Precise spatiotemporal expression of the Nodal-Lefty-Pitx2 cascade in lateral plate mesoderm establishes left–right axis, which provides vital cues for correct organ formation and function. Mutations one constituent PITX2 and, separately, Forkhead transcription factor FOXC1 independently cause a multi-system disorder known as Axenfeld–Rieger syndrome (ARS). Since cardiac involvement is an estab...

BACKGROUND Situs inversus is a rare congenital anomaly characterized by the complete inversion of thoracic and abdominal organs. Liver transplantation in such patients or from donors in situs inversus is technically challenging because of the reversed anatomic structures. A small number of successful liver transplantation cases concerning situs inverus in either recipients or donors have been r...

Situs inversus viscerum is a rare condition in which the organs are transposed to the opposite side [1]. Endoscopic procedures can be difficult in patients with situs inversus because of the left–right reversal of the visceral organs. We present a patient with choledocholithiasis who had situs inversus. Endoscopic retrograde cholangiopancreatography (ERCP) was performed with minor modification ...

Several cardiac malformations are known to be associated with situs inversus (Ivemark, 1955; Campbell and Deuchar, 1965, 1966). In addition, there are often some venous and visceral malformations, e.g. bilateral superior venm cavae (SVC), absence of a segment of the inferior vena cava (IVC), agenesis of the spleen, and symmetry of the liver. We shall discuss these five malformations, particular...

In bilateria, positioning and looping of visceral organs requires proper left-right (L/R) asymmetry establishment. Recent work in Drosophila has identified a novel situs inversus gene encoding the unconventional type ID myosin (MyoID). In myoID mutant flies, the L/R axis is inverted, causing reversed looping of organs, such as the gut, spermiduct and genitalia. We have previously shown that Myo...

The specification of left-right asymmetry of the visceral organs is precisely regulated. The earliest breakage of left-right symmetry occurs as the result of leftward flow generated by asymmetric beating of nodal cilia, which eventually induces asymmetric Nodal/Lefty/Pitx2 expression on the left side of the lateral plate mesoderm. PIERCE1 has been identified as a p53 target gene involved in the...

Situs inversus totalis is a rare autosomal recessive congenital anomaly. There are very few reports in the published literature of abdominal aortic aneurysm in patient with situs inversus totalis, all of whom underwent open aneurysm repair. DeBakey type III acute aortic dissection is life threatening condition with organ malperfusion. A 74-year-old male, referred our department with back pain a...

dextrocardia is a cardiac positional anomaly in which the heart is located in the right hemithorax with its base-to-apex axis directed to the right and caudad. situs inversus is an autosomal recessive disorder that causes organs in the chest and abdomen to be positioned in a mirror image from their normal position. dextrocardia may occur in isolation or as part of situs inversus. similarly, sit...