Déficit de hormona del crecimiento como causa de baja talla / Loss growth hormone as cause of short stature

RESUMEN Fundamento: la talla baja supone un motivo de preocupación para los padres y es una causa muy frecuente de consulta en Pediatría y en Endocrinología Pediátrica. Objetivo: presentar el caso de un adolescente con baja talla por déficit de hormona del crecimiento. Presentación del caso: paciente adolescente de 12 años de edad que acude a consulta de Endocrinología por baja talla con examen físico normal y dosificaciones de hormona del crecimiento bajas en dos estudios de estimulación (test de clonidina e hipoglucemia inducida por insulina). Conclusiones: la baja talla por déficit de hormona del crecimiento es una de las causas corregibles de este trastorno.

ABSTRACT Background: short stature is a common cause of preoccupation in parents and it's a frequent shift complaint at Pediatric Endocrinology. Objective: to show the case of a teenager who had short stature due to loss growth hormone. Case report: a 12 years-old male teenager who came to Endocrinologist because of a short stature. Physical exam was normal meanwhile hormonal lab test shown loss growth hormone on two stimulated test (clonidine test and insulin-induced hypoglycemia). Conclusions: short stature due to loss growth hormone is a latent corrigible cause of that disorder.

Influence of growth hormone replacement on neurological and psychomotor development. Case report / Influência da reposição do hormônio do crescimento no desenvolvimento neuropsicomotor. Relato de caso

ABSTRACT The height response to the use of growth hormone in short height cases has already been confirmed in the literature. The influence of the insulin-like growth factor 1 (GH-IGF1) axis components on development, function, regeneration, neuroprotection, cognition, and motor functions has been evaluated in experimental studies and in adults with central nervous system lesions. However, there is still little research on the clinical impact of hormone replacement on neurological and psychomotor development. This report presents the case of a patient with excellent weight-height recovery and, even more surprisingly, neurological and psychomotor development in response to use of growth hormone. The result strengthens the correlation between experimental and clinical findings related to cerebral plasticity response to growth hormone in children. A preterm male patient with multiple health problems during the neonatal and young infancy period, who for six years presented with a relevant deficit in growth, bone maturation, and neurological and psychomotor development. At six years of age, he had low stature (z-score −6.89), low growth rate, and low weight (z-score −7.91). He was incapable of sustaining his axial weight, had not developed fine motor skills or sphincter control, and presented with dysfunctional swallowing and language. Supplementary tests showed low IGF-11 levels, with no changes on the image of the hypothalamus-pituitary region, and bone age consistent with three-year-old children — for a chronological age of six years and one month. Growth hormone replacement therapy had a strong impact on the weight-height recovery as well as on the neurological and psychomotor development of this child.

RESUMO A resposta estatural ao uso de hormônio do crescimento na baixa estatura já está comprovada na literatura. A influência dos componentes do eixo fator de crescimento semelhante à insulina tipo 1 (GH-IGF1) sobre desenvolvimento, função, regeneração, neuroproteção, cognição e funções motoras tem sido avaliada em estudos experimentais e em adultos com lesão de sistema nervoso central. No entanto, ainda são poucas as pesquisas sobre o impacto clínico da reposição hormonal no desenvolvimento neuropsicomotor. Este relato apresenta o caso de um paciente com excelente recuperação pôndero-estatural e, de forma ainda mais surpreendente, de desenvolvimento neuropsicomotor, em resposta ao uso de hormônio do crescimento. O resultado observado fortalece a correlação entre achados experimentais e clínicos, no que diz respeito à resposta da plasticidade cerebral ao hormônio do crescimento em crianças. Paciente do sexo masculino nasceu pré-termo com múltiplos agravos no período neonatal e de lactente jovem, e que, por 6 anos, apresentou deficit relevante do crescimento, na maturação óssea e do desenvolvimento neuropsicomotor. Aos 6 anos de idade, apresentava baixa estatura (escore Z de −6,89), baixa velocidade de crescimento e baixo peso (escore Z de −7,91). Era incapaz de sustentar o peso axial, não tinha desenvolvido habilidade motora fina e nem controle esfincteriano, e apresentava também disfunção na deglutição e na linguagem. Exames complementares mostraram IGF1 baixo, sem alterações na imagem da região hipotálamo-hipofisária e idade óssea compatível com 3 anos — para a idade cronológica de 6 anos e 1 mês. A terapia de reposição com hormônio do crescimento promoveu forte impacto na recuperação pôndero-estatural e também do desenvolvimento neuropsicomotor desta criança.

Diagnostic value of MRI in pituitary stalk interruption syndrome / 中华放射学杂志

Objective To study characteristic findings and the diagnostic value of MRI in pituitary stalk interruption syndrome (PSIS). Methods Twenty-one patients with PSIS were included. Small field of view (FOV) MR1 scanning and clinic hormone detection were performed in all patients. Moreover, fluid attenuated inversion recovery (FLAIR)T,1 WI with fat-suppression sequence was performed in 8 cases. The appearance on FLAIR T1 WI and T2 WI were recorded. The shape of pituitary stalk and antehypophysis, and the signal intensity of posthypophysis were analyzed simultaneously. Results Growth hormone deficiency (GHD) was confirmed by clinic hormone detection in all 21 cases. The level of basal GH varied from 0. 03 μg,/L to 1.50 μg/L. The peak value under GH provocation was from 0. 13 μg/L to 4. 14 μg/L, and the GH was absolute default in all patients. Seventeen cases of them were combined pituitary hormone deficiency (CPHD), and 4 cases were isolated growth hormone deficiency (IGHD). The height of antepituitary was in the range from 1 mm to 3 mm, and the average value was (1.9±1.2) mm. Pituitary stalk was absent in 18 cases and showed as linear and discontinuous stalk in the other 3 cases. The high signal intensity was invisible in normal position in all cases, the high signal intensity spot in the region of infundibular recess of the third ventricle was shown in 19 cases, while it could not be found anywhere in 2 patients with diabetes insipidus. Conclusion PSIS often shows characteristic appearance on MRI, and a definite diagnosis can be made by using MRI combined with clinic hormone detection.

Effect of recombinant human growth hormone on final adult height in children with growth hormone deficiency / 中华内分泌代谢杂志

0.05).Conclusion GH improves FAH of children with GHD.Height at the initiation of puberty is the most significant determining factor for the long-term efficacy.Hence,it is important that the diagnosis should be made and treatment be initiated as early as possible to afford children with GHD the opportunity to make up much of their height deficit before puberty.Adequate dosage of GH should be used for the children taking initial treatment at puberty to attain satisfactory FAH.