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At least 217 records · Page 12Linked to original sources

Nephrotic syndrome and situs inversus viscerum: correlation or coincidence.

Asymmetric human internal organ organization has been a research topic for many years. Currently, although we know many mechanisms leading to the creation of specific internal organ organization, the problem requires further investigation. Transforming Growth Factor-beta superfamily (TGF-beta SF) molecules seem to play a major role in the process. They affect the cell by signaling through type I and II serine/threonine kinase receptors. Smad proteins seem vital in transmitting the signal between the receptor and the nucleus. We present the case of an 18-year-old female patient, who was admitted to the hospital because of steroid-sensitive nephrotic syndrome. Physical examination revealed that the patient also had a complete reversal of the intra-abdominal anatomy - situs inversus totalis. We suggest that disturbed TGF-beta and Smad signaling could be responsible for both nephrotic syndrome and situs inversus totalis.

Adolescent↗

Probable autosomal recessive inheritance of polysplenia, situs inversus and cardiac defects in an Amish family.

We report on an Amish family with five individuals in two generations with complex congenital heart disease. Autopsy findings in one and clinical examination in the others support the diagnosis of polysplenia "syndrome." In a mouse model, this spectrum of situs abnormalities and cardiovascular defects shows recessive inheritance with homozygotes having either situs solitus or situs inversus or ambiguous situs. The parents of the four affected sibs are fourth cousins. We think that the father of these four children is an affected but clinically normal homozygote, that his deceased sister was an affected homozygote, and it seems likely that they too had consanguinous parents.

Abnormalities, Multiple↗

Laparoscopic cholecystectomy in situs inversus totalis: is it safe?

A 46-year-old woman with known situs inversus totalis and recent mitral valve replacement presented with nausea and vomiting. Ultrasonography demonstrated a left-sided microlithiasic gall-bladder. Laparoscopic cholecystectomy was performed successfully. The postoperative recovery was uneventful. The literature on this subject is reviewed.

Cholecystectomy, Laparoscopic↗

Coronary angiography in a patient with mirror-image heart ("situs inversus").

A right-sided heart associated with mirror-image atrial arrangement (situs inversus) is a rare condition. The incidence of coronary arterial disease in this setting is probably similar to that in the general population. Description of coronary angiography in this condition is very rare. We have recently catheterized such a patient. The necessary approach to coronary angiography is emphasized.

Angina Pectoris↗

Situs inversus totalis with embryonal cell carcinoma of ovaries.

A very rare association between situs inversus totalis and embryonal cell carcinoma of the ovary is presented in a 20-year-old Saudi female. Recurrence of the tumor in the form of metastases to the left lobe of the liver was successfully treated using a combination of bleomycin, vinblastine++ and cis-platinum. Second-look laparotomy has confirmed the complete remission.

Adult↗

A suggested technique for "orthotopic" heart transplantation in a patient with situs inversus.

We describe a technique for "orthotopic" heart transplantation in a patient with situs inversus. The left atrial, aortic, and pulmonary artery anastomoses were performed directly, in the usual manner. The recipient's right atrium was converted into a tunnel, and the donor's right atrium was left intact. Anastomoses were therefore required between the 2 inferior venae cavae (by direct end-to-end anastomosis) and the 2 superior venae cavae (necessitating the insertion of a Dacron-graft). We suggest that even simpler techniques, perhaps not requiring the use of an artificial vascular prosthesis, are possible.

Adult↗

[Isolated levocardia and corrected transposition of the great arteries in total situs inversus].

Isolated levocardia combined with corrected transposition of the great arteries and complete situs inversus is rare. Due to discordant interrelationships between atria and ventricles and between ventricles and great arteries, both anomalies result in a normal circulation. In the presented case no associated cardiac malformations were present and the 55-year-old woman remained symptom-free for a long time until she developed an acquired valve disease. A systematic approach to chamber localisation in complex morphologic lesions of the heart is reviewed, and the value of CT, which can be applied successfully in older children and adults is demonstrated.

Cardiac Catheterization↗

Beating heart surgery in a patient with dextrocardia and complete situs inversus.

We present a 65-year-old female patient with dextrocardia and situs inversus who underwent successful coronary artery bypass without cardiopulmonary bypass. Vessels revascularized included right internal mammary artery to the left anterior descending artery and a saphenous vein graft to the first obtuse marginal branch. The procedure was performed on a beating heart through a median sternotomy with the use of a compression epicardial stabilizer. The patient was discharged to her home after an uneventful recovery. Only 12 similar cases of myocardial revascularization in patients with dextrocardia have been reported so far, and this is one of the first procedures, in patients with dextrocardia, performed off pump.

Abnormalities, Multiple↗

[Successful surgical treatment of a case of corrected transposition in situs inversus with associated anomalies].

A 33-year-old female with corrected transposition (situs inversus) with ventricular septal defect (VSD), atrial septal defect (ASD), and pulmonary stenosis (valvular and subvalvular) was operated successfully. Closure of ASD and infracristal VSD was performed through right atrial approach. In order to get complete exposure of VSD, a part of anterior leaflet of mitral valve was detached temporally. An external conduit was interposed between the morphologic left ventricle and the main pulmonary artery to relieve pulmonary stenosis. Postoperative course was uneventful except a transient complete heart block for a day. Several technical aspects of the radical operation were discussed including VSD closure method to avoid damaging the atrioventricular conducting tissues and reconstructive procedure of morphological left ventricular outflow.

Abnormalities, Multiple↗

Esophageal atresia and situs inversus--an unusual association of abnormalities--a case report.

We present a case report of a neonate with esophageal atresia and tracheoesophageal fistula. In the 31st week of gestation, maternal polyhydramnions was observed by prenatal ultrasonography. Postnatal insertion of an orogastric tube into the stomach was unsuccessful. On auscultation, the apex of the heart was heard at the right side of the thorax, while the liver was palpable 1 cm below the left rib cage. Esophageal atresia with tracheoesphageal fistula and situs inversus of the thoracic and abdominal organs was diagnosed During the first day of life, left transpleural thoracotomy was performed. The fistula was closed and esophageal anastomosis performed. One year after the operation the child had no difficulties when eating solid and liquid foods. More than 50% of infants with esophageal atresia have associated anomalies and the esophagogram showed good passage of contrast with anastomotic stricture. This was the first report of esophageal atresia with tracheoesophageal fistula repair in a patient with situs inversus treated in our Clinic.

Abnormalities, Multiple↗

A corrected transposition of the great arteries with situs inversus visceralis and cleft palate, but without other cardiac defects.

A 17-year-old female admitted for cleft palate surgery was referred for further evaluation of her cardiac condition. The patient had been diagnosed as dextrocardia at birth, without any cardiac murmurs. She has led a normal life. The apical impulse was felt at the fifth intercostal space at the right mid-clavicular line. The ECG and chest x-rays were strongly suggestive of corrected transposition of the great arteries (CTGA) with situs inversus visceralis. Findings of the two-dimensional and Dopplar echocardiograms showed CTGA with a mild morphologic tricuspid regurgitation without any other complicated cardiac anomalies. In the Japanese literature, 36 CTGA patients without associated cardiac defects have been reported. Of these, four patients revealed dextrocardia. To our knowledge, our patient is the first reported adult case without any cardiac defects, only minimal tricuspid regurgitation, situs inversus totalis and cleft palate.

Abnormalities, Multiple↗

Prenatal echocardiographic diagnosis of situs inversus totalis and transposition of the great arteries: a case report.

A case of situs inversus totalis and transposition of the great arteries (TGA) was diagnosed prenatally at 25 weeks' gestation. Postnatal echocardiographic examination confirmed the antenatal findings. This case underscores the importance of recognizing situs abnormalities during obstetric and fetal echocardiographic examination, as they are often associated with cardiac anomalies. Accurate prenatal diagnosis of structural heart defects is extremely important in family counselling and in planning obstetric and postnatal treatment.

Diabetes, Gestational↗

Agnathia, holoprosencephaly, and situs inversus: a third report.

We report on a male infant with cyclopia, agnathia, and situs inversus. In addition, anal atresia and horseshoe-kidneys were present. To our knowledge, this is the third published case of the "agnathia-holoprosencephaly-further midline defects association."

Abnormalities, Multiple↗