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The forehead is the cranial part of the forehead, and represents an important component of facial esthetics. The deformations linked to craniosynostoses are characterized by modifications in height, width, and angulation. Their surgical correction during childhood is based on well-established techniques of remodeling using resorbable osteosynthesis. Today, distraction and springs allow less invasive procedures with good outcomes. Sequellae mainly correspond to hypotrophies of the temporal region, corrected with fat grafting. The presence of a mutation increases the risk of reoperation in the first six years of life.
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OBJECTIVE: Continuous positive airway pressure (CPAP) is an important therapeutic tool in COVID-19 acute respiratory distress syndrome (ARDS) since it improves oxygenation, reduces respiratory rate and can prevent intubation and intensive care unit (ICU) admission. CPAP during pronation has seldom been described and never during sedation. DESIGN: Case series. SETTING: High dependency unit of San Carlo University Hospital (Potenza, Italy). PATIENTS: Eleven consecutive patients with COVID-19 ARDS. INTERVENTION: Helmet CPAP in prone position after failing a CPAP trial in the supine position. MAIN VARIABLE OF INTEREST: Data collection at baseline and then after 24, 48 and 72h of pronation. We measured PaO2/FIO2, pH, lactate, PaCO2, SpO2, respiratory rate and the status of the patients at 28-day follow up. RESULTS: Patients were treated with helmet CPAP for a mean±SD of 7±2.7 days. Prone positioning was feasible in all patients, but in 7 of them dexmedetomidine improved comfort. PaO2/FIO2 improved from 107.5±20.8 before starting pronation to 244.4±106.2 after 72h (p<.001). We also observed a significantly increase in Sp02 from 90.6±2.3 to 96±3.1 (p<.001) and a decrease in respiratory rate from 27.6±4.3 to 20.1±4.7 (p=.004). No difference was observed in PaCO2 or pH. At 28 days two patients died after ICU admission, one was discharged in the main ward after ICU admission and eight were discharged home after being successfully managed outside the ICU. CONCLUSIONS: Helmet CPAP during pronation was feasible and safe in COVID-19 ARDS managed outside the ICU and sedation with dexmedetomidine safely improved comfort. We recorded an increase in PaO2/FIO2, SpO2 and a reduction in respiratory rate.
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COVID-19 , Síndrome de Dificultad Respiratoria , Presión de las Vías Aéreas Positiva Contínua , Humanos , Unidades de Cuidados Intensivos , Pronación , Síndrome de Dificultad Respiratoria/terapia , SARS-CoV-2 , VigiliaRESUMEN
PURPOSE: Infantile hydrocephalus has various etiologies that may influence children's cognitive development and onset of neurological comorbidities such as epilepsy. However, few studies specifically analyzed etiologies encountered in this population. Here we report a 9-year retrospective analysis of the etiologies and short-term outcome of surgically treated patients in a major pediatric neurosurgical center in a high-income country. METHODS: This was a single-center retrospective study for the period 2010 to 2018 using the database of the French medical classification for clinical procedures (CCAM) of the Necker Hospital, Paris. We included all the patients surgically treated for hydrocephalus before the age of 2 years and reviewed digital medical files and MRI. RESULTS: Four hundred and sixty seven patients were included, with a mean age at diagnosis of 4.8 months and male predominance (M/F ratio=1/2). Etiologies comprised: intraventricular hemorrhage (27.8%), arachnoid cyst (13.9%), spinal dysraphism (12.4%), brain tumor (10.5%), associated brain malformation (8.6%), infection (7.5%), isolated aqueduct stenosis (5.1%), and other (14.1%). Epilepsy was more likely to occur in post-infection (40%) and post-hemorrhage (31%) hydrocephalus, and when brain malformation was associated (35%). Etiology, epileptic status and the number of dysfunctions influenced short-term school performance. CONCLUSION: This study identified various etiologies of infantile hydrocephalus, with different neurological outcomes. Specific follow-up is required according to these observations.
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Quistes Aracnoideos , Hidrocefalia , Disrafia Espinal , Quistes Aracnoideos/cirugía , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Lactante , Masculino , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Inorganic scintillators are widely used for fast timing applications in high-energy physics (HEP) experiments, time-of-flight positron emission tomography and time tagging of soft and hard x-ray photons at advanced light sources. As the best coincidence time resolution (CTR) achievable is proportional to the square root of the scintillation decay time it is worth studying fast cross-luminescence, for example in BaF2which has an intrinsic yield of about 1400 photons/MeV. However, emission bands in BaF2are located in the deep-UV at 195 nm and 220 nm, which sets severe constraints on photodetector selection. Recent developments in dark matter and neutrinoless double beta decay searches have led to silicon photomultipliers (SiPMs) with photon detection efficiencies of 20%-25% at wavelengths of 200 nm. We tested state-of-the-art devices from Fondazione Bruno Kessler and measured a best CTR of 51 ± 5 ps full width at half maximum when coupling 2 mm × 2 mm × 3 mm BaF2crystals excited by 511 keV electron-positron annihilation gammas. Using these vacuum ultraviolet SiPMs we recorded the scintillation kinetics of samples from Epic Crystal under 511 keV excitation, confirming a fast decay time of 855 ps with 12.2% relative light yield and 805 ns with 84.0% abundance, together with a smaller rise time of 4 ps beyond the resolution of our setup. The total intrinsic light yield was determined to be 8500 photons/MeV. We also revealed a faster component with 136 ps decay time and 3.7% light yield contribution, which is extremely interesting for the fastest timing applications. Timing characteristics and CTR results on BaF2samples from different producers and with different dopants (yttrium, cadmium and lanthanum) are given, and clearly show that the the slow 800 ns emission can be effectively suppressed. Such results ultimately pave the way for high-rate ultrafast timing applications in medical diagnosis, range monitoring in proton or heavy ion therapy and HEP.
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Luminiscencia , Conteo por Cintilación , Fotones , Tomografía de Emisión de Positrones , VacioRESUMEN
OBJECTIVE: Continuous positive airway pressure (CPAP) is an important therapeutic tool in COVID-19 acute respiratory distress syndrome (ARDS) since it improves oxygenation, reduces respiratory rate and can prevent intubation and intensive care unit (ICU) admission. CPAP during pronation has seldom been described and never during sedation. DESIGN: Case series. SETTING: High dependency unit of San Carlo University Hospital (Potenza, Italy). PATIENTS: Eleven consecutive patients with COVID-19 ARDS. INTERVENTION: Helmet CPAP in prone position after failing a CPAP trial in the supine position. MAIN VARIABLE OF INTEREST: Data collection at baseline and then after 24, 48 and 72h of pronation. We measured PaO2/FIO2, pH, lactate, PaCO2, SpO2, respiratory rate and the status of the patients at 28-day follow up. RESULTS: Patients were treated with helmet CPAP for a mean±SD of 7±2.7 days. Prone positioning was feasible in all patients, but in 7 of them dexmedetomidine improved comfort. PaO2/FIO2 improved from 107.5±20.8 before starting pronation to 244.4±106.2 after 72h (p<.001). We also observed a significantly increase in Sp02 from 90.6±2.3 to 96±3.1 (p<.001) and a decrease in respiratory rate from 27.6±4.3 to 20.1±4.7 (p=.004). No difference was observed in PaCO2 or pH. At 28 days two patients died after ICU admission, one was discharged in the main ward after ICU admission and eight were discharged home after being successfully managed outside the ICU. CONCLUSIONS: Helmet CPAP during pronation was feasible and safe in COVID-19 ARDS managed outside the ICU and sedation with dexmedetomidine safely improved comfort. We recorded an increase in PaO2/FIO2, SpO2 and a reduction in respiratory rate.
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Syndromic craniosynostoses are defined by the premature fusion of one or more cranial and facial sutures, leading to skull vault deformation, and midfacial retrusion. More recently, mandibular shape modifications have been described in FGFR-related craniosynostoses, which represent almost 75% of the syndromic craniosynostoses. Here, further characterisation of the mandibular phenotype in FGFR-related craniosynostoses is provided in order to confirm mandibular shape modifications, as this could contribute to a better understanding of the involvement of the FGFR pathway in craniofacial development. The aim of our study was to analyse early mandibular morphology in a cohort of patients with FGFR2- (Crouzon and Apert) and FGFR3- (Muenke and Crouzonodermoskeletal) related syndromic craniosynostoses. We used a comparative geometric morphometric approach based on 3D imaging. Thirty-one anatomical landmarks and eleven curves with sliding semi-landmarks were defined to model the shape of the mandible. In total, 40 patients (12 with Crouzon, 12 with Apert, 12 with Muenke and 4 with Crouzonodermoskeletal syndromes) and 40 age and sex-matched controls were included (mean age: 13.7 months ±11.9). Mandibular shape differed significantly between controls and each patient group based on geometric morphometrics. Mandibular shape in FGFR2-craniosynostoses was characterized by open gonial angle, short ramus height, and high and prominent symphysis. Short ramus height appeared more pronounced in Apert than in Crouzon syndrome. Additionally, narrow inter-condylar and inter-gonial distances were observed in Crouzon syndrome. Mandibular shape in FGFR3-craniosynostoses was characterized by high and prominent symphysis and narrow inter-gonial distance. In addition, narrow condylar processes affected patients with Crouzonodermoskeletal syndrome. Statistical analysis of variance showed significant clustering of Apert and Crouzon, Crouzon and Muenke, and Apert and Muenke patients (p < 0.05). Our results confirm distinct mandibular shapes at early ages in FGFR2- (Crouzon and Apert syndromes) and FGFR3-related syndromic craniosynostoses (Muenke and Crouzonodermoskeletal syndromes) and reinforce the hypothesis of genotype-phenotype correspondence concerning mandibular morphology.
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Acrocefalosindactilia , Disostosis Craneofacial , Craneosinostosis , Acrocefalosindactilia/diagnóstico por imagen , Acrocefalosindactilia/genética , Disostosis Craneofacial/diagnóstico por imagen , Disostosis Craneofacial/genética , Humanos , Lactante , Mandíbula/diagnóstico por imagen , Receptor Tipo 2 de Factor de Crecimiento de Fibroblastos/genética , Receptor Tipo 3 de Factor de Crecimiento de Fibroblastos/genética , SíndromeRESUMEN
The vault of the skull is a region of the neurocranium formed by a process of membranous ossification. It consists of several bones: frontal bone, parietal bone, squamous part of the temporal bone, lamina ascendens of the sphenoid, and interparietal bone. The embryological origin of the bones of the skull vault is still the subject of controversy. This can be explained by the different animal models used for these purposes, but also by the various techniques applied to this problem. At all events, it seems that the cells of the neural crest generate some of the bones of the vault and that the others are derived from the mesoderm. This uncertainty should lead readers to be extremely cautious before using the presumptive maps published in the literature. Several tissues interact with osteo-progenitor cells: neural tube, surface ectoderm and dura mater. Analysis of genes in which mutations lead to abnormalities of the skull vault has partly revealed the molecular interactions. These are very complex and are the field of very numerous experimental investigations. In the relatively near future, we can hope to discover some of the molecular networks leading to the formation of these bony structures.
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Cráneo/crecimiento & desarrollo , Animales , Femenino , Humanos , Cresta Neural/crecimiento & desarrollo , Embarazo , Cráneo/anatomía & histología , Cráneo/embriologíaRESUMEN
Secondary surgeries for single craniosynostosis surgeries are mainly esthetic refinements rather than functional indications. However, cranioplasties for bone defects correction or insufficient corrections may be undertaken. Management of syndromic craniosynostoses usually requires multiple surgical interventions, the sequence of which might vary per the genetic mutation. It is commonplace to start with posterior vault expansion before age 6 months, then treat cerebellar tonsillar herniation by the age of twelve months, and delay fronto-facial monobloc advancement until at least 18-24 months of age. Ventricular shunting is preferably avoided or delayed. Failure to respect these guidelines can significantly complicate the subsequent management. Primary fronto-orbital advancement or early facial osteotomy type Le Fort3, may compromise the subsequent fronto-facial monobloc advancement. However, this salvage secondary monobloc may be undertaken in some instances despite previous anterior osteotomies with a higher morbidity.
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Disostosis Craneofacial/cirugía , Craneosinostosis/cirugía , Procedimientos de Cirugía Plástica/métodos , Reoperación , Adolescente , Niño , Preescolar , Humanos , LactanteRESUMEN
PURPOSE: Diffusion tensor imaging (DTI) allows studying the micro and macro architecture. One of the major challenges in dysraphism is to know the morphologic organization of the spinal cord. In a preliminary work, spinal lipoma was chosen for analyzing the micro-architecture parameters and fiber morphology of the spinal cord by DTI with tractography. METHODS: Twelve patients (0-8 years) related to spinal lipomas treated between May 2017 and March 2018 were included. Tractography reconstruction of the conus medullaris of 12 patients were obtained using the MedINRIA software. The diffusion parameters have been calculated by Osirix DTImap plugin. RESULTS: We found a significant difference in the FA (p = 0.024) between two age groups (< 24 months old and > 24 months old). However, no significant differences in the mean values of FA, RD, and MD between the level of the lipoma and the level above were noted. The tractography obtained in each case was coherent with morphologic sequences and reproducible. The conus medullaris was deformed and shifted. Destruction or disorganization of fibers and any passing inside the lipomas was not observed. CONCLUSIONS: Tractography of the conus medullaris in a very young pediatric population (0-8 years old) with a spinal lipoma is possible, reproductive, and allows visualization of the spinal cord within the dysraphism. Analysis of the FA shows that the presence of a lipoma seems to have an effect on the myelination of the conus medullaris. It is during the probable myelination phase that the majority of symptoms appear. Is the myelination per se the cause?
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Lipoma/diagnóstico por imagen , Neoplasias de la Médula Espinal/diagnóstico por imagen , Médula Espinal/diagnóstico por imagen , Envejecimiento/patología , Niño , Preescolar , Imagen de Difusión Tensora , Femenino , Humanos , Procesamiento de Imagen Asistido por Computador , Lactante , Lipoma/cirugía , Masculino , Vaina de Mielina , Fibras Nerviosas/patología , Procedimientos Neuroquirúrgicos/métodos , Reproducibilidad de los Resultados , Médula Espinal/cirugía , Neoplasias de la Médula Espinal/cirugíaRESUMEN
The article which was recently published contained error. The figures and figure captions were interchanged during the publication process of the paper.
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By describing 10 new patients recruited in centres for Human Genetics, we further delineate the clinical spectrum of a Crouzon-like craniosynostosis disorder, officially termed craniosynostosis and dental anomalies (MIM614188). Singularly, it is inherited according to an autosomal recessive mode of inheritance. We identified six missense mutations in IL11RA, a gene encoding the alpha subunit of interleukin 11 receptor, 4 of them being novel, including 2 in the Ig-like C2-type domain. A subset of patients had an associated connective tissue disorder with joint hypermobility and intervertebral discs fragility. A smaller number of teeth anomalies than that previously reported in the two large series of patients evaluated in dental institutes points toward an ascertainment bias.
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Disostosis Craneofacial/genética , Genes Recesivos , Subunidad alfa del Receptor de Interleucina-11/genética , Adolescente , Adulto , Niño , Preescolar , Disostosis Craneofacial/diagnóstico por imagen , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Mutación MissenseRESUMEN
Wild dabbling ducks are the main reservoir for avian influenza (AI) viruses and pose an ongoing threat to commercial poultry flocks. Combining the (i) size of that population, (ii) their flight distances and (iii) their AI prevalence, the density of AI-infected dabbling ducks (DID) was calculated as a risk factor for the introduction of AI viruses into poultry holdings of Emilia-Romagna region, Northern Italy. Data on 747 poultry holdings and on 39 AI primary outbreaks notified in Emilia-Romagna between 2000 and 2017 were used to validate that risk factor. A multivariable Bayesian logistic regression was performed to assess whether DID could be associated with the occurrence of AI primary outbreaks. DID value, being an outdoor flock, hobby poultry trading, species reared, length of cycle and flock size were used as explanatory variables. Being an outdoor poultry flock was significantly associated with a higher risk of AI outbreak occurrence. The probability of DID to be a risk factor for AI virus introduction was estimated to be 90%. A DID cut-off of 0.23 was identified to define high-risk areas for AI virus introduction. Using this value, the high-risk area covers 43% of the region. Seventy-four per cent of the primary AI outbreaks have occurred in that area, containing 39% of the regional poultry holdings. Poultry holdings located in areas with a high DID value should be included in a risk-based surveillance programme aimed at AI early detection.
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Animales Salvajes/virología , Brotes de Enfermedades/veterinaria , Patos/virología , Virus de la Influenza A/aislamiento & purificación , Gripe Aviar/transmisión , Enfermedades de las Aves de Corral/transmisión , Animales , Teorema de Bayes , Pollos , Gripe Aviar/virología , Italia , Modelos Estadísticos , Enfermedades de las Aves de Corral/virología , Prevalencia , Factores de RiesgoRESUMEN
The complexity of treatment of faciocraniosynostosis justifies the treatment in a reference center for rare diseases. The growth disturbances in the skull and face being variable according to the type of mutation in the FGFr (Crouzon, Pfeiffer, Apert), the strategy is adapted to the phenotype according to the following principles: posterior expansion with or without distraction around 6 months to limit the descent of the cerebellum tonsils and to prevent the turricephalic development; fronto-facial monobloc advancement with internal distraction around the age of 18 months in case of severe exorbitism or breathing impairment. The dissociated strategy (fronto-orbital advancement first, followed by facial osteotomy of Le Fort 3 type). The growing evolution dictates the sequence of subsequent surgeries according to the monitoring of intracranial pressure by fundus examination and of the respiration by polysomnography. Le Fort 3 and transversal maxillary distraction may be repeated if necessary. Orthognathic surgery is almost always compulsory after the age of 14, before the aesthetic refinements which can be undertaken ultimately (rhinoplasty, genioplasty, canthopexies, fat grafting ).
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Disostosis Craneofacial/cirugía , Craneosinostosis/cirugía , Procedimientos de Cirugía Plástica/métodos , Niño , Disostosis Craneofacial/diagnóstico por imagen , Craneosinostosis/diagnóstico por imagen , Craneotomía , Humanos , Imagenología Tridimensional , Osteogénesis por Distracción , Cirugía Asistida por ComputadorRESUMEN
PURPOSE: Epidural blood patch (EBP) represents one of the best nonsurgical treatment for intracranial hypotension syndrome. Orthostatic headache caused by reduced intracranial cerebrospinal fluid (CSF) pressure, like in "spontaneous" intracranial hypotension or as consequence of lumbar puncture or anesthesiological procedure, can be managed with the injection of autologous blood on the epidural space with a successful rate of 89%, increased to 97% after a second application. MATERIALS AND METHODS: This 9-year-old girl was admitted to our department because of a suboccipital pseudomeningocele. She was previously operated on for a Chiari type I malformation by suboccipital craniectomy, C1 laminectomy and duraplasty. At the admission, she complained for nucal pain. Brain MRI showed a large suboccipital fluid collection that persisted even after the revision of the duraplasty and the placement of an external lumbar drainage. The child underwent a first injection of a mixture of blood (10 ml) and fibrin glue (10 ml) within the subcutaneous space after needle-aspiration of the collection. The same treatment was repeated 3 weeks later. RESULTS: The procedures were well tolerated and no local or systemic complications occurred. The fluid collection was significantly reduced after the first injection and it was completely effaced following the second one. Such a result remained stable after 3 months, as demonstrated by MRI. No recurrence of the pseudomeningocele was detected at the last clinical control (8-month follow-up). CONCLUSION: Subcutaneous blood patch could represent a safe and effective option for the treatment of CSF fistula, especially in case of failure of the traditional management.
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Parche de Sangre Epidural , Craniectomía Descompresiva/efectos adversos , Fístula/cirugía , Enfermedad Iatrogénica , Malformación de Arnold-Chiari/cirugía , Niño , Descompresión Quirúrgica/efectos adversos , Femenino , Fístula/etiología , HumanosRESUMEN
There is no consensus on which drugs/techniques/strategies can affect mortality in the perioperative period of cardiac surgery. With the aim of identifying these measures, and suggesting measures for prioritized future investigation we performed the first International Consensus Conference on this topic. The consensus was a continuous international internet-based process with a final meeting on 28 June 2010 in Milan at the Vita-Salute University. Participants included 340 cardiac anesthesiologists, cardiac surgeons, and cardiologists from 65 countries all over the world. A comprehensive literature review was performed to identify topics that subsequently generated position statements for discussion, voting, and ranking. Of the 17 major topics with a documented mortality effect, seven were subsequently excluded after further evaluation due to concerns about clinical applicability and/or study methodology. The following topics are documented as reducing mortality: administration of insulin, levosimendan, volatile anesthetics, statins, chronic ß-blockade, early aspirin therapy, the use of pre-operative intra-aortic balloon counterpulsation, and referral to high-volume centers. The following are documented as increasing mortality: administration of aprotinin and aged red blood cell transfusion. These interventions were classified according to the level of evidence and effect on mortality and a position statement was generated. This International Consensus Conference has identified the non-surgical interventions that merit urgent study to achieve further reductions in mortality after cardiac surgery: insulin, intra-aortic balloon counterpulsation, levosimendan, volatile anesthetics, statins, chronic ß-blockade, early aspirin therapy, and referral to high-volume centers. The use of aprotinin and aged red blood cells may result in increased mortality.
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Procedimientos Quirúrgicos Cardíacos/mortalidad , Cuidados Críticos , Anestesia , HumanosRESUMEN
BACKGROUND: There is no consensus on which drugs/techniques/strategies can affect mortality in the perioperative period of cardiac surgery. With the aim of identifying these measures, and suggesting measures for prioritized future investigation we performed the first international consensus conference on this topic. METHODS: The consensus was a continuous international internet-based process with a final meeting on June 28th 2010 in Milan at the Vita-Salute University. Participants included 340 cardiac anesthesiologists, cardiac surgeons and cardiologists from 65 countries all over the world. A comprehensive literature review was performed to identify topics that subsequently generated position statements for discussion, voting and ranking. RESULTS: Of the 17 major topics with a documented mortality effect, seven were subsequently excluded after further evaluation due to concerns about clinical applicability and/or study methodology. The following topics are documented as reducing mortality: administration of insulin, levosimendan, volatile anesthetics, statins, chronic beta-blockade, early aspirin therapy, the use of preoperative intra-aortic balloon counterpulsation and referral to high-volume centers. The following are documented as increasing mortality: administration of aprotinin and aged red blood cell transfusion. These interventions were classified according to the level of evidence and effect on mortality and a position statement was generated. CONCLUSION: This international consensus conference has identified the non-surgical interventions that merit urgent study to achieve further reductions in mortality after cardiac surgery: insulin, intra-aortic balloon counterpulsation, levosimendan, volatile anesthetics, statins, chronic beta-blockade, early aspirin therapy, and referral to high-volume centers. The use of aprotinin and aged red blood cells may result in increased mortality.
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The authors report on the imaging capabilities of a mammographic system demonstrator based on GaAs pixel detectors operating in single photon counting (SPC) mode. The system imaging performances have been assessed by means of the transfer functions: The modulation transfer function (MTF), the normalized noise power spectrum, and the detective quantum efficiency (DQE) have been measured following the guidelines of the IEC 62220-1-2 protocol. The transfer function analysis has shown the high spatial resolution capabilities of the GaAs detectors. The MTF calculated at the Nyquist frequency (2.94 cycles/mm) is indeed 60%. The DQE, measured with a standard mammographic beam setup (Mo/Mo, 28 kVp, with 4 mm Al added filter) and calculated at zero frequency, is 46%. Aiming to further improve the system's image quality, the authors investigate the DQE limiting factors and show that they are mainly related to system engineering. For example, the authors show that optimization of the image equalization procedure increases the DQE(0) up to 74%, which is better than the DQE(0) of most clinical mammographic systems. The authors show how the high detection efficiency of GaAs detectors and the noise discrimination associated with the SPC technology allow optimizing the image quality in mammography. In conclusion, the authors propose technological solutions to exploit to the utmost the potentiality of GaAs detectors coupled to SPC electronics.