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Biomedical subjects

W Wegmann

Publications and source records attributed to W Wegmann.

At least 19 recordsLinked to original sources

[Sudden cardiac death during sports due to coronariitis].

A case of sudden and unexpected death of a 29-year-old man during a basketball game is reported. Six years before death a transient ischemic attack and a membranous glomerulonephritis were diagnosed. The autopsy revealed as cause of death to be a severe lymphoplasmocytic coronariitis and, in addition, a small single focus of a lymphoplasmocytic aortitis, indicating a systemic disease. A suspected syphilitic vasculitis could not be confirmed. In the literature there are few cases of similar coronary arteriitis are described, such as Takayasu's type of arteriitis in men of young and middle age.

Adult↗

Gastrointestinal pacemaker cell tumor: clinicopathological, immunohistochemical, and ultrastructural study with special reference to c-kit receptor antibody.

Recent studies indicate that a subgroup of gastrointestinal stromal tumors, including gastrointestinal autonomic nerve tumors (GANTs), originate from stem cells that differentiate toward a pacemaker-cell phenotype. These pacemaker cells form a complex network intercalated between the autonomic nerves and the muscle walls of the gastrointestinal tract and are called interstitial cells of Cajal (ICC). The c-kit receptor (CD117) is a sensitive marker for ICC. The aim of our study was to support the hypothesis that GANTs show ICC differentiation. Seven GANTs without convincing smooth muscle or neural differentiation all showed homogeneous reactivity for the c-kit receptor. CD34 was positive in three cases. On electron microscopy, the typical features of GANT were present. Six tumors contained skeinoid fibers. Most tumors were related to the small bowel. They presented as single (two cases) or multiple (five cases) tumors. The presenting symptoms were abdominal bleeding (2), abdominal mass (2), anemia (1), and small-bowel perforation (1). In two cases, liver metastases developed. Because of the close immunohistochemical and electron microscopic similarities of these tumors to the interstitial cells of Cajal, the term gastrointestinal pacemaker cell tumor seems appropriate.

Aged↗

[Tenascin: a simple tool in the diagnosis of collagenous colitis].

BACKGROUND: Collagenous colitis is characterised clinically by chronic, voluminous, watery diarrhoea, and histopathologically by a thickened subepithelial collagen layer and infiltration of the lamina propria with inflammatory cells. In practice, the exact measurement of this subepithelial collagen layer is complicated by its blunt limit, a nonorthogradely affected cell layer and often only focal findings. Immunohistochemically, the subepithelial collagen layer stains positively with the glycoprotein tenascin, a marker for extracellular matrix remodeling. AIM: To assess if immunohistochemical staining with tenascin could be used as a simple and practical tool in the diagnosis of collagenous colitis. METHODS: 86 of the routinely examined colon biopsy specimens with the following initial diagnosis: 33 collagenous colitis, 21 lymphocytic colitis, 15 inflammatory bowel disease (ulcerative colitis or Crohn's disease), 8 unspecific inflammation and 9 without histopathological findings, were incubated with tenascin. They were then evaluated blindly by 5 different investigators (2 senior pathologists, 2 assistant doctors and 1 chief assistant of the laboratory experienced in immunohistochemistry). Each specimen was evaluated semiquantitatively from 0 (for no tenascin-positive subepithelial collagen layer) to 3 (wide, clearly tenascin-positive collagen layer). RESULTS: In specimens with the initial diagnosis "collagenous colitis" the semiquantitatively assessed subepithelial collagen layer was significantly wider than in specimens with other initial diagnosis. Experience and educational level of the investigators did not influence the results. CONCLUSIONS: Immunohistochemical incubation with tenascin is a simple, economical and rapid tool in the diagnosis of collagenous colitis. With the aid of tenascin staining collagenous colitis can be differentiated histopathologically with sufficient accuracy from other colitis.

Biomarkers↗

[Renal oncocytomatosis].

Multiple and bilateral oncocytomas are rare. There are only ten cases that have been previously described. Three of these displayed multiple and bilateral oncocytomas and microoncocytomas, a so-called oncocytomatosis. This report describes a case of renal oncocytomatosis found at autopsy. In addition, we found an adrenal adenoma, a pheochromocytoma and thoracic cicatrices 4 years after curatively resected large cell carcinoma of the lung. In the distal renal tubules we found oncocytic epithelial cells, with partial transition into microoncocytomas. Immunohistochemically, these and the main oncocytomas were CD 10 negative. These findings support the origin of oncocytoma from oncocytically transformed distal tubular epithelium. CGH analysis of the different tumors revealed no common cytogenetic changes. Coexistence of renal oncocytoma with other tumors is rare. Hitherto, coexistence of a renal oncocytomatosis with multiple tumors has not been described.

Adenoma↗

[Difficulties in differentiation between Crohn disease and ulcerative colitis].

We report on the medical history of a 44 year old woman, who has been diagnosed to suffer from ulcerative colitis, first diagnosed in 1991. Until 1994, when she had finally a colectomy, recurrent episodes occurred under full medical treatment. After colectomy the diagnosis had to be revised as histology revealed Crohn's disease. Only a few months after surgery a new episode of Crohns disease developed including the occurrence of an anal fistula.

Adult↗

[Duplication cyst of the stomach antrum as the cause of an acute gastric emptying disorder in an adult].

HISTORY AND CLINICAL FINDINGS: A 35-year old woman without previous history of gastrointestinal complaints developed increasing upper abdominal pain with nausea and postprandial vomiting over 2 days during which she had been unable to take any food. She had no fever; her general physical condition was slightly reduced. While her abdomen felt soft on palpation, she had epigastric pain on pressure but no resistance to palpation. INVESTIGATIONS: Blood picture and routine laboratory tests were unremarkable. Plain film of the abdomen demonstrated marked gastric retention. Abdominal ultrasound showed an invagination in the gastric antrum suggesting a layering phenomenon. Gastroscopy revealed a submucosal tumour-like lesion with central ulceration which obstructed gastric exit. TREATMENT AND COURSE: After conclusion of the diagnostic tests on the first hospital day partial gastric resection (Billroth I) was performed as, in the absence of any surrounding reaction, a leiomyoma had been suspected. The further course was without complication and she was discharged on the 13th hospital day. The surgical specimen revealed a duplication cyst of the gastric antrum. CONCLUSION: (Endo)sonography, computed tomography and endoscopy make it possible to diagnose duplication cyst, as long as it is included in the differential diagnosis of acute abdomen in an adult. Emergency resection of the cyst is indicated because of the acute nature of the symptoms. But even in less urgent cases or as an incidental finding it is the treatment of choice.

Abdomen, Acute↗

The phantom.

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Adult↗

[Coincidence of bone infarct and aggressive bone metastasis].

A 42-year-old male patient presented with a history of persistent pain in the right femur without trauma of 2 months, duration and an episode of bloody stools 3 months earlier with no clinical findings upon examination. X-rays and CT scan revealed a circumscribed lesion with sclerosis and periostal reaction in the right proximal femur. A three-phase bone scan showed a massive hot spot in this area. Primarily differential diagnoses included a Brodie's abscess and/or a tumor. An excisional biopsy of the area was performed and revealed the coexistence of a bone infraction and the metastasis of an adenocarcinoma of unknown origin. The lesion in the bone was resected, filled with autogenous cancellous bone and stabilized with a plate. Further intensive screening with CT of the abdomen, gastroscopy and colonoscopy led to the primary tumor, an adenocarcinoma at the rectosigmoidal junction. No other metastases were detected. This patient presented with severe pain an radiologically divergent findings: a presumably benign process on radiography, but a massive hot spot on scintigraphy. Further procedures such as a CT scan and/or MRI had to be undertaken. If the analysis includes the differential diagnosis of a malignant process, a biopsy must be obtained, and if this reveals a metastasis, the primary tumor must be sought.

Adenocarcinoma↗

[Symptomatic therapy of severe diarrhea in eosinophilic gastroenteritis with the somatostatin analog octreotide (Sandostatin)].

We describe a patient with eosinophilic gastroenteritis whose symptoms included severe watery diarrhea (1.5 l/d), abdominal cramps, and weight loss. Biopsies revealed massive eosinophilic inflammation of the entire gastrointestinal tract. The total plasma eosinophilic count was elevated by up to 30%. Infectious etiologies and chronic inflammatory bowel disease had been ruled out. By use of the somatostatin analogue octreotide the diarrhea decreased immediately in a dose-dependent fashion and the patient recovered within twelve days. In this case octreotide was helpful in controlling severe diarrhea and limiting the subsequent necessary i.v. fluid replacement.

Adult↗

Long-term outcome of idiopathic hypereosinophilic syndrome--transition to eosinophilic gastroenteritis and clonal expansion of T-cells.

We describe a patient with idiopathic hypereosinophilic syndrome, without initial gastrointestinal symptoms, and their transition to eosinophilic gastroenteritis. This patient, a 65-year-old man, presented with fever, constitutional symptoms, peripheral and bone marrow eosinophilia 20 years ago. During the course of the disease, diarrhoea and malabsorption became prominent, whereas bone marrow eosinophilia regressed completely and blood eosinophilia regressed partially. Biopsies showed a severe eosinophilic gastroenteritis of the mucosal type involving the stomach, small bowel and colon. During the final years of the patient's disease, mucosal eosinophilia became less intense and a mucosal infiltration with T-cells dominated. At autopsy, immunopathological studies of small intestines and colon specimens showed a clonal expansion of morphologically normal T-cells in the intestinal mucosa, which expressed the abnormal phenotype CD2+CD3+CD4-CD5-CD8-. Flow cytometry examination of peripheral blood revealed a corresponding abnormal population of CD3+CD4-CD8- T-cells, indicating a systemic spread of the process. The patient eventually died of non-obstructive small bowel infarction with peritonitis 20 years after the onset of the first symptoms. We postulate that the destructive eosinophilic/lymphocytic inflammation is caused by a clonal proliferation of T-lymphocytes with probable secretion of Type 2 T(helper) cell cytokines and consecutive stimulation of eosinophils.

Aged↗

[Tumor of the gastrointestinal autonomic nervous system (GAN-tumor or plexus sarcoma)].

GAN-tumors or plexosarcomas, first described by Herrera et al. in 1984, are uncommon neurogenic stromal spindle cell tumors of the intestinal tract mainly located in the stomach and the small intestine. The distinctive immunohistochemical pattern is the positive staining for vimentin and neuron-specific-enolase. Ultrastructural features are neuron-like cells with interdigitating cytoplasmic processes, dense-core neurosecretory granules, the lack of basement membranes and the presence of interstitial skeinoid fibers. Pain, chronic and acute bleeding are the most frequent but not specific symptoms and the diagnostic delay is reflected by a large average diameter of these tumors. For even smaller tumors and those with a low mitotic rate may metastasize, GAN-tumors must be considered malignant and need a radical surgical resection.

Aged↗

[2 cases of Whipple disease with different outcomes].

Whipple's disease is a rare infectious disease with systemic manifestation on different organs. In 1992 the typical rod-shaped bacillus was identified as a gram-negative actinomycete with distinctive morphologic characteristics and named as Tropheryma whippelii. The diagnosis is established by demonstrating the presence of PAS-positive macrophages in the mucosa, with large cytoplasmatic granules. We present two cases of Whipple's disease with different outcomes. Both were treated with the same antibiotics (penicillin/streptomycin). With this treatment one patient recovered completely. In a follow-up over 7 years no relapse occurred. The second patient died only 4 weeks after the diagnosis was established.

Actinomycetales Infections↗

[Liver cell carcinoma as a late complication of Alagille syndrome (arterio-hepatic dysplasia)].

The Alagille syndrome which is also known as arterio-hepatic dysplasia is an autosomal dominant inherited disorder. In several cases cytogenetic studies revealed an interstitial deletion of the short arm of chromosome 20. The hypoplasia or paucity of the interlobular bile ducts causes a chronic intrahepatic cholestasis. The association with facial dysmorphia, embryotoxon posterior, pulmonary stenosis and vertebral deformities are required for the diagnosis of the complete Alagille syndrome. The occurrence of hepatocellular carcinoma as a late complication of the Alagille syndrome was recognized only 11 years after the first publication by Alagille et al. So far 15 cases complicated by hepatocellular carcinoma have been reported. There is one family where all four siblings suffered from hepatocellular carcinoma. Our own case concerns a 31 year old man who died of hepatocellular carcinoma. The postmortem study of his medical history reaching back to childhood allowed the diagnosis of an unrecognized Alagille syndrome.

Adult↗

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↗

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↗