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Transfusion hemosiderosis newborn

Ariel Koren, Lora Profeta, Luci Zalman, Haya Palmor, Carina Levin, Ronit Bril Zamir, Stavit Shalev, Orna Blondheim
BACKGROUND: β Thalassemia major is characterized by hemolytic anemia, ineffective erythropoiesis and hemosiderosis. About 4% of the world population carries a Thalassemia gene. Management includes blood transfusions and iron chelation. However, this treatment is costly, and population screening may be significantly more cost beneficial. PURPOSE: The purpose of the current study is to analyze the cost of running a prevention program for β Thalassemia in Israel and to compare it to the actual expenses incurred by treating Thalassemia patients...
2014: Mediterranean Journal of Hematology and Infectious Diseases
Elliott P Vichinsky
α-Thalassemia mutations affect up to 5% of the world's population. The clinical spectrum ranges from an asymptomatic condition to a fatal in utero disease. Hemoglobin H disease results from mutations of three α-globin genes. Deletional forms result in a relatively mild anemia, whereas nondeletional mutations result in a moderate to severe disease characterized by ineffective erythropoiesis, recurrent transfusions, and growth delay. Hemosiderosis develops secondary to increased iron absorption, as well as transfusion burden...
May 2013: Cold Spring Harbor Perspectives in Medicine
J R Elfenbein, S Giguère, S K Meyer, L H Javsicas, L L Farina, D N Zimmel, L C Sanchez
BACKGROUND: Hepatic failure is one of the more common complications in foals requiring blood transfusion to treat neonatal isoerythrolysis. Iron intoxication is likely the cause of hepatic injury. OBJECTIVES: To determine the effects of deferoxamine on iron elimination in normal foals. ANIMALS: Thirteen neonatal foals. METHODS: Randomized-controlled trial. At 1-3 days of age, foals received either 3 L of washed packed dam's red blood cells (RBC) or 3 L of saline IV once...
November 2010: Journal of Veterinary Internal Medicine
Elliott Vichinsky
Alpha thalassemia, the most common genetic disorder of hemoglobin synthesis, affects up to 5% of the world's population. It represents a group of conditions with reduced or absent synthesis of one to all four of alpha globin genes. Deletional or nondeletional mutations occur on chromosome 16. Its severity ranges from asymptomatic to fatal in utero. Hemoglobin H disease, a mutation of three alpha globin genes, is more severe than previously recognized. Anemia, hypersplenism, hemosiderosis, growth failure, and osteoporosis are commonly noted as the patient ages...
August 2010: Annals of the New York Academy of Sciences
Elliott P Vichinsky, Kwaku Ohene-Frempong
To gain an insight into current transfusion and chelation practice in patients with sickle cell disease (SCD), a survey of international experts has been conducted. The findings demonstrate that general utilization of transfusion therapy is low, the primary barrier to treatment being concerns over resultant iron overload and the subsequent need for iron chelation therapy. Where patients were transfused, many physicians indicated that a high proportion of patients had hemosiderosis. As evidence suggests more patients with SCD could benefit from regular transfusion therapy, it is apparent that greater awareness of the need to monitor and treat iron overload in transfused patients is required...
February 2011: Pediatric Hematology and Oncology
H Mugishima, S Ohga, A Ohara, S Kojima, K Fujisawa, I Tsukimoto et al.
Transfusion-dependent Diamond-Blackfan anemia (DBA) patients opt for allogeneic hematopoietic stem cell transplantation (HSCT) as curative therapy. Clinical outcomes of 19 transplanted Japanese patients were analyzed. Prior to HSCT, 10 patients (53%) suffered hemosiderosis with organ dysfunction, and all eight with short stature (42%) had adverse effects of prednisolone. Median age at the time of HSCT was 56 months. Transplantation sources were 13 bone marrow [six human leukocyte antigen (HLA)-matched siblings, and six HLA-matched and one HLA-mismatched unrelated donors], five cord blood (two HLA-matched siblings and three HLA-mismatched unrelated donors), and one peripheral blood from haploidentical mother...
September 2007: Pediatric Transplantation
Howard A Pearson
A 30-year-old woman with transfusion-dependent, homozygous beta-thalassemia major and transfusional hemosiderosis had 2 successful pregnancies after ovulation induction and in vitro fertilization. Treatment with subcutaneous desferrioxamine (DF) was discontinued before the conception but restarted at 6 months of gestation. Elective cesarean section was performed at 35 weeks of pregnancy because of partial placenta previa. The infant was clinically normal. At the time of delivery, the maternal serum ferritin was 2000 ng/mL, serum iron/iron binding capacity (SI/TIBC) were 274/380 microg/dL, and % saturation 72%...
March 2007: Journal of Pediatric Hematology/oncology
Shouichi Ohga, Hideo Mugishima, Akira Ohara, Seiji Kojima, Kohji Fujisawa, Keiko Yagi, Masamune Higashigawa, Ichiro Tsukimoto
The epidemiology and treatment outcomes for Diamond-Blackfan anemia (DBA) were surveyed in a cohort of 54 children (M/F = 26:28) registered in Japan from 1988 to 1998. The annual incidence was 4.02 cases per million births, the median age at diagnosis was 60 days, and 59% of the cases presented by 3 months of age. Three patients had a familial occurrence. All patients received prednisolone (PSL), and cyclosporin A (CsA) was added to the therapy in 17 patients. Forty-seven patients received transfusions, and 13 underwent hematopoietic stem cell transplantation (HSCT)...
January 2004: International Journal of Hematology
W Reed, E P Vichinsky
No abstract text is available yet for this article.
May 2001: Journal of Pediatric Hematology/oncology
V Fellman, L von Bonsdorff, J Parkkinen
OBJECTIVE: To investigate whether apotransferrin administration and exchange transfusion can improve outcome in patients with the recently described recessive congenital iron overload disease, presenting with intrauterine growth retardation, severe lactic acidosis, aminoaciduria, and hemosiderosis of the liver that so far has been treatment-resistant and lethal. METHODOLOGY: Because the patients have hypotransferrinemia, hyperferritinemia, increased transferrin saturation, and bleomycin detectable iron in plasma, we designed a treatment regime aiming at decreasing free iron and iron overload...
February 2000: Pediatrics
C Borgna-Pignatti, P Marradi, S Rugolotto, A Marcolongo
Bone marrow transplantation from an HLA-identical sibling can cure thalassaemia. The risk of chemotherapy-induced sterility, however, represents a deterrent for many patients already at risk of gonadal insufficiency and reduced fertility because of the effects of transfusional iron overload. We report here the first patient transplanted for thalassaemia, after ablative therapy with busulfan and cyclophosphamide, who, despite late pubertal maturation, became pregnant and delivered a full-term, normal infant.
July 1996: Bone Marrow Transplantation
A J Janov, T Leong, D G Nathan, E C Guinan
To define further the natural history of treated Diamond-Blackfan anemia (DBA), a congenital anemia characterized by a paucity of erythroid precursors, we analyzed 76 patients diagnosed or followed at Children's Hospital, Boston, between 1931 and 1992. Although DBA is generally defined as macrocytic, we found that mean corpuscular volume in infants aged <5 months rarely exceeded the normal range and is of little diagnostic value. Macrocytosis in patients aged > or = 5 months was more striking. Nine of 16 patients who never received steroids experienced remissions, sometimes after years of transfusions...
March 1996: Medicine (Baltimore)
K Washington, D L Gossage, M R Gottfried
Review of liver biopsy or autopsy material from 33 patients with severe combined immunodeficiency or combined immunodeficiency and four patients with DiGeorge syndrome revealed a wide range of hepatic pathology. The most common abnormality was graft-versus-host disease (16 patients), followed by viral infection (4 patients had adenovirus hepatitis, 3 had cytomegalovirus hepatitis). Centrilobular fibrosis with or without veno-occlusive disease was seen in five patients. Three patients had nonspecific hepatitis, four had changes attributed to total parenteral nutrition, and two had lymphoproliferative disorders involving the liver...
July 1993: Pediatric Pathology
F J Hildner, R W Monto, J W Rebuck
No abstract text is available yet for this article.
1968: Henry Ford Hospital Medical Journal
H Reddemann
No abstract text is available yet for this article.
1967: Zeitschrift Für Kinderheilkunde
R A al-Rashid
No abstract text is available yet for this article.
December 1971: Clinical Toxicology
H B Wong
The liver in an infant or child is as liable to the same pathologies afflicting the adult liver but with certain differences in prevalence and causes. Genetic disorders are more likely to present in the paediatric age group where many involve metabolic processes such as galactosemia, phenylketonuria, glycogen storage disease and others. Many of these present in the newborn period. However, neoplasms and hamartomas also present in the newborn period, such as congenital neuroblastoma with an enormously enlarged liver, hepatoblastoma and haemangioma...
April 1986: Annals of the Academy of Medicine, Singapore
H A Pearson, J S Lobel, S A Kocoshis, J L Naiman, J Windmiller, A T Lammi, R Hoffman, J C Marsh
In the past decade, we have studied four unrelated children with what we believe is a previously unreported disorder affecting the bone marrow and exocrine pancreas. During infancy these patients had the onset of severe, transfusion-dependent, macrocytic anemia plus a variable degree of neutropenia and thrombocytopenia. Their bone marrows had normal cellularity but were characterized by remarkable vacuolization of erythroid and myeloid precursors, hemosiderosis, and ringed sideroblasts. The vacuoles probably represented manifestations of cellular degeneration and death...
December 1979: Journal of Pediatrics
R T O'Brien
No abstract text is available yet for this article.
January 1977: Seminars in Hematology
E Silvestroni, I Bianco, B Graziani, C Carboni, S Costantini
No abstract text is available yet for this article.
November 25, 1978: Lancet
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