PR29MASSETERIC‐FACIAL NERVE COAPTATION – AN ALTERNATIVE TECHNIQUE FOR FACIAL NERVE REINNERVATION

Introduction Facial nerve paralysis can be a disabling condition functionally, psychologically and aesthetically. When there has been an acquired proximal injury to the facial nerve in the presence of previously functional facial musculature, such as in acoustic neuroma surgery, neurotisation of the distal facial nerve is an appropriate choice of management. The hypoglossal nerve is most commonly used. However this is not without its limitations, notably subsequent hemilingual atrophy and facial synkinesis. We present an alternative technique of facial reinnervation utilising a motor branch of the trigeminal nerve, the nerve to masseter. We believe this technique has the potential to overcome problems encountered with use of other extra‐facial nerves. Methods Three patients with acquired facial nerve palsy following tumour resection underwent transfer of the ipsilateral masseteric nerve to facial nerve. In two patients the nerve was directly coapted to the trunk of the facial nerve while the third patient had transfer to the buccal branch. Results By twelve months postoperatively all three patients demonstrated significant improvement in facial muscle tone and symmetry at rest. All patients were able to produce a symmetrical smile with minimal synkinesis. Two of the three patients also had evidence of occasional spontaneous movements. Conclusion Use of the ipsilateral motor nerve to masseter offers an alternative technique for neurotisation of the facial nerve. The advantages of this technique include ease of dissection, constant and reliable anatomy, powerful innervation of the facial muscles, minimal donor site morbidity and the potential for return of spontaneous facial movements.     

Safety of, and biological and functional response to, a novel metallic implant for the management of focal full-thickness cartilage defects: Preliminary assessment in an animal model out to 1 year.

Focal full-thickness cartilage lesions of the human medial femoral condyle (MFC) can cause pain and functional impairment. Affected middle-aged patients respond unpredictably to existing treatments and knee arthroplasty may be required, prompting risk of revision. This study assesses the safety of, and biological and functional response to, a metallic resurfacing implant which may delay or obviate the need for traditional arthroplasty. The anatomic contour of the surgically exposed MFC of six adult goats was digitally mapped and an 11 mm diameter full-thickness osteochondral defect was created. An anchor-based Co-Cr resurfacing implant, matching the mapped articular contour, was implanted. Each goat's contralateral unoperated femorotibial joint was used as a control. Postoperative outcome was assessed by lameness examination, radiography, arthroscopy, synoviocentesis, necropsy, and histology up to 26 (n = 3) or 52 (n = 3) weeks. By postoperative week (POW) 4, goats demonstrated normal range of motion, no joint effusion, and only mild lameness in the operated limb. By POW 26 the animals were sound with only occasional very mild lameness. Arthroscopy at POW 14 revealed moderate synovial inflammation and a chondral membrane extending centrally across the implant surface. Radiographs at POWs 14 to 52 implied implant stability in the operated joints, as well as subchondral bone remodeling and mild exostosis formation in the operated and contralateral unoperated joints of some goats. By POW 26, histology revealed new trabecular bone abutting the implant. At POWs 26 and 52 MFC cartilage was metachromatic and intact in the operated and unoperated femorotibial joints. Proximal tibiae of some operated and unoperated limbs demonstrated limited subchondral bone remodeling and foci of articular cartilage fibrillation and thinning. The chondral membrane crossing the prosthesis possessed a metachromatic matrix containing singular and clustered chondrocytes. Our data imply the safety, biocompatibility, and functionality of the implant. Focal articular damage was documented in the operated joints at POWs 26 and 52, but lesions were much reduced over those previously reported in untreated defects. Expanded animal or preclinical human studies are justified.     

Susceptibility to develop celiac disease is primarily associated with HLA-DQ alleles

We have recently reported that the susceptibility to develop celiac disease (CD) seems to be primarily associated to a particular combination of an HLA-DQA1 (DQA1*0501) and an HLA-DQB1 (DQB1*0201) allele: i.e., a particular DQ alpha/beta heterodimer. To investigate whether certain DP alleles might also contribute to the genetic susceptibility, DPA1 and DPB1 genes of 94 CD patients and 132 healthy controls were examined by probing in vitro amplified DNA with sequence-specific oligonucleotide probes corresponding to all hitherto known DPA1 and DPB1 alleles. The frequencies of the DPA1*0201 and of the DPB1*0101 alleles were increased in CD patients compared to healthy controls (0.31 versus 0.14 and 0.25 versus 0.08, respectively). However, these DP alleles were in linkage disequilibrium with CD-associated DQ alleles in the normal population, and the difference in frequency of these DP alleles was no longer significant when CD patients and healthy controls carrying the CD-associated DQA1*0501 and DQB1*0201 alleles were compared. DQB1*0201 homozygous individuals were overrepresented among DQB1*0201-positive patients compared to controls. When DQB1*0201 heterozygous patients and controls were compared, nearly identical frequencies of the DPA1*0201 and the DPB1*0101 alleles were found. Thus, the observed increase of the DPA1*0201 and DPB1*0101 alleles among CD patients seems mainly to be caused by linkage disequilibrium to the CD-associated DQ alleles.     

Is MED13L-related intellectual disability a recognizable syndrome?

Introduction

MED13L-related intellectual disability is characterized by moderate intellectual disability (ID), speech impairment, and dysmorphic facial features. We present 8 patients with MED13L-related intellectual disability and review the literature for phenotypical and genetic aspects of previously described patients.

Studies on cardiac distribution and function of neuropeptide Y

High concentrations of a novel peptide, neuropeptide Y, have been demonstrated in the guinea-pig and canine heart and in the latter, a particularly high concentration was found in the region of the coronary vasculature (126 +/- 31 pmol g-1). Intra-arterial infusion of neuropeptide Y for 30 s into the coronary artery of the intact, innervated dog heart resulted in a rapid and short-lasting reduction of blood flow from 38 +/- 4 to 31 +/- 3 ml min-1 (P less than 0.05) to resume control level, 39 +/- 5 ml min-1, within 5 min. These injections were unaccompanied by changes in heart rate and aortic pressure, while there was an associated small reduction in dP/dt, used as a measure for changes in contractility. In vitro studies using the isolated, paced papillary muscle from cat, guinea-pig and rat, and spontaneously beating right atria from the guinea-pig, demonstrated no effect of NPY on active tension or beating frequency. The results indicate that NPY has vasoconstrictor properties, but under the test circumstances to lack both positive and negative inotropic and chronotropic effects.     

Genetic analysis of the CD28/CTLA4/ICOS (CELIAC3) region in coeliac disease

Abstract:  In order to extend our previous findings of genetic linkage to the CD28/CTLA4/ICOS region on chromosome 2q33 ( CELIAC3 ) in coeliac disease (CD), we have investigated 22 genetic markers in 325 Norwegian/Swedish multiplex and simplex CD families. We found both linkage and association with several markers, primarily in the multiplex material. We observed strong linkage disequilibrium (LD) between SNPs (Single Nucleotide Polymorphisms) within an LD block delimited by MH30 and D2S72. A haplotype of this region marked by the alleles −1147*T: + 49*A:CT60*G:CT61*A was significantly associated with CD, suggesting that one or more polymorphisms of this haplotype, possibly −1147*T, are involved in CD susceptibility. The CT60 SNP, a polymorphism found to be most strongly associated with some other immune-mediated diseases, was not associated with CD, as this SNP was part of both associated and non-associated haplotypes. Moreover, our results suggest that CELIAC3 harbours several independent loci contributing to CD susceptibility.     

Effect of dietary supplement on nutritional status and clinical outcome in 501 geriatric patients--a randomised study

The present study was designed to evaluate to the effect of dietary supplements on clinical outcome and nutritional status in a large group of geriatric patients (n = 501). The patients were randomised into an experimental group which received nutritional supplementation (400 kcal) as well as a standard hospital diet, and a control group on hospital diet alone. The nutritional state was measured on admission and after 8 and 26 weeks by anthropometry, serum protein analysis and a delayed hypersensitivity skin test. Protein energy malnutrition was defined as the presence of three or more abnormal parameters. 28.5% of patients showed evidence of malnutrition on admission. Hospitalisation itself resulted in a gradual deterioration in nutritional status. Nutritional supplementation generally improved nutritional state. Among those patients who were well nourished on admission, and subsequently receiving dietary supplementation, 8.3% fulfilled malnutrition criteria after 26 weeks, while 21.1% were considered malnourished in the control group (p < 0.05). The improvement observed in transport proteins was probably related to nutritional support and not just to the reversal of inflammation. In the initially well nourished group of more than 300 patients, the mortality rate was 8.6% in those given nutritional support compared to 18.6% in the control group (p < 0.02).     

Essential fatty acids in the nutrition of severely neurologically disabled children

Essential fatty acids (EFA) are important for the normal development and functioning of the brain, retina and immune competent cells. Severely neurologically handicapped children often have feeding difficulties, and the composition of the diet may be critical with respect to an optimal nutrient content. The aim of the present investigation was to evaluate if the dietary intakes and serum phospholipid concentrations of EFA were adequate in a group of severely neurologically impaired children in an institution. To achieve this, a prospective study was done. The investigation showed low dietary intakes of both n-6 fatty acids (FA) and n-3 FA. The serum concentrations of total n-6 FA, linoleic acid and 22:6n-3 (docosahexaenoic acid) as proportions of the total serum phospholipid FA concentration were initially low. The serum concentrations of 20:3n-9 and 22:5n-6 cholesterol, triacylglycerol, total saturated FA, total monounsaturated FA and apolipoproteins A-I and B were high compared with levels in a reference group of healthy children. Following supplementation with fish oil and soyabean oil, the serum lipid profile approached normal. We conclude that the study children had suboptimal intakes of EFA and that elevated serum concentrations of 20:3n-9 and 22:5n6 were useful serological markers of suboptimal EFA status. Recommended dietary allowances for EFA given as a percentage of energy underestimate EFA requirements in children with a low energy intake. Severely disabled children with feeding difficulties should probably be monitored with serum phospholipid FA measurements or calculation of dietary absolute intakes of EFA.     

对发展中国家改善用药的10点建议

WHO建议改善药品管理的工作要在国家药物政策保障之下.在许多国家,执行国家药物政策的机制是实施国家基本药物计划,其要点是强调公共领域的药品选择、采购、流通与使用的合理性.不适当的处方使医疗质量降低并导致资源浪费.本文以探讨在国家药物政策范畴内鼓励更合理地使用药品的问题为重点,在已有证据的基础上,详细阐明基本药物计划内容中的合理用药问题.本文评述了在发展中国家改善用药状况的有效策略及最新知识,并为决策者与管理者提出达到改善用药目标的建议.     

Risk factors for the development of PTSD and depression among child and adolescent victims following a 7.4 magnitude earthquake

Objective. PTSD and major depression occur frequently following traumatic exposure, both as separate disorders and concurrently. Although much of Turkey is under threat of severe earthquakes, risk factors for developing psychiatric disorders among Turkish children have not yet been studied. The aim of the study was to examine risk factors for PTSD and depression develpoment in children. Method. A total of 160 survivors (102 girls and 58 boys) severely impacted by Turkey's 7.4-magnitude quake participated in a psychiatric interview 6–20 weeks after the disaster. The mean age was 14.43. Logistic regression was used to test effects of pre-disaster, disaster-related and post-disaster factors on diagnoses, yielding odds ratios (OR). Results. CAPS indicated that 96 (60%) had PTSD, and psychiatric interview found 49 (31%) with depression. Children diagnosed with PTSD were more likely to have witnessed death (OR=2.47) and experienced an extreme parental reaction (OR=3.45). Children with depression were more likely to be male (OR=4.48), have a higher trait anxiety score (OR=1.12 for every additional point), sustain injury (OR=4.29), and have lost a family member in the quake (OR=10.96). Focusing on the 96 children with PTSD, those with comorbid depression were more likely male, have a higher trait anxiety score, and have lost of family member. Conclusions. Mental health professionals should offer support to children witnessing death or losing a family member in a disaster. The ability of the family to remain calm and reassuring also may be a key factor in preventing PTSD.