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Pulmonary Hypertension in Sickle Cell Anemia

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461. Priapism (Treatment)

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 Emergency Medicine

462. Organ Procurement Considerations in Trauma

, and most potential donors are reviewed on a case-by-case basis. Additional absolute and relative contraindications are assessed for donation of specific organs. Adaptations of the New England Organ Bank (NEOB) and the California Transplant Donor Network (CTDN) criteria are as follows: Absolute contraindications Age older than 80 years HIV infection Active metastatic cancer Prolonged hypotension or hypothermia Disseminated intravascular coagulation Sickle cell anemia or other hemoglobinopathy Relative (...) , electrolytes, glucose, ) ABO blood typing HLA typing Blood cultures Sputum Gram stain, culture, and sensitivities Urinalysis, culture, and sensitivities HIV, Epstein-Barr virus (EBV), cytomegalovirus (CMV), human T-cell leukemia virus type 1 (HTLV-1), and hepatitis B and C virus serologies Venereal disease research laboratory (VDRL) test or rapid plasma reagent (RPR) test Inguinal lymph nodes tested for evaluation of recipient sensitivity Heart donor ECG Chest radiograph Echocardiogram Cardiac

2014 eMedicine Surgery

463. Chronic Renal Failure (Diagnosis)

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

464. Altitude-Related Disorders (Diagnosis)

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

465. Genetic and Inflammatory Mechanisms in Stroke (Diagnosis)

. 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

466. Early Pregnancy Loss (Diagnosis)

, 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

467. Perioperative Management of the Female Patient

for surgery are being treated for, or have, hypertension. Some authors report that as many as 4% of women have elevated blood pressure from using oral contraceptives. Postponing elective surgical procedures until blood pressure readings are normalized is recommended. As long as the patient has stable controlled diastolic blood pressures of no greater than 110 mm Hg, they will tolerate surgery without cardiac sequelae. Antihypertensive medication is taken up to the time of surgery and resumed early (...) " preoperative laboratory tests has been called into question. As described above, an appropriate history and physical examination determine the need for tests beyond a complete blood cell (CBC) count. Prothrombin time (PT), platelet count, and activated partial thromboplastin time (aPTT) are not routinely indicated unless a bleeding disorder is suspected. Blood glucose determination is indicated only in the elderly population because of the high prevalence of diabetes mellitus. Blood urea nitrogen (BUN

2014 eMedicine.com

468. Haemophilus Influenzae Infections (Diagnosis)

Immunologic studies: Detection of the polyribosyl ribitol phosphate (PRP) polysaccharide capsule via countercurrent immunoelectrophoresis, latex particle agglutination, co-agglutination, and enzyme-linked immunosorbent assay; important adjuncts to culturing for rapid diagnosis Cerebrospinal fluid (CSF) studies (eg, Gram stain, culture, glucose/protein levels) Blood cell counts: Assessment for anemia, leukocytosis, thrombocytosis, and/or thrombocytopenia Acute phase reactants: Characteristic elevated (...) , sepsis, meningitis, pneumonia, respiratory distress, scalp abscess, conjunctivitis, and vesicular eruption NTHi infections: Commonly causes various mucosal infections, including otitis media and conjunctivitis Persons at risk for invasive H influenzae disease include the following: Children younger than 4 years Contacts (eg, household and daycare) of someone with Hib disease Persons with sickle cell disease, Persons with asplenia Individuals with HIV infection Individuals with immunoglobulin

2014 eMedicine.com

469. Haemophilus Meningitis (Diagnosis)

. Infants and young children who develop Hib meningitis take as long as 3 months to mount a type-specific response to the causative Hib strain. To varying degrees, the development of these protective immune responses is delayed and less robust in children who have immune system compromise, such as those with agammaglobulinemia, immunoglobulin G (IgG2) subclass deficiency, or various degrees of asplenia due to sickle cell anemia or other causes, as well as those with cancer, HIV infection, chronic (...) for Hib vaccination programs instituted in Chile and the Dominican Republic, where, before immunization, the annual incidence of Hib meningitis in children younger than 5 years had been higher than 20 cases per 100,000. Significant declines in incidence of Hib meningitis are reported for hospitals in Argentina and South Africa, as well as declines in percentage of positive CSF indicators of bacterial meningitis, such as elevated white blood cell count, low glucose, elevated protein, or turbidity

2014 eMedicine.com

470. 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.com

471. Erectile Dysfunction (Diagnosis)

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

472. Cardiomyopathy, Dilated (Treatment)

Neuromuscular disorders Duchenne or Becker muscular dystrophies, Friedreich ataxia, Kearns-Sayre syndrome, other muscular dystrophies Nutritional factors Kwashiorkor, pellagra, thiamine deficiency, selenium deficiency Collagen vascular diseases Rheumatic fever, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Kawasaki disease Hematological diseases Thalassemia, sickle cell disease, iron deficiency anemia Coronary artery diseases Anomalous left coronary artery from pulmonary artery (...) , cardiomegaly Older children: Dyspnea, dependent edema, elevated jugular venous pressure, cardiomegaly Probable heart disease with heart failure Chest radiography Cardiomegaly, pulmonary plethora, prominent upper lobe veins, pulmonary edema, pleural effusion, collapsed left lower lobe High probability of heart failure with or without chest infection Electrocardiography Low-voltage complexes Pericardial effusion Presence of Q waves and inversion of T waves in leads I, II, aVL, and V 4 through V 6

2014 eMedicine Pediatrics

473. Bone Marrow Transplantation, Long-Term Effects (Treatment)

, noninfectious pulmonary complications (LONIPC). This represents a group of pulmonary disorders, including , bronchiolitis obliterans with organizing pneumonia, and idiopathic pulmonary syndrome. A study examining 97 children who survived allogeneic HSCT reported the incidence rate of LONIPC at 10%, consistent with the incidence reported in the adult literature. [ ] Multivariate analysis revealed high-risk underlying disease and extensive chronic GVHD as significant risk factors for LONIPC. Although GI (...) and normal estrogen levels after undergoing HSCT with high-dose chemotherapy and hyperfractionated TBI. [ ] However, the 7 remaining females (mean age, 8.6 y) required hormone replacement therapy for biochemical and hormonal ovarian failure after HSCT. Several case series have reported the resumption of ovarian function after initial ovarian failure following HSCT. [ ] The mechanism remains unclear. Another study of females who received transplantation for sickle cell disease showed that 7 of 10 females

2014 eMedicine Pediatrics

474. Osler-Weber-Rendu Syndrome (Treatment)

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 Pediatrics

475. Bone Marrow Transplantation, Long-Term Effects (Overview)

, noninfectious pulmonary complications (LONIPC). This represents a group of pulmonary disorders, including , bronchiolitis obliterans with organizing pneumonia, and idiopathic pulmonary syndrome. A study examining 97 children who survived allogeneic HSCT reported the incidence rate of LONIPC at 10%, consistent with the incidence reported in the adult literature. [ ] Multivariate analysis revealed high-risk underlying disease and extensive chronic GVHD as significant risk factors for LONIPC. Although GI (...) and normal estrogen levels after undergoing HSCT with high-dose chemotherapy and hyperfractionated TBI. [ ] However, the 7 remaining females (mean age, 8.6 y) required hormone replacement therapy for biochemical and hormonal ovarian failure after HSCT. Several case series have reported the resumption of ovarian function after initial ovarian failure following HSCT. [ ] The mechanism remains unclear. Another study of females who received transplantation for sickle cell disease showed that 7 of 10 females

2014 eMedicine Pediatrics

476. Hypoplastic Left Heart Syndrome (Treatment)

for ventriculoaortic reconstitution. Br Heart J . 1976 Aug. 38(8):821-6. . . Balfour IC, Covitz W, Davis H, Rao PS, Strong WB, Alpert BS. Cardiac size and function in children with sickle cell anemia. Am Heart J . 1984 Aug. 108(2):345-50. . Bharati S, Lev M. The surgical anatomy of hypoplasia of aortic tract complex. J Thorac Cardiovasc Surg . 1984 Jul. 88(1):97-101. . Von Rueden TJ, Knight L, Moller JH, Ewards JE. Coarctation of the aorta associated with aortic valvular atresia. Circulation . Nov 1975. 52(5):951 (...) , this approach is not well documented in patients with hypoplastic left heart syndrome. Sano’s modification of the Norwood procedure Because of high mortality that Sano and his associates observed with the conventional Norwood procedure, they performed right ventricular outflow–to–pulmonary artery Gore-Tex graft anastomosis to provide for the pulmonary blood flow instead of conventional modified aortopulmonary shunt. [ , ] Significant improvement was demonstrated in both immediate and late mortality

2014 eMedicine Pediatrics

477. Hypoplastic Left Heart Syndrome (Follow-up)

for ventriculoaortic reconstitution. Br Heart J . 1976 Aug. 38(8):821-6. . . Balfour IC, Covitz W, Davis H, Rao PS, Strong WB, Alpert BS. Cardiac size and function in children with sickle cell anemia. Am Heart J . 1984 Aug. 108(2):345-50. . Bharati S, Lev M. The surgical anatomy of hypoplasia of aortic tract complex. J Thorac Cardiovasc Surg . 1984 Jul. 88(1):97-101. . Von Rueden TJ, Knight L, Moller JH, Ewards JE. Coarctation of the aorta associated with aortic valvular atresia. Circulation . Nov 1975. 52(5):951 (...) , this approach is not well documented in patients with hypoplastic left heart syndrome. Sano’s modification of the Norwood procedure Because of high mortality that Sano and his associates observed with the conventional Norwood procedure, they performed right ventricular outflow–to–pulmonary artery Gore-Tex graft anastomosis to provide for the pulmonary blood flow instead of conventional modified aortopulmonary shunt. [ , ] Significant improvement was demonstrated in both immediate and late mortality

2014 eMedicine Pediatrics

478. Hypoplastic Left Heart Syndrome (Overview)

study to study and include prematurity and major noncardiac malformations. Other identified risk factors include surgery in older infants (>1 mo), significant , and pulmonary venous hypertension. High Aristotle scores are also associated with poor prognosis. A study of data from the Society of Thoracic Surgeons Congenital Heart Surgery Database assessed the mortality rates and postoperative complications after the Norwood procedure in 2,557 patients. The overall mortality rate was noted as 22 (...) with sickle cell anemia. Am Heart J . 1984 Aug. 108(2):345-50. . Bharati S, Lev M. The surgical anatomy of hypoplasia of aortic tract complex. J Thorac Cardiovasc Surg . 1984 Jul. 88(1):97-101. . Von Rueden TJ, Knight L, Moller JH, Ewards JE. Coarctation of the aorta associated with aortic valvular atresia. Circulation . Nov 1975. 52(5):951-4. Jonas RA, Lang P, Hansen D, Hickey P, Castaneda AR. First-stage palliation of hypoplastic left heart syndrome. The importance of coarctation and shunt size. J

2014 eMedicine Pediatrics

479. Osler-Weber-Rendu Syndrome (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 Pediatrics

480. Cardiomyopathy, Dilated (Overview)

, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Kawasaki disease Hematological diseases Thalassemia, sickle cell disease, iron deficiency anemia Coronary artery diseases Anomalous left coronary artery from pulmonary artery, infarction Drugs Anthracycline, cyclophosphamide, chloroquine, iron overload Endocrine diseases Hypothyroidism, hyperthyroidism, hypoparathyroidism, pheochromocytoma, hypoglycemia Metabolic disorders Glycogen-storage diseases, carnitine deficiency, fatty acid (...) ataxia, Kearns-Sayre syndrome, other muscular dystrophies Nutritional factors Kwashiorkor, pellagra, thiamine deficiency, selenium deficiency Collagen vascular diseases Rheumatic fever, rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Kawasaki disease Hematological diseases Thalassemia, sickle cell disease, iron deficiency anemia Coronary artery diseases Anomalous left coronary artery from pulmonary artery, infarction Drugs Anthracycline, cyclophosphamide, chloroquine, iron

2014 eMedicine Pediatrics

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