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Diencephalic syndrome of infancy

MedGen UID:
543636
•Concept ID:
C0271889
•
Disease or Syndrome
Synonyms: Diencephalic Syndrome; Diencephalic Syndrome of Infancy
SNOMED CT: Diencephalic syndrome of infancy (45740001)

Definition

Emaciation in the setting of normal linear growth and intellectual development, which is usually associated with neoplasms involving the anterior hypothalamus in infancy or early childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiencephalic syndrome of infancy

Professional guidelines

PubMed

The diencephalic syndrome of early infancy due to silent brain tumor: with special reference to treatment.
Bain HW, Darte JM, Keith WS, Kruyff E
Pediatrics 1966 Sep;38(3):473-82. PMID: 5918397

Recent clinical studies

Diagnosis

"Failure to thrive" due to pontine glioma.
Maroon JC, Albright L
Arch Neurol 1977 May;34(5):295-7. doi: 10.1001/archneur.1977.00500170049009. PMID: 67836
Failure to thrive: the diencephalic syndrome of infancy and childhood.
Smith KR Jr, Weinburg WA, McAlister WH
J Neurosurg 1965 Sep;23(3):348-51. doi: 10.3171/jns.1965.23.3.0348. PMID: 5294539

Prognosis

Hypothalamic symptomatology and its relationship to diencephalic tumor in childhood.
Connors MH, Sheikholislam BM
Childs Brain 1977;3(1):31-6. doi: 10.1159/000119647. PMID: 844344

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