RESUMEN
Postreperfusion syndrome is one of the responsible mechanisms of portal hypertension in patients undergoing liver transplantation. And post-transplant portal hypertension causes graft dysfunction. Postreperfusion syndrome is characterized by a decrease in arterial pressure and cardiac output, and an increase in central venous pressure, pulmonary artery pressure, and pulmonary vascular resistance that occurs after the release of the portal vein clamp. Although early recovery from postreperfusion syndrome is desired, there is a little medication therapy such as the administration of calcium chloride, sodium bicarbonate, and beta-agonist for postreperfusion syndrome. We present a case of postreperfusion syndrome manifested as post-transplant portal hypertension and reversed after nitroglycerin administration. A 49-year-old Asian woman was scheduled for liver transplantation because of Budd-Chiari syndrome. After portal vein reperfusion, she experienced severe postreperfusion syndrome. Administration of ephedrine and calcium restored arterial pressure; however, pulmonary artery pressure, pulmonary vascular resistance, and central venous pressure elevations were sustained, causing right ventricular overload. This condition did not improve after hepatic artery reperfusion, and caused post-transplant portal hypertension. After nitroglycerin administration, pulmonary vascular resistance and central venous pressure decreased, mean arterial pressure increased, right heart contractility recovered, and portal hypertension disappeared. Hemodynamic improvement by nitroglycerin administration helped in diagnosing postreperfusion syndrome and avoiding unnecessary splenectomy. If portal vein pressure increases after liver transplantation, the change in hemodynamic parameters by nitroglycerin administration should be assessed, which will lead to accurate diagnosis and appropriate treatment. Furthermore, postreperfusion syndrome should be listed as a differential diagnosis of post-transplant portal hypertension.
Asunto(s)
Síndrome de Budd-Chiari , Hipertensión Portal , Femenino , Humanos , Persona de Mediana Edad , Nitroglicerina/uso terapéutico , Síndrome de Budd-Chiari/etiología , Síndrome de Budd-Chiari/tratamiento farmacológico , Hemodinámica , Resistencia Vascular , Hipertensión Portal/tratamiento farmacológicoRESUMEN
Biliary atresia is an obliterative cholangiopathy of unknown etiology. Hepatic portoenterostomy, in which obliterated extrahepatic bile ducts are resected and bile flow is restored, known as Kasai operation, is performed within 3 months after birth. While this operation enhances long-term survival of patients, the occurrence of primary malignant hepatic tumors has been increasing. We report a case of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation. A 49-year-old man, who underwent Kasai operation for biliary atresia when he was 2 months old, experienced rapidly progressive jaundice and liver dysfunction. Deceased-donor liver transplantation was performed for liver failure. Macroscopically, there was a white-yellow tumor located at the anastomotic site of hepatic portoenterostomy of the resected liver. Pathological examination revealed a well-differentiated adenocarcinoma with some Paneth cells in the neoplastic lesion. Immunohistochemically, the tumor cells were negative for cytokeratin 7 (CK7) but positive for cytokeratin 20 (CK20) and a homeobox domain-containing transcription factor (CDX2). Mucin expression in tumor cells was negative for mucin 1 (MUC1) and mucin 6 (MUC6) and positive for mucin 2 (MUC2) and mucin 5AC (MUC5AC). The pathological diagnosis was small intestinal adenocarcinoma originating from the jejunum. The patient was discharged 48 days after the operation. The patient had not experienced recurrence at 10 months after the operation. This is the first report of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation for biliary atresia. Special care should be taken for the patients after Kasai operation with acute progressive jaundice and liver dysfunction because there is a possibility of malignancy in their native liver.
Asunto(s)
Adenocarcinoma , Atresia Biliar , Neoplasias Intestinales , Humanos , Lactante , Masculino , Persona de Mediana Edad , Adenocarcinoma/diagnóstico , Atresia Biliar/cirugía , Ictericia , Hepatopatías , Trasplante de Hígado , Resultado del Tratamiento , Neoplasias Intestinales/diagnósticoRESUMEN
Tumor-to-tumor metastasis is a rare phenomenon in which primary tumor cells metastasize to other tumors. Herein, we report an extremely rare case of tumor-to-tumor metastasis of medullary thyroid carcinoma to a paraganglioma in a patient with multiple endocrine neoplasia type 2B. Based on genetic examination, a 36-year-old woman was diagnosed with multiple endocrine neoplasia type 2B when she was 24 years old. She had a history of total thyroidectomy for medullary thyroid carcinoma and bilateral adrenalectomy for pheochromocytomas, which were performed when she was 15 years and 29 years old, respectively. Follow-up computed tomography demonstrated a retroperitoneal tumor of 30 mm in diameter beside the left kidney and a liver tumor of 16 mm in diameter located in segment 6. The retroperitoneal and liver tumors were surgically resected and examined by a pathologist. Histological examination revealed the classic Zellballen pattern in the retroperitoneal tumor, rendering the diagnosis of a paraganglioma recurrence. Inside the tumor, a white nodule positive for carcinoembryonic antigen, weakly positive for calcitonin, and negative for tyrosine hydroxylase, was identified and diagnosed as a metastatic medullary thyroid carcinoma with high malignant potential. The liver lesion was diagnosed as a metastasis of the medullary thyroid carcinoma. This is the first report of tumor-to-tumor metastasis of medullary thyroid carcinoma to paraganglioma in a patient with multiple endocrine neoplasia type 2B twenty years after total thyroidectomy.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Carcinoma Medular , Neoplasia Endocrina Múltiple Tipo 2b , Paraganglioma , Neoplasias Retroperitoneales , Neoplasias de la Tiroides , Femenino , Humanos , Adulto , Adulto Joven , Adolescente , Neoplasia Endocrina Múltiple Tipo 2b/diagnóstico , Neoplasia Endocrina Múltiple Tipo 2b/genética , Neoplasia Endocrina Múltiple Tipo 2b/patología , Carcinoma Medular/diagnóstico por imagen , Carcinoma Medular/cirugía , Neoplasias de la Tiroides/diagnóstico por imagen , Neoplasias de la Tiroides/cirugía , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Neoplasias de las Glándulas Suprarrenales/cirugía , Paraganglioma/diagnóstico por imagen , Paraganglioma/cirugíaRESUMEN
BACKGROUND: Endoscopic and PTB interventions are common nonsurgical interventions for biliary anastomotic strictures that occur after liver transplantation. When these nonsurgical interventions fail, surgical re-anastomosis is considered; however, this is quite invasive and can cause additional injury that may lead to graft loss. We report a case in which conventional nonsurgical interventions failed, but a new method that involve the use of a transseptal needle-a device to create a transseptal left-heart access during cardiac catheter interventions-was successfully used in recanalization of the hepaticojejunal anastomotic obstruction. CASE: A 21-year-old man, who had received living-donor liver transplantation for biliary atresia at the age of 23 months presented with recurrent cholangitis and liver dysfunction due to a biliary anastomotic stricture of the hepaticojejunostomy. Therapeutic interventions for biliary stricture, including the PTB approach, double-balloon enteroscopic approach, and rendezvous approach failed. We then performed needle puncture of the anastomotic obstruction using a transseptal needle and succeeded in recanalizing the complete anastomotic obstruction. To perform the procedures safely, we evaluated the organ and needle positions using biplane fluoroscopy and placed a balloon in the afferent jejunal limb as a target for puncture. The 12 Fr catheter via the biliary route was removed 7 months after the procedure, without using a catheter, there was no recurrent stricture or cholangitis for 26 months. CONCLUSION: Using a transseptal needle to manage hepaticojejunal anastomotic obstruction can reduce the number of patients who need surgical re-anastomosis.
Asunto(s)
Colestasis/terapia , Yeyunostomía/métodos , Trasplante de Hígado , Agujas , Complicaciones Posoperatorias/terapia , Anastomosis Quirúrgica , Atresia Biliar/cirugía , Colangiografía , Colestasis/diagnóstico por imagen , Constricción Patológica/diagnóstico por imagen , Constricción Patológica/terapia , Fluoroscopía , Humanos , Masculino , Complicaciones Posoperatorias/diagnóstico por imagen , Punciones , Radiografía Intervencional , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
Cholesterol granuloma is a benign, tumor-like lesion with an accumulation of cholesterol crystals in the tissue and is a consequence of a chronic inflammatory reaction. It commonly occurs in the middle ear but rarely in the liver. There is only one previous case report of cholesterol granuloma of the liver, which was caused by cholesterol hepatolithiasis. We report a case of cholesterol granuloma of the liver in a patient with no intrahepatic cholesterol stones; it was difficult to rule out malignant liver tumor preoperatively. The patient was a 79-year-old woman in whom a lesion in the liver was detected on abdominal ultrasonography. She was referred to our hospital for detailed examination and treatment. Abdominal contrast-enhanced computed tomography showed a 20 mm lesion with ring enhancement in the lateral segment of the liver during the arterial and delayed phases. Since a malignant tumor could not be ruled out radiologically, laparoscopic lateral segment hepatectomy was performed for definitive diagnosis and treatment. The resection specimen showed a yellowish-white lesion measuring 15 mm in diameter. Pathological examination showed a granulomatous lesion with cholesterol crystals surrounded by foreign body giant cells. The lesion was diagnosed as cholesterol granuloma of the liver. The postoperative course was good, and the patient was discharged on postoperative day 5. She was healthy, and no recurrence of the cholesterol granuloma was detected at the 5-month follow-up. This is the first case report of cholesterol granuloma of the liver mimicking a malignant liver tumor in a patient with no intrahepatic cholesterol stones.
Asunto(s)
Litiasis , Neoplasias Hepáticas , Anciano , Colesterol , Femenino , Granuloma/diagnóstico por imagen , Granuloma/cirugía , Humanos , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/cirugíaRESUMEN
The human gut harbors a complex microbial community that performs a range of metabolic, physiological, and immunological functions. The host and its inhabiting microorganisms are often referred to as a "superorganism." Dysbiosis of gut microflora has been associated with the pathogenesis of intestinal disorders including inflammatory bowel disease, colorectal cancer, and extra-intestinal disorders such as cardiovascular disease. Therefore, gut microbiome interventions are important for the prevention and treatment of diseases. However, ethical, economic, scientific, and time constraints limit the outcome of human intervention or animal studies targeting gut microbiota. We recently developed an in vitro batch fermentation model (the Kobe University Human Intestinal Microbiota Model, KUHIMM) that is capable of hosting a majority of gut microbial species in humans and also detects the metabolites produced by microorganisms in real time. In this mini review, we elucidated the characteristics of the KUHIMM and its applicability in analyzing the effect of diet, drugs, probiotics, and prebiotics on intestinal bacteria. In addition, we introduce as examples its application to disease models, such as ulcerative colitis, in which intestinal bacteria are intricately involved in the process of pathogenesis. We also discuss the potential of the KUHIMM in precision medicine. KEY POINTS: ⢠In vitro gut fermentation model to simulate human colonic microbiota ⢠Screening of potential prebiotics and probiotic candidates in healthy model ⢠Construction of disease models of ulcerative colitis and coronary artery disease.
Asunto(s)
Microbioma Gastrointestinal , Probióticos , Animales , Disbiosis , Humanos , Prebióticos , UniversidadesRESUMEN
The aim of this study was to clarify the effect of the spore-forming and lactic acid-producing probiotic strain, Bacillus coagulans SANK 70258, on human colonic microbiota of healthy subjects and ulcerative colitis patients. A model culture system was employed to construct the in vitro human colonic microbiota, to retain the bacterial species richness and simulate the patient's disordered composition, from the fecal inoculum. Bacterial 16S rRNA gene sequencing confirmed that administration of B. coagulans SANK 70258 (at an initial concentration of 4 × 107-total cells/mL) suppressed bacteria related to the family Enterobacteriaceae in the microbiota models for both healthy subjects (P = 0.016) and ulcerative colitis patients (P = 0.023). In addition, administration of B. coagulans SANK 70258 increased bacteria related to the family Lachnospiraceae (P = 0.031), thereby enhancing butyrate production (P = 0.031) in the microbiota models of healthy subjects. However, these changes were not observed in the microbiota models of ulcerative colitis patients, likely owing to the low abundance of Lachnospiraceae species. This study demonstrates the potential of B. coagulans SANK 70258 to exhibit antimicrobial activity against harmful organisms in patients with ulcerative colitis, while improving the intestinal microenvironment by increasing butyrogenesis in healthy persons. KEY POINTS: ⢠B. coagulans SANK 70258 treatment reduced colonic Enterobacteriaceae species. ⢠B. coagulans SANK 70258 treatment enhanced butyrogenesis in healthy individuals. ⢠B. coagulans SANK 70258 treatment increased Lachnospiraceae in healthy persons. ⢠B. coagulans SANK 70258 improves the colonic microenvironment in ulcerative colitis.
Asunto(s)
Bacillus coagulans/genética , Butiratos/metabolismo , Colitis Ulcerosa/microbiología , Enterobacteriaceae/patogenicidad , Microbioma Gastrointestinal , Probióticos/uso terapéutico , Adulto , Anciano , Bacillus coagulans/metabolismo , Colitis Ulcerosa/terapia , Heces/microbiología , Femenino , Voluntarios Sanos , Humanos , Masculino , Persona de Mediana EdadRESUMEN
1,2,4-Butanetriol can be used to produce energetic plasticizer as well as several pharmaceutical compounds. Although Saccharomyces cerevisiae has some attractive characters such as high robustness for industrial production of useful chemicals by fermentation, 1,2,4-butanetriol production by S. cerevisiae has not been reported. 1,2,4-butanteriotl is produced by an oxidative xylose metabolic pathway completely different from the xylose reductase-xylitol dehydrogenase and the xylose isomerase pathways conventionally used for xylose assimilation in S. cerevisiae. In the present study, S. cerevisiae was engineered to produce 1,2,4-butanetriol by overexpression of xylose dehydrogenase (XylB), xylonate dehydratase (XylD), and 2-ketoacid decarboxylase. Further improvement of the recombinant strain was performed by the screening of optimal 2-ketoacid decarboxylase suitable for 1,2,4-butanetriol production and the enhancement of Fe uptake ability to improve the XylD enzymatic activity. Eventually, 1.7â¯g/L of 1,2,4-butanetriol was produced from 10â¯g/L xylose with a molar yield of 24.5%. Furthermore, 1.1â¯g/L of 1,2,4-butanetriol was successfully produced by direct fermentation of rice straw hydrolysate.
Asunto(s)
Butanoles/metabolismo , Hierro/metabolismo , Ingeniería Metabólica , Microorganismos Modificados Genéticamente , Saccharomyces cerevisiae , Xilosa/metabolismo , Microorganismos Modificados Genéticamente/genética , Microorganismos Modificados Genéticamente/metabolismo , Saccharomyces cerevisiae/genética , Saccharomyces cerevisiae/metabolismoRESUMEN
Desensitization with RTX has been broadly introduced in adult LT across the ABO blood type barrier. For pediatric LT, the prophylactic use of RTX has not been standardized, especially for children under 2 years of age. A 20-month-old girl with BA underwent living donor LT from her ABO-I mother. On POD 6, she developed combined T cell-mediated and AMRs. Steroid bolus injection was immediately introduced, followed by antibody-depleting therapy with PE and IVIG. Based on a peripheral blood lymphocyte analysis by fluorescence-activated cell sorting, ATG and RTX were introduced for refractory rejection. Although she recovered from the combined rejections, IHBCs were inevitable as a consequence. We recommend extending the desensitization protocol to cover children under 2 years of age in order to prevent life-threatening complications.
Asunto(s)
Sistema del Grupo Sanguíneo ABO/inmunología , Atresia Biliar/cirugía , Incompatibilidad de Grupos Sanguíneos , Rechazo de Injerto/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Trasplante de Hígado , Rituximab/uso terapéutico , Desensibilización Inmunológica , Femenino , Rechazo de Injerto/inmunología , Humanos , Lactante , Donadores VivosRESUMEN
Reducing graft thickness is essential to prevent large-for-size graft problems in pediatric living donor liver transplantation (LDLT). However, long-term outcomes of LDLT using reduced-thickness left lateral segment (LLS) grafts are unclear. In 89 patients who underwent LDLT using reduced LLS grafts between 2005 and 2017, short-term and long-term outcomes were compared between a nonanatomically reduced LLS (NAR-LLS) graft group and a reduced-thickness LLS graft group. Estimated blood loss was lower and abdominal skin closure was less needed in the recipient operation in the reduced-thickness LLS graft group. Postoperatively, portal vein (PV) flow was significantly decreased in the NAR-LLS graft group, and there was shorter intensive care unit (ICU) stay and fewer postoperative complications, especially bacteremia, in the reduced-thickness LLS graft group. Graft survival at 1 and 3 years after LDLT using reduced-thickness LLS grafts was 95.2% and 92.4%, respectively, which was significantly better than for NAR-LLS grafts. Multivariate analysis revealed that fulminant liver failure, hepatofugal PV flow before LDLT, and NAR-LLS graft were associated with poor graft survival. In conclusion, LDLT using reduced-thickness LLS grafts is a safe and feasible option with better short- and long-term outcomes in comparison with NAR-LLS grafts.
Asunto(s)
Rechazo de Injerto/mortalidad , Hepatectomía/métodos , Arteria Hepática/cirugía , Trasplante de Hígado/mortalidad , Donadores Vivos/provisión & distribución , Complicaciones Posoperatorias , Recolección de Tejidos y Órganos/métodos , Adolescente , Adulto , Niño , Preescolar , Femenino , Estudios de Seguimiento , Rechazo de Injerto/etiología , Supervivencia de Injerto , Humanos , Lactante , Trasplante de Hígado/efectos adversos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia , Adulto JovenRESUMEN
BACKGROUND: Excellent outcomes of the extreme procedure of liver resection (LR) for advanced hepatoblastoma (HB) have been achieved in recent reports. However, liver transplantation (LT) remains the only surgical treatment for patients with unresectable HB. The aim of this study was to evaluate our retrospective data for cases of advanced HB necessitating surgical intervention and analyze the prognostic factors of recurrence by comparing patients with tumors resected by LR and LT. PATIENTS AND METHODS: We retrospectively reviewed 24 children with PRETEXT II/III/IV tumors that required consideration for LT between August 2011 and September 2016. RESULT: The staging at the time of the diagnosis was PRETEXT II/III/IV in 1/13/10 patients, respectively, while the preoperative staging after neoadjuvant chemotherapy was POSTTEXT II/III/IV in 5/17/2 patients. Among those 24 patients, complete resection of the primary tumor was achieved with LT in 12 patients and LR in 12 patients. A high serum level of alpha-fetoprotein (AFP) at the time of surgery, no significant decrease in the rate of change of AFP, and low tumor shrinkage rate were related to the risk of tumor recurrence, and patients with tumors resected by LR with those risks had a higher recurrence rate than those without them. The overall survival was higher in patients with tumors resected by LT (100%) than in patients with tumors resected by LR. CONCLUSION: Patients with advanced HB with a poor response to chemotherapy should definitively be prioritized for primary LT, given the possibility of vascular invasion and microscopic residual tumor.
Asunto(s)
Hepatectomía/métodos , Hepatoblastoma/cirugía , Neoplasias Hepáticas/cirugía , Trasplante de Hígado/métodos , Niño , Preescolar , Femenino , Hepatoblastoma/mortalidad , Hepatoblastoma/patología , Humanos , Lactante , Estimación de Kaplan-Meier , Neoplasias Hepáticas/mortalidad , Neoplasias Hepáticas/patología , Masculino , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/patología , Pronóstico , Estudios Retrospectivos , Resultado del Tratamiento , alfa-Fetoproteínas/análisisRESUMEN
The shortage of deceased organs is still a serious issue in Japan. A proactive approach to using liver grafts from extended criteria donors (ECDs) may be one way of expanding the donor pool; however, if it is recklessly attempted, a recipient receiving such a marginal graft can be at risk of mortality due to primary non-function or delayed graft function. We herein report the successful outcome of a recipient receiving a severely cholestatic graft that was considered transplantable because it lacked features characteristic of a long duration of "cholestasis" according to the precise interpretation of a donor biopsy. Plasma exchange was intentionally introduced to prevent toxic insult by hyperbilirubinemia immediately after transplant. Despite transient acute kidney injury immediately after transplant, the patient's renal impairment was well managed with a renal-sparing immunosuppressive regimen consisting of basiliximab and mycophenolate mofetil. Although the use of liver grafts from ECDs still needs to be discussed, especially regarding graft selection and allocation policies, efforts not to discard valuable grafts should be undertaken in our country.
RESUMEN
PV hypoplasia may increase the risk of posttransplant complications, especially when it extends to near the SMV and SpV junction. We described our experience of 10 pediatric cases of PV hypoplasia/thrombus in which the pullout technique was required for PV reconstruction. There were five male and five female patients. The median age was 9 months, and the median weight was 8.1 kg. The indications for the pullout technique were PV hypoplasia in seven patients and PV thrombus in 3. The inflow sites of the enlarged LGV were as follows: the main PV trunk (n = 2), the SMV and SpV junction (n = 4), and the SpV (n = 4). The posterior face of the pancreas was tunneled along the PV, and the PV was returned to its original position with or without the use of an interposed vein graft. The pullout technique created a good operative field, which allowed for the complete removal of the hypoplastic PV or thrombectomy with the safe use of various interposed vein grafts.
Asunto(s)
Hepatopatías/cirugía , Trasplante de Hígado/métodos , Vena Porta/cirugía , Trombectomía/métodos , Anastomosis Quirúrgica , Prótesis Vascular , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Donadores Vivos , Masculino , Tamaño de los Órganos , Páncreas/cirugía , Complicaciones PosoperatoriasRESUMEN
NEC is an idiopathic intestinal mucosal injury that may progress to transmural bowel necrosis without mesenteric ischemia. NEC usually affects 7- to 10-day-old neonates following enteral feeding. A 10-month-old girl with no history of laparotomy underwent LDLT for acute liver failure. After starting enteral feeding on postoperative day 5, she developed abdominal distention. Diffuse PVG and PI were detected by radiologic modalities. Exploratory laparotomy revealed patchy necrosis of the intestine without perforation. The microscopic findings of a resected specimen revealed transmural coagulative necrosis with multiple small thromboses compatible with neonatal NEC features, and eosinophil infiltration was also observed. Subsequently, after the resumption of enteral feeding with cow's milk, she developed severe diarrhea, the symptoms of which were eliminated after the administration of cow's milk was stopped. These clinical and pathological findings support the speculation that NEC might have been induced by a CMA. Food allergies, which can be induced by immunosuppressive agents, should be considered as a potential cause of NEC in the setting of pediatric liver transplantation.
Asunto(s)
Enterocolitis Necrotizante/etiología , Trasplante de Hígado , Hipersensibilidad a la Leche/complicaciones , Complicaciones Posoperatorias/etiología , Enterocolitis Necrotizante/diagnóstico , Femenino , Humanos , Lactante , Donadores Vivos , Hipersensibilidad a la Leche/diagnóstico , Complicaciones Posoperatorias/diagnósticoRESUMEN
HVOO can be a critical complication in pediatric LDLT. The aim of this study was to evaluate a modified triangular technique of hepatic vein reconstruction for preventing HVOO in pediatric LDLT. A total of 298 pediatric LDLTs were performed using a left lateral segment graft by 2 methods for reconstruction of the hepatic vein. In 177 recipients, slit-shaped anastomosis was indicated with partial clamp of the IVC. A total of 121 recipients subjected to the modified triangular anastomosis with total clamp of the IVC. We compared the incidence of hepatic vein anastomotic complications between these 2 methods. Nine of the 177 cases (5.3%) treated with the conventional technique were diagnosed with outflow obstruction. All 9 cases underwent hepatic vein reconstruction with the slit-shaped hepatic vein anastomosis. In contrast, there were no cases of outflow obstruction in the 121 cases treated with the modified triangular anastomosis. The modified triangular technique of hepatic vein reconstruction with total clamping of the IVC was useful for preventing HVOO in pediatric LDLT.
Asunto(s)
Venas Hepáticas/cirugía , Enfermedad Veno-Oclusiva Hepática/prevención & control , Trasplante de Hígado/métodos , Donadores Vivos , Complicaciones Posoperatorias/prevención & control , Procedimientos Quirúrgicos Vasculares/métodos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Enfermedad Veno-Oclusiva Hepática/diagnóstico , Enfermedad Veno-Oclusiva Hepática/etiología , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/diagnóstico , Resultado del TratamientoRESUMEN
Living donor liver transplantation (LDLT) is now an established technique for treating children with end-stage liver disease. Few data exist about liver transplantation (LT) for exclusively young infants, especially infants of <3 months of age. We report our single-center experience with 12 patients in which LDLT was performed during the first 3 months of life and compare the results with those of older infants who underwent LT. All of the patients were treated at the National Center of Child Health and Development, Tokyo, Japan. Between November 2005 to November 2016, 436 children underwent LT. Twelve of these patients underwent LT in the first 3 months of life (median age, 41 days; median weight, 4.0 kg). The indications for transplantation were fulminant hepatic failure (n = 11) and metabolic liver disease (n = 1). All the patients received the left lateral segment (LLS) in situ to mitigate the problem of graft-to-recipient size discrepancy. A reduced LLS graft was used in 11 patients and a segment 2 monosegment graft was used in 1 patient. We compared the results with those of infants who were 4-6 months of age (n = 67) and 7-12 months of age (n = 110) who were treated in the same study period. There were significant differences in the Pediatric End-Stage Liver Disease score and the conversion rate of tacrolimus to cyclosporine in younger infants. Furthermore, the incidence of biliary complications, bloodstream infection, and cytomegalovirus infection tended to be higher, whereas the incidence of acute cellular rejection tended to be lower in younger infants. The overall cumulative 10-year patient and graft survival rates in recipients of <3 months of age were both 90.9%. LDLT during the first 3 months of life appears to be a feasible option with excellent patient and graft survival. Liver Transplantation 23 1051-1057 2017 AASLD.
Asunto(s)
Infecciones por Citomegalovirus/epidemiología , Enfermedad Hepática en Estado Terminal/cirugía , Rechazo de Injerto/epidemiología , Supervivencia de Injerto , Inmunosupresores/uso terapéutico , Fallo Hepático Agudo/cirugía , Trasplante de Hígado/efectos adversos , Factores de Edad , Ciclosporina/uso terapéutico , Enfermedad Hepática en Estado Terminal/mortalidad , Femenino , Estudios de Seguimiento , Rechazo de Injerto/prevención & control , Humanos , Terapia de Inmunosupresión/métodos , Incidencia , Lactante , Recién Nacido , Japón/epidemiología , Fallo Hepático Agudo/mortalidad , Trasplante de Hígado/métodos , Donadores Vivos , Masculino , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Tacrolimus/uso terapéuticoRESUMEN
After decades of dramatic surgical innovations in pediatric living donor liver transplantation (LDLT), LDLT for biliary atresia (BA) still poses various challenges. This study reviewed our experience with LDLT for children with post-Kasai BA and evaluated outcomes and prognostic factors. From 2005 to 2016, 168 post-Kasai BA LDLT patients were enrolled and divided into 3 groups by age. Patient characteristics and perioperative data were compared. Predictors of morbidity and mortality following LDLT were analyzed in 93 infants. Outcome was relatively worse in infants than older children, with overall survival at 1 and 5 years of 94.5% and 93.2%, respectively, and graft survival at 1 and 5 years of 91.1% each. Incidence of vascular complications was not significantly higher in infants. High Pediatric End-Stage Liver Disease (PELD) score (odds ratio [OR], 3.72; 95% confidence interval [CI], 1.30-10.67; P = 0.02) and portal vein (PV) hypoplasia (OR, 3.23; 95% CI, 1.10-9.52; P = 0.03) were independent risk factors for morbidity. Low weight-for-age z score (hazard ratio, 5.76; 95% CI, 1.05-31.47; P = 0.03) was identified as a significant risk factor for mortality after LDLT, but not age or absolute body weight (BW). Infants with BW deficit had a significantly smaller PV diameter (P = 0.005), greater blood loss (P = 0.001), and higher incidence of postoperative bacteremia (P = 0.01). In conclusion, high PELD score and PV hypoplasia were independent risk factors for morbidity, and BW deficit was associated with poor survival in infants with post-Kasai BA after LDLT. However, LDLT in these infants at the earliest possible time after referral is a feasible option with excellent patient survival in an experienced center. Liver Transplantation 23 1199-1209 2017 AASLD.
Asunto(s)
Atresia Biliar/cirugía , Enfermedad Hepática en Estado Terminal/cirugía , Trasplante de Hígado/métodos , Portoenterostomía Hepática/efectos adversos , Complicaciones Posoperatorias/cirugía , Adolescente , Adulto , Factores de Edad , Atresia Biliar/mortalidad , Atresia Biliar/patología , Peso Corporal , Niño , Preescolar , Enfermedad Hepática en Estado Terminal/mortalidad , Enfermedad Hepática en Estado Terminal/patología , Femenino , Supervivencia de Injerto , Humanos , Incidencia , Lactante , Japón/epidemiología , Estimación de Kaplan-Meier , Hígado/irrigación sanguínea , Hígado/patología , Donadores Vivos , Masculino , Persona de Mediana Edad , Periodo Perioperatorio , Vena Porta/patología , Vena Porta/cirugía , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/patología , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
AIM: Our aim was to analyze serial changes in the predictive variables and a scoring system retrospectively adapted to evaluate outcomes in pediatric patients with acute liver failure (ALF). METHODS: We retrospectively collected data on 65 patients with ALF. The 65 patients were divided into two groups according to the need for liver transplantation (LT) as follows: LT group (n = 54) and non-LT group (n = 11). The early determination scoring system of the indications for LT proposed by the Intractable Hepato-Biliary Diseases Study Group of Japan (JIHBDSG) was used in our study. The area under the receiver operating characteristic curve (AUROC) was calculated for the JIHBDSG score between the LT group and non-LT group at the time of diagnosis (day 0) and day 3, and day 5 after the diagnosis. RESULTS: A JIHBDSG score of >3 at day 5 was found to identify the patients requiring LT with 83.7% sensitivity, 81.8% specificity, and 83.3% diagnostic accuracy. Based on a comparison of AUROC values, the JIHBDSG score on day 5 (AUROC 0.91) was higher than that on day 0 (AUROC 0.75) and day 3 (AUROC 0.84). CONCLUSION: We showed that a serial analysis of the JIHBDSG score might be useful for predicting outcomes of ALF in pediatric patients who fulfilled the criteria of LT indication in our center. However, further studies are needed to validate our results.
RESUMEN
The patient was a boy of 7 years and 5 months of age, who underwent LDLT for acute liver failure at 10 months of age. HV stent placement was performed 8 months after LDLT because of intractable HV stenosis. At 7 years of age, his liver function deteriorated due to chronic rejection. The patient therefore underwent living donor liver retransplantation from his father. The HV was transected with the stent in situ. The IVC was resected due to stenosis. The pericardial cavity was opened and detached around the IVC to elongate the IVC. The divided ends of the IVC were joined by suturing to the posterior wall of the IVC. A new triangular orifice was made by adding an incision on the anterior wall of the IVC. The graft HV was then anastomosed to the new orifice with continuous sutures in the posterior wall and interrupted sutures in the anterior wall using 5-0 non-absorbable sutures. Doppler ultrasound showed a triphasic waveform. We successfully performed HV reconstruction without a vascular graft. This is a feasible procedure for overcoming HV stenosis in LDLT patients with an indwelling stent.
Asunto(s)
Rechazo de Injerto/cirugía , Venas Hepáticas/cirugía , Enfermedad Veno-Oclusiva Hepática/cirugía , Trasplante de Hígado/métodos , Donadores Vivos , Complicaciones Posoperatorias/cirugía , Vena Cava Inferior/cirugía , Niño , Enfermedad Veno-Oclusiva Hepática/diagnóstico , Enfermedad Veno-Oclusiva Hepática/etiología , Humanos , Masculino , Complicaciones Posoperatorias/diagnóstico , Reoperación , StentsRESUMEN
Ornithine transcarbamylase deficiency (OTCD) is a urea cycle disorder of X-linked inheritance, affecting the detoxification of excess nitrogen and leading to hyperammonemia (hyper-NH3 ). Living donor liver transplantation (LDLT) has been applied for the treatment of OTCD. This case series retrospectively reviewed two OTCD patients who experienced hyper-NH3 following LDLT. The first case was a 5-year-old girl who had onset of OTCD at 2 years of age. Ornithine transcarbamylase (OTC) enzyme activity was 62% for the donor and 15% for the recipient. The patient suffered from recurrence of hyper-NH3 within 2 months following LDLT. The second case was a 5-year-old girl who had onset of OTCD at 3 years of age. OTC enzyme activity was 42.6% for the donor and 9.7% for the recipient. The patient suffered hyper-NH3 for 12 days starting on the date of surgery. Both of the patients transiently required continuous veno-venous hemodialysis; however, they are currently doing well without intensive medical treatment. The use of asymptomatic OTCD heterozygous donors in LDLT has been accepted with careful examination. However, an OTCD heterozygous carrier donor should be avoided if there is another donor candidate, due to the potentially fatal condition of hyper-NH3 following LDLT.