Good afternoon, ladies and gentlemen. Thank you very much for coming to this session. It's very competitive out there, and I'm delighted that we've got a good number of audience. So, like Noor said, this is an area that's often neglected, and there's a huge need to learn about adolescent endocrinology. We always struggle in transition from pediatric to adult, so I'm really thankful to the scientific committee that put something like this. The only problem with it, it's a very difficult topic to put a talk together, particularly in 25 minutes, but what I thought I will do is just to go very sort of basic and talk about personal experience in Abu Dhabi, myself and my team, and flag up some publications of ours in this area. Adolescent, many of you have children who are now adolescent, and I hope you'll agree with the quote I put in. It's a difficult period. Everything is really more intense, and I personally got a few. They're all for children, and they all passed the adolescent period, so I know it by heart. And you might think I'm biased because I'm an endocrinologist, but I think adolescent endocrinologists are the most important subspecialty in here because it's hormones. Everything relates to hormones, and I think this is the best combination, having adult and pediatric endocrinologists in a big conference like AIDS. Okay, so I saw that Sarah was to talk about PCOS, and I thought for the flow, I will do puberty, and then I will choose some glands like what we've heard with the thalassemia, so I'll start with puberty. It's very interesting the transition between childhood to adulthood, and people are still not entirely sure of what actually triggers puberty. There has been a lot of important work in the literature, and people are still coming up with new hypotheses about the trigger of puberty, so we all know about gonadotrophins. We know about gonadotrophin releasing hormones. We know about caspeptin and other orphan hormones, but it is still not entirely known what triggers puberty. I would like to start with amenorrhea just to make a nice flow from Sarah's talk and also from the beautiful talk about thalassemia. So I'm just gonna give some scenarios where we, pediatric endocrinologists, see adolescents coming with amenorrhea, primarily primary amenorrhea, but it could be secondary amenorrhea as well, and I would like to briefly, I'm gonna give three examples. The first of them is the XY female. XY female, it's not that it's the commonest, but it's just the closest to my heart because that's where I trained. I've done six years of androgen sensitivity, and we used to be a European reference center for this condition. So primary amenorrhea, it's not uncommon for these ladies, the XY females, to come to us with primary amenorrhea because they have XY karyotype. And for us pediatricians, in fact, we see this condition more in the form of inguinal hernia, unlike yourselves, adult endocrinologists or adolescent endocrinologists who see primary amenorrhea more often. This was a very interesting area. I've done some basic science on this where we studied androgen receptors, and I'm very proud of this first publication I had many, many years ago, which had an incredibly high number of citations. So really fascinating stuff when it comes to the science about androgen receptor. And then PCOS, I don't know a fraction of what Sarah knows and what she talked about, but I'm glad in your closing statement you talked about mental health and PCOS. And our latest study in PCOS related to mental health is we looked at two cohorts of adolescent, one with obesity only, and the other cohort with obesity and PCOS, and we used scoring for depressive symptoms. And what we have seen is an impressive difference between the mental health status in girls with obesity with PCOS compared with girls with obesity without PCOS. These girls really struggle and have significant body dysmorphia. They hide their hair because they haven't got much of it, and the hair becomes in the face instead of being in the scalp, and they really struggle. So we have analyzed with colleagues from Mayo Clinic their depressive symptoms, and it was significant, even without statistics. You look at the scores, and it's impressive how common depressive symptoms are in girls with obesity with PCOS, unlike those who do not have PCOS. So yes, mental health is very important. And talking or continuing on amenorrhea, hypogonadism, particularly things like Turner syndrome. This is where we struggle, and this is where we need our colleagues, adult endocrinologists, as well as our gyne. There are so many guidelines on how to induce puberty and to maintain puberty, but there are many problems in this area. It's easy to pick up a protocol or a guideline and follow it, but we have issues with access to medications as well as the agreement on the timing. So we published a few articles on this, and there has been, on a survey that we have done in the Arab countries, there is a huge discrepancy of what people do on the timing. Yes, we do ask for the family history, like you said, for boys and girls, but there isn't any consensus in timing. I'm really privileged. I've been representing the Middle East in this international guidelines of Turner syndrome, which came out two months ago in the European Journal of Endocrinology, so hopefully this will help in putting together some guidelines for us to stick to, rather than having these patchy information from here and there. Availability access is a huge issue here. Yes, we've seen what you put in terms of the ethanol estradiol to induce those girls with thalassemia. Do we have ethanol estradiol? No, we don't. And more often than not, these girls you are talking about end up having OCPs to induce their puberty, and that kills the breast tissue altogether because of the large doses to start with. But if they do not have access to medicine, so I'm assuming that the idea of anything is better than nothing. And then also experience and knowledge about side effects. And we have surveyed many pediatric endocrinologists, and a large percentage of them do not know about the story of testosterone and hematocrit. And people, they start them on sostenone, and they don't even look at their full blood count. So there's a lot of issues that we need to talk, guys, pediatrics and adults. You educate us, and we prepare the patients for you. And now I will move to DSD. So these three examples are amenorrhea, but I'd like to add virilization to amenorrhea so that we can talk about DSD. And this is a fascinating story, ladies and gentlemen, that I'm going to tell you about. And I have always been inspired by gender assignment, and particularly the culture and the religion impact on that since my time of training in Cambridge. I have a lot of horror stories about how people think of gender assignment. Now, this is a family that I have worked out in Abu Dhabi about five years ago, many affected patients. And this is the first time where all the children and adults in this family were put together in this family tree because these people, every now and then, some come in Dubai, the others in Al Ain. And so I like structure of things. I'm putting things together. And this family and those 11 affected members, they have never been well-studied structurally as well as worked out genetically. So amongst this family, I have recently seen these two adolescents who came in with virilization and amenorrhea. They are sisters. And to cut a long story short, the whole family has five alpha reductase. So this is a common condition in which you get amenorrhea and then you get virilization because these are women who are XY, basically, and the conversion of testosterone to dihydrotestosterone is inhibited so they can present initially with female genitalia and they are raised as female. And once they hit puberty, they start virilizing, so the voice changes as well as their clitoris become bigger and then they have similar features to their PCOS feature. So that's what I saw with these two ladies. Now the question is, what do we do then? Doing the investigation is easy. Ordering the genetics is easy. Treatment is easy. What's not easy is to talk about gender because many of these patients in this family tree have actually reassigned their gender. Many of them have done that during the school years and others have done it during their adolescent year. What's best for them? They are XY. This is not an easy question to answer, but some of long-term data in five alpha reductase deficiency show that perhaps assigning a male gender is more compatible to the complexion of them and that's how they identify themselves. 60% of five alpha reductase deficiency patients who were assigned as females and they reassigned, they identify themselves as male. And you can imagine the difficulties in this because the last baby I saw has a genitalia looking like this, so it's not the easiest to sell it to parents that perhaps ideally, with this condition, assigning a male gender is better. And here is the conflict of culture and religion and the surgery and how the patients look and how the feeling and the expression in the future. So this was a very complex case and we ended up publishing it with some recommendations. Now, this is not just what happens or what happened in the hospital I was working with. This is well-known and this is a novel called Middlesex that shows very nicely what we don't see as physicians. So this is a very nice novel talking about a girl who was raised as a man, sorry, this is raised as a girl and then she started virilizing and then she has always felt that she identifies as a male. So lots of interesting story about this. The next thing that I'd like to talk about is adrenal. So we've done amenorrhea because of various conditions, DSD, and then a similar scenario where it comes with amenorrhea is sometimes non-21 hydroxylase deficiency of CAH. So this is a condition where a girl came in and was diagnosed with CYP17, which is a combined 17 alpha-hydroxylase, 17, it's an area that's proximal in that horrible steroid biosynthesis that is never, you can never remember. I used to just stick it in the wall of my office to remember, so this is another condition where it could be XX or it could be XY. In the XY scenario where there is no androgens, the external genitalia will look like a female and this is a family that we have encountered because three girls came in with amenorrhea and investigations showed that they do have CAH. So if I give you a break from adrenal, DSD and other amenorrhea condition and move to thyroid, we started learning in pediatrics from you guys, adult endocrinologists. We used to see Graves' disease as fascinating. Thyroid cancer we're not good at either but we started learning and I can tell you in pediatrics we see fascinating stuff. Let me give you a couple of examples about Graves' disease and a couple of example about thyroid nodules and cancer. So this is a girl that I saw at the age of 13. She came in with sweats and she had no goiter. She had ophthalmoplegia and astigmatosis and she was in thyrotoxic storm. That's how she looked. And when we looked at, she has a long story. I think I'm missing a slide here but she has a clear cut Graves' disease with extremely suppressed non-detectable TSH, very high T4 and she had positive thyrotropin antibodies. So she did have Graves' disease. She wasn't treated for it. Then she ended up having campylobacter and she was vomiting sick, sick for a long time and she collapsed with this triad. And what I learned is this is Wernicke encephalopathy that you can see primarily in hyperemesis gravidarum. Any hypercatabolic state, diabetes, ketoacidosis, pregnancy as well as this. So patients, they have these degenerative features, very symmetrical white patches in the brain and you can treat this in addition to steroids for treating the thyroid condition by giving thiamine because that reverse the vitamin B deficiency. So nothing like the usual Graves, like what we talked about or what we learned in medical school. And I think if I pinpoint five interesting cases in my 30 years career in endocrinology, this will be one of them. So this is a child, 12, 13 year old, who I saw because her mother said she just lost her fat. So the fat was lost from her legs, from her buttock became around her neck and her tummy. Lipodystrophy, how fascinating is that? And this poor girl, she continued having one autoimmune after the other. So as you can see here, don't know how clear you can see, she has severe acanthosis. So with the severe lipodystrophy, this is insulin resistance. You can see how her calf is. So no fat, no subcutaneous fat. She just lost it all. And then she presented with Addison disease because she started pigmenting. And mother rang me one day saying that her tongue is becoming darker. She failed her synapse. So this is a classical case of acquired lipodystrophy with autoimmune diseases. Not only that, she had Addison disease, but then she started having exophthalmos, a swelling of upper eyelids. She had suppressed TSH, high T3, T4, and also was diagnosed with Graves disease. Until here, she is manageable, until one day she had a sudden loss of vision, and I don't know, Dr. Ali Khalil, you might remember, a panicky message I sent in WhatsApp group of Mina saying, guys, help me. I don't know what's happening here. What do I do with Graves ortho-orbitopathies? And that's what this girl have. This is an MRI of her orbit showing the, well, it's not showing the exophthalmos, but that's what it was described. And it's very interesting, because what she had is her extraocular muscles are very thickened, and she had ophthalmoplegia. And then she had this momentary loss of vision. This is not pediatrics, guys. I haven't seen this before. And that's where we need your help. And I was directed through the Mina group a few years ago, and I was connected with the right ophthalmologist. Steroid treatment. There are a lot of more fancy treatment nowadays, intraocular injections. I can't remember the name, but there's a lot more to do. Now, every adult conference, we see lots and lots of thyroid cancer talks, thyroid genetics, which we don't. I cannot feel nodules. I had a nodule myself, and I have to convince myself that I have one until I got my thyroid out. But what we started seeing in pediatrics is a scenario like this. So this is a 13-year-old boy who came into our ER with cough and dyspnea. And this is the lung, the chest X-ray showing diffused nodular opacities throughout the lung parenchyma. This is too much for us pediatricians. So huge thyroid nodule, one in the left nodule, in the left lobe of the thyroid. And there's another mass encroaching on the carotid. This is an MRI showing the left mass on the carotid sheet and the left thyroid lobe. So we started seeing things in pediatric endocrinology that's more towards adult flavor, and we started learning. And here, definitely another panicky message for the amino group, because I haven't got a clue what to do. Well, I didn't five years ago. Now I'm getting slightly better, but cheating and looking at guidelines. So metastatic papillary thyroid cancer, which is at your fingertips, you adult colleagues, that's really scary for us. Now, lots and lots of thyroid guidelines, but this is the one that I normally use. So I've got three more example, but do prompt me, please, about the time. Metabolic bone disease is another area that I find it really interesting for us pediatricians and yourself as adult endocrinologists to collaborate with. Because as you see in this slide, there are some manifestations that's peculiar to us and some that are peculiar to you, but there are mixtures in the middle where it applies to you and us. And I took X-linked hypophosphatemic ricket as an example. Let me show you where the overlap is and how, why it's very important that we work together in this field. So I had this child who is, I think he was two years of age, when he presented to us with orbital cellulitis, he had dental abscesses, he was born by caesarean section because mother had pelvic deformities and there was no history of consanguinity. Now, he has always failed to thrive and he has this impressive ricketic picture. Mother presented during her adolescence with multiple bone deformities and she had multiple operations. And until now, she had musculoskeletal pain. She has no teeth, not a single tooth that she has and she is 135 centimeter height. So what I'm trying to say here for X-linked hypophosphatemic rickets which is inherited as X-linked, so a mother who is affected and an affected son and I keep repeating, it's important that we work together because this mother was misdiagnosed as osteogenesis imperfecta and she was planned or prepared to go on zoledronic acid while the treatment is completely different because as we know that it's FGF23-mediated disease for which burisumab is available for treatment, not zoledronic acid. Let me go very quickly through diabetes. No talk is complete without diabetes and I have to mention, I haven't done adult endocrinology but guys, type one diabetes in children is much harder. I don't know if you want to challenge me, you can but with this figure that was published some time ago in Diabetes Care, this tells our story in pediatric diabetes and how much we struggle. You see the HBA1C, this is an international study in a very rebuttable journal that proves what we say to our managers. We need more time than the adult colleagues dealing with type one diabetes and they say no. Why your adult colleagues see 20 and you only see 10 and I say because it's a different disease and they argue with us. It's a much challenging disease and be prepared. When we transfer them to you, you might swear at us because they are not gonna be in their best control so do please look after them. There are a few studies that we have done within the population of Abu Dhabi. These youngster, they like technology so do please offer them to continue on their technology when they come to you and as well as they like gadgets and they're very good, they will teach you and I how to read the software in this. But having said that, please be alert that they will not all be adherent. Even if the insurance covers everything, they might not wear their sensors. Even if the highest and the most expensive pumps are given them, do them for free, they might not utilize them very well. So it's not only the insurance that I keep telling people when I give talks in other countries, please don't be disheartened with lack of money. It's not, money is nice, of course. We all love money but it's not everything. Exercise is very important. When we transfer those youngsters to you, we've done a lot of work about exercise and this is one of consensus statements guidelines that I represented, ISPAT in this. We keep saying that monogenic diabetes is rare but perhaps it's not very rare in a region like ours. So do please think about it. In a country like UAE, we rank the second most common, we have an incidence that's five times more than Europe and alert me how many minutes do I have. I'm done, okay, very quickly. Antibodies are important, not only for monogenic diabetes but for these novel molecules to prevent diabetes like teblizumab and so on and so forth. If they come to you for counseling for Ramadan, don't waste your time and tell them, no, you are high risk, don't fast Ramadan because they will fast. So you better prepare yourself how to educate them well because they will not listen to you. These are multiple publications on that and I will finish up with the last condition which is obesity. Bariatric surgery, it's scary for us but we need to acknowledge that it's a good way of treating obesity and patients will come asking for referral to bariatric surgery, why? Because they knew it. Majority of children that we have, so this is our last publication in bariatric surgery, majority of those who come asking for referral for bariatric surgery have family history of people who has been operated. Our guidelines are not very encouraging. This is a group of children that we followed for 30 months post-bariatric surgery and the later you wait, the worse the prognosis. If the children, and it's difficult because our guidelines say we want metabolic disorders, we want diabetes, we want dyslipidemia for these children to qualify, majority of those people do not have this because when we looked at the characteristics of these children, they are very heavy and they have sleep apnea and poor quality of life rather than the metabolic. I'm not gonna say much about this. I will just finish by another quote. Adolescence is a conjugator of adulthood and childhood and there's a lot that we can do together. Thank you.

adolescent endocrinology

transition care

PCOS

hormonal health

bariatric surgery

collaborative treatment