Autologous stem cell transplantation in diffuse scleroderma: impact on hand structure and function.

BACKGROUND: The aim of the study was to assess the structural and functional effects of autologous stem cell transplantation (ASCT) on scleroderma finger clawing. METHODS: Using photocopies of hands of five scleroderma patients who underwent ASCT using photocopies of hands. Functional assessments used a standardized questionnaire. RESULTS: Pre-ASCT, synovitis and tenosynovitis were present in five and four patients, respectively. Modified Rodnan hand skin scores ranged from 6-12/12. Following pulsed chemotherapy, synovitis resolved. Tenosynovitis often did not. Post-ASCT, skin scores fell in four patients (range 0-6/12). Hand tenosynovitis resolved. With disease remission hand function globally improved. Functional improvement, noted early (+3 months) and continuously (+12 months) in disease remitters, occurred in all areas of function. Greatest hand-functional improvement related to paid employment, followed by self-care and hygiene, home-care activities and least by hobbies/sports. The second to fifth metacarpophalangeal width was reproducible and independent of ASCT therapy. In contrast, hand length and measures of abducted finger span (first to fifth fingertip and second to fifth fingertip distance) improved. Finger abduction (abducted first to fifth fingertips/second to fifth metacarpophalangeal width) was a more sensitive discriminator of finger clawing than hand length or hand length/second to fifth metacarpophalangeal width. CONCLUSION: ASCT improved hand scleroderma over 12 months and resolved previously refractory tenosynovitis. ASCT was unnecessary to treat scleroderma synovitis. ASCT secondarily improved hand function (paid employment, followed by self-care, home care, then by sport/hobbies). Loss of finger abduction was a more sensitive measure of finger clawing than apparent loss of hand length.

Changing patterns of diagnosis and treatment of infantile hypertrophic pyloric stenosis: a clinical audit of 303 patients.

This review of 303 patients with infantile hypertrophic pyloric stenosis (IHPS) concentrates on the influence of clinical audit on diagnosis, complications, and factors contributing to hospital stay. Although the audit has enabled improvement in care by pediatric surgeons, there has been less change in areas controlled by other specialities. During a 12-year period, the number of patients diagnosed solely by clinical examination decreased from 74% to 28%, and the use of diagnostic tests increased (ultrasonography from 16% to 65% and barium meal from 12% to 28%). This trend continued throughout the series despite a review after 8.5 years, which recommended fewer tests. Although there may be some benefit from earlier confirmation of IHPS (the percentage of patients with a serum chloride value of less than 85 mmol/L decreased from 26% to 15%), the need for diagnostic tests could be reduced by expectant management. Better improvement occurred with surgical complications; the incidence of mucosal perforation decreased from 7 of 151 (4.6%) in the first 6 years to 0 of 152 in the last 6 years, and wound dehiscence was reduced from 3 to 0. The wound infection rate decreased from 9% to 4%, but had fluctuations. The average length of stay was reduced from 3.7 to 3.2 days. Further reductions in hospital stay will depend on earlier operation for patients with normal electrolyte values at the time of admission (61%) and a preparedness to confidently discharge patients even if there is vomiting. From the data available, early operation on the day of admission and discharge the next day would be a reasonable strategy for the majority of patients whose admission electrolyte values are normal. Complications such as mucosal perforation and wound dehiscence should be rare. This is not to suggest that IHPS is a condition of minor consequence; the surgeon must be skilled and care meticulous. If electrolytes are disturbed at the time of operation or if unrecognized mucosal perforation occurs, what should be an uneventful illness can result in disaster.