ABSTRACT
OBJECTIVE@#To report the clinical and genetic characteristics of a rare case of Gitelman syndrome with comorbid Graves disease and ACTH-independent adrenocortical adenoma.@*METHODS@#A patient who had presented at the Nanchong Central Hospital on December 21, 2020 was selected as the study subject. Clinical data of the patient was collected. Whole-exome sequencing was carried out on DNA extracted from peripheral venous blood samples from the patient and her family members.@*RESULTS@#The patient, a 45-year-old woman, was found to have Graves disease, ACTH-independent Cushing syndrome, hypokalemia and hypomagnesemia following the discovery of an adrenal incidentaloma. MRI scan had revealed a 3.8 cm × 3.2 cm mass in the left adrenal gland. The mass was removed by surgery and confirmed as adrenocortical adenoma. DNA sequencing revealed that the patient and her sister have both harbored compound heterozygous variants of the SLC12A3 gene, namely c.1444-10(IVS11)G>A and c.179(exon1)C>T (p.T60M), which were respectively inherited from their father and mother. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c.1444-10(IVS11)G>A and c.179(exon1)C>T (p.T60M) were respectively classified as a variant of uncertain significance (PM2_Supporting+PP3) and a likely pathogenic variant (PM3_Strong+PM1+PP3).@*CONCLUSION@#The conjunction of Gitelman syndrome with Graves disease and adrenal cortex adenoma is rather rare. The newly discovered c.1444-10(IVS11)G>A variant of the SLC12A3 gene, together with the heterozygous variant of c.179(exon1)C>T (p.T60M), probably underlay the pathogenesis in this patient.
Subject(s)
Humans , Female , Middle Aged , Gitelman Syndrome/genetics , Adrenocortical Adenoma , Hypokalemia , Graves Disease/genetics , Mothers , Mutation , Solute Carrier Family 12, Member 3ABSTRACT
OBJECTIVE@#To investigate the coagulation function indicators and identify influence factors of hypercoagulability in patients with adrenocorticotropic hormone (ACTH) independent Cushing syndrome (CS).@*METHODS@#In our retrospective study, the electronic medical records system of Peking University First Hospital was searched for the patients diagnosed with ACTH independent CS on discharge from January 2014 to June 2019. Nonfunctional adrenal adenoma patients were chosen as control group and matched 1 ∶1 by body mass index (BMI), gender, and discharge date. Clinical features and coagulation function indicators were compared between the two groups.@*RESULTS@#In the study, 171 patients were included in each group. Compared with control group, activated partial thromboplastin time (APTT), and prothrombin time (PT) in ACTH independent CS group were significantly lower [(29.22±3.39) s vs. (31.86±3.63) s, P < 0.001; (29.22±3.39) s vs. (31.86±3.63) s, P < 0.001], and both D-dimer and fibrin degradation products (FDP) levels were significantly higher (P < 0.05). Percentage of APTT levels under the lower limit of reference range in the CS patients was significantly higher than that in nonfunctional group (21.6% vs. 3.5%, P < 0.001). Percentage of D-dimer levels over the upper limit of reference range in the CS patients was significantly higher than that in nonfunctional group (13.5% vs. 6.6%, P=0.041). There were three patients with deep venous thrombosis and one patient with pulmonary embolism in CS group, however none was in control group. The area under curve (AUC) of serum cortisol rhythm (8:00, 16:00 and 24:00) levels was negatively associated with the levels of PT (r=-0.315, P < 0.001) and APTT (r=-0.410, P < 0.001), and positively associated with FDP (r=0.303, P < 0.001) and D-dimer levels (r=0.258, P < 0.001). There were no differences in coagulation function indicators among different histopathologic subgroups (adrenocortical adenoma, adrenocortical hyperplasia, oncocytic adenoma, adrenocortical carcinoma). With Logistic regression analysis, the AUC of cortisol and glycosylated hemoglobin A1c (HbA1c) levels were independent risk factors for hypercoagulability in the ACTH independent CS patients (P < 0.05).@*CONCLUSION@#ACTH independent CS patients were more likely in hypercoagulable state compared with nonfunctional adrenal adenoma, especially in ACTH independent CS patients with higher levels of cortisol AUC and HbA1c. These patients should be paid attention to for the hypercoagulability and thrombosis risk.
Subject(s)
Humans , Cushing Syndrome/complications , Adrenocortical Adenoma/complications , Adrenocorticotropic Hormone , Hydrocortisone , Retrospective Studies , Glycated Hemoglobin , Adrenal Cortex Neoplasms/diagnosis , Adenoma/diagnosis , Thrombophilia/complicationsABSTRACT
O hiperaldosteronismo se define pela hipersecreção de aldosterona pelas suprarrenais, resultando em excesso de sódio e redução de potássio sanguíneo. Esta hipersecreção deve-se à síntese autônoma de aldosterona por células adrenais hiperplásicas ou neoplásicas, que agem independentemente da estimulação pelo sistema renina-angiotensina. A doença acomete felinos de adultos maduros a idosos. O excesso de aldosterona culmina em hipertensão sistêmica e/ou hipocalemia, que levam à fraqueza muscular e alterações oculares. O diagnóstico é baseado em exames laboratoriais e de imagem, e o tratamento pode ser clínico ou cirúrgico. O prognóstico é considerado favorável quando as medicações são capazes de melhorar as manifestações clínicas ou quando é possível realizar o procedimento cirúrgico. O presente trabalho visa relatar o caso de um felino macho de 13 anos, castrado, sem raça definida, com hipocalemia persistente secundária a um presuntivo tumor adrenal.
Hyperaldosteronism is defined by the hypersecretion of aldosterone by the adrenal glands resulting in excess sodium and reduced blood potassium. This hypersecretion is due to the autonomous synthesis of aldosterone by hyperplastic or neoplastic adrenal cells, which act independently of stimulation by the renin-angiotensin system. The disease affects felines in the age group from mature adults to the elderly. The excess of aldosterone culminates in systemic hypertension and/or hypokalemia, which leads to muscle weakness and ocular changes. The diagnosis is based on laboratory and imaging tests and treatment can be clinical or surgical. The prognosis is considered favorable when the medications are able to improve the clinical manifestations or when it is possible to perform the surgical procedure. The present paper aims to report the case of a 13-year-old male cat, castrated, crossbred, with persistent hypokalemia secondary to a presumptive adrenal tumor.
Subject(s)
Animals , Cats , Cats/abnormalities , Cats/physiology , Hyperaldosteronism/diagnosis , Hypertension , Adrenocortical Adenoma/diagnosisABSTRACT
El síndrome de Cushing (SC) endógeno se caracteriza por la sobreproducción crónica de cortisol y está asociado con aumento en la morbimortalidad. Presenta un conjunto de manifestaciones clínicas que pueden variar dependiendo del nivel de cortisol de cada paciente, siendo los signos más característicos de esta enfermedad la obesidad central con debilidad muscular proximal, hematomas, estrías violáceas mayores de 1 cm e hipertensión arterial.1,2 En niños la presentación clínica es diferente, siendo el aumento de peso y el retardo del crecimiento lo más frecuente.1 Se ha visto involucrado en el 2 al 3% los casos de pacientes con síndrome metabólico, principalmente diabetes mellitus e hipertensión arterial, que presentan pobre respuesta al tratamiento farmacológico
Endogenous Cushing syndrome (CS) is characterized by chronic overproduction of cortisol and is associated with increased morbidity and mortality. It presents a set of clinical manifestations that may vary depending on the level of cortisol in each patient, the most characteristic signs of this disease being central obesity with proximal muscle weakness, hematomas, purplish striae greater than 1 cm and arterial hypertension.1,2 In children the clinical presentation is different, with weight gain and growth retardation being the most frequent.1 Cases of patients with cortisol have been involved in 2 to 3%. metabolic syndrome, mainly diabetes mellitus and hypertension, which have a poor response to diabetes. pharmacological treatment
Subject(s)
Humans , Cushing Syndrome , Pregnancy , Laparoscopy , Adrenocortical AdenomaABSTRACT
Resumen Objetivo: Presentamos un caso clínico con diagnóstico de incidentaloma adrenal no funcionante asintomático y analizamos las implicaciones clínicas y el abordaje realizado. Caso clínico: Se reporta el caso de un masculino de 53 años, asintomático, con hallazgo ecográfico accidental de imagen hipoecoica de contornos bien definidos en la glándula suprarrenal derecha que presentó incremento en su tamaño. Su estudio hormonal fue negativo para hiperfunción adrenal. Resultados: Se realizó suprarrenalectomía laparoscópica con técnica de 4 trocares con resección completa de la lesión. El paciente presentó buena evolución posquirúrgica. El estudio anatomopatológico concluyó el diagnóstico de adenoma corticoadrenal no funcionante. Conclusión: Ante el hallazgo de una masa adrenal mayor de 1 cm corresponde realizar una identificación hormonal y una evaluación del riesgo de malignidad en los pacientes, los cuales, junto con parámetros imagenológicos y los síntomas presentados, permitirán definir las complicaciones en el manejo y el pronóstico del paciente. El diagnóstico diferencial de los adenomas adrenales está basado en la identificación hormonal, el conocimiento radiológico y el grado de compromiso de la lesión. El abordaje laparoscópico es de elección en las lesiones pequeñas y sin sospecha de malignidad.
Objective: We present a clinical case with diagnosis of an asymptomatic nonfunctional adrenal incidentaloma, in which we discuss the clinical implications and the approach. Clinical case: Male patient, 53 years old with an accidental sonographic finding, characterized by a hypoechoic image of well-defined contours in the right adrenal gland of less than 2 cm. The hormonal test showed no adrenal hyperfunctioning. Laparoscopic adrenalectomy technique is performed with 4 trocars with complete excision of the lesion. The patient presented good postoperative evolution. Results: The pathology study showed a well-defined and benign tumor lesion of the adrenal gland, being similar to the fascicular zone and cortical hyperplasia next to it. The diagnosis is a non-functioning adenoma of the adrenal gland derived from the fascicular zone. Conclusion: Given the finding of an adrenal mass greater than 1 cm mass corresponds perform a hormonal identification and risk assessment of malignancy in patients, which with imaging parameters (echogenicity, bilateralism and the adjacent commitment) and symptoms presented allow to identify the complications in the management and prognosis of the patient. The differential diagnosis of adrenal adenomas is based on the hormonal evaluation, radiological knowledge and the commitment of the injury.
Subject(s)
Humans , Male , Middle Aged , Laparoscopy , Adrenal Cortex Neoplasms/surgery , Adrenocortical Adenoma/surgery , Adrenalectomy/methods , Ultrasonography , Adrenal Cortex Neoplasms/diagnostic imaging , Adrenocortical Adenoma/diagnostic imaging , Incidental FindingsABSTRACT
Introducción: La Suprarrenalectomía quirúrgica es el tratamiento de elección en lesiones tumorales de la glándula suprarrenal mayores de 4 cm o aquellos que resulten funcionales. La técnica laparoscópica esta asociada a menor sangrado, morbilidad perioperatoria y estadía hospitalaria. El apoyo del abordaje single-port (LESS) se asocia además a menor dolor y mejores resultados estéticos, pero conlleva a una mayor complejidad técnica, sin embargo esta dificultad puede ser eliminada con el apoyo robótico, conservando los beneficios de la técnica single port.(AU)
Introduction: Surgical adrenalectomy is the treatment of choice in tumors of the adrenal gland larger than 4 cm or those that are functional. The laparoscopic technique is associated with less bleeding, perioperative morbidity and hospital stay. The support of the single-port approach (LESS) is also associated with less pain and better aesthetic results, but it leads to greater technical complexity, however this difficulty can be eliminated with robotic support, while retaining the benefits of the single port technique.
Subject(s)
Female , Laparoscopy , Adrenocortical Adenoma , Instructional Film and VideoABSTRACT
OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Ablation Techniques/methods , Adrenocortical Hyperfunction/surgery , Ethanol/therapeutic use , Tomography, X-Ray Computed/methods , Adrenal Cortex Neoplasms/surgery , Adrenal Gland Neoplasms/surgery , Adrenalectomy/methods , Adrenocortical Adenoma/surgery , Aldosterone/biosynthesis , Cushing Syndrome/surgery , Hyperplasia/surgery , Pheochromocytoma/surgery , Reproducibility of Results , Retrospective Studies , Treatment OutcomeABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical features of adrenocortical adenoma's diagnosis and treatment in patients aged 60 years or above.</p><p><b>METHODS</b>A retrospective study was performed with a total of 249 patients aged 60 years or above who suffered from adrenocortical adenoma and treated in Peking Union Medical College Hospital from January 2004 to January 2014.The clinical features, treatments and prognosis of the 249 patients aged 60 years or above were compared with another 249 patients which were randomly selected during the same period aged from 30 to 50 years.t-test or χ(2) test was used to analyze the data between the two groups.</p><p><b>RESULTS</b>Endocrine examinations were performed in all 249 patients aged 60 years or above.There were 144 patients diagnosed as non-functional adrenocortical adenoma, 94 cases as aldosterone-producing adenoma and 11 cases as Cushing adenoma.For the patients aged 60 years or above, the rate of cardio-cerebral vascular incident in non-functional adrenocortical adenoma group was 26.4%(38/144), which was significantly lower than that of the aldosterone-producing adenoma and Cushing adenoma group(54.3%, 57/105)(χ(2)=20.027, P=0.000). There were 91.5%(65/71) of the patients aged 60 years or above who got a relief in low blood potassium symptoms after the operation.Forty-nine point one percent(53/108) of the non-functional adrenocortical adenoma patients aged 60 years or above had a better control of their blood pressure level, while functional adrenocortical adenoma group were 64.0%(48/75) which indicated that the functional adrenocortical adenoma patients have a better control of their blood pressure then the non-functional adrenocortical adenoma patients after the operation(χ(2)=3.987, P=0.046). There were 37.1% of the patients aged 60 years or above whose fasting blood-glucose was higher than 7.1 mmol/L, while the patients aged from 30 to 50 years was 14.1%(χ(2)=22.02, P=0.000). The differences in plasma aldosterone and blood potassium between the patients aged 60 years or above and the patients aged from 30 to 50 years had statistical significance(t=10.48, -2.58; P=0.00, 0.01).</p><p><b>CONCLUSIONS</b>Most of the adrenocortical adenoma in patients aged 60 years or above is non-functional adrenocortical adenoma.Among who, patients with aldosterone-producing adenoma tend to have lower plasma aldosterone concentration and higher blood potassium level then the patients aged from 30 to 50 years.The patients aged 60 years or above with functional adrenocortical adenoma are tend to have severe cardio-cerebral vascular incidence.A few of non-functional adrenocortical adenoma patients who combine with hypertension can benefit for the operation.</p>
Subject(s)
Adult , Humans , Middle Aged , Adrenocortical Adenoma , Diagnosis , Therapeutics , Aldosterone , Metabolism , Blood Pressure , Hypertension , Prognosis , Retrospective StudiesABSTRACT
Rhabdomyolysis results from acute damage to skeletal muscles caused by various conditions, of which hypokalemia is a recognized but rare example. Although primary aldosteronism may cause severe hypokalemia leading to rhabdomyolysis, the potassium level of such patients can be within the normal range. Hypokalemia is most frequently triggered when these patients are exposed to an additional insult, such as diuretic stress. Similarly, overzealous consumption of ionic beverages with osmotic diuretic effects can cause hypokalemia. Here, we describe a patient with an aldosterone-secreting adrenal adenoma, who presented with acute rhabdomyolysis secondary to severe hypokalemia triggered by consumption of a large volume of ionic beverage for 3 weeks.
Subject(s)
Humans , Adenoma , Adrenocortical Adenoma , Beverages , Diuresis , Diuretics , Hyperaldosteronism , Hypokalemia , Muscle, Skeletal , Potassium , Reference Values , RhabdomyolysisABSTRACT
We report 2 cases of chronic estimated glomerular filtration rate (eGFR) decline after unilateral adrenalectomy due to primary aldosteronism. The patients were diagnosed with unilateral adrenal cortical adenoma releasing aldosterone. Two patients were examined for hypertension and hypokalemia. Unilateral laparoscopic adrenalectomy was performed in both cases, and pathology confirmed adrenal cortical adenoma. After adrenalectomy, hypertension and hypokalemia improved to within normal range. However, the eGFR decreased postoperatively, and abdominal computed tomography scan showed decreased kidney size compared to previous images. Kidney biopsy was performed to delineate the exact cause of renal function deterioration and revealed hypertensive changes with chronic interstitial changes, indicating that glomerular hyperfiltration with aldosterone excess masked renal function damage. Physicians have to consider the probability of postadrenalectomy eGFR decline related to chronic hypertensive change.
Subject(s)
Humans , Adrenalectomy , Adrenocortical Adenoma , Aldosterone , Biopsy , Glomerular Filtration Rate , Hyperaldosteronism , Hypertension , Hypokalemia , Kidney , Masks , Pathology , Reference Values , Renal Insufficiency, ChronicABSTRACT
17α-hydroxylase deficiency is a rare cause of congenital adrenal hyperplasia and is characterized by primary amenorrhea, delayed puberty and hypertension. Although 17α-hydroxylase deficiency mimics mineralocorticoid-induced hypertension, impaired sexual development can aid in the differential diagnosis of this disease. A 32-year-old woman, who had a history of testicular feminization syndrome, presented with hypertension. Her aldosterone level was elevated whereas plasma renin activity was reduced, and her computed tomography scan showed a left adrenal adenoma, which was thought to be an aldosterone producing adenoma. A left adrenalectomy was performed to treat hypertension; however, the condition did not improve. The hormonal tests revealed high levels of plasma progesterone, mineralocorticoid and adrenocorticotropic hormone, and low levels of 17a hydroxyprogesterone, cortisol and sex hormones. The patient was diagnosed with 17α-hydroxylase deficiency and commenced on prednisolone, which controlled hypertension. Here, we report a case of 17α-hydroxylase deficiency mimicking hyperaldosteronism via aldosterone-producing adrenal adenoma.
Subject(s)
Adult , Female , Humans , Male , Adenoma , Adrenal Hyperplasia, Congenital , Adrenalectomy , Adrenocortical Adenoma , Adrenocorticotropic Hormone , Aldosterone , Amenorrhea , Androgen-Insensitivity Syndrome , Diagnosis, Differential , Gonadal Steroid Hormones , Hydrocortisone , Hyperaldosteronism , Hypertension , Plasma , Prednisolone , Progesterone , Puberty, Delayed , Renin , Sexual DevelopmentABSTRACT
A alta prevalência de tumores da glândula adrenal deve-se, em parte, ao avanço dos métodos de imagem. Os adenomas, carcinomas e hiperplasias oriundos do córtex adrenal são responsáveis por 80 a 90% dos processos tumorais. Alguns casos são herdados e podem estar associados a efeito compressivo de massa tumoral, hipersecreção de esteroides ou manifestações clínicas em outros órgãos. Considerando as hiperplasias e tumores adrenocorticais, o objetivo desse trabalho foi auxiliar os médicos na identificação de pacientes que apresentem risco para doença hereditária. As neoplasias e hiperplasias adrenocorticais podem ser encontradas em síndromes hereditárias, como a síndrome de Li-Fraumeni, síndrome de Beckwith-Wiedemann, neoplasia endócrina múltipla do tipo I, síndrome de Gardner e no complexo de Carney. A hereditariedade também está associada com doenças adrenocorticais na hiperplasia adrenal congênita, no aldosteronismo primário e/ou na síndrome de Cushing (doença clínica ou subclínica) na hiperplasia adrenal macronodular primária. Essa revisão descreve as características clínicas e os defeitos genéticos responsáveis pelas síndromes hereditárias. Relacionamos também a classificação histopatológica dos processos expansivos com os principais sinais clínicos e os genes relacionados. A identificação de defeitos genéticos em células germinativas nessas doenças familiais permite o conhecimento de alterações somáticas em alguns tipos de processos tumorais adrenocorticais de etiologia esporádica. Considerando a prevalência dos tumores do córtex adrenal, a identificação de predisposição hereditária é essencial para assegurar a conduta clínica correta do paciente e o aconselhamento genético de seus familiares.
The adrenal gland tumors are prevalent due in part by the widespread use of imaging studies. Adenomas, carcinomas and hyperplasias, originating from the adrenal cortex, account for 80-90% of adrenal tumoral processes. Some cases are inherited and may be associated with local mass effect, steroid hypersecretion and/or clinical manifestation in other organs. In the context of adrenocortical tumors and hyperplasias, the purpose of this article is to assist physicians in identifying patients who may be at risk of hereditary diseases. Adrenocortical hyperplasias and neoplasias can be found in familial tumor syndromes, such as Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, multiple endocrine neoplasia type 1, Gardner syndrome and Carney complex. Heredity has been also associated with adrenocortical lesions in congenital adrenal hyperplasia, primary aldosteronism and/or Cushing syndrome (overt or subclinical disease) in primary macronodular adrenal hyperplasia (PMAH). This review describes the clinical recognition and genetic defects that have been found to be responsible for these hereditary diseases. Furthermore, we present the histopathologic classification of adrenocortical expansive processes in correlation to the main clinical features and related genes. The identification of germline genetic defects in such familial diseases lead to the identification of somatic alterations in a subgroup of sporadic adrenocortical lesions. Considering the prevalence of adrenocortical tumors, identification of a hereditary predisposition is essential to assure the adequate clinical management of the patient and to offer the genetic counselling to family members.
Subject(s)
Neoplastic Syndromes, Hereditary , Adrenal Cortex Neoplasms , Adrenocortical Adenoma , Adrenocorticotropic Hormone , Genetic Counseling , Beckwith-Wiedemann Syndrome , Gardner Syndrome , Li-Fraumeni Syndrome , Multiple Endocrine Neoplasia Type 1 , Genetic Diseases, Inborn/diagnosisABSTRACT
O hiperaldosteronismo primário é causa de hipertensão arterialsecundária, com possibilidade de cura após cirurgia em 30a 75% dos casos. O objetivo deste estudo foi relatar um casode hipertensão arterial secundária a adenoma adrenal produtorde aldosterona. Paciente feminino, 35 anos, natural de Beruri(AM), procedente de Manaus, foi admitida com pressão arterialde 220x125mmHg associada a fraqueza muscular de membrosinferiores e câimbras. Na história patológica pregressa, havia orelato de hipertensão arterial diagnosticada há 2 anos, em tratamentocom três classes de anti-hipertensivos. Durante internaçãoem hospital geral, o quadro relatado associado à hipocalemiae alcalose metabólica sugeriram diagnóstico de hipertensão arterialsecundária a hiperaldosteronismo primário. Níveis elevadosde aldosterona plasmática, com renina suprimida e relação aldosterona-renina elevada, confirmaram o diagnóstico de hiperaldosteronismoprimário. A tomografia computadorizada deabdome evidenciou lesão tumoral com 2,3x2,0cm em glândulasuprarrenal esquerda sugestiva de adenoma adrenal. Pacientefoi submetida a adrenalectomia à esquerda com histopatologiacompatível com adenoma adrenal. Seis meses após a cirurgia,paciente evoluiu com normalização da calemia, porém mantevehipertensão arterial com necessidade de terapia anti-hipertensiva,sem novos picos hipertensivos. Se hiperaldosteronismo primáriodiagnosticado precocemente, há possibilidade de cura dahipertensão arterial secundária após adrenalectomia, reduzindo o efeito deletério da mesma sobre os sistemas cardiovascular, cerebrovasculare renal.(AU)
The primary hyperaldosteronism is a cause of secondaryhypertension, with the possibility of healing after surgery in 30to 75% of cases. The objective of this study was to report a caseof secondary hypertension to aldosterone-producing adrenaladenoma. A female patient, 35 years, from Beruri (AM), Brazil,who came to Manaus, was admitted into the emergency room withblood pressure of 220x125mmHg, associated with lower limbmuscle weakness and cramps. In the past medical history, there wasa report of hypertension diagnosed 2 years before, and she was undertreatment with three classes of antihypertensive drugs. Duringhospitalization in a general hospital, the case reported associatedto hypokalemia and metabolic alkalosis suggested a diagnosis ofhypertension secondary to primary hyperaldosteronism. Elevatedlevels of plasmatic aldosterone, suppressed renin and highaldosterone-renin relation suggested primary hyperaldosteronism.The abdominal computerized tomography scan revealed a tumor of2.3x2.0cm in the left adrenal gland, suggesting adrenal adenoma.Patient underwent left adrenalectomy and histopathology wascompatible to adrenal adenoma. After 6 months of surgery, thepatient experienced normalization of kalemia but kept arterialhypertension, requiring anti-hypertensive therapy, but had no othershypertensive peaks. Early diagnosis of primary hyperaldosteronismallowed the cure of hypertension after adrenalectomy, reducingdeleterious effects of high blood pressure levels on the cardiovascular,cerebrovascular and renal systems.(AU)
Subject(s)
Humans , Female , Adult , Adrenocortical Adenoma/pathology , Hyperaldosteronism/diagnosis , Hypertension/etiology , Adrenalectomy/instrumentation , Aldosterone/chemistrySubject(s)
Humans , Adrenocortical Adenoma , Pseudohypoaldosteronism , Bartter Syndrome , Gitelman Syndrome , Liddle SyndromeABSTRACT
The simultaneous occurrence of renovascular hypertension and an aldosterone-producing adrenal adenoma is a rare entity. Here, we report the case of a 52-year-old female who had a coexisting aldosterone-producing adrenal adenoma and ipsilateral renal artery stenosis. She was diagnosed with the aldosterone-producing adrenal adenoma and then underwent a laparoscopic left adrenalectomy. Her blood pressure was uncontrolled after the adrenalectomy. Selective renal angiography showed left renal artery stenosis; thus, she underwent balloon angioplasty at the same sitting. Subsequently, her blood pressure returned to normal after administration of a single antihypertensive drug. This case suggests that it is important to recognize the possible coexistence of renal artery stenosis in a patient with an aldosterone-producing adrenal adenoma.
Subject(s)
Female , Humans , Middle Aged , Adenoma , Adrenalectomy , Adrenocortical Adenoma , Angiography , Angioplasty, Balloon , Blood Pressure , Hyperaldosteronism , Hypertension, Renovascular , Renal Artery ObstructionABSTRACT
A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses.
Subject(s)
Adult , Female , Humans , Adenoma , Adrenalectomy , Adrenocortical Adenoma , Aldosterone , Blood Pressure , Cushing Syndrome , Dexamethasone , Diagnosis , Hydrocortisone , Hyperaldosteronism , Hypertension , Hypokalemia , Korea , Plasma , VeinsABSTRACT
A substantial proportion of adrenal incidentalomas demonstrates subtle hormonal hypersecretion; however, adenomas that cosecrete aldosterone and cortisol are rare. We here report a case of an adrenal mass that was incidentally detected on a computed tomography scan in a 57-year-old man. The patient had a 10-year history of diabetes mellitus and a 5-year history of hypertension. Evaluation revealed hyperaldosteronemia with an elevated plasma aldosterone-to-renin ratio, hypokalemia, unsuppressed cortisol after dexamethasone administration, and elevated urinary free cortisol concentration. The appearance of the right adrenalectomy specimen indicated adrenal adenoma. Postoperatively, the blood glucose and blood pressure control improved and the urinary cortisol and aldosterone-to-renin ratio normalized. A complete endocrine evaluation in patients with incidentally discovered adrenal masses should be performed, even if the patient has a long-standing history of hypertension and diabetes, to avoid any postoperative adrenal crises.
Subject(s)
Humans , Middle Aged , Adenoma , Adrenalectomy , Adrenocortical Adenoma , Aldosterone , Blood Glucose , Blood Pressure , Dexamethasone , Diabetes Mellitus , Hydrocortisone , Hyperaldosteronism , Hypertension , Hypokalemia , PlasmaABSTRACT
Congenital adrenal hyperplasia (CAH) with 17alpha-hydroxylase/17,20-lyase deficiency is usually characterized by hypertension and primary amenorrhea, sexual infantilism in women, and pseudohermaphroditism in men. hypertension, and sexual infantilism in women and pseudohermaphroditism in men. In rare cases, a huge adrenal gland tumor can present as a clinical manifestation in untreated CAH. Adrenal cortical adenoma is an even more rare phenotype in CAH with 17alpha-hydroxylase/17,20-lyase deficiency. A 36-year-old female presented with hypertension and abdominal pain caused by a huge adrenal mass. Due to mass size and symptoms, left adrenalectomy was performed. After adrenalectomy, blood pressure remained high. Based on hormonal and genetic evaluation, the patient was diagnosed as CAH with 17alpha-hydroxylase/17,20-lyase deficiency. The possibility of a tumorous change in the adrenal gland due to untreated CAH should be considered. It is important that untreated CAH not be misdiagnosed as primary adrenal tumor as these conditions require different treatments. Adequate suppression of adrenocorticotropic hormone (ACTH) in CAH is also important to treat and to prevent the tumorous changes in the adrenal gland. Herein, we report a case of untreated CAH with 17alpha-hydroxylase/17,20-lyase deficiency presenting with large adrenal cortical adenoma and discuss the progression of adrenal gland hyperplasia due to inappropriate suppression of ACTH secretion.
Subject(s)
Adult , Female , Humans , Male , Abdominal Pain , Adrenal Glands , Adrenal Hyperplasia, Congenital , Adrenalectomy , Adrenocortical Adenoma , Adrenocorticotropic Hormone , Amenorrhea , Blood Pressure , Disorders of Sex Development , Hyperplasia , Hypertension , Phenotype , Sexual InfantilismABSTRACT
No abstract available.