A new protocol for assessing action observation and imitation abilities in children with Developmental Coordination Disorder: A feasibility and reliability study.

AIMS: To develop a new protocol for the assessment of action observation (AO) abilities and imitation of meaningful and non-meaningful gestures, to examine its psychometric properties in children with DCD and typically developing (TD) children. BACKGROUND: For learning manual skills, AO and imitation are considered fundamental abilities. Knowledge about these modalities in children with DCD is scarce and an assessment protocol is lacking. METHOD: The protocol consists of 2 tests. The AO test consists of two assembly tasks. The imitation test includes 12 meaningful and 20 non-meaningful gestures. Items of both tests are rated on a 4-point scale. Twelve children with DCD (mean age 8y3m, SD, 1.30) and 11 TD children (mean age 8y2m, SD 1.52) were enrolled. For inter-rater reliability, intraclass correlation coefficients (ICC) were calculated for the total score, weighted kappa and percentage agreement for single items. Known group validity was assessed by comparison of DCD and TD group (Wilcoxon rank sum test). For construct validity, the mABC-2 test was used. The protocol was adapted and confirmed by an intra and inter-rater reliability study (new sample of 11 DCD children, mean age 7y5m, SD 1.37). RESULTS: Excellent ICCs were reported for intra and inter-rater reliability for the final protocol. A significant difference between DCD and TD group was found for AO abilities (p < .01), for nonmeaningful gestures (p < .001). A significant correlation was reported between the AO test and the mABC-2 test (r = 56;p ≤0.0001). No significant correlations were revealed for the imitation tests. DISCUSSION AND CONCLUSION: The results support the psychometric properties of this protocol. When fully validated, it may contribute to map the deficits in AO abilities and imitation, to evaluate treatment effects of imitation and AO interventions.

Rationality in prophylactic central neck dissection in clinically node-negative (cN0) papillary thyroid carcinoma: Is there anything more to say? A decade experience in a single-center.

AIM: Papillary thyroid carcinoma (PTC) is the most common thyroid malignancy. Despite its extremely favorable prognosis, cervical lymph node metastases are a common feature of PTC and a known independent risk factor for local recurrence. However, the role of prophylactic central neck dissection (PCND) remains a matter of debate in patients with clinically node-negative (cN0) PTC. To better clarify the current role of PCND in the surgical treatment of PTC, evaluating advantages and disadvantages of PCND and outcome of cN0 PTC patients who have been treated with either total thyroidectomy alone or in combination with PCND. A review of recent literature data is performed. METHODS: Between January 2000 and December 2015, 186 consecutive patients with cN0 PTC were identified to be included in the present study. 74 of these underwent total thyroidectomy associated with PCND, while 112 patients underwent total thyroidectomy alone. The epidemiological and clinical-pathological data of all patients included were collected at diagnosis and during follow-up. RESULTS: Overall complication rate was significantly higher in the group of patients undergoing PCND (39.2% vs. 17.8%, p = 0.0006). To be specific, they presented a considerably increased risk of temporary recurrent laryngeal nerve injury (p = 0.009) and of permanent hypothyroidism (p = 0.016). Overall survival and recurrence rates did not differ between those undergoing PCND and those undergoing total thyroidectomy alone (p = 1.000 and p = 0.715, respectively). CONCLUSIONS: The results of the present study do not support the routine use of PCND in the treatment of cN0 PTC patients.

Detection of bone erosions in early rheumatoid arthritis: 3D ultrasonography versus computed tomography.

Three-dimensional (3D) volumetric ultrasonography (US) is an interesting tool that could improve the traditional approach to musculoskeletal US in rheumatology, due to its virtual operator independence and reduced examination time. The aim of this study was to investigate the performance of 3DUS in the detection of bone erosions in hand and wrist joints of early rheumatoid arthritis (ERA) patients, with computed tomography (CT) as the reference method. Twenty ERA patients without erosions on standard radiography of hands and wrists underwent 3DUS and CT evaluation of eleven joints: radiocarpal, intercarpal, ulnocarpal, second to fifth metacarpo-phalangeal (MCP), and second to fifth proximal interphalangeal (PIP) joints of dominant hand. Eleven (55.0%) patients were erosive with CT and ten of them were erosive also at 3DUS evaluation. In five patients, 3DUS identified cortical breaks that were not erosions at CT evaluation. Considering CT as the gold standard to identify erosive patients, the 3DUS sensitivity, specificity, PPV, and NPV were 0.9, 0.55, 0.71, and 0.83, respectively. A total of 32 erosions were detected with CT, 15 of them were also observed at the same sites with 3DUS, whereas 17 were not seen on 3DUS evaluation. The majority of these 3DUS false-negative erosions were in the wrist joints. Furthermore, 18 erosions recorded by 3DUS were false positive. The majority of these 3DUS false-positive erosions were located at PIP joints. This study underlines the limits of 3DUS in detecting individual bone erosion, mostly at the wrist, despite the good sensitivity in identifying erosive patients.

The superior horizontal pancreatic artery of Popova: a review and an anatomoradiological study of an important morphological variant of the pancreatica magna artery.

PURPOSE: The superior horizontal pancreatic artery was described in 1910, and after a few years, it was forgot by most investigators. This research is aimed to revive the description of this artery, describing course, pattern of branching and frequency. METHODS: More than 1,000 of angiographies including studies of the superior mesenteric artery, celiac trunk and its branches, were selected from the angiographic archives of the ex-institutes of Radiology of Siena, Rome (University of Sacro Cuore) and Perugia, and the arterial anatomy of the pancreas was studied. RESULTS: A pancreatic branch of the splenic artery running along the superior border of the pancreatic body and tail was observed in 25.93% of cases. This branch matched the description of the superior horizontal pancreatic artery and, when existing, replaced the pancreatica magna artery. For this reason, we considered the superior horizontal pancreatic artery as a variant of the pancreatica magna artery. Variable in caliber and importance, in most cases the superior horizontal pancreatic artery gave off descending branches that anastomosed with the inferior pancreatic artery. CONCLUSIONS: A superior horizontal pancreatic artery could be visualized more easily by selective angiography of the splenic artery. When coupled with the inferior pancreatic artery, the presence of the superior horizontal pancreatic artery outlined a longitudinally arranged pattern of blood supply of the distal pancreas that should be known. In particular circumstances, extended resections of the gland cutting both longitudinal arteries might jeopardize the surviving of the pancreas remnant.

A techno-economic comparison of fluidized bed gasification of two mixed plastic wastes.

A comparison between the most promising design configurations for the industrial application of gasification based, plastics-to-energy cogenerators in the 2-6 MWe range is presented. A pilot scale bubbling fluidized bed air gasifier, having a feeding capacity of 100 kg/h, provided experimental data: the syngas complete composition, the characterization of the bed material, the entrained fines collected at the cyclone and the purge material from the scrubber. Mass and energy balances and material and substance flow analyses have been therefore drawn to assess and compare design solutions utilizing two mixed plastic wastes (MPW) obtained from separate collection of plastic packaging, after different levels of pre-treatments. The related techno-economic performances have been finally estimated on the basis of the manufacturer's specifications. The study concludes that the MPW obtained after a very simple pre-treatment and fed to a gasifier coupled with a steam turbine is the solution that currently offers the higher reliability and provides the higher internal rate of return for the investigated range of electrical energy production.

Supported noble metal catalysts in the catalytic wet air oxidation of industrial wastewaters and sewage sludges.

This paper reviews some catalytic wet air oxidation (CWAO) investigations of industrial wastewaters over platinum and ruthenium catalysts supported on TiO2 and ZrO2 formulated to be active and resistant to leaching, with particular focus on the stability of the catalyst. Catalyst recycling experiments were performed in batch reactors and long-term stability tests were conducted in trickle-bed reactors. The catalyst did not leach upon treatment of Kraft bleaching plant and olive oil mill effluents, and could be either recycled or used for long periods of time in continuous reactors. Conversely, these catalysts were rapidly leached when used to treat effluents from the production of polymeric membranes containing N,N-dimethylformamide. The intermediate formation of amines, such as dimethylamine and methylamine with a high complexing capacity for the metal, was shown to be responsible for the metal leaching. These heterogeneous catalysts also deactivated upon CWAO of sewage sludges due to the adsorption of the solid organic matter. Pre-sonication of the sludge to disintegrate the flocs and improve solubility was inefficient.

[Headache as atypical presentation of celiac disease: report of a clinical case]. / La cefalea quale presentazione atipica di malattia celiaca: descrizione di un caso clinico.

Coeliac disease (CD) is a gluten dependent enteropathy with genetic predisposition. The introduction of the gluten with the diet leads to a damage of the intestinal mucosa losing the ability of absorption. Together with the "classic forms", in wich the intestinal symptomatology is prevalent, there are atypical forms, with unusual clinical presentation and silent forms with no clinical symptoms. The neurologic symptoms are not frequent and regard seizures, headache, ataxia and psychiatric problems. We report on a patient with headache since 3 years of age in which the headache the only manifestation of CD. The diagnosis of CD was made at 11 years, when he came at our observation for episodes of headache. Also the older sister is found affected by CD. After three months of gluten free diet, it was obtained the complete resolution of the headache. Also if the pathogenesis of the headache in patient with CD is unknown we think that a autoimmune, vascular or blood flow mechanism could be ipotizeable.

[Bone metabolism markers in thalassemia]. / Markers del metabolismo osseo nella talassemia.

The A.A. performed a screening on 113 patients affected by beta-thalassemia major ranging in age between 2 and 40 years, randomized among those which come to the Microcitemic Center of our Institute, and in a control group. In everybody, serum levels of calcium, phosphate, parathyroid hormone (PTH), calcitonin and 25-OH vitamin D were measured. Average serum levels of PTH were significantly (P < 0.001) lower in patients than controls and 12.4% of them were clearly under normal range, especially in the group above 16 years of age. Also serum levels of 25-OH vitamin D were lower in thalassemic subjects than in controls, because of the presence of 32 patients with values under normal limit. Our results are in agreement with current literature that underline the increasing incidence of endocrine complications in thalassemic patients which undergo to high transfusion regimens, owing to the increase of emosiderosis due to the low compliance to iron chelation therapy. Controversial is the pathogenesis of the absence of hypocalcemia in many patients with hypoparathyroidism and the determinism of the deficit of vitamin D.

Effect of light on hypericins contents in fresh flowering top parts and in an extract of St. John's Wort (Hypericum perforatum).

St. John's Wort is a medicinal plant increasingly used for its antidepressive activity. Hypericins are considered as one of the compounds contributing to the activity of the extract. These naphthodianthrones exist in various forms in Hyperici herba. Protopseudohypericin and protohypericin (protopigments) are converted into pseudohypericin and hypericin (pigments) under the action of light. The aim of this work is to study the influence of light on the phototransformation of protopigments into pigments. Two experiments were carried out. The studies were performed on one hand, on plant material in order to know the proportion of these substances in various plant parts and the possibility of transforming the protopigments into pigments under the action of sunlight; on the other hand, in the extract to determine the optimal wavelength allowing this transformation. Three parts of the fresh plant (buds, flowers, leaves) were treated with sunlight on three levels of exposure. Liquid extracts were exposed to various types of light with wavelengths ranging between 480 and 660 nm by means of diodes. The flowering tops of St. John's Wort contain a share of approximately 30% hypericins in the form of protopseudohypericin and protohypericin: buds (48%), flowers (30%), leaves (17%). After an exposure of fresh buds to sunlight for 16 hours the share of protopigments was then 32%. In the extract, the transformation of the protopigments is total and requires less energy than in the plant material. The optimal wavelength for the transformation of the protopigments in the extract is around 515 nm (green), close to the optimum absorption level of protopigments.

Craniocervical junction involvement in rheumatoid arthritis: a clinical and radiological study.

OBJECTIVE: To make a comparative evaluation of different imaging techniques for studying the craniocervical junction involvement in patients with rheumatoid arthritis (RA). Upper cervical spine involvement was compared with clinical and immunological data. METHODS: Patients (n = 47) underwent plain radiographs and computerized tomography (CT) and magnetic resonance (MR) study of the craniocervical junction. Neurological examination following clinical signs of possible atlantoaxial involvement was performed in all patients following the Ranawat classification. RESULTS: Radiographic and MR images showed craniocervical involvement in 41.3% and 61% of the patients, respectively. Immunological data were not correlated with imaging findings, whereas Ranawat class II and III of neurological involvement seem to be predictive of atlantoaxial alteration. CONCLUSION: Conventional radiography allowed us to detect 41.3% of patients with craniocervical involvement, but only in advanced stages of the disease. MR imaging had the unique potential of direct and detailed synovial visualization, especially in the gadolinium enhanced axial images, resulting in the early diagnosis of craniocervical RA.

Changes in regional cerebral blood flow after a cold hand test in systemic lupus erythematosus patients with Raynaud's syndrome.

The appearance of vasospastic features in the central nervous system (CNS) after a cold stressor test was Investigated through single photon emission computed tomography (SPECT) of regional cerebral blood flow in patients with systemic lupus erythematosus, with and without Raynaud's syndrome, and in scleroderma patients. We have shown that Raynaud's syndrome may occur in the CNS and that anticardiolipin or lupus anticoagulant positivity may favour perfusion defects.

Gated Tc-99m sestamibi SPECT versus stress-rest SPECT in detecting coronary artery disease: correlation with coronary angiography in patients without myocardial infarction.

PURPOSE: It is possible to simultaneously evaluate wall thickening and perfusion abnormalities with radionuclide techniques that use tracers such as Tc-99m MIBI. We presumed that detection of wall thickening by gated MIBI SPECT imaging in the presence of a stress-induced perfusion defect correlates with reversibility of that defect on resting images. Therefore, the aim of our study was to analyze, in patients without myocardial infarction, resting wall thickening and stress perfusion imaging as an alternative to conventional stress-rest imaging. METHODS AND RESULTS: The patients (n = 44) underwent an exercise (n = 37) or pharmacologic (n = 7) stress protocol. All patients had previous coronary angiography within 3 months. Stress-rest MIBI SPECT and gated MIBI SPECT studies were analyzed by visual scoring. The sensitivity and specificity of segmental analysis of both stress-rest MIBI SPECT perfusion and gated MIBI SPECT studies for the overall detection of coronary artery disease were, respectively, 71% and 96%. For patient evaluation for detection of coronary artery disease, stress-rest MIBI SPECT perfusion and gated MIBI SPECT studies showed a sensitivity rate of 96% for both and specificity rates of 84% and 79%, respectively. CONCLUSIONS: Our data revealed close agreement between reversible perfusion defects on stress-rest MIBI SPECT scans and significant wall thickening on gated MIBI SPECT stress images in patients without previous myocardial infarction (95%). Gated MIBI SPECT stress, without resting studies, which provide an assessment of wall motion and wall thickening, potentially allows stress defect reversibility to be evaluated in patients without previous myocardial infarction.

Cold lesion of a vertebral angioma with Tc-99m-labeled monoclonal antibodies against granulocytes.

Antigranulocyte immunoscintigraphy is indicated for diagnostic imaging to determine the location and extent of infection. We present a case of a focal cold lesion in a vertebra of a patient thought to have a septic focal lesion. Whole-body scanning and dorsal spine SPECT revealed no focal increased uptake. CT and MR studies revealed the presence of a vertebral angioma. Conditions associated with a defect of uptake in immunoscintigraphy are discussed.

Extraordinary intrathecal bone reaction in beta-thalassaemia intermedia.

We report on a patient with thalassaemia which was refractory to blood transfusions. The clinical picture was striking, and highlights the potential severity of intrathecal bone reactions after chronic intractable haemolytic anaemia.

Plasma chitotriosidase activity in patients with beta-thalassemia.

Chitotriosidase, a macrophage marker, which is extremely increased in plasma of Gaucher patients, was measured in patients with beta-thalassemia, an haematological disorder characterized by the genetic defect of beta-globin chains synthesis resulting in unproductive erythropoiesis and enormous expansion of the reticuloendothelial system. Plasma chitotriosidase was increased to a variable extent in 13 of 70 patients with beta-thalassemia major treated with the intense transfusion regimen and iron chelation therapy. It was normal in 22 and slightly elevated in 3 subjects with beta-thalassemia intermedia which were not transfused. The highest levels of plasma chitotriosidase, as high as in Gaucher patients, were found in 7 (10%) of the beta-thalassemia major patients which also had the highest degree of iron overload as judged by their serum ferritin level (> 3000 ng/ml), high SGPT level and elevated urinary iron excretion. To our knowledge, beta-thalassemia is hitherto the only disorder in which an increase of plasma chitotriosidase, comparable to that seen in Gaucher disease, may occur. The increase of plasma chitotriosidase activity in beta-thalassemia patients with high iron overload, could be related to an iron mediated damage to the lysosomal apparatus. In addition, similarly to Gaucher disease, the increased chitotriosidase production in beta-thalassemia might reflect macrophage activation probably related to the intracellular iron overload, storage of erythrocytes membrane break-down products and oxidation of excess alpha-hemoglobin subunits. Further studies are required to define the role of chitotriosidase evaluation to assess the efficacy of chelation therapy in reducing the macrophage activation due to intracellular iron overload in beta-thalassemia.

Evidence of exercise-induced myocardial ischemia in patients with primary aldosteronism: the Cross-sectional Primary Aldosteronism and Heart Italian Multicenter Study.

BACKGROUND: Primary aldosteronism (PA) is a disease associated with hypersecretion of aldosterone caused by an aldosterone-producing adrenal adenoma, bilateral adrenal hyperplasia, and, although rarely, by adrenal carcinoma. Arterial hypertension induces several cardiovascular alterations that yield a high cardiovascular risk. It has been shown that reduced myocardial perfusion at rest, assessed by thallium-201 myocardial scintigraphy, was greater in PA than in essential hypertension (EH). However, it is still unknown whether reduced myocardial perfusion at rest and/or regional function abnormalities are present during exercise-induced myocardial stress. PURPOSE: We addressed the impact of PA on myocardial ischemia and sought to identify signs of exercise-induced myocardial ischemia (assessed by MIBI-SPECT and echocardiography) in patients with PA compared to patients with EH. Patients with consistent signs of myocardial ischemia on all of the tests were studied by coronary arteriography. PATIENTS: We studied 72 patients with PA and an age/sex-matched group of 72 patients with EH enrolled in the cross-sectional Primary Aldosteronism and Heart Italian Multicenter Study (PAHIMS). METHODS: Regional function was detected from echocardiographic measurement of wall motion done at baseline and immediately after exercise. Myocardial perfusion was evaluated by SPECT imaging after injecting 99mTc-MIBI with the same-day protocol using the rest-stress sequence. RESULTS: Although the conditions of arterial pressure, duration of hypertension, and target organ damage were equivalent, the patients with PA had greater incidence of both reversible perfusion defects and abnormalities of regional function. Moreover, multiple regression analysis showed that the high plasma aldosterone level was highly predictive for SPECT ischemic score and wall motion index, suggesting that PA contributes to cardiovascular risk over and above that associated with ventricular hypertrophy. Exercise-induced myocardial ischemia in PA was not segmental but widely distributed suggesting that this phenomenon was not related to abnormal coronary perfusion. Accordingly, of the 38 patients with PA who underwent coronarography, there was no presence of significant coronary atherosclerotic lesions in 30 (78.9%) of the patients. CONCLUSIONS: The PAHIMS observed more exercise-induced moderate myocardial ischemic defects (co-detected by SPECT and echocardiograms and not segmental but widely allocated) in patients with PA than in patients with EH. This phenomenon occurred in a greater percentage of patients with PA without significant coronary lesions (78.95%, n = 38), which supports the possible presence of small-vessel intramyocardial disease.

Focal neurological deficits in children with beta-thalassemia major.

The hematologic disorder beta-thalassemia major is relatively common in Southern Italy. Stroke is a well described, though infrequently reported, complication of this disorder. We now report our experience regarding 300 children with beta-thalassemia major examined at the University of Catania, Italy, over a 20-year period. We encountered 9 patients (3%; 3 males, 6 females) with beta-thalassemia major who had hemorrhagic stroke. Two groups of patients can be identified: group 1 (2 patients 22%) with early-onset post-transfusion hemorrhage and group 2 (7 patients 77%) with delayed post-transfusion hemorrhage. In the first group, the hemorrhage occurred within 48 hours following blood transfusion. In the second group, hemorrhage occurred 7-15 days from last transfusion. In 5 patients out of 7 of this second group the first transfusion and ictal event both occurred after age five, suggesting prolonged chronic anemia might play a role in the hemorrhage.

Interferon-alpha therapy in Sicilian and Sardinian polytransfused thalassaemic patients with chronic hepatitis C.

OBJECTIVE: Our study was designed to evaluate the effects of 2 dosage schedules of recombinant interferon (IFN)-alpha (IFNalpha-2a and IFNalpha-2b) in reducing serum ALT and eradicating serum hepatitis C virus (HCV) RNA in beta-thalassaemic patients with chronic hepatitis C. DESIGN: 38 Sicilian beta-thalassaemic patients (22 males and 16 females) received intramuscular IFNalpha-2a (Roferon-A((R)); Roche) 5 MU/m(2) 3 times weekly for 6 months, followed by 3 MU/m(2) 3 times weekly for a further 6 months. 13 Sardinian beta-thalassaemic patients (7 males and 6 females) received intramuscular IFNalpha-2b (Intron(R); Schering-Plough) 3 MU/m(2) 3 times weekly for 12 months. Parallel control groups (n = 20 and n = 8, respectively) did not receive IFNalpha. All patients received continuous subcutaneous desferoxamine infusion. RESULTS: 24 (63%) Sicilian patients had a positive clinical response to IFNalpha-2a therapy. Two different patterns of response were apparent: (i) early and progressive decrease in ALT values until stable normalisation; and (ii) slower reduction of ALT values, which fluctuated on the way to normalisation. Five (21%) patients relapsed during the 12-month follow-up period. ALT levels decreased early in 5 (38%) Sardinian patients and one patient (20%) relapsed during the 12-month follow-up period. In the control groups, ALT values spontaneously normalised in 3 (10%) untreated patients. None of the patients treated with IFNalpha developed anti-IFNalpha antibodies. Viral clearance was demonstrated in 19 (50%) of 38 patients in the Sicilian group and 4 of 13 patients (31%) in the Sardinian group. CONCLUSION: Treatment with intramuscular recombinant IFNalpha-2a 5 MU/m(2) 3 times weekly for 6 months, followed by 3 MU/m(2) 3 times weekly for 6 months, appeared to be more effective than intramuscular IFNalpha-2b 3 MU/m(2) 3 times weekly for 12 months.