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Biomedical subjects

D I Williams

Publications and source records attributed to D I Williams.

At least 55 records · Page 3Linked to original sources
PubMed1980

Patterns of renal damage in the management of vesical exstrophy.

We reviewed the patterns of pyelonephritic damage that occurred during surgical management of bladder exstrophy. Renal injury patterns indicate that papillary configuration and obstruction coupled with urinary infection were the primary factors in producing damage. Diversion by the ileal conduit resulted in frequent injury, whereas the colon conduit was associated rarely with renal damage. The incidence of renal injury from bladder closure and continence attempts suggested closure as a reasonable course, especially in view of present continence results.

Bladder Exstrophy↗
PubMed1980

Familial male pseudohermaphroditism due to deficiency of 5 alpha-reductase.

Two brothers of Greek Cypriot origin, karyotype 46 XY, aged 17 and 19 years were brought up as females because of predominantly female external genitalia with clitoromegaly and urogenital sinus. At puberty there was genital masculinization with testicular enlargment and descent and development of male musculature and body habitus without gynaecomastia. Both subjects acquired male gender identity and changed from a female to a male gender role. The internal genitalia were normally formed with an ejaculate containing mature spermatozoa. In both subjects plasma testosterone levels were slightly elevated, dihydrotestosterone was in the low normal range and the plasma testosterone to dihydrotestosterone ratio was markedly elevated. Plasma oestrogens were normal whereas SHBG binding capacity was elevated. The urinary 5 beta-aetiocholanolone to 5 alpha-androsterone ratios were elevated compared with normal subjects. Basal plasma LH was normal but, the LH response to LHRH was exaggerated and basal and peak plasma levels of FSH were elevated. 5 alpha-reductase activity in genital skin homogenates was higher when androstenedione was used as a substrate, compared with testosterone, suggesting a decreased affinity of the enzyme for testosterone. The clinical and hormonal features in the two siblings are consistent with an impaired peripheral conversion of testosterone to dihydrotestosterone due to deficiency of the enzyme 5 alpha-reductase.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗
PubMed1980

Variants of the exstrophic complex.

In the split symphysis variant, a rare anomaly of bladder exstrophy, the bladder is closed with varying degrees of skin covering it. A good prognosis does exist for surgically produced continence in patients with a single bladder, although patients with bladder duplication have a greater risk of renal damage.

Bladder Exstrophy↗
PubMed1979

Blood-pressure screening and supervision in general practice.

Since April 1975 all men aged 35-69 years registered with four general practices in west central Scotland have had their blood pressure checked whenever they visit the surgery. Although the practice locations range from rural to city centre and observers comprise receptionists, nurses, and doctors, a standard procedure has been adopted for the examination, recording, follow-up, and management of high blood pressure. The results confirm that raised blood pressure is common and often goes undetected. Even when hypertension is known, casual blood pressure readings often exceed accepted normal levels. The findings also show that a population may be routinely examined through normal contact with the family doctor, and that this can provide a convenient, acceptable, and effective means of detecting and reducing raised blood pressure.

Adult↗
PubMed1979

Minimal surgical interference in the prune belly syndrome.

Twenty-seven cases of prune belly syndrome which were well in the neonatal period are reported. Eleven were seen in the first year of life and 16 presented later. The first line of treatment was to ensure proper bladder emptying, usually by urethrotomy. Thereafter a policy of minimal surgical interference was followed, operating only for proven obstruction and intractable infection. This policy appears justified as 10 of 11 early presenters have done well. In the late presenters it was accepted that some renal damage had already occurred; nonetheless, 11 of 16 cases have done well. The X-rays were reviewed and hitherto unreported medullary cysts were noted. No radiological signs were found that were of prognostic significance.

Abdominal Muscles↗
PubMed1979

Duplications of the lower urinary tract in children.

Seven cases of bladder duplication are described, and compared with 27 cases of urethral duplication. Bladder duplication was collateral while urethral duplication was usually in the sagittal plane. The conditions may coexist when the duplication of the bladder extends caudally to produce a collateral urethral duplication, but in one boy bladder duplication was associated with 4 urethrae in the sagittal plane. In 5 cases the bladders were united; in 2 cases, one bladder was excised. The results were satisfactory in 6 and in one the bladder failed to empty. The basic defects that lead to duplication are unknown. Several features of the conditons suggest that there are different aetiologies in each type. There are similarities with the exstrophy epispadias complex. Even when there are major abnormalities in other systems the general and urological prognosis is good.

Abnormalities, Multiple↗
PubMed1979

Müllerian duct remnants in the male.

Persistent remnants of the caudal Müllerian duct in the male may occasionally give rise to symptoms and pose a problem of management. We describe 13 cases which have been seen over a 24-year period. In 4 cases the remnant took the form of a cyst, whilst the other 9 had tubular utricular structures. Not all had an associated maldevelopment of the genitalia and there was no relationship between the size of a tubular remnant and the degree of genital abnormality. Infection generally appeared to be related to the presence of obstruction to the free drainage of the contents of the remnant, and epididymitis occurred where free reflux into the vasa was present. Apparent incontinence was sometimes the result of urine pooling in the utricular pouch. The radiological demonstration of these structures was not always easy and in our experience voiding cystourethrography was the best means of achieving a diagnosis. Surgical excision of these structures can be difficult, and if they do not produce symptoms they should be left alone.

Adolescent↗
PubMed1979

Complete deficiency of adenine phosphoribosyltransferase: a third case presenting as renal stones in a young child.

We report a third case of 2, 8-dihydroxyadenine stones in a child with a complete lack of the adenine salvage enzyme--adenine phosphoribosyltransferase (APRT). The propositus, a 20-month-old girl of consanguineous Arab parents, presented with multiple urinary tract infections and supposed 'uric acid' stones in the right renal pelvis and left ureter. Both parents and one brother were heterzygotes for the defect, in keeping with an autosomal recessive mode of inheritance. In contrast with the other purine salvage enzyme disorder of childhood with true uric acid stones (the Lesch-Nyhan syndrome), uric acid excretion was normal in all family members. As in our previous case, treatment with allopurinol, without alkali, has eliminated the urinary excretion of 2, 8-dihydroxyadenine: the stones were removed surgically. 2, 8-Dihydroxyadenine should be considered in any child thought to have uric acid stones and tests made to distinguish the two compounds.

Adenine↗
PubMed1979

Postoperative death in paediatric urology.

In the combined experience of two paediatric urologists over a 25 year period only 4 fatalities relating directly to the surgical procedure could be identified. Two other deaths are presented and retrospectively most of the fatalities could have been avoided by present day management but not at the time they occured. Operative mortality in relation to paediatric urology is discussed fully and it is suggested that death related to the immediate surgery should be fully avoidable in relation to paediatric urology. Death from renal failure is a different problem and is unrelated to this paper.

Child, Preschool↗
PubMed1978

Renin and blood-pressure in children with renal scarring and vesicoureteric reflux.

Plasma-renin-activity (P.R.A.) was raised in 9 of 15 hypertensive children with pyelonephritic scarring secondary to urinary-tract infection and vesicoureteric reflux and also in 8 of 100 normotensive children with such scars. P.R.A. was much less likely to fall with age in normotensive children with renal scarring than in normal children. The identification of hyperreninaemic normotensive children with renal scarring is important, since P.R.A. may prove to be of value in early identification of children at risk of developing hypertension. A longitudinal follow-up is proposed to establish this hypothesis.

Adolescent↗
PubMed1978

The long-term follow-up of surgically treated vesicoureteric reflux.

We have followed up 83% of a series of 166 patients with vesicoureteric reflux who were treated surgically more than 10 years ago. We found an incidence of hypertension of 12.8%. The need for careful follow-up of the blood pressure of patients with reflux nephropathy is stressed.

Adolescent↗