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1.
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1556612

RESUMO

El absceso hepático tiene baja incidencia, pero alta mortalidad. Su diagnóstico suele ser tardío dada la variabilidad de presentaciones clínicas. Presentamos tres casos en mujeres, de los cuales dos se manifestaron con síndrome febril con microbiología no precisada, mientras que el tercero con shock séptico secundario a colangitis, aislándose Escherichia coli. Todos fueron manejados con antibioticoterapia endovenosa empírica y drenaje quirúrgico, uno vía laparoscópica, dos percutáneos y uno asociado a una colangio-pancreatografía retrógrada endoscópica. La elección terapéutica se determina de acuerdo a la presencia de rotura, tamaño y/o loculaciones del absceso, combinando antibioticoterapia con métodos de drenaje quirúrgico mínimamente invasivos. Se contrasta y discute la bibliografía disponible, destacando la necesidad de investigaciones actualizadas en Chile.


Liver abscess has low incidence but high mortality. Its diagnosis is often delayed due to the variability of clinical presentations. We present three cases in women, two of which manifested with a febrile syndrome with unspecified microbiology, while the third presented with septic shock secondary to cholangitis, with Escherichia coli isolated. All cases were managed with empirical intravenous antibiotic therapy and surgical drainage, one through laparoscopy, two through percutaneous methods, and one associated with endoscopic retrograde cholangiopancreatography. The therapeutic approach is determined based on the presence of rupture, size, and/or loculations of the abscess, combining antibiotic therapy with minimally invasive surgical drainage methods. We discuss the available literature, emphasizing the need for updated research in Chile.

2.
Journal of Clinical Hepatology ; (12): 606-610, 2024.
Artigo em Chinês | WPRIM | ID: wpr-1013145

RESUMO

Primary biliary cholangitis (PBC) is a liver autoimmune disease with a strong genetic tendency characterized by the degeneration and necrosis of bile duct epithelial cells, and it is often observed in middle-aged and elderly women. With the continuous development of genome-wide association studies, the genetic susceptibility of PBC has attracted more and more attention. This article elaborates on the research advances in the genetic susceptibility genes closely associated with PBC, in order to provide effective targets for the treatment of PBC.

3.
Journal of Clinical Hepatology ; (12): 496-501, 2024.
Artigo em Chinês | WPRIM | ID: wpr-1013127

RESUMO

ObjectiveTo investigate the value of baseline red cell distribution width (RDW) and alkaline phosphatase (ALP) level after ursodeoxycholic acid (UDCA) treatment for one month in predicting the response to UDCA treatment in patients with primary biliary cholangitis (PBC). MethodsA retrospective analysis was performed for the data of 127 patients with PBC who were diagnosed in Department of Hepatology, The Third People’s Hospital of Jiangsu University, from January 2015 to July 2022, with data collected at baseline, after one month of treatment, and after one year of follow-up. Based on the Paris-I criteria, the patients were divided into good response group and poor response group, and the two groups were analyzed in terms of clinical and laboratory features and their association with response to UDCA. The Logistic regression method was used to investigate the independent risk factors for response to UDCA treatment. The area under the ROC curve (AUC) was used to determine the optimal cut-off values of related indicators; the patients were divided into two groups based on such values, and the two groups were compared in terms of baseline indicators and response. The independent-samples t test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups; the chi-square test was used for comparison of categorical data between two groups. ResultsCompared with the good response group, the poor response group had significantly higher levels of total bilirubin, aspartate aminotransferase/alanine aminotransferase, ALP, RDW, and RDW-CV at baseline and a significantly higher level of ALP after one month of UDCA treatment (Z=-4.792, -3.697, -2.399, -4.102, -3.220, and -4.236, all P<0.05). Compared with the good response group, the poor response group had significantly lower levels of albumin, hemoglobin, lymphocytes, hematocrit, and body mass index at baseline (Z=-3.592, -3.603, -2.602, -3.829, -2.432, all P<0.05), as well as significantly lower levels of prealbumin, albumin/globulin ratio, apolipoprotein A, and free triiodothyronine at baseline (t=4.530, 3.402, 3.485, and 3.639, all P<0.001). Compared with the poor response group, the good response group had a significantly lower proportion of patients with liver cirrhosis, gallstones/cholecystitis, or anemia (χ2=20.815, 3.892, and 12.283, all P<0.05). Baseline RDW (odds ratio [OR]=1.157, 95% confidence interval [CI]: 1.028‍ — ‍1.301, P=0.015) and ALP level after one month of treatment (OR=1.012, 95%CI: 1.005‍ — ‍1.020, P=0.002) were independent risk factors for response to UDCA, with an AUC of 0.713 and 0.720, respectively. The patients with baseline RDW≥upper limit of normal (ULN) and ALP≥2.2×ULN after one month of UDCA treatment had a lower UDCA response rate (42.6% vs 8.2%, χ2=20.813, P<0.001). ConclusionPatients with baseline RDW≥ULN and ALP≥2.2×ULN after one month of UDCA treatment tend to have a low biochemical response rate to UDCA.

4.
Journal of Clinical Hepatology ; (12): 157-160, 2024.
Artigo em Chinês | WPRIM | ID: wpr-1006442

RESUMO

Primary biliary cholangitis (PBC) is a chronic autoimmune disease of cholestasis in which immune factors lead to progressive small bile duct destruction, cholestasis, and eventually liver fibrosis, liver cirrhosis, and even liver failure. Macrophages, as a group with functional heterogeneity, play different roles in the whole disease process of PBC. This article summarizes the possible ways by which macrophages are involved in the pathogenesis of PBC and discusses their impact on the disease and the potential therapeutic targets of macrophages. It is pointed out that macrophages are mainly involved in innate immunity in PBC injury and are associated with gut microbiota dysbiosis, and they are also associated with cholestasis, liver fibrosis, and liver cirrhosis in the later stages of the disease.

5.
Journal of Clinical Hepatology ; (12): 151-156, 2024.
Artigo em Chinês | WPRIM | ID: wpr-1006441

RESUMO

Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive and non-purulent inflammation of small- and medium-sized bile ducts in the liver. Recent studies have shown that abnormal lipid metabolism is relatively common in patients with PBC, and 76% of PBC patients have dyslipidemia. The effects and harms of dyslipidemia have attracted much attention. Lipid metabolism disorders play an important role in the progression of PBC. This article mainly reviews the research advances in the manifestation, role, diagnosis, and treatment of lipid metabolism disorders in PBC, so as to provide new ideas for the treatment of PBC.

6.
Journal of Traditional Chinese Medicine ; (12): 198-204, 2024.
Artigo em Chinês | WPRIM | ID: wpr-1005371

RESUMO

ObjectiveTo observe the clinical effectiveness and safety of She medicine (畲药) Diren Zishen Formula(地稔滋肾方) combined with acupuncture as adjunctive treatment for primary biliary cholangitis with liver and kidney yin deficiency syndrome. MethodsSeventy patients of primary biliary cholangitis with liver and kidney yin deficiency syndrome were randomly divided into a control group and a treatment group, with 35 patients in each group. The control group received oral ursodeoxycholic acid capsules (250 mg per dose, three times daily). The treatment group received She medicine Diren Zishen Formula oral decoction (one dose daily, 200 ml per dose in the morning and evening, served warm) and acupuncture [bilateral Sanyingjiao (SP6), Taichong (LR3), Ganshu (BL18), Zusanli (ST36), Fenglong (ST17), once daily, 5 consecutive days per week] in addition to the same treatment as the control group. The treatment duration was three months for both groups. Comparisons were made between the two groups before and after treatment for the following parameters, which were four traditional Chinese medicine (TCM) symptoms scores (skin itching, fatigue, jaundice, and flank pain), TCM syndrome scores, liver function indicators including aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT) and total bilirubin (TBiL), liver fibrosis markers including serum laminin (LN), serum hyaluronic acid (HA), serum type Ⅳ collagen (Ⅳ-C) and serum type Ⅲ procollagen (PC-Ⅲ), and inflammatory factor indicators including serum interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α). The effectiveness of TCM syndrome between the two groups was compared and safety evaluations were also conducted after treatment. ResultsA total of 32 cases were finally analyzed in the treatment group, while the control group had 31 cases. The total effective rate of TCM syndrome in the treatment group (87.50%, 28/32) was higher than that in the control group (67.74%, 20/31) (P<0.05). After treatment, the TCM symptom scores, syndrome scores, liver function, and liver fibrosis markers in both groups signi-ficantly decreased, while in the treatment group, the inflammatory factor indicators decreased after treatment, and more decreases were found than those in the control group (P<0.05 or P<0.01). Both groups had good safety, and no adverse reactions were observed. ConclusionThe combination of She medicine Diren Zishen Formula and acupuncture as an adjunctive treatment for primary biliary cholangitis can significantly improve the clinical effectiveness, improve liver function, reduce inflammatory response, and alleviate liver fibrosis, with good safety.

7.
Medicina (B.Aires) ; 84(1): 168-170, 2024. graf
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1558464

RESUMO

Resumen La colangitis aguda es una infección de la vía biliar, asociada a la obstrucción de esta. El cultivo de la bilis es positivo en la mayoría de los casos y el agente etio lógico más frecuente es Escherichia coli. La colangitis aguda por Candida sp es un hallazgo poco común, que es más frecuente en pacientes con inmunocompromiso, uso de corticoides, tratamiento antibiótico prolongado o procedimientos quirúrgicos de la vía biliar. Presenta mos el caso de una mujer de 67 años, que no presen taba ninguno de los antecedentes mencionados, y que consultó por fiebre, dolor abdominal e ictericia. En la resonancia magnética nuclear de abdomen se constató imagen litiásica en el colédoco con dilatación de la vía biliar. Requirió drenaje endoscópico del tracto biliar. En el examen microscópico directo del líquido biliar se evidenciaron levaduras y bacilos Gram negativos, y en el cultivo se aisló Klebsiella pneumoniae productora de betalactamasa de espectro extendido (BLEE) y Candida glabrata. La paciente completó el tratamiento antibiótico con piperacilina tazobactam y anidulafungina con buena evolución. La infección de la vía biliar por la asociación de bacilos Gram negativos y Candida sp es una entidad poco frecuente, más en pacientes sin enfermedades subyacentes.


Abstract Acute cholangitis is a bile duct infection associated with bile duct obstruction. Bile culture is positive in most cases, and the most frequent etiological agent is Escherichia coli. Candida sp acute cholangitis is a rare finding, which is more common in patients with im munosuppression, use of corticosteroids, prolonged antibiotic treatment or surgical procedures of the bile duct. We present the case of a 67-year-old woman with none of the above-mentioned history who consulted for fever, abdominal pain and jaundice. MRI of the ab domen revealed a lithiasic image in the common bile duct with dilation. It required endoscopic drainage of the biliary tract. Direct microscopic examination of the bile fluid revealed gram-negative bacilli and yeast, and in the culture of bile fluid Klebsiella pneumoniae produc ing extended spectrum beta-lactamase (ESBL) and Can dida glabrata were isolated. The patient completed the antibiotic treatment with piperacillin tazobactam and anidulafungin with good evolution. Bile duct infection by association of Gram-negative bacilli and Candida sp is a rare entity, more in patients without underlying diseases.

8.
ABCD arq. bras. cir. dig ; 37: e1795, 2024. graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1549972

RESUMO

ABSTRACT BACKGROUND: Bile duct injury (BDI) causes significant sequelae for the patient in terms of morbidity, mortality, and long-term quality of life, and should be managed in centers with expertise. Anatomical variants may contribute to a higher risk of BDI during cholecystectomy. AIMS: To report a case of bile duct injury in a patient with situs inversus totalis. METHODS: A 42-year-old female patient with a previous history of situs inversus totalis and a BDI was initially operated on simultaneously to the lesion ten years ago by a non-specialized surgeon. She was referred to a specialized center due to recurrent episodes of cholangitis and a cholestatic laboratory pattern. Cholangioresonance revealed a severe anastomotic stricture. Due to her young age and recurrent cholangitis, she was submitted to a redo hepaticojejunostomy with the Hepp-Couinaud technique. To the best of our knowledge, this is the first report of BDI repair in a patient with situs inversus totalis. RESULTS: The previous hepaticojejunostomy was undone and remade with the Hepp-Couinaud technique high in the hilar plate with a wide opening in the hepatic confluence of the bile ducts towards the left hepatic duct. The previous Roux limb was maintained. Postoperative recovery was uneventful, the drain was removed on the seventh post-operative day, and the patient is now asymptomatic, with normal bilirubin and canalicular enzymes, and no further episodes of cholestasis or cholangitis. CONCLUSIONS: Anatomical variants may increase the difficulty of both cholecystectomy and BDI repair. BDI repair should be performed in a specialized center by formal hepato-pancreato-biliary surgeons to assure a safe perioperative management and a good long-term outcome.


RESUMO RACIONAL: As lesões de via biliar (LVB) impõem sequelas significativas ao paciente em termos de morbidade, mortalidade e qualidade de vida a longo prazo, devendo ser manejadas em centros especializados. Variantes anatômicas podem contribuir para um maior risco de LVB durante colecistectomia. OBJETIVOS: Relatar paciente com lesão de via biliar associado a situs inversus totalis. MÉTODOS: Paciente do sexo feminino, 42 anos, com histórico prévio de situs inversus totalis e LVB inicialmente reparada simultaneamente à lesão, há 10 anos, por um cirurgião não especializado. Ela foi encaminhada a um centro especializado devido a episódios recorrentes de colangite e um padrão laboratorial colestático. Colangiressonância revelou uma grave estenose anastomótica. Devido à sua idade jovem e colangites recorrentes, foi submetida a uma revisão cirúrgica da hepaticojejunostomia com técnica de Hepp-Couinaud. Até onde sabemos, este é o primeiro relato de reparo de LVB em um paciente com situs inversus totalis. RESULTADOS: A hepaticojejunostomia realizado prèviamente foi desfeita e refeita empregando a técnica de Hepp-Couinaud, alta na placa hilar, com uma ampla abertura na confluência dos ductos biliares em direção ao ducto hepático esquerdo. A alça de roux anterior foi mantida. A recuperação pós-operatória transcorreu sem intercorrências, o dreno foi removido no sétimo dia pós-operatório, e a paciente está agora assintomática, com bilirrubina e enzimas canalículares normais, e sem mais episódios de colestase ou colangite. CONCLUSÕES: Variantes anatômicas podem aumentar a dificuldade tanto da colecistectomia quanto do reparo de LVB, o qual deve ser realizado em um centro especializado por cirurgiões hepatobiliares para garantir um manejo perioperatório seguro e um bom resultado a longo prazo.

9.
Rev. am. med. respir ; 23(3): 183-186, dic. 2023. graf
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1559206

RESUMO

RESUMEN Durante el transcurso de la colangitis biliar primaria se puede desarrollar compromiso intersticial pulmonar: neumonía organizada, fibrosis intersticial, neumonía intersticial linfoide, neumonía intersticial no específica. A pesar de que el diagnóstico de colangitis biliar primaria usualmente precede a las manifestaciones pulmonares, puede ocurrir lo inverso. La frecuencia de enfermedad intersticial en pacientes con colangitis biliar primaria no es conocida con exactitud. Puede estar o no asociada a otras enfermeda des del tejido conectivo; por lo tanto, es necesario realizar una búsqueda sistemática de estas y de las manifestaciones pulmonares de dicha entidad. Presentamos el caso de una paciente con diagnóstico previo de colangitis biliar primaria, la cual desarrolla durante el curso de su enfermedad, afectación pulmonar intersticial.


ABSTRACT During the course of PBC, interstitial lung involvement may develop: organizing pneu monia, interstitial fibrosis, lymphoid interstitial pneumonia, or non-specific interstitial pneumonia. Although the diagnosis of PBC usually precedes pulmonary manifestations, the opposite can occur. The frequency of interstitial disease in patients with PBC is not exactly known. It may or may not be associated with other connective tissue diseases; therefore, it is necessary to carry out a systematic search of these diseases and the pulmonary manifestations of this entity. We present the case of a patient with a previ ous diagnosis of PBC, who developed interstitial lung involvement during the course of the disease.

10.
Radiol. bras ; 56(6): 301-307, Nov.-Dec. 2023. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1535050

RESUMO

Abstract Objective: To evaluate the usefulness of Anali scores, determined by magnetic resonance imaging, for predicting the prognosis of primary sclerosing cholangitis (PSC) and to analyze interobserver variability, as well as to assess the impact of periportal edema and heterogeneous signal intensity on diffusion-weighted imaging of the liver. Materials and Methods: This was a retrospective cohort study of 29 patients with PSC and baseline magnetic resonance imaging. Anali scores, without gadolinium (0-5 points) and with gadolinium (0-2 points), were calculated by two radiologists. Clinical end-points included liver transplantation, cirrhotic decompensation, and death. We calculated intraclass correlation coefficients (ICCs) for interobserver agreement on the Anali scores, performed Kaplan-Meier survival analysis comparing event-free survival among the score strata, and calculated the areas under receiver operating characteristic curves to determine sensitivity and specificity. Results: Among the patients with a clinical event, the median Anali score was 4 (interquartile range [IQR], 2-5) without gadolinium and 2 (IQR, 1-2) with gadolinium, compared with 1 (IQR, 1.0-2.5) and 1 (IQR, 0.25-1.0), respectively, among those without a clinical event. The ICC was 0.79 (95% confidence interval: 0.57-0.91) for the Anali score with gadolinium and 0.99 (95% confidence interval: 0.98-0.99) for the Anali score without gadolinium. Periportal edema and heterogeneous signal intensity in the liver on diffusion-weighted imaging showed no statistical impact on clinical events (p = 0.65 and p = 0.5, respectively). Conclusion: Anali scores correlate with clinical events in PSC, with a high level of interobserver agreement.


Resumo Objetivo: Avaliar a utilidade dos escores Anali determinados por ressonância magnética para prever o prognóstico da colangite esclerosante primária (CEP), analisar a variabilidade interobservador e avaliar o impacto do edema periportal e do sinal heterogêneo do fígado em imagens ponderadas por difusão. Materiais e Métodos: Estudo retrospectivo de coorte de 29 pacientes com CEP e ressonância magnética de base. Os escores Anali sem gadolínio (0 a 5 pontos) e com gadolínio (0 a 2 pontos) foram calculados por dois radiologistas. Os desfechos clínicos incluíram transplante de fígado, descompensação cirrótica ou morte. Foram realizados coeficiente de correlação intraclasse (CCI) para a concordância interobservador com relação ao escore Anali, análise de sobrevivência de Kaplan-Meier comparando o tempo livre de eventos de acordo com o escore, e área sob a curva característica de operação do receptor para sensibilidade e especificidade. Resultados: Nos pacientes com evento clínico, a mediana do escore Anali sem gadolínio foi 4 (intervalo interquartil [IIQ]: 2-5) e com gadolínio foi 2 (IIQ: 1-2), enquanto nos pacientes sem evento clínico o escore sem gadolínio foi 1 (IIQ:1-2,5) e com gadolínio foi 1 (IIQ: 0,25-1). A concordância interobservador com gadolínio foi CCI = 0,79 (intervalo de confiança 95%: 0,57-0,91) e sem gadolínio foi CCI = 0,99 (intervalo de confiança 95%: 0,98-0,99). O edema periportal (p = 0,65) e o sinal heterogêneo do fígado nas imagens ponderadas por difusão (p = 0,5) não apresentaram impacto nos eventos clínicos. Conclusão: Os escores Anali se correlacionam com eventos clínicos na CEP, com alto grau de concordância interobservador.

11.
Rev. colomb. gastroenterol ; 38(3)sept. 2023.
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1535925

RESUMO

Sjögren's syndrome is a systemic autoimmune disease characterized by dry eyes and mouth due to the involvement of exocrine glands. However, it can manifest with GI symptoms that cover a broad spectrum from esophageal and intestinal dysmotility, achalasia, hypochlorhydria, and chronic atrophic gastritis to pancreatic enzyme deficiency, biliary dysfunction, and liver cirrhosis, which varies in its clinical manifestations and is often associated with erroneous approaches. This article reviews the GI manifestations of Sjögren's syndrome. It presents the case of a woman in her eighth decade of life with this syndrome. She showed asymptomatic hepatobiliary disease, documented abnormalities in liver profile tests, and a subsequent diagnosis of primary sclerosing cholangitis, for which she received initial treatment with ursodeoxycholic acid. During her condition, the patient has had three episodes of cholangitis, requiring endoscopic retrograde cholangiopancreatography with no findings of stones, with scant biliary sludge and discharge of purulent bile precipitated by her underlying liver disease. The association between Sjögren's syndrome and primary sclerosing cholangitis is rare and calls for special consideration.


El síndrome de Sjögren es una enfermedad autoinmune sistémica que se caracteriza por la sequedad ocular y bucal debido a la afección de glándulas exocrinas; sin embargo, puede manifestarse con síntomas gastrointestinales que abarcan un espectro amplio desde la dismotilidad esofágica e intestinal, acalasia, hipoclorhidria y gastritis crónica atrófica hasta enzimodeficiencia pancreática, disfunción biliar y cirrosis hepática, que tiene variación en sus manifestaciones clínicas y se asocia con abordajes erróneos en muchas ocasiones. En este artículo se hace una revisión acerca de las manifestaciones gastrointestinales de síndrome de Sjögren y se presenta el caso de una mujer en la octava década de la vida con este síndrome, que cursa con enfermedad hepatobiliar asintomática, documentación de alteración en pruebas de perfil hepático y diagnóstico ulterior de colangitis esclerosante primaria, por lo que recibió un tratamiento inicial con ácido ursodesoxicólico. Durante el curso de su enfermedad ha presentado 3 episodios de colangitis, con requerimiento de colangiopancreatografía retrógrada endoscópica sin hallazgos de cálculos, con escaso barro biliar y salida de bilis purulenta, precipitada por su enfermedad hepática de base. La asociación entre el síndrome de Sjögren y la colangitis esclerosante primaria es infrecuente y justifica una consideración especial.

12.
Rev. colomb. gastroenterol ; 38(3)sept. 2023.
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1535931

RESUMO

Gallstone ileus manifests as intestinal obstruction. It occurs due to the passage of a stone and its subsequent lodging in the lumen of the digestive tract. The diagnosis is confirmed by imaging; the gold standard is abdominal tomography. Management is based on the extraction of the intraluminal calculus in one or more surgical times, depending on the patient's condition. We present the case of a patient with multiple comorbidities who showed a picture of cholangitis complicated by gallstone ileus and successfully treated with enterolithotomy. Surgical management is controversial since the optimal approach for these patients has not been established.


El íleo biliar se manifiesta como una obstrucción intestinal, se presenta por el paso de un lito y su posterior alojamiento en el lumen del tubo digestivo. El diagnóstico se confirma mediante imagenología, el patrón de oro es la tomografía abdominal. El manejo se fundamenta en la extracción del cálculo intraluminal en uno o más tiempos quirúrgicos, según el estado del paciente. Se presenta el caso de un paciente con múltiples comorbilidades, que debuta con un cuadro de colangitis complicada por íleo biliar, tratado exitosamente con enterolitotomía. El manejo quirúrgico es controversial, ya que no se ha establecido el abordaje óptimo para estos pacientes.

13.
ARS med. (Santiago, En línea) ; 48(3): 5-11, 30 sept. 2023.
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1510854

RESUMO

Introducción: el colangiocarcinoma intrahepático es un cáncer agresivo de células epiteliales de los conductos biliares intrahepáticos y su desarrollo se asocia a inflamación crónica del árbol biliar. En Chile, su epidemiología es limitada y el presente estudio tiene por objetivo describir su tasa de mortalidad. Métodos: se realizó un estudio descriptivo observacional transversal y ecológico de las defunciones por carcinoma de vías biliares en Chile durante 2017 y 2021 según sexo, grupo etario y región de residencia. Resultados: la tasa de mortalidad nacional de personas mayores a 20 años durante el periodo estudiado fue de 1,56 por cada 100.000 habitantes. La tasa de mortalidad más alta del sexo masculino se observó en 2020, siendo de 2,61. La mayor mortalidad se encontró en personas mayores a 80 años en el sexo masculino con una tasa de 24,38. A nivel regional, en Magallanes se observó la mayor tasa de mortalidad con 5,66, mientras que Tarapacá presentó la menor tasa con un valor de 0,96. Finalmente, el índice de Swaroop fue igual o mayor al 92% en todas las regiones del país. Conclusión: la mayor mortalidad por colangiocarcinoma intrahepático se presenta en personas de edad avanzada y de sexo masculino. Interesantemente la mayor mortalidad por esta causa se concentra en la zona sur de Chile. Dada la magnitud del problema que representa esta enfermedad en la salud pública nacional es que futuros estudios son necesarios para establecer medidas de prevención y/o tratamiento de esta enfermedad.


Introduction: intrahepatic cholangiocarcinoma is an aggressive cancer of epithelial cells of the intrahepatic bile ducts, and its deve-lopment is associated with chronic inflammation of the biliary tree. In Chile, its epidemiology is limited, and the present study aims to describe its mortality rate. Methods: a descriptive, cross-sectional, observational, and ecological study of deaths from bile duct carcinoma in Chile between 2017 and 2021 was performed according to sex, age group, and region of residence. Results: the national mortality rate of people over 20 years old during the study period was 1.56 per 100,000 inhabitants. The highest mortality rate for the male sex was observed in 2020, with a value of 2.61. In turn, the highest mortality rate was found in people over 80 years old in the male sex, with a rate value of 24.38. On a regional level, Magallanes had the highest mortality rate, with a rate value of 5.66, while Tarapacá had the lowest rate, with a value of 0.96. Finally, Swaroop's index was equal to or greater than 92% in all regions of the country. Conclusion: the highest mortality from intrahepatic cholangiocarcinoma occurs in older people and males. Interestingly, the highest mortality from this cause is concentrated in the southern zone of Chile. Given the magnitude of the problem that this disease represents for national public health, future studies are necessary to establish both prevention measures and treatments

14.
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1535912

RESUMO

Introduction: Post-COVID-19 cholangiopathy is a novel condition characterized by biliary tract sclerosis and elevated alkaline phosphatase levels in critically ill patients. This case series aims to describe the experience of a Latin American reference hospital in managing this condition. Methods: This case series includes patients with confirmed coronavirus disease 2019 (COVID-19) who exhibited subsequent elevation of alkaline phosphatase levels exceeding three times the normal value. The patients also had documented bile duct abnormalities observed through cholangioresonance or endoscopic retrograde cholangiopancreatography (ERCP). The clinical presentation, imaging findings, complications, and treatment approaches are described. Results: Eight patients (56.5 ± 9.2 years old, 62.5% male) were included in the study. All patients had previously experienced severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) pneumonia and required mechanical ventilation. Four patients (50%) received sedoanalgesia with ketamine, and all eight patients (100%) received propofol. All patients developed infections, such as cholangitis or liver abscesses, caused by gram-negative bacteria. The peak alkaline phosphatase level during follow-up averaged 1646.12 ± 611.3. Imaging findings revealed intrahepatic (100%) and extrahepatic (87.5%) bile duct dilation. In 75% of cases, bile molds with a black appearance were extracted. Seven patients experienced recurrent cholangitis, and three patients were referred for pre-liver transplant consultation. Conclusions: Post-COVID-19 cholangiopathy is characterized by severe cholestasis, intra- and extrahepatic bile duct dilation, formation of bile molds, and recurrent cholangitis. In our study, a possible association between sepsis caused by gram-negative bacteria and the use of sedative medications is hypothesized. Further studies are necessary to establish the most appropriate management strategies for these patients, as they currently face unfavorable long-term morbidity and mortality outcomes.


Introducción: la colangiopatía pos-COVID-19 es una entidad nueva caracterizada por esclerosis del tracto biliar y elevación de fosfatasa alcalina en el paciente críticamente enfermo. Esta serie de casos describe la experiencia de un hospital de referencia en Latinoamérica. Métodos: serie de casos que incluye a pacientes con enfermedad por coronavirus de 2019 (COVID-19) confirmada y elevación posterior de fosfatasa alcalina > 3 veces del valor normal, asociados a anormalidades en las vías biliares documentada por colangiorresonancia o colangiopancreatografía retrógrada endoscópica (CPRE). Se describe la presentación clínica, hallazgos imagenológicos, complicaciones y tratamiento. Resultados: se incluyeron a 8 pacientes (56,5 ± 9,2 años, 62,5% hombres). Todos presentaron neumonía por coronavirus del síndrome respiratorio agudo grave de tipo 2 (SARS-CoV-2) previo a desarrollo de colangiopatía, y requirieron ventilación mecánica. Cuatro pacientes (50%) recibieron sedoanalgesia con ketamina y ocho (100%) con propofol. Todos presentaron infecciones (colangitis/abscesos hepáticos) por bacterias gramnegativas. El nivel pico de fosfatasa alcalina durante el seguimiento fue en promedio 1646,12 ± 611,3. Los hallazgos imagenológicos incluyeron dilatación de la vía biliar intrahepática (100%) y extrahepática (87,5%). En el 75% se extrajo el molde biliar de apariencia negra. Siete pacientes presentaron recurrencia de colangitis y tres pacientes fueron referidos a consulta de pretrasplante hepático. Conclusiones: la colangiopatía pos-COVID-19 está caracterizada por colestasis grave con dilatación de la vía biliar intra- y extrahepática, formación de moldes biliares y colangitis recurrente. En nuestro estudio se plantea como hipótesis una posible relación con sepsis por bacterias gramnegativas y uso de medicamentos sedativos. Se requieren nuevos estudios para establecer el manejo más adecuado para estos pacientes, que hasta el momento presentan una morbimortalidad desfavorable a largo plazo.

15.
Medwave ; 23(3): e2663, 28-04-2023.
Artigo em Inglês, Espanhol | LILACS-Express | LILACS | ID: biblio-1436097

RESUMO

Introducción El síndrome de Guillain-Barré es una polirradiculoneuropatia de origen autoinmune, considerada la causa más frecuente de parálisis flácida aguda. Se han reportado diversas asociaciones del síndrome de Guillain-Barré con otras enfermedades autoinmunes no neurológicas, algunas de ellas extremadamente raras, como la que ocurre con la colangitis biliar primaria, una enfermedad crónica de etiología autoinmune cuyo diagnóstico se sustenta, además del cuadro clínico, en la alteración de las enzimas hepáticas y la presencia de anticuerpos anti-mitocondriales. Caso clínico Paciente varón de 38 años, sin antecedente de comorbilidades previas, quien luego de presentar enfermedad diarreica dos semanas antes, desarrolló debilidad ascendente de inicio subagudo asociado a parestesias en cuatro extremidades que progresó hasta generar cuadriplejia y dificultad respiratoria. Se le realizó examen citoquímico de líquido cefalorraquídeo que evidenció disociación albumino-citológica y electromiografía que mostró hallazgos compatibles con neuropatía axonal motora aguda. Recibió tratamiento con inmunoglobulina intravenosa a dosis de 0,4 gramos por kilogramo al día, logrando mejoría del cuadro neurológico. Desde su ingreso y durante la hospitalización, presentó alteración persistente de las enzimas hepáticas que seguía un patrón colestásico. Además, se agregó dolor abdominal de leve intensidad y prurito generalizado, por lo cual fue evaluado por gastroenterología, quienes solicitaron anticuerpos anti-mitocondriales que resultaron positivos. Con esta prueba, se comprobó el diagnóstico de colangitis biliar primaria. Conclusión El presente caso muestra una asociación extremadamente rara de dos enfermedades autoinmunes; síndrome de Guillain-Barré y colangitis biliar primaria, tanto así que representa el primer caso reportado, no vinculado a SARS-CoV-2.


Introduction Guillain-Barré syndrome is a polyradiculoneuropathy of autoimmune origin, considered the most frequent cause of acute flaccid paralysis. Various associations of Guillain-Barré syndrome with other non-neurological autoimmune diseases have been reported, some of them extremely rare, such as that which occurs with primary biliary cholangitis, a chronic disease of autoimmune etiology whose diagnosis is also supported by the clinical picture. , in the alteration of liver enzymes and the presence of anti-mitochondrial antibodies. Clinical case A 38-year-old male patient, with no history of previous comorbidities, who, after presenting with diarrheal disease two weeks prior, developed subacute onset ascending weakness associated with paresthesias in four extremities that progressed to quadriplegia and respiratory distress. Cerebrospinal fluid cytochemistry was performed, which showed albuminocytological dissociation and electromyography, which showed findings compatible with acute motor axonal neuropathy, for which he received treatment with intravenous immunoglobulin at 0.4g/kg/day, achieving improvement in the neurological condition. Since admission and during hospitalization, he presented persistent changes in liver enzymes which followed a cholestatic pattern, in addition to mild abdominal pain and generalized itching, for which he was evaluated by gastroenterology, who requested anti-mitochondrial antibodies that were positive. Concluding in the diagnosis of primary biliary cholangitis. Conclusion The present case shows an extremely rare association of two autoimmune diseases Guillain-Barré syndrome and primary biliary cholangitis, so much so that it represents the first case reported, not linked to SARS-CoV-2.

16.
Journal of Clinical Hepatology ; (12): 463-468, 2023.
Artigo em Chinês | WPRIM | ID: wpr-964816

RESUMO

Immune checkpoint inhibitors (ICIs) have ushered in a new era of tumor treatment; however, immunotherapy-related adverse events are critical issues that restrict the clinical application of ICIs and have attracted wide attention. The liver is one of the target organs that is easily affected. With the progress in research, scholars have found that besides hepatocytes, intrahepatic and extrahepatic bile ducts can also be attacked by the immune system, leading to the disease known as immune-related cholangitis. This article reviews the research advances in ICI-related cholangitis by summarizing related articles, in order to preliminarily reveal its clinical, pathological, and imaging features and provide clues for early identification, standard treatment, and subsequent research.

17.
Journal of Clinical Hepatology ; (12): 325-332, 2023.
Artigo em Chinês | WPRIM | ID: wpr-964791

RESUMO

Objective To systematically review the epidemiological studies on primary biliary cholangitis (PBC), and to investigate the prevalence rate of PBC in the Chinese general population and its influencing factors. Methods PubMed, Embase, The Cochrane Library, CNKI, and Wanfang Data were searched for articles on the epidemiology of PBC in China published up to 31th March 2022. Two researchers independently performed screening and data extraction, and then related analyses were performed. Results A total of 9 articles were included. The positive rate of AMA was 1 049.05/100 000 (ranging fr om 159.65/100 000 to 2287.40/100 000), and the prevalence rate of PBC was 123.68/100 000 (ranging from 42.70/100 000 to 276.59/100 000). The positive rate of AMA was 636.51/100 000 (ranging from 52.55/100 000 to 1 164.33/100 000) in men and 1 265.47/100 000 (ranging from 225.23/100 000 to 1 704.93/100 000) in women, with a male/female ratio of 1∶1.99 for the prevalence rate of AMA. The prevalence rate of PBC was 40.81/100 000 (ranging from 23.54/100 000 to 75.10/100 000) in men and 148.71/100 000 (ranging from 77.36/100 000 to 214.91/100 000) in women, with a male/female ratio of 1∶3.64 for the prevalence rate of PBC. Conclusion Different studies show great differences in the positive rate of AMA and the prevalence rate of PBC in the Chinese general population, which is mainly affected by sex, age, and region. The positive rate of AMA and the prevalence rate of PBC increase with age, and the patients aged ≥50 years have a significantly higher positive rate of AMA than those aged < 50 years. The positive rate of AMA is significantly higher than the prevalence rate of PBC. There are significantly more women than men in the AMA-positive population and the PBC patients, and the influence of sex on AMA is lower than that on PBC.

18.
Journal of Clinical Hepatology ; (12): 43-49, 2023.
Artigo em Chinês | WPRIM | ID: wpr-960703

RESUMO

In 2022, the European Association for the Study of the Liver issued Clinical practice guidelines on sclerosing cholangitis. With reference to the 2017 edition of Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline (2017) and in comparison to the corresponding contents in Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021) issued by Chinese Society of Hepatology, Chinese Medical Association, in 2021, this article summarizes the updates in diagnosis, treatment, monitoring, and management of special populations and analyzes the basis for updated recommendations and their guiding significance in optimizing the clinical management of primary sclerosing cholangitis (PSC). The comparative analysis shows that the new version of the guidelines is similar to the Chinese guidelines in terms of diagnosis, treatment, and follow-up, and it is worth learning from the technical details such as the recommended dose of ursodeoxycholic acid and long-term follow-up plan. Since PSC is a chronic refractory disease, the drugs recommended by current guidelines cannot delay or reverse disease progression, and there is still a lack of consensus statements on immunotherapy and screening protocols for end-stage complications, which might be the directions for further research.

19.
Chinese Journal of Hepatology ; (12): 40-46, 2023.
Artigo em Chinês | WPRIM | ID: wpr-986789

RESUMO

Objective: To clarify the values of autotaxin (ATX) in patients with primary biliary cholangitis (PBC) and PBC-related hepatocellular carcinoma (HCC). Methods: 179 patients with PBC were selected from prospective cohorts of autoimmune liver diseases at the time of first diagnosis of PBC in Department of Hepatology, the Fifth Medical Center of PLA General Hospital, from January 2016 to January 2018, all patients with PBC received UDCA therapy, primary endpoint was event of HCC, the follow-up period was censored at the date of HCC. The relationship between level of ATX and clinical features in patients with PBC and its potential value in predicting disease progression and PBC-related HCC were analyzed. Results: The ATX level in the peripheral blood of patients with PBC was significantly higher than that of alcoholic liver cirrhosis(ALC) (t = 3.278, P = 0.001) and healthy controls(HC) (t = 6.594, P < 0.001), however, when comparing PBC to non-PBC related HCC, no significant difference was found between the groups(t=-0.240, P = 0.811). Consistent with peripheral blood levels, histochemical staining indicated that ATX in the liver of patients with PBC was significantly higher than that of HC (Z=-3.633, P < 0.001) and ALC (Z=-3.283, P < 0.001), and the expression of ATX in PBC with advanced histological stage was significantly higher than PBC with early stage (Z=-2.018, P = 0.034). The baseline ATX level in PBC patients without developing to HCC during follow-up had significant difference to patients with developing to HCC (228.451 ± 124.093 ng/ml vs 301.583 ± 100.512 ng/ml, t = 2.339, P = 0.021). The result in multivariate logistic regression analysis showed that ATX were independent predictors of PBC related HCC(OR 1.245, 95%CI 1.097-1.413). The optimal critical value of peripheral blood ATX level at baseline for predicting HCC was 235.254 ng/ml, with the cut-off value of 0.714 in AUC of the ROC (95% CI was 0.597~ 0.857), sensitivity and specificity were 84.6% and 59.0%, respectively. Conclusion: ATX level was significantly higher in PBC patients over controls, and it's concentration was correlated with UDCA efficacy and fibrosis stage. ATX has potential values in predicting disease progression and PBC-related HCC.

20.
Chinese Journal of Hepatology ; (12): 705-709, 2023.
Artigo em Chinês | WPRIM | ID: wpr-986198

RESUMO

Objective: To investigate the clinical features and long-term prognosis of primary biliary cholangitis (PBC) in patients with past hepatitis B virus (HBV) infection. Methods: 353 cases with PBC who visited the Liver Disease Center of Beijing Friendship Hospital Affiliated to Capital Medical University between January 2000 and January 2018 were retrospectively analyzed and were divided into the past HBV infection group (156 cases) and the no HBV infection group (197 cases). The two groups' baseline clinical features were compared. Ursodeoxycholic acid response rate after one year, GLOBE score, UK-PBC score, and long-term liver transplantation-free survival rate were compared through outpatient and telephone follow-up. Results: PBC with past HBV infection had a significantly reduced female proportion compared to the no HBV infection group (91.9% vs. 79.5%, P = 0.001). However, there were no statistically significant differences in age, biochemical indices, immunological indicators, platelet count, cirrhosis proportion, and others. Ursodeoxycholic acid biochemical response rate was reduced in patients with past HBV infection at the end of one year of treatment, but the difference was not statistically significant (65.8% vs. 78.2%, P = 0.068). In addition, there were no statistically significant differences between the GLOBE score (0.57 vs. 0.59, P = 0.26) and UK-PBC 5-year (2.87% vs. 2.87%, P = 0.38), 10-year (9.29% vs. 8.2%, P = 0.39) and 15-year liver transplantation rates (16.6% vs. 14.73%, P = 0.39). Lastly, the overall 5-year liver transplantation-free survival rate had no statistically significant difference between the two groups of patients (86.4% vs. 87.5%, P = 0.796). Conclusion: Primary biliary cholangitis had no discernible effect in terms of age at onset, biochemical indices, immunological indicators, cirrhosis proportion, ursodeoxycholic acid response rate after one year, GLOBE score, UK-PBC score, or overall liver transplantation-free survival rate in patients with past hepatitis B virus infections.

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