By L. Lukjan. Syracuse University.

The disorder is relatively benign cheap propecia 1mg with visa hair loss cure4kids; the most common cause of death in adult patients with this disorder is intracranial bleeding Key Concept/Objective: To be familiar with the presentation propecia 1 mg visa hair loss in men zip up boots, course, associations, and platelet levels requiring treatment in cases of ITP The presentation of ITP as a chronic illness with a mild bleeding diathesis is not uncom- mon in adults. In most adult patients (90%), ITP will follow a course characterized by chronic thrombocytopenia; in most chil- dren, platelet counts will return to normal within 3 months. In patients with this finding, other causes of thrombocytopenia, such as hypersplenism associated with liver disease, should be considered. Other diagnoses to be considered in patients with ITP and splenomegaly are lymphoma and systemic lupus erythematosus (SLE). Although most cases of ITP are idiopathic, it is well known that ITP can be associated with a number of underlying conditions, including HIV, viral illnesses (e. In adults, in contrast to children, ITP can present as a smoldering disease. Therapy is recom- mended for asymptomatic patients whose platelet counts are persistently lower than 20,000 to 30,000/µl and for patients with significant bleeding complications. Although in most patients ITP follows a relatively benign course, serious bleeding complications are occasionally seen. Of these bleeding complications, intracranial bleeding is the most dreaded because it is commonly fatal. Intracranial bleeding is more commonly seen when the platelet count drops below 10,000/µl; other risk factors include advanced age and con- comitant medical illness. A 33-year-old woman is admitted to the hospital with altered mental status. Her physician calls you for consultation; he is concerned that the patient may have thrombotic thrombocytopenic purpura (TTP). He notes that she has anemia, thrombocytopenia, and a high fever and that she is disoriented. He asks your opinion regarding certain laboratory tests he has sent. Which of the following findings is NOT consistent with a diagnosis of TTP? Schistocytes and helmet cells seen on blood smear B. Neutrophilic leukocytosis Key Concept/Objective: To be able to recognize the laboratory abnormalities associated with TTP Patients with TTP typically present with the following pentad of signs and symptoms: (1) thrombocytopenia; (2) microangiopathic hemolytic anemia with schistocytes and helmet cells; (3) renal dysfunction, which is usually mild; (4) fever, which can be very high; and (5) neurologic symptoms, including seizures and a clouded sensorium. TTP is not associ- ated with a positive direct Coombs test; hemolysis is not immune mediated. This finding should make one consider autoimmune hemolytic anemias (such as those associated with Evan syndrome and SLE, both of which should be considered in the differential diagno- sis). The other findings are consistent with TTP and are helpful in establishing the diag- nosis and differentiating it from other causes of anemia and thrombocytopenia (such as disseminated intravascular coagulation). Prompt plasmapheresis should be instituted in patients with TTP. A dentist wishes to learn more about von Willebrand disease (vWD); he recently had a patient who has the disorder suffer excessive bleeding after dental work. In your discussion, you outline several key facts about vWD, including how these patients may require treatment before dental procedures and other surgeries. Results of coagulation studies are normal in patients with vWD B. Most cases are the result of impaired function of von Willebrand factor (vWF) D. An abnormal result on the ristocetin-induced platelet aggregation test is consistent with abnormal function of the patient’s vWF (type 2 vWD) E. It affects very few people Key Concept/Objective: To know some common facts about vWD vWD is the most common hereditary bleeding disorder. It may be the result of low levels of vWF (type I vWD, which accounts for 75% of cases); qualitative defects in vWF function (type II vWD), which can be determined by use of the ristocetin-induced platelet aggrega- tion test; or a severe or total deficiency of vWF (type 3 vWD, the least common form). The disorders of platelet function associated with vWD are not associated with decreases in platelet count.

Perhaps one-to-one attention purchase 1mg propecia hair loss on legs, or involvement with a sibling group would help propecia 5 mg without prescription hair loss 4 months after surgery. Rachel needs to have her own identity reaffirmed as an individual in her own right, and to achieve this she may need professional assistance, irrespective of whether her fits are a result of surgery or a cry for help. SUPPORT SERVICES AND BEING EMPOWERED / 115 A need for assessment I am concerned, also, following research into family support (Burke and Cigno 1996) that family attitudes towards a son or daughter with disabili- ties might result in a family becoming isolated from a community, which might otherwise support it. However, integration within the community depends largely on how the family adjusts to dealing with childhood dis- abilities, and in doing so professional help will be required to facilitate community involvement. Some families react to the experience of childhood disability by drawing away from the services that are available, making themselves increasing isolated, while others will welcome the attentions of specialist, voluntary services or neighbourly interest. Recognising the variety of family characteristics is part of the assessment process required when providing assistance. The fear is that those who most need help do not seek it. The ‘right’ to be included might be suppressed by negative family attitudes, whether from pride, lack of acceptance or some form of desperation, but the effect is that the efforts of concerned professionals will be rejected. Professionals must recognise that situations of neglect, where encountered, are within their remit to protect and provide for, helping families to overcome their own resistance to change. Professionals should also understand that some families might need coaxing to receive the help they need. This might be because they believe they ‘have to deal with their own problems’ and view the seeking of assistance as an admission of failure. The siblings of children with disabilities have then a double obstacle to overcome – the legacy of parental attitudes which might reject help when it is needed and the fact that a disabled child will, inevitably, tend to receive more attention from parents, leaving siblings as secondary carers, whose childhood will include some element of neglect. This is not true for all families but it is the experience of some, and within the scope of an assessment it is necessary to identify the needs of all family members. The needs of disabled children and siblings should be considered. Equally, the framework for practice (Department of Health, 2000a) will help the implementation of such an approach, but it has its limitations, for reasons that I shall explain. This consists of (i) child’s developmental needs, (ii) parenting capacity, and (iii) family and environmental factors. The framework is understandably child-focused within the family context, with the view that children must be assessed according to their needs. The difficulty is that siblings may not be singled out for assessment independ- ently but are considered in part only, owing to the assessment of the child with disabilities or, indeed, the child ‘in need’. Rather than allowing a degree of uncertainty to exist, it is necessary to include a fourth side to the assessment triangle, a new one to include siblings. Consequently, there should follow, ‘(iv) the needs of siblings’. Siblings should be identified independently, to signify their role and importance within the family. The needs of the child with a disability will be considered independently but not separately from those of siblings, and both assessments should include a holistic view to reflect the situation of the family. This may be what most professionals will attempt, as part of good practice, when assessing a family situation, except, to repeat the point, the needs of siblings are not made sufficiently clear within the framework. It may be stating the obvious to express this omission; nevertheless, it needs this degree of clari- fication if siblings are not to risk a form of professional exclusion. Power and independence Empowerment requires a cultural change of view within ‘normal’ non-disabled society as well as a proactive form of help for people who consider themselves disabled. Disability carries its own stigma, sometimes accepted by people with disabilities themselves: the need is to view disability as a positive attribute within the mainstream of society. Gaining control of one’s own life is a basic right we all share and, as Oliver (1990) argues, the person who is disabled is the best person to describe what their needs are. Self-empowerment enables that process, as disabled children articulate their needs and, as they grow up, reject well-intentioned, pitying attitudes disguised as caring. Siblings are often SUPPORT SERVICES AND BEING EMPOWERED / 117 caught up in this process, effectively experiencing disability in a secondary form, which will influence their views of their position in society; unfortu- nately, this will often carry ‘the stigma’ so frequently associated with disability in the past; in a literal sense, it is disability through a close rela- tionship, becoming disabled by association.

The cortico-bulbar fibers do not form a includes the somatosensory nuclei purchase propecia 1mg fast delivery hair loss solutions for women, the nuclei single pathway generic 1mg propecia with visa hair loss in men 80s costumes. The fibers end in a wide variety of nuclei cuneatus and gracilis (see Figure 33). There is of the brainstem; those fibers ending in the pontine nuclei also cortical input to the periaqueductal gray, are considered separately (see Figure 48). These axons course via the internal capsule and continue into the cerebral CLINICAL ASPECT peduncles of the midbrain (see Figure 26). The fibers Loss of cortical innervation to the cranial nerve motor involved with motor control occupy the middle third of nuclei is usually associated with a weakness, not paralysis, the cerebral peduncle along with the cortico-spinal tract of the muscles supplied. For example, a lesion on one side (described with the previous illustration; see Figure 48), may result in difficulty in swallowing or phonation, and supplying the motor cranial nerve nuclei of the brainstem often these problems dissipate in time. A patient with such a lesion will • Cranial Nerve Nuclei: The motor neurons of be able to wrinkle his or her forehead normally on both the cranial nerves of the brainstem are lower sides when asked to look up, but will not be able to show motor neurons (see Figure 8A and Figure 48); the teeth or smile symmetrically on the side opposite the the cortical motor cells are the upper motor lesion. Because of the marked weakness of the muscles neurons. These motor nuclei are generally of the lower face, there will be a drooping of the lower innervated by fibers from both sides, i. This will also affect nucleus receives input from both hemispheres. The portion of the facial This clinical situation must be distinguished from a nucleus supplying the upper facial muscles lesion of the facial nerve itself, a lower motor neuron is supplied from both hemispheres, whereas lesion, most often seen with Bell’s palsy (a lesion of the the part of the nucleus supplying the lower facial nerve as it emerges from the skull); in this case, the facial muscles is innervated only by the movements of the muscles of both the upper and lower opposite hemisphere (crossed). In some indi- nucleus may or my not receive innervation from the cortex viduals, there is a predominantly crossed of both sides or only from the opposite side makes inter- innervation. A lesion affecting the hypoglossal nucleus fibers influence all the brainstem motor nuclei, or nerve is a lower motor lesion of one-half of the tongue particularly the reticular formation, including (on the same side) and will lead to paralysis and atrophy the red nucleus and the substantia nigra, but not of the side affected. The cortico-retic- © 2006 by Taylor & Francis Group, LLC Functional Systems 127 Fronto-pontine fibers Cortico-bulbar (and Temporo-pontine fibers cortico-spinal) fibers Parieto-pontine fibers Occipito-pontine fibers FIGURE 46: Cortico-Bulbar Tracts — Nuclei of the Brainstem © 2006 by Taylor & Francis Group, LLC 128 Atlas of Functional Neutoanatomy intermingled with the lateral cortico-spinal tract (see Fig- FIGURE 47 ure 68 and Figure 69). RUBRO-SPINAL TRACT The rubro-spinal tract is a well-developed pathway in some animals. In monkeys, it seems to be involved in flexion movements of the limbs. Stimulation of this tract VOLUNTARY/NONVOLUNTARY MOTOR in cats produces an increase in tone of the flexor muscles. CONTROL NEUROLOGICAL NEUROANATOMY The red nucleus is a prominent nucleus of the midbrain. It gets its name from a reddish color seen in fresh dissec- The location of this tract within the brainstem is shown tions of the brain, presumably due to its high vascularity. The tract is said to continue throughout the tion with large neurons more ventrally located. The rubro- length of the spinal cord in primates but probably only spinal pathway originates, at least in humans, from the extends into the cervical spinal cord in humans. The fibers of CN III (oculomotor) exit through the The red nucleus receives its input from the motor areas medial aspect of this nucleus at the level of the upper of the cerebral cortex and from the cerebellum (see Figure midbrain (see Figure 65A). The cortical input is directly onto the projecting cells, thus forming a potential two-step pathway from motor CLINICAL ASPECT cortex to spinal cord. The rubro-spinal tract is also a crossed pathway, with The functional significance of this pathway in humans is the decussation occurring in the ventral part of the mid- not well known. The number of large cells in the red brain (see also Figure 48 and Figure 51B). The tract nucleus in humans is significantly less than in monkeys. The fibers then course in the lateral portion adequately described. Although the rubro-spinal pathway of the white matter of the spinal cord, just anterior to and may play a role in some flexion movements, it seems that the cortico-spinal tract predominates in the human. The role of this circuit in motor control will be explained with the cerebellum (see CRANIAL NERVE NUCLEI Figure 54–Figure 57).

You make a diagnosis of systemic lupus erythematous buy propecia 5mg fast delivery hair loss 18 year old male, a disease in which autoimmunity is known to play a central role buy generic propecia 5mg on line hair loss cure coming. Which of the following is NOT a possible mechanism of tolerance? Failure of T cells bearing low-affinity receptors to recognize antigens in the periphery C. Sequestration of an antigen from the immune system as a result of anatomic barriers D. Acquisition of anergy after ligation of the T cell receptor complex in the absence of costimulation E. T cells in the thymus with high affinity for a self-antigen undergo pos- itive selection Key Concept/Objective: To know the mechanisms of tolerance Immature lymphocytes are more susceptible to induction of tolerance. Tolerance can be induced in immature lymphocytes either centrally (thymus for T cells, bone marrow for B cells) or in the periphery. If a T cell has a high affinity for a self-antigen in the thymus, it can undergo negative selection with activation-induced death (apoptosis). Positive selec- tion in the thymus occurs when T cells bearing receptors with a moderate affinity for self- antigens receive survival and maturation signals and are exported to the periphery. Normally, these cells do not cause autoimmune phenomena because of peripheral toler- ance. The most common mechanism of peripheral tolerance is the failure of T cells bear- ing low-affinity receptors to recognize an antigen. T cells bearing receptors with high affin- ity for an antigen can remain in an inactivated state if that antigen is sequestered from the immune effector cells. Apoptosis is also a mechanism of peripheral tolerance. A third mechanism of peripheral tolerance involves the acquisition of anergy after ligation with the T cell receptor complex. The most extensively characterized mechanism of anergy induction occurs when the T cell receptor is ligated in the absence of costimulation. Another mechanism of tolerance is T cell-mediated suppression, in which regulatory T cells actively inhibit an immune response to an antigen. You are asked to see a 34-year-old pregnant woman in the emergency department who is experiencing shortness of breath. She has pulmonary edema, and an echocardiogram shows mitral stenosis. When asked, she says that many years ago, she had an illness with rash, fever, and 8 BOARD REVIEW joint pain that kept her in bed for a few weeks. On the basis of this history, you make a presumptive diagnosis of rheumatic mitral stenosis. Which of the following constitutes the best immunologic causative mechanism of rheumatic fever? Antistreptocococcal antibodies cross-reacting with myocardial antigens C. Toxins released by group A Streptococcus that cause valvular damage D. Pathogenic autoantibodies directed against the endocardium of heart valves Key Concept/Objective: To understand the mechanism of molecular mimicry In rare cases, the normal immune response to a specific microbial peptide can trigger immunity to a related self-peptide, a phenomenon known as molecular mimicry. This mechanism has been described in rheumatic heart disease and Lyme disease. In the case of rheumatic heart disease, antistreptococcal antibodies cross-react with myocardial anti- gens. Pathogenic autoantibodies have been clearly associated with myasthenia gravis, pemphigus, and a number of endocrinopathies but not rheumatic fever. A 35-year-old woman with a history of asthma and atopic dermatitis presents to your office for follow- up.

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