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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.
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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 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
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) • MicrocyticHypochromic 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: 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
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 Weight 5th% Corrected age Age
Growth Charts Weight (Kg) 5th% 75th% 25th Length(cm)
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
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
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