Anaemia sickle-cell

Fundamentals of Medicinal Chemistry, Edited by Gareth Thomas 

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Keshan disease (selenium deficiency) Sickle cell anaemia... 

Bob Richards Fred offered a little earlier, the model of what happens in sickle-cell anaemia case where there s a huge sequelae of different kinds of what might be regarded as symptomatic clusters. In the case of the mouse pup macao genes, would you say that model that has sickle-cell is a fair model in a general sort of way for what happens with the mouse pups who displayed various kinds of behaviour, including some that were labelled aggressive. 

Ingram, V. M. (1956). A specific chemical difference between the globins of normal human and sickle cell anaemia haemoglobin. Nature 178, 792-794. 

Clearly this patient has both clinical and haematological symptoms of severe anaemia. The cause is too few red cells low RBC count and PCV but the erythrocytes which are present contain a higher than usual concentration of haemoglobin (MCHC result). Iron deficiency and vitamin B12 deficiency can be ruled out by the high serum ferritin and normal MCV results respectively. The negative HbS screen rules out sickle cell anaemia which is fairly common in Africans. 

Red blood cell fractions are administered to patients suflfering from severe anaemia, including patients with sickle cell anaemia and new born babies suflfering from haemolytic disease. 

For idiosyncratic, patient related adverse reactions, less confirmatory tests are available but the number is growing. Skin, blood and urine tests are available to confirm acute and chronic allergic reactions. Genetic tests can determine the susceptibility of individuals and includes general tests such as for the porphyrias and sickle cell anaemia, and specific tests for dmg metabolism, such as acetylator status... 

Anaemia due to excessive destruction, of blood e.g. sickle cell anaemia and haemolytic anaemia. 

Despite its considerable involvement in metabolic processes, no specific deficiency syndrome in humans has been attributed to vitamin B6 (19,103). A considerable number of nonvitamin functions have been suggested, but they remain controversial (102,103,108-111). These include roles in coronary heart disease, immune response, premenstrual syndrome, sickle-cell anaemia, asthma, autism, gestational diabetes, carpal tunnel syndrome, and cancer. 

An investigation of the role played by zinc in sickle cell anaemia has shown264 that zinc decreases the amount of haemoglobin associated with red-cell membranes and inhibits the effect of calcium in causing haemoglobin retention by membranes. 

An error of a single base in each strand of DNA can bring about the amino acid residue error that causes sickle-cell anaemia. 

Allen S (2005) Understanding sickle cell anaemia. Pharmaceutical Journal 275 25-28. 

Charache S, Terrin ML, Moore RD et al. (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anaemia. New England Journal of Medicine 332 1317-1322. 

GPNotebook (2008) Sickle cell anaemia. Available at http //www.gpnotebook.co.uk/ simplepage.cfm ID=108737331 7 [Accessed 4 July 2008],... 

AW s white cell count is raised, probably due to an infection which may well have precipitated this crisis. The low haemoglobin and haematocrit are due to the excessive destruction of red cells that occurs in this haemolytic form of anaemia and the raised bilirubin is due to the rapid breakdown of the cells. Raised reticulocytes are seen in sickle cell anaemia as the body compensates for the increased cell breakdown by increasing production of red cells. Target cells are blood cells which resemble a shooting target and are found in patients with sickle cell anaemia as well as in a number of other conditions. 

Although hydroxycarbamide is a licensed medication, it is not licensed for the management of sickle cell anaemia use in this case is unlicensed or off-licence. As every pharmacist assumes a duty of care to a patient when supplying a medicine, this means that if an adverse reaction is suffered, the supplying pharmacist may assume some liability along with the doctor who prescribed it. 

As well as ensuring that the prescriber is aware of the unlicensed use of the medication it is important to bring this to the attention of the patient. This should be done without undermining the patient s confidence in either the prescriber or the prescribed medicine. This is also necessary because the patient information leaflet which you are supplying with the medication will not include anything to suggest that the product can be used in sickle cell anaemia. 

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