Luis Guillermo Toro Rendón, Luisa Fernanda Calle Tavera, Elizabeth Gutiérrez, Adriana Ocampo Mesa, J. Arbeláez
Introduction and objective. The deficiency of the Lysosomal acid lipase (LAL) activity has been related to cirrhosis due to non-alcoholic steatohepatitis (NASH). Many of the cirrhosis classified as cryptogenic are the evolution of liver disease due to fatty liver. The objective of the present study was to evaluate the lysosomal acid lipase (LAL) activity in the patients with liver cirrhosis of any etiology and establish whether low levels correlate with cryptogenic cirrhosis of origin cryptogenic or NASH. Methods. Was an analytical cohort study including 96 patients with cirrhosis of any etiology for which LAL activity was measured. Results. Fifty-five patients (58%) with cryptogenic cirrhosis or NASH were included and 41 with other etiologies. The fifty-three percent of the total population were women. The severity scores of liver disease were significantly higher in the patients with the cryptogenic cirrhosis or NASH: MELD (11.72 ± 7.3 vs 4,34 ± 5.7; p = 0.001) and Child-Pugh (7.26 ± 3.8 vs 7.26 ± 3.8; p = 0.004). LAL activity was significant lower (202.40 ± 98.8 vs 242.55 ± 121.9; p = 0.04) in the cirrhosis cryptogenic or NASH group. In the multivariate analysis, low LAL activity (< 150 nmol/spot/hour), was correlated with the presence of cryptogenic cirrhosis or NASH. Conclusions. The patients with cryptogenic cirrhosis or NASH have lower levels of LAL activity than those with cirrhosis of other etiologies. LAL activity below 150 nmol/spot/hour is predictive of the cryptogenic cirrhosis or NASH.
引言和目标。溶酶体酸性脂肪酶（LAL）活性的缺乏与非酒精性脂肪性肝炎（NASH）引起的肝硬化有关。许多被归类为隐性肝硬化的肝硬化是由脂肪肝引起的肝病的演变。本研究的目的是评估任何病因的肝硬化患者的溶酶体酸性脂肪酶（LAL）活性，并确定低水平是否与来源于隐性或NASH的隐性肝硬化相关。方法。是一项分析性队列研究，包括96名任何病因的肝硬化患者，对其LAL活性进行了测量。后果包括55名（58%）隐源性肝硬化或NASH患者和41名其他病因患者。妇女占总人口的53%。隐源性肝硬化或NASH患者的肝脏疾病严重程度评分显著较高：MELD（11.72±7.3 vs 4,34±5.7；p=0.001）和Child-Pugh（7.26±3.8 vs 7.26±38；p=0.004）。肝硬化隐源性或NASH组的LAL活性显著较低（202.40±98.8 vs 242.55±121.9；p=0.04）。在多变量分析中，低LAL活性（<150 nmol/点/小时）与隐性肝硬化或NASH的存在相关。结论。隐源性肝硬化或NASH患者的LAL活性水平低于其他病因的肝硬化患者。LAL活性低于150nmol/spot/hour可预测隐性肝硬化或NASH。
{"title":"Bajos niveles de actividad de la lipasa ácida lisosomal y su relación con el desarrollo de la cirrosis de origen criptogénica/NASH: un estudio de cohorte","authors":"Luis Guillermo Toro Rendón, Luisa Fernanda Calle Tavera, Elizabeth Gutiérrez, Adriana Ocampo Mesa, J. Arbeláez","doi":"10.52787/ZHJU9301","DOIUrl":"https://doi.org/10.52787/ZHJU9301","url":null,"abstract":"Introduction and objective. The deficiency of the Lysosomal acid lipase (LAL) activity has been related to cirrhosis due to non-alcoholic steatohepatitis (NASH). Many of the cirrhosis classified as cryptogenic are the evolution of liver disease due to fatty liver. The objective of the present study was to evaluate the lysosomal acid lipase (LAL) activity in the patients with liver cirrhosis of any etiology and establish whether low levels correlate with cryptogenic cirrhosis of origin cryptogenic or NASH. Methods. Was an analytical cohort study including 96 patients with cirrhosis of any etiology for which LAL activity was measured. Results. Fifty-five patients (58%) with cryptogenic cirrhosis or NASH were included and 41 with other etiologies. The fifty-three percent of the total population were women. The severity scores of liver disease were significantly higher in the patients with the cryptogenic cirrhosis or NASH: MELD (11.72 ± 7.3 vs 4,34 ± 5.7; p = 0.001) and Child-Pugh (7.26 ± 3.8 vs 7.26 ± 3.8; p = 0.004). LAL activity was significant lower (202.40 ± 98.8 vs 242.55 ± 121.9; p = 0.04) in the cirrhosis cryptogenic or NASH group. In the multivariate analysis, low LAL activity (< 150 nmol/spot/hour), was correlated with the presence of cryptogenic cirrhosis or NASH. Conclusions. The patients with cryptogenic cirrhosis or NASH have lower levels of LAL activity than those with cirrhosis of other etiologies. LAL activity below 150 nmol/spot/hour is predictive of the cryptogenic cirrhosis or NASH.","PeriodicalId":35700,"journal":{"name":"Acta Gastroenterologica Latinoamericana","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47570353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Martín Elizondo Barceló, Jimena Prieto Amorín, J. C. M. Presentado, Solange Gerona Sangiovanni
El empiema bacteriano espontáneo se define como la infección del hidrotórax hepático en los pacientes cirróticos. Es una causa rara de descompensación en la cirrosis, existiendo escasos reportes bibliográficos en la región, y su presencia marca un punto de inflexión en la evolución de la enfermedad con un mal pronóstico a corto plazo. Se presentan dos casos clínicos de pacientes con esta complicación que fueron derivados al Programa Nacional de Trasplante Hepático de Uruguay. Se realiza la revisión del tema jerarquizando aspectos de la presentación clínica, la fisiopatología y un enfoque terapéutico. Se hace una especial mención a la importancia del tratamiento antibiótico empírico basado en el sitio de adquisición de la infección, la epidemiología local y en la oportunidad de la resolución del hidrotórax hepático subyacente, dado el alto riesgo de complicaciones que pueden asociar los tratamientos invasivos.
{"title":"Empiema bacteriano espontáneo como complicación de la cirrosis hepática: ¿cuándo sospecharlo y cómo tratarlo? Presentación de casos","authors":"Martín Elizondo Barceló, Jimena Prieto Amorín, J. C. M. Presentado, Solange Gerona Sangiovanni","doi":"10.52787/WLHQ8266","DOIUrl":"https://doi.org/10.52787/WLHQ8266","url":null,"abstract":"El empiema bacteriano espontáneo se define como la infección del hidrotórax hepático en los pacientes cirróticos. Es una causa rara de descompensación en la cirrosis, existiendo escasos reportes bibliográficos en la región, y su presencia marca un punto de inflexión en la evolución de la enfermedad con un mal pronóstico a corto plazo. Se presentan dos casos clínicos de pacientes con esta complicación que fueron derivados al Programa Nacional de Trasplante Hepático de Uruguay. Se realiza la revisión del tema jerarquizando aspectos de la presentación clínica, la fisiopatología y un enfoque terapéutico. Se hace una especial mención a la importancia del tratamiento antibiótico empírico basado en el sitio de adquisición de la infección, la epidemiología local y en la oportunidad de la resolución del hidrotórax hepático subyacente, dado el alto riesgo de complicaciones que pueden asociar los tratamientos invasivos.","PeriodicalId":35700,"journal":{"name":"Acta Gastroenterologica Latinoamericana","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46897602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
María del Mar Roldán Alcázar, A. M. Maldonado, A. S. Gutiérrez, C. Cejudo
{"title":"Solución del caso. Hallazgo radiológico infrecuente por una hiperpresión abdominal tras un traumatismo","authors":"María del Mar Roldán Alcázar, A. M. Maldonado, A. S. Gutiérrez, C. Cejudo","doi":"10.52787/IXWG2588","DOIUrl":"https://doi.org/10.52787/IXWG2588","url":null,"abstract":"","PeriodicalId":35700,"journal":{"name":"Acta Gastroenterologica Latinoamericana","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43475741","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
María Carmen Álvarez López, Pedro Coello Ramírez, Elizabeth García Rodríguez, Mariana Ordoñez Cárdenas, Fátima Azereth Reynoso Zarzosa
Background. Allgrove Syndrome is a very rare genetic disease, which is inherited in an autosomal recessive way. The responsible gene is the AAAS, that encodes the protein ALADIN. It occurs most often in children of consanguineous parents. It is characterized by the classic triad of achalasia, alacrima, and adrenal insufficiency due to resistance to ACTH; the presence of two of the three previous manifestation events are required to establish the diagnosis. There is also a high frequency of the neurologic symptoms. Objective. Describe the clinical characteristics, age of presentation and evolution in 11 patients with Allgrove Syndrome. Methods. 11 clinical cases compatible with Allgrove Syndrome of presentation in childhood are retrospectively reviewed. Results. The average age at diagnosis was 5.9 years (range 1-16 years old). There was a predominance of the female sex (n = 7). The most common symptoms were postprandial vomiting and alacrima, present in 100% of the cases at the time of diagnosis. Adrenal insufficiency was not common; it was only documented in one patient. There was consanguinity between parents in 62.5% of the cases. Conclusions. Allgrove Syndrome is an uncommon cause of dysphagia, chronic vomiting and failure to grow in children. In case of any documented case of achalasia it is suggested to question in a directed way the presence of alacrima and adrenal insufficiency data such as seizures, hyperpigmentation of the skin and neurological alterations.
{"title":"Sindrome de allgrove en niños. Reporte de 11 casos","authors":"María Carmen Álvarez López, Pedro Coello Ramírez, Elizabeth García Rodríguez, Mariana Ordoñez Cárdenas, Fátima Azereth Reynoso Zarzosa","doi":"10.52787/ieua6903","DOIUrl":"https://doi.org/10.52787/ieua6903","url":null,"abstract":"Background. Allgrove Syndrome is a very rare genetic disease, which is inherited in an autosomal recessive way. The responsible gene is the AAAS, that encodes the protein ALADIN. It occurs most often in children of consanguineous parents. It is characterized by the classic triad of achalasia, alacrima, and adrenal insufficiency due to resistance to ACTH; the presence of two of the three previous manifestation events are required to establish the diagnosis. There is also a high frequency of the neurologic symptoms. Objective. Describe the clinical characteristics, age of presentation and evolution in 11 patients with Allgrove Syndrome. Methods. 11 clinical cases compatible with Allgrove Syndrome of presentation in childhood are retrospectively reviewed. Results. The average age at diagnosis was 5.9 years (range 1-16 years old). There was a predominance of the female sex (n = 7). The most common symptoms were postprandial vomiting and alacrima, present in 100% of the cases at the time of diagnosis. Adrenal insufficiency was not common; it was only documented in one patient. There was consanguinity between parents in 62.5% of the cases. Conclusions. Allgrove Syndrome is an uncommon cause of dysphagia, chronic vomiting and failure to grow in children. In case of any documented case of achalasia it is suggested to question in a directed way the presence of alacrima and adrenal insufficiency data such as seizures, hyperpigmentation of the skin and neurological alterations.","PeriodicalId":35700,"journal":{"name":"Acta Gastroenterologica Latinoamericana","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71005010","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Juvenile polyposis syndrome is an autosomal-dominant condition disease characterized by multiple juvenile polyps in the gastrointestinal tract. These polyps may be present in the entire digestive tract; generally in the colon. We present the case of a 16-year-old adolescent with a history of polyps, who had had bowel resections for acute abdomen on two occasions (at 5 and 11 years of age). Endoscopic procedures were carried out, finding polyps and performing a polypectomy. After 24 hours, the patient presented proctorrhagia associated with acute abdominal pain by an obstruction, for which he was surgically intervened with an exploratory laparotomy where an intussusception was found.
{"title":"Abdomen agudo en un adolescente con poliposis juvenil. A propósito de un caso","authors":"Rubén Gustavo Muñoz Cedeño, Michelle Carolina Ricaurte Enríquez, Priscila Elizabeth Martínez Ballesteros, Viviana Paullán Saní, Gema Nathalye Rodríguez Chica","doi":"10.52787/uekp2937","DOIUrl":"https://doi.org/10.52787/uekp2937","url":null,"abstract":"Juvenile polyposis syndrome is an autosomal-dominant condition disease characterized by multiple juvenile polyps in the gastrointestinal tract. These polyps may be present in the entire digestive tract; generally in the colon. We present the case of a 16-year-old adolescent with a history of polyps, who had had bowel resections for acute abdomen on two occasions (at 5 and 11 years of age). Endoscopic procedures were carried out, finding polyps and performing a polypectomy. After 24 hours, the patient presented proctorrhagia associated with acute abdominal pain by an obstruction, for which he was surgically intervened with an exploratory laparotomy where an intussusception was found.","PeriodicalId":35700,"journal":{"name":"Acta Gastroenterologica Latinoamericana","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71004921","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A case is presented of a 48-year-old patient, with a 28-year history of Crohn’s disease, with chronic constipation with outlet obstruction symptoms due to the presence of a mucosal bridge at the low rectum. Endoscopic treatment was performed with section of the bridge, without any complications, and with immediate resolution of the symptoms.
{"title":"Causa infrecuente de trastorno evacuatorio por puente mucoso rectal en una paciente con enfermedad de Crohn","authors":"Lucía Secondo, Adrián Canavesi, Alejandra Arriola, Nicolás González","doi":"10.52787/HKUW6257","DOIUrl":"https://doi.org/10.52787/HKUW6257","url":null,"abstract":"A case is presented of a 48-year-old patient, with a 28-year history of Crohn’s disease, with chronic constipation with outlet obstruction symptoms due to the presence of a mucosal bridge at the low rectum. Endoscopic treatment was performed with section of the bridge, without any complications, and with immediate resolution of the symptoms.","PeriodicalId":35700,"journal":{"name":"Acta Gastroenterologica Latinoamericana","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2020-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43826902","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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