Objective Calcitonin gene-related peptide (CGRP), neurotensin (NT) and atrial natriuretic polypeptide (ANP) have strong vaso-dilating and positive inotropic action, whereas neuropeptide Y (NPY) and endothelin (ET) have vaso-constricting effect. They all have profound effects on hemodynamics. The purpose of this study was to examine the changes in the 5 neuropeptides in elderly hypertensive patients under general anesthesia.Methods The control group comprised eight men and six women without hypertension with a mean age of (69?8)yr. The hypertension group included nine men and five women with a mean age of (71?9)yr. The patients in the hypertension group had different degrees of myocardial hypertrophy and ST-T changes. All 28 patients underwent upper abdominal surgery. Anesthesia was induced with propofol 2mg?kg -1 and fentanyl 2?g?kg -1 and intubation was facilitated with succinylcholine 2mg?kg -1. The patients were mechanically ventilated after intubation. Anesthesia was maintained with 50% N 2O and enflurane or isoflurane inhalation supplemented with intermittent doses of vecuronium. Venous blood samples were taken before anesthesia, 3min after intubation, 60 min after start of surgery, at the end of surgery and 24 h after operation for determination of plasma levels of CGRP, NT, ANP, NPY and ET by radioimmunoassay.Results In hypertension group plasma level of ANP decreased and NPY increased significantly at the end of operation and 24 h after operation (P

Objective: To analyze the clinical and molecular diagnostic status of Fanconi anemia (FA) in China. Methods: The General situation, clinical manifestations and chromosome breakage test and genetic test results of 107 pediatric FA cases registered in the Chinese Blood and Marrow Transplantation Registry Group (CBMTRG) and the Chinese Children Blood and Marrow Transplantation Registry Group (CCBMTRG) from August 2009 to January 2022 were analyzed retrospectively. Children with FANCA gene variants were divided into mild and severe groups based on the type of variant, and Wilcoxon-test was used to compare the phenotypic differences between groups. Results: Of the 176 registered FA patients, 69 (39.2%) cases were excluded due to lack of definitive genetic diagnosis results, and the remaining 107 children from 15 hospitals were included in the study, including 70 males and 37 females. The age at transplantation treatment were 6 (4, 9) years. The enrolled children were involved in 10 pathogenic genes, including 89 cases of FANCA gene, 7 cases of FANCG gene, 3 cases of FANCB gene, 2 cases of FANCE gene and 1 case each of FANCC, FANCD1, FANCD2, FANCF, FANCJ, and FANCN gene. Compound heterozygous or homozygous of loss-of-function variants account for 69.2% (72/104). Loss-of-function variants account for 79.2% (141/178) in FANCA gene variants, and 20.8% (37/178) were large exon deletions. Fifty-five children (51.4%) had chromosome breakage test records, with a positive rate of 81.8% (45/55). There were 172 congenital malformations in 80 children.Café-au-Lait spots (16.3%, 28/172), thumb deformities (16.3%,28/172), polydactyly (13.9%, 24/172), and short stature (12.2%, 21/172) were the most common congenital malformations in Chinese children with FA. No significant difference was found in the number of congenital malformations between children with severe (50 cases) and mild FANCA variants (26 cases) (Z=-1.33, P=0.185). Conclusions: FANCA gene is the main pathogenic gene in children with FA, where the detection of its exon deletion should be strengthened clinically. There were no phenotypic differences among children with different types of FANCA variants. Chromosome break test is helpful to determine the pathogenicity of variants, but its accuracy needs to be improved.
Male ; Female ; Humans ; Child ; Fanconi Anemia/genetics* ; Chromosome Breakage ; Retrospective Studies ; Exons ; China/epidemiology*