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1.

Microcephaly

A congenital abnormality in which the CEREBRUM is underdeveloped, the fontanels close prematurely, and, as a result, the head is small. (Desk Reference for Neuroscience, 2nd ed.)

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Oculodigitoesophagoduodenal syndrome [Supplementary Concept]

Digital anomalies with short palpebral fissures and atresia of esophagus, or duodenum; mutation in MYCN

Date introduced: August 25, 2010

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Galloway Mowat syndrome [Supplementary Concept]

Date introduced: August 25, 2010

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Nijmegen Breakage Syndrome

A chromosome instability syndrome resulting from a defective response to DNA double-strand breaks. In addition to characteristic FACIES and MICROCEPHALY, patients have a range of findings including RADIOSENSITIVITY, immunodeficiency, increased cancer risk, and growth retardation. Causative mutations occur in the NBS1 gene, located on human chromosome 8q21. NBS1 codes for nibrin, the key regulator protein of the R/M/N (RAD50/MRE11/NBS1) protein complex which senses and mediates cellular response to DNA DAMAGE caused by IONIZING RADIATION.

Year introduced: 2006

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Microcephaly, corpus callosum dysgenesis and cleft lip-palate [Supplementary Concept]

Corpus callosum dysgenesis, microcephaly, infantile spasm, cleft lip-palate, exophthalmos and psychomotor retardation

Date introduced: August 25, 2010

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Microcephaly, Primary Autosomal Recessive, 1 [Supplementary Concept]

mutationin MCPH1

Date introduced: November 5, 2012

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Filippi syndrome [Supplementary Concept]

Short stature, microcephaly, characteristic face, syndactyly and mental retardation; Unusual facial appearance, microcephaly, growth and mental retardation, and syndactyly

Date introduced: August 25, 2010

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