MORTALITY AND MORBIDITY IN JAMAICAN ADULTS WITH SICKLE-CELL TRAIT AND WITH NORMAL HqMOGLOBIN FOLLOWED UP FOR TWELVE YEARS

The Lancet ◽  
1976 ◽  
Vol 308 (7989) ◽  
pp. 784-786 ◽  
Author(s):  
M ASHCROFT
Author(s):  
Janhavi Mahajan ◽  
Dhruv Talwar ◽  
Sunil Kumar ◽  
Sourya Acharya ◽  
Yogesh Kakde

ECAST Or exercise collapse associated with sickle cell trait is a rare phenomenon associated with sickle cell trait and is an important presentation of sickle cell disease in sports medicine. Collapse is seen following vigorous physical activity, which is due to excessive heat, dehydration and other factors associated with physical exercise. This rare syndrome is often missed by the treating physicians as a result of a lack of knowledge about this rare entity leading to massive underreporting. It is important to identify ECAST as a cause of the collapse in young athletes to prevent mortality and morbidity and in order to provide prompt treatment. We report a case of a 25- year-old young male who was a bodybuilder and reported to the gym after a one-year-long break due to lockdown restrictions of COVID19. After a vigorous exercise session, he collapsed in the gym and was brought to the emergency department. After proper history taking and examination, he was suspected to be a case of ECAST due to a history of a similar episode three years back which was treated as a case of exertional syncope with intravenous fluid therapy and a family history of Sickle cell trait with his mother and father both having sickle cell AS Pattern. Ultimately our patient turned out to be a case of Sickle Cell Trait with evidence of AS pattern on Hb electrophoresis and a small-sized spleen visualized on CT Scan of the abdomen. The patient was managed successfully with intravenous fluids and blood transfusion and was discharged in a stable condition. He was counseled about moderating his exercise and is doing well on follow-up.


Author(s):  
Obeagu Emmanuel Ifeanyi

Malaria is an endemic disease in the developing countries like Nigeria with high mortality and morbidity rates especially in children and pregnant women who are immune competent. A lot of measures have been taken to control malaria in this part of the world but is still major problem confronting person in the malaria endemic areas. Sickle cell trait has been shown to confer selective protective advantage to malaria on the persons possessing the hemoglobin genotype. This paper discussed the selective protective advantage of sickle cell trait to malaria.


1970 ◽  
Vol 9 (3) ◽  
pp. 154-160
Author(s):  
BP Preethi ◽  
K Monika ◽  
DS Maitreyee ◽  
K Rashmi

Background: Hereditary hemolytic anaemias constitute important cause of mortality and morbidity in developing countries next only to infection and malnutrition.These group of anaemias have various clinical presentations starting from their age of onset of symptoms, failure to thrive, anaemia, prostration, jaundice, splenomegaly, cholelithiasis, cardiomegaly, congestive cardiac failure, severe life threatening infections and chronic disabilities leading to distress in the families. Methodology: An analysis of 40 cases of hereditary hemolytic anaemia in the age group of 2 months to 12 years was done in the present study. On the basis of clinical presentations, physical findings, routine hematological investigations and hemoglobin electrophoresis pattern in hemoglobin defects were carried out to identify the type of hemolytic anaemias. Results: This clinocohematological study of hereditary hemolytic anaemia showed membrane defects- Hereditary spherocytosis in 4 cases (10%). The remaining 36 cases were having diseases affecting hemoglobin molecule which included Sickle cell anaemia-5 cases (12.5%), Sickle cell trait- 1 case (2.5%), Sickle cell/β thalassemia-1 case (2.5%), β thalassemia major- 23 cases (57.5%) and β thalassemia trait 6 cases(15%). Hereditary hemolytic anaemia with enzyme defects were not observed in this study. Majority of these cases presented with progressive pallor and hepatosplenomegaly. Peripheral blood smear examination showed microcytic hypochromic anaemia (87.5%) in majority of the cases. All cases were associated with reticulocytosis. Hemoglobin electrophoresis confirmed the diagnosis. Conclusion: Inspite of advanced diagnostic inestigations, the basic hematological investigation remains first panel or step towards the approach to diagnose hereditary hemolytic anaemia and hemoglobin electrophoresis will help in confirming the diagnosis. Keywords: hereditary hemolytic anaemia; clinoco-hematological study; hemoglobin electrophoresis DOI: 10.3329/bjms.v9i3.6471Bangladesh Journal of Medical Science Vol.09 No.3 July 2010, pp.154-160


1977 ◽  
Vol 137 (3) ◽  
pp. 281a-281
Author(s):  
P. E. Mickelson

2020 ◽  
pp. 1-2
Author(s):  
Michael Alperovich ◽  
Eric Park ◽  
Michael Alperovich ◽  
Omar Allam ◽  
Paul Abraham

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.


2021 ◽  
pp. 100047
Author(s):  
Álvaro Alejandre-de-Oña ◽  
Jaime Alonso-Muñoz ◽  
Pablo Demelo-Rodríguez ◽  
Jorge del-Toro-Cervera ◽  
Francisco Galeano-Valle

Blood ◽  
1963 ◽  
Vol 22 (3) ◽  
pp. 334-341 ◽  
Author(s):  
RICHARD D. LEVERE ◽  
HERBERT C. LICHTMAN ◽  
Joan Levine

Abstract The relative rates of incorporation of Fe59 into heterogenic hemoglobins was studied in four patients with sickle cell trait. Three of the patients were free of superimposed disease, while one had active pulmonary tuberculosis. In all subjects there was a significantly greater incorporation of radioiron, per milligram of hemoglobin, into hemoglobin S than into hemoglobin A. The data indicate that in sickle cell trait the rates of synthesis of the heterogenic hemoglobins are not proportional to their circulating concentrations. Two interpretations appear possible. Since the size of the intra-marrow pool of hemoglobin S was not known, it is possible that there exists a smaller preformed pool of the abnormal hemoglobin, with the isotope making its appearance first in hemoglobin S. However, it is also possible that hemoglobin S is synthesized at a rate which is greater than that reflected by its circulating concentration. This implies that the relative concentrations of hemoglobin S and hemoglobin A vary from erythrocyte to erythrocyte, and that those cells with the greatest proportion of hemoglobin S are selectively destroyed.


1975 ◽  
Vol 250 (22) ◽  
pp. 8630-8634 ◽  
Author(s):  
JR Shaeffer ◽  
MA Longley ◽  
J DeSimone ◽  
LJ Kleve

2012 ◽  
Vol 22 (3) ◽  
pp. 331-333
Author(s):  
C. Désidéri-Vaillant ◽  
J. Sapin-Lory ◽  
L. Di Costanzo ◽  
C. Cano ◽  
D. Lambrechts ◽  
...  

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