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1.
Plant Biol (Stuttg) ; 25(5): 803-812, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37194683

ABSTRACT

Heat stress alters plant defence responses to pathogens. Short-term heat shock promotes infections by biotrophic pathogens. However, little is known about how heat shock affects infection by hemibiotrophic pathogens like Bipolaris sorokiniana (teleomorph: Cochliobolus sativus). We assessed the effect of heat shock in B. sorokiniana-susceptible barley (Hordeum vulgare cv. Ingrid) by monitoring leaf spot symptoms, B. sorokiniana biomass, ROS and plant defence-related gene expression following pre-exposure to heat shock. For heat shock, barley plants were kept at 49 °C for 20 s. B. sorokiniana biomass was assessed by qPCR, ROS levels determined by histochemical staining, while gene expression was assayed by RT-qPCR. Heat shock suppressed defence responses of barley to B. sorokiniana, resulting in more severe necrotic symptoms and increased fungal biomass, as compared to untreated plants. Heat shock-induced increased susceptibility was accompanied by significant increases in ROS (superoxide, H2 O2 ). Transient expression of plant defence-related antioxidant genes and a barley programmed cell death inhibitor (HvBI-1) were induced in response to heat shock. However, heat shock followed by B. sorokiniana infection caused further transient increases in expression of HvSOD and HvBI-1 correlated with enhanced susceptibility. Expression of the HvPR-1b gene encoding pathogenesis-related protein-1b increased several fold 24 h after B. sorokiniana infection, however, heat shock further increased transcript levels along with enhanced susceptibility. Heat shock induces enhanced susceptibility of barley to B. sorokiniana, associated with elevated ROS levels and expression of plant defence-related genes encoding antioxidants, a cell death inhibitor, and PR-1b. Our results may contribute to elucidating the influence of heat shock on barley defence responses to hemibiotrophic pathogens.


Subject(s)
Ascomycota , Hordeum , Ascomycota/physiology , Hordeum/genetics , Reactive Oxygen Species , Plants/genetics , Gene Expression , Heat-Shock Response/genetics , Plant Diseases/genetics , Plant Diseases/microbiology
2.
Plant Biol (Stuttg) ; 22(1): 70-80, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31283085

ABSTRACT

The effects of elevated glutathione levels on defence responses to powdery mildew (Euoidium longipes) were investigated in a salicylic acid-deficient tobacco (Nicotiana tabacum cv. Xanthi NahG) and wild-type cv. Xanthi plants, where salicylic acid (SA) contents are normal. Aqueous solutions of reduced glutathione (GSH) and its synthetic precursor R-2-oxothiazolidine-4-carboxylic acid (OTC) were injected into leaves of tobacco plants 3 h before powdery mildew inoculation. SA-deficient NahG tobacco was hyper-susceptible to E. longipes, as judged by significantly more severe powdery mildew symptoms and enhanced pathogen accumulation. Strikingly, elevation of GSH levels in SA-deficient NahG tobacco restored susceptibility to E. longipes to the extent seen in wild-type plants (i.e. enhanced basal resistance). However, expression of the SA-mediated pathogenesis-related gene (NtPR-1a) did not increase significantly in GSH or OTC-pretreated and powdery mildew-inoculated NahG tobacco, suggesting that the induction of this PR gene may not be directly involved in the defence responses induced by GSH. Our results demonstrate that artificial elevation of glutathione content can significantly reduce susceptibility to powdery mildew in SA-deficient tobacco.


Subject(s)
Ascomycota , Disease Resistance , Glutathione , Nicotiana , Salicylic Acid , Ascomycota/drug effects , Ascomycota/physiology , Disease Resistance/drug effects , Gene Expression Regulation, Plant , Glutathione/metabolism , Glutathione/pharmacology , Plants, Genetically Modified/chemistry , Plants, Genetically Modified/microbiology , Nicotiana/chemistry , Nicotiana/microbiology
3.
Ophthalmologe ; 109(3): 242-9, 2012 Mar.
Article in German | MEDLINE | ID: mdl-22447421

ABSTRACT

BACKGROUND: Implantation of phakic intraocular lenses (pIOL) is gaining in importance. Exact measurement of the anterior chamber is important to assure safe and effective pIOL implantation. In this study six currently available diagnostic devices were tested in order to compare accuracy of measurements (anterior chamber depth and diameter) and ease of handling. PATIENTS AND METHODS: In this study 100 eyes from 50 healthy patients were enrolled. All measurements were done by the same operator (L. K.) on the same day and under the same lighting conditions. The following devices were used to measure anterior chamber depth and diameter: IOLMaster 500, Visante OCT, UBM Vumax II, Galilei, Pentacam Classic und Orbscan IIz. Correlations between each method were assessed using Pearson's correlation. Bland Altman plots were composed to evaluate the range of agreement between two methods. RESULTS: The mean epithelial anterior chamber depth was 3.45 ± 0.38 mm with IOLMaster 500, 3.69 ± 0.33 mm with Visante OCT, 3.59 ± 0.35 mm with Pentacam, 3.67 ± 0,36 mm with Galilei, 3.48 ± 0.38 mm with Orbscan IIz and 3.52 ± 0.34 mm with UBM VumaxII. The mean white-to-white measurements were 11.98 ± 0.37 mm with IOLMaster 500 and 11.71 ± 0.36 mm with Orbscan IIz. The mean angle-to-angle distances were 11.83 ± 0. 43 mm with Visante OCT, 10.73 ± 0.38 mm with Pentacam, 12.30 ± 0.45 mm with Galilei, and 11.15 ± 0.52 mm with UBM Vumax II. In the Bland Altman plots the range of agreement was 0.5 mm between Visante OCT and IOLMaster 500, regarding anterior chamber depth as well as anterior chamber diameter. CONCLUSION: The IOLMaster 500 provides exact measurement of the anterior chamber depth and diameter and is easy to handle in the daily clinical routine.


Subject(s)
Anterior Eye Segment/anatomy & histology , Biometry/instrumentation , Corneal Topography/instrumentation , Lens Implantation, Intraocular/methods , Phakic Intraocular Lenses , Adult , Equipment Design , Equipment Failure Analysis , Female , Humans , Male , Middle Aged , Reproducibility of Results , Sensitivity and Specificity , Technology Assessment, Biomedical , Young Adult
4.
Klin Monbl Augenheilkd ; 225(1): 70-4, 2008 Jan.
Article in German | MEDLINE | ID: mdl-18236374

ABSTRACT

AIM: We examined the effectiveness of arcuate incisions by femtosecond laser treatment for high astigmatism in corneal transplants. BACKGROUND: High astigmatism is a well known complication after corneal transplantation and is difficult to treat. At present, there are many surgical procedures for the correction of high astigmatism after PKP in case of an unsuccessful contact lens therapy. PATIENTS AND METHODS: Ten patients with a high postoperative astigmatism underwent arcuate keratotomy with the Femtec laser. We performed two arcuate incisions on each transplant. With the femtosecond laser technology it is possible to perform precise incisions where the depth and length of the arcus can be programmed. RESULTS: The best corrected visual acuity was improved in eight out of ten patients. The visual acuity of two patients did not change. The average corneal astigmatism was reduced by three dioptres. The average refractive astigmatism was reduced by four dioptres. CONCLUSION: Arcuate incisions with the femtosecond laser on patients with high astigmatism subsequent to corneal transplantation are an effective and safe treatment.


Subject(s)
Astigmatism/diagnosis , Astigmatism/surgery , Corneal Surgery, Laser/methods , Corneal Transplantation/adverse effects , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Treatment Outcome
5.
J Gen Virol ; 89(Pt 3): 799-808, 2008 Mar.
Article in English | MEDLINE | ID: mdl-18272772

ABSTRACT

Tissue necroses and resistance during the hypersensitive response (HR) of tobacco to tobacco mosaic virus (TMV) are overcome at temperatures above 28 degrees C and the virus multiplies to high levels in the originally resistant N-gene expressing plants. We have demonstrated that chemical compounds that generate reactive oxygen species (ROS) or directly applied hydrogen peroxide (H(2)O(2)) are able to induce HR-type necroses in TMV-inoculated Xanthi-nc tobacco even at high temperatures (e.g. 30 degrees C). The amount of superoxide (O(2)(*-)) decreased, while H(2)O(2) slightly increased in TMV- and mock-inoculated leaves at 30 degrees C, as compared with 20 degrees C. Activity of NADPH oxidase and mRNA levels of genes that encode NADPH oxidase and an alternative oxidase, respectively, were significantly lower, while activity of dehydroascorbate reductase was significantly higher at 30 degrees C, as compared with 20 degrees C. It was possible to reverse or suppress the chemically induced HR-type necrotization at 30 degrees C by the application of antioxidants, such as superoxide dismutase and catalase, demonstrating that the development of HR-type necroses indeed depends on a certain level of superoxide and other ROS. Importantly, high TMV levels at 30 degrees C were similar in infected plants, whether the HR-type necrotization developed or not. Suppression of virus multiplication in resistant, HR-producing tobacco at lower temperatures seems to be independent of the appearance of necroses but is associated with temperatures below 28 degrees C.


Subject(s)
Gene Expression Regulation, Plant , Hot Temperature , NADPH Oxidases/metabolism , Nicotiana/virology , Oxidoreductases/metabolism , Superoxides/metabolism , Tobacco Mosaic Virus/pathogenicity , Down-Regulation , Hydrogen Peroxide/metabolism , Immunity, Innate , Necrosis , Plant Leaves/virology , Temperature , Nicotiana/enzymology , Nicotiana/immunology , Up-Regulation
6.
Curr Microbiol ; 52(4): 282-6, 2006 Apr.
Article in English | MEDLINE | ID: mdl-16550466

ABSTRACT

Analysis of virulence mechanisms of plant pathogens is often limited by the lack of genetic tools that can be used to identify genes that are preferentially expressed during their interactions with plants. In the present study, we used the newly constructed IVET (in vivo expression technique) plasmid pIviGK and the corresponding antibiotic resistance-based selection method to identify genes that encode pathogenicity factors of the soft rot-causing bacterium Pseudomonas viridiflava. These included pel, the gene encoding pectate lyase, which is responsible for the development of soft rot symptoms. We have also isolated and characterized the gene mviNpv encoding a putative novel membrane associated virulence factor of P. viridiflava. A mutation in mviNpv was shown to influence motility as well as virulence of P. viridiflava. The mviNpv gene is expressed to a moderate level in LB media and its expression increases under inducing conditions as was shown by measuring in planta expression dynamics of the fused gfp reporter gene.


Subject(s)
Genes, Bacterial , Promoter Regions, Genetic , Pseudomonas/genetics , Capsicum/microbiology , Cloning, Molecular , Mutation , Plant Diseases/microbiology , Plasmids , Polysaccharide-Lyases/genetics , Pseudomonas/pathogenicity , Virulence/genetics
8.
Mol Plant Microbe Interact ; 14(1): 31-41, 2001 Jan.
Article in English | MEDLINE | ID: mdl-11194869

ABSTRACT

Cauliflower mosaic virus strain W260 elicits a hypersensitive response (HR) in leaves of Nicotiana edwardsonii, an interspecific hybrid derived from a cross between N. glutinosa and N. clevelandii. Interestingly, we found that N. glutinosa is resistant to W260, but responds with local chlorotic lesions rather than necrotic lesions. In contrast, N. clevelandii responds to W260 with systemic cell death. The reactions of the progenitors of N. edwardsonii to W260 infection indicated that each contributed a factor toward the development of HR. In this study, we present two lines of evidence to show that the resistance and cell death that comprise the HR elicited by W260 can indeed be uncoupled. First, we showed that the non-necrotic resistance response of N. glutinosa could be converted to HR when these plants were crossed with N. clevelandii. Second, we found that cell death and resistance segregated independently in the F2 population of a cross between N. edwardsonii and N. clevelandii. We concluded that the resistance of N. edwardsonii to W260 infection was conditioned by a gene derived from N. glutinosa, whereas cell death was conditioned by a gene derived from N. clevelandii. An analysis of pathogenesis-related (PR) protein expression in response to W260 infection revealed that elicitation of PR proteins was associated with resistance rather than with the onset of cell death.


Subject(s)
Caulimovirus/pathogenicity , Nicotiana/virology , Plant Diseases/virology , Plants, Toxic , Cell Death/genetics , Genes, Plant , Hybridization, Genetic , Models, Biological , Plant Proteins/biosynthesis , Nicotiana/cytology , Nicotiana/genetics
9.
J Urol ; 165(1): 219-20, 2001 Jan.
Article in English | MEDLINE | ID: mdl-11125410

ABSTRACT

PURPOSE: We evaluated the clinical effectiveness of topical steroid application for balanitis xerotica obliterans in children and analyzed the association of any clinical response with histological findings. MATERIALS AND METHODS: Our double-blind, placebo controlled, randomized study included 40 boys in whom balanitis xerotica obliterans was diagnosed clinically by cicatricial phimosis. The severity of phimosis was graded into 4 groups. Patients were randomized to receive the topical application of 0.05% mometasone furoate or placebo. After 5 weeks phimosis severity was reevaluated and all patients underwent circumcision. Surgical specimens were histologically typed as an early, intermediate or late form of balanitis xerotica obliterans. RESULTS: Seven patients were withdrawn from the study. In the steroid group 7 boys had clinical improvement and 10 had no change. Histological study showed an early, intermediate and late form of balanitis xerotica obliterans in 5, 5 and 7 cases, respectively. Of cases with clinical improvement 5 were the early and 2 the intermediate type. In the placebo group 5 cases worsened clinically and 11 did not change. Histological evaluation revealed an early, intermediate and late form of balanitis xerotica obliterans in 3, 7 and 6 boys, respectively. Of the 5 cases with histological worsening, disease was the early, intermediate and late type in 2, 2 and 1, respectively. CONCLUSIONS: Applying a potent topical steroid affects improvement in balanitis xerotica obliterans in the histologically early and intermediate stages of disease, and may inhibit further worsening in the late stage.


Subject(s)
Anti-Inflammatory Agents/therapeutic use , Balanitis/drug therapy , Pregnadienediols/therapeutic use , Administration, Topical , Balanitis/pathology , Child , Circumcision, Male , Double-Blind Method , Glucocorticoids , Humans , Male , Mometasone Furoate , Penis/pathology , Phimosis/pathology , Pregnadienediols/administration & dosage
10.
Eur J Cardiothorac Surg ; 16(6): 660-2, 1999 Dec.
Article in English | MEDLINE | ID: mdl-10647837

ABSTRACT

OBJECTIVE: Between 1977 and 1988, 27 patients, mean age 8.9 (range 4-22) received an antibiotic sterilised aortic allograft in the setting of the Fontan procedure. This study describes the long-term follow-up of these patients. METHODS: Fifteen patients had tricuspid atresia, nine double inlet ventricles and three others. The connection with the allograft was made to the pulmonary artery on the right side of the aorta in ten and to a left-sided main pulmonary artery in eight. In nine patients the allograft was anastomosed between the right atrium and the right ventricle. RESULTS: There were five early and five late deaths. One late death may have been allograft related. Survival was 81, 74 and 68% at 5, 10 and 15 years, respectively. Conduit calcification was universal. Twelve patients underwent reoperation, freedom from reoperation was 100, 88 and 54% at 5, 10 and 15 years following the initial Fontan procedure. At reoperation the gradient across the allograft was never more than 3 mmHg. The allograft was explanted with conversion to atriopulmonary or cavopulmonary connection in nine and a second allograft was inserted between the right atrium and right ventricle in two. No mortality occurred at reoperation. 41.1% of survivors still have their original allograft. CONCLUSIONS: Although there is a significant attrition rate allograft inclusion in the Fontan circulation does not change survival, but results in an increased reoperation rate. Inclusion of a valved conduit between the right atrium and ventricle does not usually enhance the growth potential of the rudimentary ventricle. All patients are in a good functional class which may represent the strict original selection criteria. The inclusion of a valve in the Fontan circulation is not recommended.


Subject(s)
Anti-Bacterial Agents/pharmacology , Aorta, Thoracic , Blood Vessel Prosthesis Implantation/methods , Fontan Procedure , Heart Defects, Congenital/surgery , Organ Preservation/methods , Sterilization/methods , Adolescent , Adult , Blood Vessel Prosthesis Implantation/mortality , Child , Child, Preschool , Female , Follow-Up Studies , Fontan Procedure/mortality , Graft Survival , Humans , Pregnancy , Reoperation , Retrospective Studies , Survival Rate , Transplantation, Homologous , Treatment Outcome
11.
Heart ; 77(2): 173-5, 1997 Feb.
Article in English | MEDLINE | ID: mdl-9068403

ABSTRACT

In muscular pulmonary atresia, major aortopulmonary collateral arteries are characteristic of pulmonary atresia with ventricular septal defect and are rarely seen in pulmonary atresia with intact ventricular septum. Two unusual cases of muscular pulmonary atresia are reported, one with an intact septum and one with a perimembranous ventricular septal defect, closed in utero by aneurysmal tricuspid tissue. In both cases the pulmonary blood supply came entirely from aortopulmonary collaterals. In case 1 a collateral artery connected the left subclavian artery and hypoplastic pulmonary arteries, and several aortopulmonary collaterals arose from the descending aorta, without overlap between these two circulations. In case 2 the pulmonary trunk and arterial duct were absent and the pulmonary blood supply came entirely from collateral arteries. The right ventricle was of normal size and tripartite with a closed perimembranous ventricular septal defect, discovered only at postmortem examination. These observations suggest right ventricular outflow tract obstruction early in fetal development, with involution of the pulmonary trunk and sixth arch derivatives, and persistence of primitive aortopulmonary connections. The morphology in case 1 is at odds with the theoretical division of pulmonary atresia with intact septum and pulmonary atresia with ventricular septal defect into two separate pathological entities that occur at different stages in fetal development.


Subject(s)
Collateral Circulation , Pulmonary Atresia/physiopathology , Female , Heart Septum , Humans , Infant , Infant, Newborn , Male
12.
Orv Hetil ; 135(42): 2305-7, 1994 Oct 16.
Article in Hungarian | MEDLINE | ID: mdl-7970644

ABSTRACT

Two infants with endocarditis were diagnosed at our Institute within a six month period. One infant died due to severe aortic valve destruction and ruptured sinus Valsalvae aneurysm both of which developed during fetal life. The other one with tricuspid valve endocarditis was successfully treated after the early echocardiographic diagnosis. To our knowledge this patient is the only survivor with neonatal endocarditis in our country.


Subject(s)
Endocarditis, Bacterial , Fetal Diseases , Endocarditis, Bacterial/diagnostic imaging , Fatal Outcome , Fetal Diseases/diagnostic imaging , Humans , Infant, Newborn , Ultrasonography
13.
Orv Hetil ; 135(44): 2425-9, 1994 Oct 30.
Article in Hungarian | MEDLINE | ID: mdl-7970663

ABSTRACT

Fourty homografts have been implanted for correction of congenital heart disease in children. Since 1992 cryopreserved homografts are used. Mid-term follow-up data are encouraging: owing to the improved procurement and preservation techniques and the cryopreservation a remarkably enhanced graft-survival is achieved. Preprocessing ischemic time reduction and cryopreservation are the foremost determinants for the freedom of reoperation. Despite the very strict donor selection criteria the capacity of our program (ie. the number of the homografts harvested and processed) seems to be increasing progressively. In order to meet the country-wide need for homografts the Hungarian Homograft Bank is to be established in cooperation with the other cardiac and transplant teams in Hungary. Its objectives, working standards, responsibilities and facilities are outlined.


Subject(s)
Heart Valves , Tissue Banks , Tissue and Organ Procurement/methods , Heart Valves/transplantation , Humans , Hungary , Transplantation, Homologous
15.
Int J Pediatr Otorhinolaryngol ; 24(2): 171-5, 1992 Sep.
Article in English | MEDLINE | ID: mdl-1428596

ABSTRACT

Epipharyngeal teratomas are rare congenital lesions that often cause respiratory distress, stridor at birth. The management of such anomalies should include establishment of secure upper airways, radiographic exclusion of midline CNS malformations and early surgical excision to prevent malignant transformation, asphyxia or permanent facial distortion. The diagnosis of congenital stridor, dyspnea and rhinitis should be performed very carefully.


Subject(s)
Nasopharyngeal Neoplasms/diagnosis , Respiratory Sounds/etiology , Rhinitis/etiology , Teratoma/diagnosis , Humans , Infant , Magnetic Resonance Imaging , Male , Nasopharyngeal Neoplasms/complications , Nasopharyngeal Neoplasms/pathology , Teratoma/complications , Teratoma/pathology
16.
Orv Hetil ; 133(30): 1893-5, 1992 Jul 26.
Article in Hungarian | MEDLINE | ID: mdl-1635776

ABSTRACT

After palliative procedure a 2 years old girl with congenital heart disease was corrected with aortic homograft. Truncus arteriosus is a rare congenital heart disease and this is the first successfully corrected case in our country. Using homograft in this age group improves the early and late result of this type of corrective surgery.


Subject(s)
Aorta, Thoracic/transplantation , Truncus Arteriosus, Persistent/surgery , Angiocardiography , Cardiac Catheterization , Child, Preschool , Echocardiography, Doppler , Female , Humans , Truncus Arteriosus, Persistent/diagnostic imaging
17.
Grud Serdechnososudistaia Khir ; (10): 6-9, 1991 Oct.
Article in Russian | MEDLINE | ID: mdl-1782038

ABSTRACT

Operation was carried out on 143 children (aged from 5 days to 16 years) for tracheobronchial obstruction caused by vascular rings. A double arch of the aorta was found in 50 patients, double arch of the aorta with atresia of the left arch--in 50 patients, right arch of the aorta with ligamentum arteriosum--in 30, left arch of the aorta with an aberrant right subclavian artery--in 10, and a pulmonary loop in 3 patients. Stridor was the main clinical manifestation of the vascular ring. The diagnosis was established by means of two-projection chest radiography and esophagography with a barium meal, bronchoscopy, and angiography. The surgical approach was gained through a posterolateral (left or right) thoracotomy. No deaths occurred during the operation. Two infants died in the postoperative period from a severe bacterial infection of the respiratory tract. On the basis of a rich clinical experience the authors came to the conclusion that the presence of a right aortic arch (except for cases with situs inversus) is always pathognomic because it either causes the appearance of a vascular ring without any intracardiac anomaly, or it may be combined with a congenital heart disease in which a vascular ring is usually not encountered (tetralogy of Fallot, truncus arteriosus communis, etc.). The authors never encountered atresia of a segment of the right aortic arch. The last-named was either patent or absent.


Subject(s)
Abnormalities, Multiple/surgery , Aorta, Thoracic/abnormalities , Pulmonary Artery/abnormalities , Abnormalities, Multiple/diagnostic imaging , Adolescent , Angiography , Aorta, Thoracic/diagnostic imaging , Aorta, Thoracic/surgery , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Pulmonary Artery/diagnostic imaging , Pulmonary Artery/surgery , Thoracotomy
18.
Pediatr Cardiol ; 12(1): 24-7, 1991 Jan.
Article in English | MEDLINE | ID: mdl-1845294

ABSTRACT

The echocardiographic features and management of 10 children with right heart thrombus (RHT) are presented. The patients' ages ranged from 2 weeks to 12 years. Three patients were less than 3 weeks old. The echocardiographic diagnosis was confirmed in five patients by surgery or autopsy. The thrombus was detected in the right atrium (five patients), in the right ventricle (three), on the tricuspid valve or within the inferior vena cava, the superior vena cava, or its extension following Senning's operation for transposition of great arteries (TGA). Seven patients had structural heart disease: TGA in four, tricuspid atresia in three. In two of three patients with anatomically normal heart, the thrombus occurred as a complication of a ventriculoatrial and a lymphovenous shunt system. The third patient had no predisposing factor. Five patients were treated with anticoagulants or platelet anti-aggregating agents and four by surgery. Seven patients survived and were followed by echocardiography.


Subject(s)
Echocardiography , Heart Diseases/diagnostic imaging , Thrombosis/diagnostic imaging , Anticoagulants/therapeutic use , Child , Child, Preschool , Heart Diseases/drug therapy , Heart Diseases/etiology , Heart Diseases/surgery , Humans , Infant , Infant, Newborn , Retrospective Studies , Thrombosis/drug therapy , Thrombosis/etiology , Thrombosis/surgery
20.
Int Urol Nephrol ; 17(1): 53-9, 1985.
Article in English | MEDLINE | ID: mdl-3922910

ABSTRACT

Neurofibromatosis of the urinary tract is estimated to account for 20 per cent of the visceral manifestations of the generalized disease. Among its urological forms involvement of the bladder is the most common. Clinically, neurofibromatosis resembles a tumour, therefore, its early histologic diagnosis is of utmost importance. In the present case a tumour involving the posterior bladder wall was found in a 6 years old boy subjected to examination because of a terminal haematuria. First the tumour was resected, later cystectomy with implantation of the distended ureter into the sigmoid was undertaken because of progression of the tumour. Postoperative hypokalaemia responded to the usual medication. Ureteral distension was reversed by the operation. Though neurofibromatosis (NF) is histologically a benign process, it tends to recur, is invasive, and impairs urinary functions. Therefore, it may endanger life and thus requires cystectomy with surgery for urinary diversion.


Subject(s)
Neurofibromatosis 1 , Urinary Bladder Neoplasms , Child , Cystoscopy , Humans , Male , Neurofibromatosis 1/diagnostic imaging , Neurofibromatosis 1/pathology , Neurofibromatosis 1/surgery , Radiography , Ureter/transplantation , Urinary Bladder Neoplasms/diagnostic imaging , Urinary Bladder Neoplasms/pathology , Urinary Bladder Neoplasms/surgery
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