Un vaccino falcemico

Daniela Sambugaro

doi:10.53126/meb403794b

Un vaccino falcemico

Medico e Bambino ◽

10.53126/meb40394b ◽

2021 ◽

Vol 40 (6) ◽

pp. 394-394

Author(s):

Daniela Sambugaro

Keyword(s):

Sickle Cell ◽

Sickle Cell Anaemia ◽

High Specificity ◽

Post Vaccination ◽

Sickle Cell Crisis

A case of a sickle cell crisis initially diagnosed as post vaccination arthritis is described. The author stresses the high specificity of acute dactylitis with respect to the diagnosis of sickle cell anaemia.

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Sickle cell crisis as evaluated from measurements of hydroxybutyrate dehydrogenase and myoglobin in plasma.

Clinical Chemistry ◽

10.1093/clinchem/27.2.314 ◽

1981 ◽

Vol 27 (2) ◽

pp. 314-316 ◽

Cited By ~ 9

Author(s):

E F Roth ◽

P A Bardfeld ◽

S J Goldsmith ◽

E Radel ◽

J C Williams

Keyword(s):

Steady State ◽

Sickle Cell Disease ◽

Sickle Cell ◽

High Specificity ◽

Mean Value ◽

Cell Disease ◽

Sickle Cell Crisis ◽

The Mean ◽

Low Sensitivity ◽

Sickle Cell Crises

Abstract Data on plasma hydroxybutyrate dehydrogenase activity (I) and myoglobin concentration were used to evaluate painful sickle cell crises. I was increased during non-crisis steady state in patients with sickle cell disease as compared to normal values (232, SD 79.7 vs 85, SD 33 Sigma units/mL). During crisis, the mean value for I increased further to 379 (SD 139) Sigma units/mL. For 12 patients evaluated both during steady state and crisis, there was a mean increase in plasma I of 131% (SD 76%). Repeated determinations of I in sickle cell disease patients during several months while they were in steady state showed that baseline I varied by no more than 20% from the mean. Plasma myoglobin in patients with sickle cell disease was not above normal, but during crisis 21 of 39 patients tested had increased plasma myoglobin concentrations. Our data suggest that I may be a useful indicator of sickle cell crisis when the patient's own baseline value is available for comparison. Plasma myoglobin measurements give evidence of muscle damage during crisis with high specificity but low sensitivity.

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Role of Emergency Automated Red Cell Exchange in Sickle Cell Crisis: A Case Report

Clinical Medicine Insights Case Reports ◽

10.1177/1179547620970200 ◽

2020 ◽

Vol 13 ◽

pp. 117954762097020

Author(s):

Anubhav Gupta ◽

Kiran Chaudhary ◽

Rajnish Kaushik

Keyword(s):

Sickle Cell ◽

Sickle Cell Anaemia ◽

Symptomatic Improvement ◽

Red Cells ◽

Acute Chest Syndrome ◽

Compound Heterozygous ◽

Red Cell ◽

Transfusion Therapy ◽

Sickle Cell Crisis ◽

Patient At Risk

For many years main stay of treatment for sickle cell anaemia was transfusion therapy. But repeated transfusions put the patient at risk of iron overload. Automated red cell exchange is an evolving and newer technique which rapidly removes the sickle cells and has benefit in decreasing sickle cell load and related complications. Red cell exchange is a therapeutic procedure in which the patient’s whole blood is processed centrifugally in cell separator. Patient’s red cells are separated from other blood components and removed and replaced with donor red cells and colloids. We report our first experience of automated red cell exchange in 24-year-old female diagnosed case of sickle cell anaemia presented to us with acute chest syndrome with septic shock. Red cell exchange was planned to tide over the acute sickle cell crisis and provide symptomatic improvement. We also highlight that compound heterozygous thalassaemia could be associated with sickle cell disease which could make the diagnosis difficult. New generation automated Apheresis equipment’s provides better monitoring of the procedure that can be useful in severely ill patients also.

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Cardiovascular changes in children with sickle cell crisis

Cardiology in the Young ◽

10.1017/s1047951120000037 ◽

2020 ◽

Vol 30 (2) ◽

pp. 162-170

Author(s):

Richard Onalo ◽

Peter Cooper ◽

Antoinette Cilliers ◽

Uche Nnebe-Agumadu

Keyword(s):

Pulmonary Hypertension ◽

Steady State ◽

Sickle Cell ◽

Myocardial Ischaemia ◽

Sickle Cell Anaemia ◽

Cardiac Biomarkers ◽

Systemic Hypertension ◽

Cardiovascular Changes ◽

Sickle Cell Crisis ◽

The Mean

AbstractBackground:Sickle cell anaemia is characterised by frequent, sometimes serious events referred to as “crisis”. Cardiopulmonary consequences such as pulmonary hypertension and myocardial ischaemia may accompany a serious crisis.Objective:To determine the cardiovascular changes that occur during a severe sickle cell crisis.Methods:A cross-sectional comparative study of sickle cell anaemia in children (5–17 years) admitted during a severe crisis (cases) and those in steady state (controls) was conducted over a 2-year period. Effects of the crisis on the cardiopulmonary system were assessed. The diagnosis of myocardial ischaemia was made using electrocardiography and serological cardiac biomarkers, while cardiac dysfunction and the presence of pulmonary hypertension were determined using echocardiography. The presence of systemic hypertension and tachycardia was also evaluated.Results:A total of 176 patients were recruited, 92 in steady state (male:female ratio, 1.2:1) and 84 in severe crisis (male:female ratio, 1.3:1). The mean age was 10.4 ± 3.2 years for steady state and 10.5 ± 3.4 years for those in crisis. The mean heart rate in crisis was higher than in steady state (p < 0.0001). The blood pressures (systolic, p < 0.0001, diastolic, p < 0.0001, mean, p < 0.0001) as well as myocardial ischaemia scores (p < 0.0001) were higher in patients with crisis than in those in steady state. Similarly, conduction abnormalities, pulmonary hypertension, and ventricular dysfunction were more prevalent in the crisis than in the steady state.Conclusion:The present data suggest that sickle cell crisis results in a derangement of clinical, electrocardiographical, and echocardiographical parameters in children with sickle cell anaemia. Further research on these cardiovascular events may improve the overall care of these patients.

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