Long-term experience with setup and implementation of an IHE-based image management and distribution system in intersectoral clinical routine.

PURPOSE: Sharing of medical data is crucial for the proper treatment of patients as it could reduce the risk of duplicated medical tests and speed up the care process if all documents are readily available. Despite great technical progress, sharing patient data while maintaining full control over the process in an intersectoral (in Germany, this describes the different actors in the healthcare system consisting of clinic, ambulatory care, etc.) setting remains a particular challenge. This paper focuses on the successful implementation of a privacy compliant, standards-based image-management component of a personal electronic health record. METHODS: Over a 5-year period, a sharing system based on readily available IHE profiles constructed around XDS has been built. It was necessary to create interfaces for the existing hospital sub-systems to become part of the network. Specifically, the imaging workflow had to be adapted to allow for fast and easy access to DICOM images utilizing a flexible web-based image viewer. In addition to the standard XDS workflow, an Imaging Cache was established which combines the Imaging Document Source and Consumer to guarantee fast and streaming-based access to all images in the network observing the high security standards of the hospital network. RESULTS: The authors of this paper have proven that it is possible to build a fast and reliable sharing system based on IHE profiles using most of the transactions of XDS-I with some adaptions to the clinical workflow. Primary hospital systems were enabled by building adapters to overcome lack of IHE compatibility. The established system embraces the existing security mechanisms in hospital networks while connecting patients and referring physicians from outside in a secure and convenient manner. CONCLUSIONS: A state-of-the-art sharing system that is used in a productive clinical environment has been established and is ready to grow with more partners. The system is the basis for an elaborated interdisciplinary collaboration where data, and in particular images, can now be shared between medical professionals.

[Safety aspects of parenteral iron supplementation therapies in patients with chronic kidney disease]. / Sicherheitsaspekte parenteraler Eisentherapien bei Patienten mit chronischer Niereninsuffizienz.

Iron deficiency often occurs in patients with chronic kidney disease and can be effectively treated with parenteral supplementation of iron. In these patients, prompt application of iron therapy can help to reduce the dependence of erythropoietin-stimulating agents and effectively treat anemia. Correct evaluation of iron metabolism in CKD patients can be difficult. Duration of and response to therapy should always be considered while planning parenteral supplementation of iron. The main safety aspects of parenteral iron preparations relate to their possible anaphylactic potential and the potential induction of oxidative stress due to the release of free iron. However, parenteral iron supplementation is usually safe and without major side effects. Regarding current data, none of the iron preparations is showing definitive superiority. Although uncommon, iron preparations containing dextran can lead to severe side effects, therefore these preparations appear to have an inferior safety profile. Due to limited data, a comparison of third-generation iron preparations with previous preparations is not possible. Recently, for the first time, the third generation iron preparation ferumoxytol has been directly compared to iron sucrose. From this data and others, it remains unclear whether third generation iron preparations show safety-relevant superiority.

Resonantly-enhanced harmonic generation in Argon.

We present systematic investigations of harmonic generation in Argon, driven in the vicinity of a five-photon resonance by intense, tunable picosecond radiation pulses. When properly matching the laser frequency with the Stark-shifted multi-photon resonance, we observe a pronounced enhancement not only of the 5th, but also the 7th and 9th harmonic of the driving laser (i.e. at orders higher than the involved multi-photon resonance). We study the harmonic yield at different intensities and wavelengths of the driving laser to determine optimal conditions for resonantly-enhanced harmonic generation.

Extreme hyponatremia in an infant with congenital adrenal hypoplasia due to a novel NR0B1 (DAX-1) mutation.

BACKGROUND: Extreme hyponatremia (<105 mmol/l) has rarely been reported in infants. It is potentially life-threatening and requires intensive care treatment. PATIENT: We report on a male infant with absence of weight gain from birth to day 33 of life despite adequate nutrition. On admission serum sodium and potassium were 104 and 5.9 mmol/L respectively. The infant's physical status revealed dehydration, but normal activity with no apparent neurological, circulatory or respiratory impairment. MAIN RESULTS: Global adrenocortical insufficiency was diagnosed and treated with hormonal substitution in addition to intravenous application of fluid, glucose and electrolytes. The rise of serum sodium was carefully monitored and adjusted to a target rate of 0.5 mmol/L/h. X-linked adrenal hypoplasia congenita (X-AHC) was confirmed by the identification of a novel nonsense NR0B1 (DAX-1) mutation (W236X). CONCLUSIONS: X-AHC in infants may present as failure to thrive despite adequate nutrition. Extreme hyponatremia may be associated with little symptoms if developing slowly. Rehydration and slow correction of serum sodium with solutions containing less sodium than normal saline is essential.

The integration of medical images with the electronic patient record and their web-based distribution.

Medical images are currently created digitally and stored in the radiology department's picture archiving and communication system. Reports are usually stored in the electronic patient record of other information systems, such as the radiology information system (RIS) and the hospital information system (HIS). But high-quality services can only be provided if electronic patient record data is integrated with digital images in picture archiving and communication systems. Clinicians should be able to access both systems' data in an integrated and consistent way as part of their regular working environment, whether HIS or RIS. Also, this system should allow for teleconferencing with other users, eg, for consultation with a specialist in the radiology department. This article describes a web-based solution that integrates the digital images of picture archiving and communication systems with electronic patient record/HIS/RIS data and has built-in teleconferencing functionality. This integration has been successfully tested using three different commercial RIS and HIS products.

Fast-painting of human metaphase spreads using a chromosome-specific, repeat-depleted DNA library probe.

For chromosome painting, in situ suppression of repetitive DNA sequences has been well established. Such standard protocols usually require large amounts of Cot-I DNA. Recently, it has become possible to deplete repetitive DNA sequences from library probes by magnetic purification and PCR-assisted affinity chromatography. These "repeat-depleted library probes" appear to be extremely useful for Fast-FISH, a technique that omits denaturing chemical agents such as formamide in the hybridization buffer, resulting in a substantial acceleration and simplification of the complete protocol. Shown here is the application of Fast-FISH to a repeat-depleted, directly fluorochrome-labeled library probe of the q-arm of chromosome 15 (Fast-Painting) for human lymphocyte metaphase spreads. Following painting without Cot-I DNA and without formamide, visual inspection revealed sufficient chromosome painting after a few hours of hybridization. The fluorescence signals of the labeling sites were analyzed after hybridization times of 1 and 2 h (in one case, 4 h) using digital fluorescence microscopy. The painting efficiency expressed in values of relative fluorescence signal ratios was quantitatively evaluated by image analysis using line-scan procedures and area-morphometry of mean luminance. Two preparation protocols (ethanol dehydration without and with RNase A treatment followed by pepsin digestion for four different exposure times) were compared. These results indicated that RNase A treatment and pepsin digestion are steps that can be omitted.

[When children and adolescents refuse to go to school--school refusal from the viewpoint of a public health office]. / Wenn Kinder und Jugendliche nicht zur Schule wollen--Schulverweigerung aus der Sicht eines Gesundheitsamtes.

What should happen if children or adolescents refuse school? This may be due to phenomena such as school phobia, fear of school, playing truant, or a learning strike. The article demonstrates how an interdisciplinary team guided by the health authorities handles the problem of school refusal in the multifactorial field of tension of family, school and psychosocial dynamics. The present paper includes an analysis of school-related and sociological causes, a description of the legislative framework, an outline of possible treatments, as well as a discussion on aspects of prevention.

Elevated serum activity of N-acetyl-beta-glucosaminidase in essential hypertension: diagnostic value and reversal to normal values after antihypertensive therapy.

Previous studies have shown that urinary N-acetyl-beta-glucosaminidase (NAG) is elevated in patients with hypertension, even without renal disease. To elucidate the value of measuring NAG, both in urine and serum of hypertensive patients, we measured NAG activity in the serum, plasma, and 24-hour urine by the fluorimetric method in 84 patients with uncomplicated essential hypertension before and after 6 months of effective treatment. NAG activities of these hypertensive patients were compared with those of 102 healthy normotensive subjects and 97 patients with various renal diseases and controlled hypertension. Serum NAG activity was clearly greater in patients with essential hypertension (427 +/- 124 U/mL) than in normotensive subjects (380 +/- 109 U/mL) or patients with renal disorders (393 +/- 115 U/mL) (P less than or equal to 0.004). The greater was the diastolic pressure in the hypertensive group, the greater was serum NAG activity (r = +0.30, P = 0.004). Hypertensive patients with high serum NAG activity were further characterized by a more exaggerated increase in systolic pressure (34 +/- 16 v 25 +/- 15 mm Hg, P = 0.051) and total peripheral resistance (19% +/- 18% v 12% +/- 13%, P = 0.042) in response to the cold pressor test and by a greater increase in systolic pressure (56 +/- 15 v 45 +/- 13 mm Hg, P = 0.009) and diastolic pressure (11 +/- 7 v 6 +/- 9, P = 0.043) in response to bicycle exercise testing than the group with low serum NAG activity. In contrast, urinary NAG activity tended to be only slightly higher in patients with essential hypertension than in the normotensive control group (33 +/- 31 v 23 +/- 29 U/mg creatinine [cr], P = 0.062), whereas patients with renal diseases had clearly increased urinary NAG activity (87 +/- 105 U/mg cr) (P less than 0.001). Following effective antihypertensive therapy, serum NAG activity decreased in patients with essential hypertension to values of normotensive control subjects (from 427 +/- 124 U/mL to 386 +/- 106 U/mL, P less than 0.01). A significant decrease in serum NAG activity was observed in patients with both initially high as well as low pretreatment serum NAG activities (P less than 0.001 and P less than 0.02, respectively). Urinary NAG activity overall was unchanged by antihypertensive treatment. We conclude that in patients with mild essential hypertension, serum NAG activity was already elevated (whereas urinary NAG activity was not) and was normalized by effective antihypertensive treatment.(ABSTRACT TRUNCATED AT 400 WORDS)

[Scintigraphic studies of gonadal burden in radiosynoviortheses of the knee joint with yttrium 90]. / Szintigraphische Untersuchungen zur Gonadenbelastung bei Radiosynoviorthesen des Kniegelenkes mit Yttrium-90.

Yttrium-90 radiosynoviorthesis of the knee joint in rheumatic inflammatory diseases shows favourable results. Because of the supposed exposure of the patients gonads, Yttrium synoviorthesis has rarely been performed in patients below the age of 40. Scintigraphic studies in 20 patients with rheumatoid arthritis showed no significant leakage of the radionuclide from the knee joint. Gamma camera measurements both phantom and in vivo in the regions of ovary and testes revealed a local dose of 1.05 microGy/MBq (3.9 mrd/mCi) or 1.1 microGy/MBq (4.1 mrd/mCi) of the injected radioactivity. Calculation of the maximum possible load of the gonads, supposing even total leakage of the injected 90-Yttrium from the knee joint, yielded a maximum possible dose of 6 mGy (600 mrd). We conclude from these studies and calculations that Yttrium-90 synoviorthesis of the knee joint can safely be carried out in patients below the age of 40.

Clinical features of inflammatory joint and spine manifestations in Crohn's disease.

167 patients with Crohn's disease were investigated for joint and spine inflammation. Arthritis was observed in 23 patients (14%), sacroiliitis in 24 (14%), and sacroiliitis in combination with arthritis in 11 patients (7%). 15 patients (9%) had ankylosing spondylitis; 9 of them were HLA-B27 positive (60%). A parallel pattern in the course of bowel disease and joint inflammation was observed in 22 out of 34 patients with arthritis (59%). An association between the localization of Crohn's disease and the type of spondylarthritis could not be demonstrated. Patients with arthritis alone developed erythema nodosum (35%) or aphthous stomatitis more often (21%) than patients without spondylarthritis+ (6% and 12%, respectively). Other extra-intestinal manifestations of Crohn's disease did not reveal any association with the development of spondyloarthritis.

Alternatives to CYVADIC combination therapy of soft tissue sarcomas.

The CYVADIC combination has been the preferred treatment for soft tissue sarcomas for the last 10 years. Other combination therapies are necessary, because the remission rate achieved with CYVADIC is only 30%. Alternative therapies for these tumors are combinations including cis-platinum, ifosfamide, epipodophyllin, and high-dose methotrexate. Our therapeutic results with combinations of cis-platinum and ifosfamide are comparable to those achieved with CYVADIC. However, the side-effects, such as nausea, vomiting and fatigue, of cis-platinum used in the palliative treatment of these tumors are intolerable for many patients. A combination of adriamycin and ifosfamide, which leads to a higher remission rate of 44% and has lower toxicity than CYVADIC, is giving encouraging results.

Alternatives to CYVADIC-combination therapy of soft tissue sarcomas.

The CYVADIC combination has been the preferred treatment for soft tissue sarcomas for the last 10 years. Other combinations of therapy are necessary because the remission rate achieved with CYVADIC is only thirty per cent. Alternative therapies for these tumours are combinations including cis-platinum, ifosfamide, epipodophyllin and high-dose methotrexate. Our therapeutic results with combinations of cis-platinum and ifosfamide are comparable to CYVADIC. However, side-effects such as nausea, vomiting and fatigue due to cis-platinum in the palliative treatment of these tumours are intolerable for many patients. A combination of adriamycin and ifosfamide, which exhibits a higher remission rate of 44% and lower toxicity than CYVADIC, is giving encouraging results.

[HLA haplotype study in familial Crohn disease]. / HLA-Haplotypstudie bei familiärem Morbus Crohn.

Among others, genetical factors have been discussed in the aetio-pathogenesis of inflammatory bowel diseases. This is supported by many studies reporting familial accumulation of Crohn's disease and ulcerative colitis. The use of HLA typing to demonstrate a genetical disposition has so far proved disappointing. Accordingly, if there is any association with definite HLA antigens, it is likely to be weak. We therefore investigated the HLA haplotypes in 13 families with multiple occurrences of Crohn's disease. Comparing observed and expected occurrences of HLA-haplotypes, we found a pronounced tendency towards common haplotypes in the affected siblings, uncles and nieces, and cousins. Although the data suggest a possible genetical disposition, additional factors such as microorganisms or possibly nutritional habits must be considered as causes for Crohn's disease.

[Genetics of Crohn disease: study of the HLA association in 169 patients]. / Zur Genetik des Morbus Crohn: Untersuchung der HLA-Assoziation bei 169 Patienten.

Histocompatibility (HLA) antigen phenotypes have been studied in 169 patients with Crohn's disease. The following results could bei shown: HLA-Aw33, -B45 and -Cw3 showed a positive association and HLA-A26, -DR3 and -DRw8 a negative association with Crohn's disease compared to healthy controls. However, when the p-values were corrected by multiplying them by the number of determined antigens per gen-locus, the differences were not significant. Patients with a late onset of the disease (greater than 25 years) showed a statistical significant negative association with HLA-DR3. Numerous studies revealed no significant association between Crohn's disease and HLA-antigens except Smolen et al. (HLA-B12). The significant association of Crohn's disease and HLA-B12 reported by Smolen et al. could be caused by an increased frequency of HLA-B45 as we found in our patients.

[Systemic manifestations in a case of Churg-Strauss vasculitis with a clinical picture of recurrent cerebral ischemia]. / Systemische Manifestation einer Vaskulitis Churg-Strauss unter dem klinischen Bild einer rezidivierenden zerebralen Ischämie.

The case of a 67-year old male patient with allergic granulomatous angiitis (Churg-Strauss syndrome) is reported. In the case report the typical symptoms of Churg-Strauss syndrome, the therapeutic possibilities (immunosuppression, plasmapheresis) and the differential diagnosis are discussed.