Functional improvement in patients with Sprengel's deformity following Modified Green's procedure and simplified clavicle osteotomy-a study of forty cases.

PURPOSE: Sprengel's deformity though rare is the most common congenital anomaly of the shoulder region. The aim of the study was to check the effectiveness of the modified Green's procedure with simplified clavicle osteotomy in providing significant functional and radiological improvement without neurovascular complications. METHODS: Sixty-eight children of Sprengel deformity were operated by the modified Green's release. Of which forty patients (15 male, 25 female) with more than two years follow-up were evaluated retrospectively. Simplified clavicle osteotomy was done in 34 patients. Severity was graded clinically by Cavendish grading (Grade II-6, III-34) and radiologically by Rigault grading (Grade II - 35, III - 5). The average age at surgery was 5.05 years with a mean follow-up of 62 (24-145) months. RESULTS: The average improvement in Cavendish grade was 2.6 grades, and Rigault grading was 1.07 grades. Shoulder abduction improved by a mean of 360. There was no neurovascular complication in children who had simplified clavicle osteotomy, and one patient without clavicle osteotomy developed transient brachial plexus palsy. Other complications were wound gape, pleural tear and scapular winging. CONCLUSION: The modified Green's procedure with simplified clavicle osteotomy allows for excellent correction of function and cosmesis. We describe a simpler technique of clavicle osteotomy that is quick, safe and equally effective in preventing neurovascular complications. We also describe a surrogate clinical test to avoid potential neurological injury in the absence of neuromonitoring. To the best of our knowledge, this is the second-largest operative series of Sprengel's deformity.

Correspondence of continuous interstitial glucose measurement against arterialised and capillary glucose following an oral glucose tolerance test in healthy volunteers.

The aim of the present study was to validate the Glucoday continuous interstitial ambulatory glucose-monitoring device (AGD) against plasma glucose measured from arterialised venous (AV) and glucose from capillary whole blood (finger prick, FP) in non-diabetic subjects in response to an oral glucose tolerance test. Fifteen healthy overweight men (age 30-49 years, BMI 26-31 kg/m2) participated. Glucose levels were measured before, during and after consumption of an oral 75 g glucose load using twelve FP samples and forty-four 1 ml AV blood samples during 180 min. Interstitial glucose was measured via the AGD. Three venous samples for fasting insulin were taken to estimate insulin resistance. Profiles of AGD, AV and FP glucose were generated for each participant. Glucose values for each minute of the measurement period were interpolated using a locally weighted scatterplot smoother. Data were compared using Bland-Altman plots that showed good correspondence between all pairs of measurements. Concordance between the three methods was 0.8771 (Kendall's W, n 15, P < 0.001). Concordance was greater between AV and FP (W = 0.9696) than AGD and AV (W = 0.8770) or AGD and FP (W = 0.8764). Analysis of time to peak glucose indicated that AGD measures lagged approximately 15 min behind FP and AV measures. Percent body fat was significantly correlated with time to peak glucose levels for each measure, while BMI and estimated insulin resistance (homeostatic model assessment, HOMA) were not. In conclusion, AGD shows good correspondence with FP and AV glucose measures in response to a glucose load with a 15 min time lag. Taking this into account, AGD has potential application in nutrition and behaviour studies.

CT-Guided Percutaneous Drilling of Osteoid Osteoma: A Safe, Minimally Invasive and Cost-Effective Method.

BACKGROUND: CT-guided percutaneous drill resection (PDR) for osteoid osteoma fell out of vogue with the advent of radiofrequency ablation (RFA). However, the routine use of RFA is offset by its high cost and need for specialized instrumentation. AIMS AND OBJECTIVES: This study is an analysis of our series of patients treated with PDR, to know its efficacy, safety and cost-effectiveness. MATERIALS AND METHODS: This study is a retrospective analysis of prospectively collected data of 33 patients with a mean age of 10.03 years (2-21 years 13:10 M:F). The proximal femur was the commonest location, followed by shaft femur, tibial shaft, pubic ramus and lateral humeral condyle. Three were recurrences. The procedures were performed by one of four fellowship-trained paediatric orthopaedic surgeons, using a standard protocol with the help of a radiologist. CT-guided drilling was performed under sedation/short general anaesthesia using a guidewire for identification, followed by sequential drilling using a 6.5 mm cannulated drill or a triple ream drill. Patients were hospitalized overnight for pain relief and allowed protected weight bearing as per pain tolerance. They were evaluated weekly for 2 weeks and 3 monthly thereafter. RESULTS: The mean final follow-up was 19.72 months (6 months to 58 months). All patients had complete resolution of symptoms within 3 days of the procedure. There were no major complications and there were two minor complications (haematoma and cutaneous hypoaesthesia). There were no recurrences till the last follow-up. CONCLUSION: This study, one of the first and largest from India about CT-guided PDR, shows that this method can still be safely and effectively used as a primary treatment modality in OO, especially in economically challenged population. It is much more cost-effective than RFA, can be used even for recurrent tumours and can be safely be performed by a general orthopaedic surgeon without any special instrumentation.

Site-directed mutagenesis of a loop at the active site of E1 (alpha2beta2) of the pyruvate dehydrogenase complex. A possible common sequence motif.

Limited proteolysis of the pyruvate decarboxylase (E1, alpha2beta2) component of the pyruvate dehydrogenase (PDH) multienzyme complex of Bacillus stearothermophilus has indicated the importance for catalysis of a site (Tyr281-Arg282) in the E1alpha subunit (Chauhan, H.J., Domingo, G.J., Jung, H.-I. & Perham, R.N. (2000) Eur. J. Biochem. 267, 7158-7169). This site appears to be conserved in the alpha-subunit of heterotetrameric E1s and multiple sequence alignments suggest that there are additional conserved amino-acid residues in this region, part of a common pattern with the consensus sequence -YR-H-D-YR-DE-. This region lies about 50 amino acids on the C-terminal side of a 30-residue motif previously recognized as involved in binding thiamin diphosphate (ThDP) in all ThDP-dependent enzymes. The role of individual residues in this set of conserved amino acids in the E1alpha chain was investigated by means of site-directed mutagenesis. We propose that particular residues are involved in: (a) binding the 2-oxo acid substrate, (b) decarboxylation of the 2-oxo acid and reductive acetylation of the tethered lipoyl domain in the PDH complex, (c) an "open-close" mechanism of the active site, and (d) phosphorylation by the E1-specific kinase (in eukaryotic PDH and branched chain 2-oxo acid dehydrogenase complexes).

Substrate channelling in 2-oxo acid dehydrogenase multienzyme complexes.

Heteronuclear NMR spectroscopy and other experiments indicate that the true substrate of the E1 component of 2-oxo acid dehydrogenase complexes is not lipoic acid but the lipoyl domain of the E2 component. E1 can recognize the lipoyl-lysine residue as such, but reductive acylation ensues only if the domain to which the lipoyl group is attached is additionally recognized by virtue of a mosaic of contacts distributed chiefly over the half of the domain that contains the lipoyl-lysine residue. The lipoyl-lysine residue may not be freely swinging, as supposed hitherto, but may adopt a preferred orientation pointing towards a nearby loop on the surface of the lipoyl domain. This in turn may facilitate the insertion of the lipoyl group into the active site of E1, where reductive acylation is to occur. The results throw new light on the concept of substrate channelling and active-site coupling in these giant multifunctional catalytic machines.