Recessive mutations in the LAMA2 gene lead to congenital muscular dystrophy type 1A and limb girdle muscular dystrophy R23 with complete or partial laminin α2 chain deficiency. Complete laminin α2 chain deficiency presents with early onset of severe hypotonia and generalized weakness, whereas partial deficiency shows a milder and more variable course with limb girdle weakness. Here, we report a child with mildly delayed motor development, elevated serum creatine kinase levels (>1000 U/l) and brain white matter hypointensity, indicative of laminin α2 chain deficiency. In addition to a stop gain variant in exon 39, the patient was found to carry an intronic insertion of 72 bp in intron 38 of the LAMA2 gene in trans. RNA analysis revealed that this insertion results in abnormally spliced as well as wild type transcript, which explains the partial laminin α2 chain deficiency observed in the muscle biopsy.In-patient hypertension is a common problem seen in the hospital setting. Current evidence-based guidelines define and address management of hypertension in ambulatory care and hypertensive emergencies in the hospital setting. However, they lack guidance for the management of acute asymptomatic/non-emergent hypertension in the hospitalised patient. The risk-benefit of treating inpatient asymptomatic hypertension is largely unknown. In this narrative review, we discuss current evidence-based perspectives to address this clinical entity. Many of the mineral metabolite abnormalities encountered in chronic kidney disease (CKD) patients were found also associated with acute kidney injury (AKI). In the last decade, sclerostin was found to intimately affect bone mineral metabolism in CKD patients. Nothing is known about sclerostin in AKI. We looked for serum level of sclerostin in AKI patients in comparison to normal control subjects and if there is an impact on metabolic derangement, endothelial function or clinical outcome. This is a cross sectional case control observational study of 219 AKI cases (group I) beside 219 age matched normal control subjects (group II). All cases of group I were in the intensive care because of sepsis; 86 had acute on CKD (group Ib), while 133 had de novo AKI (group Ia). All studied subjects underwent estimation of serum sclerostin, parathyroid hormone (PTH), 25 hydroxy vitamin D (25 OH vit D), fibroblast growth factor 23 (FGF23), C-reactive protein (CRP), interleukin 6 (IL6), Homeostatic Model Assessment for had strong correlation with FGF23 in group I (r=0.99, P<0.001) and group II (r=1, P<0.001). Homa IR had positive correlation with serum sclerostin (r=0.148, P=0.014) and serum FGF23 (r=0.142, P=0.018) in group I. Sclerostin is intimately related to FGF23. Sclerostin level increases in AKI patients. Both sclerostin and FGF23 might increase insulin resistance but have no impact on FMD. Neither sclerostin nor FGF23 interfere with AKI outcome.Sclerostin is intimately related to FGF23. Sclerostin level increases in AKI patients. click here Both sclerostin and FGF23 might increase insulin resistance but have no impact on FMD. Neither sclerostin nor FGF23 interfere with AKI outcome.Non-invasive wearable biosensors provide an efficient way of continuously quantifying a person's biochemical parameters, and are highly valuable for predicting human physiological status and flagging risks and illness. Commercial wearable sensors are available for tracking a user's physical activities, but few could monitor user's health conditions through sweat analysis. Electronic textile (e-textile) biosensors enable new applications in this scenario because of its high flexibility/wearability, low cost, high level of electronic integration, and unobtrusiveness. However, challenges in developing e-textile sweat biosensors remain in the production of textile-based biosensing materials, skin interfacing design, and embedded data acquisition/transmission. Here, we propose a novel wearable electrochemical sweat biosensor based on conductive threads decorated with zinc-oxide nanowires (ZnO NWs) and apply it to detecting lactate and sodium in perspiration during physical exercise. The sweat biosensor is fully integrated with signal readout and data communication circuits in a wearable headband and is capable of monitoring human sweat accurately and wirelessly. We achieved the detection of lactate and sodium in linear ranges of 0-25 mM and 0.1-100 mM and limits of detection of 3.61 mM and 0.16 mM, respectively, which cover the clinically-relevant ranges of lactate and sodium in human sweat. We demonstrated accurate lactate and sodium measurements in human sweat from a healthy volunteer, and the results are in good agreement with standard test results. We also conducted on-body measurements on the same human volunteer during exercise and confirmed the robustness of the signal readout during body movements and the excellent accuracy of the testing results. Total elbow arthroplasty for the treatment of patients with severe elbow osteoarthritis is associated with postoperative activity limitations and risk of midterm complications. Elbow denervation could be an attractive therapeutic option for young, active patients. The aim of our study was to assess the feasibility of selective total elbow denervation via 2 anteriorly based approaches. Selective total elbow denervation was performed in 14 cadaver elbows by 2 fellowship-trained elbow surgeons. Lateral and medial approaches to the elbow were used. The length of skin incisions and the minimum distance between them were noted. The number of articular branches identified and their respective distances from the lateral or medial epicondyle of the humerus were recorded. The anterolateral and anteromedial approaches allowed for the identification of all mixed and sensory nerves in all 14 cases. The mean number of resultant articular branches per cadaver was 1 for the musculocutaneous nerve, 2 (range, 1-3) for therve of the forearm, as well as medial antebrachial cutaneous nerves when carrying out this procedure. Radiation therapy (RT) to the head and neck (H&N) region is critical in the management of various pediatric malignancies; however, it may result in late toxicity. This comprehensive review from the Pediatric Normal Tissue Effects in the Clinic (PENTEC) initiative focused on salivary dysfunction and dental abnormalities in survivors who received RT to the H&N region as children. This systematic review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) method. Of the 2,164 articles identified through a literature search, 40 were included in a qualitative synthesis and 3 were included in a quantitative synthesis. The dose-toxicity data regarding salivary function demonstrate that a mean parotid dose of 35 to 40 Gy is associated with a risk of acute and chronic grade ≥2 xerostomia of approximately 32% and 13% to 32%, respectively, in patients treated with chemo-radiation therapy. This risk increases with parotid dose; however, rates of xerostomia after lower dose exposure have not been reported.