Intrauterine therapy for homozygous $alpha;-thalassemia

Carr, Stephen R.; Rubin, Lewis P.; Dixon, D.; Star, Jami; Dailey, Jacquelyn

doi:10.1016/0029-7844(94)00334-a

Cited by 74 publications

(41 citation statements)

References 15 publications

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“…Some centers continue to perform a combined approach incorporating both procedures so as to achieve a more stable fetal Hct and a more prolonged interval between transfusions. The main indication for IUT continues to be fetal anemia due to red cell alloimmunization, but successful use of this procedure has also been reported in pregnancies with parvovirus B19 infection, fetomaternal hemorrhage, placental chorioangiomas, and others [8,30,65,70,84] .…”

Section: Intrauterine Transfusionmentioning

confidence: 99%

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Therapeutic management of fetal anemia: review of standard practice and alternative treatment options

Παπαντωνίου

Sifakis

Antsaklis

2012

Journal of Perinatal Medicine

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Fetal anemia, mainly due to red cell alloimmunization, is still a signifi cant cause of fetal and neonatal mortality and morbidity. The focus of current clinical research has shifted from an invasive approach to non-invasive management and treatment of affected pregnancies, and the progress in this fi eld is associated with a major improvement in perinatal outcome. During the last 50 years, intrauterine red cells transfusion (IUT), fi rst via the intraperitoneal route and later directly to fetal circulation, is the standard practice in most centers, with survival rates that exceed 90 % , particularly if anemia is diagnosed early and treated in a timely manner. In addition, plasmapheresis and intravenous administration of highdose immunoglobulin have been implicated in the treatment of pregnancies complicated with early-onset severe red cell alloimmunization, alone or in combination with IUTs before the 20 th week of pregnancy, but there are still issues to be clarifi ed further. This review article aims to provide an overview of the current standard therapeutic management and alternative treatment modalities in pregnancies complicated by fetal anemia.

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Section: Intrauterine Transfusionmentioning

confidence: 99%

“…may also be implicated in HDFN. There are also non-immune etiologic factors of fetal anemia such as parvovirus B19 infection, fetomaternal hemorrhage, homozygous thalassemia, and placental chorioangioma [8,30,70,84] .…”

Section: Introductionmentioning

confidence: 99%

Therapeutic management of fetal anemia: review of standard practice and alternative treatment options

Παπαντωνίου

Sifakis

Antsaklis

2012

Journal of Perinatal Medicine

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“…Fetal Lee et al 2007;Vichinsky 2009Vichinsky , 2012. Intrauterine transfusion appears to minimize these complications (Carr et al 1995;Vichinsky 2009;Weisz et al 2009;Yi et al 2009). However, these newborns will require lifetime transfusion therapy or stem cell transplantation (Zhou et al 2001;Yi et al 2009;Vichinsky et al 2010).…”

Section: Prevention and Prenatal Managementmentioning

confidence: 99%

Clinical Manifestations of -Thalassemia

Vichinsky¹

2013

Cold Spring Harbor Perspectives in Medicine

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“…High incidences of congenital abnormalities, such as limb defects, hydrocephalus, microcephaly, atrial septal defects, pulmonary hypoplasia, hypospadias, and skeletal dysplasia, have also been reported [2]. Survival with Hb Bart's hydrops fetalis is possible following premature delivery and treatment with post-delivery transfusions [3] or intrauterine umbilical vein transfusions (IUT) [4]. A blood transfusion with iron chelator provides symptom relief, whereas allogeneic stem cell transplantation [5] is the only curative therapy for thalassemia.…”

Section: Introductionmentioning

confidence: 99%

Transfusion Dependent Homozygous α-Thalassemia in Patients Associated with Hypospadias in Three Survivors

Ahmad¹

2015

J Blood Disord Transfus

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Alpha-thalassemia results from a dysfunction of the α-globin gene. Types of mutations include large deletions and point mutations. The most severe form of α-thalassemia is hydrops fetalis, which is caused by homozygosity of certain types of either deletion or point mutations, and some cases were the results of a combination of both. Here, we describe three cases of homozygous α-thalassemia who continue to survive, all with hypospadias. The first two cases were 5-year-old twins that were diagnosed with homozygous SEA deletion and the first description of a 20-month-old child with the genotype of the homozygous Cd 59 (GGC>GAC) mutation of the HBA2 gene. Prognoses for any α-thalassemia mutation types that are known to lead to hydrops fetalis in male fetuses should be informed about the potential survival associated with hypospadias.

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Intrauterine therapy for homozygous $alpha;-thalassemia

Abstract: Antenatal diagnosis and therapy of homozygous alpha-thalassemia can prevent the prenatal consequences of hydrops and fetal death. New technologies such as stem cell transplantation may help to avert both prenatal and postnatal consequences.

Cited by 74 publications

References 15 publications

Therapeutic management of fetal anemia: review of standard practice and alternative treatment options

Therapeutic management of fetal anemia: review of standard practice and alternative treatment options

Clinical Manifestations of -Thalassemia

Transfusion Dependent Homozygous α-Thalassemia in Patients Associated with Hypospadias in Three Survivors

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