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Biomedical subjects

G M Hunt

Publications and source records attributed to G M Hunt.

At least 19 recordsLinked to original sources

Perineal sensation: an important predictor of long-term outcome in open spina bifida.

OBJECTIVES: To see if perineal sensation in infants with open spina bifida is associated with a better long-term outcome, particularly in terms of survival, renal-related deaths and incontinence. METHODS: We conducted a prospective cohort study on a complete cohort of 117 consecutive patients with open spina bifida, whose backs were closed non-selectively at birth between 1963 and 1971. A meticulous neurological examination in infancy showed that 33 (28%) of them had perineal sensation, defined as intact sensation to pinprick in at least one dermatome on one side in the saddle area (S2-4). Data recorded within 48 h of birth and during six reviews between 1972 and 2002 were used. Details of deaths were obtained from medical records and from the Office of National Statistics. RESULTS: By December 2005, 57% (67/117) of the cohort had died. There were 50 survivors with a mean age 38 years (range 35-41). More of those with perineal sensation survived than those without (23/33 v 27/84, p<0.001). This difference was mainly caused by 19 renal deaths in those lacking perineal sensation. Crucially there were no renal-related deaths in those with perineal sensation (0/33 v 19/84, p = 0.003). Among the survivors, those with perineal sensation were more likely than the remainder to be continent of urine and faeces (10/23 v 1/27, p<0.001 and 18/23 v 9/27, p = 0.002 respectively). They were also more likely to be able to walk at least 50 m (11/23 v 5/27, p = 0.027) and never to have had pressure sores (15/23 v 9/27, p = 0.025). CONCLUSIONS: A simple assessment of perineal sensation in infancy predicts long-term outcome in terms of survival, renal prognosis and incontinence in open spina bifida.

Adolescent↗

Characterisation of the long terminal repeat regions of South African human immunodeficiency virus type 1 isolates.

The 5' long terminal repeat (LTR) region of the integrated proviral human immunodeficiency virus type I (HIV-1) template encodes cis-acting sequences for cellular proteins that are responsible for initiating viral transcription. The objective of this study was to analyse the LTR regions of isolates from a broad spectrum of South African HIV-1 infected individuals to (i) determine if sequence diversity was sufficient to allow for subtyping on the basis of this region, and (ii) to note any specific or unusual alterations in promoter binding motifs that may be common to this group of isolates or specific HIV-1 subtypes within this group. A total of 60 isolates were subtyped by heteroduplex mobility assay (HMA) and by phylogenetic analysis, using both the env and gag regions. Phylogenetic relatedness within the LTR region demonstrated the suitability of this region for use in HIV-1 subtype designation. The presence of additional NF-kappaB binding elements as well as altered USF binding sites were features common to subtype C HIV-1 isolates. Although the biological relevance of these alterations within the HIV-1 LTR with respect to viral replicative capacity and patient disease progression is unknown, there is strong support to suggest that in the presence of these features, there is increased gene transcription in subtype C isolates, and that this would be further increased in the presence of secondary infection.

Base Sequence↗

The Casey Holter lecture. Non-selective intervention in newborn babies with open spina bifida: the outcome 30 years on for the complete cohort.

The disability in neural tube defect is determined by the neurological deficit. The sensory level is a reliable measure of this deficit and predicts the disability. Achievement at the age of 30 related as well as to IQ and disability events in the history of CSF shunt. Episodes of raised intracranial pressure may damage the brain and visual pathways. They may also reduce achievement and enterprise. Blockage of the CSF shunt, when symptomatic, should be treated urgently, especially when it occurs after infancy. Blockage of the CSF shunt when asymptomatic was not usually associated with a low IQ or poor achievement.

Cerebrospinal Fluid Shunts↗

Link between the CSF shunt and achievement in adults with spina bifida.

OBJECTIVES: A few enterprising adults with shunt treated spina bifida live independently in the community, have a job in competitive employment, and drive to work in their own car. By contrast others with similar disability but lacking their motivation remain dependent on care and supervision. The aim of this study was to identify events in the history of their shunt which may have influenced their subsequent achievement. METHODS: Between June 1963 and January 1971 117 babies born in East Anglia with open spina bifida had their backs closed regardless of the severity of their condition. When reviewed in 1997 every case was ascertained. Sixty had died and the 57 survivors had a mean age of 30. These were assigned to two groups: achievers and non-achievers, according to their attainments in independence, employment, and use of a car. RESULTS: Of the 57 survivors nine had no shunt and eight of these were achievers. All were of normal intelligence (IQ>/=80) and only one was severely disabled. Of the 48 with shunts only 20 were achievers (OR 11.2, 95% confidence interval (95% CI) 1.3-96.8). Lack of achievement in these 48 was associated with revisions of the shunt, particularly when revisions were performed after the age of 2. Sixteen patients had never required a revision and 11 (69%) were achievers; 10 had had revisions only during infancy and five (50%) were achievers; 22 had had revisions after their second birthday and only four (18%) were achievers (p<0.001). Elective revisions were not performed in this cohort and in 75% of patients revisions had been preceded by clear symptoms of raised intracranial pressure. CONCLUSION: Revisions of the shunt, particularly after the age of 2, are associated with poor long term achievement in adults with spina bifida.

Activities of Daily Living↗

Open spina bifida: a complete cohort reviewed 25 years after closure.

This study summarises the long-term results of non-selective treatment of open spina bifida. Every member of a consecutive series of 117 cases was ascertained 22 to 28 years after closure of the back. 56 of the cohort had died. The condition of the 61 survivors ranged from normal to severe disability. Only 33 of the survivors were capable of living independently, 11 required supervision and some help, and 17 needed daily care which was generally provided by the parents. The two main determinants of disability and dependency were the extent of the neurological deficit and IQ. Improvements in the management of patients with open spina bifida have greatly reduced mortality, but they are less likely to influence long-term disability since that is dependent on the severity of the neurological deficit.

Activities of Daily Living↗

Intermittent self catheterization for patients with urinary incontinence or difficulty emptying the bladder.

Urinary incontinence, difficulty voiding and recurrent urinary tract infections are common in general practice. In patients with multiple sclerosis, spina bifida, intervertebral disc lesions, spinal injuries or tumours, the symptoms may be associated with a high residual volume of urine owing to a neuropathic bladder. Similar complaints may occur in elderly people or in women with gynaecological problems owing to atonic urinary retention. Provided that a significant residual volume of urine is found on abdominal examination, ultrasound, x-ray or catheterization, both groups of patients may be helped by intermittent self catheterization. Intermittent self catheterization is a safe and simple technique. By catheterizing themselves between four and six times daily patients can gain control over their bladders. Abandoning indwelling catheters or bulky external appliances does much for a patient's morale and self esteem. In addition, since the bladder is being drained effectively, urinary tract infections cease to be a problem and the kidneys are safeguarded. Severe disability is not a contraindication since patients in wheelchairs have mastered the technique despite paraplegia, an anaesthetic perineum, spinal deformity, intention tremor, mental handicap, old age or blindness. Patients should be referred to urologists for a trial of intermittent self catheterization. If unsuccessful or unacceptable it can be abandoned with no long term consequences. If it is effective the benefits may be considerable.

Adult↗

A new device for self-catheterisation in wheelchair-bound women.

The Deavin-Hunt device is designed to help paraplegic and incontinent women to catheterise themselves whilst in a wheelchair. Such patients with neuropathic bladder might otherwise be unable to use intermittent catheterisation, an effective method of controlling incontinence and preserving the kidneys.

Persons with Disabilities↗

Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood.

Between 1963 and 1970, 117 consecutive cases of open spina bifida were treated unselectively from birth, following a full neurological examination. The author reviews the outcome 16 to 20 years later. 48 died before their sixteenth birthday. Of the 69 survivors, 60 had had a shunt inserted and two were blind following shunt dysfunction; 22 were mentally retarded (IQ less than 80); 35 were wheelchair-dependent; 52 were incontinent, and 32 of these continued to need help with its management; nine weighed greater than 75 kg; one-third of both sexes had a precocious puberty; three had insulin-dependent diabetes; 12 continued to need anticonvulsant drugs; 32 had suffered from pressure sores; 33 were unable to live without help or supervision and only 17 were capable of open employment. These findings demonstrate the wide range of disabilities implied by the diagnosis of open spina bifida. The neurological findings recorded at birth were reflected in the mortality and disability of the survivors. The sensory level represented a yardstick for predicting the likely range of handicap into adult life, provided no additional impairment resulted from serious complications: such damage occurred in seven of the survivors.

Activities of Daily Living↗

The pattern of congenital renal anomalies associated with neural-tube defects.

This is a retrospective study of 190 patients with neural-tube defects for whom there were precise data, both on sensory levels (to pin-prick) and on the presence or absence of congenital renal anomalies. There were 17 renal anomalies, of which three were renal agenesis, five horseshoe kidneys, eight ureteral duplications and one simple ureterocele. There was a consistent pattern of congenital renal anomalies in relation to the extent of the neurological lesion, as measured by the sensory level. Renal agenesis was associated with a sensory level in the dermatomes T5 to T8, horseshoe kidneys with a T9 to L1 level and ureteral duplications predominantly with a sensory level in the sacral dermatomes.

Abnormalities, Multiple↗

Incidence and distribution of renal anomalies in patients with neural tube defects.

In 190 patients with neural tube defects the sensory levels to pin prick were compared with the incidence and distribution of congenital renal anomalies. 17 such anomalies comprised 3 examples of renal agenesis, 5 horseshoe kidneys, 8 ureteral duplications and one simple ureterocele. The pattern of anomalies showed that renal agenesis was associated with a sensory level in the dermatomes T5-8, horseshoe kidneys with T9-L1 and duplications predominantly with the sacral dermatomes.

Abnormalities, Multiple↗

Bacterial study of clean intermittent catheterisation in children.

Deterioration of the upper urinary tract is exceptional in children on clean intermittent catheterisation for neuropathic incontinence and is found only in those with pre-existing renal damage. This report describes a bacteriological study of 24 children, of whom 10 had renal damage and 14 did not have renal damage before clean intermittent catheterisation began. Boric acid was added as a preservative to samples of urine in preference to the use of dip-slides because it preserves pus cells as well as bacteria. The incidence of bacteriuria in the two groups was similar (78% of samples from those with, and 72% of samples from those without, pre-existing renal damage). The groups differed in that the urine of children in the group with pre-existing renal damage yielded organisms other than Escherichia coli more than twice as often as did the urine of those children without renal damage. Moreover, the children with pre-existing renal damage were more likely to have fever with urinary tract infection and some of them showed frequent changes of organisms in the urine. Whatever organism was present, however, children in the group with renal damage more often had heavy pyuria.

Adolescent↗