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At least 1,711 records · Page 95Linked to original sources

[Radiotherapy in benign diseases: Morbus Peyronie].

PURPOSE: The induratio penis plastica (IPP) or the so-called Morbus Peyronie is a burdening disease for patients with three main symptoms, induration, deviation and pain. The etiology of this fibrosing and plaque forming disease is largely unknown up to now. PATIENTS AND METHODS: The presented data refer to a retrospective univariant examination of 67 patients, which have been treated for IPP with soft X-rays in the Dermatological Clinic of the Technical University of Munich between 1990 and 1995. The aim of the study was to examine, how far a progression of the disease can be stopped with soft X-rays and how the pain symptomatic is reduced. RESULTS: In 58 of 67 examined patients (86.6%) a progression of the disease could be stopped. 25 out of 67 patients (37.3%) complained of pain before therapy. This symptomatic mostly improved totally in 21 patients (84% of the patients with pain). A complete or partial dissolution of the indurations was to be noticed in 41 of 70 indurations (58.6%). In 23 of 60 patients (38.3%) an improvement or totally regression of the deviation was observed. It could be shown that therapeutic outcome significantly correlates to a shorter duration of anamnesis (p < 0.05), smaller plaque size (p < 0.025) and a tendency to lower age of the patients. CONCLUSION: Radiotherapy of Morbus Peyronie is extremely effective and has a low rate of side effects.

Adult↗

[Metageria--clinical manifestations of a premature aging syndrome].

A 19-year old caucasian patient suffered from ulceration and scaring of his fingers since age two. During childhood, fibrosing contractures on the phalanges developed. Since puberty, his eunuch-like stature, bird-like face and exophthalmos had become under obvious. His hair was extraordinarily fine; his pubic hair rare. Radiological examination revealed ankylosis and osteoporosis of the phalanges and carpals. Angiography showed occlusion of multiple digital and interdigital arteries. Furthermore, a bilateral posterior cataract, restrictive respiratory disease, impaired glucose tolerance, hyperuricemia, proteinuria and primary hypogonadism were diagnosed. These findings are characteristic for the premature aging syndromes. The cardial symptoms of tall stature, bird-like face, pseudoexophthalmos, skleroderma-like and poikiloderma-like cutaneous lesions, scarce hair growth, early diabetes mellitus and arteriosclerosis led to the diagnosis of metageria.

Adult↗

[Primary sclerosing cholangitis].

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, characterized by fibrosing inflammation and obliteration of intra and/or extrahepatic bile ducts. The disease belongs to the most common cholestatic diseases in adults and at present is diagnosed with increasing frequency. It is very often associated with ulcerative colitis. Patients with PSC have an increased incidence of bile duct carcinomas and those with ulcerative colitis also have an increased incidence of colonic carcinomas. Immunosuppressive treatment is little effective. Ursodeoxycholic acid (UDCA) has been shown to improve liver histology in PSC. The aim is to treat patients as early as possible to prevent progression to the advanced stages of the disease. During treatment with UDCA stenoses of major ducts may develop and early endoscopic dilatation is highly effective. In patients with endstage disease, UDCA is not effective and liver transplantation is indicated.

Bile Duct Neoplasms↗

[Chronic bronchitis, COPD].

Chronic bronchitis is part and precursor of COPD, a complex disease triggered mostly by exposure to cigarette smoke. However COPD develops only in patients with specific susceptibility probably determined by genetic factors or additional risk factors. A specific type of inflammation resides in the bronchial and bronchiolar walls, that infers damage not only to airway structure but also to surrounding alveolar attachments and thus to the lung parenchyma. Chronic bronchitis, fibrosing bronchiolitis and emphysema constitute the three main stems of pathology of the disease but may coexist with varying extent. The clinical picture is therefore quite variable. Treatment exists largely in bronchodilation combining different mechanisms and using long acting drugs usually applied by inhalation. Acute exacerbations promote progression of the disease, which must be counteracted by adaptation and intensification of therapy, in some cases including non invasive or invasive ventilation. Several non-pharmacologic measures such as smoking cessation, rehabilitation, nutritional support, long term oxygen therapy, lung volume reduction and possibly lung transplantation may be available for appropriate patients and have to be considered.

Air Pollutants↗

Blunted DOCA/high salt induced albuminuria and renal tubulointerstitial damage in gene-targeted mice lacking SGK1.

Mineralocorticoids stimulate renal tubular Na(+) reabsorption, enhance salt appetite, increase blood pressure, and favor the development of renal fibrosis. The effects of mineralocorticoids on renal tubular Na(+) reabsorption and salt appetite involve the serum- and glucocorticoid-inducible kinase 1 (SGK1). The kinase is highly expressed in fibrosing tissue. The present experiments thus explored the involvement of SGK1 in renal fibrosis. To this end, SGK1-knockout mice (sgk1 (-/-)) and their wild-type littermates (sgk1 (+/+)) were implanted with desoxycorticosterone acetate (DOCA)-release pellets and offered 1% saline as drinking water for 12 weeks. The treatment led to significant increases in fluid and Na(+) intake and urinary output of fluid and Na(+) in sgk1 (+/+) mice, effects blunted in sgk1 (-/-) mice. Blood pressure increased within the first 7 weeks to a similar extent in both genotypes, but within the next 5 weeks, it increased further only in sgk1 (+/+) mice. Creatinine clearance did not change significantly but albuminuria increased dramatically in sgk1 (+/+) mice, an effect significantly blunted in sgk1 (-/-) mice. Histology after 12 weeks treatment revealed marked glomerular sclerosis and tubulointerstitial damage with interstitial fibrosis and inflammation in kidneys from sgk1 (+/+) mice, but not from sgk1 (-/-) mice. In conclusion, a lack of SGK1 protects against DOCA/high-salt-induced albuminuria and renal fibrosis.

Albuminuria↗

[Hypertrophic cranial pachymeningitis as a rare cause of headache].

Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. The condition is being reported more frequently owing to the use of cranial MRI. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. A variety of autoimmune and infectious diseases can result in this condition, which is labeled as idiopathic in the absence of any definite inciting factor. The diagnosis of hypertrophic cranial pachymeningitis is based on neuroimaging of thickened and enhancing dura mater. It can be defined pathologically on biopsy. A specific treatment is indicated in some cases of secondary hypertrophic cranial pachymeningitis. Mostly, treatment relies on corticosteroids and immunosuppressive agents. This review summarizes the current knowledge on causes, clinical presentation, diagnosis, and treatment of this disorder.

Diagnosis, Differential↗

[Interventions for benign biliary strictures].

Due to their potential for serious consequences, even including biliary liver cirrhosis, benign biliary strictures pose a considerable diagnostic and therapeutic challenge. In addition to inflammatory disease or an acute liver injury, iatrogenically caused biliary strictures following hepatobiliary surgery represent in 95% of cases the main cause for all benign entities. The diagnosis should be determined noninvasively with magnetic resonance cholangiopancreaticography (MRCP). Invasive techniques such as ERCP or percutaneous transhepatic cholangiography (PTC) should be reserved for unclear cases and first performed before the scheduled intervention. Depending on the site and cause of the stricture, surgical and interventional procedures are employed in the treatment of biliary strictures. The best results are obtained in short-segment strictures of the main bile duct. Interventional methods such as balloon dilation and/or stent application with concomitant drain insertion achieve patency rates of up to 75% after 5 and 55% after 12 years with a total complication rate of 5-8%. Due to the fact that most of the cases involve cicatricial fibroses, predisposition for recurrence of biliary strictures after interventional therapy can be very high, ranging up to 66% depending on the localization.

Bile Duct Diseases↗

[HRCT of the lung in collagenoses].

Collagen vascular diseases, representing systemic soft tissue disorders, may cause a broad spectrum of pathologic changes of the respiratory tract. The type and extent of manifestations can vary considerably among individuals and entities. This survey describes the chest radiographic and, in particular, high-resolution computed tomographic (HRCT) findings of individual lesions of the respiratory tract. It includes fibrosing alveolitis (alveolitis, interstitial pneumonia, pulmonary fibrosis) and bronchial (bronchitis/bronchiolitis, bronchiectasis), pleural and vascular manifestations, as well as lymphadenopathy and abnormalities related to therapy. We present typical patterns of changes in progressive systemic sclerosis (PSS, scleroderma), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD, Sharp syndrome), Sjögren syndrome, overlap syndrome and rheumatoid arthritis (RA). Furthermore, we describe findings which are specific for individual entities such as esophageal involvement in PSS, acute pneumonitis and pulmonary hemorrhage in SLE, lymphoproliferative disease in Sjögren syndrome and necrobiotic nodules in RA.

Arthritis, Rheumatoid↗

Cholestatic syndromes in renal transplant recipients with HCV infection.

We present two distinct types of cholestatic syndrome identified in eight renal transplant (RTx) patients with HCV infection. Four patients developed fibrosing cholestatic hepatitis (FCH) and four, vanishing bile duct syndrome (VBDS). All patients with FCH were anti-HCV (-) at the time of Tx and developed a cholestatic profile 1-4 months post-Tx, with high HCV-RNA levels. Immunosuppressive therapy was drastically reduced. Two patients died of sepsis and liver failure 16 and 18 months post-Tx, and the other two showed marked improvement and seroconverted to anti-HCV. Regarding the patients with VBDS, three were anti-HCV (-) and one was anti-HCV (+)/HBsAg (+) at the time of RTx. Two patients became anti-HCV (+) 1 year, and one patient, 3 years post-Tx. Two patients developed progressive VBDS and died of liver failure 2 and 3 years after onset, and two showed marked improvement after withdrawal of immunosuppression. In two of the patients, the progression of the disease coincided with elevation in serum HCV RNA levels. We concluded that a progressive cholestatic syndrome acquiring features of FCH or VBDS may develop in HCV-infected RTx patients. The association with high viral load implicated the virus in the pathogenesis. Drastic reduction of immunosuppression may favourably affect the outcome.

Adult↗

Bladder augmentation techniques in women.

Augmentation cystoplasty is performed in women for a variety of indications, including neurogenic bladder dysfunction, inflammatory diseases, small fibrosed bladders, idiopathic urge incontinence and enuresis. The preoperative evaluation, surgical techniques and postoperative management are reviewed. Complications of stone formation, urinary tract infections, mucus production, metabolic disturbances, hematuria and dysuria syndrome, tumors and perforations are addressed. In addition, alternative techniques such as ureterocystoplasty, autoaugmentation, seromuscular cystoplasty and the future of augmentation of the bladder utilizing techniques of tissue engineering are discussed. The management of pregnancy in women who have previously undergone augmentation cystoplasty is also reviewed.

Cecum↗

Idiopathic hypertrophic pachymeningitis: spectrum of the disease.

Hypertrophic pachymeningitis is extremely rare. It is a fibrosing inflammatory process which involves the dura mater, including the tentorium. Numerous pathological entities produce thickening of the pachymeninges, so that idiopathic hypertrophic pachymeningitis is a diagnosis of exclusion. We describe four patients with idiopathic hypertrophic pachymeningitis who had varied clinical presentation. Imaging studies revealed diffuse thickening of the pachymeninges; in one patient there was extensive dural sinus thrombosis. Since no identifiable cause was found, the cases were labelled as idiopathic.

Adult↗

Characteristics of calcaneal bone infarction: an MR imaging investigation.

OBJECTIVE: Bone infarction (BI) of the calcaneus is an uncommon entity which has received little mention in the recent literature. In this paper, we review the MR images of six calcanei with BI, which demonstrate a pattern of presentation that may explain the etiology of BI at this unusual location. DESIGN: A retrospective review was performed of the transcribed reports of the foot or ankle MR examinations at our institution. MR images of examinations with any marrow signal abnormality were reviewed for presence of BI and its distribution. PATIENTS: Based on MRI criteria, four patients had calcaneal BI (none biopsy proven); they ranged in age from 37 to 51 years old. Two patients were diagnosed with systemic lupus erythematosus, one with fibrositis, and another with polymyositis. All were treated with corticosteroids. RESULTS: Six calcanei (in four patients) contained a region of calcaneal BI. In five of the six, the lesions were entirely or predominantly located in the posterior half of the calcaneus. CONCLUSION: Two theories are proposed which may explain why BI predominantly occurs in the posterior half of the calcaneus. First, the convergence of the recurrent intraosseous calcaneal vessels may occasionally produce the equivalent of a single dominant vessel that is more prone to vascular accidents. Secondly the region between the recurrent and the epiphyseal vessels may act as a watershed zone, increasing its susceptibility to ischemia.

Adult↗

Effects of peritoneal lavage with scolicidal agents on survival and adhesion formation in rats.

Intraoperative irrigation of the peritoneal cavity with scolicidal agents is frequently recommended when dealing with traumatic or spontaneous rupture of hydatid cysts. The present experimental study was designed to examine the influence of various scolicidal agents on adhesion formation and survival. A total of 149 rats were randomly allocated to nine groups. Peritoneal lavage through a median laparotomy was performed with the following scolicidal agents. Group 1 (0.9% saline: controls), group 2 (20% hypertonic saline), group 3 (0.04% chlorhexidine gluconate), group 4 (3% hydrogen peroxide), group 5 (0.5% silver nitrate), group 6 (1% polyvinylpyrrolidone-iodine, or PVP-I ), group 7 (5% PVP-I), group 8 (0.5% cetrimide/0.05% chlorhexidine), and group 9 (10% PVP-I). The surviving animals were sacrificed on postoperative day 15. Adhesion formation was macroscopically graded by the Nair criteria. The severity of adhesion formation was evaluated microscopically using the fibrosing scoring criteria and the strain test. Group 9 (10% PVP-I) was excluded from the adhesion evaluation because all of the rats died in this group. The mortality rate was significantly higher in groups 5 and 7 than in groups 1, 2, 3, 4, 6, and 8. Adhesion scores were significantly lower in groups 1, 2, 3, and 4 than in groups 5, 6, 7, and 8. The lowest adhesion score was found in group 3 and the highest in the group 7. These results indicate that 0.04% chlorhexidine gluconate, the most potent scolicidal agent in vitro and in vivo, was associated with the lowest adhesion formation and mortality among various scolicidal agents in this experimental study.

Animals↗

[Sclerosing epithelioid fibrosarcoma].

The sclerosing epithelioid fibrosarcoma (SEF), defined as an entity by Meis-Kindblom et al. in 1995 [15] is now considered to be a variant of fibrosing fibrosarcomas. It is a rare tumor with an intermediate malignant potential leading to local recurrences in one third and to metastases in about 40% of the cases. We report six cases of this entity. At the time of diagnosis two patients of our series already showed metastases in the lungs. The tumors were located in the deep soft tissue and measured between 2.5 and 17 cm. The histology is characterized by small epithelioid cells that are arranged individually or in cords and nests and set typically in a hyaline sclerotic matrix. By immunohistochemistry, all cases were vimentin positive, however EMA positive cells are also possible. The differential diagnosis includes metastases of carcinoma, benign and malignant soft tissue tumors. The distinction of SEF from fibromatosis, fibrous histiocytomas, ossifying fibromyxoid tumors, clear cell sarcomas, epithelioid sarcomas, synovial sarcomas and extraskeletal osteosarcomas is discussed.

Adult↗

[Effects of irradiation therapy and inhibiting drugs on the pituitary and its adenomas].

Radiation therapies of pituitary adenomas induce an increase in fibroses and nuclear pleomorphism. Most growth hormone (GH) secreting pituitary adenomas react to somatostatin analogues by a distinct decrease of GH secretion. In two thirds, levels of IGF-1 can be normalized. Some cases show a shrinkage of adenomas that correlates with fibrosis of the tumor. With these drugs, thyroid stimulating hormone secreting adenomas can also be treated. Prolactin secreting adenomas are mostly treated primarily with dopamine agonists. Up to 90% of cases show a strong decrease in hormone secretion and a distinct shrinkage of the adenomas based on strong decrease in adenoma cell volume. Long-term medication with high doses of glucocorticoids induces Crooke's cells in the anterior pituitary. These are suppressed ACTH cells and characterized by increased numbers of large lysosomes and dense bundles of cytofilaments.

Antineoplastic Agents↗

[Proliferation kinetics of bronchioloalveolar tumorlets].

Bronchiolo-alveolar tumorlets--mostly clinically asymptomatic--generally are noticed as "contingent findings" in the diagnoses of interstitial fibrosing lung diseases. The growth fraction of these epithelial proliferates in the WHO classification summarized in the group of tumor like lesions was explored by immunohistochemical stainings with the PCNA and Ki67 (MIB1) antibodies and correlated with (pre-) neoplastic lesions of the lung. The proliferation indices varied between 0.029 to 0.67 (MIB1) and 0.057 to 0.81 (PCNA). Depending on the main disease the observation of cuboid metaplasias, areas of bronchiolization via tumorlets in inactive interstitial lung fibrosis up to bronchiolo alveolar tumorlets following interstitial lung disease after cytostatic treatment showed increasing proliferation indices. Accordingly to prencoplastic changes of the bronchial epithelium the findings suggest that interpretation of bronchiolo alveolar tumorlets as basic cells of malignant peripheral lung tumors seems to be possible. The value of these findings in relationship to the associated interstitial lung disease is discussed in the context of reflections related to the formal pathogenesis of so called scar cancer.

Adenocarcinoma, Bronchiolo-Alveolar↗

[Pleural reaction pattern after talc pleurodesis].

Because of the rising importance of talcum pleurodesis in the therapeutic management of malignant and benign pleural effusions in Germany, pleural samples after talcum pleurodesis were compared to microscopic, immunohistochemical, and scanning electron microscopy findings after Tetracyclin and Novantron pleurodesis in 24 patients up to 18 months after intrapleural therapy. The histomorphological findings after talcum pleurodesis show an early phase of up to 4 weeks, characterized by the presence of talcum-containing granulation tissue with giant-cell foreign-body reaction, and a subsequent late phase with organization of the granulation tissue and connective tissue formation and obliteration of the pleura sheets. The resulting characteristic linear talcum zone in the pleural connective tissue is missing in cases with tumorous infiltration of the pleura. Continuous fibroblast activation, e.g., by macrophage or foreign-body giant-cell-released mediators, seems to be the decisive factor in the fibrosing process. No therapy-related tumor growth alterations could be demonstrated.

Follow-Up Studies↗

Long-term success of endovascular treatment of benign superior vena cava occlusion with chylothorax and chylopericardium.

The most likely etiology of benign obstruction of the superior vena cava (SVC) include fibrosing mediastinitis and iatrogenic etiologies such as sclerosis and obstruction caused by pacemakers and central venous catheter. Percutaneous stenting of SVC has been used with success both in malignant and benign superior vena cava syndrome; however, long-term follow-up of endovascular procedures is not well known. We present a case of a patient with complete occlusion of SVC of benign etiology, presenting dramatically with bilateral chylothorax and chylopericardium with cardiac tamponade, who underwent successful vena caval revascularization with thrombolytic therapy and placement of self-expanding metallic stent. The 42-month follow-up could encourage endovascular procedures even in SVC syndrome of benign etiology.

Breast Neoplasms↗