[Arteriopathia calcificans infantum].

A report is given on all 11-weeek-old male child who, after normal development, suddenly became critically ill and died within 8 hours. The autopsy revealed a rare aetiologically and in its pathogenesis unclarified, always lethal clinical picture of so-called "Arteriopathia calcificans infantum" (ACI). An analysis of 90 published cases revealed an average age of the diseased children of 150.9 days and a mean duration of the disease of 32.8 days; in the large majority of the cases (64.7%), death occurred at the latest within 3 days of the first symptoms. A statistical familial concentration of the disease could neither be shown nor excluded. No indication of an exogenous cause of the disease could be found in the reports on previous parental disease, in the history of the pregnancy, in the placenta findings, the case history of the children themselves or in the clinical data. The pathological-anatomical substrate of ACI comprised a calcification, predominantly of the muscular arteries, and located at the boundary between the intima and the media, within the course of the internal elastic lamina. Depending upon the stage of the disease, we first see a fragmentation of the elastic fibres, calcific dust deposits, calcific plaques and the complete replacement of the media by concentric, in part coarsely distingrating, calcific rings. As a result of proliferation of the intima a marked constriction of the lumen occurs in the diseased arteries. Most frequently involved at 90% are the coronary arteries. The genesis of this disease has a considerable similarity to experimental calciphylactic arteriopathy, but no sensitizer mechanism can be demonstrated for ACI. Morphological similarities also exist to generalised elastorrhexis, which is also very rare. There is probably a causal relationship between the two diseases. In a hypothesis on the aetiology of the ACI, it is suggested that this disease may be triggered by a congenital enzyme defect localised in the myointimal cells of the vascular wall. These cells produce incorrectly structured mucopolysaccharides, collagen and elastic material with pathologically increased calcipexy.