Mansoura University Children’s Hospital
2014
Protocols of management of Nephrotic syndrome in childrenPediatric Nephrology Unit
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Table of content:
1. Definitions and inclusion criteria.2. Symptomatic management of nephrotic syndrome (NS).3. Renal biopsy indications and precautions.4. Protocols for steroid responsive NS.5. Protocols for steroid resistant NS.6. Protocols for membranous nephropathy.7. Protocols for IgA nephropathy.
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Definitions and inclusion criteriaCriteria for diagnosis of Nephrotic syndrome: First AM urine protein/creatinine (Up/cr) ratio >2 Essential for diagnosis Hypoalbuminemia –Serum albumin <2.5g\dl.
± Edema. Hyperlipedemia.
SDNS : Relapse during maintenance or within 2 weeks after the stoppage of treatment.FRNS: Two or more relapses within 6 months.Infrequent relapse: One relapse within 6 months of initial response, or one to three relapses in any 12-month period.Steroid resistant Nephrotic syndrome: (SRNS) Lack of complete remission despite treatment with prednisolone at a dose of
2 mg/kg/day (60 mg/m2/day) for 4 weeks. Lack of remission after 4 weeks ….at the first episode of nephrotic syndrome.
Late resistance Patients who are steroid sensitive initially, but show steroid resistance during
a subsequent relapse.Complete remission: Negative/trace Albustix result on first AM urine specimens for 3 consecutive
days with quantitative (Up/cr <0.2) confirmation in one of the samples.Partial remission:
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
No Edema >50% reduction in first AM Up/cr from presenting ratio and absolute Up/cr
(0.2-2)(non nephrotic proteinuria) Relapse: Edema Recurrence of ≥ 2+ proteinuria by urine dipstick on first AM urine specimens
for 3 consecutive days within 1 week in absence of URTI, with quantitative confirmation of nephrotic-range proteinuria (Up/cr >2.0) in one of the samples.
Indications of renal biopsy at initial presentation (atypical NS): Gross hematuria. Elevated serum creatinine with absence of prerenal etiology. Low complement level (C3). Extra-renal symptoms such as malar rash or purpura. Age less than 1 year and der than 10 years of age. Sustained hypertension (persistent elevation of blood pressure above 95th
percentile for age). Patient relapsed during first 2 weeks of maintenance therapy
(45mg/m2/EOD).
All patients with atypical NS will start full dose steroid therapy till result of biopsy
All drug doses should be calculated using patient’s dry weightDry weight: --- last known body weight before developing edema if unknown ………….use weigth for height
Available oral prednisone/prednisilone preparations:Hostacortin 5mg/tabletSolupred 5mg/tabletSolupred 20mg/tablet
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Prednisolone 5mg/tabletPredsol syrup 1 mg/mlPredsol forte syrup 3mg/mlXilone syrup 1mg/mlXilone forte syrup 3mg/mlInitial therapy:
Initial prednisone therapy is 60 mg/m 2 per day for6 weeks. 45 mg/m 2/ EOD for 6 weeks(if patient relapsed during first 2 weeks of this
step consider renal biopsy).
30mg/m2/EOD for 2 weeks.
15mg/m2/EOD for 2 weeks.
Induction Therapy for Initial Idiopathic Nephrotic Syndrome Name:…………………… Age: sex: dry weight :
Induction order: Date / /20 SA:
First Week/ /
Second Week/ /
Third Week/ /
Fourth Week/ /
60 mg/m2/d 60 mg/m2/d 60 mg/m2/d 60 mg/m2/d
Dose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
Assessment at end of 4 weeks:Clinical: BW: oedema: blood pressure:Laboratory: U/A : Pr/cr: serum albumin:Decision: : SSNS& continue week 5, 6 SRNS for biopsy
Fifth Week/ /
Sixth Week/ /
60 mg/m2/d 60 mg/m2/dDose: ……………….. Dose: ……………
Available oral prednisone/prednisilone preparations:
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Hostacortin 5mg/tabletSolupred 5mg/tabletSolupred 20mg/tabletPrednisolone 5mg/tabletPredsol syrup 1 mg/mlPredsol forte syrup 3mg/mlXilone syrup 1mg/mlXilone forte syrup 3mg/mlDisprelone 20mg/tablet
Standard Maintenance for Initial Idiopathic Nephrotic Syndrome Name:…………………… Age: sex: dry weight :
Maintenance order: Date / / 20 SA:
Week 1/ /
Week 2/ /
Week 3/ /
Week 4/ /
45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
If patient relapsed during these 2 weeks consider renal biopsy
Week 5/ /
Week 6/ /
45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ………………..
Week 7/ /
Week 8/ /
30 mg/m2/EOD 30 mg/m2/EODLast updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Dose: ………………. Dose: ………………..
Week 9/ /
Week 10/ /
15mg/m2/EOD 15 mg/m2/EODDose: ………………. Dose: ………………..
Maintenance therapy for initial idiopathic nephrotic syndrome 45 mg/m 2/ EOD for 6 weeks.
30mg/m2/EOD for 2 weeks.
15mg/m2/EOD for 2 weeks.
First relapse and infrequent relapse Steroid therapy of 60 mg/m 2 per day until proteinuria disappeared for 4-5
days(confirmed by 3 urine analysis). A maximum period of 4 weeks on 60mg/m2 /day if no remission …….late steroid resistant.
Alternate day prednisone of 45 mg/m 2for an additional six weeks and tapering of 15mg/m2 every 2 weeks.
Name:…………………… Age: sex: dry weight :
Induction: 60mg/m2/d till proteinuria disaapear: max 4 weeksDate / /20 SA:
First Week/ /
Second Week/ /
Third Week/ /
Fourth Week/ /
60 mg/m2/d 60 mg/m2/d 60 mg/m2/d 60 mg/m2/d
Dose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
Maintenance therapy: Date / /20 SA:Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Week 1/ /
Week 2/ /
Week 3/ /
Week 4/ /
45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
Week 7/ /
Week 8/ /
30 mg/m2/EOD 30 mg/m2/EODDose: ………………. Dose: ………………..
Management of SDNS and FRNSA
1- Steroid induction (60mg/m2/day) until proteinuria has disappeared for four to five days (confirmed by 3 urine analysis)………..start maintenance
2- Low dose steroid (15 mg/m2 EOD) for 6 months.If relapse occurs
B
1- Steroid induction (60mg/m2/day) until proteinuria has disappeared for four to five days (confirmed by 3 urine analysis)………..start maintenance
2- Low dose steroid (30 mg/m2 EOD) for 6 months.If relapse occurs
C
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Week 5/ /
Week 6/ /
45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ………………..
Week 9/ /
Week 10/ /
15mg/m2/EOD 15 mg/m2/EODDose: ………………. Dose: ………………..
جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
1- Steroid induction (60mg/m2/day) until proteinuria has disappeared for four to five days (confirmed by 3 urine analysis)………..start maintenance
2-Levamizol protocol (2.5 mg/kg/EOD max dose 150mg/d for 12months) Start at beginning of steroid maintainence .(Follow up CBC every 2 weeks for risk of neutropenia).3. Steroid therapy maintenance:Tapering over 1 year
D
MMF protocol
If relapse occurs
Renal Biopsy
Others IdiopathicRegardless the pathological type of idiopathic NS
E
Calcineurin inhibitors F
Cyclophosphamide
Induction Therapy for SSSDNS & SSFRNS (60mg/m2/day) until proteinuria disappeared(max 4 weeks) for 4-5 days
(confirmed by 3 urine analysis)………..start maintenance
Patient Name:………………………………………Age : Sex : Dry weigth:Induction order: Date / /20 SA:
First Week
/ /
Second Week
/ /
Third Week
/ /
Fourth Week
/ /
60 mg/m2/d 60 mg/m2/d 60 mg/m2/d 60 mg/m2/d
Dose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Induction order: Date / /20 SA:First Week
/ /
Second Week
/ /
Third Week
/ /
Fourth Week
/ /
60 mg/m2/d 60 mg/m2/d 60 mg/m2/d 60 mg/m2/d
Dose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
Induction order: Date / / 20 SA:First Week
/ /
Second Week
/ /
Third Week
/ /
Fourth Week
/ /
60 mg/m2/d 60 mg/m2/d 60 mg/m2/d 60 mg/m2/d
Dose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
Protocol of Maintenance Therapy for SD &FRNS(Maintenance A)
Patient Name:………………………………………Age : Sex : Dry weight:
Maintenance order: Date / /20 SA:
Week 1/ /
Week 2/ /
Week 3/ /
Week 4/ /
45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Week 5/ /
Week 6/ /
45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ………………..
Low dose steroid 15mg/m2/EOD for 6 monthsWeek 9-12
/ /Week 13-16
/ /Week 17-20
/ /Week 21-24
/ /15 mg/m2/EOD 15 mg/m2/EOD 15 mg/m2/EOD 15 mg/m2/EOD
Dose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
Protocol of Maintenance Therapy for SD &FRNS(Maintenance B)
Patient Name:………………………………………Age : Sex : Dry weight:
Maintenance order: Date / / 20 SA:
Week 1/ /
Week 2/ /
Week 3/ /
Week 4/ /
45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
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Week 7/ /
Week 8/ /
30 mg/m2/EOD 30 mg/m2/EODDose: ………………. Dose: ………………..
Week 25-28/ /
Week 29-32/ /
15 mg/m2/EOD 15 mg/m2/EODDose: ………………. Dose: ………………..
جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Week 5/ /
Week 6/ /
45 mg/m2/EOD 45 mg/m2/EODDose: ………………. Dose: ………………..
Low dose steroid 30mg/m 2 /EOD for 6 months Week7-10
/ /Week 11-14
/ /Week 15-18
/ /Week 19-22
/ /30 mg/m2/EOD 30mg/m2/EOD 30 mg/m2/EOD 30 mg/m2/EOD
Dose: ………………. Dose: ……………….. Dose: ……………….. Dose: ……………
Then 15mg/m 2 /EOD for 2 weeks
Protocol of Maintenance Therapy for SD &FRNS(Maintenance C)
Patient Name:………………………………………Age : Sex : Dry weight:
1. Maintenance steroid therapy:Week 1/ /
Week 2/ /
Week 3/ /
Week 4/ /
Week 5/ /
Week 6/ /
45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD 45 mg/m2/EOD
Dose: …………. Dose: …………….. Dose: …………….. Dose: …………… Dose: …………… Dose: ……………
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Week 23-26/ /
Week 27-30/ /
30 mg/m2/EOD 30 mg/m2/EODDose: ………………. Dose: ………………..
Week 31-32/ /
15mg/m2/EODDose: ……………….
جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Week 9-12/ /
Week 13-16/ /
Week 17-20/ /
Week 31-24/ /
Week 25-28/ /
Week 29-32
/ /15 mg/m2/EOD 15 mg/m2/EOD 15 mg/m2/EOD 15 mg/m2/EOD 15 mg/m2/EOD 15
mg/m2/EODDose: …………. Dose: …………….. Dose: …………….. Dose: …………… Dose: …………… Dose:
……………
2. Levamizole2.5mg/kg/EOD for 12 months(start with start of maintenance)
Week 1-4/ /
Week 5-8/ /
Week 9-12/ /
Week 13-16/ /
Week 17-20/ /
Week 21-24
/ /2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5
mg/kg/EODDose: …………. Dose: …………….. Dose: …………….. Dose: …………… Dose: …………… Dose:
……………
Week 25-28/ /
Week 29-32/ /
Week 33-36/ /
Week 37-40/ /
Week 41-44/ /
Week 45-48
/ /2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5 mg/kg/EOD 2.5
mg/kg/EODDose: …………. Dose: …………….. Dose: …………….. Dose: …………… Dose: …………… Dose:
……………
Levamizole available preparations:Katrex 40mg/tab Katrex syrup (8mg/ml)Wormine syrup 8mg/ml
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Week 7/ /
Week 8/ /
30 mg/m2/EOD 30 mg/m2/EODDose: ………………. Dose: ………………..
Week 33-36/ /
Week 37-40/ /
Week 41-44/ /
Week 45-48/ /
7.5 mg/m2/EOD 7.5 mg/m2/EOD 7.5 mg/m2/EOD 7.5 mg/m2/EOD
Dose: …………. Dose: …………….. Dose: …………….. Dose: ……………
جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Protocol of Mycophenolate Mofetil (MMF) in FRNS & SDNSPatient Name:………………………Age: sex:1. Full dose steroid induction (60mg/m2/day) till proteinuria disappears.2. Maintenance steroid + MMF:
Date Dry wt
SA Oral Pred.maintenance
MMF
Week 1-2
Week 3-4Week 5-6
Week 7-8
Week 9-10
Week 11-124th month5th month6th month7th month8th month9th month10th month11th month12th month
1- Concurrent Oral Prednisone: a. 1st week- 6th week : 45 mg/m2/ EODb. 7th -8th week : 30 mg/m2/ EODc. 9th - 10th week : 15 mg/m2/ EOD d. 11th week-12th month 7.5mg/m2 /EOD 2- MMF:*Initial starting dose: 300- 400 mg/m2/ dose BID (max.2 gm/day) *Maintenance dose: 600 mg/m2/dose BID. It should be started after initial starting dose by 2 weeks if WBC counts not showing leucopenia.
A-Reassess proteinuria after two months of (MMF) * partial or complete remission continues the protocol for 12 months.
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
* No remission Stop the protocol.
B-Available preparations: Cellcept Capsules, 500 mg, and 250 mg, Mofetil tablets 500mg, 250 mg Myfortic tablets 180, 360 mg
Protocol of Cyclosporine in SDNS, FRNS page 1
Patient Name:……………………… Age: 1. Full dose steroid induction (60mg/m2/day) till proteinuria disappear2. Maintenance steroid + Cyclosporine:Induction (3-6 months) 1- Cyclosporine Maintenance (3-5 years)
Date Dry Wt Dose Date Dry Wt Dose1st -2nd week / / …………
……
1-2 weeks / /
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
……………………
……
Serum level & adjust dose Serum level & adjust dose
3rd -6th week7th -10th week11th-12th week
/ / / /
/ /
…………………………………………
……
3rd-6thweek7th-10thweek11th-12thweek
/ // // /
4th month
5th month
6th month
9th month
12th month
15th month
18thmonth
21thmonth
24thmonth
27thmonth
30thmonth
33thmonth
36thmonth
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
Reassess :*Partial or complete remission: start maintenance*No partial or complete remission: continue induction for another 3 months
13th -16th week
17th -20th week
21th-24th week
/ /
/ /
/ /
…………
…………
…………
…………
……
Reassess :*Partial or complete remission: start maintenance*No partial or complete remission: stop protocol
“cyclosporine resistant”
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
……………………
……………………
……
Reassess :*Sustained complete remission during maintenance: ……………stop protocol*Partial remission continue for another 2 years
37-42 month
43-48 month
49-54 month
55-60n month
Second renal biopsy to asses nephrotoxicity
*** 2- Concomitant maintenance steroid therapy page 2Week 1-6 / / Week 7-8 / / Week 9-14 / /
SA:………….Dose:-----------------------
SA:………….Dose:-----------------------
SA:………….Dose:-----------------------
45mg/m2/ EOD 30mg/m2/ EOD 15mg/m2/ EODWeek 15-24 / / Month 7-12 / / Month 13-18 / /
SA:………….Dose:-----------------------
SA:………….Dose:---------------------
SA:………….Dose:-----------------------
7.5 mg/m2/ EOD 7.5 mg/m2/ EOD 7.5 mg/m2/ EODMonth 19-24 / / Month 25-30 / / Month 31-36 / /
SA:………….Dose:---------------------
SA:………….Dose:---------------------
SA:………….Dose:-----------------------
7.5 mg/m2/ EOD 7.5 mg/m2/ EOD 7.5 mg/m2/ EOD
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Follow up during protocol Serum creatinine Serum sandimmune Urine analysis
Date Value Date Value
Protocol of Tacrolimus (TAC) In SDNS, FRNS Patient Name:……………………………….……Age: … Sex:………....……….……………
1. Full dose steroid induction (60mg/m2/day) till proteinuria disappear2. Maintenance steroid + Tacrolimus
Date Dr SA TAC dose Oral Pred. Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
y wt
dose
week 1-2/ /
Serum level of TAC and adjust dose
week 3-4 / /Week 5-6Week 7-8 / /3rd month / /
Serum level of TAC and adjust dose
4th month / /5th month / /6th month / /
Serum level of TAC and adjust dose
7th month / /8th month / /9th month / /
Serum level of TAC and adjust dose
10th month
/ /
11th month
/ /
12th month
/ /
1. TAC:- -starting dose 0.1mg/kg/d divided in 2 doses for one year. -can dose up to 0.3mg/kg/d till reaching serum level of TAC: (9.8-19.4ng/ml) by the whole blood ELIZA assay or (0.5-1.5ng/ml) by serum high pressure liquid chromatography.-available preparation prograph 0.5mg, 1mg, 5mg Cap .
2. Oral Prednisone (Concomitant with TAC): -45mg/m2/EOD for 6weeks-30mg/m2/EOD for 2weeks-15mg/m2/EOD for 2weeks
3. Precautions: Before starting TAC, ALB/cr ratio, Serum Cr, Albumin , CBC, OGTT. Serum level of TAC done 2 week after starting treatment then / 3 m. F.U the patient each 2 weeks in 1st month then once monthly.
4. Relapse on TAC: add oral prednisone 60mg/m2/d till complete remission , tapering over two
months. Protocol of Cyclophosphamide for SDNS, FRNS
Patient Name:………………………SA: Age: dry weigth : Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
1. Full dose steroid induction (60mg/m2/day) till proteinuria disappear2. Maintenance steroid + Cyclophosphamide:
Cyclophosphamide monthly doses ( 500mg/m 2 monthly)
1st Month/ /
2nd Month/ /
3rd Month/ /
SA:-------------------
Cycloph.Dose:-----------------------
SA:--------------
Cycloph.Dose:-------------------
SA:-------------------
Cycloph.Dose:-----------------------
4th Month/ /
5th Month/ /
6th Month/ /
SA:-------------------
Cycloph.Dose:-----------------------
SA:-----------------
Cycloph.Dose:----------------------
SA:-------------------
Cycloph.Dose:-----------------------
Cumulative dose of Endoxan(168 mg/kg/protocol)
Date Total cumulative dose Date Total cumulative dosemg/kg mg/kgmg/kg mg/kgmg/kg
Maintenance steroid therapyWeek 1-6 / / Week 7-8 / / Week 9-14 / /
Dose:----------------------- Dose:----------------------- Dose:-----------------------
45mg/m2/ EOD 30mg/m2/ EOD 15mg/m2/ EOD
Then 7.5mg/m2/EOD for 6 months1st -2nd month / / 3rd -4th month / / 5th -6th month / /Dose:----------------------- Dose:----------------------- Dose:-----------------------
7.5mg/m2/ EOD 7.5 mg/m2/ EOD 7.5mg/m2/ EOD
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Rituximab protocol for SDNS, FRNS Indications:
1. Multiple drug resistance(cyclosporine, mycofenolate mofetil, tacrolimus, cyclophosphamide).
2. Severe adverse effects of other immune suppressives.
Contra-indications to rituximab therapy: 1. Anaphylaxis to rituximab.2. Relative contra-indications:
*Allergy to rituximab- infusion reactions are common and can usually be managed with pre-medication and slow infusion
*Pneumonitis- due to risk RALI (Rituximab associated lung injury).
Side effects of rituximab: Infusion reaction Late adverse effects
Sore throatWheezing, cough, DyspneaFeverSkin rashNausea , vomitingBradycardia\ tachycardiaHypertension\Hypotension.Nasal stiffnesLeg pain.
SepsisGranulocytopeniaLiver failurefever
Baseline, pre-treatment prior to first dose: Test Date ResultDEXAOphthalmology assessment for cataractsViral serology: HIV, HBsAg, HBsAb, HBcAb, HCVPolyomavirus PCR (Blood)Renal biopsySerum creatinineSerum calcium/phosphorusUrine protein/creatinine ratioLiver function testCD19 (peripheral blood)CD20 (peripheral blood)Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Complete blood picture
Premedications: (30-60 minute before each infusion): 1. Prednisolone 0.5mg/kg2. Avil: intravenous/8 hours.3. Paracetamole:
10-15mg/kg/dose/6 hours intravenous.4. Withhold or decrease dose of antihypertensive medications 12
hours before dose for risk of hypotension.
Sulfamethoxazole/trimethoprime prophylaxis: (5mg/kg to max 160/800mg 3 days per week) for 6 months.
Available preparations: MabThera ampoule 500mg/50mlRituxan ampoule 100mg/10mlRituximab 100mg/10ml, 500mg/50ml.
Preparation of rituximab(just before infusion): Just before administration, rituximab should be diluted with 0.9% sodium
chloride injection to a final concentration of 1-4 mg/mL. A 1-mg/mL dilution(each 1ml of Rituximab diluted in 10 ml saline) is
preferable to facilitate adjustments in the infusion and to avoid adverse effects.
Rituximab should not be mixed with other medications or i.v. fluids because there is a lack of compatibility data.
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Prepared infusions are stable in polyvinyl chloride or polyethylene bags at 2-8 C (36-46 °F) for 24 hours and at room temperature for an additional 12 hours.
Unused portions of undiluted drug must be discarded because of the absence of a preservative.
Infusion of rituximab: 1. Central or peripheral i.v. catheter.2. Should never be given by i.v. push or bolus injection.3. Blood pressure and temperature monitoring /hour during and 24
hours after infusion.4. Infusion over 6-8 hours(Gradual increase rate of infusion every hour
if no infusion reaction)(10ml/min 1st hour, 20ml/min 2nd hour, 30 ml/min 3rd , 40ml/min 4th hour then 50 ml/min from 5th hour to end of infusion).
Dose of rituximab in SDNS: 375mg/m2/dose weekly for 4 doses (max 500mg/dose):
1. If B cell depletion us confirmed on day 5-7 of first infusion (CD 19<1%), No more doses.
2. If B cell depletion not achieved after first infusion repeat infusion till B cell depletion is achieved for maximum 4 doses
Week 1 Week 2 Week 3 Week 4Date: Date: Date: Date:
Dose: mg Dose: mg Dose: mg Dose: mgDay 5 CD19:(Target <1%)
Day 5 CD19:(Target <1%)
Day 5 CD19:(Target <1%)
Day 5 CD19:(Target <1%)
Day 7protein/creatinine:
Day 7protein/creatinine:
Day 7protein/creatinine:
Day 7protein/creatinine:
Weeks 5-52: Monthly check liver function test, creatinine, Ca, Phos, (CD19), Immunoglobulins, urine microscopy and Pr:Cr.
Other immunsuppresive therapy can be tapered gradually and may be completely stopped after 6 months of Rituximab.
Relapse after Rituximab associated with B cell reconstitution during initial 12 months Rituximab therapy:
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Bring patient into remission first by steroids then Single dose Rituximab 375mg/m2 to max 500mg and confirm B cell depletion (CD 19 < 1%) at 1 week if depletion not achieved repeat weekly infusion up to max 4 doses.
Written consent must be obtained from legal guardians of patient before starting protocol after explanation of drug
benefits and potential side effects.
Protocols of management of SRNSRenal Biopsy1- All children with SRNS, whether initial or late, should undergo a renal biopsy before starting specific treatment.2- You should obtain informed consent from the parents before performing renal biopsy (look at renal biopsy chick list)3-The histological specimen must be examined by:
1. Light microscopy2. Immunofluorescence microscopy3. Electron microscopic in indicated cases by the consultant.
Renal Biopsy
Others IdiopathicRegardless the pathological type of idiopathic NS
Calcineurin inhibitors
MMF protocol
Cyclophosphamide
Rituximab protocolAll SRNS should receive Antiproteinuric drugs as long as proteinuria persist:Captopril 0.5 mg/kg/single daily dose Doses can be upgraded guided Lisinopril 0.05-mg/kg/single daily dose by blood pressure and kidney Losartan, valsartan 0.5-1 mg/kg/single daily dose functionAvailable preparations Captopril: Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Capoten, captopril, farcopril 25, 50mg/tab Hpopress 12.5, 25 mg/tab Lisinopril:
Zestril, sinopril 5,10,20 mg/tab Lisopril, lisinopril 10mg/tabMaxipril 5, 20 mg/tab.
Losartan: cozAAr, losartan 50,100mg/tab
Valsartan: Tareg 40,80,160,320 mg/tab
Relapsed SRNS patient after complete remission: 3 options without priority:1. Reinduction of full dose steroid therapy.2. Restart previous successful protocol.3. Shift to another immunosuppressive therapy.
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Protocol of Cyclophosphamide for SRNS
Patient Name:………………………SA: Age: dry weigth : Cyclophosphamide monthly doses ( 500mg/m 2 monthly)
1st Month/ /
2nd Month/ /
3rd Month/ /
SA:-------------------
Cycloph.Dose:-----------------------
SA:--------------
Cycloph.Dose:-------------------
SA:-------------------
Cycloph.Dose:-----------------------
4th Month/ /
5th Month/ /
6th Month/ /
SA:-------------------
Cycloph.Dose:-----------------------
SA:-----------------
Cycloph.Dose:----------------------
SA:-------------------
Cycloph.Dose:-----------------------
Cumulative dose of Endoxan(168 mg/kg/protocol)
Date Total cumulative dose Date Total cumulative dosemg/kg mg/kgmg/kg mg/kgmg/kg
Concomitant steroid therapy
Week 1-4 / /Week 5-10 / /Week 11-12 / /Dose:-----------------------Dose:-----------------------Dose:-----------------------
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
30mg/m2/ day (daily )45mg/m2/ EOD30mg/m2/ EODWeek 13-14 / /Week 15--20 / /6th -9th Month / /
Dose:-----------------------Dose:---------------------Dose:-----------------------
15 mg/m2/ EOD7.5 mg/m2/ EOD7.5 mg/m2/ EOD10th -12th month / /13th -15th Month / /16th- 18th Month / /
Dose:-----------------------Dose:---------------------Dose:-----------------------
7.5 mg/m2/ EOD7.5 mg/m2/ EOD7.5 mg/m2/ EOD
Side effects of cyclophospahmide: Alopecia, marrow suppression, Vomiting, hemorrhagic cystitis, risk of systemic infection
Precautions for Pulse Cyclophosphamide Therapy* Available preparation:- Endoxan (200 mg) dissolved in 10 cc saline or 1 gm dissolved in 50cc.* Administration:
- Calculated dose is put in 150cc saline and given over 1hour infusion.- Total cumulative dose of Cyclophosphamide: 168 mg/kg/protocol.
Investigations before every Endoxan pulse:1. CBC.2. Serum creatinine.3. Urine analysis.4. SGOT,SGPT, Bilirubin.
* Precautions before Endoxan administration:CBC Should be done two weeks after endoxan dose and if: -
a- Total WBCS < 4000 / cmm.b- Absolute neutrophil count < 1000 / cmm.c- Platelets < 150,000.
Do the following: 1- Postpone dose of Endoxan till recover of leucocytic count. 2- Give IV methyl prednisolone (solu - medrol) 30 mg / kg / day (Maximum 1 gm) for 3 days( persistent leucopenia)(exclude infection) 3. Granulocyte colony stimulating factor for persistent neutropeniaLast updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
4- Bone marrow aspiration if still leucopenic . 5- Reduction of next dose of endoxan by 25%.
* Precautions during and after endoxan administration:1- Ensure adequate hydration:
Fluid intake should be 1-1.5 times normal daily maintenance.2- Mesna: (Uromitexan 100 mg / ml):
* Dose: 60% of total endoxan dosage OR (360 mg / m2).* Administration: Total dose is fractionated and given direct IV as follow:
- Just before endoxan.- Just after endoxan.- 3 hours after endoxan.- 6 hours after endoxan.- 9 hours after endoxan.
3- Furosemide (lasix ampoule 10 mg/ml):1-2 mg / kg / divided every 8-12 hours are given IV.
4- Antiemetics: (If vomiting occurs), you can give:* Zofran (2 mg / ml).
- Dose: 0.15 mg / kg / dose IV.* Primperan (5 mg / ml).
- Dose: 0.2 mg / kg / every 12 hours IV.* Dexamethazone (4 mg / ml).
- Dose: 0.5 mg / kg / every 12 hours IV.
Patients with impaired kidney function, oliguria or anuria: Give 75% of calculated endoxan dose. Endoxan is added to total dose mesna and only 25-50 cc saline infusions over
2 hours. Bladder irrigation:
2ml mesna + 100 ml cold saline one hour before endoxan2ml mesna + 100 ml cold saline just after endoxan1ml mesna + 50 ml cold saline /2 hours for 24 hours1ml mesna+ 50 ml cold saline /4 hours for 24 hours.
Patients with impaired hepatic function: If SGOT, SGPT 3 folds or more above normal and/or serum bilirubin above 3 mg/dl ………. Give 75% of calculated endoxan dose.
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Protocol of Cyclosporine in SRNS page 1
Patient Name:………………………Age: Induction (3-6 months) 1- Cyclosporine Maintenance (3-5 years)
Date Wt Dose Date Wt Dose1st -2nd week / / ……………
…
1-2 weeks / /
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
……………………
……
Serum level & adjust dose Serum level & adjust dose
3rd -6th week7th -10th week11th-12th week
/ // // /
………………………………………
………
3rd-6thweek7th-10thweek11th-12thweek
/ // // /
4th month
5th month
6th month
9th month
12th month
15th month
18thmonth
21thmonth
24thmonth
27thmonth
30thmonth
33thmonth
36thmonth
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
/ /
Reassess :*Partial or complete remission: start maintenance*No partial or complete remission: continue induction for another 3 months
13th -14th week
15th -16th week
17th -20th week
21th-24th week
/ /
/ /
/ /
/ /
……………
……………
……………
………
Reassess :*Partial or complete remission: start maintenance*No partial or complete remission: stop protocol
“cyclosporine resistant”
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
……………………
……………………
……
Reassess :*Sustained complete remission during maintenance: ……………stop protocol*Partial remission continue for another 2 years
37-42 month
43-48 month
49-54 month
55-60n month
Second renal biopsy to asses nephrotoxicity
*** 2- Concomitant maintenance steroid therapy page 2Week 1-4 / /Week 5-10 / /Week 11-12 / /
Dose:-----------------------Dose:-----------------------Dose:-----------------------
30mg/m2/ day (daily )45mg/m2/ EOD30mg/m2/ EODWeek 13-14 / /Week 15--20 / /6th -9th Month / /
Dose:-----------------------Dose:---------------------Dose:-----------------------
15 mg/m2/ EOD7.5 mg/m2/ EOD7.5 mg/m2/ EOD10th -12th month / /13th -15th Month / /16th- 18th Month / /Dose:-----------------------Dose:---------------------Dose:-----------------------
7.5 mg/m2/ EOD7.5 mg/m2/ EOD7.5 mg/m2/ EODMonth 19-24 / /Month 25-30 / /Month 31-36 / /
SA:………….Dose:---------------------
SA:………….Dose:---------------------
SA:………….Dose:-----------------------
7.5 mg/m2/ EOD7.5 mg/m2/ EOD7.5 mg/m2/ EOD
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Follow up during protocol Serum creatinine Serum sandimmune Urine analysis
Date Value Date Value
Needed investigations before starting the protocol:1. Recent serum creatinine.2. Lipid profile.3. Result of renal biopsy.
* Induction3-6 months: -Cyclosporine 5-6mg/kg/daily BID(start 5mg/kg and adjust according to serum level) -Cyclosporine 2.5mg/kg/daily BID if basal serum creatinine is high. -morning and evening doses should be equal.** Maintenance for 3-5 years: Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
--Cyclosporine 3-5mg/kg/day BID. Titrate dose according to the therapeutic level. -Maintain the lowest effective dose based on efficacy (protienuria) and safety (S.cr.). “Trough level” :(150-200 ng/ml) during induction.(80-120 ng/ml)during maintenance
Available preparations of cyclosporine: Solution = Sandimmune Neo-oral, or Apramione[50 ml bottle (100mg/ml)]. Capsules = Sandimmune or Apramione 25,50,and100 mg.The capsule should not be crushed or evacuated.The syrup is photosensitive. Most common side effects:
2. Nephrotoxicity, hypertension.3. Hypertrichosis, gingival hyperplasia4. Dyslipidemia
Serum level: should be done1. 2 weeks after starting induction.2. 2 weeks after starting maintenance3. Every 3 months during maintenance4. Within 2 weeks after any dose modification.
Serum creatinine should be done :1. Before starting therapy 2. 2 weeks after induction.3. Every 3 months during maintenance.
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Protocol of Mycophenolate Mofetil (MMF) in SRNSPatient Name:………………………Age: sex:
Date Dry wt
SA Oral Pred.maintenance
MMF
Week 1-2
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Week 3-4Week 5-6
Week 7-8
Week 9-10
Week 11-12
Week 13-14
Week 15-165th month6th month7th month8th month9th month10th month11th month12th month
1- Concurrent Oral Prednisone: 1st week- 4th week : 30 mg/m2/day EOD 5th -10th week: 45 mg/m2/day EOD11th -12th week : 30 mg/m2/day EOD13th - 14th week : 15 mg/m2/day EOD 15th week-12th month 7.5mg/m2 /EOD 2- MMF:*Initial starting dose: 300- 400 mg/m2/ dose BID (max.2 gm/day) *Maintenance dose: 600 mg/m2/dose BID. It should be started after initial starting dose by 2 weeks if WBC counts not showing leucopenia.A-Reassess proteinuria after two months of (MMF) * partial or complete remission continues the protocol for 12 months. * No remission Stop the protocol.
B-Available preparations: Cellcept Capsules, 500 mg, and 250 mg, Mofetil tablets 500mg, 250 mg Myfortic tablets 180, 360 mgLast updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Protocol of Tacrolimus (TAC) In SRNS Patient Name:……………………………….…………………………….…… Serial No.:………………………………...……………………………..
Dry Weight:…………………………………..……………….…………… Surface area:………....……….……………….………….………..…..
Date Dry
wt
SA TAC dose Oral Pred. dose
week 1-2/ /
Serum level of TAC and adjust dose
week 3-4 / /Week 5-6Week 7-8 / /3rd month / /
Serum level of TAC and adjust dose
Week13-14Week 15-16 / /5th month / /6th month / /
Serum level of TAC and adjust dose
7th month / /8th month / /9th month / /
Serum level of TAC and adjust dose
10th month / /11th month / /12th month / /
1. TAC:-starting dose 0.1mg/kg/d divided in 2 doses for one year. -can dose up to 0.3mg/kg/d till reaching serum level of TAC: (9.8-19.4ng/ml) by the whole blood ELIZA assay or (0.5-1.5ng/ml) by serum high pressure liquid chromatography.-available preparation prograph 0.5mg, 1mg, 5mg Cap .
2. Oral Prednisone (Concomitant with TAC): -30mg/m2/day for 4weeks-45mg/m2/EOD for 6weeks-30mg/m2/EOD for 2weeks-15mg/m2/EOD for 2weeks
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
3. Precautions: Before starting TAC, the followings should be done ALB/cr ratio, Serum Cr,
Albumin , CBC, OGTT. Serum level of TAC done 2 week after starting treatment then / 3 m. F.U the patient each 2 weeks in 1st month then once monthly.
4. Relapse on TAC: add oral prednisone 60mg/m2/d till complete remission , tapering over two
months.
Pearls on TAC: It's a macrolid antibiotic isolated from fungus (streptomyces tsukubaensis). Mechanism of action: calcineurin inhibitor (potent cytokine suppressor more than cyclosporin with
less nephrotoxicity). Common side effects:
-Diarrhea (commonest) dose of TAC -Hyperglycaemia Regular monitoning of RBG
-Hypertension Add anti-hypertensive
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Rituximab protocol for SRNS Indications:
1. Multiple drug resistance( steroid , cyclosporine, mycofenolate mofetil, tacrolimus, cyclophosphamide).
2. Severe adverse effects of other immune suppressives.
Contra-indications to rituximab therapy: 1. Patient with SRNS known to have gene mutation for nephrotic syndrome2. Anaphylaxis to rituximab.3. Relative contra-indications:
*Allergy to rituximab- infusion reactions are common and can usually be managed with pre-medication and slow infusion
*Pneumonitis- due to risk RALI (Rituximab associated lung injury).
Side effects of rituximab: Infusion reaction Late adverse effects
Sore throatWheezing, cough, DyspneaFeverSkin rashNausea , vomitingBradycardia\ tachycardiaHypertension\Hypotension.Nasal stiffnesLeg pain.
SepsisGranulocytopeniaLiver failurefever
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Baseline, pre-treatment prior to first dose: Test Date ResultDEXAOphthalmology assessment for cataractsViral serology: HIV, HBsAg, HBsAb, HBcAb, HCVPolyomavirus PCR (Blood)Renal biopsySerum creatinineSerum calcium/phosphorusUrine protein/creatinine ratioLiver function testCD19 (peripheral blood)CD20 (peripheral blood)Complete blood picture
Premedications: (30-60 minute before each infusion): 5. Prednisolone 0.5mg/kg6. Avil: intravenous/8 hours.7. Paracetamole:
10-15mg/kg/dose/6 hours intravenous.8. Withhold or decrease dose of antihypertensive medications 12
hours before dose for risk of hypotension.
Sulfamethoxazole/trimethoprime prophylaxis: (5mg/kg to max 160/800mg 3 days per week) for 6 months.
Available preparations: MabThera ampoule 500mg/50mlRituxan ampoule 100mg/10mlRituximab 100mg/10ml, 500mg/50ml.
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Preparation of rituximab(just before infusion): Just before administration, rituximab should be diluted with 0.9% sodium
chloride injection to a final concentration of 1-4 mg/mL. A 1-mg/mL dilution(each 1ml of Rituximab diluted in 10 ml saline) is
preferable to facilitate adjustments in the infusion and to avoid adverse effects.
Rituximab should not be mixed with other medications or i.v. fluids because there is a lack of compatibility data.
Prepared infusions are stable in polyvinyl chloride or polyethylene bags at 2-8 C (36-46 °F) for 24 hours and at room temperature for an additional 12 hours.
Unused portions of undiluted drug must be discarded because of the absence of a preservative.
Infusion of rituximab: 5. Central or peripheral i.v. catheter.6. Should never be given by i.v. push or bolus injection.7. Blood pressure and temperature monitoring /hour during and 24
hours after infusion.8. Infusion over 6-8 hours(Gradual increase rate of infusion every hour
if no infusion reaction)(10ml/min 1st hour, 20ml/min 2nd hour, 30 ml/min 3rd , 40ml/min 4th hour then 50 ml/min from 5th hour to end of infusion).
Dose of rituximab in SRNS: 375mg/m2/dose weekly for 4 doses (max 500mg/dose)Week 1 Week 2 Week 3 Week 4Date: Date: Date: Date:
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Dose: mg Dose: mg Dose: mg Dose: mgDay 5 CD19:(Target <1%)
Day 5 CD19:(Target <1%)
Day 5 CD19:(Target <1%)
Day 5 CD19:(Target <1%)
Day 7protein/creatinine:
Day 7protein/creatinine:
Day 7protein/creatinine:
Day 7protein/creatinine:
Weeks 5-52: Monthly check liver function test, creatinine, Ca, Phos, (CD19), Immunoglobulins, urine microscopy and Pr:Cr.
Other immunsuppresive therapy should be continued during and 6 months after rituximab course then gradually tapered to lowest effective dose maintaining patient in remission.
Relapse after Rituximab associated with B cell reconstitution during initial 12 months Rituximab therapy: Single dose Rituximab 375mg/m2 to max 500mg and confirm B cell depletion (CD 19 < 1%) at 1 week if depletion not achieved repeat weekly infusion up to max 4 doses.
Written consent must be obtained from legal guardians of patient before starting protocol after explanation of drug
benefits and potential side effects.
Protocol for Management of Membranoproliferative GN (MPGN) Patient Name:………………………………………
Body Weight:…………………………………………
Serial No.: ……………………………………………….
Surface Area:…………………………………………
A- If the child is detected by incidental finding of urinary abnormalities without hypertension, renal impairment, or nephritis:
* No treatment. * Observe closely.
B- If the child has hypertension, heavy Proteinuria, or Nephrotic syndrome: 1- Methyl Predinsolone 10-20mg/kg/day, max. 1gm, I.V daily for 5-6 dose
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Date Dose1st
2nd 3rd
4th
5th
6th
/ // // // // // /
………………………………………………………………………………………………………………………………………………
2- Then, oral prednisolone 40-60mg/m2/max 60mg oral EOD reducing it gradually over 12-24 months
Date Dose Date Dose7th day2nd month3rd month4th month5th month6th month
/ // // // // // /
.…tab/EOD
.…tab/EOD
.…tab/EOD.…tab/EOD.…tab/EOD.…tab/EOD
7th month8th month9th month10th month11th month12th month
/ // // // // // /
.…tab/EOD
.…tab/EOD
.…tab/EOD
.…tab/EOD
.…tab/EOD
.…tab/EOD
3- Daily dipryidamole: 4-10mg/kg/day. C- If the child has significant renal impairment and crscentic glomerulonphritis: See RPGN protocols.
Protocol of Membranous Nephropathy
1. Perform appropriate investigations to exclude secondary causes in all cases of biopsy-proven MN: Infection: Hepatitis B, C, TB, Syphilis, leprosy, CMV. Drugs: Captopril, NSAIDs, Gold, Pencillamine. Autoimmune disorders: SLE, RA, autoimmune thyroididtis Neoplasm: carcinoma of bladder breast, pancreas, lymphoma,
leukemia.Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Others: DM, Sarcoidosis, Sickle cell disease, post renal transplantation.2. Idiopathic membranous nephropathy:
Non nephrotic proteinuria
Refractory cases
Corticosteroid/Cyclophosphamide protocol for Idiopathic MNPatient Name:………………………SA: Age: Dry weight :
1.Cyclical corticosteroid Cyclophosphamide regmine1st Month / / 2nd Month / / 3rd Month / /
Methylprednisone 1gm/day for 3 days:
Cyclophosphamide500mg/m2
Methylprednisone 1gm/day for 3 days:
Last updated August 2014
Asmptomatic/non nephrotic proteinuria
Nephrotic syndrome/nephrotic range proteinuria
ACE inhibitors/ARBsLipid lowering agents (statins)
Prednisone (4-8 weeks)
Steroid sensitive Steroid resistant
Cyclical corticosteroids cyclophosphamide regmine
Cyclosporine or tacrolimus protocols for SRNS
Rituximab
Alternate day steroids Taper over 3-6 months
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Day1: / /Day2: / /Day3: / /
Oral methylprednislone 0.5mg/kg/day for 27 days
Dose: BW:Date: / --- /
SA: ……………..
DOSE: ……………..
Day1: / /Day2: / /Day3: / /Oral methylprednislone
0.5mg/kg/day for 27 daysDose: BW:Date: / --- /
Serum creatinine: Serum creatinine: Serum creatinine:4th Month / / 5th Month / / 6th Month / /
Cyclophosphamide500mg/m2
SA: ……………..
DOSE: ……………..
Methylprednisone 1gm/day for 3 days:
Day1: / /Day2: / /Day3: / /Oral methylprednislone
0.5mg/kg/day for 27 daysDose: BW:Date: / --- /
Cyclophosphamide500mg/m2
SA: ……………..
DOSE: ……………..
Serum creatinine: Serum creatinine: Serum creatinine:2.Renoprotective therapy 3. Lipid lowering agent
Continued for additional 6 months after Statins 10-20 mg/day cyclical steroid /cyclophosphamide regmine
DateDrugDose
Available preparations of oral methylprednisilone:Urbasone retard 4, 8 mg/tab
Conditions associated with NS
Edema:
• Patients with underfilling edema:
– Glomerular filtration rate greater than 75 percent of normal
– Minimal change disease of acute onset
Last updated August 2014
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Date Drug Dose
جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
– Severe hypoalbuminemia (often below 1 g/dL)
– Nephrotic patients with low FENa(< 1%)
• Patients with elevated intravascular volume:
– Glomerular filtration rate less than 50 percent of normal
– Plasma albumin concentration of greater than 2 g/Dl
– Hypertension.
• MANAGEMENT
• Diet
– Dietary sodium restriction (to approximately 2 g of sodium per day)
– protein intake of around 130–140%of the recommended daily allowance according to statural age
– Hypovolemia:
Rapid infusion of plasma (20 ml/kg) OR
Albumin 20%(1 g/kg) administrated
• Diuretics:
INDICATIONS:
o Severe edema, after hypovolemia has been corrected.
TYPES:
o Furosemide: ( 1–2 mg/kg).
o Spironolactone (5–10 mg/kg)
o Amiloride (0.2–0.5 mg/kg)Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
• Albumin:
o IV 25% albumin at 1-2 g/kg/d
o Either as a continuous infusion or divided q 6-8 hours.
o Albumin treatment should continue for 4 to 6 hours before initial administration of diuretics to minimize the risk of worsening any intravascular volume depletion that may be present.
Sever edema:
• Furosemide plus albumin:
– to increase the rate of diuretic delivery to the kidney.
– Immediately effective but not long-lasting.
– NOTE : respiratory distress with congestive heart failure
Refractory edema with serious effusions:
– Immersion of the body up to the neck in a bath
– Drainage of ascites and/or pleural effusions
Thromboemboli :
– Mobilization.
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
– Avoidance of hemoconcentration
– early treatment of sepsis or volume depletion.
– Prophylactic warfarin
– High risk patients with:
1. Plasma albumin concentration below 20 g/l
2. fibrinogen level 6 g/l
3. Antithrombin III level below 70% of normal.
– Patients at risk may also be treated with low-dose aspirin and dipyridamole.
– Heparin:
– Given initially if thrombi do occur
– Alone or with thrombolytic agents.
Resume of Nephrotic SyndromeLast updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Date Serial No.
Name Age Sex:
* Diagnosis: 1ry NS: Non Relapser Infrequent Relapser Frequent
Relapser Steroid Dependent Steroid Resistant: Initial
Late2ry NS:
* Initial presentation:
Date 24h Proteinuria S. Albumin
Age
Haematuria
- ve
+ ve Micro
Macro
S. Cholesterol
BL.Pr. S. Creatinine
Other C3Others Others
* Response to steroid protocol: Responder: Clinical Ws Lab: Ws Non responder:
* Biopsy
Initial biopsy Repeated biopsyDateIndication
Findings MCNS FSGS DMP MPGN Other
MCNS FSGS DMP MPGN Other
Complication
Complications of the disease Thrombosis Infection Other
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Current Condition
قـــــــــرارا
أقر أنا ولي أمر ..................................................................................................................الطفل/
الذي يعاني من المحجوزو..............................................................................................................................
بقسم الكلي بمستشفي األطفال الجامعي بالموافقة على عمل عينة من الكلي ألهمية ذلك لتشخيص الحالة مع علمي
باحتمال حدوث مضاعفات نتيجة العينة قد يؤدي إلياستئصال كامل للكلي.
ولــي أمـــر الطـفـل ،،،،،،،
ال أوافق على أوافق على إجراء العينة إجراء العينة
.............................................................االســـم/
...................................................................االســـم/
التوقيــع/ ..........................................................التوقيــع/ ..................................................................
رقم البطاقة/ ........................................................رقم البطاقة/ ..................................................................
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
التاريـــخ/ .......................................................التاريـــخ/ ..................................................................
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Requirements for Renal Biopsy Indications for renal biopsy & attendant name: …………………………………………………………………….…………………………………..
Admission to nephrology floor at least one day before the biopsy.
Meeting with biopsy clarification team.
Please do:
PT
APTT
Bleeding time
Clotting time
Virology screen
HbsAg
HCV Antibody
HIV
Agreement of radiologist.
Consent of parents or responsible person.
Please send the patient to 1st floor ultrasound room with suitable needle &
biopsy gun to start the renal biopsy process
People who will attend the renal biopsy:
Radiology attendant
NephrologistsLast updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Pathology technician
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Steps of Renal Biopsy :
Sedation (dosage)Pethidine . Diazepam .
Ketamine . Other .
Please specify
The patient lie on ………………………………………………………………..…
Take three cores from……………………………………………………………
One for ordinary microscope examination.
One for immune fluorescent assessment.
One for frozen section preservation for electron
microscopy examination.
After Biopsy: Patient will lie on same side of biopsy for at least 12 hours, no working.
Stop oral feeding till the patient is fully conscious.
Give I.V fluids maintenance dose.
We can give pain killers (acetaminophen, paracetamole) 15mg\kg\dose
What the Nurses will do After: Measure pulse & blood pressure every 10 min. in the 1st hour,
every 30 min. in the next 2 hours, then every 2 hours till morning.
Observe abdominal girth every 4 hours.
Discharge the patient in the next day if there is no contraindication or complications.
Give patient appointment after one week to discuss the result of the
renal biopsy.
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Renal Biopsy AuditThe whole of this survey will be scanned. In order that forms are read correctly please use a black pen and mark the boxes with an X
Record Number
Operator(s)
Hospital Number
Referral Centre
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Date of Biopsy
Native
Transplant
Inpatient/ /
Day Case
Weight (Kg) Sedation (dosage)
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
. Pethidine . Diazepam .
Ketamine . Other .
Please specify
Play Preparation Yes No
Parent in Attendance Throughout Yes
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
No
Information Sheet and Story Book Received Yes No
Number of Passes NeedleTru-cut 14g Springloaded 16g
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Indication ComplicationsTransplant Hypoxia (oxygen required
on return to ward bed)Yes No
Haematuria
Protenuria
Acute glomerulonnephritis Gross haematuria Yes
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
No
HypertensionTransplant Yes NoNephrotic syndrome steroid sensitive
Nephrotic syndrome steroid resistantPain requiring regular analgesic Yes NoNephrotic syndrome atypical
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Acute renal failure
Chronic renal failure Delay in discharge Yes No
Pre CyclosporinReturn to ward Yes No
Other
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Pathology review
Number of Glomeruli Adequate LM Yes No
Last updated August 2014
2008
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
N / R
LM Yes No N / R
EM Yes No N / R
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Acute interstitial nephritis (AIN) Macroscopic polyarteritis nodes
Acute post infectious (proliferative) glomer Membranous nephropathy
Acute tubular necrosis Mesangiocapillary glomerulonephritis Type 1
Alport's syndrome Mesangiocapillary glomerulonephritis Type 2
Alport – like syndrome Mesangiocapillary glomerulonephritis Type 3
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Amyloid Macroscopic polyarteritis nodes
Analgesic nephropathy Minimal change nephrotic syndrome
Anti-GBM disease Nephrocalcinosis
Autosomal dominant PKD Nephrocalcinosis
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Autosomal recessive PKD Normal
Balkan nephropathy Polycystic Kidney Disease (other)
Chronic renal failure – uncertain aetiology Focal segmental glomerulosclerosis (primary)
Cis-plantinum toxicity Proliferative glomerulonephritis
Congential nephrotic syndrome (DMS) Pyelonephritis secondary to urolithiasis
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Congential nephrotic syndrome (Finnish) Reflux nephropathy
Congential nephrotic syndrome (FSGS) Renal artery stenosis
Congential nephrotic syndrome (minimal change) Renal artery thrombosis
Congential nephrotic syndrome (unspecified) Renal trauma
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Cortical necrosis Renal vein thrombosis
Crescentic glomerulonephritis Systemic lupus erythematosis
Cyclosporin nephrotoxicity Thin basement membrane
D+ haemolytic uraemic syndrome Tuberous sclerosis PKD
D- haemolytic uraemic syndrome Vasculitis (unspecified)
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
Drug nephrotoxicity unspecified Wegner's granulomatosis
Glomerulonephiritis (unspecified) Wilm's nephropathy
Henoch Schonlein nephritis Wilm's tumour
IgA nephropathy Other
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
If 'Other', please state
TransplantAcute parenchymal Acute vascular Mixed acute
Chronic rejection Acute interstitial nephritis Infarction
Cyclosporin toxicity Acute tubular necrosis Other
Normal End stage kidney
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
قـــــــــرارا
أقر أنا ولي أمر ..................................................................................................................الطفل/
الذي يعاني من ...............................................................................................................................................
المحجوز بقسم الكلي بمستشفي األطفال الجامعيوتلقي طفلي عقار االندوكسان (بالموافقة على
Cyclophosphamide ( باحتمال حدوثمع علمي ألهمية ذلك من هذا الدواء. وأن األطباء المعالجون قدمضاعفات
.أطلعوني على هذه المضاعفات ونسبة حدوثها
ولــي أمـــر الطـفـل ،،،،،،،
ال أوافق أوافق على إعطاء العقارعلى إعطاء العقار
.............................................................االســـم/
...................................................................االســـم/
التوقيــع/ ..........................................................التوقيــع/ ..................................................................
رقم البطاقة/ ........................................................رقم البطاقة/ ..................................................................
التاريـــخ/ .......................................................التاريـــخ/ ..................................................................
Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
قـــــــــرارا
أقر أنا ولي أمر ..................................................................................................................الطفل/
الذي يعاني من ...............................................................................................................................................
المحجوز بقسم الكلي بمستشفي األطفال الجامعيو )Rituximabتلقي طفلي عقار الريتوكسيماب (بالموافقة على
من هذاباحتمال حدوث مضاعفات مع علمي ألهمية ذلك الدواء. وأن األطباء المعالجون قد أطلعوني على هذه
.المضاعفات ونسبة حدوثها
ولــي أمـــر الطـفـل ،،،،،،،
ال أوافق أوافق على إعطاء العقارعلى إعطاء العقار
.............................................................االســـم/
...................................................................االســـم/Last updated August 2014
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جامعــة المنصــورةMansoura University
Children's مستشفـي األطفـالHospital
وحدة أمراض الكليNephrology Unit
التوقيــع/ ..........................................................التوقيــع/ ..................................................................
رقم البطاقة/ ........................................................رقم البطاقة/ ..........................................................
`.......................................................التاريـــخ/ .................................................................. /التاريـــخ
Last updated August 2014
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