Unique Characteristics of Patients with Von Hippel-Lindau Disease Defined by Various Diagnostic Criteria.

Von Hippel-Lindau (VHL) disease diagnosis is based on two criteria sets: International criteria (IC, two hemangioblastomas, one hemangioblastoma plus one visceral lesion, or VHL family history/pathogenic variant plus hemangioblastoma/visceral lesion); or Danish criteria (DC, two clinical manifestations, or VHL family history/pathogenic variant plus hemangioblastoma/visceral lesion). We aimed to compare the characteristics of patients with VHL-related pancreatic neuroendocrine tumor (vPNET) meeting either the clinical Danish criteria only (DOC) or IC to those with sporadic PNET (sPNET). The cohort included 33 patients with VHL (20 vPNETs) and 65 with sPNET. In terms of genetic testing and family history of VHL, 90.0% of the patients with vPNET in the IC group had a germline VHL pathogenic variant, and 70.0% had a family history of VHL vs. 20% and 10% in the DOC group, respectively (p < 0.05 for both). Patients with vPNET were younger at diagnosis compared with sPNET (51.6 ± 4.1 vs. 62.8 ± 1.5 years, p < 0.05). Patients in the IC group were younger at diagnosis with VHL, vPNET, pheochromocytoma, or paraganglioma (PPGL) and renal-cell carcinoma (RCC) than those in the DOC group (p < 0.05 for all comparisons). The most prevalent presenting manifestations were hemangioblastoma (42.8%) and PPGL (33.3%) vs. RCC (58.3%) and PNET (41.7%) in the IC vs. DOC groups. In conclusion, patients with vPNET meeting DOC criteria show greater similarity to sPNET. We suggest performing genetic testing, rather than solely using clinical criteria, for establishing the diagnosis of VHL.

An impact of lipid profile and lipid lowering drugs on ≥70 year olds of an upper socioeconomic class: a retrospective cohort study.

BACKGROUND: Life expectancy has greatly increased, generating an improvement in screening programs for disease prevention, lifesaving drugs and medical devices. The impact of lowering low-density lipoprotein cholesterol (LDL-C) in the very elderly is not well-established. Our aim was to explore the association of LDL-C, high density lipoprotein cholesterol (HDL-C) and lipid lowering drugs (LLDs) on cognitive decline, malignancies and overall survival. METHODS: This was a retrospective cohort study. Our study comprised 1498 (72.7%) males and 561 (27.3%) females, aged ≥70 who had attended the Institute for Medical Screening (IMS), Sheba Medical Center, Israel at least twice during 2013-2019. Data were obtained from the computerized database of the IMS. A manual quality control to identify potential discrepancies was performed. RESULTS: Overall, 6.3% of the subjects treated with LLDs (95/1421) versus 4.2% not treated (28/638), cognitively declined during the study years. No statistically significant effects of LDL-C, HDL-C and LLDs on cognitive decline were observed after correcting for age, prior stroke and other vascular risk factors. With regard to cancer, after adjusting for confounders and multiple inferences, no definite relationships were found. CONCLUSIONS: This analysis of an elderly, high socioeconomic status cohort suggests several relationships between the use of LLDs and health outcomes, some beneficial, especially, with regard to certain types of cancer, but with a higher risk of cognitive decline. Further studies are warranted to clarify the health effects of these medications in the elderly.

Delivery outcomes in subsequent pregnancy following primary breech cesarean delivery: a retrospective cohort study.

Background: Women may be unaware of the potential impact on subsequent deliveries of the decision to undergo primary breech cesarean (PBC) delivery rather than attempt external cephalic version (ECV). We investigated cesarean delivery rates and adverse maternal outcomes in the subsequent delivery following management of breech presentation by ECV versus PBC.Methods: This retrospective study identified women who delivered subsequent to breech presentation managed by attempted ECV or PBC. We assessed cesarean delivery rates and adverse maternal outcomes including hemorrhage, placental complications, infection, and obstetric complications in the subsequent delivery following attempted ECV versus PBC.Results: We identified 307 women who had attempted ECV and 508 with PBC in the index breech pregnancy. Following attempted ECV, 47 (15.3%) women had cesarean delivery in the subsequent pregnancy versus 246 (48.4%) following PBC, adjusted OR 0.24, 95% CI 0.16-0.35 for subsequent cesarean delivery after attempted ECV in the index pregnancy. The frequency of composite adverse outcomes related to cesarean delivery was 30 (9.8%) following attempted ECV versus 104 (20.5%) after PBC, p < .0001. The frequency of composite severe adverse maternal outcome was similar following attempted ECV 25 (8.8%) versus PBC 59 (11.9%).Conclusions: The current study suggests that attempted external cephalic version was associated with a five-fold decrease risk to undergo cesarean in the subsequent delivery and a significantly decreased frequency of composite adverse outcomes related to cesarean delivery in the subsequent delivery.