Giant Vascular Eccrine Spiradenoma

Giant vascular eccrine spiradenomas (GVESs) are a rare variant of the eccrine spiradenoma that develops from the sweat gland. It is different from the eccrine spiradenoma in its larger size and greater degree of vascularity. Bleeding and/or ulceration are common clinical features of this tumor, and are the reason why it is often clinically confused with a vascular or malignant tumor. Here, a rare case of GVES without bleeding or ulceration is reported

A Pediatric Case of Inflammatory Bowel Disease with Renal Amyloidosis

Amyloidosis is a rare disease that results from the deposition of extracellular protein in various body tissues, causing progressive organ dysfunction. Secondary renal amyloidosis is a rare but serious complication of chronic inflammatory bowel disease, particularly in patients with Crohn’s disease or ulcerative colitis. We report a case of secondary renal amyloidosis in a pediatric patient who reported a 16-year history of “very early onset inflammatory bowel disease”. Intensive treatment including repeated infliximab infusions improved clinical parameters of inflammatory bowel disease, although renal dysfunction showed progression. Amyloidosis should be considered in patients with IBD, particularly if they suffered disease progression

Mineral bone disorder in children with chronic kidney disease: Data from the KNOW-Ped CKD (Korean cohort study for outcome in patients with pediatric chronic kidney disease) study

BackgroundChildren with chronic kidney disease (CKD) are at high risk of mineral bone disorder (MBD), which leads to fractures, growth retardation, and cardiovascular disease. We aimed to comprehensively understand the relationship between renal function and factors related to MBD and evaluate the prevalence and distribution characteristics of MBD, specifically among Korean patients from the KNOW-PedCKD cohort.MethodsFrom the baseline data of the KNOW-PedCKD cohort, we examined the prevalence and distribution of MBD in 431 Korean pediatric CKD patients, including the level of corrected total calcium, serum phosphate, serum alkaline phosphatase, serum intact parathyroid hormone (iPTH), fibroblast growth factor 23 (FGF-23), serum vitamin D, fractional excretion of phosphate (FEP), and bone densitometry Z-scores.ResultsThe median serum calcium level remained relatively normal regardless of the CKD stage. The levels of 1,25-dihydroxy vitamin D, urine calcium-to-creatinine ratio, and bone densitometry Z-score significantly decreased with advancing CKD stage, while those of serum phosphate, FGF-23, and FEP significantly increased with CKD stage. The prevalence of hyperphosphatemia (17.4%, 23.7%, and 41.2% from CKD stages 3b, 4, and 5, respectively) and hyperparathyroidism (37.3%, 57.4%, 55.3%, and 52.9% from CKD stages 3a, 3b, 4, and 5, respectively) significantly increased with the CKD stage. Prescriptions of medications, such as calcium supplements (39.1%, 42.1%, 82.4%), phosphate binders (39.1%, 43.4%, 82.4%), and active vitamin D (21.7%, 44.7%, and 64.7%) significantly increased with CKD stage 3b, 4, and 5, respectively.ConclusionsThe results demonstrated the prevalence and relationship of abnormal mineral metabolism and bone growth according to CKD stage in Korean pediatric CKD patients for the first time

Baseline characteristics of the Korean genetic cohort of inherited cystic kidney disease

Background Identifying genetic mutations in individuals with inherited cystic kidney disease is necessary for precise treatment. We aimed to elucidate the genetic characteristics of cystic kidney disease in the Korean population. Methods We conducted a 3-year prospective, multicenter cohort study at eight hospitals from May 2019 to May 2022. Patients with more than three renal cysts were enrolled and classified into two categories, typical autosomal dominant polycystic kidney disease (ADPKD) and atypical PKD. We identified the clinical characteristics and performed a genetic analysis using a targeted gene panel. Results A total of 725 adult patients were included in the study, of which 560 (77.2%) were diagnosed with typical ADPKD and 165 (22.8%) had atypical PKD. Among the typical ADPKD cases, the Mayo imaging classification was as follows: 1A (55, 9.9%), 1B (149, 26.6%), 1C (198, 35.8%), 1D (90, 16.3%), and 1E (61, 11.0%). The atypical PKD cases were classified as bilateral cystic with bilateral atrophic (31, 37.3%), lopsided (27, 32.5%), unilateral (nine, 10.8%), segmental (eight, 9.6%), bilateral cystic with unilateral atrophic (seven, 8.4%), and asymmetric (one, 1.2%). Pathogenic variants were found in 64.3% of the patients using the ciliopathy-related targeted gene panel. The typical ADPKD group demonstrated a higher discovery rate (62.3%) than the atypical PKD group (41.8%). Conclusion We present a nationwide genetic cohort’s baseline clinical and genetic characteristics for Korean cystic kidney disease

Spontaneous Wrinkle Formation on Hydrogel Surfaces Using Photoinitiator Diffusion from Oil-Water Interface

Patterning wrinkles on three-dimensional curved or enclosed surfaces can be challenging due to difficulties in application of uniform films and stresses on such structures. In this study, we demonstrate a simple one-step wrinkle-formation method on various hydrogel structures utilizing the oil-water interfaces. By diffusion of the photoinitiator from the oil phase to the prepolymer solution in water through the interface, a characteristic cross-linking gradient is set up in the hydrogel. Then, after photopolymerization, we observe diverse patterns of wrinkles upon changing the concentration of the hydrogel or photoinitiator. As the wrinkle formation via photoinitiator diffusion through the interface requires only UV exposure for polymerization, while taking advantage of the oil-water interfacial tension, wrinkles can be developed easily on various curved structures. In addition, we illustrate the formation of wrinkles on surfaces underneath another layer of polymer or on completely enclosed surfaces, which is difficult with conventional methods. We expect that our results will lead to production of novel microstructures and provide a platform for studying the morphogenesis of wrinkles found in nature such as in curved substrates and multilayers

Cell migration in microengineered tumor environments

Recent advances in microengineered cell migration platforms are discussed critically with a focus on how cell migration is influenced by engineered tumor microenvironments, the medical relevance being to understand how tumor microenvironments may promote or suppress the progression of cancer. We first introduce key findings in cancer cell migration under the influence of the physical environment, which is systematically controlled by microengineering technology, followed by multi-cues of physico-chemical factors, which represent the complexity of the tumor environment. Recognizing that cancer cells constantly communicate not only with each other but also with tumor-associated cells such as vascular, fibroblast, and immune cells, and also with non-cellular components, it follows that cell motility in tumor microenvironments, especially metastasis via the invasion of cancer cells into the extracellular matrix and other tissues, is closely related to the malignancy of cancer-related mortality. Medical relevance of forefront research realized in microfabricated devices, such as single cell sorting based on the analysis of cell migration behavior, may assist personalized theragnostics based on the cell migration phenotype. Furthermore, we urge development of theory and numerical understanding of single or collective cell migration in microengineered platforms to gain new insights in cancer metastasis and in therapeutic strategies

A Case of Reactive Eccrine Syringofibroadenoma

Eccrine syringofibroadenoma is a rare adnexal tumor of eccrine ductal differentiation with variable clinical features. It manifests as either a solitary or multiple papules and nodules arranged in a symmetrical or linear pattern. The anatomical distribution is wide and includes the face, trunk, extremities, and rarely the nails. Histopathological findings show anastomosing cords and strands of uniform cuboidal cells surrounded by fibrovascular stroma. Herein, we report a case of reactive eccrine syringofibroadenoma which developed on the foot of a 37-year-old woman, after self-paring of tissue and subsequent infection and ulceration