• Title/Summary/Keyword: Hallermann-Streiff syndrome

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A case of Hallermann-Streiff syndrome with aphakia (무수정체안을 동반한 Hallermann-Streiff 증후군 1예)

  • Lee, Myung Chul;Choi, Im Jeong;Jung, Jin Wha
    • Clinical and Experimental Pediatrics
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    • v.51 no.6
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    • pp.646-649
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    • 2008
  • Hallermann-Streiff syndrome is a rare disease. Approximately 150 cases have been reported, including 6 cases in Korea. The authors experienced a case of Hallermann-Streiff syndrome in a 6-year-old female with aphakia. The syndrome is characterized by a bird-like face, dental abnormalities, hypotrichosis, atrophy of the skin, bilateral microphthalmia, and proportionate dwarfism. A brief review of the literature was conducted.

INTERDISCIPLINARY APPROACH A CHILD PATIENT WITH RESTRICTED MOUTH OPENING : A CASE REPORT (개구 장애를 지닌 소아환자를 대상으로한 Interdisciplinary approach)

  • Leem, Cheol-Soo;Kim, Yong-Kee
    • Journal of the korean academy of Pediatric Dentistry
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    • v.21 no.2
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    • pp.605-610
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    • 1994
  • Resricted opening of the mouth in children can be derived from a variety af extra and intra articular causes. A 5-year-old female patient with an elongated right coronoid process and with congenital missing teeth came to clinic for wearing of esthetic denture. A proper medical consultation and laboratory test had done for assessment a systemic syndrome related to her dental anomalies. The result was that her systemic findings dindn't accord specific signs and symptoms of Hallermann-Streiff syndrome. Unilateral coronoidectomy was fulfilled to improve mouth opening and subsequently endodontic and restorative procedure. Maxillary partial denture was delivered for esthetic problem. The periodic recall medical/dental check-up are recommended.

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9 Cases of Non-chromosomal Multiple Malformation Disorders with Psychomotor Retardation (정신운동발달지연을 동반한 염색체이상외 다발성기형질환 9예)

  • Moon, Han-Ku;Park, Yong-Hoon;Hah, Jeong-Ok
    • Journal of Yeungnam Medical Science
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    • v.3 no.1
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    • pp.367-374
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    • 1986
  • To make better clinical diagnosis, authors reviewed 9 patients of nonchromosomal multiple malformation disorders with psychomotor retardation, who were evaluated at pediatric department of Yeungnam University hospital for recent 2 years. We could make clinical diagnosis in 5 patients out of 9 as Aarskog syndrome, Beckwith-Wiedemann syndrome, Hallermann-Streiff syndrome, Rubinstein Taybi syndrome and Weaver syndrome. But even in diagnosed cases, there were many discrepant findings in comparison with typical cases of reference literatures and family history was positive in only one case. Moreover we could not make diagnosis in 4 patients. Therefore we think it is necessary to make a survey of unique pattern, incidence, distribution and etiologic factors of malformation disorders in our country by geneticist and pediatrician as well as to improve the laboratory aids for better diagnosis and genetic counceling.

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