Síndrome de pseudotumor cerebri en la población pediátrica: características clínicas, tratamiento y pronóstico / Pseudotumor cerebri in the paediatric population: clinical features, treatment and prognosis

Introducción El síndrome de pseudotumor cerebri (SPTC) en pacientes prepuberales presenta características que lo diferencian respecto a su presentación en la etapa pospuberal. Nuestro objetivo es describir las características de los pacientes diagnosticados de SPTC pediátrico en nuestro centro y compararlas en función de su estado puberal. Pacientes y métodos Se incluyeron a los pacientes diagnosticados de SPTC en un hospital de tercer nivel entre los años 2006 y 2019 con edades comprendidas entre uno y 18 años que cumplieran los criterios diagnósticos actualizados del SPTC. Se clasificaron en función de su estado puberal y peso corporal. Posteriormente, se analizaron los datos de las punciones lumbares, estudios de neuroimagen, valoraciones oftalmológicas, así como el régimen terapéutico recibido a lo largo de su seguimiento. Resultados Se recogieron 28 pacientes, 22 prepuberales y seis pospuberales, con edad media de 9,04 ± 2,86 años. El 83,3% de los pacientes pospuberales eran varones presentando sobrepeso/obesidad en el 66,7%. Eran varones el 27% de los pacientes prepuberales, de ellos asociaban sobrepeso el 31,8%. La sintomatología más frecuente fue cefalea (89,9%) y visión borrosa (42,9%). Todos los pacientes presentaron papiledema; un 21,4% de los casos presentaron parálisis del VI par. Se identificó un posible desencadenante en un 28,6%. El 19% presentaron recurrencia clínica, siendo todos ellos prepuberales. La resolución clínica completa se produjo en el 55,6% de los pacientes. Conclusión Pacientes con SPTC presentan menor prevalencia de obesidad en la etapa prepuberal, junto con un mayor porcentaje de etiologías secundarias y tasa de recurrencia que los pacientes pospuberales. (AU)

Introduction Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status. Patients and methods We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up. Results We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients. Conclusion Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients. (AU)

Pseudotumor cerebri in the paediatric population: clinical features, treatment and prognosis.

INTRODUCTION: Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status. PATIENTS AND METHODS: We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up. RESULTS: We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients. CONCLUSION: Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.

Pseudotumor cerebri in the paediatric population: clinical features, treatment and prognosis. / Síndrome de pseudotumor cerebri en la población pediátrica: características clínicas, tratamiento y pronóstico.

INTRODUCTION: Pseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status. PATIENTS AND METHODS: We included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up. RESULTS: We included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients. CONCLUSION: Prepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.

Lysosomal membrane permeabilization and autophagy blockade contribute to photoreceptor cell death in a mouse model of retinitis pigmentosa.

Retinitis pigmentosa is a group of hereditary retinal dystrophies that normally result in photoreceptor cell death and vision loss both in animal models and in affected patients. The rd10 mouse, which carries a missense mutation in the Pde6b gene, has been used to characterize the underlying pathophysiology and develop therapies for this devastating and incurable disease. Here we show that increased photoreceptor cell death in the rd10 mouse retina is associated with calcium overload and calpain activation, both of which are observed before the appearance of signs of cell degeneration. These changes are accompanied by an increase in the activity of the lysosomal protease cathepsin B in the cytoplasm of photoreceptor cells, and a reduced colocalization of cathepsin B with lysosomal markers, suggesting that lysosomal membrane permeabilization occurs before the peak of cell death. Moreover, expression of the autophagosomal marker LC3-II (lipidated form of LC3) is reduced and autophagy flux is blocked in rd10 retinas before the onset of photoreceptor cell death. Interestingly, we found that cell death is increased by the induction of autophagy with rapamycin and inhibited by calpain and cathepsin inhibitors, both ex vivo and in vivo. Taken together, these data suggest that calpain-mediated lysosomal membrane permeabilization underlies the lysosomal dysfunction and downregulation of autophagy associated with photoreceptor cell death.

spaP gene of Streptococcus mutans in dental plaque and its relationship with early childhood caries.

AIM: Streptococcus mutans and Streptococcus sobrinus are the main pathogens associated with the development of dental caries in humans. Recently, the real-time polymerase chain reaction (qPCR-TR) has been used for fast and exact quantification of these bacteria species. This molecular biology method has made the detection of these bacteria in saliva and dental plaque possible; additionally, it aids the development of illness risk prediction. The purpose of this prospective, analytic, transversal, observational and unicenter study was to quantify the spaP gene of the Streptococcus mutans and its correlation with caries in a group of children using isolated DNA from plaque samples processed through qPCR-TR, using specific oligonucleotides for this gene detection. MATERIALS AND METHODS: The cariogenic potential of Streptococcus mutans in the dental plaque was analysed in a group of patients aged 12 to 46 months. A descriptive statistical analysis was performed. The Spearman's correlation coefficient was used to establish the correlation between caries (dmft) index (decayed/missing/filled primary teeth), spaP gene and age group. The Wilcoxon test was used to compare MSB cultivation technique and qPCR-TR. RESULTS: In the molecular trials, a close association between caries prevalence in childhood and the presence and high proportion of the spaP gene of S. mutans was found. The average caries prevalence was 3.71, and it increased as age range increased. The highest caries prevalence was observed in female patients and in the oldest age range studied (40 46 months) which contrasts with the 12-18 months age that had a caries (dmft) index of zero. The amplification using as initiator the gene spaP of the nucleic acids extracted from the S. mutans resulted positive in 91.3% of the cases. Every child with caries was positive for the spaP and only 8.75% were negative, this group included children without caries. CONCLUSION: In conclusion, there was a correlation with infant caries prevalence and S. mutans.

DNA-PK promotes the survival of young neurons in the embryonic mouse retina.

Programmed cell death is a crucial process in neural development that affects mature neurons and glial cells, as well as proliferating precursors and recently born neurons at earlier stages. However, the regulation of the early phase of neural cell death and its function remain relatively poorly understood. In mouse models defective in homologous recombination or nonhomologous end-joining (NHEJ), which are both DNA double-strand break (DSB) repair pathways, there is massive cell death during neural development, even leading to embryonic lethality. These observations suggest that natural DSBs occur frequently in the developing nervous system. In this study, we have found that several components of DSB repair pathways are activated in the developing mouse retina at stages that coincide with the onset of neurogenesis. In short-term organotypic retinal cultures, we confirmed that the repair pathways can be modulated pharmacologically. Indeed, inhibiting the DNA-dependent protein kinase (DNA-PK) catalytic subunit, which is involved in NHEJ, with NU7026 increased caspase-dependent cell death and selectively reduced the neuron population. This observation concurs with an increase in the number of apoptotic neurons found after NU7026 treatment, as also observed in the embryonic scid mouse retina, a mutant that lacks DNA-PK catalytic subunit activity. Therefore, our results implicate the generation of DSB and DNA-PK-mediated repair in neurogenesis in the developing retina.

Allergy to latex and repeated vascular access thrombosis in haemodialysis

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Structural and chemical characterization of Yb2O3-ZrO2 system by HAADF-STEM and HRTEM.

ZrO2:Yb3+ nanocrystalline phosphors with high concentrations of ytterbium ions were prepared using the sol-gel method. X-ray diffraction, high-angle annular-dark-field scanning transmission electron microscopy (HAADF-STEM), energy dispersive X-ray spectroscopy, and high-resolution transmission electron microscopy (HRTEM) were used to characterize the nanocrystalline phosphors annealed at 1000 degrees C. Unit-cell distortion and changes in the crystalline structure of the monoclinic zirconia to tetragonal zirconia, and subsequently cubic zirconia, were observed with increased Yb concentration. Yb ions were randomly distributed into the lattice of the crystalline structure. No segregation of Yb2O3 phase was observed. The substitution of Zr atoms by Yb atoms on different crystalline phases was confirmed by the experimental results and theoretical simulations of HRTEM and HAADF-STEM.

[Tooth loss in diabetic patients with and without chronic kidney disease and dialysis]. / Pérdida de dientes en pacientes diabéticos con y sin insuficiencia renal crónica y diálisis.

AIM: To compare tooth loss (TL) in ESRD (ESRD DM) and non-ESRD (DM) type 2 diabetic patients. METHODS: Teeth loss was quantified, and dentition classified as: Non-Compromised (NCD) with > or = 25 teeth, partially compromised (PCD) with 9 to 24, and compromised (CD) with 0 to 8 teeth. RESULTS: ESRD DM and DM: n> or = 103 and 130, mean age 57.9 and 58.5 yr (p> or =0.716), and at diabetes diagnosis 38.5 and 47.8 yr (p<0.001). Edentulous 23.5% and 13.8% (p> or =0.057), NCD 24.5% and 35.4% (p> or =0.074). TL was strongly associated mainly to periodontal disease (p<0.001). For ESRD DM, a low serum albumin (<3.5 g/dl) was more prevalent in peritoneal dialysis cases (p> or =0.0014), women (p> or =0.0100), people reporting unpleasant taste (UT) (p> or =0.0174), and those with a CD (p> or =0.0242). CONCLUSIONS: There was a clear trend for more severe TL in ESRD DM cases, but no statistical difference was found. The association between low serum albumin, UT and CD imply a need for treatment of these conditions as a part of nutritional intervention in ESRD DM cases.

Functional and structural modifications during retinal degeneration in the rd10 mouse.

Mouse models of retinal degeneration are useful tools to study therapeutic approaches for patients affected by hereditary retinal dystrophies. We have studied degeneration in the rd10 mice both by immunocytochemistry and TUNEL-labeling of retinal cells, and through electrophysiological recordings. The cell degeneration in the retina of rd10 mice produced appreciable morphological changes in rod and cone cells by P20. Retinal cell death is clearly observed in the central retina and it peaked at P25 when there were 800 TUNEL-positive cells per mm(2). In the central retina, only one row of photoreceptors remained in the outer nuclear layer by P40 and there was a remarkable deterioration of bipolar cell dendrites postsynaptic to photoreceptors. The axon terminals of bipolar cells also underwent atrophy and the inner retina was subject to further changes, including a reduction and disorganization of AII amacrine cell population. Glutamate sensitivity was tested in rod bipolar cells with the single cell patch-clamp technique in slice preparations, although at P60 no significant differences were observed with age-matched controls. Thus, we conclude that rod and cone degeneration in the rd10 mouse model is followed by deterioration of their postsynaptic cells and the cells in the inner retina. However, the functional preservation of receptors for photoreceptor transmission in bipolar cells may open new therapeutic possibilities.

The autophagic machinery is necessary for removal of cell corpses from the developing retinal neuroepithelium.

Autophagy is a homoeostatic process necessary for the clearance of damaged or superfluous proteins and organelles. The recycling of intracellular constituents also provides energy during periods of metabolic stress, thereby contributing to cell viability. In addition, disruption of autophagic machinery interferes with embryonic development in several species, although the underlying cellular processes affected remain unclear. Here, we investigate the role of autophagy during the early stages of chick retina development, when the retinal neuroepithelium proliferates and starts to generate the first neurons, the retinal ganglion cells. These two developmental processes are accompanied by programmed cell death. Upon treatment with the autophagic inhibitor 3-methyladenine, retinas accumulated numerous TdT-mediated dUTP nick-end labelling-positive cells that correlated with a lack of the 'eat-me' signal phosphatidylserine (PS). In consequence, neighbouring cells did not engulf apoptotic bodies and they persisted as individual cell corpses, a phenotype that was also observed after blockade of phagocytosis with phospho-L-Serine. Supplying the retinas with methylpyruvate, a cell-permeable substrate for ATP production, restored ATP levels and the presentation of PS at the cell surface. Hence, engulfment and lysosomal degradation of apoptotic bodies were also re-established. Together, these data point to a novel role for the autophagic machinery during the development of the central nervous system.

Vimentin isoform expression in the human retina characterized with the monoclonal antibody 3CB2.

The antigen recognized by the monoclonal antibody 3CB2 (3CB2-Ag and 3CB2 mAb) is expressed by radial glia and astrocytes in the developing and adult vertebrate central nervous system (CNS) of vertebrates as well as in neural stem cells. Here we identified the 3CB2-Ag as vimentin by proteomic analysis of human glial cell line U-87 extracts (derived from a malignant astrocytoma). Indeed, the 3CB2 mAb recognized three vimentin isoforms in glial cell lines. In the human retina, 3CB2-Ag was expressed in Müller cells, astrocytes, some blood vessels, and cells in the horizontal cell layer, as determined by immunoprecipitation and immunofluorescence. Three populations of astrocytes were distinguishable by double-labeling immunohistochemistry: vimentin+/GFAP+, vimentin-/GFAP+, and vimentin+/GFAP-. Hence, we conclude that 1) the 3CB2-Ag is vimentin; 2) vimentin isoforms are differentially expressed in normal and transformed astrocytes; 3) human retinal astrocytes display molecular heterogeneity; and 4) the 3CB2 mAb is a valuable tool to study vimentin expression and its function in the human retina.

Thermoluminescence properties of undoped and Dy3+ doped ZrO2 nanophosphor under beta-ray irradiation.

The thermoluminescence (TL) of undoped and Dy3+ doped ZrO2 nanocrystals under beta-ray irradiation is reported. The TL glow curves are the result of the overlapping of four TL peaks produced partly by the intrinsic defect of highly asymmetrical monoclinic structure and partly due to defects produced during the synthesis process. The introduction of dopant ions induces changes in the glow curve due to the enhancement of high temperature peaks intensity. The results show that both undoped and doped ZrO2 nanocrystalline phosphor present good TL efficiency as well as good dose response which qualify them as a potential beta-ray dosimeter.

A new blue, green and red upconversion emission nanophosphor: BaZrO3:Er,Yb.

Strong Blue, green, and red upconversion emission of Er3+ in nanocrystalline BaZrO3:(Yb3+Er3+) is observed. Powder samples were obtained by a facile hydrothermal process at 100 degrees C. The as synthesized nanocrystallites preserve a stable cubic perovskite phase under subsequent annealing treatment up to 1000 degrees C. No other phase or segregation of other compounds was detected. Crystallites sizes were around 115 nm and well faceted. Under IR excitation in the range between 900 and 1050 nm the Er3+ blue emission was almost not present in single Er3+ doped BaZrO3, whereas it became easily observable when Yb3+ was added as codopant. Besides, both green and red upconversion emission or upconverted signal of Er3+ are enhanced by around three orders of magnitude in comparison with the single Er3+ doped BaZrO3. The strong blue emission presents dependence on both excitation power and excitation wavelength. This is the first time that upconversion emission is observed in BaZrO3. A possible mechanism for the upconversion process that leads to the observed blue, green and red emissions under NIR excitation is suggested based on the experimental results.

Structural and spectroscopic characterization of ZrO2:Eu3+ nanoparticles.

ZrO2:Eu3+ nanocrystals were prepared by the sol-gel technique. The structural and luminescence properties of europium doped zirconia with 0.5 to 2 mol% were studied by Mössbauer spectroscopy, Raman spectroscopy, X-ray diffraction (XRD), High Resolution Transmission Electron Microscopy (HRTEM) and photoluminescence (PL) under UV excitation. Structural characterization shows a crystallite size between 16 to 55 nm and monoclinic and tetragonal zirconia phases as the main crystalline structure. XRD patterns shown that the content of the active ions stabilizes the tetragonal structure of ZrO2 at 1000 degrees C, being 100% for 2 mol% Eu2O3 doped sample. Such results are in agreement with HRTEM and Raman spectroscopy. The Mössbauer spectra of the ZrO2:Eu3+ samples show a single peak near zero velocity which is attributed to Eu+3. Luminescence characterization shows the typical emission band centered at 595 and 611 nm. Change in the structure of such band was observed and explained in terms of crystalline phase change. The dependence between the fluorescence emission and the crystalline structure is discussed.

[Frontal pyomucocele]. / Piomucole frontal.

Mucoceles are strictly defined as chronic, expanding, mucosa-lined lesions of the frontal sinus and containing insipissated secretion. We present a 48-years-old female who complains right periorbital and frontal swelling of approximately 1,5 years duration. Radiological and exploratory findings confirm the diagnosis of infected mucocele (pyomucocele). Under general anesthesia an endoscopic sinus surgery was performed. One year later, the patient is alive and well with no evidence of primary disease.

Piomucocele frontal / Frontal pyomucocele

Se define como mucocele a una lesión crónica, expansiva circunscrita por mucosa que asienta en el seno frontal, la cual está llena de secrección mucosa. Presentamos el caso de una mujer de 48 años de edad que consultó por tumefacción frontal y periorbitaria derechas de 1,5 años de evolución. Los hallazgos encontrados en la exploración y en las pruebas de imagen confirmaron el diagnóstico de mucocele infectado (piomucocele). Se realizó su extirpación endoscópica bajo anestesia general. Un año más tarde se encuentra bien sin evidencia de la enfermedad por la que se la trató

Mucoceles are strictly defined as chronic, expanding, mucosa-lined lesions of the frontal sinus and containing insipissated secretion. We present a 48-years-old female who complains right periorbital and frontal swelling of approximately 1,5 years duration. Radiological and exploratory findings confirm the diagnosis of infected mucocele (pyomucocele). Under general anesthesia an endoscopic sinus surgery was performed. One year later, the patient is alive and well with no evidence of primary disease

Proinsulin in development: New roles for an ancient prohormone.

In postnatal organisms, insulin is well known as an essential anabolic hormone responsible for maintaining glucose homeostasis. Its biosynthesis by the pancreatic beta cell has been considered a model of tissue-specific gene expression. However, proinsulin mRNA and protein have been found in embryonic stages before the formation of the pancreatic primordium, and later, in extrapancreatic tissues including the nervous system. Phylogenetic studies have also confirmed that production of insulin-like peptides antecedes the morphogenesis of a pancreas, and that these peptides contribute to normal development. In recent years, other roles for insulin distinct from its metabolic function have emerged also in vertebrates. During embryonic development, insulin acts as a survival factor and is involved in early morphogenesis. These findings are consistent with the observation that, at these stages, the proinsulin gene product remains as the precursor form, proinsulin. Independent of its low metabolic activity, proinsulin stimulates proliferation in developing neuroretina, as well as cell survival and cardiogenesis in early embryos. Insulin/proinsulin levels are finely regulated during development, since an excess of the protein interferes with correct morphogenesis and is deleterious for the embryo. This fine-tuned regulation is achieved by the expression of alternative embryonic proinsulin transcripts that have diminished translational activity.

Strong visible cooperative up-conversion emission in ZrO2:Yb3+ nanocrystals.

Blue, green, and red emission was observed under infrared excitation in ZrO2:Yb3+ nanocrystals prepared by the sol-gel process. The structural characterization was performed by using XRD and HRTEM, suggesting that the crystalline phase of the nanoparticles is controlled by the active ion concentration being mainly tetragonal for 2 mol% of dopant and mainly monoclinic for 0.5 mol%. The blue emission was explained in terms of the cooperative deexcitation of an Yb-Yb pair, while the green and red bands were associated with the up-conversion of traces of Er ion. The number of photons involved in the luminescence process is analyzed in order to confirm that cooperative emission is produced by the interaction of an Yb pair and that the green and red emission are the results of energy transfer between Yb-Er ions. The high efficiency of all bands is explained in terms of the high surface area of the nanoparticles.