Ana M. Aristizabal

Hepatitis A, cardiomyopathy, aplastic anemia, and acute liver failure: A devastating scenario

Hepatitis A virus (HAV) causes an acute infection and is usually asymptomatic in children. When clinical manifestations appear, these include choluria, jaundice, and abdominal pain. Although infrequent, extra‐hepatic manifestations related to HAV have been described, affecting the heart, bone marrow, blood vessels, and other tissues.A 10‐year‐old boy from a rural area presented with a 15‐day history of malaise, fever, and jaundice; laboratory examinations were compatible with HAV infection. The patient turned encephalopathic and was remitted to our center, where laboratory examinations showed a medullary aplasia and fulminant hepatitis requiring a liver transplant that was performed 72 hours after admission. At 24 hours post transplant, the patient developed a cardiomyopathy secondary to HAV, and intravenous immunoglobulin was administered. The patient is still alive and attending his medical check‐ups.Although rare, extra‐hepatic manifestations of HAV infection have been described in 14% of cases. The groups of patients affected are usually aged and present with high bilirubin levels. Acquired aplastic anemia and myocarditis caused by HAV are uncommon, and its pathophysiology has not yet been elucidated.HAV infection is usually asymptomatic in children, although extra‐hepatic manifestations can appear requiring early detection and management.

Hepatoblastoma: Transplant Versus Resection Experience in a Latin American Transplant Center

Background Hepatoblastoma is the most common primary malignant liver tumor in children and is usually diagnosed during the first 3 years of life. Overall survival has increased 50% due to chemotherapeutic schemes, expertise surgery centers, and liver transplantation. Methods A retrospective collection of data was performed from pediatric patients with diagnosis of hepatoblastoma. Variables included demographic, diagnostic tools and histological classification; chemotherapy and surgical treatment; and outcomes and patient survival. The PRETEXT classification was applied, which included the risk evaluation, and according to the medical criterion in an individualized way, underwent resection or transplant. The morbidity of patients was evaluated by the Clavien-Dindo classification. Statistical analysis was performed according to the distribution of data and the survival analysis was carried out using the Kaplan-Meier method. Results The patients (n = 16) were divided in a resection group (n = 8) and a transplant group (n = 8). The median age at the time of diagnosis was 13.5 months. The motive for the initial consultation was the discovery of a mass; all patients had high levels of &agr;-fetoprotein and an imaging study. Ten of 16 patients required chemotherapy before the surgical procedure. In the resection group, 5 of 8 patients were classified as Clavien I and 4 of 8 patients of the transplant group were classified as Clavien II. Patient survival at 30 months was 100% in the resection group and 65% in the liver transplantation group. Conclusions To our knowledge, this is the first case report of pediatric patients with hepatoblastoma and liver resection or transplant in Colombia and Latin America. Our results are comparable with the series worldwide, showing that resection and transplant increase the survival of the pediatric patients with hepatoblastoma. It is important to advocate for an increase of reporting in the scientific literature in Latin America.

Xenotrasplantes, una realidad cercana en la práctica clínica: revisión de la literatura

Xenotransplantation could provide an unlimited supply of organs and solve the current shortage of organs for transplantation. To become a reality in clinical practice, the immunological and physiological barriers and the risk of xenozoonosis that they possess should be resolved. From the immunological point of view, in the last 30 years a significant progress in the production of transgenic pigs has prevented the hyperacute rejection. About xenozoonosis, attention has been focused on the risk of transmission of porcine endogenous retroviruses; however, today, it is considered that the risk is very low and the inevitable transmission should not prevent the clinical xenotransplantation. Regarding the physiological barriers, encouraging results have been obtained and its expected that the barriers that still need to be corrected can be solved in the future through genetic modifications.

Liver Transplantation in Hepatitis C–Infected Patients: Experience From a South American Transplant Center

BACKGROUND Around 2.4% of the worlds population is infected with hepatitis C virus (HCV), and it is the most common cause of liver transplantation (LT) in the world. Latin America (LA), with nearly 9% of the world population, has had a continuous increase in the number of LTs per year. Yet, due to the lack of mandatory data collection and a well-developed health-care system, access to transplantation is limited in most LA countries. We report the first LA experience of HCV-infected LT patients. METHODS We performed a retrospective cohort study by reviewing the medical histories of all HCV-infected LT patients between 1996 and 2016 who acquired HCV before their LT, at the Fundación Valle del Lilí, Cali, Colombia. RESULTS Between January 1996 and December 2015, a total of 770 LTs were performed, of which 75 had a cirrhotic liver due to HCV infection. With a median follow-up time of 24.4 months (interquartile range [IQR] 4.7-61.2 months), patient survival was 44.9% and 66.9% for the time periods 1996-2006 and 2007-2015, respectively. Hepatocellular carcinoma (HCC) was present in 30.6% of the patients, and overall postoperative complications had an incidence of 80%. CONCLUSIONS This is the first report of LT in HCV-infected patients in Colombia and in LA. Our results are comparable to those of other transplant centers worldwide with regard to postoperative complications and patient survival. Patients with LT in the 1996-2006 time frame had higher morbidity and mortality. Studies including larger numbers of patients are needed to determine the reason for this finding.

Donantes de órganos en muerte cerebral por herida por proyectil de arma de fuego en cráneo: una trágica realidad que beneficia receptores de órganos en Colombia

Background and objectives: Patients with brain death (BD) due to traumatic brain injury (TBI) secondary to a gunshot (GS) wound in the skull contribute to mitigate the demand for organ and tissue donors. In Colombia, 78% of the homicides are by GS. We describe our experience with BD secondary to TBI by GS organ and tissue donors at a transplant center in Cali, Colombia. Materials and methods: This is a retrospective historical cohort study for characterization of donors diagnosed with BD secondary to TBI by GS at our institution in the period 2010-2016 (n=169). Qualitative variables were assessed by proportions, continuous quantitative variables with measures of central tendency, and survival with

Aspergillus hepatic artery thrombosis in liver transplantation

We present the first cirrhotic patient who underwent liver transplantation (LT) and presented a hepatic artery thrombosis of the graft due to Aspergillus fumigatus, within the first month of LT. This culminated in graft loss, re-transplant with multiple biliary and infectious complications. To our knowledge, this is a case report of an early hepatic artery thrombosis due to Aspergillus fumigatus in an infection-free patient.

Lysosomal Acid Lipase Deficiency, a Rare Pathology: The First Pediatric Patient Reported in Colombia

Patient: Male, 14 Final Diagnosis: Lysosomal acid lipase deficiency (LAL-D) Symptoms: Dyslipidemia • isolated hepatomegaly Medication: — Clinical Procedure: Genetic sequencing Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Lysosomal acid lipase deficiency is a rare genetic metabolic lipid storage disease, with a high morbidity, and mortality, in children and adults. It is characterized by a mutation in the LIPA gene that causes an alteration of lipid metabolism, resulting in deposits of cholesterol esters and triglycerides in organs such as the liver, blood vessels, and gastrointestinal tract. Lysosomal acid lipase deficiency is predominantly caused by the mutation c.894G>A, seen in approximately 50–70% of patients. Our objective is to report the first pediatric case of lysosomal acid lipase deficiency in a pediatric patient in Colombia. Case Report: The patient is a 14-year-old boy with isolated hepatomegaly since 6 years of age without a family history of dyslipidemia. In the pediatric control, laboratory exams revealed dyslipidemia, and a hepatic biopsy was performed, revealing severe fibrosis with septation and grade 3 microvesicular steatosis (>75%). He was referred to our center and was suspected to have lysosomal acid lipase deficiency. Enzymatic activity was measured, showing absent activity. Confirmatory diagnosis with genetic sequencing showed a pathological homozygous mutation of c.894G>A. Conclusions: Lysosomal acid lipase deficiency can manifest as early- or late-onset, with variable and severe signs and symptoms. The late-onset form has a broad spectrum of manifestations with mild symptoms, leading to under-diagnosis, which increases the actual disease burden. Early diagnosis is essential to initiate enzyme replacement therapy, since the natural disease course can be changed. More studies should be conducted in Latin America to evaluate the prevalence of the disease.

Manejo actual del donante potencial de órganos y tejidos en muerte cerebral: guía de manejo y revisión de la literatura

In many cases organ transplantation is the only therapeutic option for patients with terminal essential organ diseases; however, there is an imbalance between the number of organs available and the number of potential receptors for transplants worldwide. Patients with brain death are the main source of organs for transplantation. Promoting early recognition of measures that prevent damage of organs in brain death organ donors is essential for appropriate clinical management. In this framework, it is known that aggressive clinical management of those potential organ donors using standardized protocols are associated with a greater number of transplanted organs and the reduction of the loss of potential donors by cardiac arrest. Colombia is a country with low donation rates and high transplant needs, with a waiting list that increases exponentially each year. In this review we present the international recommendations of potential organ donors management, which we hope can be adopted in institutional protocols to optimize the management of potential donors in Colombia, thus increasing transplants and meeting the needs of the patients.

En-Bloc Transplant of the Liver, Kidney and Pancreas: Experience from a Latin American Transplant Center

Case series Patient: Male, 38 • Male, 48 Final Diagnosis: En-bloc transplantation (liver, kidney, pancreas) Symptoms: Encephalopathy • adynamia • ascites • asthenia Medication: — Clinical Procedure: En-bloc transplantation Specialty: Transplantology Objective: Unusual setting of medical care Background: En-bloc transplantation is a surgical procedure in which multiple organs are transplanted simultaneously. It has some similarities with multi-organ transplantation but offers certain advantages. This report highlights the experience of our interdisciplinary group regarding the treatment and follow-up of patients who received en-bloc transplantation, with the aim of encouraging the development of this surgical technique. Case Report: The first case is a 38-year-old patient with type 1 diabetes mellitus, liver cirrhosis, and chronic kidney failure who received an en-bloc transplant of the liver, pancreas, and kidney with no intraoperative complications. He had a prolonged hospital stay due to anemia and systemic inflammatory response syndrome, which were resolved successfully. At follow-up, he had no requirement for insulin or for dialysis, or for new interventions. The second case describes a 48-year-old patient with type 2 diabetes mellitus, renal failure, and liver cirrhosis who received an en-bloc transplant of the liver, pancreas, and kidney with no complications. During the postoperative period, the patient suffered a possible episode of acute tubular necrosis, which evolved towards improvement, with a tendency to normal metabolic and renal functioning, with no additional events. The patient is currently in follow-up and is insulin-independent. Conclusions: En-bloc transplantation is a safe procedure, which is technically simple and which achieves excellent results. This procedure is indicated in patients with end-stage renal disease, cirrhosis, and diabetes mellitus that is difficult to control.