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    |  Medical Surgical Nursing Review Questions Part 3   Posted: 23 Nov 2010 11:36 PM PST | 
  |  Medical Surgical Nursing Review Questions Part 2   Posted: 23 Nov 2010 11:29 PM PST | 
  |  Marfan’s Syndrome Signs and Symptoms   Posted: 23 Nov 2010 07:29 PM PST   
 
  Definition
  Is a connective tissue disorder.The chemical make-up of the connective tissue isn't normal, as a result many of these structures aren't as stiff as they should be.It is inherited and affect many parts of the body.The syndrome is so mild that few symptoms exist.The walls of the major arteries are weakened. The aorta, the major artery that leaves the heart, often is affected. When this occurs, it get bigger (dilates) which can weaken the inner aortic wall. Under strenuous exertion, the aorta can tear. Blood can leak through these tears into the aortic wall, separating its layers – a process called aortic dissection. Another problem that may occur if the aortic wall weakens is that an aneurysm may form. Etiology  Marfan syndrome is inherited as an autosomal dominant trait. Up to 30% of cases have no family history and are so called "sporadic" cases. Pathophysiology  Single mutant allele (from affected parent).Transmitted to the offspring.Abnormal proteins in genesAbnormal membrane bound transport systemAbnormal structural proteinsAbnormal cellsAbnormal tissuesAbnormal organ abnormal system Signs and symptoms  Long, lanky frameLong thin ribsArm span significantly greater than body heightLong, spidery fingers (arachnodactyly)Funnel chestScoliosisVisual difficultiesFlat feetLearning disabilityThin, narrow faceHypotoniaJoint laxityHighly arached palateCrowded teethDislocation of lensMalformed corneaRetinal detachment Assessment  Physical examinationEchocardiogramEye examinationX-rayDoppler echo testMRICT scanBiopsyCBCBlood chemistryUrine analysis Nursing Diagnoses  Risk for injuryRisk for infectionKnowledge deficitAnxietyActivity intoleranceDisturbance in self-esteemFearFatigueDisturbed body imageImpaired physical mobilityHopelessness Nursing Interventions  Observe safety precautions all the time, keep side rails up all the time.Observe cleanliness and sterility all the time to avoid possible contamination and spread of infection.Provide correct information about the disease process, complications and possible treatment.Encourage patient to acknowledge and to express feelings to be relieved from anxiety.Ascertain ability to stand and move about and degree of assistance necessary or use of equipment.Discover patient's inner abilities and help to improve them to boost up their self-esteem.Provide rest periods, avoid unnecessary disturbance.Improve other qualities and help the patient accept their problems and help them comfort them. Complications  Dilated aortic rootDissecting aortic aneurysmAortic ruptureBacterial endocarditisHeart failureMitral valve prolapsedFrequent palpationsHigh bloodPneumothoraxscoliosis Related posts: Bipolar Disorder Signs and SymptomsPharmacology Terms & Definitions, Clinical Conditions, and Associated Signs and SymptomsGlossary of Key Terms in Pharmacology, Clinical Conditions, and Associated Signs and Symptoms
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