Kaitila et al. (1978, 1981) described 8 patients (7 boys, 1 girl) from 5 families with early-onset medial calcific arteriosclerosis with hypertension, short stature, delayed puberty, anemia, Perthes-like dysplasia of the hip, short fingers, dysplasia of second lumbar ... Kaitila et al. (1978, 1981) described 8 patients (7 boys, 1 girl) from 5 families with early-onset medial calcific arteriosclerosis with hypertension, short stature, delayed puberty, anemia, Perthes-like dysplasia of the hip, short fingers, dysplasia of second lumbar vertebra ('beaked L2'), and stiff skin. Extensive calcification of the aorta and peripheral arteries was demonstrated radiographically. Perforated gastric ulcer was a frequent cause of death. In the kidney, the glomerular tufts were shrunken and Bowman's capsules greatly dilated, giving a cystic appearance. Kaitila et al. (1981) attributed the mild anemia to mechanical hemolysis. The parents were first cousins in 1 instance. All came from the same sparsely populated area of Finland. Raitta and Kaitila (1985) described the ophthalmologic findings in 5 boys and 1 girl between 7 and 17 years of age: retinal vascular tortuosity of main vessels, precapillary arterioles, and microaneurysms of arterioles of the central fundus. Serum cholesterol, triglycerides, and lipoprotein electrophoresis were normal. Arterial biopsies showed remarkable hypertrophy of the media with normal intima. Gradually progressive renal failure and hypertension were typical. Myocardial infarction occurred at the age of 9 in one. The cerebral arteries were spared. The patients consistently showed systolic hypertension with a wide pulse pressure.