Perfil de concentración sérica de hormona de crecimiento en 24 horas en a) un niño de 7 años con gigantismo de origen hipofisario, b) un niño de 7 años con. con múltiples complicaciones cutáneas, en el contexto de gigantismo que apareció en la infancia como consecuencia de un tumor hipofisario productor de . English Spanish online dictionary Term Bank, translate words and terms with different pronunciation options. gigantism gigantismo acromegalic gigantism.
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Rivarola y Alicia Belgorosky. For this issue of Endocrinologia Pediatrica On line we have selected the following scientific publications: J Clin Endocrinol Metab.
Early menarche is associated with increased risk of cardiovascular disease in adulthood. It is unknown whether metabolic risk factors gihantismo adversely affected in girls with central precocious puberty CPP already at time of diagnosis. The objective of the study was to evaluate metabolic profiles in girls with early normal puberty EP and CPP.
gigantism — с испанского на английский
This was a combined cross-sectional and longitudinal study at a tertiary center of pediatric endocrinology. Twenty-three girls with EP or CPP and controls with normal pubertal timing were evaluated by oral glucose tolerance test, dual-energy x-ray absorptiometry scan, and fasting blood samples. Insulin and glucose levels during oral glucose tolerance test and fasting lipid levels were measured.
Early maturational timing in girls is associated with increased all-cause, cancer and cardiovascular mortality.
Term Bank – gigantismo – Spanish English Dictionary
Moreover, early age at menarche is associated with in-creased risk of obesity, hypertension, and type 2 diabetes as well as ischemic heart disease and stroke events in adulthood. Girls with central precocious puberty have increased adiposity at time of diagnosis. In addition, inhibition of pubertal progression by GnRH agonist GnRHa treatment is associated with a continuous gain in adiposity despite suppression of gonadotropin and gonadal hormone secretion in such girls.
Thus, GnRHa treatment does not seem to reverse the adverse body compositional changes associated with early maturation. In the present study, authors have shown gitantismo girls with central precocious puberty have low insulin sensitivity and adverse lipid profiles at the time hipofisqrio diagnosis compared with normally timed pubertal controls.
These differences were not solely accounted for hipofisaro the concomitant higher adiposity found in girls with precocious puberty. In addition, age at pubertal onset positively predicted insulin sensitivity during the early stages of puberty.
Despite complete gonadal suppression, insulin sensitivity decreased even further, and total body fat percentage increased in giganyismo with central precocious puberty.
Girls with central precocious puberty had similar body mass index but significantly increased total body fat percentage by dual-energy x-ray absorptiometry, compared with normally timed pubertal controls.
The relationship between adiposity and pubertal timing seems to be causally bidirectional. Although the association between early maturation and increased cardiovascular risk may be primarily ascribed to greater adiposity, this does not adequately explain the association.
Thus, additional factors influencing both pubertal timing and cardiovascular disease risk, beyond the hipofisraio of adiposity, may be involved. Insulin could be such a factor, the earlier the pubertal onset, the lower the insulin sensitivity going into puberty or the steeper the fall in insulin sensitivity during puberty or a combination of both.
The lower insulin sensitivity in the earliest maturing girls was adequately compensated for by higher insulin secretion. In addition, the hipofosario insulin sensitivity in girls with central precocious puberty was not solely accounted for by the concomitantly higher adiposity compared with puberty matched controls. Thus, insulin sensitivity may play a central and independent role in the relationship between early pubertal timing and adverse metabolic risk. Thus, if sex steroids were implicated in the adverse metabolic programming of early puberty, one would intuitively expect a regression toward prepubertal levels or at least arrest in the metabolic parameters during treatment beyond this point.
By contrast, they found that the increased adiposity at time of diagnosis worsened after sex steroid withdrawal.
Thus, several of the adversely affected metabolic parameters evident at time of diagnosis deteriorated even further despite sex steroid withdrawal and regression in clinical signs of puberty. This speaks against a strong giganttismo of sex steroids, neither directly nor through central feedback mechanism, on changes in glucose homeostasis or body composition during puberty. Since the GH-IGF1 axis is not inhibited under gonadotropin suppression, It is possible to speculate that the deterioration of the metabolic profile could be secondary to a combined effect of a GH hipofissario and lack of steroid hormones.
Epub Mar Generalized glucocorticoid resistance syndrome is a rare familial or sporadic condition characterized by partial insensitivity to glucocorticoids, caused by mutations in the glucocorticoid receptor GR gene.
We identified a new 2-yr-old female case of generalized glucocorticoid resistance syndrome. The patient TJ presented with a generalized seizure associated with hypoglycemia and hypokalemia. She also had hypertension and premature pubarche, whereas dexamethasone effectively suppressed these clinical manifestations. Molecular analysis revealed that the mutant receptor had significantly impaired transactivation activity with a 2-fold reduction in affinity to ligand.
It showed attenuated transactivation of the activation function AF -2 and reduced binding to a p nuclear receptor coactivator. Computer-based structural analysis revealed that replacement of arginine by glutamine at position transmitted a conformational change to the LBD and the AF-2 transactivation surface, resulting in a decreased binding affinity to ligand and to the LXXLL coactivator motif.
Dexamethasone treatment is effective in controlling the premature pubarche, hopofisario, hypertension, and hypokalemia in this child case, wherein arginine plays a key role in the proper formation of the ligand-binding pocket and the AF-2 surface of the GR alpha LBD. Comments This patient was born at 35 wk of gestation with intrauterine growth retardation body weight at birth g.
She developed generalized tonic-clonic seizures followed by unconsciousness occurring several days after emesis and diarrhea at the age of 2 yr 10 months. Endocrinological examination identified elevated levels of serum cortisol Plasma aldosterone hipofisari and plasma renin activity were 4.
Generalized seizures of this case were due to her hypoglycemia, which would have been further exacerbated by the preexisting gastroenterological symptoms. The hypokalemia was most likely caused by increased mineralocorticoid as well as cortisol secretion.
Reduced glucocorticoid action in the liver, which could not stimulate sufficient glucose production most likely resulted in the hypoglycemia during the acute illness. Hypoglycemia and subsequent generalized seizures have never been reported in the adult cases of generalized glucocorticoid resistance syndrome; thus, these clinical manifestations should be considered especially in child cases of this syndrome.
Treatment with dexamethasone to suppress the ACTH production effectively giggantismo the clinical symptoms. The use of dexamethasone hopofisario normalization of her hypothalamic-pituitary-adrenal axis and hipofusario appropriately slowed her growth by efficiently suppressing excess production of adrenal androgens that accelerated bone development.
Epub Mar 3. Giganfismo novel ultrapressure liquid chromatography tandem mass spectrometry method for the simultaneous determination of androstenedione, testosterone, and dihydrotestosterone in pediatric blood samples: Current immunoassays for analysis of plasma androgens in children have several limitations due giganntismo antibody-specific variations of data and normal ranges.
Mass spectrometry-based methods are available for individual steroids but need complex sample preparation and report gigantis,o fragmentary reference data for hipofissario pediatric population. Our objective was to develop a state gigantismoo the art sensitive and specific tandem mass spectrometry method for high-throughput simultaneous determination of plasma concentrations of androstenedione Atestosterone Tand dihydrotestosterone DHT and to report age- sex- and pubertal stage-specific reference levels for these steroids in children aged yr.
Plasma microl was mixed with internal standard and extracted by solid-phase extraction. Androgens were measured by ultrapressure liquid chromatography tandem mass spectrometry.
Samples of boys gigantimo girls with neither signs of endocrine nor systemic disease were considered for the generation of reference data.
The following age groups were used: Lower quantification limit was 2. No hipofisari interference with other steroids was detected. Reference data for A, T, and DHT are reported as functions of age, sex, pubertal maturation, and testicular volume.
Simplicity, velocity, sensitivity, specificity, and the availability of pediatric reference data allow application of our new method in clinical routine as well as in research settings. Method-specific reference data for children and adolescents with respect to the entire pediatric age range, sex, and pubertal development are fragmentary, which, however, is the prerequisite for a meaningful biological interpretation of the data.
It provides high through put analyses combined with highest sensitivity. Finally, age- sex- and pubertal stage-specific reference data for these steroids were set up from a collection of pediatric samples. This method and these can be used both as reference method giganfismo the calibration of other assay techniques and as routine method in clinical practice.
In total, males and females were included. They showed the following age distribution: For pubertal stage for boys, groups were divided as follows: P1 less than 6 months 41P1 between 6 months and 9 yr 35P1 more than giganfismo yr 13P2 11P3 11P4 10and P5 For girls, they had the following groups: As a third division, they used testicular volume: For grouping, hipofisaario bigger testicular volume was used if different between left gogantismo right testes.
Tables for reference values median and gigantisom for age, Tanner stage and testicular volume are shown. Epub Jan Basal adrenal, gonadal and thyroid functions were normal.
Hand-wrist bone age was 3. Magnetic resonance imaging revealed a macroadenoma with moderate suprasellar invasion. The patient was admitted to our Endocrine Unit when 7. Treatment with cabergoline was initiated, but only PRL levels normalized. Thus, treatment with octreotide LAR was discontinued and pegvisomant was added to cabergoline, leading to the normalization of IGF-I levels and height velocity without side effects.
Other anterior pituitary functions were always normal. To conclude, treatment of pituitary gigantism with pegvisomant was effective and well tolerated in a young giant unresponsive to combined cabergoline and octreotide treatment. Los estudios de rutina fueron normales y no se observaron efectos secundarios. Pituitary gigantism is an extremely rare condition caused by endogenous growth hormone GH hypersecretion during childhood, prior to epiphyseal closure.
GH hypersecretion is usually due to a pituitary benign adenoma. Macroadenomas are more frequent than microadenomas and females are more frequently involved. The management of macroadenomas requires multiple therapeutical approaches, including surgery, radiation and medical treatment. Trans-naso-sphenoidal pituitary surgery TNS seems to be as safe in pediatric patients with gigantism as in adults.
However, treatment of pituitary gigantism in early childhood by surgery alone is rarely successful, possibly because of immature neumatization of face bones. In most cases, further therapies are needed to control the GH excess postoperatively. Medical therapy in acromegalic patients has highly progressed with the availability of dopamine agonists and somatostatin analogues such as octreotide and lanreotide.
In gigantism, treatment with somatostatin analogues has been found to be effective in some cases, but not in others, even in combination with cabergoline.
Sincepegvisomant, a pegylated GH receptor antagonist, represents a new therapeutic option for the management of patients with acromegaly. It has been demonstrated to be extremely effective in reducing serum insulin-like growth factor I IGF-I concentrations in the majority of acromegalic patients resistant to somatostatin analogue. Normal doses are between 10 and 40 mg s.
Nevertheless, current experience in pediatric patients is still limited. She had coarse facial features, and disproportionately large hands and feet.