[Etiopathogenesis of non-alcoholic steatohepatitis]. / Etiopatogenia de la esteatohepatitis no alcohólica.

The <> theory is the most widely accepted explanation for the pathogenesis of non-alcoholic steatohepatitis, while insulin resistance is a key factor. In the <> the increased afflux of fatty acids to hepatocytes combined with decreased triglyceride elimination and lower fatty acid oxygenation leads to steatosis. The steatosis is not always quiescent, since the accumulated fatty acids are susceptible to a <> in which the following factors intervene: a) oxidative stress with an increase in the production of oxygen free radicals which activate transcription factors such as NF-kappa, facilitating the formation of cytokines (tumor necrosis factor-alpha, tumor growth factor beta 1, interleukin-8 and Fas ligands; b) lipid peroxidation with the formation of malondialdehyde and 4-hydroxynonenal, promoting the afflux of inflammatory cells to the liver, depleting antioxidants such as glutathione, inducing the formation of Mallory bodies and increasing collagen synthesis on activating stellate cells, and c) leptin, endotoxins and iron overload, which together induce the lesions of steatohepatitis.

Etiopatogenia de la esteatohepatitis no alcohólica / Etiopathogenesis of non-alcoholic steatohepatitis

La teoría del «doble impacto» es la más aceptada para explicar la patogenia de la esteatohepatitis no alcohólica y la resistencia a la insulina es un factor clave. En el «primer impacto» el mayor aflujo de ácidos grasos al hepatocito, combinado con una eliminación disminuida de triglicéridos y una menor oxidación de ácidos grasos, condiciona la esteatosis. Ésta no es siempre quiescente, pues los ácidos grasos acumulados son susceptibles de un «segundo impacto», en el que intervendrían: a) el estrés oxidativo, con aumento de producción de radicales libres de oxígeno que activan factores de transcripción como el factor nuclear kappa-beta, lo cual facilita la formación de citocinas (factor de necrosis tumoral alfa, factor de crecimiento tumoral beta 1, interleucina 8 y ligandos Fas; b) peroxidación lipídica con formación de malondialdehído y 4-hidroxinonenal, que promueven el aflujo de células inflamatorias al hígado, deplecionan antioxidantes tipo glutatión, inducen la formación de cuerpos de Mallory e incrementan la síntesis colágena al activar las células estrelladas, y c) la leptina, las endotoxinas y la sobrecarga de hierro, que inducen conjuntamente las lesiones de la esteatohepatitis

The «double impact» theory is the most widely accepted explanation for the pathogenesis of non-alcoholic steatohepatitis, while insulin resistance is a key factor. In the «first impact» the increased afflux of fatty acids to hepatocytes combined with decreased triglyceride elimination and lower fatty acid oxygenation leads to steatosis. The steatosis is not always quiescent, since the accumulated fatty acids are susceptible to a «second impact» in which the following factors intervene: a) oxidative stress with an increase in the production of oxygen free radicals which activate transcription factors such as NF-κß, facilitating the formation of cytokines (tumor necrosis factor-alpha, tumor growth factor beta 1, interleukin-8 and Fas ligands; b) lipid peroxidation with the formation of malondialdehyde and 4-hydroxynonenal, promoting the afflux of inflammatory cells to the liver, depleting antioxidants such as glutathione, inducing the formation of Mallory bodies and increasing collagen synthesis on activating stellate cells, and c) leptin, endotoxins and iron overload, which together induce the lesions of steatohepatitis

[Study of patients referred for elevated ferritin levels and/or transferrin saturation: significance of non-alcoholic fatty liver disease]. / Estudio de pacientes remitidos por elevación de la ferritina y/o saturación de la transferrina: importancia del hígado graso no alcohólico.

OBJECTIVE: To determine the etiology of increased ferritin concentrations and/or transferrin saturation in patients in whom classical causes were ruled out. PATIENTS AND METHOD: We studied 43 patients (35 males and 8 females) who were referred for ferritinemia greater than 300 ng/ml and or a transferrin saturation index (TSI) greater than 40%. In all patients, glycemia, cholesterol, triglycerides, uric acid, total and fractionated bilirubin, transaminase, gammaglutamyltranspeptidase, sideremia, TSI, ferritin, HFE gene mutations, ceruloplasmin and total 24-hour urine porphyrin were evaluated and abdominal ultrasonography was performed. In 14 patients liver biopsy was performed. RESULTS: Fifty-three percent was overweight and 19% was obese. Alterations in carbohydrate metabolism were detected in 33%, hypercholesterolemia was found in 14%, hypertriglyceridemia in 35%, and hyperlipemia type IIb in 16%. Thirty-two percent showed isolated elevated ferritin, 12% had elevated TSI and 56% showed elevation of both. Transaminase levels were normal in 61%. No mutation in the HFE gene was found in 10 patients, the H63D/wt mutation was found in 18, C262Y/wt in 1, C282Y/H63D in 5, C282Y/C282Y in 4, H63D/H63D in 3 and Ser65cys/wt in 1. Ultrasonography revealed steatosis in 19 patients (44%). Definitive diagnoses were HFE-linked hemochromatosis (4 patients), juvenile hemochromatosis (1 patient), hepaticocutaneous porphyria (1 patient), and non-alcoholic fatty liver disease (22 patients; 51%). Most of the remaining patients could be included under insulin resistance syndrome. Phlebotomy was performed in 25 patients, with improvement in clinical and laboratory parameters. CONCLUSIONS: Non-alcoholic fatty acid disease is frequently detected in patients with iron metabolism disorders. These patients should undergo investigations for metabolic alterations and liver ultrasonography and, if necessary, biopsy. Phlebotomy can be useful in the treatment of these patients.

Ceruloplasmina y metabolismo del hierro: sus implicaciones en la hemocromatosis, la enfermedad de Wilson y la aceruloplasminemia / Ceruloplasmine and iron metabolism: Their implications in hemochromatosis, Wilson's disease and aceruloplasminemia

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[Chronic colonic pseudo-obstruction secondary to neuroleptics]. / Pseudoobstrucción colónica crónica secundaria a neurolépticos.

Colonic pseudo-obstruction is characterized by non-mechanical chronic colonic dilatation. It is an infrequent entity that can be provoked by multiple causes, among them pharmacological. We present the case of a 74-year-old female psychiatric patient who presented abdominal bloating, diarrhea, intense electrolytic alterations and marked radiographic colonic dilatation after treatment with a neuroleptic (zuclopenthixol decanoate). Organic obstruction and other causes were ruled out and the final diagnosis was chronic colonic pseudo-obstruction secondary to the use of neuroleptics. Cisapride (20 mg/8 h) produced a slight improvement in symptoms but colonic dilatation was permanent.

[Usefulness of magnetic cholangioresonance in the study of hepatobiliary disease in patients adults with cystic fibrosis]. / Utilidad de la colangiorresonancia magnética en el estudio de la enfermedad hepatobiliar en pacientes adultos con fibrosis quística.

INTRODUCTION: Because alterations in the bile ducts found in cystic fibrosis mimic those found in primary sclerosing cholangitis, magnetic resonance cholangiography (MRC) could be a useful diagnosis technique, especially because it is non-invasive. MATERIAL AND METHODS: We prospectively studied 26 adult patients with cystic fibrosis. Of these, 11 had liver disease previously diagnosed on the basis of symptomatology, physical examination, liver function tests and abdominal ultrasound (group A) and 15 had no apparent liver disease (group B). In all patients liver function tests, abdominal ultrasound and MRC using 1.5 Teslas General Electric and Siemens systems were carried out. The images were interpreted blind by two radiologists with experience in the interpretation of biliary alterations in cystic fibrosis. RESULT: In 6 of the 11 patients in group A, MRC showed signs of liver cirrhosis (nodularity, irregular surface, splenomegaly, varicosity); 4 patients showed rose-colored images in the choledoch and intrahepatic ducts; of the 5 patients with previous non-cirrhotic liver disease, 2 showed rose-colored intrahepatic ducts, 2 showed dilatation of the intrahepatic ducts and 1 showed hepatosplenomegaly with hepatic steatosis. Of the 15 patients in group B, bile duct anomalies were found in 5. Of these, 3 showed rose-colored images of the hepatic ducts and/or choledoch, 1 showed stenosis of the common hepatic duct with rigidity of the intrahepatic ducts and 1 showed irregularities in the caliber of the intrahepatic ducts without dilatation, which were suspicious for intrahepatic lithiasis. CONCLUSIONS: MRC is a useful technique in the study of hepatobiliary disease in cystic fibrosis because it detected anomalies in all our patients previously diagnosed with liver disease and revealed ductal lesions not revealed by other non-invasive techniques.

Divertículo gigante de sigma: presentación de un caso y consideraciones sobre el manejo de esta rara complicación de la enfermedad diverticular / Giant sigmoid diverticulum

Presentamos un nuevo caso de esta entidad clínica, diagnosticado en el contexto de un cuadro séptico, con descompensación hidrópica de una cirrosis latente, fistulizado a íleon y tratado con drenajes percutáneos. El divertículo gigante de colon es una rara complicación de la enfermedad diverticular. La presentación clínica es variable, soliendo cursar con dolor y/o con masa abdominal. Aunque su etiología permanece especulativa, parece en relación con un mecanismo de pulsión en el divertículo. La radiografía simple de abdomen, el enema opaco y la tomografía axial computarizada ayudan al diagnóstico. La resección quirúrgica es el tratamiento de elección. Nuestro caso es el primero publicado tratado conservadoramente mediante drenajes percutáneos, dada la muy precaria situación clínica de la paciente (AU)

Utilidad clínica de los anticuerpos anticitoplasma de neutrófilo con especial referencia a la fibrosis quística / Clinical usefulness of antineutrophil cytoplasmic antibodies with special reference to cystic fibrosis

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[Acute hepatitis due to heatstroke]. / Hepatitis aguda grave causada por golpe de calor.

The hepatic injury is a nearly constant event in the course of a heatstroke, which rarely evolves to a severe liver failure. In these cases, the patient's survival is conditioned to an early treatment and, sometimes, an orthotopic liver transplantation is needed. We report a case of severe acute hepatitis in a 17-year-old man, due to a heatstroke during a vigorous exercise in a training program. High ambient temperature and a long time without training predisposed to the development of heatstroke in this patient. Outcome was favourably, with a total recovery in a few weeks.

[Digestive alterations in cystic fibrosis. Retrospective study of a series of 46 adult patients]. / Alteraciones digestivas en la fibrosis quística. Estudio retrospectivo de una serie de 46 pacientes adultos.

The clinical histories of 46 adult patients (24 men and 22 women, mean age 20.6 +/- 5.1 years) diagnosed of cystic fibrosis were reviewed evaluating the digestive alterations. The age at diagnosis of cystic fibrosis was 5.63 +/- 5.3 years (range: newborns-19 years). The initial diagnosis was established by ileus meconium, in four, lung disease in 15, steatorrhea in 12, lung disease and steatorrhea in 13 and following the diagnosis of cystic fibrosis in siblings in two. Four patients presented ileus meconium, nine occlusive syndrome of the distal intestine, 42 steatorrhea (20 severe, 12 moderate and 10 mild), with the severity of the steatorrhea not being associated with the severity of the respiratory insufficiency. Two patients presents rectal prolapse, five gastroesophageal reflux syndrome (four with hiatal hernia), six cholelithiasis, one recurrent pancreatitis without detection of biliary lithiasis, one neonatal cholestasis and 10 malnutrition (five severe and five moderate) fundamentally in relation to the severity of the lung disease and, to a lesser degree, liver disease. In 10 patients chronic liver disease was diagnosed corresponding to established cirrhosis in seven, indicating liver transplantation in two. In most cases, the liver disease was already manifest in adolescence even in the cirrhotic stage. Cholangiography by magnetic resonance was useful in the study of liver disease showing abnormalities which imitated primary sclerosing cholangitis. Treatment with ursodesoxicholic acid at a dosis of 20 mg/kg/day led to a significant decrease in the transaminase values and overall of gammaglutamyltranspeptidase but did not avoid complications in the cirrhotic stages. Genetic studies performed in 36 patients detected the delta F508 mutation in 69.4%, being found in almost all of the patients with ileus meconium, occlusive syndrome of the distal intestine, liver disease, cholelithiasis and malnutrition.