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About the Fyssas. Okay. And the main learning objectives are, you know, we're going to go through the structure of the Fyssas, go through some clinical clinically important uh areas and then how to draw the Fyssas. And then I have a few slides on Faisal injury, the Fyssas, you know, has reasons to love. Uh And then I have a few slides for reason for, for trouble. Now, if you look at these x rays there, um can anyone take me through what, what's happening there? Just it's not a trick question, but just to kind of wake everyone up this afternoon. So these X rays are taken ap and axial over a span of one year or nine months. Can anyone think what has happened to this patient or any any volunteers? Yeah. Yeah. Proximal proximal humerus fracture with through the Fyssas. Um That's then gone on to remodel and looks very nice. A year later, I can't quite see the detail enough to see what kind. I mean, what type of salt Harris it is, but it looks like there's a little fragment. Uh Yeah, I think so. So. Um so do you think question one? Yes. It's gone on to remodel nicely, but look at the dramatic jump. Uh Do you think this patient has had a manipulation? No, sir. Uh approximate humorous fractures in Children, I think are notoriously forgiving. And so I think, uh this has just been left alone. So I think that that initial left hand uh series, I think probably shows the initial displacement and then the accepted position and then it's gone on to remodel from 2 to 3. And actually, it looks like someone has almost reduced it from 2 to 3 because it's, I mean, I know there's extra callus there as well, but it's, it's just shifted from a displaced to an undisplaced position. It looks really good. Yeah. So, so that's exactly, and that's another reason to love. So you actually don't have to do anything. You just have to wait and watch and it goes back to normal. And if you were to do an X ray, a couple of years later, you wouldn't even know there was a fracture there. Uh And then there's, there's no magic like this. You can actually, you know, be uh slews and identify, you know how old the patient is based on their X ray. And there are more formal methods, you know, for example, looking at the development of the elbow joint uh or look at wrist x rays or even if you have a simple elbow X ray, you can make a fair guestimate on how old a patient is based on, on when these uh faisal or, or epithelial uh ossification centers develop and then there are reasons for trouble. I I don't say reasons for hate because hate is quite a strong word. Um But then there are reasons for trouble. Um And again, no trick question here. Can someone describe what's going on this patient? Got bilateral say Pfizer or is it just on the left hand side? Uh Good question. Yeah. So how, how would you, so you can see uh the left hand side definitely looks terrible, right? But even the right side is quite a severe slip. Okay. So this classical slip, uh upper femoral epiphysis and this patient uh had been walking on this for almost a year, had been symptomatic for a year uh and was having bilateral hip pain. And then finally, the left side was the one that really uh was causing her a lot of trouble and that brought her to the hospital, you know, so, you know, this is again a very challenging condition, you know, lots of complications with surgery and lots of controversy as well as doing the appropriate management. Um And then this does anyone know what this is? Yeah. So this is Blount's disease, you know, and again, you know, so just as forgiving, you know, the vices is, it can also be extremely challenging um as well. Uh So it's important to understand what the, the the various structures are that make up this magical um organ. So uh you start off with a layered structure. So this, so whenever we described, if ISIS we describe the epiphany this at the top, the meta facist uh at the bottom and we go through the various zones there does this project clearly because for me, it's quite clear. Can you see this? Yeah. Okay. All right. So you start off with the reserve zone. Okay. And in this zone, uh you have uh cells that are sparsely populated. As you can see there, there's a lot of extracellular matrix. Um And generally, you have this is where the cells store all the material that is required for rapid proliferation. Uh And this has the, the, the uh the precursor cells for the chondrocytes. Um And, and they live live in this zone and this is a really, really important zone. Uh And if you have problems there, you generally have these storage disorders like gosh a disease or you have various kinds of dysplasia is like diastolic dysplasia or niece syndrome. So, whenever you are describing the structure of the Fyssas, if you can include some clinical context, that would be nice as well, um then the second zone is the proliferative zone. So these, these cells then start proliferating and they kind of layer themselves up in this column nor fashion. Uh And they stack up longitudinally. Uh there's an increase in matrix production and there's some increasing oxygen gradient because obviously they do require some energy uh with the rapid proliferation. And in this is the zone where you have uh conditions such as gigantism, exhaust osis or achondroplasia uh affecting the growth. Uh these cells. Yes, then you have the hypertrophic zone. Uh This is where the same cells now start increasing in size. Um And the hypertrophic zone is made up into three layers. You have the zone of maturation zone of degeneration and the zone of provisional calcification. Okay. And in this zone, you have quite high volume cells reduced extracellular matrix in comparison. And this is the zone where most of the injuries. So five still injuries, fracture injuries, they occur at this zone, uh slipped femoral epiphysis as well. Uh And this is uh this, this zone is also affected by vitamin D deficiencies are Ricketts and mucopolysaccharide uh MPS disorder orders Asian so far, uh can someone explain to me why is this on the weakest of all the zones, less extracellular matrix at that level? Because it's so cellular. So yeah, exactly less strength. So, so uh fracture forces are more likely to traverse this stone. And is it bad? Where would you rather have the injury if you had a choice if I see? Well, that that's that side not going through the uh at the top of the proliferative zones are not affecting those, those mother cells replicating from top down. So, salt Harris three fours are bad for that reason. Yeah, exactly. And, and then you proceed to the, you know, transitional areas where you're then getting into osteoid deposition. So you have uh you know, vascular invasion from the uh from, from the, the vessels from the sinusoids in the primary secondary spongiosa that bring the necessary minerals that, that then mineralize the collagen scaffold that has been laid down by, by the chondrocytes. And uh we know that uh vitamin C deficiency can affect this layer because uh vitamin C deficiency can affect the vascularity. Um And so, uh when you have scurvy, uh you see uh sclerosis in, in the primary spongy also. Uh and then you have secondary spongiosa uh where you have the internal remodeling. Uh the cartilage scaffold is, is removed and you have uh normal bone that has been laid down. And uh in patients with renal osteodystrophy are, you know, renal uh failure, you get the renal sufi occurs through this zone because uh it's the osteoid deposition that is affected uh due to the metabolic changes. And that is then that becomes the weakest zone. Um Any questions so far? All right. So actually, I've just got a quick question. Um So when you have renal disease doesn't affect the zone of provisional calcification or is it the primary Spahn geos to that effect? Uh So in, in my reading, I had it that uh it affected the uh that's a good question. I'll have to double check because in my reading, I had it that it affected the secondary spongiosa secondary. Okay. Yeah. But you know what? I, I don't think it was the, the zone zone of provisional classification. Uh because when I was, I was reading through it, let, let me double check my source that I have and I'll get back to you. Sure. Okay. So in the exam, if you're asked to draw this, you uh this is kind of a simple schematic. I've taken this from the Ramchandra in Basic Science book. Uh And this is, this is quite a nice and easy way because you don't have much time, you have less than five minutes to describe all of that. Uh And so if you mentioned uh you know the various clinical features, uh you know, uh interesting clinical uh attributes to each zone. Um and then just go through the resting zone, proliferation zone, the hypertrophic zone uh with your maturation degeneration, provisional cancer, uh provisional calcification, calcification and your primary and secondary spongy also. Um So this is kind of a nice and easy way to, to draw that. I I do recommend that you go through the relevant chapter in the, in the Basic Science textbook. So, Faisal injuries is pretty common. Um it can represent 15 to 30% of pediatric fractures. Um As we discussed, major injuries are through the hypertrophic zone and there is a risk of uh injuring the growing zones and vascular elements as we discussed previously. Um uh and always remember that the standard complications of non faisal fractures also apply. So you have problems with infection, problems of bone union malunion compartments in Roman stiffness. So always keep that in mind. Uh And then there are specific risks uh you know, uh that have growth, asymmetry, uh slow growth or faisal arrest or abnormal growth. So a lot, a lot of people know the soldier Harris classification. A nice way to remember this for me is you, do you remember the first letter of S uh starting from the first letter S A L T E R? So soldier Harris one is straight across. So for s assaulter Harris to fracture, uh the fracture line exits above uh and this is, in this case, we're looking at uh in worded limb. So the the Fyssas is below and above. Meaning the fracture is crossing the metaphysis. So it is crossing the hypertrophic uh and then the primary and secondary spongiosa. So those fractures are more forgiving. Uh Soldier Harris three is a fracture that is going across the epiphany Asus. And so it's not only the Fyssas that is involved is also the resting and proliferative zones, but also the joint surface is affected as well. And so these fractures are a little less forgiving and do require accurate anatomical and stable uh fixation. And then a salter Harris four is when it's going through and through. So, so we're down to the letter T and that's going through and through across and then a Salter Harris five is a crush injury. Uh And I remember that as E for elephant, uh there is a Salter Harris six that has been later described uh and you may find it in other sources and this is when a bite out of the perricone ring of LYCRA dimension earlier over here, uh which is a, a thick band of periosteal tissue with its vascular supply, uh can get pulled out and cause uh quite a devastating injury to the Fyssas. And so assaulter Harris six injury is quite bad uh because not only do you have all the zones affected but also a stabilizing portion of the periosteum is taken away with its blood supply. Uh So, uh and it is also e centric. So you end up getting an angular deformity uh which can be quite bad. Um Any questions. So, um this slide just taken from uh one of our uh textbooks for, for pediatric orthopedics. Um And after faisal injury, you can have a sluggish growth and it can be visible on uh growth arrest lines as you can see above. Um you can also have a leg length discrepancy as well. And occasionally you can also have uh formation of seal bars which can be quite troublesome, especially in bones that grow together like the radius and ulna. You can see that you get a significant amount of positive ulnar variance and so this can cause mechanical problems. Faisal bars can be, can be classified uh in many ways over here, you can see. So, you know, a type A uh is a peripheral type, type B is a central type. Uh and then type C is, is kind of a traversing uh type. So um uh I find these, you know, these classifications um uh you know, more, more for uh academics rather than um uh important clinical application. What you need to assess is where the bar is and what sort of growth arrest or growth disturbance you're going to have. And then is it possible to excise this bar in a safe matter to then allow the rest of the Fyssas to continue growing. Uh um So if you have uh so, so the various treatments that we can offer uh and one of the things is if you're going to have a angular deformity, uh and if the limb is long enough, especially in, in patient's who are close to skeletal maturity, you can perform uh an epithet. So DCIS, which is just obliterating the uh entire growth plate uh so that you prevent an angular deformity. Uh Essentially, if you have a faisal bar of greater than 50% then you know that a lot of the is not going to grow. And so that may be a reasonable option. Um And then you may have to rely on a limb extra faisal kind of limb lengthening, you know, through frames or, or uh external devices uh to help achieve um uh the uh the growth and and kind of limerick construction. Yeah, chondrodystrophy ASIS is also another option that can be used where an external frame is is I've never seen this. I don't have much experience of this. Uh but there is a risk of premature mature faisal closure. And then there are some papers that report the risk of premature faisal closure is quite high associated uh with this uh sort of of treatment. Um Other papers have also mentioned that it is associated with low faisal function and slow growth. Um And it's usually recommended in adolescence, you shouldn't do it in very young people, people. Um There are techniques that we can use to excise the faisal bar. Um uh going through sort of an extra faisal approach as this uh this picture types uh usually um useful when the faisal bar is is less than 50% around 25 to 40%. Um if you have adequate growth remaining. Um uh And if you have an infection, then it should be at least one year free from uh any, any drainage. Um The results you can have, you know, are you can have arthroscopic assisted faisal bar excision, uh generally have relatively good success rate. Uh And then if your bar is small, you know, you generally tend to have a good result if your bar faisal bar is large, you generally have poor reported results. Uh In this paper by Staheli, they found that the mean growth uh they could achieve was around 83% of the estimated limb growth. And the main cause of uh fail inadequate bar resection. Uh and recurrence of the bicycle bar because you are in a hematoma there. So there is a potential that the uh the bark and reform even if you plug some fat in, in that area. So in summary, uh the stock um I don't want you to focus too much on the management of this. The, the important bits that you need to know for the exam are know your zones. So your resting zone, proliferative zone, hypertrophic zone. Uh The three subs owns the primary secondary spongiosa. And remember that there are reasons to love and reasons for trouble and haters do stronger work. All right. Any questions so far? Um uh Mark, if you, if you don't mind, I have another lecture to give elsewhere. Um And I was hoping that I could just carry on with my, with the last talk which was on Collagen. And oh, I would that be okay. It's my talks are not as long as described. I have to do the school run later but it's all your yo yo's okay. Yeah, I won't take long because I have to be somewhere else at three. Uh So uh you know, so I'll, but I'll finish. Well. Well, before. So it's, it's about, it's about 20 more minutes. Is that okay or if you need to go? Okay, I'll go ahead. Okay. Um Okay. So I've been asked to talk about collagen and osteogenesis, imperfecta. Okay. So the learning objectives of this, of this talk are we're going to talk about collagen, how it is formed, how it is synthesized. We'll go through some common types and then we'll go through some uh important kind of clinical information and try and get a sense of what? Oh I or the spectrum of uh conditions that that form. So I will go through the genetics, the common classification, you know, we'll go through the phenotype and then we'll talk about the management. So these are important keywords that you need to understand to, to, to know about collagen for the exam. Okay. So, uh collagen consists of uh alpha chains, uh glycine Parolin and Hydroxy Proline. Uh the alpha chains form microfiber als fibrillates, which then combined to form fibers that then um form bundles which then form your tendon. It is a right handed triple helix structure of three alpha chains. Uh And then it is a quarter staggered area uh where the triple helix is our uh layered in sort of a staggered area with covalin bonding, the tendons which are formed from collagen are parallel and they have uh they are good at, at bearing uni axle loads. And then ligaments have fibers which are more layered multidirectional less parallel and are useful are better at withstanding multidirectional loads. So how is collagen synthesized? So we'll go through the various steps of collagen synthesis and then I'll try and give you a little way. I remember this for my exam. So we know that fibroblast uh under the influence of growth factors, extra cellular membrane proteins, chemokines become activated fibroblasts and then they start translating the collagen. So this is the formation of the pre pro collagen and these form alpha chains and this happens in the rough endoplasm ICC reticulum. Uh so that so essentially your um D N A gets translated into the Mineau acids. Okay. The second stage is hydroxylation. And in the hydroxylation stage, you have prolene and lysine residues, remember it was Prell icing hydroxyproline. Um these residues get hydrox elated. So that's addition of a hydroxyl group and you require Whitman see as a catalyst for this or an enzyme for this. So, uh in the deficiency of Whitman, see it's a collagen disorder. And uh this is this is the stage of collagen production that gets affected. The third step is glycosylated in and this is glycosylated in of the license residues. Uh This is uh this and once you have glycosylated in you then have the formation of the triple helix or the three alpha helix is that, that form your pro collagen. And it is this step that is commonly affected uh in patients who have osteogenesis imperfecta, uh those who have the collagen type O I uh which is usually call, call one, a one uh gene uh defect. So all these steps happen inside the fibroblasts. And then you have exocytosis and you have protein alighted processing where you have cleavage, terminal cleavage of the triple, triple helical structures. And you have the formation of trapa collagen and then you have the cross linking where you have a quarter staggered area. Uh I have a nice picture of that. So on the next slide, so you'll be able to see that and this is through Covalin Association and um uh patient's with E D S or understand loss syndrome. Uh Some patient's with, with the DS may have issues with this cross linking and that is how the collagen uh structure is, is affected in those patients'. Okay. So this can be quite confusing. Um And so the silly way that I remembered it is you think of a hungry and thirsty, so thirsty and hungry traveler that comes to your door. So if you have someone, you know who you don't know, thirsty and hungry, you, you first let them in. So that's your intracellular process. Okay. Uh You don't understand what they're saying. So you need to be able to translate what they're saying. Okay. I know it's silly but just bear with me and because they are thirsty, you give them water. So you hydroxyl eight, okay? And then because they're hungry you give them glucose. So you glad consulate. Okay. So that's your third step. And then when they have recovered and well, you bring them uh you know, outside, you know. So, so then you sent them on their merry way and because you had this nice experience uh and made a new friend, uh you have, you have cleaved away your preconception. So that's your pro pro to lighting processing. Uh and you have made a new friend and a new connection. And so that's your cross-linking step. So this was kind of a silly way in which I remembered uh collagen synthesis. Uh So going through the steps again, so this is how it occurs. So you have your translation for and then you have formation of your triple helix and that's this schematic representation. Uh And then you have exercycle osis cross-linking and then organization uh from fiber pills, two bundles to fibers, two bundles. Um Any questions so far. Okay. Uh No, just appreciation for your way of remembering. You know, some things are just really and you just have to do it for the exam, right? You know. Uh um So here are some common types of college and again, you don't need to remember this, but this is just uh kind of uh you know, interesting stuff, right? So type one collagen, remember it is ubiquitous. So, you know, it's in bone uh in tendons and I've put one here just to remind you that it is collagen type one. Okay. And I know I have taken liberties with the spellings there. Um It's uh it's present in the remodeling phase. So it replaces, so when you have a tendon injury, it replaces type three collagen which is formed in the proliferative phase of tendon healing. Uh And so it's type three remodels to type one. Uh it is present in primary and secondary uh spongiosa, you know, type one collagen pre predominance there and it's a major. So type one collagen is affected in the collagen, uh types of osteogenesis, imperfecta. I wish you would go through in, in the next uh few slides. Type two collagen. Remember that it's present in cartilage. It forms the basis where you're glycosaminoglycans uh bind onto or or remain on. Uh and it's also present in the nucleus pulposus of vertebral discs and pathology. Uh You know, if you have a defect in type two is commonly associated with spondylopathy. Zhayl displays. Yes, type three. Um as we discussed the proliferative phase and then type nine actually stabilize the fibers of type two. Uh and they are present in the callous chondroid phase of fracture healing and defects in type nine are associated with multiple epithelial dysplasias uh and can be associated with early osteoarthritis as well. And then uh type 10 collagen anchors the uh cartilage to the bone um in the calcific zone of the articular cartilage. Um So, um you can see here this is a child with blue sclera. Uh And if you ever see a child, you know, so these are typical features, you know, there's short stature, you know, they're short limbs, you know, they're in the wheelchair and they are delightfully happy for some reason. Most, oh, I patient's I find are, are just delightfully happy. And then they can also have very, very severe presentations in the more severe forms of um, of osteogenesis, imperfecta. Okay. So what is it? So remember, it is not just brittle bones, it is a connective tissue disorder. Uh And remember we discussed collagen type one is ubiquitous. So a lot of these patient's depending on their phenotype would present with not just bone fragility but ligament, laxity, easy bruisability, they can even have cardiac issues, aortic root delectation, blue sclera is is one of the common kind of clinical features that we see. Uh but they also may have poor dentition, dentition or dental a genesis imperfecta. And they can even have scoliosis especially with the multiple compression fractures. Um and they may uh they may also present as nonaccidental injury and that should also be part of your differential. Okay. So the common mutations, you know, so psychology mutations, you know, the common one is the call one Avon mutation as this conspiracy type one is ubiquitous and it is associate oxygen is imperfecta. You also have a call to mutation. Uh and this is more commonly associated with conditions like S E D or Stickler syndrome? Okay. So how many genes are actually involved in? Uh oh I, so this slide, some of you may have already seen this. Uh This is blatantly stolen from our previous genetic stock that we had uh in Addenbrooke's Hospital. Uh And this is the East Genomics gene panel and they talk about 34 different entries and then I was looking on, on rare diseases websites. Uh and they talk about types 124, which are uh collagen dependent and then uh type 5 to 21 which are non collagen. But there are lots of, of, of subtypes. This was the classical uh celent classification of, oh I uh you know, types 124. But we now know that, you know, it is, is a lot more advanced but it still has its merits. Um And the silence classification was then further modified uh into this this or new classification which has been agreed by the International Society. I'm not so sure Mark will tell you more about the skeletal dysplasia uh societies. Uh So, um I don't want you to focus too much on, on all these genes that could be affected because, you know, in all fairness, it's very hard to, to, to keep keep tab of the current genetic status. Uh But this is kind of interesting for us because this is what we will see in the, in the clinical setting. So you can have type one. So according to the old silence classification, a type one which is a nondeforming, relatively low deforming. Oh, I with blue sclera. Uh, and then you have a type four which is kind of uh common variable. Oh, I, and I'll go through the, the various phenotypes later on. Um, so, so the reason they have, uh, kind of messed up the number, the silence numbering is because, um, you know, type one and type four are relatively milder forms of, oh I type two, you can see here is uh extremely kind of severe and really it is not compatible with life. Uh you know, it has a very severe phenotype and these patient's are do not really survive. And then type three is kind of moderate to severe osseous fragility, but they tend to have severe deformities of the long bones. So what happens in, in patient? So, so when you see somebody in the clinic, you know what is relevant to us. So remember that, uh you know, these patient's can be picked up in, in neutral on ultrasound scans. So, in neutral, um uh they generally don't tend to have long bone fractures or going uh postnatally uh rarely present with congenital fractures. They generally tend to have a normal growth velocity and height. So they end up having, you know, uh you're staying within the normal uh standard deviations and they generally tend to have straight long bones, they're usually fully ambulance. Uh they may are, they may not have any vertebral crush fractures and if they do, it might be quite minimal and their bone mineral density Z scores tend to be quite satisfactory. Um They generally have less than or equal to one fracture per year uh and they have minimal pain uh and generally have good school attendance. Okay. So they do require a little bit of, of, of surveillance but usually are quite well. Um Anyone know what the Z score means, just throw back to basic science. So why do we use the Z score in Children? Uh not the T score. Uh I think the Z score is age matched. Yeah, so it's age matched. Yeah, so, so uh T score is not relevant in, in Children. You know, it's, it's more when you compare adults to a healthy 25 year old. So in moderate, oh I um uh generally, uh you might find ultrasound findings usually at 20 weeks. So they might have bowing of the legs or they might have intrauterine fractures. Um So uh it's rare but not zero. Uh And the the risk of fractures may increase in the last trimester. Um And this is not modified by bisphosphonate therapy. Um You can occasionally have congenital fractures as they are born. Uh But you do notice that there is uh there may be a decrease in growth velocity or height. Um They may have some anterior bowing over time. Um uh The lumber, spine, bone mineral density that scores, uh are, are usually okay, but they do, uh, have, uh, quite a variation in the, in the standard deviation. They tend to have more than one fracture per annum. Uh, and they are usually absent from school due to pay, uh, more than more than five days. So, if, if you're patient fits this criteria, they, you would, you would call it a moderate, oh, I, and then the severe ones, uh you know, they have shortening of bones, they have marked impairment and growth. They're usually really are dependent. They have progressive deformity of the long bones, uh and spine and it may be unrelated to fractures. Uh They usually have very poor bone mineral density. They have more than three fractures per annum. Um and they may have chronic bone pain unless they're treated with bisphosphonate and they have very poor school attendance and require quite a lot of care. Uh And they may also get a lot of fatigue and pain. Yeah. So how do you treat these? Uh these patient's, what are your treatment options? So, always remember, you, you start with your basics as with any child that has a bone mineral density issue or fragility, you make sure that their vitamin D levels uh and calcium are adequate uh their, their, their metabolic work up is normal. Um bisphosphonates have been the workhorse in the past. Uh And even now you, there can be uh shown to reduce the overall fracture risk. Uh They tend to be better in younger and more severe forms, but there is a concern with overuse as uh you may have problems with bony remodeling and you can have uh issues like obliteration of the medullary canal. Um Does anyone know what denosumab is? Is it monoclonal antibody to rank? Yeah. Yeah. Yeah, exactly. Um And what does that do uh produced by the osteoblasts to stimulate the? Uh Yeah. So this basically binds this basically competitively. So it binds and uses up the rank ligand. Uh And so then it's not available to stimulate the osteo cast. And so you have reduced corners option. Uh So, so this is an option and then you have, you know what Terry parotitis is a recumbent P T H. Yeah. How does that work? So it's basically a recombinant form of parathyroid hormone. Uh But it is not a Pew approved in Children do to uh osteosarcoma risk and people do talk about gene therapies but they're not mainstream at the moment. Yeah. So this is an example. Again, you don't need to remember this, but this is just an example of uh you know, how do you decide whether you know therapy is required or not? So you look at, you know, uh the main thing you look at is how many, you know, fractures have they had, you know, uh whether they have spinal compression fractures, long bone fractures or multiple fractures in a younger patient. Um And then if it's yes, then you consider cycling bisphosphonate therapy. Um What does this picture show you growth arrest lines? So, so not exactly right. So there's, it's not like they've had growth arrest at these, but these lines coincide with their bisphosphonate IV infusions. So they are infusions. So they're called uh zebra lines. Um They're not exactly growth arrest lines. Uh You can, it just shows at which stage they had their treatment. Uh So uh you can consider uh cycling bisphosphonate therapy. Uh The uh and then you obviously annually assess their bone density, the ambulatory status growth and pubertal development. And this is why skeletal displays, your clinics are really important. Uh And you have other intelligent people alongside. So it's not just the um orthopedic surgeon, but you have a metabolic and a geneticist, a skeletal dysplasia nurse idea that you are following these patient's up. Um And so it's very important to make these decisions in an M D T setting. Uh And then you can, you can look at their bone mineral density and then you can decide how you continue their annual dose. Uh and how frequently you, you provide them with their annual dose. Are their, their, their dose for uh this phosphinates. Mhm. Um Always remember, you know, simple things are simple. So, uh if this is a mechanical issue, you can always treat patient's in, in different forms of splinted to protect them uh protective for miti's. And then sometimes you're in situations where uh you know, patient's quite early on present. So this is, this is a skeleton images from a skeletal survey of a newborn. Um Can anyone describe what they're, what they see here? Any takers? So you can see these kind of neurosis, both females. It's femoral Boeing and then there's also a deformity here, uh the radius and ulna as well. Um So, um if, if this is picked up so early on, you would say this is a mild, moderate, severe, must be at least, must be at least moderate because you said that Children don't have anything here in a little. And this is, this is the same patient actually a little older. So probably a few months, 6 to 8 months down the line. And you can see how dramatically the bones are deforming, you know, but they're just so, you know, he's just so young and this child is not even weight bearing, you know, but you can see how the bones tend to deformed. And so this is what, what they mean by um uh bone deformity. Uh despite, you know, despite the lack of, of any fracture. So it's not just the fractures that are causing the deformity. Uh This truly is uh you know, abnormal bone growth due to the abnormal abnormal um collagen. Now, again, uh I do not know the genetic basis of this child. This is just a child with, oh I uh and there are different treatments that you can perform. So, you know, you can use uh telescopic rods. Uh and then telescopic rods often need to be changed as you can see here. So this, this is just a solid rod that was inserted. Um uh And then, you know, over time, uh once it does its job, you have to change it and, and these are really fiddly fiddly operations, they're, they're not as easy as they look. Uh And you can see here that even though the femoral deformity is not so severe to try and fit a straight rod down because uh otherwise, if it's not straight, it's when we telescopic, you might need to create more than one osteotomy uh to, to, to have a straight bone uh for this route to go down. So in summary, remember your collagen, the synthesis. Remember you're thirsty, hungry, traveler, uh the different stages of uh phases of collagen production, translation hydroxylation like consolation, exer cytosis, pretty light processing and cross-linking. Remember? Oh, I has many different types. There's a very wide genetic variation. It's not just the silence classification. They can present your clinic in mild, moderate severe or extremely severe forms. And always remember to mention in the exam that it's an M D T management and you look at bone protection, uh simple measures like splinting and surgery as necessary and keeping in mind that these are growing Children. Uh So you might need to have appropriate implants, uh growing Children. So, thank you very much and I shall stop sharing.