Should a child with congenital disorder of glycosylation (CDG) receive oral rehydration solution for dehydration caused by diarrhea?

Oral Rehydration Therapy for Children with Congenital Disorders of Glycosylation

Yes, a child with congenital disorder of glycosylation (CDG) should receive oral rehydration solution (ORS) for dehydration caused by diarrhea, as ORS is the first-line treatment for mild-to-moderate dehydration regardless of the underlying cause of diarrhea. 1

Why ORS Works in CDG

• The sodium-glucose cotransport mechanism in the intestinal brush border remains intact during diarrhea regardless of etiology, including CDG-related diarrhea. 1
• ORS addresses the life-threatening complication of dehydration independent of the disease mechanism causing the diarrhea. 2
• The Infectious Diseases Society of America gives a strong recommendation (high-quality evidence) that reduced-osmolarity ORS is first-line therapy for mild-to-moderate dehydration in children with acute diarrhea from any cause. 1

Clinical Context for CDG Patients

• Children with CDG frequently present with gastrointestinal manifestations including protein-losing enteropathy (seen in CDG types Ia, Ib, and Ic), failure to thrive, and hepatopathy. 3, 4
• Protein-losing enteropathy occurs in approximately 29% of CDG patients across types Ia, Ib, and Ic, making fluid and electrolyte management particularly critical. 4
• The multisystem nature of CDG does not contraindicate ORS use; rather, it makes prevention of dehydration-related morbidity even more essential. 2

Dosing Algorithm Based on Dehydration Severity

Mild Dehydration (3-5% fluid deficit)

• Administer 50 mL/kg of ORS over 2-4 hours. 2, 5
• After rehydration, give 50-100 mL ORS after each loose stool for children under 2 years. 2
• For children ≥2 years, give 100-200 mL ORS after each loose stool. 2

Moderate Dehydration (6-9% fluid deficit)

• Administer 100 mL/kg of ORS over 2-4 hours. 2, 5
• Replace ongoing losses with 10 mL/kg for each watery stool and 2 mL/kg for each vomiting episode. 2, 5

Severe Dehydration (≥10% fluid deficit, shock, altered mental status)

• Begin with intravenous isotonic crystalloid (lactated Ringer's or normal saline) until pulse, perfusion, and mental status normalize. 1
• Transition to ORS to complete rehydration once the child is stable. 1

Recommended ORS Formulation

• Use low-osmolarity ORS containing 75-90 mEq/L sodium for active rehydration. 2, 5
• Commercial preparations meeting these specifications include Pedialyte, CeraLyte, and Enfalac Lytren. 1
• For maintenance therapy after rehydration, solutions with 40-60 mEq/L sodium are appropriate, but supplement with low-sodium fluids (breast milk, formula, or water) to prevent sodium overload. 5
• When purging rate is very high (>10 mL/kg/hour), solutions with 75-90 mEq/L sodium are mandatory. 5

Administration Technique for Vomiting Children

• If the child is vomiting, give small aliquots of 5-10 mL every 1-2 minutes using a spoon or syringe rather than allowing ad-libitum drinking. 1, 2, 5
• This technique successfully rehydrates >90% of children with concurrent vomiting. 1, 2
• A common pitfall is allowing a thirsty child to drink large volumes rapidly, which exacerbates vomiting. 1, 5
• If oral intake fails, consider nasogastric tube administration at 15 mL/kg/hour before escalating to IV therapy. 1, 5

Nutritional Management During Diarrhea

• Continue breastfeeding throughout the diarrheal episode without interruption. 1, 5
• Resume full-strength formula immediately after rehydration for bottle-fed infants; do not dilute formula. 1, 5
• Resume an age-appropriate usual diet during or immediately after rehydration is completed. 1
• "Gut rest" offers no benefit and may worsen nutritional status, which is particularly concerning in CDG patients who often have baseline failure to thrive. 1, 4

Absolute Contraindications to ORS

• Intestinal ileus (absent bowel sounds)—do not give oral fluids until bowel sounds return. 1, 2, 5
• Severe dehydration with shock or altered mental status—begin with IV rehydration first. 1
• Inability to tolerate oral or nasogastric intake despite proper technique. 2, 5

Monitoring for Treatment Failure

• Reassess hydration status after 2-4 hours of ORS therapy. 5
• Escalate to IV therapy if any of the following occur:
  • Stool output >10 mL/kg/hour despite adequate ORS replacement. 2, 5
  • Persistent signs of dehydration after 3-4 hours of appropriate ORS administration. 2
  • Development of severe lethargy, altered mental status, or shock. 2, 5
  • Emergence of intestinal ileus. 2, 5

Critical Pitfalls to Avoid

• Never use apple juice, sports drinks (Gatorade), or soft drinks for rehydration—their high osmolarity and inappropriate electrolyte composition can worsen diarrhea. 1, 2
• Do not withhold ORS simply because the child has an underlying metabolic disorder like CDG; the sodium-glucose cotransport mechanism functions independently of glycosylation defects. 1, 2
• When using ORS packets, provide detailed written and oral instructions to prevent mixing with inappropriate volumes of water. 1, 5
• Monitor for glucose malabsorption (rare, ~1% of cases): if stool output dramatically increases with ORS administration and decreases with IV therapy, true glucose malabsorption may be present. 1

Special Considerations for CDG Patients

• Given that CDG patients may have baseline hepatopathy, coagulopathy, and protein-losing enteropathy, maintaining adequate hydration is critical to prevent worsening of these conditions. 3, 4
• The presence of CDG does not alter the fundamental approach to rehydration; the same evidence-based ORS protocols apply. 2
• If the CDG patient has known hepatic fibrosis or steatosis, avoiding dehydration-related hypoperfusion is particularly important to prevent hepatic decompensation. 3, 4