1992
DOI: 10.1001/archpedi.1992.02160240063021
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Spontaneous Chylothorax in Noonan Syndrome

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Cited by 32 publications
(15 citation statements)
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“…Associations with congenital heart disease, chromosomal disorders (eg, Down, Turner or Noonan syndrome7), prenatal infections, birth trauma, thrombosis of the superior vena cava or other malformations (eg, primary congenital pulmonary lymphangiectasis) have been reported 4 8–10. The male/female ratio is said to be 2:1 9.…”
Section: Introductionmentioning
confidence: 99%
“…Associations with congenital heart disease, chromosomal disorders (eg, Down, Turner or Noonan syndrome7), prenatal infections, birth trauma, thrombosis of the superior vena cava or other malformations (eg, primary congenital pulmonary lymphangiectasis) have been reported 4 8–10. The male/female ratio is said to be 2:1 9.…”
Section: Introductionmentioning
confidence: 99%
“…Secondary disorders present later in life and are associated with other congenital and genetic diseases such as Noonan and Down's syndrome. 3 The most common defect is pulmonary stenosis, which can be accompanied by atrial or ventricular septal defects, patent ductus arteriosus, and hypertrophic cardiomyopathy. It is an autosomal dominant disease with variable expression and it affects both men and women.…”
Section: Discussionmentioning
confidence: 99%
“…Such occurrences can occasionally be associated with pulmonary lymphangiectasia or appear postoperatively after cardiac surgery. Goens et al [1992] summarized the 11 reported cases of congenital (n ‫ס‬ 1), spontaneous (n ‫ס‬ 5), and post-operative (n ‫ס‬ 5) chylothorax and noted that all but two eventually resolved. Histologically, the distribution of dilated lymphatic channels can be localized, leading to isolated chylothorax, or diffuse, accounting for accompanying pulmonary lymphangiectasia.…”
Section: Discussionmentioning
confidence: 99%
“…Histologically, the distribution of dilated lymphatic channels can be localized, leading to isolated chylothorax, or diffuse, accounting for accompanying pulmonary lymphangiectasia. Steroid therapy has been reported on an anecdotal basis as being successful [Goens et al, 1992] if chemical or surgical pleurodesis and restriction of dietary triglyceride fails.…”
Section: Discussionmentioning
confidence: 99%