Articles with "heterotaxy syndrome" as a keyword



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Clinical implications of airway anomalies and stenosis in patients with heterotaxy syndrome

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Published in 2022 at "Pediatric Pulmonology"

DOI: 10.1002/ppul.25981

Abstract: In heterotaxy syndrome, bronchopulmonary situs usually reflects atrial situs, resulting in either right (RAI) or left atrial isomerism (LAI). This study determines airway anomalies and its implications in patients with heterotaxy. This retrospective study included… read more here.

Keywords: isomerism; patients heterotaxy; anomalies stenosis; airway anomalies ... See more keywords
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Cardiac Rhythm Disturbances in Heterotaxy Syndrome

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Published in 2019 at "Pediatric Cardiology"

DOI: 10.1007/s00246-019-02087-2

Abstract: ObjectiveHeterotaxy syndrome is associated with complex cardiac malformations and cardiac conduction system abnormalities. Those with right atrial isomerism (RAI) have dual sinus nodes and dual atrioventricular nodes predisposing them to supraventricular tachycardia (SVT). Those with… read more here.

Keywords: heterotaxy syndrome; rhythm disturbances; lai rai; cardiac rhythm ... See more keywords
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Unusual long survival in a case of heterotaxy and polysplenia

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Published in 2020 at "Surgical and Radiologic Anatomy"

DOI: 10.1007/s00276-020-02586-5

Abstract: Heterotaxy syndrome with polysplenia is an extremely rare congenital disorder caused by a disruption in the embryonic development that results in an abnormal arrangement of the abdominal and thoracic organs. We present the case of… read more here.

Keywords: heterotaxy syndrome; case; unusual long; case heterotaxy ... See more keywords

Intra-abdominal haemorrhaging after cardiac catheterisation: the importance of recognising vascular anomalies in heterotaxy syndrome.

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Published in 2023 at "Cardiology in the young"

DOI: 10.1017/s1047951122004279

Abstract: A 2-month-old boy with a single ventricle underwent cardiac catheterisation. Inferior vena cava angiography at the end of the examination revealed local stenosis, flexion, and connection to the right hepatic vein. Six hours after catheterisation,… read more here.

Keywords: catheterisation; heterotaxy syndrome; vascular anomalies; cardiac catheterisation ... See more keywords
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Incidental abdominal heterotaxy syndrome

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Published in 2019 at "ANZ Journal of Surgery"

DOI: 10.1111/ans.15406

Abstract: Heterotaxy syndrome is a sporadic genetic condition with a prevalence of 1:10 000. It is characterized by an abnormal arrangement of thoracic or abdominal viscera and can occur in isolation or as a feature of… read more here.

Keywords: heterotaxy syndrome; position; rotation; fig ... See more keywords

Checklist for Heterotaxy Syndrome Improves Compliance with Multidisciplinary Standard of Care

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Published in 2018 at "Pediatrics"

DOI: 10.1542/peds.142.1_meetingabstract.391

Abstract: Background Heterotaxy Syndrome (HS) is a disorder with abnormal arrangements of thoracic and abdominal organs. Manifestations include congenital heart defects, increased risks of bacteremia associated with variable splenic anatomy, and intestinal malrotation that may lead… read more here.

Keywords: heterotaxy syndrome; checklist heterotaxy; standard; standard care ... See more keywords
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Heterotaxy syndrome with agenesis of dorsal pancreas and diabetes mellitus: case report and review of the literature.

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Published in 2019 at "Archives of endocrinology and metabolism"

DOI: 10.20945/2359-3997000000142

Abstract: Heterotaxy syndrome (HS) is a rare congenital condition with multifactorial heritance, characterized by an abnormal arrangement of thoraco-abdominal organs and vessels. Patients present with multiple cardiac, gastrointestinal, hepatosplenic, pancreatic, renal, neurological and skeletal disorders without… read more here.

Keywords: heterotaxy syndrome; case; report; diabetes mellitus ... See more keywords