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Sample records for adrenal insufficiency

  1. Adrenal insufficiency.

    Science.gov (United States)

    Li-Ng, Melissa; Kennedy, Laurence

    2012-10-01

    Adrenocortical insufficiency may arise through primary failure of the adrenal glands or due to lack of ACTH stimulation as a result of pituitary or hypothalamic dysfunction. Prolonged administration of exogenous steroids will suppress the hypothalamic-pituitary-adrenal axis, and hence cortisol secretion. We review briefly the causes, investigation, and treatment of adrenal insufficiency, and highlight aspects of particular relevance to patients with adrenal tumors.

  2. [Adrenal mass and adrenal insufficiency].

    Science.gov (United States)

    Martínez Albaladejo, M; García López, B; Serrano Corredor, S; Alguacil García, G

    1996-12-01

    Primary adrenal insufficiency is a non frequent disease, that is declared in young adults and in the most of the cases is produced from an autoimmune mechanism or a tuberculous disease. The incidence of these forms in the different geographic areas is dependent of degree of irradication of the tuberculosis. We report the case of a patient with latent chronic adrenal insufficiency of tuberculous origin who was affected for an addisonian crisis during an intercurrent infectious disease, which permitted the diagnosis of the addisonian crisis, and Mal of Pott was moreover detected. Evolution with corticosteroid and specific treatment was very favorable.

  3. Adrenal insufficiency: diagnosis and management.

    Science.gov (United States)

    Munver, Ravi; Volfson, Ilya A

    2006-01-01

    Adrenal insufficiency is a disorder characterized by hypoactive adrenal glands resulting in insufficient production of the hormones cortisol and aldosterone by the adrenal cortex. This disorder may develop as a primary failure of the adrenal cortex or be secondary to an abnormality of the hypothalamic-pituitary axis. Patients with adrenal insufficiency often are asymptomatic or they may present with fatigue, muscle weakness, weight loss, low blood pressure, and sometimes darkening of the skin. The presentation of adrenal insufficiency varies dramatically and poses a major diagnostic dilemma. This review focuses on the diagnosis and treatment of primary and secondary adrenal insufficiency.

  4. [Pediatric emergency: adrenal insufficiency and adrenal crisis].

    Science.gov (United States)

    Martínez, Alicia; Pasqualini, Titania; Stivel, Mirta; Heinrich, Juan Jorge

    2010-04-01

    Adrenal insufficiency is defined by impaired secretion of adrenocortical hormones. It is classified upon the etiology in primary and secondary. Rapid recognition and therapy of adrenocortical crisis are critical to survival. Patients often have nonspecific symptoms: anorexia, vomiting, weakness, fatigue and lethargy. They are followed by hypotension, shock, hypoglicemia, hyponatremia and hyperkalemia. All patients with adrenal insufficiency require urgent fluid reposition, correction of hypoglycemia and glucocorticoid replacement, in order to avoid serious consequences of adrenal crisis. After initial crisis treatment, maintenance dose of corticoids should be indicated. Mineralocorticoids replacement, if necessary, should also be initiated.

  5. Budesonide-related adrenal insufficiency.

    Science.gov (United States)

    Arntzenius, Alexander; van Galen, Louise

    2015-10-01

    Iatrogenic adrenal insufficiency is a potential harmful side effect of treatment with corticosteroids. It manifests itself when an insufficient cortisol response to biological stress leads to an Addisonian crisis: a life-threatening situation. We describe a case of a patient who developed an Addisonian crisis after inappropriate discontinuation of budesonide (a topical steroid used in Crohn's disease) treatment. Iatrogenic adrenal insufficiency due to budesonide use has been rarely reported. Prescribers should be aware of the resulting risk for an Addisonian crisis.

  6. Diagnosis and management of adrenal insufficiency.

    Science.gov (United States)

    Bancos, Irina; Hahner, Stefanie; Tomlinson, Jeremy; Arlt, Wiebke

    2015-03-01

    Adrenal insufficiency continues to be a challenge for patients, their physicians, and researchers. During the past decade, long-term studies have shown increased mortality and morbidity and impaired quality of life in patients with adrenal insufficiency. These findings might, at least partially, be due to the failure of glucocorticoid replacement therapy to closely resemble physiological diurnal secretion of cortisol. The potential effect of newly developed glucocorticoid drugs is a focus of research, as are the mechanisms potentially underlying increased morbidity and mortality. Adrenal crisis remains a threat to lives, and awareness and preventative measures now receive increasing attention. Awareness should be raised in medical teams and patients about adrenal insufficiency and management of adrenal crisis to improve clinical outcome.

  7. Novel insights into adrenal insufficiency in childhood.

    Science.gov (United States)

    Levy-Shraga, Y; Pinhas-Hamiel, O

    2014-12-01

    Adrenal insufficiency (AI) is a rare disease in childhood and adolescence that results from disruption in the hypothalamic-pituitary-adrenal axis. Pediatricians should be familiar with this entity since acute adrenal crisis is a life-threatening condition that requires immediate treatment. In the early stages of AI, the clinical manifestations may be subtle and non-specific; thus, they are frequently unrecognized. The main therapeutic approach in all forms of adrenal insufficiency is glucocorticoid replacement; the dose should be titrated appropriately to avoid under or overtreatment. Patient and family education is particularly important, to enable adjustment of dosage replacement therapy during stress and to prevent crisis. This article summarizes the current knowledge of AI and provides new insights on its management in children.

  8. Chronic Heroin Dependence Leading to Adrenal Insufficiency

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    Gautam Das

    2014-01-01

    Full Text Available Opioids have been the mainstay for pain relief and palliation over a long period of time. They are commonly abused by drug addicts and such dependence usually imparts severe physiologic effects on multiple organ systems. The negative impact of opioids on the endocrine system is poorly understood and often underestimated. We describe a patient who developed severe suppression of the hypothalamic-pituitary adrenal (HPA axis leading to secondary adrenal insufficiency due to long standing abuse of opioids.

  9. Adrenal Insufficiency - Aetiology, Diagnosis and Treatment

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    Nazma Akter

    2015-03-01

    Full Text Available Adrenal insufficiency is caused by either primary adrenal failure or by hypothalamic-pituitary impairment of the corticotropic axis. Adrenal insufficiency, first codified in 1855 by Thomas Addison, remains relevant in 2014 because of its lethal nature. Though, it is a rare disease but is life threatening when overlooked. Main presenting symptoms such as fatigue, anorexia and weight loss are nonspecific, thus diagnosis is often delayed. The diagnostic work-up is well established but some pitfalls remain. The diagnosis is adequately established by the 250 μg ACTH (adrenocorticotropic hormone stimulation test in most cases. Glucocorticoids provide life saving treatment but long-term quality of life is impaired, perhaps because therapy is not given in a physiologic way. Dehydroepiandrosterone-replacement therapy has been introduced that could help to restore quality of life. It may be useful in pubertal girls, but not in adults. Monitoring of glucocorticoid-replacement is difficult due to lack of objective methods of assessment and is therefore largely based on clinical grounds. Thus, long-term management of patients with adrenal insufficiency remains a challenge, requiring an experienced specialist.

  10. Adrenal insufficiency and adrenal replacement therapy. Current status in Spain.

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    Aulinas, Anna; Casanueva, Felipe; Goñi, Fernando; Monereo, Susana; Moreno, Basilio; Picó, Antonio; Puig-Domingo, Manel; Salvador, Javier; Tinahones, Francisco J; Webb, Susan M

    2013-03-01

    Adrenal insufficiency (AI) is a rare endocrine disease, associated to increased mortality if left untreated. It can be due to a primary failure of the adrenal glands (primary AI) or malfunctioning of the hypothalamic-pituitary-adrenal axis (HPA) (secondary AI). The lack of data on incidence/prevalence of adrenal insufficiency in Spain complicates any evaluation of the magnitude of the problem in our country. Initial symptoms are non-specific, so often there is a delay in diagnosis. Current therapy with available glucocorticoids is associated with decreased quality of life in patients with treated AI, as well as with increased mortality and morbidity, probably related to both over-treatment and lack of hydrocortisone, associated with non-physiological peaks and troughs of the drug over the 24 hours. The availability of a new drug with a modified dual release (immediate and retarded), that requires one only daily dose, improves and simplifies the treatment, increases compliance as well as quality of life, morbidity and possibly mortality. This revision deals with the knowledge on the situation both globally and in Spain, prior to the availability of this new drug.

  11. Adrenal insufficiency in patients with decompensatedcirrhosis

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Adrenal reserve depletion and overstimulation of thehypothalamus-pituitary-adrenal (HPA) axis are causesfor adrenal insufficiency (AI) in critically ill individuals.Cirrhosis is a predisposing condition for AI in cirrhotics aswell. Both stable cirrhotics and liver transplant patients(early and later after transplantation) have been reportedto present AI. The mechanisms leading to reducedcortisol production in cirrhotics are the combination oflow cholesterol levels (the primary source of cortisol), theincreased cytokines production that overstimulate andexhaust HPA axis and the destruction of adrenal glandsdue to coagulopathy. AI has been recorded in 10%-82%cirrhotics depending on the test used to evaluate adrenalfunction and in 9%-83% stable cirrhotics. The similarityof those proportions support the assumption that AI isan endogenous characteristic of liver disease. However,the lack of a gold standard method for AI assessmentand the limitation of precise thresholds in cirrhoticsmake difficult the recording of the real prevalence of AI.This review aims to summarize the present data overAI in stable, critically ill cirrhotics and liver transplantrecipients. Moreover, it provides information about thecurrent knowledge in the used diagnostic tools and thepossible effectiveness of corticosteroids administration incritically ill cirrhotics with AI.

  12. Early intervention and management of adrenal insufficiency in children.

    LENUS (Irish Health Repository)

    Moloney, Sinéad

    2012-09-01

    The endocrine disorder adrenal insufficiency includes inadequate production of the steroid hormone cortisol. This results in poor physiological responses to illness, trauma or other stressors and risk of adrenal crisis. Management is based on administration of hydrocortisone. It is important to avoid under- or over-treatment and increase the dosage during times of physiological stress. To reduce morbidity, hospital admissions and mortality, the education and empowerment of parents and carers, and prompt intervention when necessary are essential. A steroid therapy card for adrenal insufficiency containing personal information on a patient\\'s condition was developed for use by families and their specialist centres.

  13. Acute adrenal insufficiency due to primary antiphospholipid antibody syndrome

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    Kishore Kumar Behera

    2013-01-01

    Full Text Available Introduction: We report a case of acute adrenal insufficiency (AAI in a patient with antiphospholipid syndrome (APS. Case Report: A 44-year-old female patient presented to us with acute abdominal pain associated with recurrent vomiting and giddiness. On examination, her blood pressure was 80/50 mm Hg. Systemic examination was normal. Further evaluation revealed hypocortisolemia with elevated plasma adrenocorticotropin hormone indicative of primary adrenal insufficiency. Her abdominal computed tomography scan showed features of evolving bilateral adrenal infarction. Etiological work-up revealed prolonged activated thromboplastin time, which didn′t correct with normal plasma, her anti-cardiolipin antibody and lupus anticoagulant were also positive. She was diagnosed to have APS with adrenal insufficiency and she was started on intravenous steroids and heparin infusion. Conclusion: AAI due to the APS can present with acute abdominal pain followed by hypotension. A high index of suspicion is needed to make the correct diagnosis and to initiate appropriate treatment.

  14. A case of human intramuscular adrenal gland transplantation as a cure for chronic adrenal insufficiency.

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    Grodstein, E; Hardy, M A; Goldstein, M J

    2010-02-01

    Intramuscular endocrine gland transplantation has been well described as it pertains to parathyroid autotransplantation; however, transplantation of the adrenal gland is less well characterized. While adrenal autotransplantation in the setting of Cushing's disease has been described, intramuscular adrenal allotransplantation as a cure for adrenal insufficiency to our knowledge has not been previously carried out. Current treatment for adrenal insufficiency leaves patients without diurnal variation in cortisol release and susceptible to the detrimental effects of chronic hypercortisolism. We describe here the case of a 5-year-old girl with renal failure who had adrenal insufficiency following fulminant meningococcemia that led to requirements for both stress-dose steroid and mineralocorticoid replacement. Ten months after the onset of her disease, she received a simultaneous renal and adrenal gland transplant from her mother. The adrenal gland allograft was morselized into 1 mm(3) segments and implanted into three 2 cm pockets created in her rectus abdominis muscle. Three years after surgery, her allograft remains fully functional, responding well to adrenocorticotropin hormone stimulation and the patient does not require any steroid or mineral-corticoid supplementation. We believe this case represents the first description of successful functional intramuscular adrenal allograft transplantation with long-term follow up as a cure for adrenal insufficiency.

  15. Severe Psychotic Disorder as the Main Manifestation of Adrenal Insufficiency

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    Farah, Julia de Lima; Lauand, Carolina Villar; Chequi, Lucas; Fortunato, Enrico; Pasqualino, Felipe; Bignotto, Luis Henrique; Batista, Rafael Loch; Aprahamian, Ivan

    2015-01-01

    We describe a case of severe psychotic disorder as the only manifestation of primary adrenal insufficiency. A 63-year-old man presented with psychotic symptoms without any prior psychiatric history. During the clinical and laboratorial investigation, exams revealed a normovolemic hyponatremia. The patient showed no other clinical signs or symptoms compatible with adrenal insufficiency but displayed very high ACTH and low serum cortisol concentrations. Brain magnetic resonance imaging showed no significant changes, including the pituitary gland. The patient was initially treated with intravenous corticosteroids, resulting in rapid remission of the psychotic symptoms. The association between adrenal insufficiency and neuropsychiatric symptoms is rare but these symptoms can often be the first clinical presentation of the disease. PMID:25954562

  16. Severe Psychotic Disorder as the Main Manifestation of Adrenal Insufficiency

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    Julia de Lima Farah

    2015-01-01

    Full Text Available We describe a case of severe psychotic disorder as the only manifestation of primary adrenal insufficiency. A 63-year-old man presented with psychotic symptoms without any prior psychiatric history. During the clinical and laboratorial investigation, exams revealed a normovolemic hyponatremia. The patient showed no other clinical signs or symptoms compatible with adrenal insufficiency but displayed very high ACTH and low serum cortisol concentrations. Brain magnetic resonance imaging showed no significant changes, including the pituitary gland. The patient was initially treated with intravenous corticosteroids, resulting in rapid remission of the psychotic symptoms. The association between adrenal insufficiency and neuropsychiatric symptoms is rare but these symptoms can often be the first clinical presentation of the disease.

  17. Paraneoplastic (non-metastatic) adrenal insufficiency preceded the onset of primary lung cancer by 12 weeks

    OpenAIRE

    Shantha, Ghanshyam Palamaner Subash; Kumar, Anita A; Jeyachandran, Vijay; Rajamanickam, Deepan; Bhaskar, Emmanuel; Paniker, Vinod K; Abraham, Georgi

    2009-01-01

    Clinically evident adrenal insufficiency associated with lung cancer is a rare entity. Among reported cases, adrenal insufficiency has occurred with or succeeded the primary lung cancer. Adrenal insufficiency has also been secondary to metastasis to the adrenal gland. The present report concerns a 61-year-old man, a chronic smoker, who presented to us with symptomatic adrenal insufficiency. He had no evidence of lung cancer during this visit. The primary lung cancer was only identified 12 wee...

  18. Paraneoplastic (non-metastatic) adrenal insufficiency preceded the onset of primary lung cancer by 12 weeks.

    Science.gov (United States)

    Shantha, Ghanshyam Palamaner Subash; Kumar, Anita A; Jeyachandran, Vijay; Rajamanickam, Deepan; Bhaskar, Emmanuel; Paniker, Vinod K; Abraham, Georgi

    2009-01-01

    Clinically evident adrenal insufficiency associated with lung cancer is a rare entity. Among reported cases, adrenal insufficiency has occurred with or succeeded the primary lung cancer. Adrenal insufficiency has also been secondary to metastasis to the adrenal gland. The present report concerns a 61-year-old man, a chronic smoker, who presented to us with symptomatic adrenal insufficiency. He had no evidence of lung cancer during this visit. The primary lung cancer was only identified 12 weeks later. Additionally, his adrenals showed no evidence of metastasis. Hence his adrenal insufficiency had been a paraneoplastic manifestation of the lung cancer, and it had also preceded the primary by 12 weeks.

  19. Symptomatic Adrenal Insufficiency due to Bilateral Adrenal Non-Hodgkin’s Lymphoma

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    Şafak Akın

    2016-09-01

    Full Text Available Secondary involvement of the adrenal gland with non-Hodgkin’s lymphoma has been reported to occur in up to 25% of patients during the course of the disease. A 45-year-old man presented with a several month history of nausea, fatigue and weight loss. His medical history was unremarkable. Abdominal computed tomography (CT was performed and showed bilateral adrenal massive masses measuring 10x7.5 cm on the left and 4.8x4 cm on the right. He developed adrenal insufficiency in the follow-up period. The patient was started on replacement dose of prednisolone. A positron emission tomography-CT scan was acquired for further staging of the disease and showed intense fluorodeoxyglucose accumulation in both adrenal glands, additionally a slight fluorodeoxyglucose accumulation was observed in the ileocecal site. He did not accept adrenal biopsy or surgery. Histopathological examination of the ileocecal site revealed diffuse large B-cell lymphoma. He was administered rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. Bilateral adrenal lymphoma is a rare entity when compared with the incidence of adenoma and adrenal metastases of other cancers. Adrenal insufficiency may be the primary symptom of presentation, especially with bilateral involvement as in bilateral adrenal lymphoma.

  20. Ping-pong champion with adrenal insufficiency

    OpenAIRE

    2009-01-01

    A 62-year-old Japanese man, a bronze medal winner in the World Championship of table tennis when in his 20s, was diagnosed with secondary adrenocortical insufficiency due to isolated adrenocorticotropic hormone (ACTH) deficiency, and steroid administration was started. About 1 year after the diagnosis, he took part in a table tennis championship which was open to those 40 years or older. He took 10 mg hydrocortisone after breakfast as usual, played 10 matches, each of which took 20–30 min, an...

  1. Adrenal hypoplasia congenita: a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism

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    Marta Loureiro

    2015-09-01

    Full Text Available Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG, confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

  2. Delay in Diagnosis of Adrenal Insufficiency Is a Frequent Cause of Adrenal Crisis

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    Lucyna Papierska

    2013-01-01

    Full Text Available Delay of diagnosis of primary adrenal insufficiency (PAI leads to adrenal crisis which is potentially lethal complication. The objective of our work was an assessment whether the establishment of diagnosis of adrenocortical insufficiency in Poland is so much delayed as assessed in the past. We have analysed data from 60 patients with diagnosis of PAI established in our department during the past 12 years and who are still under our care. We found that the time to diagnosis of primary adrenal insufficiency in Poland exceeds 3 months in every patient and 6 months in patients admitted with symptoms of adrenal crisis. Forty-four percent of patients were diagnosed only just after the hospitalisation due to crisis, despite the evident signs and symptoms of PAI. Lack of appetite and loss of body weight occurred in all patients and for that reason a diagnosis of chronic gastric and duodenal ulcer disease was the most often incorrect diagnosis. After the proper diagnosis and treatment, in the course of 1–11 years of observation, there was only 6 imminent adrenal crises in 5 patients. Our results indicated that training of primary care physicians in the field of recognising and treatment of adrenal insufficiency is still essential.

  3. Mifepristone Accelerates HPA Axis Recovery in Secondary Adrenal Insufficiency.

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    Cohan, Pejman

    2016-01-01

    Context. Transient secondary adrenal insufficiency (SAI) is an expected complication following successful adenomectomy of ACTH-secreting pituitary adenomas or unilateral adrenalectomy for cortisol-secreting adrenal adenomas. To date, no pharmacological therapy has been shown to hasten recovery of the hypothalamic-pituitary-adrenal (HPA) axis in this clinical scenario. Case Description. A 33-year-old woman underwent uncomplicated unilateral adrenalectomy for a 3.7 cm cortisol-secreting adrenal adenoma. Postoperatively, she developed SAI and was placed on hydrocortisone 15 mg/day, given in divided doses. In the ensuing six years, the patient's HPA axis failed to recover and she remained corticosteroid-dependent. Quarterly biochemical testing (after withholding hydrocortisone for 18 hours) consistently yielded undetectable serum cortisol and subnormal plasma ACTH levels. While she was on hydrocortisone 15 mg/day, mifepristone was initiated and gradually titrated to a maintenance dose of 600 mg/day after 5 months. Rapid recovery of the HPA axis was subsequently noted with ACTH rising into the supranormal range at 4 months followed by a subsequent rise in cortisol levels into the normal range. After 6 months, the dose of hydrocortisone and mifepristone was lowered and both were ultimately stopped after 8 months. The HPA axis remains normal after an additional 16 months of follow-up. Conclusion. Mifepristone successfully restored the HPA axis in a woman with prolonged secondary adrenal insufficiency (SAI) after adrenalectomy for Cushing's syndrome (CS).

  4. Adrenal insufficiency in a child with MELAS syndrome.

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    Afroze, Bushra; Amjad, Nida; Ibrahim, Shahnaz H; Humayun, Khadija Nuzhat; Yakob, Yusnita

    2014-11-01

    Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) are established subgroups of mitochondrial encephalomyopathy. m.3243A>G a common point mutation is detected in tRNA in majority of patients with MELAS phenotype whereas m.8344A>G point mutation in tRNA is observed, in MERRF phenotype. Adrenal insufficiency has not been reported in mitochondrial disease, except in Kearns-Sayre Syndrome (KSS), which is a mitochondrial deletion syndrome. We report an unusual presentation in a five year old boy who presented with clinical phenotype of MELAS and was found to have m.8344A>G mutation in tRNA. Addison disease was identified due to hyperpigmentation of lips and gums present from early childhood. This is the first report describing adrenal insufficiency in a child with MELAS phenotype.

  5. Update on adrenal insufficiency in patients with liver cirrhosis.

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    Trifan, Anca; Chiriac, Stefan; Stanciu, Carol

    2013-01-28

    Liver cirrhosis is a major cause of mortality worldwide, often with severe sepsis as the terminal event. Over the last two decades, several studies have reported that in septic patients the adrenal glands respond inappropriately to stimulation, and that the treatment with corticosteroids decreases mortality in such patients. Both cirrhosis and septic shock share many hemodynamic abnormalities such as hyperdynamic circulatory failure, decreased peripheral vascular resistance, increased cardiac output, hypo-responsiveness to vasopressors, increased levels of proinflammatory cytokines [interleukine(IL)-1, IL-6, tumor necrosis factor-alpha] and it has, consequently, been reported that adrenal insufficiency (AI) is common in critically ill cirrhotic patients. AI may also be present in patients with stable cirrhosis without sepsis and in those undergoing liver transplantation. The term hepato-adrenal syndrome defines AI in patients with advanced liver disease with sepsis and/or other complications, and it suggests that it could be a feature of liver disease per se, with a different pathogenesis from that of septic shock. Relative AI is the term given to inadequate cortisol response to stress. More recently, another term is used, namely "critical illness related corticosteroid insufficiency" to define "an inadequate cellular corticosteroid activity for the severity of the patient's illness". The mechanisms of AI in liver cirrhosis are not completely understood, although decreased levels of high-density lipoprotein cholesterol and high levels of proinflammatory cytokines and circulatory endotoxin have been suggested. The prevalence of AI in cirrhotic patients varies widely according to the stage of the liver disease (compensated or decompensated, with or without sepsis), the diagnostic criteria defining AI and the methodology used. The effects of corticosteroid therapy on cirrhotic patients with septic shock and AI are controversial. This review aims to summarize the

  6. Guidelines regarding management of adrenal insufficiency in the Holy month of Ramadan

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    Sheelu S Siddiqi

    2012-01-01

    Full Text Available Adrenal insufficiency is a life-threatening event, so it is recommended for patients with known adrenal insufficiency to be properly educated regarding sick-day management. In the month of Ramadan, people refrain from eating and drinking during daylight hours. It is very important for patients with adrenal insufficiency, who wish to keep a fast, to be well aware of the disease, the suitable drug to be used for that particular period, warning signs, sick-day management, physical activity, and dietary limits. This article describes guidelines for the sick-day management of patients with adrenal insufficiency, in the month of Ramadan.

  7. Approach to the handling of adrenal insufficiency Manejo de la insuficiencia adrenal

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    Federico Uribe Londoño

    2005-03-01

    Full Text Available The term adrenal insufficiency refers to the hypofunction of this gland. From the etiologic point of view it may be either primary or secondary. This insufficiency manifests as inadequate serum levels of cortisol and adrenal androgens in the secondary form and of these and mineralocorticoids in the primary one. Clinical manifestations are often nonspecific and, consequently, diagnosis may be difficult to establish and treatment may be delayed with increased morbidity and mortality. This article on adrenal insufficiency includes its definition, epidemiology, etiology, physiopathology, classification, clinical presentation, diagnostic criteria and treatment guidelines. Besides, some special situations like critically ill patients and pregnant women are given special consideration. Emphasis is done on a diagnostic algorithm to make it easier for general practitioners the approach to patients with this endocrine disorder. La insuficiencia adrenal (ia se refiere a la hipofunción de dicha glándula debida a causas tanto primarias como secundarias, que resultan en niveles plasmáticos inadecuados de cortisol, andrógenos adrenales y adicionalmente, en la falla primaria de mineralocorticoides. Sus manifestaciones inespecíficas dificultan o retrasan con frecuencia el diagnóstico y tratamiento oportuno, lo cual incrementa la morbilidad y eventualmente la mortalidad de estos pacientes. Se incluyen en este artículo: definición, epidemiología, causas, fisiopatología, clasificación, manifestaciones clínicas, diagnóstico y tratamiento de la IA. Además se consideran algunas situaciones especiales como la IA en el paciente críticamente enfermo y en la mujer gestante. Finalmente se hace especial énfasis en un algoritmo diagnóstico, con la finalidad de facilitarle al médico general un enfoque ágil y oportuno de los pacientes con este problema endocrino.

  8. Acute adrenal insufficiency: an aide-memoire of the critical importance of its recognition and prevention.

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    Gargya, A; Chua, E; Hetherington, J; Sommer, K; Cooper, M

    2016-03-01

    Adrenal crisis is a life-threatening emergency that causes significant excess mortality in patients with adrenal insufficiency. Delayed recognition by medical staff of an impending adrenal crisis and failure to give timely hydrocortisone therapy within the emergency department continue to be commonly encountered, even in metropolitan teaching hospitals. Within the authors' institutions, several cases of poorly handled adrenal crises have occurred over the last 2 years. Anecdotal accounts from members of the Addison's support group suggest that these issues are common in Australia. This manuscript is a timely reminder for clinical staff on the critical importance of the recognition, treatment and prevention of adrenal crisis. The manuscript: (i) outlines a case and the clinical outcome of sub-optimally managed adrenal crisis, (ii) summarises the clinical features and acute management of adrenal crisis, (iii) provides recommendations on the prevention of adrenal crisis and (iv) provides guidance on the management of 'sick days' in patients with adrenal insufficiency.

  9. Neurosarcoidosis-associated central diabetes insipidus masked by adrenal insufficiency.

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    Non, Lemuel; Brito, Daniel; Anastasopoulou, Catherine

    2015-01-22

    Central diabetes insipidus (CDI) is an infrequent complication of neurosarcoidosis (NS). Its presentation may be masked by adrenal insufficiency (AI) and uncovered by subsequent steroid replacement. A 45-year-old woman with a history of NS presented 2 weeks after abrupt cessation of prednisone with nausea, vomiting, decreased oral intake and confusion. She was diagnosed with secondary AI and intravenous hydrocortisone was promptly begun. Over the next few days, however, the patient developed severe thirst and polyuria exceeding 6 L of urine per day, accompanied by hypernatraemia and hypo-osmolar urine. She was presumed to have CDI due to NS, and intranasal desmopressin was administered. This eventually normalised her urine output and serum sodium. The patient was discharged improved on intranasal desmopressin and oral prednisone. AI may mask the manifestation of CDI because low serum cortisol impairs renal-free water clearance. Steroid replacement reverses this process and unmasks an underlying CDI.

  10. [Content of biological antioxidants and lipid peroxidation in the adrenal cortex in experimental circulatory insufficiency].

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    Doroshkevich, N A; Antsulevich, S N; Nadol'nik, L I; Naumov, A V; Beluga, V B; Vinogradov, V V

    1991-01-01

    Thirteen-week experimental insufficiency in rats causes exhaustion of adrenal cortex function. The number of diene conjugates in the adrenals increased, the alpha-tocopherol level decreased. It is suggested that reduced level of antioxidant protection and the associated activation of lipid peroxidation processes underlie the disorders of adrenal cortex function.

  11. Community acquired bilateral upper lobe Pneumonia with acute adrenal insufficiency: A new face of Achromobacter xylosoxidans

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    Suman S Karanth

    2012-05-01

    Full Text Available AbstractAchromobacter xylosoxidans is an uncommon pathogen of low virulence known to cause serious nosocomial infection in the immunocompromised. Its inherent multi-drug resistance makes treatment difficult. Community-acquired infections are rare despite its ubiquitous existence. We present a 50-year-old immunocompetent woman who presented with one-month history of coughing with expectoration who was subsequently diagnosed with bilateral upper lobe pneumonia and acute adrenal insufficiency. Achromobacter xylosoxidans was isolated from sputum and bronchoalveolar lavage culture. The acute adrenal insufficiency recovered after appropriate antibiotic therapy. Amongst the myriad of presentations, we highlight the rarity of acute adrenal insufficiency triggered by the infection.

  12. Community acquired bilateral upper lobe pneumonia with acute adrenal insufficiency: A new face of Achromobacter Xylosoxidans.

    Science.gov (United States)

    Karanth, Suman S; Gupta, Anurag; Prabhu, Mukhyaprana

    2012-01-01

    Achromobacter xylosoxidans is an uncommon pathogen of low virulence known to cause serious nosocomial infection in the immunocompromised. Its inherent multi-drug resistance makes treatment difficult. Community-acquired infections are rare despite its ubiquitous existence. We present a 50-year-old immunocompetent woman who presented with one-month history of coughing with expectoration who was subsequently diagnosed with bilateral upper lobe pneumonia and acute adrenal insufficiency. Achromobacter xylosoxidans was isolated from sputum and bronchoalveolar lavage culture. The acute adrenal insufficiency recovered after appropriate antibiotic therapy. Amongst the myriad of presentations, we highlight the rarity of acute adrenal insufficiency triggered by the infection.

  13. Duration of adrenal insufficiency during treatment for childhood acute lymphoblastic leukemia

    DEFF Research Database (Denmark)

    Vestergaard, Therese Risom; Juul, Anders; Lausten-Thomsen, Ulrik;

    2011-01-01

    Children with acute lymphoblastic leukemia (ALL) recive high doses of glucocorticosteroid as part of their treatment. This may lead to suppression of the hypothalamic-pituitary-adrenal axis, acute adrenal insufficiency, and ultimately to life-threatening conditions. This study explores the adrena...

  14. Role of toll-like receptors and inflammation in adrenal gland insufficiency.

    Science.gov (United States)

    Kanczkowski, Waldemar; Zacharowski, Kai; Bornstein, Stefan R

    2010-01-01

    Adrenal gland insufficiency - the clinical manifestation of deficient production or action of adrenal steroids - is a life-threatening disorder. Among many factors which can predispose to primary adrenal failure, an autoimmune adrenalitis and infectious agents play a major role. The initial host defense against bacterial infections is executed primarily by the pattern recognition receptors, e.g. Toll-like receptors (TLRs), expressed in cells from the innate immune system. Upon activation, TLRs have been found to regulate various levels of innate and adaptive immunity as well as control tissue inflammation. TLRs are implicated in adrenal cell turnover and steroidogenesis during inflammation. Therefore, TLRs play a crucial role in the activation of adrenal inflammation mediating adrenal gland dysfunction during septicemia.

  15. Metabolic control in patients with type 1 diabetes mellitus at the onset of primary adrenal insufficiency

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    A A Larina

    2013-06-01

    Full Text Available Primary adrenal insufficiency is a rare disorder but it is more common in patients with type 1 diabetes mellitus (T1DM or autoimmune thyroid diseases than in the general population. T1DM may precede the development of adrenocortical insufficiency. Addison’s disease can influence the glycemic control in patients with T1DM worsening glucose metabolism. It causes the decrease of gluconeogenesis, reduction in total insulin requirement and rather often «unexplained» recurrent hypoglycemia. Glucocorticoid replacement therapy in patients with primary adrenal insufficiency and T1DM increases the insulin requirement. The article presents the development of primary adrenal insufficiency in a patient with type 1 DM and autoimmune hypothyroidism, accompanied with incidents of recurrent hypoglycemia.

  16. Adrenal Insufficiency as a Cause of Acute Liver Failure: A Case Report

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    Jamshid Vafaeimanesh

    2013-01-01

    Full Text Available Introduction. Many diseases and conditions can contribute to elevated liver enzymes. Common causes include viral and autoimmune hepatitis, fatty liver, and bile duct diseases, but, in uncommon cases like liver involvement in endocrine disorders, liver failure is also seen. Adrenal insufficiency is the rarest endocrine disorder complicating the liver. In the previously reported cases of adrenal insufficiency, mild liver enzymes elevation was seen but we report a case with severe elevated liver enzymes and liver failure due to adrenal insufficiency. Based on our knowledge, this is the first report in this field. Case Report. A 39-year-old woman was referred to emergency ward due to drowsiness and severe fatigue. Her laboratory tests revealed prothrombin time: 21 sec, alanine aminotransferase (ALT: 2339 IU/L, aspartate aminotransferase (AST: 2002 IU/L, and ALP: 90 IU/L. No common cause of liver involvement was discovered, and eventually, with diagnosis of adrenal insufficiency and corticosteroid therapy, liver enzymes and function became normal. Finally, the patient was discharged with good general condition. Conclusion. With this report, we emphasize adrenal insufficiency (primary or secondary as a reason of liver involvement in unexplainable cases and recommend that any increase in the liver enzymes, even liver failure, in these patients should be observed.

  17. Clinical features of congenital adrenal insufficiency including growth patterns and significance of ACTH stimulation test.

    Science.gov (United States)

    Koh, Ji Won; Kim, Gu Hwan; Yoo, Han Wook; Yu, Jeesuk

    2013-11-01

    Congenital adrenal insufficiency is caused by specific genetic mutations. Early suspicion and definite diagnosis are crucial because the disease can precipitate a life-threatening hypovolemic shock without prompt treatment. This study was designed to understand the clinical manifestations including growth patterns and to find the usefulness of ACTH stimulation test. Sixteen patients with confirmed genotyping were subdivided into three groups according to the genetic study results: congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH, n=11), congenital lipoid adrenal hyperplasia (n=3) and X-linked adrenal hypoplasia congenita (n=2). Bone age advancement was prominent in patients with CAH especially after 60 months of chronologic age (n=6, 67%). They were diagnosed in older ages in group with bone age advancement (Pcongenital adrenal insufficiency. ACTH stimulation test played an important role to support the diagnosis and serum 17-hydroxyprogesterone levels were significantly elevated in all of the CAH patients. The test will be important for monitoring growth and puberty during follow up of patients with congenital adrenal insufficiency.

  18. Pituitary Adenoma as a Rare Form of Secondary Adrenal Insufficiency. A Case Report

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    Ana María León Blasco

    2014-02-01

    Full Text Available Pituitary adenomas are benign tumors arising from one of the five cell types in the anterior pituitary. Secondary adrenal insufficiency occurs when the adrenal glands do not produce hormones due to the lack of pituary adrenocorticotropic hormone or hypothalamic corticotropin-releasing factor. The case of a 37-year-old female patient who started developing muscle twitching in legs and arms, muscular weakness, fatigue, muscle aches, severe and throbbing hemicrania on the right side, among other symptoms, a year ago is presented. Several tests were performed (cortisol level, adrenocorticotropic hormone, calcium and phosphorus in blood and urine, magnetic resonance imaging of the skull, leading to the diagnosis of pituitary adenoma with isolated adrenocorticotropic hormone deficiency and secondary adrenal insufficiency. Treatment with steroids and calcium supplements ensured a satisfactory recovery.

  19. Prednisolone is associated with a worse lipid profile than hydrocortisone in patients with adrenal insufficiency

    NARCIS (Netherlands)

    Quinkler, Marcus; Ekman, Bertil; Marelli, Claudio; Uddin, Sharif; Zelissen, Pierre; Murray, Robert

    2017-01-01

    OBJECTIVE: Prednisolone is used as glucocorticoid replacement therapy for adrenal insufficiency (AI). Recent data indicate that its use in AI is associated with low bone mineral density. Data on risk factors for cardiovascular disease in patients with AI treated with prednisolone are scarce, despite

  20. The relative adrenal insufficiency syndrome revisited : which patients will benefit from low-dose steroids?

    NARCIS (Netherlands)

    Ligtenberg, Jack J M; Zijlstra, Jan G

    2004-01-01

    PURPOSE OF REVIEW: Several clinical studies have given rise to optimism about low-dose steroid treatment in patients with sepsis. It reduces time to shock reversal and may even have a positive effect on mortality. The pathophysiology of the relative adrenal insufficiency syndrome has not yet been de

  1. Adrenal Insufficiency under Standard Dosage of Glucocorticoid Replacement after Unilateral Adrenalectomy for Cushing’s Syndrome

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    Kentaro Fujii

    2016-01-01

    Full Text Available Glucocorticoid replacement is needed for patients after adrenal surgery for Cushing’s syndrome; however, the adequate dosage is not easily determined. The patient was a 62-year-old woman who has had hypertension for 5 years and presented with heart failure due to hypertrophic cardiomyopathy. She consulted with us because of general fatigue, facial edema, and muscle weakness and was diagnosed with Cushing’s syndrome. A laparoscopic left adrenalectomy was performed, standard dosage of postoperative replacement was administered, and she was discharged with 30 mg/day of hydrocortisone (cortisol. However, she suffered from loss of appetite and was transferred to an emergency unit with the symptoms of adrenal insufficiency on postoperative day 15. After initial hydrocortisone replacement with 200 mg/day, the dosage was gradually decreased during hospitalization; however, reduction of hydrocortisone dosage lower than 60 mg/day was difficult because of nausea and fatigue. Her circadian cortisol profile after hydrocortisone administration showed delayed and lowered peaks, which suggested that hydrocortisone absorption in the intestine was impaired. Therefore, complicated heart failure may have led to the adrenal insufficiency in the patient. In such cases, we should consider postoperative administration of more than the standard dosage of hydrocortisone to avoid adrenal insufficiency after surgery for Cushing’s syndrome.

  2. Sphingosine-1-phosphate lyase mutations cause primary adrenal insufficiency and steroid-resistant nephrotic syndrome

    Science.gov (United States)

    Prasad, Rathi; Hadjidemetriou, Irene; Meimaridou, Eirini; Buonocore, Federica; Saleem, Moin; Hurcombe, Jenny; Bierzynska, Agnieszka; Barbagelata, Eliana; Bergadá, Ignacio; Cassinelli, Hamilton; Das, Urmi; Krone, Ruth; Hacihamdioglu, Bulent; Sari, Erkan; Yesilkaya, Ediz; Storr, Helen L.; Clemente, Maria; Fernandez-Cancio, Monica; Camats, Nuria; Ram, Nanik; Achermann, John C.; Van Veldhoven, Paul P.; Guasti, Leonardo; Braslavsky, Debora; Guran, Tulay; Metherell, Louise A.

    2017-01-01

    Primary adrenal insufficiency is life threatening and can present alone or in combination with other comorbidities. Here, we have described a primary adrenal insufficiency syndrome and steroid-resistant nephrotic syndrome caused by loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1). SGPL1 executes the final decisive step of the sphingolipid breakdown pathway, mediating the irreversible cleavage of the lipid-signaling molecule sphingosine-1-phosphate (S1P). Mutations in other upstream components of the pathway lead to harmful accumulation of lysosomal sphingolipid species, which are associated with a series of conditions known as the sphingolipidoses. In this work, we have identified 4 different homozygous mutations, c.665G>A (p.R222Q), c.1633_1635delTTC (p.F545del), c.261+1G>A (p.S65Rfs*6), and c.7dupA (p.S3Kfs*11), in 5 families with the condition. In total, 8 patients were investigated, some of whom also manifested other features, including ichthyosis, primary hypothyroidism, neurological symptoms, and cryptorchidism. Sgpl1–/– mice recapitulated the main characteristics of the human disease with abnormal adrenal and renal morphology. Sgpl1–/– mice displayed disrupted adrenocortical zonation and defective expression of steroidogenic enzymes as well as renal histology in keeping with a glomerular phenotype. In summary, we have identified SGPL1 mutations in humans that perhaps represent a distinct multisystemic disorder of sphingolipid metabolism. PMID:28165343

  3. Diabetes insipidus and adrenal insufficiency in a patient with metastatic breast cancer.

    Science.gov (United States)

    Netelenbos, T; Nooij, M A; Nortier, J W R

    2006-09-01

    A patient previously treated for bilateral breast cancer with mastectomy, radiation therapy and in remission on hormonal therapy for more than five years presented with abdominal symptoms from breast cancer relapse. She developed inappropriate polyuria and hypernatraemia, which responded to desmopressin. In combination with the absence of a high signal from the posterior lobe of the pituitary on MRI , these data indicated the presence of partial central diabetes insipidus. The anterior pituitary showed partial failure (low follicle-stimulating hormone, luteinising hormone and insulin-like growth factor-1 levels). Furthermore, primary adrenal insufficiency had developed, ascribed to bilateral tumour invasion of the adrenals. This rare combination of endocrinological failures in a patient with metastatic breast cancer is discussed.

  4. Prednisolone is associated with a worse lipid profile than hydrocortisone in patients with adrenal insufficiency

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    Marcus Quinkler

    2016-12-01

    Full Text Available Objective: Prednisolone is used as glucocorticoid replacement therapy for adrenal insufficiency (AI. Recent data indicate that its use in AI is associated with low bone mineral density. Data on risk factors for cardiovascular disease in patients with AI treated with prednisolone are scarce, despite this condition being the predominant cause of excess mortality. We aimed to address this question using real-world data from the European Adrenal Insufficiency Registry (EU-AIR. Design/methods: EU-AIR, comprising of 19 centres across Germany, the Netherlands, Sweden and the UK, commenced enrolling patients with AI in August 2012. Patients receiving prednisolone (3–6 mg/day, n = 50 or hydrocortisone (15–30 mg/day, n = 909 were identified and grouped at a ratio of 1:3 (prednisolone:hydrocortisone by matching for gender, age, duration and type of disease. Data from baseline and follow-up visits were analysed. Data from patients with congenital adrenal hyperplasia were excluded. Results: Significantly higher mean ± s.d. total (6.3 ± 1.6 vs 5.4 ± 1.1 mmol/L; P = 0.003 and low-density lipoprotein (LDL cholesterol levels (3.9 ± 1.4 vs 3.2 ± 1.0 mmol/L; P = 0.013 were identified in 47 patients on prednisolone vs 141 receiving hydrocortisone at baseline and at follow-up (P = 0.005 and P = 0.006, respectively. HbA1c, high-density lipoprotein and triglyceride levels, body mass index, systolic and diastolic blood pressure and waist circumference were not significantly different. Conclusions: This is the first matched analysis of its kind. Significantly higher LDL levels in patients receiving prednisolone relative to hydrocortisone could predict a higher relative risk of cardiovascular disease in the former group.

  5. Insuficiência adrenal na criança com choque séptico Adrenal insufficiency in children with septic shock

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    Carlos H. Casartelli

    2003-11-01

    for diagnosing and treating adrenal insufficiency in patients with septic shock. SOURCES OF DATA: Articles published in Brazilian and foreign journals selected through these publications' websites and Medline, as well as references cited in key articles. SUMMARY OF THE FINDINGS: The literature reports a range betwen 17 and 54 % for the finding of adrenal insufficiency in patients with septic shock. There is no consensus for diagnosing adrenal insufficiency in patients suffering from critical diseases, particularly in patients with septic shock. The presence of volume-refractory and catecholamine-resistant septic shock suggests this condition, while basal cortisol under 25 µg/dl is a diagnostic criterion indicating adrenal insufficiency. The adrenal stimulation test is a useful resource for identifying patients with relative adrenal insufficiency. Our testing option for adrenal stimulation in children is the use of corticotropin in low doses (0.5 µg/1,73 m². An increase of less than 9 µg/dl in the value of postcorticotropin-stimulated cortisol suggests the presence of occult (relative adrenal insufficiency. In patients with septic shock presenting adrenal insufficiency, either suspected or confirmed, the administration of hydrocortisone in shock or stress doses can be vital for a favorable clinical outcome. CONCLUSIONS: The existing data, although controversial, already provides a basis to determine when to begin hormone replacement therapy, the serum level of cortisol accepted as adequate, and the choice of corticotropin doses for performing the adrenal stimulation test and diagnosing occult or relative adrenal insufficiency in patients with septic shock.

  6. Clinical Characteristics of Patients with Adrenal Insufficiency in a General Hospital

    Science.gov (United States)

    Lee, Ye Yeon; Cho, Nan Hee; Lee, Jong Won; Kim, Nam Kyung; Kim, Hye Soon

    2017-01-01

    Background Adrenal insufficiency (AI) is a life-threatening disorder caused by the deficiency of adrenal steroid hormones. This retrospective cross-sectional study investigated the characteristics of patients with AI in Korea. Methods All consecutive patients with suspected AI who received care at a tertiary referral center in Korea in 2014 and underwent adrenocorticotropic hormone stimulation or insulin-tolerance testing were identified through a review of medical charts. Patients diagnosed with AI were enrolled. Their demographic, clinical, and treatment details were extracted. Results Of 771 patients with suspected AI, 183 (23.7%) received a definitive diagnosis. The most common reason for testing was the presence of suspicious AI-related symptoms (30.0%), followed by a history of steroid medications (23.5%). Their mean age was 66.7 years, and females predominated (67.8%). The most common symptoms were general weakness, anorexia, arthralgia, and fever. Approximately half (53.6%) had a history of steroid use. Hydrocortisone was the most common treatment (71.6%), with most patients taking a 30 mg dose (44.2%). The most common dose frequency was twice a day (78.6%). Fourteen patients were treated for adrenal crisis (n=10, 5.5%) or an intercurrent illness (n=4, 2.2%). Conclusion AI may have been caused by steroid medication use in many of the patients included in this study. The detection of AI can be improved by careful history-taking and being alert to the possibility that a patient has used steroids. PMID:28256113

  7. Hypopituitarism Presenting as Adrenal Insufficiency and Hypothyroidism in a Patient with Wilson's Disease: a Case Report.

    Science.gov (United States)

    Lee, Hae Won; Kang, Jin Du; Yeo, Chang Woo; Yoon, Sung Woon; Lee, Kwang Jae; Choi, Mun Ki

    2016-08-01

    Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.

  8. A Case of Rathke’s Cleft Cyst Associated with Transient Central Adrenal Insufficiency and Masked Diabetes Insipidus

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    Masahiro Asakawa

    2014-01-01

    Full Text Available A 73-year-old woman admitted to our hospital because of headache, poor appetite, malaise, weight loss, and vomiting was found to have central adrenal insufficiency and thyrotoxicosis due to silent thyroiditis. Polyuria developed after replacement with glucocorticoid (masked diabetes insipidus, which was controlled with nasal administration of desmopressin. Magnetic resonance imaging of the brain showed a large cystic pituitary mass (18 × 18 × 12 mm extending suprasellarly to the optic chiasm. Transsphenoidal surgery revealed that the pituitary tumor was Rathke’s cleft cyst. Following surgery, replacement with neither glucocorticoid nor desmopressin was needed any more. Therefore, it is suggested that Rathke’s cleft cyst is responsible for the masked diabetes insipidus and the central insufficiency. Furthermore, it is speculated that thyrotoxicosis with painless thyroiditis might induce changes from subclinical adrenal insufficiency to transiently overt insufficiency.

  9. Prednisolone is associated with a worse lipid profile than hydrocortisone in patients with adrenal insufficiency

    Science.gov (United States)

    Ekman, Bertil; Marelli, Claudio; Uddin, Sharif; Zelissen, Pierre; Murray, Robert D

    2017-01-01

    Objective Prednisolone is used as glucocorticoid replacement therapy for adrenal insufficiency (AI). Recent data indicate that its use in AI is associated with low bone mineral density. Data on risk factors for cardiovascular disease in patients with AI treated with prednisolone are scarce, despite this condition being the predominant cause of excess mortality. We aimed to address this question using real-world data from the European Adrenal Insufficiency Registry (EU-AIR). Design/methods EU-AIR, comprising of 19 centres across Germany, the Netherlands, Sweden and the UK, commenced enrolling patients with AI in August 2012. Patients receiving prednisolone (3–6 mg/day, n = 50) or hydrocortisone (15–30 mg/day, n = 909) were identified and grouped at a ratio of 1:3 (prednisolone:hydrocortisone) by matching for gender, age, duration and type of disease. Data from baseline and follow-up visits were analysed. Data from patients with congenital adrenal hyperplasia were excluded. Results Significantly higher mean ± s.d. total (6.3 ± 1.6 vs 5.4 ± 1.1 mmol/L; P = 0.003) and low-density lipoprotein (LDL) cholesterol levels (3.9 ± 1.4 vs 3.2 ± 1.0 mmol/L; P = 0.013) were identified in 47 patients on prednisolone vs 141 receiving hydrocortisone at baseline and at follow-up (P = 0.005 and P = 0.006, respectively). HbA1c, high-density lipoprotein and triglyceride levels, body mass index, systolic and diastolic blood pressure and waist circumference were not significantly different. Conclusions This is the first matched analysis of its kind. Significantly higher LDL levels in patients receiving prednisolone relative to hydrocortisone could predict a higher relative risk of cardiovascular disease in the former group. PMID:27864317

  10. [SF-1, a key player in adrenal and gonadal differentiation: implications in gonadal dysgenesis and primary ovarian insufficiency].

    Science.gov (United States)

    Martinerie, L; Bouvattier, C; Lombes, M

    2009-09-01

    Steroidogenic factor 1 (SF-1) gene, identified by Keith Parker in 1992, encodes for an orphan nuclear receptor, NR5A1, whose expression is detected during fetal life in adrenal and gonadal steroidogenic tissues, but also in the developing hypothalamus and in pituitary gonadotropic cells. SF-1 knock-out mouse models exhibit complete adrenal and gonadal agenesis. Human mutations of this transcription factor, were initially associated with primary adrenal failure and male gonadal dysgenesis with various degrees of under androgenization. More recently, identification of novel SF-1 mutations responsible for isolated 46, XY gonadal dysgenesis or 46, XX primary ovarian insufficiency, underscores its central role in the control and maintenance of adrenal and reproductive functions. A better understanding in the regulatory mechanisms of SF-1 signaling pathway, will open new avenues for diagnostic and therapeutic managements of sex differentiation disorders and infertilities.

  11. Current practice of glucocorticoid replacement therapy and patient-perceived health outcomes in adrenal insufficiency - a worldwide patient survey

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    Forss M

    2012-06-01

    Full Text Available Abstract Background The aim was to survey current practice in glucocorticoid replacement therapy and self-perceived health outcomes in patients with adrenal insufficiency. Methods Participants were recruited via patient organizations to respond anonymously to a web-based survey developed by clinical experts. Unique entries were set up for each patient organization enabling geographical localization of the entries. Results 1245 participants responded (primary adrenal insufficiency: 84%; secondary adrenal insufficiency: 11%; unsure: 5%. Therapies included hydrocortisone (75%, prednisone/prednisolone (11%, cortisone acetate (6% and dexamethasone (4%. Dosing regimens were once daily (10%, twice daily (42%, thrice daily (32% or other (17%. Compromised subjective health necessitating changes to physical activity or social-, work- or family life was reported by 64% of the participants. 40% of the participants reported absence from work/school in the last 3 months. Irrespective of diagnosis, 76% were concerned about long-term side-effects of therapy, mainly osteoporosis (78%, obesity (64% and cardiovascular morbidity (46%. 38% of the participants had been hospitalized in the last year. Conclusions Glucocorticoid replacement therapy among the respondents consisted primarily of hydrocortisone administered twice or thrice daily. A majority reported impact of their disease or treatment on subjective health requiring alterations in e.g. physical activity or family life. Three quarters reported concerns about long-term side-effects of the treatment. These data demonstrate - from the patients' perspective - a need for improvement in the management of adrenal insufficiency.

  12. Brain ultrasonographic findings of late-onset circulatory dysfunction due to adrenal insufficiency in preterm infants

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    Shin, Su Mi; Chai, Jee Won [Dept. of Radiology, SMG-SNU Boramae Medical Center, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2016-07-15

    The aim of this study was to characterize the brain ultrasonographic findings of late-onset circulatory dysfunction (LCD) due to adrenal insufficiency (AI) in preterm infants. Among the 257 preterm infants born at <33 weeks of gestation between December 2009 and February 2014 at our institution, 35 preterm infants were diagnosed with AI. Brain ultrasonographic findings were retrospectively analyzed before and after LCD in 14 preterm infants, after exclusion of the other 21 infants with AI due to the following causes: death (n=2), early AI (n=5), sepsis (n=1), and patent ductus arteriosus (n=13). Fourteen of 257 infants (5.4%) were diagnosed with LCD due to AI. The age at LCD was a median of 18.5 days (range, 9 to 32 days). The last ultrasonographic findings before LCD occurred showed grade 1 periventricular echogenicity (PVE) in all 14 patients and germinal matrix hemorrhage (GMH) with focal cystic change in one patient. Ultrasonographic findings after LCD demonstrated no significant change in grade 1 PVE and no new lesions in eight (57%), grade 1 PVE with newly appearing GMH in three (21%), and increased PVE in three (21%) infants. Five infants (36%) showed new development (n=4) or increased size (n=1) of GMH. Two of three infants (14%) with increased PVE developed cystic periventricular leukomalacia (PVL) and rapid progression to macrocystic encephalomalacia. LCD due to AI may be associated with the late development of GMH, increased PVE after LCD, and cystic PVL with rapid progression to macrocystic encephalomalacia.

  13. Sideroblastic anaemia and primary adrenal insufficiency due to a mitochondrial respiratory chain disorder in the absence of mtDNA deletion.

    Science.gov (United States)

    O'Grady, Michael J; Monavari, Ahmad A; Cotter, Melanie; Murphy, Nuala P

    2015-02-26

    A fatigued 8-year-old boy was found to have sideroblastic anaemia (haemoglobin 7.8 g/dL) which over time became transfusion dependent. Subtle neurological dysfunction, initially manifesting as mild spastic diplegia, was slowly progressive and ultimately led to wheelchair dependence. Elevated plasma lactate and urinary 3-methylglutaconate led to a muscle biopsy which confirmed partial complex IV deficiency. PCR in leucocytes and muscle was negative for mitochondrial DNA (mtDNA) deletions. Faltering growth prompted an insulin tolerance test which confirmed growth hormone sufficiency and adrenal insufficiency. Plasma renin was elevated and adrenal androgens were low, suggesting primary adrenal insufficiency. Glucocorticoid and mineralocorticoid replacement therapy was initiated. A renal tubular Fanconi syndrome and diabetes mellitus developed subsequently. Sideroblastic anaemia and primary adrenal insufficiency, both individually and collectively, are associated with mtDNA deletion; however, absence of the same does not exclude the possibility that sideroblastic anaemia and primary adrenal insufficiency are of mitochondrial origin.

  14. Brain ultrasonographic findings of late-onset circulatory dysfunction due to adrenal insufficiency in preterm infants

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    Su-Mi Shin

    2016-07-01

    Full Text Available Purpose: The aim of this study was to characterize the brain ultrasonographic findings of late-onset circulatory dysfunction (LCD due to adrenal insufficiency (AI in preterm infants. Methods: Among the 257 preterm infants born at <33 weeks of gestation between December 2009 and February 2014 at our institution, 35 preterm infants were diagnosed with AI. Brain ultrasonographic findings were retrospectively analyzed before and after LCD in 14 preterm infants, after exclusion of the other 21 infants with AI due to the following causes: death (n=2, early AI (n=5, sepsis (n=1, and patent ductus arteriosus (n=13. Results: Fourteen of 257 infants (5.4% were diagnosed with LCD due to AI. The age at LCD was a median of 18.5 days (range, 9 to 32 days. The last ultrasonographic findings before LCD occurred showed grade 1 periventricular echogenicity (PVE in all 14 patients and germinal matrix hemorrhage (GMH with focal cystic change in one patient. Ultrasonographic findings after LCD demonstrated no significant change in grade 1 PVE and no new lesions in eight (57%, grade 1 PVE with newly appearing GMH in three (21%, and increased PVE in three (21% infants. Five infants (36% showed new development (n=4 or increased size (n=1 of GMH. Two of three infants (14% with increased PVE developed cystic periventricular leukomalacia (PVL and rapid progression to macrocystic encephalomalacia. Conclusion: LCD due to AI may be associated with the late development of GMH, increased PVE after LCD, and cystic PVL with rapid progression to macrocystic encephalomalacia.

  15. Multidrug-Resistant Tuberculosis and Its Association with Adrenal Insufficiency: Assessment with the Low-Dose ACTH Stimulation Test

    Science.gov (United States)

    Rodríguez-Gutiérrez, René; Rendon, Adrian; Barrera-Sánchez, Maximiliano; Carlos-Reyna, Kevin Erick Gabriel; Álvarez-Villalobos, Neri Alejandro; González-Saldivar, Gloria; González-González, José Gerardo

    2016-01-01

    Background. Multidrug-resistant tuberculosis (MDR-TB) is a major public health care concern that affects the life of millions of people around the world. The association of tuberculosis and adrenal insufficiency is well known; however, it is thought to be less prevalent every time. A spike in TB incidence and a lack of evidence of this association in patients with MDR-TB call for reassessment of an illness (adrenal dysfunction) that if not diagnosed could seriously jeopardize patients' health. Objective. To determine the prevalence of adrenocortical insufficiency in patients with MDR-TB using the low-dose (1 μg) ACTH stimulation test at baseline and at 6–12 months of follow-up after antituberculosis treatment and culture conversion. Methods. A total of 48 men or women, aged ≥18 years (HIV-negative patients diagnosed with pulmonary MDR-TB) were included in this prospective observational study. Blood samples for serum cortisol were taken at baseline and 30 and 60 minutes after 1 μg ACTH stimulation at our tertiary level university hospital before and after antituberculosis treatment. Results. Forty-seven percent of subjects had primary MDR-TB; 43.8% had type 2 diabetes; none were HIV-positive. We found at enrollment 2 cases (4.2%) of adrenal insufficiency taking 500 nmol/L as the standard cutoff point value and 4 cases (8.3%) alternatively, using 550 nmol/L. After antituberculosis intensive phase drug-treatment and a negative mycobacterial culture (10.2 ± 3.6 months) adrenocortical function was restored in all cases. Conclusions. In patients with MDR-TB, using the low-dose ACTH stimulation test, a low prevalence of mild adrenal insufficiency was observed. After antituberculosis treatment adrenal function was restored in all cases. Given the increasing and worrying epidemic of MDR-TB these findings have important clinical implications that may help clinicians and patients make better decisions when deciding to test for adrenocortical dysfunction or

  16. Multidrug-Resistant Tuberculosis and Its Association with Adrenal Insufficiency: Assessment with the Low-Dose ACTH Stimulation Test

    Directory of Open Access Journals (Sweden)

    René Rodríguez-Gutiérrez

    2016-01-01

    Full Text Available Background. Multidrug-resistant tuberculosis (MDR-TB is a major public health care concern that affects the life of millions of people around the world. The association of tuberculosis and adrenal insufficiency is well known; however, it is thought to be less prevalent every time. A spike in TB incidence and a lack of evidence of this association in patients with MDR-TB call for reassessment of an illness (adrenal dysfunction that if not diagnosed could seriously jeopardize patients’ health. Objective. To determine the prevalence of adrenocortical insufficiency in patients with MDR-TB using the low-dose (1 μg ACTH stimulation test at baseline and at 6–12 months of follow-up after antituberculosis treatment and culture conversion. Methods. A total of 48 men or women, aged ≥18 years (HIV-negative patients diagnosed with pulmonary MDR-TB were included in this prospective observational study. Blood samples for serum cortisol were taken at baseline and 30 and 60 minutes after 1 μg ACTH stimulation at our tertiary level university hospital before and after antituberculosis treatment. Results. Forty-seven percent of subjects had primary MDR-TB; 43.8% had type 2 diabetes; none were HIV-positive. We found at enrollment 2 cases (4.2% of adrenal insufficiency taking 500 nmol/L as the standard cutoff point value and 4 cases (8.3% alternatively, using 550 nmol/L. After antituberculosis intensive phase drug-treatment and a negative mycobacterial culture (10.2±3.6 months adrenocortical function was restored in all cases. Conclusions. In patients with MDR-TB, using the low-dose ACTH stimulation test, a low prevalence of mild adrenal insufficiency was observed. After antituberculosis treatment adrenal function was restored in all cases. Given the increasing and worrying epidemic of MDR-TB these findings have important clinical implications that may help clinicians and patients make better decisions when deciding to test for adrenocortical

  17. Absence of exercise-induced leptin suppression associated with insufficient epinephrine reserve in patients with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

    NARCIS (Netherlands)

    Riepe, F.G.; Krone, N.; Kruger, S.N.; Sweep, C.G.J.; Lenders, J.W.M.; Dotsch, J.; Monig, H.; Sippell, W.G.; Partsch, C.J.

    2006-01-01

    OBJECTIVE: Patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency suffer from glucocorticoid and mineralocorticoid deficiency. They have insufficient epinephrine reserves and increased basal leptin levels and are often insulin resistant. In healthy subjects, an inhibitor

  18. [The state of sympathetic-adrenal system in patients with chronic cardiac insufficiency].

    Science.gov (United States)

    Nigmatullin, R R; Kirillova, V V; Dzhordzhikiia, R K; Kudrin, V S; Klodt, P M

    2009-01-01

    Activation of sympato-adrenal system plays an important role in the development of chronic cardiac failure (CCF). However, its relation to morpho-functional state of myocardium in CCF patients is virtually unknown. HPLC with electrochemical detection was used to determine plasma noradrenalin, adrenalin, and their precursors, 3,4-dioxyphenylalanine (DOPA) and dopamine, in patients with different morpho-functional changes in myocardium. The study demonstrated enhanced activity of sympato-adrenal system in patients with CCF. It showed for the first time that activity of sympato-adrenal system in CCF patients depends on the morpho-functional status of myocardium.

  19. Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing’s Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

    OpenAIRE

    Raff, Hershel; Sharma, Susmeeta T.; Nieman, Lynnette K.

    2014-01-01

    The hypothalamic-pituitary-adrenal (HPA) axis is a classic neuroendocrine system. One of the best ways to understand the HPA axis is to appreciate its dynamics in the variety of diseases and syndromes that affect it. Excess glucocorticoid activity can be due to endogenous cortisol overproduction (spontaneous Cushing’s syndrome) or exogenous glucocorticoid therapy (iatrogenic Cushing’s syndrome). Endogenous Cushing’s syndrome can be subdivided into ACTH-dependent and ACTH-independent, the latt...

  20. Adrenal Insufficiency Therapy: How to Keep the Balance between Good Quality of Life and Low Risk for Long-Term Side Effects?

    Science.gov (United States)

    Simunkova, Katerina; Husebye, Eystein S

    2016-01-01

    Glucocorticoid treatment in adrenal insufficiency remains a challenge since many patients complain of fatigue, reduced health-related quality of life and working ability. Moreover, there is a fear of increased mortality and morbidity related to adrenal crises and chronic overexposure to glucocorticoids. In order to counter these negative effects on daily life and future health, recent recommendations speak for lower replacement doses and administration forms that attempt to mimic the circadian variation in cortisol. At the same time, low replacement doses should not put the patient at an increased risk of an adrenal crisis. This chapter provides an overview of current state-of-the-art approaches to treatment and follow-up of adrenal insufficiency aimed at avoiding over- and undertreatment.

  1. Cortisol-dependent stress effects on cell distribution in healthy individuals and individuals suffering from chronic adrenal insufficiency.

    Science.gov (United States)

    Geiger, Ashley M; Pitts, Kenneth P; Feldkamp, Joachim; Kirschbaum, Clemens; Wolf, Jutta M

    2015-11-01

    Chronic adrenal insufficiency (CAI) is characterized by a lack of glucocorticoid and mineralocorticoid production due to destroyed adrenal cortex cells. However, elevated cortisol secretion is thought to be a central part in a well-orchestrated immune response to stress. This raises the question to what extent lack of cortisol in CAI affects stress-related changes in immune processes. To address this question, 28 CAI patients (20 females) and 18 healthy individuals (11 females) (age: 44.3 ± 8.4 years) were exposed to a psychosocial stress test (Trier Social Stress Test: TSST). Half the patients received a 0.03 mg/kg body weight injection of hydrocortisone (HC) post-TSST to mimic a healthy cortisol stress response. Catecholamines and immune cell composition were assessed in peripheral blood and free cortisol measured in saliva collected before and repeatedly after TSST. CAI patients showed norepinephrine (NE) stress responses similar to healthy participants, however, epinephrine (E) as well as cortisol levels were significantly lower. HC treatment post-TSST resulted in cortisol increases comparable to those observed in healthy participants (interaction effects--NE: F=1.05, p=.41; E: F=2.56, p=.045; cortisol: F=13.28, pcortisol's central involvement in post-stress lymphocyte migration from blood into immune-relevant body compartments. As such, future studies should investigate whether psychosocial stress exposure may put CAI patients at an increased health risk due to attenuated immune responses to pathogens.

  2. Reversible Adrenal Insufficiency in Three Patients With Post–Roux-en-Y Gastric Bypass Noninsulinoma Pancreatogenous Hypoglycemia Syndrome

    Directory of Open Access Journals (Sweden)

    Shelly Mathur MD

    2014-03-01

    Full Text Available Objective. Noninsulinoma pancreatogenous hypoglycemia syndrome (NIPHS is a disorder of endogenous hyperinsulinemia that is clinically distinguishable from insulinoma, with a greater preponderance after Roux-en-Y gastric bypass (RYBG. Hyperinsulinemic hypoglycemia can predispose to attenuation of counterregulatory hormone responses to hypoglycemia, and consequent suppression of the hypothalamic–pituitary–adrenal (HPA axis. This case series describes 3 individuals who were diagnosed with adrenal insufficiency (AI after undergoing RYGB, complicated by NIPHS. Methods. A retrospective chart review was performed for each individual. Chart review applied particular attention to the onset of hyperinsulinemic hypoglycemia following bariatric surgery and the dynamic testing leading to the diagnoses of NIPHS and AI. Results. In each case, reactive hypoglycemia ensued within months to years after RYGB. Cosyntropin stimulation testing confirmed the diagnosis of AI. Hydrocortisone therapy reduced the frequency and severity of hypoglycemia and was continued until successful medical and/or surgical management of hyperinsulinism occurred. Follow-up testing of the HPA axis demonstrated resolution of AI. In all cases, hydrocortisone therapy was finally discontinued without incident. Conclusion. We speculate that transient AI is a potential complication in patients who experience recurrent hyperinsulinemic hypoglycemia after RYGB. The putative mechanism for this observation may be attenuation of the HPA axis after prolonged exposure to severe, recurrent hypoglycemia. We conclude that biochemical screening for AI should be considered in individuals who develop post-RYGB hyperinsulinemic hypoglycemia. If AI is diagnosed, supportive treatment should be maintained until hyperinsulinemic hypoglycemia has been managed effectively.

  3. The effects of two different doses of hydrocortisone on cognition in patients with secondary adrenal insufficiency - Results from a randomized controlled trial

    NARCIS (Netherlands)

    Buning, Jorien Werumeus; Brummelman, Pauline; Koerts, Janneke; Dullaart, Robin P. F.; van den Berg, Gerrit; van der Klauw, Melanie M.; Tucha, Oliver; Wolffenbuttel, Bruce H. R.; van Beek, Andre P.

    2015-01-01

    Context: A wide variety in hydrocortisone (HC) substitution dose-regimens are considered physiological for patients with secondary adrenal insufficiency (SAD. However, it is likely that cognition is negatively influenced by higher cortisol exposure to the brain. Objective: To examine the effects of

  4. Takotsubo cardiomyopathy in a patient with pituitary adenoma and secondary adrenal insufficiency

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    Georgene Singh

    2015-01-01

    Full Text Available We describe a case of Takotsubo cardiomyopathy in a case of pituitary macroadenoma in acute adrenal crisis. A 48-year-old man presented with acute onset altered sensorium, vomiting, and gasping. On admission, he was unresponsive and hemodynamically unstable. He was intubated and ventilated and resuscitated with fluids and inotropes. The biochemical evaluation revealed hyponatremia, hyperkalemia, and hypocortisolism. Hyponatremia was corrected with 3% hypertonic saline. Contrast enhanced computed tomography (CT scan of the brain revealed a sellar-suprasellar mass with hypothalamic extension with no evidence of pituitary apoplexy. A diagnosis of invasive pituitary adenoma with the Addisonian crisis was made and steroid replacement was initiated. Despite volume resuscitation, he had persistent refractory hypotension, recurrent ventricular tachycardia, and metabolic acidosis. Electrocardiogram (ECG showed ST elevation and T-wave inversion in lateral leads; cardiac-enzymes were increased suggestive of acute coronary syndrome. Transthoracic echocardiography showed severe regional wall motion abnormalities (RWMAs involving left anterior descending territory and low ejection fraction (EF. Coronary angiogram revealed normal coronaries, apical ballooning, and severe left ventricular dysfunction, consistent with a diagnosis of Takotsubo′s cardiomyopathy. Patient was managed with angiotensin-converting enzyme inhibitors and B-blockers. He improved over few days and recovered completely. At discharge, ECG changes and RWMA resolved and EF normalized to 56%. In patients with Addisonian Crisis with persistent hypotension refractory to optimal resuscitation, possibility of Takotsubo′s cardiomyopathy should be considered. Early recognition of association of Takotsubos cardiomyopathy in neurological conditions, prompt resuscitation, and supportive care are essential to ensure favorable outcomes in this potentially lethal condition.

  5. Adrenal insufficiency from over-the-counter medicine as a cause of shock in rural area of Thailand: a study at Sisaket Provincial Hospital during October 2012--October 2013.

    Science.gov (United States)

    Kamrat, Nuttamon

    2015-04-01

    This retrospective study was carried out to estimate the prevalence of an adrenal crisis at a provincial hospital in Thailand over a period of 1 year, and also to explore the relationship between adrenal insufficiency and over-the-counter medicine (OTCM) ingestion. We recruited those patients admitted at Sisaket Hospital between October 2012 and October 2013 who were diagnosed with shock and adrenal insufficiency or adrenal crisis. Of 2,435 patients diagnosed with shock from all causes, 62 (2.55 %) were diagnosed with adrenal crisis, of whom 31 (50.0%) gave a history of OTCM ingestion. This study suggests adrenal crisis with shock is not that uncommon and that the use of OTCM may be the prime culprit.

  6. Managing Adrenal Insufficiency

    Science.gov (United States)

    ... with you will need to learn how to give you this injection. The injection will take the place of both hydrocortisone and Florinef pills. If you find it necessary to give yourself an injectable medication, call your health care ...

  7. Acute psychosis in the course of treatment of acute adrenal crisis with hydrocortisone in the patient with secondary adrenal insufficiency – a case study.

    Directory of Open Access Journals (Sweden)

    Jan Brykalski

    2015-08-01

    The case focuses attention on the risk of psychosis connected with the treatment of the adrenal crisis with high doses of Hydrocortisone. Because of the risk of psychiatric complications, the patients treated with high doses of corticosteroids, require an evaluation of risk factors for mental disturbances, and safety precautions in cooperation of endocrinologist and psychiatrist.

  8. 儿童肾上腺皮质功能减退症诊治进展%Etiology, diagnosis and management of adrenal insufficiency in children

    Institute of Scientific and Technical Information of China (English)

    马华梅

    2015-01-01

    Adrenal insufficiency(AI) is a disorder that can result from primary adrenal failure(primary type) or secondary adrenal disease due to impairment of the hypothalamic-pituitary axis (central type).It is characterized by glucocorticoid deficiency,with or without inappropriate mineralocorticoid or androgen production.The diagnosis and management of children with AI remain challenging as clinical characteristics of AI in children maybe non-specific and acute adrenal crisis is life-threatening,needing life-long glucocorticoid replacement treatment and education of the patient and the family.New formulations of hydrocortisone which should be able to simulate the circadian rhythm of steroid secretion are supposed to improve outcome in patients with adrenal insufficiency in the near future.%儿童肾上腺皮质功能减退(AI)是指源于下丘脑-垂体-肾上腺轴功能损害的一组综合征,可为原发肾上腺衰竭(原发性),或继发于下丘脑-垂体轴病变的肾上腺疾病(中枢性).临床表现为糖皮质激素(GC)分泌不足或作用缺陷,伴或不伴盐皮质激素分泌异常、伴或不伴雄激素分泌异常.因临床特点非特异而常不被知悉,急性肾上腺危象则可威胁生命,需要立即得到诊治.长期GC替代、家庭教育是儿童AI管理的2个重要组成部分,同时必须严密监测以防治疗不足或过度.氢化可的松新型制剂——双释放剂型已经研制成功,其对于改善患儿的治疗依从性、维持皮质醇的分泌节律、改善患儿的预后可能具有广阔的前景.

  9. Identification of Paracoccidioides brasiliensis in adrenal glands biopsies of two patients with paracoccidioidomycosis and adrenal insufficiency Identificação do Paracoccidioides brasiliensis nas amostras de glândulas adrenais de dois doentes com paracoccidioidomicose e insuficiência adrenal

    Directory of Open Access Journals (Sweden)

    Carlos Andrés Agudelo

    2009-02-01

    Full Text Available The authors report two cases of adrenal insufficiency secondary to infiltration of the adrenal glands by Paracoccidioides brasiliensis. The first patient had been treated for a chronic multifocal form of paracoccidiodomycosis 11 years ago. The diagnosis of the mycosis was done simultaneous with that of the adrenal insufficiency in the second patient. In both patients the diagnosis was done by direct visualization of fungus in adrenal biopsies. They were treated with hormonal supplements and itraconazol by 12 and six months, without relapses during the follow-up period.Os autores apresentam dois casos de insuficiência supra-renal secundária à infiltração das adrenais pelo Paracoccidioides brasiliensis. O primeiro paciente tinha sido tratado de paracoccidioidomicose crônica multifocal 11 anos atrás. No segundo paciente, o diagnóstico da micose foi feito de forma simultânea com o da insuficiência adrenal. Em ambos os pacientes, o diagnóstico foi feito pela visualização direta do fungo nas biopsias adrenais. Eles foram tratados com suplementos hormonais com itraconazol por seis a 12 meses, sem recaídas durante o período de acompanhamento.

  10. Progress in the adrenal insufficient and adrenal crisis of critical children%重症儿童肾上腺皮质功能不全及肾上腺危象研究进展

    Institute of Scientific and Technical Information of China (English)

    兰洁

    2012-01-01

    Recent studies have showed that adrenal insufficiency are common in some status of critically ill children,like systemic inflammatory response syndrome,sepsis,shock,et al.The frequency of adrenal crisis was also higher than expectation.The currrent glucocorticoid replacement therapies are inclined to lower dose [ hydrocortisone 20 ~ 25 mg/( m2· d) ].The adrenal function could be evaluated with the free cortisol index and calculated free cortisol.A new method,temperature-controlled centrifugal ultrafiltration with chemiluminescence immunoassay,has been used to detect the free cotisol,which has improved the clinical practicality.%研究发现,肾上腺皮质功能不全在重症儿童的某些疾病(如全身炎症反应综合征、脓毒血症、休克等)中普遍存在,肾上腺危象的发生率也比预期高.目前糖皮质激素替代治疗倾向于小剂量氢化可的松20~ 25 mg/( m2 ·d)口服.实验室检查用游离皮质醇指数和计算游离皮质醇来评估肾上腺皮质功能,并采用新的温度控制离心超滤法结合化学发光免疫分析检测游离皮质醇,增加了该检测的临床实用性.

  11. Medical Treatment for Acromegaly does not Increase the Risk of Central Adrenal Insufficiency: A Long-Term Follow-Up Study.

    Science.gov (United States)

    Ceccato, F; Lizzul, L; Zilio, M; Barbot, M; Denaro, L; Emanuelli, E; Alessio, L; Rolma, G; Manara, R; Saller, A; Boscaro, M; Scaroni, C

    2016-08-01

    Central adrenal insufficiency (CAI) in acromegaly may be related to pituitary adenoma or induced by various medical treatments, transsphenoidal neurosurgery (TNS) or radiotherapy (RT), alone or combined. We assessed the role of all available treatments for acromegaly in inducing CAI. We retrospectively studied 97 patients. CAI was diagnosed if morning serum cortisol was <138 nmol/l, or if its response was inadequate in the low-dose short synacthen test. Seventy-four subjects underwent TNS (and 17 of whom also underwent RT), and 23 were on primary medical therapy: overall we diagnosed 21 cases of CAI. Duration of acromegaly, invasion of cavernous sinus, disease control, and type of medical treatment were much the same for patients with and without CAI, which was identified in 18% of patients (10/57) after one TNS, and in 53% (9/17) after RT (p=0.01); repeat surgery increased the risk of CAI (p=0.02). The risk of CAI onset during the follow-up was lower among patients treated with TNS or medical therapy than after RT (p=0.035). Medical treatment did not raise the risk of CAI, whereas a 5- and 4-fold higher risk of CAI was associated with repeat TNS and RT, respectively. Basal or stimulated cortisol levels were similar among acromegalic patients without CAI and matched controls with nonsecreting pituitary lesions. A significant proportion of patients with acromegaly developed CAI over time. While primary or secondary medical treatment did not contribute to the risk of CAI, repeat TNS and RT correlated with pituitary-adrenal axis impairment.

  12. Quality of life in patients with adrenal insufficiency correlates stronger with hydrocortisone dosage, than with long-term systemic cortisol levels.

    Science.gov (United States)

    Andela, Cornelie D; Staufenbiel, Sabine M; Joustra, Sjoerd D; Pereira, Alberto M; van Rossum, Elisabeth F C; Biermasz, Nienke R

    2016-10-01

    In patients with adrenal insufficiency (AI) a higher hydrocortisone intake has been associated with more impairment in quality of life (QoL). Irrespective of age, sex and severity of AI the dosage of hydrocortisone is titrated around 20mg/D in all patients with AI based on physical and mental signs and symptoms. However, until now it is unknown whether these QoL impairments are related to increased systemic cortisol exposure. Measurement of hair cortisol levels (CORThair) can be used to assess chronic systemic cortisol exposure. This study aimed to explore whether QoL in patients with AI is associated with CORThair and daily hydrocortisone intake. We performed a cross-sectional study in 120 patients with AI on stable hydrocortisone replacement, in whom hair samples and QoL data were collected. CORThair were measured with ELISA, and QoL was assessed with validated questionnaires (SF-36, EQ-5D, HADS, MFI-20). Patients reported impairments in 14 of 15 QoL subscales (pcortisol exposure as measured by hair cortisol. This suggests that QoL impairments in patients with AI are not per se the effect of prolonged exposure to elevated systemic cortisol levels.

  13. Adrenal Insufficiency and Addison's Disease

    Science.gov (United States)

    ... in Endocrinology, Diabetes and Obesity. 2010;(3):217–223. Clinical Trials The National Institute of Diabetes and ... la salud en español Health Statistics Healthy Moments Radio Broadcast Clinical Trials For Health Care Professionals Community ...

  14. Primary bilateral adrenal non-Hodgkin's lymphoma associated with normal adrenal function.

    Science.gov (United States)

    Gu, Bin; Ding, Qiang; Xia, Guowei; Fang, Zujun; Fang, Jie; Jiang, Haowen; Yao, Mengshu

    2009-04-01

    Primary bilateral adrenal non-Hodgkin's lymphoma is rare. Adrenal insufficiency or adrenal failure as a result of tumor destruction is the main pathophysiological change of most cases. Normal adrenal function despite bulky bilateral adrenal masses is extremely rare. We present a case of primary bilateral adrenal non-Hodgkin's lymphoma associated with normal adrenal function. Positron emission tomography-computed tomography is helpful to the diagnosis.

  15. Spontaneous bilateral adrenal hemorrhage following cholecystectomy.

    Science.gov (United States)

    Dahan, Meryl; Lim, Chetana; Salloum, Chady; Azoulay, Daniel

    2016-06-01

    Postoperative bilateral adrenal hemorrhage is a rare but potentially life-threatening complication. This diagnosis is often missed because the symptoms and laboratory results are usually nonspecific. We report a case of bilateral adrenal hemorrhage associated with acute primary adrenal insufficiency following laparoscopic cholecystectomy. The knowledge of this uncommon complication following any abdominal surgery allows timey diagnosis and rapid treatment.

  16. Aortic insufficiency

    Science.gov (United States)

    ... page, please enable JavaScript. Aortic insufficiency is a heart valve disease in which the aortic valve does not close ... aortic insufficiency Images Aortic insufficiency References Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil ...

  17. 关注老年人在危重症中肾上腺皮质功能不全问题%Attention on the problem of adrenal insufficiency in elderly patients with critically illness

    Institute of Scientific and Technical Information of China (English)

    王艳

    2015-01-01

    Because of the issue of an aging population in society,the elderly patients increased,in addations,as the elderly people always have decreasing in tolerance and stress,so adrenal insufficiency easily occurred in elderly patients with critically illness, this problem must be vigilant,and need to early diagnosis,early treatment,in order to improve the treatment capacity in critical disease,and reduce the mortality rate.%社会人口老年化,老年患者增加,而老年人因耐受力及应激力下降。在患有危重症时,容易发生肾上腺皮质功能不全,这是必须警惕的问题。需要早诊断,早治疗,才能提高在危重症中的救治力,降低死亡率。

  18. Adrenal Failure due to Adrenal Metastasis of Lung Cancer: A Case Report

    Science.gov (United States)

    Faulhaber, Gustavo Adolpho Moreira; Borges, Flavia Kessler; Ascoli, Aline Maria; Seligman, Renato; Furlanetto, Tania Weber

    2011-01-01

    We report a case of a patient with adrenal failure due to bilateral adrenal metastasis of lung cancer. This is a rare presentation of lung cancer. We review the differential diagnosis of weight loss and how to make diagnosis of adrenal insufficiency. PMID:22606443

  19. Adrenal Failure due to Adrenal Metastasis of Lung Cancer: A Case Report

    Directory of Open Access Journals (Sweden)

    Gustavo Adolpho Moreira Faulhaber

    2011-01-01

    Full Text Available We report a case of a patient with adrenal failure due to bilateral adrenal metastasis of lung cancer. This is a rare presentation of lung cancer. We review the differential diagnosis of weight loss and how to make diagnosis of adrenal insufficiency.

  20. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  1. Persistence of histoplasma in adrenals 7 years after antifungal therapy

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    Deepak Kothari

    2013-01-01

    Full Text Available Adrenal histoplasmosis is an uncommon cause for adrenal insufficiency. The duration of treatment for adrenal histoplasmosis is not clear. Existing treatment regimens advocate antifungals given for periods ranging from 6 months to 2 years. We report here a rare case who showed persistence of histoplasma in adrenal biopsy 7 years after being initially treated with itraconazole for 9 months. This calls for a prolonged therapy with regular review of adrenal morphology and histology in these patients.

  2. Bilateral adrenal histoplasmosis in an immunocompetent man from Texas

    Directory of Open Access Journals (Sweden)

    Colin J. Rog

    2016-12-01

    Full Text Available Disseminated histoplasmosis affecting the adrenal gland(s of immunocompetent adults is a very rare infection. Here, we present a case of bilateral adrenal histoplasmosis in an immunocompetent, 62-year-old gentleman from Texas along with a brief review of the published literature. Given the risk of patient decompensation secondary to adrenal insufficiency and the wide availability of effective treatments, adrenal histoplasmosis must be considered even in immunocompetent adults who acquire adrenal masses.

  3. Adrenal Insufficiency in Critically Ⅲ Patients and the Impact on Ventilator Weaning%重症患者肾上腺皮质功能状态及对呼吸机撤离的影响

    Institute of Scientific and Technical Information of China (English)

    高健婷; 刘宁; 顾勤

    2012-01-01

    Objective To examine the adrenal function of critically ill patients received mechanical ventilation, and explore the relationship between the occurrence of relative adrenal insufficiency (RAI) and weaning outcome. Methods Critically ill patients who were mechanically ventilated over 48 hours were enrolled in this study. Every patient was given one shot of corticotrophin 230μg intravenously on the first day of admission and the first day of spontaneous-breathing-trial (SBT). Plasma contisol level was detected by radio-immunoassay before (TO) and 30 minutes ( T30) after the shot. Meanwhile the following parameters were recorded including APACHE II , age, and cause of disease, etc. RAI was defined as the difference between TO and T30 ≤9μg/dL. Receiver operating characteristic ( ROC) curve was used to evaluate the accuracy of the indicators towards the weaning outcome. Results A total of 43 patients with mechanical ventilation were recruited. The successful weaning group consisted 29 patients and the failure weaning group consisted 16 patients. The incidence of RAI in the successful weaning group (37. 9% ,11/ 29) was significantly lower than that in the failure weaning group ( 75.0% , 12/16) (P = 0. 017). On the first day of admission,there was no significant difference of Acortisol between the successful weaning group and the failure weaning group [ (10. 3 ±5. 7)μg/dL vs. (7. 5 ±4. 5)μg/dL, P = 0. 100). On the firstSBT day, Acortisol of the successful weaning group was significantly higher than that in the failure weaning group [ (10. 9 ± 5. 1) μg/dL vs. (4. 9 ± 2.9)μg/dL, P = 0. 043 ]. Logistic regression analysis showed that △cortisol was an independent risk factor of weaning. ROC curve analysis showed that on the first SBT day, the area under the curve of Acortisol was 0. 872; The sensitivity and the specificity of accurate judgment were 0. 813 and 0. 828 if △cortisol ≤6. 95μg/dL. Conclusions The occurrence of RAI is common in critically ill

  4. Adrenal Function Status in Patients with Paracoccidioidomycosis after Prolonged Post-Therapy Follow-Up

    Science.gov (United States)

    Tobón, Angela M.; Agudelo, Carlos A.; Restrepo, Carlos A.; Villa, Carlos A.; Quiceno, William; Estrada, Santiago; Restrepo, Angela

    2010-01-01

    This study assessed adrenal function in patients with paracoccididioidomycosis who had been treated to determine a possible connection between high antibody titers and adrenal dysfunction attributable to persistence of the fungus in adrenal gland. Adrenal gland function was studied in 28 previously treated patients, 2 (7.1%) of whom were shown to have adrenal insufficiency and 7 (259%) who showed a below normal response to stimuli by adrenocorticotropic hormone. Paracoccidioides brasiliensis was detected in the adrenal gland from one of the patients with adrenal insufficiency. Although the study failed to demonstrate a significant difference between high antibody titers and low cortisol levels, the proportion of adrenal insufficiency detected and the subnormal response to adrenocorticotropic hormone confirmed that adrenal damage is an important sequela of paracoccidioidomycosis. Studies with a larger number of patients should be conducted to confirm the hypothesis of persistence of P. brasiliensis in adrenal gland after therapy. PMID:20595488

  5. A case of non-Hodgkin's lymphoma primary arising in both adrenal glands associated with adrenal failure.

    Science.gov (United States)

    Nishiuchi, Takamasa; Imachi, Hitomi; Fujiwara, Mako; Murao, Koji; Onishi, Hiroaki; Kiguchi, Tohru; Takimoto, Hidetaka; Kushida, Yoshio; Haba, Reiji; Ishida, Toshihiko

    2009-02-01

    It is known that adrenal insufficiency is one of the complications in primary adrenal lymphoma, especially those with bilateral adrenal involvement. A 73-year-old man was referred for general fatigue and high fever to the nearest hospital. The patient was transferred to our hospital for evaluation of bilateral adrenal tumors and hyponatremia. He was diagnosed as having non-Hodgkin's lymphoma (NHL) with primaries arising in both adrenal glands. Primary adrenal lymphoma (PAL) is a rare extra-nodal NHL. Although an appropriate treatment of this disease has not been established, our case has demonstrated that the combination of rituximab and THP-COP chemotherapy could be administered, and that it improved clinical manifestations. This case raises the suggestion that malignant lymphoma should be suspected in patients with bilateral adrenal tumors that present with progressive adrenal insufficiency.

  6. Adrenal haemorrhage with cholestasis and adrenal crisis in a newborn of a diabetic mother.

    Science.gov (United States)

    Koklu, Esad; Kurtoglu, Selim; Akcakus, Mustafa; Koklu, Selmin

    2007-03-01

    The large hyperaemic foetal adrenal gland is vulnerable to vascular damage. This may occur in the neonatal period as a consequence of difficult labour, or its aetiology may not be apparent. The spectrum of presentation is considerable, ranging from asymptomatic to severe life-threatening intra-abdominal haemorrhage. The presentation of adrenal insufficiency may be delayed but the regenerative capacity of the adrenal is great, and most adrenal haemorrhage is not associated with significantly impaired function. Some reports showed that cholestatic hepatopathy with congenital hypopituitarism reversed by hydrocortisone treatment is considered in the context of the endocrine syndrome, probably as a consequence of the adrenal failure. We describe a case of bilateral adrenal haemorrhage with hepatitis syndrome and persistent hypoglycaemia in a newborn male with striking features of neonatal cholestasis and adrenal crisis.

  7. Histoplasmosis of the adrenal glands studied by CT

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    Wilson, D.A.; Muchmore, H.G.; Tisdal, R.G.; Fahmy, A.; Pitha, J.V.

    1984-03-01

    Computed tomography (CT) of the adrenal glands was performed on seven patients who had histologically proved disseminated histoplasmosis. All seven patients showed some degree of adrenal gland abnormality. The range of CT findings included minimal enlargement with faint flecks of calcium, moderate enlargement with focal low attenuation nodules, and massive enlargement with large areas of necrosis or dense calcification. The changes in each patient were bilateral and symmetrical. Adrenal gland shape was usually preserved. Finding of percutaneous adrenal biopsy, which was performed under CT guidance, made the diagnosis in one patient. Five of seven patients had adrenal insufficiency. It is concluded that the diagnosis of disseminated histoplasmosis should be considered in any patient who has bilateral adrenal gland enlargement and who resides in an endemic area, especially if there is evidence of adrenal insufficiency.

  8. Bilateral adrenal [corrected] nodules due to histoplasmosis in an elderly.

    Science.gov (United States)

    Carvalho, Flávio Pedreira de Freitas de; Curiati, José Antônio Esper; Mauad, Thaís; Incerti, Milena Mendes; Jacob Filho, Wilson

    2007-02-01

    We report a case history of an 84-year-old elderly male patient that presented with a clinical picture suggestive of adrenal failure and bilateral adrenal nodules detected by abdominal computed tomography. A fine needle-guided biopsy was inconclusive for achieving a final diagnosis. The patient died due to septic shock and the autopsy disclosed histoplasmosis with extensive bilateral necrosis of the adrenal glands. Although the adrenal involvement in chronic disseminated histoplasmosis has been described, there have been few reports of the infection being associated with adrenal insufficiency.

  9. Dehydroepiandrosterone substitution in female adrenal failure: no impact on endothelial function and cardiovascular parameters despite normalization of androgen status

    DEFF Research Database (Denmark)

    Christiansen, Jens Juel; Andersen, Niels Holmark; Sørensen, Keld E;

    2007-01-01

    BACKGROUND: Female adrenal insufficiency implicates reduced production of the adrenal androgen precursor dehydroepiandrosterone (DHEA) and low androgen levels. Oral DHEA restores androgen deficit but the clinical implications and safety of substitution therapy is uncertain. A putative DHEA receptor...... androgen levels using 6 months of DHEA replacement in this pilot study did not affect cardiovascular parameters and endothelial function in female adrenal insufficiency...

  10. Primary adrenal lymphoma with paraneoplastic syndrome

    Directory of Open Access Journals (Sweden)

    Radhika Dasararaju

    2013-01-01

    Full Text Available Context: The adrenal gland is a common site for neoplastic diseases and primary adrenal lymphoma (PAL is a rare tumor with around 120 cases reported so far. Case Report: We present a rare case of 76-year-old male who presented with headache, confusion, inappropriate body movements and abdominal pain. Adrenal biopsy revealed PAL and he has had an excellent neurologic outcome to date with chemotherapy and involved field radiation. Conclusion: The majority of cases of PAL are B cell lymphomas with diffuse large cell in 70% of cases. Clinical symptoms are variable and patients may present with abdominal pain, fever, anorexia, weight loss, fatigue or symptoms of adrenal insufficiency. Therapeutic modalities for PAL include surgery, chemotherapy and radiotherapy and corticosteroid replacement. With this case report, we hope to raise awareness about this rare disease and to include lymphoma in the differential of adrenal masses.

  11. An endocrinologist's view on relative adrenocortical insufficiency in rheumatoid arthritis.

    Science.gov (United States)

    Imrich, Richard; Vlcek, Miroslav; Aldag, Jean C; Kerlik, Jana; Radikova, Zofia; Rovensky, Jozef; Vigas, Milan; Masi, Alfonse T

    2010-04-01

    The concept of relative adrenal insufficiency (RAI) has been originally introduced to describe a situation in which critically ill patients, without any prior risk or evidence for adrenal insufficiency, have total serum cortisol levels inadequate for the severity of patients' illness. The concept provided a framework for other disease states, in which higher than normal adrenal function could be expected, such as in chronic inflammation. An intense research in RAI field highlighted some new methodological aspects that significantly improved assessment of adrenal function in chronic illness. Measurement of salivary cortisol may provide additional information on locally available cortisol in target tissues. Low levels of dehydroepiandrosterone (DHEAS) for given age and gender were confirmed as a simple and reliable indicator of decreased adrenal function, even in subjects with normal baseline cortisol or normal corticotropin-stimulated cortisol response. Combined lower DHEAS and lower baseline cortisol levels could be an example of hypocompetence of adrenocortical function, yet clinically not apparent.

  12. Adrenal cysts

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    @@ Ture cysts of the adrenal gland are lined with endothelium or epithe lium.Most lesions are asympomatic and are discovered incidentally.They may produce s ymptoms because of hemorrhage.CT findings of cysts include(Fig 1): ① Cyst are well-marginated, nonenhancing, homogeneous, fluid-cont aining masses; ② The wall may have thin peripheral calcification if previous hemor rhage has occurred.③ Cyst contents have characteristics of simple fluids(<20 HU)unle ss hemorrhage has occurred.

  13. Bilateral adrenal hemorrhage in polycythemia vera

    Directory of Open Access Journals (Sweden)

    Shruti Bhandari

    2016-09-01

    Full Text Available Bilateral adrenal hemorrhage (BAH is a rare complication typically seen in critically ill patients, which can lead to acute adrenal insufficiency and death unless it is recognized promptly and treated appropriately. We describe the case of a 64-year-old man with polycythemia vera found to be unresponsive with fever, hypotension, tachycardia, and hypoglycemia. Electrocardiogram showed ST-elevation with elevated troponin, hemoglobin, prothrombin time, and partial thromboplastin time. He required aggressive ventilator and vasopressor support. Despite primary coronary intervention, he remained hypotensive. Random cortisol level was low. He received stress dose hydrocortisone with immediate hemodynamic stability. BAH was highly suspected and was confirmed by non-contrast abdominal computed tomography. Prompt recognition and timely initiated treatment remain crucial to impact the mortality associated with acute adrenal insufficiency.

  14. Adrenal Gland Tumors: Statistics

    Science.gov (United States)

    ... Gland Tumor: Statistics Request Permissions Adrenal Gland Tumor: Statistics Approved by the Cancer.Net Editorial Board , 03/ ... primary adrenal gland tumor is very uncommon. Exact statistics are not available for this type of tumor ...

  15. Adrenal Gland Cancer

    Science.gov (United States)

    ... either benign or malignant. Benign tumors aren't cancer. Malignant ones are. Most adrenal gland tumors are ... and may not require treatment. Malignant adrenal gland cancers are uncommon. Types of tumors include Adrenocortical carcinoma - ...

  16. NonClassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Azziz Ricardo

    2010-05-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and "on-time" puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  17. Adrenal invovement in histoplasmosis

    OpenAIRE

    Norasyikin, A. Wahab; Nor Azmi, Kamaruddin; Rozita, Mohd; Suehazlyn, Zainudin

    2013-01-01

    Histoplasmosis infection is endemic in Asia and disseminated histoplasmosis (DH) is one form of its presentation (Benevides et al., 2007). DH commonly affects both adrenal glands. We describe a case of disseminated histoplasmosis complicated with hypercalcaemia in a 75-year-old immunosuppressed patient who presented with bilateral adrenal masses. The fine needle aspiration cytology of the adrenal mass was positive for Histoplasma capsulatum.

  18. Disorders of adrenal development.

    Science.gov (United States)

    Ferraz-de-Souza, Bruno; Achermann, John C

    2008-01-01

    Human adrenal development is a complex and relatively poorly understood process. However, significant insight into some of the mechanisms regulating adrenal development and function is being obtained through the analysis of individuals and families with adrenal hypoplasia. Adrenal hypoplasia can occur: (1) secondary to defects in pituitary adrenocorticotropin (ACTH) synthesis, processing and release (secondary adrenal hypoplasia; e.g. HESX1, LHX4, SOX3, TPIT, pituitary POMC, PC1); (2) as part of several ACTH resistance syndromes (e.g. MC2R/ACTHR, MRAP, Alacrima, Achalasia, Addison disease), or as (3) a primary defect in the development of the adrenal gland itself (primary adrenal hypoplasia; e.g. DAX1/NR0B1 - dosage-sensitive sex reversal, adrenal hypoplasia congenita critical region on the X chromosome 1). Indeed, the X-linked form of primary adrenal hypoplasia due to deletions or mutations in the orphan nuclear receptor DAX1 occurs in around half of male infants presenting with a salt-losing adrenal crisis, where no obvious steroidogenic defect (e.g. 21-hydroxylase deficiency), metabolic abnormality (e.g. neonatal adrenoleukodystrophy) or physical cause (e.g. adrenal haemorrhage) is found. Establishing the underlying basis of adrenal failure can have important implications for investigating associated features, the likely long-term approach to treatment, and for counselling families about the risk of other children being affected.

  19. [Hypoplasia adrenal congenita of anencephalic type: two cases with pituitary abnormalities and review of literature].

    Science.gov (United States)

    Folligan, K; Roume, J; Razavi, F; Sepaniak, S; Bouvier, R; Morel, Y; Trouillas, J

    2011-03-01

    Hypoplasia adrenal congenita is an extremely uncommon disease of early onset. This condition can be lethal in the absence of treatment. Some forms are due to the congenital adrenal hypoplasia of anencephalic type whose origin is even unknown. Here, we present two cases of congenital adrenal hypoplasia of anencephalic type with pituitary abnormalities. The two male newborns died because adrenal insufficiency in the neonatal period. The adrenal glands were hypoplastic with a histological structure of anencephalic type Immunocytochemical study of the pituitary revealed an absence of the gonadotrophs. No mutation of DAX 1 and SF-1 was found.

  20. Adrenal crisis in a patient with acute myeloid leukaemia

    OpenAIRE

    Li, Wang; Okwuwa, Ikemefuna; Toledo-Frazzini, Karla; Alhomosh, Alaaedin

    2013-01-01

    Adrenal crisis is a group of clinical manifestation predominantly with hypotensive shock, electrolyte imbalance in a patient with adrenal insufficiency or in a patient who was abruptly withdrawn from glucocorticoid treatment acute myeloid leukaemia (AML) is one of the most common acute leukaemia in adults. Though the above diseases are commonly seen in individual patients, the coexistence of both conditions in the same patient is rare. We reported a 64-year-old African-American man with a his...

  1. [Adrenal cortex insufficiency in children due to inhaled corticosteroids

    NARCIS (Netherlands)

    Eijkemans, M.C.J.; Otten, B.J.; Yntema, J.L.

    2011-01-01

    A 3-year-old boy was treated for asthmatic symptoms with fluticasone inhalations. Due to a flattening growth curve Cushing's syndrome was suspected and the dosage of fluticasone was gradually decreased after which the boy became less active and his appetite decreased. Another patient, a 7-year-old b

  2. Catecholamine dependency in a polytrauma patient : relative adrenal insufficiency?

    NARCIS (Netherlands)

    Nieboer, P.; van der Werf, T.S.; Beentjes, J.A.M.; Tulleken, J.E.; Zijlstra, J.G.; Ligtenberg, J.J.M.

    2000-01-01

    A 65-year-old polytrauma patient was admitted post-operatively to the intensive care unit. His situation deteriorated with hemodynamic instability and continuous high fever. An infectious focus could not be found and repeated cultures remained negative. Empirical administration of antibiotics and ch

  3. A case of congenital lipoid adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Mahin Hashemipour

    2012-01-01

    Full Text Available Lipoid congenital adrenal hyperplasia (lipoid CAH, a rare disorder of steroid biosynthesis, is the most severe form of CAH. In this disorder the synthesis of glucocorticoids, mineralocorticoids and sex steroids is impaired which result in adrenal failure, severe salt wasting crisis and hyperpigmentation in phenotypical female infants irrespective of genetic sex. In this report, we presented a 28-day-old phenotypic female infant, which referred with lethargy, failure to thrive and electrolyte abnormalities. Considering the clinical and biochemical findings, lipoid CAH was diagnosed and replacement therapy with standard doses of glucocorticoid and mineralocorticoid and sodium chloride was initiated. During follow-up, she had good clinical condition, but at 6 years of age, she refers with hypertension and adrenal insufficiency because of arbitrary drug discontinuation by mother. In ultrasonography an abdominal mass (the testicles was reported. Chromosome study showed 46XY pattern. Orchiectomy was performed. We recommended that in cases with clinical presentation of adrenal insufficiency if there is not the facility to determine the karyotype, repeated ultrasonography perform during follow-up. In addition, investigating the genetic bases of the disorder would help us to determine the pathogenesis of lipoid CAH in our community. It would be helpful in prenatal diagnosis and treatment of the disorder to prevent its related comorbidities.

  4. Intense Adrenal Enhancement: A CT Feature of Cardiogenic Shock

    Energy Technology Data Exchange (ETDEWEB)

    Hrabak-Paar, Maja, E-mail: maja.hrabak.paar@mef.hr [University of Zagreb School of Medicine, Department of Diagnostic and Interventional Radiology, University Hospital Center Zagreb (Croatia)

    2016-02-15

    In this report, images of intense adrenal enhancement in a 79-year-old female patient with right-sided heart failure and severe tricuspid insufficiency are presented. Only two cases of intense adrenal enhancement as a sign of cardiogenic shock were previously reported in the literature. Intense adrenal enhancement could be one of the earliest CT signs of cardiogenic shock. Its presence should be immediately reported to the referring physician as a sign of significant hemodynamic instability warranting early critical-care management.

  5. Intense Adrenal Enhancement: A CT Feature of Cardiogenic Shock.

    Science.gov (United States)

    Hrabak-Paar, Maja

    2016-02-01

    In this report, images of intense adrenal enhancement in a 79-year-old female patient with right-sided heart failure and severe tricuspid insufficiency are presented. Only two cases of intense adrenal enhancement as a sign of cardiogenic shock were previously reported in the literature. Intense adrenal enhancement could be one of the earliest CT signs of cardiogenic shock. Its presence should be immediately reported to the referring physician as a sign of significant hemodynamic instability warranting early critical-care management.

  6. Pituitary-Adrenal Axis in Prader Willi Syndrome

    Directory of Open Access Journals (Sweden)

    Olivia S. Edgar

    2016-01-01

    Full Text Available Purpose: Prader Willi syndrome (PWS is a rare genetic condition that has concurrent endocrinological insufficiencies. The presence of growth hormone deficiency has been well documented, but adrenal insufficiency (AI is not widely reported. A review was conducted to investigate its prevalence and relevance in PWS in both adults and children. Methodology: A literature review was performed with the search terms “Prader-Willi syndrome” and “adrenal insufficiency”. Results: The review found studies disagree on the prevalence and method of investigation of AI in PWS. Case studies demonstrate that patients with PWS are at risk of premature death, often secondary to respiratory infections. The possibility that this may be the result of the inability to mount an effective cortisol response has been studied, with some evidence confirming AI in PWS patients. Most reports agreed AI is present in PWS, however, Farholt et al. showed no HPA axis dysfunction in adults, suggesting that perhaps it is rare in adults, and children should be the focus of further studies. Conclusion: AI is present in some patients with PWS. Further research is required to ensure optimal treatment can be implemented and to prevent premature deaths related to adrenal insufficiency. Clinicians should have a low threshold for testing the adrenal axis and considering treatment for adrenal insufficiency in PWS patients.

  7. Spectrum of Adrenal Dysfunction in Patients with Acquired Immunodeficiency Syndrome Evaluation of Adrenal and Pituitary Reserve with ACTH and Corticotropin-Releasing Hormone Testing.

    Science.gov (United States)

    Freda, P U; Papadopoulos, A D; Wardlaw, S L; Goland, R S

    1997-07-01

    Patients with acquired immunodeficiency syndrome (AIDS) have been reported to develop abnormalities of the endocrine system and in particular of the hypothalamic-pituitary-adrenal (HPA) axis. To define the abnormalities of HPA function in AIDS patients better, we performed ACTH and ovine corticotropin-releasing hormone (oCRH) testing in a group of AIDS patients and oCRH testing in a group of healthy subjects. Our study found that in AIDS patients with normal ACTH testing, oCRH testing revealed a variety of subclinical abnormalities of ACTH and cortisol responses. Although we did not find frank adrenal insufficiency in any of these AIDS patients, it remains to be determined if any of the subclinical abnormalities we identified are predictive of clinically significant adrenal insufficiency; it may be that as AIDS patients live longer, the subclinical abnormalities will progress to adrenal insufficiency. (Trends Endocrinol Metab 1997;8:173-180). (c) 1997, Elsevier Science Inc.

  8. Imaging of insufficiency fractures

    Energy Technology Data Exchange (ETDEWEB)

    Krestan, Christian [Department of Radiology, Medical University of Vienna, Vienna General Hospital, Waehringerstr. 18-20, 1090 Vienna (Austria)], E-mail: christian.krestan@meduniwien.ac.at; Hojreh, Azadeh [Department of Radiology, Medical University of Vienna, Vienna General Hospital, Waehringerstr. 18-20, 1090 Vienna (Austria)

    2009-09-15

    This review focuses on the occurrence, imaging and differential diagnosis of insufficiency fractures. Prevalence, the most common sites of insufficiency fractures and their clinical implications are discussed. Insufficiency fractures occur with normal stress exerted on weakened bone. Postmenopausal osteoporosis is the most common cause of insufficiency fractures. Other conditions which affect bone turnover include osteomalacia, hyperparathyroidism, chronic renal failure and high-dose glucocorticoid therapy. It is a challenge for the radiologist to detect and diagnose insufficiency fractures, and to differentiate them from other bone lesions. Radiographs are still the most widely used imaging method for identification of insufficiency fractures, but sensitivity is limited, depending on the location of the fractures. Magnetic resonance imaging (MRI) is a very sensitive tool to visualize bone marrow abnormalities associated with insufficiency fractures. Thin section, multi-detector computed tomography (MDCT) depicts subtle fracture lines allowing direct visualization of cortical and trabecular bone. Bone scintigraphy still plays a role in detecting fractures, with good sensitivity but limited specificity. The most important differential diagnosis is underlying malignant disease leading to pathologic fractures. Bone densitometry and clinical history may also be helpful in confirming the diagnosis of insufficiency fractures.

  9. The role of imaging in congenital adrenal hyperplasia

    Energy Technology Data Exchange (ETDEWEB)

    Teixeira, Sara Reis; Andrade, Marco Tulio Soares; Melo, Andrea Farias; Elias Junior, Jorge, E-mail: jejunior@fmrp.usp.br [Department of Internal Medicine, Division of Radiology, Clinical Hospital, Ribeirao Preto Medical School, University of Sao Paulo (FMRP-USP), Ribeirao Preto, SP (Brazil); Elias, Paula Condé Lamparelli [Department of Internal Medicine, Division of Endocrinology, Clinical Hospital, FMRP-USP, Ribeirao Preto, SP (Brazil)

    2014-10-15

    Congenital adrenal hyperplasia (CAH) is an autossomic recessive disorder caused by impaired steroidogenesis. Patients with CAH may present adrenal insufficiency with or without salt-wasting, as well as various degrees of virilization and fertility impairment, carrying a high incidence of testicular adrenal rest tumors and increased incidence of adrenal tumors. The diagnosis of CAH is made based on the adrenocortical profile hormonal evaluation and genotyping, in selected cases. Follow-up is mainly based on hormonal and clinical evaluation. Utility of imaging in this clinical setting may be helpful for the diagnosis, management, and follow-up of the patients, although recommendations according to most guidelines are weak when present. Thus, the authors aimed to conduct a narrative synthesis of how imaging can help in the management of patients with CAH, especially focused on genitography, ultrasonography, computed tomography, and magnetic resonance imaging. (author)

  10. Volume of the adrenal and pituitary glands in depression

    DEFF Research Database (Denmark)

    Kessing, Lars Vedel; Willer, Inge Stoel; Knorr, Ulla

    2011-01-01

    Numerous studies have shown that the hypothalamic-pituitary-adrenal (HPA) axis is hyperactive in some depressed patients. It is unclear whether such hyperactivity results in changed volumes of the adrenal glands, pituitary gland and hypothalamus. We systematically reviewed all controlled studies...... on the adrenal or pituitary glands or hypothalamus volume in unipolar depressive disorder published in PubMed 1966 to December 2009. We identified three studies that investigated the volume of the adrenal glands and eight studies that examined the volume of the pituitary gland, but no studies on hypothalamus...... identified such as small population samples, different subtypes of depression and insufficient matching of patients and controls. Due to large heterogeneity of study designs and data, it was futile to make a meta-analysis. It is concluded that it remains unclear whether hyperactivity of the HPA axis results...

  11. Adrenal function in preterm infants undergoing patent ductus arteriosus ligation.

    LENUS (Irish Health Repository)

    El-Khuffash, Afif

    2013-01-01

    Targeted milrinone treatment for low left ventricular output (LVO) reduces the incidence of acute cardiorespiratory instability following ligation of patent ductus arteriosus (PDA) in preterm infants. Despite this, some infants continue to experience postoperative deterioration. Adrenal insufficiency related to prematurity has been postulated as a possible mechanism.

  12. Primary adrenal sarcomatoid carcinoma

    Directory of Open Access Journals (Sweden)

    Aftab S. Shaikh

    2014-03-01

    Full Text Available Adrenal sarcomatoid carcinomas are extremely rare tumors presenting with extensive locoregional spread at the time of diagnosis. Patients succumb to metastases within a couple of months. As a result, very few cases are reported in the literature until now. We present a case of a 62-year old female with non-functional sarcomatoid carcinoma of the right adrenal gland. There was no radiological evidence of locoregional metastases. Patient underwent right adrenalectomy. Follow up after 3 months showed para-aortic lymphadenopathy and similar left adrenal mass on computed tomography. Patient refused further treatment and succumbed to the disease. A brief case report with review of literature is presented.

  13. [Adrenal failure caused by primary adrenal non-Hodgkin lymphoma: a case report and review of the literature].

    Science.gov (United States)

    Hernández Marín, B; Díaz Muñoz de la Espada, V M; Alvarez Alvarez, R; Encinas García, S; Khosravi Shahi, P; Pérez Fernández, R; Pérez Manga, G

    2008-03-01

    We report a case of 78-year old man who presented with symptoms of adrenal insufficiency. The computed tomography (CT) scan showed the presence of bilateral adrenal masses. A CT-scan guided needle biopsy revealed diffuse large- B cell lymphoma. The absence of pathological findings in clinical, bone marrow and CT scan examinations supported the diagnosis of primary non-Hodgkin Lymphoma of the adrenal glands. The patient was treated with four cycles of R-CHOP chemotherapy with Rituximab, liposomal Doxorubicin, Cyclophosphamide, Vincristine and Prednisolone. At the end of fourth cycle there was radiological improvement but the chemotherapy was stopped because of IV grade toxicity. He completed treatment with radiotherapy of right adrenal mass. Few days after finishing radiation therapy the patient died due to a disseminated infection. No progressive disease was founded.

  14. Congenital adrenal hyperplasia

    Science.gov (United States)

    ... their genitalia during infancy. Steroids used to treat congenital adrenal hyperplasia do not usually cause side effects such as obesity or weak bones, because the doses replace the hormones that the ...

  15. Primary Ovarian Insufficiency (POI)

    Science.gov (United States)

    ... insufficiency in the adolescent : Committee opinion no. 502. Obstetrics and Gynecology, 118 , 741–745. ... Search of Answers for Those Struggling With Infertility Study finds genetic clue to menopause-like condition ...

  16. Adrenal gland and bone.

    Science.gov (United States)

    Hardy, Rowan; Cooper, Mark S

    2010-11-01

    The adrenal gland synthesizes steroid hormones from the adrenal cortex and catecholamines from the adrenal medulla. Both cortisol and adrenal androgens can have powerful effects on bone. The overproduction of cortisol in Cushing's disease leads to a dramatic reduction in bone density and an increase risk of fracture. Overproduction of adrenal androgens in congenital adrenal hyperplasia (CAH) leads to marked changes in bone growth and development with early growth acceleration but ultimately a significant reduction in final adult height. The role of more physiological levels of glucocorticoids and androgens on bone metabolism is less clear. Cortisol levels measured in elderly individuals show a weak correlation with measures of bone density and change in bone density over time with a high cortisol level associated with lower bone density and more rapid bone loss. Cortisol levels and the dynamics of cortisol secretion change with age which could also explain some age related changes in bone physiology. It is also now clear that adrenal steroids can be metabolized within bone tissue itself. Local synthesis of cortisol within bone from its inactive precursor cortisone has been demonstrated and the amount of cortisol produced within osteoblasts appears to increase with age. With regard to adrenal androgens there is a dramatic reduction in levels with aging and several studies have examined the impact that restoration of these levels back to those seen in younger individuals has on bone health. Most of these studies show small positive effects in women, not men, but the skeletal sites where benefits are seen varies from study to study.

  17. Primary bilateral adrenal B-cell lymphoma associated with EBV and JCV infection

    Directory of Open Access Journals (Sweden)

    Guzzardo Vincenza

    2009-01-01

    Full Text Available Abstract Primary lymphoma of the adrenal gland is a rare and highly aggressive disease, with only a few reports in the literature. The pathogenesis is unknown, but detection of Epstein Barr virus (EBV genome sequences and gene expression in some cases of primary adrenal lymphomas suggested the virus might be a causative agent of the malignancy. While investigating the presence of genome sequences of oncogenic viruses in a large series of adrenal tumors, both EBV and JC polyomavirus (JCV DNA sequences were detected in a diffuse large primary bilateral B-cell non-Hodgkin lymphoma of the adrenal gland, which was diagnosed only at postmortem examination in a 77 year-old woman with incidentally discovered adrenal masses and primary adrenal insufficiency. The presence of both EBV and JCV genome sequences suggests the relevance of EBV and JCV coinfection in the pathogenesis of this rare form of B-cell lymphoma.

  18. Characterization of the LPS-induced inflammation of the adrenal gland in mice.

    Science.gov (United States)

    Kanczkowski, Waldemar; Chatzigeorgiou, Antonios; Samus, Maryna; Tran, Nguyen; Zacharowski, Kai; Chavakis, Triantafyllos; Bornstein, Stefan R

    2013-05-22

    Systemic administration of endotoxin, which closely mimics the bacteria-induced systemic inflammatory response syndrome (SIRS) can ultimately lead to organ failure. Adrenal gland insufficiency is frequently diagnosed in critically ill patients; however, the underlying mechanisms are still unclear. In the present study, we studied comprehensively the characteristics of adrenal gland dysregulation, including inflammation, leukocyte infiltration and cell death in the adrenal glands in the course of LPS-induced systemic inflammation in mice. LPS enhanced expression of many proinflammatory cytokines, chemokines and adhesion molecules, which resulted in rapid recruitment of leukocytes into the adrenal gland. Furthermore, LPS-mediated inflammation was associated with increased apoptosis of adrenocortical and chromaffin cells. Our results performed in mice, suggest that LPS-induced adrenal gland inflammation and cell death might be mechanisms potentially involved in the adrenal gland dysfunction in patients with sepsis.

  19. A glucocorticoid education group meeting: an effective strategy for improving self-management to prevent adrenal crisis

    NARCIS (Netherlands)

    Repping-Wuts, H.; Stikkelbroeck, N.M.; Noordzij, A.; Kerstens, M.; Hermus, A.R.M.M.

    2013-01-01

    OBJECTIVE: To assess self-management in patients receiving glucocorticoid replacement therapy for primary or secondary adrenal failure before and 6 months after a glucocorticoid education group meeting. METHODS: All patients with primary or secondary adrenal insufficiency, treated at the Department

  20. Physical, social and societal functioning of children with congenital adrenal hyperplasia (CAH) and their parents, in a Dutch population.

    NARCIS (Netherlands)

    Sanches, S.A.; Wiegers, T.A.; Otten, B.J.; Claahsen-van der Grinten, H.L.

    2012-01-01

    BACKGROUND: Most research concerning congenital adrenal hyperplasia (CAH) and related conditions caused by primary adrenal insufficiency, such as Addison's or Cushing's disease, has focused on medical aspects rather than on patients' quality of life. Therefore, our objective was to investigate the p

  1. Imaging of adrenal disorders

    Energy Technology Data Exchange (ETDEWEB)

    Fukuchi, S. (Fukushima Medical Coll. (Japan))

    1982-03-01

    Adrenal scintillation scanning, CT and ultrasonography are compared with the conventional imaging methods. The accuracy of retroperitoneal pneumography and adrenal venography are not high, and they detected only large tumors such as Cushing's syndrome and pheochromocytoma. Scintillation scanning is highly effective for the diagnoses of primary aldosteronism and Cushing's syndrome. However, this technique does not visualize pheochromocytoma or hypopituitarism. CT is noninvasive and of high diagnostic value. It is impossible to diagnose tumors by ultrasonography unless the size is more than 3 cm.

  2. Limited contribution of NR5A1 (SF-1) mutations in women with primary ovarian insufficiency (POI)

    NARCIS (Netherlands)

    Janse, Femi; de With, Larissa M; Duran, Karen J; Kloosterman, Wigard P; Goverde, Angelique J; Lambalk, Cornelius B; Laven, Joop S E; Fauser, Bart C J M; Giltay, Jacques C; Hoek, Annemieke

    2012-01-01

    OBJECTIVE: To evaluate the significance of NR5A1 mutations in a large, well-phenotyped cohort of women with primary ovarian insufficiency (POI). Mutations in the NR5A1 gene (SF-1) were previously described in disorders of sexual development and adrenal insufficiency. Recently, a high frequency of NR

  3. Adrenal Gland Disorders

    Science.gov (United States)

    ... t live without, including sex hormones and cortisol. Cortisol helps you respond to stress and has many other important functions. With adrenal gland disorders, your glands make too much or not enough hormones. In Cushing's ... too much cortisol, while with Addison's disease, there is too little. ...

  4. Role of DAX-1 (NR0B1) and steroidogenic factor-1 (NR5A1) in human adrenal function.

    Science.gov (United States)

    El-Khairi, Ranna; Martinez-Aguayo, Alejandro; Ferraz-de-Souza, Bruno; Lin, Lin; Achermann, John C

    2011-01-01

    The nuclear receptor transcription factors DAX-1 (NR0B1) and SF-1 (NR5A1) regulate many aspects of adrenal and reproductive development and function. Disruption of the genes encoding these factors can be associated with pediatric adrenal disease. DAX-1 mutations are classically associated with X-linked adrenal hypoplasia congenita, hypogonadotropic hypogonadism and impaired spermatogenesis. However, other phenotypes are also being reported, such as isolated mineralocorticoid insufficiency, premature sexual development, primary adrenal insufficiency in a 46, XX patient and late-onset X-linked adrenal hypoplasia congenita and/or hypogonadotropic hypogonadism. SF-1 mutations have also been associated with primary adrenal insufficiency, together with 46, XY disorders of sex development. However it is emerging that SF-1 changes are a relatively rare cause of primary adrenal failure in humans, and most individuals with SF-1 mutations have a spectrum of 46, XY disorders of sex development phenotypes. These conditions range from 46, XY females with streak gonads and müllerian structures, through children with ambiguous genitalia and inguinal testes, to severe penoscrotal hypospadias with undescended testes. Therefore, the human gonad appears to be more sensitive than the adrenal gland to loss of SF-1 function. This review will focus on the expanding range of phenotypes associated with DAX-1 and SF-1 mutations.

  5. Renal and suprarenal insufficiency secondary to familial Mediterranean fever associated with amyloidosis: a case report

    Directory of Open Access Journals (Sweden)

    Sari Nagehan

    2011-08-01

    Full Text Available Abstract Introduction Familial Mediterranean fever is an autosomal recessive disease that predominantly affects people of the Mediterranean coast. One of the most frequent complications of the disease is amyloidosis. This clinical entity is known as secondary (also called AA amyloidosis. Case presentation In this report, we describe the case of a 33-year-old Turkish man with familial Mediterranean fever and chronic renal insufficiency. He was admitted to our clinic with symptoms of suprarenal insufficiency. The patient died three months later as a result of cardiac arrest. Conclusion Our aim is to make a contribution to the literature by reporting a case of combined insufficiency due to the accumulation of renal and adrenal amyloid in a patient with familial Mediterranean fever, which has very rarely been described in the literature. We hope that adrenal insufficiency, which becomes fatal if not diagnosed and treated rapidly, will come to mind as easily as chronic renal failure in clinical practice.

  6. Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Bures Vladimir

    2005-04-01

    Full Text Available Abstract We report on a 14 year old boy who presented with the symptoms abdominal pain, fever and proteinuria. A hematoma in the region of the right pararenal space was diagnosed. Prothrombin time and activated partial thromboplastin time were prolonged, lupus anticoagulant and anticardiolipin antibodies were positive and serum cortisol was normal. Ten days after admission the boy suddenly suffered generalized seizures due to low serum sodium. As well, the patient developed hemolytic anemia, acute elevated liver enzymes, hematuria and increased proteinuria. At this time a second hemorrhage of the left adrenal gland was documented. Adrenal function tests revealed adrenal insufficiency. We suspected microthromboses in the adrenals and secondary bleeding and treated the boy with hydrocortisone, fludrocortisone and phenprocoumon. Conclusion Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms.

  7. Genetic disorders involving adrenal development.

    Science.gov (United States)

    Lin, Lin; Ferraz-de-Souza, Bruno; Achermann, John C

    2007-01-01

    The past decade has seen significant advances in our understanding of the genetic aetiology of several forms of adrenal failure that present in infancy or childhood. Several of these disorders affect adrenal development and are termed 'adrenal hypoplasia'. These conditions can be broadly divided into: (1) secondary forms of adrenal hypoplasia due to panhypopituitarism (e.g. HESX1, LHX4, SOX3) or abnormalities in ACTH synthesis (TPIT) or processing (e.g. POMC or PC1); (2) adrenal hypoplasia as part of an ACTH resistance syndrome [MC2R/ACTH receptor, MRAP, AAAS (triple A syndrome)], and (3) primary defects in the development of the adrenal gland itself (primary adrenal hypoplasia). Primary adrenal hypoplasia most commonly occurs in an X-linked form due to mutations in the nuclear receptor DAX1 (NR0B1) but can occur in a poorly understood recessive form or as part of the IMAGe (intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia, genitourinary anomalies) syndrome. Defining the molecular basis of these conditions can have significant clinical implications for management, counselling and presymptomatic diagnosis, as well as providing fascinating insight into normal and abnormal mechanisms of adrenal development in humans.

  8. Betahistine in vertebrobasilar insufficiency.

    Science.gov (United States)

    Kaźmierczak, Henryk; Pawlak-Osińska, Katarzyna; Kaźmierczak, Wojciech

    2004-01-01

    The aim of this study was to observe the usefulness of betahistine dihydrochloride--Betaserc--in therapy for vestibular disorders in patients with vertebrobasilar insufficiency. Two groups of patients, in each of which were 150 patients (mean age, 52.2 years), were tested on the basis of videonystagmography and stabilometry. Betaserc was administrated in two separate doses: 8 mg three times daily and 16 mg three times daily for 120-180 days (mean, 132 days). In every case before and after therapy, visuo-oculomotor and vestibulo-oculomotor reflexes were tested, and amplitude and velocity of the sway were measured during dynamic posturographic testing. After Betaserc treatment, pathological visuo-oculomotor reactions and pathological cervical test results disappeared in most cases: Smooth pursuit improved in 59.9% of cases and saccadic movements in 55.9% of patients, and cervical nystagmus disappeared in 62.2% of tested people. During stabilometry, mean and maximal platform amplitude and mean head velocity decreased as compared with results from tests performed before treatment. These observations were significant after the greater dose of Betaserc; nonetheless, improvement was noted after both doses. The usefulness of Betaserc in vertebrobasilar insufficiency was proved, 4-6 months' therapy was sufficient, and the effect on central compensation seemed to be most probable.

  9. Cystic adrenal lesions: focus on pediatric population (a review)

    Science.gov (United States)

    CARSOTE, MARA; GHEMIGIAN, ADINA; TERZEA, DANA; GHEORGHISAN-GALATEANU, ANCUTA AUGUSTINA; VALEA, ANA

    2017-01-01

    Background and aim The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. General data Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks. Conclusion Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented

  10. Hypothalamus-pituitary-adrenal axis suppression following induction chemotherapy in children with acute lymphoblastic leukemia

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    Perdomo-Ramírez, Iván

    2016-01-01

    Full Text Available Background: Adrenal insufficiency has been reported in 46 % to 81.5 % of children receiving corticosteroids for acute lymphoblastic leukemia (ALL. Methodology: To assess the frequency of such insufficiency, 40 patients under 18 years (mean: 8.5 years with new diagnosis of ALL were studied. Base-line cortisol and adrenocorticotropin (ACTH levels were measured, and they received 5-week therapy with prednisolone. After 3 days off-steroid therapy, a stimulation test with ACTH 1 μg was done. In patients with abnormal cortisol (<18 μg/dL new ACTH tests were done and cortisol levels were determined at days 7, 14 and 30 until cortisol post-stimulation levels were normal. Results: Three days after the last steroid dose 29/40 (72.5 % had adrenal insufficiency after ACTH stimulus. At day 30 no one had abnormal cortisol levels after ACTH stimulus. All patients with adrenal suppression were over 5 years (HR 4.69; CI95 %: 1.44-15.32; p = 0.011. Conclusion: Steroids used during ALL treatment may cause adrenal insufficiency. Patients over 5 years are at high risk of developing adrenal suppression. We suggest to follow-up those patients with stress episodes after induction chemotherapy as steroid supplementation may be indicated.

  11. Adrenal cryptococcosis in an immunosuppressed patient showing intensely increased metabolic activity on (18)F-FDG PET/CT.

    Science.gov (United States)

    Papadakis, Georgios Z; Holland, Steven M; Quezado, Martha; Patronas, Nicholas J

    2016-12-01

    Disseminated cryptococcosis most commonly occurs in immunosuppressed patients and can rarely affect the adrenal glands. We report on a patient with biopsy proven bilateral adrenal cryptococcosis resulting in primary adrenal insufficiency, which was evaluated with whole-body positron emission tomography/computed tomography scan using (18)F-FDG. Both enlarged adrenal glands presented intensely increased (18)F-FDG activity in the periphery, while central necrotic regions were photopenic. Although diagnosis was established by adrenal gland biopsy, (18)F-FDG positron emission tomography/computed tomography scan can significantly contribute to the assessment of disease activity and monitoring of treatment response. Furthermore, fungal infections should always be considered when encountering hypermetabolic adrenal masses, especially in the setting of immunodeficient patients.

  12. Testicular adrenal rest tumours in congenital adrenal hyperplasia

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Hermus, A.R.M.M.; Otten, B.J.

    2009-01-01

    In adult patients with congenital adrenal hyperplasia (CAH), the presence of testicular adrenal rest tumours (TART) is an important complication leading to gonadal dysfunction and infertility. These tumours can be already found in childhood and puberty. In this paper, we review the embryological, hi

  13. IMAGe association: report of two cases in siblings with adrenal hypoplasia and review of the literature.

    Science.gov (United States)

    Phillips, Katherine; Arroyo, May R; Duckworth, Lizette Vila

    2014-01-01

    We report the postmortem findings of two siblings with gross and microscopic features consistent with IMAGe association (Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital anomalies) with an emphasis on the histopathology of the adrenal gland in this rare syndrome. The first sibling was an 8-week old male diagnosed postnatally with primary adrenal insufficiency. There was no deletion of the DAX1 gene by FISH. Examination at autopsy revealed dysmorphic features including frontal bossing, epicanthal folds, flat philtrum, cryptorchidism, penile chordee, overriding fourth toe, and height and weight below 3rd percentile. Grossly, the adrenal glands were not identified; however, microscopic examination of the suprarenal soft tissue revealed a 3 mm focus of disorganized fetal adrenal cortex with distended "cytomegalic" cells with abundant pink eosinophilic cytoplasm, vesicular nuclei, and cytoplasmic vacuolization. A minute focus of permanent adult cortex was also seen, but no adrenal medulla was identified. An autopsy of the sibling, who died 12 years previously at day 9 of life, revealed dysmorphic facial features with cryptorchidism and a large phallus. The adrenal glands were grossly hypoplastic (11 mm). Histologically, the adrenal glands showed disorganized fetal cortex with cytomegalic cells, a larger amount of permanent adult cortex, and bizarre nuclei with numerous pseudoinclusions. While there is currently limited information regarding the histopathologic adrenal findings in IMAGe association, our small case series suggests overlapping features between X-linked recessive congenital adrenal hypoplasia (cytomegalic cells with lack of permanent adult cortex) and autosomal recessive congenital adrenal hypoplasia (diminished permanent adult cortex without cytomegalic cells).

  14. ADRENAL INCIDENTALOMAS: ANALYSIS OF 126 CASES

    Institute of Scientific and Technical Information of China (English)

    李汉忠; 严维刚; 曾正陪; 肖河; 冯超; 王惠君

    2003-01-01

    Purpose. To evaluate the diagnosis and treatment of adrenal incidentalomas. Methods. One hundred and twenty-six patients with incidentalomas were analyzed, among them 98 underwent operation. Results. Eighty-eight of the adrenal incidentalomas were discovered by ultrasound. Of all the types of adrenal incidentalomas, 52 (41.3%) of them were adenomas; 43 (34.1%) were hypersecretory adrenal tumors, including 29 pheochromocytomas, 9 primary aldosteronisms, 1 adrenogenitol syndrome combined with adrenal adenoma, 2 Cushing's syndrome combined with adenomas and 2 Cushing's syndrome combined with nodular hyperplasias. All nonhypersecretory adrenal adenomas were under 6 cm, and all adrenal carcinomas were above 6 cm.Conclusions. To search for hypersecretory adrenal tumors and to detect malignant adrenal tumors are quite essential in the process of diagnosing adrenal incidentalomas. For nonhypersecretory adrenal adenomas, the size of tumor is the most important index in determining whether the tumor is benign or malignant and whether the tumor needs to be treated with operation.

  15. Imaging findings of neonatal adrenal disorders

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hye Kyung; Han, Bo Kyung; Lee, Min Hee [Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-01-01

    In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surrounded by a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenal gland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenal hemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenal hemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findings in neonates with various adrenal disorders.

  16. Diffusion MRI features of acute encephalopathy due to stopping steroid medication abruptly in congenital adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Asli Serter

    2015-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH is characterized by adrenal steroid biosynthesis defect. Steroid replacement therapy should be performed regularly in these patients. Adrenal crisis may be present in acute stress due to increased cortisol requirements or in steroid deficiency due to stopping steroid medication abruptly. In patients with acute adrenal insufficiency, severe hypotension or hypovolemic shock occurs typically. Acute encephalopathy can be seen due to hypoxia, hypervolemia, or hypoglycemia. Diffusion restriction can be seen in cortical-subcortical regions of frontal and parieto-occipital lobes and in splenium of corpus callosum. In CAH patients with neurologic symptoms, Diffusion weighted images (DWI is very important in the diagnosis and follow-up of acute encephalopathy.

  17. Adrenal Disorders and the Paediatric Brain: Pathophysiological Considerations and Clinical Implications

    Directory of Open Access Journals (Sweden)

    Vincenzo Salpietro

    2014-01-01

    Full Text Available Various neurological and psychiatric manifestations have been recorded in children with adrenal disorders. Based on literature review and on personal case-studies and case-series we focused on the pathophysiological and clinical implications of glucocorticoid-related, mineralcorticoid-related, and catecholamine-related paediatric nervous system involvement. Childhood Cushing syndrome can be associated with long-lasting cognitive deficits and abnormal behaviour, even after resolution of the hypercortisolism. Exposure to excessive replacement of exogenous glucocorticoids in the paediatric age group (e.g., during treatments for adrenal insufficiency has been reported with neurological and magnetic resonance imaging (MRI abnormalities (e.g., delayed myelination and brain atrophy due to potential corticosteroid-related myelin damage in the developing brain and the possible impairment of limbic system ontogenesis. Idiopathic intracranial hypertension (IIH, a disorder of unclear pathophysiology characterised by increased cerebrospinal fluid (CSF pressure, has been described in children with hypercortisolism, adrenal insufficiency, and hyperaldosteronism, reflecting the potential underlying involvement of the adrenal-brain axis in the regulation of CSF pressure homeostasis. Arterial hypertension caused by paediatric adenomas or tumours of the adrenal cortex or medulla has been associated with various hypertension-related neurological manifestations. The development and maturation of the central nervous system (CNS through childhood is tightly regulated by intrinsic, paracrine, endocrine, and external modulators, and perturbations in any of these factors, including those related to adrenal hormone imbalance, could result in consequences that affect the structure and function of the paediatric brain. Animal experiments and clinical studies demonstrated that the developing (i.e., paediatric CNS seems to be particularly vulnerable to alterations induced by

  18. Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia

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    S. Al-Bahri

    2014-01-01

    Full Text Available Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH. We report the case of a 39-year-old male with a history of CAH secondary to 21-α hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing’s syndrome, Addison’s disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.

  19. Ectopic Thyroid in the Adrenal Presenting as an Adrenal Incidentaloma

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    Banu Aktaş Yılmaz

    2016-12-01

    Full Text Available Adrenal incidentalomas are clinical dilemmas for the clinicians. The work up, to differentiate between malignant and benign lesions, and hyperfunctioning and nonfunctioning lesions is mandatory before the consideration of surgical resection. Ectopic thyroid tissue located in the adrenal gland (ETTAG is a very rare condition. We report a case of ETTAG presenting with adrenal incidentaloma. A 57-year-old woman was admitted with incidental right adrenal mass. Hormone evaluation showed no hormonal activity. Magnetic resonance imaging revealed a 20x17 mm lobulated solid mass, which contained millimetric hypointense nodular areas consistent with calcifications. Loss of signal intensity on out-of-phase could not be evaluated because of the calcifications. Right adrenalectomy was performed to establish the histopathological diagnosis and to rule out malignancy. Histopathological diagnosis revealed ETTAG. Her medical history was positive for multinodular goiter and bilateral subtotal thyroidectomy 32 years ago. Thyroid ultrasonography showed residual thyroid tissue in both the right and left lobes, and colloid thyroid nodules. Fine needle aspiration biopsy from the nodules revealed benign nodules. The patient has been followed up for six years, and no change in thyroid nodule sizes and no evidence of metastatic foci have been detected. ETTAG would be considered in the differential diagnosis of adrenal mass showing no hormonal activity, especially when magnetic resonance images are not consistent with adrenal adenoma. Long follow-up duration of this case suggests that it was a benign condition.

  20. [Pheochromocytomas as adrenal gland incidentalomas].

    Science.gov (United States)

    Cerović, Snezana; Cizmić, Milica; Milović, Novak; Ajdinović, Boris; Brajusković, Goran

    2002-07-01

    Adrenal incidentalomas are a heterogeneous group of pathological entities, including benign or malignant adrenocortical or medullary tumors, hormonally active or inactive lesions, which are identified incidentally during the examination of nonadrenal-related abdominal complaints. About 1.5% to 23% of adrenal incidentalomas are pheochromocytomas. Composite pheochromocytoma is a rare tumour of adrenal medulla with divergente clinical course. This type of pheochromocytoma is designated "composite" or "mixed," depending on whether pheochromocytoma and nonpheochromocytoma components show the same embryologic origin. Nonpheochromocytoma components found in the composite pheochromocytoma include ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and malignant schwannoma. The biologic behavior of composite pheochromocytomas may be as difficult to predict as more traditional pheochromocytomas; based on the number of cases reported to date the presence of areas resembling ganglioneuroblastoma or neuroblastoma does not necessary indicate a poor prognosis. Some may behave in a malignant fashion with metastasis by a component of the tumour which has neural features. Pheochromocytomas and paragangliomas are well-defined entities. Some of their nonsporadic associations and unusual morphological appearances are not universally appreciated. We report on a rare association of left adrenal CP, with typical right adrenal phochromocytoma and retroperitoneal paraganglioma, and a review of literature. We analyzed the clinical and immunohistochemical features in a 24-year-old woman with composite pheochromocytoma localized in the left adrenal gland and associated with blood pressure of 200/140 mmHg. Abdominal computed tomography and 131-J MIBG revealed a 65 x 60 mm mass in the right adrenal gland, but no revealed 45 x 40 mm retroperitoneal mass and 20 x 20 mm mass in the left adrenal region. Serum and urinary adrenaline levels were high, and catecholamine levels in the blood sample of

  1. [Congenital Adrenal Hyperplasia in Adults].

    Science.gov (United States)

    Vrbíková, Jana

    2016-01-01

    Congenital adrenal hyperplasia is a life-long disease requiring an integrated therapy. It may negatively influence the quality of life. In childhood, the main problems of the care of these patients involve sex determination and ensuring optimum growth and puberty. The therapeutic goals for adults are the prevention of Addisonian crisis and ensuring the best possible quality of life, including fertility.Key words: androgens - cardiovascular risk - congenital adrenal hyperplasia - bone density - testicular rest tumors.

  2. Insufficiency fracture after radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Dong Ryul; Huh, Seung Jae [Dept.of Radiation Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2014-12-15

    Insufficiency fracture occurs when normal or physiological stress applied to weakened bone with demineralization and decreased elastic resistance. Recently, many studies reported the development of IF after radiation therapy (RT) in gynecological cancer, prostate cancer, anal cancer and rectal cancer. The RT-induced insufficiency fracture is a common complication during the follow-up using modern imaging studies. The clinical suspicion and knowledge the characteristic imaging patterns of insufficiency fracture is essential to differentiate it from metastatic bone lesions, because it sometimes cause severe pain, and it may be confused with bone metastasis.

  3. Determination of Adrenal Volume by MRI in Healthy Children

    DEFF Research Database (Denmark)

    Mouritsen, Annette; Lindhardt Johansen, Marie; Wohlfart-Veje, Christine;

    2014-01-01

    Adrenal disorders like Congenital Adrenal Hyperplasia result in abnormal adrenal size and morphology, but little is known about the clinical value of magnetic resonance imaging (MRI) in determining adrenal volume....

  4. New insights into the controversy of adrenal function during critical illness.

    Science.gov (United States)

    Boonen, Eva; Bornstein, Stefan R; Van den Berghe, Greet

    2015-10-01

    Critical illness represents a life-threatening disorder necessitating recruitment of defence mechanisms for survival. Herein, the hypothalamic-pituitary-adrenal axis is essential. However, the relevance of a relative insufficiency of the hypothalamic-pituitary-adrenal axis in critical illness, which is diagnosed by a suppressed cortisol response to exogenous adrenocorticotropic hormone (ACTH) irrespective of the plasma cortisol concentration, is controversial. Findings from several studies have provided insights that clarify at least part of this controversy. Rather than an activated hypothalamic-pituitary-adrenal axis, ACTH-independent regulators have been reported to contribute to increased cortisol availability during critical illness. One of these regulators is reduced cortisol breakdown, mediated by suppressed expression and activity of cortisol metabolising enzymes in the liver and kidneys. This downstream mechanism increases concentrations of plasma cortisol, but the ensuing feedback-inhibited ACTH release, when sustained for more than 1 week, has been shown to negatively affect adrenocortical integrity and function. Reduced adrenocortical ACTH signalling could explain reduced cortisol responses to exogenous ACTH. Whether such reduced cortisol responses in the presence of raised plasma (free) cortisol identifies adrenal failure needing treatment is unlikely. Additionally, reduced cortisol breakdown affects the optimum dose of hydrocortisone treatment during critical illness. Identification of patients with an insufficient hypothalamic-pituitary-adrenal axis response and the optimum treatment for this disorder clearly need more well designed preclinical and clinical studies.

  5. Cushing syndrome due to adrenal tumor

    Science.gov (United States)

    Adrenal tumor - Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the ... or cancerous (malignant). Noncancerous tumors that can cause ... Adrenal adenomas Micronodular hyperplasia Cancerous tumors that ...

  6. Genetics Home Reference: primary macronodular adrenal hyperplasia

    Science.gov (United States)

    ... Genetics Home Health Conditions PMAH primary macronodular adrenal hyperplasia Enable Javascript to view the expand/collapse boxes. ... Open All Close All Description Primary macronodular adrenal hyperplasia ( PMAH ) is a disorder characterized by multiple lumps ( ...

  7. Principles and management of adrenal cancer

    Energy Technology Data Exchange (ETDEWEB)

    Javadpour, N. (ed.)

    1987-01-01

    Principles and Management of Adrenal Cancer is a comprehensive presentation of the medical and surgical management of neoplastic diseases of the adrenal glands. It consists of two parts. The first provides an overview of the embryology, anatomy, physiology, pathology, and advances in methods of diagnosis and imaging techniques. The second deals with specific diseases of the adrenal cortex and medulla. With 121 figs.

  8. Relationship between adrenal function and prognosis in patients with severe sepsis

    Institute of Scientific and Technical Information of China (English)

    YANG Yi; LIU Ling; ZHAO Bo; LI Mao-qin; WU Bin; YAN Zheng; GU Qin; SUN Hua; QIU Hai-bo

    2007-01-01

    Background It is known that the hypothalamic-pituitary-adrenal (HPA) axis is highlighted by stimulation, such as sepsis,trauma, etc, when corticortropin increases and plasma cortisol levels enhance. Relative adrenal insufficiency is not uncommon in critically ill patients and may occur in severe sepsis patients with high plasma cortisol levels. It has been demonstrated that a short corticotropin test has a good prognostic value and is helpful in identifying patients with septic shock at high risk for death, but it has not been established for all severe sepsis patients, especially in China. The aim of this study is to explore the relationship between adrenal function and prognosis in patients with severe sepsis.Methods This prospective study was conducted between July and December 2004 in 6 teaching hospitals. Two hundred and forty patients with severe sepsis were enrolled in this study. A short corticotropin stimulation test was performed in all patients by intravenous injection of 250 μg of corticotropin. Blood samples were taken immediately before the test (T0), 30 (T30) and 60 (T60) minutes afterward, and the plasma cortisol concentration was measured by radio-immunoassay. At the onset of severe sepsis, the following parameters were recorded: age, sex, Acute Physiology and Chronic Health Evaluation (APACHE) Ⅱ, heart rate, mean arterial pressure (MAP), arterial partial pressure of oxygen (PaO2)/fraction of inspired oxygen (FiO2), peripheral blood of hemoglobin, platelets and leukocyte concentration and the number of organ failure. Patients were designated into two groups (survival and non-survival groups) according to the 28-day mortality. Relative adrenal insufficiency was defined as the difference between T0 and the highest value of T30 or T60 (△Tmax) ≤9 μg/dl.Results (1) Two hundred and forty patients with severe sepsis were included in this study, with 134 patients in the survival group and 106 in the non-survival group. The 28-day mortality was 44

  9. Adrenal Hypoplasia Congenita Presenting as Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Jennifer L. Flint

    2013-01-01

    Full Text Available We report on a patient with genetically confirmed adrenal hypoplasia congenita (AHC whose presentation and laboratory abnormalities were consistent with the more common condition, congenital adrenal hyperplasia (CAH. The patient presented with failure to thrive and salt wasting. General appearance showed marked hyperpigmentation and normal male genitalia. He displayed mildly elevated 17-hydroxyprogesterone and markedly elevated 11-deoxycortisol levels at baseline and with ACTH stimulation testing. Results were consistent with 11β-hydroxylase deficiency. He required glucocorticoids and high doses of mineralocorticoids. The marked elevation in 11-deoxycortisol directed our clinical reasoning away from a hypoplastic condition and towards a hyperplasic adrenal condition. Sequencing of the DAX1 gene (named for dosage-sensitive sex reversal (DSS locus and the AHC locus on the X chromosome revealed a missense mutation. A review of the literature revealed that elevated 11-deoxycortisol levels have been noted in kindreds with DAX1 mutations, but only when measured very early in life. A mouse model has recently been described that displays elevated 11-deoxycorticosterone levels and evidence for hyperplasia of the zona glomerulosa of the adrenal gland. We conclude that DAX1 testing may be considered in patients with laboratory evidence of 11β-hydroxylase deficiency, especially in those with severe salt wasting.

  10. Função adrenal na sepse e choque séptico Adrenal function in sepsis and septic shock

    Directory of Open Access Journals (Sweden)

    Cristiane Freitas Pizarro

    2007-11-01

    Full Text Available OBJETIVO: Rever os critérios diagnósticos e o tratamento de insuficiência adrenal, em pacientes da faixa etária pediátrica, com sepse grave e choque séptico. FONTES DOS DADOS: Os artigos foram selecionados através das bases de dados MEDLINE (1966-junho 2007, Embase (1994-2007 e Cochrane Library (2000-2007. As seguintes palavras-chave foram utilizadas: choque séptico, sepse, corticosteróides, insuficiência adrenal e crianças. SÍNTESE DOS DADOS: Não existe um critério bem estabelecido e aceito para definir insuficiência adrenal em pacientes criticamente enfermos. A incidência de insuficiência adrenal varia de acordo com o critério utilizado, podendo alcançar desde valores inferiores a 15% até superiores a 61%. O teste rápido de estímulo com hormônio adrenocorticotrófico (ACTH é largamente utilizado como um teste simples para a identificação de não responsividade adrenocortical, mas existe muita discussão quanto à dose de corticotropina a ser utilizada. A dose de 250 µg é a dose padrão. Recentemente, baixas doses de corticotropina (1 µg têm sido propostas, com a sugestão de que elas possam ter uma maior sensibilidade. Dúvidas ainda persistem quanto à eficácia da reposição com baixas doses de corticosteróides em crianças com choque refratário às catecolaminas. Mais estudos são necessários para determinar se o tratamento de tais pacientes alteraria morbidade e/ou mortalidade. CONCLUSÃO: Insuficiência adrenal é comum em crianças com sepse grave e choque séptico e pode contribuir para o desenvolvimento de choque refratário às catecolaminas. Contudo, dúvidas ainda persistem em relação à eficácia da terapêutica com baixas doses de corticosteróides.OBJECTIVE:To review diagnostic criteria and treatment of adrenal insufficiency in pediatric patients with severe sepsis and septic shock. SOURCES: Articles were selected using MEDLINE (1966-June 2007, Embase (1994-2007 and Cochrane Library (2000

  11. Hypothalamo-pituitary and immune-dependent adrenal regulation during systemic inflammation.

    Science.gov (United States)

    Kanczkowski, Waldemar; Alexaki, Vasileia-Ismini; Tran, Nguyen; Großklaus, Sylvia; Zacharowski, Kai; Martinez, Antoine; Popovics, Petra; Block, Norman L; Chavakis, Triantafyllos; Schally, Andrew V; Bornstein, Stefan R

    2013-09-03

    Inflammation-related dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis is central to the course of systemic inflammatory response syndrome or sepsis. The underlying mechanisms, however, are not well understood. Initial activation of adrenocortical hormone production during early sepsis depends on the stimulation of hypothalamus and pituitary mediated by cytokines; in late sepsis, there is a shift from neuroendocrine to local immune-adrenal regulation of glucocorticoid production. Therefore, the modulation of the local immune-adrenal cross talk, and not of the neuroendocrine circuits involved in adrenocorticotropic hormone production, may be more promising in the prevention of the adrenal insufficiency associated with prolonged sepsis. In the present work, we investigated the function of the crucial Toll-like receptor (TLR) adaptor protein myeloid differentiation factor 88 (MyD88) in systemic and local activation of adrenal gland inflammation and glucocorticoid production mediated by lipopolysachharides (LPSs). To this end, we used mice with a conditional MyD88 allele. These mice either were interbred with Mx1 Cre mice, resulting in systemic MyD88 deletion, predominantly in the liver and hematopoietic system, or were crossed with Akr1b7 Cre transgenic mice, resulting thereby in deletion of MyD88, which was adrenocortical-specific. Although reduced adrenal inflammation and HPA-axis activation mediated by LPS were found in Mx1(Cre+)-MyD88(fl/fl) mice, adrenocortical-specific MyD88 deletion did not alter the adrenal inflammation or HPA-axis activity under systemic inflammatory response syndrome conditions. Thus, our data suggest an important role of immune cell rather than adrenocortical MyD88 for adrenal inflammation and HPA-axis activation mediated by LPS.

  12. Adrenal hemorrhage in a newborn.

    Science.gov (United States)

    Abdu, Arebu T; Kriss, Vesna M; Bada, Henrietta S; Reynolds, Eric W

    2009-09-01

    Sometimes in the course of care in a neonatal intensive care unit, there may be a rush to intervene in cases where limited intervention is actually the correct course. One such example is that of neonatal adrenal hemorrhage. We present the case of a male term neonate with shock, metabolic acidosis, distended abdomen, and falling hematocrit. His prenatal and delivery histories were uneventful except for a nuchal cord. Apgar scores were 9 and 9. Because of his dramatic presentation, certain members of the medical team suggested immediate surgical intervention. However, a calm and careful evaluation revealed the true diagnosis and course of action. Ultrasound of the abdomen showed a mass between the liver and kidney, but the origin was difficult to identify. A computed tomography scan supported the diagnosis of right adrenal hemorrhage. His serum cortisol level was normal. The patient was managed conservatively and discharged home after a 1-week stay in the hospital. Subsequent abdominal ultrasound showed resolving adrenal hemorrhage with minimal calcification. A review of the pertinent literature is presented. Physicians should remember adrenal hemorrhage when evaluating a newborn infant with shock, acidosis, abdominal distention, and falling hematocrit and that conservative management is usually indicated.

  13. Quantification of the adrenal cortex hormones with radioimmunoassay

    Energy Technology Data Exchange (ETDEWEB)

    Badillo A, V.; Carrera D, A. A.; Ibarra M, C. M., E-mail: vbadillocren@hotmail.co [Universidad Autonoma de Zacatecas, Unidad Academica de Estudios Nucleares, Calle Cipres No. 10, Fracc. La Penuela, 98068 Zacatecas (Mexico)

    2010-10-15

    The pathologies of the adrenal cortex -adrenal insufficiency and Cushing syndrome- have their origin on the deficit or hypersecretion of some of the hormones that are secreted by the adrenal cortex, which is divided in three zones anatomically defined: the external zone, also called the zona glomerulosa, which is the main production site of aldosterone and mineralocorticoids; the internal zone, or zona reticularis, that produces androgens; and the external zone, or zone 1 orticotrop, which is responsible for producing glucocorticoids. In this work, a quantitative analysis of those hormones and their pathologic trigger was made; the quantification was made in the laboratory by means of highly sensitive and specific techniques, in this case, the radioimmunoassay, in which a radioisotope I-125 is used. This technique is based on the biochemical bond-type reaction, because it requires of a substance called the linker, which bonds to another called ligand. This reaction is also known as antigen-antibody (Ag-Ab), where the results of the reaction will depend on the quantity of antigen in the sample and on its affinity for the antibody. In this work, a 56 patients (of which 13 were men and 43 women) study was made. The cortisol, the ACTH, the androsterone and the DHEA values were very elevated in the majority of the cases corresponding to women, predominating cortisol; while in men, a notorious elevation of the 17 {alpha}-OH-PRG and of the DHEA-SO{sub 4} was observed. Based on that, we can conclude that 51 of them did not have mayor complications, because they just went to the laboratory once, while the remaining 5 had a medical monitoring, and they visited the laboratory more than one occasion, tell about a difficulty on their improvement. According to the results, an approximate relation of 8:2 women:men, respectively, becomes clear to the hormonal pathologies of the adrenal cortex. (Author)

  14. [Adrenal injury in blunt abdominal trauma].

    Science.gov (United States)

    Abakumov, M M; Smoliar, A N; Barmina, T G; Boĭko, A V; Shalimova, I G

    2009-01-01

    10 patients with adrenal damage were observed during 2.5 years. It amounted 0.93% of all patients with closed abdominal injuries. The right adrenal gland was traumatized in all cases evidently due to it's compression between right lobe of liver and vertebral column. Adrenal damage is observed quite often in combination with injuries of right liver lobe, right kidney and retroperitoneal hematoma formation. 5 patients underwent laparotomy on account of intra-abdominal bleeding, but adrenal damage was never revealed. Ultrasound and tomographic semiotics of adrenal damage was worked out, which allowed ascertaining diagnosis in 80% on application of ultrasound study and in 100% at computer tomography. Injury of one adrenal gland was not accompanied by adrenal failure and did not require hormonal replacement therapy.

  15. Intraoperative identification of adrenal-renal fusion

    Science.gov (United States)

    Boll, Griffin; Rattan, Rishi; Yilmaz, Osman; Tarnoff, Michael E

    2015-01-01

    Adrenal - renal fusion is a rare entity defined as incomplete encapsulation of the adrenal gland and kidney with histologically adjacent functional tissue. This report describes the first published intraoperative identification of this anomaly during laparoscopic adrenalectomy. The patient was a 59-year-old man with chronic hypertension refractory to multiple antihypertensives found to be caused by a right-sided aldosterone-producing adrenal adenoma in the setting of bilateral adrenal hyperplasia. During laparoscopic adrenalectomy, the normal avascular plane between the kidney and adrenal gland was absent. Pathologic evaluation confirmed adrenal - renal fusion without adrenal heterotopia. Identified intraoperatively, this may be misdiagnosed as invasive malignancy, and thus awareness of this anomaly may help prevent unnecessarily morbid resection. PMID:26195881

  16. Adrenal adrenoceptors in heart failure

    Directory of Open Access Journals (Sweden)

    Claudio ede Lucia

    2014-07-01

    Full Text Available Heart failure (HF is a chronic clinical syndrome characterized by the reduction in left ventricular (LV function and it represents one of the most important causes of morbidity and mortality worldwide. Despite considerable advances in pharmacological treatment, HF represents a severe clinical and social burden. Sympathetic outflow, characterized by increased circulating catecholamines (CAs biosynthesis and secretion, is peculiar in HF and sympatholytic treatments (as β-blockers are presently being investigated for the treatment of this disease. Adrenal gland secretes Epinephrine (80% and Norepinephrine (20% in response to acetylcholine stimulation of nicotinic cholinergic receptors on the chromaffin cell membranes. This process is regulated by adrenergic receptors (ARs: α2ARs inhibit CA release through coupling to inhibitory Gi-proteins, and βARs (mainly β2ARs stimulate CA release through coupling to stimulatory Gs-proteins. All ARs are G-protein-coupled receptors (GPCRs and GPCR kinases (GRKs regulate their signaling and function. Adrenal GRK2-mediated α2AR desensitization and downregulation are increased in HF and seem to be a fundamental regulator of CA secretion from the adrenal gland. Consequently, restoration of adrenal a2AR signaling through the inhibition of GRK2 is a fascinating sympatholytic therapeutic strategy for chronic HF. This strategy could have several significant advantages over existing HF pharmacotherapies (antiadrenergic, such as bAR-blockers minimizing side-effects on extra-cardiac tissues and reducing the chronic activation of the renin–angiotensin–aldosterone and endothelin systems.The role of adrenal ARs in regulation of sympathetic hyperactivity opens interesting perspectives in understanding pathophysiology of HF and identifying new potential therapeutic targets.

  17. Silent intravascular lymphoma initially manifesting as a unilateral adrenal incidentaloma.

    Science.gov (United States)

    Takahashi, Yoshiko; Iida, Keiji; Hino, Yasuhisa; Ohara, Takeshi; Kurahashi, Toshifumi; Tashiro, Takashi; Chihara, Kazuo

    2012-01-01

    Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of malignant lymphoma. Although the involvement of adrenal glands in IVLBCL is often observed, primary adrenal IVLBCL is rare. Most reported cases of adrenal IVLBCL showed bilateral lesions resulting in rapidly progressive adrenal failure and poor prognosis. Here, we report a case of slowly progressive primary adrenal IVLBCL manifesting initially with unilateral adrenal incidentaloma. This case is a silent IVLBCL and shows that the enlargement of both adrenal glands can be followed.

  18. Adrenal Crisis Caused by Inhaled Fluticasone in an Adolescent with Cystic Fibrosis and Advanced Hepatopathy: A Case Report

    Directory of Open Access Journals (Sweden)

    C. Denne

    2012-01-01

    Full Text Available Inhaled corticosteroids are widely accepted in the treatment of cystic fibrosis. Long-term use may cause systemic complications, especially high-dose fluticasone. We report about a young man who presented with encephalopathy after excessive physical activity caused by secondary adrenal insufficiency. He recovered quickly after systemic corticosteroid replacement therapy. This problem is considered to be underdiagnosed in clinical practice.

  19. [The Insufficiency Type of Metabolic Balance and Nutritional Status in Patient With Heart Failure].

    Science.gov (United States)

    Obrezan, A G; Spitsa, S A

    2016-08-01

    A lot of data has been accumulated at present on the metabolic and nutritional insufficiency in patients with chronic heart failure (CHF). Researchers have noted an actuality of the study of nutritional and metabolic insufficiency in CHF patients as independent factors affecting the course of CHF and because of its relationship with triggers of inflammation, sympathetic-adrenal system, and markers of functional state of the cardiovascular system (CVS). In some works attention has been given to relationship of nutritional and metabolic insufficiency with autonomic regulation of cardiac rhythm. Attempts have been made to systematize obtained data, create contemporary pathophysiological portrait of CHF development and highlight prevailing mechanisms of progression of the disease. This review summarizes accumulated data on nutritional insufficiency in patients with CHF and systematizes metabolic changes according to types of metabolism and CHF functional class. We have also made an attempt of creating figurative representation of pathophysiological mechanism of development of nutritional insufficiency in CHF. We have emphasized the problem of inadequate knowledge on contribution of inflammatory markers in development of nutritional insufficiency especially in patients with I-II functional classes of CHF. We also present suggestions concerning strategy of studying nutritional insufficiency in CHF, its classification according to CHF functional class, stratification of risk factors of its development, and the methods of its prevention and correction.

  20. Review of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?

    OpenAIRE

    Lee, Peter A; Houk, Christopher P.

    2010-01-01

    There is ample historical verification of 46,XX congenital adrenal hyperplasia (CAH) patients being born with essentially male genitaliawhile outcome information is scant. Prior to glucocorticoid therapy, most patients died very young from adrenal insufficiency. Most available reports from laterchildhood, contain little information concerning sexual identity. Reports on older individuals lack adequate information about sexual identity and quality of life. The difficulty in assessing the relat...

  1. Review of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?

    OpenAIRE

    Lee PeterA; Houk ChristopherP

    2010-01-01

    There is ample historical verification of 46,XX congenital adrenal hyperplasia (CAH) patients being born with essentially male genitaliawhile outcome information is scant. Prior to glucocorticoid therapy, most patients died very young from adrenal insufficiency. Most available reports from laterchildhood, contain little information concerning sexual identity. Reports on older individuals lack adequate information about sexual identity and quality of life. The difficulty in assessing the rela...

  2. Primary bilateral adrenal intravascular large B-cell lymphoma associated with adrenal failure.

    Science.gov (United States)

    Fukushima, Ayumi; Okada, Yosuke; Tanikawa, Takahisa; Onaka, Takashi; Tanaka, Aya; Higashi, Takehiro; Tsukada, Junichi; Tanaka, Yoshiya

    2003-07-01

    We report a rare case of bilateral primary adrenal non-Hodgkin's lymphoma with adrenal failure. A 66-year-old woman developed symptoms of adrenal failure. The cause of adrenal failure was suspected to be malignant lymphoma based on the high levels of serum soluble interleukin-2 receptor and LDH. Bilateral adrenalectomy was performed and pathological examination showed intravascular large B-cell lymphoma (IVL). Although complete remission was achieved, recurrence occurred three months later with brain metastases. IVL should be suspected in patients with bilateral adrenal tumors who present with rapidly progressive adrenal failure.

  3. What Are Some Types of Adrenal Gland Disorders?

    Science.gov (United States)

    ... Adrenal Gland Tumors Most adrenal gland tumors—abnormal growths on the adrenal glands—are not cancerous. They often do not cause symptoms or require treatment. However, adrenal gland tumors can produce a variety of different hormones, leading hormone levels to get too high. Adrenal ...

  4. High risk of adrenal insufficiency in adults previously treated for idiopathic childhood onset growth hormone deficiency

    DEFF Research Database (Denmark)

    Lange, Martin; Feldt-Rasmussen, Ulla; Svendsen, Ole Lander;

    2003-01-01

    The aim was to reevaluate a group of adults treated for idiopathic childhood onset GH deficiency (GHD) after 18 yr without GH treatment. Twenty-six (11 females) patients participated. All but two had isolated GHD. Childhood diagnosis was established by insulin tolerance test (ITT). The patients w...

  5. High risk of adrenal insufficiency in adults previously treated for idiopathic childhood onset growth hormone deficiency

    DEFF Research Database (Denmark)

    Lange, Martin; Feldt-Rasmussen, Ulla; Svendsen, Ole Lander;

    2003-01-01

    The aim was to reevaluate a group of adults treated for idiopathic childhood onset GH deficiency (GHD) after 18 yr without GH treatment. Twenty-six (11 females) patients participated. All but two had isolated GHD. Childhood diagnosis was established by insulin tolerance test (ITT). The patients...... were retested with an ITT to evaluate adult GH status. In five patients, an arginine and a synacthen test were performed instead of an ITT. Eleven of 25 patients had a subnormal cortisol response to ITT or synacthen. Ten patients had a GH peak less than 3.0 microg/liter (0.5. +/- 0.5 microg....../liter), whereas 16 patients displayed a normal GH response (12.3 +/- 10.6 microg/liter) after ITT. IGF-I values were decreased in the patients with a pathological retest as well as in patients with a normal GH response compared with controls (P

  6. Adrenal steroidogenesis disruption caused by HDL/cholesterol suppression in diethylstilbestrol-treated adult male rat.

    Science.gov (United States)

    Haeno, Satoko; Maeda, Naoyuki; Yamaguchi, Kousuke; Sato, Michiko; Uto, Aika; Yokota, Hiroshi

    2016-04-01

    The synthetic estrogen diethylstilbestrol is used to prevent miscarriages and as a therapeutic treatment for prostate cancer, but it has been reported to have adverse effects on endocrine homeostasis. However, the toxicity mechanism is poorly understood. Recently, we reported that diethylstilbestrol impairs adrenal steroidogenesis via cholesterol insufficiency in adult male rats. In the present study, we found that the adrenal cholesterol level was significantly reduced without of the decrease in other precursors in the adrenal steroidogenesis 24 h after a single dose of diethylstilbestrol (0.33 μg/g body mass). The serum HDL/cholesterol level was also reduced only 12 h after the diethylstilbestrol exposure. The level of Apo E, which is indispensable for HDL/cholesterol maturation, was decreased in both the HDL and VLDL/LDL fractions, whereas the level of Apo A1, which is an essential constituent of HDL, was not altered in the HDL fraction. Because the liver is a major source of Apo E and Apo A1, the secretion rates of these proteins were examined using a liver perfusion experiment. The secretion rate of Apo A1 from the liver was consistent between DES-treated and control rats, but that of Apo E was comparatively suppressed in the DES-treated rats. The disruption of adrenal steroidogenesis by diethylstilbestrol was caused by a decrease in serum HDL/cholesterol, which is the main source of adrenal steroidogenesis, due to the inhibition of Apo E secretion from the liver.

  7. Bilateral adrenal haemorrhage secondary to intra-abdominal sepsis: a case report.

    LENUS (Irish Health Repository)

    Egan, Aoife M

    2012-01-31

    INTRODUCTION: Bilateral adrenal haemorrhage is a rare cause of adrenal failure. Clinical features are non-specific and therefore a high index of suspicion must be maintained in patients at risk. Predisposing factors include infection, malignancy and the post-operative state. CASE PRESENTATION: We report the case of a patient who underwent a left hemicolectomy with primary anastomosis and formation of a defunctioning loop ileostomy for an obstructing colon carcinoma at the splenic flexure. En-bloc splenectomy was performed to ensure an oncologic resection. The patient developed a purulent abdominal collection post-operatively and became septic with hypotension and pyrexia. This precipitated acute bilateral adrenal haemorrhage with consequent adrenal insufficiency. Clinical suspicion was confirmed by radiological findings and a co-syntropin test. Following drainage of the collection, antibiotic therapy and corticosteroid replacement, the patient made an excellent recovery. CONCLUSION: This case highlights the importance of prompt diagnosis and treatment of adrenal failure. In their absence, this condition can rapidly lead to death of the patient.

  8. Laparoscopic Management of a Complex Adrenal Cyst

    Directory of Open Access Journals (Sweden)

    Koichi Kodama

    2015-01-01

    Full Text Available Adrenal cysts are rare, and their clinical management remains controversial. We report a case involving an adrenal cyst with a complicated appearance on radiological studies. Unenhanced computed tomography revealed a unilocular, noncalcified, hypoattenuating mass with a thin wall in the left adrenal gland. The lesion gradually increased in size from 10 to 50 mm at two-year follow-up. On contrast-enhanced magnetic resonance imaging, a mural nodule with contrast enhancement was observed. The entire adrenal gland was excised en bloc via a lateral transperitoneal laparoscopic approach without violating the principles of surgical oncology. The pathological diagnosis was an adrenal pseudocyst. Laparoscopic adrenalectomy is a safe option for the treatment of complex adrenal cysts, while maintaining the benefits of minimal invasiveness.

  9. Laparoscopic Resection of an Adrenal Schwannoma

    Science.gov (United States)

    Konstantinos, Toutouzas G.; Panagiotis, Kekis B.; Nikolaos, Michalopoulos V.; Ioannis, Flessas; Andreas, Manouras; Geogrios, Zografos

    2012-01-01

    Background and Objectives: Schwannomas are tumors originating from Schwann cells of the peripheral nerve sheath (neurilemma) of the neuroectoderm. Rarely, schwannomas can arise from the retroperitoneum and adrenal medulla. We describe a case of a 71-y-old woman who presented with an incidentally discovered adrenal tumor. Methods: Ultrasound and computed tomography scans revealed a lesion with solid and cystic areas originating from the left adrenal gland. The patient underwent complete laparoscopic resection of the tumor and the left adrenal gland. Results: Histopathological examination and immunohistochemical staining of the excised specimen revealed a benign schwannoma measuring 5.5×5×3.7 cm. To our knowledge, few other cases of laparoscopic resection of adrenal schwannomas have been reported. Conclusion: Because preoperative diagnosis of adrenal tumors is inconclusive, complete laparoscopic excision allows for definitive diagnosis with histological evaluation and represents the treatment of choice. PMID:23484583

  10. Adrenal Myelolipoma- A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Vijayalaxmi V. Suranagi

    2009-05-01

    Full Text Available Adrenal myelolipoma (AML is a rare benign tumour composed of mature adipose tissue and hematopoietic tissue. Very few cases have been reported. Most of these patients are asymptomatic. We present a rare case of Adrenal Myelolipoma where the patient presented with hypertension and a clinical suspicion of Pheochromocytoma, which turned out to be an Adrenal myelolipoma. Adrenal myelolipoma is a rare entity, not encountered frequently and can occur as an incidental finding. Awareness regarding this entity is very much essential to exclude surgical exploration or extensive surgery.

  11. Genetics Home Reference: X-linked adrenal hypoplasia congenita

    Science.gov (United States)

    ... X-linked adrenal hypoplasia congenita X-linked adrenal hypoplasia congenita Enable Javascript to view the expand/collapse ... Open All Close All Description X-linked adrenal hypoplasia congenita is a disorder that mainly affects males. ...

  12. Insufficient Sleep Is a Public Health Epidemic

    Science.gov (United States)

    ... this? Submit Button Past Emails CDC Features Insufficient Sleep Is a Public Health Problem Language: English Español ( ... insufficient sleep is an important public health concern. Sleep-Related Unhealthy Behaviors The Behavioral Risk Factor Surveillance ...

  13. CT diagnosis of adrenal abnormalities in patients with primary non-adrenal malignancies

    Energy Technology Data Exchange (ETDEWEB)

    Hussain, S.; Seltzer, S.E.; Abrams, H.L.; Belldegrun, A.; Richie, J.P.

    1986-05-01

    Fifty-seven patients with primary non-adrenal malignancy were found to have unsuspected adrenal abnormality on CT. In 33, comparison of histophatologic findings and/or the patients' hospital course or follow-up lead to the diagnosis of adrenal metastases (23), benign non-functioning adenomas (7), metastasis with hyperplasia (1), benign hyperplasia (1), and fatty infiltration (1). The analysis of CT findings indicated that: I) A heterogenous adrenal mass showing contrast enhancement was always metastatic, II) Nonfunctioning adenomas were always 3 cm or smaller in diameter, III) Bilateral adrenal masses and growth of adrenal mass on follow-up CT or regression on treatment indicated metastases, and IV) metastatic disease could not be excluded purely on the basis of the size of the adrenal mass.

  14. Puberty and fertility in congenital adrenal hyperplasia.

    NARCIS (Netherlands)

    Otten, B.J.; Stikkelbroeck, M.M.L.; Claahsen-van der Grinten, H.L.; Hermus, A.R.M.M.

    2005-01-01

    Congenital adrenal hyperplasia (CAH) is a disorder of adrenal steroid synthesis. The symptoms and signs of CAH depend on the degree of enzyme deficiency; severe salt-wasting (SW) form, less severe simple virilizing (SV) form and mild nonclassic (NC) form. In this paper, puberty and fertility in CAH

  15. Unilateral adrenal hemorrhagic infarction in essential thrombocythemia.

    Science.gov (United States)

    Burnet, G; Lambert, M; Annet, L; Lefebvre, C

    2015-12-01

    Adrenal hemorrhage is a rare disease associated with various conditions. We report a case of a 68-year-old woman with abdominal and back pain. The diagnostic work-up showed a left adrenal gland infarction associated with essential thrombocythemia. Treatment consisted in painkillers and treating the underlying condition in order to prevent further thrombotic events.

  16. Severe Hyperkalemia and Bilateral Adrenal Metastasis

    Directory of Open Access Journals (Sweden)

    Michael Nagler

    2009-01-01

    Full Text Available Adrenal metastases are a common finding in metastatic lung and breast cancer. Often there are no clinical symptoms suggesting them. In this paper, we present a case of a 66-year-old man with metastatic lung cancer suffering from severe hyperkaliemia due to hypoaldosteronism as a result of bilateral adrenal metastasis.

  17. Effect of low-dose glucocorticoid on corticosteroid insufficient patients with acute exacerbation of chronic obstructive pulmonary disease

    Institute of Scientific and Technical Information of China (English)

    袁光雄

    2014-01-01

    Objective To investigate the effect of low-dose glucocorticoid on prognosis of critical illness-related corticosteroid insufficient(CIRCI)patients with acute exacerbation of chronic obstructive pulmonary disease(AECOPD).Methods A total of 385 eligible patients met the criteria of AECOPD were admitted from January 2010 to December 2012.The AECOPD patients co-morbid with CIRCI screened by an adrenal corticotrophic hormone test within 12 hours after admission were randomly divided

  18. Genetics of primary ovarian insufficiency.

    Science.gov (United States)

    Rossetti, R; Ferrari, I; Bonomi, M; Persani, L

    2017-02-01

    Primary ovarian insufficiency (POI) is characterized by a loss of ovarian function before the age of 40 and account for one major cause of female infertility. POI relevance is continuously growing because of the increasing number of women desiring conception beyond 30 years of age, when POI prevalence is >1%. POI is highly heterogeneous and can present with ovarian dysgenesis and primary amenorrhea, or with secondary amenorrhea, and it can be associated with other congenital or acquired abnormalities. In most cases POI remains classified as idiopathic. However, the age of menopause is an inheritable trait and POI has a strong genetic component. This is confirmed by the existence of several candidate genes, experimental and natural models. The variable expressivity of POI defect may indicate that, this disease may frequently be considered as a multifactorial or oligogenic defect. The most common genetic contributors to POI are the X chromosome-linked defects. Here, we review the principal X-linked and autosomal genes involved in syndromic and non-syndromic forms of POI with the expectation that this list will soon be upgraded, thus allowing the possibility to predict the risk of an early age at menopause in families with POI.

  19. Study of awareness of adrenal disorders among interns and postgraduate students of Hamidia Hospital, Bhopal

    Science.gov (United States)

    Chittawar, Sachin; Dubey, T. N.; Sharma, Jitendra; Khandare, Sagar

    2017-01-01

    Introduction: Adrenal disorders could be a life-threatening emergency, hence requires immediate therapeutic management. For this awareness regarding its diagnosis, management, and treatment is prime important. Aims and Objective: To study the awareness of adrenal disorders among interns and postgraduates students of Hamidia Hospital, Bhopal. Materials and Methods: A cross-sectional questionnaire-based study was performed. Fifty-six participants, i.e., 1st, 2nd, and 3rd years postgraduate residents of general medicine (n = 14 × 3) and interns (n = 14) were included in the study. There were 12 questions on adrenal insufficiency, adrenal adenoma, congenital adrenal hyperplasia (CAH), nonclassical CAH (NCCAH), pheochromocytoma, and Conn's syndrome. One mark was awarded for each correct response. Results: In the present study, 14 (25%) participants scored < 5 marks, 33 (58.9%) scored between 6 and 9, and 9 (16.1%) scored between 10 and 12. The mean score among the participants was 6.38 ± 2.505, with a range from 2 to 11 marks. The number of correct answers by postgraduates residents of 1st year was 101, 2nd year was 95, and 3rd year was 93 and interns scored 68 out of total 168 questions in each group. Mean awareness score for residents of 1st, 2nd, 3rd years participants and interns was 7.21 ± 2.806, 6.79 ± 2.119, and 6.64 ± 2.818 and 6.63 ± 2.505, respectively. Most of the participants recorded correct responses related to diagnosis (57.7%) followed by responses related to treatment (64.3%). Answers to a question regarding how commonly is adrenal insufficiency diagnosed in medical Intensive Care Unit, none of the individuals responded correctly. Conclusion: There was a lack of awareness regarding diagnosis, management, and treatment of adrenal disorders in central India. We need to prioritize training related to these illnesses in our postgraduate teaching curriculum in practice. PMID:28217529

  20. Effects of intra-abdominal pressure on adrenal gland function and morphology in rats.

    Science.gov (United States)

    Akkapulu, Nezih; Tirnaksiz, Mehmet Bulent; Kulac, Ibrahim; Tezel, Gaye Guler; Hayran, Mutlu; Dogrul, Ahmet Bulent; Cetinkaya, Erdinc; Yorganci, Kaya

    2015-01-01

    Intra-abdominal hypertension and abdominal compartment syndrome (IAH/ACS) are life-threatening conditions and caused by several clinical status. Although there is insufficient data regarding its effects on adrenal glands. This study aimed to identify whether elevated intra-abdominal pressure (IAP) caused any alteration on the morphology and function of adrenal glands in a rat model. Twenty four Sprague-Dawley male rats were included in the study. Animals were allocated into 4 groups. IAP was elevated to 15 mmHg for one hour and four hours in group 2 and 4. Group 1 and 3 were sham groups. Blood samples were taken for the assessment of plasma adrenaline, noradrenaline, and corticosterone levels and adrenalectomies were performed to evaluate apoptosis. Blood adrenaline, noradrenaline and corticosterone levels were significantly higher in the study groups compared with the sham groups. However, there were no significant changes in apoptotic index scores in the study groups as compared to sham groups. These results support that increased IAH leads to discharge of catecholamine and corticosterone from the adrenal glands. Failure to demonstrate similar changes in apoptotic index score may be concluded as apoptosis is not a leading pathway for impairment of adrenal glands during IAH period.

  1. Silent Intravascular Lymphoma Initially Manifesting as a Unilateral Adrenal Incidentaloma

    Directory of Open Access Journals (Sweden)

    Yoshiko Takahashi

    2012-01-01

    Full Text Available Intravascular large B-cell lymphoma (IVLBCL is a rare subtype of malignant lymphoma. Although the involvement of adrenal glands in IVLBCL is often observed, primary adrenal IVLBCL is rare. Most reported cases of adrenal IVLBCL showed bilateral lesions resulting in rapidly progressive adrenal failure and poor prognosis. Here, we report a case of slowly progressive primary adrenal IVLBCL manifesting initially with unilateral adrenal incidentaloma. This case is a silent IVLBCL and shows that the enlargement of both adrenal glands can be followed.

  2. Adrenal pathology in childhood: a spectrum of disease

    Energy Technology Data Exchange (ETDEWEB)

    Paterson, Anne [Royal Belfast Hospital for Sick Children, 180 Falls Road, Belfast BT12 6BE (United Kingdom)

    2002-10-01

    Adrenal lesions in children may present with an asymptomatic adrenal mass lesion, an endocrinopathy, a hypertensive or metabolic crisis or a paraneoplastic syndrome. Some of the underlying disease processes remain localised within the adrenal gland or may develop into disseminated disease. The role of the radiologist is to confirm the adrenal location of a lesion and to document any other foci of disease. Ultrasound, CT, MR imaging and metaiodobenzylguanidine scintigraphy are the most commonly utilised imaging modalities for examining the adrenal glands and their pathologies in children. This paper reviews the spectrum of adrenal disease in childhood and presents the information as a series of radiological appearances: anomalies of adrenal shape, location, number and size, cystic adrenal masses, solid adrenal mass lesions, and adrenal calcifications are all examined. The radiological findings must always be interpreted in conjunction with the patient's age, the clinical history, the findings on physical examination and the available biochemical data. (orig.)

  3. Adrenal Collision Tumor: Coexistence of Pigmented Adrenal Cortical Oncocytoma and Ganglioneuroma

    Directory of Open Access Journals (Sweden)

    Hye Seung Lee

    2016-01-01

    Full Text Available Background. Adrenal collision tumors (ACTs, in which distinct tumors coexist without intermingling in the same adrenal gland, are rare and their actual prevalence is unknown. ACTs commonly consist of adrenal cortical adenoma, pheochromocytoma, or metastatic malignant tumor. Case Report. A 32-year-old woman who had been experiencing gastric discomfort for one month was referred to our hospital with abnormal imaging findings. The physical examination and the laboratory data including endocrine studies were unremarkable. Abdomen computed tomography (CT and magnetic resonance imaging (MRI showed two adjacent masses in the left suprarenal fossa, and a laparoscopic left adrenalectomy was done. Histological and immunohistochemical (IHC examinations revealed two distinct tumors: a pigmented adrenal cortical oncocytoma (ACO and a ganglioneuroma, respectively. Conclusion. Both tumors are rare in the adrenal gland and exist as ACTs only exceptionally rarely. This is the first reported case of coexisting oncocytoma and ganglioneuroma in the same adrenal gland to our knowledge.

  4. Lipid peroxidation in adrenal and testicular microsomes.

    OpenAIRE

    1981-01-01

    Studies were carried out to determine the actions of and interactions between ascorbate, NADPH, Fe2+, and Fe3+ on lipid peroxidation in adrenal and testicular microsomes. Ascorbate-induced malonaldehyde production was maximal in adrenal and testicular microsomes at an ascorbate concentration of 1 X 10(-4)M. Fe2+, at levels between 10(-6) and 10(-3)M, produced concentration-dependent increases in lipid peroxidation in adrenal and testicular microsomes; Fe2+ had a far greater effect than Fe3+ i...

  5. Exocrine pancreatic insufficiency following esophagectomy.

    Science.gov (United States)

    Huddy, J R; Macharg, F M S; Lawn, A M; Preston, S R

    2013-08-01

    Weight loss following esophagectomy is a management challenge for all patients. It is multifactorial with contributing factors including loss of gastric reservoir, rapid small bowel transit, malabsorption, and adjuvant chemotherapy. The development of a postoperative malabsorption syndrome, as a result of exocrine pancreatic insufficiency (EPI), is recognized in a subgroup of patients following gastrectomy. This has not previously been documented following esophageal resection. EPI can result in symptoms of flatulence, diarrhea, steatorrhea, vitamin deficiencies, and weight loss. It therefore has the potential to pose a significant level of morbidity in postoperative patients. There is some evidence that patients with proven EPI (fecal elastase-1 PERT). We observed symptoms compatible with EPI in a subgroup of patients following esophagectomy. We hypothesized that this was contributing to malabsorption and malnutrition in these patients. To investigate this, fecal elastase-1 was measured in postoperative patients, and in those with proven EPI, a trial of PERT was commenced in combination with specialist dietary education. At routine postoperative follow-up, which included assessment by a specialist dietitian, those patients with symptoms suggestive of malabsorption were given the opportunity to have their fecal elastase-1 measured. PERT was then offered to patients with fecal elastase-1 less than 200 μg/g (EPI) as well as those in the 200-500 μg/g range (mild EPI) with more severe symptoms. Fecal elastase-1 was measured in 63 patients between June 2009 and January 2011 at a median of 4 months (range 1-42) following surgery. Ten patients had fecal elastase-1 less than 200 μg/g, and all had failed to maintain preoperative weight. All accepted a trial of PERT. Nine (90%) had symptomatic improvement, and seven (70%) increased their weight. Thirty-nine patients had a fecal elastase-1 in the 200-500 μg/g range. Twelve were given a trial of PERT based on level of

  6. Postnatal changes in adrenal size in very low-birth-weight infants: sonographic evaluation for the prediction of late-onset glucocorticoid-responsive circulatory collapse.

    Science.gov (United States)

    Iijima, Shigeo; Uga, Naoki; Ohzeki, Takehiko

    2010-06-01

    We investigated the postnatal pattern of changes in adrenal size in very low-birth-weight (VLBW) infants and its relation to late-onset glucocorticoid-responsive circulatory collapse (LGCC) that may be associated with adrenal insufficiency. In 36 VLBW infants born at birth and unchanged at 3 weeks; group B (N = 24), the actual adrenal area was greater than or equal to the predicted value and decreased at 3 weeks; and group C (N = 6), the actual adrenal area was less than the predicted value and unchanged at 3 weeks. Five infants developed LGCC, and all five were in group A. These observations suggest that the life of the adrenal fetal zone might be extended beyond 3 weeks after birth in some VLBW infants and that prolonged fetal zone activity might correlate with LGCC. On the other hand, adrenal maturation might have already occurred at birth in some VLBW infants. Sonographic evaluation of adrenal size may enable prediction of subsequent LGCC in VLBW infants.

  7. Activities against hemostatic proteins and adrenal gland ultrastructural changes caused by the brown widow spider Latrodectus geometricus (Araneae: Theridiidae) venom.

    Science.gov (United States)

    Guerrero, Belsy; Finol, Hector J; Reyes-Lugo, Matias; Salazar, Ana M; Sánchez, Elda E; Estrella, Amalid; Roschman-González, Antonio; Ibarra, Carlos; Salvi, Ivan; Rodríguez-Acosta, Alexis

    2010-01-01

    Brown widow spider (BrWS) (Latrodectus geometricus) venom produces intense systemic reactions such as cramps, harsh muscle nociceptive, nauseas, vomiting and hypertension. The proposed pathogenic mechanisms resulting in these accidents have principally been damages occurring at the nervous system. However, it is suspected that there is also damage of the adrenal glands, as a result of the experimental animal's clinical manifestations, which developed symptoms compatible with acute adrenal insufficiency. We have currently found that the adrenal gland is damaged by this venom gland homogenates (VGH) producing severe alterations on cortex cells resulting in death by acute adrenal insufficiency. In general, the ultrastructural study on the glands of mice under transmission electronic microscopy observations showed alterations in the majority of the intracellular membranes within 3 to 24h. BrWSVGH also showed specific actions on extracellular matrix proteins such as fibronectin, laminin and fibrinogen. In addition, zymogram experiments using gelatin as substrates detected gelatinolytic activity. The molecular exclusion fractionation of crude BrWSVGH resulted in 15 fractions, of which F1 and F2 presented alpha/beta-fibrinogenase and fibronectinolytic activities. Fractions F6, F14 and F15 showed only alpha-fibrinogenase activity; in contrast, the gelatinolytic action was only observed in fraction F11. Only metalloproteinase inhibitors abolished all these proteolytic activities. Our results suggest that adrenal cortex lesions may be relevant in the etiopathogenesis of severe brown widow spider envenoming. To our knowledge, this is the first report on adrenal gland damages, fibrinogenolytic activity and interrelations with cell-matrix adhesion proteins caused by L.geometricus VGH. The venom of this spider could be inducing hemostatic system damages on envenomed patients.

  8. A case of adrenal Cushing’s syndrome with bilateral adrenal masses

    Directory of Open Access Journals (Sweden)

    Ya-Wun Guo

    2016-05-01

    Full Text Available A functional lesion in corticotrophin (ACTH-independent Cushing’s syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and 131I-6β-iodomethyl-19-norcholesterol (131I-NP-59 scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL. No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2μg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and 131I-NP-59 scintigraphy are good diagnostic methods for Cushing’s syndrome presenting with bilateral adrenal masses.

  9. Moderate dose inhaled corticosteroid-induced symptomatic adrenal suppression: case report and review of the literature.

    Science.gov (United States)

    Schwartz, Richard H; Neacsu, Otilia; Ascher, David P; Alpan, Oral

    2012-12-01

    Inhaled corticosteroids (ICS) are drugs of choice for persistent asthma. Less than 500 µg/d of fluticasone are believed to be safe. We found 92 cases of adrenal suppression in PubMed; among these cases there were 13 children who took 500 µg/d or less of fluticasone. Adrenal insufficiency was diagnosed in a 7-year-old boy on 460 µg ICS for 16 months, with a diagnosis of chronic persistent asthma. A random cortisol was nondetectable as was an early morning cortisol. ICS have greatly improved the day-to-day lives of children with chronic persistent asthma. Parents of children younger than 12 years, who use at least 400 µg of inhaled fluticasone (or bioequivalent), must be given oral and written instructions about warning symptoms of hypocortisolism. Major stress such as surgery, gastrointestinal, bronchopulmonary, or other systemic infections, and heat stress may mandate a written plan of action for use by hospital physicians.

  10. [Amyotrophic lateral sclerosis and respiratory insufficiency].

    Science.gov (United States)

    Siirala, Waltteri; Korpela, Jaana; Vuori, Arno; Saaresranta, Tarja; Olkkola, Klaus T; Aantaa, Riku

    2015-01-01

    Amyotrophic lateral sclerosis (ALS) is a disease causing degeneration of motor neurons, without any curative treatment. The most common cause of death is respiratory arrest due to atrophy of the respiratory musculature. ALS-associated respiratory insufficiency differs in mechanism from the more common causes of dyspnea, such as diseases of pulmonary or cardiac origin. Recognizing the respiratory insufficiency can be challenging for a clinician. It should be possible to predict the development of respiratory insufficiency in order to avoid leaving the treatment decisions concerning respiratory insufficiency to emergency services. Noninvasive ventilatory support can be used to alleviate the patient's dyspnea. It is actually recommended as the first-line treatment of ALS-associated respiratory insufficiency.

  11. Ultrasound diagnosis of adrenal hemorrhage in meningococcemia

    Energy Technology Data Exchange (ETDEWEB)

    Sarnaik, A.P.; Sanfilippo, D.J.K.; Slovis, T.L.

    1988-07-01

    Adrenal hemorrhage (AH) is a well-described complication of the neonatal period, anticoagulant therapy, and overwhelming bacterial infection especially with N. meningitis. Until recently the diagnosis of acute AH was based predominantly on autopsy findings. Ultrasound and computed tomography examinations have been successfully used for antemortem detection of AH in neonates and anticoagulated patients. We report two patients with fulminant meningococcal infection who demonstrated bilateral adrenal hemorrhages on ultrasonography.

  12. Immunologic, hemodynamic, and adrenal incompetence in cirrhosis

    DEFF Research Database (Denmark)

    Risør, Louise Madeleine; Bendtsen, Flemming; Møller, Søren

    2015-01-01

    dysfunction, but is not responsive to volume expansion. Recent research indicates that development of hepatic nephropathy represents a continuous spectrum of functional and structural dysfunction and may be precipitated by the inherent immunologic, adrenal, and hemodynamic incompetence in cirrhosis. New...... research explores several new markers of renal dysfunction that may replace serum creatinine in the future and give new insight on the hepatic nephropathy. Our understanding of the pathophysiological mechanisms causing the immunologic, adrenal, and hemodynamic incompetence, and the impact on renal...

  13. Metabolism of adrenal cholesterol in man

    Science.gov (United States)

    Borkowski, Abraham; Delcroix, Claude; Levin, Sam

    1972-01-01

    The synthesis of adrenal cholesterol, its esterification and the synthesis of the glucocorticosteroid hormones were studied in vitro on human adrenal tissue. It was found that the synthesis of adrenal cholesterol may normally be small in the zona “fasciculata,” particularly when compared with the synthesis of the glucocorticosteroid hormones, that it is several times higher in the zona “reticularis” where esterified cholesterol is less abundant, and that under ACTH stimulation it increases strikingly and proportionally to the degree of esterified adrenal cholesterol depletion. On the other hand, the relative rate of esterification as well as the concentration of free adrenal cholesterol are remarkably stable: they do not differ according to the adrenal zonation and are unaffected by ACTH. Furthermore, from a qualitative point of view, the relative proportions of Δ1 and Δ2 cholesteryl esters formed in situ are similar to those anticipated from their relative concentrations, suggesting that the characteristic fatty acid distribution of the adrenal cholesteryl esters results from an in situ esterification rather than from a selective uptake of the plasma cholesteryl esters. Besides, the in vitro esterification reveals a propensity to the formation of the most unsaturated cholesteryl esters. Regarding hydrocortisone and corticosterone, their synthesis tends to be more elevated in the zona “fasciculata.” Despite its higher cholesterol concentration the zona “fasciculata” should not therefore be viewed as a quiescent functional complement to the zona “reticularis” and the cortical distribution of glucocorticosteroid hormone synthesis is quite distinct from that of adrenal cholesterol synthesis. PMID:4338120

  14. Giant Adrenal Myelolipoma Masquerading as Heart Failure

    Directory of Open Access Journals (Sweden)

    Parijat S. Joy

    2014-03-01

    Full Text Available Adrenal myelolipomas are rare benign tumors of the adrenal cortex composed of adipose and hematopoietic cells. They have been postulated to arise from repeated stimulation by stress, inflammation and ACTH oversecretion. Myelolipomas are usually detected incidentally on imaging and do not require any active intervention besides regular follow-up by imaging. However, myelolipomas may insidiously grow to large sizes and cause mass effects and hemorrhage. Timely diagnosis and surgical resection are curative and lifesaving.

  15. Giant adrenal myelolipoma masquerading as heart failure.

    Science.gov (United States)

    Joy, Parijat S; Marak, Creticus P; Nashed, Nadia S; Guddati, Achuta K

    2014-01-01

    Adrenal myelolipomas are rare benign tumors of the adrenal cortex composed of adipose and hematopoietic cells. They have been postulated to arise from repeated stimulation by stress, inflammation and ACTH oversecretion. Myelolipomas are usually detected incidentally on imaging and do not require any active intervention besides regular follow-up by imaging. However, myelolipomas may insidiously grow to large sizes and cause mass effects and hemorrhage. Timely diagnosis and surgical resection are curative and lifesaving.

  16. Idiopathic Adrenal Hematoma Masquerading as Neoplasm

    Directory of Open Access Journals (Sweden)

    Kazuki Sasaki

    2012-04-01

    Full Text Available We report herein a case of idiopathic adrenal hematoma. A 59-year-old Japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the right upper abdominal cavity. The tumor was suspected to originate from either the posterior segment of the liver or the right adrenal gland. His chief complaint was weight loss of 8 kg over the previous 6 months. He had no past medical history and took no medications, including no anticoagulants. Laboratory data were almost normal except for a slight elevation of PIVKA-II. The origin of the tumor was found to be the adrenal gland, as angiography revealed the blood supply to the mass to derive from the right superior and inferior adrenal arteries. A fine needle biopsy of the lesion was unable to confirm the diagnosis. Open right adrenalectomy was performed. The histopathological findings of the surgical specimen revealed a hematoma with normal adrenal tissue. In the absence of any obvious etiology, the diagnosis was idiopathic adrenal hematoma.

  17. Cervical insufficiency and cervical cerclage.

    Science.gov (United States)

    Brown, Richard; Gagnon, Robert; Delisle, Marie-France; Gagnon, Robert; Bujold, Emmanuel; Basso, Melanie; Bos, Hayley; Brown, Richard; Cooper, Stephanie; Crane, Joan; Davies, Gregory; Gouin, Katy; Menticoglou, Savas; Mundle, William; Pylypjuk, Christy; Roggensack, Anne; Sanderson, Frank; Senikas, Vyta

    2013-12-01

    Objectif : La présente directive clinique a pour but de fournir un cadre de référence que les cliniciens pourront utiliser pour identifier les femmes qui sont exposées aux plus grands risques de connaître une insuffisance cervicale, ainsi que pour déterminer les circonstances en présence desquelles la mise en place d’un cerclage pourrait s’avérer souhaitable. Résultats : La littérature publiée a été récupérée par l’intermédiaire de recherches menées dans PubMed ou MEDLINE, CINAHL et The Cochrane Library en 2012 au moyen d’un vocabulaire contrôlé (p. ex. « uterine cervical incompetence ») et de mots clés appropriés (p. ex. « cervical insufficiency », « cerclage », « Shirodkar », « cerclage », « MacDonald », « cerclage », « abdominal », « cervical length », « mid-trimester pregnancy loss »). Les résultats ont été restreints aux analyses systématiques, aux essais comparatifs randomisés / essais cliniques comparatifs et aux études observationnelles. Aucune restriction n’a été appliquée en matière de date ou de langue. Les recherches ont été mises à jour de façon régulière et intégrées à la directive clinique jusqu’en janvier 2011. La littérature grise (non publiée) a été identifiée par l’intermédiaire de recherches menées dans les sites Web d’organismes s’intéressant à l’évaluation des technologies dans le domaine de la santé et d’organismes connexes, dans des collections de directives cliniques, dans des registres d’essais cliniques et auprès de sociétés de spécialité médicale nationales et internationales. Valeurs : La qualité des résultats est évaluée au moyen des critères décrits dans le rapport du Groupe d’étude canadien sur les soins de santé préventifs (Tableau). Recommandations 1. Les femmes qui sont enceintes ou qui planifient connaître une grossesse devraient faire l’objet d’une évaluation visant les facteurs de

  18. The agonistic adrenal: melatonin elicits female aggression via regulation of adrenal androgens.

    Science.gov (United States)

    Rendon, Nikki M; Rudolph, Lauren M; Sengelaub, Dale R; Demas, Gregory E

    2015-11-22

    Classic findings have demonstrated an important role for sex steroids as regulators of aggression, but this relationship is lacking within some environmental contexts. In mammals and birds, the adrenal androgen dehydroepiandrosterone (DHEA), a non-gonadal precursor of biologically active steroids, has been linked to aggression. Although females, like males, use aggression when competing for limited resources, the mechanisms underlying female aggression remain understudied. Here, we propose a previously undescribed endocrine mechanism regulating female aggression via direct action of the pineal hormone melatonin on adrenal androgens. We examined this in a solitary hamster species, Phodopus sungorus, in which both sexes are highly territorial across the seasons, and display increased aggression concomitant with decreased serum levels of sex steroids in short 'winter-like' days. Short- but not long-day females had increased adrenal DHEA responsiveness co-occurring with morphological changes in the adrenal gland. Further, serum DHEA and total adrenal DHEA content were elevated in short days. Lastly, melatonin increased DHEA and aggression and stimulated DHEA release from cultured adrenals. Collectively, these findings demonstrate that DHEA is a key peripheral regulator of aggression and that melatonin coordinates a 'seasonal switch' from gonadal to adrenal regulation of aggression by direct action on the adrenal glands.

  19. Ovarian adrenal rest tissue in congenital adrenal hyperplasia--a patient report.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Hulsbergen- van de Kaa, C.A.; Otten, B.J.

    2006-01-01

    We report a young girl who died in an Addisonian crisis due to previously undiagnosed congenital adrenal hyperplasia (CAH), in whom ovarian adrenal rest tissue was detected at postmortem histopathological examination. This is a very rare complication in female patients with CAH with only two previou

  20. Primary Bilateral Non-Hodgkin’s Lymphoma of the Adrenal Gland Presenting as Incidental Adrenal Masses

    Directory of Open Access Journals (Sweden)

    Christopher Rizzo

    2015-01-01

    Full Text Available Although lymphoma may occasionally involve the adrenal glands as part of a generalized disease process, primary adrenal lymphoma (PAL is a rare disease. We present a case of a 62-year-old woman with a history of mild/moderate hereditary spherocytosis with a well-compensated baseline haemoglobin, who presented with rapidly progressive symptomatic anaemia. During the diagnostic workup, imaging revealed bilateral large adrenal masses and she was later diagnosed with diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL, with the adrenal glands being the dominant site of the disease. The patient was started on systemic chemotherapy, but her disease progressed with neurological involvement which responded to second-line therapy. Her adrenal disease however was refractory to further therapy.

  1. Fuel oil-induced adrenal hypertrophy in ranch mink (Mustela vison): effects of sex, fuel oil weathering, and response to adrenocorticotropic hormone.

    Science.gov (United States)

    Mohr, F C; Lasley, B; Bursian, S

    2010-01-01

    Environmental contamination by petroleum hydrocarbons from anthropogenic sources can be a cause of stress for free-ranging wildlife. The response of wildlife to chemical contaminants requires that the hypothalamic-pituitary-adrenal (HPA) axis be precisely regulated to allow for proper glucocorticoid-mediated adaptive responses. Chronic oral exposure to low concentrations of bunker C fuel oil causes the development of adrenal hypertrophy in male ranch mink (Mustela vison) without increasing serum or fecal glucocorticoid concentrations. This hypertrophy is an adaptive response to fuel oil-induced adrenal insufficiency. To determine if the same phenomenon occurs in female mink or male mink exposed to artificially weathered fuel oil, female mink were fed 0 ppm (mineral oil) or 420 ppm fuel oil and male mink were exposed to 0 ppm, 420 ppm fuel oil, or 480 ppm artificially weathered fuel oil in the diet for 60-62 days. At the end of the exposure, serum glucocorticoid concentrations were assayed along with body and organ weight measurements. Fecal glucocorticoid concentrations were assayed at time points throughout the exposure. Male mink fed fuel oil or weathered fuel oil and female mink fed fuel oil had adrenal enlargement without any significant increases in the serum or fecal concentration of glucocorticoids, which is consistent with fuel oil-induced adrenal insufficiency. To address the physiological consequences of adrenal insufficiency, fuel oil-exposed male mink were administered an adrenocorticotropic hormone (ACTH) stimulation test. Fuel oil-exposed animals had a smaller incremental increase in serum glucocorticoid concentration after ACTH challenge compared to control animals. Our findings provide further evidence that the HPA axis of fuel oil-exposed animals is compromised and, therefore, not able to respond appropriately to the diverse stressors found in the environment.

  2. The adrenal cortex and life.

    Science.gov (United States)

    Vinson, Gavin P

    2009-03-05

    The template for our understanding of the physiological role of the adrenal cortex was set by Hans Selye, who demonstrated its key involvement in the response to stress, of whatever origin, and who also introduced the terms glucocorticoid and mineralocorticoid. Despite this, from the late 1940s on there was certainly general awareness of the multiple actions of glucocorticoids, including effects on the thymus and immune system, cardiovascular system, water balance, and the CNS. For these reasons, and perhaps because in the early studies of the actions of individual steroids there was less clear-cut difference between them, there was some initial resistance to the use of these terms. Today they are universal and unchallenged. It can be argued that, with respect to the glucocorticoids, this term colours our perception of their physiological importance, and may be misleading. By taking evidence from disease states, emphasis is placed on extreme conditions that do not necessarily reveal normal physiology. In particular, evidence for the role of glucocorticoid regulation of gluconeogenesis and blood glucose in the normal subject or animal is inconclusive. Similarly, while highly plausible theories explaining glucocorticoid actions on inflammation or the immune system as part of normal physiology have been presented, direct evidence to support them is hard to find. Under extreme conditions of chronic stress, the cumulative actions of glucocorticoids on insulin resistance or immunocompromise may indeed seem to be actually damaging. Two well-documented and long recognized situations create huge variation in glucocorticoid secretion. These are the circadian rhythm, and the acute response to mild stress, such as handling, in the rat. Neither of these can be adequately explained by the need for glucocorticoid action, as we currently understand it, particularly on carbohydrate metabolism or on the immune system. Perhaps we should re-examine other targets at the physiological

  3. What Are the Treatments for Congenital Adrenal Hyperplasia (CAH)?

    Science.gov (United States)

    ... Publications What are the treatments for congenital adrenal hyperplasia (CAH)? Skip sharing on social media links Share ... sees many CAH cases. 6 The Congenital Adrenal Hyperplasia Research, Education & Support (CARES) Foundation strongly recommends delaying ...

  4. Imaging of the adrenal gland lesions

    Energy Technology Data Exchange (ETDEWEB)

    Herr, Keith [Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA (United States); Muglia, Valdair F. [Universidade de Sao Paulo (FMRP/USP), Ribeirao Preto, SP (Brazil). Faculdade de Medicina; Koff, Walter Jose [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Faculdade de Medicina. Dept. de Cirurgia; Westphalen, Antonio Carlos, E-mail: antonio.westphalen@ucsf.edu [Departments of Radiology and Biomedical Imaging and Urology, School of Medicine, University of California, San Francisco, CA (United States)

    2014-07-15

    With the steep increase in the use of cross-sectional imaging in recent years, the incidentally detected adrenal lesion, or 'incidentaloma', has become an increasingly common diagnostic problem for the radiologist, and a need for an approach to classifying these lesions as benign, malignant or indeterminate with imaging has spurred an explosion of research. While most incidentalomas represent benign disease, typically an adenoma, the possibility of m alignant involvement of the adrenal gland necessitates a reliance on imaging to inform management decisions. In this article, we review the literature on adrenal gland imaging, with particular emphasis on computed tomography, magnetic resonance imaging, and photon-emission tomography, and discuss how these findings relate to clinical practice. Emerging technologies, such as contrast-enhanced ultrasonography, dual-energy computed tomography, and magnetic resonance spectroscopic imaging will also be briefly addressed. (author)

  5. Principles and management of adrenal cancer

    Energy Technology Data Exchange (ETDEWEB)

    Javadpour, N.

    1987-01-01

    This book provides information on adrenal diseases of latest developments and guides the clinicians in the care of their patients. The book is divided into two parts. The first section gives an overview of the embryology, anatomy, physiology, markers, pathology, imaging and the current progress in the field. The second edition covers specific diseases of the adrenal cortex and medulla. The increasingly significant roles played by steroids, catecholamines, blockers, computed tomography and magnetic resonance are elucidated and discussed. The contents include: Overview of progress; current problems, and perspectives - embryology anatomy, physiology, and biologic markers; pathology; advances in diagnosis; imaging techniques; adrenal disorders in childhood; primary aldosteronism; Cushing's syndrome; carcinoma; pheochromocytoma; neuroblastoma; metastatic disease; surgical management; and subject index.

  6. Imaging of the adrenal gland lesions

    Directory of Open Access Journals (Sweden)

    Keith Herr

    2014-08-01

    Full Text Available With the steep increase in the use of cross-sectional imaging in recent years, the incidentally detected adrenal lesion, or "incidentaloma", has become an increasingly common diagnostic problem for the radiologist, and a need for an approach to classifying these lesions as benign, malignant or indeterminate with imaging has spurred an explosion of research. While most incidentalomas represent benign disease, typically an adenoma, the possibility of malignant involvement of the adrenal gland necessitates a reliance on imaging to inform management decisions. In this article, we review the literature on adrenal gland imaging, with particular emphasis on computed tomography, magnetic resonance imaging, and photon-emission tomography, and discuss how these findings relate to clinical practice. Emerging technologies, such as contrast-enhanced ultrasonography, dual-energy computed tomography, and magnetic resonance spectroscopic imaging will also be briefly addressed.

  7. A success story in congenital adrenal hyperplasia.

    Science.gov (United States)

    Kriplani, Alka; Lunkad, Amol; Agarwal, Nutan; Kulshreshtha, Bindu; Ariachery, C Aminni

    2012-12-01

    Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders characterized by enzyme defects in adrenal steroidogenic pathways. CAH due to 21-hydroxylase deficiency accounts for 95 % of cases. This case was diagnosed to have simple virilizing type of CAH and started on dexamethasone, and underwent genitoplasty and clitoroplasty at 25 years of age, then was married 3 years after surgery and conceived spontaneously 2 years after marriage, to deliver a healthy male baby. Thus, proper diagnosis and treatment with steroids and genitoplasty can give females with CAH a normal sexual, normal menstrual, and reproductive function.

  8. Cystic Pheochromocytoma Presenting as Adrenal Cyst

    Science.gov (United States)

    Abdulsalam, Mohammed Shafi; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-01-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male. PMID:28050427

  9. Radiological findings of congenital lipoid adrenal hyperplasia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Jeong; Shin, Joo Yong; Lee, Hee Jung; Lee, Jin Hee; Sohn, Cheol Ho; Lee, Sung Moon; Kim, Hong; Woo, Seong Ku; Suh, Soo Ji [Keimyung Univ. School of Medicine, Taegu (Korea, Republic of)

    2001-05-01

    Congenital lipoid adrenal hyperplasia (CLAH) is a rare autosomal recessive disorder characterized by the marked accumulation of lipids and cholesterol in the adrenal cortex, and the failure of adrenal steroids to synthesise. We report the ultrasound (US), computed tomographic (CT), and magnetic resonance (MR) imaging findings in a four-day-old female neonate with CLAH.

  10. 儿童肾上腺危象的早期识别与治疗%Early recognition and treatment of adrenal crisis in children

    Institute of Scientific and Technical Information of China (English)

    张育才; 任玉倩

    2015-01-01

    Adrenal crisis is a life-threatening emergency caused by the destruction or altered function of the adrenal gland with a primary deficit in cortisol secretion(primary adrenal insufficiency)or by hypotha-lamic-pituitary pathologies determining a deficit of adrenocorticotropic hormone(secondary adrenal insuffi-ciency).Infection and abrupt end glucocorticoid treatment are the major precipitating causes of adrenal crisis. Patients with adrenal crisis typically present with hypovolemic shock or hypotension,nausea,vomiting,and fe-ver responding well to parenteral hydrocortisone administration.The main laboratory findings include lower serum cortisol concentrations,hyponatremia,hypoglycaemia and/or hyperkalemia.Delay diagnosis of adrenal insufficiency leads to adrenal crisis which is potentially lethal complication.Empirical glucocorticoid replace-ment should be initiated as soon as the suspicious of adrenal crisis,or sooner if the patient presents in adrenal crisis in critically ill children.%肾上腺危象是肾上腺皮质功能障碍的危急重症,原因是由于肾上腺病变导致皮质激素缺乏(原发性肾上腺功能障碍),或下丘脑-垂体-肾上腺轴功能障碍导致促肾上腺皮质激素分泌的缺陷,使受其调控的肾上腺糖皮质激素分泌功能受损。感染或停服激素是肾上腺危象的主要触发因素,典型临床表现是低血容量性休克或低血压、恶心、呕吐和可的松反应敏感的发热反应。主要实验室特点是低血液皮质醇水平、低钠血症、低血糖症和(或)高钾血症。延误肾上腺功能障碍的诊断经常是发生致死性肾上腺危象的原因。危重症患儿疑似肾上腺危象或存在危象表现时,应及早使用经验性糖皮质激素替代治疗。

  11. Bilateral adrenal histoplasmosis in an immunocompetent man Histoplasmose adrenal bilateral em um homem imunocompetente

    Directory of Open Access Journals (Sweden)

    Carlos Frederico Lopes Benevides

    2007-04-01

    Full Text Available Histoplasmosis is a fungal disease that is endemic in Brazil. It may present as chronic pulmonary infection or in disseminated form. Disseminated histoplasmosis frequently affects the adrenal gland; however, unilateral involvement in immunosuppressed patients is the usual presentation. We report a case of an elderly immunocompetent male with history of weight loss, fever and bilateral adrenal mass who was successfully treated with itraconazole.Histoplasmose é uma doença fúngica endêmica no Brasil que pode se apresentar como infecção pulmonar crônica ou na forma disseminada. A histoplasmose disseminada freqüentemente acomete a glândula adrenal; entretanto, ocorre mais em pacientes imunossuprimidos e de forma unilateral. Relatamos um caso de um homem idoso imunocompetente com história de perda de peso, febre e massa adrenal bilateral que foi tratada com itraconazol, com sucesso.

  12. Ultrasound follow up of testicular adrenal rest tumors with congenital adrenal hyperplasia: Report of three cases

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Jeong Yeon; Kim, Dong Won; Yoon, Seong Kuk; Nam, Kyung Jin [Dept. of Radiology, Dong-A University Hospital, Busan (Korea, Republic of)

    2014-12-15

    While testicular adrenal rest tumor is generally a rare intratesticular tumor, it is frequent in patients with congenital adrenal hyperplasia. The tumors are diagnosed and followed up by ultrasound examination because these tumors are non-palpable and symptomless in most cases and always benign. Ultrasound imaging features change depending on how congenital adrenal hyperplasia is controlled. We herein report three cases of testicular adrenal rest tumors with different usual and unusual imaging findings and follow-up imaging. Patient 1 was a 14-year-old boy who presented with poor compliance to medication. Patient 2 and 3 were a 10-year-old and 13-year-old boy who presented with precocious puberty and short stature, respectively. Ultrasound examinations demonstrated oval hypoechoic masses and irregular speculated hyperechoic masses in the testes and different serial imaging findings.

  13. Adrenal incidentaloma in neurofibromatosis type 1

    Directory of Open Access Journals (Sweden)

    Tančić-Gajić Milina

    2008-01-01

    Full Text Available INTRODUCTION Neurofibromatosis type 1 is one of the most common genetically transmitted diseases with a high index of spontaneous mutations and extremely varied and unpredictable clinical manifestations. It is diagnosed by the existence of certain clinical criteria. The presence of numerous localised cutaneous neurofibromas or a plexiform neurofibroma is virtually pathognomonic of neurofibromatosis type 1. The incidence of pheochromocytoma in neurofibromatosis type 1 is 0.1-5.7%. CASE OUTLINE A 56-year old female patient was admitted for further evaluation of incidental adrenal tumour previously diagnosed on computerized tomography (CT. She had previously unrecognized neurofibromatosis type 1 and a clinical picture which could remind of pheochromocytoma. None of the catecholamine samples in 24 hr urine indicated functionally active pheochromocytoma. Chromogranin A was moderately increased. Decision for operation was made after performing the image techniques. Adrenal incidentaloma had features of pheochromocytoma on abdominal magnetic resonance imaging (MRI, with positive 131I-MIBG (iodine 131-labelled metaiodobenzylguanidine scintigraphy. After being treated with phenoxybenzamine and propranolol, she was operated on. The pathohistological finding showed the case of left adrenal pheochromocytoma. CONCLUSION Detailed diagnostic procedure for pheochromocytoma should be performed with patients having neurofibromatosis type 1 and adrenal incidentaloma. Pheochromocytomas are rare tumours with fatal outcome if not duly recognized and cured.

  14. Imaging features of benign adrenal cysts

    Energy Technology Data Exchange (ETDEWEB)

    Sanal, Hatice Tuba [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey)]. E-mail: tubasanal@yahoo.com; Kocaoglu, Murat [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Yildirim, Duzgun [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Bulakbasi, Nail [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Guvenc, Inanc [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Tayfun, Cem [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Ucoz, Taner [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey)

    2006-12-15

    Benign adrenal gland cysts (BACs) are rare lesions with a variable histological spectrum and may mimic not only each other but also malignant ones. We aimed to review imaging features of BACs which can be helpful in distinguishing each entity and determining the subsequent appropriate management.

  15. Mielolipoma adrenal bilateral em um cão Bilateral adrenal myelolipoma in a dog

    Directory of Open Access Journals (Sweden)

    Silvia de Araújo França

    2008-08-01

    Full Text Available O presente relato descreve um caso de mielolipoma adrenal bilateral em uma cadela Poodle de 13 anos de idade. O cão morreu por peritonite séptica aguda, decorrente de ulceração e perfuração intestinal por corpo estranho linear. O neoplasma foi achado incidentalmente durante a necropsia. Macroscopicamente, as adrenais estavam redondas e com volume e aumentados. Ao corte elas eram friáveis, vermelho-amarronzadas, intercaladas por áreas branco-amareladas levemente proeminentes. Histologicamente, extensas áreas do parênquima continham adipócitos bem diferenciados, células hematopoéticas e macrófagos com hemossiderina, achados característicos de mielolipoma adrenal.A case of bilateral adrenal myelolipoma in a 13-year-old female Poodle is reported. Acute septic peritonitis due to intestinal ulceration and perforation caused by a linear foreign body was the cause of death. The adrenal neoplasm was an incidental necropsy finding. The adrenal glands were grossly rounded, enlarged and friable, and had red-brownish parenchyma interwoven by slightly prominent white-yellowish foci. Histologically, the findings of differentiated adipocytes in large areas of the parenchyma, in association to hematopoietic cells and macrophages with hemosiderin were consistent with the diagnosis of adrenal myelolipoma.

  16. Genotype-Phenotype Analysis in Congenital Adrenal Hyperplasia due to P450 Oxidoreductase Deficiency

    Science.gov (United States)

    Krone, Nils; Reisch, Nicole; Idkowiak, Jan; Dhir, Vivek; Ivison, Hannah E.; Hughes, Beverly A.; Rose, Ian T.; O'Neil, Donna M.; Vijzelaar, Raymon; Smith, Matthew J.; MacDonald, Fiona; Cole, Trevor R.; Adolphs, Nicolai; Barton, John S.; Blair, Edward M.; Braddock, Stephen R.; Collins, Felicity; Cragun, Deborah L.; Dattani, Mehul T.; Day, Ruth; Dougan, Shelley; Feist, Miriam; Gottschalk, Michael E.; Gregory, John W.; Haim, Michaela; Harrison, Rachel; Haskins Olney, Ann; Hauffa, Berthold P.; Hindmarsh, Peter C.; Hopkin, Robert J.; Jira, Petr E.; Kempers, Marlies; Kerstens, Michiel N.; Khalifa, Mohamed M.; Köhler, Birgit; Maiter, Dominique; Nielsen, Shelly; O'Riordan, Stephen M.; Roth, Christian L.; Shane, Kate P.; Silink, Martin; Stikkelbroeck, Nike M. M. L.; Sweeney, Elizabeth; Szarras-Czapnik, Maria; Waterson, John R.; Williamson, Lori; Hartmann, Michaela F.; Taylor, Norman F.; Wudy, Stefan A.; Malunowicz, Ewa M.; Shackleton, Cedric H. L.

    2012-01-01

    Context: P450 oxidoreductase deficiency (PORD) is a unique congenital adrenal hyperplasia variant that manifests with glucocorticoid deficiency, disordered sex development (DSD), and skeletal malformations. No comprehensive data on genotype-phenotype correlations in Caucasian patients are available. Objective: The objective of the study was to establish genotype-phenotype correlations in a large PORD cohort. Design: The design of the study was the clinical, biochemical, and genetic assessment including multiplex ligation-dependent probe amplification (MLPA) in 30 PORD patients from 11 countries. Results: We identified 23 P450 oxidoreductase (POR) mutations (14 novel) including an exonic deletion and a partial duplication detected by MLPA. Only 22% of unrelated patients carried homozygous POR mutations. p.A287P was the most common mutation (43% of unrelated alleles); no other hot spot was identified. Urinary steroid profiling showed characteristic PORD metabolomes with variable impairment of 17α-hydroxylase and 21-hydroxylase. Short cosyntropin testing revealed adrenal insufficiency in 89%. DSD was present in 15 of 18 46,XX and seven of 12 46,XY individuals. Homozygosity for p.A287P was invariably associated with 46,XX DSD but normal genitalia in 46,XY individuals. The majority of patients with mild to moderate skeletal malformations, assessed by a novel scoring system, were compound heterozygous for missense mutations, whereas nearly all patients with severe malformations carried a major loss-of-function defect on one of the affected alleles. Conclusions: We report clinical, biochemical, and genetic findings in a large PORD cohort and show that MLPA is a useful addition to POR mutation analysis. Homozygosity for the most frequent mutation in Caucasians, p.A287P, allows for prediction of genital phenotype and moderate malformations. Adrenal insufficiency is frequent, easily overlooked, but readily detected by cosyntropin testing. PMID:22162478

  17. Paraganglioma funcional extra-adrenal

    Directory of Open Access Journals (Sweden)

    Laura Arroyo-Martínez

    2006-03-01

    Full Text Available Los paragangliomas funcionales son tumores raros, se originan del tejido cromafín extraadrenal productor de catecolaminas, con frecuencia son malignos y tienen alta incidencia de enfermedad persistente o recurrente¹. Se les conoce como: glomus, quemodectomas, paragangliomas cromafines y glomerulocitomas. La localización es diversa y refleja la distribución paraganglionar en el cuerpo, desde la base del cráneo hasta el piso pélvico. Los paragangliomas se encuentran en donde hay ganglios del sistema autónomo, sin embargo, aproximadamente el 90% de estos tumores aparecen en las glándulas suprarrenales (y constituyen los feocromocitomas y el 10% restante tienen una ubicación extraadrenal, mas se ha dicho que su incidencia puede ser subestimada, variando del 18% al 22% en adultos, y en niños hasta un 30%. Los extra-adrenales se originan con mayor frecuencia en el abdomen (85%, otros en el tórax (12% y más raramente en la cabeza y el cuello (3% ². Los estudios de imágenes y la medición de la producción no fisiológica de catecolaminas pueden ayudar en el diagnóstico de esta entidad. La cirugía es el tratamiento de elección. Presentamos aquí el caso de una paciente de 32 años, primigesta con HTAIE que requirió cesárea, quien tuvo un postparto tórpido y pese a múltiples tratamientos antihipertensivos su patología fue de difícil manejo, con complicaciones oftálmicas. Tiempo después la paciente se estudia por hiperhidrosis, se solicitan exámenes de laboratorio e imágenes y se le documenta incidentalmente, una tumoración retroperitoneal izquierda, se le amplían los estudios, y se llega al diagnóstico correcto. La tumoración requirió resección quirúrgica. Tuvo un postoperatorio satisfactorio y la paciente egresó con control en la Consulta Externa.Functioning paragangliomas are rare tumors that produce catecholamines. They originate from extra-adrenal chromaffin cells. They are frequentIy malignant and are associated

  18. Laparoscope resection of ectopic corticosteroid-secreting adrenal adenoma.

    Science.gov (United States)

    Wang, Xian-Ling; Dou, Jing-Tao; Gao, Jiang-Ping; Zhong, Wen-Wen; Jin, Du; Hui, Lüzhao; Lu, Ju-Ming; Mu, Yi-Ming

    2012-01-01

    Tumors originating from ectopic adrenal tissue are relatively rare. In this article, we describe a case with Cushing's syndrome caused by an ectopic adrenal adenoma. A 38 year-old male patient presenting with cushingoid appearance for 2 years was diagnosed to have ACTH-independent Cushing's syndrome based on endocrinological evaluation. Mutiple radiological examinations detected bilateral adrenal atrophy. When the images were investigated in a more expanded scope, a 3.0×3.5×5.3 cm mass was detected in the anterior of left renal hilum and left renal vein. The mass was successfully resected with intraoperative endoscopy and pathological evaluation revealed an ectopic adrenal tumor. It is suggested that when the endocrinlogically confirmed adrenal neoplasm could not be well and definitely localized, the possibility of ectopic adrenal should be presumed and further radiography examinations should extend to the field where ectopic adrenal usually presents.

  19. Blunt adrenal gland trauma in the pediatric population.

    Science.gov (United States)

    Roupakias, Stylianos; Papoutsakis, Marinos; Mitsakou, Paraskevi

    2011-07-01

    A retrospective review of the literature was performed to determine the natural history, prevalence, prognosis and management of adrenal injury associated with blunt abdominal trauma in pediatric population. Blunt adrenal injury in children is uncommon, rarely isolated, and typically present as part of a multi organ trauma. Adrenal hemorrhage is being diagnosed more frequently since the emergence of computed tomography in modern emergency rooms. Obstetric birth trauma during vaginal delivery of a macrosomic fetus may result in neonatal adrenal hemorrhage. In children appear to be an incidental finding that resolves on follow-up imaging. Most of these injuries are self-limited and do not require intervention. The differential diagnosis of an adrenal neoplasm, especially in children with an isolated adrenal hemorrhage, must be considered. The presence of adrenal hemorrhage in the absence of a trauma history should alert to the possibility of pediatric inflicted injury.

  20. Adrenal Lymphangioma Masquerading as a Catecholamine Producing Tumor

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    Israel Hodish

    2015-01-01

    with an adrenal cystic lesion and biochemical testing concerning for pheochromocytoma. The pertinent diagnostic and imaging features of adrenal lymphangiomas are reviewed. Methods. We describe a 59-year-old patient who presented with hyperhidrosis and a 2.2 by 2.2 cm left adrenal nodule. Biochemical evaluation revealed elevated plasma-free normetanephrine, urine normetanephrine, urine vanillylmandelic acid, and urine norepinephrine levels. Elevated plasma norepinephrine levels were not suppressed appropriately with clonidine administration. Results. Given persistent concern for pheochromocytoma, the patient underwent adrenalectomy. The final pathology was consistent with adrenal lymphangioma. Conclusions. Lymphangiomas are benign vascular lesions that can very rarely occur in the adrenal gland. Imaging findings are generally consistent with a cyst but are nonspecific. Excluding malignancy in patients presenting with adrenal cysts can be difficult. Despite its benign nature, the diagnosis of adrenal lymphangioma may ultimately require pathology.

  1. [Chronic respiratory insufficiency and the elderly patient].

    Science.gov (United States)

    Cobarzan, Daniel

    2012-01-01

    Chronic respiratory failure is a complex entity of varied etiology and physio-pathological mechanisms. It is mainly characterised by the respiratory system's difficulty in ensuring correct aeration at rest, resulting initially in insufficient oxygenation of arterial blood. Treatment is adapted to each etiology and aims to compensate for respiratory failure and to ensure the oxygenation of the organism.

  2. Surgical strategy for mild ischemic mitral insufficiency

    Institute of Scientific and Technical Information of China (English)

    GU Cheng-xiong; WEI Hua; YU Yang

    2010-01-01

    @@ To the Editor: We read with the interest the article by FAN Hong-guang and colleagues~1 having obtained outstanding early and long-term clinical outcomes of left ventricular restoration for the patients with postinfarction ventricular aneurysm and low left ventricular ejection fraction (LVEF) of mean 37.7%. We would like to comment on surgical strategy for mild ischemic mitral insufficiency.

  3. AMH as Predictor of Premature Ovarian Insufficiency

    DEFF Research Database (Denmark)

    Lunding, Stine Aa; Aksglæde, Lise; Anderson, Richard A

    2015-01-01

    CONTEXT: The majority of Turner syndrome (TS) patients suffer from accelerated loss of primordial follicles. Low circulating levels of anti-Müllerian hormone (AMH) may predict the lack of spontaneous puberty in prepubertal girls and imminent premature ovarian insufficiency (POI) in TS women...

  4. [Postoperative respiratory insufficiency and its treatment].

    Science.gov (United States)

    Kösek, V; Wiebe, K

    2015-05-01

    The development of a postoperative respiratory insufficiency is typically caused by several factors and include patient-related risks, the extent of the procedure and postoperative complications. Morbidity and mortality rates in acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are high. It is important to have consistent strategies for prevention and preoperative conditioning is essential primarily for high-risk patients. Treatment of established postoperative lung failure requires early tracheotomy, protective ventilation (tidal volume 6 ml/kg body weight), elevated positive end expiratory pressure (PEEP, 10-20 mmH2O), recurrent bronchoscopy and early patient mobilization. In critical cases an extracorporeal lung assist is considered to be beneficial as a bridge to recovery and for realizing a protective ventilation protocol. Different systems with separate indications are available. The temporary application of a lung assist allows thoracic surgery to be performed safely in patients presenting with insufficient respiratory function.

  5. Convergence Insufficiency/Divergence Insufficiency Convergence Excess/Divergence Excess: Some Facts and Fictions

    OpenAIRE

    Edward Khawam; Bachir Abiad; Alaa Boughannam; Joanna Saade; Ramzi Alameddine

    2015-01-01

    Great discrepancies are often encountered between the distance fixation and the near-fixation esodeviations and exodeviations. They are all attributed to either anomalies of the AC/A ratio or anomalies of the fusional convergence or divergence amplitudes. We report a case with pseudoconvergence insufficiency and another one with pseudoaccommodative convergence excess. In both cases, conv./div. excess and insufficiency were erroneously attributed to anomalies of the AC/A ratio or to anomalies ...

  6. Cushing syndrome associated with an adrenal tumour.

    Science.gov (United States)

    Vieira, Helena; Brain, Caroline

    2012-08-27

    Cushing syndrome (CS) in children is a rare disorder that is most frequently caused by an adrenal tumour or a pituitary corticotrophin-secreting adenoma. The management is challenging and requires an individualised approach and multidisciplinary care. We present the case of a 23-month-old female child with a history of excessive weight gain, growth failure, hirsutism, acne and behavioural difficulties. Investigations revealed elevated serum midnight cortisol and 24 h urinary free cortisol. Overnight dexamethasone suppression testing showed no suppression of cortisol levels. Abdominal imaging revealed a right-sided suprarenal mass. She underwent right adrenalectomy and the histology showed an adrenal cortical carcinoma. There was clinical improvement with catch-up growth and weight normalisation. Despite being rare in clinical practice, in a child with weight gain, hirsuitism and growth failure the diagnosis must be considered. The overall prognosis of CS in childhood is good, but challenges remain to ensure normal growth and body composition.

  7. A case of treatment in a patient with synchronous bilateral renal cell carcinoma and simultaneous metastatic involvement of both adrenal glands: Clinical observation

    Directory of Open Access Journals (Sweden)

    V. R. Latypov

    2014-01-01

    Full Text Available Synchronous bilateral renal cell carcinoma occurs in 1.4 % of cases. The probability of bilateral adrenal metastases from renal cell carcinoma is less than 0.5 %. The clinical observation presents a case of synchronous bilateral renal cell carcinoma and simultaneous metastatic involvement of both adrenal glands. A 55‑year-old male patient was adm tted with the signs of hematuria and anemia to the Unit of Urology, Clinic of General Surgery, Siberian State Medical University. He was found to have synchronous bilateral renal cell carcinoma and simultaneous bilateral adrenal involvement. Sequential surgical treatment – radical nephrectomy (with adrenal gland removal on the right side and, after 3 months, adrenalectomy and kidney resection on the left side were performed. All the organs removed displayed tumors that proved to be renal cell carcinomas (a clear cell variant. There were lymph node metastases in the right-sided renal portal. Postoperatively, the investigators performed hormone replacement therapy for adrenal insufficiency, an immunotherapy cycle, three cycles of targeted therapy withsorafenib and sunitinib (at an interval of 0.5–2 years, and insulin therapy for new-onset diabetes mellitus. The duration of a follow-up was 6.2 years. When describing the case, the patient was alive and showed a generalized tumorous process with extensive tumor involvement of the solitary kidney. Sunitinib therapy was used.

  8. Functional ectopic adrenal carcinoma in a dog

    Science.gov (United States)

    Taylor, Jim A.; Lee, Maris S.; Nicholson, Matthew E.; Justin, Robert B.

    2014-01-01

    An 11-year-old spayed female pit bull terrier was presented with a 2-month history of polyuria, polydipsia, polyphagia, and panting. Serum chemistry, blood and urine analysis, and tests for hyperadrenocorticism suggested an adrenal tumor. Abdominal ultrasound identified a mass caudal to the right kidney. The mass was completely excised and histopathology was consistent with endocrine carcinoma. Three years later there was no evidence of recurrence or metastasis. PMID:25183891

  9. Renal infarction associated with adrenal pheochromocytoma.

    Science.gov (United States)

    Thewjitcharoen, Yotsapon; Atikankul, Taywin; Sunthornyothin, Sarat

    2013-09-01

    The coexistence of pheochromocytoma and renal artery stenosis had been reported occasionally from the possible mechanism of catecholoamine-induced vasospasm and extrinsic compression of renal artery in some reported cases. However, renal infarction caused by pheochromocytoma is an uncommon phenomenon. Herein, we report an interesting case of adrenal pheochromocytoma associated with renal artery thrombosis, which should be included in the differential diagnosis of pheochromocytoma patients who present with abdominal pain.

  10. Endoscopic Retroperitoneal Adrenalectomy for Adrenal Metastases

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    Gintaras Simutis

    2014-01-01

    Full Text Available Objectives. To evaluate whether retroperitoneal approach for adrenalectomy is a safe and effective treatment for adrenal metastases (AM. Methods. From June 2004 to January 2014, nine consecutive patients with AM were treated with endoscopic retroperitoneal adrenalectomy (ERA. A retrospective study was conducted, and clinical data, tumor characteristics, and oncologic outcomes were acquired and analyzed. Results. Renal cancer was the primary site of malignancy in 44.4% of cases. The mean operative time was 132 ± 10.4 min. There were 5 synchronous and 4 metachronous AM. One patient required conversion to transperitoneal laparoscopic procedure. No mortality or perioperative complications were observed. The median overall survival was 11 months (range: 2–42 months. Survival rates of 50% and 25% were identified at 1 and 3 years, respectively. At the end of the study, 4 patients were alive with a mean observed follow-up of 20 months. No patients presented with local tumor relapse or port-site metastases. Conclusions. This study shows that ERA is a safe and effective procedure for resection of AM and advances the surgical treatment of adrenal disease. The use of the retroperitoneal approach for adrenal tumors less than 6 cm can provide very favorable surgical outcomes.

  11. Left-sided giant adrenal myelolipoma secreting catecholamine

    Directory of Open Access Journals (Sweden)

    Sujatha Udupa

    2012-01-01

    Full Text Available Adrenal myelolipoma (AML is a rare benign tumor composed of mature adipose and hematopoietic tissue. Most of these patients are asymptomatic and the tumors are non-secreting. We present a case with a large functional adrenal myelolipoma, wherein the patient was hypertensive and biochemistry revealed increase in 24 hours urinary Vanillylmandelic Acid (VMA, a metabolite of catecholamine. The mass was removed surgically and diagnosed as adrenal myelolipoma on histopathological examination. Both his blood pressure and urinary VMA returned to normal following surgery, which suggested that the mass was functioning and was secreting catecholamine. To the best of our knowledge, a catecholamine secreting adrenal myelolipoma has been reported in the literature only once previously. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine, as seen in our case. We also review the literature on functioning adrenal myelolipoma.

  12. The adrenal gland and the patient with pulmonary tuberculosis infected with human immunodeficiency virus

    Directory of Open Access Journals (Sweden)

    Ifedayo Adeola Odeniyi

    2017-01-01

    Full Text Available Background: The adrenal gland is not spared from the involvement by tuberculosis. One of the recognized causes of adrenal insufficiency (AI is tuberculosis. AI, mostly at the subclinical level, is common in persons with pulmonary tuberculosis (PTB infection, occurring in about 23% of patients. Coinfection with PTB and human immunodeficiency virus (HIV may compromise adrenocortical function and produce significant adrenocortical insufficiency. Objective: To determine if coinfection with tuberculosis and HIV have a compound effect on adrenocortical function in persons with HIV and PTB coinfection. Materials and Methods: Persons with sputum-positive PTB, treatment naive, who met our inclusion criteria, were selected. All the recruited patients were screened for HIV and those positive for HIV infection had confirmatory test. A baseline blood samples for cortisol, fasting plasma glucose, full blood count, and electrolytes were collected between 8.00 h and 9.00 h immediately before administration of adrenocorticotropic hormone (ACTH. The persons received an intravenous bolus injection of 1 μg ACTH (Alliance Pharmaceuticals Ltd., Chippenham, Wiltshire SN15 2BB and blood sample was drawn for cortisol level at 30 min. Results: Forty-four people with PTB infection and forty people with PTB and HIV coinfection met the inclusion criteria of the study. The adrenal response to 1 μg ACTH stimulation in participants with PTB and PTB and HIV coinfection showed that the mean basal cortisol level in the 2 groups was not statistically significant; however, 30-min post-ACTH stimulation cortisol level was 630.84 ± 372.17 and 980.36 ± 344.82 nmol/L (P < 0.001 and increment was 367.79 ± 334.87 and 740.77 ± 317.97 nmol/L (P < 0.001, respectively. Fourteen persons (31.8% with PTB has subnormal adrenal response to ACTH stimulation while only 2 (5% persons with PTB and HIV coinfection has subnormal response. Conclusion: AI, at subclinical level, was less frequent in

  13. Curative surgical management of isolated adrenal recurrence of oesophageal adenocarcinoma.

    LENUS (Irish Health Repository)

    O'Sullivan, K E

    2013-01-01

    Adrenal metastases of oesophageal adenocarcinoma are rarely detected in the clinical setting, more frequently being found as an incidental postmortem finding in the presence of widespread metastases. With improvements in the sensitivity of radiological diagnostic modalities, the incidence of adrenal tumour detection is on the rise. We report herein a particularly rare case of primary operative management by adrenalectomy for an isolated right-sided adrenal metastasis secondary to oesophageal adenocarcinoma, with a long-term survival.

  14. Curative surgical management of isolated adrenal recurrence of oesophageal adenocarcinoma.

    Science.gov (United States)

    O'Sullivan, K E; Moriarty, A R; Larkin, J O; Reynolds, J V

    2013-04-29

    Adrenal metastases of oesophageal adenocarcinoma are rarely detected in the clinical setting, more frequently being found as an incidental postmortem finding in the presence of widespread metastases. With improvements in the sensitivity of radiological diagnostic modalities, the incidence of adrenal tumour detection is on the rise. We report herein a particularly rare case of primary operative management by adrenalectomy for an isolated right-sided adrenal metastasis secondary to oesophageal adenocarcinoma, with a long-term survival.

  15. Rifampicin induced adrenal crisis in an uncommon setting

    Science.gov (United States)

    Ray, Animesh; Suri, J. C.; Gupta, Mansi

    2013-01-01

    Adrenal crisis occurs when there is decreased secretions of steroid hormones (mainly cortisol) from the adrenal glands due to varied reasons. It may arise due to a primary adrenal condition or due to decreased hormonal signals from the pituitary secondary to a hypofunctioning pituitary. Hypopituitarism may result due to direct causes like trauma, tumour, infection or it may be due to some vascular insult as seen in Sheehan syndrome. We report an unusual presentation of Sheehan syndrome in the form of life-threatening adrenal crisis precipitated by the usage of rifampicin. PMID:24339502

  16. Rifampicin induced adrenal crisis in an uncommon setting

    Directory of Open Access Journals (Sweden)

    Animesh Ray

    2013-01-01

    Full Text Available Adrenal crisis occurs when there is decreased secretions of steroid hormones (mainly cortisol from the adrenal glands due to varied reasons. It may arise due to a primary adrenal condition or due to decreased hormonal signals from the pituitary secondary to a hypofunctioning pituitary. Hypopituitarism may result due to direct causes like trauma, tumour, infection or it may be due to some vascular insult as seen in Sheehan syndrome. We report an unusual presentation of Sheehan syndrome in the form of life-threatening adrenal crisis precipitated by the usage of rifampicin.

  17. Ultrasound diagnosis of adrenal gland hemorrhage in newborn; Diagnostyka ultrasonograficzna krwawien do nadnerczy u noworodkow

    Energy Technology Data Exchange (ETDEWEB)

    Bieganowska-Klamut, Z.; Dybiec, E.; Wieczorek, P.; Charanicz-Bartler, H. [Zaklad Radiologii Pediatrycznej, Akademia Medyczna, Lublin (Poland)

    1995-12-31

    4 cases of adrenal gland hemorrhage in the newborns diagnosed by ultrasound examination were described. In 3 cases the hemorrhage was in one adrenal gland and in one the hemorrhage was in both. In 1 newborn control ultrasound and CT examination performed after 7 weeks demonstrated pseudocyst of right adrenal gland. In 2 other cases control ultrasound examination showed normalization of adrenal glands. The authors suggest differential diagnosis between the adrenal hemorrhage and neuroblastoma of adrenal gland. (author) 7 refs, 2 figs

  18. The chronic cerebrospinal venous insufficiency syndrome.

    Science.gov (United States)

    Zamboni, P; Galeotti, R

    2010-12-01

    Chronic cerebrospinal venous insufficiency (CCSVI) is a syndrome characterized by stenosies of the internal jugular and/or azygous veins (IJVs-AZ) with opening of collaterals and insufficient drainage proved by reduced cerebral blood flow and increased mean transit time in cerebral MRI perfusional study. The present review is aimed to give a comprehensive overview of the actual status of the art of the diagnosis and treatment of this condition. As far as the origin of venous narrowing is concerned, phlebographic studies of the IJVs and AZ systems demonstrated that venous stenoses were likely to be truncular venous malformations; mostly, they are intraluminal defects such as malformed valve, septa webs. CCSVI condition has been found to be strongly associated with multiple sclerosis (MS), a disabling neurodegenerative and demyelinating disease considered autoimmune in nature. In several epidemiological observations performed at different latitudes on patients with different genetic backgrounds, the prevalence of CCSVI in MS ranges from 56% to 100%. To the contrary, by using venous MR and/or different Doppler protocols, CCSVI was not detected with the same prevalence. Two pilot studies demonstrated the safety and feasibility in Day Surgery of the endovascular treatment of CCSVI by means of balloon angioplasty (PTA). It determines a significant reduction of postoperative venous pressure. Restenosis rate was found out elevated in the IJVs, but negligible in the AZ. However, PTA seems to positively influence clinical and QoL parameters of the associated MS and warrants further randomized control trials.

  19. Inflammatory manifestations of experimental lymphatic insufficiency.

    Directory of Open Access Journals (Sweden)

    Raymond Tabibiazar

    2006-07-01

    Full Text Available BACKGROUND: Sustained lymph stagnation engenders a pathological response that is complex and not well characterized. Tissue inflammation in lymphedema may reflect either an active or passive consequence of impaired immune traffic. METHODS AND FINDINGS: We studied an experimental model of acute post-surgical lymphedema in the tails of female hairless, immunocompetent SKH-1 mice. We performed in vivo imaging of impaired immune traffic in experimental, murine acquired lymphatic insufficiency. We demonstrated impaired mobilization of immunocompetent cells from the lymphedematous region. These findings correlated with histopathological alterations and large-scale transcriptional profiling results. We found intense inflammatory changes in the dermis and the subdermis. The molecular pattern in the RNA extracted from the whole tissue was dominated by the upregulation of genes related to acute inflammation, immune response, complement activation, wound healing, fibrosis, and oxidative stress response. CONCLUSIONS: We have characterized a mouse model of acute, acquired lymphedema using in vivo functional imaging and histopathological correlation. The model closely simulates the volume response, histopathology, and lymphoscintigraphic characteristics of human acquired lymphedema, and the response is accompanied by an increase in the number and size of microlymphatic structures in the lymphedematous cutaneous tissues. Molecular characterization through clustering of genes with known functions provides insights into processes and signaling pathways that compose the acute tissue response to lymph stagnation. Further study of genes identified through this effort will continue to elucidate the molecular mechanisms and lead to potential therapeutic strategies for lymphatic vascular insufficiency.

  20. Apparent diffusion coefficient of normal adrenal glands

    Directory of Open Access Journals (Sweden)

    Sara Reis Teixeira

    Full Text Available Abstract Objective: To assess the feasibility and reliability of apparent diffusion coefficient (ADC measurements of normal adrenal glands. Materials and methods: This was a retrospective study involving 32 healthy subjects, divided into two groups: prepubertal (PreP, n = 12, aged from 2 months to 12.5 years (4 males; 8 females; and postpubertal (PostP, n = 20, aged from 11.9 to 61 years (5 males; 15 females. Diffusion-weighted magnetic resonance imaging (DW-MRI sequences were acquired at a 1.5 T scanner using b values of 0, 20, 500, and 1000 s/mm2. Two radiologists evaluated the images. ADC values were measured pixel-by-pixel on DW-MRI scans, and automatic co-registration with the ADC map was obtained. Results: Mean ADC values for the right adrenal glands were 1.44 × 10-3 mm2/s for the PreP group and 1.23 × 10-3 mm2/s for the PostP group, whereas they were 1.58 × 10-3 mm2/s and 1.32 × 10-3 mm2/s, respectively, for the left glands. ADC values were higher in the PreP group than in the PostP group (p < 0.05. Agreement between readers was almost perfect (intraclass correlation coefficient, 0.84-0.94; p < 0.05. Conclusion: Our results demonstrate the feasibility and reliability of performing DW-MRI measurements of normal adrenal glands. They could also support the feasibility of ADC measurements of small structures.

  1. Apparent diffusion coefficient of normal adrenal glands*

    Science.gov (United States)

    Teixeira, Sara Reis; Elias, Paula Condé Lamparelli; Leite, Andrea Farias de Melo; de Oliveira, Tatiane Mendes Gonçalves; Muglia, Valdair Francisco; Elias Junior, Jorge

    2016-01-01

    Objective To assess the feasibility and reliability of apparent diffusion coefficient (ADC) measurements of normal adrenal glands. Materials and methods This was a retrospective study involving 32 healthy subjects, divided into two groups: prepubertal (PreP, n = 12), aged from 2 months to 12.5 years (4 males; 8 females); and postpubertal (PostP, n = 20), aged from 11.9 to 61 years (5 males; 15 females). Diffusion-weighted magnetic resonance imaging (DW-MRI) sequences were acquired at a 1.5 T scanner using b values of 0, 20, 500, and 1000 s/mm2. Two radiologists evaluated the images. ADC values were measured pixel-by-pixel on DW-MRI scans, and automatic co-registration with the ADC map was obtained. Results Mean ADC values for the right adrenal glands were 1.44 × 10-3 mm2/s for the PreP group and 1.23 × 10-3 mm2/s for the PostP group, whereas they were 1.58 × 10-3 mm2/s and 1.32 × 10-3 mm2/s, respectively, for the left glands. ADC values were higher in the PreP group than in the PostP group (p < 0.05). Agreement between readers was almost perfect (intraclass correlation coefficient, 0.84-0.94; p < 0.05). Conclusion Our results demonstrate the feasibility and reliability of performing DW-MRI measurements of normal adrenal glands. They could also support the feasibility of ADC measurements of small structures. PMID:28057963

  2. High grade primary adrenal intravascular large B-cell lymphoma manifesting as Addison disease Linfoma intravascular de alto grado de células B grandes y origen suprarrenal que se manifiesta en forma de enfermedad de Addison

    Directory of Open Access Journals (Sweden)

    J. Venizelos

    2007-08-01

    Full Text Available We report a rare case of a 68 aged male who presented with adrenal failure and was diagnosed of high grade large B-cell lymphoma primarily arising in the adrenal glands. The patient was administrated with additional chemotherapy but he passed away 7 months later due to infection in the lungs. Intravascular lymphoma should be suspected in patients with bilateral adrenal masses who present with rapidly progressive adrenal insufficiency.Publicamos el caso poco frecuente de un varón de 68 años de edad que debutó con insuficiencia adrenal y fue diagnosticado de linfoma de alto grado de células B grandes ubicado principalmente en las glándulas suprarrenales. Al paciente le administraron quimioterapia adicional, pero falleció 7 meses después de infección pulmonar. El linfoma intravascular debe sospecharse en los pacientes con masas suprarrenales bilaterales que presenten insuficiencia adrenal rápidamente progresiva.

  3. Renal Cell Carcinoma Mimicking Adrenal Tumor

    Directory of Open Access Journals (Sweden)

    Mohammad Kazem Moslemi

    2010-01-01

    Full Text Available There are a variety of causes of adrenal pseudotumors on computerized tomography (CT scan, including upper-pole renal mass, gastric diverticulum, prominent splenic lobulation, pancreatic mass, hepatic mass, and periadrenal varices. We present a case of a large subhepatic mass that discrimination of its origin from neighborhood organs was difficult preoperatively. Our patient was a 58 years old man, that three months after an unsuccessful operation in another center for a pseudoadrenal mass underwent a very difficult subcapsular tumorectomy in our center.

  4. Extra-adrenal Pheochromocytoma in an Adolescent

    Directory of Open Access Journals (Sweden)

    Abdullah, Ibrahim

    2011-05-01

    Full Text Available A 17-year-old male with symptoms of headache and diaphoresis presented to the emergency department. He had eight months of noted hypertension attributed to medications. On arrival his blood pressure was 229/117mmHg, and he was ill-appearing. His blood pressure was managed aggressively, and he was diagnosed with extra-adrenal pheochromocytoma by computed tomography. He eventually underwent resection of the mass. Children with severe, symptomatic hypertension should be evaluated for pheochromocytoma. Although rare, it is curable. Failure to diagnose carries a high risk of morbidity and mortality. [West J Emerg Med. 2011;12(2:258-261.

  5. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency - management in adults.

    Science.gov (United States)

    Ambroziak, Urszula; Bednarczuk, Tomasz; Ginalska-Malinowska, Maria; Małunowicz, Ewa Maria; Grzechocińska, Barbara; Kamiński, Paweł; Bablok, Leszek; Przedlacki, Jerzy; Bar-Andziak, Ewa

    2010-01-01

    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is one of the most common autosomal recessive hereditary diseases. The impairment of cortisol synthesis leads to excessive stimulation of the adrenal glands by adrenocorticotropic hormone (ACTH), adrenal hyperplasia, and excessive androgen synthesis. The syndrome is characterised by a considerable correlation between the genotype and the phenotype with the type of CYP21A2 gene mutation affecting the severity of 21-hydroxylase deficiency. The clinical manifestations of CAH in adults result from adrenocortical and adrenomedullary insufficiency, hyperandrogenism, and the adverse effects of glucocorticosteroids used for the treatment of the condition. Non-classic CAH may sometimes be asymptomatic. In patients with classic CAH obesity, hyperinsulinaemia, insulin resistance, and hyperleptinaemia are more often seen than in the general population. These abnormalities promote the development of metabolic syndrome and its sequelae, including endothelial dysfunction, and cardiovascular disease. Long-term glucocorticosteroid treatment is also a known risk factor for osteoporosis. Patients with CAH require constant monitoring of biochemical parameters (17a-hydroxyprogesterone [17-OHP] and androstenedione), clinical parameters (body mass, waist circumference, blood pressure, glucose, and lipids), and bone mineral density by densitometry. The principal goal of treatment in adults with CAH is to improve quality of life, ensure that they remain fertile, reduce the manifestations of hyperandrogenisation in females, and minimise the adverse effects of glucocorticosteroid treatment. Patients with classic CAH require treatment with glucocorticosteroids and, in cases of salt wasting, also with a mineralocorticosteroid. Radical measures, such as bilateral adrenalectomy, are very rarely needed. Asymptomatic patients with non-classic CAH require monitoring: treatment is not always necessary. Medical care for patients

  6. [Congenital adrenal hyperplasia due to 21-hydroxylase deficiency--management in adults].

    Science.gov (United States)

    Ambroziak, Urszula; Bednarczuk, Tomasz; Ginalska-Malinowska, Maria; Małunowicz, Ewa Maria; Grzechocińska, Barbara; Kamiński, Paweł; Bablok, Leszek; Przedlacki, Jerzy; Bar-Andziak, Ewa

    2010-01-01

    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is one of the most common autosomal recessive hereditary diseases. The impairment of cortisol synthesis leads to excessive stimulation of the adrenal glands by adrenocorticotropic hormone (ACTH), adrenal hyperplasia, and excessive androgen synthesis. The syndrome is characterised by a considerable correlation between the genotype and the phenotype with the type of CYP21A2 gene mutation affecting the severity of 21-hydroxylase deficiency. The clinical manifestations of CAH in adults result from adrenocortical and adrenomedullary insufficiency, hyperandrogenism, and the adverse effects of glucocorticosteroids used for the treatment of the condition. Non-classic CAH may sometimes be asymptomatic. In patients with classic CAH obesity, hyperinsulinaemia, insulin resistance, and hyperleptinaemia are more often seen than in the general population. These abnormalities promote the development of metabolic syndrome and its sequelae, including endothelial dysfunction, and cardiovascular disease. Long-term glucocorticosteroid treatment is also a known risk factor for osteoporosis. Patients with CAH require constant monitoring of biochemical parameters (17a-hydroxyprogesterone and androstenedione), clinical parameters (body mass, waist circumference, blood pressure, glucose, and lipids), and bone mineral density by densitometry. The principal goal of treatment in adults with CAH is to improve quality of life, ensure that they remain fertile, reduce the manifestations of hyperandrogenisation in females, and minimise the adverse effects of glucocorticosteroid treatment. Patients with classic CAH require treatment with glucocorticosteroids and, in cases of salt wasting, also with a mineralocorticosteroid. Radical measures, such as bilateral adrenalectomy, are very rarely needed. Asymptomatic patients with non-classic CAH require monitoring: treatment is not always necessary. Medical care for patients with CAH

  7. [Lipid peroxidation in the adrenal cortex during exhausting stress].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Naumov, A V; Vinogradov, V V

    1990-05-01

    Under prolonged stress which is connected with exhaustion of functional resources of adrenal cortex the activation of lipid peroxidation processes in this gland was found. It is possible that the reason for such lipid peroxidation activation is the decrease in the content of adrenal cortex ascorbic acid and alpha-tocopherol.

  8. Incidence and Cause of Hypertension During Adrenal Radiofrequency Ablation

    Energy Technology Data Exchange (ETDEWEB)

    Yamakado, Koichiro, E-mail: yama@clin.medic.mie-u.ac.jp; Takaki, Haruyuki [Mie University School of Medicine, Department of Interventional Radiology (Japan); Yamada, Tomomi [Mie University School of Medicine, Department of Translational Medicine (Japan); Yamanaka, Takashi; Uraki, Junji; Kashima, Masataka; Nakatsuka, Atsuhiro; Takeda, Kan [Mie University School of Medicine, Department of Interventional Radiology (Japan)

    2012-12-15

    Purpose: To evaluate the incidence and cause of hypertension prospectively during adrenal radiofrequency ablation (RFA). Methods: For this study, approved by our institutional review board, written informed consent was obtained from all patients. Patients who received RFA for adrenal tumors (adrenal ablation) and other abdominal tumors (nonadrenal ablation) were included in this prospective study. Blood pressure was monitored during RFA. Serum adrenal hormone levels including epinephrine, norepinephrine, dopamine, and cortisol levels were measured before and during RFA. The respective incidences of procedural hypertension (systolic blood pressure >200 mmHg) of the two patient groups were compared. Factors correlating with procedural systolic blood pressure were evaluated by regression analysis.ResultsNine patients underwent adrenal RFA and another 9 patients liver (n = 5) and renal (n = 4) RFA. Asymptomatic procedural hypertension that returned to the baseline by injecting calcium blocker was found in 7 (38.9%) of 18 patients. The incidence of procedural hypertension was significantly higher in the adrenal ablation group (66.7%, 6/9) than in the nonadrenal ablation group (11.1%, 1/9, P < 0.0498). Procedural systolic blood pressure was significantly correlated with serum epinephrine (R{sup 2} = 0.68, P < 0.0001) and norepinephrine (R{sup 2} = 0.72, P < 0.0001) levels during RFA. The other adrenal hormones did not show correlation with procedural systolic blood pressure. Conclusion: Hypertension occurs frequently during adrenal RFA because of the release of catecholamine.

  9. EXPRESSION OF ENDOGENOUS OUABAIN IN MULTIPLE ADRENAL TUMORS

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective To explore expression of endogenous ouabain(EO) in multiple adrenal tumors.Methods Thirty-one cases of adrenal tumors and 6 cases of healthy adrenal tissues were selected. The expression of EO in the adrenal tiss ue was detected with immunohistochemical streptavidin peroxadase conjugated(SP) method.Results Most of EO positive products were localized in cy toplasm of the zona reticularis of human adrenal cortex, and positive products s howed to be fine granular. There was no positive signal in the medulla. EO showe d on diffused positive in patients with pheochromocytoma accompanied high blood pressure[SBP:(165.22±7.61) mmHg, DBP:(105.52±4.26) mmHg], but there were neg ative in ones with normative blood pressure[SBP:(118.52±4.58) mmHg, DBP:(83±3.60) m mHg]. The expression of EO was positive in all adrenocortical hyperplasic, aden oma an d carcinoma, no matter its high or normative blood pressure. The degree of expre ssion of EO in adrenal tissues was related to the level of BP.Conclusion Expression of endogenous ouabain(EO) in health y adrenal tissue and adrenal tumors was a valuable morphological and pathophysio logical clue for the research on ouabain.

  10. Adrenal incidentaloma in thalassemia: a case report and literature review.

    Science.gov (United States)

    Gamberini, Maria Rita; Prandini, Napoleone; Chiodi, Elisabetta; Farneti, Carlotta; Garani, Maria Chiara

    2011-03-01

    In the last 30 years the development and widespread use of modern imaging techniques has caused a 20-time increase in the diagnosis of adrenal incidentaloma (AI). Among AIs myelolipoma (ML) is reported with a frequency up to 10%. In the literature 8 patients with adrenal masses in thalassaemia or chronic haemolytic anaemia have been reported: five cases were shown to have extramedullary haematopoiesis (EH) and 3 ML. We describe here a case of an adult male affected by beta thalassaemia intermedia and large bilateral lipomatous adrenal masses. The patient was referred to our ward at the age of 55 and underwent hormonal testing, MRI, and SPECT/CT scans. Adrenal masses were hormonally inactive, and fat-containing on MRI and CT scans. SPECT/CT examination with 99mTccolloid demonstrated the presence of marrow tissue. ML and EH are the only two tumours with marrow tissue among lipomatous adrenal masses. In our patient a brown nodular mass was resected and histologically classified as ML. In benign adrenal masses, radiological follow-up is indicated; in case of large bilateral masses adrenal function tests are suggested periodically in order to detect possible adrenal hypofunction.

  11. Volume of the adrenal and pituitary glands in depression

    DEFF Research Database (Denmark)

    Kessing, Lars Vedel; Willer, Inge Stoel; Knorr, Ulla

    2011-01-01

    Numerous studies have shown that the hypothalamic-pituitary-adrenal (HPA) axis is hyperactive in some depressed patients. It is unclear whether such hyperactivity results in changed volumes of the adrenal glands, pituitary gland and hypothalamus. We systematically reviewed all controlled studies ...

  12. Synchronous Bilateral Adrenal Metastases from Papillary Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Kaan Gokcen

    2014-12-01

    Full Text Available We report a case of synchronous bilateral adrenal metastasis of renal cell carcinoma. The contralateral metastatic adrenal mass was treated by the laparoscopic transperitoneal approach. The renal mass and its huge ipsilateral metastatic adrenal gland were removed en bloc with open procedure. A 54-year-old man presented to our clinic with left-sid renal cell carcinoma synchronously bilateral adrenal metastases. The primary tumor was localized in the upper-mid pole of the kidney. The diagnosis was established preoperatively by computed tomography. The size of the contralateral adrenal mass was 65 x 45 mm, but the ipsilateral metastatic adrenal mass was huge (140 x 65 mm. After all analysis and other scannings for any metastasis, a contralateral lapararoscopic transperitoneal adrenalectomy and a left open nephroadrenalectomy were performed simultaneously. Synchronous bilateral adrenal metastases from primary renal cell carcinoma without another metastasis is very rare. The optimal surgical procedure should be selected according to the metastatic adrenal masses size and the patient%u2019s status.

  13. Early Hormonal Influences on Cognitive Functioning in Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Resnick, Susan M.; And Others

    1986-01-01

    Reports the results of cognitive test performance and early childhood activities in individuals with congenital adrenal hyperplasia, an autosomal recessive disorder associated with elevated prenatal adrenal androgen levels, demonstrating the effects of early exposure to excess androgenizing hormones on sexually dimorphic cognitive functioning.…

  14. Internal Ca2+ mobilization and secretion in bovine adrenal chromaffin cells

    DEFF Research Database (Denmark)

    Cheek, T R; Thastrup, Ole

    1989-01-01

    )-mobilizing muscarinic agonists to induce secretion reflects the fact that the 50 nM rise in [Ca2+]i they elicit is insufficient to trigger the exocytotic machinery. A recent report, however, has demonstrated that some of the nicotine-induced rise in [Ca2+]i could originate from the InsP3-releasable Ca2......+ store. The role of this Ca2+ store in secretion from bovine adrenal chromaffin cells is therefore unclear. In order to investigate in more detail the role of the InsP3-sensitive Ca2+ store in secretion from these cells, we have used a combination of an InsP3-mobilizing muscarinic agonist...

  15. [Transcranial electrostimulation in chronic cerebral vascular insufficiency].

    Science.gov (United States)

    Voropaev, A A; Mochalov, A D

    2006-01-01

    The method of transcranial electrostimulation (TCES) has been used for treatment of 68 patients with chronic cerebral vascular insufficiency, stages I and II. A treatment course included 7 daily procedures. The influence of TCES was evaluated clinically, by EEG, transcranial ultrasonic Doppler study and hemodynamic indices in arteries and veins as well as by expression of trait and state anxiety. All the parameters were compared to those of the control group which was treated using conventional methods. TCES resulted in normalization of cerebral vascular reactivity, a decrease of venous circulation disturbances, positive influence on cerebral blood flow and EEG parameters, that corresponded to global improvement of the patients' state, regress of cephalgic syndrome and reduction of trait and state anxiety. The method is simple and safety and can be recommended for wide application including outpatient setting.

  16. Surgical technique: Retroperitoneoscopic approach for adrenal masses in children.

    Science.gov (United States)

    Yankovic, F; Undre, S; Mushtaq, I

    2014-04-01

    Laparoscopic adrenalectomy is considered to be the standard of care for the surgical excision of adrenal masses. The transperitoneal laparoscopic and retroperitoneoscopic approaches are described. Both are safe and as effective as open adrenalectomy, with the added benefit of the minimally invasive approach. It can be utilized for patients requiring surgery for a phaeochromocytoma, adrenal adenoma, adrenal adenocarcinoma, Cushing's syndrome, neuroblastoma, and an incidentaloma. Relative contraindications include previous surgery of the liver or kidney, large tumours (>8-10 cm in diameter) or coagulation disorders. Although the transperitoneal route is used more widely, the retroperitoneal approach provides direct access to the adrenal gland and easy visualization of the adrenal vein. It avoids also colonic mobilization, minimizes the risk of injury to hollow viscera, and the potential risk of adhesion formation. However, the reversed orientation of the kidney and hilum, combined with a significantly smaller working space, may make this approach difficult to master.

  17. Adrenal vein sampling in the diagnosis of aldosteronism

    Directory of Open Access Journals (Sweden)

    Deipolyi AR

    2015-06-01

    Full Text Available Amy R Deipolyi,1 Rahmi Oklu2 1Vascular and Interventional Radiology, NYU Langone Medical Center, New York, NY, USA; 2Interventional Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA Abstract: Primary aldosteronism causes 15%–25% of cases of drug-resistant hypertension. Adrenal vein sampling (AVS is a procedure entailing the measurement of aldosterone from both adrenal veins, to diagnose an adrenal source of excess aldosterone secretion. Because unilateral adrenal etiologies of primary aldosteronism may be surgically resected, identifying these sources by venous sampling is critical. Technical aspects of the procedure are reviewed, with emphasis on strategies to avoid common difficulties during AVS. Keywords: primary aldosteronism, hypertension, venous sampling, adrenal adenoma

  18. Synchronous laparoscopic resection of colorectal and renal/adrenal neoplasms.

    Science.gov (United States)

    Ng, Simon S M; Lee, Janet F Y; Yiu, Raymond Y C; Li, Jimmy C M; Leung, Ka Lau

    2007-08-01

    Synchronous laparoscopic resections of coexisting abdominal diseases are shown to be feasible without additional postoperative morbidity. We report our experience with synchronous laparoscopic resection of colorectal carcinoma and renal/adrenal neoplasms with an emphasis on surgical and oncologic outcomes. Five patients diagnosed to have synchronous colorectal carcinoma and renal/adrenal neoplasms (renal cell carcinoma in 2 patients, adrenal cortical adenoma in 2 patients, and adrenal metastasis in 1 patient) underwent synchronous laparoscopic resection. The median operative time was 420 minutes and the median operative blood loss was 1000 mL. Three patients developed minor complications, including wound infection in 2 patients and retention of urine in 1 patient. There was no operative mortality. The median duration of hospital stay was 11 days. At a median follow-up of 17.6 months, no patient developed recurrence of disease. Synchronous laparoscopic resection of colorectal and renal/adrenal neoplasms is technically feasible and safe.

  19. Adrenal G protein-coupled receptor kinase-2 in regulation of sympathetic nervous system activity in heart failure

    Institute of Scientific and Technical Information of China (English)

    Katie; A; Mc; Crink; Ava; Brill; Anastasios; Lymperopoulos

    2015-01-01

    Heart failure(HF), the number one cause of death in the western world, is caused by the insufficient performance of the heart leading to tissue underperfusion in response to an injury or insult. It comprises complex interactions between important neurohormonal mechanisms that try but ultimately fail to sustain cardiac output. The most prominent such mechanism is the sympathetic(adrenergic) nervous system(SNS), whose activity and outflow are greatly elevated in HF. SNS hyperactivity confers significant toxicity to the failing heart and markedly increases HF morbidity and mortality via excessive activation of adrenergic receptors, which are G protein-coupled receptors. Thus, ligand binding induces their coupling to heterotrimeric G proteins that transduce intracellular signals. G protein signaling is turned-off by the agonist-bound receptor phosphorylation courtesy of G protein-coupled receptor kinases(GRKs), followed by βarrestin binding, which prevents the GRK-phosphorylated receptor from further interaction with the G proteins and simultaneously leads it inside the cell(receptor sequestration). Recent evidence indicates that adrenal GRK2 and βarrestins can regulate adrenal catecholamine secretion, thereby modulating SNS activity in HF. The present review gives an account of all these studies on adrenal GRKs and βarrestins in HF and discusses the exciting new therapeutic possibilities for chronic HF offered by targeting these proteins pharmacologically.

  20. Giant adrenal cyst - a case report and classification; Cisto gigante da adrenal - relato de caso e classificacao

    Energy Technology Data Exchange (ETDEWEB)

    Pitaki, Sergio Augusto Munhoz; Louveira, Maria Helena [Clinica Radiologica Pitaki, Curitiba, PR (Brazil). Alphasonic; Rorato, Luciana; Antonik, Gustavo; Andrade, Andre Luis Lugnani de; Teixeira, Ana Maria Gonzaga [Faculdade Evangelica de Medicina do Parana (FEMPAR), Curitiba, PR (Brazil)

    1999-12-01

    Cystic lesions of the adrenal gland are relatively rare, but they must be considering in the differential diagnosis of abdominal masses. The authors report a case of a giant adrenal cyst assessed by ultrasonography, computed tomography and magnetic resonance imaging, and outline their features and classification. (author)

  1. Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Sweep, C.G.J.; Blickman, J.G.; Hermus, A.R.M.M.; Otten, B.J.

    2007-01-01

    OBJECTIVE: Testicular adrenal rest tumours (TART) are a well-known complication in adult male patients with congenital adrenal hyperplasia (CAH), with a reported prevalence of up to 94%. In adulthood, the tumours are associated with gonadal dysfunction most probably due to longstanding obstruction o

  2. Convergence Insufficiency/Divergence Insufficiency Convergence Excess/Divergence Excess: Some Facts and Fictions.

    Science.gov (United States)

    Khawam, Edward; Abiad, Bachir; Boughannam, Alaa; Saade, Joanna; Alameddine, Ramzi

    2015-01-01

    Great discrepancies are often encountered between the distance fixation and the near-fixation esodeviations and exodeviations. They are all attributed to either anomalies of the AC/A ratio or anomalies of the fusional convergence or divergence amplitudes. We report a case with pseudoconvergence insufficiency and another one with pseudoaccommodative convergence excess. In both cases, conv./div. excess and insufficiency were erroneously attributed to anomalies of the AC/A ratio or to anomalies of the fusional amplitudes. Our purpose is to show that numerous factors, other than anomalies in the AC/A ratio or anomalies in the fusional conv. or divergence amplitudes, can contaminate either the distance or the near deviations. This results in significant discrepancies between the distance and the near deviations despite a normal AC/A ratio and normal fusional amplitudes, leading to erroneous diagnoses and inappropriate treatment models.

  3. Convergence Insufficiency/Divergence Insufficiency Convergence Excess/Divergence Excess: Some Facts and Fictions

    Directory of Open Access Journals (Sweden)

    Edward Khawam

    2015-01-01

    Full Text Available Great discrepancies are often encountered between the distance fixation and the near-fixation esodeviations and exodeviations. They are all attributed to either anomalies of the AC/A ratio or anomalies of the fusional convergence or divergence amplitudes. We report a case with pseudoconvergence insufficiency and another one with pseudoaccommodative convergence excess. In both cases, conv./div. excess and insufficiency were erroneously attributed to anomalies of the AC/A ratio or to anomalies of the fusional amplitudes. Our purpose is to show that numerous factors, other than anomalies in the AC/A ratio or anomalies in the fusional conv. or divergence amplitudes, can contaminate either the distance or the near deviations. This results in significant discrepancies between the distance and the near deviations despite a normal AC/A ratio and normal fusional amplitudes, leading to erroneous diagnoses and inappropriate treatment models.

  4. Characterization of novel StAR (steroidogenic acute regulatory protein mutations causing non-classic lipoid adrenal hyperplasia.

    Directory of Open Access Journals (Sweden)

    Christa E Flück

    Full Text Available CONTEXT: Steroidogenic acute regulatory protein (StAR is crucial for transport of cholesterol to mitochondria where biosynthesis of steroids is initiated. Loss of StAR function causes lipoid congenital adrenal hyperplasia (LCAH. OBJECTIVE: StAR gene mutations causing partial loss of function manifest atypical and may be mistaken as familial glucocorticoid deficiency. Only a few mutations have been reported. DESIGN: To report clinical, biochemical, genetic, protein structure and functional data on two novel StAR mutations, and to compare them with published literature. SETTING: Collaboration between the University Children's Hospital Bern, Switzerland, and the CIBERER, Hospital Vall d'Hebron, Autonomous University, Barcelona, Spain. PATIENTS: Two subjects of a non-consanguineous Caucasian family were studied. The 46,XX phenotypic normal female was diagnosed with adrenal insufficiency at the age of 10 months, had normal pubertal development and still has no signs of hypergonodatropic hypogonadism at 32 years of age. Her 46,XY brother was born with normal male external genitalia and was diagnosed with adrenal insufficiency at 14 months. Puberty was normal and no signs of hypergonadotropic hypogonadism are present at 29 years of age. RESULTS: StAR gene analysis revealed two novel compound heterozygote mutations T44HfsX3 and G221S. T44HfsX3 is a loss-of-function StAR mutation. G221S retains partial activity (∼30% and is therefore responsible for a milder, non-classic phenotype. G221S is located in the cholesterol binding pocket and seems to alter binding/release of cholesterol. CONCLUSIONS: StAR mutations located in the cholesterol binding pocket (V187M, R188C, R192C, G221D/S seem to cause non-classic lipoid CAH. Accuracy of genotype-phenotype prediction by in vitro testing may vary with the assays employed.

  5. Adrenal gland infection by serotype 5 adenovirus requires coagulation factors.

    Directory of Open Access Journals (Sweden)

    Lucile Tran

    Full Text Available Recombinant, replication-deficient serotype 5 adenovirus infects the liver upon in vivo, systemic injection in rodents. This infection requires the binding of factor X to the capsid of this adenovirus. Another organ, the adrenal gland is also infected upon systemic administration of Ad, however, whether this infection is dependent on the cocksackie adenovirus receptor (CAR or depends on the binding of factor X to the viral capsid remained to be determined. In the present work, we have used a pharmacological agent (warfarin as well as recombinant adenoviruses lacking the binding site of Factor X to elucidate this mechanism in mice. We demonstrate that, as observed in the liver, adenovirus infection of the adrenal glands in vivo requires Factor X. Considering that the level of transduction of the adrenal glands is well-below that of the liver and that capsid-modified adenoviruses are unlikely to selectively infect the adrenal glands, we have used single-photon emission computed tomography (SPECT imaging of gene expression to determine whether local virus administration (direct injection in the kidney could increase gene transfer to the adrenal glands. We demonstrate that direct injection of the virus in the kidney increases gene transfer in the adrenal gland but liver transduction remains important. These observations strongly suggest that serotype 5 adenovirus uses a similar mechanism to infect liver and adrenal gland and that selective transgene expression in the latter is more likely to be achieved through transcriptional targeting.

  6. Bilateral Adrenal Incidentalomas: A Rare Presentation of Lung Cancer

    Directory of Open Access Journals (Sweden)

    Halit Diri

    2015-01-01

    Full Text Available Adrenal incidentalomas are found incidentally during a radiologic examination performed for indications other than an adrenal disease, and 15% of them are bilateral adrenal masses. This study describes a 51-year-old male smoker patient admitted with diabetes mellitus. An abdominal ultrasonography performed due to his anemia revealed bilateral adrenal masses. His chest X-ray showed abnormal 10 cm opacity at the right upper lung, and brain, thorax, and abdomen CT scans showed multiple lesions compatible with lung cancer metastases. The pathological examination of the transthoracic lung biopsy specimen was consistent with lung adenocarcinoma. Findings in this patient indicate that, in middle aged patients with bilateral adrenal mass and a history or finding of any malignancy, the first diagnosis which should be considered is adrenal metastasis, and confirming the diagnosis by adrenal biopsy may be useless. Furthermore, screening all smoking patients by chest X-ray or thoracic CT for lung cancer may not be accepted as a routine procedure, but in smokers admitted to a hospital due to signs and symptoms attributed to a pulmonary disease, at least a chest X-ray should be requested.

  7. Bilateral Adrenal Metastasis in Bronchogenic Carcinoma: Case Report

    Directory of Open Access Journals (Sweden)

    Nargess Afzali

    2009-01-01

    Full Text Available "nAdrenal metastases from bronchogenic carcinoma are found in approximately one third of patients at autopsy. Tumors that commonly metastasize to the adrenal gland include bronchogenic carcinoma, breast carcinoma and melanoma and small cell lung cancer, accounting for 6% of the adrenal metastasis. Routine preoperative upper abdominal CT scanning reveals an adrenal mass in approximately 10% of the patients. In selected patients, excision of the primary lung tumor and the isolated adrenal metastasis may improve survival. "nHere a 82-year-old female is presented with a history of 3 months pleuritic chest pain, chronic cough, dyspnea and also abdominal distention and discomfort. Bronchoscopy revealed tumoral vegetation in the posterior segment bronchus of the right lung upper lobe. It was diagnosed as an undifferentiated lung carcinoma pathologically. Abdominal CT scan revealed bilateral large heterogeneous masses in the adrenal glands. Biopsy of the adrenal masses revealed their metastatic nature. The patient was expired before excision of the abdominal masses because of lung cancer with pericardial invasion.   

  8. Dexamethasone-suppression adrenal scintigraphy in hyperandrogenism: concise communication

    Energy Technology Data Exchange (ETDEWEB)

    Gross, M.D.; Freitas, J.E.; Swanson, D.P.; Woodbury, M.C.; Schteingart, D.E.; Beierwaltes, W.H.

    1981-01-01

    To assess the contribution of adrenal-derived androgens in women with hirsutism, adrenal scintigrams under dexamethasone suppression (DS) were performed on 35 women with increasing facial or body hair and irregular or absent menses. Based upon the DS regimen chosen (8 mg/d for 2 days or 4 md/d for 7 days before the injection of 6..beta..-(/sup 131/I)iodomethylnorcholesterol), three imaging patterns were identified. The first was the absence of uptake before 3 days (8-mg DS) or before 5 days (4-mg DS) after injection. This imaging pattern was seen in 17 of the 35 patients studied and was considered normal. The second pattern was bilateral uptake earlier than 3 days (8-mg DS regimen) or 5 days (4-mg DS) after injection. This was seen in 13 of the 35 patients and was interpreted as bilateral early visualization. Adrenal-vein catheterization performed on six patients with this pattern showed increased adrenal-vein testosterone. The third pattern, observed in five patients, was unilateral early visualization, which in four cases investigated to date was the result of an adrenocortical adenoma. This study confirms the adrenal cortex as a source of androgens in women with hirsutism and hyperandrogenism and demonstrates that DS adrenal scintigraphy can be utilized to identify those women in whom adrenal-derived androgens contribute to their hyperandrogenism.

  9. Acute adrenal crisis after orthopedic surgery for pathologic fracture

    Directory of Open Access Journals (Sweden)

    Hashimoto Nobuyuki

    2007-03-01

    Full Text Available Abstract Background Adrenal crisis after surgical procedure is a rare but potentially catastrophic life-threatening event. Its manifestations, such as hypotension, tachycardia, hypoxia, and fever mimic the other more common postoperative complications. Clinical outcome is dependent upon early recognition of the condition and proper management with exogenous steroid administration. Case presentation We report a 75-year-old man who presented with shock immediately after surgery for a femoral fracture from lung cancer metastasis. Anemia and severe hyponatremia were detected. Despite adequate fluid resuscitation, nonspecific symptoms including hypotension, tachycardia, hypoxia, fever and confusion occurred. Emergent CT revealed enlarged bilateral adrenal glands. Under the diagnosis of adrenal crisis due to metastatic infiltration of adrenal glands, the patient was treated with appropriate steroid replacement resulting in rapid improvement and recovery. Conclusion We describe a case of adrenal crisis caused by the lack of adrenal reserve based on metastatic involvement and surgical stress, the first published case of adrenal crisis after surgery for a pathologic fracture from lung cancer metastasis. Surgeons treating pathologic fractures should be aware of this complication and familiar with its appropriate therapy because of increasing opportunity to care patients with metastatic bone tumors due to recent advances in cancer treatment.

  10. Premature ovarian insufficiency: Pathogenesis and management

    Directory of Open Access Journals (Sweden)

    Anna J Fenton

    2015-01-01

    Full Text Available The term premature ovarian insufficiency (POI describes a continuum of declining ovarian function in a young woman, resulting in an earlier than average menopause. It is a term that reflects the variable nature of the condition and is substantially less emotive than the formerly used "premature ovarian failure" which signaled a single event in time. Contrary to the decline in the age of menarche seen over the last 3-4 decades there has been no similar change in the age of menopause. In developed nations, the average age for cessation of menstrual cycles is 50-52 years. The age is younger among women from developing nations. Much has been written about POI despite a lack of good data on the incidence of this condition. It is believed that 1% of women under the age of 40 years and 0.1% under the age of 30 years will develop POI. Research is increasingly providing information about the pathogenesis and treatments are being developed to better preserve ovarian function during cancer treatment and to improve fertility options. This narrative review summarizes the current literature to provide an approach to best practice management of POI.

  11. Physical, social and societal functioning of children with congenital adrenal hyperplasia (CAH and their parents, in a Dutch population

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    Sanches Sarita A

    2012-02-01

    Full Text Available Abstract Background Most research concerning congenital adrenal hyperplasia (CAH and related conditions caused by primary adrenal insufficiency, such as Addison's or Cushing's disease, has focused on medical aspects rather than on patients' quality of life. Therefore, our objective was to investigate the physical, social and societal functioning of children with CAH and their parents in a Dutch population. Methods The study is descriptive and cross-sectional. Self-designed questionnaires, based on questionnaires developed in the Netherlands for different patient groups, were sent to parents of children with CAH between 0 and 18 years old. Participants were recruited through the Dutch patient group for Adrenal Disease (NVACP and six hospitals in the Netherlands. Three different questionnaires were designed for parents: for children aged 0 - 4, aged 4 - 12 and aged 12 - 18. Additionally, a fourth questionnaire was sent to adolescents with CAH aged 12 - 18. Main outcome measures were experienced burden of the condition, self-management and participation in several areas, such as school and leisure time. Results A total of 106 parents returned the questionnaire, 12 regarding pre-school children (0-4 years, 63 regarding primary school children (4-12 years, and 32 regarding secondary school children (12-18 years, combined response rate 69.7%. Also, 24 adolescents returned the questionnaire. Children and adolescents with CAH appear to be capable of self-management at a young age. Experienced burden of the condition is low, although children experience several health related problems on a daily basis. Children participate well in school and leisure time. Few children carry a crisis card or emergency injection with them. Conclusions Overall, our research shows that, according to their parents, children with CAH experience few negative effects of the condition and that they participate well in several areas such as school and leisure time. However

  12. Laparoscopic Nephrectomy with Adrenalectomy for Synchronous Adrenal Myelolipoma and Renal Cell Carcinoma

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    Kallappan Senthil

    2015-01-01

    Full Text Available Introduction. Adrenal myelolipomas are uncommon nonfunctioning tumors of the adrenal. Synchronous renal cell carcinomas with adrenal myelolipomas are very rare. We present the case report of adrenal myelolipoma with synchronous RCC managed laparoscopically. Case Report. A 60-year-old old gentleman presented with incidental right upper polar mass with right adrenal mass. Metastatic work-up was negative. Laparoscopic radical nephrectomy with adrenalectomy was done under general anesthesia. The biopsy report was right kidney clear cell adenocarcinoma (T1b with right adrenal myelolipoma. Conclusion. This is the first case report of laparoscopic adrenalectomy with nephrectomy for ipsilateral synchronous renal cell carcinoma with adrenal myelolipoma.

  13. 60 YEARS OF POMC: Adrenal and extra-adrenal functions of ACTH.

    Science.gov (United States)

    Gallo-Payet, Nicole

    2016-05-01

    The pituitary adrenocorticotropic hormone (ACTH) plays a pivotal role in homeostasis and stress response and is thus the major component of the hypothalamo-pituitary-adrenal axis. After a brief summary of ACTH production from proopiomelanocortin (POMC) and on ACTH receptor properties, the first part of the review covers the role of ACTH in steroidogenesis and steroid secretion. We highlight the mechanisms explaining the differential acute vs chronic effects of ACTH on aldosterone and glucocorticoid secretion. The second part summarizes the effects of ACTH on adrenal growth, addressing its role as either a mitogenic or a differentiating factor. We then review the mechanisms involved in steroid secretion, from the classical Cyclic adenosine monophosphate second messenger system to various signaling cascades. We also consider how the interaction between the extracellular matrix and the cytoskeleton may trigger activation of signaling platforms potentially stimulating or repressing the steroidogenic potency of ACTH. Finally, we consider the extra-adrenal actions of ACTH, in particular its role in differentiation in a variety of cell types, in addition to its known lipolytic effects on adipocytes. In each section, we endeavor to correlate basic mechanisms of ACTH function with the pathological consequences of ACTH signaling deficiency and of overproduction of ACTH.

  14. Histologic and Immunohistochemical classification of 41 bovine adrenal gland neoplasms

    DEFF Research Database (Denmark)

    Grossi, Anette Blak; Leifsson, Páll S.; Jensen, Henrik Elvang;

    2013-01-01

    Tumors of the adrenal glands are among the most frequent tumors in cattle; however, few studies have been conducted to describe their characteristics. The aim of this study was to classify 41 bovine adrenal neoplasms from 40 animals based on macroscopic and histologic examination, including....... An immunohistochemistry panel consisting of antibodies against melan A, synaptophysin, and CNPase was considered most useful to classify bovine adrenal tumors. However, the distinction between benign and malignant adrenocortical tumors was based on histologic features as in human medicine....

  15. Right adrenal abscess -- an unusual complication of acute apendicitis.

    Science.gov (United States)

    Dimofte, Gabriel; Dubei, Liviu; Lozneanu, Lili-Gabriela; Ursulescu, Corina; Grigora Scedil, Mihai

    2004-09-01

    Acute appendicitis represents one of the most frequent abdominal emergencies encountered in everyday surgical practice. Local infectious complications are not unusual and retroperitoneal abscesses after acute retrocaecal appendicitis have been previously described. The authors present the case of a 22-years-young female patient, admitted for a right iliac fossa abscess, secondary to gangrenous appendicitis. A right adrenal mass 35/40 mm was revealed during preoperative ultrasound evaluation, which evolved in an adrenal abscess that spontaneously drained 10 days after appendectomy and retrocecal drainage. Adrenal abscesses are exceptionally rare, with only a few cases being reported in the literature, but none of these after acute appendicitis.

  16. [Isolated bilateral adrenal metastasis from renal cancer. Case report].

    Science.gov (United States)

    Rabii, R; Joual, A; Naciri, K; Guessous, H; el Mrini, M; Benjelloun, S

    1999-01-01

    The authors report an uncommon case of bilateral synchronous adrenal gland metastases from left renal cell carcinoma. The diagnosis was established by abdominal ultrasound and computed tomography. The surgical approach initially consisted of left radical nephrectomy and ipsilateral adrenalectomy. Histologically, the tumor of the left adrenal gland was identical to the left renal cell carcinoma. Subsequent contralateral adrenalectomy showed an adrenal metastasis identical to the left renal cell carcinoma. Patient follow-up was good with no recurrence of the disease after one year. This is an uncommon case for renal cancer. The treatment and prognosis are discussed.

  17. [Phenotype B primitive adrenal lymphoma, diagnosed by percutaneous aspiration biopsy].

    Science.gov (United States)

    Dahami, Z; Debbagh, A; Dakir, M; Hafiani, M; Joual, A; Bennani, S; el Mrini, M; Benjelloun, S

    2001-01-01

    The authors report a case of primary adrenal lymphoma in a 30-year old-female who complained of lumbar pain and was in poor general condition. Ultrasonography and CT scan revealed a heterogeneous mass with necrosis in the left adrenal gland. Non-Hodgkin's lymphoma of B-cell origin was determined by ultrasound-guided aspiration biopsy of the adrenal mass. Taking this case and the findings in the literature into consideration, the features of this disease have been reviewed and the problem of diagnosis, treatment and prognosis have been examined.

  18. Congenital adrenal hyperplasia: Treatment and outcomes.

    Science.gov (United States)

    Kamoun, Mahdi; Feki, Mouna Mnif; Sfar, Mohamed Habib; Abid, Mohamed

    2013-10-01

    Congenital adrenal hyperplasia (CAH) describes a group of autosomal recessive disorders where there is impairment of cortisol biosynthesis. CAH due to 21-hydroxylase deficiency accounts for 95% of cases and shows a wide range of clinical severity. Glucocorticoid and mineralocorticoid replacement therapies are the mainstays of treatment of CAH. The optimal treatment for adults with CAH continues to be a challenge. Important long-term health issues for adults with CAH affect both men and women. These issues may either be due to the disease or to steroid treatment and may affect final height, fertility, cardiometabolic risk, bone metabolism, neuro-cognitive development and the quality-of-life. Patients with CAH should be regularly followed-up from childhood to adulthood by multidisciplinary teams who have knowledge of CAH. Optimal replacement therapy, close clinical and laboratory monitoring, early life-style interventions, early and regular fertility assessment and continuous psychological management are needed to improve outcome.

  19. Congenital adrenal hyperplasia: Treatment and outcomes

    Directory of Open Access Journals (Sweden)

    Mahdi Kamoun

    2013-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH describes a group of autosomal recessive disorders where there is impairment of cortisol biosynthesis. CAH due to 21-hydroxylase deficiency accounts for 95% of cases and shows a wide range of clinical severity. Glucocorticoid and mineralocorticoid replacement therapies are the mainstays of treatment of CAH. The optimal treatment for adults with CAH continues to be a challenge. Important long-term health issues for adults with CAH affect both men and women. These issues may either be due to the disease or to steroid treatment and may affect final height, fertility, cardiometabolic risk, bone metabolism, neuro-cognitive development and the quality-of-life. Patients with CAH should be regularly followed-up from childhood to adulthood by multidisciplinary teams who have knowledge of CAH. Optimal replacement therapy, close clinical and laboratory monitoring, early life-style interventions, early and regular fertility assessment and continuous psychological management are needed to improve outcome.

  20. Oxytocin and hypothalamo-pituitary- adrenal axis

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    Berrak Ç. Yeğen

    2010-05-01

    Full Text Available BSTRACT: Upon exposure to different types of stressors, neuroendocrine and behavioral responses that include the activation of the hypothalamus-pituitary-adrenal (HPA axis are given to allow the individuals to cope with stress conditions. It was proven that oxytocin, anonapeptide released from the posterior pituitary, has behavioral and stress-attenuating effects by dampening HPA activity. On the other hand, the neuropeptide was also shown to exert anti-inflammatory effects through the modulation of immune and inflammatory processes in several experimental models of tissue injury. The findings of recent studies suggest that the anti-inflammatory effect of oxytocin depends on its role on HPA axis activity and subsequent release of cortisol. Thus, oxytocin seems to restrain the activity within the HPA-axis, which becomes overactive during many inflammatory processes

  1. Inflammatory mediators in mastitis and lactation insufficiency.

    Science.gov (United States)

    Ingman, Wendy V; Glynn, Danielle J; Hutchinson, Mark R

    2014-07-01

    Mastitis is a common inflammatory disease during lactation that causes reduced milk supply. A growing body of evidence challenges the central role of pathogenic bacteria in mastitis, with disease severity associated with markers of inflammation rather than infection. Inflammation in the mammary gland may be triggered by microbe-associated molecular patterns (MAMPs) as well as danger-associated molecular patterns (DAMPs) binding to pattern recognition receptors such as the toll-like receptors (TLRs) on the surface of mammary epithelial cells and local immune cell populations. Activation of the TLR4 signalling pathway and downstream nuclear factor kappa B (NFkB) is critical to mediating local mammary gland inflammation and systemic immune responses in mouse models of mastitis. However, activation of NFkB also induces epithelial cell apoptosis and reduced milk protein synthesis, suggesting that inflammatory mediators activated during mastitis promote partial involution. Perturbed milk flow, maternal stress and genetic predisposition are significant risk factors for mastitis, and could lead to a heightened TLR4-mediated inflammatory response, resulting in increased susceptibility and severity of mastitis disease in the context of low MAMP abundance. Therefore, heightened host inflammatory signalling may act in concert with pathogenic or commensal bacterial species to cause both the inflammation associated with mastitis and lactation insufficiency. Here, we present an alternate paradigm to the widely held notion that breast inflammation is driven principally by infectious bacterial pathogens, and suggest there may be other therapeutic strategies, apart from the currently utilised antimicrobial agents, that could be employed to prevent and treat mastitis in women.

  2. The controversy on chronic cerebrospinal venous insufficiency

    Directory of Open Access Journals (Sweden)

    Paolo Zamboni

    2013-08-01

    Full Text Available The objective of this review is to analyze the actual scientific controversy on chronic cerebrospinal venous insufficiency (CCSVI and its association with both neurodegenerative disorders and multiple sclerosis (MS. We revised all published studies on prevalence of CCSVI in MS patients, including ultrasound and catheter venography series. Furthermore, we take into consideration other publications dealing with the pathophysiologic consequences of CCSVI in the brain, as well as ecent data characterizing the pathology of the venous wall in course of CCSVI. Finally, safety and pilot data on effectiveness of endovascular CCSVI treatment were further updated. Studies of prevalence show a big variability in prevalence of CCSVI in MS patients assessed by established ultrasonographic criteria. This could be related to high operator dependency of ultrasound. However, 12 studies, by the means of more objective catheter venography, show a prevalence >90% of CCSVI in MS. Global hypo-hypoperfusion of the brain, and reduced cerebral spinal fluid dynamics in MS was shown to be related to CCSVI. Postmortem studies and histology corroborate the 2009 International Union of Phlebology (UIP Consensus decision to insert CCSVI among venous malformations. Finally, safety of balloon angioplasty of the extracranial veins was certainly demonstrated, while prospective data on the potential effectiveness of endovascular treatment of CCSVI support to increase the level of evidence by proceeding with a randomized control trial (RCT. Taking into account the current epidemiological data, including studies on catheter venography, the autoptic findings, and the relationship between CCSVI and both hypo-perfusion and cerebro-spinal fluid flow, we conclude that CCSVI can be definitively inserted among the medical entities. Research is still inconclusive in elucidating the CCSVI role in the pathogenesis of neurological disorders. The controversy between the vascular and the

  3. Testicular adrenal rest tumors in adult males with congenital adrenal hyperplasia: evaluation of pituitary-gonadal function before and after successful testis-sparing surgery in eight patients.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Otten, B.J.; Takahashi, S.; Meuleman, E.J.H.; Hulsbergen- van de Kaa, C.A.; Sweep, C.G.J.; Hermus, A.R.M.M.

    2007-01-01

    CONTEXT: In male patients with congenital adrenal hyperplasia (CAH), testicular adrenal rest tumors (TART) are frequently present. These tumors can interfere with testicular function. Intensifying glucocorticoid therapy does not always lead to tumor regression and improvement of testicular function.

  4. Early respiratory insufficiency in the ALS patient: a case study.

    Science.gov (United States)

    Houseman, Gail; Kelley, Mary

    2005-08-01

    Respiratory insufficiency is a problem that develops in nearly all people diagnosed with amyotrophic lateral sclerosis (ALS). Noninvasive positive pressure ventilation (NIPPV) is the treatment of choice for ALS patients with respiratory insufficiency. Forced vital capacity (FVC) is the test most commonly used to qualify ALS patients for NIPPV; however, some research suggests FVC may not be the best tool to measure early respiratory insufficiency in all patients with ALS. This case study introduces an ALS patient who had normal FVC results, symptoms of respiratory insufficiency, and abnormal nocturnal oximetry. After NIPPV initiation, the patient reported improved sleep and less daytime fatigue, which he associated with the start of NIPPV treatment.

  5. Clinical and genetic analysis of a Korean patient with late-onset X-linked adrenal hypoplasia congenita and hypogonadotropic hypogonadism: identification of a novel mutation in the NR0B1 gene.

    Science.gov (United States)

    Lee, Y-W; Won, J C; Ki, C-S; Lee, H Y; Ahn, H S; Lee, Y K; Kim, Y H; Kim, C-H

    2008-01-01

    Adrenal hypoplasia congenita (AHC) is caused by mutations in the NR0B1 gene on chromosome Xp21.3-p21.2. It manifests as X-linked primary adrenal failure in early infancy or childhood and as hypogonadotropic hypogonadism (HHG) at puberty. Although studies of AHC patients and mutations in the NR0B1 gene have been reported throughout the world, there has previously been only one other case report from Korea. We encountered a 23-year old Korean male with delayed-onset AHC/HHG who had been previously diagnosed with adrenal insufficiency of unknown aetiology at age 13 years. Delayed puberty and incomplete HHG were observed. Direct sequencing of the NR0B1 gene revealed the patient to have a novel insertion mutation (c.959_960insT; Leu321ProfsX68). Although AHC is believed to be rare, it should be considered in a differential diagnosis of patients showing late-onset primary adrenal insufficiency.

  6. Unilateral pulmonary edema during laparoscopic resection of adrenal tumor

    Science.gov (United States)

    Prakash, Smita; Nayar, Pavan; Virmani, Pooja; Bansal, Shipra; Pawar, Mridula

    2015-01-01

    Despite technological, therapeutic and diagnostic advancements, surgical intervention in pheochromocytoma may result in a life-threatening situation. We report a patient who developed unilateral pulmonary edema during laparoscopic resection of adrenal tumor. PMID:26330724

  7. Unilateral pulmonary edema during laparoscopic resection of adrenal tumor

    Directory of Open Access Journals (Sweden)

    Smita Prakash

    2015-01-01

    Full Text Available Despite technological, therapeutic and diagnostic advancements, surgical intervention in pheochromocytoma may result in a life-threatening situation. We report a patient who developed unilateral pulmonary edema during laparoscopic resection of adrenal tumor.

  8. GATA transcription factors in adrenal development and tumors.

    Science.gov (United States)

    Parviainen, Helka; Kiiveri, Sanne; Bielinska, Malgorzata; Rahman, Nafis; Huhtaniemi, Ilpo T; Wilson, David B; Heikinheimo, Markku

    2007-02-01

    Of the six GATA transcription factors, GATA-4 and GATA-6 are expressed in the mouse and human adrenal with distinct developmental profiles. GATA-4 is confined to the fetal cortex, i.e. to the less differentiated proliferating cells, while GATA-6 is expressed both in the fetal and adult adrenal. In vitro, GATA-4 regulates inhibin-alpha and steroidogenic factor-1 implicated in normal adrenal function. GATA-6 probably has roles in the development and differentiation of adrenocortical cells, and in the regulation of steroidogenesis. GATA-4 expression is dramatically upregulated and GATA-6 downregulated in gonadotropin dependent mouse adrenocortical tumors. This is accompanied by the appearance of luteinizing hormone receptor (LHR). In vitro, GATA-4 transactivates LHR promoter, and gonadotropins upregulate GATA-4 levels. Human adrenal tumors occasionally express GATA-4, whereas GATA-6 levels are usually lower than normal.

  9. Development of the adrenal axis in the neonatal rat

    Energy Technology Data Exchange (ETDEWEB)

    Guillet, Ronnie

    1977-01-01

    Plasma corticosterone and ACTH concentrations were determined in neonatal rats 1, 7, 14, and 21 days old, under a variety of experimental conditions, to obtain more information on the postnatal development of the rat hypothalamo-adrenal (HHA) axis. The results indicate that: (1) there is a diminution followed by an increase in responsiveness of the adrenal gland, but the pituitary response to direct hormonal stimulation is unchanged during the first three postnatal weeks; (2) continued stimulation of the adrenal by ACTH or of the central nervous system (CNS) or hypothalamus by corticosterone is necessary during early postnatal development to allow normal maturation of the HHA axis; and (3) feedback inhibition is operative by birth, at least to a moderate degree. Taken together, the studies suggest that both the adrenal and pituitary glands are potentially functional at birth, but that the hypothalamic and CNS mediators of the stress response are not mature until at least the second or third postnatal week. (ERB)

  10. Adrenal function of neonates with respiratory failure%呼吸衰竭新生儿肾上腺皮质功能状态分析

    Institute of Scientific and Technical Information of China (English)

    沈云琳; 黄绮薇; 张宇鸣; 张育才; 田国力

    2009-01-01

    目的 研究呼吸衰竭新生儿(新生儿呼吸窘迫综合征、肺炎和重症湿肺)肾上腺皮质功能变化、肾上腺皮质功能不全(AI)的发生率及其与病情的关系.方法 研究对象为人住我院的呼吸衰竭新生儿55例(其中早产儿33例,足月儿22例),分别检测清晨血清基础皮质醇和促肾上腺皮质激素(ACTH)浓度,及小剂量ACTH刺激试验30 min后血清皮质醇峰值.血清皮质醇峰值浓度<200 μg/L为合并AI.结果 呼吸衰竭早产儿基础皮质醇浓度较足月儿高[(139.2±85.4)μg/L vs(92.1±75.0)μg/L,P=0.040 7],而小剂量ACTH刺激试验前后皮质醇差值及ACTH浓度则较足月儿低[(122.3±56.4)μg/L vs(198.2±77.9)μg/L,P=0.000 1;(5.22±2.40)ng/L vs(8.66±5.41)ng/L.P=0.008 4].呼吸衰竭新生儿合并AJ的发生率为20.0%(11/55),其中早产儿组为21.2%(7/33),足月儿组为18.2%(4/22).需机械通气呼吸衰竭新生儿AI的发生率(29.4%)高于非机械通气新生儿(4.8%).AI新生儿中无死亡病例.结论 呼吸衰竭早产儿肾上腺皮质和垂体功能较足月儿差.呼吸衰竭新生儿合并AJ的发生率较高.需机械通气的呼吸衰竭新生儿AJ的发生率高于机械通气者.未发现AJ与病死率相关.小剂量ACTH刺激试验可较好地评估新生儿肾上腺皮质功能.%Objective To observe the adrenal function in neonates with respiratory failure(neonatal respiratory distress syndrome,pneumonia and severe wet lung),the incidence of adrenal insufficiency,and the relationships between adrenal function and lung diseases.Methods Fifty-five cases of neonates(the preterm group of 33 cases,the term group of 22 cases)were enrolled the study.Serum cortisol values and plasma adrenocorticotropic hormone(ACTH)concentration were detected in the morning.Peak serum cortisol values were detected after 30 minutes low-dose(1 μg/1.73 m2)ACTH stimulation test.Adrenal insufficiency was defined as the peak serum cortisol values < 200 μg/L.Results The mean

  11. Stage 4S Bilateral Adrenal Neuroblastoma in a Newborn

    Directory of Open Access Journals (Sweden)

    Rahul Gupta

    2014-02-01

    Full Text Available Stage 4S bilateral adrenal neuroblastoma presenting in the neonatal period is extremely rare. A 1-day-old male with 4S bilateral adrenal neuroblastoma complicated by marked hepatomegaly managed by chemotherapy is being reported. The provisional diagnosis of neuroblastoma was made in the fetal life during the last trimester of pregnancy. Cardiomyopathy due to doxorubicin cytotoxicity developed over ensuing years, which is being treated.

  12. 肾上腺淋巴瘤临床特点及预后分析%Clinical features and outcomes of adrenal lymphoma

    Institute of Scientific and Technical Information of China (English)

    吴艳; 杨丽娟; 巴建明; 吕朝晖; 母义明; 陆菊明; 潘长玉; 谷伟军; 窦京涛; 杨国庆; 金楠; 杜锦; 郭清华; 欧阳金芝; 王先令

    2013-01-01

    than 1 year. Conclusion The adrenocortical insufficiency may occur in patients with adrenal lymphoma with bilateral adrenal glands involved. Its diagnosis depends on acupuncture or pathological examination. The prognosis of adrenal lymphoma patients is poor. The survival time of such patients is usually less than 1 year after operation.

  13. Imaging of Adrenal Masses with Emphasis on Adrenocortical Tumors

    Directory of Open Access Journals (Sweden)

    Anders Sundin

    2012-01-01

    Full Text Available Because of the more widespread and frequent use of cross-sectional techniques, mainly computed tomography (CT, an increasing number of adrenal tumors are detected as incidental findings (“incidentalomas”. These incidentaloma patients are much more frequent than those undergoing imaging because of symptoms related to adrenal disease. CT and magnetic resonance imaging (MRI are in most patients sufficient for characterization and follow-up of the incidentaloma. In a minor portion of patients, biochemical screening reveals a functional tumor and further diagnostic work-up and therapy need to be performed according to the type of hormonal overproduction. In oncological patients, especially when the morphological imaging criteria indicate an adrenal metastasis, biopsy of the lesion should be considered after pheochromocytoma is ruled out biochemically. In the minority of patients in whom CT and MRI fail to characterize the tumor and when time is of essence, functional imaging mainly by positron emission tomography (PET is available using various tracers. The most used PET tracer, [18F]fluoro-deoxy-glucose (18FDG, is able to differentiate benign from malignant adrenal tumors in many patients. 11C-metomidate (11C-MTO is a more specialized PET tracer that binds to the 11-beta-hydroxylase enzyme in the adrenal cortex and thus makes it possible to differ adrenal tumors (benign adrenocortical adenoma and adrenocortical cancer from those of non-adrenocortical origin.

  14. Neural plasticity occurs in the adrenal medulla of asthmatic rats

    Institute of Scientific and Technical Information of China (English)

    FENG Jun-tao; LI Xiao-zhao; HU Cheng-ping; WANG Jun; NIE Hua-ping

    2010-01-01

    Background Airway symptoms in asthma are related to decrease of epinephrine secretion, which may be ascribed to elevated nerve growth factor (NGF) in the organism.The aim of this study was to monitor the neuroendocrine alteration in the adrenal medulla of asthmatic rats.Methods Sixteen rats were randomly divided into two groups (n=8), control group and asthma group, and the asthmatic rats were sensitized and challenged with ovalbumin (OVA).The levels of NGF, epinephrine and norepinephrine in serum were detected by enzyme linked immunosorbent assay (ELISA), the NGF expression in adrenal medulla was detected by immunohistochemistry, and the changes in the ultrastructure of the adrenal medulla was observed by electron microscopy.Results The NGF expression was increased in asthmatic rats compared with control rats.Compared with control rats,the results indicated that the epinephrine level was decreased in asthmatic rats, but no significant difference was found in norepinephrine levels.We found more ganglion cells in the adrenal medulla of asthmatic rats than in control rats, with NGF immunostaining mainly located in these ganglion cells.Electron microscopic images showed the density of chromaffin granula decreased and there was shrunken nucleolemma in the adrenal medullary cells of asthmatic rats.Conclusion The innervation of the adrenal medulla is changed in asthmatic rats, and it may contribute to the epinephrine decrease in asthma.

  15. Ontogeny of innervation of rat and ovine fetal adrenals.

    Science.gov (United States)

    Engeland, W C; Wotus, C; Rose, J C

    1998-01-01

    The formation of adrenocortical zonation occurs in rats during late gestation. Since adult cortical function is modulated by neural mediators, it is possible that the development of differentiated function is dependent on cortical innervation. The goal of this study was to compare the pattern and timing of rodent and ovine adrenal innervation during late organogenesis by staining with antibodies directed against the neuropeptides vasoactive intestinal peptide (VIP), calcitonin gene-related peptide (CGRP) and neuropeptide tyrosine (NPY) and the catecholamine biosynthetic enzyme, tyrosine hydroxylase (TOH). Rat adrenals were collected from fetal days 17-21 (term=21 days) and ovine adrenals from fetal days 101-136 (term=145 days). Adrenals were fixed, cryosectioned at 100 microns and immunostained using Cy3-conjugated secondary antibodies. In both species, staining of VIP, CGRP, NPY and TOH fibers was observed in the capsule and subcapsular layers of the cortex during gestation. In late gestation, VIP- and NPY-positive ganglions cells were observed near the medulla extending processes toward the outer cortex; in ovine adrenals, fibers from ganglion cells appeared to surround nests of outer cortical (presumably, zona glomerulosa) cells. These data show that phenotypically distinct neural elements appear at different stages of adrenocortical development. The presence of neural elements in contact with adrenal cortical cells supports the possibility for neural control of adrenocortical development.

  16. The Role of gsp Mutations on the Development of Adrenal Cortical Tumors and Adrenal Hyperplasias

    Directory of Open Access Journals (Sweden)

    Maria Candida Barisson Villares Fragoso

    2016-07-01

    Full Text Available Somatic GNAS point mutations, commonly known as gsp mutations, are involved in the pathogenesis of McCune Albright syndrome and have also been described in autonomous hormone-producing tumors, such as somatotropinoma, corticotrophoma, thyroid cancer, ovarian and testicular Leydig cell tumors and primary macronodular adrenocortical hyperplasia (PMAH. [1-3]The involvement of gsp mutations in adrenal tumors was first described by Lyons et al. in 1990. Since then, several studies have detected the presence of gsp mutations in adrenal tumors, but none of them could explain its presence along or the mechanism that leads to tumor formation and hormone hypersecretion. As a result, the molecular pathogenesis of the majority of sporadic adrenocortical tumors remains unclear. [3] PMAH has also been reported with gsp somatic mutations in a few cases. Fragoso et al. in 2003 identified two distinct gsp somatic mutations affecting arginine residues on codon 201 of GNAS in a few patients with PMAH who lacked any features or manifestations of McCune Albright syndrome. Followed by this discovery, other studies have continued looking for gsp mutations based on strong prior evidence demonstrating that increased cAMP signaling is sufficient for cell proliferation and cortisol production. [2, 4] With consideration for the previously reported findings, we conjecture that although somatic activating mutations in GNAS are a rare molecular event, these mutations could probably be sufficient to induce the development of macronodule hyperplasia and variable cortisol secretion.In this manuscript, we revised the presence of gsp mutations associated with adrenal cortical tumors and hyperplasia.

  17. [EFFICIENT TREATMENT OF CHRONIC RESPIRATORY INSUFFICIENCY IN PATIENTS WITH KYPHOSCOLIOSIS].

    Science.gov (United States)

    Tsvetkova, O A; Pal'man, A D; Abdulaeva, G B

    2015-01-01

    We report efficient treatment of chronic respiratory insufficiency in patients with congenital kyphoscoliosis by non-invasive auxiliary ventilation and low-flow oxygen therapy. It proved possible to effectively control severe chronic respiratory insufficiency under conditions of a pulmonological ward without application of means and measures of intensive therapy.

  18. [THERAPY OF TRANSIENT LACTASE INSUFFICIENCY OF CHILDREN IN PECTORAL AGE].

    Science.gov (United States)

    Marushko, Yu V; Iovitsa, T V

    2015-01-01

    Thus, we have discovered that the children of the first half-year of life have different degrees of severity of transient lactase insufficiency basing on the results of hydrogen respiratory test. It was set that the starting dose of enzyme lactase must depend on the degree of severity of displays of transient lactase insufficiency, taking into account the indexes of hydrogen respiratory test.

  19. Tumor necrosis factor alpha affect hydrocortisone expression in mice adrenal cortex cells mainly through tumor necrosis factor alpha-receptor 1

    Institute of Scientific and Technical Information of China (English)

    XIA Hai-ming; FANG Yuan; HUANG Pei-lin

    2011-01-01

    Background Tumor necrosis factor alpha (TNF-α) is important in promoting relative adrenal insufficiency (RAI) due to systemic inflammatory response syndrome (SIRS).We identified the TNF-α receptor involved in the inhibition of adrenal corticotrophin (ACTH)-stimulated hydrocortisone release by studying the expression of TNF-α receptors in adrenal cortex Y1 cells and the effect of downregulating TNF receptors on ACTH-stimulated hydrocortisone release.Methods We used real-time PCR and immunocytochemistry to evaluate the expression of TNF receptors on Y1 cells.TNF-receptor 1 (TNF-R1) DNA fragments corresponding to the short hairpin RNA (shRNA)-sequences were synthesized and cloned into pcDNATM 6.2-GW/EmGFP expression vector.Knockdown efficiency of TNF-R1 expression was evaluated in miRNA transfected and mock-miRNA transfected Y1 cells by quantitative real-time PCR (Q-PCR).Hydrocortisone expression levels were determined in TNF-R1-knockdown and control Y1 cells treated with TNF-α and ACTH.Results Mouse adrenal cortex Y1 cells were positive for type I TNF-R1,but not type Ⅱ TNF-receptor (TNF-R2).Blocking TNF-R1 expression resulted in loss of TNF-α-mediated inhibition of ACTH-stimulated hydrocortisone expression,suggesting a role for the TNF-R1 related signaling pathway in ACTH-stimulated hydrocortisone synthesis.Conclusion The inhibitory effect of TNF-α on ACTH-stimulated hydrocortisone synthesis was mediated via TNF-R1 in adrenal cortex.

  20. [Adrenal tumors as a cause of hypertension].

    Science.gov (United States)

    Grimaldi, Franco

    2010-01-01

    Hypertension is one of the main risk factors for cardiovascular diseases, which today are the primary cause of mortality in developed countries. To decrease the mortality and morbidity due to cardiovascular disease, it is necessary to treat hypertension correctly on the basis of an accurate diagnosis. A secondary cause of hypertension must be suspected if it is severe or resistant to treatment, if there is a sudden increase in blood pressure in a patient hitherto well controlled, or if it develops in childhood or in a person under 30 years of age, especially if not obese and without a family history of hypertension. In these instances the frequency of hypertension varies from 1-5% for mild to moderate to 10-20% for severe or refractory hypertension. One of the principal causes of secondary hypertension is related to adrenal gland tumors or dysfunctions such as Cushing's syndrome, primary aldosteronism (Conn's syndrome) and pheochromocytoma. This paper will discuss the clinical presentation and diagnostic tests pertinent to these neoplasms, organ damage assessment, utilization of imaging techniques, and the medical and surgical options.

  1. High {sup 18}F-fluorodeoxyglocose uptake in adrenal histoplasmosis; a case report

    Energy Technology Data Exchange (ETDEWEB)

    Umeoka, Shigeaki; Saga, Tsuneo; Togashi, Kaori [Kyoto University (Japan). Department of Nuclear Medicine and Diagnostic Imaging; Koyama, Takashi; Higashi, Tatsuya [Kyoto University Hospital (Japan). Department of Radiology; Ito, Noriyuki; Kamoto, Toshiyuki; Ogawa, Osamu [Kyoto University (Japan). Department of Urology; Kotani, Hirokazu [Kyoto University (Japan). Department of Pathology

    2005-12-01

    Adrenal histoplasmosis is one of the most common adrenal granulomatous infections in endemic areas. Although CT or MRI findings of adrenal histoplasmosis have been documented, there are no reports regarding {sup 18}F-fluorodeoxyglocose (FDG) positron emission tomography (PET) findings. We report a case of bilateral adrenal histoplasmosis showing a significantly high uptake of {sup 18}F-fluorodeoxyglocose on PET study. Adrenal histoplasmosis should be considered as one of the differential diagnoses in cases of adrenal tumors with intense FDG uptake, even in non-endemic areas. (orig.)

  2. (18)F-FDG PET/CT in bilateral primary adrenal T-cell lymphoma.

    Science.gov (United States)

    Santhosh, Sampath; Mittal, Bhagwant Rai; Shankar, Praveen; Kashyap, Raghava; Bhattacharya, Anish; Singh, Baljinder; Das, Ashim; Bhansali, Anil

    2011-01-01

    Primary adrenal lymphoma is extremely rare. We report a young patient who presented with non- specific symptoms of fever and abdominal pain. Conventional imaging modalities demonstrated bilateral bulky adrenal masses, and whole-body fluorine-18-fluorodesoxyglucose ((18)F-FDG) positron emission tomography/computed tomography showed intense (18)F-FDG-avid bilateral adrenal masses with no evidence of extra-adrenal spread. A pathological diagnosis of non-Hodgkin lymphoma of peripheral T-cell type was made. The present case indicates that primary adrenal lymphoma should be included in the differential diagnosis of bilateral adrenal masses.

  3. Adrenal lesions encountered in current medical practice − a review of their radiological imaging

    Directory of Open Access Journals (Sweden)

    Vanesha Naidu

    2013-11-01

    Full Text Available Modern radiological technology has transformed the way that adrenal lesions are currently investigated. The contemporary radiologist has been catapulted to the forefront in the management of adrenal disease. With the increasing use of cross-sectional imaging, adrenal lesions are being serendipitously discovered in radiological studies undertaken for non-adrenal-related conditions – the so-called adrenal ‘incidentaloma’. This review discusses the imaging modalities available for characterising these lesions, highlighting current concepts and controversies in differentiating benign from malignant pathology. The article also provides a brief overview of the spectrum of adrenal pathology commonly encountered in the adult population.

  4. Evidence of adrenal failure in aging Dax1-deficient mice.

    Science.gov (United States)

    Scheys, Joshua O; Heaton, Joanne H; Hammer, Gary D

    2011-09-01

    Dosage-sensitive sex reversal, adrenal hypoplasia congenita (AHC) critical region on the X chromosome, gene 1 (Dax1) is an orphan nuclear receptor essential for development and function of the mammalian adrenal cortex and gonads. DAX1 was cloned as the gene responsible for X-linked AHC, which is characterized by adrenocortical failure necessitating glucocorticoid replacement. Contrary to these human data, young mice with genetic Dax1 knockout (Dax1(-/Y)) exhibit adrenocortical hyperfunction, consistent with the historic description of Dax1 as a transcriptional repressor that inhibits steroidogenic factor 1-dependent steroidogenesis. This paradox of molecular function and two apparently opposite phenotypes associated with Dax1 deficiency in mice and humans is compounded by the recent observations that under certain circumstances, Dax1 can serve as a transcriptional activator of steroidogenic factor 1. The recently revealed role of Dax1 in embryonic stem cell pluripotency, together with the observation that its expression in the adult adrenal is restricted to the subcapsular cortex, where presumptive undifferentiated progenitor cells reside, has led us to reexamine the phenotype of Dax1(-/Y) mice in order to reconcile the conflicting mouse and human data. In this report, we demonstrate that although young Dax1(-/Y) mice have enhanced steroidogenesis and subcapsular adrenocortical proliferation, as these mice age, they exhibit declining adrenal growth, decreasing adrenal steroidogenic capacity, and a reversal of their initial enhanced hormonal sensitivity. Together with a marked adrenal dysplasia in aging mice, these data reveal that both Dax1(-/Y) mice and patients with X-linked AHC exhibit adrenal failure that is consistent with adrenocortical subcapsular progenitor cell depletion and argue for a significant role of Dax1 in maintenance of these cells.

  5. Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review

    Directory of Open Access Journals (Sweden)

    Giovanni Scala Marchini

    Full Text Available CONTEXT: Synthesis of cortisol and aldosterone is impaired in patients with congenital adrenal hyperplasia (CAH because of 21-hydroxylase deficiency. Men with CAH have low fertility rates compared with the normal population, and this is related to testicular adrenal rest tumors. Findings of azoospermia in combination with a testicular tumor on ultrasound are likely to have a mechanical cause, especially when in the testicular mediastinum. The preferred treatment method consists of intensive corticoid therapy. However, when the tumor is unresponsive to steroid therapy, surgical treatment should be considered. CASE REPORT: We present the case of a male patient with CAH due to 21-hydroxylase deficiency who presented a testicular tumor and azoospermia. Treatment with low daily corticoid doses had previously been started by an endocrinologist, but after 12 months, no significant change in sperm count was found. Although the adrenocorticotrophic hormone and 17-hydroxyprogesterone levels returned to normal values, the follicle-stimulating hormone (FSH, luteinizing hormone and testosterone levels remained unchanged. Ultrasound examination confirmed that the testicles were small and heterogenous bilaterally, and revealed a mosaic area at the projection of the testis network bilaterally. Magnetic resonance imaging confirmed the finding. Testicular biopsy revealed the presence of preserved spermatogenesis and spermiogenesis in 20% of the seminiferous tubules in the right testicle. The patient underwent testis-sparing tumor resection. After 12 months of follow-up, there was no tumor recurrence but the patient still presented azoospermia and joined an intracytoplasmic sperm injection program.

  6. Localization of aldosterone-producing adenoma on computed tomography. A comparative study with adrenal scintigraphy and plasma aldosterone concentration in the adrenal or renal vein

    Energy Technology Data Exchange (ETDEWEB)

    Haruyama, K.; Shigetomi, S.; Yamazaki, M.; Toki, T.; Yaginuma, K.; Fukuchi, S. (Fukushima Medical Coll. (Japan))

    1982-09-01

    An abdominal CT scan was performed on six patients with primary aldosteronism, one with idiopathic hyperaldosteronism and one with glucocorticoid responsive hyperaldosteronism; in an attempt to evaluate the utility of this noninvasive procedure. Diagnosis of hyperaldosteronism was made by demonstrating the elevated plasma aldosterone concentration and aldosterone secretion rate, normal excretion rate of urinary 17-OHCS and 17-KS, and low plasma renin activity. The CT scan correctly predicted unilateral adrenal adenoma in all the patients with primary aldosteronism of which the findings were identical to those demonstrated by surgery. The diameter of these tumors ranged from 10 x 7 x 6 to 19 x 17 x 14 mm. Also the CT scan in idiopathic hyperaldosteronism and glucocorticoid responsive hyperaldosteronism showed bilateral adrenal hyperplasia and bilateral normal adrenal glands, respectively. The pathological findings in these two cases disclosed the adrenal hyperplasia of zona glomerulosa and adrenal hyperplasia of zona subglomerulosa accompanied by a normal thickness of the adrenal gland, respectively. The precision of the CT scan, adrenal scintigraphy and determination of plasma aldosterone in the adrenal or renal veins were almost equal to the diagnosis of the localization of adrenal adenoma. It is concluded that the CT scan is a noninvasive and most useful method for the localization of aldosterone-producing adenoma and helpful in distinguishing adrenal adenoma from adrenal hyperplasia.

  7. Recommendations for treatment of nonclassic congenital adrenal hyperplasia (NCCAH): an update.

    Science.gov (United States)

    Trapp, Christine M; Oberfield, Sharon E

    2012-03-10

    Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders. 21-Hydroxylase deficiency, in which there are mutations in CYP21A2 (the gene encoding the adrenal 21-hydroxylase enzyme), is the most common form (90%) of CAH. In classic CAH there is impaired cortisol production with diagnostic increased levels of 17-OH progesterone. Excess androgen production results in virilization and in the newborn female may cause development of ambiguous external genitalia. Three-fourths of patients with classic CAH also have aldosterone insufficiency, which can result in salt-wasting; in infancy this manifests as shock, hyponatremia and hyperkalemia. CAH has a reported incidence of 1:10,000-1:20,000 births although there is an increased prevalence in certain ethnic groups. Nonclassic CAH (NCCAH) is a less severe form of the disorder, in which there is 20-50% of 21-hydroxylase enzyme activity (vs. 0-5% in classic CAH) and no salt wasting. The degree of symptoms related to androgen excess is variable and may be progressive with age, although some individuals are asymptomatic. NCCAH has an incidence of 1:1000-1:2000 births (0.1-0.2% prevalence) in the White population; an even higher prevalence is noted in certain ethnic groups such as Ashkenazi Jews (1-2%). As many as two-thirds of persons with NCCAH are compound heterozygotes and carry a severe and mild mutation on different alleles. This paper discusses the genetics of NCCAH, along with its variable phenotypic expression, and reviews the clinical course in untreated patients, which includes rapid early childhood growth, advanced skeletal age, premature adrenarche, acne, impaired reproductive function in both sexes and hirsutism as well as menstrual disorders in females. Finally, it addresses treatment with glucocorticoids vs. non treatment and other therapies, particularly with respect to long term issues such as adult metabolic disease including insulin resistance, cardiovascular disease, metabolic syndrome

  8. Pancreatic exocrine insufficiency in critically ill adult patients.

    Science.gov (United States)

    Ma, Lijie; Liu, Yuhao; Lu, Zhifeng; Zhao, Li; Wang, Sheng

    2016-03-01

    Pancreatic exocrine insufficiency is usually present in patients with pancreatic diseases. Surprisingly, recent studies indicated that patients with critical illness often suffer from pancreatic injury due to non-specific reasons other than pancreatic diseases, and pancreatic exocrine insufficiency is also commonly observed in critically ill adult patients without preexisting pancreatic diseases. It is well known that malnutrition is the main clinical consequence of pancreatic exocrine insufficiency, thus, the high incidence of pancreatic exocrine insufficiency is most likely to be an important contributor of malnutrition which is a frequent problem associated with detrimental clinical outcomes in critically ill patients admitted into intensive care unit. In order to prevent pancreatic exocrine insufficiency due to primary pancreatic diseases, pancreatic enzyme replacement therapy is indispensable to treat indigestion, malabsorption and nutritional deficiency. Similarly, pancreatic enzyme supplementation has the potential to be an adjuvant therapy in critically ill patients with enteral nutrition therapy, which may be helpful to improve the nutritional status and the prognosis of critically ill patients by reducing the occurrence of malnutrition. Here, we reviewed the diagnostic methods of pancreatic exocrine function, the epidemiology and risk factors of pancreatic exocrine insufficiency, and potential treatment strategies for pancreatic exocrine insufficiency in critically ill adult patients.

  9. Magnetic resonance imaging in osteomalacic insufficiency fractures of the pelvis

    Energy Technology Data Exchange (ETDEWEB)

    Kanberoglu, K. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey)]. E-mail: zkanber@istanbul.edu.tr; Kantarci, F. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Cebi, D. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Yilmaz, M.H. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Kurugoglu, S. [Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Bilici, A. [Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey); Koyuncu, H. [Department of Physical Medicine and Rehabilitation, Cerrahpasa Medical Faculty, Istanbul University, Istanbul (Turkey)

    2005-01-01

    AIMS: To report the magnetic resonance imaging (MRI) findings of osteomalacic insufficiency fractures of the pelvis. MATERIALS AND METHODS: In all, 12 persons presenting with chronic pelvic pain and with a definitive diagnosis of osteomalacia (OM) were enrolled in this study. Radiological work-up included direct radiographs (n=12), computed tomography (n=5), scintigraphy n=12) and MRI (n=12). The location of the insufficiency fractures and corresponding MRI appearances were evaluated. Depending on the presence or absence of signal intensity around the fractures, the lesions were grouped into active and chronic forms. RESULTS: A total of 34 insufficiency fractures were depicted on imaging studies. MRI demonstrated 33 of the lesions. All the insufficiency fractures were seen as a hypointense lines or fissures on T1- and T2-weighted and STIR MR images. There was a high frequency of insufficiency fractures at the sacrum and iliac bone (16/34, 47%). Overall, 11 chronic-type (no abnormal signal intensity around the fissure) and 22 active-type (abnormal signal intensity around the fissure) insufficiency fractures were detected by MRI. Follow-up MR examinations of 2 subjects showed that abnormal signal intensity had disappeared after appropriate treatment. CONCLUSION: The results of this study showed that the iliac and sacral bones are frequently involved in patients with OM. MRI can determine the clinical activity of the disease, and can monitor the response to treatment of the active type of insufficiency fractures.

  10. Autoimmune polyglandular syndrome type 2 manifested as Hashimoto's thyroiditis and adrenocortical insufficiency, in Turner syndrome woman, with onset following introduction of treatment with recombinant human growth hormone.

    Science.gov (United States)

    Cyniak-Magierska, Anna; Lasoń, Agnieszka; Smyczyńska, Joanna; Lewiński, Andrzej

    2015-01-01

    Autoimmune polyglandular syndrome is a constellation of signs and symptoms of simultaneous insufficiencies of several endocrine glands. Autoimmune polyglandular syndrome type 2 (APS 2) may be diagnosed when the adrenocortical insufficiency is associated with an autoimmune thyroid disease (Hashimoto's thyroiditis or Graves' disease), and/or insulin-dependent diabetes mellitus. Turner syndrome is the most common chromosomal disorder in females, caused by complete or partial X chromosome monosomy. We present the case of a 20-year-old woman with Turner syndrome, in whom APS 2 (Hashimoto's thyroiditis and adrenocortical insufficiency) has been diagnosed after introduction of recombinant human growth hormone (rhGH) therapy. In Turner syndrome, examination of the patient must regularly be conducted in order to diagnose a possible onset of autoimmune diseases; respective treatment must be applied as soon as the diagnosis is established. In particular, therapy of rhGH, used for short stature treatment, may be a trigger factor of adrenal insufficiency. The cortisol level in blood should be assessed before rhGH administration and carefully monitored during the therapy, especially in case of autoimmune thyroid disease coexistence.

  11. Development of automated detection of radiology reports citing adrenal findings

    Science.gov (United States)

    Zopf, Jason; Langer, Jessica; Boonn, William; Kim, Woojin; Zafar, Hanna

    2011-03-01

    Indeterminate incidental findings pose a challenge to both the radiologist and the ordering physician as their imaging appearance is potentially harmful but their clinical significance and optimal management is unknown. We seek to determine if it is possible to automate detection of adrenal nodules, an indeterminate incidental finding, on imaging examinations at our institution. Using PRESTO (Pathology-Radiology Enterprise Search tool), a newly developed search engine at our institution that mines dictated radiology reports, we searched for phrases used by attendings to describe incidental adrenal findings. Using these phrases as a guide, we designed a query that can be used with the PRESTO index. The results were refined using a modified version of NegEx to eliminate query terms that have been negated within the report text. In order to validate these findings we used an online random date generator to select two random weeks. We queried our RIS database for all reports created on those dates and manually reviewed each report to check for adrenal incidental findings. This survey produced a ground- truth dataset of reports citing adrenal incidental findings against which to compare query performance. We further reviewed the false positives and negatives identified by our validation study, in an attempt to improve the performance query. This algorithm is an important step towards automating the detection of incidental adrenal nodules on cross sectional imaging at our institution. Subsequently, this query can be combined with electronic medical record data searches to determine the clinical significance of these findings through resultant follow-up.

  12. Aldo-Keto Reductases 1B in Adrenal Cortex Physiology.

    Science.gov (United States)

    Pastel, Emilie; Pointud, Jean-Christophe; Martinez, Antoine; Lefrançois-Martinez, A Marie

    2016-01-01

    Aldose reductase (AKR1B) proteins are monomeric enzymes, belonging to the aldo-keto reductase (AKR) superfamily. They perform oxidoreduction of carbonyl groups from a wide variety of substrates, such as aliphatic and aromatic aldehydes or ketones. Due to the involvement of human aldose reductases in pathologies, such as diabetic complications and cancer, AKR1B subgroup enzymatic properties have been extensively characterized. However, the issue of AKR1B function in non-pathologic conditions remains poorly resolved. Adrenal activities generated large amount of harmful aldehydes from lipid peroxidation and steroidogenesis, including 4-hydroxynonenal (4-HNE) and isocaproaldehyde (4-methylpentanal), which can both be reduced by AKR1B proteins. More recently, some AKR1B isoforms have been shown to be endowed with prostaglandin F synthase (PGFS) activity, suggesting that, in addition to possible scavenger function, they could instigate paracrine signals. Interestingly, the adrenal gland is one of the major sites for human and murine AKR1B expression, suggesting that their detoxifying/signaling activity could be specifically required for the correct handling of adrenal function. Moreover, chronic effects of ACTH result in a coordinated regulation of genes encoding the steroidogenic enzymes and some AKR1B isoforms. This review presents the molecular mechanisms accounting for the adrenal-specific expression of some AKR1B genes. Using data from recent mouse genetic models, we will try to connect their enzymatic properties and regulation with adrenal functions.

  13. Clinical value of isotope methods in adrenal morphology investigations

    Energy Technology Data Exchange (ETDEWEB)

    Jakubowski, W.; Feltynowski, T.; Graban, W. (Akademia Medyczna, Warsaw (Poland))

    1981-01-01

    In the years 1976-1980 in 105 patients 124 adrenal scintigrams were obtained, including 50 in patients with primary hyperaldosteronism syndrome, 27 with Cushings syndrome, 8 after bilateral total adrenalectomy, 4 with adrenal virilizing tumours, 6 with phaeochromocytoma, 10 with normal adrenal function. The results of scintigraphy were compared with those of arteriography, phlebography, computer tomography and postoperative findings. The clinical value of scintigraphy was found to be greatest in cases of adrenocortical disease. In cases of phaeochromocytoma isotope angioscintigraphy is a useful method for localizing the tumour since it makes possible demonstration of the vascular bed of the tumour. A close correlation was demonstrated between the results of scintigraphy and those of computer tomography. In the group of 27 patients treated surgically for adrenocortical disease in 87% of cases an agreement was found between the results of scintigraphy and the result of the operation. For different adrenocortical diseases this per cent was: 77% for Cushings syndrome, 91% for primary hyperaldosteronism, 100% for adrenal virilizing tumours. The study showed that adrenal scintigraphy is a very valuable diagnostic method of high clinical usefulness in adrenocortical diseases.

  14. The prevalence of adrenal incidentaloma in routine clinical practice.

    LENUS (Irish Health Repository)

    Davenport, Colin

    2011-03-10

    The prevalence of adrenal incidentaloma (AI) on computed tomography (CT) in the general population has been reported to be as high as 4.2%. However, many of the previous studies in this field utilised a prospective approach with analysis of CT scans performed by one or more radiologists with a specialist interest in adrenal tumours and a specific focus on identifying the presence of an adrenal mass. A typical radiology department, with a focus on the patient\\'s presenting complaint as opposed to the adrenal gland, may not be expected to diagnose as many adrenal incidentalomas as would be identified in a dedicated research protocol. We hypothesised that the number of AI reported in routine clinical practice is significantly lower than the published figures would suggest. We retrospectively reviewed the reports of all CT thorax and abdomen scans performed in our hospital over a 2 year period. 3,099 patients underwent imaging, with 3,705 scans performed. The median age was 63 years (range 18-98). Thirty-seven true AI were diagnosed during the time period studied. Twenty-two were diagnosed by CT abdomen (22\\/2,227) and 12 by CT thorax (12\\/1,478), a prevalence of 0.98 and 0.81% with CT abdomen and thorax, respectively, for AI in routine clinical practice.

  15. The prevalence of adrenal incidentaloma in routine clinical practice.

    LENUS (Irish Health Repository)

    Davenport, Colin

    2012-02-01

    The prevalence of adrenal incidentaloma (AI) on computed tomography (CT) in the general population has been reported to be as high as 4.2%. However, many of the previous studies in this field utilised a prospective approach with analysis of CT scans performed by one or more radiologists with a specialist interest in adrenal tumours and a specific focus on identifying the presence of an adrenal mass. A typical radiology department, with a focus on the patient\\'s presenting complaint as opposed to the adrenal gland, may not be expected to diagnose as many adrenal incidentalomas as would be identified in a dedicated research protocol. We hypothesised that the number of AI reported in routine clinical practice is significantly lower than the published figures would suggest. We retrospectively reviewed the reports of all CT thorax and abdomen scans performed in our hospital over a 2 year period. 3,099 patients underwent imaging, with 3,705 scans performed. The median age was 63 years (range 18-98). Thirty-seven true AI were diagnosed during the time period studied. Twenty-two were diagnosed by CT abdomen (22\\/2,227) and 12 by CT thorax (12\\/1,478), a prevalence of 0.98 and 0.81% with CT abdomen and thorax, respectively, for AI in routine clinical practice.

  16. Double-hit primary unilateral adrenal lymphoma with good outcome

    Directory of Open Access Journals (Sweden)

    Marković Olivera

    2014-01-01

    Full Text Available Introduction. Primary adrenal non-Hodgkin’s lymphoma (NHL is a rare neoplasm with poor prognosis. On the other side, double-hit lymphomas with BCL2 and MYC translocation are characterized by advanced disease stage, extranodal and central nervous system involvements at presentation or disease progression. Case report. We reported a 73-year-old male patient with double-hit primary adrenal lymphoma and preserved adrenal function, showing a favorable clinical course. Computed tomography of abdomen showed a 9 7 cm mass of the left adrenal gland. Laparatomy with left adrenalectomy was done and histological examination revealed diagnosis of a diffuse large B-cell NHL (DLBCL, non-GCB subtype. The patient was treated with 6 cycles of R-CHOP chemotherapy with reduced doses of doxorubicin because of the decreased left verticle ejection fraction. The patient was followed up regularly for 20 months with no evidence of tumor recurrence despite the inherently poor prognostic profile and double-hit phenotype of the disease. Conclusion. R-CHOP chemotherapy in combination with adrenalectomy can be an effective first-line regimen for primary adrenal DLBCL, despite the inherently poor prognostic profile (non-GCB subtype, bulky disease, elevated lactate dehydrogenase and double-hit phenotype of the disease.

  17. Role of the adrenal medulla in control of blood pressure and renal function during furosemide-induced volume depletion

    DEFF Research Database (Denmark)

    Hasbak, Philip; Petersen, Jørgen Søberg; Shalmi, Michael

    1995-01-01

    Farmakologi, furosemide, adrenaline, renal function, adrenal medullectomy, arterial blood pressure......Farmakologi, furosemide, adrenaline, renal function, adrenal medullectomy, arterial blood pressure...

  18. A clinical update on female androgen insufficiency--testosterone testing and treatment in women presenting with low sexual desire.

    Science.gov (United States)

    Burger, Henry G; Papalia, Mary-Anne

    2006-05-01

    The diagnosis of female androgen deficiency syndrome is suggested by complaints of a diminished sense of well being, persistent unexplained fatigue and decreased sexual desire, sexual receptivity and pleasure in a woman who is oestrogen-replete and in whom no other significant contributing factors can be identified. The diagnosis is supported by the finding of low circulating concentrations of free testosterone. Barriers to its recognition include the non-specificity of the symptoms and methodological problems due to insensitive testosterone assays. Barriers to its treatment include the unavailability of satisfactory forms of testosterone for administration to women and lack of data regarding long-term safety. Although several conditions lead to clear-cut androgen deficiency, such as hypopituitarism, adrenal and ovarian insufficiency, glucocorticoid therapy and use of oral contraceptives and oral oestrogens, it is important for clinicians to recognise that in normal women, androgen levels decline by 50% from the early 20s to the mid 40s, and hence age-related androgen insufficiency may occur in women in their late 30s and 40s, as well as postmenopausally. Satisfactory measurements of free testosterone requires a sensitive and reliable assay for total testosterone, and quantitation of sex hormone binding globulin, from which free testosterone is readily calculated. Adverse effects of testosterone treatment are few if replacement is monitored to achieve physiological circulating testosterone concentrations. Currently, available methods include testosterone implants and testosterone creams, and transdermal patches and sprays are in development.

  19. Genetics Home Reference: intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and ...

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    ... IMAGe syndrome intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies Enable Javascript to view ... combination of intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies is commonly known by ...

  20. Genetics Home Reference: congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency

    Science.gov (United States)

    ... due to 11-beta-hydroxylase deficiency congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency Enable Javascript ... PDF Open All Close All Description Congenital adrenal hyperplasia (CAH) due to 11-beta-hydroxylase deficiency is ...

  1. Muscle strength in girls with congenital adrenal hyperplasia.

    Science.gov (United States)

    Rodda, C; Jones, D A; Round, J; Grant, D B

    1987-05-01

    To assess whether exposure to excessive adrenal androgens increases muscle strength in girls with congenital adrenal hyperplasia, eighteen girls with 21 hydroxylase type congenital adrenal hyperplasia (CAH), aged 4.3-12.1 years were studied and compared with 78 healthy control girls aged 4-16 years. Maximum voluntary isometric strength was measured using a muscle testing chair, and the highest value from at least three attempts with each leg was recorded. Height and weight were also measured in the patients and controls. When compared with normal girls of similar age, some of the girls with CAH seemed unusually strong, but this difference was less marked when the CAH girls were compared with controls of similar weights. However, our data suggest that girls diagnosed later in childhood may have unusual muscle strength.

  2. Feminizing adrenal tumors: Our experience about three cases

    Directory of Open Access Journals (Sweden)

    Chentli Farida

    2013-01-01

    Full Text Available Feminizing adrenal tumors (FATs are very rare as they account for less than 2% of all the adrenal neoplasms. Their prognosis is deemed to be very poor. We aimed to present a mono centre (adult and pediatric experience over a long period of time (January 1980 to Jun 2012. During the study period, we observed only three cases in men aged 22 (2 cases and 45 (1 case. They all consulted for a painful gynecomastia, decreased libido and impotency. Estradiol was high in two cases at presentation, and after a relapsing tumor in the third one. All had big adrenal tumors (5.9, 6, and 17 cm, and a mixed secretion composed by high estradiol and cortisol. The pathological study argued for malignancy in two cases. But, only one had diffuse metastasis and died 4 years after diagnosis; the others diagnosed one and three years ago are still alive without any metastasis or relapsing.

  3. Pure Androgen-Secreting Adrenal Adenoma Associated with Resistant Hypertension

    Directory of Open Access Journals (Sweden)

    René Rodríguez-Gutiérrez

    2013-01-01

    Full Text Available Pure androgen-secreting adrenal adenoma is very rare, and its diagnosis remains a clinical challenge. Its association with resistant hypertension is uncommon and not well understood. We present an 18-year-old female with a 10-year history of hirsutism that was accidentally diagnosed with an adrenal mass during the evaluation of a hypertensive crisis. She had a long-standing history of hirsutism, clitorimegaly, deepening of the voice, and primary amenorrhea. She was phenotypically and socially a male. FSH, LH, prolactin, estradiol, 17-hydroxyprogesterone, and progesterone were normal. Total testosterone and DHEA-S were elevated. Cushing syndrome, primary aldosteronism, pheochromocytoma, and nonclassic congenital adrenal hyperplasia were ruled out. She underwent adrenalectomy and pathology reported an adenoma. At 2-month followup, hirsutism and virilizing symptoms clearly improved and blood pressure normalized without antihypertensive medications, current literature of this unusual illness and it association with hypertension is presented and discussed.

  4. Ganglioneuroma of adrenal gland in a patient with Turner syndrome.

    Science.gov (United States)

    Kamoun, Mahdi; Mnif, Mouna Feki; Rekik, Nabila; Belguith, Neila; Charfi, Nadia; Mnif, Lilia; Elleuch, Mouna; Mnif, Fatma; Kamoun, Thouraya; Mnif, Zeinab; Kamoun, Hassen; Sellami-Boudawara, Tahia; Hachicha, Mongia; Abid, Mohamed

    2010-04-01

    A 15-year-old girl with Turner syndrome was unexpectedly found to have a left suprarenal mass. Extensive investigations showed a clinically and biochemically inapparent mass. Computed tomography disclosed a well-defined solid lesion in the left adrenal measuring 6.5 x 5 cm with minimal contrast enhancement. Laparoscopic adrenalectomy was done. Histologic examination revealed an encapsulated mass originated from the left adrenal medulla. Tumor tissue comprised abundant collagen fibers and spindloid cells admixed with mature ganglion cells. The tumor was diagnosed as left adrenal ganglioneuroma. According to literature, we report the eighth case of ganglioneuroma complicating Turner syndrome. Patients with this syndrome are predisposed to the development of neuroblastoma and related tumors. Reasons for this predisposition might relate to genetic and hormonal factors. Given that these tumors are often limited stage and of good prognosis, we recommend their screening in all patients with Turner syndrome.

  5. Subchondral insufficiency fracture of the femoral head in younger adults

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, Takuaki; Nakashima, Yasuharu; Shuto, Toshihide; Jingushi, Seiya; Iwamoto, Yukihide [Graduate School of Medical Sciences, Kyushu University, Department of Orthopaedic Surgery, Fukuoka (Japan)

    2007-06-15

    We report two cases of subchondral insufficiency fracture of the femoral head observed in younger adults without any history of overexertion. In both cases, MRI revealed an irregular, discontinuous low-intensity band on the T1-weighted images. Both patients were treated operatively, and histological examination confirmed the diagnosis of subchondral fracture. A diagnosis of subchondral insufficiency fracture needs to be put in as one of the diagnoses in younger patients with a hip pain. (orig.)

  6. [Study of left ventricular function in valvular cardiopathies (mitral insufficiency and aortic insufficiency].

    Science.gov (United States)

    Herreman, F; Brun, P; Cannet, G; Savin, E; Vannier, D

    1974-10-01

    A study of the left ventricular function based on the haemodynamic data combined with those provided by biplane cineangiography was performed in 35 cases with left ventricular volume overload (20 cases of mitral incompetence and 15 of aortic insufficiency). The importance of the haemodynamic changes and of the adaptation mechanisms set up were described. The more intense dilatation-hypertrophy of aortic incompetence than of mitral incompetence plays an essential part. The role of Starling's mechanism is underlined. Estimation of the contractile value of the myocardium, taken into account the mechanical overload and the conditions of late-diastolic lengthening of the fibre and of impedance to left ventricular ejection was determined. An obvious myocardial failure, demonstrated in approximately one third of the cases, by determination of some contractility indices estimated in the ejection phase, Vf sigma max in particular, the only one valid in the presence of valvular regurgitation. In the other cases, the moderate decrease of myocardial contractility was masked by compensatory mechanisms.

  7. Adrenal hyperactivity, injuries and hominization Hiperactivación adrenal, noxas y hominización

    Directory of Open Access Journals (Sweden)

    William Alvarez Gaviria

    2001-02-01

    Full Text Available In this revision it is postulated that the hominization is a by-product of the endocrine-metabolic adjustment to that hominid ancestors were subjected about 5 million years ago. A bigger adrenalization index is demonstrated in the hominids that partly could explain our fenotype, our behavior and the biggest propensity to certain pathologies. En esta revisión se postula que la hominización es un subproducto del ajuste endocrino-metabólico a que fueron sometidos los ancestros de los homínidos hace unos 5 millones de años. Se demuestra un mayor índice de adrenalización en los homínidos que pudiera en parte explicar nuestro fenotipo, nuestra conducta y la mayor propensión a determinadas patologías.

  8. Effects of adrenal steroids on the bone metabolism of children with congenital adrenal hyperplasia.

    Science.gov (United States)

    Lin-Su, Karen; New, Maria I

    2007-11-01

    The primary treatment for patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is glucocorticoid replacement therapy, which at supraphysiologic levels can result in diminished bone accrual and lead to osteopenia and osteoporosis. Unlike other diseases treated with chronic glucocorticoid therapy, previous studies of patients with 21OHD have not demonstrated a detrimental effect of glucocorticoid treatment on bone mineral density (BMD). It has been postulated that the elevated androgens typically found in these patients have a protective effect on bone integrity, but the precise mechanism remains unknown. We propose that the inhibitory effect of corticosteroid therapy on bone formation is counteracted by estrogen's effect on bone resorption through the RANK-L/osteoprotegerin (OPG) system. A better understanding of the mechanism by which patients with 21OHD are protected against bone loss may lead to novel therapeutic measures to prevent or treat osteopenia and osteoporosis in other conditions, including postmenopausal women.

  9. A brief history of adrenal research: steroidogenesis - the soul of the adrenal.

    Science.gov (United States)

    Miller, Walter L

    2013-05-22

    The adrenal is a small gland that escaped anatomic notice until the 16th century, and whose essential role in physiology was not established until the mid 19th century. Early studies were confounded by failure to distinguish the effects of the cortex from those of the medulla, but advances in steroid chemistry permitted the isolation, characterization and synthesis of many steroids by the mid 20th century. Knowledge of steroid structures, radiolabeled steroid conversions, and the identification of accumulated urinary steroids in diseases of steroidogenesis permitted a generally correct description of the steroidogenic pathways, but one confounded by the failure to distinguish species-specific differences. The advent of cloning technologies and molecular genetics rapidly corrected and clarified the understanding of steroidogenic processes. Our laboratory in San Francisco was one of several contributing to this effort, focusing on human steroidogenic enzymes, the genetic disorders in their biosynthesis and the transcriptional and post-translational mechanisms regulating enzyme activity.

  10. Gonadal development and growth in 46,XX and 46,XY individuals with P450scc deficiency (congenital lipoid adrenal hyperplasia)

    DEFF Research Database (Denmark)

    Müller, J; Torsson, A; Damkjaer Nielsen, M;

    1991-01-01

    pathology. Gonadectomy was performed in the 2 surviving 46,XY individuals at the age of 7 years, and histological examination showed normal testicular morphology but very few germ cells. Postmortem examination of the testes of the 2-month-old subject showed normal testicular histology, and quantitative...... normal breast and pubic hair development after oral estrogen replacement and topical testosterone administration. The glucocorticoid and mineralocorticoid replacement was adjusted in accordance with repeated measurements of serum sodium and serum potassium, plasma renin concentration and blood pressure......We have investigated gonadal development and growth in 4 individuals (3 with 46,XY and 1 with 46,XX karyotype) with P450scc deficiency. One patient died at 2 months of age from adrenal insufficiency, while the remaining 3 individuals were healthy and developed normally (age at follow-up: 18, 10...

  11. Long-term follow-up of a female with congenital adrenal hyperplasia due to P450-oxidoreductase deficiency.

    Science.gov (United States)

    Bonamichi, Beatriz D S F; Santiago, Stella L M; Bertola, Débora R; Kim, Chong A; Alonso, Nivaldo; Mendonca, Berenice B; Bachega, Tania A S S; Gomes, Larissa G

    2016-10-01

    P450 oxidoreductase deficiency (PORD) is a variant of congenital adrenal hyperplasia that is caused by POR gene mutations. The POR gene encodes a flavor protein that transfers electrons from nicotinamide adenine dinucleotide phosphate (NADPH) to all microsomal cytochrome P450 type II (including 21-hydroxylase, 17α-hydroxylase 17,20 lyase and aromatase), which is fundamental for their enzymatic activity. POR mutations cause variable impairments in steroidogenic enzyme activities that result in wide phenotypic variability ranging from 46,XX or 46,XY disorders of sexual differentiation, glucocorticoid deficiency, with or without skeletal malformations similar to Antley-Bixler syndrome to asymptomatic newborns diagnosed during neonatal screening test. Little is known about the PORD long-term evolution. We described a 46,XX patient with mild atypical genitalia associated with severe bone malformation, who was diagnosed after 13 years due to sexual infantilism. She developed large ovarian cysts and late onset adrenal insufficiency during follow-up, both of each regressed after hormone replacement therapies. We also described a late surgical approach for the correction of facial hypoplasia in a POR patient.

  12. Scrotal hematoma as a sign of adrenal hemorrhage in newborns

    Directory of Open Access Journals (Sweden)

    Renata Gonçalves

    Full Text Available CONTEXT: Bluish discoloration and swelling of the scrotum in newborns can arise from a number of diseases, including torsion of the testes, orchitis, scrotal or testicular edema, hydrocele, inguinal hernia, meconium peritonitis, hematocele, testicular tumor and traumatic hematoma. Forty-two cases of scrotal abnormalities as signs of neonatal adrenal hemorrhage were found in the literature. CASE REPORT: We present a case of scrotal hematoma due to adrenal hemorrhage in a newborn. Conservative treatment with clinical follow-up was adopted, with complete resolution within 10 days. The possible differential diagnoses are reviewed and discussed.

  13. Etiology of common childhood acute lymphoblastic leukemia: the adrenal hypothesis

    DEFF Research Database (Denmark)

    Schmiegelow, K.; Vestergaard, T.; Nielsen, S.M.;

    2008-01-01

    The pattern of infections in the first years of life modulates our immune system, and a low incidence of infections has been linked to an increased risk of common childhood acute lymphoblastic leukemia (ALL). We here present a new interpretation of these observations--the adrenal hypothesis......--that proposes that the risk of childhood ALL is reduced when early childhood infections induce qualitative and quantitative changes in the hypothalamus-pituitary-adrenal axis that increase plasma cortisol levels. This may directly eliminate leukemic cells as well as preleukemic cells for the ALL subsets...

  14. Uncommon Cause of Acute Adrenal Failure - Case Report

    Directory of Open Access Journals (Sweden)

    Tariq

    2010-12-01

    Full Text Available Adrenomyeloneuropathy is a rare X-linked inherited disorder of peroxisomes characterized by accumulation of very-long-chain fatty acids (VLCFA in the central and peripheral nervous system, adrenal glands and testes, leading to dysfunction of these organs and systems (1. Here, we report a case of adrenomyeloneuropathy presenting initially as acute adrenal crisis, which progressed rapidly within one year to variant neurological manifestations, dementia, sensory, motor and psycho-intellectual dysfunction, and generalized spasticity. Turk Jem 2010; 14: 103-5

  15. Xanthogranulomatous adrenalitis in a neonate: CT and US findings

    Energy Technology Data Exchange (ETDEWEB)

    Trinavarat, Panruethai; Sasiwimonphan, Kewalee [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Radiology, Faculty of Medicine, Bangkok (Thailand); Sansopha, Lalana [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Pathology, Faculty of Medicine, Bangkok (Thailand); Vejchapipat, Paisarn [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Surgery, Faculty of Medicine, Bangkok (Thailand); Sosothikul, Darintr [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Paediatrics, Faculty of Medicine, Bangkok (Thailand)

    2009-03-15

    We report a 46-day-old female infant with xanthogranulomatous adrenalitis. Ultrasonography showed a complex, solid-cystic right suprarenal mass with poorly defined margins. Colour flow Doppler revealed the solid portion of the mass to be vascular. CT demonstrated a heterogeneous cystic and solid mass with some contrast enhancement in the inferior part of the lesion. There was compression of the adjacent upper pole of the right kidney and the lateral aspect of the inferior vena cava. The mass was completely removed; histological examination revealed xanthogranulomatous adrenalitis. At the time of this report she remained well 3 years following surgery. (orig.)

  16. Arecoline inhibits catecholamine release from perfused rat adrenal gland

    Institute of Scientific and Technical Information of China (English)

    Dong-yoon LIM; Il-sik KIM

    2006-01-01

    Aim: To study the effect of arecoline, an alkaloid isolated from Areca catechu, on the secretion of catecholamines (CA) evoked by cholinergic agonists and the membrane depolarizer from isolated perfused rat adrenal gland. Methods: Adrenal glands were isolated from male Sprague-Dawley rats. The adrenal glands were perfused with Krebs bicarbonate solution by means of a peristaltic pump. The CA content of the perfusate was measured directly using the fluorometric method.Results: Arecoline (0.1-1.0 mmol/L) perfused into an adrenal vein for 60 min produced dose- and time-dependent inhibition in CA secretory responses evoked by acetylcholine (ACh) (5.32 mmol/L), 1.1-dimethyl-4-phenyl piperazinium iodide (DMPP) (100 μmol/L for 2 min) and 3-(m-choloro-phenyl-carbamoyl-oxy)-2-butynyl trimethyl ammonium chloride (McN-A-343) (100 μmol/L for 2 min). However, lower doses of arecoline did not affect CA secretion of high K+ (56 mmol/L); higher doses greatly reduced CA secretion of high K+. Arecoline also failed to affect basal catecholamine output. Furthermore, in adrenal glands loaded with arecoline (0.3 mmol/L), CA secretory response evoked by Bay-K-8644 (10 μmol/L), an activator of L-type Ca2+ channels, was markedly inhibited, whereas CA secretion by cyclopiazonic acid (10 μmol/L), an inhibitor of cytoplasmic Ca2+-ATPase, was not affected. Nicotine (30 μmol/L), which was peffused into the adrenal gland for 60min, however, initially enhanced ACh-evoked CA secretory responses. As time elapsed, these responses became more inhibited, whereas the initially enhanced high K+-evoked CA release diminished. CA secretion evoked by DMPP and McNA-343 was significantly depressed in the presence of nicotine. Conclusion:Arecoline dose-dependently inhibits CA secretion from isolated perfused rat adrenal gland evoked by activation of cholinergic receptors. At lower doses arecoline does not inhibit CA secretion through membrane depolarization, but at larger doses it does. This inhibitory

  17. Adrenal Rest Tumor from the Greater Omentum Mimicking Exophytic Hepatocellular Carcinoma (HCC): A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hyung Jo; Kim, Seong Hoon; Shin, Hyun Woong [Daegu Fatima Hospital, Daegu (Korea, Republic of); Park, Jae Bok [Catholic University of Daegu, Gyeongsan (Korea, Republic of); Jo, Hyun Chul; Son, Mi Young; Kim, Tae Bong [Daegu Veterans Hospital, Daegu (Korea, Republic of)

    2010-02-15

    Adrenal rest tumors are aberrant adrenocortical tissue which has been most commonly described in abdominal and pelvic sites. To our knowledge, there has been no previous description of an adrenal rest tumor of the greater omentum. We present a case of a pathologically confirmed adrenal rest tumor of the greater omentum in a 76-year- old man

  18. File list: NoD.Oth.50.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

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  9. Absence of clinically relevant growth acceleration in untreated children with non-classical congenital adrenal hyperplasia.

    NARCIS (Netherlands)

    Pijnenburg-Kleizen, K.J.; Borm, G.F.; Otten, B.J.; Schott, D.A.; Akker, E.L. van den; Stokvis-Brantsma, W.H.; Voorhoeve, P.G.; Bakker, B.; Claahsen-van der Grinten, H.L.

    2012-01-01

    BACKGROUND/AIMS: In classical congenital adrenal hyperplasia (CAH), elevation of adrenal androgens leads to accelerated growth and bone maturation with compromised adult height. In untreated children with non-classical CAH (NC-CAH), in which adrenal androgens are generally only slightly increased, g

  10. [Adrenal hemorrhage in a newborn with factor V Leiden--a clinical case].

    Science.gov (United States)

    Vulkova, A; Kovacheva, K; ionov, M; Rosmanova, R; Atanasova, V; Slavkova, N; Ivanov, P

    2009-01-01

    Neonatal adrenal hemorrhage is frequently associated with birth trauma, perinatal asphyxia, septicemia, coagulation defects and thromboembolism. We report a case of bilateral adrenal hemorrhage in newborn baby with perinatal asphyxia. Ultrasound findings of subacute adrenal hemorrhage draw attention to its presumable antenatal genesis. DNA analysis for thrombophilic mutations identifies factor V Leiden.

  11. Urine Steroid Metabolomics as a Biomarker Tool for Detecting Malignancy in Adrenal Tumors

    NARCIS (Netherlands)

    Arlt, Wiebke; Biehl, Michael; Taylor, Angela E.; Hahner, Stefanie; Libe, Rossella; Hughes, Beverly A.; Schneider, Petra; Smith, David J.; Stiekema, Han; Krone, Nils; Porfiri, Emilio; Opocher, Giuseppe; Bertherat, Jerome; Mantero, Franco; Allolio, Bruno; Terzolo, Massimo; Nightingale, Peter; Shackleton, Cedric H. L.; Bertagna, Xavier; Fassnacht, Martin; Stewart, Paul M.

    2011-01-01

    Context: Adrenal tumors have a prevalence of around 2% in the general population. Adrenocortical carcinoma (ACC) is rare but accounts for 2-11% of incidentally discovered adrenal masses. Differentiating ACC from adrenocortical adenoma (ACA) represents a diagnostic challenge in patients with adrenal

  12. Insufficient milk supply and breast cancer risk: a systematic review.

    Directory of Open Access Journals (Sweden)

    Jacqueline M Cohen

    Full Text Available BACKGROUND: An association between insufficient milk supply, the inability of a mother's breast milk to provide sufficiently for her infant, and breast cancer has been suggested by observations in animal models. To determine if an association has been reported in epidemiological studies of human breast cancer, a systematic review of the literature has been conducted. We also sought to identify the methodological limitations of existing studies to guide the design of any future prospective studies in this field. METHODOLOGY/PRINCIPAL FINDINGS: PubMed, EMBASE, Web of Science, BIOSIS, and CAB abstracts were searched. We selected any study that (1 assessed breast cancer in association with breastfeeding history and (2 examined the relationship between insufficient milk supply with breast cancer. Seven relevant studies were identified that met both criteria. There was statistically significant heterogeneity among the results which likely reflects clinically significant differences in definitions of insufficient milk supply and reference groups that were used. Among premenopausal women who had experienced insufficient milk supply, odds ratios (ORs for breast cancer risk ranged from 0.9 to 16.3. Among postmenopausal women, ORs ranged from 0.6 to 6.7. Based on the range of odds ratios obtained in the studies reported in this review, it remains unclear if there is a true association between insufficient milk supply and breast cancer. CONCLUSIONS/SIGNIFICANCE: Although some studies have shown a strong positive association, there is no consistent evidence for an effect of insufficient milk supply on breast cancer risk. Exposure definitions are in need of improvement in order to focus on primary insufficient milk supply. Reference groups consisting of women who have successfully breastfed may also introduce positive bias (inflation of the odds ratio into study results because of the protective effect of prolonged breastfeeding in the control group.

  13. Ultrasonographic visualization of the adrenal glands of healthy ferrets and ferrets with hyperadrenocorticism.

    Science.gov (United States)

    Kuijten, Andrea Maria; Schoemaker, Nico J; Voorhout, George

    2007-01-01

    A protocol was developed to compare the ultrasonographic characteristics of the adrenal glands of 21 healthy ferrets and 37 ferrets with hyperadrenocorticism. By using specific landmarks, the adrenal glands were imaged in 97% of the cases. The adrenal glands of ferrets with hyperadrenocorticism had a significantly increased thickness, with changes in shape, structure, and echogenicity compared to the adrenal glands of healthy ferrets. Based on the findings of the study, adrenal glands may be classified as abnormal when they have a rounded appearance, increased size of the cranial/caudal pole (thickness >3.9 mm), a heterogeneous structure, increased echogenicity, and/or signs of mineralization.

  14. Lymphocytic adrenal medullitis and lymphocytic thyroiditis in a laboratory beagle dog.

    Science.gov (United States)

    Doi, Takuya; Tomonari, Yuki; Kawasako, Kazufumi; Yamada, Naoaki; Tsuchitani, Minoru

    2017-02-04

    Lymphocytic adrenal medullitis characterized by inflammation and atrophy in the medulla of the bilateral adrenal glands was observed in an 18-month-old male laboratory beagle dog. It might be that the present lymphocytic adrenal medullitis is an autoimmune-mediated disease as the histological characteristics are consistent with an autoimmune pathogenesis. However, the actual cause remains unclear as the existence of serum autoantibodies against the adrenal medulla could not be confirmed. Although this dog also contracted lymphocytic thyroiditis along with serum thyroglobulin autoantibodies, indicating that the thyroiditis occurred with an autoimmune basis; the relation between the adrenal medullitis and thyroiditis is unknown.

  15. MRI of occult sacral insufficiency fractures following radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Mammone, J.F. [Dept. of Radiology, Thomas Jefferson Univ. Hospital, and Jefferson Medical Coll., Philadelphia, PA (United States); Schweitzer, M.E. [Dept. of Radiology, Thomas Jefferson Univ. Hospital, and Jefferson Medical Coll., Philadelphia, PA (United States)

    1995-02-01

    Following radiation therapy, marrow abnormalities noted on magnetic resonance imaging (MRI) are frequent and may mimic metastases. Specific radiotherapy changes are usually easily identifiable; however, traumatic lesions cause more interpretive difficulties. We assessed the incidence and MRI characteristics of insufficiency fractures in this population. During a 5-year span (1987-1991), 546 patients received pelvic radiotherapy for primary malignancies. MRI was performed in 25 of these patients at least 3 months after treatment. The mean dose in this group was 53 Gy. These MRI scans were retrospectively reviewed for the appearance of the sacrum with particular attention to the presence of insufficiency fractures. This was correlated with clinical course and scintigraphic findings. Presumed insufficiency fractures on MRI paralleled the sacral side of the sacroiliac joint, enhanced with Gd-DTPA, were most prominent or initially seen anteriorly, and had ill-defined margins on all imaging sequences. The incidence of occult sacral insufficiency fractures was at least 20%. Insufficiency fractures of the sacrum in the post-radiotherapy patient are a relatively frequent occurrence which can mimic metastases. Consideration of this phenomenon and knowledge of differential features may avoid overdiagnosis of osseous metastases. (orig.)

  16. Antiphospholipid Antibody Syndrome Presenting with Unilateral Adrenal Hemmorhage.

    Science.gov (United States)

    Ullah, Kifayat; Butt, Ghias; Neopane, Sippy; Arshi, Shahana

    2016-06-01

    The antiphospholipid antibody syndrome presents with vascular thrombosis which involve both arterial and venous systems. The clinical presentation of antiphospholipid antibody syndrome includes obstetric complications leading to recurrent abortions, presence of circulating antibodies against phospholipids, and multi-organ thromboembolisms. We report a case of a patient who presented with unilateral adrenal hemorrhage and subsequently found to have antiphospholipid antibody syndrome and lupus nephritis.

  17. Primitive neuroectodermal tumor of adrenal: clinical presentation and outcomes.

    Science.gov (United States)

    Dutta, Deep; Shivaprasad, K S; Das, Ram Narayan; Ghosh, Sujoy; Chowdhury, Subhankar

    2013-01-01

    Primitive neuroectodermal tumor (PNET) of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm) on computed tomography (CT) was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE). This is the first report of adrenal peripheral PNET (pPNET) from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma.

  18. Primitive neuroectodermal tumor of adrenal: Clinical presentation and outcomes

    Directory of Open Access Journals (Sweden)

    Deep Dutta

    2013-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm on computed tomography (CT was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen, vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE. This is the first report of adrenal peripheral PNET (pPNET from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma.

  19. Adrenal myelolipoma within myxoid cortical adenoma associated with Conn's syndrome

    Institute of Scientific and Technical Information of China (English)

    Hong-sheng LU; Mei-fu GAN; Han-song CHEN; Shan-qiang HUANG

    2008-01-01

    The coexistence of myelolipoma within adrenal cortical adenoma is extremely rare, for both tumors present usually as separate entities. There are only 16 such cases reported worldwide. To the best of our knowledge, the case we reported here is the first one of myxoid adrenal cortical adenoma associated with myelolipoma reported. A 32-year-old Chinese woman with 4-year history of hypertension was presented in our study. Computed tomography (CT) of the abdomen showed a large heterogeneously-enhancing mass (4.5cm in diameter) in the left suprarenal region. Clinical history and laboratory results suggest a metabolic disorder as Conn's syndrome. The patient underwent a left adrenalectomy, and a histopathological study confirmed the mass to be a myxoid adrenal cortical adenoma containing myelolipoma. The patient was postoperatively well and discharged uneventfully. In the present case report, we also discuss the etiology of simultaneous myelolipoma and adrenal adenoma associated with Conn's syndrome, and the methods of the diagnosis and differential diagnosis.

  20. Dopamine-secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacks.

    Science.gov (United States)

    Erem, Cihangir; Kocak, Mustafa; Cinel, Akif; Erso, Halil O; Reis, Abdulkadir

    2008-01-01

    Adrenal ganglioneuromas are rare tumors originating from the neural crest tissue of the sympathetic nervous system. The clinical presentation for most patients is asymptomatic, and most of these tumors are hormone silent. We report a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient. A 46-year-old woman was admitted to our hospital with a 2-month history of right flank pain, and a 2-year history of paroxysmal hypertensive attacks associated with headaches, palpitations, nervousness, and sweating. Abdominal CT and MRI revealed a solid round tumor approximately 4 cm in diameter on the upper pole of the right kidney. Urinary levels of dopamine and homovanillic acid were slightly elevated, although urinary levels of metanephrine and normetanephrine were suppressed. The urinary levels of epinephrine, norepinephrine, and vanillylmandelic acid were within normal limits. Right adrenalectomy was performed for treatment purposes. Histological diagnosis of the tumor was a ganglioneuroma originating from the adrenal medulla. In conclusion, this is a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient.

  1. A Case of Congenital Adrenal Hyperplasia Mimicking Cushing's Syndrome

    Science.gov (United States)

    Kim, Hye Jeong; Kang, Mira; Kim, Jae Hyeon; Kim, Sun Wook; Chung, Jae Hoon; Min, Yong-Ki; Lee, Moon-Kyu; Kim, Kwang-Won

    2012-01-01

    Congenital adrenal hyperplasia (CAH) is characterized by decreased adrenal hormone production due to enzymatic defects and subsequent rise of adrenocorticotrophic hormone that stimulates the adrenal cortex to become hyperplastic, and sometimes tumorous. As the pathophysiology is basically a defect in the biosynthesis of cortisol, one may not consider CAH in patients with hypercortisolism. We report a case of a 41-yr-old man with a 4 cm-sized left adrenal tumorous lesion mimicking Cushing's syndrome who was diagnosed with CAH. He had central obesity and acanthosis nigricans involving the axillae together with elevated 24-hr urine cortisol level, supporting the diagnosis of Cushing's syndrome. However, the 24-hr urine cortisol was suppressed by 95% with the low dose dexamethasone suppression test. CAH was suspected based on the history of precocious puberty, short stature and a profound suppression of cortisol production by dexamethasone. CAH was confirmed by a remarkably increased level of serum 17-hydroxyprogesterone level. Gene mutation analysis revealed a compound heterozygote mutation of CYP21A2 (I173N and R357W). PMID:23166432

  2. Effect of copper deficiency on plasma and adrenal catecholamines

    Energy Technology Data Exchange (ETDEWEB)

    Kennedy, B.W.; Bhathena, S.J.; Fields, M.; Voyles, N.R.; Timmers, K.I.; Recant, L.

    1986-03-01

    Copper (Cu) ion is an essential component of enzymes involved in catecholamine (CAT) metabolism. Copper deficiency (CuD) has been shown to affect the CAT content of brain (decreased norepinephrine (NE) and dopamine (DA)) and heart (increased DA and decreased NE). It is thus possible that plasma and adrenal CAT could be altered by CuD. The authors, then, investigated the effect of CuD on plasma and adrenal CAT in rats fed copper-deficient (0.6 ..mu..g Cu/g) or copper-supplemented (6.0 ..mu..g Cu/g) diets with either 62% starch (S) or fructose (F) as the carbohydrate source for 7 weeks after weaning. CuD was ascertained by decreased plasma Cu and ceruloplasmin activity. CuD increased the levels of all three CAT (NE, P < 0.001, epinephrine (E), P < 0.0001 and DA, P < 0.01) in plasma, but had no effect on adrenal CAT content. Dietary F had no significant effect on plasma CAT compared to S but did increase E in adrenal tissue (P < 0.001). They have previously demonstrated that CuD is accompanied by increased plasma glucose, triglyceride and cholesterol and decreased insulin. Thus, increased plasma CAT along with decreased plasma insulin may explain, in part, the glucose intolerance and abnormal lipid metabolism observed in CuD.

  3. Adrenal pseudocyst as a cause of postpartum abdominal pain.

    Science.gov (United States)

    Zanghì, A; Di Vita, M; Lo Menzo, E; Fiorica, F; Cavallaro, A; Cimino, L; Piccolo, G; Palmucci, S; Cappellani, A

    2012-01-01

    Among adrenal masses only 0.06% to 0.18% are cysts and among them, pseudocysts are the second (39%) most common lesions. Due to the increased use of imaging studies their incidence seems to have increased. Most of these lesions are incidentally found during radiologic investigation or at the time of autopsy, and only rarely they are detected in pregnant women. The latter scenario warrants emergency surgery, due to the risk of rupture. We present a case of a 39-years old woman presenting with unrelenting left flank pain due to a large adrenal pseudocyst soon after her first delivery. Four months after, she presented to our surgical division for persistent pain and anemia so underwent an extensive work up that showed a large pseudocystic mass (8 x 8 cm ) of the left adrenal gland. Once the neoplastic and parasitic etiology of the lesion were excluded, she underwent uneventful laparoscopic adrenalectomy. She was discharged home three days postoperatively. This case is the 13th case in the English literature and at the same time is the first case of adrenal pseudocyst occurred four months after delivery.

  4. Adrenal hyperplastic and degenerative changes in beluga whales.

    Science.gov (United States)

    Lair, S; Béland, P; De Guise, S; Martineau, D

    1997-07-01

    Thirty stranded beluga whales (Delphinapterus leucas) from the St. Lawrence Estuary (Quebec, Canada) population and five animals from the Hudson Bay aboriginal hunt (North-west Territories, Canada) were examined. Twenty one animals from the St. Lawrence Estuary had mild to severe adrenal lesions and four whales from the Hudson Bay population were affected by minimal adrenal changes. Cortical hyperplasia was observed in 24 adult beluga whales all from the St. Lawrence Estuary. Bilateral cortical cysts and cellular vacuolar degeneration were observed in the adrenal glands of 19 beluga whales from both populations. The cysts, filled with a cortisol-rich liquid, were present in both sexes. Beluga whales with adrenal cysts were significantly older than animals without cysts, and the severity of the lesions increased with age. Nodular hyperplasia of the medulla was observed in seven of the beluga whales, all from the St. Lawrence Estuary population. All lesions could be part of a normal aging process. The adrenocortical lesions might be due to stress or adrenocorticolytic xenobiotics, while the medullary hyperplasia might be caused by hypoxia or exposure to estrogenic xenobiotics.

  5. COMPARATIVE MORPHOFUNCTIONAL CHARACTERISTIC OF ADRENAL GLANDS IN ARTERIAL HYPERTENSION

    Directory of Open Access Journals (Sweden)

    S. Yu. Alyabyeva

    2015-01-01

    Full Text Available The article is devoted to the study of morphological changes of the adrenal glands in arterial hyperten-sion. Adrenals investigated persons who do not suffer in life and suffering from hypertension during the life of hypertension and died from various causes – is incompatible with the life of a mechanical trauma, cerebral hemorrhage, and acute left ventricular failure. In each case, each of the adrenal glands were assessed: the presence and severity of focal and diffuse mononuclear infiltration, the number of lympho-cytes, monocytes, plasma cells and fibroblasts arranged in the respective zones of the cortex and medulla, the severity of hyperemia and edema of various structural parts, the number of zones cortex and medulla endocrinocytes with pycnotic nuclei in the reticular zone evaluated the distribution of secretory cells with lipofuscin and the degree of saturation of the cytoplasm of the secretory cells, in secretory cells of medul-la evaluated nuclear-cytoplasmic ratio, the severity of vacuolization and basophils cytoplasm revealed a number of regularities of morphological changes depending from the various clinical manifestations of hypertension, defining the ultimate option of dying. At the heart tanatogenesis version are more pro-nounced morphological features hyperfunctions glomerular zone – namely delipidization left and right glands. The beam and netted areas contralateral glands embodiment of dying of a heart compared to the brain, is more pronounced hyperemia. When cardiac variant tanatogenesis more pronounced focal lym-phoid infiltration and vacuolization in secretory cells of left adrenal medulla.

  6. Adrenal GRK2 upregulation mediates sympathetic overdrive in heart failure.

    Science.gov (United States)

    Lymperopoulos, Anastasios; Rengo, Giuseppe; Funakoshi, Hajime; Eckhart, Andrea D; Koch, Walter J

    2007-03-01

    Cardiac overstimulation by the sympathetic nervous system (SNS) is a salient characteristic of heart failure, reflected by elevated circulating levels of catecholamines. The success of beta-adrenergic receptor (betaAR) antagonists in heart failure argues for SNS hyperactivity being pathogenic; however, sympatholytic agents targeting alpha2AR-mediated catecholamine inhibition have been unsuccessful. By investigating adrenal adrenergic receptor signaling in heart failure models, we found molecular mechanisms to explain the failure of sympatholytic agents and discovered a new strategy to lower SNS activity. During heart failure, there is substantial alpha2AR dysregulation in the adrenal gland, triggered by increased expression and activity of G protein-coupled receptor kinase 2 (GRK2). Adrenal gland-specific GRK2 inhibition reversed alpha2AR dysregulation in heart failure, resulting in lowered plasma catecholamine levels, improved cardiac betaAR signaling and function, and increased sympatholytic efficacy of a alpha2AR agonist. This is the first demonstration, to our knowledge, of a molecular mechanism for SNS hyperactivity in heart failure, and our study identifies adrenal GRK2 activity as a new sympatholytic target.

  7. Mistaken gender identity in non-classical congenital adrenal hyperplasia

    OpenAIRE

    Kukreti, Prerna; Kandpal, Manish; Jiloha, R C

    2014-01-01

    Gender identity is the sense of belonging that one feels for a particular sex psychologically and socially, independent of one's biological sex. There is much less systematic data on gender identity in females with congenital adrenal hyperplasia (CAH). We report a case of non-classical CAH presenting as a case of gender identity disorder.

  8. Mistaken gender identity in non-classical congenital adrenal hyperplasia.

    Science.gov (United States)

    Kukreti, Prerna; Kandpal, Manish; Jiloha, R C

    2014-04-01

    Gender identity is the sense of belonging that one feels for a particular sex psychologically and socially, independent of one's biological sex. There is much less systematic data on gender identity in females with congenital adrenal hyperplasia (CAH). We report a case of non-classical CAH presenting as a case of gender identity disorder.

  9. Work up of incidental adrenal mass: state of the art.

    Science.gov (United States)

    Bada, Maida; Castellan, Pietro; Tamburro, Fabiola R; Berardinelli, Francesco; Neri, Fabio; Cindolo, Luca; Schips, Luigi

    2016-11-18

    Due to the increasing use of radiological investigations, the detection of incidental adrenal masses has become even more frequent. Therefore, it is crucial to identify the nature of the adrenal mass in order to decide the type of treatment that should be undertaken. Toward this goal, biochemical tests are useful in order to assess catecholamines levels for the presence of a pheochromocytoma or cortisol excess in case of Cushing's syndrome. Furthermore, the dexamethasone suppression test and late-night salivary cortisol may be useful in measuring plasma cortisol, respectively, in the blood and urine. Hyperaldosteronism could be suspected in the presence of arterial hypertension. With regard to imaging modalities, the contrast washout and Hounsfield units estimation might play a role as indicators on computed tomography. In terms of treatment, a surgical approach is most suitable for a hyperfunctioning adrenal mass irrespective of size, and for nonfunctioning masses >4 cm. For indeterminate smaller lesions, with washout >50%, <10 Hounsfield Unit, nonfunctioning, benign-appearing, undergoing a follow-up in regular intervals is more appropriate in order to estimate mass growth. This paper summarizes recent findings on the management of incidental adrenal masses, with a special focus on the use of imaging, surgical management and follow-up modalities in improving patient outcomes.

  10. Adrenal metastasis as first presentation of hepatocellular carcinoma

    Directory of Open Access Journals (Sweden)

    Zacharakis Evangelos

    2005-07-01

    Full Text Available Abstract Background Metastases from hepatocellular carcinoma (HCC can be found in the lung and adrenal gland. We report case of a patient who presented with adrenal metastasis as the first clinical manifestation of HCC. Case presentation A patient was referred for surgical treatment for a tumor in retro-peritoneal space. The computerized tomography (CT scan revealed a mass originating from the left adrenal gland. The patient underwent left adrenalectomy and the exploration of abdominal cavity did not reveal any other palpable lesions. Histologically, the resected lesion was a poorly differentiated metastatic tumor from HCC. Seven months later patient was readmitted complaining of cachexia, icterus, and significant weight loss. CT scan revealed hyperdense lesions of the liver Conclusion HCC may have atypical presentations like in present case. Fine needle aspiration/tru-cut® biopsy might be useful in the investigation of an accidentally discovered adrenal mass regardless of the size and can lead to the detection of a primary tumor.

  11. Serotonergic stimulation of the rat hypothalamo-pituitary-adrenal axis

    DEFF Research Database (Denmark)

    Mikkelsen, Jens D; Hay-Schmidt, Anders; Kiss, Alexander

    2004-01-01

    Acute stimulation of the hypothalamo-pituitary-adrenal (HPA) axis by selective serotonin reuptake inhibitors (SSRIs) is mediated by several postsynaptic 5-HT receptor subtypes. Activation of 5-HT(1A) and 5-HT(2A) receptors increases plasma corticosterone levels, and it is likely that these recept...

  12. Congenital adrenal hyperplasia with localized aggressive periodontitis and amelogenesis imperfecta.

    Science.gov (United States)

    Ajlan, Sumaiah Abdulbaqi

    2015-11-01

    Congenital adrenal hyperplasia (CAH) is an inherited medical condition that implies defects in steroid biosynthesis. The dental findings of a female patient with CAH are reported. The patient suffered from severe periodontal tissue destruction, obvious enamel defects, as well as some occlusal problems. The management approach is presented and the possibility of interrelation of her dental findings with her medical condition is discussed.

  13. GPCRs of adrenal chromaffin cells & catecholamines: The plot thickens.

    Science.gov (United States)

    Lymperopoulos, Anastasios; Brill, Ava; McCrink, Katie A

    2016-08-01

    The circulating catecholamines (CAs) epinephrine (Epi) and norepinephrine (NE) derive from two major sources in the whole organism: the sympathetic nerve endings, which release NE on effector organs, and the chromaffin cells of the adrenal medulla, which are cells that synthesize, store and release Epi (mainly) and NE. All of the Epi in the body and a significant amount of circulating NE derive from the adrenal medulla. The secretion of CAs from adrenal chromaffin cells is regulated in a complex way by a variety of membrane receptors, the vast majority of which are G protein-coupled receptors (GPCRs), including adrenergic receptors (ARs), which act as "presynaptic autoreceptors" in this regard. There is a plethora of CA-secretagogue signals acting on these receptors but some of them, most notably the α2ARs, inhibit CA secretion. Over the past few years, however, a few new proteins present in chromaffin cells have been uncovered to participate in CA secretion regulation. Most prominent among these are GRK2 and β-arrestin1, which are known to interact with GPCRs regulating receptor signaling and function. The present review will discuss the molecular and signaling mechanisms by which adrenal chromaffin cell-residing GPCRs and their regulatory proteins modulate CA synthesis and secretion. Particular emphasis will be given to the newly discovered roles of GRK2 and β-arrestins in these processes and particular points of focus for future research will be highlighted, as well.

  14. Hemorrhagic shock due to spontaneous rupture of adrenal pheochromocytoma

    Directory of Open Access Journals (Sweden)

    Bittencourt José A.

    2003-01-01

    Full Text Available The spontaneous rupture of an adrenal pheochromocytoma is an extremely rare event; however, it is potentially fatal. We report a case of spontaneous rupture of pheochromocytoma followed by an extensive retroperitoneal hematoma and hypovolemic shock. The correct diagnostic assessment determined the adequate surgical approach and a favorable outcome for the patient.

  15. Concomitant testicular seminoma and ectopic adrenal tissue of the cord in a 45-year-old male.

    Science.gov (United States)

    Floyd, Michael S; Itam, Sarah; Nasir, Nyla; Weerasinghe, Suboda M; Irwin, Paul P; Maddineni, Satish B

    2014-01-01

    Aberrant adrenal tissue near the adrenal gland is common, but the finding of ectopic adrenal tissue in structures around the spermatic cord and testis is rare. We describe a case of concomitant seminoma and ectopic adrenal tissue of the spermatic cord occurring in an adult patient who had undergone orchidopexy as a child.

  16. Congenital adrenal hyperplasia: a case report with premature teeth exfoliation and bone resorption.

    Science.gov (United States)

    Angelopoulou, Matina V; Kontogiorgos, Elias; Emmanouil, Dimitris

    2015-06-01

    Congenital adrenal hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by insufficient production of cortisol. The aim of this case report was to present a child with CAH, premature exfoliation of primary teeth and accelerated eruption of his permanent teeth related to bone resorption. A 4.5-year-old Caucasian boy with CAH and long-term administration of glucocorticoids was referred for dental restoration. Clinical examination revealed primary molars with worn stainless steel crowns, severe attrition of the upper canines, and absence of the upper incisors. Before the completion of treatment, abnormal mobility of the first upper primary molars and the lower incisors was detected, and a few days later the teeth exfoliated prematurely. Histologic examination revealed normal tooth structure. Alkaline phosphatase and blood cells values were normal. Eruption of the permanent dentition was also accelerated. Tooth mobility was noticed in the permanent teeth as soon as they erupted, along with bone destruction. Examination revealed an elevated level of receptor activator of nuclear factor-κB ligand and lower-than-normal osteoprotegerin and vitamin D levels. The patient was treated with vitamin D supplements, and his teeth have been stable ever since. CAH is a serious chronic disorder appearing in children with accelerated dental development and possibly premature loss of primary teeth.

  17. Embryology of the adrenal glands and its relevance to diagnostic imaging

    Energy Technology Data Exchange (ETDEWEB)

    Barwick, T.D. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom)]. E-mail: tara.barwick@btinternet.com; Malhotra, A. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom); Webb, J.A.W. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom); Savage, M.O. [Department of Paediatric Endocrinology, St Bartholomew' s Hospital, London (United Kingdom); Reznek, R.H. [Department of Diagnostic Radiology, St Bartholomew' s Hospital, London (United Kingdom)

    2005-09-01

    An understanding of the embryology of the adrenal glands is necessary to appreciate the location of adrenal ectopic, or rest, tissue which can occur anywhere along the course of gonadal descent. This tissue usually has no clinical significance, but may become hyperplastic in patients with primary or secondary adrenal pathology. In congenital adrenal hyperplasia, hyperplastic rest tissue may present as a soft-tissue mass, particularly in the gonads and retroperitoneum, and may be mistaken for tumour. The adrenal in the neonate is proportionately much larger than in the adult; in renal ectopy or agenesis the ipsilateral adrenal is normally sited and may be mistaken for a kidney because of its size. This review article illustrates the embryology of the adrenal with particular emphasis on the relevance of embryology to pathology.

  18. Doppler ultrasound study and venous mapping in chronic venous insufficiency.

    Science.gov (United States)

    García Carriazo, M; Gómez de las Heras, C; Mármol Vázquez, P; Ramos Solís, M F

    2016-01-01

    Chronic venous insufficiency of the lower limbs is very prevalent. In recent decades, Doppler ultrasound has become the method of choice to study this condition, and it is considered essential when surgery is indicated. This article aims to establish a method for the examination, including venous mapping and preoperative marking. To this end, we review the venous anatomy of the lower limbs and the pathophysiology of chronic venous insufficiency and explain the basic hemodynamic concepts and the terminology required to elaborate a radiological report that will enable appropriate treatment planning and communication with other specialists. We briefly explain the CHIVA (the acronym for the French term "cure conservatrice et hémodynamique de l'insuffisance veineuse en ambulatoire"=conservative hemodynamic treatment for chronic venous insufficiency) strategy, a minimally invasive surgical strategy that aims to restore correct venous hemodynamics without resecting the saphenous vein.

  19. Aldo-keto reductases 1B in adrenal cortex physiology

    Directory of Open Access Journals (Sweden)

    Emilie PASTEL

    2016-07-01

    Full Text Available Aldose reductase proteins are cytosolic monomeric enzymes, belonging to the aldo-keto reductase (AKR superfamily. They perform oxidoreduction of carbonyl groups from a wide variety of substrates such as aliphatic and aromatic aldehydes or ketones. The Aldose reductase subgroup (AKR1B is one of the most characterized because of its involvement in human diseases such as diabetic complications resulting from the ability of its human archetype AKR1B1 to reduce glucose into sorbitol. However the issue of AKR1B function in non pathologic condition remains poorly resolved. Adrenal steroidogenesis is strongly associated with high production of endogenous harmful lipid aldehyde by-products including isocaproaldehyde (4-methylpentanal derived from cholesterol side chain cleavage (the first step of steroid synthesis and 4-hydroxynonenal (4- HNE that can both be reduced by AKR1B proteins. More recently, some AKR1B isoforms have been shown to be endowed with prostaglandin F synthase activity, suggesting that in addition to possible scavenger function, they could instigate paracrine signals. Interestingly, previous studies have established that the adrenal gland is one of the major site for human and murine AKR1B expression suggesting that their detoxifying/signaling activity could be specifically required for the correct handling of adrenal function. Moreover chronic effects of ACTH result in a coordinated regulation of genes encoding the steroidogenic enzymes and some AKR1B isoforms.This review presents the molecular mechanisms accounting for the adrenal specific expression of some AKR1B genes. Using data from recent mouse genetic models, we will try to connect their enzymatic properties and regulation with adrenal functions.

  20. Adrenal morpho-functional alterations in patients with acromegaly.

    Science.gov (United States)

    Scaroni, C; Selice, R; Benedini, S; De Menis, E; Arosio, M; Ronchi, C; Gasperi, M; Manetti, L; Arnaldi, G; Polenta, B; Boscaro, M; Albiger, N; Martino, E; Mantero, F

    2008-07-01

    Acromegaly is associated with a greater morbidity and higher incidence of tumors, possibly due to the permissive role of elevated GH and IGF-I levels. In the general population, adrenal masses are frequently discovered (prevalence 1-5%) at computed tomography (CT). We evaluated the prevalence of adrenal lesions in patients with acromegaly. We studied 94 acromegalic patients, 54 females (mean age 55.0+/-16.0 yr) and 40 males (mean age 50+/-14 yr) referred to 5 Endocrinology Units between 2001-2003; 49 had active disease and 45 had been treated with surgery and/or were controlled with medical therapy. Abdominal CT showed adrenal lesions in 27 patients; 9 of them had unilateral masses (10%) with benign features (diameter 0.5-3 cm) and 18 had hyperplasia (14 monolateral and 4 bilateral), with no significant differences between patients with active vs controlled disease, and with no correlation between prevalence of masses and duration of disease, GH and IGF-I levels. Hormone study (urinary free cortisol, catecholamines/metanephrines, upright plasma renin activity and aldosterone, morning plasma ACTH and low-dose dexamethasone suppression test) disclosed no major endocrine alterations. During a 1-yr follow-up, the adrenal masses increased in size in 3 cases and 1 patient also developed subclinical Cushing's syndrome. Adrenal lesions seem more frequent in acromegaly than in the general population, but no single factor (GH/IGF-I levels or disease duration) predicts them. The masses appear to be benign and nonhypersecreting, but a longer follow-up is recommended to disclose any changes in their morphofunctional state.

  1. Nuclear imaging to characterize adrenal tumors: Comparison with MRI

    Institute of Scientific and Technical Information of China (English)

    Simone; Maurea; Pier; Paolo; Mainenti; Valeria; Romeo; Carmine; Mollica; Marco; Salvatore

    2014-01-01

    AIM:To describe the role of nuclear imaging modalities using nor-cholesterol,metaiodobenzylguanidine(MIBG)and fluorine-deoxy-glucose(FDG)in adrenal tumors for lesion characterization in comparison with magnetic resonance(MR).METHODS:Population was classified in group 1 consisting of 30 patients with non-hypersecreting unilateral adrenal masses,in group 2 consisting of 34 patients with hypersecreting(n=19)or non-hypersecreting(n=15)adrenal adenomas and in group 3 consisting of 18 patients with chromaffin-tissue tumors(CTT),of which 14 were pheochromocytomas while 4 were paragangliomas(n=4).All patients underwent MR and nuclear studies(nor-cholesterol,MIBG and FDG).Pathology samples(n=63)or follow-up data in adenomas(n=19)were used as standard of reference forimaging studies interpretation.RESULTS:In group 1,MR findings were not highly accurate for lesion characterization,while the results of nuclear scans showed abnormal nor-cholesterol,MIBG and FDG concentration in all cases of adenomas,pheos and malignant tumors,respectively.In group 2,no differences in MR parameters were found between hyperfunctioning and non-hyperfunctioning adenomas,while nor-cholesterol uptake was significantly higher in hyperfunctioning compared to non-hyperfunctioning lesions.In group 3,no differences in MR parameters were found between benign and malignant CCT,while MIBG uptake was significantly higher in malignant compared to benign tumors.CONCLUSION:On the basis of our findings,nuclear imaging modalities using specific target agents are able to better characterize adrenal tumors,compared with MR.In particular,radionuclide techniques are able to identify the nature of adrenal incidentalomas and to differentiate between hypersecreting and non-hypersecreting adenomas as well as between benign and malignant CTT.

  2. Excess Frequent Insufficient Sleep in American Indians/Alaska Natives

    Directory of Open Access Journals (Sweden)

    Daniel P. Chapman

    2013-01-01

    Full Text Available Objective. Frequent insufficient sleep, defined as ≥14 days/past 30 days in which an adult did not get enough rest or sleep, is associated with adverse mental and physical health outcomes. Little is known about the prevalence of frequent insufficient sleep among American Indians/Alaska Natives (AI/AN. Methods. We assessed racial/ethnic differences in the prevalence of frequent insufficient sleep from the combined 2009-2010 Behavioral Risk Factor Surveillance Survey among 810,168 respondents who self-identified as non-Hispanic white (NHW, , non-Hispanic black (NHB, , Hispanic (, or AI/AN (. Results. We found significantly higher unadjusted prevalences (95% CI of frequent insufficient sleep among AI/AN (34.2% [32.1–36.4] compared to NHW (27.4% [27.1–27.6]. However, the age-adjusted excess prevalence of frequent insufficient sleep in AI/AN compared to NHW was decreased but remained significant with the addition of sex, education, and employment status; this latter relationship was further attenuated by the separate additions of obesity and lifestyle indicators, but was no longer significant with the addition of frequent mental distress to the model (PR  =  1.05; 95% CI : 0.99–1.13. This is the first report of a high prevalence of frequent insufficient sleep among AI/AN. These results further suggest that investigation of sleep health interventions addressing frequent mental distress may benefit AI/AN populations.

  3. Cerebrovascular Reactivity in Complex Diagnostics of Vertebrobasilar Insufficiency

    Directory of Open Access Journals (Sweden)

    Goydenko V. S.

    2016-12-01

    Full Text Available The article presents the results of the assessment of cerebrovascular reactivity parameters due to transcranial Doppler sonography in 104 patients (62 women and 42 men with spondilogenic vertebrobasilar insufficiency. Mean age of patients was 42.9±12.2 years. It was found that the assessment of cerebrovascular reactivity and type of response to functional loading tests allows to confirm vertebrobasilar stage insufficiency, to determine the activity of various regulatory mechanisms of compensation of cerebral circulation and adaptation reserve of the cerebral vascular system.

  4. Stem cells in the development and differentiation of the human adrenal glands.

    Science.gov (United States)

    Terada, Tadashi

    2015-01-01

    There are no studies on stem cells (SCs) and development and differentiation (DD) of the human adrenal glands. The SCs in DD of the adrenal glands were herein investigated histochemically and immunohistochemically in 18 human embryonic adrenal glands at gestational week (GW) 7-40. At 7 GW, the adrenal glands were present, and at 7 GW, numerous embryonic SCs (ESCs) are seen to create the adrenal cortex. The ESCs were composed exclusively of small cells with hyperchromatic nuclei without nucleoli. The ESCs were positive for neural cell adhesion molecule, KIT, neuron-specific enolase, platelet-derived growth factor receptor-α, synaptophysin, and MET. They were negative for other SC antigens, including chromogranin, ErbB2, and bcl-2. They were also negative for lineage antigens, including cytokeratin (CK)7, CK8, CK18, and CK19, carcinoembryonic antigen, carbohydrate antigen 19-9, epithelial membrane antigen, HepPar1, mucin core apoprotein (MUC)1, MUC2, MUC5AC, and MUC6, and cluster differentiation (CD)3, CD45, CD20, CD34, and CD31. The Ki-67 labeling index (LI) was high (Ki-67 LI = around 20%). α-Fetoprotein was positive in the ESCs and adrenal cells. The ESC was first seen in the periphery of the adrenal cortex at 7-10 GW. The ESC migrates into the inner part of the adrenal cortex. Huge islands of ESC were present near the adrenal, and they appeared to provide the ESC of the adrenal. At 16 GW, adrenal medulla appeared, and the adrenal ESCs were present in the periphery or the cortex, in the cortical parenchyma, corticomedullary junctions, and in the medulla. The adrenal essential architecture was established around 20 GW; however, there were still ESCs. At term, there are a few ESCs. These data suggest that the adrenal glands were created by ESCs.

  5. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors.

    Science.gov (United States)

    Fassnacht, Martin; Arlt, Wiebke; Bancos, Irina; Dralle, Henning; Newell-Price, John; Sahdev, Anju; Tabarin, Antoine; Terzolo, Massimo; Tsagarakis, Stylianos; Dekkers, Olaf M

    2016-08-01

    : By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. We predefined four main clinical questions crucial for the management of adrenal incidentaloma patients, addressing these four with systematic literature searches: (A) How to assess risk of malignancy?; (B) How to define and manage low-level autonomous cortisol secretion, formerly called 'subclinical' Cushing's syndrome?; (C) Who should have surgical treatment and how should it be performed?; (D) What follow-up is indicated if the adrenal incidentaloma is not surgically removed? SELECTED RECOMMENDATIONS: (i) At the time of initial detection of an adrenal mass establishing whether the mass is benign or malignant is an important aim to avoid cumbersome and expensive follow-up imaging in those with benign disease. (ii) To exclude cortisol excess, a 1mg overnight dexamethasone suppression test should be performed (applying a cut-off value of serum cortisol ≤50nmol/L (1.8µg/dL)). (iii) For patients without clinical signs of overt Cushing's syndrome but serum cortisol levels post 1mg dexamethasone >138nmol/L (>5µg/dL), we propose the term 'autonomous cortisol secretion'. (iv) All patients with '(possible) autonomous cortisol' secretion should be screened for hypertension and type 2 diabetes mellitus, to ensure these are appropriately treated. (v) Surgical treatment should be considered in an individualized approach in patients with

  6. Nocturnal foot blood flow in patients with arterial insufficiency

    DEFF Research Database (Denmark)

    Jelnes, Rolf; Tønnesen, K H

    1984-01-01

    was on average the same in patients with normal circulations and in patients with different degrees of arterial insufficiency (mean: 2.0 +/- 0.8 ml min-1 100 g-1). During sleep the blood flow nearly doubled in patients with normal circulations; no systematic change was seen in patients with intermittent...

  7. Postoperative repeated respiratory insufficiency and thyrotoxicosis in molar pregnancy.

    Science.gov (United States)

    Cekic, B; Geze, S; Ulusoy, H; Coskun, I; Erturk, E

    2012-06-01

    Following the removal of a hydatiform mole in a 34-year-old, 14-week pregnant patient, thyrotoxicosis and respiratory insufficiency attacks were twice unexpectedly repeated. The symptoms were resolved with the administration of plasmapheresis, antithyroid and β-blocker drugs and non-invasive mechanical ventilation; however, she was again operated due to prolonged elevated β-hCG.

  8. Scintigraphic demonstration of lower extremity periostitis secondary to venous insufficiency

    Energy Technology Data Exchange (ETDEWEB)

    Gensburg, R.S.; Kawashima, A.; Sandler, C.M.

    1988-07-01

    The scintigraphic findings on bone imaging in two patients with extensive lower extremity periostitis secondary to venous insufficiency are presented. One of these patients had bilateral disease. The use of (/sup 67/Ga)citrate scanning in an attempt to exclude concurrent osteomyelitis is also addressed.

  9. Traumatic Tricuspid Insufficiency Requiring Valve Repair in an Acute Setting.

    Science.gov (United States)

    Enomoto, Yoshinori; Sudo, Yoshio; Sueta, Tomonori

    2015-01-01

    Tricuspid insufficiency due to penetrating cardiac trauma is rare. Patients with tricuspid insufficiency due to trauma can tolerate this abnormality for months or even years. We report a case of a 66-year-old female with penetrating cardiac trauma on the right side of her heart that required tricuspid valve repair in an acute setting. She sustained cut and stab wounds on her bilateral forearms and in the neck and epigastric region. She had cardiac tamponade and developed pulseless electrical activity, which required emergency surgery. The right ventricle and superior vena cava were dissected approximately 5 cm and 2 cm, respectively. After these wounds had been repaired, the patient's inability to wean from cardiopulmonary bypass suggested rightsided heart failure; transesophageal echocardiography revealed tricuspid insufficiency. Right atriotomy was performed, and a detailed examination revealed that the tricuspid valve septal leaflet was split in two. There was also an atrial septal injury that created a connection with the left atrium; these injuries were not detected from the right ventricular wound. After repair, weaning from cardiopulmonary bypass with mild tricuspid insufficiency was achieved, and she recovered uneventfully. This case emphasized the importance of thoroughly investigating intracardiac injury and transesophageal echocardiography.

  10. Cauda equina syndrome presentation of sacral insufficiency fractures

    Energy Technology Data Exchange (ETDEWEB)

    Muthukumar, T.; Butt, S.H.; Cassar-Pullicino, V.N.; McCall, I.W. [The Robert Jones and Agnes Hunt Orthopaedic Hospital, Department of Radiology, Oswestry, Shropshire (United Kingdom)

    2007-04-15

    Sacral insufficiency fractures are a well recognised cause for low back, buttock and groin pain in the elderly. However, over a 4 year period, four patients have presented with symptoms of cauda equina syndrome, who were found on investigation to have acute sacral insufficiency fracture without any other aetiological spinal abnormality. Four patients who presented to the spinal surgeons of our institution with symptoms of cauda equina syndrome were referred for spinal MR. Sagittal and axial T1 and T2 weighted turbo spin echo sequences of the lower thoracic and lumbar spine were performed on all patients. Subsequent studies included MR of the sacrum supplemented where appropriate by CT and technetium MDP bone scintigraphy. No evidence of a compressive lesion of the lower thoracic or lumbar spine was present in any of the four patients. Dedicated MR examination of the sacrum in these patients revealed unilateral acute insufficiency fractures involving zone 1 from S1 to S3 extending from the sacro-iliac joint to the lateral margin of the sacral foramen. There was no evidence of compression of the sacral nerve roots. The possible mechanism for the symptomatic presentation is discussed. Sacral insufficiency fractures should be excluded in elderly or osteoporotic patients presenting with cauda equina syndrome who have no evidence of compression in the thoraco-lumbar MR studies. (orig.)

  11. The clinical usefulness of NP-59 scintigraphy in adrenal cortical diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Duk Kyu [College of Medicine, Donga Univ., Pusan (Korea, Republic of)

    1997-03-01

    {sup 131}I-6-{beta}-iodomethyl-19-norcholesterol (NP-59) has an advantage to assess adrenal dysfunction caused by adrenal cortical disorders. The aim of this study is to evaluate the clinical usefulness of NP-59 scintigraphy in each adrenal disease. Ten patients who did eleven NP-59 adrenal scintigraphies at Dong-A University Hospital from March 1990 to December 1996 were selected as the subject. Among the subject there were 5 cases of Cushing's syndrome, 2 cases of incidentaloma, 1 case of metastatic adrenal tumor, liver cirrhosis with hirsutism and hypertension respectively. Among 5 cases of Cushing's syndrome, there were 2 cases of Cushing's disease, 2 cases of adrenal adenoma and 1 case of adrenal carcinoma. There are no disagreement between clinical diagnosis and scan finding in Cushing's syndrome. In 2 incidentaloma cases, even though one is interpretated as a functioning tumor, both of 2 cases could avoid unnecessary biopsy according to scintigraphy result. One case of hirsutism, clinically adrenal originated, revealed the normal scintigraphic finding after dexamethasone suppression scan. It could suggest that the etiology of hirsutism was extra-adrenal origin. One case of hypertension took the study to exclude the possibility of primary aldosteronism. Normal suppression scan finding revealed that primary aldosteronism did not exist in this case. In conclusion, NP-59 scintigraphy was very useful in diagnosis and differential diagnosis of Cushing's syndrome and it could avoid unnecessary biopsy in the incidental adrenal tumor.

  12. Repeated successful induction of fertility after replacing hydrocortisone with dexamethasone in a patient with congenital adrenal hyperplasia and testicular adrenal rest tumors.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Otten, B.J.; Sweep, C.G.J.; Hermus, A.R.M.M.

    2007-01-01

    OBJECTIVE: To report repeated successful induction of fertility in an adult male patient with congenital adrenal hyperplasia (CAH) and testicular adrenal rest tumors (TART). DESIGN: Case report. SETTING: Radboud University Nijmegen Medical Centre. PATIENT(S): A 23-year-old male CAH patient with bila

  13. Estimation of the Mechanism of Adrenal Action of Endocrine-Disrupting Compounds Using a Computational Model of Adrenal Steroidogenesis in NCI-H295R Cells

    Directory of Open Access Journals (Sweden)

    Ryuta Saito

    2016-01-01

    Full Text Available Adrenal toxicity is one of the major concerns in drug development. To quantitatively understand the effect of endocrine-active compounds on adrenal steroidogenesis and to assess the human adrenal toxicity of novel pharmaceutical drugs, we developed a mathematical model of steroidogenesis in human adrenocortical carcinoma NCI-H295R cells. The model includes cellular proliferation, intracellular cholesterol translocation, diffusional transport of steroids, and metabolic pathways of adrenal steroidogenesis, which serially involve steroidogenic proteins and enzymes such as StAR, CYP11A1, CYP17A1, HSD3B2, CYP21A2, CYP11B1, CYP11B2, HSD17B3, and CYP19A1. It was reconstructed in an experimental dynamics of cholesterol and 14 steroids from an in vitro steroidogenesis assay using NCI-H295R cells. Results of dynamic sensitivity analysis suggested that HSD3B2 plays the most important role in the metabolic balance of adrenal steroidogenesis. Based on differential metabolic profiling of 12 steroid hormones and 11 adrenal toxic compounds, we could estimate which steroidogenic enzymes were affected in this mathematical model. In terms of adrenal steroidogenic inhibitors, the predicted action sites were approximately matched to reported target enzymes. Thus, our computer-aided system based on systems biological approach may be useful to understand the mechanism of action of endocrine-active compounds and to assess the human adrenal toxicity of novel pharmaceutical drugs.

  14. Estimation of the Mechanism of Adrenal Action of Endocrine-Disrupting Compounds Using a Computational Model of Adrenal Steroidogenesis in NCI-H295R Cells.

    Science.gov (United States)

    Saito, Ryuta; Terasaki, Natsuko; Yamazaki, Makoto; Masutomi, Naoya; Tsutsui, Naohisa; Okamoto, Masahiro

    2016-01-01

    Adrenal toxicity is one of the major concerns in drug development. To quantitatively understand the effect of endocrine-active compounds on adrenal steroidogenesis and to assess the human adrenal toxicity of novel pharmaceutical drugs, we developed a mathematical model of steroidogenesis in human adrenocortical carcinoma NCI-H295R cells. The model includes cellular proliferation, intracellular cholesterol translocation, diffusional transport of steroids, and metabolic pathways of adrenal steroidogenesis, which serially involve steroidogenic proteins and enzymes such as StAR, CYP11A1, CYP17A1, HSD3B2, CYP21A2, CYP11B1, CYP11B2, HSD17B3, and CYP19A1. It was reconstructed in an experimental dynamics of cholesterol and 14 steroids from an in vitro steroidogenesis assay using NCI-H295R cells. Results of dynamic sensitivity analysis suggested that HSD3B2 plays the most important role in the metabolic balance of adrenal steroidogenesis. Based on differential metabolic profiling of 12 steroid hormones and 11 adrenal toxic compounds, we could estimate which steroidogenic enzymes were affected in this mathematical model. In terms of adrenal steroidogenic inhibitors, the predicted action sites were approximately matched to reported target enzymes. Thus, our computer-aided system based on systems biological approach may be useful to understand the mechanism of action of endocrine-active compounds and to assess the human adrenal toxicity of novel pharmaceutical drugs.

  15. Asymptomatic testicular adrenal rest tumours in adolescent and adult males with congenital adrenal hyperplasia: basal and follow-up investigation after 2.6 years.

    NARCIS (Netherlands)

    Stikkelbroeck, N.M.; Hermus, A.R.M.M.; Suliman, H.M.; Jager, G.J.; Otten, B.J.

    2004-01-01

    AIM: To study the course of asymptomatic testicular adrenal rest tumours in patients with congenital adrenal hyperplasia (CAH) and the association between tumour changes and glucocorticoid therapy adjustments. PATIENTS AND METHODS: Fifteen male patients with CAH (21-hydroxylase deficiency), in whom

  16. Image Registration of Cone-Beam Computer Tomography and Preprocedural Computer Tomography Aids in Localization of Adrenal Veins and Decreasing Radiation Dose in Adrenal Vein Sampling

    NARCIS (Netherlands)

    Busser, W.M.H.; Arntz, M.; Jenniskens, S.F.M.; Deinum, J.; Hoogeveen, Y.L.; Lange, F. de; Schultze Kool, L.J.

    2015-01-01

    We assessed whether image registration of cone-beam computed tomography (CT) (CBCT) and contrast-enhanced CT (CE-CT) images indicating the locations of the adrenal veins can aid in increasing the success rate of first-attempts adrenal vein sampling (AVS) and therefore decreasing patient radiation do

  17. Ultrasonographic adrenal gland findings in healthy semi-captive cheetahs (Acinonyx jubatus).

    Science.gov (United States)

    Kirberger, Robert M; Tordiffe, Adrian S W

    2016-05-01

    Cheetahs in captivity are believed to suffer from stress predisposing them to poor health. To date fecal glucocorticoids have been used as a non-invasive indicator of chronic stress. This study examines, the feasibility of transabdominal adrenal gland ultrasonography in cheetahs and determined normal adrenal measurements that can potentially be used as a more reliable indicator of chronic stress and/or adrenal function. Thirty-three adult cheetahs, aged between 2 and 13 years, accommodated in large off-display camps were examined over 9 days under general anesthesia. The adrenals were readily identified, with the right adrenal being more difficult to find and measure than the left, and were smaller than those expected in similar sized dogs. The left adrenal was shorter and slightly more oval shaped than the right with a length and cranial pole width at a 95% prediction interval of 16.3-22.4 and 4.1-8.7 mm. The same measurements for the right adrenal were 16.8-26.2 and 3.4-10.8 mm, respectively. Corticomedullary ratios were larger for the left adrenal. When corrected for body size, females had significantly longer and greater left adrenal corticomedullary ratios than males. Adrenal measurements did not correlate with left renal length, body size measurements, or enclosure size. Measurements that increased with age included the cortical and total adrenal widths. Adrenal ultrasonography offers potential benefits in assessment of individual cheetah adrenal pathology or the evaluation of stress induced adrenomegally especially in combination with other evaluations such as non-invasive fecal glucocorticoid analyses. Zoo Biol. 35:260-268, 2016. © 2016 Wiley Periodicals, Inc.

  18. Adrenal androgen hyperresponsiveness to adrenocorticotropin in women with acne and/or hirsutism: adrenal enzyme defects and exaggerated adrenarche.

    Science.gov (United States)

    Lucky, A W; Rosenfield, R L; McGuire, J; Rudy, S; Helke, J

    1986-05-01

    To determine the adrenal contribution to elevated plasma androgens in 31 young hyperandrogenemic women with acne and/or hirsutism, we compared their responses to ACTH with those of 14 normal women. Each subject was given a low dose (10 micrograms/m2) of synthetic ACTH-(1-24) (Cortrosyn) after administration of 1.5 mg dexamethasone the night before the test. Thirty and 60 min responses of plasma 17 alpha-hydroxypregnenolone (17-Preg), 17 alpha-hydroxyprogesterone, (17-prog), dehydroepiandrosterone (DHEA), androstenedione, 11-deoxycortisol, and cortisol were measured. Eighteen (58%) patients had increased responses of at least one 17-ketosteroid or adrenal androgen precursor. All patients had cortisol responses within the range of those of the 14 normal subjects. Nine patients (29%) had evidence of steroid biosynthetic enzyme deficiencies, either mild congenital adrenal hyperplasia or the heterozygote state; after ACTH, 4 of these patients had elevated 17-prog in the range of values in heterozygote carriers of 21-hydroxylase deficiency, 2 had elevated levels of 11-deoxycortisol compatible with 11 beta-hydroxylase deficiency, and 3 had elevated levels of 17-Preg and DHEA, suggestive of 3 beta-hydroxysteroid dehydrogenase deficiency. Another 9 subjects (29%) had 17-ketosteroid (DHEA and/or androstenedione) hyperresponsiveness to ACTH with associated elevated 17-Preg responses. As a group, their patterns suggested relatively deficient 3 beta-hydroxysteroid dehydrogenase and relatively hyperactive C lyase without impairment of cortisol secretion. This pattern resembles exaggerated adrenarche, and we postulate that these 9 patients have hyperplasia of the zona reticularis. Neither basal levels of plasma androgens (free testosterone and DHEA sulfate) nor menstrual history predicted which patients would have abnormal ACTH responses. Although 5 of 11 (45%) patients with acne alone had abnormal responses to ACTH, 10 of 14 patients with acne and hirsutism (71%) had abnormal

  19. Adrenal extramedullary hematopoiesis associated with β-thalassemia major

    Directory of Open Access Journals (Sweden)

    Bijan Keikhaei

    2012-01-01

    Full Text Available The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hema - topoiesis (EMH. EMH is a rare complication in thalassemia major (TM and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdominal computed tomography scan revealed a right well-defined suprarenal mass 7.7¥7.3¥5.8 cm in size. The diagnosis of EMH was confirmed with ultrasonographic-guided fine needle biopsy. Treatment options which include intensified regular blood transfusion and hydroxyurea have been started.

  20. Bone mineral status in children with congenital adrenal hyperplasia.

    Science.gov (United States)

    Fleischman, Amy; Ringelheim, Julie; Feldman, Henry A; Gordon, Catherine M

    2007-02-01

    Congenital adrenal hyperplasia (CAH) is caused by a deficiency in an adrenal enzyme resulting in alterations in cortisol and aldosterone production. Bone status is affected by chronic glucocorticoid therapy and excess androgen exposure in children with CAH. This cross-sectional study enrolled participants with 21-hydroxylase deficiency from a pediatric referral center. Bone mineral density in the participants was normal when compared to age, gender and ethnicity adjusted standards, with respect to chronological age or bone age. Lean body mass was positively correlated with bone mineral content (BMC), independent of fat mass (p bone status may be explained by the differential effects of glucocorticoids on growing bone, beneficial androgen effects, or other disease specific factors.

  1. Retroperitoneal extra-adrenal paraganglioma: a rare but important diagnosis.

    LENUS (Irish Health Repository)

    Ahmad, S

    2012-01-31

    BACKGROUND: Extra-adrenal paragangliomas of the retroperitonium are infrequently diagnosed. Their malignant behaviour cannot be predicted on initial clinical and histological assessment. These tumours have higher propensity for subsequent metastasis compared with pargangliomas at other sites. AIM: We aim to describe a case report of an incidental finding of left retroperitoneal paraganglioma in a young man who presented with right flank pain. We also aim to emphasize the importance of diagnosis and the malignant potential of these tumours. METHOD: Patient\\'s clinical notes, operative findings, imaging studies and laboratory investigations including histology results were reviewed. A literature search was done to look into the incidence, presentation, follow-up plan and treatment options for these tumours. CONCLUSION: Surgical resection is the only available curative option for extra-adrenal paragangliomas. Metastasis is observed years after surgery, hence long-term follow-up is required.

  2. Biochemical Mechanisms controlling Bioreactivity of Adrenal Chromaffin Cells

    Science.gov (United States)

    1988-06-17

    conditioning in the laboratory rat . B. Examination of the morphological changes that accompany changes in bioreactivity of the rat adrenal medulla: When... Laboratory Rat . Proc. Western Pharmacol. Soc. 29: 315-318, 1986. Campbell, D. C., Hardie, D. G. and Vulliet, P. R. Identification of four Phosphorylation...Robert Lennox (New York, Plenum Press, 1987) pp 367-374. Vulliet, P. R., Loskutoff, N. and Kraemer, D. A Technique of Embryo Transfer in the

  3. Biosynthesis and metabolism of steroid hormones by human adrenal carcinomas

    Directory of Open Access Journals (Sweden)

    Brown J.W.

    2000-01-01

    Full Text Available Over a 15-year period, our university-based laboratory obtained 125 adrenal tumors, of which 15 (12% were adrenal cortical carcinomas. Of these, 6 (40% of the carcinomas occurred in patients with clear clinical manifestations of steroid hormone excess. Adrenal cortical carcinoma cells derived from the surgically resected tumors in 4 of these patients were isolated and established in primary culture. Radiotracer steroid interconversion studies were carried out with these cultures and also on mitochondria isolated from homogenized tissues. Large tumors had the lowest steroidogenic activities per weight, whereas small tumors had more moderately depressed enzyme activities relative to cells from normal glands. In incubations with pregnenolone as substrate, 1 mM metyrapone blocked the synthesis of corticosterone and cortisol and also the formation of aldosterone. Metyrapone inhibition was associated with a concomitant increase in the formation of androgens (androstenedione and testosterone from pregnenolone. Administration of metyrapone in vivo before surgery in one patient resulted in a similar increase in plasma androstenedione, though plasma testosterone levels were not significantly affected. In cultures of two of four tumors examined, dibutyryl cAMP stimulated 11ß-hydroxylase activity modestly; ACTH also had a significant stimulatory effect in one of these tumors. Unlike results obtained with normal or adenomatous adrenal cortical tissues, mitochondria from carcinomatous cells showed a lack of support of either cholesterol side-chain cleavage enzyme complex or steroid 11ß-hydroxylase activity by Krebs cycle intermediates (10 mM isocitrate, succinate or malate. This finding is consistent with the concept that these carcinomas may tend to function predominantly in an anaerobic manner, rather than through the oxidation of Krebs cycle intermediates.

  4. Cisplatin-induced peptic ulcers, vagotomy, adrenal and calcium modulation.

    Science.gov (United States)

    Aggarwal, S K; San Antonio, J D; Sokhansanj, A; Miller, C

    1994-04-01

    Cytochemical and autoradiographic studies in Wistar rats [Crl:(WI)BR] show that cisplatin treatment (9 mg/kg) inhibits the release of acetylcholine from the axonal endings of the stomach smooth muscle resulting in bloating of the stomach and ulceration. Cisplatin also induces corticosteroid release from the adrenal gland stimulating peptic ulceration. Vagotomy helps ameliorate the effect but not eliminate it. Calcium supplementation restores normal neuromuscular function to gastric smooth muscle, thereby eliminating the gastro-intestinal toxicity due to cisplatin.

  5. Refractory hypoglycemia in a patient with functional adrenal cortical carcinoma

    Directory of Open Access Journals (Sweden)

    Katia Regina Marchetti

    2016-11-01

    Full Text Available Adrenacarcinomas are rare, and hypoglycemic syndrome resulting from the secretion of insulin-like growth factor II (IGF-II by these tumors have been described infrequently. This study describes the case of a young woman with severe persistent hypoglycemia and a large adrenal tumor and discusses the physiopathological mechanisms involved in hypoglycemia. The case is described as a 21-year-old woman who presented with 8 months of general symptoms and, in the preceding 3 months, with episodes of mental confusion and visual blurring secondary to hypoglycemia. A functional assessment of the adrenal cortex revealed ACTH-independent hypercortisolism and hyperandrogenism. Hypoglycemia, hypoinsulinemia, low C-peptide and no ketones were also detected. An evaluation of the GH–IGF axis revealed GH blockade (0.03; reference: up to 4.4 ng/mL, greatly reduced IGF-I levels (9.0 ng/mL; reference: 180–780 ng/mL, slightly reduced IGF-II levels (197 ng/mL; reference: 267–616 ng/mL and an elevated IGF-II/IGF-I ratio (21.9; reference: ~3. CT scan revealed a large expansive mass in the right adrenal gland and pulmonary and liver metastases. During hospitalization, the patient experienced frequent difficult-to-control hypoglycemia and hypokalemia episodes. Octreotide was ineffective in controlling hypoglycemia. Due to unresectability, chemotherapy was tried, but after 3 months, the patient’s condition worsened and progressed to death. In conclusion, our patient presented with a functional adrenal cortical carcinoma, with hyperandrogenism associated with hypoinsulinemic hypoglycemia and blockage of the GH–IGF-I axis. Patient’s data suggested a diagnosis of hypoglycemia induced by an IGF-II or a large IGF-II-producing tumor (low levels of GH, greatly decreased IGF-I, slightly decreased IGF-II and an elevated IGF-II/IGF-I ratio.

  6. Arteries of the adrenal glands in ostriches (Struthio camelus

    Directory of Open Access Journals (Sweden)

    Angelita das Graças de Oliveira Honorato

    2012-03-01

    Full Text Available The growth of rational ostrich breeding and their byproducts has attracted interest from researchers to increase the studies in this animal. Thus, basic research areas, such as morphology, become necessary to provide the applied areas with knowledge. Aiming to contribute to the knowledge on the vascular arrangements of the adrenal glands, 30 ostriches (Struthio camelus were used, four days old, who had their arterial components marked with a 50% stained aqueous solution of Neoprene Latex ¨ 450 ¨ and fixed in a 10% diluted solution of formaldehyde. The coelomic cavity was exposed for identifying these glands, which are paired organs that are covered by loose connective tissue, symmetrically arranged in the two antimeres, laterally to the descending aorta, caudally to the lungs, and cranio-medially to the cranial lobes of the kidneys. The arterial blood supply, in both antimeres, is derived from the right and left adrenal arteries, the right and left cranial renal artery branches, and the right branches of the descending aorta. Regardless of the origin, the number of branches going to the adrenal glands ranged from one to two and one to three respectively, in the left and right antimeres.

  7. PET studies of parkinsonian patients treated with autologous adrenal implants.

    Science.gov (United States)

    Guttman, M; Burns, R S; Martin, W R; Peppard, R F; Adam, M J; Ruth, T J; Allen, G; Parker, R A; Tulipan, N B; Calne, D B

    1989-08-01

    Transplantation of autologous adrenal medulla tissue into the striatum has recently been proposed as a treatment for Parkinson's disease. We report the use of positron emission tomography (PET) to evaluate patients who had adrenal implants placed into the right caudate. 6-[18F] fluoro-L-dopa (6-FD) scans were performed to study the integrity and activity of the implant, and the nigrostriatal dopamine system before and six weeks after transplantation surgery. [68Ga] Gallium-ethylenediaminetetraacetate (Ga) scans were also performed to assess the blood brain barrier. The Ga scans performed on two patients showed increased permeability of the blood brain barrier at the surgical site. 6-FD PET scans in five patients did not show a consistent change in striatal uptake following adrenal medullary implantation after six weeks. Further assessment of implant viability with 6-FD PET scans after longer follow up may provide useful information if the blood-brain barrier becomes re-established with the passage of time.

  8. Testosterone-secreting adrenal adenoma in a peripubertal girl

    Energy Technology Data Exchange (ETDEWEB)

    Kamilaris, T.C.; DeBold, C.R.; Manolas, K.J.; Hoursanidis, A.; Panageas, S.; Yiannatos, J.

    1987-11-13

    A 15-year-old girl who presented with primary amenorrhea and virilization had an adrenocortical adenoma that secreted predominantly testosterone. To the authors' knowledge, she is the first peripubertal and second youngest patient with a testosterone-secreting adrenal tumor described. Serum dehydroepiandrosterone sulfate and urinary 17-ketosteroid an 17-hydroxycorticosteroid levels were normal. A tumor was located by a computed tomographic (CT) scan and by uptake of 6-..beta..-(/sup 75/Se) selenomethylnorcholesterol. Microscopic examination of the tumor showed typical features of an adrenocortical adenoma with no histologic features characteristic of Leydig cells. Postoperatively, her hirsutism regressed, she rapidly went through puberty, and regular monthly menstruation started four months later. Finding the source of testosterone in a virilized patient can be difficult. Eleven of the 14 previously described patients with testosterone-secreting adrenal tumors initially underwent misdirected surgery on the ovaries. Review of these cases revealed that results of hormone stimulation and suppression tests are unreliable and that these tumors are usually large. Therefore, CT scanning of the adrenal glands is recommended in all patients suspected of having a testosterone-secreting tumor.

  9. Adrenal Pathology in the Adult: A Urological Pathologist's Perspective.

    Science.gov (United States)

    Hansel, Donna E; Reuter, Victor E

    2016-09-01

    Adrenal gland diagnostics can pose significant challenges. In most academic and community practice settings, adrenal gland resections are encountered less frequently than other endocrine or genitourinary specimens, leading to less familiarity with evolving classifications and criteria. The unique dichotomy between cortical and medullary lesions reflects the developmental evolution of these functionally independent components. Adrenal cortical lesions at resection include hyperplasia, adenoma, and carcinoma, with some cases straddling the boundary between these distinct clinical classifications. The lack of immunohistochemical or molecular markers to definitively categorize these intermediate lesions enhances the diagnostic challenge. In addition, modified terminology for oncocytic and myxoid cortical lesions has been proposed. Medullary lesions are somewhat easier to categorize; however, the prediction of aggressive behavior in pheochromocytomas remains a challenge due to a lack of reliable prognostic biomarkers. Recent work by the Cancer Genome Atlas Project and other research groups has identified a limited subset of molecular and signaling pathway alterations in these 2 major neoplastic categories. Ongoing research to better define prognostic and predictive biomarkers in cortical and medullary lesions has the potential to enhance both pathologic diagnosis and patient therapy.

  10. Stereotactic Radiotherapy for Adrenal Gland Metastases: University of Florence Experience

    Energy Technology Data Exchange (ETDEWEB)

    Casamassima, Franco, E-mail: f.casamassima@dfc.unifi.it [Clinical Radiobiological Institute, University of Florence, Florence (Italy); Livi, Lorenzo [Department of Radiation-Oncology, University of Florence, Florence (Italy); Masciullo, Stefano; Menichelli, Claudia; Masi, Laura [Clinical Radiobiological Institute, University of Florence, Florence (Italy); Meattini, Icro [Department of Radiation-Oncology, University of Florence, Florence (Italy); Bonucci, Ivano [Clinical Radiobiological Institute, University of Florence, Florence (Italy); Agresti, Benedetta; Simontacchi, Gabriele [Department of Radiation-Oncology, University of Florence, Florence (Italy); Doro, Raffaela [Clinical Radiobiological Institute, University of Florence, Florence (Italy)

    2012-02-01

    Purpose: To evaluate a retrospective single-institution outcome after hypofractionated stereotactic body radiotherapy (SBRT) for adrenal metastases. Methods and Materials: Between February 2002 and December 2009, we treated 48 patients with SBRT for adrenal metastases. The median age of the patient population was 62.7 years (range, 43-77 years). In the majority of patients, the prescription dose was 36 Gy in 3 fractions (70% isodose, 17.14 Gy per fraction at the isocenter). Eight patients were treated with single-fraction stereotactic radiosurgery and forty patients with multi-fraction stereotactic radiotherapy. Results: Overall, the series of patients was followed up for a median of 16.2 months (range, 3-63 months). At the time of analysis, 20 patients were alive and 28 patients were dead. The 1- and 2-year actuarial overall survival rates were 39.7% and 14.5%, respectively. We recorded 48 distant failures and 2 local failures, with a median interval to local failure of 4.9 months. The actuarial 1-year disease control rate was 9%; the actuarial 1- and 2-year local control rate was 90%. Conclusion: Our retrospective study indicated that SBRT for the treatment of adrenal metastases represents a safe and effective option with a control rate of 90% at 2 years.

  11. Feminizing Adrenal Carcinoma Presenting with Heart Failure and Ventricular Tachycardia

    Directory of Open Access Journals (Sweden)

    Anjana Harnoor

    2012-01-01

    Full Text Available We present a case of feminizing adrenal carcinoma with severe elevation in serum estradiol and otherwise unexplained congestive heart failure with ventricular arrhythmia and review the literature on feminizing adrenal tumors and the potential relationship between estrogen and cardiac problems. A 54-year-old man presented with congestive heart failure and ventricular arrhythmia. Imaging revealed a large adrenal mass. Hormonal evaluation revealed a very high serum level of estradiol, elevated DHEA-sulfate and androstenedione, and lack of cortisol suppression on a low-dose overnight dexamethasone suppression test. The patient underwent a left adrenalectomy with subsequent normalization of serum estradiol. Surgical pathology examination established adrenocortical carcinoma MacFarlane stage II. Upon 15-month followup, the patient continued to have a normal serum estradiol level, his cardiac function was significantly improved, and he had no further episodes of ventricular arrhythmia. To the best of our knowledge, the serum estradiol level that was detected in our case is the highest that has been reported. Further, we hypothesize that the very high serum concentration of estradiol in our case may have played a role in his cardiac presentation with congestive heart failure and arrhythmia, particularly as these problems resolved with normalization of his serum estradiol level.

  12. Adrenal adenocarcinoma with Kartagener's syndrome: A case report

    Science.gov (United States)

    HU, WANLI; CHENG, LONG; CHENG, BEI; ZHANG, PENG; XIAO, HE; WU, WENBO; WANG, XINGHUAN

    2015-01-01

    The present study reports the case of a 44-year-old woman with an adrenal tumor, complicated by Kartagener's syndrome (KS). The patient was admitted to Zhongnan Hospital (Wuhan, China), and presented with an 8-week history of vertigo and extended history of a recurrent cough, accompanied by sputum and a congested nose. Computed tomography indicated a mass on the right adrenal gland and situs inversus. A right adrenal tumor combined with KS was diagnosed, and resection of the tumor was performed following relief of respiratory symptoms and control of blood pressure. During six months of follow up the patient recovered well from surgery and blood pressure remained stable. This case revealed that patients exhibiting KS may suffer from serious respiratory infections as a result of impaired defense mechanisms against microbes in the airway. Therefore, comprehensive management of infection, safe anesthesia and appropriate surgical procedures for the avoidance of inflammation and trauma are the most significant factors required for the success of the treatment. PMID:26788182

  13. Primary Epithelioid Angiosarcoma of the Adrenal Gland: Report of a Case and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Malek Mohamed Ayadi

    2016-09-01

    Full Text Available Primary mesenchymal neoplasms of the adrenal gland are extremely rare. The most common primary sarcoma is adrenal angiosarcoma. We report the case of a 51-year-old patient who presented with left flank pain. After ultrasound investigations, the patient underwent surgical removal of an adrenal tumor. The gross adrenal specimen showed extensive cystic changes with old hemorrhage and necrosis. Histologically, irregular branching vascular channels were seen intermixed with solid areas of epithelioid cells. Immunohistochemical staining of tumor cells was positive with antibodies to cytokeratin and CD31. After a 12 months follow-up, the patient is still well with no sign of a relapse. Because of the epithelioid appearance and frequent expression of epithelial immunohistochemical markers, primary epithelioid angiosarcoma of the adrenal gland can be confounded with adrenal carcinomatous metastases. We report our findings with a brief literature review and discussion of differential diagnosis. [J Interdiscipl Histopathol 2016; 4(3.000: 67-69

  14. Prenatal exposure to bisphenol A disrupts adrenal steroidogenesis in adult mouse offspring.

    Science.gov (United States)

    Medwid, Samantha; Guan, Haiyan; Yang, Kaiping

    2016-04-01

    The present study sought to determine if prenatal exposure to bisphenol A (BPA) alters adrenal steroidogenesis in adult offspring. Pregnant mice were exposed to BPA (25mg BPA/kg food pellet) via diet from day 7 to the end of pregnancy. At eight weeks of age, offsprings were sacrificed, blood samples and adrenal glands were collected for hormone assays and western blot analysis, respectively. We found that: (1) BPA increased adrenal gland weight in both males and females; (2) although BPA elevated plasma corticosterone levels in both sexes, it stimulated the expression of StAR and cyp11A1, the two rate-limiting factors in the steroidogenic pathway, only in female adrenal glands; and interestingly (3) BPA did not alter plasma ACTH levels or adrenal expression of the key steroidogenic transcription factor SF-1 in either sex. Taken together, the present study provides novel insights into the long-term consequences of developmental BPA exposure on adrenal steroidogenesis.

  15. A new pathological scoring method for adrenal injury in rats with severe acute pancreatitis.

    Science.gov (United States)

    Deng, Wenhong; Hui, Yuanjian; Yu, Jia; Wang, Weixing; Xu, Sheng; Chen, Chen; Xiong, Xincheng

    2014-12-01

    These studies investigated the appearance and function of adrenal glands in rats with severe acute pancreatitis (SAP) and established a new histopathological score to evaluate adrenal histopathological changes. Severe acute pancreatitis relied on retrograde infusion of 5% sodium taurocholate into the bile-pancreatic duct. The damage of SAP was estimated by serum amylase, secretory phospholipase A2 and pancreatic histopathology. Light and electron microscopy of adrenal gland, and the levels of serum corticosterone were investigated. These results showed that the generally ascending trend of adrenal pathological score was inversely proportional to the generally descending trend of serum corticosterone levels, but parallel with the changes of pancreatic histopathology. Herein, the new adrenal histopathological score was effective in the evaluation of adrenal injury following SAP. It may indirectly reflect the variation of serum cortisol levels and the severity of pancreatitis to a certain extent.

  16. Subchondral insufficiency fracture of the femoral head after liver transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Iwasaki, Kenyu; Yamamoto, Takuaki; Nakashima, Yasuharu; Mawatari, Taro; Motomura, Goro; Ikemura, Satoshi; Iwamoto, Yukihide [Kyushu University, Department of Orthopaedic Surgery, Fukuoka (Japan)

    2009-09-15

    A 53-year-old woman developed a subchondral insufficiency fracture of the right femoral head after undergoing a liver transplantation. Radiographs obtained at her first visit demonstrated a slight subchondral collapse in the superolateral portion of the femoral head. Magnetic resonance imaging (MRI) disclosed an irregular, discontinuous, low-intensity band on the T1-weighted image. After 7 months of conservative treatment, the hip pain and the radiograph abnormalities had both disappeared. On the follow-up T1-weighted MR image obtained 17 months after the onset, the band of low signal intensity was not obvious. A subchondral insufficiency fracture is one of the diagnoses to be considered in patients presenting with hip pain after a liver transplantation. (orig.)

  17. [Irreversible coma following hypoglycemia in Sheehan syndrome with adrenocortical insufficiency].

    Science.gov (United States)

    Sas, A M; Meynaar, I A; Laven, J S; Bakker, S L; Feelders, R A

    2003-08-23

    A 24-year-old woman of Somali origin delivered at term after an uncomplicated pregnancy. Post-partum haemorrhage resulted in hypovolaemic shock which was treated by hysterectomy. Five days later she became comatose due to unrecognised hypoglycaemia which caused severe irreversible brain damage and status epilepticus. Treatment in the intensive care unit with artificial respiration, prednisolone, desmopressin, inotropic support, barbiturates and an anaesthetic under EEG guidance was unsuccessful. The patient died 28 days post-partum. The hypoglycaemia was due to a combination of (a) inadequate glucose intake and (b) lack of counter-regulatory mechanisms due to a deficiency of steroids and growth hormone as a result of loss of pituitary function (Sheehan syndrome) together with adrenocortical insufficiency. The combination of Sheehan syndrome and primary adrenocortical insufficiency has not been described previously in the literature.

  18. Treatment of insufficient lactation is often not evidence-based

    DEFF Research Database (Denmark)

    Axelsson, Paul Bryde; Bjerrum, Flemming; Løkkegaard, Ellen Christine Leth

    2014-01-01

    INTRODUCTION: Breast milk has many advantages over formula for infants in developed and developing countries alike. Despite intentions of breastfeeding, some women develop insufficient lactation. Treatment options traditionally include breastfeeding education and pharmacotherapy. MATERIAL...... AND METHODS: An electronic questionnaire regarding treatment of insufficient lactation was sent to all obstetric departments (n = 21) and neonatal wards (n = 17) in Denmark. Three main questions were included which focused on: breastfeeding education for women, use of pharmacotherapy and availability of local...... guidelines. RESULTS: In all, 30 out of a total of 38 departments participated; and among those, 93% offered some form of breastfeeding education. 50% used either metoclopramide or syntocinon to promote lactation. None used domperidone. 73% had a local clinical guideline. 77% offered sessions with a lactation...

  19. [Palmitoyl-CoA-dehydrogenase from rabbit adrenals, liver and myocardium].

    Science.gov (United States)

    Doroshkevich, N A; Mandrik, K A; Vinogradov, V V

    1988-01-01

    Partially purified preparations of palmitoyl-CoA dehydrogenase from rabbit adrenal glands, liver and heart tissues exhibited similar kinetic parameters. Km value constituted 6.58, 5.26 and 6.67 microM for the enzyme from adrenal glands, liver and heart tissues, respectively. At the same time, palmitoyl-CoA dehydrogenase possessed lower catalytic capacity in adrenal glands due to the decreased amount of the enzyme as compared with that of liver or heart tissues.

  20. Metachronous Bilateral Isolated Adrenal Metastasis from Rectal Adenocarcinoma: A Case Report

    Science.gov (United States)

    Jabir, H.; Tawfiq, N.; Moukhlissi, M.; Akssim, M.; Guensi, A.; Kadiri, B.; Bouchbika, Z.; Taleb, A.; Benchekroun, N.; Jouhadi, H.; Sahraoui, S.; Zamiati, S.; Benider, A.

    2014-01-01

    We report a case of adrenal metastasis from colorectal cancer in a 54-year-old woman. Nine months after resection for advanced rectal carcinoma, a computed tomography scan revealed bilateral adrenal metastasis. The level of serum carcinoembryonic antigen was normal. A bilateral adrenalectomy was performed after chemotherapy. Histopathological examination showed adenocarcinoma, compatible with metastasis from the rectal cancer. Adrenal metastasis should be considered in the patients' follow-up for colorectal cancer. PMID:24860684

  1. Metachronous bilateral isolated adrenal metastasis from rectal adenocarcinoma: a case report.

    Science.gov (United States)

    Jabir, H; Tawfiq, N; Moukhlissi, M; Akssim, M; Guensi, A; Kadiri, B; Bouchbika, Z; Taleb, A; Benchekroun, N; Jouhadi, H; Sahraoui, S; Zamiati, S; Benider, A

    2014-01-01

    We report a case of adrenal metastasis from colorectal cancer in a 54-year-old woman. Nine months after resection for advanced rectal carcinoma, a computed tomography scan revealed bilateral adrenal metastasis. The level of serum carcinoembryonic antigen was normal. A bilateral adrenalectomy was performed after chemotherapy. Histopathological examination showed adenocarcinoma, compatible with metastasis from the rectal cancer. Adrenal metastasis should be considered in the patients' follow-up for colorectal cancer.

  2. CT-guided fine needle aspiration cytology diagnosis of extra-adrenal pheochromocytoma

    OpenAIRE

    Rangaswamy M; Kumar Sandeep; Asha M; Manjunath G

    2010-01-01

    Pheochromocytoma is a rare tumor, accounting for <0.1% of the hypertensive population. Extra-adrenal pheochromocytomas (EAPs) are rarer still, accounting for 10% of all pheochromocytomas. Pheochromocytomas are functional catecholamine-secreting tumors of the paraganglionic chromaffin cells found in the adrenal medulla and the extra-adrenal paraganglia cells. EAPs are readily detected by computed tomography (CT) as soft tissue masses closely associated with the entire length o...

  3. Diffusion MRI features of acute encephalopathy due to stopping steroid medication abruptly in congenital adrenal hyperplasia

    OpenAIRE

    Asli Serter; Alpay Alkan; Demet Demirkol

    2015-01-01

    Diffusion MRI features of acute encephalopathy due to stopping steroid medication abruptly in congenital adrenal hyperplasia Asli Serter, Alpay Alkan and Demet Demirkol Annals of Indian Academy of Neurology. 18.3 (July-September 2015): p342. Copyright: COPYRIGHT 2015 Medknow Publications and Media Pvt. Ltd. http://www.annalsofian.org/ Full Text: Byline: Asli. Serter, Alpay. Alkan, Demet. Demirkol Congenital adrenal hyperplasia (CAH) is characterized by adrenal steroid biosynthesis defect. Ste...

  4. Metachronous Bilateral Isolated Adrenal Metastasis from Rectal Adenocarcinoma: A Case Report

    Directory of Open Access Journals (Sweden)

    H. Jabir

    2014-01-01

    Full Text Available We report a case of adrenal metastasis from colorectal cancer in a 54-year-old woman. Nine months after resection for advanced rectal carcinoma, a computed tomography scan revealed bilateral adrenal metastasis. The level of serum carcinoembryonic antigen was normal. A bilateral adrenalectomy was performed after chemotherapy. Histopathological examination showed adenocarcinoma, compatible with metastasis from the rectal cancer. Adrenal metastasis should be considered in the patients’ follow-up for colorectal cancer.

  5. Subtrochanteric Femoral Insufficiency Fracture Following Bisphosphonate Therapy for Osseous Metastases

    OpenAIRE

    Bush, Lisabeth A.; Chew, Felix S.

    2015-01-01

    We present the case of an insufficiency fracture of the femoral shaft in a 61-year-old man who had received bisphosphonate therapy to reduce the fracture risk from lytic renal cell carcinoma metastases to the spine. Approximately 1.5 years after beginning monthly intravenous infusions of zoledronic acid (Zometa), the patient complained of persistent thigh pain. Radionuclide bone scan showed mildly increased activity in the lateral subtrochanteric cortex of the right femur, where there was foc...

  6. Vitamin D insufficiency in Greenlanders on a westernized fare

    DEFF Research Database (Denmark)

    Rejnmark, Lars; Jorgensen, M.E.; Pedersen, M.B.;

    2004-01-01

    fare (group A), 45 Greenlanders living in Nuuk on a westernized fare (group B), 54 Greenlanders (group C), and 43 Danes (Group D) living in Denmark (55 degrees N) on a westernized fare. Blood specimens were drawn both summer and winter. Vitamin D insufficiency (plasma 25 OHD ... of vitamin D status. Changes from a traditional to a westernized fare are associated with a reduced vitamin D status in Greenlanders. Vitamin D supplementation should be considered....

  7. [Fetal circulation in normal pregnancy and in placental insufficiency].

    Science.gov (United States)

    Ivanov, B; Malinova, M

    2010-01-01

    The fetal circulation is different from the adult circulation. One of the quite common conditions that are challenging to the developing fetus is placental hypoxia. Regardless of its cause, placental vascular insufficiency is commonly assumed to be an important factor in the development of intrauterine growth retardation. Several mechanisms are involved in the fetal adaptation to the decompensation during hypoxemia. Doppler Ultrasound technologies can help to evaluate of the fetal wellbeing.

  8. Insufficient Milk Supply and Breast Cancer Risk: A Systematic Review

    OpenAIRE

    Jacqueline M Cohen; Hutcheon, Jennifer A; Julien, Sofi G.; Tremblay, Michel L.; Rebecca Fuhrer

    2009-01-01

    BACKGROUND: An association between insufficient milk supply, the inability of a mother's breast milk to provide sufficiently for her infant, and breast cancer has been suggested by observations in animal models. To determine if an association has been reported in epidemiological studies of human breast cancer, a systematic review of the literature has been conducted. We also sought to identify the methodological limitations of existing studies to guide the design of any future prospective stu...

  9. Insufficient Evidence of Purported Lunar Effect on Pollination in Ephedra.

    Science.gov (United States)

    Margot, Jean-Luc

    2015-10-01

    It has been suggested that the timing of pollination in Ephedra foeminea coincides with the full moon in July. The implication is that the plant can detect the full moon through light or gravity and that this trait is an evolutionary adaptation that aids the navigation by pollinating insects. Here we show that there are insufficient data to make such a claim, and we predict that pollinations of E. foeminea do not in general coincide with the full moon.

  10. Chronic cerebrospinal venous insufficiency and venous stenoses in multiple sclerosis

    DEFF Research Database (Denmark)

    Blinkenberg, M; Akeson, P; Sillesen, H;

    2012-01-01

    The traditional view that multiple sclerosis (MS) is an autoimmune disease has recently been challenged by the claim that MS is caused by chronic cerebrospinal venous insufficiency (CCSVI). Although several studies have questioned this vascular theory, the CCSVI controversy is still ongoing. Our...... aim was to assess the prevalence of CCSVI in Danish MS patients using sonography and compare these findings with MRI measures of venous flow and morphology....

  11. Adolescent Premature Ovarian Insufficiency Following Human Papillomavirus Vaccination

    OpenAIRE

    Deirdre Therese Little MBBS, DRANZCOG, FACRRM; Harvey Rodrick Grenville Ward Bsc(Med), MBChB, DMCOG, FCOG(SA), MMed (O&G), FRANZCOG

    2014-01-01

    Three young women who developed premature ovarian insufficiency following quadrivalent human papillomavirus (HPV) vaccination presented to a general practitioner in rural New South Wales, Australia. The unrelated girls were aged 16, 16, and 18 years at diagnosis. Each had received HPV vaccinations prior to the onset of ovarian decline. Vaccinations had been administered in different regions of the state of New South Wales and the 3 girls lived in different towns in that state. Each had been p...

  12. Outcome of surgical treatment of post-traumatic tricuspid insufficiency

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Objective: To investigate the optimal time and procedure of surgical treatment of traumatic tricuspid insufficiency.Methods: From May 1984 to September 2004, eight patients underwent operation for traumatic tricuspid valve insufficiency. All patients, male, aged from 7 to 67 years [median: 38 years, mean: (38.5±18.1) years]. The intervals between trauma and operation ranged from 1 month to 20 years [ median: 19 months, mean: ( 52.5±80.3 ) months) ]. In seven patients, tricuspid insufficiency was attributed to blunt chest trauma including vehicle accident in three patients and the other patient is a stab wound. Diagnosis was confirmed by echocardiography. Pre-operative cardiac functions in patients were classified as New York Heart Association (NYHA) classes Ⅱ-Ⅳ.During operation, the anterior leaflet of the tricuspid valve was completely or partially flailed as a result of chordal rupture in all patients. Chordal rupture of septal leaflet was found in one patient. Anterior leaflet was perforated in two patients. Septal leaflet was retracted and adherent to ventricular septum in two patients. Valve repair was intended for all patients. Finally, valve repair was performed successfully in 3 patients and tricuspid replacement was performed in 5 patients.Results: No early or late death occurred. With a follow-up through clinical manifestation and echocardiography for 7-129 months [ median: 39 months, mean: (53.4 ± 42.8) months ], all patients were classified as NYHA class I, without any changes.Conclusions: The satisfactory treatment of traumatic tricuspid insufficiency can be obtained by surgical treatment. Earlier surgery may increase the feasibility of tricuspid valve repair and prevent the deterioration of right ventricular function.

  13. Iatrogenic velopharyngeal insufficiency caused by neonatal nasogastric feeding tube.

    Science.gov (United States)

    Pollack, Aron Z; Ward, Robert F; DeRowe, Ari; April, Max M

    2014-08-01

    Complications from a prolonged nasogastric tube intubation, though seldom reported, are well described. Herein we describe the first two reported cases of velopharyngeal insufficiency secondary to velopharyngeal scarring and immobility from repetitive nasogastric tube insertions and prolonged use. Differing only in location, the proposed pathophysiologic mechanism of injury is identical to that of the nasogastric tube syndrome, a rare and serious, well described entity consisting of bilateral vocal fold paralysis due to pressure-induced ulceration of the posterior cricoarytenoid musculature.

  14. A Solved Model to Show Insufficiency of Quantitative Adiabatic Condition

    Institute of Scientific and Technical Information of China (English)

    LIU Long-Jiang; LIU Yu-Zhen; TONG Dian-Min

    2009-01-01

    The adiabatic theorem is a useful tool in processing quantum systems slowly evolving,but its practical application depends on the quantitative condition expressed by Hamiltonian's eigenvalues and eigenstates,which is usually taken as a sufficient condition.Recently,the sumciency of the condition was questioned,and several counterex amples have been reported.Here we present a new solved model to show the insufficiency of the traditional quantitative adiabatic condition.

  15. Prevalence of renal insufficiency in individuals with obesity

    OpenAIRE

    Vasiljević Nađa; Zuković Milica; Žakula Dejan; Živadinović Nenad; Žarković Boris; Živković Bojana; Zlatić Nataša; Zogović Jovana; Ležaić Višnja

    2011-01-01

    Introduction. The association of obesity with renal function disorders has been demonstrated by some studies. Objective. The aim of this study was to assess the prevalence of renal insufficiency (RI), measured as glomerular filtration rate (GFR) <60 ml/min and impact of weight loss on kidney function in a cohort of 109 obese patients (body mass index - BMI >25 kg/m2), without previous kidney disease, and who underwent the dietician’s treatment. Methods. According to body mass inde...

  16. FEQ for Standard OFDM Systems with Insufficient CP

    Institute of Scientific and Technical Information of China (English)

    CHENShaoping; YAOTianren

    2004-01-01

    In Orthogonai frequency division multiplexing (OFDM) systems, when the length of the Cyclic prefix (CP) is shorter than the channel length, there exist Intersymbol interference (ISI) and Interchannel interference (ICI) and the one-tap frequency domain equalizer is not applicable any more. A Time-domain equalizer (TEQ) is usually used in the receiver to reduce the duration of the overall response of the transmission system, and therefore minimize the ISI and ICI. However, the optimum design of TEQ turns out to be a difficult task. In this paper, we propose a Frequency domain equalizer (FEQ) for standard OFDM systems with insufficient CP by employing the null side sub-carriers and CP redundancy. The equalizer has a sparse matrix structure and thus a low computational complexity. Theoretical anaiysis and simulation results indicate that it can efficiently remove ISI and ICI caused by insufficient CP and recover the transmitted data. Moreover, we derive the condition for the existence and uniqueness of FEQ, i.e., the combined length of CP and null subcarriers is not shorter than the channel order, which means that the insufficiency of CP in time domain can be compensated by the redundancy in frequency domain.

  17. Impact of placental insufficiency on fetal skeletal muscle growth.

    Science.gov (United States)

    Brown, Laura D; Hay, William W

    2016-11-01

    Intrauterine growth restriction (IUGR) caused by placental insufficiency is one of the most common and complex problems in perinatology, with no known cure. In pregnancies affected by placental insufficiency, a poorly functioning placenta restricts nutrient supply to the fetus and prevents normal fetal growth. Among other significant deficits in organ development, the IUGR fetus characteristically has less lean body and skeletal muscle mass than their appropriately-grown counterparts. Reduced skeletal muscle growth is not fully compensated after birth, as individuals who were born small for gestational age (SGA) from IUGR have persistent reductions in muscle mass and strength into adulthood. The consequences of restricted muscle growth and accelerated postnatal "catch-up" growth in the form of adiposity may contribute to the increased later life risk for visceral adiposity, peripheral insulin resistance, diabetes, and cardiovascular disease in individuals who were formerly IUGR. This review will discuss how an insufficient placenta results in impaired fetal skeletal muscle growth and how lifelong reductions in muscle mass might contribute to increased metabolic disease risk in this vulnerable population.

  18. Color Doppler in the Assessment of Uteroplacental Circulation Insufficiency

    Directory of Open Access Journals (Sweden)

    Ahmad Soltani Shirazi

    2010-05-01

    Full Text Available Usage of color Doppler ultrasound in the diagnosis of uteroplacental or fetal-placental vascular insufficiency is based on the theory that many of these insufficiencies are due to small vessel disease in the uteroplacental or fetal-placental vasculature which ultimately results in fetal intrauterine growth retardation, increase in prenatal mortality and morbidity and fetal neurological development. "nIn a prospective study on patients who were sus-pected for developing uteroplacental insufficiency, color Doppler ultrasound was done and the results were compared with neonatal weight (one of the most important criteria for IUGR determination which was measured just after delivery."nDirect significant relation was showed to be present between prepartum vascular changes detected in Doppler ultrasound and prognosis of IUGR. "nThree vessel types were assessed in this study:"n1. Umbilical-middle cerebral arteries"n2. Uterine arteries"n3.Venous system (umbilical, ductus venosus, IVC, which is used to assess the compensation process in fetal circulation."nThree Doppler indices of vascular resistance were studied and their abnormalities according to the age of pregnancy were assessed.

  19. 肾上腺功能不全与肝衰竭的研究进展%Adrenal insufficiency and liver failure

    Institute of Scientific and Technical Information of China (English)

    潘晨; 庄泓

    2007-01-01

    肝衰竭是一个涉及全身多器官功能的复杂的严重病变,预后差.肝衰竭患者常发生多器官功能衰竭,与许多因素影响有关.最近被提出的肾上腺功能不全就是其中一个重要因素,称为"肝-肾上腺综合征".肾上腺衰竭正在成为危重病患者发病率和病死率明显升高的重要原因.肾上腺功能不全与肝功储备及病变的严重程度相关,可引起血液动力学的不稳和肾功能不全,增加病死率.此文就近年来肾上腺功能不全在肝衰竭中的临床意义、诊断和防治上的新认识作了综述.

  20. Ultrasonographic evaluation of adrenal gland size compared to body weight in normal dogs.

    Science.gov (United States)

    Soulsby, Stacy N; Holland, Merrilee; Hudson, Judith A; Behrend, Ellen N

    2015-01-01

    The accepted cut-off value for adrenal gland maximum diameter of 0.74 cm to distinguish adrenal gland enlargement in dogs regardless of body weight may not be appropriate for small to medium breed dogs. The purpose of the current retrospective study was to examine adrenal gland dimensions as a function of body weight in healthy dogs in three weight categories ( 30 kg) representing small, medium, and large breeds, respectively, to establish greater confidence in determining if adrenal gland size is abnormal. The measurements of length (sagittal plane), cranial and caudal pole thickness (sagittal and transverse planes), and caudal pole width (transverse plane) of both adrenal glands were obtained ultrasonographically in clinically healthy dogs (n = 45) with 15 dogs in each weight group. Findings support our hypothesis that adrenal gland size correlates with body weight in normal dogs, and more precise reference intervals should be created for adrenal gland size by categorizing dogs as small, medium, or large breed. The caudal pole thickness of either adrenal gland in a sagittal plane was the best dimension for evaluating adrenal gland size based on low variability, ease, and reliability in measurement.

  1. Visualisation of the normal adrenals at SPET examination with {sup 111}In-pentetreotide

    Energy Technology Data Exchange (ETDEWEB)

    Jacobsson, Hans [Department of Radiology, Karolinska Hospital, 171 76, Stockholm (Sweden); Department of Hospital Physics, Karolinska Hospital, Stockholm (Sweden); Bremmer, Staffan [Department of Radiology, Karolinska Hospital, 171 76, Stockholm (Sweden); Larsson, Stig A. [Department of Hospital Physics, Karolinska Hospital, Stockholm (Sweden)

    2003-08-01

    On single-photon emission tomography (SPET) examinations with indium-111 pentetreotide, we have often observed a small ''hot'' spot close to the upper portion of the left kidney. While it was initially believed that this represented a tumour in the tail of the pancreas or in the left adrenal, we now understand that it represents normal adrenal uptake. Since the adrenals have not been described as normally visualised, this uptake may interfere with the interpretation of the examination. We have studied how often the normal adrenals are visualised at such examinations. One hundred consecutive clinical {sup 111}In-pentetreotide examinations in adults including SPET of the abdomen and with normal findings were studied. All examinations with a hot spot attributable to the adrenals were, when available, compared with computed tomography (CT) or magnetic resonance imaging (MRI) of the abdomen performed on clinical grounds within 3 months before or at any time after the scintigraphy. In 11 patients both adrenals were visualised. In 27 patients only the left and in one only the right adrenal was visualised. CT or MRI examinations were available for comparison in 25 of these 39 patients. Two of these showed a pathological finding in the left adrenal. A conservative interpretation is that the normal adrenal is visualised on one or both sides in at least one-quarter of adults at SPET examination with {sup 111}In-pentetreotide. (orig.)

  2. A review of the anatomy and clinical significance of adrenal veins.

    Science.gov (United States)

    Cesmebasi, Alper; Du Plessis, Maira; Iannatuono, Mark; Shah, Sameer; Tubbs, R Shane; Loukas, Marios

    2014-11-01

    The adrenal veins may present with a multitude of anatomical variants, which surgeons must be aware of when performing adrenalectomies. The adrenal veins originate during the formation of the prerenal inferior vena cava (IVC) and are remnants of the caudal portion of the subcardinal veins, cranial to the subcardinal sinus in the embryo. The many communications between the posterior cardinal, supracardinal, and subcardinal veins of the primordial venous system provide an explanation for the variable anatomy. Most commonly, one central vein drains each adrenal gland. The long left adrenal vein joins the inferior phrenic vein and drains into the left renal vein, while the short right adrenal vein drains immediately into the IVC. Multiple variations exist bilaterally and may pose the risk of surgical complications. Due to the potential for collaterals and accessory adrenal vessels, great caution must be taken during an adrenalectomy. Adrenal venous sampling, the gold standard in diagnosing primary hyperaldosteronism, also requires the clinician to have a thorough knowledge of the adrenal vein anatomy to avoid iatrogenic injury. The adrenal vein acts as an important conduit in portosystemic shunts, thus the nature of the anatomy and hypercoagulable states pose the risk of thrombosis.

  3. Can single-phase dual-energy CT reliably identify adrenal adenomas?

    Energy Technology Data Exchange (ETDEWEB)

    Helck, A.; Hummel, N.; Meinel, F.G.; Johnson, T.; Nikolaou, K.; Graser, A. [University of Munich, Institute for Clinical Radiology, Munich (Germany)

    2014-07-15

    To evaluate whether single-phase dual-energy-CT-based attenuation measurements can reliably differentiate lipid-rich adrenal adenomas from malignant adrenal lesions. We retrospectively identified 51 patients with adrenal masses who had undergone contrast-enhanced dual-energy-CT (140/100 or 140/80 kVp). Virtual non-contrast and colour-coded iodine images were generated, allowing for measurement of pre- and post-contrast density on a single-phase acquisition. Adrenal adenoma was diagnosed if density on virtual non-contrast images was ≤10 HU. Clinical follow-up, true non-contrast CT, PET/CT, in- and opposed-phase MRI, and histopathology served as the standard of reference. Based on the standard of reference, 46/57 (80.7 %) adrenal masses were characterised as adenomas or other benign lesions; 9 malignant lesions were detected. Based on a cutoff value of 10 HU, virtual non-contrast images allowed for correct identification of adrenal adenomas in 33 of 46 (71 %), whereas 13/46 (28 %) adrenal adenomas were lipid poor with a density ≥10 HU. Based on the threshold of 10 HU on the virtual non-contrast images, the sensitivity, specificity, and accuracy for detection of benign adrenal lesions was 73 %, 100 %, and 81 % respectively. Virtual non-contrast images derived from dual-energy-CT allow for accurate characterisation of lipid-rich adrenal adenomas and can help to avoid additional follow-up imaging. (orig.)

  4. Aberrant expression of glucagon receptors in adrenal glands of a patient with Cushing's syndrome and ACTH-independent macronodular adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Valeria de Miguel

    2010-06-01

    Full Text Available Adrenocorticotropin (ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH is a rare cause of Cushing´s syndrome, characterized by bilateral adrenal lesions and excess cortisol production despite ACTH suppression. Cortisol synthesis is produced in response to abnormal activation of G-protein- coupled receptors, such as gastric inhibitory peptide, vasopressin, beta adrenergic agonists, LH/hCG and serotonin receptors. The aim of this study was to analyze the expression of glucagon receptors in adrenal glands from an AIMAH patient. A patient with ACTH-independent Cushing´s syndrome and bilateral macronodular adrenal hyperplasia was screened for altered activation of adrenal receptors by physiological (mixed meal and pharmacological (gonadotrophin releasing hormone, ACTH and glucagon tests. The results showed abnormally high levels of serum cortisol after stimulation with glucagon. Hypercortisolism was successfully managed with ketoconazole treatment. Interestingly, a 4-month treatment with a somatostatin analogue (octreotide was also able to reduce cortisol secretion. Finally, Cushing's syndrome was cured after bilateral adrenalectomy. Abnormal mRNA expression for glucagon receptor in the patient´s adrenal glands was observed by Real-Time PCR procedure. These results strongly suggest that the mechanism of AIMAH causing Cushing´s syndrome in this case involves the illicit activation of adrenal glucagon receptors. This is the first case reported of AIMAH associated with ectopic glucagon receptors.

  5. Exposure to an Extremely-Low-Frequency Magnetic Field Stimulates Adrenal Steroidogenesis via Inhibition of Phosphodiesterase Activity in a Mouse Adrenal Cell Line.

    Science.gov (United States)

    Kitaoka, Kazuyoshi; Kawata, Shiyori; Yoshida, Tomohiro; Kadoriku, Fumiya; Kitamura, Mitsuo

    2016-01-01

    Extremely low-frequency magnetic fields (ELF-MFs) are generated by power lines and household electrical devices. In the last several decades, some evidence has shown an association between ELF-MF exposure and depression and/or anxiety in epidemiological and animal studies. The mechanism underlying ELF-MF-induced depression is considered to involve adrenal steroidogenesis, which is triggered by ELF-MF exposure. However, how ELF-MFs stimulate adrenal steroidogenesis is controversial. In the current study, we investigated the effect of ELF-MF exposure on the mouse adrenal cortex-derived Y-1 cell line and the human adrenal cortex-derived H295R cell line to clarify whether the ELF-MF stimulates adrenal steroidogenesis directly. ELF-MF exposure was found to significantly stimulate adrenal steroidogenesis (p steroidogenesis via an increase in intracellular cAMP caused by the inhibition of phosphodiesterase activity in Y-1 cells. The same mechanism may trigger the increase in adrenal steroid secretion in mice observed in our previous study.

  6. Alu Sx repeat-induced homozygous deletion of the StAR gene causes lipoid congenital adrenal hyperplasia.

    Science.gov (United States)

    Eiden-Plach, Antje; Nguyen, Huy-Hoang; Schneider, Ursula; Hartmann, Michaela F; Bernhardt, Rita; Hannemann, Frank; Wudy, Stefan A

    2012-05-01

    Lipoid congenital adrenal hyperplasia (Lipoid CAH) is the most severe form of the autosomal recessive disorder CAH. A general loss of the steroid biosynthetic activity caused by defects in the StAR gene manifests as life-threatening primary adrenal insufficiency. We report a case of Lipoid CAH caused by a so far not described homozygous deletion of the complete StAR gene and provide diagnostic results based on a GC-MS steroid metabolomics and molecular genetic analysis. The patient presented with postnatal hypoglycemia, vomiting, adynamia, increasing pigmentation and hyponatremia. The constellation of urinary steroid metabolites suggested Lipoid CAH and ruled out all other forms of CAH or defects of aldosterone biosynthesis. After treatment with sodium supplementation, hydrocortisone and fludrocortisone the child fully recovered. Molecular genetic analysis demonstrated a homozygous 12.1 kb deletion in the StAR gene locus. The breakpoints of the deletion are embedded into two typical genomic repetitive Alu Sx elements upstream and downstream of the gene leading to the loss of all exons and regulatory elements. We established deletion-specific and intact allele-specific PCR methods and determined the StAR gene status of all available family members over three generations. This analysis revealed that one of the siblings, who died a few weeks after birth, carried the same genetic defect. Since several Alu repeats at the StAR gene locus increase the probability of deletions, patients with typical symptoms of lipoid CAH lacking evidence for the presence of both StAR alleles should be analyzed carefully for this kind of disorder.

  7. The diagnosis and treatment of critical illness-related corticosteroid insufficiency%危重症相关性皮质醇不足的诊治研究进展

    Institute of Scientific and Technical Information of China (English)

    张哲哲

    2011-01-01

    In recent years,relative adrenal insufficiency in critical patients has become a hot issue.With progression of studies,this definition is insufficient in accuracy.Thus a new definition of critical illnessrelated corticosteroid insufficiency(CIRCI)is developed.Now CIRCI have not had consentaneous diagnostic criteria.Moreover,the benefits of this diagnose to corticosteroid treatment in critical patients remains unclear.This article will review on the diagnosis of CIRCI and current situation of corticosteroid treatment.%近年来,危重症患者合并的“肾上腺相对功能不全”已成为研究热点,随着研究进展,学者们认为该定义不够准确.因此提出了危重症相关性皮质醇不足(critical illness-related corticosteroid insufficiency,CIRCI)这一概念.CIRCI 目前尚无统一诊断标准,其对危重症患者激素治疗的指导作用也不甚明确.本文仅就目前CIRCI诊断以及激素替代治疗的现状进行综述.

  8. Endovascular therapy for chronic cerebrospinal venous insufficiency in multiple sclerosis

    Directory of Open Access Journals (Sweden)

    Marc A. Lazzaro

    2011-07-01

    Full Text Available Recent reports have emerged suggesting that multiple sclerosis (MS may be due to abnormal venous outflow from the central nervous system, termed Chronic Cerebrospinal Venous Insufficiency (CCSVI. These reports have generated strong interest and controversy over the prospect of a treatable cause of this chronic debilitating disease. This review aims to describe the proposed association between CCSVI and MS, summarize the current data, and discuss the role of endovascular therapy and the need for rigorous randomized clinical trials to evaluate this association and treatment.

  9. Use of Cervical Pessary in the Management of Cervical Insufficiency.

    Science.gov (United States)

    Timofeev, Julia

    2016-06-01

    Prevention of spontaneous preterm birth is an important public health priority. Pessary may be a potential therapy in cases of cervical insufficiency, in singleton and multiple gestations. Availability of transvaginal sonography for accurate assessment of cervical length is allowing for the tailoring of therapy to a more specific subset of patients who may benefit from this treatment. Pessary therapy is attractive given the favorable side effect profile, low cost, and ease of placement and removal. Large randomized trials are ongoing to validate initial favorable findings.

  10. Pharmacokinetics and pharmacodynamics of roxatidine in patients with renal insufficiency.

    OpenAIRE

    1995-01-01

    1. Roxatidine acetate, a new histamine H2-receptor antagonist, was administered in the evening (75 mg p.o.) to eight patients with renal insufficiency (CLCR 8-17 ml min-1) for 12 days and plasma drug concentrations were measured. 2. Ambulatory intragastric pH was monitored following the last dose and values were compared with those on day 1 when all patients received a placebo. 3. The terminal elimination half-life (mean +/- s.d.) of roxatidine was 10.8 +/- 2.4 h and its oral clearance was 17...

  11. VENOUS INSUFFICIENCY AND THROMBOEMBOLIC DISEASE IN BARIATRIC SURGERY PATIENTS

    Directory of Open Access Journals (Sweden)

    Bonno van BELLEN

    2013-09-01

    Full Text Available Context Morbid obesity is associated with various co-morbidities, including chronic venous insufficiency. Bariatric surgery is the only effective treatment for morbid obesity, but with potential risks and possible complications, including venous thromboembolism. Objective To determine the prevalence of clinical and ultrasonographic signs of chronic venous insufficiency in morbid obese patients in preparation for bariatric surgery and the incidence of post-operative venous thromboembolic disease. Methods Patients on work-up for bariatric surgery of Centro Terapêutico Especializado em Fígado (CETEFI and Pro-Gastro surgical teams of the Hospital Beneficência Portuguesa de São Paulo were included. The analysed data were pre-operative findings for venous insufficiency (CEAP - clinical, etiological, anatomical, physiopathologic - classification and venous ultrassonographic findings, type of surgery (open or laparoscopic, abdominal circumference, body mass index (BMI and post-operative ultrassonography search for venous insufficiency and deep venous thrombosis. Results Between March 2007 and December 2009, 95 patients candidates for bariatric surgery had clinical and duplex scan evaluation of the lower limbs venous system. Of the 95 patients, 53 were submitted to the surgical procedure. There was a predominance of women (77.9%, the average age was 38.5 years, average preoperative weight 124.6 kg and average BMI of 45.5 kg/m2. Regarding obesity, 16.8% were obese, and 83.1% were morbidly obese. In relation to the venous findings, 86.3% of the patients did fit CEAP classification less than 3 and 13.7% greater than or equal to 3. Among the post-operative complications, there were four cases of wound infection. Three patients developed post-operative distal venous thrombosis (7.5%, but no one had clinically manifested pulmonary embolism. Conclusion No relation between BMI, CEAP classification and venous ultrassonographic findings were found. Although

  12. French Military Intervention in Mali: Inevitable, Consensual yet Insufficient

    OpenAIRE

    2013-01-01

    This practice note offers an account of France’s military intervention in Mali launched on 11 January 2013. Firstly, it provides an analysis of the events that led up to the dual crisis in the country’s northern and southern regions and to a new wave of armed conflict between government and rebel forces. Then, it is argued that although the French military intervention was framed as inevitable and based on a broad consensus, it will nevertheless be insufficient to address the root sociopoliti...

  13. Central dislocation of the hip secondary to insufficiency fracture

    Directory of Open Access Journals (Sweden)

    Moe Thaya

    2010-03-01

    Full Text Available We present a case report of a 45-year old man who sustained a central dislocation of the hip secondary to an insufficiency fracture of the acetabulum. At the time of presentation he was on alendronate therapy for osteoporosis which had been previously investigated. CT scanning of the pelvis was useful for pre-operative planning which confirmed collapse of the femoral head but no discontinuity of the pelvis. The femoral head was morcellized and used as bone graft for the acetabular defect and an uncemented total hip replacement was performed.

  14. Adrenal glucocorticoids regulate adipsin gene expression in genetically obese mice.

    Science.gov (United States)

    Spiegelman, B M; Lowell, B; Napolitano, A; Dubuc, P; Barton, D; Francke, U; Groves, D L; Cook, K S; Flier, J S

    1989-01-25

    Adipsin expression at the protein and mRNA levels is greatly reduced in several distinct syndromes of obesity in the mouse: genetic obesity due to the db/db and ob/ob genes, and a chemically induced model secondary to neonatal exposure to monosodium glutamate. We considered first the possibility that the adipsin gene might be identical to the db or ob locus and the lowered expression of this protein might result from a mutation in this gene. We show here that the adipsin structural gene is located on chromosome 10 and hence is physically distinct from any obesity genes so far identified in the mouse. A major role for the adrenal gland and adrenal glucocorticoids in the aberrant regulation of adipsin in these models of obesity is indicated by several experiments. Adrenalectomy of the ob/ob mouse raises the circulating levels of adipsin protein and the amount of this mRNA in epididymal fat pads (5-fold), although neither is increased to the levels seen in lean controls. Exogenous administration of corticosterone completely blocks the effects of adrenalectomy on adipsin, suggesting that the effect of this endocrine ablation is through reduction of adrenal glucocorticoids. Corticosterone administration also causes suppression in the levels of adipsin mRNA and protein in lean mice, although this decrease is never as severe as that seen in obese mice. The effect of exogenous corticosterone in lean mice occurs within 2 days and hence is not secondary to the obesity which these hormones eventually elicit. These results indicate that glucocorticoids can regulate adipsin expression in vivo and strongly suggest that the hyperglucocorticoid state seen in certain obese models plays a significant role in lowering adipsin mRNA and protein levels. Quantitative analysis of these experiments suggests that other as yet unknown neuroendocrine factors also function to suppress adipsin in obesity.

  15. Adrenal function in asthmatic children treated with inhaled budesonide

    DEFF Research Database (Denmark)

    Bisgaard, H; Pedersen, S; Damkjaer Nielsen, M;

    1991-01-01

    The effect of the inhaled topical steroid budesonide on adrenal function was evaluated in 33 children (aged 7-15 years) with moderate bronchial asthma. The trial was designed as a prospective single-blind study of the effect of budesonide in daily doses of 200 microgram through 400 microgram to 800...... significantly during treatment with 800 microgram budesonide as compared to treatment with 200 microgram budesonide (95% confidence interval: 74%-112%). It is concluded, that budesonide is a topical steroid with a favourable ratio between topical and systemic effects in asthmatic children....

  16. THE RESULTS OF SURGICAL TREATMENT OF PATIENTS WITH ADRENAL TUMORS

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    A. A. Kostin

    2016-01-01

    Full Text Available Malignant adrenal tumors such as adrenocortical cancer (ACC and malignant pheochromocytoma (MPCC have a particularly aggressive course, leading to higher mortality in these patients, especially in the later stages of the disease. In this regard, there is a statistically significant difference in survival of patients, depending on the time of detection and initiation of treatment.Purpose. Analysis of the results of surgical treatment of patients with adrenal tumors.Patients and methods. 55 patients aged from 17 to 75 years (median 50 ± 13 years with primary tumor neoplasms of the adrenal gland underwent surgical treatment in the period from 1999 to 2014 in the Department of Onco-urology, P. Hertsen MORI. 18 adrenalectomy (32,7% completed laparoscopy, 36 (65,5% – open access, 1 (1,8% – videoassistance. Among them, retroperitoneal lymph node dissection with ipsilateral hand made in 14 (25.5%, combined operations with resection of adjacent organs – 7 (12,7%, thrombectomy with resection of the inferior Vena cava – 3 (5,5% cases. The median time was 100 ± 73 minutes in open surgical procedures – 183 ± 55 minutes, laparoscopic – 60 ± 30 minutes. The volume of blood loss – 300 ± 1136 ml. According to the routine morphological studies, 24 (43,6% patients the tumor were benign, the remaining 31 (56,4% – malignant.Results. Long-term results have been traced in 84% of patients in group with ACC. The observation time for patients varied from 2 to 167 months, with a median of 64.5 (IQR 22–111 months. Median survival without progression and 5-year overall and tumor survival was: I stage – 93 months and 67 ± 13%, II stage – 30 months and 75 ± 21%, III stage– 18 months and 67 ± 27%, IV stage – 10 months and 25 ± 21%. The observation time for patients with MPCC varies from 12 to 102 months, with a median of 60 (IQR 18–102 months. Median survival without progression was 26 (IQR 15 to 38 months, 5-year overall and tumor survival

  17. Adrenal fatigue does not exist: a systematic review

    OpenAIRE

    Cadegiani, Flavio A.; Kater, Claudio E.

    2016-01-01

    Background The term “adrenal fatigue” (“AF”) has been used by some doctors, healthcare providers, and the general media to describe an alleged condition caused by chronic exposure to stressful situations. Despite this, “AF” has not been recognized by any Endocrinology society, who claim there is no hard evidence for the existence. The aim of this systematic review is to verify whether there is substantiation for “AF”. Methods A systematic search was performed at PUBMED, MEDLINE (Ebsco) and Co...

  18. [Unilateral spontaneous adrenal hematoma: an unusual cause of retroperitoneal hemorrhage].

    Science.gov (United States)

    Vendrell, J R; Alcover, J; Alcaraz, A; Llopis, J; Gutierrez, R; Barranco, M A; Carretero, P

    1996-01-01

    Presentation of one case of spontaneous retroperitoneal haemorrhage in a 72 year-old male, his first symptom being a left retrothoracic pain of sudden onset. The supplementary studies performed (ultrasound, CAT and MNR) pointed to the adrenal gland as the origin of the haemorrhage. Faced eith the uncertainty of the etiological diagnosis, a left supra-renalectomy was conducted which confirmed the pathological anatomy of the piece and the presence of massive haematoma of the suprarenal gland with no other pathological data. The clinical, diagnostic and therapeutical aspects are discussed.

  19. Localization of a pheochromocytoma using I-123 MIBG adrenal scintigraphy.

    Directory of Open Access Journals (Sweden)

    Shirkare S

    1994-04-01

    Full Text Available In a patient with the clinical diagnosis of pheochromocytoma, the localization of the tumor is essential for planning treatment. Recently, we have performed I-123 metaiodobenzylguanidine (MIBG adrenal scintigraphy in a patient presenting with a history of paroxysmal hypertension. Scintigraphy accurately located an ectopic unilateral pheochromocytoma. The scintigraphic diagnosis was confirmed by surgery and a diagnosis of ectopic unilateral pheochromocytoma was made by histopathological examination. This case report illustrates the specific diagnosis of pheochromocytoma by I-123 MIBG scintigraphy which is especially useful when other diagnostic procedures are equivocal.

  20. Effects of DDT on bobwhite quail adrenal gland

    Science.gov (United States)

    Lehman, J.W.; Peterle, T.J.; Mulls, C.M.

    1974-01-01

    A wide range of responses to sublethal levels of DDT exist, many of which are species specific and vary within each species depending upon age, sex, and physiological state. Sublethal levels of DDT do cause an increase in the adrenal cortical tissue of bobwhite quail, which may cause increased secretion of corticosteroids, and in turn affect reproduction. A delicate homeostatic balance exists within the avian endocrine system which may be disturbed by feeding sublethal levels of chlorinated hydrocarbon pesticides. This adverse effect on the endocrine system may cause subtle reproductive failures which go unnoticed until the population is greatly reduced.

  1. [Dynamics of lipid peroxidation and steroidogenesis in adrenal cortex during stress].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Vinogradov, V V

    1990-01-01

    The phase character of lipid peroxidation has been found in the rabbit adrenal cortex in the process of adaptation to extreme loads. Under acute stress the activation of lipid peroxidation is directly dependent on the hormonal synthesis processes. Under conditions of the prolonged stress factor an enhancement of the lipid peroxidation intensity in the adrenal cortex coincides with a decrease in the steroidogenesis rate.

  2. Marked left ventricular hypertrophy mimicking hypertrophic cardiomyopathy associated with steroid therapy for congenital adrenal hyperplasia.

    Science.gov (United States)

    Scirè, Giuseppe; D'Anella, Giorgio; Cristofori, Laura; Mazzuca, Valentina; Cianfarani, Stefano

    2007-06-01

    We describe a child treated with high-dose steroid therapy for congenital adrenal hyperplasia who showed marked left ventricular hypertrophy mimicking hypertrophic cardiomyopathy associated with steroid therapy for congenital adrenal hyperplasia. Cardiomyopathy reversed completely when an appropriate steroid therapeutic regimen was established.

  3. Spontaneous rupture of adrenal pheochromocytoma in a patient with Von Recklinghausen's disease

    OpenAIRE

    Azhough, Ramin; Barband, Ali Reza; Motayagheni, Negar; Niafar, Mitra; Pourfathi, Hojjat

    2009-01-01

    Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen's disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, irreversible hemodynamic shock, and death.

  4. Spontaneous rupture of adrenal pheochromocytoma in a patient with Von Recklinghausen′s disease

    Directory of Open Access Journals (Sweden)

    Azhough Ramin

    2009-01-01

    Full Text Available Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen ′ s disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, irreversible hemodynamic shock, and death.

  5. Spontaneous rupture of adrenal pheochromocytoma in a patient with Von Recklinghausen's disease

    Science.gov (United States)

    Azhough, Ramin; Barband, Ali Reza; Motayagheni, Negar; Niafar, Mitra; Pourfathi, Hojjat

    2009-01-01

    Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen's disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, irreversible hemodynamic shock, and death. PMID:19881191

  6. Hepatic lipase gene is transcribed in rat adrenals into a truncated mRNA

    NARCIS (Netherlands)

    A.J.M. Verhoeven (Adrie); D. Carling; H. Jansen (Hans)

    1994-01-01

    textabstractRat adrenals contain a lipase activity that is indistinguishable from hepatic lipase (HL) present in liver. Expression of HL mRNA in adrenals was studied using the method of reverse transcription-polymerase chain reaction (RT-PCR). A 596-bp fragment of HL cD

  7. The diversity of abnormal hormone receptors in adrenal Cushing's syndrome allows novel pharmacological therapies

    Directory of Open Access Journals (Sweden)

    Lacroix A.

    2000-01-01

    Full Text Available Recent studies from several groups have indicated that abnormal or ectopic expression and function of adrenal receptors for various hormones may regulate cortisol production in ACTH-independent hypercortisolism. Gastric inhibitory polypeptide (GIP-dependent Cushing's syndrome has been described in patients with either unilateral adenoma or bilateral macronodular adrenal hyperplasia; this syndrome results from the large adrenal overexpression of the GIP receptor without any activating mutation. We have conducted a systematic in vivo evaluation of patients with adrenal Cushing's syndrome in order to identify the presence of abnormal hormone receptors. In macronodular adrenal hyperplasia, we have identified, in addition to GIP-dependent Cushing's syndrome, other patients in whom cortisol production was regulated abnormally by vasopressin, ß-adrenergic receptor agonists, hCG/LH, or serotonin 5HT-4 receptor agonists. In patients with unilateral adrenal adenoma, the abnormal expression or function of GIP or vasopressin receptor has been found, but the presence of ectopic or abnormal hormone receptors appears to be less prevalent than in macronodular adrenal hyperplasia. The identification of the presence of an abnormal adrenal receptor offers the possibility of a new pharmacological approach to control hypercortisolism by suppressing the endogenous ligands or by using specific antagonists for the abnormal receptors.

  8. LCAT deficiency in mice is associated with a diminished adrenal glucocorticoid function

    NARCIS (Netherlands)

    Hoekstra, Menno; Korporaal, Suzanne J. A.; van der Sluis, Ronald J.; Hirsch-Reinshagen, Veronica; Bochem, Andrea E.; Wellington, Cheryl L.; Van Berkel, Theo J. C.; Kuivenhoven, Jan Albert; Van Eck, Miranda

    2013-01-01

    containing lipoproteins can provide cholesterol for synthesis of glucocorticoids. Here we assessed adrenal glucocorticoid function in LCAT knockout (KO) mice to determine the specific contribution of HDL-cholesteryl esters to adrenal glucocorticoid output in vivo. LCAT KO mice exhibit an 8-fold high

  9. Significant growth of adrenal lymphangioma: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Ki Ho Kim

    2015-01-01

    Conclusion: Lymphangiomas are most commonly located in the neck, axillary region and mediastinum, which are rare at adrenal gland. Transperitoneal laparoscopic removal of the adrenal lymphangiomas is regarded as a safe, effective and minimally invasive approach. In our case, laparoscopic removal of the tumor was successfully performed via a transperitoneal approach.

  10. Candesartan decreases the sympatho-adrenal and hormonal response to isolation stress

    Directory of Open Access Journals (Sweden)

    Ines Armando

    2001-03-01

    Full Text Available A change from group housing to isolation in unfamiliar metabolic cages represents, for rodents, a significant emotional stress. We studied the effect of candesartan, a peripheral and central angiotensin II AT1-receptor antagonist, on the hormonal and sympathetic response to acute isolation. We pretreated rats with 1 mg/kg/day candesartan for 13 days via subcutaneously implanted osmotic minipumps, followed by 24-hour isolation in individual metabolic cages. We measured brain, pituitary and adrenal angiotensin II (Ang II receptor binding by quantitative autoradiography and adrenal hormones and catecholamines by RIA and HPLC. Isolation increased adrenal catecholamines, aldosterone and corticosterone, AT1-receptor binding in the zona glomerulosa and AT2-receptor binding in the adrenal medulla. Candesartan pretreatment decreased adrenal catecholamines, aldosterone and corticosterone, AT1-receptor binding in adrenal zona glomerulosa and medulla, pituitary gland and the hypothalamic paraventricular nucleus, and AT2-receptor binding in adrenal medulla, but increased AT2-receptor binding in zona glomerulosa. We conclude that peripheral and central AT1-receptor blockade with candesartan decreases the sympatho-adrenal and hormonal response to acute stress. Our results indicate that Ang II is an important stress hormone and suggest that blockade of the physiologically active AT 1-receptors could influence stress-related disorders.

  11. Magnetic Resonance Imaging of the Vocal Folds in Women with Congenital Adrenal Hyperplasia and Virilized Voices

    Science.gov (United States)

    Nygren, Ulrika; Isberg, Bengt; Arver, Stefan; Hertegård, Stellan; Södersten, Maria; Nordenskjöld, Agneta

    2016-01-01

    Purpose: Women with congenital adrenal hyperplasia (CAH) may develop a virilized voice due to late diagnosis or suboptimal suppression of adrenal androgens. Changes in the vocal folds due to virilization have not been studied in vivo. The purpose was to investigate if the thyroarytenoid (TA) muscle is affected by virilization and correlate…

  12. Adolescent Premature Ovarian Insufficiency Following Human Papillomavirus Vaccination

    Directory of Open Access Journals (Sweden)

    Deirdre Therese Little MBBS, DRANZCOG, FACRRM

    2014-10-01

    Full Text Available Three young women who developed premature ovarian insufficiency following quadrivalent human papillomavirus (HPV vaccination presented to a general practitioner in rural New South Wales, Australia. The unrelated girls were aged 16, 16, and 18 years at diagnosis. Each had received HPV vaccinations prior to the onset of ovarian decline. Vaccinations had been administered in different regions of the state of New South Wales and the 3 girls lived in different towns in that state. Each had been prescribed the oral contraceptive pill to treat menstrual cycle abnormalities prior to investigation and diagnosis. Vaccine research does not present an ovary histology report of tested rats but does present a testicular histology report. Enduring ovarian capacity and duration of function following vaccination is unresearched in preclinical studies, clinical and postlicensure studies. Postmarketing surveillance does not accurately represent diagnoses in adverse event notifications and can neither represent unnotified cases nor compare incident statistics with vaccine course administration rates. The potential significance of a case series of adolescents with idiopathic premature ovarian insufficiency following HPV vaccination presenting to a general practice warrants further research. Preservation of reproductive health is a primary concern in the recipient target group. Since this group includes all prepubertal and pubertal young women, demonstration of ongoing, uncompromised safety for the ovary is urgently required. This matter needs to be resolved for the purposes of population health and public vaccine confidence.

  13. The Soft Palate Friendly Speech Bulb for Velopharyngeal Insufficiency

    Science.gov (United States)

    Kahlon, Monaliza; Gupta, Shilpa; Dhingra, Parvinder Singh

    2016-01-01

    Velopharyngeal insufficiency is an anatomic defect of the soft palate making palatopharyngeal sphincter incomplete. It is an important concern to address in patients with bilateral cleft lip and palate. Speech aid prosthesis or speech bulbs are best choice in cases where surgically repaired soft palate is too short to contact pharyngeal walls during function but these prosthesis have been associated with inadequate marginal closure, ulcerations and patient discomfort. Here is a case report of untreated bilateral cleft lip and palate associated with palatal insufficiency treated by means of palate friendly innovative speech bulb. This modified speech bulb is a combination of hard acrylic and soft lining material. The hard self-curing acrylic resin covers only the hard palate area and a permanent soft silicone lining material covering the soft palate area. A claw-shaped wire component was extended backwards from acrylic and was embedded in soft silicone to aid in retention and approximation of two materials. The advantage of adding the soft lining material in posterior area helped in covering the adequate superior extension and margins for maximal pharyngeal activity. This also improved the hypernasality, speech, comfort and overall patient acceptance. PMID:27790589

  14. Urinary hemosiderin: role in evaluation of chronic venous insufficiency

    Directory of Open Access Journals (Sweden)

    Ashish Lal Shrestha

    2012-08-01

    Full Text Available Chronic venous insufficiency (CVI leads to skin changes with dermal hemosiderin deposition. We studied the presence of hemosiderin in the urine to assess if this could be used as a biochemical marker for CVI. Hereby we present a case control study conducted in a tertiary care centre in South India. There were 100 cases with evidence of advanced CVI (the Clinical-Etiology-Anatomy-Pathophysiology classification: C5, C6 confirmed by duplex scanning. Controls were 50 patients with leg ulcers due to other etiologies. All patients were subjected to urinary hemosiderin testing. In all 100 patients with CVI (C5 and C6 disease axial venous reflux was confirmed by duplex ultrasound. Superficial venous reflux was noted in 71% of patients and deep venous reflux in 54.%. Primary venous insufficiency was the etiology in 81% of patients. Only 4/100 patients had detectable amounts of hemosiderin in the urine. Urine hemosiderin testing to determine presence or absence of CVI yielded the following values: positive predictive value-80%; negative predictive value-33%; sensitivity-4% and specificity-98%. The test could not be recommended as a marker of CVI. In Indian patients urinary hemosiderin is not a useful screening test in CVI.

  15. Enzyme replacement therapy for pancreatic insufficiency: present and future

    Directory of Open Access Journals (Sweden)

    Fieker A

    2011-05-01

    Full Text Available Aaron Fieker1, Jessica Philpott1, Martine Armand21Division of Digestive Diseases, University of Oklahoma, OKC, OK, USA; 2INSERM, U476 "Nutrition Humaine et Lipides", Marseille, F-13385 France; Univ Méditerranée Aix-Marseille 2, Faculté de Médecine, IPHM-IFR 125, Marseille, F-13385 FranceAbstract: Pancreatic enzyme replacement therapy is currently the mainstay of treatment for nutrient malabsorption secondary to pancreatic insufficiency. This treatment is safe and has few side effects. Data demonstrate efficacy in reducing steatorrhea and fat malabsorption. Effective therapy has been limited by the ability to replicate the physiologic process of enzyme delivery to the appropriate site, in general the duodenum, at the appropriate time. The challenges include enzyme destruction in the stomach, lack of adequate mixing with the chyme in the duodenum, and failing to deliver and activate at the appropriate time. Treatment is begun when clinically significant malabsorption occurs resulting in steatorrhea and weight loss. Treatment failure is addressed in a sequential fashion. Current research is aimed at studying new enzymes and delivery systems to improve the efficiency of action in the duodenum along with developing better means to monitor therapy.Keywords: exocrine pancreatic insufficiency, chronic pancreatitis, cystic fibrosis, pancreatic enzyme replacement therapy, lipase, lipids

  16. Autonomous homing control of a powered parafoil with insufficient altitude.

    Science.gov (United States)

    Tao, Jin; Sun, Qing-Lin; Tan, Pan-Long; Chen, Zeng-Qiang; He, Ying-Ping

    2016-11-01

    In order to realize safe and accurate homing of a powered parafoil under the condition of insufficient initial altitude, a multiphase homing path is designed according to the flight characteristics of the vehicle. With consideration that the traditional control methods cannot ensure the quality of path following because of the nonlinear, large inertial and longtime delay existed in the system and strong disturbances in a complex environment, a homing controller, composed of the vertical and horizontal trajectory tracking controllers, is designed based on active disturbance rejection control (ADRC). Then autonomous homing simulation experiment of the powered parafoil with insufficient altitude is carried on in a windy environment. The simulation results show that the planned multiphase homing trajectory can fulfill the requirements of fixed-point homing and flare landing; the designed homing controller can overcome the influences of uncertain items of the internal and external disturbances, track the desired homing path more rapidly and steadily, and possesses better control performances than traditional PID controllers.

  17. Review of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?

    Directory of Open Access Journals (Sweden)

    Lee PeterA

    2010-12-01

    Full Text Available There is ample historical verification of 46,XX congenital adrenal hyperplasia (CAH patients being born with essentially male genitaliawhile outcome information is scant. Prior to glucocorticoid therapy, most patients died very young from adrenal insufficiency. Most available reports from laterchildhood, contain little information concerning sexual identity. Reports on older individuals lack adequate information about sexual identity and quality of life. The difficulty in assessing the relative impact of multiple dynamic environmental factors on the development of sexual identity, self- and body esteem and overall adjustment to life is clear. Nevertheless, it remains unclear whether those infants whose masculine genitalia at birth resulted in an initial male assignment would have enjoyed a better adult outcome had they been allowed to remain male rather than the female reassignment that most received. Further, one could ask whether a male sex of rearing should be considered in 46,XX CAH infants with male external genitalia. After reviewing available literature, we conclude that because those extremely virlized 46,XX CAH patients who were reared male with healthy social support demonstrated satisfactory levels of social and sexual function as adults a male sex assignment should be considered in these types of infants when social and cultural environment are supportive.

  18. Review of Outcome Information in 46,XX Patients with Congenital Adrenal Hyperplasia Assigned/Reared Male: What Does It Say about Gender Assignment?

    Science.gov (United States)

    Lee, Peter A; Houk, Christopher P

    2010-01-01

    There is ample historical verification of 46,XX congenital adrenal hyperplasia (CAH) patients being born with essentially male genitaliawhile outcome information is scant. Prior to glucocorticoid therapy, most patients died very young from adrenal insufficiency. Most available reports from laterchildhood, contain little information concerning sexual identity. Reports on older individuals lack adequate information about sexual identity and quality of life. The difficulty in assessing the relative impact of multiple dynamic environmental factors on the development of sexual identity, self- and body esteem and overall adjustment to life is clear. Nevertheless, it remains unclear whether those infants whose masculine genitalia at birth resulted in an initial male assignment would have enjoyed a better adult outcome had they been allowed to remain male rather than the female reassignment that most received. Further, one could ask whether a male sex of rearing should be considered in 46,XX CAH infants with male external genitalia. After reviewing available literature, we conclude that because those extremely virlized 46,XX CAH patients who were reared male with healthy social support demonstrated satisfactory levels of social and sexual function as adults a male sex assignment should be considered in these types of infants when social and cultural environment are supportive.

  19. Diminished epinephrine response to hypoglycemia despite enlarged adrenal medulla in trained rats

    DEFF Research Database (Denmark)

    Stallknecht, B; Kjaer, M; Mikines, K J

    1990-01-01

    Studies in humans have indicated that trained athletes compared with sedentary subjects have an increased capacity to secrete epinephrine. To investigate whether this is due to an adaptation induced by physical training or a selection phenomenon, rats were swim trained (T) 10 wk for 6 h....../day or served as controls being either sedentary freely eating (C), food restricted (FR), sham swim trained (ST), or cold stressed (CS). Adrenal glands were weighted and cross sectioned for light microscopic determination of size of the adrenal medulla. Endurance-trained compared with control rats had heavier......)]. Cold stress or sham swim training did not increase adrenal weight or volume of adrenal medulla (P greater than 0.05). To stimulate adrenal medulla secretion, rats had an insulin-induced hypoglycemia. Insulin dose needed to suppress plasma glucose below 4.0 mM was four times greater in sedentary...

  20. Preliminary report: effect of adrenal androgen and estrogen on bone maturation and bone mineral density.

    Science.gov (United States)

    Arisaka, O; Hoshi, M; Kanazawa, S; Numata, M; Nakajima, D; Kanno, S; Negishi, M; Nishikura, K; Nitta, A; Imataka, M; Kuribayashi, T; Kano, K

    2001-04-01

    To clarify the independent physiological roles of adrenal androgen and estrogen on bone growth, we compared the lumbar spine bone mineral density (BMD) in prepubertal girls with virilizing congenital adrenal hyperplasia (CAH) (n = 17) and girls with central precocious puberty (CPP) (n = 18). When BMD was analyzed according to chronologic age, no significant differences were found between CPP and CAH patients. However, when adjusted to bone age, BMD was statistically higher in CAH than in CPP subjects. This finding suggests that adrenal androgen, as well as estrogen, plays an important role in increasing BMD. Adrenal androgen may act on bone not only as androgen, but as estrogen after having been metabolized into an aromatized bone-active compound in peripheral tissues, such as bone and fat. Therefore, adrenal androgen may have a more important role in increasing BMD than previously realized.

  1. [Hyperprogesteronism due to bilateral adrenal carcinomas in a cat with diabetes mellitus].

    Science.gov (United States)

    Quante, S; Sieber-Ruckstuhl, N; Wilhelm, S; Favrot, C; Dennler, M; Reusch, C

    2009-09-01

    An 8 year old male castrated Russian Blue cat with polyuria, polydipsia, polyphagia, abdominal enlargement, unkempt and easily epilated hair coat and abdominal alopecia is described. As a first step diabetes mellitus was diagnosed. Further work-up by ultrasonography revealed severe bilateral enlargement of the adrenal glands. Hypercortisolism was suspected and therefore ACTH stimulation test and dexamethasone suppression test were performed. In all samples cortisol concentrations were below the detection limit of the assay used. Various precursor hormones were measured and high progesterone concentrations were found. Histologically, the adrenal masses were characterised as bilateral adrenal carcinomas of the adrenal cortex. The case report demonstrates that adrenal gland tumors are also capable to secrete sex hormones instead of cortisol. Clinical signs of hyperprogesteronism are identical to those of hypercortisolism.

  2. Temporal and spatial distribution of mast cells and steroidogenic enzymes in the human fetal adrenal.

    Science.gov (United States)

    Naccache, Alexandre; Louiset, Estelle; Duparc, Céline; Laquerrière, Annie; Patrier, Sophie; Renouf, Sylvie; Gomez-Sanchez, Celso E; Mukai, Kuniaki; Lefebvre, Hervé; Castanet, Mireille

    2016-10-15

    Mast cells are present in the human adult adrenal with a potential role in the regulation of aldosterone secretion in both normal cortex and adrenocortical adenomas. We have investigated the human developing adrenal gland for the presence of mast cells in parallel with steroidogenic enzymes profile and serotonin signaling pathway. RT-QPCR and immunohistochemical studies were performed on adrenals at 16-41 weeks of gestation (WG). Tryptase-immunopositive mast cells were found from 18 WG in the adrenal subcapsular layer, close to 3βHSD- and CYP11B2-immunoreactive cells, firstly detected at 18 and 24 WG, respectively. Tryptophan hydroxylase and serotonin receptor type 4 expression increased at 30 WG before the CYP11B2 expression surge. In addition, HDL and LDL cholesterol receptors were expressed in the subcapsular zone from 24 WG. Altogether, our findings suggest the implication of mast cells and serotonin in the establishment of the mineralocorticoid synthesizing pathway during fetal adrenal development.

  3. Incidentally Detected Kaposi Sarcoma of Adrenal Gland with Anaplastic Features in an HIV Negative Patient

    Directory of Open Access Journals (Sweden)

    Zeliha Esin Celik

    2016-01-01

    Full Text Available Kaposi sarcoma (KS, a vascular tumor caused by infection with human herpesvirus 8 (HHV8, is a systemic disease that can present with cutaneous lesions with or without visceral involvement. Very few cases of KS, most of which were associated with AIDS, have been reported in the adrenal gland. Anaplastic transformation of KS is a rare clinical presentation known as an aggressive disease with local recurrence and metastatic potential. We report here a 47-year-old HIV negative male presented with extra-adrenal symptoms and had an incidentally detected anaplastic adrenal KS exhibited aggressive clinical course. To the best of our knowledge, this is the first case of anaplastic primary adrenal KS without mucocutaneous involvement but subsequently developed other side adrenal metastases in an HIV negative patient.

  4. Acute Adrenal Crisis in an Asthmatic Child Treated with Inhaled Fluticasone Proprionate

    Directory of Open Access Journals (Sweden)

    Ratzan Susan

    2010-07-01

    Full Text Available Adrenal suppression secondary to prolonged inhaled corticosteroid use is usually limited to biochemical abnormalities, with no obvious clinical effects. Acute adrenal crisis is much rarer event but has been reported with increasing frequency. We report a case of a 7-year-old asthmatic child who presented with an acute history of lethargy after a respiratory infection. He was maintained on 220 g/day of fluticasone propionate for several years. Initial evaluation revealed severe adrenal suppression, with undetectable cortisol levels and minimal response after stimulation with ACTH. After fluticasone was discontinued, a gradual recovery of the adrenal axis was seen. This case shows that acute adrenal crisis may be a consequence even at the usual prescribed doses, stressing the importance of using the lowest dose of inhaled steroids needed to control symptoms and having an increased awareness of this complication.

  5. Acute Adrenal Crisis in an Asthmatic Child Treated with Inhaled Fluticasone Proprionate

    Directory of Open Access Journals (Sweden)

    Angela H. Santiago

    2010-01-01

    Full Text Available Adrenal suppression secondary to prolonged inhaled corticosteroid use is usually limited to biochemical abnormalities, with no obvious clinical effects. Acute adrenal crisis is much rarer event but has been reported with increasing frequency. We report a case of a 7-year-old asthmatic child who presented with an acute history of lethargy after a respiratory infection. He was maintained on 220 μg/day of fluticasone propionate for several years. Initial evaluation revealed severe adrenal suppression, with undetectable cortisol levels and minimal response after stimulation with ACTH. After fluticasone was discontinued, a gradual recovery of the adrenal axis was seen. This case shows that acute adrenal crisis may be a consequence even at the usual prescribed doses, stressing the importance of using the lowest dose of inhaled steroids needed to control symptoms and having an increased awareness of this complication.

  6. [Maternal adrenal necrosis in the third trimester of pregnancy: a rare complication of antiphospholipid syndrome].

    Science.gov (United States)

    Legendre, G; Vauthier-Brouzes, D; Cornet, A; Al Hawari, M; Renard-Penna, R; Piette, J-C; Dommergues, M

    2008-04-01

    Adrenal necrosis, a rare life threatening complication of antiphospholipid syndrome, is difficult to diagnose during pregnancy. We report the case of a 33-year-old woman with bilateral adrenal necrosis which started during the third trimester of her second pregnancy. Antiphospholipid syndrome had been diagnosed few years ago, after a thrombotic event. The pregnancy was uneventful until 36 weeks plus five days, when the patient was admitted for bilateral back ache, initially considered as uterine contractions. Labour was induced because pain persisted and was associated with major thrombocytopenia. A healthy infant was delivered vaginally on the second day, adrenal failure was diagnosed based on intense asthenia, persistent severe lumbar pain, low blood sodium and cortisol. Bilateral adrenal oedema was documented by CT scan and MRI. Symptoms resolved following administration of hydrocortisone and fludrocortisone. This case illustrates the difficulty to diagnose adrenal necrosis in the third trimester of pregnancy.

  7. Ovarian thecal metaplasia of the adrenal gland in association with Beckwith-Wiedemann syndrome

    Institute of Scientific and Technical Information of China (English)

    Eslam; Y; Wassal; Mouhammed; Amir; Habra; Rafael; Vicens; Priya; Rao; Khaled; M; Elsayes

    2014-01-01

    Beckwith-Wiedemann syndrome(BWS) is an overgrowth syndrome associated with increased risk to develop malignancies including adrenocortical carcinoma. Ovarian thecal metaplasia of the adrenal gland is a rare tumorlike mesenchymal lesion in BWS patients that lacks detailed radiological description. We report a 17-yearold female patient with BWS, associated with bilateral Wilms tumor, hepatic hemangiomatosis, pancreatic neuroendocrine tumor, and a phyllodes tumor of the right breast. Surveillance abdominal ultrasound identified a right adrenal mass that was further characterized by computed tomography and magnetic resonance imaging. Radiologically, this mass displayed features that overlap with adrenocortical carcinoma and pheochromocytoma but after pathological examination this proved to be an ovarian thecal metaplasia of the adrenal gland. Adrenal masses in BWS should raise the suspicion for adrenocortical carcinoma though other adrenal tumors including ovarian thecal metaplasia can be seen in these patients.

  8. Incidentally Detected Kaposi Sarcoma of Adrenal Gland with Anaplastic Features in an HIV Negative Patient

    Science.gov (United States)

    Celik, Murat; Sen, Erdem; Cebeci, Hakan; Ata, Ozlem; Yavas, Cagdas

    2016-01-01

    Kaposi sarcoma (KS), a vascular tumor caused by infection with human herpesvirus 8 (HHV8), is a systemic disease that can present with cutaneous lesions with or without visceral involvement. Very few cases of KS, most of which were associated with AIDS, have been reported in the adrenal gland. Anaplastic transformation of KS is a rare clinical presentation known as an aggressive disease with local recurrence and metastatic potential. We report here a 47-year-old HIV negative male presented with extra-adrenal symptoms and had an incidentally detected anaplastic adrenal KS exhibited aggressive clinical course. To the best of our knowledge, this is the first case of anaplastic primary adrenal KS without mucocutaneous involvement but subsequently developed other side adrenal metastases in an HIV negative patient.

  9. Chronic stress induces adrenal hyperplasia and hypertrophy in a subregion-specific manner.

    Science.gov (United States)

    Ulrich-Lai, Yvonne M; Figueiredo, Helmer F; Ostrander, Michelle M; Choi, Dennis C; Engeland, William C; Herman, James P

    2006-11-01

    The adrenal gland is an essential stress-responsive organ that is part of both the hypothalamic-pituitary-adrenal axis and the sympatho-adrenomedullary system. Chronic stress exposure commonly increases adrenal weight, but it is not known to what extent this growth is due to cellular hyperplasia or hypertrophy and whether it is subregion specific. Moreover, it is not clear whether increased production of adrenal glucocorticoid after chronic stress is due to increased sensitivity to adrenocorticotropic hormone (ACTH) vs. increased maximal output. The present studies use a 14-day chronic variable stress (CVS) paradigm in adult male rats to assess the effects of chronic stress on adrenal growth and corticosterone steroidogenesis. Exogenous ACTH administration (0-895 ng/100 g body wt) to dexamethasone-blocked rats demonstrated that CVS increased maximal plasma and adrenal corticosterone responses to ACTH without affecting sensitivity. This enhanced function was associated with increased adrenal weight, DNA and RNA content, and RNA/DNA ratio after CVS, suggesting that both cellular hyperplasia and hypertrophy occurred. Unbiased stereological counting of cells labeled for Ki67 (cell division marker) or 4,6-diamidino-2-phenylindole (nuclear marker), combined with zone specific markers, showed that CVS induced hyperplasia in the outer zona fasciculata, hypertrophy in the inner zona fasciculata and medulla, and reduced cell size in the zona glomerulosa. Collectively, these results demonstrate that increased adrenal weight after CVS is due to hyperplasia and hypertrophy that occur in specific adrenal subregions and is associated with increased maximal corticosterone responses to ACTH. These chronic stress-induced changes in adrenal growth and function may have implications for patients with stress-related disorders.

  10. Chronic cardiac pressure overload induces adrenal medulla hypertrophy and increased catecholamine synthesis.

    Science.gov (United States)

    Schneider, Johanna; Lother, Achim; Hein, Lutz; Gilsbach, Ralf

    2011-06-01

    Increased activity of the sympathetic system is an important feature contributing to the pathogenesis and progression of chronic heart failure. While the mechanisms and consequences of enhanced norepinephrine release from sympathetic nerves have been intensely studied, the role of the adrenal gland in the development of cardiac hypertrophy and progression of heart failure is less well known. Thus, the aim of the present study was to determine the effect of chronic cardiac pressure overload in mice on adrenal medulla structure and function. Cardiac hypertrophy was induced in wild-type mice by transverse aortic constriction (TAC) for 8 weeks. After TAC, the degree of cardiac hypertrophy correlated significantly with adrenal weight and adrenal catecholamine storage. In the medulla, TAC caused an increase in chromaffin cell size but did not result in chromaffin cell proliferation. Ablation of chromaffin α(2C)-adrenoceptors did not affect adrenal weight or epinephrine synthesis. However, unilateral denervation of the adrenal gland completely prevented adrenal hypertrophy and increased catecholamine synthesis. Transcriptome analysis of microdissected adrenal medulla identified 483 up- and 231 downregulated, well-annotated genes after TAC. Among these genes, G protein-coupled receptor kinases 2 (Grk2) and 6 and phenylethanolamine N-methyltransferase (Pnmt) were significantly upregulated by TAC. In vitro, acetylcholine-induced Pnmt and Grk2 expression as well as enhanced epinephrine content was prevented by inhibition of nicotinic acetylcholine receptors and Ca(2+)/calmodulin-dependent signaling. Thus, activation of preganglionic sympathetic nerves innervating the adrenal medulla plays an essential role in inducing adrenal hypertrophy, enhanced catecholamine synthesis and induction of Grk2 expression after cardiac pressure overload.

  11. Alterations in prealbumin concentration after adrenal autotransplantation for Parkinson's disease.

    Science.gov (United States)

    Abram, S R; Kruskal, J B; Allen, G S; Burns, R S; Parker, R; Tulipan, N

    1990-05-01

    The cerebrospinal fluid of eight patients with Parkinson's disease who underwent adrenal medullary autotransplantation was analyzed using SDS-polyacrylamide gel electrophoresis. A protein, subsequently identified as prealbumin, was noted to change in concentration over the intraoperative to 18-month postoperative time course. The qualitative changes observed on visual inspection were confirmed and quantified using laser densitometry. The concentration of prealbumin increased by an average of 90% when the intraoperative and 12-month samples were compared. This increase persisted at 18 months. The ratio of prealbumin to albumin also increased from intraoperative to 12 months by an average of 56%. This suggests that the increases in PA are the result of choroid plexus activation rather than a nonspecific breakdown of the blood-brain barrier. Given the association of prealbumin with other nervous system diseases, as well as its known ability to bind multiple substances, these findings may have important implications. Alterations in prealbumin may be responsible for the improvement seen in some patients who receive adrenal medullary autotransplants. Alternatively, prealbumin may be implicated in the pathophysiology of Parkinson's disease.

  12. Impact of molecular genetics on congenital adrenal hyperplasia management.

    Science.gov (United States)

    Balsamo, A; Baldazzi, L; Menabò, S; Cicognani, A

    2010-09-01

    Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders caused by mutations in genes encoding the enzymes involved in one of the 5 steps of adrenal steroid synthesis or the electron donor P450 oxidoreductase (POR) enzyme. Steroid 21-hydroxylase deficiency (21-OHD), the principal focus of this review, accounts for about 90-95% of all CAH cases, and its biochemical and clinical severity depends on the underlying CYP21A2 gene disruption. Molecular genetic advancements have been achieved in recent years, and the aim of this review is to attempt to highlight its contribution to the comprehension and management of the disease. When possible, we will try to achieve this goal also by providing some results from our personal experience regarding: some aspects of CYP21A2 gene analysis, with basic genotype/phenotype relationships; its crucial role in both genetic counselling and in prenatal diagnosis and treatment in families at risk for 21-OHD; its help in the comprehension of the severity of the disease in patients diagnosed by neonatal screening and possibly treated before an evident salt-loss crisis or before performing adequate blood sampling; its usefulness in the definition of post ACTH 17-hydroxyprogesterone values, discriminating between non-classic, heterozygote and normal subjects; and finally the contribution of genes other than CYP21A2 whose function or dysfunction could influence 21-hydroxylase activity and modify the presentation or management of the disease.

  13. Executive functioning in children with congenital adrenal hyperplasia.

    Science.gov (United States)

    Agoston, A Monica; Gonzalez-Bolanos, Maria Teresa; Semrud-Clikeman, Margaret; Vanderburg, Nancy; Sarafoglou, Kyriakie

    2017-01-01

    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is a disorder characterized by impaired cortisol synthesis leading to excessive production of adrenal androgens. Prenatal and postnatal exposure to excess androgens may increase neural vulnerability to insult and affect cognitive functions, particularly dopamine-dependent neural circuits responsible for executive functioning (EF). Our study aimed to investigate relationship between more pronounced androgen exposure and EF-related behaviors in children with CAH, as well as sex differences in these associations. Parents of patients with CAH (n=41, boys=17, girls=24; age: M=8.41, SD=4.43) completed the Behavior Rating Inventory of Executive Function (BRIEF), a measure assessing behavioral manifestations of EF. Assessments of bone age advancement, a proxy of cumulative androgen exposure, were analyzed. Advanced bone age predicted more inhibition difficulties in boys but not in girls, and more difficulties in all other BRIEF domains in the total sample. Excessive androgen production affected EF such that more advanced bone age led to more EF-related difficulties. Sex differences in inhibition may result from estrogen exposure moderating the impact of androgens in girls but not in boys. Future interventions may include targeting EF in patients with CAH to enhance quality of life and reduce cognitive consequences associated with this disease.

  14. Enabling secure, distributed collaborations for adrenal tumor research.

    Science.gov (United States)

    Stell, Anthony; Sinnott, Richard; Jiang, Jipu

    2010-01-01

    Many e-Health strategies rely on the secure integration of datasets that have previously resided in isolated locations, but can now in principle be accessed over the Internet. Of paramount importance in the health domain is the need for the security and privacy of data that is transmitted across these networks. One such collaboration, which spans several specialist centres across France, Germany, Italy and the UK, is ENSAT - the European Network for the Study of Adrenal Tumors. The rarity of the tumors under study means the value of accessing, aggregating and comparing data from many centres is great indeed. However this is especially challenging given that ENSAT require clinical and genomic data to be seamlessly linked, but in such a way that the information governance, ethics and privacy concerns of the patients and associated stakeholders involved are visibly satisfied. Key to this is the clear separation of clinical and genomic data sets and support for rigorous patient-identity protecting access control. This is especially challenging when such data sets exist across different organisational boundaries. In this paper we describe a prototype solution offering a security-oriented tailored portal supported by a layered encryption-driven linkage technology (VANGUARD) that offers precisely such patient-privacy protecting capabilities. We describe the architecture, implementation and use to date of this facility to support the ENSAT adrenal cancer research network.

  15. Small bowel adenocarcinoma mimicking a large adrenal tumor

    Directory of Open Access Journals (Sweden)

    Ivović Miomira

    2013-01-01

    Full Text Available Introduction. Adenocarcinoma of the small bowel is a rare gastrointestinal neoplasm usually affecting the distal duodenum and proximal jejunum. Because of their rarity and poorly defined abdominal symptoms, a correct diagnosis is often delayed. Case Outline. We present a 43-year-old woman admitted at the Clinic for Endocrinology due to a large tumor (over 7 cm of the left adrenal gland. The tumor was detected by ultrasound and confirmed by CT scan. The patient complained of abdominal pain in the left upper quadrant, fatigue and septic fever. Normal urinary catecholamines excluded pheochromocytoma. The endocrine evaluations revealed laboratory signs of subclinical hypercorticism: midnight cortisol 235 nmol/L, post 1 mg - overnight Dexamethasone suppression test for cortisol 95.5 nmol/L and basal ACTH 4.2 pg/mL. Plasma rennin activity and aldosterone were within the normal range. Surgery was performed. Intraoperative findings showed signs of acute peritonitis and a small ulceration of the jejunum below at 70 cm on the anal side from the Treitz’s ligament. Adrenal glands were not enlarged. Patohistology and immunochemistry identified adenocarcinoma of the jejunum without infiltration of the lymphatic nodules. The extensive jejunal resection and lavage of the peritoneum were performed. Due to complications of massive peritonitis, the patient died seven days after surgery. Conclusion. Poorly defined symptoms and a low incidence make the diagnosis of small bowel carcinoma, particularly of the jejunal region, very difficult in spite of the new endoscopic techniques.

  16. Hyponatraemia secondary to nivolumab-induced primary adrenal failure

    Directory of Open Access Journals (Sweden)

    Harris Trainer

    2016-11-01

    Full Text Available Checkpoint inhibitors, such as ipilimumab and pembrolizumab, have transformed the prognosis for patients with advanced malignant melanoma and squamous non-small-cell lung cancer, and their use will only expand as experience is gained in a variety of other malignancies, for instance, renal and lymphoma. As the use of checkpoint inhibitors increases, so too will the incidence of their unique side effects, termed immune-related adverse events (irAEs, which can affect dermatological, gastrointestinal, hepatic, endocrine and other systems. Nivolumab is a monoclonal antibody that blocks the human programmed death receptor-1 ligand (PD-L1 found on many cancer cells and is licensed for the treatment of advanced malignant melanoma. We describe the first case of nivolumab-induced adrenalitis resulting in primary adrenal failure presenting with hyponatraemia in a 43-year-old man with malignant melanoma. The case highlights the potentially life-threatening complications of checkpoint inhibitors and the need for patient education and awareness of irAEs among the wider clinical community because such side effects require prompt recognition and treatment.

  17. Safety and tolerability of voriconazole in patients with baseline renal insufficiency and candidemia.

    NARCIS (Netherlands)

    Oude Lashof, A.M.L.; Sobel, J.D.; Ruhnke, M.; Pappas, P.G.; Viscoli, C.; Schlamm, H.T.; Rex, J.H.; Kullberg, B.J.

    2012-01-01

    Acutely ill patients with candidemia frequently suffer from renal insufficiency. Voriconazole's intravenous formulation with sulfobutylether beta-cyclodextrin (SBECD) is restricted in patients with renal insufficiency. We evaluated the use of intravenous voriconazole formulated with SBECD in candide

  18. Evaluation of adrenal function and hormone replacement in patients with pituitary adenoma after surgery%垂体瘤患者术后肾上腺功能与激素替代的评估

    Institute of Scientific and Technical Information of China (English)

    王琳; 李强

    2016-01-01

    Objective Surgical treatment is the first choice for treatment in pituitary adenomas except for prolactinomas�In part of patients after operation would occur hypopituitarism,which with acute adrenal crisis is the most serious, and chronic adrenal insufficiency is different degree affects the patient's quality of life�Therefore,it is very important to accurately assess the hypothalamic pituitary adrenal( HPA) axis in patients with pituitary adenoma�But there is still a lack of reliable methods for evaluating adrenal axis�We reviewed the harm of adrenal insufficiency,HPA axis evaluation method of the new progress and postoperative glucocorticoid replacement treatment of new ideas, cleared the importance of reassessment and regular follow⁃up and the development of individual programs.%手术是除泌乳素瘤外的垂体瘤的首选治疗方案,部分患者在术后可发生垂体功能减退,其中以急性肾上腺危象最为严重,而慢性肾上腺功能低下则不同程度的影响着患者的生活质量,因此术后对下丘脑⁃垂体⁃肾上腺( HPA)轴进行准确的评估对垂体瘤术后患者的合理治疗显得至关重要,但目前尚缺乏对肾上腺轴进行评估的可靠方法。本文综述了肾上腺功能低下的危害、HPA轴评估方法的新进展以及术后糖皮质激素替代治疗的新观念,明确了术后重新评估和定期随访以及制定个体化方案的重要性。

  19. Impact of insufficient sleep on total daily energy expenditure, food intake, and weight gain

    OpenAIRE

    Markwald, Rachel R.; Edward L. Melanson; Smith, Mark R.; Higgins, Janine; Perreault, Leigh; Eckel, Robert H.; Wright, Kenneth P.

    2013-01-01

    Insufficient sleep is associated with obesity, yet little is known about how repeated nights of insufficient sleep influence energy expenditure and balance. We studied 16 adults in a 14- to 15-d-long inpatient study and quantified effects of 5 d of insufficient sleep, equivalent to a work week, on energy expenditure and energy intake compared with adequate sleep. We found that insufficient sleep increased total daily energy expenditure by ∼5%; however, energy intake—especially at night after ...

  20. Adrenal function evaluation using ACTH stimulation test in children with sepsis and septic shock%促肾上腺皮质激素刺激对脓毒症及脓毒性休克患儿肾上腺功能评估的意义

    Institute of Scientific and Technical Information of China (English)

    张育才; 章赛吉; 滕国良; 田国力; 徐梁; 曹瑞芬; 张宇鸣

    2008-01-01

    目的 探讨小剂量(1μg/1.73 m2)促肾上腺皮质激素(ACTH)刺激实验评估儿童脓毒症和脓毒性休克肾上腺功能状态的价值.方法 患儿入院24h内完成基础皮质醇(T0)测定,静脉注射1μg/1.73m2 ACTH,30 min后测定血液皮质醇(T1),根据T0和皮质醇增值(△max=T1-T0)判断肾上腺功能,以△max≤90μg/L为肾上腺功能障碍(AI)指标.结果 62例中,脓毒症53例,脓毒性休克9例,病死率为27.4%(17/62).肾上腺功能障碍(adrenal insufficiency,AI)发生率40.3%(25/62),其中脓毒症和脓毒性休克患儿AI发生率分别是39.6%和44.4%,差异无显著统计学意义(P>0.05).两组脓毒症和脓毒性休克平均T0和T1分别是(318.6±230.4)μg/L、(452.3±230.7)μg/L和(454.7±212.7)μg/L、(579.3±231.9)μg/L,差异无统计学意义(P>0.05).存活组和死亡组患儿T0、T1分别是(320.5±223.9)μg/L、(462.3±212.0)μg/L和(384.3±258.3)μg/L、(500.7±470.6)μg/L,两组AI发生率分别是37.8%和47.1%,差异无统计学意义(P>0.05).T0和T1水平与儿童危重病例评分(PCIS)有关(P0.05).结论 儿童脓毒症和脓毒性休克患儿AI发生率较高.小剂量ACTH刺激实验可以判断严重感染患者肾上腺功能,可为激素治疗提供依据.%Objective Sepsis and septic shock remain a common problem that results in significant mortality and morbidity in pediatric intensive care units(PICU).According to literature,the use of more physiologic steroid replacement therapy is associated with hemodynamic and survival benefits in adult patients with relative adrenal insufficiency (RAI) and catecholamine-resistant septic shock.But little information is available in children.The aim of the current prospective study was to determine the prevalence of adrenal insufficiency in children with sepsis and septic shock using a low-dose adrenocorticotropic hormone(ACTH)stimulation test (1μg/1.73 m2) in children.Methods The authors performed cortisol estimation at baseline and after low-dose (1μg/1