Neumatosis portal y gástrica secundaria a suboclusión intestinal. Éxito del manejo no quirúrgico / No disponible

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Portal venous gas and pneumatosis intestinalis secondary to intestinal subocclusion: success of non-surgical management.

The association of intestinal and portal pneumatosis in the same patient usually occurs in intestinal ischemic-necrotic processes, with ominous prognosis. However, there are forms of presentation outside this context, with a radically different management and evolution. We present the case of a patient with portal and gastric pneumatosis, managed conservatively successfully. The clinical presentation and a multidisciplinary management will be critical in the decision-making process to obtain favorable results.

Efficacy and safety of ERCP in a low-volume hospital.

BACKGROUND AND AIMS: there is little scientific evidence on the outcomes of endoscopic retrograde cholangiopancreatography (ERCP) performed in low-volume hospitals; however, in our country, it is growing up its implementation. The objectives of our study were to evaluate the efficacy and safety of this technique performed by two endoscopists with basic training in a center of this nature and analyze the learning curve in the first procedures. PATIENTS AND METHODS: single-center retrospective study of the first 200 ERCP performed in our hospital (analyzing the evolution between the first 100 and 100 following procedures), comparing them with the quality standards proposed in the literature. RESULTS: from February 2009 to April 2011, we performed 200 ERCP in 169 patients, and the most common indications were: Choledocholithiasis (77 %), tumors (14.5 %) and other conditions (8.5 %). The cannulation rate rose from 85 % in the first 100 ERCPto 89 % in the next 100 procedures, clinical success from 81 % to 87 %, decreasing the post-ERCP acute pancreatitis rate from 11 % to 4 %, upper gastrointestinal bleeding (UGIB) from 3 % to 2 % and acute cholangitis from 4 % to 1 %. There was a death from a massive UGIB in a cirrhotic patient in the first group of patients and a case of biliary perforation resolved by surgery in the second one. CONCLUSIONS: the results obtained after performing 200 procedures support the ability to practice ERCP in low-volume hospitals obtaining levels of efficacy and safety in accordance with published quality standards.

Eficacia y seguridad de la CPRE en un hospital con bajo volumen / Efficacy and safety of ERCP in a low-volume hospital

Introducción y objetivos: existe poca evidencia científica sobre los resultados de la CPRE realizada en hospitales con bajo volumen, sin embargo su puesta en marcha en nuestro medio es creciente. Los objetivos de nuestro estudio son evaluar la eficacia y seguridad de dicha técnica realizada por dos endoscopistas biliares noveles en un centro de estas características y analizar la curva de aprendizaje en los primeros procedimientos. Pacientes y métodos: estudio retrospectivo de las primeras 200 CPRE practicadas en nuestro hospital, analizando la progresión entre los 100 primeros procedimientos y los 100 segundos, comparándolos con los estándares de calidad propuestos en la literatura. Resultados: desde febrero de 2009 hasta abril de 2011 se realizaron 200 procedimientos a 169 pacientes con las siguientes indicaciones: coledocolitiasis (77 %), neoplasias (14,5 %) y otras patologías (8,5 %). La tasa de canulación ascendió del 85 % en las 100 primeras CPRE al 89 % en las siguientes, el éxito clínico del 81 % al 87 %, disminuyendo la tasa de pancreatitis aguda post-CPRE del 11 al 4 %, la de hemorragia digestiva alta del 3 al 2 % y la de colangitis aguda del 4 al 1 %. Hubo un éxitus secundario a una hemorragia digestiva alta en una paciente cirrótica en el primer grupo y un caso de perforación biliar resuelto mediante cirugía en el segundo. Conclusiones: los resultados obtenidos tras la realización de 200 procedimientos apoyan la posibilidad de practicar CPRE en hospitales con bajo volumen consiguiendo niveles de eficacia y seguridad acorde con los estándares de calidad publicados(AU)

Background and aims: there is little scientific evidence on the outcomes of endoscopic retrograde cholangiopancreatography (ERCP) performed in low-volume hospitals; however, in our country, it is growing up its implementation. The objectives of our study were to evaluate the efficacy and safety of this technique performed by two endoscopists with basic training in a center of this nature and analyze the learning curve in the first procedures. Patients and methods: single-center retrospective study of the first 200 ERCP performed in our hospital (analyzing the evolution between the first 100 and 100 following procedures), comparing them with the quality standards proposed in the literature. Results: from February 2009 to April 2011, we performed 200 ERCP in 169 patients, and the most common indications were: Choledocholithiasis (77 %), tumors (14.5 %) and other conditions (8.5 %). The cannulation rate rose from 85 % in the first 100 ERCP to 89 % in the next 100 procedures, clinical success from 81 % to 87 %, decreasing the post-ERCP acute pancreatitis rate from 11 % to 4 %, upper gastrointestinal bleeding (UGIB) from 3 % to 2 % and acute cholangitis from 4 % to 1 %. There was a death from a massive UGIB in a cirrhotic patient in the first group of patients and a case of biliary perforation resolved by surgery in the second one. Conclusions: the results obtained after performing 200 procedures support the ability to practice ERCP in low-volume hospitals obtaining levels of efficacy and safety in accordance with published quality standards(AU)

Sarcoma de Kaposi con afectación colorrectal y del canal anal / Kaposi's sarcoma with colorectal and anal canal involvement

El sarcoma de Kaposi es una neoplasia vascular de bajo grado, de la cual existen cuatro variantes fundamentales, una de las cuales se relaciona con el virus de la inmunodeficiencia humana. Sus manifestaciones más características son la presencia de lesiones mucocutáneas y la afectación de los ganglios linfáticos. El tracto gastrointestinal está implicado en el 40% de los casos, aunque la aparición de lesiones en el recto y en el canal anal es excepcional. Presentamos el caso de un varón de 39 años con virus de la inmunodeficiencia humana positivo, diagnosticado endoscópicamente de sarcoma de Kaposi con afectación colorrectal y del canal anal, y sin lesiones cutáneas asociadas que presentó una respuesta parcial al tratamiento antirretroviral combinado con quimioterapia sistémica y una remisión local al aplicar radioterapia sobre la lesión anal (AU)

Kaposi's sarcoma (KS) is a low-grade vascular tumor, with four main variants, one of which is fairly prevalent in HIV-infected patients. Mucocutaneus and lymph node involvement is characteristic. The gastrointestinal tract is involved in 40% of patients, but rectal and anal canal involvement is exceptional. We report the case of a 39-year-old HIV-infected man with an unusual presentation of KS with colorectal and anal canal involvement in the absence of cutaneous disease. The patient was treated with highly active antiretroviral therapy and systemic chemotherapy, with partial response. Local radiation therapy of the rectum produced local remission (AU)

[Kaposi's sarcoma with colorectal and anal canal involvement]. / Sarcoma de Kaposi con afectación colorrectal y del canal anal.

Kaposi's sarcoma (KS) is a low-grade vascular tumor, with four main variants, one of which is fairly prevalent in HIV-infected patients. Mucocutaneus and lymph node involvement is characteristic. The gastrointestinal tract is involved in 40% of patients, but rectal and anal canal involvement is exceptional. We report the case of a 39-year-old HIV-infected man with an unusual presentation of KS with colorectal and anal canal involvement in the absence of cutaneous disease. The patient was treated with highly active antiretroviral therapy and systemic chemotherapy, with partial response. Local radiation therapy of the rectum produced local remission.

Síndrome constitucional asociado a hepatotoxicidad por metformina / Constitutional syndrome associated to metformin induced hepatotoxictyn

La metformina es un antidiabético oral frecuentemente utilizado en el tratamiento de la diabetes mellitus tipo 2. En un 5-20% de los pacientes provoca trastornos gastrointestinales inespecíficos. Son mucho más raros otros efectos secundarios de mayor envergadura, como la acidosis láctica. Se han documentado algunos casos aislados de hepatotoxicidad por este fármaco. Presentamos el caso de un paciente con síndrome constitucional y alteración de la bioquímica hepática atribuible a hepatotoxicidad por metformina, tras descartarse mediante múltiples estudios una etiología tumoral y observarse una resolución completa del cuadro con la suspensión del fármaco

Metformin is an oral antidiabetic agent frequently used to manage type II diabetes. This drug produces nonspecific gastrointestinal symptoms in 5-20% of patients and, more rarely, has also been associated with severe adverse effects such as lactic acidosis. Only a few isolated cases of hepatotoxicity due to this drug have been documented. We report the case of an 83-year-old man with constitutional syndrome and hepatic biochemical alterations, which were attributedto metformin after ruling out an oncologic etiology and observing complete clinical and biochemical resolution after withdrawal of the drug

Usefulness of penicillamine-stimulated urinary copper excretion in the diagnosis of adult Wilson's disease.

OBJECTIVE: Diagnosis of Wilson's disease (WD) is reliant on liver biopsy (LB) and measurement of hepatic copper. The aim of this study was to determine the usefulness of penicillamine-stimulated urinary copper excretion (PS-UCE), a non-invasive diagnostic test, for the diagnosis of WD in adults. MATERIAL AND METHODS: In this prospective study of patients with suspected WD, total serum copper, ceruloplasmin, basal 24-h UCE and PS-UCE levels were measured. LB with copper determination was performed in those patients with persistent hypertransaminasemia and low ceruloplasmin or basal UCE > 40 microg/24 h. Diagnosis was established if the ceruloplasmin level was found to be < 20 mg/dl and hepatic copper > 250 microg/g. Results. A total of 115 patients were studied; LB was performed in 43, and WD was diagnosed in 6 (13.9%). Significant differences between WD and non-WD patients were found for basal UCE (WD: median 134.3 microg/24 h versus non-WD: median 19.0 microg/24 h (p < 0.05)) and PS-UCE (WD: median 1284.0 microg/24 h versus non-WD: median 776.0 microg/24 h; p < 0.01). In the ROC (receiver-operated curve) analysis, PS-UCE was the best discriminant between WD and non-WD (area under the curve (AUC) = 0.911, best cut-off point 1057 microg/24 h, 100% sensitivity, 82.3% specificity). CONCLUSIONS: PS-UCE is probably a useful non-invasive test in the diagnosis of WD, improving the selection of patients for diagnostic liver biopsy. Patients with PS-UCE under 1057 microg/24 h only rarely will suffer from WD and are unlikely to benefit from LB.

[Constitutional syndrome associated to metformin induced hepatotoxicity]. / Síndrome constitucional asociado a hepatotoxicidad por metformina.

Metformin is an oral antidiabetic agent frequently used to manage type II diabetes. This drug produces nonspecific gastrointestinal symptoms in 5-20% of patients and, more rarely, has also been associated with severe adverse effects such as lactic acidosis. Only a few isolated cases of hepatotoxicity due to this drug have been documented. We report the case of an 83-year-old man with constitutional syndrome and hepatic biochemical alterations, which were attributed to metformin after ruling out an oncologic etiology and observing complete clinical and biochemical resolution after withdrawal of the drug.

La hemocromatosis como polémica fuente de donación de sangre / Hemochromatosis: controversy for blood donation

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Respuesta de los autores: Fenotipo, genotipo e historia de las enfermedades de sobrecarga de hierro / Author’s response: Phenotype, genotype and history of hereditary iron overload diseases

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Respuesta. Dermatomiositis, lipodistrofia y resistencia a la insulina / Reply

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[Hereditary hemochromatosis: phenotypic study in a Spanish population]. / Hemocromatosis hereditaria: estudio fenotípico de una población española.

BACKGROUND AND OBJECTIVE: Hereditary hemochromatosis (HH) displays an important phenotypic variability and is a disease influenced by many factors. PATIENTS AND METHOD: We included 88 patients with HH. Main clinical and laboratory data were analyzed, and the influence of 6 variables on intensity of iron overload was evaluated. RESULTS: In 38.6% (95% confidence interval [CI], 28.5-49.6%) patients, none of the typical symptoms of the disease was observed. 30,9% (95% CI, 21.7-41.7%) showed abnormalities of the glucose metabolism. We detected an increase in sideremia in 75.0% patients (CI 95%, 64.4-83.3%), transferrin saturation index (TSI) in 95.4% (CI 95%, 88.1-98.5%) and ferritin in 93.2% (CI 95%, 85.1-97.1%) of patients. In addition, we observed increased values of GPT and alkaline phosphatase in an appreciable percentage of patients. Ferritin was significantly higher in men (1329.4 [913.2] ng/ml vs 656.6 [644.5] ng/ml; p < 0.001), and in those older than 45 years (1293.9 [1006.9] ng/ml vs 868.9 [642.8] ng/ml; p = 0.023] and in not blood donors (1205.2 [926.8] vs 524.8 [365.9] ng/ml; p < 0.001). TSI was 81.9% (19.6) in C282Y homozygotes and 65.7% (19.2) in the rest of HFE genotypes (p = 0.002). Differences of TSI with regard to sex, age or status of blood donor were not detected. Sideremia was significantly higher in patients infected by virus C (251.8 [24.4] microg/dl vs 182.8 [45.8] microg/dl; p = 0.001). CONCLUSIONS: HH patients have a noticeable phenotypic variability, and for that reason clinical symptoms are only orientative for the diagnosis. The relationship between HH and glucose metabolism should be investigated further. Iron parameters can be influenced by age, sex, HFE genotype, blood donation, alcohol intake and hepatitis C virus infection.

Hemocromatosis hereditaria: estudio fenotípico de una población española / Hereditary hemochromatosis: phenotypic study in a Spanish population

Fundamento y objetivo: La hemocromatosis hereditaria (HH) presenta una importante variabilidad fenotípica y es una enfermedad en la que influyen muchos factores. Pacientes y método: Incluimos a 88 pacientes diagnosticados de HH, en los que se analizó los principales datos clínicos y analíticos y se valoró la influencia de 6 variables en la intensidad de la sobrecarga de hierro. Resultados: Un 38,6% (intervalo de confianza [IC] del 95%, 28,5-49,6) de los pacientes no mostró ninguno de los síntomas típicos de la enfermedad. Un 30,9% (IC del 95%, 21,7-41,7) presentaba alteraciones del metabolismo de la glucosa. Se detectó elevación de la sideremia en un 75,0% (IC del 95%, 64,4-83,3), del índice de saturación de la transferrina (IST) en un 95,4% (IC del 95%, 88,1-98,5) y de la ferritina sérica en un 93,2% (IC del 95%, 85,1-97,1) de los casos, respectivamente, además de objetivarse una elevación de la alaninoaminotransferasa y de la fosfatasa alcalina en un porcentaje apreciable de pacientes. La ferritina se elevó significativamente más en varones (1.329,4 [913,2] frente a 656,6 [644,5] ng/ml; p < 0,001), en mayores de 45 años (1.293,9 [1.006,9] frente a 868,9 [642,8] ng/ml; p = 0,023) y en no donantes de sangre (1205,2 [926,8] frente a 524,8 [365,9] ng/ml; p < 0,001). El IST fue del 81,9 (19,6%) en los homocigotos C282Y y del 65,7 (19,2%) en el resto de genotipos HFE (p = 0,002). No se detectó diferencias del IST respecto al sexo, la edad o la situación de donante de sangre. La sideremia fue significativamente mayor en los infectados por el virus de la hepatitis C (251,8 [24,4] frente a 182,8 [45,8] µg/dl; p = 0,001]). Conclusiones: Los pacientes de HH presentan una marcada variabilidad fenotípica, por lo que los síntomas sirven únicamente como orientación diagnóstica. Debe profundizarse en la relación entre la HH y el metabolismo de la glucosa. Los índices relacionados con el hierro pueden estar influidos por la edad, el sexo, el genotipo, la donación habitual de sangre, la ingesta de alcohol y la infección por el virus de la hepatitis C

Background and objective: Hereditary hemochromatosis (HH) displays an important phenotypic variability and is a disease influenced by many factors. Patients and method: We included 88 patients with HH. Main clinical and laboratory data were analyzed, and the influence of 6 variables on intensity of iron overload was evaluated. Results: In 38.6% (95% confidence interval [CI], 28.5-49.6%) patients, none of the typical symptoms of the disease was observed. 30,9% (95% CI, 21.7-41.7%) showed abnormalities of the glucose metabolism. We detected an increase in sideremia in 75.0% patients (CI 95%, 64.4-83.3%), transferrin saturation index (TSI) in 95.4% (CI 95%, 88.1-98.5%) and ferritin in 93.2% (CI 95%, 85.1-97.1%) of patients. In addition, we observed increased values of GPT and alkaline phosphatase in an appreciable percentage of patients. Ferritin was significantly higher in men (1329.4 [913.2] ng/ml vs 656.6 [644.5] ng/ml; p < 0.001), and in those older than 45 years (1293.9 [1006.9] ng/ml vs 868.9 [642.8] ng/ml; p = 0.023] and in not blood donors (1205.2 [926.8] vs 524.8 [365.9] ng/ml; p < 0.001). TSI was 81.9% (19.6) in C282Y homozygotes and 65.7% (19.2) in the rest of HFE genotypes (p = 0.002). Differences of TSI with regard to sex, age or status of blood donor were not detected. Sideremia was significantly higher in patients infected by virus C (251.8 [24.4] µg/dl vs 182.8 [45.8] µg/dl; p = 0.001). Conclusions: HH patients have a noticeable phenotypic variability, and for that reason clinical symptoms are only orientative for the diagnosis. The relationship between HH and glucose metabolism should be investigated further. Iron parameters can be influenced by age, sex, HFE genotype, blood donation, alcohol intake and hepatitis C virus infection

[Chronic urticaria and Helicobacter pylori]. / Urticaria crónica e infección por Helicobacter pylori.

BACKGROUND AND OBJECTIVE: We intended to analyze the influence of Helicobacter pylori eradication on the clinical evolution of patients with chronic idiopathic urticaria. PATIENTS AND METHOD: The evolution of dermatological symptoms after eradication was studied in a group of 55 patients previously diagnosed with chronic idiopathic urticaria who were infected by Helicobacter pylori. RESULTS: There was a partial or complete improvement in 74.6% (CI 95%, 61-85%) of studied patients. We could not demonstrate a statistically significant influence of sex, age or endoscopic lesions on the clinical evolution of urticaria after eradication. CONCLUSIONS: These results suggest a possible usefulness of Helicobacter pylori eradication in some patients with chronic idiopathic urticaria. Controlled and randomized studies are necessary to confirm it.