Congenital cardiac malformations in Adams-Oliver syndrome

Whole genomic hprt clones were used in Southern analysis to screen the integrity of the hprt gene in a family that includes a patient with HPRT enzyme deficiency causal to Lesch-Nyhan syndrome. A 5 kb DNA sequence deletion was found to have its endpoints in the first and third introns. The probes identified the carrier status of female family members, aided by an RFLP carried by the mother's normal X-chromosome.     

Prospective study on the value of endosonographic follow-up after surgery for esophageal carcinoma

Background: Half of the patients who undergo surgery for cancer of the esophagus or gastric cardia present with recurrent disease within 2 years after the operation. We investigated the value of endosonography for the early detection of recurrent disease. Methods: Forty-three patients entered a follow-up protocol in which endosonography was performed every 6 months within the first 2 years after resection. Results: During 16 of a total of 66 examinations, suspicious abnormalities were found. In three patients free fluid was seen, but recurrence could not be confirmed during follow-up. In eight patients suspicious lymph nodes were seen; six died within 6 months, one was alive with a proven recurrence at 6 months, and one was alive without recurrence at 22 months. In five patients focal wall-thickening or a mass was seen; three died within 6 months, and two were alive with a proven recurrence at 2 and 5 months. After exclusion of free fluid, the positive predictive value of abnormalities on endoscopic ultrasonography (EUS) was 92%. Conclusions: Endosonography, performed at 6-month intervals after resection of cancer of the esophagus or gastric cardia, is accurate in the early detection of locoregional recurrent disease. Two thirds of the patients were still without symptoms when the recurrence was found. (Gastrointest Endosc 1997;46:487-91.)     

A novel technique of ab interno glaucoma surgery: follow-up results after 24 months

Purpose It was the aim of this study to investigate the efficacy, longevity, and safety of a new ab interno intervention for the treatment of primary open-angle glaucoma (POAG). Methods The previously described method of radiofrequency-mediated “sclerothalamotomy ab interno” was applied in 53 eyes of consecutive patients with POAG between April 2002 and July 2002. Average preoperative intraocular pressure (IOP) was 25.6±2.3 mmHg (range 18–48 mmHg). Sclerothalamotomies were carried out with a custom-made high-frequency dissection 19 G probe (tip 0.3×1 mm) applying bipolar current with a frequency of 500 kHz (tip temperature 130°C). Results After a follow-up period of 24 months, the average IOP was 15.0±1.6 mmHg (range 11–20 mmHg) (p<0.005). The average number of topical agents was 2.6±1.0 (range 1–5) preoperatively. Twenty-four months after surgery such agents were used in only five (9.6%) eyes and the average was 0.21±0.53 (range 0–2). Transient IOP elevation was observed in 12 of 53 eyes (22.6%) postoperatively. In all cases elevated IOP could efficiently be controlled with topical medication. In general, IOP dropped continuously over the course of the 6 months following surgery and then remained constant. Conclusions This study indicates that sclerothalamotomy ab interno is a safe and efficient surgical method for the treatment of POAG. Long-term results clearly demonstrate the longevity of IOP reduction.     

Increase in the degree of phosphorylation of circulating fibrinogen under thrombolytic therapy with urokinase

Human fibrinogen is phosphorylated in vivo to an equal extent at two positions, one at Ser 3 located on fibrinopeptide A, the other at Ser 345 of the A alpha-chain. As has been shown previously, the degree of phosphorylation of the circulating fibrinogen pool can be determined in vitro from the ratio between the HPLC peaks formed by phosphorylated and non-phosphorylated fibrinopeptide A which has been cleaved from plasma fibrinogen by thrombin or reptilase. Plasma samples were obtained from patients with venous thrombosis undergoing fibrinolytic therapy with urokinase (n = 8). The degree of phosphorylation increased from about 35% before treatment to values between 50% and 70% within 48 hours. It remained at these high levels as long as urokinase was administered and declined slowly thereafter. This behaviour of the degree of phosphorylation of fibrinogen is explained by a model which assumes that fibrinogen is secreted in the phosphorylated form and then dephosphorylated in the circulation by an up to now unidentified phosphatase by first order kinetics. When this system is in steady state, the degree of phosphorylation is about 25% under normal conditions. If the elimination rate of fibrinogen is greatly enhanced by fibrinogenolysis the system will approach a new steady state with a higher degree of phosphorylation, the magnitude of which will depend on the new ratio of dephosphorylation and elimination.     

Safety and efficacy of pimecrolimus cream 1% in the daily practice: Results of a patient self‐observation study in patients with atopic dermatitis

Background: Pimecrolimus cream 1% has proven to be well‐tolerated and effective in controlled clinical studies in patients with atopic dermatitis (AD). In a 15‐week patient self‐observation study, safety and efficacy was investigated in the daily practice. Patients and methods: 3502 patients with AD (mean age 26.2 ± 18 years, 62% female) received pimecrolimus cream 1% from 810 physicians in the German Federal Republic.The severity of the disease was assessed at baseline, two times during the 15‐week observation period and at the end of treatment.Patients recorded daily the degree of erythema and pruritus. At the end of treatment, safety and efficacy were assessed by the physician based on patient's daily records and by the patient. Results: The percentage of patients with severe or massive AD decreased from 25% to 7%, whereas the percentage of patients without or with mild symptoms increased from 9% to 55%.The efficacy of treatment was rated by physicians as good or very good in 83.5% of cases and by 79% of patients.At baseline 35% of the patients were free of flares as compared to 75% at the end of therapy. Disease control was better in patients who followed the recommended treatment algorithm for pimecrolimus cream.Tolerability was mostly rated as good or very good. Conclusion: Treatment with pimecrolimus cream 1% for patients with AD is well‐tolerated and effective in daily practice.     

Multicenter Randomized Comparison of Xenon and Isoflurane on Left Ventricular Function in Patients Undergoing Elective Surgery

Background: Volatile anesthetics are commonly used for general anesthesia. However, these can induce profound cardiovascular alterations. Xenon is a noble gas with potent anesthetic and analgesic properties. However, it is uncertain whether xenon alters myocardial function. The aim of this study was therefore to investigate left ventricular function during anesthesia with xenon compared with isoflurane.

Methods: The authors performed a randomized multicenter trial to compare xenon with isoflurane with respect to cardiovascular stability and adverse effects in patients without cardiac diseases scheduled for elective surgery. Two hundred fifty-nine patients were enrolled in this trial, of which 252 completed the study according to the protocol. Patients were anesthetized with xenon or isoflurane, respectively. Before administration of the study drugs and at four time points, the effects of both anesthetics on left ventricular function were investigated using transesophageal echocardiography.

Results: Global hemodynamic parameters were significantly altered using isoflurane (P < 0.05 vs. baseline), whereas xenon only decreased heart rate (P < 0.05 vs. baseline). In contrast to xenon, left ventricular end-systolic wall stress decreased significantly in the isoflurane group (P < 0.05 vs. baseline). Velocity of circumferential fiber shortening was decreased significantly in the xenon group but showed a more pronounced reduction during isoflurane administration (P < 0.05 vs. baseline). The contractile index (difference between expected and actually measured velocity of circumferential fiber shortening) as an independent parameter for left ventricular function was significantly decreased after isoflurane (P < 0.0001) but unchanged using xenon.     

Clinical trial comparing plasma arc and conventional halogen curing lights for orthodontic bonding.

The purpose of this clinical trial was to evaluate the reliability and time saved with a plasma arc curing unit (Apollo 95E, Dental/Medical Diagnostic Systems, Woodland Hills, Calif) compared with a conventional curing unit (Ortholux XL 3000, 3M Unitek, St Paul, Minn) for direct bracket bonding with resin adhesive. Forty-five patients were involved in the study, and 608 brackets were bonded in a contralateral quadrant pattern. The patients were followed for a mean (+/- standard deviation) period of 11 +/- 3.2 months. Survival analysis was carried out to compare the failure rate for the 2 techniques. The time required for bonding with each technique was also recorded. The mean survival time was 399 days, and there were no significant differences in survival time between the 2 bonding methods. Twelve bonding failures were reported with each technique. The curing time per bracket was significantly reduced with the plasma curing light compared with a conventional curing unit (65 +/- 19 vs 82 +/- 31 seconds). The plasma arc curing light can save chair-time without affecting the bonding failure rate.     

Intensity-Modulated Radiotherapy (IMRT) of Localized Prostate Cancer

PURPOSE: The present status of intensity-modulated radiation therapy (IMRT) for treatment of localized prostate cancer is discussed. METHODS: The technological basis of IMRT and the rationale for the use in treatment of prostate cancer are described. Clinical results from the literature are presented and treatment strategies for further reduction of safety margins are outlined. RESULTS AND DISCUSSION: Multiple planning studies demonstrated the dosimetric advantage of IMRT compared to three-dimensional conformal radiotherapy. Though randomized studies are missing, retrospective studies indicate that improved dose distributions of IMRT transfer into improved rates of local control and/or lower rates of rectal toxicity. However, with standard safety margins the benefit of IMRT seems to be limited. Image guidance is considered to be essential to reduce errors of patient setup and internal motion of the prostate.     

Minimal instructions improve the performance of laypersons in the use of semiautomatic and automatic external defibrillators

Introduction  

There is evidence that use of automated external defibrillators (AEDs) by laypersons improves rates of survival from cardiac arrest, but there is no consensus on the optimal content and duration of training for this purpose. In this study we examined the use of semiautomatic or automatic AEDs by laypersons who had received no training (intuitive use) and the effects of minimal general theoretical instructions on their performance.