9 results on '"Detterich J"'
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2. Myocardial Iron Overload Causes Subclinical Myocardial Dysfunction in Sickle Cell Disease.
- Author
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Fujikura K, Cheng AL, Suriany S, Detterich J, Arai AE, and Wood JC
- Subjects
- Humans, Myocardium, Predictive Value of Tests, Anemia, Sickle Cell complications, Anemia, Sickle Cell diagnosis, Cardiomyopathies complications, Cardiomyopathies etiology, Iron Overload diagnostic imaging, Iron Overload etiology
- Published
- 2022
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3. Tricuspid regurgitant jet velocity and myocardial tissue Doppler parameters predict mortality in a cohort of patients with sickle cell disease spanning from pediatric to adult age groups - revisiting this controversial concept after 16 years of additional evidence.
- Author
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Shah P, Suriany S, Kato R, Bush AM, Chalacheva P, Veluswamy S, Denton CC, Russell K, Khaleel M, Forman HJ, Khoo MCK, Sposto R, Coates TD, Wood JC, and Detterich J
- Subjects
- Adolescent, Adult, Age Factors, Blood Flow Velocity, Disease-Free Survival, Female, Follow-Up Studies, Humans, Male, Middle Aged, Myocardium, Risk Factors, Survival Rate, Anemia, Sickle Cell complications, Anemia, Sickle Cell diagnostic imaging, Anemia, Sickle Cell mortality, Anemia, Sickle Cell physiopathology, Echocardiography, Doppler, Tricuspid Valve Insufficiency diagnostic imaging, Tricuspid Valve Insufficiency etiology, Tricuspid Valve Insufficiency mortality, Tricuspid Valve Insufficiency physiopathology
- Abstract
Sickle cell disease (SCD) is a monogenic hemoglobinopathy associated with significant morbidity and mortality. Cardiopulmonary, vascular and sudden death are the reasons for the majority of young adult mortality in SCD. To better understand the clinical importance of multi-level vascular dysfunction, in 2009 we assessed cardiac function including tricuspid regurgitant jet velocity (TRV), tissue velocity in systole(S') and diastole (E'), inflammatory, rheologic and hemolytic biomarkers as predictors of mortality in patients with SCD. With up to 9 years of follow up, we determined survival in 95 children, adolescents and adults with SCD. Thirty-eight patients (40%) were less than 21 years old at initial evaluation. Survival and Cox proportional-hazards analysis were performed. There was 19% mortality in our cohort, with median age at death of 35 years. In the pediatric subset, there was 11% mortality during the follow up period. The causes of death included cardiovascular and pulmonary complications in addition to other end-organ failure. On Cox proportional-hazards analysis, our model predicts that a 0.1 m/s increase in TRV increases risk of mortality 3%, 1 cm/s increase in S' results in a 91% increase, and 1 cm/s decrease in E' results in a 43% increase in mortality. While excluding cardiac parameters, higher plasma free hemoglobin was significantly associated with risk of mortality (p=.049). In conclusion, elevated TRV and altered markers of cardiac systolic and diastolic function predict mortality in a cohort of adolescents and young adult patients with SCD. These predictors should be considered when counseling cardiovascular risk and therapeutic optimization at transition to adult providers., (© 2020 Wiley Periodicals LLC.)
- Published
- 2021
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4. Progressive vasoconstriction with sequential thermal stimulation indicates vascular dysautonomia in sickle cell disease.
- Author
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Veluswamy S, Shah P, Khaleel M, Thuptimdang W, Chalacheva P, Sunwoo J, Denton CC, Kato R, Detterich J, Wood JC, Sposto R, Khoo MCK, Zeltzer L, and Coates TD
- Subjects
- Adolescent, Child, Child, Preschool, Female, Humans, Infant, Infant, Newborn, Male, Primary Dysautonomias etiology, Vascular Diseases etiology, Anemia, Sickle Cell complications, Microvessels physiopathology, Primary Dysautonomias pathology, Temperature, Vascular Diseases pathology, Vasoconstriction
- Abstract
Persons with sickle cell disease (SCD) exhibit subjective hypersensitivity to cold and heat perception in experimental settings, and triggers such as cold exposure are known to precipitate vaso-occlusive crises by still unclear mechanisms. Decreased microvascular blood flow (MBF) increases the likelihood of vaso-occlusion by increasing entrapment of sickled red blood cells in the microvasculature. Because those with SCD have dysautonomia, we anticipated that thermal exposure would induce autonomic hypersensitivity of their microvasculature with an increased propensity toward vasoconstriction. We exposed 17 patients with SCD and 16 control participants to a sequence of predetermined threshold temperatures for cold and heat detection and cold and heat pain via a thermode placed on the right hand. MBF was measured on the contralateral hand by photoplethysmography, and cardiac autonomic balance was assessed by determining heart rate variability. Thermal stimuli at both detection and pain thresholds caused a significant decrease in MBF in the contralateral hand within seconds of stimulus application, with patients with SCD showing significantly stronger vasoconstriction (P = .019). Furthermore, patients with SCD showed a greater progressive decrease in blood flow than did the controls, with poor recovery between episodes of thermal stimulation (P = .042). They had faster vasoconstriction than the controls (P = .033), especially with cold detection stimulus. Individuals with higher anxiety also experienced more rapid vasoconstriction (P = .007). Augmented vasoconstriction responses and progressive decreases in perfusion with repeated thermal stimulation in SCD are indicative of autonomic hypersensitivity in the microvasculature. These effects are likely to increase red cell entrapment in response to clinical triggers such as cold or stress, which have been associated with vaso-occlusive crises in SCD., (© 2020 by The American Society of Hematology.)
- Published
- 2020
- Full Text
- View/download PDF
5. Mental stress causes vasoconstriction in subjects with sickle cell disease and in normal controls.
- Author
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Shah P, Khaleel M, Thuptimdang W, Sunwoo J, Veluswamy S, Chalacheva P, Kato RM, Detterich J, Wood JC, Zeltzer L, Sposto R, Khoo MCK, and Coates TD
- Subjects
- Autonomic Nervous System, Humans, Stress, Psychological, Vasoconstriction, Anemia, Sickle Cell complications, Vascular Diseases
- Abstract
Vaso-occlusive crisis (VOC) is a hallmark of sickle cell disease (SCD) and occurs when deoxygenated sickled red blood cells occlude the microvasculature. Any stimulus, such as mental stress, which decreases microvascular blood flow will increase the likelihood of red cell entrapment resulting in local vaso-occlusion and progression to VOC. Neurally mediated vasoconstriction might be the physiological link between crisis triggers and vaso-occlusion. In this study, we determined the effect of mental stress on microvascular blood flow and autonomic nervous system reactivity. Sickle cell patients and controls performed mentally stressful tasks, including a memory task, conflict test and pain anticipation test. Blood flow was measured using photoplethysmography, autonomic reactivity was derived from electrocardiography and perceived stress was measured by the State-Trait Anxiety Inventory questionnaire. Stress tasks induced a significant decrease in microvascular blood flow, parasympathetic withdrawal and sympathetic activation in all subjects. Of the various tests, pain anticipation caused the highest degree of vasoconstriction. The magnitude of vasoconstriction, sympathetic activation and perceived stress was greater during the Stroop conflict test than during the N-back memory test, indicating the relationship between magnitude of experimental stress and degree of regional vasoconstriction. Baseline anxiety had a significant effect on the vasoconstrictive response in sickle cell subjects but not in controls. In conclusion, mental stress caused vasoconstriction and autonomic nervous system reactivity in all subjects. Although the pattern of responses was not significantly different between the two groups, the consequences of vasoconstriction can be quite significant in SCD because of the resultant entrapment of sickle cells in the microvasculature. This suggests that mental stress can precipitate a VOC in SCD by causing neural-mediated vasoconstriction., (Copyright© 2020 Ferrata Storti Foundation.)
- Published
- 2020
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6. Individuals with sickle cell disease have a significantly greater vasoconstriction response to thermal pain than controls and have significant vasoconstriction in response to anticipation of pain.
- Author
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Khaleel M, Puliyel M, Shah P, Sunwoo J, Kato RM, Chalacheva P, Thuptimdang W, Detterich J, Wood JC, Tsao J, Zeltzer L, Sposto R, Khoo MCK, and Coates TD
- Subjects
- Adaptation, Physiological, Adult, Anemia, Sickle Cell metabolism, Anemia, Sickle Cell therapy, Blood Transfusion, Case-Control Studies, Female, Heart Function Tests, Humans, Hypoxia metabolism, Hypoxia physiopathology, Male, Microvessels metabolism, Microvessels physiopathology, Pain diagnosis, Regional Blood Flow, Temperature, Anemia, Sickle Cell complications, Anemia, Sickle Cell physiopathology, Pain etiology, Pain physiopathology, Vasoconstriction
- Abstract
The painful vaso-occlusive crises (VOC) that characterize sickle cell disease (SCD) progress over hours from the asymptomatic steady-state. SCD patients report that VOC can be triggered by stress, cold exposure, and, pain itself. We anticipated that pain could cause neural-mediated vasoconstriction, decreasing regional blood flow and promoting entrapment of sickle cells in the microvasculature. Therefore, we measured microvascular blood flow in the fingers of both hands using plethysmography and laser-Doppler flowmetry while applying a series of painful thermal stimuli on the right forearm in 23 SCD patients and 25 controls. Heat pain applied to one arm caused bilateral decrease in microvascular perfusion. The vasoconstriction response started before administration of the thermal pain stimulus in all subjects, suggesting that pain anticipation also causes significant vasoconstriction. The time delay between thermal pain application and global vasoconstriction ranged from 5 to 15.5 seconds and increased with age (P < .01). Although subjective measures, pain threshold and pain tolerance were not different between SCD subjects and controls, but the vaso-reactivity index characterizing the microvascular blood flow response to painful stimuli was significantly higher in SCD patients (P = .0028). This global vasoconstriction increases microvascular transit time, and may promote entrapment of sickle cells in the microvasculature, making vaso-occlusion more likely. The rapidity of the global vasoconstriction response indicates a neural origin that may play a part in the transition from steady-state to VOC, and may also contribute to the variability in VOC frequency observed in SCD patients., (© 2017 Wiley Periodicals, Inc.)
- Published
- 2017
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7. The role of blood rheology in sickle cell disease.
- Author
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Connes P, Alexy T, Detterich J, Romana M, Hardy-Dessources MD, and Ballas SK
- Subjects
- Anemia, Sickle Cell complications, Anemia, Sickle Cell genetics, Anemia, Sickle Cell metabolism, Blood Coagulation, Hematocrit, Hemolysis, Humans, Mutation, Oxidative Stress, Prognosis, Anemia, Sickle Cell blood, Blood Viscosity, Erythrocyte Deformability, Hemorheology
- Abstract
Studies performed in the last decades have highlighted the need to better understand the contribution of the endothelium, vascular function, oxidative stress, inflammation, coagulation, hemolysis and vascular adhesion mechanisms to the pathophysiology of acute vaso-occlusive like events and chronic organ damages in sickle cell disease (SCD). Although SCD is a hemorheological disease, a few works focused on the contribution of blood viscosity, plasma viscosity, red blood cell deformability and aggregation in the pathophysiology of SCD. After a brief description of basic hemorheology, the present review focuses on the role of the hemorheological abnormalities in the causation of several SCD complications, mainly in sickle cell anemia and hemoglobin (Hb) SC disease. Several genetic and cellular modulators of blood rheology in SCD are discussed, as well as unresolved questions and perspectives.
- Published
- 2016
- Full Text
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8. Comparison of biventricular dimensions and function between pediatric sickle-cell disease and thalassemia major patients without cardiac iron.
- Author
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Meloni A, Detterich J, Berdoukas V, Pepe A, Lombardi M, Coates TD, and Wood JC
- Subjects
- Adolescent, Anemia, Sickle Cell metabolism, Anemia, Sickle Cell pathology, Blood Pressure, Child, Female, Heart Ventricles metabolism, Heart Ventricles pathology, Humans, Iron Overload etiology, Iron Overload metabolism, Iron Overload pathology, Magnetic Resonance Imaging, Male, Sex Factors, Stroke Volume, Transfusion Reaction, Ventricular Dysfunction, Left metabolism, Ventricular Dysfunction, Left pathology, Ventricular Function, Right, beta-Thalassemia metabolism, beta-Thalassemia pathology, Anemia, Sickle Cell physiopathology, Heart Ventricles physiopathology, Iron metabolism, Iron Overload physiopathology, Ventricular Dysfunction, Left physiopathology, beta-Thalassemia physiopathology
- Abstract
Patients with chronic anemia develop compensatory ventricular dilation, even when maintained on chronic transfusion regimens. It is important to characterize these effects to interpret pathological changes in cardiac dimensions and function introduced by iron overload and sickle cell vasculopathy. Our primary goal was to compare biventricular dimensions and function assessed by cardiovascular magnetic resonance (CMR) in pediatric, chronically-transfused sickle-cell disease (SCD) and thalassemia major (TM) patients who had normal cardiac iron levels. Moreover, we explored systematic sex differences in ventricular dimensions in both populations. We identified 261 studies suitable for analysis from 64 patients with SCD (34 females) and 49 patients with TM (20 females). All demographic and CMR parameters were inversely weighted by the number of exams. In both populations, males had larger left and right ventricular dimensions than females, with a more marked effect observed in patients with SCD. Compared to patients with TM, patients with SCD showed significantly greater biventricular dilation and left ventricular hypertrophy. This difference could not be explained by different hemoglobin levels, cardiac iron overload, and systolic blood pressure. The left ventricular (LV) ejection fraction (EF) for the males and the right ventricular (RV) EF for both the sexes were comparable between SCD and TM groups, while females with SCD had significantly lower LV EF than females with TM. Our results represent important baseline findings that place changes introduced by iron overload as well as systemic and pulmonary vasculopathy in proper context., (Copyright © 2012 Wiley Periodicals, Inc.)
- Published
- 2013
- Full Text
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9. Low-shear red blood cell oxygen transport effectiveness is adversely affected by transfusion and further worsened by deoxygenation in sickle cell disease patients on chronic transfusion therapy.
- Author
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Detterich J, Alexy T, Rabai M, Wenby R, Dongelyan A, Coates T, Wood J, and Meiselman H
- Subjects
- Adolescent, Adult, Anemia, Sickle Cell metabolism, Biological Transport, Blood Viscosity physiology, Child, Cross-Sectional Studies, Efficiency physiology, Female, Hemoglobin, Sickle metabolism, Humans, Male, Oxygen Consumption physiology, Shear Strength, Time Factors, Young Adult, Anemia, Sickle Cell blood, Anemia, Sickle Cell therapy, Erythrocyte Transfusion adverse effects, Erythrocytes metabolism, Oxygen metabolism
- Abstract
Background: Simple chronic transfusion therapy (CTT) is a mainstay for stroke prophylaxis in sickle cell anemia, but its effects on hemodynamics are poorly characterized. Transfusion improves oxygen-carrying capacity, reducing demands for high cardiac output. While transfusion decreases factors associated with vasoocclusion, including percent hemoglobin (Hb)S, reticulocyte count, and circulating cell-free Hb, it increases blood viscosity, which reduces microvascular flow. The hematocrit-to-viscosity ratio (HVR) is an index of red blood cell oxygen transport effectiveness that varies with shear stress and balances the benefits of improved oxygen capacity to viscosity-mediated impairment of microvascular flow. We hypothesized that transfusion would improve HVR at high shear despite increased blood viscosity, but would decrease HVR at low shear., Study Design and Methods: To test this hypothesis, we examined oxygenated and deoxygenated blood samples from 15 sickle cell patients on CTT immediately before transfusion and again 12 to 120 hours after transfusion., Results: Comparable changes in Hb, hematocrit (Hct), reticulocyte count, and HbS with transfusion were observed in all subjects. Viscosity, Hct, and high-shear HVR increased with transfusion while low-shear HVR decreased significantly., Conclusion: Decreased low-shear HVR suggests impaired oxygen transport to low-flow regions and may explain why some complications of sickle cell anemia are ameliorated by CTT and others may be made worse., (© 2012 American Association of Blood Banks.)
- Published
- 2013
- Full Text
- View/download PDF
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