Alex J. Auseon, DO, FACC

  • Director, Cardiology Fellowship Program
  • Associate Professor of Clinical Internal Medicine
  • The Ohio State University Medical Center

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Neuronopathies are diseases of nerve cell bodies medicine woman cast donepezil 10 mg cheap, including motor neuron diseases such as spinal muscular atrophies or sensory ganglionopathies. In Western countries, the most common causes are diabetes mellitus and alcoholism. In Italy, a study of people 55 years and older documented a probable diagnosis of polyneuropathy in 4% and possible diagnosis in 7%. The most common risk factor was diabetes, present in 44% of patients diagnosed with probable polyneuropathy. Even after diagnostic workup, the etiology remains unknown in one third of patients. The primary insult to neuron or axon, often toxic or metabolic, causes degeneration in the distal parts of the nerve, most removed from the trophic influence of the nerve cell body. Failure to maintain the axonal transport results in Walleriantype degeneration distally with centripetal progression. Clinically, sensory symptoms begin in the toes and feet with numbness and paresthesias, often described as tingling, pinsandneedles sensation, or stabbing or burning pain. Neuropathic pain from small fiber involvement is suggested by the perception of pain to a nonnoxious tactile stimulation (allodynia) or increased pain response to noxious stimulation (hyperalgesia). The report of pain more bothersome at rest, such as during the night, usually suggests neuropathic origin. When the sensory deficit reaches to below knee level, the hands become affected and stockingandglove sensory deficit develops. Patients with advanced sensory neuropathy may show hypesthesia over the anterior chest and abdomen, typically when the sensory level in the legs reaches the midthigh. In general, sensory complaints predominate and motor deficits occur late in the course and are typically limited to the distal muscles. Patients may have weakness of toe/foot extensor muscles followed by interossei hand muscles, and atrophy of intrinsic foot and hand muscles. In a patient with this typical pattern who has a history or laboratory documentation of diabetes mellitus, alcohol abuse, B12 deficiency, uremia, or preceding exposure to a known neurotoxin or drug, further diagnostic workup is unlikely to change the diagnosis. Patients without an obvious cause or any patient with an atypical clinical presentation should undergo further testing, including electrophysiological testing. The clinical features of myelinopathies include proximal muscle weakness, the pattern of disproportionately mild muscle atrophy despite marked weakness, early loss of deep tendon reflexes, tremor, and enlarged nerves. A hereditary polyneuropathy usually has a chronic indolent course over years, and patients often have few or no complaints of paresthesias despite sensory deficit on examination. Patients with small fiber neuropathy have impairment of pain and temperature modalities and autonomic dysfunction. Autonomic symptoms include postural hypotension and syncope, heat intolerance due to impaired sweating, or coldness of the extremities. Bladder or bowel dysfunction is common, and erectile dysfunction occurs early in men.

Diseases

  • Ectodermal dysplasia Margarita type
  • Primary orthostatic tremor
  • Parkinson dementia Steele type
  • Marfanoid mental retardation syndrome autosomal
  • Cutaneous larva migrans
  • Osteonecrosis
  • Dentophobia
  • Achondrogenesis type 2
  • Chands syndrome
  • Beriberi

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Strap cells with eosinophilic cytoplasm medicine 5852 5 mg donepezil visa, occasionally with cross striations, may be seen, but are few in number. The cytoplasm contains abundant glycogen, often appearing as vacuoles and producing a characteristic "spider cell". The lack of significant infiltration of adjacent tissues; absence of cellular pleomorphism, mitoses, and necrosis; and the presence of muscle maturation at the periphery of the lesion are features indicative of a rhabdomyoma. Pediatric patients present at or within several months of birth with subglottic lesions that may result in potentially life-threatening airway obstruction and haemorrhage. In addition, about half of all pediatric patients with subglottic haemangiomas may have haemangiomas in other locations, most of which are cutaneous, rarely visceral. Grossly, haemangiomas are soft and compressible and range from red to blue, depending on the degree of vascularity. Microscopically, haemangiomas are categorized into capillary and cavernous types, and often demonstrate a lobular pattern of growth. Juvenile haemangiomas are usually cellular and of the capillary type while in adults, they are are more often cavernous. The granular cells are spindle-shaped and may be confused with a desmoplastic stromal reaction. But in the correct clinical setting of a positive family history of hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber syndrome), typical lesions (in any location) and episodic bleeding can help to define the syndrome. Vascular vocal cord polyps occur exclusively on the true vocal cord and are separated from haemangiomas by a large amount of extravascular fibrin. If the lesion is biopsied rather than excised, unusually excessive bleeding may give a clue as to the type of lesion encountered. Although the preferred treatment is laser excision, therapy has included expectant management, systemic steroids, intralesional sclerosing agents and surgical excision 277,439, 1235,1680,2227,2305,2591. The distinction between a cystic hygroma with involvement of the larynx versus a pri- mary lymphangioma underscores a semantic and clinical dilemma. The exact anatomic location and neck examination are needed to exclude a primary lymphangioma 659,1194,1235,1487. Of 30 cases, 84% occurred in women and the peak incidence was the fourth decade (range 6-56 years) 315. In adults, the most common site is the posterior half of the true vocal cord, while in children the subglottis is the site of predilection. Most patients have only a single tumour, but in 2-10% of individuals, multiple tumours may be found 924. Hoarseness is the usual presenting symptom of laryngeal tumours while stridor or airway obstruction (often mistaken for asthma) is characteristic of those in the trachea. Macroscopy the majority of tumours are firm, polypoid or sessile, and less than two centimetres.

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Here treatment for plantar fasciitis discount donepezil 5 mg line, purified allergen is injected subcutaneously on a weekly basis in increasing doses to patients with severe allergies. It is thought that IgGblocking antibodies, possibly of IgG4 class, may be involved although this remains controversial. Changes in the balance between Th1 and Th2 cells are also thought to be important. This approach is used for severe allergic rhinoconjunctivitis due to pollen, dust mite and animal exposure and anaphylaxis to bee and wasp venom (see Clinical Case Scenario 16. Repeated exposure leads to a change in the balance of Th1 and Th2 cells, the production of Treg cells and a gradual reduction in allergenspecific IgE balanced with an increase in allergenspecific IgG. Because his symptoms were not controlled by his current medical treatment, he was offered desensitizing immunotherapy. He opted for continuous sublingual therapy, rather than winter injections, and was given daily sublingual grass pollen tablets. After the first year of treatment, there was a marked improvement in his symptoms, and by the end of the 3year course, his symptoms were minimal and easily controlled by occasional antihistamine tablets. This involves working in the countryside, checking and repairing electricity pylons and cables. He has had summer hay fever since the age of 14 with typical itchy, gritty eyes, sneezing, blocked and runny nose and sinus pain. He had previously managed with regular nonsedating antihistamines and a nasal steroid spray. However, since starting his new job 2 years ago, his symptoms had become much worse, with increasing headaches and difficulty in concentration, which was affecting his work. He had required two courses of oral steroid tablets to settle his symptoms in the current pollen season. Chapter 16 Immunological therapy 209 less effective and requires much higher antigen doses to achieve effects. The identification of the precise antigens and their production in recombinant form will undoubtedly assist in the preparation of new and better immunotherapeutic agents. The use of antiIgE receptor monoclonal antibodies such as omalizumab may have potential in this respect and trials are under way. Vaccination to modify autoimmune disease has been used in experimental animal systems and early clinical trials, for example, in experimental autoimmune encephalomyelitis and multiple sclerosis, using either inactivated autoreactive T cells themselves as the vaccine or peptides derived from the Tcell receptors of myelin basic proteinreactive T cells. Oral or parenteral administration of autoantigens has been performed in attempts to reestablish tolerance.

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Clinical features Clinically medicine xalatan donepezil 5 mg purchase mastercard, acute onset of pain in one or more cranial or peripheral nerves is followed by motor and sensory deficit in the distribution of the affected nerve. The classic presentation is a focal mononeuropathy that does not seem to localize to one of the typical compression sites, especially if associated with pain and systemic features such as fever, weight loss, or rash. Within days or weeks, additional nerves are affected, resulting in progressive mononeuropathy multiplex. Large fiber involvement may lead to acute weakness or sensory loss from nerve infarction. With disease progression, the picture of distal asymmetric or symmetric polyneuropathy develops. Less commonly, vasculitic neuropathy presents primarily as symmetric polyneuropathy. Patients often present with signs of systemic involvement (fever, weight loss, anorexia). They should be carefully evaluated for evidence of underlying collagen vascular disorder and infection, including involvement of the skin (rash). Endorgan involvement may be present, depending on the underlying vasculitic condition. West Nile virus and cytomegalovirus, and the bacterial infections of syphilis and Lyme disease, cause painful polyradiculopathy. Electrophysiological testing reveals features of acute axonal nerve lesions; that is, reduced or absent compound motor action potentials and sensory nerve action potentials, with only minor changes in conduction velocity, distal motor latency, or Fwaves. Acute onset vasculitis neuropathy may result in focal nerve lesion temporarily mimicking conduction block due to the time required to result in distally reduced nerve excitability from Wallerian degeneration ("pseudoconduction block"). Nerve biopsy, typically the sural or superficial peroneal nerve, and muscle biopsy should be performed expeditiously in all patients with suspected vasculitis neuropathy. Perivascular and transmural mononuclear cell infiltrates with necrosis of the blood vessel walls and occlusion of epineural blood vessels are pathognomonic. Treatment Investigations All patients with suspected vasculitic neuropathy need to be evaluated urgently, due to rapid progression of the disease and response to treatment. Diagnosis is especially difficult in patients with isolated vasculitis of the peripheral nervous system, and depends on histological demonstration of characteristic findings on nerve and muscle biopsies. Testing for collagen vascular disease may indicate systemic vasculitis, with elevation of erythrocyte sedimentation rate, Creactive protein, and other features of inflammation. Highdose steroid therapy is also appropriate for postsurgical inflammatory neuropathy.

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Similarly 86 treatment ideas practical strategies discount 5 mg donepezil visa, rash and arthralgia were the most common signs during the Zika virus outbreak on Yap Island (14). Consistent with our findings, fever was present in <50% of confirmed cases in various studies (28,29). Fever in the mother may reflect a more inflammatory infection or may be unrelated to Zika virus and result from co-infection with other pathogens. Fever can also be associated with other conditions, such as premature rupture of membranes resulting from genital tract infection. Data from ongoing prospective cohort studies may help further elucidate this finding. Zika virus infection can lead 252 to birth defects and pregnancy complications even when the mother is asymptomatic, but this report contains only surveillance data. Women had to be symptomatic to trigger reporting to the MoH, and thus, it is not possible to estimate the burden of disease in asymptomatic women and their infants. The MoH prioritized and directed its limited resources to testing of symptomatic pregnant women during this relatively sudden and explosive epidemic. Fetal loss has been documented in an experimental animal model of marmoset monkeys. The inoculation of Zika virus into pregnant females caused prolonged fetal and placental viral replication and a maternal associated host response and increased activity of proinflammatory cytokines (30). In our study, women reported as symptomatic during the first trimester of pregnancy had 4 times greater odds of fetal loss that those with later symptoms, after controlling for maternal age. In Brazil, 13 (7%) fetal losses and 4 cases of microcephaly were reported among 186 women with known pregnancy outcome. Cesarean sections were more prevalent in Brazil compared with our cohort in the Dominican Republic (81% vs. However, many distinguishing clinical and laboratory features between dengue virus and Zika virus are familiar to clinicians adept at diagnosing dengue virus infection. Second, our assessment of birth defects is limited to visible abnormalities, such as microcephaly, in live births. Birth defects were not reported on fetal losses, and there were no radiographic, ophthalmologic, or audiologic assessments to ascertain inconspicuous birth defects. These centers are not equipped to evaluate brain radiographic abnormalities associated with Zika virus infection. Third, clinical data are missing for almost one third of the women and pregnancy outcome is not known in 39% of cases. Fourth, true disease burden in pregnancy is underestimated because we have no data for asymptomatic women not captured by passive surveillance.

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This area should be sampled in a maxillectomy specimen a) if the globe has not been removed (as the lamina papyracea represents the lateral orbital margin) 5 medications related to the lymphatic system purchase donepezil 5 mg, or b) if orbital exenteration has been performed. The roof of the ethmoid complex is formed by a medial extension of the orbital plate of the frontal bone, which projects to articulate with the cribriform plate. Sphenoid sinus the average adult sphenoid sinus measures 20 mm high, 23 mm long, and 17 mm wide. The relationship of the posterior extension of the sphenoid in relation to the sella turcica is variable. The sphenoid sinus septum is usually in the midline, and anteriorly aligned with the nasal septum. With the exception of the sinus roof, the other sinus walls are of variable thickness depending on the degree of pneumatization. The sinus roof relates to the floor of the anterior cranial fossa, anteriorly; the optic chiasm and. The lateral sphenoid wall is related to the orbital apex, the optic canal, the optic nerve, and the cavernous sinus, containing the internal carotid artery. The sinus floor is the roof of the nasopharynx, and the anterior sinus wall is the back of the nasal fossa. Behind the orbital rims, each sinus roof/orbital floor slants obliquely upward so that the highest point of the sinus is in the posteromedial portion, lying directly beneath the orbital apex. The medial antral wall is the inferior lateral wall of the nasal cavity ("party wall"). The anterior sinus wall is the facial surface of the maxilla that is perforated by the infraorbital foramen below the orbital rim. The floor of the sinus is lowest near the second premolar and first molar teeth and usually lies 3-5 mm below the nasal floor. This pattern of drainage in the erect position is accomplished by intact ciliary action. The maxillary hiatus is a bony window leading to the interior of the maxillary sinus. The hiatus is normally partially covered by portions of four bones: the perpendicular plate of the palatine bone, posteriorly; the lacrimal bone, anterosuperiorly; the inferior turbinate, inferiorly, and above the turbinate attachment, the uncinate process of the ethmoid bone. The margin (black arrow) of the tumour in the maxillary sinus is separable from the obstructed secretions because of different densities. The tumour (white arrow) extends through the roof of the ethmoid and along the roof of the orbit (white arrowhead).

Stoll Alembik Dott syndrome

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It is likely that other genetic or environmental factors have a significant role in determining age of onset medicine prescription drugs discount donepezil 5 mg without a prescription. Because of meiotic instability with a tendency of increasing the expansion size during spermatogenesis, juvenileonset cases with very large expansions usually have an affected father. Predictive genetic testing of asymptomatic at-risk relatives of affected patients is currently available and governed by international guidelines. Illness may emerge at any time of life, with the highest occurrence between 35 and 40 years of age. There is also a large variability in the clinical presentation and some of this variability is predictable; for example, the juvenileonset form may present with parkinsonism (the so-called Westphal variant), while the late-onset form may present with chorea alone. Motor impersistence is another important associated feature, whereby individuals are unable to maintain tongue protrusion or eyelid closure. Dystonia tends to occur when the disease is advanced or is associated with the use of dopaminergic medications. Dysphagia tends to be the most prominent in the terminal stage and aspiration is a common cause of death. Typically, the impairment begins as selective deficits involving psychomotor, executive, and visuospatial abilities and progresses to more global impairment, although higher cortical language tends to be spared. Although inherited in an autosomal dominant fashion, the symptoms are non-progressive with no alterations in cognitive or behavioral functions. The presence of sensorimotor neuropathy may suggest an alternative diagnosis of neuroacanthocytosis. The diagnosis is supported by the presence of acanthocytes on a peripheral blood smear in the context of appropriate clinical presentation. In general, treatment of chorea is not recommended unless it is causing disabling functional or social impairment. The disease tends to be progressive, with death occurring within 15 years of diagnosis. Involuntary choreic and dystonic movements of the orofacial region, as well as tongue and lip biting, are virtually diagnostic, although a full spectrum of movement disorders may be seen. Psychiatric symptoms are typical and include apathy, depression, anxiety, and obsessive-compulsive syndrome. Other agents including tetrabenazine and amantadine have been shown to improve chorea. Traditional neuroleptics such as haloperidol can improve chorea, but are associated with increased risk of tardive dyskinesia, dystonia, difficulty swallowing, and gait disturbances, and should not be considered as first-line agents. Valproic acid may be useful in the long-term management of aggression and irritability. Neuroacanthocytosis Diagnosis is usually made on the basis of family history, morphological analysis of erythrocytes, and an elevated plasma creatine kinase level. The most consistent neuropathological finding is extensive loss of predominantly small and medium-sized neurons and gliosis in the caudate, putamen, pallidum, and substantia nigra, with relative sparing of the subthalamic nucleus and cerebral cortex.

Recurrent laryngeal papillomas

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There are no data to suggest that one dopamine agonist is more efficacious than another treatment syphilis donepezil 5 mg cheap. However, an association has been reported between treatment with pergolide and the development of fibrotic valvular heart disease. Most published studies conclude that treatment with ergot dopamine agonists (pergolide and cabergoline in most cases), particularly at high daily doses and for periods of 6 months or longer, was associated with a substantially increased risk of newly diagnosed cardiac valve regurgitation. Levodopa has also been proven to be better at improving symptoms than dopamine agonists in numerous studies. However, patients will develop motor complications with longterm levodopa therapy. The gastric mucosa is another site of Pharmacotherapy for symptom control Pharmacotherapy for neuroprotection As previously indicated, no adequate clinical trials have provided definitive evidence for pharmacological neuroprotection. While many agents have appeared promising based on laboratory studies, the symptomatic effects of the study medications commonly confound the clinical neuroprotection end points in clinical trials. Rasagiline 1 mg per day met all three primary end points, consistent with the drug having a diseasemodifying Table 50. Nonergot derivatives - Pramipexole - Ropinirole - Piribedil - Rotigotine - Apomorphine b. Prevention of motor complications A number of strategies have been developed to prevent or delay the occurrence of motor complications, including dyskinesias and motor fluctuations characteristic of later stages of the disease. Motor complications can be simply divided into motor fluctuations and dyskinesias. Typically, patients may begin to experience a wearingoff (endofdose) effect, because the motor improvement after a dose of levodopa becomes reduced in duration over time and parkinsonism reappears. Subsequently, dyskinesias emerge at peak dose levels and are classically choreiform. The goal is to maintain dopaminergic stimulation to minimize "off " time while not accentuating dyskinesias. However, the strategies that accomplish this goal are increasingly more difficult as the disease progresses, requiring both the attention and pharmacological understanding of the treating physicians. In addition, compliance as well as therapeutic adherence is required of the patients, supported by their caregivers. Treatment of endofdose wearing off Endofdose wearing off is the most common and usually the first sign of motor complications. Patients develop a loss of response to a dose of medication before it is time to take the next dose, usually within 4 hours of the earlier dose. If they take their next dose of medication, their symptoms will improve again until the next dose begins to wear off. Treatment depends on the severity of the problem and on how dopaminergic therapy was initiated in the early stage of the disease.

Peters anomaly

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It causes major and recurring outbreaks of animal and human disease in subSaharan Africa and has recently spread to northern Africa treatment 0f ovarian cyst buy discount donepezil 5 mg, the Sinai and Arabian peninsulas. The virus is transmitted by both mosquito bites and contact with infected animal carcasses, abortion tissue and blood products. In most patients, infection presents with fever, nausea, vomiting, abdominal and respiratory symptoms. Less than 5% of patients develop meningoencephalitis, often late in the illness, with confusion, meningism, hyperreflexia, and depressed conscious state. Less than 8% of patients develop severe disease, characterized by hemorrhage, hepatitis, renal failure, retinitis, and encephalitis. Laboratory investigation shows elevated liver enzymes, hepatic and renal failure, and prolonged prothrombin and partial thromboplastin times. Powassan and deer tick virus encephalitis Powassan and deer tick viruses belong to the tickborne encephalitis serogroup of flaviviruses. They are found in the northern United States, Canada, and the Primorski region of Russia. Powassan virus is transmitted by Ixodes cookei and deer tick virus Ixodes scapularis. The viruses are also isolated from Dermacentor andersoni, Ixodes marxi, and Ixodes spinipalpis. Pathologically, these viruses cause widespread inflammation with perivascular and focal parenchymal lymphocytic infiltration with associated necrosis, especially in the cortical gray matter, and to a lesser degree the cerebellum, brainstem, and spinal cord, followed by gliosis. Patients with encephalitis suffer from drowsiness, confusion, seizure, ataxia, tremor, ocular symptoms, dysarthria, and respiratory failure. Australian encephalitis: Murray Valley encephalitis and Kunjin encephalitis Both Murray Valley encephalitis and Kunjin encephalitis are caused by closely related mosquitoborne flaviviruses endemic in Australia and Papua New Guinea. Murray Valley encephalitis can also be transmitted by Anopheles mosquitoes and, based on serological findings, other domestic and wild animals have also been implicated as hosts. Clinical Murray Valley encephalitis occurs in 1/1000 infections, while Kunjin virus infection is less common. Patients with Kunjin virus infection present with mild fever, headache, photophobia, rash, arthralgia, myalgias, lymphadenopathy, and, less often, encephalitis, exhibiting cranial nerve palsies, dyskinesia, tremor, parkinsonism, and flaccid paralysis due to anterior horn cell involvement; seizure may occur in children. It causes infrequent small outbreaks among horses and humans in Queensland and northern New South Wales in Australia. Like the Nipah virus, its natural reservoir is the Pteropus fruit bat, and the virus is transmitted to humans through the respiratory secretion of sick horses.

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Amul, 34 years: Persistent tics can occur many times a day, almost daily, or on and off throughout a period of greater than one year. When present, it should always arouse suspicion of secondary infection or malignant change. Inhibition of protein C, its cofactors, antithrombin activity, prothrombin, factor X, protein Z pathway, and plasmin diminishes coagulation factor inactivation and fibrinolysis. Interrelationships of undernutrition and neurotoxicity: Food for thought and research attention.

Flint, 44 years: Data supporting their utility is limited, however, and their use cannot be recommended as a standard of practice. Livestock largely consists of dromedary camels (Camelus dromedarius) and small ruminants (goats and sheep). The defensins (human neutrophil proteins1, 2, 3 and 4) are cyclic peptides of 2943 amino acids which insert into the membranes of their targets. Adult-onset focal dystonias the temporal pattern distinguishes between the great majority of dystonias that are continual.

Joey, 48 years: Malignant soft tissue tumours 35 Macroscopy Tumours are generally smooth, nodular or pedunculated (polypoid), with a number being fungating or ulcerating. Agranulocyto sis and subacute combined degeneration developed after repeated exposure. The Th2 phenotype drives the production of a number of cytokines, which promote the differentiation, activation, and survival of eosinophils. Main symptoms were defined as fever, maculopapular rash, arthralgia, and conjunctivitis.

Ronar, 54 years: The clinical spectrum of melioidosis ranges from the acute septicemic form, which is often fulminant, to chronic disease, which mimics other common infections like tuberculosis (2). Acute meningitis associated with fever and neck stiffness is a manifestation of secondary syphilis and may be associated with a rash. Previous monkeypox outbreaks occurred in Likouala Department, in 273 M Emerging Infectious Diseases In January 2017, two suspected monkeypox cases were reported in Moual village in Likouala Department of Republic of the Congo. Bands of oligoclonal IgG are present, nearly all of which are directed against measles virus antigen.

Hengley, 53 years: There was a modest, but not significant improvement in central conduction time, but no benefit clinically. Without appropriate treat ment, alcoholic optic neuropathy can lead to irreversible optic nerve atrophy. However, highdose intravenous corticosteroid treatment may be considered as an option in rapidly progressive cases as well as thiamine, folate, and other B vitamins (especially B12). The main improvement in dopamine agonists in the last few years has been the introduction of slowrelease preparations of oral dopamine agonists and patch formulations.

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References

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  • Lecumberri R, Marques M, Diaz-Navarlaz MT, et al. Maintained effectiveness of an electronic alert system to prevent venous thromboembolism among hospitalized patients. Thromb Haemost 2008;100(4):699-704.
  • Feldmann E, Broderick JP, Kernan WN, et al: Major risk factors for intracerebral hemorrhage in the young are modifiable, Stroke 36(9):1881-1885, 2005.
  • Barthel HR, Haselwood D, Longley S, 3rd, Gold MS, Altman RD. Randomized controlled trial of diclofenac sodium gel in knee osteoarthritis. Semin Arthritis Rheum 2009; 39(3):203-12.