Familial Cancer最新文献

{"title":"Haplotype analysis detects MLH1 founder variant in Indian Lynch syndrome patient cohort.","authors":"Harsh Sheth, Jyoti Sadhwani, Abhinav Jain, S G Thenral, Vedam Ramprasad, D Timothy Bishop","doi":"10.1007/s10689-024-00436-6","DOIUrl":"https://doi.org/10.1007/s10689-024-00436-6","url":null,"abstract":"<p><p>Lynch syndrome (LS) is an autosomal dominant hereditary cancer predisposition syndrome whereby the lifetime risk of developing gastrointestinal and genitourinary cancers rises by to over 50%. It is caused by heterozygous variants in the DNA mismatch repair genes- MLH1, MSH2, MSH6 and PMS2, with the majority detected in MLH1 and MSH2. Recurrently observed LS-associated variants in apparently unrelated individuals have either arisen de novo in different families due to mutation hotspots or are inherited from a common ancestor (founder) that lived several generations back. Testing for founder variants can facilitate molecular diagnosis of LS more efficiently and cost effectively than screening for all possible variants in the MMR genes. Here, we report a study of the missense variant c.306G > T in the MLH1 gene, the first potential founder variant identified in LS patients of Indian ethnicity. Haplotype analysis consisting of 25 LS carriers with the MLH1 c.306G > T variant and 100 healthy controls confirmed a shared haplotype in cases spanning a 27.8 kb region encompassing the c.306G > T variant (𝝌² = 96.418; p = < 0.0001). Age of variant analysis suggests the variant to have arisen in the population approximately 800 years (95% CI: 670-934 years) ago. Furthermore, it is estimated that c.306G > T variant is likely to be observed in 6.4% of all LS patients of Indian ethnicity. These findings have important implications for genetic counselling and molecular diagnosis of Lynch syndrome.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"13"},"PeriodicalIF":1.8,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142863791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bilateral familial retinoblastoma diagnosed via optical coherence tomography following a normal funduscopic exam.","authors":"Mitchell T Allphin, Aparna Ramasubramanian","doi":"10.1007/s10689-024-00424-w","DOIUrl":"10.1007/s10689-024-00424-w","url":null,"abstract":"<p><p>Retinoblastoma (RB) is the most common intraocular malignancy in children, and patients with family history of retinoblastoma are at high risk of disease. While intensive screening programs have led to earlier diagnosis and higher rates of globe salvage, visual outcomes have not improved. Handheld optical coherence tomography (HH-OCT) is a non-invasive imaging modality that can be utilized for screening, diagnosis, and monitoring of Familial RB. We present a case of a patient who was found to have bilateral Familial RB with HH-OCT after having a normal fundoscopic exam. This case demonstrates the utility of HH-OCT imaging and suggests that further research is needed to see whether screening guidelines should require OCT imaging during screening.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"12"},"PeriodicalIF":1.8,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142828181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recurrent paraneoplastic nephrotic syndrome; insights from a Lynch syndrome patient with multiple malignancies.","authors":"Myrthe A de Jong, Marije Slingerland, Lukas J A C Hawinkels, Maartje Nielsen, Augustinus S L P Crobach, Eveline S M de Jonge-Muller, Antonius J Rabelink, Alexandra M J Langers","doi":"10.1007/s10689-024-00435-7","DOIUrl":"10.1007/s10689-024-00435-7","url":null,"abstract":"<p><p>Nephrotic syndrome is a common clinical presentation of glomerulopathy. A glomerulopathy as a paraneoplastic manifestation caused by underlying malignancy is rare. In patients with a solid tumor, membranous nephropathy is the most frequent paraneoplastic glomerulopathy. We present a case of recurrent paraneoplastic nephrotic syndrome caused by minimal change disease in a patient with Lynch syndrome. Over the years, a decrease in creatinine clearance and nephrotic-range proteinuria repeatedly functioned as a warning signal for underlying malignancies; consecutively, a colon adenocarcinoma, renal cell carcinoma and gastric adenocarcinoma were diagnosed. After treatment of the malignancies the nephrotic syndrome resolved without immunosuppressive therapy. Our patient also developed a primary lung carcinoma thrice, which did not cause an exacerbation of the minimal change disease. To further elucidate the mechanism behind the development of this phenomenon, we performed immunohistochemical analysis for vascular endothelial growth factor (VEGF) on the different tumor specimens. We found a high VEGF expression in the gastro-intestinal tumors, whereas the VEGF expression in the lung tumors was low, suggesting an association between VEGF expression and the development of paraneoplastic minimal change disease. This case report not only underlines the importance of considering a malignancy as a cause for (recurrent) nephrotic syndrome, especially in patients with an increased risk of developing malignancies like Lynch syndrome patients, but also suggests a role for VEGF in the pathogenesis of paraneoplastic minimal change disease.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"11"},"PeriodicalIF":1.8,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142767641","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sarcomas arising in MEN1 patients: demonstrating LOH of the MEN1 locus and loss of menin expression.","authors":"Rachel S van Leeuwaarde, Thorvardur R Halfdanarson, Shwetha M Sudhakar, Ruud W J Meijers, Andrew L Folpe, Lodewijk A A Brosens","doi":"10.1007/s10689-024-00433-9","DOIUrl":"https://doi.org/10.1007/s10689-024-00433-9","url":null,"abstract":"<p><p>Multiple endocrine neoplasia type 1 (MEN1) is a hereditary tumor syndrome characterized by endocrine tumors, typically from parathyroid, pancreatic, or anterior pituitary origin. In addition, benign cutaneous soft tissue tumors are prevalent in MEN1 patients. Although sarcomas have been reported in MEN1 patients it is unclear if these tumors should be considered as part of the MEN1 syndrome. Here, five patients with a MEN1 syndrome and a sarcoma are described. In all five sarcomas loss of heterozygosity of the MEN1 gene and loss of expression of menin are shown, suggesting that sarcomas may be a phenotypic expression of MEN1 syndrome.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"10"},"PeriodicalIF":1.8,"publicationDate":"2024-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142750249","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A family-based approach to cascade genetic testing in a pediatric cancer genetics clinic.","authors":"Rida Haider, Lauren Desrosiers-Battu, Sarah Scollon, Pawel Stankiewicz, Philip J Lupo, Sharon E Plon","doi":"10.1007/s10689-024-00434-8","DOIUrl":"10.1007/s10689-024-00434-8","url":null,"abstract":"<p><p>Hereditary cancer predisposition disorders account for up to 10% of all pediatric cancers. Genetic counseling for families of the proband includes risk assessment and recommendations for cascade genetic testing for parents and siblings, but there is no standardized method for cascade testing in place resulting in variability in how clinics approach cascade genetic testing. We explored the uptake and outcomes associated with a family-based approach to cascade testing, for non-syndromic cancer predisposition disorders, at a pediatric cancer genetics clinic serving an ethnically diverse patient population. A retrospective chart review was conducted to evaluate test uptake in the parents and siblings of 106 pediatric probands. The study included 99 mothers, 97 fathers, 116 full siblings, and 53 half siblings who were recommended testing due to genetic risk. Of these relatives, 156 (43%) had documentation of completed cascade testing within twenty-four months after the proband's result disclosure. Completion of cascade testing varied by the type of family member and degree of relatedness. 41% of mothers (41/99) were tested in comparison to 26% of fathers (26/97) and 70.6% of full siblings (82/116) were tested compared to 13.2% of half siblings (7/53). Statistical analysis using chi-squared tests revealed that siblings were more likely to have completed testing than parents (p < 0.001). Furthermore, amongst parents, mothers were more likely to complete testing than fathers (p = 0.03) and amongst siblings, full siblings were more likely to complete testing than half siblings (< 0.001). The proband's age (p = 0.008), parents' preferred language (p = 0.002), and interpreter use during visit (p = 0.004) were the factors associated with differences in test uptake amongst siblings, whereas the proband's race/ethnicity (p = 0.019) was the only factor associated with differences in test uptake amongst parents. The most common barriers noted in charts for lack of test completion included country of residence, lack of insurance, and loss to follow-up. In conclusion, we found that test uptake differed significantly among relatives of a proband with siblings being more likely to test than parents. We also found differences in the demographic and clinical factors associated with test uptake in parents and siblings. Future studies need to validate these differences and further explore the underlying cause of variation in test uptake among relatives.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"8"},"PeriodicalIF":1.8,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142675505","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cascade genetic testing in hereditary cancer: exploring the boundaries of the Italian legal framework.","authors":"Liliana Varesco, Francesco Di Tano, Juri Monducci, Stefania Sciallero, Daniela Turchetti, Claudia Bighin, Giulia Buzzatti, Irene Giannubilo, Lucia Trevisan, Linda Battistuzzi","doi":"10.1007/s10689-024-00430-y","DOIUrl":"10.1007/s10689-024-00430-y","url":null,"abstract":"<p><p>Despite its clinical value, cascade genetic testing (CGT) in hereditary cancer syndromes remains underutilized for a number of reasons, including ineffective family communication of genetic risk information. Therefore, alternative strategies are being explored to improve CGT uptake rates; one such strategy is direct contact with at-risk relatives by healthcare professionals with proband consent. It is unclear how Italian laws and regulations pertaining to CGT-including the EU General Data Protection Regulation (GDPR)-should be understood and implemented in the context of such alternative strategies. The authors constructed a hypothetical case about CGT, reviewed laws and regulations on informed consent, privacy, and the right not to know, and analyzed how those laws and regulations might apply to different communicative strategies relevant to the case and aimed at supporting CGT. A constitutionally consistent reading of Italian law and of the GDPR, an integral part of the Italian privacy framework, suggests that multiple communicative approaches may be legally permissible in Italy to support the CGT process. This includes direct contact by healthcare professionals with proband consent, provided certain conditions are met. Understanding the effectiveness of such approaches in improving CGT uptake will require further research efforts.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"9"},"PeriodicalIF":1.8,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142675509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hereditary breast and ovarian cancer genetic testing in unselected patients: example of private supplementation of public healthcare service.","authors":"Francesca Fiorentino, Giovanni Innella, Federica Balducci, Laura Marullo, Giulia Lanzoni, Sara Miccoli, Laura Cardarelli, Daniela Turchetti, Sergio Tempesta","doi":"10.1007/s10689-024-00426-8","DOIUrl":"10.1007/s10689-024-00426-8","url":null,"abstract":"<p><p>In the Emilia-Romagna region (Northern Italy), the identification and management of women at familial/hereditary risk of breast and ovarian cancer is guided by a well-established regional protocol. Here we report the results of the experience of private supplementation of public healthcare service in offering the possibility to undergo BRCA1/2 testing and/or multigene panel testing (MGPT) within a well-defined pathway to women unfulfilling regional criteria. Out of 177 patients referred to our center who underwent BRCA1/2 testing, 175 tested negative while two (1.1%) resulted carriers of pathogenic variants in BRCA2; 69 patients also underwent MGPT, and in four cases (5.8%) a pathogenic variant were found (two in ATM and one in CHEK2 and RAD51C, respectively). Overall, this private supplementation of territorial public healthcare system has made it possible to confirm the validity of regional criteria for genetic testing access (concordance: 98.9%), but also to identify carriers of pathogenic variants of BRCA1/2 that would have escaped regional protocol, to support the effectiveness of MGPT for the identification of rare cases (not BRCA) at mild/high risk, and to provide reassurance to women who were found to be non-carriers of pathogenic variants, who may benefit from a more accurate assessment of their risk.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"7"},"PeriodicalIF":1.8,"publicationDate":"2024-11-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142675510","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The genetic landscape of Lynch syndrome in the Israeli population.","authors":"Aasem Abu Shtaya, Sofia Naftaly Nathan, Inbal Kedar, Eitan Friedman, Elizabeth Half, Gabi Lidzbarsky, Gili Reznick Levi, Ido Laish, Lior Katz, Lily Bazak, Lilach Peled Peretz, Lina Basel Salmon, Liza Douiev, Marina Lifshitc Kalis, Menachem Schechter, Michal Barzily-Rokni, Nadra Nasser Samra, Naim Abu-Freha, Ofir Hagari-Bechar, Ori Segol, Samar Mattar, Sarit Farage Barhom, Shikma Mordechai, Shiri Shkedi Rafid, Stavit A Shalev, Tamar Peretz-Yablonski, Zohar Levi, Revital Bruchim, Chana Vinkler, Rinat Bernstein-Molho, Sari Lieberman, Yael Goldberg","doi":"10.1007/s10689-024-00432-w","DOIUrl":"10.1007/s10689-024-00432-w","url":null,"abstract":"<p><p>Deciphering the spectrum and founder disease-causing variants (DCVs) in specific populations can shape and facilitate the diagnostic process of Lynch Syndrome (LS). The aim of this report was to comprehensively update on the genetic landscape of LS in the ethnically diverse Israeli-Jewish population. The cohort included 1080 carriers from 588 families; some from underrepresented, understudied Israeli ethnic groups recruited from 8 genetic institutes and high-risk clinics throughout the country. Variant classification was performed according to the American College of Medical Genetics criteria. A total of 157 DCVs were identified, 12 are reported here for the first time, and 9 reclassified. MSH2 DCVs were identified in 286 families (49%). Most DCVs (125/157, 80%) were noted in one or two families only. Sixteen DCVs, each detected in ≥ 5 families, and accounted for LS in 378/588 (64%) families. Constitutional mismatch repair deficiency (CMMRD) was diagnosed in 7 families. Twenty-five carriers (2.3%) had an additional DCV or risk alleles in another cancer susceptibility gene. In conclusion, MMR gene variant distribution in Israel is diverse. MSH2 is most commonly mutated due to founder DCVs. Though the 16 prevalent LS-associated DCVs were frequently detected in our cohort, none of them is frequently reported in the general population. These data should facilitate variant interpretation, spouse and cascade testing.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"6"},"PeriodicalIF":1.8,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11567984/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142638578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mainstreaming cancer genetics: feasibility of an advanced nurse practitioner-led service diagnosing Lynch syndrome from colorectal cancer in Ireland.","authors":"Mechelle Loughrey, Lauren V O'Connell, Lynda McSorley, Sean Martin, Ann Hanly, Des C Winter, Ian M Frayling, Kieran Sheahan, Rory Kennelly","doi":"10.1007/s10689-024-00427-7","DOIUrl":"https://doi.org/10.1007/s10689-024-00427-7","url":null,"abstract":"<p><p>Colorectal cancer (CRC) is a common cancer in Ireland. Of all CRCs, 2-4% are attributable to Lynch Syndrome (LS), the most common CRC predisposition syndrome. LS is caused by constitutional pathogenic variants (PVs) affecting mismatch repair (MMR) genes with resultant MMR protein deficiency (dMMR). Screening of all CRCs with MMR immunohistochemistry (IHC) testing is advocated to increase the detection of LS. However, successful implementation requires appropriate downstream management. In Ireland the traditional pathway involves referral to cancer genetics services to assess eligibility for genetic testing. Cancer genetics services in Ireland face many challenges in providing uniform access to timely healthcare with current wait times for assessment in excess of 1 year. An increasingly adopted pathway is that of mainstreaming, whereby genetic testing is managed locally by a multidisciplinary team member. Our institution therefore implemented an Advanced Nurse Practitioner (ANP)-led service with responsibility for the LS Diagnostic Pathway and mainstream genetic testing. Data was extracted from a prospectively maintained database of all newly diagnosed CRC patients discussed at our institutions CRC multidisciplinary meeting (MDM) between January 1st, 2023, and May 31st, 2024. MMR IHC testing was performed in 97.9% of the 385 patients diagnosed with CRC. The median time from histological confirmation of CRC to the availability of the MMR IHC report was 6 days. All 51 patients (100%) who required sequential tumor testing underwent BRAF V600 ± MLH1 promoter methylation testing. Additionally, 100% of the 14 patients eligible for mainstream genetic testing were referred to the ANP-led genetics service. The median time from the initial MDM discussion to the initiation of genetic testing was 69 days, while the median time from testing to the availability of results was 19 days. Patients received their results within a median of 21 days. MMR IHC testing increases the detection of LS through identification of dMMR tumours. Successful downstream delivery of clinical services, however, requires appropriate subsequent management, in a resource-limited environment. Our institutional experience demonstrates the feasibility, efficiency, and effectiveness of an ANP-led mainstreaming model of care for hereditary colorectal cancer.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"2"},"PeriodicalIF":1.8,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142638574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Germline pathogenic variants in RNF43 in patients with and without serrated polyposis syndrome.","authors":"Heidi Hesselø Brinch, Anna Byrjalsen, Zuzana Lohse, Andreas Ørslev Rasmussen, John Gásdal Karstensen, Britta Schlott Kristiansen, Anne Marie Jelsig","doi":"10.1007/s10689-024-00428-6","DOIUrl":"10.1007/s10689-024-00428-6","url":null,"abstract":"<p><p>Serrated Polyposis Syndrome (SPS) is characterized by multiple and/or large serrated polyps in the colon and an increased risk of colorectal cancer (CRC). The etiology is largely unknown, but in a subset of patients with SPS, monoallelic pathogenic variants in RNF43 are detected. To date, however, the penetrance and phenotypic spectrum of patients carrying pathogenic variants (PV) in RNF43 are poorly described. We present eight patients both with and without serrated polyps from four unrelated families with likely pathogenic variants (LPV) in RNF43 and compare the results to current literature. The patients were referred to genetic counseling due to suspicion of hereditary cancer. They underwent genetic testing with custom NGS gene panels including RNF43 as part of a routine genetic work-up. Three LPVs, one multi-exon deletion and two nonsense variants, were detected in four families. Family I had a history of CRC and serrated polyps, but in the three other families (II‒IV) there was no history of CRC or serrated polyps. Colonoscopies in the probands of these families did not reveal any serrated polyps and/or CRC despite some of them being relatively old. Our findings suggest that the penetrance of RNF43-related disease is much lower than previously thought, and raise questions about the connection between RNF43 and disease. The results highlight the complexity of genetic counseling in RNF43 positive families- particularly in families without polyposis. Further research is needed to elucidate the role of RNF43 in the risk of SPS and CRC.</p>","PeriodicalId":12336,"journal":{"name":"Familial Cancer","volume":"24 1","pages":"3"},"PeriodicalIF":1.8,"publicationDate":"2024-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11568000/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142638572","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}