Interconsulta en maltrato infantojuvenil: Característica de la población con sospecha de maltrato infantojuvenil del Hospital de Pediatría Juan P. Garrahan / Consultation on child abuse: Characteristics of a population with a high level of suspicion of child abuse at Hospital de Pedatría Juan P. Garrahan

El maltrato infantojuvenil constituye un problema de salud pública, presenta consecuencias negativas en el adecuado desarrollo de los niños y adolescentes. Es consecuencia de una multiplicidad de factores individuales, sociales, psicológicos y culturales que puede incluso desembocar en la muerte del niño o adolescente. El objetivo fue realizar una descripción sociodemográfica e identificar posibles variables específicas de maltrato en la población que realizó alguna consulta al hospital y que fue derivada por el médico tratante al Consultorio Multidisciplinario de Protección Infantojuvenil (CMPIJ). Se trató de un estudio descriptivo, prospectivo de corte transversal, realizado entre julio de 2016 y mayo de 2017 en el Hospital de Pediatría Juan P. Garrahan. El criterio de inclusión fue la población que realizó alguna consulta al hospital, ya sea por sospecha de maltrato o por otros motivos y fue derivada por el médico tratante al CMPIJ. Se evaluaron 96 casos. el 67,7% de las sospechas fueron de abuso sexual infantil (ASI), el 22,9% de maltrato físico y el 9,4% de trastorno facticio aplicado a otro. En los casos de alta sospecha de ASI, el 75% de los pacientes eran de sexo femenino; en el 50% de los casos, el supuesto agresor era conviviente y el 50% de las madres refirió antecedentes de malos tratos en la infancia. En los casos de alta sospecha de maltrato físico, el 53% de los pacientes era de sexo masculino, en el 83,3% de los casos el supuesto agresor era conviviente y el 76,5% de las madres refirió antecedentes de malos tratos en infancia. Se concluye la importancia de contar con datos fidedignos en lo relativo al maltrato Infantojuvenil para permitir elaborar estrategias de prevención tanto a nivel asistencial como de Salud Pública (AU)

Child abuse is a public health problem with a negative impact on the adequate development of children and adolescents. It is a consequence of multiple individual, social, psychological, and cultural factors that may even result in the death of the child or adolescent. The aim of this study was to provide a sociodemographic description and to identify possible specific variables associated with abuse in the population that visited the hospital and was referred to the Multidisciplinary Child Protection Clinic (MDCPC). A prospective, descriptive, cross-sectional study was conducted between July 2016 and May 2017 at Hospital de Pediatría Juan P. Garrahan. The inclusion criterion was the population that visited the hospital, either for suspicion of abuse or for other reasons, who were referred to the MDCPC by the treating physician. Overall, 96 cases were evaluated; 67.7% of the suspicions were of childhood sexual abuse (CSA), 22.9% for child battering, and 9.4% for Munchausen syndrome by proxy. Among the cases with a high level of suspicion of CSA, 75% were female; in 50% of the cases the supposed aggressor lived in the household, and 50% of the mothers reported a history of child abuse. In the cases of a high level of suspicion of child battering, 53% of the patients were male, in 83.3% of the cases the aggressor was living in the household, and 76.5% of the mothers reported a history child abuse. In conclusion, trustworthy data on child abuse are necessary to develop prevention strategies at both the care and public health levels (AU)

Prevalencia de trastornos del desarrollo en consultorios de mediano riesgo del Hospital Garrahan / Prevalence of developmental disorders in patients seen at the aoutpatient clinic of the Garrahan Hospital

Los trastornos del desarrollo son problemas relevantes y de gran impacto para la familia y la sociedad. Según Glascoe aproximadamente el 15-18 por ciento de los niños en Estados Unidos tienen alteración del desarrollo o de la conducta. Los datos en países en vías de desarrollo son muy escasos. Objetivos. Determinar la prevalencia de trastornos del desarollo (TD) en niños de 1 año a 5 años, 11 meses y 29 días que asisten al consultorio de Mediano Riesgo (MR) del hospital de Pediatría Juan P. Garrahan. diferenciar y caracterizar a los niños con trastorno del desarrollo evidente o sospechoso. Materiales y métodos: Estudio de corte transversal, prospectivo y descriptivo realizado entre 07/2007 y 03/2008. Se incluyeron pacientes de 1 año a 5 años 11 meses y 29 días que consultaron al sector de Mediano Riesgo con trastornos del desarrollo evidente o sospechoso por evaluación clínica asistemática o presencia de factores de riesgo. Los evidentes fueron derivados para evaluación especifica del desarrollo. A los sospechosos se les realizó un interrogatorio del desarrollo y se les administró la prueba de pesquisa PRUNAPE. Resultados: Sobre un total de 922 pacientes elegidos al azar, el 45.5 por ciento (N=420) fueron incluidos por presentar alteraciones evidentes o sospechosas de trastornos del desarrollo para la población elegida en ese período fue del 20 por ciento (N=186) para los evidentes y 17.5 por ciento (N=161) para los sospechosos. Del grupo de pacientes sospechosos (N=161), el 62.2 por ciento (N=100) no pasó la prueba de pesquisa o presentó algún tipo de trastorno de conducta o disfluencia, que requirió intervención terapéutica orientada por el pediatra. Cuando la sospecha fue de los padres (N=20): un 75 por ciento falló en la PRUNAPE (N=15), un niño presentó trastorno de la conducta que requirió intervención terapéutica (N=1) y un niño disfluencia o trartamudez (N=1).

beta-Crustacyanin, the blue-purple carotenoprotein of lobster carapace: consideration of the bathochromic shift of the protein-bound astaxanthin.

The crystal structure of a beta-crustacyanin allows an analysis of the various proposals for the mechanism of the bathochromic shift from orange to purple-blue of astaxanthin in this lobster carotenoprotein. Structural and previous chemical and biophysical studies suggest that extension of conjugation by coplanarization of the beta-ionone rings with the polyene chain and polarization resulting from hydrogen bonding at the C(4) and C(4') keto groups may be mainly responsible for the bathochromic shift. Additional contributions may arise from medium effects and possibly from bowing of the polyene chain on binding. Previous biophysical data revealing a somewhat symmetrical polarization of astaxanthin in crustacyanin are thereby also accounted for. A puzzling feature remains unexplained: the bathochromic shifts, larger than that of astaxanthin, shown by some cyclopentenedione carotenoids in reconstituted carotenoproteins. This mini review enlarges on the original analysis and conclusions of Cianci et al. [(2002), Proc. Natl Acad. Sci. USA, 99, 9795-9800].

Dermatitis atópica: nuestra experiencia en talleres para pedres / Atopic dermatitis: our experience with parental´s workshop education

La Dermatitis Atópica (DA) es una enfermedad inflamatoria crónica de la piel, que se presenta más frecuentemente durante la infancia. El prurito intenso y la hiprerreactividad cutánea son los signos cardinales de esta enfermedad. Una complicación muy frecuentemente observada, aunque no siempre tenida en cuenta, es la afección de la vida familiar y de la relación del niño con el medio que lo rodea. Estos pacientes suelen presentar trastornos del sueño, de su conducta, su rendimiento escolar y su relación con los demás, secundarios a la alteración de su descanso, su alimentación y el sentimiento negativo que despiertan sus lesiones cutáneas. Es en la consulta de esta patología frecuente, donde hemos aprendido la importancia del manejo adecuado de las situaciones descriptas para obtener una buena evolución de la enfermedad; asimismo creemos que la salud se logra a través de la prevención y la educación. Con el objetivo de brindarles información correcta sobre esta afección a las familias afectadas, y de conocer cuales son los inconvenientes que esta enfermedad les presenta día a día, hemos organizado Taller para padres de niños con diagnóstico de Dermatitis Atópica. En este trabajo presentamos los resultados obtenidos en los 6 primeros talleres realizados.

The C1 subunit of alpha-crustacyanin: the de novo phasing of the crystal structure of a 40 kDa homodimeric protein using the anomalous scattering from S atoms combined with direct methods.

The previously unknown crystal structure of the C(1) subunit of the carotenoid-binding protein alpha-crustacyanin has been determined using the anomalous scattering available at 1.77 A wavelength to determine the partial structure of the S atoms intrinsic to the native protein. The resulting 'heavy-atom' phases, in conjunction with near-atomic resolution (d(min) = 1.15 A) data, were then used to initiate successful structure determination using a direct-methods approach. This is, to the authors' knowledge, the first time such a small anomalous signal ( approximately 1%) has been used to aid the determination of a macromolecular structure. As well as the structure itself, the methods used during data collection and those used in the elucidation of the sulfur 'heavy-atom' partial structure are described here. As predicted, the C(1) subunit adopts a tertiary structure typical of the lipocalin superfamily: an eight-stranded antiparallel beta-barrel with a repeated +1 topology. The beta-barrel has a calyx shape with the two molecules in the asymmetric unit interacting in such a way that the open ends of each calyx face each other, although they do not form a single elongated pocket. A comparison of this structure with those of other members of the lipocalin superfamily has allowed speculation as to the nature of carotenoid binding by the protein.

Structure of lobster apocrustacyanin A1 using softer X-rays.

The molecular basis of the camouflage colouration of marine crustacea is often provided by carotenoproteins. The blue colour of the lobster carapace, for example, is intricately associated with a multimacromolecular 16-mer complex of protein subunits each with a bound astaxanthin molecule. The protein subunits of crustacyanin fall into two distinct subfamilies, CRTC and CRTA. Here, the crystal structure solution of the A(1) protein of the CRTC subfamily is reported. The problematic nature of the structure solution of the CRTC proteins (both C(1) and A(1)) warranted consideration and the development of new approaches. Three putative disulfides per protein subunit were likely to exist based on molecular-homology modelling against known lipocalin protein structures. With two such subunits per crystallographic asymmetric unit, this direct approach was still difficult as it involved detecting a weak signal from these sulfurs and suggested the use of softer X-rays, combined with high data multiplicity, as reported previously [Chayen et al. (2000), Acta Cryst. D56, 1064-1066]. This paper now describes the structure solution of CRTC in the form of the A(1) dimer based on use of softer X-rays (2 A wavelength). The structure solution involved a xenon derivative with an optimized xenon L(I) edge f" signal and a native data set. The hand of the xenon SIROAS phases was determined by using the sulfur anomalous signal from a high-multiplicity native data set also recorded at 2 A wavelength. For refinement, a high-resolution data set was measured at short wavelength. All four data sets were collected at 100 K. The refined structure to 1.4 A resolution based on 60 276 reflections has an R factor of 17.7% and an R(free) of 22.9% (3137 reflections). The structure is that of a typical lipocalin, being closely related to insecticyanin, to bilin-binding protein and to retinol-binding protein. This A(1) monomer or dimer can now be used as a search motif in the structural studies of the oligomeric forms alpha- and beta-crustacyanins, which contain bound astaxanthin molecules.

Apocrustacyanin A1 from the lobster carotenoprotein alpha-crustacyanin: crystallization and initial X-ray analysis involving softer X-rays.

The A1 subunit of the carotenoprotein alpha-crustacyanin, isolated from lobster carapace, has been crystallized using the vapour-diffusion method. The crystals, grown in solutions of ammonium sulfate containing methylpentanediol (MPD), diffracted to 2. 0 A. The crystals are stable to radiation. The space group of the crystals is P2(1)2(1)2(1). The unit-cell parameters are a = 41.9, b = 80.7, c = 110.8 A. 'Standard structure determination' has been unsuccessful within this crustacyanin family. Instead, an approach based on the S atoms is being undertaken involving softer X-rays at the SRS, Daresbury.

Hallazgos clínicos y valor de los análisis en el examen prequirúrgico / Clinical findings and value of laboratory tests in pre-surgical examination

En un trabajo prospectivo se evaluaron los hallazgos clínicos y la utilidad de los análisis previosa la cirugía realizados como medio de prevenir morbimortalidad perioperatoria. La muestra incluyó 192 pacientes vistos durante 45 días de consultorio. Se detectaron 31 niños con infección respiratoria, 10 con anemia, 7 con otras intercurrencias infecciosas, uno con eosinofilia grave (toxocariasis), uno con hipertensión arterial no controlada y otros 7 con diversas patologías. En 187 a los que se efectuaron exámenes de laboratorio, se observaron las siguientes alteraciones: anemia en 8/31 pacientes menores de 2 años y en 2 adolescentes mujeres. La presencia de anemia ferropénica en los grupos etarios susceptibles de presentarla fue significativa (p<0,005). En 3 niños se hallaron alteraciones de los glóbulos blancos, no sospechadas previamente. Ningún paciente presentó trastornos de las plaquetas o del coagulograma en forma persistente. A 189 niños se les realizó electrocardiograma. La sensibilidad del método fue de 44,4 por ciento y la especificidad del 98,3 por ciento para el diagnóstico de cardiopatía. La causa más frecuente de suspensión de la cirugía fue la infección respiratoria. Entre los menores de 2 años, la segunda causa fue la anemia. Se operaron 126 niños que tuvieron evolución favorable. Concluímos que la anamnesis cuidadosa y el examen físico minucioso son las evaluaciones más importantes. El dosaje de hemoglobina es el estudio de laboratorio más útil. Los demás estudios debieran realizarse de acuerdo con la historia del paciente y el examen clínico, ya que raramente se detectaron anormalidades en pacientes asintomáticos.

Purification, crystallization and initial X-ray analysis of the C1 subunit of the astaxanthin protein, V600, of the chondrophore Velella velella.

The subunit C1 of the carotenoid-binding protein, V600, of the chondrophore Velella velella has been purified and crystallized. The crystals, which were grown by the vapour-diffusion method from ammonium sulfate as the major precipitant, diffract beyond 3 A and show little radiation damage over long periods (greater than 100 h) on a Cu Kalpha rotating-anode X-ray source. The space group of the crystals is P212121 with cell dimensions a = 42.0, b = 80.9, c = 110. 6 A.

Partial improvement of crystal quality for microgravity-grown apocrustacyanin C1.

The protein apocrustacyanin C(1) has been crystallized by vapour diffusion in both microgravity (the NASA space shuttle USML-2 mission) and on the ground. Rocking width measurements were made on the crystals at the ESRF Swiss-Norwegian beamline using a high-resolution psi-circle diffractometer from the University of Karlsruhe. Crystal perfection was then evaluated, from comparison of the reflection rocking curves from a total of five crystals (three grown in microgravity and two earth controls), and by plotting mosaicity versus reflection signal/noise. Comparison was then made with previous measurements of almost 'perfect' lysozyme crystals grown aboard IML-2 and Spacehab-I and reported by Snell et al. [Snell, Weisgerber, Helliwell, Weckert, Hölzer & Schroer (1995). Acta Cryst. D51, 1099-1102]. Overall, the best diffraction-quality apocrustacyanin C(1) crystal was microgravity grown, but one earth-grown crystal was as good as one of the other microgravity-grown crystals. The remaining two crystals (one from microgravity and one from earth) were poorer than the other three and of fairly equal quality. Crystal movement during growth in microgravity, resulting from the use of vapour-diffusion geometry, may be the cause of not realising the 'theoretical' limit of perfect protein crystal quality.

Partial improvement of crystal quality for microgravity-grown apocrustacyanin C1.

The protein apocrustacyanin C1 has been crystallized by vapour diffusion in both microgravity (the NASA space shuttle USML-2 mission) and on the ground. Rocking width measurements were made on the crystals at the ESRF Swiss-Norwegian beamline using a high-resolution psi-circle diffractometer from the University of Karlsruhe. Crystal perfection was then evaluated, from comparison of the reflection rocking curves from a total of five crystals (three grown in microgravity and two earth controls), and by plotting mosaicity versus reflection signal/noise. Comparison was then made with previous measurements of almost 'perfect' lysozyme crystals grown aboard IML-2 and Spacehab-1 and reported by Snell et al. [Snell, Weisgerber, Helliwell, Weckert, Holzer & Schroer (1995). Acta Cryst. D51, 1099-1102]. Overall, the best diffraction-quality apocrustacyanin C1 crystal was microgravity grown, but one earth-grown crystal was as good as one of the other microgravity-grown crystals. The remaining two crystals (one from microgravity and one from earth) were poorer than the other three and of fairly equal quality. Crystal movement during growth in microgravity, resulting from the use of vapour-diffusion geometry, may be the cause of not realising the 'theoretical' limit of perfect protein crystal quality.

Crystallization and initial X-ray analysis of beta-crustacyanin, the dimer of apoproteins A2 and C1, each with a bound astaxanthin molecule.

Crystals of beta-crustacyanin, a carotenoid-binding protein from lobster carapace, have been grown under oil from solutions containing sodium potassium phosphate as precipitant. They grow slowly over a period of months to reach maximal dimensions of 0.5 x 0.1 x 0.1 mm, and belong to space group P622 with cell dimensions: a = b = 124.39, c = 188.86 A and gamma = 120 degrees. The crystals diffract to beyond 3 A but are very radiation sensitive, limiting the resolution of usable data. The unit-cell volume suggests that there are two beta-crustacyanin molecules per asymmetric unit.

Crystallization of apocrustacyanin on the International Microgravity Laboratory (IML-2) mission.

Rod-shaped crystals of apocrustacyanin C1 have been grown under microgravity on the International Microgravity Laboratory (IML-2) NASA space shuttle mission using the vapour-diffusion set-up of the Advanced Protein Crystallization Facility (APCF). The crystals obtained under microgravity are compared with crystals grown simultaneously in ground control experiments in identical APCF reactors, and with those obtained in the laboratory. The degree of reproducibility of the results in microgravity was also tested. Statistically, the microgravity-grown crystals are larger and of better X-ray diffraction quality than those grown in the ground controls but inferior to the best crystals grown in sitting drops, in the laboratory. Diffracting crystals, the best to 2.3 A, were produced in seven out of the eight reactors in microgravity, whereas the eight ground control reactors yielded only one poorly formed crystal suitable for diffraction studies, which also diffracted to 2.3 A. The crystals belong to the space group P2(1)2(1)2(1) with two subunits per asymmetric unit.

Crystallisation of alpha-crustacyanin, the lobster carapace astaxanthin-protein: results from EURECA.

Crystallisation of alpha-crustacyanin, the lobster carapace astaxanthin-protein was attempted under microgravity conditions in EURECA satellite using liquid-liquid diffusion with polyethyleneglycol (PEG) as precipitant; in a second reaction chamber phenol and dioxan were used as additives to prevent composite crystal growth. Crystals of alpha-crustacyanin grown under microgravity from PEG were larger than those grown terrestrially in the same apparatus under otherwise identical conditions. On retrieval, the crystals from PEG were shown to be composite and gave a powder diffraction pattern. The second reaction chamber showed leakage on retrieval and had also been subjected to rapid temperature variation during flight. Crystal fragments were nevertheless recovered but showed a powder diffraction pattern. It is concluded, certainly for liquid-liquid diffusion using PEG alone, that, for crustacyanin, although microgravity conditions resulted in an increase in dimensions of crystals, a measurable improvement in molecular ordering was not achieved.

Crystallization and initial X-ray analysis of the C2-subunit of crustacyanin.

Crystals of the C2-subunit of crustacyanin have been grown from solutions containing ammonium sulphate and 2-methyl-2,4-pentanediol as co-precipitants. The crystals belong to space group P2(1)2(1)2(1) (a = 42.0 A, b = 80.9 A, c = 110.8 A) with two subunits per asymmetric unit and diffract beyond 2.2 A resolution.

Complete sequence and model for the C1 subunit of the carotenoprotein, crustacyanin, and model for the dimer, beta-crustacyanin, formed from the C1 and A2 subunits with astaxanthin.

The complete sequence has been determined for the C1 subunit of crustacyanin, an astaxanthin-binding protein from the carapace of the lobster Homarus gammarus (L.). The polypeptide, 181 residues long, is similar (38% identity) to the other main subunit, A2 and to plasma retinol-binding protein. The tertiary structure of the C1 subunit has been modelled on that derived for the A2 subunit from the coordinates of retinol-binding protein. Residues lining the putative binding cavities and at the putative carotenoid binding sites of the two subunits are highly conserved. The carotenoid environments are characterized by a preponderance of aromatic and polar residues and the absence of charged side-chains. A tentative model for the dimer, beta-crustacyanin, formed between the two subunits with their associated carotenoid ligands, is discussed. The model is based on the crystal structure of the dimer of bilin-binding protein, a member of the same superfamily. This structure has enabled us to examine mechanisms for the bathochromic spectral shift of the protein-bound carotenoid and to identify likely contact regions between dimers in octameric alpha-crustacyanin.

Complete sequence and model for the A2 subunit of the carotenoid pigment complex, crustacyanin.

The complete sequence has been determined for the A2 subunit of crustacyanin, an astaxanthin-binding protein from the carapace of the lobster Homarus gammarus. The polypeptide chain is 174 residues long and is similar to proteins of the retinol-binding protein superfamily. Some regions of the sequence are most similar to the retinol-binding protein, beta-lactoglobulin subgroup, while the disulphide bonding pattern is more akin to that seen in the porphyrin binding proteins insecticyanin and bilin-binding protein. It is beginning to appear as though this superfamily of proteins, characterized by a similar gross structural framework, may be further subdivided into interrelated subclasses. Model building based on the coordinates of the known structure of human plasma retinol-binding protein and on empirical prediction algorithms has allowed the putative identification of side-chains which line the binding cavity. This pocket is larger than in retinol binding protein and beta-lactoglobulin but does not allow the carotenoid to adopt a folded conformation. The amino acid composition of the pocket does not support a 'charge-shift'-type hypothesis to support the bathochromic shift phenomenon which takes place on interaction of the chromophore with the protein. Instead aromatic side-chains may play a prominent role.