1/9. 'Catastrophic' antiphospholipid syndrome.When 'catastrophic' is applied as an adjective to the antiphospholipid syndrome, it implies a characteristic presentation due to predominantly small blood vessel thrombosis leading to rapidly progressive failure of multiple organs and a frequently fatal outcome. We present the case of a 48-year-old woman who presented with the 'catastrophic' antiphospholipid syndrome without previous history of coagulation disorder or connective tissue disease that illustrates the difficulties in diagnosing and managing this disorder. We also review the factors that have been reported to have a role in the development of this condition and show how this case throws light on its pathogenesis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Choroidal vascular occlusion in a child with a connective tissue disease and complement c4 deficiency.OBJECTIVE: To report the histopathologic findings in the eyes of a patient with a connective tissue disease and complement deficiency. DESIGN: Human postmortem ocular histopathologic study. INTERVENTION: A 15-year-old female died from complications of a connective tissue disease of uncertain etiology, particularly acute respiratory distress syndrome. Abnormalities seen in the eyes at autopsy were consistent with complement activation, granulocyte aggregation, and leukocyte embolization. MAIN OUTCOME MEASURES: Both eyes were examined by light microscopy. RESULTS: Some choroidal vessels were occluded by platelet-fibrin thrombi and occasionally by aggregates of granulocytes and fibrin. Serous retinal detachment involving the macula and peripheral retina was present in both eyes. CONCLUSIONS: This is a report of the ocular histopathologic findings in a patient with connective tissue disease and complement c4 deficiency. The light microscopy findings were consistent with complement activation with granulocyte aggregation and leukocyte embolization and may represent another mechanism to explain the clinical findings in patients with connective tissue disease, particularly systemic lupus erythematosus.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/9. drainage of subcutaneous lymphatic fluid for the management of respiratory distress in a case of generalized lymphangiectasia in an infant.A 10-month-old girl was referred to our hospital because of congenital and persistent bilateral chylothorax and generalized lymphedema as well as long-standing respiratory disturbance. Radiological studies showed a diffuse network of superficial lymphatic vessels without major trunks throughout her entire body as well as the lung. She was diagnosed with systemic lymphangiomatosis complicated with pulmonary lymphangiectasia. Percutaneous puncture in the lower leg was performed to discharge the lymphatic fluid and proved to be effective for the respiratory disturbance. This procedure is safe and easy and effectively improves the quality of life of the patient and the family in case of such a persistent disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/9. Pulmonary vascular lesions in the adult respiratory distress syndrome caused by inhalation of zinc chloride smoke: a morphometric study.Two soldiers were fatally injured by accidental inhalation of zinc chloride (ZnCl2) from a smoke bomb. Although exposed to a relatively short but high smoke concentration, acute injury was minor and for 10 days the patients were clinically satisfactory. Unexpectedly, both then rapidly developed features typical of severe adult respiratory distress syndrome with pulmonary hypertension. intubation and mechanical ventilation were instigated on day 15 (patient no. 1) and day 12 (patient no. 2) after the inhalation, but death followed at days 25 and 32, respectively. lung vascular injury was assessed by angiography and morphometric techniques. The lungs showed extensive interstitial and intra-alveolar space fibrosis. Vessels showed a significant lumen reduction by contracture (that is, reduction in vessel external diameter) affecting preacinar and intraacinar arterial and venous segments, the extent of injury suggesting that hexite causes more severe venous injury than seen in other types of adult respiratory distress syndrome. In microvessels there was obliteration and widespread occlusion by endothelial cell proliferation and clot. No evidence of infection was identified during life or at autopsy. It is unclear whether the long lag time was due to the fact that the infection was not a complicating event or because steroids, administered prophylactically, had sufficed to delay, but not to prevent, the amplification of injury that seems responsible for the adult respiratory distress syndrome.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
5/9. Ischemic necrosis of both lower extremities as a result of the microembolism syndrome complicating the adult respiratory distress syndrome caused by escherichia coli pneumonia and septicemia.Extensive gangrene of both lower extremities necessitating bilateral above-the-knee amputations complicated the adult respiratory distress syndrome (ARDS) caused by escherichia coli pneumonia and septicemia in a 52-yr-old man. Concurrent with the evolution of tissue necrosis, peripheral blood leukocyte and platelet counts fell, and pulmonary vascular resistance increased. Adequacy of the cardiac output was confirmed by repeated thermodilution cardiac output measurements, and major vascular occlusion was excluded surgically. fibrin degradation products and thrombocytopenia were present, but the other usual criteria for disseminated intravascular coagulation were absent. Small vessel thrombosis by fibrin and leukocytes was observed histologically in the amputated extremities. These findings suggest that gangrene was due to the "microembolism syndrome"--diffuse small vessel occlusion by fibrin thrombi complicating ARDS. This unusual complication of ARDS may occur without abnormalities suggestive of diffuse intravascular coagulation in routine laboratory tests of blood coagulation. It should be suspected and treated promptly to avoid severe disability in survivors.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
6/9. Tissue damage caused by activated complement and granulocytes in shock lung, post perfusion lung, and after amniotic fluid embolism: ramifications for therapy.The complement system evolved as a beneficial antimicrobial system. However when activated during extracorporeal perfusion, as with hemodialysis or cardiopulmonary bypass, modest pulmonary dysfunction associated with granulocyte aggregation and embolization can occur. When complement activation is more massive and prolonged as with severe sepsis, trauma and acute pancreatitis or during infusions of amniotic fluid or other lipid-rich suspensions, severe pulmonary damage which we often recognize as shock lung may occur. Therapeutic ramifications of these conclusions are evident. Thus, high doses of corticosteroids (or of non-steroidal anti-inflammatory agents, such as ibuprofen--herein not discussed) have the ability to prevent aggregation and embolization of stimulated granulocytes to patent vessels downstream and also inhibit their production of toxic oxygen radicals. These beneficial properties suggest the use of these agents may be appropriate in shock states, particularly shock lung or during suspected amniotic fluid infusion. Appropriate clinical trials to substantiate this suggestion are awaited with interest.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/9. Fatal adult respiratory distress syndrome in a scalded child after immunization with attenuated virus (measles, mumps and rubella).A fatal adult respiratory distress syndrome (ARDS) occurred in a 15-month-old child who had suffered minor scalding during the febrile response to combined attenuated virus immunization (measles, mumps and rubella [MMR]). Despite vigorous efforts the child died 26 days after the accident. It is suggested that the scalding suppressed the normal immune response to the viremia and that the latter (i.e. most likely the measles viremia) caused the lung damage which, in turn, led to the ARDS. Histologically the lung presented a peculiar change with fibroblastic nodules, vessel wall inflammation and signs as observed in ARDS.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/9. adult respiratory distress syndrome associated with antiphospholipid antibody syndrome.Antiphospholipid antibody syndrome (APS) has been shown to be associated with arterial and venous thromboses, recurrent miscarriages, and thrombocytopenia. We describe 3 cases of adult respiratory distress syndrome (ARDS) in patients with primary APS. The autopsy in one patient revealed no evidence of vasculitis, but extensive small vessel thrombosis of multiple organs including the lungs was seen. No infectious process, connective tissue diseases, or pulmonary edema could be documented and there was no clinical or laboratory evidence of vasculitis in the other 2 patients. The 2 patients who survived responded dramatically to intravenous steroids.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Intravascular lymphomatosis presenting as adult respiratory distress syndrome.An unusual case of intravascular lymphomatosis caused by small noncleaved, non-Burkitt's lymphoma, which presented with adult respiratory distress syndrome, is described. Extensive invasion of the small- and medium-size blood vessels of the lung, liver, spleen, kidneys, heart, esophagus, stomach, small and large intestines, bladder, and brain-but not the bone marrow or peripheral blood-is documented. The possible mechanism and the unusual features of this case are discussed in comparison with previously reported cases. The pertinent literature is reviewed. The problem of diagnosing this pathological entity is emphasized.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |