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Biomedical subjects

J D van Gool

Publications and source records attributed to J D van Gool.

At least 19 recordsLinked to original sources
PubMed1995

The effect of posterior sagittal anorectoplasty and its variants on lower urinary tract function in children with anorectal malformations.

The effect of posterior sagittal anorectoplasty (PSARP) and its variants on lower urinary tract function was investigated urodynamically in 32 patients with anorectal malformations. In 27 patients urodynamic evaluation was performed before and after surgery and in 4 it was done postoperatively only. One child was clinically assessed with no postoperative urodynamic study. Minor postoperative changes in the specific preoperative urodynamic pattern were observed in 4 cases but the changes did not seem to be related to surgery. In 3 boys with rectourethral fistulas detrusor failure consistent with autonomic denervation was noted postoperatively. Standard posterior sagittal anorectoplasty was performed in 1 of the 3 boys and posterior sagittal anorectoplasty combined with additional transabdominal procedures was done in the other 2. In general our findings suggest that posterior sagittal anorectoplasty and its variants do not affect lower urinary tract function unless these surgical techniques are combined with major transabdominal procedures and extensive retrovesical dissection.

Anal Canal↗
PubMed1994

Lower urinary tract dysfunction in children with benign sacrococcygeal teratoma.

We evaluated 8 girls and 3 boys with benign sacrococcygeal teratoma for lower urinary tract dysfunction. All children underwent ultrasonography of the urinary tract, voiding cystourethrography and at least 2 standard urodynamic evaluations. Mean patient age at first urodynamic study was 59 months (range 1 to 136). Nine of the 11 children had abnormal studies, including detrusor instability and associated pelvic floor overactivity during micturition in 2, an anatomical infravesical obstruction in 2 and neurogenic bladder-sphincter dysfunction in 5 (2 detrusor hyperreflexia with sphincter dyssynergia, 2 hyporeflexic bladder-sphincter function and 1 detrusor hyporeflexia with normal sphincter function). Although the major cause of neurogenic lower urinary tract dysfunction seems to be related to surgical trauma of the pelvic plexus and sacral nerves, we observed tethered spinal cord as a primary cause in 2 patients. The incidence of voiding dysfunction in children with sacrococcygeal teratoma is high and is usually neurogenic in origin.

Abdominal Neoplasms↗
PubMed1994

Urodynamic evaluation of children with the caudal regression syndrome (caudal dysplasia sequence).

A total of 50 children with the caudal regression syndrome (caudal dysplasia sequence) underwent urodynamic evaluation of lower urinary tract function. Of the patients 15 had isolated sacral agenesis, 13 had sacral agenesis in combination with an anorectal malformation and 22 had an anorectal malformation without sacral anomaly. Neurogenic bladder-sphincter dysfunction was observed in 11 of the 15 children with isolated sacral agenesis (73%) and in 10 of the 13 children with combined sacral agenesis and anorectal malformation (77%). The pattern of neurogenic bladder-sphincter dysfunction in both groups did not differ significantly. In the group of 22 children with isolated anorectal malformation only 1 had signs of neurogenic bladder-sphincter dysfunction. These results indicate that anorectal malformation per se does not influence lower urinary tract function and that the sacral anomaly is the determining factor for neurogenic bladder-sphincter dysfunction in children with anorectal malformation.

Abnormalities, Multiple↗
PubMed1992

Historical clues to the complex of dysfunctional voiding, urinary tract infection and vesicoureteral reflux. The International Reflux Study in Children.

The prevalence of nonneuropathic bladder/sphincter dysfunction was assessed with a questionnaire in 310 of the 386 children enrolled in the European branch of the International Reflux Study in Children. Despite the exclusion criteria (neuropathic bladder, anatomical malformations other than vesicoureteral reflux and overt dysfunctional voiding or urge incontinence), the prevalence of bladder/sphincter dysfunction was as high as 18%. Four patterns of dysfunction emerged: urge syndrome, staccato voiding, fractionated and incomplete voiding, and voiding postponement. The questionnaire proved helpful in detecting low profile cases of bladder/sphincter dysfunction, as well as indicating the need for further urodynamic studies. A strong correlation was established between recurrences of urinary tract infections, as well as disappearance of vesicoureteral reflux (negative correlation) and nonneuropathic bladder/sphincter dysfunction. This finding implies that detection and treatment of bladder/sphincter dysfunction are essential in every child with the complex of recurrent urinary tract infection and vesicoureteral reflux.

Child↗
PubMed1992

Functional daytime incontinence: clinical and urodynamic assessment.

Assessment of children with daytime wetting starts with the distinction between 'enuresis diurna' and 'functional incontinence', incontinence being defined as any form of wetting caused by bladder/sphincter dysfunction. Standard history-taking does not allow for a sharp enough distinction: pertinent questions have to be asked about daytime wetting, night-time wetting, micturition, and about urge and reactions to urge. By using urodynamics to expose the pathophysiology behind the patterns of bladder/sphincter dysfunction, these questions were formulated and validated in a series of 156 children, referred with persistent daytime wetting to a programme for cognitive bladder training. With history-taking organized into a simple questionnaire, complemented by urodynamics, four patterns of bladder/sphincter dysfunction emerged: urge syndrome, staccato voiding, fractionated and incomplete voiding, and lazy bladder syndrome. A strong correlation was found between recurrent urinary tract infections and non-neuropathic bladder/sphincter dysfunction, implying that detection and treatment of bladder/sphincter dysfunction is essential in every child with recurrent urinary tract infections, especially in the presence of vesico-ureteral reflux.

Adolescent↗
PubMed1992

Functional daytime incontinence: non-pharmacological treatment.

In children with 'functional incontinence', defined as any form of (daytime) wetting caused by non-neuropathic bladder/sphincter dysfunction, most signs and symptoms are rooted in habitual non-physiological responses to signals from bladder and urethra. These responses develop at toddler age, when children learn how to remain dry. Once they have become a habit, incomplete bladder emptying and recurrent urinary tract infections come into play, reiterating the non-physiological responses into fixed patterns of bladder/sphincter dysfunction with functional incontinence as the leading symptom. Non-pharmacological treatment of functional incontinence implies relearning and training the normal responses to signals from bladder and urethra: a cognitive process, with perception of the signals reinforced by biofeedback. This type of treatment is best combined with long-term chemoprophylaxis. Severe cases will benefit from anticholinergic drugs, as adjuvants to the training programme. Urodynamics play a crucial role in documenting the specific patterns of incontinence and in providing biofeedback. For a successful programme, psychological screening of the children is indispensable.

Adolescent↗
PubMed1991

[Effect of intermittent catheterization on urinary tract infections and incontinence in children with spina bifida].

Although the primary objective of clean intermittent (self) catheterization (CIC) was to regain continence in (older) children with myelomeningocele (MMC), we used the method also in the very young with detrusor-sphincter dyssynergia, to prevent future damage from the combined effects of obstruction and urinary tract infection (UTI). The results, in terms of incidence of UTI and degree of incontinence before and during CIC, were assessed in 61 children with MMC who had started CIC between 1985 and 1988. Mean follow up was 45.3 +/- 27.3 months, mean age at the start of CIC was 6.9 +/- 4.6 years. The incidence of UTI during CIC proved significantly lower than before CIC (chi 2 = 20.92, p less than 0.001), and the improvement in incontinence was also statistically significant (chi 2 = 45.16, p less than 0.001). In children with MMC, CIC seems ideally suited to prevent damage to the kidneys from the life-long problem of recurrent UTI's. It will also help in preventing bladder wall fibrosis--an important cause of loss of detrusor compliance--secondary to repeated UTIs.

Adolescent↗
PubMed1989

Intra-uterine cystography for evaluation of prenatal obstructive uropathy.

To evaluate the risk for kidney damage in a male fetus with obstructive uropathy, a percutaneous bladder puncture was performed at 26 weeks gestational age and contrast was injected into the fetal bladder. A clear picture was obtained of the bladder with marked widening of the prostatic urethra and posterior urethral valves, and massive bilateral vesicoureteral reflux could be demonstrated. By adding cysto-urethrography to a diagnostic puncture of the fetal bladder it could be proved that the dilatation of the upper urinary tracts was caused by high-pressure vesico-ureteral reflux incurring progressive damage to the renal parenchyma.

Female↗
PubMed1981

5-oxoprolinuria due to hereditary 5-oxoprolinase deficiency in two brothers--a new inborn error of the gamma-glutamyl cycle.

Two brothers, aged 16 and 11 years, had recurrent episodes of vomiting, diarrhoea and abdominal pain, starting in infancy. In spite of extensive investigations no cause of their enterocolitis could be established. After several years symptomatic treatment was discontinued without any recurrence of symptoms. Their father and several paternal relatives have had kidney stones. Both boys developed urolithiasis and an oxalate-containing stone was removed from the elder brother's kidney. He had no hypercalciuria. His glomerular and tubular function tests were normal. Gas chromatography of urine from both brothers revealed massive excretion of L-5-oxoproline (pyroglutamic acid). Glutathione levels in erythrocytes of both patients were normal. The activities of enzymes of the gamma-glutamyl cycle were analysed in erythrocytes, leukocytes and cultured skin fibroblasts. The level of glutathione synthetase was normal, as was the affinity of this enzyme for its substrate gamma-glutamyl-cysteine. Feedback inhibition of gamma-glutamyl-cysteine synthetase by glutathione was also normal. Both patients had a specific deficiency of 5-oxoprolinase, the activity of which was 2-4% of that of control subjects. Their parents had intermediate 5-oxoprolinase activities in fibroblasts, indicating a recessive mode of inheritance. Thus, 5-oxoprolinuria in these two patients was due to a lack of 5-oxoprolinase, i.e., a new inborn error in the gamma-glutamyl cycle.

Adolescent↗