Analysis Tools
growth/size/body
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• 85% exhibit atrial situs inversus, 7.5% with incomplete inversus, and 7.5% with left isomerism
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• all have right-sided stomach, however the angle between the cardia and the pylorus of the stomach varies
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• 20% exhibit abdominal heterotaxy, with an abdomen characterized by a symmetrical liver, hypoplastic spleen, small-lobed pancreas, and malrotation of the common gastrointestinal mesentery
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• 7.5% exhibit left atrial isomerism
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• 4 of 40 exhibit left isomerism of the lung, in which both sides of the lung are unilobed or pseudobilobed, and associated with hyparterial bronchi
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• 40% exhibit situs inversus of the lung and 50% show incomplete inversus
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• 25% show visceroatrial heterotaxy
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• each organ shows primary situs inversus with variable degrees of irregularity, ranging from almost complete form of situs inversus to left isomerism
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cardiovascular system
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• mutants sometimes exhibit posterior vena cava interruption
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• mutants exhibit cardiovascular anomalies that are seen in various combinations
• 10% exhibit tetralogy of Fallot; seen in 20% of mutants with abdominal heterotaxy and 25% of mutants with visceroatrial heterotaxy
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• mutants sometimes have an endocardial cushion defect
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• seen occasionally
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• seen occasionally
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• 85% exhibit atrial situs inversus, 7.5% with incomplete inversus, and 7.5% with left isomerism
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• 7.5% exhibit left atrial isomerism
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dextrocardia
(
J:48533
)
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• mutants sometimes display aortic stenosis
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digestive/alimentary system
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• all have right-sided stomach, however the angle between the cardia and the pylorus of the stomach varies
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• 42.5% show incompletely inverted looping of the intestine, 35% show malrotation, and 20% almost complete inversion of the intestinal loop
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hematopoietic system
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• 55% have normal sized spleen which is located along the great curvature of the right-sided stomach and 9% show a dysplastic spleen which is located bilaterally across the central line of the abdomen
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• 22% have a hypoplastic spleen
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liver/biliary system
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• 35% show situs inversus of the liver, 20% have primary situs inversus with incomplete lobation, and 45% have left isomerism of the liver, in which the hepatic portion of the posterior vena cava is absent
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respiratory system
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• 4 of 40 exhibit left isomerism of the lung, in which both sides of the lung are unilobed or pseudobilobed, and associated with hyparterial bronchi
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• 40% exhibit situs inversus of the lung and 50% show incomplete inversus
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endocrine/exocrine glands
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• 68% have a warped pancreas which runs along the duodenal looping, but is almost normal in size, and the rest have severe hypoplasia of the pancreas
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immune system
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• 55% have normal sized spleen which is located along the great curvature of the right-sided stomach and 9% show a dysplastic spleen which is located bilaterally across the central line of the abdomen
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• 22% have a hypoplastic spleen
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| tetralogy of Fallot | DOID:6419 |
OMIM:187500 |
J:48533 | |
