scholarly journals Prevalence of Sickle Cell Anemic Subjects from Gadchiroli District, Maharashtra, India

2021 ◽  
Vol 9 (12) ◽  
pp. 1598-1602
Author(s):  
S. R. Kohchale
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Psychological Trauma ◽  
High Morbidity ◽  
Beta Globin ◽  
Cell Disease ◽  
Alpha Globin ◽  
Study District

Abstract: Sickle cell disease is caused by Mutations in the HBB gene. Hemoglobin consists of four protein subunits, typically, two subunits called alpha-globin and two subunits called beta-globin. People with sickle cell anemia suffers with high morbidity and with many intercurrent infections, people of study district are with high economic burden, terminate fatality in childhood state and have the emotional and psychological trauma including the family members the exact magnitude of the problem in the study district is still obscure. The study conducted from April 2009 to April 2012 to know the prevalence of sickle cell anemia by month long survey and by visiting all PHC’S and RH of district and data collected to know the prevalence of sickle cell trait and sickle cell disease total 7763 cases were recorded in present study and age wise, gender wise and caste wise distribution recorded and the data was analyzed statistically. Keywords: Sickle, anemia, Gadchiroli, Haemoglobin, beta-globin

PATHOGENESIS OF HYPOSTHENURIA IN PERSONS WITH SICKLE CELL ANEMIA OR THE SICKLE CELL TRAIT

PEDIATRICS ◽  
1960 ◽  
Vol 26 (2) ◽  
pp. 249-254
Author(s):  
L. Schlitt ◽  
H. G. Keitel
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Renal Damage ◽  
Sickle Cell Trait ◽  
Urinary Concentration ◽  
Cell Disease ◽  
Renal Vessels

Hyposthenuria was investigated in subjects with sickle cell trait and in patients with sickle cell anemia. The following were observed: 1) in subjects with sickle cell trait both normal and reduced maxima of urinary concentration are found, whereas all untreated patients with sickle cell anemia over 6 months of age have hyposthenuria; 2) hyposthenuria becomes increasingly more severe with advancing age in both sickle cell anemia and sickle cell trait; 3) in a 6-month-old patient with sickle cell anemia and hyposthenuria, the maxima of urinary concentration returned to normal after two transfusions of normal erythrocytes. Reasons are presented for favoring the hypothesis that hyposthenuria in sickle cell disease is due to renal damage, possibly from intravascular sickling of erythrocytes in renal vessels or from the presence of "free" circulating S-hemoglobin.

Sickle Cell Disease

2013 ◽  
Author(s):  
Alexandra Szabova ◽  
Kenneth R Goldschneider
Keyword(s):  
African Americans ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Anesthetic Management ◽  
Cell Disease

0.2% of African-Americans have sickle cell anemia while, with 8% to 10% have sickle cell trait. This chapter provides an overiew of the etiology, pathophysiology, and treatment of sickle cell anemia as they affect anesthetic management—before, during, and after surgery.

Sickle Cell Disease

2018 ◽  
Author(s):  
Ann Ng ◽  
Erin S. Williams
Keyword(s):  
African American ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Organ Damage ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Systemic Involvement ◽  
History Of

Sickle cell anemia (sickle cell disease) is a common hemoglobinopathy with anywhere from 90,000 to 100,000 Americans affected. This chronic condition has a predominance in populations of African descent, occurring in approximately 1 out of 365 African American births, compared to 1 out of 16,300 Hispanic births. The sickle cell trait can be detected in 1 of 13 African American births. One of the most common complications associated with sickle cell anemia, vaso-occlusive crises by sickled cells, results in severe pain. Other issues associated with this condition include acute chest syndrome, lung infections, end organ damage, and stroke. With improvements in the management and prevention of pain crises, infection, and other systemic involvement, these patients are living longer, thus increasing the potential for surgical needs. Whether it is for routine surgeries or surgeries that are due to the natural history of the disease; the pediatric anesthesiologist must be knowledgeable of the management of these patients in order to prevent morbidity and mortality.

scholarly journals Studies on Abnormal Hemoglobins

Blood ◽  
1955 ◽  
Vol 10 (5) ◽  
pp. 405-415 ◽  
Author(s):  
KARL SINGER ◽  
LILY SINGER ◽  
SEYMOUR R. GOLDBERG
Keyword(s):  
Sickle Cell Disease ◽  
Genetic Control ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Negro Population ◽  
Different Types ◽  
Adult Hemoglobin

Abstract (1) Four Negro patients with mild sickle cell-thalassemia disease (heterozygous for the genes for S hemoglobin and for thalassemia) are described. In contrast to reports in the literature, some of these patients are only mildly anemic, or not anemic at all. In three, the values for MCV and MCH are decreased, but in one, all hematologic indices are normal. All four individuals show leptocytosis and elevated reticulocyte levels. (2) Hemoglobin analyses, consisting of a combination of electrophoresis and the alkali denaturation technic, demonstrate the S + A + F pattern in three, and the S + A pattern in the fourth. These patterns are considered pathognomonic for sickle cell-thalassemia disease. They may be sharply differentiated from the S + F pattern, encountered in classical (homozygous) sickle cell anemia, and from the A + S pattern found in the heterozygous sickle cell trait. The various types of hemoglobin are reported in the sequence of their quantitative representation in the hemolysate. Hemoglobin analysis is indispensable for the recognition of the different types of sickle cell disease. (3) Evidence is cited that clinically almost asymptomatic sickle cell-thalassemia disease is probably not too rare in the American Negro population. (4) The genetic aspects of the production of fetal hemoglobin are discussed. It is postulated that the production of fetal hemoglobin is also under genetic control. The genes for fetal hemoglobin are not alleles of the genes for normal adult hemoglobin and are physiologically almost completely suppressed by the latter. Pathologic genes may render this suppression incomplete.

scholarly journals SICKLE CELL ANEMIA IN QUILOMBOLAS: A LITERATURE REVIEW

2018 ◽  
Vol 5 (1) ◽  
pp. 34
Author(s):  
Renan Pires Ferreira Borges ◽  
Rafael Felipe Carvalho Canutto ◽  
Kassita Alvarenga Gomes ◽  
Carlos Alberto Rodrigues Junior ◽  
Fellipe Camargo Ferreira Dias ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Structural Changes ◽  
Sickle Cell Trait ◽  
Cell Disease ◽  
Hematological Disorders ◽  
The North ◽  
Generic Term ◽  
Abnormal Hemoglobin

Introduction: Sickle cell disease is a generic term that encompasses a set of hematological disorders linked to structural changes in blood cells, leading to the production of an abnormal hemoglobin called HbS, the 'S' being derived from English sickle. The objective of this study was to discuss the profile of sickle cell anemia in quilombola communities by reviewing the existing literature in order to provide updated data to professionals dealing with this population. Development: The prevalence of HbS heterozygotes in Brazil is higher in the North and Northeast regions, between 6% and 10%, and considerably lower in the South and Southeast regions, with prevalence between 2% and 3%. In this study, it was found that 6.6% to 11.5% of the population of quilombolas in the state of Tocantins had abnormal hemoglobins. Conclusion: Few studies evaluate the prevalence of hemoglobinopathies in quilombola communities. Since quilombolas usually live in isolated communities of society, the chances of marriages between individuals with sickle cell trait are greater than in the general population, thus increasing the probability of being born to individuals affected by the disease. Keywords: Sickle cell disease, Quilombolas, Epidemiology. RESUMO Introdução: Doença falciforme é um termo genérico que engloba um conjunto de desordens hematológicas ligadas a alterações estruturais das células sanguíneas, levando à produção de uma hemoglobina anormal denominada HbS, sendo o ‘S’ derivado do inglês sickle. O objetivo deste estudo foi abordar o perfil da anemia falciforme nas comunidades quilombolas por meio de revisão da literatura existente, a fim de fornecer dados atualizados aos profissionais que lidam com essa população. Desenvolvimento: No Brasil, a prevalência de heterozigotos HbS é maior nas regiões do Norte e Nordeste, entre 6% e 10%, e consideravelmente menor nas regiões Sul e Sudeste, com uma prevalência entre 2% e 3%. Neste estudo, encontrou-se que 6,6% a 11,5% da população de quilombolas no estado do Tocantins apresentaram hemoglobinas anormais. Conclusão: Poucos estudos avaliam a prevalência de hemoglobinopatias em comunidades quilombolas. Visto que os quilombolas costumam viver em comunidades isoladas da sociedade, as chances de ocorrerem casamentos entre indivíduos com traço falciforme, tornam-se maiores que na população em geral, aumentando assim a probabilidade de nascerem indivíduos acometidos pela doença. Palavras-chave: Doença falciforme, Quilombolas, Epidemiologia.

Plasma lipids in Iranians with sickle cell disease: Hypocholesterolemia in sickle cell anemia and increase of HDL-cholesterol in sickle cell trait

2006 ◽  
Vol 365 (1-2) ◽  
pp. 217-220 ◽  
Author(s):  
Zohreh Rahimi ◽  
Ahmad Merat ◽  
Mansour Haghshenass ◽  
Hamid Madani ◽  
Mansour Rezaei ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Plasma Lipids ◽  
Sickle Cell Trait ◽  
Hdl Cholesterol ◽  
Cell Disease

GLOMERULAR FILTRATION RATE IN CHILDREN WITH SICKLE CELL DISEASE

PEDIATRICS ◽  
1950 ◽  
Vol 5 (1) ◽  
pp. 127-129
Author(s):  
PHILIP L. CALCAGNO ◽  
JOHN MCLAVY ◽  
THOMAS KELLEY
Keyword(s):  
Renal Function ◽  
Glomerular Filtration Rate ◽  
Sickle Cell Disease ◽  
Glomerular Filtration ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Filtration Rate ◽  
Sickle Cell Trait ◽  
Cell Disease

Renal function has been studied quantitatively in five patients with sickle cell anemia, all of whom were proved to have reduced glomerular filtration rates. Two patients with sickle cell trait revealed normal glomerular filtration rates. Possible mechanisms responsible for reduced glomerular filtration flows are discussed.

Microsurgery in the Sickle Cell Trait Population: Is It Actually Safe?

2020 ◽  
pp. 1-2
Author(s):  
Michael Alperovich ◽  
Eric Park ◽  
Michael Alperovich ◽  
Omar Allam ◽  
Paul Abraham
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Free Flap ◽  
Near Infrared ◽  
Sickle Cell Trait ◽  
Cell Disease ◽  
Flap Transfer ◽  
Free Flap Transfer ◽  
Patient Reported ◽  
Deep Inferior Epigastric Perforator

Although sickle cell disease has long been viewed as a contraindication to free flap transfer, little data exist evaluating complications of microsurgical procedures in the sickle cell trait patient. Reported is the case of a 55-year-old woman with sickle cell trait who underwent a deep inferior epigastric perforator (DIEP) microvascular free flap following mastectomy. The flap developed signs of venous congestion on postoperative day two but was found to have patent arterial and venous anastomoses upon exploration in the operating room. On near-infrared indocyanine green angiography, poor vascular flow was noted despite patent anastomoses and strong cutaneous arterial Doppler signals. Intrinsic microvascular compromise or sickling remains a risk in the sickle cell trait population as it does for the sickle cell disease population. Just like in sickle cell disease patients, special care should be taken to optimize anticoagulation and minimize ischemia-induced sickling for patients with sickle cell trait undergoing microsurgery.

scholarly journals Is there a role for selective vasodilation in the management of sickle cell disease?

Blood ◽  
1988 ◽  
Vol 71 (3) ◽  
pp. 597-602 ◽  
Author(s):  
GP Rodgers ◽  
MS Roy ◽  
CT Noguchi ◽  
AN Schechter
Keyword(s):  
Blood Flow ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Microvascular Obstruction ◽  
Controlled Study ◽  
Control Group ◽  
Cell Disease ◽  
Retinal Arteriolar

Abstract To test the hypothesis that microvascular obstruction to blood flow at the level of the arteriole may be significant in individuals with sickle cell anemia, the ophthalmologic effects of orally administered nifedipine were monitored in 11 steady-state patients. Three patients with evidence of acute peripheral retinal arteriolar occlusion displayed a prompt reperfusion of the involved segment. Two other patients showed fading of retroequatorial red retinal lesions. Color vision performance was improved in six of the nine patients tested. The majority of patients also demonstrated a significant decrease in the amount of blanching of the conjunctiva which reflects improved blood flow to this frequently involved area. Such improvements were not observable in a control group of untreated stable sickle cell subjects. These findings support the hypothesis that inappropriate vasoconstriction or frank vasospasm may be a significant factor in the pathogenesis of the microvascular lesions of sickle cell disease and, further, that selective microvascular entrapment inhibition may offer an additional strategy to the management of this disorder. We believe a larger, placebo-controlled study with nifedipine and similar agents is warranted.

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