Adherence to treatment in children with growth hormone deficiency, small for gestational age and Turner syndrome in Mexico: results of the Easypod™ connect observational study (ECOS).

BACKGROUND: Assessing adherence to growth hormone (GH) is challenging. The Easypod™ connect device delivers pre-set doses of recombinant human GH (r-hGH) and stores a digital record of adherence that can be shared with healthcare provider. We assessed adherence to r-hGH delivered with Easypod™ according to the approved pediatric indications for r-hGH: growth hormone deficiency (GHD), born small for gestational age (SGA) who failed to show catch-up growth and Turner syndrome (TS). METHODS: ECOS (NCT01555528) was a multicenter (24 countries), 5-year, longitudinal, observational study, which aimed to evaluate country-specific adherence to r-hGH therapy prescribed via the Easypod™ electronic injection device. The primary endpoint was yearly adherence. Secondary endpoints were height velocity, height velocity standard deviation scores (SDS), height, height SDS and IGF-1 concentrations. Clinical and auxological data were obtained from medical records and adherence from Easypod™ logs. RESULTS: This study included 147 Easypod™-naïve Mexican children assessed during 3 years (mean age: 9.96 ± 3.41 years, 56.8% boys, mean height SDS at baseline: - 2.17 ± 0.97): 118 with GHD, 24 SGA and 5 with TS. A total of 105 (71.4%) patients were GH naïve. Overall median adherence was > 90% over the first year of treatment and > 80% at 3 years. Adherence was not different by r-hGH indication or between GH-naïve or experienced patients. At 1-year follow-up, mean change in height SDS was 0.57 ± 0.34, whereas mean height velocity SDS was 2.85 ± 2.51. In all, 84.7% patients had normal IGF-1 concentrations at 1-year follow-up. Adherence was associated with change in height SDS (r = 0.239, p = 0.005) and height velocity SDS (r = 0.194, p = 0.027). CONCLUSION: Adherence rates with the Easypod™ device are high and maintained over time in GHD, SGA and TS Easypod™-naïve Mexican patients. High adherence is associated with better outcomes. Easypod™ assists physicians in monitoring adherence to r-hGH.

Ocular neuromyotonia: an unusual case after radiotheraphy for nasopharyngeal carcinoma.

Ocular neuromyotonia (ONM) is an infrequent disorder characterised by recurrent episodes of binocular diplopia caused by paroxysmal contraction of one or several extraocular muscles innervated by the same cranial nerve. It can be triggered spontaneously or caused by prolonged contraction of specific eye muscle(s) and is usually related to a local intracranial radiotherapy antecedent. We report the case of a 46-year-old woman who developed intermittent episodes of binocular diplopia eight years after radiotherapy for a nasopharyngeal carcinoma. After a complete neuro-ophthalmic assessment we diagnosed the case as an abducens nerve neuromyotonia. Although it is infrequent, radiotherapy to the nasopharynx is a possible cause of ONM, due to the proximity to the base of the skull and extraocular motor nerve pathways, especially that of the VI cranial nerve, as is the case presented in this article, about a patient whose history is a nasopharyngeal carcinoma treated with local radiotherapy.

Neuromiotonía ocular: un infrecuente caso tras carcinoma nasofaríngeo tratado con radioterapia local / Ocular neuromyotonia: An unusual case after radiotheraphy for nasopharyngeal carcinoma

La neuromiotonía ocular es una patología poco frecuente caracterizada por episodios recurrentes de diplopía binocular ocasionada por una contracción paroxística mantenida de uno o más músculos extraoculares inervados por un mismo nervio craneal, espontáneamente o inducidos por una versión concreta mantenida en el tiempo, normalmente relacionado con un antecedente de radioterapia local intracraneal. Presentamos el caso de una mujer de 46 años que presenta episodios de diplopía binocular recurrentes, diagnosticada de neuromiotonía ocular del VI nervio craneal izquierdo a los 8 años de padecer un cáncer de cavum tratado mediante radioterapia local y en completa remisión. Aunque es poco frecuente, la radiación a nivel de cavum debe tenerse en cuenta como potencial causa de neuromiotonía ocular, por su proximidad a la base del cráneo y su estrecha relación con el trayecto de los nervios oculomotores, especialmente el VI par craneal, como el caso que se presenta en este artículo.(AU)

Ocular neuromyotonia is an infrequent disorder characterised by recurrent episodes of binocular diplopia caused by paroxysmal contraction of one or several extraocular muscles innervated by the same cranial nerve. It can be triggered spontaneously or caused by prolonged contraction of specific eye muscle(s) and is usually related to a local intracranial radiotherapy antecedent. We report the case of a 46-year-old woman who developed intermittent episodes of binocular diplopia 8 years after radiotherapy for a nasopharyngeal carcinoma. After a complete neuro-ophthalmic assessment we diagnosed the case as an abducens nerve neuromyotonia. Although it is infrequent, radiotherapy to the nasopharynx is a possible cause of ocular neuromyotonia, due to the proximity to the base of the skull and extraocular motor nerve pathways, especially that of the VI cranial nerve, as is the case presented in this article, about a patient whose history is a nasopharyngeal carcinoma treated with local radiotherapy.(AU)

[Fluoroangiographic study of the retina in healthy siblings of diabetic patients]. / Estudio fluorangiográfico de retina en hermanos sanos de pacientes diabéticos.

There are still great incognites in the etiology and physiopathology of the so called chronic complications of diabetes mellitus. There are specially many questions about the origin of the diabetic microangiopathy which is the cause of several alterations like the diabetic retinopathy. Genetic, immunological and environmental factors have been involucrated in its development. In order to study the genetic involvement, in fifteen normal brothers and sisters of known diabetics type I a retina fluorangiographic study was performed. In all the cases the fluorangiography was normal suggesting that the genetic factors must coexist with the metabolic disturbance to produce retina vessels damage.

[Diabetic ketoacidosis]. / Cetoacidosis diabética.

Is the most commun and serious complication of the diabetes mellitus in the childhood and adolescent, the current mortality rate in most tertiary hospitals for children is about 2 percent. Is the metabolic consequence of a deficiency of insulin, it can be precipitating for stress, or inappropriate knowledge of the management and in some cases of subclinical diabetes or it still has not been diagnostic. The majority of instances of diabetic ketoacidosis can be prevented by early recognition of ketosis, and on appropriate ambulatory management.