| Autor: |
Phillip G. Holtzapple, H. Lawrence Vallet, William C. Yakovac, Thomas Moshang, Alfred M. Bongiovanni, Walter R. Eberlein |
| Rok vydání: |
1972 |
| Předmět: |
|
| Zdroj: |
The Journal of Pediatrics. 80:269-274 |
| ISSN: |
0022-3476 |
| DOI: |
10.1016/s0022-3476(72)80589-4 |
| Popis: |
A child with Noonan syndrome presented with failure to thrive, edema of the extremities, and a seemingly mild cardiac lesion at age four months. The edema progressed with the development of anasarca at six years, accompanied by severe hypoproteinemia. Investigation revealed a protein-losing enteropathy (intestinal lymphangiectasis) which responded to medium-chain triglycerides and a low-fat diet. The improvement was transient, and a compartmental shift of fluids further stressed an already compromised myocardium. The pulmonary valvular stenosis was relieved surgically, but the patient died postoperatively. It is suggested that this intestinal abnormality may be another manifestation of a generalized defect in cardiovascular-lymphatic organogenesis in this syndrome. Perhaps the cardiovascular lesion should be corrected before attempting to relieve the malabsorption. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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