Perforation of the Pharynx in the Newborn: A Condition Mimicking Esophageal Atresia

Three cases of perforation of the pharynx in the newborn are presented. This condition resembles esophageal atresia, in that: (a) the symptoms are identical; (b) in both conditions there is failure to pass a nasogastric tube; (c) opacification of the esophagus through a nasogastric tube gives x-ray pictures confusingly similar. Careful examination of the radiographs can differentiate the two conditions. Although the three patients presented were operated on, conservative treatment is probably adequate for this condition.     

The utility of the population approach applied to bioequivalence in patients: comparison of 2 formulations of cyclosporine

Mixed-effect modeling was used to compare the population pharmacokinetics of 2 formulations of cyclosporine in patients. An open-label, multicenter, conversion study in stable, 6-month post-renal allograft recipients was conducted to compare the safety and pharmacokinetics of oral Pliva Cyclosporine Soft Gelatin Capsules (USP Modified) with Neoral (cyclosporine soft gelatin capsules, USP Modified) in stable post-renal transplant patients. Blood samples were collected predose and for 12 hours postdose on days 1, 14, 15, 28, and 29. Whole-blood samples were analyzed for cyclosporine using high-performance liquid chromatography and mass spectroscopy. Estimates of pharmacokinetic parameters were generated using noncompartmental and population compartmental pharmacokinetic analysis. Moreover, the effects of demographic factors on the pharmacokinetics of cyclosporine were evaluated using the nonlinear mixed-effects modeling program NONMEM. The rate and extent of bioavailability of cyclosporine did not differ between Pliva Cyclosporine Soft Gelatin Capsules and Neoral. In the final model, gender and actual body weight significantly affected the central and peripheral volumes of distribution. In addition, the pharmacokinetics of cyclosporine was defined robustly in this patient population using population pharmacokinetic approaches. Results indicate that the Pliva Cyclosporine Soft Gelatin Capsules and Neoral are bioequivalent when administered to renal transplant patients. Pliva Cyclosporine Soft Gelatin Capsules can then be substituted for Neoral in stabilized patients without anticipating dose adjustments.     

Spigelian Hernia

We report on the evolution in concept and techniques that allowed us to improve the treatment of spigelian hernia, operable in day surgery in 90% of cases and through a preperitoneal and recently a preperitoneal and subfascial prosthetic repair (PHS). Background data. We propose an innovative use of the PHS mesh for spigelian hernia repair. With this new implementation, we confront the standard surgical technique and its postoperative period. Methods. From January 1992 to March 2004, we performed 2,500 hernia surgical operations, including 32 spigelian hernia repairs (1.3% of total case series). The first surgical approach used for 20 of these 32 patients (62.5% of total spigelian hernias), all electively operated on, was a classical preperitoneal repair (Wantz), performed when possible by size of defect and weight (Body Mass Index) of the patient, under local anesthesia and on a day-surgery basis. Our new modified technique takes place through the insertion of a PHS large-type mesh, whose bottom underlay portion lies flat in the preperitoneal space with the connector obliterating the hernial orifice and with the overlay portion lying on the internal oblique muscle, covered by the aponeurosis of the external oblique muscle. Results. Our modification to the classical technique consisted only in the application of a product, such as the PHS, in a hernia defect, which presented with an orifice of the size of the connector and, therefore, was easily repairable with the use of the PHS device. This approach is easier than the preperitoneal approach, its always suitable for local anaesthesia, and it gives a more comfortable postoperative period. The surgical approach may be performed completely in day surgery. Conclusions. We believe that spigelian hernia surgical repair should always be performed by means of a preperitoneal prosthesis under local anaesthesia when the patients clinical and physical conditions allow for it, always in day surgery, and using the PHS mesh when the hernia defect size fits with the connector diameter. This last possibility seems to be easier and more comfortable for the patient in the postoperative period.     

Health promotion and disease prevention for older adults: Intervention themes and strategies used in Quebec local community health centres and seniors' day centres

OBJECTIVE: Despite the considerable potential of disease prevention and health promotion (DPHP) among older adults, extant data suggest that this field of intervention is still underdeveloped. To shed further light on this issue, this paper presents the results of an inventory of DPHP interventions for older adults conducted in local community health centres (CLSCs) and seniors' day centres in the province of Quebec. METHODS: All CLSCs (N=147) and day centres (N=124) were invited to participate (response rates: 74% and 79%). Data were collected through telephone interviews. Interventions were coded according to type of intervention strategies and target themes. RESULTS: Awareness-raising and health education strategies emerged as the most frequently-cited type of intervention strategies, reported by 77% of CLSCs and 95% of day centres, respectively. The two themes reported by a majority of CLSCs were physical health (87%) and community issues (58%). Lifestyle habits (92%) and social issues (92%) were the two most frequently-cited themes in day centres. DISCUSSION: DPHP for older adults is substantially well developed in terms of intervention offerings in the two types of organizations under study. However, the range of available interventions requires expansion to increase the potential of DPHP programs to tackle the numerous challenges posed by the aging of the population.     

Seasonal congestive heart failure mortality and hospitalisation trends, Quebec 1990-1998

STUDY OBJECTIVE: To describe seasonal congestive heart failure (CHF) mortality and hospitalisations in Quebec, Canada between 1990-1998 and compare trends in CHF mortality and morbidity with those in France. DESIGN: Population cohort study. SETTING: Province of Quebec, Canada. PATIENTS: Mortality data were obtained from the Quebec Death Certificate Registry and hospitalisation from the Quebec Med-Echo hospital discharge database. Cases with primary ICD-9 code 428 were considered cases of CHF. RESULTS: Monthly CHF mortality was higher in January, declined until September and then rose steadily (p<0.05). Hospital admissions for CHF declined from May until September (moving averages analysis p<0.0001). Seasonal mortality patterns observed in Quebec were similar to those observed in France. CONCLUSION: CHF mortality in Quebec is highest during the winter and declines in the summer, similar to observations in France and Scotland. This suggests that absolute temperatures may not necessarily be that important but increased CHF mortality is observed once environmental temperatures fall below a certain "threshold" temperature. Alternatively better internal heating and warmer clothing required for survival in Quebec may ameliorate mortality patterns despite colder external environments.     

Family of two patients with congenital lipoid adrenal hyperplasia due to StAR mutation

We are reporting the case of two sisters born to nonrelated French Canadian parents. Patient A is of female phenotype with 46,xy, and patient B with 46,xx. The children had severe manifestations of mineralocorticoid deficiency at the age of 11 and 4.5 months, respectively. Residual cortisol secretion seemed present until the age of 3 years for patient A and until 15 months in the case of her sister. Both patients responded to glucocorticoid and Florinef treatment. Patient A did not show any androgen secretion and gonadectomy was performed at the age of 13.4 years; estrogen therapy was started at the age of 14 years resulting in a good breast development and an increase of growth velocity. In patient B, a progressive development of secondary sex characters occurred at 11.6 years of age followed at 14 years by menarche associated with a normal secretion of LH, FSH and estradiol; regular menstruations continued up to her last visit at the age of 25 years. We identified a homozygous L275P mutation on the StAR gene of both patients and a heterozygous L275P mutation on that of their mother and father. In transfection analysis in COS-1 cells, the mutant L275P was well-expressed, but its StAR activity was 87% impaired. The remaining activity of the L275P StAR mutant is consistent with the moderate severity of clinical onset of manifestations.     

Errorless academic compliance training: improving generalized cooperation with parental requests in children with autism

OBJECTIVE: Children with autism often demonstrate distress and oppositionality when exposed to requests to complete academic or household tasks. Errorless academic compliance training is a success-focused, noncoercive intervention for improving child cooperation with such activities. In the present study, the authors evaluated treatment and generalization effects of this intervention on four children diagnosed with autism. METHOD: In a multiple baseline across-subjects design, parents delivered a range of academic and nonacademic requests to their children to determine the probability of compliance for each request. A hierarchy of academic requests ranging from those yielding high compliance (level 1) to those yielding low compliance (level 4) was then developed. Treatment began with the concentrated delivery of level 1 requests, with praise and reward for compliant responses. Over several weeks, children were gradually introduced to requests from subsequent probability levels with continued reward for compliance. RESULTS: High compliance levels were demonstrated throughout and following treatment. Evidence of generalization to untrained academic requests and nonacademic requests emerged. Treatment gains were maintained up to 6 months after treatment. CONCLUSIONS: Errorless academic compliance training appears to be an effective intervention for enhancing generalized compliance in children with autism.