WBR0082

Author	[[PageAuthor::Aditya Govindavarjhulla (Reviewed by Jad Al Danaf, Rim Halaby, M.D. [1], and Yazan Daaboul)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Microbiology
Sub Category	SubCategory::Reproductive
Prompt	[[Prompt::A 26-year-old G1P0 pregnant woman with no prenatal care presents to her physician's office at 8 weeks gestation for low-grade fever and cough that started on the same day. She just returned from a trip to Africa where she was exposed 4 days ago to her sick niece who had similar symptoms, and a maculopapular rash that "looked like measles but lasted for only 3 days". Further history reveals the patient recently immigrated to the United States and did not receive her childhood vaccinations. She does not smoke or drink alcohol. She does not take any medications and has no known allergies. Her blood pressure is 110/72 mmHg, heart rate is 80/min, temperature is 38.3 °C (100.9 °F), and respiratory rate is 16/min. Physical examination is remarkable for a widespread maculopapular rash on her face and trunk, along with palpable posterior auricular and occipital lymphadenopathy. Which of the following congenital disorders is most commonly associated with the infectious agent responsible for this patient's condition?]]
Answer A	AnswerA::Sensorineural deafness
Answer A Explanation	AnswerAExp::The most common manifestation of congenital rubella syndrome is sensorineural deafness. The risk of sensorineural deafness in infected patients is highest in the first trimester.
Answer B	AnswerB::Turner syndrome
Answer B Explanation	[[AnswerBExp::Turner syndrome is caused by a genetic disorder characterized by an absence of an X chromosome due to non-dysjunction. Patient's karyotype demonstrates a 44+XO pattern. Patients with Turner syndrome often have short stature when their syndrome is left untreated, neck and shoulder webbing, and ovarian dysgenesis. Turner syndrome is also associated with bicuspid aortic valve, shield chest, coarctation of the aorta (classically preductal), cystic hygroma, and horseshoe kidney. Turner syndrome is not classically associated with rubella infection during pregnancy.]]
Answer C	AnswerC::Down syndrome
Answer C Explanation	[[AnswerCExp::Down syndrome (trisomy 21) is the most common congenital disorder resulting in live births. It is characterized by intellectual disability, prominence of the epicanthal folds, flat facies, and single palmar crease. It is not classically associated with rubella infection during pregnancy.]]
Answer D	AnswerD::Tetralogy of Fallot
Answer D Explanation	[[AnswerDExp::Tetralogy of Fallot (ToF) is a congenital heart disease (CHD) caused by the anterosuperior displacement of the infundibular septum. It is characterized by the presence of 4 anomalies: Pulmonary stenosis, ventricular septal defect (VSD), right ventricular hypertrophy (RVH), and an overriding aorta. Although an associated between rubella and ToF have been described, ToF is not the most common association with rubella during pregnancy.]]
Answer E	AnswerE::Flipper-like limbs
Answer E Explanation	[[AnswerEExp::Phocomelia (also known as flipper-limbs or seal-limbs) is a teratogenic effect of thalidomide, a medication initially prescribed to pregnant women to treat pregnancy-associated nausea and vomiting. It is associated with phocomelia (abnormal limbs) and amelia (loss of limbs) in the fetuses.]]
Right Answer	RightAnswer::A
Explanation	[[Explanation::Rubella (German measles) is an enveloped single-stranded + RNA virus that belongs to the togavirus family. Infection by rubella is characterized by a 3-day maculopapular rash that starts on the face and often spreads to the trunk and extremities, resembling the rash in measles. Among children and adults who receive no rubella vaccination, the symptoms of rubella range from a very mild symptomatic course to development of fever, cough, coryza, arthralgia, conjunctivitis, and lymphadenopathy (especially posterior auricular and occipital). These symptoms are eventually followed by a maculopapular rash, as described in this patient, and often self-resolves within 3 days. Unvaccinated pregnant women infected with rubella are at risk of transmission of the infection to their fetuses, resulting in Congenital rubella syndrome (CRS). When rubella infection occurs during early pregnancy, serious complications, such as stillbirth and congenital cardiac defects, may result. The risks of congenital rubella syndrome decrease in the second trimester, and further in the third trimester. Congenital rubella syndrome may manifest as any/many of the following: Cataracts, congenital heart disease such as patent ductus arteriosus, congenital sensorineural deafness, and developmental delay. Educational Objective: In unvaccinated pregnant women, rubella infection may lead to congenital rubella syndrome in the newborn. The syndrome may manifest as sensorineural deafness, congenital cataract and other eye abnormalities, congenital heart defects such as patent ductus arteriosus, and developmental delay. References: Zgorniak-Nowosielska I, Zawilinska B, Szostek S. Rubella infection during pregnancy in the 1985-86 epidemic: follow-up after seven years. Eur J Epidemiol. 1996;12(3):303-8]]
Approved	Approved::Yes
Keyword	WBRKeyword::rubella, WBRKeyword::congenital rubella syndrome, WBRKeyword::pregnancy, WBRKeyword::fetal transmission, WBRKeyword::Microbiology, WBRKeyword::Virus, WBRKeyword::Virology, WBRKeyword::RNA virus
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