Tommy's Concepts: 548-600

[Anonymous]

548.
A young lady named Ayn Rand is breast feeding her baby. But she comes to you asking what the difference is between dairy milk and her own breast milk in terms of vitamins/nutrients? What do you say?

A) As you recall, we said human milk has LESS protein, BETTER iron absorption, much MORE vitamin C, and much less vitamin K than cow milk (so this is why many women eat vit K supplements).

549.
Case: You have a 5 day old baby named J.D. Salinger. On physical exam, you note he has an asymmetric Moro reflex. The biceps carry no reflex. PE demonstrates his left arm is slightly turned inward. Which 2 cord segments are affected? AND, what is the dx?

A) C5 and C6 are damaged...this is Erb Duchenne syndrome.

550.
Case: A young boy named Michael Chrichton comes in with a hand that appears looking like a "claw". BAM! You know the dx, now tell me which cord segments are involved?

A) The Klumpke's syndrome is C7, C8 and T1 lesions.

551.
Case: I, Tommy, actually saw and treated this case with my own hands a couple of days ago...a 10 year old boy came in with recurrent UTIs. His mother said he had a congenital disease called "Prune Belly Syndrome".
Which organ system does this dx hit often?

A) Not to be confused with Potter's syndrome, PRUNE Belly Syndrome lesions the kidneys.

552.
Case: A twenty something couple walk into your clinic with a baby that has cyclical hypoxic events that are not very predictable. But, the baby is noted to choke and gag when he feeds. Then he stops for a moment, and breathing continues until the next "event." He otherwise has a normal PE and history of birth was non traumatic. What is this disease?

A) He has choanal atresia, where his nasal obstruction may cause death from asphyxia. During attempted inspiration, the tongue is pulled to the palate, and obstruction of the oral airway results. Especially during feeding, he must "close" his mouth on the nipple, and he can't breathe!

553.
Case: You see a 14 year old boy named Tom Clancy who is vigorously itching his scalp and losing tufts of hair. With a clinic fluorescent lamp, you see patches of blue green areas lighting up in the dark when shined on his head. What bug is this?

A) This is Tinea capitis. Give an anti fungal.

554.
Case: Let us say that you have a patient and you need to know the level of reducing sugars in the urine. Do you use the Clinitest or the methylene blue stain test?

A) The Clinitest. The Methylene blue test helps identify white cells in the feces.

555.
Case: You are asked which test starting with the letter "B" can help identify lesions in the LOWER intestinal tract like intussusception.

A) Barium enema test

556.
Case: A 12 year old boy named John Keats comes into your office with easy bruising and petechiae all over his body. It came on suddenly after a cold. There is no hepatosplenomegaly and he has a mild fever, other than that, his PE is normal. His CBC demonstrates thrombocytopenia. Does this look like ALL or something else?

Answer: This is ITP, which follows a viral infection usually. The disease should resolve on its own in two weeks.

557.
Case: For the previous patient, John Keats, if the symptoms don't resolve in 2 weeks, what can you give him?

A) You may consider giving steroids and gamma globulins.

558.
Case: You see two patients coming in with wheezes and both look like asthma. But your attending tells you one of the patients has bronchiolitis. How can you tell the difference?

A) These two diseases present so similiar, but that is why HISTORY is so important. The family history and PMH should reveal prior episodes and a family history of asthma. So, make sure you always pay attention to history and not only the HPI.

559.
Case: You have a worried 30 year old lady named Erich Maria Remarque who brings her daughter in because the daughter is 3 years old and she can copy a circle, but NOT an square. Is she behind?

A) No, most 3 year olds cannot copy a square, but can copy a circle!

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560.
Q) Other than HIV infection, which conditions should make you give a two year old the pneumococcal vaccine?

A) Think and choose steroid use, splenectomy, sickle cell anemia, kidney failure, and SLE.

561.
Q) A 9 year old male named Stephen King wakes up in the middle of the night with a facial tick and twich and when he goes to his parents' bedroom, his attack suddenly stops. Later in the ER, his EEG is normal. Do you start seizure meds?

A) No, this is benign partial childhood epilepsy, and he will outgrow it usually. Be careful, he stayed conscious during the attack and the attack was short in duration.

562.
Two baby children named Anne Tyler and Wallace Stegner come into your office. The mothers say that during feedings, the baby Anne drools and gags and coughs! After crying, the coughing does not stop! This sounds like choanal atresia, but both are NOT. The other baby Wallace has bilious vomiting....Which kid has a tracheoesophageal fistula and which one has Duodenal atresia?

A) Wallace, which has bilious vomiting, has duodenal atresia. Anne, with the gagging and coughing has tracheoesophageal fistula.

563.
Case: Your attending hints the next baby coming in has a face that is very round and soft. He is not retarded mentally but he is short in height. His liver and kidneys are slightly large. He has a defect in his clotting but the hypoglycemia is notable. What is the disease and missing enzyme?

A) The child has Von Gierke's disease and is missing an enzyme in gluconeogenesis called glucose 6 phosphatase.

564.
Q) Appearing in 1 in 4000 births, pyloric stenosis occurs when in childhood and tell me if it has bile in the vomit?

A) Pyloric stenosis occurs a few weeks, NOT HOURS, after birth. It does NOT have bile in the vomit.

565.
Case: A male name Jeff Wiley who is 32 years old confesses to you that he lies on his tax returns and embezzles money at work. Does Federal law say you must inform the federal authorities?

A) No.

566.
Case: A pregnant female comes in with Phenylketonuria. What exact enzyme is missing?

A) Phenylalanine hydroxylase

567.
Case) You WILL see this case a lot...A 5 year old kid with a week long fever also comes in with dry cracked lips, shedding of the skin, and edema and rash all over, and cervical lymphadenopathy. What is the disease? What body part(s) does it affect?


A) Kawasaki syndrome. This is a vasculitis or medium and large coronary vessels.

568.
Case: A young patient of your named Thomas Wolfe comes in before he is entering a US college. Oh, you give him MMR, diphteria, tetanus, polio vaccines. But, do you HAVE to give him his Hep B shot? What about his H. flu B shot?

A) No, they are recommended, but NOT required.

569.
Case: Again, you will see BILLIONS of asthma patients. Other than albuterol, many use steroids. But additional meds include Ipratropium and Zileuton and Zafirlukast. What is the MOA of these THREE meds:

Iptratropium...antimuscarinic on receptors
Zileuton...blocks lipoxygenase
Zafirlukast...blocks leukotriene receptors

570.
Case: You examine a newborn child who presents with dark lower extremities but a light pink upper extremities. PE reveals a machine gun sound over the heart. What is happening?

A) Patent ductus arteriosis and a Coarctation of the aorta

571.
A 21 year old mother comes in with a question about breastfeeding since she has a vaginal yeast infection. Can she use the proper drug safely?

A) Yes, topical administration of nystatin for Candida is SAFE for breastfeeding.

572.
Case: The previous breastfeeding mother asks you if she can use benzodiazepines because she is anxious about being a newborn mom and methylphenidate for her ADD for graduate schooling. She still wishes to breastfeeding. What do you say?

A) CNS drugs, Steroids, PTU, and alcohol and SOME antibiotics like ciprofloxacin and tetracyclines should be avoided for breastfeeding moms.

573.
Case: A young child named Albert Einstein comes in with cystic fibrosis. He often develops respiratory infections as sequelae. The radiologist comes back and says the trachea is deviated to the left side and you had previously heard absent sounds on the right side. What is the pathophys?

A) These patients often develop pulmonary infections which lead to rupture from cysts caused by S. aureus. Thus, a PNEUMOTHORAX ensued.

574.
Case: A young boy named Issac Newton was eating a lot of frozen flavored ice cubes and holding them in his cheeks. Later, his mother brought him to the ED because his cheeks were slightly swollen, cool, and erythmatous. What is the name of this condition?

A) Fat injury from the cold is PANNICULITIS.

575.
Case: A young child patient of yours is getting his immunization shots and reacts poorly to the DTaP shot. Which one component is likely to have caused the reaction (Choices: Diphtheria/Tetanus/Pertussis)?


A) The Pertussis part is usually culpable if there is an adverse reaction.

576.
Case: An eight year old boy named Johann Kepler was playing basketball when he noticed gradual pain, stiffness in the hip area through the playing season (three months). A radiograph showed femoral head necrosis. What dx is this?

A) This is Legg-Calve-Perthes disease.

577.
Case: Same clinical case presentation as HY Concept 576, but this boy basketball player is quite overweight. Other than Legg Calve Perthes dx, what is likely the problem?

a) Slipped capital femoral epiphysis.

578.
Case: Now, another member of the boys' basketball team named Ernest Rutherford started playing soccer in field sprayed with insecticides. He later started the typical cholinergic symptoms of "DUMBELS" or urination, pooping, sweating, salivating, etc. Other than Atropine, what ELSE can you often use that starts with the letter, "P"?

A) Pralidoxime, which reactivates acetylcholinesterase.

579.
Case: A neonate born named Alfred Hitchcock suffered from sepsis due to E-coli. There is a strong correlation between this bug and galactossemia, which we already studied (recall hepatomegaly, hypoglycemia, jaundice?). What is the exact enzyme that is missing?

A) Galactose 1 Phosphate Uridyltransferase

580.
KNOW that Fetal Alcohol Syndrome is EVERYWHERE. So understand exactly how it presents and what organs are involved. Understand it can "look" like Cerebral Palsy and/or Down's, so watch the demonstration of the history. You need to present these cases to Child Protective Services.

581.
Case: If I gave you a case of a cyanotic newborn baby and showed you a RADIOGRAPH with a "boot shaped heart" and slight pulmonary vascular markings, what common dx is that, and which specific finding is most important to determine if the baby will survive?

A) This is the Tetralogy of Fallot and the degree of pulmonary stenosis predicts the outcome of the baby.

582.
Case: When you take tests and go into clinics, you will see common things commonly. So, say I have six patients with one of the following:
1-PKU
2-Cleft Palate
3-Clubfoot
4-Hypospadias
5-Phocomelia
6-Myelo-meningocele
Which is the MOST common one you will see?

A) It is hypospadias, one in five hundred....

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583.
HARD CASE: Listen, you see a child who is a GIRL in your clinic and she looks like she is autistic. She is 4 years old. Her mother said that she seemed fine until 1 1/2 years of age and then there was neurodevelopmental arrest and then sudden regression. Her PE resembles Cerebral Palsy with loss of motor functions. Two attendings come in and tell you that it is NOT cerebral palsy or autism. They say that it only happens to girls, the gene defect is MECP2, and she has short stature and an enlarged head as a hint to you. What is this dx that starts with an "R"?

A) Rett's syndrome

584.
Case: You will definitely see this on tests and clinics. You have a girl who is 12 years old and she is short for her age group at this time. BUT...labs reveal elevated FSH and LH signaling .... what? And you note a history of UTIs and hypertension. What is this common dx which occurs in 1 in 2000 women? Can she have children? What meds do you give?

A) You WILL see Turner's syndrome. They have ovarian failure and FSH and LH will be elevated. The coarctation of the aorta is related to the HTN. Due to ovarian failure, she sadly cannot have children. You need to give her GH or somatotropin. And estrogen at the later stages.

585.
Case: You see a woman in your clinic with seizures, mental retardation, and skin lesions. Your attending TELLS you this is classic tuberous sclerosis. What is the genetics here? AD, AR, XR, XD?
A) AD, or Autosomal Dominant

586.
Case: "Tyrosine is a precursor for what amino acid?" asks your attending.

A) Dopamine (VERY CRITICAL) Think "I married a man named TYler (Tyrosine) who became a DOPe! (Dopamine)."

587.
Case: We covered the fact that PKU is a def. what enzyme.... ? And in PKU, what primary food group must the patient NOT eat?

A) This AR dx (missing phenylalanine hydroxylase) must be treated with amino acid bars (among other Rx) and you must tell your patient to avoid meat, dairy, and nuts. Plus, tell them to be aware of some sodas and potato chips, which are high in aspartame and phenylalanine.

588.
A five year old boy patient of yours comes in with an overdose of a common drug used for bed wetting (starts with letter "i"). How do you think he will present?

A) This is imipramine, a tricyclic. Overdose presents with lethargy, epilepsy, heart rhythm irregularities.

589.
Case: A 30 month old child presents with small bowel obstruction seen on x-ray. His PE has bad colicky abdominal pain with bloody diarrhea and vomiting. You feel a mass in the epigastrium. What is going on?

A) This is intussusception. Very common.

590.
Case: Recall what I said, to REALLY LEARN and RETAIN, you must study the differential diagnoses TOGETHER. So, the last case was intussusception. But there is another dx that is similar in presentation and the most common congenital dx of the ileum that involves the vitelline duct and ectopic pancreatic and gastic tissue with the GI bleeding. What is this???

A) Meckel's diverticulum (very diff to diagnose at birth)

591.
Case: HARD, but doable: You are in a city in Israel where the incidence of this dx is 6 in 1000, very very common. A 5 year old boy is slowly starting to lose his protective reflexes, and becoming ataxic (demyelination). He is getting worsening respiratory problems. This disease name starts with the letter "K" and sounds like the word "Cab". What is the deficient enzyme here? What is the inheritance?

A) This is Krabbe dx. Krabbe disease is an autosomal recessive sphingolipidosis caused by deficient activity of the lysosomal hydrolase galactosylceramide beta-galactosidase (GALC). GALC degrades galactosylceramide, a major component of myelin. The elevated levels lead to widespread destruction of oligodendroglia in the CNS and to subsequent demyelination. Death from respiratory failure often results.
592.
Case: In the same "category" as the previous concept is this...you see a patient at 6 months of age with hepatosplenomegaly, lung problems, failure to thrive and psychomotor retardation. Your attending sadly tells you the patient will likely die by age 3. What is this dx that starts with the letter N.... and then P...... ? What enzyme is deficient?

A) This is Nieman Pick Disease and results from the deficient activity of sphingomyelinase, a lysosomal enzyme encoded by a gene located on chromosome bands 11p15.1-p15.4. The enzymatic defect results in pathologic accumulation of sphingomyelin (which is a ceramide phospholipid) and other lipids in the monocyte-macrophage system.

593.
Case: This HYer is so close but so far from the LAST HYer concept. So, listen up...you have another patient who is an Ashkenazi Jew with hepatosplenomegaly, pancytopenia, and mild skeletal disease, and you are thinking Nieman Pick, but this is NOT it...this is Gaucher disease. OK, so what enzyme is missing?

A) Gaucher disease is a lipid storage disease, characterized by the deposition of glucocerebroside in cells of the macrophage-monocyte system. Deficiency of a specific lysosomal hydrolase, acid beta-glucocerebrosidase leads to the symptoms. Unlike Nieman Pick disease, there IS a medical treatment! You must get everything right for your patients!

594.
Another similar case! This time you got close enough to know the ataxia, lost reflexes, slurred speech is Metachromatic leukodystrophy. So close to Krabbe's and Gaucher's in presentation...what is the enzyme missing?

A) arylsulfatase A!!!

595.
Case: A young patient comes in with a triad of meningomyelocele, spina bifida, and hydrocephalus. He is 8 months old with a large head. What is the name of the disease? And what is the Rx?

A) This is Arnold Chiari syndrome and you need to give acetozolamide.

596.
There are two distinct signs that a baby was shaken abusively...ie "shaken baby syndrome". What are they? You MUST recognize them for the child's sake!

A) You may see a floppy baby with retinal hemorrhages and subdural hematoma.

597.
Case: You are seeing the delivery of a baby from a 18 year old young female with SLE. What is her baby most at risk for (name the organ system)?

A) SLE is assoc. with complete heart block towards the child.

598.
Case: An attending nephrologist comes in and explains to you that he has a patient with a defect in the proximal renal tubular reabsorption of phosphate. The patient is a young child and is short for his age. He tells you this is Vitamin D resistant rickets. What is the inheritance type?

A) X-linked dominant

599.
Case: ANOTHER child comes in with vitamin D resistant rickets. The most common rickets in the the USA. How will the child walk towards you?

A) The rickets causes bow leggedness and will result in a duck waddle.

600.
Case: I sadly saw this one myself....but let's say you see a deceased newborn infant with a prominent occiput and low set ears. His hands are clenched with rocker bottom feet. Which trisomy is this? 13, 18, or 21???

A) This is Trisomy 18

[dora]

save time for many of others

[nikki]

thank you so much

if you need something step1 stuff, lemme know.

i'll send it to you if i have what you need.

nikki@connectionless.net