BACKGROUND Addison’s disease(AD)is a rare but potentially fatal disease in Western countries,which can easily be misdiagnosed at an early stage.Severe adrenal tuberculosis(TB)may lead to depression in patients.CASE S...BACKGROUND Addison’s disease(AD)is a rare but potentially fatal disease in Western countries,which can easily be misdiagnosed at an early stage.Severe adrenal tuberculosis(TB)may lead to depression in patients.CASE SUMMARY We report a case of primary adrenal insufficiency secondary to adrenal TB with TB in the lungs and skin in a 48-year-old woman.The patient was misdiagnosed with depression because of her depressed mood.She had hyperpigmentation of the skin,nails,mouth,and lips.The final diagnosis was adrenal TB that resulted in the insufficient secretion of adrenocortical hormone.Adrenocortical hormone test,skin biopsy,T cell spot test of TB,and adrenal computed tomography scan were used to confirm the diagnosis.The patient’s condition improved after hormone replacement therapy and TB treatment.CONCLUSION Given the current status of TB in high-burden countries,outpatient doctors should be aware of and pay attention to TB and understand the early symptoms of AD.展开更多
Rationale:Adrenal histoplasmosis can present as bilateral adrenal enlargement with constitutional symptoms and/or adrenal insufficiency.Often these patients are initially investigated as secondary adrenal metastases b...Rationale:Adrenal histoplasmosis can present as bilateral adrenal enlargement with constitutional symptoms and/or adrenal insufficiency.Often these patients are initially investigated as secondary adrenal metastases before eventually their diagnosis established by histopathological examination of the adrenal tissues.Patients concerns:We report 4 cases of patients aged 55-78 who presented with hypocortisolism and bilateral adrenal masses.Diagnoses:Bilateral adrenal histoplasmosis.Interventions:Three patients received antifungal treatment for at least one year while 1 patient passed away before the initiation of antifungal treatment.Outcomes:Two patients’adrenal function recovered not requiring any steroid replacement,while 1 patient still requires long-term steroid replacement.Lessons:Adrenal histoplasmosis should be excluded in patients from endemic areas presenting with bilateral adrenal masses and adrenal insufficiency.Usually patients have an exposure to bird droppings,bat guano or soil contaminated with histoplasmosis fungi.Histopathological examination of biopsied adrenal tissues is still the gold standard investigation as serology and molecular diagnostic methods are still not widely available in most centers in Malaysia.展开更多
Objective:To evaluate the epidemiological,demographic,clinical features,treatment approaches,and survival of patients followed up for adrenal incidentaloma.Methods:Data from 46 patients who were treated and followed u...Objective:To evaluate the epidemiological,demographic,clinical features,treatment approaches,and survival of patients followed up for adrenal incidentaloma.Methods:Data from 46 patients who were treated and followed up due to adrenal incidentaloma in the Endocrinology Department of Mersin University Health Research and Application Hospital between 2010 and 2014 were retrospectively analyzed.Results:Of the cases included in the study,13 were male,33 were female,and the mean age was 54.09±10.7 years.The most common reason for admission was abdominal pain in 34.78%of the patients,the most commonly diagnosed radiological method was dynamic adrenal CT in 60.87%,and the most common location was the left adrenal gland.The mean lesion diameter was between 26.8±16.5 mm.The frequency of hypertension was 50%,obesity 47.8%,type 2 diabetes 21.7%,osteoporosis 42.8%,and metabolic syndrome 41.3%.According to hormonal evaluation results,non-functional adrenal adenoma(NFAA)was found in 82.61%,subclinical Cushing’s syndrome(SCS)in 15.21%,and aldosteronoma in 2.1%.Myelolipoma,pheochromocytoma,and adrenocortical adenoma were diagnosed in 8 cases undergoing adrenalectomy.One patient died due to liver failure.No hormonal activation or growth in lesion size was detected during the follow-up of the patients.Conclusion:Due to the very different pathological and radiological appearances of adrenal incidentaloma,it is important to evaluate demographic,etiological,clinical,laboratory,and radiological data as a whole in the treatment and follow-up.展开更多
BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in ...BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.展开更多
Background: The suppression of the hypothalamic-pituitary-adrenal axis by cortisol-secreting adrenocortical tumors is well recognized and requires peri- and postoperative hydrocortisone substitution. Case Presentation...Background: The suppression of the hypothalamic-pituitary-adrenal axis by cortisol-secreting adrenocortical tumors is well recognized and requires peri- and postoperative hydrocortisone substitution. Case Presentation: A 48-year-old female patient with hypertension and progressive weight gain, the clinical signs of hypercorticism motivated a hormonal workup revealing an independent ACTH Cushing’s syndrome: with urinary free cortisol (UFC) at 649 nmol/24h (4× normal), adrenocorticotropin hormone (ACTH) at 1.5 ng/l. The rest of the hormonal workup was not performed due to a lack of financial means. An Adrenal CT scan showed a 4 cm right adrenal adenoma. The patient underwent a right adrenalectomy with an adrenal adenoma on pathological examination. The contralateral side was normal. The patient was treated with hydrocortisone 30 mg/d for 6 weeks then 15 mg/d, during the monitoring we noted a persistence of the adrenal insufficiency for now 4 years. Basal cortisol levels during follow-up were very low (<3 μg/dl) ruling out the need for synacthen stimulation tests. Conclusion: Adrenal cortisol tumors are recognized by suppression, the duration of hypothalamic-pituitary-adrenal axis suppression is variable from 11 to 60 months depending on the series, which depends on the duration, severity of hypercortisolism, tumor size and other unknown factors. A longer follow-up of these patients is necessary to look for recovery of the contralateral adrenal gland.展开更多
Antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) are frequently discussed together and considered two closely related diseases.APS involves multiple organ systems,but APS-related endocrine manife...Antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) are frequently discussed together and considered two closely related diseases.APS involves multiple organ systems,but APS-related endocrine manifestations are rare.^([1])Among them,adrenal insufficiency (AI) is the first endocrine manifestation of APS.The prompt diagnosis of AI is critical,as this disorder has high morbidity and mortality if left untreated.Here,we report a rare case of acute AI caused by APS secondary to SLE.CASEIn August 2022,a 39-year-old woman without a significant family history of medical diseases presented to our emergency department (ED) for low back pain,nausea,anorexia,fatigue,hypoglycemia,and hypotension.One month prior,she had fatigue,loss of appetite,low back pain,cough with sputum.展开更多
The adrenal gland secretes a variety of hormones that regulate physiological functions,and adrenal lesions can lead to insidious or sudden onset diseases that are easily misdiagnosed.Recently,several young patients wi...The adrenal gland secretes a variety of hormones that regulate physiological functions,and adrenal lesions can lead to insidious or sudden onset diseases that are easily misdiagnosed.Recently,several young patients with unknown sudden and refractory cardiogenic shock or cardiac arrest(CA)were successfully treated in our emergency department by the application of venoarterial extracorporeal membrane oxygenation(VA-ECMO).These life-threatening conditions were finally determined to be caused by adrenal lesions,which highlight the bridging role of VA-ECMO.[1]展开更多
Introduction: Surgical management of adrenal tumors has greatly improved over the past years, with laparoscopic adrenalectomy being the gold standard. However, Open adrenalectomy is indicated in large adrenal tumors, ...Introduction: Surgical management of adrenal tumors has greatly improved over the past years, with laparoscopic adrenalectomy being the gold standard. However, Open adrenalectomy is indicated in large adrenal tumors, malignant tumors and large phaeochromocytomas. We report surgical outcomes of 18 cases of functional adrenal tumors from 2007 to 2022. Methods: We conducted a retrospective cross-sectional and descriptive study in three tertiary hospitals in Yaounde, Cameroon. We reviewed files of patients who underwent adrenalectomy over a period of 15 years from July 2007 to July 2022. Clinical and diagnostic components of adrenal tumors, indications and surgical outcomes were analyzed. Results: A total of 18 patients were included in our study. The average age of patients was 38.33 years, with a female-to-male sex ratio of 2:1. Weight gain (72.2%) was the most represented clinical sign. The secretory nature of tumor and malignancy represented 55.5% and 33.3% of the operative indications and all 18 (100%) of the patients had open adrenalectomy. Vascular injury was the most common intraoperative complication with 5.63%, while acute adrenal insufficiency (16.7%) was the most common post-operative complication. The average tumor size was 6.22 cm and the mean duration of hospitalization was 11.61 days. Adenoma 7 (38.9%) and adrenocortical carcinoma 5 (27.8%) were the frequent histological types. One patient died two months post-surgery from anemia-related complications. Conclusion: The success of adrenal surgery is linked to multidisciplinary patient care and the experience of the surgeon. Conventional surgery still has indications with satisfactory short- and medium-term results in our context.展开更多
Purpose: Data on microarray gene expression The Gene Expression Omnibus (GEO) provided information on gene expression. Transcription GEO provided two profiles of human NK cells from breast and adrenal tumors (GSE17950...Purpose: Data on microarray gene expression The Gene Expression Omnibus (GEO) provided information on gene expression. Transcription GEO provided two profiles of human NK cells from breast and adrenal tumors (GSE179509 and GSE143383). Data processing and normalization The Dseq2 tool in the R programming language was used to standardize the raw data from GEO. The following analyses were carried out: fold change and P-value analysis, volcano plot, network analysis, GEPIA, and David pathway analysis. In this paper, using Venny software, we discovered 2 genes that are shared by neurotransmitters and NK cells in breast cancer and adrenal cancer. Between these genes and the pathways, they are a part of, we discovered a network. Pathway analysis revealed that these genes are mostly linked to the neurotransmitter and apoptotic pathways. In breast and adrenal tumors, the genes HRH1 and GABRD were discovered to be connected to NK cells. In response to breast and adrenal tumors, almost all of these genes are effective. It is thus postulated that the diagnosis of breast and adrenal cancer may be affected by the up-or down-regulation of these genes. Methods: Microarray gene expression data gene expression data was obtained from the Gene Expression Omnibus (GEO) Transcription 2 profile data of human NK cells from human breast and adrenal cancers were obtained from GEO (GSE179509 and GSE143383). Processing and normalization of data the raw data from GEO were normalized with the Dseq2 package in the R software. Fold change and P value analysis, Volcano plot, network analysis, GEPIA, and David pathway analysis were performed. Results: In this article, we found genes common to neurotransmitters with NK cells in adrenal cancer and breast cancer with Venny program, resulting in 2 genes. We identified a network between these genes and pathways they belong to. Pathway analysis showed that these genes are mostly associated with apoptosis and neurotransmitters pathway. Conclusion: HRH1 and GABRD genes were found to be associated with NK cells in breast and adrenal cancers. Almost all these genes are effective in response to breast and adrenal cancers. Therefore, it is hypothesized that downregulation or upregulation of these genes may affect breast and adrenal cancer diagnosis.展开更多
The occurrence of adrenal crisis after retroperitoneal laparoscopic unilateral adrenalectomy is usually concealed.If not timely diagnosis and treatment,it may cause shock,and even lead to death.It is very difficult to...The occurrence of adrenal crisis after retroperitoneal laparoscopic unilateral adrenalectomy is usually concealed.If not timely diagnosis and treatment,it may cause shock,and even lead to death.It is very difficult to distinguish the clinical manifestations of adrenal crisis from nausea,vomiting,fatigue,gas separation from the lower diaphragm,abdominal pain,hypotension,hypertension,fever and hypothermia after operation.This makes it very difficult to identify and diagnose adrenal crisis early.This article mainly discusses the early recognition,diagnosis and treatment of adrenal crisis after unilateral adrenalectomy by retroperitoneoscope.展开更多
Cushing’s syndrome is the set of clinical manifestations secondary to a chronic excess of glucocorticoids. Bilateral macronodular adrenal hyperplasia with subclinical cortisol secretion is the most common, but its pr...Cushing’s syndrome is the set of clinical manifestations secondary to a chronic excess of glucocorticoids. Bilateral macronodular adrenal hyperplasia with subclinical cortisol secretion is the most common, but its prevalence remains unknown. We describe a case of bilateral macronodular adrenal hyperplasia. This is a 36-year-old female patient who had been consulting for secondary amenorrhea and developing asthenia for 4 months. The clinical examination noted an overweight patient with high blood pressure, facio-trunk obesity, hirsutism and purple stretch marks in the abdomen and thighs. Biologically, hypokalemia at 2.9 meq/l (3.5 - 5.4), normal calcemia at 90 mg/l (85 - 104), fasting blood sugar was 0.84 g/l (0.7 - 1), the tests for minute, low and high dexamethasone suppression test revealed insufficient suppression of cortisol. The cortisoluria collected from the second day to the third day of the high dexamethasone suppression test was at 186 μg/24 h (<60), the ACTH (Pg/ml) was undetectable (6.4 - 49.8). The diagnosis of an independent adrenocorticotrophin (ACTH) Cushing syndrome was made and the adrenal CT scan revealed bilateral macronodular hyperplasia. A bilateral adrenalectomy was performed and a complete remission of Cushing syndrome was achieved. We prescribed to her, hydrocortisone 20 mg/day and alpha-fludrocortisone 25 to 50 μg/day, This medical observation showed that macro-nodular adrenal hyperplasia with overt Cushing syndrome can occur in the third decade of life. Bilateral adrenalectomy has resulted in a complete cure for Cushing’s syndrome, but may be fraught with complications.展开更多
Background:Although guidelines recommend systemic therapy even in patients with limited extrahepatic metastases from hepatocellular carcinoma(HCC),a few recent studies suggested a potential benefit for resection of ex...Background:Although guidelines recommend systemic therapy even in patients with limited extrahepatic metastases from hepatocellular carcinoma(HCC),a few recent studies suggested a potential benefit for resection of extrahepatic metastases.However,the benefit of adrenal resection(AR)for adrenal-only metastases(AOM)from HCC was not proved yet.This is the first study to compare long-term outcomes of AR to those of sorafenib in patients with AOM from HCC.Methods:The patients with adrenal metastases(AM)from HCC were identified from the electronic records of the institution between January 2002 and December 2018.Those who presented AM and other sites of extrahepatic disease were excluded.Furthermore,the patients with AOM who received other therapies than AR or sorafenib were excluded.Results:A total of 34 patients with AM from HCC were treated.Out of these,22 patients had AOM,6 receiving other treatment than AR or sorafenib.Eventually,8 patients with AOM underwent AR(AR group),while 8 patients were treated with sorafenib(SOR group).The baseline characteristics of the two groups were not significantly different in terms of age,sex,number and size of the primary tumor,timing of AM diagnosis,Child-Pugh and ECOG status.After a median follow-up of 15.5 months,in the AR group,the 1-,3-,and 5-year overall survival rates(85.7%,42.9%,and 0%,respectively)were significantly higher than those achieved in the SOR group(62.5%,0%and 0%at 1-,3-and 5-year,respectively)(P=0.009).The median progression-free survival after AR(14 months)was significantly longer than that after sorafenib therapy(6 months,P=0.002).Conclusions:In patients with AOM from HCC,AR was associated with significantly higher overall and progression-free survival rates than systemic therapy with sorafenib.These results could represent a starting-point for future phase II/III clinical trials.展开更多
BACKGROUND Adrenal tuberculosis usually presents with bilateral involvement.It has special characteristics in computed tomography(CT)images,such as small size,low attenuation in the center,and peripheral rim enhanceme...BACKGROUND Adrenal tuberculosis usually presents with bilateral involvement.It has special characteristics in computed tomography(CT)images,such as small size,low attenuation in the center,and peripheral rim enhancement,which differ from those of primary tumors.CASE SUMMARY A 42-year-old female presented to the hospital with low back pain.She had been diagnosed with hypertension as well as pulmonary and cerebral tuberculosis but denied having any fever,fatigue,anorexia,night sweats,cough,or weight loss.Abdominal CT revealed an irregular 6.0 cm×4.5 cm mass with uneven density in the right adrenal gland,while the left adrenal gland was normal.No abnormalities were observed in plasma total cortisol(8 am),adrenocorticotropic hormone,aldosterone/renin ratio,blood catecholamines,or urine catecholamines.A fineneedle aspiration biopsy of the right adrenal gland provided evidence of tuberculosis.After three years of anti-tuberculosis treatments,the large mass in the right adrenal gland was reduced to a slight enlargement.CONCLUSION This is a case of unilateral adrenal tuberculosis with CT imaging characteristics mimicking those of a malignant tumor.Extended anti-tuberculosis therapy is recommended in such cases.展开更多
BACKGROUND Adrenal myelolipoma is a rare,benign,non-functioning mass that occurs in the adrenal gland.It is composed of an admixture of hematopoietic elements and mature adipose tissue,similar to bone marrow.Even at l...BACKGROUND Adrenal myelolipoma is a rare,benign,non-functioning mass that occurs in the adrenal gland.It is composed of an admixture of hematopoietic elements and mature adipose tissue,similar to bone marrow.Even at large sizes,adrenal myelolipomas are usually asymptomatic and often incidentally found by ultrasonography or computed tomography(CT)scan.This paper describes an unusual case of adrenal myelolipoma presenting as flank pain.CASE SUMMARY A 50-year-old male with severe right flank pain underwent a CT scan revealing a huge mass extending into the suprarenal space.The mass showed a fat component with retroperitoneal hemorrhage.The tumor was treated laparoscopically,and pathologic examination revealed features of myelolipoma originating from the adrenal gland.CONCLUSION Adrenal myelolipomas are generally asymptomatic and can be treated conservatively.However,rupture and hemorrhage of the tumor can cause symptoms requiring surgical removal.展开更多
BACKGROUND Congenital adrenal hyperplasia(CAH),which is caused by a mutation of the steroidogenic acute regulatory(StAR)gene.Affected patients are usually characterized by adrenal insufficiency in the first year of li...BACKGROUND Congenital adrenal hyperplasia(CAH),which is caused by a mutation of the steroidogenic acute regulatory(StAR)gene.Affected patients are usually characterized by adrenal insufficiency in the first year of life,salt loss,glucocorticoid and mineralocorticoid deficiency,and female external genitalia,regardless of chromosomal karyotype.Patients with non-classical lipoid CAH usually develop glucocorticoid deficiency and mild mineralocorticoid deficiency at 2-4 years of age.CASE SUMMARY Herein,We report the case of a woman with non-classic lipoid CAH combined with Graves’disease.Her chromosome karyotype was 46,XX,and highthroughput sequencing revealed two missense variants in the StAR gene:c.229C>T(p.Q77X)and c.814C>T(p.R272C),which were inherited from both parents(non-close relatives).The patient was treated for Graves’disease in a timely manner and the dosage of glucocorticoid was adjusted during the treatment of Graves’disease.CONCLUSION This is the first case of non-classic lipoid CAH combined with Graves’disease reported in the Chinese population.In addition to conventional glucocorticoid replacement therapy,timely adjustments were made to the dosages of thyroid hormone and glucocorticoid to avoid adrenal crisis as a consequence of the increased demand and accelerated metabolism of glucocorticoids when the patient was diagnosed with Graves’disease.展开更多
We present 7 adults atopic asthmatics that were referred due to repeatedly relapsing asthma requiring oral courses of prednisolone. All were steroid responsive yet steroid dependant and were screened for adrenal insuf...We present 7 adults atopic asthmatics that were referred due to repeatedly relapsing asthma requiring oral courses of prednisolone. All were steroid responsive yet steroid dependant and were screened for adrenal insufficiency after changes to inhaled therapy and other standard anti-asthma treatments failed to improve the situation. All were deemed to be compliant. 4 used long-term intranasal steroids in addition to inhaled corticosteroids. No topical steroid creams were used by these patients. Adrenal suppression was examined via a 9 am cortisol level and a short synacthen test (using intravenous tetracosactide 250 mcg) along with measurement of ACTH (Adreno-Cortico-Trophic Hormone). The tests were performed in periods off prednisolone. We report the observed beneficial effects after treatment of the adrenal insufficiency with hydrocor-tisone acetate replacement therapy in these cases, and the benefit to their exacerbations, hospital admission and the requirement for prednisolone courses. These patients would have been stepped up to yet higher doses of inhaled steroids and even referred for anti-IgE treatment etc. Adrenal suppression is well described in the medical literature for asthmatics, but the beneficial outcomes of treating this are unknown. These 7 cases have been followed for up to 3 years since hydrocorti-sone replacement therapy. Most improved to a stable asthma without frequent exacerbations nor requirement for prednisolone and only 1 had a hospital admission.展开更多
Numerous surgical modalities are available to treat adrenal lesions. Minimally-invasive approaches for adrenalectomy are indicated in most circumstances, and new evidence continues to be accumulated. In this context, ...Numerous surgical modalities are available to treat adrenal lesions. Minimally-invasive approaches for adrenalectomy are indicated in most circumstances, and new evidence continues to be accumulated. In this context, current indications for open surgical adrenalectomy(OS-A), minimally-invasive adrenalectomy(MI-A), and laparoendoscopic single-site adrenalectomy(LESS-A) remain unclear. A comprehensive Englishlanguage literature review was performed using MEDLINE/Pub MED to identify articles and guidelines pertinent to the surgical management of adrenal tumors. A comprehensive chart review was performed for three illustrative cases. Clinical recommendations were generated based on relevant literature and the expertise of the investigator group. MI-A offers advantages over OS-A in properly selected patients, who experience fewer complications, lower blood loss, and shorter hospital stays. Robot-assisted laparoscopic and retroperitoneoscopic adrenalectomy may offer advantages over transperitoneal surgery, and LESS-A may be an even less-invasive option that will require further evaluation. MI-A remains the surgical treatment of choice for most adrenal lesions. Tumor size and stage are the primary indications for selecting alternative treatment modalities. OS-A remains the gold standard for large tumors(> 10 cm) and suspected or known advanced stage malignancy. LESS-A appears to be an appropriate initial approach for small tumors(< 4-5 cm), including pheochromocytoma and isolated adrenal metastases.展开更多
Congenital Adrenal Hyperplasia is a group of autosomal recessive disorders resulting from deficiency of enzymes essential for the synthesis of cortisol.Disease of the adrenal cortex,but there may be involvement adreno...Congenital Adrenal Hyperplasia is a group of autosomal recessive disorders resulting from deficiency of enzymes essential for the synthesis of cortisol.Disease of the adrenal cortex,but there may be involvement adrenomedullary.Cortisol and epinephrine are directly related to the individual’s stress response.Lower values of epinephrine in children with congenital adrenal hyperplasia could be related to increased clinical complications and hospitalizations rate.We evaluated the serum values of metanephrines and normetanephrines in children and adolescents with classic congenital adrenal hyperplasia and primary hypothyroidism and possible correlations with disease and hospitalizations.Cross-sectional study involved 29 patients(10 simple virilizing and 19 salt-wasting),and control group of 28 patients with primary hypothyroidism(10 overt and 18 subclinical).There were no differences in age(p=0.24)and metanephrine(p=0.34)or normetanephrine values(p=0.85)between groups.Hospitalization rate was higher in the cases than in the controls(51 x 12).We conclude the serum values of metanephrine and normetanephrine in patients with congenital adrenal hyperplasia were within the normal values of reference,with no significant difference of group with primary hypothiroidism.The number of hospitalizations in the case was high in relation to the control,mainly in salt-wasting.展开更多
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 inappro...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 dif-ferent 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 existing published information regarding AI in patients with liver cirrhosis.展开更多
As one kind of infectious diseases of adrenal gland, adrenal tuberculosis can result in a life-threatening disorder which is called primary adrenal insufficiency(PAI) due to the destruction of adrenal cortex. Computed...As one kind of infectious diseases of adrenal gland, adrenal tuberculosis can result in a life-threatening disorder which is called primary adrenal insufficiency(PAI) due to the destruction of adrenal cortex. Computed tomography(CT) and magnetic resonance imaging(MRI) play significant roles in the diagnosis of this etiology of PAI based on the CT and MRI appearances of the adrenal lesions. In this mini-review, we intend to study the CT and MRI features of adrenal tuberculosis, which could be helpful to both endocrinologist and radiologist to establish a definitive diagnosis for adrenal tuberculosis resulting in PAI.展开更多
文摘BACKGROUND Addison’s disease(AD)is a rare but potentially fatal disease in Western countries,which can easily be misdiagnosed at an early stage.Severe adrenal tuberculosis(TB)may lead to depression in patients.CASE SUMMARY We report a case of primary adrenal insufficiency secondary to adrenal TB with TB in the lungs and skin in a 48-year-old woman.The patient was misdiagnosed with depression because of her depressed mood.She had hyperpigmentation of the skin,nails,mouth,and lips.The final diagnosis was adrenal TB that resulted in the insufficient secretion of adrenocortical hormone.Adrenocortical hormone test,skin biopsy,T cell spot test of TB,and adrenal computed tomography scan were used to confirm the diagnosis.The patient’s condition improved after hormone replacement therapy and TB treatment.CONCLUSION Given the current status of TB in high-burden countries,outpatient doctors should be aware of and pay attention to TB and understand the early symptoms of AD.
文摘Rationale:Adrenal histoplasmosis can present as bilateral adrenal enlargement with constitutional symptoms and/or adrenal insufficiency.Often these patients are initially investigated as secondary adrenal metastases before eventually their diagnosis established by histopathological examination of the adrenal tissues.Patients concerns:We report 4 cases of patients aged 55-78 who presented with hypocortisolism and bilateral adrenal masses.Diagnoses:Bilateral adrenal histoplasmosis.Interventions:Three patients received antifungal treatment for at least one year while 1 patient passed away before the initiation of antifungal treatment.Outcomes:Two patients’adrenal function recovered not requiring any steroid replacement,while 1 patient still requires long-term steroid replacement.Lessons:Adrenal histoplasmosis should be excluded in patients from endemic areas presenting with bilateral adrenal masses and adrenal insufficiency.Usually patients have an exposure to bird droppings,bat guano or soil contaminated with histoplasmosis fungi.Histopathological examination of biopsied adrenal tissues is still the gold standard investigation as serology and molecular diagnostic methods are still not widely available in most centers in Malaysia.
文摘Objective:To evaluate the epidemiological,demographic,clinical features,treatment approaches,and survival of patients followed up for adrenal incidentaloma.Methods:Data from 46 patients who were treated and followed up due to adrenal incidentaloma in the Endocrinology Department of Mersin University Health Research and Application Hospital between 2010 and 2014 were retrospectively analyzed.Results:Of the cases included in the study,13 were male,33 were female,and the mean age was 54.09±10.7 years.The most common reason for admission was abdominal pain in 34.78%of the patients,the most commonly diagnosed radiological method was dynamic adrenal CT in 60.87%,and the most common location was the left adrenal gland.The mean lesion diameter was between 26.8±16.5 mm.The frequency of hypertension was 50%,obesity 47.8%,type 2 diabetes 21.7%,osteoporosis 42.8%,and metabolic syndrome 41.3%.According to hormonal evaluation results,non-functional adrenal adenoma(NFAA)was found in 82.61%,subclinical Cushing’s syndrome(SCS)in 15.21%,and aldosteronoma in 2.1%.Myelolipoma,pheochromocytoma,and adrenocortical adenoma were diagnosed in 8 cases undergoing adrenalectomy.One patient died due to liver failure.No hormonal activation or growth in lesion size was detected during the follow-up of the patients.Conclusion:Due to the very different pathological and radiological appearances of adrenal incidentaloma,it is important to evaluate demographic,etiological,clinical,laboratory,and radiological data as a whole in the treatment and follow-up.
文摘BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.
文摘Background: The suppression of the hypothalamic-pituitary-adrenal axis by cortisol-secreting adrenocortical tumors is well recognized and requires peri- and postoperative hydrocortisone substitution. Case Presentation: A 48-year-old female patient with hypertension and progressive weight gain, the clinical signs of hypercorticism motivated a hormonal workup revealing an independent ACTH Cushing’s syndrome: with urinary free cortisol (UFC) at 649 nmol/24h (4× normal), adrenocorticotropin hormone (ACTH) at 1.5 ng/l. The rest of the hormonal workup was not performed due to a lack of financial means. An Adrenal CT scan showed a 4 cm right adrenal adenoma. The patient underwent a right adrenalectomy with an adrenal adenoma on pathological examination. The contralateral side was normal. The patient was treated with hydrocortisone 30 mg/d for 6 weeks then 15 mg/d, during the monitoring we noted a persistence of the adrenal insufficiency for now 4 years. Basal cortisol levels during follow-up were very low (<3 μg/dl) ruling out the need for synacthen stimulation tests. Conclusion: Adrenal cortisol tumors are recognized by suppression, the duration of hypothalamic-pituitary-adrenal axis suppression is variable from 11 to 60 months depending on the series, which depends on the duration, severity of hypercortisolism, tumor size and other unknown factors. A longer follow-up of these patients is necessary to look for recovery of the contralateral adrenal gland.
文摘Antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) are frequently discussed together and considered two closely related diseases.APS involves multiple organ systems,but APS-related endocrine manifestations are rare.^([1])Among them,adrenal insufficiency (AI) is the first endocrine manifestation of APS.The prompt diagnosis of AI is critical,as this disorder has high morbidity and mortality if left untreated.Here,we report a rare case of acute AI caused by APS secondary to SLE.CASEIn August 2022,a 39-year-old woman without a significant family history of medical diseases presented to our emergency department (ED) for low back pain,nausea,anorexia,fatigue,hypoglycemia,and hypotension.One month prior,she had fatigue,loss of appetite,low back pain,cough with sputum.
基金Ruiyi Emergency Medical Research Fund(2021-22)Science and Technology Innovation Program of Hunan Province(2020SK53707)。
文摘The adrenal gland secretes a variety of hormones that regulate physiological functions,and adrenal lesions can lead to insidious or sudden onset diseases that are easily misdiagnosed.Recently,several young patients with unknown sudden and refractory cardiogenic shock or cardiac arrest(CA)were successfully treated in our emergency department by the application of venoarterial extracorporeal membrane oxygenation(VA-ECMO).These life-threatening conditions were finally determined to be caused by adrenal lesions,which highlight the bridging role of VA-ECMO.[1]
文摘Introduction: Surgical management of adrenal tumors has greatly improved over the past years, with laparoscopic adrenalectomy being the gold standard. However, Open adrenalectomy is indicated in large adrenal tumors, malignant tumors and large phaeochromocytomas. We report surgical outcomes of 18 cases of functional adrenal tumors from 2007 to 2022. Methods: We conducted a retrospective cross-sectional and descriptive study in three tertiary hospitals in Yaounde, Cameroon. We reviewed files of patients who underwent adrenalectomy over a period of 15 years from July 2007 to July 2022. Clinical and diagnostic components of adrenal tumors, indications and surgical outcomes were analyzed. Results: A total of 18 patients were included in our study. The average age of patients was 38.33 years, with a female-to-male sex ratio of 2:1. Weight gain (72.2%) was the most represented clinical sign. The secretory nature of tumor and malignancy represented 55.5% and 33.3% of the operative indications and all 18 (100%) of the patients had open adrenalectomy. Vascular injury was the most common intraoperative complication with 5.63%, while acute adrenal insufficiency (16.7%) was the most common post-operative complication. The average tumor size was 6.22 cm and the mean duration of hospitalization was 11.61 days. Adenoma 7 (38.9%) and adrenocortical carcinoma 5 (27.8%) were the frequent histological types. One patient died two months post-surgery from anemia-related complications. Conclusion: The success of adrenal surgery is linked to multidisciplinary patient care and the experience of the surgeon. Conventional surgery still has indications with satisfactory short- and medium-term results in our context.
文摘Purpose: Data on microarray gene expression The Gene Expression Omnibus (GEO) provided information on gene expression. Transcription GEO provided two profiles of human NK cells from breast and adrenal tumors (GSE179509 and GSE143383). Data processing and normalization The Dseq2 tool in the R programming language was used to standardize the raw data from GEO. The following analyses were carried out: fold change and P-value analysis, volcano plot, network analysis, GEPIA, and David pathway analysis. In this paper, using Venny software, we discovered 2 genes that are shared by neurotransmitters and NK cells in breast cancer and adrenal cancer. Between these genes and the pathways, they are a part of, we discovered a network. Pathway analysis revealed that these genes are mostly linked to the neurotransmitter and apoptotic pathways. In breast and adrenal tumors, the genes HRH1 and GABRD were discovered to be connected to NK cells. In response to breast and adrenal tumors, almost all of these genes are effective. It is thus postulated that the diagnosis of breast and adrenal cancer may be affected by the up-or down-regulation of these genes. Methods: Microarray gene expression data gene expression data was obtained from the Gene Expression Omnibus (GEO) Transcription 2 profile data of human NK cells from human breast and adrenal cancers were obtained from GEO (GSE179509 and GSE143383). Processing and normalization of data the raw data from GEO were normalized with the Dseq2 package in the R software. Fold change and P value analysis, Volcano plot, network analysis, GEPIA, and David pathway analysis were performed. Results: In this article, we found genes common to neurotransmitters with NK cells in adrenal cancer and breast cancer with Venny program, resulting in 2 genes. We identified a network between these genes and pathways they belong to. Pathway analysis showed that these genes are mostly associated with apoptosis and neurotransmitters pathway. Conclusion: HRH1 and GABRD genes were found to be associated with NK cells in breast and adrenal cancers. Almost all these genes are effective in response to breast and adrenal cancers. Therefore, it is hypothesized that downregulation or upregulation of these genes may affect breast and adrenal cancer diagnosis.
文摘The occurrence of adrenal crisis after retroperitoneal laparoscopic unilateral adrenalectomy is usually concealed.If not timely diagnosis and treatment,it may cause shock,and even lead to death.It is very difficult to distinguish the clinical manifestations of adrenal crisis from nausea,vomiting,fatigue,gas separation from the lower diaphragm,abdominal pain,hypotension,hypertension,fever and hypothermia after operation.This makes it very difficult to identify and diagnose adrenal crisis early.This article mainly discusses the early recognition,diagnosis and treatment of adrenal crisis after unilateral adrenalectomy by retroperitoneoscope.
文摘Cushing’s syndrome is the set of clinical manifestations secondary to a chronic excess of glucocorticoids. Bilateral macronodular adrenal hyperplasia with subclinical cortisol secretion is the most common, but its prevalence remains unknown. We describe a case of bilateral macronodular adrenal hyperplasia. This is a 36-year-old female patient who had been consulting for secondary amenorrhea and developing asthenia for 4 months. The clinical examination noted an overweight patient with high blood pressure, facio-trunk obesity, hirsutism and purple stretch marks in the abdomen and thighs. Biologically, hypokalemia at 2.9 meq/l (3.5 - 5.4), normal calcemia at 90 mg/l (85 - 104), fasting blood sugar was 0.84 g/l (0.7 - 1), the tests for minute, low and high dexamethasone suppression test revealed insufficient suppression of cortisol. The cortisoluria collected from the second day to the third day of the high dexamethasone suppression test was at 186 μg/24 h (<60), the ACTH (Pg/ml) was undetectable (6.4 - 49.8). The diagnosis of an independent adrenocorticotrophin (ACTH) Cushing syndrome was made and the adrenal CT scan revealed bilateral macronodular hyperplasia. A bilateral adrenalectomy was performed and a complete remission of Cushing syndrome was achieved. We prescribed to her, hydrocortisone 20 mg/day and alpha-fludrocortisone 25 to 50 μg/day, This medical observation showed that macro-nodular adrenal hyperplasia with overt Cushing syndrome can occur in the third decade of life. Bilateral adrenalectomy has resulted in a complete cure for Cushing’s syndrome, but may be fraught with complications.
基金the Ethics Committee of Fundeni Clinical Institute,Bucharest,Romania(59425/3.12.2019).
文摘Background:Although guidelines recommend systemic therapy even in patients with limited extrahepatic metastases from hepatocellular carcinoma(HCC),a few recent studies suggested a potential benefit for resection of extrahepatic metastases.However,the benefit of adrenal resection(AR)for adrenal-only metastases(AOM)from HCC was not proved yet.This is the first study to compare long-term outcomes of AR to those of sorafenib in patients with AOM from HCC.Methods:The patients with adrenal metastases(AM)from HCC were identified from the electronic records of the institution between January 2002 and December 2018.Those who presented AM and other sites of extrahepatic disease were excluded.Furthermore,the patients with AOM who received other therapies than AR or sorafenib were excluded.Results:A total of 34 patients with AM from HCC were treated.Out of these,22 patients had AOM,6 receiving other treatment than AR or sorafenib.Eventually,8 patients with AOM underwent AR(AR group),while 8 patients were treated with sorafenib(SOR group).The baseline characteristics of the two groups were not significantly different in terms of age,sex,number and size of the primary tumor,timing of AM diagnosis,Child-Pugh and ECOG status.After a median follow-up of 15.5 months,in the AR group,the 1-,3-,and 5-year overall survival rates(85.7%,42.9%,and 0%,respectively)were significantly higher than those achieved in the SOR group(62.5%,0%and 0%at 1-,3-and 5-year,respectively)(P=0.009).The median progression-free survival after AR(14 months)was significantly longer than that after sorafenib therapy(6 months,P=0.002).Conclusions:In patients with AOM from HCC,AR was associated with significantly higher overall and progression-free survival rates than systemic therapy with sorafenib.These results could represent a starting-point for future phase II/III clinical trials.
文摘BACKGROUND Adrenal tuberculosis usually presents with bilateral involvement.It has special characteristics in computed tomography(CT)images,such as small size,low attenuation in the center,and peripheral rim enhancement,which differ from those of primary tumors.CASE SUMMARY A 42-year-old female presented to the hospital with low back pain.She had been diagnosed with hypertension as well as pulmonary and cerebral tuberculosis but denied having any fever,fatigue,anorexia,night sweats,cough,or weight loss.Abdominal CT revealed an irregular 6.0 cm×4.5 cm mass with uneven density in the right adrenal gland,while the left adrenal gland was normal.No abnormalities were observed in plasma total cortisol(8 am),adrenocorticotropic hormone,aldosterone/renin ratio,blood catecholamines,or urine catecholamines.A fineneedle aspiration biopsy of the right adrenal gland provided evidence of tuberculosis.After three years of anti-tuberculosis treatments,the large mass in the right adrenal gland was reduced to a slight enlargement.CONCLUSION This is a case of unilateral adrenal tuberculosis with CT imaging characteristics mimicking those of a malignant tumor.Extended anti-tuberculosis therapy is recommended in such cases.
基金Supported by a National Research Foundation of Korea(NRF)Grant Funded by the Korean Government(Ministry of Science and ICT),No.2019R1G1A1100422.
文摘BACKGROUND Adrenal myelolipoma is a rare,benign,non-functioning mass that occurs in the adrenal gland.It is composed of an admixture of hematopoietic elements and mature adipose tissue,similar to bone marrow.Even at large sizes,adrenal myelolipomas are usually asymptomatic and often incidentally found by ultrasonography or computed tomography(CT)scan.This paper describes an unusual case of adrenal myelolipoma presenting as flank pain.CASE SUMMARY A 50-year-old male with severe right flank pain underwent a CT scan revealing a huge mass extending into the suprarenal space.The mass showed a fat component with retroperitoneal hemorrhage.The tumor was treated laparoscopically,and pathologic examination revealed features of myelolipoma originating from the adrenal gland.CONCLUSION Adrenal myelolipomas are generally asymptomatic and can be treated conservatively.However,rupture and hemorrhage of the tumor can cause symptoms requiring surgical removal.
文摘BACKGROUND Congenital adrenal hyperplasia(CAH),which is caused by a mutation of the steroidogenic acute regulatory(StAR)gene.Affected patients are usually characterized by adrenal insufficiency in the first year of life,salt loss,glucocorticoid and mineralocorticoid deficiency,and female external genitalia,regardless of chromosomal karyotype.Patients with non-classical lipoid CAH usually develop glucocorticoid deficiency and mild mineralocorticoid deficiency at 2-4 years of age.CASE SUMMARY Herein,We report the case of a woman with non-classic lipoid CAH combined with Graves’disease.Her chromosome karyotype was 46,XX,and highthroughput sequencing revealed two missense variants in the StAR gene:c.229C>T(p.Q77X)and c.814C>T(p.R272C),which were inherited from both parents(non-close relatives).The patient was treated for Graves’disease in a timely manner and the dosage of glucocorticoid was adjusted during the treatment of Graves’disease.CONCLUSION This is the first case of non-classic lipoid CAH combined with Graves’disease reported in the Chinese population.In addition to conventional glucocorticoid replacement therapy,timely adjustments were made to the dosages of thyroid hormone and glucocorticoid to avoid adrenal crisis as a consequence of the increased demand and accelerated metabolism of glucocorticoids when the patient was diagnosed with Graves’disease.
文摘We present 7 adults atopic asthmatics that were referred due to repeatedly relapsing asthma requiring oral courses of prednisolone. All were steroid responsive yet steroid dependant and were screened for adrenal insufficiency after changes to inhaled therapy and other standard anti-asthma treatments failed to improve the situation. All were deemed to be compliant. 4 used long-term intranasal steroids in addition to inhaled corticosteroids. No topical steroid creams were used by these patients. Adrenal suppression was examined via a 9 am cortisol level and a short synacthen test (using intravenous tetracosactide 250 mcg) along with measurement of ACTH (Adreno-Cortico-Trophic Hormone). The tests were performed in periods off prednisolone. We report the observed beneficial effects after treatment of the adrenal insufficiency with hydrocor-tisone acetate replacement therapy in these cases, and the benefit to their exacerbations, hospital admission and the requirement for prednisolone courses. These patients would have been stepped up to yet higher doses of inhaled steroids and even referred for anti-IgE treatment etc. Adrenal suppression is well described in the medical literature for asthmatics, but the beneficial outcomes of treating this are unknown. These 7 cases have been followed for up to 3 years since hydrocorti-sone replacement therapy. Most improved to a stable asthma without frequent exacerbations nor requirement for prednisolone and only 1 had a hospital admission.
文摘Numerous surgical modalities are available to treat adrenal lesions. Minimally-invasive approaches for adrenalectomy are indicated in most circumstances, and new evidence continues to be accumulated. In this context, current indications for open surgical adrenalectomy(OS-A), minimally-invasive adrenalectomy(MI-A), and laparoendoscopic single-site adrenalectomy(LESS-A) remain unclear. A comprehensive Englishlanguage literature review was performed using MEDLINE/Pub MED to identify articles and guidelines pertinent to the surgical management of adrenal tumors. A comprehensive chart review was performed for three illustrative cases. Clinical recommendations were generated based on relevant literature and the expertise of the investigator group. MI-A offers advantages over OS-A in properly selected patients, who experience fewer complications, lower blood loss, and shorter hospital stays. Robot-assisted laparoscopic and retroperitoneoscopic adrenalectomy may offer advantages over transperitoneal surgery, and LESS-A may be an even less-invasive option that will require further evaluation. MI-A remains the surgical treatment of choice for most adrenal lesions. Tumor size and stage are the primary indications for selecting alternative treatment modalities. OS-A remains the gold standard for large tumors(> 10 cm) and suspected or known advanced stage malignancy. LESS-A appears to be an appropriate initial approach for small tumors(< 4-5 cm), including pheochromocytoma and isolated adrenal metastases.
文摘Congenital Adrenal Hyperplasia is a group of autosomal recessive disorders resulting from deficiency of enzymes essential for the synthesis of cortisol.Disease of the adrenal cortex,but there may be involvement adrenomedullary.Cortisol and epinephrine are directly related to the individual’s stress response.Lower values of epinephrine in children with congenital adrenal hyperplasia could be related to increased clinical complications and hospitalizations rate.We evaluated the serum values of metanephrines and normetanephrines in children and adolescents with classic congenital adrenal hyperplasia and primary hypothyroidism and possible correlations with disease and hospitalizations.Cross-sectional study involved 29 patients(10 simple virilizing and 19 salt-wasting),and control group of 28 patients with primary hypothyroidism(10 overt and 18 subclinical).There were no differences in age(p=0.24)and metanephrine(p=0.34)or normetanephrine values(p=0.85)between groups.Hospitalization rate was higher in the cases than in the controls(51 x 12).We conclude the serum values of metanephrine and normetanephrine in patients with congenital adrenal hyperplasia were within the normal values of reference,with no significant difference of group with primary hypothiroidism.The number of hospitalizations in the case was high in relation to the control,mainly in salt-wasting.
文摘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 dif-ferent 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 existing published information regarding AI in patients with liver cirrhosis.
文摘As one kind of infectious diseases of adrenal gland, adrenal tuberculosis can result in a life-threatening disorder which is called primary adrenal insufficiency(PAI) due to the destruction of adrenal cortex. Computed tomography(CT) and magnetic resonance imaging(MRI) play significant roles in the diagnosis of this etiology of PAI based on the CT and MRI appearances of the adrenal lesions. In this mini-review, we intend to study the CT and MRI features of adrenal tuberculosis, which could be helpful to both endocrinologist and radiologist to establish a definitive diagnosis for adrenal tuberculosis resulting in PAI.
