[Mediastinal space-occupying lesion as a rare cause of atrial arrhythmia]. / Mediastinale Raumforderung als seltene Ursache atrialer Rhythmusstörungen.

HISTORY AND CLINICAL FINDINGS: A 61 year old man presented with newly developed tachyarrhythmia. Echocardiographically a tumour of unclear origin in the left atrium was diagnosed. INVESTIGATIONS: The heart rate in the ECG was arrhythmic at 146/min; intermittently sinus rhythm was established. Transoesophageal echocardiography revealed a 90 x 50 mm tumour with sharp borders displacing both atria. The chest x-ray showed a mediastinal rounded mass in the dorsal part overlapping the right mediastinal border. Computer tomography was unable to discriminate the atrial wall from the tumour masses; hence the difference between an (intra-)cardiac tumour (i.e. myxoma) and a tumour of extracardiac (mediastinal) origin could not be established. Additional magnetic resonance tomography led to the most probable diagnosis of a bronchogenic cyst, which spread ventrally up to the ascending aorta and dorsally to the paravertebral region, thus displacing and shrinking the left pulmonary artery and the left atrium. THERAPY: For certain exclusion of malignancy and because of displacement of other thoracic structures the patient was operated on this mediastinal mass by anterolateral thoracotomy. Histologically a dysontogenetic, benign cyst with an inner layer of mesothel and nerval as well as vascular structures in the capsule was diagnosed. Since the operation the patient has persistent sinus rhythm without symptoms of relevant rhythm disorders. An overview of different kinds of intrathoracical cysts is given.

[Percutaneous reconstruction of a pelvic vascular occlusion using the "kissing-balloon" technique]. / Perkutane Rekonstruktion eines Beckengefässverschlusses in "kissing-balloon"-Technik.

UNLABELLED: A 43-year-old patient was admitted for severe claudication in his right leg, doppler ultrasound and angiography revealed an occluded right common iliac artery. Using the cross-over-approach from the contralateral side, it was possible to navigate a guide wire through the occlusion, and after passage with an excimer laser an ipsilateral retrograde puncture with placement of a sheath was performed. In order to protect the the aortoiliac bifurcation, a primary stent implantation of the occluded side was carried out using the kissing balloon technique. Afterwards three stents were placed. Subsequently the patient completely improved; treadmill exercise test and duplex sonography showed good results. CONCLUSION: Using the kissing balloon technique, laser-assisted recanalisation of iliac artery occlusions even involving the aortoiliac bifurcation compare favorable to other more invasive treatments. A surgical procedure can thus be avoided.

Coexisting hyperparathyroidism, renal osteodystrophy and psoriatic arthritis.

We report the case of a 60-year-old woman with hyperparathyroidism, renal osteodystrophy and psoriatic arthritis. The coexistence of findings of hyperparathyroidism and renal osteodystrophy has been described and there are also reports of patients suffering from renal arthropathy mimicking hyperparathyroidism. To our knowledge, there is no description to date of a case displaying findings of the co-occurrence of these conditions in a patient. We would like to emphasize that attention should be paid to the possible diagnosis of a coexisting inflammatory rheumatic disease when rheumatological symptoms of recent onset occur in patients with long-standing renal osteodystrophy and/or symptoms mimicking hyperparathyroidism occur in these patients.

Sternocostoclavicular hyperostosis: its progression and radiological features. A study of 12 cases.

Twelve cases of sternocostoclavicular hyperostosis were followed up over four to 16 years. The patients underwent repeated radiological examinations of the sternocostoclavicular joints and the sternum, and the extrasternal osseous manifestations of the disease were studied to show changes in the radiological features during long term follow up. Five of 12 (41%) patients had extrasternal manifestations. With the exception of one patient extrasternal manifestations were first detected by scintigraphs because they were asymptomatic. With respect to the sternal manifestations the initial radiological diagnosis was made during an acute phase while painful swelling over the sternum and decreased mobility of the shoulders occurred. The radiological examinations showed the signs of a proliferative destructive arthritis in most patients. In contrast with the frequent occurrences of clinical symptoms, the radiological signs of progression take several years to become detectable. There are no specific bacteriological, serological or histological findings. Usually a permanent increase in the erythrocyte sedimentation rates is found. Sternocostoclavicular hyperostosis is a slowly progressing disease, characterised by a chronic aseptic destructive sternoclavicular arthritis with a reactive low turnover sclerosis that begins in a similar way to an enthesopathy and ends after several decades with total ankylosis. The radiological identification of retrosternal proliferation of soft tissue by computed tomography was found to be a valuable criterion for the differential diagnosis from other benign hyperostotic processes of the sternoclavicular region.

Localization of occult persisting medullary thyroid carcinoma before microsurgical reoperation: high sensitivity of selective venous catheterization.

In 14 patients with occult persisting medullary thyroid carcinoma, tumor tissue was removed by microsurgical reoperation in 13 of 14 patients. This resulted in biochemical improvement in all but 1 patient and biochemical cure in 3 patients (21%). The lateral compartment of the neck or the upper mediastinum was involved in all but 1 patient. Before microsurgical reoperation, selective venous catheterization (SVC) for serum sampling along with serum calcitonin (CT) determination was done and compared to other localization methods. Tumor tissue could be localized correctly by SVC in 89% (CT gradient 1.21-2.02), computed tomography in 38%, and ultrasound in 28%. In patients with an elevated CT level after initial surgery and clinically occult disease, SVC is recommended for localization of tumor tissue. The affected side of the neck should be reoperated on with microdissection of the central and lateral compartment of the neck and the upper mediastinum. With this procedure, the cure rate of reoperation in patients with persistent occult MTC can be improved.

Hypertension in rats induced by renal grafts from renovascular hypertensive donors.

Renal transplantations were performed, using microsurgical techniques, with adult male two-kidney, one clip hypertensive rats (n = 9) and sham-operated normotensive Wistar-Kyoto rats (n = 8) as kidney donors and with F1 hybrids, bred from Wistar-Kyoto and stroke-prone spontaneously hypertensive rat parents, as recipients. Systolic blood pressure before surgery was 200 +/- 2.7 mm Hg in hypertensive and 115 +/- 1.7 mm Hg in normotensive donors and 144 +/- 7.1 and 138 +/- 3.5 mm Hg in the two groups of recipients. Renal hypertension in donors was maintained for 14 weeks before surgery was performed and the nonischemic kidneys were transplanted. Bilaterally nephrectomized recipients of renal grafts from hypertensive donors developed sustained hypertension (185 +/- 3.9 mm Hg). In contrast, in recipients of renal grafts from normotensive donors, blood pressure decreased significantly to the level of the donors (111 +/- 3.7 mm Hg). Posttransplantation hypertension in recipients of renal grafts from hypertensive donors was associated with intrarenal vascular hypertrophy, smaller kidneys, a decreased glomerular filtration rate, an increased plasma urea concentration, and polydipsia as compared with normotensive transplanted controls. Renal pyelograms revealed no gross anatomic alterations of transplanted kidneys. Our data indicate that secondary damage to the renal grafts caused by high perfusion pressure before transplantation can induce hypertension in recipients of these kidneys. Furthermore, our data suggest that renal mechanisms may be necessary to maintain borderline hypertension in F1 hybrids.

[Dilatation treatment of esophageal stenoses in young patients with recessive epidermolysis bullosa dystrophica]. / Dilatationsbehandlung von Osophagusstenosen bei jungen Patienten mit rezessiver Epidermolysis bullosa dystrophica.

Recessive dystrophic epidermolysis bullosa is a hereditary disorder affecting both skin and esophageal mucosa. The esophagus is frequently affected resulting in almost complete obstruction. We report here the use of inflatable dilatator balloons as a new treatment of esophageal stenosis in three young patients. Dilatation therapy was tolerated well in all three cases and lead to a long standing success. The patients lived without complaints and they did eat normally up to 2.5 years following dilatation.

[Cardiomyopathy in hypocalcemia]. / Kardiomyopathie bei Hypocalcämie.

A 48-year-old woman developed a hypocalcaemic cardiomyopathy, the hypocalcaemia being due to hypoparathyroidism after three previous thyroid operations for goitre with tracheal compression. She had signs of severe cardiac failure, but no tetany. She was put on calcium and vitamin D3 medication which raised calcium concentration. The cardiac status improved, as did the radiological and echocardiographic findings, without the patient having received any diuretics, digitalis or afterload lowering drugs.

[Extraosseous calcification in juvenile dermatomyositis. The ineffectiveness of EHDP]. / Extraossäre Calcifikationen bei juveniler Dermatomyositis. Unwirksamkeit von EHDP.

A now 20-year-old man was first diagnosed as having dermatomyositis when aged 12 years. At that time he had muscular weakness and heart failure followed a year later by heterotopic calcifications. For over seven years he was treated with corticoids, at times also with azathioprine or methotrexate. Nonetheless the calcifications progressed. Because of the extensive calcifications, predominantly of the extremities, he is severely disabled and has been confined to a wheel-chair. For one year he was treated with EHDP (1-hydroxyethylidenediphosphonate), up to 16 mg/dl per day. In addition he received prednisolone (10 mg/d) and azathioprine (100 mg/d). But radiologically there has been further slight progression of the calcifications, indicating that EHDP has been ineffective in this patient.

A newly developed spine deformity index (SDI) to quantitate vertebral crush fractures in patients with osteoporosis.

The available methods to quantitate vertebral deformity in osteoporotics are not satisfactory in comparing follow-up measurements in patients. This paper describes a newly developed 'spine deformity index' (SDI) which allows the quantitation of the extent of vertebral fractures. It is based on the observation that, in 110 normal persons, the heights of all vertebral bodies were related to each other in a predictable and constant manner. This relation was independent of the body height of the individual and was preserved despite growth acceleration during the last century. Since in all but one of our osteoporotic patients the 4th thoracic vertebra was unfractured we were able to compare the actual size of their fractured vertebrae to the calculated presumable original heights. The differences between presumable original and actual heights gave a measure of the extent of vertebral compression and allowed to define an index representing the sum of all spinal fractures in osteoporotics. The method was applied retrospectively to X-rays of 39 patients with idiopathic osteoporosis. Thirty-two of them were treated orally with 80 mg sodium fluoride, 1,000 mg calcium and 3000 IE vitamin D daily. Treatment resulted in a reduction of the progression of vertebral deformity. Seven inadequately treated patients had more pronounced progression of vertebral deformity.

Prediction of breakthrough patterns of organics from laboratory tests.

The investigations have shown that it is possible to predict the breakthrough behavior of complex mixtures in a pilot-plant filter. The aim of future studies will be to predict the breakthrough behavior of waterworks filters in the same way as discussed here. This seems to be fairly simple if the filters are working with adsorption only. If, in addition, biological processes occur in a filter (as is generally observed), these processes have to be taken into account because an additional mechanism exists for removal of organic substances in the activated carbon filter. Investigations in a new research program will also need to consider the influence of ozone on biodegradation. Use of the methods already discussed, can help to optimize existing plants, as well as a proper control of the efficiency of the regeneration processes.

[Value of various radiological study results in the follow-up of Camurati-Engelmann disease]. / Die Wertigkeit verschiedener radiologischer Untersuchungsverfahren in der Verlaufskontrolle der Camurati-Engelmannschen Krankheit.

Camurati-Engelmann disease is a rare progressive bone dysplasia; involvement of the skull base can lead to deafness, vestibular disturbances, facial paralysis and damage to the optic nerves. Treatment with corticosteroids, calcitonin and diphosphonates promises only very limited success. Conservative treatment of compression of the cranial nerves is almost ineffective, but the aim of surgical treatment is decompression of involved nerves. The differential diagnosis depends on radiological findings and clinical symptoms. Follow-up depends on radiographic examination and skeletal scintigraphy for showing the extent of the disease. CT may help in demonstrating compression of cranial nerves and define the indications for surgical decompression.

[Pulmonary candida manifestation with cavitation in immunosuppressed patients]. / Pulmonale Candidamanifestation mit kavernöser Einschmelzung bei immunsupprimierten Patienten.

The roentgenologic pattern of the pulmonary manifestation of candida species, resulting in a rapid development of pulmonary cavitations with mycetoma-like structures, was described in three patients. All patients, undergoing antineoplastic chemotherapy because of acute leukemia, presented with fever and expectoration, which were resistant to various antibiotic regimes. Cultures of blood and urine were sterile; but Torulopsis glabrata, a candida species, was found in multiple cultures of the sputum of all patients and also in a bronchoscopic lavage obtained from one patient after reconstitution of the granulopoesis. The roentgenologic appearance of the infiltrates was accompanied by a rise of the Ig-M immunoglobulins against candida. Following intravenous treatment with amphotericin B a reduction of the cavitation and of the infiltrates to small residues was observed. Simultaneously the body temperature and the sputum became normal and a fall in the immunoglobulin titers was found. The diagnostic problems of pulmonary cavitation and especially of pulmonary mycosis in immunosuppressed and therapy-induced granulocytopenic and thrombocytopenic patients with acute leukemia were discussed.

[Therapy of esophageal stenoses in recessive epidermolysis bullosa dystrophica]. / Zur Therapie von Osophagusstenosen bei rezessiver Epidermolysis bullosa dystrophica.

Stenosis or complete occlusion of the oesophagus are potentially life-threatening complications of recessive dystrophic epidermolysis bullosa. Consequences are malnutrition, growth retardation, aspiration, or cachexia. Total replacement of the oesophagus by colon interposition has been recommended in such patients. We report on successful conservative management. We applied recently developed knowledge concerning the defective collagenase involved in this disorder and oesophageal dilatation. Phenytoin has been shown to reduce the excessive production of collagenase and thereby to diminish blistering of skin and mucous membranes and stricture formation of the oesophagus. Stepwise dilatation of oesophageal strictures instead of bouginage represents a less traumatic way to restore the oesophageal lumen. The lumen can be maintained by soft nasogastric feeding tubes which may be removed later on after successful dilatation. Oesophageal passage has been maintained for up to 4 years. The management of these severe complications of recessive dystrophic epidermolysis bullosa requires interdisciplinary efforts of dermatologists, internists and otorhinolaryngologists.

Management of esophageal stenosis in recessive dystrophic epidermolysis bullosa.

Total replacement of the esophagus by colonic interposition has been recommended as the treatment of esophageal obstruction in recessive dystrophic epidermolysis bullosa. We report our experience in the conservative management of esophageal blisters, strictures, and complete occlusion in 5 patients (aged 2-61 yr). Our therapy consists of a combination of the following principles: (a) inhibition of collagenase formation by oral phenytoin to reduce epithelial detachment; (b) pureed or semiliquid food because minor trauma by hard food particles may induce blistering and result in scarring of the upper esophagus, and larger food particles may obstruct an esophageal stricture; (c) avoidance of tangential shearing forces induced by bougienage and endoscopy and instead use of inflatable dilatator balloons which produce vertical pressure that seems to be less harmful; and (d) long-term nasogastric tube feeding, which may relieve even tight strictures. Our observations suggest that successful long-term conservative management of esophageal stenoses in dystrophic epidermolysis bullosa is possible.

[Paget disease of the skeleton. Internal medicine aspects]. / Morbus Paget des Skelettes. Internistische Aspekte.

Paget's disease of bone seems to be a slow virus infection of a single or several areas of the skeleton. Pagetic lesions are rather common among elderly people, but the disease does not manifest itself very often. Compared to the incidence of Paget's disease in England it seems to be rarer in this country. The pelvis is most frequently involved, followed by bones of the leg, skull, lumbar spine. The patients suffer from pain and deformities, arthroses of the adhering ankles, increased temperature of the area, nerve irritation and nerve damage due to increased bone growth (e.g. hearing loss due to Paget's disease of the skull). The development of sarcoma is rare and is only seen in severe cases. Diagnosis is made by X-ray, confirmed by bone biopsy, if necessary. Asymptomatic lesions are detected by bone scintigraphy. The activity of the disease is expressed by increased alkaline serum phosphatase and urinary hydroxyproline excretion. For treatment calcitonins and diphosphonates (especially EHDP, Diphos) are used. Both inhibit the overactive osteoclasts, and the increased bone turnover is normalized. The patients feel considerable relief; the elevated biochemical parameters fall to about 50% of initial values after calcitonin or EHDP monotherapy. In severe cases the combination of both substances may be profitable. The cytostatic drug mithramycin which can also be effective is only needed in exceptional cases.

[Paget's osteitis deformans. Significance and control]. / Ostitis deformans Paget. Bedeutung von Verlaufskontrollen.

Follow-up observation of patients with Paget's disease by conventional radiology is useful to evaluate the spontaneous course of the disease in untreated cases and to determine the regeneration of the affected bone after therapy. This also includes complications of the otitis deformans Paget--mainly complete or incomplete fractures, severe deformations as well as rare malignant changes.