[Recommendations to develop Care Models for patients with Heart Failure (MAIC Project) from macromanagement]. / Recomendaciones para desarrollar Modelos Asistenciales de atención al paciente con Insuficiencia Cardiaca (Proyecto MAIC): Estudio Delphi.

BACKGROUND: The general objective of the study was to reflect on the key factors to advance in optimal models of care for Heart Failure (HF) and specifically, on the macromanagement elements most necessary for the development of comprehensive HF management models. MATERIAL AND METHODS: An Advisory Committee, composed of 15 experts and a multidisciplinary group of 31 additional experts, was appointed, together forming a Delphi panel of 46 experts. Based on a systematic bibliographic review and the analysis of the care course of the patient with HF, an initial battery of key factors for the development of HF care models was identified by the Advisory Committee. This proposal was adjusted and prioritized by the Delphi panel applying Delphi Rand/UCLA methodology. RESULTS: After two Delphi rounds, 75 key factors grouped into 7 challenges were defined. In the first of the challenges, related to the development of HF management models, 16 key factors were identified, 7 of which were valued as high priority and related to the establishment of common objectives, resources for the continuity of care and improving the measurement of health outcomes. CONCLUSIONS: The definition of management elements at the macro level was considered a priority to advance in the development of optimal models of assistance to HF.

Placental mesenchymal dysplasia associated with hepatic and pulmonary hamartoma.

This report describes a 31-week stillborn female infant with placental mesenchymal dysplasia (PMD) in association with hepatic mesenchymal hamartoma (HMH) and pulmonary hamartoma. Placental mesenchymal dysplasia was initially misdiagnosed as a partial mole. However, histologically, no trophoblastic proliferation or inclusions were observed. Differential diagnosis of the hepatic mass with similar tumors is discussed. To our knowledge, this is the first case of lung hamartoma reported in a fetus and the first case related to PMD and HMH. A common anomalous development of the mesoderm, a reparative post-injury process and a genetic mechanism, have been proposed to explain their pathogenesis.

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)

Primary extragastrointestinal stromal tumors in the omentum and mesentery: a clinicopathological and immunohistochemical study.

BACKGROUND/AIMS: This study aimed to describe the clinical, histological and immunohistochemical characteristics of primary extragastrointestinal stromal tumors (EGISTs) of the omentum and mesentery diagnosed in the Hospital 12 de Octubre, in Madrid, Spain, from 1993-2005. METHODOLOGY: The clinical data and histological and immunohistochemical findings of primary mesenchymal neoplasias were revised using the Department of Pathological Anatomy databases. RESULTS: Six EGISTs were identified. Three were primarily of the omentum and 3 mesenteric. They were found in 4 males and 2 females with an average age of 65.16 years. All were c-KIT positive, and the majority CD34 positive, while 3 were positive for muscle-specific actin. The 3 omentum cases had a mixed spindle/epithelioid pattern and low mitotic rate, while the 3 mesenteric cases had a spindle pattern, with a high mitotic rate in 2 cases, where hepatic metastasis appeared at 6 and 32 months respectively. The 3 omentum cases were alive at the time of writing, and free of disease at 16, 21 and 34 months of follow-up. EGISTs represent 11.9% of GIST cases diagnosed in the hospital over the period 2000-2005. CONCLUSIONS: In this study primary EGISTs of the omentum and mesentery showed clinicopathological and immunohistochemical characteristics similar to those previously in the literature for GISTs of the digestive tract, which supports the hypothesis that these tumors originate from extragastrointestinal c-KIT positive cells. Mesenteric location appears to be associated with a poorer prognosis.

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

["Teruel feet". Care and treatment of frostbite wounds in the hospitals of Navarra during the civil war]. / "Los pies de Teruel". Asistencia y tratamiento de las heridas por congelación en los hospitales navarros durante la guerra civil.

The care of combatants with lesions caused by frostbite during the battle of Teruel, which was fought in extreme weather conditions and in temperatures as low as twenty degrees below zero, was the period of greatest medical activity and the highest rates of occupation in the military hospitals of Navarre during the civil war of 1936-1939. From November 1937 to March 1938, 375 cases of frostbite were registered in the provincial establishments, amongst which there was a predominance of cases of dry gangrene partially affecting the lower extremity, which was popularly known as "Teruel feet". Some of the medical staff, conscious of the exceptional nature of the casuistry, registered statistics, clinical cases and personal impressions of the evolution of the lesions and the effectiveness of the treatments. In treating this affectation they employed medicines, surgical techniques and novel therapeutic procedures that were not widely used in the medical milieu of the time. However, the limited duration of the problem, the inconclusive results of the treatments and the differing opinions on their effectiveness -questions that are considered in this article- restricted the subsequent medical repercussion of the experiences of frostbite developed during the wartime period in Teruel.

'Los pies de Teruel'. Asistencia y tratamiento de las heridas por congelación en los hospitales navarros durante la guerra civil / 'Teruel feet'. Care and treatment of frostbite wounds in the hospitals of Navarra during the civil war

La atención a combatientes con lesiones por congelación sufridas durante la batalla de Teruel, desarrollada en condiciones meteorológicas extremas y temperaturas de hasta veinte grados bajo cero, supuso la etapa de mayor actividad asistencial y tasas de ocupación más altas en los hospitales militares navarros durante toda la contienda civil de 1936-1939. De noviembre de 1937 a marzo de 1938 se registraron en los establecimientos de la provincia más de 375 casos de congelación, entre los que predominaban las gangrenas secas con afectación parcial de la extremidad inferior, lo que popularmente se denominó 'pies de Teruel'. Algunos facultativos, conscientes de la excepcionalidad de la casuística, registraron estadísticas, casos clínicos e impresiones personales sobre la evolución de las lesiones y la efectividad de sus tratamientos. En ellos, emplearon fármacos, técnicas quirúrgicas y procedimientos terapéuticos novedosos en el tratamiento de esta afección y poco difundidos en el entorno médico del momento. Sin embargo, la limitación temporal del problema, los resultados poco concluyentes de los tratamientos y las opiniones controvertidas sobre su efectividad, cuestiones que se analizan en este artículo, limitaron la repercusión médica posterior de las experiencias sobre congelaciones desarrolladas durante la etapa bélica turolense

The care of combatants with lesions caused by frostbite during the battle of Teruel, which was fought in extreme weather conditions and in temperatures as low as twenty degrees below zero, was the period of greatest medical activity and the highest rates of occupation in the military hospitals of Navarre during the civil war of 1936-1939. From November 1937 to March 1938, 375 cases of frostbite were registered in the provincial establishments, amongst which there was a predominance of cases of dry gangrene partially affecting the lower extremity, which was popularly known as 'Teruel feet'. Some of the medical staff, conscious of the exceptional nature of the casuistry, registered statistics, clinical cases and personal impressions of the evolution of the lesions and the effectiveness of the treatments. In treating this affectation they employed medicines, surgical techniques and novel therapeutic procedures that were not widely used in the medical milieu of the time. However, the limited duration of the problem, the inconclusive results of the treatments and the differing opinions on their effectiveness –questions that are considered in this article– restricted the subsequent medical repercussion of the experiences of frostbite developed during the wartime period in Teruel

Focal hyperplastic hepatocellular nodules in hepatic venous outflow obstruction: a clinicopathological study of four patients and 24 nodules.

AIMS: In hepatic venous outflow obstruction (Budd-Chiari syndrome), focal hepatocellular nodules are occasionally discovered showing variable morphology. These could be interpreted either as neoplastic (adenoma), regenerative (large regenerative nodule) or reactive to abnormal vasculature (focal nodular hyperplasia). The aim of this study was to investigate their histogenesis and to determine their morphological characteristics in order to provide diagnostic criteria. MATERIAL AND METHODS: Twenty-four hepatocellular nodules were studied, which were found in three explanted livers and in one additional autopsied liver from four patients with Budd-Chiari syndrome. As controls, we employed three explanted livers without nodules from patients who also suffered from Budd-Chiari syndrome. We attempted to classify the nodules morphologically as either adenoma-like, large regenerative nodule or focal nodular hyperplasia-like, using criteria from the literature. RESULTS: Out of the four cases, we observed two nodules in each of two livers, five in the third one and up to 15 in the remaining one. The size of the nodules ranged from 4 to 25 mm. Eleven nodules could be categorized as large regenerative nodules (two of them with a central scar), seven as focal nodular hyperplasia-like and six as adenoma-like. Some large regenerative nodules showed proliferated arteries with muscular hyperplasia similar to that seen in focal nodular hyperplasia. In the individual livers we could find nodules of various categories. Patchy or diffuse monoacinar regeneration was seen in most cases (six out of seven cases) in the macroscopically non-nodular liver parenchyma. In addition, thrombotic obstruction of portal vein branches was present in all except one of the nodular cases, but in none of the controls. Thus, it appears that portal venous obstructions are frequently, but not invariably associated with the development of nodules. CONCLUSIONS: The hepatocellular nodules seen in livers from patients with Budd-Chiari syndrome share morphological characteristics with large regenerative nodules, focal nodular hyperplasia and hepatocellular adenomas. Their multiplicity, the existence of mixed lesions, the frequent hepatocellular regenerative background as well as the frequently associated portal venous obstructions suggest that these nodules are regenerative in nature and conditioned by an uneven blood perfusion throughout the liver. In their differential diagnosis, the clinicopathological context in which they occur is of paramount importance and should allow recognition that those resembling adenomas may not be true neoplasms.

Clinicopathological features of nine cases of non-cirrhotic portal hypertension: current definitions and criteria are inadequate.

AIMS: The clinicopathological features of nine patients with non-cirrhotic portal hypertension were studied and an attempt was made to apply the descriptive criteria of experts to the morphological alterations of the livers in order to classify them adequately. METHODS AND RESULTS: Clinical and biochemical data and the alterations in livers resected at transplantation (n=7) or at autopsy (n=2) were gathered in five males and four females (ages 15-78 years) without aetiological factors for chronic hepatic disease who had oesophageal varices and splenomegaly in the absence of typical cirrhosis. Noting the luminal obstruction of the three hepatic vascular trees, hyperplastic nodule size and distribution, and the density of fibrosis, an attempt was made to assign each case to one of the following diagnostic categories: idiopathic portal hypertension, diffuse nodular regenerative hyperplasia, partial nodular transformation and incomplete septal cirrhosis. When a case could not be categorized into one of these groups, it was listed as non-cirrhotic irregular architectural transformation. Only three cases could be assigned to one pure diagnostic category (two diffuse nodular regenerative hyperplasias and one incomplete septal cirrhosis). Three other cases could not be classified due to the heterogeneity of their lesions. In the remaining three cases, the hepatic morphology was a mixture of hilar partial nodular transformation combined with another abnormal architectural pattern in the peripheral parenchyma: diffuse nodular regenerative hyperplasia in two cases and idiopathic portal hypertension in the other. In seven cases, old thromboses in the hilar portal tree were observed. Stenoses were observed in some of the arterial branches in five cases and in some hepatic venous branches in four. However, no obstructions could be discovered in small or large portal veins in the two classical diffuse nodular regenerative hyperplasia cases. CONCLUSIONS: The hepatic morphology in this group of non-cirrhotic portal hypertension patients was an abnormal remodelling of the liver associated with the frequent development of irregular hyperplastic nodules and frequent obstructions of the pre- and intrahepatic vascular lumens. It was very difficult to apply the nomenclature proposed by international experts.

Enfermedad de Menetrier localizada en gemelos monocigóticos / Localized Menetrier´s disease in monozygotic twins

No disponible

Protocolo y guía para el diagnóstico histopatológico de hepatocarcinoma: subprotocolo de Anatomía Patológica para un protocolo general hospitalario de hepatocarcinoma / Protocol and guide for the histopathological diagnosis of hepatocarcinoma: Subprotocol of pathological anatomy for a general hospital protocol for hepatocarcinoma

No disponible

Dermatofibroma parasitized by Leishmania in HIV infection: a new morphologic expression of dermal Kala Azar in an immunodepressed patient.

Visceral leishmaniasis is a protozoan infection that may complicate the course of patients with human immunodeficiency virus (HIV). Dermatofibroma is a cutaneous fibrohistiocytic lesion considered neoplastic by some authors and inflammatory by others. Eruptive dermatofibromas have been described in patients with HIV infection or with other altered immunity situations. We present the case of a 32-year-old, HIV-positive man with visceral leishmaniasis who complained of the appearance of a cutaneous lesion in the leg formed by the coexistence of dermatofibroma and Leishmania parasitic colonization. As far as we know, this type of association has not been reported previously. We consider that the dermatofibroma could have developed as an unusual form of fibrohistiocytic reaction to leishmania. From a practical approach, we recommend the search of leishmaniasis in dermatofibroma in immunosuppressed patients.

Hepatitis C virus-related fibrosing cholestatic hepatitis after cardiac transplantation: is azathioprine a contributory factor?

We report a patient who acquired hepatitis C virus (HCV) infection at cardiac transplantation, developing fibrosing cholestatic hepatitis (FCH) with early liver failure and a fatal outcome. FCH is a recently described clinicopathological entity characterized by a cholestatic pattern of serum liver enzyme abnormalities, a progressive course leading to liver failure, and a pathological picture defined by periportal fibrosis, neutrophilic infiltrates and signs of histological cholestasis. Although it was initially described secondary to hepatitis B virus infection, it has also been recently related to HCV infection. Some histopathological features consistent with azathioprine hepatotoxicity like cholestasis, perisinusoidal fibrosis, veno-subocclusive lesions and nodular regenerative hyperplasia were also observed in this case. Therefore, a direct cytopathic effect of HCV and the concurrent pathogenic role of azathioprine hepatotoxicity may be involved in the development of this complication of transplantation.

Fibrosing cholestatic hepatitis in hepatitis C virus-infected renal transplant recipients.

Severe hepatitis C virus (HCV)-related fibrosing cholestatic hepatitis leading to early liver failure has been reported only exceptionally. Of 259 HCV-infected renal transplant (RT) patients in one hospital unit, four (1.5%) are described, representing the first series of this particular post-RT disease. Patient mean age was 55.7 yr. Three were men. All had pretransplant, hepatitis B surface antigen-negative and were anti-HCV antibodies positive. Three of them showed pretransplant mild liver enzyme abnormalities, and all received kidneys from HCV-negative donors. All were on steroids, cyclosporine, and azathioprine (AZA). The clinical pattern appeared early after RT (mean, 11.5 mo). In three patients, hyperbilirubinemia (6.5 to 20 mg/dl) and high alkaline phosphatase levels (428 to 859 IU/L) were observed. Also, in all subjects, high gamma glutamyl transpeptidase levels (639 to 4270 IU/L), mild aspartate aminotransferase and alanine aminotransferase abnormalities, and serum HCV RNA were observed. Liver biopsy revealed diffuse fibrosis, leukocyte infiltrates, and different degrees of cholestasis, with typical signs of HCV hepatitis in only one patient. Two patients developed subfulminant liver failure and died 2 and 3 mo after biopsy, respectively. One patient also suffered hepatic failure, receiving a liver transplant. The fourth is alive on dialysis awaiting a combined kidney and liver transplant. It is concluded that fibrosing cholestatic hepatitis is a new, early, and severe complication after RT in HCV(+) patients, which appears in patients with ongoing HCV infection under AZA therapy, despite a nonaggressive immunosuppressive protocol. Both HCV and AZA could play a concurrent role in the pathogenesis of this severe complication after RT.