[A case of Prader-Willi syndrome]. 1989

S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda

Prader-Willi syndrome is characterized by hypotonia, hypomentia, hypogonadism and obesity. A case of a 10-year-5-month-old girl who was diagnosed to have the typical symptoms associated with the Prader-Willi syndrome is described in the present report. The following are the dental findings. 1) Enamel hypoplasia, crowding over the anterior teeth and narrow dental arch were found. However there was no high palate in the maxilla. 2) The mesio-distal width of the present teeth were small compared with the national average. 3) According to X-ray cephalometric analysis, a retardation of the growth of the maxilla and mandible was found. 4) Because of hypomentia and the difficult management of the patient, dental treatment was performed under general anesthesia.

UI MeSH Term Description Entries
D008445 Maxillofacial Development The process of growth and differentiation of the jaws and face. Development, Maxillofacial,Developments, Maxillofacial,Maxillofacial Developments
D011218 Prader-Willi Syndrome An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229) Labhart-Willi Syndrome,Royer Syndrome,Labhart-Willi-Prader-Fanconi Syndrome,Prader Labhart Willi Syndrome,Prader-Labhart-Willi Syndrome,Royer's Syndrome,Willi-Prader Syndrome,Labhart Willi Prader Fanconi Syndrome,Labhart Willi Syndrome,Prader Willi Syndrome,Royers Syndrome,Syndrome, Labhart-Willi,Syndrome, Labhart-Willi-Prader-Fanconi,Syndrome, Prader-Labhart-Willi,Syndrome, Prader-Willi,Syndrome, Royer,Syndrome, Royer's,Syndrome, Willi-Prader,Willi Prader Syndrome
D002508 Cephalometry The measurement of the dimensions of the HEAD. Craniometry
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003744 Dental Enamel Hypoplasia An acquired or hereditary condition due to deficiency in the formation of tooth enamel (AMELOGENESIS). It is usually characterized by defective, thin, or malformed DENTAL ENAMEL. Risk factors for enamel hypoplasia include gene mutations, nutritional deficiencies, diseases, and environmental factors. Enamel Hypoplasia,Enamel Agenesis,Enamel Hypoplasia, Dental,Hypoplasia, Dental Enamel,Hypoplastic Enamel,Agenesis, Enamel,Enamel Ageneses,Enamel Hypoplasias,Enamel, Hypoplastic,Hypoplasia, Enamel
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

Related Publications

S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
October 1991, East African medical journal,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
December 2006, Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
January 1965, Pediatrie,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
January 2013, Enfermeria clinica,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
January 2014, Zhonghua er ke za zhi = Chinese journal of pediatrics,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
October 1997, Revista espanola de anestesiologia y reanimacion,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
April 2018, Journal francais d'ophtalmologie,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
January 1999, La Pediatria medica e chirurgica : Medical and surgical pediatrics,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
May 1982, Ugeskrift for laeger,
S Y Hsiao, and T Fukao, and M Koro, and Y Funakoshi, and T Hieda
June 2006, Nihon rinsho. Japanese journal of clinical medicine,
Copied contents to your clipboard!