RESUMEN
UNLABELLED: AIMS OF REVIEW: the intent of the current manuscript is to critically review the studies on pituitary gland dysfunction in early childhood following traumatic brain injury (TBI), in comparison with those in adults. Search of the literature: The MEDLINE database was accessed through PubMed in April 2015. Results were restricted to the past 15 years and English language of articles. Both transient and permanent hypopituitarisms are not uncommon after TBI. Early after the TBI, pituitary dysfunction/s differ than those occurring after few weeks and months. Growth hormone deficiency (GHD) and alterations in puberty are the most common. After the one to more years of TBI, pituitary dysfunction tends to improve in some patients but may deteriorate in others. GH deficiency as well as Hypogonadism and thyroid dysfunction are the most common permanent lesions. Many of the symptoms of these endocrine defects can pass unnoticed because of the psychomotor defects associated with the TBI like depression and apathy. Unfortunately pituitary dysfunction appear to negatively affect psycho-neuro-motor recovery as well as growth and pubertal development of children and adolescents after TBI. Therefore, the current review highlights the importance of closely following patients, especially children and adolescents for growth and other symptoms and signs suggestive of endocrine dysfunction. In addition, all should be screened serially for possible endocrine disturbances early after the TBI as well as few months to a year after the injury. Risk factors for pituitary dysfunction after TBI include relatively serious TBI (Glasgow Coma Scale score < 10 and MRI showing damage to the hypothalamic pituitary area), diffuse brain swelling and the occurrence of hypotensive and/or hypoxic episodes. IN CONCLUSION: There is a considerable risk of developing pituitary dysfunction after TBI in children and adolescents. These patients should be clinically followed and screened for these abnormalities according to an agreed protocol of investigations. Further multicenter and multidisciplinary prospective studies are required to explore in details the occurrence of permanent pituitary dysfunction after TBI in larger numbers of children with TBI. This requires considerable organisation and communication between many disciplines such as neurosurgery, neurology, endocrinology, rehabilitation and developmental paediatrics.
Asunto(s)
Lesiones Encefálicas/fisiopatología , Enfermedades de la Hipófisis/fisiopatología , Hipófisis/fisiopatología , Adolescente , Adulto , Lesiones Encefálicas/complicaciones , Lesiones Encefálicas/diagnóstico por imagen , Niño , Preescolar , Femenino , Escala de Coma de Glasgow , Humanos , Hipopituitarismo/diagnóstico por imagen , Hipopituitarismo/fisiopatología , Hipotálamo/diagnóstico por imagen , Hipotálamo/fisiopatología , Masculino , Enfermedades de la Hipófisis/diagnóstico por imagen , Enfermedades de la Hipófisis/etiología , Radiografía , Maduración SexualRESUMEN
Most of the endocrine complications in thalassaemia are attributable to iron overload which may be the result of economic circumstances (expense of the chelation therapy), late onset of chelation therapy or poor compliance with the iron chelation therapy. The major difficulties reported by hematologists or pediatric endocrinologists experienced in thalassaemias or thalassaemia syndromes in following growth disorders and endocrine complications were: lack of familiarity with medical treatment of endocrine complications (40%), interpretation of endocrine tests (30%), costs (65%), absence of paediatric endocrinologist for consultation on growth disorders and endocrine complications (27%), facilities (27%), other (e.g. lack of collaboration and on-time consultation between thalassaemic Centers supervised by hematologists and endocrinologists) (17%). Because any progress we make in research into growth disorders and endocrine complications in thalassaemia should be passed on to all those suffering from it, guaranteeing them the same therapeutic benefits and the same quality of life, on the 8th of May, 2009 in Ferrara (Italy), the International Network on Endocrine Complications in Thalassemia (I-CET) was founded. The I-CET group is planning to conduct, in Ferrara in May 2012, a workshop, "MRI and Endocrine Complications in Thalassaemia", and in Doha (Qatar) in September 2012, a 3-day intensive course entitled, "Growth disorders and Endocrine Complications in Thalassaemia", to provide interested pediatricians, physicians and hematologists from all over the world with an in-depth approach to the diagnosis and management of growth and endocrine disorders in thalassaemic patients.
Asunto(s)
Enfermedades del Sistema Endocrino/complicaciones , Hierro , Talasemia/complicaciones , Transfusión Sanguínea , Terapia por Quelación , Enfermedades del Sistema Endocrino/patología , Enfermedades del Sistema Endocrino/prevención & control , Humanos , Hierro/sangre , Hierro/toxicidad , Talasemia/epidemiología , Talasemia/patologíaRESUMEN
Patients with beta-thalassemia major (beta-thalassemia) frequently have bone disorders of multifactorial etiology. We attempted to analyze the relationship between the bone mineral density ([BMD] measured by dual-photon absorptiometry) and auxanologic parameters, degree of siderosis, function of the growth hormone (GH)/insulin-like growth factor-I (IGF-I)/IGF-binding protein-3 (IGFBP3) axis, calcium-phosphate balance, parathyroid hormone (PTH), and cytokines (interleukin-1beta [IL-1] and tumor necrosis factor-alpha [TNF-alpha]) in 30 prepubertal children with beta-thalassemia major and 15 age-matched children with constitutional short stature (CSS), who have normal glucose tolerance and thyroid function. Children with beta-thalassemia had a significantly decreased BMD and mean BMD% for age and sex (0.75+/-0.24 g/cm2 and 71%+/-10%, respectively) versus children with CSS (1.06+/-0.3 g/cm2 and 92%+/-7%, respectively). Thalassemic patients had significantly lower circulating concentrations of IGF-I and IGFBP3 (49+/-21 ng/mL and 1.2+/-0.25 mg/L, respectively) compared with control children (153+/-42 ng/mL and 2.1+/-0.37 mg/L, respectively). The GH response to provocation by clonidine and glucagon was defective (peak GH < 7 microg/L) in 12 of the 30 thalassemic children. Serum concentrations of IL-1beta and TNF-alpha did not differ among the two study groups. Hypocalcemia was detected in five of the 30 thalassemic patients: hypoparathyroidism was diagnosed in two of the five and rickets in the other three. BMD was highly correlated with the circulating concentrations of IGF-I and IGFBP3, as well as with the auxanologic parameters (age, weight, height, height standard deviation score [HSDS], and body mass index [BMI]). It is suggested that increasing the circulating IGF-I concentration through aggressive nutritional therapy and/or GH/IGF-I therapy with supplementation with vitamin D and/or calcium might improve bone growth and mineralization and prevent the development of osteoporosis and consequent fractures in these patients. Such therapy requires blinded controlled trials.
Asunto(s)
Densidad Ósea/fisiología , Talasemia beta/fisiopatología , Absorciometría de Fotón , Adolescente , Hormona Adrenocorticotrópica/administración & dosificación , Hormona Adrenocorticotrópica/sangre , Hormona Adrenocorticotrópica/farmacología , Factores de Edad , Fosfatasa Alcalina/sangre , Fosfatasa Alcalina/efectos de los fármacos , Antropometría , Antihipertensivos/administración & dosificación , Antihipertensivos/farmacología , Estatura/efectos de los fármacos , Estatura/fisiología , Índice de Masa Corporal , Peso Corporal/efectos de los fármacos , Peso Corporal/fisiología , Densidad Ósea/efectos de los fármacos , Huesos/efectos de los fármacos , Huesos/fisiología , Calcifediol/sangre , Calcio/sangre , Estudios de Casos y Controles , Niño , Clonidina/administración & dosificación , Clonidina/farmacología , Interpretación Estadística de Datos , Relación Dosis-Respuesta a Droga , Femenino , Fármacos Gastrointestinales/administración & dosificación , Fármacos Gastrointestinales/farmacología , Glucagón/administración & dosificación , Glucagón/farmacología , Crecimiento/efectos de los fármacos , Crecimiento/fisiología , Hormona del Crecimiento/sangre , Hormona del Crecimiento/fisiología , Hormonas/sangre , Humanos , Hidrocortisona/sangre , Hipocalcemia/sangre , Hipocalcemia/fisiopatología , Hipotiroidismo/sangre , Hipotiroidismo/fisiopatología , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina/sangre , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina/efectos de los fármacos , Factor I del Crecimiento Similar a la Insulina/efectos de los fármacos , Factor I del Crecimiento Similar a la Insulina/metabolismo , Interleucina-1/sangre , Hierro/sangre , MasculinoRESUMEN
We evaluated the radiological, biochemical and growth hormone (GH)/insulin-like growth factor-I (IGF-I) changes in 10 children with severe protein-energy malnutrition (PEM) who had rachitic manifestations (group 1), 10 children with severe PEM without clinical signs of rickets (group 2), and 10 children with normal body weight-for-length and -age, suffering from vitamin-D-deficiency with signs of florid rickets (group 3) and 10 normal age-matched children (group 4). Serum calcium (Ca2+), phosphorus (PO4), and albumin concentrations were markedly decreased in the two groups with PEM. Malnourished children with rickets had significantly higher serum alkaline phosphatase (ALP) concentrations compared to the malnourished group without rachitic manifestations. Radiological evaluation of the two groups who had rachitic manifestations revealed demineralization of long bones, thinning of the bony cortex, increased formation of osteoid tissue, and metaphyseal changes including cupping, fraying, and flaring. The incidence of these radiological findings did not differ among the well-nourished and the malnourished groups with clinical signs of rickets. However, the incidence of fracture of the shaft was higher (40 per cent) in the malnourished group compared to the well-nourished group (10 per cent) with rickets. In the malnourished group without clinical evidence of rickets, demineralization and cortical thinning was detected in 40 per cent without significant metaphyseal changes. Basal concentrations of GH and peak GH response to clonidine were significantly elevated and IGF-I concentrations were significantly depressed in the malnourished groups v. the other two groups. There were no significant differences in the fasting and the clonidine provoked GH levels or IGF-I concentrations between the rachitic children (group 3) and the normal children. These data suggest that in rachitic children there is not a major role for circulating GH (and by implication IGF-I) on bone mineralization. However, during malnutrition decreased IGF-I production can slow or stop epiphyseal growth and might contribute to the demineralization of the cortex of long bones.
Asunto(s)
Desnutrición Proteico-Calórica/fisiopatología , Raquitismo/sangre , Raquitismo/fisiopatología , Huesos/diagnóstico por imagen , Huesos/metabolismo , Huesos/fisiopatología , Estudios de Casos y Controles , Egipto , Hormona del Crecimiento/sangre , Humanos , Lactante , Factor I del Crecimiento Similar a la Insulina/metabolismo , Radiografía , Raquitismo/diagnóstico por imagenRESUMEN
Serum growth hormone (GH), cortisol, free thyroxine (FT4), thyroid-stimulating hormone (TSH), and insulin like growth factor I (IGF-I) concentrations were measured in 15 children with sickle cell disease (SCD) together with their heights < 5th percentile for age and gender, and in 15 healthy age-matched children who had normal variant short stature (NVSS). GH response to an oral dose of clonidine (0.15 mg/m2) and cortisol response to ACTH stimulation were determined in the two groups. Children with SCD had significantly lower serum concentrations of IGF-I and decreased GH response to stimulation. Eight out of the 15 children with SCD did not mount an appropriate GH response to clonidine provocation (> 10 micrograms/l). CT scanning of the hypothalamic-pituitary area in those eight children with SCD revealed a partial or complete empty sella in all of them. It appears that defective GH release, and consequently low IGF-I production and slow growth velocity in children with SCD might be secondary to hypoxic-vascular insults to their hypothalamic-pituitary axis during one or more of the sickling episodes.
Asunto(s)
Clonidina , Hormona del Crecimiento/sangre , Crecimiento , Enfermedad de la Hemoglobina SC/sangre , Factor I del Crecimiento Similar a la Insulina/metabolismo , Tiroxina/sangre , Estudios de Casos y Controles , Niño , Femenino , Enfermedad de la Hemoglobina SC/diagnóstico por imagen , Enfermedad de la Hemoglobina SC/patología , Humanos , Hipotálamo/diagnóstico por imagen , Masculino , Omán , Hipófisis/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
We evaluated arginine-induced insulin and growth hormone (GH) secretion in ten children with vitamin D deficiency rickets and compared these values with those of eight age-matched control children. All rachitic children had biochemical (increased serum alkaline phosphatase activity and decreased calcium x phosphate product) and clinical evidence for rickets. After an intravenous infusion of arginine-HCl (10% solution, 0.5 g/kg), blood samples were obtained for the measurement of serum insulin and GH concentrations. The mean insulin level 30 min after the start of the infusion was 22.2 +/- 17.1 microU/ml for the rachitic children. This value is significantly below that for the normal children, 63.4 +/- 38.7 microU/ml (p = 0.004). Neither the fasting insulin level nor any others after the arginine infusion differed significantly from those for the control children. There were no significant differences in the fasting or the arginine-stimulated GH levels between the rachitic and control children. The concentrations of insulin-like growth factors did not differ between the two groups.