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We present an unusual case of bilateral dacryoadenitis in a middle-aged patient with adult-onset Still’s disease (AOSD). We reviewed relevant clinical studies addressing the association between lacrimal lesions and AOSD. A 50-year-old Chinese woman with a 4 year history of recurrent fever and rashes was admitted to the hospital. She had also developed nodules on both eyelids 10 months before admission. After undergoing resection of the left lacrimal gland, the patient received steroids and immunosuppressive therapy. The patient showed good postoperative recovery during the 20 month follow-up. In this case, the pathological examination conducted after orbital surgery helped clinicians differentiate between dacryoadenitis and other orbital lesions. In a review of the literature, dacryoadenitis occurred after the onset of AOSD, and all cases showed non-granulomatous chronic inflammation by histopathology, which indicated that the lacrimal gland may be an inflammatory target and is affected by systemic inflammation in AOSD.

The purpose of this study is to evaluate the effectiveness of intensity-modulated radiation therapy (IMRT) combined with periorbital triamcinolone acetonide injection in treating thyroid eye disease (TED) patients with active extraocular muscle but low CAS. The retrospective observational study was conducted. A total of 156 eligible patients were selected from the TED patient database of the Ophthalmology Department of West China Hospital of Sichuan University. The patient's relevant medical history was recorded, including gender, age of initial onset, onset eye, the interval between onset and the first visit to our hospital, extraocular muscle condition, smoking status, CAS, NOSPECS, thyroid function, symptoms and signs, and the condition of the affected extraocular muscle. We found that IMRT combined with periorbital triamcinolone acetonide injection has a good therapeutic effect on TED patients with low CAS but significant active extraocular muscles, and there is a significant improvement at 3 months after treatment. Thyroid function and CAS can affect the curative effect of IMRT. Patients with hyperthyroidism had worse effect than those with normal thyroid function. The higher the CAS, the better the effect. [ABSTRACT FROM AUTHOR]

In this work, we developed a visible‐light‐driven method for the selective synthesis of amides and N‐acylureas from carboxylic acids and thioureas. This protocol was featured as avoidance of additional oxidants and transition metal catalysts, simple manipulations, low cost, broad substrate scope, and good functional group tolerance. As only oxygen serves as the oxidation reagent, this method provides a promising synthesis candidate for the formation of N‐aryl amides and N‐acylureas, including late‐stage functionalization of complex pharmaceutical molecules and biologically active molecules. [ABSTRACT FROM AUTHOR]

Ganglioneuroblastoma (GNB) is a neoplasm that arises from the primitive cells of the sympathetic nervous system during childhood. The current case is very unique because of the initial primary tumor manifestation in the orbit and an adrenal tumor being observed later during follow-up.

Neonatal cholestasis is a potentially life-threatening condition requiring prompt diagnosis. Mutations in several different genes can cause progressive familial intrahepatic cholestasis, but known genes cannot account for all familial cases. Here we report four individuals from two unrelated families with neonatal cholestasis and mutations in NR1H4, which encodes the farnesoid X receptor (FXR), a bile acid-activated nuclear hormone receptor that regulates bile acid metabolism. Clinical features of severe, persistent NR1H4-related cholestasis include neonatal onset with rapid progression to end-stage liver disease, vitamin K-independent coagulopathy, low-to-normal serum gamma-glutamyl transferase activity, elevated serum alpha-fetoprotein and undetectable liver bile salt export pump (ABCB11) expression. Our findings demonstrate a pivotal function for FXR in bile acid homeostasis and liver protection.

The Late Quaternary activity characteristics of secondary faults located between the main active faults at the boundaries of large basins are of great significance to the overall understanding of regional seismic hazards. The Wulashan Northern Fault (WNF) is located on the northern side of the Ordos Block, within the Northern Margin Fault Basin in North China, between the Sertengshan Piedmont Fault and Daqingshan Piedmont Fault. Current research on the geometry and kinematics of the WNF needs to be improved. In this study, we aimed to determine the shallow structural characteristics and Late Quaternary activity of the WNF using shallow seismic exploration and composite drilling geological cross-sectional analysis. The results indicate that the WNF is not a single surface fault but multiple branches with a northward-dipping stepped surface distribution. The latest activity of the F1 branch with a maximum coseismic vertical dislocation of 0.9 m occurred before 47.08 ± 3.7 ka B.P. The latest and older activities of the branch of F2 with a maximum coseismic vertical dislocation of 0.96 m and 1.15 m occurred before 73.8 ± 2.8 ka B.P. and 91.2 ± 4.4 ka B.P., respectively. According to a series of empirical relationships between length of surface rupture and magnitude, the maximum potential magnitude of the earthquake was determined to be M = 6.5-7.0. We argue that even though the Late Quaternary activity of the WNF was weaker than that of the other boundary faults of the Hetao Basin, the local urban and rural planning and land and resources construction in the Hetao Basin region should pay attention to the seismic risk of the WNF as an independent section in the future for the effect of secular tectonic loading. [ABSTRACT FROM AUTHOR]

Background: The present study reviewed the clinicopathological features and outcomes of bilateral lacrimal gland lesions. Methods: The data of 113 patients who underwent lacrimal gland biopsy at the West China Hospital of Sichuan University, China, between January 1, 2010, and December 31, 2021, are presented in this case series. The patients all presented with bilateral lacrimal gland lesions. The collected data included patient demographics, clinical features, the results of laboratory examinations, imaging presentations, histopathological diagnoses, treatments, and outcomes. Results: The mean age of the 113 enrolled patients was 47.4 ± 14.9 years (range, 11–77 years) with a predominance of females (54.9%, n = 62). The lacrimal gland was the source of the majority of biopsy tissue (98.2%, n = 111). The most prevalent etiology was immunoglobulin G4-related ophthalmic disease (IgG4-ROD) (32.7%, n = 37), followed by idiopathic orbital inflammation (IOI) (28.3%, n = 32), mucosa-associated lymphoid tissue (MALT) lymphoma (17.7%, n = 20), reactive lymphoid hyperplasia (RLH) (10.6%, n = 12), and mantle cell lymphoma (4.4%, n = 5). Patients with IOI were significantly younger than those with IgG4-ROD and MALT lymphoma (t = 2.932, P = 0.005; t = 3.865, P<0.001, respectively). Systemic symptoms were more prevalent among patients with IgG4-ROD (χ2 = 7.916, P = 0.005). The majority of patients were treated with surgery (53.1%, n = 60), with surgery combined with corticosteroid therapy (21.2%, n = 24) being the second most common treatment. The majority of patients (91.2%, n = 103) attained complete resolution, stable disease, or significant improvement. Conclusion: In conclusion, there are several aetiologies associated with bilateral lacrimal gland lesions, the most prevalent being IgG4-ROD, IOI, and MALT lymphoma. Systemic symptoms were more common in patients with IgG4-ROD. The majority of patients who presented with bilateral lesions of the lacrimal glands responded satisfactorily to treatment, with favorable results. [ABSTRACT FROM AUTHOR]

Cenegermin, a recombinant human nerve growth factor, is an orphan drug approved for neurotrophic keratitis. The safety information on the label is incomplete, and the adverse reactions noted are mostly mild and tolerable. However, the occurrence of painful epithelial plagues and irreversible corneal deposits after cenegermin usage have been reported. Real-world data on long-term ocular safety are lacking. We aimed to assess the cenegermin-associated eye safety profile in the FDA pharmacovigilance database. The signals of cenegermin-related ocular adverse events (AEs) from 2018 to 2022 were quantified using the reporting odds ratio (ROR) and information component (IC). The grading system was used to prioritize the signals. We identified 3288 cases of cenegermin-related ocular AEs and 56 positive ocular-related signals. Fifty unexpected signals of ocular AE were identified. Eye ulcer was classified as a designated medical event. Twenty AEs, including corneal perforation, eye infection, corneal deposits, and eye inflammation, were recognized as important medical event. The median onset time for ocular AEs was 6 days (interquartile range [IQR]: 1–29 days). This study revealed new cenegermin-related ocular AE signals. Clinical practice requires close monitoring to early identify and manage adverse reactions that may cause occurrence of serious irreversible consequences. [ABSTRACT FROM AUTHOR]