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A wide range of endeavors has been made to propose various approaches to reduce Greenhouse gas emissions in the agricultural sector. The present study investigates the impacts of Surface Water Distribution Systems (SWDS) modernization in reducing groundwater overexploitation, energy consumption, and carbon emission in the agriculture sector. Four modernization alternatives, including an improved manual-based system (A1, A2), off-line, and real-time automatic control systems (A3, A4), are developed and tested on a real test case in Central Iran, which is confronted with severe water shortages. The results reveal that SWDS's operating system modernization improves 4 ~ 21% surface water distribution through the alternatives A1 ~ A4. This surface water distribution enhancement led to groundwater over-extraction reduction. Spatial analysis reveals that 0.075, 0.100, 0.281, and 0.470 of the irrigation district's cultivated area was thoroughly fulfilled by the delivered surface water and no need for groundwater extraction due to alternatives A1 ~ A4, respectively. Closure of several active tubewells up to 1,668 semi-deep and 497 deep tube-well were verified. SWDS' modernization led to 5, 7, 20, and 30% of energy consumption and consequently 1,864.90, 2,714.33, 8,427.19, and 12,674.32 tC ha-1 carbon emission reduction in alternatives A1 ~ A4, respectively. This study's results show that modernization of off-farm operating systems - responsible for surface water conveyance and distribution from a dam reservoir to farms - resulted in significant environmental benefits through improving the reliability of systems supplied by surface water and reducing the tendency of the farmers to groundwater resources. [ABSTRACT FROM AUTHOR]

Rectal gland is an important organ for osmoregulation in the sharks, and has species-specific features which are dependent on fish size. In this investigation, 36 individual of Brown banded Bamboo sharks (Chiloscyllium punctatum) were caught from Dervish's Creek located at the northern of Persian Gulf in both autumn and spring seasons. After weighting, biometry, sex determination and the numbering of sharks, the rectal gland were removed and weight were measured and then immediately fixed in Bouinâs solution. Histological sections of 5 micrometers were prepared and stained with Hematoxylin and eosin and then studied by using light microscope.The morphological results showed that about the size of the rectal gland, there was no significant difference between males and females (p>0.05) but the smaller sharks have larger rectal glands in proportion to their body size comperated to larger sharks (p

Ramp lesions (RLs) are peripheral lesions that occur in the posterior part of the medial meniscus or where it attaches to the joint capsule. The classification of the medial meniscus RLs has been the focus of numerous studies and publications. This review provides an overview of RL's current classification and treatment options in anterior cruciate ligament deficient knees. The study also aims to present a more practical classification system for RLs to assist in treatment decision-making. For the first time, we also presented a new surgical treatment for incomplete inferior and double-complete RL based on the posterior knee arthroscopy that provides direct access to the posterior meniscal borders, enabling effective treatment and stronger biomechanical repair., Level of Evidence: Level V., Competing Interests: The authors declare no conflict of interest., (© 2024 The Author(s). Journal of Experimental Orthopaedics published by John Wiley & Sons Ltd on behalf of European Society of Sports Traumatology, Knee Surgery and Arthroscopy.)

Background: The aim of this study was to compare the clinical results of kinematic alignment (KA) with those of mechanical alignment (MA) in single-stage bilateral total knee arthroplasty., Methods: In this double-blinded randomized controlled trial, 65 patients who had bilateral knee osteoarthritis underwent simultaneous bilateral total knee arthroplasty. One knee was randomly selected to be operated on with the calipered-KA technique and the other with MA. The participants were assessed via the Oxford Knee Score, Western Ontario and McMaster Universities Osteoarthritis Index questionnaire, and visual analog scale before the surgery and the same plus the Forgotten Joint Score at their last follow-up visit, 2 years postoperatively. Maximum knee flexion and the time reaching maximum knee flexion, named the recovery time, were also recorded. Hip-knee-ankle angle, medial proximal tibial angle, and lateral distal femoral angle were measured before and after the surgery using 3-joint-view radiographs., Results: At 2 years, there were significant differences between the KA and MA techniques in terms of duration of surgery, recovery time, and final Western Ontario and McMaster Universities Osteoarthritis Index, Forgotten Joint Score, and maximum flexion range in favor of KA (P < .05), but no significant difference in visual analog scale score or Oxford Knee Score. In patients who have a preferred knee, the KA knee was preferred over the MA knee by most patients. No prosthetic failure or revision was reported in either group., Conclusions: The KA technique yields acceptable functional outcomes compared to the MA technique. The KA technique was associated with a shorter surgery time, a faster recovery time, and higher patient satisfaction in 2-year follow-ups. Larger multicenter studies with longer follow-ups are warranted to confirm these findings., Level of Evidence: I., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Periprosthetic joint infection (PJI) poses a significant challenge in total knee arthroplasty (TKA), with recurrence rates as high as 14-28%, leading to substantial morbidity and treatment costs. When conventional treatments fail, knee fusion and above-the-knee amputation (AKA) emerge as alternative options. Existing literature offers conflicting views on the efficacy and impact of knee fusion versus AKA with varied outcomes and limitations., Methods: This retrospective national study spanning 2010-2022 investigates Knee Fusion and AKA as options for addressing Knee PJI. Utilizing PearlDiver Patient Records Database, procedural, and reimbursement data on over 100 million individuals from all the US was evaluated. Readmission rates, costs, and complications of the mentioned procedures were assessed using ICD-9 and ICD-10 codes within a 90-day period and one-year post-operation. Statistical analyses, including chi-square tests and regression models, were conducted using integrated R software., Results: The study reveals a substantial escalation (p < 0.0001) in the proportion of patients opting for AKA compared to arthrodesis. While age as a demographic factor showed no significant difference, arthrodesis patients exhibited lower comorbidity scores (3.6 ± 2.9 vs. 4.6 ± 3.4, p < 0.001). Arthrodesis correlated with higher 90-day thromboembolism rates (9.2% vs. 7.3%, p < 0.001), blood transfusion requirements (23.2% vs. 14.4%, p < 0.001), and acute renal failure incidence (p = 0.008) but demonstrated lower rates of urinary tract infections (p = 0.047) and cerebrovascular accidents (p < 0.001). At 1 year, arthrodesis was associated with higher infection rates (38.7% vs. 36.4%, p < 0.001). Arthrodesis patients had significant increased 90-day and 1-year readmission rates and hospitalization costs ($12,732 vs. $18,826, p < 0.001)., Conclusions: We found higher rates of 1-year thromboembolism, infection, acute renal failure, and readmission in the arthrodesis group. AKA patients had more sepsis and cerebrovascular accidents. A patient-centered conversation is best for persistent infections and failed revision TKA. Considering the patient's quality of life, goals, and health status, this discussion should cover each procedure's risks and complications., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Chronic Achilles tendinopathy following Haglund disease is a common ankle condition that is difficult to manage. In this study, we will compare the clinical outcomes of debridement and ostectomy with and without FHL tendon transfer in treating Haglund deformity., Methods: Forty eligible patients aged >50 years who did not respond to conservative treatment were randomly divided into 2 groups for surgical approach: using flexor hallucis longus (FHL) tendon transfer (FHL group) or "standard procedure" (control group). The main surgical treatment included debridement and ostectomy. AOFAS and VISA-A scores were obtained from all patients pre- and postoperatively., Results: Twenty patients were assigned to each of the 2 groups and were observed for at least 1 year. The American Orthopaedic Foot & Ankle Society (AOFAS) ankle hindfoot scores and the Victorian Institute of Sports Assessment self-administered Achilles (VISA-A) questionnaires scores improved more in the FHL group; however, the average difference in the change in scores did not reach the levels previously reported for minimal clinically important differences. Surgical complications and hallux function were not significantly different between the 2 groups., Conclusion: We found that FHL tendon transfer may improve the clinical outcome scores of Achilles tendon treatment in patients aged >50 years using debridement and ostectomy. However, tendon transfer increases the time of surgery and creates additional skin incisions, which may cause more short-term wound complications, and the significance of the differences in outcome improvement may not be clinically meaningful., Level of Evidence: Level II, grade A recommendation., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Disclosure forms for all authors are available online., (© The Author(s) 2024.)

Background: To investigate the genetics of early-onset progressive cerebellar ataxia in Iran, we conducted a study at the Children's Medical Center (CMC), the primary referral center for pediatric disorders in the country, over a three-year period from 2019 to 2022. In this report, we provide the initial findings from the national registry., Methods: We selected all early-onset patients with an autosomal recessive mode of inheritance to assess their phenotype, paraclinical tests, and genotypes. The clinical data encompassed clinical features, the Scale for the Assessment and Rating of Ataxia (SARA) scores, Magnetic Resonance Imaging (MRI) results, Electrodiagnostic exams (EDX), and biomarker features. Our genetic investigations included single-gene testing, Whole Exome Sequencing (WES), and Whole Genome Sequencing (WGS)., Results: Our study enrolled 162 patients from various geographic regions of our country. Among our subpopulations, we identified known and novel pathogenic variants in 42 genes in 97 families. The overall genetic diagnostic rate was 59.9%. Notably, we observed PLA2G6, ATM, SACS, and SCA variants in 19, 14, 12, and 10 families, respectively. Remarkably, more than 59% of the cases were attributed to pathogenic variants in these genes., Conclusions: Iran, being at the crossroad of the Middle East, exhibits a highly diverse genetic etiology for autosomal recessive hereditary ataxia. In light of this heterogeneity, the development of preventive strategies and targeted molecular therapeutics becomes crucial. A national guideline for the diagnosis and management of patients with these conditions could significantly aid in advancing healthcare approaches and improving patient outcomes., (© 2024. The Author(s).)

Purpose: The reliable data on the incidence of hypermobile lateral meniscus (HLM) and its clinical manifestations, diagnostic methods and therapeutic approaches are limited. This systematic study aimed to review available treatment options for HLM and the outcomes of each approach., Methods: A systematic search was performed in four electronic databases (PubMed, EMBASE, Scopus, Web of Science) to identify studies in which arthroscopically confirmed cases of HLM were treated surgically or nonsurgically, and the required data comprising study characteristics, patient data, treatment approaches and outcome measures were extracted from eligible studies., Results: Twenty studies with a total of 212 patients (138 males and 74 females) and 219 knees were included. The most frequently reported symptoms were locking sensations, knee pain, giving way and snapping. Treatments used by the studies were: radiofrequency energy in two studies; arthroscopic partial meniscectomy in one study; open surgery in two studies; and arthroscopic meniscal repair in 17 studies. Eleven studies used an all-inside repair method and an inside-out meniscal repair was reported in eight studies. Three studies reported the usage of posterior arthroscopy for therapeutic or diagnostic approaches. Evaluation of symptom resolution was the main outcome measurement for which almost all of the studies stated relief of symptoms after intervention., Conclusion: Despite the lack of definite consensus about the most appropriate intervention for HLM, therapeutic preference was directed towards arthroscopic all-inside and inside-out repair techniques. Although the surgeon's decision remains the key factor in choosing the most suitable treatment option for each individual, posterior arthroscopic meniscal repair may be considered as a better option for HLM treatment according to the findings of this review., Level of Evidence: Level IV., (© 2024 European Society of Sports Traumatology, Knee Surgery and Arthroscopy.)

Background: Variants in the Aristaless-related homeobox (ARX) gene lead to a variety of phenotypes, with intellectual disability being a steady feature. Other features can include severe epilepsy, spasticity, movement disorders, hydranencephaly, and ambiguous genitalia in males. X-linked Ohtahara syndrome or Type 1 early infantile epileptic encephalopathy (EIEE1) is a severe early-onset epileptic encephalopathy with arrested psychomotor development caused by hemizygous mutations in the ARX gene, which encodes a transcription factor in fundamental brain developmental processes., Methods: We presented a case report of a 2-year-old boy who exhibited symptoms such as microcephaly, seizures, and severe multifocal epileptic abnormalities, and genetic techniques such as autozygosity mapping, Sanger sequencing, and whole-exome sequencing., Results: We confirmed that the patient had the NM&#95;139058.3:c.84C>A; p.(Cys28Ter) mutation in the ARX gene., Conclusion: The patient with EIEE1 had physical symptoms and hypsarrhythmia on electroencephalogram. Genetic testing identified a causative mutation in the ARX gene, emphasizing the role of genetic testing in EIEE diagnosis., (© 2024 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals LLC.)

Purpose: Total hip arthroplasty (THA) is commonly used worldwide in pelvic osteoarthritis treatment. This surgery can change the spinopelvic parameters, which in turn affects the performance of the patients after surgery. However, the relationship between functional disability following THA and spinopelvic alignment is not completely understood. The limited available studies have also been conducted on the population with spinopelvic malalignments. This study aimed to examine the changes in spinopelvic parameters after primary THA in patients with normal preoperative spinopelvic characteristics and the association of these parameters with the performance, gender, and age of the patients after THA., Methods: Fifty-eight eligible patients with unilateral primary hip osteoarthritis (HOA) scheduled for total hip arthroplasty between February and September 2021 were studied. Spinopelvic parameters including pelvic incidence (PI), sacral slope (SS), and pelvic tilt (PT) were measured before surgery and three months after surgery, and the relationship between these parameters and patients' performance (Harris hip score) was assessed. Also, the relationship between the age and gender of the patients with these parameters was evaluated., Results: The mean age of study participants was 46.03 ± 14.25. Three months after THA, sacral slope decreased with the mean difference of 4.31 ± 10.26 degrees (p = 0.002) and Harris hip score (HHS) increased by 19.41 ± 26.55 points (p < 0.001). With increasing age in patients, the mean SS and PT decreased. Among the spinopelvic parameters, SS (β = 0.11) had a greater effect than PT on postoperative HHS changes and among the demographic parameters, age (β = -0.18) had a greater effect on HHS changes than gender., Conclusion: Spinopelvic parameters are associated with age, gender, and patient's function after THA as sacral slope decreased and HHS increased after THA, and aging is accompanied by lowering of PT and SS., (© 2023. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Background: There is still debate over whether to sacrifice the posterior cruciate ligament (PCL) during total knee arthroplasty (TKA). Several studies reported the costs and benefits of each approach regarding technical difficulties in PCL balancing and postoperation complications. In this study, we aimed to evaluate PCL needling as a safe method for balancing the cruciate-retained TKA (CR-TKA)., Methods: In this comparative study, 120 patients underwent CR-TKA and were divided into 2 groups. Fifty-four patients with an acceptable PCL tightness were included in group A, and 66 cases with a tight PCL were included in group B. In group B, needle pie-crusting of the PCL was performed instead of releasing the PCL from its insertions as the standard procedure. The participants' functional outcomes, pain severity, knee range of motion, and PCL laxity were evaluated during multiple follow-ups in 3 years postoperatively., Results: The participants' age, weight, and body mass index did not differ between the 2 groups. The mean age of the patients was 69 ± 5.9 years. The functional outcomes and range of motion of the patients in the 2 groups significantly improved after the operation compared to the preoperative status, but the postoperative score was not significantly different between groups ( P > .05). Clinical examinations were normal in all patients in both groups in all follow-up stages., Conclusions: PCL balancing is a time-consuming yet essential step for the outcome of CR-TKA and patient satisfaction. PCL needling technique shows promising results and a few complications for PCL balancing in CR-TKA., (© 2023 The Authors.)

Background: Orthopedic surgeons favor an intramedullary guiding system on the femoral component during total knee arthroplasty (TKA); nevertheless, improper positioning of the entry point affects the final alignment. We have designed a new femoral cutting system for TKA that uses the distal and posterior femoral condyles as reference points for the setting of the cutting system regardless of the femoral canal. This study aims to evaluate the outcomes of this new guiding system., Methods: We enrolled a series of 75 consecutive knees undergoing TKA. The alpha, gamma, and hip-knee-ankle (HKA) angles were assessed three months postoperatively. Also, surgical time and intraoperative blood loss were recorded for all patients., Results: Fifteen patients underwent TKA using the mechanical alignment (MA) strategy, and 60 underwent kinematically aligned (KA) TKA. Both groups showed normal coronal and sagittal alignment 3 months postoperatively. The mean intraoperative blood loss was 213.11 ± 52.73 ml, which was not different between the two groups (n.s.). The mean surgical time was 43.12 ± 11.62 min, which was significantly shorter in the KA-TKA (41.11 ± 3.77 min) than in the MA-TKA (49.34 ± 4.56 min) ( P  < 0.001)., Conclusion: Using the new guiding system with good femoral alignment, we introduced the easily palpable and available condylar surface as a new landmark for cutting the distal femur in TKA., Level of Evidence: IV., Competing Interests: Conflict of InterestAll authors declared no conflict of interest., (© Indian Orthopaedics Association 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Leukodystrophies (LDs) are a heterogeneous group of progressive neurological disorders and characterized by primary involvement of white matter of the central nervous system (CNS). This is the first report of the Iranian LD Registry database to describe the clinical, radiological, and genomic data of Persian patients with leukodystrophies. From 2016 to 2019, patients suspicious of LDs were examined followed by a brain magnetic resonance imaging (MRI). A single gene testing or whole-exome sequencing (WES) was used depending on the neuroradiologic phenotypes. In a few cases, the diagnosis was made by metabolic studies. Based on the MRI pattern, diagnosed patients were divided into cohorts A (hypomyelinating LDs) versus cohort B (Other LDs). The most recent LD classification was utilized for classification of diagnosed patients. For novel variants, in silico analyses were performed to verify their pathogenicity. Out of 680 registered patients, 342 completed the diagnostic evaluations. In total, 245 patients met a diagnosis which in turn 24.5% were categorized in cohort A and the remaining in cohort B. Genetic tests revealed causal variants in 228 patients consisting of 213 variants in 110 genes with 78 novel variants. WES and single gene testing identified a causal variant in 65.5% and 34.5% cases, respectively. The total diagnostic rate of WES was 60.7%. Lysosomal disorders (27.3%; GM2-gangliosidosis-9.8%, MLD-6.1%, KD-4.5%), amino and organic acid disorders (17.15%; Canavan disease-4.5%, L-2-HGA-3.6%), mitochondrial leukodystrophies (12.6%), ion and water homeostasis disorders (7.3%; MLC-4.5%), peroxisomal disorders (6.5%; X-ALD-3.6%), and myelin protein disorders (3.6%; PMLD-3.6%) were the most commonly diagnosed disorders. Thirty-seven percent of cases had a pathogenic variant in nine genes (ARSA, HEXA, ASPA, MLC1, GALC, GJC2, ABCD1, L2HGDH, GCDH). This study highlights the most common types as well as the genetic heterogeneity of LDs in Iranian children., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Various uses of posterior knee arthroscopy have been shown, including all-inside repair of posterior meniscal lesions, posterior cruciate ligament (PCL) reconstruction or PCL avulsion fixation, extensile posterior knee synovectomy for pigmented villonodular synovitis or synovial chondromatosis, posterior capsular release in the setting of knee flexion contractures, and loose bodies removal. Posterior arthroscopy provides direct access to the posterior meniscal borders for adequate abrasion and fibrous tissue removal. This direct view of the knee posterior structures enables the surgeon to create a stronger biomechanical repair using vertical mattress sutures. During PCL reconstruction, posterior arthroscopy gives the surgeon proper double access to the tibial insertion site, which can result in less acute curve angles and the creation of a more anatomic tibial tunnel. Moreover, it gives the best opportunity to preserve the PCL remnant. Arthroscopic PCL avulsion fixation is more time-consuming with a larger cost burden compared to open approaches, but in the case of other concomitant intra-articular injuries, it may lead to a better chance of a return to pre-injury activities. The high learning curve and overcaution of neuromuscular injury have discouraged surgeons from practicing posterior knee arthroscopy using posterior portals. Evidence for using posterior portals by experienced surgeons suggests fewer complications. The evidence suggests toward learning posterior knee arthroscopy, and this technique must be part of the education about arthroscopy. In today's professional sports world, where the quick and complete return of athletes to their professional activities is irreplaceable, the use of posterior knee arthroscopy is necessary.

Background: In irreducible bucket-handle medial meniscal tears (BHMMTs), the displaced central fragment is rotated 180° or more, meaning reduction is impossible without derotation of the tear. In chronic cases, the rotated meniscus is deformed and degenerated; thus, the issue of reproducibility and repairability arises. This study presents the clinical outcomes of chronic irreducible and unstable locked BHMMTs. Radiological outcomes were also evaluated using magnetic resonance imaging (MRI)., Methods: This is a retrospective study of 37 patients with 11 cases of irreducible BHMMT and 26 cases of reducible but unstable chronic BHMMT who underwent operations between 2011 and 2016. Posterior arthroscopy was performed after temporary meniscus fixation using a provisional needle fixation technique. After the posterior repair with vertical mattress sutures was completed using an all-inside technique, the classic outside-in technique was performed for the anterior third of the meniscus. The Lysholm, International Knee Documentation Committee (IKDC), and Tegner activity scores were obtained from all patients before surgery and at the latest follow-up. Radiological evaluations were performed using MRI before surgery and at 6 months postoperatively., Results: Between 2011 and 2016, a total of 37 consecutive patients with irreducible and chronic BHMMTs underwent surgery. The average postoperative follow-up was 7.2 ± 1.4 years (mean ± SD). Postoperative Lysholm (89.57 ± 2.7) and IKDC (87.22 ± 3.2) scores improved significantly at the last follow-up when compared with the pre-operative scores (38.44 ± 4.5 and 23.52 ± 7.8, respectively). According to the Tegner activity scale, patients' postoperative activity levels remained unchanged compared to preoperative levels at the last follow-up., Conclusion: Posterior knee arthroscopy with the all-inside posterior suture and inside-out anteromedial suture technique presented in this study yielded excellent clinical outcomes when used to repair chronic irreducible or unstable BHMMTs., Level of Evidence: Level IV., (© 2023. The Author(s).)

Introduction: Fixation of plateau posterolateral fracture (PLF) is challenging because the fracture site is mostly covered by vital neurovascular structures. We operated on 15 cases of PLF using a direct lateral approach. This study aims to report on clinical results., Material and Methods: Between 2017 and 2019, 15 cases of PLFs were fixed with a direct lateral approach and a tricortical autologous bone graft from the iliac crest. A depression of more than 2 mm was indicated for the surgical treatment. Clinical evaluation included Lysholm score, International Knee Documentation Committee Score (IKDC) score, and Tegner activity scale after two years follow-up., Results: The last follow-up was at 24 months after the operation. The mean postoperative Tegner activity scale did not change significantly compared to before the injury (6.5 (6-7) vs. 7 (6-8, p  = 0.5)). The postoperative IKDC and Lysholm scores improved significantly compared to before the operation ( p  < 0.001). The full range of motion was seen in all patients except one who was manipulated after three months., Conclusions: Surgical treatment using a direct lateral approach is a safe procedure for PLFs that results in good, short-term clinical and radiologic outcomes without fibular osteotomy or compromising the important neurovascular structures., Level of Evidence: IV., Competing Interests: Conflict of InterestThe authors have no conflicts of interest to declare that are relevant to the content of this article., (© Indian Orthopaedics Association 2023, Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Purpose: Studies have suggested some predictors for hamstring tendon (HT) autograft diameter based on anthropometric factors and preoperative magnetic resonance imaging (MRI) with variable results. Some authors have attributed the variability to gender differences. This prospective cohort reports the sensitivity and specificity of anthropometric and MRI predictors in males and females separately to determine the difference., Methods: Forty-two eligible patients who underwent anterior cruciate ligament reconstruction (ACLR) and MRI in our center were included. ACLR was performed by the senior surgeon using a 4-stranded HT autograft for all patients. A blinded musculoskeletal radiologist measured the cross-sectional area (CSA) of gracilis and semitendinosus tendons using the free-hand region of interest tool for all patients. An orthopaedic resident (PGY4) collected anthropometric factors and measured intraoperative autograft diameter., Results: Mean intraoperative autograft diameter was 8.0 mm. Females had a significantly lower autograft diameter (7.4 vs. 8.2, P < 0.001), smaller gracilis (6.9 vs. 7.9, P = 0.003) and semitendinosus CSA (11.5 vs. 12.8, P = 0.014) compared to males. ROC curve analysis resulted different cut-off values with high sensitivity and specificity for semitendinosus and combined CSA regarding gender., Conclusion: Based on the results of this study, CSA of either isolated or combined HTs on preoperative axial MRI, height, and weight are the strongest predictors of intraoperative autograft diameter. It is suggested to consider different cut-offs for males and females to have a better clinical guide for surgeons., Level of Evidence: Level II., (© 2023. The Author(s).)

Background: Insufficient amounts of survival motor neuron protein is leading to one of the most disabling neuromuscular diseases, spinal muscular atrophy (SMA). Before the current study, the detailed characteristics of Iranian patients with SMA had not been determined., Objective: To describe the key demographic, clinical, and genetic characteristics of patients with SMA registered in the Iranian Registry of SMA (IRSMA)., Methods: IRSMA has been established since 2018, and the demographic, clinical, and genetic characteristics of patients with SMA were recorded according to the methods of treat neuromuscular disease (TREAT-NMD) project., Results: By October 1, 2022, 781 patients with 5q SMA were registered. Of them, 164 patients died, the majority of them had SMA type 1 and died during the first 20 months of life. The median survival of patients with type 1 SMA was 23 months. The consanguinity rate in 617 alive patients was 52.4%, while merely 24.8% of them had a positive family history. The most common type of SMA in live patients was type 3. Morbidities were defined as having scoliosis (44.1%), wheelchair dependency (36.8%), tube feeding (8.1%), and requiring mechanical ventilation (9.9%). Most of the registered patients had a homozygous deletion of SMN1, while the frequency of patients with higher copy numbers of SMN2, was less in more severe types of the disease. Earlier onset of the disease was significantly seen in patients with lower copy numbers of SMN2. The neuronal apoptosis inhibitory protein (NAIP) gene deletion was associated with a higher incidence of more severe types of SMA, higher dependency on ventilators, tube feeding, and earlier onset of the disease., Conclusions: The IRSMA is the first established Iranian nationwide registry of patients with SMA. Using this registry, decision-makers, researchers, and practitioners can precisely understand the epidemiology, characteristics, and genetics of patients with SMA in Iran.

Objectives: The increasing number of total hip arthroplasties (THA) has led to increased patient demands and expectations, making it crucial to assess patients' ability to "forget" their implants in daily life. This study aimed to determine the reliability and validity of a Persian version of the Forgotten Joint Score (P-FJS) in THA patients., Methods: The questionnaire was translated bidirectionally with the permission of the questionnaire designer. Data were collected from 2018 to 2020 and included 142 patients who had undergone THA by the same surgeon at least one year ago. Participants completed the FJS questionnaire twice within a one-week interval, and the validity, reliability, and feasibility of the questionnaires were assessed using statistical tests on the HHS and OHS forms completed by all participants., Results: In 142 patients (52.1% male) with a mean age of 65 ± 0.5 years who answered the questionnaires, P-FJS correlated strongly with OHS and HHS. The internal consistency (α = 0.91) and reproducibility of the questionnaire were excellent. None of the floor and ceiling effects were detected., Conclusion: The P-FJS questionnaire in the THA is considered a legitimate, repeatable, and self-administered survey that can be compared to its English-language counterpart. In addition, it is noteworthy that this version does not show any floor or ceiling effects., Competing Interests: None

Purpose: Protein arginine methyltransferase 7 (PRMT7) is a member of a family of enzymes that catalyzes the methylation of arginine residues on several protein substrates. Biallelic pathogenic PRMT7 variants have previously been associated with a syndromic neurodevelopmental disorder characterized by short stature, brachydactyly, intellectual developmental disability, and seizures. To our knowledge, no comprehensive study describes the detailed clinical characteristics of this syndrome. Thus, we aim to delineate the phenotypic spectrum of PRMT7-related disorder., Methods: We assembled a cohort of 51 affected individuals from 39 different families, gathering clinical information from 36 newly described affected individuals and reviewing data of 15 individuals from the literature., Results: The main clinical characteristics of the PRMT7-related syndrome are short stature, mild to severe developmental delay/intellectual disability, hypotonia, brachydactyly, and distinct facial morphology, including bifrontal narrowing, prominent supraorbital ridges, sparse eyebrows, short nose with full/broad nasal tip, thin upper lip, full and everted lower lip, and a prominent or squared-off jaw. Additional variable findings include seizures, obesity, nonspecific magnetic resonance imaging abnormalities, eye abnormalities (i.e., strabismus or nystagmus), and hearing loss., Conclusion: This study further delineates and expands the molecular, phenotypic spectrum and natural history of PRMT7-related syndrome characterized by a neurodevelopmental disorder with skeletal, growth, and endocrine abnormalities., Competing Interests: Conflict of Interest Megan Li is an employee of Invitae. Erin Torti, Amber Begtrup, and Rhonda E Schnur are employees of GeneDx, Inc. All other authors declare no conflicts of interest., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Studies have proved that in addition to the inlet and outlet views, the intraoperative lateral sacral view is required to ensure the correct entry point, reduce operating time, and lower radiation exposure. Considering the complex anatomy of the sacrum, we showed a safe corridor for sacroiliac joint (SIJ) screw insertion that was accessible using only inlet and outlet fluoroscopic views., Methods: From 2013 to 2020, we enrolled 215 patients who underwent percutaneous SIJ screw insertion. Our experience in SIJ screw insertion is presented using only two views (inlet and outlet). We reported on the radiation exposure time, operating time, rate of screw malposition, neurologic injury, and revision surgery., Results: The screw malposition rate was 5.5%, including 11 foraminal perforations and one perforated anterior sacral cortex. Paresthesia after the surgery was observed in six patients (2.8%). No revision surgery or screw removal was performed. The radiation exposure and operation time for each screw were 21 ± 4.5 s and 13.5 min, respectively., Conclusion: The most anterior and the lowest part of the S1 vertebra can be easily found using intraoperative inlet and outlet views. It is a safe corridor for SIJ screw insertion with low radiation time, neurologic injury, and revision rates.

This study investigates the effect of structural modification actions on the existing stormwater collecting system in Eastern Tehran to increase the hydraulic capacity and create suitable conditions for the passage of runoff in the critical points of the canal. First, the hydraulic conditions within the stormwater collecting system were simulated using the Stormwater Management Model (SWMM) model before/after the modification to investigate the rehabilitation results. Three critical locations along the main canal were recognized as the most vulnerable points. Then, based on field visits and brainstorming sessions, rehabilitation methods were presented, and three practical solutions, including canal deepening, canal widening, and their combination, were investigated for each. Then, local investigating based on the rehabilitation alternatives for each critical location was conducted using the HEC-RAS. Finally, the SWMM model was used again to evaluate the overall operational performance of the stormwater collecting system after the rehabilitation. The results revealed that it is necessary to implement two alternatives of deepening and widening to provide adequate transmission runoff capacity during rainfalls with various return periods. More specifically, the localized redesign of the eastern flood diversion canal had an acceptable improvement in reducing flooding problems so that for floods with a return period of 10 years, the number of node flooding dropped from 4 to 0, inundated areas from 17% to 0, and the overflow volume from (10-45) to 0. Moreover, the proposed local rehabilitation reduced the overflow volume from (30-65), (43-74), and (70-92) in the status quo to (4-12), (11-27), and (24-36) for rainfall with 25, 50, and 100-year return periods.

The study proposed a practical approach for low impact development (LID) placement in dense residential urban catchments considering social, economic, and technical criteria. The objective is to shift the current technical orientation in LIDs' locating to a practical one with a specific level of residents' cooperation. Four stages were conducted, including (i) acquiring the residents' socio-economic information; (ii) hydraulic simulation of the stormwater collection system in the status quo, (iii) regionalization of the urban catchment using data mining, and (iv) proposing specific LIDs locating scenarios. The field study lasted around four months in a dense residential area with a combination of old/new buildings in the eastern Tehran metropolis. Regionalization was conducted by combining pre-processing/clustering techniques, resulting in six regions (preliminary results) and four regions after the clustering integration in the post-processing study. The regions have different priorities for the LIDs locating, including (i) cooperative cluster, 57 sub-catchments (SCs) and appropriate for rain barrel (RB) and green roof (GR) locating; (ii) semi-cooperative cluster, 12 SCs, appropriate for RB or GR locating; (iii) 15 SCs with infrastructural constraints in the third cluster and appropriate for RB; and (iv) non-cooperative cluster, 34 SCs, inappropriate for LIDs locating. Accordingly, seven locating scenarios were specified, and the developed EPA-SWMM model was modified to evaluate the scenarios for mitigating the study area's node flooding problems. The finding shows that the overflow volume reduced in a range of 36.7-93.1%, 28.3-78.7%, and 16.3-66.4% under the rainfall with 2, 5, and 10 years return periods, respectively.

Ultra-rare biallelic pathogenic variants in geranylgeranyl diphosphate synthase 1 (GGPS1) have recently been associated with muscular dystrophy/hearing loss/ovarian insufficiency syndrome. Here, we describe 11 affected individuals from four unpublished families with ultra-rare missense variants in GGPS1 and provide follow-up details from a previously reported family. Our cohort replicated most of the previously described clinical features of GGPS1 deficiency; however, hearing loss was present in only 46% of the individuals. This report consolidates the disease-causing role of biallelic variants in GGPS1 and demonstrates that hearing loss and ovarian insufficiency might be a variable feature of the GGPS1-associated muscular dystrophy., (© 2022 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Preservation of the meniscus in bucket handle medial meniscal tears (BHMMTs) and posterior meniscocapsular (ramp) lesions is challenging. Current efforts are being made in the scientific community to (1) introduce new ways of gaining easier and better field of view over the repair site and (2) increase the chance of meniscal healing through effective augmentation procedures. The current note introduces a way to achieve good exposure of posterior knee compartments and proposes an augmentation technique that involves exposing the local non-articulating subchondral cancellous bone and making in situ clots., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this article., (Copyright © 2022. Published by Elsevier Inc.)

Purpose: This study reported the outcomes of locked bucket-handle medial meniscal tear (BHMMT) repairs using an arthroscopic posterior approach during anterior cruciate ligament (ACL) reconstruction., Methods: Between 2011 and 2014, 48 patients with BHMMTs and ACL tears who met the eligibility criteria were enrolled in the present study. BHMMTs were assessed using a posterolateral transseptal portal and repaired using a posteromedial portal. Transportal ACL reconstruction was performed using hamstrings autograft. Patients were assessed based on their IKDC and Lysholm scores and Tegner activity level. Meniscal healing was clinically evaluated based on the absence of swelling, joint line tenderness, locking, and catching; McMurray test results; and the need for meniscectomy., Results: According to follow-up assessments, the average IKDC and Lysholm scores improved significantly after 3-5 years (P < 0.001) CONCLUSION:  Excellent clinical outcomes were obtained when locked BHMMTs were repaired using an all-inside suture technique that employed posteromedial and posterolateral transseptal portals., Level of Evidence: IV., (© 2021. European Society of Sports Traumatology, Knee Surgery, Arthroscopy (ESSKA).)

Background: This study aimed to report objective and subjective knee stability scores for patients who underwent acute repair of avulsed posterolateral corner (PLC) structures or acute reconstruction of midsubstance tears combined with delayed reconstruction of cruciate ligaments., Methods: A total of 48 sport and vehicle accident traumatic patients were enrolled in a three-year follow-up study. The patients were investigated by clinical exams, subjective and objective International Knee Documentation Committee (IKDC) score, Tegner score, Lysholm score, and stress imaging. All scores were compared between the reconstruction and repair groups., Results: Subjective IKDC scores were obtained at 83.3±9.6 and 88.3±4.39 for the reconstruction and repair groups, respectively. Only two patients in the reconstruction group had abnormal objective IKDC scores. Based on the Tegner score, 15 out of 18 patients in the repair group and 20 out of 24 patients in the reconstruction group regained their pre-injury functional level. Mean Lysholm scores for the reconstruction and repair groups were estimated at 83.4±8.2 and 88.2±4.1, respectively. Mean lateral joint opening differences between two knees in the reconstruction and repair groups were -0.2±0.1mm and 0.5±0.1mm, respectively. There were no statistically significant differences between groups outcomes. We had no failure of treatment at the final follow-up., Conclusion: Acute intervention within 3 weeks after PLC injuries combined with delayed cruciate ligaments reconstructions showed favorable outcomes. Both repair and reconstruction are effective when deciding based on the type of injury (i.e., avulsion and midsubstance tear)., Competing Interests: The authors declare that there is no conflict of interest.

Purpose: This study investigates the effects of the all-inside repair of posterosuperior popliteomeniscal fascicle (PMF) on lateral meniscus stabilization using a posterior arthroscopic approach., Methods: Between 2015 and 2018, 17 patients with hypermobile lateral meniscus (HLM) underwent posterior knee arthroscopy for PMF repair. The all-inside repair was performed through posteromedial transseptal and posterolateral portals using a suture hook technique. Patients were clinically assessed based on IKDC and Lysholm scores., Results: Both IKDC and Lysholm scores improved significantly after an average follow-up of 3.5 years (P < 0.001). No patients underwent reoperation, and no complications associated with posterior knee arthroscopy were reported., Conclusion: The all-inside suture hook technique using posterolateral and posteromedial transseptal portals fixes HLM with excellent IKDC and Lysholm scores., Level of Evidence: Level IV., (© 2021. The Author(s).)

Low impact development (LID) methods have been shown to be efficient in reducing the peak flow and total volume of urban stormwater, which is a top priority for effective urban stormwater management in many municipalities. However, decision-makers need information on the effects of LIDs and their associated costs before allocating limited resources. In this study, the Storm Water Management Model (SWMM) was used to investigate the effects of five different LID scenarios on urban flooding in a district in Tehran, Iran. The LID scenarios included rain barrel (RB) at two sizes, bio-retention cell (BRC), and combinations of the two structures. The results showed that significant node flooding and overflow volume would occur in the study area under the existing conditions, especially for rainfall events with longer return periods. BRC and combinations of BRC and RBs were the most effective options in reducing flooding, while the smaller-size RB was the cheapest alternative. However, normalized cost, obtained through dividing the total cost by the percent reduction in node flooding and/or overflow volume, was smallest for BRC. The results of this study demonstrate how hydraulic modeling can be combined with economic analysis to identify the most efficient and affordable LID practices for urban areas.

Childhood leukodystrophies are a fast-growing field of pediatric neurology practice. Epidemiologic studies on the incidence of these disorders in children show different results. This is the first report of childhood leukodystrophies incidence from Iran. The enrolled patients were recruited from the neurometabolic bioregistry system that was organized in 2010 in the Children's Medical Center, Tehran, Iran. Herein is reported the incidence rate of leukodystrophies in those patients who were residents of 2 big popular provinces near Iran's capital city Tehran, with an average child population of 2 988 800 children. Ninety cases of leukodystrophies from Tehran and Alborz provinces who were registered between 2010 and 2016 in the bioregistry system were enrolled in this study. The annual incidence of inherited white matter disorders was 3.01/100 000, the highest number compared with those found in other studies using similar methods throughout the world. One of the main cause of this higher incidence could be the higher number of consanguineous marriages in Iran.

Background: The proportion of newborns recalled during neonatal screening programs for congenital hypothyroidism (CH) varies substantially by country and may be higher in settings where povodine iodine (PVP-I) is used during delivery. We assessed this hypothesis by substituting PVP-I for chlorhexidine (CHL) and evaluated the reduction in the recall rate of the Irainian newborn screening program., Methods: This study investigated 2282 neonates of mothers admitted to a local hospital for delivery between December 2012 and October 2013. We measured thyorid stimulating hormone (TSH) levels in heel-prick blood specimens of infants, aged between 3 and 5 days, born to mothers who received PVP-I (phase I) and those who received CHL after withdrawal of PVP-I from obstetric procedures (phase II). Then we compared the median TSH levels and the recall rate based on a TSH level ≥5 mU/L., Results: Of 2282 cases, 1094 infants were born to mothers exposed to PVP-I during phase I (PVP-I group) and 1188 ones were born to mothers exposed to chlorhexidine in phase II (CHL group); 6.56% of the PVP-I group and 1.91% of the CHL group were recalled later during screening (p<0.001). The median TSH level was significantly higher in the PVP-I group compared to the CHL group (1.35 vs. 1.00, p<0.001)., Conclusions: Replacement of iodine-containing antiseptics by iodine-free ones, during delivery resulted in a significant reduction in the recall rate of the Iranian screening program for CH.