Parental Perception Of Improvement In Prelinguistic Skills In Kids With Autism In Two Months Of Goal Directed Cognitive Approach

The study aimed to assess the parental perception of improvement in prelinguistic skills in children with Autism Spectrum disorder [ASD] after 1 and 2 months of Goal Directed Cognitive Approach. This was assessed by a questionnaire filled by parents of 26 consecutive ASD kids at a neurodevelopmental center. Parents were asked to rate these skills on a Likert scale of 1-5 before and after 1 month and 2 months of GDCA to check for any improvement. 69% of ASD kids were boys. Mean age at presentation was 2.6 years. The children were subjected to GDCA for an average duration of 13.7 months. Of these, 11.5% had seizures and 34.6 % had hyperactivity as a comorbidity. The average duration of sustained eye to eye contact at the time of presentation was 5.96 sec, which improved to 10.3 sec after 1 month ( p-0.0002) and further improved to 12.14 seconds at 2 months (p-0.005). Improvements in turn taking at 1 month( p-0.005) and after 2 months (p-0.002) and purposeful noises after 2 months of GDCA (p-0.003) and imitation after first month of treatment (p-0.0001) were significant. Responding and joint attention did not show any significant change with 2 months of therapy. Therefore, children with autism do improve significantly in eye contact, turn taking, imitation, purposeful noises with 2 months of GDCA. Long term follow up is required to assess responding and joint attention

Correction of Femoral Acetabular Impingement at the Time of Primary THA

Background: Primary total hip arthroplasty (THA) is considered one of the most cost effective and functionally beneficial procedures to treat end-stage coxarthrosis worldwide. However, in all regions of the world, there is a small percentage of patients that are plagued by residual anterior hip pain and have limited hip flexion. One explanation for this problem is bone and soft tissue impingement in the anterior hip region. In the native hip, the problem is described as femoral acetabular impingement (FAI). FAI is a form of developmental dysplasia of the hip (DDH). Not infrequently, these dysplastic acetabula are also retroverted. In primary THA, a retroverted boney acetabulum adversely affects prosthetic hip function. Specifically, when the acetabular cup is inserted in an anteverted position and the native acetabulum is retroverted, the proximal femur will still impinge upon the retroverted acetabular bone with flexion and internal rotation. This causes mechanical instability, pain, and prosthetic subluxation. We aptly name this condition prosthetic femoral acetabular impingement (PFAI). Methods: In this study we address PFAI with an anterior acetabular bone wall reduction (AABWR). In a consecutive series of 426 primary THA’s, we prospectively removed all impinging anterior retroverted bone during the THA procedure. All acetabular cups were placed between 25-35 degrees of anteversion. Retroverted acetabular bone extending beyond the acetabular cup was removed along with impinging capsular tissues. All femoral stems were positioned between 15-20 degrees. Results: The study group consisted of 426 THA’s. Three hundred patients (70%) had an AABWR. There were 140 females (47%) and 160 males (53%). The average amount of bone resected in the AABWR group was 1.32 cm (0.3 cm to 3.4 cm). For females, the average bone resection measured 1.1 cm (0.3 to 2.0 cm). For males, the average bone resection measured 1.53 cm (0.3 cm to 3.4 cm). Harris Hip Scores (HHS) at minimum of 1 year follow-up (range 1 to 11.5 years) averaged 91 (64 to 100) for the entire group. In the AABWR group, HHS averaged 92 (71 to 100). Average hip flexion was 110 degrees (100 to 130 degrees). In the non-AABWR group, HHS averaged 87 (71 to 100). Average flexion was 109 degrees (88 to 125 degrees). In the AABWR group, 12 patients (4%) experienced groin pain symptoms. On a scale from 0 to 4, the peak groin pain rating was 1 in 10 of the 12 patients and the remaining 2 rated his/her pain at a 2. As time progressed, 50% of these patients saw their groin pain resolve. In the non-AABWR group, 2 patients (1.6%) experienced groin pain and both patients rated his/her pain at a 1. Discussion: Maximizing hip flexion and function for the active patient undergoing primary THA requires meticulous surgical technique. PFAI may be one reason for unexplained anterior hip pain in the highly active patient that demands higher hip flexion and rotation. Our experience shows that the anterior acetabular rim and part of the anterior column can be removed at the time of primary THA without compromising the THA procedure. The AABWR is now an integral part of our primary THA technique

Changing women's roles, changing environmental knowledges: evidence from Upper Egypt

The aim of this paper is to investigate the ways in which changing gender roles in a Bedouin community in Upper Egypt, brought about by settlement over the last 20 years on the shores of Lake Nasser, have impacted on the accumulation and development of indigenous environmental knowledges by Bedouin women. The research was carried out among four groups of Ababda Bedouin in the Eastern Desert of Egypt and involved in-depth monthly conversations carried out over a period of 12 months. The main conclusions of the study are that the women of the study area have had to develop new knowledges which, in some cases, are now different from those held by men because of the different economic circumstances in which many find themselves; that these knowledges are fluid, dynamic and ever-changing with their own internal dynamism; and that socially constructed notions of gender are vital in the development process, notions that are sensitive to both men's and women's interests and their interrelationships

Ciprofloxacin-Resistant Neisseria meningitidis, Delhi, India

Decreased susceptibility of Neisseria meningitidis isolates to ciprofloxacin emerged from an outbreak in Delhi, India. Results of antimicrobial susceptibility testing of the meningococcal isolates to ciprofloxacin and further sequencing of DNA gyrase A quinolone-resistance–determining region confirmed the emergence of ciprofloxacin resistance in the outbreak

Mutations with pathogenic potential in proteins located in or at the composite junctions of the intercalated disk connecting mammalian cardiomyocytes: a reference thesaurus for arrhythmogenic cardiomyopathies and for Naxos and Carvajal diseases

In the past decade, an avalanche of findings and reports has correlated arrhythmogenic ventricular cardiomyopathies (ARVC) and Naxos and Carvajal diseases with certain mutations in protein constituents of the special junctions connecting the polar regions (intercalated disks) of mature mammalian cardiomyocytes. These molecules, apparently together with some specific cytoskeletal proteins, are components of (or interact with) composite junctions. Composite junctions contain the amalgamated fusion products of the molecules that, in other cell types and tissues, occur in distinct separate junctions, i.e. desmosomes and adherens junctions. As the pertinent literature is still in an expanding phase and is obviously becoming important for various groups of researchers in basic cell and molecular biology, developmental biology, histology, physiology, cardiology, pathology and genetics, the relevant references so far recognized have been collected and are presented here in the following order: desmocollin-2 (Dsc2, DSC2), desmoglein-2 (Dsg2, DSG2), desmoplakin (DP, DSP), plakoglobin (PG, JUP), plakophilin-2 (Pkp2, PKP2) and some non-desmosomal proteins such as transmembrane protein 43 (TMEM43), ryanodine receptor 2 (RYR2), desmin, lamins A and C, striatin, titin and transforming growth factor-β3 (TGFβ3), followed by a collection of animal models and of reviews, commentaries, collections and comparative studies

The selective post-translational processing of transcription factor Nrf1 yields distinct isoforms that dictate its ability to differentially regulate gene expression

Upon translation, the N-terminal homology box 1 (NHB1) signal anchor sequence of Nrf1 integrates it within the endoplasmic reticulum (ER) whilst its transactivation domains [TADs, including acidic domain 1 (AD1), the flanking Asn/Ser/Thr-rich (NST) domain and AD2] are transiently translocated into the ER lumen, whereupon the NST domain is glycosylated to yield an inactive 120-kDa glycoprotein. Subsequently, these TADs are retrotranslocated into extra-luminal subcellular compartments, where Nrf1 is deglycosylated to yield an active 95-kDa isoform. Herein, we report that AD1 and AD2 are required for the stability of the 120-kDa Nrf1 glycoprotein, but not that of the non-glycosylated/de-glycosylated 95-kDa isoform. Degrons within AD1 do not promote proteolytic degradation of the 120-kDa Nrf1 glycoprotein. However, repositioning of AD2-adjoining degrons (i.e. DSGLS-containing SDS1 and PEST2 sequences) into the cyto/nucleoplasm enables selective topovectorial processing of Nrf1 by the proteasome and/or calpains to generate a cleaved active 85-kDa Nrf1 or a dominant-negative 36-kDa Nrf1γ. Production of Nrf1γ is abolished by removal of SDS1 or PEST2 degrons, whereas production of the cleaved 85-kDa Nrf1 is blocked by deletion of the ER luminal-anchoring NHB2 sequence (aa 81–106). Importantly, Nrf1 activity is positively and/or negatively regulated by distinct doses of proteasome and calpain inhibitors