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[Radiologic approach to endocrine pathology in childhood].

The new imaging techniques (ultrasound, CT, digital subtraction angiography) have greatly modified the diagnostic approach to the endocrinopathies in pediatric age, notably if of surgical interest. The main clinical indications to the ultrasound and/or CT study of hypophysis, thyroid, adrenals and female internal genital tract are synthesized in this paper. A diagnostic flow-chart is also suggested for each clinical entity.

Adrenal Gland Diseases↗

Laparoscopic adrenalectomy and adrenal-preserving surgery.

PURPOSE OF REVIEW: The aim of this paper is to define the current role of laparoscopy in the management of surgical adrenal diseases evaluating the surgical aspects, the indications and contraindications of laparoscopic adrenalectomy, focusing also on the most innovative tendencies in the laparoscopic adrenal-preserving surgery. RECENT FINDINGS: Recent publications have described some interesting new indications that need to be confirmed by long-term follow up. The present review mainly focuses on defining the state of the art of current adrenal laparoscopic surgery. SUMMARY: Laparoscopic adrenalectomy is becoming the 'platinum standard' for the treatment of the adrenal surgical diseases and it should be considered the treatment of choice for benign adrenal diseases. In cases of malignancy and conservative surgery, adrenalectomy appears to be very promising, although a longer follow up and further studies are still needed to accurately assess the role played by these procedures. Finally, who should do laparoscopic adrenalectomy? Every patient who requires the ablation of the adrenal should receive laparoscopic opportunity. And the surgeons? Only those with advanced laparoscopic skills and a good knowledge of adrenal anatomy and pathophysiology will obtain the same excellent results currently reported in the literature.

Adrenal Gland Diseases↗

[Limits, potentials and prospects of x-ray study in the diagnosis of adrenal changes].

Data of clinical and x-ray examinations of the adrenals in 250 patients with various endocrine diseases are analyzed. The author considers a correctly performed pneumographic examination of the adrenals to be an effective method for the diagnosis of the diseases of this organ of internal secretion. In some cases pneumography of the adrenals should be supplemented with other x-ray examination methods (angiography, infusion urography) or scintigraphy. If a pneumographic examination fails (due to bad preparedness of the patient, or insufficient quantity of the infused gas, or features of the retroperitoneal fat, or retroperitoneal fat adhesions, etc.) it is undesirable to repeat it. Other types of x-ray examination or scanning should be performed in such cases.

Adolescent↗

Bilateral adrenal hemorrhage and adrenal insufficiency in a patient with lymphomatous adrenal infiltration following administration of a fusion toxin (DAB486 interleukin-2).

DAB486IL-2 is a novel fusion toxin in which the ADP-ribosyltransferase and membrane-translocating domains of diphtheria toxin have been combined with the interleukin-2 (IL-2) gene, creating a recombinant protein capable of selectively intoxicating cells bearing the high-affinity IL-2 receptor. Clinical activity has been documented in Hodgkin disease and the non-Hodgkin lymphomas; toxicities have been minimal and include mild hepatic transaminitis, proteinuria, and hypersensitivity reactions. In this report, a patient with tumor-stage cutaneous T-cell lymphoma developed clinical adrenal failure with bilateral adrenal hemorrhage and necrosis 7 weeks after completing a 5-day course of treatment with DAB486IL-2. The relationship of fusion toxin therapy to the development of this unusual toxicity is discussed.

Adrenal Gland Diseases↗

Adrenal cytomegaly.

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Adrenal Gland Diseases↗

Hypoglycemia and the sympathoadrenal system: neurogenic symptoms are largely the result of sympathetic neural, rather than adrenomedullary, activation.

The relative contributions of the sympathetic nervous system and the adrenal medullae, the two components of the sympathoadrenal system, to the manifestations of hypoglycemia are largely unknown. We tested the hypothesis that the neurogenic symptoms of hypoglycemia are largely the result of sympathetic neural activation. To do so, we quantitated neurogenic symptoms, as well as norepinephrine (NE) kinetics and selected hemodynamic changes, during hyperinsulinemic euglycemic and stepped hypoglycemic clamps in 15 healthy control subjects (Controls) and four bilaterally adrenalectomized patients (ADX). Plasma epinephrine responses to hypoglycemia were virtually absent in ADX, as expected. Neurogenic symptom scores increased to higher values during the hypoglycemic compared with the euglycemic clamps in both Controls (P < 0.0001) (e.g., final scores of 7.8 +/- 1.2 vs. 3.0 +/- 0.7) and ADX (P < 0.0001) (e.g., final scores of 10.8 +/- 4.1 vs. 2.5 +/- 1.0). Plasma NE concentrations (P < 0.0001) and systemic NE spillover (P = 0.0007) increased during the hypoglycemic compared with the euglycemic clamps in Controls but not in ADX. Similarly, heart rate increased (P = 0.0104), diastolic blood pressure decreased (P = 0.0003), and forearm blood flow increased (P < 0.0001) during the hypoglycemic compared with the euglycemic clamps in Controls but not in ADX. These data indicate that the neurogenic symptoms of hypoglycemia are largely the result of sympathetic neural, rather than adrenomedullary, activation. They also suggest that the plasma NE and hemodynamic responses to hypoglycemia are largely the result of adrenomedullary, rather that sympathetic neural, activation.

Adrenal Gland Diseases↗

Disseminated herpes simplex infection with cystic fibrosis: a case report.

A 6 months old female infant presented with history of fever, cough and severe respiratory distress. There was past history of recurrent attacks of pneumonia. She succumbed to the illness after a hospital stay of 7 days. Postmortem revealed morphological evidence of cystic fibrosis along with herpes simplex infection of liver and adrenals. The co-existence of disseminated herpes simplex infection and cystic fibrosis is very rare.

Adrenal Gland Diseases↗

Management of the adrenal "incidentaloma".

The suprarenal fossa has been a clinically silent area of the body where nonfunctional lesions of soccer ball size could go undetected until they disturbed functions of adjacent organs. Unless an adrenal tumor was suspected by biochemical or clinical screening information, early diagnosis was an exception, and localization usually required invasive techniques. With the advent of noninvasive computer-assisted radiologic and sonographic techniques, surgeons now have the reverse problem of early diagnosis of asymptomatic adrenal mass: do we need to know about nonfunctioning "incidentalomas" and what further action is compelled by their discovery? Sophisticated adrenal imaging techniques, even if noninvasive, should be be utilized as a localizing technique after the biochemical determination of adrenal disease is established. Localization should not precede the biochemical confirmation of endocrine active adrenal disease. For adrenal masses discovered in asymptomatic patients, predictability of curative adrenal surgery is only as good as the strength of the causal link between the clinical evidence of adrenal disease and the localizing information; thus the indication for operation does not rely solely upon the resolving power of adrenal imaging to demonstrate adrenal masses. This causal link is not strengthened by one radiographic test that confirms the results of another in the absence of evidence that adds functional significance to the radiographic findings. For the "incidentaloma" of the adrenal that might represent neoplasm, techniques similar to those employed for thyroid masses should be used for diagnosis rather than the routine prescription of operation based on the mere presence of a space-occupying lesion. Direct correlation of imaging studies applied to prior patient findings is necessary before operation is indicated for adrenal "incidentalomas."

Adrenal Gland Diseases↗

Growth abnormalities associated with adrenal disorders and their management.

Linear growth can be disturbed in paediatric adrenal disease associated with endocrine hypo- or hyperfunction. Tall stature is a feature in some patients with adrenocorticotropic hormone resistance syndromes and short stature is recognized in the IMAGe (intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia congenita and genital anomalies) association. In autoimmune Addison's disease, growth is usually normal. In congenital adrenal hyperplasia, height may be compromised by advanced skeletal maturation or by suppressed growth, particularly in the neonatal period due to excess glucocorticoid treatment. In virilizing adrenal tumours, height is increased at diagnosis, but after surgical cure final height is usually in the normal range. In Cushing's disease, height was abnormally short in 50% of patients at presentation. After successful treatment, spontaneous catch-up growth was not seen. This led to a diagnosis of growth hormone (GH) deficiency in 80% of patients. With GH replacement, catch-up growth and long-term benefit occurred. Disturbance of linear growth is an important feature of many patients with adrenal disorders in childhood. Assessment of its pathogenesis and careful management are necessary to ensure optimal final adult height.

Adrenal Gland Diseases↗