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1.
Am J Transplant ; 2024 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-38908484

RESUMO

Transplantation of non-US citizen residents remains controversial. We evaluate national trends in transplant activity among pediatric noncitizen residents (PNCR). Pediatric liver and kidney transplant data were obtained from the Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients. Data on transplanted organs, region, waitlist additions, procedures, and citizenship status were analyzed from 2012-2022. Rates of PNCR transplantation activity were compared with population rates from the US Census Bureau. On average, 713 ± 47 pediatric liver and 1039 ± 51 kidney patients were added to the waitlist, with 544 ± 32 liver and 742 ± 33 kidney transplants performed annually. Of these, PNCR comprised 1.5% and 3.3% of liver and kidney waitlist additions and 1.5% and 2.9% of liver and kidney transplant procedures, respectively. There were no significant changes in waitlist or transplant activity nationwide over the study period. There was a significant geographic variation in the percentage of waitlist additions and transplants across the United Network for Organ Sharing regions among the PNCR for liver and kidney transplantation. This is the first study to evaluate national trends in transplantation activity among PNCRs. The significant regional variation in transplantation activity for PNCR may suggest multilevel structural and systemic barriers to transplant accessibility.

2.
Pediatr Transplant ; 28(1): e14669, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38059422

RESUMO

PURPOSE: Immediate extubation (IE) following liver transplantation (LT) is increasingly common in adult patients. This study reviews our center's experience with IE in children following LT to determine characteristics predictive of successful IE and its effects on post-operative outcomes. METHODS: We performed a retrospective chart review of all patients who underwent LT at our institution between January 2005 and November 2022. Patients with concomitant lung transplants and chronic ventilator requirements were excluded. RESULTS: Overall, 235 patients met study criteria. IE was achieved in 164 (69.8%) patients across all diagnoses and graft types. Of IE patients, only two required re-intubation within 3 days post-transplant. IE patients exhibited significantly shorter ICU (2 [1, 3 IQR] vs. 4 [2, 4 IQR] days, p < .001) and hospital lengths of stay (17 [12, 24 IQR] vs. 22 [14, 42 IQR] days, p = .001). Pre-transplant ICU requirement, high PELD/MELD score, intraoperative transfusion, cold ischemia time, and pressor requirements were risk factors against IE. There was no association between IE and recipient age or weight. The proportion of patients undergoing IE post-transplant increased significantly over time from 2005 to 2022 (p < .001), underscoring the role of clinical experience and transplant team learning curve. CONCLUSION: Spanning 18 years and 235 patients, we report the largest cohort of children undergoing IE following LT. Our findings support that IE is safe across ages and clinical scenarios. As our center gained experience, the rate of IE increased from 40% to 83%. These trends were associated with lower ICU and LOS, the benefits of which include earlier patient mobility and improved resource utilization.


Assuntos
Transplante de Fígado , Criança , Humanos , Extubação , Tempo de Internação , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Tempo
3.
Pediatr Transplant ; 28(1): e14693, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38317339

RESUMO

BACKGROUND: Pulmonary calcification (PC) is a rare clinical entity observed following liver transplantation (LT). Most often identified in adults or in patients with concomitant renal failure, PC is rarely reported in children. While the clinical course of PC is largely benign, cases of progressive respiratory failure and death have been reported. Additionally, PC may mimic several other disease processes making diagnosis and management challenging. Currently, little is reported regarding the diagnosis, management, and long-term outcomes of children with PC following LT. METHODS: We performed a retrospective chart review of patients undergoing LT at our institution between 2006 and 2023. We identified two patients who developed PC following LT. Their diagnosis, clinical course, and long-term outcomes are reported. A literature review of the presentation, diagnosis, management, and outcomes of adult and pediatric patients with PC post-LT was also performed. CONCLUSIONS: Pulmonary calcifications are a rare but notable complication after pediatric liver transplantation. Our case series adds to the limited literature on this clinical entity in children but also highlights the fact that effective diagnosis and treatment may be safely accomplished without the use of lung biopsy.


Assuntos
Transplante de Fígado , Pneumopatias , Insuficiência Respiratória , Adulto , Criança , Humanos , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos , Pneumopatias/etiologia , Pneumopatias/cirurgia , Progressão da Doença
4.
Pediatr Transplant ; 28(5): e14818, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38940480

RESUMO

INTRODUCTION: Allograft dysfunction within the first week posttransplant is an uncommon but known complication following liver transplantation. Seventh-Day Syndrome (7DS) is a rare complication of allograft dysfunction following liver transplantation characterized by the rapid clinical deterioration of a formerly well-functioning allograft within the first week posttransplant. The etiology of 7DS is unknown, and treatment options remain limited. While cases of graft survival have been reported, the risk of mortality remains exceedingly high without urgent retransplantation. METHODS: Patient data was retrospectively analyzed and a literature review performed. RESULTS: We present a unique case of split liver transplantation into two pediatric recipients in which one recipient developed rapidly progressive graft failure approximately 1 week postoperatively requiring urgent retransplantation while the other recipient had an unremarkable postoperative course. Upon clinical manifestation of progressive graft failure, the patient was treated with thymoglobulin, rituximab, intravenous immunoglobulin, and plasmapheresis. Despite this, the patient's clinical status continued to decline and she underwent retransplantation 11 days following her initial liver transplant. CONCLUSION: Seventh-Day Syndrome is a rare complication following liver transplantation that is associated with a high risk of morbidity and mortality. Our case adds to the limited literature on 7DS in children and is the first to report a comparative posttransplant clinical course in two recipients who received split grafts from the same donor.


Assuntos
Transplante de Fígado , Complicações Pós-Operatórias , Reoperação , Humanos , Transplante de Fígado/efeitos adversos , Feminino , Síndrome , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Estudos Retrospectivos , Rejeição de Enxerto/etiologia , Criança , Pré-Escolar , Sobrevivência de Enxerto , Disfunção Primária do Enxerto/etiologia , Disfunção Primária do Enxerto/diagnóstico , Lactente
5.
Ann Surg ; 276(6): e1107-e1113, 2022 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-34183509

RESUMO

OBJECTIVE: This study aims to quantify the number of patent-holding surgeons and determine their specialty demographics. SUMMARY BACKGROUND DATA: The number of intellectual property filings related to surgery has exponentially increased over the past 40 years, yet surgeon inventor status among these inventions remains poorly defined. METHODS: A query of the United States Patent and Trademark Office (USPTO) Patent Full-Text and Image Database was performed over the years 1993 to 2018. Patents related to surgery were defined as surgical devices, implantables, dressings, introducers, and sterilization equipment based on Cooperative Patent Classification (CPC) code. Inventor names were cross-indexed with names of active Fellows in the American College of Surgeons (FACS) as of 2019. Surgeon inventors were identified and differences between specialty and sex were evaluated. RESULTS: A total of 275,260 patents related to surgery were issued over the study period. The number of surgical patents has increased by 462% from 4593 per year to 21,241 per year. A total of 9008 patents were held by a total of 2164 surgeons (4% of FACS). This represents 3.3% of all surgical patents with a mean of 5 patents (range 1-346) per patent-holding surgeon. Specialties with the largest number of patent holders include neurosurgery (9%) and orthopedic surgery (8%). Ninety-seven percent of patent-holding surgeons were male. CONCLUSIONS: 3.3% of patents related to surgery involve a surgeon inventor, and although the number of surgical patents has shown an exponential increase, surgeon involvement in these inventions has grown minimally. Surgical innovation training may offer an opportunity to reduce these discrepancies and increase surgeon involvement as patent holders.


Assuntos
Neurocirurgia , Cirurgiões , Masculino , Humanos , Estados Unidos , Feminino , Invenções , Procedimentos Neurocirúrgicos , Criatividade
6.
BMC Pediatr ; 22(1): 52, 2022 01 20.
Artigo em Inglês | MEDLINE | ID: mdl-35057772

RESUMO

BACKGROUND: Triple gallbladder is a rare congenital anomaly of the biliary tract that can be associated with heterotopic tissue. Gallbladder triplication results from the failure of rudimentary bile ducts to regress during embryological development, and can be difficult to distinguish from Todani type II choledochal cysts and biliary duplication cysts. CASE PRESENTATION: A 2-year-old patient presented to our institution with intermittent abdominal pain for 1 year. She had elevated transaminases with imaging concerning for a choledochal cyst. After assessment with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography, she was diagnosed with a gallbladder multiplication and a common bile duct stricture. She underwent laparoscopic cholecystectomy, which confirmed the diagnosis of triple gallbladder. One of the three gallbladders demonstrated heterotopic gastric mucosa on final pathology, including at the cystic duct margin. Follow up testing with a technetium 99 m scan demonstrated a subtle focus of increased activity in the right upper abdomen at the expected location of the common bile duct, concerning for the presence of residual gastric mucosa. The patient remains well without abdominal pain. CONCLUSIONS: We describe the first case of heterotopic gastric mucosa in a triple gallbladder in a young patient presenting with chronic abdominal pain. We also demonstrate the safety and feasibility of laparoscopic cholecystectomy in young children with triple gallbladder. Finally, we propose an interdisciplinary approach to the management of common bile duct strictures in the setting of ectopic acid secretion, involving a combination of medical management, endoscopic intervention, and possible salvage laparoscopic Roux-en-Y hepaticojejunostomy.


Assuntos
Cisto do Colédoco , Vesícula Biliar , Abdome/patologia , Criança , Pré-Escolar , Colangiopancreatografia Retrógrada Endoscópica , Cisto do Colédoco/complicações , Feminino , Vesícula Biliar/diagnóstico por imagem , Vesícula Biliar/cirurgia , Mucosa Gástrica/patologia , Humanos
7.
J Vasc Surg ; 74(1): 79-89.e2, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33340698

RESUMO

OBJECTIVE: To evaluate the outcomes of various surgical approaches in the treatment of renovascular hypertension and midaortic syndrome (MAS) in children. METHODS: We performed a retrospective medical record review of patients who had undergone surgery for renovascular hypertension from 2010 to 2018 at our center under the care of a multidisciplinary team. The operative interventions included mesenteric artery growth improves circulation (MAGIC), tissue expander-stimulated lengthening of arteries (TESLA), aortic bypass using polytetrafluorethylene, renal artery reimplantation, and autotransplantation. The MAGIC procedure uses the meandering mesenteric artery as a free conduit for aortic bypass. The TESLA procedure is based on lengthening the normal distal aorta and iliac arteries by gradual filling of a retroaortic tissue expander for several weeks, followed by resection of the stenotic aorta and subsequent primary reconstruction. RESULTS: A total of 39 patients were identified, 10 with isolated renal artery stenosis, 26 with MAS, and 3 with systemic inflammatory vasculitis. The median age at presentation and surgery was 6.4 years (range, 0-16.3 years) and 9.3 years (range, 0-9.2 years), respectively. The MAS-associated syndromes included neurofibromatosis type 1 (15.4%) and Williams syndrome (5.1%), although most cases were idiopathic. At surgery, 33.3% had had stage 1 hypertension (HTN), 53.8% stage 2 HTN, and 12.8% normal blood pressure with a median of three antihypertensive medications. Follow-up of 37 patients at a median of 2.5 years demonstrated normal blood pressure in 86.1%, stage 1 HTN in 8.3%, and stage 2 HTN in 5.6%, with a median of one antihypertensive medication for the entire cohort. CONCLUSIONS: The patterns of vascular involvement leading to renovascular hypertension in children are variable and complex, requiring thoughtful multidisciplinary planning and surgical decision-making. The MAGIC and TESLA procedures provide feasible approaches for aortic bypass and reconstruction using autologous tissues and will result in normalization of blood pressure in 85% of children 2.5 years after surgery.


Assuntos
Aorta/cirurgia , Doenças da Aorta/cirurgia , Arteriopatias Oclusivas/cirurgia , Hipertensão Renovascular/cirurgia , Obstrução da Artéria Renal/cirurgia , Procedimentos Cirúrgicos Vasculares , Adolescente , Fatores Etários , Aorta/diagnóstico por imagem , Aorta/fisiopatologia , Doenças da Aorta/complicações , Doenças da Aorta/diagnóstico por imagem , Doenças da Aorta/fisiopatologia , Arteriopatias Oclusivas/complicações , Arteriopatias Oclusivas/diagnóstico por imagem , Arteriopatias Oclusivas/fisiopatologia , Pressão Sanguínea , Prótese Vascular , Implante de Prótese Vascular/instrumentação , Criança , Pré-Escolar , Feminino , Humanos , Hipertensão Renovascular/diagnóstico , Hipertensão Renovascular/etiologia , Hipertensão Renovascular/fisiopatologia , Artéria Ilíaca/fisiopatologia , Artéria Ilíaca/cirurgia , Lactente , Masculino , Artérias Mesentéricas/crescimento & desenvolvimento , Artérias Mesentéricas/fisiopatologia , Artérias Mesentéricas/transplante , Artéria Renal/fisiopatologia , Artéria Renal/cirurgia , Obstrução da Artéria Renal/diagnóstico por imagem , Obstrução da Artéria Renal/etiologia , Obstrução da Artéria Renal/fisiopatologia , Reimplante , Estudos Retrospectivos , Síndrome , Fatores de Tempo , Expansão de Tecido/instrumentação , Dispositivos para Expansão de Tecidos , Transplante Autólogo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos , Procedimentos Cirúrgicos Vasculares/instrumentação
8.
Pediatr Transplant ; 25(8): e14117, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34414641

RESUMO

BACKGROUND: While reviewing outcomes metrics and data from the SRTR, it became apparent that prospective assessment of the SRTR reporting cohorts would be an important proactive strategy for internal quality control. It was particularly important to identify the number of patient deaths and graft failures within 1 year of transplant that would result in being flagged by the UNOS and the MPSC. METHODS: A simple Microsoft Excel line graph was created to visually display retrospective, current, and future SRTR cohorts. Data provided by the SRTR CUSUM (https://securesrtr.transplant.hrsa.gov/srtr-reports/cusum-charts/) Reports and the SRTR 1 Year Expected Survival Excel Worksheet (https://securesrtr.transplant.hrsa.gov/srtr-reports/current-release/) were leveraged to identify whether programs were in jeopardy of being flagged by UNOS/MPSC for outcomes. RESULTS & CONCLUSIONS: The creation of this visual tool has greatly improved team understanding of SRTR report cohorts, as well as the risk of being flagged by regulatory agencies, for adverse outcomes.


Assuntos
Transplante de Órgãos/mortalidade , Avaliação de Resultados em Cuidados de Saúde/métodos , Melhoria de Qualidade , Sistema de Registros , Boston , Criança , Humanos , Estados Unidos
9.
Pediatr Transplant ; 24(2): e13623, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31828929

RESUMO

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.


Assuntos
Peso Corporal , Seleção do Doador/métodos , Artéria Hepática , Transplante de Fígado/métodos , Complicações Pós-Operatórias/etiologia , Trombose/etiologia , Doadores de Tecidos , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Modelos Logísticos , Masculino , Análise Multivariada , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Trombose/epidemiologia
10.
Pediatr Transplant ; 24(6): e13736, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32432836

RESUMO

TE measures liver stiffness to assess fibrosis. Its use in post-transplant patients was reported in few small pediatric studies. We evaluated TE ability to predict liver graft fibrosis in a large cohort while comparing it to the performance of APRI and FIB-4. We also investigated the effect of graft type on LSMs. Patients at Boston Children's Hospital who underwent LT and LSM ≤ 1 year from biopsy (2007-2018) were eligible. Ninety-four patients (45%M) aged 1-21 years (89% < 18 years; 13% < 2 years) were eligible. Median time between transplant/biopsy and LSM was 5.1 years and 52 days, respectively. Thirty-nine percent received whole-liver grafts, 54% TV grafts, and 6% as part of MV. At LSM, median ALT was 25 [IQR 16-33] IU/L. Twenty-one percent had METAVIR ≥ F2. LSM was statistically higher among those with significant fibrosis (METAVIR ≥ F2) compared to those with METAVIR F0/F1 (median [IQR] 7.5 [4.6, 13.6] vs 5.1 [4.0, 6.4] kPa, respectively) (P = .005 by Wilcoxon rank-sum test). APRI and FIB-4 distributions were not different across METAVIR stages. The AUROC for LSM was 0.71 (95% CI 0.56-0.85) with an optimal cut-point of 6.5 kPa. Graft type had no influence on the AUROC for LSM. TE is useful for assessing significant graft fibrosis in children and young adult LT recipients and performs better than APRI and FIB-4. TV grafts demonstrate similar correlation with histology as whole-liver grafts.


Assuntos
Técnicas de Imagem por Elasticidade/métodos , Transplante de Fígado/métodos , Pediatria/métodos , Transplantados , Aloenxertos , Biópsia , Boston , Pré-Escolar , Feminino , Fibrose , Sobrevivência de Enxerto , Hospitais Pediátricos , Humanos , Lactente , Inflamação , Fígado/fisiopatologia , Cirrose Hepática/patologia , Masculino , Pressão , Curva ROC , Estudos Retrospectivos , Transplante Homólogo , Resultado do Tratamento
11.
J Am Soc Nephrol ; 30(2): 201-215, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30655312

RESUMO

BACKGROUND: Whole-exome sequencing (WES) finds a CKD-related mutation in approximately 20% of patients presenting with CKD before 25 years of age. Although provision of a molecular diagnosis could have important implications for clinical management, evidence is lacking on the diagnostic yield and clinical utility of WES for pediatric renal transplant recipients. METHODS: To determine the diagnostic yield of WES in pediatric kidney transplant recipients, we recruited 104 patients who had received a transplant at Boston Children's Hospital from 2007 through 2017, performed WES, and analyzed results for likely deleterious variants in approximately 400 genes known to cause CKD. RESULTS: By WES, we identified a genetic cause of CKD in 34 out of 104 (32.7%) transplant recipients. The likelihood of detecting a molecular genetic diagnosis was highest for patients with urinary stone disease (three out of three individuals), followed by renal cystic ciliopathies (seven out of nine individuals), steroid-resistant nephrotic syndrome (nine out of 21 individuals), congenital anomalies of the kidney and urinary tract (ten out of 55 individuals), and chronic glomerulonephritis (one out of seven individuals). WES also yielded a molecular diagnosis for four out of nine individuals with ESRD of unknown etiology. The WES-related molecular genetic diagnosis had implications for clinical care for five patients. CONCLUSIONS: Nearly one third of pediatric renal transplant recipients had a genetic cause of their kidney disease identified by WES. Knowledge of this genetic information can help guide management of both transplant patients and potential living related donors.


Assuntos
Sequenciamento do Exoma/métodos , Transplante de Rim/métodos , Medicina de Precisão/métodos , Insuficiência Renal Crônica/genética , Insuficiência Renal Crônica/cirurgia , Adolescente , Boston , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Predisposição Genética para Doença/epidemiologia , Testes Genéticos/métodos , Rejeição de Enxerto , Sobrevivência de Enxerto , Hospitais Pediátricos , Humanos , Transplante de Rim/efeitos adversos , Masculino , Prognóstico , Insuficiência Renal Crônica/fisiopatologia , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Análise de Sobrevida , Transplantados/estatística & dados numéricos , Resultado do Tratamento
12.
Pediatr Transplant ; 23(7): e13553, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31328864

RESUMO

Kidney transplantation remains the treatment of choice for children with ESRD. Optimal perioperative management is critical in small recipients of ASK to assure adequate graft perfusion. We present a single-center experience outlining management for patients weighing <20 kg who underwent primary renal transplantation with ASKs between 2007 and 2016. Sixty-three patients met study criteria and underwent 34 living-related, six living-unrelated, and 23 deceased donor kidney transplants. Median age and weight at transplant were 25 months (IQR 18-37 months; range 11 months-6 years) and 11.0 kg (IQR 9.2-14.5 kg; range 7.1-19.5 kg). Eighty-nine percent of patients required vasoactive agents intra-operatively, with twenty patients requiring prolonged vasoactive agents post-operatively. Intra-operatively, patients received 51.9 mL/kg of crystalloid, 27.3 mL/kg of 5% albumin, and 13.6 mL/kg of packed red blood cells. Most (93.7%) patients were extubated on POD#0. Weights peaked on post-operative days three through five. Over a median follow-up of 49 months (IQR 31-86 months; range 0-130 months), four grafts were lost, two due to thrombosis and two secondary to chronic rejection. There was one patient death six months post-transplant due to causes unrelated to transplantation. Graft survival at 1, 5, and 10 years was 98.4%, 96.6%, and 84.2%, respectively. Of surviving allografts, the median 1, 5, and 10 years post-transplant eGFR was 122.9, 90.0, and 59.2 mL/min/1.73 m2 as determined by the 2009 Schwartz formula. Renal transplantation in small children using ASKs requires meticulous perioperative management including adequate fluid resuscitation and judicious use of pressors to assure adequate graft perfusion. The use of ASKs from living or deceased donors results in satisfactory short and long-term outcomes.


Assuntos
Falência Renal Crônica/cirurgia , Transplante de Rim , Doadores Vivos , Doadores de Tecidos , Adulto , Albuminas/uso terapêutico , Criança , Pré-Escolar , Soluções Cristaloides/uso terapêutico , Transfusão de Eritrócitos , Feminino , Sobrevivência de Enxerto , Humanos , Imunossupressores/uso terapêutico , Lactente , Estimativa de Kaplan-Meier , Rim/cirurgia , Falência Renal Crônica/mortalidade , Masculino , Nefrectomia , Tamanho do Órgão , Período Perioperatório , Estudos Retrospectivos , Resultado do Tratamento , Vasoconstritores/uso terapêutico , Adulto Jovem
13.
Pediatr Transplant ; 23(7): e13568, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31515909

RESUMO

End-organ disease caused by CMV is a significant cause of morbidity and mortality in pediatric SOT recipients. Pediatric transplant centers have adopted various approaches for CMV disease prevention in this patient population. We observed significant practice variation in CMV testing, prophylaxis, and surveillance across SOT groups in our center. To address this, we implemented evidence-based standardized protocols and measured outcomes pre- and post-implementation of these protocols. We performed retrospective chart review for SOT recipients from 2009 to 2014 at Boston Children's Hospital. Using descriptive statistics, we measured practice improvement in provision of appropriate prophylaxis, occurrence of neutropenia and associated complications, and occurrence of CMV DNAemia and CMV disease pre- and post-intervention. The pre- and post-intervention periods included 141 and 109 patients, respectively. With the exception of kidney transplant recipients, provision of appropriate valganciclovir prophylaxis improved across SOT groups post-intervention (P < .01). Occurrence of >1 episode of neutropenia was greater in the preintervention period (30% vs 10%, P < .001). In both periods, neutropenia was associated with few episodes of invasive infections. The occurrence of CMV disease did not differ and was overall low. However, due to routine surveillance a significantly greater number of asymptomatic CMV DNAemia episodes were identified and treated in the post-intervention period. Implementation of standardized prevention protocols helped to improve the provision of appropriate prophylaxis to patients at risk for CMV acquisition, increased the diagnosis and treatment of asymptomatic CMV DNAemia, and decreased episodes of recurrent neutropenia in patients receiving prophylaxis.


Assuntos
Infecções por Citomegalovirus/prevenção & controle , Transplante de Órgãos/normas , Adolescente , Alemtuzumab/uso terapêutico , Antivirais/uso terapêutico , Basiliximab/uso terapêutico , Boston , Criança , Pré-Escolar , Citomegalovirus , Infecções por Citomegalovirus/complicações , DNA Viral , Daclizumabe/uso terapêutico , Feminino , Humanos , Lactente , Masculino , Transplante de Órgãos/efeitos adversos , Estudos Retrospectivos , Risco , Esteroides/uso terapêutico , Transplantados , Valganciclovir/uso terapêutico
14.
J Vasc Surg ; 67(6): 1664-1672, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29342430

RESUMO

BACKGROUND: Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta. Patients with disease refractory to medical management will usually require either endovascular therapy or surgery with use of prosthetic graft material for bypass or patch angioplasty. We report our early experience with a novel approach using a tissue expander (TE) to lengthen the normal native arteries in children with MAS, allowing primary aortic repair without the need for prosthetic graft material. METHODS: We conducted a retrospective review of patients with MAS undergoing the TE-stimulated lengthening of arteries (TESLA) procedure at our institution from 2010 to 2014. Data are presented as mean (range). RESULTS: Five patients aged 4.8 years (3-8 years) underwent the TESLA procedure. Stages of this procedure include the following: stage I, insertion of retroaortic TE; stage II, serial TE injections; and stage III, final repair with excision of aortic stenosis and primary end-to-end aortic anastomosis. Stage II was completed in 4 months (1-9 months) with 12 (7-20) TE injections. Goal lengthening was achieved in all patients. Stage III could not be completed in one patient because of extreme aortic inflammation, which precluded safe excision of the aortic stenosis and required use of a prosthetic bypass graft. The other four patients completed stage III with two (one to three) additional vessels also requiring reconstruction (renal or mesenteric arteries). At 3.2 years (1-6 years) of follow-up, all patients are doing well. CONCLUSIONS: The TESLA procedure allows surgical correction of MAS without the need for prosthetic grafts in young children who are still growing.


Assuntos
Aorta Abdominal , Arteriopatias Oclusivas/cirurgia , Procedimentos Endovasculares/métodos , Procedimentos de Cirurgia Plástica/métodos , Dispositivos para Expansão de Tecidos , Anastomose Cirúrgica/métodos , Aortografia , Arteriopatias Oclusivas/diagnóstico , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Desenho de Prótese , Estudos Retrospectivos , Síndrome , Fatores de Tempo , Resultado do Tratamento
15.
Pediatr Transplant ; : e13250, 2018 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-29888545

RESUMO

HB is the most common primary liver tumor in children. Complete tumor excision, either by partial resection or by total hepatectomy and liver transplantation, in combination with chemotherapy provides the best chance for cure. We performed a retrospective analysis of patients who underwent liver transplantation for HB and herein present our 14-year single-institution experience. Twenty-five patients underwent liver transplantation for HB at a median age of 26 months (IQR: 15-44). Graft survival was 96%, 87%, and 80% at 1, 3, and 5 years, respectively. There were four patient deaths, three of them due to disease recurrence within the first year post-transplant. Ten-year overall survival was 84%. Three recipients initially presented with pulmonary metastases and underwent resection of metastatic disease, of which two are alive at 3.9 years. Of three patients who underwent salvage transplants, two are alive at 1.5 years after transplant. Non-survivors were associated with lower median alpha fetoprotein value at presentation compared to survivors (21 707 vs 343 214; P = .04). In conclusion, the overall long-term outcome of primary liver transplantation for HB is excellent. Tumor recurrence was the highest contributor to mortality. Even patients with completely treated pulmonary metastases prior to transplant demonstrated a favorable survival.

16.
Pediatr Nephrol ; 33(5): 881-887, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-29243158

RESUMO

OBJECTIVE: Primary hyperoxaluria type-1 (PH-1) is a rare genetic disorder in which normal hepatic metabolism of glyoxylate is disrupted resulting in diffuse oxalate deposition and end-stage renal disease (ESRD). While most centers agree that combined liver-kidney transplant (CLKT) is the appropriate treatment for PH-1, perioperative strategies for minimizing recurrent oxalate-related injury to the transplanted kidney remain unclear. We present our management of children with PH-1 and ESRD on hemodialysis (HD) who underwent CLKT at our institution from 2005 to 2015. METHODS: On chart review, three patients (2 girls, 1 boy) met study criteria. Two patients received deceased-donor split-liver grafts, while one patient received a whole liver graft. All patients underwent bilateral native nephrectomy at transplant to minimize the total body oxalate load. Median preoperative serum oxalate was 72 µmol/L (range 17.8-100). All patients received HD postoperatively until predialysis serum oxalate levels fell <20 µmol/L. All patients, at a median of 7.5 years of follow-up (range 6.5-8.9), demonstrated stable liver and kidney function. CONCLUSIONS: While CLKT remains the definitive treatment for PH-1, bilateral native nephrectomy at the time of transplant reduces postoperative oxalate stores and may mitigate damage to the renal allograft.


Assuntos
Hiperoxalúria Primária/cirurgia , Falência Renal Crônica/cirurgia , Transplante de Rim/métodos , Transplante de Fígado/métodos , Nefrectomia/métodos , Oxalatos/sangue , Criança , Pré-Escolar , Feminino , Humanos , Hiperoxalúria Primária/complicações , Lactente , Rim/fisiopatologia , Falência Renal Crônica/etiologia , Masculino , Estudos Retrospectivos , Resultado do Tratamento
18.
J Vasc Interv Radiol ; 28(8): 1184-1188, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28735935

RESUMO

A 5-year-old boy with midaortic syndrome who had undergone aortic bypass and bilateral renal artery grafts presented to the emergency department 1 year after surgery with symptoms of nausea, vomiting, and abdominal pain. Because of delay in diagnosis of bilateral renal artery thrombosis, his condition progressed to anuric renal failure. He underwent catheter-directed thrombolysis 7 days after presentation with administration of tissue plasminogen activator and heparin infusion over a 24-hour period. There was successful resolution of thrombus and complete recovery of renal function to baseline. The patient had normal renal function at 6-month follow-up.


Assuntos
Anticoagulantes/uso terapêutico , Fibrinolíticos/uso terapêutico , Heparina/uso terapêutico , Artéria Renal/cirurgia , Terapia Trombolítica/métodos , Trombose/tratamento farmacológico , Ativador de Plasminogênio Tecidual/uso terapêutico , Pré-Escolar , Humanos , Testes de Função Renal , Masculino , Trombose/etiologia
19.
Pediatr Transplant ; 21(5)2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28393434

RESUMO

Standard management of intra-abdominal pediatric solid tumors requires complete resection. However, tumors with multiple organ and vascular involvement present a unique surgical challenge. We conducted a retrospective chart review of four patients, aged 2-14 years, undergoing MVT for intra-abdominal tumors with significant involvement of the visceral arteries and/or portomesenteric venous system at our institution. Indications for MVT included hepatocellular carcinoma, inflammatory myofibroblastic tumor, and two cases of hepatoblastoma. Grafts included liver, stomach, small bowel, and pancreas in all patients, with two patients also receiving spleens, and one, a partial esophageal transplant. Median hospital stay was 80 days. Postoperative complications included reoperation for abdominal hematoma and bowel obstruction, steroid responsive intestinal rejection, wound dehiscence, fungemia, seizures, and chyle leak with pleural effusion. One patient developed Epstein-Barr virus-associated complications which responded well to treatment. On follow-up (range 2.8-7.8 years), all patients have satisfactory graft function and no evidence of recurrent disease. MVT is an effective means of achieving complete gross resection of intra-abdominal malignancies in patients with multiple organ and vascular involvement.


Assuntos
Neoplasias Abdominais/cirurgia , Carcinoma Hepatocelular/cirurgia , Hepatoblastoma/cirurgia , Neoplasias Hepáticas/cirurgia , Neoplasias de Tecido Muscular/cirurgia , Transplante de Órgãos/métodos , Neoplasias Abdominais/patologia , Adolescente , Carcinoma Hepatocelular/patologia , Criança , Pré-Escolar , Feminino , Hepatoblastoma/patologia , Humanos , Lactente , Neoplasias Hepáticas/patologia , Masculino , Invasividade Neoplásica , Neoplasias de Tecido Muscular/patologia
20.
Paediatr Anaesth ; 27(7): 718-725, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28557286

RESUMO

BACKGROUND: Liver transplantation represents a major surgery involving a highly vascular organ. Reports defining the scope of bleeding in pediatric liver transplants are few. AIMS: We conducted a retrospective analysis of liver transplants performed at our pediatric tertiary care center to quantify blood loss, blood product utilization, and to determine predictors for massive intraoperative bleeding. METHODS: Pediatric patients who underwent isolated liver transplantation at Boston Children's Hospital between 2011 and 2016 were included. The amount of blood product transfused in the perioperative period and the incidence of postoperative complications were reported. Univariable and multivariable logistic regressions were used to determine predictors for massive bleeding, defined as estimated blood loss exceeding one circulating blood volume within 24 hours. RESULTS: Sixty-eight children underwent liver transplantation during the study period and were included in the analysis. Multivariable logistic regression analysis identified the following independent predictors of massive bleeding: preoperative hemoglobin level <8.5 g/dL (OR 11.09, 95% CI 1.87-65.76), INR >1.5 (OR 11.62, 95% CI 2.36-57.26), platelet count <100 109 /L (OR 7.92, 95% CI 1.46-43.05), and surgery duration >600 minutes (OR 6.97, 95% CI 0.99-48.92). CONCLUSIONS: Pediatric liver transplantation is associated with substantial blood loss and a significant blood product transfusion burden. A 43% incidence of massive bleeding is reported. Further efforts are needed to improve bleeding management in this high-risk population.


Assuntos
Transplante de Fígado/efeitos adversos , Hemorragia Pós-Operatória/epidemiologia , Perda Sanguínea Cirúrgica , Transfusão de Sangue/estatística & dados numéricos , Criança , Pré-Escolar , Feminino , Hemoglobinas/análise , Humanos , Incidência , Lactente , Coeficiente Internacional Normatizado , Masculino , Contagem de Plaquetas , Complicações Pós-Operatórias/epidemiologia , Valor Preditivo dos Testes , Estudos Retrospectivos , Centros de Atenção Terciária , Tromboelastografia
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