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Initial treatment of cystic fibrosis. Frequency of transient bulging fontanel

E S Roach1, S H Sinal

  • 1Section of Pediatric Neurology, Bowman Gray School of Medicine of Wake Forest University, Winston-Salem, North Carolina.

Clinical Pediatrics
|August 1, 1989
PubMed

Insights

Newly diagnosed cystic fibrosis infants may experience a bulging fontanel after starting enzyme replacement therapy. This transient symptom, indicative of increased intracranial pressure, typically resolves with continued treatment.

Area of Science:

  • Pediatrics
  • Gastroenterology
  • Neurology

Background:

  • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Infants with CF often require enzyme replacement therapy.
  • Potential side effects of treatments in infants require careful monitoring.

Purpose of the Study:

  • To investigate the occurrence of bulging anterior fontanel in infants with newly diagnosed cystic fibrosis.
  • To determine the relationship between pancreatic enzyme replacement and increased intracranial pressure in this population.
  • To describe the clinical course and resolution of this specific symptom.

Main Methods:

  • Observational study of infants diagnosed with cystic fibrosis.
  • Monitoring for bulging anterior fontanel following initiation of pancreatic enzyme replacement.
  • Clinical assessment, including neuroimaging and cerebrospinal fluid pressure measurements in select cases.

Main Results:

  • Four out of 123 hospitalized infants (3.3%) with newly diagnosed cystic fibrosis developed a bulging anterior fontanel.
  • The symptom appeared within 1-6 days of starting pancreatic enzyme replacement.
  • Cerebrospinal fluid pressure was elevated in two of three infants assessed; neuroimaging was otherwise normal. The condition resolved within weeks to months.

Conclusions:

  • A bulging anterior fontanel can be a transient indicator of increased intracranial pressure in infants with cystic fibrosis treated with pancreatic enzymes.
  • This finding suggests a potential link between enzyme replacement therapy and intracranial pressure changes in young CF patients.
  • Further research is warranted to understand the underlying mechanisms and long-term implications.

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