Age and comorbidities deeply impact on clinical outcome of patients with myelodysplastic syndromes.

BACKGROUND: Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal disorders, with very different prognosis in given individuals; age and comorbidities are emerging as relevant patient-related factors influencing clinical outcome in MDS. Our aim was to evaluate the impact of age, comorbidities and disease severity (IPSS and IPSS-R prognostic scores) in a "real-life" series of MDS patients. METHODS: 318 patients with available assessment of comorbidities at diagnosis and consecutively registered into the Registro Ligure delle Mielodisplasie were analyzed. Comorbidities were evaluated according to HCT-CI and MDS-CI comorbidity indexes. Overall survival (OS) and the probability of death among patients who did not develop acute myeloid leukemia (NLD cumulative incidence) were analyzed. RESULTS: Comorbidities were seen in 177 (55.7%) patients. An older age (>75 y) had a significant negative impact on OS (p=0.008). HCT-CI was not associated with OS. MDS-CI was of prognostic significance (p=0.001), but the association was limited to pts with IPSS or IPSS-R "lower-risk". In multivariate analysis, MDS-CI remained an independent factor associated with OS and with an increased risk of NLD both when controlling for IPSS (p=0.019 and p=0.001, respectively) and for IPSS-R (p=0.048 and p=0.002, respectively). CONCLUSIONS: Evaluation of age and comorbidities according to a tailored tool such is MDS-CI helps to predict survival in patients with MDS and should be incorporated to current prognostic scores.

Circadian and circannual rhythms of several enzymes of lysosomal origin in human plasma.

The circadian and circannual group rhythms in the plasma concentrations of the following lysosomal enzymes were studied in women and men: beta-D-N-acetylglucosaminidase, beta-D-glucuronidase, beta-D-glucosidase, beta-D-galactosidase, alpha-D-galactosidase, alpha-L-fucosidase and alpha-D-mannosidase. The circadian rhythm was detected in all the tested enzymes of women, and only in alpha-D-galactosidase, beta-D-glucosidase, alpha-D-mannosidase and beta-D-N-acetylglucosaminidase of men. A statistically significant difference between genders in the circadian rhythm was exhibited by beta-D-galactosidase, beta-D-glucosidase, beta-D-N-acetylglucosaminidase, beta-D-glucuronidase, alpha-D-galactosidase and alpha-L-fucosidase. A circannual rhythm was detected in all the tested enzymes, with the exception of beta-D-glucuronidase and beta-D-N-acetylglucosaminidase, without any statistically significant difference between genders. The group rhythms of some of the enzymes (alpha-D-galactosidase, beta-D-glucosidase, beta-D-galactosidase) showed similar values of both circadian and circannual acrophases, suggesting that they may be subjected as a group to the same chronobiological coordination. The chronobiological rhythms of lysosomal enzymes were different from those of lactate dehydrogenase and alpha 1-antitrypsin, indicating that these rhythms are not merely reflecting fluctuations of the water content of plasma. No in-phase relationship was observed between the circadian and circannual rhythms of plasma cortisol and those of the tested lysosomal enzymes, excluding a direct chronobiological relationship between this hormone and lysosomal enzymes.

[Splenohepatic hemangiosarcoma with laceration of the spleen]. / Emangiosarcoma splenoepatico con lacerazione di milza

A case of a 31-years-old male with pathologic rupture of the spleen and hemoperitoneum, caused by a primitive hemangiosarcoma and occurred during a weight-lifting is described. A metastatic involvement of the liver was also noted. The patient was splenectomized and died within 2 months.

[Case studies of the histological characterization of primary tumors of the testis]. / Contributo casistico alla tipizzazione istologica dei tumori primitivi del testicolo.

One hundred-ninety nine cases of testicular tumours, of which 189 (94,9%) germ cell tumours, have been studied by histological methods. Seminoma with its two subtypes (anaplastic and spermatocytic) is the most frequent tumour (110 cases i.e. 55,2%), followed by embryonal carcinoma (30 cases: 15,3%), teratocarcinoma (25 cases: 13,2%), teratoma (12 cases: 6,3%) and choriocarcinoma (5 cases: 2,6%). Tumours showing more than one histologic pattern are frequent. The distinctive histologic features have studied, with emphasis of those useful for diagnostic and prognostic purposes. The ages mostly involved by various tumours are stressed. The rarest tumours are briefly described. Histologic data suggest that germinal tumours are originated by the same primordial germ cell which is able to develop various degrees of differentiation. Histologic and histogenetic features of limphomas and tumours of specialized gonadal stroma are also discussed.

[1 year of automatic management of an analytical laboratory using a part-time computer]. / Un anno di gestione automatica del Laboratorio di Analisi mediante utilizzazione "part time" di un elaboratore elettronico

This paper is a report on a one year-automatic management of the Clinical Lab. of the Hospital of Gallarate (Italy) by means of a "part-time" computerization system. Technologies and procedures used for in-patients and out-patients are analysed: by this kind of management many advantages are obtained but some partly unremovable drawbacks, are identified which are mainly caused by the current management of clinical Departments.

[Surgical treatment of neck lymph nodes in squamous cell carcinoma of the pyriform sinus]. / Il trattamento chirurgico dei linfonodi del collo nel carcinoma squamocellulare del seno piriforme.

The present study reports the results of 66 patients surgically treated for squamous cell carcinoma of the pyriform sinus between 1984 and 1996. Twenty eight patients underwent mono!ateral neck dissection and bilateral neck dissection was performed in 38 cases, for a total of 104 radical neck dissections. Of these, 73 (71%) were modified type III dissections, 17 (16%) were classical, and 14 (13%) were modified type I and II dissections. The primary lesion was strictly lateralized in 47 cases (71%), while median structures were involved in 19 patients. The primary tumor was staged pT1 in 2 patients, pT2 in 29, pT3 in 19, and pT4 in 16. The overall incidence of lymph node metastases was 79% (9 pN1, 3 pN2a, 33 pN2b, 7 pN2c) which was not correlated with T stage (50% pT1, 72% pT2, 89% pT3, 81% pT4). Occult nodal metastases were present in 42% of cases (8/19) with an incidence that increased from 11% (1/9) for pT1-2 to 70% for pT3-4 (7/10). The bilateral metastases (11%) were uniformly distributed between strictly lateral neoplasms and those tumors involving the midline. The incidence of bilateral metastases reached 19% only in patients with T4 cancers. Occult controlateral metastases were found in 12% of patients not having clinical evidence of metastases on the contro-lateral side of neck dissection (4/33). Nodal metastases never involved the I and V levels. Our data did not permit an assessment of the incidence of retropharyngeal lymph node metastases. In view of these results and considering current knowledge of the anatomy of lymphatic drainage, a selective II-IV dissection extending to the level VI on the side of the tumor appears justified in cases clinically staged as NO. In our view, when the lesion involves the posterior wall of the pharynx, neck dissection should be extended to the lateral retropharyngeal lymph nodes. Selective dissection of the controlateral side of the neck should be performed in patients having either locally advanced primary lesions or with lesions approaching the midline. In the presence of metastases which are either clinically or intraoperatively evident, neck dissection should be extended to additional lymph node levels.

A new device for the care of Congenital Central Hypoventilation Syndrome patients during sleep.

Congenital Central Hypoventilation Syndrome (CCHS) is a genetic disease that causes an autonomous nervous system dysregulation. Patients are unable to have a correct ventilation, especially during sleep, facing risk of death. Therefore, most of them are mechanically ventilated during night and their blood oxygenation is monitored, while a supervisor keeps watch over them. If low oxygen levels are detected by the pulse-oximeter, an alarm fires; the supervisor deals with the situation and, if there is neither a technical problem nor a false alarm, wakes the subject, as CCHS patients usually recover from hypoxia when roused from sleep. During a single night multiple alarms may occur, causing fractioned sleep for the subject and a lasting state of anxiety for supervisors.

Design of a 3D surface scanner prototype suitable for MR.

The detection of body movements during MR examination could help in reducing motion artifacts or to get patient responses during functional magnetic resonance. It can be supported by a slit scanner, that combines a camera with a light stripe projector to obtain 3D coordinates of points forming the external surface of the body. In this work we propose a slit scanner prototype based on a miniaturized projector without moving parts. Just small sized hardware is required to analyze the video signal, operating in time domain instead of spatial domain. To accomplish this, the camera is placed with its pixel columns as more parallel as possible to the projected light stripes and the camera video signal is analyzed by a resistor transistor logic after analog processing.

Quantitative evaluation protocol for upper limb motor coordination analysis in patients with ataxia.

Objective and quantitative measurement is crucial in the definition of functional impairment and in the tracking of disease progress over time of patients affected by progressive pathologies, such as ataxia. A new experimental procedure for the quantitative description of upper limb movement and coordination analysis was developed by the integration of an optoelectronic system and dedicated electronic board with four visual and pressure stimuli. 20 passive retroreflective markers were placed on the subject's body and two types pointing tests were defined: in the first one, the subjects were asked to reach with the index finger five consecutive times each of the three targets ("repetitive test"), and in the second one, the subjects were asked to randomly reach the targets with the index finger ("random test"). The preliminary results showed that patients affected by ataxia took more time with a less smooth finger tip movement to perform the reaching tests when compared to healthy subjects. The velocity was lower and its profile was more irregular in ataxic subjects. The new developed experimental procedure seems to be very promising in the quantitative description of upper limb movements of pathological and healthy subjects and it seems to be able to distinguish the impairments due to different levels of ataxia.

Active monitoring insole: a wearable device for monitoring foot load distribution in home-care context.

Post surgical patients need to resume a correct load distribution over both legs in order to facilitate bone osteogenesis. We propose a device for continuously monitoring lower limbs loads. It can be used by the patient to be aware of his loading conditions, by the physician or physical therapist to perform a quantitative analysis of applied loads. We tested the device on two subjects and verified its capability to detect reduced loads on the affected limb and its usefulness in helping to recognize in which walking phases.

Integrating telemonitoring with clinical information systems: a case study.

Current health scenarios have witnessed increased attention for home care as a way to enhance the quality of service provision and reduce costs. In recent times, clinical information systems (CISs) have undergone significant development and they now include useful applications for doctors, nurses and other health professionals. The present work investigates the possible integration of a home-based telemonitoring system within a CIS to enhance its potentials. Test results showed a smooth integration and highlighted multiple advantages such as use of a single interface for the management of several data types, inclusion of health-related data about in-hospital and home periods, workflow creation and check points during home care provision, use of consistent criteria for data security and privacy protection.

The hydrogen bond in ice probed by soft x-ray spectroscopy and density functional theory.

We combine photoelectron and x-ray absorption spectroscopy with density functional theory to derive a molecular orbital picture of the hydrogen bond in ice. We find that the hydrogen bond involves donation and back-donation of charge between the oxygen lone pair and the O-H antibonding orbitals on neighboring molecules. Together with internal s-p rehybridization this minimizes the repulsive charge overlap of the connecting oxygen and hydrogen atoms, which is essential for a strong attractive electrostatic interaction. Our joint experimental and theoretical results demonstrate that an electrostatic model based on only charge induction from the surrounding medium fails to properly describe the internal charge redistributions upon hydrogen bonding.

A wearable device for a fully automated in-hospital staff and patient identification.

In the health care context, devices for automated staff / patient identification provide multiple benefits, including error reduction in drug administration, an easier and faster use of the Electronic Health Record, enhanced security and control features when accessing confidential data, etc. Current identification systems (e.g. smartcards, bar codes) are not completely seamless to users and require mechanical operations that sometimes are difficult to perform for impaired subjects. Emerging wireless RFID technologies are encouraging, but cannot still be introduced in health care environments due to their electromagnetic emissions and the need for large size antenna to operate at reasonable distances. The present work describes a prototype of wearable device for automated staff and patient identification which is small in size and complies with the in-hospital electromagnetic requirements. This prototype also implements an anti-counterfeit option. Its experimental application allowed the introduction of some security functions for confidential data management.

Chronobiological study of several enzymes of lysosomal origin in human plasma.

The possible occurrence of circadian and circannual rhythms in the plasma concentrations of the following enzymes of lysosomal origin was assessed: beta-D-N-acetylglucosaminidase (EC 3.2.1.30) beta-D-glucuronidase (EC 3.2.1.31), beta-D-glucosidase (EC 3.2.1.21), beta-D-galactosidase (EC 3.2.1.22), alpha-D-galactosidase (EC 3.2.1.23), alpha-L-fucosidase (EC 3.2.1) and alpha-D-mannosidase (EC 3.2.1.24). The circadian rhythm was studied in 16 women (aged: 17-24 years) and 13 men (age: 23 years) volunteers; the circannual rhythm, in 10 women and 8 men (age: 20-25 years). The circadian rhythm was detected in all the tested enzymes of women, and only in alpha-D-galactosidase, beta-D-glucosidase, alpha-D-mannosidase and beta-D-acetylglucosaminidase of men. A statistically significant difference between genders in the circadian rhythm was exhibited by beta-D-galactosidase (MESOR; amplitude) beta-D-glucosidase (MESOR; amplitude; acrophase) beta-D-N-acetylglucosaminidase, beta-D-glucuronidase and alpha-D-galactosidase (MESOR) and alpha-L-fucosidase (amplitude, acrophase). A circannual rhythm was detected in all the tested enzymes with the exception of beta-D-glucuronidase and beta-D-N-acetylglucosaminidase; no statistically significant difference between genders was detected. The group rhythms of some of the enzymes (alpha-D-galactosidase, beta-D-glucosidase, beta-D-galactosidase) showed similar values of both circadian and circannual acrophases, suggesting that they may subjected as a group to the same chronobiological coordination, possibly mediated by hormones. The chronobiological rhythms of lysosomal enzymes were different from those of lactate dehydrogenase and alpha 1-antitrypsin, indicating that these rhythms are not merely reflecting fluctuations of the water content of plasma. No in-phase relationship was observed between the circadian and circannual rhythms of plasma cortisol and those of the tested lysosomal enzymes, excluding a direct chronobiological and possibly functional relationship between this hormone and lysosomal enzymes.

[Single-contrast enema after colon lavage in the elderly patient]. / Il clisma monocontrasto previo lavaggio del colon nel paziente anziano.

Double-contrast enema is often unsuccessful in elderly patients because of their poor cooperation and colon cleaning. This work was aimed at showing that a well-done single-contrast exam with simple colon lavage can make a very good alternative to double-contrast enema in elderly patients. Thus, 66 consecutive elderly patients (age range: 70-90 years) were submitted, over a 6-month period (June-December, 1994), to single-contrast enema, after colon cleaning with oral whole-gut lavage, using an osmotically balanced electrolyte solution (Isocolan, Bracco) containing polyethylenglycol (PEG 4000). We examined, in each patient: a) the tolerance to and effectiveness of bowel cleaning, according to the liquid volume drunk by each subject, needed to obtain clear diarrhea; b) radiograph quality. Successful colon cleaning was achieved with a mean (2.5-3 liters) and a large (3.5-4 liters) amount of solution, in 80% and 20% of our patients, respectively. Tolerance was good in the whole series except for 3 patients with emesis. Moreover, radiograph quality was good in 85% of the subjects. Finally, barium enema results, compared with endoscopic results, yielded 27 true positives, 3 false negatives (small neoplastic lesions) and 1 false positive. Therefore, in our opinion, these results confirm the role of single-contrast enema, combined with gut lavage, in elderly patients.

Electronic structure effects from hydrogen bonding in the liquid phase and in chemisorption: an integrated theory and experimental effort.

A closely integrated theoretical and experimental effort to understand chemical bonding using X-ray spectroscopic probes is presented. Theoretical techniques to simulate XAS (X-ray absorption spectroscopy), XES (X-ray emission spectroscopy), RIXS (resonant inelastic X-ray scattering) and XPS (X-ray photoelectron spectroscopy) spectra have been developed and implemented within a density functional theory (DFT) framework. In combination with new experimental techniques, such as high-resolution XAS on liquid water under ambient conditions and XES on complicated surface adsorbates, new insight into e.g. hydrogen-bonded systems is obtained. For the (3x2) overlayer structure of glycine/Cu(110), earlier work has been extended to include adsorbate-adsorbate interactions. Structures are optimized for large cluster models and for periodic boundary conditions. It is found that specific features in the spectra arise from hydrogen-bonding interactions, which thus have important effects at the molecular-orbital level. XAS on liquid water shows a pronounced pre-edge feature with significant intensity, while the spectrum of ice shows only little intensity in this region. Theoretical spectrum calculations, based on instantaneous structures obtained from molecular-dynamics (MD) simulations, show that the pre-edge feature in the liquid is caused by water molecules with unsaturated hydrogen bonding. Some aspects of the theoretical simulations will be briefly discussed.

The structure of the first coordination shell in liquid water.

X-ray absorption spectroscopy and x-ray Raman scattering were used to probe the molecular arrangement in the first coordination shell of liquid water. The local structure is characterized by comparison with bulk and surface of ordinary hexagonal ice Ih and with calculated spectra. Most molecules in liquid water are in two hydrogen-bonded configurations with one strong donor and one strong acceptor hydrogen bond in contrast to the four hydrogen-bonded tetrahedral structure in ice. Upon heating from 25 degrees C to 90 degrees C, 5 to 10% of the molecules change from tetrahedral environments to two hydrogen-bonded configurations. Our findings are consistent with neutron and x-ray diffraction data, and combining the results sets a strong limit for possible local structure distributions in liquid water. Serious discrepancies with structures based on current molecular dynamics simulations are observed.