Glomerulonefritis extracapilar tipo I con coexistencia de anticuerpos anti-MBG y ANCA-p positivos / No disponible

No disponible

Marcadores de desdiferenciación podocitaria en un paciente con glomerulonefritis colapsante / No disponible

No disponible

Papuloeritrodermia de Ofuji. Como manifestación de linfoma cutáneo a propósito de dos casos / Ofuji’s erythroderma as a clinical manifestation of cutaneous lymphoma in two patients

Se revisan dos casos de eritrodermia de larga evolución. El primero correspondiente a una parapsoriasis en placas y el segundo a un linfoma cutáneode células T (micosis fungoide) en los que destacaban bandas horizontales, lineales, de piel sana coincidentes con los pliegues de flexión en tórax yabdomen que constituían el denominado signo de la tumbona (deck chair sign), marcador típico de la papuloeritrodermia de Ofuji. Llegando a la conclusiónque debemos considerarla como patrón de expresividad clínica de diversos procesos entre ellos algunos de carácter maligno

We report two cases of long term development erythroderma. In the first case the diagnosis of parapsoriasis was confirmed and in the second one acutanoeus T cell Lymphoma (mycosis fungoides) was associated the erythroderma characteristically showed an extensive papular eruption sparing thetransverse abdominal and trunk folds: “the deck-chair sign” as a typical sign of the papuloerythroderma of Ofuji. We agree that Ofuji’s papuloerythrodermais a pattern of expression of many inflamatory dermatoses as well as a variety of pathological processes even malignant

Soluble HLA-G in heart transplantation: their relationship to rejection episodes and immunosuppressive therapy.

The aims of this study were to quantify the level of soluble HLA-G in heart transplant patients, to determine the relationship between the sHLA-G levels and the appearance of acute rejection episodes, and to identify the influence of immunosuppressive therapy on sHLA-G levels. Analysis of sHLA-G, measured by enzyme-linked immunosorbent assay in the transplant patients, revealed the existence of two similarly sized groups of patients. One group displayed a significant increase (p < 0.001) in sHLA-G during the first month after transplantation while the other group maintained low levels of the molecule (0-30 ng/ml) throughout the study. The latter group displayed a high incidence of recurrent severe rejection. A significant increase (p < 0.01) in sHLA-G 2 hours after administration of immunosuppressive treatment (mycophenolate mofetil, cyclosporine A/FK506, corticoids) was found. These results suggest that sHLA-G participates in the induction of certain levels of immunological tolerance in these recipients.

[Acute renal failure in a patient with myelodysplastic syndrome and paroxysmal nocturnal hemoglobinuria phenotype]. / Fracaso renal agudo en pacientes con síndrome mielodisplásico y fenotipo de hemoglobinuria paroxística nocturna.

Hemoglobin and myoglobin heme pigments and iron have acute and chronic nephrotoxic effects, which are often associated with massive hemolysis and rhabdomyolysis. We report a patient with a myelodysplastic syndrome and paroxysmal nocturnal hemoglobinuria phenotype who developed an acute renal failure after a severe haemolytic crisis. There was not evidence of renal vascular pathology, urinary tract obstruction or prerenal factors. Renal biopsy showed features of acute tubular necrosis, with extended iron deposits in tubule cell cytoplasm and tubulo-interstitial fibrosis and atrophy. The patient was oliguric requiring hemodialisys during three weeks, recovering renal function on the fourth week after admission. This case underlines the nephrotoxic role of heme pigment and iron, and possible pathophysiologic mechanisms involved in acute and chronic toxicity of both agents are reviewed.

Fracaso renal agudo en pacientes con síndrome mielodisplásico y fenotipo de hemoglobinuria paroxística nocturna / Acute renalfailure in a patient with myelodysplastic syndrome and paroxysmal nocturnal hemoglobinuria fenotype

El pigmento heme y el hierro contenidos en la hemoglobina y mioglobina tienen un efecto nefrotóxico agudo y crónico, que es manifiesto en situaciones de hemólisis masiva y rabdomiólisis. Describimos el caso de un paciente diagnosticado de síndrome mielodisplásico con fenotipo de hemoglobinuria paroxística nocturna que desarrolló un fracaso renal agudo tras una crisis hemolítica severa. No se encontraron evidencias de patología vascular renal, obstrucción de vías urinarias ni factores prerrenales. La biopsia renal mostró cambios compatibles con necrosis tubular aguda, intensos depósitos de hierro en las células tubulares, fibrosis y atrofia tubulo-intersticial. El paciente se mantuvo en oligoanuria durante las primeras semanas, precisando hemodiálisis, recuperando la función renal en la cuarta semana. En este caso se remarca el papel nefrotóxico del pigmento heme y del hierro y se revisan los mecanismos fisiopatológicos implicados en la toxicidad aguda y crónica de ambos agentes (AU)

Hemoglobin and myoglobin heme pigments and iron have acute and chronic nephrotoxic effects, which are often associated to massive hemolysis and rabdomyolisis. We report a patient with a myelodysplastic syndrome and paroxysmal nocturnal hemoglobinuria fenotype who developed an acute renal failure after a severe haemolytic crisis. There was not evidence of renal vascular pathology, urinary tract obstruction or prerrenal factors. Renal biopsy showed features of acute tubular necrosis, with extended iron deposits in tubular cells cytoplasm and tubulo-intersticial fibrosis and atrophy. The patient was oliguric requering hemodialisys during three weeks, recovering renal function on the fourth week after admission. This case underlines the nephrotoxic role of heme pigment and iron, and possible pathophysiologic mechanisms involved in acute and chronic toxicity of both agents are reviewed (AU)

Acroqueratoelastoidosis de Costa / Acrokeratoelastoidosis

La acroqueratoelastoidosis (AKE) de Costa es una forma de queratodermia palmopalmar poco frecuente que puede aparecer de forma esporádica o tener un patrón autosómico dominante. Rara vez está presente en el nacimiento; en la mayoría de las ocasiones lo hace a partir de la adolescencia en forma de pápulas translúcidas, amarillentas o del color de la piel normal. Tienen una localización típica en las eminencias tenar e hipotenar, pudiendo también afectar las palmas de las manos y las zonas laterales de los dedos. La asociación de hiperhidrosis es un hecho habitual. Histológicamente, los datos más característicos son hiperqueratosis, discreta acantosis, fragmentación y desestructuración de las fibras elásticas de la dermis reticular. Los tratamientos son pocos eficaces. Comentamos el caso de una niña de 10 años afectada por esta enfermedad y revisamos el diagnóstico diferencial con el grupo de las llamadas acroqueratodermias papulares marginales (AU)

Antithrombin III prevents early pulmonary dysfunction after lung transplantation in the dog.

BACKGROUND: Ischemia-reperfusion injury with the resulting inflammatory response is a devastating complication of lung transplantation; much of the tissue damage could be diminished by control of the inflammatory response. Recent studies have show that antithrombin III (AT III) has an anti-inflammatory effect in addition to its established role in the regulation of blood coagulation. Thus, we hypothesized that the administration of AT III might help to prevent ischemia-reperfusion injury after lung transplantation. METHODS AND RESULTS: The study was performed in a dog model of orthotopic lung transplantation. Dogs were randomly assigned to receive either vehicle (controls) or AT III. We observed that in control dogs, during the 180-minute period after lung transplantation, the arterial O(2) partial pressure decreased and both the alveolar-arterial O(2) difference and the pulmonary vascular resistance increased. By contrast, these parameters remained unchanged in the group of dogs receiving AT III. Dogs with transplants receiving AT III did not show an increase in cell adhesion molecules, and histological examination revealed almost an absence of inflammatory response. The administration of AT III produced a marked increase in serum prostacyclin (PGI(2)) levels, whereas in control dogs, the PGI(2) levels did not change. The beneficial effect of AT III was not observed when dogs received indomethacin to prevent the stimulation of PGI(2) release by AT III. CONCLUSIONS: Our results demonstrate that AT III prevents ischemia-reperfusion injury in a dog model of lung transplantation and that this effect is conditioned by an increase in PGI(2) production.

Ectopic ACTH syndrome caused by pulmonary carcinoid tumourlets.

The differential diagnosis of Cushing's syndrome is a major challenge to clinical endocrinologists, especially those infrequent cases referred to as occult ectopic ACTH syndromes. Although bronchial carcinoids are well known to be a cause of Cushing's syndrome due to ectopic ACTH secretion, very few cases of carcinoid tumourlets causing an ACTH ectopic syndrome have been reported, and their origin remains controversial. For some authors, tumourlets and typical carcinoids represent distinct pathological entities, whilst others hold that tumourlets are merely microscopic carcinoid tumours. We report a patient with an aggressive Cushing's syndrome that required bilateral adrenalectomy, diagnosed 22 years before a 3-cm lung nodule became apparent on routine chest X-ray. The biopsy after lung surgery revealed a typical peripheral bronchial carcinoid surrounded by tumourlets. Both tumourlets and carcinoid tumour showed strongly positive ACTH immunostaining. Recently, Arioglu et al. (1998) reported a case of Cushing's syndrome caused by pulmonary carcinoid tumourlets, concluding that this entity should be considered in the differential diagnosis of occult ectopic ACTH syndrome. Furthermore, we consider that the carcinoid tumourlets found in our patient, were the initial source of ACTH, leading to Cushing's syndrome with a rapid onset, and that a loss of cell proliferation control in one of such tumourlets many years later, could have resulted in the development of a typical carcinoid tumour, reinforcing the theory of a common origin of these lesions.

Interferon therapy in hemodialysis patients with chronic hepatitis C virus infection induces a high rate of long-term sustained virological and biochemical response.

AIM: The aim of the present study was to analyze the efficacy and tolerance of interferon (IFN) therapy in hemodialysis (HD) patients with chronic hepatitis C virus (HCV) infection. Specifically, we assessed whether the "normalization" of serum ALT levels was associated with the disappearance of the HCV-RNA. METHODS: Thirteen hemodialysis patients with chronic hepatitis C were treated for one year with 3 MU of alpha-IFN. The primary end point was a sustained virological response defined as the absence of HCV-RNA in the last follow-up; the secondary end points were normalization of the serum ALT levels and histological improvement. ALT was considered "normal" below 27 IU/l. RESULTS: Ten patients completed the treatment, which was discontinued in the other 3 (23%). By the end of the treatment a virological response was observed in 8 of the 10 patients (80%) who completed the one-year IFN therapy. Biochemical response was associated with a virological response in 8 of the 9 patients in whom ALT levels became normal. Three patients had a biochemical and virological relapse in the follow-up. Two of them received a further year of IFN therapy, which resulted in a sustained biochemical and virological response. In all patients who underwent a liver biopsy (n = 5), the inflammation score improved. After a median follow-up of 5 years (range 2 - 7), a sustained response was observed in 6 (46%) of the 13 patients enrolled. Two patients with a sustained response received a kidney transplant and after more than 6 years still maintain a biochemical and virological response. Side effects included flu-like syndrome (n = 8), hemoglobin decrease (n = 8), thrombocytopenia (n = 3), depression (n = 1) and seizures (n = 1). CONCLUSION: IFN treatment over a one-year period produces a high rate of long-term virological response in HD patients, associated to a biochemical response in all cases.

[Efficacy of fecal detection of Helicobacter pylori with the HpSA technique in patients with upper digestive hemorrhage]. / Eficacia de la determinación fecal de Helicobacter pylori mediante la técnica HpSA en enfermos con hemorragia digestiva alta.

BACKGROUND: The rapid urease test is the most commonly used test in the diagnosis of Helicobacter pylori infection in patients with upper gastrointestinal hemorrhage. However, some studies have suggested that results of this test are frequently false negative when blood is present. An effective new enzyme immunoassay for determining H. pylori antigens in stools has recently begun to be used. AIM: To determine the efficacy of the H. pylori stool antigen test (HpSAT) in patients with upper gastrointestinal hemorrhage. PATIENTS AND METHODS: Thirty-two patients with upper gastrointestinal hemorrhage were prospectively studied from November 1998 to April 1999. In all patients the following tests were performed in the first 72 hours after onset of bleeding and 24 hours after hospital admission: upper gastrointestinal endoscopy, biopsy samples for the rapid urease test and histological study, blood samples for serology, stool samples for HpSAT, and the 13C urea breath test. Criteria for infection was a positive result in at least two of the four diagnostic techniques, except in the case of HpSAT. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated. RESULTS: Of the 32 patients, 23 (71.8%) were infected. The results of four HpSAT (12.5%) were negative and 28 were positive (87.5%). HpSAT showed high sensitivity (95.6%) but low specificity (33.3%). The PPV and NPV were 78.5% and 75% respectively. Of the 32 HpSAT, 25 (78.1) were performed in melenic stools: 22 were positive and 3 were negative. Seventy-five percent of negative HpSAT and 78.5% of positive HpSAT corresponded to melenic stools. CONCLUSIONS: HpSAT is a rapid, non-invasive technique that does not appear to be influenced by the presence of blood. Consequently, it can be applied in patients with upper gastrointestinal hemorrhage. The rapid urease test showed high sensitivity, specificity and PPV and should remain the first-line test in patients with upper gastrointestinal hemorrhage. HpSAT is appropriate as a second-line technique and is useful when the rapid urease test is negative and infection is strongly suspected, when no samples for the rapid urease test have been taken and when endoscopy cannot be performed. The result obtained in the present study should be confirmed in future studies with larger samples.

Hemorragia subepitelial de pelvis renal (lesión de Antopol-Goldman). Presentación de cuatro casos y revisión de la literatura / Subepithelial hemorrhage of the renal pelvis (Antopol-Goldman lesion). Four new cases and review of the literature

OBJETIVOS: El hematoma subepitelial de pelvis renal (Lesión de Antopol-Goldman) es una rara entidad que preferentemente se diagnostica en la clínica como una lesión neoplásica. MÉTODOS: Presentamos 4 casos nuevos de hematoma subepitelial de pelvis renal diagnosticados en nuestro hospital desde 1989. RESULTADOS: Nuestros casos se presentaron clínicamente por hematuria y dolor lumbar, preferentemente en el lado izquierdo (3 de 4 ). Tras nefrectomía, macroscópicamente se observó en todos los casos un hematoma subepitelial de extensión variable pudiendo llegar a ocupar la pelvis y cálices renales, asociándose a cambios histológicos de hidronefrosis, infarto cortical, renomegalia o angioma renal. Adicionalmente, dos de nuestros pacientes se presentaron por dilatación del sistema pielocalicial, y un tercero presentó carcinoma urotelial de vía urinaria inferior, siendo por tanto la lesión de Antopol-Goldman un hallazgo incidental. En el caso restante se identificó como causa del hematoma pélvico la presencia de hemangiomas renales múltiples. CONCLUSIONES: El diagnóstico preoperatorio de la lesión de Antopol-Goldman es difícil aunque debería incluirse como diagnóstico diferencial todos aquellos casos con hematuria y alteraciones de pelvis renal en las técnicas de imagen, pues su diagnóstico podría implicar un tratamiento conservador con pieloplastia o nefrectomia parcial. (AU)

Actinomycotic ulcer of the oral mucosa: an unusual presentation of oral actinomycosis.

Although the oral mucosa is often the site of entry of actinomyces into the deeper tissues, actinomycosis in the oral mucosa is extremely rare. Actinomycotic lesions are usually described as either single or multiple abscesses or indurated masses with hard fibrous walls and soft central loculations. Actinomyces israelii is the principal cause of human actinomycosis. We present a rare case of actinomycosis caused by Actinomyces odontolyticus; it presented primarily as a long-standing ulcer of the oral mucosa mimicking a squamous cell carcinoma.

Carcinoma epidermoide vesical de tipo verrucoso asociado a esquistosomiasis / Verrucous vesical epidermoid carcinoma associated with achistosomiasis

Introducción: Presentamos un caso de carcinoma vesical en un paciente con esquistosomiasis del mismo órgano. Esta neoplasia es una complicación tardía que afecta a pacientes con infestación crónica no tratada. Métodos y resultados: Un paciente inmigrante presentó hematuria macroscópica de tres años de evolución con polaquiuria y nicturia. La ecografía y urografía intravenosa mostraron un vejiga de baja capacidad y pared engrosada, con asimetría y rigidez parietal izquierdo del suelo. La TAC fue sugestiva de neoplasia infiltrante en cara anterior. Se realizó el diagnóstico histopatológico de carcinoma epidermoide de tipo verrucoso asociado a esquistosomiasis vesical y uretral. Comentario: La infestación vesical por esquistosoma haematobio es una entidad que, a pesar de ser poco frecuente en nuestro medio, hay que tener presente por el aumento de la inmigración y los viajeros procedentes de países endémicos, ya que no diagnosticarse temprano puede llevar a graves complicaciones y comprometer la vida del paciente (AU)

[Study of 13 cases of anisakiasis in the province of Cordoba]. / Estudio de 13 casos de anisakiasis en la provincia de Córdoba.

Anisakiasis, or anisakidosis, is a parasitic zoonosis due to the infestation by nematodes of the Anisakidae family, mainly by Anisakis simplex. Notwithstanding its world wide distribution, in our country its appearance is quite recent (1991) with only 19 cases previously reported. We refer 13 cases diagnosed in different hospitals in the province of Córdoba, Spain, from September 1994 to July 1998 which represents the biggest series described in Spain so far. All the patients had a clinical onset as acute abdomen, so that they required early surgery in which a narrowing and inflammatory intestinal segment was observed and subsequently resected. Pathology revealed in such segments an intense eosinophilic infiltrate in the mucosa. Only in one of the cases parasitic fragments were detected in the intestinal mucosa and in the 12 remaining cases the diagnosis was immunological by IgE specific for Anisakis simplex determination and antigens detection of the nematode with monoclonal antibodies. As interesting epidemiologic antecedent we shall mention the fact that all patients referred a usual raw fish consumption (mainly anchovy with vinegar) which is host of third-stage larval of the parasite.

[Subepithelial hemorrhage of renal pelvis (Antopol-Goldman lesion). Report of 4 cases and review of the literature]. / Hemorragia subepitelial de pelvis renal (lesión de Antopol-Goldman). Presentación de cuatro casos y revisión de la literatura.

OBJECTIVES: The subepithelial hematoma of renal pelvis (Lesion of Antopol-Goldman) is a rare entity that preferably is diagnosed clinically as a neoplasic lesion. METHODS: We present four new cases of subepithelial hematoma of renal pelvis diagnosed in our hospital from 1989. RESULTS: Our cases presented clinically with hematuria and flanc pain, preferably in the left side (3 out of 4). After nephrectomy, all the cases showed a subepithelial hematoma of variable extension that can occupy the renal pelvis and calices, associated to hidronefrosis, cortical infartion, renomegaly or renal angioma. Additionally, two of our patients presented with dilation of the pielocalicial system, and a third one presented with urotelial carcinoma of the ureter, being therefore the lesion of Antopol-Goldman an incidental discovery. In the remaining case, the presence of multiple renal hemangiomas was identified as cause of the renal pelvic hematoma. CONCLUSIONS: The preoperatore diagnosis of the lesion of Antopol-Goldman is difficult although it should be included as differential diagnosis in those cases with hematuria and alterations of renal pelvis in the image techniques, because an early diagnosis could imply a conservative treatment with pieloplastia or partial nephrectomy.