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1.
Cheng F, Qin J‐J, Yu M‐N, Zhang F, Li X‐C, Sun B‐C, Kong L‐B, Wang X‐H. De novo phyllodes tumor in an adolescent female after liver transplantation.
Pediatr Transplantation 2011: 15:E12–E14. © 2009 John Wiley & Sons A/S. Abstract: Phyllodes tumor of the breast is a rare disease constituting 0.3–0.9% of all breast neoplasms. Occurring mainly in females aged 35 to 55 yr, the disease is especially rare among adolescent females. There is no published literature about de novo phyllodes tumor after liver transplantation. Here we describe a case of de novo phyllodes tumors in an adolescent female after liver transplantation from a living donor for Wilson disease.  相似文献   

2.
The combination of pediatric multivisceral and kidney transplantation leads to additional recipient risks due to the number of anastomoses and to the small sizes of donor structures. The inclusion of donor kidneys, ureters, and a bladder patch en bloc with multivisceral organs decreases the number and complexity of anastomoses and has not yet been reported. Four patients were transplanted in this fashion; three underwent multivisceral‐kidney and one underwent liver‐kidney transplantation. The first patient was a 3‐year‐old male with polycystic kidney disease and congenital hepatic fibrosis. The second was a 7‐year‐old female with complications from necrotizing enterocolitis. The third was a 12‐month‐old male with megacystis microcolon intestinal hypoperistalsis syndrome and secondary hydronephrosis, and the fourth was a 3‐year‐old male with multiple intestinal resections secondary to incarcerated hernia. The third patient developed a right ureteral stenosis with an intact bladder patch. The fourth child expired from maintained abdominal sepsis. The first 3 patients maintained normal graft function. There were no cases of thrombosis, arterial stenosis, or urinary leakages. These reported cases demonstrate that small pediatric en bloc transplantation of the multivisceral organs and dual kidneys with a bladder patch anastomosis is a feasible and less complex alternative to the standard procedure.  相似文献   

3.
We present a case of a three‐yr‐old child with a history of multisystem Langerhans cell histiocytosis treated with systemic chemotherapy, who developed progressive liver failure and received an orthotopic split liver transplant while continuing on chemotherapy. One month following transplant, he developed acute graft‐vs.‐host disease of the skin and gastrointestinal tract. Peripheral blood chimerism studies post‐transplant demonstrated an increasing predominance of donor lymphocytes and granulocytes. Shortly after, the patient developed vitiligo, and two yr after transplantation, the patient developed skin manifestations of psoriasis. We discuss and review the current literature, which demonstrates that chimerism following liver transplantation is rare and in our patient may be related to his profound immunosuppression around the time of liver transplant as well the development of acute graft‐versus‐host disease. While autoimmune disease can occur after solid organ and stem cell transplant, our patient developed skin manifestations of autoimmunity after liver transplantation, which is also rarely described.  相似文献   

4.
Primary hyperoxaluria type 1 (PH1) is an inherited metabolic disease that culminates in ESRF. Pre‐emptive liver transplantation (pLTx) treats the metabolic defect and avoids the need for kidney transplantation (KTx). An institutional experience of pediatric PH1 LTx is reported and compared to the literature. Between 2004 and 2015, eight children underwent pLTx for PH1. Three underwent pLTx with a median GFR of 40 (30–46) mL/min/1.73 m2 and five underwent sequential combined liver‐kidney transplantation (cLKTx); all were on RRT at the time of cLKTx. In one case of pLTx, KTx was required eight and a half yr later. pLTx was performed in older (median 8 vs. 2 yr) and larger children (median 27 vs. 7.75 kg) that had a milder PH1 phenotype. In pediatric PH1, pLTx, ideally, should be performed before renal and extrarenal systemic oxalosis complications have occurred, and pLTx can be used “early” or “late.” Early is when renal function is preserved with the aim to avoid renal replacement. However, in late (GFR < 30 mL/min/1.73 m2), the aim is to stabilize renal function and delay the need for KTx. Ultimately, transplant strategy depends on PH1 phenotype, disease stage, child size, and organ availability.  相似文献   

5.
To evaluate clinical and laboratory findings of these patients and the efficacy of liver transplantation in children with hepatocellular carcinoma (HCC) and hepatoblastoma (HB) associated with tyrosinemia. Among 113 children with liver tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular clinic visits for tyrosinemia, elevated alpha-fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given chemotherapy including cisplatin and adriamycin. In three patients, living-related liver transplantation was performed. They had no treatment after transplantation. All of them are disease free. One patient was treated with chemotherapy and right hepatectomy. She had no suitable donor for living-related liver transplantation. Three months after completing chemotherapy, she had recurrent tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living-related liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for tyrosinemia. In these patients liver transplantation is curative both for liver tumor and tyrosinemia.  相似文献   

6.
One of the main limiting factors in pediatric liver transplantation is donor availability. For adults, DCD liver grafts are increasingly used to expand the donor pool. To improve outcome after DCD liver transplantation, ex situ machine perfusion is used as an alternative organ preservation strategy, with the supplemental value of providing oxygen to the graft during preservation. We here report the first successful transplantation of a pediatric DCD liver graft after hypothermic oxygenated machine perfusion. The full‐size liver graft was derived from a 13‐year‐old, female DCD donor and was end‐ischemic pretreated with dual hypothermic oxygenated machine perfusion. Arterial and portal pressures were set at 18 and 4 mm Hg, slightly lower than protocolized settings for adult livers . During 2 hours of machine perfusion, portal and arterial flows increased from 100 to 210 mL/min and 30 to 63 mL/min, respectively. The pretreated liver graft was implanted in a 16‐year‐old girl with progressive familial intrahepatic cholestasis type 2. Postoperative AST, ALT, and prothrombin time normalized within a week. The recipient quickly recovered and was discharged from the hospital after 18 days. One year after transplantation, she is in excellent condition with a completely normal liver function and histology. This case is the first report of successful transplantation of a pediatric DCD liver graft after hypothermic oxygenated machine perfusion and illustrates the potential role of ex situ machine perfusion in expanding the donor pool and improving outcome after pediatric liver transplantation.  相似文献   

7.
The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with β‐thalassemia intermedia. A patient, 6‐year‐old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver. Hepatocellular injury due to acute graft rejection might have contributed to elevated ferritin levels by causing release of stored iron from the hemosiderotic liver graft. Iron chelation and phlebotomy therapies were started simultaneously in the early postoperative period to avoid iron‐related organ toxicity and transplant failure. Follow‐up with monthly phlebotomies after discharge yielded a favorable outcome with normal transplant functions. Thalassemia intermedia patients can be candidates of liver donors to decrease pretransplant waitlist mortality. After transplantation of a hemosiderotic liver, it is important to monitor the recipient in terms of iron overload and toxicity. Early attempts to lower iron burden including chelation therapy and/or phlebotomy should be considered to avoid organ toxicity and transplant failure.  相似文献   

8.
Dantuluri S, Urs A, Karthik SV. Follicular carcinoma of thyroid following successful liver transplantation – A report. Abstract: Follicular carcinoma of the thyroid is a relatively rare malignancy in childhood even in paediatric solid organ transplant recipients. The risk of developing de novo malignancies after liver transplantation is higher compared to the general population. We report an 18‐yr‐old girl who had successfully undergone liver transplantation five yr earlier for neonatal sclerosing cholangitis complicated by the development of dysplastic nodules. Baseline immunosuppression was with tacrolimus and prednisolone. Mycophenolate mofetil was later added in view of steroid‐resistant episodes of graft rejection. She subsequently suffered from marked obesity and essential hypertension needing antihypertensive medication. Five yr after liver transplantation, she presented with a right‐sided thyroid swelling that was rapidly progressive with no associated lymphadenopathy and normal systemic examination. Ultrasound of her neck revealed a solid lesion in the right lobe of the thyroid gland with ill‐defined margins, and a diagnostic right thyroid lobectomy confirmed the diagnosis of follicular carcinoma with focal capsular and vascular invasion. She underwent total thyroidectomy and currently remains well on thyroxine supplements. Our report highlights the need for high level of suspicion and prompt investigation into any abnormal lesion in the long‐term follow‐up of solid organ transplant recipients.  相似文献   

9.
The natural history and survival of children with fibrocystic liver–kidney disease undergoing solid organ transplantation have infrequently been described. We report outcomes in a cohort of US children with fibrocystic liver–kidney disease receiving solid organ transplants over 20 yr. Retrospective cohort study of pediatric transplant recipients with diagnoses of fibrocystic liver–kidney disease from 1/1990 to 3/2010, using data from the SRTR. Subjects were categorized by the first transplanted organ: LT, KT, or SLK. Primary outcomes were death, re‐transplant, transplant of the alternate organ, or initiation of dialysis. Seven hundred and sixteen subjects were transplanted in this period. Median age at first transplant was 9.7 yr. Of the LT, 14 (19%) required a second liver transplant at median of 0.2 yr, and five (7%) required kidney transplant or dialysis at a median of 9.0 yr. Of the KT, 188 (31%) required a second kidney transplant or dialysis at a median of 5.9 yr. Twenty‐nine (5%) subsequently received liver transplant at a median of 6.0 yr. Among patients in this registry, far more children underwent kidney than liver transplants. The risk of subsequently needing transplantation of an alternate organ was low.  相似文献   

10.
Englert C, Ganschow R. Liver transplantation in a child with liver failure due to chronic graft‐versus‐host disease after allogeneic hematopoietic stem cell transplantation from the same unrelated living donor. Abstract: We report a case of a six‐yr‐old boy who developed chronic GVHD of the liver, intestines, and skin following allogeneic hematopoietic SCT. The boy received an allogeneic hematopoietic stem cell transplant at the age of two yr because of early recurrence of ALL. Chimerism analysis showed complete chimerism. In the following year, he developed GVHD despite adequate immunosuppressive therapy. Liver biopsy showed liver GVHD resulting in liver cirrhosis by the age of five yr. LTx was performed with a left liver lobe from the unrelated donor from whom the stem cells had been taken. Immunosuppressive therapy consisted of low‐dose steroids and low‐dose cyclosporine. The postoperative course was uneventful. Graft function was excellent, and we performed protocol biopsies at seven days and three wk as well as three, six, and nine months after transplantation; none of these showed any signs of rejection or GVHD. Immunosuppressive therapy was discontinued nine months after LTx. Three yr after transplantation, the boy is in good condition with normal graft function. To our knowledge, this is the first report on LTx following allogeneic hematopoietic SCT from the same unrelated living donor.  相似文献   

11.
LT has emerged as a surgical treatment for UCDs. We hypothesize that LT can be safely and broadly utilized in the pediatric population to effectively prevent hyperammonemic crises and potentially improve neurocognitive outcomes. To determine the long‐term outcomes of LT for UCDs, charts of children with UCD who underwent LT were retrospectively reviewed at an academic institution between July 2001 and May 2012. A total of 23 patients with UCD underwent LT at a mean age of 3.4 yr. Fifteen (65%) patients received a whole‐liver graft, seven patients (30%) received a reduced‐size graft, and one patient received a living donor graft. Mean five‐yr patient survival was 100%, and allograft survival was 96%. Mean peak blood ammonia (NH3) at presentation was 772 μmol/L (median 500, range 178–2969, normal <30–50). After transplantation, there were no episodes of hyperammonemia. Eleven patients were diagnosed with some degree of developmental delay before transplantation, which remained stable or improved after transplantation. Patients without developmental delay before transplantation maintained their cognitive abilities at long‐term follow‐up. LT was associated with the eradication of hyperammonemia, removal of dietary restrictions, and potentially improved neurocognitive development. Long‐term follow‐up is underway to evaluate whether LT at an early age (<1 yr) will attain improved neurodevelopmental outcomes.  相似文献   

12.
Merli L, Grimaldi C, Monti L, Nobili V, Francalanci P, de Ville de Goyet J. Liver transplantation for refractory severe pruritus related to widespread multifocal hepatic focal nodular hyperplasia (FNH) in a child: Case report and review of literature.
Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S. Abstract: FNH is a rare and benign tumor of the liver. It is not a conventional indication for liver transplantation, and no transplant for FNH in a child has been reported to date. Multifocal FNH growing in adolescent age to a widespread tumor invading the whole liver and associated with severe refractory pruritus was an unusual indication for transplantation in a 13‐yr‐old girl. The operation and the follow‐up were uneventful, allowing full recovery and disappearance of pruritus.  相似文献   

13.
Urahashi T, Mizuta K, Sanada Y, Wakiya T, Yasuda Y, Kawarasaki H. Liver graft volumetric changes after living donor liver transplantation with segment 2 graft for small infants. Abstract: LT for small infants weighing <5 kg with liver failure might require innovative techniques for size reduction and transplantation of small grafts to avoid large‐for‐size graft, but little is known about post‐transplant graft volumetric changes. Five of 172 children who underwent LDLT received monosegment or reduced monosegment grafts using a modified Couinaud’s segment II (S2) graft for LDLT. Serial CT was used to evaluate the changes in the GV and other factors before LDLT and one and three months after LDLT. The shape of these grafts was classified into an OL type and an LL type. The GV increased in all patients one month after LDLT, whereas the GV decreased three months after LDLT in OL in comparison with one month after LDLT. The GRWR of the OL type has tended to decrease at three months, whereas the LL type showed a continuous increase with time, but finally they had adapted graft size for their body size. In conclusion, the volume of S2 grafts after LDLT had unique changes toward the ideal volume for the child weight when they received the appropriate liver volume.  相似文献   

14.
Outcomes of pediatric liver transplantation have constantly improved in the last decade. Living‐related liver transplantation does not seem to improve long‐term outcomes following liver transplantation, but few studies have evaluated immunological parameters of the alloimmune response after living vs. deceased donor organ transplantation. We analyzed numbers of regulatory T cells, lymphocyte subsets, and serum cytokine concentrations in 12 pediatric recipients of living‐related liver transplants and in 28 pediatric recipients of deceased donor organs during their annual follow‐ups. Transplant recipients who underwent living donor organ transplantation had significantly higher numbers of regulatory T cells and IL‐4 serum concentrations than recipients of deceased donor organs; both of these factors are associated with beneficial outcomes and transplantation tolerance. Living‐related liver transplantation may have potentially beneficial immunological aspects, although long‐term outcomes do not seem to be better in recipients of living donor organs than in recipients of deceased donor organs. Further studies are needed to compare immunological aspects of the two transplant procedures.  相似文献   

15.
Gozzini S, Perera MTPR, Mayer DA, Mirza DF, Kelly DA, Muiesan P, Sharif K. Liver transplantation in children using non‐heart‐beating donors (NHBD).
Pediatr Transplantation 2010: 14:554–557. © 2010 John Wiley & Sons A/S. Abstract: Selected livers from controlled NHBD are accepted for OLT in adults. Recent evidence has shown good medium‐term outcome. The purpose of this study was to report our experience of pediatric OLT with whole and partial grafts from NHBD, analyzing complications and outcome. Retrospective review of all the recipients who underwent primary OLT between December 2005 and December 2008, using livers from NHBD. Four children (one male child) mean age was 9.5 yr (0.2–17), mean weight was 26 kg (range 2.6–48), underwent OLT using NHBD. Mean donor age was 14.2 yr, and mean WIT (systolic BP <50 mmHg to cold perfusion) 12.2 min (range 10–15). Two children received reduced grafts and two full grafts. Mean cold ischemia time was 7.18 h (range 6–8). Liver function tests one wk and nine months post‐OLT confirmed a good graft function. One child was treated for two episodes of acute rejection. Post‐transplant complications included two cases of mild ischemic cholangiopathy treated conservatively. Graft and patient survival was 100% with a mean follow‐up of 19 months (range 8.1–43.4). Short‐ to medium‐term follow‐up suggests that liver grafts from young NHBD with short warm and cold ischemia times can be safely utilized in pediatric transplantation.  相似文献   

16.
Hepatic artery thrombosis (HAT) following pediatric liver transplantation increases morbidity and risk of graft failure. We performed a retrospective chart review of all patients who underwent deceased‐donor liver transplantation from August 2002 to July 2016. Multi‐organ transplant recipients were excluded. We examined the incidence of HAT at our institution and sought to identify associated donor or recipient risk factors. A total of 127 deceased‐donor liver transplant patients with a median age of 1.7 years (IQR 0.67‐6.7) were identified. Of those, 14 developed HAT, all weighing under 25 kg. Among 100 patients under 25 kg, whole‐liver graft recipients had an odds ratio of 3.98 (95% confidence interval [CI]: 1.03, 15.34; P = .045) for developing HAT compared with split‐liver graft recipients. Within the whole‐liver recipient group under 25 kg, 11 patients developed HAT with a median donor‐to‐recipient ratio (DRWR) of 0.9 (IQR: 0.7‐1.2) compared with a median DRWR of 1.4 (IQR: 1.1‐1.9) for those who did not develop HAT. Multivariate analysis showed DRWR to be an independent risk factor for HAT in patients weighing under 25 kg who received whole organ grafts, with an odds ratio of 3.89 (95% CI: 1.43, 10.54; P = .008) for each 0.5 unit decrease in DRWR. Our results suggest that in recipients under 25 kg 1) split‐liver grafts may have a lower rate of HAT and 2) selecting whole organ donors with a higher DRWR may decrease the incidence of HAT.  相似文献   

17.
Udagawa T, Kamei K, Ogura M, Tsutsumi A, Noda S, Kasahara M, Fukuda A, Sakamoto S, Shigeta S, Tanaka H, Kuroda T, Matsuoka K, Nakazawa A, Nagai T, Uemura O, Ito S. Sequential liver–kidney transplantation in a boy with congenital hepatic fibrosis and nephronophthisis from a living donor.
Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S. Abstract: A five‐yr‐old boy developed chronic liver failure and ESKD because of CHF and juvenile NPHP. He underwent sequential liver and kidney transplantation with a compatible blood type from his father, at five yr, seven months and five yr, 11 months old, respectively. Because the patient was not in ESKD, we initially performed LDLT because of significant portal hypertension. Even after LDLT, his ascites was not ameliorated, and he needed continuous drainage of ascites and daily albumin and gamma globulin infusion. Thereafter, he progressed to ESKD and needed hemodialysis for one month before LDKT. CDC crossmatch for donor B cells in the warm test, FCXM for B cell IgG, and flow PRA for donor class II were positive before LDKT. After pretreatment of three courses of plasma exchange and intravenous gamma globulin, LDKT was performed. Two weeks after LDKT, AIHA concomitant with autoimmune thrombocytopenia, also called Evans syndrome, occurred because of passenger lymphocytes from the donor; however, the patient was successfully treated with intravenous methylprednisolone. Eighteen months have passed since LDKT, and liver and kidney function in both the recipient and donor are normal.  相似文献   

18.
MSUD occurs due to deficiency of enzyme BCKAD required for metabolism of leucine, isoleucine, and valine leading to the accumulation of these and their ketoacids causing acute metabolic decompensation manifesting as encephalopathy or sudden death. The patient requires special protein‐restricted diet to survive. As this enzyme is expressed in liver, liver transplantation has been successfully performed as a cure. We report two patients of MSUD who underwent LDLT while their livers were used as a domino graft for other biliary cirrhotic patients. A 22‐month‐old male child diagnosed as a case of classic MSUD underwent LDLT from an altruistic aunt as donor following which his serum leucine levels normalized on an unrestricted protein diet. His liver was used as a domino graft. A 38‐month‐old female child with diagnosed MSUD underwent LDLT from a swap donor, and her liver was used as a domino graft. Her DQ improved post‐transplant. LDLT from non‐heterozygous donors is a cure for classical MSUD. Their livers can be used as domino grafts for non‐MSUD cases.  相似文献   

19.
Complete microscopic tumor resection is critical for successful treatment of hepatoblastoma, and this may include when liver transplantation is required. For tumors involving the IVC or PV, complete resection should include the involved IVC or PV to ensure full tumor clearance. When this is required, the venous reconstruction at transplant or post‐excision can be challenging. We present the management of an 18‐month‐old girl with PRETEXT Stage IV (P, V, F) hepatoblastoma and IVC involvement, where native caval resection and reconstruction was required. The preoperative staging following neoadjuvant chemotherapy was POSTTEXT Stage IV (P, V, F). An orthotopic liver transplantation was performed using a left lateral segment graft from a deceased adult donor. With native hepatectomy, retrohepatic IVC resection from just above the hepatic venous confluence to just above the entry of the right adrenal vein was performed. For caval reconstruction, a venous graft from a deceased donor was used. The graft included the lower IVC with the right common iliac vein and a short stump of the left common iliac vein. The common iliac was a perfect size match for the IVC, and the three natural ostia matched the upper cava, lower cava, and the outflow from the donor left hepatic vein. The patient had an uneventful postoperative course and remains well and disease‐free 2 years after transplant with continued patency of the reconstructed cava. When indicated, a donor iliac vein graft with its natural ostia should be considered in caval reconstruction for pediatric liver transplantation.  相似文献   

20.
The technique of liver splitting offers an effective way of increasing the donor pool and decreasing pediatric waiting list mortality. A donor liver is divided in such a way that the left lateral liver graft can be transplanted into a small child and the right extended liver graft into an adult. This innovative technique did not harm the adult recipient pool. Because of its technical complexity and the initial poor results after split liver transplantation (SLT) this procedure has slowly gained acceptance in the Transplantation Community after its first introduction in 1988 (4). Small children with end stage liver disease suffered the most from the extreme shortage of cadaveric donor organs due to the difficulty of finding size-matched donors. The successful surgical development of SLT and a better donor and recipient selection have led to a reduction of the pediatric pretransplant mortality to nearly zero and to results comparable with those after whole organ transplantation (WLT). By splitting a donor organ into two 'full' hemi-grafts and providing a small adult ( < 60 kg) or a big child ( > 30 kg) with the full left graft and a medium-sized adult (60-80 kg) with the full right graft, a small-for-size situation for adolescents or adults can be avoided and the total number of available grafts can be increased. It is the goal to provide each recipient with its customized graft in the near future. However, splitting for two adults requires high technical skills and profound knowledge of the anatomic variations and should be performed in centers with large transplantation experience.  相似文献   

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