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Pulmonary Hypertension in Sickle Cell Anemia

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441. Altitude-Related Disorders (Overview)

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

442. Medical Treatment of Stroke (Follow-up)

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

443. Hyperbaric Oxygen Therapy

, resulting in decreased edema. As a gradient of oxygenation is based on blood flow, oxygen tissue tensions can be returned, allowing for the host defenses to properly function. [ ] Animal studies suggest that a decreased neutrophil adherence to ischemic venules is observed with elevated oxygen pressures (2.5 ATA). [ , , ] Reperfusion injury is diminished, as HBOT generates scavengers to destroy oxygen radicals. [ ] Compartment syndrome also is a continuum of injury that occurs when compartment pressures (...) and Hyperbaric Medical Society, Inc.; 2003. chap 1, 2, 4, 5, 7, 8. Leslie CA, Sapico FL, Ginunas VJ, Adkins RH. Randomized controlled trial of topical hyperbaric oxygen for treatment of diabetic foot ulcers. Diabetes Care . 1988 Feb. 11(2):111-5. . Boerema I, et al. Life without blood. A study of the influence of high atmospheric pressure and hypothermia on dilution of the blood. J Cardiovascular Surg . 1960. 1:133-146. Bassett BE, Bennett PB. Introduction to the physical and physiological bases

2014 eMedicine Surgery

444. Hyperbaric Oxygen

, resulting in decreased edema. As a gradient of oxygenation is based on blood flow, oxygen tissue tensions can be returned, allowing for the host defenses to properly function. [ ] Animal studies suggest that a decreased neutrophil adherence to ischemic venules is observed with elevated oxygen pressures (2.5 ATA). [ , , ] Reperfusion injury is diminished, as HBOT generates scavengers to destroy oxygen radicals. [ ] Compartment syndrome also is a continuum of injury that occurs when compartment pressures (...) and Hyperbaric Medical Society, Inc.; 2003. chap 1, 2, 4, 5, 7, 8. Leslie CA, Sapico FL, Ginunas VJ, Adkins RH. Randomized controlled trial of topical hyperbaric oxygen for treatment of diabetic foot ulcers. Diabetes Care . 1988 Feb. 11(2):111-5. . Boerema I, et al. Life without blood. A study of the influence of high atmospheric pressure and hypothermia on dilution of the blood. J Cardiovascular Surg . 1960. 1:133-146. Bassett BE, Bennett PB. Introduction to the physical and physiological bases

2014 eMedicine Surgery

445. Osler-Weber-Rendu Disease (Diagnosis)

be either hemorrhagic or ischemic. Of patients who have pulmonary AVMs, 2% per year are estimated to have a stroke, and 1% per year are estimated to develop a brain abscess. Retinal arteriovenous aneurysms occur only rarely. Patients are also at risk for high-output cardiac failure, migraines and further sequelae. Frequent nosebleeds and melena may result from telangiectasia in the nose and GI tract. Patients with the severe form of HHT have heavy bleeding and resultant iron-deficiency anemia. Recurrent (...) . Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore) . 2007 Jan. 86(1):1-17. . Pierucci P, Murphy J, 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

2014 eMedicine Surgery

446. Osteoporosis (Diagnosis)

Ankylosing spondylitis Rheumatoid arthritis Systemic lupus erythematosus Hematologic and neoplastic disorders Hemochromatosis Hemophilia Leukemia Lymphoma Multiple myeloma Sickle cell anemia Systemic mastocytosis Thalassemia Metastatic disease Medications Anticonvulsants Antipsychotic drugs Antiretroviral drugs Aromatase inhibitors Chemotherapeutic/transplant drugs: cyclosporine, tacrolimus, platinum compounds, cyclophosphamide, ifosfamide, high-dose methotrexate [ ] Furosemide Glucocorticoids (...) be evident, especially in patients with an altered center of gravity from severe kyphosis. [ ] Patients may have difficulty performing tandem gait and performing single limb stance. See for more detail. Diagnosis Baseline laboratory studies include the following: Complete blood count: May reveal anemia Serum chemistry levels: Usually normal in persons with primary osteoporosis Liver function tests Thyroid-stimulating hormone level: Thyroid dysfunction has been associated with osteoporosis 25

2014 eMedicine Surgery

447. Osler-Weber-Rendu Disease (Overview)

be either hemorrhagic or ischemic. Of patients who have pulmonary AVMs, 2% per year are estimated to have a stroke, and 1% per year are estimated to develop a brain abscess. Retinal arteriovenous aneurysms occur only rarely. Patients are also at risk for high-output cardiac failure, migraines and further sequelae. Frequent nosebleeds and melena may result from telangiectasia in the nose and GI tract. Patients with the severe form of HHT have heavy bleeding and resultant iron-deficiency anemia. Recurrent (...) . Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore) . 2007 Jan. 86(1):1-17. . Pierucci P, Murphy J, 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

2014 eMedicine Surgery

448. Osteoporosis (Overview)

Ankylosing spondylitis Rheumatoid arthritis Systemic lupus erythematosus Hematologic and neoplastic disorders Hemochromatosis Hemophilia Leukemia Lymphoma Multiple myeloma Sickle cell anemia Systemic mastocytosis Thalassemia Metastatic disease Medications Anticonvulsants Antipsychotic drugs Antiretroviral drugs Aromatase inhibitors Chemotherapeutic/transplant drugs: cyclosporine, tacrolimus, platinum compounds, cyclophosphamide, ifosfamide, high-dose methotrexate [ ] Furosemide Glucocorticoids (...) be evident, especially in patients with an altered center of gravity from severe kyphosis. [ ] Patients may have difficulty performing tandem gait and performing single limb stance. See for more detail. Diagnosis Baseline laboratory studies include the following: Complete blood count: May reveal anemia Serum chemistry levels: Usually normal in persons with primary osteoporosis Liver function tests Thyroid-stimulating hormone level: Thyroid dysfunction has been associated with osteoporosis 25

2014 eMedicine Surgery

449. Urinary Tract Infection, Male (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 Emergency Medicine

450. Spleen, Trauma

infarction Causes of splenic infarction include the following: Embolic - Endocarditis, atherosclerotic plaque, cardiac thrombus, and metastasis (gastric and/or pancreatic) Local thrombosis - Sickle cell disease, myelofibrosis, myelolymphoproliferative disorders (chronic myelogenous leukemia type 1), polycythemia vera, and Gaucher disease Vasculitis - Polyarteritis nodosa, systemic lupus erythematosus, and drug abuse Pancreatitis SA aneurysm Splenic torsion Previous Next: Computed Tomography At most (...) ; this characteristic probably explains the higher mortality associated with blunt abdominal trauma than with penetrating injury. Spleen, trauma. Chest radiograph shows a peripherally calcified mass in the left upper quadrant under the diaphragm. The mass represents a calcified splenic hematoma. Missed splenic rupture or delayed diagnosis is associated with a 10-fold increase in mortality over the rate associated with prompt recognition of injury. Therefore, the radiologist must have a high index of suspicion

2014 eMedicine Radiology

451. Splenic Infarct (Diagnosis)

) or vigorous activity (eg, skiing in high-altitude locations) can precipitate sickling and splenic infarction by the above-described mechanism. [ ] In myelofibrosis, the splenic parenchyma is infiltrated by extramedullary hematopoiesis, causing congestion of the splenic circulation. In malignant hematologic diseases (eg, chronic myeloid leukemia), increased splenic oxygen requirements secondary to an increased splenic mass, coupled with a decreased oxygen-carrying capacity secondary to the anemia (...) of sickle cell disease. Clin Gastroenterol Hepatol . 2010 Jun. 8 (6):483-9; quiz e70. . Franklin QJ, Compeggie M. Splenic syndrome in sickle cell trait: four case presentations and a review of the literature. Mil Med . 1999 Mar. 164(3):230-3. . Sheikha A. Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary. Can J Surg . 2005 Oct. 48(5):377-81. . . O'Keefe JH Jr, Holmes DR Jr, Schaff HV, Sheedy PF 2nd, Edwards WD. Thromboembolic splenic

2014 eMedicine Surgery

452. Early Pregnancy Loss (Follow-up)

, 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

453. Transfusion-Induced Iron Overload (Follow-up)

parameters. Pediatrics . 2003 Jan. 111(1):91-6. . . Files B, Brambilla D, Kutlar A, et al. Longitudinal changes in ferritin during chronic transfusion: a report from the Stroke Prevention Trial in Sickle Cell Anemia (STOP). J Pediatr Hematol Oncol . 2002 May. 24(4):284-90. . Borgna-Pignatti C, Castriota-Scanderbeg A. Methods for evaluating iron stores and efficacy of chelation in transfusional hemosiderosis. Haematologica . 1991 Sep-Oct. 76(5):409-13. . Kolnagou A, Economides C, Eracleous E (...) Cardiovasc Imaging . 2005 Oct. 21(5):531-8. . Wood JC, Enriquez C, Ghugre N, Tyzka JM, Carson S, Nelson MD. MRI R2 and R2* mapping accurately estimates hepatic iron concentration in transfusion-dependent thalassemia and sickle cell disease patients. Blood . 2005 Aug 15. 106(4):1460-5. . Sparacia G, Midiri M, D'Angelo P, Lagalla R. Magnetic resonance imaging of the pituitary gland in patients with secondary hypogonadism due to transfusional hemochromatosis. MAGMA . 1999 May. 8(2):87-90. . Sparacia G, Iaia

2014 eMedicine.com

454. Sports Physicals (Follow-up)

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

455. Erectile Dysfunction (Follow-up)

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

456. Altitude-Related Disorders (Follow-up)

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

457. Splenomegaly (Diagnosis)

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

458. Sports Physicals (Diagnosis)

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

459. Urinary Tract Infection, Males (Follow-up)

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

460. Genetic and Inflammatory Mechanisms in Stroke (Follow-up)

. 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

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