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Pulmonary Hypertension in Sickle Cell Anemia

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301. Hematologic Disease and Pregnancy (Overview)

, 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

302. Oral Manifestations of Systemic Diseases (Overview)

) 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

303. Early Pregnancy Loss (Overview)

, 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

304. 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

305. 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

306. Erectile Dysfunction (Treatment)

each agent alone failed. Few adverse effects occur. The most common is a painful erection and urethral burning, which occurs in fewer than 10% of patients. A topical gel formulation of alprostadil for treatment of ED has been developed. [ ] However, it has not been approved for use by the FDA. Vascular endothelial growth factor One area of research has involved the use of vascular endothelial growth factor (VEGF), an angiogenic growth factor and endothelial cell mitogen. VEGF is produced (...) 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

2014 eMedicine.com

307. Oral Manifestations of Systemic Diseases (Follow-up)

) 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

308. Osler-Weber-Rendu Syndrome (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.com

309. Erectile Dysfunction (Follow-up)

each agent alone failed. Few adverse effects occur. The most common is a painful erection and urethral burning, which occurs in fewer than 10% of patients. A topical gel formulation of alprostadil for treatment of ED has been developed. [ ] However, it has not been approved for use by the FDA. Vascular endothelial growth factor One area of research has involved the use of vascular endothelial growth factor (VEGF), an angiogenic growth factor and endothelial cell mitogen. VEGF is produced (...) 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

2014 eMedicine.com

310. 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

312. 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 (...) overload. Ann Intern Med . 2004 May 18. 140(10):846; author reply 846-7. . Porter JB, Davis BA. Monitoring chelation therapy to achieve optimal outcome in the treatment of thalassaemia. Best Pract Res Clin Haematol . 2002 Jun. 15(2):329-68. . Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med . 1998 Jul 2. 339(1):5-11. . . Adams RJ, Brambilla D. Discontinuing

2014 eMedicine.com

313. Hematologic Disease and Pregnancy (Follow-up)

, 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

314. 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

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

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

316. Hypoplastic Left Heart Syndrome (Diagnosis)

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 (...) survival to the time of hospital discharge after the Norwood procedure is nearly 75%. [ ] Success rates are higher in uncomplicated cases and lower in cases in which important preoperative risk factors are present, such as age greater than 1 month, significant preoperative tricuspid insufficiency, pulmonary venous hypertension, associated major chromosomal or noncardiac abnormalities, and prematurity. Note the following: High Aristotle scores (>20) are associated with high hospital mortality and low

2014 eMedicine Pediatrics

317. Osler-Weber-Rendu Syndrome (Follow-up)

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 Pediatrics

318. 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 Pediatrics

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

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

320. The Fetus as a Patient: Prenatal Diagnosis and Fetal Therapy (Follow-up)

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

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