Actinin BioID reveals sarcomere crosstalk with oxidative metabolism through interactions with IGF2BP2.

Actinins are strain-sensing actin cross-linkers that are ubiquitously expressed and harbor mutations in human diseases. We utilize CRISPR, pluripotent stem cells, and BioID to study actinin interactomes in human cardiomyocytes. We identify 324 actinin proximity partners, including those that are dependent on sarcomere assembly. We confirm 19 known interactors and identify a network of RNA-binding proteins, including those with RNA localization functions. In vivo and biochemical interaction studies support that IGF2BP2 localizes electron transport chain transcripts to actinin neighborhoods through interactions between its K homology (KH) domain and actinin\u27s rod domain. We combine alanine scanning mutagenesis and metabolic assays to disrupt and functionally interrogate actinin-IGF2BP2 interactions, which reveal an essential role in metabolic responses to pathological sarcomere activation using a hypertrophic cardiomyopathy model. This study expands our functional knowledge of actinin, uncovers sarcomere interaction partners, and reveals sarcomere crosstalk with IGF2BP2 for metabolic adaptation relevant to human disease

Massive Idiopathic Nonsurgical Pneumoperitoneum with the Football Sign in a Preterm Infant: A Case Report

Pneumoperitoneum is the abnormal presence of air in the peritoneal cavity. In cases of massive pneumoperitoneum, abdominal radiographs may reveal “the football sign,” which results from air outlining the abdominal cavity and surrounding the falciform ligament. Although most cases of pneumoperitoneum result from visceral perforation, there is increased awareness of other causes, resulting in an entity referred to as nonsurgical pneumoperitoneum (NSP). We report a case of nonsurgical pneumoperitoneum with a football sign on supine radiographs in a preterm 1-month-old infant who had a complex medical history. The patient developed sepsis and underwent an exploratory laparotomy, which ruled out perforation as well as any intra-abdominal cause for the pneumoperitoneum and infection

Concurrent Malignant Infantile Osteopetrosis and Hypophosphatasia in a Six-year-old Boy: A Case Report

Malignant infantile osteopetrosis is a rare inherited disease of bone metabolism, in which osteoclast dysfunction and diminished bone turnover lead to diffuse sclerosis with obliteration of the medullary cavities and narrowing of the skull base neural foramina. We report a case of malignant infantile osteopetrosis with bone marrow failure and optic atrophy that co-occurred with hypophosphatasia, another rare inherited bone disease, in a 6-year-old boy. Key imaging signs of these rare diseases are discussed

Primary Synovial Chondromatosis of the Knee in a 12-Year-Old Boy: A Case Report

Primary synovial chondromatosis is an uncommon, benign synovial proliferative disorder rarely seen in children. The most commonly affected joint is the knee, followed by the hip. The symptoms of this condition are progressive and insidious and may include pain, joint stiffness, and swelling. Radiography is an appropriate first imaging modality that typically shows multiple calcified loose bodies within the affected joint space. Magnetic resonance imaging should then be performed to evaluate for the presence of noncalcified cartilaginous loose bodies and soft tissue involvement prior to surgical treatment that is necessary to prevent permanent joint damage. The recurrence of primary synovial chondromatosis is common, especially in cases of incomplete surgical excision. We report a case of primary synovial chondromatosis in a child. The diagnostic and the treatment considerations of the case are briefly discussed

A Rare Aneurysmal Bone Cyst of the Rib in an Infant: A Case Report

An aneurysmal bone cyst (ABC) is a rare benign expansile lesion of the bone that consists of hemorrhagic cystic spaces separated by fibrous connective tissue containing osteoid and multinucleated giant cells. The lesion may be primary or secondary. We present a case of ABC in a 7-month-old girl who had a growing, antibiotic-refractory mass in the left chest wall. A computed tomography of the chest confirmed the anatomic location of the mass, but a percutaneous image-guided biopsy and video-assisted thoracoscopic surgery performed during work-up were nondiagnostic. The patient ultimately had a partial rib resection via left thoracotomy. On histologic examination, the mass was found to be an ABC. To our knowledge, this is the second case reported on aneurysmal bone cyst of the rib in an infant. The presented case confirms that ABCs can affect any bone and suggests that the inclusion of an expansile osteolytic lesion of the ribs in differential diagnosis is necessary to facilitate timely treatment that warrants excellent results

Osteochondral Lesions in Pediatric and Adolescent Patients

Osteochondral lesions are acquired, potentially reversible injuries of the subchondral bone with or without associated articular cartilage involvement. Injury results in delamination and potential sequestration of the affected bone. Although an association with mechanical and traumatic factors has been established, the etiology remains poorly understood. These lesions commonly occur in the knee; articular surfaces of the elbow, ankle, hip, and shoulder are also affected. Osteochondral lesions are relatively common in children and adolescents, and the incidence is increasing. Prognosis of these lesions depends on stability, location, and size of the lesion.Imaging has an essential role in the diagnosis, staging, and management of osteochondral lesions. Many of these lesions are first diagnosed by plain film. MRI adds value by identifying unstable lesions that require surgical intervention. This review focuses on the clinical and imaging features of osteochondral lesions of the knee, elbow, and ankle. Imaging criteria for staging and management are also reviewed

A Sustainable Future in Agriculture: An Investigation into Support Systems for Natural and Organic Farmers in Morocco

With the assistance of Ribat Al Fath this project identified the relationships that natural and organic farmers form within the Moroccan agricultural sector and how these relationships influence the farmers abilities to combat agricultural challenges. The team conducted interviews with farmers, and governmental and non-governmental organizations and analyzed the commonly mentioned themes in these interviews. The team used these themes to determine shortcomings in the support system for natural and organic farmers in Morocco and possible solutions to the farmers challenges. Based on the results, the team developed a set of recommendations to improve this support system and created a prototype booklet that compiles useful networking data to distribute to farmers throughout Morocco

Osteochondral lesions in pediatric and adolescent patients.

Osteochondral lesions are acquired, potentially reversible injuries of the subchondral bone with or without associated articular cartilage involvement. Injury results in delamination and potential sequestration of the affected bone. Although an association with mechanical and traumatic factors has been established, the etiology remains poorly understood. These lesions commonly occur in the knee; articular surfaces of the elbow, ankle, hip, and shoulder are also affected. Osteochondral lesions are relatively common in children and adolescents, and the incidence is increasing. Prognosis of these lesions depends on stability, location, and size of the lesion.Imaging has an essential role in the diagnosis, staging, and management of osteochondral lesions. Many of these lesions are first diagnosed by plain film. MRI adds value by identifying unstable lesions that require surgical intervention. This review focuses on the clinical and imaging features of osteochondral lesions of the knee, elbow, and ankle. Imaging criteria for staging and management are also reviewed

Osteochondral lesions in pediatric and adolescent patients.

Osteochondral lesions are acquired, potentially reversible injuries of the subchondral bone with or without associated articular cartilage involvement. Injury results in delamination and potential sequestration of the affected bone. Although an association with mechanical and traumatic factors has been established, the etiology remains poorly understood. These lesions commonly occur in the knee; articular surfaces of the elbow, ankle, hip, and shoulder are also affected. Osteochondral lesions are relatively common in children and adolescents, and the incidence is increasing. Prognosis of these lesions depends on stability, location, and size of the lesion.Imaging has an essential role in the diagnosis, staging, and management of osteochondral lesions. Many of these lesions are first diagnosed by plain film. MRI adds value by identifying unstable lesions that require surgical intervention. This review focuses on the clinical and imaging features of osteochondral lesions of the knee, elbow, and ankle. Imaging criteria for staging and management are also reviewed