Clinical manifestation

Skin loose, inelastic, hanging in folds, and demonstrating decreased elastic recoil on stretching; patient looks much older than chronologic age

Internal organ involvement: gastrointestinal tract: diverticula of small and large bowel; rectal prolapse; umbilical, inguinal, and hiatal hernias

Pulmonary: bronchiectasis, emphysema, cor pulmonale

Cardiovascular: cardiomegaly; congestive heart failure; murmurs; aortic aneurysms Skeletal: dislocation of hips; osteoporosis; growth retardation; delayed fontanelle closure; ligamentous laxity

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