Ludovico Guarini MD Pediatric Hematology Residency Beth Isreal Medical Center .. Fellowship: Coulmbia University .. Director of Pediatric Hematology at Maimonides Medical Center .. Castle Connolly Top Doctor .. DrMDK.com
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Male Speaker: Infections involving the joints sometimes? Dr. Guarini: They do, although it's not the most common problem and this about people -- Male Speaker: More but are they arthritic type of? Dr. Guarini: Yeah, what they tend to do is that they actually have bone problems, but their bone problems are more related what is so called sickle-cell crisis. And the sickle-cell crisis shows itself in different ways. For instance, the infant often has what is called a hand-foot; a disease, because there the bones in the hand and the feet, swell up and they are very painful, and obviously, a little baby who cannot communicate well, will be very cranky or very irritable without a true reason and occasionally, you see the swelling in the hands and the feet. Male Speaker: So these patients should be immunized against pneumococcal disease? Dr. Guarini: Absolutely. The advent -- they have been immunized against pneumococcal disease with something called the pneumococcal vaccine which was not conjugated, and that was given at age 2. Now, there is a pneumococcal vaccine that can be actually given much earlier, and it starts to work about age two months with a complete immunization by the age of one year. At the moment, it is not yet clear whether or not this immunization so early will eventually eliminate the need of pulling this patient on prophylactic penicillin. There is no study available at this time, and since the prophylaxis with penicillin is essentially freed of complication, the moment all patients with sickle-cell disease should be on penicillin. Male Speaker: Penicillin complexes conduct for about 50 years now, isn't it? Dr. Grimmy: The prophylaxis with penicillin, these are close to that, I mean it's been for a very long time. There was a very big study in the very early 80s, that is essentially established in terms of the way to go for this particular disorder. And one of the things that here is important to notice is that the kids with sickle cell disease use to die very commonly prior to this. While instead death in differs 20 years of life the patients with sickle cell disease. It's fortunately a rare event, a level that they are higher than the general population, but it is a rare event. Male Speaker: So besides getting penicillin giving the kid a vaccine that gets them -- would help these kids in treat? Dr. Guarini: Well, there are many things that one needs to do for instance, one of the most devastating complications of sickle cell disease is something called this as a stroke. The sickle cell disease affects the blood vessels and all other things, and young kids with sickle cell disease may have stroke. Instead there is a significant incident of this problem, the stroke is essential plug-in collusion. Male Speaker: What shall we do to prevent that complication? Dr. Guarini: Well, interestingly enough in the last 10 years, a lot of studies done on this, and there is a very good evidence, very good scientist that say that measuring the blood flow in some critical blood vessels in the brain through something called a transcranial doppler; which sounds terrible, but it's actually, it's a very simple and not in basic procedure. It is essentially an ultrasound at the blood vessels and we do ultrasounds for all kinds of things. That actually gives us a very good idea whether or not a person is at --

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