Influence of birth weight on primary surgical management of newborns with esophageal atresia

BackgroundTo determine if birth-weight (BW) influences primary surgical management of newborns undergoing operation for esophageal atresia and tracheo-esophageal fistula (EA-TEF).MethodsNewborns undergoing repair of esophageal atresia at a single specialist centre between 1999 and 2017 were categorised into three groups based on BW; Group A < 1.5 kg, Group B <2.5 kg and Group C >2.5 kg. Outcome data analysed were (i) technical ability of the surgeon to perform primary esophageal anastomosis, (ii) anastomotic leak, (iii) anastomotic stricture, (iv) esophageal replacement, (v) need for other procedures notably fundoplication, aortopexy, tracheostomy and (vi) mortality. Statistical analysis was performed using a two-tailed Fisher's exact test and logistic regression.Results198 patients underwent surgery for EA-TEF during the study period, Group A (n = 13), Group B (n = 73) and Group C (n = 112). Inability to perform a primary anastomosis was significantly higher in Group A vs Group B (p = 0.003) and Group C (p = 0.004). Birthweight was a significant variable in the ability to perform a primary esophageal anastomosis (OR 1.009, p = 0.004). Mortality rate was significantly higher in Group A vs Group C (P = 0.0158).ConclusionsVery low birth weight infants are less likely to achieve a definitive primary anastomosis during emergent repair of esophageal atresia, and have a higher mortality.     

Reassessing the role of surgery in the elderly or chronically sick with proximal extrahepatic cholangiocarcinoma

BackgroundMost data on postoperative outcomes among patients with proximal extrahepatic cholangiocarcinoma are reported by single institutions. The purpose of this study was to analyze postoperative outcomes stratified by age and comorbidities.MethodsPatients with proximal extrahepatic cholangiocarcinoma who underwent a resection were identified in the National Cancer Database. Pathologic, postoperative, and survival outcomes were compared based on age and Charlson-Deyo comorbidity index.ResultsAmong the 1,579 patients, the average age was 66 years, and 9.4% of patients were older than 80 years. Most patients had a Charlson-Deyo score of 0 (72.4%), with the minority having scores of 1 (20.5%) or ≥2 (7.1%). Patients ≥80 years had a higher 90-day mortality rate compared with patients 65 to 79 and <65 years (21.3% vs 12.0% vs 7.4%, P < .001). Patients with a Charlson-Deyo score ≥2 had longer duration of stay, greater likelihood of requiring an unplanned readmission, and a higher 90-day mortality rate compared with patients with a lower comorbidity index. Median survival of patients <65, 65 to 79, and ≥80 years was 31, 24, and 17 months, respectively. A similar trend was seen with increasing Charlson-Deyo score (0: 27 months, 1: 25 months, ≥2: 20 months). On multivariable analysis, age ≥80 years (hazard ratio = 1.52, P = .01) and Charlson-Deyo score ≥2 (hazard ratio = 1.45, P = .01) were associated with poor survival.ConclusionIn patients with proximal extrahepatic cholangiocarcinoma, age ≥80 years and greater comorbidity index are associated with increased risk of 90-day mortality and poor overall survival. This suggests that resections in high-risk patient populations should be approached with caution.     

Response of the ENPP1-Deficient Skeletal Phenotype to Oral Phosphate Supplementation and/or Enzyme Replacement Therapy: Comparative Studies in Humans and Mice

Inactivating mutations in human ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) may result in early-onset osteoporosis (EOOP) in haploinsufficiency and autosomal recessive hypophosphatemic rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the rachitic phenotype, but elevating plasma phosphorus concentrations in ARHR2 patients may increase the risk of ectopic calcification without increasing bone mass. To assess the risks and efficacy of conventional ARHR2 therapy, we performed comprehensive evaluations of ARHR2 patients at two academic medical centers and compared their skeletal and renal phenotypes with ENPP1-deficient Enpp1^asj/asj mice on an acceleration diet containing high phosphate treated with recombinant murine Enpp1-Fc. ARHR2 patients treated with conventional therapy demonstrated improvements in rickets, but all adults and one adolescent analyzed continued to exhibit low bone mineral density (BMD). In addition, conventional therapy was associated with the development of medullary nephrocalcinosis in half of the treated patients. Similar to Enpp1^asj/asj mice on normal chow and to patients with mono- and biallelic ENPP1 mutations, 5-week-old Enpp1^asj/asj mice on the high-phosphate diet exhibited lower trabecular bone mass, reduced cortical bone mass, and greater bone fragility. Treating the Enpp1^asj/asj mice with recombinant Enpp1-Fc protein between weeks 2 and 5 normalized trabecular bone mass, normalized or improved bone biomechanical properties, and prevented the development of nephrocalcinosis and renal failure. The data suggest that conventional ARHR2 therapy does not address low BMD inherent in ENPP1 deficiency, and that ENPP1 enzyme replacement may be effective for correcting low bone mass in ARHR2 patients without increasing the risk of nephrocalcinosis. © 2021 American Society for Bone and Mineral Research (ASBMR).     

Brief report: Comorbid psychiatric disorders of autistic disorder

Conclusion The behavioral and/or psychiatric symptoms in autistic people as described above have been viewed by many professionals dealing with autistic populations as associated features of autism that may result from these individuals' inability to cope with the environmental demands and physical discomfort. Traditionally, in treating individuals with autism, special education intervention including behavioral modifications has been the main emphasis. Such an approach has made some progress in milder and uncomplicated cases of autism. However, if many of these behavioral and/or psychiatric symptoms in those with more severe associated features can be viewed as symptoms of various comorbid psychiatric disorders, there are data suggesting that with an appropriate evaluation, predrug workups, a specific diagnosis, and multiple measures of outcome, pharmacotherapy can be a safe and efficacious adjunct treatment for some symptoms in autistic persons. Nevertheless, the data presented here were obtained mainly from autistic children. A great deal of work remains to be done Future research should put more emphasis on developing agreeable, reliable, and valid diagnostic instruments for identifying comorbid psychiatric disorders in autistic people. Future research should also emphasize employing a randomized double-blind placebo-controlled crossover design, as well as involving multicenters and using uniformed diagnostic criteria to study autistic adolescents and adults.     

The triple flash electroretinogram and its significance in macular diseases

Purpose: The purpose of this study was to evaluate recovery data for the b-wave of the electroretinogram (ERG) elicited using multiple flash stimulation with increasing stimulus intervals in normal controls and in patients with macular diseases. The results will describe effects of age and macular disease and define indexes characterizing the recovery process. Methods: Scotopic Ganzfeld flash ERGS were elicited using interstimulus intervals of 140, 280, and 560 ms. Relative b-waves were calculated as the ratio b_{140 ms}/ b_{560 ms} and b_{280 ms}/b_{560 ms}, respectively. Responses obtained in 134 eyes of 134 normal controls served as reference data. Fifty-four patients with different macular diseases were also examined and their data compared to the reference data. Results: Relative b-wave amplitudes correlated with interstimulus interval and with flash luminance, but not with age. All patients had a normal ERG when recorded following the standard of clinical electroretinography. A sigmoidal model was suggested, providing three indexes characterizing the b-wave recovery process. Relative b-waves and recovery indexes varied in age-related macular degeneration, central serous retinopathy, vitelliform macular degeneration, Stargardt's disease, and pattern dystrophy. Conclusion: The triple flash ERG reflects energy-yielding and -utilizing mechanisms. It proved to be more sensitive in detecting functional lesions in macular diseases than the standardized explorating procedure. The b-wave recovery model allows differentiation between two independent mechanisms contributing to the b-wave recovery process. One or more of the three characterizing indexes are affected in different macular diseases.     

Changes in newspaper coverage of cardiovascular health issues in conjunction with a community-based intervention

Numerous community-based prevention projects, with significant media components, have been conducted over the past decade. Multiple evaluation strategies have been used to document the effectiveness of these interventions, including intermediate measures of community impact such as assessment of media coverage. As part of the evaluation of a community-based intervention (the Bootheel Heart Health Project), dissemination of information on cardiovascular disease (CVD) was measured through a media content analysis of newspapers. Data were analyzed from 23 newspapers in six rural counties in southeastern Missouri for the period October 1988 through August 1993. An increase was observed in CVD-related coverage in the pre-intervention period (mean articles per month = 31.5) compared with the post-intervention period (mean articles per month = 50.7) (F = 10.2; P = 0.003). In supporting data from a separate randomized risk factor survey of 1510 residents in the same area, respondents reported hearing of heart health coalitions primarily through local newspapers. The current study documents increasing print media coverage of cardiovascular health issues in a high-risk, rural area and shows that media content analysis can be a useful evaluation tool in community-based interventions.