Histologic evaluation of organ preservation injury and correlation with cold ischemia time in 13 intestinal grafts.

Lesions produced in the graft mucosa due to harvesting, storage, and implantation must be graduated to assess the subsequent protocolized biopsy specimens. The aim is to identify type and intensity of graft mucosal lesions observed immediately after implantation. Congestion, hemorrhage, microthrombi, neutrophilic infiltrates, shortening of villi, epithelial detachment, erosion, and crypt loss were separately evaluated by two pathologists in mucosal biopsy specimens from 13 grafts. Each change was assessed as normal, mild, moderate, or severe and by splintering the summation of points a global score was designed. Cold ischemia time was registered. Correlation between the pathologists' evaluations and between final preservation injury degree and cold ischemia time was determined using the "index of correlation rho (ρ)" (Spearman's test). The same changes were assessed in 19 biopsy specimens from day 2 to day 6 (3.6 ± 1.1) to determine their evolution. Congestion was found in 7 biopsy specimens, microthrombi in 2, hemorrhage in 4, neutrophils in 6, villous atrophy in 8, epithelial detachment in 9, erosions in 2 and/or crypt loss in 2. The maximum degree of preservation injury was expressed as intense congestion and hemorrhage associated with epithelial detachment and villous atrophy. The global preservation score was grade 3 in 2 cases, grade 2 in 5, grade 1 in 2, and grade 0 in 4. There was positive correlation (ρ = 0.915) in the evaluation between pathologists (P < .01), total agreement in 9 biopsy specimens, and partial agreement (only 1 point disagreement) in 4. Mean cold ischemia time was 327 ± 101 min. (135-480). There was positive correlation (ρ = 0.694) between preservation score and cold ischemia time (P < .01). In the follow-up biopsy procedures, histological injury decreased by at least one grade in every case. Additionally, karyorrhexis was observed in 3 grafts and very occasional apoptosis in 2 others. This scale achieves good reproducibility and allows graduate preservation injury in intestinal transplantation.

Histologic evaluation of post-implantation immediate C4d deposition in 13 intestinal grafts: correlation with cell-based crossmatching, cold ischemia time, and preservation injury.

C4d deposits are predictive of humoral rejection in kidney and heart transplantation. The aim of this study was to identify C4d deposit patterns in intestinal mucosa of the grafts on biopsy specimens obtained immediately after implantation and to detect if it could be a valuable tool to predict humoral or acute rejection. A second objective was to search for a statistically significant relationship between positive C4d deposition and other collected variables. Thirteen immediately post-transplantation mucosal graft biopsy specimens, formalin fixed, underwent immunohistochemical stain for C4d deposits. Diffuse intense staining of capillary endothelium was considered positive and absent, focal or weak stains as negative. Preservation injury grade and cold ischemia times were registered for each case. Donor-specific preformed antibodies were detected by complement dependent cytotoxicity serologic technique (crossmatching). Another 19 endoscopic follow-up biopsy specimens from days 2 to 6 were also evaluated. Statistical studies were made using the index of correlation ρ (Spearman's test). Diffuse intense C4d deposits were observed in 2 grafts, focal and weak in 5, and completely negative in 6. The mean cold ischemia time was 327 ± 101 minutes. Two cases showed diffuse positive deposits, 1 had a positive crossmatch and the cold ischemia time was 360 minutes whereas the other had not preformed antibodies and its cold ischemia time was 475 minutes. Humoral or acute rejection was not observed in follow-up mucosal biopsy specimens. There was no statistically significant relationship between the C4d deposition, cold ischemia time, crossmatching results, and preservation injury degree. In conclusion, C4d deposition was not a helpful tool for diagnosis of humoral rejection and prediction of acute rejection during the early post-transplantation period.

Influence of antiviral therapy in the long-term outcome of recurrent hepatitis C virus infection following liver transplantation.

INTRODUCTION: Severity of recurrent hepatitis C virus (HCV) infection in liver transplant recipients (LTR) is variable and the influence of different factors, including the administration of antiviral therapy in the long-term outcome is controversial. METHODS: We analyzed the outcome of a cohort of HCV-infected LTR who were transplanted in our institution. Patients were divided into 2 groups (severe and non-severe HCV disease) depending on the presence of a fibrosis score of F ≥ 2 in the Scheuer index and/or fibrosing cholestasic hepatitis (FCH) in a graft biopsy. Risk factors were studied using logistic regression analysis. Survival of patients was estimated using Kaplan-Meier plots. A total of 146 patients were followed for a mean of 58 months. RESULTS: Fifty-six (34%) patients developed severe HCV disease and showed shorter survival (P < 0.024). Donor age (odds ratio [OR]: 1.04; 95% confidence interval [CI]: 1.02-1.06) and pre-transplant viral load (VL) >10(6) UI/mL (OR: 3.5; 95% CI: 1.42-10.61) were the only factors associated with severe HCV infection. Over-immunosuppression (OR: 2.3; 95% CI: 1.2-4.41) was specifically associated with the development of FCH. Overall, patient survival in recipients who received a full course of anti-HCV therapy was higher than in patients who did not complete antiviral therapy (P = 0.004) or received no treatment (P = 0.007). Patients with non-severe HCV infection have a higher probability of receiving a full course of antiviral therapy (P = 0.033). CONCLUSION: In conclusion, donor age, pre-transplant VL, and over-immunosuppression were associated with the long-term development of severe HCV recurrence in liver grafts. Administration of a full course of antiviral therapy was associated with better survival.

Encuesta rápida de ceguera evitable en Argentina en 2013 / Rapid assessment of avoidable blindness in Argentina in 2013

INTRODUCCIÓN Según datos de 2010, hay 32,4 millones de personas ciegas en el mundo (3,2 millones en América). La población mayor de 50 años concentra el 82%. Según el Censo Nacional 2010, el 24% de la población de Argentina son adultos mayores de 50 años, lo que lo convierte en el país más envejecido de Latinoamérica, con una expectativa de vida de 77,6 años. Es fundamental contar con información específica para evaluar el avance de las estrategias implementadas y diseñar otras nuevas, adecuadas a las necesidades presentes de la población. OBJETIVOS Describir la prevalencia de ceguera, deficiencia visual y sus causas en adultos de más de 50 años en Argentina. MÉTODOS Se realizó un estudio poblacional transversal con muestreo aleatorio, con 82 conglomerados de 50 personas de 50 años o más en todo el país y metodología de Encuesta Rápida de Ceguera Evitable (ERCE). Incluyó medición de agudeza visual (AV) con examen del cristalino y evaluación del polo posterior por oftalmoscopia directa. RESULTADOS De 4100 individuos elegidos, 92% fueron examinados. Hubo una prevalencia de ceguera total de 0,7%. Las principales causas de ceguera fueron catarata (44%), retinopatía diabética (16%), enfermedades de polo posterior (16%), glaucoma (8%), defecto refractivo no corregido (8%) y DMRE (Degeneración Macular Relacionada a la Edad, 4%). La cobertura de cirugía de catarata en la población estudiada fue del 97,3%. Un 82% de los ojos operados de catarata mostraron buenos resultados visuales (AV ≥20/60) y un 9,2%, resultados pobres (AV ≤20/200). DISCUSIÓN La prevalencia de ceguera en la población argentina de adultos de 50 años o más es baja. Esto refleja los resultados de las estrategias implementadas. La catarata sigue siendo la principal causa de ceguera y deficiencia visual severa; en igual proporción contribuyen la suma de patologías del segmento posterior. Debido a la alta cobertura de la cirugía de catarata, la transición epidemiológica y la tendencia poblacional, enfermedades como retinopatía diabética y alteraciones de polo posterior tendrán un impacto mayor en el futuro.

Liver transplantation from anti-hepatitis C virus-positive donors: our experience.

BACKGROUND: Hepatitis C (HCV) is among the most common causes of end-stage liver disease worldwide. The donor shortage leads us to consider alternative organ sources such as HCV-positive donors. The outcomes of these transplants must be evaluated thoroughly since there is universal recurrence of disease among HCV-positive liver transplant recipients. METHODS: From January 2005 to April 2011, we performed 143 liver transplants (OLT) to treat end-stage liver disease secondary to HCV infection. Thirteen patients (9,1%) received livers from HCV-positive donors. A control group consisted of 130 HCV-positive patients who underwent OLT during the same period with organs from HCV-negative donors. Donor HCV status was assessed by 2 tests: HCV antibodies and viral load. Not only recipient and graft survivals were analyzed, but also frequency, timing and severity of hepatitis recurrence. RESULTS: Among 143 transplants performed in HCV-positive recipients during a 6-year period from January 1, 2005, to April 30, 2011, 9.1% of patients received an organ from an anti-HCV-positive donor, 72.7% of whom showed a negative viral load. The vast majority (80%) of our patients suffered hepatitis during their follow-up, 22.4% of which were severe cases. CONCLUSIONS: No significant difference in patient or graft survival was observed between the 2 groups. A high percentage of grafts with initial positive serology for HCV showed no viral replication. Grafts from HCV-positive donors can be considered to be a safe, effective source for liver donation.

Biological behavior due to cell proliferation markers of gastrointestinal stromal tumors.

BACKGROUND/AIMS: The mitotic index and tumor size are currently the main prognostic indicators of gastrointestinal stromal tumors (GIST). The purpose of this study is to investigate the expression of different immunohistochemical markers and their relation to mortality and relapse, and especially concerning high-risk tumors. METHODOLOGY: We did a retrospective study of 68 patients who underwent surgery from 1997 to 2007 with a diagnostic of gastrointestinal stromal tumor. RESULTS: The median follow-up period was 29 months. Relapse and mortality rates were 35.3% (24 cases) and 41.2% (28 cases), respectively. The mitotic index was related to p53 and the cellular proliferation index -Ki67- (p = 0.006 and p = 0.003, respectively). Considering both high and intermediate-risk neoplasms, a significant relation to Ki67 was obtained (p = 0.008). Relapse was related to the mitotic index (p = 0.032) and Ki67 (p = 0.024). Concerning mortality, statistically significant results were obtained with necrosis variables (p = 0.02), mitotic index (p = 0.013), p53 (p = 0.024) and Ki67 (p = 0.033). CONCLUSIONS: Ki67 could be considered a prognostic marker for both relapse and mortality. Concerning high risk GIST, the usefulness the p53 protein and Ki67 nuclear antigen markers was also evident concerning relapse and mortality.

Surgical management of hereditary colorectal cancer: surgery based on molecular analysis and family history.

The importance of colorectal cancer (CRC) is increasing. A proportion show a hereditary component, as in Lynch syndrome and Familial Adenomatous Polyposis, and a recently defined entity as well, namely, Familial Colorectal Cancer type X. The high probability to develop CRC in these groups may, at the time of recognition, change surgical management, including its timing or even the surgical technique. In some cases prophylactic surgery can play an important role. The possibility of using tools that allow recognition of the aforementioned syndromes, including microsatellite instability, immunohistochemistry for DNA mismatch repair system proteins, and especially their mutations, is on the basis of therapeutic strategies that differ from those employed in sporadic CRC cases.

Surgical management of hereditary colorectal cancer: surgery based on molecular analysis and family history

The importance of colorectal cancer (CRC) is increasing. Aproportion show a hereditary component, as in Lynch syndromeand Familial Adenomatous Polyposis, and a recently defined entityas well, namely, Familial Colorectal Cancer type X. The highprobability to develop CRC in these groups may, at the time ofrecognition, change surgical management, including its timing oreven the surgical technique. In some cases prophylactic surgerycan play an important role. The possibility of using tools that allowrecognition of the aforementioned syndromes, including microsatelliteinstability, immunohistochemistry for DNA mismatchrepair system proteins, and especially their mutations, is on thebasis of therapeutic strategies that differ from those employed insporadic CRC cases(AU)

Chronic intestinal pseudo-obstruction: a diagnosis to be considered.

Chronic intestinal pseudoobstruction (CIPO) is a rare entity characterized by recurrent clinical episodes of intestinal obstruction in which no mechanical cause is identified. There are multiple causes for this syndrome but two main groups can be distinguished: a) secondary to a systemic non-gastrointestinal disease; and b) primary or idiopathic originated from alterations in the components of the intestinal wall. The latter forms are the most uncommon and their diagnosis is generally difficult. In the present article, we describe nine patients with CIPO that were diagnosed in our center over the last six years. Four of them were diagnosed with primary or idiopathic form of CIPO and another four were clearly secondary to a systemic disease. The ninth case, which was initially diagnosed as secondary, is probably also a primary form of the disease. The number of patients diagnosed in our center, even thought small, makes us to hypothesize that the prevalence of CIPO is probably greater than is generally believed and that the reasons of its rarity are the incomplete understanding of its physiopathology and the difficulties to achieve a correct diagnosis.

Chronic intestinal pseudo-obstruction: a diagnosis to be considered

Chronic intestinal pseudoobstruction (CIPO) is a rare entitycharacterized by recurrent clinical episodes of intestinal obstructionin which no mechanical cause is identified. There are multiplecauses for this syndrome but two main groups can be distinguished:a) secondary to a systemic non-gastrointestinal disease;and b) primary or idiopathic originated from alterations in thecomponents of the intestinal wall. The latter forms are the mostuncommon and their diagnosis is generally difficult. In the presentarticle, we describe nine patients with CIPO that were diagnosedin our center over the last six years. Four of them were diagnosedwith primary or idiopathic form of CIPO and another four wereclearly secondary to a systemic disease. The ninth case, whichwas initially diagnosed as secondary, is probably also a primaryform of the disease. The number of patients diagnosed in our center,even thought small, makes us to hypothesize that the prevalenceof CIPO is probably greater than is generally believed andthat the reasons of its rarity are the incomplete understanding ofits physiopathology and the difficulties to achieve a correct diagnosis(AU)

A pilot trial of fenofibrate for the treatment of non-alcoholic fatty liver disease.

BACKGROUND: Dyslipidaemia and insulin resistance are two important risk factors for non-alcoholic fatty liver disease. Both factors can improve with fenofibrate. AIMS: To evaluate the effect of fenofibrate on the clinical, analytical and histological evolution of patients with non-alcoholic fatty liver disease. SUBJECTS AND METHODS: Sixteen consecutive patients with biopsy-confirmed non-alcoholic fatty liver disease were treated with 200mg/day of fenofibrate for 48 weeks. A clinical and biochemical follow-up was done every 3 months. A new liver biopsy was performed in all patients at the end of therapy. RESULTS: All patients completed 48 weeks of therapy with fenofibrate, without adverse events. At the end of the study, a significant decrease in triglyceride, glucose, alkaline phosphatase and gamma-glutamyl transpeptidase and an increase of apolipoprotein A1 levels were found. Insulin levels and insulin resistance showed a trend to decrease. Moreover, a reduction in the proportion of patients with abnormal aminotransferase levels (>45IU/L) was observed (alanine aminotransferase: 93.7% vs. 62.5%, p=0.02; aspartate aminotransferase: 50% vs. 18.7%, p=0.02). The body mass index did not show any significant change, but the proportion of patients with metabolic syndrome decreased significantly (43.7% vs. 18.7%, p=0.04). A control biopsy after treatment revealed a decrease in the grade of hepatocellular ballooning degeneration (p=0.03), but the grade of steatosis, lobular inflammation, fibrosis or non-alcoholic fatty liver disease activity score did not change significantly. CONCLUSIONS: In patients with non-alcoholic fatty liver disease, treatment with fenofibrate is safe and improves metabolic syndrome, glucose and liver tests. However, its effects on liver histology are minimal.

[Epstein-Barr virus infection and acute cholestatic hepatitis]. / Infección por el virus de Epstein-Barr y hepatitis aguda colestásica.

Epstein-Barr virus (EBV) is a herpesvirus whose only reservoir host is the human. It is transmitted by oropharyngeal secretions. Primary EBV infection is usually asymptomatic, but sometimes it causes infectious mononucleosis with fever, lymphadenopathies, splenomegaly and pharyngitis. Acute infection is diagnosed by serology (heterophile or specific antibodies). Immunofluorescence and molecular biologic techniques may be used to demonstrate the presence of EBV in biopsy specimens. Mild and transient elevations of serum aminotransferases are common, thus liver biopsy is usually not necessary to confirm the diagnosis. Severe cholestasis is rare (5%). We describe a patient with cholestatic hepatitis and acute EBV infection with atypical lymphocytes and positive anti-VCA IgM. The patient had taken drugs (ibuprofen, paracetamol and valerian). The bad evolution of the patient, the history of exposure to drugs, and the few cases of cholestatic hepatitis due to EBV infection reported, led us to consider liver biopsy. Molecular biologic techniques confirmed the presence of EBV in liver tissue however histologic features did not exclude the toxic aetiology or the concomitant effect of drugs and EBV infection.

First Spanish series of intestinal transplantation in adult recipients.

BACKGROUND: short-bowel transplantation has experienced a substantial growth worldwide following improved results from the late 1990's on, and its coverage by Medicare. According to the International Registry (1985-2005), a total of 1,292 intestinal transplants for 1,210 patients in 65 hospitals across 20 countries have been carried out thus far. OBJECTIVE: to know short-term (6 months) results regarding patient and graft survival from the first Spanish series of intestinal transplants in adult recipients. MATERIAL AND METHODS: we present our experience in the assessment of 20 potential candidates to short-bowel transplantation between June 2004 and October 2005. Of these, 10 patients were rejected and 4 were transplanted, which makes up the sample of our study. RESULTS: to this date 5 transplants have been carried out in 4 patients (2 retransplants, 2 desmoid tumors, 1 short bowel syndrome after excision as a result of mesenteric ischemia). Upon study completion and after a mean follow-up of 180 days (range 90-190 days) all recipients are alive, and all grafts but one (75%) are fully operational, with complete digestive autonomy. All patients received induction with alemtuzumab except one, who received thymoglobulin; in all induction was initiated with no steroids. CONCLUSIONS: intestinal transplantation represents a therapeutic option that is applicable in our setting and valid for recipients with an indication who have no other feasible alternative to keep their intestinal failure under control.

Incidence of de novo nonmelanoma skin tumors after liver transplantation for alcoholic and nonalcoholic liver diseases.

INTRODUCTION: Skin tumors are the most common malignancies after orthotopic liver transplantation (OLT). They have been related to sunlight exposure, tobacco consumption, and immunosuppression. The aim of this study was to compare the incidence of de novo skin tumors (nonmelanoma) in patients who underwent liver transplantation for alcoholic cirrhosis versus nonalcoholic diseases. PATIENTS AND METHODS: Between April 1986 and July 2004, we performed 1000 OLT in a population of 888 recipients. This study was performed in a sample of 701 adult recipients who survived >2 months after transplantation: 276 patients (39.4%) underwent OLT for alcoholic cirrhosis (AC-group), and 425 (60.6%) for nonalcoholic disease (N-AC). The overall incidence of de novo skin tumors was 3.5% (25 tumors): 5.4% (15 tumors) in the AC-group and 2.4% (10 tumors) in the N-AC group (P = .027). Two patients developed two tumors. There were 19 men and 4 women, mean age at OLT of 54.4 +/- 6.8 years (range, 40 to 66 years). The mean time from OLT to tumor diagnosis was 66.1 +/- 51.4 months (range, 3 to 165 months): 56.4 +/- 44.4 months in the AC-group versus 80.6 +/- 59.8 months in the N-AC group (P = NS). Histologically, 17 tumors (68%) were basal cell carcinomas and eight tumors (32%) were squamous cell carcinomas (P = .128). Fourteen patients (60.8%) were smokers: 11 patients (84.6%) in the AC-group versus 3 patients (30%) in the N-AC group (P = .012). All the patients underwent tumor resection, with only one patient dying, because of lymph node invasion of the neck. CONCLUSION: There was a higher incidence of de novo skin tumors among patients who smoked who underwent OLT for alcoholic cirrhosis.

Primera serie nacional de trasplante de intestino en receptores adultos / First Spanish series of intestinal transplantation in adult recipients

Introducción: el trasplante de intestino, con la mejoría en los resultadosdesde finales de los años 90 y desde su cobertura por el Medicare,ha experimentado un crecimiento sustancial a nivel internacional.En la actualidad, según el Registro Internacional (1985-2005),se han realizado un total de 1.292 trasplantes de intestino en 1.210pacientes en 65 hospitales distribuidos por 20 países.Objetivo: conocer los resultados a corto plazo (6 meses) entérminos de supervivencia del paciente y del injerto de la primeraserie nacional de trasplante de intestino en receptores adultos.Material y métodos: presentamos nuestra experiencia en laevaluación de 20 potenciales candidatos a trasplante intestinal entrejunio de 2004 y octubre de 2005. De ellos, fueron desestimadosun total 10 pacientes y fueron trasplantados 4, lo que constituyela muestra de nuestro estudio.Resultados: hasta la fecha se han realizado 5 trasplantes en 4pacientes (2 retrasplantes, 2 tumores desmoides, y 1 síndrome deintestino corto tras exéresis por isquemia mesentérica). Al final delestudio y tras un seguimiento medio de 180 días (rango, 90-190días), todos los receptores están vivos, y todos los injertos, a excepciónde uno (75%), están funcionando plenamente, con autonomíadigestiva completa. Todos los pacientes recibieron induccióncon alemtuzumab excepto uno que recibió timoglobulina y entodos se inició la inducción sin esteroides.Conclusiones: el trasplante intestinal constituye una opciónterapéutica aplicable en nuestro medio y válida en receptores enquienes está indicado y que no tienen otra alternativa válida paracontrolar su insuficiencia intestinal

Background: short-bowel transplantation has experienced asubstantial growth worldwide following improved results from thelate 1990s on, and its coverage by Medicare. According to the InternationalRegistry (1985-2005), a total of 1,292 intestinal trasplantsfor 1,210 patients in 65 hospitals across 20 countries have been carriedout thus far.Objective: to know short-term (6 months) results regardingpatient and graft survival from the first Spanish series of intestinaltransplants in adult recipients.Material and methods: we present our experience in the assessmentof 20 potential candidates to short-bowel transplantationbetween June 2004 and October 2005. Of these, 10 patientswere rejected and 4 were transplanted, which makes up thesample of our study.Results: to this date 5 transplants have been carried out in4 patients (2 retransplants, 2 desmoid tumors, 1 short bowelsyndrome after excision as a result of mesenteric ischemia).Upon study completion and after a mean follow-up of 180days (range 90-190 days) all recipients are alive, and all graftsbut one (75%) are fully operational, with complete digestiveautonomy. All patients received induction with alemtuzumabexcept one, who received thymoglobulin; in all induction wasinitiated with no steroids.Conclusions: intestinal transplantation represents a therapeuticoption that is applicable in our setting and valid for recipientswith an indication who have no other feasible alternative tokeep their intestinal failure under control

Infección por el virus de Epstein-Barr y hepatitis aguda colestásica / Epstein-Barr virus infection and cholestatic hepatitis

El virus de Epstein-Barr (VEB) es un herpesvirus cuyo único huésped es el hombre, al que infecta por vía orofaríngea. La primoinfección generalmente es asintomática o cursa como una mononucleosis infecciosa que se caracteriza por fiebre, adenopatías, esplenomegalia y amigdalitis. El diagnóstico de la infección aguda suele ser serológico (anticuerpos heterófilos o específicos para el VEB), aunque el virus también puede detectarse en los tejidos mediante inmunohistoquímica o por técnicas de biología molecular. Habitualmente la infección por el VEB produce una alteración leve y autolimitada de las transaminasas (AST y ALT), por lo que no suele precisarse una biopsia hepática, y solo en el 5% causa una hepatitis aguda colestásica (HAC). Presentamos a un paciente con una HAC e infección aguda por el VEB, con linfocitos atípicos en sangre periférica y anticuerpos IgM contra la cápside VCA, que había tomado tóxicos (ibuprofeno, paracetamol y valeriana). La evolución tórpida del cuadro, la relación cronológica con la exposición a fármacos y los escasos casos publicados de HAC atribuidos al VEB, recomendaron realizar una biopsia hepática. Aunque el estudio de biología molecular en el tejido hepático resultó positivo para el VEB, los hallazgos morfológicos no permitieron excluir el origen tóxico ni la posible interacción entre fármacos y el propio virus en la etiología del cuadro

Epstein-Barr virus (EBV) is a herpesvirus whose only reservoir host is the human. It is transmitted by oropharyngeal secretions. Primary EBV infection is usually asymptomatic, but sometimes it causes infectious mononucleosis with fever, lymphadenopathies, splenomegaly and pharyngitis. Acute infection is diagnosed by serology (heterophile or specific antibodies). Immunofluorescence and molecular biologic techniques may be used to demonstrate the presence of EBV in biopsy specimens. Mild and transient elevations of serum aminotransferases are common, thus liver biopsy is usually not necessary to confirm the diagnosis. Severe cholestasis is rare (5%). We describe a patient with cholestatic hepatitis and acute EBV infection with atypical lymphocytes and positive anti-VCA IgM. The patient had taken drugs (ibuprofen, paracetamol and valerian). The bad evolution of the patient, the history of exposure to drugs, and the few cases of cholestatic hepatitis due to EBV infection reported, led us to consider liver biopsy. Molecular biologic techniques confirmed the presence of EBV in liver tissue however histologic features did not exclude the toxic aetiology or the concomitant effect of drugs and EBV infection

Chronic primary intestinal pseudo-obstruction from visceral myopathy.

Chronic intestinal pseudo-obstruction is an uncommon syndrome characterized by relapsing episodes suggesting intestinal obstruction during which no mechanical causes are identified to account for symptoms. Etiologic factors may be manifold. Among them a number of neurologic conditions, gastrointestinal smooth muscle myopathies, endocrino-metabolic and autoimmune diseases, and the use of selected drugs stand out. We report a case of chronic intestinal pseudo-obstruction originating in a sporadic, primary intestinal myopathy that corresponds to no type thus far described. A histological study of the intestinal wall showed disrupted muscle bundles and the presence of interstitial edema. Myocytes had severe degenerative changes, and no alterations were seen in submucosal and myenteric plexus neurons. The activity of enzyme complexes in the mitochondrial respiratory chain, and of thymidine phosphorylase was normal. No mitochondrial DNA changes were seen.

Pseudo-obstrucción intestinal crónica primaria debida a miopatía visceral / Chronic primary intestinal pseudo-obstruction from visceral myopathy

La pseudo-obstrucción intestinal crónica es un síndrome infrecuentecaracterizado por episodios recidivantes, sugestivos de obstrucciónintestinal, durante los cuales no se detectan causas mecánicasque justifiquen la sintomatología. Los factores etiológicospueden ser múltiples. Entre ellos destacan diversas enfermedadesneurológicas, miopatías de la musculatura lisa gastrointestinal, enfermedadesendocrino-metabólicas y autoinmunes y el uso de determinadosfármacos. Presentamos un caso de pseudo-obstrucciónintestinal crónica originada por una miopatía intestinalprimaria y esporádica que no corresponde a ningún tipo descritohasta el momento. El estudio histológico de la pared intestinalmostró que los haces musculares estaban desestructurados y queexistía edema intersticial. Los miocitos presentaban marcadoscambios degenerativos y no existían alteraciones en las neuronasde los plexos submucoso y mientérico. La actividad de los complejosenzimáticos de la cadena respiratoria mitocondrial y de la timidinafosforilasa fue normal. No se detectaron alteraciones en elADN mitocondrial

Chronic intestinal pseudo-obstruction is an uncommon syndromecharacterized by relapsing episodes suggesting intestinalobstruction during which no mechanical causes are identified toaccount for symptoms. Etiologic factors may be manifold. Amongthem a number of neurologic conditions, gastrointestinal smoothmuscle myopathies, endocrino-metabolic and autoimmune diseases,and the use of selected drugs stand out. We report a case ofchronic intestinal pseudo-obstruction originating in a sporadic,primary intestinal myopathy that corresponds to no type thus fardescribed. A histological study of the intestinal wall showed disruptedmuscle bundles and the presence of interstitial edema. Myocyteshad severe degenerative changes, and no alterations wereseen in submucosal and myenteric plexus neurons. The activity ofenzyme complexes in the mitochondrial respiratory chain, and ofthymidine phosphorylase was normal. No mitochondrial DNAchanges were seen

Pseudomyxoma peritonei: a clinicopathologic analysis and follow-up of 21 patients.

BACKGROUND/AIMS: Pseudomyxoma peritonei is an uncommon disease characterized by the presence of mucinous peritoneal implants associated with an abdominal neoplasm. Our objective is to consider the characteristics of this entity in our western Mediterranean urban population. METHODOLOGY: All cases diagnosed with pseudomyxoma peritonei by our hospital during a period of 16 years were reviewed. Data from their clinical records and the biopsy samples were analyzed. RESULTS: We found 21 cases of pseudomyxoma peritonei with a male/female ratio of 10/11 and a mean age of 59 years. The predominant presentation symptom was abdominal pain (17 cases, 6 of them with acute abdomen). The most frequent primary site of origin of the pseudomyxoma was the appendix (10 cases). The histologic diagnosis was malignant (associated with carcinoma) in 17 cases and indeterminate behavior in 4. The follow-up was available for 15 patients (mean follow-up of 41 months), while six patients have been lost. Nine patients have died during the follow-up and the other 6 patients are still alive after follow-up. CONCLUSIONS: Laparotomy is the main tool for diagnosing pseudomyxoma peritonei. The appendix is the most frequent primary site of origin of pseudomyxoma peritonei, followed by bowel; the latter being more important than previously described. In most cases the histology is malignant. The prognosis is bad with a mortality greater than 60% at 5 years.

Mujer joven con colestasis y ductopenia / No disponible

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