Clinical Manifestations

The clinical manifestations of sickle cell disease involve every organ system (Table 1). The most common presentations are acute pain crises, cerebral vascular accidents (CVA), acute chest syndrome (ACS), cholelithiasis, and splenic infarctions. Acute pain crisis is an episode of acute pain not caused by any other factor than sickle cell. It occurs in the vast TABLE 1 Systemic Features of Sickle Cell Anemia TABLE 1 Systemic Features of Sickle Cell Anemia Chronic hepatitis (secondary to multiple...

Lymphoma Involving Specific Head and Neck Sites

Lymphoma involving the thyroid gland is rare, accounting for only 2 to 3 of all cases of lymphoma and less than 10 of thyroid malignancies. Women are affected more frequently than men are by a ratio of 2.7 1. The median age at presentation is over 60 years. The most common subtypes are DLBCL and follicular Grade-3 lymphoma, accounting for at least 80 of cases. A rare but interesting subtype is marginal zone B-cell lymphoma, which arises from MALT. In many cases, this lymphoma...

Inflammatory Autoimmune

Pemphigus is a rare disease that affects mucosal membranes. Although the term pemphigus may be erroneously used interchangeably with the condition bullous pemphigoid, it is a separate entity warranting a separate discussion. Pemphigus is characterized by vesicular lesions and bulla. There are multiple subtypes, with pemphigus vulgaris and pemphigus foliaceus being the most common (7). Other subtypes include pemphigus vegetans, pemphigus erythematosus, pemphigus herpetiformis,...

Infectious Diseases Mycobacterial Infection

The incidence of mycobacterial infections has undergone a varied course over the past 50 years. For an approximate 30-year period following 1950, the incidence in mycobacterial infection declined annually in excess of 5 . This was followed by a paradoxical sharp increase at the rate of approximately 20 during the late 1980s and early 1990s, due to multiple factors such as acquired immunodeficiency syndrome (AIDS), drug resistance, and population influx from endemic areas. With improved...

Neuromuscular Disorders

Many neuromuscular diseases cause dysphagia in fact, the presenting symptom is often dysphagia. This is not surprising, considering the complex neuromuscular coordination required to execute a normal swallow. Although stroke patients develop sudden dysphagia, patients with other degenerative and nondegenerative neuromuscular diseases have a more insidious onset of dysphagia. Motor neuron diseases causing bulbar palsy or pseudobulbar palsy usually develop a progressive dysphagia and dysarthria...

Erythema Multiforme

EM is an acute, widely distributed hypersensitivity reaction associated with circulating immune complexes that are deposited in the basement membranes of the superficial vessels of the skin and mucosa. Subsequent complement activation produces vasculitis and thrombosis, leading to tissue ischemia and necrosis of the adjacent epithelium. The intensity of the skin and mucosal reaction varies from a localized minimal erythematous FIGURE 40 Erythema multiforme. Young adult male with rapid onset of...

Allergy and Dermatitis

There is clear evidence that otitis media with effusion is highly related to an allergic diathesis. When this converts to chronic draining otitis media, the allergic component would seem to still be relevant, although direct evidence is scant (17-19). Therefore, the surgeon must consider allergy evaluation, based on a patient history of other allergic diatheses, especially of the unified respiratory epithelium. Patients with chronic draining ear and allergic rhinitis, chronic rhinosinusitis,...

Angioedema

Angioedema is an uncommon and usually self-limited swelling of the deep dermis. It can, however, be complicated by potentially serious adverse consequences, including death. Although IgE-mediated mechanisms are not the major cause of angioedema, they are among the few that can be clearly identified, and thus this topic will be discussed here. Angioedema occurs together with urticaria approximately 85 of the time in 15 of instances, it occurs alone. Angioedema (Fig. 1) is the abrupt and...

Pemphigus And Pemphigoid

Pemphigus and pemphigoid are autoimmune diseases that produce blistering of skin and or mucosa. In pemphigus, intraepithelial blistering is caused by destruction of desmogleins that connect epithelial cells. Its most common variant, pemphigus vulgaris, begins with ulcerating lesions in the mouth, as the outer epithelium of the blister sloughs. Later, skin is also involved. In pemphigoid, the basement membrane is attacked, resulting in subepithelial blisters. Mucosal involvement nearly always...

Congenital Syphilis

Fetal infection with syphilis is highly dependent on the mother's stage of disease. Mothers with untreated primary or secondary disease are extremely likely to pass the infection, while those with latent disease have rates of transmission as low as 2 . Appropriate treatment of the mother during pregnancy generally prevents fetal infection. When infection does occur, the outcome can range from spontaneous abortion to a healthy-appearing child at birth with only latent infection. Similar to...

Crohns Disease and Ulcerative Colitis

Department of Medicine, Division of Gastroenterology, Inflammatory Bowel Disease Center, Mount Sinai School of Medicine, New York, New York, U.S.A. Clinical Manifestations 299 Crohn's disease CD and ulcerative colitis, collectively part of the inflammatory bowel diseases IBD , are both diseases of the gastrointestinal tract that arise in genetically susceptible individuals. Both CD and ulcerative colitis, though clinically different entities, are thought to be part of a spectrum of diseases...

Porphyrias

Porphyrias are a group of metabolic disorders characterized by defects in the synthesis of heme, a metalloporphyrin that is the product of porphyrin metabolism. Presentation of the disorder typically involves abdominal pain, vomiting, constipation, hypertension, tachycardia, photosensitivity, psychosis, and neuropathy. Neuropathy is estimated to affect 10 to 40 of patients and is primarily motor 126 . Cranial neuropathies have been described but facial paralysis is rare 127 . Although the...

Tuberculosis

Tuberculosis is caused by an infection with the bacterium Mycobacterium tuberculae. In 2004, 14,517 cases of tuberculosis were reported in the United States 17 . The infection is spread via respiratory droplets. Pulmonary symptoms predominate, but any organ system can be affected. Spread to the ear can be via direct extension through the nasopharynx, from hematogenous spread, or on rare occasions, via direct implantation through a preexisting tympanic perforation. Tubercular otitis media occurs...

Mucous Membrane Pemphigoid

MMP is a chronic autoimmune mucocutaneous disease in which autoantibodies directed at structural proteins of the hemidesmosome destroy the epithelial-connective tissue attachment at the level of the basement membrane, producing a subepithelial separation 24,25 . The protein targets of the autoantibodies include BP-1, BP-2, and laminin-5 epiligrin , all components of the epithelial anchoring apparatus. MMP is a generalized term for a group of closely related disease processes 26 . The term oral...

Otic Diseases

Acute otitis media is a suppurative infection of the middle-ear cavity. Facial nerve involvement is considered a complication of the infection and often occurs from direct pathogen invasion of a dehiscent portion of the facial nerve course, most commonly in the horizontal segment of the facial nerve. Treatment consists of systemic antibiotic therapy against the most common pathogens Haemophilus influenzae, Moraxella catarrhalis, and Streptococcus pneumoniae. Drainage of the...

Cerebrovascular Disease

Cerebrovascular disease is the most common disorder causing neurogenic oral and oropharyngeal dysphagia 3 . Swallowing disorders have been reported in 27 to 50 of patients suffering from CVAs 4 . The incidence is not limited to severe strokes, because even patients with mild-to-moderate strokes have a poorer long-term outcome directly attributable to their swallowing disorder 5 . Given the complex neurophysiology associated with swallowing, a wide range of stroke locations can result in...

Migraine Syndrome

Migraine Aura Fortification

Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles, California, U.S.A. Clinical Manifestations 336 Complications and Prognosis 340 Migraine is a syndrome characterized by periodic headaches. Often, however, patients experience other symptoms, including hearing loss, tinnitus, and dizziness and in some cases, these can be the only symptoms. Migraine is nearly always familial and occurs in complex patterns and settings. The association of migraine and...

Mycobacterial Infections

Tuberculosis TB, from Mycobacterium tuberculosis and or Mycobacterium bovis is a major cause of morbidity and mortality in HIV disease. It usually presents as reactivation of a pulmonary primary focus, with a risk of 7 to 10 per year for HIV-infected persons regardless of CD4 lymphocyte count, versus 10 per lifetime for HIV-negative persons. There can be involvement of the lungs, central nervous system CNS , or other organs, with rhinosinusitis, diffuse or localized scrofula...

Definitions

Fungal sinusitis, when used loosely, can be a misleading term. It actually refers to a spectrum of fungal-associated diseases of the nose and paranasal sinuses, each with a unique presentation and management implications Table 1 . When communicating with TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses is used to indicate controversy TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses is used to indicate controversy topical nasal steroids, antifungals...