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1.

Purpose

To clarify correlations between spinal fracture and delayed paralysis in patients with diffuse idiopathic skeletal hyperostosis (DISH) using computed tomography (CT) with multiplanar reformatting (CT-MPR). DISH increases susceptibility to unstable spinal fractures, leading to neurological deterioration. The pathomechanism of the neurological injury is unclear.

Methods

This multicenter retrospective study included 42 DISH patients (32 male; 10 female) treated for 45 spinal fractures during a 5-year period. The mean age at the time of injury was 77.1 ± 10.1 years. The cause of injury, delay in diagnosis, fracture location, and neurological status were recorded, and anterior- and posterior-column fractures, a fracture displacement over 3 mm, and posterior-column ankylosis were assessed using CT-MPR.

Results

Most fractures (73.8%) resulted from trivial trauma, such as falling from a standing or sitting position. Diagnosis was delayed in 47.6% of the patients, primarily due to delays in seeking medical attention (65.0%). Although 78.6% of the patients were neurologically intact at the time of injury, 54.8% developed paralysis, defined by a change in one or more Frankel-score levels during short-term follow-up. Of the fractures, 39.1% were in the vertebral body, and 60.9% were at the disc level. Fractures with posterior-column ankylosis were significantly associated with delayed paralysis.

Conclusions

CT-MPR was useful for evaluating spinal fractures and determining treatment in patients with DISH. Fractures associated with posterior-column ankylosis resulted in unstable three-column injuries that led to delayed neurological deterioration. Early surgical stabilization of such fractures is recommended to avoid delayed paralysis.  相似文献   

2.
BackgroundDiffuse idiopathic skeletal hyperostosis (DISH) increases the spine's susceptibility to unstable fractures that can cause neurological deterioration. However, the detail of injury is still unclear. A nationwide multicenter retrospective study was conducted to assess the clinical characteristics and radiographic features of spinal fractures in patients with DISH.MethodsPatients were eligible for this study if they 1) had DISH, defined as flowing ossification along the anterolateral aspect of at least four contiguous vertebral bodies, and 2) had an injury in the ankylosing spine. This study included 285 patients with DISH (221 males, 64 females; mean age 75.2 ± 9.5 years).ResultsThe major cause of injury was falling from a standing or sitting position; this affected 146 patients (51.2%). Diagnosis of the fracture was delayed in 115 patients (40.4%). Later neurological deterioration by one or more Frankel grade was seen in 87 patients (30.5%). The following factors were significantly associated with neurological deficits: delayed diagnosis (p = 0.033), injury of the posterior column (p = 0.021), and the presence of ossification of the posterior longitudinal ligament (OPLL) (p < 0.001). The majority of patients (n = 241, 84.6%) were treated surgically, most commonly by conventional open posterior fixation (n = 199, 69.8%). Neurological improvement was seen in 20.0% of the conservatively treated patients, and in 47.0% of the patients treated surgically.ConclusionsMinor trauma could cause spinal fractures in DISH patients. Delayed diagnosis, injury of the posterior column, and the presence of OPLL were significantly associated with neurological deterioration. Patients with neurological deficits or unstable fractures should be treated by fixation surgery.  相似文献   

3.
4.
Primary membranous nephropathy (MN) is an organ-specific autoimmune disease mainly caused by autoantibodies acting against the podocyte antigen M-type phospholipase A2 receptor 1 (PLA2R). Herein we present the clinical and histologic findings, including PLA2R staining, of early recurrent MN after kidney transplantation that was successfully treated with rituximab.A 60-year-old Japanese man had end-stage renal failure due to steroid-resistant primary MN and underwent ABO-incompatible living donor kidney transplantation. At 1 month after transplantation, a protocol biopsy revealed positive granular staining of IgG, C4d, and PLA2R on glomerular capillaries (GCs) without any abnormalities on light microscopy (LM). Although the patient had low-level proteinuria, recurrent MN was suspected based on the positive PLA2R staining; he was treated with an angiotensin receptor blocker and a single dose of 200 mg rituximab. However, proteinuria gradually increased to 877 mg/d. At 21 months after transplantation, a graft biopsy revealed spikes along the outer aspects of GC on LM, with stronger staining for PLA2R than that at 1 month after transplantation. A single dose of 500 mg rituximab was added, which effectively reduced proteinuria, and clinical remission continued until 3 years after transplantation. The latest graft biopsy showed reduced staining of PLA2R. The disease activity and therapeutic effect were well-reflected in the intensity of PLA2R staining.An approach intending an early diagnosis by protocol biopsy using PLA2R immunostaining is made and early treatment with rituximab will help reduce the risk of kidney graft loss due to recurrent primary MN.  相似文献   

5.

Introduction

With the increasing number of elderly kidney donor candidates due to the lack of available donors, prostate cancer has sometimes been detected in these candidates during pretransplant screening examinations. There are currently no guidelines or consensus on prostate cancer screening and treatment in donors. We retrospectively evaluated the clinical course of donor candidates with prostate cancer.

Methods

Between January 2006 and December 2016, 9 donor candidates for living related kidney transplantation were incidentally diagnosed with prostate cancer at our institution. All male kidney transplant donor candidates routinely received prostate-specific antigen (PSA) testing. The patients with PSA levels > 4.0 ng/mL underwent prostate biopsies. For future kidney transplantation, treatment for localized prostate cancer was prostatectomy.

Results

Seven low- or intermediate-risk patients according to the D'Amico risk classification underwent endoscopic prostatectomy, while 2 high-risk patients underwent high dose-rate brachytherapy to prioritize prostate cancer treatment. Of the 7 who underwent surgery, 3 patients ultimately became living related kidney transplantation donors for their wives. There was no recurrence of PSA elevation after treatment.

Conclusion

This study showed that donor candidates with prostate cancer could safely donate a kidney after a thorough evaluation to exclude those with high-risk prostate cancer. Transmission of prostate cancer through kidney transplantation seems unlikely and robot-assisted laparoscopic prostatectomy may be feasible for donor candidates with localized prostate cancer.  相似文献   

6.
BackgroundThe need for donor pool expansion remains an important task for kidney transplantation. The aim of this study is the evaluation of primary nonfunction (PNF) from donation after circulatory death (DCD) kidneys.MethodsBetween 1996 and 2017, 100 kidney transplants from DCD donors were conducted in our department. We retrospectively analyzed PNF of kidney transplant recipients from DCD donors in terms of donors’ and recipients’ epidemiologic characteristics.ResultsOf 100 grafts, 95 recipients (95.0%) had discontinued hemodialysis at the time of hospital discharge. Only 5 recipients (5.0%) developed PNF. All 5 PNF recipients received a single graft from an expanded criteria donor (ECD). The mean donor age in the PNF group was 65.0 (SD, 6.2) years. Significant differences between the PNF group and discontinued dialysis group were found for donor age (P < .01) and for the use of ECD kidneys (P < .02). Nevertheless, no significant difference was found between groups for several factors: a history of hypertension and cerebrovascular events, terminal creatinine levels, and graft weight.ConclusionThe incidence of PNF from DCD kidneys was very low. Although ECD kidneys in older donors might be a significant risk factor for PNF, these findings suggest that DCD kidneys should be used more frequently for donor expansion.  相似文献   

7.

Background

Cost-utility analysis of surgery for degenerative lumber spondylolisthesis (DS) is essential for healthcare providers and patients to select appropriate treatment. The purpose of this study was to review the cost-utility of decompression alone versus decompression with fusion for DS.

Methods

A retrospective review of 99 consecutive patients who were treated for Meyerding grade 1 DS at two representative spine centers was performed. Patients with significant spinal instability were treated by decompression with fusion (F group, 40 patients); all others were treated by decompression surgery alone (D group, 59 patients). All patients were followed for three years. Demographic and radiographic data, health-related quality of life (HRQoL), and the direct cost for surgery were analyzed, and the incremental cost-effectiveness ratio (ICER) was determined using cost/quality-adjusted life years (QALY).

Results

There were no differences between the groups in baseline demographics (D vs. F: age 68 ± 9 vs. 66 ± 7 years; 37% vs. 40% female) or HRQoL (ODI: D, 41 ± 16 vs. F, 46 ± 13%). The F group had a higher initial-surgery cost ($18,992 ± 2932) but lower reoperation frequency (7%) than the D group ($7660 ± 2182 and 12%, respectively). The three-year total direct cost was higher for F than for D ($19,222 ± 3332 vs. $9668 ± 6,168, p = .01). ICER was higher for F at one year ($136,408 ± 187,911 vs. $237,844 ± 212,049, p < .01), but was comparable for F and D at three years (D, $41,923 ± 44,503 vs. F, $51,313 ± 32,849, p = .17).

Conclusion

At the three-year follow-up, the two methods had comparable cost-utility. Both methods were cost-effective (defined as an ICER within three times the per-capita gross domestic product).  相似文献   

8.

Background

Locomotive syndrome is a condition in which the ability to lead a normal life is restricted owing to a dysfunction in one or more of the parts of the musculoskeletal system. Although lumbar spinal canal stenosis (LSS) is considered to cause locomotive syndrome, a detailed assessment of the association between two pathologies has not yet been reported.

Methods

The clinical data of patients aged > 65 years old who planned to undergo surgery for LSS at multiple institutions were prospectively collected from April 2016 to August 2017. A total of 200 participants comprising 120 men and 80 women were enrolled in this study. Association of severity of LSS evaluated by Zurich Claudication Questionnaire scores with three locomotive syndrome risk tests (Stand-up Test, the Two-step Test, and a 25-question risk assessment) and Timed Up-and-Go Test were evaluated.

Results

In the total assessment of locomotive syndrome, 96.5% of the participants were diagnosed as grade 2, and the remaining 3.5% were diagnosed as grade 1. When the participants were divided into 3 groups according to the LSS severity, the scores of all locomotive syndrome risk tests were significantly worse with increasing LSS severity. Logistic regression analysis revealed that LSS severity was positively correlated with the risk level of locomotive syndrome evaluated by the Two-step Test (OR = 3.45, CI = 1.33–8.96).

Conclusions

All LSS patients with surgical indications were diagnosed as having locomotive syndrome. In addition, our results indicated that LSS severity is potentially associated with the progression of locomotive syndrome. The treatment of LSS may be beneficial in alleviating the risk for locomotive syndrome.  相似文献   

9.
The purpose of this report is to describe our experience using cardiopulmonary bypass with selective cerebral perfusion in 10 patients whose aneurysms required cross-clamping the aorta and brachiocephalic vessels. Cerebral perfusion was carried out with individual roller pumps. Flow rate was 11.2 ± 4.8 ml per kilogram of body weight per minute to the right axillary artery, 8.5 ± 1.0 to the right common carotid artery, 7.0 ± 2.0 to the left common carotid artery, and 3.9 to the left subclavian artery. All the patients came off bypass smoothly, and only 3 required small amounts of inotropic agents postoperatively. One patient with mycotic aneurysm died in the fourth postoperative week of massive bleeding due to disruption of the suture line. No cerebral complications were experienced. The operative results of this series suggest the usefulness of cardiopulmonary bypass with selective cerebral perfusion, and reasonably favorable outlook for patients with aneurysm involving the aortic arch and the ascending aorta.  相似文献   

10.

Background

There have been few studies that have reported the influence of kidney transplantation on the quality of life (QOL) of patients of preemptive kidney transplantation (PKT) and nonpreemptive kidney transplantation (NPKT).

Material and Methods

Fifty patients of PKT and 49 patients of NPKT were employed as study subjects. A questionnaire survey using Short Form 36 and Kidney Disease QOL on patients' physical and psychological QOL was performed for these patients prior to transplantation and 1 month, 3 months, and 1 year after transplantation.

Results

The analysis of results has revealed that transplantation clearly has improved the physical and psychological QOL in patients with end-stage renal disease. For the items regarding physical burdens incurred by the transplantation, patient QOL deteriorated on a single occasion 1 month after the transplantation while it was improved 1 year after the transplantation. For the items regarding psychological burdens, the mental condition of the patients was improved overall without deterioration over time. Concerning the “Effect of Kidney Disease” and “Burden of Kidney Disease,” QOL was significantly better in PKT than NPKT at baseline before transplantation, although the significant difference gradually decreased 1 month and 3 months after the transplantation and disappeared after 1 year.

Conclusion

Transplantation certainly improved the QOL of patients with end-stage renal disease. Before transplantation, PKT was clearly better than NPKT in the QOL items associated with “Burden of Kidney Disease.” This indicated that patients of PKT have improved QOL compared to patients of NPKT, and that the overall awareness of kidney disease is decreased. A postoperative gap in mental and bodies was observed especially in PKT, however, could be overcome by nursing interventions.  相似文献   

11.
12.

Introduction

Lymphatic leakage after kidney transplantation is a relatively frequent complication but sometimes resistant to treatment, and there is no fixed treatment algorithm. The effectiveness of therapeutic lymphangiography for postoperative lymphatic or chyle leakage has been reported, but few reports are available regarding patients who have undergone kidney transplantation. In this study, we report our experience with lymphangiography as a therapeutic tool for lymphatic leakage after kidney transplantation.

Patients and methods

Intranodal lymphangiography for lymphatic leakage was performed in 4 patients (3 male, 1 female; age range, 38 to 70 years old) after living kidney transplantation at the Osaka City University Hospital in Japan. The amount of drainage before lymphangiography was 169 to 361 mL/day. The procedure for intranodal lymphangiography was as follows: the inguinal lymph node was punctured under ultrasound guidance, and the tip of the needle was instilled at the junction between the cortex and the hilum, after which Lipiodol was slowly and manually injected.

Results

Lymphangiography was technically successful in 3 out of the 4 patients. In all successful cases, the amount of drainage decreased and leakage finally stopped without additional therapy such as sclerotherapy or fenestration. In 2 cases, we were able to directly detect the leakage site using lymphangiography. The time between lymphangiography and leakage resolution ranged from 8 to 13 days. There were neither complications of lymphangiography nor recurrence of lymphatic leakage in the successful cases.

Conclusions

Intranodal lymphangiography may be not only a diagnostic tool but also an effective, minimally-invasive, and safe method for treatment of lymphatic leakage resistant to drainage after kidney transplantation.  相似文献   

13.
IntroductionMycophenolate mofetil has improved long-term outcomes of kidney transplantation. However, the impact of mycophenolic acid (MPA) trough level on the development of de novo donor-specific anti-HLA antibody (DSA) is unclear. We examined the relation between MPA trough level and de novo DSA development.MethodWe retrospectively studied 617 kidney recipients whose MPA trough level and de novo DSA data were available. All patients underwent primary kidney transplant from living donors from 2008 to 2014, and were chronically treated with a calcineurin inhibitor, mycophenolate mofetil, and +/- steroids. They were equally divided into 4 groups according to the mean trough level of MPA (mMPA) at 1 year post-transplantation: Group 1, mMPA < 2.14 ng/mL (n = 152); Group 2, mMPA 2.14-2.83 ng/mL (n = 157); Group 3, mMPA 2.83-3.57 ng/mL (n = 153); and Group 4, mMPA ≥ 3.57 ng/mL (n = 155). The groups were compared by incidence rate of de novo DSA, graft survival rate, and renal function.ResultsThe incidence rates of de novo DSA were 33.3% in Group 1, 23.7% in Group 2, 22.9% in Group 3, and 30.3% in Group 4 (P = .158). Although there was no significant difference in graft survival rates, a significant difference of renal functions was noted: the higher the renal function, the lower the MPA trough level.ConclusionThe mMPA trough level at 1 year post-transplantation was not statistically associated with the incidence rate of de novo DSA after kidney transplantation.  相似文献   

14.

Background

With the goal of in vivo cultivation of human hepatocytes that have not been sufficient in full differentiation in vitro, the advantage of neonatal thymectomy was verified on expansion of xenogeneic human hepatocyte in the micro-miniature pig (MMP).

Methods

The thymus was excised immediately after the birth of the MMPs via cesarean section. Newborns were fed by artificial feeding under specific pathogen-free conditions. The thymectomized and nonthymectomized littermates were transplanted with human hepatocytes via a portal vein with or without partial hepatectomy at the MMP adult stage.

Results

The growth of thymectomized MMPs and the sham operated littermates was not significantly different; the former weighed 1.98 ± 0.30 kg (average ± standard deviation, n = 4) and the latter weighed 2.28 ± 0.39 kg (n = 4) at 1 month of age, and 17.48 ± 1.92 kg and 16.75 ± 2.68 kg at 12 months of age. Blood thymosin α1 concentrations in the thymectomy group were significantly lower than in the control group (0.22 ± 0.05 ng/mL vs 0.46 ± 0.16 ng/mL; n = 4, 12 months old, P = .029). After human hepatocyte transplantation, human albumin levels were detectable on day 28 in the peripheral blood of the thymectomy plus hepatectomy group (14.3 ± 4.9 ng/mL [± range, n = 2]) but were not detectable even on day 21 in the control group.

Conclusions

Neonatal thymectomy was successfully achieved in infantile MMPs born via cesarean section. These pigs were considered to be an ideal in vivo bioreactor for human hepatocytes.  相似文献   

15.
BackgroundDespite advancements in the management of kidney transplantation (KT), kidney transplant recipients (KTRs) have a higher risk of mortality than the age-matched general population. Improvement of long-term graft and patient survival is a significant issue. Therefore we investigated the effects of postoperative nutritional status on graft and patient survival and explored the predictive factors involved in nutritional status.MethodsOur retrospective study included 118 KTRs who underwent KT at our hospital. Clinical and laboratory data were obtained from medical charts. The prognostic nutritional index (PNI) was used to assess nutritional status. Changes in nutritional status after KT were monitored and the effect of nutritional status on graft and patient survival was investigated. The variables involved in nutritional status were also explored.ResultsThe KTRs in this cohort comprised 66 men and 52 women with a median age of 47 years at KT. There were 16, 32, and 22 cases of cadaveric, preemptive, and ABO-incompatible KTs, respectively. Postoperative PNI gradually improved and was stable from 6 months after KT. Although graft survival was regulated by ABO-compatibility, independent predictors for patient survival were history of dialysis, PNI, and serum-corrected calcium levels. Preemptive KT and inflammatory status contributed to PNI.ConclusionsNutritional status of KTRs improved over time after KT and could contribute to patient survival. Optimal nutritional educational programs and interventions can lead to better outcomes in KTRs. Further studies are needed to validate our results and develop appropriate nutritional educational programs, interventions, and exercise programs.  相似文献   

16.

Objectives

More than two decades have passed since the first living donor liver transplantation was performed in Japan in 1989. There are many reports about problems in adherence to taking medication and medical follow-ups in children who received liver transplants, because there is no transition strategy for those children and parents or guardians. The objective of this study is to measure the effect of nature and outdoor activity to improve children's medical adherence.

Methods

We recruited participants from 9-year-old children who are attending the outpatient liver transplant clinic in a stable condition (no event such as rejection or surgical procedure within 6 months). We took participants to a snow camp and measured its effect by using the IKIRU CHIKARA (IKR) tool, which contain 28 items divided into 3 categories: psychosocial ability, moral fitness, and physical ability. Children were tested on three occasions, before, just after, and 1 month after the camp.

Results

Eight patients participated in the snow camp and 7 patients were eligible for the study. The average age was 12.6 with a range 10 to 17 years. There were 3 girls and 4 boys. The average IKR scores before, just after, and 1 month after the camp were 127.9, 131.5, and 126.6, respectively.

Conclusion

An outdoor activity such as a snow camp can be safely conducted, and it is an acceptable option to incorporate within a pediatric liver transplant program. There were no significant changes in IKR scores during this short observation. Longer observation is needed to measure the effect of nature and outdoor activities.  相似文献   

17.

Background

The aim of the present study is to simulate and better understand the long-term interplay between alumina matrix composite (AMC) femoral head and aqueous environment. In particular, we focused on clarifying the role of alumina grains on zirconia phase stability and mechanical equilibrium in AMC head during aging processes in a clinically-relevant time frame.

Methods

The tested AMC head consists of an alumina matrix (82 vol.%) reinforced by yttria-stabilized zirconia (17 vol.%), chromium oxide (0.5 vol.%), and strontium aluminate (0.5 vol.%). The accelerated hydrothermal aging testing (at 134 °C, 2-bars pressure) was conducted up to 20 h. According to ASTM standard, 1-h aging under these conditions corresponds approximately to 2 yrs in vivo exposure. Confocal Raman and fluorescence spectroscopy were applied to quantify surface tetragonal-to-monoclinic phase transformation of zirconia and surface stresses in the AMC head. The Mehl–Avrami–Johnson (MAJ) theory was applied, which allowed modeling the micromechanisms of nucleation and growth of monoclinic sites during the transformation process. The obtained results were compared to those of monolithic zirconia (3Y-TZP) femoral heads.

Results

The 3Y-TZP head showed a rapid increase of transformation rate beyond the aging time of 5 h (simulated as ~10 yrs in vivo), suggesting the initiation of the transformation cascade toward the neighboring zirconia grains (growth mechanism). On the other hand, MAJ analysis revealed that the growth mechanism was completely absent and the nucleation of the monoclinic phase was partially prevented in the AMC head even after the 20-h aging (~40 yrs in vivo). In addition, the stress accumulation in the AMC head was restricted at a quite low level throughout the aging simulation.

Conclusion

Those results suggest that the presence of stable and hard alumina in the AMC can play a considerable role in slowing down the destabilization processes by spatially encompassing zirconia grains.  相似文献   

18.
The HMG-CoA reductase inhibitor (statin), which reduces serum cholesterol, has been demonstrated in the control of immune responses and may potentially play an important role in the regulation of acute and chronic rejection in organ transplantations. We investigated the graft-protective effect of a kind of statin, pravastatin, in the survival of fully major histocompatibility complex--mismatched murine cardiac allograft transplantation. Fully vascularized heterotopic hearts from C57BL/6 donors were transplanted into CBA recipients through microsurgical techniques. CBA recipients transplanted with a C57BL/6 heart received oral administration of 40, 120, or 400 μg/kg/day of pravastatin from the day of transplantation to 7 days afterward. Immunohistochemical staining studies were performed to determine whether intimal formation of coronary arteries in the transplanted cardiac allografts was preserved and also to conduct morphometric analysis. Untreated CBA recipients rejected C57BL/6 cardiac grafts acutely (median survival time [MST] 7 days). CBA recipients exposed with 40 and 120 μg/kg/day of pravastatin had a small prolonged allograft survival (MSTs of 10 and 9 days, respectively). However, the MST of CBA recipients exposed to 400 μg/kg/day of pravastatin was significantly effective for allograft survival (MST 50 days). Immunohistochemical staining assessments on 4 weeks after grafting showed suppression of intimal hyperplasia in allograft coronary arteries. Pravastatin could induce the prolongation of fully major histocompatibility complex--mismatched cardiac allograft through the protection of the coronary artery.  相似文献   

19.

Background

Although living donor liver transplantation for obese recipients has increased, it has not been determined that posttransplant outcomes in obese recipients are inferior compared with nonobese recipients.

Methods

From January 2001 to December 2016, there was a total of 58 (6%) obese patients (body mass index?≥30) in a cohort of 973 adult patients that underwent living donor liver transplantation. Propensity score matching and classification were performed based on the type of obesity, and there were 58 patients in the obese group and 141 patients in the nonobese group. We performed comparative analysis of posttransplant outcomes including Model for Early Allograft Function (MEAF) scoring and early allograft dysfunction (EAD).

Results

EAD was found in 11 (19%) and 31 (22%) patients in the obese and nonobese groups, respectively (P = .71). The obese group had a higher MEAF score than the nonobese group (5.2 vs 4.5, P = .007). The mean hospitalization of the obese group was shorter than in the nonobese group (32 vs 42 days, P = .003). Other posttransplant outcomes were similar between the obese and nonobese groups, including acute cellular rejection (8 vs 10 cases, P = .17), early graft failure (8 vs 12 cases, P = .30), index hospital mortality (6 vs 11 cases, P = .58), and comprehensive complication index (26.0 vs 24.6, P = .76).

Conclusion

Posttransplant outcomes of the obese group were not inferior to the nonobese group. However, obesity can impact the severity of EAD and the incidence of early graft failure, based on significantly higher MEAF scores.  相似文献   

20.
BackgroundIntrapulmonary shunt (IPS) is recognized in 10% of chronic liver disease patients. Liver transplantation (LT) is associated with a high risk of morbidity and mortality in patients with IPS.Patients and methodsOf 519 pediatric LT cases between November 2005 and October 2018, 50 patients with IPS were enrolled in this study. The patients were divided into 3 groups, according to the shunt ratio, calculated by scintigraphy: mild (15%-20%, n = 26), moderate (20%–40%, n = 19), and severe (> 40%, n = 5). We compared the patients’ characteristics before LT and the outcomes of LT between these groups.ResultsThe major original disease resulting in LT in the mild and moderate groups was biliary atresia (73.1% and 52.6%, respectively), while that in the severe group was congenital portosystemic shunt (60%). The median ages at LT were 7.5, 6.1, and 8.3 years in the mild, moderate, and severe groups, respectively. All of the mild and moderate IPS patients lived; however, 3 patients with severe IPS (60.0%) died within 3 months. The shunt ratios of the mild and moderate IPS patients normalized within 2 years after LT, while the 2 surviving severe IPS patients showed a slight improvement. The autopsy findings of the lung in 1 deceased severe IPS patient showed medial hypertrophy and proliferation of intimal cells of the pulmonary arteries, suggesting a diagnosis of portopulmonary hypertension.ConclusionsLT can be safely performed for mild and moderate IPS patients; however, LT for severe IPS patients should be carefully indicated because concomitant portopulmonary hypertension may be masked by IPS.  相似文献   

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