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A wide array of hematological disorders encountered in internal medicine has manifestations in the oral cavity and the facial region.
While a few of the book's images have the unclassifiable look of Hallucinogenia, the majoriti??y are produced by a cross-fertilization between classes.
There is a section on races, civilizations and customs of the countries shown on a map of the world, a world whose continents and islands seem to have the ability to rearrange themselves at will; there are sections on food, clothing, games, and urban architecture.
While a few of the book's images have the unclassifiable look of Hallucinogenia, the majority are produced by a cross-fertilization between classes. In general, the term diet refers to food and drink consumption (diaita (Greek) ‘way of life’).
Nutrition refers to a process by which living organisms physiologically absorb and metabolise food to ensure growth, energy production, repair of tissue and ultimately reproduction of the species (nutritio (Latin) ‘to nourish’) (Macpherson, 1995).
A ‘balanced’ diet refers to the minimum consumption of nutrients to provide the essential requirements of the body. Conversely, the majority of diet-related health problems in developed countries relate to excessive intake of essential and non-essential food and drink. The basal metabolic rate (BMR) is measured by the amount of oxygen inhaled compared to the amount of carbon dioxide excreted. Thermogenesis is the increase in energy output in response to food intake, cold exposure and psychological influences.
Kilocalorie (kcal): the amount of heat required to raise 1 kg of water 1 °C in temperature. In the moderately active woman, the BMR accounts for 1500 kcal a day out of a total daily energy expenditure of about 2300 kcal. The process whereby the energy derived from food products is made available to the various forms of work required by the body is known as metabolism.
Catabolism: the destructive process by which energy is produced with the breaking down of tissue into waste products.
Dietary standards have been used in Britain since the 19th century and are published to improve the health of the nation.
Sugars: should make up no more than 15% of the carbohydrate intake and can be subdivided into monosaccharides and disaccharides. Polysaccharides: do not have the crystalline form of sugar, and are used in the human diet as a source of starch and roughage.
Consumption of sugars (refined carbohydrates) is a major aetiological feature in dental caries and is discussed later.
The glycaemic index measures the rate at which glucose is absorbed through the intestine and its subsequent level in the blood. High-GI foods trigger the release of insulin for the removal of excess glucose from the blood for storage as glycogen or fat. Vitamin is a term that applies to a group of substances which are required in small amounts and are essential for growth and development.
Table 7.5 Sources of vitamins, their functions and deficiency states including those of oral relevance.
Carotenes, including beta-carotene, vitamin C and vitamin E, are among the many substances in food that have antioxidant properties. People who consume large amounts of yellow fruits and dark green or orange vegetables seem to be less prone to some forms of cancer.
Vitamin C may also form part of the body’s defence against the harmful effects of reactive oxygen species. Alpha-tocopherol (and to a lesser extent other tocopherols) can help prevent the oxidation of LDL and the formation of free radicals that may damage cells and cause heart disease. People suffering from Crohn’s disease and other intestinal diseases affecting absorption of nutrients.
Most people should be able to obtain their vitamin requirements by eating a healthy balanced diet. Minerals are inorganic substances, which are required in the structural composition of the hard and soft body tissues. Table 7.6 Sources of minerals, their function and deficiency states, including those of oral relevance.
Water is not formally a nutrient but additional sources of water are essential in the diet. A third of their diet made up from bread and cereals, choosing wholegrain, wholemeal, brown or ‘high-fibre’ varieties whenever possible. At least five portions of fruit and vegetables every day, including fresh, frozen, tinned, dried or juiced.
A moderate amount of meat, fish and alternatives such as pulses, eggs, nuts and beans, choosing lower-fat versions when possible. Less fatty and sugary foods and drinks, such as margarine, butter, cream, chocolate and biscuits, soft drinks, sweets, jam, cakes and ice cream. Milk, cheese, yoghurt, fromage frais and other dairy products in moderate amounts, choosing lower-fat varieties whenever possible. Figure 7.4 is a pictorial representation of a balanced diet, published by the Health Education Authority. Osteomyelitis of the mandible as a result of sickle cell disease: Report and literature review. Sickle cell anaemia: A review of the dental concerns and a retrospective study of dental and bony changes. Cephalometric measurements and facial deformities in subjects with  thalassaemia major. Main clinical manifestations of a bleeding diathesis: An often disregarded aspect of medical and surgical history taking. Bleeding in the heritable connective tissue disorders: Mechanisms, diagnosis and treatment. Bleeding manifestations in severely thrombocytopenic children with immune thrombocytopenic purpura.
Giant platelet disorders in African-American children misdiagnosed as idiopathic thrombocytopenic purpura. Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD). The importance of local hemostatic procedures in dental extractions and oral mucosal bleeding of haemophiliac patients.
Hemarthrosis of the temporomandibular joint in a patient with hemophilia B: Report of case. Hemophilic arthropathy of the temporomandibular joint: Review of the literature, a case report, and discussion. But these completely unarticulated struts would have been able to propel the organism by only the most minute of increments.
The leading influence seems to be Hieronymus Bosch.7 Serafini echoes The Garden of Earthly Delights, for instance, in his artificially patterned flora, fantastical architecture, incongruously coupled objects and cryptic rituals. After sections on flora and fauna, there is one on a genus consisting in of pairs of legs, which support not bodies but various surprising terminations such as umbrellas, balls of yarn, striped pods out of which tigers burst when ripe, numerous free-form shapes.
Perhaps the strangest section is the one on language, where the materiality of the letter is taken to the extreme.
The type of food and the amount eaten is an environmental factor in the aetiology of several diseases and variations in the morbidity and mortality between the world’s populations. Deficiency states, whilst no longer an overt problem in developed countries, continue to cause disease states in underdeveloped countries, due to famine or war. Excessive consumption of refined carbohydrates, saturated fats, salt and alcohol have been linked to obesity, cardiovascular disease, high blood pressure, type 2 diabetes, dental caries and certain cancers. Obesity is graded according to a person’s weight in kilograms, divided by their height in metres squared, and is known as the body mass index (Table 7.1). For an average man the BMR accounts for 1600 kcal out of a total energy expenditure of 2500 kcal.
The recommended daily amount (RDA) dates back to 1979 and was meant to be applied to groups of people to minimise the risk of undernutrition and was set high compared with the average requirement.


The term is applied because the hydrogen and oxygen are in the proportion to form water (hydrates). Proteins constitute an essential part of the diet as a source of energy and for replacement and repair of tissues. This attribute can help to counter the effects of reactive oxygen species (free radicals) which are produced by the body’s normal metabolic processes. Beta-carotene (but not preformed vitamin A) may play a role in the body’s natural defence mechanism against cancer by helping to destroy reactive oxygen species, but more research is needed to clarify this. It may work directly by destroying free radicals, indirectly by helping to reactivate vitamin E, or in both of these ways. Minor elements include copper, zinc, fluorine, chromium, molybdenum and selenium, which are components of many enzymes and are essential in trace amounts.
Most adults are eating too much (on average 9 g of salt per day (two teaspoonfuls) which should be reduced to less than 6 g). This is necessary to provide for the development of the child and to meet the physiological changes of the mother’s body.
And the tentacles, which might be expected to snare food, are too short to carry it to the organism's mouth, at whichever end that might be situated.
There is a relatively abstract section on natural (or from our point of view unnatural) elements, along with scientific machines for analyzing them.
Here letters and words proliferate into different species beyond our own world's superficial differences of language.
More specifically the term diet is used to refer to a prescribed course of eating and drinking for therapeutic benefit. According to Maslow’s hierarchy of needs (cited in Naidoo and Wills, 1998), basic physiological needs, such as hunger or thirst, must be satisfied first before an individual can find self-fulfilment. Where one or more of the essential nutrients are absent in the diet or there is inadequate absorption from the gastrointestinal tract, this is referred to as malnutrition. Hence the proportion of energy expenditure by the BMR is approximately two thirds of the total.
The Committee on Medical Aspects of Food Policy’s (COMA) Panel on Dietary Reference Values published their report in 1991. The rate at which pure glucose is absorbed and raises the blood glucose level is given a score of 100. They should make up no more than 30% of the diet of which a maximum 10% should be saturated fats.
Vegetable oils are unsaturated fats, are liquid at room temperature and include soya bean, maize and sunflower. The liver can produce all the bodily requirements for cholesterol, but a high consumption of saturated fats leads to high blood levels of circulatory cholesterol. The adult human requires eight amino acids which it cannot produce or store and these are known as essential amino acids (Table 7.4). Research is continuing into the possible role of vitamin C in the prevention of heart disease and cancer.
The recommended daily intake is 1–2 litres and the body can only survive a few days without water. These need to be provided by an increased intake of essential carbohydrates, proteins, iron, calcium and folates and vitamins C and D. Since what is designated the head is only an amorphous blob in all the examples found, it may even be that we are dealing here with a fragment of a larger, perhaps stranger, organism. This involves regulation of the quantity, timing and category of food and drink, either under professional guidance or self regulated.
Health promotion in affluent nations, in which these dietary components are freely available, is aimed at awareness and empowerment of the individual to eat a ‘healthy’ diet, i.e.
Low-GI foods are digested slowly and raise glucose level in the blood by a small amount, and hence only low levels of insulin are produced and the body feels satisfied for longer.
The body can produce non-essential amino acids, but a diet of mixed proteins is recommended for health. Many vitamins are coenzymes, which have an essential function in a chemical reaction catalysed by a specific enzyme. Fruit and vegetables are a good source of water-soluble vitamins but they also contain fibre and antioxidants, which are not provided by a vitamin tablet.
Table 7.6 gives sources of minerals, their function and deficiency states, including those of oral relevance. There is increased demand in hot climates, following strenuous exercise, in times of fever and in diarrhoea and vomiting.
In the final 3 months of pregnancy, an additional 200 kcal are required daily, and in lactation 500 kcal extra a day. No longer confined to the plane of the page, they rise above it with the help of gas-filled balloons or are dropped onto it by tiny parachutes. The reader is recommended to review Scientific Basis of Nutritional Education: a Synopsis of Dietary Values (Health Education Authority, 1992), for specific detail, but dietary reference values are an estimate of the range of requirements of nutrients for a population or group and are intended for guidance. Carbohydrates are stored in liver and muscle as glycogen, but excess is converted into fat for storage.
Free radicals readily oxidise polyunsaturated fatty acids in food and in cell membranes in the body to give lipid peroxides which can also damage cells.
There is currently no evidence that vitamins prevent colds and, until such time as the evidence proves that vitamin supplements are beneficial to the healthy person, they are best avoided. It has become fashionable to drink bottled water which may be carbonated at source or by the manufacturer before bottling. There is some evidence that vitamin D deficiency in pregnancy can cause enamel hypoplasia and thus predispose to caries in the deciduous dentition (Rugg-Gunn, 1993).
Education concerning obesity should be focused on awareness that food consumption must match physical activity.
Such guidance is required for a scientific basis for food counselling, meal provision and food labelling.
Table 7.5 outlines sources of vitamins, their function and deficiency states, including those of oral relevance. Peroxides, for example those formed by the oxidation of low-density lipoprotein (LDL) cholesterol, may play a part in the formation of the ‘plaque’ which can build up on the walls of the arteries and eventually cause heart disease. Some groups, such as pregnant women, vegans, etc., may benefit after consultation with a physician. In the case of calcium deficiency in the mother, calcium cannot be removed from the calcified tissues of her teeth, and any increase in caries is due to a high-sucrose diet. No matter how broad a range of stimuli is provided by our physical apparatus of seeing, our interpretive reading of those stimuli is necessarily a partial one. Wolfgang-Rainer Mann's recent book, The Discovery of Things,5 claims that Aristotle's early text Categories was not, as some assert, the common sense prolegomenon to his later, more significant work.
The soya bean is high in protein, and is available as tofu (soybean curd) and miso (fermented bean paste). People with oedema, high blood pressure or heart diseases should check the sodium levels of bottled water.
We select those features of the object which are felt to be salient or definitive --and what comes forward as definitive is that which corresponds to an already existing definition. If Aristotle's text feels like common sense today, that is because the ideas put forth there have become so generally accepted as scarcely to seem like philosophy at all, though they were far from self-evident in their time.
Yet it too foregrounds its material nature, if only because, as a fictional construct, it is opaque to our understanding. The dietary requirements of an individual throughout life are dependent upon the energy and growth requirements, for example during pregnancy, lactation and childhood. For the micronutrients the reference nutrient intake (RNI) is used, which represents the amount of nutrient which is sufficient for almost all individuals. What we see is what we have been prepared to see by systems of classification or categorization. In a radical departure from Plato's doctrine of the Forms, Aristotle asserts that the fundamental entities are ordinary things and their features.


There is enough consistency in the labelling of sections and the use of individual characters to foster some hope that this is a cryptogram; but into what known language would these utterly foreign signs be decoded?
In addition the degree of physical activity and concomitant disease status must be considered.
A sort of Rosetta stone is depicted at the beginning of the language section; however, it provides no illumination, pairing the language of the Codex with a more archaic, and equally fantastical, hieroglyphic system. Encyclopedias can be broadly divided into two kinds: those that are ordered alphabetically and those that are arranged systematically.
Atrophic glossitis "flattening of the tongue papillae" resulting in a smooth and erythematous tongue may mimic migratory glossitis [Table 1]. Despite this distinction it will be useful for us to remind ourselves here of some of the things that Michel de Certeau has said about glossolalia. Migratory glossitis, also known as geographic tongue, is a condition of unknown etiology that affects 12% of the population. It does provide a quick and convenient way of locating and extracting information, bypassing all that is irrelevant to the item sought. Read in sequence, though, the alphabetical encyclopedia produces only surreal juxtapositions comparable to Raymond Roussel's famous encounter between an umbrella and a sewing-machine upon an operating table -an image taken straight from the files of hallucination. Similarly, glyptolalia is not-a-writing that becomes the abstract material manifestation of writing per se. In atrophic glossitis, these areas do not have a white keratotic border and they increase in size rather than changing in position. The systematic encyclopedia at first seems to do better, to promise an escape from the hallucinatory into a realm of serene order. Angular stomatitis (painful fissures at the corners of the mouth) and cheilosis (dry scaling of the lips and corners of the mouth) are also common findings associated with iron deficiency anemia. It presents an ordered view of the universe and the things of this world, as well as of the particular civilization that produced the encyclopedia (and the order of the world picture depicted therein). The encyclopedia that systematizes and classifies, the kind I am addressing, provides its readers with a way of seeing things -logically, and as a consequence also visually. When angular cheilitis is due to opportunistic infections brought on by decreased resistance secondary to nutrient deficiencies, treatment should focus on antifungal therapy, correction of the nutrient deficiency and diet modification to make eating a more comfortable experience. Yet this way of seeing things can at times be linked to that other way of seeing things, the hallucinatory. It is also associated with koilonychias (or spoon nails), pagophagia and dysphagia due to pharyngoesophageal ulcerations and esophageal webs.
And insofar as the systematic encyclopedia presents us with a categorized way of seeing things, it creates a sense that ordered knowledge is the authoritative reality, while it may be -must in a sense always be- provisional, incomplete, and to that degree delusory. The operating table is most obviously another image to be added to those of the umbrella and the sewing-machine. Felt initially to be an accurate reflection of the real, the system at its extreme reveals itself to be arbitrary, unreal. Sickle cell anemia (HbSS) is the most common type and represents the homozygous form where the individuals inherit a double dose of the abnormal gene that codes for hemoglobin S. The sickle hemoglobin abnormality is caused by substitution of valine for glutamic acid in the sixth position from the NH 2 terminal end of the β-globin chain. With decreased oxygen tension, the abnormal hemoglobin polymerizes, forming fluid polymers (tactoids) that cause the red cells to deform into a characteristic sickle shape that may plug different areas of the microcirculation or large vessels. However, we have one such encyclopedia that can be held in the hands, experienced directly. It is the Codex Seraphinianus, perpetrated by Luigi Serafini in 1981 and since published in a number of different countries.
All tissues and organs within the body are at risk of damage due to sickling.Although relatively uncommon, a number of orofacial changes have also been observed in sickle cell disease. These orofacial changes in HbSS as reported in the literature include midfacial overgrowth attributable to marrow hyperplasia, [13],[14] other skull and jaw changes such as increased thickening of the skull and osteoporotic changes, [15],[16] mandibular infarction that may be followed by osteosclerosis, [17],[18] osteomyelitis of the mandible, [19],[20],[21],[22] anesthesia or paraesthesia of the mental nerve, [23],[24] asymptomatic pulpal necrosis, [25] orofacial pain, enamel hypomineralization and diastema [Table 1].
While exhibiting at every turn the look of orderly classification, Serafini's encyclopedia is fundamentally hallucinatory, as we shall see. And if this term is a classification, it is also a term -or rather a state- that challenges our fundamental notions of classification.
Surgical exploration, however, revealed a large hematoma between the periosteum and the lateral aspect of the ramus of the mandible.
The boy also exhibited gingival enlargement, which was considered to be an outcome of repeated hemorrhagic episodes and fibrous repair.
Gingival biopsies reported the presence of erythrocyte-filled intraepithelial blood vessels in the gingival epithelium.Saint Clair de Velasquez and Rivera reported, in a study, that the most common soft tissue oral manifestation of sickle cell anemia in a Venezuelan population was buccal mucosa pallor, while the most common hard tissue finding was enlarged medullary spaces. Based on genetic and clinical entities, thalassaemia are classified as homozygous, heterozygous or compound heterozygous.
Patients with the most severe form of the disease rarely survive into adulthood because of cardiac failure, chronic anemia and hypoxia. All hemorrhagic hemostatic disorders, both inherited and acquired, may produce a variety of orofacial manifestations, including petechiae, nose bleeding, spontaneous and post-traumatic gingival hemorrhages and prolonged post-extraction bleeding [Table 2].
These features are also seen in secondary thrombocytopenia due to the myelophthisic syndrome, autoimmune disorder, aplastic anemia [Figure 1]a and b, infections, collagen vascular disease, disseminated intravascular coagulopathy and drugs.
It is an autoimmune disease characterized by the production of antibodies against ones own platelets. These antibodies adhere to the platelets and are recognized and destroyed by the reticuloendothelial system.
Consequently, the platelet count gradually diminishes and is insufficient for the maintenance of primary hemostasis. Common manifestations of this hemorrhagic disorder are gingival hemorrhage, purpura, epistaxis, petechiae and menorrhagia. Type 1 disease is the most common form of VWD, accounting for approximately 80% of all cases. It is transmitted as an autosomal-dominant trait and thus there may be evidence of a family history of excessive bleeding. Most forms of the disease show incomplete penetrance of the phenotype and variable expressivity of bleeding symptoms within families.
Type 2N VWD, in which isolated low FVIII levels occur, also shows a recessive pattern of inheritance and, thus, here again, a family history may be absent.
If the history suggests VWD and oral surgery is contemplated in these patients, hematology consultation should be obtained and the patient's blood should be typed. Hemophilia A is a deficiency of factor VIII while hemophilia B (Christmas disease) is a deficiency of factor IX. Sonis and Musselman [57] reported an average 29.1 bleeding events per year, serious enough to require factor replacement in F VIII-deficient patients, of which 9% involved oral structures.
Many minor oral bleeds, such as those from the gingiva or dental pulp, can be controlled by local measures while more major forms will require factor replacement.
These rare disorders include defects of fibrinogen, prothrombin, factor V, combined deficiency of factors V and VIII, factor VII, factor X, deficiency of vitamin K-dependent factors (II, VII, IX, X), factor XI, factor XII and factor XIII deficiency. Homozygous deficiency varies from 1 in 500,000 for factor VII deficiency to 1 in 2 million for prothrombin. Surprisingly, even dental specialists avoid these candidates and contribute to the conversion of a simple dental patient to an oral surgical patient. Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent disease of hemopoiesis or hemostasis or a latent one that may subsequently manifest itself.
These manifestations must be properly recognized if the patient must receive appropriate diagnosis and referral for treatment.



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