The Ets-1 transcription factor is involved in pterygial angiogenesis.

Pterygial pathology is characterized by abnormal corneal epithelial proliferation, stromal modulation, matrix degradation and a strong tendency for otherwise absent corneal vascularization. As the proto-oncogene Ets-1 is known to play a key role in angiogenesis and matrix degradation in other tissues, its involvement in corneal vascularization was investigated. Fifteen pterygia representing two groups were studied. Group 1 consisted of five clinically active pterygia, and group 2 consisted of 10 samples of clinically non-active pterygia. (35)S-labelled ets-1 antisense and sense riboprobes were used for in-situ hybridization of Ets-1 transcription factor in all pterygia. The cytoplasm of blood vessel endothelial cells showed strong expression of ets-1 mRNA in all group 1 pterygia. In contrast, no expression of ets-1 was found in group 2 pterygia. Proto-oncogene ets-1 expression has been shown for the first time in the metaplastic pterygium, an eye tissue of unknown pathogenesis.

[Langerhans' cell histiocytosis of the liver. Differential diagnosis of a rare chronic destructive sclerosing cholangitis]. / Langerhans-Zell-Histiozytose der Leber. Differenzialdiagnose einer seltenen destruierenden, sklerosierenden Gallenwegserkrankung.

We report on the difficult differential diagnosis of liver involvement in disseminated Langerhans' cell histiocytosis (LCH). Three years after treatment of LCH involving the skull and pelvic bones, an 18-year-old girl presented with abdominal pain and cholestatic liver disease. At this time, liver biopsy showed portal infiltrates which were diagnosed as chronic non-suppurative destructive cholangitis. Two years later, she was icteric under progredient hepatic failure. A second liver biopsy revealed biliary fibrosis and granulomatous inflammation with destruction of the portal bile ducts. The morphological changes in both liver biopsies could be identified as LCH by immunohistochemical detection of CD1a and S-100-positive Langerhans' cells. Morphological changes and clinical findings in LCH of the liver may resemble primary sclerosing cholangitis or chronic non-suppurative destructive cholangitis. Therefore, LCH is an important differential diagnosis of chronic destructive cholangitis with cholestatic liver disease, especially in children and young adults. The diagnosis can be verified by S-100 and CD1a immunohistochemistry.

Synovial chondromatosis of the temporomandibular joint: clinical, diagnostic, and histomorphologic findings.

Objective. The primary diagnosis of synovial chondromatosis of the temporomandibular joint is extremely rare. Often many months, or even years pass before the diagnosis is confirmed. Study Design. Eight patients with synovial chondromatosis were treated by arthrotomy. In addition to the definitive diagnosis, histologic classification according to the stages proposed by Milgram was undertaken and confirmed with the literature. Results. The predominant symptoms of synovial chondromatosis, including pain, swelling, restricted movement of the mandible, and crepitation, were evaluated. Nevertheless, more than 80% of the patients were previously primarily treated on the basis of other tentative diagnoses. Conclusion. Synovial chondromatosis of the temporomandibular joint should be included in the differential diagnosis of chronic swelling and pain in the preauricular region, and the patient should be sent for appropriate diagnostics and therapy at an early stage.

[Massive pulmonary capillary occlusion by microthrombi. Unexpected cause of fatal right heart failure during liver transplantation]. / Massiver Pulmonalkapillarenverschluss durch Mikrothromben. Unerwartete Ursache eines tödlichen Rechtsherzversagens während Lebertransplantation.

We report the case of a 46-year old man who developed an unexpected fatal cardiac failure during liver transplantation. Attempts at resuscitation were unsuccessful. At necropsy the lungs showed numerous microthrombi occluding small lung vessels and pulmonary capillaries. Thrombi were not found in other organs. The source of this extensive thrombus formation is not known. The thrombi could have been developed within the liver, the venous blood stream between liver and lungs or the pulmonary capillaries. In our experience, this complication is very rare, and a risk profile is not known.

Small bowel obstruction in acute myelogenous leukemia: stenosis or paralysis?

We describe a patient with acute myelogenous leukemia who suffered a small bowel obstruction on the second day of chemotherapy. The patient had to be operated immediately, and the terminal ileum and a part of the colon was removed. The resected specimen showed leukemic infiltration (chloroma) of the bowel with marked atrophy of the muscular layer. However, there was no complete stenosis. For this reason we believe that the reason for the acute abdominal symptoms on the second day of chemotherapy could be paralysis of the bowel due to muscular atrophy.

Immunohistochemical patterns of human liver sinusoids under different conditions of pathologic perfusion.

This study reports the effects of altered hepatic perfusion on the sinusoidal bed and the phenotypic characteristics of sinusoidal endothelial cells (SECs). Sinusoids were studied by the application of endothelial cell markers (CD31, CD34, CD105, and ATZ 11) in lesions with localized increased perfusion (liver cell adenoma, focal nodular hyperplasia, and macroregenerative nodule), in chronic congestion, in decreased portovenous inflow (portal vein thrombosis), and in decreased arteriohepatic perfusion (obliterative arteriopathy in chronic allograft rejection). SECs react in a sensitive and uniform way to all investigated conditions of different pathologic liver perfusion: expression of CD31, CD34, and ATZ 11 by SEC is found in inflow areas, CD105-positive SECs are found at the end of the sinusoidal blood stream. This blood flow-orientated phenotypic shift of SECs was accompanied by a perisinusoidal accumulation of activated hepatic stellate cells and collagen IV. These findings are helpful in liver biopsies and provide new insights into the angioarchitecture of benign nodular lesions.

Creutzfeldt-Jakob disease (CJD) in a case of suspected chronic heavy metal poisoning.

We describe a patient who died of suspected heavy metal poisoning after a nine-month history of rapidly worsening dementia. Autopsy at a forensic-pathological institute established the postmortem diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) based on demonstration of the proteinase-resistant prion protein (PrPsSc) in Western-Blot on native brain tissue. Microscopic examination of the macroscopically largely inconspicuous brain revealed marked spongiform changes in the gray matter--mainly affecting the cerebral cortex, nucleus caudatus, and putamen--with confluent vacuoles. Patchy or perivacuolar deposits of PrPSc were found as well as granular PrPsc deposits. The cerebellum contained focal PrPsc deposits. There was an astrogliosis in the white matter and a proliferation of microglia in the gray matter with a simultaneous clear reduction in neuronal elements. The differential diagnosis is discussed, as is the potential risk to those performing autopsy on forensic cases with a clinical picture of rapidly progressing dementia, especially in cases where a prion disease is not initially suspected.

[Histomorphology versus three-dimensional ultrasound morphology of the rotator cuff]. / Histomorphologie versus dreidimensionale Sonomorphologie der Rotatorenmanschette.

AIM: Sonographic evaluation is a reliable method for the detection of rotator cuff tears. However, the diagnostic value of the different echogenic patterns of the rotator cuff, especially in elderly patients, has led to a controversial discussion. The present study elucidates whether three-dimensional ultrasound can increase the diagnostic significance of different sonographic echogenicity patterns of tendons compared to histopathological tissue degeneration. METHOD: To evaluate the sonographic appearance of tendons in elderly patients, the sonographic echogenicity of 33 rotator cuff specimens with macroscopic absence of rotator cuff lesions was classified in three groups and compared with the histopathological morphology. RESULTS: The sonographic appearance of the degenerated rotator cuff in elder patients correlates with a reduced sonographic echogenicity. The two-dimensional ultrasound evaluation of the rotator cuff led to a sensitivity of 84.0%, a specificity of 42.9%, a positive predictive value of 63.6%, a negative predictive value of 69.2%, and an accuracy of 65.2%. The three-dimensional ultrasound evaluation of the rotator cuff led to a sensitivity of 91.6%, a specificity of 50.0%, a positive predictive value of 66.6%, a negative predictive value of 84.6% and an accuracy of 71.%, respectively. CONCLUSION: As a high incidence of false positive results has to be taken into consideration, neither the two-dimensional, nor the three-dimensional ultrasound evaluation seems to lead to a reliable correlation.

Infarction and laceration of liver parenchyma caused by wedged CO(2) venography before tips insertion.

We describe the fatal outcome of an elective TIPS procedure performed in a 43-year-old man with alcoholic cirrhosis. Wedged hepatic venography with CO(2) was the reason for infarction and laceration of liver parenchyma resulting in a subcapsular hematoma and subsequent intra-abdominal bleeding. This is the first report of this complication after the use of CO(2) in a cirrhotic patient.

[Perforation of mucocele-like transformed Hartmann stump]. / Perforation eines mucocelenartig transformierten Hartmannstumpfs.

We report an unusual complication in a 53-year-old woman following ileostomy for Crohn's disease 22 years previously. A stenosis of the distal colonic segment was the reason for the formation and subsequent rupture of a huge colonic mucocele. To our knowledge, this is the first report of a ruptured mucocele of colonic origin after ileostomy.

Neurologic complications in immune-mediated heparin-induced thrombocytopenia.

OBJECTIVE: To evaluate neurologic complications in patients with immune-mediated heparin-induced thrombocytopenia (HIT) with respect to incidence, clinical characteristics, outcome, and therapy. METHODS: One hundred and twenty consecutive patients with immune-mediated HIT were recruited over a period of 11 years and studied retrospectively for the occurrence of neurologic complications. Diagnosis of HIT was based on established clinical criteria and confirmed by detection of heparin-induced antibodies using functional and immunologic tests. RESULTS: Eleven of the 120 patients (9.2%) presented with neurologic complications; 7 suffered from ischemic cerebrovascular events, 3 from cerebral venous thrombosis, and 1 had a transient confusional state during high-dose heparin administration. Primary intracerebral hemorrhage was not observed. The relative mortality was much higher (Chi-square test, p < 0.01) in HIT patients with neurologic complications (55%) as compared to patients without neurologic complications (11%). The mean platelet count nadir in neurologic patients was 38 +/- 25 x 10(9)/l on average, and was lower in patients with fatal outcome compared to those who survived (21 +/- 13 x 10(9)/l versus 58 +/- 21 x 10(9)/l; p < 0.05, Wilcoxon test). In three patients neurologic complications preceded thrombocytopenia. There was a high coincidence of HIT-associated neurologic complications with other HIT-associated arterial or venous thrombotic manifestations. CONCLUSION: Neurologic complications in HIT are relatively rare, but associated with a high comorbidity and mortality. HIT-associated neurologic complications include cerebrovascular ischemia and cerebral venous thrombosis. They may occur at a normal platelet count.

The three-dimensional ultrasound evaluation of the rotator cuff--an experimental study.

OBJECTIVE: An accurate sonographic diagnosis of rotator cuff lesions significantly depends on image quality and on the experience of the operator. The present study was performed to determine whether the use of three dimensional (3D) sonography further increases the diagnostic yield of ultrasound. METHODS: In an experimental study 22 artificial rotator cuff lesions (seven full thickness and 15 partial thickness tears) of cadaveric shoulder joints were evaluated by ultrasound with two and three dimensional imaging in a water basin. RESULTS: With 3D ultrasound, rotator cuff lesions were more often correctly diagnosed (sensitivity of 77%) than with conventional 2D sonography (sensitivity of 64%). Specificity was 85 and 69%, respectively. In partial thickness tears in particular, 3D imaging was the superior method reaching a sensitivity and specificity of 73 and 77%, respectively, compared to 53 and 61%, respectively, with 2D ultrasound. With 2D and 3D ultrasound eight and 11 out of 13 tendons, respectively, were correctly diagnosed as intact confirmed by histopathological examination. CONCLUSION: Although the results of an experimental study may not reflect the clinical situation, 3D ultrasound appears to facilitate diagnosis of partial thickness rotator cuff tears.

Segmental hepatic vein thrombosis associated with heparin-induced thrombocytopenia II.

We report the case of a 55-year-old man who developed heparin-induced thrombocytopenia II after a vertebral fracture. Autopsy revealed segmental hepatic vein thrombosis of the right lobe with subacute congestion and an activation of hepatic stellate cells. This case shows that heparin-induced thrombocytopenia II is a possible cause of the Budd-Chiari syndrome.

Is traumatic axonal injury (AI) associated with an early microglial activation? Application of a double-labeling technique for simultaneous detection of microglia and AI.

The aim of the present study was to determine whether axonal injury (AI) induces a microglial reaction within 15 days after brain trauma. In 40 selected cases of confirmed AI, the topographical relation of AI and microglial reaction was assessed using an immunohistochemical double-labeling technique for simultaneous demonstration of AI using beta-amyloid precursor protein (beta-APP) antibody and of microglia using CD68 antibody. Although traumatic injury was usually followed by a moderate early diffuse rise in the number of CD68-reactive cells in the white matter, increases in macrophages in areas of AI accumulation were only sporadic and did not occur until after 4 days. At survival intervals of 5-15 days a moderate microglial reaction in regions of beta-APP-positive injured axons was detected, at maximum, in half of the case material. During this interval AI-associated satellitosis-like clusters or stars described by other authors after a survival time of more than 7 weeks were an isolated phenomenon. The prolonged microglial reaction as well as the reduction of beta-APP-positive AI during longer survival periods supports the hypothesis that AI is not primarily chemotactically attractive and that the damage to a portion of beta-APPstained axons may be partly reversible. Most cases clearly require a prolonged interval of more than 15 days before initiation of the final scavenger reaction. For forensic purposes the increase in the number of microglial cells within the region of AI accumulation after a survival time of more than 5 days and the multiple and distinct demonstration of star-like microglial reactions within the white matter after survival times exceeding 7 weeks may provide valuable postmortem information on the timing of a traumatic event.

Enhanced reactivity of Alz-50 antibody in brains of sudden infant death syndrome victims versus brains with lethal hypoxic/ischemic injury. Diagnostic significance after application of the ImmunoMax technique on routine paraffin material.

Alz-50 antibody is immunoreactive with brain tissue of subjects with Alzheimer's disease and can also be demonstrated by immunocytochemistry in neurons of vibratome-prepared brain tissue of victims of sudden infant death syndrome (SIDS). The application of a slightly modified ImmunoMax method enabled us to demonstrate Alz-50 immunoreactivity in paraffin-embedded material. The Alz-50 epitope was detected in the hippocampus region and in nuclei of the medulla oblongata at the level of the inferior olivary protuberance in three diagnostic groups: victims of SIDS (n = 10), infants dying of subacute hypoxia/ischemia with subsequent (re-)perfusion (n = 9), and infants dying of acute ischemia without (re-) perfusion (n = 7). Quantitative evaluation of the hippocampal cortex and the nucleus olivaris inferior disclosed a significantly (P < 0.05) higher percentage of Alz-50-reactive neurons in SIDS cases than in the control groups (hippocampal cortex and the nucleus olivaris; SIDS victims: median = 100%; subacute hypoxia/ischemia: median = 33.6-81%; acute ischemia: median = 89.2-99%). Semiquantitative analysis revealed an equally pronounced preponderance of Alz-50-reactive neurons in SIDS victims versus the control groups. This greater expression in SIDS victims may be due to an ongoing hypoxia/ischemia during agony, but the present paucity of knowledge prohibits definitive elucidation. Nevertheless, the method described here appears to offer the realistic possibility of distinguishing SIDS cases from cases of sudden death in infants due to other causes, i.e., it offers for the first time a positive criterion for the diagnosis of SIDS.

[Dual accidental carbon monoxide poisoning]. / Zweifache akzidentelle Kohlenmonoxidvergiftung.

The bodies of 2 young men were discovered in the hut of a small garden plot. Due to the unclear circumstances of the deaths, a coroner's inquest was ordered. The cause of death was a carbon monoxide poisoning in both victims. At first the source of carbon monoxide was not detectable. According to the reconstruction of the fatal event, the only possible source of carbon monoxide was the charcoal grill found in the hut.