A comparative in vivo ultrasonometric evaluation of normal and delayed fracture healing in sheep tibiae

OBJECTIVE:

To compare normal and delayed bone healing by measuring ultrasound conduction velocity across the bone callus.

METHODS:

A model of transverse linear and 5 mm resection osteotomies of sheep tibiae was used. Fourteen sheep were operated on and were divided into two groups of seven according to osteotomy type. The procedure was performed on the right tibiae and the intact left tibiae were used as controls. The transverse and axial ultrasound velocities were measured at 30-day intervals for 90 days, after which the animals were killed and both the right and left tibiae were resected for in vitro biomechanical analysis.

RESULTS:

Both the transverse and axial ultrasound velocities progressively increased, but the increase was smaller for the delayed union that resulted from the resection osteotomy. The mechanical resistance was higher for the normally healed tibiae that resulted from a linear osteotomy; this result closely correlated with the ultrasound velocity results. Significant differences were found for the comparisons between the intact and operated tibiae in both groups and between the groups for both the transverse and axial ultrasound velocities, but the differences were greater for the latter.

CONCLUSION:

We conclude that in vivo transverse and axial ultrasound velocities provide highly precise information about the healing state of both linear and resection diaphyseal osteotomies, but the axial ultrasound velocity most likely has greater discriminatory power. This method has the potential for clinical application in humans.     

Totally robotic soave pull-through procedure for Hirschsprung’s disease: lessons learned from 11 consecutive pediatric patients

Pediatric Surgery International - Since Hirschsprung’s disease (HSCR) already proved to benefit from robotic surgery, we aimed at describing a wider series of patients with this rare disease...     

Serum phosphorylated neurofilament heavy-chain levels reflect phenotypic heterogeneity and are an independent predictor of survival in motor neuron disease

Journal of Neurology - To investigate the prognostic role and the major determinants of serum phosphorylated neurofilament heavy -chain (pNfH) concentration across a large cohort of motor neuron...     

A new POLG1 mutation with peo and severe axonal and demyelinating sensory–motor neuropathy

Background Progressive external ophthalmoplegia (PEO) is a mitochondrial disorder associated with defective enzymatic activities of oxidative phosphorylation (OXPHOS), depletion of mitochondrial DNA (mtDNA) and/or accumulation of mtDNA mutations and deletions. Recent positional cloning studies have linked the disease to four different chromosomal loci. Mutations in POLG1 are a frequent cause of this disorder. Methods We describe two first–cousins: the propositus presented with PEO,mitochondrial myopathy and neuropathy, whereas his cousin showed a Charcot– Marie–Tooth phenotype. Neurophysiological studies, peroneal muscle and sural nerve biopsies, and molecular studies of mtDNA maintenance genes (ANT1, Twinkle, POLG1, TP) and non dominant CMT–related genes (GDAP1, LMNA, GJB1) were performed. Results A severe axonal degeneration was found in both patients whereas hypomyelination was observed only in the patient with PEO whose muscle biopsy specimen also showed defective OXPHOS and multiple mtDNA deletions. While no pathogenetic mutations in GDAP1, LMNA, and GJB1 were found, we identified a novel homozygous POLG1 mutation (G763R) in the PEO patient. The mutation was heterozygous in his healthy relatives and in his affected cousin. Conclusions A homozygous POLG1 mutation might explain PEO with mitochondrial abnormalities in skeletal muscle in our propositus, and it might have aggravated his axonal and hypomyelinating sensory–motor neuropathy. Most likely, his cousin had an axonal polyneuropathy with CMT phenotype of still unknown etiology.     

Controlled multicenter trial of tiopronin and D-penicillamine for rheumatoid arthritis

Fifty-seven patients took part in a controlled double-blind trial between tiopronin and D-penicillamine as basic treatment for rheumatoid arthritis. Thirty-nine (19 receiving tiopronin, 20 receiving D-penicillamine) completed the trial after 1 year. Both drugs resulted in a decrease of the erythrocyte sedimentation rate, Ritchie index, and Lee index and in a sparing effect on symptomatic antiinflammatory therapy. Improvement in these variables was statistically highly significant at any interval with tiopronin, but was sometimes less or not at all significant with D-penicillamine. Nevertheless, the difference in effects between the 2 drugs never reached statistical significance. Six patients receiving tiopronin and 6 receiving D-penicillamine were taken out of the experiment because of side effects.