Analysis of the Trend Over Time of High-Urgency Liver Transplantation Requests in Italy in the 4-Year Period 2014-2017.

BACKGROUND: The national protocol for the handling of high-urgency (HU) liver organ procurement for transplant is administered by the Italian National Transplant Center. In recent years, we have witnessed a change in requests to access the program. We have therefore evaluated their temporal trend, the need to change the access criteria, the percentage of transplants performed, the time of request satisfaction, and the follow-up. METHODS: We analyzed all the liver requests for the HU program received during the 4-year period of 2014 to 2017 for adult recipients (≥18 years of age): all the variables linked to the recipient or to the donor and the organ transplants are registered in the Informative Transplant System as established by the law 91/99. In addition, intention to treat (ITT) survival rates were compared among 4 different groups: (1) patients on standard waiting lists vs (2) patients on urgency waiting lists, and (3) patients with a history of transplant in urgency vs (4) patients with a history of transplant not in urgency. RESULTS: Out of the 370 requests included in the study, 291 (78.7%) were satisfied with liver transplantation. Seventy-nine requests (21.3%) have not been processed, but if we consider only the real failures, this percentage falls to 13.1% and the percentage of satisfied requests rises to 86.9%. The average waiting period for liver transplantation (LT) is 1.7 days and most requests (74%) are met in less than 24 hours, if we consider the hours between the registration of the request and the donor reporting . The percentage of late retransplantations is 2.1%. The clinical indication for HU-LT that appears to improve over time is hepatic artery thrombosis (82.5%). The overall 1-year patient survival is 68.3%. The overall 1-year graft survival, performed on all the patients, is 89% and all the indications for HU-LT appear to go well over time with an average survival rate greater than 85%. CONCLUSIONS: The indications for HU-LT are changing according to the changes in the hepatologic field in recent years. The centralized management of requests has proven to be successful in optimizing responses. Urgent LT is confirmed to be lifesaving in its timeliness.

Transport of Human Organs in Italy: Location, Time, and Performances.

INTRODUCTION: The outcome of transplantation activities depends on a variety of unpredictable factors. Up-to-date criteria on organ allocation foresee an efficient transport chain along with compliant performance parameters. METHODS AND OBJECTIVES: The Centro Nazionale Trapianti and the Department of Civil, Chemical, Environmental, and Materials Engineering of the University of Bologna (respectively, CNT and DICAM) have been updating a national database of organ transplantation activities to investigate performance parameters and the main causes of disruption. RESULTS: Between June 2015 and July 2016, 617 of 1061 organs have been shipped by air (making for 486 flight events), of which 407 were accompanied by medical equipment. Origin/destination and distance matrixes have been drawn for both road and air transport. Each airport node is ranked based on the n° of organs ingoing/outgoing and each route link on its frequency. Performance parameters such as average speed, distance covered, and time have been computed and compared with each organ's cold ischemia time (CIT). Average distance frontiers are rather homogeneous, but much effort is necessary to reduce the number of events performed with approximately 90% or more of CIT spent. CONCLUSIONS: The monitoring of organ transplantation activities' performance is a standalone action within Europe to support strategic policies to optimize the system. Thus, a clearer awareness on performances and issues related to organ transport has been made possible: analyses show that the higher uncertainty associated with total time of displacement by air is due to the steps which take place by road (length and paths must be optimized) and lung transports generally perform weaker than heart transports due to longer average distances travelled and smaller average speeds, often resulting in a total displacement time greater than 90% of CIT.

Analysis of the Organ Offers Received From European Union Countries Before and After the Introduction of a Dedicated Information Technology Portal: The COORENOR/FOEDUS Portal.

INTRODUCTION: To optimize the use of nontransplantable organs in their own territory, the European Commission, as part of a project led by Italy, has promoted the use of an information technology (IT) portal, the COORENOR portal, developed by the Czech Republic in 2012, which evolved to become FOEDUS in 2015. METHODS: To evaluate the impact of the portal on our reality, we analyzed the number and type of offers received and organs imported in the previous 48 months (period A) as well as the 48 months after the introduction of the portal (period B). We also examined the origin and the offer mode. RESULTS: The offers received were 404 and 753, respectively, in the two periods, with 315 (41.8%) organs received through the portal. The organs transplanted were 53 and 64, respectively, in the two periods; 20 (31.2%) were sent through the portal. The most commonly offered organs are lungs (36.7% and 29.3% of offers in periods A and B, respectively). The most transplanted organ is the liver (59.4% and 45% of transplants in periods A and B, respectively). The use of the portal has gradually increased, growing from 16.4% of the offer mode in 2012 to 84.7% in 2016. CONCLUSIONS: The increase of offers related to the increase of donations and the attitude to the sharing of resources has determined an increase of 19.2% of total transplants, especially for certain types such as pediatric transplants. The portal, ensuring speed and simultaneity of offer, real time sharing of information and transparency of allocation, is also used for trade in the International Partnership Agreements. Therefore, transplants have been conditioned by the existing agreements with Greece, Malta, and the countries of the South Transplant Alliance.

Hepatitis C Virus Positive Patients on the Waiting List for Liver Transplantation: Turnover and Characteristics of the Population on the Eve of the Therapeutic Revolution With Direct-Acting Antivirals.

INTRODUCTION: Antivirals direct acting (DAA) for hepatitis C virus (HCV) have brought a revolution in the field of transplantation. It is likely to think that in the future patients on the waiting list for liver transplantation (LT) will no longer be registered for HCV-related cirrhosis but for liver disease from other causes. On the eve of this change, we show a snapshot of the Italian waiting list for LT. METHODS: From October 1, 2012 to September 30, 2013, we estimated the total number of patients on the liver waiting list as intention to treat (ITT), the number of incident cases, and the delistings, particularly in the HCV positive (HCV+) population. Gender, median age, etiology and prognosis of liver disease, presence of hepatocellular carcinoma (HCC), reason for delisting, mean waiting time for LT, and rate of death on waiting list were evaluated. RESULTS: In the time period, there were 517 new patients who were HCV+ (median age, 53 years): 255 (49.3%) mono-infected with HCV, 236 (45.7%) co-infected with HCV and hepatitis B virus (HBV), 11 (2.1%) co-infected with HCV and human immunodeficiency virus (HIV), and 15 (2.9%) co-infected with HCV, HBV, and HIV. The median model for end-stage liver disease (MELD) score at listing was 17 and HCC was present in 206 (39.8%) cases. HCV+ patients delisted were 442 (61.9%), 355 (80.3%) for LT. The mean waiting time to transplantation was 1.9 months; the percentage of death was 7.6%. CONCLUSIONS: This snapshot of the waiting list for LT in the year before the advent of DAA drugs will allow us to assess whether and how they will change the waiting list for LT when we start to look at the impact of new therapies on the waiting list.

Optimizing the Use of Available Lungs.

BACKGROUND: Organ transplantation, the treatment of choice in organ failure, is penalized by the lack of organs. Because the increase in the number of donors is not proportional throughout the different age groups, there is no increase in lung transplantations. The aim of this work was to analyze the use of available lungs and evaluate strategies that may help increase transplantations. METHODS: We analyzed the activity of lung transplantation in 2015, divided into various allocation programs. We also examined the surplus organs, in particular, their origin, their destination, their offer's outcome, the characteristics of the donor and the proposed organ, and the reasons for rejection. RESULTS: In 2015, 112 lung transplantations were performed: 66 (68.9%) with regional organs, 46 (41.1%) with extraregional organs; 21 (45.6%) of these were allocated as emergencies/return, and 25 (54.4%) as surplus (19 in the North macroarea, 6 in the South macroarea). The number of surplus lungs was 148: 67 from the North macroarea, 71 from the South macroarea, and 10 from abroad. No organ procured in the North macroarea was transplanted in the South macroarea, whereas 6 lungs coming from the South macroarea were transplanted in the North. CONCLUSIONS: The acceptance criteria are not the same in different transplant centers and they include not only clinical parameters, but also ischemia time and composition of the waiting list at the time of the offer, quality and accessibility of the intensive care units where the donor is located, and organizational reasons. Offering organs which can not be transplanted within the region to other centers, without clinical foreclosures is a system that increases transplant activities by maximizing the available resources.

Liver Allocation in Urgent MELD Score ≥30: The Italian Experience.

INTRODUCTION: Patients with an urgent MELD score ≥30 are managed by the Italian Operative National Transplant Center on the basis of a division of Italy into 2 main areas, the northern macro area (NMA) and the southern macro area (SMA). The object of this study was to evaluate the possibility and the need to transform the MELD score ≥30 macro area-based program into a nationwide one. PATIENTS AND METHODS: When a region reports the presence of a patient with a MELD score ≥30, the same macro area-compatible donors, in the absence of urgent national and 1B status, are offered primarily to this recipient. RESULTS: From August 2014 to August 2015, 132 requests for patients with urgent MELD score ≥30, 98 from the NMA and 34 from the SMA, were handled. The average waiting list in the NMA was significantly different from that of the SMA (2.74 ± 2.29 vs 4.5 ± 3.98, P < .05). A total of 73.7% of the received requests (n = 97) were satisfied: the NMA met 80.4% of the requests (n = 77), whereas the SMA met 55.5% (n = 20). A total of 35 requests (26.5%), 21 from the NMA (60%) and 14 (40%) from the SMA, were not met. The average waiting time of these recipients for a liver was significantly different between the NMA and the SMA (3.14 ± 3.21 vs 5.78 ± 4.59; P < .05). CONCLUSIONS: The MELD score is a priority allocation, and the longer the waiting time to transplantation for these recipients, the more their mortality increases. Given the differences in waiting times between the NMA and SMA, we should start thinking about transforming the macro area program into a national one.

Transportation of Organs by Air: Safety, Quality, and Sustainability Criteria.

INTRODUCTION: The outcomes of organ transplantation activities are greatly affected by the ability to haul organs and medical teams quickly and safely. Organ allocation and usage criteria have greatly improved over time, whereas the same result has not been achieved so far from the transport point of view. Safety and the highest level of service and efficiency must be reached to grant transplant recipients the healthiest outcome. OBJECTIVES: The Italian National Transplant Centre (CNT), in partnership with the regions and the University of Bologna, has promoted a thorough analysis of all stages of organ transportation logistics chains to produce homogeneous and shared guidelines throughout the national territory, capable of ensuring safety, reliability, and sustainability at the highest levels. METHODS: The mapping of all 44 transplant centers and the pertaining airport network has been implemented. An analysis of technical requirements among organ shipping agents at both national and international level has been promoted. A national campaign of real-time monitoring of organ transport activities at all stages of the supply chain has been implemented. Parameters investigated have been hospital and region of both origin and destination, number and type of organs involved, transport type (with or without medical team), stations of arrival and departure, and shipping agents, as well as actual times of activities involved. RESULTS: National guidelines have been issued to select organ storage units and shipping agents on the basis of evaluation of efficiency, reliability, and equipment with reference to organ type and ischemia time. Guidelines provide EU-level standards on technical equipment of aircrafts, professional requirements of shipping agencies and cabin crew, and requirements on service provision, including pricing criteria. CONCLUSIONS: The introduction in the Italian legislation of guidelines issuing minimum requirements on topics such as the medical team, packaging, labeling, safety and integrity, identification, real-time monitoring of temperature, and traceability of the organ during the logistics chain is deemed a valid response to the necessity of improving safety, reliability, and sustainability of organ transplantation activities in Italy.

Solid Organ Transplantation in HIV+ Recipients: Italian Experience.

INTRODUCTION: According to current estimates, there are about 540,000 patients who are infected with HIV in Western Europe, of which about 3100 are potential candidates for organ transplantation. In Italy, there are currently 85 HIV patients on the transplant list. METHODS: Organ transplantation activity in HIV recipients from 2002 to December 2014 was assessed from the database provided by the Transplant Center of Modena until the year 2011. For the years 2012 to 2014, data are from the Transplant Information System (SIT). The follow-up data have been extracted from the function "Quality" of the SIT. RESULTS: The transplant centers on Italian territory that meet the requirements according to national protocol are in total 29: 11 for the liver, 9 for the kidney including 1 pediatric, 3 for the heart, 3 for the lungs, and for 3 for the combined kidney-pancreas. Since 2002, 257 organ transplantations were carried out, including 185 liver, 59 kidney, 5 combined liver-kidney, 5 combined kidney-pancreas, 2 heart, and 1 double lung. The first cause of death is represented by co-hepatitis C virus infection, in particular in 26 liver recipients (37%) and in 3 kidney recipients (20%). CONCLUSIONS: The analysis showed that transplantation activity in HIV is on the rise, especially in the last 2 years, with an outcome similar to that reported in the literature.

Cerebral microemboli detected by transcranial doppler in patients treated with extracorporeal membrane oxygenation.

INTRODUCTION: Cerebrovascular complications rate in patients treated with extracorporeal membrane oxygenation (ECMO) is about 7%. Ischemic stroke may be caused by solid or gaseous microemboli due to thrombosis within the circuit or cannula. Transcranial Doppler (TCD) is the only method able to detect microembolic signals (MES) in real time. The objective of this study was to detect possible MES by TCD in patients treated with veno-venous (VV) and veno-arterial (VA) ECMO and to test for a relation between the number of MES and the 6-month clinical outcome of these patients. METHODS: This is a monocentric observational prospective study in patients consecutively admitted and treated with ECMO at our regional ECMO referral center in 18 months. TCD detection of MES was performed in patients upon initiation of treatment and then repeated during treatment. RESULTS: Two hundred and forty-eight TCD monitoring were performed in 42 VV and 11 VA ECMO patients. MES were detected in 26.2% of VV ECMO patients and in 81.8% of VA ECMO patients (P < 0.001). In both subgroups of patients, no correlation was found between MES detection and extracorporeal flow velocities or aPTT values. In VA ECMO patients, an inverse correlation between left ventricular ejection fraction and MES grading was found (P = 0.037). In both groups, no clinical neurological impairments correlated to MES detection were found at 6 months follow-up. CONCLUSIONS: MES were found in both ECMO configurations; independently from their pathophysiology, MES do not seem to influence clinical outcome. Multicenter studies are still required with more extensive cases to confirm these results.

Macrophage activation syndrome in a child affected by malaria: the choice of steroid.

Macrophage activation syndrome is a potentially fatal clinical syndrome caused by an excessive activation and proliferation of macrophages and T cells, leading to an exaggerated inflammatory reaction. It is well known that it can complicate the course of different conditions, especially autoimmune, lympho-proliferative, infectious diseases and drugs. Many infective pathogens can trigger the syndrome but the association with malaria has rarely been described, especially in children. We report a child with severe malaria complicated by MAS, in whom the clinical appearance of this syndrome could be considered as worsening of malaria itself. Furthermore, the use of steroids as first choice drugs in this complication, but arguable in malaria, has been highlighted. Clinicians should be aware of this syndrome when malaria does not respond to conventional therapy, since early diagnosis and prompt treatment may dramatically reduce the mortality associated with this condition.

Estimation of biogas produced by the landfill of Palermo, applying a Gaussian model.

In this work, a procedure is suggested to assess the rate of biogas emitted by the Bellolampo landfill (Palermo, Italy), starting from the data acquired by two of the stations for monitoring meteorological parameters and polluting gases. The data used refer to the period November 2005-July 2006. The methane concentration, measured in the CEP suburb of Palermo, has been analysed together with the meteorological data collected by the station situated inside the landfill area. In the present study, the methane has been chosen as a tracer of the atmospheric pollutants produced by the dump. The data used for assessing the biogas emission refer to night time periods characterized by weak wind blowing from the hill toward the city. The methane rate emitted by the Bellolampo dump has been evaluated using a Gaussian model and considering the landfill both as a single point source and as a multiple point one. The comparison of the results shows that for a first approximation it is sufficient to consider the landfill of Palermo as a single point source. Starting from the monthly percentage composition of the biogas, estimated for the study period, the rate of biogas produced by the dump was evaluated. The total biogas produced by the landfill, obtained as the sum of the emitted component and the recovered one, ranged from 7519.97 to 10,153.7m3/h. For the study period the average monthly estimations of biogas emissions into the atmosphere amount to about 60% of the total biogas produced by the landfill, a little higher than the one estimated by the company responsible for the biogas recovery plant at the landfill.

[Evaluation of operating theatre for the risk prevention]. / Valutazione della qualità della Sala Operatoria in funzione della prevenzione del rischio.

In this study we analyse factors that predispose to risk and we value the importance of quality and reliability into operating theatre. Patient safety result from ability to plan and manage organizations able to reduce probability of mistakes (Prevention) and to recover and contain their consequences (Protection). The principal motives of mistakes are: - deficiency of sharing procedures between different professional figures for risk prevention; - deficiency of an effective integration between professional figures in operating theatre; - deficiency of charitable precise run to guarantee the continuity of interventions on patient. A risk management program have to take care on this sentinel events and to set up a survey-information system to characterize risks and correction strategies. To prevent patient change, wrong side identification, not working devices or deficiency of surgery materials, it would be useful for operating theatre figures and for anesthetists to work out all together a procedure for admission in operating theatre and a pre-operating check list. The best way to carry out a safety and quality attendance based on standardized procedures and protocols, is to set up risk management firm units. It is necessary to activate and to adjust survey system and effective management training.

Apoptosis during chick inner ear development: some observations by TEM and TUNEL techniques.

In order to clarify the occurrence, distribution and possible role of apoptosis during inner ear development, the ultrastructural aspects (by TEM) (at 9-19 incubation day and 1 day after hatching) and the distribution of the apoptotic phenomenon (by the TdT-mediated dUTP nick end-labeling technique), were studied in the crista ampullaris of chick embryo at 5-19 days of incubation to hatching and of postnatal 1-day old chick. We found, in the sensorial epithelium, dark supporting cells in chick embryos and mainly dark hair cells in postnatal chicks, both with ultrastructural features consistent with those of apoptosis. The presence of apoptotic phenomena was confirmed by the TUNEL technique. According to our findings, it is hypothesized that apoptosis in the inner ear may be involved: 1) at first, in macroscopic remodelling of the membranous labyrinth in early developmental stages, 2) later, in the correct differentiation of the hair and of the supporting cells, leading to characteristic cellular pattern formation and 3) finally, in physiological cell turnover of the postnatal chicken sensorial epithelium of the crista.

Crystal structure of the dimeric phosphoenolpyruvate carboxykinase (PEPCK) from Trypanosoma cruzi at 2 A resolution.

ATP-dependent phosphoenolpyruvate carboxykinase (PEPCK) (ATP: oxaloacetate carboxylyase (transphosphorylating), EC 4.1.1.49) is a key enzyme involved in the catabolism of glucose and amino acids in the parasite Trypanosoma cruzi, the causative agent of Chagas' disease. Due to the significant differences in the amino acid sequence and substrate specificity of the human enzyme (PEPCK (GTP-dependent), EC 4.1.1.32), the parasite enzyme has been considered a good target for the development of new anti-chagasic drugs. We have solved the crystal structure of the recombinant PEPCK of T. cruzi up to 2.0 A resolution, characterised the dimeric organisation of the enzyme by solution small angle X-ray scattering (SAXS) and compared the enzyme structure with the known crystal structure of the monomeric PEPCK from Escherichia coli. The dimeric structure possesses 2-fold symmetry, with each monomer sharing a high degree of structural similarity with the monomeric structure of the E. coli PEPCK. Each monomer folds into two complex mixed alpha/beta domains, with the active site located in a deep cleft between the domains. The two active sites in the dimer are far apart from each other, in an arrangement that seems to permit an independent access of the substrates to the two active sites. All residues of the E. coli PEPCK structure that had been found to interact with substrates and metal cofactors have been found conserved and in a substantially equivalent spatial disposition in the T. cruzi PEPCK structure. No substrate or metal ion was present in the crystal structure. A sulphate ion from the crystallisation medium has been found bound to the active site. Solution SAXS data suggest that, in solutions with lower sulphate concentration than that used for the crystallisation experiments, the actual enzyme conformation may be slightly different from its conformation in the crystal structure. This could be due to a conformational transition upon sulphate binding, similar to the ATP-induced transition observed in the E. coli PEPCK, or to crystal packing effects. The present structure of the T. cruzi PEPCK will provide a good basis for the modelling of new anti-chagasic drug leads.

Pulmonary involvement in juvenile dermatomyositis: a two-year longitudinal study.

OBJECTIVE: To investigate the prevalence and features of asymptomatic pulmonary involvement in juvenile dermatomyositis (JDM). METHODS: Twelve JDM patients underwent pulmonary function tests at baseline, 12 and 24 months. Disease activity, duration, serum lactate dehydrogenase (LDH) values and antinuclear antibody (ANA) titres were also evaluated. RESULTS: Five patients showed lung impairment at baseline and four at 12 and 24 months. Forced expiratory volume in 1s, forced vital capacity (FVC), carbon monoxide diffusing capacity (DLCO) and alveolar volume were the most frequently altered variables, indicating a restrictive pattern and impairment of diffusion. The prevalence and features of pulmonary alterations did not change during follow-up. FVC values were significantly lower in active JDM patients and were inversely related to LDH. DLCO values were significantly lower in ANA-positive patients. About half of the patients of this small case series of JDM had asymptomatic lung disease. CONCLUSIONS: We suggest that lung function should be evaluated at disease onset and regularly during follow-up, as pulmonary function tests can detect otherwise unpredictable pulmonary involvement.

Calcification processes in the chick otoconia and calcium binding proteins: patterns of tetracycline incorporation and calbindin-D28K distribution.

In order to clarify the otoconia formation and turnover, tetracycline, an antibiotic that precipitates at calcifying fronts and serves as a fluorescent marker, was injected into eggs at different stages of chick embryonic development, as well as into postnatal chicken and into adult animals. The changes in the intensity, location patterns and time course of fluorescent labelling in each examined stage in the otolithic organs was studied. The presence and distribution of calbindin (CB)-D28K, one of the calcium-binding proteins constantly found in the mammalian and chicken cochlea and also in otolithic membrane of some adult mammals, was studied. Results in embryonal stages, postnatal and adult animals allow us to postulate that otoliths are mainly produced during the embryonal phase, but they may also be produced throughout the whole life span. Results also indicate that otoconia are dynamic structures which undergo turnover. The correspondence between the patterns of CB-D28K immunoreactivity and tetracycline fluorescence may indicate that CB-D28K participates in the formation of otoconia.

Incidence of occult cancer in children presenting with musculoskeletal symptoms: a 10-year survey in a pediatric rheumatology unit.

OBJECTIVES: To assess the frequency and types of cancer found in children presenting to our Unit with musculoskeletal symptoms over a 10-year period. METHODS: The medical records of patients with musculoskeletal symptoms and a final diagnosis of cancer were reviewed. In each case age, gender, presenting symptoms, laboratory data, diagnostic procedures, provisional and final diagnoses, and time between clinical onset and correct diagnosis were reviewed. RESULTS: An underlying neoplasia was found in 10 of 1,254 patients (<1%) complaining of musculoskeletal symptoms. The types of malignancies found included acute lymphocytic leukemia (ALL) (6 cases), lymphoma (2 cases), neuroblastoma (1 case), and Ewing's sarcoma (1 case). The mean time between disease onset and final diagnosis was 3.2 months. The most common presenting feature was monoarthritis, involving the larger joints such as the elbows, knees or ankles. Juvenile idiopathic arthritis (JIA) was the most frequent provisional diagnosis. In the preliminary hematologic evaluation, eight patients had an increased erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) value. White blood cell (WBC) count was normal in almost all children, with a normal differential count. Lactic dehydrogenase (LDH) was raised in all children. Bone marrow aspirates and lymph node or bone biopsies were necessary to reach the final diagnosis. CONCLUSIONS: A malignancy should always be excluded in children with musculoskeletal symptoms, especially when the clinical pattern is not characteristic of a specific rheumatic disease. Routine laboratory tests may be misleading. The simultaneous presence of high LDH or alpha-hydroxybutyric dehydrogenase (alpha-HBDH) levels and raised ESR or CRP, even with normal blood cell counts, should lead to additional investigations. RELEVANCE: All patients presenting with arthritis or other musculoskeletal symptoms should have a thorough clinical examination. Disproportionate pain levels and an atypical pattern of "arthritis," especially in the presence of systemic manifestations, suggest a possible underlying malignancy.

Pulmonary function in children affected by juvenile spondyloarthropathy.

OBJECTIVE: Pleuropulmonary involvement in adult spondyloarthropathy (SpA) has been thoroughly investigated. SpA is usually detected by conventional radiology as fibrosis of the upper lobes in about 30% of asymptomatic patients. Pulmonary function tests (PFT) reveal decreased vital capacity and total lung capacity, as well as increased residual volume. Juvenile SpA (JSpA) is a rare clinical condition, and no extensive investigations have been carried out on pulmonary involvement in JSpA. We studied prevalence and features of PFT alterations in patients with JSpA over a 2 year followup and analyzed the relationship between PFT and disease duration, disease activity, and chest and spine mobility. METHODS: Eighteen patients with JSpA, with no clinical signs of lung disease and normal chest radiographs, underwent PFT--static and dynamic volumes, diffusing capacity for carbon monoxide (DLCO), at enrollment (T0), at 12 months (T1), and at 24 months later (T2). Disease activity was defined at each investigation by clinical and hematological data. RESULTS: Significant functional lung impairment was detected in 33% of patients (reduced forced vital capacity in 22% and DLCO in 11%). No significant change in the prevalence and features of PFT alterations was detected at T1 and T2; no relationship was found between PFT and duration, activity, and clinical scores of the disease. CONCLUSION: Thirty-three percent of JSpA patients without clinical symptoms and no radiological findings of lung involvement show PFT alterations, mainly characterized by a restrictive pattern. No progression or modification in PFT developed over 2 years. No correlation was found between PFT and disease duration, activity, and clinical scores.

Human parvovirus B19 infection: its relationship with systemic lupus erythematosus.

OBJECTIVES: The clinical presentation and outcome of four cases of human parvovirus-B19 (HPV-B19) infection, initially diagnosed as systemic lupus erythematosus (SLE), were reviewed and compared with similar cases previously reported in the literature. The relationship between HPV-B19 infection and SLE is discussed. METHODS: The medical records of four patients with documented HPV-B19 infection, initially diagnosed as SLE, were reviewed and studied in detail. A Medline search from 1985 to 1997 was performed to identify other cases reported in the literature in which a relationship between HPV-B19 and SLE had been identified in both adults and children. RESULTS: In all of our cases, the clinical findings (fever, rash, arthritis and malaise) and hematologic data (leukopenia, thrombocytopenia, anemia, presence of autoantibodies, hypocomplementemia, etc.) had initially suggested a diagnosis of juvenile SLE. Subsequently, evidence of HPV-B19 infection at the time of clinical presentation was ascertained. In three of these cases, the disease course was self-limiting with complete clinical remission and normalization of hematologic abnormalities within 18 months; one case, however, had persistent disease activity and repeated exacerbations. CONCLUSIONS: The occurrence of HPV-B19 infection has been documented in patients with SLE, in particular in relation to disease onset. Similarities in clinical and immunological features of viral infections and SLE at presentation may hinder the differential diagnosis between these two conditions. The family history, a self-limiting disease course and certain disease specific clinical aspects may help the pediatrician formulate an accurate diagnosis. In our patients, HPV-B19 infection may have mimicked the onset of SLE in three cases, but triggered the disease in one.