Skeletal dysplasias represent in vivo models of genetic defects. Achondrogenesis type II (Langer-Saldino), caused by a genetic defect in the major cartilage matrix protein, collagen type II, is a rare and severe skeletal dysplasia. It comprises a severe derangement of the fetal growth plate cartilage with subsequent ossification defects. In this study, we analyzed the matrix composition and cell differentiation pattern in 3 relatives with achondrogenesis type II. Most strikingly we found a strongly reduced collagen type II and moderately reduced aggrecan proteoglycan content in the dysplastic cartilage matrix. Type II collagen is, at least to some extent, replaced by collagens type I III, and VI. Ultrastructural analysis of the dysplastic cartilage matrix demonstrated a distended rER (rough endoplasmic reticulum), which is typical for this condition and most likely related to improper processing and retention of genetically altered type II collagen. Immunostaining for type IIA and X collagens suggest a severe delay in chondrocyte maturation. Thus, the genetic defect in the present cases leads most likely to a severe retention of collagen type II in the rER and, therefore, a strongly reduced collagen deposition and replacement by other interstitial collagens. However, the latter are less efficient in binding aggrecan proteoglycans in the dysplastic cartilage matrix. Additionally, a delay in chondrocyte maturation appears to be important in achondrogenesis type II.

Background: In contrast to former clinical trials concerning the ablation of non-neoplastic Barrett's epithelium (BE), Schulz et al. (Gastrointest Endosc, 2000) obtained a complete squamous regeneration after argon plasma coagulation (APC) in 98,6% of treated patients. The aim of this prospective study was to evaluate for the first time the effectiveness of APC at a high power setting for the ablation of BE in a multicenter trial. Methods: In 8 study centers, 60 patients (mean age 57, range 27-77; 47 male, 13 female) with endoscopic and histologically proven non-neoplastic BE (length of BE 1-8 cm) were recruited for treatment by APC in combination with esomeprazole. After baseline documentation by video endoscopy (VE) plus chromoendoscopy with 0,5% methylene blue (MB) and four quadrant biopsies (4QB) including the gastroesophageal transition zone, BE was treated by repeated APC using a power setting of 90 W; the number of APC sessions was restricted depending on the length of BE. During the treatment period, all patients received esomeprazole 80 mg daily; 2 weeks after completion of treatment the esomeprazole dose was reduced to 20 mg on demand daily or adapted to the result of 24 h pH monitoring. Endoscopic examinations including VE, chromoendoscopy with MB, and 4QB depending on pretreatment length of BE were performed 3 weeks, 6 and 12 months after completion of treatment. The effect of ablation was classified either as complete remission (CR; complete macroscopic and microscopic regression of BE), partial remission (macroscopic regression more than 50% depending on pretreatment length of BE) or minor response (macroscopic regression of BE less than 50%). Results: 51/60 recruited patients were treated within the study. 3 patients were lost for follow-up (FU) after complications had occurred. In 37 of 48 patients (77%) CR was achieved after a mean FU of 14 months (range 12-32). To achieve complete ablation of BE (mean length 3,6 cm), a mean of 2,6 APC sessions (range 1-5) were used. Transient complications (chest pain, fever) were noted in 9/51 patients (17,6%). Major complications (stricture formation, bleeding, perforation) occurred in 5/51 patients (9,8%). Conclusions: The fact that esophageal cancer incidence rate in non-neoplastic BE is low and the goal of ablation treatment namely complete ablation of BE is not reached in all patients together with the risk for morbidity due to ablation does not justify APC for ablation of non-neoplastic BE.

Aim: To demonstrate that a new oral 2L gut cleansing solution PEG+E plus ascorbate (2L PEG+E) is as safe and well tolerated as the standard 4L PEG+E bowel preparation in risk group patients undergoing colonoscopy.

Die Koloskopie ist eine etablierte Methode zur diagnostischen Sicherung von Erkrankungen des Kolons bzw. zum Tumorscreening, wobei der Einsatz von hochvolumigen Darmvorbereitungslösungen von den Patienten beklagt wird. 4 Liter einer PEG 3350 plus balanzierten Elektrolyten (PEG+E) enthaltenen Darmspüllösung sind der Standard. Eine modifizierte 2 Liter PEG+E Lösung plus Vitamin C wurde entwickelt, um die einzunehmende Menge zu reduzieren. Diese randomisierte, verblindete, multizentrische Vergleichsstudie wurde durchgeführt, um die Wirksamkeit, Akzeptans und Sicherheit der neuen 2 Liter PEG+E Lösung (Moviprep) im Vergleich zur etablierten 4 Liter PEG+E Lösung (Klean-Prep) zu testen. Beide Darmvorbereitungslösungen wurden geteilt eingenommen mit nächtlicher Pause. Der primäre Studienendpunkt war die Kolonreinheit ermittelt via Videoaufzeichnungen. Weitere Endpunkte waren der Reinigungsgrad per Darmabschnitt und Beurteilung der Darmvorbereitung, Geschmacks, Patientenzufriedenheit bzw. -akzeptans aber auch die Sicherheit.

Einleitung: Entgegen vorherigen Erfahrungen mit endoskopischen Verfahren zur Ablation von Barrettschleimhaut beschrieb die Arbeitsgruppe von Schulz et al. (GE 2000) eine 99% komplette Ablation mittels Argon-Plasma-Koagulation (APC). Im Rahmen einer Multicenterstudie sollte nun überprüft werden, ob die vermutlich durch Anwendung einer hohen Energiestufe erreichte Ablationsrate nachvollzogen werden kann.

Aims: De‐differentiated chondrosarcoma is characterized by the presence of two distinct chondroid and nonchondroid tumour portions. The aim of our study was to investigate the distribution of extracellular matrix components in this tumour entity and thus to shed light on its histogenetic origin.Methods and resultsHistochemical and immunohistochemical analyses were performed for collagen subtypes I, II, III and VI and cartilage proteoglycans in three samples of de‐differentiated as well as conventional chondrosarcomas (various grades). In the chondroid tumour areas of de‐differentiated chondrosarcoma, typical cartilage matrix components could be detected similar to chondroid areas of grade 1 and 2 conventional chondrosarcomas. In contrast, the tumour matrix of the nonchondroid portions of de‐differentiated chondrosarcomas contained matrix molecules which are typical for fibroblastic tissue. This matrix composition was not identical with less differentiated (nonchondroid) areas of grade 2 and 3 conventional chondrosarcomas.ConclusionsOur results confirm the chondroid nature of the differentiated portion of de‐differentiated chondrosarcoma and indicate a nonchondrocytic nature of the nonchondroid portion. De‐differentiated chondrosarcoma should not be considered as a ‘de’‐differentiated chondrosarcoma (grade 4 neoplasm), but as a tumour entity showing two types of mesenchymal differentiation.

Some of the clinical features of obstructive sleep apnoea syndrome (OSA) are suggestive of impaired cerebral blood flow. Cerebral blood flow alterations might, for example, be responsible for headaches, which are frequent complaints in patients with OSA. Even the high frequency of ischaemic cerebral complications in patients with OSA might be caused in part by sleep apnoea-associated impairment of cerebral perfusion. Previous studies have demonstrated reduced total cerebral blood flow in patients with OSA, but regional changes of cerebral perfusion have not been studied up to now. We performed SPECT studies using 99mTc-(d,l)-hexamethyl-propylenaminoxim (HMPAO) as a tracer in 14 adult patients with moderate to severe OSA (AHI > 30/h; mean AHI 59.2 +/- 4.3). The injection of the tracer took place between 2:00 and 4:00 a.m. while repeated episodes of obstructive apnoea were detected by polysomnography during stage II sleep. Data acquisition took place at 7:30 a.m. All measurements were repeated some nights later under effective treatment with nCPAP. Visual analysis showed marked frontal hyperperfusion in 5 patients. When regional perfusion indices were calculated for 32 regions of interest statistical analysis showed reduced perfusion of the left parietal region. These changes were completely reversed by effective nCPAP therapy. These data suggest that OSA is associated with reversible changes of regional cerebral perfusion. The underlying pathophysiologic mechanisms are matter of speculation so far. There might be an apnoea-associated effect of local vascular autoregulation mechanisms acting to compensate systemic blood flow alterations or blood gas changes in OSA. The observed frontal hyperperfusion might be caused by activation of the frontal lobe by repetitive cortical arousals.

Chondrocyte differentiation is characterized by distinct cellular phenotypes, which can be identified by specific extracellular matrix gene expression profiles. By applying in situ analysis on the mRNA and protein level in a series of benign and malignant human chondrogenic neoplasms, we were able to identify for the first time different phenotypes of neoplastic chondrocytes in vivo: 1) mature chondrocytes, which synthesized the characteristic cartilaginous extracellular tumor matrix, 2) cells resembling hypertrophic chondrocytes of the fetal growth plate, 3) cells resembling so-called dedifferentiated chondrocytes, and 4) well differentiated chondrocytic cells, which expressed type I collagen, indicating the presence of post-hypertrophic differentiated neoplastic chondrocytes. Chondrocytes exhibiting a range of phenotypes were found to be present in the same neoplasm. The different observed phenotypes, including the dedifferentiated phenotype, were in contrast to the anaplastic cells of high-grade chondrosarcomas. Comparison of expression data with tumor morphology revealed a relationship between the cellular phenotypes, the tumor matrix composition, and the matrix and cell morphology within the neoplasms. The distinctly different phenotypes of neoplastic chondrocytes are the basis of the characteristic high biochemical and morphological heterogeneity of chondroid neoplasms and shed light on their biological and clinical behavior.

Clear cell chondrosarcoma is a rare mesenchymal neoplasm of unclear differentiation. Besides having a chondrogenic nature, an osteogenic differentiation was also proposed. In this study, expression analysis of extracellular matrix genes, which are specific for different mesenchymal cell differentiation pathways, were used to get a better understanding of origin and differentiation pattern of the clear cell chondrosarcoma tumor cells. Our in situ analysis of two cases shows that (1) chondrocytic cell differentiation as marked by the expression of cartilage collagen type II and proteoglycans is a characteristic feature within the development of the neoplasm, (2) multifocal chondrocyte hypertrophy as shown by the expression of type X collagen does occur, and (3) no significant expression of collagen type I, the main gene product of osteoblastic cells, is found by the neoplastic cells. Thus, our study indicates that clear cell chondrosarcoma shows a chondrogenic, but not osteogenic, differentiation and represents a true chondrosarcoma. The unusual scarcity of its extracellular and the multifocal expression of type X collagen marks clear cell chondrosarcoma as a chondrosarcoma tumor entity of a particular cell differentiation pattern. The expression of cartilage type collagens represents a distinct marker from bone metastases of clear cell neoplasms of other origins.

The introduction of laparoscopic cholecystectomy appeared to be associated in many institutions with an increased incidence of biliary injury. Therefore, over a period of one year we prospectively collected the data of all patients who were referred to our unit with a suspected bile duct problem after cholecystectomy. Between August 1992 and August 1993, 32 patients with endoscopically identified problems were included in the study. Twenty-three out of 32 had early complications (occurred within the first 30 days after cholecystectomy). Thirteen out of these 23 patients showed a bile leakage, 7 retained stones and 2 duct strictures and in 1 patient the hepatic duct was clipped. Only 10/23 early bile duct problems occurred after laparoscopic cholecystectomy. 19/23,patients (82%) were treated successfully on the endoscopic route. All 11 patients with late complications had strictures associated in 7 cases with stones. All late complications appeared after open surgery. Endoscopic or percutaneous therapy was successful in 7/11 patients (63%). With the exception of one minor bleeding, no major complication occurred. Summing up, endoscopic therapy is the therapy of choice in the case of bile duct problems after cholecystectomy with a high success rate and low complication rate. To date, in our area there are no clear indications that bile duct problems after laparoscopic surgery are more frequent than after open cholecystectomy.

A 24-year-old patient presented with severe back pain in the lumbar and sacral regions and septic temperatures up to 40 degrees C. Lasègue's sign was bilaterally positive. An enhanced cell count was seen in the CSF (1877 cells/microliters, erythrocytes 1024/microliters). Total protein concentration was 4440 mg/dl. The patient also suffered from granulocytic pleocytosis. X-ray as well as scintigrams revealed inflammation of the presacral soft parts involving the os sacrum and bone marrow. Treatment with doxycycline (200 mg/d), fosfomycin (5 g/d), netilmicin (400 mg/d) and ofloxacin (200 mg/d) succeeded in curing the meningitis, whereas the presacral phlegmon persisted. Endoscopic examination showed a rod-shaped foreign body in the rectum that had penetrated deeply into the mucosa and required deformation and removal by laser. The plastic rod of 10 cm length was probably a liquorice stick. The patient denied introducing it per anum; he suspected having swallowed it while drunk. The inflammation subsided rapidly after removal of the foreign body.

A 24-year-old patient presented with severe back pain in the lumbar and sacral regions and septic temperatures up to 40-degrees-C. Lasegue's sign was bilaterally positive. An enhanced cell count was seen in the CSF (1877 cells/mul, erythrocytes 1024/mul). Total protein concentration was 4440 mg/dl. The patient also suffered from granulocytic pleocytosis. X-ray as well as scintigrams revealed inflammation of the presacral soft parts involving the os sacrum and bone marrow. Treatment with doxycyclin (200 mg/d), fosfomycin (5 g/d), netilmicin (400 mg/d) and ofloxacin (200 mg/d) succeeded in curing the meningitis, whereas the presacral phlegmon persisted. Endoscopic examination showed a rod-shaped foreign body in the rectum that had penetrated deeply into the mucosa and required deformation and removal by laser. The plastic rod of 10 cm length was probably a liquorice stick. The patient denied introducing it per anum; he suspected having swallowed it while drunk. The inflammation subsided rapidly after removal of the foreign body.

See also Acute Infective Colitis Caused by Endemic Pathogens in Western Europe: Endoscopic Features