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

Conditions that are often associated with an increased incidence of renal anomalies include imperforate anus, congenital vertebral abnormalities, and Fanconi anemia; excretory urography should be done if such a condition is present. Urography is also useful to provide baseline data in conditions associated with later development of urinary problems, such as myelodysplasia, prune-belly syndrome, and exstrophy of the bladder. In addition, urography serves as a periodic check for complications of treatment (hydronephrosis, obstruction) in patients with urinary diversion. Certain signs, eg, dribbling, hematuria after mild trauma, unexplained pneumothorax or pneumomediastinum in a neonate, and neonatal abdominal mass, call for immediate urography. In many conditions that were formerly thought to be associated with major urinary abnormalities, urography is not called for. Such is the case in hypospadias, deformities of the external ear alone, and undescended testes. Dehydration is the only absolute contraindication to urography.
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PMID:Urography in children: when should it be done? 2. Conditions other than infection. 71 36

We evaluated urodynamically and radiologically 54 children with myelodysplasia and neurological deficits at or below the S1 level. Baseline urodynamic testing was normal in 13 patients (24%), while 12 (22%) had an upper motor neuron and 13 (24%) had a lower motor neuron type of dysfunction. A total of 7 patients (13%) had a mixed upper and lower motor neuron type, and 9 (17%) had only lower motor neuron dysfunction of the urethral sphincter with a normally contractile bladder. Followup studies varying in time from 1 month to 10 years showed a changing neurourological lesion in 29 patients (54%): 25 deteriorated while 4 improved. Of the 54 children 20 had hydronephrosis and/or vesicoureteral reflux. Incontinence was the major problem in 41 patients (75%), and was managed initially with pharmacological agents and/or clean intermittent catheterization, with 9 of the 41 eventually requiring surgery. Urodynamic assessment reveals a variable picture that does not correlate well with the apparent neurological examination. Despite the low level of the neurological deficit, many children may be at risk for urinary tract deterioration. These findings emphasize the importance of continuous surveillance and appropriate management in this group of myelodysplastic children who have the greatest potential for a normal life.
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PMID:Urodynamic dysfunction in walking myelodysplastic children. 163 36

Bladder dysfunction in myelodysplasia may present a significant hazard to the upper tract and a threat to kidney function. Urodynamic features of high leak pressure and detrusor-sphincter dyssynergia have been associated with an increased risk. We have developed an objective score to describe urodynamic findings in myelodysplasia. The score includes consideration of bladder compliance, detrusor contractility and reflux, in addition to leak pressure and sphincter behavior. In 171 myelodysplastic patients a significant correlation was demonstrated between the score and upper tract studies at the time of urodynamics and the score and the management decision. Of 73 myelodysplasia patients with normal upper tracts at the first urodynamics study hydronephrosis later developed in 14. The score was a potent predictor of outcome (p = 0.0006).
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PMID:An objective score to predict upper tract deterioration in myelodysplasia. 199 4

Recent reports have suggested that the prophylactic use of clean intermittent catheterization in selected infants with myelodysplasia and with normal upper tracts may prevent their deterioration. The selection of these patients at risk depends on urodynamic criteria. We reviewed our experience with clean intermittent catheterization instituted when upper tract deterioration was found. Of 130 patients with myelodysplasia followed at our clinic 25 (19%) had or presented with radiological upper urinary tract deterioration. A total of 21 patients had vesicoureteral reflux and 4 had hydronephrosis without reflux. Treatment consisted of clean intermittent catheterization alone in 5 patients and combined with anticholinergic medication in 16. Four patients with no post-void residual urine were carefully observed without intervention. Urodynamic evaluation was performed selectively in 11 of these 25 patients for persistence of upper urinary tract deterioration or urinary incontinence after institution of treatment. Of the 21 children with reflux 19 (90%) had resolution or improvement for an average followup of 47 months. Hydronephrosis completely resolved in the 4 patients without reflux. Thus, the over-all resolution or improvement rate was 92% for an average of 41 months. Although these excellent results may not be superior to the prophylactic use of clean intermittent catheterization, they validate the assumption that upper tract deterioration can be reversed. The application of current urodynamic selection criteria may result in overtreating a significant number of infants who otherwise may not have upper tract deterioration. Further refinements in these criteria may be necessary to select better not only patients at risk but also to determine the frequency of imaging necessary in this urologically heterogeneous population. In addition, careful consideration should be given to urethral dilation as a third and equally viable alternative to prevent upper tract deterioration.
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PMID:Management of children with myelodysplasia: urological alternatives. 223 40

Periodic assessment of upper urinary tract anatomy and renal size is an important component in the urological management of children with myelodysplasia (spina bifida). As a correlation to a previous study of renal growth in children with spina bifida determined by excretory urography, we evaluated 297 renal ultrasonographic examinations in 145 patients with spina bifida and compared the renal size and growth pattern to those of normal children. Patients with known vesicoureteral reflux (grade 2 or greater), congenital renal anomalies, hydronephrosis, renal scarring or urinary tract surgery were excluded. Mean values and standard deviations for sonographically determined renal length were calculated. In general, mean renal length for each age group was below mean values for normal children. A normal renal growth curve for children with spina bifida, based on sonographic renal measurements, is developed for clinical use.
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PMID:Sonographic assessment of normal renal size in children with myelodysplasia. 304 39

Optimum urologic management of children with myelodysplasia includes periodic assessment of upper tract anatomy and renal growth. To investigate the perception that many children with spina bifida but without a history of intrinsic renal disease have small kidneys when compared with age-matched standard renal growth charts, 212 children with spina bifida were studied retrospectively, measuring renal length on excretory urograms. Patients with known vesicoureteric reflux of grade 2 or greater, congenital renal anomalies, hydronephrosis, renal scarring, or urinary diversion were excluded. For 95 patients aged 2 days to 19 days, 249 urograms were available for evaluation. Renal lengths were measured on the supine 5-min urogram. Mean values and standard deviations were calculated for each age group. Mean renal length for each age group fell below the mean of the standard curves devised by Hodson and Currarino, with increasing deviation from the mean with age.
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PMID:Normal renal growth in children with myelodysplasia. 348 82

Of 45 children with an artificial sphincter 6 had elevated filling pressure of the detrusor and/or uninhibited contractions of the bladder after the implantation surgery, which produced hydronephrosis in 3 and urinary incontinence in 3. All affected individuals had myelodysplasia (17 per cent of this subgroup), 3 had evidence of tethering of the spinal cord and 3 may have had unrecognized detrusor hypertonicity, which was masked by the low outlet resistance before sphincter implantation. Corrective surgery consisted of either decompressive laminectomy with untethering of the spinal cord or augmentation cystoplasty when indicated. Factors affecting the change in the detrusor postoperatively, and recommendations for improved selection and surveillance of individuals undergoing implantation of the artificial device are promulgated.
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PMID:Detrusor instability: a delayed complication in association with the artificial sphincter. 371 68

Hydronephrosis has been reported to be present at birth in 6 to 28 per cent of newborns with myelodysplasia. It is unknown whether this hydronephrosis is secondary to abnormal in utero voiding dynamics and is truly present at birth or whether it is acquired postnatally secondary to spinal shock after back closure. We reviewed the perinatal course of 47 myelodysplastic newborns and conclude that the true in utero incidence of hydronephrosis is lower than that suggested in the literature, and that the hydronephrosis seen in many of these newborns is acquired after back closure and is transient in nature.
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PMID:Implications of hydronephrosis in the newborn with myelodysplasia. 373 9

Early diagnosis and intervention in the child with myelodysplasia can effectively improve and preserve renal function in those newborns presenting with abnormalities at birth or who are at risk for deterioration of renal function from infection, vesicoureteral reflux and/or obstruction. During a 1-year period 10 newborns with myelodysplasia were seen. Hydronephrosis was present in 6, reflux in 3 and urinary tract infection in 3. In each newborn adequate decompression of the bladder and complete resolution of the hydronephrosis were achieved. Uroradiographic evaluation was helpful in determining the best mode of therapy for each individual.
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PMID:Hydronephrosis in the asymptomatic neonate with myelodysplasia. 683 3

To assess the preservation of sacral function despite denervation higher on the spinal cord in children with myelodysplasia, we examined 151 myelomeningocele patients presenting for urodynamic evaluation from 1980 to 1992 with a lesion at L3 or above on neurological examination. Of the 151 patients 70 (46%) had denervation of the external sphincter on initial evaluation and 81 (54%) had sacral sparing, that is normal or near normal electrical potentials of the external urethral sphincter and/or retained reflexic bladder activity regardless of the presence or absence of sacral reflexes. Of the 81 patients with sacral sparing 57 (70%) had detrusor-sphincter dyssynergia and 21 had synergy or denervation of the sphincter in association with retained detrusor reflexia. Whereas only 18% of children age 1 year or younger with retained sacral function had upper urinary tract deterioration on initial radiological studies (manifested by reflux or hydronephrosis), 57% had deterioration when evaluated after age 1 year. None of the patients without retained sacral function had evidence of upper urinary tract damage at younger than 1 year but 28% of those studied beyond age 1 year had changes. Patients with flaccid lower extremities can retain sacral function, which puts the upper urinary tract at risk of deterioration. Our results show that this risk increases with time.
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PMID:Retained sacral function in children with high level myelodysplasia. 760 77


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