Persistent hyperinsulinemic hypoglycemia presenting with a rare complication: West syndrome.
BACKGROUND
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is characterized by disproportional secretion of insulin from pancreatic beta-cells. Although one of the manifestations of hypoglycemia is West syndrome, it is rarely reported in PHHI.


METHODS
A 6 month-old girl who was followed up with the diagnosis of PHHI was admitted to hospital with the complaint of jerky movements at her extremities. EEG revealed the typical pattern of hypsarrhythmia leading to the diagnosis of West syndrome.


CONCLUSIONS
To our knowledge, there is only one report in the literature of West syndrome as a manifestation of PHHI, and that was the hyperammoniemic form of the disease. The present report is the first of normoammoniemic PHHI leading to West syndrome. We wish to highlight the potential risks of PHHI, especially in inadequately treated patients, and to emphasize that close neurological follow-up is very important in children who suffer from PHHI.
Camurdan
M Orhun
MO
Department of' Pediatric Endocrinology, Gazi University, Ankara, Turkey.
Cinaz
Peyami
P
Serdaroğlu
Ayşe
A
Bideci
Aysun
A
Demirel
Fatma
F
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D044903:Congenital Hyperinsulinism / Q000150:complications*; D003981:Diazoxide / Q000627:therapeutic use; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D009207:Myoclonus / Q000175:diagnosis / Q000209:etiology*; D009543:Nifedipine / Q000627:therapeutic use; D010634:Phenobarbital / Q000627:therapeutic use; D013036:Spasms, Infantile / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome
D000927:Anticonvulsants; D000324:Adrenocorticotropic Hormone; D009543:Nifedipine; D003981:Diazoxide; D010634:Phenobarbital