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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor=[[User:Gonzalo Romero|Gonzalo A. Romero, M.D.]] [mailto:gromero@wikidoc.org]
|QuestionAuthor=[[User:Gonzalo Romero|Gonzalo A. Romero, M.D.]], {{SSK}} (Reviewed by Serge Korjian)
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Anatomy, Behavioral Science/Psychiatry, Embryology, Pathology, Pharmacology
|MainCategory=Anatomy, Behavioral Science/Psychiatry, Embryology, Pathology, Pharmacology
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|MainCategory=Anatomy, Behavioral Science/Psychiatry, Embryology, Pathology, Pharmacology
|MainCategory=Anatomy, Behavioral Science/Psychiatry, Embryology, Pathology, Pharmacology
|SubCategory=Cardiology, General Principles
|SubCategory=Cardiology, General Principles
|Prompt=A 5-year-old boy is brought to the emergency department by his mother for difficulty breathing and cyanosis. Physical examination reveals cyanotic extremities and bounding jugular venous pulsations. Initial imaging reveals the cardiac anomaly shown below. The physician suspects a medication related congenital abnormality. What is the most likely presentation of the mother considering the drug she was presumably prescribed?
|Prompt=A 5-year-old boy is brought to the emergency department by his mother for difficulty breathing and cyanosis. Physical examination reveals cyanotic extremities and bounding jugular venous pulsations. Initial imaging reveals the cardiac anomaly shown below. The physician suspects a medication related congenital abnormality. What is the most likely presentation of the mother considering the drug she was likely prescribed?
[[File:WBR0514a.jpg|400px]]
[[File:WBR0514a.jpg|400px]]
|Explanation=The picture is showing an atrialized portion of the right ventricle. The opening of the tricuspid valve is displaced towards the apex of the right ventricle of the heart (congenital apical displacement of the tricuspid valve that typically causes significant tricuspid regurgitation). This cardiologic malformation is known as [[Ebstein anomaly]], which is highly associated with [[Lithium]] use during pregnancy. Lithium is used to treat [[bipolar disorder]], which is characterized by intense depressive symptoms alternated with manic episodes (characterized bydistractibility, irresponsibility, seeks pleasure without regard to consequences (hedonistic), grandiosity-inflated self-esteem, flight of ideas-racing thoughts, in goal-directed activity/psychomotor agitation, insomnia, talkativeness or pressured speech.
|Explanation=[[File:Ebstein_diagram.jpg|500px]]<br>
[[File:Ebstein_diagram.jpg|center|500px]]
Ebstein’s anomaly is a congenital heart disease characterized by abnormalities of the tricuspid valve, right atrium and right ventricle. Apical displacement of the tricuspid valve leaflets leads to an atrialized right ventricle that is often too small to circulate sufficient blood. Ebstein's anomaly is relatively rare with an incidence of 1 in 200000. Patient's with severe forms present with cyanosis, dyspnea, and failure to thrive. A majority of these patients have early presentation soon after PDA closure as they and may be dependent on the PDA for pulmonary blood flow. The development of Ebstein's anomaly has be associated with Lithium intake particularly in the first trimester. Lithium is mood stabilizer that remains a first line agent for the treatment of bipolar disorder. Bipolar disorder classically presents with manic episodes characterized by significant distractibility, irresponsibility, hedonistic behavior, grandiosity, racing thoughts, psychomotor agitation, insomnia, and talkativeness or pressured speech.
|AnswerA=Mild episodes of increased shopping, rapid speech and insomnia lasting at least 2 years
 
|AnswerAExp=[[Cyclothymia]] is a milder form of bipolar lasting at least for 2 years, which commonly is treated with psychotherapy
|AnswerA=Multiple mild episodes of increased impulsivity, rapid speech and insomnia for at least 2 years
|AnswerAExp=[[Cyclothymia]] is a milder form of bipolar lasting for at least for 2 years. Treatment of cyclothemia is commonly with psychotherapy.
|AnswerB=Hypersomnia, overeating, and mood reactivity
|AnswerB=Hypersomnia, overeating, and mood reactivity
|AnswerBExp=This describes [[atypical depression]], which is typically treated with MAO inhibitors
|AnswerBExp=These are the classical symptoms of [[atypical depression]]. Treatment typically involves MAO inhibitors and psychotherapy.
|AnswerC=Alternating intense sad symptoms with episodes of increased shopping, rapid speech and insomnia intense in nature
|AnswerC=Alternating symptoms of depression with episodes of increased impulsivity, rapid speech and insomnia
|AnswerCExp=See overall explanation.
|AnswerCExp=Bipolar disorder classically presents with manic episodes characterized by significant distractibility, irresponsibility, hedonistic behavior, grandiosity, racing thoughts, psychomotor agitation, insomnia, and talkativeness or pressured speech.
 
|AnswerD=Recurring intrusive thoughts, feelings, or sensations that cause severe distress; relieved partly by repetitive actions
|AnswerD=Recurring intrusive thoughts, feelings, or sensations that cause severe distress; relieved partly by repetitive actions
|AnswerDExp=This refers to [[OCD]] (Obsessive compulsive personality disorder) which is commonly treated with [[SSRIs]]
|AnswerDExp=These are the classical symptoms of [[obsessive compulsive disorder]] (OCD) which is commonly treated with [[SSRIs]]
|AnswerE=Excessive and unreasonable fear that interferes with normal function
|AnswerE=Excessive and unreasonable anxiety that interferes with normal function
|AnswerEExp=This refers to specific [[phobia]], which is treated with SSRIs
|AnswerEExp=Generalized anxiety disorder is characterized by excessive and unreasonable anxiety that interferes with normal function. It is treated with SSRIs.
|EducationalObjectives=Bipolar disorder is treated with lithium, a mood stabilizer, which is associated with Ebstein anomaly, an atrialization of the right ventricle.
|EducationalObjectives=Lithium is mood stabilizer used for the treatment of bipolar disorder that is associated with the devlopment of Ebstein's anomaly if administered to women in the first trimester. Bipolar disorder classically presents with manic episodes characterized by significant distractibility, irresponsibility, hedonistic behavior, grandiosity, racing thoughts, psychomotor agitation, insomnia, and talkativeness or pressured speech.
 
|References=Romfh A, Pluchinotta FR, Porayette P, Valente AM, Sanders SP. Congenital Heart Defects in Adults : A Field Guide for Cardiologists. J Clin Exp Cardiolog. 2012;(Suppl 8) '''(Image)'''<br>
|References=Romfh A, Pluchinotta FR, Porayette P, Valente AM, Sanders SP. Congenital Heart Defects in Adults : A Field Guide for Cardiologists. J Clin Exp Cardiolog. 2012;(Suppl 8) '''(Image)'''<br>
First Aid 2014 p. 518
First Aid 2014 p. 518

Revision as of 22:19, 6 November 2014

 
Author [[PageAuthor::Gonzalo A. Romero, M.D., Serge Korjian M.D. (Reviewed by Serge Korjian)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Anatomy, MainCategory::Behavioral Science/Psychiatry, MainCategory::Embryology, MainCategory::Pathology, MainCategory::Pharmacology
Sub Category SubCategory::Cardiology, SubCategory::General Principles
Prompt [[Prompt::A 5-year-old boy is brought to the emergency department by his mother for difficulty breathing and cyanosis. Physical examination reveals cyanotic extremities and bounding jugular venous pulsations. Initial imaging reveals the cardiac anomaly shown below. The physician suspects a medication related congenital abnormality. What is the most likely presentation of the mother considering the drug she was likely prescribed?

]]

Answer A AnswerA::Multiple mild episodes of increased impulsivity, rapid speech and insomnia for at least 2 years
Answer A Explanation [[AnswerAExp::Cyclothymia is a milder form of bipolar lasting for at least for 2 years. Treatment of cyclothemia is commonly with psychotherapy.]]
Answer B AnswerB::Hypersomnia, overeating, and mood reactivity
Answer B Explanation [[AnswerBExp::These are the classical symptoms of atypical depression. Treatment typically involves MAO inhibitors and psychotherapy.]]
Answer C AnswerC::Alternating symptoms of depression with episodes of increased impulsivity, rapid speech and insomnia
Answer C Explanation [[AnswerCExp::Bipolar disorder classically presents with manic episodes characterized by significant distractibility, irresponsibility, hedonistic behavior, grandiosity, racing thoughts, psychomotor agitation, insomnia, and talkativeness or pressured speech.]]
Answer D AnswerD::Recurring intrusive thoughts, feelings, or sensations that cause severe distress; relieved partly by repetitive actions
Answer D Explanation [[AnswerDExp::These are the classical symptoms of obsessive compulsive disorder (OCD) which is commonly treated with SSRIs]]
Answer E AnswerE::Excessive and unreasonable anxiety that interferes with normal function
Answer E Explanation AnswerEExp::Generalized anxiety disorder is characterized by excessive and unreasonable anxiety that interferes with normal function. It is treated with SSRIs.
Right Answer RightAnswer::C
Explanation [[Explanation::

Ebstein’s anomaly is a congenital heart disease characterized by abnormalities of the tricuspid valve, right atrium and right ventricle. Apical displacement of the tricuspid valve leaflets leads to an atrialized right ventricle that is often too small to circulate sufficient blood. Ebstein's anomaly is relatively rare with an incidence of 1 in 200000. Patient's with severe forms present with cyanosis, dyspnea, and failure to thrive. A majority of these patients have early presentation soon after PDA closure as they and may be dependent on the PDA for pulmonary blood flow. The development of Ebstein's anomaly has be associated with Lithium intake particularly in the first trimester. Lithium is mood stabilizer that remains a first line agent for the treatment of bipolar disorder. Bipolar disorder classically presents with manic episodes characterized by significant distractibility, irresponsibility, hedonistic behavior, grandiosity, racing thoughts, psychomotor agitation, insomnia, and talkativeness or pressured speech.
Educational Objective: Lithium is mood stabilizer used for the treatment of bipolar disorder that is associated with the devlopment of Ebstein's anomaly if administered to women in the first trimester. Bipolar disorder classically presents with manic episodes characterized by significant distractibility, irresponsibility, hedonistic behavior, grandiosity, racing thoughts, psychomotor agitation, insomnia, and talkativeness or pressured speech.
References: Romfh A, Pluchinotta FR, Porayette P, Valente AM, Sanders SP. Congenital Heart Defects in Adults : A Field Guide for Cardiologists. J Clin Exp Cardiolog. 2012;(Suppl 8) (Image)
First Aid 2014 p. 518]]

Approved Approved::Yes
Keyword WBRKeyword::Ebstein anomaly, WBRKeyword::Lithium, WBRKeyword::Bipolar disorder
Linked Question Linked::
Order in Linked Questions LinkedOrder::