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Review: recent developments in adrenal surgery.

The improvements of biochemical methods and imaging techniques in the diagnosis of adrenal disorders have changed the surgical management with regard to indication for operation, surgical approach to the adrenals and the extent of resection. This study reviews recent developments in surgical strategy of adrenal disorders based on surgical treatment of 246 patients from 1975 to 1993 at the Medical School of Hannover. Today extraperitoneal incisions are used with increasing frequency in small benign tumours to minimize the surgical trauma. The indication for operation of adrenal incidentalomas should be considered in all patients with hyperfunctional lesions and suspected malignancy. Bilateral total adrenalectomy is attended with the need for life-long adrenocortical supplementation resulting in an impaired quality of life. In this respect MEN 2 patients with bilateral adrenomedullary disease benefit from unilateral or bilateral subtotal resections with preservation of adrenocortical function. In conclusion, adrenal surgery today aims at the reduction of surgical trauma, early diagnosis and treatment of adrenal malignancies, and the preservation of adrenocortical function.

Adrenal Gland Diseases↗

Evaluation of adrenal masses in patients with bronchogenic carcinoma using 18F-fluorodeoxyglucose positron emission tomography.

OBJECTIVE: The purpose of this study was to assess the usefulness of positron emission tomography (PET) with 18F-fluorodeoxyglucose (FDG) when differentiating benign from metastatic adrenal masses in patients with bronchogenic carcinoma. SUBJECTS AND METHODS: For our prospective study, any patient presenting to our institution with pathologically proven bronchogenic carcinoma and an adrenal mass was eligible. Thirty-three adrenal masses (mean size, 3 cm; range, 1-9 cm) in 27 patients were revealed by CT. PET was performed in all 27 patients and interpreted as positive when FDG uptake in the adrenal mass was greater than background activity or negative when FDG uptake in the adrenal mass was equal to or less than background activity. In addition, semiquantitative analysis was performed by computing a standardized uptake ratio. All studies were reviewed independently by three radiologists and then correlated with biopsy and CT findings. Specificity and sensitivity for determining metastatic disease to the adrenal gland were calculated. RESULTS: FDG uptake was positive (abnormally increased) in 25 adrenal masses. Twenty-three (92%) of the 25 masses were metastatic disease. The mean standardized uptake ratio of these was 6.28 (range, 3.22-14.41). The remaining two masses (8%) that had positive FDG uptake showed no tumor at percutaneous biopsy. The standardized uptake ratio values for these two masses were 3.0 and 3.7. FDG uptake was negative (normal) in eight adrenal masses. All these lesions were benign as proven by biopsy (n = 2) and CT attenuation values of less than 10 H (n = 6). The mean standardized uptake ratio value for these eight lesions classified as benign was 1.77 (range, 0.93-3.70). The sensitivity for detecting metastatic disease was 100%, and the specificity was 80%. CONCLUSION: PET with FDG is an accurate, noninvasive way to differentiate benign from metastatic adrenal masses in patients with bronchogenic carcinoma.

Adrenal Gland Diseases↗

Nocardial adrenal abscess: CT and MR findings.

To our knowledge, four bacterial adrenal abscesses in adults have already been reported in the international literature, but an adrenal Nocardia abscess has never been described previously. In this report the CT and MR imaging appearances and the differential diagnosis of the entity are discussed. The mass could resemble a malignancy. The observation of a rapid growth and colliquation of the mass helped in distinguishing it from a malignancy. The associated pulmonary infection provided a further clue to the diagnosis. The diagnosis was confirmed by surgery.

Abscess↗

Ultrasonography in the diagnosis of neonatal adrenal haemorrhage.

Two cases of neonates with adrenal haemorrhage are presented. The clinical, radiological and ultrasonographical signs are described. The future role of echography as compared to other radiological procedures, including computerized tomography, is discussed. Ultrasonography is recommended as a screening method, and is well suited for assessing the size and internal structure of pathological processes.

Adrenal Gland Diseases↗

Computed tomography and angiography in the evaluation of adrenal diseases.

Computed tomography, adrenal venous blood sampling, phlebography and arteriography were compared as diagnostic methods in 50 proven cases of adrenal disease. The diagnostic accuracy was 90 per cent for CT, 100 per cent for adrenal venous sampling, 75 per cent for phlebography and 58 per cent for arteriography. It was necessary in some instances to correlate the findings for more than one modality to reach a correct diagnosis. From an analysis of the different examination methods a sequence in the radiologic evaluation of the pathologic adrenal gland is suggested. CT can be used to advantage as the primary imaging method. However, the other modalities, still play an important role.

Adolescent↗

The prenatal diagnosis of adrenoleukodystrophy. Demonstration of increased hexacosanoic acid levels in cultured amniocytes and fetal adrenal gland.

Amniocentesis was performed in two women heterozygous for adrenoleukodystrophy (ALD). One fetus was male, and the hexacosanoic acid (C26) level in the cultured amniotic cells was 0.808 microgram per mg of protein, compared to 0.104 +/- 0.069 (S.D.) in controls. Pregnancy was interrupted at 22 wk gestation. The fetal adrenal cortex showed the ultrastructural inclusions characteristic of ALD and C26 accounted for 35% of the fatty acids in the cholesterol esters extracted from this tissue, more than one thousand times control. The second amniocentesis was performed in a woman who was also heterozygous for an electrophoretic variant of glucose 6- phosphate dehydrogenase (G6PD), and a member of a kindred showing genetic linkage of loci for ALD and G6PD. The fetus was female and the C26 level in cultured amniotic cells was 0.577. Pregnancy was interrupted at 11 wk for reasons unrelated to ALD. Study of C26 level and G6PD type in cultured fetal tissues confirmed heterozygosity for ALD.

Adolescent↗

Localization of the steroidogenic acute regulatory protein in human tissues.

The rate-limiting step in steroid hormone production in the adrenal cortex and gonads, the translocation of cholesterol from the outer to the inner mitochondrial membranes, is mediated by the steroidogenic acute regulatory protein (StAR). Heretofore, the localization of StAR in human adult and fetal tissues has not been defined. To this end, expression of StAR was detected in formalin-fixed, paraffin-embedded specimens using a polyclonal antiserum raised against recombinant human StAR. Primordial follicles of adult ovaries did not contain StAR, whereas antral follicles stained intensely in the thecal layer, with occasional staining of granulosa cells. Corpora lutea were intensely stained, but with a patchy distribution. Corpora albicantia did not stain. A luteoma of pregnancy stained with patches of moderate intensity. Ovaries with hyperthecosis contained areas of intense thecal staining. An ovarian Leydig cell tumor stained intensely, whereas granulosa cell tumors were negative. Ovarian adenocarcinomas, borderline tumors, teratomas, cystadenomas, and a Brenner tumor displayed no specific StAR immunostaining. Testicular Leydig cells stained moderately to intensely, as did a testicular Leydig cell tumor. Sertoli cells stained weakly in some specimens. Seminomas and testicular germ cell tumors were negative. There was minimal to moderate staining in the adrenal glomerulosa and faciculata and minimal staining in the reticularis, while the medulla was negative. Adrenal cortical adenomas, hyperplasias, and carcinomas all contained areas of StAR staining. The renal distal tubules stained with moderate to marked intensity. Renal carcinomas had occasional modest staining. No immunostaining was found in the placenta. Fetal ovaries contained sporadic stromal cells displaying intense StAR staining, particularly in the hilar region. Oocytes from a 32-week fetal ovary showed moderate to intense staining. Fetal testes displayed intense Leydig cell staining. The neocortex of the fetal adrenal glands displayed only minimal StAR staining, whereas moderate to intense staining was found in the fetal zone. The fetal kidneys had moderate StAR staining of the distal convoluted tubules. We conclude that StAR is localized to normal and neoplastic cells in the gonads and adrenal cortex, which produce large amounts of pregnenolone. StAR protein was not detected in the placenta, documenting that placental progestin synthesis occurs through StAR-independent mechanisms. The presence of StAR in cells that do not express cholesterol side-chain cleavage enzyme cytochrome P450, including renal distal tubules, Sertoli cells, and fetal oocytes, suggests that StAR has roles in metabolic processes in addition to stimulating pregnenolone synthesis.

Adrenal Gland Diseases↗

Clinicopathologic characteristics of septicemia in sickle cell disease.

Bacterial septicemia occurred in 22 of 210 pediatric patients with sickle cell disease over a nine-year period, an attack rate of 10.5%. Children less than 2 1/2 years old with hemoglobin SS were at greatest risk; Streptococcus pneumoniae was the most common pathogen. Although sudden febrile illness developed in nine patients, in 13 a gradual prodrome was associated with symptoms for more than 24 hours before evaluation. Both initial body temperature and WBC count were highly variable. In all six fatal cases, autopsy disclosed adrenal hemorrhagic necrosis, which in three was diffuse and typical of that seen in the Waterhouse-Friderichsen syndrome. Recommendations in patients with sickle cell disease and fever, even if only of moderate degree, include the rapid initiation of antibiotic therapy and the use of corticosteroids at the first sign of circulatory insufficiency.

Adolescent↗

Clitoral cyst as a cause of ambiguous genitalia.

BACKGROUND: Ambiguous genitalia need appropriate evaluation, because a serious disease can manifest as this condition. CASE: A 28-year-old woman was referred with a "penis" present from age 12 years that enlarged throughout puberty but recently was unchanged. Differential diagnosis included true hermaphroditism; adrenal hyperplasia; clitoral, ovarian, and adrenal neoplasms; stromal hyperthecosis; polycystic ovarian syndrome; and exogenous androgen exposure. Free testosterone was not elevated. Dehydroepiandrosterone sulfate, 17-hydroxyprogesterone, total testosterone, androstenedione, deoxycorticosterone, 11-deoxycortisol, chromosomal analysis (46,XX), an intravenous pyelogram, and pelvic ultrasound were normal. The patient elected surgical removal. The mass was a chronic inflamed epidermoid cyst. CONCLUSION: If this patient had been appropriately evaluated earlier, she would have been spared anxiety and embarrassment over a simple epidermoid cyst.

Adrenal Gland Diseases↗

CT evaluation of active adrenal histoplasmosis.

We describe three patients with puzzling systemic illnesses in whom computed tomography (CT) led to the diagnosis of active adrenal histoplasmosis. CT was performed in two patients because of gastrointestinal symptoms and weight loss and in the third because of fever developing 2 years after an apparent cure of histoplasmosis. All three patients had adrenal enlargement on CT with features of granulomatous adrenal disease in two. Hormone assays performed after CT supported the diagnosis of adrenal insufficiency and fine-needle adrenal aspiration biopsy with special stains of the aspirates showed histoplasma organisms in all three patients. The combination of adrenal enlargement on CT and laboratory findings of adrenal insufficiency should suggest the diagnosis of histoplasmosis, especially in endemic areas.

Adrenal Gland Diseases↗

The surgical treatment of adrenal disease.

Since the advent of cortisone replacement therapy in 1950 made adrenal surgery possible, it has had the reputation of being fraught with medical and technical difficulties. However, with good endocrine medical cooperation, expert anaesthesia and sound technical expertise, excellent results are obtainable.

Adrenal Cortex Hormones↗

Perinatal haemorrhagic necrosis of the adrenal gland. A clinical and radiological evaluation of 24 consecutive cases.

Up to 1970 only13 fully verified and successfully treated cases of neonatal adrenal haemorrhage were on record. As uniform diagnostic and therapeutic guiding principles are still lacking, our experience of 24 consecutive cases appears worth presentation.--The most frequent clinical signs were a palpable mass in 21 cases, jaundice in 14 patients and urinary infection or sepsis in 10 neonates.--Intravenous urography revealed the diagnosis with a reasonable degree of reliability in all our cases. No additional information of significance was obtained at aortography, employed in 2 of our cases.--Surgical exploration with drainage of the haematoma or adrenalectomy was performed in 18 cases, 6 were treated conservatively.--Recovery was uneventful in all but for one conservatively treated patient. In this case death was caused by a second profuse bleeding, which proved fatal in spite of all life saving efforts.

Adrenal Gland Diseases↗

Adrenaloma: a call for more aggressive management.

We review our experience from the surgical management of 57 patients (24 males, 33 females) with a mean age of 48.5 years who underwent adrenalectomy because of the computed tomography (CT) finding of a "nonfunctioning" adrenal tumor (adrenaloma). We found that CT consistently underestimated the real histologic size of the adrenal tumor (p = 0.001). Of the 57 resected tumors, 23 were cortical adenomas, 7 myelolipomas, 8 adrenal cysts, 11 nodular hyperplasias, 2 primary adenocarcinomas, 2 metastatic carcinomas, and 4 pheochromocytomas. The mean diameter was 5.89 cm and the mean weight 114.07 g. The mean diameter of the resected primary adenocarcinomas was 3.0 cm and 4.5 cm, respectively. The operative mortality was zero and the perioperative morbidity minimal. The mean operating time was 137 minutes (range 60-240 minutes). The posterior approach had the shortest operating time and the laparoscopic approach the shortest hospital stay and the least postoperative need for narcotics. During the 6.2 years mean follow-up period, five patients with preoperative hypertension remained normotensive, and both patients with the resected primary adenocarcinomas are alive without recurrence. We suggest a more liberal surgical approach to patients with adrenalomas because: (1) even small tumors can be malignant or potentially lethal (e.g., pheochromocytomas); (2) some tumors that appear to be nonfunctioning may in reality be functioning; and (3) other nonfunctioning tumors may, with time (and without prior notice), function. The low risk of adrenalectomy especially via the laparoscopic approach can provide an early definitive diagnosis and treatment, avoiding the cost of repeated CT scans and other studies as suggested by the currently prevailing conservative management of these tumors.

Adenocarcinoma↗

Differential ovarian and adrenal vein catheterization.

Twenty-two hirsute women underwent percutaneous adrenal and ovarian vein catheterization to differentiate and localize excessive hormonal output. All studies were done under fluoroscopic control; catheter placement was verified by venography, and blood samples were withdrawn for hormonal analysis. The right ovarian vein was successfully sampled in 42 per cent of attempts; the left ovarian vein, in 75 per cent; the right adrenal vein, in 56 per cent; and the left adrenal vein, in 100 per cent. Bilateral catheterization did not prove clinically useful. First, anatomic variations in venous size and drainage made catheterization and bilateral sampling difficult. Second, adrenal secretion is both episodic and parallel, necessitating both simultaneous catheterization and serial sampling for adequate diagnosis. The stress of the procedure may provoke increased adrenal output. Third, since ovarian secretion is not parallel, and since increased hormone output has been documented in that ovary containing developing follicles or a corpus luteum, distinguishing ovarian dysfunction proved difficult. Finally, for a time-consuming procedure, patient discomfort cannot be disregarded. This technique has not proved to be a reliable means of determining the site of androgen hypersecretion and thus cannot be recommended in the routine evaluation of female hirsutism.

17-Ketosteroids↗