نتایج جستجو برای: facial dysmorphism

تعداد نتایج: 60752  

Journal: :Human molecular genetics 2015
Nadine Rosin Nursel H Elcioglu Filippo Beleggia Pinar Isgüven Janine Altmüller Holger Thiele Katharina Steindl Pascal Joset Anita Rauch Peter Nürnberg Bernd Wollnik Gökhan Yigit

DNA double-strand breaks (DSBs) are highly toxic lesions, which, if not properly repaired, can give rise to genomic instability. Non-homologous end-joining (NHEJ), a well-orchestrated, multistep process involving numerous proteins essential for cell viability, represents one major pathway to repair DSBs in mammalian cells, and mutations in different NHEJ components have been described in microc...

Journal: :Journal of medical genetics 1992
D Viljoen P Beighton

Schwartz-Jampel syndrome is a rare autosomal recessive disorder. Joint contractures, generalised myotonia, skeletal anomalies, and facial dysmorphism are common features; malignant hyperthermia is a potentially lethal complication during anaesthesia.

Journal: :Oman medical journal 2012
Josef Finsterer

The 1.4Mb tandem-duplication in the PMP22 gene at 17p11.2 usually manifests as hereditary sensorimotor polyneuropathy with foot deformity, sensorineural hearing-loss, moderate developmental delay, and gait disturbance. Hypertelorism and marked phenotypic variability within a single family has not been reported. In a single family, the PMP22 tandem-duplication manifested as short stature, sensor...

Journal: :Journal of medical genetics 1993
C E de Die-Smulders R P Droog M van Dijk J P Fryns

A malformed female infant is described. In addition to cardiac, renal, and skeletal (rib) anomalies, severe intrauterine growth retardation and distinct facial dysmorphism were present. The question is raised whether this child represents a new syndrome.

Journal: :Journal of medical genetics 1990
L Turolla M Clementi R Tenconi

A boy presenting with an incomplete form of the acrocallosal syndrome is described. The syndrome shows clinical variability and it is stressed that none of the components is constant and facial dysmorphism is not always characteristic.

Journal: :International Journal of Contemporary Pediatrics 2021

Ectodermal dysplasia is a rare entity with incidence of 1 in 1,00,000 births male predominance. Most commonly it presents appendageal abnormality facial dysmorphism. The two most common types ectodermal dysplasias are hypohidrotic (Christ-Siemens-Touraine syndrome) and hidrotic (Clouston syndrome). Clinical recognition varies depending on severity symptoms associated complications. prognosis go...

2015
Darae Lee Ja Hye Kim Ja Hyang Cho Moon-Yun Oh Beom Hee Lee Gu-Hwan Kim Jin-Ho Choi Han-Wook Yoo

Mowat-Wilson syndrome is an extremely rare genetic disease that is characterized by intellectual disability, facial dysmorphism, Hirschsprung’s disease, and other congenital anomalies. This disorder is caused by heterozygous mutations or deletions in the zinc finger E-box-binding homeobox-2 gene (ZEB2). Thus far, approximately 200 cases of Mowat-Wilson syndrome have been reported worldwide. In ...

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