Luis Carlos Jiménez Romero

Trasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años

BACKGROUND AND OBJECTIVE Familial amyloid polyneuropathy (FAP) is the most prevalent type of hereditary systemic amyloidosis. It is an autosomal dominant disease characterized by the deposition of an abnormal variant transthyretin. It has a worldwide distribution, with localized endemic areas in Portugal, Sweden and Japan. In Spain there is an endemic focus, located in Mallorca. Liver transplantation is the only curative option for patients with FAP. The aim of this study was to describe the clinical and demographic characteristics of patients transplanted with a diagnosis of PAF. MATERIAL AND METHOD Six patients with PAF underwent liver transplantation between April 1986 and December 2012. RESULTS The mean age was 57.7+16 years, patients of Spanish origin were older than 60 years. All patients had progressive symptoms as mixed polyneuropathy. In 2 patients, combined heart-liver transplants sequentially were performed. Patient survival and graft was 80% at one, 3 and 5 years. CONCLUSIONS The only effective treatment for etiologic PAF is liver transplantation. Early detection is the key to the treatment and control, avoiding the irreversible organ damage.

Complete splenic embolization for the treatment of refractory ascites after liver transplantation

Refractory ascites is an uncommon complication that may develop postoperatively after liver transplantation. The diagnosis and treatment of this condition is a real challenge. We report two cases of patients who underwent a transplant due to cryptogenic cirrhosis and developed refractory ascites during the immediate postoperative period. This is a serious complication associated with decreased survival by up to one year and a reduced quality of life. After ruling out the main causes of ascites, a portal hyperflow was a potential etiology. This condition perpetuates itself with splenic circulation and brings about a reduction in the hepatic arterial flow. Therefore, if arterial blood flow to the spleen is diminished, venous return and portal circulation will be reduced and arterial blood flow will improve. Splenic artery embolization is a procedure introduced many years ago for the management of splenic artery steal syndrome and small-for-size living donor liver transplantation. This procedure is performed in order to reduce portal hyperflow and consequently, ascites. In conclusion, splenic artery embolization is a therapeutic option for the treatment of refractory ascites after liver transplantation.

Liver Transplantation as Curative Treatment in Severe Pulmonary Hypertension due to Hepatic Vascular Malformations in Rendu-Osler-Weber Disease

Introduction Hereditary haemorragic telangiectasia (HHT) is an autosomal dominant disease characterised by development of arterio-venous malformations (AVM) more common in mucosa, brain, lung, liver and gastrointestinal tract. Pulmonary hypertension (PH) is defined as mean pulmonary arterial pressure (mPAP) > 25 mmHg. Liver transplatation is still controversial in this patients. MaterialsMethods Woman, 38 years old, with personal history of eclampsia, HELLP síndrome and HHT type 2 (Rendu-Osler-Weber) with exon 9 in the ALK1 gen mutation that presents as systemic manifestations self-limited epistaxis and liver AVM with mainly arterio-systemic intrahepatic shunt which lead to high output cardiac arrest and moderate to severe PH with a NYHA II functional class. Lung and brain AVM were not observed in radiologic tests and bubble test was positive due to extracardiac shunt (>6 heart-beats). Results Due to the high risk related to liver transplantation (LT) in this patient, transarterial embolization of liver vascular malformations was performed first, with a 24% reducción in cardiac output (table 1) and development of several complications as intranodal reentry tachycardia and ischemic hepatitis. Because of partial improvement treatment with bevacizumab was iniciated, despite this, the patient suffered recurrent episodes of cardiac arrest and liver function deterioration (MELD 25). In July 2016, orthotopic LT was performed with a cold ischemia time of 355 min and a warm ischemia time of 40 min, during which he bled profusely requiring massive blood transfusion. After LT, a 53.2% reduction in the cardiac output was observed in a right catheterism, because extracardiac shunt was eliminated. Furthermore, a pulmonary vascular resistance (PVR) reduction, wich means an absence of vascular branch remodelation, and a normalization in mPAP was shown; which demonstrate the resolution of HP after LT (table 2). Table. No title available. Table. No title available. Conclusion - LT might be a curative treatment in patients with haemorrhagic vascular diseases if severe HP and cardiac arrest are due to liver AVM - Liver AVM embolization in moderate-severe HP with elevated cardiac output has mild results - Diagnosis of Lung AVM causing HP, by angio-TC or angiography, is essential because might contraindicated isolated LT.

Use of Norepinephrine in Pancreas Donors and its Influence on Patient and Graft Survival

Introduction Brain death originates a vasomotor center dysfunction, a decrease in catecholamine release and finally vasodilatation secondary to the decrease of the peripheral vascular resistence. To this we should add the relative dehydration resulting from a mixture of previous water restriction and polyuria secondary to vasopresin deficiency that will originate hypovolemia and hypotension in a potential donor. Because the most important factor in the viability and graft function is adequate donor perfusion, it has been postulated that donor catecholamines administration might have a beneficial effect on kidney function, while its effect on other organs is unknown. The aim of this study was to determine if norepinephrine in the donor is associated with recipient and pancreatic graft survival. Methods We performed a retrospective and comparative study, between the patients transplantated with a pancreatic graft proceeding from a donor where norepinephrine was used (Group A) and patients transplantated with a graft where norepinephrine was not used (Group B). Results 165 patients that underwent simultaneous pancreatic-kidney transplantation were included. The mean recipient age was 38.9 years, while the donor age was 28.6 years. 58.4% of the donors and 66.5% of the recipients were men. Grafts from group A presented an adequate function in 69% of the cases (p=0.03). The patient actuarial survival at 1.3 and 5 years in group A was: 94.9%, 93.3% and 92.5% respectively and in group B: 96.6%, 89.5% and 89.5% (p=0.6). The graft actuarial survival in group A was 82.8%, 78.9%, 72.9%, as compared to 75,9%, 68,4% and 63,8% respectively in group B (p=0.05). Conclusion Pancreatic grafts proceeding from donors who received norepinephrine present a significantly increased graft survival compared to grafts proceeding from donors without norepinephrine. No differences were seen in the patient survival between both groups.

Tumores mucinosos del apéndice: incidencia, diagnóstico y tratamiento quirúrgico

INTRODUCTION Mucinous tumors of the appendix are a rare pathology, with a prevalence below 0.5%. Clinical presentation usually occurs during the sixth decade of life, and mucinous tumors can clinically mimic acute appendicitis. The aim of this study is to describe the clinical and demographic variables, therapeutic procedure and diagnosis of these tumors. We analyze the association between mucinous tumors and pseudomyxoma peritonei (PP), as well as the association with colorectal and ovarian tumors. METHODS A retrospective study was performed including patients who underwent an appendectomy between December 2003 and December 2014. RESULTS Seventy-two mucinous tumors of the appendix were identified among 7.717 patients reviewed, resulting in a prevalence of 0.9%. Mean age at presentation was 64 years, 62% patients were female and 38% males. An incidental diagnosis was made in 43% of patients. Mucinous tumors of low malignant potential were significantly related to the presence of pseudomyxoma peritonei, identified in 16 (22%) of the cases. We also observed an increased risk of ovarian mucinous tumors in patients with a diagnosis of appendiceal mucinous neoplasm. In our sample, 22 (30.5%) patients showed a synchronous or metachronous colorectal cancer. CONCLUSIONS Appendiceal mucinous tumors are frequently an incidental finding. The diagnosis of mucinous tumors of low malignant potential is a factor associated with the development of pseudomyxoma peritonei. Histologic tumor grade and the presence of peritoneal dissemination will determine surgical treatment that can vary, from appendectomy to cytoreductive surgery.