Search PubMed⌕ Search

Biomedical subjects

W Wegmann

Publications and source records attributed to W Wegmann.

At least 37 records · Page 2Linked to original sources
PubMed1995

Karyomegalic interstitial nephritis: further support for a distinct entity and evidence for a genetic defect.

Karyomegalic interstitial nephritis was first described in 1979 by Mihatsch, who was reporting three such cases. We report here four additional cases as well as two family investigations. Our findings support the association of karyomegaly and interstitial nephritis as a distinct entity. Typical clinical features are asymptomatic progressive renal failure in the third decade of life and recurrent infections, mostly of the upper respiratory tract. Histologic alterations consist of markedly enlarged and hyperchromic nuclei in many tubular epithelial cells throughout the nephron accompanied by interstitial fibrosis in the surrounding atrophic tubules. Karyomegaly is not limited to the kidneys. In one case, autopsy revealed karyomegaly in epithelial and mesenchymal cells of many other organs. However, no association of karyomegaly with further histologic damage is evident except in the kidneys. Because of the familial clustering, karyomegalic interstitial nephritis seems to be an inherited disease. Examination of the nuclear proliferation-associated structures proliferating cell nuclear antigen/cyclin, Ki 67, and p53 suggests an inhibition of mitosis in karyomegalic cells. The finding of the same HLA haplotype, A9/B35, in four of six HLA-typed cases suggests the possibility of a genetic defect on chromosome 6, which is inherited and linked to the HLA locus.

Adult↗
PubMed1995

p53 protein expression in colorectal adenomas: an immunohistochemical study using an antigen retrieval system.

The immunohistochemical expression of the p53 gene product was examined in 91 colorectal adenomas from patients without (group 1, 50 cases) or with (group 2, 41 cases) concurrent sporadic colorectal carcinoma, and in 15 additional cases of randomly selected carcinomas from group 2 patients. Immunohistochemical reactions were performed with the DO-7 monoclonal and the CM1 polyclonal antibodies, following microwave irradiation of the tissues in an antigen retrieval solution, and the proportion of the immunoreactive cells was semiquantitatively assessed. p53 protein immunoreactivity was present in 46.1% (42, of 91, i.e., 20 out of 50 of group 1 and 22 out of 41 of group 2) and 33% (30 of 91, i.e. 14 out of 50 of group 1 and 16 out of 41 of group 2) of the adenomas using DO-7 and CM1 antibody, respectively. High p53 expression (i.e. immunolabelling of more than 30% of the tumour cell nuclei) was found in 13.2% of the adenomas (12 of 91, i.e. three out of 50 of group 1 and nine out of 41 of group 2; P = 0.025 using the chi 2 test) using the DO-7 antibody, and in 6.6% of the cases (six of 91, i.e. two out of 50 of group 1 and four out of 41 of group 2) using the CM1 antibody. In carcinomas, 80% of the cases (i.e. 12 of 15) were found to express p53 protein with both antibodies. p53 immunoreactivity in colorectal adenomas increased with the degree of dysplasia: only five (17.8%) of the 28 adenomas with mild dysplasia were found to be DO-7 positive, while all of them remained CM1 negative. From the 50 adenomas exhibiting moderate dysplasia, 28 (56%) were DO-7 positive, and 22 (44%) were CM1 positive. Finally, from the 13 adenomas with severe dysplasia, nine (69.2%) and eight (61.5%) were found to be positive with the DO-7 and the CM1 antibody, respectively. Our results indicate that an increased number of group 2 adenomas express p53 protein, when compared with group 1 adenomas, and suggest that a strong correlation exists between p53 protein expression and the degree of dysplasia in colorectal neoplasms.

Adenoma↗
PubMed1995

A deletion mutant of the LMP1 oncogene of Epstein-Barr virus is associated with evolution of angioimmunoblastic lymphadenopathy into B immunoblastic lymphoma.

The latent membrane protein 1 (LMP1) oncogene is one of the major proteins synthesized by the Epstein-Barr virus (EBV). It is expressed in Reed-Sternberg cells of Hodgkin's disease (HD), tumor cells of nasopharyngeal carcinoma (NPC), and immunoblasts of angioimmunoblastic lymphadenopathy (AILD). A particular LMP1 deletion mutant was recently identified in NPC and clinically and histologically aggressive HD. We studied two patients with AILD that subsequently progressed into immunoblastic lymphoma (IBL) in order to investigate whether the LMP1 deletion mutant was implicated in progression of AILD into IBL. Immunohistology and in situ hybridization were performed on diagnostic biopsies. DNA extracted from fresh frozen material was used for rearrangement studies and polymerase chain reaction (PCR) based amplification and sequencing of portions of the LMP1 gene. Immunohistochemistry revealed B cell origin of both cases of IBL. In the first patient clonal rearrangement of the immunoglobulin heavy-chain gene was present in IBL but not in AILD. In this patient, scattered immunoblasts of AILD and numerous tumor cells of B-IBL were shown to contain EBV transcripts (EBER1) and to express LMP1. Sequence analysis of the LMP1 gene from AILD and IBL in the first, and from IBL in the second patient, revealed identical deletions and point mutations. This LMP1 deletion mutant is identical to those which have been reported in HD and NPC. Its association with evolution of AILD into B-IBL, aggressive HD and NPC, suggests that this particular mutant is more widespread than originally thought and is clinically relevant.

Adult↗
PubMed1994

[Prolonged course of an idiopathic hypereosinophilic syndrome with transition to eosinophilic gastroenteritis].

Idiopathic hypereosinophilic syndrome (IHES) is a multi-system disorder of unknown origin with eosinophilic infiltration of bone marrow and various organs, including the gastrointestinal tract. Involvement of the heart has a poor prognosis. The etiology of eosinophilic gastroenteritis (EGE) also is unclear. In this disease there is exclusive involvement of the gastrointestinal tract (including liver/bile ducts) and prognosis usually is good. Transition from one disease to the other has not yet been described. We report the 20 years' history of a male patient with an initial IHES without gastrointestinal symptoms and transition to a clinically pure EGE with resolution of any other involvement. At the age of 45 years the patient developed IHES with fever and constitutional symptoms. Fluctuating eosinophilia and symptoms necessitated continuous steroid therapy. After a 10 years' disease course watery diarrhea and malabsorption syndrome became more and more prominent. On the other hand, bone marrow eosinophilia regressed completely and blood eosinophilia partially. Biopsies showed a severe EGE of the mucosal type with involvement of the whole gastrointestinal tract. Besides the prominent eosinophilia there was a dense infiltration of the intestinal mucosa with T-lymphocytes invading the epithelium. We postulate that the destructive eosinophilic/lymphocytic inflammation is caused by a pathologic proliferation of T-lymphocytes with liberation of type 2 helper cell cytokines and consecutive stimulation of eosinophils.

Aged↗
PubMed1994

[Recurrent and metastasizing hemangiopericytoma of the meninges with paraneoplastic hypoglycemia].

Primary meningeal hemangiopericytomas (formerly referred to as angioblastic meningiomas) are by most authors no longer considered to be actual meningiomas but rather thought to be intracranial hemangiopericytomas. Their biological behavior is usually malignant, with recurrences and metastases, often at intervals of years. Both intracranial and extracranial hemangiopericytomas may, however rarely, be accompanied by paraneoplastic hypoglycemias. Our own characteristic observations are based on a female patient aged 67 at the time of her death, in whom a meningeal tumor was resected first at the age of 41. Later recurrencies were removed at the age of 52 and 58 respectively. Three years prior to her death liver metastases had developed followed by increasingly frequent attacks of early morning hypoglycemia with blood sugar levels ranging between 1.4-2.3 mmol/l. Specific examinations revealed low endocrine production of insulin and a distinctly decreased insulin-like growth factor (IGF) I of 25 ng/mb (normal 120-130) and a normal value of total IGF II of 724 ng/ml (normal 400-900), though with a big macromolecular share. The observed paraneoplastic hypoglycemia is probably brought about by coincidence of blocked hepatic glucose production, suppressed lipolysis and increased peripheral glucose uptake. Autopsy revealed a third intracranial recurrence of meningeal hemangiopericytoma and a large metastatic liver. No other sites of metastases were found. Histologic, immunohistologic and electron microscopic findings showed the characteristics features of a hemangiopericytoma. Light microscopic pictures of the primary tumor, recurrences and metastases were identical. Additional autopsy findings were a papillary carcinoma of the right kidney, an angiomyolipoma of the left kidney and a thecoma of the left ovary.

Aged↗
PubMed1994

[Signet ring cell carcinoma of Vater's papilla: a very rare malignancy].

We describe a rare variant of carcinoma of the ampulla of Vater. A 70-year-old man with several illnesses and a newly appeared jaundice was investigated and a signet-ring cell carcinoma of the ampulla of Vater diagnosed. We performed a local transduodenal excision. One year after operation the patient is asymptomatic. This is the second case report in the literature.

Aged↗
PubMed1994

[Nephroblastoma-like kidney adenoma: case report of an extremely rare kidney tumor].

The nephroblastoma-like adenoma of the kidney is an extremely rare tumor with similarities to the nephroblastoma (Wilm's tumor) of the adult. In the literature, different terms are applied to these lesions. We present the case of a 64-year-old woman who underwent a nephrectomy for suspicion of renal cell carcinoma. Histologically the tumor consisted of tubular and papillary formations of monomorphic tumor cells without mitoses or cell-atypias and showed no fibrous capsule. The differential diagnosis to the nephroblastoma of the adult was difficult. Immunohistochemically the tumor expressed the embryonic form of the neural cell adhesion molecule (N-CAM) interstitially and not in the epithelial tumor cell as seen in Wilm's tumor. For the biologic tumor-definition it is important to collect and follow these very rare cases.

Adenoma↗
PubMed1993

[Simultaneous occurrence of lymphocytic gastritis and lymphocytic colitis with transition to collagenous colitis].

Lymphocytic gastritis and lymphocytic colitis are two rare disorders of unknown etiology, only diagnosable by histology. Simultaneous occurrence of lymphocytic colitis and lymphocytic gastritis has not been described up to now. A 69-year-old female patient was examined because of crampy abdominal pain and watery diarrhea. Laboratory tests did not reveal an etiology and in colonoscopy the colon and terminal ileum were normal. Histology disclosed lymphocytic colitis. Esophagogastroduodenoscopy showed erosive bulbitis. Biopsies of the stomach revealed lymphocytic gastritis. A second colonoscopy one year later showed the development of collagenous colitis.

Aged↗
PubMed1993

Diagnostic tools for differentiating between pleural mesothelioma and lung adenocarcinoma in paraffin embedded tissue. Part I: Immunohistochemical findings.

Specimens of 27 histologically definite mesotheliomas and 34 proven adenocarcinomas were examined with a panel of 14 antibodies: pan-epithelial antibody Lu-5, anti-keratin-18, anti-keratin-7, Ber-EP4, anti-Leu-M1, HEA-125, anti-carcino-embryonic antigen (CEA), anti-blood group-related antigens (anti-BGR A, B, H), B72.3, anti-placental alkaline phosphatase (PLAP), anti-vimentin and BMA-120 used to determine their value in the differentiation between pleural mesothelioma and lung adenocarcinoma. Lu-5, anti-cytokeratin-7 and -18, B 72.3 and PLAP reacted in a high percentage of cases with both mesothelioma and adenocarcinoma. Anti-CEA and anti-Leu-M1 did not react with any of the 27 mesotheliomas tested but showed a reaction in 75% (anti-CEA) and 66% (anti-Leu-M1) of the lung adenocarcinomas. Seventeen percent of the adenocarcinomas and 96% of the mesotheliomas showed a positive reaction with anti-vimentin. Ber-EP4 was demonstrated in all lung adenocarcinomas, but only in 2 mesotheliomas in a focal manner (7%). HEA-125 and anti-BGR A, B, H reacted with 83% (HEA-125) and 75% (anti-BGR A, B, H) of the lung adenocarcinomas. The statistical parameters, sensitivity and efficiency were estimated and a normogram for judging the diagnostic power of a single antibody for the differential diagnosis of mesothelioma versus adenocarcinoma was developed. According to this, Ber-EP4, HEA-125, anti-BGR A, B, H and anti-CEA were, in descending order, the most powerful discriminatory antibodies.

Adenocarcinoma↗
PubMed1993

Diagnostic tools for differentiating pleural mesothelioma from lung adenocarcinoma in paraffin embedded tissue. II. Design of an expert system and its application to the diagnosis of mesothelioma.

A panel of 14 antibodies (panepithelial antibody Lu-5, anti-keratin-18, anti-keratin-7, Ber-EP4, anti-Leu-M1, HEA-125, anti-carcinoembryonic antigen, anti-blood group-related antigens A, B, H, B72.3, anti-placental alkaline phosphatase, anti-vimentin and BMA-120), which have been evaluated for use in differentiating mesothelioma from lung adenocarcinoma, was applied to a group of 24 suspected mesotheliomas. Using the established qualitative, descriptive criteria derived from monovariate statistical analysis of the tumour control groups (definite mesotheliomas, adenocarcinomas), a definitive allocation was possible in only 25% of suspected cases. We therefore constructed two "expert systems", based on multivariate discriminant analysis with either the ALLOC 80 program for ordinal data or a newly developed analysis program for binomial data. With these two systems diagnostic allocation of suspected mesotheliomas was improved to 75% and 79%. The use of binomial data ("positive" versus "negative") in conjunction with the probability-based test system is of particular interest because the primary data are easy to record and the test results have a higher statistical probability.

Adenocarcinoma↗
PubMed1993

Immune-mediated type A gastritis and glomerulonephritis.

The first two cases reported in the literature of patients with immune-mediated type A gastritis with microcarcinoids (as part of a polyglandular syndrome type II in one) in combination with chronic glomerulonephritis are presented. Immune-mediated type A gastritis appears to be another immunologic disorder that can be associated with chronic glomerulonephritis.

Adult↗
PubMed1993

[Perforated small intestine diverticulum as a cause of acute abdomen in Ehlers-Danlos syndrome].

Based on a rare case of perforated small-bowel diverticulum combined with Ehlers-Danlos syndrome (EDS) we investigated in a retrospective study several swiss hospitals for symptomatic small-bowel diverticulosis aiming at any clue on EDS. We came up with 15 more cases, 5 of them with perforation, yet none showed any sign of EDS. A review of the literature on either small-bowel diverticulosis or the intestinal complications of EDS amounted to 131 cases of perforation and 11 cases of symptomatic small-bowel diverticulosis in combination with EDS, 2 of them suffering from perforation. Regarding the rarity of these diseases the number of patients presenting both is surprising. Although the EDS-specific histopathological changes of the skin could not be shown in the specimen of the intestinum, it seems quite reasonable to suggest an increased incidence of diverticulosis in patients with EDS, since the weakness of the connective tissue allows an easy protrusion of the mucosa through the intestinal wall.

Abdomen, Acute↗
PubMed1992

[Secondary oxalosis following small bowel resection with kidney insufficiency and oxalate vasculopathy].

A 58-year-old female patient admitted to hospital for advanced renal failure had a 40 years' history of Crohn's disease and had undergone ileocecal resection. Nevertheless, chronic diarrhea persisted. Subsequently calcium oxalate stones in the urine were repeatedly observed. Progressive renal failure developed. The investigation of the patient showed severe steatorrhea and pronounced hyperoxaluria, and renal biopsy showed severe chronic interstitial nephritis with calcium oxalate crystals. The skin biopsy revealed severe calcium oxalate vasculitis. The pathophysiology and therapy of secondary hyperoxaluria due to small bowel resection are discussed.

Calcium Oxalate↗
PubMed1991

[Combined oral and endoscopic mepacrine therapy in therapy-resistant symptomatic giardiasis].

A case of extremely refractory infection with Giardia lamblia is described. A 28-year-old male patient with a 7-year history of mushy diarrhea and epigastric pain was repeatedly treated orally and intravenously, without success, with metronidazole, tinidazole, ornidazole, and mepacrine. The rate of success for these drugs is generally more than 90%. Only therapy with mepacrine orally in combination with intraduodenal administration, was successful. Therapeutic failure of 5-nitroimidazole derivatives is described in relation to immune deficiency syndromes and IgA deficit. None of these defects were found in this case.

Administration, Oral↗
PubMed1991

[Gastritis associated with Gastrospirillum hominis--a zoonosis?].

We found spiral bacteria (non-Helicobacter pylori, SB) in gastric biopsies of 5 patients corresponding to an incidence of 0.3%. The bacteria were found on the surface of the gastric mucosa and in part tightly packed within the crypts. Contrary to Helicobacter pylori, most of them had no direct contact to the surface and crypt epithelium. They are distinctly coiled, 3.6-5.5 microns in length and on average 0.5 microns thick. Ultrastructural studies revealed sheathed flagella at each pole. In one case the bacteria displayed periplasmic fibrils in pairs as also described in cultures of SB from cats' stomachs. In all 5 cases there was histological evidence of inflammation of the gastric mucosa, i.e. one acute diffuse gastritis, one case of granulomatous and three of slight to medium grade chronic gastritis. Biopsies of 2 patients showed a positive urease reaction in the CLO test. Morphologically very similar SB occur as commensals in the stomachs of various animals, in particular dogs and cats. We investigated the stomachs of four dogs and four cats and found all to be infested with SB. The bacteria were found not only on the surface of the mucosa and in crypts, but within the glands of the corpus and antrum and often also within parietal (oxyntic) cells. Yet despite bacterial colonization there was no evidence of gastritis in dogs. However, all of the cats' stomachs showed slight to medium grade chronic gastritis. Cultivation of SB has not been successful so far, with the exception of cats' stomachs. Since the germs have been defined only morphologically, the question as to how close the relationship is among SB of various origins must for the time being remain unanswered. Furthermore, species-specific pathogenicity and the possibility of contagion from animal to man has not yet been clarified.

Adult↗
PubMed1990

[Chronic gastritis. Pathogenesis, clinical aspects and therapy].

Based on clinical and histopathological features, three different types of chronic gastritis can be distinguished: gastritis A is confined to the proximal stomach and is the result of an autoimmunological process with antibodies against parietal cells and intrinsic factor. It is a rare disorder and may lead to pernicious anaemia. Distal, antral gastritis B is caused by an infection of the gastric mucosa with Helicobacter pylori (HP). Its incidence increases with age (greater than 50% above age 50). Gastritis C may be caused by drugs and alcohol but is mainly found in gastric remnants after partial resection as a consequence of biliary reflux. Gastritis A rarely causes symptoms. A relationship between gastritis B and C and non-ulcer dyspepsia (NUD) is highly controversial. Gastritis B is very closely associated with peptic ulcer disease. HP-positive antral gastritis seems to be the prerequisite for ulcer formation. All 3 types of gastritis carry an increased risk of malignancy, which seems to be positively correlated with hypoacidity, the chronicity of the inflammation and the histopathological feature of intestinal metaplasia. Noninvasive diagnostic tests (serology, breath tests, scintigraphy) are available, but the diagnosis of gastritis is still mainly based on endoscopy and biopsy. Treatment of gastritis A is not possible. Gastritis B can be healed by eradication of HP using bismuth salts and antibiotics but only combinations of 2-3 compounds seem to afford long-term positive results. Further clinical trials are needed to determine whether such treatment is justified in patients with gastritis and NUD. In patients with chronic recurrent peptic ulcers eradication of HP prevents relapses and heals ulcer disease. Gastritis C complicated by severe symptoms or ulcer can only be successfully treated by a biliary diversion, i.e. Roux-Y-gastrojejunostomy. The discovery that gastritis B is caused by HP improves our understanding of peptic ulcer disease and will change therapeutic concepts.

Algorithms↗
PubMed1990

[Dissecting aneurysm of the common iliac artery in Marfan syndrome: rupture following sports activities].

In a 40-year-old man who collapsed after sporting activity and died shortly after in spite of surgery, autopsy revealed a small ruptured dissecting aneurysm of the left common iliac artery. At the site of rupture, in the aorta and in multiple visceral arteries extensive mucoid degeneration of the media was found. Additional findings (bone deformities, bullous emphysema of the apices of both lungs, and familial affliction) made it possible to establish the diagnosis of an oligosymptomatic form of Marfan syndrome.

Adult↗