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| | s001108a - Pyropoikilocytosis |
 | | Molecular determinants of clinical expression of hereditary elliptocytosis and pyropoikilocytosis. | | | All other pertinent laboratory studies (eg, immune hemolysis, sepsis, hereditary spherocytosis, etc) were noncontributory. | | | A pathogenetic and probably causal relationship with apparent elliptocytosis seems clear as three sibs have that condition. |
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http://www.emory.edu/WHSCL/grady/amreport/litsrch00/s001108a.html
(3375 words)
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| | Statements of Principles — hereditary spherocytosis |
| | (b) the relationship suggested between the hereditary spherocytosis and the particular service of a person is a relationship set out in paragraph 8(1)(e), 9(1)(e) or 70(5)(d) of the Act. | | | (b) the relationship suggested between the hereditary spherocytosis and the particular service of a person is a relationship set out in paragraph 8(1)(e), 9(1)(e), 70(5)(d), or 70(5A)(d) of the Act. | | | The factor set out in paragraph 1(a) applies only where: |
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http://www.dva.gov.au/pensions/statemnt/p014.htm
(926 words)
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| | Case of the Month September 2002 |
| | This, with the patient history and the strong family history, is indicative of hereditary spherocytosis. | | | The patient in this case was certain that her pain would be relieved by splenectomy because several family members had experienced pain that was relieved by splenectomy. | | | Hereditary spherocytosis may range from a clinically asymptomatic condition to a full-blown hemolytic anemia. |
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http://www.hsc.wvu.edu/som/pathology/medtechcases/cases2002/case9sep02/mtcasesep02discussion.htm
(290 words)
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| | HealthcarePros |
| | Hereditary spherocytosis is the most common inherited hemolytic anemia in Caucasians, with an incidence of approximately 1 in 5,000. | | | More than 400 affected children have been registered in our program during the past 20 years. | | | A splenectomy for hereditary spherocytosis may also cause an increased risk of stroke, myocardial infarction and pulmonary hypertension. |
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http://www.childrens.com/Healthcare_professionals/Faxnotes/01may14.cfm
(432 words)
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| | BloodGivers.com --- Blood Disorders - Spherocytosis |
| | A child has 25% to 50% chance of inheriting the condition from its parent having this condition. | | | The condition can occur from infancy to old age. | | | Three, if there is a consequential failure of bone marrow, the patient can suffer from heart failure. |
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http://www.bloodgivers.com/spherocytosis.asp
(338 words)
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| | [No title] |
| | (1993) defined the molecular defect responsible for clinically asymptomatic hereditary elliptocytosis and hereditary pyropoikilocytosis (HPP) in 2 unrelated families from Cagliari, Sardinia. | | | The family had previously been studied by Goodman et al. | | | Biochemical studies were consistent with the parents being heterozygous for alpha-I/74 hereditary elliptocytosis and the proband being homozygous. |
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http://zeta.embl-heidelberg.de:8000/srs5bin/cgi-bin/wgetz?-e+[omim-id:182870]
(3621 words)
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| | American Family Physician: Hereditary spherocytosis |
| | Friedman and associates[5] described five patients in the seventh through ninth decades of life who were found to have hereditary spherocytosis. | | | Hereditary spherocytosis should be considered in patients exhibiting splenomegaly, anemia and jaundice. | | | Splenectomy may be essentially curative in the majority of patients. |
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http://www.findarticles.com/p/articles/mi_m3225/is_n2_v39/ai_7553269
(1041 words)
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| | Anemia |
| | Failure to give appropriate genetic counseling for hereditary forms of anemia | | | Pathophysiology: Chronic anemia can be primary or secondary. | | | Anemia that persists for 6 months or more (eg, hereditary spherocytosis [HS]) is clearly chronic; however, an anemia that lasts only 2 months (eg, iron deficiency that is being treated) also should be considered a chronic anemia, and other explanations must be sought. |
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http://razianesth.freeservers.com/Anemia.htm
(2628 words)
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| | NORD - Anemia, Hereditary Spherocytic Hemolytic |
| | During an aplastic crisis, a person with Hereditary Spherocytic Hemolytic Anemia should seek immediate medical attention. | | | Studies are being conducted in the use of immunoglobulin (human) as a treatment for Hereditary Spherocytic Hemolytic Anemia. | | | Genetic counseling will be of benefit for people with Hereditary Spherocytic Hemolytic Anemia and their families. |
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http://www.indwes.edu/Faculty/bcupp/things/Blood/HereditarySpherocyticHemolyticAnemia.html
(1955 words)
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| | Effectiveness of the partial splenectomy in the treatment of hereditary spherocytosis |
| | We analyzed the main hematological and clinical features in 13 patients with hereditary spherocytosis submitted to partial splenectomy. | | | Although the number of patients and the post-surgical follow-up is reduced, the hematological recovery observed in our cases suggest that partial splenectomy is a beneficial surgical procedure for the treatment of the typical and severe form of hereditary spherocytosis. | | | Effectiveness of the partial splenectomy in the treatment of hereditary spherocytosis |
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http://www.imbiomed.com.mx/Innsz/Nnv52n3/english/Znn003-01.html
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| | Partial Splenectomy Helps Some Hereditary Blood Disorders |
| | This natural breakdown process can be accelerated in certain hereditary anemias. | | | "The historic treatment of hereditary spherocytosis is to remove the child's spleen. | | | "Sixteen of the 25 children had hereditary spherocytosis and nine had other disorders," Dr. Oldham explained. |
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http://healthlink.mcw.edu/article/1031002457.html
(738 words)
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| | eMedicine - Spherocytosis, Hereditary : Article by Gus Gonzalez, MD |
| | Medicine is a constantly changing science and not all therapies are clearly established. | | | Background: Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity of clinical features, ranging from an asymptomatic condition to fulminant hemolytic anemia. | | | Hereditary hemolytic anemia has been described in patients with a complete deficiency of protein 4.2. |
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http://www.emedicine.com/med/topic2147.htm
(3784 words)
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| | hereditary spherocytosis : find related signs & medical symptoms |
| | Your use of this site is subject to certain terms and conditions. | | | hereditary spherocytosis : find related signs & medical symptoms | | | Select the links below to find your symptoms from A to Z. Diseases |
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http://www.myelectronicmd.com/getlinks.php?id=1201
(257 words)
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| | Spherocytosis - Wikipedia, the free encyclopedia |
| | The standard treatment for spherocytosis is splenectomy, the surgical removal of the spleen. | | | Experimental gene therapy exists to treat spherocytosis in lab mice; however, this treatment has not yet been tried on humans and because of the risks involved in human gene therapy, it may never be. | | | Spherocytosis is an auto-hemolytic anemia (a disease of the blood) characterized by the production of red blood cells that are sphere-shaped, rather than donut-shaped. |
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http://en.wikipedia.org/wiki/Spherocytosis
(576 words)
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| | Treatment of Anemia in Children at Mayo Clinic in Rochester, Minn. |
| | If a chronic disorder is diagnosed, it may be necessary to treat the disorder before controlling the anemic condition. | | | Anemia induced by a disorder such as sickle cell anemia, hereditary spherocytosis or thalassemia may require ongoing treatments. | | | The only known cure for most of these conditions is a bone marrow transplant. |
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http://www.mayoclinic.org/peds-anemia-rst
(358 words)
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| | Pathology |
| | Acquired stomatocytosis may be seen in liver disease and hyperalimentation (lipid) therapy. | | | A chronically anemic individual with a normal MCV would fit with possible hereditary spherocytosis. | | | Hereditary stomatocytosis is an autosomal dominant disorder, in which a membrane abnormality leads to increased permeability to sodium. |
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http://www.med-ed.virginia.edu/courses/path/innes/rcd/membrane.cfm
(749 words)
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| | Hereditary spherocytosis - Wikipedia, the free encyclopedia |
| | This is termed spherocytosis; there are various other causes for this phenomenon (other forms of hemolysis), but it is typically more marked in hereditary spherocytosis. | | | Hereditary spherocytosis is a genetic disorder of the red blood cells (called "erythrocytes") that makes them less flexible and, therefore, more prone to hemolysis. | | | In longstanding cases and in patients who have taken iron supplementation or received numerous blood transfusions, iron overload may be a significant problem, being a potential cause of cardiomyopathy and liver disease. |
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http://en.wikipedia.org/wiki/Hereditary_spherocytosis
(473 words)
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| | Indian Pediatrics - Editorial |
| | Fifty percent of patients with hereditary spherocytosis give a history of jaundice in the neonatal period but it is often passed over as physiological jaundice(2). | | | She has been asympto-matic with a hemoglobin of 11 g/dL. | | | Diagnosis of hereditary spherocytosis is suspected when spherocytes are seen on the peripheral smear. |
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http://www.indianpediatrics.net/feb2004/feb-199.htm
(351 words)
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| | spherocytosis |
| | Children with spherocytosis require immunization against the pneumococcus bacterium and prohylactic antibiotic treatment as well to lessen the risk of sepsis. | | | Definitive treatment of spherocytosis is surgical removal of the spleen, but this is delayed as long as possible in children (usually age 5 or 6) because of the vital role the spleen plays in protecting us against overwhelming infections. | | | The excessive rate of breakdown of red cells in the baby's bloodstream can cause severe jaundice and may require exchange transfusion. |
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http://www.drhull.com/EncyMaster/S/spherocytosis.html
(257 words)
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| | Archives of Medical Research |
| | The authors only included as controls patients with mechanical heart valve prosthesis and malignant hematological diseases who might or might not have had hemolytic anemia, and healthy blood donors. | | | (1), and were surprised at the 100% sensitivity and specificity for that diagnostic test, data interpreted as indicating that CHT is pathognomonic for Hereditary Spherocytosis (HS). | | | Archives of Medical Research is proud to have Esther Orozco-Orozco, Ph.D. as a member of its Editorial Committee, and congratulates Dr. Orozco effusively for the recent awarding to her of the Pasteur Medal. |
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http://www.unam.mx/serv_hem/revistas/medical/1998/spring/15.html
(1168 words)
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| | PEDIATRIC ANEMIA |
| | Chronic disease, chronic liver disease; nonanemic hemoglobinopathy (e.g., AS, AC); transfusion; chemotherapy; chronic myelocytic leukemia; hemorrhage; hereditary spherocytosis | | | Autoimmine hemolytic anemia, alloimmune hemolysis, hereditary spherocytosis, some cases of Heinz body hemolytic anemias | | | May be normal immediately after a hemolytic episode as older, more enzyme-deficient cells have been lysed. |
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http://webpages.charter.net/saabrio/HEMONC_Pediatric_Anemia.htm
(1468 words)
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| | hereditary spherocytosis -- Encyclopædia Britannica |
| | Such hereditary differences may be caused by many factors. | | | inability to coordinate voluntary muscular movements; term also usually describes unsteady, lurching gait; most ataxias are hereditary and caused by degeneration of spinal cord, brain, or other parts of nervous system; other symptoms include tremors in head and arms, slurred and slow speech, skeletal deformities, and muscle weakness; Friedreich's ataxia most common form;... | | | More results on "hereditary spherocytosis" when you join. |
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http://www.britannica.com/eb/article-9040127?tocId=9040127
(777 words)
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| | hereditary spherocytosis - General Practice Notebook |
| | The disorder is characterised by spherocytosis and increased numbers of reticulocytes on the peripheral blood smear. | | | Hereditary spherocytosis is a disorder of the red blood cell membrane, leading to haemolytic anaemia. | | | It is inherited as an autosomal dominant condition often linked to chromosome 8. |
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http://www.gpnotebook.co.uk/medwebpage.cfm?ID=1597308936
(185 words)
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| | Archives of Medical Research |
| | The clinical suspicion of hereditary spherocytosis (HS) must be confirmed at the clinical laboratory. | | | Archives of Medical Research Volume 28, No. 2, pp. | | | The osmotic fragility test (OFT) and the autohemolysis test (AHT) are the worldwide accepted assays to establish a definite diagnosis of HS; however, they have some disadvantages. |
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http://www.unam.mx/serv_hem/revistas/medical/1997/winter/16.html
(320 words)
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| | Physiopathology |
| | Other less frequent mutations are also responsible for hereditary spherocytosis and are encountered in the spectrin genes or the protein 4.2 gene. | | | Hereditary spherocytosis is reported as the most frequent hereditary hemolytic anemia in Western popultations, its prevalence being estimated at 1 per 5000. | | | In most of the cases these mutuations are responsible for a decrease in protein production which leads to the decrease or the absence of one or more red cell membrane proteins. |
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http://erasmeinfo.ulb.ac.be/globule/English/memb_phyp.htm
(216 words)
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| | HEREDITARY SPHEROCYTOSIS |
| | A hereditary disorder that leads to a chronic hemolytic anemia due to an abnormality in the red blood cell membrane. | | | This disorder is caused by a defective gene. | | | The medical glossary has been made possible by a generous donation from: |
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http://www.medhelp.org/glossary/new/gls_2227.htm
(90 words)
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| | Hereditary Spherocytosis Page-Information and links |
| | This is my attempt to organize links to various sources to make research a bit easier. | | | Hereditary Spherocyotsis runs in my family and, in researching it on the web, I became frustrated that the information I was seeking was scattered all over the place. | | | In addition to HS, many of the links will take you to web pages with information on many other different blood disorders including ITP, sickle-cell anemia, and thalassemia. |
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http://www.angelfire.com/vi/maggie/spherocytosis.html
(118 words)
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| | A genetic defect in the binding of protein 4.1 to spectrin in a kindred with hereditary spherocytosis. |
| | Indirect evidence suggests that the genetic defect in hereditary spherocytosis lies in the erythrocyte membrane skeleton, a submembranous meshwork of proteins (principally spectrin, actin, and protein 4.1) responsible for membrane shape and structural stability. | | | Presumable, the nonfunctional spectrin was the product of the autosomal dominant gene responsible for the hereditary spherocytosis in this kindred. | | | One population (41 +/- 2 per cent) lacked the ability to bind 4.1, but the other functioned normally. |
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http://www.arclab.org/medlineupdates/abstract_6215583.html
(207 words)
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| | Hemic and Lymphatic Diseases |
| | Guidelines on Therapeutic products to treat haemophilia and other hereditary coagulation disorders - UK Haemophilia Centre, Oxford | | | Hereditary Spherocytosis Clinical Resources [links; MacCall and Cleveland] | | | A case of Hereditary Spherocytosis - Queens Univ. (CA) |
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http://www.mic.ki.se/Diseases/C15.html
(1903 words)
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| | anemiadaphne1 |
| | C (normally don't lyse until 49 deg C) Treatment = frequent transfusion | | | Peripheral blood = 10 - 50% stomatocytes in hereditary stomatocytosis | | | Increased osmotic fragility (Increased permeability to both Sodium and Potassium resulting in increased water flowing into the cell "swollen cells" |
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http://www.ic.arizona.edu/ic/srl/heme/anemiadaphne6.html
(103 words)
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| | Hereditary spherocytosis |
| | - reduces spherocytosis -> Hb levels return to normal |
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http://homepage.mac.com/guitarbloke/Surgical_sieve/Haematology/Haemolytic_anaemias/Hereditary_spherocytosis.html
(186 words)
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| | aplastic anemia -- Encyclopædia Britannica |
| | (also called Cooley's anemia, Mediterranean anemia, or hereditary leptocytosis), a group of blood disorders characterized by a deficiency of hemoglobin, the blood protein that supplies oxygen to tissues. | | | Its cause is an inherited abnormality in the synthesis of one or more of the polypeptide chains that make up the globin part of hemoglobin. |
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http://www.britannica.com/eb/article?tocId=9008007
(822 words)
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| | Energy Citations Database (ECD) - Energy and Energy-Related Bibliographic Citations |
| | Availability information may be found in the Availability, Publisher, Research Organization, Resource Relation and/or Author (affiliation information) fields and/or via the "Full-text Availability" link. | | | Energy Citations Database (ECD) Document #7321231 - Hereditary spherocytosis |
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http://www.osti.gov/energycitations/product.biblio.jsp?osti_id=7321231
(64 words)
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| | Hereditary spherocytosis in zebrafish riesling illustrates evolution of erythroid beta-spectrin structure, and function ... |
| | Hereditary spherocytosis in zebrafish riesling illustrates evolution of erythroid beta-spectrin structure, and function in red cell morphogenesis and membrane stability -- Liao et al. | | | Hereditary spherocytosis in zebrafish riesling illustrates evolution of erythroid beta-spectrin structure, and function in red cell morphogenesis and membrane stability | | | Articles by Liao, E. Articles by Zon, L. Development, Vol 127, Issue 23 5123-5132, Copyright © 2000 by Company of Biologists |
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http://dev.biologists.org/cgi/content/abstract/127/23/5123
(620 words)
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| | ETH e-collection |
| | Effect of splenectomy on opsonization and deformability of erythrocytes in hereditary spherocytosis with band 3 and spectrin/ankyrin deficiency |
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http://e-collection.ethbib.ethz.ch/show?type=diss&nr=14167
(30 words)
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