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Biomedical subjects

K Shepherd

Publications and source records attributed to K Shepherd.

28 records · Page 2Linked to original sources

Low molecular weight IgM in primary biliary cirrhosis.

Low molecular weight IgM is the monomeric subunit of pentameric IgM and is not generally found in the blood of healthy individuals. Using a sensitive immunoblotting technique, low molecular weight IgM was detected in all 17 patients with primary biliary cirrhosis and constituted up to 5% of the total circulating IgM. This low molecular weight IgM moiety correlated significantly with total IgM (p less than 0.01) but not with the specific biliary cirrhosis mitochondrial autoantibody anti-M2. Furthermore it was not possible to show that a partially purified sample of low molecular weight IgM contained M2 binding activity. Mitogen stimulated peripheral blood mononuclear cells from two of four patients were observed to secrete low molecular weight IgM in vitro, a finding seen in only one of six healthy subjects. Immunoblot analysis of patients sera revealed the presence of other oligomers of IgM in addition to low molecular weight IgM. In conclusion this study suggests that during the enhanced IgM synthesis observed in primary biliary cirrhosis a defect occurs in the assembly of the IgM pentamer with release of monomeric and oligomeric IgM into the circulation. The pathogenic significance of these circulating low molecular weight IgM species is unknown.

Adult↗

Faecal incontinence in childhood: a multidisciplinary approach including biofeedback.

One hundred and seven children with faecal incontinence were evaluated and managed over a 3 year period by a multidisciplinary team. After initial clinical assessment, evaluation of defaecatory mechanisms (using a balloon model) and assessment of personal-social development and self-concept were undertaken. Management was based on initial bowel evacuation, short-term laxatives, and habit training involving systematic use of positive reinforcement; 69 children received biofeedback conditioning. Idiopathic megacolon with constipation and soiling was the most common finding (98 cases). Other diagnoses included previously undiagnosed neurogenic bowel (three cases), post-surgical and anomalies (four cases), and psychogenic encopresis (two cases). Idiopathic megacolon was characterized by decreased rectal sensation, increased threshold for external sphincter relaxation and an inability to evacuate. Faecal incontinence was associated with an undesirably low social self-concept (70% of the 40 evaluated), but was not related to a delay in development (mean general developmental quotient = 105 +/- 8, for the 35 tested). Family psychopathology warranting referral for family therapy was found in 14 children (13%). The management programme yielded a short-term (3 months) cure rate of 68% and a long-term (12 months) cure rate of 90%, with 10% having continued soiling which varied from occasional to several incidents/week. No significant improvement in self-concept was observed overall, although marked improvements were observed in some children. We conclude that disordered defaecatory dynamics are a major determinant of faecal incontinence in children. Undesirably low social self-concepts but normal developmental ability accompany this condition. Management is facilitated by a multidisciplinary approach, acknowledging the role of both behavioural and physiological components of the problem.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Low molecular weight IgM in juvenile chronic arthritis.

Low molecular weight IgM, the monomeric subunit of pentameric IgM, was clearly detected by immunoblotting and filtration chromatographic techniques in six patients with juvenile chronic arthritis and in trace quantities in a further eight of 24 patients studied. This low molecular weight IgM moiety contributed up to 33% of the total circulating IgM and was strongly associated with raised serum concentrations of IgM and the presence of antinuclear antibodies, extractable antinuclear antibodies, and rheumatoid factor. Immunoblot analysis of positive serum samples showed small quantities of other low molecular weight oligomers of IgM in addition to monomeric IgM. It is postulated that the presence of low molecular weight IgM in the serum of patients with juvenile chronic arthritis reflects a disorder of the intracellular assembly of IgM subunits during a stimulated IgM immune response. The pathogenetic role of low molecular weight IgM remains uncertain.

Antibodies, Antinuclear↗

Neurogenic faecal incontinence in children with spina bifida: rectosphincteric responses and evaluation of a physiological rationale for management, including biofeedback conditioning.

The rectosphincteric responses of 22 spina bifida (SB) children, aged 3.0 to 11.0 years, with frequent neurogenic faecal incontinence were investigated by clinical and anomanometric techniques and a bowel habilitation programme based on the physiological rationale of timed regular and complete evacuations, regulation of bowel habit and faecal consistency by diet and biofeedback conditioning of rectosphincteric responses. Eighteen patients had perianal anaesthesia, flail anus and absent anorectal reflex, but some degree of (reduced) rectal sensation. Four had reduced perianal sensation and normal to high pressure external sphincter and a megarectum apparently causing an absence of rectal sensation. Twenty were unable to expel the rectal balloon voluntarily. After biofeedback exercises (2-5 sessions) and a home programme of evacuation and diet, 21 of the 22 achieved satisfactory continence over 2-6 months with improved rectal sensation and ability to evacuate voluntarily. This experience suggests that early anorectal evaluation and a comprehensive bowel habilitation programme may be an effective approach to the prevention of disability due to faecal incontinence in SB children.

Anal Canal↗

Resting energy expenditure of children attending a rehabilitation programme following head injury.

STATEMENT OF PURPOSE: Increased resting energy expenditure following head injury is well documented, but whether this increase extends into rehabilitation and whether this is affected by changes in body composition have not been studied. The aim of this study was to determine whether children attending a rehabilitation program following head injury had altered energy expenditure and body composition. METHODS: Measurements of resting energy expenditure by indirect calorimetry were performed in 21 head injured children (mean age 10.2 +/- 3.8 years). Measurement of body composition was performed using total body potassium. RESULTS: Measured resting energy expenditure values were widely distributed, ranging from 52.3-156.4% of predicted values, yet the mean percentage predicted using Schofield weight, Schofield weight and height and World Health Organization predictive equations were 97.5%, 97.4% and 98.6%, respectively. Mean percentage of expected total body potassium for weight, height and age for head injured children were 85.1 +/- 15.5%, 89.1 +/- 14.1% and 86.9 +/- 15.9%, thus all showed significant depletion. CONCLUSIONS: During rehabilitation, using predictive equations to estimate resting energy expenditure in this group revealed a small bias on average but very large bias at the individual level. Head injured children had altered resting energy expenditure and body composition.

Adolescent↗

Reduced body cell mass following severe head injury in children: implications for rehabilitation.

STATEMENT OF PURPOSE: Increased proteolysis, muscle catabolism and altered body composition have been well documented after severe head injury, but the extent of these effects in children, and whether they extend into rehabilitation, have not been studied. This study determined nutritional status and body composition, with particular reference to the body cell mass (BCM), of head injured children at entry into a rehabilitation programme, and compared body composition analysis with anthropometric nutritional assessment. METHODS: Nineteen head injured children (nine males, 10 females, mean age 9.1 +/- 4.3 years range 1.2-15.1 years) were measured for height, weight and total body potassium (TBK, a measure of body cell mass) on referral to rehabilitation after the acute phase (mean 38.1 days post-injury). Data was compared with expected normative data derived from healthy age and gender matched children. Nutritional status was determined by two separate criteria based on either anthropometric or body composition methods. RESULTS: The mean percentage of expected TBK for height was 84.4 +/- 15%, significantly below the clinically acceptable level for body cell mass (90% of expected). Using the anthropometric definition, only 1/19 was undernourished, whereas 12/19 had poor nutritional status using body composition (chi 2 = 7.58, p = 0.006). CONCLUSIONS: The data revealed a significant depletion in the metabolically active BCM in the presence of normal anthropometry, suggestive of significant muscle wasting. These findings have important pathophysiological and clinical implications in the rehabilitation of children following major head trauma.

Adolescent↗

Oral-motor dysfunction and feeding problems in infants with myelodysplasia.

Oral motor function and feeding problems have received little attention in infants with myelomeningocele (MMC). We compared objective video assessments of oral motor skills, diet and feeding function of 20 infants (aged 6 months) with MMC, with data from 20, age, gender, and socioeconomically matched healthy infants using the Feeding Assessment Schedule (FAS), Feeding Environment Check (FEC), Tester's Ratings of Infant Behaviour (TRIB), an Infant Feeding Questionnaire (IFQ), and a 24 hour dietary analysis (DQA). MMC infants had significant feeding difficulty, related to sensory motor integration problems such as hypotonicity, positioning, and hand function. Despite these problems, there was minimal behavioural disruption in infants with MMC and their mothers experienced no marked food aversion or negative feeding experiences. MMC infants consumed feedings which were significantly lower in energy than controls, with possible imbalance of other nutrients. These studies indicate that compared with healthy infants MMC infants have significantly altered oral motor function which we speculate could be early features of the Chiari II malformation. The dietary changes may reflect altered energy requirement. Early identification and management of feeding problems associated with this condition is emphasized.

Arnold-Chiari Malformation↗