Water droplet evaporation in varied gravity and electric fields.

Sessile water droplet evaporation in varied gravity and electric fields has been experimentally studied. Specifically, the influences of gravity and electric fields are investigated in the context of the heat flux distribution beneath the droplets, as well as the droplet mechanics and resulting shapes. Experimental testing was carried out during a European Space Agency (ESA) Parabolic Flight Campaign (PFC 66). The droplets tested evaporated with a pinned contact line, a single wettability condition, and varied droplet volume and substrate heat flux. The peak heat transfer was located at the contact line for all cases. The peak heat flux, average heat flux, and droplet evaporation rate were shown to vary strongly with gravity, with higher values noted for hypergravity conditions and lower values in microgravity conditions. The droplet thermal inertia was shown to play a significant role, with larger droplets taking more time to reach thermal equilibrium during the parabolic testing period. No significant impact of the electric field on the droplet evaporation was noted for these test conditions.

Reversal of rocuronium induced neuromuscular block with sugammadex or neostigmine: a large observational study.

BACKGROUND: This 'real-life' study aimed to analyze the time from the start of neostigmine or sugammadex administration to recovery to a train of four ratio (TOFr) of 0.9 in a real-life in patients receiving rocuronium. The secondary aims were to assess the proportion of patients: presenting TOFr < 0.9 after 5, 10, and 20 min from reversal agent administration, receiving opioids for intraoperative analgesia and extubated in the post-anesthesia care unit (PACU). METHODS: This was a multisite, prospective, nonrandomized, observational real-life study. Reversal agent was administered at either T2 reappearance or at a post-tetanic count of 1 or 2. Drugs dosages were free according to each investigator's usual practice. RESULTS: Three hundred fifty-nine patients were enrolled onto the study. Time from reversal administration to TOFr to 0.9 is significantly faster in the sugammadex group than in the neostigmine group (shallow block: 2.2 vs. 6.9 min, respectively; P < 0.0001; deep block: 2.7 vs. 16.2 min, respectively; P < 0.0001). The number of patients with TOFr < 0.9 at 5, 10, and 20 min post-reversal agent administration was higher in the neostigmine than in the sugammadex group. Just five patients did not receive opioids. All patients were extubated in the operative room except for a single patient in the sugammadex group who was extubated following PACU admission. CONCLUSIONS: This real-life study confirms that reversal time is faster in patients receiving sugammadex than in those receiving neostigmine. TOFr < 0.9 20 min after reversal was only present in patients treated with neostigmine.

Bubble growth analysis during subcooled boiling experiments on-board the international space station: Benchmark image analysis.

This work compares four different image processing algorithms for the analysis of image data obtained during the Multiscale Boiling Experiment of ESA, executed on-board the International Space Station. Two separate experimental campaigns have been performed in 2019 and 2020, aiming to investigate boiling phenomena in microgravity, with and without the presence of shear flow and electric field. A heated substrate, at the bottom of the test cell, creates a temperature profile across the liquid bulk above it. A laser beam hits a designated microcavity at the middle of the substrate, to initiate nucleation of a single, isolated bubble. In the presence of shear flow or electric field forces, the bubble slides or detaches respectively, leaving the cavity free for the nucleation and growth of a new bubble. The growth of such a bubble within the prescribed temperature profile is studied for varying experimental conditions (i.e. pressure, heat flux, subcooling temperature) by capturing high speed, black and white video images. The presence of light reflections at random locations around the bubble contour vary with bubble size and population. This, combined with the refraction induced optical distortion of vertical image dimension close to the heater, make the accurate detection of bubbles contour a real challenge. Four research teams, namely the University of Pisa (UNIPI), the Institute of Fluid Mechanics of Toulouse (IMFT), the joint group of Aix Marseille University (AMU) and Kutateladze Institute of Thermophysics (IT), and the joined group of Aristotle University of Thessaloniki (AUTH), Technical University of Darmstadt (TUD) and Foundation of Research and Technology in Crete (FORTH), developed separate specialized algorithms to: a) detect bubble edges and b) use these edges to calculate basic bubble geometrical features, such as contact line diameter, bubble diameter and contact angles. These four different approaches diverge in complexity and concept. In the absence of reference measurements at microgravity conditions, measurements efficiency is evaluated based on the comparison of the estimated bubble geometrical features along with pertinent physical arguments. Results show that the efficiency of each approach varies with the nature of measurement. The studied benchmark dataset is published allowing other research groups to test further their own image processing algorithms.

Effect of postconditioning on mitochondrial dysfunction in experimental aortic cross-clamping.

BACKGROUND: Cross-clamping of the aorta during abdominal aortic aneurysm surgery induces muscle ischaemia with resultant morbidity. This study tested whether ischaemic postconditioning would decrease mitochondrial dysfunction in skeletal muscle by reducing oxidative stress. METHODS: Three groups (9 rats each) underwent surgery, including a control group without ischaemia and an ischaemia-reperfusion group that had 3 h ischaemia induced by aortic clamping and collateral vessel ligation, followed by 2 h of reperfusion. The third group had ischaemia for 3 h then underwent postconditioning comprising three short intervals of ischaemia-reperfusion at the onset of reperfusion. Activity of complexes I, II, III and IV of the mitochondrial respiratory chain was monitored in gastrocnemius muscle, along with oxidative stress measured by dihydroethidium (DHE) staining and antioxidant defence determined by measurement of glutathione levels. RESULTS: Ischaemia-reperfusion alone caused a significant reduction in maximal oxidative capacity (-31.8 per cent; P = 0.002), activity of complexes II, III and IV (-34.5 per cent; P = 0.007) and complex IV activity (-30.6 per cent; P = 0.039). It also increased reactive oxygen species (DHE staining increased to 223.1 per cent of control value; P = 0.027) and reduced antioxidant defence (glutathione level -28.6 per cent; P = 0.039). Postconditioning counteracted these deleterious effects by increasing mitochondrial complex I, II, III and IV activities, restoring muscle DHE staining and preserving glutathione content. CONCLUSION: Ischaemic postconditioning protects skeletal muscle mitochondria against ischaemia-reperfusion injury by reducing oxidative stress and preserving antioxidant defence in an experimental model. Mitochondrial protection to reduce reperfusion injury in clinical vascular surgery may be warranted.

Knowledge of residual curarization: an Italian survey.

BACKGROUND: The use of neuromuscular blocking agents (NMBAs) is widespread in anesthetic practice; little is known about the current use of these drugs in Italy. This survey was conducted to obtain information about the most commonly used clinical tests and the train-of-four (TOF) ratios that are considered as being reliable for assessing recovery from neuromuscular blockade at the end of anesthesia and the estimated occurrence rates of post-operative paralysis in Italian hospitals. METHODS: The questionnaire was given to Italian anesthesiologists attending the 62nd National Congress of the Italian Society of Anesthesia, Analgesia and Intensive Therapy. Collected data were stratified by age and the total number of surgical procedures performed in the hospitals concerned. RESULTS: Seven hundred and fifty-four correctly compiled questionnaires were collected (response rate 88.7%). Seventy three percent of the respondents only used clinical tests for monitoring the level of neuromuscular blockade. The main clinical tests cited for the evaluation of residual paralysis were keeping the head lifted up for 5 s, protruding the tongue and opening the eyes. TOF was used by 35% of the respondents on a routine basis. Only 24% of the interviewed anesthesiologists reported that before extubation, a TOF ratio of at least 0.9 should be reached. CONCLUSIONS: Most Italian anesthetists assess the recovery from neuromuscular blockade only by clinical signs. There is poor awareness about the inability of such techniques to indicate even a significant amount of residual neuromuscular block. A more extensive use of quantitative instrumental monitoring is required for the more rational use of NMBAs.

Measurement of the sensitivity of different commercial assays in the diagnosis of CMV infection in pregnancy.

To evaluate the performance of different commercial assays for the detection of recent cytomegalovirus (CMV) in pregnancy, the sensitivity and specificity of assays for CMV-specific IgM antibodies were compared. Routine specimens from pregnant women were screened for CMV IgM using the Abbott AxSYM assay. Sera that were reactive according to AxSYM were further tested for IgM by other commercial assays. In selected IgM positive samples a CMV IgG avidity assay (Radim) and virus isolation from urine (shell vial) were also performed. The positivity rate for IgM anti-CMV by AxSYM was relatively high (140 out of 492, combining reactive and grayzone results). Only 26 of the 140 samples were positive for IgM according to Radim. The IgG avidity was low in 16 of the 43 samples tested, and the Radim and DiaSorin IgM assays were negative in 5 of them; 2 of the latter cases were also positive for viral isolation according to a shell vial method. There are differences in the sensitivity of the commercially available tests for CMV antibodies. CMV screening in pregnancy is performed as a first step by immunoassays and the choice of highly sensitive IgM test associated with further serological and virological methods could help to identify early primary infections.

Does circulating BNP normalize after heart transplantation in patients with normal hemodynamic and right and left heart functions?

BACKGROUND: Increased brain natriuretic peptide (BNP) in cardiovascular disease is thought to be a compensatory protective mechanism allowing to delay the occurrence of terminal heart failure. Heart transplantation should normalize the neuroendocrine balance but BNP remains elevated in stable heart-transplant recipients (Htx). Such increase has been related to persistent endothelial and cardiac dysfunctions. The purpose of this study was to determine whether selected Htx, presenting with normal hemodynamic and cardiac systolic and diastolic functions on both side of the heart, show a normalization of their BNP plasma values. METHODS: Of a cohort of well-being 26 Htx, we selected 12 patients with normal hemodynamics and left and right heart systolic and diastolic functions and compared their circulating BNP, cyclic guanosine monophosphate (cGMP) (the BNP second messenger) and endothelin-1 (ET) values with that of 12 age-, body mass index- and mean arterial pressure-matched controls. Cardiac function determination by echodoppler included cardiac filling pressures assessment using tissue Doppler imaging. Blood samples for biological and hormonal determinations were drawn at rest, within 15 min before echocardiography. RESULTS: As selected, hemodynamic and left and right heart systolic and diastolic functions were located in the normal range in Htx. Plasma ET value was also similar in Htx and controls (20.7 +/- 0.9 vs. 19.6 +/- 0.9 fmol/mL). However, circulating BNP, like cGMP, was still significantly increased after heart transplantation, when compared with controls (33.8 +/- 8.5 vs. 4.0 +/- 0.9 pg/mL, p = 0.002 and 8.2 +/- 1.1 vs. 4.4 +/- 0.3 nmol/L, p = 0.003) for BNP and cGMP, respectively, in Htx and controls. Interestingly, the sole correlation observed was between BNP and cGMP (r = 0.85, p < 0.0001) after heart transplantation. CONCLUSIONS: After heart transplantation, BNP remained increased despite the normalization of hemodynamic and cardiac systolic and diastolic functions. This suggests that such endocrine heart stimulation should not be viewed only as a hemodynamic marker in Htx. Further studies will be useful to investigate the role of pro-inflammatory cytokines and whether elevated BNP still possesses antifibrotic properties, further supporting the interest of enhancing its activity after heart transplantation.

Differentiation and characterization of rat adipose tissue mesenchymal stem cells into endothelial-like cells.

In this study, mesenchymal stem cells were isolated from rat adipose tissue (AD-MSCs) to characterize and differentiate them into endothelial-like cells. AD-MSCs were isolated by mechanical and enzymatic treatments, and their identity was verified by colony-forming units (CFU) test and by differentiation into cells of mesodermal lineages. The endothelial differentiation was induced by plating another aliquot of cells in EGM-2 medium, enriched with specific endothelial growth factors. Five subcultures were performed. The expression of stemness genes (OCT4, SOX2 and NANOG) was investigated. The presence of CD90 and the absence of the CD45 were evaluated by flow cytometry. The endothelial-like cells were characterized by the evaluation of morphological changes and gene expression analysis for endothelial markers (CD31, CD144, CD146). Characterization of AD-MSCs showed their ability to form clones, to differentiate in vitro and the OCT-4, SOX-2, NANOG genes expression. Immunophenotypic characterization showed the CD90 presence and the CD45 absence. The endothelial-like cells showed morphological changes, the expression of CD31, CD144, CD146 genes and the presence of CD31 membrane receptor. Matrigel assay showed their ability to form network and vessels-like structures. This study lays the foundations for future evaluation of the potential AD-MSCs pro-angiogenic and therapeutic role.

Evidence for Tunisian-Like Pestiviruses Presence in Small Ruminants in Italy Since 2007.

The genus Pestivirus, which belongs to the Flaviviridae family, includes ssRNA+ viruses responsible for infectious diseases in pigs, cattle, sheep, goats and other domestic and wild ruminants. Like most of the RNA viruses, pestivirus has high genome variability with practical consequences on disease epidemiology, diagnosis and control. In addition to the officially recognized species in the genus Pestivirus, such as BVDV-1, BVDV-2, BDV and CSFV, other pestiviruses have been detected. Furthermore, most of the ruminant pestiviruses show low or absent species specificity observed in serological tests and are able to infect multiple species. Particularly, small ruminants are receptive hosts of the most heterogeneous group of pestiviruses. The aim of this study was to carry out the molecular characterization of pestiviruses isolated from sheep and goats in Sicily, Italy. Phylogenetic analysis of two viral genomic regions (a fragment of 5'-UTR and the whole Npro regions) revealed the presence of different pestivirus genotypes in the analysed goat and sheep herds. Two of five viral isolates were clustered with BVDV-1d viruses, a strain widespread in Italy, but never reported in Sicily. The other three isolates formed a distinct cluster with high similarity to Tunisian isolates, recently proposed as a new pestivirus species. This represents the first evidence for Tunisian-like pestivirus presence in small ruminants in Italy. Furthermore, one of the isolates was collected from a goat, representing the first isolation of Tunisian-like pestivirus from this species.

Rett syndrome: a wide clinical and autonomic picture.

BACKGROUND: Rett Syndrome is a neurodevelopmental disorder almost exclusively affecting females, characterized by a broad clinical spectrum of signs and symptoms and a peculiar course. The disease affects different body systems: nervous, muscolo-skeletal, gastro-enteric. Moreover, part of the symptoms are related to the involvement of the autonomic nervous system. In the Tuscany Rett Center at Versilia Hospital, we collected data from 151 subjects with a clinical diagnosis of classical or variant RTT syndrome. For each subject, we assessed the severity of the condition with clinical-rating scales (ISS, PBZ), we quantified the performance of the autonomic nervous system, and we performed genetic analysis. We used multivariate statistical analysis of the data to evaluate the relation between the different clinical RTT forms, the cardiorespiratory phenotype, the different genetic mutations and the severity of the clinical picture. Individuals were classified according to existing forms: Classical RTT and three atypical RTT: Z-RTT, Hanefeld, Congenital. A correlation between C-Terminal deletions and lower severity of the clinical manifestations was evident, in the previous literature, but, considering the analysis of autonomic behaviour, the original classification can be enriched with a more accurate subdivision of Rett subgroups, which may be useful for early diagnosis. RESULTS: Present data emphasize some differences, not entirely described in the literature, among RTT variants. In our cohort the Z-RTT variant cases show clinical features (communication, growth, epilepsy and development), well documented by specific ISS items, less severe, if compared to classical RTT and show autonomic disorders, previously not reported in the literature. In this form epilepsy is rarely present. In contrast, Hanefeld variant shows the constant presence of epilepsy which has an earlier onset In Hanefeld variant the frequency of apneas was rare and, among the cardiorespiratory phenotypes, the feeble type is lacking. CONCLUSION: A quantitative analysis of the different autonomic components reveals differences across typical and atypical forms of RTT that leads to a more accurate classification of the groups. In our cohort of RTT individuals, the inclusion of autonomic parameter in the classification leads to an improved diagnosis at earlier stages of development.

Wettability influence on the onset temperature of pool boiling: Experimental evidence onto ultra-smooth surfaces.

In this article we study systematically the effect of wettability on the onset of boiling on the same nanometrically smooth surface. By grafting different monolayers of molecules, we were able to explore the wettability from the equilibrium static contact angle, θ0=0° to θ0=110°, without changing the surface topography. The superheat temperature at the onset of pool boiling was measured and eventually a non-classical trend of TONB as a function of wettability was observed. The nucleation site densities for the different grafting cases were also measured by image analysis. Moreover, we propose a novel theoretical interpretation to this phenomenon linking nucleation and the molecular diffusion coefficient. MD simulation results support this approach.

Bovine generalised glycogenosis type II. Uptake of lysosomal alpha-glucosidase by cultured skeletal muscle and reversal of glycogen accumulation.

acid alpha-glucosidase (EC 3.2.1.20) was purified from fetal bovine muscle by affinity chromatography on concanavalin A and Sephadex G-100 and added to the culture medium of mature muscle cultures from animals affected by glycogenosis type II. The enzyme activity in homogenates of treated cultures was significantly increased within 4 h of the addition of enzyme, was maximal by 18 h and the internalised activity was stable for at least 48 h after the removal of the enzyme from the culture medium. The acid alpha-glucosidase activity was internalised with an uptake constant of 300 nM and a Vmax of uptake of 133 nmol/h per mg protein. The glycogen concentration in affected cultures treated with exogenous acid alpha-glucosidase for 24 h had decreased by 20% and after a further 24 h of culture was comparable to the concentration observed in cultures from non-affected animals.

Natural bone marrow transplantation in cattle with Pompe's disease.

Adding acid alpha-glucosidase to cultures of Pompe's disease muscle has resulted in enzyme uptake and reduction in concentration of glycogen. However, bone marrow transplantation has been unsuccessful as a treatment. Immune rejection may have contributed to this failure. Twin calves share a placenta and carry lymphoreticular cells of each other's type, they become lymphoreticular chimeras in utero and immune rejection does not occur. One natural and three sets of twins produced by embryo transfer were studied in Pompe's disease cattle. Chimerism persisted throughout life and the situation was analogous to a transplant of histocompatible bone marrow stem cells. The activity of acid alpha-glucosidase in leucocytes and in biopsies of the semitendinosus muscle and the mean activity in diaphragm, spleen and lymph node obtained after death from affected twins were significantly higher than in single affected calves. Glycogen concentration was lowered in liver, spleen and lymph node but not in muscles. The affected twins showed clinical signs and changes in muscle similar to those seen in affected single calves. It is concluded that bone marrow transplantation is unlikely to be a successful treatment for Pompe's disease.

Membrane interactions involved in the induction of interferon-alpha by Mycoplasma pneumoniae.

Interferon (IFN) induction by Mycoplasma pneumoniae (MP) in human peripheral blood mononuclear cells (PBMC) has been studied. We show that IFN yields depend on the concentration of both lymphocytes and MP. The effective IFN inducer appears to be the mycoplasma membrane, and IFN production occurs without significant lymphocyte proliferation. Data obtained by both positive and negative selection experiments suggest that the PBMC subpopulation induced by MP is B lymphocyte, as opposed to the main subpopulation induced by NDV that is monocyte. Evidence is also provided suggesting that the membrane interaction between MP and the B cells is mediated by MHC Class II antigens.

Pool film boiling experiments on a wire in low gravity: preliminary results.

This paper reports preliminary results for pool film boiling on a wire immersed in almost saturated FC72 recently obtained during an experimental campaign performed in low gravity on the European Space Agency Zero-G airplane, (reduced gravity level 10(-2)). This is part of a long-term research program on the effect of gravitational and electric forces on boiling. The reported data set refers to experiments performed under the following conditions: (1) Earth gravity without electric field, (2) Earth gravity with electric field, (3) low gravity without electric field, and (4) low gravity with electric field. Although a decrease of gravity causes a heat transfer degradation, the electric field markedly improves heat exchange. This improvement is so effective that, beyond a certain field value, the heat flux is no longer sensitive to gravity. Two main film boiling regimes have been identified, both in normal and in low gravity: one is affected by the electric field and the other is practically insensitive to the field influence.

Further study on the specificity and incidence of neutralizing antibodies to interferon (IFN) in relapsing remitting multiple sclerosis patients treated with IFN beta-1a or IFN beta-1b.

The development of neutralizing antibodies (NAbs) to interferon (IFN) is a common phenomenon of IFN beta therapy for relapsing-remitting multiple sclerosis (RRMS) patients. Here we examine the specificity of NAbs developed during therapy for RRMS with recombinant interferon (rIFN) beta-1a or rIFN beta-1b, and study the effect of switching from rIFN beta-1a to rIFN beta-1b on the incidence and specificity of NAbs. The relative ability to neutralize rIFN beta-1a and beta-1b was assayed in sera positive for NAbs derived from RRMS patients treated with either rIFN beta-1a (N=9) or rIFN beta-1b (N=16), while the incidence and specificity of NAbs to IFN beta developed during therapy were studied in 50 RRMS patients who were treated for two years with rIFN beta-1a followed by a further year either switching to rIFN beta-1b (N=34) or continuing treatment with rIFN beta-1a (N=16). The results show that all positive sera, independent of the source, may recognize both forms of rIFN beta and that a further year of treatment does not significantly affect the incidence and specificity of the NAbs developed during the first two years of treatment even if treatment is switched to a different type of IFN beta. The data then suggests that it is unlikely that the administration of rIFN beta-1b to anti-rIFN beta-1a NAbs-positive patients can overcome the inhibitory effect exerted by the serum antibodies (and vice versa), and that a further period of treatment with IFN beta-1b in patients previously treated with rIFN beta-1a does not significantly change the pattern of antibody response to IFN beta.

Evaluating hazardous air pollutants in Australia.

Hazardous air pollutants (HAPS) have been evaluated for their health and environmental significance on a targeted and campaign basis in Australia until recently. Individual States and Territories have been undertaking targeted monitoring studies and have numerous control strategies aimed at controlling HAPS emissions with the focus largely on volatile organic compounds (VOCs), polycyclic aromatic hydrocarbons (PAHs) and selected heavy metals, with some limited work on exposure assessment. There has been little evaluation of the potential health or environmental effects of the monitored concentrations of these substances and few toxicological or epidemiological studies have been conducted-none in the community setting in Australia. Moreover, there has not been an agreed method for assessing risks from HAPS in ambient air, with different jurisdictions utilising different international benchmarks. Recently, the National Environmental Health (EnHealth) Council commenced developing a risk assessment methodology, which is being used in the development of ambient air quality guidelines for selected HAPS in Western Australia. In 1999, the Commonwealth Government established the Living Cities-Toxics Program, designed to assess the state of knowledge on HAPS in Australia with the aims of identifying and prioritising HAPS, identifying information gaps and informing the development of national air quality standards and national management strategies. The Commonwealth Government commenced a number of projects in 2000 to progress these aims.

Carcinogen risk assessment. Can we harmonise?

Harmonisation of risk assessment (RA) is one of the priorities for sound chemical management set by Chapter 19, Agenda 21 of the United Nations Conference on the Environment and Development (UNCED) 1992 Earth Summit. The benefits of harmonisation are self evident and include transportability and consistency of RA outcomes, transparency and efficiency of process, and credible science. The outcomes of carcinogen RA are a description or classification of the carcinogenic hazard, the conditions under which cancers may be induced, and an estimate of a dose or exposure which poses a minimal, or otherwise defined, risk in exposed human populations. Weight-of-evidence based systems which classify carcinogenic hazards are part of, but do not substitute for, the risk assessment process. Carcinogen RA is based on assessment of appropriate toxicological and exposure data sets, which may have much in common. However, national policy frameworks can differ to the extent that RA outcomes may be quite different for the same chemical(s). Historically, differences in science policy have been greater for cancer RA compared to other toxic endpoints, with a tendency to differentiate cancer RA on the basis of presumed mechanism (i.e. genotoxic or non-genotoxic) and relevance to humans (some carcinogenic responses in animals may be considered not relevant for human RA). Significant strides towards harmonisation are being made, with reassessment of some national policies and participation in international harmonisation programmes, such as the ones being managed by the International Programme for Chemical Safety (IPCS). Alternative approaches to quantitative carcinogen RA are being considered which are more amenable to harmonisation, and one such approach being developed in Australia in connection with contaminated sites will be discussed.

Cyclophosphamide plus vincristine and prednisone in the treatment of severe pemphigus vulgaris refractory to conventional therapy.

Five patients with severe pemphigus vulgaris refractory to conventional therapy with azathioprine and corticosteroids were treated with cyclophosphamide, vincristine and prednisone. One patient was not evaluable, while the remaining four patients showed a complete response. Duration of response was in the range of 13-94 months. Toxicity was mainly represented by alopecia, myelosuppression and gastrointestinal side-effects such as nausea/vomiting. Although cyclophosphamide and vincristine may induce severe side-effects, this association may be useful in controlling severe disease resistant to previous conventional therapies.

Biologic relevance of elevated red cell adenosine deaminase activity in myelodysplastic syndromes and paroxysmal nocturnal hemoglobinuria.

Red cell adenosine deaminase (ADA-RBC) activity in patients with myelodysplastic syndromes and paroxysmal nocturnal hemoglobinuria is significantly increased compared to that observed in normal controls. ADA-RBC activity is not related to fetal hemoglobin concentration, but it is significantly correlated with hemoglobin concentration at diagnosis and with the degree of morphologic dysplasia in the erythroid lineage. The results of our study suggest that the observed enzymatic abnormality may constitute a non-specific manifestation of the stem cell alteration that determines these disorders.