Cases reported "Purpura"

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11/19. Electrolyte changes caused by hydrotherapy in purpura fulminans.

    purpura fulminans is a rare manifestation of meningococcemia that in its full-blown form has a predictive death value of 61%. Those patients who survive usually develop gangrenous lesions that involve skin and underlying structures, mostly of the extremities and sometimes of the cheeks. Experience with early excision and skin grafting of these lesions has generally been unsatisfactory since, due to the unique pathophysiology of the disease and involvement of the most distal branches of the cutaneous circulation, the lesions are not completely demarcated until well after complete recovery from the acute phase of the disease. Recently there was an outburst of purpura fulminans in Southern california and other parts of the country. During the months of January and February 1986 we were consulted on five cases. These ranged from two months to six years in age and consisted of two boys and three girls. One two-month-old died during the acute phase, another six-year-old remained in shock and in need of hemodynamic and respiratory support and succumbed three weeks after the onset of the disease, during which time all four extremities showed progressive necrosis. Of the three patients that survived, one three-year-old girl resolved her purpuric lesions except for small necrotic patches on the buttocks that did not require surgical intervention. The other two children were left with gangrenous lesions of the upper and lower extremities over 30% of total body surface area. One of these two patients demonstrated an electrolyte disturbance.(ABSTRACT TRUNCATED AT 250 WORDS)
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12/19. Post-transfusion purpura: a report of five patients and a review of the pathogenesis and management.

    We report five cases of post-transfusion purpura. Despite having profound thrombocytopenia, their management consisted only of steroid administration if serious bleeding was present. In one patient without bleeding, no specific therapy was given. This patient recovered uneventfully. Two patients died. One of these died from complicating illnesses rather than bleeding. The other suffered brain death, the result of a ruptured cerebral aneurysm. Four patients were PLA1 negative. The fifth patient had an antibody to an unidentified platelet-specific antigen.
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13/19. The role of splenectomy in the treatment of thrombocytopenic purpura due to systemic lupus erythematosus.

    splenectomy in the treatment of thrombocytopenic purpura associated with lupus erythematosus has afforded satisfactory overall results in a group of ten patients. Postoperative deaths occurred in two patients, each of whom had significant underlying problems. Clinical manifestations of SLE-induced thrombocytopenic purpura included ecchymoses, petechiae, menorrhagia, epistaxis, and hematuria. splenectomy in this disorder should be reserved for cases in whom corticosteroids do not produce satisfactory results, or in whom unacceptably high doses are required. Follow-up indicates long term control of thrombocytopenic purpura following splenectomy.
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14/19. Munchausen's syndrome by proxy (child abuse) presenting as apparent autoerythrocyte sensitization syndrome: an unusual presentation of Polle syndrome.

    Munchausen's syndrome was initially described in 1951. It has become increasingly recognized since that time. Recently, Munchausen's syndrome by proxy or Polle syndrome has been described. In this disease entity, a parent causes a factitious disease in a child. The disease may require extensive medical testing and the result may even be death. A wide variety of presentations of Polle syndrome have been reported including cardiac arrest, recurrent sepsis, and factitious bleeding. Autoerythrocyte sensitization, a syndrome of spontaneous purpura, was initially described in 1955. The diagnostic test described is intradermal injection of autologous blood which produces an area of painful purpura. The syndrome is therefore postulated to be due to hypersensitivity in the patient's own RBCs. The majority of patients with autoerythrocyte sensitization have had psychiatric disorders and the symptoms have improved only with psychotherapy. An infant who had a clinical picture of autoerythrocyte sensitization and who was later found to be a victim of Polle syndrome is reported.
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15/19. Fulminant rocky mountain spotted fever. Its pathologic characteristics associated with glucose-6-phosphate dehydrogenase deficiency.

    Three patients with documented fulminant rocky mountain spotted fever (RMSF) (death on or before day 5 of illness) had severe multisystemic injury as shown by clinical signs and laboratory data, but on microscopic examination showed minimal evidence of the typical mononuclear leukocytic response to rickettsial vascular infection and injury. thrombosis was more extensive than in classic RMSF, with fibrin thrombi located in foci of rickettsial infection. These patients had a rash either preterminally or not at all, particularly severe rickettsia-associated pulmonary lesions, and other shock-related lesions, eg, centrilobular hepatic necrosis. All three patients were male blacks with glucose-6-phosphate dehydrogenase deficiency, a condition recently associated with severity of RMSF. diagnosis of fulminant RMSF requires awareness of its pathologic and epidemiologic aspects, and use of rickettsial isolation or specific immunofluorescence.
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16/19. Cutaneous pathobiology mediated by chemotherapy.

    A histopathologic study of side effects of chemotherapy on normal skin was made by light microscopy and radioautography after incorporation of tritiated thymidine. Two psoriatic patients were studied after methotrexate treatment and one after polychemotherapy for an osteosarcoma 2--5 days after administration of methotrexate. Four biological effects can occur: cellular death, recruitment of cells from a resting phase into the cell cycle of proliferation, synchronization of cells in that cycle and blockage of cells in specific phases. Sub-lethal signs and necrosis were recognized in the epidermis as vacuolar alterations, cytoplasmic inclusions, swelling of keratinocytes, apoptosis and dyskeratosis. Bullae resulted from epidermal necrolysis. The labelling index of normal looking keratinocytes was very high. Within capillaries, plump and hyperchromatic endothelial cells were increased in number, as was their labelling index. fibroblasts and cells of the eccrine glands were also labelled in number. The number of labelled nuclei within these cell lines reflects the recruitment and synchronization in the cell cycle and the blockage in s phase.
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17/19. Pathological findings in hanging and wedging deaths in infants and young children.

    Records of the Adelaide Children's Hospital Histopathology Department were reviewed for cases of deaths resulting from hanging or wedging occurring in early childhood and infancy. The 14 cases identified were analyzed with respect to age, sex, circumstances of death, and postmortem findings. The mean age at death was 14 months (range, 6-36 months) and the male to female ratio was 9:5. In one case, death occurred in a baby car seat, another in a pram/stroller, and in another a curtain cord was responsible. In the remaining 11 cases, death occurred in the baby's crib. In eight cases the mechanism of death was hanging with partial suspension, including six cases in which part of the infant's clothing became caught on the crib. Petechial hemorrhages on the face were found in all of the hanging deaths whereas intrathoracic petechiae were identified in only two cases. Only one of the wedging deaths showed facial petechiae whereas intrathoracic petechiae were identified in four of the six cases. Conjunctival hemorrhages were only recorded in only two of the 14 cases.
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18/19. Thrombotic complications of BMT: association with protein c deficiency.

    A decrease in levels of circulating anticoagulant protein C has been shown to occur following autologous BMT, and this deficiency may contribute to a hypercoagulable state placing patients at risk for thromboembolic events. We report four patients who suffered a variety of thrombotic complications following BMT (non-bacterial thrombotic endocarditis, superior vena cava thrombosis, thrombotic stroke, purpura fulminans, small bowel infarction secondary to diffuse microvascular thrombosis), which were preceded by or temporally related to decreased levels of protein C. Treatment with fresh frozen plasma (FFP) led to slight, temporary increases in protein C levels but infusions of FFP did not prevent either death or extension of the thrombus in these four cases, suggesting the need for higher protein C doses and/or concomitant anticoagulation. Though no direct causal relationship between these thrombotic complications and the protein c deficiency can be proved, a generalized hypercoagulable state caused by protein c deficiency may have contributed to the development, severity or progression of these complications.
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19/19. Postmortem extravasation of blood potentially simulating antemortem bruising.

    A case of florid postmortem extravasation of blood, potentially simulating antemortem bruising, is presented. A 98-year-old woman died in hospital, the cause of death being certified as congestive cardiac failure. After burial, it was apparent that the grave had been disturbed by crowbars and shovels. exhumation was performed and autopsy revealed considerable apparent facial bruising as well as lacerations and fractures. There was no documentation by the medical or nursing staff of any injuries to the deceased preceding death. There was also no documentation of injury by the funeral directors. Subsequently, two men admitted to removing the body from the grave and mutilating it. Thus, what was apparently facial bruising was, in fact, postmortem extravasation of blood simulating antemortem bruising. The degree of extravasation was considered to be related to the severity of the injuries, loose subcutaneous tissues of the head and neck, and dependent position of the body upon return to the grave. This case demonstrates the degree of postmortem extravasation of blood that may occur in particular circumstances and may simulate antemortem bruising. In other circumstances, the postmortem extravasation of blood may well have led investigators to pursue inquiries regarding homicide.
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