney does amyloid deposit?

A

Mesangium

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11
Q

What is the general pathogenesis behind nephritic syndrome?

A

Immune-complex deposition which activates complement – C5a is released with activated complement which attracts neutrophils that can mediate damage

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12
Q

Why do you get periorbital edema with nephritic syndrome?

A

Salt retention – accumulates in the loose connective tissues around the eyes

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13
Q

What is the composition of the crescent seen in rapidly progressive glomerulonephritis?

A

Fibrin + macrophages (inflammatory debris)

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14
Q

What is the most common type of renal disease in lupus?

A

diffuse proliferative glomerulonephritis leading to RPGN and renal failure/death

[if they have nephrotic syndrome presentation, then think membranous nephropathy]

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15
Q

What is sterile pyuria?

A

Sterile pyruia with a negative urine culture – it suggests urethritis due to Chlamydia trachomatis or Neisseria gonorrhoea

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16
Q

Which types of kidney stones are radiopaque? Radiolucent?

A

Radiopaque [can be seen on x-ray]

  • calcium oxalate/phosphate
  • ammonium magnesium phosphate

Radiolucent [cannot be seen on x-ray]

  • uric acid
  • cystine
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17
Q

What cells in the kidney produce EPO?

A

Renal peritubular interstitial cells

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18
Q

What are the paraneoplastic syndromes of renal cell carcinoma?

A
  • EPO
  • renin
  • PTHrp
  • ACTH
19
Q

What is the effect of loss of VHL (3p) tumor suppressor gene?

A
  1. increased IGF-1 (promoting growth)

2. increased HIF transcription factor (which increases VEGF and PDGF)

20
Q

What are the features of Beckwith-Wiedemann syndrome?

A
  1. Wilms tumor
  2. neonatal hypoglycemia
  3. muscular hemihypertrophy - muscles on 1 half of the body are larger than the other half of the body
  4. organomegaly (ex. tongue)

**Associated with WT2 mutation

21
Q

What are the different syndromes associated with a WT1 deletion vs WT1 mutation?

A

WT1 deletion
-WAGR syndrome

WT1 mutation
-Denys-Drash syndrome [Wilms tumor, progressive renal (glomerular disease), male pseudohermaphroditism]

22
Q

What are the two pathways of urothelial (transitional cell) carcinoma development?

A

Papillary - papillary growth starting as low grade, progressing to high grade then invasion

Flat -high grade that progresses to invasion, associated with early p53 mutations

**“FIELD DEFECT” – so recurrence occurs b/c the mutations are generally present throughout the entire tissue

23
Q

What type of bladder cancer is associated with a urachal remnant?

A

Adenocarcinoma of the bladder (present AT THE DOME of the bladder)

24
Q

What is the most common cause of death in adults with growth hormone cell adenomas?

A

Cardiomegaly – also watch out for secondary diabetes due to elevated GH and IGF-1

25
Q

Where do craniopharyngiomas occur in children?

A

derived from pituitary gland embryonic tissue within the sella tursica

aka Rathke pouch tumors

26
Q

How does Sheehan syndrome present?

A

Poor lactation, loss of pubic hair, fatigue

27
Q

How does elevated thyroid hormone affect cholesterol and glucose levels?

A

Hypocholesterolemia

Hyperglycemia [due to gluconeogenesis and glycogenolysis]

28
Q

What is the most common enzyme deficiency leading to hypothyroidism?

A

Thyroid peroxidase (dyshormonogenetic goiter)

29
Q

What tissue is specific to follicular adenoma and carcinoma?

A

fibrous capsule surrounding follicle – it is not invading in the adenoma, but it is invaded in the carcinoma

FINE NEEDLE ASPIRATION – cannot differentiate b/t follicular adenoma vs carcinoma b/c you can not see whether there is fibrous capsule invasion or not

30
Q

What are the 4 carcinoma exceptions that initially spread through the blood rather than the lymphatics?

A
  • renal cell carcinoma to renal vein
  • hepatocellular carcinoma to hepatic vein
  • follicular carcinoma
  • choriocarcinoma
31
Q

What are the urinary cAMP levels with hyperparathyroidism?

A

PTH binds Gs receptor increases adenylate cyclase which increases cAMP – b/c there is so much PTH signaling the cAMP accumulation spills out in to the urine

32
Q

Numbness and tingling around the lips?

A

Hypocalcemia

33
Q

What is pseudohypoparathyroidism?

A

End-organ resistance to PTH due to defective Gs signaling – there will be hypocalcemia with increased PTH levels – the defective Gs is due to an autosomal dominant trait associated with short stature and short 4th and 5th digits

34
Q

What is the cause of hyperosmolar non-ketotic coma?

A

high glucose (over 500 mg/dL) leading to life-threatening diuresis with hypotension and coma – there is a lack of ketones due to the small amount of circulating insulin

35
Q

What are the symptoms of somatostatinomas?

A
  • Achlorhydria - due to inhibition of gastrin

- Cholelithiasis with steatorrhea – due to inhibition of CCK

36
Q

What is “Whipples triad?”

A

Pancreatic insulinoma which consists of fasting hypoglycemia (less than 50 mg/dL), symptoms of hypoglycemia and immediate relief with IV glucose adminsitration

37
Q

What are the features of a glucagonoma?

A

Tumor of the pancreatic ALPHA cells that lead to the overproduction of glucagon. This generally presents with:
Dermatitis (necrolytic migratory erythema) [painful skin lesions]
Diabetes (hyperglycemia)
DVT
Depression

**elevated glucose dilutes Na+

38
Q

Why is there immunosuppression with Cushing’s syndrome?

A
  1. inhibits PLA2
  2. inhibits IL-2
  3. Inhibits histamine release from mast cell
39
Q

What happens to the adrenals in exogenous corticosteroid administration?

A

Both adrenal atrophy

40
Q

What are the causes of chronic adrenal insufficiency?

A
  1. autoimmune destruction - most common in the west
  2. TB - most common in the developing world
  3. metastatic carcinoma - ESP from the LUNG
41
Q

What are the rules of 10 with pheochromocytoma?

A

10% bilateral
10% familial
10% malignant
10% located outside of adrenal medulla (esp bladder wall!)

42
Q

What tumors are associated with von Hippel-Lindau disease?

A
  1. hemangioblastoma of the cerebellum
  2. renal cell carcinoma
  3. pheochromocytoma
43
Q

What are the 3 disorder associations of pheochromocytomas?

A
  1. MEN 2A and 2B
  2. NF type 1
  3. VHL

Boards Final Review (7 decks)

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