We report five cases of asymptomatic patients with persistently abnormal liver function tests in whom copper metabolism abnormalities resulted in a misleading suspicion of Wilson's disease. Ceruloplasmin levels assessed by nephelometric immunoassay, single radial immunodiffusion and the enzymatic method averaged 53.8, 61 and 52.8% of the mean value obtained in age- and sex-matched controls (p less than 0.001). Twenty-four-hour urinary copper excretion was higher than the normal range in three instances. Four patients exhibited hypertriglyceridemia. Liver histology showed fatty change with or without sinusoidal fibrosis. Electron microscopic examination unexpectedly disclosed mitochondrial and lysosomal changes identical to those described in Wilson's disease. The present observations indicate that biochemical and ultrastructural changes suggestive for Wilson's disease may be observed in the absence of increased liver copper content. Whether such cases represent isolated cases of heterozygosity for the Wilson's disease gene remains to be elucidated.
Endoscopic treatment of superficial gastric cancer has been reported to be effective by many Japanese teams. In this study, the Nd:YAG laser was used to treat superficial gastric carcinoma in inoperable Caucasian patients with the aim of obtaining a complete response, i.e., disappearance of the lesion endoscopically and biopsy specimens negative for cancer. Eighteen patients unsuitable for surgery with various endoscopic patterns of superficial gastric cancer were treated with the Nd:YAG laser. The endoscopic pattern was type I in 4 patients, type II in 10 (5 type IIa, 1 type IIb, 2 type IIc, 2 mixed IIa + IIc), and type III in 4. Staging by echoendoscopy was performed in 11 patients (T1N0). Nd:YAG laser destruction of the gastric tumor was performed in all cases, with a mean of 4.4 laser sessions per patient. Tumor response was assessed by endoscopy and biopsy. Follow-up averaged 33 +/- 23 (SD) months. Five patients died of diseases unrelated to gastric cancer. An initial complete response was obtained in 16 (89%) patients after a mean of 1.7 laser sessions; histologic evidence of cancer persisted in 2 patients during the entire follow-up period. Among patients with an initial complete response, recurrence was observed in 2. One of them was successfully re-treated. At the end of the follow-up period, 14 (77.7%) of the 18 patients had a complete tumoral response; only 4 patients had histologic evidence of cancer. In 3 of these 4 patients, pretherapeutic echoendoscopic staging had not been performed. Among the 14 patients exhibiting a complete response, 3 had negative biopsy results more than 5 years after diagnosis. No complications occurred. In gastric cancer classified as T1N0 on the basis of pretherapeutic echoendoscopy, a high tumor response rate and even 5-year disease-free survival can be obtained with endoscopic Nd:YAG laser treatment. Endoscopic laser destruction thus appears to be a valuable therapeutic alternative to surgery in inoperable patients with superficial gastric cancer.
We report a new case of S.C.C. of the large bowel with multiple liver metastases. A resection of the primary tumour and liver biopsies were performed with administration of a postoperative chemotherapy (5-Fluorouracil). After a stabilization of 3 months, the metastases were rapidly progressive and the patient died a year after the diagnosis. About 70 cases of S.C.C. of the colon and rectum have been described in the literature. It is most common in the fifth decade and occurs equally in male and female. The most frequent locations are the rectum and the sigmoid. Clinical and physical features and common diagnostic methods do not differentiate the S.C.C. from adenocarcinoma. Treatment is the same but the prognosis of S.C.C. appears to be worse than that of adenocarcinoma.
We report a new case of S.C.C. of the large bowel with multiple liver metastases. A resection of the primary tumour and liver biopsies were performed with administration of a postoperative chemotherapy (5-Fluorouracil). After a stabilization of 3 months, the metastases were rapidly progressive and the patient died a year after the diagnosis. About 70 cases of S.C.C. of the colon and rectum have been described in the literature. It is most common in the fifth decade and occurs equally in male and female. The most frequent locations are the rectum and the sigmoid. Clinical and physical features and common diagnostic methods do not differentiate the S.C.C. from adenocarcinoma. Treatment is the same but the prognosis of S.C.C. appears to be worse than that of adenocarcinoma
We retrospectively reviewed the dynamic CT examinations of eight patients with pancreatic lymphoma. Four tumors were rounded masses with well-defined contours, four were more infiltrating lesions. The median cross-sectional diameter of the tumors was 6 cm (range 2.5-12 cm). At dynamic CT, the tumors were hypodense (n = 8) and somewhat heterogeneous (n = 6). Additional features were enlarged lymph nodes, 1-3 cm in diameter (n = 5), dilatation of the biliary tract and pancreatic duct (n = 5), abnormalities in the fat around the celiac trunk and/or the superior mesenteric artery (n = 4), and venous stenosis or occlusion (n = 7). The CT findings of pancreatic lymphoma are more various than has been previously reported. Findings such as small tumor size, well-defined contours, tumor heterogeneity, pancreatic duct dilatation, and venous invasion may be seen. Pancreatic lymphoma cannot be reliably distinguished from pancreatic carcinoma by CT findings alone.
A case of chronic biliary fasciolasis is reported, which was confirmed by endoscopic retrograde cholangiography. After unsuccessful attempts of treatment with classic antiparasitic drugs, cure was obtained with triclabendazole the absorption of which was studied.
A case of chronic biliary fascioliasis is reported, which was confirmed by endoscopic retrograde cholangiography. After unsuccessful attempts of treatment with classic antiparasitic drugs, cure was obtained with triclabendazole the absorption of which was studied.
Clinical presentation, changes in liver function test results, and liver morphology were examined in 41 consecutive patients with vitamin A hepatoxicity. The cause of liver disease was suspected at initial interview in only 13 instances, whereas histological evidence of fat-storing cell hyperplasia with fluorescent vacuoles led to the diagnosis in the remaining cases. Cirrhosis was found in 17, mild chronic hepatitis in 10, noncirrhotic portal hypertension in 5, and "increased storage" alone in 9 cases. During a mean follow-up period of 4.6 years, 6 patients died of causes related to the liver disease. A precise appraisal of drug consumption was obtained in 29 cases. Among them the total cumulative intake was the highest in patients with cirrhosis (423 +/- 103 x 10(6) IU) and significantly lower in those with noncirrhotic liver disease (88.5 +/- 41; P less than 0.02). The smallest continuous daily consumption leading to cirrhosis was 25,000 IU during 6 years, whereas higher daily doses (greater than or equal to 100,000 IU) taken during 21/2 years resulted in similar histological lesions. It was concluded that at least in some western countries chronic vitamin A consumption might represent an appreciable cause of chronic liver disease, the recognition of which mainly relies on expert liver biopsy interpretation. The data also indicate that prolonged and continuous consumption of doses in the low "therapeutic" range can result in life-threatening liver damage.
Single antibody-secreting spot-forming cells (SFC) of the 3 main isotypes were counted in lymphoid cells from the gut lamina propria (LP), Peyer's patches (PP), mesenteric nodes (MN) and spleen (SP) of rats immunized 2-6 times intraduodenally (ID) or intraperitoneally (IP) with cholera toxin (CT). Responses for all isotypes peaked in all tissues after 4 ID- or IP-immunizations at much larger values than previously reported, and significantly decreased thereafter, except in LP and PP after IP-injections, where IgA- or IgG-SFC, but not IgM-SFC, only appeared or increased after 6 IP-doses. The highest IgA-SFC numbers (17% of tested cells) were in LP after 4 ID-doses. The isotype ration was igA > IgG > IgM in LP and PP after ID-injections, but IgG > IgA > IgM in MN and SP after both ID- and IP-routes. The isotype dispersion was much larger in LP and PP than in MN and SP. Our data show that at least 4 IP CT-doses were required to only elicit a few IgG- and almost no IgA-SFC in LP and PP, outlining the need for intestinal CT-immunizations to induce strong mucosal IgA-SFC responses. We also show good systemic responses elicited by enteral and parenteral routes, and the small contribution of PP in total SFC, particularly after parenteral immunizations.
The authors review the recent literature about the methodology of oesophageal pH monitoring, which has a high sensitivity and a high specificity for assessing gastro-oesophageal reflux. Combined electrodes offer most advantages. Ambulatory recording should be done in hospital under standard conditions (meals), particularly for clinical studies. The best clinical indication is to detect pathological reflux in case of atypical symptoms with negative oesophagoscopy. The authors give their normal values in a series of measurements with two systems as well as their results in a series of cases of oesophagitis of various grades.
Defecatory difficulties may be a source of great distress to patients. Normal defecation requires the coordination of abdominal and pelvic muscles. Throughout the last decade, many studies have demonstrated various and often mixed abnormalities of these mechanisms. Pathophysiological studies are able to determine, in a specific patient, the most probable causes of the obstructive symptom. Progress in the management of such disorders can only be achieved by understanding their pathophysiology.
The feasibility of liver regeneration determination with [2-11C]thymidine and positron emission tomography was investigated in partially hepatectomized rats. Serial tomographic scans were performed over a 120-minute period after injection of [2-11C]thymidine together with tritium-labeled thymidine. Within 10 minutes after injection, positron emission tomography scans showed a twofold higher hepatic uptake in regenerating than in nonregenerating livers. Time-activity curves over the liver area indicated that the maximal uptake was followed by a faster decrease of 11C radioactivity in controls than in regenerating animals, so that total 11C activity remaining in the liver at 120 minutes accounted for 68% of maximum in regenerating and only 38% in controls. Tissue distribution studies performed at 120 minutes showed that total 11C radioactivity, expressed in percent injected dose per gram, was six times higher in regenerating livers than in controls (0.62% +/- 0.07% in regenerating livers and 0.10% +/- 0.03% in nonregenerating livers; P less than 0.001) and correlated with 3H radioactivity measured in the nuclear fraction (r = 0.92; P less than 0.001). When the hepatic uptake was expressed in percent of dose per organ, the difference between both groups increased (2.31% +/- 0.23% in regenerating livers and 0.29% +/- 0.02% in nonregenerating livers; P less than 0.001) because of higher weight of regenerating livers than of nonregenerating livers (3.83 +/- 0.11 g in regenerating livers and 2.96 +/- 0.16 g in nonregenerating livers; P less than 0.001). In other organs examined, no difference in 11C radioactivity was found between the two groups of rats. These results indicated the potential usefulness of [2-11C]thymidine and positron emission tomography for noninvasive measurement of liver regeneration.
The authors review the recent literature about the methodology of oesophageal pH monitoring, which has a high sensitivity and a high specificity for assessing gastrooesophageal reflux. Combined electrodes offer most advantages. Ambulatory recording should be done in hospital under standard conditions (meals), particularly for clinical studies. The best clinical indication is to detect pathological reflux in case of atypical symptoms with negative oesophagoscopy. The authors give their normal values in a series of measurements with two systems as well as their results in a series of cases of oesophagitis of various grades.
We studied digestive manifestations occurring during follow-up of 19 cases of myotonic dystrophy. GI symptoms occurred in 10 patients (53%), mainly dysphagia (n = 7). In patients undergoing investigations (n = 7), their digestive troubles were probably or definitively related to the neurological disease in 6 cases. Upper GI endoscopy was normal in 5 dysphagic patients. The oesophageal manometry was abnormal in 3 of 4 patients. Complications developed in 3 cases (16%). One patient presented two episodes of spontaneous pneumoperitoneum. A second one developed a chronic colonic pseudo-obstruction with megacolon treated by subtotal colectomy. Manometric studies revealed oesophageal aperistalsis, low amplitude of gastro-jejunal contractions but persistence of migrating motor complexes and a normal recto-anal reflex. A third patient developed an acute pseudo-obstruction with ischemic colitis. In conclusion, myotonic dystrophy frequently disturbs digestive tract motility and may cause complications such as intestinal pseudo-obstruction.