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[Holoprosencephaly with neurogenic hypernatremia]

J M Cuisset1, J C Cuvellier, L Vallée

  • 1Service des maladies infectieuses et de neurologie infantiles, CHRU de Lille, hôpital Roger-Salengro, Lille.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|February 12, 1999
PubMed
Summary

Neurogenic hypernatremia, a rare complication of semi-lobar holoprosencephaly, presents without dehydration signs. Differentiating it from other hypernatremia causes is crucial as it lacks specific complications.

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Area of Science:

  • Pediatric Neurology
  • Endocrinology
  • Genetics

Background:

  • Semi-lobar holoprosencephaly is a rare congenital brain malformation.
  • Neurogenic hypernatremia can be an infrequent complication in these patients.
  • Distinguishing neurogenic hypernatremia from other causes is clinically important.

Observation:

  • Two pediatric cases of semi-lobar holoprosencephaly were analyzed.
  • Patients presented with chronic hypernatremia and hyperosmolarity.
  • Clinical signs of dehydration were notably absent.

Findings:

  • The hypernatremia was ultimately attributed to a neurogenic origin.
  • This highlights a specific presentation of neurogenic hypernatremia.
  • The condition was observed in children aged 7 months and 4 years.

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Implications:

  • Accurate diagnosis of neurogenic hypernatremia is essential.
  • It requires differentiation from other hypernatremia etiologies.
  • This condition does not present with specific, characteristic complications.