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Pulmonary Hypertension in Sickle Cell Anemia

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401. Splenomegaly (Overview)

of blood-borne pathogens, especially encapsulated organisms, may lead to abscess formation. Because many splenic abscesses may be indolent in presentation, splenic size may increase as the abscess enlarges. This is a relatively uncommon, but important, process to recognize and treat. Splenic sequestration Acute splenic sequestration crisis (ASSC) is a major cause of morbididty and mortality in children with sickle cell disease and other hereditary hemolytic anemias. ASSC is characterized by sudden (...) infections such as Epstein-Barr virus–induced mononucleosis. Hyperplastic splenomegaly Removal of abnormal blood cells from the circulation (either cells with intrinsic defects or cells coated with antibody) is the usual source of hyperplastic splenomegaly. In some cases, extramedullary hematopoiesis (ie, myeloproliferative disease) results in hyperplasia. [ ] Congestive splenomegaly Cirrhosis with portal hypertension, splenic vein occlusion (thrombosis), or congestive heart failure (CHF) with increased

2014 eMedicine.com

402. Sports Physicals (Overview)

may affect the athlete's class. Blood pressure (BP): Although a BP measurement is rarely an indication for disqualification from sports, abnormalities are often first noted during the sports physical examination setting. Athletes with BP changes can be referred for follow-up care with a primary physician. Certain sports may cause significant BP elevations, and this may be a reason to limit an athlete's participation. The BP must be evaluated more than once, and normal BPs for the athlete's age (...) , and vertical leaping. Table 2. Classification of Hypertension by Age Group [ ] Age Group, y Significant Hypertension, mm Hg Severe Hypertension, mm Hg Systolic BP Diastolic BP Systolic BP Diastolic BP Children 6-9 10-12 122 126 78 82 130 134 86 90 Adolescents 13-15 15-18 136 142 86 92 144 150 92 98 Source: Report of the Second Task Force on Blood Pressure Control in Children, 1987. [ ] Previous Next: Other Screening Evaluations The role of other screening evaluations before sports participation has been

2014 eMedicine.com

403. Chronic Renal Failure (Overview)

of CKD but should not by itself be considered a marker of it, because elevated blood pressure is also common among people without CKD. In an update of its CKD classification system, the NKF advised that GFR and albuminuria levels be used together, rather than separately, to improve prognostic accuracy in the assessment of CKD. [ , ] More specifically, the guidelines recommended the inclusion of estimated GFR and albuminuria levels when evaluating risks for overall mortality, cardiovascular disease (...) < 30 mL/min/1.73 m²) that endocrine/metabolic derangements or disturbances in water or electrolyte balance become clinically manifest. Signs of metabolic acidosis in stage 5 CKD include the following: Loss of lean body mass Muscle weakness Signs of alterations in the way the kidneys are handling salt and water in stage 5 include the following: Peripheral edema Pulmonary edema Hypertension Anemia in CKD is associated with the following: Fatigue Reduced exercise capacity Impaired cognitive and immune

2014 eMedicine.com

404. Erectile Dysfunction (Overview)

Dyslipidemia Hypertension Neurologic causes Epilepsy Stroke Multiple sclerosis Guillain-Barré syndrome Alzheimer disease Trauma Respiratory disease Chronic obstructive pulmonary disease Sleep apnea Endocrine conditions Hyperthyroidism Hypothyroidism Hypogonadism Diabetes Penile conditions Peyronie disease Epispadias Priapism Psychiatric conditions Depression Widower syndrome Performance anxiety Posttraumatic stress disorder Nutritional states Malnutrition Zinc deficiency Hematologic diseases Sickle cell (...) is a thorough history that includes the following: Sexual history Medical history Psychosocial history A physical examination is necessary for every patient, emphasizing the genitourinary, vascular, and neurologic systems. A focused examination entails evaluation of the following: Blood pressure Peripheral pulses Sensation Status of the genitalia and prostate Size and texture of the testes Presence of the epididymis and vas deferens Abnormalities of the penis (eg, hypospadias, Peyronie plaques

2014 eMedicine.com

405. Altitude-Related Disorders (Treatment)

Populations at High Altitude Large numbers of individuals go to high altitudes for work and recreation, and some individuals have special medical problems. Despite similarities to altitude illness in healthy individuals, ascent to high altitude by persons with underlying cardiac disease, [ ] pulmonary disease, and sickle cell anemia deserves special mention. Coronary Artery Disease Unacclimatized persons with coronary artery disease may develop increased anginal symptoms following ascent to altitude (...) vasoconstriction raises pulmonary artery pressure in sojourners to high altitude. With idiopathic , ascent to altitude results in even higher pulmonary artery pressures. These patients are likely to experience additional symptoms, such as fatigue, dyspnea, or even syncope. An increase in supplemental oxygen or the use of pulmonary vasodilators may be helpful to ameliorate altitude symptoms. Prior to traveling to high altitude, persons with idiopathic pulmonary hypertension should consult a physician familiar

2014 eMedicine.com

406. Haemophilus Influenzae Infections (Treatment)

. Therefore, reevaluate these children (including with lumbar punctures and chest radiography) for an infectious focus and obtain repeat blood cultures. Administer parenteral antibiotics for at least 2-5 days and guide subsequent therapy based on the focus of infection. If no focus is identified, substitute oral antibiotics to complete 7-14 days of therapy, as in other gram-negative bloodstream infections/bacteremias. Studies have been conducted to determine the optimal treatment duration for gram (...) recommendations for adults aged 19 years and older. Recommendations related to the Hib vaccine remained the same as in 2014. Hib vaccination is recommended in the following populations: Certain adults at increased risk for Hib infection (patients with anatomical or functional asplenia or sickle cell diseases or who are undergoing elective splenectomy should receive one dose of Hib vaccine) who have not previously received the vaccine, except individuals with human immunodeficiency (HIV) infection

2014 eMedicine.com

407. Hematologic Disease and Pregnancy (Treatment)

, hypertensive disorders, intrauterine growth restriction (IUGR), and cesarean delivery among women with sickle cell disease. [ ] The following odds ratios were significantly increased for women with sickle cell disease: pneumonia (9.8), sepsis (6.8), cerebral venous thrombosis (4.9), eclampsia (3.2), IUGR (2.9), DVT (2.5), stroke (2.0), pulmonary embolism (1.7), postpartum infection (1.4), and pyelonephritis (1.3). The mortality rate for women with sickle cell disease was 6 times that for women without (...) by hemoglobin electrophoresis. Anemia occurs as a result of the sickle hemoglobinopathies. Deoxygenation of the abnormal red blood cells (RBCs) results in sickling. These permanently damaged RBCs are then removed by the reticuloendothelial system, with the average RBC lifespan reduced to 17 days. The result is a chronic compensated anemia, with Hb typically measured between 6.5 and 9.5 g/dL. The sickle shape also results in altered motion through the microvasculature. This altered motion can predispose

2014 eMedicine.com

408. Early Pregnancy Loss (Treatment)

, factor XIII deficiency, congenital hypofibrinogenemia and afibrinogenemia, and sickle cell anemia. Women with sickle cell anemia are at increased risk for fetal loss, possibly because of placental-bed microinfarcts. Management For couples who have had an SAB due to a suspected genetic cause, the standard of care is to offer genetic counseling. Because advanced age increases the risk of an abnormal karyotype in a conceptus, amniocentesis is routinely offered for all pregnant women of advanced maternal (...) are predictive of adverse obstetric outcome in patients with SLE. Disease before conception Onset of SLE during pregnancy Underlying renal disease Other obstetric and medical conditions associated with APLAs are listed below. Obstetric conditions associated with APLAs [ ] Abnormal fetal heart rate tracings Preterm deliveries Pregnancy wastage Medical conditions associated with APLAs Arterial and venous thrombosis Autoimmune thrombocytopenia Autoimmune hemolytic anemia Livedo reticularis Chorea Pulmonary

2014 eMedicine.com

409. Genetic and Inflammatory Mechanisms in Stroke (Treatment)

. Selected single-gene disorders are discussed. Sickle cell anemia Cerebrovascular complications in sickle cell anemia (SCA) are the result of polymerized red blood cells at low oxygen tensions, resulting in small vessel occlusion and sickle-related arterial disease. SCA is seen in approximately 6% of children with stroke, [ ] but one quarter of individuals with SCA experience a stroke by age 45 years and the highest incidence for ischemic stroke is at 2-5 years. [ ] By the onset of early adulthood (...) pupils, hypotonic bladder, gut malrotation and hypoperistalsis, and pulmonary hypertension. [ ] There is a risk for aortic, cervical, and intracranial arterial dissection. [ ] They are also at risk for steno-occlusive small-vessel disease, moyamoya disease, and aneurysmal large-vessel disease. [ ] NF1 Neurofibromatosis type 1 (NF1) is a disorder resulting from a dysfunctional tumor suppressor gene in the Ras signaling pathway. The cerebrovascular expression of disease is excessive smooth cell

2014 eMedicine.com

410. Erectile Dysfunction (Treatment)

by vascular smooth muscle, endothelial, and inflammatory cells. It increases production of nitric oxide (NO), which results in improves endothelial function and blood flow in chronic ischemic disorders. [ , ] Direct intracavernosal injection of recombinant VEGF protein or adenoviral VEGF that contains plasmids has shown dramatic results on cavernosography in animal models with arteriogenic, venogenic, and neural forms of ED. Burchardt et al identified VEGF 165 as the predominant isoform in the corpora (...) is the infrequency of pellet placement (only every 3-6 months). The use of exogenous androgens suppresses natural androgen production. Elevation of serum androgen levels has the potential to stimulate prostate growth and may increase the risk of activating a latent cancer. Periodic prostate examinations, including digital rectal examinations, prostate-specific antigen (PSA) determinations, and blood counts (ie, complete blood count [CBC]), are recommended in all patients receiving supplemental androgens

2014 eMedicine.com

411. Osteoporosis (Primary) (Treatment)

), continuation of treatment for up to 10 years (oral) or 6 years (intravenous), with periodic evaluation, should be considered. The risk of atypical femoral fracture, but not osteonecrosis of the jaw, clearly increases with the duration of bisphosphonate therapy, but such rare events are outweighed by vertebral fracture risk reduction in high-risk patients. For women not at high fracture risk, a drug holiday of 2 to 3 years can be considered after 3 to 5 years of BP treatment. Selective estrogen receptor (...) of Systematic Reviews study found that exercise may help prevent bone loss and fractures in postmenopausal women. The most effective type of exercise on BMD for the neck of the femur was found to be non–weight-bearing, high-force exercise, such as lower limb resistance strength training; combination exercise programs were most effective for BMD at the spine. [ ] Although swimming is not a weight-bearing exercise that will improve BMD, it does provide chest expansion, spinal extension, and low-impact

2014 eMedicine.com

412. Osler-Weber-Rendu Syndrome (Treatment)

. Patients should be monitored for symptoms and signs of blood loss and anemia with yearly stool guaiac testing and complete blood count (CBC) with differential. Patients should be screened for pulmonary, hepatic, and CNS AVMs at the time of diagnosis and at the onset of any suggestive symptoms and signs. Children who have a parent with HHT carry a 50% chance of harboring the same genetic mutation. Accordingly, pulmonary AVM screening and long-term follow-up are advocated for these children, beginning (...) , Henderson KJ, et al. New definition and natural history of patients with diffuse pulmonary arteriovenous malformations: twenty-seven-year experience. Chest . 2008 Mar. 133(3):653-61. . Trembath RC, Thomson JR, Machado RD, et al. Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N Engl J Med . 2001 Aug 2. 345(5):325-34. . Press OW, Ramsey PG. Central nervous system infections associated with hereditary hemorrhagic telangiectasia. Am

2014 eMedicine.com

413. Oral Manifestations of Systemic Diseases (Treatment)

, and—in the case of heparin-induced thrombocytopenia—skin necrosis. [ ] In the oral cavity, petechiae and ecchymoses may be visible on the soft palate and buccal mucosa. [ ] Gingival bleeding is common and often spontaneous. [ ] Hemorrhagic bullae that appear as deep‒red-to-violaceous or black blisters may appear on mucosal surfaces in cases of severe thrombocytopenia. [ ] Red blood cell disorders Anemias Common anemias associated with oral manifestations include iron-deficiency anemia and macrocytic anemia (...) : Hematologic Disorders Hematologic malignancies and blood cell dyscrasias are a remarkably diverse collection of diseases with numerous systemic sequelae. The etiology, diagnostic criteria, prognosis, and treatment of each condition is unique and complex. As such, a complete analysis is not within the scope of this review. The oral manifestations of numerous hematologic disorders are summarized. Please refer to the references for more detailed information. [ ] White blood cell disorders Leukemia Leukemia

2014 eMedicine.com

414. Osteoporosis (Treatment)

), continuation of treatment for up to 10 years (oral) or 6 years (intravenous), with periodic evaluation, should be considered. The risk of atypical femoral fracture, but not osteonecrosis of the jaw, clearly increases with the duration of bisphosphonate therapy, but such rare events are outweighed by vertebral fracture risk reduction in high-risk patients. For women not at high fracture risk, a drug holiday of 2 to 3 years can be considered after 3 to 5 years of BP treatment. Selective estrogen receptor (...) of Systematic Reviews study found that exercise may help prevent bone loss and fractures in postmenopausal women. The most effective type of exercise on BMD for the neck of the femur was found to be non–weight-bearing, high-force exercise, such as lower limb resistance strength training; combination exercise programs were most effective for BMD at the spine. [ ] Although swimming is not a weight-bearing exercise that will improve BMD, it does provide chest expansion, spinal extension, and low-impact

2014 eMedicine.com

415. Osteoporosis (Secondary) (Treatment)

), continuation of treatment for up to 10 years (oral) or 6 years (intravenous), with periodic evaluation, should be considered. The risk of atypical femoral fracture, but not osteonecrosis of the jaw, clearly increases with the duration of bisphosphonate therapy, but such rare events are outweighed by vertebral fracture risk reduction in high-risk patients. For women not at high fracture risk, a drug holiday of 2 to 3 years can be considered after 3 to 5 years of BP treatment. Selective estrogen receptor (...) of Systematic Reviews study found that exercise may help prevent bone loss and fractures in postmenopausal women. The most effective type of exercise on BMD for the neck of the femur was found to be non–weight-bearing, high-force exercise, such as lower limb resistance strength training; combination exercise programs were most effective for BMD at the spine. [ ] Although swimming is not a weight-bearing exercise that will improve BMD, it does provide chest expansion, spinal extension, and low-impact

2014 eMedicine.com

416. Urinary Tract Infection, Males (Treatment)

prognosis, such as old age, debility, renal calculi, recent hospitalization or instrumentation, diabetes, sickle cell anemia, underlying carcinoma, or intercurrent cancer chemotherapy, the antimicrobial coverage should be broadened and an antipseudomonal agent should be added. Adult males with UTI should receive a 10- to 14-day course of antibiotics. Outpatient regimens include a fluoroquinolone, trimethoprim-sulfamethoxazole (TMP-SMZ), minocycline, or nitrofurantoin (should not be given if glomerular (...) with high baseline creatinine levels, younger age, and a high peripheral white blood cell (WBC) count. Emphysematous pyelonephritis Patients with diabetes are prone to develop emphysematous pyelonephritis, which is characterized by gas formation in the urinary tract. It often requires immediate nephrectomy for survival. Previous Next: Orchitis, Cystitis, and Urethritis Therapy Orchitis For viral orchitis, supportive therapy with scrotal support, cold compresses, and bedrest is all that is needed

2014 eMedicine.com

417. Medical Treatment of Stroke (Treatment)

stroke prevention may include the use of antihypertensive medications, anticoagulants, platelet antiaggregants, 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors (statins), smoking cessation, dietary intervention, weight loss, and exercise. Modifiable risk factors include the following: Hypertension Air pollution Cigarette smoking Diabetes Dyslipidemia Atrial fibrillation Sickle cell disease Postmenopausal HRT Depression Diet and activity Weight and body fat Secondary Prevention (...) and will be discussed in Secondary Prevention. Hypertension Hypertension is the most important modifiable risk factor for stroke and intracerebral hemorrhage (ICH), and the risk of stroke increases progressively with increasing blood pressure, independent of other factors. [ , ] Both behavioral lifestyle changes and pharmacologic therapy are important parts of the comprehensive strategy recommended in the Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High

2014 eMedicine.com

418. Transfusion-Induced Iron Overload (Treatment)

and high iron burdens. [ ] This agent has an acceptable tolerability profile and appears to have similar efficacy to deferoxamine in reducing the iron burden in transfused patients with sickle cell disease. [ ] In a comparative study of beta-thalassemia patients, noninferiority was demonstrated in the group of patients who were allocated to the higher dose groups (deferasirox doses of 20 or 30 mg/kg) for baseline liver iron concentrations (LIC) of 7 mg/g dry weight or greater when compared (...) , Antonijevic N, Jankovic G, Babic D, Colovic M. [Aplastic anemia--clinical characteristics and survival analysis] [Serbian]. Srp Arh Celok Lek . 1998 Jul-Aug. 126(7-8):234-8. . Aul C, Gattermann N, Germing U, Runde V, Heyll A. [Myelodysplastic syndromes. The epidemiological and etiological aspects] [German]. Dtsch Med Wochenschr . 1992 Aug 14. 117(33):1223-31. . Hassan M, Hasan S, Giday S, et al. Hepatitis C virus in sickle cell disease. J Natl Med Assoc . 2003 Oct. 95(10):939-42. . Halonen P, Mattila J

2014 eMedicine.com

419. Priapism (Overview)

be necessary to treat underlying sickle cell disease (SCD), a cause of low-flow priapism Penile blood gas (PBG) measurement: Allows differentiation between high- and low-flow priapism Imaging studies Penile duplex Doppler ultrasonography: To help identify and locate fistulas in patients with high-flow priapism Pelvic angiography: To help confirm the fistula’s location Chest radiography or computed tomography (CT) scanning: Used if the patient’s history is consistent with a malignant or metastatic condition (...) to treat priapism and as a possible means of preventing full-blown episodes in patients with sickle cell disease. [ ] High-flow priapism may result from the following forms of genitourinary trauma: Straddle injury Intracavernous injections resulting in direct cavernosal artery injury Rare causes of priapism include the following: (massive amyloid infiltration) (one case report) Black widow spider bites [ ] Asplenia (rare association, occasionally noted to be priapism of the high-flow type) Vigorous

2014 eMedicine.com

420. Sports Physicals (Treatment)

may affect the athlete's class. Blood pressure (BP): Although a BP measurement is rarely an indication for disqualification from sports, abnormalities are often first noted during the sports physical examination setting. Athletes with BP changes can be referred for follow-up care with a primary physician. Certain sports may cause significant BP elevations, and this may be a reason to limit an athlete's participation. The BP must be evaluated more than once, and normal BPs for the athlete's age (...) , and vertical leaping. Table 2. Classification of Hypertension by Age Group [ ] Age Group, y Significant Hypertension, mm Hg Severe Hypertension, mm Hg Systolic BP Diastolic BP Systolic BP Diastolic BP Children 6-9 10-12 122 126 78 82 130 134 86 90 Adolescents 13-15 15-18 136 142 86 92 144 150 92 98 Source: Report of the Second Task Force on Blood Pressure Control in Children, 1987. [ ] Previous Next: Other Screening Evaluations The role of other screening evaluations before sports participation has been

2014 eMedicine.com

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