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Management of Pain in Sickle Cell Disease - Practice Guidelines
Evaluation of Syncope - Article
ABSTRACT: Syncope is a transient and abrupt loss of consciousness with complete return to preexisting neurologic function. It is classified as neurally mediated (i.e., carotid sinus hypersensitivity, situational, or vasovagal), cardiac, orthostatic, or neurogenic. Older adults are more likely to have orthostatic, carotid sinus hypersensitivity, or cardiac syn- cope, whereas younger adults are more likely to have vasovagal syncope. Common nonsyncopal syndromes with similar presentations include seizures, metabolic and psychogenic disorders, and acute intoxication. Patients presenting with syncope (other than neurally mediated and orthostatic syncope) are at increased risk of death from any cause. Useful clinical rules to assess the short-term risk of death and the need for immediate hospitalization include the San Francisco Syncope Rule and the Risk Stratification of Syncope in the Emergency Department rule. Guidelines suggest an algorithmic approach to the evaluation of syncope that begins with the history and physical examination. All patients presenting with syncope require electrocardiography, orthostatic vital signs, and QT interval monitoring. Patients with cardiovascular disease, abnormal electrocardiography, or family history of sudden death, and those presenting with unexplained syncope should be hospitalized for further diagnostic evaluation. Patients with neurally mediated or orthostatic syncope usually require no additional testing. In cases of unexplained syncope, further testing such as echocardiography, grade exercise testing, electrocardiographic monitoring, and electrophysiologic studies may be required. Although a subset of patients will have unexplained syncope despite undergoing a comprehensive evaluation, those with multiple episodes compared with an isolated event are more likely to have a serious underlying disorder.
ABSTRACT: Group B streptococcus is the leading cause of early-onset neonatal sepsis in the United States. Universal screening is recommended for pregnant women at 35 to 37 weeks’ gestation. The Centers for Disease Control and Prevention recently updated its guideline for the prevention of early-onset neonatal group B streptococcal disease. The new guideline contains six important changes. First, there is a recommendation to consider using sensitive nucleic acid amplification tests, rather than just routine cultures, for detection of group B streptococcus in rectal and vaginal specimens. Second, the colony count required to consider a urine specimen positive is at least 104 colony-forming units per mL. Third, the new guideline presents separate algorithms for management of preterm labor and preterm premature rupture of membranes, rather than a single algorithm for both conditions. Fourth, there are minor changes in the recommended dose of penicillin G for intrapartum chemoprophylaxis. Fifth, the guideline provides new recommendations about antibiotic regimens for women with penicillin allergy. Cefazolin is recommended for women with minor allergies. For those at serious risk of anaphylaxis, clindamycin is recommended if the organism is susceptible or if susceptibility is unknown, and vancomycin is recommended if there is clindamycin resistance. Finally, the new algorithm for secondary prevention of early-onset group B streptococcal disease in newborns should be applied to all infants, not only those at high risk of infection. The algorithm clarifies the extent of evaluation and duration of observation required for infants in different risk categories.
ABSTRACT: Patients with suspected food allergies are commonly seen in clinical practice. Although up to 15 percent of parents believe their children have food allergies, these allergies have been confirmed in only 1 to 3 percent of all Americans. Family physicians must be able to separate true food allergies from food intolerance, food dislikes, and other conditions that mimic food allergy. The most common foods that produce allergic symptoms are milk, eggs, seafood, peanuts, and tree nuts. Although skin testing and in vitro serum immunoglobulin E assays may help in the evaluation of suspected food allergies, they should not be performed unless the clinical history suggests a specific food allergen to which testing can be targeted. Furthermore, these tests do not confirm food allergy. Confirmation requires a positive food challenge or a clear history of an allergic reaction to a food and resolution of symptoms after eliminating that food from the diet. More than 70 percent of children will outgrow milk and egg allergies by early adolescence, whereas peanut allergies usually remain throughout life. The most serious allergic response to food allergy is anaphylaxis. It requires emergency care that should be initiated by the patient or family using an epinephrine autoinjector, which should be carried by anyone with a diagnosed food allergy. These and other recommendations presented in this article are derived from the Guidelines for the Diagnosis and Management of Food Allergy in the United States, published by the National Institute of Allergy and Infectious Diseases.
Hereditary Hemochromatosis - Article
ABSTRACT: Hereditary hemochromatosis is an autosomal recessive disorder that disrupts the body’s regulation of iron. It is the most common genetic disease in whites. Men have a 24-fold increased rate of iron-overload disease compared with women. Persons who are homozygous for the HFE gene mutation C282Y comprise 85 to 90 percent of phenotypically affected persons. End-organ damage or clinical manifestations of hereditary hemochromatosis occur in approximately 10 percent of persons homozygous for C282Y. Symptoms of hereditary hemochromatosis are nonspecific and typically absent in the early stages. If present, symptoms may include weakness, lethargy, arthralgias, and impotence. Later manifestations include arthralgias, osteoporosis, cirrhosis, hepatocellular cancer, cardiomyopathy, dysrhythmia, diabetes mellitus, and hypogonadism. Diagnosis requires confirmation of increased serum ferritin levels and transferrin saturation, with or without symptoms. Subtyping is based on genotypic expression. Serum ferritin measurement is the most useful prognostic indicator of disease severity. Liver biopsy is performed to stage the degree of fibrosis with severe ferritin elevation or transaminitis, or to diagnose nonclassical hereditary hemochromatosis in patients with other genetic defects. Treatment of hereditary hemochromatosis requires phlebotomy, and the frequency is guided by serial measurements of serum ferritin levels and transferrin saturation. Iron avidity can result from overtreatment. If iron avidity is not suspected, it may mimic undertreatment with persistently elevated transferrin saturation. Dietary modification is generally unnecessary. Universal screening for hereditary hemochromatosis is not recommended, but testing should be performed in first-degree relatives of patients with classical HFE-related hemochromatosis, those with evidence of active liver disease, and patients with abnormal iron study results. Screening for hepatocellular carcinoma is reserved for those with hereditary hemochromatosis and cirrhosis.
Dermoscopy for the Family Physician - Article
ABSTRACT: Noninvasive in vivo imaging techniques have become an important diagnostic aid for skin cancer detection. Dermoscopy, also known as dermatoscopy, epiluminescence microscopy, incident light microscopy, or skin surface microscopy, has been shown to increase the clinician’s diagnostic accuracy when evaluating cutaneous neoplasms. A handheld instrument called a dermatoscope or dermoscope, which has a transilluminating light source and standard magnifying optics, is used to perform dermoscopy. The dermatoscope facilitates the visualization of subsurface skin structures that are not visible to the unaided eye. The main purpose for using dermoscopy is to help correctly identify lesions that have a high likelihood of being malignant (i.e., melanoma or basal cell carcinoma) and to assist in differentiating them from benign lesions clinically mimicking these cancers. Colors and structures visible with dermoscopy are required for generating a correct diagnosis. Routinely using dermoscopy and recognizing the presence of atypical pigment network, blue-white color, and dermoscopic asymmetry will likely improve the observer’s sensitivity for detecting pigmented basal cell carcinoma and melanoma. A two-step algorithm based on a seven-level criterion ladder is the foundation for dermoscopic evaluation of skin lesions. The first step of the algorithm is intended to help physicians differentiate melanocytic lesions from the following nonmelanocytic lesions: dermatofibroma, basal cell carcinoma, seborrheic keratosis, and hemangioma. The second step is intended to help physicians differentiate nevi from melanoma using one of several scoring systems. From a management perspective, the two-step algorithm is intended to guide the decision-making process on whether to perform a biopsy, or to refer or reassure the patient.
ABSTRACT: Serious health problems, risky behavior, and poor health habits persist among adolescents despite access to medical care. Most adolescents do not seek advice about preventing leading causes of morbidity and mortality in their age group, and physicians often do not find ways to provide it. Although helping adolescents prevent unintended pregnancy, sexually transmitted infections, unintentional injuries, depression, suicide, and other problems is a community-wide effort, primary care physicians are well situated to discuss risks and offer interventions. Evidence supports routinely screening for obesity and depression, offering testing for human immunodeficiency virus infection, and screening for other sexually transmitted infections in some adolescents. Evidence validating the effectiveness of physician counseling about unintended pregnancy, gang violence, and substance abuse is scant. However, physicians should use empathic, personal messages to communicate with adolescents about these issues until studies prove the benefits of more specific methods. Effective communication with adolescents requires seeing the patient alone, tailoring the discussion to the individual patient, and understanding the role of the parents and of confidentiality.
ABSTRACT: Onychomycosis is a fungal infection of the nails that causes discoloration, thickening, and separation from the nail bed. Onychomycosis occurs in 10% of the general population, 20% of persons older than 60 years, and 50% of those older than 70 years. It is caused by a variety of organisms, but most cases are caused by dermatophytes. Accurate diagnosis involves physical and microscopic examination and culture. Histologic evaluation using periodic acid–Schiff staining increases sensitivity for detecting infection. Treatment is aimed at eradication of the causative organism and return to a normal appearance of the nail. Systemic antifungals are the most effective treatment, with meta-analyses showing mycotic cure rates of 76% for terbinafine, 63% for itraconazole with pulse dosing, 59% for itraconazole with continuous dosing, and 48% for fluconazole. Concomitant nail debridement further increases cure rates. Topical therapy with ciclopirox is less effective; it has a failure rate exceeding 60%. Several nonprescription treatments have also been evaluated. Laser and photodynamic therapies show promise based on in-vitro evaluation, but more clinical studies are needed. Despite treatment, the recurrence rate of onychomycosis is 10% to 50% as a result of reinfection or lack of mycotic cure.
ABSTRACT: Swift diagnosis and treatment are critical for good outcomes in patients with nontraumatic subarachnoid hemorrhage, which is usually caused by a ruptured aneurysm. This type of stroke often results in death or disability. Rates of misdiagnosis and treatment delays for subarachnoid hemorrhage have improved over the years, but these are still common occurrences. Subarachnoid hemorrhage can be more easily diagnosed in patients who present with severe symptoms, unconsciousness, or with thunderclap headache, which is often accompanied by vomiting. The diagnosis is more elusive in patients who present in good condition, yet these patients have the best chance for good outcome if they are correctly diagnosed at the time of presentation. Physicians should be alert for warning headaches, which are often severe, and headaches that feel different to the patient. Other symptoms may include nausea, vomiting, impaired consciousness, nuchal rigidity, orbital pain, focal neurologic deficits, dysphasia, lightheadedness, and dizziness. The most important risk factors for subarachnoid hemorrhage include cigarette smoking, hypertension, heavy alcohol use, and personal or family history of aneurysm or hemorrhagic stroke. The first step in the diagnostic workup is noncontrast computed tomography of the head. If computed tomography is negative or equivocal, a lumbar puncture should be performed. Subsequent imaging may include computed tomographic angiography, catheter angiography, and magnetic resonance angiography.