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|Prompt=A 20-year-old woman is contacted regarding an abnormal pap smear on her last annual physical. The smear showed dysplasia of squamous cells from the surface of the cervix. The degree of dysplasia is deemed CIN1. The molecular mechanism of oncogenesis of the causal organism is similar to the defect underlying which disease? | |Prompt=A 20-year-old woman is contacted regarding an abnormal pap smear on her last annual physical. The smear showed dysplasia of squamous cells from the surface of the cervix. The degree of dysplasia is deemed CIN1. The molecular mechanism of oncogenesis of the causal organism is similar to the defect underlying which disease? | ||
|Explanation=This young woman has an abnormal pap smear as a result of infection with Human Papilloma Virus(HPV). HPV causes cervical dysplasia when the E6 and E7 proteins bind tumor suppressor proteins within the cell. E6 mediates the degradation of the tumor suppressor p53, which is mutated in Li-Fraumeni syndrome. E7 binds to and inactivates Rb which is mutated in familial retinoblastoma. Rb protein normally acts as a sensor of DNA damage and cellular stress to stop cell cycle progression at the R (restriction) point between G1 and S phase. Nonfunctional Rb leads to uninhibited progression through the cell cycle. | |Explanation=This young woman has an abnormal pap smear as a result of infection with Human Papilloma Virus(HPV). HPV causes cervical dysplasia when the E6 and E7 proteins bind tumor suppressor proteins within the cell. E6 mediates the degradation of the tumor suppressor p53, which is mutated in Li-Fraumeni syndrome. E7 binds to and inactivates Rb which is mutated in familial retinoblastoma. Rb protein normally acts as a sensor of DNA damage and cellular stress to stop cell cycle progression at the R (restriction) point between G1 and S phase. Nonfunctional Rb leads to uninhibited progression through the cell cycle. | ||
|AnswerA=Familial retinoblastoma | |AnswerA=Familial retinoblastoma | ||
|AnswerAExp=Familial retinoblastoma is caused by mutations in the RB1 gene. HPV infection causes cervical cell dysplasia by expressing the E7 protein that sequesters and tags the RB1 gene for degradation. | |AnswerAExp=Familial retinoblastoma is caused by mutations in the RB1 gene. HPV infection causes cervical cell dysplasia by expressing the E7 protein that sequesters and tags the RB1 gene for degradation. | ||
|AnswerB=Familial adenomatous polyposis | |AnswerB=Familial adenomatous polyposis | ||
|AnswerBExp=Familial adenomatous polyposis (FAP) is caused by mutations in the APC gene. These patients develop thousands of polyps in their colons by the third decade of life. | |AnswerBExp=Familial adenomatous polyposis (FAP) is caused by mutations in the APC gene. These patients develop thousands of polyps in their colons by the third decade of life. | ||
|AnswerC=Neurofibromatosis type I | |AnswerC=Neurofibromatosis type I | ||
|AnswerCExp=[[Neurofibromatosis]] is caused by mutations in the NF1 gene. | |AnswerCExp=[[Neurofibromatosis]] is caused by mutations in the NF1 gene. Patients have café-au-lait spots and cutaneous neurofibromas. | ||
|AnswerD=Hereditary breast-ovarian cancer syndrome | |AnswerD=Hereditary breast-ovarian cancer syndrome | ||
|AnswerDExp=Hereditary breast-ovarian cancer syndrome (HBOC) is caused by mutations in the BRCA1 and BRCA2 genes. Female patients with these mutations have a 40%-80% lifetime risk of breast cancer and an 11%-40% lifetime risk of ovarian cancer. | |AnswerDExp=Hereditary breast-ovarian cancer syndrome (HBOC) is caused by mutations in the BRCA1 and BRCA2 genes. Female patients with these mutations have a 40%-80% lifetime risk of breast cancer and an 11%-40% lifetime risk of ovarian cancer. | ||
|AnswerE=Cowden’s sydrome | |AnswerE=Cowden’s sydrome | ||
|AnswerEExp=[[Cowden’s syndrome]] is caused by mutations in the PTEN gene. | |AnswerEExp=[[Cowden’s syndrome]] is caused by mutations in the PTEN gene. | ||
|EducationalObjectives=HPV causes oncogenesis when E7 binds to and inactivates Rb which is mutated in familial retinoblastoma. | |EducationalObjectives=HPV causes oncogenesis when E7 binds to and inactivates Rb which is mutated in familial retinoblastoma. | ||
|References=First Aid 2014 page 579 | |References=First Aid 2014 page 579 | ||
First Aid 2012 page 543 | First Aid 2012 page 543 | ||
|RightAnswer=A | |RightAnswer=A | ||
|WBRKeyword=Microbiology, Cervical intraepithelial neoplasia, Cervix, Cervical cancer, Cervical, Virus, Viruses, DNA virus, DNA viruses, HPV, Cancer, Retinoblastoma, Tumor suppressor, | |WBRKeyword=Microbiology, Cervical intraepithelial neoplasia, Cervix, Cervical cancer, Cervical, Virus, Viruses, DNA virus, DNA viruses, HPV, Cancer, Retinoblastoma, Tumor suppressor, | ||
|Approved=Yes | |Approved=Yes | ||
}} | }} | ||
Revision as of 22:28, 15 March 2014
| Author | PageAuthor::William J Gibson |
|---|---|
| Exam Type | ExamType::USMLE Step 1 |
| Main Category | MainCategory::Genetics, MainCategory::Microbiology |
| Sub Category | SubCategory::Oncology, SubCategory::General Principles, SubCategory::Infectious Disease |
| Prompt | [[Prompt::A 20-year-old woman is contacted regarding an abnormal pap smear on her last annual physical. The smear showed dysplasia of squamous cells from the surface of the cervix. The degree of dysplasia is deemed CIN1. The molecular mechanism of oncogenesis of the causal organism is similar to the defect underlying which disease?]] |
| Answer A | AnswerA::Familial retinoblastoma |
| Answer A Explanation | AnswerAExp::Familial retinoblastoma is caused by mutations in the RB1 gene. HPV infection causes cervical cell dysplasia by expressing the E7 protein that sequesters and tags the RB1 gene for degradation. |
| Answer B | AnswerB::Familial adenomatous polyposis |
| Answer B Explanation | AnswerBExp::Familial adenomatous polyposis (FAP) is caused by mutations in the APC gene. These patients develop thousands of polyps in their colons by the third decade of life. |
| Answer C | AnswerC::Neurofibromatosis type I |
| Answer C Explanation | [[AnswerCExp::Neurofibromatosis is caused by mutations in the NF1 gene. Patients have café-au-lait spots and cutaneous neurofibromas.]] |
| Answer D | AnswerD::Hereditary breast-ovarian cancer syndrome |
| Answer D Explanation | AnswerDExp::Hereditary breast-ovarian cancer syndrome (HBOC) is caused by mutations in the BRCA1 and BRCA2 genes. Female patients with these mutations have a 40%-80% lifetime risk of breast cancer and an 11%-40% lifetime risk of ovarian cancer. |
| Answer E | AnswerE::Cowden’s sydrome |
| Answer E Explanation | [[AnswerEExp::Cowden’s syndrome is caused by mutations in the PTEN gene.]] |
| Right Answer | RightAnswer::A |
| Explanation | [[Explanation::This young woman has an abnormal pap smear as a result of infection with Human Papilloma Virus(HPV). HPV causes cervical dysplasia when the E6 and E7 proteins bind tumor suppressor proteins within the cell. E6 mediates the degradation of the tumor suppressor p53, which is mutated in Li-Fraumeni syndrome. E7 binds to and inactivates Rb which is mutated in familial retinoblastoma. Rb protein normally acts as a sensor of DNA damage and cellular stress to stop cell cycle progression at the R (restriction) point between G1 and S phase. Nonfunctional Rb leads to uninhibited progression through the cell cycle. Educational Objective: HPV causes oncogenesis when E7 binds to and inactivates Rb which is mutated in familial retinoblastoma. |
| Approved | Approved::Yes |
| Keyword | WBRKeyword::Microbiology, WBRKeyword::Cervical intraepithelial neoplasia, WBRKeyword::Cervix, WBRKeyword::Cervical cancer, WBRKeyword::Cervical, WBRKeyword::Virus, WBRKeyword::Viruses, WBRKeyword::DNA virus, WBRKeyword::DNA viruses, WBRKeyword::HPV, WBRKeyword::Cancer, WBRKeyword::Retinoblastoma, WBRKeyword::Tumor suppressor |
| Linked Question | Linked:: |
| Order in Linked Questions | LinkedOrder:: |