1/13. 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 = rate (Clic here for more details about this article) |
2/13. 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 = 1keywords = rate (Clic here for more details about this article) |
3/13. 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 = 7keywords = rate (Clic here for more details about this article) |
4/13. 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 = 33.415915842413keywords = mortality, rate (Clic here for more details about this article) |
5/13. 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 = 3keywords = rate (Clic here for more details about this article) |
6/13. Systemic capillary leak syndrome preceding plasma cell leukaemia.We report a patient with plasma cell leukaemia with systemic capillary leak syndrome, a rare disorder often associated with monoclonal gammopathy. In this patient, the manifestation of capillary leak syndrome antedated the diagnosis of plasma cell leukaemia by 5-6 months. During that time, he was repeatedly admitted to the hospital with weight gain, congestive cardiac failure, cough and anasarca in the presence of normal renal function, liver function and normal echocardiography. On presentation, a serum protein electrophoresis showed monoclonal IgG; the blood smear showed 60% plasma cells with a total count of 4.4 x 10(9)/l. A bone marrow aspirate showed replacement of the normal marrow by sheets of immature plasma cells. His systemic capillary leak syndrome initially responded to decongestive therapy with terbutaline and aminophylline but later on he became refractory to them and responded to vincristine, doxorubicin and dexamethasone (VAD) combination therapy only transiently. Danocrine and pentoxifylline, added during VAD chemotherapy, did not produce a durable response in capillary leak syndrome, which finally responded to autologous peripheral blood stem cell transplantation (PBSCT). After PBSCT, he remained free of capillary leak for 10 months without terbutaline, pentoxifylline corticosteroids, aminophylline or danocrine. His disease relapsed without recurrence of the capillary leak. He died 15 months after PBSCT and 20 months after the diagnosis of plasma cell leukaemia.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
7/13. Gemcitabine-induced systemic capillary leak syndrome.Systemic capillary leak syndrome (SCLS) is a rare disorder with a high mortality rate, characterized by rapidly developing edema, weight gain and hypotension, hemoconcentration and hypoproteinemia. This syndrome is caused by sudden, reversible capillary hyperpermeability with a rapid extravasation of plasma from the intravascular to the interstitial space. Even though SCLS has been suggested to be the pathogenic mechanism for the pulmonary toxicity of gemcitabine (GCB), a new deoxycytidine analogue with structural similarities to cytosine arabinoside, a direct correlation between GCB and SCLS has never been reported. We describe a case of repeated SCLS after GCB administration in a 51-year-old male with locally-advanced non-small-cell lung cancer treated with a combination of cisplatin and GCB. The detection of GCB-induced SCLS supports the hypothesis that SCLS could be the pathogenic way of GCB pulmonary toxicity. This finding can help to better understand and treat the potentially deadly GCB-related acute respiratory distress syndrome that is being recognized.- - - - - - - - - - ranking = 29.415915842413keywords = mortality, rate (Clic here for more details about this article) |
8/13. Decreased hepatic uptake of gallium-67 citrate in haemophagocytic syndrome occurring concomitantly with capillary leak syndrome.We describe a 4-year-old girl with haemophagocytic syndrome (HPS) in whom hepatic gallium-67 citrate (Ga-67) uptake was suppressed when the disease was in its acute phase and returned to normal when the disease was in remission. The prominent clinical feature of this case was the occurrence of systemic capillary leak syndrome (CLS). Because extravasation of plasma proteins may be the result of vascular endothelial injury in CLS, loss of hepatic Ga-67 uptake may reflect insult to the hepatic sinusoidal endothelium. This case suggests a possible role of sinusoidal endothelial cells in the mechanism of hepatic Ga-67 uptake and indicates the need for further study of Ga-67 uptake in patients with HPS.- - - - - - - - - - ranking = 5keywords = rate (Clic here for more details about this article) |
9/13. granulocyte colony-stimulating factor-induced capillary leak syndrome confirmed by extravascular lung water measurements.The study purpose was to report the first case of granulocyte colony-stimulating factor (G-CSF)-induced capillary leak syndrome (CLS) in which serial extravascular lung water (EVLW) measurements were performed and to compare this case with previously reported cases. To identify previously reported cases, we performed a literature search, using pubmed with the following search terms: CLS, EVLW, G-CSF, granulocyte-macrophage colony-stimulating factor (GM-CSF) and stem cell transplantation and the references in the bibliographies of the papers retrieved. To obtain additional information about these cases, we contacted the authors by e-mail. We describe the case of a 68-year-old woman who developed severe CLS during G-CSF treatment after autologous haematological stem cell transplantation. CLS is caused by damage to the endothelial cells, resulting in extravasation of plasma proteins and fluid from the capillaries into the extravascular space. This is illustrated by high values of EVLW and pulmonary vascular permeability, necessitating mechanical ventilation. We found five other case reports in the literature. The white blood cell count at the onset of the CLS varied from very low (zero) to very high (90,500/mul). The symptoms began on day 5-9 of the G-CSF treatment. All patients had fever. Three patients were mechanically ventilated and four received renal replacement therapy. Two patients died. Treatment with G-CSF can induce fatal CLS. Monitoring of EVLW in patients with severe CLS may be useful to guide fluid therapy and improve oxygenation.- - - - - - - - - - ranking = 1keywords = rate (Clic here for more details about this article) |
10/13. Severe gemcitabine-induced capillary-leak syndrome mimicking cardiac failure in a patient with advanced pancreatic cancer and high-risk cardiovascular disease.Pancreatic adenocarcinoma is the fourth most common cause of cancer-related mortality in the developed world. The nucleoside analogue gemcitabine is established standard therapy for advanced disease. Gemcitabine can occasionally cause non-dose-dependent pulmonary toxicity, and the systemic complication of capillary-leak syndrome, which may mimic cardiogenic pulmonary oedema. Here, we present a case of capillary-leak syndrome in a patient with pancreatic cancer treated with gemcitabine, with a past history of severe cardiovascular dysfunction and coronary arteriosclerosis.- - - - - - - - - - ranking = 28.415915842413keywords = mortality (Clic here for more details about this article) |
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