Syndrome de résistance à l’Adrénocorticotrophine Hormone (ACTH): à propos d’un cas

  • Morgiane Solange Tognidé Sêlomin Houngbadji
  • Babacar Niang
  • Djibril Boiro
  • Aminata Mbaye
  • Abdoulaye Seck
  • Abdoulaye Aliou Ndongo
  • Indou Deme Ly
  • Ousmane Ndiaye
Keywords: ACTH resistance, cortisol deficiency, hypoglycaemia

Abstract

Adrenocorticotropic hormone (ACTH) insensitivity syndrome is one of the rare causes of adrenal insufficiency in children. All described inherited ACTH insensitivity forms are of autosomal recessive origin. In our resource-poor Countries, many of these rare diseases are ignored or not diagnosed due to inadequate technical equipments. We report the case of a 4-month old infant hospitalized for refractory hypoglycaemias. Despite the patient had generalized and severe melanodermia, digestive disorders and ion channel disorders the diagnosis of cortisol deficiency was only diagnosed retrospectively during respiratory arrest with favorable outcome under hydrocortisone therapy. This study aims to highlight the clinical, laboratory and therapeutic features of peripheral cortisol deficiency, without enzymatic blocks, including this adrenocorticotropic hormone (ACTH) insensitivity syndrome.

Keywords: ACTH resistance, cortisol deficiency, hypoglycaemia

Author Biographies

Morgiane Solange Tognidé Sêlomin Houngbadji
Centre Hospitalier National d’Enfants Albert Royer-Fann, Dakar, Sénégal
Babacar Niang
Centre Hospitalier National d’Enfants Albert Royer-Fann, Dakar, Sénégal
Djibril Boiro
Service de Pédiatrie du Centre Hospitalier Abass Ndao de Dakar, Dakar, Sénégal
Aminata Mbaye
Centre Hospitalier National d’Enfants Albert Royer-Fann, Dakar, Sénégal
Abdoulaye Seck
Institut Pasteur de Dakar, Dakar, Sénégal
Abdoulaye Aliou Ndongo
Aristide Le Dantec, CHU de Dakar, Dakar, Sénégal
Indou Deme Ly
Centre Hospitalier National d’Enfants Albert Royer-Fann, Dakar, Sénégal
Ousmane Ndiaye
Centre Hospitalier National d’Enfants Albert Royer-Fann, Dakar, Sénégal
Published
2019-02-18
Section
Articles

Journal Identifiers


eISSN: 1937-8688