Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A consecutive series of 90 children with anorectal malformations was reviewed for urologic problems related to lower urinary tract dysfunction. Neurogenic bladder-sphincter dysfunction was seen in 22 patients (24%), all but one of whom had sacral agenesis. Vesicoureteral reflux was present in 60% of the patients with dysfunction, and 32% had reflux nephropathy. Urinary incontinence was present in 91% of the patients who had bladder-sphincter dysfunction. However, the management of bladder-sphincter dysfunction and urinary incontinence was not as straightforward as for patients with myelodysplasia because the parents were more reluctant to accept the therapeutic measures. It was particularly difficult to introduce clean intermittent catheterization (CIC), especially in older patients, because genital and urethral sensation often was undisturbed. Only if CIC had been started in the neonatal period or early infancy were there no problems with acceptance and parental compliance. The authors emphasize the importance of urodynamic testing of neonates and infants who have an anorectal malformation and associated sacral agenesis in identifying those who have neurogenic bladder-sphincter dysfunction. Consequently, patients with lower urinary tract dysfunction should receive prompt treatment, including CIC if necessary, to prevent or reduce secondary urologic morbidity, especially loss of renal function.
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PMID:Urologic problems in anorectal malformations. Part 2: functional urologic sequelae. 886 70

An original Russian drug pikamilone with central (vegetotropic, vascular) and peripheral (vasodilation) mechanism of action and m-cholinolytic atropine were investigated for effect on detrusor function in the accumulation phase in 26 children with neurogenic bladder due to myelodysplasia. The detrusor function was assessed at retrograde cystometry performed before and after the drugs administration. The cystotonograms were estimated using a computer program of Relief urosystem according to the standard and additional indices. Pikamilone was found to potentiate the effects of atropine. This may be due to improvement of circulation and reduction of detrusor hypoxia. It is stated that selective chemotherapy of neurogenic bladder is uneffective. The preference should be given to combination of the drugs affecting main components of its dysfunction pathogenesis. Pikamilone should be referred to basic drugs for treatment of neurogenic bladder in spinal patients as its combination with atropine possesses a unique efficacy in restoration of the reservoir function of the bladder.
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PMID:[Validation of the treatment of the neurogenic bladder in children with myelodysplasia using pikamilon]. 957

We report a case of a bladder-wall abscess in a patient with myelodysplasia and a neurogenic bladder. The aetiology of the abscess was obscure, but the patient had recurrent urinary tract infections and was being managed by clean intermittent catheterisation.
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PMID:Bladder-wall abscess in a girl with neurogenic bladder. 1066 66

Spina bifida and myelodysplasia are associated with neurogenic abnormalities of the bladder and bowel function. All children with myelodysplasia require an evaluation of their urinary tract with ultrasound and urodynamics to confirm normal bladder and kidney function. Patients with anatomical and functional abnormalities require treatment, the mainstay being intermittent catheterization and anticholinergic medication. The treatment goals for patients with a neurogenic bladder are the preservation of the upper urinary tract, bladder and bowel continence, independence, autonomy, and facilitation of self-esteem. A minority of children will not respond to conservative therapy and will ultimately require surgical intervention. This review will discuss the surgical options for bladder augmentation, bladder neck reconstruction and closure, as well as the methods for the creation of continent catheterizable stomas. The timing, indications, and description for each procedure will be addressed. Finally, the antegrade continence enema procedure will be described for the management of refractory fecal incontinence.
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PMID:Surgical management of the neurogenic bladder and bowel. 1574 70

Symptomatic ejaculatory duct (ED) calculi, typically composed of uric acid, carbonate apatite and calcium phosphate, or calcium phosphate in the form of hydroxyapatite, are rare occurrences. We report a case of bilateral, large ED calculi in a patient with spina bifida myelomeningocele and an augmented neurogenic bladder. A 25-year-old Caucasian male, not compliant with his urological management, presented with abdominal pain, difficulty in self-catheterization, nausea and vomiting. Two of eight large struvite calculi, which blocked the urethra, were identified at the right ED. After endoscopic calculi removal, further management included bladder irrigation and infection control modalities. This case highlights the importance of clean intermittent catheterization, bladder irrigation, and routine urologic management necessary for patients with myelodysplasia and neurogenic bladder. It is the first recorded case to demonstrate the augmented bladder as an initiator of ejaculatory duct calculi in patients with an open bladder neck and spastic external sphincter.
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PMID:Obstructive ejaculatory duct calculi in a patient with bladder augmentation and myelomeningocele. 2097 Mar 84

This article provides a comprehensive summary of the clinical approach, investigative modalities and management of a child with neurogenic bladder disease due to myelodysplasia. It is aimed at pediatric physicians and surgeons working in developing nations. The methodologies suggested are simple and can be practised even in resource poor regions. The goal of management is avoidance of Chronic kidney disease and for this, meticulous bladder management is the key.
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PMID:Management of Neurogenic Bladder. 2855 89

Myelodysplasia includes a group of developmental anomalies resulting from defects that occur during neural tube closure. Urological morbidity in patients with myelodysplasia is significant and if not treated appropriately in a timely manner can potentially lead to progressive renal failure, requiring dialysis or transplantation. We report the case of a 13-year old girl with neurogenic bladder who presented chronic renal failure secondary to lipomyelomeningocele with retethering of cord. She was managed with urinary indwelling catheterization until optimization of renal function and then underwent detethering of cord with excision and repair of residual lipomeningomyelocele. Her renal parameters improved gradually over weeks and then were managed on self clean intermittent catheterization. The case emphasizes the need for considering retethering of spinal cord in children with myelodysplasia where symptoms of neurogenic bladder and recurrent urinary tract infections occur.
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PMID:Chronic Renal Failure Secondary to Unrecognized Neurogenic Bladder in A Child with Myelodysplasia. 2869 33


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