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Biomedical subjects

J L Doppman

Publications and source records attributed to J L Doppman.

At least 91 records · Page 5Linked to original sources
PubMed1995

Role of surgery in management of adrenocorticotropic hormone-producing islet cell tumors of the pancreas.

BACKGROUND: Ectopic adrenocorticotropic hormone-producing islet cell tumors of the pancreas (ACTH-ICT) are a rare cause of Cushing's syndrome with a severe and rapidly progressive clinical course. METHODS: Charts were reviewed on all patients evaluated and treated for proven Cushing's syndrome caused by ACTH-ICT (n = 12), specifically for the role of surgery in the management of this disease. RESULTS: Ten (83%) of twelve patients with ACTH-ICT had liver metastases at the time of diagnosis (eight of eight with Zollinger-Ellison syndrome, two of four without Zollinger-Ellison syndrome). Surgical management of the primary tumor included three patients who underwent distal pancreatectomy combined with hepatic resection and one patient who underwent laparoscopic enucleation of a tumor from the pancreatic tail. Eight of twelve patients underwent bilateral adrenalectomy to control symptoms of Cushing's syndrome, including three patients who underwent concurrent distal pancreatectomy and hepatic resection. Six of twelve patients died of the disease within 2 1/2 years of diagnosis, four are alive with progressive hepatic metastases, and one has biochemical evidence of disease. CONCLUSIONS: ACTH-ICT of the pancreas is an aggressive tumor, particularly when there is coproduction of gastrin. The benefit of aggressive surgical resection of primary or metastatic ACTH-ICT has not been established. However, palliative bilateral adrenalectomy is justified, because no patients had biochemical cures after aggressive surgical resection in this series.

ACTH Syndrome, Ectopic↗
PubMed1995

Selective venous sampling from the cavernous sinuses is not a more reliable technique than sampling from the inferior petrosal sinuses in Cushing's syndrome.

The purpose of this study was to compare ACTH levels in unstimulated samples obtained from the cavernous sinuses (CS) to unstimulated and CRH-stimulated samples obtained from the inferior petrosal sinuses (IPS) in 15 patients with surgically proven Cushing's disease. After unstimulated samples were obtained through 5-French catheters placed in both IPS, tracker catheters were introduced into both cavernous sinuses, and unstimulated samples were obtained within 5 min of the initial set. The Tracker catheters were removed, CRH was administered, and CRH-stimulated samples were obtained from the IPS. We compared the central to peripheral ACTH ratios in unstimulated samples from the cavernous sinuses to unstimulated and CRH-stimulated samples from the IPS as a basis for distinguishing pituitary from ectopic ACTH production. In addition, we compared the ability of the intercavernous and interpetrosal ACTH ratios to correctly predict the site of the microadenoma. Unstimulated levels of ACTH in the cavernous sinuses were generally higher than unstimulated levels of ACTH in the petrosal sinuses. However, 3 of 15 patients failed to show central to peripheral ACTH ratios greater than 2 in unstimulated samples from the cavernous sinuses and were thus falsely negative for the diagnosis of Cushing's disease (test sensitivity, 80%). By comparison, the test sensitivity for the petrosal sinus samples was 87% for the unstimulated samples and 100% for the CRH-stimulated samples. Lateralization was correct in 6 of 15 patients based on CS samples and in 9 of 15 patients based on IPS samples. Because of the 20% false negative rate, CS sampling without CRH stimulation is not recommended for the differential diagnosis of ACTH-dependent hypercortisolism.

Adenoma↗
PubMed1995

Surgical interruption of intradural draining vein as curative treatment of spinal dural arteriovenous fistulas.

To establish if interruption of the intradural draining spinal vein or surgical excision are curative treatments for spinal dural arteriovenous fistulas (AVFs), the medical records and radiographic studies of 19 patients with spinal dural AVFs and progressive myelopathy were reviewed. Spinal arteriograms were obtained before and within 2 weeks after surgery in 19 patients, and after a delay of 4 months or more in 11 patients. The mean clinical and arteriographic follow up was at 37 and 35 months, respectively. In the 11 patients who underwent excision of the dural AVF there was no evidence of a residual lesion upon immediate or delayed postoperative arteriography. Surgery in eight patients consisted of simple interruption of the intradural draining vein as it entered the subarachnoid space. In six of these patients the vein draining the AVF intrathecally provided the only venous drainage of the AVF. In these six patients there was no immediate (six of six) or delayed (four of six) arteriographic evidence of residual or recurrent flow through the AVF. Two patients had an AVF with both intra- and extradural venous drainage; after intradural division of the draining vein there was residual flow through the AVF into the extradural venous system. In one of these two patients intrathecal venous drainage was reestablished, which required additional therapy. In the other patient the extradural AVF spontaneously thrombosed and was not evident on delayed follow-up arteriography. In patients with spinal dural AVFs with only intrathecal medullary venous drainage, which includes most patients with these lesions, surgical interruption of the intradural draining vein provides lasting and curative treatment. In patients with both intra- and extradural drainage of the AVF, complete excision of the fistula or interruption of the intra- and extradural venous drainage of the fistula is indicated. In patients in whom a common vessel supplies the spinal cord and the dural AVF, simple surgical interruption of the vein draining the AVF is the treatment of choice, as it provides lasting obliteration of the fistula and it is the only treatment that does not risk arterial occlusion and cord infarction. Simple interruption of the venous drainage of a spinal dural AVF provides lasting occlusion of the fistula, as it does for cranial dural AVFs, if all pathways of venous drainage are interrupted. This result provides further evidence that the venous approach to the treatment of dural AVFs can be used successfully.

Adult↗
PubMed1995

Intraoperative ultrasound-directed resection of pituitary tumors.

Microadenomas of the pituitary vary in size, particularly those related to Cushing's disease. They are often not visualized on preoperative magnetic resonance (MR) imaging and may be difficult to find during surgical exploration of the pituitary. To enhance intraoperative localization of pituitary adenomas, we assessed the feasibility of using ultrasound to detect and localize pituitary tumors. Intraoperative ultrasound (IS) in the axial and sagittal planes was performed with an Intrascan system using a 12-MHz mechanically oscillating, end-firing transducer. Interpretation of the scans was performed by two individuals, who were usually blinded to the results of preoperative MR imaging or petrosal sinus sampling. Twenty-eight patients were examined. Eighteen of these patients had microadenomas (1.5-7 mm), all with Cushing's disease; nine had macroadenomas (10-20 mm), three of which were adrenocorticotropic hormone-secreting, three growth hormone-secreting, two thyroid-stimulating hormone-secreting, and one nonfunctioning; and one patient had an intrasellar craniopharyngioma. Normal sellar and parasellar structures, such as intrapituitary cysts, the intracavernous carotid arteries, and the diaphragma sella were easily visualized. Twenty-three of the 28 tumors, including 13 of the 18 microadenomas, were detected on IS (82% sensitivity). Tumors were seen as hyperechoic masses in 19 patients, mixed echogenicity in three, and isoechoic in one. In most macroadenomas IS allowed visualization of the interface between the tumor and the normal pituitary gland. These results indicate the potential of IS to aid the intraoperative localization and definition of pituitary tumors.

Adenoma↗
PubMed1995

High cervical intravagal hypercellular parathyroid gland as the etiology of severe persistent primary hyperparathyroidism.

The preoperative localization and operative management of a high cervical intravagal parathyroid adenoma in a young woman with severe persistent primary hyperparathyroidism is presented. The etiology of persistent hyperparathyroidism is most frequently a missed adenoma that is often in an ectopic location; of these, undescended adenomas represent a small but important proportion. If clinical suspicion and preoperative localization procedures suggest an undescended gland, a direct surgical approach using a high cervical incision and a unilateral exploration is often successful.

Adenoma↗
PubMed1994

Cushing's syndrome in children and adolescents. Presentation, diagnosis, and therapy.

BACKGROUND AND METHODS: Cushing's syndrome is rare in children and adolescents. We analyzed the clinical presentation, diagnostic evaluation, and treatment of 59 patients with Cushing's syndrome between the ages of 4 and 20 years who were admitted to the National Institutes of Health during the period from 1982 to 1992. The cause of hypercortisolism was identified by low- and high-dose dexamethasone suppression tests, the ovine corticotropin-releasing hormone (CRH) stimulation test, imaging studies, and bilateral sampling of the inferior petrosal sinuses combined with administration of CRH. RESULTS: Fifty patients had Cushing's disease, six had primary adrenal disease, and three had ectopic corticotropin secretion. The initial signs were excessive weight gain in 90 percent of the patients and growth retardation in 83 percent. Most patients (81 percent) had normal bone age at the time of diagnosis. Forty-seven percent had hypertension, whereas only 19 percent had mental or behavioral problems. The high-dose dexamethasone suppression test and the CRH stimulation test identified 68 and 80 percent, respectively, of the patients with Cushing's disease. Magnetic resonance imaging of the pituitary indicated the presence of tumor in 52 percent of the patients with pituitary adenomas. The maximal central-to-peripheral ratio of plasma corticotropin during sampling of the interior petrosal sinuses was > or = 2.5 in all the patients with Cushing's disease and < 2.5 in those with ectopic corticotropin secretion. Remission of hypercortisolism was achieved in 48 of the 49 patients who underwent transsphenoidal surgery for Cushing's disease, in all 6 of the patients who underwent adrenalectomy for primary adrenal disease, and in the 2 patients in whom the ectopic source of corticotropin could be identified. CONCLUSIONS: Weight gain and growth retardation are common clinical characteristics of Cushing's syndrome in children and adolescents. Diagnostic evaluation of such patients with CRH stimulation alone and combined with inferior petrosal sinus sampling and imaging studies is accurate, and therapy is usually successful.

Adenoma↗
PubMed1994

Pituitary magnetic resonance imaging in normal human volunteers: occult adenomas in the general population.

OBJECTIVE: To determine the prevalence of focal lesions of the pituitary gland that suggest the presence of a pituitary adenoma in asymptomatic persons. DESIGN: 100 normal volunteers (70 women, 30 men; age range, 18 to 60 years old) were studied by high-resolution magnetic resonance imaging (MRI) of the pituitary gland before and after administration of gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA). SETTING: Occult pituitary adenomas are identified at autopsy in 3% to 27% of unselected asymptomatic patients. The frequency of incidental pituitary adenomas detected by MRI in normal persons is unknown. MEASUREMENTS: The MRI scans from volunteers were randomly mixed with scans of 57 patients with Cushing disease and interpreted independently by three blinded reviewers. RESULTS: Seven women (10%) and three men (10%) had focal areas of decreased signal intensity in the pituitary gland after administration of Gd-DTPA. The lesions ranged from 3 to 6 mm in greatest diameter and were diagnosed as pituitary adenomas by at least two of the three reviewers. When similar lesions were detected on MRI scans in patients with Cushing disease, the positive predictive value for identification of an adenoma at that site was 86%. CONCLUSIONS: About 10% of the normal adult population have pituitary abnormalities on MRI scans that are compatible with the diagnosis of asymptomatic pituitary adenomas. Most pituitary adenomas remain asymptomatic and do not require treatment.

Adenoma↗
PubMed1994

Adrenocorticotropic hormone--secreting islet cell tumors: are they always malignant?

PURPOSE: To evaluate the frequency with which benign occult islet cell tumors cause ectopic adrenocorticotropic hormone (ACTH) syndrome. MATERIALS AND METHODS: Ten patients with Cushing syndrome due to the production of ACTH by a pancreatic islet cell tumor were studied. In addition, 53 cases of ACTH-secreting islet cell tumors in the English-language literature were reviewed. RESULTS: All 10 of the authors' patients had malignant islet cell tumors. Liver metastases were present in all 10 patients at presentation. Five patients are dead, four patients are alive with liver metastases, and one patient is alive without gross evidence of residual tumor after distal pancreatectomy and right hepatectomy. Eight of the 10 islet cell carcinomas produced gastrin in addition to ACTH. In the 53 reported cases of ectopic ACTH production, there was only one benign adenoma with a prolonged follow-up. CONCLUSION: When ectopic ACTH production is caused by an islet cell tumor, the tumor is large and malignant and has usually metastasized to the liver by the time Cushing syndrome is diagnosed. No occult ACTH-producing islet cell tumor was encountered in the authors' experience or in a review of the literature.

ACTH Syndrome, Ectopic↗
PubMed1994

Intraoperative US of the pituitary gland. Work in progress.

PURPOSE: To evaluate the use of ultrasound (US) of the pituitary gland during transsphenoidal surgery as a means of detecting microadenomas in patients with Cushing disease. MATERIALS AND METHODS: Thirteen patients with Cushing disease and one with acromegaly underwent US during transsphenoidal surgery. Mechanically oscillating transducers (10 MHz [n = 8] or 15 MHz [n = 6]) mounted on a 15-cm-long probe were used. RESULTS: Seven adenomas were definitely visualized, two were poorly seen, and four were overlooked. Small tumors (< 5 mm in diameter) tended to be hypoechoic; larger ones, hyperechoic. All four overlooked adenomas were imaged with the 15-MHz transducer. The cavernous sinuses and internal carotid arteries could be seen, but minimal invasion of the medial wall of the cavernous sinus could not. CONCLUSION: Pituitary microadenomas can be detected with transsphenoidal US; however, evaluation of larger series with instrumentation developed specifically for transsphenoidal application will be necessary to determine the ultimate value of this technique.

Acromegaly↗
PubMed1994

Coronary artery calcification: assessment with electron beam CT and histomorphometric correlation.

PURPOSE: To assess the reliability of electron beam computed tomography (CT) in the detection of calcific deposits in coronary arteries. MATERIALS AND METHODS: The authors quantitatively evaluated a total of 4,298 segments of coronary arteries with electron beam CT and histomorphometry. RESULTS: Regression analysis of the electron beam CT calcium score versus histomorphometric calcium area produced an r2 value of .92 (r = .96; P < .0001). Ninety-three percent (78 of 84) of all coronary arteries with stenosis of 76%-100% contained calcific deposits, and 20% (17 of 83) of all coronary arteries with stenosis of 0%-50% contained calcific deposits. CONCLUSION: The amount of calcific deposits detected with electron beam CT correlates highly with histomorphometric measurements. Also, the amount of calcific deposits correlates well with the degree of coronary artery stenosis. Electron beam CT, therefore, is a promising noninvasive technique that can help depict the presence and extent of atherosclerotic plaques.

Adult↗
PubMed1994

Coronary artery disease: prediction with in vitro electron beam CT.

PURPOSE: To investigate the potential to predict coronary artery disease with electron beam computed tomography (CT). MATERIALS AND METHODS: Fifty heart specimens were examined with electron beam CT and histomorphometric techniques to measure calcific deposits as a percentage of blockage. RESULTS: In group A specimens (from cadavers of patients with symptomatic coronary artery disease), calcific deposits were identified at electron beam CT in 41% (584 of 1,426) of all segments of coronary arteries; in group B specimens (with asymptomatic coronary artery disease), in 24% (369 of 1,535) of all segments; and in the normal control specimens, in 4% (47 of 1,337) of all segments; segments (group A vs control specimens, P < .0001; group B vs control specimens, P = .02). On the basis of logistic regression analysis, coronary artery disease would be predicted if the average electron beam CT calcium score exceeds 0.32. The sensitivity and specificity of electron beam CT in the detection of calcific deposits per coronary artery was 94% and 76%, respectively. CONCLUSION: The electron beam CT calcium score appears to be an effective predictor of coronary artery disease.

Adult↗
PubMed1994

Decreased delta-sleep and plasma delta-sleep-inducing peptide in patients with Cushing syndrome.

To evaluate the sleep disturbances of patients with Cushing syndrome and to examine the relationship between the sleep disturbances and plasma levels of delta-sleep-inducing peptide-like immunoreactivity (DSIP-LI), we performed three polysomnographic/endocrinological studies in patients with Cushing syndrome. In study 1, polysomnography was studied in 12 patients and 12 matched normal volunteers. In addition, DSIP-LI was measured every 30 min for 24 h in 9 patients with Cushing syndrome and 12 normal volunteers. The percentage of time spent in delta sleep (stages 3 and 4) was significantly reduced in patients with Cushing syndrome (5.8 +/- 1.4%; mean +/- SEM) compared to normal volunteers (14.0 +/- 2.5%) (p < 0.01). REM sleep indices, however, were not significantly different between the two groups. There was a significant negative correlation between amount of delta sleep and 08.00 h DSIP-LI (r = -0.43, p < 0.05), which is against the notion of a causal relationship between DSIP-LI and delta sleep. The circadian rhythm of plasma DSIP-LI was found to be similar in Cushing patients and normal volunteers. In study 2, we measured plasma levels of delta-sleep-inducing peptide-like immunoreactivity (DSIP-LI) at 08.00 h in 65 patients with Cushing syndrome and 49 normal volunteers. The 08.00 h DSIP-LI concentrations of 797 +/- 57 pmol/l (mean +/- SEM) in the patients with Cushing syndrome were significantly reduced compared to the level of 1,062 +/- 99 pmol/l found in the normal volunteers (p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenocorticotropic Hormone↗