Nephrotic Syndrome

Yun Chi Cheng

Saturday, January 25, 14

Patient JC
! 6 year old Japanese male ! Admitted for relapse of nephrotic syndrome (NS)

Saturday, January 25, 14

Anthropometrics
! JC
! Height 3 9, 114.3 cm ! ~50 percentile ! Weight 44 lb, 20 kg ! ~47 percentile

Saturday, January 25, 14

Biochemical
Tests Total Protein Serum Albumin Na+ K+ 25-hydroxyvitamin D CHOL TG VLDL LDL HDL Proteinuria
Saturday, January 25, 14

Patients Values 5.0 g/dL 3.0 g/dL 133mEq/L 3.3 mEq/L 20 ng/mL 180 mg/dL 100 mg/dL 40 mg/dL 150 mg/dL 30 mg/dL 30-50g/d

Normal Values 6.0-8.0 g/dL 3.5-5 g/dL 136-145 mEq/L 3.5-5.5 mEq/L 25-80 ng/mL 120-230 mg/dL 20-130 mg/dL 7-32 mg/dL <130 mg/dL >45 mg/dL

Social Hx
! Lives with family ! Cultural-oriented ! Loves to play

Saturday, January 25, 14

PMHx
! Previously diagnosed with nephrotic syndrome (NS) at age 4

Saturday, January 25, 14

Nephrotic Syndrome
! Children main causes ! Minimal change disease, glomerulonephritis (60%) ! Focal glomerulosclerosis (10%) ! All forms of proliferative glomerulonephritis (10%) ! Membranous glomerulonephritis (5%) ! Secondary to systemic disorder (5%)

Saturday, January 25, 14

Clinical- HPI
! First onset
! Treatment ! High-dose administration of oral corticosteroid ! Treated repeatedly w/ intravenous pulse methylprednisolone ! Stayed in remission ! Low-dose corticosteroid w/concomitant of cyclosporine and mycophenolate mofetil

Saturday, January 25, 14

Clinical- HPI
! Relapse
! Treatment ! Initially, administered w/ Intravenous methylprednisolone ! Ineffective! ! Administered tacrolimus ! Stopped administration of cyclosporine ! Proteinuria decreased ! 2 weeks later, developed herpes zoster rash and proteinuria began to increase ! Day 27, suddenly developed generalized tonic convulsion
Saturday, January 25, 14

Clinical- HPI
! Cont.
! MRI displayed multiple lesions w/abnormal signals in bilateral parietal and occiptal regions ! Posterior reversible encephalopathy (PRES) associated w/ administration of tacrolimus ! A month after first convulsion! nystagmus and tremor in upper limb and limb appeared and he became drowsy ! Diagnosed with Wernickes encephalpathy (WE) ! After pyretic episode, developed disseminated intravascular coagulation (DIC)

! Recovered!
! From DIC, high blood pressure, proteinuria dropped.
Saturday, January 25, 14

Clinical- Medical Dx
! Medical diagnosis
! Wernicke Syndrome secondary to nephrotic syndrome ! Hypertension (170/90 mmHg) ! Medications:
! ! ! ! ! ! ! Intravenous methylprednisolone Oral corticosteroid cyclosporine Tacrolimus Rituximab (treat proteinuria) plasmapheresis (treat proteinuria) 100 mg/day thiamine (treat WE)

Saturday, January 25, 14

Dietary Hx
Usual Intake
Breakfast 1C miso soup ! C white rice ! C Natto 1TB tsukemono (japanese pickles) 1C soymilk Lunch 1C miso soup 1TB tsukemono 1 oz. broiled mackerel 1C white rice 1C apple juice Snack 8pc Salted peanuts Dinner 1C miso soup 1TB tsukemono 1C green tea 6pc California roll

Saturday, January 25, 14

Dietary Hx

Saturday, January 25, 14

Energy, Protein, Fluid Requirements

! Energy
! RDA"70kcal/kg x 20kg = 1400 kcal/d ! WHO ! REE= Males (22.7 x 20 [kg]) + 495= 908.14 ! Stress factor: 1.2 x 1.2(AF) ! =1200 kcal/day ! !1300 kcal/day

! Protein (1.1g/kg)
! 1.1g(18.2 kg) = 20.2 g PRO/day

! Fluid
! 1000mL + 50mL/kg (10kg) = 1500mL
Saturday, January 25, 14

Nurtrition Diagnosis
! Primary
! Excessive sodium intake r/t nephrotic syndrome and eating habits AEB usual dietary intake, blood pressure 170/90, edema, Na+ 133.mEq/L.

! Alternative
! Inadequate protein intake r/t massive proteinuria AEB albumin 3.4 g/dL, proteinuria 30-50g/day, and usual dietary intake.

Saturday, January 25, 14

Plan Management
! Objective: ! Manage the symptoms associated with this syndrome ! Reduce edema and hypertension ! Hypoalbuminemia ! Maintain RDA protein intake ! Hyperlipidemia ! Limit total fat and cholesterol ! Maintain nutrition stores and prevent progression of renal failure ! 1200 mg vitamin D and 800 mg calcium

Saturday, January 25, 14

Monitoring
! Weight
! Ins and outs ! Growth chart

! Labs
! Blood and urine test ! Albumin, proteinuria, Na+, K+, vit D, Ca2+, TG, GFR ! Nitrogen balance

! Diet change

Saturday, January 25, 14

Communication
! Restrict sodium 2-3 grams/day ! Encourage use of linoleic and omega-3 FA ! handout

Saturday, January 25, 14

Saturday, January 25, 14

Evidence Analysis Paper

! Can a low protein diet reduce proteinuria in patients with nephrotic syndrome?
! P: patients with nephrotic syndrome ! I: low protein diet ! C: high protein diet ! O: proteinuria

Saturday, January 25, 14