Ehlers‐Danlos syndrome type IV: A subset of patients distinguished by low serum levels of the amino‐terminal propeptide of type III procollagen

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Abstract
Serum levels of procollagen type III aminopropeptide (P‐III‐NP), a peptide released during conversion of type III procollagen to collagen, were abnormally low in six of 10 patients with Ehlers‐Danlos syndrome (EDS) type IV (arterial‐ecchymotic type) and low‐normal in 4. The serum P‐III‐NP levels correlated with the amount of type III procollagen secreted by the patients' cultured fibroblasts. Serum P‐III‐NP determination is a simple test that identifies a major subgroup of patients with this lifethreatening, dominantly inherited connective tissue disorder and may be especially helpful in making the diagnosis in children.