Bilateral progressive essential iris atrophy and keratoconus with coincident features of posterior polymorphous dystrophy: a case report and proposed pathogenesis.

We report the first case known to us of an apparent bilateral association of essential iris atrophy (EIA) and keratoconus (KC), with coincident features of posterior polymorphous dystrophy (PPD). Based on this case and the published natural history and findings of both the irido corneal endothelial (ICE) syndrome and PPD, we propose a new hypothesis for the pathogenesis of the ICE syndrome with associated KC and/or PPD. We suggest that, similar to the genetics of retinoblastoma, the predisposition for either the ICE syndrome or for PPD is inherited as an inactive allele, the so-called "first hit." Inactivation of the second allele, or "second hit," which could occur at any time, might be the product of the background mutation rate or of an environmental trigger. Dedifferentiation or an abnormality in normal development could occur after the first or second hit, resulting in varying clinical patterns. We also concur with other investigators that PPD could be part of the spectrum of the ICE syndrome, owing to similarities in their clinical presentations, histopathology, specular and electron microscopy, and natural history.     

Evaluation of 99Tcm Technegas ventilation scintigraphy in the diagnosis of pulmonary embolism.

In the diagnosis of pulmonary embolism, perfusion lung scintigraphy offers high sensitivity but low specificity. The specificity can be significantly increased by the use of combined ventilation and perfusion studies. Most aspects of perfusion lung scintigraphy are uniformly accepted but the technique of ventilation imaging varies from centre to centre. This study describes a new technique for the performance of ventilation scintigraphy using a suspension of ultrafine carbon particles labelled with 99Tcm ("Technegas"). The technique combines the ready availability of 99Tcm and its optimal imaging properties with an easily administered radiopharmaceutical of particle size sufficiently small to deposit in the alveoli. Of 63 patients studied by conventional perfusion scintigraphy plus Technegas ventilation scintigraphy, images of diagnostic quality were obtained in all. 31 of these patients also had a ventilation study using 81Krm gas and in only one instance did the two methods of ventilation imaging lead to differing interpretations. We conclude that high quality diagnostic images may be obtained using this new technique, which can be made available on both a routine and an emergency basis, thus improving the service provided for patients suspected of having pulmonary embolism.     

Surgical resection in the management of small cell carcinoma of the lung

In an attempt to define the role of initial surgical resection in patients with undifferentiated small cell carcinoma of the lung, we reviewed the experience of the Veterans Administration Surgical Oncology Group (VASOG). One hundred forty-eight patients with small cell carcinoma of the lung had undergone a potentially "curative" resection. This represented 4.7% of "curative" resections carried out in four major prospective adjuvant chemotherapy trials. In the early trials (101 patients), 16 patients (15.8%) died within the first 30 postoperative days. These patients have been excluded from the analysis of long-term survival, since in the more recent trials postoperative deaths were excluded prior to randomization. In the 132 patients remaining, the 5 year survival rate by the life-table method was 23.0%. The tumor of each was classified pathologically by the TNM system. Five-year survival rates for each category were as follows: T1 N0 M0 59.9%, T1 N1 M0 31.3%, T2 N0 M0 27.9%, T2 N1 M0 9.0%, and any T3 or N2 3.6%. The effect of postoperative adjuvant chemotherapy was evaluated in each of the trials. No beneficial effect of the adjuvant therapy was noted with a one or two course regimen of either nitrogen mustard or cyclophosphamide, but possible benefit, although not significant, was noted in a prolonged intermittent chemotherapy trial of cyclophosphamide either alone or alternating with methotrexate. In the most recent trial of prolonged intermittent courses of 1-(2-chlorethyl)-3-cyclohexyl-l-nitrosourea (CCNU) and hydroxyurea, a 5 year survival rate of 80.8% was noted in those receiving adjuvant chemotherapy as compared to a 38.1% in the control group. We conclude that resection is definitely indicated in patients with T1 N0 M0 lesions and probably indicated in those with T1 N1 M0 or T2 N0 M0 lesions. Primary surgical resection is contraindicated in patients with any other TNM category.     

Quality measurement and control in physician decision making: state of the art.

The status of the concept of controlling the quality of physicians is reviewed as are studies that have been done on methods to measure and improve quality. The conclusions are dismal given the rhetoric and actions on attempts to measure and control quality of physician decision making. Massive attempts are being made, for example, mandating PSROs to monitor quality before there is a methodology. Cost and quality reviews have, at most, a marginal impact and do not deserve the expenditures to conduct them. Studies on utilization review show minimal impact on reducing utilization. Administrative reviews reduce utilization for certain specific, narrowly defined procedures such as injections. There is no operational definition of "unnecessary" utilization. The tendency is to regard the lowest levels as optimal, presumably because they result in lower expenditures. Bureaucratic reviews do not provide incentives to decision makers the way various types of HMO delivery types do from current evidence. Hence, HMOs, deductibles and coinsurance, and competition have greater promise for limiting expenditures than do utilization reviews. Quality audits are also marginally effective because of limited promise of changing provider behavior given current methods of doing so. They miss the organizational aspects in which incentives are generated. Research is needed, therefore, on alternative forms of quality and cost control such as HMOs, physician risk sharing, competitive models, and deductibles and coinsurance. Until the much needed research has been done, the amount of resources spent on review should be minimized. In the meantime, the review processes should concentrate on extreme variations of very narrowly defined criteria of proven validity while improving the review methodology by systematic research on quality monitoring.     

Autogenous artery and vein grafts in common carotid-supraclinoid carotid anastomoses

Microsurgical techniques employing either autogenous saphenous arterial grafts or venous grafts were used for common carotid-supraclinoid carotid anastomoses (CC-SCAs) in dogs. The CC-SCA provides a large volume of blood to a major vessel supplying the brain. Within 10 to 14 days postoperatively, angiography and postmortem examination showed arterial patency in 9 (69%) of 13 dogs and venous patency in 2 (25%) of 8 dogs. The discrepancy in patency between the arterial and venous grafts may be the result of the differences in construction of arteries and veins, technical failure, shear stress, turbulence, and/or boundary layer separation. The application of autogenous arterial grafts (i.e., radial, hypogastric) in humans seems justified.     

A phase II study of carboplatin and paclitaxel in esophageal cancer.

BACKGROUND: This study was conducted to evaluate the efficacy and toxicity of combination carboplatin and paclitaxel in patients with esophageal cancer. MATERIALS AND METHODS: Thirty-five patients were enrolled. Patients were treated with paclitaxel 200 mg/m(2) intravenously (i.v.) over 3 h and carboplatin i.v. at an AUC of 5 mg/h/ml. Thirty-three patients were assessable for toxicity and objective response. RESULTS: A total of 166 treatment courses were administered with a median of five courses per patient. The objective response rate was 43% [90% confidence interval (CI) 0.3-0.58] by the intention-to-treat analysis. The median response duration was 2.8 months (90% CI 2.1-5.4). The median survival time was 9 months (90% CI 7-13.8) and the 1-year survival rate was 43% (90% CI 0.29-0.57). The major grade 3-4 toxicity observed was neutropenia, occurring in 17 patients (52%). There were no treatment-related deaths. CONCLUSIONS: The combination of carboplatin and paclitaxel is an moderately active and tolerable regimen in advanced esophageal cancer.     

New non-cocaine-containing topical anesthetics compared with tetracaine-adrenaline-cocaine during repair of lacerations

OBJECTIVE: To compare the effectiveness of three new topical anesthetics that do not contain cocaine (prilocaine-phenylephrine, tetracaine-phenylephrine [tetraphen], and tetracaine-lidocaine-phenylephrine) to that of tetracaine-adrenaline-cocaine (TAC) during laceration repair in children. DESIGN: Prospective, randomized, double-blind clinical trial. SETTING: The emergency department of an urban children's hospital. PARTICIPANTS: Children 1 year of age or older with a laceration /= 5 years of age using a visual analogue scale (VAS). Suture technicians, research assistants, and parents also scored pain using a seven-point Likert scale. In addition, suture technicians completed an anesthetic effectiveness scale. RESULTS: There was consistently no difference demonstrated between the effectiveness of tetraphen and that of TAC for each outcome measure of each observer group. A statistically significant difference was seen among anesthetics when comparing VAS and Likert scale scores of suture technicians and Likert scale scores of research assistants. Based on post hoc analyses, these statistically significant differences were between TAC and prilocaine-phenylephrine (suture technician VAS and Likert scale) and between TAC and tetracaine-lidocaine-phenyl-ephrine (suture technician Likert scale), but not between TAC and tetraphen. When power analyses were performed using alpha = 0.05 and beta = 0.20, it was possible to detect a difference of 1.2 VAS units for each of the observer groups. Based on anesthetic effectiveness scale scores, the three new topical preparations collectively performed significantly better on the face and scalp than on the extremities (relative risk = 1.83; 95% confidence interval 1.20 < relative risk < 2.79). CONCLUSION: This study demonstrated the effectiveness and safety of three new non-cocaine-containing topical anesthetics. Consistently, there was no statistical difference demonstrated between the effectiveness of tetraphen and that of TAC for each outcome measure of each observer group. Tetraphen offers an effective alternative to TAC during laceration repair in children.     

Satisfaction with midwife-managed care in different time periods: a randomised controlled trial of 1299 women.

OBJECTIVE: To compare women's satisfaction with midwife-managed care with 'shared care' over three different time periods. DESIGN: Randomised controlled trial. SETTING: Glasgow Royal Maternity Hospital, Glasgow, UK. PARTICIPANTS: 1299 women experiencing normal pregnancy (consent rate: 82%). Six hundred and forty-eight women were randomised to midwife-managed care and 651 to 'shared care'. METHODS: Three self-report questionnaires were sent to women's homes. The questionnaires examined: satisfaction with antenatal care at 34-35 weeks' gestation, and satisfaction with intrapartum, hospital- and home-based postnatal care at seven weeks postnatally. The third questionnaire reviewed satisfaction with intrapartum care seven months after delivery. FINDINGS: Women in both groups were satisfied. However, women in the midwife-managed group were more highly satisfied in relation to the dimensions examined: relationships with staff, information transfer, choices and decisions, and social support. The differences between the two groups were evident for all time periods (i.e. antenatal, intrapartum and postnatal periods) and were sustained at seven-month follow-up. This is illustrated in the mean scores for relationships with staff, as measured at 34-35 weeks' gestation (possible range -2; very negative attitudes to 2; very positive attitudes). Women in the midwife-managed group scored a mean of 1.22 compared to 0.74 for the 'shared care' group (mean diff: 0.48; 95% CI: 0.42 to 0.55). While women in both groups were more likely to make positive rather than negative comments in open-ended questions, the midwife-managed group were more likely to make positive comments whereas the 'shared care' group were more likely to make negative comments. CONCLUSION: Midwife-managed care for healthy pregnant women which is integrated into existing services improves satisfaction with antenatal, intrapartum and postnatal care.