nutrition implications of infantile congenital nephrotic
TRANSCRIPT
Nutrition Implications of Congenital
Nephrotic Syndrome
Vehik Nazaryan
UMD-CP Dietetic Intern
03/20/2014
Overview
Definition of NS Causes
Symptoms
Clinical Manifestation of NS
Diagnosis
Treatment
Prognosis
Case Report Medical and Diet History
Nutrition Diagnosis
Goals
Congenital Nephrotic Syndrome What 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
glomerulus
High levels of protein passing from the blood in to the urine
Causes
It is inherited, passed down through families Long arm of Chromosome 19th
Abnormal form of protein called nephrin
Symptoms
Cough
Decreased urine output
Foamy appearance of urine
Low Birth Weight
Poor appetite
Swelling(total body) Signs of disease
Clinical Manifestations
Proteinuria
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 Utero
Ultrasound 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 eyes
Pitting edema over the legs
Fluid in the peritoneal cavity causing ascites
Generalized 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
Treatment
Early 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
Prognosis
Disorder leads to Infection
Malnutrition
Kidney failure
Death by age 5-many children die within the fist
year
Tests Following tests should be performed:
• Complete blood count
• Metabolic panel Levels of serum electrolytes
Calcium
Phosphorus
BUN
Creatinine
Case Report
YR 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 Illness
Prenatal and postnatal mild bilateral pelviectasis
Hypoalbuminemia
Proteinuria
Hyperlipidemia
History of Present Illness
ELBW infant with mild contractures and
distal hyperextensibility
Since her last visit admitted to CNMC for
catheter dysfunction
tPA instillation
Prophylactic Lovenox
Results of Physical Exam
Well looking with normal body
composition
No peritoneal edema
Mild-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 History
Pt 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/day
15 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: NKFA Patient has not been exposed to many food allergens
secondary to dietary restrictions
Labs
Lab 02/11 02/12 02/21 Normal
Range
Sodium H145 139, 142 136 132-143
Potassium H6.2 4.6, 4.7 5.2 3.5-5.8
Chloride H110 H108, 109 104 97-106
BUN H31 H24, 25 H32 1-14
Creatinine H0.6 H1.2, 1.2 H0.7 0.2-0.5
Calcium L7.0 8.1, 8.2 8.6 8.1-11
Total
Protein
L4.5 4.6-7.8
Albumin L1.5 L1.1 L1.4 2.3-4.7
Medications
Medication 400mg Dosage
Albumin infusion 3 times a week
Multivitamin with iron 1 ml daily
Lasix 4mg daily
Bicitra 3mEq 3 times a day,
Spironolactone 2.4 mg twice a day
Lisinopril 0.3 mg twice a day
Cholecalciferol 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 Diagnosis
PES statements
1. 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 Requirements
DRI= 80Kcal/kg/day 80 x 6.7 kg = 536Kcal/day
Calorie 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/day
Based on Holiday-Segar method
Goal is to gain 12g day
Goal is to grow 0.48 mm/day
Weight in Kg Fluid Needs
1-10kg 100ml/kg
Nephrotic Syndrome
General Renal Intervention
Energy DRI
Protein DRI Do not supplement to
replace urinary losses
Sodium 1-3 mEq/kg/day
Potassium Restriction not needed
Phosphorus Restriction not needed
Fluid Will vary according to urine
output consult renal team
Diet Order
17 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