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- Neonatal Bartter syndrome associated with ileal atresia and cystic fibrosis. [Journal Article]
- Indian J Nephrol 2013 Jan; 23(1):47-50.
A rare case of neonatal Bartter syndrome presenting with severe hyperkalemia is reported in a preterm child born to consanguineous parents. This child also had ileal atresia, and meconium plugs were found at laparotomy. The diagnosis of cystic fibrosis was subsequently made on genetic testing. Despite full intensive care management and surgical interventions, he died of respiratory failure after 70 days. This is the first reported case of such conglomeration of pathologies in a newborn child. Second, in highlighting this case we want clinicians to be aware that a subtype of neonatal Bartter syndrome can present with initial hyperkalemia so that an erroneous diagnosis of pseudohypoaldosteronism is not made when this is seen in combination with hyperkalemia and hyperrenin hyperaldosteronism.
- Adrenal incidentalomas: A collection of six interesting cases and brief review of literature. [Journal Article]
- Indian J Endocrinol Metab 2012 Dec; 16(Suppl 2):S378-81.
Adrenal incidentalomas (AI) are detected in approximately 4-7% of patients in imaging studies. Majority are benign, but careful evaluation is warranted to rule out carcinoma and functional adenomas.The purpose of presenting these cases is to highlight the approach to management of AI in terms of diagnosis, follow-up, and treatment.Seven patients presenting in the endocrine clinic with AI were evaluated for their presenting clinical features and investigated.Case 1 was a 49-year-old female, with adrenal androgen secreting adrenocortical carcinoma with amenorrhoea which was mistaken as menopause. She had minimal hirsutism, which was mistaken as postmenopausal hirsutism. Case 2 was a 39-year-old male, presenting with hyperglycemia found to have Conns' syndrome with aldosterone producing adenoma on routine ultrasound. Case 3 was a 32-year-old male, presenting with gastritis and bloating, where ultrasound showed bilateral large adrenal masses revealed as diffuse large B cell lymphoma on biopsy. Case 4 was a 21-year-old boy, who had pheochromocytoma misdiagnosed as benign intracranial hypertension (HTN). Case 5 was a 59-year-old hypertensive male, presenting with fever had pheochromocytoma with catecholamine excess, producing fever. Case 6 was isolated adrenal tuberculosis who presented with chronic diarrhea.AI are common, though prevalence varies depending on the reason for scanning, the characteristics of the population studied, and the radiological techniques used. Most are non-secreting cortical adenomas. AI should be evaluated both biochemically and radiologically. When a hormonal disorder is suspected clinically, targeted, diagnostic testing for autonomous cortisol secretion, pheochromocytoma, and hyperaldosteronism is indicated.
- Hormones other than aldosterone may contribute to hypertension in 3 different subtypes of primary aldosteronism. [Journal Article, Research Support, Non-U.S. Gov't]
- J Clin Hypertens (Greenwich) 2013 Apr; 15(4):264-9.
Blood pressure (BP) level is similar in patients with 3 subtypes of primary aldosteronism (PA), even though aldosterone levels may vary. Glucocorticoids and adrenomedullary hormones may be influenced and may contribute to hypertension in PA. The authors' objective was to investigate the influence of PA on adrenal gland secretion and the roles of these hormones in hypertension. Patients diagnosed with PA (229 cases) were enrolled and classified into 3 subgroups: aldosterone-producing adenoma (APA), unilateral nodular adrenal hyperplasia (UNAH), and idiopathic hyperaldosteronism (IHA). Patients with essential hypertension served as the control group (100 cases). Concentration of the above hormones was measured and compared between groups. Level of plasma adrenocorticotrophic hormone (ACTH) in patients with APA was significantly lower than that in patients with IHA (P<.001) and UNAH (P<0.5). The 24-hour urinary free cortisol and adrenomedullary hormone levels were highest in patients with IHA, lower in patients with APA, and lowest in patients with UNAH. Systolic BP level was positively correlated with 8 am plasma cortisol level (r=0.142, P=.039) and plasma ACTH level (r=0.383, P=.016). Cortisol and adrenomedullary hormones were different between PA subtypes and they might involve regulation of BP in those patients.
- Aldosterone Directly Affects Apelin Expression and Secretion in Adipocytes. [JOURNAL ARTICLE]
- J Mol Endocrinol 2013 Apr 2.
There is a high incidence of metabolic syndrome among patients with primary aldosteronism (PA), which has been recently associated with an unfavorable cardiometabolic profile. However, the underlying mechanisms have not been clarified in detail. Characterizing aldosterone target genes in adipocytes will help elucidate the deleterious effects associated with aldosterone excess. Apelin, a novel adipokine, exerts beneficial effects on obesity-associated disorders and cardiovascular homoeostasis. The objective of the study is to investigate the effect of high aldosterone levels on apelin expression and secretion and the underlying mechanisms involved in adipocytes. In vivo, a single dose aldosterone injection acutely decreased apelin serum levels and its adipose tissues production, which demonstrated a clear inverse relationship between the level of plasma aldosterone and plasma apelin. Experiments using 3T3-L1 adipocytes showed that aldosterone decreased apelin expression and secretion in a time- and dose-dependent manner. This effect was reversed by glucocorticoid receptor (GR) antagonists or GR knockdown; meanwhile, putative HREs in the apelin promoter were identified. Subsequently, we verified that both glucocorticoids and mineralocorticoids regulated apelin expression through GR activation although no synergistic effect was observed. Additionally, detailed potential mechanisms involved a p38 MAPK signaling pathway. In conclusion, our findings strengthen a direct interaction between aldosterone and apelin in adipocytes, which has important implications for hyperaldosteronism or PA associated cardiometabolic syndrome and hoists apelin on the list of potent therapeutic target for PA.
- Adrenal venous sampling for primary hyperaldosteronism in patients with concurrent chronic kidney disease. [Journal Article]
- J Vasc Interv Radiol 2013 May; 24(5):726-33.
To assess the risk of developing contrast-induced nephropathy (CIN) and to evaluate the technical success of adrenal venous sampling (AVS) in patients with chronic kidney disease (CKD).AVS was performed in 25 patients with primary hyperaldosteronism and concurrent CKD to distinguish between unilateral and bilateral adrenal disease. One of the 25 patients underwent repeat AVS, for a total of 26 samplings. All patients received a hydration protocol before and after the procedure. Acute kidney injury (AKI) (increase in creatinine of 0.5 mg/dL or>25% above baseline) and diagnostic yield were determined.CKD was stage III in 20 patients (80%), stage IV in 4 patients (16%), and stage V in 1 patient (4%). Median contrast volume was 25 mL (range, 10-250 mL). Of 26 studies, 25 (96%) were diagnostic; the one nondiagnostic AVS was repeated with success. Despite their elevated risks, only 2 of 25 patients (8%) developed AKI, and neither patient required treatment.AVS can be performed safely with a high degree of technical success and low risk of CIN in patients with primary hyperaldosteronism and concurrent advanced CKD.
- Association of CYP11B2 polymorphisms with susceptibility to primary aldosteronism: a meta-analysis. [JOURNAL ARTICLE]
- Endocr J 2013 Mar 27.
The association of CYP11B2 gene polymorphisms with the risk of primary aldosteronism (PA) was controversial in previous studies. Here we selected two commonly studied CYP11B2 alleles: T-344C, A2718G to explore their associations with PA risk by meta-analyses of published case-control studies. Six electronic databases were searched for relevant studies up to November 2012. Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated using random or fixed effects model. Seven studies (621 cases and 1027 controls) on T-344C polymorphism, three studies (327 cases and 336 controls) on A2718G polymorphism were finally included. Then signi?cant association was observed between T-344C polymorphism and idiopathic hyperaldosteronism (IHA) under three genetic models (CC vs TT, OR=0.544, 95%CI=0.324~0.914; CT vs TT, OR=0.554, 95%CI=0.406~0.757; CC+CT vs TT, OR=0.542, 95%CI=0.402~0.731). But patients with aldosterone-producing adenoma had no signi?cant association with T-344C polymorphism under all genetic models except CT vs TT model. Concerning A2718G polymorphism, a decreased PA risk was observed only under GG+GA vs AA model. But this association disappeared after removing the studies not in Hardy-Weinberg equilibrium. The evidence accumulated suggested that -344C allele may be associated with decreased risk of IHA and there was still no enough evidence to indicate the association of A2718G polymorphism with PA risk.
- Adrenal Diseases During Pregnancy: Pathophysiology, Diagnosis and Management Strategies. [JOURNAL ARTICLE]
- Am J Med Sci 2013 Mar 19.
: Adrenal diseases-including disorders such as Cushing's syndrome, Addison's disease, pheochromocytoma, primary hyperaldosteronism and congenital adrenal hyperplasia-are relatively rare in pregnancy, but a timely diagnosis and proper treatment are critical because these disorders can cause maternal and fetal morbidity and mortality. Making the diagnosis of adrenal disorders in pregnancy is challenging as symptoms associated with pregnancy are also seen in adrenal diseases. In addition, pregnancy is marked by several endocrine changes, including activation of the renin-angiotensin-aldosterone system and the hypothalamic-pituitary-adrenal axis. The aim of this article was to review the pathophysiology, clinical manifestation, diagnosis and management of various adrenal disorders during pregnancy.
- Anesthetic management of a patient with Bartter's syndrome undergoing bilateral sagittal split osteotomy. [Journal Article]
- Saudi J Anaesth 2012 Oct-Dec; 6(4):419-22.
Bartter's syndrome is an unusual (estimated incidence is 1.2 per million people) but important congenital form of secondary hyperaldosteronism; due to abnormalities in renal handling of electrolytes. It is associated with hypertrophy and hyperplasia of the juxtaglomerular cells, normal blood pressure, and hypokalemic alkalosis withoutedema.We present a 22-year-old woman with Bartter's syndrome underwent bilateral sagittal split osteotomy to correct mandibular prognathic. The anesthetic management of Bartter's syndrome should be relevant to the pathophysiology of the syndrome. Therefore, it should be directed toward maintaining cardiovascular stability, control of associated fluid, electrolyte and acid-base derangements, and the prevention of renal damage.
- Hypertension Caused by Primary Hyperaldosteronism: Increased Heart Damage and Cardiovascular Risk. [JOURNAL ARTICLE]
- Rev Esp Cardiol 2013 Jan; 66(1):47-52.
OBJECTIVES:Primary hyperaldosteronism is the most common cause of secondary hypertension. Elevated aldosterone levels cause heart damage and increase cardiovascular morbidity and mortality. Early diagnosis could change the course of this entity. The objective of this report was to study the clinical characteristics, cardiac damage and cardiovascular risk associated with primary hyperaldosteronism.
METHODS:We studied 157 patients with this diagnosis. We analyzed the reason for etiological investigation, and the routinely performed tests, including echocardiography. We used a cohort of 720 essential hypertensive patients followed in our unit for comparison.
RESULTS:Compared with essential hypertensive patients, those with hyperaldosteronism were younger (56.9 [11.7] years vs 60 [14.4] years; P<.001), had higher blood pressure prior to the etiological diagnosis (136 [20.6] mmHg vs 156 [23.2] mmHg), more frequently had a family history of early cardiovascular disease (25.5% vs 2.2%; P<.001), and had a higher prevalence of concentric left ventricular hypertrophy (69% vs 25.7%) and higher cardiovascular risk. Specific treatment resulted in optimal control of systolic and diastolic blood pressures (from 150.7 [23.0] mmHg and 86.15 [14.07] mmHg to 12.69 [15.3] mmHg and 76.34 [9.7] mmHg, respectively). We suspected the presence of hyperaldosteronism because of resistant hypertension (33.1%), hypokalemia (38.2%), and hypertensive crises (12.7%). Only 4.6% of these patients had been referred from primary care with a suspected diagnosis of hyperaldosteronism.
CONCLUSIONS:Hyperaldosteronism should be suspected in cases of resistant hypertension, hypokalemia and hypertensive crises. The diagnosis of hyperaldosteronism allows better blood pressure control. The most prevalent target organ damage is left ventricular hypertrophy. Full English text available from:www.revespcardiol.org/en.
- [A case of postoperative convulsive seizure following tranexamic acid infusion during aortic valve replacement]. [English Abstract, Journal Article]
- Masui 2013 Feb; 62(2):186-9.
We present a case of postoperative convulsive seizure in an 84-year-old man who underwent an aortic valve replacement. The patient had hypertension associated with hyperaldosteronism and chronic interstitial nephritis. The duration of cardiopulmonary bypass was 74 min. A generalized seizure lasting approximately 1 minute occurred at 1 hour after the patient's arrival in the intensive care unit. A total of 9 generalized seizures, which were aborted by the intravenous administration of diazepam (5 mg), occurred every 30 min. For seizure control, the continuous administration of midazolam (2 mg x hr(-1)) was initiated. On the day after the discontinuation of the midazolam, a generalized seizure recurred and an infusion of sodium thiopental was started. No further seizures were observed. On the sixth postoperative day, the patient was extubated and discharged without any neurological abnormalities. Imaging showed old small areas of cerebral infarction in the basal ganglia, which were not thought to have contributed to the seizures. The blood sugar, sodium, and calcium levels were within the normal limits. The seizures were likely due to a total dose of 8 g of tranexamic acid (TXA) administered intraoperatively. Possible mechanisms of TXA-induced seizures include blockage of inhibitory cortical y -aminobutyric acid-A receptors.