Fragile X Syndrome and Attentional Deficits

This study examined whether boys with Fragile X syndrome have a characteristic behavioural profile associated with the underlying genetic abnormality. Parents of 49 boys with Fragile X syndrome, 45 boys with Down's syndrome and 42 boys with intellectual disabilities of unknown aetiology were interviewed. Measures included the Childhood Behaviour Checklist, parent and teacher versions (CBCL), Parental Account of Childhood Symptoms (PACS) and Vineland social age and social quotient derived from the MRC Schedule of Handicaps, Behaviour and Skills (HBS).     

Expression of c-erbB-2 protein in keratinocytes of oral mucosal lichen planus and subsequent squamous cell carcinoma

Oral mucosal lichen planus (OMLP) is a well recognized mucosal disease with unknown etiology. Considerable controversy exists as to whether OMLP is intrinsically premalignant, or if the disorder facilitates the development of oral mucosal squamous cell carcinoma (OMSCC) by external factors. The aim of the present study was to investigate the expression of c-erbB-2 protein in the keratinocytes of initial biopsies of oral mucosal disorders diagnosed as OMLP with no evidence of epithelial dysplasia. and to compare the results with the expression of c-erbB-2 protein in subsequent biopsies obtained from the same patients. These results were compared with the findings from control groups (patients with dysplasia with no evidence of OMLP, patients with OMSCC with no evidence of OMLP and normal oral mucosa). The expression of the c-erbB-2 protein was evaluated by immunohistochemical staining of the gene product with the avidin-biotin-complex method using paraffin-embedded tissue sections. Five of the initial biopsies from patients with OMLP expressed the c-erhB-2 protein and one did not. None of the OMLP cases that subsequently showed evidence of dysplasia expressed the c-erhB-2 protein, and of the three OMSCC specimens from the patients with OMLP. two were negative and one expressed c-erbB-2 protein. The specimens from the control groups all expressed the c-erhB-2 protein. The results indicated the probability of the absence of c-erbB-2 staining being an indication of a potential for neoplastic transformation in OMLP with dysplastic changes.     

CHILDREN'S CONSENT TO PSYCHIATRIC TREATMENT: ALL OR NOTHING?

An adolescent with a history of attempted suicide who refused to involve his father in the treatment plan is presented. Issues surrounding consent to psychiatric treatment in childhood are discussed and the assessment process outlined. Contributions by primary healthcare professionals are emphasised.     

Structure and solution conformation of the cytostatic cyclic tetrapeptide WF-3161, cyclo[L-leucyl-L-pipecolyl-L-(2-amino-8-oxo-9, 10-epoxydecanoyl)-D-phenylalanyl]

The sequence and configuration of amino acids in the cytostatic cyclic tetrapeptide WF-3161 are established as cyclo(L-Leu-L-Pip-L-Aoe-D-Phe) where Pip = pipecolic acid and Aoe = 2-amino-8-oxo-9,10-epoxydecanoic acid. In chloroform, WF-3161 adopts a conformation with a possible gamma-turn between Leu NH and Aoe C = O and a cis amide bond between Leu and Pip. The torsion angles for this conformation are L-Aoe, phi, -95 degrees, psi, +85 degrees, omega, -155 degrees; D-Phe, phi, +120 degrees, psi, -80 degrees, omega, -175 degrees; L-Leu, phi, -145 degrees, psi, +35 degrees, omega, -10 degrees; L-Pip, phi, +20 degrees, psi, -135 degrees, omega, -170 degrees. The cis,trans,trans,trans amide bond sequence is related to the dimethyl sulfoxide conformation of chlamydocin, another cytostatic cyclic tetrapeptide.     

Early Results of the Mau Osteotomy for Correction of Moderate to Severe Hallux Valgus: A Review of 24 Cases

In our retrospective study, we report the objective results of the Mau osteotomy in the treatment of hallux valgus. We reviewed the results of 24 cases of moderate to severe hallux valgus deformities corrected with the Mau osteotomy of the first metatarsal combined with a distal soft-tissue procedure. Follow-up was possible in 24 cases. Preoperatively the mean hallux valgus and first intermetatarsal angles were 31.3 degrees and 16.6 degrees respectively, and were corrected postoperatively to an average of 13.00 degrees+/-7.15 degrees and 9.80 degrees+/-2.43 degrees respectively (P< .001). In the sagittal plane, the first metatarsal was shortened by an average of 2.00 mm. Two (8.3%) cases had dorsal elevation of the osteotomy fragment. Complications included 3 recurrences of the deformity, 1 frank nonunion, 8 dorsal cortical nonunions, 5 cases of undercorrection, and 1 case of broken hardware that was present in the nonunion that went on to revision. There were no superficial or deep infections, and no cases of transfer metatarsalgia were noted. In this series, the use of an oblique first metatarsal osteotomy with a dorsal shelf resulted in reliable and powerful correction of the first intermetatarsal angle in patients with moderate to severe hallux valgus. Particular attention should be paid to severe IM angles and the possibility of undercorrections. Despite ambulation postoperatively, the Mau osteotomy minimized dorsal malunion and the incidence of transfer metatarsalgia. LEVEL OF CLINICAL EVIDENCE: 4.     

Digital mucoid cyst excision by using the bilobed flap technique and arthroplastic resection

There are a number of proposed causes and treatment approaches for digital mucoid cysts. The described treatment outcomes for this cyst have been variable, with the highest success rate reported with complete excision and single-lobe skin flap closure. This report describes a bilobed flap reconstruction in conjunction with resection of the head of the middle phalanx. A retrospective review was undertaken to evaluate the recurrence rate, complications, and patient satisfaction with this combined procedure. Fifteen patients with an average follow-up of 4.6 years were evaluated. There were no recurrences, flap failures, or other major complications. The use of this flap allows for greater exposure than traditional semi-elliptical incisions while allowing the wide excisional defects to be closed primarily.     

Best-practice algorithms for the use of a bilayered living cell therapy (Apligraf®) in the treatment of lower-extremity ulcers

Tissue-engineered skin substitutes such as Apligraf have emerged over the past 20 years as among the most carefully studied and efficacious of the advanced wound modalities. These products have been proven as effective enhancements to general wound care, promoting wound closure particularly in instances where conventional wound care fails. Marketed for hard-to-heal wounds since 1998, Apligraf has become part of standard wound care in many wound centers across the United States. Despite this situation, few general wound care guidelines incorporate advanced and active wound-healing technologies, such as tissue-engineered skin products. Because of this deficiency, appropriate patient selection and proper use of these product remain largely unaddressed within the general wound care community. Here, we describe the development of guidelines surrounding optimal use of the bilayered living cell therapy, Apligraf, in the treatment of the two types of lower extremity ulcers for which the product is FDA approved: venous leg ulcer and diabetic foot ulcer. The guidelines detailed in this article focus on the identification and selection of patients who are at risk for failure of standard wound care therapy and thus appropriate for Apligraf treatment. The intended audience for these guidelines is the general wound care practitioner, for whom the developed treatment algorithms and accompanying figure legends should provide practical, user-friendly direction simplifying both patient selection and appropriate use of Apligraf within the context of good wound-healing practice.