Can oral rehydration solution powder be given to a child with congenital disorder of glycosylation who has loose stools?

ORS for Loose Stools in Congenital Glycosylation Defects

Yes, oral rehydration solution (ORS) powder can and should be given to children with congenital disorders of glycosylation (CDG) who have loose stools, as ORS is the first-line therapy for dehydration from diarrhea regardless of the underlying cause. 1

Rationale for ORS Use in CDG

The 2017 Infectious Diseases Society of America guidelines explicitly state that reduced osmolarity ORS is recommended as first-line therapy for mild to moderate dehydration in infants, children, and adults with acute diarrhea from any cause. 1 This universal applicability is critical—ORS works through the coupled sodium-glucose cotransport mechanism in the intestinal brush border, which remains intact regardless of the etiology of diarrhea. 1

While congenital disorders of glycosylation affect protein glycosylation and can cause chronic diarrhea through various mechanisms (including intestinal dysfunction), the fundamental principle of fluid and electrolyte replacement remains unchanged. The child still loses water and electrolytes through loose stools and requires replacement to prevent dehydration-related morbidity and mortality.

Specific Dosing Guidelines

For Prevention of Dehydration (No Signs of Dehydration)

• Children <2 years: 50-100 mL of ORS after each loose stool 1
• Children >2 years: 100-200 mL of ORS after each loose stool 1

For Active Rehydration (Mild to Moderate Dehydration)

• Mild dehydration (3-5% deficit): 50 mL/kg ORS over 2-4 hours 2, 3
• Moderate dehydration (6-9% deficit): 100 mL/kg ORS over 2-4 hours 2, 3

For Ongoing Loss Replacement

• 10 mL/kg for each watery stool 2, 3
• 2 mL/kg for each vomiting episode 2, 3

Recommended ORS Formulation

Use low-osmolarity ORS with the following composition: 1, 4

• Sodium: 75-90 mEq/L
• Potassium: 20 mmol/L
• Glucose: 75-111 mmol/L
• Total osmolarity: 245-311 mOsm/L

Commercially available products include Pedialyte, CeraLyte, or Enfalac Lytren. 1, 2 These are preferable to home-prepared solutions as they ensure accurate electrolyte concentrations.

Critical Pitfalls to Avoid

Never Use These Fluids

Do not use apple juice, Gatorade, sports drinks, or soft drinks for rehydration, as they contain inappropriate electrolyte content and excessive osmolality that can worsen diarrhea. 2, 5

Administration Technique

If the child is vomiting, administer small volumes (5-10 mL) every 1-2 minutes via spoon or syringe rather than allowing ad libitum drinking from a cup. 1 This approach successfully rehydrates >90% of children with concomitant vomiting. 1

Feeding Considerations

Continue breast-feeding throughout the illness and resume age-appropriate diet immediately after rehydration. 1 Do not dilute formula or restrict feeding—the outdated practice of "gut rest" provides no benefit and can worsen nutritional status. 1

When to Escalate Care

Switch to intravenous rehydration if: 1, 2

• Severe dehydration with shock or altered mental status is present
• ORS therapy fails after adequate trial
• Intestinal ileus develops (absent bowel sounds)
• The child cannot tolerate oral or nasogastric intake

Consider nasogastric tube administration at 15 mL/kg/hour if the child cannot tolerate oral intake but is not in shock. 1, 2

Special Considerations for CDG

While CDG can cause chronic gastrointestinal manifestations including protein-losing enteropathy and malabsorption, these do not contraindicate ORS use. The only absolute contraindications to ORS are: 4

• Altered mental status
• Complete inability to tolerate oral/nasogastric fluids
• Intestinal ileus
• Severe anatomical gastrointestinal abnormalities

Monitor closely for signs of treatment failure: continued high stool output (>10 mL/kg/hour), persistent dehydration signs after 3-4 hours of ORS therapy, or development of severe lethargy. 1 These warrant reassessment and possible escalation to IV therapy.

The key principle is that ORS addresses the life-threatening complication of dehydration regardless of the underlying disease causing diarrhea. 1 In CDG patients with chronic loose stools, ORS serves both as acute treatment during dehydration episodes and as maintenance therapy to prevent dehydration.