Using Renastart™ in the management of congenital nephrotic syndrome | Case Study

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DESCRIPTION

A case study on the use of Renastart in the management of an infant with congenital nephrotic syndrome.

The patient is a 4 week old female who was diagnosed with congenital nephrotic syndrome.

 

 

Weight (Kg): 3.7

Percentile on the growth chart: 50th

The child presented with acute renal failure, oliguria and nephrotic range proteinuria at the age of 4 weeks.

Biochemistry at diagnosis

Potassium (mmol/l) ⬆ 7.5 Ref Range* (3.6-4.8)
Phosphate (mg/dl) ⬆ 10.7 Ref Range* (3.1-7.4)
Urea (mg/dl) ⬆ 99 Ref Range* (6-45)
Creatinine (mg/dl) ⬆ 1.32 Ref Range* (0.17-0.42)
GFR (ml/min) 17

Medical management

The child was commenced on peritoneal dialysis (PD) and intubated (due to metabolic acidosis). Total Parenteral Nutrition (TPN) was initiated as per hospital protocol.

After stabilisation, the child was started on expressed breast milk via nasogastric tube. Blood potassium, phosphate and urea levels returned to be within reference range. Urine production was normal and TPN stopped.

 

Biochemistry after initial management and stabilisation

Potassium (mmol/l) 3.8 Ref Range* (3.6-4.8)
Phosphate (mg/dl) 4.9 Ref Range* (3.1-7.4)
Urea (mg/dl) 12 Ref Range* (6-45)
Creatinine (mg/dl) ⬆ 0.72 Ref Range* (0.17-0.42)
GFR (ml/min) 34

* Hospital reference ranges.

Weight (Kg): 4.1

Percentile on the growth chart: 3rd

At 9 weeks of age, the child’s kidney function started to decline (chronic kidney disease). Dietetic intervention was necessary as blood potassium level started to increase.

Biochemistry

Potassium (mmol/l) ⬆ 5.0 Ref Range* (3.6-4.8)
Phosphate (mg/dl) ⬇ 3.0 Ref Range* (3.1-7.4)
Urea (mg/dl) 26 Ref Range* (6-45)
Creatinine (mg/dl) ⬆ 0.82 Ref Range* (0.17-0.42)
GFR (ml/min) 29

Calculation of nutritional requirements

96-100 kcal/kg/day1 . 2.4 g protein/kg1 . Potassium restriction required due to raised blood potassium. No fluid restriction was in place.

Dietary intake

552 ml expressed breast milk at 23 ml/hr. Started at 5 ml/hr and gradually increased to 23 ml/hr with the aim to further increase the rate until energy requirements were met.

Feed provides

Energy 382 kcal (93 kcal/kg)
Protein 6.24 g (1.5 g/kg)
Potassium 260 mg (1.6 mmol/kg)
Phosphorus 83 mg
Sodium 72 mg (0.8 mmol/kg)

Aim

Provide an enteral feed which is lower in potassium but still provides enough protein and phosphate.

Feeding plan

Tube feeding – continuous 23 ml/hr – Use Renastart exclusively for a limited period of time to reduce potassium intake and thus blood potassium levels.

23 ml/hr of 20% Renastart for 24 hours = 552 ml

Feed provides

Energy 522 kcal (135 kcal/kg)
Protein 8.28 g (2.0 g/kg)
Potassium 121 mg (0.8 mmol/kg)
Phosphorus 105 mg
Sodium 265 mg (2.8 mmol/kg)

The child’s electrolytes were monitored regularly.

Biochemistry after first dietetic intervention

Potassium (mmol/l) ⬇ 3.0 Ref Range* (3.6-4.8)
Phosphate (mg/dl) 4.4 Ref Range* (3.1-7.4)
Urea (mg/dl) 45 Ref Range* (6-45)
Creatinine (mg/dl) ⬆ 1.39 Ref Range* (0.17-0.42)
GFR (ml/min) 17

After 24 hours, the child’s blood potassium level had reduced to below the reference range. Therefore, the feeding regime was adjusted to increase the potassium intake.

Adjusted feeding regimen

Feeding plan:

Tube feeding - continuous 23 ml/hr - 276 ml Renastart and 276 ml breast milk

over 24hrs = 552 ml

55 g Renastart (20% dilution) + 276 ml breast milk
 

New feeding regimen provides

Energy 465 kcal (113 kcal/kg)
Protein 7.58 g (1.85 g/kg)
Potassium 191 mg (1.2 mmol/kg)
Phosphorus 94 mg
Sodium 168 mg (1.8 mmol/kg)

 

Biochemistry after second dietetic intervention

Potassium (mmol/l) 3.8 Ref Range* (3.6-4.8)
Phosphate (mg/dl) 4.4 Ref Range* (3.1-7.4)
Urea (mg/dl) ⬇ 1 Ref Range* (6-45)
Creatinine (mg/dl) ⬆ 1.73 Ref Range* (0.17-0.42)
GFR (ml/min) 16

The child’s potassium level increased to be within the normal range. The child’s electrolyte levels continued to be monitored regularly and feed was adjusted as needed following regular dietetic reviews.

Weight (Kg) 8.3
Height (cm) 75.5
Percentile on the growth chart Weight: < 3rd
Height: < 3rd

At 2 years of age, haemodialysis rather than PD was commenced and the child had a total nephrectomy. A fluid restriction was in place and the blood results showed an increasing phosphate and potassium level.

Biochemistry

Potassium (mmol/l) ⬆ 5.5 Ref Range* (3.6-4.8)
Phosphate (mg/dl) ⬆ 2.22 Ref Range* (1-1.9)
Urea (mg/dl) ⬆ 117 Ref Range* (12-48)
Creatinine (mg/dl) ⬆ 3.55 Ref Range* (0.24-0.41)
GFR (ml/min) 12

Calculation of nutritional requirements

78-82 kcal/kg/day1 . 1.2 g protein/kg1 . Potassium and phosphate restriction required due to raised blood potassium / phosphate. Fluid restriction max 500 mL/day. Phosphate: < 400 mg/day.

Medications

Medication Reason for use
Alphaleo Active vitamin D supplement
Omeprazole Gastroesophageal reflux
Soluvit Water soluble vitamins
Calcium Carbonate Phosphate binder
Kayexalate Potassium binder
Erythropoeitin Promote red blood cell production
Venofer Iron deficiency anaemia
Genotonorm Human growth hormone
Forlax Laxative
Amlodipine High blood pressure

Feeding regimen

95 g standard infant formula, 5 g protein supplement, 25 g maltodextrin (5% dilution) made up to 500 ml with water.

Feed provides

Energy 606 kcal (60 kcal/kg)
Protein 14.27 g (1.43 g/kg)
Potassium 554 mg (1.42 mmol/kg)
Phosphorus 393 mg
Sodium 190 mg (0.83 mmol/kg)

The child required more energy and protein but less phosphate.

Therefore, feeding regimen was adapted:

35 g Renastart, 78 g standard infant formula, 10 ml fat emulsion, 5 g protein supplement, 25 g maltodextrin (5% dilution) made up to 500 ml with water.

Feed provides

Energy 735 kcal (86 kcal/kg)
Protein 15.10 g (1.82 g/kg)
Potassium 494 mg (1.53 mmol/kg)
Phosphorus 361 mg
Sodium 240 mg (2.55 mmol/kg)

Biochemistry after starting the new adapted feeding regimen:

Potassium (mmol/l) 4.3 Ref Range* (3.6-4.8)
Phosphate (mg/dl) 1.65 Ref Range* (1-1.9)
Urea (mg/dl) ⬆ 137 Ref Range* (12-48)
Creatinine (mg/dl) ⬆ 1.73 Ref Range* (0.17-0.42)
GFR (ml/min) 16

The child had regular dietetic reviews with the feed being adjusted as needed according to the biochemistry and nutritional requirements. The child had a transplant at age 4.

* Hospital reference ranges

Renastart is specifically designed for the dietary management of paediatric kidney disease. It can be used when a lower intake of potassium and phosphate is needed, whilst still providing other essential nutrients such as energy, protein, vitamins and minerals, as demonstrated in this case study.

1. Royle, J. Chapter 12: Kidney Disease. In: Shaw V, editor. Clinical Paediatric Dietetics. 4: John Wiley & Sons Ltd.; 2015. p. 242-81.

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