Entry - 208300 - ASCITES, CHYLOUS - OMIM

 
ICD+
208300

ASCITES, CHYLOUS


Clinical Synopsis
 

Abdomen
- Chylous ascites, congenital
Limbs
- Arm swelling
Eye
- Bilateral glaucoma
Inheritance
- Autosomal recessive
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▼ TEXT

Lee and Young (1953) described chylous ascites in 2 sisters under 1 year of age. One also had swelling of one arm evident at 1 week and the entire body somewhat later and developed bilateral glaucoma in the first 6 months of life. Both this patient and the younger sister had spontaneous clearing of the manifestations. Chylous ascites and chylous pleural effusions probably occur at times with hereditary lymphedema, but this condition in its various forms is usually dominant. Flores et al. (1979) reported congenital chylous ascites in a brother and sister with first-cousin parents. Lymphangiography showed no abnormality. Intestinal biopsy showed mucosal edema but no lymphangiectasia.


▼ REFERENCES

  1. Flores, S., Leungas, J., Arredondo-Vega, F., Guizar-Vazquez, J. Chylous ascites in sibs from a consanguineous marriage. Am. J. Med. Genet. 3: 145-148, 1979. [PubMed: 474627, related citations] [Full Text]

  2. Lee, C.-H., Young, J. R. Chylous ascites in siblings. J. Pediat. 42: 83-86, 1953. [PubMed: 13011715, related citations] [Full Text]


Creation Date:
Victor A. McKusick : 6/3/1986
Edit History:
mimadm : 2/19/1994
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/26/1989
marie : 3/25/1988
reenie : 6/3/1986

208300

ASCITES, CHYLOUS


SNOMEDCT: 52985009;   ICD10CM: I89.8;   ORPHA: 1160;  



TEXT

Lee and Young (1953) described chylous ascites in 2 sisters under 1 year of age. One also had swelling of one arm evident at 1 week and the entire body somewhat later and developed bilateral glaucoma in the first 6 months of life. Both this patient and the younger sister had spontaneous clearing of the manifestations. Chylous ascites and chylous pleural effusions probably occur at times with hereditary lymphedema, but this condition in its various forms is usually dominant. Flores et al. (1979) reported congenital chylous ascites in a brother and sister with first-cousin parents. Lymphangiography showed no abnormality. Intestinal biopsy showed mucosal edema but no lymphangiectasia.


REFERENCES

  1. Flores, S., Leungas, J., Arredondo-Vega, F., Guizar-Vazquez, J. Chylous ascites in sibs from a consanguineous marriage. Am. J. Med. Genet. 3: 145-148, 1979. [PubMed: 474627] [Full Text: https://doi.org/10.1002/ajmg.1320030205]

  2. Lee, C.-H., Young, J. R. Chylous ascites in siblings. J. Pediat. 42: 83-86, 1953. [PubMed: 13011715] [Full Text: https://doi.org/10.1016/s0022-3476(53)80113-7]


Creation Date:
Victor A. McKusick : 6/3/1986

Edit History:
mimadm : 2/19/1994
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/26/1989
marie : 3/25/1988
reenie : 6/3/1986



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OMIM® and Online Mendelian Inheritance in Man® are registered trademarks of the Johns Hopkins University.
Copyright® 1966-2024 Johns Hopkins University.

NOTE: OMIM is intended for use primarily by physicians and other professionals concerned with genetic disorders, by genetics researchers, and by advanced students in science and medicine. While the OMIM database is open to the public, users seeking information about a personal medical or genetic condition are urged to consult with a qualified physician for diagnosis and for answers to personal questions.
OMIM® and Online Mendelian Inheritance in Man® are registered trademarks of the Johns Hopkins University.
Copyright® 1966-2024 Johns Hopkins University.
Printed: June 8, 2024