Generalized Pustular Psoriasis (GPP) is a dermatological autoinflammatory disease that rarely occurs in children and is associated with complex genetic factors. GPP pathogenesis has been associated with mutations in IL36RN gene, which encodes an interleukin-36 receptor antagonist. GPP usually occurs without a history of psoriasis in the patients or their family members. This case report describes the clinical course of a 3-year-old toddler with GPP. The diagnosis of GPP was confirmed through a comprehensive series of examinations, and genetic testing revealed an IL36RN mutation, providing further insight into the genetic basis of the condition. This case highlights the importance of a genetic perspective for diagnosing GPP, particularly in children.
Congenital midline cervical cleft (CMCC) is a rare developmental anomaly of the anterior neck, often misdiagnosed due to its similarity to other cervical malformations. It results from impaired midline fusion of the branchial arches, leading to a linear skin defect with a fibrotic cord and, in some cases, a sinus tract. Left untreated, CMCC can cause progressive contracture, restricted neck mobility, and aesthetic deformities. This review examines the embryological basis, clinical presentation, histopathological characteristics, differential diagnosis, and surgical management of CMCC, with a focus on Z-plasty as the preferred reconstructive technique. Z-plasty effectively lengthens the scar, prevents recurrent contracture, and restores normal neck contour. In addition, we present a case of a 3-day-old female neonate with CMCC, successfully treated with Z-plasty reconstruction, reinforcing the importance of early intervention. Emerging genetic research suggests a potential hereditary component in CMCC, warranting further investigation into its molecular underpinnings. Advances in regenerative medicine and surgical innovation may improve treatment outcomes, offering new possibilities for personalized management of congenital cervical anomalies.
Background: The prognosis of coronavirus disease-2019 (COVID-19) in solid organ transplant recipients is extremely poor. The efficacy and safety of various treatments used in COVID-19 have not been fully explored in kidney transplant recipients (KTRs). This study aims to investigate the safety and efficacy of nirmatrelvir/ritonavir versus remdesivir in adult KTRs infected with COVID-19. Methods: This single-center, retrospective, comparative study, conducted at a tertiary care hospital, enrolled adult KTRs with polymerase chain reaction-confirmed COVID-19 infection receiving nirmatrelvir/ritonavir or remdesivir from April 2021 to October 2023. The primary endpoint was the percentage of participants with COVID-19-related hospitalization within 28 days post-treatment completion. Secondary endpoints included graft rejection, patient and graft survival, worsening COVID-19 stage, and drug–drug interactions. Results: The nirmatrelvir/ritonavir and remdesivir groups consisted of 32 and 115 participants, respectively. The primary endpoint demonstrated no statistically significant differences between the groups. More patients in the remdesivir group progressed to a worse COVID-19 stage (0% vs. 12.7%, p < 0.05). The nirmatrelvir/ritonavir group experienced statistically more significant drug–drug interactions compared to the remdesivir group (90% vs. 0%, p < 0.05), mainly with calcineurin inhibitors. Conclusions: No statistically significant difference was found between nirmatrelvir/ritonavir and remdesivir in reducing COVID-19-related hospitalization among adult kidney transplant recipients.