Adult Outcomes: Hirschsprung Disease

OK, good. Because there's something wrong with the other phone. This is the 3rd phone out. I, I've, I've, I've tried but hope, hopefully this works. So I was telling you why these studies can be made in Finland studies, and the main reason is that we have a social security number. For all Finns. And we can track all our patients back. We know their whereabouts and we also know their sort of medical history from the national records. Let's go on with the talk. We did a study on adults. Uh, with this disease, um, we've studied the bowel function and we assessed also the gastrointestinal quality of life. Um, uh. The basis for this study was that actually there are no or very few controlled studies in adults who have been operated on for her for her in their childhood. And There are some follow up studies. There are many follow up studies, but no studies that have that have used control populations of healthy healthy individuals. This was a population-based cross-section study and operate. In our institution in 1960 and 1986 of the patients have died and had migrated left us 143 patients who were eligible for the study. Uh, most of the patients had clinical interview and we used 86 match controls without any previous surgery for our patients. The operations that were performed. We mainly Duhamel operations. about the life that the figures have changed the other operation used with the founder and also the the European operation the operation in about 10% of our patients and 25% of patients had. OK. But what happened to the flight? Sorry about that. Hold on one second. OK, Um We compared the patients also the patients with the responders who took part in the study with the non the nonresponders, and in this study we didn't find any, any difference between the between the responders and on the nonsense in terms of surgery, the type of surgery. And the and the and the and the and the. We used the function of validate score for the function in in health the average score is 1.1. The low score means that that function and the high score means a very good bow function. We also a score score that is 36 valid score for health related quality of life. This it records the physical and social function and also most states of all the patients. The average score in healthy adults is 125. 8. The maximum score is 144. Uh, you, you can see from this slides, uh, slide that, that, uh, that, uh. There were problems in the, in the, in the bowel function when compared with the with the with the controls. Uh, the The Uh, constipation was much more frequent, with the, uh, uh, patients, uh, sodium was much more frequent. The certificate was, was much more frequent and also the problems were much more frequent in the patients than in the controls. The mean bowel function score of the adult patients with 17.1. And it was 19.1, and this this difference was statistically significant. Uh, however, uh, uh. 25% of all the patients scored the full 20 points uh as opposed to 50% of the con con con to controls. 13% of the patients report frequent soiling. 2 had accidents and 10% had complication that requires treatment. And this figure shows how the Uh It's disappeared again, but you control it? Can we just control it and advance the size. So Doctor, I'll be moving your slides. You just tell me when you, when you want the next slide. I'll be moving them, OK, OK. Oh sorry, thanks. So I don't touch that. OK, that's OK. So uh from this uh figure you can see that um. That definitely the bowel functions of the patients with with the with the bowel function is not at the same level than in health in individuals. The the gas of life on the other hand is mostly at the same level than in the in the health population. There are some, some individuals that had the worst outcome in terms of life, but the people who did worse also in the healthy population. Um In the patients, the GI scores, the scores were marginally lower in the patients, but this difference was not statistically significant. However, 22% of the patients had a score lower than 110 that indicates poor get different quality of life. The patients scored worse in 11 on the on the 36 items and and these items are on the on the on the on the on the table. Um Interestingly, the patients scored better. In the ability to participate in this activities and and and also in the quality of sleep and control. This is very difficult to explain but this kind of questionnaire type studies can give results like this. Uh By doing a sort of multivariate logistic regression analysis, we assess the predictors of poor outcomes, which means the score lower than 17. And the poor quality of life, which means score lower than 110. And this where age surgery after surgery. and primary diversion and in the in the in the quality of life issues that predicts what age, gender, the body mass index and bowel function score and what we found out that the increasing age was the only significant predictor of poor functional outcome. And we also found that age was inversely related to the score in the patients but not in controls, and this is sort of a worrying finding, which means that we may expect that our patients that might do quite well when they're young adults might not do so well when they age. And in terms of quality of life, the low bowel function score was the only predictor of poor quality of life. Next slide please. We conclude that the bowel functions continues to be impaired in a significant proportion of adults with er. Abnormalities in the urge to defecate, fecal soiling, constipation, and social problems in relation to bowel function affect about 1/3 to 15% of the patients. Increasing age increases the risk of poor functional outcome. However, the overall gastrointestinal quality of life appears to be comparable its controls, uh, but decreased, um, uh, Gaston's quality of life was found in 1/5 of the patients. And then I continue with the with the next slide. But some of our more recent studies concerning the outcomes of long segment gangliosisgnosis affecting the entire colon with or without small. in this next slide please. We looked at all the concept of patients who were born between 1984 and 2013 and treated. In our institution and these patients suffered from total colonic anosis with a very extension to the small bowel. This is sort of retrospective case note review. And the patient characteristics bowel function dependence on nutrition growth and survival. Next slide please. In total we identified 25 patients, 21 patients, 15 of these patients were male and 6 female. of the patient for me. And syndrome were found in 4 patients. 2 had causes hair ablation, typical Finnish syndrome which occurs also in the Amish population in the US. 21 patient had a, had a central uh hyperventilation syndrome, had that syndrome with a neuroblastoma, and one patient had a Sch-Wardenberg syndrome. The median follow-up of these patients were 6.5 years. This gives you the next like gives you idea what so how how the patient were doing by the level of 5 patients had the anosis extending very near. To do it, do it in general uh uh uh uh lecture. So they, they have basically sort of total bow egg in their nose. all the patients and for us to alive. Only one has been weaned from parental. The and and all of these patients have a bowel continue. One of the patients and underwent successful small bowel transportation you know and the other patient is waiting for for small about transportation. There were 4 patients who were where the agnosis extended to the mid small bowel level. Uh, I think the phone, his phone went out again. Um Mark, we bring the microphone back up. I, why don't you try to call back in. I think the phone looks like the phone crashed again. So while we are getting Doctor Rintala back, I just, I don't know how many people were able to hear why Finland has such good long-term outcomes, but they have a social security number that is national and they can track medical history, so even if your patient have an operation in another hospital, you can still look at that because when we try to do our long-term follow-up, we always. Have the risk of the ones responding. Many people would say are the ones doing well or are the ones not doing well, so we have a kind of a, a biased view. And in this case they can track all patients from life until death. They can have access to the entire medical history, and I think this is fantastic for learning and to learn what truly happens with a disease as it progresses over years. So hello, hello, yeah. We can hear you. Do you hear me? Yes, perfect. You can go ahead, Dr. Intala. OK, yeah, yeah. Andrea, can you, can you check the previous slide, OK. Yeah, OK, And so I was left, I was left to the in patients who had a gangnosis extending to the mid small bowel. And these patients have a genuine you know pull through um uh uh. But Two of these patients had had a syndromatic disease that makes small transplantation contraindicated two of these patients died because of of of severe bowel motility and the recurrent septic septic infections. None of these patients have been weaned on on nutrition and only one of the patients have about continue continue. There were several patients with the was was less than 50% of. of the patients had pouch and one had a left mark type operation. All the patients patients are alive. None of these patients are on the end and all and and all patients have continuity. They were. 5 patients with the purely colony to the and the notices this patient and they all alive that we can be and and and they all still have about continue nextli please. Um This shows the the the the the duration of of the uh of the uh you can see from this slide that it extends to the mid uh meet the small bowel or more approximately very few of the patients can uh can uh can be weaned from uh from. and also this affects the survival the longer the the the second the survival next like this. And these are the functional outcomes. In patients who had a pouch anal anastomosis, all of the patients have voluntary bowel movement. Motions of the 9 which have been toilet trained. The stooling frequency for 24 hours is 4, ranging between 1 and 210. 4 of the 10 patients have bowel motions at nighttime. 2 of them have uh have have have fe something uh to some degree. None of the patients is suffering from constipation. Um, most of the patients had, had at least one episode of enteritis, um, in these patients, um, uh, the episodes were treated by oral, uh, and, and antibiotics, mainly, uh, uh, metronidazole or cifloxacillin. Obstru episodes were common also and these were mainly treated by by by the injections some of the patients require more than 2 injection and maximum 6 next. To conclude, Intended their noses beyond 50 centimeters of of this island, the achievement of functional continuity or autonomy is exceptional and long-term survival poor without interstellar transportation. If the egg gangnosis is, this is outlook bowel function is reassuring after uh uh a pouch anal anastomosis, although obstructive symptoms and enter colitis are frequent but manageable with Botox and metronidazole. We need to continue, uh, to follow up these patients to assess the long-term outcomes, outcomes, um, after, after, uh, other pouch, anastomosis. Thank you very much. Thank you so much Doctor Rintala. If anybody has any questions.