Endoscopic Retrograde Cholangiopancreatography Study in Alagille''s Syndrome: First Report

Endoscopic retrograde cholangiopancreatography revealed the association between intra- and extra-hepatic biliary tree hypoplasia in an 8-year-old boy affected by Alagille's syndrome. The patient, in whom chronic cholestasis had been present from birth, presented characteristic facies, retarded growth, hepatomegaly, and splenomegaly. Chromosome studies were normal. HIDA Tc 99m appeared late in the gallbladder and emptied into the bowel after 3 h. Endoscopic retrograde cholangiopancreatography showed marked and diffuse narrowing of the extrahepatic biliary ducts and uniform narrowing of the intrahepatic ducts with reduced arborization. Menghini needle biopsy revealed no interlobular ducts in the portal tracts. The last two techniques confirmed the clinical picture and made surgical diagnosis unnecessary. This is, as far as we know, the first time endoscopic retrograde cholangiopancreatography has been used in the diagnosis of Alagille's syndrome.     

Outcome of Acute Monosymptomatic Optic Neuritis in Northern Sardinia,Italy

Purpose: To define the outcome of acute monosymptomatic optic neuritis (AMON) in Northern Sardinia, Italy. Methods: Records of all AMON patients admitted at the Institute of Ophthalmology, University of Sassari, between 1991 and 2002 were reviewed. The Kaplan-Meier method was used to calculate the cumulative probability of remaining free from clinically definite multiple sclerosis (CDMS). Results: 22 patients (16 women, 6 men) met the inclusion criteria. Mean age at onset was 29.4 years. Mean age-adjusted incidence was 0.91 per 100,000 population per year. Best-corrected visual acuity (BCVA) at presentation was < 20/200 in 12 (54.5%) patients. Brain MRI revealed evidence of two or more demyelinating lesions in all patients. Five (22.7%) eyes had final BCVA < 20/200. Fifteen (68.2%) patients developed CDMS within 7 years. The cumulative probability of remaining free from CDMS was 45% after 36 months and 26% after 82 months. Conclusions: Results suggest that Sardinian patients with AMON may have a worse visual prognosis and a higher risk of developing CDMS.     

Cognitive dysfunction and hepatitis C virus infection

Cognitive dysfunction in patients with chronic hepatitis C virus(HCV) infection is a distinct form of minimal hepatic encephalopathy(MHE). In fact, the majority of HCV-positive patients, irrespective of the grading of liver fibrosis, display alterations of verbal learning, attention, executive function, and memory when they are evaluated by suitable neuropsychological tests. Similarities between the cognitive dysfunction of HCV patients and MHE of patients with different etiologies are unclear. It is also unknown how the metabolic alterations of advanced liver diseases interact with the HCV-induced cognitive dysfunction, and whether these alterations are reversed by antiviral therapies. HCV replication in the brain may play a role in the pathogenesis of neuroinflammation. HCV-related brain dysfunction may be associated with white matter neuronal loss, alterations of association tracts and perfusion. It is unclear to what extent, in patients with cirrhosis, HCV triggers an irreversible neurodegenerative brain damage. New insights on this issue will be provided by longitudinal studies using the protocols established by the diagnostic and statistical manual of mental disorders fifth edition for cognitive disorders. The domains to be evaluated are complex attention; executive functions; learning and memory; perceptual motor functions; social cognition. These evaluations should be associated with fluorodeoxyglucose positron emission tomography and magnetic resonance imaging(MRI) protocols for major cognitive disorders including magnetic resonance spectroscopy, diffusion tensor imaging, magnetic resonance perfusion, and functional MRI. Also, the characteristics of portal hypertension, including the extent of liver blood flow and the type of portal shunts, should be evaluated.     

The Abscopal Effect in the Era of Cancer Immunotherapy: a Spontaneous Synergism Boosting Anti-tumor Immunity?

Radiotherapy is one of the main treatment strategies used in cancer. Aside from the local control of the disease, which is mediated by a direct cytotoxic effect on tumor cells, radiotherapy has also been shown to exert immune-mediated local and systemic effects. Radiotherapy can elicit anti-tumor responses in distant sites from the radiation field; this phenomenon is known as the abscopal effect and has been described in patients previously treated with immune checkpoint blockade (ICB). Considering that the efficacy of immunotherapy has been demonstrated only in a subset of patients—who often benefit with lasting responses—efforts are ongoing to potentiate its activity with the development of new combination strategies. Radiotherapy might represent a potential candidate for a synergistic combination with immunotherapy, by improving the immunogenicity of tumors and by enhancing local and systemic immune effects. This review aims to summarize the current pre-clinical and clinical data on the immune effects of radiotherapy and their potential implications for cancer immunotherapy.

Open image in new window

     

99mTc-tetrofosmin SPECT in solitary pulmonary nodule evaluation

A correct differential diagnosis between benign and malignant lesions is mandatory in patients with solitary pulmonary nodule (SPN). The aim of the present study was to investigate whether 99mTc-tetrofosmin SPECT may play a role in SPN evaluation. A consecutive series of 111 patients with an uncalcified 相似文献   

A device to select the optimal time to replace the pacemaker activated by a lithium-iodine battery: results of 8 years of experience

The discharge curve of lithium-iodine batteries does not havefeatures which make the clinical decision to replace the pacemakereasy. The output voltage fall corresponding to the end of theirlife is evidenced by variations of the output variables (frequencyand/or length of pulse) and the time of elective replacementtime (ER) is arbitrarily established by the manufacturer, ERmay be too early or too late in relation to the threshold valueof myocardial stimulation. Furthermore, the variables indicatingend of life may be changed by troubles in the circuit components,irrespective of the state of battery. A device to detect theoptimal time for replacement (OR) of pacemaker was studied in1692 subjects with implanted LEM VVl electro-stimulators. Thisdevice consists of a resistance of 110 connected in serieswith the catheter through a magnetic switch, which is activatedby placing a magnet over the skin. The transient connectionof the resistor reduces the output voltage by an amount dependingon the resistive load between the electrodes. In a follow-up of 35 000 months the OR device revealed a verygood reliability. Pacemaker replacement, established when stimulation becomesineffective during the application of the magnet or when thespike amplitude in the standard oscilloscope rapidly declined,was necessary in 77 patients (six of them were excluded dueto a circuit blackout). The OR device was useful in 53 patients(74.6%) with a chronic myocardial threshold of more than 2.5V, useless in 18 patients with a myocardial threshold lowerthan 2.5 V; to obviate this limitation a new OR device is describedfeigning a temporary increment of battery impedance. The employmentof the OR device rather than using ER enabled the patients togain 1333 months of stimulation. The gain of pacing varied froma few months to 3 years, with an average of 18.7 months.     

The cerebellar network: from structure to function and dynamics

Since the discoveries of Camillo Golgi and Ramón y Cajal, the precise cellular organization of the cerebellum has inspired major computational theories, which have then influenced the scientific thought not only on the cerebellar function but also on the brain as a whole. However, six major issues revealing a discrepancy between morphologically inspired hypothesis and function have emerged. (1) The cerebellar granular layer does not simply operate a simple combinatorial decorrelation of the inputs but performs more complex non-linear spatio-temporal transformations and is endowed with synaptic plasticity. (2) Transmission along the ascending axon and parallel fibers does not lead to beam formation but rather to vertical columns of activation. (3) The olivo-cerebellar loop could perform complex timing operations rather than error detection and teaching. (4) Purkinje cell firing dynamics are much more complex than for a linear integrator and include pacemaking, burst-pause discharges, and bistable states in response to mossy and climbing fiber synaptic inputs. (5) Long-term synaptic plasticity is far more complex than traditional parallel fiber LTD and involves also other cerebellar synapses. (6) Oscillation and resonance could set up coherent cycles of activity designing a functional geometry that goes far beyond pre-wired anatomical circuits. These observations clearly show that structure is not sufficient to explain function and that a precise knowledge on dynamics is critical to understand how the cerebellar circuit operates.     

Identification of Mycobacterium avium subsp. paratuberculosis in Biopsy Specimens from Patients with Crohn's Disease Identified by In Situ Hybridization

Crohn's disease is a chronic inflammatory disease of the gastrointestinal tract of unknown etiology. We report on the presence of cell wall-deficient Mycobacterium avium subsp. paratuberculosis in 35 of 48 paraffin-embedded tissue specimens from 33 patients with Crohn's disease by in situ hybridization with IS900 as a probe.