Evidence-based clinical practice guideline for diagnosis of neonatal and infantile cholestasis and management of its anticipated life-threatening conditions

Abstract Background Neonatal and infantile cholestasis (NIC) is a challenging presentation in pediatric medicine, as timely detection of treatable causes, whether surgical or medical, is crucial for better outcomes. Methods Using the GRADE methodology, we prioritized essential clinical health questions and retrieved related recommendations from an available high-quality practice guideline for NIC diagnosis and management of anticipated life-threatening conditions to be applied in our Egyptian healthcare setting. Our panel conducted a comprehensive database search for guidelines, applied eligibility criteria, and performed quality appraisals. An adolopment for an original clinical practice guideline was then undertaken. Discussion Our guideline provided recommendations and tools for implementation regarding the diagnosis and management of anticipated life-threatening conditions of NIC in Egypt. We offered a clinical stepwise approach for infants with NIC, starting with careful history, thorough physical examination, and direct visualization of stool color. After 2 weeks of life, infants with jaundice should undergo fractionation of serum bilirubin. At the first encounter with a cholestatic infant (proven or suspected), Vitamin K 1 should be administered to avoid bleeding. Besides, all age-appropriate immunizations should be given to cholestatic infants if there are no other contraindications. Referral to tertiary care (Pediatric hepatologist) for the proper management of various etiologies is recommended. Conclusion This adapted guideline will aid decision-making related to NIC’s diagnosis and management of anticipated life-threatening conditions in Egyptian primary healthcare settings.