651 research outputs found

    Sydenham's chorea - clinical and therapeutic update 320 years down the line

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    No Abstract. South African Medical Journal Vol. 96(9) (Part 2) 2006: 906-91

    Is cardiac surgery warranted in children with Down syndrome? A case-controlled review

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    Objectives. To compare children with Down syndrome and children without Down syndrome and investigate whether there is a significant difference in the burden that is placed on the health care system between these two groups only in respect of the repair of congenital heart disease at Red Cross War Memorial Childrenā€™s Hospital, Cape Town, South Africa. Design. This study is a retrospective case control review. Setting. Red Cross War Memorial Childrenā€™s Hospital, Cape Town, South Africa.Subjects. The sample group of 50 Down syndrome children who had received cardiac surgery between January 1998 and June 2003 was compared with a control group of 50 nonsyndromic children who had received cardiac surgery during the same period. Outcome measures. Sex and diagnoses (cardiac and noncardiac), number of days spent in hospital and in ICU, complication rates, re-operation rates, early mortality rates, planned further cardiac surgery. Costs of these outcomes were not quantified in exact monetary terms. Results. There was no significant difference between the two groups in terms of the burden that was placed on the health care system. Similar complication rates, re-operation rates and early mortality rates were recorded for both groups. The Down syndrome group appeared to benefit more from cardiac surgery than the non-Down syndrome group. Conclusion. Denying cardiac surgery to children with Down syndrome does not improve the efficiency of resource allocation. It is therefore not reasonable to suggest that the problem of scarce resources can be ameliorated by discriminating against children with Down syndrome

    Sydenham's chorea - clinical and therapeutic update 320 years down the line

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    Post-streptococcal neuropsychiatric movement disorders (PNM) were first described in the Middle Ages, but today more than 300 years later, confusion remains surrounding the terminology, treatment and monitoring of these conditions. Rheumatic fever is currently the major cause of acquired heart disease among children in South Africa.1 The incidence of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) is not declining. Recent figures quote the incidence of rheumatic fever as 0.6 - 0.7/1 000 population in the USA and Japan compared with 15 - 21/1 000 population in Asia and Africa.2 In a study conducted in 1975 in Soweto, South Africa, 12 050 schoolchildren were examined and 19.2/1 000 had rheumatic heart disease.3 A 2002 report from a cardiology workshop highlighted the belief among clinicians that South Africa is currently in the midst of a rheumatic fever epidemic.4,5 Sydenhamā€™s chorea (SC) is a major manifestation of ARF. Accordingly, in the South African context when PNMs are diagnosed, treatment strategies must always include the prevention of RHD

    Cerebellar modulation of memory encoding in the periaqueductal grey and fear behaviour

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    The pivotal role of the periaqueductal grey (PAG) in fear learning is reinforced by the identification of neurons in male rat ventrolateral PAG (vlPAG) that encode fear memory through signalling the onset and offset of an auditory-conditioned stimulus during presentation of the unreinforced conditioned tone (CS+) during retrieval. Some units only display CS+ onset or offset responses, and the two signals differ in extinction sensitivity, suggesting that they are independent of each other. In addition, understanding cerebellar contributions to survival circuits is advanced by the discovery that (i) reversible inactivation of the medial cerebellar nucleus (MCN) during fear consolidation leads in subsequent retrieval to (a) disruption of the temporal precision of vlPAG offset, but not onset responses to CS+, and (b) an increase in duration of freezing behaviour. And (ii) chemogenetic manipulation of the MCN-vlPAG projection during fear acquisition (a) reduces the occurrence of fear-related ultrasonic vocalisations, and (b) during subsequent retrieval, slows the extinction rate of fear-related freezing. These findings show that the cerebellum is part of the survival network that regulates fear memory processes at multiple timescales and in multiple ways, raising the possibility that dysfunctional interactions in the cerebellar-survival network may underlie fear-related disorders and comorbidities
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