Patients suffering from severe factor VII deficiency may present with serious bleeding problems. No clear guidelines exist regarding therapy in such patients in case of a large bleeding or surgery. Indeed, it has been postulated that some patients with severe factor VII deficiency may never present with overt bleeding problems. However, in factor-VII-deficient patients who have previously demonstrated a clinical tendency to bleed, surgery is expected to cause excessive bleeding. We present two females suffering from a severe factor VII deficiency (FVII:C < 0.01 U mL(-1) ) with a distinct history of haemorrhagic diathesis. Due to recurrent bleeding in the past, or for circumstantial reasons, surgery was demanded over a 4-year period on a total of seven occasions. To assist haemostasis during and after joint surgery on five occasions and for embolization and subsequent removal of a large haemangioma of the occipital region, recombinant factor VIIa (NovoSeven) was utilized in doses approximating 20 μg kg(-1) b.w. every 6 h beginning immediately before surgery and continued until 30 h to 13 days postoperatively, depending of the size of the respective procedure. Using this approach, we observed normal haemostasis, and there were no signs of excessive postoperative bleeding or wound haematoma. No adverse reactions or side-effects were observed, and there were no complaints or clinical signs indicative of thrombotic complications. As judged from the clinical course of these seven minor and major surgeries, recombinant factor VIIa appears to be highly efficaceous and safe in the treatment patients with severe factor VII deficiency undergoing surgery.
Fifty-six patients were investigated by repetitive cystometry in the supine or sitting position. The incidence of detrusor hyperreflexia and the circumstances under which it was found did not differ from previous studies. The cystometry data concerning volumes showed a remarkable degree of variability. Values for first sensation and maximal capacity could vary more than 500%. An increment from the first cystometry to the fourth was consistently found. The increments were most pronounced when cystometry with fast filling followed cystometry with medium filling regardless of the patient's position. The major factor in the changes is considered to be the patient's adaptation to the investigational situation. This finding might have major significance in the evaluation of drug studies where repeated cystometries with different time intervals are used.
Neurologically normal children with recurrent urinary tract infections (UTIs), night- and daytime wetting, and urge and painful voiding may have staccato voiding due to pelvic floor contractions. The immediate effect of non-invasive urodynamic biofeedback (BF) therapy was assessed using a historical follow-up study in 31 children aged 5-15 years suffering from urodynamically proven overactive urethra during voiding. A long-term follow-up study was performed to investigate whether improvement was maintained. Twenty-four children (77.5%) benefited from the treatment. Of these 16 (51.5%) were cured, while 8 (26%) had a pronounced reduction in their symptoms. Although the flow was normalized in 17 (55%) and nearly normalized in 7 (22.5%), there was no significant correlation between subjective and objective criteria of improvement. Similarly, no relationship was found between the initial urodynamic characteristics and the treatment outcome. During a mean follow-up time of 4 years (range: 1-7.5 years) two of the initially cured patients relapsed. They were recurred with a refresher course. Three had had a single or a few episodes of cystitis in the course of several years. Of the patients with pronounced reduction in their symptoms, three relapsed. A refresher course was attempted in two patients; one was successful. It can thus be concluded that BF is an effective way of treating this disturbance and the beneficial effect is to a wide degree maintained.
In adults and children suffering from urge, urge incontinence, frequent voidings, and nocturia, urodynamic examination often confirms the diagnosis of detrusor instability. We assessed the outcome of biofeedback therapy in 15 children aged 6-12 years and 7 adults aged 20-52 years, all with cystometrically proven detrusor instability. The detrusor pressure was visually conveyed to the patient during repeated bladder fillings. The patient was instructed to interrupt detrusor pressure increments by tensing the pelvic floor musculature. None of the children were found completely cured; however, 9 showed a marked decrease in either the number of the extent of symptoms. Two children showed moderate improvement, while 4 remained unaffected by the treatment. One adult was completely cured, 2 improved moderately, and 4 showed no improvement. None of these patients were converted to stable cystometry. Upon termination of the BF treatment a follow-up study was conducted for patients exhibiting pronounced or moderate improvement. The beneficial effect was maintained except in one case.
Recent studies have shown the occurrence of rhythmic urethral pressure variations (UPV) in the normal female urethra in the awake state. The nature of these variations still needs further elucidation. In this study we investigated the changes in urethral pressure (UP) and in UPV as they occur in healthy females awake and asleep. UPV, intravesical-(IVP) and rectal pressure (RP), together with anal electromyography (EMG) and electroencephalography (EEG) were measured in 10 healthy fertile female volunteers. The registration was done in the awake state, during falling asleep and during sleep. During falling asleep the average urethral pressure as well as the UPV amplitude and frequencies decreased and remained at a lower stable level during sleep. Urethral pressure and its variations seemed to be interrelated since the UPV frequency and amplitude decreased when the average UP decreased. From this study it appears that urethral pressure is influenced by extrinsic factors such as the waking, resting and sleeping states. Although some extrinsic modulation of urethral pressure variations occurs, their persistance in sleep suggests their local nature.
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