GIGANTISMO HIPOFISARIO PDF

Marco A. Rivarola y Alicia Belgorosky. J Clin Endocrinol Metab. Context: Early menarche is associated with increased risk of cardiovascular disease in adulthood.

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Marco A. Rivarola y Alicia Belgorosky. J Clin Endocrinol Metab. Context: Early menarche is associated with increased risk of cardiovascular disease in adulthood.

It is unknown whether metabolic risk factors are adversely affected in girls with central precocious puberty CPP already at time of diagnosis. Objective: The objective of the study was to evaluate metabolic profiles in girls with early normal puberty EP and CPP.

Design and Setting: This was a combined cross-sectional and longitudinal study at a tertiary center of pediatric endocrinology. Patients and Intervention: 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.

Main Outcome Measures: 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.

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 that girls with central precocious puberty have low insulin sensitivity and adverse lipid profiles at the time of diagnosis compared with normally timed pubertal controls.

These differences were not solely accounted for by 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 girls 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 effect 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 lower 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 influence 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 increment and lack of steroid hormones.

Epub Mar CONTEXT: 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.

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.

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 level 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 controlled the clinical symptoms. The use of dexamethasone allowed normalization of her hypothalamic-pituitary-adrenal axis and thus appropriately slowed her growth by efficiently suppressing excess production of adrenal androgens that accelerated bone development. Epub Mar 3. CONTEXT: Current immunoassays for analysis of plasma androgens in children have several limitations due to antibody-specific variations of data and normal ranges.

Mass spectrometry-based methods are available for individual steroids but need complex sample preparation and report only fragmentary reference data for the pediatric population. OBJECTIVE: Our objective was to develop a state of the art sensitive and specific tandem mass spectrometry method for high-throughput simultaneous determination of plasma concentrations of androstenedione A , testosterone T , and dihydrotestosterone DHT and to report age-, sex-, and pubertal stage-specific reference levels for these steroids in children aged yr.

Androgens were measured by ultrapressure liquid chromatography tandem mass spectrometry. Samples of boys and girls with neither signs of endocrine nor systemic disease were considered for the generation of reference data. The following age groups were used: less than 1 wk, 2 wk to 2 months, months, months, yr, yr, yr, yr, yr, and over 16 yr. No relevant 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. 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 for 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: less than 1 wk eight males, six females , 2 wk to 2 months 19 males, 23 females , 3—5 months 14 males, 10 females , 6—11 months four males, 10 females , 1—3 yr nine males, eight females , 4—6 yr 10 males, 13 females , 7—9 yr 10 males, 14 females , 10—12 yr 18 males, 18 females , 13—15 yr 25 males, 19 females , and more than 16 yr 21 males, 10 females.

For pubertal stage for boys, groups were divided as follows: P1 less than 6 months 41 , P1 between 6 months and 9 yr 35 , P1 more than 9 yr 13 , P2 11 , P3 11 , P4 10 , and P5 For girls, they had the following groups: P1 less than 8 yr 71 , P1 more than 8 yr 14 , P2 10 , P3 10 , P4 11 , and P5 As a third division, they used testicular volume: 1—2 ml less than 6 months 40 , 1—2 ml between 6 months and 9 yr 26 , 1—2 ml more than 9 yr 7 , 3—4 ml 7 , 5—10 ml 9 , 11—15 ml 15 , and more than 15 ml For grouping, the bigger testicular volume was used if different between left and right testes.

Tables for reference values median and range for age, Tanner stage and testicular volume are shown. Horm Res Paediatr. Epub Jan Abstract A 3.

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.

Estaba prepuberal. 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. Since , pegvisomant, 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.

She did not complain visual field deficiencies. She was prepubertal. Basal adrenal, gonadal and thyroid functions were normal for age, as were routine hematological findings and urine osmolarity. Magnetic resonance imaging MRI revealed an intrasellar pituitary macroadenoma with moderate suprasellar invasion.

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Category:Gigantism

From Wikimedia Commons, the free media repository. In humans, this condition is caused by over-production of growth hormone in childhood before the long bone epiphyses closes resulting in persons between 7 feet 2. Subcategories This category has the following 4 subcategories, out of 4 total. Media in category "Gigantism" The following 19 files are in this category, out of 19 total.

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Acromegalia

Em , foram publicadas diretrizes para o manejo da acromegalia, o qual envolve, muitas vezes, uma abordagem multidisciplinar. Acromegaly is a disabling and disfiguring illness, which, if not adequately controlled, decreases life expectancy. Cardiovascular and respiratory complications represent the main causes of death in acromegalic patients. Progress in all therapeutic modalities has been made, allowing biochemical disease control in more patients. In , the guidelines for management of acromegaly were published which encompass, many times, a multidisciplinary approach. In this article, we critically evaluate what is available in Brazil that allows us to follow the guidelines established in the diagnosis and treatment consensus.

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Bibliographic Reviews – Ed. 29

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