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Topic: Paroxysmal nocturnal hemoglobinuria

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	eMedicine - Paroxysmal Nocturnal Hemoglobinuria : Article by Emmanuel C Besa
		Paroxysmal nocturnal hemoglobinuria (PNH) is a descriptive term for the clinical manifestation of red cell breakdown with release of hemoglobin into the urine that is manifested most prominently by dark-colored urine in the morning.
		PNH may occur at any age, from children (10%) as young as 2 years to adults as old as 83 years, but it frequently is found among adults, with a median age at the time of diagnosis of 42 years (range, 16-75 y) from a series in England of 80 consecutive patients.
		PNH III cells are markedly sensitive, requiring one fifteenth to one twentieth of complement for an equal degree of lysis.
	www.emedicine.com /med/topic2696.htm (5075 words)

	Paroxysmal Nocturnal Hemoglobinuria (Site not responding. Last check: 2007-10-15)
		Cytogenetic and morphological abnormalities in paroxysmal nocturnal haemoglobinuria.
		Glycosyl phosphatidylinositol (GPI)-anchored molecules and the pathogenesis of paroxysmal nocturnal hemoglobinuria.
		Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder characterized by clonal blood cells that are deficient in glycosylphosphatidylinositol-anchored proteins because of somatic mutations of the PIG-A gene.
	www.thedoctorsdoctor.com:16080 /diseases/pnh.htm (8559 words)

	Paroxysmal nocturnal hemoglobinuria - Wikipedia, the free encyclopedia
		Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease characterised by hemolytic anemia, thrombosis and red urine due to breakdown of red blood cells.
		PNH is the only hemolytic anemia caused by an acquired intrinsic defect in the cell membrane.
		Quite paradoxically, the destruction of red blood cells (hemolysis) is neither paroxysmal or nocturnal the majority of the time (this constellation of symptoms is seen in only 25% of patients).
	en.wikipedia.org /wiki/Paroxysmal_nocturnal_hemoglobinuria (689 words)

	Hospital Practice: Paroxysmal Nocturnal Hemoglobinuria (Site not responding. Last check: 2007-10-15)
		In addition, PNH is associated with increased susceptibility to bacterial infection, presumably secondary to neutropenia; this is related to a third association--namely, with bone marrow failure, reminiscent of aplastic anemia.
		Bessler M et al: Paroxysmal nocturnal haemoglobinuria (PNH) is caused by somatic mutations in the PIG-A gene.
		Takeda J et al: Deficiency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria.
	www.hosppract.com /genetics/9709gen.htm (3997 words)

	Hemoglobinuria - paroxysmal nocturnal hemoglobinuria pnh
		Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease characterised by hemolytic anemia, thrombosis and red urine due to breakdown of red blood cells.
		PNH is the only hemolytic anemia caused by an acquired intrinsic defect in the cell membrane.
		The proteins paroxysmal nocturnal hemoglobinuria which use this anchor are needed for platelts to clot properly, and their absence leads to a hypercoagulable state.
	www.medicalgeo.com /Med-Diseases-H---Hi/Hemoglobinuria.html (767 words)

	Sloan-Kettering - Paroxysmal Nocturnal Hemoglobinuria
		Paroxysmal nocturnal hemoglobinuria (PNH) results from a mutation in a hematopoietic stem cell; the mutated cell then expands in the bone marrow.
		PNH patients can also develop aplastic anemia, which is accompanied by a decreased platelet and/or white blood count in addition to anemia.
		PNH can be cured by bone marrow transplantation; this procedure should be considered on an individual basis, based on the patient's age and symptoms.
	www.mskcc.org /mskcc/html/5483.cfm (222 words)

	Rare Pediatric Disease Database
		Paroxysmal nocturnal hemoglobinuria (PNH) is a general term used to describe a rare disorder that can affect many different kinds of blood cells.
		People with PNH will usually have three major characteristics: destruction of their red blood cells, bone marrow that is unable to make the correct cells (aplastic anemia) and an increased risk of having blood clots in their blood vessels (thrombotic events).
		PNH is a rare disease that can happen to anyone from the age of two to 83, but it tends to occur more in teenagers or young adults.
	www.madisonsfoundation.org /content/3/1/display.asp?did=545 (1434 words)

	Hemoglobinuria, Paroxysmal Nocturnal
		Paroxysmal Nocturnal Hemoglobinuria is a decrease of red blood cells (anemia) caused by a defect in the membrane of the red blood cells.
		Symptoms of Paroxysmal Nocturnal Hemoglobinuria include severe abdominal or back pain occurring during the phase where the oxygen carrying portion of the red blood cell (hemoglobin) is released from the red blood cells (hemolysis).
		Paroxysmal Nocturnal Hemoglobinuria is a disease that may occur at any age, but is most common in men between the ages of 20 to 45.
	hw.healthdialog.com /kbase/nord/nord670.htm (1117 words)

	Paroxysmal Nocturnal Hemoglobinuria
		PNH is characterized by a decreased number of red blood cells (anemia), and the presence of blood in the urine (hemoglobinuria) and plasma (hemoglobinemia), which is evident after sleeping.
		PNH is associated with a high risk of major thrombotic events, most commonly thrombosis of large intra-abdominal veins.
		PNH blood cells are deficient in an enzyme known as PIG-A, which is required for the biosynthesis of cellular anchors.
	www.ncbi.nlm.nih.gov /disease/PNH.html (245 words)

	Paroxysmale nächtliche Hämoglobinurie: Klinik und durchflußzytometrische Analytik
		In summary the rare disease PNH or the GPI deficiency is caused by somatic mutation in the PIG-A gene.
		Correction of aplastic anaemia complicating paroxysmal nocturnal haemoglobinuria: absence of eradication of the PNH clone and dependence of response on cyclosporin A administration.
		Paroxysmal nocturnal hemoglobinuria (PNH) is caused by somatic mutation in the PIG-A-gene
	www.ma.uni-heidelberg.de /inst/ikc/pnhengl.htm (3038 words)

	The Sidney Kimmel Comprehensive Cancer Center At Johns Hopkins
		Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood.
		PNH affects only 1-2 persons per million of the population and is a disease of young adults (median age of diagnosis 35-40 years of age) with occasional cases diagnosed in childhood or adolescence.
		PNH is caused when mutations of the PIG-A gene occur in a bone marrow stem cell.
	www.hopkinskimmelcancercenter.org /CANCERTYPES/paroxysmal-nocturnal-hemoglobinuria.cfm?CANCERID=341 (1035 words)

	Paroxysmal Nocturnal Hemoglobinuria
		Paroxysmal Nocturnal Hemoglobinuria, or otherwise known simply as PNH, is the blood disease I have.
		Paroxysmal nocturnal hemoglobinuria is a rare form of hemolytic anemia that results when the body’s immune system destroys its own red blood cells.
		The red blood cells in PNH are very sensitive to being destroyed by the body’s complement system of proteins, which forms the basis for the diagnostic testing of PNH.
	members.aol.com /rebecca270/myhomepage/profile.html (587 words)

	Case 51: Paroxysmal Nocturnal Hemoglobinuria with Thrombotic Budd-Chiari Syndrome and Renal Cortical Hemosiderin -- ...
		nocturnal hemoglobinuria phenotype in 60% of the cells.
		MR of the kidneys, liver and spleen in paroxysmal nocturnal hemoglobinuria.
		MRI of the kidneys in paroxysmal nocturnal hemoglobinuria.
	radiology.rsnajnls.org /cgi/content/full/225/1/67 (1444 words)

	Hemoglobinuria, Paroxysmal Nocturnal
		It is possible that the main title of the report Hemoglobinuria, Paroxysmal Nocturnal is not the name you expected.
		Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem cell disorder.
		Researchers believe that two factors are necessary for the development of PNH: an acquired somatic mutation of the PIG-A gene, which affects hematopoietic stem cells; and a predisposition to the multiplication and expansion of these defective stem cells.
	www.webmd.com /a-to-z-guides/Hemoglobinuria-Paroxysmal-Nocturnal (642 words)

	In Vitro Proliferation and Differentiation of Megakaryocytic Progenitors in Patients with Aplastic Anemia, Paroxysmal ...
		In Vitro Proliferation and Differentiation of Megakaryocytic Progenitors in Patients with Aplastic Anemia, Paroxysmal Nocturnal Hemoglobinuria, and the Myelodysplastic Syndromes
		Increased sensitivity to complement or erythroid and myeloid progenitors in paroxysmal nocturnal hemoglobinuria.
		Increased sensitivity to complement of megakaryocyte progenitors in paroxysmal nocturnal haemoglobinuria.
	stemcells.alphamedpress.org /cgi/content/full/18/6/428 (2471 words)

	Clinical Trial: Eculizumab to Treat Paroxysmal Nocturnal Hemoglobinuria
		Patients 18 years of age and older with PNH who require blood transfusions for anemia and have received at least four transfusions in the 12 months preceding evaluation for this study may be eligible to enroll.
		Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder of the hematopoietic stem cell characterized by intravascular hemolysis, hemoglobinuria, anemia, and thrombosis.
		Since the pathogenesis of PNH is due to the inability of PNH red cells and platelets to inhibit the activation of terminal complement, it is logical to hypothesize that a terminal complement inhibitor could effectively stop the intravascular hemolysis, obviate or lessen the need for transfusions, and possibly decrease the propensity of life threatening thrombosis.
	clinicaltrials.gov /ct/show/NCT00098280?order=1 (1176 words)

	Mutation analysis of the PIG-A gene in Korean patients with paroxysmal nocturnal haemoglobinuria -- Yoon et al. 55 (6): ...
		Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal stem
		Identification and characterization of an inherited mutation of PIG-A in a patient with paroxysmal nocturnal hemoglobinuria.
		Paroxysmal nocturnal hemoglobinuria clones in patients with myelodysplastic syndromes.
	jcp.bmjjournals.com /cgi/content/full/55/6/410 (1827 words)

	Paroxysmal nocturnal haemoglobinuria: Nature's gene therapy? -- Johnson and Hillmen 55 (3): 145 -- Molecular Pathology
		Paroxysmal nocturnal haemoglobinuria is caused by somatic mutations in the PIG-A gene.
		Characterization of genomic PIG-A gene: a gene for glycosylphosphatidylinositol-anchor biosynthesis and paroxysmal nocturnal hemoglobinuria.
		Genetic instability and the etiology of somatic PIG-A mutations in paroxysmal nocturnal hemoglobinuria.
	mp.bmjjournals.com /cgi/content/full/55/3/145 (5383 words)

	Paroxysmal Nocturnal Hemoglobinuria - Associated Content
		PNH (paroxysmal nocturnal hemoglobinuria) is an extremely rare blood disease that affects between 8,000-10,000 individuals in Western Europe and North America.
		PNH was named after the dark urine individuals with PNH acquire.
		PNH is a rare blood disease that causes debilitating symptoms for individuals affected with the condition.
	www.associatedcontent.com /article/189485/paroxysmal_nocturnal_hemoglobinuria.html (549 words)

	Hemoglobinuria, Paroxysmal Nocturnal
		It is possible that the main title of the report Hemoglobinuria, Paroxysmal Nocturnal is not the name you expected.
		Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem cell disorder.
		The specific symptoms of PNH vary greatly and affected individuals usually do not exhibit all of the symptoms potentially associated with the disorder.
	my.webmd.com /hw/health_guide_atoz/nord670.asp (525 words)

	Aortic valve replacement and coronary revascularization in paroxysmal nocturnal hemoglobinuria -- Knobloch et al. 2 ...
		Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic
		Biology of GPI anchors and pathogenesis of paroxysmal nocturnal hemoglobinuria.
		Percutaneous transluminal coronary angioplasty in a patient with paroxysmal nocturnal hemoglobinuria.
	icvts.ctsnetjournals.org /cgi/content/full/2/4/647 (1311 words)

	Paroxysmal Nocturnal Hemoglobinuria
		Researchers believe that the early death of red blood cells is related to the abnormal development of certain molecules on the cell's surface.
		Paroxysmal nocturnal hemoglobinuria cause red or brown urine.
		In some cases of paroxysmal nocturnal hemoglobinuria, a bone marrow transplant may be considered.
	www.mayoclinic.org /anemia/pnhemoglobinuria.html (221 words)

	Paroxysmal Nocturnal Hemoglobinuria (PNH)
		Paroxysmal nocturnal hemoglobinuria (PNH) is a stem cell disorder in which the affected cells are deficient in GPI-anchored proteins.
		About 20-25% of patients with aplastic anemia and MDS have been found to demonstrate small clones of PNH cells, so studies for PNH may also be indicted in patients with these diagnoses.
		Paroxysmal Nocturnal Hemoglobinuria in Patients with Bone Marrow Failure Syndromes.
	www.medicine.uiowa.edu /Path_Handbook/handbook/test1123.html (424 words)

	News - Eculizumab Benefits Patients with Paroxysmal Nocturnal Haemoglobinuria (Site not responding. Last check: 2007-10-15)
		Eculizumab appears to reduce intravascular haemolysis, haemoglobinuria, as well as the need for transfusion in patients with paroxysmal nocturnal haemoglobinuria (PNH), and is associated with improved quality of life, new research suggests.
		PNH results from a somatic mutation of the PIG-A gene in a haematopoietic stem cell.
		Eleven transfusion-dependent patients with PNH were given infusions of eculizumab (600 mg) once a week for 4 weeks.
	www.docguide.com /news/content.nsf/news/8525697700573E1885256E3300351779 (530 words)

	Respective Roles of Decay-Accelerating Factor and CD59 in Circumventing Glomerular Injury in Acute Nephrotoxic Serum ...
		paroxysmal nocturnal hemoglobinuria (1), the absence of DAF
		Paroxysmal nocturnal hemoglobinuria: deficiency in factor H-like functions of the abnormal erythrocytes.
		Amelioration of the lytic abnormalities of paroxysmal nocturnal hemoglobinuria with decay-accelerating factor.
	www.jimmunol.org /cgi/content/full/172/4/2636 (4097 words)

	Impaired growth and elevated Fas receptor expression in PIGA+ stem cells in primary paroxysmal nocturnal hemoglobinuria ...
		Deficiency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria.
		Apoptosis resistance of blood cells from patients with paroxysmal nocturnal hemoglobinuria, aplastic anemia, and myelodysplastic syndrome.
		Araten, D.J., Nafa, K., Pakdeesuwan, K., and Luzzatto, L. Clonal populations of hematopoietic cells with paroxysmal nocturnal hemoglobinuria genotype and phenotype are present in normal individuals.
	www.jci.org /cgi/content/full/106/5/689 (4191 words)

	The PIG-A Mutation and Absence of Glycosylphosphatidylinositol-Linked Proteins Do Not Confer Resistance to Apoptosis in ...
		PAROXYSMAL NOCTURNAL hemoglobinuria (PNH) is an acquired clonal hematologic disorder that is characterized by a wide variety
		A total of 26 patients with PNH were studied, including 17 women and 9 men, with a median age of 33 years (range, 17 to 66).
		Lewis SM, Dacie JV: The aplastic anaemia-paroxysmal nocturnal haemoglobinuria syndrome.
	www.bloodjournal.org /cgi/content/full/92/7/2541 (5262 words)

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