Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation
Elevated Tricuspid Regurgitant Velocity (TRV) has been related to higher mortality in adults and to hemolysis, lower oxygen saturation during 6-minute walk test and acute chest syndrome (ACS) in children with sickle cell disease (SCD). Hydroxyurea (HU) has reduced TRV value in children and adults. W...
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| Format: | Article |
| Language: | English |
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MDPI AG
2011-09-01
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| Series: | Hematology Reports |
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| Online Access: | http://www.pagepress.org/journals/index.php/hr/article/view/2625 |
| _version_ | 1797717641480110080 |
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| author | Raffaella Colombatti Elena Varotto Silvia Ricato Daniel Nardo Nicola Maschietto Simone Teso Marta Pillon Chiara Messina Ornella Milanesi Laura Sainati |
| author_facet | Raffaella Colombatti Elena Varotto Silvia Ricato Daniel Nardo Nicola Maschietto Simone Teso Marta Pillon Chiara Messina Ornella Milanesi Laura Sainati |
| author_sort | Raffaella Colombatti |
| collection | DOAJ |
| description | Elevated Tricuspid Regurgitant Velocity (TRV) has been related to higher mortality in adults and to hemolysis, lower oxygen saturation during 6-minute walk test and acute chest syndrome (ACS) in children with sickle cell disease (SCD). Hydroxyurea (HU) has reduced TRV value in children and adults. We describe a three year old HbSS child with recurrent ACS, hypoperfusion of the left lung, mild hemolysis and persistent TRV elevation. TRV did not normalize after HU, despite improvement in clinical conditions and in baseline laboratory parameters related to hemolysis and blood viscosity, but normalized after bone marrow transplantation (BMT). Our experience suggests that in young patients, TRV reduction can be a positive concomitant effect of BMT. |
| first_indexed | 2024-03-12T08:39:18Z |
| format | Article |
| id | doaj.art-e271c18b6c6b40d784251f79a2c6bc07 |
| institution | Directory Open Access Journal |
| issn | 2038-8322 2038-8330 |
| language | English |
| last_indexed | 2024-03-12T08:39:18Z |
| publishDate | 2011-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Hematology Reports |
| spelling | doaj.art-e271c18b6c6b40d784251f79a2c6bc072023-09-02T17:03:38ZengMDPI AGHematology Reports2038-83222038-83302011-09-0132e12e1210.4081/hr.2011.e121619Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantationRaffaella Colombatti0Elena Varotto1Silvia Ricato2Daniel Nardo3Nicola Maschietto4Simone Teso5Marta Pillon6Chiara Messina7Ornella Milanesi8Laura Sainati9Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaCardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaCardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaClinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaCardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaUniversity of Padova, PadovaBone Marrow Transplant Unit, Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaBone Marrow Transplant Unit, Clinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaCardiology Unit, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaClinic of Pediatric Hematology Oncology, Department of Pediatrics, Azienda Ospedaliera-University of Padova, PadovaElevated Tricuspid Regurgitant Velocity (TRV) has been related to higher mortality in adults and to hemolysis, lower oxygen saturation during 6-minute walk test and acute chest syndrome (ACS) in children with sickle cell disease (SCD). Hydroxyurea (HU) has reduced TRV value in children and adults. We describe a three year old HbSS child with recurrent ACS, hypoperfusion of the left lung, mild hemolysis and persistent TRV elevation. TRV did not normalize after HU, despite improvement in clinical conditions and in baseline laboratory parameters related to hemolysis and blood viscosity, but normalized after bone marrow transplantation (BMT). Our experience suggests that in young patients, TRV reduction can be a positive concomitant effect of BMT.http://www.pagepress.org/journals/index.php/hr/article/view/2625sickle-cell disease, pulmonary hypertension, tricuspid regurgitant jet velocity, hydroxyurea, bone marrow transplantation. |
| spellingShingle | Raffaella Colombatti Elena Varotto Silvia Ricato Daniel Nardo Nicola Maschietto Simone Teso Marta Pillon Chiara Messina Ornella Milanesi Laura Sainati Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation Hematology Reports sickle-cell disease, pulmonary hypertension, tricuspid regurgitant jet velocity, hydroxyurea, bone marrow transplantation. |
| title | Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation |
| title_full | Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation |
| title_fullStr | Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation |
| title_full_unstemmed | Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation |
| title_short | Tricuspid regurgitant velocity elevation in a three-year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation |
| title_sort | tricuspid regurgitant velocity elevation in a three year old child with sickle cell anemia and recurrent acute chest syndromes reversed not by hydroxyurea but by bone marrow transplantation |
| topic | sickle-cell disease, pulmonary hypertension, tricuspid regurgitant jet velocity, hydroxyurea, bone marrow transplantation. |
| url | http://www.pagepress.org/journals/index.php/hr/article/view/2625 |
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