Focal neuromyotonia: do I love you?

We present a rare case of focal neuromyotonia in a 73-year-old woman with a follow up of 5years. The clinical picture showed a fixed contraction of the 3rd and 4th finger of the left hand. Similar to other published cases, our patient suffered from COPD and was treated with beta-2-sympathomimetics. This clinical picture shows a rare but rather salient differential diagnosis of Dupuytren's contracture. EMG of the affected muscles may yield a diagnosis and prevent the patient from a long and ineffective treatment "odyssey

Assessment of long-term donor-site morbidity after harvesting the latissimus dorsi flap for neonatal myelomeningocele repair

BACKGROUND AND AIM The latissimus dorsi flap (LDF) has been employed very successfully over decades to cover large soft-tissue defects. Its donor-site morbidity has been extensively investigated in adults - but not in children - and is considered to be nonrestrictive. The aim of this long-term study was to assess donor-site morbidity with the modified Constant score more than 8 years after coverage of large myelomeningocele (MMC) defects with a reverse latissimus dorsi flap. METHODS Within the first days after birth, the reverse latissimus dorsi muscle flap was used uni- or bilaterally in three neonates to cover a large MMC defect. Bilateral shoulder function was tested more than 8 years postoperatively according to the modified Constant score. RESULTS The mean age at follow-up was 11.7 years. None of the patients experienced any pain or shoulder restrictions during normal daily activities. They all managed to position both of their arms comfortably above the head. Forward flexion was normal in all patients as was abduction and external rotation. Dorsal extension was minimally reduced on the operated side. Internal rotation was symmetric in all patients; the extent of active movement varied from excellent to poor. CONCLUSIONS Our long-term data suggest that there is no specific and significant impairment of shoulder function after using the distally pedicled reverse LDF for neonatal MMC repair

Efficacy of cultured epithelial autografts in pediatric burns and reconstructive surgery.

BACKGROUND: Cultured epithelial autografts are regularly used in burn patients, but they have not been tested in patients undergoing reconstructive surgery. The aim of this study was to analyze and compare the efficacy of cultured grafts in both burn and reconstructive surgery patients. METHODS: In six children with severe and massive burns, full-thickness areas were grafted with cultured grafts. In another six children with hypertrophic or hyperpigmented scars, or both, cultured grafts were used to cover defects resulting from scar excision or deep dermabrasion. RESULTS: In burn surgery the final cover rate averaged 60% (range, 0% to 100%). The functional and cosmetic results were good and at least equivalent to results after conventional grafting. Fragility, infection, and, in particular, mechanical instability of cultured grafts during the first weeks after transplantation were the main problems encountered. In reconstructive surgery the final cover rate was 100% in all patients. The functional and cosmetic results were very good and considered better than those obtained by using conventional grafting techniques. No major management problems were encountered. CONCLUSIONS: In massively burned children, cultured epithelial autografts represent an effective additional and potentially lifesaving method to conventional grafting. Questions remain regarding the use of this technique to treat less severe burns. For resurfacing-type scar revisions, cultured epithelial autografts yield excellent results that appear to be superior to those of conventional techniques

Frequency and conduction velocity analysis of the abductor pollicis brevis muscle during early fatigue

The physiological behavior of the abductor pollicis brevis (APB) muscle during early stage of fatigue is important as a reference for future clinical assessment of a pathologically altered muscle, as e.g. in carpal tunnel syndrome. The purpose of this study was to assess changes of force and surface electromyograms (sEMG) during early stage of fatigue of the APB. Thumb abduction force and sEMG derived from a multi-electrode array were recorded during isometric contraction. Electrode placement over the innervation zone (IZ) and the muscle tendon interface were avoided. The sEMGs of two adjacent electrode pairs were selected for the analysis, which yielded (a) motor unit conduction velocities (MUCV) derived from a correlation analysis between the EMGs and (b) mean frequencies obtained by using either fast Fourier (FMF) or Wavelet Transform (WMF). Early fatigue resulted in a relative decay rate of force (-2.1%( *)s(-1)), MUCV (-1.5%( *)s(-1)), FMF (-4.1%( *)s(-1)), WMF (-3.7%( *)s(-1)) and in a change of the power spectrum shape. Lower mean frequencies were observed at greater distances from the IZ independently of fatigue. The APB muscle seems to be fast fatigable and the relative decay rate of mean frequency was significantly larger than the one of force and MUCV

Focal neuromyotonia: do I love you?

We present a rare case of focal neuromyotonia in a 73-year-old woman with a follow up of 5 years. The clinical picture showed a fixed contraction of the 3rd and 4th finger of the left hand. Similar to other published cases, our patient suffered from COPD and was treated with beta-2-sympathomimetics. This clinical picture shows a rare but rather salient differential diagnosis of Dupuytren's contracture. EMG of the affected muscles may yield a diagnosis and prevent the patient from a long and ineffective treatment "odyssey"

Markers to evaluate the quality and self-renewing potential of engineered human skin substitutes in vitro and after transplantation

We screened a series of antibodies for their exclusive binding to the human hair follicle bulge. In a second step these antibodies were to be used to identify basal keratinocytes and potential epithelial stem cells in the human epidermis and in engineered skin substitutes. Of all the antibodies screened, we identified only one, designated C8/144B, that exclusively recognized the hair follicle bulge. However, C8/144B-binding cells were never detected in the human epidermal stratum basale. In the bulge C8/144B-binding cells gave rise to cytokeratin 19-positive cells, which were also tracked in the outer root sheath between bulge and the hair follicle matrix. Remarkably, cytokeratin 19-expressing cells were never detected in the hair follicle infundibulum. Yet, cytokeratin 19-expressing keratinocytes were found in the epidermal stratum basale of normal skin as a subpopulation of cytokeratin 15-positive (not C8/144B-positive) basal keratinocytes. Cytokeratin 19/cytokeratin 15-positive keratinocytes decreased significantly with age. We suggest that cytokeratin 19-expressing cells represent a subpopulation of basal keratinocytes in neonates and young children (up to 1.5 years) that is particularly adapted to the lateral expansion of growing skin. Our data show that cytokeratin 19 in combination with cytokeratin 15 is an important marker to routinely monitor epidermal homeostasis and (at least indirectly) the self-renewing potential of engineered skin