Patau and Edwards Syndromes in a University Hospital: beyond palliative care
ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes...
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| Format: | Article |
| Language: | English |
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Sociedade de Pediatria de São Paulo
2023-12-01
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| Series: | Revista Paulista de Pediatria |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0103-05822024000100437&lng=en&tlng=en |
| _version_ | 1827587364125409280 |
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| author | Ligia Marçola Ivete Zoboli Rita Tiziana Verardo Polastrini Silvia Maria de Macedo Barbosa Mário Cícero Falcão Paula de Vicenzi Gaiolla |
| author_facet | Ligia Marçola Ivete Zoboli Rita Tiziana Verardo Polastrini Silvia Maria de Macedo Barbosa Mário Cícero Falcão Paula de Vicenzi Gaiolla |
| author_sort | Ligia Marçola |
| collection | DOAJ |
| description | ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30–36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2–304) and 25 days (13–58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families. |
| first_indexed | 2024-03-09T00:15:55Z |
| format | Article |
| id | doaj.art-376f9dc3dbc84739861dc63ecb71b284 |
| institution | Directory Open Access Journal |
| issn | 1984-0462 |
| language | English |
| last_indexed | 2024-03-09T00:15:55Z |
| publishDate | 2023-12-01 |
| publisher | Sociedade de Pediatria de São Paulo |
| record_format | Article |
| series | Revista Paulista de Pediatria |
| spelling | doaj.art-376f9dc3dbc84739861dc63ecb71b2842023-12-12T07:52:51ZengSociedade de Pediatria de São PauloRevista Paulista de Pediatria1984-04622023-12-014210.1590/1984-0462/2024/42/2023053Patau and Edwards Syndromes in a University Hospital: beyond palliative careLigia Marçolahttps://orcid.org/0000-0001-8854-937XIvete Zobolihttps://orcid.org/0000-0001-8297-8229Rita Tiziana Verardo Polastrinihttps://orcid.org/0000-0002-1862-3084Silvia Maria de Macedo Barbosahttps://orcid.org/0000-0003-2082-9521Mário Cícero Falcãohttps://orcid.org/0000-0002-5658-3992Paula de Vicenzi Gaiollahttps://orcid.org/0000-0003-4367-1157ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30–36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2–304) and 25 days (13–58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0103-05822024000100437&lng=en&tlng=enTrisomy 13 syndromeTrisomy 18 syndromeHeart defects, congenitalPalliative care |
| spellingShingle | Ligia Marçola Ivete Zoboli Rita Tiziana Verardo Polastrini Silvia Maria de Macedo Barbosa Mário Cícero Falcão Paula de Vicenzi Gaiolla Patau and Edwards Syndromes in a University Hospital: beyond palliative care Revista Paulista de Pediatria Trisomy 13 syndrome Trisomy 18 syndrome Heart defects, congenital Palliative care |
| title | Patau and Edwards Syndromes in a University Hospital: beyond palliative care |
| title_full | Patau and Edwards Syndromes in a University Hospital: beyond palliative care |
| title_fullStr | Patau and Edwards Syndromes in a University Hospital: beyond palliative care |
| title_full_unstemmed | Patau and Edwards Syndromes in a University Hospital: beyond palliative care |
| title_short | Patau and Edwards Syndromes in a University Hospital: beyond palliative care |
| title_sort | patau and edwards syndromes in a university hospital beyond palliative care |
| topic | Trisomy 13 syndrome Trisomy 18 syndrome Heart defects, congenital Palliative care |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0103-05822024000100437&lng=en&tlng=en |
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