[Retrocession of acromegaly after radical excision of a voluminous, bifocal, endocrine tumor of the pancreas. Starting point for the discovery of growth hormone releasing factor].
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Biomedical subjects
Publications and source records attributed to C Partensky.
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This report describes the histologic, immunocytochemical, and ultrastructural study of a multihormonal carcinoid tumor of the pancreas, secreting a growth hormone releasing factor (GRF) which provoked acromegaly. The patient presented a nonfamilial multiple endocrine neoplasia, type 1. The absence of radiologic signs of a pituitary adenoma in conjunction with elevated plasma levels of pancreatic polypeptide, glucagon, somatostatin, as well as growth hormone (GH), led to the discovery of the tumor. Its surgical excision produced a rapid disappearance of most of the clinical and biologic disorders. No immunoreactive GH was found in the tumor using radioimmunoassay and immunocytochemistry. In contrast, three peptides with GH-releasing activity were extracted and characterized. Immunocytochemistry showed that the GRF-reactive cells, together with rare somatostatin-storing cells, made up areas which demonstrated a medullary pattern of growth with extracellular amyloid deposits. Under electron microscopic examination, actively secreting cells were observed which carried endocrine granules of 100 to 150 nm in diameter. The other regions of the tumor presented a different type of growth and were composed of pancreatic polypeptide-, glucagon-, or somatostatin-reacting cells. Cells immunostained with antisera raised against beta-endorphin were also noted. These data suggest that GRF may be a new biologic marker for pancreatic endocrine tumors.
Six cases of hepatic alveolar echinococcosis with involvement of the hepatic hilum and cholestasis were treated by percutaneous biliary drainage. Clinical and morphological follow-up ranged from 18 to 34 months. A decrease of jaundice and bilirubinemia and the regression of the intrahepatic bile duct dilatation were observed in all cases. Biliary drainage was associated with percutaneous drainage of an hepatic necrotic cavity in four cases. Left hepatectomy was performed later in three cases. These results are encouraging and suggest that percutaneous biliary drainage is an effective and useful procedure for biliary drainage in hepatic alveolar echinococcosis with cholestasis due to obstruction of the intrahepatic bile ducts.
In exceptional cases, acromegaly develops as the clinical expression of an ectopic secretion of Growth Hormone (GH) or Growth Hormone-Releasing Factor (GRF), tumorous in origin. In the present report, we describe an instance of acromegaly caused by the secretion of GRF from a voluminous pancreatic tumor. The resection of this tumor resulted in a temporary disappearance of the biological and clinical symptoms of acromegaly, which then reappeared in conjunction with a rise in plasma GRF. From this pancreatic tumor, substances displaying a potent GRF activity were isolated and characterized. Amino acid analyses revealed that they were related to 3 peptides containing respectively 44, 40 and 37 aminoacids. The largest (hp GRF (1-44)-NH2) referred as hp GRF or somatocrinin is considered to be the primary molecule. The pancreatic tumor was multisecreting as proved by high plasma levels of somatostatin, pancreatic polypeptide and glucagon, normalized after the tumor removal, taken together with the immunocytochemical demonstration of the presence of these peptides in the tissue and with the isolation of somatostatin. In contrast hypercalcemia associated with an elevated plasma level of IR-PTH was unmodified by tumor removal. Diagnosis of acromegaly as ectopic endocrine syndrome will probably be facilitated by plasma GRF radioimmunoassay, as a result of production of anti synthetic GRF antibodies.
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Gastrectomy was performed in 30 patients with superficial gastric cancer, equivalent to 12 p. cent of cases operated upon for gastric neoplasms since 1970. Presenting signs were epigastralgia in 19 cases (in 12 cases of the ulcer type), external bleeding from the digestive tract in 4 cases, dyspeptic disorders in 2 cases, and anaemia in one patient. The lesion was asymptomatic in 4 cases, while in 3 patients it was detected by endoscopy after polypectomy or for achlorhydria. In one case the lesion was discovered completely fortuitously. Cancer was suspected by the endoscopist from gross pathological findings in 7 of the 30 cases. An adenocarcinoma was diagnosed after biopsy in 26 cases, and suspected in the remaining 4 patients. Sub-total gastrectomy was employed in 27 cases, total gastrectomy in 2 cases, and extension to a total gastrectomy in one patient. Gross pathology showed cancer of type I : 8 cases, type II (IIa : 1, IIb : 0, IIc : 10) : 11 cases, type III : 6 cases, and mixed types IIc + III : 5 cases. The submucosa was invaded 17 times, the other 13 lesions involving the mucosa only. There were no immediate postoperative deaths. Three patients were not seen again, while 7 died from secondary causes : 5 for reasons unrelated to their cancer and 2 from unknown causes. By applying strict attention to surgical procedures and precise examination of histological data it should be possible to obtain postoperative survivals comparable to those of Japanese series (80 to 90 p. cent at 5 years, 70 p. cent at 10 years).
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Peritoneo-jugular shunt with Le Veen's valve was performed in 16 cirrhotic patients with intractable ascites. The immediate effects were remarkable, with only one early death. In the long term, however, the results progressively deteriorated. Relapses occurred frequently (60%) and sometimes repeatedly, and were due to obstruction of the shunt, which had to be replaced, or in some cases to insufficient flow rate. Numerous complications were observed including, in particular, infections and digestive haemorrhages. Consumption coagulopathy was detected by laboratory tests in 5 cases, but did not produce bleeding. After 8 to 25 months, 31% of the patients are still alive.
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The authors report on two cases of severe ulcerated forms of acute colitis, successfully treated by total colectomy. Laboratory and X-ray investigations, as well as pathological examination of the removed section did not provide a definite etiopathologic classification. They voice the hypothesis of an infectious involvement and discuss the diagnostic and therapeutic problems evoked by these ulcerated forms of acute colitis. These are distinct from Crohn's disease and from ulcerative colitis. Their etiology remains unknown.
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After distal splenorenal anastomosis, as described by Warren, in 23 patients, early assessment of results (after 2 weeks and 2 months) by endoscopic examination and celiomesenteric angiography was used to evaluate the effectiveness of the procedure and its effects on hepatic portal perfusion. The distal splenorenal shunt remained open in almost all cases (22 out of 23), the varices diminished, and postoperative hemorrhage rarely occurred (3/23 cases). The portogastric disconnection was incomplete in 8 patients, preservation of hepatoportal flow not being constant, as 6 cases with partial and 2 cases with complete portal thrombosis were observed.
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Having had a case of necrotising enterocolitis which occured on the third day after delivery and which was successfully treated by total colectomy with secondary restoration of bowel continuity, the authors discuss the pathogenicity of this condition. It can be secondary to a haemorragic or a septic obstetrical complication. It may equally be the sequel of a functional paralytic ileus which, although rare, is one of the classical complications following labour and Caesarean operations. In this way it can be compared to those cases of enterocolitis which are secondary to ileus, such as are found in patients who have a tumour of the colon or in patients who have been treated with neuroleptic drugs.
In order to study the distribution of somatostatin in the upper digestive tract in man, biopsies were taken through endoscopy or at surgery from the fundus, antrum, and duodenal bulb in 15 subjects with no gastroduodenal lesion, 12 patients with severe antral and/or fundic atrophy in the sampling area, 28 patients with an active duodenal ulcer, and 14 patients with a nonmalignant gastric ulcer. The specimens were extracted in 2 N acetic acid and tested for somatostatin content with a specific radioimmunoassay. In the control subjects, the somatostatin concentration (nanograms per milligram of wet weight) was 0.60 +/- 0.12 in the fundus, 1.68 +/- 0.33 in the antrum, and 1.35 +/- 0.30 in the duodenal bulb. Atrophy of the gastric mucosa was associated with a reduction of the somatostatin concentration in the fundus and the antrum. No significant variation was observed in the present series of patients with gastric ulcer. Duodenal ulcer was associated with a reduction of the somatostatin concentration in the antrum (P less than 0.02). These results indicate that somatostatin is widely distributed from fundus to duodenal bulb in adult human subjects, and that lower antral concentrations are observed in patients with duodenal ulcer.