Module 3 Flashcards

1
Q

A 15-year-old girl is brought to the clinic by her mother due to absent menstruation. She worryingly states that her daughter has not started menstruating despite both her elder sisters “hitting puberty before the age of 13.” The patient says that she feels weird that all her friends in school go through periods and think that something might be wrong with her body. She denies any weight loss, anorexia, abdominal or pelvic pain, and sleep problems. She adds that she exercises modestly every day and mostly eats green vegetables and lean meat. Her vital signs are within normal limits, and her body mass index is 25 kg/m2. Examination shows well-developed secondary sexual characteristics with breasts at Tanner stage IV, and pubic hair at Tanner stage V. She is not sexually active. What is the best next step in the management of this patient?

  1. Reassure and follow up in 6 months
  2. Serum FSH and LH levels
  3. Pelvic ultrasound
  4. Chromosomal analysis
A
  1. Reassure and follow up in 6 months

POINTS

This patient does not fall into the diagnosis of primary amenorrhea.
Primary amenorrhea is defined as the failure to reach menarche. Evaluation should be undertaken if there are no sexual characteristics by 13 years of age, if menarche has not occurred five years after initial breast development, or if the patient is 15 years or older.
This patient has tanner stage IV and tanner stage V secondary sexual characteristics; therefore, waiting 6 more months before making a diagnosis of primary amenorrhea is advised.
Primary amenorrhea can occur because of central or peripheral causes. Investigations such as chromosomal analysis, pelvic ultrasound, and FSH levels can help in the diagnosis.

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2
Q

A 6-year-old male is brought to the clinic for a well-child visit. On exploring his progress in school, the parents reveal that he is struggling to keep up with his peers in class, and they are afraid that he will be held back. During the evaluation, the clinician notes that the child has no mastery of conservation and inductive reasoning. At which of the following ages should a child normally develop these functions?

  1. 4 to 6
  2. 5 to 8
  3. 7 to 11
  4. 10 to 13
A
  1. 7 to 11

POINTS

A child can focus on concrete operations during middle childhood.
This age corresponds to the “concrete operational” stage defined by Piaget.
At this stage, the child can use concepts of conservation and inductive reasoning.
During 7-11 years of age, the child is still not capable of using abstract thoughts.

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3
Q

A 17-year-old boy presents following a syncopal episode during football practice. He denies any injury. He does not smoke, drink alcohol, or use illicit drugs. Family history is remarkable for the sudden death of his father at 40 years of age due to cardiac failure. Vitals are temperature 98.6 F (37°C) (98.6°F), heart rate 84 breaths/min, blood pressure 130/80 mm Hg, and respiration rate 16 breaths/min. Physical examination demonstrates a bifid carotid pulse with a brisk upstroke. Chest auscultation is significant for a fourth heart sound and a systolic ejection murmur without carotid radiation that decreases in intensity while squatting and increases with Valsalva. Echocardiography confirms the suspected diagnosis. What is the best management plan for this patient?

  1. Check electrocardiogram (ECG) and return to sports activities if normal.
  2. Obtain ambulatory Holter monitoring and return to sports activities if normal.
  3. Advise against participation in competitive sports but permit low-intensity exercise.
  4. Advise against all strenuous activity.
A
  1. Advise against participation in competitive sports but permit low-intensity exercise.

POINTS

This patient likely has hypertrophic cardiomyopathy (HCM). HCM occurs due to mutations of genes encoding cardiac contractile proteins. It leads to left ventricular hypertrophy and, subsequently, left ventricle outflow tract (LVOT) obstruction and diastolic dysfunction.
In HCM, syncope is caused by inadequate cardiac output with exertion secondary to LVOT. The murmur of HCM increases with Valsalva and standing and decreases with squatting and handgrip.
HCM can lead to acute LVOT obstruction, arrhythmia, and myocardial ischemia, especially during exertion. All these can cause sudden cardiac death and heart failure.
The current consensus is to refrain from participation in competitive sports, especially in patients with a high risk of sudden cardiac death (syncope, family history, etc.). Low to moderate-intensity exercise outside of competitive sports is generally permitted. Shared decision-making between the patient and the provider is advised to arrive at the optimal plan regarding physical activities.

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4
Q

A 12-year-old female presents to the provider accompanied by her mother for a well-adolescent visit. She admits to being anxious about the ongoing physical changes in her body. She reports that she started noticing breast buds palpable under the areola when she was ten and a half years of age. Which of the following is the most likely finding in this patient’s physical exam?

  1. Adult pubic hair that has not spread to the medial thighs
  2. Sparse, lightly pigmented, and straight pubic hair located at the medial border of the labia
  3. Terminal hair that fills the entire triangle overlaying the pubic region
  4. Terminal hair that extends beyond the inguinal crease onto the thigh
A
  1. Sparse, lightly pigmented, and straight pubic hair located at the medial border of the labia

POINTS

Puberty is a time of rapid and complex changes involving overlapping components: hormonal, physical, and cognitive. Tanner staging, also known as Sexual Maturity Rating (SMR), is an objective classification system that providers use to document and track the development and sequence of secondary sex characteristics of children during puberty.
In females, the normal onset of puberty ranges from 8 to 13 years of age, with an average age of ten in White Americans and age 8.9 years in African-Americans. Puberty in females begins with developing breast buds under the areola, also known as thelarche, and represents entry into Tanner stage 2. As puberty progresses, the glandular tissue of the breasts increases in size and changes in contour.
In females, thelarche is followed in 1 to 1.5 years by the onset of sexual hair (pubic and axillary), known as pubarche. The patient in this clinical scenario is considered to be Tanner stage 2.
Tanner stage 1 is preadolescent, and there is no pubic hair. Tanner stage 2 is characterized by the first appearance of sparse, lightly pigmented, and straight pubic hair located at the medial border of the labia. Tanner stage 3, hair is coarser, darker, more curled, and spreads over the middle of the pubic bone. Tanner stage 4, pubic hair is adult but has not spread over the medial thigh. Tanner stage 5, pubic hair is adult and extends beyond the inguinal crease into the thighs in a triangular shape.

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5
Q

A 7-year-old boy visits the clinic for a regular check-up. His parents report that he has difficulty with reading and learning math in school. His IQ score was 64. Which of the following statement is correct about the patient’s diagnosis?

  1. There are delayed language abilities and speech between ages 1 and 3.
  2. The diagnosis is most often made at entry into school.
  3. During the first year, there are delays in psychomotor skills.
  4. Most patients have a defined syndrome
A
  1. The diagnosis is most often made at entry into school.

POINTS

Mild intellectual disability is defined as an intelligence quotient from 50 to 70.
An organized preschool may detect mild intellectual disability but is usually not diagnosed until school entry.
Moderate intellectual disability is associated with delays in language abilities and speech between the ages of one and three.
Severe Intellectual disability is associated with delays in psychomotor skills before age one.

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6
Q

A 7-year-old boy is brought in by his parents for being uncooperative, disruptive, and sometimes combative in school. He cannot control his temper and argues with adults and other authority figures. What duration of symptoms is required to make the most likely diagnosis?

  1. 2 weeks
  2. 4 weeks
  3. 6 months
  4. 12 months
A
  1. 6 months

POINTS
This child presents with oppositional defiant disorder as evidenced by his pattern of irritable mood, disruption, and argumentative behavior towards authority figures.
The DSM-5 defines the minimum duration of symptoms as 6 months for the diagnosis of oppositional defiant disorder to be made.
If symptoms are present in only one setting (such as at home), then the diagnosis can be further specified as mild. If symptoms are present in two settings, then severity is specified as moderate. If symptoms are present in three or more settings, then it is classified as severe.
Oppositional defiant disorder is commonly co-morbid with other impulse control disorders such as attention-deficit/hyperactivity disorder and intermittent explosive disorder.

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7
Q

A 16-year-old nulliparous girl presents to the hospital with a 2-day history of heavy vaginal bleeding with clots. She denies sexual activity and missed her period one week ago. Which of the following is the best initial step in the management of this patient?

  1. Reassurance
  2. Progesterone prescription
  3. Urine pregnancy testing
  4. Pelvic ultrasound
A
  1. Urine pregnancy testing

POINTS

The patient needs a pregnancy test in the office as soon as possible.
Many patients have difficulty being honest about sexual activity.
The most important diagnoses to rule out are miscarriage and ectopic pregnancy.
An ultrasound is also indicated, but not before a urine pregnancy test.

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8
Q

A 16-year-old male with autism receives occupational therapy at school but has not been successful with shaving before school. He lives at home with his mother and sisters. The patient is being teased by the other students. What is the best option for treatment?

  1. Have the patient grow a beard
  2. Have the teacher assist him when he gets to school
  3. Find a male teen to role model for shaving
  4. Have all the males in the class form a shaving group
A
  1. Find a male teen to role model for shaving

Shaving at school as a group would only be appropriate if there were multiple patients in need of this activity.
In this case, shaving should be kept at home.
A role model would be of assistance as would an electric razor.
A role model can help the child to see things in an organized manner.

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9
Q

A 17-year-old girl presents to the provider as she is concerned about a lack of menstruation. She reports being anxious about her periods as her female peers at school started having it since last year. On examination, her vital signs are normal. Her breast examination reveals Tanner stage four. She has axillary and pubic hair. Her BMI is 19. Which of the following is true regarding her condition?

  1. Breast development or thelarche occurs four years before menarche
  2. A patient’s lifestyle should be evaluated for exercise, eating habits, emotional stress, and drug abuse
  3. The evaluation of secondary sexual characteristics requires lab work and a full pelvic exam
  4. Thyroid-stimulating hormone, prolactin, and beta-human chorionic gonadotropin is not part of the workup for her condition
A
  1. A patient’s lifestyle should be evaluated for exercise, eating habits, emotional stress, and drug abuse

POINTs
She has a typical presentation of primary amenorrhea. Breast development or thelarche occurs two years before menarche.
A provider can evaluate secondary sexual characteristics even prior to a full pelvic exam.
Simple blood work consisting of thyroid stimulating hormone and prolactin levels and a pregnancy test should be performed.
The incidence of primary amenorrhea is under 1% in the United States. The prevalence does not vary according to national origin or ethnic group.

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10
Q

A 13-year-old White American female presents to the clinic for a well adolescent visit. She was noticed to be anxious about the ongoing physical changes on her body. She had her first menstrual period at the age of 10 and has regular menstrual cycles every 28 days lasting for 5 days. She changes 4 fully soaked pads per day. The physical exam shows pink palpebral conjunctiva, breast and papilla elevated as a small mound, the diameter of areola is increased, pubic hair is sparse, lightly pigmented, straight, and located at the medial border of labia. She is concerned when will her height increase. Which of the following time period would you expect a peak in her height growth?

  1. Between Tanner stage 1 and 2
  2. Between Tanner stage 2 and 3
  3. Between Tanner stage 3 and 4
  4. Between Tanner stage 4 and 5
A
  1. Between Tanner stage 2 and 3

POINTS

Puberty is a time of rapid and complex changes involving overlapping components: hormonal, physical, and cognitive. Tanner Staging, also known as Sexual Maturity Rating (SMR), is an objective classification system that providers use to document and track the development and sequence of secondary sex characteristics of children during puberty.
Changes that are associated with, but not directly measured by, Tanner Staging, include bone growth and fusion, body composition and linear growth, and hematocrit values. Tanner Staging, rather than chronological age, should be used in assessing pubertal development.
In females, the normal onset of puberty ranges from 8 to 13 years old, averaging age 10 years in White Americans and age 8.9 years in African-Americans. Puberty in females begins with the development of breast buds under the areola, also known as thelarche, and represents entry into Tanner Stage 2. As puberty progresses, the glandular tissue of the breast increases in size and changes in contour. In females, thelarche is followed in 1 to 1.5 years by the onset of sexual hair (pubic and axillary), known as pubarche. Menarche, the onset of menses, arrives on average at age 12.5 years, regardless of ethnicity, thelarche on average by 2.5 years (range 0.5-3 years).
Between Tanner Stage 2 and 3 breast development, females experience peak height velocity. African-American females have closer to 3 years between their thelarche and menarche, accounting for greater height potential.

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11
Q

A 7-year-old child with Down syndrome and attention deficit hyperactivity disorder (ADHD) displays a lot of behavior problems at home. For example, when her older sister has friends over, the patient interferes with games and destroys art projects. Which of the following would be the most effective form of behavioral modification?

  1. Put the child in time-out for 30 minutes in a locked room
  2. Do not allow the child to play with her sibling and her friends
  3. Engage the child in other activities
  4. Refer the child to a therapist
A
  1. Engage the child in other activities

POINTS
Providing other activities is an example of redirection, a non-punitive method of behavior change that is highly effective for young children and individuals with concrete thinking. Teaching appropriate behavior is more effective than using punishment.
Positive reinforcement, such as sticker charts and rewards and negative reinforcement, such as time-outs, should be immediate, concise, and appropriate for the behavior to increase the likelihood of the behavior reoccurring. The more time that passes between the positive behavior and the positive reinforcement, the less likely the behavior will recur. The more time that passes between the negative behavior and the negative reinforcement, the more likely the behavior will recur.
Punishments lasting longer than 1 day are less effective because the consequences on day two do not immediately follow the unwanted behavior. Further, if punishment is too harsh or long-lasting, it can be hard for the parent to continue to implement the consequence.
Most behavior programs focus on reinforcing positive behavior through rewards. When positive behavior is rewarded, it increases the chances of that same behavior reoccurring.

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12
Q

A 13-year-old child is brought in by his mother because he is acting strangely. He has become “lazier” at school, and as a result, his grades have decreased. She believes he has been more depressed because he spends his free time sitting in his room and eating more than usual. On physical exam, his conjunctivae are injected, but there are no other pertinent findings. What is the best next step?

  1. Referral to a psychiatrist for depression
  2. Urine drug screen
  3. Call child protective services
  4. Referral to ophthalmologist
A
  1. Urine drug screen

POINTS

Cannabis intoxication is commonly associated with injected conjunctiva and a temporary increase in appetite.
Cannabis use has also been shown to cause lethargy, impairment of attention, memory, executive functioning, and short-term memory, which can lead to problems in school for students.
Chronic use may lead to long-term effects on cognitive performance, “amotivational syndrome,” a loss of energy and will to work.
Urine drug screen is the most efficient test to assess for THC toxicity, as positive results for THC have been reported up to 10 days after weekly use and up to 56 days after daily use.

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13
Q

A school official has contacted his local provider to develop a workshop on childhood bullying prevention. They are attempting to be preemptive to educate staff as to what to keep an eye out for. They have been advised that many forms of bullying have been identified. During a workshop on bullying in the discussion, which of the following types of bullying will be emphasized as the most common form?

  1. Physical
  2. Verbal
  3. Relational
  4. Cyberbullying
A
  1. Verbal

POINTS

Bullying can be physical, verbal, or emotional. Verbal bullying such as name-calling is a common form of bullying on both genders.
Bullying through electronic means, also called cyberbullying, is prevalent and can occur along with verbal bullying and physical bullying.
Relational bullying can be direct or indirect and includes gossiping and spreading rumors.
Bullying can be one-on-one or as a group. Group bullying has historically been referred to as mobbing.

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14
Q

You are seeing your patient, Justin, in the office for a 14-year-old adolescent visit. Justin has mild persistent asthma but is otherwise healthy. His mother comments on how much he has grown in the past year and is now towering over her! You review his growth chart with him and note that he has gained 10 cm in height and 12 kg in weight. During his exam, what would you would expect his Tanner staging and testicular volume to be?

  1. Tanner III and volume of 6 to 12 ml
  2. Tanner IV and volume of 6 to 12 ml
  3. Tanner IV and volume of 12 to 20 ml
  4. Tanner V and volume of 12 to 20 ml
A
  1. Tanner IV and volume of 12 to 20 ml

POINTS

The boy in this vignette achieved peak growth velocity in the past year. Thus, although he might have been Tanner stage III at the height of his growth, it is more likely that he will be Tanner stage IV during this exam.
Testicular volume varies from individual to individual during each stage. However, there are ranges which correspond to each pubertal stage with Tanner stage I <1.5 ml, Tanner stage II 1.6 to 6 ml, Tanner stage III 6 to 12 ml, Tanner stage IV 12 to 20 ml, and Tanner stage V >20 ml.
The pubertal growth spurts correspond closely to the pubertal stage and not to chronological age. Girls achieve peak growth velocity at Tanner stage III at 8.25 cm/year. Boys achieve peak growth velocity at Tanner stages III to IV at 9.5cm/year.
Males and females achieve peak growth velocity at different stages of development. Boys achieve this peak on average 2 years later than girls.

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15
Q

A 16-year-old girl with no past medical history presents before starting the school year. She expresses interest in joining her school’s cross-country team. She admits to being a picky eater, but denies restricting food intake, binge eating, or self-induced vomiting. She has been having some irregularities in her menstrual cycle recently that started over the summer during training camp. She is not on any medications. Her body mass index (BMI) is 18.5 kg/m2. A urine pregnancy test in the office is negative. For which of the following is this patient at greatest risk?

  1. Rhabdomyolysis
  2. Anorexia nervosa
  3. Amenorrhea
  4. Polycystic ovary syndrome
A
  1. Amenorrhea

POINTS

which includes low bone mineral density (BMD), menstrual irregularities (typically oligo- or amenorrhea), reduced energy availability, and other metabolic, immunologic, and cardiovascular anomalies caused by a relative energy deficiency.
The International Olympic Committee (IOC) Consensus statement on RED-S notes the prevalence of secondary amenorrhea in long-distance runners to be as high as 65%. That same statement notes the prevalence of disordered eating among adult and adolescent female elite athletes is about 20% and 13%, respectively.
RED-S is caused by a relative energy deficiency, resulting in a hypogonadotropic hypogonadism and a hypoestrogenic state. This leads to the amenorrhea, low BMD, and stress fractures seen in this condition. Estrogen levels, menstrual cycles, and BMD will improve after lowering the intensity of exercise or increasing body weight and nutrition status.
Nutrition is important when participating in intense physical exercise, such as organized sports with regular practice and competition sessions. Along with rest, proper nutrient intake is needed for the body to repair itself after intense exercise. Athletes should be screened for healthy eating habits, and consume adequate amounts of calcium and vitamin D.

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16
Q

A 12-year-old adolescent male presents to his primary care provider’s office with his mother. The mother is concerned that her son only likes to play with girls, is involved in common plays with them (plays with dolls, feminine toys and avoids rough and tumble plays), often tries to dress up like them for the last six months. This pattern has progressively increased, and the patient tries to dress up like a girl every day and insists on doing so at school as well for the last one month. She is also concerned that whenever she had tried to engage him in addressing the concern, the patient becomes avoidant and secludes himself from the rest of the family. On exam, the patient appears withdrawn, disinterested and makes little eye contact. What should be the next step of management?

  1. Prescribe hormonal treatment for the child
  2. Reassure the mother that this is age appropriate behavior
  3. Make a referral for child psychiatry and psychology
  4. Make a referral for an endocrinologist
A
  1. Make a referral for child psychiatry and psychology

POINTS

The first step to evaluate the individual for gender dysphoria is to make a referral to child psychiatry and psychology, before getting an endocrinology referral.
it is important to evaluate, rule out or treat any underlying psychiatric illnesses. For example, possible depression in the case vignette.
It is important not to neglect or ignore the patient’s or parents’ concern for gender dysphoria.
It is appropriate to start hormonal treatment for this patient, but first underlying possible depression should be adequately treated and controlled.

17
Q

An 8-year-old male presents with a large superficial mass on his back which looks like a bag of worms. The mass started developing on his back when he was 3 years old. It has gradually increased in size. On physical examination, the mass is well-circumscribed, grey in color, and ovoid in shape. There is the presence of golden-brown dome-shaped masses on the iris of both eyes. Which of the following will most likely be present in this patient?

  1. Cafe au lait macules
  2. Melanocytic nevi
  3. Nevus depigmentosus
  4. Splenomegaly
A
  1. Cafe au lait macules

POINTS

A plexiform neurofibroma is essentially pathognomonic for neurofibromatosis type 1.
Cafe au lait macules, intertriginous freckling, optic gliomas, Lisch nodules, and skeletal dysplasias are often found in patients with neurofibromatosis type 1.
Plexiform neurofibromas most commonly occur on the head/neck, trunk, and extremities. They surround multiple nerve fascicles and can grow to be large. If superficial, they commonly present as a skin-colored or hyperpigmented nodular swelling. Deeper lesions arising from spinal nerve roots may become highly irregular and tortuous.
For most cases, complete surgical excision is the preferred treatment, with local recurrence extremely rare. There are currently no alternative therapies for cutaneous neurofibromas.

18
Q

A 16-year-old female is brought by her mother to see a primary healthcare provider because of disturbing behavior. She complains that the patient had always been outgoing and friendly and used to share everything with her, but now, she is cold and withdrawn. She has also recently been caught with illicit drugs in her school where her grades have been plummeting. On being privately questioned, the patient attributes her changes to the recent move to a new neighborhood. She identifies as bisexual and says her mother is unaware of her sexual orientation. On further questioning, she admits to having no friends and believes that “life is over-rated anyway.” During the interview, the provider notices parallel scars on her wrist. What is the appropriate next step in the management of this patient?

  1. Utilize the patient health questionnaire
  2. Utilize the suicide risk screen
  3. Immediate treatment with fluoxetine commenced
  4. Mandatory inpatient surveillance and treatment with citalopram
A
  1. Utilize the suicide risk screen

POINTS
The patient in the question is exhibiting symptoms of depression such as social withdrawal, use of illicit drugs, loss of interest, and anhedonia.
Risk factors for the development of suicide in this patient are social isolation, substance use, and life-changing events like moving to a new neighborhood.
Some of the signs suggestive of impending suicide are the loss of interest in activities, abusing drugs, social isolation, self-harm, etc.
One of the first steps in management is to determine the severity and intensity of depression, along with the risk of suicide. This is done by the use of questionnaires and of the two, the most appropriate is the suicide risk screen, which is especially helpful in assessing young patients.

19
Q

A mother requests a urine drug test for her 15-year-old son. She is concerned that he may be using drugs since he recently joined a group of friends that are known to use marijuana. He denies using any drugs, but the mother insists on performing a urine drug test. When talking with the adolescent patient privately, he continues to deny using drugs and states he does not want to undergo a urine drug test. The patient does not appear to be intoxicated or confused and demonstrates that he has developmentally appropriate decisional capacity. Which of the following is the most appropriate management strategy for this patient?

  1. Perform the urine drug test as requested by the mother
  2. Do not perform the urine drug test and call child protective services
  3. Disregard the patient’s wishes and further discuss with the mother the risks, benefits, and consequences of performing the urine drug test
  4. Do not perform the urine drug test and discuss confidential counseling options for alcohol or drug use problems that do not require parental consent or notification
A
  1. Do not perform the urine drug test and discuss confidential counseling options for alcohol or drug use problems that do not require parental consent or notification

POINTs

Many state laws recognize that cognitively mature minors can independently consent to medical services related to alcohol and drug use problems similar to an emancipated minor.
Medical diagnosis and treatment of alcohol and drug use problems are specific services that minors can pursue or refuse without parental consent and notification.
Adolescents or cognitively mature minors cannot be forced to perform drug testing without their consent, even though a parent requests it.
Calling child protective services on the mother for requesting an undesired drug test on her son is not a form of child abuse or neglect. Instead, this is an opportunity for the healthcare provider to counsel and assist the patient and mother navigate the common and challenging experience of adolescent use of alcohol and drugs.

20
Q

A 17-year-old male patient comes to the primary care physician for a pre-participation sports physical examination. He has no active complaints. He has no significant past medical history, and his growth is appropriate for his age. His blood pressure is 110/70 mmHg, pulse rate is 80 beats/min, and respiratory rate is 13 breaths/min. His physical examination reveals a grade 3 out of 6 ejection systolic murmur that is heard best along the left sternal border. His further testing reveals that the heart murmur decreases in intensity when the patient is asked to rapidly squat and increases in intensity when the patient is asked to bear down as if having a bowel movement. Which of the following findings is most likely to be seen in his echocardiography?

  1. Prolapse of the mitral valve
  2. Right ventricular outflow obstruction
  3. Thickening of the interventricular septum
  4. A bicuspid aortic valve
A
  1. Thickening of the interventricular septum

POINTS

Hypertrophic obstructive cardiomyopathy (HOCM) is an autosomal dominant disorder caused by genetic variants that code for a portion of the contractile element of the cardiomyocyte.
The mutations which cause hypertrophic cardiomyopathy allow for heightened myofilament calcium sensitivity, thickening of the interventricular septum, and eventual obstruction of blood flow.
Although hypertrophic cardiomyopathy is commonly asymptomatic, symptoms of obstruction can result in chest pain during exertion, tachycardia with shortness of breath, syncope, and sudden cardiac death.
Hypertrophic cardiomyopathy is the most commonly inherited cardiac disorder, with a prevalence of 1 in 500. It is the leading cause of sudden death in young individuals and currently has no definitive cure.