Characterisation of the onset and severity of adrenal and thyroid dysfunction associated with CTLA4-related hypophysitis.

CONTEXT: The use of the CTLA4 inhibitor, ipilimumab, has proven efficacious in the treatment of melanoma, renal carcinoma and non-small cell lung cancer; however, it is associated with frequent immune-related adverse events (irAE). Ipilimumab-induced hypophysitis (IIH) is a well-recognised and not infrequent endocrine irAE. OBJECTIVE: To investigate the timing of onset and severity of adrenal and thyroid hormone dysfunction around the development of IIH in patients treated for melanoma. DESIGN: Aretrospective review of hormone levels in consecutive adult patients treated with ipilimumab (3 mg/kg) for advanced melanoma as monotherapy or in combination with a PD-1 inhibitor. RESULTS: Of 189 patients, 24 (13%; 13 males; 60.5 ± 12.2 years) presented with IIH at a median of 16.1 (range: 6.7-160) weeks after commencing treatment, occurring in 14 (58%) after the fourth infusion. At the presentation of IIH, corticotroph deficiency was characterised by an acute and severe decrease in cortisol levels to ≤83 nmol/L (≤3 µg/dL) in all patients, often only days after a previously recorded normal cortisol level. Free thyroxine (fT4) levels were observed to decline from 12 weeks prior to the onset of cortisol insufficiency, with the recovery of thyroid hormone levels by 12 weeks after the presentation of IIH. A median fall in fT4 level of 20% was observed at a median of 3 weeks (IQR: 1.5-6 weeks) prior to the diagnosis of IIH. CONCLUSION: IIH is characterised by an acute severe decline in cortisol levels to ≤83 nmol/L at presentation. A fall in fT4 can herald the development of ACTH deficiency and can be a valuable early indicator of IIH.

[Spontaneous adrenal hematomas. Retrospective analysis of 20 cases from a tertiary center]. / Hématomes surrénaliens non traumatiques : série rétrospective de 20 cas.

INTRODUCTION: Spontaneous adrenal hemorrhages (AH) are a rare condition with no consensus about their management. METHODS: Patients were identified using the Medicalization of the Information System Program database, imaging software and a call for observations to internists, intensivists and obsetricians working at our institution. Adult patients whose medical records were complete and whose diagnosis was confirmed by medical imaging were included. RESULTS: From 2000 to 2007, 20 patients were identified, including 15 were women. The clinical onset of AH was non-specific. In five cases, AH occurred during pregnancy; four of them were unilateral and right sided. The etiology of the other fifteen (bilateral adrenal hemorrhage in 11) were as follows: antiphospholipid syndrome (n=8), heparin-induced thrombocytopenia (n=4), essential thrombocythemia (n=3), spontaneous AH due to oral anticoagulants (n=1), complication of a surgical act (n=3), and sepsis (n=3). In seven cases, two causes were concomitant. The diagnosis of AH was often confirmed by abdominal CT. An anticoagulant treatment was initiated in 16 cases. Ten of the eleven patients presenting with bilateral adrenal hematomas were treated using a long-term substitute opotherapy. One patient died because of a catastrophic antiphospholipid syndrome. CONCLUSION: The clinical onset of HS is heterogeneous and non-specific. The confirmatory diagnosis is often based on abdominal CT. The search for an underlying acquired thrombophilia is essential and we found in this study etiological data comparable to the main series in the literature. Adrenal insufficiency is most of the time definitive in cases of bilateral involvement.

Allelic Variants of ARMC5 in Patients With Adrenal Incidentalomas and in Patients With Cushing's Syndrome Associated With Bilateral Adrenal Nodules.

Objective: Germline ARMC5 mutations are considered to be the main genetic cause of primary macronodular adrenal hyperplasia (PMAH). PMAH is associated with high variability of cortisol secretion caused from subclinical hypercortisolism to overt Cushing's syndrome (CS), in general due to bilateral adrenal nodules and rarely could also be due to non-synchronic unilateral adrenal nodules. The frequency of adrenal incidentalomas (AI) associated with PMAH is unknown. This study evaluated germline allelic variants of ARMC5 in patients with bilateral and unilateral AI and in patients with overt CS associated with bilateral adrenal nodules. Methods: We performed a retrospective multicenter study involving 123 patients with AI (64 bilateral; 59 unilateral). We also analyzed 20 patients with ACTH pituitary independent overt CS associated with bilateral adrenal nodules. All patients underwent germline genotyping analysis of ARMC5; abdominal CT and were classified as normal, possible or autonomous cortisol secretion, according to the low doses of dexamethasone suppression test. Results: We identified only one pathogenic allelic variant among the patients with bilateral AI. We did not identify any pathogenic allelic variants of ARMC5 in patients with unilateral AI. Thirteen out of 20 patients (65%) with overt CS and bilateral adrenal nodules were carriers of pathogenic germline ARMC5 allelic variants, all previously described. The germline ARMC5 mutation was observed in only one patient with bilateral AI; it was associated with autonomous cortisol secretion and showed to be a familial form. Conclusion: The rarity of germline ARMC5 mutations in AI points to other molecular mechanisms involved in this common adrenal disorder and should be investigated. In contrast, patients with overt Cushing's syndrome and bilateral adrenal nodules had the presence of ARMC5 mutations that were with high prevalence and similar to the literature. Therefore, we recommend the genetic analysis of ARMC5 for patients with established Cushing's syndrome and bilateral adrenal nodules rather than patients with unilateral AI.

Open adrenalectomy: A 20-year review of our experience in a developing country.

Introduction: The aim of this study was to present our 20-year experience regarding open adrenalectomy (OA) during laparoscopic era in a developing country Turkey. Materials and Methods: A retrospective and descriptive study of patients with adrenal mass undergoing OA in the surgery department of our hospital, between January 1993 and January 2013, was carried out. All operations were performed by two surgeons. Results: Ninety patients who underwent OA in our clinic were reviewed retrospectively. The mean number of adrenal operations per month during this period was 0.38 ± 0.12. The patient included 35 men (38.8%) and 55 women (61.2%), with a mean age of 46.4 ± 17 years. The mean body mass index was 28.4 ± 5.25, and the mean American Society of Anesthesiologists score was 2.6 ± 0.57. The mean operative time was 88 ± 27 min. The mean maximum diameter of all the lesions was 4.8 ± 1.3 cm (range: 1.2-21 cm). The mean blood loss was 118 ± 23 ml during the operations. Postoperative complications were observed in four patients (5.5%). There was no mortality. The length of hospital stay was 6.2 ± 2.1 days. The most frequent type of the histological type was benign adenoma (48.8%). Conclusion: OA in a developing country is a safe method as an alternative for laparoscopic adrenalectomy which has a difficult learning curve.

RésuméIntroduction: Le but de cette étude est de présenter nos 20 ans dæexpérience de læadrénalectomie ouverte (OA) lors de la laparoscopie dans un pays en développement. Matériaux et méthodes: Une étude rétrospective et descriptive a été prévue dans le service de chirurgie générale de notre hôpital, incluant des patients ayant subi entre janvier 1993 et janvier 2013 une adrénalectomie ouverte pour une masse adrrénalienne. Toutes les opérations ont été effectuées par 2 chirurgiens. Résultats: Quatre-vingt-dix patients qui ont subi une adrénalectomie ouverte dans notre clinique ont été évalués rétrospectivement. Le nombre moyen dæopérations adrénaliennes par mois au cours de cette période était de 0,38 ± 0,12. Læâge moyen des patients était de 46,4 ± 17 ans: 35 (38,8%) étaient des hommes et 55 (61,2%) étaient des femmes. Læindice de masse corporelle (IMC) moyen était de 28,4 ± 5,25 et le score moyen de læAmerican Society of Anesthesiologists (ASA) était de 2,6 ± 0,57. La durée moyenne d'opération était de 88 ± 27 minutes. Le diamètre moyen de toutes les lésions était de 4,8 ± 1,3 cm (entre 1,2 et 21 cm). La quantité moyenne de saignements rencontrés au cours des opérations était de 118 ± 23 ml. Des complications postopératoires ont été observées chez quatre patients (5,5%). La mortalité næa été observée chez aucun des patients. La durée moyenne dæhospitalisation était de 6,2 ± 2,1 jours. Le type histologique le plus courant était læadénome bénin (48,8%). Conclusion: Dans un pays en développement, læadrénalectomie ouverte est une alternative sûre à læadrénalectomie laparoscopique qui a une courbe dæapprentissage difficile.

Abnormal linear growth in paediatric adrenal diseases: Pathogenesis, prevalence and management.

Abnormal adrenal function can interfere with linear growth, potentially causing either acceleration or impairment of growth in paediatric patients. These abnormalities can be caused by direct effects of adrenal hormones, particularly glucocorticoids and sex steroids, or be mediated by indirect mechanisms such as the disturbance of the growth hormone-insulin-like growth factor-1 axis and aromatization of androgens to oestrogens. The early diagnosis and optimal treatment of adrenal disorders can prevent or minimize growth disturbance and facilitate improved height gain. Mechanisms of growth disturbance in the following abnormal states will be discussed; hypercortisolaemia, hyperandrogenaemia and obesity. Prevalence and features of growth disturbance will be discussed in ACTH-dependent and ACTH-independent Cushing's syndrome, adrenocortical tumours, premature adrenarche, congenital adrenal hyperplasia and adrenal insufficiency disorders. Recommendations for management have been included.

Clinical Benefits of Unilateral Adrenalectomy in Patients with Subclinical Hypercortisolism Due to Adrenal Incidentaloma: Results from a Single Center.

INTRODUCTION: This study was designed to evaluate the results of unilateral laparoscopic adrenalectomy in patients with subclinical hypercortisolism (SH) due to adrenal incidentaloma (AI) concerning the main cardiometabolic disorders. METHODS: We have studied between January 2000 to December 2015, 645 patients with AI (283 males and 362 females; mean age 61.9 ± 10 years) and we found 70 patients with SH (27 males and 43 females; mean age 61.9 ± 8.4 years). Twenty-six (37%) SH patients (6 males and 20 females; mean age 58.7 ± 7.1 years) underwent unilateral laparoscopic adrenalectomy, whereas 44 SH patients (21 males and 23 females; mean age 63.9 ± 9.9 years) performed a conservative treatment. All SH patients were evaluated at diagnosis and after follow-up (mean 12 months; range 9-15 months). RESULTS: In only SH patients undergoing unilateral adrenalectomy we found a statistical significant reduction of the arterial hypertension and metabolic syndrome (p < 0.05, respectively). In particular we observed a reduction of 24-h systolic blood pressure and "non-dipper" pattern (p < 0.05, respectively) evaluated with ambulatory blood pressure monitoring (ABPM). CONCLUSIONS: Our study confirm the high prevalence of SH in AI, and the unilateral laparoscopic adrenalectomy seemed to have a beneficial effect on some cardiometabolic disorders.

Prevalence of hormonal and endocrine dysfunction in patients with lichen planopilaris (LPP): A retrospective data analysis of 168 patients.

BACKGROUND: Studies on the pathophysiology and comorbidities associated with lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are limited. OBJECTIVE: The purpose of this study was to determine the prevalence of androgen excess in the postmenopausal LPP population, in relation to demographics and comorbidities. METHODS: A retrospective data analysis of 413 patients with LPP, FFA, and LPP/FFA seen in the Department of Dermatology at the Cleveland Clinic Foundation in Ohio between 2005 and 2015 was conducted. Of this cohort, 168 patients met the inclusion criteria. RESULTS: Androgen excess was identified in 31.5% (n = 53) of the 168 patients with LPP and all subtypes (P < .001). Androgen deficiency was identified in 32.1% (n = 17) of the 53 patients with FFA (P < .001). The androgen excess group was significantly more likely to present with hirsutism, seborrheic dermatitis, polycystic ovary syndrome, ovarian cysts, or a combination of these (P < .001). LIMITATIONS: This study was limited by being retrospective. CONCLUSION: Our study demonstrated that LPP is associated with androgen excess, and FFA is associated with androgen deficiency.

Bilateral adrenalectomy: a review of 10 years' experience.

INTRODUCTION The 2012 British Association of Endocrine and Thyroid Surgeons audit report showed that only 86 of 1359 patients who underwent adrenalectomy had a bilateral operation; thus the experience with this procedure remains limited. METHODS Retrospective review of patients undergoing bilateral adrenalectomy in a tertiary referral centre. RESULTS Between November 2005 and January 2016, bilateral adrenalectomy was performed in 23 patients (6 male, 17 female, age 43 ± 4 years) diagnosed with Cushing's disease (n = 13), hereditary phaeochromocytomas (n = 6), adrenocortical cancer (n = 2), colorectal metastatic disease (n = 1) and adrenocortical adenomas (n = 1). A laparoscopic transperitoneal approach was used in 17 patients, with one conversion to open. Three patients had open adrenalectomies for adrenocortical cancer and for simultaneous phaeochromocytomas and pancreatic neuroendocrine tumours in a patient with Von Hippel-Lindau syndrome. Three patients with Cushing's had a bilateral retroperitoneoscopic operation. The mean operating time was 195 ± 16 minutes for laparoscopic operations (n = 16), 243 ± 44 minutes for open adrenalectomies (n = 4) and 151 ± 12 minutes for retroperitoneal operations. It was significantly shorter for Cushing's disease than for phaeochromocytomas (162 ± 8 vs. 257 ± 39 minutes, P < 0.01). Median length of hospital stay was 5 days. Postoperative complications (Clavien-Dindo classification) included one chest infection (level 2), one postoperative haemorrhage and two chest drains for pneumothorax (level 3), two postoperative cardiac arrests (level 4) and one late cancer death from complications related to uncontrolled hypercortisolism (level 5). DISCUSSION Synchronous bilateral adrenalectomy remains an infrequent operation. The laparoscopic approach is feasible in the majority of patients. It is likely that the retroperitoneoscopic adrenalectomy will become the standard approach for bilateral operations.

Adrenal disorders: Is there Any role for vitamin D?

An emerging branch of research is examining the linkage between Vitamin D and nonskeletal disorders, including endocrine diseases. In this regard, a still little studied aspect concerns the involvement of vitamin D in adrenal gland disorders. Adrenal gland disorders, which might be theoretically affected by vitamin D unbalance, include adrenal insufficiency, Cushing's syndrome, adrenocortical tumors and hyperaldosteronism. In this review, we provide an updated document, which tries to collect and discuss the limited evidence to be found in the literature about the relationship between vitamin D and adrenal disorders. We conclude that there is insufficient evidence proving a causal relationship between vitamin D levels and adrenal disorders. Evidence coming from cross-sectional clinical studies can hardly clarify what comes first between vitamin D unbalance and adrenal disease. On the other hand, longitudinal studies monitoring the levels of vitamin D in patients with adrenal disorders or, conversely, the possible development of adrenal pathologies in subjects affected by impaired vitamin D levels would be able to elucidate this still unclear issue.

Experiência de 20 anos em adrenalectomia videolaparoscópica / Twenty-year experience in laparoscopic adrenalectomy

Introdução: As operações na glândula adrenal são realizadas para determinados cânceres, todas as massas biologicamente ativas, metástases, massas com mais de 4-5 cm encontradas incidentalmente e hiperplasia adrenal primária. Métodos: Estudo transversal e descritivo. Foram analisados os prontuários dos pacientes submetidos a adrenalectomia videolaparoscópica entre agosto de 1994 e novembro de 2014. Resultados: Foram realizadas 146 adrenalectomias videolaparoscópicas. Em 134 casos, foi realizada com sucesso, mas em 12 casos (8,2%), o procedimento foi convertido. Foram 97 pacientes do sexo feminino e 49 do sexo masculino, com idade variando de 9 a 81 anos (média de 46,7 anos). Foram removidas 56 adrenais direitas, 75 esquerdas e 15 bilaterais. O tamanho médio das adrenais foi de 5,7 cm, variando de 0,9 a 15 cm. A mediana do tempo de internação hospitalar pós-operatória foi de 4,5 dias. A mediana do tempo de cirurgia foi de 144 minutos. Houve complicações em 22,5% dos casos (maiores ­ casos em que houve conversão para cirurgia aberta, necessidade de reinternação hospitalar e óbito­ e menores), sendo 10,9% complicações intraoperatórias e 11,6% pós-operatórias. Apenas sete (4,7%) pacientes foram considerados complicações maiores. Conclusão: A cirurgia realizada em nosso serviço está de acordo com o descrito na literatura, com taxas aceitáveis de complicações, com motivos de conversão compatíveis e com as indicações totalmente aceitáveis e condizentes. A adrenalectomia videolaparoscópica é a cirurgia de escolha para patologias cirúrgicas da glândula adrenal, exceto em casos de carcinoma adrenal localmente invasivo com comprometimento de outras estruturas (AU)

Introduction: Adrenal gland surgery is performed for some types of cancer, all biologically active masses, metastases, masses larger than 4-5 cm found incidentally, and primary adrenal hyperplasia. Methods: A cross-sectional, descriptive study. Medical records of patients who underwent laparoscopic adrenalectomy from August 1994 to November 2014 were analyzed. Results: A total of 146 laparoscopic adrenalectomies were performed. In 134 cases, laparoscopic adrenalectomy was successfully performed, but in 12 cases (8.2%), the procedure was converted. There were 97 female patients and 49 male patients. Fifty-six right adrenal glands and 75 left adrenal glands were removed, and 15 patients had both of them removed. The average size of adrenal glands was 5.7 cm, ranging from 0.9 to 1.5 cm. The median length of postoperative hospital stay was 4.5 days, ranging from 1 to 55 days. The median surgery time was 144 minutes. There were 22.5% of complications (major ones ­ cases that required conversion to open surgery, hospital readmission, and death ­ and minor ones), of which 10.9% were intraoperative and 11.6% were postoperative. Only seven (4.7%) patients were classified as having major complications. Conclusion: The surgery performed in our department is consistent with the literature, showing acceptable rates of complications, compatible reasons for conversion, and completely acceptable and consistent indications. Laparoscopic adrenalectomy is the surgery of choice for diseases of the adrenal gland, except for locally invasive adrenal carcinoma compromising other structures (AU)

CT screening for lung cancer: Frequency of enlarged adrenal glands identified in baseline and annual repeat rounds.

OBJECTIVES: To determine the frequency of adrenal enlargement of participants in a CT-screening program for lung cancer and demonstrate the progression during follow-up, separately for baseline and annual repeat rounds. MATERIALS AND METHODS: HIPAA-compliant informed consent was obtained in 4,776 participants. The adrenal gland was defined as enlarged if it measured ≥6 mm at its largest diameter. Logistic regression analyses were performed. RESULTS: At baseline, 202 (4 %) of 4,776 participants had adrenal enlargement. Significant factors were age (OR = 1.4, 95 % CI: 1.2-1.7) and current smoker (OR = 1.8, 95 % CI: 1.3-2.4). Follow-up 7-18 months after baseline for 133 cases with adrenal enlargement <40 mm showed it decreased or was stable in 85 (64 %), and increased by <10 mm in 48 (36 %). Five (0.04 %) cases of adrenal enlargement were newly identified, none increased beyond 40 mm on follow-up. Adrenal enlargement was a significant predictor of a subsequent diagnosis of lung cancer (OR = 2.0, 95 % CI: 1.2-3.4). CONCLUSION: Participants with adrenal enlargement <40 mm identified at baseline and on repeat screening could be reasonably assessed on subsequent annual screening. Adrenal enlargement increased with increasing pack-years of smoking. Adrenal enlargement was an independent predictor of a subsequent diagnosis of lung cancer. KEY POINTS: • Adrenal enlargement was seen in 4 % of participants at baseline screening. • Age and currently smoking were significantly associated with adrenal enlargement. • 0.04 % of participants were newly identified with adrenal enlargement. • Annual follow-up for adrenal enlargement <40 mm was appropriate. • Adrenal enlargement was an independent predictor of a diagnosis of lung cancer.

Adrenal haemorrhage in term neonates: a retrospective study from the period 2001-2013.

OBJECTIVE: To assess the incidence, risk factors and clinical presentations of neonatal adrenal haemorrhage (NAH) in uncomplicated, singleton and term deliveries. METHODS: A retrospective analysis of 26,416 term neonates delivered between 2001 and 2013, and screened with abdominal ultrasonography. RESULTS: Of the 26,416 neonates, 74 (0.28%) displayed NAH; the male/female ratio was 1.55:1. Vaginal delivery was significantly more frequent than caesarean section among them (71 versus 3; 95.9% versus 4.1%). Unilateral bleeding occurred on the right side in 36 (48.7%), and on the left in 34 (45.9%), without a significant difference; bilateral haematomas were found in four cases (5.4%). The most common risk factors were macrosomia (16, 21.6%) and fetal acidaemia (23, 31%), while four (5.4%) neonates exhibited pathological acidaemia. Clinical presentations included jaundice in 37 (50%), anaemia in six (8.1%) and an adrenal insufficiency in only one (1.3%) case. In three cases, neuroblastoma was diagnosed. CONCLUSIONS: Vaginal delivery, macrosomia and fetal acidaemia are the most important risk factors for NAH. The adrenal glands on both sides were similarly involved. In the healthy neonates with NAH, the clinical presentations were mild, with spontaneous regression. Differentiation of NAH from tumours is of considerable importance.

[Presentations and management of adrenal hypertension]. / Hypertension artérielle d'origine surrénale : reconnaître, évaluer et prendre en charge.

The frequency of the diagnosed causes of secondary hypertension is only known from hospital-based records, which probably overestimate the true prevalence. Excluding oral contraceptive users and cases with renal failure, their overall frequency was estimated at 1 percent in the eighties, 5 percent in the nineties, and 9 percent in recent years. This increase in frequency was mostly due to an increased number of diagnosed cases of endocrine hypertension. The diagnosis of endocrine hypertension is not synonymous with the diagnosis of a surgically correctable form of hypertension. Indeed, hypertension is surgically curable in a minority of patients, mostly in patients with aldosterone-secreting adenomas or with pheochromocytomas or functional paragangliomas. The presentation, screening, diagnosis and therapeutic management of endocrine hypertension are discussed in the present issue.

Chronic kidney disease and hypothalamic-pituitary axis dysfunction: the chicken or the egg?

Hormonal derangements at the level of the hypothalamic-pituitary axis are often seen with the worsening of kidney function. This may not be surprising given the role of the kidney in synthesis, metabolism and elimination of many of these hormones. Traditionally, these derangements have been understood as a consequence of kidney failure. Conversely, recent evidence points towards the implication of such hormonal disorders in the genesis of CKD. In this review we present arguments supporting both the role of hypothalamic-pituitary axis dysfunction as a consequence of uremic complications and a culprit in disease incidence and progression. Focus is given to evidence regarding thyroidal, adrenal and gonadal axes.

Adrenal haemorrhage due to heparin-induced thrombocytopenia.

Adrenal haemorrhage (AH) is a rare but potentially devastating complication of heparin-induced thrombocytopenia (HIT). Neither the prevalence nor the natural history of AH due to HIT are known. The objectives of this study were to identify the spectrum of AH causes, to characterise the frequency of AH due to HIT and determine the natural history of HIT-associated AH. All patients with incident adrenal haemorrhage from January 2002 through June 2012 seen at the Mayo Clinic were identified. Over this time frame, there were a total of 115 patients with AH of which 11 cases (10%; mean age 67 ± 8 years; 73% female) were associated with HIT. Of these, all but one occurred in the postoperative setting and involved both adrenal glands (89%) with acute adrenal insufficiency at the time of diagnosis. Cases were found incidentally during an evaluation for fever, shock, abdominal pain or mental status changes. All HIT patients experienced venous thrombosis at other locations including deep venous thromboses (n=14), pulmonary emboli (n= 4) and arterial thrombosis (n=2). Four patients undergoing total knee arthroplasty had "spontaneous HIT" with AH in the absence of identifiable heparin exposure. Other causes of AH included trauma (29%), sepsis (15%), antiphospholipid antibody syndrome (10%), and metastatic disease (12%). In conclusion, AH is an important but seldom recognised presumed thrombotic complication of HIT, which usually occurs in the postoperative period, especially after orthopaedic procedures. This syndrome can occur in the apparent absence of heparin exposure, especially following major joint replacement surgery.

Laparoscopic adrenalectomy--assessing the learning curve.

UNLABELLED: Laparoscopic adrenalectomy (LA) has become the "gold standard" for treatment of most of adrenal tumors in last few years. It has many benefits comparing to open surgery, but still is considered as complicated procedure requiring experienced surgical team. THE AIM OF THE STUDY: was to assess the learning curve of laparoscopic adrenalectomy and the outcome of the first consecutive 154 LA. MATERIAL AND METHODS: 154 consecutive patients undergoing LA between 2007 and 2010 were reviewed. Collected data included: patients demographics, clinical and histological diagnosis, side and length of operation, conversions to open surgery, complications and hospitalization time. Learning curve was evaluated by dividing all patients into three groups (group I - first 50 patients, group II - second 50 patients and group III last 54 patients). Differences between the groups were analyzed. RESULTS: There were 154 LAs performed. Indications for LA were hormonally inactive adrenal adenoma(n=57), Conn's syndrome (n=30), Cushing's syndrome (n=28), pheochromocytoma (n=27), adrenal cyst (n=8), and others (n=4). Mean tumor size was 45.28 mm. There were 79 left-sided and 75 right-sided procedures and the average time of hospitalization was 4.64 days. Mean operative time was statistically different between the groups (216.2 min. - 164.6 min. - 131.9 min.; p<0.01) as well as the number of conversions to open surgery (18% - 4% - 3.7%; p=0.013). There was not any significant difference in the number of complications between analyzed groups (2% - 2% -3.7%). CONCLUSION: To improve the outcome of LA it is necessary to perform approximately 40 to 50 procedures.

Screening for adrenal-endocrine hypertension: overview of accuracy and cost-effectiveness.

Formal studies have not been performed to assess the cost-effectiveness of screening strategies for endocrine causes of hypertension. However, an understanding of the diagnostic accuracy of available screening tests and the clinical settings where disease identification will lead to improved health outcomes form the basis for a cost-effective strategy. Primary aldosteronism screening should be selective and restricted to settings where knowledge of the diagnosis has the greatest chance of improving health outcomes. Pheochromocytoma is rare; however, because it is a potentially fatal disease, screening strategies should err on the side of not missing the diagnosis, especially in high-risk clinical settings.

Outcomes after laparoscopic adrenalectomy.

BACKGROUND: Laparoscopic adrenalectomy (LA) has become the standard of care for many conditions requiring removal of the adrenal gland. Previous studies on outcomes after LA have had limitations. This report describes the 30-day morbidity and mortality rates after LA and analyzes factors affecting operative time, hospital length of stay (LOS), and postoperative morbidity. METHODS: Patients undergoing LA in 2007 and 2008 were identified from the American College of Surgeons' National Surgical Quality Improvement Program (NSQIP). Using multivariate analysis of variance (ANOVA) and logistic regression, 52 demographic/comorbidity variables were analyzed to ascertain factors affecting operative time, LOS, and morbidity. RESULTS: The mean age of the 988 patients was 53.5 ± 13.7 years, and 60% of the patients were women. The mean body mass index (BMI) of the patients was 31.8 ± 7.9 kg/m(2). The 30-day morbidity and mortality rates were 6.8% and 0.5%, respectively. The mean and median operative times were 146.7 ± 66.8 min and 134 min, respectively. The mean and median hospital stays were 2.6 ± 3.1 days and 2 days, respectively. Compared with independent status, totally dependent functional status was associated with a 9.5-day increase in LOS (P = 0.0006) and an increased risk for postoperative morbidity (odds ratio [OR], 14.7; 95% confidence interval [CI], 2.4-91.9; P < 0.0001). Peripheral vascular disease (OR, 7.3; 95% CI, 1.7-31.7; P = 0.008) also was associated with increased 30-day morbidity. Neurologic and respiratory comorbidities were associated with increased LOS (P < 0.05). American Society of Anesthesiology (ASA) class 4 patients had a longer operative time than ASA class 1 patients (P = 0.002). CONCLUSIONS: The morbidity and mortality rates after LA are low. Dependent functional status and peripheral vascular disease predispose to postoperative morbidity. Dependent status, higher ASA class, and respiratory and neurologic comorbidities are associated with longer operative time and LOS.

Carney complex and other conditions associated with micronodular adrenal hyperplasias.

Carney complex (CNC) is a multiple neoplasia syndrome that is inherited in an autosomal dominant manner and is characterized by skin tumors and pigmented lesions, myxomas, schwannomas, and various endocrine tumors. Inactivating mutations of the PRKAR1A gene coding for the regulatory type I-α (RIα) subunit of protein kinase A (PKA) are responsible for the disease in most CNC patients. The overall penetrance of CNC among PRKAR1A mutation carriers is near 98%. Most PRKAR1A mutations result in premature stop codon generation and lead to nonsense-mediated mRNA decay. CNC is genetically and clinically heterogeneous, with specific mutations providing some genotype-phenotype correlation. Phosphodiesterase-11A (the PDE11A gene) and -8B (the PDE8B gene) mutations were found in patients with isolated adrenal hyperplasia and Cushing syndrome, as well in patients with PPNAD. Recent evidences demonstrated that dysregulation of cAMP/PKA pathway can modulate other signaling pathways and contributes to adrenocortical tumorigenesis.

Adrenal hematoma after nephrectomy.

OBJECTIVE: The objective of our study was to retrospectively define the incidence of adrenal hematoma on CT or MRI after nephrectomy. MATERIALS AND METHODS: Between January 2008 and June 2009, 465 patients underwent nephrectomy at our institution. Of these, patients without both preoperative and postoperative abdominal CT or MR studies within 6 months of surgery (n = 83) and those with documented adrenalectomy at the time of radical nephrectomy (n = 33) were excluded. Thus, 349 patients (292 male, 57 female; mean age, 60.2 years; range, 2-88 years) were included in the study. Preoperative and postoperative CT or MR studies were reviewed by two radiologists in consensus for the presence or absence of adrenal hematoma. Diagnosis of adrenal hematoma was made by imaging findings and follow-up. The incidence of adrenal hematoma was calculated with a 95% CI. RESULTS: Of the 349 patients (partial nephrectomy, n = 232; radical nephrectomy, n = 117), 19 patients developed adrenal hematoma after nephrectomy (partial nephrectomy, n = 12; radical nephrectomy, n = 7) with an incidence of 5.4% (95% CI, 3.1-7.8%). All adrenal hematomas were located on the side of nephrectomy (right adrenal gland, n = 8; left adrenal gland, n = 11). In nine patients, the adrenal hematoma appeared as a round or oval lesion on postoperative imaging with a mean size of 2.6 cm (range, 1.5-4.0 cm). In 10 patients, the adrenal hematoma appeared as diffuse enlargement of the adrenal gland on postoperative imaging with a mean thickness of 1.3 cm (range, 0.6-1.8 cm). CONCLUSION: An adrenal hematoma was identified in 5.4% of patients on CT or MRI after nephrectomy. A new round or oval adrenal mass after nephrectomy should be differentiated from a metastasis.