Childhood Nephrotic Syndrome:The Clinical Pathway
Cherry Mammen, MD, FRCPC, MHScDouglas G. Matsell, MDCM, FRCPCDivision of Nephrology, BC Children’s HospitalGrand RoundsNov 13th, 2015
Cherry Mammen, MD, FRCPC, MHScDouglas G. Matsell, MDCM, FRCPCDivision of Nephrology, BC Children’s HospitalGrand RoundsNov 13th, 2015
Learning Objectives
To review the clinicalpresentation and treatment ofchildhood nephrotic syndrome
To share our recentlydeveloped childhood nephroticsyndrome clinical pathway
To discuss potential barriersfor pathway implementationfrom BC pediatricians’perspective
To review the clinicalpresentation and treatment ofchildhood nephrotic syndrome
To share our recentlydeveloped childhood nephroticsyndrome clinical pathway
To discuss potential barriersfor pathway implementationfrom BC pediatricians’perspective
Nephrotic Syndrome - Definition
Proteinuria
EdemaHypoalbuminemia
Nephrotic Syndrome - Diagnosis
The commonest pediatricglomerular disease
200
250
300
350
400
BCCH 2008-2013
0
50
100
150
Nephrotic Syndrome - Incidence
Childhood Type 1 Diabetes Mellitus
Daneman, Pediatric Diabetes 2009
Nephrotic Syndrome - Incidence
Childhood Crohn’s Disease
Griffiths, Inflamm Bowel Dis, 2011
Nephrotic Syndrome - Incidence
Ault, Clinical Nephrology 2012
Where do our NS patients live?
Mean 2688.5mg/m2STD 915.3mg/m2N=75
Nephrotic SyndromeLocal Practice Variation
Mean 2688.5mg/m2STD 915.3mg/m2N=75
What is a clinical pathway?
structured multidisciplinary plan of care used to channel the translation of evidence
into local structures details the steps in a course of treatment or
care has time-frame or criteria-based
progression standardized care for a specific clinical
problem, procedure or episode of care in aspecific population
structured multidisciplinary plan of care used to channel the translation of evidence
into local structures details the steps in a course of treatment or
care has time-frame or criteria-based
progression standardized care for a specific clinical
problem, procedure or episode of care in aspecific population
Kinsman L et al, BMC Medicine, 2010
Reasons to develop a pathway
“Too much practice variation in our division” “Hospital administrators are pressuring us to be
more involved in quality improvement” “Patients need to be more involved in their care” “Our documentation is scattered and needs
standardization” “They will improve efficiency and patient care”
“Too much practice variation in our division” “Hospital administrators are pressuring us to be
more involved in quality improvement” “Patients need to be more involved in their care” “Our documentation is scattered and needs
standardization” “They will improve efficiency and patient care”
Nephrotic Syndrome Clinical Pathway
NS Clinical Pathway Resources
NS Case History
4 y.o. male presents to community ERwith “edema”
Noticed swelling 3 wks ago & worsening Started around time of a viral URTI Face, abdomen, & lower legs No major discomfort & able to ambulate
Went to walk-in clinic 2 wks ago Diagnosed with “allergies”
4 y.o. male presents to community ERwith “edema”
Noticed swelling 3 wks ago & worsening Started around time of a viral URTI Face, abdomen, & lower legs No major discomfort & able to ambulate
Went to walk-in clinic 2 wks ago Diagnosed with “allergies”
Nephrotic Syndrome Case History
No gross hematuria Slightly reduced urine output (2x/day)
Drinks 1.5L/day with high salt diet No vomiting or diarrhea No headaches, SOB, or abdominal pain No fever, rash, or joint complaints Past medical history unremarkable No medications or allergies No family history of renal disease Immunizations “up to date”
No gross hematuria Slightly reduced urine output (2x/day)
Drinks 1.5L/day with high salt diet No vomiting or diarrhea No headaches, SOB, or abdominal pain No fever, rash, or joint complaints Past medical history unremarkable No medications or allergies No family history of renal disease Immunizations “up to date”
Nephrotic Syndrome Evaluation
Nephrotic Syndrome Case: Physical Exam
BP: 105/68 (<95th%ile),HR: 84, RR:24, afebrile
Wt: 20 kg, Ht:105 cm BSA: 0.76m2
Periorbital edema Lower limb pitting edema Abdomen: mild ascites, no
tenderness or guarding No scrotal edema CVS: warm, strong pulses, cap
refill <2 sec Resp: Slightly ↓ A/E to bases
BP: 105/68 (<95th%ile),HR: 84, RR:24, afebrile
Wt: 20 kg, Ht:105 cm BSA: 0.76m2
Periorbital edema Lower limb pitting edema Abdomen: mild ascites, no
tenderness or guarding No scrotal edema CVS: warm, strong pulses, cap
refill <2 sec Resp: Slightly ↓ A/E to bases
Nephrotic Syndrome Evaluation
NS Case Investigations (Urine)
Urinalysis: 5 g/L protein, 10-20 RBC’s/HPF Urine PCR: 1250 mg/mmol
NS Case Investigations: Labs
CBC: WBC 5, Hb 145, Platelets 350 BUN 5 mmol/L, Cr 40 umol/L Estimated GFR 114 ml/min/1.73m2 (normal)
Na+ 135, K+ 4.2, Cl- 102, HCO3- 24 Albumin 18 g/L Cholesterol panel not needed Additional nephritic work-up not needed
eg: C3, C4, ANA, anti-ds DNA
CBC: WBC 5, Hb 145, Platelets 350 BUN 5 mmol/L, Cr 40 umol/L Estimated GFR 114 ml/min/1.73m2 (normal)
Na+ 135, K+ 4.2, Cl- 102, HCO3- 24 Albumin 18 g/L Cholesterol panel not needed Additional nephritic work-up not needed
eg: C3, C4, ANA, anti-ds DNA
Where are we in the pathway?
Nephrotic Syndrome “Red Flags”
Where are we in the pathway?Induction Treatment
NS Treatment: Preventing Complications
• Types of nephrotic syndrome• Complications of nephrotic syndrome• Prednisone course & side effects• How to dip urine for protein• Dietary recommendations• Immunizations and infections• Ongoing care
• Types of nephrotic syndrome• Complications of nephrotic syndrome• Prednisone course & side effects• How to dip urine for protein• Dietary recommendations• Immunizations and infections• Ongoing care
Patient Home Monitoring
Home Monitoring Example
Dietary Management
Education resources: sodium content of
foods sample menus label reading food intake records BC Healthline *811
Education resources: sodium content of
foods sample menus label reading food intake records BC Healthline *811
Optimizing Bone Health
Immunizations
Detailed immunization history Obtain immunization records for chart Avoid live vaccines on “high dose” prednisone
Annual flu shot recommended Need to know if VZV series complete
If not, may need VZIG for exposure Recommend Pneumovax (PPV23)
Detailed immunization history Obtain immunization records for chart Avoid live vaccines on “high dose” prednisone
Annual flu shot recommended Need to know if VZV series complete
If not, may need VZIG for exposure Recommend Pneumovax (PPV23)
Where are we in the pathway?Surveillance Checklist
Back to Case
Started on therapy with no complications Patient goes into remission in week 2
Trace or negative protein on dipstick x 3 days Worksheets being completed adequately Growth has been normal Ophthalmology check-up & spinal x-rays normal Off Prednisone in 24 wks, but………
Call from parents: patient has developed URTI &urine dips are 3+ for 3 days with mild edema
Started on therapy with no complications Patient goes into remission in week 2
Trace or negative protein on dipstick x 3 days Worksheets being completed adequately Growth has been normal Ophthalmology check-up & spinal x-rays normal Off Prednisone in 24 wks, but………
Call from parents: patient has developed URTI &urine dips are 3+ for 3 days with mild edema
Where are we in the pathway?Relapse Treatment
Important NS Definitions to know
Our Clinical Pathway Team
Barriers to PathwayImplementation
Questions & DiscussionQuestions & Discussion
