A survey of some genetical characters in Ethiopian tribes. I. Glutathione stability and glucose-6-phosphate dehydrogenase activity in red blood cells.
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Biomedical subjects
Publications and source records attributed to A Adam.
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Amniocentesis and subsequent tests are reported on a fetus conceived of a rare mating type: its mother has an intermediate level of beta hexosaminidase A (HEX A), characteristic of carriers of Tay-Sachs disease (TSD), while the father suffers from an adult-onset GM2 gangliosidosis (AOG) with severe HEX A deficiency. Activity of HEX A in the cultured fetal cells was very low when measured by the heat-inactivation method, thus showing the typical biochemical phenotype of TSD fetuses. However, upon separation of HEX isozymes by ion exchange chromatography, residual HEX A (17 per cent of total HEX) was demonstrated. Also in contrast to TSD fetuses, this fetus' fibroblasts were able to synthesize the precursor of alpha chains of HEX, and ultrastructural examination of its brain revealed few atypical lamellar bodies, unlike those found in TSD fetuses of the same gestational age. It is therefore concluded that the fetus was not affected with TSD, but rather with AOG.
In patients with obstructive jaundice caused by unresectable malignant tumors, biliary endoprostheses inserted percutaneously or endoscopically can provide excellent palliation. Conventional plastic stents are associated with a relatively high rate of occlusion caused by biliary sludge. Migration is another significant problem. Self-expandable, metallic stents can be inserted percutaneously via a small transhepatic track but expand to achieve a relatively large internal diameter. This minimizes the problem of occlusion due to encrusted bile and reduces the rate of reintervention. Migration rarely occurs. Metallic stents have also been employed in the management of recurrent benign biliary strictures unsuitable for surgery. In those patients in whom the frequency of radiological intervention is unacceptably high, such endoprostheses can provide a means of preventing restenosis.
Hepatic intraarterial injection of Lipiodol has been used by various authors to enhance the diagnostic accuracy of computed tomography in hepatic tumors. The technique appears safe and seems free from serious complications when used judiciously. We report a case in which injection of hepatic intraarterial Lipiodol precipitated acalculous cholecystitis requiring urgent laparotomy.
Twenty-five patients with stenosis of the vena cava (21) and other large veins (4) have been treated with self-expanding Gianturco metallic stents. Eighteen patients had superior vena cava syndrome. In 17, the stricture was due to malignant superior vena cava compression recurrent after maximum tolerance radiotherapy and/or chemotherapy. In 16 of these patients there was early symptomatic relief. In 1 patient with a benign posttraumatic superior vena cava stricture, the stenosis was not relieved, and occlusion occurred after 1 month. Stenoses associated with dialysis shunts were relieved in 2 other patients. Two malignant and one benign inferior vena cava stenoses were relieved either until death, or in the benign case, for 30 months. One malignant subclavian vein obstruction occluded after 24 h due to stent misplacement and another with extrinsic mediastinal compression remained patent until death, extensive thrombus having been lysed prior to stent placement. The results of this short series suggest that the Gianturco self-expanding stent in the vena cava and large veins is easy and safe to place, and in most cases produces almost immediate palliation of the distressing effects of venous obstruction, often in a preterminal and inoperable patient.
Metal endoprostheses of the Wallstent type were successfully inserted percutaneously and endoscopically in 80 consecutive patients with malignant obstructive biliary stenoses, who were followed for up to 18 months. The indication for treatment was jaundice due to malignant biliary obstruction. Repeat radiological investigations were performed if the patient had symptoms suggesting stent occlusion. After stent implantation, 88% of patients demonstrated a serum bilirubin decrease by more than 50%. We observed a 15% rate of serious complications, including a 10% rate of cholangitis with septicemia. There were no cases of stent migration or occlusion due to encrustation of bile. Recurrent jaundice occurred in 17.5% of patients due to progressive tumor growth after 3-10 months. In 5 of these patients, tumor overgrowth was redilated and/or restented. Of the 80 patients, 34% are alive after 2-12 months (mean: 242 days); of these, two-thirds are free of jaundice. Sixty-six percent of patients died between 3 days and 1.5 years (mean: 133 days). Although autopsy investigations revealed the possibility of tumor growth onto the inner surface of the stent, through the mesh of the endoprosthesis, no stent occlusion by tumor ingrowth into the lumen occurred. Self-expandable stainless steel endoprostheses provide good palliation in patients with malignant obstructive jaundice.
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Perforation of the esophagus is a very rare complication of metallic esophageal stent insertion. Two cases are presented in which esophageal perforations were caused by the sharp ends of metallic stents impinging on the esophageal wall. In retrospect, both perforations might have been prevented by additional stent insertion.
A 60-year-old man with a malignant esophageal perforation could not be treated by conventional covered metallic stents because the upper esophagus was dilated. The perforation was eventually closed by deployment of a prototype, conical covered Wallstent.
The positive bronchus sign is the CT finding of a bronchus leading to or contained within the primary mass. A prospective study was performed for the purpose of establishing the correlation between the above sign on CT and the visual and pathological findings on bronchoscopy. The predictive value for the positive bronchus sign was found to be 94% and that of the negative bronchus sign 62%. CT is useful in predicting the likelihood of subsequent bronchoscopy providing positive results.
PURPOSE: In this retrospective multicenter study, the authors analyzed the clinical efficacy of different metallic stents in the palliative treatment of patients with neoplastic obstructive jaundice. PATIENTS AND METHODS: Two hundred forty patients were treated in four European centers. Causes of obstruction were pancreatic carcinoma (n = 84), biliary neoplasm (n = 99), metastases in hilar nodes (n = 34), primary or secondary liver tumors (n = 4), and other tumors (n = 19). A total of 388 metallic stents were used: 300 Wallstents, 35 nitinol Strecker stents, 40 Gianturco-Rosch Z stents, and 13 tantalum Strecker stents. RESULTS: Overall 25- and 50-week survival rates were 42% and 16%, respectively; the 30-day mortality rate was 14.6%. Two deaths were related to the procedure (0.8%); 19 patients (8%) had major complications. The 25-week patency rate was significantly higher for the nitinol Strecker stents and the Wallstents (78% and 67%, respectively) than for the Z stents and the tantalum Strecker stents (30% and 20%, respectively) (P < .01 and P < .001, respectively). Average patency was 8.3, 5.9, 2.3, and 4.0 months, respectively. Reintervention due to stent obstruction was necessary in 53 patients. CONCLUSION: The Wallstent and the nitinol Strecker stents were the most effective in achieving long-term palliation. Patency was significantly affected by the level of obstruction but not by the type of obstructing tumor.