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Biomedical subjects

Christopher R J Woodhouse

Publications and source records attributed to Christopher R J Woodhouse.

18 recordsLinked to original sources

Congenital adrenal hyperplasia and lower urinary tract symptoms.

OBJECTIVES: To assess urinary symptoms in adult women with congenital adrenal hyperplasia (CAH), as feminizing surgery in infancy is current standard practice for CAH and one of the indications for surgery is to reduce urinary symptoms. PATIENTS, SUBJECTS AND METHODS: In a case-control study, 19 women with CAH, of whom 16 had had childhood feminizing genital surgery, and age-matched women with no CAH, were evaluated. Subjects and controls completed the Bristol Female Lower Urinary Tract Symptoms (BFLUTS) questionnaire. RESULTS: Urge incontinence was reported in 13 (68%) patients and three (16%) controls (P = 0.003); stress incontinence was present in 47% and 26%, respectively (P = 0.31). Results from the controls were comparable with those documented in larger studies on normal populations. Nine of the patients felt that their urinary symptoms had an adverse effect on their lives, compared with only one of the controls (P = 0.008). CONCLUSION: Patients with a diagnosis of CAH are more likely to have significant urinary symptoms than normal controls. At present it is not clear whether this is a result of surgery or an effect of CAH. In at least two-thirds of patients surgery did not achieve the objective of reducing urinary symptoms.

Adolescent↗

The pitfalls of vaginal construction.

OBJECTIVE: To evaluate a group of young women undergoing complex vaginoplasty, which may be defined as where there has already been previous complex pelvic surgery and the patient is unsuitable for conservative treatment or minor surgical revision. PATIENTS AND METHODS: All patients who had undergone a complex vaginoplasty between 1997 and 2004 were reviewed retrospectively. Information was recorded on the underlying condition, previous surgery and preoperative assessment. The operative technique and complications were noted, and the outcome in terms of menstruation and sexual activity. In all, 15 patients fulfilled the criteria (mean age 22.2 years, range 17-36) and the mean follow-up was 4.75 (0.75-7) years. RESULTS: Fourteen patients had complex congenital anomalies of the lower genital tract and 11 of these had associated anomalies of the urinary and gastrointestinal system; four were XY females. The 15 patients had had 31 vaginal procedures, including ileal vaginoplasty, mobilization of perineal skin flaps and split-skin grafting. Three patients required osteotomies to increase pelvic outlet diameter. The commonest complications were stenosis (six patients) and fistula formation (four). Eight patients are now sexually active; the remaining seven have not attempted sexual intercourse. CONCLUSIONS: Complications are common and can be major; most patients require many repeat operations to achieve a patent vagina. Unfortunately at present, this is the only option for this group of young women to achieve a functional vagina. Patients with complex congenital anomalies of the reproductive and genital tracts are increasingly surviving into adulthood, and the numbers of these patients is likely to increase in the future.

Adolescent↗

Renal outcome in adults with renal insufficiency and irregular asymmetric kidneys.

BACKGROUND: The commonest cause of end-stage renal failure (ESRF) in children and young adults is congenital malformation of the kidney and urinary tract. In this retrospective review, we examine whether progression to ESRF can be predicted and whether treatment with angiotensin converting enzyme inhibitors (ACEI) can delay or prevent this. METHODS: We reviewed 78 patients with asymmetric irregular kidneys as a consequence of either primary vesico-ureteric reflux or renal dysplasia (Group 1, n = 44), or abnormal bladder function (Group 2, n = 34). Patients (median age 24 years) had an estimated GFR (eGFR) < 60 ml/min/1.73 m2 with at least 5 years of follow up (median 143 months). 48 patients received ACEI. We explored potential prognostic factors that affect the time to ESRF using Cox-regression analyses. RESULTS: At start, mean (SE) creatinine was 189 (8) mumol/l, mean eGFR 41 (1) ml/min 1.73 m2, mean proteinuria 144 (14) mg/mmol creatinine (1.7 g/24 hrs). Of 78 patients, 36 (46%) developed ESRF, but none of 19 with proteinuria less than 50 mg/mmol and only two of 18 patients with eGFR above 50 ml/min did so. Renal outcome between Groups 1 and 2 appeared similar with no evidence for a difference. A benefit in favour of treatment with ACEI was observed above an eGFR of 40 ml/min (p = 0.024). CONCLUSION: The similar outcome of the two groups supports the nephrological nature of progressive renal failure in young men born with abnormal bladders. There is a watershed GFR of 40-50 ml/min at which ACEI treatment can be successful at improving renal outcome.

Adolescent↗

Linear growth after enterocystoplasty in children and adolescents: a review.

The interposition of bowel in continuity with the urinary tract has allowed for the preservation of renal function and continence in children with bladder exstrophy, as well as neurogenic and valve bladders. Although bladder augmentation with ileum or colon has been shown to be safe, the long-term effects of metabolic acidosis in addition to abnormalities in linear growth and bone metabolism remain largely unknown. We reviewed the literature to critically examine linear growth in children who have had bladder augmentation with a particular emphasis on the correlation between acid-base status, bone mineralization and growth. The majority of studies suggest that linear growth is not affected by bladder augmentation. In the short-term, children post-augmentation have varying degrees of metabolic acidosis which, overtime, appears to resolve with no affect on linear growth. In a single study, bladder augmentation led to significant bone demineralization almost a decade after surgery, however, even in these children no decrease in linear growth was noted. No alterations in bone density levels were seen with short-term follow-up.

Acid-Base Equilibrium↗

Urolithiasis in enterocystoplasties.

The incidence of stones in patients with enterocystoplasty is reported as 12-52.5%. Most patients will have multiple physical factors such as immobility, need for self catheterisation and poor urine drainage, so that it is not certain that an intestinal reservoir is the cause of stones on its own. There is little or no evidence that mucus is an aetiological factor. Foreign bodies in the reservoir, such as staples, increase the risk of stone formation from 13% to 43%. Stones require surgical removal. Minimally invasive techniques may be used for small stones. A low velocity disintegrator is required so that fragments are not propelled into the intestinal mucosa. Stones are infective in origin in 86% of cases, but 14% are sterile. Metabolic screen shows that 80% of patients have risk factors for at least three different types of stone. All patients have raised pH (mean 6.93) and hypocitraturia. Raised serum and urinary calcium, hyperoxaluria and hyperuricosuria are found in up to 33% of patients.

Humans↗

Bladder, bowel and bones--skeletal changes after intestinal urinary diversion.

Impaired bone metabolism following urinary diversion through intestinal segments has always been a controversial subject of unclear clinical relevance. Whereas the perpetuated pathophysiological considerations seem conclusive in theory, the role of acidosis and malabsorption is less clear in animal experimentation and, even more so, in the clinical reality of modern continent diversion. In hardly any of the available contemporary case series was overt derangement of the acid-base balance, rickets or osteomalacia encountered. No consistent changes in osteotropic serum parameters could be found with normal calcium and phosphate in all patients. The assumption that colonic reservoirs have a higher risk of developing metabolic bone disease could not be confirmed by clinical data. As early correction of base excess is easy and probably a common policy in patients with intestinal urinary reservoirs, it will be virtually impossible to further study the natural history of bone metabolism after urinary diversion. While there is no need for a bone specific follow-up in asymptomatic adults with a normal acid-base balance, particular attention should be paid to children and to all patients with impaired renal function.

Acidosis↗

Renal transplantation in adults with abnormal bladders.

Since January 1, 1985, we have performed 73 renal transplants in 66 patients with abnormal bladders who had end-stage renal failure as a consequence of urologic abnormalities (mean age 32 years). Their outcome is compared with 58 renal transplants in 54 patients (mean age 40 years) who had renal failure from primary vesicoureteric reflux or renal dysplasia and whose bladder function was considered to be normal. There is no difference in actuarial graft survival in the two groups at 10 years (abnormal 66%, normal bladders 61%), although longer follow-up is showing an advantage for normal bladders, with a kidney half-life of 29 to 33 years compared with 15 years for the abnormal bladder group. Similarly, actuarial patient survival at 10 years is 86% in both groups. Current renal function is better in the group with normal bladders. At latest follow-up, the abnormal, unaugmented bladder group (n=34) has been followed for 92 (87) months (mean [median]) and has a plasma creatinine of 178 (161) micromol/L, whereas the normal bladder group (n=33) has been followed for 104 (93) months and has a creatinine concentration of 143 (140) micromol/L. A strict policy, since 1991, of prophylactic antibiotics for the first 6 months has halved the subsequent incidence of urinary tract infection. Urinary tract infections only produced problems in patients with abnormal bladders. Renal transplantation into the abnormal lower urinary tract is successful but requires careful preoperative evaluation and posttransplant follow-up.

Adult↗

The ambitions of adolescents born with exstrophy: a structured survey.

OBJECTIVE: To determine the factors that control quality of life as perceived by adolescent patients with bladder exstrophy, and to compare their views using standard instruments. PATIENTS AND METHODS: Sixteen patients (mean age 19 years, range 16-21, 11 male and five female) were recruited from the departmental database; they represented 46% of those available for the study. After giving informed consent, each had a semi-structured interview, augmented by completing a self-reported scale, with the principal investigator. They then completed the Culture-Free Self-Esteem Inventory-2 (CFSEI-2) and the Brief Symptom Inventory (BSI). The interviews were recorded on audiocassette, transcribed verbatim and evaluated using interpretative phenomenological analysis. RESULTS: In the interviews there was a remarkable consistency in the domains identified as important to the patients. There was a wish to be normal and to be treated as such. This was defined (amongst other items) as being able to void with an appropriate noise, being treated as peers at school and at home, and having an umbilicus. All patients reported some bullying (all but one in the past), but only severely in three. Concerns about self-image centred on scars and genital appearance. Very similar and effective coping strategies had been created, including practical (e.g. suitable clothes) and emotional (e.g. joking, control of revealed information) aspects. Special arrangements made to help (care by a special assistant or use of a disabled lavatory at school) served only to emphasize their abnormality and were resented. No overt psychiatric or psychological morbidity was detected. There was no difference in scores with the CFSEI-2 or BSI from established age-related norms. CONCLUSION: This study confirms the anecdotally reported strong resilience and personality of adolescents with exstrophy. The domains that patients considered important were not those that their carers might have expected or that are used in standard quality-of-life instruments. No morbidity was identified by the two instruments used. In exstrophy, and perhaps in other uncommon conditions, the patients' views of relevant domains should be considered in assessing quality of life.

Adaptation, Psychological↗

The management of haemorrhagic cystitis with sodium pentosan polysulphate.

OBJECTIVE: To assess the use of sodium pentosan polysulphate (SPP) for haemorrhagic cystitis (HC), a potentially life-threatening side-effect in patients treated with pelvic radiotherapy or cyclophosphamide, and which can be difficult to manage as patients often have significant comorbidity. PATIENTS AND METHODS: Between September 1991 and December 2000, 60 consecutive patients (24 women and 36 men) with haemorrhagic cystitis were primarily treated with SPP; 53 patients had had radical radiotherapy for pelvic malignancy and seven systemic cyclophosphamide. All patients were screened for blood dyscrasia and residual/primary urothelial malignancy with imaging, urine cytology and cystoscopy. RESULTS: In all, 51 patients were available for follow-up; the median (range) interval between completing treatment and developing haematuria was 4.5 (0.08-39.4) years, the duration of treatment 180 (21-1745) days and patients were followed for 450 (19-4526) days from the onset of haematuria. All patients were started on SPP at an initial dose of 100 mg three times daily. In 21 patients the dose was gradually reduced to a maintenance dose of 100 mg and in 10 further patients SPP was stopped because the haematuria stopped completely. Twenty patients died while on treatment from causes not directly related to their haematuria. CONCLUSION: We recommend the use of SPP as the primary method of managing haemorrhagic cystitis associated with pelvic radiotherapy or systemic chemotherapy.

Anti-Inflammatory Agents, Non-Steroidal↗

The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study.

BACKGROUND: The effects on sexual function of surgical removal of parts of the clitoris are unknown. For infants with intersex conditions and ambiguous genitalia being raised female, this surgery is often undertaken in early childhood. Our aim was to assess the effects of surgery on sexual outcome in this population. METHOD: We did a cross-sectional study to which we recruited 39 adults who had intersex conditions with ambiguous genitalia who were living as female from clinical (n=15) and peer-support (n=24) settings. We obtained data by use of a postal questionnaire, incorporating a validated sexual function assessment inventory. We also obtained hospital notes of 36 respondents who did not want to remain anonymous, and did genital examinations of 19 women. We assessed sexual problems in relation to surgical history and compared the results for our population to those of a healthy control group. FINDINGS: Of the 39 individuals enrolled, 28 had been sexually active and all had sexual difficulties. The 18 women who had undergone clitoral surgery had higher rates of non-sensuality (78%) and of inability to achieve orgasm (39%) than did the ten who had not had surgery (20% [p=0.002] and 0% [p=0.03], respectively). INTERPRETATION: Sexual function could be compromised by clitoral surgery. Debate on the ethics of the use of this surgery in children should be promoted and further multicentre research is needed to ensure representative samples and comprehensive outcome assessment. Meanwhile, parents and patients who consent to clitoral surgery should be fully informed of the potential risks to sexual function.

Adolescent↗

Diagnosis of perforated enterocystoplasty.

In the operation of enterocystoplasty, now widely practised, segments of bowel are used to augment or replace the urinary bladder. An occasional complication is perforation, and this may present in non-specialist settings. We investigated the management of spontaneous perforations among 264 patients with enterocystoplasty followed by one surgeon for 2-18 years. Patients' charts were examined for data on presentation, diagnosis and treatment. 10 patients had thirteen perforations; data were available for nine perforations in 9 patients. Mean time from enterocystoplasty to perforation was 45 months. Presentation was shoulder pain in 1 and abdominal pain (with or without fever) in 8. Perforation was diagnosed without delay in 3, but the initial diagnosis was urinary tract infection in 4 and small-bowel obstruction in 2. Ultrasound was the most useful investigation being diagnostic in 6 of 7 cases; contrast cystography showed a leak in only 2 of the 6 patients in whom it was performed. Treatment was successful in 8 cases (surgery 6; percutaneous drainage 2); 1 patient, who remained undiagnosed, was treated medically and died. Patients with enterocystoplasty need to be educated about this potentially lethal complication, so that they can alert non-specialist clinicians to what may have happened. In any patient with enterocystoplasty who reports abdominal pain or shoulder pain, perforation has to be ruled out.

Abdominal Pain↗