KSAP volume 3 module 1 Questions with Correct
Answers
Family donor for someone with ADPKD? What kind of testing Correct
Answer-Direct mutation analysis:
- test the recipient: if positive check if the donor has the same mutation
- no point testing the donor first as there can be nonpathogenic mutations
of PKD1/2
Linkage analysis: needs 3 affected family across 2 generations
US: >10 cysts diagnostic, negative ultrasound (no cysts) <30year old is
only 94% sensitive for ADPKD due to a PKD1 mutation (and only 70%
sensitive for a PKD2 mutation)
CT/ MRI: >10 diagnostic; absence excluded the disease but 1-10?
diagnosis still in doubt
Lithium toxicity Correct Answer-Half life 12-60 hours
Acute:
Neuro/ cardiac manifestations
Chronic:
- nephrogenic DI
,- chronic tubulointerstitial nephropathy
- hypercalcemia, hypocalciuria, hyperparathyroidism
Li toxicity- indications for RRT Correct Answer-- serum level >4 in
AKI/CKD
or regardless of level
- altered mentation
- seizures
- dysrhythmias
>> intermittent HD
- extend till Li level <1
- minimum 6H
- check levels serially over subsequent 12H t identify rebound increase
that would require additional dialysis
** rebound can occur due to ongoing absorption from GI tract if
extended release form has been ingested
MPGN differentials Correct Answer-- lupus:
>>IgA and C1q n addition to IgG,IgM,C3 and k/l
>>"full house pattern"
- hep C
>> polyclonal immune complexes
,>> granular deposition of IgM, C2 and relatively equal k/l
>>IgG deposition is variable
- chronic bacterial
>> polyclonal IC deposition
Monoclonal Ig
>>monoclonal IC deposition
IF of the biopsy - differentials ? Correct Answer-- Ig positive,
complement positive
>> monoclonal: plasma cell dyscrasia
>> polyclonal: hep C
-Ig negative, complement pos
>> dense deposit disease/C3
- Ig neg, complement neg
>> TMA
C3 glomerulopathies Correct Answer-- dysregulation of the alternative
complement pathway; C3 deposition in absence of significant Ig
, - abnormal factor H - inhibitor of the alternative pathway, can lead to
unregulated complement activation, leading to C3 glomerulopathy
** C3 deposition without other significant immune deposition
Liddle syndrome Correct Answer-- HTN
- hypoK
- metabolic alkalosis
- suppressed renin, aldosterone not elevated
>> AD, early onset HTN
-activating mutation of ENaC > inc Na reabsorption
inc K secretion from principal cells
> H excretion via the H+ATPase at the apical membrane of the alfa
intercalated cells
> MC activity suppressed
Treatment: block ENAC with amiloride or triamterene
Urinary anion gap Correct Answer-Urine Na + K -Cl
If negative renal response is appropriate in setting of acidosis
Answers
Family donor for someone with ADPKD? What kind of testing Correct
Answer-Direct mutation analysis:
- test the recipient: if positive check if the donor has the same mutation
- no point testing the donor first as there can be nonpathogenic mutations
of PKD1/2
Linkage analysis: needs 3 affected family across 2 generations
US: >10 cysts diagnostic, negative ultrasound (no cysts) <30year old is
only 94% sensitive for ADPKD due to a PKD1 mutation (and only 70%
sensitive for a PKD2 mutation)
CT/ MRI: >10 diagnostic; absence excluded the disease but 1-10?
diagnosis still in doubt
Lithium toxicity Correct Answer-Half life 12-60 hours
Acute:
Neuro/ cardiac manifestations
Chronic:
- nephrogenic DI
,- chronic tubulointerstitial nephropathy
- hypercalcemia, hypocalciuria, hyperparathyroidism
Li toxicity- indications for RRT Correct Answer-- serum level >4 in
AKI/CKD
or regardless of level
- altered mentation
- seizures
- dysrhythmias
>> intermittent HD
- extend till Li level <1
- minimum 6H
- check levels serially over subsequent 12H t identify rebound increase
that would require additional dialysis
** rebound can occur due to ongoing absorption from GI tract if
extended release form has been ingested
MPGN differentials Correct Answer-- lupus:
>>IgA and C1q n addition to IgG,IgM,C3 and k/l
>>"full house pattern"
- hep C
>> polyclonal immune complexes
,>> granular deposition of IgM, C2 and relatively equal k/l
>>IgG deposition is variable
- chronic bacterial
>> polyclonal IC deposition
Monoclonal Ig
>>monoclonal IC deposition
IF of the biopsy - differentials ? Correct Answer-- Ig positive,
complement positive
>> monoclonal: plasma cell dyscrasia
>> polyclonal: hep C
-Ig negative, complement pos
>> dense deposit disease/C3
- Ig neg, complement neg
>> TMA
C3 glomerulopathies Correct Answer-- dysregulation of the alternative
complement pathway; C3 deposition in absence of significant Ig
, - abnormal factor H - inhibitor of the alternative pathway, can lead to
unregulated complement activation, leading to C3 glomerulopathy
** C3 deposition without other significant immune deposition
Liddle syndrome Correct Answer-- HTN
- hypoK
- metabolic alkalosis
- suppressed renin, aldosterone not elevated
>> AD, early onset HTN
-activating mutation of ENaC > inc Na reabsorption
inc K secretion from principal cells
> H excretion via the H+ATPase at the apical membrane of the alfa
intercalated cells
> MC activity suppressed
Treatment: block ENAC with amiloride or triamterene
Urinary anion gap Correct Answer-Urine Na + K -Cl
If negative renal response is appropriate in setting of acidosis
