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(Chest. 1974;65:628-631.)
© 1974 American College of Chest Physicians

The Cardiopathy of Mulibrey Nanism, a New Inherited Syndrome

Leena Tuuteri M.D.1; Jaakko Perheentupa M.D.2; and Juhani Rapola M.D.3

1 Docent in Pediatric Cardiology, University of Helsinki, and Cardiologist, University Children's Hospital
2 Associate Professor in Pediatrics, University of Helsinki
3 Docent in Pathology, University of Helsinki, and Chief of Pathology Section, University Children's Hospital

We report 24 cases of a new, probably autosomal recessive syndrome, mulibrey nanism. All patients had severe growth retardation and characteristic changes in tissues of mesodermal origin. Cardiovascular symptoms suggestive of pericardial constriction were prognostically most important. Two patients died with signs of cardiac failure. In both, postmortem examination showed a thickened and adherent pericardium as well as pathologic changes in the myocardium. The condition of four severely affected patients improved after pericardiectomy. The clinical and hemodynamic findings suggested similar affection of the pericardium in the other cases.




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N Karlberg, H Jalanko, J Perheentupa, and M Lipsanen-Nyman
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Copyright © 1974 by the American College of Chest Physicians.