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Objective:To investigate the effect of ultrasound-guided stellate ganglion block (SGB) on cerebral oxygen metabolism and serum S100B protein during carotid endarterectomy (CEA).Methods:Patients aged 40-75 years old, classified as Grade Ⅱ-Ⅲ by the American Society of Anesthesiologists (ASA), and underwent elective CEA under general anesthesia at the Affiliated Suzhou Hospital of Nanjing Medical University from June 2021 to April 2023 were prospectively enrolled. They were randomly divided into an SGB group and a control group. Before anesthesia induction, the SGB group underwent ipsilateral SGB under the ultrasound guidance, while the control group did not undergo SGB. The right subclavian vein catheterization was performed under the ultrasound guidance during the general anesthesia. The mean arterial pressure (MAP) and heart rate (HR) were recorded before induction of general anesthesia (T0), during tracheal intubation (T1), before vascular occlusion (T2), after vascular opening (T3), and at the end of surgery (T4), as well as the pressure of the jugular vein bulb at each time point from T1 to T4. Arterial blood and jugular venous bulb blood were collected at various time points for blood gas analysis. Jugular venous bulb oxygen saturation (SjvO 2), arteriovenous oxygen content difference (AVDO 2), cerebral oxygen extraction rate (COER), lactate production rate (LPR) and lactate oxygen index (LOI) were calculated. The serum S100B concentration in the jugular vein bulb blood at various time points was detected with enzyme-linked immunosorbent assay. The incidence of postoperative hoarseness, hematoma, dizziness, diaphragmatic nerve block, nausea, and vomiting were recorded. Results:A total of 82 patients conducted CEA were included, with 41 patients in the SGB group and 41 in the control group. During anesthesia induction and surgery in the SGB group, HR was significantly lower than that in the control group, and the MAP and HR during tracheal intubation and at the beginning of surgery were also more stable than those in the control group (all P<0.05). In the SGB group, the changes in SjvO 2, AVDO 2, and COER were relatively smaller from T1 to T3, while SjvO 2 increased, and AVDO 2 and CEOR decreased at T4. In contrast, the control group showed a decrease in SjvO 2, AVDO 2, and COER at T3 and a slight increase at T4. At all time points, SjvO 2 in the SGB group was significantly higher than that in the control group ( P<0.05). AVDO 2 and COER in both groups gradually decreased over time, and the control group was significantly higher than the SGB group at all time points (all P<0.05). LPR and LOI increased at T1 to T4 in both groups, reaching their highest value at T3 and decreasing at T4. There was statistically significant difference at T4 and at T2 in the control group (all P<0.05). The LPR and LOI of the control group were significantly higher than those of the SGB group at all time points (all P<0.05). In addition, the serum S100B levels in both groups increased first and then decreased, but the T2-T4 levels in the SGB group were significantly lower than those in the control group at all time points (all P<0.05). The incidence of perioperative adverse events in the SGB group was significantly lower than that in the control group ( P<0.05). Conclusion:Performing ipsilateral SGB before CEA surgery can effectively inhibit stress response, maintain intraoperative hemodynamic stability, improve brain tissue oxygen supply, and have a certain neuroprotective effect.
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The clinical features and gene mutation characteristics of 2 children who suffered from Cornelia de Lange syndrome(CdLs)and were admitted to Affiliated Hospital of Zunyi Medical University in March 2019 were retrospectively analyzed.The 2 cases developed in infancy and presented with intractable epilepsy were accompanied with developmental retardation and special appearance.It was obvious that the 2 children had SMC1A gene mutations on X chromosome.Case 1 was frameshift mutations in the SMC1A gene at c. 2561dupA(p.K854fs), and case 2 was mutations in the SMC1A gene at c. 3441+ 1G>A(exon24)splicing mutation.These were heterozygous de novo mutations, and weren′t detected in their parents, which was not reported in literatures.In this study, 2 cases of CdLs were caused by SMC1A gene mutation, which enriched the human gene mutation database.CdLs should be considered in children with early-onset epilepsy, especially appearance and developmental retardation.Genetic testing is the most important diagnostic method.
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The clinical characteristics, diagnosis and treatment, and the gene mutation of 2 different phenotypes patients developed with tyrosine hydroxylase deficiency (THD) were retrospectively analyzed.Case 1 was a severe infantile parkinsonism accompanied with motor retardation, which started with psychomotor retardation without dystonia in infantile period.Clinical symptoms were fluctuating.Case 2 was a mild dopa-responsive dystonia, which started with progressive lower extremity dystonia in school age.The genetic study revealed that both patients had heterozygous mutations in tyrosine hydroxylase ( TH) gene.Case 1 was compound heterozygous mutations in the TH gene at c. 457C>T(paternal) and c. 698G>A (maternal). Case 2 was compound heterozygous mutations in the TH gene at c. 457C>T(paternal) and c. 1481C>T (maternal). Both patients dramatically improved after the treatment with Levodopa.THD should be considered in any children with or without mental retardation presenting with fluctuations symptoms or fluc-tuations dyskinesia.Genetic testing is the most important diagnostic method.
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Objective To observe the therapeutic effects and side effects of FLAG regimen(fludarabine, Ara-C, and granulocyte-colony stimulating factor( C-CSF) for refractory or relapsed acute myeloid leukemia( AML). Methods Twenty-six AML were treated with fludarabine plus intermediate-dose Ara-C and G-CSF,of whom 15 cases belonged to refractory and 11 cases belonged to relapsed. Results After two courses of treatment, 14 cases were completely relieved (53. 8% ) and 5 cases were partially relieved (19.2% ). The overall effective rates was 73.1%. The main side effects were severe myelosuppression and non-hematological toxicity was mild. Conclusion FLAG regimen was very effective for refractory or relapsed acute myeloid leukemia and was well tolerated. The treatment-related mortality rate was low,so it provided a treatment choice for these patients.