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  • 1
    Electronic Resource
    Electronic Resource
    Social integration, sexual behaviour and fertility in patients with bladder exstrophy —a long-term follow up (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 678-683 
    Details
    ISSN: 1432-1076
    Keywords: Bladder exstrophy ; Interpersonal relations ; Sexual behaviour ; Fertility and education
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract After primary bladder closure or urinary diversion, other factors apart from the reconstruction itself gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility. Between 1968 and July 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime. Of the remaining 102 patients 48 attend school, 4 are in college, 40 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic centre and 6 are younger than 6 years of age. A total of 95% of the patients with continent urinary diversion are continent day and night, whereas only three of five patients with a sling plasty (incontinent epispadias) or with primary bladder closure followed by a Young-Dees procedure are continent. None of the patients showed deterioration of renal function. In 25 females the external genitalia were reconstructed. Fixation of the uterus was done in 13 to correct or prevent uterine prolapse. Of the 17 women older than 18 years of age with genital reconstruction, 16 are satisfied with the cosmetic result. All adults engage in sexual intercourse. Five women have delivered seven children by Caesarean section. Of the 35 male adults 32 underwent reconstruction of the external genitalia and 34 males achieve erection. One developed necrosis of the penis early in life following primary bladder closure performed at an outside hospital. Penile deviation was present in 11 of the 32 patients with genital reconstruction, which is distressing in only 2. Thirty patients are satisfied with the cosmetic result. After genital reconstruction 9 males developed epididymitis, necessitating two orchiectomies and three vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas ejaculation was normal in the three men who did not undergo genital reconstruction and in two patients prior to post-pubertal reconstruction. Furthermore, two of these three men have fathered four children.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957152
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  • 2
    Electronic Resource
    Electronic Resource
    Social integration, sexual behaviour and fertility in patients with bladder exstrophy – a long-term follow up (1996)
    Springer
    European journal of pediatrics 155 (1996), S. 678-683 
    Details
    ISSN: 1432-1076
    Keywords: Key words Bladder exstrophy ; Interpersonal relations ; Sexual ; behaviour ; Fertility and education
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract After primary bladder closure or urinary diversion, other factors apart from the reconstruction itself gain importance for individuals with the exstrophy-epispadias complex: social integration and, after reaching puberty, sexuality and fertility. Between 1968 and July 1994 115 patients with bladder exstrophy or incontinent epispadias underwent surgery at our institution. A total of 104 patients could be followed, 2 of whom died in the meantime. Of the remaining 102 patients 48 attend school, 4 are in college, 40 have completed or are currently undergoing vocational training, 3 are unemployed, 1 lives in a therapeutic centre and 6 are younger than 6 years of age. A total of 95% of the patients with continent urinary diversion are continent day and night, whereas only three of five patients with a sling plasty (incontinent epispadias) or with primary bladder closure followed by a Young-Dees procedure are continent. None of the patients showed deterioration of renal function. In 25 females the external genitalia were reconstructed. Fixation of the uterus was done in 13 to correct or prevent uterine prolapse. Of the 17 women older than 18 years of age with genital reconstruction, 16 are satisfied with the cosmetic result. All adults engage in sexual intercourse. Five women have delivered seven children by Caesarean section. Of the 35 male adults 32 underwent reconstruction of the external genitalia and 34 males achieve erection. One developed necrosis of the penis early in life following primary bladder closure performed at an outside hospital. Penile deviation was present in 11 of the 32 patients with genital reconstruction, which is distressing in only 2. Thirty patients are satisfied with the cosmetic result. After genital reconstruction 9 males developed epididymitis, necessitating two orchiectomies and three vasectomies. No patient with reconstruction of the external genitalia can ejaculate normally or has fathered children, whereas ejaculation was normal in the three men who did not undergo genital reconstruction and in two patients prior to post-pubertal reconstruction. Furthermore, two of these three men have fathered four children. Conclusion Education, occupation and social development of patients with urinary diversion are uneventful. The cosmetic results achieved by genital reconstruction are satisfactory. In female patients, antefixation of the uterus should be performed before or together with an introitusplasty to prevent uterine prolapse. In male patients, however, surgery is performed at the expense of fertility. Detailed discussions with the patients and their parents should include not only the question of primary bladder closure versus urinary diversion, but also the pros and cons of correction of the external and – in females – the internal genitalia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957152
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    T. Bergan (ed.) Infectiology, vol. 1 urinary tract infections (1997)
    Springer
    Infection 25 (1997), S. 380-380 
    Details
    ISSN: 1439-0973
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01740825
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  • 4
    Electronic Resource
    Electronic Resource
    Hypoparathyreoidismus und Innenohrschwerhörigkeit (1997)
    Springer
    Monatsschrift Kinderheilkunde 145 (1997), S. 347-352 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Hypoparathyreoidismus ; Innenohrschwerhörigkeit ; Renale Dysplasie ; Key words Hypoparathyroidism ; Deafness ; Renal dysplasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Three children with primary, nonfamilial hypoparathyreoidism presented with high-grade sensorineural deafness. Based on clinical and molecular evidence, one could be diagnosed as Kearns-Sayre syndrome. The basis of the association in the other cases remains unclear. The combination of symptoms seems to be more frequent than peviously assumed, affecting 3 of 9 children treated at our clinic because of primary, nonfamilial hypoparathyreoidism. Genetically determined dysfunction of cochlear haircell motility and haircell damage as consequence of reduced calcium concentrations in peri- and endolymph are discussed as pathogenetic factors of sensorineural deafness in cases of hypoparathyreoidism. Two of the children showed a marked renal dysplasia. Similar familial cases with associated kidney malformations have been reported in the literatur, indicating the possibiliy of a common cause for hypoparathyreoidism, sensorineural deafness and renal dysplasia. Discussion: As a practical consequence of our experience we recommend laboratory investigations (i. e. parathyreoid hormone) in order to exclude an underlying hypoparathyreoidism in patients with sensorineural deafness. Vice versa, an audiogram should be performed in patients with idiopathic hypoparathyreoidism for early recognition and treatment of associated sensorineural deafness.
    Notes: Zusammenfassung Es werden 3 Kinder mit nicht-familiärem Hypoparathyreoidismus beschrieben, die durch eine hochgradige Hörstörung cochleärer Genese auffielen. Während bei einem der Kinder klinisch und molekulargenetisch ein Kearns-Sayre-Syndrom nachgewiesen werden konnte, bleibt die Ursache des gemeinsamen Auftretens der Störungen bei 2 Kindern ungeklärt. Die Assoziation scheint häufiger als bisher angenommen: sie betrifft 3 der in der Kinderklinik Mainz betreuten 9 Kinder mit primärem Hypoparathyreoidismus. Genetisch determinierte Störungen der Haarzellmotilität der Cochlea sowie Haarzellschädigung als Folge chronisch verringerter Kalziumkonzentrationen in der Peri- und Endolymphe werden als pathogenetische Faktoren der sensorineuralen Hörstörung bei idiopathischem Hypoparathyreoidismus diskutiert. Beim Kearns-Sayre-Syndrom spielt der ATP-Mangel eine besondere Rolle und erklärt möglicherweise die Progredienz der Hörstörung trotz Normalisierung des Serumkalziumspiegels unter der Therapie mit 1,25-Dihydroxycholecalciferol. Bei 2 der 3 Patienten fand sich eine ausgeprägte renale Dysplasie. In der Literatur wurden ähnliche, familiäre Fälle mit assoziierter Nierenfehlbildung beschrieben, die auf eine gemeinsame Ursache von Hypoparathyreoidismus, sensorineuraler Hörstörung und renaler Dysplasie hinweisen. Diskussion: Die beschriebenen Fälle legen die Empfehlung nahe, im Rahmen der Abklärung einer Innenohrschwerhörigkeit einen Hypoparathyreoidismus auszuschließen. Umgekehrt sollte bei allen Patienten mit Hypoparathyreoidismus eine eingehende pädaudiologische Diagnostik zur rechtzeitigen Erfassung und Behandlung einer assoziierten Hörstörung erfolgen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050131
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  • 5
    Electronic Resource
    Electronic Resource
    Thrombotisch-thrombozytopenische Purpura mit akuter Niereninsuffizienz bei einem Knaben mit Lupus-ähnlicher Systemerkrankung (1997)
    Springer
    Monatsschrift Kinderheilkunde 145 (1997), S. 1176-1181 
    Details
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Thrombotisch-thrombozytopenische Purpura ; Hämolytisch-urämisches Syndrom ; Systemischer Lupus erythematodes ; Plasmapherese ; Key words Thrombotic thrombocytopenic purpura ; Hemolytic uremic syndrome ; Systemic lupus erythematosus ; Lupus-like-syndrome ; Plasma exchange
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A 139/12 year old boy presented with the clinical and laboratory signs of a systemic lupus erythematosus (SLE)-like illness. Three months later he developed acute renal failure associated with hemolytic anemia and thrombocytopenia while being treated with prednisone and a non-steroidal antiphlogistic agent. Kidney biopsy revealed endotheliotropic microangiopathy. A thrombocytopenic purpura/hemolytic uremic syndrome was diagnosed according to clinical and histological findings. Plasma exchange, hemodialysis and immunosuppressive therapy with prednisone and cyclophosphamide resulted in a normalization of the hematologic findings, and the kidney function improved. The cyclophosphamide therapy was continued over 21/2 years. Three years after the acute episode the boy is free of symptoms with glomerular filtration rate of 71 ml/min/1.73 qm. Discussion. The association of SLE with thrombotic thrombocytopenic purpura is rare, has predominantly been described in adults and has a high mortality rate. Our case demonstrates the success of an early aggressive therapy including plasma exchange, hemodialysis, prednisolone and cyclophosphamide.
    Notes: Zusammenfassung Ein 139/12 Jahre alter Knabe entwickelte 3 Monate nach Beginn einer Lupus-ähnlichen Systemerkrankung unter Prednisontherapie ein akutes Nierenversagen mit hämolytischer Anämie und Thrombozytopenie. Die Nierenbiopsie zeigte eine thrombotische Mikroangiopathie. Unter Behandlung mit Plasmaaustausch, immunsuppressiver Therapie und Hämodialyse normalisierten sich die hämatologischen Befunde, und die Nierenfunktion besserte sich. Die Cyclophosphamidtherapie wurde über 21/2 Jahre fortgesetzt. Drei Jahre nach dem akuten Nierenversagen ist der Junge unter antihypertensiver Behandlung symptomfrei und hat eine normale glomeruläre Filtrationsrate. Diskussion. Die Assoziation eines systemischen Lupus erythematodes mit einer thrombotisch-thrombozytopenischen Purpura ist in der Literatur bisher nur selten und fast nur bei Erwachsenen beschrieben worden; sie hat eine hohe Mortalität. Der dargestellte Fall demonstriert den Erfolg einer frühzeitigen aggressiven Therapie.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001120050216
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