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Query: UMLS:C0026986 (myelodysplastic syndrome)
14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A new technique is described which enables children with neurogenic bladder secondary to myelodysplasia to learn self-catheterization prior to their school years. Inexpensive dolls, complete with male or female genitalia, and a specially designed mirror to magnify the female children's genitalia, were used with four children (three girls, one boy) with mental ages of five years. Each child successfully demonstrated self-catheterization within two days, and gradual progress toward complete independence has been promoted.
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PMID:Teaching intermittent self-catheterization to young children with myelodysplasia. 46 19

In a retrospective study, the pre-operative urodynamic findings of 41 myelodysplastic patients who had undergone enterocystoplasty for intractable incontinence or deteriorating upper urinary-tracts were compared with findings from similar studies. An objective score combining five urodynamic parameters, the Hostility Score, was calculated before and after surgery to assess its clinical applicability in guiding and monitoring patient management. A score of greater than or equal to 5 should be avoided, if possible, as deleterious upper urinary-tract changes can occur even while on conservative management. In this series the mean pre-operative Hostility Score was 5.6, with 26 patients having a mean score of greater than or equal to 5. After surgery the mean score was reduced to 2.8, with no patient having a score of greater than 4. We conclude that the Hostility Score is a reliable tool for the management and follow-up of patients who have neurogenic bladder disease as a result of myelodysplasia.
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PMID:Evaluation of augmentation cystoplasty for severe neuropathic bladder using the hostility score. 159 97

In children with myelodysplasia and a low-level spinal cord lesion (low lumbar-sacral), detrusor hyperactivity together with dyssynergic urethral function forms the main pathophysiological basis for incontinence. Pharmacological treatment of incontinence due to neurogenic bladder dysfunction has been tried, mainly with anticholinergics and alpha-adrenoceptor antagonists. In this study, the effects of the alpha-adrenoceptor agonist phenylpropanolamine on urodynamic parameters and incontinence were investigated in 10 patients. Effects on incontinence were evaluated in a double-blind crossover trial. Plasma concentrations of phenylpropanolamine were measured by means of gas chromatography-mass spectrometry. Phenylpropanolamine reduced detrusor hyperactivity and improved continence, but the effect was not so pronounced as to make the patients continent.
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PMID:Effect of phenylpropanolamine on incontinence in children with neurogenic bladders. A double-blind crossover study. 160 97

Ninety-seven children with neurogenic bladder and myelodysplasia were subjected to complex examination. The somatic innervation of the sphincter urethrae externus and sphincter ani muscles was appraised by the method of sacral guided reactions by a CT-01 electromyograph and by an original method consisting in registration of the reflex response of the named anatomical structures according to the fluctuations of the intraurethral and intraanal pressure. It was established that maintained, partly disturbed, and absolutely absent somatic innervation of the sphincters of the bladder and rectum is encountered in patients with myelodysplasia. The more the innervation of the sphincters disturbed is, the more the urinary bladder is excluded from the "accumulation-evacuation" urodynamic cycle and the lesser the probability of disorders of urodynamics of the upper urinary tract is, and vice versa. The obtained information is important in choosing the therapeutic tactics for patients with a neurogenic bladder.
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PMID:[Electrophysiologic diagnosis of neurogenic bladder in children with myelodysplasia]. 194 83

Myelodysplasia represents the most common cause of neurogenic bladder dysfunction in children. The specific histological features associated with myelodysplastic bladders have not been previously characterized. Our objective was to study the relationship between smooth muscle and connective tissue in control and myelodysplastic bladders using classical morphometric analysis with the assistance of an automated image analysis system. Gross histological analysis of the bladder specimens of normal stillborn fetuses showed organized muscle bundles embedded in a small amount of connective tissue. The bladder specimens of myelomeningocele stillborn fetuses showed a marked paucity of muscle bundles as well as a significantly diminished size of the muscle bundles. The myelomeningocele bladder specimens obtained from patients undergoing autopsy and those undergoing augmentation cystoplasty revealed significant interfascicular and pericellular infiltration of the smooth muscle by dense connective tissue. Quantitative morphometric analysis showed that the myelomeningocele stillborn fetuses have a significant increase in the volumetric content of connective tissue compared to control stillborn fetuses. The bladders of myelomeningocele patients who underwent autopsy or augmentation cystoplasty had a 3-fold increase in connective tissue when compared to normal controls. These findings reveal that structural changes in the histological components of the myelodysplastic bladder can be demonstrated not only in patients of varying ages undergoing autopsy or augmentation cystoplasty but also in the developing fetus. These findings enhance our understanding of the relationship of connective tissue proliferation to smooth muscle in the myelodysplastic bladder. We discuss the relationship of these findings to pathological detrusor morphology and detrusor dysfunction.
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PMID:Bladder wall abnormalities in myelodysplastic bladders: a computer assisted morphometric analysis. 201 85

During the last 3 years 20 patients (median age 8 years) have undergone construction of a continent urinary reservoir at our pediatric urology service. Of the patients 12 had failed exstrophy reconstruction, 7 had myelodysplasia with a neurogenic bladder and 1 had extensive bladder damage as a result of a previous operation. In 5 patients a continent reservoir was constructed after cystectomy performed in early childhood. Techniques of reconstruction included the Mitrofanoff procedure (4 patients), a modification of the Indiana pouch (12), a modification of the Benchekroun procedure (2), the use of the appendix in situ as a continence mechanism (2) and a modified Kropp procedure using a duplicate vagina for catheterization (1). The morbidity from these procedures was acceptable and consisted primarily of chronic diarrhea (4 patients), stone formation within the pouch (5) and the need to revise the continence mechanism (7). All but 1 patient maintained stable or improved upper tracts after diversion. Over-all, 85% of the patients experienced diurnal continence on an intermittent catheterization regimen, while 75% were dry at night. The plicated/intussuscepted ileal nipples required revision because of difficulty with catheterization (7 patients) and urinary leakage. We currently favor the use of the Benchekroun or Mitrofanoff techniques for the ease of construction and minimal requirements for revision.
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PMID:Continent urinary reservoirs in pediatric urological practice. 237

The experience of 2 surgeons working independently at separate institutions is reported to demonstrate the use of the Kropp urethral lengthening and implantation procedure. This experience includes 18 patients between 6 and 19 years old (median age 11.6 years) with neurogenic bladder dysfunction. The etiology of the neuropathic bladder was myelodysplasia in 16 patients and sacral agenesis in 2. There were 10 boys and 8 girls. All patients had failed trials of clean intermittent catheterization with adjunctive pharmacological manipulation. Incontinence was a significant social problem. Of the 18 patients 2 had undergone prior urinary diversion and the Kropp procedure was used as part of undiversion. One patient had had 2 previous failed attempts at continence using an artificial urinary sphincter. Augmentation cystoplasty was an adjunctive maneuver in 14 patients; ileum was used in 8, sigmoid in 4 and the ileocecum in 2 (both of whom had been diverted previously). The bladder capacity in the 4 patients in whom augmentation was not performed ranged from 200 to 450 ml. Of the 18 patients 17 achieved a good result and 14 were considered to have achieved an excellent result. The 1 failure is the patient who previously had failed to gain control with an artificial sphincter. Of the 4 patients who did not undergo bladder augmentation 3 required adjunctive anticholinergics to achieve continence.
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PMID:Experience with the Kropp anti-incontinence procedure. 270 4

Clean, intermittent catheterization was instituted in 38 babies with myelodysplasia who were thought to be at risk for upper urinary tract deterioration because of neurogenic bladder dysfunction. There were 19 patients 2 weeks to 12 months of age, 11 were 1 to 2 years of age, and 8 were older than 2 years. Effectiveness of clean, intermittent catheterization was determined by maintenance of upper urinary tract stability. Upper urinary tracts improved or remained stable in 13 of 16 infants (81%) with reflux and 16 of 18 infants (89%) with detrusor-sphincter dyssynergia. Bacteriuria was present in 16 (42%), with only 2 infants (5%) having a febrile episode; no infant required hospitalization because of urinary tract infections. No further complications were identified in infants who were cleanly and intermittently catheterized. Most families found clean, intermittent catheterization of their infants easy to master and not stressful, and their children adjusted to it at an early age.
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PMID:Clean, intermittent catheterization of infants with neurogenic bladder. 274 Jan 79

From 1978 to 1985, 57 myelodysplasia patients with urinary and defecatory dysfunction underwent surgical treatment by modified seromuscular ileal flap fixation to the bladder. Followup was 1 to 88 months. Bladder capacity did not decrease, and voiding time and urine flow rate significantly improved. A urinary substitute sensation appeared in 45 of 46 patients (97.8 per cent) and urinary incontinence improved in 36 of 37 (97.3 per cent). A fecal substitute sensation appeared in 31 of 46 patients (67.4 per cent) and constipation improved in 22 (47.8 per cent). Operative complications were encountered in 5 of 57 patients (8.8 per cent), including 3 cases of prolonged paralytic ileus, 1 obstructive ileus and 1 wound herniation. Modified seromuscular ileal flap fixation to the bladder appears to be indicated for patients with the lower type of neurogenic bladder with neither a low compliance bladder nor high grade vesicoureteral reflux.
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PMID:A study of functional recovery for urination and defecation in patients with myelodysplasia: a modified seromuscular ileal flap fixation to the bladder. 291 47

Prostatic abscess is an unusual occurrence in the era of modern antibiotics. In 5 patients with myelodysplasia extravasation of contrast material to the boundaries of the prostate was noted on voiding cystourethrography. All patients were adolescents who, with varying degrees of compliance, had managed the neurogenic bladder with intermittent catheterization since childhood. All patients had chronic urinary tract infections. In 2 patients subjected to transurethral resection the prostatic parenchyma was completely replaced by the suppurative process. Neither patient demonstrated much clinical improvement after resection. The possible etiological factors and various therapeutic options are discussed for the previously unreported condition of chronic prostatic abscess in myelomeningocele patients.
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PMID:Prostatic suppuration and destruction in patients with myelodysplasia: a newly recognized entity. 317 48


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