Protocol for the treatment of cystoid macular edema secondary to retinitis pigmentosa and other inherited retinal dystrophies.

Inherited retinal dystrophies (IRD) are the leading cause of legal blindness in the working population. Cystic macular edema (CME) is one of the treatable causes of visual loss, affecting up to 50% of the patients. A bibliographic review has been carried out combining "inherited retinal dystrophy", "retinitis pigmentosa", "macular oedema" and a diagnostic-therapeutic protocol according to the levels of evidence and recommendations of the "US Agency for Healthcare Research and Quality". This protocol has been discussed in the monthly meetings of the XAREA DHR group with the participation of more than 25 ophthalmologists, creating a consensus document. The etiology of CME is multifactorial: dysfunction of the blood-retinal barrier, retinal pigment epithelium, and Müller cells, inflammation, and vitreous traction. OCT is the test of choice for the diagnosis and follow-up of CME associated with IRD. The drugs with the highest degree of scientific evidence are carbonic anhydrase inhibitors (IAC). Intravitreal corticosteroids, anti-VEGF, and vitrectomy with peeling of the internal limiting membrane do not have sufficient evidence. A treatment scheme is proposed for the CME in IRD in adults, another for pediatric patients and another for IRD and cataract surgery. Oral and topical IACs are effective in the treatment of CME secondary to IRD. Treatment with corticosteroids, anti-VEGF, and vitrectomy are second-line options. Randomized clinical trials are required to establish the therapeutic scale in these patients.

[Translated article] Imaging diagnosis of vertebral metastasis.

The spine is the third most frequent location for metastatic disease, after the lung and liver. On the other hand, the most frequent bone tumours are metastases and the spine is the main location. A review of the different imaging techniques available, both radiological and nuclear medicine, and the morphological appearance of spinal metastases in each of them is performed. Magnetic resonance imaging is the best imaging modality for detection of spinal metastases. It is important to make the differential diagnosis between vertebral fracture of osteoporotic and pathological cause. Spinal cord compression is a serious complication of metastatic disease and its assessment by imaging through objective scales is decisive for estimating spinal stability and therefore establishing treatment. Lastly, percutaneous intervention techniques are briefly discussed.

Perifoveal exudative vascular anomalous complex (PEVAC).

We present three cases of patients aged 66, 80 and 23, who presented unilateral vision loss. Optical coherence tomography (OCT) in all of them showed macular oedema and a rounded lesion with hyperreflective wall, and fluorescein angiography (FAG) in two of them showed hyperfluorescent perifoveal aneurysmal dilations with exudation. None of the cases showed response to treatment after one year of follow-up, finally being diagnosed with Perifoveal Exudative Vascular Anomalous Complex (PEVAC).

Imaging diagnosis of vertebral metastasis. / Diagnóstico por imagen de las metástasis vertebrales.

The spine is the third most frequent location for metastatic disease, after the lung and liver. On the other hand, the most frequent bone tumors are metastases and the spine is the main location. A review of the different imaging techniques available, both radiological and nuclear medicine, and the morphological appearance of spinal metastases in each of them is performed. Magnetic resonance imaging is the best imaging modality for detection of spinal metastases. It is important to make the differential diagnosis between vertebral fracture of osteoporotic and pathological cause. Spinal cord compression is a serious complication of metastatic disease and its assessment by imaging through objective scales is decisive for estimating spinal stability and therefore establishing treatment. Lastly, percutaneous intervention techniques are briefly discussed.

Two case reports of acute zonal occult outer retinopathy (AZOOR): Importance of multimodal diagnosis.

A presentation is made of two cases of acute zonal occult outer retinopathy (AZOOR); one a young man of 19 years, and the other a 42-year-old woman. The young man complained of unilateral scotoma and photopsia. The woman presented with bilateral visual loss and photopsia. Multimodal imaging, including fundus photography, fluorescein angiography, fundus autofluorescence, spectral-domain optical coherence tomography, and visual field testing, supported the diagnosis of AZOOR. The differential diagnosis is complicated, since it has clinical features in common with other retinopathies. This means that it is essential to use modern imaging tests, especially those where the characteristic trizonal pattern is shown, such as in autofluorescence and OCT.

[Scientific Presentations at the meetings of the Spanish Pediatric Nephrology Association (AENP), 1988-2007]. / Comunicaciones científicas en los congresos de la Asociación Española de Nefrología Pediátrica (AENP), 1988-2007.

UNLABELLED: OBJECTIVES AND STUDY: To find out that characteristics of the scientific presentations given at the AENP's meetings in the past 20 years. METHODS: We reviewed in the scientific programs of the AENP's meetings of the past 20 years: number of presentations, number of participating institutions, institutions that provided the majority of the presentations, presentation format, number of studies involving experimental nephrology, topics most commonly presented. RESULTS: There have been 1,119 presentations in the past 20 years, 45/year between 88-92 and 67/year between 03-07. Ninety-one institutions participated in the meetings, 17/year between 88-92 and 34/year between 03-07. Pediatric Nephrology unit from the H. La Paz (Madrid) contributed the most presentations. Poster presentations were accepted at the ANEP meetings after 1995. Since then, 369 of the 815 presentations followed this format. Between 88-07 only 16 presentations dealt with experimental nephrology. The most common topics of presentation were glomerular disease (203) and urinary tract infection/VUR (132). Fifty-one presentations dealt with dialysis (almost 2/3 peritoneal). Transplantation was the topic of 123 presentations. Of the 21 presentations on molecular genetics only one happened before 1998. CONCLUSIONS: The poster is a useful alternative in scientific presentations which has allowed an increase in presentations, authors and institutions participating in the ANEP meetings. The main topic of presentation was glomerular disease. The frequency of presentations dealing with transplantation has increased in the last years. The past decade has seen more presentations on molecular genetics, but presentations dealing with experimental nephrology are still infrequent.

[Restless legs syndrome in patients diagnosed with attention deficit hyperactivity disorder]. / Sindrome de piernas inquietas en pacientes diagnosticados de trastorno por deficit de atencion/hiperactividad.

INTRODUCTION: Restless legs syndrome (RLS) is a common neurological disease. RLS has been linked to various psychiatric disorders, especially with attention deficit hyperactivity disorder (ADHD). AIMS: The main objective was to describe the frequency of RLS in pediatric patients diagnosed with ADHD. Secondary objectives of the study were describe other sleep disorders in ADHD patients. PATIENTS AND METHODS: A multicentre prospective study was conducted in nine Spanish centers. We included children aged 6-18 years diagnosed with ADHD between January and June 2015. Data were collected by 13 researchers doctors through an interview with the parent/caregiver and with the child. To assess the degree of functioning of patients with ADHD we used the Children's Global Assessment Scale. The Sleep Disturbance Scale for Children was applied to screening sleep disorders in childhood. RESULTS: A sample of 73 patients was collected. Five patients (6.8%) met diagnostic criteria for RLS: four of them definitive and one probable. CONCLUSIONS: RLS is a frequent condition in adulthood but also in adolescence and childhood. ADHD patients have an increased risk of an RLS.

TITLE: Sindrome de piernas inquietas en pacientes diagnosticados de trastorno por deficit de atencion/hiperactividad.Introduccion. El sindrome de piernas inquietas (SPI) es una patologia neurologica comun. Se ha relacionado con diferentes trastornos psiquiatricos, especialmente con el trastorno por deficit de atencion/hiperactividad (TDAH). Objetivos. El objetivo principal fue describir la frecuencia del SPI en pacientes pediatricos diagnosticados de TDAH. Los objetivos secundarios del estudio fueron describir otros trastornos del sueño en pacientes con TDAH. Pacientes y metodos. Se realizo un estudio descriptivo prospectivo multicentrico en nueve centros españoles de niños de 6-18 años con diagnostico de TDAH entre enero y junio de 2015. Los datos fueron recogidos por 13 medicos investigadores mediante entrevista con el padre/cuidador y con el menor. Para valorar el grado de funcionamiento de los pacientes con TDAH se utilizo la Children's Global Assessment Scale. Se aplico la Sleep Disturbance Scale for Children de Bruni para el cribado del trastorno de sueño de la infancia. Resultados. Se recogio una muestra de 73 pacientes. Cinco pacientes (6,8%) cumplen criterios diagnosticos de SPI: cuatro de ellos definitivos y uno probable. Conclusiones. El SPI es una entidad frecuente en la edad adulta, pero tambien en la adolescencia y en la infancia. Los pacientes con TDAH tienen un riesgo aumentado de presentar de manera concomitante un SPI.

[Treatment of obesity in a hospital endocrinology clinic. Influence of parental body mass index]. / Tratamiento de la obesidad en la consulta de endocrinología de un hospital. Influencia del índice de masa corporal de los padres.

INTRODUCTION: Parental obesity is a risk factor for childhood obesity. The aim of this study was to determine if parental obesity influences the adherence and success of obesity treatment in a hospital paediatric endocrinology clinic. MATERIAL AND METHODS: An analytical, prospective, longitudinal study was conducted on obese children aged 4-14. An initial body mass index (BMI), and again at 6 months after receiving health, hygiene and dietary recommendations. Success was considered as a decrease of 0.5 in the BMI Z-score, and adherence to attending the 6-month review. Parental BMI was determined to identify overweight. The χ(2) test was used for qualitative variables and the T-Student test for quantitative (significance, p<<.05). RESULTS: The study included 100 children (52 male), 9.9±2.7 years old, BMI 28.1± 4.5kg/m(2) and BMI Z-Score 3.11±0.98. (85% had a BMI Z-score>3). More than half (59%) of the children had one or both parents obese (41 fathers and 37 mothers were obese). Treatment was not adhered to by 25 children. Adherence was worse if both parents were obese OR 3.65 (1.3 to 10.5) (P<=.01) and adherence was better if the mother was not obese, although the father was (P=.01). The treatment had significant success in 40 patients. If the mother was the only obese one in the family, the possibility of treatment failure was greater OR 5.6 (1.4 to 22.4) (P<.01) CONCLUSIONS: A high percentage of children with severe obesity have obese parents. The mother has an important influence on adherence and response to treatment for the severely obese child.

[Evaluation of isometric force in lower limbs and body composition in preterm infants]. / Valoración de fuerza isométrica en extremidades inferiores y composición corporal en prematuros.

INTRODUCTION: Strength is a physical quality with a clear influence on quality of life. It is determined by the structure of the musculoskeletal system, and depends on the muscular structure. It has been described that prematurity conditions both qualities. The aims of this study are to determine whether prematurity is associated with strength or body composition and evaluate the relationship between prematurity, strength and muscle mass. MATERIAL AND METHODS: Case-control study. Participants were premature 7-to-11 year-old children and full-term birth controls. Strength was measured by a strength gauge and body composition from DEXA (duel-energy X-ray absorptiometry) scans. A total of 89 subjects were included and divided into three groups: 30 prematures with birth-weight ≤ 1500g, 29 prematures with birth-weight 1500-2000g, and 30 controls. RESULTS: Weight and BMI z-score was lower in the premature group. No differences were found in muscular mass or strength between groups. A ratio was established between strength and weight or muscular mass. It was observed that it was possible for them to move four times their weight, without finding any differences between groups or a relationship with birth-weight. CONCLUSIONS: Between 7 and 11 years of age, children who were premature have lower weight and BMI than the rest of the children. However, there were no differences in body composition or strength between preterm children and controls.

[Amount of sleep and changes in its patterns in children less than two years old]. / Cuantificación del sueño y presencia de alteraciones en la duración del sueño en niños menores de 2 años.

OBJECTIVES: The aim is to study the sleep duration and the 24-hour sleep distribution pattern in children under 2 years-old from two different areas of Spain, as well as to determine the prevalence of abnormal sleep duration and the influence of age on this prevalence, and compare them with the already published data. MATERIAL AND METHODS: An observational, descriptive and cross-sectional study on children from two health centers: one in Castilla y León and the other in Asturias. Their parents completed the Brief Infant Sleep Questionnaire (BISQ). Daytime and nighttime sleep duration was assessed, with percentiles being used to evaluate the results. Two groups were created: one according to the age (children under or above 6 months), and the other according to the area. RESULTS: A total of 125 children were studied (73 males). The nighttime sleep duration increased from 8.28±2.06 hours to 10.43±1.21 hours (P=.0001) and the daytime sleep decreased from 4.61±2.66 to 1.96±0.79 hours (P=.0001) for children under 6 months and above 6 months, respectively. The children from Castilla y León slept more during daytime than those from Asturias (3.91±2.67 vs 3.00±2.09 hours, P=.041). Nineteen children had a total sleep duration below the 2nd percentile. A sleep duration below normal percentiles was observed in children under 6 months (14/72 vs 5/53, P=.006). CONCLUSIONS: As children grow older, they substitute daytime sleep for nighttime sleep. Approximately 15% of the children under 2 years-old may have changes in sleep duration, and is more common in children under 6 months.

Fatty acid composition of liver lipids during ontogeny of the chick embryo: effect of a single dose of triiodothyronine.

Fatty acid composition of chick liver was determined throughout embryo development. Fourteen, seventeen and twenty day old chick embryos as well as newborn chicks were studied. Chromatograms show that in all cases the same fatty acids were presented, even when lipid fractions such as phospholipids, cholesterol and triacylglycerol were studied separately. The lack of poly-unsaturated fatty acids is in contrast with the results obtained in previous investigations on rats. A slight increase in the unsaturated/saturated fatty acid ratio as the embryo grew older was observed. The effect of a single dose of 3-3'-5-Triiodothyronine (T3) on composition of fatty acids throughout the embryonary period was also studied. The hormone only affects fatty acid levels in the 20th and 21st days of incubation.

A study of the hepatic lipids during chick embryo development. Effect of triiodothyronine.

The content of hepatic phospholipids, cholesterol and triglycerides was studied in 14, 17 and 20 day old chick embryos and newborn chicks (21 incubation days). An increase in the total lipid content and in the other lipidic fractions throughout embryo development is observed, mainly between 17 and 20 days of incubation, remaining practically constant until the hatchability day. Nevertheless a sharp depletion of triglycerides in broken eggshell day is revealed. Effect of a single dose of 3-3'-5-Triiodothyronine (T3) on hepatic lipid levels was determined. Throughout embryonary period, the hormone only affects triglyceride levels enhancing its biosynthesis in the 20th incubation day. All the other variations are described in newborn animals.

[Effect of the thyroid hormone, triiodothyronine on fatty acid chain elongation of mitochondrial phospholipids (author's transl)]. / Efecto de la hormona tiroidea triiodotironina sobre la elongación de los ácidos grasos de fosfolípidos mitocondriales.

The effect of the Thyroid Hormone Triiodothyronine (T3) on composition of Fatty Acid of Mitochondrial Phospholipids is studied. In animals (Gallus domesticus var. Cornis and White Roc.) treated with an injection of T3 containing 0.04 microgram/g body weight, increase length and insaturation increases of the hydrocarbon chains of fatty acids in mitochondrial phospholipids are observed in relation to the corresponding controls. This elongation is maximum at 14 hours after treatment with the hormone. Animals treated with T3 showed a higher proportion of stearic and eicosatrienoic acids than controls. This increase is accompanied by an equivalent decrease of the palmitic and oleic acids, their respective biosynthetic precursors. The highest proportion of long chain and unsatured fatty acids can be observed in phosphatidylethanolamines and phosphatidylcholines.

[The effect of the thyroid hormone triiodothyronine on the incorporation of 32P into brain phospholipids in rat and chicken (author's transl)]. / Efecto de la triiodotironina sobre la incorporación de P32 a fosfolípidos cerebrales de pollo y rata.

The incorporation of 32P to brain phospholipids has been studied, in chicken as well as in rat. Both animal species were at different stage of brain development, although degrees of psychomotive development were identical. It is observed that the effect of triiodothyronine is more pronounced when the degree of brain myelination is smaller. The radioactive precursor is frequently incorporated to choline and ethanolamine phospholipids, and this incorporation is higher in hyperthyroid animals with a low degree of myelination. The triiodothyronine activates, in both animal species, the salvage pathway of phospholipid biosynthesis. This activation takes place 36 h before hormone action in chicken, while in rat, the most significant differences occur 36 h after hormone action.