Table 3.
Genetic Mutations and Clinical Characteristics of 3 Reported Patients with HPS-7
Pt | Gene | Mutations | Gender | Age | Ethnicity/Nationality | Consanguinity | Clinical Features | Reference |
---|---|---|---|---|---|---|---|---|
1 | DTNBP1 | c.307C>T; p.Gln103* homozygous | female | 48 yrs | Portuguese | Yes (parents are first cousins) | Oculocutaneous albinism, bleeding tendency | 10 |
2 | DTNBP1 | c.177G>A;p.Trp59* homozygous | female | 77 yrs | Caucasian | Yes (parents are first cousins) | Oculocutaneous albinism, bleeding tendency, colitis | 13 |
3 | DTNBP1 | c.307C>T; p.Gln103* homozygous | male | 6 yrs | Paraguayan | No | Oculocutaneous albinism, easy bruising, mild delay in motor and language development | current paper |
Mutation nomenclature is based on accession NM_032122.4.