CASE REPORT Moebius syndrome with macular hyperpigmentation, skeletal anomalies, and hypoplasia of pectoralis major muscle in an Egyptian child

We report a 4 month old female infant, 3rd in order of birth of the first cousin consanguineous parents. The patient has congenital right facial nerve palsy, with asymmetry of facial expression during crying and difficulty in swallowing. Associated anomalies include abnormal facial features, bilateral finger anomalies, bilateral talipes equinovarus, kyphoscoliosis, hypotonia, high frequency hearing loss. Bilateral macular hyperpigmentation was detected in our patient on fundus examination which was not reported previously in Moebius syndrome cases. In addition there is hypoplasia of the right pectoralis major muscle.KEYWORDS Moebius syndrome; Macular hyperpigmentation; Pectoralis major muscle; Cranial nerves; Ptosis; Facial nerve pals

Case Report: Trichorhinophalangeal syndrome II, expanding the clinical spectrum

We report a 4.5 year old Egyptian male child, fourth in the order of birth of healthy remote consanguineous parents. He has typical facial as well as skeletal features of Trichorhinophalangeal syndrome (TRPS) II. The facial features included bilateral downward slanting palpebral fissures, bulbous nose, long filtrum, retromicrognathia, sparse hair in the scalp and thick eyebrows. The skeletal features included retarded bone age, cone shaped epiphyses of the phalanges and multiple exostoses. The patient has also growth retardation, moderate mental retardation and hyperlaxity of the right knee joint. However our patient has some features not reported in TRPS II patients. These included bilateral partial ptosis, long eye lashes, preauricular skin tag, short 2nd right finger, short metacarpals of both thumbs. So we have to expand the clinical spectrum. Karyotype demonstrated 46,XY,del 8(q23.3-q24.1).Keywords: Trichorhinophalangeal syndrome; Langer–Giedion syndrome; Exostosis; Short stature; Cone shaped epiphyse

Pemanfaatan Sumberdaya Desa Dan Optimalisasi Penggunaan Media Sosial Instagram

Secara geogrfis, Desa prijekngablak terletak diwilayah kecamatan karanggeneng Kabupaten Lamongan. Disebelah utara berbatasan dengan Desa karangrejo, disebelah timur berbatasan dengan Desa njuwet, disebelah selatan berbatasan dengan desa karangrejo. Secara umum mata pencaharian warga desa prijekngablak adalah sebagai petani baik tambak maupun petani padi. Terletak berhadapan dengan sungai Bengawan solo. Desa prijekngablak memiliki jajanan khas yaitu kerupuk ladu dan Recek (Rengginang). Namun masyarakat masih kurang pemahaman akan produksi dan pemasaran yang baik dan dapat menarik minta konsumen. Sehingga masyarakat di wilayah lain kurang mengetahui informasi tentang jajanan khas Desa Prijekngablak. Jika hal tersebut tidak dikelola dengan baik maka akan berakibat bagi perkembangan bisnis ladu dan rengginang. Hasil dari program kerja yang diadakan oleh program studi Manajemen kelompok 09 dengan membantu masyarakat dalam hal Produksi, packaging, dan Marketing agar dapat membantu masyarakat dalam keberlangsungan bisnisnya. Dalam hal produksi, Recek (Rengginang) akan dimodifikasi menjadi rengginang mini yang diberi rasa-rasa di atasnya. Lalu untuk packaging, akan diberi kemasan yang lebih menarik yaitu K-Pack dan diberi stiker. Sedangkan untuk pemasaran, rengginang akan di jual melalui media sosial agar masyarakat luas

Cornelia-de Lange syndrome in an Egyptian infant with unusual bone deformities

We report a 4 month old female infant with the typical features of Cornelia-de Lange syndrome. What was striking in our patient was the presence of skeletal anomalies not reported previously. These included arachnodactly of both fingers and toes, flexion of thumbs at metacarpophalengeal joints, bilateral short big toes, angulation of the lower part of the bones of right forearm and both legs with multiple skin folds. Also biochemical and X-ray evidence of rickets was detected mostly due to malnutrition and failure to thrive. The patient died at the age of 5 months with bronchopneumoniaand gastroenteritis

Encouraging Optimal Aging through a Lifestyle Based on Islamic Psychospiritual Science

Ageing is an inevitable natural process that can give rise to concerns at both individual and societal levels if strategic planning is not in place to address the associated changes and ensure the well-being and satisfaction of this population. This article aims to explore the significance of healthcare for the elderly and the application of Islamic psychospiritual science in promoting healthy ageing by emphasizing lifestyle factors. The research employed a survey instrument administered to 307 randomly selected respondents, including elderly individuals and caregivers. The study was conducted across Malaysia from February 2022 to April 2022. The collected data were analyzed using the Statistical Package for the Social Sciences (SPSS) version 22 software. The study findings indicate that all the elements encompassing an Islamic psychospiritual lifestyle, including the physical, psychological, spiritual, and social aspects, hold great significance. The Cronbach's alpha (α) score for this study was calculated at 0.934, indicating a high level of internal consistency. The results of this study offer valuable insights for family members, caregivers, welfare organizations, nursing homes, and other entities involved in elderly care. These findings can guide the optimization of care aspects, particularly in light of the challenges posed by the Covid-19 pandemic. Additionally, the study's outcomes can inform the development of modules or guidelines for elderly care, facilitating the caregiving process

Shear Modes, Criticality and Extremal Black Holes

We consider a (2+1)-dimensional field theory, assumed to be holographically dual to the extremal Reissner-Nordstrom AdS(4) black hole background, and calculate the retarded correlators of charge (vector) current and energy-momentum (tensor) operators at finite momentum and frequency. We show that, similar to what was observed previously for the correlators of scalar and spinor operators, these correlators exhibit emergent scaling behavior at low frequency. We numerically compute the electromagnetic and gravitational quasinormal frequencies (in the shear channel) of the extremal Reissner-Nordstrom AdS(4) black hole corresponding to the spectrum of poles in the retarded correlators. The picture that emerges is quite simple: there is a branch cut along the negative imaginary frequency axis, and a series of isolated poles corresponding to damped excitations. All of these poles are always in the lower half complex frequency plane, indicating stability. We show that this analytic structure can be understood as the proper limit of finite temperature results as T is taken to zero holding the chemical potential fixed.Comment: 28 pages, 7 figures, added reference

Thermal Stability and Performance Testing of Oil based CuO Nanofluids for Solar Thermal Applications

For solar thermal systems, nanofluids have been proposed as working fluids due to their enhanced optical and thermal properties. However, nanoparticles may agglomerate over time, heating and thermal cycles. Even though pristine nanofluids have be proven to enhance performance in low temperature application, it is still unclear if nanofluids can meet the reliability requirements of solar thermal applications. To investigate this, the present study conducted experiments with several formulations of oil‐based CuO nanofluids in terms of their maximum operational temperature and their stability upon cyclic heating. In the samples tested, the maximum temperature ranged from 80oC to 150 22 oC and the number of heating cycles of ranged from 5 to 45, with heating times of between 5 to 60 minutes. The results showed that heating temperature, heating cycles, and heating time all exacerbated agglomeration of the samples. Following these experiments, orthogonal experiments were designed to improve the preparation process and the resultant thermal‐impulse stability. Thermal properties of these samples were characterized and thermal performance in an ‘on‐sun’ linear Fresnel solar collector was measured. All tests revealed that thermal performance of solar collecting system could be enhanced with nanofluids, but that thermal stability still needs to be further improved for industrial applications

Investigating the Impact of E-Servicescape Dimensions and Experience & Risk Factors on E-WOM: Mediating Role of Trust

Nowadays, one of the important topics in e-commerce which has become a concern of many countries is electronic word of mouth due to increasing use of Internet for activities such as shopping or obtaining information. The purpose of this study is to investigate the effect of e-service factors as well as experience and risk on E-WOM by considering the mediating role of the trust variable. The statistical population of this research consists of buyers ftom Tehran/Karaj from Alibaba website. 384 samples were selected by Cochran's formula and data was collected by distributing questionnaires. The research data were analyzed by structural equation modeling using Amos. The results show that e-services dimensions as well as experience have a positive and significant effect on trust and E-WOM. Trust also has positive and significant effect on E-WOM. The results emphasize the importance of focusing on improving the quality of e-services in online stores and user experience of buyers on their participation in advertising

A systematic review of variables used to assess clinically acceptable alignment of unilateral transtibial amputees in the literature.

Prosthetic alignment is a subjective concept which lacks reliability. The outcome responsiveness to prosthetic alignment quality could help to improve subjective and instrument assisted prosthetic alignment. This study was aimed to review variables used to assess clinically acceptable alignment in the literature. The search was done in some databases including: Google Scholar, PubMed, EBSCO, EMBASE, ISI Web of Knowledge and Scopus. The first selection criterion was based on abstracts and titles to address the research questions of interest. The American Academy of Orthotics and Prosthetics checklists were used for paper risk of bias assessment. A total of 25 studies were included in this study. Twenty-four studies revealed the critics of standing position or walking to locate clinically acceptable alignment, only one study measured outcomes in both situations. A total of 253 adults with transtibial amputations and mean age of 48.71 years participated in included studies. The confidence level of included studies was low to moderate, and before-after trial was the most common study design (n = 19). The joint angle, load line location with respect to joints and center of pressure-related parameters were reported as sensitive outcomes to prosthetic alignment quality in standing posture. The amount of forces at various parts of gait cycle and time of events were sensitive to prosthetic alignment quality during walking. Standing balance and posture and temporal parameters of walking could help to locate clinically acceptable alignment.N/