[Optic nerve hypoplasia and growth hormone deficiency: de Morsier's syndrome].
The association of optic nerve hypoplasia and deficiency of the septum pellucidum and hypopituitarism is known as the De Morsier syndrome or septo-optic dysplasia. Four children with blindness and growth retardation due to growth hormone deficiency are described. Frequent recurrent episodes of severe hypoglycaemia, starting in the newborn period, were observed in three of them. Although visual impairment was noted during the first year and growth failure was detected between the first and sixth year of life in all four patients, the diagnosis of septo-optic dysplasia was delayed for two to six years. Early diagnosis and treatment with growth hormone should prevent attacks of hypoglycaemia and their sequelae and bring about normal growth.
D000015:Abnormalities, Multiple / Q000209:etiology; D001766:Blindness / Q000209:etiology; D002648:Child; D004392:Dwarfism / Q000209:etiology; D005260:Female; D013006:Growth Hormone / Q000172:deficiency*; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009759:Nystagmus, Pathologic / Q000209:etiology; D009900:Optic Nerve / Q000002:abnormalities*; D012688:Septum Pellucidum / Q000002:abnormalities*; D013577:Syndrome
D013006:Growth Hormone
Optikushypoplasie und Wachstumshormonmangel: De-Morsier-Syndrom.