Home ventilation of a child with motor and sensory neuropathy.

摘要: A paediatrician describes a young child who was eventually diagnosed as having autosomal recessive hereditary motor and sensory neuropathy type III the uncertainties surrounding decision to support at home on domiciliary ventilation. The child's mother gives her reaction decision, we also invited comments from public health physician, nurse, an intensive care paediatrician. The child, girl, born 37 weeks' gestation by elective caesarean section because of severe intrauterine growth retardation. Her birth weight 1830 g. She spent first two weeks in special baby unit, there difficulty establishing feeding. smiled 9 aware environment people. Throughout life cognitive development always normal. However, 3-6 months age she made no attempt lift head or roll could not sit even when supported. appeared feed normally, but 6 failing thrive. third parents' three children. There family history neurological disease. The known be seriously unwell until admitted district general hospital paediatric ward old with respiratory distress. Pneumonia confirmed chest radiograph. Then it became apparent that had widespread muscular weakness failure muscles. paralysed, sedated, ventilated. An underlying neuromuscular problem diagnosed, …