Management of Protein-Losing Enteropathy in a 44-Day-Old Infant
The immediate management for protein-losing enteropathy (PLE) in a 44-day-old infant should focus on rehydration, nutritional support with a specialized formula, and identification of the underlying cause.
Initial Assessment and Stabilization
- Evaluate for signs of dehydration: general appearance, eyes, mucous membranes, tears, capillary refill time, urine output, heart rate, and blood pressure 1
- Assess severity of hypoproteinemia and presence of edema
- Monitor vital signs, including weight (important marker for fluid status)
- Check serum albumin, total protein, and electrolytes
Immediate Nutritional Management
Formula Selection
- Switch to an amino acid-based formula (AAF) as first-line treatment 2
- AAFs are the only completely non-allergenic formulas and are effective in patients with failure to thrive
- Extensively hydrolyzed formulas may be considered if AAF is not available, but have higher risk of reactivity
Feeding Approach
- Continue feeding during episodes of loose stools, as withholding feeds can reduce enterocyte renewal and increase intestinal permeability 1
- Consider nasogastric tube feeding if unable to meet nutritional requirements orally 1
- Provide increased caloric intake, starting with 120 kcal/kg per day for "catch-up" weight gain, potentially increasing to 150 kcal/kg/day 1
Special Dietary Considerations
- If lymphangiectasia is suspected as the cause of PLE:
Fluid and Electrolyte Management
- For mild to moderate dehydration: oral rehydration therapy (ORT) with oral rehydration solution (ORS) 1
- For severe dehydration: initial fluid bolus of 20 ml/kg IV 1
- Administer 10 ml/kg of ORS for each liquid stool and 2 ml/kg for each episode of vomiting 1
- Continue rapid fluid replacement until clinical signs of hypovolemia improve, targeting adequate central venous pressure and urine output >0.5 ml/kg/h 1
Diagnostic Workup
- Confirm PLE diagnosis with fecal alpha-1-antitrypsin clearance 4, 5
- Rule out other causes of hypoproteinemia:
- Malnutrition
- Proteinuria (kidney disease)
- Impaired protein synthesis (liver disease)
- Protein loss through skin 4
- Identify underlying cause:
- Stool cultures for infectious causes
- Serologic evaluation for inflammatory conditions
- Cardiac screening (echocardiography) to rule out cardiac causes 2
- Endoscopy with biopsy if gastrointestinal inflammation suspected
- Consider abdominal scintigraphy with Tc99m-labeled human serum albumin to assess and localize protein loss through GI tract 6
Supportive Care
- Supplement fat-soluble vitamins (A, D, E, K) as these may be malabsorbed 3
- Monitor for micronutrient deficiencies, especially in patients on dietary fat restriction 3
- Consider supplemental iron (1 mg/kg/d) by 6 months of age 2
- Monitor growth (weight and height/length) regularly 2
Treatment Based on Underlying Cause
If Food Protein-Induced Enterocolitis Syndrome (FPIES) is suspected:
- Eliminate suspected trigger foods 2
- Recognize that tolerance typically develops by 1-2 years of age for milk/soy FPIES 2
If Cardiac Cause (e.g., post-Fontan procedure):
- Cardiac consultation for management of underlying cardiac condition 2
If Inflammatory Bowel Disease:
- Anti-inflammatory medications as appropriate 7
- In severe cases with obstruction, surgical consultation may be needed 7
Monitoring and Follow-up
- Regular assessment of:
- Serum albumin and total protein levels
- Growth parameters (weight, length/height)
- Nutritional status
- Development of complications (edema, infections)
Prognosis
Prognosis depends on the underlying cause of PLE. With appropriate nutritional support and treatment of the underlying condition, many infants can achieve resolution of protein losses 4.