Response to biosynthetic human growth hormone in patients with isolated type I-A and idiopatic growth hormone deficiencyResponse to treatment with biosyntetic hGH was studied in four patients with isolated GH deficiency tyoe I-A. This response was compared with that of four patients with GH deficiency of the idiopathic type submitted to the same treatment. Patients were five boys and three girls aged 5 to 1 6 years. Ail them showed yearly growth rates of idiopathic isolated GH deficiency: they grew 11.2 cm, mean, over first year of treatment. Two among four patients ciency typg I-A had severe growth retardation and peculiar facies and three of them had a family history of another brother with the same picture. All patients were treated with biosynthetic hGH (Genotropin Kabi), in subcutaneous doses of 0.5 U • kg -week for aproximately one year. Treatment results were good in those four patients with idiopathic isolated GH deficiency: •they grew 11,2 cm, mean, over the first year of treatment. Two among four patients assumed to have an isolated GH deficiency of the I-A type showed a good response, similar to that of idiopathic GH deficiency patients, whife the other two subjects grew well only through the first three months, but thereafter their growth rate decreased to that shown before treatment. Children with idipathlc GH deficiency respond well to exogenous administration of hGH, whereas patients with GH deficiency of the I-A type present a variable response to this treatment. (Key words: growth hormone deficiency type I-A, idiopathic treatment, biosynthetic hGH.)Las causas de deficiencia de hormona de crecimiento (GH) son numerosas y pueden agruparse en congenitas (frecuentemente asociadas a defectos de la h'nea media de la cara o del craneo), traumaticas (incluyendo injurias neonatales), infecciosas, inmunologicas, vasculares, tumores hipotalamo-hipoftsiarios, secundarias a irradiacion del craneo, idiopaticas y geneticas (por alteracion ge"nica en la codification de la hormona o falta de respuesta a ella por defectos de receptores) 1 .De todas las mencionadas, la mas comiin es la deficiencia idiopatica, en que no puede demostrarse lesion organica durante la vida del paciente y cuya naturaleza es un enigma, habiendose sugerido que un proceso autoinmune pudiera ser el responsable, sin que hasta el momento existan pruebas convincentes de ello. El estudio retrospectivo de la historia de los nifios con deficiencia idiopatica de GH revela alta incidencia de problemas perinatales como partos en podalica, forceps, trabajo de parto prolongado o inusualmente corto. Se ha sugerido que esto pudiera causarhipopituitarismo, aunque clinicamente no se detecte asfixia o sufrimiento fetal. Por otro lado, si el sistema endocrino fetal tiene una funcion en la induction del parto, es posible que una enfermedad hipotalamica o hipofisiaria del feto contribuya a un parto anormal y morbilidad neonatal.Entre las causas geneticas de deficiencia de GH esta una deletion (u otra alteracion) del gen de la GH (hGH-N) ubicado en el brazo la...
Right lung agenesis in a newbornAgenesis of the lung is an extremely rare anomaly t has been reported as an isolated defect or in association with other congenital malformations. The case of a full term newborn girl with isolated right lung agenesis is described. She was asymptomatic, except for slight tachypnea in the first few days of life, which was associated to meconium in the amniotic fluid but not in her tracheal aspirates, Clinically there was evidence of displacement of heart's apical impulse and sounds to the right side of the thorax, which on chest X ray films looked opaque, with intercostal space retraction and mediastinal displacement to the same side. Absence of right pulmonary artery, veins and parenchima were documented by X ray and radionuclide angiography and pulmonary scintigraphy. Heart and abdominal ultrasonography showed no additional abnormalities. She was dismissed from hospital stage 12 days and her course was uneventfuil until age seven weeks, when she was admitted because of acute pneumonia which caused her death a few days later.(Key words: Lung agenesis, pulmonary artery, newborn.]
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