RESUMO
TITLE: Linfoma B intravascular hipotalamico en una paciente inmunocompetente.
Assuntos
Hipotálamo/irrigação sanguínea , Linfoma de Células B/patologia , Neoplasias Vasculares/patologia , Idoso , Terapia Combinada , Confusão/etiologia , Irradiação Craniana , Feminino , Glucocorticoides/uso terapêutico , Humanos , Hipotálamo/diagnóstico por imagem , Hipotálamo/patologia , Hipotireoidismo/etiologia , Imunocompetência , Linfoma de Células B/complicações , Linfoma de Células B/diagnóstico por imagem , Linfoma de Células B/terapia , Invasividade Neoplásica , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Transtornos do Sono do Ritmo Circadiano/etiologia , Neoplasias Vasculares/complicações , Neoplasias Vasculares/diagnóstico por imagem , Neoplasias Vasculares/terapia , Vasopressinas/deficiência , Redução de PesoRESUMO
BACKGROUND: Children initially diagnosed with isolated GH deficiency (IGHD) have a variable rate to progress to combined pituitary hormone deficiency (CPHD) during follow-up. OBJECTIVE: To evaluate the development of CPHD in a group of childhood-onset IGHD followed at a single tertiary center over a long period of time. PATIENTS AND METHODS: We retrospectively analyzed data from 83 patients initially diagnosed as IGHD with a mean follow-up of 15.2 years. The Kaplan-Meier method and Cox regression analysis was used to estimate the temporal progression and to identify risk factors to development of CPHD over time. RESULTS: From 83 patients initially with IGHD, 37 (45%) developed CPHD after a median time of follow up of 5.4 years (range from 1.2 to 21 years). LH and FSH deficiencies were the most common pituitary hormone (38%) deficiencies developed followed by TSH (31%), ACTH (12%) and ADH deficiency (5%). ADH deficiency (3.1 ± 1 years from GHD diagnosis) presented earlier and ACTH deficiency (9.3 ± 3.5 years) presented later during follow up compared to LH/FSH (8.3 ± 4 years) and TSH (7.5 ± 5.6 years) deficiencies. In a Cox regression model, pituitary stalk abnormalities was the strongest risk factor for the development of CPHD (hazard ratio of 3.28; p = 0.002). CONCLUSION: Our study indicated a high frequency of development of CPHD in patients initially diagnosed as IGHD at childhood. Half of our patients with IGHD developed the second hormone deficiency after 5 years of diagnosis, reinforcing the need for lifelong monitoring of pituitary function in these patients.
Assuntos
Hormônio Adrenocorticotrópico/deficiência , Nanismo Hipofisário/epidemiologia , Hormônio Foliculoestimulante/deficiência , Hormônio do Crescimento Humano/deficiência , Hipopituitarismo/epidemiologia , Hormônio Luteinizante/deficiência , Tireotropina/deficiência , Vasopressinas/deficiência , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Progressão da Doença , Feminino , Seguimentos , Humanos , Hipopituitarismo/patologia , Hipotálamo/patologia , Estimativa de Kaplan-Meier , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Hipófise/patologia , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Tempo , Adulto JovemRESUMO
The septic shock is characterized by decrease in median arterial pressure; many researchers have been related a deficiency in vasopressin release during the septic shock. Lipopolysaccharide administration is used to induce septic shock model in animals. We investigated the heme-oxygenase (HO) inhibition during the endotoxemic shock-like conditions. The LPS administration induced a significant decrease in MAP (-15.4 +/- 1.2 mmHg at second hour, -25.8 +/- 8.7 mmHg at fourth hour, and -22.3 +/- 8.6 mmHg at sixth hour) with a concomitant increase in heart rate (486.3 +/- 55.0, 531.8 +/- 53.8, and 510.0 +/- 55.3 bpm, respectively), a significant decrease in diuresis (from 1.1 +/- 0.7 to 0.4 +/- 0.3/100g body weight at fourth hour), and a transitory decrease in body temperature (from 37.0 +/- 0.5 to 35.4 +/- 0.8 degrees C at second hour). An increase in plasma arginine vasopressin (AVP) concentration (from 3.2 +/- 0.9 to 19.0 +/- 5.7 pg/mL at the first hour) occurred in these animals and was present for 2 h after LPS administration, returning close to basal levels thereafter and remaining unchanged until the end of the experiment. When LPS was combined with the i.c.v. administration of HO inhibitor, we observed a sustained increase in plasma AVP concentration, attenuation in the drop of MAP, and increase in antidiuresis induced by LPS treatment. These data suggest that central HO pathway may activate a control mechanism that attenuates AVP secretion during endotoxemia and may consequently regulate the MAP and diuretic output.
Assuntos
Heme Oxigenase (Desciclizante)/metabolismo , Choque Séptico/metabolismo , Vasopressinas/deficiência , Animais , Arginina Vasopressina/sangue , Arginina Vasopressina/efeitos dos fármacos , Pressão Sanguínea/efeitos dos fármacos , Deuteroporfirinas/administração & dosagem , Deuteroporfirinas/farmacologia , Diurese/efeitos dos fármacos , Inibidores Enzimáticos/farmacologia , Frequência Cardíaca/efeitos dos fármacos , Heme Oxigenase (Desciclizante)/antagonistas & inibidores , Hipotensão/etiologia , Lipopolissacarídeos , Masculino , Ratos , Ratos Wistar , Choque Séptico/complicações , Choque Séptico/urina , Vasopressinas/metabolismoRESUMO
L-3H-fucose was injected into the lateral cerebral ventricle of vasopressin-deficient Brattleboro and control Long-Evans rats which were subsequently killed at several time intervals after the injection. The hypothalamus and the neurohypophysis were processed for light- and electronmicroscopic radioautography. Other complementary experiments using immunocytochemical and enzyme-histochemical techniques were also undertaken. L-3H-fucose was incorporated into newly synthesized glycoproteins in the Golgi apparatus of supraoptic and paraventricular neurons, and later on labelled glycoproteins migrated to lysosomes and the plasma membrane surrounding the perikaryon. The Golgi apparatus of the vasopressin-deficient neurons remained heavily labelled as long as 3 days after injection, in sharp contrast with the normal neurons in which there was a remarkable decrease of label in the Golgi region between 4 and 24 h after the isotope administration. Labelled glycoproteins also migrated to the neurohypophysis and were mainly found in the axonal plasma membrane, vesicles and axoplasm. The renewal of glycoproteins in the neurohypophysis of Brattleboro rats was faster than in the normal rats and this was attributed to the lack of formation of products which are normally packaged in secretory granules in the perikaryon and released at the axon terminal in the neurohypophysis. Colchicine caused a disturbance in the topography of the organelles of the perikaryon and the most striking features were the displacement of Golgi stacks to the periphery of the perikaryon and an accumulation of mitochondria in this neuronal region. No secretory granules were observed in the vasopressin-deficient neurons of untreated or colchicine-treated Brattleboro rats. By contrast, secretory granules (most of them labelled with 3H-fucose) were concentrated in the perikaryon of colchicine-treated Long-Evans rats. In these rats, colchicine caused a severe block in the migration of 3H-fucose-labelled glycoproteins to the neurohypophysis, but this did not occur in the Brattleboro rats. The results of the experiments were interpreted in the light of the genetic defect known to occur in Brattleboro rats which causes the inability to produce vasopressin and also remarkable morphological and physiological changes in the affected neurons.
Assuntos
Hidrolases Anidrido Ácido , Glicoproteínas/biossíntese , Hipotálamo/metabolismo , Neuro-Hipófise/metabolismo , Animais , Autorradiografia , Colchicina/farmacologia , Feminino , Fucose , Hipotálamo/ultraestrutura , Monoéster Fosfórico Hidrolases/metabolismo , Neuro-Hipófise/ultraestrutura , Ratos , Ratos Brattleboro , Vasopressinas/deficiênciaRESUMO
We examined plasma arginine-vasopressin concentrations by radioimmunoassay in two brothers, aged 6 and 7.5 years, with familial central diabetes insipidus inherited as an autosomal dominant trait. Plasma AVP was measured in relation to increasing plasma osmolality induced by water deprivation and hypertonic saline infusion. The brother with the more severe urinary concentrating defect had no detectable AVP when his plasma osmolality was as high as 306 mOsm/kg; the other brother had detectable but clearly subnormal AVP concentrations. The one brother tested had an apparently normal end-organ response to exogenous vasopressin. Chlorpropamide had a significant antidiuretic effect in the brother with detectable AVP levels, and a lesser effect in the other brother . Our findings suggest that intrafamilial variation in the severity of congenital DI is related to the degree of vasopressin deficiency.
Assuntos
Diabetes Insípido/genética , Vasopressinas/sangue , Arginina Vasopressina/sangue , Criança , Clorpropamida/farmacologia , Diabetes Insípido/sangue , Diurese/efeitos dos fármacos , Genes Dominantes , Humanos , Masculino , Concentração Osmolar , Linhagem , Radioimunoensaio , Vasopressinas/deficiênciaRESUMO
The usual treatment for recurrent syndrome of inappropriate secretion of antidiuretic hormone has been fluid restriction. Recently White and Fetner described an adult with SIADH successfully managed with lithium carbonate. Described here is a child with recurrent SIADH who was diagnosed as having an acute hyponatremic episode and who then relapsed twice in a two-month period while chronic fluid restriction was attempted. He has now been maintained on 300 mg/day of lithium carbonate and is asymptomatic with normal serum sodium concentration and urine osmolalities. Lithium appears to be effective in the management of recurrent SIADH and may allow control in a patient who cannot comply with long-term fluid restriction.