scholarly journals Retinal Occlusive Vasculitis in a Patient with Hyperimmunoglobulin E Syndrome

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Mohsen Farvardin ◽  
Mohammad Hassan Jalalpour ◽  
Mohammad Reza Khalili ◽  
Golnoush Mahmoudinezhad ◽  
Fereshteh Mosavat ◽  
...  

Background. Hyperimmunoglobulin E syndrome (HIES), or Job’s syndrome, is a primary immunodeficiency disorder that is characterized by an elevated level of IgE with values reaching over 2000 IU ( normal < 200   IU ), eczema, and recurrent staphylococcus infection. Affected individuals are predisposed to infection, autoimmunity, and inflammation. Herein, we report a case of HIES with clinical findings of retinal occlusive vasculitis. Case Presentation. A 10-year-old boy with a known case of hyperimmunoglobulin E syndrome had exhibited loss of vision and bilateral dilated fixed pupil. Fundoscopic examination revealed peripheral retinal hemorrhaging, vascular sheathing around the retinal arteries and veins, and vascular occlusion in both eyes. A fluorescein angiography of the right eye showed hyper- and hypofluorescence in the macula and hypofluorescence in the periphery of the retina, peripheral arterial narrowing, and arterial occlusion. A fluorescein angiography of the left eye showed hyper- and hypofluorescence in the supranasal area of the optic disc. Macular optical coherence tomography of the right eye showed inner and outer retinal layer distortion. A genetic study was performed that confirmed mutations of the dedicator of cytokinesis 8 (DOCK 8). HSV polymerase chain reaction testing on aqueous humor and vitreous was negative, and finally, the patient was diagnosed with retinal occlusive vasculitis. Conclusion. Occlusive retinal vasculitis should be considered as a differential diagnosis in patients with hyperimmunoglobulin E syndrome presenting with visual loss.

1976 ◽  
Vol 45 (5) ◽  
pp. 514-519 ◽  
Author(s):  
S. David Gertz ◽  
Marshall L. Rennels ◽  
Michael S. Forbes ◽  
Junichiro Kawamura ◽  
Toshiaki Sunaga ◽  
...  

✓ The effects of temporary vascular occlusion with surgical clips on the underlying endothelial lining were studied with scanning (SEM) and transmission (TEM) electron microscopy. Twenty-five rabbits were anesthetized and both common carotid arteries exposed. A Heifetz clip was used to occlude the right carotid artery for 5, 15, and 30 minutes, and 2 hours in five animals each. The clips were removed and the vessels immediately perfused with glutaraldehyde. In the five remaining animals, the right carotid arteries were occluded for 30 minutes followed by removal of the clip and resumption of blood flow for 30 minutes prior to fixation. Combined SEM and TEM examination of the endothelium of compressed segments revealed “craters” and “balloons,” blebs and vacuoles, swollen mitochondria, dilated granular endoplasmic reticulum, and subendothelial edema. There were also areas of endothelial cell flattening, discontinuity, and desquamation exposing the subendothelial tissues. Following restoration of flow, platelets and fibrin were found adherent to altered endothelial cells and to exposed subendothelial tissues. Endothelial craters and balloons were also found distal and, significantly less frequently, proximal to the site of occlusion. It is suggested that antiplatelet aggregating agents may prove beneficial for the prevention of thrombus formation at the site of the clip as well as craters and balloons distal to the clip following procedures requiring temporary vascular occlusion.


2017 ◽  
Author(s):  
Mark A Creager

Peripheral arterial diseases (PADs) compromise blood flow to the limbs. Common causes of arterial obstruction include atherosclerosis, thrombus, embolism, vasculitis, arterial entrapment, adventitial cysts, fibromuscular dysplasia, arterial dissection, trauma, and vasospasm. The most frequently encountered cause of PAD is peripheral atherosclerosis. This chapter considers its epidemiology and risk factors, as well as its diagnosis, including clinical presentation and noninvasive diagnostic tests. This chapter also discusses acute arterial occlusion, atheroembolism, popliteal artery entrapment, thromboangiitis obliterans, and acrocyanosis, as well as the etiology, diagnosis, and treatment of Raynaud phenomenon. The chapter contains 4 tables and 7 figures. Tables describe the Fontaine classification and clinical categories of chronic limb ischemia, provide examples of leg segmental pressure measurements in a patient with calf claudication and foot pain, and summarize secondary causes of Raynaud phenomenon. Figures include a photograph of an ischemic foot demonstrating dependent rubor, measurement of the ankle:brachial index, ultrasonography of a stenosis of the right common femoral artery, magnetic resonance angiograms of patients with calf claudication, arteriograms of critical ischemia of the foot and of disabling claudication of the leg, and ischemia of the toes caused by atheroemboli. This chapter contains 80 references.


2012 ◽  
Author(s):  
Mark A Creager

Peripheral arterial diseases (PADs) compromise blood flow to the limbs. Common causes of arterial obstruction include atherosclerosis, thrombus, embolism, vasculitis, arterial entrapment, adventitial cysts, fibromuscular dysplasia, arterial dissection, trauma, and vasospasm. The most frequently encountered cause of PAD is peripheral atherosclerosis. This chapter considers its epidemiology and risk factors, as well as its diagnosis, including clinical presentation and noninvasive diagnostic tests. This chapter also discusses acute arterial occlusion, atheroembolism, popliteal artery entrapment, thromboangiitis obliterans, and acrocyanosis, as well as the etiology, diagnosis, and treatment of Raynaud phenomenon. Tables describe the Fontaine classification and clinical categories of chronic limb ischemia, provide examples of leg segmental pressure measurements in a patient with calf claudication and foot pain, and summarize secondary causes of Raynaud phenomenon. Figures include a photograph of an ischemic foot demonstrating dependent rubor, measurement of the ankle:brachial index, ultrasonography of a stenosis of the right common femoral artery, magnetic resonance angiograms of patients with calf claudication, arteriograms of critical ischemia of the foot and of disabling claudication of the leg, and ischemia of the toes caused by atheroemboli. This review contains 4 highly rendered figures, 7 tables, and 80 references.


1988 ◽  
Vol 64 (3) ◽  
pp. 1229-1238 ◽  
Author(s):  
T. R. Chappell ◽  
S. S. Cassidy ◽  
F. Schwiep ◽  
M. Ramanathan ◽  
R. L. Johnson

The purpose of these experiments was to quantify stagnant intrapulmonary blood caused by a pulmonary arterial occlusion (PAO). The hypothesis was that the diffusing capacity of the lung for CO (DLCO) would be altered little by PAO when measured with the usual inspired concentrations (0.3%) of CO, since stagnant blood distal to the occlusion takes up CO for 20 s or more before significant CO backpressure would develop. However, higher levels of CO (i.e., greater than or equal to 3%) would equilibrate faster with capillary blood (within 5-10 s), and DLCO measured 10-20 s subsequent to the high CO exposure would reflect only the DLCO in the unoccluded regions. Thus the fractional reduction in DLCO measured with 3% CO, with respect to that measured with 0.3% CO, should be related to the fractional occlusion of the pulmonary artery in a predictable way. We occluded the right pulmonary artery (RPAO), the left pulmonary artery (LPAO), or the left lower lobar artery (LLPAO) and found that DLCO measured during rebreathing a 0.3% CO mixture was 80, 87, and 94%, respectively, of the preocclusion value, whereas the DLCO measured during rebreathing a 3.3% CO mixture was 59, 73, and 87% of the preocclusion value. A computer model was developed to predict the reduction in DLCO at different levels of CO exposure that would be caused by varying fractions of PAO. Our data indicated that RPAO corresponded to a 42% vascular occlusion, LPAO a 35% occlusion, and LLPAO a 20% occlusion. Measurement of DLCO using low and high concentrations of CO might be useful in assessing the fraction of vascular bed occluded and in following noninvasively the course of vascular occlusion in a variety of pulmonary diseases.


2004 ◽  
Vol 91 (05) ◽  
pp. 991-999 ◽  
Author(s):  
Andrej Tarkowski ◽  
Maria Bokarewa

SummaryDefensins, cationic peptides with bacteriolytic properties, are abundantly found at inflammation sites and in human coronary vessels. Vascular occlusive diseases, such as myocardial infarction, pulmonary embolism, and peripheral arterial occlusion are presently treated by thrombolytic intervention using staphylokinase, a plasminogen activator of bacterial origin. In this study we assessed a possible interaction between defensins and staphylokinase, both molecules being present in an acutely ill patient. Using an ELISA-based system, we found that staphylokinase and defensins displayed a strong and dose-dependent binding. In contrast, urokinase, another plasminogen activator of endogenous origin, displayed only minimal binding to defensins. Next, we proved that interaction between staphylokinase and defensins led to fuctional consequences resulting in a significant decrease (p<0.002) of plasminogen activation capacity upon complex formation. In contrast, urokinase retained most of its activity even in 10-fold molar excess of defensins. Finally, we found that staphylokinase-triggered lysis of fibrin was efficiently inhibited in the presence of defensins. To assess structural requirements for staphylokinase/defensin interaction, six staphylokinase mutant variants were studied. Inactivation pattern of the tested staphylokinase variants suggested a direct binding of defensins to serine protease-like domain of staphylokinase. In conclusion, we show complex formation between staphylokinase and α-defensins resulting in a significant reduction of fibrinolytic activity. This finding may have clinical implications, since fibrinolytic effects of staphylokinase may be downregulated at the site of vascular occlusion.


2017 ◽  
Author(s):  
Mark A Creager

Peripheral arterial diseases (PADs) compromise blood flow to the limbs. Common causes of arterial obstruction include atherosclerosis, thrombus, embolism, vasculitis, arterial entrapment, adventitial cysts, fibromuscular dysplasia, arterial dissection, trauma, and vasospasm. The most frequently encountered cause of PAD is peripheral atherosclerosis. This chapter considers its epidemiology and risk factors, as well as its diagnosis, including clinical presentation and noninvasive diagnostic tests. This chapter also discusses acute arterial occlusion, atheroembolism, popliteal artery entrapment, thromboangiitis obliterans, and acrocyanosis, as well as the etiology, diagnosis, and treatment of Raynaud phenomenon. The chapter contains 4 tables and 7 figures. Tables describe the Fontaine classification and clinical categories of chronic limb ischemia, provide examples of leg segmental pressure measurements in a patient with calf claudication and foot pain, and summarize secondary causes of Raynaud phenomenon. Figures include a photograph of an ischemic foot demonstrating dependent rubor, measurement of the ankle:brachial index, ultrasonography of a stenosis of the right common femoral artery, magnetic resonance angiograms of patients with calf claudication, arteriograms of critical ischemia of the foot and of disabling claudication of the leg, and ischemia of the toes caused by atheroemboli. This chapter contains 80 references.


1991 ◽  
Vol 65 (05) ◽  
pp. 635-635 ◽  
Author(s):  
Claude Juhan ◽  
Serge Haupert ◽  
Gilles Miltgen ◽  
Nadine Girard ◽  
Pierre Dulac

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S507-S507
Author(s):  
Lauren F Collins ◽  
Jessica G Shantha ◽  
Peter L Nesper ◽  
Anandi N Sheth ◽  
Amani A Fawzi ◽  
...  

Abstract Background Mechanisms underlying the rising burden of non-AIDS comorbidities (NACM) among persons with HIV (PWH) remain unclear. Microvasculopathy may link HIV-related chronic inflammation and premature multimorbidity, similar to diabetes and other conditions characterized by inflammatory end-organ damage. We used a novel retinovascular imaging tool, optical coherence tomography angiography (OCTA), to evaluate the retina as a convenient assessment of microvascular health among PWH. Methods Data from 4 PWH who underwent OCTA (Zeiss CIRRUSTM HD-OCT 5000) at the Emory Eye Center from 2018-2020 were analyzed. Demographics, HIV-specific indices and NACM were summarized at the time of OCTA. Images were reviewed qualitatively and metrics of microvascular health – the foveal avascular zone (FAZ) area and vessel density (VD) from the superficial capillary plexus (SCP) – were calculated by ImageJ. Results The median age was 39 years, 100% were male, 100% were black, 25% had ever smoked, and median body mass index was 25.4 kg/m2. Median time since HIV diagnosis was 19 years, all patients had a history of clinical AIDS, including 2 with prior cytomegalovirus retinitis. Median current CD4 count was 84 cells/mm3, 100% were prescribed antiretroviral therapy and 50% had HIV viral suppression. Prevalent NACM included (each n=1): hypertension, dyslipidemia, diabetes, chronic kidney disease and asthma. Qualitatively, all 7 of the eyes evaluated by OCTA had evidence of microvascular pathology: 2 eyes demonstrated diffuse capillary nonperfusion, while the remaining 5 eyes had focal areas of nonperfusion around the FAZ. Mean FAZ area was 0.31 (SD±0.10) mm2 and mean VD of the SCP was 43.9% (SD±10.9%). Retinovascular pathology identified by fundoscopy and OCTA is shown in the figure. Figure. Retinal imaging of a PWH with bilateral retinal vasculitis. Fundus photos of the right (A) and left (C) eyes show retinal vasculitis highlighted by the red arrows. OCTA of the right (B) and left (D) maculae (3X3 scan Zeiss AngioplexTM) show the FAZ areas outlined in yellow, both of irregular contour. OCTA of the left macula demonstrates areas of significant flow voids marked by the asterisks and the FAZ area is enlarged. Conclusion Among patients with longstanding HIV, OCTA identified microvascular abnormalities in all retinae examined. Retinovascular evaluation by OCTA is a feasible, non-invasive technique for assessing microvascular health and findings support additional study in a larger, more diverse group of PWH. Screening tools targeting microvasculopathy among PWH may aid in earlier detection of those at greatest risk of NACM and allow for aggressive risk-modification strategies. Disclosures All Authors: No reported disclosures


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