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[Clinical and histopathologic polymorphism in cystic fibrosis]
Revista De Pediatrie, Obstetrica Si Ginecologie. Pediatria
|January 1, 1989
Summary
Cystic fibrosis (CF) in infants often presents with liver and intestinal issues. Hepatic steatosis, linked to malabsorption, is a key finding, with early diagnosis crucial for managing this complex pediatric disease.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Genetic Disorders
Context:
- Cystic fibrosis (CF), also known as mucoviscidosis, is a multi-system genetic disorder.
- Infants with CF exhibit diverse clinical and pathological presentations, particularly affecting the liver and intestines.
- Recent data on CF pathophysiology highlights the need for detailed anatomoclinical studies.
Purpose:
- To conduct an anatomoclinical study of 30 infants diagnosed with mucoviscidosis.
- To emphasize the clinical and pathohistologic polymorphism of CF, focusing on hepatic and intestinal involvement.
- To investigate the early onset and specific types of hepatic lesions in infants with CF.
Summary:
- The study analyzed 30 infants (13 neonates) with CF, noting varied presentations.
- Specific hepatic lesions (Bodian cirrhosis, mucus stoppers) occurred in 10%, while nonspecific lesions like hepatic steatosis were dominant.
- Atrophy of intestinal villi in hepatic steatosis suggests malabsorption. Early hepatic lesions and clinical expression indicate disease severity.
Impact:
- Highlights hepatic steatosis and intestinal villi atrophy as indicators of malabsorption in pediatric CF.
- Underscores the importance of hepatic biopsy for diagnosing liver involvement in CF.
- Emphasizes that early clinical signs, especially hepatic steatosis and dystrophy in the first six months, warrant high suspicion for CF etiology.