Nutrition Implications of Congenital Nephrotic Syndrome

1. Nutrition Implications of Congenital Nephrotic Syndrome Vehik Nazaryan UMD-CP Dietetic Intern 03/20/2014

2. Overview • Definition of NS • Causes • Symptoms • Clinical Manifestation of NS • Diagnosis • Treatment • Prognosis • Case Report • Medical and Diet History • Nutrition Diagnosis • Goals

3. Congenital Nephrotic Syndrome What is it? • Is a Is a very rare form of nephrotic syndrome, mostly in families of Finish origin that developesshortly 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

4. Causes • It is inherited, passed down through families • Long arm of Chromosome 19th • Abnormal form of protein called nephrin

5. Symptoms • Cough • Decreased urine output • Foamy appearance of urine • Low Birth Weight • Poor appetite • Swelling(total body) Signs of disease

6. 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) • MicrocyticHypochromic Anemia—due to transferrin loss • Erythrocyte sedimentation rate is increased due to increased fibrinogen and other plasma contents

7. 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

8. 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

9. 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

10. Prognosis • Disorder leads to • Infection • Malnutrition • Kidney failure • Death by age 5-many children die within the fist year

11. Tests Following tests should be performed: • Complete blood count • Metabolic panel • Levels of serum electrolytes • Calcium • Phosphorus • BUN • Creatinine

12. 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

13. Weight and Height • Weight: 6.7 kg • less than 5th Percentile • Corrected weight percentile: 5thpercentil • Weight age: 5th percentile • Height : 60.5 cm • Percentile: less than 5th percentile • Corrected height percentile: less than 5thpercentile • Weight/Height percentile:50th percentile

14. History of Present Illness • Prenatal and postnatal mild bilateral pelviectasis • Hypoalbuminemia • Proteinuria • Hyperlipidemia

15. 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

16. 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

17. Growth Charts Weight 5th% Corrected age Age

18. Growth Charts Weight (Kg) 5th% 75th% 25th Length(cm)

19. 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.

20. 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

21. Labs

22. Medications

23. 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.

24. 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

25. Nephrotic SyndromeGeneral Renal Intervention

26. 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

27. Formula provides: • Energy: 114kcal/kg = 764kcal/day • Protein:2.3 g/kg = 15.4g/day • Fluids: 653ml/day

28. 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