leontiasis ossea

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. Synonym(s): leontiasis ossea, macrocephaly, macrocephalia, megacephalia, megalocephaly, megalocephalia, Virchow disease [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

单词	leontiasis ossea
释义	DictionarySeemegacephaly leontiasis ossea 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. Synonym(s): leontiasis ossea, macrocephaly, macrocephalia, megacephalia, megalocephaly, megalocephalia, Virchow disease [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
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