Síndrome papular purpúrico en guante y calcetín / Papular purpuric gloves and socks syndrome

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Síndrome posflebítico / Post-thrombotic syndrome

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Dolor abdominal y síndrome de secreción inadecuada de hormona antidiurética, forma de presentación de porfiria aguda intermitente / Abdominal pain and syndrome of inappropriate antidiuretic hormone secretion as clinical presentation of acute intermittent porphyria

La porfiria aguda intermitente (PAI) es una enfermedad poco frecuente que se caracteriza por dolor abdominal acompañado en muchas ocasiones de síntomas inespecíficos. Describimos el caso de una paciente con dolor abdominal y síndrome de secreción inadecuada de hormona antidiurética (SIADH) cuyo estudio etiológico permitió el diagnóstico final de PAI (AU)

Acute intermittent porphyria (AIP) is a rare condition characterized by abdominal pain and a wide range of nonspecific symptoms. We report the case of a woman with abdominal pain and syndrome of inappropriate antidiuretic hormone secretion (SIADH) as clinical presentation of AIP. The diagnosis was achieved through the etiologic study of the SIADH (AU)

Abdominal pain and syndrome of inappropriate antidiuretic hormone secretion as clinical presentation of acute intermittent porphyria. / Dolor abdominal y síndrome de secreción inadecuada de hormona antidiurética, forma de presentación de porfiria aguda intermitente.

Acute intermittent porphyria (AIP) is a rare condition characterized by abdominal pain and a wide range of nonspecific symptoms. We report the case of a woman with abdominal pain and syndrome of inappropriate antidiuretic hormone secretion (SIADH) as clinical presentation of AIP. The diagnosis was achieved through the etiologic study of the SIADH.

Fiebre y exantema maculopapular como primeras manifestaciones de linfoma / Fever and maculopapular exanthema as first manifestation of lymphoma

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[Collagenase-3 (MMP-13) expression in epithelial cancers of the eyelids]. / Expresión de colagenasa-3 (MMP-13) en carcinomas epiteliales de los párpados.

PURPOSE: To determine, by means of immunohistochemistry, tumoral expression of collagenase-3, a matrix metalloproteinase linked to cancer and to basal cell and squamous cell carcinomas of the eyelid. MATERIAL AND METHOD: Retrospective review of 23 basal cell carcinomas and 25 squamous cell carcinomas of the eyelid treated at different hospitals during the last five years. We evaluated collagenase-3 expression and the possible relationship to patient and tumour characteristics which included age, sex, histological type, tumour location and surgical margins status. RESULTS: 65% of the basal cell carcinomas and 88% of the squamous cell carcinomas of the eyelids stained positively for collagenase-3. In both cases, the immunostaining was localized in the cytoplasm of the malignant cells and, occasionally in the surrounding stromal cells. CONCLUSIONS: Statistical analysis showed no significant association between collagenase-3 immunostaining and patients or tumour characteristics but the expression of this protein was more intense in the epithelial tumoral cells located at the invading front which could explain the aggressive behaviour of this kind of tumours.

[Progressive multifocal leukoencephalopathy in patients with AIDS: is there a change in patients treated with highly active antiretroviral therapies?]. / Leucoencefalopatía Multifocal Progresiva en pacientes con SIDA: Hay algún cambio en pacientes bajo terapias antirretrovirales de gran actividad?

AIMS: Progressive multifocal leukoencephalopathy (PML) develops in up to 4-8% of all AIDS patients. Before highly active antiretroviral therapies (HAART) the median survival was only 4-6 months. In this study we analyzed epidemiological parameters in AIDS-related LMP patients in search for differences in the incidence and prognosis between before and after HAART era. METHODS: Retrospective review of clinical histories of patients diagnosed of AIDS and PML at Hospital Meixoeiro in Vigo, Spain, between 01/01/94-31/05/97 (Before-HAART period) and 01/06/97-30/04/00 (After-HAART period). PML was diagnosed by clinical and neuroimaging criteria, with biopsy in 2 cases and positive JC virus hibridation in CSF in another case. RESULTS: We identified 12 patients (global prevalence of 3.8%, without differences between periods): 11 males, 10 intravenous drugs users (IDU), mean age of 38 years (31-43). In 6, LMP was the first opportunistic infection. When PML was diagnosed, 6 patients had a HIV viral load (VL) > 250.000 copies of RNA/ml (range, 254.003-3.170.000), and overall a mean CD4 lymphocytes counts of 89 x 10(6)/ml (40-134). Three patients received cytarabine + Interferon with zidovudine (2 patients) and zidovudine + lamivudine (1 patient) and other patient HAART + cidofovir, but no improvement was observed. The median survival was 10 months in before-HAART group and 17 months in after-HAART group, with a survival > 48 months in 2 patients in the last group. CONCLUSIONS: LMP is observed in severely immunosuppressed IDU men (VL > log 5 copies RNA/ml and CD4 < 150 x 106/ml). Complementary treatments were ineffective and only in patients with HAART a prolonged survival was observed.

Leucoencefalopatía Multifocal Progresiva en pacientes con SIDA: ¿hay algún cambio en pacientes bajo terapias antirretrovirales de gran actividad? / Progressive multifocal leukoencephalopathy in aids patients: any change in the highly active antiretrovi ral therapies era?

Objetivos: La leucoencefalopatía multifocal progresiva (LMP) afecta al 4-8 por ciento de pacientes con SIDA, con supervivencias de 4-6 meses antes de las terapias antirretrovirales de gran actividad (TARGA). Estudiamos las variables epidemiológicas de pacientes con SIDA y LMP para evaluar posibles diferencias en incidencia y evolución entre la época pre y postTARGA. Métodos: Revisión retrospectiva de historias de pacientes con infección VIH y LMP del Hospital Meixoeiro de Vigo entre el 01/01/9431/05/97 (periodo preTARGA) y 01/06/97-30/04/00 (periodo TARGA).El diagnóstico de LMP se realizó mediante criterios clínicos y de neuroimagen, obteniéndose confirmación biópsica en 2 casos e hibridación + para virus JC en LCR en otro caso. Resultados: Identificamos 12 pacientes con LMP (prevalencia global del 3,8 por ciento, sin diferencias entre periodos): 11 varones, 10 usuarios de drogas parenterales (UDVP), edad media de 38 años (31-43). En 6, la LMP fue la primera infección oportunista. Al diagnóstico de LMP, 6 pacientes presentaban carga viral del VIH>250.000 copias ARN/ml (rango, 254.000-3.170.000), y en todos se obtuvieron recuentos medios de CD4 de 89 x106/l (40-134). Recibieron citarabina+Interferón 3 pacientes (2 con AZT y 1 AZT+3TC) y 1 paciente TARGA+cidofovir, todos sin mejoría. La mediana de supervivencia en el grupo preTARGA fue 9 meses y en el TARGA 17 meses, observándose en 2 pacientes del último supervivencias >48 meses. Conclusiones : La LMP se observa principalmente en varones UDVP, con inmunodeficiencia avanzada (CV>log 5 copias ARN/ml y CD4<150 x106/l). Los tratamientos adyuvantes empleados fueron inefectivos y sólo en pacientes bajo TARGA se consiguieron supervivencias prolongadas (AU)

Aims: Progressive multifocal leukoencephalopathy (PML) develops in up to 4-8% of all AIDS patients. Before highly active antiretroviral therapies (HAART) the median survival was only 4-6 months. In this study we analyzed epidemiological parameters in AIDS-related LMP patients in search for differences in the incidence and prognosis between before and after HAART era. Mehods : Retrospective review of clinical histories of patients diagnosed of AIDS and PML at Hospital Meixoeiro in Vigo, Spain, between 01/01/94-31/05/97 (Before-HAART period) and 01/06/97-30/04/00 (After-HAART period). PML was diagnosed by clinical and neuroimaging criteria, with biopsy in 2 cases and positive JC virus hibridation in CSF in another case. Results: We identified 12 patients (global prevalence of 3.8%, without differences between periods): 11 males, 10 intravenous drugs users (IDU), mean age of 38 years (31-43). In 6, LMP was the first opportunistic infection. When PML was diagnosed, 6 patients had a HIV viral load (VL)>250.000 copies of RNA/ml (range, 254.000-3.170.000), and overall a mean CD4 lymphocytes counts of 89 x 10 6/ml (40-134). Three patients received cytarabine+Interferon with zidovudine (2 patients) and zidovudine+lamivudine (1 patient) and other patient HAART+cidofovir, but no improvement was observed. The median survival was 10 months in before-HAART group and 17 months in after-HAART group, with a survival >48 months in 2 patients in the last group. Conclusions: LMP is observed in severely immunosuppressed IDU men (VL>log 5 copies RNA/ml and CD4<150 x106/ml). Complementary treatments were ineffective and only in patients with HAART a prolonged survival was observed (AU)

Variaciones estacionales en la hospitalización y mortalidad por insuficiencia cardiaca crónica en Vigo / Seasonal variation in chronic heart failure hospitalizations and mortality in Vigo. Spain

Objetivos: Son conocidas las variaciones estacionales en la morbimortalidad de procesos como la cardiopatía isquémica, pero hay escasos datos referentes a la insuficiencia cardiaca crónica (ICC). Este estudio analizó las variaciones estacionales en la hospitalización y muerte por ICC en Vigo.Métodos: El Servicio de Documentación Clínica del Hospital Meixoeiro (419 camas, 167.000 habitantes >14 años) facilitó datos clínicos y estadísticos de las altas con diagnóstico de ICC (Código CIE9: 428). Los datos se dividieron en 3 periodos cuatrimestrales: invernal (noviembrefebrero, años 1997-98, 98-99 y 99-00), primaveral (marzo-junio, años 97, 98 y 99) y veraniego (julio-octubre, años 97, 98 y 99). Estudiamos también tasas de hospitalización y mortalidad en Cardiología, Medicina Interna y Geriatría, que atendieron al 81 por ciento de todas las ICC ingresadas. Resultados: Hubo 1472 altas con el diagnóstico de ICC (52 por ciento varones, 83 por ciento >65 años, estancia media global: 13,8 días). Existió aumento de hospitalizaciones invernales tanto globales -4,9 (invierno) vs 3,2 (verano)-, como de servicios médicos totales -9,3 (invierno) vs 6,2, (verano). Este aumento invernal también se observó en servicios específicos: Cardiología: +4,9 por ciento, Medicina Interna: +6,5 por ciento, Geriatría: +3,2 por ciento.No hubo variaciones estacionales en la letalidad o mortalidad atribuible a ICC. El diagnóstico de ICC conllevó un riesgo de muerte 4 veces superior para los pacientes con dicho diagnóstico (OR: 3,81; IC: 3,28-4,42).Conclusiones: El diagnóstico de ICC conlleva un exceso de mortalidad intrahospitalaria. Hubo un significativo aumento de hospitalizaciones invernales por ICC. Aquellas medidas dirigidas al control de este creciente problema deberían tomar en cuenta estas observaciones (AU)

[Seasonal changes in hospitalization and mortality resulting from chronic heart failure in Vigo]. / Variaciones estacionales en la hospitalización y mortalidad por insuficiencia cardiaca crónica en Vigo.

OBJECTIVES: Circannual variation in morbi-mortality for ischemic heart disease is well-known but there are few data focusing on chronic heart failure (CHF). This report analyzes seasonal variations in CHF hospitalizations and mortality in Vigo, Northwest of Spain. METHODS: Data on hospital discharge reports with a diagnosis of CHF (3-digit ICD9 code 428) were obtained from the Clinical Documentation Service at Hospital Meixoeiro (419 beds, population: 167.000 inhabitants > 14 years old). Data were divided in three 4-months periods: winter (november-february, years: 1997-98, 98-99 and 99-00), spring (mars-june, years: 97, 98 and 99) and summer (july-october, years: 97, 98 and 99). Hospitalization rates and mortality were also studied at Cardiology, Internal Medicine and Geriatrics (attending to 81% of patients with CHF). RESULTS: A total of 1.472 CHF hospitalizations were registered (52% male, 83% > 65 years, mean inhospital stay: 13.8 days). Significatives winter increases were noted in global hospitalizations -4.9% (winter) vs. 3.2@1000 (summer) and overall medical services admissions -9.3% (winter) vs. 6.2@1000 (summer). These increases also were observed at specific medical services (Cardiology: +4.9%, Internal Medicine: +6.5%, Geriatrics: +3.2%). There was not seasonal differences in letality or attributable mortality for CHF. Death in patients with a diagnosis of CHF was 4 times more likely. (OR: 3.81; 95% CI: 3.28-4.42). CONCLUSIONS: There are a striking increase in winter hospitalizations for CHF. This diagnosis is associated with an excess of inhospital mortality. Preventive and therapeutic measures taking in account this observation are warranted to reduce the burden of this growing problem.

[Usefulness of autoantibodies in the study of autoimmune liver diseases and prevalence of autoimmune extrahepatic manifestations]. / Utilidad de los autoanticuerpos en el estudio de las enfermedades hepáticas autoinmunes y prevalencia de manifestaciones extrahepáticas autoinmunes.

BACKGROUND: Autoantibodies are prominent findings in the diagnosis of autoimmune liver diseases. However, their usefulness is limited due to the high reported prevalence in others nonautoimmune disorders. The purpose of this report was to assess the significance of these markers in patients with autoimmune liver diseases and to determine the prevalence of extrahepatic autoimmune phenomena. METHODS: We evaluated the samples from all the patients with altered biochemical liver parameters (ALT, AST, alkaline phosphatase or bilirubin) and a complete profile of autoimmunity [Anti-nuclear (ANA), anti-mitocondrial (AMA), anti-smooth muscle (SMA) and anti-liver/kidney microsomes (LKM1) antibodies] received in the Immunology Laboratory from 1993 to 1996. The records of the patients with at least one positive serologic marker were retrospectively reviewed. Autoimmune liver diseases (Autoimmune hepatitis (AIH), Primary biliary cirrhosis (PBC) and Overlap syndromes) were diagnosed according to composite clinical, analytical, histological or response-to-treatment parameters. RESULTS: Samples from 548 patients were analyzed. Of these 85 (15.5%) were positive for at least one antibody. Disorders and autoantibodies were: Autoimmune liver diseases: 18 (4 AIH, 11 PBC, 3 Overlap syndromes); alcohol-induced liver disease: 14 (5 ANA, 9 SMA), Chronic HCV infection: 28 (9 ANA, 17 SMA, 2 ANA + SMA), Chronic HCV + AIH: 2 (1 ANA, 1 ANA + SMA); other liver diseases: 7 (4 ANA, 1 AMA, 2 SMA); other diseases with liver involvement: 10 (8 ANA, 2 SMA); no liver disease (normal): 6 (3 ANA, 1 AMA, 2 SMA). In 75% (64/85) of the positivities processes regarded as immunological liver disease were not found. We identified in 12 out of 20 patients with autoimmune liver diseases others autoimmune extrahepatic processes; in 4 before a diagnosis of liver disease was made. CONCLUSIONS: Autoimmune serologic markers are useful in the study of liver diseases. However, due to inespecifity each individual patient deserves a careful evaluation. Autoimmune extrahepatic manifestations are often found and in some cases allow to recognize the hepatic involvement.

Utilidad de los autoanticuerpos en el estudio de las enfermedades hepáticas autoinmunes y prevalencia de manifestaciones extrahepáticas autoinmunes / Usefulness of autoantibodies in the study of autoinmune liver diseases and prevalence of autoinmune extrahepatic manifestations

Fundamento: Los autoanticuerpos constituyen hallazgos prominentes en las enfermedades hepáticas autoinmunes. Su utilidad está limitada por su frecuente presencia en procesos hepáticos no autoinmunes. Nuestro objetivo fue evaluar la utilidad de tales marcadores en enfermedades hepáticas autoinmunes y determinar la coexistencia con otras alteraciones autoinmunes extrahepáticas. Métodos: Estudiamos muestras llegadas al Laboratorio de Inmunología (periodo 1993-1996) de pacientes con alteraciones bioquímicas hepáticas (ALT, AST, fosfatasa alcalina y bilirrubina) y perfil serológico completo de autoinmunidad [anticuerpos antinucleares (ANA), antimitocondriales (AMA), antimúsculo liso (AML) y antimicrosomales hígado/riñón (LKM1)]. Revisamos retrospectivamente las historias con algún marcador positivo. Los procesos autoinmunes hepáticos [hepatitis autoinmune (HAI), cirrosis biliar primaria (CBP) y síndromes de superposición)] se diagnosticaron según criterios clínicos, analíticos, histológicos o de respuesta al tratamiento. Resultados: Se recibieron muestras correspondientes a 548 pacientes, de las cuales 85 (15 porciento) resultaron positivas para algún autoanticuerpo. Los procesos y anticuerpos fueron: enfermedades hepáticas autoinmunes: 18 (4 HAI, 11 PBC, 3 Síndromes de Superposición); hepatopatía alcohólica: 14 (5 ANA, 9 AML); infección crónica VHC: 28 (9 ANA, 17 AML, 2 ANA+AML); infección VHC+HAI: 2 (1 ANA, 1 ANA+AML); otras hepatopatías: 7 (4 ANA, 1 AMA, 2 SMA); otros procesos con afectación hepática: 10 (8 ANA, 2 AML); sin patología: 6 (3 ANA, 1 AMA, 2 AML). El 75 porciento (64/85) de las positividades correspondieron a procesos no considerados como hepatopatías autoinmunes. En 12 de 20 procesos autoinmunes (60 porciento) se encontraron manifestaciones extrahepáticas; en 4 antecediendo al reconocimiento de la hepatopatía. Conclusiones: Los hallazgos serológicos autoinmunes resultan útiles para el estudio de las hepatopatías, pero su inespecificidad obliga a una evaluación individual. Las manifestaciones autoinmunes extrahepáticas son frecuentes y orientan en algunos casos hacia el reconocimiento de la afectación hepática (AU)

beta-Amyloid precursor protein (beta APP) in human gut with special reference to the enteric nervous system.

The distribution of the beta-amyloid precursor protein (betaAPP) in the human gastrointestinal tract, from esophagus trough rectum, was studied using immunoblotting, as well as combined immunohistochemical and image analysis (optic microdensitometry) techniques. The study was focused on the enteric nervous system. betaAPP was detected by means of a monoclonal antibody (22C11), which recognizes all betaAPP isoforms as well as betaAPP-like proteins. Immunoblotting revealed two main protein bands, one corresponding to full-length betaAPPs (estimated molecular masses of approximately 97-115 kDa); the other corresponded to a protein with estimated molecular masses of 55 kDa. Specific betaAPP immunoreactivity (IR) was found in the submucous and myenteric plexuses localized in the supporting glial cells rather than in neurons. Differences were encountered neither in the localization nor in the intensity of immunostaining among different segments of the gastrointestinal tract. Moreover, no age-dependent changes were found. betaAPP IR was also regularly observed in blood vessels, primarily labelling endothelial cells. Our results provide evidence for the occurrence of betaAPP in human gastrointestinal tract of healthy people in both neuronal and nonneuronal tissues. Whether or not these findings have functional or clinical relevance remains to be clarified in future studies.

Immunoreactivity for beta/A4 protein, but not for its precursor, in human chromaffin cells.

The present study was designed to establish a) whether chromaffin cells of the human adrenal medulla express immunoreactivity for beta-amyloid precursor protein (beta APP) and/or beta-amyloid protein (beta/A4); and b) whether cells expressing one or both of the above-mentioned proteins display immunoreactivity for the low- (gp75) and/or the high-affinity (gp140-trkA) nerve growth factor receptor. To identify chromaffin cells and their supporting cells, chromogranin A, neurofilament proteins, and S-100 protein were studied in parallel. Beta APP and beta/A4 immunoreactivity (IR) was observed primarily labeling two different cell populations, without colocalization: Beta APP IR was found in the adrenal cortical cells, which were mainly localized in the reticulate layer and in the blood vessel walls, whereas beta/A4 IR was observed in the chromaffin cells. Furthermore, supporting cells were also immunoreactive for beta/A4, and sympathetic ganglionic cells were immunoreactive for both beta APP and beta/A4. Interestingly, clusters of cells expressing beta/A4 IR also displayed gp 75 IR and/or gp140-trkA IR. Finally, all chromaffin cells (identified by chromogranin A IR) were immunolabeled for the 200 kDa neurofilament subunit, but not for a phosphorylated epitope of this protein. These results demonstrate the occurrence of beta/A4 IR, but not of beta APP, in the chromaffin cells of the human adrenal gland. The complementary distribution of amyloid-related proteins, and the possible involvement of neurotrophins in beta/A4 metabolism are discussed.