Nutritional status as a predictive factor for paediatric tuberous sclerosis complex-associated kidney angiomyolipomas: a retrospective analysis.

The purpose of this study is to determine the predictive factors of tuberous sclerosis complex (TSC)-associated kidney disease and its progression in children. Retrospective review of children with TSC in a tertiary children's hospital was performed. Relevant data were extracted, and Cox proportional hazards regression was used to establish predictors of kidney lesions. Logistic regression was conducted to identify factors predicting chronic kidney disease (CKD) and high-risk angiomyolipomas (above 3 cm). Kidney imaging data were available in 145 children with TSC; of these, 79% (114/145) had abnormal findings. The only significant predictive factor for cyst development was being female (HR = 0.503, 95% CI 0.264-0.956). Being female (HR = 0.505, 95% CI 0.272-0.937) and underweight (HR = 0.092, 95% CI 0.011-0.800) both lowers the risk of having angiomyolipomas, but TSC2 mutations (HR = 2.568, 95% CI 1.101-5.989) and being obese (HR = 2.555, 95%CI 1.243-5.255) increases risks. Ten (12%) of 81 children with kidney function tested demonstrate CKD stages II-V, and only angiomyolipomas above 3 cm predict CKD. Additionally, 13/145 (9%) children had high-risk angiomyolipomas, whereby current age (adjusted odds ratio (aOR) 1.015, 95% CI 1.004-1.026) and being overweight/obese (aOR 7.129, 95% CI 1.940-26.202) were significantly associated with angiomyolipomas above 3 cm. CONCLUSIONS: While gender and genotype are known predictors, this study includes the novel finding of nutritional status as a predictor of TSC-associated kidney disease. This study sheds light on a possible complex interplay of hormonal influences, obesity, and kidney angiomyolipomas growth, and further investigations focusing on the impact of nutritional status on TSC-associated kidney disease are warranted. WHAT IS KNOWN: • Gender and genotype are well-studied predictive factors in TSC kidney disease. WHAT IS NEW: • Nutritional status may influence the development and the progression of kidney lesions in children with TSC and should not be overlooked. • Management guidelines of TSC-associated kidney disease can address nutritional aspects.

Guarding the gatekeepers: a comprehensive approach to control nosocomial measles.

PURPOSE: Despite substantial vaccination progress, persistent measles outbreaks challenge global elimination efforts, particularly within healthcare settings. In this paper, we critically review the factors contributing to measles outbreak and effective control measures for nosocomial transmission of measles. METHODS: We systematically searched electronic databases for articles up to 17th May, 2023. This was performed by two independent reviewers, with any disagreements resolved by a third reviewer. We also searched governmental and international health agencies for relevant studies. RESULTS: Forty relevant articles were systematically reviewed, revealing key factors fuelling measles outbreak in healthcare settings, including high transmissibility capability; high intensity exposure; delayed care; failure to use protective equipment and implement control measures; vaccine failure; unclear immunisation history and lack of registries; and lacking recommendation on healthcare workers' (HCWs) measles vaccination. To combat these challenges, successful control strategies were identified which include early notification of outbreak and contact tracing; triaging all cases and setting up dedicated isolation unit; strengthening protective equipment use and physical measures; improving case detection; determining immunity status of HCWs; establishing policy for measles vaccination for HCWs; management of exposed personnel; and developing a pre-incident response plan. CONCLUSION: A coordinated and comprehensive approach is essential to promptly identify and manage measles cases within healthcare settings, necessitating multifactorial strategies tailored to individual settings. These findings provide a valuable foundation for refining strategies to achieve and maintain measles elimination status in healthcare environments.

Henoch-Schönlein Purpura in an Obese Adult with Elevated Transaminase: A Case Report and Literature Review.

Background: Henoch-Schönlein Purpura (HSP) cases are uncommon among adults due to their self-limiting nature and difficulty in diagnosis. The involvement of the hepatobiliary system and the effect of obesity is still not well understood among adults. Case Description: A 28-year-old obese male presented with an acute onset of generalized palpable purpura, fever, abdominal discomfort, and arthralgias. Laboratory examination indicated leukocytosis (43,100/µL), thrombocytosis (550,000/µL), and elevated transaminase levels (AST 125 U/L, ALT 131 U/L). Skin biopsy revealed perivascular inflammatory infiltrates in the superficial dermal vessels. Treatment of corticosteroids and antibiotics resulted in progressive clinical improvement. Conclusion: Although rare in adults, recognizing multiple presentations of HSP allows early diagnosis. This case highlights elevated transaminase levels and obesity contributed to increased inflammatory responses that may complicate HSP diagnosis. Despite severity, a complete recovery is possible if diagnosed early and managed appropriately.