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Immunosuppressants Flashcards

1
Q

What bilirubin is the better indicator of extravascular hemolysis?

A

Unconjugated bilirubin

Unconjugated bilirubin levels rise due to increased hemolysis.

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2
Q

What cells are associated with CKD, liver disease, and PKD?

A

Burr cells (echinocytes)

These cells are indicative of various pathological conditions.

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3
Q

Why does ethanol (EtOH) poisoning cause hypoglycemia?

A

Elevated NADH:NAD+ in liver inhibits gluconeogenesis

This disruption affects glucose production.

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4
Q

What is the normal anion gap value?

A

<12 mEq/L

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5
Q

Name some ingestions causing anion gap metabolic acidosis

A
  • Aspirin
  • Ethylene glycol
  • Propylene glycol
  • Methanol
  • INH
  • Metformin

These substances can disrupt normal metabolic processes.

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6
Q

What condition can MSUD (BCKDH deficiency) result in if untreated?

A

Anion-gap metabolic acidosis

This metabolic derangement can have serious consequences.

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7
Q

What do mutations in glutamate dehydrogenase lead to?

A

Hyperinsulinemia hyperammonemia syndrome

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8
Q

What disease is caused by a deficiency in beta-glucocerebrosidase?

A

Gaucher disease

It has autosomal recessive inheritance and presents with HSM, bone pain, and more.

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9
Q

What are the key presentations of Hurler syndrome?

A
  • Corneal clouding
  • Coarse facies
  • Skeletal problems
  • HSM
  • Early death (before 10 years)

Hurler syndrome is caused by alpha-l-iduronidase deficiency.

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10
Q

What is Niemann-Pick disease caused by?

A

Deficiency in sphingomyelinase

It presents with HSM, a cherry-red spot on maculae, and variable neuro deficits.

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11
Q

What can aspirin overdose cause?

A

Combined metabolic acidosis (lactic acidosis) and respiratory alkalosis

This occurs due to CNS stimulation leading to hyperventilation.

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12
Q

What is the capsule of Hib made of?

A

Polyribosylribitol phosphate

This is the target of the Hib vaccine.

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13
Q

What is the vaccine target for salmonella typhi?

A

Vi capsule

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14
Q

What does Protein A bind to?

A

Fc portion of IgG

This surface protein is associated with S. Aureus.

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15
Q

What is M protein an antigen of?

A

S. Pyogenes

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16
Q

What is the mechanism of bleomycin?

A

Generation of free radicals causing DNA strand breaks

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17
Q

What does the BEP regimen stand for?

A

Bleomycin, Etoposide, Cisplatin

It is commonly used for testicular cancer and Hodgkin lymphoma.

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18
Q

What does Cytarabine (ara-C) inhibit?

A

DNA polymerase

It is used in the treatment of AML and has hematologic side effects.

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19
Q

What phases is Etoposide specific for?

A

S- and G2-phase specific topoisomerase II inhibitor

It is commonly used to treat small cell carcinoma and testicular cancer.

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20
Q

What causes Cri-du-Chat syndrome?

A

Deletion at 5p

It presents with microcephaly and intellectual disability.

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21
Q

What are the characteristics of Williams syndrome?

A
  • Microdeletion at 7p
  • Elfin facies
  • Intellectual disability
  • Hypercalcemia
  • Cardiovascular problems

This genetic disorder has distinctive physical and health features.

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22
Q

What causes cutaneous larva migrans?

A

Hookworm larvae (e.g., Ancylostoma braziliense)

It leads to skin manifestations due to larval migration.

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23
Q

What are Birbeck granules?

A

Tennis-racket shaped on EM identifying Langerhans cells

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24
Q

What does CD14 help recognize?

A

PAMPs

CD14 is found on the surface of macrophages.

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25
What does plasmin degrade?
Fibrin and fibrinogen
26
What is the initiator caspase?
Caspase-9
27
What is the executioner caspase?
Caspase-3
28
What does cord factor activate?
Macrophages and induces release of TNF-a ## Footnote It is a surface glycolipid of Mtb.
29
What is produced by SHiN species?
IgA protease
30
What does prostacyclin inhibit?
Platelet activation ## Footnote It promotes vasodilation.
31
What role does PGE2 play?
Regulates uterine tone, promotes vasodilation, and modulates inflammation ## Footnote It also promotes fever.
32
What vascular effect can serotonin promote?
Vasoconstriction or vasodilation ## Footnote It can act directly or via endothelial cell-derived nitric oxide.
33
What is the most common renal malignancy in young children?
Wilms tumor ## Footnote It presents with unilateral abdominal mass and hematuria.
34
What urinary adverse effect is most associated with cyclophosphamide?
Hemorrhagic cystitis
35
What condition are both bleomycin and busulfan associated with?
Pulmonary fibrosis
36
What causes Type IV RTA?
Aldosterone deficiency or resistance in the DCT/CD ## Footnote This results in hyperkalemia and normal anion gap metabolic acidosis.
37
What metabolic derangement do loop or thiazide diuretics cause?
Hypokalemia with metabolic alkalosis ## Footnote Urine shows increased potassium and sodium excretion.
38
What deposits are seen on a renal biopsy of lupus nephritis?
Glomerular subendothelial immune complex deposits ## Footnote It may also show mesangial proliferation.
39
What are the clinical findings of lupus nephritis?
* Hematuria * Proteinuria * Progressive loss of renal function
40
What 3 findings are seen in LM of Focal Segmental Glomerulosclerosis (FSGS)?
* Segmental sclerosis * Mesangial hypercellularity * Collapse of glomerular capillaries ## Footnote FSGS is the most common primary glomerular disease in the USA.
41
What are the risk factors for FSGS?
* HIV * IVDU * Obesity * Unilateral renal agenesis ## Footnote It is more common among young black men.
42
What common medication is associated with membranous glomerulonephropathy?
NSAID use
43
What cancer is a risk factor for minimal change disease?
Lymphoma
44
What is Hyperglycemic Hyperosmolar Syndrome (HHS)?
Acute hyperglycemia without ketoacidosis ## Footnote It is typically seen in T2DM.
45
What is the most common cause of MPGN Type I?
HCV infection ## Footnote HBV can also cause MPGN but is less likely.
46
What is characterized by splitting of the GBM?
MPGN ## Footnote It presents with a double-contour or tram-track appearance.
47
What is a common laboratory finding in MPGN?
Serum hypocomplementemia
48
What are the LM biopsy findings of MPGN?
* Thickening of the GBM * Splitting of the GBM * Glomerular hypercellularity * Increased mesangial matrix ## Footnote The tram-track sign is characteristic.
49
What are the most common causes of nephritic-nephrotic syndrome?
* MPGN * DPGN
50
What EM finding characterizes diabetic glomerulosclerosis?
Dramatic thickening of the capillary basement membrane without immune complexes
51
What is the best treatment for diabetic nephropathy?
ACE inhibitors ## Footnote They decrease efferent arteriolar tone.
52
What does AT-II do at the JGA?
Increases sensitivity of tubuloglomerular feedback
53
What does binding of ADH to V2 receptors directly activate?
Adenylyl cyclase ## Footnote V2 receptors are part of the Gs signaling pathway.
54
What does an elevated BUN/Cr ratio (>20:1) indicate in the setting of AKI?
Prerenal AKI ## Footnote Increased urea reabsorption occurs during hypoperfusion.
55
What is the most common cause of Potter sequence?
Bilateral renal agenesis ## Footnote It results from failure of the ureteric bud to develop.
56
What is urate nephropathy?
Interstitial deposition of uric acid ## Footnote It can be seen in gout.
57
What is colchicine used for?
Treatment of gout ## Footnote Its significant adverse effect is agranulocytosis.
58
What renal adverse effect can Indinavir cause?
Crystal-induced nephropathy ## Footnote It presents with hematuria and needle-shaped crystals.
59
What is the adverse effect of Dolutegravir?
Elevated serum lipase, elevated CPK, and elevated serum creatine
60
What does HIV nephropathy most often manifest as?
Focal segmental glomerulosclerosis (FSGS)
61
What two organisms are most associated with struvite stones?
* Proteus * Klebsiella
62
What does nitrite positive urine suggest?
Presence of gram-negative organisms
63
What is the most common type of kidney stone?
Calcium oxalate stones ## Footnote They primarily result from hypercalciuria.
64
What molecule, when in urine, prevents formation of calcium oxalate stones?
Citrate
65
What causes calcium phosphate stones?
* Hypercalciuria * Dehydration * Hyperparathyroidism ## Footnote They precipitate in alkaline urine.
66
What shape do uric acid crystals form?
Rhomboid or rosette-shaped
67
What EKG findings are early signs of severe hyperkalemia?
* Peaked T waves * PR interval elongation ## Footnote These signs appear with serum potassium >6-7 mEq/L.
68
What does lack of ADH secretion cause?
Maximally dilute urine (<200 mOsm/kg, specific gravity <1.005)
69
What can low oxygen pressures at high altitudes cause?
* Cerebral edema * Pulmonary edema * Respiratory alkalosis ## Footnote Symptoms include headache and altered mental status.
70
What does acetazolamide do in altitude sickness?
Causes a metabolic acidosis ## Footnote This stimulates hyperventilation and increases oxygenation.
71
What do ARBs impair in relation to urine pH?
Maximal lowering of urinary pH (ARBs can slightly increase urine pH)
72
What effect does bicarbonate administration have on potassium?
Alkalinizes serum and decreases serum potassium (pH shift of potassium into cells)
73
What can potassium administration stimulate?
Aldosterone release ## Footnote This leads to collecting duct H+ secretion.
74
What does PTH inhibit in the renal tubules?
Sodium/phosphate cotransport in the PCT ## Footnote This promotes phosphate excretion.
75
What does AT-II stimulate in the renal tubules?
Na/H exchange ## Footnote This promotes sodium, water, and bicarbonate reabsorption.
76
What do early DCT cells do in relation to calcium?
Promote calcium/sodium exchange on the basolateral membrane
77
How is bicarbonate excreted in beta-intercalated cells?
Via an apical bicarbonate/chloride exchanger
78
What is the general function of the collecting tubule?
Reabsorbing sodium (and water) in exchange for secreting potassium and H+
79
What is seen histologically in lupus nephritis?
Deposition of immune complexes at the glomerulus ## Footnote This leads to complement cascade and damage.
80
What are the histologic features of diabetic nephropathy?
* Diffuse GBM thickening * Kimmelstiel-Wilson nodules ## Footnote These nodules are pathognomonic for the condition.
81
What complement effect does Staphylococcus-associated Glomerulonephritis present with?
* Hematuria * Proteinuria * Renal failure * Low serum complement ## Footnote It occurs in the setting of a staph infection.
82
What drugs are most associated with acute interstitial nephritis?
* Antibiotics * Penicillins * Cephalosporins * Rifampin * Sulfa drugs * TMP-SMX * Loop diuretics * Thiazide diuretics * NSAIDs * PPIs ## Footnote These can cause nephritis via haptenization.
83
What autoimmune causes are associated with acute interstitial nephritis?
* SLE * Sjogren * Sarcoidosis
84
What is the classic triad of AIN?
* Fever * Rash * Eosinophilia ## Footnote However, the complete triad is rarely seen in practice.
85
What are the common antibiotics associated with acute interstitial nephritis (AIN)?
Antibiotics include: * Penicillins * Cephalosporins * Rifampin * Sulfa drugs * TMP-SMX
86
What are the classic symptoms of acute interstitial nephritis (AIN)?
Classic triad includes: * Fever * Rash * Eosinophilia
87
What autoimmune diseases can cause acute interstitial nephritis (AIN)?
Autoimmune causes include: * SLE * Sjogren's * Sarcoidosis
88
What lab changes are typically observed in acute interstitial nephritis (AIN)?
Lab changes include: * Elevated BUN * Elevated Cr * Eosinophilia * Elevated IgE
89
What findings in urinalysis (UA) can help distinguish AIN from pyelonephritis?
UA findings include: * Hematuria * Pyuria * Eosinophiluria * WBC casts
90
What is the definitive diagnosis method for acute interstitial nephritis (AIN)?
Definitive diagnosis is through biopsy
91
What is the typical prognosis for acute interstitial nephritis (AIN)?
Prognosis is usually complete recovery, but some may have persistent elevation of serum BUN and Cr, leading to CKD
92
What are the phases of acute tubular necrosis (ATN)?
Phases include: * Inciting event * Maintenance (oliguric) * Recovery (polyuria)
93
What is diffuse cortical necrosis and its associations?
Diffuse cortical necrosis is caused by DIC + vasospasm and is associated with: * Abruption placentae * Septic shock
94
What triggers renal papillary necrosis?
Triggered by recent infection or immune stimulus, presenting as gross hematuria and proteinuria
95
Where does the activation of calcidiol to active calcitriol occur?
Activation occurs in the PCT (proximal convoluted tubule)
96
What is the net effect of FGF-23 in the body?
FGF-23 lowers serum phosphate by inhibiting PCT phosphate reabsorption
97
What stimulates the release of parathyroid hormone (PTH)?
PTH release is stimulated by: * Low vitamin D * Low calcium * High phosphate
98
What are common manifestations of renal failure?
Manifestations include: * Metabolic acidosis * Dyslipidemia * Hyperkalemia * Uremia * Anorexia, N/V * Pericarditis * Encephalopathy * Growth retardation * EPO decreased * Renal osteodystrophy
99
What is the GFR threshold for end-stage renal disease (ESRD)?
GFR < 15
100
What are the treatment options for renal failure?
Treatment options include: * Strict BP control * Low phosphorus diet * +/- EPO * Dialysis * Transplant
101
What causes renal osteodystrophy?
Caused by: * Poor activation of Vitamin D * Elevated phosphate * Secondary hyperparathyroidism
102
What is calciphylaxis?
Calciphylaxis is abnormal calcium phosphate deposition in small blood vessels, often during renal failure-induced hyperphosphatemia
103
What is the most common primary renal malignancy in adults?
Renal Cell Carcinoma (RCC)
104
What are the subtypes of renal cell carcinoma?
Subtypes include: * Clear Cell Carcinoma * Papillary Carcinoma * Chromophobe Carcinoma
105
What is the main treatment for renal oncocytomas?
Treatment is nephrectomy to exclude malignancy
106
What is the most common renal malignancy in early childhood?
Nephroblastoma (Wilms tumor)
107
What is Fanconi syndrome?
Fanconi syndrome is abnormal function of the proximal tubules leading to loss of amino acids, glucose, phosphates, and urates in urine
108
What is the most common cause of nephrogenic diabetes insipidus (DI)?
The most common cause is lithium toxicity
109
What are the drugs commonly associated with acute tubular necrosis (ATN)?
Drugs include: * Aminoglycosides * IV contrast dyes * Vancomycin * Cisplatin * Pentamidine * Foscarnet * Cidofovir * Tenofovir
110
What is the hallmark of IgA nephropathy?
IgA nephropathy often presents with gross hematuria after a viral infection
111
What are the characteristics of membranous nephropathy under TEM?
Shows uniform subepithelial deposits
112
What does elevated c-ANCA indicate?
Elevated c-ANCA (anti-PR3) is seen in Granulomatosis with polyangiitis (GPA)
113
What causes euvolemic hyponatremia in SIADH?
Caused by water reabsorption without sodium reabsorption, leading to low serum sodium and high urine osmolality
114
What is the effect of thiazide diuretics on calcium reabsorption?
Thiazides increase calcium reabsorption in the distal convoluted tubule
115
What condition is caused by WT1 deletion?
WAGR complex
116
What are the components of Drash syndrome?
* Pseudohermaphroditism * CKD * Wilms tumor
117
What is the mean age of diagnosis for VHL?
Around 29 years
118
What are the findings associated with VHL?
* Hemangioblastomas of the retina * Hemangioblastomas of the cerebellum * Hemangioblastomas of the medulla * RCC in around 50% of individuals
119
What is the primary use of Bethanechol?
Treat postoperative or neurogenic ileus and urinary retention. Nonselective muscarinic agonist.
120
True or False: IV dextrose affects plasma osmolality significantly.
False
121
What causes hyponatremia in SIADH?
ADH causes water reabsorption while leaving sodium in the urine
122
What is the role of alpha-1 receptor agonists in the bladder?
Promote contraction of the bladder sphincter
123
What is a typical ABG presentation of normal anion gap metabolic acidosis?
* Low pH * Low bicarbonate * Low pCO2 * Normal anion gap (<12 mEq/L)
124
What can cause an elevation in BUN without an increase in serum creatinine?
* Heavy protein ingestion * Gastrointestinal bleeding
125
What is the embryological origin of the urogenital system?
Urogenital ridge of intermediate mesoderm
126
What is the function of the metanephros?
Becomes the permanent kidney
127
What is contraction alkalosis caused by?
* Loss of fluid without loss of bicarbonate * Hemorrhage * Vomiting * Diarrhea * Diuretic use
128
What are thiazides primarily used for?
* Hypertension * Heart failure * Idiopathic hypercalciuria * Nephrogenic DI * Osteoporosis
129
What are common adverse effects of thiazide diuretics?
* Hypokalemic metabolic alkalosis * Hyponatremia * Hyperglycemia * Hyperlipidemia * Hyperuricemia * Hypercalcemia
130
What are the main adverse effects of loop diuretics?
* Ototoxicity * Hypokalemia * Hypomagnesemia * Dehydration * Allergy (sulfa) * Alkalosis (metabolic) * Nephritis (interstitial) * Gout
131
What characterizes Hepatorenal Syndrome?
Rapid-onset AKI without evidence of renal ischemia
132
What is the typical histological finding in Polyarteritis nodosa?
Necrotizing inflammation of small renal arteries
133
What does Alport syndrome present with histologically?
Thinning of the GBM
134
What is the primary histological finding in PIGN?
Enlarged and hypercellular glomeruli
135
What does IgA nephropathy typically occur after?
1-3 days after a viral illness
136
What is the main treatment for acute renal transplant rejection?
Immunosuppressive therapy
137
What condition is associated with increased urine calcium?
Formation of calcium oxalate stones
138
What characterizes HIV nephropathy?
FSGS
139
What initiates the pathophysiology of diabetic nephropathy?
Microalbuminuria
140
Fill in the blank: Methacholine, bethanechol, and pilocarpine are examples of _______.
muscarinic agonists
141
What is the mechanism of action of neostigmine?
AChE inhibitor used in MG treatment
142
What is the genetic mutation associated with Ataxia telangiectasia?
ATM gene mutation
143
What is the primary association of HNPCC?
Colon cancer
144
What stimulates pyruvate carboxylase?
Elevated acetyl-CoA and glucagon
145
What are the two main calcineurin inhibitors?
* Cyclosporine * Tacrolimus
146
What does MELAS stand for?
* Myopathy * Encephalopathy * Lactic acidosis * Stroke-like episodes
147
What does cyclosporine complex with?
Cyclophilin ## Footnote Cyclosporine is an immunosuppressant that inhibits calcineurin in T cells.
148
What does tacrolimus complex with?
FKBP-12 ## Footnote Tacrolimus also inhibits calcineurin in T cells.
149
What is the effect of calcineurin inhibition in T cells?
Inhibition of IL-2 signaling ## Footnote IL-2 signaling is crucial for T cell activation and proliferation.
150
What does daclizumab do?
Antagonizes IL-2 by binding to the IL-2 receptor on T cells ## Footnote Daclizumab is used in transplant rejection and autoimmune diseases.
151
What is the mechanism of action of mycophenolate?
Decreases purine synthesis by inhibiting IMPDH ## Footnote Mycophenolate is an immunosuppressant often used in organ transplantation.
152
What does MELAS stand for?
Mitochondrial myopathy, Encephalopathy, Lactic acidosis, Stroke-like episodes ## Footnote MELAS is a genetic disorder that affects multiple systems.
153
What are the histological features of MELAS?
Ragged red fibers ## Footnote These fibers indicate mitochondrial dysfunction.
154
What type of genetic disorder is Duchenne muscular dystrophy?
X-linked recessive disorder ## Footnote It primarily affects males and is caused by mutations in the dystrophin gene.
155
What causes achondroplasia?
Mutation in FGFR-3 ## Footnote Achondroplasia is an autosomal dominant disorder, with most cases being sporadic.
156
What is the inheritance pattern of Rett syndrome?
X-linked dominant disorder ## Footnote It primarily affects females and is associated with mutations in the MECP2 gene.
157
What is familial hypercholesterolemia caused by?
Inherited abnormality in ApoB-100 or defective LDL receptors ## Footnote It leads to elevated LDL levels from birth.
158
What are the clinical features of dysbetalipoproteinemia?
Premature atherosclerosis, tuberoeruptive xanthomas, xanthoma striatum palmare ## Footnote It is caused by deficiency of ApoE.
159
What does citrate inhibit?
PFK-1 ## Footnote PFK-1 catalyzes the rate limiting step of glycolysis.
160
What stimulates pyruvate kinase?
F16BP ## Footnote F26BP stimulates PFK-1, which is also involved in glycolysis regulation.
161
What are the classic symptoms of Thrombotic Thrombocytopenic Purpura?
Fever, Thrombocytopenia, MAHA, Neurologic symptoms, Mild AKI ## Footnote MAHA stands for microangiopathic hemolytic anemia.
162
What is the primary cause of duodenal ulcers?
Underlying H. pylori infection ## Footnote H. pylori is a common bacterium associated with gastrointestinal ulcers.
163
What is a common complication of systemic sclerosis?
GERD due to esophageal dysmotility ## Footnote Esophageal dysmotility leads to reflux symptoms.
164
What antibody is associated with systemic sclerosis?
Anticentromere antibody ## Footnote This antibody is often found in patients with limited scleroderma.
165
What does achalasia refer to?
Dysphagia with solids and liquids due to increased lower esophageal sphincter tone ## Footnote It results in difficulty swallowing and regurgitation.
166
What is the primary characteristic of acute lymphoblastic leukemia (ALL)?
Rapid proliferation of lymphoblasts in bone marrow ## Footnote This leads to anemia and thrombocytopenia.
167
What type of lymphoblasts are typical in preadolescents with ALL?
B lymphoblasts ## Footnote In adolescents and young adults, T lymphoblasts are more common.
168
What is the association between Down syndrome and leukemia?
Increased risk of AML and ALL/LBL ## Footnote Down syndrome is linked to poorer outcomes in ALL.
169
What cancers are associated with EBV?
Hodgkin disease, Burkitt lymphoma, nasopharyngeal carcinoma ## Footnote EBV is a known oncogenic virus.
170
What is the relationship between HIV and cancer?
Associated with NHL and Kaposi sarcoma ## Footnote HIV significantly increases the risk of certain malignancies.
171
What does immune thrombocytopenic purpura typically follow?
A URI in young children ## Footnote It presents with thrombocytopenia and petechiae but no significant bleeding.

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