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Diagno- tonetti used the term scleroderma to describe sis begins with a thorough medical history and a patient with dark leatherlike skin who lost physical exam purchase tricor 160 mg online cholesterol chart range. A complete blood count order generic tricor on-line cholesterol chart age, uri- Scleroderma Foundation estimates that 300 buy tricor line top cholesterol lowering foods,000 nalysis, autoantibody testing, measurement of people in the United States have scleroderma. Scleroderma is four times more common often with symptom-free remissions that can in women than in men, and the average age of last for years. Lupus Two major forms of scleroderma are rec- patients should limit exposure to sunlight, never ognized. This form of scleroderma tends to may trigger scleroderma in those who are genet- regress or stop progressing without treatment. Scleroderma is four times However, it can be progressive and disfiguring, more common in women than in men, suggest- requiring treatment to control disease activity. No single test will the extent of skin tightening: limited cutaneous confirm the diagnosis of scleroderma. Scleroderma patients tightening is confined to the fingers, hands, and should not use tobacco, should avoid exposure arms below the elbows, and may involve tighten- to cold and stress, exercise to help keep skin and ing of skin on the feet and legs below the knees. The most common initial signs and symptoms Sjögren’s Syndrome of scleroderma are secondary Raynaud’s phe- Sjögren’s syndrome is a chronic, slowly progres- nomenon, thickening and tightening of the skin sive autoimmune disease that affects the exo- of the fingers, and pain in two or more joints. Early symptoms can also include heartburn, dif- The Sjögren’s Syndrome Foundation estimates ficulty swallowing, or shortness of breath. Scleroderma Localized Systemic Scleroderma Scleroderma Linear Limited Cutaneous Diffuse Cutaneous Morphea Scleroderma Scleroderma Scleroderma Figure 2–5  Types of scleroderma. Chapter Two Immunity and Disease L 23 About half of the cases are called primary ranging from the merely annoying, to extreme Sjögren’s syndrome. The Type I Hypersensitivity and anaphylaxis hallmark symptoms of Sjögren’s syndrome are There are several different types of hypersensi- dry eyes and mouth. Type I is a local allergy, occurring rapidly The etiology of Sjögren’s syndrome is idio- where the allergen encounters the body. Genetic factors, hormones, and environ- I reaction is triggered by IgE, the immunoglobu- mental triggers such as viral infections play a lin that responds to the presence of allergens. A first-degree relative with autoimmunity The allergy problems arise because the IgE also increases the risk for Sjögren’s syndrome sev- binds to mast cells and induces them to release enfold. Sjögren’s syndrome often affects women histamine and other potent chemicals respon- during their childbearing years, suggesting a sible for allergy symptoms. This tissue fluid causes edema, or misdiagnosed because its signs and symptoms swelling, where the allergen was encountered. The aver- Edema in the skin causes the welts and itching age time from the onset of signs and symptoms of hives. Antihistamines inhibit the Diagnosis begins with a thorough medical effects of histamine. Tear and saliva tests, autoantibody testing, and lip or salivary gland biopsy may be performed. Sjögren’s syndrome has no cure and cannot Epinephrine Treatment for Life- be prevented. Certain allergic individuals must Allergy carry epinephrine at all times in an epinephrine-injection device, Allergy, or hypersensitivity, is an extreme immune which can be used for self-injection in an emergency. The nor- Think Critically mally harmless antigen that causes an allergic response is called an allergen. The allergic response to allergens causes problems 24 L Part I Mechanisms of Disease In some people immediate hypersensitivity action of T cells, which require time to recog- causes a systemic, acute allergic response (ana- nize the allergen, reproduce and differentiate, phylaxis) that may be life-threatening. Delayed that cause anaphylaxis are found in foods such hypersensitivity includes the skin reaction to as peanuts, in latex, in some medications such poison ivy or poison oak, contact dermatitis as penicillin, and in the venom of stinging insects from wearing latex gloves (Figure 2–6 ), and the such as wasps and bees. Antihista- of anaphylaxis include a sudden drop in blood mines are not effective against delayed hyper- pressure, narrowing of the airways, rapid and sensitivity, but corticosteroids provide some weak pulse, hives, and nausea and vomiting. Table 2–3  summarizes the four types of Antihistamines, corticosteroids, and epinephrine hypersensitivities. A commonly used allergy the response to an incompatible blood transfu- test is the skin test, in which a small amount sion. A person with type A blood has A antigens of the suspected allergen is placed on or below on their red cells and antibodies against type B the skin to assess the skin’s response to the blood in their serum. Types of skin tests include the skin type B transfusion, his or her antibodies interact prick test, intradermal test, and skin patch test. Other examples of cytotoxic hypersensitivity include blood to an Rh- and Rh incompatibility during pregnancy.

The program pro- available and order tricor 160mg cholesterol steroid, possibly generic tricor 160mg mastercard test cholesterol jean coutu, less useful buy tricor 160 mg on line cholesterol weight, and the family’s rela- vides a variety of in-house cognitive, psychosocial, and tionship to the community and community-based sup- skill groups and activities, but the primary work and so- ports becomes more salient. They are accompanied into the 496 Textbook of Traumatic Brain Injury community by a small group of peers (usually three other in the judgment of the clinician, are simply not possible. Activities vary but are al- When families react to such feedback with resistance, ways associated with skill development. The overall goals skepticism, or even anger, clinicians often see the family of the program are the development and enhancement of as being unaware, or in denial, and in need of education. These types of supports and find a way of working with the family around the goals are extremely helpful to families in the long run. The ser- in question without placing the family in a position of giv- vice coordination aspect alone relieves families of much of ing up hope. Doing so requires a good bit of clinical savvy the logistical and practical, if not emotional, burdens. The for individuals with an injury, which may help normalize following principles are meant as possible tools for the as much as possible the family role and life cycle issues. In our experi- Principle #1: Ask the Person With the Injury What He or ence, there is an ongoing sense of loss and visible grieving, She Wants. Sometimes, clinicians become so caught up not just by family members but also by the individuals dealing with family expectations and demands that they themselves about their “lost self”-who they used to be, fight the battle of what is realistic without ever inquiring who they thought they were going to become, and their what the injured person wants. This may become be insistent that their injured son will go back to law less prominent with increased socialization opportunities school, the eager-to-please son may be harboring his own and increased success in the community but rarely en- doubts about whether he still wants to do so. In working with families whose member takes a number of sessions privately with the injured ado- was injured 10, 15, or even 20 years earlier, we still see lescent or young adult to help the person sort out what his grief, anger, guilt, and even denial. The usual pattern is or her goals are and how they may be different from the that these emotions erupt periodically and present in goals of the rest of the family. Remember what any good marital therapist knows: each Another interesting clinical observation is how family person’s set of perceptions is absolutely real for them. Perceptions are driven autonomy within the family and/or community or self- not by cold, clear observation of obvious facts but by inter- advocacy. These hopes routines, roles, and rules or as potentially dangerous situ- must be dealt with gently and with respect. This may stem from fear for and protectiveness of least, do not immediately and offhandedly dismiss these the injured individual and from the many years of strug- hopes as unrealistic; it will be experienced as a crushing gling to establish a new family homeostasis. Many families have had ties, may lead to a need for further family restructuring experience with professionals who made pronouncements and education. Even Family Expectations in less severe cases, we really do not know what any given individual will be capable of-in both directions. Patients It is not uncommon for families to express goals, hopes, who look like they will make good recoveries languish; pa- and expectations for the person with the brain injury that, tients with severe impairments make achievements never The Family System 497 dreamed possible. Clinicians develop a set of expectations ents in a collaborative process of discovery to see how they on the basis of probabilities derived from experience. Some families, in the face of such explicit com- of deficit will not go back to work, then 5% will. How does parison (which they probably have never done), begin on one know if this family represents the exception and not their own to modify their expectations. Clinicians owe it to the family to keep their mit skepticism but are clear about wanting to move for- minds open. Other families may in fact be in full-blown denial- but again, contradicting them only fuels the denial (be- Principle #4: Never Underestimate Motivation. The process is then to families will succeed at what they put their minds to; it implement the first step with support, see how it goes, and does mean that clinicians should not short circuit the keep implementing steps as long as the person is succeed- power of families who have a strong need to achieve a goal ing. Ongoing monitoring and discussion are essential to until they have given themselves a chance to try. What assistance does she need on examina- the spirit of their goal, without necessarily endorsing the tions and papers? By breaking the “unrealis- down into steps and take one at a time, and find the spirit tic” goal down into steps, the professional can support of the goal and substitute reasonable alternatives. When it is in fact unrealis- practice, because families are often unrealistic about fu- tic, both the injured person and his or her family will grad- ture goals soon after brain injury, it is most often the case ually realize that and be more at peace with letting go of that the spirit of the goal is identified first and then broken the goal because they gave it their best shot. A bright (2) Find the spirit of the goal and substitute reasonable young woman in college had the (realistic) goal of becom- alternatives. Her parents believe it is still possible and satisfy the underlying need by substituting another, for her to succeed and want her to resume college and take more reasonable goal.

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Thyroid hormone deficiency during infancy causes both mental retardation and growth impairment buy tricor without a prescription hdl cholesterol ratio and risk, as discussed below order tricor ideal cholesterol ratio for an individual would include. Fortunately discount 160 mg tricor visa cholesterol free cheese, this rarely occurs today because thyroid hormone deficiency is usually detected in newborn infants and hormone therapy is given at the proper time. Animal studies have demonstrated that thyroid hormones inhibit nerve cell replication in the brain and stimulate the growth of nerve cell bodies, the branching of dendrites, and the rate of myelinization of axons. These effects of thyroid hormones are presumably a result of their ability to regulate the expression of genes involved in nerve cell replication and differentiation. For example, a person who is deficient in thyroid hormones and who does not receive thyroid hormone therapy during childhood will not grow to a normal adult height. If this condition occurs in a child, it will cause growth retardation (see Chapter 31). In tissues such as skeletal muscle, heart, and liver, thyroid hormones have direct effects on the synthesis of many structural and enzymatic proteins. For example, they stimulate the synthesis of structural proteins of mitochondria, as well as the formation of many enzymes involved in intermediary metabolism and oxidative phosphorylation. Thyroid hormones also promote the growth and calcification of bones in the skeleton. Deficiency in the thyroid hormone early in life leads to delayed, abnormal development of bone and can lead to dwarfism. The rate at which oxidative phosphorylation occurs is reflected in the amount of oxygen consumed by the body because oxygen is the final electron acceptor at the end of the electron transport chain. The total amount of oxygen consumed and body heat produced over a 24-hour period is the combination of that needed at rest and that needed during activity. Thyroid hormones regulate the basal rate at which oxidative phosphorylation takes place in cells. As a result, they set the basal rates of body heat production and of oxygen consumed by the body. Thyroid hormone levels in the blood must be within normal limits for basal metabolism to proceed at the rate needed for a balanced energy economy of the body. If thyroid hormones are present in excess, oxidative phosphorylation is accelerated and body heat production and oxygen consumption are abnormally high. The converse occurs when the blood concentrations of T and T are lower than normal. The thermogenic action of the thyroid hormones is poorly understood at the molecular level. The thermogenic effect takes many hours to appear after the administration of thyroid hormones to a human or animal, probably because of the time required for changes in gene expression. These novel uncoupling proteins may have a role in the thermogenic action of thyroid hormones. In addition to regulating the rate of basal energy metabolism, thyroid hormones influence the rate at which most of the pathways of intermediary metabolism operate in their target cells. When thyroid hormones are deficient, carbohydrate, lipid, and protein metabolism are slowed, and the response of these pathways to other regulatory hormones is decreased. In contrast, these metabolic pathways run at an abnormally high rate when thyroid hormones are present in excess. Thus, thyroid hormones can be viewed as amplifiers of cellular metabolic activity. The amplifying effect of thyroid hormones on intermediary metabolism is mediated through the transcription of gene-encoding enzymes involved in these metabolic pathways. Thyroid hormones provide negative feedback to control the hypothalamic–pituitary axis. An important action of the thyroid hormones is the regulation of their own secretion. This action of T on thyrotrophs is thought to be a result of changes in gene expression in these cells. These changes result from dysregulation of nervous system function and altered metabolism.

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