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Sökning: L773:0021 4787

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1.
  • Touraine, Philippe, et al. (författare)
  • Pituitary function and the response to GH therapy in patients with Langerhans cell histiocytosis : analysis of the KIMS database
  • 2022
  • Ingår i: Yosetsu Gakkai Shi/Journal of the Japan Welding Society. - 0021-4787. ; 187:3, s. 373-381
  • Tidskriftsartikel (refereegranskat)abstract
    • Objective: To analyze the effectiveness and safety of growth hormone (GH) replacement treatment in adult patients with Langerhans cell histiocytosis (LCH) and GH deficiency (GHD) enrolled in KIMS (Pfizer International Metabolic Database). Patients and methods: Patients with LCH and GHD were studied at baseline and some of them after 1 year of GH treatment. The effectiveness of GH is presented as change after 1 year of treatment (mean, 95% CI). The LCH population was compared to two other groups of patients enrolled in KIMS, granulomatous and lymphocytic hypophysitis. Results: At baseline, 81 adults with LCH (27 with childhood onset, 56% females), mean age at GHD onset of 29 (15) years were studied. Diabetes insipidus was diagnosed in 86% of patients. Analysis of 1 year of GH treatment was possible in 37 patients. One-year cross-sectional values for the GH dose were 0.39 (S.D. ± 0.21) mg and -0.5 (-1.2 to 0.2) for insulin-like growth factor-1 S.D. Total cholesterol decreased 0.9 (-1.5 to -0.3 (mmol/L); P < 0.05); AGHDA-QoLscore (n = 20) was improved by 2.8 points (-5.6 to 0.0; P < 0.05), while mean BMI increased 0.6 ± 3 kg/m2 (95% CI: -0.2 to 1.4). All these effects did not differ from the two other groups after adjusting for age, gender, and baseline values. In 20 of 77 patients included in the safety analysis, 36 serious adverse events were reported during 435 patient-years (82.8/1000); no new safety signals were reported. Conclusion: After 1 year of GH treatment in patients with LCH, metabolic variables and quality of life improved, with no new safety signals.
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2.
  • Kok, Joeri, et al. (författare)
  • Femoral strength and strains in sideways fall : Validation of finite element models against bilateral strain measurements
  • 2021
  • Ingår i: Journal of Biomechanics. - : Elsevier BV. - 1873-2380 .- 0021-9290. ; 122
  • Tidskriftsartikel (refereegranskat)abstract
    • Low impact falls to the side are the main cause of hip fractures in elderly. Finite element (FE) models of the proximal femur may help in the assessment of patients at high risk for a hip fracture. However, extensive validation is essential before these models can be used in a clinical setting. This study aims to use strain measurements from bilateral digital image correlation to validate an FE model against ex vivo experimental data of proximal femora under a sideways fall loading condition. For twelve subjects, full-field strain measurements were available on the medial and lateral side of the femoral neck. In this study, subject-specific FE models were generated based on a consolidated procedure previously validated for stance loading. The material description included strain rate dependency and separate yield and fracture strain limits in tension and compression. FE predicted fracture force and experimentally measured peak forces showed a strong correlation (R2 = 0.92). The FE simulations predicted the fracture initiation within 3 mm distance of the experimental fracture line for 8/12 subjects. The predicted and measured strains correlated well on both the medial side (R2 = 0.87) and the lateral side (R2 = 0.74). The lower correlation on the lateral side is attributed to the irregularity of the cortex and presence of vessel holes in this region. The combined validation against bilateral full-field strain measurements and peak forces has opened the door for a more elaborate qualitative and quantitative validation of FE models of femora under sideways fall loading.
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3.
  • Bengtsson, Daniel, 1975-, et al. (författare)
  • Corticotroph Pituitary Carcinoma in a Patient With Lynch Syndrome (LS) and Pituitary Tumors in a Nationwide LS Cohort
  • 2017
  • Ingår i: Journal of Clinical Endocrinology and Metabolism. - : OXFORD UNIV PRESS INC. - 0021-972X .- 1945-7197. ; 102:11, s. 3928-3932
  • Tidskriftsartikel (refereegranskat)abstract
    • Context: Lynch syndrome (LS) is a cancer-predisposing syndrome caused by germline mutations in genes involved in DNA mismatch repair (MMR). Patients are at high risk for several types of cancer, but pituitary tumors have not previously been reported. Case: A 51-year-old man with LS (MSH2 mutation) and a history of colon carcinoma presented with severe Cushing disease and a locally aggressive pituitary tumor. The tumor harbored a mutation consistent with the patients germline mutation and displayed defect MMR function. Sixteen months later, the tumor had developed into a carcinoma with widespread liver metastases. The patient prompted us to perform a nationwide study in LS. Nationwide Study: A diagnosis consistent with a pituitary tumor was sought for in the Swedish National Patient Registry. In 910 patients with LS, representing all known cases in Sweden, another two clinically relevant pituitary tumors were found: an invasive nonsecreting macroadenoma and a microprolactinoma (i.e., in total three tumors vs. one expected). Conclusion: Germline mutations in MMR genes may contribute to the development and/or the clinical course of pituitary tumors. Because tumors with MMR mutations are susceptible to treatment with immune checkpoint inhibitors, we suggest to actively ask for a family history of LS in the workup of patients with aggressive pituitary tumors.
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