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Ataxia telangiectasia (AT) is a rare neurodegenerative genetic disorder due to bi-allelic mutations in the Ataxia Telangiectasia Mutated (ATM) gene. The aim of this paper is to better define the immunological profile over time, the clinical immune-related manifestations at diagnosis and during follow-up, and to attempt a genotype–phenotype correlation of an Italian cohort of AT patients. Retrospective data of 69 AT patients diagnosed between December 1984 and November 2019 were collected from the database of the Italian Primary Immunodeficiency Network. Patients were classified at diagnosis as lymphopenic (Group A) or non-lymphopenic (Group B). Fifty eight out of 69 AT patients (84%) were genetically characterized and distinguished according to the type of mutations in truncating/truncating (TT; 27 patients), non-truncating (NT)/T (28 patients), and NT/NT (5 patients). In 3 patients, only one mutation was detected. Data on age at onset and at diagnosis, cellular and humoral compartment at diagnosis and follow-up, infectious diseases, signs of immune dysregulation, cancer, and survival were analyzed and compared to the genotype. Lymphopenia at diagnosis was related per se to earlier age at onset. Progressive reduction of cellular compartment occurred during the follow-up with a gradual reduction of T and B cell number. Most patients of Group A carried bi-allelic truncating mutations, had a more severe B cell lymphopenia, and a reduced life expectancy. A trend to higher frequency of interstitial lung disease, immune dysregulation, and malignancy was noted in Group B patients. Lymphopenia at the onset and the T/T genotype are associated with a worst clinical course. Several mechanisms may underlie the premature and progressive immune decline in AT subjects.

Highlights Confirmed animal poisoning cases were 215/497 (43.2%) from 2009 to 2018. Anticoagulant rodenticides (79.2%) were the main cause of animal poisoning. The most affected areas are located along the coast. Dogs and cats were 40.0% of confirmed animal poisoning., Intentional poisoning represents a serious risk to domestic and wild animals, and it can be an environmental and human health issue as well . This paper is a retrospective study, which covers a decade, based on animal poisoning cases and poisoned baits that were submitted for diagnostic examinations to the Veterinary Medical Research Institute for Piedmont, Liguria and the Aosta Valley (IZS-PLVA) in Liguria region. All data were collected through a passive surveillance system introduced in Italy by a decree of the Ministry of Health in January 2009. 43.2% of the animal poisoning cases were confirmed by toxicological analysis, whereas toxic agents were detected in 31.1% of the baits. The most affected animal species were dogs and cats, followed by synanthropic birds,. Only 4% of the total poisoning events analysed involved wild animals and cases of livestock poisoning were minimal. An increased number of cases in January, March, April and August was noticed, but no seasonal trend was detected. The most affected areas were the ones with the highest level of urbanization and population density. The major cause of the poisonings and the most common substances detected in the examined baits were anticoagulants whereas cholinesterase inhibitors, organochlorine pesticides and carbamates were detected in a minor number of cases. This study raises concerns about deliberate animal poisoning in ligurian region and highlights the necessity to fight this phenomenon as it endangers animals, humans and environment.

Background:Animal-Assisted Interventions (AAI) is the new way to indicate what was previously known as “Pet Therapy”, as activities can be done either with the conventional “pets” (dogs, cats and rabbits) or with horses and donkeys.Children with JIA have several problems in terms of adherence both due to the atavistic fear of the needle and due to nausea and vomiting - the most important side effects of Methotrexate – often since the 2-3 days before the assumption to immediately after it.Sure that animals can help children to forget this fear and to avoid the psycological conditions which enhance nausea, for the first time in Italy (and probably in Europe) it was designed a specific AAI program for these children.Objectives:To promote a general state of psycho-physical well-being in children and families about:manage of therapy; reduce discomfort and anxiety caused by entering hospital; improve self-esteem and the response to the stress generated by the execution of therapy and disease management; strengthen communication and socialization; stimulate the affective area through the activities of animal care.Methods:Dogs and cats are part of the recreational activities once a week in an equipped area in the OIRM Hospital (no alternative gateway was needed).Paediatric Rheumatologists selected two different groups of children: the first one (5 children in the pilot study) every 15 days; the second one (5 children) every month; the selection was made looking at the therapeutic scheme.Every session, one hour, has 3 clearly distinct stages:Welcome and organization: children say hello to dogs and cats, open the toolkits specifically designed for the intervention, express their state of mind and are encouraged to tell their own stories.Therapy:parents prepare and inject the drug to their children under medical or Health Professional control without discontinuation of the activities with animals.Play and socializing:children are involved in petting and other activities with animals; they are also involved in manipulative activities (design, puppets shows, modelling clay, animal care, ball retriving, etc). This step has the aim to relieve stress and discomfort due to medical procedures.Visual Analogic Scales (VAS) were part of the toolkit, to let the researchers evaluate the effects of the activity directly from the children experience.For the first time, we will control also the animal health status and wellness condition monitoring behavioural parameters and salivary cortisol level during each session.Results:The pilot project started in October 2019 and nowaday we closed 12 meetings, 4 on October, 4 on November, 3 on December and 2 on January, with the participation of 2 dogs (Golden and Labrador Retriever) and 1 cat (Devon Rex) in each one.All children love to play with animals, seek their closeness at the time of therapy and enjoy playing all together with the dogs; no one cry or refuse therapy and, since the third session, no one has nausea before, during or after the injection. Parents have reached a certain level of confidence: they stay quietly in the waiting room or go away to have a drink or to run an errand (it becomes a moment of relaxing for them too).Animals remain in healthy and wellness conditions during the activity.Conclusion:These preliminary data seem that AAI to be useful in helping patients in JIA to overcome some problems related to their pathology.Disclosure of Interests:UGO VIORA: None declared, Patrizia Ponzio: None declared, Maria Teresa Mascarino: None declared, Barbara Picco: None declared, Paolo Guiso: None declared, Eleonora Battista: None declared, Silvana Martino: None declared, Davide Montin Speakers bureau: Not relevant for the topic, Marta Dellepiane: None declared, Germana Rosso: None declared

Background:Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. Musculoskeletal ultrasound (MSUS) is a reliable tool in the assessment of chronic inflammatory arthropathies. MSUS in JIA has demonstrated a higher sensitivity for detecting synovitis and tenosynovitis as compared to physical examination. The occurrence of subclinical synovitis (Sub-S: MSUS+/ physical examination -) seems more frequent in wrist and foot joints; the clinical significance of Sub-S in real-life practice is still debated. Methotrexate (MTX) is the most widely used first-line DMARD in JIA therapy. Weekly treatment with MTX leads to clinical remission (CR) in 50-70% of patients. After a variable period of CR (usually 6-18 months), MTX is discontinued. Relapse rate after MTX suspension ranges between 40-50%; no predictors of disease flare have been identified so far.Objectives:We designed a cohort study in order to explore if MSUS monitoring during MTX tapering was able to predict disease flare.Methods:JIA patients in CR (as defined by the JADAS score) for at least 12 months were enrolled in the study. Patients at first attempt of suspension (G1) were tapered as follows: 1 week of suspension every 3 weeks for 3 months + 1 dose every 2 weeks for 3 months; if CR persisted, MTX was stopped. Patients who had a previous flare during/after MTX tapering (G2) had a similar tapering schedule but the step with 1 MTX dose every 2 weeks lasted 6 months. All patients underwent a complete MSUS of 48 joints every 3 months; clinicians who performed physical examinations and follow-up were blinded to US findings for the entire study period.Results:18 consecutive patients were enrolled between April 2018 and September 2019; patients had prevalently oligoJIA (55.5%) and RF- polyJIA (22.2%). Patients had been treated with MTX for 24.7 months (17.7–48.3), CR had been achieved 4.2 months after MTX start; 61.1% were at their first attempt of MTX tapering (G1).Baseline MSUS:at T0 MSUS detected 9/18 patients (50.0%) with Sub-S (MSUS+). Affected sites at T0 were distributed as follows: 4 MCP joints, 9 MTP joints, 1 f-IP joints, 11 knees. No significant differences resulted in comparing demographic and baseline disease features between MSUS- and MSUS+ patients at T0.Follow-up MSUS: 14 patients (77.8%) completed the entire study protocol, 4 patients are still ongoing. 7 patients relapsed: 42.9% during tapering, 1 of them relapsed during a VZV infection and was excluded from further analysis. We considered as Tlast-MSUS the last available MSUS before relapse or final MSUS (i.e. three months after MTX withdrawal) for not-relapsed subjects.At Tlast8 patients had at least 1 Sub-S. Sub-S per patient at Tlastwere more than Sub-S at T0 (2.85 vs 0.53 p=0.03) but the presence of Sub-S was not related with disease flare (50.0 vs 44.4% p=1). MSUS found 27 Sub-S of the small joints (sMSUS): 88.9% were in the feet, they had an OMERACT grading of 1. sMSUS+ patients were older (8.7 vs 3.9, p=0.002) therefore a weight-induced sub-S not related with JIA could be presumed.Kaplan-Meier curves were analyzed comparing MSUS results at T0 and Tlast, both considering all Sub-S and excluding small feet joints (pMSUS). The best performance was achieved with MSUS at Tlastand pMSUS (figure below, p=0.11).Conclusion:•Sub-S are present in 50% of patients in clinical remission >12 months.•Sub-S in older patients interest often feet small joints; these Sub-S may be of mechanical origin and are not associated with disease flare.•Sub-S increase during MTX tapering.Further patients must be enrolled to understand if Sub-S excluding feet small joints may predict disease flare.References:[1]Amendment of the OMERACT US definitions of joints’ features in healthy children using the DOPPLER technique. Collado P et al, Pediatr Rheumatol Online J. 2018.[2]Preliminary Definitions for the Sonographic Features of Synovitis in Children. Roth J, et al; Arthritis Care Res. 2017.Disclosure of Interests:Francesco Licciardi: None declared, Marta Dellepiane: None declared, Carlotta Covizzi: None declared, Fabiana Figus: None declared, Irene Azzolin: None declared, Davide Montin Speakers bureau: Not relevant for the topic, Annamaria Iagnocco Grant/research support from: Abbvie, MSD and Alfasigma, Consultant of: AbbVie, Abiogen, Alfasigma, Biogen, BMS, Celgene, Eli-Lilly, Janssen, MSD, Novartis, Sanofi and Sanofi Genzyme, Speakers bureau: AbbVie, Alfasigma, BMS, Eli-Lilly, Janssen, MSD, Novartis, Sanofi

Astute control of brain activity states is critical for adaptive behaviours and survival. In mammals and birds, electroencephalographic recordings reveal alternating states of wakefulness, slow wave sleep and paradoxical sleep (or rapid eye movement sleep). This control is profoundly impaired in narcolepsy with cataplexy, a disease resulting from the loss of orexin/hypocretin neurotransmitter signalling in the brain. Narcolepsy with cataplexy is characterized by irresistible bouts of sleep during the day, sleep fragmentation during the night and episodes of cataplexy, a sudden loss of muscle tone while awake and experiencing emotions. The neural mechanisms underlying cataplexy are unknown, but commonly thought to involve those of rapid eye movement-sleep atonia, and cataplexy typically is considered as a rapid eye movement sleep disorder. Here we reassess cataplexy in hypocretin (Hcrt, also known as orexin) gene knockout mice. Using a novel video/electroencephalogram double-blind scoring method, we show that cataplexy is not a state per se, as believed previously, but a dynamic, multi-phased process involving a reproducible progression of states. A knockout-specific state and a stereotypical paroxysmal event were introduced to account for signals and electroencephalogram spectral characteristics not seen in wild-type littermates. Cataplexy almost invariably started with a brief phase of wake-like electroencephalogram, followed by a phase featuring high-amplitude irregular theta oscillations, defining an activity profile distinct from paradoxical sleep, referred to as cataplexy-associated state and in the course of which 1.5-2 s high-amplitude, highly regular, hypersynchronous paroxysmal theta bursts (∼7 Hz) occurred. In contrast to cataplexy onset, exit from cataplexy did not show a predictable sequence of activities. Altogether, these data contradict the hypothesis that cataplexy is a state similar to paradoxical sleep, even if long cataplexies may evolve into paradoxical sleep. Although not exclusive to overt cataplexy, cataplexy-associated state and hypersynchronous paroxysmal theta activities are highly enriched during cataplexy in hypocretin/orexin knockout mice. Their occurrence in an independent narcolepsy mouse model, the orexin/ataxin 3 transgenic mouse, undergoing loss of orexin neurons, was confirmed. Importantly, we document for the first time similar paroxysmal theta hypersynchronies (∼4 Hz) during cataplexy in narcoleptic children. Lastly, we show by deep recordings in mice that the cataplexy-associated state and hypersynchronous paroxysmal theta activities are independent of hippocampal theta and involve the frontal cortex. Cataplexy hypersynchronous paroxysmal theta bursts may represent medial prefrontal activity, associated in humans and rodents with reward-driven motor impulse, planning and conflict monitoring.

The evolution of digitization technologies (2D and 3D) and the consequent wide availability of digital representations of artworks has ignited a number of works aimed at providing tools and platforms for archival, visual presentation, and integration of all those media. This chapter reviews some available instruments, focusing first on the platforms that could support the CH/DH professional in the single or cross-analysis of multimedia representations. Then, we discuss the available approaches for structuring and integrating the available data, presenting both the GIS-based solutions and the more recent Web-based systems. Some discussion on open issues concludes the contribution.

Concurrent chemoradiotherapy has become the standard of care for patients with inoperable squamous cell head and neck carcinoma. More recently, induction chemotherapy has been adopted as an approach in the management of these patients. We report the results of a phase II trial associating induction chemotherapy and concomitant chemoradiotherapy in a series of patients with inoperable squamous cell head and neck cancer. Twenty-nine patients with advanced squamous cell carcinoma ineligible for surgery were enrolled. Induction chemotherapy with docetaxel 75 mg/m(2) and cisplatin 75 mg/m(2) every 21 days was administered for two cycles. Radiotherapy followed the induction phase. During radiotherapy, docetaxel was administered weekly at the dose of 33 mg/m(2) . Primary end point of the study was feasibility of treatment. Six (18%) patients failed to conclude the treatment schedule. Although response rates in evaluable patients were very high (disease control rate >90%), toxicities were a matter of concern. The reported treatment schedule proved infeasible. However, some modifications in ancillary therapies aimed at exploiting its efficacy could make it practicable.

IntroductionThe Psychiatry Clinic Affektiva has developed a care pathway for patients with bipolar disorder (BD). It consists of one outpatient clinic and two inpatient wards. Clinical observations suggest that patients with BD benefit from treatment in a dedicated unit with tailored care programs and that better outcomes are achieved in terms of reduced average length of stay and safer transitions to outpatient care for this group.AimsThe aim is to improve the care of bipolar patients by increasing bed-availability and overall continuity in the care pathway and monitoring core aspects in the management of outpatients in order to reduce admissions and to facilitate standardized treatment and collaboration between in- and outpatients unit.MethodWe developed a structure to monitor and measure specific outcomes such as readmission within 28 days, relapse within 12 months and the use of lithium in this group. We also developed a standardized care protocol in order to improve the safety and the equality.ResultsAt this stage our preliminary results from our efforts are promising but further monitoring is needed to confirm our hypothesis. More data will be collected during 2017.ConclusionsAffektiva Psychiatry Clinic has developed a model for continuously monitoring several essential aspects in the care of patients with BD and providing a specific care program.Disclosure of interestThe authors have not supplied their declaration of competing interest.

A considerable proportion of the candidates for multichannel cochlear implantation consists of patients with profound bilateral hearing loss caused by sclerosing and/or ossifying labyrinthitis as a complication of meningitis. The spread of bacteria and viruses from the meninges to the inner ear is generally thought to be either via the subarachnoid sheaths following the nerves in the internal acoustic canal, or more directly through the cochlear aqueduct. The infective stage can be followed by fibrosis of the labyrinth and later by new bone formation which may proceed to almost complete obliteration of the labyrinth. Computed tomography remains the first examination to perform in order to have a precise map of the inner ear and temporal bone anatomy as well as to depict any abnormality of the round window or calcification of the membranous labyrinth. In many centres CT is the first imaging modality chosen in the evaluation of cochlear-implant candidates, providing higher spatial resolution and unique information on skeletal structures. Temporal bone MR examination adds useful information before surgery because only thin T2-weighted sequences may depict focal or diffuse obliteration of the membranous labyrinth fluid space before calcification with a high degree of sensitivity. Moreover, MR plays a role in planning surgery, because a remaining postmeningitis labyrinthitis enhancement seems to predict a rapid evolution towards labyrinthine calcification.

Different papers [1–5] suggest the existence of complex peripheral and, overall, central alterations in the vestibular system as a consequence of a trauma in the cervical portion. Thus we studied optokinetic and visual vestibular system behavior in subjects who previously underwent a cervical whiplash injury. The results of our research could be both interesting from a clinical point of view and useful for medicolegal goals. Our methods could be suitable for pointing out alterations in central sites as it is known that the nystagmus resulting from the contemporary optokinetic and roto-acceleration stimulation with counterdirectional nystagmus is significantly weakened in brain stem and cerebellar lesions and more widely in the presence of lesions in the posterior cranial fossa [6–9]. The visuo-vestibular interaction has not yet been adopted in the study of cervical whiplash injury.

The management, processing and visualization of color information is a critical subject in the context of the acquisition and visualization of real objects. Especially in the context of Cultural Heritage, artifacts are so complex or hard to handle that the appearance information has to be extracted from a set of images. The images usually have to be registered to the 3D model of the objects, in order to transfer the needed information. Hence, the problem of image-to-geometry registration has been thoroughly studied by the Computer Graphics and Computer Vision community. Several methods have been proposed, but a fully automatic and generic solution is still missing. Moreover, small misalignments often lead to visible artifacts in the final colored 3D models. In this paper, we propose a method to refine the alignment of a group of images which has been already registered to a 3D model. Taking advantage of the overlapping among the images, and applying a statistical global method based on Mutual Information, the registration error is distributed among all the elements of the dataset. Hence, the quality of color projection is improved, especially when dealing with small details. The method was tested on a number of heterogeneous Cultural Heritage objects, bringing to a visible improvement in the rendering quality. The method is fully automatic, and it does not need powerful hardware or long processing time. Hence, it represents a valid solution for a wide application on CH artifacts.

IntroductionThe MOODS-SR is a self-report instrument consisting of 161 dichotomous items. It is designed to assess lifetime presence of mood spectrum psychopathology. Recently, it has been proposed that a subset of 33 items can be used to distinguishing bipolar disorder.AimTo evaluate psychometric properties of a 33-item subset and to propose a clinically relevant cut-off for screening for bipolar disorder.MethodsPatients with mood disorders were recruited from outpatient services at Sahlgrenska University Hospital. Patients and a convenience sample of healthy controls were offered to fill in the MOODS-SR. A post-hoc analysis was conducted for the 33-items subset of the MOODS-SR.ResultsThe subset showed high internal consistency (Cronbach α = 0.95). The mean scores of patients with bipolar disorder (22.7 ± 6.4) were significantly higher than those of the unipolar (11.3 ± 4.9) and control group (7.0 ± 7.0, P < 0.005). A significant correlation was found between YMRS (r = 0.50, P < 0.005) and the 33-item subset, but not with MADRS (r = –0.22, P = 0.223).ConclusionThe 33-item subset of MOODS-SR showed promising psychometric properties, including good known-group validity. It discriminated bipolar patients from unipolar patients and healthy subjects. The clinical usefulness of these finding needs further investigation.Disclosure of interestThe authors have not supplied their declaration of competing interest.

Concurrent chemoradiotherapy has become the standard of care for patients with inoperable squamous cell head and neck carcinoma. More recently, induction chemotherapy has been adopted as an approach in the management of these patients. We report the results of a phase II trial associating induction chemotherapy and concomitant chemoradiotherapy in a series of patients with inoperable squamous cell head and neck cancer. Twenty-nine patients with advanced squamous cell carcinoma ineligible for surgery were enrolled. Induction chemotherapy with docetaxel 75 mg/m(2) and cisplatin 75 mg/m(2) every 21 days was administered for two cycles. Radiotherapy followed the induction phase. During radiotherapy, docetaxel was administered weekly at the dose of 33 mg/m(2) . Primary end point of the study was feasibility of treatment. Six (18%) patients failed to conclude the treatment schedule. Although response rates in evaluable patients were very high (disease control rate90%), toxicities were a matter of concern. The reported treatment schedule proved infeasible. However, some modifications in ancillary therapies aimed at exploiting its efficacy could make it practicable.

Introduction Mood Spectrum Self Report (MOODS-SR) is an innovative instrument to assess mood spectrum symptomatology. This may provide a better way of conceptualizing the psychopathology of affective disorders. Aim Psychometric evaluation of the Swedish adaptation of MOODS-SR, lifetime version. Methods Patients with affective disorders were recruited from outpatient services at Sahlgrenska University Hospital. Patients and a convenience sample of healthy controls were offered to fill in the MOODS-SR. This is a preliminary analysis of the collected data; data from more participants will be presented at the conference. Internal consistency was evaluated using Cronbach's α and the mean score on subdomains compared between groups using a Student's t-test in STATA. Results September 2014, 22 patients with affective disorders and 67 controls had answered the MOODS-SR. The age (mean ± sd) of patients was 39.8 ± 11.8 years compared to 40.7 ± 12.7 years of controls (p=0.79). The patient and the control group consisted of females by 73% and 69% respectively (p=0.33). Cronbach's α was 0.89 in patients and 0.90 in controls regarding the total score on the 7 subdomains; i) mood depressive, ii) mood manic, iii) energy depressive, iv) energy manic, v) cognition depressive, vi) cognition manic, vii) rhythmicity. The mean scores on the 7 subdomains were all statistically significantly higher among patients (p value Conclusion The Swedish MOODS-SR, lifetime version, has good internal consistency among both patients with affective disorders and controls. Furthermore, the patient group displayed consistently higher mean scores than the control group in all subdomains.

Alterations of the vestibulo-ocular reflex, optokinetic nystagmus, and visuo-vestibular-ocular reflex, have already been described in patients with AIDS and HIV-1 positive asymptomatic subjects. The introduction to the clinical practice of posturographic techniques allows us to study, with precision, postural perturbation that may be present when performing Romberg’s test and to study the vestibulo-spinal reflex as a component of the vestibular system. The relative lack of studies on posturography and AIDS, encouraged us to continue our research on the vestibular system both in asymptomatic HIV-1 seropositive patients and in patients with AIDS (IV stage according to the classification proposed by the Centre for Disease Control). Recordings were made in group 1 (control group, 55 normal subjects), in group 2 (15 asymptomatic HIV-positive subjects), and in group 3 (15 patients with AIDS stage IV). Static and dynamic posturography were carried out using Tonnies platform system (Tonnies GmbH & Co., Wurzburg, Germany) and the data were analysed with Tonnies Posturographic Tübingen (TPOST) software vers. 5.19. In asymptomatic HIV+ subjects, we observed an increase in RW, RA and M3 reflex latency. AIDS patients (stage IV) exhibited significant alterations in almost all the posturographic parameters and the electromyographic potentials. Our results validate static and dynamic posturography as a method for otoneurological investigation and appear to confirm that the entire vestibular system is involved since the earliest stages of the HIV infection. In the HIV+ subjects, a variable dysfunction in the reflex control to long latency was observed, which is correlated with the alteration of the central dopaminergic system; in AIDS patients, the central nervous system damage appears more important, globally distributed and correlated also with immunosuppression.

Although quality of life (QoL) has become an important aspect of cancer rehabilitation, psychometric studies on thyroid cancer patients are rare. We performed a case-controlled study on QoL in patients with differentiated thyroid carcinoma (DTC). QoL was evaluated in 61 patients with a history of DTC diagnosed from1 to 23 yr earlier. An undetectable thyroglobulin (Tg) level after recombinant human TSH (rhTSH) testing was considered the best predictor of cure. QoL was evaluated by means of a general psychiatric interview, the self-rating Kellner Symptoms Questionnaire (KSQ) and the Hamilton Depression Scale (HDS). QoL was also evaluated in a control group of subjects on L-T4 therapy with a non-toxic multinodular goiter diagnosed from1 to 25 yr earlier. DTC and control subjects were similar in age, male-female distribution and concomitant psychiatric therapies. Per-week dosage of L-T4 was higher in DTC patients than in controls (p0.01). In neither group of subjects was there any correlation between current TSH levels or interval from diagnosis and KSQ or HDS scores. Only in DTC patients was there a positive correlation between age and KSQ (p0.05) or HDS (p0.01) scores. There was a significant difference in overall KSQ scores between DTC (33.4 +/- 2.1) and control (24.5 +/- 1.9; p0.01) subjects. The subscales of KSQ showed a significant inter-group difference. HDS scores were higher in DTC subjects (35.8 +/- 1.0) than in controls (30.0 +/- 1.1; p0.01). HDS score was significantly (p = 0.02) higher in female than in male DTC patients. In patients with papillary carcinoma there was a positive correlation between the MACIS (metastases, age, completeness, invasiveness, size) score and KSQ (p = 0.01) or HDS (p0.01) scores. After rhTSH testing, detectable Tg levels were found in 13% of DTC patients. In Tg-positive patients, KSQ and HDS scores were not different from those of Tg-negative patients. After an 8-14 month period, a significant decrease in the KSQ scale somatization (p = 0.02) was found in a sub-set of 31 DTC patients. In conclusion, even in the age of rhTSH testing, DTC patients suffer an impairment of their QoL, as noted when short-term L-T4 withdrawal was the gold standard. Longitudinal evaluation seems to indicate a slight improvement in QoL when safe rhTSH testing is extensively used in the management of the disease.

Background: Restoration of intestinal continuity in patients with ileostomy after total colectomy or with colostomy after Hartmann's procedure is a major operation. Herein we illustrate the validity of gasless laparoscopically assisted reversal using abdominal wall lifting. Methods: The operation was performed on 10 patients from February 1997 to May 1999. Seven of them had a left iliac stoma after a Hartmann resection, and three had an ileostomy after total colectomy. Results: The laparoscopic reversal was completed in eight patients; the two others were converted to an open procedure. Three major complications occurred (30%). There were no deaths. The average operation time was 192 min (range, 125-265). Time of discharge from surgery averaged 9.5 days. Mean follow-up of these patients was 12 months and negative. Conclusions: Laparoscopically assisted ileo- or colorectal anastomosis without pneumoperitoneum and using a laparotenser can be considered for the reversal of patients with ileostomy or colostomy. Even taking the high rate of intraoperative or postoperative complications into consideration, the advantages that make such a laparoscopic approach suitable include reduced trauma related to a second major abdominal operation, reduced postoperative pain, and fewer cutaneous tissues exposed to bacterial contamination. Moreover, the use of a laparotenser makes it possible to operate on elderly patients with cardiovascular diseases. In the absence of pneumoperitoneum, it becomes possible to use traditional instruments, with a consequent reduction in costs.

We studied the behaviour of Vestibular Nystagmus (VOR), of Optokinetic Nystagmus (OKN) and of Visuo-Vestibular-Ocular-Reflex (VVOR) in seven normal subjects and in thirty-two patients who had undergone cervical trauma in an automobile accident with the so called "whiplash mechanism". Thirteen subjects underwent examination within the first three months after the accident (first group), six subjects between the third and the sixth months (third group). Ocular movements was recorded according to the usual method by means of a Tonnies electronystagmograph with eight channels. The subjects, head blocked, sat on a Tonnies rotatory chair Pro model which was placed in the middle of a rotatory cylindrical chamber 2 metres in diameter and 1.9 metres in height. The width internal area was covered with thirty-two black vertical contrast. The rotatory cylinder was lighted from above by a 100 W bulb and was driven by a direct current engine which turned it clockwise and counterclockwise up to 200 degrees/sec., maximum speed, with preset acceleration ranging from 1 degree to 2 degrees/sec. All the subjects underwent to Rotatory Vestibular Stimulation by Stop test from a constant angular velocity of 90 degrees/sec. with clockwise and counterclockwise rotation, "stare type" Optokinetic stimulation with a cylinder rotation velocity of 30 degrees/sec. for 60 seconds and to contemporary Rotatory Vestibular and Optokinetic Stimulation (VVOR) so that OKN was VOR counterdirectional. The results of our experience show a statistically significative mean gain decrease of VOR and VVOR nystagmus (beating OKN direction) calculated on the first three beats in the patients of the first group and a significative increase of OKN mean gain in all the patients of the three groups. Furthermore, in sixteen out of thirty-two patients (seven in the first group, two in the second and seven in the third) we observed (during VVOR examination, immediately after stop) a nystagmus beating VOR direction lasting from 3 to 15 seconds.

The aim of our retrospective study was to verify the results of surgical treatment of rectal cancer in a homogeneous case series, evaluating the various factors that can influence the prognosis and long-term results. The prognostic factors taken into consideration were: Duke's stage; grading; colloid component; location of tumour; type of surgical intervention; age; sex; duration of the symptoms; length of normal rectum below the lower border of the tumour correlated to stage and grading. One hundred and sixty-five patients were operated with a radical approach: 50 abdominoperineal resections (APR) and 115 sphincter-saving resections (SSR) were performed. There were 90 males and 75 females. The mean age was 63 years. Total survival was 61.7% after 5 years and 50% after 10 years. In our study neither the age nor the sex, duration of symptoms or location of the tumours proved to have an influence on survival; while Duke's state turned out to be decisive for survival; also the colloid tumour component proved to have a worse prognosis. The 5-10-year survival rate was respectively 53.6% and 49% in the APR and 65.7% and 50.9% in the SSR (p = n.s.). The data we have collected show that APR and SSR operations have analogous efficiency.

During sonographic (US) studies of the neck for the nodal staging of patients with laryngeal neoplasms, the authors observed that, especially in advanced cases, also the primary lesion could be imaged with this method. Therefore, US capabilities were investigated in assessing the local stage of laryngeal neoplasms; the results were compared with clinical and surgical findings. Fifty normal subjects of various ages were previously examined to assess the normal US anatomy of laryngeal structures. The presence of calcifications within the thyroid cartilage is the major obstacle to US imaging of the larynx and is directly related to age; indeed, only 40% of subjects can be examined at the age of 70. Seventeen patients with advanced laryngeal tumors were examined by US. Laryngeal imaging was possible, either in part or completely, in 16 of 17 cases. The results were in agreement with clinical staging in 14 cases; in 2 cases US proved clinical understaging by detecting lesion spread to anterolateral perilaryngeal structures. To conclude, US is not suggested as the method of choice in patients with laryngeal neoplasms. However, in subjects with advanced tumors, also US can image the lesion directly, and even detect tumor spread to surrounding tissues, especially in the anterior and lateral directions. In a few cases, this can contribute to preoperative staging and to select the correct therapeutic approach.

The use of intravenous immunoglobulins (IVIG) has provided a substantial therapeutic advance in the treatment of antibody deficiency syndromes improving the quality of life of the affected patients. Among the advantages there are the possibility to employ higher doses to reach higher serum levels, with a more efficacious prophylaxis against infections; the prevention of chronic lung disease if IVIG are prescribed early, at diagnosis or at least at the onset of symptoms; the significant improvement of pulmonary function. The IVIG which are likely to be the best choice are the "intact Fc" preparations. In these products, or at least in some preparations, all IgG subclasses are present, which are endowed with specific and differentiated functions. Another important requirement is the presence of specific antibodies (e.g. anti Mycoplasmas, anti Campylobacter, anti Echovirus, anti pyogenic bacteria). The benefits of the prophylactic use of IVIG in preterm and low birthweight infants to prevent neonatal and late-onset infections are widely accepted. Lastly, IVIG are employed successfully in some autoimmune diseases as PTI and immune cytopenias, myasthenia gravis, acute and chronic inflammatory demyelinating polyneuropathy, Kawasaki syndrome, childhood recurrent seizures, juvenile chronic arthritis.

This study was undertaken to determine the results of resection of hepatocellular carcinoma in cirrhotic patients in Europe, using the same criteria as in the Orient for detection, surgical management, and pathology of the tumors. Seventy-two patients had a liver resection. One- and 3-yr survival rates were 68% and 51%, respectively. Survival rate was significantly higher in Child's/Pugh's class A than in class B-C patients. Patients with a thickly encapsulated tumor lived longer than those with an infiltrating tumor and had in addition a significantly lower incidence of cancer recurrence. Class A patients with a thickly encapsulated hepatocellular carcinoma had a 77% 3-year survival rate. There was no relation between the size of the tumor or the presence of symptoms and survival. These data suggest that good results can be achieved by resection of hepatocellular carcinomas in European cirrhotic patients. A thickly encapsulated tumor and an adequate liver function are the main determinants of low cancer recurrence and high survival. The clinical results in this series are similar to those reported from the Orient.

The relative lack of data on the dynamic posturography led us to start a study in order to give our contributions to the standardization of M1, M2, M3, response parameters in normal subjects. Our research was carried out on 35 normal subjects aged 21 to 50. All of them were standing in Romberg's position on a Tönnies model board in a normally lit and ventilated room. We performed two tests: the first one open-eyed staring at no point, the second, 5 minutes later, closed-eyed. The EMG signals were obtained by surface electrodes on triceps sural and front tibial muscles. The EMG recording was determined by a "tilt" movement of the board at a steady speed of 50 per sec. and 4 wide. We use a XT 286 IBM computer with "T POST" software for checking and testing the data. Our results showed a significant variation in the value of the duration parameter in open-eyed and closed-eyed tests. Latency and area values were inferior to those obtained by other authors, except for Diener and Dichgans (3) whose results differ in latency value only.

SUMMARYA case of monochorial and monoamniotic pregnancy is described. Of the four products of conception, two were apparently normal (stillborn) and two were monsters.On the basis of current classifications, the latter two can be defined one as acardiacus acephalus and the other as acardiacus amorphus. After presenting the data resulting from all the investigations carried-out (radiography, sex chromatin, histological examinations, injection of the placental and foeto-placental circulation) the etiopathogenetical factors to be examined in determining congenital serious deformities are discussed in relation to the case under examination and on the basis of the most recent findings.Two etiopathogenetical hypotheses are then suggested, resting on the supposition of a genie lesion which directly conditions either the subdivision into several embryos following ontogenesis or the « monstruosity » of the placental circulation.On the basis of the results obtained it can be held that further discoveries on the subject will derive from genetics and experimental embryology.

Sixteen gastrectomized patients underwent surgical treatment for alkaline reflux gastritis by means of a Roux-en-Y loop duodenal diversion. Long-term evaluation of results was performed 5-9 years later. Ten patients (62.5%) showed good results, with absence of digestive symptoms and with an increase in body weight. Two patients (12.5%) had moderate results, with presence of sporadic and mild epigastric pain. Four patients (25%) had unsatisfactory results, with persistence of epigastric pain and absence of body weight increase. No patient had recurrent biliary vomiting or endoscopic evidence of endogastric biliary reflux. Among the six patients with moderate and unsatisfactory results, two had a significant alcoholic intake, two showed a high degree of anxiety on psychological assessment, and two had both factors. Alcoholism and psychological disturbances should be considered exclusion criteria when evaluating a gastrectomized patient for surgical cure of alkaline reflux gastritis.

A 60-yr old Italian woman presented with repeated gastrointestinal hemorrhages due to ruptured esophageal varices. No evidence of liver disease could be demonstrated by laboratory tests or by multiple liver biopsies. Angiography revealed a patent portal trunk and the presence of esophageal varices. A side-to-side portacaval shunt was performed, which caused the disappearance of the esophageal varices. There was no recurrent digestive hemorrhage during a 24-month follow-up. A mild deterioration of liver function was demonstrated by laboratory data 16 months after surgical operation and some mild episodes of hepatic encephalopathy occurred. This case can be considered an example of idiopathic portal hypertension, a rare pathological condition with a higher prevalence in certain geographical areas. Its etiology is not known. One important aspect of this entity is its potential to evaluate the effects of surgical portacaval shunt procedure in the absence of liver damage.

Mortality due to recurrent variceal esophageal bleeding secondary to portosystemic shunt thrombosis is high. Early diagnosis of shunt thrombosis is therefore necessary. For these reasons, patients who have undergone a portal diversion must be controlled periodically. To this end, frequent controls, using reliable, riskless and inexpensive methods are needed. In this work, 34 patients who underwent different types of portal systemic shunts were studied by ultrasonography. Diagnosis by ultrasonography (confirmed by radiography showing esophageal varices and sometimes by arteriography) was positive with direct vision of the anastomosis in 65 p. 100 of cases and with indirect signs of patency or thrombosis of the anastomosis in 32 p. 100 of cases. This method failed to conclude in 3 p. 100 of our cases.

Between the years 1981 and 1983 we treated with Ceftriaxone (Cx) 34 children--aged 15 days to 13 years--affected with serious infections: 18 infections of lower respiratory tract, 1 sepsis caused by E. Coli, 1 meningitis with cloudy cerebrospinal fluid, 1 submandibular adenitis with otitis, 1 otitis, 12 infections of the urinary tract caused by Proteus mirabilis, E. Coli, Klebsiella oxitocica and Klebsiella pneumoniae. Whenever bacteria were isolated by cultures, sensibility in vitro to Cx was tested. Cx was given i.m. or i.v. at a dose ranging from 50 to 135 mg/Kg/die according to the age and the seriousness of the infections; in 17 children Cx was administered once daily, in the other patients in two divided doses. The following laboratory measurements were obtained before, during and after treatment: complete blood cell count, platelet count, total bilirubin, creatinine, SGOT, SGPT, alkaline phosphatase and urinalysis. Patients were also monitored daily for clinical signs and symptoms such as fever, general conditions, heart rate, respiratory rate, blood pressure. Twenty children showed a good clinical response (1 sepsis, 1 otitis, 1 adenitis, 1 meningitis, 12 infections of the urinary tract, 4 infections of the lower respiratory tract); urine sterilization was achieved after three days of therapy in all patients with infections of the urinary tract. Remarkable clinical and radiological improvement in 9 patients with infections of lower respiratory tract was observed while in only 4 children with bronchopneumonia therapy was ineffective although the dosage of Cx was adeguate; in these patients a further antibiotic treatment was necessary for a complete recovery.(ABSTRACT TRUNCATED AT 250 WORDS)