Nutrition Implications of Congenital Nephrotic Syndrome

Vehik NazaryanUMD-CP Dietetic Intern03/20/2014

OverviewDefinition of NS

CausesSymptomsClinical Manifestation of NSDiagnosisTreatmentPrognosis

Case ReportMedical and Diet HistoryNutrition DiagnosisGoals

Congenital Nephrotic SyndromeWhat is it? Is a Is a very rare form of nephrotic

syndrome, mostly in families of Finish origin that developes shortly after birth

characterized by: An increase in permeability of the capillary walls of

the glomerulusHigh levels of protein passing from the blood in to

the urine

CausesIt is inherited, passed down

through families Long arm of Chromosome 19th

Abnormal form of protein called nephrin

SymptomsCoughDecreased urine outputFoamy appearance of urineLow Birth Weight Poor appetiteSwelling(total body) Signs of

disease

Clinical ManifestationsProteinuria

Hypoalbuminemia

Ascites and in some cases edema

Hyperlipidemia/High cholesterol

Predisposition for coagulation

Rarely high blood pressure Note:(most pt are normoteinsive)

Microcytic Hypochromic Anemia—due to transferrin loss

Erythrocyte sedimentation rate is increased due to increased fibrinogen and other plasma contents

Diagnosis in UteroUltrasound done on pregnant mother before birth

may show larger-than-normal placenta

Pregnant mother may have a screening test done during pregnancy

Alpha-fetoprotein in a sample of amniotic Fluid

Diagnosis After Birth Infant will show signs of severe fluid retention and swelling

Puffiness around eyesPitting edema over the legsFluid in the peritoneal cavity causing ascitesGeneralized edema

High Blood Pressure

Abnormal heart or lung sounds

Signs of malnutrition

Urinalysis reveals fat and large amount of protein in the urine.

Low total protein in the blood

Genetic test to confirm the diagnosis

TreatmentEarly and aggressive treatment is needed with:

Diuretics Antihypertensive agents

Antibiotics to control infections

NSAIDs( Nonsteroidal anti-inflammatory drugs) to reduce the amount of protein leaking into the urine

Fluid restriction to help control swelling

Kidney removal Dialysis Kidney transplant

PrognosisDisorder leads to

InfectionMalnutritionKidney failureDeath by age 5-many children die within

the fist year

TestsFollowing tests should be

performed:• Complete blood count• Metabolic panel

Levels of serum electrolytesCalciumPhosphorusBUNCreatinine

Case ReportYR is a 10 month old Hispanic

female who was diagnosed with Congenital Nephrotic Syndrome on 06/25/2013

YR was born at 31 weeks of gestation

Corrected age: 7months +3 weeks

The pregnancy was complicated by fetal pelviectasis at 20 weeks

Weight and Height Weight: 6.7 kg

less than 5th Percentile Corrected weight percentile: 5th percentil Weight age: 5th percentile

 Height : 60.5 cm

Percentile: less than 5th percentile Corrected height percentile: less than

5thpercentile

•Weight/Height percentile:50th percentile

History of Present IllnessPrenatal and postnatal mild bilateral

pelviectasis

Hypoalbuminemia

Proteinuria

Hyperlipidemia

History of Present IllnessELBW infant with mild contractures

and distal hyperextensibility

Since her last visit admitted to CNMC for catheter dysfunction

tPA instillation Prophylactic Lovenox

Results of Physical ExamWell looking with normal body

compositionNo peritoneal edemaMild-to-moderate abdominal

distention with ascites Soft abdomen that was

nontender to palpation

Growth Charts

5th%

Corrected age

Weight

Age

Growth Charts

Weight (Kg)

Length(cm)

75th%5th%

25th

Diet HistoryPt is on a 38 cal/oz Similac Advance

Pt takes all feeds PO

Current feeding regimen:Daytime feeds: 55ml x7 feeds/day (2 hours apart)Night time feeds: 75ml x3 feeds per day(3 hours

apart) total of 610ml/day15 scoops of formula + 1 tablespoon + 490ml of

water= 600 ml of formula.

Vitamin and Mineral Supplements

Cholecalciferol 800 units daily

One multivitamin with iron 1 ml daily

Food Allergies: NKFAPatient has not been exposed to many food

allergens secondary to dietary restrictions

LabsLab 02/11 02/12 02/21 Normal

RangeSodium H145 139, 142 136 132-143Potassium

H6.2 4.6, 4.7 5.2 3.5-5.8

Chloride H110 H108, 109 104 97-106BUN H31 H24, 25 H32 1-14Creatinine

H0.6 H1.2, 1.2 H0.7 0.2-0.5

Calcium L7.0 8.1, 8.2 8.6 8.1-11Total Protein

L4.5 4.6-7.8

Albumin L1.5 L1.1 L1.4 2.3-4.7

MedicationsMedication 400mg Dosage

Albumin infusion 3 times a week

Multivitamin with iron 1 ml dailyLasix 4mg daily

Bicitra 3mEq 3 times a day, Spironolactone 2.4 mg twice a day

Lisinopril 0.3 mg twice a dayCholecalciferol 800 units daily

Erythropoietin 400 units subcutaneously twice a week

Lovenox 0.16ml subcutaneously twice daily

Calcium Carbonate 400 mg added to full days worth of feeds

Nutrition Related DiagnosisPES statements1. Inadequate fluid intake related

to nephrotic syndrome as evidenced by formula concentrated to 38Kcal/oz

2. Suboptimal growth rate related to nephritic syndrome as evidenced by average weight gain less than 15g a day.

Estimated RequirementsDRI= 80Kcal/kg/day

80 x 6.7 kg = 536Kcal/dayCalorie requirements for catch-up growth= 110-120-

Kcal/kg

Protein Requirements: 1.2 g/kg/day = 1.2 x 6.7=8.04g/day

Fluid requirements=670ml/dayBased on Holiday-Segar method

Goal is to gain 12g dayGoal is to grow 0.48 mm/day

Weight in Kg Fluid Needs1-10kg 100ml/kg

Nephrotic SyndromeGeneral Renal InterventionEnergy DRIProtein DRI Do not

supplement to replace urinary losses

Sodium 1-3 mEq/kg/dayPotassium Restriction not neededPhosphorus Restriction not neededFluid Will vary according to

urine output consult renal team

Diet Order17 scoops + 1 teaspoon +488 ml

+50ml= 654 ml formula

Day time feeds:59ml x 7 feeds/day=413ml

Night time feeds: 80ml x 3 feeds/day= 240ml

Formula provides:

Energy: 114kcal/kg = 764kcal/day

Protein:2.3 g/kg = 15.4g/day

Fluids: 653ml/day

Goals Continue Similac Advance PO

Advance feedings to q3 hrs intervals

Continue to monitor Sodium, Potassium, Chloride, Calcium, Creatinine and BUN

Monitor weights Goal is to gain 15 g/day

Begin introduction of renal appropriate solid foods