10-year-old girl presents to the Pediatric Critical Care Unit with a five-day history of abdominal pain, vomiting, and progressive altered mental status. On examination, she's lethargic, GCS 10, weakness of the upper and lower right side, 4 over 5 vital signs, ARCA rate 154, respiratory rate 21, blood pressure 109 over 74, initial labs show normal, they are normal except for bicarbonate of 10, anion gap of 35, lactic acid 1.3, ammonia 67, beta-hydroxybutyrate 2.7, normal is less than 0.5. The family reports that she had similar symptoms three years ago. She's 10 now, but no diagnosis was determined at that time. After adequate hydration, the next step in the management should include insulin infusion until bicarbonate is at least 16, oral L-carnitine, intralipid infusion, branched chain amino acids free formula, glucose-free IV fluids, methionine valine free formula, glucose-free IV fluids, high-protein containing diet. Which one of those, okay, so 10-year-old, acidosis, right, what else you need to grab from there? Okay, I will go to, so you rehydrated the patient. People are still answering. Okay. Can you guys see the correct answer? Okay. That is intralipid infusion, branch amino acids, free formula. Who has this one? Okay. This is a very tough question, because it doesn't really fit into any of the algorithms that I tried to teach you in the earlier lecture. The answer is they say the patient has maple syrup urine disease. And probably MSUD probably is a good answer on the boards, because it is one they do like to test on. The rationale goes a lot into what the enzymatic deficiency is, branch chain alpha-keto acid dehydrogenase. There's a lot of different complexes of that enzyme, which can sort of determine the severity of it. Traditionally, if we skip the middle, traditionally the metabolic phenotype of MSUD on the basis of residual BCKD enzyme activity is termed classic, if it's less than 3%, or intermediate in 3% to 30%, so there can be different manifestations of it. Rarely people have partial branch chain keto acid dehydrogenase deficiency, which manifests only intermittently. In classic, 75% of cases, clinical onset usually occurs within the first weeks after birth, and includes a maple syrup odor, acute metabolic decompensation with feeding problems and drowsiness, followed by progressive coma with involuntary movements, seizures, and respiratory failure. Diagnosis is usually established by establishing plasma branch chain amino acid levels, including allylucine, which is pathognomonic, and their corresponding BCKs in urine. Treatment consists of dietary leucine restriction, branch chain amino acid-free medical foods, dish supplementation isoleucine and valine. As I mentioned, you do have to supplement that after holding all your leucine, and frequent clinical and biochemical monitoring. Let's go on to the next slide. It's the next one? The next one? Yeah. The next question, you mean? No, no. I think there's more to the answer. Yeah. Methionine and valine-free formulas are given only to patients with proven propionic acidemia or methylmalonic acidemia. L-carnitine is not needed in treatment of MSUD, and the patient appears to have intermittent form of MSUD. If we go back to the actual question, in terms of, like, test-taking, if you think it's... Can you go back one more? If you think about metabolic disease, how often do you not want to give glucose? So, D and E can be pretty much eliminated quickly. And insulin infusion, you don't really hear about hyperglycemia here, so you're probably not going to give insulin. You don't think it's diabetic ketoacidosis. And then you're stuck between B and C, and there's nothing really to point to needing carnitine, which is more about... It's usually more of the mitochondrial issues. And you're sort of stuck by elimination with C. So, if all else fails, C would be a good guess here. Intralipids provide lots of calories and trying to keep away from branched-chain amino acids. That's the best I can do. What ICU would do that without a definitive diagnosis? Absolutely zero. You know? Yeah. Yeah. I think for these ones, you know, like, just learn the algorithm, you know, like Mike showed a little, you know, ketones, no ketones. And, again, make a little, you know, sticker there in your kitchen and review it. Because unless you do metabolic, you know, just all the big details, I won't get them. No. I think there'll be... I think there's some basic concepts. That's why they have geneticists. That's why we have biochemical. Even within genetics, there's biochemical geneticists now, and they would be advising us on this. Okay. So, there's one more sort of test-taking strategy there. Yesterday, you heard, if you hear ammonia, think liver disease. The clue here, so there's an asterisk to that, that is liver disease or an error of metabolism. And so that clue here was this is recurrent. And so that little detail was there on purpose. Right. And so that can help you with differentiating that, other than the fact that the question is today and not yesterday. Yeah. Yeah. Okay. 12-month-old boy admitted to the pediatric ICU after a cardiac arrest from a submersion injury. He had been resuscitated after a 20-minute downtime. Despite maximal therapy aimed at optimizing neurologic protection, he progresses to brain death. Two neuroexams and apnea testing 12 hours apart have been performed and are consistent with absence of brainstem function. Which of the following findings during the brain death examination is inconsistent with the determination of brain death? A, apnea test performed with absence of respiratory effort with rise in PCO2 from 45 to 60. B, apnea test performed with absence of respiratory effort with rise in PCO2 from 45 to 70. And spinal reflexes noted on examination. C, EEG showing isoelectric activity after spinal reflexes noted on examination. D, flow study showing no cerebral blood flow but detectable serum level of phenobarbital. E, no response to deep painful stimuli, pupils at mid-position, or fully dilated, absence of light, corneal, calf, gag, and oculovestibular reflexes. So which one is inconsistent? Should I go? Okay. Okay. Who has that one? Oh. Are we all done? Well, it looks like everyone picked this one up. It's a challenging question because, one, it mentions inconsistent, and that's not typically correct, and you tend not to use that in question writing. If we go to the rationale on the next, the important thing to remember here, the key to this, is that it says for children older than 30 days, besides two examinations separated by an observation period of at least 12 hours, two apnea tests are required. In order for the apnea test to be consistent, brain death, there must be a rise in PCO2, of at least 20 millimeters of mercury, and the PCO2 must be greater than or equal to 60 with no respiratory effort. Spinal reflexes may be seen, as you all know. Confirmatory studies such as EEG and brain flow may be helpful to shorten observation period when it might be difficult to confirm, such as when toxins or medications are present in detectable levels, and when there are ambiguous findings. An EEG finding consistent with brain death is isoelectric or no activity. A brain flow study consistent with brain death would show no flow. It's actually an interesting question, because in New York State, we're actually just changing our brain death with the newest guidelines. In New York State, we only have to use one apnea test, but the new guidelines stipulate two apnea tests. We're actually changing our hospital policies. So it's the 20 millimeters. So I see a hand up in the distance. I have a couple of questions. I know it's probably old, but EEG is no longer considered a confirmed brain death. That is correct. So should we assume that that would not be a concern for students at the hospital? Probably not. Yeah. And then we shouldn't consider a brain death exam when somebody has a detectable level of phenobarbinol therapy. So are there guidelines for that? Yeah. It is not a good question, that for sure. So you're right. We have issues with this question as well. Yeah. There are new clarifications here. But, you know… Yeah. But also, I think even the new guidelines talk about whether there are clinically significant levels of… What if the level is one or two? I thought the guidelines for phenobarbital were that they can't be super therapeutic according to the new brain death criteria. Right. It's just so anything less than 30, I don't remember correctly, is acceptable. Yeah. There are levels of the drugs. You would probably need a confirmatory test if someone had phenobarbital measurable, in which case you might want to get your flow study. But here… That's why we get the flow study. I'm sorry? Yeah. Right. Right. If you can't do it all with the apnea testing. Yeah. Okay. And likely they will ask you a question being inconsistent. Like Mike was saying, you know, the way the questions are said is like, from all these, which one is consistent with, right? Okay. Today all full-term evaluated for progressive sleepiness and poor feeding, point-of-care glucose within normal limits. Vital signs normal examination show lethargy and poor suck. Arterial blood gas, pH 7.48, PCO2 study, PO2 92, Vicar 23, CBC 12,000, hemoglobin 15, platelets 334, ammonia 672. Which of the followings is the most likely diagnosis? A, medium-chain acetyl-CoA, dehydrogenase deficiency, D, neonatal sepsis, C, arginine non-succinic aciduria, D, propionic acidemia, E, non-ketotic hyperglycemia, glycemia. Okay. Paul, what's abnormal there? What calls your attention? And then just answer. Okay. Okay. So we'll choose the correct one. So we keep moving so we can do all the questions. No, no, you're fine. I just put the... Yeah. Okay. Okay, pretty good. So this was the example of the patient with hyperammonemia and a normal pH. So it's hyperammonemia without acidosis, making it go in the direction of urea cycle abnormality. So acute encephalopathy, severe apnemia, inborn error metabolism is likely. Differentiating between the remaining... Let's see. Sepsis would be considered, but the normal vital signs sort of go against that. And one would have an ammonia of 670 or less. Yeah. Hyperammonia is most characteristic of urea cycle defects. That's arginine non-succinic aciduria. It's one of the defects within the urea cycle, but could also present in infants with organic acidemias and fatty oxidation disorders who tend to be more... tend to be more acidotic with both of those. Infants with propionic acidemia will have an acidosis. Fatty acid oxidation defects such as MCAD most frequently result in hyperglycemia. Also, those patients tend to be acidotic and to not have ketones. Neonates. So it's... That question seems to be reasonably straightforward. Okay. Okay. Five-year-old admitted to the PICU community hospital for observation. She was referred by her pediatrician, who is concerned about VP shunt malfunction. The patient was born at 26 weeks of gestation with a grade 4 intraventricular hemorrhage. The VP shunt was placed for hydrocephalus. She has residual development delay with a seizure disorder. CT performed on admission shows no interval change with small lateral ventricles. On PICU day two, the attending physician notices that the patient has episode of bradycardia while sleeping with mildly elevated blood pressure. On questioning, the mother says that the patient wakes up when stimulated, but remains irritable when awakened, and then insists on being left alone to rest. Her naps have been unusually prolonged. Pupillary responses are within normal limits bilaterally. And a repeat CT is unchanged. Which of the following is the best management approach at this time? A, continue observation, monitoring for concrete signs of caution trial, as a confirmation of diagnosis before any intervention. B, repeat CT daily until there is a clear sign of interval change. C, stabilize her and discuss with the neurosurgeon the possibility of transfer to an advanced facility with pediatric neurosurgical experience. D, obtain 24-hour EEG to rule out subclinical non-convulsive seizures. Okay. What was the other one, top patient? It's not there. Okay. Who has it? I don't know who has it. Oh. I don't think I was assigned it. Oh, okay. I'm sorry. I didn't know if you guys had it. Yeah. I think it was you. Oh, it was me? That's okay. So the correct answer was stabilize and discuss transfer to a facility with a neurosurgeon. And questions like this, certainly fair game, you know, sort of thinking back to your PALS of when to transfer to seek specialized care. So you have a patient with a VP shunt who has changed in neurologic exam. And so the priority is to have that patient in a place that can get definitive care if this truly is a worsening shunt malfunction. I believe that's probably what the rationale says. Right. And yeah. And you know, this one, the 2% of the people answer, I mean, even in the other facility, you will not continue, you know, just observation and wait until the kid shows you that she or he is down, down hill. Yeah. I've seen neurosurgeons say that. It's not a shunt. Yeah. Now, to be fair, if you are in a place that has a neurosurgeon, it wouldn't be wrong to get the EEG to see if that's an alternate explanation. And so that's, I think that was the other one that was the common answer. And the clue in the stem of that question is they purposely put out you're in a community where they pick you. Right. So it's like, that should alert you to like, oh, they're asking about transfer criteria, right? That's where this might be going. And in the emergency, somebody said tap the shunt. In the emergency, if I'm in an environment that I don't have anyone who can help me, I'm a community hospital, I will tap the shunt and then transfer the patient. But I don't think there is a need to tap it here. Okay. Previously, a healthy 12-year-old girl with influenza, pneumonia, and acute respiratory stress syndrome has just been intubated in the emergency department. She had been ill with high fevers for one week while her parents were seeking care from their chiropractor. They decided to seek traditional medical care when she developed increased work of breathing, said that she could not catch her breath, and begged to be taken to the hospital. In addition to the implementation of ventilator settings with a lung protective strategy, which of the following interventions will most likely increase her chances of survival? Oseltamivir, prophylactic amication to prevent bacterial superinfection, institution of internal nutrition within 48 hours, or dexmedetomidine instead of benzos for sedation. Always feed the child, always feed the child. This goes along with my desire to put a banner across the front door of the PICU that says feed the babies. Right. Yeah. So I have some problems with how the question was written, right? So the stem had a lot of red herrings out there in terms of trying to trip you up. And internal nutrition within 48 hours of, it's a little bit older data, but exam questions are a little bit older data. So there are multitude of studies with an association between early provision of enteral nutrition in the first 48 hours, and improved 60 or 90 day mortality in the PICU. But they are retrospective observational studies, we have no RCT data. So there's a little bit of like, will this really improve her outcome? Do we have definitive evidence that that would be true, is a little bit questionable. But there's a multitude of retrospective data. And then the other issues are, Oseltamivir, it's too late, right? We're later on in the course of illness, so there's no data to suggest that that's actually going to change her outcome. And then prophylactic amikacin, right? Influenza associated pneumonia in terms of poor outcomes, MRSA, I'm not so sure amikacin is your right choice in that setting. Sorry. And dexametatomidine instead of benzodiazepine for sedation. We don't know that that makes it, will improve her outcome. So it's kind of a little bit of a weird answer option. Right. So I'm not so sure it's the antiretroviral. Always feed the child. Previously, a 80-year-old girl presents to the emergency department after falling from her horse. She had multiple trauma, including severe traumatic brain injury, GCS-5, and pulmonary contusions with aspiration pneumonia. Abdominal RCT shows isolated grade 3 liver lack and multiple long bone fractures. She's currently under neuromuscular blockade, sedated, and mechanical ventilated on moderate ventilator settings. She's on Norepi at 0.05 microns per kilo per minute to augment her blood pressure to maintain CPP greater than 60. She's also on 3% normal saline infusion to maintain serum sodium between 145-150. Blood glucose has been maintained between 80 to 100 on main isabi fluids of 0.9 on normal saline with 20 milliequivalents of KCL. Hemoglobin has been stable at 11. She's not acidotic. And other than hypernatremia, her electrolyte levels have been normal. It is now 16 hours after the injury. Which of the following nutritional plans is recommended for her? Subpar internal nutrition, high in carbohydrate and low protein, subpar internal nutrition, low in carbohydrate, low in protein, and high in fat. Subpar internal nutrition is contraindicated. C, parenteral nutrition, low in carbohydrate, high in protein, internal nutrition is contraindicated. Wait 72 hours after her injury before starting nutrition, begin internal nutrition. Okay. You guys know this by now. Yay! All right. So, yeah. So, early parenteral nutrition, papanic trial, right? Early parenteral nutrition, not appropriate. And so that's why all of the first three answers are not appropriate. Wait 72 hours after injury before starting nutrition. I mean, honestly, clinically, no one would fault you with that as long as you're getting to goal nutrition within the first seven days. But the most correct answer is there's no reason to not feed this patient. So begin internal nutrition today. And most of the time when they ask you something, all these different options, and they talk about nutrition, the right answer is feed them, right? That would become a more controversial question and wouldn't be on the boards, right? So that becomes a more individual physician opinion. But the other markers you have there, she's perfused. She's not on norepi for hypotension or for resuscitation. She's on it for CPP. So presumably, perfusion is normal. So you should be able to feed them. And like we said yesterday in the trauma talk, I mean, it's not that you're titrating the norepi app, right? She's there, even if it's a lot higher than what they are telling you, but you are just to maintain CPP. Okay. We are tight and we have still several questions. So 40-year-old admitted to the PICU with vomiting and abdominal distention, past medical history is remarkable for intermittent bouts of self-limited pain, non-requiring hospitalization. She has had no prior surgeries, is fully immunized, and takes no medications. IV access is established, and 40 per kilo of normal selenium is given. Temperature is 39, heart rate 150, blood pressure 90 over 70, respiratory rate 24, and oxygen saturation is 99% in room AR. Standard exam shows dry mucous membranes, sinus tachycardia, lung, clear trascultation. The abdomen is distended, tender, without organomegaly or discoloration. Extremities are cold, capillary reflux is four seconds. Labs show a sodium of 136, potassium 2.9, chloride 101, bicarb 16, BON 19, creatinine is 1, glucose 78. And CBC shows a white count of 14, hemoglobin 15, hematocrit 45, platelets 500. The images shown below are most consistent with which of the following, malrotation with maggot volvulus, interception, rupture, MEKL, superior mesenteric artery syndrome, or incarcerated hernia. And that's the image. Okay, so those are the options. And here are the answers. Okay, I will move forward because we have to finish in one minute. So the correct answer is malrotation with midgut volvulus. And I agree. So the choices people picked, right, those are the two natural choices. And then ruptured meckles are like, what's on my differential for this description? The clues that it's malrotation with midgut volvulus are the pictures in terms of what they're trying to show you is the whirling sign where you're telescoping around the short pedicle in terms of malrotation with midgut volvulus. Could this be late intussusception? Sure. It's a little bit out of the age range classically for intussusception, which makes the midgut volvulus, which can occur at any age, more likely. The double bubble sign, right, I kind of gave a hint as, like, that can occur with multiple things. Duodenal atresia is not on this list, nor would it present this way for patients. But any sort of obstruction proximal is going to create that. Could it be intussusception? Yes. But the appearance of the whirling on CT is kind of diagnostic for the malrotation with midgut volvulus. Do we have time for one more, Teresa? Yeah. Do you see what time I can? Yeah. Go ahead. Go ahead? OK. Previously healthy lactose intolerant four-year-old child with no known allergies was intubated 12 hours ago for methicillin-resistant staph aureus associated respiratory failure and is on conventional mechanical ventilation. Which of the following is the most appropriate option for initiation of nutrition delivery for this patient? Gastric feeding tube placement and administration of soy-based nutritional formula, administration of parenteral nutrition with 1.5 grams per kilo per day of protein concentration, gastric feeding tube placement with administration of complete milk protein-based formula, or de-immune modulating nutrition with delivery of high levels of 3-fatty acid and glutamine. These are good choices people have made. So the correct answer is gastric feeding tube placement with administration of complete milk protein-based formula. What I did not share with you, well, I don't know why you would know it, is that medical formulas contain little or no lactose. And so it's like a red herring for you in terms of this lactose intolerant child, which is trying to steer you towards making the difficult choice between transpyloric, which you know, wait, I'm supposed to give gastric first, versus this lactose intolerant. So I'm going to choose the wrong route to get to the lactose intolerance, but in fact, medical formulas contain little to no lactose. When you want to have non-cow milk-based protein is when patients have cow milk protein allergy. And so, okay. And no, there's no evidence to support the use of immune-modulating diets in pediatric A medical mission to a developing African nation has been scheduled to provide ongoing medical support and education to a rural village approximately 30 miles from the capital. During a group planning meeting, a participant suggests providing pediatric intensive care services to the country's children. She has identified local pediatricians and nurses in the capital city hospital who are willing to participate. Which of the following considerations favors the development of a pediatric intensive care service? A, the child mortality rate is 55 over 1,000 live births, B, an orthopedic surgical program is being developed to treat spinal and limb deformities, C, the mortality rate of children in the region where vertically acquired HIV is climbing, D, the village does not have access to ball socks or continuous positive pressure, positive pressure device, and E, the local hospital is developing plans for an adult medical surgical ICU. Okay. So, here are all the options. I was assigned this, but I don't do global health and full confession. I didn't know the answer to this one either. So if we flip to the rationale, if you happen to know that there is a guideline somewhere out there that suggests that a reason to build a PICU is to support a surgical service, this is a great easy question for you. If you don't know that, then you're falling back on your test taking skills and process of elimination. So which of these things are different from the others? And so you had two answer choices that were mortality is increasing. How would you differentiate between those two? So maybe those aren't the answers because you can't pick one over the other. You had another one about, well, if you don't have access to a pulse ox, then you're probably not going to be able to build a PICU. It's going to be really far to go. The two left, if there's an adult med surge and is it reasonable to tack on a PICU with that versus the surgical program, hard to know, right? But at least you've got it down to 50%, answer something, forget about it, and move on. Yeah. Yeah, I agree. Yeah. Just remember, the orthopedists get whatever they want. Whoever brings more money to the hospital, right? And choosing the last one was that if they're building to establish an adult ICU, adding on something before they're already established will probably hinder development of that adult med surge. Oh, that's, yeah. So that's reasonable and goes into sort of your resource building. If they're already committed to the adult med surge ICU, let them finish that process before you also try to do a PICU all at the same time. Sort of the crawl, run, walk, or wait, I did that backwards, crawl, walk, run aspect of building things. That's a good thought, as good as any. Sure. If you have any questions about this in exam, it's going to be one question. Which of the following is an absolute contraindication to renal transplant in children? A, mild cardiac dysfunction, B, chronic hepatitis B infection, C, active sepsis, D, history of hemodialysis, E, mild intermittent asthma. Okay. The majority got it right, yeah. So yeah, for the most part, this one's fairly straightforward. The first thing you're going to do before you give someone a transplant is wipe out their immune system, and so you probably wouldn't do that in the setting of active sepsis. Now the thought about if you have chronic viral infection, that certainly would make you think and go, hmm, weighing risks and benefits, but probably not an absolute contraindication. In fact, that's sometimes an indication for a liver transplant as an example. So that's basically the bottom line there. And what was option five? Yeah, option five is mild intermittent asthma. That is a contraindication for nothing. That's a contraindication for nothing. OK, so we go to the next one. OK, 16-month-old boy with single ventricle physiology presents to the pediatric ICU after a prolonged cardiac arrest. He previously underwent stage two palliation. At this time, his exam demonstrate no signs of neurologic activity. Given the prognosis, his parents opt to redirect care to conform measures and request that life-sustaining support be removed. Which of the following most accurately reflects the use of an opiate medication given at the time of removal of support? A, the use of a medication that could suppress respiratory drive is unethical. B, patients who have suffered severe neurologic injury as shown by the lack of response seen on examination do not need pain control. C, the decision to use opiates should be left up to the family of the patient and be given as much as is requested by the family. D, the use of opiates is acceptable and is intended to ameliorate dyspnea and pain. I think this goes back to that question of the principle of the double effect, and everyone answered it very correctly, very well, and I'm glad for that. Certainly it is ethical to give medicines, even if they can suppress respiration to treat pain. We have no idea whether people with severe neurologic injuries can perceive pain. We have to use our best judgment, and leaving it up to the family is a little bit challenging, so I think the correct answer is there, and everyone got it. Good. Seventeen-year-old sustained traumatic brain injury in a motorcycle accident three months ago. After prolonged hospitalization, she's admitted to an acute rehab facility and is making improvement in activities of daily living. When her gastrostomy tube dislodges into her peritoneum, she becomes septic. She sustains a prolonged period of hypotension, and there is evidence of hypoxic ischemic encephalopathy on imaging. She's now intubated and on vasopressors. Her parents request that they do not want her to be resuscitated if her heart stops. Which of the following is the most appropriate course of action? A, review the components of resuscitation with the parents to decide what they wish for their child and re-evaluate treatment options with them frequently. B, inform the parents that it is not appropriate to provide intensive care without the option of resuscitation. C, enter a DNR order and stop the vasopressors since it is a component of resuscitation. D, urge the patients to discontinue all life-sustaining treatment at this time. Okay. Yay. Let's know that. Okay, 98% got it right. Okay. Go ahead. You picked this one out as well. I think that this type of question is a lot more clear now than it was maybe 10 or 15 years ago. I think if you asked this question among this population of intensivists 15 years ago, you get a different spread of answers. But in this case, reviewing the components of resuscitation with the parents and ask, try to find out what they want, what would be in the child's best interest, and re-evaluate those treatment options frequently is very, very important. Certainly, we make patients DNR all the time now and maintain mechanical ventilation, vasopressor support along the way. Okay. We have seven questions, four minutes. This is like divorce, you know. An immunized three-year-old boy is admitted to the pediatric ICU with history of ongoing tactile fevers, headache, and shortness of breath for five days. On admission, he has headache, chest discomfort. He is afebrile, both socks, 88% that improved to 92% when placed on a cannula. On exam, lethargic but without focal neurologic deficits and moderate respiratory distress. Labs show white count of 320, hemoglobin of 5, platelets 100, sodium 138, chloride 100, potassium 4.2, bicarb 18, creatinine 0.4, B1N10, glucose 60, uric acid 4, calcium 8.5, and phosphorus 5. Which of the following is the most appropriate next course of action? A, blood transfusion. B, CT head scan. C, allopurinol. D, leukapheresis. And we have the lecture yesterday. I just am going fast. Yeah, I can't see them. Okay. This one is a little bit tricky because, you know, certainly if we asked this question 10 years ago, the answer would have just been straightforward, leukapheresis. Now, there is more and more evidence, albeit mostly adult, that routine leukapheresis may not be associated with outcomes. And so, you probably might not be the right answer for routine hyperleukocytosis. However, what you would do instead, which is give a respirator case because you already have an elevated uric acid and start chemotherapy right away in order to immediately bring down the white count, isn't an answer choice. So, of the things on there, the best one is leukapheresis. So, what this is asking you to recognize is that there's hyperleukocytosis and it's symptomatic. You have neurologic symptoms and pulmonary symptoms. And those are your two most common organ systems that are going to be symptomatically affected by the hyperleukocytosis. And so, that is where your priority is to decrease the white count as quickly as possible. Like I said, the only choice here that you were given is with leukapheresis. Now, the blood product transfusion, little red herring, they gave you a hemoglobin of 5. If I were writing this question, I might not make it quite that tricky. I might give it a hemoglobin of 6 or 6.5. Clearly, this patient will require red cell transfusion at some point in time. But right now, it's probably not the priority. I think it gave you that you're hemodynamically stable. So, it's not an emergency to transfuse with red cells. And a red cell transfusion right now could make things worse by further increasing your hyperviscosity. And remember, a bag of red cells is also a bag of potassium. So, you have a patient with high risk for tumor lysis syndrome. And you could potentially worsen that with your red cell transfusion if given when it's not absolutely necessary. So, I think those were the choices. Okay. Yeah. So, there is only one question left. And so, we can do it quickly. And Ira is coming here to give you all the hints how to pass this exam. A 10-year-old boy with sickle cell disease presents to emergency room with fever, tachycardia, and severe pain in his extremities. His vital signs are temperature 39, heart rate 130, blood pressure 80 over 50 with a mean of 60, and respiratory rate 28. Referral blood count is drawn. A 20-per-kilo bolus is given. And he was on antibiotics and opioid. Review of the systems with the patient mother reveals that the child has been taking hydroxyurea medication. Initial lab studies are drawn. Which of the following is the most likely hemoglobin variant present? Hemoglobin F, H, A2, or E? Okay. Okay. I will just close it. All right. Fantastic. Most of you got this one. This one is if you know that the mechanism of action of hydroxyurea is to increase fetal hemoglobin or hemoglobin F, great. This is an easy one. If not, we're back to process of elimination. And generally, if they're asking which is most likely or which is most common and three of the answer choices are something you've never heard of, maybe it's not those as a rule of thumb. Not always, but a rule of thumb. And, in fact, those other three hemoglobins, H and E, are associated with thalassemia. H2 is a very rare variant of hemoglobin A.

Pediatric Critical Care

abdominal pain

altered mental status

metabolic acidosis

maple syrup urine disease

branched-chain amino acids

intralipid infusion

leucine restriction