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In the nineteenth century, the ideology of thrift was pervasive in didactic nonfiction, which encouraged readers to engage with frugality as an economic and moral stance. Samuel Smiles’ Thrift (1875) emphasized the importance of thrift to individuals and society, positioning it within the domestic setting. To understand how thrift was enacted in the nineteenth-century home, this article examines the ideology of thrift in cookbooks and recipes. These writings sought to practically enable readers to thriftily engage with food, making the most of ingredients creatively and frugally. While scholarly attention that highlights ideological discourses within cookbooks focuses on the cultural discussions authors include around recipes, little attention has been paid to how ideologies are present within recipes themselves. This article applies a close literary and structural reading to recipes, arguing that cookery was “the handmaid of thrift” and that recipes were textual tools, enabling readers to incorporate thrift into their lives.

Background Although renal cell carcinoma (RCC) is believed to have a strong hereditary component, there is a paucity of published guidelines for genetic risk assessment. A panel of experts was convened to gauge current opinions. Methods A North American multidisciplinary panel with expertise in hereditary RCC, including urologists, medical oncologists, clinical geneticists, genetic counselors, and patient advocates, was convened. Before the summit, a modified Delphi methodology was used to generate, review, and curate a set of consensus questions regarding RCC genetic risk assessment. Uniform consensus was defined as ≥85% agreement on particular questions. Results Thirty-three panelists, including urologists (n = 13), medical oncologists (n = 12), genetic counselors and clinical geneticists (n = 6), and patient advocates (n = 2), reviewed 53 curated consensus questions. Uniform consensus was achieved on 30 statements in specific areas that addressed for whom, what, when, and how genetic testing should be performed. Topics of consensus included the family history criteria, which should trigger further assessment, the need for risk assessment in those with bilateral or multifocal disease and/or specific histology, the utility of multigene panel testing, and acceptance of clinician-based counseling and testing by those who have experience with hereditary RCC. Conclusions In the first ever consensus panel on RCC genetic risk assessment, 30 consensus statements were reached. Areas that require further research and discussion were also identified, with a second future meeting planned. This consensus statement may provide further guidance for clinicians when considering RCC genetic risk assessment. Lay summary The contribution of germline genetics to the development of renal cell carcinoma (RCC) has long been recognized. However, there is a paucity of guidelines to define how and when genetic risk assessment should be performed for patients with known or suspected hereditary RCC. Without guidelines, clinicians struggle to define who requires further evaluation, when risk assessment or testing should be done, which genes should be considered, and how counseling and/or testing should be performed. To this end, a multidisciplinary panel of national experts was convened to gauge current opinion on genetic risk assessment in RCC and to enumerate a set of recommendations to guide clinicians when evaluating individuals with suspected hereditary kidney cancer.

INTRODUCTION AND OBJECTIVES:Renal cell carcinoma (RCC) caused by a known germline genetic alteration accounts for 5-8% of all RCC cases. Some patient populations may be enriched for more frequent g...

Purpose Approximately 5% to 8% of renal cell carcinoma (RCC) is hereditary. No guidelines exist for patient selection for RCC germline mutation testing. We evaluate how age of onset could indicate the need for germline mutation testing for detection of inherited forms of kidney cancer. Patients and Methods We analyzed the age distribution of RCC cases in the SEER-17 program and in our institutional hereditary kidney cancer population. The age distributions were compared by sex, race, histology, and hereditary cancer syndrome. Models were established to evaluate the specific age thresholds for genetic testing. Results The median age of patients with RCC in SEER-17 was 64 years, with the distribution closely approaching normalcy. Statistical differences were observed by race, sex, and subtype (P < .05). The bottom decile cutoff was ≤ 46 years of age and slightly differed by sex, race, and histology. The mean and median ages at presentation of 608 patients with hereditary kidney cancer were 39.3 years and 37 years, respectively. Although age varied by specific syndrome, 70% of these cases were found to lie at or below the bottom age decile. Modeling age-based genetic testing thresholds demonstrated that the 10th percentile maximized sensitivity and specificity. Conclusion Early age of onset might be a sign of hereditary RCC. Even in the absence of clinical manifestations and personal/family history, an age of onset of 46 years or younger should trigger consideration for genetic counseling/germline mutation testing and may serve as a useful cutoff when establishing genetic testing guidelines.

342 Background: Approximately 1-4% of renal cell carcinoma (RCC) is hereditary. No guidelines exist for patient selection for RCC genetic counseling. We evaluate how age of onset could guide referral for genetic testing. Methods: We analyzed the age distribution of RCC cases in the Surveillance and Epidemiology and End Results (SEER-17) program and from our institutional hereditary kidney cancer population. The age distributions were compared by sex, race, histology, and hereditary syndrome. Models were established to evaluate the specific age thresholds for genetic counseling. Results: The median age of RCC in SEER-17 was 64 years old with the distribution closely approaching normalcy. Statistical differences were observed by race, sex, and subtype (p < 0.05). The bottom decile overall was 46 years of age and slightly differed by sex, race, and histology. The mean and median age of 608 cases of hereditary kidney cancer was 39.3 and 37 years old. While age varied by specific syndrome, 70% of the hereditary cases lied below the bottom age decile. Modeling demonstrated that age alone could limit the number of patients for counseling and that the 10th percentile maximized sensitivity and specificity. Conclusions: Besides associated clinical manifestations and personal/family history, early age of onset in RCC could be a sign of a hereditary kidney cancer. In the absence of clinical signs, an age of onset ≤46 should trigger referral for genetic counseling and may serve as a useful cutoff when establishing genetic testing guidelines.

We recently described 5 unrelated children with autoimmune lymphoproliferative syndrome (ALPS) whom had dominant interfering mutations in Fas, a transmembrane protein of the tumor necrosis receptor family that mediates apoptosis of lymphoid cells. All 5 children with ALPS had heterozygous mutations in Fas; 4 of the mutant Fas molecules were shown to dominantly interfere with the induction of apoptosis by wild type Fas. The 5 children had massive non-mailgnant lymphadenopathy, autoimmune thrombocytopenia, hemolytic anemia, hyper-gammaglobulinemia as well as significant expansion of an unsusual subset of T cells that express T-cell receptors but neither CD4 nor CD8 (CD4-/CD8-). As seen in MRL/lpr mice, which also have autoimmune phenotype and elevated CD4-/CD8- T-cells, our patients demonstrated defective programmed cell death in response to Fas stimulation. A sixth ALPS patient has now been studied and found not to have a mutation in either allele of the Fas gene. Clinically this patient is indistinguishable from the previously characterized ALPS patients and like them has defective Fas-mediated apoptosis, in-vitro. Furthermore this patient's parents have normal Fas mediated apoptosis. The pattern of inheritance and our molecular charactreization of this ALPS patient are consistent with a recessive defect in an intracellular signalling molecule of the Fas apoptotic pathway and that both parents are carriers for this mutation.