Primary aldosteronism without hypertension:A case report and review of the literature / Hyperaldostéronisme primaire sans hypertension artérielle : à propos d'un cas et revue de la littérature

We describe the case of a patient who presented with hyperaldosteronism without arterial hypertension. She had been referred for consultation for persistent severe hypokalaemia despite oral KCl supplementation. The absence of hypertension had been proven by repeated clinical measurements and by ABPM. Hyperaldosteronism had been demonstrated by hormonal assays and catheterization of the adrenal veins. Abdominal CT revealed a left adrenal adenoma. Finally, the anatomopathological examination of the surgical specimen confirmed the adenoma. After the intervention, serum potassium normalized. The clinical case is completed by a review of the literature of hyperaldosteronisms without arterial hypertension.

Nous décrivons le cas d'une patiente qui s'est présentée avec un hyperaldostéronisme sans hypertension artérielle. Elle a été adressée en consultation pour une hypokaliémie sévère persistante malgré une supplémentation orale en chlorure de potassium (KCl). L'absence d'hypertension a été prouvée par des mesures cliniques répétées et par mesure ambulatoire de la pression artérielle (MAPA). L'hyperaldostéronisme a été mis en évidence par des dosages hormonaux et un cathétérisme des veines surrénales. Le scanner abdominal a révélé un adénome surrénalien gauche. Enfin, l'examen anatomopathologique de la pièce opératoire a confirmé l'adénome. Après l'intervention, le potassium sérique s'est normalisé. Le cas clinique est complété par une revue de la littérature des hyperaldostéronismes sans hypertension artérielle.

Surgical findings and outcomes after unilateral adrenalectomy for primary hyperaldosteronism in cats: a multi-institutional retrospective study.

CASE SERIES SUMMARY: Twenty-nine cats from different institutions with confirmed or highly suspected primary hyperaldosteronism treated by unilateral adrenalectomy were retrospectively included in this study. The most frequent clinical signs were lethargy (n = 20; 69%) and neck ventroflexion (n = 17; 59%). Hypokalaemia was present in all cats, creatinine kinase was elevated in 15 and hyperaldosteronism was documented in 24. Hypertension was frequently encountered (n = 24; 89%). Preoperative treatment included potassium supplementation (n = 19; 66%), spironolactone (n = 16; 55%) and amlodipine (n = 11; 38%). There were 13 adrenal masses on the right side, 15 on the left and, in one cat, no side was reported. The median adrenal mass size was 2 × 1.5 cm (range 1-4.6 × 0.4-3.8); vascular invasion was present in five cats, involving the caudal vena cava in four cats and the renal vein in one. Median duration of surgery was 57 mins. One major intraoperative complication (3%) was reported and consisted of haemorrhage during the removal of a neoplastic thrombus from the caudal vena cava. In 4/29 cats (14%), minor postoperative complications occurred and were treated medically. One fatal complication (3%) was observed, likely due to disseminated intravascular coagulation. The median duration of hospitalisation was 4 days; 97% of cats survived to discharge. The potassium level normalised in 24 cats within 3 months of surgery; hypertension resolved in 21/23 cats. Follow-up was available for 25 cats with a median survival of 1082 days. Death in the long-term follow-up was mainly related to worsening of comorbidities. RELEVANCE AND NOVEL INFORMATION: Adrenalectomy appears to be a safe and effective treatment with a high rate of survival and a low rate of major complications. Long-term medical treatment was not required.

Repeated vertebral compression fractures in young adult may imply functional adrenal tumor.

BACKGROUND: Patients with adrenal Cushing's syndrome (ACS) typically present with central obesity, hirsutism, hypertension, or glucose intolerance, which can be easily identified by a clinical physician. However, recognizing those with subclinical CS or those with less common symptoms and signs is challenging to the subspecialist, which can lead to delayed diagnosis and treatment. We report a case who presented with repeated vertebral fractures in 6 months. Typical physical appearance of CS was not shown so that suspicions were not raised until severe osteoporosis was demonstrated from bone marrow density study. From our case report, endocrine tests and image survey should always be considered in young patients with repeat vertebral fractures. CASE PRESENTATION: A 48-year-old man presented with severe back pain for 3 months. Second and fifth lumbar spine (L2 and L5) vertebral compression fractures were noted from X-ray and magnetic resonance imaging (MRI), and vertebroplasty was performed by orthopedic surgeons. After 1 month, a newly developed compression fracture of the ninth to twelfth thoracic spine and L4-L5 were noted. Severe osteoporosis was noted from the hip bone mineral density test, and he was referred to an endocrinologist for analysis. Serial endocrine tests confirmed hypercortisolism, and subsequent abdomen MRI showed a left adrenal tumor. ACS was diagnosed. Left laparoscopic adrenalectomy was performed, and the patient received cortisol supplement for 12 months. Thereafter, no new fractures were identified. CONCLUSIONS: ACS should be considered and carefully verified in middle-aged adults who present with severe osteoporosis and repeated vertebral compression fracture.

Management recommendations for pancreatic manifestations of von Hippel-Lindau disease.

Von Hippel-Lindau disease (VHL) is a multineoplasm inherited disease manifesting with hemangioblastoma of the central nervous system and retina, adrenal pheochromocytoma, renal cell carcinoma, pancreatic neuroendocrine tumors and cysts, and neoplasms/cysts of the ear, broad ligament, and testicles. During 2018-2020, the VHL Alliance gathered several committees of experts in the various clinical manifestations of VHL to review the literature, gather the available evidence on VHL, and develop recommendations for patient management. The current report details the results of the discussion of a group of experts in the pancreatic manifestations of VHL along with their proposed recommendations for the clinical surveillance and management of patients with VHL. The recommendations subcommittee performed a comprehensive systematic review of the literature and conducted panel discussions to reach the current recommendations. The level of evidence was defined according to the Shekelle variation of the Grading of Recommendations, Assessment, Development, and Evaluation grading system. The National Comprehensive Cancer Network Categories of Evidence and Consensus defined the committee members' interpretation of the evidence and degree of consensus. The recommendations encompass the main aspects of VHL-related pancreatic manifestations and their clinical management. They are presented in a clinical orientation, including general planning of screening and surveillance for pancreatic neuroendocrine tumors, utility of biochemical biomarkers, the optimal choice for imaging modality, indirect risk stratification, indications for tissue sampling of VHL-related pancreatic neuroendocrine tumors, and interventions. These recommendations are designed to serve as the reference for all aspects of the screening, surveillance, and management of VHL-related pancreatic manifestations.

Hypokalemia-Induced Rhabdomyolysis Caused by Adrenal Tumor-Related Primary Aldosteronism: A Report of 2 Cases.

BACKGROUND Primary aldosteronism, also known as Conn's syndrome, is a clinical condition caused by excessive production of aldosterone. The classic presenting signs of primary aldosteronism are hypertension and hypokalemia. However, rhabdomyolysis induced by severe hypokalemia is a rare manifestation of primary aldosteronism. There were only a few cases presented in the English literature over the last 4 decades. CASE REPORT We present 2 cases, a 53-year-old man and a 46-year-old man, with severe hypokalemia-induced rhabdomyolysis caused by adrenal tumor-related primary aldosteronism. Both of these patients were under medical treatment with oral anti-hypertension drug for hypertension, but were poorly controlled. They both presented to the Emergency Department with muscle weakness and pain. Laboratory testing showed elevated creatinine phosphokinase (CPK) and low serum potassium levels. Hypokalemia-induced rhabdomyolysis was suspected. A further endocrine survey showed low PRA (plasma renin activity) and high aldosterone levels, finding which are compatible with primary aldosteronism. Computed tomography (CT) was arranged for further evaluation, and adrenal tumors were found in both cases. Both patients underwent robotic-assisted laparoscopic adrenalectomy. In both cases, there was no recurrence of hypokalemia without potassium supplementation, and their hypertension was under better control at further follow-up visits. CONCLUSIONS Hypokalemic rhabdomyolysis is a rare manifestation of primary aldosteronism. It might be difficult to making a diagnosis when rhabdomyolysis and severe hypokalemia are the first manifestations in patients with primary aldosteronism. The use of diuretics for hypertension treatment might be a risk factor for extremely low potassium levels, which can induce rhabdomyolysis in patients with primary aldosteronism.

Seizure and coma secondary to Conn's syndrome: a case report.

BACKGROUND: Conn's syndrome is a curable condition if identified properly. It is characterized by autonomous secretion of aldosterone from the adrenal gland cortex. Its morbidity is related to the increased risk of cardiovascular diseases. CASE PRESENTATION: We report the case of a 48-year-old man of African descent presenting with generalized tonic-clonic seizure and coma secondary to hypertensive encephalopathy. A biochemical evaluation revealed a very high aldosterone level and an undetectable renin level, both are compatible with primary aldosteronism. The presentation of the following confirms the diagnosis of primary aldosteronism: spontaneous hypokalemia, an undetectable renin level, and a high aldosterone level. Abdominal computed tomography revealed a left adrenal adenoma. Adrenal venous sampling confirmed lateralization of aldosterone excretion from the left adrenal gland. Our patient underwent left laparoscopic adrenalectomy that confirmed a left functional adrenal adenoma. After 12 months of follow up, his hypertension was controlled on only one antihypertensive drug which was down from four drugs preoperatively. CONCLUSION: Conn's syndrome, in this case, was complicated by coma secondary to seizure. Adrenalectomy normalized the hypokalemia and improved resistant hypertension. Potassium supplementation and several antihypertensives were discontinued as our patient became normokalemic and normotensive on one antihypertensive agent.

Glucocorticoid-deficient hypoadrenocorticism secondary to intravascular lymphoma in the adrenal glands of a dog.

CASE REPORT: A 2-year-old neutered male German Shepherd dog was presented with weakness, poor appetite and weight loss. Glucocorticoid-deficient hypoadrenocorticism was diagnosed with undetectable pre- and post-ACTH cortisol concentrations but normal sodium and potassium concentrations. Despite appropriate supplementation with glucocorticoids, the patient's weakness progressed and neurological deficits developed. The patient was euthanased. Histopathological analysis of multiple organs, including the adrenal glands, showed an accumulation of neoplastic lymphocytes within blood vessels, consistent with a diagnosis of intravascular lymphoma. Histologically, in both adrenal glands, the architecture of the zona fasciculata and reticularis was disrupted by blood vessels congested with a neoplastic population of T-lymphocytes; the zona glomerulosa remained intact. CONCLUSION: This is the first report of intravascular lymphoma causing glucocorticoid-deficient hypoadrenocorticism in a dog.

Refractory diarrhea: A paraneoplastic syndrome of neuroblastoma.

Neuroblastoma (NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB in our hospital treated from 1996 to 2006 were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide (VIP) immunohistochemical staining results, treatment, and prognosis. All patients were boys with 3-8 loose or watery stools each day and routine fecal tests were normal. Abdominal tumors were identified by B-ultrasound. Drugs were ineffective. Three patients underwent surgery, and the remaining three patients received surgery and chemotherapy. Diarrhea stopped after treatment in five patients. Two patients died due to intractable hypokalemia. The tumor was located in the adrenal gland in four patients, in the upper retroperitoneum in one patient, and in the presacral area in one patient. Pathologic findings were NB and ganglioneuroblastoma. Five patients were at clinical stage I-II, and one was at stage III. Four patients survived (followed-up for 6 mo to 4 years). Immunohistochemical staining for VIP was positive. Refractory diarrhea is a paraneoplastic syndrome of NB and is rare. Patients aged 1-3 years who present with chronic intractable diarrhea should be followed closely. Intractable diarrhea, hypokalemia, and dysplasia are the initial clinical manifestations. Increased VIP is characteristic of this disease. Potassium supplementation plays a vital role in the treatment procedure, especially preoperatively. The prognosis of diarrheal NB is good following appropriate treatment.

A rare case of juvenile hypertension: coexistence of type 2 multiple endocrine neoplasia -related bilateral pheochromocytoma and reninoma in a young patient with ACE gene polymorphism.

BACKGROUND: Pheochromocytoma and reninoma represent two rare diseases causing hypertension. We here reported a rare case of association between type 2 multiple endocrine neoplasia related bilateral pheochromocytoma and reninoma. Moreover, polymorphism of ACE gene, which is known to be related to an increase of cardiovascular risk, has been found in the same patient. CASE PRESENTATION: A 24 year old Caucasian man came to our attention for severe hypertension, resistant to anti-hypertensive polytherapy. At the age of twenty he had undergone total thyroidectomy with lymphadenectomy for medullary carcinoma. Genetic testing showed a RET mutation of codon 918 (exon 16) not documented in other family members. During the follow-up, a progressive increase of urinary metanephrines and catecholamines was recorded. Our evaluation confirmed the presence of severe hypertension (220/140 mmHg) and a severe increase of urinary catecholamines and metanephrines. Due to the presence of hypokalemia, other causes of hypertension were researched leading to the discovery of hyperreninemia (236 µUI/ml) with mild hyperaldosteronism, and a mild increase of the renal artery resistance at ultrasound. An abdominal MRI showed multiple adrenal masses and a right kidney nodular lesion of about 2 cm. The patient underwent bilateral adrenalectomy and right nephrectomy, and histology confirmed the presence of bilateral pheochromocytoma and right reninoma. The post-surgery laboratory evaluation showed a rapid reduction of the urinary metanephrines while plasma renin level remained low in spite of the bilateral adrenalectomy without any mineralocorticoid supplementation. To further investigate these unusual feature, we performed genetic testing for the ACE gene, which revealed the presence of ACE I/D polymorphism. CONCLUSION: This unique report describes the association between two rare causes of hypertension in the same patient. Furthermore, the absence of requirement of mineralocorticoid supplementation in spite of bilateral adrenalectomy, represent an uncommon and interest finding.

Persistent hypertension after adrenalectomy for an aldosterone-producing adenoma: weight as a critical prognostic factor for aldosterone's lasting effect on the cardiac and vascular systems.

BACKGROUND: Primary aldosteronism caused by an aldosterone producing adrenal tumor/aldosteronoma (APA), is a potentially curable form of hypertension, via unilateral adrenalectomy. Resolution of hypertension (HTN) is not as prevalent after tumor resection, as are the normalization of aldosterone secretion, hypokalemia, and other metabolic abnormalities. Here, we review the immediate and long-term medical outcomes of laparoscopic adrenalectomy in patients with an APA, and attempt to identify any distinctive sex differences in the management of resistant HTN. METHODS: We performed a retrospective review of the prospective adrenal database at the University of Wisconsin between January 2001 and October 2010. Of the 165 adrenalectomies performed, 32 were for the resection of an APA. Patients were grouped according to their postoperative HTN status. Those patients with normal blood pressure (≤120/80 mm Hg) and on no antihypertensive medication (CURE) were compared with those who continued to require medication for blood pressure control (HTN). We evaluated sex, age, body mass index, tumor size, duration of time with high blood pressure, and the differences in systolic and diastolic blood pressure following adrenalectomy. Statistical analysis was performed using Student's t-test. Statistical significance was defined as a P value of <0.05. RESULTS: We identified 32 patients with an APA based on biochemical and radiographic studies, two patients were excluded, due to missing data. There were 19 males (63%) and 11 (37%) females, with a mean age was 48.3 ± 2.1 y, and mean tumor size was 24 ± 3 mm. Postoperatively, patients required significantly fewer antihypertensive medications (1.5 ± 0.2 versus 3.3 ± 0.3, P < 0.001). Nine patients (31%) had complete resolution of their HTN, requiring no postoperative antihypertensive medication. The only significant difference between the sexes, was a lower body mass index in women (27.6 ± 1.7 versus 33.4 ± 2.1 kg/m(2), P = 0.04). Ninety percent of the cohort had at least a 20 mm Hg decline in their systolic blood pressure postoperatively, placing them in the prehypertensive or normal blood pressure categories. Sixty-six percent of the CURE patients required at least 6 mo for resolution of their HTN. All 20 patients who presented with hypokalemia, had immediate resolution postoperatively and did not require continuance of the preoperative spironolactone or potassium supplementation. CONCLUSIONS: Laparoscopic adrenalectomy for aldosterone producing adenoma results in the normalization of, or more readily manageable blood pressure in 90% of patients, within 6 mo. Metabolic disturbances are immediately corrected with tumor resection. Weight is an important contributing factor in resolving HTN.

Long-term results of a prospective, randomized trial comparing retroperitoneoscopic partial versus total adrenalectomy for aldosterone producing adenoma.

PURPOSE: The indication for laparoscopic total or partial adrenalectomy in patients with aldosterone producing adrenal adenoma remains controversial. We compared retroperitoneoscopic partial and total adrenalectomy for aldosterone producing adrenal adenoma in a prospective, randomized, multicenter trial. MATERIALS AND METHODS: Patients with aldosterone producing adrenal adenoma were randomized to retroperitoneoscopic partial or total adrenalectomy. Patient characteristics, surgical data, complications and postoperative clinical results were analyzed statistically. RESULTS: From July 2000 to March 2004, 212 patients were enrolled in this study, including 108 and 104 who underwent total and partial adrenalectomy, respectively. The 2 groups were comparable in patient age, gender, body mass index and tumor site. Mean followup was 96 months in each group. No conversion to open surgery was needed and no major complications developed. Partial adrenalectomy required a shorter operative time than total adrenalectomy but this did not attain statistical significance. Intraoperative blood loss in the partial adrenalectomy group was significant higher than in the total adrenalectomy group (p <0.05) but no patient needed blood transfusion. All patients in each group showed improvement in hypertension, and in all plasma renin activity and aldosterone returned to normal after surgery. No patient required potassium supplements postoperatively. In the total and partial adrenalectomy groups 32 (29.6%) and 29 patients (27.9%), respectively, were prescribed a decreased dose of or fewer antihypertensive medicines at final followup. CONCLUSIONS: Retroperitoneoscopic partial adrenalectomy is technically safe. It has therapeutic results similar to those of total adrenalectomy in patients with primary aldosteronism due to aldosteronoma.

Atrioventricular dissociation due to pheochromocytoma in a young adult.

Atrioventricular dissociation can be a manifestation of an underlying noncardiac disease.We present a patient who underwent pacemaker implantation because of intermittent atrioventricular dissociation and medically untreatable supraventricular arrhythmias, which could not be induced by electrophysiological testing. The arrhythmias proved to be due to a pheochromocytoma. After left adrenalectomy, both the supraventricular arrhythmias and the atrioventricular dissociation disappeared. Adequate recognition and treatment of pheochromocytoma can reverse atrioventricular dissociation and may avoid unnecessary procedures such as electrophysiological testing and pacemaker implantation.

Treatment of resistant hypertension.

AIM: Resistant hypertension (RH) is a common clinical problem. Patients with RH have increased cardiovascular risk. These patients also have high risk for having reversible causes of hypertension and may potentially benefit from special diagnostic or therapeutic considerations. The purpose of this review was to discuss RH, its definition, recognition, evaluation and treatment. METHODS: Authors define RH and the implications of this definition. They present latest data on its prevalence, prognostic implications, genetics, and patient characteristics. Elements of pseudoresistance and possible etiologies of treatment resistance are also identified. Lastly, diagnostic and therapeutic approaches to RH are discussed, focusing on antihypertensive medication classes that have proven benefit in patients with RH, and also on novel therapeutic approaches in these patients. CONCLUSION: RH is a common clinical problem and carries an increased risk for cardiovascular morbidity and mortality, as well as target organ damage. Patients with RH are aat high risk for reversible causes of hypertension and may benefit from special diagnostic or therapeutic considerations. Elements of pseudoresistance, intake of interfering substances and secondary causes of hypertension should be searched for and corrected, if possible. Therapeutic lifestyle modifications should be emphasized. Medical therapy includes optimizing diuretic use and considering the use of mineralocorticoid antagonists as add on antihypertensive agents. Novel approaches include surgical and transcatheter techniques, chronotherapy, and new classes of antihypertensive agents.

Weekly clodronate treatment prevents bone loss and vertebral fractures in women with subclinical Cushing's syndrome.

INTRODUCTION: Chronic mild endogenous glucocorticoid excess has been shown to cause bone loss and to increase fracture risk in both post-menopausal and premenopausal women. Currently, it is unclear if patients with subclinical Cushing's syndrome (SCS) with osteoporosis or osteopenia may benefit from antiresorptive treatment and the type of therapy to be given. OBJECTIVE: This pilot randomized study was aimed at evaluating the effects of 12-month im administration of clodronate (100 mg every week) on vertebral and femoral bone mineral density (BMD), bone turnover markers and on subjective pain in premenopausal women with SCS due to adrenal incidentalomas. METHODS: Forty-six women (age, 43.1+/-7.7 yr) with SCS due to adrenal incidentaloma and osteoporosis/osteopenia were randomized to receive clodronate plus supplement of Calcium (500 mg daily) and Vitamin D3 (800 mg daily) (group 1, no.=23) or supplements only (group 2, no.=23). Both groups were similar in terms of age, body mass index, cortisol levels, BMD values, and bone turnover markers. All of the women were re-evaluated after 12 months. RESULTS: After 12 months of treatment, in group 1, a significant increase in lumbar BMD occurred (p=0.04), while bone turnover markers decreased by about one third (p<0.05). In group 2, bone turnover markers did not change and BMD values slightly decreased (p=ns). The differences in bone turnover markers and in lumbar BMD between the two groups were significant (p<0.05, all). No new vertebral fracture occurred in group 1, while in group 2 the spine radiographies revealed 2 new fractures and a worsening of two pre-existent fractures. An improvement in subjective back pain, assessed by visual analogue scale pain score was observed in group 1 (from 4.3+/-2.7 to 2.9+/-2.0; p<0.05) but not in group 2 (from 4.4+/-3.1 to 4.2+/-3.4; p=ns). No significant changes occurred in cortisol secretion or clinical picture of the SCS during the study. CONCLUSIONS: Intramuscular administration of clodronate effectively increased lumbar BMD values, preserved bone mass at the femoral neck, stabilized vertebral fracture index, and decreased subjective back pain in pre-menopausal women with SCS. Since the untreated group continued to lose bone, antiresorptive treatment should be considered in patients with SCS, according to the prevision of surgical treatment, prevalent fractures, BMD values, age, concomitant morbidities, and desire for pregnancy.

Perioperative management of phaeochromocytoma.

Phaeochromocytoma is a rare catecholamine producing tumour, feared for its life threatening cardiovascular disturbances during anaesthesia. Improved medical and anesthetic management resulted in reduction of perioperative phaeochromocytoma resection mortality from about 50% in the pioneer period to near 0% nowadays. Cardiomyopathy is usually reversible if managed properly. Stress related or (inverted) Tako Tsubo cardiomyopathy is a recent finding, deserving our attention. Preoperative alpha blockade should be performed to achieve cardiovascular stability and decrease uncontrolled intraoperative surges in blood pressure. During anaesthesia, additional antihypertensive (also mainly alpha blocking) agents are essential to prevent and overcome hypertensive crises. Magnesium sulphate is a safe and promising agent in improving cardiovascular stability and should have a place in standard therapy. A careful selection of anaesthetic drugs and techniques that cause the least hypertension is most important. Preoperative and intraoperative beta-blockade can only be used as adjuvant therapy, mainly to control tachycardia and other rhythm disturbances. Postoperatively, the patient is transferred to the intensive care unit where adequate management of haemodynamic and metabolic complications takes place.

An additional child case of an aldosterone-producing adenoma with an atypical presentation of peripheral paralysis due to hypokalemia.

Aldosterone-producing adenoma, which is characterized by hypertension, hypokalemia, and elevated aldosterone levels with suppressed plasma renin activity, is a rare condition during childhood and is also potentially curable. To the best of our knowledge, nearly 25 cases of childhood aldosterone-secreting adenoma have been reported in the literature to date. Here we describe a 13-yr-old girl with primary hyperaldosteronism secondary to aldosterone-secreting adenoma. The patient was admitted to our hospital with the neuromuscular complaints of muscle weakness and inability to walk due to hypokalemia. She had been misdiagnosed as having hypokalemic periodic paralysis 2 months before admission and her symptoms had radically improved with potassium supplementation. However, her blood pressure levels had increased and her symptoms reappeared 2 days prior to being observed during hospitalization in our institution. Laboratory examinations revealed hypokalemia (2.1 mEq/l), and increased serum aldosterone levels with suppressed plasma renin activity. Abdominal ultrasonography and abdominal magnetic resonance imaging revealed left adrenal mass. Laparoscopic adrenalectomy was performed and histopathological examinations showed benign adrenal adenoma. Serum aldosterone levels and blood pressure levels returned to normal after surgical intervention. This case demonstrates the importance of a systemic evaluation including blood pressure monitorization of children with hypokalemia as intermittent hypertension episodes may be seen; cases without hypertension may be misdiagnosed as rheumatological or neurological disorders such as hypokalemic periodic paralysis, as in our case.

Assessment and therapy of selected endocrine disorders.

Diabetes remains the most commonly encountered endocrinopathy with the incidence of type 2 doubling in the past decade. The prevalence of diabetes is projected to continue to increase dramatically over the next several decades unless major public health initiatives are successful in stemming this growth. Both type I and 2 diabetics more frequently require surgical and critical care than their non-diabetic counterparts. Type 1 and 2 diabetics also sustain greater peri-operative morbidity and mortality. Careful preoperative assessment and appropriate perioperative intervention may limit this. There is increasing evidence that maintenance of normal blood glucose in the perioperative period and during critical illness is beneficial for diabetic and non-diabetic patients. More data will hopefully be forthcoming to substantiate recent reports and identify the mechanisms of improved outcome. Thyroid disease remains a commonly encountered pathology that is more readily identified and controlled in the modern era of radioimmune assays of thyroid hormone and successful medical and surgical therapies. Severe hypothyroidism and thyroid storm are associated with significant increases in perioperative morbidity and mortality. Recognition of these entities or those at risk for developing them post operatively is crucial in initiating timely and effective therapy. Primary Al is uncommon, but results in glucocorticoid and mineralocorticoid deficiency. Tertiary Al is far more common, most often secondary to iatrogenic therapy with exogenous glucocorticoids for the management of chronic diseases such as connective tissue disorders, anti-rejection regimes, and severe asthma. Glucocorticoid replacement or supplementation is needed on a case-by-case basis and should be individualized based on chronic steroid dose, duration, and stress of the surgical procedure. Perioperative steroid dosing regimes now recommend lower doses for shorter periods than previously suggested. More recently Al has been recognized in two populations, elderly patients undergoing major surgery and a subgroup of patients with septic shock. Timely diagnosis using synthetic ACTH stimulation testing and stress glucocorticoid, and possibly mineralocorticoid therapy, seems to reverse these processes and improve recovery. Although uncommon, patients with pheochromocytoma who undergo open or laparoscopic resections remain diagnostic and therapeutic challenges. Perioperative outcome seems to have improved, in part, related to newer therapies and less invasive surgeries when indicated. The appropriate preoperative assessment and management of patients with various endocrinopathies is important to optimize outcome and limit avoidable complications. Hopefully additional evidence based guidelines will be forth-coming particularly in caring for the ever increasingly encountered perioperative diabetic.

Prevalence of subclinical Cushing's syndrome in 70 patients with adrenal incidentaloma: clinical, biochemical and surgical outcomes.

Subclinical Cushing's syndrome (SCS) is being detected with increased frequency in patients with adrenal incidentaloma. In the current study, we evaluated the prevalence of SCS in 70 patients with adrenal incidentaloma and compared the main findings on them with other patients with nonfunctional adrenal incidentaloma (NFA). Overnight 3 mg dexamethasone (DXM) suppression test to exclude cortisol hypersecretion, and high dose DXM suppression test to find out patients with SCS, were applied to all subjects. Afterwards, biochemical and clinical findings of patients with SCS were compared with the other patients with NFA. Four of the 70 patients with adrenal incidentaloma were found to have SCS, with a prevalence of 5.7%. Basal ACTH and DHEA-S levels were significantly lower (p < 0.05 and p < 0.01, respectively), and midnight cortisol and 24-hour urinary free cortisol levels were significantly higher in patients with SCS (p < 0.001 and p < 0.05, respectively). Biochemical and metabolic bone parameters were similar in patients with SCS and in patients with NFA. Hypertension, diabetes mellitus, and obesity were more common in patients with SCS. One of the patients with SCS developed adrenocortical insufficiency following unilateral adrenalectomy which lasted for about 6 months. Suppressed ACTH and DHEA-S levels, and high midnight cortisol levels may be some clues for SCS in patients with adrenal incidentaloma. Since patients with SCS frequently have risk factors for atherosclerosis such as hypertension, diabetes, and obesity, and the surgical management of SCS with adrenalectomy may offer an advantage. Patients undergoing adrenalectomy should be followed for the development of adrenal insufficiency.