Cases reported "Critical Illness"

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1/3. Catapulting through life stages. When younger adults are diagnosed with life-threatening illnesses.

    knowledge of developmental stages through the life cycle has always been a hallmark of quality nursing care. The knowledge base gleaned from the older adult literature, such as Schachter-Shalomi and Miller's construct of sage-ing (1995), can help nurses understand that many of the completion tasks usually associated with aging suddenly are thrust to the forefront for younger adult patients diagnosed with a life-threatening illness. Using this knowledge base, nurses can facilitate developmental stage work facing younger adult patients whose illness catapults them into more mature stages for which they may have been unprepared. When younger adult patients are so diagnosed, nurses need to recognize the signs of catapulting life stage work and support it. It is no small task to complete the gestalt of one's life tapestry, but it is especially difficult when one is young.
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2/3. Unmasked adult-onset urea cycle disorders in the critical care setting.

    Most often, urea cycle disorders have been described as acute onset hyperammonemia in the newborn period; however, there is a growing awareness that urea cycle disorders can present at almost any age, frequently in the critical care setting. This article presents three cases of adult-onset hyperammonemia caused by inherited defects in nitrogen processing in the urea cycle, and reviews the diagnosis, management, and pathophysiology of adult-onset urea cycle disorders. Individuals who have milder molecular urea cycle defects can lead a relatively normal life until a severe environmental stress triggers a hyperammonemic crisis. Comorbid conditions such as physical trauma often delay the diagnosis of the urea cycle defect. Prompt recognition and treatment are essential in determining the outcome of these patients.
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3/3. plasma exchange for treatment of thrombotic thrombocytopenic purpura in critically ill patients.

    OBJECTIVE: Description of diagnostic procedures, treatment modalities and intensive care management of patients with thrombotic thrombocytopenic purpura (TTP). DESIGN: Descriptive study. SETTING: internal medicine intensive care Unit (University Hospital of Vienna). patients: Six patients (two after allogeneic bone marrow transplantation), treated for 12 episodes of TTP. INTERVENTIONS: Treatment with plasma exchange (fresh frozen plasma, 50-80 ml/kg per day), prednisone (0.75 mg/kg b.i.d.) and, in some cases, vincristine. Supportive therapy as needed. MEASUREMENTS AND RESULTS: patients were admitted to the ICU because of neurological symptoms with acute onset (42% mild, 58% severe), hemolysis and thrombocytopenia. Additional symptoms were fever (50%), bleeding tendency (50%), acute renal failure (42%) and metabolic derangement (8%). Initial laboratory values showed thrombocytopenia (median 17 G/l), hemolysis (median hemoglobin 10.0 g/dl, lactate dehydrogenase 635 U/l, reticulocyte count 175 G/l) with red cell fragmentation. Coagulation tests were normal. Respiratory assist was needed in six episodes (severe seizures, cardiopulmonary resuscitation). In patients without preexisting hematological abnormality the platelet counts exceeded 100 G/l after 3-8 cycles of plasma exchange. In patients after bone marrow transplantation, the platelet counts never exceeded 40 G/l, but the lactate dehydrogenase levels dropped significantly. The neurological symptoms disappeared in all patients and renal function normalized. One patient died before the initiation of therapy. Three patients relapsed 1-3 times between 2 weeks and 5 months after the last episode. The relapses were associated with symptoms similar to the first episode and responded promptly to plasma therapy. CONCLUSIONS: TTP is a rare, but life-threatening disorder. It needs immediate diagnosis and has a good prognosis after adequate treatment with plasma exchange.
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