[Hydrocolonic sonography--potentials and limitations of ultrasonographic diagnosis of colon diseases]. / Hydrokolonsonographie - Möglichkeiten und Grenzen einer sonographischen Diagnostik von Dickdarmerkrankungen.

Hydrocolonic sonography - potentials and limitations of ultrasonographic diagnosis of colon diseases. Ultrasonic examination has become increasingly important in the diagnosis of diseases of the gastrointestinal tract. In neoplastic diseases of the large intestine, however, the diagnostic value of conventional transabdominal sonography is limited. The sonographic evaluation of the colon could be improved by the retrograde instillation of water into the colon in a procedure called hydrocolonic sonography. This technique allows the visualization of the colon sonographically from the rectosigmoid transition to the cecum. Hydrocolonic sonography enables a detailed sonographic examination of the hypoechoic colonic lumen, the five individual layers of the colon wall and the surrounding connective tissue, thus providing additional information and allowing a more precise diagnosis of many diseases of the colon. Colonic polyps and carcinomas appear sonographically as echogenic structures projecting from the intestinal wall into the lumen. Colonic polyps larger than 7 mm in diameter and carcinomas of the colon can be detected with great sensitivity. In addition, detailed evaluation of the structure of the bowel wall permits more precise staging of tumors of the colon. Aside from such localized changes, hydrocolonic sonography can also demonstrate changes in the wall structure in chronic inflammatory large bowel diseases such as Crohn's disease and ulcerative colitis. In addition hydrocolonic sonography has been proven to be of great value for the ultrasonographically-guided hydrostatic reduction of intussusception in children. On the other hand virtual colonoscopy is another promising new imaging method for the diagnosis of colon diseases. The differences between hydrcolonic sonography and virtual colonoscopy and their potential for screening purposes of colon cancer will be discussed.

Vascular endothelial growth factor receptor-2 and neuropilin-1 form a receptor complex that is responsible for the differential signaling potency of VEGF(165) and VEGF(121).

The two most abundant secreted isoforms of vascular endothelial growth factor A (VEGF(165) and VEGF(121)) are formed as a result of differential splicing of the VEGF-A gene. VEGF(165) and VEGF(121) share similar affinities at the isolated VEGF receptor (VEGFR)-2 but have been previously demonstrated to have differential ability to activate VEGFR-2-mediated effects on endothelial cells. Herein we investigate whether the recently described VEGF(165) isoform-specific receptor neuropilin-1 (Npn-1) is responsible for the difference in potency observed for these ligands. We demonstrate that although VEGFR-2 and Npn-1 form a complex, this complex does not result in an increase in VEGF(165) binding affinity. Therefore, the differential activity of VEGF(165) and VEGF(121) cannot be explained by a differential binding affinity for the complex. Using an antagonist that competes for VEGF(165) binding at the VEGFR-2.Npn-1 complex, we observe specific antagonism of VEGF(165)-meditated phosphorylation of VEGFR-2 without affecting the VEGF(121) response. These data indicate that the formation of the complex is responsible for the increased potency of VEGF(165) versus VEGF(121). Taken together, these data suggest a receptor-clustering role for Npn-1, as opposed to Npn-1 behaving as an affinity-converting subunit.

Lanceolate hair-J (lahJ): a mouse model for human hair disorders.

Lanceolate hair-J (lahJ) arose spontaneously in 1994 on the DBA/1LacJ inbred background at The Jackson Laboratory. Mutant mice were runted, alopecic, and lacked vibrissae. As they aged, their skin wrinkled. Affected mice developed a noninflammatory, proliferative skin disease with follicular dystrophy. Hair fibers developed a number of abnormalities including periodic nodules along the shaft (trichorrhexis nodosa), compaction resembling trichorrhexis invaginata, spiral fractures, broken tips, and lance-shaped tips. This mutation exhibits some characteristics that resemble an autosomal recessive ichthyosiform disease that occurs in humans characterized in part by peculiar, invaginating, multinodal, hair shaft abnormalities known as Netherton's syndrome. Periodic nodules also resemble the human genetic based disease monilethrix. This autosomal recessive mouse mutation, allelic with lanceolate hair (lah), based on breeding studies, is located on mouse Chromosome 18, within a cluster of genes coding for adhesion molecules. Homozygotes for either of these allelic mouse mutations have elevated serum IgE levels, a feature also common with human Netherton's syndrome.

Sex difference in the validity of vertebral deformities as an index of prevalent vertebral osteoporotic fractures: a population survey of older men and women.

Morphometric methods have been developed for standardized assessment of vertebral deformities in clinical and epidemiologic studies of spinal osteoporosis. However, vertebral deformity may be caused by a variety of other conditions. To examine the validity of morphometrically assessed vertebral deformities as an index of osteoporotic vertebral fractures, we developed an algorithm for radiological differential classification (RDC) based on a combination of quantitative and qualitative assessment of lateral spinal radiographs. Radiographs were obtained in a population of 50- to 80-year-old German women (n = 283) and men (n = 297) surveyed in the context of the European Vertebral Osteoporosis Study (EVOS). Morphometric methods (Eastell 3 SD and 4 SD criteria, McCloskey) were validated against RDC and against bone mineral density (BMD) at the femur and the lumbar spine. According to RDC 36 persons (6.2%) had at least one osteoporotic vertebral fracture; among 516 (88.9%) nonosteoporotics 154 had severe spondylosis, 132 had other spinal disease and 219 had normal findings; 14 persons (2.4%) could not be unequivocally classified. The prevalence of morphometrically assessed vertebral deformities ranged from 7.3% to 19.2% in women and from 3.5% to 16.6% in men, depending on the stringency of the morphometric criteria. The agreement between RDC and morphometric methods was poor. In men, 62-86% of cases with vertebral deformities were classified as nonosteoporotic (severe spondylosis or other spinal disease) by RDC, compared with 31-68% in women. Among these, most had wedge deformities of the thoracic spine. On the other hand, up to 80% of osteoporotic vertebral fractures in men and up to 48% in women were missed by morphometry, in particular endplate fractures at the lumbar spine. In the group with osteoporotic vertebral fractures by RDC the proportion of persons with osteoporosis according to the WHO criteria (T-score < -2.5 SD) was 90.0% in women and 86.6% in men, compared with 67.9-85.0% in women and 20.8-50.0% in men with vertebral deformities by various methods. Although vertebral deformities by most definitions were significantly and inversely related to BMD as a continuous variable in both sexes [OR; 95% CI ranged between (1.70; 1.07-2.70) and (3.69; 1.33-10.25)], a much stronger association existed between BMD and osteoporotic fractures defined by RDC [OR; 95% CI between (4.85; 2.30-10.24) and (15.40; 4.65-51.02)]. In the nonosteoporotic group individuals with severe spondylosis had significantly higher BMD values at the femoral neck (p < 0.01) and lumbar spine (p < 0.0004) compared with the normal group. On the basis of internal (RDC) and external (BMD) validation, we conclude that assessment of vertebral osteoporotic fracture by quantitative methods alone will result in considerable misclassification, especially in men. Criteria for differential diagnosis as used within RDC can be helpful for a standardized subclassification of vertebral deformities in studies of spinal osteoporosis.

[Diagnosis of chronic inflammatory bowel disease by ultrasonography]. / Diagnostik von chronisch-entzündlichen Darmerkrankungen durch Sonographie.

Abdominal sonography as a noninvasive diagnostic method has proven to be of great value in evaluating patients with chronic inflammatory bowel diseases. The ultrasound pattern of bowel inflammation is characterized by a thickened and hypoechoic inflamed bowel wall, which appears like a target on transverse scans. Both Crohn's disease and ulcerative colitis can be detected with great sensitivity by abdominal ultrasonography. Ultrasonography (u.s.) reveals the intra- and extramural spread of the inflammation. U.s. is suitable method for the detection of abdominal complications, such as fistulas, abscesses and strictures, especially in patients with Crohn's disease. Duplex and color Doppler ultrasonography are helpful in the detection of the splanchnic hemodynamic changes in patients with intestinal inflammation. This offers a noninvasive and indirect means of assessing disease activity. Both endorectal ultrasonography and MRT are the most sensitive methods to detect fistulas and abscesses in patients with Crohn's disease. Because of the diagnostic possibilities, the unstressful nature of the procedure and the general acceptance by the patients, next to endoscopy, ultrasonography is the most important diagnostic procedure both as a primary diagnostic imaging modality and for the follow-up of patients with inflammatory bowel diseases.

Severe osteoporosis in familial Hajdu-Cheney syndrome: progression of acro-osteolysis and osteoporosis during long-term follow-up.

Hajdu-Cheney syndrome is an autosomal dominant inherited osteodysplastic bone disease with the hallmarks of acro-osteolysis, skull deformations, and generalized osteoporosis. Very few patients have been followed long-term with respect to the prognosis of acro-osteolysis and osteoporosis. Here we describe a 39-year-old woman and her 19-year-old daughter who are both affected with the Hajdu-Cheney syndrome. Skeletal lesions were followed in the mother between the ages of 22 and 39 years. The acro-osteolytic lesions progressed markedly and caused shortening of several fingers; some end phalanges had completely disappeared. Severe spinal osteoporosis with serial vertebral fractures was found at the age of 22 years. New vertebral fractures developed until the age of 33 years, but did not progress afterward. High turnover osteoporosis was found in the bone histology of iliac crest biopsies performed at the ages of 22 and 34 years. Bone mineral content (BMC) was strikingly decreased at the age of 34 years (T score -5.1 SD) and did not significantly change during further follow-up. In the daughter, BMC failed to increase between the ages of 12 and 19 years and was also markedly decreased (T score -4.4 SD). This suggests that osteoporosis in Hajdu-Cheney syndrome is related to a low peak bone mass and a high bone turnover, leading to insufficient bone formation compared with the increased bone resorption.

Clinical stable systemic mastocytosis with interferon alpha-2b therapy.

Systemic mastocytosis (SM) with progressive infiltration of various organs by mast cells is a rare disease with a mean survival of 2 years. So far there is no established therapy. We describe a patient with progressive mastocytosis who had a clinical response to interferon alpha-2b 3 x 5 Mio U s.c. three times a week. The response is stable 3 years after initiation of therapy and includes reduction of abdominal lymphadenopathy, ascites, decrease of serum and urinary histamine levels as well as pulmonary reticulonodular markings.

[Coincidence of primary sclerosing cholangitis and sarcoidosis--case report and review of the literature]. / Koinzidenz von primär sklerosierender Cholangitis und Sarkoidose--Fallbericht und Literaturübersicht.

Primary sclerosing cholangitis (PSC) and sarcoidosis are diseases of unknown etiology which are rarely associated. Here we report the case of a 31-year-old man with PSC and pulmonary sarcoidosis. In addition, we include a brief review on PSC and sarcoidosis, in particular with respect to etiopathogenesis and diagnosis.

[Ultrasound guided puncture of the portal vein in transjugular intrahepatic portasystemic stent shunt (TIPSS)]. / Sonographisch gesteuerte Punktion der Pfortader beim transjugulären intrahepatischen portosystemischen Stentshunt (TIPSS).

Successful completion of a transjugular intrahepatic portosystemic stentshunt (TIPSS) relies on knowledge of the individual hepatic vascular anatomy. The patients referred for TIPSS have an increased risk of contrast-medium-induced renal failure, and therefore the potential for a complete angiographic work-up during the procedure is limited. The same patient population also carries an increased risk of bleeding, which necessitates a rather accurate guiding technique for portal punctures. We have established transcutaneous sonographic guidance as a standard technique for transjugular portal puncture, reducing complication rates and intervention time. Competing imaging modalities for guidance are discussed.

Diagnosis and differential diagnosis of ulcerative colitis and Crohn's disease by hydrocolonic sonography.

OBJECTIVES: The entire length of the colon, starting at the rectosigmoid junction and ending at the cecum, can be visualized by transabdominal sonography after retrograde water instillation into the colon. By this method, termed hydrocolonic sonography, it is possible to evaluate in detail the lumen, the colon wall, and the surrounding tissue. Five layers of different echogenicity can be differentiated within the colon wall. METHODS: In a prospective study of 440 patients, we compared the value of conventional abdominal sonography and hydrocolonic sonography with that of colonoscopy, in the diagnosis and differential diagnosis of ulcerative colitis and colonic Crohn's disease. RESULTS: In 93% of patients with Crohn's disease, the normal five-layer structure of the colonic wall was no longer in evidence, and the wall appeared hypoechogenic and clearly thickened. In contrast, in patients with ulcerative colitis, the five-layer structure could clearly be discerned, and although the colon wall remained hypoechogenic, it was only moderately thickened. Colonic Crohn's disease and ulcerative colitis were detectable by hydrocolonic sonography, with a sensitivity of 96% and 91%, respectively, whereas the sensitivity achieved by conventional abdominal sonography was only 71% and 62%, respectively. Furthermore, hydrocolonic sonography made possible the differentiation of Crohn's disease from ulcerative colitis in 93% of the cases. CONCLUSION: This new diagnostic procedure could thus advantageously be introduced for the diagnosis, differential diagnosis, and follow-up of patients with chronic inflammatory large bowel diseases.

[Parapneumonic ARDS. The radiomorphologic transition of a pneumonia into an ARDS]. / Das parapneumonische ARDS. Der radiomorphologische Ubergang einer Pneumonie in ein ARDS.

Serial chest X-rays of 14 patients with parapneumonic ARDS (PARDS) were analysed retrospectively. Typical findings on chest X-ray films occurred after a latency period of 12-24 h following the clinical start of PARDS. We found some "early signs" of PARDS, such as central interstitial paravascular edema (14/14), bilateral hilar infiltrates (9/14) and the absence of pleural effusion (12/14).

[The diagnosis and differential diagnosis of Crohn's disease and ulcerative colitis by hydrocolonic sonography]. / Diagnose und Differentialdiagnose von Morbus Crohn und Colitis ulcerosa durch Hydrokolonsonographie.

As part of a prospective study the value of hydrocolonic sonography in the diagnosis and differential diagnosis of Crohn's disease (CD) and ulcerative colitis (UC) was assessed and compared with that of coloscopy as standard reference. Of 440 patients who were examined, 41 (23 men, 18 women; mean age 25 +/- 6 years) had CD and 36 (20 men, 16 women; mean age 32 +/- 8 years) had UC. CD and UC were correctly diagnosed by hydrocolonic sonography in 39 and 33 cases, respectively (sensitivity 95 and 91%, specificity 100 and 99%, respectively). Among healthy subjects five layers of variable echo density were demonstrated in the intestinal wall, while in all those with CD the wall was clearly thickened and in 36 of them the wall structure was also abnormal. In 16 patients the wall structure was homogeneous without evidence of layers and in 20, layers 1-3 were not demonstrable and layers 4 and 5 only rudimentary. In contrast, the 33 patients with UC diagnosed by hydrocolonic sonography had only slight thickening of the intestinal wall and all layers were present in 30 of them. CD and UC were correctly distinguished in 91%. Hydrocolonic sonography makes it possible to demonstrate in detail the structural changes in the intestinal wall in CD and UC so that the two conditions can be distinguished.

[Diagnosis of colonic tumors and chronic inflammatory colonic diseases by hydrocolonic sonography]. / Diagnose von Dickdarmtumoren und chronisch entzündlichen Dickdarmerkrankungen durch Hydrokolonsonographie.

Retrograde instillation of water into the colon makes it possible to visualize the colon sonographically from the rectosigmoid transition to the cecum in 97% of the patients examined. The sonographic views obtained using this technique, which is called hydrocolonic sonography, show the echo-free intestinal lumen, the five individual layers of the colon wall and the connective tissue surrounding the colon. Colonic polyps and carcinomas appear sonographically as echogenic structures projecting from the intestinal wall into the lumen. Colonic polyps larger than 7 mm diameter can be identified in 91% of cases, and the sensitivity of the technique in the detection of carcinomas of the colon is 97%. In addition, detailed evaluation of the structure of the bowel wall permits more precise staging of tumors of the colon. Aside from such localized changes, hydrocolonic sonography can also demonstrate typical changes in the wall structure in chronic inflammatory large-bowel diseases. In acute Crohn's disease the normal stratified appearance of the colonic wall is no longer in evidence and the wall appears visible thickened. In contrast, in patients with acute ulcerative colitis the normal sonographic stratified appearance of the colonic wall is maintained. Hydrocolonic sonography enables a detailed sonographic examination of the colonic lumen and the colon wall, thus providing additional information and allowing more precise diagnosis of many diseases of the colon.

[Influence of sonography on indications for laparoscopic cholecystectomy]. / Der Einfluss der Sonographie auf die Indikation zur laparoskopichen Cholecystektomie.

Because of therapeutic consequences preoperative sonography should be performed with a check list. Nevertheless false positive and false negative findings result in cases of gall-stones in the ductus cysticus, of acute cholecystitis and thickness of the gall-bladder wall. Therefore where sonographic findings differ from the intraoperative laparoscopic situs the operating surgeon should change to conventional cholecystectomy early enough to avoid complications.