Cornelia de Lange syndrome and congenital diaphragmatic hernia

PurposeThere is a known association between Cornelia de Lange syndrome (CdLS) and congenital diaphragmatic hernia (CDH), with CDH being the cause of death in 5%–20% of CdLS cases. We aimed to identify and describe patients with CDLS and CDH. We hypothesized that CdLS would be associated with high-risk CDH and poor outcomes.MethodsCDH Study Group patients from 1995 to 2019 were included. Those with CdLS were reviewed retrospectively. Rates of repair and outcomes were compared between patients with and without CdLS.ResultsWe identified 9,251 CDH patients. Of those, 21 had confirmed CdLS. CdLS patients had a lower birth weight (2.2 ± 0.57 kg) than non-CdLS patients (2.9 ± 0.64 kg) (p < 0.001). 5-min Apgar scores were lower in CdLS patients (6, 4–7) than non-CdLS patients (7, 5–8) (p = 0.014). Only 33% of CdLS patients underwent diaphragmatic repair compared to 84.2% of non-CdLS patients (p < 0.001). Mortality was 76% for CdLS patients compared with 29% for non-CdLS patients (p < 0.001). Of the 7 CdLS patients who underwent repair, 5 survived to hospital discharge.ConclusionsInfants with CdLS and CDH have a poor prognosis. However, CdLS patients who undergo repair can survive to discharge; therefore, the concomitant diagnosis of CdLS and CDH is not necessarily a contraindication to repair. Early recognition of these anomalies can assist with counseling and prognostication.Type of studyRetrospective comparative studyLevel of evidenceIII     

Despite Equivalent Outcomes,Men Receive Neoadjuvant Chemotherapy Less Often Than Women for Lymph Node-Positive Breast Cancer

Annals of Surgical Oncology -     

Comparison of multiple oncotype DX® from the same patient

For women with breast cancer in whom multiple Oncotype DX^® Recurrence Scores (RS) are obtained, RS concordance utilizing current NCCN recommendations has not been evaluated. Patients with two or more RS were identified. RS were stratified by NCCN guidelines and compared for concordance. Twenty-four patients were evaluated. RS concordance varied by tumor type: 100% in the same tumor, 91.7% in multiple ipsilateral tumors, 71.4% in contralateral tumors, and 66.7% in in-breast recurrent tumors. RS concordance for multiple assays in the same patient is not high enough to omit Oncotype DX^® testing for each tumor.     

Skin angiography assisted mastectomy in secondary breast angiosarcoma: Complete clinical response after neoadjuvant immunotherapy

Radiation-induced breast angiosarcoma, or secondary angiosarcoma (SAS), is a rare entity with a high risk of metastatic recurrence. Herein, we describe the use of intraoperative fluorescence-based skin angiography to guide surgical resection following a novel immunotherapy-based regimen for SAS resulting in a complete pathological response.     

Examining the Macrosystem Level of Influence on Community Health Worker Effectiveness in the State of Nebraska: A Qualitative Approach

Community health workers (CHWs) serve as the linkage between community and providers and are stakeholders for bridging services to the public. However, integration of CHWs into health care organizations is often lacking. This study explored macrosystem level barriers faced by CHWs and their ability to do their jobs effectively. Using qualitative interviews from CHWs (n?=?28) in Nebraska, we used an abductive approach to derive the following themes: (1) CHWs and client macrosystem barriers, (2) CHW workforce supports, and (3) macrosystem solutions for CHW workforce sustainability. Study results also found various macrosystem barriers affecting CHW workforces including immigration policies, insurance policies, funding sources, supervisor support, and obstacles for health seeking of clients. Moreover, through the lens of CHWs, results revealed the need to provide and advocate for solutions that prioritize the needs of CHWs as they continue to fill a crucial gap in community healthcare systems.

     

Gallstones: advantages and disadvantages of five treatment alternatives

Cholecystectomy is the gold standard treatment for symptomatic gallstones. However, within the last decade peroral drug chemolysis, methyl-tert-butyl ether lavage, shock wave lithotripsy, and percutaneous cholecystolithotomy have been introduced. This article compares and contrasts the presently available therapeutic modalities for gallstones and highlights the limitations of each treatment option.     

Preventing alcohol problems: survey of Canadian medical schools.

In preparation for a national conference on medical education in the prevention of alcohol problems, a survey of conference participants was conducted. Participants were undergraduate and postgraduate representatives from each Canadian medical school and representatives from 11 provincial and territorial alcohol and other drug agencies. There was agreement that physicians and medical schools have important roles in prevention and treatment of alcohol problems, with "traditional" medical roles seen as the most important. Current training is variable and was seen as inadequate, with more time devoted to treatment than prevention. To correct this situation, renewed priorities and faculty leadership are needed. Respondents felt that there should be uniform standards for assessing undergraduate students' skills in dealing with alcohol problems. Provincial alcohol and other drug agencies are underused in medical education in the prevention and treatment of alcohol problems.     

The International Classification of Diseases: the structure and content of the Tenth Revision

The Tenth Revision of the International Classification of Diseases is due to be published this year, and introduced from 1993. The aim of the classification is to facilitate comparisons between countries at the same point in time and within, and between, countries over time. The provision of comparable statistics is essential for facilitating policy decisions relating to health promotion and disease prevention, and for the collection of epidemiological data for research purposes. This article describes the major changes and important features in this revision, and compares them to the Ninth Revision of the International Classification of Diseases.     

Volatile organic compounds in the blood of persons in Kuwait during the oil fires

Between March and November of 1991, approximately 9000 workers from 43 different countries battled the burning oil wells in Kuwait. To document the exposure of persons in Kuwait during the oil well fires to volatile organic compounds (VOCs), we obtained samples of blood from 14 U.S. personnel in Kuwait City in May of 1991 (group I) and 40 American firefighters working in the oil fields in October of 1991 (group II). Concentrations of VOCs in group I and group II were compared with those of a random sample of 114 persons in the United States (reference group). The median concentrations of VOCs in group I were equal to or lower than those in the reference group. We found significant differences between the median concentrations of several VOCs in group 11 and the reference group. Median levels of ethylbenzene were about 10 times higher among group II than among the reference group (0.53 g/I vs 0.052 g/l). Median levels of benzene, m-/p-xylene, o-xylene, styrene, and toluene among group II were more than double those of the reference group. Although firefighters had higher median concentrations of VOCs than the reference group, those American personnel in Kuwait not involved in fighting the oil fires had concentrations of VOCs comparable to those in the reference group. Blood VOC measurements indicate a significant increase in exposure to VOCs in firefighters, but do not demonstrate this in personnel in Kuwait City.