Persistence and effectiveness of guselkumab treatment in patients with moderate-to-severe plaque psoriasis in a non-interventional real-world setting: The SPRING study.

BACKGROUND: Guselkumab is a monoclonal antibody that blocks the IL-23 pathway with proven efficacy and tolerability in the treatment of moderate-to-severe plaque psoriasis. OBJECTIVES: To assess the persistence, effectiveness and safety of guselkumab in patients with moderate-to-severe psoriasis in real clinical practice in Spain. METHODS: SPRING was a Phase IV, retrospective and non-interventional study analysing patients with moderate-to-severe plaque psoriasis who had initiated guselkumab under clinical practice conditions at least 12 months before inclusion in the study. The primary endpoint was persistence (non-persistence: discontinuation or interruption ≥90 days). Effectiveness was assessed using the Psoriasis Area Severity Index (PASI) and Investigator Global Assessment (IGA). Dermatology Life Quality Index (DLQI) and safety were also evaluated. RESULTS: A total of 284 patients were included between September 2020 and June 2021. The 1-year probability of persistence was 89.6% (86.1%-93.3%). The 1-year probability of persistence was also calculated according to prior biologic treatment, being 90.3% for biologic-naïve patients and 89.5% for patients who received one or more biologic therapies before guselkumab. Additionally, patients were also classified based on the frequency of the administration of guselkumab treatment; the 1-year probability of persistence was 91.9% in patients receiving guselkumab according to the Summary of Product Characteristics and 89.3% in patients with lengthened intervals of administration. After 1 year, PASI 90 was achieved by 56.4% of patients, IGA 0/1 response and BSA <3% were achieved by 65.5% and 77.8% of patients, respectively, and 65.8% achieved a minimal clinically significant difference (>4-point reduction) in the DLQI score at 1 year. Twenty-six adverse reactions (4 of them serious) were reported in 16 patients. CONCLUSIONS: This study suggests that guselkumab has high persistence in real clinical practice in Spain, independently of the previous biologic treatments and changes in the frequency of treatment. Effectiveness and safety are consistent with previously published data.

First report of eprinomectin-resistant isolates of Haemonchus contortus in 5 dairy sheep farms from the Pyrénées Atlantiques département in France.

Infection of sheep by gastrointestinal nematodes (GIN) in pastoral systems such as those found in the South Western area of France, the Pyrénées Atlantiques, is one of the main reasons for economic loss and degradation of their welfare. In the present study, the efficacy of eprinomectin (EPN) was monitored on farms from this area following suspicion of lack of anthelmintic efficacy. Suspicions were raised by veterinarians, based on clinical signs ranging from milk and body condition loss, to anaemia, and mortality. Resistance was evaluated according to the World Association for the Advancement for Veterinary Parasitology (WAAVP) guidelines using fecal egg count reduction tests reinforced by individual analysis of drug concentration in the serum of all treated ewes by high-performance liquid chromatography (HPLC). EPN was administered by subcutaneous (SC) and topical (T) route according to manufacturer's requirements, as well as by the oral route (O) with the topical solution according to off-labelled practices in the field. For the first time in France, the presence of resistant isolates of Haemonchus contortus to EPN was observed in 5 dairy sheep farms. The HPLC dosages showed exposure of worms to concentrations compatible with anthelmintic activity for animals treated by the SC and O routes. By contrast, they showed under exposure to the drug of most individuals treated by the T route. EPN is the only null milk withdrawal anthelmintic molecule currently available. The presence of resistant isolates of the pathogenic H. contortus to EPN in this important dairy region requires an urgent change in grazing, and sometimes production, systems.

[A protocol for the treatment of retinopathy of prematurity in Spain]. / Protocolo de tratamiento de la retinopatía del prematuro en España.

OBJECTIVE: To prepare a protocol for the treatment of retinopathy of prematurity (ROP) agreed by the majority of Spanish ophthalmologists dedicated to this topic. MATERIAL AND METHOD: A draft of the protocol was produced taking into account the experience of the participants and up to date publications. This draft was corrected by all the ophthalmologists participating in the project, and the final document was agreed by all of them. RESULTS: We present general guidelines as an aid for the treatment of ROP, including treatment criteria, treatment methods, a calendar of action, and follow-up. CONCLUSIONS: It is important to have a common working protocol for the treatment of ROP to improve care and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain.

Protocolo de tratamiento de la retinopatía del prematuro en España / A protocol for the treatment of retinopathy of prematurity in Spain

Objetivo: Realizar un protocolo de tratamiento de la retinopatía del prematuro (ROP) consensuado por la mayor parte de oftalmólogos españoles dedicados al tema. Material y método: Se realizó un borrador del protocolo según la experiencia de los participantes y las publicaciones actualizadas. Este borrador fue corregido por los participantes en el protocolo y se llegó al documento final consensuado por todos los participantes. Resultados: Se presentan las directrices generales para realizar el tratamiento de la ROP, incluyendo criterios de tratamiento, metodología de actuación, calendario de actuación y seguimiento. Conclusiones: Es importante disponer de un protocolo de actuación común en el tratamiento de la ROP para mejorar la actuación y evitar errores. Aunque cada centro hospitalario deba adaptar el protocolo a su actividad clínica, es recomendable que existan un mínimo de procedimientos consensuados por todos los oftalmólogos dedicados a la ROP (AU)

Objective: To prepare a protocol for the treatment of retinopathy of prematurity (ROP) agreed by the majority of Spanish ophthalmologists dedicated to this topic. Material and method: A draft of the protocol was produced taking into account the experience of the participants and up to date publications. This draft was corrected by all the ophthalmologists participating in the project, and the final document was agreed by all of them. Results: We present general guidelines as an aid for the treatment of ROP, including treatment criteria, treatment methods, a calendar of action, and follow-up. Conclusions: It is important to have a common working protocol for the treatment of ROP to improve care and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain (AU)

Programa de cribado para la retinopatía del prematuro en España / Screening program for retinopathy of prematurity in Spain

Objetivo: Realizar un protocolo de cribado de la retinopatía del prematuro (ROP), consensuado por la mayor parte de oftalmólogos españoles dedicados al tema. Material y método: Se realizó un borrador del protocolo según la experiencia de los participantes y las publicaciones actualizadas. Este borrador fue corregido por los participantes en el protocolo y se llegó al documento final consensuado por todos los participantes. Resultados: Se presentan las directrices generales para realizar el cribado de la ROP, incluyendo criterios de inclusión y exclusión, metodología de exploración y calendario de actuación. Conclusiones: Es importante disponer de un protocolo de actuación común en el cribado de la ROP para mejorar la actuación y evitar errores. Aunque cada centro hospitalario deba adaptar el protocolo a su actividad clínica es recomendable que existan un mínimo de procedimientos consensuados por todos los oftalmólogos dedicados a la ROP (AU)

Objective: To prepare a retinopathy of prematurity (ROP) screening program as agreed by most of Spanish ophthalmologists dedicated to this topic. Material and method: A draft of the protocol was produced taking into account the experience of the participants and current publications. This draft was corrected by all the ophthalmologists participating in the project and the final document produced was agreed by all of them. Results: We present general guidelines to help in the screening of ROP, including treatment criteria, treatment methods, and a calendar of action. Conclusions: It is important to have a common working protocol in the screening of ROP to improve the action and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain (AU)

Screening program for retinopathy of prematurity in Spain.

OBJECTIVE: To prepare a retinopathy of prematurity (ROP) screening program as agreed by most of Spanish ophthalmologists dedicated to this topic. MATERIALS AND METHODS: A draft of the protocol was produced taking into account the experience of the participants and current publications. This draft was corrected by all the ophthalmologists participating in the project and the final document produced was agreed by all of them. RESULTS: We present general guidelines to help in the screening of ROP, including treatment criteria, treatment methods, and a calendar of action. CONCLUSIONS: It is important to have a common working protocol in the screening of ROP to improve the action and to avoid mistakes. Although individual Hospitals may adapt the protocol to their daily activity, it is recommended that there is a minimal working protocol agreed by most of professionals dedicated to pediatric ophthalmology in Spain.

¿Es la persistencia de ductus arterioso un factor de riesgo para la retinopatía del prematuro? / Is patent ductus arteriosus a risk factor for retinopathy of prematurity?

Introducción: Evaluar si la presencia de persistencia de ductus arterioso (PDA) aumenta la proporción de retinopathy of prematurity (ROP, «retinopatía del prematuro») o su gravedad. Pacientes y método: Se incluyó en el estudio una cohorte consecutiva de prematuros diagnosticados de PDA por ecocardiograma nacidos en el Hospital Universitario Miguel Servet desde enero de 2006 hasta mayo de 2009. Comparamos la proporción y gravedad de ROP en este grupo en comparación con un grupo control sin PDA aleatoriamente elegido. Resultados: Estudiamos un total de 131 prematuros: 79 con PDA y 52 sin PDA. Encontramos relación estadísticamente significativa entre la presencia de PDA y la proporción de ROP (p=0,03) (OR=2,41; 95% CI 1,08–5,38), sin que existieran diferencias significativas en la edad gestacional (EG) entre los grupos. Sin embargo, al usar un modelo de regresión múltiple ajustado a la EG, hay una relación significativa entre ROP y EG (β=−0,322; p=0,003), pero no existe relación estadísticamente significativa entre ROP y PDA (p=0,073 para proporción de ROP y p=0,20 para estadio de ROP). Conclusiones: La presencia de PDA no aumenta el riesgo de desarrollar ROP ni su gravedad, una vez que se considera el efecto de la EG en la presencia de PDA (AU)

Objective: To evaluate whether the presence of patent ductus arteriosus (PDA) increases the rate of retinopathy of prematurity (ROP) or its severity. Patients and method: A consecutive cohort of premature infants diagnosed with PDA by echocardiography born in the Miguel Servet University Hospital from January 2006 to May 2009 was included in the study. We compared the rate and severity of ROP in this group of patients with a control group of randomly-chosen premature infants with a normal echocardiography. Results: We evaluate a total of 131 premature infants (79 with PDA and 52 without PDA). We found a statistically significant association between the presence of PDA and the rate of ROP (p=0.03) (OR=2.41; 95% CI 1.08–5.38). However, using a multiple regression model adjusted for the gestational age (GA), a significant reverse association was found between ROP and GA (β=−0.322; p=0.003), but there was no statistically significant correlation between ROP and PDA (p=0.073 for rate of ROP and a p=0.20 for stage of ROP). Conclusions: The presence of PDA does not increase the risk of developing ROP or its severity, once considered the effect of gestational age in PDA (AU)

[Is patent ductus arteriosus a risk factor for retinopathy of prematurity?]. / ¿Es la persistencia de ductus arterioso un factor de riesgo para la retinopatía del prematuro?

OBJECTIVE: To evaluate whether the presence of patent ductus arteriosus (PDA) increases the rate of retinopathy of prematurity (ROP) or its severity. PATIENTS AND METHOD: A consecutive cohort of premature infants diagnosed with PDA by echocardiography born in the Miguel Servet University Hospital from January 2006 to May 2009 was included in the study. We compared the rate and severity of ROP in this group of patients with a control group of randomly-chosen premature infants with a normal echocardiography. RESULTS: We evaluate a total of 131 premature infants (79 with PDA and 52 without PDA). We found a statistically significant association between the presence of PDA and the rate of ROP (p=0.03) (OR=2.41; 95% CI 1.08-5.38). However, using a multiple regression model adjusted for the gestational age (GA), a significant reverse association was found between ROP and GA (ß= -0.322; p=0.003), but there was no statistically significant correlation between ROP and PDA (p=0.073 for rate of ROP and a p=0.20 for stage of ROP). CONCLUSIONS: The presence of PDA does not increase the risk of developing ROP or its severity, once considered the effect of gestational age in PDA.

Microftalmía por deficiencia materna de vitamina A como complicación de derivación biliopancreática: comunicación de un caos / Microphthalmia for maternal vitamina A deficiency: complications of biliopancreatic diversión: report of a case

Los procedimientos quirúrgicos bariáticos pueden ocasionar graves alteraciones nutricionales que pueden afectar a la embriogénesis con riegos potenciales para el feto, pudiendo asociarse con una amplio espectro malformativo principalmente a nivel ocular. Se presenta el caso de una mujer que, tres años después de someterse a una derivación biliopancreática por obesidad mórbida, tuvo un recién nacido a término con una microfatmía bilateral severa. En la madre se confirmó una deficiencia de vitamina A con unos niveles séricos de 0,05 mg/dl (rango normal de 0,3-0,8 mg/dl) y en el recién nacido se realizaron las exploraciones complementarias para descartar otras causas etiológicas asociadas con microftalmía y se evaluó el estado vitamínico A, D, E y K que mostró una concentración también baja de vitamina A (< 0,10 mcg/ml. Rango normal 0,25-0,45 mcg/ml). Se hace una revisión de los principales aspectos relacionados con la deficiencia materna de vitamina A y la anoftamía/microftalmía (AU)

Bariatric surgery can carry important nutritional deficiencies that might impair embryogenesis with potential risks to the fetus. It could be associated with a wide spectrum of fetal malformations, mainly congenital eye malformations. We report the case of a woman who had undergone biliopancreatic diversion 3 years before and gave birth to a child with severe bilateral microphtalmia. The mother had a documented vitamin A deficiency with serum levels of 0,05 mg/dl (normal reference range 0,3-0,8 mg/dl). In the newborn baby other causes of microphthalmia were evaluated, showing low vitamin A levels (<0,10 mcgr/ml, normal range 0,25-0,45 mcgr/ml). The most important aspects related to maternal hypovitaminosis A and microphthalmia/anophtalmia have been checked (AU)

[New anaesthetic technique in diode laser treatment of retinopathy of prematurity]. / Nueva técnica anestésica en el tratamiento con láser diodo de la retinopatía del prematuro.

INTRODUCTION: Anaesthesia in premature infants can have many complications. Although the application of diode laser is less painful than cryotherapy, there has to be adequate immobilization of the patient to provide a correct focus of the spot. At Hospital Infantil de Zaragoza, the same standard anaesthetic technique has been applied since 1999, obtaining sedation with inhaled anaesthetic agents combined with topical anaesthesia. We analyse the results obtained on the application of this technique. MATERIAL AND METHOD: The study included 72 consecutive premature infants treated with diode laser for retinopathy of prematurity (ROP), using an anaesthetic technique combining inhalatory sedation and topical anaesthesia. The personal data of each patient was collected (gestational age, birth weight, postconceptional age at the time of initial treatment, associated systemic disorders) together with information related to the surgical intervention (duration, intraoperative and postoperative complications). RESULTS: Intraoperative complications occurred in 12 cases (16.66%). These were self-limited in 9 cases and only 3 cases required orotracheal intubation (4.16%). Postoperative complications occurred in 4 cases (5.55%) during the 48 h following treatment. No statistically significant relationship was found between the presence of intraoperative complications and the mean gestational age and birth weight, and the presence of apnoea, intraventricular haemorrhage or a permeable ductus. A statistically significant relationship was found between the presence of postoperative complications and significant intraventricular haemorrhage. CONCLUSIONS: This anaesthetic technique combining inhalatory gases and topical anaesthesia is safe, with few complications and comfortable for the surgeon.

Nueva técnica anestésica en el tratamiento con láser diodo de la retinopatía del prematuro / New anaesthetic technique in diode laser treatment of retinopathy of prematurity

Introducción: La anestesia en prematuros es susceptible de tener múltiples complicaciones. Aunque la aplicación del láser diodo es menos dolorosa que la crioterapia, se debe conseguir una correcta inmovilización del paciente para un enfoque y una aplicación adecuados del mismo. Desde 1999 está protocolizada en el Hospital Infantil de Zaragoza una técnica anestésica que utiliza sedación con agentes inhalatorios asociada a anestesia tópica. Analizamos los resultados obtenidos en la utilización de dicha técnica. Material y método: Se incluyen 72 prematuros consecutivos tratados con láser por retinopatía del prematuro (ROP) utilizando la técnica anestésica de sedación inhalatoria y anestesia tópica. Se han recogido datos de los pacientes (edad gestacional, peso al nacer, edad posconcepcional en el momento del tratamiento inicial, patología sistémica asociada) y del acto quirúrgico (tiempo de duración y complicaciones intraoperatorias y postoperatorias). Resultados: Se produjeron complicaciones intraoperatorias en 12 casos (16,66 %). En 9 casos fueron autolimitadas y sólo 3 precisaron intubación orotraqueal (4,16 %). En 4 casos (5,55 %) se produjeron complicaciones postoperatorias en las 48 h posteriores al tratamiento. No se encontró relación estadísticamente significativa entre la presencia de complicaciones intraoperatorias y la media de edad gestacional y peso al nacer o la presencia de apneas, hemorragia intraventricular o ductus permeable. Se encontró relación estadísticamente significativa entre la presencia de complicaciones postoperatorias y la presencia de hemorragia intraventricular significativa. Conclusiones: La técnica anestésica con gases inhalatorios asociada a anestesia tópica es segura, con escasa proporción de complicaciones y cómoda para el cirujano (AU)

Introduction: Anaesthesia in premature infants can have many complications. Although the application of diode laser is less painful than cryotherapy, there has to be adequate immobilization of the patient to provide a correct focus of the spot. At Hospital Infantil de Zaragoza, the same standard anaesthestic technique has been applied since 1999, obtaining sedation with inhaled anaesthetic agents combined with topical anaesthesia. We analyse the results obtained on the application of this technique. Material and method: The study included 72 consecutive premature infants treated with diode laser for retinopathy of prematurity (ROP), using an anaesthestic technique combining inhalatory sedation and topical anaesthesia. The personal data of each patient was collected (gestational age, birth weight, postconceptional age at the time of initial treatment, associated systemic disorders) together with information related to the surgical intervention (duration, intraoperative and postoperative complications). Results: Intraoperative complications occurred in 12 cases (16.66 %). These were self-limited in 9 cases and only 3 cases required orotracheal intubation (4.16 %). Postoperative complications occurred in 4 cases (5.55 %) during the 48 h following treatment. No statistically significant relationship was found between the presence of intraoperative complications and the mean gestational age and birth weight, and the presence of apnoea, intraventricular haemorrhage or a permeable ductus. A statistically significant relationship was found between the presence of postoperative complications and significant intraventricular haemorrhage. Conclusions: This anaesthestic technique combining inhalatory gases and topical anaesthesia is safe, with few complications and comfortable for the surgeon (AU)

[Diode laser therapy in retinopathy of prematurity: more than a decade of experience]. / Tratamiento con láser diodo en la retinopatía del prematuro: más de una década de experiencia.

BACKGROUND: Retinopathy of prematurity (ROP) is one of the most important causes of blindness in childhood. The introduction of diode laser has represented a significant advance in its treatment. The aim of the present study was to evaluate our results in the treatment of this entity after more than a decade of experience. PATIENTS AND METHODS: One hundred eighty-two eyes in 92 premature infants with ROP treated with diode laser in the Hospital Universitario Miguel Servet from 1992 to 2003 were studied. The characteristics of the population (gestational age, birth weight, sex, stage and affected zone) and treatment outcomes were analyzed. The change in the criteria indicating this treatment was also analyzed. RESULTS: In the treated population, mean gestational age (27.9 weeks) and birth weight (1015.9 g) were similar to those in other published studies. A favorable outcome was achieved in 169 of the 182 cases (92.8 %). The change in criteria increased the proportion of favorable outcomes to 96.1 %. DISCUSSION: Diode laser therapy is currently the treatment of choice in ROP. This treatment, based on guidelines for earlier intervention, provide greater efficacy without increasing morbidity.

Tratamiento con láser diodo en la retinopatía del prematuro: más de una década de experiencia / Diode laser therapy in retinopathy of prematurity: More than a decade of experience

Antecedentes: La retinopatía del prematuro (ROP) constituye una de las causas más importantes de ceguera en la infancia. La introducción del láser diodo ha constituido un avance significativo en su tratamiento. El presente estudio pretende evaluar nuestros resultados en el tratamiento de esta patología, con más de una década de experiencia. Pacientes y métodos: Se han estudiado 182 ojos de 92 prematuros con ROP tratados con láser diodo en el Hospital Universitario Miguel Servet desde 1992 hasta 2003. Se analizan las características de la población (edad gestacional, peso al nacimiento, sexo, estadio y zona de la enfermedad) y el resultado del tratamiento. Se evalúa además el cambio en los criterios de indicación del mismo. Resultado: La población tratada tiene una media de edad gestacional (27,9 semanas) y peso al nacimiento (1.015,9 g) similares a las de otros autores. Se ha logrado evolución favorable en 169 de 182 casos (92,8 %). Con el cambio en los criterios ha aumentado la proporción de evoluciones favorables al 96,1 %. Discusión: Actualmente el láser diodo es el tratamiento de elección en la ROP. El tratamiento, basándose en pautas de actuación más precoces, proporciona mayor eficacia sin aumentar la morbilidad

Background: Retinopathy of prematurity (ROP) is one of the most important causes of blindness in childhood. The introduction of diode laser has represented a significant advance in its treatment. The aim of the present study was to evaluate our results in the treatment of this entity after more than a decade of experience. Patients and methods: One hundred eighty-two eyes in 92 premature infants with ROP treated with diode laser in the Hospital Universitario Miguel Servet from 1992 to 2003 were studied. The characteristics of the population (gestational age, birth weight, sex, stage and affected zone) and treatment outcomes were analyzed. The change in the criteria indicating this treatment was also analyzed. Results: In the treated population, mean gestational age (27.9 weeks) and birth weight (1015.9 g) were similar to those in other published studies. A favorable outcome was achieved in 169 of the 182 cases (92.8 %). The change in criteria increased the proportion of favorable outcomes to 96.1 %. Discussion: Diode laser therapy is currently the treatment of choice in ROP. This treatment, based on guidelines for earlier intervention, provide greater efficacy without increasing morbidity

[Familial hypomagnesemia with hypercalciuria and nephrocalcinosis. Association with ocular abnormalities]. / Hipomagnesemia familiar con hipercalciuria y nefrocalcinosis y asociación con alteraciones oculares.

BACKGROUND: Familial hypomagnesemia with hypercalciuria and nephrocalcinosis is an unusual disease that usually leads to end-stage renal failure. There is no specific treatment and, to a variable degree, patients with this disease present ocular abnormalities. The illness is due to a defect in the reabsorption of magnesium and calcium at the thick ascending limb of Henle because of a mutation of the PCLN-1 gene, which encodes a protein, paracellin-1, which intervenes in the reabsorption of both cations. OBJECTIVE: To review outcome and the incidence of ocular abnormalities in our patients and in cases described in Spain and to compare the incidence found with that in groups from other countries. METHOD: Retrospective study of a group of patients with familial hypomagnesemia with hypercalciuria and nephrocalcinosis diagnosed at a hospital. RESULTS: There were six girls and three boys with clinical symptoms of polyuria, polydipsia, and less frequently, urinary tract infections and lithiasis. All had hypomagnesemia, hypercalciuria and nephrocalcinosis. Five of the patients had renal failure at diagnosis and four underwent transplantation without recurrence. Eight patients had diverse ocular abnormalities. Eighty-one percent of Spanish patients had ocular abnormalities compared with 24 % of those from other countries. There was no evidence of successful medical treatment. CONCLUSIONS: Almost half of the patients presented chronic renal failure at diagnosis and most of the patients reached end-stage renal failure in the second or third decade of life. Normal glomerular filtration rate was found only in patients diagnosed at an early age. The most frequent extra-renal association in Spanish patients (81 %) corresponded to ocular abnormalities. Effective treatment consists of kidney transplantation that completely corrects the tubular disorder.

Hipomagnesemia familiar con hipercalciuria y nefrocalcinosis y asociación con alteraciones oculares / Familial hypomagnesemia with hypercalciuria and nephrocalcinosis. Association with ocular abnormalities

Antecedentes: La hipomagnesemia familiar con hipercalciuria y nefrocalcinosis es una enfermedad rara que sin tratamiento específico suele conducir a insuficiencia renal terminal y que en grado variable presenta alteraciones oculares. La enfermedad se debe a un defecto en la reabsorción de magnesio y calcio en el segmento grueso de la rama ascendente del asa de Henle debido a una mutación del gen PCLN1, que codifica una proteína, la paracelina-1, que interviene en la reabsorción de ambos cationes. Objetivo: Revisar la evolución clínica de nuestros pacientes y la incidencia de anomalías oculares en nuestros casos y en los casos descritos procedentes de España, comparándola con grupos de otros países. Método: Estudio retrospectivo de un grupo de pacientes con esta enfermedad diagnosticados en un hospital. Resultados: Se presentan 6 niñas y 3 niños con síntomas de poliuria, polidipsia y en menor frecuencia infección del tracto urinario y litiasis. Todos tenían hipomagnesemia, hipercalciuria y nefrocalcinosis. Cinco presentaban insuficiencia renal al ser diagnosticados y cuatro fueron trasplantados sin presentar recidiva de su enfermedad. Ocho presentaban anomalías oculares diversas. El 81 por ciento de los pacientes españoles presentaron anomalías oculares frente al 24 por ciento de otros países. No existe evidencia de tratamiento eficaz. Conclusiones: Casi la mitad de los casos presentaron insuficiencia renal crónica en el momento del diagnóstico y la mayoría de los pacientes alcanzan la insuficiencia renal terminal en la segunda o tercera década de la vida. Sólo los casos de diagnóstico en edades tempranas tenían un filtrado glomerular normal. La asociación extrarrenal más frecuente en pacientes españoles (81 por ciento) corresponde a alteraciones oculares. El tratamiento efectivo es el trasplante renal, que corrige completamente el trastorno tubular. (AU)

[Ophthalmological exploration of children with Down's syndrome. Main results and comparison with a control group]. / Exploración oftalmológica en niños con síndrome de Down: principales resultados y comparación con un grupo control.

BACKGROUND: Down's Syndrome is the most frequent chromosomal aberration. There is a wide variety of symptoms. From an ophthalmological point of view, numerous alterations associated with Down's Syndrome have been described. MATERIAL AND METHODS: We have carried out a complete ophthalmic exploration on 60 children with Down's Syndrome and 60 control children. RESULTS: The first group showed a high percentage of refraction errors (90%), nystagmus (28%), strabismus (48%) and cataracts (13%). If we compare these results with those of the control group we find that the Down's group has a frequency significantly higher both in refraction errors as a whole (p < 0.001) and myopia (p < 0.01), hypermetropia (p < 0.02) and stigmatism (p < 0.001). They have also shown a frequency significantly higher of strabismus (p < 0.001). CONCLUSION: All of the observed alterations can have a negative influence on the appropriate educational development of these children. An early ophthalmological exploration would be advisable in children with Down's Syndrome.