6533b7d2fe1ef96bd125ec4e

RESEARCH PRODUCT

Myopathic form of arthrogryposis and microcirculation lesion.

Uta Burck-lehmannHans H. GoebelAnna Fidziańska

subject

ArthrogryposisArthrogryposisMalePathologymedicine.medical_specialtyVascular diseasebusiness.industryMusclesInfantAnatomyBlood Coagulation Disordersmedicine.diseaseThrombosisPathophysiologyMicrocirculationLesionNeurologyMuscular DiseasesmedicineHumansPlateletNeurology (clinical)medicine.symptombusinessNeutrophil aggregation

description

A microvascular lesion characterized by extensive platelet aggregation, thrombosis, vascular damage with hemorrhages was found in the muscle of a 2-month-old boy with a myopathic form of the arthrogryposis syndrome. The lesion morphologically resembled the vascular leakage seen in immunologically mediated tissue injury. A degradative effect of proteases released during platelet and neutrophil aggregation on the muscle and joints is suggested.

10.1016/0022-510x(89)90148-2https://pubmed.ncbi.nlm.nih.gov/2809625