Decreased Long-Term Severe Acute Respiratory Syndrome Coronavirus 2-Specific Humoral Immunity in Liver Transplantation Recipients 12 Months After Coronavirus Disease 2019.

Long-term humoral immunity and its protective role in liver transplantation (LT) patients have not been elucidated. We performed a prospective multicenter study to assess the persistence of immunoglobulin G (IgG) antibodies in LT recipients 12 months after coronavirus disease 2019 (COVID-19). A total of 65 LT recipients were matched with 65 nontransplanted patients by a propensity score including variables with recognized impact on COVID-19. LT recipients showed a lower prevalence of anti-nucleocapsid (27.7% versus 49.2%; P = 0.02) and anti-spike IgG antibodies (88.2% versus 100.0%; P = 0.02) at 12 months. Lower index values of anti-nucleocapsid IgG antibodies were also observed in transplantation patients 1 year after COVID-19 (median, 0.49 [interquartile range, 0.15-1.40] versus 1.36 [interquartile range, 0.53-2.91]; P < 0.001). Vaccinated LT recipients showed higher antibody levels compared with unvaccinated patients (P < 0.001); antibody levels reached after vaccination were comparable to those observed in nontransplanted individuals (P = 0.70). In LT patients, a longer interval since transplantation (odds ratio, 1.10; 95% confidence interval, 1.01-1.20) was independently associated with persistence of anti-nucleocapsid IgG antibodies 1 year after infection. In conclusion, compared with nontransplanted patients, LT recipients show a lower long-term persistence of anti-severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antibodies. However, SARS-CoV-2 vaccination after COVID-19 in LT patients achieves a significant increase in antibody levels, comparable to that of nontransplanted patients.

Changes in humoral immune response after SARS-CoV-2 infection in liver transplant recipients compared to immunocompetent patients.

The protective capacity and duration of humoral immunity after SARS-CoV-2 infection are not yet understood in solid organ transplant recipients. A prospective multicenter study was performed to evaluate the persistence of anti-nucleocapsid IgG antibodies in liver transplant recipients 6 months after coronavirus disease 2019 (COVID-19) resolution. A total of 71 liver transplant recipients were matched with 71 immunocompetent controls by a propensity score including variables with a well-known prognostic impact in COVID-19. Paired case-control serological data were also available in 62 liver transplant patients and 62 controls at month 3 after COVID-19. Liver transplant recipients showed a lower incidence of anti-nucleocapsid IgG antibodies at 3 months (77.4% vs. 100%, p < .001) and at 6 months (63.4% vs. 90.1%, p < .001). Lower levels of antibodies were also observed in liver transplant patients at 3 (p = .001) and 6 months (p < .001) after COVID-19. In transplant patients, female gender (OR = 13.49, 95% CI: 2.17-83.8), a longer interval since transplantation (OR = 1.19, 95% CI: 1.03-1.36), and therapy with renin-angiotensin-aldosterone system inhibitors (OR = 7.11, 95% CI: 1.47-34.50) were independently associated with persistence of antibodies beyond 6 months after COVID-19. Therefore, as compared with immunocompetent patients, liver transplant recipients show a lower prevalence of anti-SARS-CoV-2 antibodies and more pronounced antibody levels decline.

Trasplante hepatorrenal simultáneo en pacientes adultos con hiperoxaluria primaria. Experiencia del Hospital Universitario 12 de Octubre / Liver-kidney simultaneous transplantation in adult patients with primary hyperoxaluria. Experience at Hospital Universitario 12 de Octubre

La hiperoxaluria primaria es un trastorno del metabolismo autosómico recesivo que origina una hiperproducción hepática de oxalato, que no puede ser metabolizado por el hígado y se elimina por vía renal formando litiasis, nefrocalcinosis y ocasionando un deterioro progresivo y precoz de la función renal que, generalmente, a pesar del tratamiento médico precisará de una terapia renal sustitutiva. La hiperoxaluria primaria (HOP) tipo 1 es el trastorno más frecuente, se debe a un déficit de la enzima alanina-glicolato aminotransferasa que se encuentra en los peroxisomas hepáticos. Por tanto, el trasplante hepatorrenal simultáneo (THRS) es el tratamiento definitivo para los pacientes con enfermedad renal crónica terminal. Sin embargo, algunos resultados sugieren que la morbimortalidad es mayor al realizar este procedimiento frente al trasplante renal aislado. Se presentan cinco pacientes adultos con hiperoxaluria primaria y un filtrado glomerular medio de 20,2 ± 1,3 ml/min/1,73 m2 a los que se les realizó un THRS entre 1999 y 2015 en el Hospital Universitario 12 de Octubre. No se observó recurrencia de la enfermedad ni pérdida del injerto hepático o renal durante el postoperatorio y únicamente un episodio de rechazo agudo tardío sin pérdida del injerto renal. La supervivencia de los receptores fue del 100% con una mediana de seguimiento de 84 meses. Debido a que el THRS permite la curación de la enfermedad y constituye una técnica segura, con una baja morbimortalidad y elevada supervivencia, debe considerarse como el tratamiento de elección en la hiperoxaluria primaria con enfermedad renal terminal (AU)

Primary hyperoxaluria (PH) is a metabolic liver disease with an autosomal recessive inheritance that results in oxalate overproduction that cannot be metabolized by the liver. Urinary excretion of oxalate results in lithiasis and nephrocalcinosis leading to a progressive loss of renal function that often requires renal replacement therapy despite medical treatment. Type 1 PH is the most common form and is due to a deficiency in the alanine-glycolate aminotransferase enzyme found in hepatic peroxisomes. Therefore, a liver-kidney simultaneous transplant (LKST) is the definitive treatment for end-stage renal disease (ESRD) patients. However, some studies suggest that the morbidity and mortality rates are greater when this procedure is performed instead of only a kidney transplant (IKT). Herein, we report five patients with PH and a mean glomerular filtration rate of 20.2 ± 1.3 ml/min/1.73 m2 who received a LKST between 1999 and 2015 at the Hospital Universitario 12 de Octubre. Recurrence and liver or kidney graft loss was not observed during the postoperative period and only one case of late acute rejection without graft loss was diagnosed. The recipient survival rate was 100% with a median follow up of 84 months. As LKST is a curative and safe procedure with a low mortality and high survival rate, it must be considered as the treatment of choice in adults with HP and ESRD (AU)

Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment. / 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.

Tumores mucinosos del apéndice: incidencia, diagnóstico y tratamiento quirúrgico / Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment

Introducción: El tumor mucinoso de apéndice tiene una incidencia inferior al 0,5% entre todos los tumores digestivos. Suele presentarse en la sexta década de la vida, con una clínica parecida a la de la apendicitis aguda. El objetivo de este estudio es describir los aspectos demográficos, clínicos, diagnósticos y terapéuticos de estos tumores. Además, se analiza la asociación entre tumores mucinosos con pseudomixoma peritoneal (PP) y la relación que presentan con los tumores de ovario y colorrectales. Métodos: Se realizó un estudio retrospectivo de todas las apendicectomías practicadas en nuestro centro desde diciembre de 2003 hasta diciembre de 2014. Resultados: Entre 7.717 apendicectomías diagnosticamos un tumor mucinoso apendicular en 72 pacientes, lo que representa una incidencia de 0,9%. La edad media era de 64 años; eran mujeres el 62% y hombres, el 38%. El diagnóstico fue incidental en el 43% de los casos. El PP se presentó en 16 casos (22%), con una asociación estadísticamente significativa entre este tumor y el tumor de bajo potencial maligno. La cirugía programada se realizó en 42 casos y la urgente en 30. De los 72 tumores mucinosos del apéndice, 22 (30,5%) también presentaron cáncer de colon sincrónico o metacrónico. Conclusiones: Los tumores mucinosos de apéndice son con frecuencia hallazgos incidentales. El PP se asocia con un tumor mucinoso de bajo potencial maligno y el tratamiento puede comprender desde una apendicectomía hasta una cirugía citorreductiva, dependiendo del grado histológico del tumor y de la diseminación peritoneal (AU)

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 (AU)

Trasplante hepático y cardiaco simultáneo en pacientes con polineuropatía amiloidótica familiar / Simultaneous liver and heart transplantation in patients with familial amyloid polyneuropathy

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Trasplante hepático como tratamiento de la polineuropatía amiloidótica familiar en pacientes mayores de 60 años / Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years

Fundamento y objetivo: La polineuropatía amiloidótica familiar (PAF) constituye el tipo más prevalente de amiloidosis sistémica hereditaria. Es una enfermedad autosómica dominante que se caracteriza por el depósito de una variante anómala de la transtiretina. Tiene una distribución mundial, con zonas endémicas localizadas en Portugal, Suecia y Japón. En España hay un foco endémico, localizado en Mallorca. El trasplante hepático es la única opción curativa para los pacientes con PAF. El objetivo de este estudio fue describir las características clínicas y demográficas de los pacientes trasplantados con diagnóstico de PAF. Material y método: Se evaluaron 6 pacientes trasplantados por PAF entre abril de 1986 y diciembre de 2012. Resultados: La edad media fue de 57,7 + 16 años, los pacientes de origen español eran mayores de 60 años. Todos los pacientes presentaban síntomas progresivos en forma de polineuropatía mixta. En 2 pacientes se realizó un doble trasplante hepatocardiaco secuencial, efectuándose en primer lugar el trasplante hepático. La supervivencia del paciente y del injerto fue del 80% a los uno, 3 y 5 años. Conclusiones: El único tratamiento etiológico eficaz para la PAF es el trasplante hepático. Una detección temprana es la clave para el tratamiento y el control, evitándose el daño orgánico irreversible (AU)

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 (AU)

[Liver transplantation as treatment of familial amyloid polyneuropathy in patients older than 60 years]. / 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.

Rotura hepática espontánea secundaria al uso de esteroides anabolizantes / Spontaneous hepatic rupture associated with the use of anabolic steroids

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Enfermedad de Hirschsprung, mutación del protooncogén RET y tiroidectomía profiláctica / Hirschsprung's disease, RET proto-oncogene mutation and prophylactic thyroidectomy

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