scholarly journals Prevalence of Pulmonary Hypertension in Sickle Cell Anemiai Patient in KSA

Author(s):  
Eman AbdulAziz Balbaid ◽  
Manal abdulaziz Murad ◽  
Hoda Jehad Abousada ◽  
Abdurrahman Yousuf Banjar ◽  
Mashael Abdulghani Taj ◽  
...  

Introduction: Pulmonary hypertension (PH) is a relatively common and severe complication of SCI and an independent risk factor for mortality. Sickle cell disease is considered one of the most common diseases in the Kingdom of Saudi Arabia. When a healthy disease related to cardiovascular health is highlighted, sickle cell anemia may be the most common and related disease in high pulmonary pressure. In this study, we aimed to determine prevalence of PHTN in SCA patient, and associated risk factors with it.   Methodology: This is an analytical cross-sectional study conducted in kingdom of Saudi Arabia (General population, SCA patient and CVD patient), from 29/7/2020 till 15/11/2020. The study was depending on online self-reported questionnaire that included assessing the demographic factors as gender, nationality besides, disease-related information:  SCA patient , CVD patient and DM patient. Results: we received 794 responses to our questionnaire where 93.5% of them were Saudi Arabian.  The prevalence of sickle cell anemia is 8.8%. Male represented 29.8% of patients while female represented 52.2% of patients. In SCA patients, the prevalence of PHTN was 31.8%. Moreover, it was found that having cardiac disease is considered a risk factor for developing PHTN where 37.7% of patients having cardiac disease had PHTN compared with 6.2% of health patients (OD: 9.16, 95% CI: 5.5479 to 15.13, P=0.000) while diabetes mellitus increase risk for developing PHTN by more than seven fold (OD: 7.6, 95% CI; 4.7175 to 12.4, P=0.000) and disorder of nervous system by 12 folds (OD: 12.7; 95% CI: 7.6658 to 21.09, P=0.000). Conclusion: we had found that the prevalence if SCA among Saudi Arabia is 8.8% with a higher prevalence in female than male. Moreover, the prevalence of PHTN in SCA patients was high about 31.8% which is much higher than its prevalence in normal individuals. Moreover, it was found that having cardiac disease is considered a risk factor for developing PHTN besides, having diabetic condition and disorder of nervous system which increased risk for developing PHTN in SCA by nine, seven and 12-fold respectively.

2020 ◽  
Vol 8 (12) ◽  
pp. 1183-1188
Author(s):  
Eman Abdulaziz Balbaid ◽  
◽  
Manal Abdulaziz Murad ◽  
Hoda Jehad Abousada ◽  
Nada Faiez Alshanbari ◽  
...  

Introduction: Pulmonary hypertension (PH) is a relatively common and severe complication of SCI and an independent risk factor for mortality. Sickle cell disease is considered one of the most common diseases in the Kingdom of Saudi Arabia. When a healthy disease related to cardiovascular health is highlighted, sickle cell anemia may be the most common and related disease in high pulmonary pressure. In this study, we aimed in this study to determine prevalence of PHTN in SCA patient, and associated risk factors with it. Methodology: This is an analytical cross-sectional study conducted in kingdom of Saudi Arabia (General population, SCA patient and CVD patient), from 29/7/2020 till 15/11/2020. The study was depending on online self-reported questionnaire that included assessing the demographic factors as gender, nationality besides, disease-related information: SCA patient , CVD patient and DM patient. Results: we received 794 responses to our questionnaire where 93.5 % of them were Saudi Arabian. The prevalence of sickle cell anemia is 8.8 %. Male represented 29.8 % of patients while female represented 52.2 % of patients. In SCA patients, the prevalence of PHTN was 31.8 %. Moreover, it was found that having cardiac disease is considered a risk factor for developing PHTN where 37.7 % of patients having cardiac disease had PHTN compared with 6.2 % of health patients (OD: 9.16, 95 % CI: 5.5479 to 15.13, P=0.000) while diabetes mellitus increase risk for developing PHTN by more than seven fold (OD: 7.6, 95 % CI 4.7175 to 12.4, P=0.000) and disorder of nervous system by 12 folds (OD: 12.7 95 % CI: 7.6658 to 21.09, P=0.000). Conclusion: we had found that the prevalence if SCA among Saudi Arabia is 8.8 % with a higher prevalence in female than male. Moreover, the prevalence of PHTN in SCA patients was high about 31.8 % which is much higher than its prevalence in normal individuals. Moreover, it was found that having cardiac disease is considered a risk factor for developing PHTN besides, having diabetic condition and disorder of nervous system which increased risk for developing PHTN in SCA by nine, seven and 12-fold respectively.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 787-787 ◽  
Author(s):  
James G. Taylor ◽  
Vikki G. Nolan ◽  
Gregory J. Kato ◽  
Mark Gladwin ◽  
Martin H. Steinberg

Abstract Polymerization of hemoglobin in sickle cell anemia (HbSS) results in red blood cell damage, vasoocclusive disease and hemolytic anemia. Plasma hemoglobin and arginase released during hemolysis produce endothelial dysfunction and lead to vascular complications such as leg ulceration, priapism, pulmonary hypertension and increased risk of death. Examination of 2 independent patient groups from the NIH and CSSCD revealed considerable heterogeneity in the rates of hemolysis as estimated by LDH. We therefore hypothesized that inter-individual variation in hemolytic rate influences the phenotypic expression of HbSS. To test this hypothesis, we have defined a hemolysis phenotype as a clinical variable in our 2 HbSS groups. At NIH, LDH was analyzed as a quantitative trait in 166 adults to define a subgroup above the 75th percentile suggestive of exaggerated hemolysis (n= 42, mean LDH 645). When compared with 42 cases in the lowest LDH quartile (mean 230), the hyperhemolysis group had significantly lower hemoglobin and higher AST, bilirubin, and absolute reticulocyte levels supporting this phenotypic classification. HbF, arginine:ornithine ratios and arginase 1 activity were also significantly different. Clinically, the hyperhemolysis group had an increased prevalence of leg ulcers (OR 5.10; P=0.007), priapism (OR 3.67; P=0.16), and pulmonary hypertension (TRV > 3.0 m/s; OR 12.00; P<0.001). In addition, these patients have fewer severe vasoocclusive pain crises (3.1 ER visits/yr. with high LDH vs. 9.1/yr. with low LDH; P=0.004), supported by a lower prescription rate for hydroxyurea (37% with high LDH vs. 61% with low LDH; P=0.06). To validate these findings, we repeated the LDH phenotypic assignment analysis using 451 comparable CSSCD adults greater than 30 years of age. An identical pattern of laboratory results was seen when comparing high and low LDH quartiles. Hyperhemolysis was defined by anemia, elevated total bilirubin and AST and lower HbF. The most striking observations for this group were the significantly higher proportion of males (55% vs. 27% with low LDH; P<0.001), occurrence of leg ulcers (OR 3.44; P<0.001), and frequent death (33% vs. 16% with low LDH; P=0.003). There were also trends towards more prevalent priapism and stroke in the hemolysis group. After adjustment for HbF and gender, the associations between hyperhemolysis and risk of leg ulceration and risk of death remained significant in the CSSCD. Together, these findings suggest that excessive hemolysis results in a vascular HbSS phenotype characterized by leg ulcers, priapism, pulmonary hypertension, possibly stroke, and death. The lack of an association between excess hemolysis and pain/ACS suggests this phenotype is distinct from vasoocclusion/hyperviscosity. The consistent association of hyperhemolysis with this spectrum of symptoms, even after HbF correction, indicates the existence of unknown determinants for hemolysis. Future environmental and genetic studies of hyperhemolysis may provide novel mechanistic insights into the pathogenesis and treatment of HbSS vascular complications. From a clinical standpoint, these data further support the use of LDH as a simple biomarker for identifying a sub-set of HbSS patients at high risk for hemolysis-driven complications and death, who may otherwise be overlooked by clinicians because of infrequent vasoocclusive pain events.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1003-1003
Author(s):  
Benjamin Vazquez ◽  
Santosh L. Saraf ◽  
Robert E. Molokie ◽  
Michel Gowhari ◽  
Johara Hassan ◽  
...  

Abstract Hyperfiltration, which has been implicated in the early stages of development of chronic kidney disease (CKD) in diabetes mellitus, is a common finding in sickle cell anemia (SCA) patients with normal kidney function. We hypothesized that it may be a risk factor for the development of microalbuminuria, an early indicator of glomerular injury that is observed in 40-60% of adult patients with sickle cell anemia (SCA). In the cross-sectional analysis, 233 adult SCA patients treated at the University of Illinois at Chicago Sickle Cell Center with baseline estimated glomerular filtration rates (eGFR) determined by the MDRD equation and albuminuria concentrations were screened. Data was collected from steady-state visits defined as four weeks from a vaso-occlusive pain episode or red blood cell transfusion. Eight-two patients with normoalbuminuria (urine albumin concentration < 30mg/g creatinine) and an eGFR ≥ 90mL/min/1.73m2 were evaluated of which 38 (46%) had normal eGFR (defined as eGFR 90-150mL/min/1.73m2) and 44 (54%) had hyperfiltration (defined as eGFR >150mL/min/1.73 m2). On univariate analysis, the median age of patients with hyperfiltration was 23 years compared to 34 years in patients with a normal eGFR (p<0.0001). Additionally, patients with hyperfiltration had lower hemoglobin F concentrations (3.9 vs. 7.3%, p=0.01) and higher absolute reticulocyte counts (390 vs. 288 x103/µL, p=0.03) than patients with normal eGFR, respectively. On multivariate analysis, hyperfiltration was independently associated with age (OR 0.9, 95% CI: 0.8-0.9; p=0.0001) and the natural log of hemoglobin F concentration (OR 0.5, 95% CI: 0.2-1.0; p=0.04). Forty-six patients with normoalbuminuria and eGFR ≥ 90mL/min/1.73m2 were followed longitudinally for progression to microalbuminuria. At a median follow up of 30 months (range 1-81 months), 35% (16/46) of patients developed microalbuminuria with a mean time to progression of 49 months (range 11-57 months). The average time to develop microalbuminuria in patients with hyperfiltration was 33 months (42%, 11/26) compared to 62 months in patients with normal eGFR (25%, 5/20) (age-adjusted HR 4.0, 95% CI 1.5 – 10.4; log-rank p=0.016) (Figure 1). In summary, hyperfiltration in SCA patients with normoalbuminuria was associated with younger age and lower hemoglobin F concentration on cross sectional analysis and with higher rates of progression to microalbuminuria on longitudinal analysis. Future research to better understand the development of hyperfiltration and its contribution to kidney damage may help guide future therapies for sickle cell nephropathy. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1671-1671 ◽  
Author(s):  
Maxwell Westerman ◽  
Arnold Pizzey ◽  
Jocelyn Hirschman ◽  
Mario Cerino ◽  
Ada Eze ◽  
...  

Abstract Plasma hemoglobin (Hb) is a scavenger of nitric oxide (NO), which is a likely contributor to the pathogenesis and treatment of clinical abnormalities such as pulmonary hypertension and vasocclusive episodes in sickle cell anemia (SCA) and possibly in thalassemia syndromes, where pulmonary hypertension has been described most frequently in splenectomised patients with thalassemia intermedia (TI). Since plasma Hb consists of both free Hb and RBC-derived microvesicle Hb (Greenwalt. Vox Sang1991;61:14), the relationship between plasma Hb and circulating vesicles may be significant. We have measured plasma Hb and plasma vesicle levels in adults with SCA and thalassemia intermedia (TI). Patients with SCA were all untransfused and TI patients had no transfusions during the previous 3 months. Plasma Hb values in healthy adult controls (1.77±0.2, n=7) were significantly lower than in SCA (11.21±2.08mg/dl, n=15, p=0.003) or in splenectomised TI patients (48.46 ±3.66mg/dl, n=5, p=0.0038). Plasma Hb levels were significantly greater in TI as compared to levels in SCA (p=0.001). Vesicle numbers in SCA were 12.59±3.65 x103/ul (n=21, p< 0.001, SCA v control) and in TI were 24.19 ±12.23 (n=7, p< 0.001, TI v control). Thus both plasma Hb and circulating vesicle levels in SCA and TI were significantly greater than healthy controls. Plasma Hb was significantly greater in TI than in SCA and circulating vesicles markedly greater in TI than in SCA. Furthermore there was a significant correlation between plasma Hb and vesicle numbers in SCA, TI and normal controls (n=26, R2=0.59, p=0.0015). An analysis of splenectomised versus non-splenectomised TI patients revealed a further trend; both plasma Hb and vesicle numbers were significantly higher in splenectomised (n=5, n=7 respectively) than in non-splenectomised patients (n=4, n=4 respectively). Plasma Hb and vesicle numbers were respectively 48.5±3.6 and 24.19±12.2 in splenectomised patients compared to 17.18±5.58 (p=0.014) and 4.36±0.81 (p=0.008) in non-splenectomized TI patients. These findings show that raised plasma Hb levels are related to increments in vesicle numbers in TI and SCA. Splenectomy in TI, which is associated with increased risk of pulmonary hypertension and thrombosis, is associated with increased vesicle numbers and plasma Hb. We suggest that the scavenger characteristics of Hb containing vesicles for NO may differ from the scavenger characteristics of free Hb and thus require detailed study..


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 431-431
Author(s):  
James W. Sargent ◽  
Charles T. Quinn

Abstract Stroke is a serious complication of sickle cell anemia (SS). Steady-state hemoglobin (Hgb) desaturation, which is common in SS and often thought to be benign, could contribute to the risk of stroke because it perturbs endothelial function in SS (Lancet2003;362:1450) and might limit oxygen delivery to the brain. One previous study of SS patients found an association between nocturnal oxygen desaturation and strokes, transient ischemic attacks, and seizures (Lancet2001;357:1656). Nocturnal oxygen saturation is cumbersome to measure, and the association between daytime Hgb saturation and stroke has not been reported. We hypothesized that daytime steady-state Hgb desaturation was associated with and preceded overt stroke in children with SS. We performed a nested case-control study of the Dallas Newborn Cohort (Blood2004;103:4023). Cases were defined as cohort subjects with SS or sickle-β0-thalassemia (Sβ0) who had clinically overt ischemic strokes in the preceding 12 years. Controls were cohort subjects with SS or Sβ0 but without overt stroke who were reported previously (Br J Haematol2005;131:129). We collected mean steady-state oxygen saturations by pulse oximetry (SpO2) and mean steady-state hematologic data for cases (pre-stroke) and controls. For cases we also identified one SpO2 measurement and one blood count that was recorded closest to but preceding each stroke (the proximate pre-stroke values). We further calculated a predicted SpO2 for cases using a model that includes age, sex, and mean steady-state values of Hgb and reticulocyte count (Br J Haematol2005; 131:129) and calculated odds ratios for stroke by logistic regression. We identified 22 cases (100% SS; 68% male; mean age 7.9 years) and 390 controls (98% SS; 55% male; mean age 9.5 years). The steady-state SpO2 of cases (94.2%, standard deviation [SD] 3.0) was significantly lower than controls (96.3%, SD 3.0) (P=0.0014). The proximate pre-stroke SpO2 of cases (93.7% SD 3.8) was even lower than steady-state and significantly lower than controls (P=0.0006). The predicted steady-state SpO2 for cases was 95.2% (SD 1.4), meaning that the observed SpO2 of cases (94.2%) was lower than would be expected for SS patients without stroke given age, sex, and steady-state values of Hgb and reticulocyte count. The odds ratio for stroke was 1.32 (95% confidence interval 1.15 – 1.51) for each unit (1%) decrease in SpO2 while simultaneously controlling for age, sex, and steady-state values of Hgb and reticulocyte count (P<0.0001). In summary, we found that children with SS who develop a stroke have lower pre-stroke steady-state SpO2 values than children with SS who do not develop a stroke. Moreover, in children who develop stroke, the measured SpO2 value decreases as the time interval to stroke narrows. The odds of stroke increase as the SpO2 decreases. In conclusion, steady-state Hgb desaturation is a risk factor for ischemic stroke in children with SS. Decline in steady-state SpO2 over time further increases this risk. Hgb desaturation is an easily measured, potentially modifiable risk factor that could be used to identify children with SS who have an increased risk of overt ischemic stroke. Figure Figure


Author(s):  
Fatmah Alsharif ◽  
Wedad Almutairi ◽  
Faygah Shibily ◽  
Fatmah Alhothari ◽  
Fidaa Batwa ◽  
...  

Background: Lymphedema is a condition in which excessive fluid accumulates in soft tissues. It is a common complication of breast cancer treatments. It can lead to serious consequences and interfere with the activity of daily living. This study aimed to determine the level of awareness of breast-cancer-related lymphedema (BCRL) among women with breast cancer in the Kingdom of Saudi Arabia. This was a descriptive quantitative cross-sectional design that included a convenience sample of women diagnosed with breast cancer in the Kingdom of Saudi Arabia. Data were collected by distributing a self-administrated online questionnaire consisting of four parts, including demographic data (five items), the status of education about BCRL (three items), basic medical history of breast cancer (six items), and BCRL level of awareness of risk factors and management (nine items). Results: In total, 95 out of 135 of participants did not know about lymphedema, 119 of the participants (88.1%) did not receive any explanation about the possibility of lymphedema from their medical team before surgery, and 121 of them (89.6%) did not receive it after surgery. The most significant factor affecting participants’ level of awareness regarding BCRL was the lack of information about the possibility of BCRL occurrence, which was not provided to them by the medical team. Recommendation: Early and continuous education for future management is essential to prevent problems related to BCRL and improve quality of life.


Healthcare ◽  
2021 ◽  
Vol 9 (5) ◽  
pp. 592
Author(s):  
Shamoukh Alshahrani ◽  
Abrar Alshuaibi ◽  
Malak Alkhaldi ◽  
Pradeep Koppolu

Aim: The present study aims to evaluate the perception and awareness of interdental aids in different regions of Saudi Arabia. Methods: A cross-sectional questionnaire-based study was conducted, in order to evaluate the perception and knowledge of patients towards oral hygiene products among the population of the Kingdom of Saudi Arabia. In total, 812 out of 1124 participants responded and completed the survey (response rate 72.2%). The data were collected using a self-administered structured questionnaire in English and Arabic. All statistical analyses were carried out using the SPSS 20 software. p < 0.05 was used to indicate statistical significance. Results: A total of 812 participants responded, of which 486 participants (60%) declared using a toothpaste and toothbrush for cleaning their teeth. The cohort consisted of 274 (34%) females who brushed twice daily, while 96 (33%) males brushed their teeth once a day and 18% of the participants did not even brush once a day. The results indicated that 332 (64%) female participants and 174 (60%) male participants had perception and knowledge of the use of dental floss or any other device to clean between their teeth, while 174 (48.50%) male participants and 174 (49.10%) female participants cleaned their tongue with the same brush, rather than using a tongue scrubber or any other aids. Conclusion: The total awareness of interdental aids in Saudi Arabia is unsatisfactory, as demonstrated by the participants not being conscious or informed about the maintenance of their oral health. A majority of participants did not report adopting basic techniques, such as tongue brushing. This study reveals that no interdental aids were used by 16% of the participants. Thus, it is crucial to develop an effective educational program which emphasizes oral healthcare in this population.


2021 ◽  
Vol 10 (13) ◽  
pp. 2907
Author(s):  
Alba Martínez-Escudé ◽  
Guillem Pera ◽  
Anna Costa-Garrido ◽  
Lluís Rodríguez ◽  
Ingrid Arteaga ◽  
...  

Thyroid hormones may be a risk factor for the development of non-alcoholic fatty liver disease (NAFLD) and its progression to liver fibrosis. The aim of this study is to investigate the relationship between thyroid stimulating hormone (TSH) levels, NAFLD, and liver fibrosis in the general population. A descriptive cross-sectional study was performed in subjects aged 18–75 years randomly selected from primary care centers between 2012 and 2016. Each subject underwent clinical evaluation, physical examination, blood tests and transient elastography. Descriptive and multivariate logistic regression analyses were used to identify factors associated with NAFLD and fibrosis. We included 2452 subjects (54 ± 12 years; 61% female). Subjects with TSH ≥ 2.5 μIU/mL were significantly associated with obesity, atherogenic dyslipidemia, metabolic syndrome (MetS), hypertransaminasemia and altered cholesterol and triglycerides. The prevalence of NAFLD and liver fibrosis was significantly higher in subjects with TSH ≥ 2.5 (μIU/mL). We found a 1.5 times increased risk of NAFLD, 1.8 and 2.3 times increased risk of liver fibrosis for cut-off points of ≥ 8.0 kPa and ≥ 9.2 kPa, respectively, in subjects with TSH ≥ 2.5 μIU/mL compared with TSH < 2.5 μIU/mL (control group), independent of the presence of MetS. These findings remained significant when stratifying TSH, with values ≥ 10 μIU/mL.


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