Complete and partial agenesis of the dorsal pancreas within one family.
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Biomedical subjects
Publications and source records attributed to F Schreiber.
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Peritoneal serous papillary carcinoma is a rare primary tumor of the peritoneum. We report here a case diagnosed by laparoscopy, and summarize the clinicopathological features previously reported in patients with this tumor. Laparoscopy was performed in a 66-year-old woman with high-protein ascites and a three-month history of lower abdominal pain. Macroscopically, the parietal and visceral peritoneum was studded with prominent white nodules up to 5 mm in diameter. Multiple biopsies revealed a tubulopapillary serous adenocarcinoma. After exclusion of metastatic peritoneal carcinomatosis (especially ovarian cancer) and malignant mesothelioma, the diagnosis of peritoneal serous papillary carcinoma was established. The patient was placed on chemotherapy (first-line: 5-fluorouracil, adriamycin, and mitomycin C; second-line: paclitaxel). She died 22 months after diagnosis. The present case is also remarkable for the coexistence of granulomatous peritonitis overshadowing the malignant nature of the process. This case report emphasizes the importance of laparoscopy with multiple biopsies in the workup of undetermined exudative ascites.
Hb Sherwood Forest has been so far identified in only one patient in 1977. This study describes the second detection of this hemoglobin variant by routine high performance liquid chromatography (HPLC) in a diabetic patient and her healthy grand niece. In both, glycosylated hemoglobin (HbA1c) values were excessively elevated (52%), as determined by HPLC with a cation exchange column. The latex agglutination test showed values of HbA1c in the expected normal range. Citrate agar electrophoresis revealed a hemoglobin variant with a mobility similar to HbF. Amino acid analysis and DNA sequence analysis revealed an Arg-->Thr exchange at codon 104 of the beta-chain. This sequence has been described as Hb Sherwood Forest in 1977. The hemoglobin variant is clinically silent and might be confused with excessively high HbA1c in routine measurement of glycosylated hemoglobin.
Eighty-eight endoscopists (mean age 41 years, range 29-76 years) and a control group of 100 persons of similar ages were investigated for the prevalence of antibodies (ABs) to Helicobacter pylori, using a quantitative enzyme-linked immunosorbent assay (ELISA) to IgG, two semiquantitative ELISAs to IgG and IgA, and a latex test to IgG and IgM antibodies. The prevalence of antibodies to H. pylori in endoscopists was 48% (quantitative ELISA), 56% (semiquantitative ELISA to IgG), 62% (latex test), and 57% by combined evaluation of semiquantitative ELISAS to IgG and IgA. The respective numbers in the control group were 47%, 48%, 48% and 51%. None of the differences was significant. In both groups, endoscopists and controls, there was a significantly higher H. pylori positivity in older subjects compared to younger persons, but there was no difference between the two groups. The prevalence of ABs was independent to the number of endoscopies previously performed, and independent of protective measures taken, such as wearing gloves during the procedures. Antibody titers as measured with quantitative ELISA showed a positive correlation with the length of time the subject had been active as an endoscopist, but no correlation with the total number of endoscopies performed. In conclusion, the prevalence of ABs to H. pylori in endoscopists follows the age-dependent pattern known from the general population. The regular performance of gastrointestinal endoscopies poses no additional risk of infection with H. pylori in Austria.
A consecutive series of 10 patients with pancreatic stones due to chronic pancreatitis was treated by extracorporeal shockwave lithotripsy during an investigation period of 2 years. The first step of the therapeutic regime included an endoscopic sphincterotomy prior to shockwave treatment. The targeting procedure was performed strictly under sonographic control. All patients were treated with a second-generation electrohydraulic lithotriptor, fragmentation could be achieved in all 10 cases. In 7 patients stonefreeness could be reached by endoscopic stone extraction, all these patients were symptom-free over a follow-up period of 12 months. In 3 patients complete duct clearance could not be achieved, these patients presented with minor symptoms over the follow-up period.
Stone fragments remaining in the gallbladder are an important problem after ESWL. Cisapride (CIS) improves gallbladder contraction and hence we decided to investigate whether clearance of stone fragments after ESWL for radiolucent gallbladder stones can be increased by cisapride. Six to 15 months (median 12) after ESWL 48 patients with remaining gallstone fragments of less than 5 mm in diameter were randomized either to Group A, who received cisapride 10 mg t.i.d. orally for 3 months in addition to oral litholysis (OLL) with ursodeoxycholic acid 500 mg/day and chenodeoxycholic acid 500 mg/day, or to Group B, who continued solely with OLL. All patients had started OLL within the fortnight-preceding ESWL. Gallbladder contractility, as measured by oral cholecystography with a fatty meal, was intact in all patients prior to ESWL. Maximal diameter and number of fragments were assessed by ultrasound (5 mHz) in different positions of the patient at the beginning of the study and after 3 months. Total clearance of fragments, which includes clearance of all sludge, occurred in only 3 patients, two of whom received only OLL. After 3 months the number of fragments decreased in 6 patients in Group A and in 7 patients in Group B. Three patients stopped taking cisapride before completion of the study, two because of diarrhoea, and one because of dysuria. All symptoms were readily reversible after discontinuing cisapride. In conclusion, cisapride combined with OLL does not enhance clearance of the gallbladder when fragments are still present one year after ESWL.
Complete agenesis of the dorsal pancreas has rarely been described. Complete agenesis of the dorsal pancreas in a female who developed insulin-dependent diabetes mellitus at the age of 39 years is reported. The diagnosis of agenesis of the dorsal pancreas was suspected by abdominal ultrasound and confirmed by abdominal computed tomography (CT), magnetic resonance imaging, and endoscopic retrograde pancreatography. Her exocrine pancreatic function was essentially normal. Both of the patient's sons also had agenesis of the body and tail of the pancreas verified by abdominal CT but had no evidence of diabetes mellitus. This familial occurrence of agenesis of the dorsal pancreas suggests that hereditary mechanisms may play a role in the pathogenesis of this anomaly.
Tiamulin pharmacokinetic parameters were determined in 8 2-month-old male improved Swiss Landrace piglets after intake of 2,000 mg/kg feed, 500 mg/kg feed, 12.5 mg/ml aqueous solution administered via a stomach tube and 180 mg/kg feed offered ad libitum. In all cases, the total tiamulin dose received was 10 mg/kg body weight (bw) per day. For the 2,000 mg/kg and 500 mg/kg treatments, animals were restrictively fed a commercial mix in amounts corresponding to 3-fold their maintenance requirement of digestible energy. The piglets first individually received the amount of medicated feed and immediately thereafter the rest of the daily ration. The highest tiamulin serum concentrations (Cmax), the largest area under the curve (AUC0-->infinity), the largest absorption rate constant (Ka), and the shortest time at which the maximum serum concentration occurred (tmax) were obtained after administration via stomach tube followed in the respective order by the 2,000 mg/kg, 500 mg/kg and 180 mg/kg treatments. Ad libitum feeding of the medicated mix at 180 mg/kg failed to provide tiamulin serum concentration above minimum inhibitory concentrations (MIC) of some representative microorganisms. In conclusion, tiamulin concentration in medicated feed strongly influences its rate and extent of absorption and consequently serum concentrations. Larger tiamulin concentration in feed enhances its bioavailability. The common practice adopted by national regulatory agencies for the registration of a new drug is to conduct pharmacokinetic studies after administration agencies for the registration of a new drug is to conduct pharmacokinetic studies after administration via a stomach tube. This practice should be reevaluated because this mode of administration does not correspond to that in routine use.
The case of an 41 year old female is reported, who underwent surgical correction of a congenital cyst of the common bile duct 17 years ago. Because of repeated pain attacks in the right upper quadrant in connection with fever and serochemical signs of cholestasis the patient was admitted to our unit. The exploration with ERCP and CT now showed a common channel in combination with a congenital cyst of the common bile duct. The risk of the anomalous pancreaticobiliary junction upon either the biliary tract or the pancreatic duct will be discussed, also the fact of an higher incidence of malignant tumors of the biliary system. The therapeutic procedures will be discussed.
Six weeks after his return from a two-week vacation in Croatia a 52 year-old janitor from Graz complained of loss of appetite, fever, headache, and a 9-kg weight loss. The spleen was enlarged to 16cm as measured by sonography. Laboratory tests revealed pancytopenia, a prolonged prothrombin time and elevation of serum LDH concentration. While repeated bone marrow biopsy showed no signs of leishmaniasis, high antibody titers against leishmania antigen led to the diagnosis of kala-azar. The indirect immunofluorescent antibody test (1:128) and a haemagglutination-inhibition test (1:512) showed diagnostic elevations of titers. Therapy with pentostam led to prompt defervescence and resulted in a full recovery of the patient. After six weeks a marked decrease of antibody titers in the haemagglutination-inhibition test (1:16) could be observed. Leishmaniasis has to be considered in patients with fever of unknown origin who return from Mediterranean countries. Despite a negative bone marrow biopsy a diagnosis is possible on the basis of serological tests. This is important because effective therapy is available as illustrated by this patient and because of the fact that the disease runs a lethal course if the diagnosis is missed.
A huge common bile duct stone of 6 to 3 cm in diameter in a 64 year old lady was approached by ESWL, after endoscopic therapy such as mechanical lithotripsy and removal with the Dormia basket had failed because of the extraordinary size of this stone. A surgical therapy was not possible because of pronounced vascular and cardiopulmonary risk, so ESWL was applied to this patient. The stone was fragmented in two sessions of ESWL under sonographic targeting and the fragments were removed endoscopically. After one month one fragment still remained in the common bile duct, which then was removed also endoscopically. Two months later the ERC showed a stone-free common bile duct. This case demonstrates, that even common bile duct stones of extraordinary size can be removed by combining ESWL with endoscopic techniques. Following the trend to minimally invasive therapy, sonography should be preferred over XRay in diagnosis before ESWL as well as during ESWL.
A patient with multiple hamartoma syndrome or Cowden's disease with multiple gastrointestinal polyps and malignant melanoma is presented. The syndrome is characterized by hamartomatous tumors of the skin, fibrocystic disease of the breasts, gastrointestinal polyps and disease of the thyroid gland such as goiter and adenoma. In addition, other abnormalities and malformations occur in the skeletal system, central nervous system and urogenital tract. Recognition of this syndrome is important because of the association with malignant tumors of the breast and thyroid gland and with malignant melanoma. Gastrointestinal hamartomatous polyps may lead to the diagnosis of Cowden's disease which must be separated from other intestinal polyposis syndromes.
270 patients with a scintigraphically cold thyroid nodule of sonographically increased (n = 34), diminished (n = 72) or neutral (n = 86) echogenity or cystic criteria (n = 78) were subjected to fine needle aspiration biopsy. This revealed unequivocal malignancy in 8 and follicular neoplasia in another 30 patients, 10 of whom proved to have malignomas on further evaluation. A total of 12 papillary and 2 follicular carcinomas, 2 non-Hodgkin lymphomas, 1 sarcoma and the metastasis of a breast carcinoma were diagnosed. The most sensitive criteria for malignancy were diminished echogenity, an inhomogeneous echo pattern and the occurrence of a solitary nodule. The incidence of malignancy was increased among males but not among especially young persons. There was no sonographic feature that would permit omission of fine needle aspiration.
The serum enzyme pattern, consisting of GOT, GPT, lactate dehydrogenase, gamma glutamyl transpeptidase and alkaline phosphatase, was investigated in 128 patients with sonographically verified liver metastases. Gamma glutamyl transpeptidase and alkaline phosphatase turned out to be the most sensitive enzymes, being elevated in 89% and 88%, respectively. The GOT was elevated in 45% GPT in 37.5% and lactate dehydrogenase in 56% of all cases. The enzyme elevation did not correlate with the degree of liver involvement. In conclusion, pathological serum enzyme patterns are useful for the detection and follow up of liver metastases. Normal serum enzyme levels do not rule out the presence of liver metastases.
On the occasion of 35 episodes of oesophageal injection therapy in which partly the intravascular and partly the perivascular injection technique were used, real time sonographic measurements were performed on the veins of the portal system immediately before and after sclerotherapy. In the case of intravascular injection, both the portal and the splenic vein and the confluens of the splenic and the superior mesenteric vein showed an increase in diameter, whereas in the cases of perivascular injection such changes were minimal or absent. The practical applicability of such sonographic calibrations could possibly lie in certain conclusions that could be drawn concerning the degree of vascular compression or obliteration during each session of sclerotherapy.
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