PubMed · 612833
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H Ishikawa. 1977. [CRST syndrome].. https://pubmed.ncbi.nlm.nih.gov/612833/
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In a 10-year-old boy with pseudohypoparathyroidism type Ia (PHP-Ia) echocardiography detected calcifications within the ventricular septum. This is thought to be the first report of intracardiac calcifications in PHP. Echocardiography should be done routinely in all patients with this rare disorder.
Renal ultrasound examination, performed following a urinary tract infection in a 4.5-year-old girl with triple X syndrome, showed multiple echogenic foci at the corticomedullary junction in both kidneys. She was asymptomatic but had hypertension with echocardiographic evidence of left ventricular hypertrophy. Computerised tomographic scan revealed foci of calcification in the kidneys, spleen and pancreas. On biopsy calcification was found in the internal and external elastic laminae of the superficial temporal artery and in the internal elastic lamina of a renal arcuate artery. Intimal fibrosis was mild and focal. No other arterial calcification was demonstrated radiographically or by ultrasound. Biochemical and hormonal profiles revealed no abnormality except hypercalciuria. The aetiology and prognosis of this child's condition are unknown. Although similar ultrasound and histological appearances have been described in pseudoxanthoma elasticum and in idiopathic arterial calcification of infancy, there is no evidence that the child has either of these conditions.
The early detection of coronary atherosclerosis may be impossible if we continue to depend on its pathophysiologic effects (ischemia) for our screening tests. Insoluble crystalline calcium phosphate, which is ubiquitous in our inorganic and biologic worlds, precipitates relatively early in atherosclerotic lesions. Since coronary calcification is specific for atherosclerosis and since calcium is a strong radiation absorber in the X-ray frequency range, sensitive radiographic techniques such as dual-energy subtraction fluoroscopy and ultrafast computed tomography hold promise as screening tests for this disease.