Pulmonary artery pressure and the acute chest syndrome in homozygous sickle cell disease.

Denbow, C E; Chung, Edward; Serjeant, G. R.

doi:10.1136/hrt.69.6.536

Cited by 6 publications

(4 citation statements)

References 14 publications

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“…Sickle cell anemias are inherited disorders of hemoglobin synthesis that result from the substitution of glutamic acid at the sixth position of its β-globin chain by valine (HbS) 7 . The HbS gene is found in high frequency in many parts of Africa, India, the Middle East, the Mediterranean area, and aparts of Turkey 8 .…”

Section: Discussionmentioning

confidence: 99%

“…Nephropathy was defined as the presence of at least one of renal dysfunction as microalbuminuria and proteinuria, hyperecchogenity and/or thinning in renal cortex on ultrasonography, low creatinine clearance 4 . Pulmonary hypertension was defined as >25 mmHg mean resting pulmonary artery pressure or >30 mmHg following exercise and <15 mmHg pulmonary capillary pressure 7 . Avascular necrosis was defined as the presence of radiologically evident typical bone lesions 4 .…”

Section: Definitions Of Sickle Cell Disease Related Conditionsmentioning

confidence: 99%

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Türkiye’ de orak hücre hastalığına sahip hastalarda eritrosit alloimmünizasyonu: tek merkez geriye dönük kohort çalışması

Solmaz

Karacaoğlu

Gereklioğlu

et al. 2016

Cukurova Medical Journal (Çukurova Üniversitesi Tıp Fakültesi Dergisi)

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AbstractÖz Purpose: We aimed to investigate erythrocyte alloimmunization frequency and related factors in our region where SCD is common. Material and Methods: This study was planned as a single center, cross-sectional and retrospective cohort study. A total of 216 patients who had been followed up due to SCD [Hemoglobin (Hb) SS, Hb S-β thalassemia, Hb S-α thalassemia] were included in this study. Patients were divided to two groups according to amount of transfusion. The patients who had received less than 6 transfusions per year and who did not have the history of erythropheresis were allocated to Group 1, and the patients who had received 6 or more simple transfusion per year or who had undergone erythrocyte exchange were allocated to Group 2. Results: Of 216 SCD patients included in the study. Alloimmunization was detected in 67 (31.0%) out of 216 patients who underwent transfusion, and in 17 (30.4%) out of 56 patients in Group 1 and in 50 (31.3%) out of 160 patients in Group 2. When the patients were analyzed according to alloimmunization development, our study revealed that neither SCD complications are a risk factor for alloimmunization nor alloimmunization increases mortality rates. Conclusion: High alloimmunization frequency found in our study suggests the insufficient adherence of alloimmunization-prevention policies in RBC transfusions performed except experienced institutions. Therefore alloimmunization may be reduced or prevented through performing extended red cell typing among SCD patients.Amaç: Orak hücre hastalığının sık görüldüğü bölgemizde eritrosit alloimmmünizasyon sıklığını ve ilişkili durumları araştırmayı amaçladık. Gereç ve Yöntem: bu çalışma tek merkezli, zamansal kesitli ve geriye dönük kohort çalşıması olarak planlanmıştır.Toplamda 216 orak hücre hastalığı [Hemoglobin (Hb) SS, Hb S-β talasemi, Hb S-α talasemi] tanısı olan hasta çalışmaya dahil edilmiştir. Hastalar transfüzyon miktarına gore iki gruba ayrılmıştır. Yılda 6'dan daha az transfüzyon alan ya da eritroferez öyküsü olmayan hastalar Grup 1'e, yılda 6 ve daha fazla basit transfüzyon alan ya da eritroferez işlemine alınan hastalar Grup 2'ye dahil edilmiştir. Bulgular: Çalışmamıza 216 hasta dahil edilmiştir. Transfüzyon tedavisi alan toplam 216 hastanın 67 (%31.0)'sinde, Grup 1'deki 56 hastanın 17'sinde (%30.4), Grup 2'deki 160 hastanın 50'sinde (%31.3) tespit edilmiştir. Hastalar alloimmünizasyon gelişimi açısından analiz edildiğinde, çalışmamız ne orak hücre komplikasyonlarının alloimmünizasyon gelişimi için ne de alloimmünizasyonun ölüm için bir risk faktörü olmadığını göstermiştir. Sonuç:Çalışmamamızda bulunan yüksek alloimmünizasyon sıklığı, tecrübeli merkezler dışında yapılan transfüzyonlarda alloimmünizasyonu önleyici politikalara yeteri kadar uyulmadığı konusunda fikir vermektedir. Bu nedenle orak hücre anemili hastalarda alloimmünizasyon, ayrıntılı eritrosit antijen tanımlama işlemi yapılarak azaltılabilir veya önlenebilir.

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Section: Discussionmentioning

confidence: 99%

Section: Definitions Of Sickle Cell Disease Related Conditionsmentioning

confidence: 99%

Türkiye’ de orak hücre hastalığına sahip hastalarda eritrosit alloimmünizasyonu: tek merkez geriye dönük kohort çalışması

Solmaz

Karacaoğlu

Gereklioğlu

et al. 2016

Cukurova Medical Journal (Çukurova Üniversitesi Tıp Fakültesi Dergisi)

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“…Actually, Herrick did describe cardiac insufficiency in his patient, but in addition to his anemia, that patient had recurring multiple types of infections prevalent both in the American and Caribbean public of the time. Chest pain in patients with sickle-cell crises has attracted considerable attention [81][82][83] and has been variously attributed to pulmonary vascular lesions or to small infarctions within the bone marrow of the ribs or the sternum and still other areas of the thorax. Surprisingly, little attention has been aimed at the heart, although most episodes of chest pain in sickle-cell anemia occur during crises, and the painful crises have been demonstrated to be associated with documented disturbances in the electrical activity of the heart.…”

Section: James 32nd Herrick Lecturementioning

confidence: 99%

Homage to James B. Herrick: A Contemporary Look at Myocardial Infarction and at Sickle-Cell Heart Disease

James

2000

Circulation

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“…To the best of our knowledge, a limited number of data systems for assessing organ damage, determining patient workflow according to management plans, and ensuring data reliability are available. [ 6 , 9 ] Transfusion policies, which can reduce maternal and fetal losses, may vary among centers. [ 8 , 10 ] Allo-immunization continues to be problematic, despite the development of methods to detect erythrocyte antigens.…”

Section: Introductionmentioning

confidence: 99%

Organ damage mitigation with the Baskent Sickle Cell Medical Care Development Program (BASCARE)

Özdoğu

Boğa

Asma³

et al. 2018

Medicine

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The Eastern Mediterranean is among the regions where sickle cell disease (SCD) is common. The morbidity and mortality of this disease can be postponed to adulthood through therapies implemented in childhood. The present study focuses on the organ damage-reducing effects of the Baskent Sickle Cell Medical Care Development Program (BASCARE), which was developed by a team who lives in this region and has approximately 25 years of experience. The deliverables of the program included the development of an electronic health recording system (PRANA) and electronic vaccination system; the use of low citrate infusion in routine prophylactic automatic erythrocyte exchange (ARCE) programs including pregnant women; the use of leukocyte-filtered and irradiated blood for transfusion; the use of magnetic resonance imaging methods (T2∗) for the management of transfusion-related hemosiderosis; and the implementation of an allogeneic hematopoietic stem cell transplantation protocol for adult patients. The sample was composed of 376 study subjects and 249 control subjects. The hospital's Data Management System and the central population operating system were used for data collection. BASCARE enabled better analysis and interpretation of complication and mortality data. Vaccination rates against influenza and pneumococcal disease improved (21.5% vs 50.8% and 21.5% vs 49.2%, respectively). Effective and safe ARCE with low citrate infusion were maintained in 352 subjects (1003 procedures). Maternal and fetal mortality was prevented in 35 consecutive pregnant patients with ARCE. Chelating therapy rates reduced from 6.7% to 5%. Successful outcomes could be obtained in all 13 adult patients who underwent allogeneic peripheral stem cell transplantation from a fully matched, related donor. No patients died by day 100 or after the first year. Cure could be achieved without graft loss, grades III to IV acute graft versus host disease, extensive chronic graft versus host disease, or other major complications. The BASCARE program significantly improved patient care and thereby prolonged the life span of SCD patients (42 ± 13 years vs 29 ± 7 years, P < .001). We may recommend using such individualized programs in centers that provide health care for patients with SCD, in accordance with holistic approach due to the benign nature but malignant course of the disease.

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Pulmonary artery pressure and the acute chest syndrome in homozygous sickle cell disease.

Abstract: Conclusions-Repeated attacks of acute chest syndrome by the mean age of 12 (range eight to 16) years have not had a discernible effect upon pulmonary artery pressure. (Br Heart3J 1993;69:536-538)

Cited by 6 publications

References 14 publications

Türkiye’ de orak hücre hastalığına sahip hastalarda eritrosit alloimmünizasyonu: tek merkez geriye dönük kohort çalışması

Türkiye’ de orak hücre hastalığına sahip hastalarda eritrosit alloimmünizasyonu: tek merkez geriye dönük kohort çalışması

Homage to James B. Herrick: A Contemporary Look at Myocardial Infarction and at Sickle-Cell Heart Disease

Organ damage mitigation with the Baskent Sickle Cell Medical Care Development Program (BASCARE)

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