Table 1. Summary of clinical features of the affected proband (11) identified in the present study and those previously reported probands (1–10).
Proband | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
Features | HPO | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 |
Sex | Male | Male | Male | Male | Male | Male | Male | Male | Male | Male | Male | |
Age (years) | 15 | 13 | 5 | 6 | 22 | 11 | UNK | UNK | UNK | UNK | 4 | |
TAF1 Variant (hg19) | p.Ile1337Thr | p.Ile1337Thr | p.Cys807Arg | p.Arg1246Trp | p.Asn1517His | p.Arg1431His | p.Met21Leu | p.Glu1428Pro | p.Asn493Asp | p.Arg1190Cys | p.Ser1600Gly | |
Inheritance pattern | Maternal | Maternal | De novo | De novo | De novo | De novo | Maternal | Maternal | Maternal | Maternal | Maternal | |
Delayed gross motor development | HP:002194 | + | + | + | ++ | + | + | UNK | UNK | UNK | UNK | + |
Delayed speech and language development | HP:0000750 | + | + | + | ++ | + | + | UNK | UNK | UNK | UNK | + |
Intellectual disability | HP:0001249 | + | + | + | + | + | + | + | + | + | + | + |
Prominent supraorbital ridges | HP:0000336 | + | + | - | + | + | - | UNK | UNK | UNK | UNK | + |
Prominent forehead | HP:0011220 | + | + | + | - | - | + | UNK | UNK | UNK | UNK | + |
Long philtrum | HP:0000343 | + | + | - | + | - | + | UNK | UNK | UNK | UNK | + |
Thickened helices | HP:0009748 | + | + | - | - | + | + | UNK | UNK | UNK | UNK | + |
Macrotia | HP:0000400 | + | + | + | + | + | + | UNK | UNK | UNK | UNK | + |
Broad upturned nose | HP:0000463 | + | + | + | + | - | + | UNK | UNK | UNK | UNK | + |
Bulbous nasal tip | HP:0000414 | + | + | + | + | - | + | UNK | UNK | + | + | + |
Myopia | HP:0000545 | - | - | + | + | + | - | UNK | UNK | UNK | UNK | + |
Hypoplasia of the cerebellar vermis | HP:0001320 | + | + | ++ | - | - | - | UNK | UNK | UNK | UNK | + |
Hypoplasia of the corpus callosum | HP:0002079 | + | + | + | UNK | + | UNK | UNK | UNK | UNK | UNK | + |
Hypotonia | HP:0001290 | + | + | + | + | + | - | UNK | UNK | UNK | UNK | + |
Hyporeflexia | HP:0001315 | + | + | + | + | + | + | UNK | UNK | UNK | UNK | + |
Gait abnormalities | HP:0001288 | + | + | + | - | + | - | UNK | UNK | UNK | UNK | + |
Balance problems | HP:0002141 | + | + | - | + | + | - | UNK | UNK | UNK | UNK | + |
Postural instability | HP:0001251 | + | + | - | - | + | - | UNK | UNK | UNK | UNK | + |
Bradykinesia | HP:0002067 | - | - | - | - | + | - | UNK | UNK | UNK | UNK | + |
Short digits | HP:0011927 | - | - | + | - | - | + | UNK | UNK | UNK | UNK | + |
Autistic behaviors | HP:0000729 | + | + | + | - | + | + | UNK | UNK | UNK | UNK | - |
Recurrent hand flapping | HP:0100023 | UNK | UNK | UNK | UNK | UNK | UNK | UNK | UNK | UNK | UNK | + |
‘+’ indicates mild or simple presence of the phenotype; ‘++’ indicates a more pronounced presence of the phenotype; ‘–’ indicates an absent feature. Abbreviation: UNK, unknown.