Symptoma

Sickle Cell - Beta-Thalassemia

HbS-Beta-Thalassemia Syndrome

Sixty hours after presentation, he developed pain similar to that of sickle cell vaso-occlusion, for which he received small amounts of analgesics. He died approximately 84 hours after presentation. [ncbi.nlm.nih.gov]

The latest data on research and developments will be presented. [prnewswire.com]

Diagnosis [ edit ] Patient may present with symptomatic anemia or with sickle crises. [en.wikipedia.org]

  • Weight Gain

    In acutely ill children, synbiotics have been linked to greater weight gain and fewer bacterial illnesses after antibiotics are ended. The evidence consistently supports supplementation of antibiotics with probiotics. [goodearthnaturalfoods.com]

  • Severe Clinical Course

    One SB0 case with exceptionally high HbF (32 per cent) with severe clinical course was found. In this case, the high HbF did not ameliorate the clinical severity. Heterogeneity in each type of sickle cell/beta-thalassemia is discussed. [ncbi.nlm.nih.gov]

  • Regurgitation

    NT-proBNP levels correlated with measures of pulmonary artery systolic pressure (tricuspid regurgitant jet velocity and right ventricular systolic pressure), left atrial area and diastolic dysfunction. [ncbi.nlm.nih.gov]

  • Osteopenia

    Seventeen (32%) patients had osteopenia/osteoporosis and 30 (57%) had osteosclerosis. Bone resorption was diminished in patients with osteosclerosis and increased in those with osteopenia/osteoporosis. [haematologica.org]

Medical Workup: The initial laboratory test to screen for sickle cell disease/trait is a Sickledex ; if positive then hemoglobin electrophoresis is required. Hb AS, with Hb S up to 45 % is acceptable for flying duties. [asams.org]

  • Microalbuminuria

    Four had mild proteinuria, and six had microalbuminuria. Serum EPO and aldosterone were higher in S-beta thal patients than controls, but there was no difference in PRA between the two groups. [ncbi.nlm.nih.gov]

  • Hyperuricosuria

    One third of the S/b thai patients showed hyperuricosuria, and a statistically significant negative correlation was noted between serum uric acid and its fractional excretion in all S/b thai patients (n 41, r -0.450, p 0.01). [karger.com]

For a period of 52 months prior to starting treatment, the total score of severity for 59 evaluable patients was 1182 points (3068 patient-weeks), while for the 12,018 patient-weeks of the trial this parameter fell to only 82 points. [ncbi.nlm.nih.gov]

Beta Thalassemia Treatment & Management: Approach Considerations, Surgical Treatment 2015 In-text: (Advani, 2015) Your Bibliography: Advani, P. (2015). Beta Thalassemia Treatment & Management: Approach Considerations, Surgical Treatment. [citethisforme.com]

This could lead to a treatment for sickle cell disease and beta thalassemia, both diseases in need of safer and more effective treatments. The results were presented at the 54th annual meeting of the American Society of Hematology (ASH). [fiercebiotech.com]

Beta-plus cells, after treatment, produced hemoglobin comparable to a healthy individual, and were thus cured. [news-medical.net]

[…] edit ] Treatment is the same as for patients with sickle cell disease. [en.wikipedia.org]

It is associated with a poorer prognosis. Hemoglobin SD, hemoglobin SE, and hemoglobin SO These types of sickle cell disease are more rare and usually don’t have severe symptoms. [healthline.com]

However, in some cases the symptoms of beta-zero thalassemia are worse than those seen in homozygous sickle cell disease and is often associated with a poor prognosis. [hinapatelfoundation.org]

Prognosis As discussed above, the prognosis for individuals with the most serious types of thalassemia has improved drastically in the last several years following recent medical advances in transfusion, chemo-, and transplantation therapy. [medical-dictionary.thefreedictionary.com]

And the doctor briefs us regularly about this report and from this, we are hopeful that his prognosis is well. [healthtalk.org]

The relationship between these etiologic factors and indications for transfusions are discussed. [ncbi.nlm.nih.gov]

NCT00972231 15 Sickle Cell Anemia in an Arab Bedouin Village in the Northern Israel Completed NCT00481039 16 CD34 (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation Recruiting NCT01966367 Early Phase 1 17 Epidemiology [malacards.org]

References Modell B, Darlison M (2008) Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 86: 480-487. Fucharoen S, Weatherall DJ (2012) The Hemoglobin E Thalassemia’s. [omicsonline.org]

Author information Affiliations Non-Communicable Disease Program, Ministry of Health, Kingdom of Saudi Arabia Nasser AbdulRahman AlHamdan & Fahad Mohammad AlSwaidi Field Epidemiology Training Program, and Ministry of Health, Kingdom of Saudi Arabia Nasser [nature.com]

More studies are needed to better define the contribution of hemostatic activation to the pathophysiology of SCD and beta-thalassemia. [eurekaselect.com]

Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system. SCD is characterized by a remarkable phenotypic complexity. [natureasia.com]

[…] chain, which produces a hemoglobin tetramer (alpha2/beta S2) that is poorly soluble when deoxygenated. 3 The polymerization of deoxy hemoglobin ( Hb ) S is essential to vasoocclusive phenomena. 3 However, polymerization alone does not account for the pathophysiology [asams.org]

The use of hydroxyurea for the prevention of sickle cell crises in patients with homozygous HbS disease is now well established. [ncbi.nlm.nih.gov]

Early treatment helps to prevent serious and life threatening health problems such as: complicated infections organ damage stroke [newbornscreening.on.ca]

Literatur Angastiniotis MA, Hadjiminas MG (1981) Prevention of thalassaemia in Cyprus. Lancet: 369–371 Google Scholar Angastiniotis M, Modell B, Englezos P, Boulyjenkov V., Prevention and csontrol of maemoglobinopathies. [link.springer.com]

There are various treatment options to help prevent some of the symptoms and complications of sickle cell disease. [no-more-heart-disease.com]