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A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study

Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the Count...

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Autores principales: Mohor, Cosmin Ioan, Fleaca, Sorin Radu, Oprinca Muja, Alexandra, Oprinca, George Calin, Roman, Mihai Dan, Chicea, Radu, Boicean, Adrian Gheorghe, Dura, Horatiu, Tanasescu, Ciprian, Ion, Nicolas Catalin Ionut, Faur, Mihai, Bacila, Ciprian Ionut, Batar, Florina, Mohor, Calin Ilie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9144318/
https://www.ncbi.nlm.nih.gov/pubmed/35621846
http://dx.doi.org/10.3390/jcdd9050135
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author Mohor, Cosmin Ioan
Fleaca, Sorin Radu
Oprinca Muja, Alexandra
Oprinca, George Calin
Roman, Mihai Dan
Chicea, Radu
Boicean, Adrian Gheorghe
Dura, Horatiu
Tanasescu, Ciprian
Ion, Nicolas Catalin Ionut
Faur, Mihai
Bacila, Ciprian Ionut
Batar, Florina
Mohor, Calin Ilie
author_facet Mohor, Cosmin Ioan
Fleaca, Sorin Radu
Oprinca Muja, Alexandra
Oprinca, George Calin
Roman, Mihai Dan
Chicea, Radu
Boicean, Adrian Gheorghe
Dura, Horatiu
Tanasescu, Ciprian
Ion, Nicolas Catalin Ionut
Faur, Mihai
Bacila, Ciprian Ionut
Batar, Florina
Mohor, Calin Ilie
author_sort Mohor, Cosmin Ioan
collection PubMed
description Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the County Clinical Emergency Hospital of Sibiu, who was hospitalized from birth until death. The patient suffered cardio-respiratory arrest due to severe hypoxia and septicemia on the background of a series of complex cardiac malformations associated with congenital abdominal organ anomalies. Examination of the body revealed a common atrium with complete atrioventricular canal defect, left ventricular hypertrophy, right ventricle hypoplasia, truncus arteriosus, superior vena cava duplication, bilobation of the lungs, situs ambiguous of the abdominal organs with right-sided stomach, a midline liver, gall bladder agenesis, multiple right-sided spleens and complete inversion of the intestines and pancreas. Histopathology concluded that the patient suffered cardiac lesions consistent with infantile lactic acidosis, as well as pulmonary modifications suggesting congenital alveolar dysplasia and altered hepatic architecture compatible with fibrosis.
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spelling pubmed-91443182022-05-29 A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study Mohor, Cosmin Ioan Fleaca, Sorin Radu Oprinca Muja, Alexandra Oprinca, George Calin Roman, Mihai Dan Chicea, Radu Boicean, Adrian Gheorghe Dura, Horatiu Tanasescu, Ciprian Ion, Nicolas Catalin Ionut Faur, Mihai Bacila, Ciprian Ionut Batar, Florina Mohor, Calin Ilie J Cardiovasc Dev Dis Case Report Polysplenia syndrome represents a type of left atrial isomerism characterized by multiple small spleens, often associated with cardiac malformations and with situs ambiguus of the abdominal organs. The case presented is of a one-month-old male infant, weighing approximately 3000 g, born at the County Clinical Emergency Hospital of Sibiu, who was hospitalized from birth until death. The patient suffered cardio-respiratory arrest due to severe hypoxia and septicemia on the background of a series of complex cardiac malformations associated with congenital abdominal organ anomalies. Examination of the body revealed a common atrium with complete atrioventricular canal defect, left ventricular hypertrophy, right ventricle hypoplasia, truncus arteriosus, superior vena cava duplication, bilobation of the lungs, situs ambiguous of the abdominal organs with right-sided stomach, a midline liver, gall bladder agenesis, multiple right-sided spleens and complete inversion of the intestines and pancreas. Histopathology concluded that the patient suffered cardiac lesions consistent with infantile lactic acidosis, as well as pulmonary modifications suggesting congenital alveolar dysplasia and altered hepatic architecture compatible with fibrosis. MDPI 2022-04-27 /pmc/articles/PMC9144318/ /pubmed/35621846 http://dx.doi.org/10.3390/jcdd9050135 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Mohor, Cosmin Ioan
Fleaca, Sorin Radu
Oprinca Muja, Alexandra
Oprinca, George Calin
Roman, Mihai Dan
Chicea, Radu
Boicean, Adrian Gheorghe
Dura, Horatiu
Tanasescu, Ciprian
Ion, Nicolas Catalin Ionut
Faur, Mihai
Bacila, Ciprian Ionut
Batar, Florina
Mohor, Calin Ilie
A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study
title A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study
title_full A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study
title_fullStr A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study
title_full_unstemmed A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study
title_short A Rare Case of Polysplenia Syndrome Associated with Severe Cardiac Malformations and Congenital Alveolar Dysplasia in a One-Month-Old Infant: A Complete Macroscopic and Histopathologic Study
title_sort rare case of polysplenia syndrome associated with severe cardiac malformations and congenital alveolar dysplasia in a one-month-old infant: a complete macroscopic and histopathologic study
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9144318/
https://www.ncbi.nlm.nih.gov/pubmed/35621846
http://dx.doi.org/10.3390/jcdd9050135
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