Wilson’s disease is a disease which is marked by defective copper metabolism. You wind up with far too much copper in your liver, your central nervous system, and also in the kidney. The liver and the central nervous system are the two that really present to you most often, in terms of clinical symptoms. Now on the lab tests, what are you going to see? The serum copper is low and the serum ceruloplasmin, which is the carrier protein, is low. You are going to do a 24 hour urine copper. That’s going to be really high. And you are going to have your local, friendly hepatologist do a liver biopsy and you will note that the liver copper content is quite elevated, and that’s the diagnostic test. So if you are worried about Wilson’s disease, get someone to do a liver biopsy on that child.
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The presentation can be all over the map. It can present as acute fulminant hepatitis. If it does, the tip-off is that these children have acute hemolytic anemia in association with their fulminant liver failure. If you see that combination, think Wilson’s disease until proven otherwise. They may present as a chronic active hepatitis, cirrhosis, portal hypertension, variceal bleeding. They may be totally asymptomatic with just a moderately elevated transaminitis. Some of these children do present with the neurologic symptoms more than the liver symptoms and they can be very subtle. Changes in personality, school performance, behavior characteristics that the parents come in and say, “This just isn’t anything like the child I used to know.” Be very aware of that. The Kayser-Fleischer rings, the brown pigment around the iris, is always talked about in the books. Very difficult to see and you usually are not going to see it until the mid-teenage years.
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The treatment for Wilson’s disease depends on when your diagnose is, which is why early diagnosis is essential. If there is no cirrhosis you are going to treat these patients with chelating agents, like penicillamine. However, if fully developed cirrhosis has already occurred, or they are presenting to you in fulminant liver failure, you have no other option but liver transplantation. There is another reason why diagnosis is so very important, because once you identify the index case you’ve got to screen those family members because you may be sitting on an asymptomatic sibling who you can treat very successfully with chelating agents, even if the index case is quite sick when you see them the first time.
There are five basic food groups, vegetables, fruits, grains, dairy products and the protein groups. What is an RDA? It won’t be uncalled for to see a question where they define it and you have to pick out the answer. “Levels for intakes for the central nutrients that, on the basis of scientific knowledge, are judged by the Food and Nutrition Board to be adequate to meet the known nutrition needs of practically all healthy persons.” It’s pretty vague, isn’t it? What’s an essential nutrient? Cheap generic medications at online canadian pharmacy: generic viagra, cialis, kamagra etc. That’s something that’s got to be provided exogenously usually from your diet because our bodies cannot synthesize it at a sufficient rate to meet the needs of our body. There are some things that we can make but we can’t make enough of and we need to get them from our foods in order to avoid having a deficiency.
What are the eight major categories of nutrients? All of these are considered nutrients, so there are eight that our body needs that are really essential. You can’t have a normally functioning body without all of them. Be aware that when we are born we are mostly water. But when we are young adults we become more and more fat. And when we are elderly, you are 70% fat. Total body water is affected, of course, by our age, our sex, our body composition and the total body water decreases with age and changes in body fat. All pretty obvious. The consequences of providing adequate amounts of nutrients without adequate amounts of fluid, dehydration, excessive renal solid load and an inefficient use and waste of calories. What are our daily maintenance fluid requirements for infants and children? Again, it’s not uncommon to ask really simple things about how much water infants or children at different ages might require per day. Again, you should be able to figure that out. There often are questions like, 10 kg infants should be given so much fluid per day and they’ll list a number, various numbers, and you’ll have to figure out what the water requirements are. Seems sort of simple but surprisingly some people don’t know it. Reasons why infants are more susceptible to dehydration? Large body surface area, obviously because they have a higher body content of water and they require more. And again because the kidneys have limited capacity to handle a solid load, it takes more water to basically excrete the solid load. Again, why the infants are more likely get dehydrated? Well because they can’t tell us they are thirsty. I guess they can cry and that’s an indication to us that they are hungry or that they are thirsty, but until a youngster can really vocalize we don’t often know. But that’s not too common. We also want to show you that oxidation of food stuffs produces water as an end product. It’s interesting that from fat you get more grams of water than you start with, but with carbohydrates you get less and from protein.
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Breast milk or infant formulas provide the majority of fluid requirements. Human milk, 89% of it is water. Oxidation of ingested milk results in approximately 90% of volume available as free water so you often have pediatricians and family wanting to give their children additional water. Well, if there’s a question about their nutrition, if they are certainly borderline, you either let them take more breast milk or more formula because again most of it is water anyway and when the nutrients get metabolized it forms even more water. Energy is provided by food, it is chemical energy obtained after digestion. I don’t know if you remember this, but the unit of measuring energy is joule or the calorie in what a joule is equal to, and the difference between a kilo calorie and the little calorie. It’s the amount of heat energy to raise 1 kg of water 1 degree centigrade. That’s a kilo calorie.
Smoke inhalation; fires tend to release very noxious substances. If you are burning plastics or some other substances you may have very toxic smoke. Early that is going to cause bronchospasm, some lung consolidation. As you go from a day or two, you are going to start getting pulmonary edema from capillary leak in your lungs, and as you get a little bit further out you are going to tend to get bronchopneumonia.
The therapy for this is entirely supportive. If they need airway management and ventilatory support, that’s what they get. Steroids have been advocated in the past, but in controlled trials they seem to be of no benefit and may increase the risk of infection. So steroids are no longer advocated.
What’s the problem with carbon monoxide? Well, it binds to hemoglobin very very avidly. If it is bound to hemoglobin, oxygen can’t bind to hemoglobin. So you get hemoglobin that’s all bound up and the only oxygen that you have left is that that’s dissolved in the plasma, which is very little oxygen. It also shifts the oxygen-hemoglobin curve to the left, and it, in and of itself, is a direct poison to the cytochrome oxidase system, so even if you did have oxygen around, your body couldn’t utilize it and you would be stuck with anaerobic metabolism.
So how do you diagnose this? Well, a history of exposure is helpful. If somebody is in a fire, it’s pretty straightforward and is something you would screen for. The problem is, oftentimes there is uncertain exposure. It’s in the home environment and there has been a dysfunction of a combustion device in the home. It may be in the middle of winter. Someone who doesn’t have a heating system and they are burning charcoal in a relatively enclosed space, which will release carbon monoxide and poison everyone.
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The symptomatology is dyspnea, headache from the lack of oxygen. They will start getting irritable as they get progressively hypoxemic, nausea, vomiting, dizziness, very confused, oftentimes very combative, leading to lack of consciousness, coma, and death. How do you diagnose this? A couple of things to be aware of. Your blood gas measures partial pressure of oxygen, so it measures your PO2. It doesn’t directly measure oxygen saturation. Your PO2 in your blood stream will be fine. The problem is that your hemoglobin has no oxygen bound to it. So your blood gas, as far as your PO2, will look fine, the calculated saturation that shows up on your blood gas will also be fine. How about pulse oximetry? Well, unfortunately carboxyhemoglobin absorbs light energy very similar to hemoglobin. So a pulse oximetry will mislead you into thinking your hemoglobin is fully saturated with oxygen. It’s fully saturated. The problem is that it’s with carbon monoxide. So neither a blood gas or pulse oximetry are helpful and they may be very falsely misleading. What you need to do is send arterial blood for what’s called “co-oximetry” where they will measure saturation, oxygen/hemoglobin saturations directly in the laboratory.
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Therapy; obviously you want to remove them from the environment in which they have been poisoned. You want to give them 100% oxygen. The half-life of carboxyhemoglobin is dependent on the PO2 in the blood stream. You are at room air, the half-life of carboxyhemoglobin is hours, like five to six hours. If you get somebody on 100% oxygen, you take it down to 90 minutes, and if you have a hyperbaric chamber that’s just sitting there waiting for a patient, you apply 2 ½ times atmospheric pressure and 100% oxygen; you get the half-life of carboxyhemoglobin down to 30 minutes. The other care is really supportive. Support the organ dysfunction, the neurologic sequelae, the pulmonary dysfunction that you may see secondary to the carbon monoxide poisoning.
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Heat stroke is a medical emergency. These patients need very aggressive cooling. This may involve packing them in ice externally. It may involve administering cool fluids to every orifice and IV site to bring down their body temperature rapidly. They need to be fluid resuscitated, they are essentially all dehydrated and vasodilated, and they need ongoing supportive care.
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Now there is a specific syndrome in young children that has been reported, of hemorrhagic shock and encephalopathy syndrome. Which is basically unexplained severe hyperthermia in young children without an infectious etiology defined, which is associated with a very significant encephalopathy. These patients develop shock, DIC and actually have a high mortality rate. In the initial series, 100% mortality rate was reported. Now survivors have been reported, but it still does have a high mortality rate. It is uncommon but has been the source of questions in the past.
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Smoke inhalation; another environmental disaster. If you happen to be in a fire you get asphyxiated. Fire consumes oxygen, it leaves what you are breathing to be relatively oxygen-free, and although you are having air exchange you are not getting oxygen to your tissues. You may have a thermal injury, although this is a much less common cause of death, where you actually have flame injury to the tissues. A thermal injury with super-heated environments, gases especially if there is steam involved, you may get airway injury from the high temperatures. This is typically supraglottic. By the time it gets down to your lungs the gases have usually cooled enough that you don’t have burning all the way down your lungs. You may begin to develop airway edema. If you have a child who has stridor following a burn injury, they have soot in their oropharynx, you want to establish an airway quickly before it swells shut. So burns to the lip or nose, soot in the mouth, singed nasal hair, stridor, hoarseness or respiratory distress, are all indications for intubation. Don’t wait and hope that they are going to get better. They are going to get worse before they get better and they need an airway.
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Carbon monoxide; combustion releases carbon monoxide. When it’s in a fire it will be associated with soot and other things. You can get carbon monoxide poisoning in other environments. Exhaust systems of furnaces may dysfunction and everyone in the home may in fact have carbon monoxide poisoning. It’s a colorless, odorless gas so they won’t know it until they are losing consciousness or having changes. We are going to talk a little about the pathophysiology, the diagnosis and the therapy. Again, it’s colorless, odorless, non-irritating gas. Sounds like a pretty good way to kill somebody, to me.
Prognosis; well, not surprisingly, the longer you have been under water and the longer you have been dead, the more likely you are to stay dead. So if you have been underwater for a long time you don’t do so well. If it’s taken resuscitation a long time to get your heart going, you don’t do so well. If you are still apneic by the time you get to the emergency department, you don’t do so well. If you are still pulseless, despite ACLS coming into the emergency department, you don’t do so well. People who are going to do well from near drowning typically have relatively rapid neurologic improvement. Even though they got CPR at the scene, you see them six or eight hours later and they are reaching for their endotracheal tubes and trying to pull it out. In the absence of neurologic improvement over the first day, their outcome is relatively dismal. The degree of metabolic acidosis, in some studies, has been shown to be a helpful predictor in outcome.
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Okay, moving on to environmental exposures. We see environmental exposures very rarely in the ICU. The Board questions almost always have one of these topics reflected; heat stroke, smoke inhalation, or carbon monoxide poisoning.
Heat stroke; heat stroke basically refers to a very high temperature, oftentimes as high as 106 or 107 degrees, where bad things begin to happen to the enzymes in your body. How does this happen? Well, you can have increased heat generation either from pyrogens generating a fever, exercise if you go out to run a marathon across Death Valley, not surprisingly your body temperature rises. Or if you have hypothalamic disregulation problems. Some children with severe neurologic injuries don’t have good hypothalamic regulation and they don’t regulate their temperature well. So they have increased generation of heat. Usually both problems of heat generation and heat dissipation are involved with the development of heat stroke. You can impair heat dissipation with bundling. Bundling decreases both convection and conductive losses of body temperature. If you are in a very hot environment, you have decreased radiation losses to the environment if it is very humid. So if it’s very hot and humid you have decreased evaporative heat losses to the environment, and if you have a congenital difficulty with sweating, you don’t want to go live in a hot environment and get overheated.
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What’s the pathophysiology of heat stroke? Usually you end up getting dehydrated. When you get dehydrated you have hemodynamic changes. Typically what the body would do with hypovolemia is vasoconstrict. Because your temperature is high, the body is trying to unload some of the heat, and what happens is that the body vasodilates. So you have low intravascular volume and you vasodilate. You can imagine that your hemodynamics don’t stay very good and you get very hypotensive. You may have tissue damage from the hypotension, and also directly. As your body starts hitting 42 degrees or above, 106 or 107, you start having enzymatic dysfunction, direct injury to the cells in your body, and a lot of secondary injury to the body. You’ll have altered mental status, you may develop ARDS from some of the pulmonary injuries, and some of the activation of some of the mediators within your body. Myocardial ischemia as you drop your blood pressure, you are tachycardic because you are very hot and your myocardial oxygen supply is very poor so you get myocardial ischemia. You get tissue breakdown of your muscles, with rhabdomyolysis, which gives you secondary renal failure. You develop DIC, hypoglycemia, hypocalcemia, and you end up dead.
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