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Adrenocortical function and dysfunction in the fetus and neonate.

Under normal circumstances, the fetus is exposed to very low concentrations of cortisol until late in gestation. Perturbations of the intra-uterine environment resulting in fetal exposure to increased cortisol may have consequences not only in infancy, but also into adult life. In the postnatal period, developmental immaturity and/or the effects of critical illness on adrenal function may result in insufficient cortisol production to maintain homeostasis in the face of acute stress or illness, a situation that has been labelled 'relative adrenal insufficiency' in other acutely ill populations. The definition of inadequate adrenal function in the newborn and its possible relationship to adverse outcomes in both premature and term infants are only beginning to be characterized.

Adrenal Gland Diseases↗

Imaging evaluation of the non-functioning indeterminate adrenal mass.

With the increasing use of abdominal cross-sectional imaging in the investigation of patient symptoms and in cancer staging, incidental adrenal masses are frequently detected. The most common clinical question is whether these masses are benign or malignant. Benign adrenal masses such as myelolipomas, lipid-rich adenomas, adrenal cysts and adrenal haemorrhage have pathognomonic imaging findings. However, there remains a significant overlap between the imaging appearances of some lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas. Our review looks at the recent advances in computed tomography, magnetic resonance imaging and positron emission tomography, which can be used to assist in the distinction between benign adenomas and malignant lesions of the adrenal gland.

Adenoma↗

Diagnosis of primary aldosteronism: from screening to subtype differentiation.

Numerous studies conducted in recent years have reported an increase in the prevalence of primary aldosteronism (PA). This increase has arisen because of changes in our screening methods used to detect PA, notably the widespread use of the ratio of plasma aldosterone concentration to plasma renin activity. A positive screening result, however, is not diagnostic and requires a confirmatory test. Strategies for screening and confirmation of PA and the techniques to identify the two main subtypes of PA--aldosterone-producing adenoma (APA) and bilateral adrenal hyperplasia (BAH)--are particularly important because hypertension in APA can be cured by adrenalectomy, whereas individuals affected with BAH can receive targeted medical treatment with mineralocorticoid receptor antagonists.

Adenoma↗

Adrenal cortical and medullary imaging.

Adrenal disease can be manifested by endocrine dysfunction or anatomic abnormalities detected by cross-sectional imaging modalities. With the advent of newer and more reliable in vitro assays and a better understanding of the spectrum of adrenal pathology, the physician can now adopt a more accurate and cost-effective approach to the diagnosis of adrenal disease. Both functional and anatomic imaging modalities can play an important role in the evaluation of the incidental adrenal mass, the early detection of adrenal metastases, differentiation of the various causes of Cushings's syndrome, selection of patients for potentially curative surgery in primary aldosteronism and adrenal hyperandrogenism, and localization of pheochromocytomas and neuroblastomas. The usefulness of the adrenal cortical radiopharmaceutical, 131I-6-beta-iodomethylnorcholesterol (NP-59), and the adrenal medullary radiopharmaceuticals, 131I and 123I-metaiodobenzylguanidine (MIBG), is detailed for these various clinical settings and the role of NP-59 and MIBG is contrasted to that of the cross-sectional modalities, computed tomography and magnetic resonance imaging (MRI). Incidental adrenal masses are common, but malignancies are few. Imaging studies select those patients who require a further evaluation by biopsy examination or adrenalectomy. In the hyperfunctioning endocrine states, such as Cushing's syndrome, primary aldosteronism, adrenal androgenism, and pheochromocytoma, correlation of biochemical findings with both functional and anatomic imaging is necessary to avoid inappropriate and ineffective surgical intervention, yet not miss an opportunity for curative resection. Lastly, MIBG and MRI are complementary in the detection and staging of neuroblastoma.

3-Iodobenzylguanidine↗

[Bilateral post-traumatic adrenal hemorrhage. Report of a case with acute adrenal insufficiency].

Bilateral adrenal haemorrhage of traumatic origin is rarely observed or possibly missed in severely multi-traumatised patients. It can lead to a potentially fatal adrenal shock. Its emergency diagnosis is made by imaging techniques, usually by CT-scan. Early substitution therapy has to be done. This complication emphasizes the importance of an immediate abdominal morphological exploration in multi-traumatized patients when this is feasible.

Acute Disease↗

Laparoscopic direct supragastric left adrenalectomy.

BACKGROUND: In this paper a novel laparoscopic approach to the left adrenal gland by the transabdominal anterior route is presented. This approach avoids an extensive viscera dissection to gain access to the left adrenal gland. METHODS: The first step of the procedure is the division of the gastrophrenic ligament and the section of 1 or 2 short gastric vessels in order to mobilize the gastric fundus. The gastric fundus is then pulled down, allowing a wide exposure of the left crus of the diaphragm, the perirenal fat, and the superior edge of the pancreatic body. The diaphragmatic-adrenal channel runs on the left crus, crosses the middle adrenal artery, and, usually, joins the left adrenal vein before its junction with the left renal vein. By pulling on the diaphragmatic vein, exposure of the adrenal vein is facilitated. The adrenal vein is then isolated and divided between clips. Using the monopolar electrocautery to control arteries and small veins, the mobilization of the gland is then completed. The adrenal gland is then placed in a plastic bag to prevent cell spillage and removed through an enlarged umbilical incision. RESULTS: During a 20-month period, 6 consecutive patients with left adrenal gland neoplasms have been operated on with the above mentioned original approach. The diameter of the adrenal mass ranged from 3 cm to 6 cm. No conversion to open surgery or complications have been registered. The mean operative time was 126 minutes. The mean length of hospitalization was 4.1 days (range 3 to 6). CONCLUSIONS: This approach offers a complete visualization of the left adrenal gland, avoiding mobilization of the spleen, pancreatic tail, and left flexure of the colon, and allows an early and easy control of the left adrenal vein so adrenalectomy can be safely performed.

Adrenal Gland Diseases↗

[Outcome of non-operated adrenal masses in 126 patients observed from 1986 to 1999].

STUDY AIM: CT scan performed for non-adrenal related symptoms detects an adrenal mass or 'incidentaloma' in 0.4 to 4.3% of cases, and most authors advocate a non-operative policy, after minimal but careful work-up aimed at excluding pheochromocytoma and aldosteronoma. The breakthrough of laparoscopic adrenalectomy has led some to challenge this attitude. This retrospective study focused on the outcome of non-operated adrenal masses. PATIENTS AND METHOD: From 1986 through 1999, 126 patients (64 men and 62 women) presented with an incidental mass of the adrenal fossa, and a non-surgical attitude was elicited. Mean size was 36.5 mm in diameter. All patients underwent an in-depth clinical, biochemical and imaging work-up. They have been stratified into two groups: group I: no contraindication to surgery (n = 95); and group II: contraindication to surgery (frail patients, invasive adrenal or metastatic extra-adrenal cancer) (n = 31). RESULTS: With a mean follow-up of 4.3 years, 17 patients were lost to follow-up (13.5%), including 11/95 in group I; 36 were dead (28.5%), including 12/95 in group I (no adrenal-related death) and 24/31 in group II; 72 were alive and well without operation, including only one in group II; one patient was operated for a benign adrenal adenoma removed at the time of surgery for aortic aneurysm. CONCLUSION: Careful clinical, biochemical, imaging and nor-iodo-cholesterol scintigraphy with definite uptake by the adrenal mass, a strong indicator of benignancy, allows surgical indication to be postponed, and is likely to cancel it if, at one-year follow-up, imaging studies show no change in the mass.

Adenoma↗

The incidental nonhyperfunctioning adrenal mass: an imaging algorithm for characterization.

Nonhyperfunctioning adrenal lesions such as cysts, myelolipomas, adrenal haemorrhage, adenoma and metastases are described. Definitive imaging features that help characterize adrenal cysts, myelolipomas and adrenal haemorrhage are illustrated and the differentiation of benign from malignant adrenal lesions using an algorithmic approach based on lipid sensitive imaging is provided.

Adrenal Gland Diseases↗

The ratio of androstenedione:11 beta-hydroxyandrostenedione is an important marker of adrenal androgen excess in women.

OBJECTIVE: To determine if the ratio of serum androstenedione (A):11 beta-hydroxyandrostenedione (OHA) would be helpful in differentiating adrenal from ovarian hyperandrogenism. DESIGN/SETTING: Prospective study of outpatients being evaluated for hyperandrogenism. PATIENTS/PARTICIPANTS: Normal women (n = 27), those with hyperandrogenic chronic anovulation (n = 25), and 7 with adult onset of congenital adrenal hyperplasia (CAH) because of 21-hydroxylase deficiency. INTERVENTIONS: Fasting serum between 8:00 A.M. and 9:00 A.M. Patients with hyperandrogenic chronic anovulation and CAH received dexamethasone (DEX) 2 mg for 7 days. MAIN OUTCOME MEASURES: Serum testosterone (T), unbound T, dehydroepiandrosterone sulfate (DHEAS), A, and 11 beta-OHA by radioimmunoassay. RESULTS: Serum 11 beta-OHA and DHEAS were elevated in 52% and 40% of patients with hyperandrogenic chronic anovulation and in 7 of 7 and 1 of 7 patients with CAH. The ratio of A:11 beta-OHA was significantly higher (P less than 0.05) in hyperandrogenic chronic anovulation and significantly lower (P less than 0.05) in CAH compared with controls. Serum A:11 beta-OHA correlated with T (r = 0.58, P less than 0.05). The ratios of A:11 beta-OHA were similar and significantly lower in CAH and hyperandrogenic chronic anovulation patients who were DEX sensitive compared with those who were not DEX sensitive. The ratio correlated with the percentage suppression of T, unbound T, and A after DEX (P less than 0.01). There were no differences with measurements of DHEAS and 11 beta-OHA. Using the mean ratio of controls (1.3) as a cutoff value, the sensitivity of the A:11 beta-OHA in detecting adrenal hyperandrogenism, as assessed by DEX sensitivity, was 100%, the specificity was 84%, and the predictive value was 67%. CONCLUSIONS: The ratio of A:11 beta-OHA appears to be an excellent marker for identifying patients with adrenal hyperandrogenism and CAH.

Adolescent↗

Massive adrenal hemorrhage in neonatal neuroblastoma.

In a neonate with exsanguinating intraperitoneal bleeding admitted with a provisional diagnosis of ruptured liver due to birth trauma, laparotomy revealed the source of hemorrhage to be an adrenal neuroblastoma. This case prompted a review of cases of abdominal neuroblastoma admitted to the Neonatal Surgical Unit in Alder Hey Children's Hospital from 1953 to 1976. The features of 10 cases are presented: in three of them there was hemorrhage into the tumor. Of the 10 cases, six survived tumor free from 2 to 12 yr and there is one short-term survivor. The purpose of this presentation is to emphasize the possibility of an underlying neuroblastoma in cases of neonatal adrenal hemorrhage and also the relatively good prognosis in neuroblastoma presenting in the neonatal period.

Adolescent↗