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Congenital Hemolytic Anemia (Hemolytic Anemia, Congenital)

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Hemolytic anemia due to various intrinsic defects of the erythrocyte.

Also Known As:

Hemolytic Anemia, Congenital; Hemolytic Anemia, Hereditary; Anemia, Hemolytic, Congenital; Hereditary Hemolytic Anemia; Anemia, Congenital Hemolytic; Anemia, Hereditary Hemolytic; Anemias, Congenital Hemolytic; Anemias, Hereditary Hemolytic; Congenital Hemolytic Anemias; Hemolytic Anemias, Congenital; Hemolytic Anemias, Hereditary; Hereditary Hemolytic Anemias; Anemia, Hemolytic, Hereditary

Networked: 218 relevant articles (4 outcomes, 13 trials/studies)

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Disease Context: Research Results

Related Diseases

1.	Hyperbilirubinemia
2.	Thalassemia
3.	Hereditary Spherocytosis
4.	Hemolytic Anemia
5.	beta-Thalassemia (Cooley's Anemia)

Experts

1.	Rice, Henry E: 6 articles (03/2015 - 02/2003)
2.	Oldham, Keith T: 3 articles (03/2015 - 02/2003)
3.	Ware, Russell E: 3 articles (12/2010 - 02/2003)
4.	Crary, Shelley E: 2 articles (03/2015 - 04/2012)
5.	Langer, Jacob C: 2 articles (03/2015 - 04/2012)
6.	Fucharoen, Suthat: 2 articles (04/2009 - 01/2004)
7.	Brugnara, Carlo: 1 article (10/2015)
8.	Alper, Seth L: 1 article (10/2015)
9.	Shmukler, Boris E: 1 article (10/2015)
10.	Gambale, Antonella: 1 article (10/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Congenital Hemolytic Anemia:

1.	Glucose (Dextrose)FDA LinkGeneric 09/15/1996 - "Molecular analysis of glucose phosphate isomerase deficiency associated with hereditary hemolytic anemia." 04/30/1963 - "[Utilization of glucose in erythrocytes of human subjects affected by non-spherocytic congenital hemolytic anemia]." 10/01/1979 - "End stage failure in a patient with congenital hemolytic anemia attributable to glucose phosphate isomerase deficiency was treated successfully with maintenance hemodialysis and renal transplantation. " 10/01/1980 - "Successful long-term phenobarbital therapy of hyperbilirubinemia in congenital hemolytic anemia due to glucose phosphate isomerase deficiency." 07/01/1985 - "Since the discovery of glucose 6-phosphate dehydrogenase (G6PD) and of pyruvate kinase deficiencies, erythroenzymopathies associated with hereditary hemolytic anemia have been extensively investigated. "
2.	Phenobarbital (Luminal)FDA Link 10/01/1980 - "Phenobarbital treatment is recommended in patients with congenital hemolytic anemia in whom hyperbilirubinemia is not improved by splenectomy or in whom splenectomy is not indicated. " 10/01/1980 - "Successful long-term phenobarbital therapy of hyperbilirubinemia in congenital hemolytic anemia due to glucose phosphate isomerase deficiency."
3.	Pyruvate KinaseIBA 03/01/1981 - "[Thirteen cases of erythrocyte pyruvate kinase deficiency associated with hereditary hemolytic anemia--clinical and biochemical studies (author's transl)]." 01/01/1980 - "Clinical, familial and biochemical studies from six unrelated Spanish patients with hereditary hemolytic anemia and erythrocyte pyruvate-kinase (PK) deficiency are described. " 10/01/1975 - "The scanning electron microscope was used to study the surface morphologic features of erythrocytes from a Basenji dog with hereditary hemolytic anemia due to a pyruvate kinase-deficient erythrocytes (RBC). " 05/15/1997 - "We studied the PK-LR gene in 15 unrelated Italian patients with congenital hemolytic anemia associated with erythrocyte pyruvate kinase (PK) deficiency. " 03/01/1997 - "Molecular analysis of 29 pyruvate kinase-deficient patients from central Europe with hereditary hemolytic anemia."
4.	5'-Nucleotidase (5' Nucleotidase)IBA 05/30/1983 - "Three families with hereditary hemolytic anemia and pyrimidine 5'-nucleotidase deficiency: electrophoretic and kinetic studies." 08/30/1999 - "[Pyrimidine-5'-nucleotidase deficiency--congenital hemolytic anemia with basophilic stippling of erythrocytes]." 02/01/1981 - "Two cases of hereditary hemolytic anemia with pyrimidine 5'-nucleotidase deficiency." 12/08/1980 - "A new variant of hereditary hemolytic anemia due to erythrocyte pyrimidine 5'-nucleotidase deficiency was found in Japan. " 07/26/1977 - "Three cases of hereditary hemolytic anemia with pyrimidine 5'-nucleotidase deficiency in a Japanese family."
5.	IronIBA 05/01/1967 - "Radioactive iron (59Fe) excretion through the urine and feces after intravenous iron-59 administration in congenital hemolytic anemias." 11/01/2007 - "The method of cardiovascular T2 magnetic resonance imaging (MRI) allows in vivo estimation of iron in the heart and liver and was used to measure the degree of iron overload in 10 transfused MDS patients (average 90 blood units) and in 3 patients with congenital hemolytic anemia. " 01/01/1998 - "The hemochromatosis alleles predictably contribute to an iron burden from other causes, commonly facilitate the expression of porphyria cutanea tarda, and their clinical expression may be accelerated by hereditary hemolytic anemias. " 03/01/1964 - "[STATE OF LOW IRON LEVEL IN CASES OF CONGENITAL HEMOLYTIC ANEMIA, SUCH AS SICKLE CELL ANEMIA AND THALASSEMIA]." 01/01/1975 - "An only mild hyperplasia of the erythropoietic system in the bone marrow does also not effect iron absorption which was still under the control of available storage iron in patients with hereditary spherocytosis, nonspherocytic congenital hemolytic anemia due to glucose-6-phosphate dehydrogenase deficiency, acquired hemolytic anemia and vitamin B12 deficiency induced megaloblastic anemia.."
6.	Proteins (Proteins, Gene)IBA 07/01/1989 - "Besides the analysis of erythrocyte membrane proteins the classical methods used in the study of congenital hemolytic anemias cannot be missed. " 07/01/2011 - "Defects or deficiencies in red cell membrane skeletal proteins often undermine the integrity and stability of the plasma membrane, and consequently cause hereditary hemolytic anemias. " 11/01/2010 - "Deficiencies of EMS proteins in humans lead to the hemolytic anemia Hereditary Spherocytosis (HS) which includes a subpopulation with no known genetic defect. " 07/01/1989 - "Recently numerous defects of erythrocyte membrane proteins have been described in hereditary hemolytic anemias. " 07/01/1989 - "The hereditary hemolytic anemias include a heterogeneous class of disorders caused by defects in the proteins that constitute the membrane skeleton of the red blood cell. "
7.	CalciumIBA 11/01/1984 - "In this study, we used an ion-selective membrane electrode to measure ionized calcium in hepatic bile of control +/+ mice and nb/nb mice with hereditary hemolytic anemia. " 12/20/1975 - "In order to elucidate the role of the Ca2+ accumulation in hemolytic process, erythrocytic calcium concentration has been studied by atomic absorption spectroscopy in 18 cases of congenital hemolytic anemias and in 19 cases of acquired auto-immune hemolysis. "
8.	ChromiumIBA 01/01/1962 - "[Radioactive iron and chromium study of erythropoiesis and destruction of red corpuscles in congenital hemolytic anemias]."
9.	Adenosine Monophosphate (AMP)IBA 04/01/1986 - "Uncertainties regarding the role of pyrimidine nucleotidase (PyrNase) in AMP catabolism were resolved by studies of erythrocytes from normal controls, controls with young mean cell ages, and patients with hereditary hemolytic anemia due to severe deficiency of PyrNase. "
10.	hepcidinIBA 01/01/2012 - "Study of serum hepcidin in hereditary hemolytic anemias."

Therapies and Procedures

1.	Splenectomy 04/01/2008 - "Although preliminary data demonstrate successful laboratory and clinical outcomes after partial splenectomy in various congenital hemolytic anemias, conclusive data comparing the efficacy of partial splenectomy to total splenectomy are not reported. " 04/01/2012 - "There is a need for randomized clinical trials and improved data collection of different types of splenectomy in congenital hemolytic anemias. " 03/01/2015 - "In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. " 03/01/2015 - "The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. " 03/01/2015 - "Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry."
2.	Kidney Transplantation 10/01/1979 - "End stage failure in a patient with congenital hemolytic anemia attributable to glucose phosphate isomerase deficiency was treated successfully with maintenance hemodialysis and renal transplantation. "
3.	Renal Dialysis (Hemodialysis) 10/01/1979 - "End stage failure in a patient with congenital hemolytic anemia attributable to glucose phosphate isomerase deficiency was treated successfully with maintenance hemodialysis and renal transplantation. "
4.	Transplantation (Transplant Recipients) 06/01/1990 - "Transplantation studies in mice with congenital hemolytic anemia." 11/01/2003 - "Adoptive immunotherapy to increase the level of donor hematopoietic chimerism after nonmyeloablative marrow transplantation for severe canine hereditary hemolytic anemia." 01/01/2001 - "Severe canine hereditary hemolytic anemia treated by nonmyeloablative marrow transplantation." 01/01/1981 - "Long-term survival and reversal of iron overload after marrow transplantation in dogs with congenital hemolytic anemia."
5.	Electrodes (Electrode) 11/01/1984 - "In this study, we used an ion-selective membrane electrode to measure ionized calcium in hepatic bile of control +/+ mice and nb/nb mice with hereditary hemolytic anemia. "