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    agustin castiella

    Pyogenic liver abscess is a relatively rare disease, often concerning elderly patients in bad general condition and with underlying diseases. We report the case of a 77-year-old female with gastric cancer and liver abscesses by... more
    Pyogenic liver abscess is a relatively rare disease, often concerning elderly patients in bad general condition and with underlying diseases. We report the case of a 77-year-old female with gastric cancer and liver abscesses by Streptococcus anginosus.
    Chronic outcome following acute idiosyncratic drug-induced liver injury (DILI) is not yet defined. This prospective, long-term follow-up study aimed to analyze time to liver enzyme resolutions to establish the best definition and risk... more
    Chronic outcome following acute idiosyncratic drug-induced liver injury (DILI) is not yet defined. This prospective, long-term follow-up study aimed to analyze time to liver enzyme resolutions to establish the best definition and risk factors of DILI chronicity. 298 out of 850 patients in the Spanish DILI Registry with no preexisting disease affecting the liver and follow-up to resolution or ⩾1 year were analyzed. Chronicity was defined as abnormal liver biochemistry, imaging test or histology one year after DILI recognition. Out of 298 patients enrolled 273 (92%) resolved ⩽1 year from DILI recognition and 25 patients (8%) were chronic. Independent risk factors for chronicity were older age [OR:1.06, p=0.011], dyslipidemia [OR:4.26, p=0.04] and severe DILI [OR:14.22, p=0.005]. Alanine aminotransferase (ALT), alkaline phosphatase (ALP) and total bilirubin (TB) median values were higher in the chronic group during follow-up. Values of ALP and TB >1.1 xULN and 2.8 xULN respectively, in the second month from DILI onset, were found to predict chronic DILI (p<0.001). Main drug classes involved in chronicity were statins (24%) and antiinfectives (24%). Histological examination in chronic patients demonstrated two cases with ductal lesion and seven with cirrhosis. One year is the best cut-off point to define chronic DILI or prolonged recovery, with risk factors being older age, dyslipidemia and severity of the acute episode. Statins are distinctly related to chronicity. ALP and TB values in the second month could help predict chronicity or very prolonged recovery.
    La hemocromatosis hereditaria (HH) tipo 1 se debe a mutaciones del gen HFE, localizado en el cromosoma 6 (6p21.3). Se caracteriza por aumento de la absorción intestinal de hierro y su almacenamiento progresivo en el hígado, piel,... more
    La hemocromatosis hereditaria (HH) tipo 1 se debe a mutaciones del gen HFE, localizado en el cromosoma 6 (6p21.3). Se caracteriza por aumento de la absorción intestinal de hierro y su almacenamiento progresivo en el hígado, piel, páncreas, huesos, corazón y órganos endocrinos. El pronóstico de los pacientes depende del diagnóstico temprano de la enfermedad y del comienzo temprano del tratamiento.Se revisan las historias clínicas de los miembros de esta familia (8 varones y 5 mujeres) con HH tipo 1 estudiados y seguidos entre 1985 y 2008 en el Hospital Donostia de San Sebastián. Se realizó estudio molecular del gen HFE y de los parámetros de sobrecarga férrica en todos los familiares (17) de dos generaciones. En los familiares con la mutación C282Y/H63D y fenotipo incompleto, la cuantificación de hierro hepático se realizó por resonancia magnética (RM).La mutación C282Y en homocigosis estaba presente en 3 familiares, en heterocigosis (asociada a la H63D) en otros 3 y en heterocigosis sola en 6. La biopsia hepática reveló fibrosis y/o cirrosis en 4 familiares (3 con C282Y/C282Y y otro con C282Y/-) mayores de 40 años con hepatomegalia, artralgias y, en algunos, pigmentación cutánea, astenia e hipogonadismo. La RM hepática en dos familiares con heterocigosis (C282Y/H63D) e índice de saturación de transferrina mayor del 50% no detectó sobrecarga férrica tras varios años de seguimiento, lo que ha evitado la realización de sangrías.Se comentan las formas clínicas de la HH tipo 1 en esta familia y las alteraciones moleculares encontradas. La RM hepática se debería incorporar en el seguimiento de los individuos con alteraciones del metabolismo férrico y mutaciones en el gen HFE.Hereditary hemochromatosis (HH) type I is due to mutations in the HFE gene, located on chromosome 6 (6p21.3). This disease is characterized by increased intestinal iron absorption and progressive storage in the liver, skin, pancreas, bones, heart and endocrine organs. Patient prognosis depends on early diagnosis and prompt treatment of the disease.We reviewed the medical records of family members (eight men and five women) with HH type I, studied and followed-up between 1985 and 2008 in Hospital Donostia, San Sebastian (Spain). Molecular studies were performed of the HFE gene and of iron overload parameters in all the family members (N = 17) belonging to two generations. In family members with the C282Y/H63D mutation and incomplete phenotype, liver iron quantification and monitoring was done by magnetic resonance imaging (MRI).The homozygous C282Y mutation was present in three family members, the heterozygous mutation (associated with H63D) in three, and the heterozygous mutation alone in six. Liver biopsy revealed fibrosis and / or cirrhosis in four family members (three with C282Y/C282Y and another with C282Y/-) aged over 40 years with hepatomegaly, arthralgia, and in some cases, skin pigmentation, fatigue, and hypogonadism. Liver MRI in two family members with the heterozygous mutation (C282Y/H63D) and transferrin saturation greater than 50% showed no iron overload after several years of monitoring, avoiding the need for phlebotomy.We present the clinical manifestations of HH type 1 in this family and the molecular alterations found. Liver MRI should be incorporated into the monitoring of individuals with impaired iron metabolism and HFE gene mutations.1 motako hemokromatosi hereditarioa (HH) 6 (6p21.3) kromosoman dagoen HFE genearen mutazioagatik gertatzen da. Burdinaren heste-xurgapena handitzen da, eta apurka, burdin hori gibelean, azalean, pankrean, hezurretan, bihotzean eta organo endokrinoetan pilatzen da. Gaixoen pronostikoa gaixotasunaren diagnosi goiztiarraren eta tratamendua goiz hastearen arabera aldatzen da1 motako HH duen familia bateko kideei (8 gizon eta 5 emakume) 1985 eta 2008 artean Donostiako Ospitalean aztertutako historia klinikoak berraztertu dira. Bi belaunalditako familiartekoen (17) HFE genearen eta burdinaren gainkarga-parametroen azterketa molekularra egin zen. C282Y/H63D mutazioa eta fenotipo osatugabea zuten familiartekoen kasuan, burdin hepatikoaren kuantifikazioa erresonantzia magnetikoaren (EM) bidez egin zen.3 familiartekok zuten C282Y mutazioa homozigosian, beste hiruk heterozigosian (H63Drekin lotuta) eta beste seik heterozigosian bakarrik. Biopsia hepatikoaren arabera, 4 familiartekok fibrosi edota zirrosia zuten (3 familiartekok C282Y/C282Y eta besteak con C282Y/-). Familiako kide horiek 40 urtetik gorakoak ziren, hepatomegalia eta artralgia zituzten, eta batzuek azaleko pigmentazioa, astenia eta hipogonadismoa ere bai. Hainbat urtez monitorizatzen aritu ostean, erresonantzia magnetiko hepatikoak ez zuen hauteman burdinaren gainkarga heterozigosia eta siderofilina-saturazio indizea %50etik gorakoa zuten bi familiartekorengan (C282Y/H63D). Horrek odol-ateratzeak saihestu zituen.Familia honetako kideengan 1 motako HH gaixotasunak dituen forma klinikoen eta asaldura molekularren gaineko iruzkinak egin dira. Erresonantzia magnetiko hepatikoa burdinaren metabolismoan asaldurak dituzten eta HFE genearen mutazioak dituzten pertsonei jarraipena egiteko erabili beharko litzateke.
    ABSTRACT
    We studied the efficacy of local injections of botulinum toxin in the treatment of patients with achalasia. Four patients diagnosed of achalasia using manometric, radiologic and endoscopic criteria, were treated with botulinum toxin (80... more
    We studied the efficacy of local injections of botulinum toxin in the treatment of patients with achalasia. Four patients diagnosed of achalasia using manometric, radiologic and endoscopic criteria, were treated with botulinum toxin (80 U) injected directly into lower esophageal sphincter (LES), via a sclerotherapy injector. Response to treatment was assessed by changes in symptom scores and LES pressure. All determinations were repeated after 10, 30, 90, 120 and 180 days of treatment. The patients improved after the initial injection. This improvement was accompanied by improved relaxation of the LES. Two patients relapsed after 30 and 65 days and the other two patients remained symptom-free 5 months after treatment. Botulinum toxin is probably a safe and effective alternative for the treatment of achalasia and should be considered in patients in whom pneumatic dilation has failed or who are poor surgical candidates. Long-term evaluation of the safety and efficacy of botulinum toxi...
    The hepatotoxic potential of statins is controversial. The objectives of this study were to describe the relative frequency of hepatotoxicity caused by statins and the phenotypes found in Spain. The incidence of hepatotoxicity attributed... more
    The hepatotoxic potential of statins is controversial. The objectives of this study were to describe the relative frequency of hepatotoxicity caused by statins and the phenotypes found in Spain. The incidence of hepatotoxicity attributed to statins in the Spanish Hepatotoxicity Registry (REH) were studied and compared with those attributed to other drugs. Between April 1994 and August 2012, the REH included a total of 858 cases of which 47 (5.5 %) were attributed to statins. Of these, 16 were due to atorvastatin (34 %); 13 to simvastatin (27.7 %); 12 to fluvastatin (25.5 %); 4 to lovastatin (8.5 %) and 2 to pravastatin (4.3 %). Statins represented approximately half of the cardiovascular group which occupied 3er place (10 %), after anti-infectious agents (37 %) and central nervous system drugs (14 %). The hepatocellular pattern was predominant, especially in the simvastatin group (85%), the cholestatic/mixed pattern was more frequent with fluvastatin (66 %) and had a similar distrib...
    To determine whether the product of multiplying age by liver iron concentration (LIC) (fibrosis index; cut-off, 480,000), platelets, transaminases, and ferritin values are related to the risk of high grade fibrosis. A retrospective study... more
    To determine whether the product of multiplying age by liver iron concentration (LIC) (fibrosis index; cut-off, 480,000), platelets, transaminases, and ferritin values are related to the risk of high grade fibrosis. A retrospective study of 32 patients with hereditary hemochromatosis (HH) with phenotypic expression. All patients had a liver biopsy with LIC. In 7 patients a magnetic resonance imaging (MRI) scan (1.5 T) was obtained with LIC following Alustiza's protocol. Liver biopsy: fibrosis grade (F) 0-2 in 23 patients; F 3-4 in 9. Fibrosis index (FI) showed a specificity of 68%, sensitivity of 85.7%, positive predictive value (PPV) of 42.8%, and negative predictive value (NPV) of 94.4% for high-grade fibrosis. Platelet count ( < 200,000) revealed a NPV of 94.7% for F3-4. Aspartate transaminase (AST) levels above the upper limit of normal showed a NPV of 94.4%; ferritin levels (> 1,000) a NPV of 75%, and MRI-derived LIC x age (> 480,000) a NPV of 80%. The combination ...
    To evaluate the quantification of liver iron concentration using 1-Tesla magnetic resonance imaging (MRI) and its ability to diagnose or rule out hemochromatosis. To evaluate the role of 1.5-Tesla MRI in inconclusive cases. Between 2002... more
    To evaluate the quantification of liver iron concentration using 1-Tesla magnetic resonance imaging (MRI) and its ability to diagnose or rule out hemochromatosis. To evaluate the role of 1.5-Tesla MRI in inconclusive cases. Between 2002 and 2006, we used 1-Tesla MRI (Gandon method) and liver biopsy to quantify the liver iron concentration in 31 patients. Moreover, we used 1.5-Tesla MRI (according to Alústiza's model) and liver biopsy to determine the liver iron concentration in 10 additional patients and to check the results of 10 patients in whom 1-Tesla MRI detected iron overload. In the first group of 31 patients, liver biopsy classified the liver iron concentration as normal (<36 micromol.Fe/g) in 11 patients, as hemosiderosis (36-80 micromol.Fe/g) in 15, and as hemochromatosis (>80 micromol.Fe/g) in 5. The correlation with the values calculated at MRI was 100% in the 5 cases with hemochromatosis; in the 15 patients with hemosiderosis, 5 were correctly classified and t...
    Hereditary hemochromatosis is the most common cause of iron overload. The diagnosis of hereditary hemochromatosis has improved since Feder et al. isolated the HFE gene in 1996 and discovered the mutations related with this disease.... more
    Hereditary hemochromatosis is the most common cause of iron overload. The diagnosis of hereditary hemochromatosis has improved since Feder et al. isolated the HFE gene in 1996 and discovered the mutations related with this disease. Nevertheless, in many cases genetic tests for hereditary hemochromatosis are negative. These cases require diagnostic confirmation by quantifying the concentration of iron in the liver (LIC); this has traditionally been accomplished by liver biopsy. Many studies have shown that it is possible to quantify LIC using MRI. However, a consensus has yet to be reached about the most appropriate technique or whether it is possible to reproduce the same methods of calculation on different MRI units. This article reviews the current state of these questions and points to possible lines to standardize this noninvasive method of quantifying LIC in the future.
    To analyze the indications, efficacy and safety of sphincteroplasty in our centre. A retrospective study of sphincteroplasty in 53 cases of papilla at high risk was performed in 2004-2006. The procedure consisted of duodenoscopy with... more
    To analyze the indications, efficacy and safety of sphincteroplasty in our centre. A retrospective study of sphincteroplasty in 53 cases of papilla at high risk was performed in 2004-2006. The procedure consisted of duodenoscopy with Olympus TJF 145 Videoduodenoscope, approach to the biliary tract using a catheter with a guidewire, and dilatation of the papilla with a dilatation balloon catheter using a syringe with a manometer for control of the filling pressure. The indications included intradiverticular papilla in 26 patients (49%), stenosis of a previous sphincterotomy in 19 patients (35.8%), small size of the papilla in 4 patients (7.5%), Billroth II gastrectomy in 3 patients (5.6%), and coagulopathy in one patient (1.9%). The efficacy was 97.8%, with all the calculi extracted from the common bile duct in 84.4% of the patients, even though 21 of the patients (39.6%) had calculi with a diameter equal to or greater than 10 mm. Seven patients (13.2%) presented complications: haemo...
    1. Gastroenterol Hepatol. 2005 Jun-Jul;28(6):361. [Celiac disease and selective IgA deficit]. [Article in Spanish]. Vita Garay LA, Bengoechea Gallastegui L, Abalo Durán C, Fernández Fernández J, Castiella Eguzkiza A. PMID: 15989820... more
    1. Gastroenterol Hepatol. 2005 Jun-Jul;28(6):361. [Celiac disease and selective IgA deficit]. [Article in Spanish]. Vita Garay LA, Bengoechea Gallastegui L, Abalo Durán C, Fernández Fernández J, Castiella Eguzkiza A. PMID: 15989820 [PubMed - indexed for MEDLINE]. ...
    To determine the epidemiological characteristics of liver disease secondary to alpha-1-antitrypsin deficiency and associated processes in the Spanish population. We reviewed the medical records of adults with liver abnormalities due to... more
    To determine the epidemiological characteristics of liver disease secondary to alpha-1-antitrypsin deficiency and associated processes in the Spanish population. We reviewed the medical records of adults with liver abnormalities due to alpha-1-antitrypsin deficiency diagnosed between 1981 and 2001 in the Hospital Donosti in San Sebastian (Spain) as well as the cases published in the literature before 1999. Diagnosis was based on clinical and biochemical data, imaging tests and/or liver biopsy and/or necroscopy together with serum values of alpha-1-antitrypsin and phenotyping. Fifty cases of liver disease secondary to alpha-1-antitrypsin deficiency (45 from the literature and 5 from our hospital) were included. There were 34 men and 16 women aged between 18 and 77 years. Fifteen (30%) had relatives with alpha-1-antitrypsin deficiency. Hepatitis and/or neonatal cholestasis were confirmed in 4 and alcoholism was confirmed in 17. Of the series, 8 (16%) had portal fibrosis and 29 (58%) h...
    To determine the prevalence of coeliac disease amongst the population with unexplained chronic hypertransaminasemia in our region. A prospective study was carried out on 147 consecutive patients with chronic hypertransaminasemia, having... more
    To determine the prevalence of coeliac disease amongst the population with unexplained chronic hypertransaminasemia in our region. A prospective study was carried out on 147 consecutive patients with chronic hypertransaminasemia, having previously discarded alcoholic cause, hepatotoxic drugs, B, C and Delta viral infections, autoimmune hepatitis, primary biliary cirrhosis, Jemochromatosis, alfal-antitrypsin deficiency, Wilson's disease, congestive liver and illicit drug use. Serum Ig A to gliadin and endomysium antibodies were determined. Intestinal biopsy was carried out in cases those positive for one or both antibodies. One patient was positive for both IgA to gliadin and to endonisyum antibodies, whereas another three patients were positive to IgA to gliadin only. A duodenal biopsy proved normal in two, a total villous atrophy in one and subtotal atrophy in other. 1. The prevalence of coeliac disease amongst the population with unexplained chronic hypertransaminasemia in our...
    to determine the clinical, microbiological, diagnostic and therapeutic characteristics of pyogenic liver abscesses of bacterial origin. retrospective analysis of pyogenic liver abscesses diagnosed at the Aránzazu Hospital in San Sebastián... more
    to determine the clinical, microbiological, diagnostic and therapeutic characteristics of pyogenic liver abscesses of bacterial origin. retrospective analysis of pyogenic liver abscesses diagnosed at the Aránzazu Hospital in San Sebastián (northern Spain) between 1989 and 1998. we studied 45 patients with pyogenic liver abscesses of bacterial origin (30 men, 15 women, mean age 61 years 11 months). The site of the liver abscess was biliary in 28.9% of the patients, portal in 11.1%, and unknown in 33.3%. Elevated erythrocyte sedimentation rate (95.5%), leukocytosis (86.7%) and fever (82.2%) were the most frequent clinical and laboratory findings. The abscesses were solitary in 55. 5% of the patients. Echography was diagnostic in 68.4% of patients, and computed tomography was diagnostic in 100%. Cultures of pus from the abscess and blood were positive in 77.1% and 50% respectively. Of the abscesses diagnosed as being of bacterial origin, 44.4% involved multiple organisms. Escherichia c...
    Bronchogenic lung carcinoma, melanoma and breast cancer are the neoplasms which have most frequently been reported to metastasize to the stomach. These lesions are usually located on the fundus and on the upper part of the gastric body.... more
    Bronchogenic lung carcinoma, melanoma and breast cancer are the neoplasms which have most frequently been reported to metastasize to the stomach. These lesions are usually located on the fundus and on the upper part of the gastric body. They are usually asymptomatic with the diagnosis being made at necropsy. We present a patient who developed gastrointestinal bleeding as the first symptom of squamous lung cancer secondary to a gastric metastasis.
    Bacteriemia is a frequent infection in patients with hepatic cirrhosis, which is associated with a high mortality. Spontaneous bacteriemia is generally caused by gramnegative bacilli. We present an episode of Salmonella hadar's... more
    Bacteriemia is a frequent infection in patients with hepatic cirrhosis, which is associated with a high mortality. Spontaneous bacteriemia is generally caused by gramnegative bacilli. We present an episode of Salmonella hadar's spontaneous bacteriemia in a patient with cirrhosis of the liver and transjugular intrahepatic portosystemic shunt. The role of the prosthesis in the pathogenesis of bacteriemia in hepatic cirrhosis is discussed.

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