1/36. indocyanine green angiography and pathophysiology of multifocal posterior pigment epitheliopathy.PURPOSE: To clarify the pathophysiology of multifocal posterior pigment epitheliopathy (MPPE), or bullous retinal detachment (RD)-an unusual manifestation of central serous chorioretinopathy (CSC)-we evaluated indocyanine green (ICG) angiographic findings of patients with MPPE. methods: indocyanine green angiography was performed on 45 eyes of 26 patients with MPPE in our clinic during a 4-year period and compared with clinical and fluorescein angiographic (FA) findings. RESULTS: Ophthalmoscopically, in the posterior pole there were multiple yellowish-white retinal exudations, associated with flat, serous RD and bullous RD in the lower periphery. fluorescein angiography demonstrated multiple massive leakages from the choroid into the subretinal space. These leakage sites corresponded to the retinal exudations. indocyanine green angiography showed hyperfluorescence in the posterior pole of the choroid. The hyperfluorescence was first seen in the middle phase and became prominent in the late phase. This finding seems to be due to extravasation from the choriocapillaris. After laser photocoagulation of the leakage sites seen on FA, the leakages stopped and the retinal exudations and RD were resolved. indocyanine green angiography, however, revealed hyperfluorescence in the posterior pole that was seen in active stage. DISCUSSION AND CONCLUSION: These ICG angiographic findings for MPPE show that hyperpermeability of the choroidal vessels may be the primary causative lesion. This is followed by an intrastromal accumulation of the extravasated choroidal fluid, which may be subclinical. Involvement of the retinal pigment epithelium may be secondary, and then the disease becomes manifest with RD. In MPPE, a severe form of CSC, the retinal pigment epithelium is involved extensively and widely, and prognosis is unfavorable. We conclude that MPPE and CSC represent opposite ends of a common morbid spectrum.- - - - - - - - - - ranking = 1keywords = leak (Clic here for more details about this article) |
2/36. Systemic toxicity following administration of sirolimus (formerly rapamycin) for psoriasis: association of capillary leak syndrome with apoptosis of lesional lymphocytes.BACKGROUND: sirolimus (formerly rapamycin) is an immunosuppressive agent that interferes with T-cell activation. After 2 individuals with psoriasis developed a capillary leak syndrome following treatment with oral sirolimus lesional skin cells and activated peripheral blood cells were analyzed for induction of apoptosis. OBSERVATIONS: A keratome skin specimen from 1 patient with sirolimus-induced capillary leak syndrome had a 2.3-fold increase in percentage of apoptotic cells (to 48%) compared with an unaffected sirolimus-treated patient with psoriasis (21%). Activated peripheral blood T cells from patients with psoriasis tended to exhibit greater spontaneous or dexamethasone-induced apoptosis than did normal T cells, particularly in the presence of sirolimus. CONCLUSIONS: Severe adverse effects of sirolimus include fever, anemia, and capillary leak syndrome. These symptoms may be the result of drug-induced apoptosis of lesional leukocytes, especially activated T lymphocytes, and possibly release of inflammatory mediators. Because patients with severe psoriasis may develop capillary leak from various systemic therapies, clinical monitoring is advisable for patients with inflammatory diseases who are treated with immune modulators.- - - - - - - - - - ranking = 19452.576311583keywords = capillary leak syndrome, capillary leak, leak syndrome, capillary, leak (Clic here for more details about this article) |
3/36. Treatment of the systemic capillary leak syndrome with terbutaline and theophylline. A case series.BACKGROUND: The systemic capillary leak syndrome is a rare idiopathic disorder characterized by recurrent episodes of hypotension and hemoconcentration due to sudden transient extravasation of 10% to 70% of plasma. mortality rates 5 years after diagnosis have been reported to be 76%. OBJECTIVE: To assess the efficacy of a prophylactic regimen for the systemic capillary leak syndrome. DESIGN: Case series. SETTING: Tertiary referral center. patients: Eight patients followed over the past 18 years. INTERVENTION: Oral terbutaline plus aminophylline or theophylline. MEASUREMENTS: Long-term clinical follow-up. RESULTS: During a median follow-up of 9 years (range, 2 to 18 years), two patients (25%) died: one during an acute episode and one of complications related to long-term corticosteroid therapy. The other six patients are alive and healthy. The frequency and severity of the episodes decreased by a median of 30-fold. Recurrences were associated with decreased serum theophylline levels, possibly caused by enzyme induction or autoinduction. The extended-release form of medication was more successful. Sympathomimetic side effects were significant. CONCLUSIONS: A regimen of terbutaline and theophylline seems to be effective prophylaxis against the systemic capillary leak syndrome. maintenance of therapeutic drug levels was associated with favorable results.- - - - - - - - - - ranking = 20778.874063761keywords = capillary leak syndrome, capillary leak, leak syndrome, systemic capillary leak syndrome, systemic capillary leak, systemic capillary, capillary, leak (Clic here for more details about this article) |
4/36. Lethal capillary leak syndrome after a single administration of interferon beta-1b.Interferon beta-1b (IFNbeta-1b) is a potent immunomodulatory drug in the treatment of MS. We report a lethal capillary leak syndrome after the administration of IFNbeta-1b in a patient with disseminated white matter disease, monoclonal gammopathy, and acquired C1 inhibitor (C1-INH) deficiency. IFNbeta-1b may cause a transient release of proinflammatory cytokines finally resulting in an uninhibited activation of the complement cascade in patients with C1-INH deficiency.- - - - - - - - - - ranking = 13519.92588098keywords = capillary leak syndrome, capillary leak, leak syndrome, capillary, leak (Clic here for more details about this article) |
5/36. Pulmonary infiltrates after cytokine therapy for stem cell transplantation. Massive deposition of eosinophil major basic protein detected by immunohistochemistry.interleukin-2 (IL-2), a product of activated T-cells, is now being used in a number of protocols for cancer immunotherapy. In one stem cell transplantation protocol for breast cancer, IL-2 is used together with interferon-gamma (IFN-gamma) and cyclosporine to stimulate a graft-versus-tumor response and improve the likelihood of a prolonged remission. We present the case of a patient who developed peripheral eosinophilia, perihilar infiltrates, and hypoxemia after autologous stem cell transplantation and the use of recombinant IL-2 and IFN-gamma. Histologic analysis of transbronchial lung biopsies demonstrated a few eosinophils within the bronchial submucosa. Immunostaining using antibodies directed against eosinophil major basic protein (MBP), however, revealed massive extracellular deposition of this toxic granule protein throughout the lung parenchyma. IL-2 therapy is well known to induce a peripheral eosinophilia and to be associated with the capillary leak syndrome characterized by weight gain, edema, and oliguria. The findings noted in this case report suggest that the eosinophil activation that accompanies immunologic therapy with IL-2 can result in direct toxicity to the lung and a localized vascular leak syndrome. This syndrome should be considered in the differential diagnosis of pulmonary infiltrates that occur acutely after bone marrow transplantation with cytokine augmentation.- - - - - - - - - - ranking = 3164.369531371keywords = capillary leak syndrome, capillary leak, leak syndrome, capillary, leak (Clic here for more details about this article) |
6/36. Adjuvant treatment of severe acute pancreatitis with C1 esterase inhibitor concentrate after haematopoietic stem cell transplantation.BACKGROUND: With an incidence of 4%, acute pancreatitis is a common complication of bone marrow or peripheral haematopoietic stem cell transplantation, which contributes significantly to morbidity and mortality in these patients. In most cases, the pathogenesis of acute pancreatitis cannot be attributed to a single pathogenetic factor, as treatment toxicity, acute graft versus host disease, infection, and cholestasis may all contribute. Acute pancreatitis is characterised by inflammation and activation of digestive proenzymes leading to autodigestive destruction of the pancreas and systemic activation of protease cascades including the complement system. AIM: To describe the effects of human C1 esterase inhibitor in two children, who developed severe acute pancreatitis with considerable complement activation after allogeneic haematopoietic stem cell transplantation. methods: Both children showed clinical features resembling those observed in capillary leakage syndrome. In both patients, treatment with C1 esterase inhibitor concentrate contributed to a rapid clinical stabilisation. CONCLUSIONS: These observations strongly support the proposed pathophysiological concept that early treatment with C1 esterase inhibitor interferes with the activation of the complement system in acute pancreatitis. Inhibition of complement activation prevents its adverse effects on vascular function and permeability, and thus stabilises intravascular fluid status and prevents multiorgan failure in acute pancreatitis.- - - - - - - - - - ranking = 524.68007821057keywords = capillary leak, capillary, leak (Clic here for more details about this article) |
7/36. Randomized trial of hydroxyethyl starch versus gelatine for trauma resuscitation.BACKGROUND: Previous studies have demonstrated the rapid increase in systemic capillary permeability after blunt trauma and its association with poor outcome. There are theoretical advantages in resuscitation with colloid fluids, which are well retained in the vascular compartment during times of capillary leak. The aim of this study was to compare the effects of posttrauma resuscitation with hydroxyethyl starch (HES) (molecular mass, 250 kDa) or gelatine (molecular mass, 30 kDa), the hypothesis being that HES would reduce capillary leak. methods: Forty-five patients suffering blunt trauma were randomized on admission to receive either gelatine (Gelofusine) (n = 21) or HES (Pentaspan) (n = 24) for the first 24 hours, after which the choice of fluid was at the discretion of the clinician. The mean injury severity score for the HES and gelatine groups were 20.0 (range, 9-41) and 18.1 (range, 9-32), respectively (p = 0.43). capillary permeability was assessed by urine albumin excretion rate for the first 24 hours. For 5 days the daily mean P(O2)/F(IO2) ratio, serum c-reactive protein, hemoglobin, white cell and platelet counts, prothrombin, and activated partial thromboplastin time were recorded. RESULTS: capillary permeability was lower in HES-treated patients during the first 24 hours. Log mean (95% confidence interval) albumin excretion rate for gelatine and HES groups at 6 hours were 117.5 (84.9) and 46.8 (24.3) microg/min (p = 0.011), at 12 hours were 54.9 (30.0) and 17.2 (7.6) microg/min (p = 0.001), and at 24 hours were 50.5 (23.4) and 23.6 (16.3) microg/min (p = 0.030), respectively. The mean (95% confidence interval) P(O2)/F(IO2) ratio for the HES and gelatine groups 48 hours after admission were 324 (44) and 267 (43) mm Hg, respectively (p = 0.03). The mean (95% confidence interval) serum c-reactive protein in the HES and gelatine groups 24 hours after admission were 72.4 (19.2) and 105.7 (30.1) mg/L, respectively (p = 0.03). There were no significant differences in any of the hematologic parameters during the first 48 hours. CONCLUSION: The results suggest that compared with gelatine, resuscitation with HES reduces posttrauma capillary leak.- - - - - - - - - - ranking = 1612.7254942978keywords = capillary leak, systemic capillary, capillary, leak (Clic here for more details about this article) |
8/36. C1-esterase inhibitor in graft failure after lung transplantation.Graft failure after lung transplantation may occur immediately after transplantation due to reperfusion injury or later due to rejection and infection. Although the pathological mechanisms are not completely known, the clinical findings are similar to the adult respiratory distress syndrome. In this condition, the blood coagulation contact system and the complement system are activated, leading to a capillary leak syndrome. Activation of the contact as well as the complement system is regulated by a common inhibitor, C1-esterase inhibitor (C1-INH). We report on two patients who received high doses of C1-INH for 2 days during graft failure either due to reperfusion injury immediately after transplantation or due to an acute rejection 2 months after double-lung transplantation. In both cases of graft failure, a capillary leak syndrome occurred with pleural effusions of 7 l to more than 10 l per day. In case 1 disturbance of gas exchange during severe reperfusion injury could not be treated effectively with other treatment modalities like nitric oxide ventilation or surfactant administration. With the use of C1-INH, pleural effusions reduced within 12 h, leading to normal graft function within 4 days. In the second recipient, acute rejection forced the use of extracorporeal membrane oxygenation (ECMO) within 24 h despite immunosuppressive therapy. After administration of C1-INH, pleural effusions reduced from 19 l per day to 300 ml within 3 days of treatment. ECMO was discontinued after C1-INH treatment and the patient extubated 2 weeks later. This experience indicates that C1-INH may play a role in the management of capillary leak syndrome after lung transplantation.- - - - - - - - - - ranking = 8111.9555285882keywords = capillary leak syndrome, capillary leak, leak syndrome, capillary, leak (Clic here for more details about this article) |
9/36. Pulmonary capillary leak syndrome with intravenous cyclosporin A in pediatric renal transplantation.Despite frequent use of intravenous (i.v.) cyclosporin A (CsA) in the early post-operative course of transplant recipients, allergic reactions have been infrequently described. Of 134 transplants, we report four pediatric renal transplant recipients with severe reaction to i.v. CsA with pulmonary capillary leak syndrome. pulmonary edema developed at a mean time of 3.5 h after commencement of i.v. CsA, with two patients requiring mechanical ventilation. Discontinuation of i.v. CsA and conversion to oral CsA was followed by rapid resolution of pulmonary edema, suggesting that cremaphor, the solubilizing agent in the i.v. formulation, is likely to be responsible for this adverse response. skin prick testing with cremaphor was negative in all patients and alternative mechanisms for the cremaphor response are proposed. It is likely that inadequate mixing of the i.v. CsA solution triggered this reaction, by delivering a higher concentration of cremaphor at the start of the CsA infusion. pulmonary edema in the early post-transplant course in the absence of obvious fluid overload should prompt the diagnosis of an i.v. CsA reaction. This life-threatening reaction is easily reversible if recognized, and can be managed easily without compromise to the allograft, by discontinuing i.v. CsA and switching early to an oral CsA formulation.- - - - - - - - - - ranking = 13519.92588098keywords = capillary leak syndrome, capillary leak, leak syndrome, capillary, leak (Clic here for more details about this article) |
10/36. Non-Hodgkin's lymphoma presenting as anasarca: probably mediated by tumor necrosis factor alpha (TNF-alpha).Two patients presented with anasarca, fevers and sweats. Subsequent evaluation revealed aggressive lymphoproliferative disease. Both patients were treated with CHOP chemotherapy. One patient responded with spontaneous, vigorous diuresis and complete resolution of the edema. She relapsed two months later with recurrent edema that responded a second time to salvage chemotherapy. The second patient died of gram positive sepsis a week after diagnosis. As anasarca is an unusual presenting symptom of non-Hodgkin's lymphoma, we postulated that the malignant cells were secreting a cytokine that resulted in "vascular leakage" of fluid and development of diffuse edema. Several serum cytokine levels were tested. Both patients had elevated TNF-alpha levels, which could have been the cause of the edema; or there might be yet another unidentified mediator that was responsible for the anasarca. We report these two cases to bring to attention the unusual nature of this presentation.- - - - - - - - - - ranking = 0.25keywords = leak (Clic here for more details about this article) |
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