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PLATYPNEA-ORTHODEOXIA SYNDROME - A RARE CAUSE OF PERIPHERAL CYANOSIS IN CHILD /

PLATIPNEA-ORTODEOKSIJA SINDROM - REDAK UZROK PERIFERNE CIJANOZE DETETA

Authors

 

Dejana Bajić 1,2, Nikola Eić 1,2, Dragica Bulajić 2, Nemanja Todorović2, Zlatko Božić3

1Institute for Child and Youth Health Care of Vojvodina, Hajduk Veljkova 10, Novi Sad, Serbia
2Faculty of Medicine, University of Novi Sad, Hajduk Veljkova 3, Novi Sad, Serbia
3Primary Healthcare Center Bač, Bačka 2, Bač, Serbia

 

UDK: 616-056.7-053.2


The paper was received / Rad primljen: 23.02.2020.

Accepted / Rad prihvaćen: 02.03.2020.

 


Correspondence to:


Dr Dejana Bajić,
Institute for Child and Youth Health Care of Vojvodina
Hajduk Veljkova 10
21 000 Novi Sad, Serbia
+381 60 623 12 90
e-mail: dejnadeki@gmail.com

 

 

Sažetak

 

Uvod: Platipnea-ortodeoksija sindrom je redak klinički entitet koji se manifestuje arterijskom desaturacijom kada je pacijent u uspravnom položaju, a povlači se u horizontalnom. Prezentujemo Vam slučaj deteta uzrasta 3 meseca sa perifernom cijanozom u uspravnom položaju u čijoj osnovi je bio redak poremećaj-platipnea ortodeoksija sindrom. Prikaz slučaja: U kliničkoj slici pored mramorizovane kože, bila je uočljiva periferna cijanoza u uspravnom položaju i plavičaste sklere. Laboratorijske analize (izuzev povišenih laktata i D dimera), rendgensko snimak pluća i srca, ultrazvuk abdomena, CT grudnog koša i abdomena i elektroencefalogram su bili uredni. Ehokardiografskim nalazom detektovano je prisusutvo u interatrijalnom septumu secundum defekta i desno-levog šanta. Diskusija: Brojni su uzroci periferne cijanoze i svi moraju biti pažljivo razmotreni u okviru diferencijane dijagnoze. Najčešće je u osnovi  intrakardijalni šant, poremećaj odnosa ventilacija/perfuzija u plućnom parenhimu i plućni arterijsko-venski šant. Zaključak: Periferna cijanoza je deo kliničke slike različitih bolesti, ali njena manifestacija samo u uspravnom položaju diferencijalno dijagnostički može da uputi na redak entitet platiinea ortodeoksija sindrom.

 

 

Ključne reči:

ortodeoksija, periferna cijanoza, intrakardijalni šant, desno-levi šant

 

 

Abstract

 

Introduction: Platypnea-orthodeoxia syndrome is a rare clinical entity manifesting with arteriosus desaturation when a patient is in upright position, and retreats when a patient is lying. We are presenting a case of a 3-month old child with the symptoms of peripheral cyanosis in upright position caused by a rare platypnea -orthodeoxia syndrome. Case Report: In the clinical picture beside marmorized skin peripheral cyanosis was visible in upright position along with the bluish sclerae. Laboratory analyses (except for the increased lactates and D dimers), Chest X ray, Ultrasound of Abdomen, CT Thorax and Abdomen and electroencephalogram were normal. Echocardiographic finding showed the presence of a secundum defect in the interatrial septum and right-to -left shunt. Discussion:  The causes of peripheral cyanosis are numeral and all of them must be carefully inspected within a differential diagnosis. The most common causes are intracardiac shunt, pulmonary parenchymal ventilation /perfusion mismatch, and pulmonary arteriovenous shunts. Conclusion: Peripheral cyanosis is a part of a clinical picture of different diseases, but its manifestation only in upright position can indicate a rare entity of platypnea- orthodeoxia syndrome detected within a differential diagnosis.  

 

 

Key words:

orthodeoxia, peripheral cyanosis, intracardiac shunt, right to left shunt

 

 

References:

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PDF Bajić D. et al • MD-Medical Data 2020;12(1): 033-036

 

 

 

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