Sudden Death in a Patient with Undiagnosed Diabetes, Acute Pancreatitis and Acute Adrenalitis

It has been well documented that acute pancreatitis and diabetic ketoacidosis (DKA) co-occur, and both of them present either as a cause or result of the other [1] [2]. Recently, hypertriglyceridemia has been reported to co-occur as well [3] [4];the involvement of acute adrenalitis in the setting of acute pancreatitis and DKA has not been reported. We report a case of sudden death due to diabetic ketoacidosis, acute pancreatitis and acute adrenalitis in a 37-year-old female. She was found unresponsive and breathless in bed by her mother at their residence. At the time of the autopsy, both lungs were expanded and sigmoid diverticulosis was discovered on gross examination. Histology of the pancreas and the adrenal glands revealed acute inflammation despite appearing normal upon gross examination. The postmortem toxicology report showed high levels of acetone (12 mg/dL) in the postmortem blood and extremely high levels of glucose (>500 mg/dL) in the postmortem vitreous fluid. This is the first reported case of fatal diabetic ketoacidosis co-occurring with acute pancreatitis and acute adrenalitis.

Evaluation of primary adrenal insufficiency secondary to tuberculous adrenalitis with computed tomography and magnetic resonance imaging:Current status

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.

Occult Adrenal Insufficiency in Patients with Chronic Renal Disease

Background: Addison’s disease is a rare disorder of the adrenal cortex that leads to inadequate production of cortisol initially followed by aldosterone and androgens. Its manifestations are usually slow and non-specific with potential for life-threatening adrenal crisis following hypermetabolic demands (infection, trauma, surgery). Patients: Over the past 10 years, 19 CRD-patients were diagnosed with occult PAI in our center. Results: Unprovoked hypotension was the most common manifestations of occult PAI and was the unmasking event in 11 (58%). It was without significant cardiac and/or severe systemic sepsis and was refractory to isotonic saline infusions. Equal number of the remaining patients (n = 2) presented with persistent and inexplicable electrolytes abnormalities viz. 1) hyponatremia despite restricted oral fluid intake, lack of dehydration and massive fluid overload, as well as 2) hyperkalemia despite potassium-restricted diet, hyperkalemic drugs and adequate therapy with Furosemide and low-potassium dialysis-baths. On the other hand, similar proportions presented with unprovoked 3) progressive weight loss, decrease appetite and cachexia as well as 4) frequent hypoglycemic attacks. All patients were treated and were medically stable after 29 (2 - 60) months of follow up. Autoantibodies to 21-hydroxylase enzyme were positive in 16 (90%). At diagnosis, and subsequent follow up, only 7 patients (37%) had multi-endocrine dysfunction of whom 2 with type 1 and 5 with type 2. Conclusion: High index of suspicion should be exerted in diagnosis of PAI in patients with CRD, since its clinical picture is similar to CRD manifestations and complications. In those patients, confirmatory tests and specific management can save their lives. .

Addison's disease caused by adrenal tuberculosis may lead to misdiagnosis of major depressive disorder: A case report

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.

Bilateral adrenal histoplasmosis in a Malaysian tertiary hospital:Report of four cases

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.

Human-like adrenal features in Chinese tree shrews revealed by multi-omics analysis of adrenal cell populations and steroid synthesis

The Chinese tree shrew(Tupaia belangeri chinensis)has emerged as a promising model for investigating adrenal steroid synthesis,but it is unclear whether the same cells produce steroid hormones and whether their production is regulated in the same way as in humans.Here,we comprehensively mapped the cell types and pathways of steroid metabolism in the adrenal gland of Chinese tree shrews using single-cell RNA sequencing,spatial transcriptome analysis,mass spectrometry,and immunohistochemistry.We compared the transcriptomes of various adrenal cell types across tree shrews,humans,macaques,and mice.Results showed that tree shrew adrenal glands expressed many of the same key enzymes for steroid synthesis as humans,including CYP11B2,CYP11B1,CYB5A,and CHGA.Biochemical analysis confirmed the production of aldosterone,cortisol,and dehydroepiandrosterone but not dehydroepiandrosterone sulfate in the tree shrew adrenal glands.Furthermore,genes in adrenal cell types in tree shrews were correlated with genetic risk factors for polycystic ovary syndrome,primary aldosteronism,hypertension,and related disorders in humans based on genome-wide association studies.Overall,this study suggests that the adrenal glands of Chinese tree shrews may consist of closely related cell populations with functional similarity to those of the human adrenal gland.Our comprehensive results(publicly available at http://gxmujyzmolab.cn:16245/scAGMap/)should facilitate the advancement of this animal model for the investigation of adrenal gland disorders.

Clinical,Biochemical,and Radiological Retrospective Analysis in Patients with Adrenal Incidentaloma-A Secondary Publication

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.

Overview of Adrenals Tumors in Dakar Hospitals

Introduction: Adrenal tumours (AT) are commonly encountered in clinical practice. For any patient presenting an adrenal mass, there are two crucial points to consider: is the adrenal mass malignant and is it hormonally active? The objective of our study was to evaluate the epidemiological, diagnostic, therapeutic, and evolutionary aspects of AT in a developing country. Methodology: We conducted a retrospective study spanning 17 years (from January 2005 to October 2022) in four Dakar hospital services. We included all patient medical records with explored AT. Results: AT was diagnosed in 35 patients who had a mean age of 36.62 years (range: 12-79), and a female predominance (26 women to 9 men). Among these patients, 17.2 % had incidental AT. Hypertension was the commonest presenting symptom in 27 patients (77%), which was associated with Mnards triad in 14 patients (40%). Abdominal pain was reported by 20 patients (57%), and 14 presented with an abdominal mass. Among the patients, 29 had functional AT: 22 with pheochromocytoma, 5 with primary hyperaldosteronism, and 2 with hypercortisolism. Imaging evaluations revealed that 33 patients had unilateral AT and one had bilateral AT. Tumour sizes varied, 5 patients had tumours 10 cm. Two patients presented with metastases. The therapeutic approach involved adrenalectomy in 32 patients. Perioperative complications were observed in five patients, including haemorrhage (two patients), hypotension (two cases), and hypertensive crisis (one case). One patient with bilateral pheochromocytoma developed acute adrenal insufficiency postoperatively, followed by abdominal herniation. Histopathological examination confirmed pheochromocytoma in 21 patients, Conns adenoma in 5, adrenocortical carcinoma in 3, cortisol adenoma in 2, and adrenal cyst in 1 patient. Non-functional AT included adrenocortical carcinoma (three patients), adrenal cyst (one patient), and pheochromocytoma (two patients). After a one-year follow-up, 29 patients with benign tumours had favourable outcomes, while death occurred within six months of diagnosis in five cases. A patient who had bilateral pheochromocytomas, with complications, died before surgery. Conclusion: In our practice, AT are predominantly pheochromocytomas, being typically diagnosed when they become symptomatic large tumours and, due to this large size, laparotomy is the preferred surgical approach. Unfortunately, the prognosis for malignant tumours is poor.

Diagnosis of CAH in a Sub Saharan Country: Visible Part of Iceberg

Introduction :Congenital adrenal hyperplasia (CAH) is the most common cause of primary adrenal insufficiency. It is a rare monogenic recessive disorder. In African setting in absence of neonatal screening, the diagnosis is still late, based on a clinical approach. During this clinical enquiry, information from past history or pedigree of the patient is of a huge importance and may revealed surprises. Patients and Methods: In this observational study, we retrospectively included all patients with a diagnosis of CAH. The diagnosis of CAH was retained based on a high 17 hydroxyprogesterone level in addition to clinical and morphological findings. From patients’ files, we extracted data on family history of disease, pedigree, clinical findings and genetics when available of 39 patients from two endocrinopeadiatric centers. Results: In 13 (30%) families, we found 20 reported deaths of infant less than 12 months. In these 13 families, half of the patients followed had 21 hydroxylase deficiencies and had 11 hydroxylase deficiencies. Unsurprisingly, we suspected adrenal insufficiency in these patients at verbal autopsy even in families with a patient with 11 hydroxylase deficiency. Other non DSD malformations or genetic disorders with apparently no link with CAH were reported in 3 families. The father of a patient reported to have hypospadias. Conclusion: Each diagnosis of CAH made in our context is visible part of an iceberg. Behind a diagnosis of CAH made in our setting, is a long course of care, a dramatic past history revealing access to appropriate care disparity. Neonatal screening should thus be considered as an emergency.

Venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock induced by adrenal lesions:a case report and review of the literature

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]

Acute adrenal insufficiency caused by antiphospholipid syndrome

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.

Persistent postoperative hypotension caused by subclinical empty sella syndrome after a simple surgery:A case report

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.

Primary adrenal Ewing sarcoma:A systematic review of the literature

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.

Dehydroepiandrosterone sulfate supplementation in health and diseases

Dehydroepiandrosterone sulfate(DHEAS)is a hormone produced by the zona reticularis of the adrenal gland and the ovaries.Initially considered as an inert compound merely serving as an intermediate in the conversion of cholesterol to androgens,interest in DHEA began to grow in the 1960s when it was found that DHEAS is the most abundant steroid hormone in human plasma and that its levels decline with age.In many countries,DHEA is considered a nutritional supplement.It has been used for a multitude of conditions which include sexual dysfunction,infertility,genitourinary syndrome of menopause,musculoskeletal disorders,cardiovascular diseases,ageing,neurological diseases,autoimmune conditions,adrenal insufficiency,and anorexia nervosa.We describe an overview of the historical evolution of DHEA,its physiology,and the disease states where it has been evaluated as a supplement.

Prolonged Adrenal Insufficiency after Unilateral Adrenalectomy for Cortisolic Adenoma

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.

Surgical Management of Adrenal Tumors: Experience of Three Tertiary Hospitals in Yaounde, Cameroon

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.

Relation between Neurotransmitters and NK Cells in Adrenal and Breast Cancer

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.

The Diagnosis and Treatment of Virilizing and Fem- inizing Adrenal Syndrome

Objective: To inquire into diagnosis, and treatment of virilizing andfeminizing a-drenal syndrome, differential diagnosis between benign and malignant sex hormoneproducing adrenal neo-plasma and, treatment principles of congenital adrenal hyperplasia (CAH).Methods: Eight cases of CAH and 5 cases of sex hormone producing adrenal neoplasma were admitted tohospital during 1986-1996. The former included 3 rare cases of 17 a hydroxylase deficiency. Thelatter included 3 cases of feminizing adrenal tumor and 2 cases of virilizing adrenal tumor.Results: Weight, size and CT of the tumor, DHEA, 17 -ks, sex hormone levels, infiltration, andmetastasis were closely related to the degree of differentiation of the tumors. Conclusion:Virilizing and feminizing adrenal neoplasm were removed surgically by different incisions. Modifiedsubcostal incision was recommended as the best choice for huge adrenal mass. Corticoadrenal hormonetreatment fa CAH should be individualized according to the different types of the disease. Sexhormones were not suitable for children suffering from 17 hydroxylase deficiency before puberty.

鼻用皮质类固醇的临床选择

鼻用皮质类固醇是治疗鼻部炎症的常用药物。本文系统介绍了药物的代谢过程、化学结构、分子作用机制,对比了不同药物的药理学指标和临床应用指标,为临床选择提出指导性建议。

不典型Ramsay-Hunt综合征1例

Ramsay-Hunt综合征是1907年Ramsay Hunt提出的由面神经膝状神经节炎引起的面瘫、耳部疱疹和耳痛三联征,面神经受侵犯最常见,也可侵犯III～XII脑神经,迷走神经受损报道较少。本文就作者诊治的1例进行分析，以提高对本症的认识。