1/36. Thalamic and hypothalamic tumors of childhood: endocrine late effects.Children who have received chemotherapy and radiation therapy for treatment of thalamic/hypothalamic tumors are at risk for late effects, specifically endocrine dysfunction. Evaluation of growth and pubertal development, thyroid function and integrity of the hypothalamic-pituitary-adrenal axis should be undertaken in a prospective manner. Issues of metabolic disturbances such as obesity, altered body composition/bone density as well as ultimate fertility also need to be addressed by ongoing prospective evaluations.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
2/36. Polyglandular endocrine failure in a patient with amyloidosis secondary to familial mediterranean fever.familial mediterranean fever (FMF) is 1 of the major causes of secondary amyloidosis. Renal involvement is the main clinical complication and it mostly presents with nephrotic syndrome and chronic renal failure. Although deposition of amyloid has been reported in several endocrine glands such as the adrenal, thyroid, and testes, clinically significant functional impairment is uncommon. Herein, we describe a patient in whom the diagnosis of FMF was based on molecular screening and who presented with recurrent hypoglycemic attacks and extensive amyloid deposition affecting various organ function including adrenal, thyroid, parathyroid, testes, intestinal system, and the heart.- - - - - - - - - - ranking = 2keywords = adrenal (Clic here for more details about this article) |
3/36. Isolated adrenocorticotropin deficiency presenting with impaired renin-angiotensin-aldosterone system and suppressed parathyroid hormone-vitamin d axis.We report here a 47-year-old woman with isolated adrenocorticotropin (ACTH) deficiency (IAD). She presented impaired renin-angiotensin-aldosterone (R-A-A) system and suppressed parathyroid hormone (PTH)-vitamin d system. She showed severe hyponatremia due to secondary adrenocortical insufficiency, which was deteriorated by hypoaldosteronism. She also showed hyperphosphatemia and relative hypercalcemia with suppressed PTH-vitamin d axis. Moreover, she showed hypothyroidism, which was thought to be important to maintain normal Ca levels under secondary hypoadrenalism via decrease in bone resorption by T3. Replacement with glucocorticoid completely normalized PTH-vitamin d axis and R-A-A system. Thus, the present case implicates that severe adrenocortical deficiency due to IAD might affect both R-A-A system and PTH-vitamin d axis. These findings suggest that the ACTH-cortisol axis has an important role in mineral metabolism in vivo.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
4/36. An endocrinopathy characterized by dysfunction of the pituitary-adrenal axis and alopecia universalis: supporting the entity of a triple H syndrome.We demonstrate the rare disorder of triple H syndrome in a 25-year-old man. He was pointed out as having short stature, at -5.9 s.d., and diagnosed as GH deficient at 6 years old. Approximately a year ago, he noticed systematic hair loss. He lost body weight by 7 kg during the last half year. He was admitted to Jichi Medical School Hospital because of unconsciousness. Physical findings showed disturbance of consciousness with japan coma Scale I-3. He had emaciation and alopecia universalis. Laboratory findings showed plasma glucose was as low as 1.11 mmol/l. GH and ACTH deficiency with hypoadrenocorticism were clarified. His intelligence was in the low normal range with a WAIS IQ of 70, and anterograde amnesia was suggested in the presence of a little, but not significant, morphological change in the hippocampus on a magnetic resonance imaging scan. Replacement by a physiological dose of hydrocortisone normalized plasma glucose, and restored body weight and growth of hair during the 7 month therapeutic period. The present finding strongly supports a clinical entity of triple H syndrome, including ACTH deficiency, alopecia universalis and anterograde amnesia, and that there may be some variation of the triad among the subjects.- - - - - - - - - - ranking = 4keywords = adrenal (Clic here for more details about this article) |
5/36. Adrenal toxicity in dogs and cats as a contributing cause of hormonal and immune destabilization.The adrenal cortex is regarded as the organ most vulnerable to toxicity within the endocrine system. The production of cortisol, among the many steroidal hormones produced by the cortex, may suffer as a result. In a veterinary clinical practice, household dogs and cats with a wide variety of diseases ranging from allergies to cancer commonly have a cortisol deficiency or defect that triggers endocrine imbalances and immune system destabilization. The causes of deficient cortisol are linked primarily to genetics but also to acquired adrenal damage likely stemming from environmental toxins. An innovative blood test to determine relevant endocrine-immune imbalances in pets and a treatment method based on low-dosage steroidal medication, as a form of cortisol replacement therapy, are described. Despite a prevailing reluctance to use steroidal medications long term because of the fear of side effects, extended and even life-time usage of these medications at low, physiologic dosages has been applied successfully for decades and appears to be gaining wider acceptance. The validity of a combined testing and treatment method for humans based on the veterinary model deserves investigation as a tool with which to identify and correct toxic damage to adrenal function.- - - - - - - - - - ranking = 3keywords = adrenal (Clic here for more details about this article) |
6/36. Bilateral adrenal cysts and ectopic pancreatic tissue in beckwith-wiedemann syndrome: is a conservative approach acceptable?beckwith-wiedemann syndrome is a common overgrowth syndrome associated with an increased risk of neoplasias which might be explained by the nature and localization of the genetic defect. While malignant tumors are often associated with hemihypertrophy, benign tumors are also found. We report a patient with the typical features of beckwith-wiedemann syndrome with two histologically different abdominal tumors, bilateral cystic adrenals and ectopic pancreatic tissue present at birth. In both tumors no malignancy could be detected. Ectopic pancreatic tissue is rarely seen and has been described in beckwith-wiedemann syndrome only once. After extirpation of the ectopic pancreatic tissue the cystic adrenals were left in situ since macroscopically no normal adrenal tissue could be identified and separated. Regular ultrasound examinations revealed complete resolution of the cystic adrenals within 24 months. Thus it seems that a conservative approach in selected tumors associated with the beckwith-wiedemann syndrome might be acceptable.- - - - - - - - - - ranking = 8keywords = adrenal (Clic here for more details about this article) |
7/36. Endocrine emergencies: recognizing signs and symptoms.Endocrine emergencies may present to the pediatric practitioner in the office setting in a variety of forms. Four of the more common pediatric endocrine emergencies (DKA, symptomatic hypoglycemia, adrenal insufficiency, and hypocalcemia) have been discussed here. The recommended approach to a child or adolescent with an endocrine emergency involves recognizing clinical disease, stabilizing the patient with basic and advanced life support intervention, and transferring the patient to a facility which can provide appropriate definitive care.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
8/36. Sparse hair and multiple endocrine disorders in two women heterozygous for adrenoleukodystrophy.We describe two sisters (40 and 42 years old) heterozygous for adrenoleukodystrophy who have multiple endocrine disorders. In addition to the characteristic neurological symptoms, the younger patient has addison disease and primary hypothyroidism attributable to autoimmune thyroiditis, and the older one has graves disease. Both patients have loss of body hair and sparse scalp hair, which have not been reported previously in women heterozygous for adrenoleukodystrophy. After the institution of glucocorticoid replacement therapy, the younger sister, who has adrenal insufficiency, has shown unexpected neurological improvement.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
9/36. Functional tumors of the organ of Zuckerkandl.Chromaffin-reacting pheochromocytomas of the adrenal medulla are the most frequently encountered functional paraganglionic neoplasms. However, extra-adrenal pheochromocytomas as well as non-chromaffin paragangliomas, including those of the carotid body and glomus jugulare, may produce symptoms from catecholamine secretion. One of the extra-adrenal sites from which these tumors arise is from a collection of para-aortic, paraganglion cells around the origin of the inferior mesenteric artery. This collection of paraganglia was described in fetuses by Zuckerkandl in 1901 and has subsequently been referred to as the organ of Zuckerkandl. The diagnosis and management of these neoplasms differ somewhat from that of adrenal pheochromocytomas, but excellent results are often obtained by excision of these lesions. Four patients with functional tumors of the organ of Zuckerkandl are reviewed together with the other reported cases in the literature.- - - - - - - - - - ranking = 4keywords = adrenal (Clic here for more details about this article) |
10/36. Endocrine complications of the acquired immunodeficiency syndrome.Endocrine syndromes in human immunodeficiency virus infection, although not as common as infectious, renal, or neurologic manifestations, are now more frequently recognized. These include unique thyroid function abnormalities, adrenal insufficiency, electrolyte abnormalities, hypogonadotrophic hypogonadism, and multiple endocrine side effects of medications used to treat complications of human immunodeficiency virus infection.- - - - - - - - - - ranking = 1keywords = adrenal (Clic here for more details about this article) |
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