Effect Of Genotype On Pulmonary Hypertension In Patients With Thalassemia

Abstract Introduction Pulmonary hypertension is one of the major complications in patients with thalassemia. The prevalence is higher among patients with non - transfusion dependent thalassemia (NTDT) than those patients with thalassemia major. Patients with NTDT have distinct genetic subgroups. Therefore, the effects of different genotype groups on pulmonary hypertension in patients with NTDT were assessed. Methods A cross-sectional study was conducted in patients with NTDT aged ≥ 10 years old at Srinagarind University Hospital and Udonthani Hospital. Pulmonary hypertension was defined as a tricuspid regurgitation velocity > 2.9 m/s by trans-thoracic echocardiography. All patients gave consent and the protocol was approved by the Ethics committee of Faculty of Medicine, KhonKaen University and Udonthani Hospital. Clinical characteristics and laboratory data that literature indicated as risk factors for pulmonary hypertension were collected. Genotypes were determined by hemoglobin typing and DNA analysis. The effect of genotype group on pulmonary hypertension was evaluated by using multivariate logistic regression analysis. Results Two hundred twenty two patients were recruited, 3 patients were excluded (1 patient with congenital heart disease and 2 patients with valvular heart disease). In a total of 219 patients, pulmonary hypertension was found in 24 patients (10.96%). All patients were categorized into 2 groups according to genetic data that included: 1) β-thalassemia (140, 63.93%) 2) α-thalassemia and combined α and β-thalassemia (79, 36.07%).Genotype groups were statistically and significantly associated with pulmonary hypertension based on the adjusted odds ratios after adjustment for other factors. Patients with β-thalassemia had a statistically significant higher risk for pulmonary hypertension compared to patients with α-thalassemia and patients with combined α and β-thalassemia had an odds ratio of 9.47 (p=0.036). Conclusion Genotype group is an independent risk factor for pulmonary hypertension in patients with NTDT. Echocardiography should be routinely recommended in patients with β-thalassemia. In patients with α-thalassemia and combined α and β-thalassemia, pulmonary hypertension is uncommon. Therefore, echocardiography should be only performed when these patients have a clinical indication. Abbreviation Hb CS = Hemoglobin Constant spring, Hb Pakse´= Hemoglobin Pakse´ Abbreviation PHT= pulmonary hypertension, AOR= adjusted odds ratio, 95% CI= 95% confidence interval Disclosures: No relevant conflicts of interest to declare.

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Abstract WP211: The Association Between Arterial Stiffness and Perivascular Space Topography in Stroke Patients

Stroke ◽

10.1161/str.47.suppl_1.wp211 ◽

2016 ◽

Vol 47 (suppl_1) ◽

Author(s):

Jae-Han Bae ◽

Kwang-Yeol Park ◽

Jeong-Min Kim ◽

Moo-Suk Park

Keyword(s):

Basal Ganglia ◽

Arterial Stiffness ◽

Confidence Interval ◽

Odds Ratio ◽

Laboratory Data ◽

Multivariate Logistic Regression Analysis ◽

Perivascular Space ◽

University Hospital ◽

Centrum Semiovale ◽

High Degree

Background: Arterial stiffness aggravation is known to increase cerebral small vessel disease burden. We investigated the association between brachial-ankle pulse wave velocity (baPWV) and the topography of MRI visible perivascular space in acute stroke population. Method: We analyzed clinical and laboratory data of acute ischemic stroke or transient ischemic attack patients who had been admitted to Chung-Ang University Hospital within 7 days after symptom onset between January 1st 2014 and May 31th 2015. We included those patients who underwent both brain MR imaging including T2 weighted sequence and baPWV. The topography of perivascular space was examined in basal ganglia and centrum semiovale level with validating scale (score 0-4), and dichotomized as low (score3). Result: A total of 481 patients were included (mean age 68.2±12.3, 44.7% male). When the distribution of baPWV were divided into quartile, the proportions of high PVS in basal ganglia was 10%, 20%, 40% and 40%, respectively. The proportion of high PVS in centrum semiovale was 20%, 40%, 36%, and 49%, respectively. Multivariate logistic regression analysis revealed that the high degree of basal ganglia PVS was associated with baPWV>2255cm/s (odds ratio 1.7, confidence interval 1.0-2.7, p=0.048) after adjusting age>70 years, hypertension, current smoking status and previous stroke history. The high degree of centrum semiovale PVS showed marginal association with baPWV>2255cm/s (odds ratio=1.4, confidence interval 0.9-2.3, p=0.137) after adjusting age>70 years and hypertension. Conclusion: Our pilot study suggests potential pathophysiological association between arterial stiffness and PVS topography.

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Clinical and laboratory characteristics associated with referral of hospitalized elderly to palliative care

Einstein (São Paulo) ◽

10.1590/s1679-45082018ao4092 ◽

2018 ◽

Vol 16 (1) ◽

Author(s):

Suelen Pereira Arcanjo ◽

Luis Alberto Saporetti ◽

José Antonio Esper Curiati ◽

Wilson Jacob-Filho ◽

Thiago Junqueira Avelino-Silva

Keyword(s):

Older Adults ◽

Palliative Care ◽

Laboratory Data ◽

Stage Iv ◽

University Hospital ◽

Clinical Indication ◽

Care Group ◽

Chronic Obstructive ◽

Stepwise Logistic Regression ◽

Advanced Dementia

ABSTRACT Objective To investigate clinical and laboratory characteristics associated with referral of acutely ill older adults to exclusive palliative care. Methods A retrospective cohort study based on 572 admissions of acutely ill patients aged 60 years or over to a university hospital located in São Paulo, Brazil, from 2009 to 2013. The primary outcome was the clinical indication for exclusive palliative care. Comprehensive geriatric assessments were used to measure target predictors, such as sociodemographic, clinical, cognitive, functional and laboratory data. Stepwise logistic regression was used to identify independent predictors of palliative care. Results Exclusive palliative care was indicated in 152 (27%) cases. In the palliative care group, in-hospital mortality and 12 month cumulative mortality amounted to 50% and 66%, respectively. Major conditions prompting referral to palliative care were advanced dementia (45%), cancer (38%), congestive heart failure (25%), stage IV and V renal dysfunction (24%), chronic obstructive pulmonary disease (8%) and cirrhosis (4%). Major complications observed in the palliative care group included delirium (p<0.001), infections (p<0.001) and pressure ulcers (p<0.001). Following multivariate analysis, male sex (OR=2.12; 95%CI: 1.32-3.40), cancer (OR=7.36; 95%CI: 4.26-13.03), advanced dementia (OR=12.6; 95%CI: 7.5-21.2), and albumin levels (OR=0.25; 95%CI: 0.17-0.38) were identified as independent predictors of referral to exclusive palliative care. Conclusion Advanced dementia and cancer were the major clinical conditions associated with referral of hospitalized older adults to exclusive palliative care. High short-term mortality suggests prognosis should be better assessed and discussed with patients and families in primary care settings.

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Echocardiographic Probability of Pulmonary Hypertension in Myeloproliferative Neoplasms Patients in Chiang-Mai University

Blood ◽

10.1182/blood-2019-126599 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 5390-5390

Author(s):

Yanika Jindamai ◽

Ekarat Rattarittamrong ◽

Arintaya Phrommintikul ◽

Lalita Yongsmith ◽

Thanawat Rattanathammethee ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Conflicts Of Interest ◽

Task Force ◽

Myeloproliferative Neoplasms ◽

White Blood Cells ◽

Philadelphia Chromosome ◽

University Hospital ◽

Chiang Mai ◽

European Respiratory Society ◽

Number Of Patients

Introduction: Pulmonary hypertension (PHT) is an emerging complication of myeloproliferative neoplasms (MPNs). The aim of this study was to determine the prevalence and risk factors of high echocardiographic probability of PHT according to 2015 The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) Guideline. The echocardiographic probability of PHT was assessed by peak tricuspid regurgitation velocity (TRV max) and echocardiographic signs suggesting PHT. Methods: This was a cross-sectional study conducted in Chiang-Mai University Hospital during January 2019 and July 2019. Patients aged 18 years or older with Philadelphia chromosome negative MPNs including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF) were eligible after obtaining informed consents. Patients who were known to have a preexisting PHT from any causes were excluded. All enrolled patients had an echocardiography performed by a cardiologist for evaluating the probability of PHT according to 2015 ESC/ERS guideline. Primary outcome of the study was the prevalence of MPNs patients who had high echocardiographic probability of PHT. Secondary outcomes were risk factor of high echocardiographic probability of PHT. Results: A total of 23 patients with a median age of 64 years (range 45-87) were enrolled. There were 14 PV (60.9%), 8 ET (34.8%), 1 MF (4.3%) patients included. There were13 male patients (56.5%). The most common driver mutation was JAK2V617F mutation (19 patients, 82.6%). Common co-morbid diseases were hypertension (26.1%), combined hypertension and dyslipidemia (13%), and combined hypertension and diabetes (8.7%), respectively. The majority of patients (73.9%) did not have history of thrombosis. However, 57.2% and 62.5% of PV and ET patients were classified as high risk of thrombosis. Twenty two patients (95.6%) received both antiplatelets and cytoreductive agents with 9 patients (39.1%) also underwent phlebotomy. Hydroxyurea was only cytoreductive drug prescribed in this study. The median time from diagnosis to echocardiogram evaluation was 66 months (range 9-6,242 months). Median (range) hemoglobin was 11.7 g/dl (9.7-16.8), median white blood cells count was 7.5x109/L (4.0-24.1), and median platelet count was 374x109/L (171-931). No patient with high echocardiographic probability of PHT was detected. One patient (4.3%) patient had intermediate probability and 22 (95.7%) patients had low probability. The median value of TRV max was 2.42 m/s (range 1.93-2.90). Conclusions: No MPNs patients (0/23) with high echocardiographic probability of PHT detected in this study. Further study with higher number of patients is warrant for determine the prevalence of PHT in Thai MPN patients. Table Disclosures No relevant conflicts of interest to declare.

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Risk Factors for Pulmonary Hypertension In Patients with Thalassemia Intermedia

Blood ◽

10.1182/blood.v116.21.2069.2069 ◽

2010 ◽

Vol 116 (21) ◽

pp. 2069-2069

Author(s):

Mehran Karimi ◽

Khaled M Musallam ◽

Maria Domenica Cappellini ◽

Amal El-Beshlawy ◽

Khawla Belhoul ◽

...

Keyword(s):

Pulmonary Hypertension ◽

United Arab Emirates ◽

Conflicts Of Interest ◽

Previous History ◽

Multivariate Logistic Regression Analysis ◽

Iron Chelation ◽

Thalassemia Intermedia ◽

Group I ◽

History Of ◽

Group Ii

Abstract Abstract 2069 Introduction: Mechanisms leading to pulmonary hypertension (PHT) in patients with thalassemia intermedia (TI) are still controversial. Moreover, clinical and laboratory characteristics of patients who eventually develop PHT have not been identified. Our aim was to identify factors that characterize TI patients who develop PHT. Methods: Data was retrospectively retrieved from the Thalassemia Intermedia Registry, a database of 584 TI patients currently registered at six comprehensive care centers in Lebanon, Italy, Iran, Egypt, United Arab Emirates, and Oman. Institutional review boards at each center approved the study protocol. Two Groups of patients were identified: Group I, TI patients with documented PHT (n=64; mean age 37.3 ± 10.6; 44% males); and Group II, age- and sex-matched TI patients without PHT (n=64; mean age 37.9 ± 11.4; 44% males). Collected data included demographics, laboratory parameters, disease-complications, and received treatments that may influence PHT development and reflected the period prior to PHT occurrence. Results: There were no statistically significant differences in mean platelet counts, total or fetal hemoglobin levels between the two Groups. However, mean serum ferritin level was higher in Group I compared to Group II (1233.2 ± 499.2 vs. 654.7 ± 234.5 ng/ml; P=0.01). Moreover, mean nucleated red blood cell (NRBC) count was higher in Group I compared to Group II (354.2 ± 199.2 vs. 214.7 ± 94.5 ×10⋀6/l; P=0.03). A higher proportion of patients were splenectomized (84.4% vs. 46.9%; P<0.001) or had a previous history of thromboembolic events (40.6% vs. 7.8%; P<0.001) in Group I compared to Group II. Conversely, a higher proportion of patients received transfusion (78.1% vs. 56.2%; P<0.001), iron chelation (62.5% vs. 37.5%; P<0.001), or hydroxycarbamide (34.4% vs. 17.2%; P<0.001) therapy in Group II compared to Group I. There were no statistically significant differences in the proportion of patients with heart failure, prothrombotic mutations, or receiving antiplatelet or anticoagulant therapy between the two Groups. Multivariate logistic regression analysis revealed that patients in Group I are more likely to be splenectomized (OR:4.9, 95%CI:1.9-8.5); transfusion-naive (OR:3.5, 95%CI:2.1-6.25); on no hydroxycarbamide (OR:2.6, 95%CI:1.1-5.25) or iron chelation therapy (OR:2.3, 95%CI:1.2-4.25); and have NRBC count >300 ×10⋀6/l (OR:2.59, 95%CI:1.69-6.05) or a previous history of thromboembolism (OR:3.69, 95%CI:2.38-7.05). Conclusion: Splenectomy, previous history of thromboembolism, and a high NRBC count characterize TI patients who develop PHT. Transfusion, iron chelation, and hydroxycarbamide therapy deserve evaluation for a protective role against PHT in TI. Disclosures: No relevant conflicts of interest to declare.

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Features Associated With Pulmonary Hypertension In Splenectomized Patients With Hemoglobin E/β-Thalassemia Disease

Blood ◽

10.1182/blood.v122.21.3457.3457 ◽

2013 ◽

Vol 122 (21) ◽

pp. 3457-3457

Author(s):

Suporn Chuncharunee ◽

Vichai Atichartakarn ◽

Umaporn Udomsubpayakul ◽

Napaporn Archararit

Keyword(s):

Pulmonary Hypertension ◽

Conflicts Of Interest ◽

Laboratory Data ◽

Systolic Pressure ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Pulmonary Artery Systolic Pressure ◽

Serum Lactate Dehydrogenase ◽

Ventricular Ejection Fraction ◽

Hemoglobin E

Abstract Introduction Pulmonary hypertension (PHT) associated with thalassemia hemoglobinopathy is now an accepted clinical entity. Due to a high prevalence of thalassemia hemoglobinpathy worldwide, it is the most common entity of PHT. Despite the commonness, its pathogenesis is not yet completely understood. Although asplenia is a known risk factor, PHT does not develop in all splenectomized patients. The present study was therefore done to search for other associated features. Patients and Methods Sixty-one clinically stable splenectomized hemoglobin E/β-thalassemia disease (E/β-Thal) adult outpatients, on no medication aside from folic acid and who received no blood transfusion in the preceding 4 weeks, were prospectively studied. All gave written informed consent, and study protocol was approved by the institution ethics committee on studies in humans (#0774/2548). Transthoracic echocardiogram was used to evaluate cardiac function and to estimate pulmonary artery systolic pressure (PASP). PHT was defined as an estimated PASP ≥36 mmHg. Clinical features and laboratory data were dichotomized according to the presence (PHT+) or absence (PHT-) of PHT, and statistical analysis was done by STATA version 10 (Stata Corp, Texas), considering a P value<0.05 as statistically significant. Results Of the 61 patients, 32 (52.5%) were PHT+, 14 of whom were female. There was no gender difference between the 2 groups (p=0.246). All had normal left ventricular ejection fraction, and none had positive serology for human immunodeficiency virus. Results expressed in mean ± SD or median (range) of the various measured parameters and their statistically significant differences are shown in the following table. Conclusions PHT was found in 52.5% of the 61 splenectomized E/β-Thal patients with no gender preponderance. Although PHT was found mostly after the first decade of splenectomy, its prevalence did not correlate with time elapsed since the procedure. Features associated with PHT in splenectomized E/β-Thal patients were greater transfusion requirement, lower RBC counts, higher serum lactate dehydrogenase (LDH) and lower serum cholesterol levels, as a reflection of more severe disease and more severe ineffective erythropoiesis; together with a higher serum soluble vascular cell adhesion molecule-1 (VCAM-1) levels, likely from chronic hypoxemia. Disclosures: No relevant conflicts of interest to declare.

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Management of PAH in Adults with Congenital Heart Disease: Impact and Dilemmas

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-11.4.198 ◽

2013 ◽

Vol 11 (4) ◽

pp. 198-206

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Congenital Heart ◽

Adult Congenital Heart Disease ◽

University Hospital ◽

Children's Hospital ◽

Children’S Hospital ◽

Adult Congenital ◽

The Ohio State University

Guest editor Richard Krasuski, MD, convened a group of experts by telephone on January 17, 2013, to discuss current trends in diagnosis and treatment of pulmonary hypertension among patients with congenital heart disease. Joining the call were Professor Maurice Beghetti, Head of Pediatric Subspecialties, Division Head of Pediatric Cardiology Unit, Children's University Hospital, Geneva, Switzerland; Curt Daniels, MD, Director, Adolescent and Adult Congenital Heart Disease Program Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio; Wayne J. Franklin, MD, Texas Children's Hospital, Houston; and Michael J. Landzberg, MD, Associate Director, Adult Pulmonary Hypertension Program and Director, Boston Adult Congenital Heart Program, Boston Children's Hospital.

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Deep Tracheal Extubation Using Dexmedetomidine in Children With Congenital Heart Disease Undergoing Cardiac Catheterization: Advantages and Complications

Seminars in Cardiothoracic and Vascular Anesthesia ◽

10.1177/1089253219870628 ◽

2019 ◽

Vol 23 (4) ◽

pp. 387-392 ◽

Cited By ~ 1

Author(s):

Nischal K. Gautam ◽

Kayla Bober ◽

James A. Pierre ◽

Olga Pawelek ◽

Evelyn Griffin

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Cardiac Catheterization ◽

Odds Ratio ◽

Congenital Heart ◽

Noninvasive Positive Pressure Ventilation ◽

Tracheal Extubation ◽

Postoperative Recovery ◽

Positive Pressure

Objective. Deep tracheal extubation using dexmedetomidine is safe and provides smooth recovery in children with congenital heart disease undergoing cardiac catheterization. Design. Single-institution, retrospective study of prospectively collected data. Participants. All patients aged between 1 month and 5 years who underwent general endotracheal anesthesia for diagnostic and interventional cardiac catheterizations in the cardiac catheterization suite from January 2015 (change in standard operating procedure) through October 2016 (approval of institutional review board for study). Measurement and Main Results. One hundred and eighty-nine patients (81%) of the 232 patients who underwent cardiac catheterization during the study period were noted to undergo deep tracheal extubation. Cyanotic heart disease was present in 87 patients (46%), history of prematurity in 51 (27%), and pulmonary hypertension in 26 (14%) patients. A documented smooth recovery in the postoperative care unit (PACU) requiring no additional analgesics or sedatives was observed in 91% of the patients. The majority of patients required no airway support after deep extubation (n = 140, 74%, P = .136). The presence of pulmonary hypertension (odds ratio = 4.45, P = .035) and presence of a cough on the day of the procedure (odds ratio = 7.10, P = .03) were significantly associated with the use of oxygen or use of oral airway for greater than 20 minutes in the PACU. After extubation, there were no reported events of aspiration, the use of noninvasive positive pressure ventilation, reintubation, heart block, or systemic hypotension requiring treatment or cardiac arrest. Conclusions. Deep extubation using dexmedetomidine in infants and toddlers after cardiac catheterization is feasible and enables smooth postoperative recovery with minimal adverse effects.

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Sharing of Worldwide Spread Knowledge Using Hypermedia Facilities & Fast Communication Protocols (Mosaic and World Wide Web): The Example of ExPASy

Methods of Information in Medicine ◽

10.1055/s-0038-1634588 ◽

1995 ◽

Vol 34 (01/02) ◽

pp. 75-78 ◽

Cited By ~ 5

Author(s):

R. D. Appel ◽

O. Golaz ◽

Ch. Pasquali ◽

J.-C. Sanchez ◽

A. Bairoch ◽

...

Keyword(s):

World Wide Web ◽

World Wide ◽

Relevant Literature ◽

Communication Protocols ◽

Laboratory Data ◽

Central Element ◽

University Hospital ◽

New Information ◽

The University ◽

Link Information

Abstract:The sharing of knowledge worldwide using hypermedia facilities and fast communication protocols (i.e., Mosaic and World Wide Web) provides a growth capacity with tremendous versatility and efficacy. The example of ExPASy, a molecular biology server developed at the University Hospital of Geneva, is striking. ExPASy provides hypermedia facilities to browse through several up-to-date biological and medical databases around the world and to link information from protein maps to genome information and diseases. Its extensive access is open through World Wide Web. Its concept could be extended to patient data including texts, laboratory data, relevant literature findings, sounds, images and movies. A new hypermedia culture is spreading very rapidly where the international fast transmission of documents is the central element. It is part of the emerging new “information society”.

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