Observations on some additional abnormalities in situs inversus viscerum.
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The abnormal findings in a case of Situs inversus totalis are described. The duodenum was placed abnormally and retained its primitive mesentery. The proximal 22 in of jejunum were retroperitoneal. The attachment of the root of the mesentery to the posterior abdominal wall had a 7-shaped appearance, and there was a partial failure of the primitive mesocolon to adhere to the posterior abdominal wall. The common hepatic artery arose from the superior meseneric artery, which also provided a branch to the proximal jejunal loop. The right vagus nerve was found anterior to the oesophagus at the oesophageal hiatus in the diaphragm, and the left vagus was posterior. A double ureter was present on the right side. The findings are discussed in relation to mid-gut development. (+info)
Liver transplantation in patients with situs inversus.
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Two patients with situs inversus and biliary atresia were treated with hepatic transplantation, one with an auxiliary liver and the other with an orthotopic graft which was placed using a piggy-back technique. Both transplants functioned well initially. The auxiliary liver was rejected after 1 1/2 months, and the patient died after an attempt at retransplantation many months later. The recipient of the orthotopic liver has perfect liver function 10 months postoperatively. (+info)
Asymmetry of cilia and of mice and men.
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Evidence is given for the opinion that cilia in the early embryo, by their work, determine the laterality of the body; without ciliary work body laterality would be randomized. More exactly, monocilia in the primitive node are responsible for this determination. They have been described as being of the 9+0 type, but with dynein arms and with a gyrating movement. The orientation of the monocilia on the epithelium is of no importance but the direction of their gyration is, as may also be the shape of the node. The chirality of the cilia is thus reflected directly in the asymmetry of the body. The dynein arms go clockwise as seen from the base to tip and the ciliary rotation is in the same direction. The resulting waterflow is towards the left and so is the movement of the forming heart. In most subgroups of the immotile-cilia syndrome this mechanism does not work and equally many individuals will be born with situs inversus as with situs solitus. An exception is the immotile-cilia subgroup, named 'microtubule transposition', which is characterized by all cilia having a 9+0 structure throughout most of their length. (+info)
Abnormal nodal flow precedes situs inversus in iv and inv mice.
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We examined the nodal flow of well-characterized mouse mutants, inversus viscerum (iv) and inversion of embryonic turning (inv), and found that their laterality defects are always accompanied by an abnormality in nodal flow. In a randomized laterality mutant, iv, the nodal cilia were immotile and the nodal flow was absent. In a situs inversus mutant, inv, the nodal cilia was motile but could only produce very weak leftward nodal flow. These results consistently support our hypothesis that the nodal flow produces the gradient of putative morphogen and triggers the first L-R determination event. (+info)
Situs inversus with cholelithiasis.
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Situs inversus totalis is a form of heterotaxia which is usually detected accidentally while investigating for any associated condition. If undetected, this condition can create a diagnostic puzzle. We report one such case in which situs inversus was associated with cholelithiasis. (+info)
A locus for primary ciliary dyskinesia maps to chromosome 19q.
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Primary ciliary dyskinesia is an autosomal recessive condition characterised by chronic sinusitis, bronchiectasis, and subfertility. Situs inversus occurs in 50% of cases (Kartagener syndrome). It has an estimated incidence of 1 in 20 000 live births. The clinical phenotype is caused by defective ciliary function associated with a range of ultrastructural abnormalities including absent dynein arms, absent radial spokes, and disturbed ciliary orientation. The molecular genetic basis is unknown. A genome scan was performed in five Arabic families. Using GENEHUNTER, a maximal multipoint lod score (HLOD) of 4.4 was obtained on chromosome 19q13.3-qter at alpha (proportion of linked families) = 0.7. A 15 cM critical region is defined by recombinations at D19S572 and D19S218. These data provide significant evidence for a PCD locus on chromosome 19q and confirm locus heterogeneity. (+info)
Neonatal arterial switch operation for transposition of the great arteries in a patient with mirror image dextrocardia and situs inversus totalis.
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The neonatal arterial switch operation has become the standard therapy for D-transposition of the great arteries in the absence of left ventricular outflow tract obstruction. We describe our experience of successful arterial switch operation after balloon atrial septostomy in a 5-day-old infant girl who had atrial and visceral situs inversus totalis, mirror image dextrocardia, and D-transposition of the great arteries. To our knowledge, ours is the first report of this operation in a patient with this anatomy. (+info)
Laparoscopic cholecystectomy and appendectomy in situs inversus totalis.
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Situs inversus totalis is an uncommon anatomic anomaly that complicates diagnosis and management of acute abdominal pain. Expedient diagnosis of common intraperitoneal disease processes such as biliary colic, acute appendicitis and diverticulitis is often delayed as a result of seemingly incongruous physical findings. We present the case of a young woman with prior emergency room visits for complaints of a vague left upper quadrant abdominal pain. An ultrasound performed on her third presentation revealed visceral situs inversus with cholelithiasis and dilated intra- and extrahepatic biliary ducts. Standard laparoscopic cholecystectomy and cholangiography with a mirror-image surgical approach was performed successfully and without complication. (+info)