Complications of intravesical oxybutynin chloride therapy in the pediatric myelomeningocele population

PURPOSE: We report our experience with the intravesical administration of oxybutynin chloride with particular focus on the incidence and characterization of untoward effects and inconvenience of therapy. MATERIALS AND METHODS: From 1990 to 1995, 23 children 5 to 11 years old with myelodysplasia were treated with intravesical oxybutynin chloride. Initial dose was 1.25 mg. in 5 cc sterile water instilled during routine catheterization 3 times daily, which was increased as tolerated and clinically indicated. Oral anticholinergic, antispasmodic and sympathomimetic medications were discontinued during therapy. We reviewed therapeutic indications, doses, frequency duration, reason for discontinuation and untoward effects. Patients/parents were surveyed for convenience of treatment as well as side effects and their timing with respect to drug administration and dose. RESULTS: In 15 patients (65%) treatment was discontinued and oral formulations were resumed or other therapy was required due to side effects, ineffectiveness or inconvenience. Seven patients had untoward effects, ranging from facial flushing and dizziness to agoraphobia and hyperactivity. Six patients discontinued therapy due to side effects after 1 day to 2 years (mode 1 month) at doses of 1.25 to 5 mg., including 5 who previously had side effects from oral oxybutynin chloride. Inconvenience of therapy was noted irrespective of the degree of independence of the child for performing intravesical therapy. CONCLUSIONS: Untoward effects and inconvenience are the most common reasons for discontinuing intravesical oxybutynin chloride therapy for neurogenic bladder dysfunction. Children who previously had side effects from oral oxybutynin chloride are more likely to have them during intravesical therapy.     

Follow-up of long-time treatment with intravesical oxybutynin for neurogenic bladder in children

Functional imaging of a language task using positron emission tomography was performed as part of the preoperative assessment of a patient with a left supplementary motor area (SMA) tumor. Positron emission tomography scans were obtained during language tasks (verb generation and word reading of visually presented nouns) that normally lead to increased blood flow in the SMA relative to a control condition (visual fixation). In the patient, the normal SMA response was an order of magnitude larger in the region of the tumor. Other regions, such as left inferior frontal cortex and right cerebellum, showed equivalent activation in the patient and normal subjects. Histopathologic study revealed an anaplastic astrocytoma. Thus, this exaggerated vascular response to local neuronal activation occurred in the setting of a proliferation of glial cells. This is consistent with models of coupling of regional CBF and neuronal activity that implicate glia as the mediator between neurons and vasculature. The concept that tumoral disruption of normal vascular responses could, in some cases, potentially enhance rather than dampen the response is proposed.     

Pharmacokinetics and effects of intravesical oxybutynin on the paediatric neurogenic bladder

We have recently shown that the Ca2+ response in endothelial cells evoked by readdition of Ca2+ to the medium after store depletion caused by a submaximal concentration of agonist can involve Ca2+ release from Ca2+ stores sensitive to both inositol 1,4, 5-trisphosphate and ryanodine. The present experiments were performed to determine whether this mechanism might also exist in other types of cell. For this purpose, we used the human carcinoma cell line A431, which has a varied resting [Ca2+]i. We found that the amplitude of the Ca2+ response evoked by Ca2+ readdition did not correlate with the amplitude of the preceding UTP-evoked Ca2+ release, but did positively correlate with the initial [Ca2+]i. An inspection of the two patterns of response seen in this study (the large biphasic and small plateau-shaped Ca2+ responses) revealed that there is an accelerating rise in [Ca2+]i during the biphasic response. Application of ryanodine during the plateau-shaped Ca2+ response reversibly transformed it into the biphasic type. Unlike ryanodine, caffeine did not itself evoke Ca2+ release, but it caused a further [Ca2+]i rise when [Ca2+]i had already been elevated by thapsigargin. These data suggest that in A431 cells, as in endothelial cells, the readdition of Ca2+ after agonist-evoked store depletion can evoke Ca2+-induced Ca2+ release. This indicates that Ca2+ entry may be overestimated by this widely used protocol.     

Possibilities of ambulation in children with myelodysplasia]

This article reviews four reports that have addressed the efficacy of spinal instrumentation to enhance spinal fusion in patients with degenerative spondylolisthesis. These papers serve to highlight some of the common problems with interpreting and applying the clinical findings of such studies: small, nonuniform patient selection; variable instrumentation techniques; relatively brief follow-up periods (usually less than 5 years); lack of a uniform scale for clinical assessment of patients; and lack of consensus on the definition and clinical documentation of motion segment instability.     

Outcome analysis of vesicoureteral reflux in children with myelodysplasia

PURPOSE: Vesicoureteral reflux in children with myelodysplasia is usually secondary to abnormal bladder storage. The purpose of this study was to assess the outcome of vesicoureteral reflux in children with myelodysplasia. MATERIALS AND METHODS: We retrospectively analyzed the records of 319 children with myelodysplasia who presented to our institution between 1978 and 1985. Of these children 95 presented with or had reflux during followup and they were treated with prophylactic antibiotics. Clean intermittent catheterization and anticholinergic medication were added to the regimen when indicated. RESULTS: Reflux resolved in 63% of these patients with nonsurgical management. Temporary cutaneous vesicostomy was performed in 23 children (24%) with persistent high grade reflux or evidence of upper tract deterioration. Ureteral reimplantation and augmentation cystoplasty were performed in 18 (20%) and 8 (8%) patients, respectively. No patient had progression to chronic renal failure and scars developed in only 14 kidneys (10%). CONCLUSIONS: In the majority of cases (63%) reflux resolved with nonsurgical management. Reflux in these patients should not be treated in isolation. The management of reflux is primarily aimed at improving bladder storage. The combination of aggressive nonsurgical therapy and close observation is recommended. This regimen leads to the satisfactory resolution of reflux with minimal renal morbidity.     

Magnetic resonance detection of myelodysplasia in children with Currarino triad

PURPOSE: To evaluate the role of MRI in the detection of myelodysplasia in children with Currarino triad. MATERIALS AND METHODS: Six patients (two girls, four boys, aged 7 months-14 years, mean age 6 years) were studied with MRI, voiding cystourethrogram and barium enema or fistulography. CT and ultrasonography were also performed in two patients. RESULTS: All patients presented with partial agenesis of the sacrum. Three patients suffered from an intermediate form of anorectal malformation (ARM) and three had a high form of ARM. The presacral masses consistent with Currarino triad included anterior meningocoele in three patients, lipoma in two patients and anterior lipomeningocoele in one patient. MRI diagnosed tethering of the spinal cord in four of six patients. The tethering of the spinal cord was due to a lipomeningocoele in one patient, an intradural lipoma in one patient and a lipoma of the filum in two patients. CONCLUSION: The association of Currarino triad with tethered spinal cord seems more common than generally reported in the literature. Preoperative MRI of the lumbosacral spine is essential to detect significant myelodysplasia in all patients with Currarino triad.     

Long-term follow-up of fascia lata transfer for the paralytic hip in myelodysplasia

We evaluated the results of fifty-eight fascia lata transfers and anterior hip releases in thirty-three myelodysplastic patients with a minimum follow-up of 10.9 years. The procedure does not appear to achieve hip stability or prevent recurrent flexion deformity. Pelvic obliquity occurs secondary to scoliosis and results in increased instability of the hip on the high side and ischial decubitus ulcers on the low side. The degree of scoliosis and pelvic obliquity is related to the level of neural involvement. Similarly, ambulatory status is dependent on neural function and is not affected by instability of the hip.     

Long-term follow-up of cytostatic intravesical instillation in patients with superficial bladder carcinoma. Is short-term, intensive instillation better than maintenance therapy?

Change of road user attitudes is often advocated as a necessary condition for improvement of road safety. The relation between road user attitudes and accident risk is, however, not well known in road safety research. The objective of this study was to find out whether attitudes are of importance to road safety, i.e. the hypothesis is that drivers having attitudes considered correct according to the traffic code, have lower accident risk than other drivers. The attitudes of a representative sample of Norwegian driver's license holders as well as their accident involvement and driving distance were surveyed by mail questionnaires in two phases. When no other factor is taken into account, accident risk is found to be affected by driver attitudes. When, on the other hand, the age of drivers is taken into account, the relation between attitudes and accident risk disappears. It is concluded that age and annual mileage are more important to accident risk than are attitudes, and more knowledge of the relationship between attitudes and road accident risk is needed.     

Long-term follow-up of combined therapy with large-dose zinc sulfate and low-dose penicillamine in children with hepatolenticular degeneration

This was a prospective study designed to evaluate the extent to which intestinal mucosal compromise occurs in adult critical care patients with and without systemic inflammatory response syndrome (SIRS) and to correlate the degree of intestinal injury with outcome. Ten patients from a university hospital surgical intensive care unit were identified who manifested SIRS at the time of admission to the intensive care unit. Five other critical care patients without SIRS were also evaluated. The Acute Physiology and Chronic Health Evaluation II score was determined. Intestinal mucosal viability was assessed by serial measurement of serum and urine iFABP intestinal fatty acid binding protein (iFABP), a sensitive and specific marker for mucosal injury. Outcome in terms of the development of multiorgan dysfunction syndrome, adult respiratory distress syndrome, and survival was determined. iFABP was detectable in the serum or urine in 8 out of 10 patients with SIRS. Among the 4 patients with detectable serum iFABP, 2 died and 1 developed severe adult respiratory distress syndrome. Nine of 11 patients without detectable serum iFABP recovered without major morbidity. iFABP was detectable in most patients with SIRS, suggesting that subclinical intestinal mucosal compromise is a frequent component of this syndrome. When iFABP was detectable, particularly in the serum, the prognosis was poor, even in the absence of SIRS, indicating that iFABP may be a relevant and independent predictor of outcome in critical care patients.     

Long-term therapy of bronchial asthma with ingacort

To study the effectiveness of ACE-inhibitors in diabetic nephropathy (DN) 12 male and 16 female patients aged 13-21 years with DN having normal blood pressure (BP) were given ramipril (tritace) in a dose 2.5-5 mg/day in the course of 12-24 weeks. Efficacy and safety of the treatment were assessed by changes in albuminuria and proteinuria, BP. Reduction of albuminuria occurred in 19(79.1%) out of 24 patients with microalbuminuria, in 13(54.1%) of them urine excretion of albumin returned to normal levels. All the patients with proteinuria and macroalbuminuria benefited from ramipril therapy because their proteinuria diminished or even disappeared (2 cases). The persistence of the antiproteinuria effect on posttreatment week 12 was 66.6%. Ramipril effect on BP was minimal. It is inferred that ramipril is effective in the treatment of DN at the stage of microalbuminuria and proteinuria in patients with normal BP.     

Long-term outcome and prognostic determinants in children with hypertrophic cardiomyopathy

OBJECTIVES: We sought to determine clinical, angiographic, and echocardiographic predictors of survival in children with isolated hypertrophic cardiomyopathy (HCM) in a large pediatric centre. BACKGROUND: Sudden death is a catastrophic outcome of HCM in childhood but has been difficult to predict. Current therapies might provide for improved outcome if factors identifying high risk can be identified. METHODS: Records of 99 patients diagnosed with HCM from 1958 to 1997 at <18 yr were reviewed for clinical, angiographic (n = 62) and echocardiographic (n = 83) predictors of survival outcome. The effects of clinical characteristics on sudden death (including resuscitated sudden death) were individually tested in Cox's proportionate hazard modeling. RESULTS: Seventy-one subjects were male. Median age at diagnosis was 5.0 yr with a medical follow-up interval of 4.8 yr. Thirty-seven of 97 patients had a family history of HCM. Ambulatory electrocardiograms (ECG) in 78 patients demonstrated supraventricular tachycardia in 16 and ventricular tachycardia in 21. Death or resuscitated sudden death occurred in 18 patients. Sudden death rate was 2.7%/yr after age 8 yr. Cox's proportionate survival modeling revealed increased corrected QT interval (QTc) dispersion on ECG (relative risk [RR] 1.61 per 20 ms increment, p < 0.0003), ventricular tachycardia (VT) on ambulatory ECG (RR 3.75, p < 0.006) and myocardial bridging of the LAD coronary (RR 12.0, p < 0.003) to be associated with reduced time to death or resuscitated sudden death. CONCLUSIONS: Detailed assessment of ECGs, ambulatory ECGs, and coronary angiography can assist in identifying which children with HCM are at risk for sudden death.     

Long-term home oxygen therapy

Trichomonas vaginalis was diagnosed in 42 (19%) of 227 adult males with urethral discharge. In 27 men (15%) T. vaginalis was isolated together with Neisseria gonorrhoeae. Non-gonococcal urethritis was diagnosed in 15 patients and T. vaginalis was isolated from 47% of such patients. Stained smear preparations, i.e. RapiDiff and acridine orange of modified Diamond's media, were superior to wet smear microscopy for the identification of T. vaginalis. RapiDiff stain was the most sensitive and identified 41 of 42 (98%) positive cultures. It is recommended that all turbid culture media should be stained for the optimal diagnosis of trichomoniasis.     

Behavior therapy with aggressive children

Modern behavior therapy strategies which reduce aggressive behavior are based on developmental psychopathological assessments. These help to confirm intervention steps adapted to each specific age group. The extensive spectrum of methods in child behavior therapy offers different cognitive and social elements which are used to help the child effectively. The training for aggressive children by PeTERMANN and PeTERMANN (1994) which generally includes behavior-oriented family counselling shall be considered.     

Long-term intravenous tocolytic therapy

Eighteen women required continuous intravenous tocolytic therapy with either ritodrine hydrochloride or magnesium sulfate for greater than 48 hours because of repetitively recurrent preterm labor; these were compared with a similar group of women successfully treated in less than 48 hours in a retrospective, case-controlled study. The mean gestational age at the time of diagnosis was 31 weeks for both groups. Tocolytic selection was similar in both groups, although the dosage per hour was significantly greater with long-term therapy. The mean interval from initiation of therapy until delivery was 41 days in the study group, compared with 39 days among controls (not statistically significant). The mean gestational age at delivery was 36 weeks in both groups. There were no significant difference in various measures of fetal outcome between groups. These data demonstrate that long-term intravenous tocolytic therapy can be a safe and effective means of prolonging gestation in those women who fail to respond to conventional treatment.     

Endoscopic treatment of vesicoureteral reflux in patients with myelodysplasia

PURPOSE: We assessed the usefulness of and indications for endoscopic treatment of vesicoureteral reflux in myelodysplasia patients. MATERIALS AND METHODS: A total of 26 patients treated with intermittent catheterization was divided into 11 (16 ureters) with and 15 without vesicoureteral reflux. In 9 patients (13 ureters) endoscopic correction was performed with 3% atelo-collagen and without anesthesia at the outpatient clinic. In each ureter we obtained the sum of scores for 4 risk factors for upper urinary tract deterioration: bladder compliance less than 10 ml./cm. water, grade 2 to 3 bladder deformity, detrusor-sphincter dyssynergia and urethral closure pressure 50 cm. water or greater. RESULTS: No reflux was demonstrated immediately after the initial collagen injection but cystography 3 to 6 months later showed recurrent reflux in 5 ureters (38%). Repeat injection cured the reflux, with results persisting for an average of 17 months. Mean risk factor score for patients without vesicoureteral reflux was significantly lower than that for patients with reflux. In patients treated with intermittent catheterization and anticholinergic agents the mean score for ureters with an increased or unchanged reflux grade was significantly greater than for those with a decreased grade. CONCLUSIONS: Endoscopic treatment of reflux appears to be safe and useful in patients with myelodysplasia. The treatment is preferable in those with high risk factor scores due to the possibility of increased reflux grade in such patients.     

Values in behavior therapy with children.

Contends that psychotherapy is a process of social influence and cannot be value-free. The stages of therapy are examined, and recommendations for dealing with children in therapy at specific clinical stages (e.g., entrance into treatment, goal selection, and termination) are included. (32 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Long-term captopril therapy in asymptomatic patients with severe chronic aortic insufficiency

BACKGROUND: Clinical history of patients with severe chronic aortic regurgitation is characterized by a long period without any symptom, although the left ventricle enlarges progressively. The administration of oral vasodilators could reduce ventricular enlargement or its progression, delaying the development of myocardial dysfunction and/or the need for valvular surgery. OBJECTIVES: To verify the efficacy of long-term captopril therapy to reduce left ventricular mass and dimensions in patients with severe chronic aortic regurgitation. METHODS: This is a prospective echocardiographic study in which each individual patient is considered his own control case. Eleven asymptomatic patients with severe chronic aortic regurgitation in sinus rhythm, who had an ejection fraction greater than 50% and were not taking cardiovascular drugs, were orally administered captopril at the maximum tolerated dosage (127 +/- 13 mg/day). Follow-up lasted for 24 +/- 3 months. RESULTS: Left ventricular telediastolic diameter decreased from 69 +/- 5 to 61 +/- 3 mm (p < 0.01), telesystolic diameter decreased from 48 +/- 5 to 41 +/- 4 mm (p < 0.01); ejection fraction increased from 56 +/- 4 to 61 +/- 3% (p < 0.001); myocardial mass decreased from 208 +/- 32 to 174 +/- 27 g/m2 (p < 0.01), and mean wall stress from 264 +/- 35 to 203 +/- 25 mmHg (p < 0.001). All variations were still significant at 6 months. CONCLUSIONS: These results suggest that captopril has a favourable effect on left ventricular mass, dimensions and load conditions, and could favourably influence the natural history of chronic aortic regurgitation. The efficacy of medical treatment can be verified through serial echocardiographic study.     

Long-term psychosocial and adaptive outcomes in children with arteriovenous malformations.

Objective: To describe the long-term social-emotional and adaptive functioning of a small sample of children and adolescents with repaired arteriovenous malformations (AVMs). Participants: Five children (11-15 years of age) with AVMs in the cerebellum and frontal, temporal, and parietal lobes. Main Outcome Measures: Vineland Adaptive Behavior Scales, Behavior Assessment System for Children, Parent Stress Inventory, Children's Depression Inventory, Revised Children's Manifest Anxiety Scale, and a clinical interview. Results: Although overall emotional functioning appeared adequate, there were suggestions of defensiveness and areas of worry. Global adaptive functioning was below average for most children, and parents have concerns about their child's socialization. Conclusion: These children are adapting variably, with more positive than negative findings. The results can help guide clinicians to generate effective rehabilitation strategies to improve the quality of life of children with AVMs. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Long-term follow-up study of vigabatrin in pretreated children with West syndrome

The purpose of this study was to test the comparative efficacy and toxicity of empiric gentamicin and ciprofloxacin, in combination with piperacillin, in febrile patients with treatment-induced neutropenia. Fifty patients were prospectively randomized to receive piperacillin plus gentamicin (PG), and 46 were randomized to receive piperacillin plus ciprofloxacin (PC). The groups were similar in age, sex, diagnosis, duration of neutropenia, and incidence of positive cultures. The two antibiotic regimens were associated with comparable rates of defervescence in the patients with gram-positive bacteremia. In the patients with gram-negative bacteremia and those with negative cultures, however, defervescence was more prompt in the PC group. In particular, 27% of the culture-negative patients on PC, compared to only 5% of those on PG, defervesced within 72 hr (P = 0.015). Because of the more prompt defervescence in the PC group, amphotericin B was used less frequently; 78% of the patients on PG compared with only 56% of those on PC were started on amphotericin B (P = 0.025). PC is an effective alternative to the more traditional PG for treatment of febrile neutropenic hosts who have not been given prophylactic quinolones. More important, PC appears to hasten defervescence compared with PG, especially in culture-negative patients and those with gram-negative bacteremia, and may decrease the necessity of additional antimicrobial agents such as amphotericin B.