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Definitions and summaries of various non-inflammatory connective tissue diseases, including marfan syndrome, ehlers-danlos syndrome, and scurvy. These conditions can be caused by genetic disorders or nutritional issues and result in impaired collagen synthesis or stability, leading to weak connective tissue and specific manifestations. Understanding these diseases is crucial for accurate diagnosis and effective treatment.
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caused by genetic disorders or nutrional issueslesion is due to a dysfunction in synthesizing either collagen fibrillin/elasticmanifests as impaired collagen results in unstable and weak collagen fiber bundles and result in CT weakness TERM 2
DEFINITION 2 genetic disorder of specific protein syntheses of collagen, elastic, synth by fibroblastsmarfan's syndrome-autosomal dom involves body fibroblastsehlersDanlos sundrome- autosomal dom, o TERM 3
DEFINITION 3 scurvyloss of or lack of vitamin c, collagen synthesizing cofactorw/o c collagen will not be formed effectively TERM 4
DEFINITION 4 impaired fibillin synthesis which decreases stabilitic of elastic fibers TERM 5
DEFINITION 5 affects synth of collagen resulting in weak tissue fiber bundles
vit c is necessary cofactor for the stabilization of collagen fibers after synth TERM 7
DEFINITION 7 non inflammatoryautosomal dominantimpaired fibillin synth which decreases stability of elastic fibersmanifests as weak blood vessel walls-aorta rupture, skeletal abnormalities dure to weak bone CT, joint hypermobilitycharacterized by long fingers TERM 8
DEFINITION 8 noninflammatory CT diseaseautosomal dominantaffects synthesis of collagen resulting in weak tissue fibermanifests as weak blood vessel walls/aorta rupture, joint hypermobilitiy-being double jointed, fragile skin susceptible to bruising and bleeding, poor wound healing usually utilizing secondary intention TERM 9
DEFINITION 9 noninflammdue to lack of vit cnecessary for stabilization of collagen fibers after synth-post translationalmanifests as fragile skin susceptible to bleeding and poor wound healing TERM 10
DEFINITION 10 mixed connective tissue disease
impairs the transfer of nutrients and impairs capillary permeability TERM 17
DEFINITION 17 prevalence highest in young to middle age womenskin rash butterfly over cheeks-wolfbite-->lupusarthritis from synovial inflammationgolmerulonephritis from vasculitis and /or BMpulmonary dysfunction from vasculitis and/or BMblood disorders-anemia, decreased leukocytes and platelets TERM 18
DEFINITION 18 specific to skin whereas lupus is widespreadcaused by an autoimmune reaction where lymphocytes respond to unknown antigen in skin CTresults in the release of inflamm cytokines TERM 19
DEFINITION 19 increased collagen production and depositsdermis layer is mostly affectedvasculitis is common TERM 20
DEFINITION 20 dermis is thick and restrictedskin ulcers and calcificationGI Kidney and respiratory systems are also affected