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By: V. Peer, M.A.S., M.D.

Vice Chair, Sidney Kimmel Medical College at Thomas Jefferson University

Diagnosing -thalassemia major: - - Thalassemia Minor: aka heterozygous -chain thalassemia - - - - these patients are usually asymptomatic Mild microcytic anemia is usually the only finding Diagnosing is also with hemoglobin electrophoresis Since this condition is asymptomatic sleep aid oriental yoga music purchase provigil amex, no treatment is necessary Hemoglobin electrophoresis will show an elevation of HbF Peripheral blood smear will show a microcytic hypochromic anemia -Thalassemias: Silent Carriers: this form is caused by a mutation or deletion of only one locus insomnia by faithless generic provigil 100 mg line. As a result sleep aid sonata discount provigil 200 mg free shipping, "ringed sideroblasts" are created and can be seen on peripheral smear insomnia causes buy provigil uk. If acquired, causes such as alcohol, isoniazid, chloramphenicol, lead exposure, collagen vascular disease, and myelodysplastic syndromes should be explored. Since stores of B12 can last for 3 years in the liver, there is usually not an dietary insufficiency. Competition from organisms (diphyllobothrium latum ­ the fish tapeworm) can cause B12 deficiency Usually normocytic/normochromic, however at times may be microcytic and hypochromic. Management of this condition involves treatment/management of the underlying condition. Commonly the patient eats the "tea and toast" diet the best sources for folate are green leafy vegetables Other common causes aside from dietary insufficiency are: alcoholism, pregnancy, folate antagonists, hemolysis, hemodialysis. Intrinsic ­ these are factors that are hereditary in nature, including: Sickle cell disease, thalassemias, hemoglobin C disease 2. Extrinsic ­ there are acquired factors causing hemolysis, including: Immune regulated hemolysis, mechanical hemolysis (prosthetic heart valves), toxic insults (drugs, poisons, etc). If the hemolysis occurs within the reticuloendothelial system, "extravascular hemolysis" occurs. Pneumo, Neisseria) а Give patient pneumococcal vaccine, Hib vaccine, meningococcal vaccine. Vaso-occlusive crisis causing severe pain (due to microcirculation obstruction by sickled red blood cells) Splenic sequestration crisis What is a splenic sequestration crisis? Blood pools into the spleen, resulting in splenomegaly and the subsequent development of hypovolemic shock. There are two possible causes for this, IgG antibodies or IgM antibodies IgG causes а "warm" autoimmune hemolytic anemia. This causes extravascular hemolysis where the primary site of sequestration is the spleen. This causes intravascular hemolysis and complement activation, where the primary site of sequestration is the liver. Diagnosing: - Direct Coombs test: If +ve = warm, if ­ve = cold Treatment: If mild, no treatment is necessary. Signs/Symptoms: - - - Altered mental status Hemolytic anemia Thrombocytopenia Petechial bleeding (pinpoint bleeding) Mucosal bleeding (ie epistaxis, menorrhagia, hemoptysis) Excessive bleeds after injury and/or surgical procedures Treatment: Plasmapharesis is required to maintain life, corticosteroids and splenectomy may also be required. IgG antibodies adhere to and destroy the platelets which are then removed by splenic macrophages. Acutely а Is a self-limited condition seen in children, where the condition is almost always preceeded by a viral infection. The platelet count will be low with abnormally large platelets on peripheral smear. There is also an activation of fibrinolytic mechanisms, thus leading to hemorrhages. Vitamin K deficiency is seen in very ill patients who are being fed through a tube, as well as those who are using oral warfarin as an anti-coagulant. If patient has a severe bleed, fresh frozen plasma should be given as it contains all of the clotting factors. Prognosis dependent on amount of lymphocytes and Reed-Sternberg cells (best when increased lymphocytes and decreased Reed-Sternberg cells). Progression: Fatty Streak а Proliferative Plaque а Complex Atheroma Adverse Effects: - - - - - Ischemia Infarction Peripheral vascular disease Thrombus Emboli Locations: Most commonly in the abdominal aorta, coronary arteries, popliteal arteries, and carotid arteries. In children, the most common tumor is the "Rhabdomyoma" and is associated with tuberous sclerosis. In young people, the most commonly encountered valve disorders are: Mitral prolapse, mitral stenosis, or bicuspid aortic valves. There are two mechanisms by which this condition can cause a paroxysmal tachycardia: 1. Supraventricular Tachycardias ­ All impulses get through to the ventricle in this condition, whereas in normal circumstances only one Atrial impulse gets through. Orthodromic Reciprocation Tachycardia ­ creates a re-entry loop that causes multiple depolarizations of the atria. Common causes of sinus bradycardia are: Ischemia, Increased Vagal Tone, Anti-arrhythmics ** Patients are usually symptomatic and may present with only fatigue ** Atropine will block vagal stimulation and thus elevate the sinus rate.

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The most promising bisphosphonates in clinical use include alendronate insomnia mayo clinic purchase 100mg provigil free shipping, etidronate insomnia funny quotes generic provigil 100mg free shipping, risedronate sleep aid kirkland order provigil visa, and zoledronate sleep aid child purchase provigil 100mg with visa. These bisphosphonates potently inhibit bone resorption without significantly affecting bone deposition. The main side effects of oral bisphosphonates occur in the gastrointestinal tract. Nausea and diarrhea occur in 20­30% of patients treated with high doses of etidronate, but are rarely seen with the lower doses that are used for the treatment of otosclerosis. Amplification-Most patients with otosclerosis have normal cochlear function with excellent speech discrimination and are therefore good hearing aid candidates. Before proceeding with surgery, patients should be encouraged to try a hearing aid (or aids). Some patients become successful hearing aid users and can therefore avoid surgery and its risks. However, although there is no risk to the patient with hearing aid use, there are some significant disadvantages when compared with the result of a successful surgery. The disadvantages include a poorer sound quality, cosmesis, cost, maintenance requirements, being able to hear only when the aid is in use, occlusion effect, and comfort. In practice, most patients under age 60 with good sensorineural reserve prefer to have surgery, but there are also many satisfied hearing aid users in this population. However, the efficacy of these agents has not been definitively proved, and otologists vary widely in their recommendations regarding the use of these medications. Sodium fluoride therapy-Fluoride reduces osteoclastic bone resorption and increases osteoblastic bone formation. Together, these actions may promote recalcification and reduce bone remodeling in actively expanding osteolytic lesions. Sodium fluoride is also thought to inhibit proteolytic enzymes that are cytotoxic to the cochlea and that may lead to a sensorineural hearing loss. Fluoride therapy has been found to significantly arrest the progression of sensorineural hearing loss in the low and high frequencies. Sodium fluoride is typically dosed at 50 mg daily in a patient with evidence of active disease. With stabilization, as evidenced by hearing stabilization, reduced tinnitus, reduced dizziness, fading of an injected mucous membrane over an active focus (Schwartze sign), and radiologic signs of recalcification, a daily maintenance dose of 25 mg is administered. Fluoride therapy is contraindicated in patients with chronic nephritis and chronic rheumatoid arthritis, as well as in pregnant and lactating women, children, and patients with a demonstrated allergy to fluoride. Gastrointestinal disturbances are the most common adverse effect of fluoride therapy. Taking enteric-coated capsules after meals largely prevents these adverse effects. Because there is also the rare possibility of skeletal fluorosis, a skeletal survey should be taken at intervals during the treatment. Many otologists recommend the use of sodium fluoride in patients with new-onset otosclerosis, rapidly progressive disease, or inner ear symptoms such as sensorineural hearing loss and dizziness. Patients with cochlear otosclerosis may be treated for longer periods of time or even indefinitely. Bisphosphonates-Bisphosphonates are potent antiresorptive agents that are useful for the prevention and treatment of osteoporosis and other conditions characterized by increased bone remodeling. Indications for surgery-Most patients with conductive hearing loss due to otosclerosis can be treated surgically (Table 51­3). The average patient with otosclerosis and a bone conduction level of 0­25 dB in the speech range and an air conduction level of 45­65 dB is a suitable candidate for surgery. An air-bone gap of at least 15 dB and discrimination scores of 60% or better are preferred. Surgery may be inadvisable in individuals whose occupation or activities demand considerable physical strain or precise balance (eg, pilots, scuba divers, and construction workers).

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Computer-controlled semiautomatic grid perimetry devices such as the Octopus or Humphrey field analyzer are used to examine the central 30 degree field of vision (modern campimetry; sleep aid antidepressant cheap 100 mg provigil with amex. Reproducible visual field findings are important in follow-up to exclude any enlargement of the defects sleep aid keeps me awake order provigil american express. Peripheral optic cup in a temporal and inferior location (with damage to the optic nerve fibers in this area) insomnia 2 trimester pregnancy cheapest provigil. Advanced generalized thinning of the neuroretinal rim with an increasingly visible lamina cribrosa and nasal displacement of the blood vessels insomnia 08 electro remix discount 100 mg provigil mastercard. Total glaucomatous atrophy of the optic nerve: Complete atrophy of the neuroretinal rim, kettleshaped optic cup, bayonet kinks in the blood vessels on the margin of the optic disk, some of which disappear. The optic disk is surrounded by a ring of chorioretinal atrophy (glaucomatous halo) due to pressure atrophy of the choroid and lysis of the retinal pigmented epithelium. The arc-shaped scotoma has expanded into a ring-shaped scotoma surrounding the focal point. As the focal point degenerates, the center of vision disappears and only a peripheral residual field of vision remains. The standardized examination conditions in automatic perimetry not only permit early detection of glaucoma; the reproducible results also aid in the prompt diagnosis of worsening findings. In addition to the early progressive optic nerve and visual field defects, arcshaped defects also occur in the nerve fiber layer. The angle of the anterior chamber characteristically remains open throughout the clinical course of the disorder. Epidemiology: Primary open angle glaucoma is by far the most common form of glaucoma and accounts for over 90% of adult glaucomas. The incidence of the disorder significantly increases beyond the age of 40, reaching a peak between the ages of 60 and 70. Patients with a positive family history are at greater risk of developing the disorder. Etiology (See also physiology and pathophysiology of aqueous humor circulation): the cause of primary open angle glaucoma is not known, although it is known that drainage of the aqueous humor is impeded. The primary lesion occurs in the neuroretinal tissue of the optic nerve as compression neuropathy of the optic nerve. Symptoms: the majority of patients with primary open angle glaucoma do not experience any subjective symptoms for years. However, a small number of patients experience occasional unspecific symptoms such as headache, a burning sensation in the eyes, or blurred or decreased vision that the patient may attribute to lack of eyeglasses or insufficient correction. The patient may also perceive rings of color around light sources at night, which has traditionally been regarded as a symptom of angle closure glaucoma. Primary open angle glaucoma can be far advanced before the patient notices an extensive visual field defect in one or both eyes. It is crucial to diagnose the disorder as early as possible because the prognosis for glaucoma detected in its early stages is far better than for advanced glaucoma. Where increased intraocular pressure remains undiagnosed or untreated for years, glaucomatous optic nerve damage and the associated visual field defect will increase to the point of blindness. Elevated intraocular pressure in a routine ophthalmic examination is an alarming sign. Fluctuations in intraocular pressure of over 5 ­ 6 mm Hg may occur over a 24-hour period. The angle of the anterior chamber is open and appears as normal as the angle in patients without glaucoma. Examination of the optic nerve reveals whether glaucomatous cupping has already occurred and how far advanced the glaucoma is. Where the optic disk and visual field are normal, ophthalmoscopic examination of the posterior pole under green light may reveal fascicular nerve fiber defects as early abnormal findings. Noise field perimetry is suitable as a screening test as it makes the patient aware of scotomas and makes it possible to detect and describe them. The patient is shown a flickering monitor displaying what resembles image noise on a television set.

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Syndromes

  • Your child has a high fever or severe pain or seems sicker than is usual for an ear infection.
  • A humpback, or kyphosis
  • Speech problems
  • Loss of consciousness
  • You have symptoms of this disorder
  • Thin, wide mouth

In dark races there is intense selection for dark hair sleep aid for teens best 200mg provigil, but in Caucasians there is no strong selection in favour of any particular colour insomnia 4 days discount 200mg provigil visa. The inheritance of red hair appears to be dominant to its absence and hypostatic to brown and black (Wassermann sleep aid visuals buy discount provigil 100mg line, 1974) sleep aid doxylamine buy provigil now. A major gene for red hair has been assigned to chromosome 4 and there is evidence that a major locus for brown hair is located on chromosome 19 (Ortonne and Prota, 1993). Dark hair predominates in the world, with black and dark brown hair generally associated with the dark-skinned races (Wassermann, 1974), although blonding among these people, for example the Australian aborigines, is not unknown (Abbie and Adey, 1953). There have been suggestions that it is possible to determine the racial origin of an unknown hair from a microscopical examination of its pigmentation (see Seta et al. According to Wassermann (1974) genital hair is usually lighter than scalp hair and often a reddish tint will be seen even in brown pubic hair. In fairhaired individuals, however, the pubic hair, axillary hair, eyebrows and eyelashes are much darker than the scalp hair (Rook and Dawber, 1982). The fair hair of some children will gradually darken as they grow up, and may eventually become brown. Greying or whitening of the hair is known as canities and occurs in all persons to some extent. The age of onset is variable and hereditary to some extent (Rook and Dawber, 1982). About 50 per cent of (Australian) people are at least 50 per cent grey at 50 years old, irrespective of sex or hair colour (Keogh and Walsh, 1965). In Negroes the onset occurs on average about ten years later than in Caucasians (Rook and Dawber, 1982). Onset is often noticed earlier in dark-haired individuals because of the greater contrast between the dark and the white hairs. White hair has no melanin pigment (Montagna and Parakkal, 1974) and the white colour seen is the result of the light interacting with the keratin protein and the physical structure of the hair. It is important to recognize that visible existing hairs do not change colour in the greying process. Rather, the process can occur only by reducing, or ceasing, the pigmentation in growing hairs while they are still being formed in the follicle, or by the production in subsequent hair cycles of hairs of reduced or no pigmentation (Montagna and Parakkal, 1974). The explanations, therefore, of stories of hair going grey overnight or going white due to worry usually lie in the sudden or rapid loss of dark hairs (see section 1. Likewise, reports of returning colour most likely result from the growth of new pigmented hairs (Rook and Dawber, 1982). The cellular processes which cause greying have not been elucidated as yet but are the subject of various lines of research. Animal studies suggest that heredity is a significant factor (Ortonne and Prota, 1993). Menkes kinky hair syndrome, an X-linked recessive inherited progressive brain disease, causes (among other defects) the hair to be a generally lighter colour. The lightening of colour is the result of impaired copper absorption which consequently reduces the activity of follicular tyrosinase, for which it acts as a coenzyme, and this affects melanin production (Comaish, 1981). In kwashiorkor, a severe protein-calorie deficiency, the hair is also lightened and may become golden, blond, rusty or light brown, in general or in patches (Desai et al. These colour changes can be used diagnostically but they are not necessarily seen in all cases (Bradfield, 1981). Alopecia diffusa: diffuse, generalized loss of scalp hair as may occur as a result of fever, malnutrition, anaemia, glandular problems or the use of drugs. Alopecia, male pattern: the term sometimes used to denote common baldness, the condition of degeneration and loss of scalp hair seen to some extent in most people of older years. It is a condition which is initiated in the scalp by the action of androgens and is also known as androgenic alopecia. Alopecia, traction: loss of hair as may arise from the strong and sustained or repeated tension or traction on hair follicles. Apocrine gland: a type of sweat gland of uncertain function which forms with some follicles. Arrector pili muscle: (also erector pili muscle) smooth muscle attached to the posterior side of the hair follicle and which runs up at an angle to the papillary layer of the dermis.

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