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Topic: Hereditary spherocytosis

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	Hereditary spherocytosis definition - Medical Dictionary definitions of popular medical terms
		Hereditary spherocytosis: A genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice (yellowing) and splenomegaly (enlargement of the spleen).
		Hereditary spherocytosis is most common in people of northern European ancestry.
		The treatment of hereditary spherocytosis is to remove the spleen (splenectomy).
	www.medterms.com /script/main/art.asp?articlekey=3724 (532 words)

	Hereditary spherocytosis - Wikipedia, the free encyclopedia
		Hereditary spherocytosis is a genetic disorder of the red blood cells (called "erythrocytes") that makes them less flexible and, therefore, more prone to hemolysis.
		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 caused by a variety of molecular defects in the genes that code for spectrin, ankyrin, protein 4.1, and other erythrocyte membrane proteins.
	en.wikipedia.org /wiki/Hereditary_spherocytosis (473 words)

	Spherocytosis - Wikipedia, the free encyclopedia
		They are most commonly found in immunologically-mediated hemolytic anemias and in hereditary spherocytosis, but the former would have a positive direct Coombs antibody test and the latter would not.
		Spherocytosis is an autosomal dominant trait, most commonly (though not exclusively) found in Northern European and Japanese families, although an estimated 25% of cases are due to spontaneous mutations.
		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.
	en.wikipedia.org /wiki/Spherocytosis (576 words)

	American Family Physician: Hereditary spherocytosis (Site not responding. Last check: 2007-11-03)
		Hereditary spherocytosis (also known as congenital hemolytic jaundice) is an inherited hemolytic anemia characterized by defective erythrocyte membranes.
		Hereditary spherocytosis is usually diagnosed in childhood, and hyperbilirubinemia in infancy may be the first manifestation.
		Because the hemolysis associated with hereditary spherocytosis occurs in the spleen and not intravascularly, hemoglobinemia is not seen.
	www.findarticles.com /p/articles/mi_m3225/is_n2_v39/ai_7553269 (1041 words)

	Statements of Principles — hereditary spherocytosis (Site not responding. Last check: 2007-11-03)
		"hereditary spherocytosis" means a congenital haemolytic condition arising from a defect in one of the proteins in the red blood cell membrane leading to a decreased ratio of surface area to volume of the cell and the consequent development of spherocytes, attracting ICD code 280.0;
		"Hereditary spherocytosis" means a congenital haemolytic condition arising from a defect in one of the proteins in the red blood cell membrane leading to a decreased ratio of surface area to volume of the cell and the consequent development of spherocytes and attracting the ICD code of 282.0.
		In hereditary spherocytosis the life-span of the red blood cell is shortened because of a defect in the red cell membrane and, as the bone marrow is not able to adequately replenish the prematurely destroyed cells, anaemia may result.
	www.dva.gov.au /pensions/statemnt/p014.htm (926 words)

	s001108a - Pyropoikilocytosis (Site not responding. Last check: 2007-11-03)
		Hereditary elliptocytosis, spherocytosis and related disorders: consequences of a deficiency or a mutation of membrane skeletal proteins.
		Abstract: Hereditary elliptocytosis (HE) is a heterogeneous disorder of red blood cells frequently associated with abnormal limited tryptic digestion of the alpha I domain of spectrin and impaired spectrin dimer self-association.
		Hereditary spherocytosis, elliptocytosis and pyropoikilocytosis represent a group of disorders that are due to deficiency or dysfunction of one of the membrane skeletal proteins (Fig.
	www.emory.edu /WHSCL/grady/amreport/litsrch00/s001108a.html (3375 words)

	Hereditary Spherocytosis and Hereditary Elliptocytosis: Anemias Caused by Hemolysis: Merck Manual Professional
		Hereditary spherocytosis and hereditary elliptocytosis are congenital RBC membrane disorders.
		Hereditary spherocytosis (chronic familial icterus; congenital hemolytic jaundice; chronic acholuric jaundice; familial spherocytosis; spherocytic anemia) is an autosomal dominant disease with variable gene penetrance.
		In hereditary spherocytosis, the cell membrane surface area is decreased disproportionately to the intracellular content.
	www.merck.com /mmpe/sec11/ch131/ch131e.html (556 words)

	Case of the Month September 2002 (Site not responding. Last check: 2007-11-03)
		The abnormal “tail” on the graph of the incubated osmotic fragility is consistent with spherocytosis.
		This, with the patient history and the strong family history, is indicative of hereditary spherocytosis.
		Hereditary spherocytosis may range from a clinically asymptomatic condition to a full-blown hemolytic anemia.
	www.hsc.wvu.edu /som/pathology/medtechcases/cases2002/case9sep02/mtcasesep02discussion.htm (290 words)

	Physiopathology (Site not responding. Last check: 2007-11-03)
		Hereditary spherocytosis is reported as the most frequent hereditary hemolytic anemia in Western popultations, its prevalence being estimated at 1 per 5000.
		Hereditary spherocytosis is found in all populations but its prevalence is very low in African populations.
		Other less frequent mutations are also responsible for hereditary spherocytosis and are encountered in the spectrin genes or the protein 4.2 gene.
	erasmeinfo.ulb.ac.be /globule/English/memb_phyp.htm (216 words)

	CIGNA - Anemia, Hereditary Spherocytic Hemolytic
		Hereditary Spherocytic Hemolytic Anemia is a rare blood disorder characterized by defects within red blood cells (intracorpuscular) that result in a shortened survival time for these cells.
		Hereditary Spherocytic Hemolytic Anemia is caused by an inherited metabolic defect.
		Hereditary Nonspherocytic Hemolytic Anemia is a group of rare inherited blood disorders characterized by defective red blood cells that are not sphere-shaped.
	www.cigna.com /healthinfo/nord81.html (1809 words)

	Anemia, Hereditary Spherocytic Hemolytic
		Hereditary Spherocytic Hemolytic Anemia is a rare blood disorder characterized by defects within red blood cells (intracorpuscular) that result in a shortened survival time for these cells.
		The sphere shape of the red blood cells is the hallmark of Hereditary Spherocytic Hemolytic Anemia, and this abnormality may be identified under a microscope.
		Hereditary Spherocytic Hemolytic Anemia is caused by an inherited metabolic defect.
	my.webmd.com /hw/health_guide_atoz/nord81.asp (465 words)

	spherocytosis (Site not responding. Last check: 2007-11-03)
		Spherocytosis is an inherited disorder of the red blood cells.
		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.
		Children with spherocytosis require immunization against the pneumococcus bacterium and prohylactic antibiotic treatment as well to lessen the risk of sepsis.
	www.drhull.com /EncyMaster/S/spherocytosis.html (257 words)

	Pathology
		Hereditary spherocytosis is characterized by numerous spherocytes on the PBS (right).
		Hereditary elliptocytosis is usually inherited as an autosomal dominant trait.
		Hereditary stomatocytosis is an autosomal dominant disorder, in which a membrane abnormality leads to increased permeability to sodium.
	www.med-ed.virginia.edu /courses/path/innes/rcd/membrane.cfm (749 words)

	[No title]
		Some cases are harder to detect than others because hereditary spherocytosis is a syndrome with variable severity of the structural protein defect.
		Hereditary spherocytosis is associated with increased autohemolysis, with some decrease in the degree of hemolysis in the glucose-added tube.
		To specifically diagnose the protein abnormalities of hereditary spherocytosis, one must analyze red cell membrane proteins in gels, which separate proteins based on size and change.
	www.cap.org /apps/portlets/contentViewer/show.do?printFriendly=true&contentReference=cap_today%2Fq_and_a%2F0805qa.html (596 words)

	HealthcarePros
		Hereditary spherocytosis is the most common inherited hemolytic anemia in Caucasians, with an incidence of approximately 1 in 5,000.
		The life-long risk of this complication is illustrated by the case of a young child whose maternal grandfather, with hereditary spherocytosis, died of overwhelming pneumococcal sepsis 47 years following his successful splenectomy.
		A splenectomy for hereditary spherocytosis may also cause an increased risk of stroke, myocardial infarction and pulmonary hypertension.
	www.childrens.com /Healthcare_professionals/Faxnotes/01may14.cfm (432 words)

	BloodGivers.com --- Blood Disorders - Spherocytosis
		However hereditary spherocytosis is generally a mild disease which does not cause many complications.
		Spherocytosis can be passed from parents to their offspring.
		Families with a history of spherocytosis should have their offspring screened for this disorder.
	www.bloodgivers.com /spherocytosis.asp (338 words)

	eMedicine - Spherocytosis, Hereditary : Article by Gus Gonzalez, MD
		Background: Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity of clinical features, ranging from an asymptomatic condition to fulminant hemolytic anemia.
		Biochemical spectrin deficiency and the degree of spectrin deficiency are reported to correlate with the extent of spherocytosis, the degree of abnormality on osmotic fragility test results, and the severity of hemolysis.
		Hereditary hemolytic anemia has been described in patients with a complete deficiency of protein 4.2.
	www.emedicine.com /med/topic2147.htm (3916 words)

	Partial Splenectomy Helps Some Hereditary Blood Disorders
		Hereditary blood disorders are serious illnesses that lead to the destruction of red blood cells, causing fatigue, anemia, jaundice and enlargement of the spleen.
		While they are relatively rare, one of the more common types of hereditary anemias is hereditary spherocytosis (HS), which often shows up in infancy or early childhood.
		In the study, 25 children with hereditary anemia had a portion of their spleen removed, preserving 10% to 20% percent of the tissue.
	healthlink.mcw.edu /article/1031002457.html (738 words)

	Indian Pediatrics - Editorial
		Hereditary spherocytosis is a rare cause of neonatal hyperbilirubinemia and Medline search from 1966 onwards revealed only one case from India(1).
		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).
		Diagnosis of hereditary spherocytosis is suspected when spherocytes are seen on the peripheral smear.
	www.indianpediatrics.net /feb2004/feb-199.htm (351 words)

	A genetic defect in the binding of protein 4.1 to spectrin in a kindred with hereditary spherocytosis. (Site not responding. Last check: 2007-11-03)
		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.
		To test this premise we systematically assayed the interactions of spectrin, the major skeletal protein, in six kindreds with autosomal dominant hereditary spherocytosis.
	www.arclab.org /medlineupdates/abstract_6215583.html (207 words)

	[No title] (Site not responding. Last check: 2007-11-03)
		In type I hereditary spherocytosis, the molecular defect is in the N-terminal portion of the spectrin beta chain (Goodman et al., 1982).
		In a family with a chronic hemolytic form of hereditary elliptocytosis and, by biochemical analysis, a truncated beta-spectrin chain with deletion of a peptide fragment near the C-terminus, Gallagher et al.
		1 to spectrin in a kindred with hereditary spherocytosis.
	zeta.embl-heidelberg.de:8000 /srs5bin/cgi-bin/wgetz?-e+[omim-id:182870] (3621 words)

	Combination of Two Mutant Alpha Spectrin Alleles Underlies a Severe Spherocytic Hemolytic Anemia -- Wichterle et al. 98 ...
		Characterization of the underlying molecular defect in hereditary spherocytosis associated with spectrin deficiency.
		-spectrin synthesis in burst-forming units-erythroid in lethal hereditary spherocytosis.
		Recessive hereditary spherocytosis is associated with an abnormal alpha spectrin subunit.
	www.jci.org /cgi/content/full/98/10/2300 (4641 words)

	AccessMedicine - Williams Hematology: Disorders of the Red Blood Cell Membrane: Hereditary Spherocytosis, ...
		"Hereditary spherocytosis is an inherited hemolytic anemia characterized by spherically shaped erythrocytes on the blood film, reticulocytosis, and splenomegaly.
		The principal cellular defect is the propensity to lose membrane surface area during passage through the splenic circulation, leading to spherical shape and decreased deformability.
		Hereditary elliptocytosis is characterized by the presence of elliptical erythrocytes on the blood film, often with no or very slight shortening of red cell survival.
	www.accessmedicine.com /content.aspx?aID=2141946 (260 words)

	MedlinePlus Medical Encyclopedia: Congenital spherocytic anemia
		Having a family history of spherocytosis increases the risk for this disorder.
		Surgery to remove the spleen (splenectomy) cures the anemia of spherocytosis.
		Families with a history of spherocytosis should have their children screened for this disorder.
	www.nlm.nih.gov /medlineplus/ency/article/000530.htm (493 words)

	Archives of Medical Research (Site not responding. Last check: 2007-11-03)
		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.
		(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).
		As we stated (2), CHT is an easy-to-perform assay with a high degree of sensitivity and specificity that only requires a maximum of focusing to be adequately performed.
	www.unam.mx /serv_hem/revistas/medical/1998/spring/15.html (1168 words)

	hereditary spherocytosis -- Encyclopædia Britannica (Site not responding. Last check: 2007-11-03)
		More results on "hereditary spherocytosis" when you join.
		In some plant species, hereditary differences in growth are due to differences in the rate of production of hormones called gibberellins.
		He was usually chosen because of his ability and wisdom, though in a few tribes the office was hereditary.
	www.britannica.com /eb/article-9040127?tocId=9040127 (777 words)

	Congenital spherocytic anemia (Site not responding. Last check: 2007-11-03)
		A family history of spherocytosis increases the risk for this disorder.
		Splenectomy (surgical removal of the spleen) cures the anemia of spherocytosis.
		Families with a history of spherocytosis should have their children screened for this disorder.
	www.pennhealth.com /ency/article/000530.htm (446 words)

	Treatment of Anemia in Children at Mayo Clinic in Rochester, Minn. (Site not responding. Last check: 2007-11-03)
		Anemia induced by a disorder such as sickle cell anemia, hereditary spherocytosis or thalassemia may require ongoing treatments.
		Hereditary spherocytosis causes the red blood cell membrane to be smaller and rounder than normal.
		Treating hereditary spherocytosis often involves removing the spleen, a procedure which can usually be done in children older than 3.
	www.mayoclinic.org /peds-anemia-rst (358 words)

	Hereditary spherocytosis; new guidelines -- Bolton-Maggs 89 (9): 809 -- Archives of Disease in Childhood
		Hereditary spherocytosis (HS) is the commonest cause of haemolysis
		spherocytosis (HS) is the commonest cause of inherited haemolysis
		The role of prophylactic cholecystectomy during splenectomy in children with hereditary spherocytosis.
	adc.bmjjournals.com /cgi/content/full/89/9/809 (2780 words)

	Effectiveness of the partial splenectomy in the treatment of hereditary spherocytosis
		To evaluate the hematological and clinical response to partial splenectomy in a group of children with 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.
	www.imbiomed.com.mx /Innsz/Nnv52n3/english/Znn003-01.html (217 words)

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