scholarly journals COVID-19 in Saudi Patients With Sickle Cell Disease: A Retrospective Multi-Center Study

Cureus ◽  
2021 ◽  
Author(s):  
Ohoud Kashari ◽  
Badriah Alghamdi ◽  
Abdulqader Al-Hebshi ◽  
Aljawharah Asiri ◽  
Ebtehal Fallatah ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Multi Center Study ◽  
Saudi Patients ◽  
Center Study

scholarly journals Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload

2007 ◽  
Vol 82 (4) ◽  
pp. 255-265 ◽  
Author(s):  
Ellen B. Fung ◽  
Paul Harmatz ◽  
Meredith Milet ◽  
Samir K. Ballas ◽  
Laura De Castro ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Morbidity And Mortality ◽  
Cell Disease ◽  
Multi Center Study ◽  
Center Study

Hospitalization Rate and Regional Differences in Comprehensive Care in Transfused Patients with Sickle Cell Disease Compared to Thalassemia: A Report from the Multi-Center Study of Iron Overload.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3189-3189
Author(s):  
Ellen B. Fung ◽  
Paul Harmatz ◽  
Meredith Milet ◽  
Samir K. Ballas ◽  
Laura DeCastro ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Liver Biopsy ◽  
Iron Overload ◽  
Sickle Cell ◽  
Comprehensive Care ◽  
Organ Injury ◽  
Standards Of Care ◽  
Cell Disease ◽  
Multi Center Study ◽  
Center Study

Abstract Red blood cell transfusions are often used to prevent the complications of thalassaemia (Thal) and sickle cell disease (SCD), though chronic therapy frequently results in iron overload-related organ injury. The Multi-Center Study of Iron Overload is a prospective, natural history study conducted to compare the comorbidities of iron overload in Thal with SCD. This report examines differences in morbidity and comprehensive care by disease and geographic region. 584 subjects aged 12 years and over, were screened for inclusion from 30 clinical hematological centers in the US, Canada and the UK. Socioeconomic and clinical data from subjects with iron overload (LIC of ≥ 10 mg/g dry wt by biopsy) and either Thal (N=142, 54% Male) or Tx-SCD (N=199, 43% Male) were collected. A group of non-iron overloaded SCD (NonTx-SCD N=64; 50% Male) were also enrolled. Tx-SCD were hospitalized more frequently (4.1± 3.8 times/year) compared to Thal (1.8± 1.7) or NonTx-SCD (2.1± 1.7; p<0.001) subjects, though average length of stay was similar (Tx-SCD: 7.0± 5.3 days; Thal: 5.7± 4.6; NonTx-SCD: 5.3± 3.8, p=0.16). Within the Tx-SCD group, adult subjects were 2.4 times more likely to be hospitalized in the previous 12 months compared to pediatric subjects (p=0.004, 95%CI 1.3–4.4). Roughly half of the SCD hospitalizations were for pain, whereas subjects with Thal were hospitalized for a variety of reasons, most often secondary to infection. Subjects with Thal were more likely to have routine iron assessments (LIC by biopsy: 66% vs. 37%), and screening for iron related organ injury such as thyroid function (TSH: 85% vs. 33%) and cardiac function (ECHO: 75% vs. 54%; EKG: 60% vs. 41%) compared to Tx-SCD (all p<0.001). Subjects with Thal had 3.4 times higher odds of having a recent liver biopsy compared to Tx-SCD (p<0.001; 95%CI: 2.2–5.3). Pediatric Tx-SCD subjects were more likely to have a biopsy compared to adults (46.5 vs. 23.5; p <0.001), however this likelihood was not related to serum ferritin level, duration of transfusion or chelation. Combining Thal and Tx-SCD groups, liver biopsy was more commonly performed at international vs. U.S. centers, (p<0.001), however the size of the clinical center was unrelated. Whereas, both regional and center size differences were observed in ECHO and TSH testing (p<0.01). A total of 26 subjects have died or exited from the study due to significant medical events. The mortality among adult Tx-SCD subjects was 2.8 fold greater than that of Thal (p=0.017). These data suggest that despite significant morbidity and iron burden, Tx-SCD subjects are monitored on a less frequent basis for iron related organ damage compared to Thal subjects. Differences were also observed in standards of care by region and center size. This disparity in care requires attention and guidelines for the assessment and management of Tx-SCD subjects should be developed and supported until the effect of iron overload in this population is better understood.

Limitations of Clinical Trials in Sickle Cell Disease: A Case Study of the Multi-center Study of Hydroxyurea (MSH) Trial and the Stroke Prevention (STOP) Trial

Hematology ◽  
2007 ◽  
Vol 2007 (1) ◽  
pp. 482-488 ◽  
Author(s):  
Michael R. DeBaun ◽  
Joshua J. Field
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Stop Trial ◽  
History Of ◽  
Secondary Analyses ◽  
Multi Center Study ◽  
Center Study

AbstractIn the past two decades, two landmark randomized controlled trials (RCT) have been completed among individuals with sickle cell disease (SCD), the Multi-center Study of Hydroxyurea (MSH) trial and the Stroke Prevention (STOP) trial. The MSH trial tested the hypothesis that hydroxyurea will reduce the frequency of painful episodes for adults with hemoglobin SS who had a history of 3 or more painful episodes per year. The STOP trial tested the hypothesis that among children with hemoglobin SS and an elevated transcranial Doppler (TCD) velocity measurement, blood transfusion therapy would decrease the risk of an initial stroke. After completion, both trials have defined standard care for individuals with hemoglobin SS. The purpose of this review is to examine the limitations of the MSH and STOP trials. In the context of these trials, we will examine the effects of narrow inclusion criteria that primarily include participants with hemoglobin SS and secondary analyses that are prone to false-positive results. In addition, we describe how after publication of these two trials use of hydroxyurea and TCD assessment has drifted towards a standard practice without evidence of therapeutic efficacy among groups that were excluded from the trials. Finally, we suggest that rigorously conducted RCTs or at the minimum multicenter observation studies with strong methodology should be performed in these excluded subgroups to confirm a benefit of hydroxyurea or TCD measurement.

Renal Involvement in Saudi Patients with Sickle Cell Disease. A Single Institution Experience.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3734-3734
Author(s):  
Hikmat N. Abdel-Razeq ◽  
Ali Bajouda ◽  
Manar Khalil ◽  
Muneera Al-Shareef ◽  
Nauman Siddiqi
Keyword(s):  
Sickle Cell Disease ◽  
Serum Creatinine ◽  
Sickle Cell ◽  
Urine Analysis ◽  
Renal Involvement ◽  
Urine Volume ◽  
Cell Disease ◽  
Study Results ◽  
Renal Tubular ◽  
Saudi Patients

Abstract Background: Renal involvement in patients with sickle cell disease has been reported to be as high as 20%. This can take the form of proteinuria, decreased GFR, isothenuria or hematuria. Our own clinical observation of patients attending our sickle cell clinic or admitted to the medical ward at our institution suggests lower rates of renal involvement than previously reported. In this report, we evaluate Saudi patients with sickle cell disease (SS) at our institution for renal involvement. Patients and Methods: Consecutive afebrile patients attending sickle cell clinic or admitted to inpatient wards were screened. Serum creatinine, urine analysis and 24 hr urine collection for creatinine clearance (GFR) and proteinuria were performed. Data on demographics and co-morbidities (DM, HTN, hepatitis B and C) and medications were obtained. Analysis was performed using SPSS 10.0®. Results: 62 patients were available for study; results are summarized in tables. Seven of 48 patients (15%) had proteinuria >300mg/d while two (4.2%) had proteinuria of more than 1.0 gram/24 hrs. Nearly 20% had GFR < 60ml/min. None of the patients had renal tubular acidosis. Serum creatinine was normal in all patients including those with low GFR and proteinuria. Conclusions: Contrary to our initial believes, significant percentage of Saudi sickle cell patients, like others, have renal involvement with low GFR and proteinuria. Serum Creatinine alone doesn’t indicate this decline in renal function. We recommend annual evaluation for proteinuria and GFR determination in all sickle cell patients, and those at risk should be aggressively managed with nephroprotective agents like angiotensin converting enzyme (ACE) inhibitors. Age (yrs) M:F BMI Hepatitis C Hydroxyurea NSAID 22.1+/−8.1 23:39 19.3+/−5.1 9 (17.3%) 29 (58%) 33 (66%) Urine Volume (ml/24 hrs) Specific Gravity Serum Creatinine (u mol/L) GFR Proteinuria (mg/24hrs) 1927 +/− 780 1.011 +/− 0.005 40.6 +/− 12.9 113.7 +/− 47.7 256 +/− 371 (20–2390)

Sonographic Assessment of Spleen Size in Saudi Patients with Sickle Cell Disease

1998 ◽  
Vol 18 (3) ◽  
pp. 217-220 ◽  
Author(s):  
Ahmed H. Al-Salem ◽  
Soror Al-Aithan ◽  
Prabhakar Bhamidipati ◽  
Ali Al Jam'a ◽  
Ibrahim Al Dabbous
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Spleen Size ◽  
Cell Disease ◽  
Sonographic Assessment ◽  
Saudi Patients

scholarly journals Clinical and Laboratory Predictors of Painful Vaso-Occlusive Crisis Among Sickle Cell Disease Patients: A Single-Center Study in Saudi Arabia

Cureus ◽  
2021 ◽  
Author(s):  
Ossama Zakaria ◽  
Rayan A Buhalim ◽  
Fahad Almulhim ◽  
Faisal A Al Jabr ◽  
Aqeel Alrashid ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Single Center ◽  
Cell Disease ◽  
Single Center Study ◽  
Center Study

scholarly journals Reasons for Hospitalization of Sickle Cell Disease Patients in the Eastern Province of Saudi Arabia: A Single-Center Study

Cureus ◽  
2021 ◽  
Author(s):  
Ossama M Zakaria ◽  
Rayan A Buhalim ◽  
Faisal A Al Jabr ◽  
Mohammed N AlSaeed ◽  
Ibrahim A Al-Hajji ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Single Center ◽  
Cell Disease ◽  
Eastern Province ◽  
Single Center Study ◽  
Center Study
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