Immune-mediated necrotizing myopathy: A comprehensive review of the pathogenesis, clinical features, and treatments.

Immune-mediated necrotizing myopathy (IMNM) is a rare and newly recognized autoimmune disease within the spectrum of idiopathic inflammatory myopathies. It is characterized by myositis-specific autoantibodies, elevated serum creatine kinase levels, inflammatory infiltrate, and weakness. IMNM can be classified into three subtypes based on the presence or absence of specific autoantibodies: anti-signal recognition particle myositis, anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myositis, and seronegative IMNM. In recent years, IMNM has gained increasing attention and emerged as a research hotspot. Recent studies have suggested that the pathogenesis of IMNM is linked to aberrant activation of immune system, including immune responses mediated by antibodies, complement, and immune cells, particularly macrophages, as well as abnormal release of inflammatory factors. Non-immune mechanisms such as autophagy and endoplasmic reticulum stress also participate in this process. Additionally, genetic variations associated with IMNM have been identified, providing new insights into the genetic mechanisms of the disease. Progress has also been made in IMNM treatment research, including the use of immunosuppressants and the development of biologics. Despite the challenges in understanding the etiology and treatment of IMNM, the latest research findings offer important guidance and insights for delving deeper into the disease's pathogenic mechanisms and identifying new therapeutic strategies.

Pathogenic role and clinical significance of neutrophils and neutrophil extracellular traps in idiopathic inflammatory myopathies.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of chronic autoimmune diseases characterized by muscle damage and extramuscular symptoms, including specific skin rash, arthritis, interstitial lung disease, and cardiac involvement. While the etiology and pathogenesis of IIM are not yet fully understood, emerging evidence suggests that neutrophils and neutrophil extracellular traps (NETs) have a role in the pathogenesis. Recent research has identified increased levels of circulating and tissue neutrophils as well as NETs in patients with IIM; these contribute to the activation of the type I and type II interferons pathway. During active IIM disease, myositis-specific antibodies are associated with the formation and incomplete degradation of NETs, leading to damage in the lungs, muscles, and blood vessels of patients. This review focuses on the pathogenic role and clinical significance of neutrophils and NETs in IIM, and it includes a discussion of potential targeted treatment strategies.

Application of a free preputial tube graft coupled with urethral plate urethroplasty combined with a Buck's fascia integral covering for the single-stage repair of severe hypospadias.

Objective: To assess the outcome of a free preputial tube graft coupled with urethral plate urethroplasty combined with a Buck's fascia integral covering (BFIC) for the single-stage repair of severe hypospadias in children. Materials and methods: A retrospective study was performed on 40 children with hypospadias who were treated in our hospital from December 2017 to February 2022. The inclusion criteria were as follows: (1) the patient had proximal hypospadias, or penile curvature over 30° after degloving the penis; and (2) the patient underwent surgery for hypospadias for the first time. A free preputial tube graft coupled with urethral plate urethroplasty combined with a Buck's fascia integral covering was performed, and all patients were followed up for more than 6 months. Results: After degloving the foreskin, the urethral orifice was retracted to the perineum and scrotum in 20 cases, penoscrotal in 8 cases, and penile in 12 cases. Single-stage repair was achieved without complications in 34 (85%) patients. The remaining six patients experienced postoperative complications: urethrocutaneous fistula occurred in five cases and glans dehiscence with urethrocutaneous fistula in one case. No urethral diverticulum occurred in any case. A neomeatus with a vertically oriented slit-like appearance was achieved at the tip of the glans in all cases, with one exception. Conclusion: The single-stage operation with a free preputial tube graft coupled with urethral plate urethroplasty combined with a Buck's fascia integral covering in the treatment of severe hypospadias achieves favorable functional and cosmetic outcomes.