Fanconi's anemia treated by allogeneic marrow transplantation

Eight patients with Fanconi's anemia were given cyclophosphamide alone (seven patients) or combined with procarbazine and antithymocyte globulin (one patient) followed by marrow grafts from HLA-identical siblings. All patients had engraftment. Seven developed acute and three chronic graft-versus-host disease (GVHD). Three patients died with GVHD and infectious complications (days 19, 56, and 82) and one with an intracerebral hemorrhage (day 540). Four patients are surviving 647- 3435 days after grafting, two are well, and two have chronic GVHD that is improving. These results show that Fanconi's anemia can be treated successfully by allogeneic marrow transplantation.     

Die Aufklärungspflicht bei endoskopischen Operationen

Ohne Zusammenfassung     

Pain after barium enema: effect of CO2 and air on double-contrast study

One hundred fifty-one consecutive patients scheduled for double-contrast barium enema studies were assigned randomly to insufflation with either air or carbon dioxide (CO2) in a double-blind, prospective trial. Within 24 hours after the enema study, the patients were contacted by telephone by an interviewer, who completed a standard questionnaire. Radiographs from the enema studies were assessed for quality by two radiologists. Pain experienced after the procedure was graded from 0 (none) to 4 (severe). Clinically relevant (grades 2-4) pain was experienced by 30% of patients after insufflation with room air, compared with 11% of patients in whom CO2 was used for insufflation (P = .005). The mean pain score for CO2 was 0.4, and for room air, 1.2 (P less than .005). Although five patients experienced grade 4 pain after insufflation with air, no patient reported severe pain after CO2 insufflation. Post-evacuation films confirmed there was significantly less residual gas in the CO2 group. The quality of radiographs was equal in the two groups. CO2 has advantages for use in the double-contrast barium enema examination.     

Biosynthesis and secretion of factor VII, protein C, protein S, and the Protein C inhibitor from a human hepatoma cell line

Using specific radioimmunoassays, 8 day cultures of Hep G2 cells were shown to contain in their supernatants 16, 74, and 828 ng/mL and in their cell lysates, 8, 55, and 48 ng/2 X 10(8) cells of factor VII, protein C, and protein S, respectively. These proteins and the protein C inhibitor were functionally active, and each of these activities was neutralized by their respective polyclonal antibodies. Although vitamin K had a modest effect, warfarin decreased the activity of secreted factor VII, protein C, and protein S by 50% to 90%. Protein C and protein S antigens were reduced three- to fourfold by warfarin. The protein C inhibitor antigen and activity were unaffected by vitamin K or warfarin treatment. Intrinsic labeling and immunoprecipitation indicated that factor VII, protein S, and the protein C inhibitor were secreted as 52,000, 77,000, and 58,000 molecular weight (mol wt) proteins, respectively. Protein C was secreted as a single-chain protein of about 65,000 mol wt, indicating that all of the vitamin K- dependent proteins are translated and secreted as single-chain molecules. Each of the four proteins studied represented their plasma protein counterparts structurally, functionally, and immunochemically. Thus, all of the known soluble components of the protein C pathway are produced by liver parenchymal cells.     

Centrilobular emphysema: CT-pathologic correlation

Over a 5-year period, 25 patients who had undergone chest computed tomography (CT) died and were autopsied. Their lungs were fixed in the inflated state and were assessed for the presence and severity of centrilobular emphysema (CLE). Three radiologists independently evaluated the CT scans for nonperipheral low-attenuation areas, peripheral low-attenuation areas, pulmonary vascular pruning, pulmonary vascular distortion, and pulmonary density gradient. The CT criterion that best correlated with the presence and severity of CLE was the nonperipheral low-attenuation area. With this CT criterion, lung destruction was correctly identified in 13 of 15 cases. The absence of this criterion resulted in correct identification of eight of ten normal lungs. These preliminary data suggest that CLE can be reliably identified and quantified with current CT scanners.     

Pulmonary embolism after hip or knee replacement: postoperative changes on pulmonary scintigrams in asymptomatic patients

Serial pulmonary imaging has proved to be effective in the evaluation of patients undergoing total joint arthroplasty. A clinical dilemma arises in asymptomatic patients whose postoperative pulmonary images differ from the preoperative images. The authors prospectively evaluated 403 patients with serial imaging to determine the significance of changed postoperative images in asymptomatic patients undergoing total hip or knee arthroplasty. Twenty-two (5.5%) patients had significant changes on postoperative images. Seventeen were asymptomatic; all but one underwent pulmonary angiography. Documented pulmonary emboli were demonstrated in 100% of patients whose postoperative images changed to indicate a high probability of pulmonary embolism, 71% whose images changed to a moderate probability, and 0% whose images changed to indeterminate probability. Overall, pulmonary emboli occurred in 76% of all asymptomatic patients with significantly change postoperative images. Asymptomatic pulmonary embolism is a significant occurrence after total hip or knee repair, and a changed lung scan with appropriate clinical evaluation is an accurate indicator of pulmonary emboli in asymptomatic postarthroplasty patients.     

Cervical internal carotid artery dissecting hemorrhage: diagnosis using MR

Two men underwent high-resolution magnetic resonance (MR) imaging of the internal carotid artery (ICA) 12 and 16 days after spontaneous dissection of this vessel. One underwent follow-up MR imaging 7 weeks later. T1-weighted images were obtained in both cases, and T2-weighted images were obtained in one patient. In both cases, the MR findings corresponded to the angiographic abnormalities. On both the T1- and T2-weighted images, there was a hyperintense lesion expanding the wall and narrowing the lumen of the ICAs. Follow-up MR imaging showed complete resolution of the mural lesion. Axial images best demonstrated the anatomic and MR signal alterations. The hyperintensity of the lesion on both T1- and T2-weighted images indicated a short T1 and a long T2 as expected in a subacute hematoma. High-resolution MR imaging, therefore, can specifically demonstrate a thrombosed carotid dissection noninvasively at least as early as 12 days. The potential to diagnose carotid dissection in the acute phase using high-field-strength MR imaging and its importance for the prevention of embolic strokes are also discussed.     

Effect of repeated sampling and prestimulation on saliva buffer capacity and flow rate values in children

Abstract— Great variations reported in the frequency distribution of buffer capacity values may partly be due to methodological differences in saliva sampling. In this study we wanted to see to what extent these variations are due to repetition and prestimulation. 9-yr-old schoolchildren ( n : 41, 23 girls and 18 boys), who had never had saliva samples taken before, participated in the present study. The repetition tests were carried out between 9 and 11 a.m. on three subsequent days. The effect of prestimulation was tested 4 days later with the same subjects. The buffer capacity of the samples was determined electrometrically immediately after sampling using the commercial Dentobuff-test. The intraindividual analysis revealed a significant increase in flow rate but not in buffer capacity along with repetition of the sampling. The analysis further showed that the buffer capacity of children accustomed to saliva sampling increased significantly through prestimulation of 1 min. The use of prestimulation resulted in substantial changes also in the frequency distribution of the buffer capacity values. Our results emphasize the importance of proper sampling conditions in saliva collection when screening children for high caries risk.