Resting state FMRI reveals diminished functional connectivity in a mouse model of amyloidosis.

INTRODUCTION: Functional connectivity (FC) studies have gained immense popularity in the evaluation of several neurological disorders, such as Alzheimer's disease (AD). AD is a complex disorder, characterised by several pathological features. The problem with FC studies in patients is that it is not straightforward to focus on a specific aspect of pathology. In the current study, resting state functional magnetic resonance imaging (rsfMRI) is applied in a mouse model of amyloidosis to assess the effects of amyloid pathology on FC in the mouse brain. METHODS: Nine APP/PS1 transgenic and nine wild-type mice (average age 18.9 months) were imaged on a 7T MRI system. The mice were anesthetized with medetomidine and rsfMRI data were acquired using a gradient echo EPI sequence. The data were analysed using a whole brain seed correlation analysis and interhemispheric FC was evaluated using a pairwise seed analysis. Qualitative histological analyses were performed to assess amyloid pathology, inflammation and synaptic deficits. RESULTS: The whole brain seed analysis revealed an overall decrease in FC in the brains of transgenic mice compared to wild-type mice. The results showed that interhemispheric FC was relatively preserved in the motor cortex of the transgenic mice, but decreased in the somatosensory cortex and the hippocampus when compared to the wild-type mice. The pairwise seed analysis confirmed these results. Histological analyses confirmed the presence of amyloid pathology, inflammation and synaptic deficits in the transgenic mice. CONCLUSIONS: In the current study, rsfMRI demonstrated decreased FC in APP/PS1 transgenic mice compared to wild-type mice in several brain regions. The APP/PS1 transgenic mice had advanced amyloid pathology across the brain, as well as inflammation and synaptic deficits surrounding the amyloid plaques. Future studies should longitudinally evaluate APP/PS1 transgenic mice and correlate the rsfMRI findings to specific stages of amyloid pathology.

Diffusion kurtosis imaging to detect amyloidosis in an APP/PS1 mouse model for Alzheimer's disease.

PURPOSE: Amyloid deposition in the brain is considered an initial event in the progression of Alzheimer's disease. We hypothesized that the presence of amyloid plaques in the brain of APP/presenilin 1 mice leads to higher diffusion kurtosis measures due to increased microstructural complexity. As such, our purpose was to provide an in vivo proof of principle for detection of amyloidosis by diffusion kurtosis imaging (DKI). METHODS: APPKM670/671NL /presenilin 1 L166P mice (n = 5) and wild-type littermates (n = 5) underwent DKI at the age of 16 months. Averaged diffusion and diffusion kurtosis parameters were obtained for multiple regions (hippocampus-cortex-thalamus-cerebellum). After DKI, mice were sacrificed for amyloid staining. RESULTS: Histograms of the frequency distribution of the DKI parameters tended to shift to higher values. After normalization of absolute values to the cerebellum, a nearly plaque-free region, mean, radial, and axial diffusion kurtosis were significantly higher in APP/presenilin 1 mice as compared to wild-type in the cortex and thalamus, regions demonstrating substantial amyloid staining. CONCLUSION: The current study, although small-scale, suggests increased DKI metrics, in the absence of alterations in diffusion tensor imaging metrics in the cortex and thalamus of APP/presenilin 1 mice with established amyloidosis. These results warrant further investigations on the potential of DKI as a sensitive marker for Alzheimer's disease.

[Metastatic Basal cell carcinoma in the axilla: reconstruction with a lateral pectoral island flap]. / Carcinoma basocelular metastásico en la axila: reconstrucción mediante un colgajo en isla pectoral lateral.

Although basal cell carcinoma (BCC) is one of the most common forms of cancer worldwide, it rarely occurs in the axilla. Only 31 cases have been reported in the literature. The incidence of metastatic BCC, particularly in areas not exposed to the sun, is very low. We present a new case of axillary BCC with lymph node metastases and the results of an extensive review of cases previously reported in the literature. BCC in the axilla is rare and metastasis is exceptional. Factors other than UV radiation probably contribute to its development. The lateral pectoral island flap was used for surgical closure. This method is useful for the reconstruction of axillary defects, obtaining excellent cosmetic and functional results. This flap should therefore be considered for the repair of large surgical defects in the axilla.

[Home parenteral nutrition registry in Spain for the year 2010: NADYA-SENPE Group]. / Registro del año 2010 de nutrición parenteral domiciliaria en España: Grupo NADYA-SENPE.

OBJECTIVES: To report the Group Registry NADYASENPE data about home parenteral nutrition (HPN) in Spain in 2010. MATERIAL AND METHODS: A descriptive study of the database of the national registry of HPN of NADYA-SENPE (December 10, 2009 to December 10, 2010). For the calculation of prevalence the latest data published by the Institute National Statistics Office (01/01/2009) was used. RESULTS: There were registered 148 patients from 23 hospitals, 86 women (58.11%) and 9 children (6.08%). The average age of the 139 patients older than 14 years was 53.06 ± 15.41 years. The average duration of HPN was 316.97 days/patient. The most common diagnosis in those younger than 14 years was short bowel traumatic with 5 cases (55.55%) and in those older than 14 years, palliative care cancer with 29 cases (19.59%). The reason for the indication for HPN was short bowel syndrome in 74 cases (47%). The access via most frequently recorded was tunneled catheter in 36 cases (22.78%) followed by implanted port-catheters in 13 cases (8.23%) and other pathways in 3 cases (1.90%). There were 23 catheterrelated infections (82.14%) which represented 0.49 /1,000 days of PN, all of which occurred in cases older than 14 years. During the year 24 episodes of HPN ended, the most frequent cause was the transition to oral nutrition in 12 episodes (50%). It was reported that patients had a normal activity in 70 episodes of HPN (44.30%) with complete autonomy in 88 episodes (55.69%). Some patients 39 (24.68%) were potential candidates for intestinal transplantation. CONCLUSIONS: The number of registered patients is slightly lower than the previous year, although the number of participating hospitals is the same. The most frequent complication remains catheter-related infection but its incidence has decreased from previous years, presenting the lowest rate since the creation of the record. Differences in participation in the registry observed in the Autonomous Communities causes the development of implementation strategies. There is a gradual increase in day length of HPN over the years, which suggests the chronic treatments of some patients and obliges to study the existence of a possible confounding factor, in case there is an oversight of closing an episode. Therefore, it is necessary to update the registry with warning systems that facilitate periodic review of the patients and optimize the validity of registration.

Wegener's granulomatosis: a new entity in the growing differential diagnosis of Degos' disease.

Wegener's granulomatosis (WG) is a multisystemic vasculitis, with skin involvement in 14% of cases and with palpable purpura, subcutaneous nodules and necrotic papules as the common features.(1) We present a patient diagnosed with WG who had multiple whitish papules similar to those of malignant atrophic papulosis (Degos' disease), which appeared during a flare of his disease. Lesions of malignant atrophic papulosis are said to be pathognomonic; nevertheless, various diseases with similar clinical lesions have been described. To our knowledge, this is the first reported case of such lesions in a patient with WG, and we suggest WG should be included in the differential diagnosis of Degos' disease.

Esthesioneuroblastoma recurrence presenting as a syndrome of inappropriate antidiuretic hormone secretion.

BACKGROUND: Esthesioneuroblastoma is an uncommon intranasal tumor. These neuroendocrine neoplasms are rarely associated with excess hormone syndromes, and only nine cases of inappropriate antidiuretic hormone syndrome (SIADH) secondary to an esthesioneuroblastoma have been described. In all these cases, electrolyte abnormalities were seen when the tumor initially developed. We report a unique case of esthesioneuroblastoma recurrence manifesting as SIADH as the solely presenting feature. METHODS AND RESULTS: A 34-year-old woman was referred to us for evaluation for hyponatremia. She had undergone resection of an esthesioneuroblastoma at age 18 with radiotherapy. The patient had undergone annual CT of the nasal area and was considered to be disease free. The study of the hyponatremia was consistent with a SIADH, and MRI revealed an intranasal mass. The resection of the tumor reversed the hyponatremia, and pathologic analysis revealed a recurrence of esthesioneuroblastoma. CONCLUSIONS: Biochemical analysis should be performed in the follow-up of patients with esthesioneuroblastoma. In our case, the biochemical abnormality led to the diagnosis of the recurrence.

Caracterización del factor de transferencia a partir de leucocitos no inducidos con virus Sendai / Characterization of the transfer factor obtained from Sendai virus non-induced leucocytes

El uso extensivo en la práctica clínica del factor de transferencia (FT) se ha visto limitado por aspectos relacionados con su caracterización bioquímica y la garantía de seguridad, pues este producto debre cumplir con las regulaciones que aseguran la inocuidad de los productos hemoderivados. El hecho de que su proceso de obtención dependa del procesamiento de donaciones de sangre con menos de 24 horas de almacenamiento limita su disponibilidad. Con el objetivo de incrementar la cantidad de unidades de FT disponibles para el tratamiento clínico, se realizó la caracterización de varios lotes de FT obtenidos de donaciones de hasta 48 horas; el producto fue pasteurizado, como garantía de la inactivación de posibles contaminantes virales. La evaluación de sus propiedades bioquímicas y de sus efectos biológicos permitió demostrar la identidad y potencia de este producto. Se demostró además su inocuidad mediante ensayos preclínicos de tolerancia local y toxicidad.

Loss of H-cadherin protein expression in human non-small cell lung cancer is associated with tumorigenicity.

Abnormalities in the H-cadherin gene have been described in several human cancers. However, their biological significance remains undetermined. To investigate the role of H-cadherin in non-small cell lung cancer (NSCLC), a chimera H-cadherin-green fluorescent protein (GFP) expressed in Cos-7 cells was used to identify an anti-H- cadherin antibody, HCD-1. Western blot analysis was performed in six NSCLC cell lines and 35 pairs of primary NSCLC tumors and nonmalignant lung tissue obtained from surgical resections using HCD-1. Loss of H-cadherin expression was seen in five (83%) of the six NSCLC cell lines, whereas loss of E-cadherin was seen in three (50%) of the six. H-cadherin expression was lost in 15 (43%) of 35 NSCLC surgical tumor specimens, whereas E-cadherin expression was lost in 6 (17%) of 35. H-cadherin was expressed in all of the nonmalignant lung tissue from all of the surgical specimens. Fourteen of 35 tumors were heterotransplanted s.c. in nude mice. Tumorigenicity in nude mice was associated with both loss of H-cadherin expression (P = 0.03) and loss of E-cadherin expression (P = 0.05). Loss of H-cadherin was also associated with a more advanced local tumor growth, although the difference was not significant. The results indicate that loss of H-cadherin is frequent in human NSCLC and suggest that it facilitates the implantation and local growth of human NSCLC tumors.

Eosofilia elevada como manifesticiones viscerales: VI. Infección por larva de nemátodo de 427 µ / High eosinophilia with visceral manifestations: VI. Nematode's larva infection of 427 µ

Se discute el estado actual del conocimiento sobre los cuadros clínicos de eosinofilia por helmintiasis parenteral, quedando aquí englobados los síndromes de Löeffler, eosinofilia tropical, granulomatosis larvaria, endoftalmitis por nemátodos y de Friess-Pierrou. Se revisan los posibles diagnósticos etiológicos en estos pacientes. Se presenta el caso de una niña de 18 meses de edad con gran eosinofilia y hepatomegalia, en la que se encontró una larva de 427 µ de largo en el hígado. Se determinó que la larva no corresponde a un parásito habitual del hombre ni a parasitos de animales que ya se hayan encontrado en el hombre. Se opinó que tal larva puede ser un nemátodo parásito de plantas o de insectos, o quizá del género Logibucca, parásito de serpientes y murciélagos. Sea cual sea su posición taxonómica, agranda enormemente las posibilidades etiológicas del síndrome aquí estudiado

[Disseminated peritoneal leiomyomatosis with malignant degeneration. A case report]. / Leiomiomatosis peritoneal diseminada con degeneración maligna. Informe de un caso.

Leiomyomatosis peritonealis disseminata (LPD), is a rare condition of unknown cause, characterized by the development of numerous nodules throughout the peritoneal cavity which appear during reproductive age, especially with pregnancy or associated with prolonged exposure to oral contraceptive agents. Histologically the nodules have the appearance of benign leiomyomata. LPD is thought to have originated from metaplasia of submesothelial multipotential mesenchymal cells. The treatment of the this disorder has been surgical castration, presumably to remove the hormonal stimulus necessary for tumor growth. Only two cases of malignant leiomyomatosis peritonealis disseminata have been reported. We report one more case of LPD with malignant degeneration which appears to be extremely rare.