Achar's Textbook Of Pediatrics Pdf 21 |VERIFIED|

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In a domino liver transplantation (DLT), the domino donor receives a traditional liver transplant, typically to correct an underlying metabolic disorder, while providing an otherwise anatomically healthy liver to the domino recipient (figure 1-2). The metabolic disorder of the donor would not manifest in the recipient due to sufficient compensation by the presence of the appropriate enzyme in the remainder of the body. An extraordinary pediatric DLT in the Hospital "Papa Giovanni XXIII" of Bergamo was performed. Two selected patients were undergoing this exceptional surgery. The donor patient was a 17-year-old female who had maple syrup urine disease (MSUD), a rare metabolic syndrome, characterized by progressive hepatic encephalopathy and cognitive impairment. Conventional treatments consisted in a protein-restricted diet, with supplementation of essential amino acids. Liver transplantation is curative because it allows sufficient metabolic activity and a normal diet. The recipient patient was a 10-year-old female that presented Joubert Syndrome and an idiopathic liver cirrhosis with portal hypertension. Once written informed consents were obtained, the patients underwent pediatric DLT. In both patients, we have induced intravenous general anesthesia according to hospital protocol: fentanyl 2 mcg/kg, midazolam 0,2 mg/kg, propofol 3 mg/Kg, cisatracurium 0.02 mg/ Kg and vitamin K 1mg/kg. An intra-arterial catheter to continuously monitor BP, central venous catheter, bispectral index (BIS) and cerebral oximetry using near infrared spectroscopy (NIRS) were positioned. The maintenance of general anesthesia was obtained with lower minimum alveolar concentration (MAC 0.6) of sevorane, cisatracurio 2mcg/kg/min, remifentanil 0,08-0,15 mcg/Kg/min and calcium chloride 6 mg/kg/h, while acid base and electrolyte balances were corrected. Furthermore the donor patient required a continuous perioperative infusion treatment of 20% lipofundin 10 ml/h and 10% Glucose 130 ml/h and aminoacidemia dosage every six hours . Thirty minutes before vena cava declamping, we administered metilprednisolone 10 mg/kg. Concerning fluid therapy, we maintained euvolemia administering crystalloids at the speed of 10 ml/Kg/h and 3 ml/Kg of 5% albumin. In the pre-anhepatic phase, to achieve the target values of mean arterial pressure (MAP) between 60 and 75 mmHg and central venous pressure (CVP) between 8 and 12 mmHg, we started a continuous infusion of norepinephrine (0.03-0.15mcg/kg/min) and epinephrine (0.03-0.10mcg/kg/min). In the neo-hepatic phase, hemodynamic stability was observed and vasopressor requirements were quickly decreased in both patients until the amine infusion was stopped. Diuresis always remained valid, with no need to be stimulated with mannitol or furosemide. At the end of the surgery, the correction of coagulopathy, according to the Thrombelastograph (TEG), was not needed and blood glucose and lactate levels were within normal limits (Table 1; Figure 3). Thus, our results show excellent liver function on admission to the Intensive Care Unit, with optimal clinical outcome. The excellent results obtained and the absence of the onset of new metabolic diseases show that DLT could be a lasting success in pediatrics. 2b1af7f3a8