UWorld Flashcards
(31 cards)
A kid comes to the clinic and complains of 10 episodes of vomiting a day once a month for the past 6 months. At its peak, she is vomiting 4+ times an hour. Family history is significant for migraine headaches. She has no symptoms between episodes and no underlying disorder is found after extensive work up. How do you treat her?
She has cyclic vomiting syndrome. Acutely treat with hydration, anti-emetics and parental reassurance. Due to its relation to migraine headaches and abdominal migraines, may also benefit from sumatriptan.
A mom brings in her 2 month old infant complaining of jaundice, light-colored stools and dark urine. Physical exam shows hepatomegaly. Labs show a conjugated hyperbilirubinemia. How do you make the diagnosis in this kid? Then how do you treat him?
He likely has biliary atresia. Initial step in diagnosis is RUQ ultrasound looking for absent or abnormal gallbladder. Next diagnostic step may be scintigraphy that shows no uptake into the small intestine. Gold standard is intra-operative cholangiogram. Ultimately this kid will need a transplant. For now you could do the Kasai procedure (hepatoportoenterostomy).
Under what 4 medical circumstances do minors not need parental consent?
Emergency care
STIs
Substance abuse
Prenatal care
What situations make a minor emancipated from his or her parents?
Homeless
Married
Military
Parent
Financially independent
High school graduate
A mom brings in her 6 month old daughter complaining of poor feeding, irritability, decreased activity and vomiting. On physical exam she has a bulging anterior fontanel, prominent scalp veins and widely spaced cranial sutures. Chart review shows that her head circumference has increased from 40th percentile to 90th percentile in 2 months. Her head CT is shown below. How do you treat her? How would your work up be different if she was asymptomatic and had the same physical exam finding?
This child is showing signs of increased intracranial pressure due to hydrocephalus. She will need a ventricular shunt placed to the peritoneum, pleura or right atrium. Note that you would’ve done an MRI had she been asymptomatic with increased head circumference.
A mom brings in her 10 month old boy because he is not walking yet. Birth history is significant for prematurity. Physical exam reveals hypertonia and hyperreflexia most prominent in the lower extremities. Both of his feet point down and inward. On passive range of motion testing, he resists more strongly with faster testing. What are risk factors for his condition?
Delivery before 32 weeks is the greatest risk factor for cerebral palsy. Other risk factors include:
- IUGR
- Intrauterine infection
- Antepartum hemorrhage
- Placental pathology
- Multiple gestation
- Maternal alcohol ingestion
- Maternal tobacco use
5 types of cerebral palsy
Spastic, athetoid, rigid, tremulous and ataxic
A mom brings in her child to the clinic and you diagnose him with spastic cerebral palsy. What is your plan going forward with this kid?
He will need PT/OT his whole life.
Baclofen and botulinum toxin can be given for spasticity
Surveillance for development of common comorbid conditions like scoliosis, intellectual disability, epilepsy and strabismus.
A 5 year old obese girl presents with coarse axillary and pubic hair. She has not yet developed breast buds. She has some mild facial acne and body odor. What organ is most likely responsible for her symptoms?
Adrenal gland. Increased adiposity results in increased insulin. Insulin can stimulate the adrenal glands to produce androgens and begin adrenarche. It can also stimulate the ovaries to produce estrogen and progesterone, resulting in thelarche. Additionally, increased adiposity causes increased leptin secretion. Leptin can cause central puberty activation by inducing pulsatile GnRH secretion, resulting in increased LH/FSH and thelarche.
At what ages in boys and girls is puberty defined as premature.
< 8 in girls and < 9 in boys
For what conditions is isolated premature adrenarche a risk factor?
PCOS, DM II and metabolic syndrome
You are working in the NICU and a 2 week old baby born at 34 weeks with DiGeorge syndrome and truncus arteriosus begins to vomit and has bloody stools. The only new change in his regiment was initiation of enteral feeding with formula 2 days ago. Physical exam shows increased abdominal girth. X-ray is shown below.
Prematurity, low birthweight, reduced mesenteric perfusion and hypotension are all risk factors for NEC. This kid has pneumatosis intestinalis that could progress to pneumoperitoneum (shown below) without intervention. He needs broad-spectrum antibiotics to prevent shock and bowel resection.
How does C. difficile typically present in infants?
Kids < 1 year are usually asymptomatic, possibly due to lack of toxin receptors in the colon
For the past year, your 16 year old patient has had uncontrollable facial grimacing, neck jerking, eye blinking, grunting and snorting. He can suppress these movements, but not for long. He has associated OCD and ADHD. How do you treat him?
He meets DSM-5 criteria for Tourette disorder. First line therapy are the 1st generation antipsychotics (pimozol, haloperidol, fluphenazine). However, pimozol can cause long QTc and the others have lots of side effects. The 2nd generation antipsychotic risperidone is commonly used. Alpha-agonists (clonidine, guanfacine) are an alterantive. CBT is key.
A 4 month old boy presents with lactic acidosis, hypoglycemia, hyperuricemia and hyperlipidemia. Physical exam reveals a child with fat cheeks, short stature, thin extremities and a protuberant abdomen. There is appreciable hepatosplenomegaly and enlargement of the kidneys. What is the most likely cause of his symptoms?
Von Gierkes’ disease (type I glycogen storage disease, glucose-6-phosphatase deficiency)
A 2 week old baby presents with feeding difficulties and heart failure. Physical exam reveals a hypotonic baby with hepatosplenomegaly. What is the most likely cause of this child’s heart failure?
He likely has Pompe’s disease (type II glycogen storage disease, acid maltase deficiency). These kids get heart failure due to progressive hypertrophic obstructive cardiomyopathy.
A mother brings in her baby complaining of growth retardation and seizures. Physical exam reveals a non-protuberant abdomen, hepatosplenomegaly and normal sized kidneys. Labs show hypoglycemia, hyperlipidemia, elevated LFTs, fasting ketosis and normal lactate and uric acid levels. What is causing this child’s condition?
Deficiency in glycogen debranching enzyme activity (type III glycogen storage disease)
A mother brings in her 18 month old baby complaining of failure to thrive. Physical exam shows hepatosplenomegaly. Imaging reveals a cirrhotic liver. What is the most likely glycogen storage disease?
Amylopectinosis (type IV, branching enzyme deficiency)
A child presents with growth retardation. Physical exam reveals hepatomegaly. Labs show a mild hypoglycemia, hyperlipidemia and hyperketosis. Lactic acid and uric acid levels are normal. What is the most likely cause of this relatively benign condition?
Liver phosphorylase deficiency
This child had abdominal distention and bilious vomiting at 12 hours of life. What are risk factors for this condition?
The triple bubble sign indicates jejunal atresia. Unlike duodenal atresia (shown below), it is not associated with chromosomal abnormalities. It is typically caused by vascular bowel ischemia in utero. This can be due to drugs like cocaine and nicotine. It can also be seen in kids with CF meconium ileus that caused a volvulus and bowel infarction in utero.
An infant has failed to pass meconium for 48 hours. KUB is shown below. What is your diagnosis?
Hirschsprung disease. Note the distended bowel loops and abscence of air in the rectum.
How do you differentiate central vs. peripheral precocioius puberty in a girl?
In central, LH and FSH will be elevated. This finding should always be followed with head imaging.
4 causes of microcytic anemia
Iron deficiency
Thalassemia
Lead poisoning
Sideroblastic anemia
4 causes of normocytic anemia and low retic
Leukemia
Aplastic anemia
Infection
Medication side effect
