11/9/2015. Childhood Nephrotic Syndrome: The Clinical Pathway. Learning Objectives. Nephrotic Syndrome - Definition. Proteinuria.

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1 Childhood Nephrotic Syndrome: The Clinical Pathway Cherry Mammen, MD, FRCPC, MHSc Douglas G. Matsell, MDCM, FRCPC Division of Nephrology, BC Children s Hospital Grand Rounds Nov 13th, 2015 Learning Objectives To review the clinical presentation and treatment of childhood nephrotic syndrome To share our recently developed childhood nephrotic syndrome clinical pathway To discuss potential barriers for pathway implementation from BC pediatricians perspective Nephrotic Syndrome - Definition Proteinuria Hypoalbuminemia Edema 1

Nephrotic Syndrome - Diagnosis BCCH 2008-2013 The commonest pediatric

Incidence Childhood Type 1 Diabetes Mellitus Daneman, Pediatric Diabetes 2009

2 Nephrotic Syndrome - Diagnosis BCCH The commonest pediatric glomerular disease Nephrotic Syndrome - Incidence Childhood Type 1 Diabetes Mellitus Daneman, Pediatric Diabetes 2009 Nephrotic Syndrome - Incidence Childhood Crohn s Disease Griffiths, Inflamm Bowel Dis,

3 Nephrotic Syndrome - Incidence Ault, Clinical Nephrology 2012 Where do our NS patients live? Nephrotic Syndrome Local Practice Variation Mean mg/m2 STD mg/m2 N=75 3

criteria-based progression standardized care for a specific clinical problem, procedure or episode of care in a specific population Kinsman L et al, BMC Medicine, 2010 Reasons to

4 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 a specific 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 Nephrotic Syndrome Clinical Pathway 4

5 NS Clinical Pathway Resources NS Case History 4 y.o. male presents to community ER with 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 5

6 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 Nephrotic Syndrome Evaluation Nephrotic Syndrome Case: Physical Exam BP: 105/68 (<95 th %ile), HR: 84, RR:24, afebrile Wt: 20 kg, Ht:105 cm BSA: 0.76m 2 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 6

Nephrotic Syndrome Evaluation NS Case Investigations (Urine) Urinalysis: 5 g/l protein, 10-20 RBC

mmol/l, Cr 40 umol/l Estimated GFR 114 ml/min/1.73m 2 (normal) Na + 135, K + 4.

7 Nephrotic Syndrome Evaluation NS Case Investigations (Urine) Urinalysis: 5 g/l protein, 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/1.73m 2 (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 7

8 Where are we in the pathway? Nephrotic Syndrome Red Flags Where are we in the pathway? Induction Treatment 8

9 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 Patient Home Monitoring 9

10 Home Monitoring Example Dietary Management Education resources: sodium content of foods sample menus label reading food intake records BC Healthline *811 Optimizing Bone Health 10

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,

11 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) 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 11

12 Where are we in the pathway? Relapse Treatment Important NS Definitions to know 12

13 Our Clinical Pathway Team Barriers to Pathway Implementation Questions & Discussion 13

Nephrotic Syndrome. Sara Alsharhan PharmD candidate, KSU 2014

Nephrotic Syndrome. Sara Alsharhan PharmD candidate, KSU 2014 Nephrotic Syndrome Sara Alsharhan PharmD candidate, KSU 2014 Outline Introduction Nephrotic syndrome classifications Signs and symptoms Diagnoses Management Complications Monitoring Case presentation Introduction