[Pancreatic lipomatosis revealing Johanson-Blizzard syndrome].
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Biomedical subjects
Publications and source records attributed to O Ernst.
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BACKGROUND: Adrenal incidentaloma poses a perplexing problem for physicians. The challenge is to identify the rare functioning or malignant adrenal tumor that should be removed. PATIENTS AND METHODS: From 1970 until December 1996, we managed 579 patients with adrenal hyperplasia or neoplasm. Of these, 110 were followed without any operation and 469 were operated on. The histology from the operated patients revealed 145 pheochromocytomas, 72 Cushing's syndrome, 98 aldosteronomas, 21 virilizing tumors, 3 feminizing tumors, 98 non-functioning adrenalomas, 17 metastases, 7 cysts, and 15 miscellaneous tumors. RESULTS: Among the 98 non-functioning adrenal tumors, 79 were incidentalomas. Histological examination of 103 operated incidentalomas found 55 adenomas (5 functioning=aldosteronomas), 12 benign pheochromocytomas, 3 malignant or suspicious pheochromocytomas, 8 cystic lymphangiomas, 4 metastases, 4 angiomas, 3 ganglioneuromas, 4 myelolipomas, 1 hyperplasia, 2 hematomas, 2 cysts, and 5 adreno-cortical carcinomas. Among the non-operated incidentalomas, the most frequent findings were adenomas, metastases, hematomas, cysts and pseudotumoral hyperplasia; three disappeared spontaneously. No patients died of unrecognized adrenocortical carcinoma. CONCLUSION: Our experience confirms that in case of incidentaloma, the first concern is a nonfunctional pheochromocytoma (15%), and the less common occurrence is adreno-cortical carcinoma (2%). We recommend a restrictive surgical approach in the setting of adrenal incidentalomas.
BACKGROUND: Despite cure of primary aldosteronism by surgical resection, hypertension persists postoperatively in 30% to 50% of patients. The aim of this study was to determine factors influencing long-term outcome of blood pressure after unilateral adrenalectomy for primary aldosteronism. METHODS: Records of 100 patients who underwent unilateral adrenalectomy for primary aldosteronism from 1970 through 1997 were reviewed. Patients were distributed in 2 groups according to whether blood pressure was normal (criteria of World Health Organization). Clinical, biochemical, and pathologic data were compared. RESULTS: All patients were biochemically cured. Blood pressure was normal in 56 patients and improved in 44 (mean follow-up, 69 and 59 months). Persistent hypertension correlated with age, known duration and seriousness of preoperative hypertension, family history of hypertension, no preoperative response to spironolactone, and contralateral adrenal hypertrophy. Gender, surgical approach, and pathologic findings were not predictive factors of blood pressure outcome. The prevalence of hypertension was almost the same in these postoperative patients as the prevalence of essential hypertension in a random population of the same age. CONCLUSIONS: Early unilateral adrenalectomy allows cure or improvement of hypertension in all patients with primary aldosteronism induced by unilateral excessive source of aldosterone secretion regardless of the pathologic findings. Persistent hypertension suggests that coexisting essential hypertension is present.
Magnetic resonance cholangiopancreatography (MRCP) has received much attention in the recent literature as a noninvasive alternative to endoscopic retrograde cholangiography, primarily for biliary calculus disease, but also for the less common indication of evaluation of biliary anomalies. We present a case of Caroli's disease in which the diagnosis can be clearly inferred by MRCP. The findings of MRCP and endoscopic retrograde cholangiopancreatography are similar. This new procedure could be a noninvasive alternative to direct cholangiography and perhaps will become the first-choice imaging technique for diagnosing Caroli's disease.
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OBJECTIVE: This study describes the MR imaging features of congenital hepatic fibrosis in four children who were examined with MR cholangiopancreatography. CONCLUSION: MR cholangiopancreatography effectively reveals biliary cysts, dilatation of intrahepatic bile ducts, and polycystic kidney disease, findings often associated with congenital hepatic fibrosis. This diagnosis should be suspected when these biliary and renal abnormalities are associated with hepatosplenomegaly in a patient with normal liver function.
OBJECTIVE: The purpose of this study was to evaluate the ability of MR cholangiography to reveal the characteristics of biliary abnormalities found in primary sclerosing cholangitis. CONCLUSION: Our results suggest that MR cholangiography could be useful in the diagnosis of primary sclerosing cholangitis. Slightly dilated peripheral bile ducts unconnected to the central ducts in several hepatic segments are a characteristic MR sign of primary sclerosing cholangitis. However, other studies are necessary to establish the usefulness of MR cholangiography in relation to other imaging techniques for evaluating primary sclerosing cholangitis.
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A 15-month-old girl, who presented with biliary cirrhosis secondary to cystic fibrosis with refractory ascites and recurrent intestinal bleeding, underwent percutaneous transjugular intrahepatic portosystemic shunting. Immediately following the procedure the ascites disappeared and no further bleeding occurred. The stent shunt was patent on Doppler ultrasound until the 22nd day. The patient died on day 22 because of liver failure due to a low-flow syndrome with severe hepatic ischaemia, but with no recurrence of bleeding or ascites.
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OBJECTIVE: The aim of this study was to assess the sensitivity of MR imaging in the diagnosis of liver hemochromatosis and its ability to quantify hepatic iron concentration (HIC). SUBJECTS AND METHODS: MR images were prospectively obtained in 58 patients suspected to have hemochromatosis. We used a scanner with a 0.5-T magnet and two sequences: gradient-echo T1-weighted (400/12 [TR/TE], 90 degrees flip angle) and gradient-echo T2*-weighted (700/30, 30 degrees flip angle) sequences. Measurement of the liver-to-muscle signal-intensity ratio was compared with the HIC value measured at biopsy for each patient. RESULTS: Both MR sequences showed significant correlation between decreased signal-intensity ratios and increased HIC (r = -.87 for T1-weighted sequences and r = -.74 for T2*-weighted sequences). The sensitivity and specificity of the T2*-weighted sequence (signal-intensity ratio < 0.8) to detect iron overload (HIC > 36 mumol/g) were 91% and 88%, respectively. The best correlation was obtained with T2*-weighted sequences, when patients had an HIC less than 100 mumol/g (r = -.71); with T1-weighted sequences, the best correlation was obtained when patients had an HIC of 100-324 mumol/g (r = -.67). We found a significant correlation between the HIC revealed on MR images, calculated from both sequences, and that measured at biopsy when patients had an HIC of less than 300 mumol/g (r = -.93, p < .01). CONCLUSION: MR imaging shows promise in differentiating normal from abnormal hepatic iron concentration and in grossly quantifying moderate degrees of hepatic iron overload.
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MR cholangiopancreatography (MRCP) is a new technique allowing noninvasive investigation of the bile ducts and pancreatic duct. Due to the extremely intense signal of water on T2-weighting, MR sequences can be obtained only demonstrating liquids. The bile and pancreatic ducts can therefore be studied spontaneously even in the case of major cholestasis. The contraindications of MR cholangiography are exclusively those of MR. MRCP can visualize the level of a bile duct obstruction and often the nature of this obstruction (stone, tumour). Complementary axial T1- and T2-weighted sequences can also visualize the parenchyma around the ducts. MR cholangiography therefore appears to be a technique of the future for noninvasive investigation of the bile ducts.
We report MRI and CT findings of an intrapancreatic accessory spleen. The lesion was hypervascular on CT and isointense to the spleen on MRI. This uncommon localisation may simulate a pancreatic mass. If the diagnosis is suspected, it can be confirmed by scintigraphy.
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BACKGROUND: Improvement of preoperative imaging of pheochromocytomas and abdominal paragangliomas may render routine laparotomy questionable as the surgical approach of choice for these lesions. METHODS: We studied the records of 100 patients with chromaffin tumors who underwent abdominal exploration. The disease was familial in 28 patients and was malignant in 19. Seventy-five patients had intraadrenal disease (bilateral in 13). Computed tomography (CT), metaiodobenzylguanidine (MIBG) scintigraphy, and magnetic resonance imaging (MRI) were performed since 1979, 1984, and 1987 in 97, 73, and 43 patients, respectively. False-positive and false-negative results were defined as any discrepancy between imaging results and surgical findings. RESULTS: Overall accuracy of preoperative localization was 85% with CT scan, 77% with MIBG scintigraphy, and 86% with MRI. In unilateral pheochromocytoma, accuracy was 94% with CT scan, 80% with MIBG scintigraphy, and 96% with MRI. When all three studies were performed (n = 38), overall accuracy was 97% and only one extraadrenal tumor in a patient with familial pheochromocytoma was overlooked. CONCLUSIONS: The outstanding accuracy of available imaging techniques questions the strategy of routine laparotomy for sporadic and seemingly benign pheochromocytomas, favoring more elective approaches such as the posterior approach or laparoscopy.