Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing's syndrome.Methods The clinical data of 22 cases with severe adrenal Cushing's syndrome(severe group),and 136 cases with...Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing's syndrome.Methods The clinical data of 22 cases with severe adrenal Cushing's syndrome(severe group),and 136 cases with mild or moderate adrenal Cushing's syndrome(non-severe group)were reviewed.The clinical features were analyzed by comparing the differences between these two groups when patients were admitted to hospital.We discussed the clinical managements of patients with severe adrenal Cushing's syndrome by comparing the differences with non-severe group after preoperative preparation,and with themselves before and after preoperative preparation.The effects of surgery were evaluated by comparing the differences between pre-operation and post-operation on patients with severe adrenal Cushing's syndrome.Results At admission,serum/urine cortisol,disease course,and blood pressure were significantly higher in the severe group than those in non-severe group(P<0.05 or P<0.01),serum potassium and ACTH level were decreased significantly in the severe group than those in non-severe group [(3.01±0.75 vs 3.62±0.48)mmol/L,P<0.01;(6.47±2.91 vs 8.21±3.22)pg/ml,P<0.01].However,no significant difference was observed in diastolic blood pressure,serum potassium,and fasting plasma glucose between these two groups after preoperative preparation(all P>0.05).And then,we performed adrenalectomy.The symptoms of 22 cases with severe adrenal Cushing's syndrome were obviously alleviated after 3 months.During follow-up,5 cases of primary bilateral macronodular adrenal hyperplasia(BMAH)and 1 case of primary pigmented nodular adrenocortical disease(PPNAD)were treated with contralateral adrenalectomy.Conclusion Sufficient preoperative preparation is essential for patients with severe adrenal Cushing's syndrome because of its high level serum cortisol with severe complications.If preparation fails before surgery,cortisol lowering medication or emergency unilateral adrenalectomy is necessary.Severe patients with BMAH and PPNAD were firstly performed unilateral adrenalectomy and followed-up closely,and then,contralateral adrenalectomy is needed when the recurrence of hypercortisolism appeared.展开更多
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 ca...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 heterogene- ously-enhancing mass(4.5 cm 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.展开更多
Cardiac arrest is one of the leading causes of death and represents maximal stress in humans. After restoration of spontaneous circulation, post-cardiac arrest syndrome is the predominant disorder in survivors. Beside...Cardiac arrest is one of the leading causes of death and represents maximal stress in humans. After restoration of spontaneous circulation, post-cardiac arrest syndrome is the predominant disorder in survivors. Besides the post-arrest brain injury, the post-resuscitation myocardial stunning, and the systemic ischemia/reperfusion response, this syndrome is characterized by adrenal insufficiency, a disorder that often remains undiagnosed. The pathophysiology of adrenal insufficiency has not been elucidated. We performed a comprehensive search of three medical databases in order to describe the major pathophysiological disturbances which are responsible for the occurrence of the disorder. Based on the available evidence, this article will help physicians to better evaluate and understand the hidden yet deadly post-cardiac arrest adrenal insufficiency.展开更多
BACKGROUND Adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome(CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adreno...BACKGROUND Adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome(CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adrenocortical disease,ACTH-independent macronodular adrenal hyperplasia, and bilateral adrenocortical adenomas(BAAs) or carcinomas. BAAs causing ACTHindependent CS are rare; up to now, fewer than 40 BAA cases have been reported. The accurate diagnosis and evaluation of BAAs are critical for determining optimal treatment options. Adrenal vein sampling(AVS) is a good way to diagnose ACTH-independent CS.CASE SUMMARY A 31-year-old woman had a typical appearance of CS. The oral glucose tolerance test showed impaired glucose tolerance and obviously increased insulin and Cpeptide levels. Her baseline serum cortisol and urine free cortisol were elevated and did not show either a circadian rhythm or suppression with dexamethasone administration. The peripheral 1-deamino-8-D-arginine-vasopressin(DDVAP)stimulation test showed a delay of the peak level, which was 1.05 times as high as the baseline level. Bilateral AVS results suggested the possibility of BAAs.Abdominal computed tomography showed bilateral adrenal adenomas with atrophic adrenal glands(right: 3.1 cm × 2.0 cm × 1.9 cm; left: 2.2 cm × 1.9 cm × 2.1 cm). Magnetic resonance imaging of the pituitary gland demonstrated normal findings. A left adenomectomy by retroperitoneoscopy was performed first,followed by resection of the right-side adrenal mass 3 mo later. Biopsy results of both adenomas showed cortical tumors. Evaluations of ACTH and cortisol showed a significant decrease after left adenomectomy but could still not be suppressed, and the circadian rhythm was absent. Following bilateral adenomectomy, this patient has been administered with prednisone until now,all of her symptoms were alleviated, and she had normal blood pressure without edema in either of her lower extremities.CONCLUSION BAAs causing ACTH-independent CS are rare. AVS is of great significance for obtaining information on the functional state of BAAs before surgery.展开更多
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 ...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.展开更多
BACKGROUND Ectopic Cushing syndrome(ECS)is a rare condition commonly associated with neuroendocrine tumors(NET),mainly bronchial carcinoids.The association of paraneoplastic syndrome with Merkle cell carcinoma(MCC)is ...BACKGROUND Ectopic Cushing syndrome(ECS)is a rare condition commonly associated with neuroendocrine tumors(NET),mainly bronchial carcinoids.The association of paraneoplastic syndrome with Merkle cell carcinoma(MCC)is limited to individual case reports.CASE SUMMARY In this article we report an unusual and striking presentation of ECS in a patient with known metastatic MCC.An elderly patient presented with new onset severe hypertension,hyperglycemia and hypokalemia,muscle wasting,and peripheral edema.A diagnosis of adrenocorticotropic hormone dependent,non-pituitary,Cushing syndrome was established.Medical therapy inhibiting adrenal function was promptly started but unfortunately the patient survived only a few days after diagnosis.CONCLUSION The occurrence of an aggressive form of ECS in patients with NET should be recognized as an ominous event.To our knowledge,the association of this complication in a patient with MCC had not been reported.展开更多
BACKGROUND Co-morbidity of SRY gene turner syndrome(TS)with positive SRY gene and nonclassical congenital adrenal hyperplasia(NCAH)is extremely rare and has never been reported to date.CASE SUMMARY In this article,we ...BACKGROUND Co-morbidity of SRY gene turner syndrome(TS)with positive SRY gene and nonclassical congenital adrenal hyperplasia(NCAH)is extremely rare and has never been reported to date.CASE SUMMARY In this article,we present a 14-year-old girl who was referred to our hospital with short stature(weight of 43 kg and height of 143 cm,<-2 SD)with no secondary sexual characteristics(labia minora dysplasia).Laboratory tests indicated hypergonadotropic hypogonadism with significantly increased androstenedione and 17-hydroxyprogesterone(17-OHP)levels.This was accompanied by the thickening of the extremity of the left adrenal medial limb.The patient’s karyotype was 45,X/46,X,+mar,and cytogenetic analysis using multiplex ligation-dependent probe amplification and high-throughput sequencing indicated that the SRY gene was positive with compound heterozygous mutations in CYP21A2 as the causative gene for congenital adrenal hyperplasia.The sites of the suspected candidate mutations were amplified and verified using Sanger sequencing.The patient was finally diagnosed as having SRY positive TS with NCAH.The patient and her family initially refused medical treatment.At her most recent follow-up visit(age=15 years old),the patient presented facial hair,height increase to 148 cm,and weight of 52 kg,while androstenedione and 17-OHP levels remained high.The patient was finally willing to take small doses of hydrocortisone(10 mg/d).CONCLUSIONIn conclusion, upon evaluation of the patient mentioned in the report, we feel that17-OHP measurement and cytogenetic analysis are necessary for TS patients evenin the absence of significant virilization signs. This will play a significant role inguiding diagnosis and treatment.展开更多
We report a case of a 35-year-old lady who presented with Cushingoid features and associated raised urinary metanephrine.The patient underwent open adrenelectomy.Histopathological examination revealed adreno-cortical ...We report a case of a 35-year-old lady who presented with Cushingoid features and associated raised urinary metanephrine.The patient underwent open adrenelectomy.Histopathological examination revealed adreno-cortical carcinoma with microscopic lymphovascular invasion.Postoperative period was uneventful and is on follow-up for the last one year and is doing well.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
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 Adrenal incidentaloma(AI)has been frequently encountered in the clinical setting.It has been shown that primary aldosteronism(PA)or subclinical Cushing’s syndrome(SCS)are the representative causative disea...BACKGROUND Adrenal incidentaloma(AI)has been frequently encountered in the clinical setting.It has been shown that primary aldosteronism(PA)or subclinical Cushing’s syndrome(SCS)are the representative causative diseases of AI.However,the coexistence of PA and SCS has been reportedly observed.Recently,we encountered a case of AI,in which PA and SCS coexisted,confirmed by histopathological examinations after a laparoscopic adrenalectomy.We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARY A 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg.A subsequent computed tomography scan revealed right ureterolithiasis,which was the cause of right abdominal pain,and right AI measuring 22 mm×25 mm.After the disappearance of right abdominal pain,subsequent endocrinological examinations were performed.Aldosterone-related evaluations,including adrenal venous sampling,revealed the presence of bilateral PA.In addition,several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma.A laparoscopic right adrenalectomy was then performed.The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma,while CYP11B2 immunoreactivity was absent in this adenoma.However,in the adjacent nonneoplastic adrenal,multiple CYP11B2-positive adrenocortical micronodules were detected,showing the presence of aldosterone-producing adrenocortical micronodules.CONCLUSION Careful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.展开更多
In order to improve the treatment outcome and facilitate the clinical practice,a diagnostic classification of heterogeneous disease-polycystic ovary syndrome(PCOS)is proposed.PCOS is classified into 2 main types(Ⅰ,Ⅱ...In order to improve the treatment outcome and facilitate the clinical practice,a diagnostic classification of heterogeneous disease-polycystic ovary syndrome(PCOS)is proposed.PCOS is classified into 2 main types(Ⅰ,Ⅱ)and 4 subtypes(Ⅰa,Ⅰb,Ⅱa,Ⅱb):PCOSⅠrefers to cases with hyperandrogenemia derived from the ovary(Ⅰa)or from both the ovary and the adrenal cortex(Ⅰb).PCOS Ⅱ refers to cases with both hyperandrogenemia and hyperinsulinemia,while Ⅱb being severer than Ⅱa,like hyperthecosis.Better efficacy of integrative medicine treatment on this classification is mentioned.展开更多
Objective: To explore the relationship between TCM Syndrome typing and adrenocortical function in elderly patients with severe sepsis, and to see whether TCM Syndrome Differentiation can provide clinical clues in iden...Objective: To explore the relationship between TCM Syndrome typing and adrenocortical function in elderly patients with severe sepsis, and to see whether TCM Syndrome Differentiation can provide clinical clues in identifying relative adrenal insufficiency (RAI) in patients with severe sepsis. Methods: Sixty-one old patients with severe sepsis were classified into four types according to TCM Syndrome Differentiation: The severe invasion of toxic-heat type (Type SITH, n = 21); the Qi stagnation and blood stasis type (Type QSBS, n = 11); the sudden depletion of Yang-Qi type (Type SDYQ, n = 16); and the exhaustion of Qi-Yin type (Type EOQY, n = 13). The base-line level of plasma cortisol in patients of different types and their response to corticotropin stimulation were compared, which were also compared with those of 12 healthy elderly persons synchronously. Results: The base-line level of plasma cortisol was not significantly different between patients of different Syndrome types ( P>0. 05), but they were all sgnificantly higher than that in the healthy persons (P<0. 05). Compared with Type QSBS and Type EOQY, Type SITH and Type SDYQ showed less cortisol concentration increment after corticotropin stimulation ( P<0. 05). RAI was more prevalent in patients of Type SITH and Type SDYQ than in patients of Type QSBS and Type EOQY (57% vs 25% , P<0. 01). Conclusion: In old patients with severe sepsis, different TCM Syndrome types are associated with different adrenocortical function status. TCM Syndrome differentiation can provide clinical clues in i-dentifying old patients with severe sepsis who have also RAI.展开更多
A 74-year-old woman presented with symptoms consistent with hyperadrenocorticism and hyperca- techolaminism. She had a cushingoid appearance and her cortisol level was elevated. Her serum dopamine and noradrenalin lev...A 74-year-old woman presented with symptoms consistent with hyperadrenocorticism and hyperca- techolaminism. She had a cushingoid appearance and her cortisol level was elevated. Her serum dopamine and noradrenalin levels were also elevated. Computed tomography detected a left adrenal mass measuring 3.5 cm x 3.0 cm in diameter. Metaiodobenzylguanidine scintigraphy was negative. Unexpectedly, the serum Serum carcinoembryonic antigen (CEA) level waselevated. Fluorodeoxyglucose positron emission tomography showed increased uptake in the adrenal tumor only, with a maximum standardized uptake value of 2.8. Selective venography and blood sampling revealed that the concentrations of cortisol, catecholamines and CEA were significantly elevated in the vein draining the tumor. A diagnosis of CEA-producing benign adenoma was made. After preoperative management, we performed a combined lateral and anterior transpedtoneal laparoscopic adrenectomy. Her vital signs remained stable during surgery. Histopathological examination revealed a benign adenoma. Her cortisol, catecholamine and CEA levels normalized immediately after surgery. We present, to the best of our knowledge, the first case of CEA-producing adrenal adenoma, along with a review of the relevant literature, and discuss our laparoscopic surgery techniques.展开更多
Autism spectrum disorders (ASDs) are epidemically explosive clinical entities, but their pathogenesis is still unclear and a definitive cure does not yet exist. Rett syndrome (RTT) is a rare genetically determined cau...Autism spectrum disorders (ASDs) are epidemically explosive clinical entities, but their pathogenesis is still unclear and a definitive cure does not yet exist. Rett syndrome (RTT) is a rare genetically determined cause of autism linked to mutations in the X-linked MeCP2 gene or, more rarely, in CDKL5 or FOXG1. A wide phenotypical heterogeneity is a known feature of the disease. Although several studies have focused on the molecular genetics and possible protein changes at different levels, to date very little attention has been paid to fatty acids in this disease, which could be considered as a natural paradigm for the ASDs. To this regard, a quite enigmatic feature of the disease is the evidence in the affected patients of an extensive peroxidation of polyunsaturated fatty acids (arachidonic acid, AA, docosaexahenoic acid, DHA, adrenic acid, AdA and, to a lesser extent, eicosapentaenoic acid, EPA), in contrast with amelioration of the redox changes and phenotypical severity following the supplementation of some of those same fatty acids (DHA + EPA). Therefore, fatty acids may represent a kind of Janus Bifrons in the particular context of RTT. Here, we propose a rational explanation for this apparent “fatty acid paradox” in RTT. A better understanding of this paradox could also be of help to get a better insight into the complex mechanism of action for polyunsaturated fatty acids in health and disease.展开更多
BACKGROUND Empty sella is an anatomical and radiological finding of the herniation of the subarachnoid space into the pituitary fossa leading to a flattened pituitary gland.Patients with empty sella may present with v...BACKGROUND Empty sella is an anatomical and radiological finding of the herniation of the subarachnoid space into the pituitary fossa leading to a flattened pituitary gland.Patients with empty sella may present with various symptoms,including headache due to intracranial hypertension and endocrine symptoms related to the specific pituitary hormones affected.Here,we report a female patient who developed persistent postoperative hypotension caused by subclinical empty sella syndrome after a simple surgery.CASE SUMMARY A 47-year-old woman underwent vocal cord polypectomy under general anesthesia with endotracheal intubation.She denied any medical history,and her vital signs were normal before the surgery.Anesthesia and surgery were uneventful.However,she developed dizziness,headache and persistent hypotension in the ward.Thus,intravenous dopamine was started to maintain normal blood pressure,which improved her symptoms.However,she remained dependent on dopamine for over 24 h without any obvious anesthesia-and surgery-related complications.An endocrine etiology was then suspected,and further examination showed a high prolactin level,a low normal adrenocorticotropic hormone level and a low cortisol level.Magnetic resonance imaging of the brain revealed an empty sella.Therefore,she was diagnosed with empty sella syndrome and secondary adrenal insufficiency.Her symptoms disappeared one week later after daily glucocorticoid supplement.CONCLUSION Endocrine etiologies such as pituitary and adrenal-related dysfunction should be considered in patients showing persistent postoperative hypotension when anesthesia-and surgery-related factors are excluded.展开更多
Two patients had a long history of hypertension and one of them also had a nephrotic-range proteinuria secondary to focal segmental scleronephrosis which had been diagnosed by kidney biopsy. They presented with uncont...Two patients had a long history of hypertension and one of them also had a nephrotic-range proteinuria secondary to focal segmental scleronephrosis which had been diagnosed by kidney biopsy. They presented with uncontrolled hypertension. Laboratory examination suggested hypersecretion of catecholamines by the left adrenal glands in both patients and primary aldosteronism in one of them. A computed tomography scan revealed small nodules on left adrenal gland in both patients. Patients underwent laparoscopic left adrenalectomy. After surgery, blood pressure was normalized and proteinuria was resolved. Most interestingly, prominent adipocytes infiltrated were detected in the adrenal cortex and were associated with the presence of lymphocytes, which suggested that adipocytes might have a role in the pathogenesis of these diseases in our patients.展开更多
文摘Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing's syndrome.Methods The clinical data of 22 cases with severe adrenal Cushing's syndrome(severe group),and 136 cases with mild or moderate adrenal Cushing's syndrome(non-severe group)were reviewed.The clinical features were analyzed by comparing the differences between these two groups when patients were admitted to hospital.We discussed the clinical managements of patients with severe adrenal Cushing's syndrome by comparing the differences with non-severe group after preoperative preparation,and with themselves before and after preoperative preparation.The effects of surgery were evaluated by comparing the differences between pre-operation and post-operation on patients with severe adrenal Cushing's syndrome.Results At admission,serum/urine cortisol,disease course,and blood pressure were significantly higher in the severe group than those in non-severe group(P<0.05 or P<0.01),serum potassium and ACTH level were decreased significantly in the severe group than those in non-severe group [(3.01±0.75 vs 3.62±0.48)mmol/L,P<0.01;(6.47±2.91 vs 8.21±3.22)pg/ml,P<0.01].However,no significant difference was observed in diastolic blood pressure,serum potassium,and fasting plasma glucose between these two groups after preoperative preparation(all P>0.05).And then,we performed adrenalectomy.The symptoms of 22 cases with severe adrenal Cushing's syndrome were obviously alleviated after 3 months.During follow-up,5 cases of primary bilateral macronodular adrenal hyperplasia(BMAH)and 1 case of primary pigmented nodular adrenocortical disease(PPNAD)were treated with contralateral adrenalectomy.Conclusion Sufficient preoperative preparation is essential for patients with severe adrenal Cushing's syndrome because of its high level serum cortisol with severe complications.If preparation fails before surgery,cortisol lowering medication or emergency unilateral adrenalectomy is necessary.Severe patients with BMAH and PPNAD were firstly performed unilateral adrenalectomy and followed-up closely,and then,contralateral adrenalectomy is needed when the recurrence of hypercortisolism appeared.
文摘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 heterogene- ously-enhancing mass(4.5 cm 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.
文摘Cardiac arrest is one of the leading causes of death and represents maximal stress in humans. After restoration of spontaneous circulation, post-cardiac arrest syndrome is the predominant disorder in survivors. Besides the post-arrest brain injury, the post-resuscitation myocardial stunning, and the systemic ischemia/reperfusion response, this syndrome is characterized by adrenal insufficiency, a disorder that often remains undiagnosed. The pathophysiology of adrenal insufficiency has not been elucidated. We performed a comprehensive search of three medical databases in order to describe the major pathophysiological disturbances which are responsible for the occurrence of the disorder. Based on the available evidence, this article will help physicians to better evaluate and understand the hidden yet deadly post-cardiac arrest adrenal insufficiency.
文摘BACKGROUND Adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome(CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adrenocortical disease,ACTH-independent macronodular adrenal hyperplasia, and bilateral adrenocortical adenomas(BAAs) or carcinomas. BAAs causing ACTHindependent CS are rare; up to now, fewer than 40 BAA cases have been reported. The accurate diagnosis and evaluation of BAAs are critical for determining optimal treatment options. Adrenal vein sampling(AVS) is a good way to diagnose ACTH-independent CS.CASE SUMMARY A 31-year-old woman had a typical appearance of CS. The oral glucose tolerance test showed impaired glucose tolerance and obviously increased insulin and Cpeptide levels. Her baseline serum cortisol and urine free cortisol were elevated and did not show either a circadian rhythm or suppression with dexamethasone administration. The peripheral 1-deamino-8-D-arginine-vasopressin(DDVAP)stimulation test showed a delay of the peak level, which was 1.05 times as high as the baseline level. Bilateral AVS results suggested the possibility of BAAs.Abdominal computed tomography showed bilateral adrenal adenomas with atrophic adrenal glands(right: 3.1 cm × 2.0 cm × 1.9 cm; left: 2.2 cm × 1.9 cm × 2.1 cm). Magnetic resonance imaging of the pituitary gland demonstrated normal findings. A left adenomectomy by retroperitoneoscopy was performed first,followed by resection of the right-side adrenal mass 3 mo later. Biopsy results of both adenomas showed cortical tumors. Evaluations of ACTH and cortisol showed a significant decrease after left adenomectomy but could still not be suppressed, and the circadian rhythm was absent. Following bilateral adenomectomy, this patient has been administered with prednisone until now,all of her symptoms were alleviated, and she had normal blood pressure without edema in either of her lower extremities.CONCLUSION BAAs causing ACTH-independent CS are rare. AVS is of great significance for obtaining information on the functional state of BAAs before surgery.
文摘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.
文摘BACKGROUND Ectopic Cushing syndrome(ECS)is a rare condition commonly associated with neuroendocrine tumors(NET),mainly bronchial carcinoids.The association of paraneoplastic syndrome with Merkle cell carcinoma(MCC)is limited to individual case reports.CASE SUMMARY In this article we report an unusual and striking presentation of ECS in a patient with known metastatic MCC.An elderly patient presented with new onset severe hypertension,hyperglycemia and hypokalemia,muscle wasting,and peripheral edema.A diagnosis of adrenocorticotropic hormone dependent,non-pituitary,Cushing syndrome was established.Medical therapy inhibiting adrenal function was promptly started but unfortunately the patient survived only a few days after diagnosis.CONCLUSION The occurrence of an aggressive form of ECS in patients with NET should be recognized as an ominous event.To our knowledge,the association of this complication in a patient with MCC had not been reported.
文摘BACKGROUND Co-morbidity of SRY gene turner syndrome(TS)with positive SRY gene and nonclassical congenital adrenal hyperplasia(NCAH)is extremely rare and has never been reported to date.CASE SUMMARY In this article,we present a 14-year-old girl who was referred to our hospital with short stature(weight of 43 kg and height of 143 cm,<-2 SD)with no secondary sexual characteristics(labia minora dysplasia).Laboratory tests indicated hypergonadotropic hypogonadism with significantly increased androstenedione and 17-hydroxyprogesterone(17-OHP)levels.This was accompanied by the thickening of the extremity of the left adrenal medial limb.The patient’s karyotype was 45,X/46,X,+mar,and cytogenetic analysis using multiplex ligation-dependent probe amplification and high-throughput sequencing indicated that the SRY gene was positive with compound heterozygous mutations in CYP21A2 as the causative gene for congenital adrenal hyperplasia.The sites of the suspected candidate mutations were amplified and verified using Sanger sequencing.The patient was finally diagnosed as having SRY positive TS with NCAH.The patient and her family initially refused medical treatment.At her most recent follow-up visit(age=15 years old),the patient presented facial hair,height increase to 148 cm,and weight of 52 kg,while androstenedione and 17-OHP levels remained high.The patient was finally willing to take small doses of hydrocortisone(10 mg/d).CONCLUSIONIn conclusion, upon evaluation of the patient mentioned in the report, we feel that17-OHP measurement and cytogenetic analysis are necessary for TS patients evenin the absence of significant virilization signs. This will play a significant role inguiding diagnosis and treatment.
文摘We report a case of a 35-year-old lady who presented with Cushingoid features and associated raised urinary metanephrine.The patient underwent open adrenelectomy.Histopathological examination revealed adreno-cortical carcinoma with microscopic lymphovascular invasion.Postoperative period was uneventful and is on follow-up for the last one year and is doing well.
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.
文摘BACKGROUND Adrenal incidentaloma(AI)has been frequently encountered in the clinical setting.It has been shown that primary aldosteronism(PA)or subclinical Cushing’s syndrome(SCS)are the representative causative diseases of AI.However,the coexistence of PA and SCS has been reportedly observed.Recently,we encountered a case of AI,in which PA and SCS coexisted,confirmed by histopathological examinations after a laparoscopic adrenalectomy.We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARY A 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg.A subsequent computed tomography scan revealed right ureterolithiasis,which was the cause of right abdominal pain,and right AI measuring 22 mm×25 mm.After the disappearance of right abdominal pain,subsequent endocrinological examinations were performed.Aldosterone-related evaluations,including adrenal venous sampling,revealed the presence of bilateral PA.In addition,several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma.A laparoscopic right adrenalectomy was then performed.The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma,while CYP11B2 immunoreactivity was absent in this adenoma.However,in the adjacent nonneoplastic adrenal,multiple CYP11B2-positive adrenocortical micronodules were detected,showing the presence of aldosterone-producing adrenocortical micronodules.CONCLUSION Careful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.
文摘In order to improve the treatment outcome and facilitate the clinical practice,a diagnostic classification of heterogeneous disease-polycystic ovary syndrome(PCOS)is proposed.PCOS is classified into 2 main types(Ⅰ,Ⅱ)and 4 subtypes(Ⅰa,Ⅰb,Ⅱa,Ⅱb):PCOSⅠrefers to cases with hyperandrogenemia derived from the ovary(Ⅰa)or from both the ovary and the adrenal cortex(Ⅰb).PCOS Ⅱ refers to cases with both hyperandrogenemia and hyperinsulinemia,while Ⅱb being severer than Ⅱa,like hyperthecosis.Better efficacy of integrative medicine treatment on this classification is mentioned.
基金This study was supported by the National "973" Project of China (No. G2000057004)
文摘Objective: To explore the relationship between TCM Syndrome typing and adrenocortical function in elderly patients with severe sepsis, and to see whether TCM Syndrome Differentiation can provide clinical clues in identifying relative adrenal insufficiency (RAI) in patients with severe sepsis. Methods: Sixty-one old patients with severe sepsis were classified into four types according to TCM Syndrome Differentiation: The severe invasion of toxic-heat type (Type SITH, n = 21); the Qi stagnation and blood stasis type (Type QSBS, n = 11); the sudden depletion of Yang-Qi type (Type SDYQ, n = 16); and the exhaustion of Qi-Yin type (Type EOQY, n = 13). The base-line level of plasma cortisol in patients of different types and their response to corticotropin stimulation were compared, which were also compared with those of 12 healthy elderly persons synchronously. Results: The base-line level of plasma cortisol was not significantly different between patients of different Syndrome types ( P>0. 05), but they were all sgnificantly higher than that in the healthy persons (P<0. 05). Compared with Type QSBS and Type EOQY, Type SITH and Type SDYQ showed less cortisol concentration increment after corticotropin stimulation ( P<0. 05). RAI was more prevalent in patients of Type SITH and Type SDYQ than in patients of Type QSBS and Type EOQY (57% vs 25% , P<0. 01). Conclusion: In old patients with severe sepsis, different TCM Syndrome types are associated with different adrenocortical function status. TCM Syndrome differentiation can provide clinical clues in i-dentifying old patients with severe sepsis who have also RAI.
文摘A 74-year-old woman presented with symptoms consistent with hyperadrenocorticism and hyperca- techolaminism. She had a cushingoid appearance and her cortisol level was elevated. Her serum dopamine and noradrenalin levels were also elevated. Computed tomography detected a left adrenal mass measuring 3.5 cm x 3.0 cm in diameter. Metaiodobenzylguanidine scintigraphy was negative. Unexpectedly, the serum Serum carcinoembryonic antigen (CEA) level waselevated. Fluorodeoxyglucose positron emission tomography showed increased uptake in the adrenal tumor only, with a maximum standardized uptake value of 2.8. Selective venography and blood sampling revealed that the concentrations of cortisol, catecholamines and CEA were significantly elevated in the vein draining the tumor. A diagnosis of CEA-producing benign adenoma was made. After preoperative management, we performed a combined lateral and anterior transpedtoneal laparoscopic adrenectomy. Her vital signs remained stable during surgery. Histopathological examination revealed a benign adenoma. Her cortisol, catecholamine and CEA levels normalized immediately after surgery. We present, to the best of our knowledge, the first case of CEA-producing adrenal adenoma, along with a review of the relevant literature, and discuss our laparoscopic surgery techniques.
文摘Autism spectrum disorders (ASDs) are epidemically explosive clinical entities, but their pathogenesis is still unclear and a definitive cure does not yet exist. Rett syndrome (RTT) is a rare genetically determined cause of autism linked to mutations in the X-linked MeCP2 gene or, more rarely, in CDKL5 or FOXG1. A wide phenotypical heterogeneity is a known feature of the disease. Although several studies have focused on the molecular genetics and possible protein changes at different levels, to date very little attention has been paid to fatty acids in this disease, which could be considered as a natural paradigm for the ASDs. To this regard, a quite enigmatic feature of the disease is the evidence in the affected patients of an extensive peroxidation of polyunsaturated fatty acids (arachidonic acid, AA, docosaexahenoic acid, DHA, adrenic acid, AdA and, to a lesser extent, eicosapentaenoic acid, EPA), in contrast with amelioration of the redox changes and phenotypical severity following the supplementation of some of those same fatty acids (DHA + EPA). Therefore, fatty acids may represent a kind of Janus Bifrons in the particular context of RTT. Here, we propose a rational explanation for this apparent “fatty acid paradox” in RTT. A better understanding of this paradox could also be of help to get a better insight into the complex mechanism of action for polyunsaturated fatty acids in health and disease.
文摘BACKGROUND Empty sella is an anatomical and radiological finding of the herniation of the subarachnoid space into the pituitary fossa leading to a flattened pituitary gland.Patients with empty sella may present with various symptoms,including headache due to intracranial hypertension and endocrine symptoms related to the specific pituitary hormones affected.Here,we report a female patient who developed persistent postoperative hypotension caused by subclinical empty sella syndrome after a simple surgery.CASE SUMMARY A 47-year-old woman underwent vocal cord polypectomy under general anesthesia with endotracheal intubation.She denied any medical history,and her vital signs were normal before the surgery.Anesthesia and surgery were uneventful.However,she developed dizziness,headache and persistent hypotension in the ward.Thus,intravenous dopamine was started to maintain normal blood pressure,which improved her symptoms.However,she remained dependent on dopamine for over 24 h without any obvious anesthesia-and surgery-related complications.An endocrine etiology was then suspected,and further examination showed a high prolactin level,a low normal adrenocorticotropic hormone level and a low cortisol level.Magnetic resonance imaging of the brain revealed an empty sella.Therefore,she was diagnosed with empty sella syndrome and secondary adrenal insufficiency.Her symptoms disappeared one week later after daily glucocorticoid supplement.CONCLUSION Endocrine etiologies such as pituitary and adrenal-related dysfunction should be considered in patients showing persistent postoperative hypotension when anesthesia-and surgery-related factors are excluded.
文摘Two patients had a long history of hypertension and one of them also had a nephrotic-range proteinuria secondary to focal segmental scleronephrosis which had been diagnosed by kidney biopsy. They presented with uncontrolled hypertension. Laboratory examination suggested hypersecretion of catecholamines by the left adrenal glands in both patients and primary aldosteronism in one of them. A computed tomography scan revealed small nodules on left adrenal gland in both patients. Patients underwent laparoscopic left adrenalectomy. After surgery, blood pressure was normalized and proteinuria was resolved. Most interestingly, prominent adipocytes infiltrated were detected in the adrenal cortex and were associated with the presence of lymphocytes, which suggested that adipocytes might have a role in the pathogenesis of these diseases in our patients.