RESUMEN
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Asunto(s)
Hígado Graso/etiología , Proteínas Quinasas Activadas por AMP , Adipocitos/metabolismo , Citocinas/biosíntesis , Citocinas/genética , Ácidos Grasos/metabolismo , Hígado Graso/epidemiología , Hígado Graso/metabolismo , Femenino , Radicales Libres , Hepatocitos/metabolismo , Humanos , Hiperglucemia/complicaciones , Hiperglucemia/metabolismo , Hiperinsulinismo/complicaciones , Hiperinsulinismo/metabolismo , Inflamación , Resistencia a la Insulina , Sobrecarga de Hierro/complicaciones , Sobrecarga de Hierro/metabolismo , Leptina/metabolismo , Peroxidación de Lípido , Masculino , Modelos Biológicos , Complejos Multienzimáticos/metabolismo , FN-kappa B/metabolismo , Oxidación-Reducción , Estrés Oxidativo , Receptores Activados del Proliferador del Peroxisoma/metabolismo , Proteínas Serina-Treonina Quinasas/metabolismo , Factores de Riesgo , Triglicéridos/metabolismoRESUMEN
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.
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Hígado Graso/sangre , Ferritinas/sangre , Hemocromatosis/sangre , Antígenos de Histocompatibilidad Clase I/análisis , Proteínas de la Membrana/análisis , Transferrina/análisis , Hígado Graso/diagnóstico , Hígado Graso/terapia , Femenino , Pruebas Hematológicas , Hemocromatosis/diagnóstico , Hemocromatosis/terapia , Proteína de la Hemocromatosis , Humanos , Pruebas de Función Hepática , Masculino , Persona de Mediana Edad , FlebotomíaAsunto(s)
Degeneración Hepatolenticular , Proteínas Recombinantes de Fusión , Adenosina Trifosfatasas/deficiencia , Adenosina Trifosfatasas/genética , Adenosina Trifosfatasas/fisiología , Adolescente , Adulto , Enfermedades de los Ganglios Basales/etiología , Encéfalo/patología , Proteínas de Transporte de Catión/deficiencia , Proteínas de Transporte de Catión/genética , Proteínas de Transporte de Catión/fisiología , Ceruloplasmina/deficiencia , Quelantes/uso terapéutico , Terapia por Quelación , Cobre/metabolismo , ATPasas Transportadoras de Cobre , Lámina Limitante Posterior/química , Lámina Limitante Posterior/patología , Femenino , Genes Recesivos , Hepatitis Crónica/etiología , Degeneración Hepatolenticular/diagnóstico , Degeneración Hepatolenticular/genética , Degeneración Hepatolenticular/fisiopatología , Degeneración Hepatolenticular/terapia , Humanos , Trasplante de Hígado , Masculino , Trastornos Mentales/etiología , Persona de Mediana Edad , Molibdeno/uso terapéutico , Penicilamina/uso terapéutico , Embarazo , Complicaciones del Embarazo , Pronóstico , Trientina/uso terapéutico , Zinc/uso terapéuticoAsunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/sangre , Fibrosis Quística/sangre , Fibrosis Quística/inmunología , Proteínas de la Membrana , Adulto , Factores de Edad , Péptidos Catiónicos Antimicrobianos , Proteínas Sanguíneas/inmunología , Niño , Fibrosis Quística/complicaciones , Ensayo de Inmunoadsorción Enzimática , Técnica del Anticuerpo Fluorescente , Humanos , Infecciones por Pseudomonas/complicaciones , Infecciones por Pseudomonas/inmunología , Sensibilidad y Especificidad , EspañaRESUMEN
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.
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Antipsicóticos/efectos adversos , Clopentixol/análogos & derivados , Clopentixol/efectos adversos , Seudoobstrucción Colónica/inducido químicamente , Anciano , Seudoobstrucción Colónica/diagnóstico , Femenino , HumanosRESUMEN
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.
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Enfermedades de los Conductos Biliares/diagnóstico , Fibrosis Quística/complicaciones , Cirrosis Hepática/diagnóstico , Imagen por Resonancia Magnética/métodos , Adolescente , Adulto , Enfermedades de los Conductos Biliares/etiología , Femenino , Humanos , Cirrosis Hepática/etiología , Masculino , Estudios ProspectivosRESUMEN
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.
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Golpe de Calor/complicaciones , Hepatitis/etiología , Enfermedad Aguda , Adolescente , Hepatitis/diagnóstico , Hepatitis/terapia , Humanos , Masculino , Necrosis , Esfuerzo FísicoRESUMEN
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.
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Fibrosis Quística/complicaciones , Enfermedades Gastrointestinales/complicaciones , Adulto , Fibrosis Quística/diagnóstico , Femenino , Humanos , Masculino , Estudios RetrospectivosAsunto(s)
Fibrosis Quística/diagnóstico , Fibrosis Quística/genética , Genotipo , Humanos , Sudor/químicaAsunto(s)
Enfermedades de los Conductos Biliares/complicaciones , Fibrosis Quística/complicaciones , Hepatopatías/complicaciones , Enfermedades de los Conductos Biliares/diagnóstico , Enfermedades de los Conductos Biliares/terapia , Biomarcadores , Colagogos y Coleréticos/uso terapéutico , Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/metabolismo , Humanos , Hepatopatías/diagnóstico , Hepatopatías/terapia , Ácido Ursodesoxicólico/uso terapéuticoRESUMEN
Toxic hepatitis is an infrequent secondary effect of amoxycillin-clavulanic acid with fewer than 30 well documented cases in the literature. Presentation is usually that of acute cholestasis several weeks after the initiation of treatment with this antibiotic. A new case of hepatic toxicity by amoxycillin-clavulanic acid is reported in a 47-years-old male who had taken this drug for a diarrhea syndrome. A brief review of the literature is made with special emphasis on the anatomopathologic aspects.
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Amoxicilina/efectos adversos , Antibacterianos/efectos adversos , Enfermedad Hepática Inducida por Sustancias y Drogas/etiología , Ácidos Clavulánicos/efectos adversos , Penicilinas/efectos adversos , Ácido Clavulánico , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Droxicam in a nonsteroid antiinflammatory from the oxicam family which acts as a pro-drug, being transformed into pyroxicam after being hydrolized in the stomach and has induced several cases of cholestatic or mixed hepatitis. A clinical observation in which droxicam provoked initial cholestatic hepatitis which later developed into chronic autoimmune hepatitis is presented. It has been postulated that, after causing cholestatic hepatitis by hypersensitivity and within the context of a previous autoimmune entity such as vitiligo, this drug triggered a silent autoimmune liver disease which was demonstrated clinical, analytical and histopathological manifestations 18 months later and required permanent immunosuppressive treatment.
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Antiinflamatorios no Esteroideos/efectos adversos , Enfermedades Autoinmunes , Enfermedad Hepática Inducida por Sustancias y Drogas/etiología , Colestasis/inducido químicamente , Hepatitis/inmunología , Piridinas/efectos adversos , Anciano , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedad Hepática Inducida por Sustancias y Drogas/patología , Colestasis/patología , Enfermedad Crónica , Estudios de Seguimiento , Hepatitis/tratamiento farmacológico , Hepatitis/patología , Humanos , Inmunosupresores/uso terapéutico , Hígado/patología , Masculino , Factores de TiempoRESUMEN
We present four cases of hepatitis with clinical features indicating a direct link with Droxicam. In all the cases, the presentation was that of acute hepatitis with subsequent resolution, whereas one patient developed autoimmune chronic active hepatitis. A full evaluation including ultrasound, liver biopsy, and serologic markers supported the diagnosis. Due to the fact that the "Dirección General de Farmacia y Productos Sanitarios" has approved the postponement of dispensation of products with Droxicam from February 25, 1995, this drug should not be considered anymore in the future as a potential cause of liver injury (acute or chronic hepatitis).