Between March 1993 and February 1999, 14 children aged from eight months to 14.75 years were admitted to the paediatric intensive-care unit with meningococcal septicaemia in association with severe peripheral ischaemia. Of these, 13 were operated upon, eight of whom had early fasciotomies. Five children died. Of the nine survivors, one had no amputations while in the other eight 14 limb segments were amputated. We review the case histories and propose a protocol for the early management of these children.
We tested prospectively for hepatitis C virus (HCV) in one orthopaedic surgeon's operative practice for one year. Of 425 consecutive patients, 19 (4.5%) were positive for HCV infection using a second-generation screening assay. The highest correlation with a positive test was the presence of tattoos and the second highest was intravenous drug abuse, but only after a second interview, since most patients did not report this risk on the initial questionnaire. Based on the criteria of the US Public Health Services algorithm, nine (47%) of the patients with a positive initial screening test or 2.2% of the 425 patients, had hepatitis C (both anti-HCV-positive and elevated alanine aminotransferase). In this group of nine, the presence of tattoos had the highest and intravenous drug abuse the second highest correlation, also after the second interview. There is no vaccine available for the prevention of HCV infection, and prophylactic immunoglobulin therapy has no proven value for primary exposure.
For clinical, psychological and social reasons the diagnosis of Duchenne muscular dystrophy should be established as early as possible. In a survey of 83 families with 93 affected boys, the diagnosis was missed in every case referred to an orthopaedic surgeon (37 patients). In the whole group there was a mean delay of 2.0 years (0 to 6 years) during which time inappropriate treatment, difficulties in communication with parents, much parental anxiety and further pregnancies occurred. A serum creatine kinase estimation is a simple outpatient test which should be carried out on any boy with clumsy or abnormal gait, with flat feet or with an unexplained equinus deformity.
The majority of patients with chronic infantile and juvenile forms of spinal muscular atrophy survive to adult life. Forty-four patients have been reviewed at an average of 17 years after diagnosis. The subdivision of patients into four groups, based on the maximal physical function developed by the individual, correlates well with the onset and severity of secondary deformity of the limbs and spine. This information allows anticipation of the problems and plans for their treatment to be made from early childhood. After analysis of the orthotic and surgical treatment received by these patients, a specific programme of care is recommended for each of the functional groups.