How to Trip Rapid Review

Step 1: Select articles relevant to your search (remember the system is only optimised for single intervention studies)

Step 2: press

Step 3: review the result, and maybe amend the or if you know better! If we're unsure of the overall sentiment of the trial we will display the conclusion under the article title. We then require you to tell us what the correct sentiment is.

470 results for

Pulmonary Hypertension in Sickle Cell Anemia

by
...
Alerts

Export results

Use check boxes to select individual results below

SmartSearch available

Trip's SmartSearch engine has discovered connected searches & results. Click to show

321. Renal Failure, Acute (Diagnosis)

Diagnosis The following conditions should be considered in the differential diagnosis of acute renal failure (ARF): Alcoholic Ketoacidosis Anemia, Sickle Cell Aneurysm, Abdominal Congestive Heart Failure and Pulmonary Edema Diabetic Ketoacidosis Hemolytic Uremic Syndrome Pediatrics, Dehydration Pediatrics, Diabetic Ketoacidosis Pediatrics, Inborn Errors of Metabolism Pediatrics, Sickle Cell Disease Pediatrics, Urinary Tract Infections and Pyelonephritis Renal Calculi Renal Failure, Chronic and Dialysis (...) hypertension White blood cell (WBC) casts - Pyelonephritis Eosinophiluria - Acute allergic interstitial nephritis, atheroembolism Crystalluria - Acyclovir, sulfonamides, methotrexate, ethylene glycol toxicity, radiocontrast agents (Mild crystalluria can be a normal finding) Previous Next: BUN The urea concentration correlates poorly with the GFR. Because urea is highly permeable to renal tubules, urea clearance varies with urine flow rate. Urea is filtered freely, but reabsorption along the tubule

2014 eMedicine Emergency Medicine

322. Oral Manifestations of Systemic Diseases (Diagnosis)

) and the progenitor cell lineage primarily effected (lymphoid or myeloid). [ , ] Systemically, dysregulated leukocyte proliferation results in a suppression of normal hematopoiesis, often leading to anemia and thrombocytopenia. Of course, as leukocytes are a critical component of the immune system, patients with leukemias are also at increased risk of infection. Cutaneous manifestations include leukemic skin infiltrations (leukemia cutis), as well as nonspecific lesions (eg, cutaneous infections, vasculitis (...) , 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

2014 eMedicine.com

323. Osler-Weber-Rendu Syndrome (Diagnosis)

Smad4) - These are described in JPHT, [ , ] which is also autosomal dominant, involves chromosome 18, and combines clinical manifestations of HHT and The first two (HHT types 1 and 2) account for approximately 85% of cases. In addition, some families show no links to any of the known loci. One patient with HHT and pulmonary hypertension with no mutation in ENG , ACVRL1 , or SMAD4 but was found to have a nonsense mutation in BMPR2 . [ ] Previous Next: Epidemiology United States statistics OWRD (ie (...) 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

2014 eMedicine.com

324. Transfusion-Induced Iron Overload (Diagnosis)

patients with transfusion-dependent thalassemia in the National Institutes of Health (NIH) registry, 23% had iron overload as documented by a liver iron concentration of 15 mg/g dry weight or greater. [ ] Around 15,000 patients with sickle cell disorder and estimated and 5,000 with myelodysplastic syndromes and other acquired refractory anemias require blood transfusions. [ ] International In a Japanese cohort of transfusion-dependent patients with myelodysplastic syndrome and aplastic anemia, one (...) . Hepatitis C virus in sickle cell disease. J Natl Med Assoc . 2003 Oct. 95(10):939-42. . Halonen P, Mattila J, Suominen P, et al. Iron overload in children who are treated for acute lymphoblastic leukemia estimated by liver siderosis and serum iron 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

2014 eMedicine.com

326. Hematologic Disease and Pregnancy (Diagnosis)

, 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 (...) Gynaecol Br Commonw . 1972 May. 79(5):396-409. . Powars DR, Sandhu M, Niland-Weiss J, et al. Pregnancy in sickle cell disease. Obstet Gynecol . 1986 Feb. 67(2):217-28. . Smith JA, Espeland M, Bellevue R, et al. Pregnancy in sickle cell disease: experience of the Cooperative Study of Sickle Cell Disease. Obstet Gynecol . 1996 Feb. 87(2):199-204. . Chakravarty EF, Khanna D, Chung L. Pregnancy outcomes in systemic sclerosis, primary pulmonary hypertension, and sickle cell disease. Obstet Gynecol . 2008

2014 eMedicine.com

327. Renal Failure, Acute (Treatment)

Diagnosis The following conditions should be considered in the differential diagnosis of acute renal failure (ARF): Alcoholic Ketoacidosis Anemia, Sickle Cell Aneurysm, Abdominal Congestive Heart Failure and Pulmonary Edema Diabetic Ketoacidosis Hemolytic Uremic Syndrome Pediatrics, Dehydration Pediatrics, Diabetic Ketoacidosis Pediatrics, Inborn Errors of Metabolism Pediatrics, Sickle Cell Disease Pediatrics, Urinary Tract Infections and Pyelonephritis Renal Calculi Renal Failure, Chronic and Dialysis (...) hypertension White blood cell (WBC) casts - Pyelonephritis Eosinophiluria - Acute allergic interstitial nephritis, atheroembolism Crystalluria - Acyclovir, sulfonamides, methotrexate, ethylene glycol toxicity, radiocontrast agents (Mild crystalluria can be a normal finding) Previous Next: BUN The urea concentration correlates poorly with the GFR. Because urea is highly permeable to renal tubules, urea clearance varies with urine flow rate. Urea is filtered freely, but reabsorption along the tubule

2014 eMedicine Emergency Medicine

328. Osler-Weber-Rendu Disease (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 (...) haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet . 2004 Mar 13. 363(9412):852-9. . Abdalla SA, Letarte M. Hereditary haemorrhagic telangiectasia: current views on genetics and mechanisms of disease. J Med Genet . 2006 Feb. 43(2):97-110. . . Rigelsky CM, Jennings C, Lehtonen R, Minai OA, Eng C, Aldred MA. BMPR2 mutation in a patient with pulmonary arterial hypertension and suspected hereditary hemorrhagic telangiectasia. Am J Med Genet A . 2008 Oct 1. 146A(19):2551-6. . Begbie

2014 eMedicine Surgery

329. Transfusion and Autotransfusion (Follow-up)

use of whole blood transfusion is the US military buddy transfusion system, best known as the US Navy’s walking blood bank onboard naval warships, such as the USS Cole. Next: Indications Anemia and hemorrhagic shock defined Active hemorrhage resulting in shock is one of the few evidence-based established indications for transfusion. Anemia describes a diminished circulating red cell mass, expressed as grams of hemoglobin per 100 cc of whole blood. Anemia may arise as a result of external loss (...) , internal destruction, inadequate production, or a combination of these entities. While most patients experiencing active hemorrhage become anemic, anemia in itself is not necessarily an indication for transfusion. The result of severe bleeding is hemorrhagic shock, and shock is defined as the inadequate supply of oxygen to support cellular metabolism. Red cell mass repletion is only one facet of therapy for hemorrhagic shock and may not be used in every case. Goal of PRBC transfusion Based

2014 eMedicine Surgery

330. Bone Marrow Transplantation, Long-Term Effects (Diagnosis)

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 (...) patients who have had exposure to chest or neck radiation should have thyroid function tests upon entry to long-term follow-up. Any abnormalities should prompt a referral to an endocrinologist or reproductive specialist for a thorough assessment. Pulmonary dysfunction Relatively few children surviving hematopoietic stem cell transplantation (HSCT) have clinically significant pulmonary symptoms; however, as many as 33-55% have abnormal pulmonary function test (PFT) results and abnormal lung function

2014 eMedicine Pediatrics

331. Osler-Weber-Rendu Disease (Overview)

Smad4) - These are described in JPHT, [ , ] which is also autosomal dominant, involves chromosome 18, and combines clinical manifestations of HHT and The first two (HHT types 1 and 2) account for approximately 85% of cases. In addition, some families show no links to any of the known loci. One patient with HHT and pulmonary hypertension with no mutation in ENG , ACVRL1 , or SMAD4 but was found to have a nonsense mutation in BMPR2 . [ ] Previous Next: Epidemiology United States statistics OWRD (ie (...) 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

2014 eMedicine Surgery

332. Transfusion and Autotransfusion (Treatment)

use of whole blood transfusion is the US military buddy transfusion system, best known as the US Navy’s walking blood bank onboard naval warships, such as the USS Cole. Next: Indications Anemia and hemorrhagic shock defined Active hemorrhage resulting in shock is one of the few evidence-based established indications for transfusion. Anemia describes a diminished circulating red cell mass, expressed as grams of hemoglobin per 100 cc of whole blood. Anemia may arise as a result of external loss (...) , internal destruction, inadequate production, or a combination of these entities. While most patients experiencing active hemorrhage become anemic, anemia in itself is not necessarily an indication for transfusion. The result of severe bleeding is hemorrhagic shock, and shock is defined as the inadequate supply of oxygen to support cellular metabolism. Red cell mass repletion is only one facet of therapy for hemorrhagic shock and may not be used in every case. Goal of PRBC transfusion Based

2014 eMedicine Surgery

333. Osler-Weber-Rendu Disease (Follow-up)

. 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 (...) haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet . 2004 Mar 13. 363(9412):852-9. . Abdalla SA, Letarte M. Hereditary haemorrhagic telangiectasia: current views on genetics and mechanisms of disease. J Med Genet . 2006 Feb. 43(2):97-110. . . Rigelsky CM, Jennings C, Lehtonen R, Minai OA, Eng C, Aldred MA. BMPR2 mutation in a patient with pulmonary arterial hypertension and suspected hereditary hemorrhagic telangiectasia. Am J Med Genet A . 2008 Oct 1. 146A(19):2551-6. . Begbie

2014 eMedicine Surgery

334. The Fetus as a Patient: Prenatal Diagnosis and Fetal Therapy (Overview)

and ultrasonography for nuchal translucency) Second-trimester screening tests may include the following: Maternal serum alpha-fetoprotein (MSAFP) Serum β-hCG Unconjugated estriol (uE3) Inhibin A Maternal hexosaminidase test Fetal cells in maternal circulation The “triple screen” includes MSAFP, serum β-hCG, and uE3; the addition of inhibin A results in the “quadruple screen.” The panel findings, along with gestational age, can suggest a number of fetal abnormalities, depending on the results pattern. Prenatal (...) , population screening programs have been effective in identifying carriers, including couples whose offspring are at risk. Couples with identified genetic risk can be counseled to undergo definitive invasive testing, such as chorionic villus sampling or amniocentesis (discussed below). [ ] Fetal cells in maternal circulation Building on the awareness that fetal cells can be found in the maternal circulation (the basis of the Kleihauer-Betke test for maternal-fetal transfusion), researchers have expended

2014 eMedicine Pediatrics

335. Thalassemia (Overview)

): . Barnett CF, Hsue PY, Machado RF. Pulmonary hypertension: an increasingly recognized complication of hereditary hemolytic anemias and HIV infection. JAMA . 2008 Jan 23. 299(3):324-31. . Morris CR, Gladwin MT, Kato GJ. Nitric oxide (...) type of anemia in children of Italian origin. He noted abundant nucleated red blood cells (RBCs) in the peripheral blood, which he initially thought was erythroblastic anemia, an entity that von Jaksh described earlier. Before long, Cooley realized that erythroblastemia is neither specific nor essential in this disorder and that the term erythroblastic anemia was nothing but a diagnostic catchall. Although Cooley was aware of the genetic nature of the disorder, he failed to investigate

2014 eMedicine Pediatrics

336. Thalassemia Intermedia (Overview)

with this disease can have symptoms that range from mild anemia, with only a rare need for transfusions, to chronic hemolysis and the development later in life of transfusion dependence. The following are histologic images from patients with thalassemia intermedia. Peripheral blood film in thalassemia intermedia. Basophilic stippling in thalassemia intermedia. Nucleated red blood cell in thalassemia intermedia. See also , , , , , and . Next: Pathophysiology Normal hemoglobin is a tetramer composed of two alpha (...) . Haematologica . 2007 May. 92(5):658-65. . . Succar J, Musallam KM, Taher AT. Thalassemia and venous thromboembolism. Mediterr J Hematol Infect Dis . 2011. 3(1):e2011025. . . Karimi M, Khanlari M, Rachmilewitz EA. Cerebrovascular accident in beta-thalassemia major (beta-TM) and beta-thalassemia intermedia (beta-TI). Am J Hematol . 2008 Jan. 83(1):77-9. . Morris CR, Gladwin MT, Kato GJ. Nitric oxide and arginine dysregulation: a novel pathway to pulmonary hypertension in hemolytic disorders. Curr Mol Med

2014 eMedicine Pediatrics

337. 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 (...) these abnormalities resolve. Consequently, most infants should remain in room air with acceptable oxygen saturation (pulse oximeter) in the low 70s. An exceptional circumstance is the infant with severe hypoxemia caused by pulmonary venous hypertension. Achieving a slightly higher PaCO 2 , in the range of 45-50 mm Hg, can increase pulmonary vascular resistance. This can be accomplished by intubation, sedation, mechanical hypoventilation, or the addition of nitrogen or carbon dioxide (FIO 2 of 15-19

2014 eMedicine Pediatrics

338. Bone Marrow Transplantation, Long-Term Effects (Follow-up)

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 (...) patients who have had exposure to chest or neck radiation should have thyroid function tests upon entry to long-term follow-up. Any abnormalities should prompt a referral to an endocrinologist or reproductive specialist for a thorough assessment. Pulmonary dysfunction Relatively few children surviving hematopoietic stem cell transplantation (HSCT) have clinically significant pulmonary symptoms; however, as many as 33-55% have abnormal pulmonary function test (PFT) results and abnormal lung function

2014 eMedicine Pediatrics

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

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 (...) patients who have had exposure to chest or neck radiation should have thyroid function tests upon entry to long-term follow-up. Any abnormalities should prompt a referral to an endocrinologist or reproductive specialist for a thorough assessment. Pulmonary dysfunction Relatively few children surviving hematopoietic stem cell transplantation (HSCT) have clinically significant pulmonary symptoms; however, as many as 33-55% have abnormal pulmonary function test (PFT) results and abnormal lung function

2014 eMedicine Pediatrics

340. Renal Failure, Acute (Overview)

Diagnosis The following conditions should be considered in the differential diagnosis of acute renal failure (ARF): Alcoholic Ketoacidosis Anemia, Sickle Cell Aneurysm, Abdominal Congestive Heart Failure and Pulmonary Edema Diabetic Ketoacidosis Hemolytic Uremic Syndrome Pediatrics, Dehydration Pediatrics, Diabetic Ketoacidosis Pediatrics, Inborn Errors of Metabolism Pediatrics, Sickle Cell Disease Pediatrics, Urinary Tract Infections and Pyelonephritis Renal Calculi Renal Failure, Chronic and Dialysis (...) hypertension White blood cell (WBC) casts - Pyelonephritis Eosinophiluria - Acute allergic interstitial nephritis, atheroembolism Crystalluria - Acyclovir, sulfonamides, methotrexate, ethylene glycol toxicity, radiocontrast agents (Mild crystalluria can be a normal finding) Previous Next: BUN The urea concentration correlates poorly with the GFR. Because urea is highly permeable to renal tubules, urea clearance varies with urine flow rate. Urea is filtered freely, but reabsorption along the tubule

2014 eMedicine Emergency Medicine

To help you find the content you need quickly, you can filter your results via the categories on the right-hand side >>>>