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  • 1
    ISSN: 1432-198X
    Keywords: Chronic renal failure from infancy ; Neurological outcome ; Nutritional support ; No aluminium therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Progressive encephalopathy, developmental delay, microcephaly, electroencephalogram (EEG) and computed tomographic (CT) scan abnormalities have been reported in 80% of children with chronic renal failure (CRF) in infancy. Malnutrition, aluminium intoxication and psychosocial deprivation are proposed as causes. In 15 children with CRF from infancy we evaluated the effect of no aluminium salts and early vigorous nutritional and psychosocial support, in addition to the standard therapy, on neurological development. Six patients underwent dialysis (2 at birth) and 3 received transplants. None of our patients were given aluminium therapy. The nutritional status of the patients in the first 2 years of life was assessed with the waterlow classification. At the end of the follow-up period (mean 50 months range 14–148 months), patients underwent neurodevelopmental assessment, head CT scan, EEG, nerve conduction velocity (NCV) and auditory brain stem evoked response (ABER). None of our patients developed progressive encephalopathy or recurrent seizures. All have a normal neurological examination apart from hypotonia. Microcephaly was present in 5 patients. There was a good correlation between malnutrition in the first 2 years of life and microcephaly. Developmental delay was present in 3 patients; all 3 were microcephalic. There was evidence of brain atrophy on CT scan in only 3 patients. EEG was abnormal in 6 patients, but only severe in 1 patient. Only 1 patient had diminished NCV; all patients had a normal ABER. We conclude that a policy of no oral aluminium therapy and early nutritional support leads to better neurological outcome in children with CRF from infancy.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2013
    Keywords: Children ; Head injury ; Coma ; Evoked Potentials ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Follow-up brainstem auditory evoked potential (BAEP) and somatosensory evoked potential (SEP) studies were performed within 72 hours after admission in 127 children with severe head injury (Glasgow Coma Scale score of ≤8) in order to predictquo ad vitam outcome of posttraumatic coma. Outcomes were categorised as brain death and survival. On first assessment 50 comatose children had normal BAEPs and SEPs. 78% of them survived and 22% deteriorated and died. 45 had abnormal findings. 69% of them improved and survived whilst 31 % deteriorated and died. 32 children did not have recordable BAEPs and SEPs. All of them died. Thus, comatose children with normal EP studies have in 78% good prognosis and a bad outcome can be reliably predicted.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-2013
    Keywords: Key words auditory neuropathy ; brainstem auditory evoked potentials ; cochlear microphonics ; hereditary motor and sensory neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The aim of this work was to assess the he ’ g impairment in patients with hereditary motor and sensory neuropathy (HMSN). Elevation of pure tone thresholds in the presence of preserved inner ear function as suggested by cochlear microphonics (CM), absent or markedly abnormal brainstem auditory evoked potentials (BAEP), and elevation of speech perception out of proportion to the pure tone loss were found in the patients. From 28 members of a Gypsy family, we examined two siblings aged 31 and 30 years and their nephew aged 20 years, all suffering from HMSN that was associated with auditory neuropathy. All three affected members with difficulty of understanding speech had following investigations: pure tone and speech audiograms, BAEP, cochlear microphonics, and nerve conduction studies (NCV). Results: the older two siblings had a flat 80 dB audiogram, whereas the younger one has flat 20 dB audiogram on the Lt. ear and 30 dB audiogram on the Rt. ear. All had no speech comprehension and no BAEP. Two patients had preserved cochlear microphonics on one ear. Peripheral nerves were electrically not elicitable, however, at the beginning of the disease nerve conduction was slow. Conclusion: in all three affected members with distinct clinical picture of HMSN their hearing impairment was proved to be due to severe auditory neuropathy in the presence of preserved inner ear function.
    Type of Medium: Electronic Resource
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