Critical, Inpatient & Outpatient Care for Pediatric Transplantation Patients

Critical, Inpatient & Outpatient Care for Pediatric Transplantation Patients



Oh [Applause] you good morning everyone welcome to two grand round today's speaker is one of our soon-to-be graduating third-year residents in the pediatric program dr. alia Alia bolas I was trying desperately to pronounce it the right way dr. bullet came to us from Egypt where he trained as a pediatrician and worked for some time in Egypt and Kuwait Preiser relocating here to redo his residency on completion he will be moving to Gaillac to join a private practice group please welcome dr. Bullard welcome to our Grand Rounds thank you all for coming today so today our topic will be critical inpatient and outpatient care for Pediatrics transplantation patients and I have a nice story from Middle East from my previous experience so the topic would be like four sections first section I'm going to present the case from Middle East it's very challenging case regarding a patient was the recent renal transplantation and then we will discuss the hematopoietic stem cell transplantation then solid organ transplantation then the last part would be what's the role of general pediatrician to deal with patients of transplantation and they'll be like transition flight was from ancient Egypt so the case like we had the case in our pick you at Jarrah Hospital this was in Kuwait 2015 so she was five years old female presented with the status epilepticus she had like abnormal behavior she was very irritable it was sort of screaming and crying and put heating for three days then she had the high grade fever and severe itching for two days then she had decreased activity and diarrhea like three times per day non no blood no mucus and mum billiot ihnen bloody vomit us for one day so she has very significant past medical history shared in the stage renal disease due to bilateral renal dysplasia and she had had a renal transplantation Roma deceased donors three months ago so her medication shows and actually actually must tackle Imus and mycophenolate hospital and could try boxes all ganciclovir under the pain folic acid and magnesium she had normal development for vaccination and his family history was irrelevant so by examination shares like the very fast heart rate per tolerate her blood pressure was high and her temperature was 38.5 filthiest and she was very agitated she was she had very aggressive behavior like she scratches three nurses she had extensive scratch marks on her lip edema fingers alterations while afrikaans arrival injection and excessive Olek secretion have chest she had like bilaterally corner entry no problem her heart was doing fine ahead of dementia like a liver the first three centimeter below of the margin and had get close to be to blood working fine heroically had Glasgow Coma Scale was just eight over 15 she had bilateral constricted sluggish pupils and generalized hyper tuning and hyperreflexia with abnormal movements on many many stimulation so you know the differential diagnosis for her mental status we do vitamin we did it there with it here so vitamin vascular like stroke migraines and fiction and enjoyed Hoxton accident abused metabolic intussusception but not in this case new players and seizures but in head kids in particular because she was a transplant patient so we said like she had the picture of encephalopathy versus into polite so we said maybe in civilized maybe do two immunosuppression she was in a lot of immunosuppressive drugs and she may be team Vince polite HSV in polite or cryptococcal meningitis and she was hypertensive she maybe she was hypertensive encephalopathy allopathic or uremic in telepathic because maybe the some sort of grass the rejection we are going to discuss all of this in the presentation and maybe drug toxicity or electro disturbance because she had a significant gastrin sites or graft related infection but usually this is not very accused it takes time like HIV or EBV so World Cup has TBC was not there what no it's not significant for anything TMT so sometimes we do minimal zinc wait so but only things that sort of normal is have eighty alt LDH empty cake and we said maybe because of the seizures and continuous convulsion shows anesthetist but like head kidney functions was fine Evian and creatine this in milling will might promote but this is normal and sodium was 132 this will not give you a convulsion tour status epilepticus her venous blood gases were fine sports with a CT brain and this was normal with the CSF for her so it has a white blood cell count opposed mine but the more Manos would like ninety percent versus like we had think was fine but in like wait milli more measures and the culture was negative and we did viral she studies like for a good panel like include CMV EPV and she was simply positive with PCR like 9800 34 so we said here maybe the our working diagnosed for it was like CMV and polite they want shoes intubated she was ventilated to protect her airway for better control convulsions in the convulsions were controlled with the keppra phenobarbital or literacy team phenobarbital midazolam infusion but she continues to show like abnormal movement to minimally stimulation we started like big guns for her like meropenem eco planning which is very very likely but the medication for gram positive organisms including mister ganciclovir for the possible steam vents alights and amphotericin B for possible fungal infection so blood pressure was hard to control with Andy Devine and hydralazine but unfortunately the patient developed well in cardiac arrest and passed away on day three but this is not the end of the story like scores during here to stay we weaken contacted the transplant center in Kuwait City and like saying with chicken and other recipients so the second kidney recipient had developed similar symptoms and died week later and the heart recipient has died after cardiac arrest to some new psychiatric symptoms and the medical well she spotted fit button device any organism both patient records showed that the deceased the donor was 28 years old Indian male who presented with chest infection and seizure and the CT head and CSF culture of the donors were on markable anybody has any clue what's going with this kid okay so see like somewhere at India so the actual is a sunfish it's a transplant center contacted the deceased donors family – the family confirms that he was bitten by a domestic dog in India while he was in vacation two months prior to his distinct ways like he was in vacation he went to Kuwait he was bit by the dog bitten by the dog and he came back to Kuwait and he died inquiry and he of course he didn't have it the he didn't get any prophylaxis and actually the asked is what happened to the dog and the dog died soon after he bit by the fish do you know what's happening right now directly so the cost of this of his distress was just pneumoniae sepsis and seizure so there was some patients have got the transplant in Saudi Arabia so they contacted them all so like is the liver recipient developed drooling and hydrophobia and died after 34 days and norton brain about she showed Negri body's rabies RNA was detected in brain and size of the patient and there was like two corners for two different patient's in Saudi Arabia those explanted and what they patients they get the reflux and they remained well they didn't die so the corneas were sent to CDC in Atlanta and ladies was confirmed it confirmed in the donut explanted corneas by PCR and the ribs strain is known to be endemic around Indiana police and the tiny borders in this area so I did some research like I thought this case was published like in 2017 Tabby's in 2015 then I came here in 2016 and it was published in 2017 course they published this case but is this not didn't only happen in Kuwait like at least there is like ten reported rabies outbreaks in transplantation the last one in the United States was in 2013 and Maryland and the donor was bit by a raccoon so the last one documented in literature it was in China and was explained it in and 2017 stood each opening very hard so I take this case because its forth like very overwhelming very like racing just to understand how fragile and overwhelmed those patients so first the first section we are going to do the hematopoietic stem cell transplantation but first I will introduce you the doctor here it is the earliest known physician ever he was like his first record was in 2645 BC he was double boarded in medicine and then mentioned in the record and he was he was the first one in history to like describe the diabetes and he said frequent urination so much with expensive transplantation you know like it is the simple definition it's like infusing the blood stem cells with Roma donor to a recipient so answers like different types so if we go from down so sing genic if you like you are doing between I did identical twins and advise arises by this was the first time like in 1956 the first stem cell transplantation was between identical twins in 1956 autologous if the donor and the recipient are the same person and allergenic is the donor and recipient are different people and of course there's like fully matches as related donation can come from usually sibling rarely parent if it's very unusual there is a history of continuity and unrelated if sometimes it comes from like umbilical cord to blood banks or if there is a living donor so sources you can like collect because there are three main main sources like brains the stem cells from bone model compressor blood or from umbilical cord powerful essential unit of course the you know the human leukocyte antigens HLAs needs to be tested for major histocompatibility look hi so we have Class A which a B and C n plus 2 D are DQ and I think this DP o at least six loci routinely are analyzed for umbilical cord blood bank but usually 8 to 10 for live donor both that other than the umbilical cords so epidemiology like there is like a huge rise in the trend of stem cell transplantation like in 2012 there was like Waterboys more than 2000 cases but this keeps ink reading and the trends are affected by improvement supportive care course including dealing with graft-versus-host prevention and treatment and donor availability now there's like umbilical cord blood banks and registries that expanded live donor programs and reduce the intensity conditioning like especially for the cases that not malignant like heads or him applaud him ago hemoglobin evasive and hello hello identical stem cell like this comes from first-degree natives usually mothers and of course because there's like now new techniques just such as like cyclophosphamide infusion so types as we say it like we have the Bohr model breath or blood and the umbilical cord blood so warm models like the most common it usually needs and it's easier the donor will be in grown sides and you will collect the the stem cells from the iliac crest it has advanced of high engraftment rate and loaded to do a chronic graft-versus-host but this advanced like pain for the donor sometimes you cannot find like very a identical donor or the peripheral blood there you need like to give the colony stimulating factor for three to five days in put the donor and a Cerises machine and then a has the advantage of high engraftment rates also and higher stem cell yield but sometimes the disadvantage will be like there is higher higher rates from chronic graft-versus-host disease or umbilical cord this is the easiest one to collect but sometimes not very sufficient but it's very good like the patient usually does not have any viral infection and it's good also lower rates for graft-versus-host disease so indications like malignant diseases of course of this latest acute lymphoblastic leukemia species were very high-risk patients or relapsed disease acute myeloid leukemia in high-risk features nostril and relaxed disease myelodysplastic syndromes non-hodgkin lymphomas logical informants some current research for solid tumors like even this of Ewing sarcoma forcing high risk neuroblastoma and sometimes in brain tumors if you would like to avoid the radiation for the brain growing brain indication for non malignant disease so you need like to make a balance so if the phenotype is very severe and there is like an available donor to scale this can be a very good solution especially for cases was primary immunodeficiency such as severe combined immune deficiency skid or X length chronic granulomatous disease which could alteration and wrong and this the common examples clip can help prevent neurological and logic progression and metabolic diseases because of the replacement of the deficient enzymes in the monocytes and this usually takes like months for the monocytes to migrate from the bone model to the CNS so some indications of course like primary immune deficiency specially of severe phenotype and the him of hemoglobin hemoglobin number see there see me a major sickle-cell disease of course if there is like inherited bone model failure syndromes Fanconi anemia severe aplastic anemia diamond Black Swan anemia or the aplastic anemia and a lot of metabolic genetic disorders like Oh stupid roses you'd be like a fatal disease you could polysaccharide OSes you can like make a balance between giving the enzyme infusion or doing the the transplant depends on the availability of the donor and then also like leukodystrophies other miscellaneous like such as Boucher disease only man dick disease a lot of indication so what are the risk or course there's like acute and long-term success it is as we saw with our patients so that risk depends on the conditioning its intensity is like Milo a plated in the malignancies or reduce the intensity conditioning and Mon malignancies courses there is like pre-existing comorbidities fireteam serve exposure and the force the stem celery source source and all of this influence the risk of complication and transplant related mortality but in general it's better in children as an adult so the transplant related mortality is up between 5% and 10% so one of the most common complication is infections or in the owners is reconstitution so the neutrophil engraftment typically occurs like two to three weeks after the transplant the natural killers usually after one month and t-cell function like we embedded by intent like especially in during periods of the prophylaxis like in the beginning or therapy of graphic graft-versus-host disease but for those like pubs with prophylaxis against the graft-versus-host disease usually they do the switch the lymph side stretching from IgG to IgM usually from six to eight months so of course like bacteremia steps some fungal infection and frequent particularly during the need to panic phase the first three weeks and sports are the Potala viruses such as RSV an adenovirus can be like devastated in this in the moon'll compromised state and primary in fiction or reactivation of CMV of EBV is like a lot of guidelines international and institutional guidelines how to to to do surveillance how to catch this early and of course you know EBV like it's very simple virus in the immunocompetent ation can give you mononucleosis but in those patients can give you lymphoproliferative disorders which can range from benign disease the lymphoma and leukemia acyclovir prophylaxis sometimes indicated in patient with switches v1 zero positive patients and usually like for one year or something after the transplant and it gives some protection against varicella-zoster virus has what's called beak a virus can Kojima logics the spikes especially for the for the kids with renal transplant will because this later and renal dysfunction and numerous also usually it would be like hum prophylaxis during the immuno sufficient behavior suppression in period and of course it is like out of guidelines from the CDC how to deal with neutropenia usually it looks like our supine neutropenic patients like if you have a kid with fever usually you will check like with CBC blood culture cover the most if you team 50 milligram per kg FDA's are but there's like tons of guidelines available and then of course like the immunization sometimes the timing of live attenuated viruses you need like there is also the guidelines for when to start or not to start live simulated by a vaccine people's eyes is like happens almost an old children's vertex publicity like officially with the case of neutropenia can happen anywhere from like mouse to lose the rectum and it can induce translocation of bacteria or secondary hsv-1 fungal infection usually it needs like narcotics something like magic mouthwash a lot of the treatment nutritional support so this usually improves with the improvement of neutrophil accounts additional support as we said like a lot of them a lot of the kids directly after the transplant who has decreased intake a lot of nausea and Ricky a malabsorption makuu's eyes and they have increased metabolic needs because of the catabolic state it was like malignancy or mini deficient zero current infection so usually we need some sort of interest feeding in preceding is like most preferred very preferred more preferred than the IV parenteral nutrition because this like will give benefit to the liver by promoting billary flow this will avoid something like sinusoidal obstructive syndrome and piriformis in graft-versus-host disease there's what's called sinusoidal obstructive syndromes it's like Syria specificities in one to ten of patients depends on the center and it happens because of like injury of usually it happens that feels like pre-existing liver disease or allergenic transplant or high risk neuroblastoma or if the fuse like busulfan or cyclophosphamide treatment it involves occlusion of sinusoidal znews due to micros from PI and then the liver will be swollen tenfold then we'll see fluid retention policies actually has a patient with acute acute myeloid leukemia she develop sinusoidal obstructive syndrome and we are dealing with her so she's the the case is mild or moderate we deal with dialysis but our patient with severe also so we do to provide was like promising results so pulmonary complications between fiction volume overload sometimes also we will have like neem unites from the alkylating agents idiopathic pneumonia syndrome and chronic graft-versus-host disease those potala failure that needs intubation and ventilation usually with poor prognosis than the usual fish so the main thing is like graft-versus-host disease it's a big deal it can be acute it can be chronic so for a cute dress with the versus host disease so what happens like the immune it's immune based complications like its antigen exposure and there's a doughnut t-cells because you infused donor t-cells that they infuse the donor t-cells like attacked the recipient tissues usually the excuse one it's like skin GI and liver because they have the they are rich in antigen presenting cells so GI can happen from colon stomach duodenum so there's like a lot of staging system like for each like system and for the overall picture and depends on it like you you decide to treat this with corticosteroids or you can add more medication this is vacuous the chronic grass versus host disease like it happens like months after the transplant usually around six months and it can be a devastating complication we have an attending in our pediatric floor he used to say like you are replacing one disease with another disease like is the main diseases to replace the special if you develop chronic graft-versus-host it's like a very devastating this looks like clinically looks like systemic lupus or systemic sclerosis usually results in dry eyes dry mouth fatigue debilitating skin muscle joint liver got on lung disease usually it needs prolonged immunosuppression and you do prolong the demonstration this well may result an opportunistic infection more incidence of opportunistic infections and it's like it has also endured and dysfunction usually the lung and liver and chronic grass has to say that it replaces the original disease so for those who develop breast where's the salt once the graft-versus-host is an active or stop proper fish champion of time we can start like weaning then installation see what's going to happen for there is like a complex protocols for this but because of there is like a tolerance between the donor T cells and that epeans usually not expected to receive a lifelong immune suppression unless you develop like severe chronic graft-versus-host disease this is unlike the solid organ transplantation which usually needs lifelong immunity occasionally I have a flight like to compare between both of this at the end of the scope so it is like a lot of screening recommendations for the latex like this between the primary care physician and the transplant centers like for example here for iron overload usually we do like annual serum ferritin it elevated you do like an MRI and of course the management will be chelation or phlebotomy is with like funds then like renal Foreman re cardiac metabolic spiro it like this like huge deal for houses usually like fix it and arranged by the transplant centers by row it growth mineral density osteonecrosis reproductive all or like the covered the patient from every ethics so the next section will be on solid organ transplantation just like some slides also from ancient Egypt like they would like little bit advanced in surgery like this is on in the symbols here and this like the actual one they have like artificial limbs and actually they have like a medullary nail here like one of the mummies and they like they will go to denture by they did attempt in the solid border position so so it organ transplantation I'm going mainly to talk about kidneys because like around fifty percent of the plants plants so it's organ transplantation in pediatric patients or kidneys so of all renal transplants around seven percent happens in the pediatric age group and usually under listen and in a children from six to twelve years but it can happen in very young kids so of course at any age level or at any like disease stage the three-year survival of patients following a deceased donor or living donor transplant exceeds a survival under dialysis so by any main like the transplant is better than the ballast so the most common indications renal transplantation is the congenital anomalies like at Lygia hyperplasia dysplasia obstructive uropathy focus segment Angulo mitosis is the most common three indications and of course the immunosuppressive therapy has to say it like 50% like all the patients needs long-term immunosuppression like lifelong so 50% is like a columnist and microsimulation hospital and Sydney's on like 50% but sometimes patient will not to rate this one so it's replace but a cesarean corticoids is the thin shield but now was using the TAC and MMF have raised the possibility of course extort withdrawal or avoidance from the beginning because to avoid the long term complication of steroids outcomes like acute rejection rates have declined and recent leads from like around 50% ones but its invented so now like in percent those the acute rejection now's like less common than before and of course it's like a lot of variables depend on the is it like a disease the donor versus lived honored more with the deceased donor more with the african-american more if there's like mismatches in the HLA special dr or of course if there's like no induction therapy and of course if you have more than five life types lifetime transfusion with after the fifth power one year the risk or acute rejection increase so the reversal of acute rejection still not very successful like as we said like 16% will develop acute rejection and around half of them will be reversed it like more was they lived or not more than the disease do not so like around 4% of the living bowler donor and 6% of the deceased donor recipient developed graft failure ended so from the confer confirmed grass period dead chronic rejection is the most common so chronic rejection is common more common than the acute rejection like the chronic rejection happens in 41 percent and there is like other causes of the graft failure something like acute rejection as we said like only seven per 6 to 7% grafts from boses and like the vessel the graph gets from both disease records like original disease can happen again in the content it kidney and death was functioning graft from other complications it's like eight point six but there's the most common cause of graft failure is the chronic rejection the probability is at the graft would survive at years one and five like images to be 92 and 79 for living donor and H 3 and 65 for disease donor so we have like this on the scribble T living donor is bigger than the disease donor but depends on availability but like eighty percent of patients will have like a functioning graph by age of five years and of course the patient survival exceeds like 95% at five years as you say like primary codes of this being infection so infection is almost 1 and graft fitter is the second and then there is another causes like cardio pulmonary disease and cancer due to immunosuppression so again infectious complications same thing now you'll have like life long immunity patients who are born to viral the period of fiction and as this is like this is comes after the rejection and this is what's called bkv virus it affects like it lives in 90% of general population but it's like an important codes to cause the lace kidney graft loss tmv can be a problem also and can cause like chronic rejection long term issues like mini kidney many pediatric kidney recipients failed to reach their normal general adult height and of course if the transplant is little bit earlier it's like before six years of age it gives you better results so baseline renal function is an important factors you know like the kids with renal failure they have like something you do low growth hormone and of course the effect of corticosteroids will add to this so treatment with recombinant human growth hormone and decrease as possible the doses of corticosteroids or to avoid it at all improves the condition so it's like hyperlipidemia hypertension obesity unknowns the doobie diabetes it can complicate the transplantation mainly because of the immunosuppressive drugs like penicillin and other drugs also like liners this can can give you like the side effects from like hypertension hyperlipidemia and this may affect the outcome in the children so post transplant malignancy has been increasing progressively it was most like to say it like most of them are related to EBV and as we said like EBV lives in like 90 percent or month lines percent of population it's give you like mononucleosis but in immunosuppressed kids like those they can develop like lymphoma and severe problem other risk factors being age younger than Athenians all other risk factor for like long term issues it will be like this if you are less than 18 y trece male 6 and along with immune solution so medication non-adherence to like it happens in 60% of patients Pishin adolescent patients specially because a lot of these medication have like h2 hesitation and gum hyperplasia the officials have adolescent adolescent ation day like okay so i will stop taking my medication it gives me a lot of facial hair gum hyperplasia that's a significant problem like it's now found in like the 32 percent of patient like around stairs patients are non-adherent and six of them would develop like rejection because of non adherence so this is a big deal so for other organ transplants like of course we have like liver transplants usual for biliary atresia acute aquatic and of course the metabolic disease which there is like hot transplant also for congenital disease mainly on cardio basis and lung transplant for cystic fibrosis congenital heart disease mainly and primarily hypertension so this is a graft versus host and hostess graph so sometimes confusing so graft-versus-host this like if you are going to transplant like stem cells it's like is the hematopoietic stem cell transplantation programs developed by time and there's no need at all for long term immunosuppression on this chronic graft-versus-host is developed so you are giving the donor cells and not the donor cells are like attacking their recipients organs you as you said like dec you to be skin GI and the chronic is kind of fixed anybody it looks like systemic lupus but usually colons happens and it's not a big deal here unless it's like chronic here's the host versus grass it's like what's called grass failure like what happens like that is EP and commune system is attacking their donated organ like if you have the kidneys want to learn so you the eb ins lymphocytes are attacking this kidney and this will give you the the rejection it's like only with solid organ transplantation no tolerance develop and it's long like life lifetime immunosuppressed so this will be the last section in the talk so the role of general pediatrician so just this is a temple in egypt called city or first Abydos so it looks like any other Egyptian temple but there's like something interesting here so if you don't like the zoom out on this one in the temple you see this so the temple was built around like three thousand years ago and those discovered like at nine 1848 you see anything cific and the tank just like a helicopter here and the tank and spaceship nobody knows what this maybe something would come in the future so there is a scientific explanation for this but this is not our rule as general paediatrician the stage if knowledge is thrown so if anybody is interested I will kill you exact what does the explanation is after the tow so what's our role at the general pediatrician for what our role at the general pediatricians for those vulnerable like very challenging patients very fragile patients so first of thing like we need to understand the principles on both within the transplantation what graft-versus-host versus graft and this is the main indication for this talk and what are the medication used in the immunosuppression how what are the complications that we anticipate so this is like a huge list of complication like for this one a climbers it can cause hypertension it can cause neuropathy okay cause renal dysfunction like uses for renal transplantation but the medication itself can cause like as you see Mia and a lot of problems hyperglycemia diabetes hyperkalemia hyperlipidemia a lot of things and microphone elite also can give you like GI problems like diarrhea and can give you like the problems in the CBT like leukopenia anemia it can add to the sepsis risk of course it can induce cytomegalovirus CMV viremia little bit more common in patients on microsimulation fatigue and of course lymphoproliferative disordered it can increase its little bit so prednisone we're just like destroyed you know like testicles third retention throws myopathy protein catabolism and like in burden healing mister irregularities cataracts and glaucoma is a cell pain can replace Mike assimilate more fatigue but still it has like some like lik Phineas from such being able modification and increase the risk of infection and new Blasi so the second role for Jim pediatrician like need to diagnose entities that common common ends like those kids will have flu will have like throat infection will have gas trans right so you need like and you'll be like the first line so as a pediatrician we need to know when to treat them when to refer them what the wrong side but who you are they're the first line so be aware of the timeline of the rapidity of the referral and of course know what are the red flags for organ rejection like for example if you are having a fever that's not improve in the first 24 hours so maybe it's not just the rhinovirus that you diagnose maybe is like something else happening with this wonderful patient so if you have failure to improve in the first 24 hours the residual extra distance deeper or recurrent fevers or is this like high elevated white blood cell count or is this like reckonin somatic complaints like unexplained nausea abdominal pain that it's not explained by the awesome diagnosis all of unexplained organic specifically AB usually suspicion needs lab like it has this patient it's better to do a CBC and CMP to know what's happening with the kidney function what's happening with the CBC just like opinion leukocytosis there's like a Nemean explains from site opinion so if there's like any of those like i think the best approach is like treat to contact the transplant team at the earliest possible time and except advise any necessary next steps because you know this can like develop overwhelming sepsis in very short time so also you have to support and explain and comfort to offer comfort for the families that you said like non-adherence is like it happens in 30% of other lesson patients so you need to talk to them to tell them yes it can cause some side effects but like it will avoid a lot of over long term complications and then to support them maybe they will need like some psychology refer to psychology or psychiatrist so this is our one of our duties so of course like be careful for with drug interactions like this so you are in like Malta polypharmacy drugs at the climates and micro phenolate the fringes alone so some medications like increase the plasma levels such as arrests or mice interest or mice and not the chromatin is not that much but better to avoid it from antifungals some like calcium channel blockers and others like committed to provide this can also all increase the levels of the plasma levels of alignment and there is like medication that can decrease such as like cephalosporin which commonly we use them but we need like maybe touch base was with the transplant center before starting the dinner or something for them or third-generation cephalosporin so and I think the last one we have is the vaccination so vaccinations are not like for the kids the kids with renal transplantation and then long-term new suppression usually like the live attenuated divider vaccines are better to be avoided like varicella rotavirus measles mumps rubella PCE small books energy so we have two quizzes through the first one here they took about seven years old girl was homozygous sickle cell anemia underwent stem cell transplantation from unrelated HLA identical donor seven months ago she has been complaining seven months ago but she has been complaining of fatigue for two weeks and now has developed a feeling for her mouse being dry on physical examination she has was what on a specific crash over the trunk and on and no cyanosis goes on this she has shorty and heroes of vital notes but no other significant adenopathy and no fever was mentioned so the most likely cause of her symptoms is a B C D D D like EBV so we have become Izzie and dr. Atkinson be anybody that's because like six months seven months and it looks like the Simek lupus because you have fatigue and this rash so it looks like so the chronograph versus with it actually EBV is like very good the salt but the usual it does not go – so I think I'll go with this voice – which of the following statements about renal transplantation is true the the most common cause of greener grass failure associate chronic rejection so yeah survival after transplantation of kidneys from a disease the own– or the shorter than survive and rise is wrong because as we said like without like these donor or live donor both of them I have much better prognosis than dialysis and both transplants the malignancy is a problem of other nodes like the same it happened children and of course monster the monster the drug has nothing to do with this my reference a lot of guidelines if anybody wants the presentation I can send it on and I'm for question I have more of a comment we are always available to help you guys with this stuff so feel free to contact us before the transplant center or both but we can be the conduits for that the other thing is we also have because you guys are the ones who do this I have a list of post at least bone marrow transplant recommendations for immunization schedule realization so if you ever need any of that information just feel free to call us it was a great talk he gives an update on Kaymer ism or did that come up with you know in the in the case of solid organ transplantation I know a lot of centers have actually had success there's protocols I guess had but I don't know if you in your reading if you came up on a be identical thing well basically yeah that I know that there are protocols in terms of transfusion protocols and other sorts of things where you see a no Pittsburgh's been a leading kind of observation where they should come off of all immunosuppression which is desirable I know you want to get off some of the more serious serious depression but there's certainly case reports what I don't know is where the state of the art stands in terms of inducing timer ISM or protocol and maybe marky-mark folks know yeah so I like what while I was addition that I came through some of them but it was like very advanced so just I read about them but it's likely and I can speak for again bone marrow transplant he met a poetic stem-cell transplant there are a lot of non malignant diseases now where we are accepting Kaymer ism so you mentioned you know happily identical transplants and those results in Kaymer isms that give you enough of normal stem cells to overcome the disease process without and they're still working on this but without giving you the toxicity that you would expect with a chimeric marrow as opposed to a fully matched marrow or fully and grafted marrow does that make sense thanks to that I mean I have a patient who's a transplant bit tough you know he's using is a living related transplant she's now almost him and primarily because of you know the psychosocial problems and lost follow up things like that Genesis came off of human a suppression and has never had a really a little bit CMP post transplant and bailers unveiling but it has not been on immune suppression and if it's stunning you know it does their numbers of case reports of that but it's obviously immune chimerism that's occurring and of course I'm sure the closer you are to the HLA kinda thing the better but in this is solid organ like your real recipient say if there's any way to predict better I really don't know for solid organ transplants but that's what they're looking at for sickle cell disease and it's a it's a very hot topic right now in our world because you know there's a lack of donors which is a big problem so you know I used one thing in your slide that was interesting to me based on our journal Club yesterday from the NICU that the risk of failure for kidney transplants window significant appeared had a lifelong v blood transfusions like the risk of acute yes rejection increase if you have liked more than 500 plants have fat transfusions retention I think not the toes talking about the kidney kidney transplant sighs yeah so I think it's more than if you have more than transplant oh let's mean w chicken transfusion yeah so I guess my question is because the theme why I think maybe there like you have like more transfusions it's like maybe we'll induce like more incidence of conviction of TMZ or sometimes also like I think we do the irradiated one to kill the lymphocytes to avoid the graft-versus-host but nothing really nothing this was not in the kidney may be in the stem cell consequently so I think maybe this like mainly the same be an EVP virus great talk thank you with regard to the transfusions it's my understanding that it's the exposure to the minor blood group antigens that sensitizes Olympus i'ts so that you have a higher likelihood of developing rejection when you've been transfused and I never knew there was a specific number but but I did know that the more transfusions you've had of RBC's that were likely you are to reject an and it's um it's related to the minor antigens yeah and maybe you could update there's two things one is and because it has relevance with the renal transplantation I know that the Pittsburgh folks were kind of pioneers with this but now there the hepatitis C they're actually using hepatitis C positive donors because the tree antiviral therapies post-transplant are so effective that even though you see I used to be an absolute no-no that you'd never transplant a Hep C into a patient so it's kind of stunning but the gives us an idea we're at the antiviral therapies in 2019 very encouraging so that's a that's certainly a positive thing the other thing is going to you know ask was in terms of and dr. Atkins could probably answer this absolute contraindications to solid organ transplantation and I used to be kind of blood group and other things specifically are there any absolute contraindications in terms of matching you know in terms of things like that or no not really you might want take a swing at that maybe some experience with liver transplants from hepato blastoma which by the way I think we're going to be seeing more liver transplants because we're being much more aggressive with therapy for metastatic hepatoblastoma now but it's mainly you know infections and recurrent disease in my world at least any other questions or comments in the room we've got a few minutes I can unmute the phones if you are on the line and you do not want to ask a question or make a comment please place your phone on mute now the conference is now in talk mode it is anyone on the line have a question or comment going once going twice ok thank you very much sir excellent [Applause] you

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