Well talk about management of indirect hyperbilirubinemia next. First thing is hydration, and I don’t’ care what the cause of indirect hyperbilirubinemia is, if you have a baby who is coming in with levels that are potentially approaching the exchange transfusion, you want to hydrate these kids. The key thing is that there is - unless they are very dehydrated or have some contraindications - the way you want to hydrate them is orally. You don’t want to give these kids IV fluids. Again, the reason you want to do that is you want to use the benefit of the enterohepatic circulation and get that stool and that bilirubin out of the gut as quickly as possible. So we’ll try to feed aggressively, bottle supplement if they are on breast feeding, and then consider even NG tube feedings if they can’t take in enough fluids. Another thing that I’ll do, a nice trick, is actually if they haven’t had a stool in awhile just give them a glycerin suppository in the hopes of increasing the stool output that you may get and get a bilirubin drop in that kind of way.
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The second is phototherapy. You aren’t probably going to get asked this on the Boards because it’s fairly controversial, not a lot of great studies recently. I think most people at this point are using different criteria for hemolytic and non-hemolytic hyperbilirubinemia. Full term babies, hemolytic, I think the goal is still to keep bilirubins less than 20. So if you have bilirubins that seem to be approaching 20, you may want to start phototherapy. With the advent of home phototherapy it often makes our life easier in terms of getting that accomplished. Non-hemolytic is .. I think people are probably willing to accept levels of 25 or even up to 29 or so without getting too alarmed. I tend to be a little more aggressive. So if you are getting bilirubins up into the 20’s even with early breast milk jaundice, I will go ahead and start phototherapy. Relatively benign intervention overall.
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The third intervention is one that we hope to avoid if at all possible, which is an exchange transfusion. Again, hemolytic, if you have levels that get much above 20 and you can’t bring them down with phototherapy, or you start phototherapy and they continue to rise, then you need to consider an exchange transfusion. If you are dealing with premature infants then you are going to need to think about using lower levels at which to do exchange transfusions. Non-hemolytic, very controversial. I think people have said that there has never been a case of kernicterus in early breast milk jaundice, and apparently there is one report recently where a level was 40 and a baby developed kernicterus. So I’m not sure we can say that absolutely, but it seems at least that kernicterus is quite unusual, if not virtually impossible in breast feeding jaundice. So because exchange transfusions have such high morbidity and really relatively high mortality rates as well, especially as we are doing them less and less often, it is probably … you really want to avoid doing an exchange transfusion for a baby who has jaundice just from late onset breast milk jaundice. Canadian viagra
Then the enterohepatic circulation, which we talked about briefly before, the most common cause of problems with enterohepatic circulation and reabsorbing too much bilirubin is breast-feeding jaundice - some people call it “early breast milk jaundice” - where there just isn’t enough breast milk getting in. What happens is that anything that causes slowing in passage of stool through the gut will lead to hyperbilirubinemia. Some of those things could be poor feeding, perhaps from neurologic problems, or if the baby has cleft lip and palate and is having difficulty with feeding, that could contribute. Vomiting, things like a partial obstruction, achalasia, those kind of problems could produce hyperbilirubinemia, or anything that causes decreased stooling. Meconium plugging would be an example of the things that could cause decreased stooling.
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Then on to decreased clearance. Still we are on indirect hyperbilirubinemia. Premature infants will have decreased conjugation by liver enzymes. They just don’t have enough liver enzymes around to handle the bilirubin load. In full term babies you actually can see it. In premature babies glucuronyl transferase deficiency. This is one that there’s no easy way to remember it. You just need to memorize this one. Crigler-Najjar is type I and that is autosomal recessive and has a poor prognosis, largely because the enzyme is completely absent. Type II glucuronyl transferase deficiency is by contrast autosomal dominant, generally fairly benign and in that one there is not an absolute loss of enzyme. It’s more just a deficiency of glucuronyl transferase. One more glucuronyl transferase deficiency is Gilbert’s, autosomal dominant and also fairly benign. An interesting problem, one that I’ve never seen but is kind of one they may ask about on the Boards, is Lucey-Driscoll which is a maternal gestational hormone that interferes with conjugation. Because this maternal factor is transient, the problem is going to be transient but can produce fairly high levels of bilirubin.
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Inborn errors of metabolism, galactosemia, tyrosinemia, can interfere with clearance and the important thing here is they may start as an unconjugated hyperbilirubinemia but can become conjugated. So they will show up on both your differential diagnosis, both for indirect hyperbilirubinemia and direct hyperbilirubinemia. There are endocrinologic problems, hypothyroidism, hypopituitarism, these two can present in the conjugated form as well. Then late-onset breast milk jaundice, in contrast to the breast-feeding jaundice, this isn’t a problem with the amount of breast milk that is there. It seems that there is an intrinsic factor in the breast milk that causes hyperbilirubinemia and again is not related to adequacy of nutrition. Generally what you will see with this late breast milk jaundice is it won’t present in the first week of life, it will present more in the second or third week of life. Another thing to note about the early breast feeding jaundice is the timing. You should see that peaking somewhere around 3-7 days. If you see a baby that is getting dramatically jaundiced in the first day or two of life, don’t call that early breast feeding jaundice. I don’t care how well the breast feeding is going in the first day or two of life, babies are not getting a lot of breast milk. So it doesn’t matter even if breast feeding is not going well, it shouldn’t contribute to markedly high bilirubin levels. I’ve seen that mistake made a number of times where people will call bilirubin that is in the mid-teens at 36 hours of life, say “Oh, that’s breast milk jaundice.” No, it’s not.
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Just a word about management of early breast feeding jaundice and later breast milk jaundice. The important thing is that there is absolutely no reason to stop breast feeding. As a matter of fact you want to kind of continue to encourage breast feeding unless they have tremendously high levels, and you want to make sure you are getting in as much as possible. All you really need to do is supplement with formula. So you can breast feed then give formula afterwards. Whether you want to give it in a bottle or syringe is up to you. But the important thing is there is no reason to stop breast feeding.
Eating disorders. Anorexia versus bulimia. Anorexics are those really really thin women. They are about 5’8″ and they weigh about 80 pounds. Bulimics are normal weight and sometimes overweight. The anorexics starve themselves, the bulimics binge and purge. They eat all these Twinkies; they eat all these donuts and then the throw it all back up. The anorexics have to weigh like less than 85% of their normal body weight, and they have to have missed three consecutive periods, if they are post-menarche. Bulimics just basically do the eating and then the purging. Now there’s all kinds of funny variations. There’s anorexics who will purge, there’s bulimics who will starve themselves. Sometimes they won’t do the bingeing and purging, sometimes they will use laxatives or diuretics. There’s a thing called Russell’s sign, which is scraping the back of their palm in bulimics. What’s that from? Making yourself throw up. They remove tooth enamel, they become hypokalemic, bulimics do.
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Two zebras: this is important, under eating disorder differential. There’s two zebras, particularly I think for neurologists. One is Klüver-Bucy. When somebody loses their temporal lobes because they had such bad seizures, the neurosurgeon had to take them both out. Or they have something like Herpes encephalitis and they lose a temporal lobe. They get this syndrome where they are hyper-oral, hyper-sexual, constantly masturbating or constantly mounting things. It’s really a tragic disorder. Loss of rage, sometimes loss of memory, very docile. And also Kleine-Levin syndrome. Classically adolescent males with hyper-somnolence and bingeing. The way I tell people to remember it is to think about adolescent males and what they do normally; sleep a lot and eat a lot. Right? Just multiply that tenfold and that’s Kleine-Levin. And there are mental status changes that you will see with that. Patients may also be confused or psychotic.
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Adjustment disorders. An adjustment disorder is having so bad a to a situation that you are suicidal. Post-traumatic stress disorder is the opposite. Post-traumatic stress disorder is you see something that’s not normal. Unfortunately, in some neighborhoods to see a lot of violence and stuff like that. But in post-traumatic stress you see something that would rattle anybody. You are raped, or you are assaulted, or you see combat, or an accident. That would disturb anybody. Adjustment disorder, you have something that everybody has had. You know, loss of a job, something like that. A breakup of a marriage or a relationship or an engagement. Something that’s pretty much part of the normal human experience but it goes beyond normal sadness. Your adjustment to that is terrible. You are suicidal or you are just intensely anxious, more so that your average person would be.
Gender identity disorder. How does that differ from transvestic fetishism? The male or female is uncomfortable with their assigned biological gender. What does that mean, assigned biological gender? Just means what you were born as. “I was born as a man and I feel like I’m trapped in a man’s body. I’m really a woman.” Or vice-versa. Often in childhood. This starts early. The kid may start dressing in the opposite gender clothing and they just kind of always felt like they should have been a little boy, or they should have been a little girl. And eventually they save up their money and commonly have sex reassignment surgery, a sex change operation. Sexually these people may be attracted to men, women, both, neither. It’s very independent of homosexuality or heterosexuality. It’s not who I’m attracted to, it’s who I am. Now classic Boards question is “How do you differ this from transvestic fetishism?” The person with gender identity disorder, that guy, he’s dressing like a woman because he want to be a woman and he feels inside that he is a woman. The transvestic fetishes patient, he’s dressing that way because he feels sexy, he feels aroused when he does that.
Homosexuality has been a controversy among psychiatrists for decades, even Freud addressed it. He wrote a letter to a homosexual patient’s mother and talked about it, “You know, it’s not that bad” and stuff like that. Currently it is not considered as pathology, per se, by the American Psychiatric Association. The APA came out and said this is not pathology. There was a time in history when there used to be what was called ego-syntonic versus ego-dystonic homosexuality where gay men who were happy with themselves were ego-syntonic, gay men who were not happy with themselves were ego-dystonic. People who have done research in this area have come to the conclusion that most gay men have what is called a “coming out” process. That is a period where they are unhappy with their sexual orientation before they accept their sexual orientation. So they got rid of that ego-syntonic, ego-dystonic stuff.
Sexual and gender identity disorders. Sexual dysfunctions: problems with desire, orgasm, function and pain. Always consider medical causes. Don’t say, “Gosh, you have this repressed hostility and that’s why you are impotent.” Well, maybe you have diabetes. Thioridazine, Mellaril. Remember there’s three Board important things about thioridazine or Mellaril. Trazodone. What can trazodone do, or Desyrel? Priapism. Painful erection that you can get with trazodone, which is an antidepressant. In reality, you don’t really see a lot of priapism with that. You’ll see women come in and say, “You know what? I can’t have an orgasm anymore since starting Prozac and this is ruining my relationship.” Or you’ll see women come in and say, “You know, I’m not interested in sex anymore, and this is really okay because I don’t like men. Men are a pain in the butt, I’m not in a relationship and actually it makes my life easier.” Similarly you will see men who will say, “Since I started Prozac it takes two hours for an orgasm, and this is terrible. It’s ruining my relationship. I don’t even want to have sex anymore.” Or you’ll have men come in and say, “You know, sex used to be five minutes and now it’s 35 minutes. Sometimes you will see SSRIs used for premature ejaculation specifically.
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Paraphilias. paraphilias are what used to be called the perversions. Commonly associated with males. Include exhibitionism. Guys like to expose themselves and they get sexually aroused by doing that. Fetishism. This is sort of like where a person is into boots, or leather, or rubber, or something like that. Now people will ask, and patients will ask, “Gosh my husband really likes to see me wear boots. Is that pathology?” and the answer is, not necessarily. It’s pathology when the wife comes in and says that the “Husband likes the boot more than he likes me.” Or, “We can’t just have sex. We can’t just make love. It’s 2 o’clock in the morning and I’ve got to put on those damn boots, otherwise he can’t …” It’s a problem when either the spouse or the partner or the law says it’s a problem. Frotteurism (?): rubbing against a non-consenting partner, like on a subway or an elevator. Obviously that’s always going to be a problem because you’ve got a non-consenting partner. Pedophilia: obviously always a problem, sex with children. That’s always considered pathology. Sexual masochism, “I like to be tied up.” Sexual sadism, “I like to tie my partner up” that’s pathology if it interferes with your relationship or the law. But you know, a lot of people will come in and say, “Yeah, I like to tie my wife up, I like it, she likes it.” Not necessarily pathology. Voyeurism: people will come in and say, “Hey, I like to watch Baywatch. Is that pathology?” No. It’s pathology if you have binoculars and you are looking into your neighbors windows and you are now on probation because of your behavior.
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Transvestic fetishism, what used to be called cross-dressing or transvestism. Paraphilia, not otherwise specified, like bestiality, sex with animals, necrophilia, sex with corpses, and telephone scatologia, making dirty phone calls. Transvestic fetishism is a person, usually a male, classically heterosexual. He likes women and likes to wear women’s clothing.
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Generalized anxiety disorder. Chronic excessive worry about real life events. Okay, it’s not like a phobia where somebody is worried about … they see a praying mantis and they kind of freak out. This is like, “I’m worried about my job, my finances, my health, my parents health, my husband’s health.” It’s what we all do but they do it so much and so often that they really need a psychiatrist because it’s really getting out of hand. Health care mall
Agoraphobia is not a specific phobia. A specific phobia is a thing that isn’t a social situation. It has nothing to do with people. If it’s people it’s a social phobia. Specific phobia is airplanes, blood, bats, trains, tunnels, thunder, dark, water. It’s things like that. Spiders, snakes, insects. Agoraphobia is … now if agoraphobia wasn’t associated with panic disorder they might have thrown it in there but agoraphobia, literally speaking, is fear of open places. But in reality it plays out as fear of going outside your home and mixing in the world. Sometimes you can see it without panic attacks, but really classically, and I think for the Boards, it’s very associated with panic disorder. The agoraphobia usually goes hand-in-hand with panic, because it’s secondary to the panic, classically at least.
Free floating anxiety: I’m nervous and I don’t know why. I’m feeling anxious and I don’t know what I’m anxious about. “What are you worried about?” “I don’t know.” One of the things to screen for is first of all, if it’s panic or not panic. That’s definitely an important thing. If there’s no panic attacks - in other words they are not having the tachycardia and palpitations - just “I’m feeling nervous and I don’t know why.” You definitely want to do a medical workup and make sure they don’t have thyroid or something weird like carcinoid or pheochromocytoma. A lot of people come in with this free floating anxiety and what you do is send them to the psychiatrist and the psychiatrist puts them on Prozac, starts them in therapy, has trouble coming up with a diagnosis, and then maybe it gets better and maybe it doesn’t. That was back in the old days with the Freudian cartoons where the guy comes in and sits on the couch once a week and talks about his childhood. That was supposedly what that was supposed to treat, that sort of free floating anxiety. But it doesn’t really have a neat name anymore.
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