Cases reported "Catastrophic Illness"

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1/3. Catastrophic antiphospholipid syndrome: remission following leg amputation in 2 cases.

    OBJECTIVE: The antiphospholipid syndrome is characterized by venous and arterial thrombotic events that are often recurrent, thrombocytopenia, recurrent fetal loss, and elevated titers of antiphospholipid antibodies. A subtype of patients with a particularly overwhelming clinical picture has been termed catastrophic antiphospholipid syndrome (CAPS). In this report, we present 2 patients who exhibited a similar multisystem disorder associated with gangrenous changes in the lower extremities. methods: Two patients with CAPS are presented, highlighting the impact of this disorder on the patients and the response to various therapeutic modalities. RESULTS: Both patients had pulmonary, cardiac, cutaneous, and neurologic findings consistent with CAPS. In addition, they had large purulent leg ulcers associated with livedo reticularis. amputation of the legs in each case induced remission of the systemic illness. CONCLUSIONS: We believe that infection plays a significant role in the pathogenesis and amplification of the antiphospholipid syndrome. In certain patients, this association probably is mediated via immune mechanisms, which also enhance the genesis of atherosclerosis. After the foci of infection (suppurative leg ulcers) were removed, the underlying illness improved. These case studies provide an opportunity to study the interrelationship between several confounding factors that converge and lead to the development of this autoimmune condition.
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2/3. Catastrophic antiphospholipid antibody syndrome.

    Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.
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3/3. Is posttraumatic stress a viable model for understanding responses to childhood cancer?

    The application of a trauma model to understanding the impact of life-threatening illness has been informative. In the case of childhood cancer patients, it appears clear that a full PTSD syndrome is not the normative response either during or after treatment. Some aspects of cancer diagnosis and treatment, however, are experienced as traumatic by a subset of children, some of whom report symptoms of posttraumatic stress. There is some evidence that children may respond to cancer treatment as a repeated trauma, with the result of more subtle changes in affect modulation, world view, and interpersonal relationships. This area requires further investigation. The trauma model is also useful in understanding parental responses to childhood cancer. The epidemiologic data to date regarding posttraumatic stress symptoms in parents of childhood cancer survivors is consistent with the trauma literature regarding responses to moderate-magnitude traumatic exposure. These findings have important implications for clinical interventions for families of childhood cancer patients. More research is needed in the prediction and prevention of the long-term distress reported by so many parents of children who have undergone successful treatment for life-threatening illness.
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