Managing cleft lip and palate. Language outcomes are important.
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Biomedical subjects
Publications and source records attributed to A Harding.
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Tubal pregnancy can be safely and effectively managed by MTX or CO2 laparoscopic salpingotomy techniques. Methotrexate may be superior because of its simplicity, requiring only basic laparoscopic skills, whereas laparoscopic salpingotomy necessitates operative laparoscopic input.
In 32 women with unruptured tubal ectopic pregnancies we undertook conservative laparoscopic treatment [local injection of 20 mg methotrexate (n = 18), laser salpingotomy (n = 14)]. The results of serial quantitative beta HCG measurement were followed until either a negative level was reached or until rising levels necessitated alternative/additional therapy. Plateaued values of beta HCG were observed in both the successful (n = 16) and the unsuccessful cases (n = 5). To test the hypothesis that daily variation in the assay could account for some or all of the observed plateaued results in successful cases, the sera were retested serially on the same 'run'. In only one case did laboratory variation account for the observed plateau. The clinical implications of the findings are discussed. We conclude that serially monitored beta HCG results after conservative treatment of ectopic pregnancy may show plateaued values without indicating failure of treatment.
This paper presents a comprehensive screening procedure for describing the speech characteristics commonly associated with cleft palate and/or velopharyngeal dysfunction. A unique method of representing the information visually is proposed. The theoretical background and recommendations for clinical application are discussed. This procedure is designed for use by specialist and non-specialist speech and language therapists working in this field. It has been developed primarily with a child population but can be used with all age groups. It provides a structure for assessment, record keeping, report writing and research, thereby facilitating the development of intercentre studies.
The Wernicke-Korsakoff syndrome is a neuropathological term which encompasses two clinical syndromes in thiamine-deficient alcoholics, Wernicke's encephalopathy and Korsakoff's psychosis. Wernicke's encephalopathy is characterised by eye and gait disorders and mental confusion, and can lead to the profound and permanent amnesia known as Korsakoff's psychosis. Despite this specific difference, both conditions appear to have identical neuropathology with haemorrhages and other lesions around the ventricular system. The memory deficit has been attributed to a number of brain lesions, including a recent suggestion that brain pathways utilising particular neurotransmitters are specifically damaged. To examine this, the number of chemically identified neurons in particular brain regions was quantified in patients with Wernicke's encephalopathy alone or in combination with Korsakoff's psychosis and compared with age- and sex-matched controls. Noradrenaline, a neurotransmitter thought to be involved in the process of selective attention, is localised in pathways projecting to the cortex. Our patients with either Wernicke's encephalopathy or additional Korsakoff's psychosis do not differ from controls in the distribution and number of these cells. Serotonin is another neurotransmitter that has been linked with alcohol dependency. Both patient groups have a profound loss of serotonin-containing neurons compared with controls. The loss of forebrain neurons containing acetylcholine in patients with Alzheimer's disease has implicated this neurotransmitter in the maintenance of memory functions. There was a large variation in the number of these forebrain neurons in thiamine-deficient alcoholics compared with controls. Cholinergic cell loss reflected the severity of cognitive dysfunction, but was not exclusive to patients with amnesia. Two thalamic nuclei are involved in relaying memories for storage and retrieval, the anterior and mediodorsal thalamic nuclei. While patients with Wernicke's encephalopathy often had neuronal loss in the mediodorsal nucleus, only patients with Korsakoff's psychosis had cell loss in both medial thalamic nuclei. The results suggest that cumulative lesions contribute to the amnesia seen in thiamine-deficient alcoholics, including deficits in serotonergic, cholinergic and medial thalamic pathways.
OBJECTIVE: To identify cases of inherited prion diseases in Britain and to assess their phenotypic features. DESIGN: Screening study of patients suspected clinically to have Creutzfeldt-Jakob disease and other neurodegenerative diseases by prion protein gene analysis. SETTING: Biochemical research department. SUBJECTS: Patients suspected to have Creutzfeldt-Jakob disease and other neurodegenerative diseases. RESULTS: Two patients with symptoms characteristic of sporadic Creutzfeldt-Jakob disease were found to have inherited prion protein disease (PrP lysine 200), with a mutation at codon 200 of the prion protein gene. Both were homozygous at codon 129 of the gene. One patient was a man aged 58 of British descent while the other was of Libyan Jewish origin. CONCLUSION: Two foci of inherited prion disease are known, among Libyan Jews and in Slovakia. A separate British focus of the disease may also exist. Heterozygosity at codon 129 may lead to reduced penetrance of the mutation.
An "early surgery" and a "delayed surgery" group of cleft palate cases' speech has been recorded and judged in two ways. Initially four different listener groups gave their "impressions" of the speech samples followed by the two speech therapist groups assessing specific aspects of speech. For analytical purposes the delayed surgery group was divided into "repaired" and "unrepaired" hard palate groups. The general conclusion was that delaying hard palate surgery may be responsible for the persistence of noticeable abnormal speech patterns until after hard palate repair. However, despite their higher frequency in the "delayed unrepaired" group, errors of tongue placement and airstream direction were common to all groups. The relative influence of maturation, surgery and speech therapy on speech progress has not been considered. It is suggested that a final review of oro-facial growth and speech as each subject reaches 17 years old would give more definitive results. Then the subjects might discuss the relative importance of facial appearance and speech results.
A group of 22 white males (ages, 28 to 40 years) with histories of chronic alcohol abuse ranging from 1 to 21 years were evaluated for trabecular bone mineral density in the left femoral neck area. A group of age and weight-matched white males with no history of alcoholism served as controls. All participants completed medical history questionnaires regarding fracture history, dietary habits, medications, and physical activity. Singh femoral trabecular indices also were measured for the alcoholic participants. Analysis of bone mineral data as measured by dual photon absorptiometry revealed no statistically significant reduction in the bone mineral densities of the alcoholic group when compared to the controls. No differences were seen in the areas of the femoral neck, Ward's triangle, and greater trochanter. Duration of alcoholism also was not correlated with the degree of osteopenia. Singh index measurements of right and left femoral heads were compared and revealed no significant differences within individual patients. Left femoral Singh index values averaged 5.5 (range, 4 to 6) for the alcoholic group and were not indicative of decreased bone mineral density. It is concluded from this study that chronic alcoholism associated with heavy smoking most likely has a debilitating effect on the trabecular bone density of white males, yet clinical and radiographic evidence is not widely manifested in men under the age of 40 years.
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Cleft palate speech is generally described in terms of nasal resonance, nasal emission and compensatory articulations. A longitudinal study of children at different stages of surgical treatment revealed a distinction between passive and active cleft-type speech characteristics whereby passive characteristics were thought to be the product of structural abnormality or dysfunction and active characteristics were specific articulatory gestures replacing intended consonants. Passive and active patterns of articulation are described and defined in the context of three longitudinal studies of subjects who were at various stages of two different surgical regimes: five bilateral cleft lip and palate (BCLP) subjects aged 1;6-4;6, 12 mixed unilateral cleft lip and palate (UCLP) and BCLP subjects aged 4;6-7;6 and nine mixed UCLP and BCLP subjects aged 9;0-11;0. Reference is also made to data from 12 mixed cleft-type subjects aged 13;0 who had been treated with different surgical timing regimes. Comparison is made between the incidence of active versus passive processes in relation to oral structure. At age 4;6 speech samples taken from BCLP subjects who had been treated with 1-stage versus 2-stage palate repair all evidenced both active and passive processes. The lack of differentiation in speech results irrespective of their current surgical status, i.e. completely repaired palates versus residual cleft of the hard palate, was unexpected. Cleft-type processes in completely repaired subjects might be accounted for by the inevitable anterior defect following repair of a bilateral cleft. Older subjects with structural defects also evidenced more cleft-type processes. The relevance of distinguishing between active and passive processes is underlined by consideration of the effects of structural changes following surgery. The effect of surgery on seven subjects' speech is discussed using the active/passive distinction. Active cleft-type characteristics did not change as a direct result of surgery, whereas passive characteristics were largely eliminated following surgery. A specific distinction is made between active and passive nasal fricatives, with the implication that active nasal fricatives may not be affected by surgical intervention, whereas passive nasal fricatives may be eliminated by surgery. Accurate distinction between active and passive patterns of articulation may serve to identify those cleft-type speech error patterns most likely to respond to surgical intervention. Indications from this study are that active cleft-type characteristics require destabilization in a course of speech and language therapy before the potential benefits of surgery can be properly assessed. An analytical protocol for the interpretation of speech samples is presented and some therapy strategies are proposed for active and passive processes.
In 1994 the present authors proposed a speech assessment protocol for speech disorders associated with cleft palate and/or velopharyngeal dysfunction known as GOS.SP.ASS. (Great Ormond Street Speech Assessment). In a recent survey undertaken to review the different speech assessment protocols used in six cleft palate centres in the UK, GOS.SP.ASS. was selected from six protocols as the optimal procedure for clinical and research purposes. The process of identifying an optimal procedure involved analysis of completed forms for each assessment. Analysis of the completed GOS.SP.ASS. forms revealed significant ambiguities in the protocol which led to differences in form completion. This paper describes important revisions to the original GOS.SP.ASS. protocol in order to ensure comparable data from different clinicians. This detailed speech assessment is now complemented by the Cleft Audit Protocol for Speech (CAPS), a tool recommended for clinical audit. As a result of close collaboration in their preparation, the results are directly comparable. In addition, the speech elicitation sentences and the phonetic diagram have been modified.