Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management
Prader-Willi syndrome is a rare congenital disorder characterized by obesity, hypotonia, hypogonadism and developmental delay. The genetic disorder is caused by microdeletion on chromosome 15 on the paternal chromosome. The syndrome is a 2-stage disorder with hypotonic early infantile phase and...
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| Format: | Article |
| Language: | English |
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JCDR Research and Publications Pvt. Ltd.
2014-04-01
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| Series: | National Journal of Laboratory Medicine |
| Subjects: | |
| Online Access: | http://www.njlm.net/articles/PDF/2007/4-%206780_F(H)_PF1(PAG)_PFA(AK)_PF2(AGAK).pdf |
| _version_ | 1818514898386157568 |
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| author | Yatish Bevinaguddaiah. Shivakumar Shivanna Tejesh C Anandaswamy Vinayak Pujari S |
| author_facet | Yatish Bevinaguddaiah. Shivakumar Shivanna Tejesh C Anandaswamy Vinayak Pujari S |
| author_sort | Yatish Bevinaguddaiah. |
| collection | DOAJ |
| description | Prader-Willi syndrome is a rare congenital disorder
characterized by obesity, hypotonia, hypogonadism and
developmental delay. The genetic disorder is caused
by microdeletion on chromosome 15 on the paternal
chromosome. The syndrome is a 2-stage disorder with
hypotonic early infantile phase and an obese childhood
phase. These individuals have abnormal physiologic
response to hypoxia and hypercapnia, narrow oropharyngeal
space, thick secretions and exaggerated response to
sedatives posing increased anaesthetic risk. We describe
the anaesthetic management of a patient with PraderWilli syndrome where in laparoscopic orchidopexy was
performed successfully under general anaesthesia. |
| first_indexed | 2024-12-11T00:21:59Z |
| format | Article |
| id | doaj.art-d802883e0816438b9eb5eb98d88ae2c1 |
| institution | Directory Open Access Journal |
| issn | 2277-8551 2455-6882 |
| language | English |
| last_indexed | 2024-12-11T00:21:59Z |
| publishDate | 2014-04-01 |
| publisher | JCDR Research and Publications Pvt. Ltd. |
| record_format | Article |
| series | National Journal of Laboratory Medicine |
| spelling | doaj.art-d802883e0816438b9eb5eb98d88ae2c12022-12-22T01:27:42ZengJCDR Research and Publications Pvt. Ltd.National Journal of Laboratory Medicine2277-85512455-68822014-04-0132131510.7860/NJLM/2014/6780:2007Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and ManagementYatish Bevinaguddaiah.0Shivakumar Shivanna1Tejesh C Anandaswamy2Vinayak Pujari S3Assistant Professor, Department of Anaesthesiology, M S Ramaiah Medical College and Hospitals, Bangalore, India.Professor, Department of Anaesthesiology, M S Ramaiah Medical College and Hospitals, Bangalore, India.Associate Professor, Department of Anaesthesiology, M S Ramaiah Medical College and Hospitals, Bangalore, India.Associate Professor, Department of Anaesthesiology, M S Ramaiah Medical College and Hospitals, Bangalore, India.Prader-Willi syndrome is a rare congenital disorder characterized by obesity, hypotonia, hypogonadism and developmental delay. The genetic disorder is caused by microdeletion on chromosome 15 on the paternal chromosome. The syndrome is a 2-stage disorder with hypotonic early infantile phase and an obese childhood phase. These individuals have abnormal physiologic response to hypoxia and hypercapnia, narrow oropharyngeal space, thick secretions and exaggerated response to sedatives posing increased anaesthetic risk. We describe the anaesthetic management of a patient with PraderWilli syndrome where in laparoscopic orchidopexy was performed successfully under general anaesthesia.http://www.njlm.net/articles/PDF/2007/4-%206780_F(H)_PF1(PAG)_PFA(AK)_PF2(AGAK).pdfhypogonadismlaparoscopic orchidopexy prader-willi syndromeoculocutaneous albinism |
| spellingShingle | Yatish Bevinaguddaiah. Shivakumar Shivanna Tejesh C Anandaswamy Vinayak Pujari S Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management National Journal of Laboratory Medicine hypogonadism laparoscopic orchidopexy prader-willi syndrome oculocutaneous albinism |
| title | Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management |
| title_full | Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management |
| title_fullStr | Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management |
| title_full_unstemmed | Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management |
| title_short | Prader-Willi syndrome with Oculocutaneous Albinism: Anaesthetic Implications and Management |
| title_sort | prader willi syndrome with oculocutaneous albinism anaesthetic implications and management |
| topic | hypogonadism laparoscopic orchidopexy prader-willi syndrome oculocutaneous albinism |
| url | http://www.njlm.net/articles/PDF/2007/4-%206780_F(H)_PF1(PAG)_PFA(AK)_PF2(AGAK).pdf |
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