Purtscher-like retinopathy in a paediatric patient with haemolytic uraemic syndrome: A case report and literature review.

An 8-year-old boy presented with fever, vomits, bloody diarrhoea, and blurred vision. The patient was diagnosed with Haemolytic Uraemic Syndrome (HUS) due to the symptoms and a positive Verotoxin stool test. Funduscopic examination showed retinal involvement in both eyes, peri-papillary paleness, retinal haemorrhages, and soft "Purtscher Fleckens" exudates. A favourable outcome was achieved after hospital admission and systemic treatment. Dialysis treatment was not needed due the preserved diuresis. Although Purtscher-like retinopathy is very uncommon, ocular examination is mandatory in patients with pancreatitis, autoimmune diseases, and thrombotic microangiopathies, such as HUS.

Retinopatía Purtscher-like en paciente pediátrico con síndrome urémico hemolítico: reporte de caso y revisión de la literatura / Purtscher-like retinopathy in a paediatric patient with haemolytic uraemic syndrome: A case report and literature review

Se presenta el caso clínico de un paciente de 8 años de edad con un cuadro febril, vómitos, diarrea con sangre y visión borrosa. El paciente fue diagnosticado de síndrome urémico hemolítico (SHU) por clínica y detección de verotoxina en materia fecal. La exploración fundoscópica mostró compromiso retiniano en ambos ojos, palidez peripapilar, hemorragias retinianas y exudados blandos en forma de Purtscher «flecken». La evolución fue favorable tras el ingreso y tratamiento sistémico, no requiriendo diálisis por diuresis conservada. La exploración oftalmológica de estos pacientes es fundamental para el estudio del paciente, ya que, a pesar de ser infrecuente, la retinopatía Purtscher-like puede verse en cuadros de pancreatitis, enfermedades autoinmunes y microangiopatías trombóticas como el SHU (AU)

An 8-year-old boy presented with fever, vomits, bloody diarrhea, and blurred vision. The patient was diagnosed with haemolytic uraemic syndrome (HUS) due to the symptoms and a positive verotoxin stool test. Funduscopic examination showed retinal involvement in both eyes, peri-papillary paleness, retinal haemorrhages, and soft Purtscher «fleckens» exudates. A favourable outcome was achieved after hospital admission and systemic treatment. Dialysis treatment was not needed due the preserved diuresis. Although Purtscher-like retinopathy is very uncommon, ocular examination is mandatory in patients with pancreatitis, autoimmune diseases, and thrombotic microangiopathies, such as HUS (AU)

Purtscher-like retinopathy in a paediatric patient with haemolytic uraemic syndrome: A case report and literature review. / Retinopatía Purtscher-like en paciente pediátrico con síndrome urémico hemolítico: reporte de caso y revisión de la literatura.

An 8-year-old boy presented with fever, vomits, bloody diarrhea, and blurred vision. The patient was diagnosed with haemolytic uraemic syndrome (HUS) due to the symptoms and a positive verotoxin stool test. Funduscopic examination showed retinal involvement in both eyes, peri-papillary paleness, retinal haemorrhages, and soft Purtscher «fleckens¼ exudates. A favourable outcome was achieved after hospital admission and systemic treatment. Dialysis treatment was not needed due the preserved diuresis. Although Purtscher-like retinopathy is very uncommon, ocular examination is mandatory in patients with pancreatitis, autoimmune diseases, and thrombotic microangiopathies, such as HUS.

Pars Planitis en la infancia / Pars Planitis in childhood

Objetivo: Evaluar la respuesta al tratamiento con corticoides e inmunomoduladores en niños con pars planitis. Materiales y método: Se realiza un estudio retrospectivo y observacional de nueve historias clínicas de pacientes con diagnóstico de pars planitis atendidos en el servicio de oftalmología del Hospital de Pediatría Juan P. Garrahan desde el año 2010. Resultados: De los nueve pacientes 6 eran niños y 3 niñas. El motivo de consulta principal fue disminución de la visión. La mayoría de los pacientes fueron derivados por otros oftalmólogos para tratamiento. El 100% sufrió afectación ocular bilateral. Las complicaciones fueron: catarata, queratopatía en banda, hipertensión ocular y edema macular cistoide. El 100% de los pacientes recibió tratamiento con corticoides por vía oral e inmunomoduladores. A algunos se les efectuó inyecciones de triamcinolona subtenoniana y criocoagulación. Conclusiones: con el tratamiento con prednisona e inmunomoduladores se logró mejoría en la agudeza visual en 15 ojos y 3 ojos mantuvieron igual agudeza visual. La pars planitis es una patología crónica con un pronóstico visual reservado que requiere de un seguimiento estrecho y tratamiento prolongado por parte de un equipo interdisciplinario (AU)

Objective: To assess response to steroid and immunomodulating treatment in children with pars planitis. Material and methods: A retrospective observational study was conducted reviewing nine clinical charts of children with a diagnosis of pars planitis seen at the Department of Ophthalmology of the Pediatric Hospital Juan P. Garrahan since 2010. Results: Of nine patients, six were boys and three were girls. Main complaint was loss of vision. The majority of patients were referred to our hospital by other ophthalmologists for treatment. All children had bilateral eye involvement. Complications observed were: cataracts, band keratopathy, ocular hypertension, and cystoid macular edema. All patients received oral steroids and immunomodulating treatment. In some subtenonian injection of triamcinolone and cryocoagulation was performed. Conclusions: Prednisone and immunomodulating treatment resulted in an improvement of visual acuity in 15 eyes and three eyes visual acuity remained unchanged. Pars planitis is a chronic disease with an uncertain visual prognosis that requires close follow-up and prolonged treatment by an interdisciplinary team (AU)

Síndrome del niño sacudido o Shaken Baby Syndrome: Lesiones oculares / Shaken Baby Syndrome: Eye lesions

Introducción: el síndrome del niño sacudido se incluye en la clasificación del maltrato físico. Las lesiones oculares se deben a la tracción producida sobre la retina a partir del vítreo del niño, fuertemente unido a ella, con desgarro de las capas retinales y colección de sangre en la cavidad resultante (retinosquisis hemorrágica). Objetivo: describir las manifestaciones oftalmológicas iniciales y la evolución del síndrome del bebé sacudido. Materiales y métodos: se realiza un estudio descriptivo y retrospectivo de cuatro pacientes con el síndrome del niño sacudido. Las variables analizadas son: sexo, edad, fondo de ojos, ecografía ocular, tratamiento y evolución visual. Resultados: los casos 1 y 2 eran gemelos, uno de ellos presentó manifestaciones a nivel del fondo de ojos y el segundo no, ambos requirieron intervención neuroquirúrgica. El tercero presentaba lesiones a nivel ocular con opacidad de medios por lo cual se le realizó en ambos ojos vitrectomía exploradora. El cuarto caso se trataba de una paciente de 6 meses con hemovítreo en ojo derecho y retinosquisis hemorrágica en ojo izquierdo, y requirió vitrectomía para la resolución del hemovítreo, aunque el resultado visual fue desfavorable. Conclusión: el examen oftalmológico contribuye al diagnóstico del síndrome del bebé sacudido por lo que es necesario en la evaluación de los niños pequeños que presenten lesiones sospechosas de abuso (AU)

Introduction: Shaken baby syndrome is included in the classification of physical abuse. Ocular lesions in the infant are due to traction to the retina from the adjacent vitreous causing with tears of the retinal layers and blood collection in the resulting cavity (hemorrhagic retinoschisis). Objective: To describe early ocular manifestations and outcome of shaken baby syndrome. Material and methods: A retrospective, descriptive study was conducted in four patients with shaken baby syndrome. Sex, age, ocular fundus, ultrasonography, treatment, and visual outcome were analyzed. Results: Cases 1 and 2 were twins, one of them presented with fundus manifestations, the other did not; both required neurosurgical intervention. The third infant had eye lesions with media opacities and therefore exploratory vitrectomy was performed in both eyes. The fourth was a 6-month-old baby girl with hemovitreous in the right and hemorrhagic retinoschisis in the left eye. She required vitrectomy to resolve the hemovitreous, but the result was poor. Conclusion: Ophthalmological examination was useful in the diagnosis of shaken baby syndrome and should therefore be performed in little children in whom child abuse is suspected (AU)

El uso de válvulas de Ahmed en el tratamiento del glaucoma refractario en niños / Use of Ahmed valves in the treatment of refractory glaucoma in children

Objetivo: evaluar la efectividad del implante de la válvula de Ahmed (VA) en niños desde los 2 años de vida hasta los 16 años de edad, que presentaron glaucoma refractario al tratamiento quirúrgico convencional, atendidos en el Hospital Garrahan durante el periodo comprendido entre el 1 de enero de 2006 hasta el 30 de Junio de 2014. Resultados: Se incluyeron 16 ojos de 13 pacientes con un total de 17 VA colocadas. La presión intraocular (PIO) promedio de las tres últimas tomas antes de la colocación de la VA fue de 25,38 mm Hg con un rango de 19,33 mm Hg a 39,33 mm Hg. Todos estos pacientes se encontraban con tratamiento farmacológico tópico y nueve pacientes (69,2%) estaban medicados con acetazolamida vía oral. Trece ojos de 16 incluidos en este trabajo (81,25%) recibieron más de una cirugía filtrante previa. Los 3 ojos (18,75%) no operados previamente eran glaucomas secundarios a uveítis; se decidió colocar el dispositivo de drenaje de primera elección. Un solo ojo (6,25%) incluido en la presente serie requirió la colocación de una segunda VA. La PIO promedio al año de cirugía de la válvula fue de 13,36 mm Hg. y en los pacientes a los que se logró seguir por cinco años la PIO se mantuvo por debajo de 20 mm Hg. Ninguno de los pacientes a los que se les colocó válvula necesitó inhibidores de la anhidrasa carbónica por vía oral. Un 64,72% de los ojos a los que se les colocó VA no necesitó medicación para regular la PIO. Del 35,28% de los 17 ojos posquirúrgicos que debieron usar medicación tópica encontramos que un ojo (5,88%) requirió una droga, cuatro ojos (23,52%) demandaron el uso de dos drogas y un solo ojo (5,88%) necesitó tres drogas tópicas para mantener su PIO menor a 20 mm Hg. Las complicaciones posquirúrgicas tardías de la colocación de VA fueron en cuatro casos (23,5%) quistes conjuntivales perivalvulares, en uno (5,88%) hubo exposición del tubo que requirió su reposicionamiento, un caso (5,88%) presentó alteraciones de la motilidad ocular y otro (5,88%) desprendimiento de retina. Conclusión: La colocación de la VA es efectiva para el tratamiento del glaucoma infantil refractario porque se consiguió una disminución del 100% de la presión intraocular, con y sin medicación coadyuvante en los diversos controles de nuestro grupo de pacientes estudiados. El 64,7% de los pacientes no necesitó medicación tópica posterior a la cirugía y ninguno de ellos necesitó tratamiento con medicación sistémica para regular la PIO (AU)

Objective: To assess the effectiveness of Ahmed valve (AV) implantation in children between 2 and 16 years of age with glaucoma refractory to conventional surgical treatment seen at the Garrahan Hospital from January 1, 2006 to June 30, 2014. Results: 16 eyes of 13 patients were included of a total of 17 AV implanted. Mean intraocular pressure (IOP) of the last three taken before AV placement was 25.38 mm Hg with a range of 19.33 mm Hg to 39.33 mm Hg. All the patients were receiving topical drug treatment and nine (69.2%) received oral acetazolamide. Thirteen of 16 eyes included in the study (81.25%) underwent more than one previous filtration surgery. The three eyes (18.75%) that did not undergo surgery had glaucoma secondary to uveitis; a first-choice drainage device was placed. Only one eye (6.25%) included in this series required placement of a second AV. Mean IOP one year after implantation of the valve was 13.36 mm Hg and in those patients followed-up for five years IOP remained below 20 mm Hg. None of patients in whom an AV was implanted needed oral carbonic anhydrase inhibitors. Overall, 64.72% of the eyes with an AV implant did not need medication to regulate IOP. Postsurgically, of 35.28% of 17 eyes that needed topical medication, we found that one eye (5,88%) required one drug, four eyes (23.52%) needed two drugs, and only one eye (5.88%) needed three drugs to maintain IOP below 20 mm Hg. Late surgical complications of AV placement were perivalvular conjunctival cysts in four cases (23.5%), exposition of the tube that needed repositioning in one case (5.88%), alterations of ocular movements in one (5.88%), and retinal detachment in another (5.88%). Conclusion: AV implantation is effective in the treatment of refractory childhood glaucoma as it decreases IOP by 100% with or without coadjuvant medication in the follow-up of our patients. Of all patients, 64.7% did not need topical medication after surgery and none of the patients needed systemic medication to regulate IOP (AU)

[Retinopathy of prematurity in the XXI century in a developing country: an emergency that should be resolved]. / La retinopatía del prematuro en el siglo XXI en un país en desarrollo: una urgencia que debe ser resuelta.

INTRODUCTION: Retinopathy of prematurity (ROP) is the first cause of childhood blindness in Argentina and is reaching epidemic proportions. To gain greater insight into the issues involved in this epidemic, we analyzed the characteristics of the infants referred for treatment of very severe ROP to the Dr. Juan P. Garrahan Pediatric Hospital, located in the city of Buenos Aires. METHODS: We performed a retrospective study of patients referred for ROP from 01/01/96 to 12/31/03. Very severe ROP was defined as threshold disease or worse in both eyes, plus disease in zones 1 or 2, or Rush disease. Unusual patients were defined as those with very severe ROP with a gestational age (GA) of > 31 weeks or birth weight (BW) > 1500 g. Patients were divided into two groups according to the distance between the referring center and our hospital: group A consisted of those from nearby areas ( 70 km from the hospital. RESULTS: During the study period, the number of cases progressively increased while the proportion of "unusual" patients slightly decreased. The total number of infants referred was 809 and 14 % were unusual patients; 55 % had very severe ROP, 89 % were treated, and 7 % were referred too late and lost the opportunity for treatment. Group B comprised 61 % of the infants referred. Very severe ROP was present in 53 % of patients in group A and in 56 % of those in group B. The mean GA was 29.5 +/- 3.1 weeks in group A and 30.8 +/- 2.6 in group B (p < 0.001); 90.5 % of the patients in group A and 64 % of those in group B required mechanical ventilation (p < 0.001). No predictors for very severe ROP were found in multifactorial analysis. CONCLUSIONS: We report a childhood epidemic of blindness in which the prevalence of very severe ROP increased during the study period and the disease occurred in large, fairly mature newborns. The differences found in relation to the referring center and the lack of correlation between very severe ROP and prematurity or the use of mechanical ventilation suggests substantial disparity in healthcare, which should be taken into account when planning prevention strategies.

La retinopatía del prematuro en el siglo XXI en un país en desarrollo: una urgencia que debe ser resuelta / Retinopathy of prematurity in the XXI century in a developing country: an emergency that should be resolved

Introducción: La retinopatía del prematuro (RdP) es la primera causa de ceguera infantil en Argentina, de alcances epidémicos. Con el objetivo de mejorar el conocimiento de factores involucrados en esta epidemia, analizamos las características de los niños derivados para tratamiento de RdP severa al Hospital de Pediatría Juan P. Garrahan, ubicado en la ciudad de Buenos Aires. Métodos: Revisión retrospectiva de pacientes admitidos por RdP entre el 1 de enero de 1996 y el 31 de diciembre de 2003. RdP severa = enfermedad umbral en ambos ojos o peor, enfermedad plus en zona 1 o 2, o enfermedad de Rush. Pacientes “inusuales” son definidos aquéllos con RdP grave y edad gestacional (EG) > 31 semanas o peso al nacer (PN) > 1.500 g. Los pacientes se dividieron en 2 grupos según distancia de procedencia en relación a nuestro hospital: grupo A de procedencia cercana (≤ 70 km) y grupo B amás de 70 km. Resultados: Durante el período de estudio hubo un aumento progresivo del número de casos, con escasa reducción de casos “inusuales”. El total de niños referidos fue de 809, con 14% de casos “inusuales”; 55 % presentó formas severas, 89% recibió tratamiento y 7% perdió oportunidad por derivación tardía. El 61 % perteneció al grupo B; la incidencia de RdP severa fue de 53 % en A y 56 % en B. La media de EG fue 29,5 +/- 3,1 semanas en el grupo A y de 30,8 +/- 2,6 en el B (p < 0,001); 90,5 % en A y 64 % en B requirió asistencia ventilatoria mecánica (p < 0,001). No se encontraron factores predictivos de RdP grave en el análisis multivariable. Conclusiones: En esta epidemia de ceguera durante la niñez, la prevalencia de formas severas de RdP aumentó durante los años del estudio y la enfermedad ocurre aún en prematuros cercanos al término. Las diferencias según el lugar de procedencia y la falta de asociación de RdP grave con inmadurez o uso de ventilación mecánica, sugieren una importante disparidad en los cuidados de salud que debería tomarse en cuenta al planificar estrategias de prevención

Introduction Retinopathy of prematurity (ROP) is the first cause of childhood blindness in Argentina and is reaching epidemic proportions. To gain greater insight into the issues involved in this epidemic, we analyzed the characteristics of the infants referred for treatment of very severe ROP to the Dr. Juan P. Garrahan Pediatric Hospital, located in the city of Buenos Aires. Methods We performed a retrospective study of patients referred for ROP from 01/01/96 to 12/31/03. Very severe ROP was defined as threshold disease or worse in both eyes, plus disease in zones 1 or 2, or Rush disease. Unusual patients were defined as those with very severe ROP with a gestational age (GA) of > 31 weeks or birth weight (BW) > 1500 g. Patients were divided into two groups according to the distance between the referring center and our hospital: group A consisted of those from nearby areas (≤ 70 km) and group B comprised those from areas > 70 km from the hospital. Results During the study period, the number of cases progressively increased while the proportion of “unusual” patients slightly decreased. The total number of infants referred was 809 and 14 % were unusual patients; 55 % had very severe ROP, 89% were treated, and 7% were referred too late and lost the opportunity for treatment. Group B comprised 61 % of the infants referred. Very severe ROP was present in 53 % of patients in group A and in 56 % of those in group B. The mean GA was 29.5 +/- 3.1 weeks in group A and 30.8 +/- 2.6 in group B (p < 0.001); 90.5% of the patients in group A and 64 % of those in group B required mechanical ventilation (p < 0.001). No predictors for very severe ROP were found in multifactorial analysis. Conclusions We report a childhood epidemic of blindness in which the prevalence of very severe ROP increased during the study period and the disease occurred in large, fairly mature newborns. The differences found in relation to the referring center and the lack of correlation between very severe ROP and prematurity or the use of mechanical ventilation suggests substantial disparity in healthcare, which should be taken into account when planning prevention strategies

Cognitive and performance-based treatments for panic attacks in people with varying degrees of agoraphobic disability.

Compared the effectiveness of cognitive therapy and performance-based exposure as treatments for panic attacks. Subjects were 48 panicky individuals selected without regard to agoraphobic disability, and who varied widely in that respect. Subjects were assigned randomly to either cognitive treatment, performance-based exposure treatment, a combined cognitive/performance treatment, or a no-treatment control condition. All three treatments led to marked and enduring improvements in panic, and did not differ from one another in effectiveness, whereas the control condition produced little benefit. However, on several measures of phobia and panic-related cognitions, performance exposure was significantly more effective than cognitive therapy. Degree of agoraphobic disability had a significant bearing on panic treatment effectiveness. Whereas 94% of the low agoraphobia Ss were free of panic after treatment, only 52% of the high agoraphobia Ss became panic-free. The findings suggest that when panic treatment research excludes people with serious phobias, as it has routinely done in recent years, an overly positive estimate of panic treatment effectiveness can result.

Generalization of therapeutic changes in agoraphobia: the role of perceived self-efficacy.

Investigated the extent and mechanisms of therapeutic generalization across distinct areas of agoraphobic dysfunction. Twenty-seven severe agoraphobics were each given performance-based treatment for some phobic areas while leaving their other phobias untreated. Behavioral tests revealed that (a) the treated phobias improved significantly more than the untreated (transfer) phobias, (b) the transfer phobias improved significantly more than control phobias, and (c) the transfer benefits were highly variable within and between subjects. Analyses of possible cognitive mechanisms revealed that perceived self-efficacy accurately predicted treatment and transfer effects even when alternative factors such as previous behavior, anticipated anxiety, anticipated panic, perceived danger, and subjective anxiety were held constant. In contrast, these alternative factors lost most or all predictive value when self-efficacy was held constant. Agoraphobia thus appears to be neither a unitary entity nor a mere collection of independent phobias, but a complexly patterned problem governed largely by self-perceptions of coping efficacy.