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Q. Describe glomerular diseases that occur due to nonamyloid fibrillar deposits?

G. dis. é fibrillar deposits incl.: [amyloidosis, fibrillary G.N. & immunotactoid Gp.].
Other rare G. dis. incl.: fibronectin Gp. & collagenofibrotic Gp. Whereas all amyl-
oid fibrils are Congo-red +ve, all other forms of fibrillary deposition are ch.ch. by
Congo-red -ve fibrils. Fibrillary G.N. & immunotactoid Gp. are distinguished fr.
each other by E/M.; fibrils tht ch.ch. fibrillary G.N. are  smaller & typically
randomly oriented as opposed to the larger & organized fibrils of immunotactoid
Gp.. L.M. for both: variable & incl. focal or diffuse G.N. with or without crescent
formation, a membranous pattern, M.P. & amyloid-like pattern.
Ptn. é both fibrillary & immunotactoid dis. present é signif. NRP; other findings
incl.: microscopic hematuria, H.T. & R.I. tht may progress rapidly. Approximately
half of the ptn. dev. ESRD within 2-6 y.. Both diso. cn recur in R.Tx. transplant.
Immunotactoid Gp. is far less common thn fibrillary G.N.. Some ptn. é both
fibrillary G.N. & immunotactoid Gp. hv or will dev. lymphoproliferative diso. or
other malignancy. A careful search for a lymphoproliferative dis. & other
malignancies shd be part of the routine evaluation of ptn. é immunotactoid Gp.
Fibrillary & immunotactoid G. dis. are difficult to ttt & there’re no controlled
trials to guide thpy. Rituximab- hs bn ass. é complete or partial remission of
Prot in case rep., but evidence of efficacy fr. randomized trials is not available.
Ptn thpy directed é findings on L.M. (e.g. i.v. Cph & steroids for crescentic G.N.,
or Csp. or other reg. for those é M.N. features). Ptn. é fibrillary G.N. or immun-
otactoid Gp. ass. é CLL, related B cell lymphomas, or other diso. may respond to
therapy directed at the underlying disorder. DX. or R.Tx. cn be performed in ptns
progress to ESRD. Alth. recurrent dis. in the graft is common, rate of progression
is generally slower -thn native kidney. é × C +q -^^
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Q. 1. Describe various types of P.D.?
PD cn be performed in a continuous or intermittent fashion. Continuous amb-
ulatory peritoneal dialysis (CAPD) involves multiple exch. dur. day (us. 3) foll. by
an overnight dwell. Modification: one nighttime exch. é an exch. device, resulting
in [2 overnight exchanges + 3 exch. dur. day].
Automated P.D.(APD): consist of continuous cycler P.D. (CCPD), nightly inter-
mittent P.D. (NIPD) & tidal P.D. (TPD). CCPD: Long daytime dwell + several
cycles overnight. Minority of those under-going CCPD don’t hv daytime dwell &
others must hv daytime/mid-day exchange.
Some intermittent techniques, e.g. nightly intermittent P.D. (NIPD) or intermi-
ttent P.D. (IPD), hv ttt periods ("wet" abdomen) alternating e times dur. wch Pr.
cavity hs bn drained of Dzt ("dry" abdomen).Tidal P.D. (TPD): exch. in wch Pr.
cavity always contains at least some Dzt (us. 1/2 full) × improves comfort &
facilitates drainage in some ptns. Ptn. may or may not hv a daytime dwell.
Intermittent reg. typically utilize multiple short dwells & automated technology to
operate at near maximum solute clearance rates. Of the intermittent techniques,
TPD is infrequently used as it’s v. expensive (needs a large No. of Dzt) & techn-
ically difficult to perform. Incr. solute clearances hv also not bn confirmed in
ptns, despite findings in animal models. Most frequently, TPD is used to +drain-
age problems in selected ptns. As recommended in European Best Practice guidelines,
it cn also be used in those é inflow/outflow pain & in ptn.s é slow Pr. drainage.
Continuous flow P.D. (CFPD): another technique in wch two Pr. Cth. or one cth.
é two ports provide continuous inflow & outflow of Dzt. Since Dzt is constantly
refreshed, clearances similar to tht obtained é daily HDX cn be obtained. CFPD
ws 1
st
introduced decades ago, but ws subsequently abandoned because of
technical & financial limitations.There hs bn a renewed interest in this technique
because of incr. recognition of frequently inadequate clearances é other PD mod-
alities & the introduction of technical innovations tht may make CFPD feasible.
Q.2. Compare CAPD vs APD as P.D. modality?
CAPD & APD seem to provide similar clinical outcomes incl. M.R. Both modalities
× similar [mortality & hospitalization rates, risk of peritonitis, & fluid leaks]. APD
may be ass. é relatively more time for work & social ☺ activities. Relative
effect of CAPD & APD on Kru is controversial. Observational study showed:
higher risk of complete loss of R. function in 1
st
y. ass. é APD comp. to CAPD.
However, no difference ws detected in the systemic review é this study.
*********************************************************** é ×
Q.3. Explain how to start P.D. prescription?
At the start of DX when ptn hv some Kru , most ptns cd do any PD technique
and us. be able to achieve Kt/V & vol. control targets. However, once Kru is
negligible, modality choice may need to match Pr. transport ch.ch. to optimize
solute clearance & U.F. It’s important to individualize ptn’s dwell times & vol.,
so tht target dose of PD cn be achieved. PD is considered adequate in most ptn if
weekly total Kt/V for urea is at least 1.7 & if considering Cr. Cl.: weekly cr. Cl.:
at least 50-60 L/w./1.73 m2 B.S.A. (é some variation based upon transporter status) .
Attainment of these goals must be documented & then monitored over time. It’s
often necessary, to incr. ptn’s DX prescription (by qNo. of exch. or dwell vol.) as
Kru is lost or there’s a decr. in net permeability of Pr. membrane.
Q. 4. What is the role of peritoneal equilibration test in P.D. prescription? é ×
1
st
step in optimizing PD prescription is to determine Pr membrane transport
ch.ch. using PET Based upon published norms. The dwell time is then matched
to transport type in effort to qdaily clearances & U.F. while + gluc. absorption.
Rapid transporters : Consider, the problem in "rapid" transporters of cr. fr. bld to
Dzt. They achieve almost total equilibration betw. pl. & Dzt for urea & cr. in a
few h.s. They also are rapid absorbers of Dzt glucose× removing the osmotic
stimulus to U.F.. Net effect =they begin to absorb Dzt with the glucose leading,
after 2-3h. h., to reductions in U.F. vol. & net solute clearance (as the solutes tht
hv diffused into Dzt are also absorbed back into systemic circulation).. In this setting,
standard CAPD, wch utilizes prolonged dwell times, might not produce sufficient
fluid or solute removal. This wd necessitate more frequent use of hypertonic Dzt
(2.5 or 4.25 % dextrose) , potentially inducing hyperglycemia, hypertriglyceridemia,
and/or wt gain fr. q glucose absorption, or icodextrin. A better alternative in
many cases is multiple short dwells é standard Dzt as é NIPD.
Slow transporters: Us. need long dwell times to adequately remove small
solutes. U.F. is not a problem, since glucose is also slowly absorbed.
Q. 5. What is the effect of residual renal function on P.D. prescription?
Ptns typically hv some Kru when they’re started on maintenance DX. In this
setting, it may not be as important to match dwell time é transporter type, since
the ur. vol. cn replace the need for U.F. & the minimal renal solute excretion
augments tht due to DX. However, as Kru gradually decr. over time, matching
of dwell time to transport type becomes increasingly important.
Q. 6. How to choose of P. D. modality?
Most ptn. on maintenance PD need continuous DX é CAPD or CCPD to achieve
adequate weekly solute clearances. CAPD cn be used, é typical minimum DX of
4, 2 L. exch./d., incl. one overnight exch.. Standard reg. cn be modified by
changing either dwell time, vol. or No. of exch./d. Exch. are performed manually.
CCPD is an automated form in wch a cycler delivers 3-6 exch. while ptn. sleeps
é 12-15 h. daytime dwell. Weekly clearances are similar to CAPD but more Dzt
fluid is required each day. U.F. rates may be lower é automated forms of thpy.
We us. let ptn. choose betw. CAPD & CCPD based upon lifestyle or personal
issues (desire to work, wish to not do any exch. dur. day, or, in a ptn who is not
able to do exch. themselves). CCPD provided signif. more time for work, family
& social activities thn CAPD. Sleep apnea may be more responsive to NPD.
Further recomm. are made after DX dose hs bn documented é 24 h. Dzt
collection for vol. & cr. Cl. or Kt/V, or it’s determined fr. PET test.
Ptns shd be monitored for loss of Kru. The ass. fall in solute clearance cn us. be
reversed by qDX dose via an q dwell vol. or No. of exch./d.. Either of these
modalities will qnet Dzt flow rate, thereby qclearance of urea & other small
solutes by keeping concentration gradient for solute diffusion . APD is superior
to CAPD in optimizing fluid & Ssc in some ptns . This’s because automated
techniques cn combine larger dwell vol., long nocturnal sessions, and add
daytime exch., thereby moderately q solute & fluid removal.
Body surface area : In addition to transport type, Kru & life style choice, another
f. to consider when prescribing PD is BSA because both Kt/V & weekly Cr.cl.
must be normalized to body size. For example, an absolute cr. cl. of 60 L/w.
represents different degrees of dialytic solute removal in small vs large ptns.
Large ptn typically need high Dzt. vol., esp. é slow transporters & hv no Kru. é
CAPD, dwell volume is typically 2.5-3 L. & some ptns require 5 exch./d..
In CCPD, ptn. us. need 12-14 h. of dwell time while on the cycler, using large
instilled vol. of Dzt; an alternative is a shorter cycler time (9-11 h.) +1-2 day-
time exch.. A large instilled vol. is also required é daytime dwell & some ptn.
need to perform one manual CAPD exch. to meet the goal for Ssc. However,
qnightly Dzt flow may be superior to adding a manual daytime exch..
Intermittent PD: Use of intermittent therapies hs evolved as we hv become
better able to match dwell time & vol. to transport characteristics. Rapid
transporters, do best é short dwells & may initially achieve adequate clearan-
ces é "dry" day option on CAPD. However, they may need to change their pres-
cription to IPD if they lose their Kru. If NIPD is used é 8-12 h. DX, the dwell
time may be only 1-2 h./exch. é 1.5-2.0 L dwell vol.s. This requires 8-20 L. of
Dzt fluid/d.. Adequate solute clearances cn be achieved é this reg. even though
ptn. hs a "dry" day. However, unless Kru is signif., most ptns need a "wet" day.
10-15 % of ptn might do best é NIPD or daytime DAPD. DAPD is similar to
NIPD except tht exchanges are performed manually dur. the day.
Classic IPD: a form of DX. in wch multiple short exch. are performed on inter-
mittent basis. A typical reg. of 12-15 two L., one h. dwells performed 3-4 d./w..
Over 50 h. us. required to attain clearances similar to CAPD. However, higher
target doses of DX are recommended because of the peaks in BUN occ. on off-
DX d.s. For these reasons, this form of PD is not recommended for chronic use.

Some intermittent techniques. Intermittent reg. 1/2 full)  improves comfort & facilitates drainage in some ptns.g. drainage. (TPD): exch. (NIPD) or intermittent P. Cl.D.2.There hs bn a renewed interest in this technique because of incr. it cn also be used in those é inflow/outflow pain & in ptn. Most frequently. wch Pr.D. Continuous flow P.D. Cl. M.A.R. transport ch. CFPD ws 1st introduced decades ago. However. *********************************************************** é  Q. risk of peritonitis. function in 1st y. modality? CAPD & APD seem to provide similar clinical outcomes incl.F. Compare CAPD vs APD as P.3. but ws subsequently abandoned because of technical & financial limitations. TPD is infrequently used as it’s v. (CFPD): another technique in wch two Pr. recognition of frequently inadequate clearances é other PD modalities & the introduction of technical innovations tht may make CFPD feasible. prescription? At the start of DX when ptn hv some Kru . ass./1. expensive (needs a large No.D.. Relative effect of CAPD & APD on Kru is controversial. be able to achieve Kt/V & vol.ch. clearances similar to tht obtained é daily HDX cn be obtained. so tht target dose of PD cn be achieved. of Dzt) & technically difficult to perform.: at least 50-60 L/w. despite findings in animal models. é relatively more time for work & social ☺ activities. cavity always contains at least some Dzt (us. As recommended in European Best Practice guidelines.S. It’s important to individualize ptn’s dwell times & vol. cavity hs bn drained of Dzt ("dry" abdomen). Incr. (é some variation based upon transporter status) . & fluid leaks]. may or may not hv a daytime dwell. most ptns cd do any PD technique and us. solute clearances hv also not bn confirmed in ptns. Explain how to start P. TPD is used to drainage problems in selected ptns. to CAPD.D. e. Ptn. Attainment of these goals must be documented & then monitored over time. PD is considered adequate in most ptn if weekly total Kt/V for urea is at least 1. APD may be ass. once Kru is negligible. Of the intermittent techniques. Q. control targets.s é slow Pr. Since Dzt is constantly refreshed. It’s .7 & if considering Cr. modality choice may need to match Pr. nightly intermittent P. (IPD).73 m2 B. é APD comp. Cth. in wch Pr.Tidal P. to optimize solute clearance & U. typically utilize multiple short dwells & automated technology to operate at near maximum solute clearance rates. However. Both modalities  similar [mortality & hospitalization rates. or one cth. é two ports provide continuous inflow & outflow of Dzt. hv ttt periods ("wet" abdomen) alternating e times dur. Observational study showed: higher risk of complete loss of R.: weekly cr.D. no difference ws detected in the systemic review é this study.

However. wch utilizes prolonged dwell times. and/or wt gain fr. standard CAPD. How to choose of P. 6. D. CAPD & CCPD based upon lifestyle or personal issues (desire to work. U..F. the problem in "rapid" transporters of cr.s. U. after 2-3h. exch. They also are rapid absorbers of Dzt glucose removing the osmotic stimulus to U. fr. while ptn. since glucose is also slowly absorbed. or No. vol. potentially inducing hyperglycemia. Rapid transporters : Consider./d. bld to Dzt. modality? Most ptn. membrane. Slow transporters: Us.F.D. What is the effect of residual renal function on P. let ptn. In this setting. Q. to reductions in U. on maintenance PD need continuous DX é CAPD or CCPD to achieve adequate weekly solute clearances.25 % dextrose) . choose betw. prescription? Ptns typically hv some Kru when they’re started on maintenance DX. or. cn be modified by changing either dwell time. Q. sleeps é 12-15 h.F. over time. it may not be as important to match dwell time é transporter type. hypertriglyceridemia. & net solute clearance (as the solutes tht hv diffused into Dzt are also absorbed back into systemic circulation). prescription? é  1st step in optimizing PD prescription is to determine Pr membrane transport ch.) as Kru is lost or there’s a decr. A better alternative in many cases is multiple short dwells é standard Dzt as é NIPD. need long dwell times to adequately remove small solutes.. vol. This wd necessitate more frequent use of hypertonic Dzt (2. wish to not do any exch. 5. in net permeability of Pr. In this setting. CCPD is an automated form in wch a cycler delivers 3-6 exch.D. or dwell vol. matching of dwell time to transport type becomes increasingly important.often necessary. in a few h.. daytime dwell. day. of exch. or icodextrin. 2 L. Exch. rates may be lower é automated forms of thpy. as Kru gradually decr. vol. é typical minimum DX of 4. Q. Net effect =they begin to absorb Dzt with the glucose leading. is not a problem. & the minimal renal solute excretion augments tht due to DX. in a ptn who is not . ptn’s DX prescription (by No. of exch. 4. while  gluc.F.. absorption.F. Weekly clearances are similar to CAPD but more Dzt fluid is required each day. dur. Standard reg. since the ur. We us.  glucose absorption. to incr. are performed manually./d.F. incl. h.ch. The dwell time is then matched to transport type in effort to daily clearances & U. What is the role of peritoneal equilibration test in P. one overnight exch. might not produce sufficient fluid or solute removal. & Dzt for urea & cr.5 or 4. using PET Based upon published norms. They achieve almost total equilibration betw. pl. cn replace the need for U.. CAPD cn be used.

5-3 L. The ass. us. even though ptn. be reversed by DX dose via an  dwell vol. this form of PD is not recommended for chronic use. are made after DX dose hs bn documented é 24 h. In CCPD. APD is superior to CAPD in optimizing fluid & Ssc in some ptns . of exch. This requires 8-20 L./exch. However. on offDX d. Intermittent PD: Use of intermittent therapies hs evolved as we hv become better able to match dwell time & vol. If NIPD is used é 8-12 h. and add daytime exch. to meet the goal for Ssc.s. or Kt/V. cl. must be normalized to body size. represents different degrees of dialytic solute removal in small vs large ptns. us. Large ptn typically need high Dzt./d. Either of these modalities will net Dzt flow rate. of dwell time while on the cycler. do best é short dwells & may initially achieve adequate clearances é "dry" day option on CAPD... more time for work. nightly Dzt flow may be superior to adding a manual daytime exch./d. A large instilled vol. required to attain clearances similar to CAPD. an alternative is a shorter cycler time (9-11 h... . unless Kru is signif. For example. using large instilled vol. PET test.) +1-2 daytime exch. é CAPD. Rapid transporters. an absolute cr. esp. Dzt collection for vol. & some ptns require 5 exch. Further recomm. to transport characteristics. to consider when prescribing PD is BSA because both Kt/V & weekly Cr. family & social activities thn CAPD. dwells performed 3-4 d. However. thereby clearance of urea & other small solutes by keeping concentration gradient for solute diffusion .able to do exch. Adequate solute clearances cn be achieved é this reg.. A typical reg. Over 50 h. fall in solute clearance cn us. For these reasons. the dwell time may be only 1-2 h. are performed on intermittent basis. higher target doses of DX are recommended because of the peaks in BUN occ. ptn. CCPD provided signif. of 12-15 two L.. However. is also required é daytime dwell & some ptn. However.s. themselves). Ptns shd be monitored for loss of Kru. DX. the day. Cl. another f. or No. one h. é 1.. é slow transporters & hv no Kru. need to perform one manual CAPD exch. of Dzt fluid/d. most ptns need a "wet" day. of Dzt. dwell volume is typically 2. Classic IPD: a form of DX. they may need to change their prescription to IPD if they lose their Kru. thereby moderately  solute & fluid removal. This’s because automated techniques cn combine larger dwell vol. long nocturnal sessions.. of 60 L/w.cl. or it’s determined fr. in wch multiple short exch. Sleep apnea may be more responsive to NPD. vol. 10-15 % of ptn might do best é NIPD or daytime DAPD. hs a "dry" day. Body surface area : In addition to transport type. need 12-14 h. DAPD is similar to NIPD except tht exchanges are performed manually dur. & cr.5-2.0 L dwell vol.. Kru & life style choice./w...