leontiasis ossea


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Related to leontiasis ossea: porphyria, platybasia

meg·a·ceph·a·ly

(meg'ă-sef'ă-lē),
A condition, either congenital or acquired, in which the head is abnormally large; usually applied to an adult cranium with a capacity of over 1450 mL.
[mega- + G. kephalē, head]

McCune-Albright syndrome

An autosomal dominant condition (OMIM:174800) due to altered regulation of cAMP, endocrinopathy (e.g., hyperthyroidism) and hypophosphatemia.

Clinical findings
Precocious puberty, polyostotic (cystic fibrous dysplasia) spontaneous fractures at young age, café-au-lait spots on skin, ovarian cysts.
 
Lab
Cyclical 4–6-week fluctuations of plasma oestrogen; afflicted young girls have decreased gonadotropins, decreased response to LH-RH; increased testosterone, increased alkaline phosphatase.
 
Molecular pathology
Defects in GNAS, which encodes a G protein that modulates various membrane signalling cascasdes, cause McCune-Albright syndrome.

Management
Aromatase inhibitor testolactone.

leontiasis ossea

Enlargement and distortion of facial bones, giving one the appearance of a lion. It can occur as a complication of hyperparathyroidism, Paget's disease, uremia, and other conditions.
See also: leontiasis
References in periodicals archive ?
(3,6) The rarest pattern--and that most representative of an advanced disease state--is uremic leontiasis ossea which presents as marked expansion of the mandible and maxilla, occasionally with obliteration of the maxillary sinuses and splaying of the teeth.
Leontiasis ossea is believed to be the rarest and manifests clinically as widening of the interdental spaces, flattening of the nares and nasal bridge, and jaw enlargement.
Leontiasis ossea, slipped epiphyses and granulosa cell tumor of the testis with renal disease: report of a case with autopsy findings.