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By K. Orknarok. State University of New York College Maritime College at Fort Schuyler.

Inherited haemolytic anaemia Complications Achronically high serum bilirubin predisposes to the Hereditary spherocytosis formation of pigment gallstones discount abana 60 pills free shipping. Chronic haemolysis predisposes to folate deficiency and thus levels should Definition be monitored and replacement given as required buy abana 60pills with mastercard. Par- An autosomal dominant condition in which the red cells vovirus infections that cause a temporary bone marrow are spherical. Hereditary elliptocytosis is an autosomal failure may result in an aplastic crisis. Investigations r Haemolysis is suggested by a rise in bilirubin, high Incidence urinary urobilinogen (due to bilirubin breakdown Commonest inherited haemolytic anaemia; 1 in 5000. In intravascular haemolysis, red cell fragments are Aetiology/pathophysiology seen in the blood film, whereas spherocytes may be There is a high new mutation rate with 25% of patients present in extravascular haemolysis. The underlying cause is cell life span can be demonstrated using labelled red aweakness in the link between the cytoskeleton and cells. This may be a quantitative or 474 Chapter 12: Haematology and clinical immunology functional abnormality of any of the membrane proteins Incidence (spectrin, ankyrin, protein 4. These cells are more rigid than normal and As HbF synthesis is normal, it presents at 6 months. Sex Clinical features M = F Spherocytosis may present as neonatal jaundice or anaemia with chronic malaise and splenomegaly. Nor- Geography mal infections cause a relative increase in haemolysis and Occurs most frequently in Africa, Middle East, India and may result in jaundice. Aetiology Investigations Apoint mutation on chromosome 11 results in a sub- Anaemia is usually mild. A blood film will demonstrate stitution valine for glutamine at the sixth codon on the the spherocytes, but this cell morphology is not diagnos- β globin chain to form haemoglobin (Hb)S. Thediagnosiscanbeconfirmedbydemonstratingthe dehydration, hypoxia and cold may precipitate a sickle osmotic fragility of the red blood cells. Patients are given Pathophysiology pneumococcal vaccinations and prophylactic antibiotics HbS molecules, when deoxygenated tend to aggregate post splenectomy. The red blood cells become inflex- ible and sickle shaped and become trapped in the mi- Haemoglobinopathies crocirculation, especially within bones, resulting in mi- Haemoglobinopathies are abnormalities in the nor- crovessel occlusion. Normal haemoglobin is made up of four polypeptide chains Clinical features each containing a haem group. HbA is the main adult Sickle cell trait (the carrier state) is asymptomatic, but form comprising two α chains and two β chains. Sickle cell also have a minor haemoglobin HbA2,which makes up anaemia is a clinical spectrum ranging from asymp- around 2% of the circulating haemoglobin and con- tomatic to severe haemolytic anaemia and recurrent sists of two α chains and two δ chains. Painful vascular occlusive crises typically haemoglobins result from: produce symptoms of bone pain and pleuritic chest pain r Abnormal globin chain production such as thalas- with a low-grade fever. Other patterns of crisis: r Acute sequestration (pooling of blood in liver and Sickle cell anaemia spleen) requires transfusion for apparent hypo- Definition volaemia. Autosomal recessive condition in which there is abnor- r Pulmonary infarction may occur in association with mal structure of the globin chain. Chapter 12: Haemoglobin disorders and anaemia 475 Complications syndrome or cerebral infarction require exchange blood Patients have a susceptibility to infections including transfusionstoremovesicklecells. Transfusionsmayalso streptococcal infections and osteomyelitis often due be indicated in patients with regular severe crises and to salmonella. Prognosis Retinal detachment and proliferative retinopathy may Thereismarkedvariationintheseverityofthecondition, result in blindness. See also complications of haemolytic some patients have a relatively normal life span with few anaemia (page 473). Blood film shows a α-Thalassaemia high reticulocyte count and sickle shaped red blood cells. Definition r Sickle screening tests use a reducing solution, which Inherited haemoglobinopathy with defective synthesis causes HbS to precipitate. Aetiology r X-ray of the tubular bones may show destruction and α-Thalassaemia is caused by gene deletions. There are medullary sclerosis together with periosteal bone for- four copies of the α gene, two on each chromosome 16. Management Clinical features Treatment is largely symptomatic with prophylactic an- r Deletion of all four copies of the α gene (–/–) prevents tibiotics,folicacidandpneumococcalvaccination. This disorder agement of a painful crisis includes oxygenation, ade- is also termed haemoglobin Bart’s (γ4)hydrops syn- quate hydration and analgesia. Acute se- r Deletion of three genes (–/α-) causes HbH disease (a questration requires blood transfusion, as patients be- moderate anaemia with splenomegaly and the pro- comeshocked. Normal Investigations Full blood count shows microcytosis with or without Sickle Trait anaemia. These mutations may result in no β chain production Investigations (β0)orveryreducedproduction (β+). The reticulocyte count is noproductionofβ globinandhavetheclinicalpicture raised and there are nucleated red cells. Management Excess α chains precipitate in the red blood cells r Thalassaemiaminordoesnotrequiretreatment;how- or combine with δ resulting in increased HbA2, and ever, iron supplements should be avoided unless γ resulting in increased levels of fetal haemoglobin co-existent iron deficiency has been demonstrated.

Integrated tick control An integrated approach which uses personal protection methods buy 60pills abana otc, tick monitoring generic abana 60pills overnight delivery, habitat modification and acaricide application may be most effective in controlling ticks. Tick control measures should be tailored to the biology and seasonality of particular species. When choosing control measures, the type of habitat, density and activity of the human population, incidence of infection in the vector species, extent to which tick control is necessary, and degree of environmental modification that is acceptable should be carefully considered. Construct artificial homes or manage for mosquito predators such as bird, bat and fish species. Do not introduce non-native species of fish or other predators into the wetland for mosquito control. Reduce mosquito breeding habitat: Reduce the number of isolated, stagnant, shallow (5-7 cm deep) areas. Construct a vegetation buffer between the adjacent land and the wetland to filter nutrients and sediments. Install fences to keep livestock from entering the wetland to reduce nutrient-loading and sedimentation problems. In ornamental/managed ponds: Add a waterfall, or install an aerating pump, to keep water moving and reduce mosquito larvae. Keep the surface of the water clear of free-floating vegetation and debris during times of peak mosquito activity. Chemical control It may be necessary to use alternative mosquito control measures if the above are not possible or ineffective. The environmental impact of vector control measures should be evaluated and appropriate approvals should be granted before undertaken. This method is deemed least damaging to non-target wildlife and should be used before adulticides. During periods of flooding, the number and extent of breeding sites is usually too high for larvicidal measures to be feasible. Open marsh water management Control mosquitoes by introducing their natural predators to areas of tidal marsh using a system of pools connected by radial ditches. Fish feed on mosquitoes during high tide, then retreat to sumps or reservoirs at low tide. Environmental management – adapting behaviour of people and animals People: Wear light coloured clothing which covers arms and legs. Use impregnated mosquito netting when sleeping outdoors or in an open unscreened structure. Avoid physical exertion, and use colognes and perfumes sparingly as these may attract mosquitoes. Note that some repellents cause harm to wildlife species, particularly amphibians. Use screened housing with measures to eliminate mosquitoes from inside structures. Alter flow rate and water levels to disturb snail habitats and their food sources: Include ‘v’ shaped banks in irrigation channels. Remove vegetation/silt in channels to avoid a drop in velocity which may lead to further vegetation growth and good habitat for snails. Note that personnel involved in the manual removal of vegetation are increasing their exposure to snails. Flow rate should only be addressed with knowledge of the ecology of the snail in question e. Borrow-pits, small pools and ponds serving no special purpose (for humans, wildlife or livestock) may be drained to eliminate breeding sites. Expose snail habitat: Remove littoral vegetation from the sides of canals feeding irrigation projects to expose snail habitat. Thought should be given to downstream conditions and the potential for the liberated snails to recolonise new habitat. Where possible dry out littoral zones to strand snail populations, however take into account the specific ecology and the resilience of the target species. Chemical control Use of molluscicides may cause environmental damage and should be avoided. Applications are usually restricted to places frequently used by people for swimming, bathing etc. Environmental management – adapting behaviour of people and animals People: Where possible, avoid new human settlements near infested wetlands. It is safest to consider all freshwater bodies in endemic areas as potential transmission sites if sites otherwise not identified. For agricultural workers at constant risk of infection, periodic examination and treatment may be the most feasible approach to disease control.

Michel Foucault generic abana 60 pills on-line, in The Birth of the Clinic buy abana 60pills without prescription, quoted French revolutionary dreamers who imagined how in a ceaselessly supervised environment, citi- zens properly instructed in simple dietary regimens and imbued with a Spartan sense of duty, would remain healthy and happy until natural death at an advanced age. Dictator- 150 Coercive medicine ship was, however, a first necessary step towards ultimate liberation. The first public health government department was established in the year of the Revolution, 1789. It is a paradox that the Age of Enlighten- ment, which destroyed the false certainties of religious dogmas and freed man from superstition, forged, at the same time, new chains for the enslavement of man, by regarding him as a machine, governed by materialistic and deterministic laws. In the 19th century, the messianic streak of public health was heard of no more, having been replaced by medical polic- ing, which took on tasks such as the compulsory examination of prostitutes. The concept of a medical police was developed in Germany in the 17th and 18th centuries, as part of mercan- tilist politics, aimed at securing greater power and wealth for 30 the monarch and the state. At the beginning of the 19th century, commonly used terms were Staatsarzneikunde (state medicine), Staatsarzneiwissenschaft (state medical sci- ence), or Gesundheit des Staates (the health of the nation). Rudolf Virchow, the founder of cell pathology, compared the human body to the state, and the cells to the citizens. Weindling showed how these ideas had 31 formed the foundation of Nazi health policy. Lord Rosebery, the future leader of the Liberal party, addressed an audience at Glasgow University in 1900 and stated that: An Empire such as ours requires as its first condition an Imperial Race - a race vigorous and industrious and intrepid. The survival of the fittest is an absolute truth in 32 the conditions of the modern world. Food reformers, such as the national tennis champion, Eus- tace Miles, in his 1902 book, Avenues to Health, advocated the establishment of a national diet, which would increase the vitality and the moral strength of the nation. Health was a duty: to ourselves, to our own nation, to all nations, and to 33 posterity. This combination of social Darwinism, moralism, and lifestyl- ism is strikingly similar to the modern ideology of healthism. Who now remembers what Henri de Mondeville wrote in 1320 in his Chirurgie: Anyone who believes that anything can be suited to every- one is a great fool, because medicine is practised not on 34 mankind in general, but on every individual in particular. Health was normality, disease was either the result of an unhealthy lifestyle or a sign of heredi- tary degeneration. The glorification of health (which was equated with beauty) and the inculpation of the sick received whole-hearted support from the medical profession. Only in 152 Coercive medicine the last decade has it become possible in Germany to examine objectively the ideology of public health in Nazi Germany, and many excellent German language analyses are now available. Professional secrecy was no longer a binding precept, as the public good had to take precedence over individual interest. The misuse of tobacco and alcohol were the greatest threats to the national health, for which the liberalism of the pre-Nazi era was blamed. The criteria of a useful life were in men, the ability to fight for the fatherland, and in women, to bear healthy, racially pure 35 children. Tobacco manufacturers were prohibited from advertising their products by appealing to women, sports- 36 men, or car-drivers. Even the leisure time of workers needed state supervision, in a system called Freizeitgestaltung (organisation of free-time). Communist medicine was first outlined in Voyage en Icarie by Etienne Cabet (1788-1856), a French revolutionary and a follower of Babeuf. In Icaria, the ideal communist state, the doc- tor did not have to depend on private practice as he was a salaried member of the community and medical service was free for all. Intemperate drinking and eating, lack of exercise, sexual overindulgence or tobacco smoking (about which Cabet had particularly strong feelings) were not tolerated. Only those individuals who had desirable mental and physical qualities were allowed to have children. None of this required imposition from above, as it was supported by a national democratic consensus. The results of decades of health promotion in communist countries should be carefully studied and evaluated by those who intend to introduce similar principles in Western democracies. What benefits, for example, have been observed in state-organised, compul- sory cervical cancer screening programmes in communist countries? When a delegation of prominent British physicians visited Russia in 1960, they were impressed by the Soviet emphasis on health promotion. While many middle-aged men and women appear drab and weary, the children and young people seem to be healthy, happy, and friendly. Von Mises makes the point that the difference between communism and fascism on the one hand, and socialism on the other is only 39 in the means by which to achieve identical ends. This per- manent tutelage, which von Mises called etatism, and British commentators call the nanny state, exists, as yet, in Western democracies only in a diluted version because of various con- stitutional, philosophical, moral and political obstacles. As Talmon showed, the Left starts from the premise that man is perfectible, as Rousseau believed, and by changing the unhealthy environment, created by an unfettered capitalism, man can be made healthy and happy, even though at times some degree of coercion might become necessary. For example, the poor are known to suffer more from diseases and have shorter life-expectancy, but should this be blamed on their lifestyle or on the political conditions which are the causes of poverty? By linking poverty with disease (which is not unreasonable on its own), Marxists promise that in a classless society the health of the poor will improve.