Oncocitoma suprarrenal / Adrenal oncocytoma

Introducción: El oncocitoma suprarrenal es un tumor infrecuente e incidental y sin manifestaciones clínicas propias. Objetivo: Presentar un caso de oncocitoma suprarrenal y sus particularidades diagnósticas y terapéuticas. Caso clínico: Paciente masculino de 34 años de edad con antecedentes de salud, que se presentó por dolor lumbo-abdominal derecho, sin irradiación ni otros síntomas acompañantes. El examen físico fue normal. El ultrasonido informó un tumor de unos 7 cm de diámetro, localizado hacia el polo superior del riñón derecho. El origen suprarrenal se definió con la tomografía abdominal contrastada. Los valores sanguíneos de hormonas de la corteza suprarrenal fueron normales. Se hizo la exéresis total del tumor, mediante laparotomía convencional. El riñón estaba normal. El examen histopatológico notificó oncocitoma suprarrenal y lo ratificó la inmunohistoquímica. El paciente evolucionó satisfactoriamente. Conclusiones: El oncocitoma suprarrenal es un tumor infrecuente y de hallazgo fortuito. La tomografía abdominal contrastada no tiene alta especificidad para diferenciarlo de otros tumores suprarrenales. El perfil inmunohistoquímico del tumor es concluyente en el diagnóstico definitivo(AU)

Introduction: Adrenal oncocytoma is a rare and incidental tumor without its own clinical manifestations. Objective: To present a case of adrenal oncocytoma and its diagnostic and therapeutic characteristics. Clinical case: 34-year-old male patient with a medical history, who presented with right lumbo-abdominal pain, without radiation or other accompanying symptoms. The physical exam was normal. The ultrasound reported a tumor of about 7 cm in diameter, located towards the upper pole of the right kidney. The adrenal origin was defined with contrast abdominal tomography. The blood levels of hormones of the adrenal cortex were normal. Total excision of the tumor was performed by conventional laparotomy. The kidney was normal. Histopathological examination reported adrenal oncocytoma and immunohistochemistry confirmed it. The patient evolved satisfactorily. Conclusions: Adrenal oncocytoma is a rare and fortuitous tumor. Contrast abdominal tomography does not have high specificity to differentiate it from other adrenal tumors. The immunohistochemical profile of the tumor is conclusive in the definitive diagnosis(AU)

Neoplasia oncocítica adrenocortical de potencial maligno incierto: reporte de un caso / Adrenocortical oncocytic neoplasm with uncertain malignant potential: a case report

Resumen Objetivo Describir el caso clínico de una paciente con neoplasia oncocitica adrenocortical, tratado quirúrgicamente en una clínica de Lima, Perú. Caso clínico Paciente mujer de 26 años ingresa a emergencia por dolor abdominal inespecífico. Se evidencia tumoración de 15x14x12 cm dependiente de glándula suprarrenal izquierda por lo que se decide tratamiento quirúrgico. Al análisis patológico se evidencia neoplasia oncocítica de potencial maligno incierto. Discusión Las neoplasias oncocíticas adrenocorticales son entidades poco frecuentes, con escasos reportes de casos de esta enfermedad. Para clasificarlas, se usa la escala de Weiss modificada. Obtenemos una neoplasia oncocítica de potencial maligno incierto, cuyo tratamiento incluye la cirugía de resección de tumor y observación. Conclusión Considerar a las neoplasias oncocíticas dentro del diagnóstico diferencial de incidentalomas adrenales.

Objective To describe a case report of a oncocytic adrenocortical neoplasm, treated surgically in a clinic in Lima, Peru. Case report A 26-year-old woman is admitted to the emergency due to nonspecific abdominal pain. A tumor measuring 15x14x12 cm dependent on left adrenal gland is evidenced, so surgical treatment is decided. Pathological analysis evidences oncocytic neoplasia of uncertain malignant potential. Discussion Oncocytic adrenocortical neoplasms are rare entities, with few case reports of this disease. To classify them, the modified Weiss scale is used. We obtain an oncocytic neoplasm of uncertain malignant potential, whose treatment includes surgery for tumor resection and observation. Conclusion Consider oncocytic neoplasms within the differential diagnosis of adrenal incidentalomas.

Construction of transgenic mice with specific Cre recombinase expression in the zona fasciculata in adrenal cortex / 生理学报

The adrenal gland is an important endocrine organ of human body. CYP11B1 gene was specifically expressed in the zona fasciculata in adrenal cortex. In order to better study the function of genes specifically expressed in the zona fasciculata in adrenal cortex, the mice with Cre recombinase specifically expressed in the zona fasciculata in adrenal cortex were constructed. It was then confirmed that CYP11B1 was specifically expressed in adrenal glands. Then, using CRISPR/Cas9 technique, CYP11B1-2A-GfpCre recombinant vector was constructed and subsequently injected into the fertilized eggs of mice. It was confirmed that the Cre gene was mainly expressed in the zona fasciculata in adrenal cortex of CYP11B1Cre mice by using mTmG and LacZ staining. The CYP11B1Cre mice were then mated with cystathionine γ-lyase (CTH) mice, thereby generating CTH/CYP11B1Cre mice. It was also confirmed that CTH gene in the zona fasciculata in adrenal cortex was specifically knocked out in these mice. These results suggest that transgenic mice with specific Cre recombinase expression in the zona fasciculata in adrenal cortex were constructed successfully. This animal model can be a powerful tool for the study of the function of genes expressed in the zona fasciculata in adrenal cortex.

Adrenal Cortical Neoplasm with Uncertain Malignant Potential Arising in the Heterotopic Adrenal Cortex in the Liver of a Patient with Beckwith-Wiedemann Syndrome

Patients with Beckwith-Wiedemann syndrome (BWS) are predisposed to developing embryonal tumors, with hepatoblastoma being the most common type. Our patient showed hemihypertrophy, macroglossia, and paternal uniparental disomy in chromosome 11 and was diagnosed with BWS. When the patient was 9 months old, a 2.5×1.5 cm oval hypoechoic exophytic mass was detected in the inferior tip of his right liver. Preoperative imaging identified it as hepatoblastoma; however, histologic, immunohistochemistry, and electron microscopic findings were compatible with adrenal cortical neoplasm with uncertain malignant potential. The origin of the adrenal tissue seemed to be heterotopic. Here, we describe for the first time an adrenal cortical neoplasm with uncertain malignant potential arising in the heterotopic adrenal cortex located in the liver of a patient with BWS.

Bilateral Avascular Necrosis of the Femoral Head in a Patient with Asymptomatic Adrenal Incidentaloma / 대한고관절학회지

Except when caused by direct and definite mechanisms (e.g., injury of the vessels to the femoral head), the pathophysiology of avascular necrosis of the femoral head has not yet been fully elucidate. While non-traumatic avascular necrosis of the femoral head is known to be caused by alcohol, steroids and various diseases, it may also occur without such events in a patient's history. Herein, a case of bilateral avascular necrosis of the femoral head caused by asymptomatic adrenal cortex incidentaloma which was initially misdiagnosed as idiopathic is reported along with a literature review.

Molecular Mechanisms of Primary Aldosteronism

Primary aldosteronism (PA) results from excess production of mineralocorticoid hormone aldosterone by the adrenal cortex. It is normally caused either by unilateral aldosterone-producing adenoma (APA) or by bilateral aldosterone excess as a result of bilateral adrenal hyperplasia. PA is the most common cause of secondary hypertension and associated morbidity and mortality. While most cases of PA are sporadic, an important insight into this debilitating disease has been derived through investigating the familial forms of the disease that affect only a minor fraction of PA patients. The advent of gene expression profiling has shed light on the genes and intracellular signaling pathways that may play a role in the pathogenesis of these tumors. The genetic basis for several forms of familial PA has been uncovered in recent years although the list is likely to expand. Recently, the work from several laboratories provided evidence for the involvement of mammalian target of rapamycin pathway and inflammatory cytokines in APAs; however, their mechanism of action in tumor development and pathophysiology remains to be understood.

The human adrenal cortex: growth control and disorders

This review summarizes key knowledge regarding the development, growth, and growth disorders of the adrenal cortex from a molecular perspective. The adrenal gland consists of two distinct regions: the cortex and the medulla. During embryological development and transition to the adult adrenal gland, the adrenal cortex acquires three different structural and functional zones. Significant progress has been made in understanding the signaling and molecules involved during adrenal cortex zonation. Equally significant is the knowledge obtained regarding the action of peptide factors involved in the maintenance of zonation of the adrenal cortex, such as peptides derived from proopiomelanocortin processing, adrenocorticotropin and N-terminal proopiomelanocortin. Findings regarding the development, maintenance and growth of the adrenal cortex and the molecular factors involved has improved the scientific understanding of disorders that affect adrenal cortex growth. Hypoplasia, hyperplasia and adrenocortical tumors, including adult and pediatric adrenocortical adenomas and carcinomas, are described together with findings regarding molecular and pathway alterations. Comprehensive genomic analyses of adrenocortical tumors have shown gene expression profiles associated with malignancy as well as methylation alterations and the involvement of miRNAs. These findings provide a new perspective on the diagnosis, therapeutic possibilities and prognosis of adrenocortical disorders.

Corticosteroids Treatment in Spinal Cord and Neuromuscular Disorders

Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of the vertebrates, as well as the synthetic analogs of these hormones that are synthesized in the laboratories. Two main classes of corticosteroids, glucocorticoids, and mineralocorticoids, are involved in a wide range of physiologic processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior. Corticosteroids have been used for almost 60 years in medicine and their roles in patients have always been discussed by researchers and clinicians dedicated in the related field. Currently, they are still used in the treatment of patients with neurological disorders. Usually, corticosteroids are used in the treatment of various inflammatory diseases and conditions. In this review, we present five key indications, i.e., neuromyelitis optica, acute spinal cord injury, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, polymyositis/dermatomyositis for the systemic use of corticosteroids in neurology based on a mix of quality of evidence, prevalence, and impact on disease management.

A novel mutation in the DAX1 gene in a newborn with adrenal hypoplasia congenita in Korea

Adrenal hypoplasia congenita (AHC) is a rare cause of adrenal insufficiency during neonatal period. Mutations in the gene coding for DAX1 cause X-linked adrenal hypoplasia. Most affected patients are shown to have salt wasting and hyperpigmentation on the skin during the neonatal period and require intensive medical care. In addition, it is usually associated with hypogonadotropic hypogonadism in adolescence. The DAX1 gene is expressed in the adrenal cortex, pituitary gland, hypothalamus, testis, and ovary. We report on a patient with genetically confirmed AHC whose initial clinical presentations were consistent with congenital adrenal hyperplasia. A point mutation in the DAX1 gene identified in this report resulted in a truncated DAX1 protein. Our patient was diagnosed with AHC.

Effect of Osthole on Adrenocortical Function in Y1 Mouse Adrenocortical Tumor Cells / 中国中西医结合杂志

<p><b>OBJECTIVE</b>To study the effect of osthole (Ost) on adrenocortical function in Y1 mouse adrenocortical tumor cells.</p><p><b>METHODS</b>Y1 mouse adrenocortical tumor cells were taken as subjects in this experiment. In 10.0%, 1.0%, and 0.1% serum DMEM-F12 medium, Y1 cells were treated with 1, 10, 25, 50, 100, and 200 micromol/L Ost for 24 and 48 h. 0.1% Dimethyl Sulfoxide (DMSO) was taken as negative control group and 1 mmol/L (Bu) 2cAMP as positive control group. Cell growth morphology was observed under inverted microscope. Contents of corticosterone were tested by ELISA. Expression levels of steroids synthase such as Star, Cyp11a1, Cyp21a1, Hsd3b2, Cyp11b1, Cyp11b2, Cyp17a1, and Hsd17b3 mRNA were detected by Real time quantitative PCR (RT-qPCR).</p><p><b>RESULTS</b>Y1 cell proliferation was obviously inhibited by 100 and 200 micromol/L Ost, and its inhibitory effect was more significant in 0.1% serum medium. Compared with the negative control group, gene expressions of Star, Cyp11a1 , Cyp21a1, Hsd3b2, Cyp11b1, Cyp17a1, and Hsd17b3 were significantly enhanced in the posi- tive control group (P < 0.05). Y1 cell corticosterone levels significantly increased in 50 micromol/L Ost treatment group after 24-and 48-h intervention (P < 0.05). Contents of corticosterone increased more obviously in 25 and 50 +/- mol/L Ost treatment groups after 48-h intervention, as compared with 24-h intervention (P < 0.01). After 24-h intervention, expression levels of Star, Cyp21a1, and Hsd3b2 genes were significantly up-regulated in 25 and 50 lLmol/L Ost groups (P < 0.05). Star gene expression was further enhanced after 48-h intervention (P < 0.05). However, Ost showed no effect on Cyp11a1 (P > 0.05). Additionally, gene expressions of Cyp11b1 and Cyp17a1 were significantly enhanced by 10, 25, and 50 pLmolIL Ost after treatment for 24 and 48 h (P < 0.05). Ost showed no obvious effect on Cyp11b2 and Hsd17b3 expressions.</p><p><b>CONCLUSION</b>Ost could regulate adrenal cortex function and promote corticosterone synthesis and secretion through strengthening gene expressions of steroidogenic enzymes.</p>

Primary Adrenal Insufficiency in a Newborn With Adrenal Hypoplasia Congenita Caused by a Mutation of the DAX1 Gene

Adrenal hypoplasia congenita (AHC) is a rare inherited disorder of the adrenal gland caused by deletion or mutation of the dosage-sensitive sex-reversal AHC critical region on the X chromosome, gene 1 (DAX1) gene. The DAX1 gene is expressed in the adrenal cortex, the pituitary gland, the hypothalamus, the testis, and the ovary. Most affected infants present with failure to thrive, salt wasting, and hypoglycemic seizure in early life. Immediate mineralocorticoid and glucocorticoid replacement is essential. Most boys with AHC present with hypogonadotropic hypogonadism, resulting in failure to enter puberty and the need for testosterone treatment. However, a recent study revealed that the onset of puberty in boys with AHC can be variable, ranging from arrested or absent to precocious. We describe a case involving a newborn who presented with primary adrenal insufficiency due to a mutation of the DAX1 gene and was finally diagnosed with AHC.

POD-1/Tcf21 overexpression reduces endogenous SF-1 and StAR expression in rat adrenal cells

During gonad and adrenal development, the POD-1/capsulin/TCF21transcription factor negatively regulates SF-1/NR5A1expression, with higher SF-1 levels being associated with increased adrenal cell proliferation and tumorigenesis. In adrenocortical tumor cells, POD-1 binds to the SF-1 E-box promoter region, decreasing SF-1 expression. However, the modulation of SF-1 expression by POD-1 has not previously been described in normal adrenal cells. Here, we analyzed the basal expression of Pod-1 and Sf-1 in primary cultures of glomerulosa (G) and fasciculata/reticularis (F/R) cells isolated from male Sprague-Dawley rats, and investigated whether POD-1 overexpression modulates the expression of endogenous Sf-1 and its target genes in these cells. POD-1 overexpression, following the transfection of pCMVMycPod-1, significantly decreased the endogenous levels of Sf-1 mRNA and protein in F/R cells, but not in G cells, and also decreased the expression of the SF-1 target StAR in F/R cells. In G cells overexpressing POD-1, no modulation of the expression of SF-1 targets, StAR and CYP11B2, was observed. Our data showing that G and F/R cells respond differently to ectopic POD-1 expression emphasize the functional differences between the outer and inner zones of the adrenal cortex, and support the hypothesis that SF-1 is regulated by POD-1/Tcf21 in normal adrenocortical cells lacking the alterations in cellular physiology found in tumor cells.

Effect of Schisandra chinensis on interleukins, glucose metabolism, and pituitary-adrenal and gonadal axis in rats under strenuous swimming exercise / 中国结合医学杂志

<p><b>OBJECTIVE</b>To investigate the effect of Chinese medicine (CM) Schisandra chinensis on interleukin (IL), glucose metabolism, and pituitary-adrenal and gonadal axis of rats after strenuous navigation and exercise.</p><p><b>METHODS</b>A total of 45 Sprague-Dawley rats were randomized into the quiet control group, the stress group, and the CM group (15 in each group). The CM group received 2.5 g/kg of Schisandra chinensis twice per day for one week before modeling. Except the quiet controls, rats were trained using the Bedford mode for 10 days. On the 11th day, they performed 3 h of stressful experimental navigation and 3 h of strenuous treadmill exercise. The levels of serum testosterone (T), cortisol (CORT), luteinizing hormone (LH), IL-1, IL-2, and IL-6 were tested by radioimmunoassay and enzyme-linked immunosorbent assay, respectively. The adrenal cortex ultrastructure was observed using electron microscopy.</p><p><b>RESULTS</b>Compared with the quiet control group, after navigation and strenuous exercise, blood glucose was increased, and T level was decreased in the stress group (both P<0.01). The blood glucose, CORT, IL-1 and IL-2 levels were significantly reduced in the CM group (P<0.05 or P<0.01) as compared with the stress group. Electron microscopy revealed that the rats in the CM group had a smaller decrease in adrenal intracellular lipid droplets and higher levels of apoptosis than those in the stress group.</p><p><b>CONCLUSIONS</b>Schisandra chinensis can reduce serum CORT and blood glucose levels in stressed rats. It appears to protect the cell structure of the adrenal cortex, and offset the negative effects of psychological stress and strenuous exercise related to immune dysfunction. Schisandra chinensis plays a regulatory role in immune function, and can decrease the influence of stress in rats.</p>

Effect of induced diabetes mellius on the histological structure of the renal cortex of adult male albino rats and the possible protective role of licorice extract

Diabetes is one of the most common chronic metabolic disorders worldwide. One of the major complications of type 2 diabetes is diabetic nephropathy. The present study was to investigate the effect of type 2 diabetes on the histological structure of the renal cortex of adult male albino rats and the role of licorice ethanolic extract on diabetic renal affection. Forty adult male albino rats were utilized. They were classified into three main groups: the control group [group I], the experimental diabetic group [group II], and the possible protected group [group III]. Type 2 diabetes was induced in rats in groups II and III by giving them a high-fat diet and a single low dose of streptozotocin. Diabetic rats were divided into two subgroups: untreated subgroup IIa and treated subgroup IIb. The possible protected group received licorice ethanolic extract concomitant with the high-fat diet and the single low dose of streptozotocin. At the end of the experiment, the kidneys were dissected out and processed for light and electron microscopic examination. Fasting blood glucose level, fasting insulin level, serum urea, and creatinine were estimated and statistically analyzed. Examination of the renal cortex of untreated diabetic subgroup IIa demonstrated glomerulosclerosis and distorted podocyte foot processes. The cells lining convoluted tubules revealed thick basement membranes, disorganization of basal infoldings, and mitochondrial disarrangement. The area% of positive Bax immunoreaction was significantly increased in subgroup IIa as compared with subgroup IIb and group III. Examination of the renal cortex of the treated diabetic animals [subgroup IIb] revealed little improvement and failure of licorice extract to normalize renal cortical changes, most probably due to late intervention. In contrast, the protected group [group III] revealed a nearly preserved normal architecture. Changes in the renal cortical structure were attenuated with prophylactic therapy of licorice ethanolic extract

Role of losartan on the renal cortex damage induced by cisplatin in adult male albino rats

Cisplatin is a potent anticancer agent for the treatment of solid tumors, but its use is limited by its adverse effects. This research aimed to evaluate the effect of losartan on cisplatin nephrotoxicity in adult male albino rats. Twenty-five male albino rats were divided into four groups. The first was the control group. Group II received a single injection of cisplatin. Group III received losartan for 6 days. Group IV received a single injection of cisplatin in addition to losartan ingestion for 6 days. Renal tissues were prepared for light and transmission electron microscopic examinations. Glomerular diameter and cell number were measured by means of an image analyzer. Also, glomerular capillary basement membrane [GBM] thickness, filtration slit width, and pedicel length were recorded and statistically analyzed. The renal cortical sections of group II rats showed loss of normal appearance of renal corpuscles with enlarged glomeruli and increased glomerular cellularity. Degenerative changes and necrosis of the lining cells of proximal convoluted tubule were noted. Intertubular hemorrhage and cellular infiltration were detected. Electron microscopic examination revealed fusion of the foot processes with significant increase in the thickness of GBM and filtration slit width. Damage of proximal convoluted tubule cells was seen more than damage of distal convoluted tubules. The renal cortical sections of group III rats showed vacuolated cytoplasm of some tubules lining cells. Electron microscopic examination revealed some ultrastructural changes in the distal convoluted tubule lining cells. Most of the renal corpuscles of rats treated with losartan and cisplatin appeared normal, with presence of some enlarged corpuscles as well as cytoplasmic vacuolation of the tubular cells. Electron microscopic examination revealed a significant decrease in GBM thickness and filtration slit width in comparison with cisplatin-received rats. From this study, it was obvious that losartan ameliorates the histological changes induced by cisplatin given at a dose of 3 mg/kg in adult male rats

Effect of aqueous extract of Hibiscus sabdariffa on hyperthyroidism-induced changes in the renal cortex of rats: a histological study

Hyperthyroidism is a condition resulting from hypersecretion of thyroid hormones [T3 and T4]. It affects multiple organ systems, including the renal system. This study was set to evaluate the protective effect of Hibiscus sabdariffa [roselle] on the damaging effect induced by hyperthyroid state in the rat renal cortex. Twenty-four adult male albino rats were used and divided into four groups of six rats each. Group I was the control group. In group II, the rats received aqueous extracts of roselle at a daily dose of 500 mg/kg body weight. In group III, the rats were given a daily oral dose of thyroxin [100 micro g/kg body weight] dissolved in distilled water through gavage for 1 month. In group IV, the rats were given an aqueous extract of roselle at a daily dose of 500 mg/kg body weight 3 h before thyroxin administration at 100 micro g/kg body weight. At the end of the experimental period, blood samples wer collected for thyroid hormone [T3 and T4] assay. Kidney specimens were processed for immunohistochemical and histological study using light and electron microscopes. Morphometric analysis of the proximal convoluted tubule [PCT] diameter was carried out. A statistically significant elevation in the levels of T3 and T4 was observed in the thyroxin-treated group. Also, a significant increase in the diameter of PCT was detected in this group. Histologically, some malpighian corpuscles were partially atrophied. Effacement of podocyte foot processes with thickening of the filtration barrier was observed. In addition, tubulointerstitial injury in the form of PCT dilatation, peritubular hemorrhage, and inflammatory cellular infiltration was also seen. Immunohistochemical examination of the thyroxin-treated group revealed excess actin fibers in the PCT cells, indicating exposure of these cells to stress. Furthermore, there was significant improvement in the histological and immunohistochemical pictures toward normal in the thyroxin and roselle-treated group. Roselle has a potent protective effect against the damaging effect induced by the hyperthyroid state in the rat renal cortex

Adrenal Cortical Scintigraphy for Lateralization of Bilateral Adrenal Nodules in Primary Aldosteronism

No abstract available.

A Korean boy with atypical X-linked adrenoleukodystrophy confirmed by an unpublished mutation of ABCD1 / 소아과

X-linked adrenoleukodystrophy (X-ALD) is a rare peroxisomal disorder, that is rapidly progressive, neurodegenerative, and recessive, and characteristically primary affects the central nervous system white matter and the adrenal cortex. X-ALD is diagnosed basaed on clinical, radiological, and serological parameters, including elevated plasma levels of very long chain fatty acids (VLCFA), such as C24:0 and C26:0, and high C24:0/C22:0 and C26:0/C22:0 ratios. These tests are complemented with genetic analyses. A 7.5-year-old boy was admitted to Department of Pediatrics, Chungnam National University Hospital with progressive weakness of the bilateral lower extremities. Brain magnetic resonance imaging confirmed clinically suspected ALD. A low dose adrenocorticotropic hormone stimulation test revealed parital adrenal insufficiency. His fasting plasma levels of VLCFA showed that his C24:0/C22:0 and C26:0/C22:0 ratios were significantly elevated to 1.609 (normal, 0-1.390) and 0.075 (normal, 0-0.023), respectively. Genomic DNA was extracted from peripheral whole blood samples collected from the patient and his family. All exons of ABCD1 gene were amplified by polymerase chain reaction (PCR) using specific primers. Amplified PCR products were sequenced using the same primer pairs according to the manufacturer's instructions. We identified a missense mutation (p.Arg163Leu) in the ABCD1 gene of the proband caused by the nucleotide change 488G>T in exon 1. His asymptomatic mother carried the same mutation. We have reported an unpublished mutation in the ABCD1 gene in a patient with X-ALD, who showed increased ratio of C24:0/C22:0 and C26:0/C22:0, despite a normal VLCFA concentrations.

Insuficiência adrenal aguda / Acute adrenal insufficiency

A insuficiência adrenal (IA) consiste em síndrome clínica rara, decorrente da deficiência de glicocorticoides e/ou mineralocorticoides, podendo ser primária. A insuficiência adrenal aguda consiste em emergência endócrina rara, resultante da diminuição súbita do cortisol circulante, ou de aumento significativo da demanda por esse hormônio em pacientes com algum grau de disfunção adrenal, ocorrendo mais frequentemente no contexto da IA primária. O prognóstico da doença depende do reconhecimento e intervenção terapêutica precoces

Adrenal insuficiency (AI) consists of a rare clinical syndrome resulting from glucocorticoids and/or mineralocorticoids deficiency. Adrenal insufficiency may be primary. The acute AI is a rare endocrine emergency resulting from sudden decrease of circulating cortisol or, elevated demand for this hormone in patients with some degree of adrenal disfunction, occuring more frequently in primary AI. The prognosis depends on early recognition and precocious therapeutic intervention