Is There Any Difference in Anesthetic Management of Biliary Atresia and Glycogen Storage Disease Patients Undergoing Liver Transplantation?

Jawan, Bruno; Tseng, Chia Chih; Chen, Yaw Sen; Wang, Chih Chi; Cheng, Yu; Huang, Tung‐Liang; Eng, Hock Liew; Chiu, King-Wah; Wang, Shir Hor; Lin, Chia‐Yu; Lin, Tzu-Chao; Liu, Yueh Wei; Chen, Chao Long

doi:10.1016/j.jss.2005.01.004

Cited by 11 publications

(7 citation statements)

References 23 publications

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“…The information for each patient was anonymized and deidentified before analysis. The general anesthesia, intraoperative monitoring and blood transfusion protocols used [9] , as well as their documentation [10] , were similar to those described in previously published reports [5,9,[11][12][13] . Estimated blood losses were made through visual estimations of a small (250 mL) suction bottle (Receptal ® Accurate Measurement Device, Hospira, Inc. Lake Forest, Il 60045, United States) and the weight of laparotomy sponges.…”

Section: Patient Selection and Data Collectionmentioning

confidence: 68%

Living donor liver transplantation with body-weight more or less than 10 kilograms

Yang

Huang

Chen

et al. 2015

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AIM:To compare the outcomes of pediatric patients weighing less than or more than 10 kg who underwent liver transplantation. METHODS: Data for 196 pediatric patients who underwent living donor liver transplantation betweenJune 1994 and February 2011 were reviewed retrospectively. The information for each patient was anonymized and de-identified before analysis. The data included information regarding the pre-transplant conditions, intraoperative fluid replacement and outcomes for each patient. The 196 patients were divided into two groups: those with body weights of less than 10 kg were included in group 1 (G1; n = 101), while those with body weights of more than 10 kg were included in group 2 (G2; n = 95). For each group, the patients' ages, body weights, heights, pediatric end stage liver disease scores, anesthesia times, and warm and cold ischemic times were analyzed. In addition, between-group comparisons were also made. Mann-Whitney U tests were used to compare all the variables except for complications and survival rates, which were analyzed using χ 2 tests and Kaplan-Meier tests, respectively. RESULTS:The general medical conditions of the G1 Retrospective StudyLiving donor liver transplantation with body-weight more or less than 10 kilograms patients were worse than those of the G2 patients, as shown by the higher pediatric end stage liver disease scores and poorer Z -scores. In addition, the preoperative Hb and serum albumin levels were all lower for the G1 patients than for the G2 patients. The G1 patients also had significantly more intraoperative blood loss than the G2 patients. In addition, the intraoperative fluid requirements for the G1 patients, including leukocyte poor red blood cell transfusions, 5% albumin infusions and crystalloid infusions, were significantly higher than those for the G2 patients. The risk of intraoperative portal vein thrombosis was higher for the patients in G1 than for those in G2. However, the one-year survival rates (95.9% and 96.8% for G1 and G2, respectively) and three-year survival rates (94.9% and 94.6% for G1 and G2, respectively) for both groups were similar. CONCLUSION:Patients weighing less than 10 kg typically have poorer conditions, but their survival rates are comparable to those of children weighing more than 10 kg. Core tip: Although pediatric patients weighing less than 10 kg who underwent living donor liver transplantation usually had worse pre-transplant conditions, more intraoperative bleeding and more portal vein complications, their three-year survival rate was 94%, a rate comparable to that achieved by children weighing more than 10 kg. A prerequisite for achieving these excellent results is good and effective teamwork. Cooperation between surgeons, plastic surgeons, and anesthesiologists in the operating theater is fundamental, but the contributions of radiologists are also critical. Radiologists should be available in the operating room to perform Doppler ultrasounds or place vascular stents, as required.Yang SC, Huang CJ, Chen CL, Wang CH,...

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Section: Patient Selection and Data Collectionmentioning

confidence: 68%

Living donor liver transplantation with body-weight more or less than 10 kilograms

Yang

Huang

Chen

et al. 2015

WJG

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“…LT has been reported in several patients to correct the hypoglycemia and other biochemical abnormalities, and growth impairment 11–13. Prior to surgery, good metabolic control needs to be established and glucose levels should be carefully maintained preoperatively and during surgery with intravenous dextrose 14…”

Section: Discussionmentioning

confidence: 99%

Long-term results of living donor liver transplantation for glycogen storage disorders in children

et al. 2007

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Liver transplantation (LT) may be indicated in glycogen storage disorders (GSD) when medical treatment fails to control the metabolic problems or when hepatic adenomas develop. We present our institutional experience with living donor LT (LDLT) for children with GSD. A total of 244 patients underwent primary LDLT at our institution from June 1994 to December 2005. A total of 12 (5%) children (8 female and 4 male) were afflicted with GSD and were not responsive to medical treatment. Nine patients had GSD type I and 3 had GSD type III. The median age at the time of transplantation was 7.27 yr (range, 2.4-15.7). All patients presented with metabolic abnormalities, including hypoglycemia, and lactic acidosis. In addition, 4 patients presented with growth retardation. A total of 11 patients received left lobe grafts and 1 received a right lobe graft. The mean graft-to-recipient weight ratio was 1.25 (range, 0.89-1.61). Two patients had hepatic vein stenoses that were treated by balloon dilatation; 1 patient had bile leak, which settled spontaneously. The overall surgical morbidity rate was 25%. Three patients had hepatic adenomas in the explanted liver. There was a single mortality at 2 months posttransplantation due to acute pancreatitis and sepsis. The mean follow up was 47.45 months. The metabolic abnormalities were corrected and renal function remained normal. In patients with growth retardation, catch-up growth was achieved posttransplantation. In conclusion, LDLT is a viable option to restore normal metabolic balance in patients with GSD when medical treatment fails. Long-term follow-up after LT for GSD shows excellent graft and patient survival. Liver Transpl 13:848-852, 2007. © 2007 AASLD.Received September 15, 2006; accepted February 6, 2007. Carbohydrate metabolism in the liver is responsible for plasma glucose homeostasis. Glycogen storage diseases (GSD) are metabolic disorders that result in abnormal storage amounts and/or forms of glycogen in tissues. 1 GSD type I (von Gierke disease) is caused by deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestinal mucosa with glycogen overloading in these organs. Classically, patients with GSD type I either present in the neonatal period with hypoglycemia and lactic acidosis, or present at 3-4 months of age with hepatomegaly and/or hypoglycemic seizures.2 The other manifestations include systemic acidosis, hyperlipidemia, hyperuricemia, and growth retardation. Without effective treatment, long-term complications occur including gout, osteoporoAbbreviations: GSD, glycogen storage disorders; LT, liver transplantation; LDLT, living donor LT.

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“…3,5,6 The anesthesia management followed the protocol of the Department of Anesthesiology. 7,8 Red blood cell transfusions were not given to patients with hemoglobin levels > 8.0 g/ dL and as long as the intravascular volume was sufficient to maintain normal hemodynamics.…”

Section: Methodsmentioning

confidence: 99%

Solitary pulmonary nodule in the liver transplant candidate: Importance of diagnosis and treatment

et al. 2010

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Our objectives were to define the incidence and etiology of solitary pulmonary nodules (SPNs) in patients undergoing living donor liver transplantation (LDLT), describe a diagnostic approach to the management of SPNs in LDLT, and define the impact of SPNs on the overall survival of adult LDLT recipients. Nine patients (9/152, 5.9%) were diagnosed with an SPN on the basis of chest radiography findings during the pretransplant survey. All were male. The mean age was 52 years. All the patients had hepatitis B virus-related cirrhosis with hepatocellular carcinoma. All were asymptomatic for the lung lesion. All underwent contrast-enhanced chest computed tomography (CT) to verify the presence and possible etiology of the SPNs. In 3 cases, CT was used to definitely determine that there was no pulmonary nodule; in 2, CT led to a definite diagnosis of pulmonary tuberculosis. In 4, CT led to a definite identification of an SPN but not to an etiological diagnosis. Two patients underwent outright thoracoscopy and biopsy of their SPNs. Biopsy showed cryptococcosis in both patients. One received a therapeutic trial of an antituberculosis treatment, and repeat CT after 1 month showed a regression in the size of the SPN. A diagnosis of tuberculosis was made. One patient had an inconclusive whole body positron emission tomography scan and subsequently underwent thoracoscopy where biopsy showed tuberculosis. A concomitant malignancy, either primary lung cancer or metastasis from the liver tumor, was not identified. All patients were surviving with their original grafts and were lung infection-free. The overall mean posttransplant follow-up was 54 months (range ¼ 33-96 months). Patients undergoing transplantation require a thorough clinical evaluation to exclude overt and occult malignancies that would preclude the planned procedure. This is important because routine immunosuppression use after transplantation may often result in the body's loss of immunoregulatory functions and thus in a myriad of problems, including oncological and infectious processes. Occult infections may manifest as fulminant, life-threatening sepsis after transplantation.Indeterminate pulmonary nodules in transplant candidates pose both diagnostic and therapeutic

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Is There Any Difference in Anesthetic Management of Biliary Atresia and Glycogen Storage Disease Patients Undergoing Liver Transplantation?

Cited by 11 publications

References 23 publications

Living donor liver transplantation with body-weight more or less than 10 kilograms

Living donor liver transplantation with body-weight more or less than 10 kilograms

Long-term results of living donor liver transplantation for glycogen storage disorders in children

Solitary pulmonary nodule in the liver transplant candidate: Importance of diagnosis and treatment

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